Vol.

Comprehensive
Textbook of Echocardiography

Vol.
Comprehensive
Textbook of Echocardiography

Editor

Navin C Nanda MD

Distinguished Professor of Medicine and Cardiovascular Disease and

Director, Heart Station/Echocardiography Laboratories
University of Alabama at Birmingham and the University of
Alabama Health Services Foundation
The Kirklin Clinic, Birmingham, Alabama, USA
President, International Society of Cardiovascular Ultrasound

Under the Aegis of

The International Society of Cardiovascular Ultrasound
and
The Indian Academy of Echocardiography

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Comprehensive Textbook of Echocardiography (Vol. 2)
First Edition: 2014
ISBN978-93-5090-634-7
Printed at:

Dedicated to
My late parents
Balwant Rai Nanda MD and Mrs Maya Vati Nanda
My wife
Kanta Nanda MD
Our children
Nitin Nanda, Anita Nanda Wasan MD and Anil Nanda MD
Their spouses Sanjeev Wasan MD and Seema Tailor Nanda, and
our grandchildren Vinay and Rajesh Wasan, and Nayna and Ria Nanda

Contributors
Masood Ahmad M
 D FRCP (C) FACP FACC

FAHA FASE

Division of Cardiology
Department of Internal Medicine
University of Texas Medical Branch
Galveston Texas, USA

Dheeraj Arora DNB PDCC MNAMS

Institute of Critical Care and Anesthesia
Medanta The Medicity
Gurgaon, Haryana, India

Mohammad Al-Admawi MD
King Faisal Specialist Hospital and
Research Center
Heart Center
Riyadh, Saudi Arabia

Bader Almahdi MD

Manreet Basra MBBS

Monodeep Biswas MBBS MD

Professor of Medicine
University at Buffalo School of Medicine
and Biological Sciences
New York, USA

Division of Cardiology
Geisinger-Community Medical
Center, and The Wright Center for
Graduate Medical Education
Scranton, Pennsylvania, USA

Charles E Beale MD
Department of Medicine
Division of Cardiovascular Diseases
Stony Brook University Medical Center
Stony Brook, New York, USA

Roy Beigel MD
The Heart Institute, Cedars Sinai Medical
Center, Los Angeles, California, USA
The Leviev Heart Center Sheba Medical
Center, Affiliated to the Sackler
School of Medicine
Tel Aviv University, Tel Aviv, Israel

Steven Bleich MD
Department of Medicine
Division of Internal Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA

O Julian Booker MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA

Eduardo Bossone MD PhD FCCP FESC FACC

Echocardiography and Vascular Lab

Assistant Professor of Medicine
New York University School of Medicine
New York, New York, USA

Via Principe Amedeo Lauro (AV), Italy

Heart Department, University of
Salerno, Scuola Medica Salernitana
Salerno, Italy
Department of Cardiac Surgery IRCCS
Policlinico San Donato, Milan, Italy

Division of Cardiology
Department of Medicine
University of Texas Medical Branch
Galveston, Texas, USA

Kunal Bhagatwala MBBS

Luis Bowen MD

Division of Cardiovascular Disease

University of Alabama at Birmingham
Birmingham, Alabama, USA

Department of Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA

Neeraj Awasthy FNB

Aditya Bharadwaj MD

Gerald Buckberg MD

Department of Cardiology
Loma Linda University and
VA Medical Centers
Loma Linda, California, USA

Department of Cardiothoracic Surgery

David Geffen School of Medicine
University of California-Los Angeles
Los Angeles, California, USA

Heart Center, St Christophers Hospital

for Children and
Section of Cardiology
Department of Pediatrics, Drexel
University College of Medicine
Philadelphia, Pennsylvania, USA

Aarti H Bhat MBBS

Michael J Campbell MD

Assistant Professor
Division of Pediatric Cardiology
Seattle Childrens Hospital and
University of Washington
Seattle, Washington, USA

Department of Pediatrics
Division of Pediatric Cardiology
Duke University Medical Center
Durham, North Carolina, USA

Piers Barker MD

Nicole Bhave MD

FRCP FACC

Department of Pediatrics
Division of Pediatric Cardiology
Duke University Medical Center
Durham, North Carolina, USA

University Health Network

Toronto General Hospital
University of Toronto
Toronto, Ontario, Canada

Professor Emeritus
Division of Cardiology
UC-Irvine School of Medicine
Irvine, California, USA

King Faisal Specialist Hospital and

Research Center
Heart Center
Riyadh, Saudi Arabia

Ahmed Almomani MBBS

Fortis Escorts Heart Institute

New Delhi, India

Rula Balluz MD MPH

Ricardo Benenstein MD

Premindra PAN Chandraratna MD

viii

Comprehensive Textbook of Echocardiography

Leon H Charney

Michele D Alto MD PhD

Daniel Forsha MD

Division of Cardiology
New York University Medical Center
New York, New York, USA

Department of Cardiology
Second University of Naples:
Monaldi Hospital, Naples, Italy

Department of Pediatrics
Division of Pediatric Cardiology
Duke University Medical Center
Durham, North Carolina, USA

Farooq A Chaudhry M
 D FACP FACC

David Daly MD

FASE FAHA

Professor of Medicine
Director, Echocardiography Laboratories
Associate Director, Mount Sinai Heart
Network, Icahn School of Medicine at
Mount Sinai, Zena and Michael A Wiener
Cardiovascular Institute and
Marie-Jose and Henry R Kravis Center
for Cardiovascular Health
New York, New York, USA

Preeti Chaurasia MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama

Reema Chugh MD FACC

Consultant in Cardiology/Specialist in
Adult Congenital Heart Disease and
Heart Disease in Pregnancy
Kaiser Permanente Medical Center
Panorama City, California, USA

Krishnaswamy Chandrasekaran MD
Mayo Clinic, Scottsdale, Arizona, USA
Rochester, Minnesota, USA

Michael Chen MD
University of Washington
Seattle, Washington DC, USA

HK Chopra MD
Moolchand City Hospital
New Delhi, India

Department of Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA

Hisham Dokainish M
 D FRCPC
Associate Professor of Medicine
McMaster University
Director of Echocardiography and
Medical Diagnostic Units
Hamilton Health Sciences
Hamilton, Ontario, Canada

Maximiliano German
Amado Escauela MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA

Bahaa M Fadel MD
King Faisal Specialist Hospital and
Research Center
Heart Center
Riyadh, Saudi Arabia

Naveen Garg MBBS Dip. Cardiology

Fellow, Noninvasive Cardiac Lab
Indraprastha Apollo Hospitals
New Delhi, India

Luna Gargani MD
Institute of Clinical Physiology
National Research Council
Pisa, Italy

Eleonora Gashi DO MPhil

Robert P Gatewood Jr MD FACC

Division of Cardiology
Fondazione Cardiocentro Ticino
Lugano, Switzerland

Division of Cardiovascular Disease

University of Alabama at Birmingham
Birmingham, Alabama, USA

Honorary Consultant
Imperial and King's Colleges, London, UK

University of Illinois Hospital & Health

Science System
Jesse Brown VA Medical Center
Chicago, Illinois, USA

Francesco Faletra MD

Francesco Ferrara MD

David Cosgrove MD

Leon J Frazin MD

Fellow, Division of Cardiology

Allegheny General Hospital
Pittsburgh, Pennsylvania, USA

Heart Department, University of Salerno

Scuola Medica Salernitana
Salerno, Italy
Department of Internal Medicine and
Cardiovascular Sciences
University Federico II of Naples
Naples, Italy

Director, Division of Clinical Cardiology

Program Director, Cardiovascular
Fellowship, Lenox Hill Hospital
New York, USA

Department of Cardiology
Loma Linda University and
VA Medical Centers, Loma Linda
California, USA

Senior Cardiology Fellow

Lenox Hill Hospital
Non-Invasive Cardiology
New York, New York, USA

Abid Ali Fakhri MD

Cecil Coghlan MD

Neil L Coplan MD FACC

Gary P Foster MD

Brandon Fornwalt MD PhD

Assistant Professor of Pediatrics
Department of Pediatrics
University of Kentucky
Lexington, Kentucky, USA

Chief of Cardiac Services

Kaleida Heath; Clinical Associate
Professor of Medicine
University at Buffalo School of
Medicine and Biological Sciences
Buffalo Cardiology and Pulmonary
Associates, Main Street Williamsville
New York, USA

Shuping Ge MD FAAP FACC FASE

Chief, Section of Cardiology
St Christophers Hospital for Children
Associate Professor of Pediatrics
Drexel University College of Medicine
Philadelphia, Pennsylvania, USA
Acting Chair, Pediatric Cardiology
Deborah Heart and Lung Center
Browns Mills, New Jersey, USA

Contributors

ix

Gopal Ghimire MD DM MRCP

Donald Hagler MD

Rachel Hughes-Doichev MD FASE

Division of Cardiovascular Diseases

University of Alabama at Birmingham
Birmingham, Alabama, USA

Mayo Clinic
Rochester, Minnesota, USA

Temple University School of Medicine

Pittsburgh, Pennsylvania, USA

Stephanie El-Hajj MD

Arzu Ilercil MD

Nina Ghosh MD

Department of Internal Medicine

Louisiana State University Health
Sciences Center
Baton Rouge, Louisiana, USA

Associate Professor of Medicine

Department of Cardiovascular Sciences
University of South Florida
Tampa, Florida, USA

Kamran Haleem MD

Trevor Jenkins MD

Division of Cardiovascular Medicine

Brigham and Womens Hospital
Harvard Medical School
Francis Street
Boston, Massachusetts, USA

Edward Gill MD
Professor of Medicine and
Cardiology, University of Washington
Seattle, Washington DC, USA

Rohit Gokhale MBBS

University at Buffalo
Buffalo, New York, USA

Aasha S Gopal MS MD FACC FAHA FASE

Associate Professor of Medicine
Stony Brook University
Stony Brook, New York, USA
Director, Advanced Echocardiography
St Francis Hospital, Washington Blvd
Roslyn, New York, USA

Willem Gorissen
Clinical Market Manager
Toshiba Medical Systems Europe
Zoetermeer, The Netherlands

Luis Gruberg MD FACC

Department of Medicine
Division of Cardiovascular Diseases
Stony Brook University Medical Center
Stony Brook, New York, USA

Rakesh Gupta MD
JROP Healthcare
New Delhi, India

Fadi G Hage MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Section of Cardiology, Birmingham
Veterans, Administration Medical Center
Birmingham, Alabama, USA

Yale University
New Haven, Connecticut, USA

Dan G Halpern MD
St Lukes-Roosevelt Hospital Center
Columbia University, College of
Physicians and Surgeons
New York, New York, USA

Rachel Harris MD MPH

Morehouse School of Medicine
Section of Cardiology
Assistant Professor
Echo Lab Co-Director
Grady Memorial Hospital
Atlanta, Georgia, USA

Christine Henri MD
Department of Cardiology
Heart Valve Disease Clinic
CHU Sart Tilman, University of
Lige, Belgium

Julien IE Hoffman MD
Department of Pediatrics
University of California
San Francisco, California, USA

Brian D Hoit MD
Director of Echocardiography
Harrington Heart & Vascular Center
University Hospitals of Cleveland
Texas, USA

Steven J Horn MD FACC FASE FASNC

SUNY Buffalo
Buffalo, New York, USA

Ming Chon Hsiung MD

Cardiologist
Cheng Hsin General Hospital
Taipei, Taiwan

Harrington Heart and

Vascular Institute
University Hospital Case
Medical Center, Cleveland
Ohio, USA

Madhavi Kadiyala MD
Saint Francis Hospital, Roslyn
New York, USA

Arshad Kamel MD
Department of Medicine
University of Alabama at Huntsville
Huntsville, Alabama, USA

Abdallah Kamouh MD
University of Buffalo
Buffalo, New York, USA

Poonam Malhotra Kapoor MD

All India Institute of
Medical Sciences
New Delhi, India

Kanwal K Kapur MD
DM Cardiology, Sr Consultant and Chief
Noninvasive Cardiology
Indraprastha Apollo Hospitals
New Delhi, India
Department of Noninvasive Cardiology
Indraprastha Apollo Hospitals
New Delhi, India

Nidhi M Karia MBBS

Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA

Jarosaw D Kasprzak MD
Chair and Department of Cardiology
Biegaski Hospital
Medical University of Lodz
Lodz, Poland

Comprehensive Textbook of Echocardiography

Martin G Keane MD FACC FAHA FASE

Arthur J Labovitz MD

Gerald R Marx MD

Professor of Medicine
Cardiology Section
Director of Echocardiography
Temple University School of Medicine
Parkinson Pavilion, Suite
North Broad Street, Philadelphia
Pennsylvania, USA

Professor of Medicine
Chair, Department of
Cardiovascular Sciences
University of South Florida
Tampa, Florida, USA

Associate Professor
Harvard School of Medicine
Senior Associate Cardiology
Boston Childrens Hospital
Boston, Massachusetts, USA

Jennifer K Lang MD

Wilson Mathias Jr MD

University at Buffalo
Buffalo, New York, USA

Heart Institute (InCor)

The University of So Paulo School of
Medicine and Fleury Group
So Paulo, Brazil

Tuba Kemalolu z MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA

Anant Kharod MD
Department of Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA

Jennifer Kiessling MD
Division of Cardiovascular
Diseases, University of
Alabama at Birmingham
Birmingham, Alabama, USA

Allan L Klein M
 D FRCP(C) FACC

Roberto M Lang MD
University of Chicago
Medical Center
Chicago, Illinois, USA

Fabrice Larrazet MD PhD

Department of Cardiology
Hpital Saint Camille
Bry sur Marne, France

Steve W Leung MD
Assistant Professor of Medicine
Division of Cardiovascular Medicine
University of Kentucky
Lexington, Kentucky, USA

FAHA FASE

Angele A A Mattoso MD
Heart Institute (InCor)
The University of So Paulo School of
Medicine, So Paulo, Brazil and
Santa Izabel Hospital, Salvador, Bahia

Sula Mazimba MD MPH

Division of Cardiovascular
Disease University of Alabama at
Birmingham
Birmingham, Alabama, USA

Anjlee M Mehta MD
Fellow, Division of Cardiology
Dartmouth-Hitchcock Heart and
Vascular Center
Lebanon, New Hampshire, USA

Director, CV Imaging Research and

Pericardial Center
Professor of Medicine, Cleveland Clinic
Heart and Vascular Institute
Department of Cardiovascular Medicine
Cleveland, Ohio, USA

Sachin Logani MD

Smadar Kort MD FACC FASE

Javier Lpez MD PhD

Division of Cardiovascular Disease

University of Alabama at Birmingham
Birmingham, Alabama, USA

Professor of Medicine
State University of New York
Stony Brook Director
Non Inavasive Cardiac Imaging Director
Echocardiography Diretor
Valve Center, Stony Brook Medicine
Stony Brook, New York, USA

Hospital Clinico Universitario de

Valladolid, Spain

Yatin Mehta MD MNAMS FRCA FAMS

Itzhak Kronzon M
 D FASE FACC FACP

Department of Medicine
Division of Cardiovascular Diseases
Stony Brook University
Medical Center, Stony Brook
New York, USA

FIACTA FTEE FICCM

Judy R Mangion MD
Division of Cardiovascular Medicine
Brigham and Womens Hospital
Harvard Medical School
Francis Street, Boston
Massachusetts, USA

FESC FAHA

Professor of Cardiology
Hofstra University
North Shore LIJ, School of Medicine
Chief of Noninvasive Cardiac Imaging
Lenox Hill Hospital
Noninvasive Cardiology
New York, New York, USA

Kruti Jayesh Mehta MBBS PGDCC

CN Manjunath MD DM
Director, Professor and Head
Department of Cardiology
Sri Jayadeva Institute of Cardiovascular
Sciences and Research
Bannergutta Road
Bengaluru, Karnataka, India

Institute of Critical
Care and Anesthesia
Medanta The Medicity
Gurgaon, Haryana, India

Julien Magne PhD

Department of Cardiology
Heart Valve Disease Clinic
CHU Sart Tilman, University of
Lige, Belgium

Andrew P Miller MD
Cardiovascular Associates
Birmingham, Alabama, USA

Contributors

Dilbahar S Mohar MD

Ryozo Omoto MD

Eugenio Picano MD PhD

Division of Cardiology
UC-Irvine School of Medicine
Irvine, California, USA

Professor Emeritus, Saitama Medical

University
Honorary Director, Saitama Medical
University Hospital
Moro-Hongou, Moroyama
Iruma-Gun, Saitama, Japan

Institute of Clinical Physiology

National Research Council
Pisa, Italy

Caroline Morbach MD
Yale University
New Haven, Connecticut, USA

Ahmad S Omran MD FACC FESC FASE

Loma Linda University Medical Center

Loma Linda, California , USA
Eisenhower Medical Center
Rancho Mirage, California, USA

Consultant Cardiologist
Head, Non-Invasive Cardiology Lab
King Abdulaziz Cardiac CenterRiyadh
Health AffairsMinistry of National Guard
Kingdom of Saudi Arabia

Nagaraja Moorthy MD DM

Jatinder Singh Pabla BSc (Hons) MBBS

Hoda Mojazi-Amiri MD

Assistant Professor
Department of Cardiology
Sri Jayadeva Institute of Cardiovascular
Sciences and Research
Bengaluru, Karnataka, India

Hirohiko Motoki MD
Cardiovascular Research
Imaging Fellow, Cleveland Clinic
Foundation, Cleveland, Ohio, USA

Bernhard Mumm
President and COO
TomTec Imaging Systems
GmbH, Edisonstr
Unterschleissheim, Germany

Rachel Myers RDCS

Allegheny General Hospital
Pittsburgh, Pennsylvania, USA

Navin C Nanda MD
Distinguished Professor of Medicine and
Cardiovascular Disease and
Director, Heart Station/Echocardiography
Laboratories, University of Alabama at
Birmingham and the University of
Alabama Health Services Foundation
The Kirklin Clinic, Birmingham
Alabama, USA, President, International
Society of Cardiovascular Ultrasound

Elizabeth Ofili MD MPH FACC

Morehouse School of Medicine
Chief of Section of Cardiology
Associate Dean of Clinical Research
Professor of Medicine
Atlanta, Georgia, USA

xi

Luc A Pierard MD PhD

Department of Cardiology
Heart Valve Disease Clinic
CHU Sart Tilman, University of
Lige, Belgium

Atif N Qasim MD MSCE

Assistant Professor of Medicine
University of California
San Francisco, California, USA

MRCP

Department of Cardiovascular Medicine

Northwick Park Hospital, Harrow, UK

Shyam Padmanabhan MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA

Ramdas G Pai MD
Professor of Medicine
Loma Linda University
Medical Center
Loma Linda, California, USA

Natesa G Pandian MD
Professor, Tufts University School of
Medicine, Director, Heart Valve Center
Co-Director, Cardiovascular
Imaging Center
Director, Cardiovascular Ultrasound
Research, Tufts Medical Center
Boston, Massachusetts, USA

Satish K Parashar MD
Metro Heart Institute
New Delhi, India

Anita Radhakrishnan MD
Fellow, Division of Cardiology
Allegheny General Hospital
Pittsburgh, Pennsylvania, USA

Peter S Rahko MD
Professor of Medicine
Division of Cardiovascular Medicine
Department of Medicine, University of
Wisconsin School of Medicine and Public
Health, Madison, Wisconsin, USA

Rajesh Ramineni MD
University of Texas Medical Branch
Galveston, Texas, USA

JRTC Roelandt MD
Professor of Cardiology
Honorary Chairman, Thoraxcentre
Erasmus University Medical
Centre, Rotterdam
The Netherlands

Lindsay Rogers MD

Department of Pediatrics
Division of Pediatric Cardiology
Vanderbilt University Medical Center
Nashville, Tennessee, USA

Heart Center, St Christophers

Hospital for Children and
Section of Cardiology
Department of Pediatrics
Drexel University, College of Medicine
Philadelphia, Pennsylvania, USA

Ashvin K Patel MD

Asad Ullah Roomi MD

University of Wisconsin School of

Medicine and Public Health
Madison, Wisconsin, USA

Prince Sultan Cardiac Center

Military Hospital Riyadh
Riyadh, Kingdom of Saudi Arabia

David A Parra MD

xii

Comprehensive Textbook of Echocardiography

Jos Alberto San Romn MD PhD FESCC

Teresa Sevilla MD

Robert J Siegel MD

Hospital Clnico, Universitario de

Valladolid, Spain

Hospital Clnico Universitario de

Valladolid, Spain

The Heart Institute, Cedars Sinai

Medical Center, Beverly Boulevard
Los Angeles, California, USA

Emanuele Romeo MD

James Seward MD

Department of Cardiology
Second University of Naples
Monaldi Hospital, Naples, Italy

Mayo Clinic
Rochester, Minnesota, USA

Utpal N Sagar MD
Advanced Cardiovascular Imaging Fellow
Heart and Vascular Institute
Department of Cardiovascular Medicine
Cleveland, Ohio, USA

Hamid Reza Salehi MD

Research Fellow in Echocardiography
Tufts Medical Center
Boston, Massachusetts, USA

Ivan S Salgo MD MSc

Philips Healthcare
Andover, Massachusetts, USA

Giovanni Di Salvo MD
King Faisal Specialist
Hospital and Research Center
Heart Center
Riyadh, Saudi Arabia


Benoy Nalin Shah BSc
(Hons) MBBS MRCP

Department of Cardiovascular Medicine

Northwick Park Hospital
Harrow, UK
Cardiovascular Biomedical
Research Unit
Royal Brompton Hospital
London, UK
National Heart and Lung Institute
Imperial College
London, UK

Chetan Shenoy MBBS

Fellow in Cardiovascular Disease
Tufts Medical Center
Boston, Massachusetts, USA

Mark V Sherrid MD

Director, Echocardiography Lab

Associate Professor of Medicine
New York University
Langone Medical Center
New York, New York, USA

Director, Echocardiography Laboratory

Roosevelt Division
Program Director, Hypertrophic
Cardiomyopathy Program
St. Luke's-Roosevelt Hospital Center
Professor, Clinical Medicine
Columbia University, College of
Physicians and Surgeons
New York, New York, USA

Nelson B Schiller MD

Savitri Shrivastava MD DM FACC FAMS

Muhamed Saric MD PhD

Professor of Medicine
University of California
San Francisco
UCSF Division of Cardiology
Parnassus Avenue
San Francisco, California, USA

Roxy Senior MD DM FRCP FESC FACC

Cardiovascular Biomedical
Research Unit, Royal
Brompton Hospital, London, UK
National Heart and Lung Institute
Imperial College, London, UK
Department of Cardiovascular Medicine
Northwick Park Hospital, Harrow, UK

Satinder P Singh MD FCCP

Professor, Radiology and
MedicineCardiovascular Disease
Chief, Cardiopulmonary Radiology
Chief, 3D Lab, Director, Cardiac CT
Director, Combined Cardiopulmonary
and Abdominal Imaging
Fellowship Program
University of Alabama at Birmingham
Birmingham, Alabama, USA

Siddharth Singh MD MS
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA

Chittur A Sivaram MD
David Ross Boyd Professor
Vice Chief of Cardiovascular Section
Associate Dean for Continuing
Professional Development
University of Oklahoma
Health Sciences Center
Oklahoma City, Oklahama, USA

Sushilkumar K Sonavane MD
Assistant Professor
Cardiopulmonary Radiology
University of Alabama at Birmingham
Department Radiology
Birmingham, Alabama, USA

Vincent L Sorrell MD

Director Pediatric and Congenital Heart

Diseases Fortis Escorts Heart Institute
New Delhi, India

Anthony N DeMaria Professor of

Medicine, Assistant Chief
Division of Cardiovascular Medicine
University of Kentucky
Lexington, Kentucky, USA

Peter Sidarous MD

Jonathan H Soslow MD

Research Associate
UC-Irvine School of Medicine
Irvine, California, USA

Department of Pediatrics
Division of Pediatric Cardiology
Vanderbilt University Medical Center
Nashville, Tennessee, USA

Khadija Siddiqui DO

Anna Agnese Stanziola MD

Division of Cardiology
Department of Medicine
University of Texas Medical Branch
Galveston, Texas, USA

Clinical and Surgery Department

Division of Respiratory Medicine
University Federico IIof Naples
Naples, Italy

Contributors

Sharath Subramanian MD

George Thomas MD

Isidre Vilacosta MD PhD FESCC

Medical College of Wisconsin

Milwaukee, Wisconsin, USA

Department of Cardiology
Saraf Hospital, Kochi, Kerala, India

Lissa Sugeng MD

Wendy Tsang MD

Hospital Clnico
San Carlos
Madrid, Spain

Associate Professor
Director of Yale Echo Lab and
YRCG Echo Corelab
Section of Cardiovascular Medicine
Division of Medicine
Yale University School of Medicine
New Haven, Connecticut, USA

Jie Sun MD PhD

Heart Center, St Christophers Hospital
for Children and Section of Cardiology
Department of Pediatrics
Drexel University College of Medicine
Philadelphia, Pennsylvania, USA

Aylin Sungur MD

University Health Network, Toronto

General Hospital, University of Toronto
Toronto, Ontario, Canada

Jeane M Tsutsui MD

Leon Varjabedian MD

Teena Tulaba RDCS

Karina Wierzbowska-Drabik MD

Allegheny General Hospital

Pittsburgh, Pennsylvania, USA

Padmini Varadarajan MD
Department of Cardiology
Loma Linda University and VA Medical
Centers, Loma Linda, California, USA

Azhar Supariwala MD

Mahdi Veillet-Chowdhury MD

Division of Cardiology
St Lukes-Roosevelt Hospital Center
New York, New York, USA

Stony Brook University Medical Center

Health Sciences Center
Stony Brook, New York, USA

Department of Cardiology
Loma Linda University and VA
Medical Centers
Loma Linda, California, USA

Kiyoshi Tamura PhD

Hitachi Aloka Medical, Ltd.
Imai, Ome-Shi, Tokyo, Japan

Rohit Tandon MBBS MD

Dayanand Medical College and
Hospital Unit, Hero DMC
Heart Institute
Ludhiana, Punjab, India

University of Buffalo
Buffalo, New York, USA

Heart Institute (InCor), The University of

So Paulo School of Medicine and Fleury
Group, So Paulo, Brazil

Division of Cardiovascular Disease

University of Alabama at Birmingham
Birmingham, Alabama, USA

Pooja Swamy MD

Victor Vacanti MD

Colette Veyrat MD
Centre National de la Recherche
Scientifique Honorary Researcher
Department of Cardiovascular Medicine
LInstitut Mutualiste de Montsouris
Boulevard Jourdan, Paris Cedex, France

IB Vijayalakshmi MD DM (Card) FICC

FIAMS FIAE FICP FCSI FAMS DSc

Professor of Pediatric Cardiology

Sri Jayadeva Institute of Cardiovascular
Sciences and Research
Bengaluru, Karnataka, India

University of Buffalo
Buffalo, New York, USA

Chair and Department of

Cardiology
Biegaski Hospital
Medical University of Lodz
Lodz, Poland

Timothy D Woods MD
Associate Professor of
Medicine and Radiology
Medical College of Wisconsin
Cardiology Division
Milwaukee, Wisconsin, USA

Siu-Sun Yao MD FACC

Division of Cardiology
Valley Health System
Ridgewood
New Jersey, USA

Elisa Zaragoza-Macias MD MPH

Cardiovascular
Diseases Fellow
University of Washington
Seattle, Washington, USA

xiii

Preface
Monumental strides have occurred in the evolution of echocardiography since its first introduction in the 1950s.
It began with A-mode and M-mode echocardiography which progressed to real time two-dimensional echocardiography
in the 1970s after a hiatus of several years. This development completely revolutionized the field of noninvasive cardiac
imaging; and within a few years of its introduction, there were hardly any cardiology divisions in any hospital anywhere in
the world which did not own an ultrasound machine. The next few years saw the development of continuous and pulsed
wave Doppler and color Doppler flow imaging which provided assessment of cardiac hemodynamics to supplement
the structural information obtained using two-dimensional echocardiography. Other advances rapidly followed
or occurred concurrently. These included stress echocardiography, transesophageal echocardiography, contrast
echocardiography and tissue Doppler and velocity vector imaging. More recently, further innovations were introduced
such as live/real time three-dimensional echocardiography and both two-and three-dimensional speckle tracking
echocardiography which have obviated some of the limitations of the previous techniques and have further enhanced
the clinical usefulness of echocardiography. To this day, echocardiography represents the most useful and most costeffective noninvasive modality available for the assessment of various cardiac disease entities. The development of
allied noninvasive technologies like magnetic resonance imaging and computed tomography has further added to
the information provided by echocardiography and are useful and important additions to the armamentarium of the
cardiologists and other patient care providers in the comprehensive assessment and management of cardiac disease.
The aim of the current book is to provide an overview of the subject of clinical echocardiography as it is practiced
to-day. Given the many advances that have not only been recently introduced but are also ongoing in this field it would
be very difficult for anyone to realistically come up with a comprehensive book on echocardiography but an attempt has
been made to cover as many topics as possible in this book. In addition, the supplementary information provided by
magnetic resonance imaging and computed tomography is also included in this book.
The book consists of a total of 85 chapters organized into seven sections. The first section deals with the basics of
ultrasound, Doppler, speckle tracking, three-dimensional echocardiography and instrumentation. A short history
of echocardiography and Doppler are also included in this section. The second section consists of various aspects
of echocardiography and ultrasound examination. M-mode and two- and three-dimensional transthoracic and
transesophageal examination, nonstandard planes, various aspects of Doppler assessment including tissue Doppler,
velocity vector and speckle tracking imaging, assessment of endothelial function, contrast echocardiography for
evaluation of left ventricular endocardial border opacification and myocardial perfusion, transpharyngeal echo,
epiaortic echocardiography and both intracardiac and intravascular ultrasound are dealt with in this section. In
addition, examination with a small hand-held ultrasound system, peripheral ultrasound, echocardiographic artifacts,
quantification techniques in echocardiography and echocardiography training form a part of this section. Valvular heart
disease is covered in the next section. It deals with evaluation of mitral valve disease, mitral regurgitation, aortic stenosis
including assessment of low gradient stenosis with preserved left ventricular function, aortic regurgitation, aortic disease,
tricuspid and pulmonary valves, pulmonary hypertension, infective endocarditis and prosthetic valves. Rheumatic heart
disease is also included in this section. Section 4 deals with two- and three-dimensional echocardiographic assessment
of systolic and diastolic function of both left and right ventricles. Newer aspects of structure and function to assess cardiac
motion, evaluation of left atrial function, ventricular assist devices, pacemakers and intracardiac defibrillators and use
of echocardiography for the assessment of cardiac hemodynamics and guidance of therapy are also included in this
section. The next section contains chapters covering ischemic heart disease, coronary arteries and coronary flow reserve,

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Comprehensive Textbook of Echocardiography

different aspects of stress echocardiography including three-dimensional stress echocardiography, obstructive and
non-obstructive cardiomyopathies, differentiation of ischemic and nonischemic cardiomyopathy, pericardial disorders
and tumors and masses. Section 6 deals with congenital heart disease and consists of chapters on fetal cardiac imaging,
M-mode and two- and three-dimensional assessment of pediatric congenital heart disease, ventricular function, adult
congenital heart disease and acquired heart diseases in childhood. The final section in the book, Section 7, covers
systemic diseases, life-threatening conditions, echocardiography in women and the elderly, echocardiography for the
electrophysiologist and lung ultrasound. A separate chapter assesses the future of echocardiography and ultrasound.
Lastly, two chapters cover the allied techniques of magnetic resonance imaging and cardiac computed tomographic
imaging. A very large number of echocardiographic images and other figures illustrate most of the chapters of the book
and six DVDs contain numerous movie clips to supplement the images. These represent a major highlight of the book.
All chapters in this book are written by well-known experts in the field of echocardiography and ultrasound. Because
of the large number of contributors, some overlap of content and chapters do exist in the book. This has been deliberately
not excluded because it provides a different perspective to the reader and also serves to reinforce important concepts
and echocardiographic findings.
Navin C Nanda MD

Acknowledgments
I am most grateful to all the contributors from different countries of the world who have taken valuable time off from
their busy schedule to prepare chapters for this book. I am also grateful to the faculty, clinical and research fellows,
medical residents, and observers, both past and present, from our institution who have directly or indirectly helped
in the preparation of this book. Special mention needs to be made of Kunal Bhagatwala, Nidhi M Karia, Steven Bleich,
Aylin Sungur, Tuba Kemalolu z, Kruti Jayesh Mehta, Maximiliano German Amado Escauela and Ming Hsuing
for their invaluable help. I wish to express my thanks to the International Society of Cardiovascular Ultrasound and
the Indian Academy of Echocardiography for agreeing to have the book under their aegis. Special thanks to all the
members of the Indian Academy of Echocardiography including the current President Dr ST Yavagal as well as Drs
Satish Parashar, HK Chopra and Rakesh Gupta for their unstinting support of this project. I especially appreciate the
constant support and encouragement of Shri Jitendar P Vij (Group Chairman) and Mr Ankit Vij (Managing Director) of
M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India, in helping publish this book and also all their
associates particularly Ms Chetna Malhotra Vohra (Senior ManagerBusiness Development) and Ms Saima Rashid
(Development Editor) who have been prompt, efficient and most helpful. I also deeply appreciate the help of Lindy
Chapman, Administrative Associate at the University of Alabama at Birmingham, who provided excellent editorial and
secretarial assistance, and Diane Blizzard, Office Associate, for her help. Last but not least, I appreciate the patience,
understanding and support of my wife, Kanta Nanda.

Contents

xix

Contents
Volume 1

Section 1: History and Basics

1. History of Echocardiography

Fadi G Hage, Anant Kharod, David Daly, Navin C Nanda

History of Ultrasound 4
The Development of Clinical Cardiac Ultrasound: A-Mode and M-Mode Echocardiography 4
Two-Dimensional Echocardiography 8
Conventional Doppler Ultrasound 9
Color Doppler Ultrasound 11
Contrast Echocardiography 11
Transesophageal Echocardiography 13
Tissue Doppler and Speckle Tracking Imaging 14
Three-Dimensional Echocardiography 14
Perspective 19

2. Early Cardiac Flow Doppler Era: A Key for a New Clinical

Understanding of Cardiology

24

Colette Veyrat
The Preflow Doppler Era: Paucity of Existing Noninvasive Tools 25
Explosive Emergence of the Flow Concept, an Indispensable Mutation from
Pressure Measurements, which Prepared the Doppler Flow Era 27
Return to the Doppler Technique in Search of a Noninvasive Tool
Documenting the Flow Concept 28

3. Basics of Ultrasound

55

Caroline Morbach, Kamran Haleem, Lissa Sugeng

General Physics 55
Imaging by Ultrasound 57
Image Optimization and Equipment 60
Artifacts 61
Doppler Ultrasound 63

4. Doppler EchocardiographyMethodology, Application and Pitfalls

George Thomas

Doppler in Cardiology 65
Doppler Instrumentation 66
Continuous Wave Doppler 68
Pulsed Wave Doppler 69
Color Doppler 71

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Comprehensive Textbook of Echocardiography

Power Doppler 71
Tissue Doppler 72
The Doppler Methodology 72
Information Derived from Doppler 73

5. Basics of 3D Ultrasound

74

Ivan S Salgo, Wendy Tsang, Nicole Bhave, Roberto M Lang

Evolution of 3D Echocardiography 74
Transducer Technology 76
Beam Forming 77
Rendering 78
Limitations in 3D Image Quality 80
3D Echocardiography Quantification 81

6. Speckle Tracking Acquisition: Basics and Practical Tips

87

Willem Gorissen, Navin C Nanda

M-Mode (1D Speckle Tracking) 87

Two-Dimensional Speckle Tracking 88
R-R Interval 91
Standard Views 91
Standardization 91
Two-Dimensional Speckle Tracking Limitation 92
Speckle Tracking Versus Tissue Doppler Imaging 92
Tissue Doppler Imaging Versus Speckle Tracking 92
Three-Dimensional Acquisition 92
Multiview Monitoring During Live Acquisition 97
Multiview Orientation 97
Gain Setting 97
Patient Breath-Hold 98
Arrhythmias 98

7. Instrumentation for Transesophageal Echocardiography Including

New Technology

99

Ryozo Omoto, Kiyoshi Tamura

Kinds of Transesophageal Echo (TEE) 99

What Makes Image Quality 104
Artifacts 107
Safety Considerations 110
Current and Future Technologies 112
In the Future 116

Section 2: Echocardiography/Ultrasound Examination and Training

8. M-Mode Examination
Kamran Haleem, Caroline Morbach, Lissa Sugeng
Historical Perspective 119
Underlying Concept 119

119

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Color M-Mode 120

Advantages and Disadvantages of M-Mode 120
Use of M-Mode 121

9. The Complete Transthoracic Echocardiography

132

Rachel Hughes-Doichev, Anita Radhakrishnan, Abid Ali Fakhri

Teena Tulaba, Rachel Myers

Getting Started 132

Echocardiographic Imaging Windows and Planes 135
Imaging Modalities 135
Parasternal Window 137
Apical Window 146
Subcostal Window 155
Suprasternal Notch Window 159
Three-Dimensional Echocardiography 159
Left Ventricle Chamber Quantification and Regional Wall Motion Determination 161

10. The Standard Transthoracic Examination: A Different Perspective

164

Atif N Qasim, Nelson B Schiller

Set-Up and Patient Positioning 164
Imaged Planes 166

11. Nonstandard Echocardiographic Examination

188

Navin C Nanda, Aylin Sungur, Kunal Bhagatwala, Nidhi M Karia, Tuba Kemalolu z

Right Parasternal Examination Planes 188

Right and Left Supraclavicular Examination 189
Left Parasternal and Apical Planes for Examination of Coronary Arteries 190
Examination of Left Atrial Appendage 212
Examination from the Back 216
Abdominal Examination 220

12. Technique and Applications of Continuous Transthoracic Cardiac Imaging

224

Premindra PAN Chandraratna, Dilbahar S Mohar, Peter Sidarous

Feasibility of Continuous Cardiac Imaging 224
Limitations 237

13. The Basics of Performing Three-Dimensional Echocardiography

Steven Bleich, Navin C Nanda, Satish K Parashar, HK Chopra, Rakesh Gupta

3D Technology 240
3D Examination Protocol 241
Left Parasternal Approach 244
Apical Approach 244
Subcostal Approach 244
Suprasternal Approach 244
Supraclavicular Approach 244
Right Parasternal Approach 246
Color Doppler Imaging 248
Advantages/Disadvantages of 3D Echocardiography 262

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Comprehensive Textbook of Echocardiography

14. How to do Three-Dimensional Transthoracic Echocardiography Examination

268

Fabrice Larrazet, Colette Veyrat

History 268
Methods for Data Acquisition 268
Left Ventricular Assessment 270
Reproducibility 272
Regional LV Function 276
Aortic Regurgitation 280
Aortic Annulus 280
Mitral Stenosis 280
Mitral Regurgitation 282
Tricuspid Valve Disease 283
Pulmonic Valve Disease 284
Advances in Pediatric and Fetal Cardiac Pathologies 285

15. Point-of-Care Diagnosis with Ultrasound Stethoscopy

291

JRTC Roelandt

Battery-Powered Ultrasound Imagers 291

The Traditional Physical Examination 292
The New Physical Examination 293
Acute Care Environment 294
Screening 294
Preparticipation Screening of Athletes 295
Imaging in Remote Areas and Developing Countries 295
Training Requirements 295
Future Directions 296

16. Spectral Doppler of the Hepatic Veins

299

Bahaa M Fadel, Bader Almahdi, Mohammad Al-Admawi, Giovanni Di Salvo

Imaging of the Hepatic Veins 299

Physiological and Other Factors that Affect Hepatic Venous Flow 302
Doppler Pattern of the Hepatic Veins Versus the Superior Vena Cava 304
Transthoracic Echocardiography 304
Transesophageal Echocardiography 305
Technical Considerations 305
Hepatic Venous Flow in Disease States 305
Limitations, Technical Pitfalls and Artifacts 319

17. Spectral Doppler of the Pulmonary Veins

Bahaa M Fadel, Bader Almahdi, Mohammad Al-Admawi, Giovanni Di Salvo

Historical Perspective 325

Imaging of the Pulmonary Veins 325
Physiological Factors that Affect Pulmonary Venous Flow 329
Pulmonary Venous Flow in Disease States 331
Limitations and Technical Pitfalls 342
Artifacts 343

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18. Tissue Doppler Imaging

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349

Hisham Dokainish
Technical Considerations 349
Development of Tissue Doppler Imaging 350
Current Clinical Uses of TD Imaging 350

19. Speckle Tracking Echocardiography: Clinical Usefulness

360

Shyam Padmanabhan, Siddharth Singh, Navin C Nanda

Cardiac Muscular Anatomy, Cardiac Mechanics 360

What is Strain? 362
Two-Dimensional Speckle Tracking Echocardiography (2D STE) 365
Image Acquisition and Processing 367
Clinical Application of 2D STE 367
Three-Dimensional Speckle Tracking Echocardiography (3D STE) 372
Clinical Applications of 3D STE 373
Limitations of Speckle Tracking Echocardiography 374

20. Echocardiographic Assessment of Global and Segmental

Function Using Velocity Vector ImagingTM

380

Michael J Campbell, David A Parra, Daniel Forsha, Piers Barker, Jonathan H Soslow

Application of Velocity Vector Imaging by Age and Disease Group 390

Dyssynchrony, Velocity Vector Imaging Analysis 400
Reproducibility and Correlation Between Vendors 401
Future Directions 404

21. Contrast Echocardiography

416

Jatinder Singh Pabla, Benoy Nalin Shah, Roxy Senior

What is Ultrasound Contrast? 416

How does Ultrasound Contrast Work? 417
Indications for the Use of Ultrasound Contrast 426
Why Should I Use Ultrasound Contrast Agents? 428
Practical Tips 431
Safety of Ultrasound Contrast Agents 434
Saline Contrast Echocardiography 435

22. Myocardial Perfusion Echocardiography

441

Angele A A Mattoso, Jeane M Tsutsui, Wilson Mathias Jr

Acute Coronary Syndromes 443
Assessment of Myocardial Viability 443
Chronic Coronary Artery Disease 443

23. Endothelial Dysfunction

Naveen Garg, Kanwal K Kapur
History 450
Endothelial Functions 450
Endothelial Dysfunctions 451

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Comprehensive Textbook of Echocardiography

Role of Acetylcholine 451

Shear Stress and Flow-Mediated Dilatation 452
Vasoactive Molecules Involved in Vasoregulation 454
NO Release 455
Methodology for Assessing Endothelial Function 455
Analysis of Shear Stress and Flow-Mediated Dilatation Response 457
Limitations 458
Factors Affecting the Flow-Mediated Dilatation 463
Clinical Utility 465
Other Noninvasive Methods to Assess Endothelial Function 465
Assessment of Endothelial Function and Future Directions 471

24. How to do a Two-Dimensional Transesophageal Examination

480

Andrew P Miller, Navin C Nanda

Patient Selection and Consent 480
Preparation, Conscious Sedation and Esophageal Intubation 480
The TEE Examination 481

25. Upper Transesophageal and Transpharyngeal Examination

487

Stephanie El-Hajj, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia, Fadi G Hage
Technique and Recognition of Vessels 487
Application 495

26. How to Perform a Three-Dimensional Transesophageal Echocardiogram

507

Elisa Zaragoza-Macias, Michael Chen, Edward Gill

Three-Dimensional Transesophageal Technology 507

Performing 3D TEE Evaluation 508
Specific Uses of 3D TEE 512
Guidelines and Final Recommendations 514

27. Three-Dimensional Examination to Evaluate Valvular Heart Disease:

The Value of an Added Dimension
Nina Ghosh, Judy R Mangion

Data Acquisition 515

3D Echo Image Optimization 516
3D Echo of the Mitral Valve 516
3D Echo of the Aortic Valve 520
3D Echo of the Pulmonic Valve 522
3D Echo of the Tricuspid Valve 523

Case Examples of 3D Echo in Valvular Heart Disease 525

Case Study 1: Paravalvular Leak Mechanical MV 525

Case Study 2: MV Repair and Aortic Valve Replacement 526
Case Study 3: S/P Cardiac Transplant with Right Heart Failure, Tricuspid Valve Replacement 526
Case Study 4: Flail Middle-Scallop, Posterior Leaflet, MV 526
Case Study 5: Bileaflet MV Prolapse, Moderate to Severe Mitral Insufficiency 527
Case Study 6: Severe Aortic Stenosis, Evaluate for Possible TAVR 527
Case Study 7: Rheumatic Mitral Stenosis 527

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xxv

Case Study 8: S/P Balloon Aortic Valvuloplasty 527

Case Study 9: Mechanism and Severity of Eccentric Mitral Insufficiency 528
Case Study 10: Question of Carcinoid Involvement of the Pulmonic Valve 528

28. Three-Dimensional Echocardiographic Guidance of Percutaneous Procedures 531

Muhamed Saric, Ricardo Benenstein

Fluoroscopy Versus Echocardiography in Guiding Percutaneous Interventions 532

Transseptal Puncture: A Common Element of Many Interventional Procedures 532
Valvular Disease 533
Device Closure of Cardiac Shunts 548
Occlusion of the Left Atrial Appendage 559
Guidance of Electrophysiology Procedures 566
Miscellaneous Procedures 569

29. Three-Dimensional Echocardiography in the Operating Room

577

Ahmad S Omran

Mitral Valve Disease 577

Aortic Valve Disease 582
Tricuspid Valve Disease 589
Native Valve Endocarditis 597
Prosthetic Valve Dysfunction 605
Cardiac Masses 617
Limitations of 3D TEE, Future Directions 628

30. Epiaortic Ultrasonography

638

Dheeraj Arora, Yatin Mehta

Background for Epiaortic Ultrasonography Examination 638

Indications 638
Epiaortic Probe and Preparation 638
Imaging Views/Planes 639
Role of Epiaortic Ultrasonography in Aortic Pathology 640
Advantages of Three-Dimensions over Two-Dimensions in Epiaortic Ultrasonography 641

31. Intracardiac Echocardiography

643

Krishnaswamy Chandrasekaran, Donald Hagler, James Seward

Equipment and the Catheters 643

Imaging Specifications 644
Intracardiac Echocardiography: Clinical Applications 644
Intracardiac Echocardiography during Electrophysiology (EP) Intervention 644
Intracardiac Echocardiography during Structural Intervention 648

32. Intravascular Ultrasound Imaging

Sachin Logani, Charles E Beale, Luis Gruberg, Smadar Kort

Principles of Ultrasound Technology 655

Image Acquisition 655
Intravascular Ultrasound Examination 656
Image Interpretation 657
Utility of Intravascular Ultrasound in Clinical Practice 659
Safety Considerations 661
Future Perspectives 661

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Comprehensive Textbook of Echocardiography

33. Peripheral Vascular Ultrasound

663

Ricardo Benenstein, Muhamed Saric

Ultrasound Diagnosis of Carotid Artery Diseases 663
Ultrasound Diagnosis of Femoral Access Complications 694

34. Advanced Noninvasive Quantification Techniques in Echocardiography

705

Bernhard Mumm, Navin C Nanda

Technological Background of the Different Advanced Quantification Tools 706

Clinical Applications of Advanced Three-Dimensional Echo Quantification Tools 721
Right Ventricular Quantification 723
Mitral Valve Assessment 725
Aortic Valve Assessment 727
Conclusion and Future Outlook 728

35. Artifacts in Echocardiography

732

Shyam Padmanabhan, Navin C Nanda, Aylin Sungur, Tuba Kemalolu z,

Kunal Bhagatwala, Nidhi M Karia, Kruti Jayesh Mehta, Rohit Tandon

Acoustic Shadowing and Acoustic Enhancement 733

Reverberation Artifacts 734
Mirror Image Artifacts 735
Double Image Artifacts 736
Side Lobe Artifact 736
Artifacts Secondary to Use of Electronic Equipment 736
Aliasing 736
Range Ambiguity 736
Artifacts in Three-Dimensional Echocardiography 736
Techniques to Identify and Eliminate Artifacts 737

36. Echocardiography Training

750

Monodeep Biswas, Steven Bleich, Navin C Nanda

Training of Noncardiologists 752

Training for Cardiac Sonographers 753
Training in Computed Tomography and Magnetic Resonance Imaging 755
Certification and Maintenance of Proficiency 758
Appropriate Use Criteria 758

Section 3: Valvular Heart Disease

37. Echocardiography in Acute Rheumatic Fever and Chronic
Rheumatic Heart Disease
IB Vijayalakshmi

Echocardiography in the Diagnosis of Carditis in ARF 765

Chronic Rheumatic Heart Disease 775
Mitral Valve Diseases 775
Mitral Stenosis 776
Mitral Regurgitation 791
Aortic Valve Diseases 802

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Aortic Stenosis 802

Aortic Regurgitation 806
Tricuspid Valve Diseases 812
Tricuspid Stenosis 812
Tricuspid Regurgitation 813

38. Echocardiographic Assessment of Mitral Valve Disease

826

C N Manjunath, Nagaraja Moorthy, Luis Bowen, Navin C Nanda

Overview 826
Echocardiographic Assessment of Mitral Stenosis 826
Echocardiographic Assessment of Mitral Regurgitation 847
Assessment of Severity of Mitral Regurgitation 863

39. Mitral Regurgitation

880

Luc A Pierard, Christine Henri, Julien Magne

Etiology 880
Mechanisms 884
Severity of Mitral Regurgitation 885
Mitral Regurgitation Consequences 889
Sequential Evaluation of Chronic Asymptomatic Mitral Regurgitation 890
Feasibility of Mitral Valve Repair 892
Role of Exercise Echocardiography 892

40. Aortic Stenosis

896

Timothy D Woods, Ashvin K Patel, Sharath Subramanian

Normal Aortic Valve Anatomy 896

Etiology of Aortic Stenosis 897
Echocardiography in Aortic Stenosis 898
Aortic Valve Doppler Examination 904
Use of Stress Echo and Strain in Evaluation of Aortic Stenosis 912
Indications and Appropriateness for Echocardiography in Aortic Valve Stenosis 913

41. Low-Gradient, Severe Aortic Stenosis with Depressed and

Preserved Ejection Fraction

919

Eleonora Gashi, Neil L Coplan, Itzhak Kronzon

Myocardial Response to Chronic Aortic Stenosis 920

High-Flow, High-Gradient Aortic Stenosis in Setting of Normal Ejection Fraction 920
Low-Flow, Low-Gradient Aortic Stenosis in Setting of Low Ejection Fraction 920
Low-Flow, Low-Gradient Aortic Stenosis in Setting of Normal Ejection Fraction 921
Mechanisms Behind PLFLG-AS 924
Role of Surgical Aortic Valve Replacement (SAVR) in Aortic Stenosis 926
SAVR in Low-Flow, Low-Gradient Aortic Stenosis with Low Ejection Fraction 927
SAVR in Paradoxical Low-Flow, Low-Gradient Aortic Stenosis with Normal Ejection Fraction 927

42. Aortic Regurgitation

Arzu Ilercil, Arthur J Labovitz
AR Etiologies 930
Quantification of AR Severity 936
Timing of Aortic Valve Surgery 941

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43. Echocardiographic Evaluation of Aortic Disease

945

Martin G Keane

Echocardiographic Evaluation of the Aorta 945

Aortic Aneurysms 951
Aortic Dissection 954
Common Genetic Syndromes Affecting the Aorta 958
Aortic Atheroma 959
Aortic Trauma and Free Rupture 961
Coarctation of the Aorta 963

44. Transesophageal Echocardiography in the Diagnosis of Aortic Disease

967

Leon J Frazin
The Anatomical Relationship of the Aorta and Esophagus 967
Imaging the Aorta with Transesophageal Echocardiography 967

45. Echocardiographic Examination of the Tricuspid Valve

984

Poonam Malhotra Kapoor, Kunal Bhagatwala, Nidhi M Karia, Navin C Nanda

The Anatomy of Tricuspid Valve (TV) 984

M-Mode Echocardiography 984
Two-Dimensional (2D) Transthoracic Examination 986
Two-Dimensional Transesophageal Examination 988
Three-Dimensional Examination 988
Tricuspid Regurgitation 990
Tricuspid Stenosis 1004
Tricuspid Valve Prolapse: Flail Tricuspid Valve 1007

46. Echocardiographic Assessment of Pulmonary Valve

1031

Hoda Mojazi-Amiri, Padmini Varadarajan, Ramdas G Pai

Epidemiology 1031
Pulmonary Stenosis 1032
Pulmonary Regurgitation 1036
Echocardiographic Evaluation 1037
Ross Procedure 1038
Postpulmonary Valve Surgery: Monitoring Sequelae 1039
Other Complementary Techniques for Evaluation of Pulmonary Valves 1040

47. Echocardiography in Infective Endocarditis

1042

Javier Lpez, Teresa Sevilla, Jos Alberto San Romn, Isidre Vilacosta,
Maximiliano German Amado Escanuela, Kunal Bhagatwala, Nidhi M Karia, Navin C Nanda

Echocardiographic Findings in Infective Endocarditis 1043

Special Considerations in Patients with Infective Endocarditis 1047
Role of Echocardiography in the Prognostic Stratification of Infective Endocarditis 1050
Indications of Echocardiography in Infective Endocarditis 1058

48. The Role of Echocardiography in Pulmonary Hypertension

1063

Michele D' Alto, Francesco Ferrara, Emanuele Romeo, Anna Agnese Stanziola, Eduardo Bossone
Conventional Echocardiography 1063
Nonconventional Echocardiography 1070
Diagnostic Algorithm in Pulmonary Hypertension 1073

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49. Echocardiographic Assessment of Prosthetic Valves

xxix

1080

Aditya Bharadwaj, Pooja Swamy, Gary P Foster, Padmini Varadarajan, Ramdas G Pai

Types of Prosthetic Valves 1080

Assessment of Prosthetic Valves 1082
Prosthetic Valve Dysfunction 1087
Other Complementary Imaging Modalities 1092

50. Three-Dimensional Transthoracic and Transesophageal Echocardiographic

Evaluation of Prosthetic Valves

1094

Steven Bleich, Navin C Nanda

Three-Dimensional Visualization of Prosthetic Valves 1094
Three-Dimensional Transthoracic Echocardiographic Assessment of Prosthetic Valves 1095
Three-Dimensional Transesophageal Echocardiographic Assessment of Prosthetic Valves 1100

Volume 2

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

51. M-Mode and Two-Dimensional Echocardiographic Assessment of
Left Ventricular Systolic Function

1115

Anjlee M Mehta, Navin C Nanda

Visual Estimation of Left Ventricular Systolic Function 1115
M-Mode and Two-Dimensional Transthoracic Echocardiographic Methods for
Assessment of Left Ventricular Systolic Function 1116
Doppler Echocardiographic Methods of Assessment of Left Ventricular Function 1119
Two-Dimensional Speckle Tracking Echocardiography and Velocity Vector Imaging 1120
Myocardial Performance Index 1120
Contrast Echocardiography in the Assessment of Left Ventricular Systolic Function 1121
ArterialVentricular Coupling 1121
Three-Dimensional Transthoracic Echocardiography 1122

52. How to Assess Diastolic Function

1124

Hisham Dokainish
Integrating Echocardiographic Variables for Accurate Diagnosis of Diastolic Function 1130
Novel Imaging Techniques and Future Directions 1131

53. Evaluation of the Right Ventricle

Vincent L Sorrell, Steve W Leung, Brandon Fornwalt

General Overview 1134

Right Ventricle Morphology 1135
Echocardiography 1136
Speckle Tracking 1141
Hemodynamics 1143
Other Imaging Modalities 1144

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54. Three-Dimensional Echocardiographic Assessment of LV and RV Function

1149

Aasha S Gopal
3D Quantitation of the Left Ventricle 1149
3D Quantitation of the Right Ventricle 1165

55. Newer Aspects of Structure/Function to Assess Cardiac Motion

1176

Gerald Buckberg, Navin C Nanda, Julien IE Hoffman, Cecil Coghlan

Basic Heart Function 1177

State-of-the-Art 1180
Composite of State-of-the-Art Reports 1181
Novel Mechanical and Timing Interdependence between Torsion and Untwisting 1184
The Normal Heart 1185
The Septum 1194
The Right Ventricle 1198
Other Considerations 1198

56. Echocardiography in Assessment of Complications Related to Permanent

Pacemakers and Intracardiac Defibrillators

1210

Ahmed Almomani, Khadija Siddiqui, Masood Ahmad

Normal Echocardiographic Findings in Permanent Pacemakers/Implantable
Cardioverter-Defibrillators 1210
Pacemaker and Implantable Cardioverter-Defibrillator-Related Complications 1212
Tricuspid Regurgitation 1212
Masses: Lead Infection and Thrombus 1214
Myocardial Perforation 1215
Deleterious Effects of Right Ventricular Apical Pacing on Left Ventricular Function 1217

57. Echocardiographic Evaluation of Ventricular Assist Devices

1222

Peter S Rahko

Clinical Uses of Ventricular Assist Devices 1224

Reverse Remodeling 1226
Types of Devices 1226
Preoperative Echocardiographic Evaluation 1229
Immediate Postsurgical Evaluation 1234
Serial Changes in Cardiac Structure and Function 1234
Complications of Left Ventricular Assist Devices 1240
Evidence of Underfilling of the Left Ventricle 1246
Optimizing Left Ventricular Assist Device Settings 1248
Explantation 1249
Percutaneous Continuous Flow Devices 1250

58. Echocardiographic Assessment of Left Atrial Function

Utpal N Sagar, Hirohiko Motoki, Allan L Klein

Anatomy 1255
Physiology 1256
Functional Assessment 1257
Left Atrial Pathophysiology 1259

1255

Contents

59. The Use of Echocardiography to Assess Cardiac

Hemodynamics and Guide Therapy

xxxi

1264

Roy Beigel, Robert J Siegel

Right Atrial Pressure/Central Venous Pressure 1264

Pulmonary Artery Hemodynamics 1269
Left-Sided Filling Pressures 1273
Additional Parameters for Estimation of Left Atrial Pressure 1279
Stroke Volume, Stroke Distance, Cardiac Output, and Systemic Pulmonary Shunts (QP/QS) 1280

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders,

Tumors and Masses

60. Echocardiography in Ischemic Heart Disease

1289

Chetan Shenoy, Hamid Reza Salehi, Francesco F Faletra, Natesa G Pandian

Detection of Ischemia 1289

Role in Acute Coronary Syndromes 1292
Mechanical Complications of Myocardial Infarction 1294
Role of Echocardiography in Chronic Ischemic Cardiomyopathy 1298
Novel Echocardiography Techniques in Ischemic Heart Disease 1301

61. Stress Echocardiography

1306

Azhar Supariwala, Siu-Sun Yao, Farooq A Chaudhry

Fundamentals of Stress Echocardiography 1306

Types of Stress Echocardiography 1307
Interpretation of Stress Echocardiography 1309
Stress Echocardiography: Future Directions 1319

62. Squatting Stress Echocardiography

1323

Premindra PAN Chandraratna, Dilbahar S Mohar, Peter Sidarous

Squatting Echocardiography 1324

63. Three-Dimensional Stress Echocardiography

Rajesh Ramineni, Masood Ahmad

Two-Dimensional Stress Echocardiography 1328

Three-Dimensional Transducers 1329
Advantages of Three-Dimensions in Stress Imaging 1329
Three-Dimensional Image Acquisition 1330
Three-Dimensional Stress Protocol 1331
Postacquisition Analysis 1331
Review of Studies Comparing Three-Dimensional Stress
Echocardiography to Current Standards 1331
Differences between 2DSE and 3DSE in Wall Visualization 1334
Parametric Imaging in Three-Dimensional Stress Echocardiography 1334
Role of Contraction Front Mapping in RT3DSE 1334
Contrast in Three-Dimensional Stress Testing 1335

1328

xxxii

Comprehensive Textbook of Echocardiography

64. Echocardiographic Assessment of Coronary ArteriesMorphology and

Coronary Flow Reserve

1337

Karina Wierzbowska-Drabik, Jarosaw D Kasprzak

The Assessment of Coronary Morphology and Flow in
Transthoracic and Transesophageal Studies 1337
Visualization of Coronary Arteries 1337
Distal Coronary Flow and Coronary Flow Reserve 1340
Congenital Abnormalities of the Coronary Arteries 1343

65. Echocardiography in Hypertrophic Cardiomyopathy

1348

Dan G Halpern, Mark V Sherrid

Definitions and Types of Hypertrophy 1349

Mid-Left Ventricular Hypertrophic Cardiomyopathy 1356
Differential Diagnosis 1359
Treatment Strategies in Hypertrophic Cardiomyopathy 1361

66. Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1369

Rohit Gokhale, Manreet Basra, Victor Vacanti, Steven J Horn, Aylin Sungur,
Robert P Gatewood Jr, Navin C Nanda

Cardiomyopathies 1369
Dilated Cardiomyopathy (DCM) 1370
Secondary Findings in Dilated Cardiomyopathy 1372
The Role of Echocardiography in Optimizing Heart Failure 1376
Echocardiography in Assessing Ventricular Remodeling 1379
Findings in Dilated Cardiomyopathy Based on Etiology 1379
Restrictive Cardiomyopathy 1397
Other Infiltrative Cardiomyopathies 1405
Infectious and Metabolic Cardiomyopathies 1405
Carcinoid Heart Disease 1407

67. Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy:

Comparison with Other Noninvasive Modalities
1418
Sula Mazimba, Arshad Kamel, Navin C Nanda, Maximiliano German Amado Escanuela,
Kunal Bhagatwala, Nidhi M Karia

Echocardiographic Assessment of Ischemic and Nonischemic Cardiomyopathy 1419

M-Mode Echocardiography 1419
Two-Dimensional/Three-Dimensional/Doppler Echocardiography 1421
Echocardiographic Distinction between Ischemic Cardiomyopathy and
Nonischemic Dilated Cardiomyopathy 1425
Other Noninvasive Imaging Modalities 1425

68. Pericardial Disease

Trevor Jenkins, Brian D Hoit
Acute Pericarditis 1436
Pericardial Effusion 1436

1435

Contents

xxxiii

M-Mode and Two-Dimensional Echocardiography 1437

Pericardial Tamponade 1438
Constrictive Pericarditis 1444
Effusive-Constrictive Pericarditis 1448
Congenital Anomalies 1448
Multimodality Imaging of the Pericardium 1450

69. Three-Dimensional Echocardiographic Assessment in Pericardial Disorders 1452

O Julian Booker, Navin C Nanda
Two-Dimensional Transthoracic Echocardiography Versus Three-Dimensional
Transthoracic Echocardiography in Pericardial Effusion 1453
Two-Dimensional Transthoracic Echocardiography Versus Three-Dimensional
Transthoracic Echocardiography in Constriction 1456
Two-Dimensional Transthoracic Echocardiography Versus Three-Dimensional
Transthoracic Echocardiography in Pericardial Masses 1458

70. Echocardiographic Assessment of Cardiac Tumors and Masses

1462

Leon Varjabedian, Jennifer K Lang, Abdallah Kamouh, Steven J Horn, Tuba Kemalolu z
Aylin Sungur, Kruti Jayesh Mehta, Kunal Bhagatwala, Nidhi M Karia
Maximiliano German Amado Escauela, Robert P Gatewood Jr, Navin C Nanda

Echocardiographic Assessment of Cardiac Tumors and Masses 1462

Primary Benign Cardiac Tumors 1464
Malignant Primary Cardiac Tumors 1484
MICE 1511

Section 6: Congenital Heart Disease

71. Fetal Cardiac Imaging

1527

Aarti H Bhat

Scope of Fetal Cardiology 1527

Indications for Fetal Cardiac Evaluation 1528
Fetal Physiology 1528
Indications for Fetal Echocardiography 1529
Extracardiac Reasons and Associations for Fetal Heart Disease 1529
Fundamentals of Fetal Cardiac Imaging 1530
Case Studies 1556

72. M-mode and Two-Dimensional Echocardiography in

Congenital Heart Disease
Neeraj Awasthy, Savitri Shrivastava
Part 1: Basics of Imaging and Sequential Segmental Analysis 1562

Patient Preparation 1562

Imaging 1563
Dextrocardia 1570
Principles of Sequential Chamber Analysis 1575

1561

xxxiv

Comprehensive Textbook of Echocardiography

Part 2: Left-to-Right Shunts: Atrial Septal Defect, Ventricular Septal Defect, Patent Ductus Arteriosus, and
Aortopulmonary Window 1582

General Features: Shunt Lesions 1582

Atrial Septal Defects 1585
Ventricular Septal Defect 1591
Patent Ductus Arteriosus 1599
Aortopulmonary Window 1602
Gerbode Defect 1603

Part 3: Atrioventricular Septal Defects 1604

Part 4: Congenital Left Ventricular and Right Ventricular Inflow Anomalies 1610

Congenital Anomalies of Mitral Valve 1610

Congenital Abnormalities of Tricuspid Valve 1616
Part 5: Left Ventricular Outflow Tract Obstruction 1618

Valvular Aortic Stenosis 1618

Subvalvular Aortic Stenosis 1624
Supravalvular Aortic Stenosis 1626
Aortic Regurgitation 1628
Sinus of Valsalva Aneurysm 1630
Aortocameral Communications 1632

Part 6: Echocardiographic Anatomy of Tetralogy of Fallot with Pulmonary Stenosis 1633

Aortic Override 1633

Double Outlet Right Ventricle 1644
Truncus Arteriosus 1650
Part 7: Complete Transposition of Great Arteries 1653

Transposition of Great Vessels (TGA) 1653

Part 8: Atrioventricular and Ventriculoarterial Discordance 1664
Part 9: Pulmonary Veins 1670

Normal Flow Pattern of Pulmonary Veins 1670

Anomalies of Pulmonary Veins 1672
Total Anomalous Pulmonary Venous Connection 1673
Anomalies of Systemic Veins 1678

Part 10: Imaging of Coronary Anomalies and Pulmonary Arteries 1684

Coronary Artery Anomalies 1684

Coronary Arteriovenous Fistula 1688
Coronary Aneurysms 1688
Part 11: Echocardiographic Evaluation of Aortic Arch and Its Anomalies 1690

Abnormal Formation of Arch 1690

Coarctation of Aorta (CoA) 1692
Interruption of Aortic Arch 1694
Aortic Aneurysm 1695

Part 12: Univentricular Heart and Heterotomy Syndrome 1696

Univentricular Atrioventricular Connections 1697

Tricuspid Atresia 1700

Contents

xxxv

Mitral Atresia and Hypoplastic Left Heart Syndrome 1701

Heterotaxy Syndrome 1704

73. Real Time 3D Echocardiography for Quantification of Ventricular Volumes,

Mass and Function in Children with Congenital and Acquired Heart Diseases 1721
Shuping Ge, Jie Sun, Lindsay Rogers, Rula Balluz

Left Ventricular Volumes, Ejection Fraction, and Mass 1722

Right Ventricular Volumes, Ejection Fraction, and Mass 1723
Single Ventricular Volumes, Ejection Fraction, and Mass 1725
Three-Dimensional Analysis of Regional Wall Motion, Synchrony, and Strain 1726

74. Three-Dimensional Echocardiography in Congenital Heart Disease

1733

Steven Bleich, Gerald R Marx, Navin C Nanda, Fadi G Hage

Shunt Lesions/Septal Defects 1733

Common Atrium 1747
Aortopulmonary Window 1751
Patent Ductus Arteriosus (PDA) 1751
Conotruncal Anomalies 1754
Outflow Tract Obstruction 1766
Aortic Arch Anomalies 1770
Atrial and Atrioventricular Valve Abnormalities 1773
Other Abnormalities 1776
Double Outlet Right Ventricle 1779
Sinus of Valsalva Aneurysm 1784

75. Echocardiography in the Evaluation of Adults with

Congenital Heart Disease

1791

Reema Chugh

Key Concepts of Echocardiography in Adults with Congenital Heart Disease 1793

Simple Congenital Heart Defects in Adults 1798
Valvular Disease 1813
Complex Congenital Heart Defects 1826

76. Echocardiographic Evaluation for Acquired

Heart Diseases in Childhood
Jie Sun, Rula Balluz, Lindsay Rogers, Shuping Ge

Infective Endocarditis 1856

Modified Duke Criteria for the Diagnosis of Infective Endocarditis 1857
Echocardiographic Findings 1857
Complications of Infective Endocarditis 1859
Rheumatic Heart Disease 1859
Jones Criteria, Updated 1992 1859
Kawasaki Disease 1861
Coronary Ectasia and Aneurysms by Echocardiography 1861

1856

xxxvi

Comprehensive Textbook of Echocardiography

Section 7: Miscellaneous and Other Noninvasive Techniques

77. Echocardiography in Systemic Diseases

1867

Mahdi Veillet-Chowdhury, Smadar Kort

Systemic Lupus Erythematosus 1867

Rheumatoid Arthritis 1868
Hypereosinophilic Syndrome 1868
Systemic Sclerosis 1869
Renal Disease 1871
Amyloidosis 1872
Carcinoid 1874
Chagas Disease 1875
Sarcoidosis 1876
Thyroid Disorders 1879
Nutritional Deficiency 1880

78. Echocardiography in Women

1886

Jennifer Kiessling, Navin C Nanda, Tuba Kemalolu z, Aylin Sungur,

Kunal Bhagatwala, Nidhi M Karia

Differences in Echocardiographic Measurements and Technical Considerations 1886

Structural Heart Disease: MVP, Mitral Stenosis, and Mitral Annular Calcification 1888
Ischemic Heart Disease/Stress Echocardiography/Polycystic Ovarian Syndrome 1889
Takotsubo Cardiomyopathy 1899
Congenital Heart Disease 1900
Echocardiography in Pregnancy, Peripartum Cardiomyopathy, Fetal Echocardiography 1902

79. Echocardiography in the Elderly

1921

Gopal Ghimire, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia

Aortic Atherosclerosis and Penetrating Aortic Ulcer 1921

Aortic Valve Sclerosis 1923
Aortic Stenosis 1924
Aortic Aneurysm 1934
Aortic Dissection 1937
Left Ventricular Mass, Dimensions, and Function 1942
Echocardiography in Stroke Patients: Assessment of Coronary Stenosis 1943
Mitral Annular Calcification 1946
Prosthetic Valves 1948

80. How to do Echo for the Electrophysiologist

Chittur A Sivaram

Echocardiography in Supraventricular Tachycardia 1957

Left Atrium 1960
Atrial Septum 1962
Pulmonary Veins 1963
Inferior Vena Cava 1964
Echocardiography in Ventricular Tachycardia 1966
Echocardiography in Cardiac Implantable Electronic Devices 1967

1957

Contents

81. Echocardiography in Life-Threatening Conditions

xxxvii

1969

Rachel Harris, Elizabeth Ofili

Chest Trauma 1969

Blunt Chest Trauma 1969
Penetrating Chest Trauma 1972
Acute Mitral Regurgitation 1972
Acute Severe Aortic Regurgitation 1972
Aortic Dissection 1974
Debakey Classification 1974
The Stanford Classification 1974
Pulmonary Thromboembolic Disease 1976
Air Embolism 1977
Hypovolemia 1977
Large Intracardiac Thrombus 1978

82. Lung Ultrasound in Cardiology

1982

Luna Gargani, Eugenio Picano

Physical and Physiological Basis of Lung Ultrasound 1982

Methodology 1983
Pulmonary Interstitial Edema 1984
Pleural Effusion 1985
Pulmonary Embolism 1985
Acute Respiratory Distress Syndrome 1986
Pneumothorax 1986
Cardiopulmonary Ultrasound: An Integrated Approach 1987
Limitations 1987

83. The Future of Echocardiography and Ultrasound

1990

David Cosgrove

Plane Wave Ultrafast Imaging 1990

Trends in Scanners 1991
Doppler 1993
Microbubbles 1993
Elastography 1994
Light and Sound 1995
Therapeutic Applications of Ultrasound 1996

84. A Primer on Cardiac MRI for the Echocardiographer

Madhavi Kadiyala, Aasha S Gopal

Quantitative Left and Right Ventricular Assessment 1998

Strain Assessment 1999
Left Ventricular Structure 2000
Myocarditis and Sarcoidosis 2004
Cardiac Hypertrophy 2006
Cardiomyopathies 2008
Velocity Mapping, Flow and Shunt Assessment 2008
Valvular Heart Disease and Prosthetic Valves 2009

1998

xxxviii

Comprehensive Textbook of Echocardiography

Pericardial Disease 2014

Normal Variants and Masses 2016
Limitations of Cardiac MRI and CT 2017
Glossary of Cardiac MRI Sequences 2020

85. Cardiac CT Imaging

2023

Satinder P Singh, Sushilkumar K Sonavane

Challenges for Cardiac Computed Tomography 2024

Radiation Dose 2025
Patient Selection 2027
Technique 2027
Image Postprocessing 2028
Image Analysis 2032
Pitfalls and Artifacts 2034
Diagnostic Accuracy of Coronary Computed Tomography Angiogram 2040
Coronary Plaque 2041
Prognostic Information from Coronary Computed Tomography Angiogram 2042
Cardiac Function 2042
Myocardial Perfusion 2042
How to Improve Accuracy of Computed Tomography Angiogram in Determining
Flow Limiting Disease 2044
Clinical Indications 2044
Index

I-i

SECTION 4
Left and Right Ventricles,
Left Atrium, Hemodynamics

Chapters
Chapter 51 M-Mode and Two-Dimensional Echocardiographic
Assessment of Left Ventricular Systolic Function
Chapter 52 How to Assess Diastolic Function
Chapter 53 Evaluation of the Right Ventricle
Chapter 54 Three-Dimensional Echocardiographic
Assessment of LV and RV Function
Chapter 55 Newer Aspects of Structure/Function to Assess
Cardiac Motion
Chapter 56 Echocardiography in Assessment of Complications
Related to Permanent Pacemakers and Intracardiac
Defibrillators

Chapter 57 Echocardiographic Evaluation of Ventricular

Assist Devices
Chapter 58 Echocardiographic Assessment of Left
Atrial Function
Chapter 59 The Use of Echocardiography to Assess
Cardiac Hemodynamics and Guide Therapy

CHAPTER 51
M-Mode and Two-Dimensional
Echocardiographic Assessment of Left
Ventricular Systolic Function
Anjlee M Mehta, Navin C Nanda

Snapshot

Visual Estimation of Left Ventricular Systolic Function

M-Mode and Two-Dimensional Transthoracic Echocar-

diographic Methods for Assessment of Left Ventricular

Systolic Function

Doppler Echocardiographic Methods of Assessment of

Left Ventricular Function

Two-Dimensional Speckle Tracking Echocardiography

and Velocity Vector Imaging

INTRODUCTION
The evaluation of left ventricular systolic function by
echocardiography has undergone many recent advance
ments. Assessment of ejection fraction as a surrogate for
left ventricular systolic function is one of the primary
clinical questions for which echocardiograms are obtained.
A review of methodologies for determining ejection
fraction and/or left ventricular function by M-mode and
two-dimensional (2D) echocardiography allows for a
better understanding of advantages, disadvantages, and
appropriate indications for echocardiographic evaluation
of left ventricle (LV) systolic function.

Myocardial Performance Index

Contrast Echocardiography in the Assessment of Left

Ventricular Systolic Function

ArterialVentricular Coupling

Three-Dimensional Transthoracic Echocardiography

VISUAL ESTIMATION OF LEFT

VENTRICULAR SYSTOLIC FUNCTION
Visual estimation of LV ejection fraction, in the eyes of
an experienced echocardiographer, is a quick method
for determination of systolic function and is widely used
to help make immediate decisions in clinical settings. A
framework for evaluation involving division of the LV into
16 segments was proposed by the American Society of
Echocardiography in 1989. In this model, the LV is divided
into a basal level, mid (or papillary) level, and apical level.
There are six segments at both basal and midventricular
levels, and four segments at the apex. In 2002, the

1116

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 51.1: Seventeen-segment model with correspondence to coronary artery distribution. (LAD: Left anterior descending artery;
LCX: left circumflex artery; RCA: Right coronary artery).
Source: Reproduced with permission from Pereztol-Valdes O, Candell-Riera J, et al. Correspondence between left ventricular
17 myocardial segments and coronary arteries. Eur Heart J. 2005(26):263743.

American Heart Association Writing Group on Myocardial

Segmentation and Registration for Cardiac Imaging
added a 17th segment encompassing the apical cap, the
segment beyond the end of the LV cavity1 (Fig. 51.1). This
model allows for segmental determination of regional wall
motion abnormalities and utilizes a scoring system based
on the motion and systolic thickening of each segment.
The walls are evaluated in multiple echocardiographic
views including a parasternal long-axis ([PLAX], or apical
3-chamber [A3Ch]/apical long-axis [ALA]), parasternal
short-axis (PSAX), apical 4-chamber (A4Ch), and apical
2-chamber (A2Ch) view for correlation. Each segment
can be scored from 1 to 5 with the following definitions:
1 = normal or hyperkinesis, 2 = hypokinesis, 3 = akinesis
(negligible thickening), 4 = dyskinesis (paradoxical systolic
motion), and 5 = aneurysmal (diastolic deformation). The
score for each segment is added up to give a total score.
This score is then divided by the number of segments to
create a wall motion index score. A normal ventricle has a
wall motion score of 1.1,2
The blood supply from a particular coronary artery to
each segment can also be defined. In general, segments
1, 2, 7, 8, 13, 14, and 17 are generally supplied by the left
anterior descending artery, segments 3, 4, 9, 10, and 15

by the right coronary artery (if dominant), and segments

5, 6, 11, 12, and 16 by the left circumflex artery. As a
result, if regional wall motion abnormalities are involved,
describing the segments involved can allow one to
surmise which epicardial coronary vessel may be involved
(Fig. 51.2).1,2

M-MODE AND TWO-DIMENSIONAL

TRANSTHORACIC ECHOCARDIOGRAPHIC METHODS FOR ASSESSMENT OF LEFT VENTRICULAR
SYSTOLIC FUNCTION
The Teichholz formula using M-mode echocardiography
was one of the earliest methods developed for assessment
of left ventricular ejection fraction (LVEF). In a PLAX
view, inferior and lateral to the mitral valve chordae,
measurements of the left ventricular end-diastolic internal
diameter and the left ventricular end-systolic internal
diameter are made. These measurements are then used to
calculate end-diastolic and end-systolic volumes, and the
difference between these two volumes divided by the enddiastolic volume can be used to calculate the LVEF.3

Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function

1117

Fig. 51.2: Typical distributions of the right coronary artery (RCA), the left anterior descending (LAD), and the circumflex (CX) coronary
arteries. The arterial distribution varies between patients. Some segments have variable coronary perfusion. J Am Soc Echocardiogr.
2005;18(12):144063.

M-mode is also used to obtain the E point septal

separation (EPSS), an indirect estimation of global
LV function. In the setting of LV dysfunction, there is
increased separation between the E point (peak of mitral
valve opening) and the ventricular septum. With LV
chamber enlargement and dysfunction, the mitral valve is
shifted further away from the septum and there is reduced
transmitral flow (and reduced stroke volume) relative
to chamber size. Typically, an EPSS > 1 cm is considered
abnormal.4
Using the Quinones method, measurements are
taken at several minor-axis locations of the LV in three 2D
echocardiographic views (PLAX, A4Ch, and ALA views).
The minor-axis measurement locations in the PLAX view
are at the base and midcavity levels. In the A4Ch and
ALA views, measurements are taken at the upper third,
middle third, and lower thirds of the LV in end-systole and
end-diastole. The contribution of the apex to the LVEF is
made by a qualitative assessment of apical wall motion
abnormalities.5

The Baran, Rogal, and Nanda method for quantification

of LVEF, in the absence of wall motion abnormalities,
requires end-systolic and end-diastolic measurements of
the LV minor axis at the midventricular level and the LV
major axis from the apex to the base of the LV. These values
are obtained in an A4Ch view (Fig. 51.3). End-diastolic and
end-systolic volumes are then calculated using a modified
cylinderellipse formula in which the LV is assumed to be a
combination of a cylinder and prolate ellipse (Fig. 51.4). If
wall motion abnormalities are present, then measurement
of the minor axes at three equidistant points that divide
the LV into three regions are obtained. Each region
contributes one-third to the total ejection fraction and the
chance of including wall motion abnormalities in one of
the regions is increased. The total LVEF is an average of the
LVEFs obtained from each of the three regions.6
Another method for assessing left ventricular systolic
function is to calculate a fractional area change. Measure
ments of minor axis dimensions are taken from M-mode
echocardiograms obtained with 2D echocardi
ographic

1118

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 51.3: Apical four-chamber view of a normal heart at endsystole and end-diastole. In method A, left ventricular (LV) minor
axis D is measured at end-systole and end-diastole at the midventricular cavity level. The left ventricular major axis is measured
from the apex of the left ventricle to the base of the mitral valve.
In method B, measurements of the regional left ventricular minor
axes, D1, D2, and D3 are measured at three equidistance points
at the upper, middle, and lower third of the left ventricular cavity at
end-systole and end-diastole of the same cardiac cycle. The major
axis L is measured as before. Directions I, L, R, and S are inferior,
left, right, and superior, respectively. (LA: Left atrium; RA: Right
atrium; RV: Right ventricular).
Source: Reproduced with permission from Baran AO, et al.
Ejection fraction determination without planimetry by two-dimensional echocardiography: a new method. J Am Coll Cardiol.
1983;1:14718.

guidance. The left ventricular internal dimension in

diastole (LVIDd) and left ventricular internal dimension
in systole (LVIDs) are used in the formula for fractional
shortening, measured at the endocardium (FSendo [%])
such that, FSendo = 100 (LVIDd LVIDs)/(LVIDd).7
Unfortunately, fractional shortening at the endoc
ardium is affected by changes in left ventricular geometry
and loading conditions. Another parameter called midwall
fractional shortening (MWFS) is less influenced by left
ventricular geometry and has been shown to be useful
in detection of early systolic dysfunction in hypertensive
patients with concentric left ventricular hypertrophy.7
MWFS, unlike FSendo, does not assume uniformity of
systolic thickening throughout the myocardium, and is
therefore less likely to overestimate contractile function.
In reality, inner wall (subendocardial) and outer wall
(epicardial) thickening fractions are not equal, and the
inner wall contributes more to systolic thickening than the
outer wall.8 This is more pronounced in conditions with
increased relative wall thickness/altered left ventricular

Fig. 51.4: Modified cylinderellipse formula. A, cross-sectional

area of cylinder (hatched); (D: Diameter of circle A; L: Length of
entire object; LVV: Left ventricular volume).
Source: Reproduced with permission from Baran AO, et al. Ejection fraction determination without planimetry by two-dimen
sional echocardiography: a new method. J Am Coll Cardiol.
1983;1:14718.

wall geometry, such as hypertrophied hearts. In addition

to the M-mode measurements used in FSendo, the MWFS
also uses measurements of septal wall thickness at diastole
(SWTd) and posterior wall thickness (PWTd) at diastole and
incorporates a separate equation for the inner shell (inner
wall) with the following formulas.7,8
Inner shell = ([LVIDd + SWTd/2
+ PWTd/2]3 LVIDd3 + LVIDs3)1/3LVIDs
MWFS= ([LVIDd SWTd/2 PWTd/2]
[LVIDs inner shell])
(LVIDd + SWTd/2 + PWTd/2) 100
The biplane method of discs, or modified Simpsons
rule, recommended by the American Society of
Echocardiography is one of the most commonly applied 2D
techniques for obtaining the left ventricular volumes used
in calculating an LVEF. The left ventricular endocardial
border is traced during end-diastole and end-systole in
orthogonal planes that include the apex (e.g. A4Ch and
A2Ch views; Fig. 51.5). The ventricle is then divided, along
the long axis, into a series of ellipsoid discs of equal height
(Fig. 51.6). Computer software then determines the volume
of each disc (height disc area). All the volumes are added
to obtain the total LV volumes in systole and diastole and
allow for calculation of the LVEF (EDV ESV/EDV).
Limitations of the biplane method of discs include
endocardial dropout and apical foreshortening that result
in incorrectly small ventricular volumes. It also assumes

Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function

1119

Fig. 51.6: Use of Simpsons rule. Using this rule, the volume of left
ventricle is usually calculated by approximating areas along the
apical axis by circles and employing axial integration.
Source: Reproduced with permission from Ghosh A, Nanda
NC, Maurer G. Three-dimensional reconstruction of echocardiographic images using the rotation method. Ultrasound Med Biol.
1982;8(6);65561.

DOPPLER ECHOCARDIOGRAPHIC
METHODS OF ASSESSMENT OF LEFT
VENTRICULAR FUNCTION
Fig. 51.5: Two-dimensional measurements for volume calculations using biplane method of disks (modified Simpsons rule) in
apical four-chamber (A4C) and apical two-chamber (A2C) views
at left ventricular end-diastole (LV EDD) and at left ventricular endsystole (LV ESD). Papillary muscles should be excluded from the
cavity in the tracing.
Source: Reproduced with permission from Lang RM, Bierig M,
Devereux RB, et al. Recommendations for chamber quantification: a report from the American Society of Echocardiographys
Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European
Association of Echocardiography, a branch of the European Society of Cardiology. J Am SocEchocardiogr. 2005;18(12):144063.

that the ventricle is ellipsoidal, which is not always the

case (e.g. LV aneurysm). Poor acoustic windows, as is
commonly seen in obese, ventilated, or severe chronic
obstructive pulmonary disease (COPD) patients, can also
make accurate endocardial tracing very difficult.
Calculation of a sphericity index is another method that
has been used as a marker of left ventricular dysfunction.
The sphericity index is the ratio of the LV long-axis
dimension and LV short-axis dimension. With negative LV
remodeling that occurs in dilated cardiomyopathies, heart
failure, myocardial infarcts, and valvular regurgitation, the
LV adapts by becoming more spherical. A normal elliptical
LV has a sphericity index of 1.5.9 As the heart becomes
more spherical, this value decreases and approaches one
signifying a more spherical LV.

Tissue Doppler Imaging

Tissue Doppler imaging (TDI) is based on the principle
that myocardial velocities, like blood flow velocities, can
be differentiated based on their different amplitudes and
Doppler frequencies. The peak systolic ejection velocity
represented by the S-wave on velocity tracings is obtained
by placing a sample volume 1 cm above (apical) the medial
(septal) side of the mitral annulus and obtaining the tissue
velocity as the LV moves toward the apex in systole.7
When the LV is in the early filling phase of diastole and
moving away from the apex, this velocity is represented
by the E-wave on velocity tracings. The A-wave velocity is
obtained during late diastole as the atria contract and the
LV moves away from the apex.7 These waves represent the
longitudinal motion of the LV (base to apex shortening).
The contribution of longitudinal fiber shortening
and myocardial contractile velocity to left ventricular
function forms the basis for analysis of the mitral annular
descent velocity by tissue Doppler echocardiography.
2D echocardiography guides placement of the M-mode
cursor at the mitral annulus at two sites in the A4Ch,
A2Ch, and ALA views.10 The maximal color-coded velocity
toward the transducer during left ventricular ejection is
the peak mitral annular descent velocity. This velocity is
independent of endocardial definition, but only looks at
longitudinal movement of the LV walls during systole and
is influenced by loading conditions and heart rate. Values
of < 7 cm/s suggest LV dysfunction.10

1120

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 51.7: Teis Index. Time a is the interval between cessation and
onset of mitral inflow. It includes isovolumic contraction time (ICT),
ejection time (ET), and isovolumic relaxation time (IRT). Left ventricular ejection time b is the duration of the left ventricular outflow
velocity profile left ventricle (LV) outflow. The index of combined
left ventricular systolic and diastolic function (the sum of isovolumic contraction time and isovolumic relaxation time divided by
ejection time) is calculated as (a b)/b.

TWO-DIMENSIONAL SPECKLE
TRACKING ECHOCARDIOGRAPHY
AND VELOCITY VECTOR IMAGING
In addition to inward and longitudinal motion, the LV also
rotates and twists during the cardiac cycle. To quantify the
complexity of cardiac motion, a technique called speckle
tracking has been developed. Speckles are small groups
of myocardial pixels created by the interaction between
ultrasound beams and the myocardium.11 Many vendors
have developed algorithms for tracking these speckles.
Speckle tracking measures aspects of strain, or myocardial
deformation, that occur during the cardiac cycle. Radial
strain (thickening of the myocardium during the inward
motion of the ventricle), longitudinal strain (percentage
decrease in length of the myocardium during systole as the
base moves toward the apex), and circumferential strain
(change in length along the circumferential perimeter) can
be assessed.11 In addition to measuring strain and strain
rate, speckle tracking also assesses the rotation, twist, and

torsion of the heart. Rotation is defined as the movement

of the heart in relation to an axis through the middle of the
LV cavity from the apex to the base. Twist is the difference
between the rotation of the apex and the base. Torsion
is defined as the twist normalized to the length of the LV
cavity (i.e. twist divided by the vertical distance between
the apex and base).11 There are many ongoing studies
showing that changes in these parameters are useful in
subclinical detection of systolic dysfunction prior to a
visual or measured reduction in LVEF.11
Velocity vector imaging also uses speckle tracking
and incorporates this tracking into velocity vectors taken
from the LV endocardium and epicardium to follow the
direction of the LV myocardium. Unlike for TDI, where
the myocardial velocities being interrogated must be
from tissue moving parallel to the ultrasound beam (only
movements toward and away from the probe), speckle
tracking and velocity vector imaging are not limited
by the angle at which velocities are obtained and can
better account for movements of the myocardium in
multiple directions.11 A more detailed description of these
modalities and their use assessing systolic and diastolic
left ventricular dysfunction can be found in other chapters
of this book.

MYOCARDIAL PERFORMANCE INDEX

Myocardial performance index, or the Teis Doppler
index, is the sum of isovolumic contraction time (ICT)
and isovolumic relaxation time (IRT) divided by ejection
time (ET) and, as such, reflects global (combined systolic
and diastolic) cardiac function.12 It provides a measure of
ventricular function independent of ventricular geometry.
The ICT corresponds to the interval between mitral valve
closure and aortic valve opening as measured by pulsed
Doppler from the apical position. Physiologically, this
correlates with influx of calcium into the mycoplasma.12
The IRT is the interval between aortic valve closure and
onset of mitral valve opening and represents the removal
of calcium from the myoplasm by calcium-ATPases.12 The
ejection time is the interval from the onset to the end of the
LV outflow velocity pattern13 (Fig. 51.7).
The presence of arrhythmias including atrial
fibrillation, frequent atrial and ventricular ectopy, and
tachycardias limit the application of the Tei-index. Pseudo
normalization of the index also limits its applicability in
patients with restrictive filling patterns.14

Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function

1121

Figs 51.8A and B: Contrast echocardiography. (A) Precontrast. The left ventricle (LV) cavity shows multiple trabeculations
(arrowhead) in the apex consistent with noncompaction. LV endocardial border is not well visualized in this area; (B) Postcontrast. Following injection of the contrast agent, the LV cavity is completely filled with contrast echoes, resulting in complete delineation of the
endocardium.

CONTRAST ECHOCARDIOGRAPHY
IN THE ASSESSMENT OF LEFT
VENTRICULAR SYSTOLIC FUNCTION
Commercially available echo contrast agents are widely
used to assist in determination of left ventricular systolic
function and evaluation of cardiac chambers and
myocardial perfusion. In patients with poor acoustic
windows, contrast can be given to help enhance detection
of the endocardial border and result in more accurate
visual estimates of left ventricular systolic function and
measurements of left ventricular volumes. Echo contrast
agents consist of reflective microbubbles. They are injected
intravenously and pass through right heart, the pulmonary
circulation, and into the left side of the heart, where they
opacify the left heart chambers and help delineate the
endocardial borders. The technique is similar to the more
invasive left ventriculogram obtained by injection of
contrast during left heart catheterization15,16 (Figs 51.8A
and B). Other features that can be adjusted to improve
contrast opacification include harmonic imaging and low
mechanical index imaging. With these advances, contrast
echocardiography provides improved endocardial border
imaging, resulting in better detection of wall motion
abnormalities, ventricular volume, and ejection fraction.
The result is a more accurate estimate of LV systolic
function.

ARTERIALVENTRICULAR COUPLING
The concept of arterialventricular coupling (EA/ELV) looks
at how properties of the arterial system affect the function
of the LV. Several studies have looked at how effective
arterial elastance (EA, arterial load) and left ventricular
end-systolic elastance (ELV, LV performance) relate and
affect cardiac performance especially in conditions
where the arterial tree becomes thicker and stiffer like
aging, heart failure, and hypertension.17 EA is calculated
as end-systolic pressure (ESP) divided by stroke volume
(SV) and serves as an index of the vascular load on the
LV. ESP is estimated as systolic blood pressure times 0.9
and SV is EDV ESV as obtained from 2D/Doppler echo
methods. ELV is noninvasively calculated using a modified
single-beat method to estimate end-systolic elastance
from arm-cuff pressures (systolic and diastolic BP), echoDoppler SV, echo-derived ejection fraction, and estimated
normalized ventricular elastance at arterial end-diastole.
It represents a relatively load-independent measure of LV
performance. Arterialventricular coupling is evaluated as
the ratio of these values (EA/ELV) and maximal efficiency is
attained when EA/ELV approaches 0.5.17 In states of elevated
afterload (e.g. aging, heart failure, and hypertension), there
is increased total peripheral resistance, left ventricular
concentric remodeling, inefficient arterialventricular
coupling, and ultimately, impaired LV function.18,19

1122

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

A community-based study by Redfield et al. speculated

that an increase in heart failure with preserved ejection
fraction (HFnlEF) especially amongst elderly women,
may be related to maintenance of optimal arterial
ventricular coupling.20 In an effort to maintain stroke
volume in the setting of higher arterial elastance as a
result of aging, there is an increase in left ventricular
systolic stiffness. Unfortunately, increases in these
parameters are not without consequence. Redfield et al.
note that altered LV chamber geometry (e.g. hypertrophy,
concentric remodeling, or fibrosis) to maintain arterial
ventricular coupling and resultant impaired LV relaxation
(diastolic dysfunction) could contribute to the increase
in HFnlEF they observed in elderly women.20 A study by
Lam et al also noted an increase in arterial elastance and
left ventricular end-systolic elastance in hypertensive and
HFnlEF patients as compared to healthy controls.21

THREE-DIMENSIONAL TRANS
THORACIC ECHOCARDIOGRAPHY
With 2D transthoracic echocardiography (2D TTE), only
one slice of the LV can be obtained at a time. Obtaining
other slices to fully examine the LV requires moving the
transducer and adjusting the angle of the transducer in
various positions. With three-dimensional transthoracic
echocardiography (3D TTE), the transducer emits
hundreds of ultrasound waves through the heart allowing
one to obtain a full volume 3D data set of the entire LV.16 This
3D volume can then be cropped using any desired plane
angulation. For example, a single apically acquired 3D
data set potentially allows for display and analysis of all the
standard apical 2D views (apical 2-, 3-, 4-, and 5-chamber
views). This data set can also be used for analysis of shortaxis views from the apex to the base of the LV. In 2D echo,
many geometric assumptions are made about LV shape.16
As mentioned earlier, many of the formulas, including
the commonly used Simpsons biplane method of discs,
calculate LV volumes based on areas determined from
only two imaging planes.
According to a meta-analysis by Dorosz et al. Threedimensional echocardiography (3DE) provided more
precise and accurate quantification of LV volumes and
LVEF compared to two-dimensional echocardiography
(2DE).22 3DE volumes were obtained by either a slice
method or a mesh method. The slice method involved
manual tracing of equally spaced individual long-or shortaxis slices at end-systole and end-diastole. The mesh

method required identification of 35 points at the apex and

mitral annulus in the two- and four-chamber end-diastolic
and end-systolic views. Software using automated borderdetection created a 3D endocardial shell of the LV from
which a volume was calculated.22 They noted that 3DE also
had less intraobserver and interobserver variability when
compared with 2DE and under-represented true values
approximately 50% less often. Dorosz et al. did find that
compared to cardiac magnetic resonance imaging (CMR),
3DE underestimated LV volumes and there was significant
variability in the LV volumes. This was more pronounced
in patients with poor windows or large ventricles due to
inability to fit the entire ventricle into the sector scan. They
acknowledged that using CMR as a gold standard might
be problematic due to errors in border detection and
controversy surrounding the inclusion of basal LV planes.22
Three-Dimensional transthoracic echocardiography
continues to be increasingly used in assessment of ventri
cular volumes, ejection fractions, valvular disorders, cong
enital heart disease, and evaluation of cardiac masses.23
Further discussion regarding the specific advantages of
this modality can be found in other chapters in this book.

REFERENCES
1. Pereztol-Valds O, Candell-Riera J, Santana-Boado C, et
al. Correspondence between left ventricular 17 myocardial
segments and coronary arteries. Eur Heart J. 2005;
26(24):263743.
2. Lang RM, Bierig M, Devereux RB, et al; Chamber
Quantification Writing Group; American Society of
Echocardiographys Guidelines and Standards Committee;
European Association of Echocardiography. Recomm
endations for chamber quantification: a report from the
American Society of Echocardiographys Guidelines and
Standards Committee and the Chamber Quantification
Writing Group, developed in conjunction with the
European Association of Echocardiography, a branch of
the European Society of Cardiology. J Am Soc Echocardiogr.
2005;18(12):144063.
3. Wilson DJ, North N, Wilson RA. Comparison of Left
Ventricular Ejection Fraction Calculation Methods.
Echocardiography. 1998;15(8 Pt 1):70912.
4. Feigenbaum, H. Role of M-mode technique in todays
echocardiography. J Am Soc Echocardiogr. 2010;23:24057.
5. Quinones MA, Waggoner AD, Reduto LA, et al. A new,
simplified and accurate method for determining ejection
fraction with two-dimensional echocardiography. Circu
lation. 1981;64(4):74453.
6. Baran AO, Rogal GJ, Nanda NC. Ejection fraction deter
mination without planimetry by two-dimensional
echocardiography: a new method. J Am Coll Cardiol.
1983;1(6):14718.

Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function

7. Otto CM. Textbook of Clinical Echocardiography.

Philadelphia: Saunders Elsevier; 2009.
8. Palmiero P, Maiello M, Nanda NC. Is echo-determined
left ventricular geometry associated with ventricular
filling and midwall shortening in hypertensive ventricular
hypertrophy? Echocardiography. 2008;25(1):206.
9. Oh JK, Seward JB, Tajik AJ. The Echo Manual. 6th ed.
Philadelphia: Lippincott Williams & Wilkins; 2006.
10. Gulati VK, Katz WE, Follansbee WP, et al. Mitral annular
descent velocity by tissue Doppler echocardiography as
an index of global left ventricular function. Am J Cardiol.
1996;77(11):97984.
11. Biswas M, Sudhakar S, Nanda NC, et al. Two- and threedimensional speckle tracking echocardiography: clinical
applications and future directions. Echocardiography.
2013;30(1):88105.
12. Lax JA, Bermann AM, Cianciulli TF, et al. Estimation of
the ejection fraction in patients with myocardial infar
ction obtained from the combined index of systolic and
diastolic left ventricular function: a new method. J Am Soc
Echocardiogr. 2000;13(2):11623.
13. Arnlv J, Ingelsson E, Risrus U, et al. Myocardial perfor
mance index, a Doppler-derived index of global left
ventricular function, predicts congestive heart failure in
elderly men. Eur Heart J. 2004;25(24):22205.

14. Karatzis EN, Giannakopoulou AT, Papadakis JE, et al.
Myocardial performance index (Tei index): evaluating its
application to myocardial infarction. Hellenic J Cardiol.
2009;50(1):605.
15. Miller AP, Nanda NC. Contrast echocardiography: new
agents. Ultrasound Med Biol. 2004;30(4):42534.
16. Mehta AM, Singh P, Nanda NC, et al. Left ventricular
systolic function assessment by echo doppler examination.

1123

Proceedings of the Preconference CME Program of

the XV Annual Conference of the Indian Academy of
Echocardiography, February 1114, 2010, Kochi, India.
17. Chantler PD, Lakatta EG, Najjar SS. Arterial-ventricular
coupling: mechanistic insights into cardiovascular
performance at rest and during exercise. J Appl Physiol.
2008;105(4):134251.
18. Fernandes VR, Polak JF, Cheng S, et al. Arterial stiffness is
associated with regional ventricular systolic and diastolic
dysfunction: the Multi-Ethnic Study of Atherosclerosis.
Arterioscler Thromb Vasc Biol. 2008;28(1):194201.
19. Saba PS, Ganau A, Devereux RB, Pini R, et al. Impact of
arterial elastance as a measure of vascular load on left
ventricular geometry in hypertension. J Hypertens. 1999;
17(7):100715.
20. Redfield MM, Jacobsen SJ, Borlaug BA, et al. Age- and
gender-related ventricular-vascular stiffening: a comm
unity-based study. Circulation. 2005;112(15): 225462.
21. Lam CS, Roger VL, Rodeheffer RJ, et al. Cardiac structure
and ventricular-vascular function in persons with heart
failure and preserved ejection fraction from Olmsted
County, Minnesota. Circulation. 2007;115(15):198290.
22. Dorosz JL, Lezotte DC, Weitzenkamp DA, et al. Perfor
mance of 3-dimensional echocardiography in measuring
left ventricular volumes and ejection fraction: a syste
matic review and meta-analysis. J Am Coll Cardiol.
2012;59(20):1799808.

23. Nanda NC, Miller AP. Real time three-dimensional
echocardiography: specific indications and incremental
value over traditional echocardiography. J Cardiol. 2006;
48(6):291303.

CHAPTER 52
How to Assess Diastolic Function
Hisham Dokainish

Snapshot

Integrating Echocardiographic Variables for Accurate

Novel Imaging Techniques and Future Directions

Diagnosis of Diastolic Function

INTRODUCTION
In patients presenting with dyspnea, accurate assessment
of left ventricular (LV) systolic and diastolic function is of
utmost importance to establish or exclude heart failure as
a cause or component of dyspnea. Echocardiography with
Doppler readily assesses LV diastolic function; advantages
include that echocardiography is noninvasive, does not
require radiation, is portable, rapid, readily available,
and in competent hands, it can provide an accurate and
comprehensive assessment of LV systolic and diastolic
function. Correct assessment of LV diastolic function is
relevant in patients with both depressed and preserved LV
ejection fraction (EF < 50%, and 50%, respectively). Tissue
Doppler (TD) imaging has been useful in demonstrating
impaired LV relaxation in the setting of preserved left
ventricular ejection fraction (LVEF), which, in the setting
of increased cardiac volume, can result in elevated LV
filling pressures and dyspnea due to diastolic heart failure.
TD imaging is not always critical in patients with depressed
LVEF, since such patients by definition have impaired LV
relaxation, and thus significant increases in volume will
result in increases in LV filling pressure due to impaired
LV compliance. Thus, in depressed LVEF, transmitral
flow velocities (E and A, and E/A) and deceleration
time, pulmonary venous Doppler, left atrial volume, and
pulmonary artery (PA) pressures suffice for the accurate
assessment of LV filling pressures. Overall, diastolic
assessment by echo Doppler can be readily achieved in

by using a comprehensive diastolic assessmentincor

porating many 2-dimensional (2D), conventional, and
tissue Doppler variablesas opposed to relying on any
single, diastolic parameter, which can lead to errors.

Two-Dimensional Echocardiography:
Left Ventricular Mass and Wall Motion,
and Left Atrial Size
According to current guidelines, the following three
criteria are needed for the diagnosis of diastolic heart
failure (DHF): clinical picture consistent with HF, demon
stration of preserved LVEF, and demonstration of diastolic
dysfunction.1 Clinically, diastolic dysfunction, secondary
to impaired LV relaxation and increased LV stiffness, is
usually demonstrated by echocardiography and Doppler.26
The best correlate of symptoms and survival in DHF is
elevation of left atrial (or left ventricular filling) pressure,
readily estimated using comprehensive echocardiography
with Doppler.1,2 Demonstration of preserved LVEF is
readily demonstrated with 2D echocardiography.7 It should
be noted that DHF is a term used relatively intercha
ngeably with HF with preserved LVEF and HF with
normal LVEF.
In DHF, LVEF is preserved 50%, yet left atrial
pressuressynonymous with LV filling pressures in
the absence of obstructive mitral valve (MV) disease
are elevated, causing increased pulmonary venous

Chapter 52: How to Assess Diastolic Function

1125

Fig. 52.1: Left ventricular hypertrophy in a patient with diastolic

dysfunction. Chronic hypertension is a common scenario for the
development of diastolic dysfunction, and the hypertrophied left
ventricle (LV) develops impaired relaxation, and in the right loading conditions, can result in elevated left atrial (LA) pressure. This
patient had concentric LV hypertrophy (LV mass index = 119 g/m2).

Fig. 52.2: Presence of left atrial dilation in the patient with left
ventricular diastolic dysfunction. The same patient as in
Figure 52.1 has severely dilated left atrium (LA) from chronic
elevation in LA pressures in the setting of left ventricular hypertrophy from chronic hypertension. Note that the LA does not appear
significantly dilated by anteroposterior diameter in Figure. 52.1; this
is the reason current guidelines recommend the measurement of LA
volume in the apical views. This patient had severe LA enlargement,
with an unindexed LA volume of 137 mL and an indexed volume of
72 mL/m2.

pressures and dyspnea at rest or during exertion.16 In

order for left atrium (LA) pressures to be elevated in the
absence of significantly depressed LVEF, LV relaxation
and compliance generally are depressed, most often
occurring in hypertensive or ischemic heart disease.25
Two-dimensional echocardiography, therefore, identifies
LV abnormalities that create the substrate for LV diastolic
dysfunction: LV hypertrophy and LV wall motion
abnormalities. Increased LV mass ( 90 g/m2 for women
and 115 g/m2 for men; i.e. LV hypertrophy) is common
in patients with DHF5 (Figs 52.1 and 52.2). Previous
studies have correlated increasing degrees of LV mass
with increasing LV diastolic dysfunction and filling
pressures.8 In addition, since LVEF can be preserved even
in the presence of significant coronary artery disease,
LV wall motion abnormalities create the substrate for
significant LV diastolic dysfunction even in the patient
with preserved LVEF who may have a diagnosis of DHF.
Therefore, accurate identification of LV wall motion
abnormalities is of great importance in the assessment of
the patient with potential diastolic dysfunction. Since LA
pressures are elevated in patients with significant diastolic
dysfunction in the presence of increased preload, and

since the LA cannot adequately empty in to the LV during

diastole in this hemodynamic scenario, LA enlargement
( 30 mL/m2) is usually seen.5 Increasing LA size correlates
with increasing LV filling pressures and worse outcome in
patients with diastolic HF.9 In addition, LA size has been
called the barometer of LV diastolic dysfunction or LV
filling pressures, although certainly other entities, such as
atrial fibrillation or chronic hypertension, can result in LA
enlargement in the absence of significant elevation of LA
pressure.6 It has therefore been said that LA volume has a
better negativeas opposed to positivepredictive value
for significant diastolic dysfunction and heart failure;
that is, a normal or small LA largely excludes significantly
elevated LA pressure, while a large LA volume may occur
in the absence of significant LA dilation.10 Studies have
also shown that LA volume is a much better measurement
of LA enlargement than a simple anteroposterior diameter,
and therefore is the recommended way to measure LA size
by echocardiography.6 It is also important to integrate 2D
echocardiographic variables in the assessment of diastolic
function; for instance, in cases of ischemic or infiltrative
heart disease, significant LV hypertrophy may be absent,
yet LA volumes are often enlarged.5

1126

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 52.3: Transmitral diastolic inflow for the assessment of left

ventricular filling pressures. (Left panel): mitral inflow in the normal heart shows early transmitral diastolic inflow greater than
late transmitral diastolic inflow (E > A) due to rapid diastolic function and normal left ventricular filling pressures (LVFP); (Middle
panel): in the patient with impaired LV relaxation but normal LVFP,
E is lower than A, as the LV depends more on atrial kick for LV
filling; this is also termed Grade I diastolic dysfunction (DD); (Right
panel): in the patient with pseudonormal filling pattern, there is
impaired LV relaxation but E > A, and is caused by elevated LA
pressures; this is also termed Grade I diastolic dysfunction (DD).
Valsalva maneuver and tissue Doppler imaging can help distinguish normal from pseudonormal filling (see Fig. 52.5 and text for
details).

Fig. 52.4: Restrictive transmitral filling pattern. When left ventricular

(LV) filling pressures are severely elevated due to increased LV
diastolic stiffness, early transmitral diastolic flow (E) has a high
velocity because there is an initial high gradient between very
elevated left atrial (LA) pressure and LV diastolic pressure.
However, due to elevated resting LV diastolic pressures, the LA
and LV pressures rapidly equilibrate, resulting in a rapid deceleration time (DT) of transmitral E. In general, in a patient with cardiac disease, restrictive filling occurs when E/A > 2 and DT < 150
milliseconds. A = late transmitral diastolic velocity.

Identification of Diastolic Dysfunction

and Demonstration of Elevated Left
Ventricular Filling Pressures

this pattern is termed pseudonormalization. In markedly

elevated LV filling pressure in which LV stiffness is high,
the MV is forced open early due to high LA pressure,
but there is rapid equilibration with the high resting
LV diastolic pressure resulting in a rapid deceleration
time of E. This pattern is termed restrictive filling
(Fig. 52.4).35 The grades of diastolic function, as assessed
using comprehensive echo Doppler examination, are
shown in Figure 52.5.

Transmitral Doppler
Pulsed Doppler interrogation of mitral valve diastolic
flow (mitral inflow pattern) is critical for the assessment
of LV filling pressures. Early mitral filling depends on
intrinsic LV relaxation, and the difference between LA and
LV early (or opening) diastolic pressure.5 In a healthy,
young heart with normal, rapid diastolic suction, the LV
literally sucks blood into the LV, resulting in rapid LA
emptying. In this scenario, there is a relatively tall E-wave
and a shorter A (late diastolic or atrial contraction wave;
Fig. 52.3). In an LV with impaired relaxation but normal
LV filling pressures, there is no rapid LV diastolic suction,
thus LA emptying is more gradual and results in a relatively
low velocity E-wave; LA emptying is therefore dependent
on LA contraction and results in a relatively high amplitude
A-wave. In the setting of impaired LV relaxation and mildly
elevated LA pressure, high LA pressure that drives open
the MV, resulting in a large E-wave and smaller A-wave;

Valsalva Maneuver
In the Valsalva maneuver in which the patient forces
expiration against a closed glottis, there is increased
intrathoracic pressure that results in decrease in right
heart filling which by definition, results in decreased LV
filling (decreased preload). Since a pseudonormal filling
pattern exists in the setting of elevated LA pressure in
the presence of impaired LV relaxation, this decrease in
preload lowers LA pressure, which then unmasks the
underlying impaired relaxation pattern (i.e. E > A in the
setting of impaired relaxation and with Valsalva maneuver
changes the transmitral pattern to E < A; Fig. 52.5). On the

Chapter 52: How to Assess Diastolic Function

Fig. 52.5: Grades of left ventricular diastolic dysfunction. Left

ventricular (LV) diastolic function ranges from normal (Grade 0)
to impaired relaxation (Grade I), to pseudonormal (Grade II), to
restrictive (Grade III), and irreversibly restrictive (Grade IV). LV
relaxation and left atrial pressures (LAp) increase from Grades 0
to IV, as does LA volume. Mitral valve inflow (MVI), tissue Doppler
imaging, Valsalva maneuver, flow propagation velocity (Vp), and
pulmonary venous flow are all helpful in distinguishing grades of
LV diastolic function and should be used together for an integrated
approach to the assessment of diastolic function as recommended
in current guidelines. (Adapted from ref. 31).

other hand, in the setting of normal diastolic function, the

decrease in preload resulting from the Valsalva maneuver
preserves the E > A pattern, without changing it to E < A.
Therefore, one of the main uses of the Valsalva maneuver
similar to tissue Doppler e'is to help distinguish normal
from pseudonormal filling pattern. In the presence
of a restrictive filling pattern, the Valsalva maneuver
will decrease preload and therefore help distinguish
irreversible restrictive filling pattern (in which E >> A
will not change) from reversible restrictive filling, where
decreased preload changes the restrictive filling pattern
to either pseudonormal (E > A) or impaired relaxation
pattern (E < A), since the increased intrathoracic pressure
resulting from Valsalva decreases LA pressure.

Tissue Doppler Imaging

The best estimate of LV relaxation, which is relatively
nonload dependent in patients with cardiac disease, is
tissue Doppler early diastolic mitral annular velocity
(e');1114 the slower the LV relaxation, the lower the e'
velocity. The resulting E/e' ratio, has been validated as a
reasonably reliable non-invasive indicator of LV filling

1127

pressure in patients with preserved or depressed LVEF

(Figs 52.6A to D).1114 It should be mentioned that one
study, performed in the intensive care unit in patients in
decompensated heart failure, questioned the correlation
of /e' and LV filling pressures.15 In patients with normal
hearts, E/e' does not accurately predict LV filling pressure
due to correlation of e' with LV filling pressures in such
subjects, as opposed to lack of such a correlation in patients
with cardiac disease.16 The E/e' ratio has since been demon
strated to be useful in estimating LV filling pressures in
hypertrophic cardiomyopathy,17 sinus tachycardia,18 atrial
fibrillation,19 and postcardiac transplantation.20 The E/A
ratio, mitral deceleration time, and E/e' ratio have all been
shown to be useful echocardiographic indicators of LV
diastolic dysfunction, and more particularly, of elevated
LV filling pressures.5 These variables have also been shown
to be markers of outcome in patients with HF.9,21

Flow Propagation Velocity

In the apical four-chamber view, color Doppler imaging
can be used and then M-mode applied to semi-quantitate
blood flow across the mitral valve to the LV apex (Fig. 52.7).
In this way, the early diastolic filling wave by color M-mode,
which appears in red color as blood flow from the mitral
valve level to the LV apex can be identified. The slope of this
early diastolic color M-mode wave (Vp) is rapid (vertical)
in patients with normal diastolic function due to rapid
diastolic suction in which blood quickly flows from MV to
LV apex. However, in the presence of increasingly impaired
relaxation, this slope become flatter and flatter, reflecting
increasingly impaired LV relaxation. A ratio, E/Vp, similar
to E/e, has therefore been developed and validated, and
correlates to mean LA pressure.22 An E/Vp > 15 reasonably
correlates with PCWP > 15 mm Hg, although there are
many hemodynamic, rhythmic, and myocardial motion
variables that can impact this relationship. Furthermore,
some studies have shown that, in comparison to invasive
measurement of LV filling pressures, E/e' appears more
accurate than E/Vp.23

Pulmonary Venous Flow

Pulmonary venous flow is also of great importance in the
assessment of LV diastolic function. In the normal heart
with rapid ventricular suction, the diastolic pulmonary
venous wave is augmented due to rapid flow through the
pulmonary veins into the LA, through the mitral valve, and

1128

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 52.6A to D: Tissue Doppler imaging for the assessment of left ventricular (LV) diastolic function. Figure A shows an apical fourchamber view with moderate to severe dilation of the left atrium (LA) with an LA volume index of 39 mL/m2; Figure B demonstrates
elevated early transmitral diastolic velocity (E) = 117 cm/s; Figure C shows very depressed mitral lateral annular early diastolic relaxation
velocity (e') = 3.5 cm/s; Figure D shows very depressed septal tissue Doppler (TD) early diastolic velocity (e') = 2.5 cm/s. Therefore,
E/e' septal = 47, and E/e' lateral = 33, indicating severely impaired LV relaxation with elevated LV filling pressures. (PCWP: Pulmonary
capillary wedge pressure).

into the LV. Therefore, in the completely normal heart, the

dominant PV diastolic wave corresponds to the dominant
transmitral E-wave and is a sign of normal LV lussotropic
function; the completely normal heart therefore has
PV S < D. However, when LV relaxation becomes impaired,
PV flow during LV diastole becomes truncated, and
therefore most filling occurs during LV systole, resulting
in S > D, which corroborates to transmitral E < A (Grade
I diastolic dysfunction; Fig. 52.8).24 When LA pressure
becomes elevated, PV flow during LV systole decreases,
as LA pressure in the setting of a closed MV prevents
normal PV flow, and PV flow becomes higher in diastolic
when the mitral valve opens, relieving elevated LA

pressure; this results in PV S < D, corresponding to E > A

(Grade II diastolic dysfunction). Therefore, PV flow is
subject to pseudonormalization in the same way as
transmitral flow. In restrictive filling, the PV pseudonormal
pattern becomes more exaggerated, with S << D, with a
rapid deceleration time of D. While the PV S/D ratio reflects
mean LA pressure, the PV atrial reversal wave reflects LV
end-diastolic pressure, as, if the LA contracts against the
high diastolic pressure in the LV, blood will preferentially
flow backward into the PV, as opposed to transmitrally into
the LV. Therefore, PV Ar wave is higher and longer than
transmitral A, rendering PV Ar-A a measure of LV enddiastolic pressure.

Chapter 52: How to Assess Diastolic Function

Fig. 52.7: Color Doppler flow propagation velocity (Vp) in the

assessment of left ventricular diastolic function. Placing the color
Doppler sample volume from the mitral annular level to the left
ventricular (LV) apex, the more rapid the LV relaxation, the faster
blood travels from the mitral annular level to the LV apex, and
hence the more vertical the color Doppler mitral inflow M-mode
and the more rapid the Vp slope (left panel). On the other hand,
the more impaired the LV relaxation, the slower it takes for blood
to go from the mitral annular to the LV apex, hence a flatter Vp
slope (right panel).

Pulmonary Artery Pressure

In the setting of elevated LA pressure, this pressure is
transmitted back into the pulmonary veins and across
the pulmonary venous-capillary bed into the pulmonary
arterioles and into the pulmonary arteries; therefore,
pulmonary arterial hypertension (PAH) can result.25 In this
way, PA pressure estimated by addition of an estimate of
RA pressure (by assessing IVC size and response to
respiration) is a good surrogate marker of significant,
and often chronic, LA pressure elevation (Figs 52.9A
and B). As recommended by guidelines, PASP > 35 mm Hg
often accompanies advanced or significant LV diastolic
dysfunction with elevated LA pressures.5 However, as
with LA volume, the presence of significant PAH does
not necessarily mean significant diastolic dysfunction, as
significant elevations in pulmonary vascular resistance
(PVR) due to intrinsic lung disease must first be excluded.
Likewise, normal PASP can be helpful in excluding
significant and long-standing LA pressure elevation,
provided a complete TR jet is obtained with correct
Doppler sample volume angulation in respect of the
direction of TR, and with correct RA pressure estimation.
PA end-diastolic pressure, which in the absence of
significant elevations in PVR can be a good estimate of

1129

Fig. 52.8: Pulmonary venous Doppler velocities in the assessment of left atrial pressures. In normal heart, systolic pulmonary
venous (PV) flow (S) is lower than diastolic PV flow (D), as rapid
left ventricular (LV) suction during diastole results in elevated PV D
velocities; thus, PV S < D. In patients with impaired LV relaxation
and elevated LA pressure, when the mitral valve (MV) is closed in
ventricular systole, the elevated LA pressure prevents PV S flow,
and therefore most flow occurs when the MV opens, resulting in
S < D. Therefore, as with mitral inflow velocities, PV velocities
are also prone to pseudonormal filling. In this example, there is
impaired LV relaxation due to cardiomyopathy, but LA pressure
is not elevated; thus, when the MV is closed (LV systole), there is
unimpeded flow through the PV, and hence S > D.

mean LA pressure, can be estimated from the pulmonary

regurgitation diastolic wave, with RA pressure then added
to it as is done with PASP.26

Assessment of Diastolic Function in Nonsinus

Rhythm and Other Special Situations
One commonly encountered scenario where it can be
challenging to accurately assess LV diastolic function
by echocardiography is in atrial fibrillation. Owing to
elevated heart rate, irregular RR intervals, and loss of
atrial contraction, echo Doppler assessment can be
difficult. However, mitral DT < 150 milliseconds, lack of
variation in E-wave velocity despite varying RR intervals
(as there remains an elevated opening gradient between
the LA and LV at mitral openingearly diastolic filling
E-wavedespite longer diastolic filling periods when the
LA pressure should decrease), IVRT < 65 milliseconds,
elevation of E/e' (>11), and the presence of pulmonary
hypertension in the absence of lung disease, are all clues
to the presence of elevated LA pressure in the setting of
AF.5,27,28 The E/e' ratio can also be used in patients with AF,

1130

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 52.9A and B: Pulmonary artery systolic pressure in the assessment of left ventricular diastolic function. In patients with significant left ventricular (LV) diastolic dysfunction with chronically elevated LV filling pressures, back pressure through the left atrium (LA),
into the pulmonary veins, and across the pulmonary venous capillary bed into the pulmonary arterioles and pulmonary arteries (PA),
results in elevation of PA pressure. Thus, PA systolic pressure elevation, in the absence of significant intrinsic lung disease and resultant
elevated pulmonary vascular resistance (PVR) is a reasonable correlate of elevated LA pressures. PA systolic pressure can be estimated by Doppler using the tricuspid regurgitation peak systolic velocity and adding to it an estimate of right atrial (RA) pressure. This image
shows a TR velocity of 3.64 m/s, equivalent to a TR systolic pressure of 53 mm Hg, which indicates at least moderate PA hypertension
in a patient with chronically elevated LA pressure due to ischemic cardiomyopathy and diastolic dysfunction. (RV: Right ventricular).

Fig. 52.10: An integrated approach to the assessment of left ventricular diastolic function: normal LV ejection fraction. As recommended in the current guidelines, use of multiple echo Doppler
parameters results in a more accurate assessment of left ventricular (LV) diastolic function than using any single echo Doppler
parameter in isolation. In the patient with normal LV ejection fraction (EF), it is reasonable to start with early transmitral diastolic
velocity/tissue Doppler early diastolic velocity (E/e'), as it can be
difficult to discern whether a patient with preserved LVEF has impaired or normal LV relaxation. Following E/e', other echo Doppler
variables are added to result in an accurate assessment of LV
diastolic function (from ref. 5).

although with somewhat lower accuracy than in patients

in sinus rhythm, for the estimation of LV filling pressures,
as long as greater than five cardiac cycles are used and
averaged (which holds for any Doppler parameter
in AF).19 In patients who are in supraventricular
tachycardia, atrial flutter, paced rhythm, or heart block,
LV diastolic assessment can be very difficult, although
the presence of both significant LA enlargement and
pulmonary hypertension in the absence of lung disease
can be an important clue to elevated LA pressures in
these scenarios. Another unclear scenario is the effect of
significant mitral regurgitation (MR) on e' and the E/e'
ratio in estimating LV filling pressures. It has been shown
that in patients with secondary MR (due to LV disease),
E/e' accurately predicted PCWP; however, in patients with
primary MR (due to a primary mitral valve abnormality),
E/e' was not reliably predictive of PCWP.29

INTEGRATING ECHOCARDIOGRAPHIC
VARIABLES FOR ACCURATE
DIAGNOSIS OF DIASTOLIC FUNCTION
The use of a single diastolic variable (such as E/e' or LA
volume in isolation) can lead to significant errors in
the assessment of LV diastolic function.5 It is therefore

Chapter 52: How to Assess Diastolic Function

1131

Fig. 52.11: An integrated approach to the assessment of left

ventricular diastolic function: depressed ejection fraction. As recommended in the current guidelines, use of multiple echo Doppler parameters results in a more accurate assessment of left
ventricular (LV) diastolic function than using any single echo
Doppler parameter in isolation. In the patient with depressed
LV ejection fraction (EF), it is reasonable to start with early and
late transmitral diastolic inflow velocities and deceleration time
(E, A and DT, respectively), as it can assumed that patients with
depressed LVEF (< 50%) have, by definition, impaired LV relaxation. Following transmitral diastolic flow, other echo Doppler variables are added to result in an accurate assessment of LV diastolic
function (from ref. 5).

Fig. 52.12: Longitudinal strain by speckle echocardiography in

the demonstration of systolic myocardial dysfunction in a patient
with normal left ventricular ejection fraction. This elderly patient
presented with dyspnea and underwent echocardiography with
speckle imaging. Left ventricular ejection fraction (LVEF) was
calculated at 55%, while global longitudinal peak strain (GLPS
Avg) was 11.1, consistent with significantly decreased
myocardial systolic function (normal longitudinal systolic strain
less than 16%). GLPS Avg was obtained by averaging GLPS in
the apical long-axis (LAX), four-chamber (A4C), and two-chamber
(A2C) views. There are substantial data using tissue Doppler and
speckle imaging demonstrating significant systolic abnormalities
in patients with diastolic dysfunction and heart failure despite
normal LVEF. This patient was subsequently diagnosed with diastolic heart failure and single vessel coronary artery disease.

of great importance to integrate several variables2D,

conventional, and tissue Dopplerin order to arrive at a
correct diastolic assessment. Indeed, current guidelines
recommend an integrated approach of many diastolic
variables (Figs 52.10 to 52.12), and data have shown that
additional echocardiographic variables, when added to
E/e' can result in more accurate diastolic determination,
compared to invasively measured LV filling pressures, than
E/e' alone.30 Not infrequently, echo Doppler parameters
appear to conflict: for instance, in a patient with normal
LVEF, E/e' = 13, but LA volume is not enlarged, E < A, and
there are normal pulmonary pressures by Doppler. In such
cases, the E/e' ratio should likely be dropped, since all
other variables point toward normal LV filling pressures.
Therefore, as a rule, multiple echo Doppler parameters of
LV diastolic function should be assessed in every patient,31
and the conclusion to which most parameters point
should be the overall diastolic assessment, with outlying
parameters discarded. In most cases of conflicting echo
Doppler diastolic parameters, a cogent conclusion can be

reached, although in some cases, the diastolic assessment

may remain equivocal. Above all, no single diastolic
parameter should be used in isolation to arrive at a
diastolic conclusion in a given patient.5

NOVEL IMAGING TECHNIQUES AND

FUTURE DIRECTIONS
Non-Doppler-based 2D imaging (speckle echocardi
ography) tracks signature grayscale characteristic of
points in the LV myocardium, thus providing information
on displacement, velocity, deformation, and deformation
rate (strain and strain rate, respectively), independent
of angulation and cardiac translational motion.32
Such variables have provided detailed information on
myocardial mechanics in hypertensive heart disease,
hypertrophic cardiomyopathy, diastolic and systolic LV
failure, as well as in cases of pulmonary hypertension.3335
Currently, such speckle-based measures are being studied
to assess their role in identifying patient outcome in

1132

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

various heart failure states. One of the most attractive

features of speckle tracking is that it can demonstrate the
presence of systolic abnormalities, especially regional
ones, in the presence of preserved LVEF in patients with
cardiac disease (see Fig. 52.12).36 Speckle tracking can
also demonstrate systolic and diastolic abnormalities in
multiple vectors (longitudinal, radial, circumferential,
and rotational) as characteristic myocardial markers are
tracked throughout the cardiac cycle and in space as the
heart translates in the thoracic cavity. In particular, patients
with diastolic dysfunction and DHF have been shown to
have preserved LV twist (systole) and untwist (diastole)
but impaired longitudinal strain, whereas patients with HF
with depressed EF have impaired twist/untwist as well as
depressed longitudinal and circumferential strain.3034

SUMMARY
Comprehensive echocardiography with 2D imaging, and
spectral and color Doppleras well as newer techniques
like speckle strain echocardiographyprovide a complete
assessment of cardiac diastolic function. This assessment,
which includes LV mass and regional wall motion
assessment, LA volume, transmitral, pulmonary venous,
and tissue Doppler as well as estimation of PA systolic and
diastolic pressures, can provide accurate assessments of
diastolic function in the majority of patients. It is important
to note that, as recommended in current guidelines, use
of any single echo Doppler diastolic variable (e.g. only
E/e') in isolation, can lead to errors. Therefore, it is of
utmost importance that a comprehensive assessment of
LV diastolic function include integration of all available 2D
and Doppler, and tissue Doppler variables to arrive at the
most accurate diastolic assessment.

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1. Paulus WJ, Tschpe C, Sanderson JE, et al. How to diagnose
diastolic heart failure: a consensus statement on the diag
nosis of heart failure with normal left ventricular ejection
fraction by the Heart Failure and Echocardiography
Associations of the European Society of Cardiology. Eur
Heart J. 2007;28(20):253950.
2. Zile MR, Baicu CF, Gaasch WH. Diastolic heart failure
abnormalities in active relaxation and passive stiffness of
the left ventricle. N Engl J Med. 2004;350(19):19539.
3. Oh JK, Hatle L, Tajik AJ, et al. Diastolic heart failure can be
diagnosed by comprehensive two-dimensional and Doppler
echocardiography. J Am Coll Cardiol. 2006;47(3):5006.

4. Lester SJ, Tajik AJ, Nishimura RA, et al. Unlocking the

mysteries of diastolic function: deciphering the Rosetta
Stone 10 years later. J Am Coll Cardiol. 2008;51(7):67989.
5. Nagueh SF, Appleton CP, Gillebert TC, et al. Recomm
endations for the evaluation of left ventricular diastolic
function by echocardiography. J Am Soc Echocardiogr.
2009;22(2):10733.
6. Abhayaratna WP, Seward JB, Appleton CP, et al. Left atrial
size: physiologic determinants and clinical applications.
J Am Coll Cardiol. 2006;47(12):235763.
7. Lang RM, Bierig M, Devereaux RB, et al. Recommendations
for chamber quantification. J Am Soc Echocardiogr 2005;
18:144063.
8. Dokainish H, Sengupta R, Pillai M, et al. Assessment of
left ventricular systolic function using echocardiography
in patients with preserved ejection fraction and elevated
diastolic pressures. Am J Cardiol. 2008;101(12):176671.
9. Dokainish H, Zoghbi WA, Lakkis NM, et al. Incremental
predictive power of B-type natriuretic peptide and
tissue Doppler echocardiography in the prognosis of
patients with congestive heart failure. J Am Coll Cardiol.
2005;45(8):12236.

10. Dokainish H, Zoghbi WA, Lakkis NM, et al. Optimal
noninvasive assessment of left ventricular filling pressures:
a comparison of tissue Doppler echocardiography and
B-type natriuretic peptide in patients with pulmonary
artery catheters. Circulation. 2004;109(20):24329.
11. Sohn DW, Chai IH, Lee DJ, et al. Assessment of mitral
annulus velocity by Doppler tissue imaging in the
evaluation of left ventricular diastolic function. J Am Coll
Cardiol. 1997;30(2):47480.
12. Nagueh SF, Middleton KJ, Kopelen HA, et al. Doppler tissue
imaging: a noninvasive technique for evaluation of left
ventricular relaxation and estimation of filling pressures.
J Am Coll Cardiol. 1997;30(6):152733.
13. Ommen SR, Nishimura RA, Appleton CP, et al. Clinical utility
of Doppler echocardiography and tissue Doppler imaging
in the estimation of left ventricular filling pressures: A
comparative simultaneous Doppler-catheterization study.
Circulation. 2000;102(15):178894.
14. Kasner M, Westermann D, Steendijk P, et al. Utility of
Doppler echocardiography and tissue Doppler imaging
in the estimation of diastolic function in heart failure
with normal ejection fraction: a comparative Dopplerconductance catheterization study. Circulation. 2007;
116(6):63747.
15. Mullens W, Borowski AG, Curtin RJ, et al. Tissue Doppler
imaging in the estimation of intracardiac filling pressure
in decompensated patients with advanced systolic heart
failure. Circulation. 2009;119(1):6270.
16. Firstenberg MS, Levine BD, Garcia MJ, et al. Relationship
of echocardiographic indices to pulmonary capillary
wedge pressures in healthy volunteers. J Am Coll Cardiol.
2000;36(5):16649.

Chapter 52: How to Assess Diastolic Function

17. Nagueh SF, Lakkis NM, Middleton KJ, et al. Doppler
estimation of left ventricular filling pressures in patients
with hypertrophic cardiomyopathy. Circulation. 1999;99(2):
25461.
18. Nagueh SF, Mikati I, Kopelen HA, et al. Doppler estimation
of left ventricular filling pressure in sinus tachycardia. A
new application of tissue Doppler imaging. Circulation.
1998;98(16):164450.
19. Sohn DW, Song JM, Zo JH, et al. Mitral annulus velocity in
the evaluation of left ventricular diastolic function in atrial
fibrillation. J Am Soc Echocardiogr. 1999;12(11):92731.
20. Sundereswaran L, Nagueh SF, Vardan S, et al. Estimation
of left and right ventricular filling pressures after heart
transplantation by tissue Doppler imaging. Am J Cardiol.
1998;82(3):3527.
21. Yu CM, Sanderson JE, Marwick TH, et al. Tissue Doppler
imaging a new prognosticator for cardiovascular diseases.
J Am Coll Cardiol. 2007;49(19):190314.
22. Garcia MJ, Ares MA, Asher C, et al. An index of early left
ventricular filling that combined with pulsed Doppler peak
E velocity may estimate capillary wedge pressure. J Am Coll
Cardiol. 1997;29(2):44854.
23. Rivas-Gotz C, Manolios M, Thohan V, et al. Impact of left
ventricular ejection fraction on estimation of left ventri
cular filling pressures using tissue Doppler and flow
propagation velocity. Am J Cardiol. 2003;91(6):7804.
24. Appleton CP, Galloway JM, Gonzalez MS, et al. Estimation
of left ventricular filling pressures using two-dimensional
and Doppler echocardiography in adult patients with
cardiac disease. Additional value of analyzing left atrial
size, left atrial ejection fraction and the difference in
duration of pulmonary venous and mitral flow velocity at
atrial contraction. J Am Coll Cardiol. 1993;22(7):197282.
25. Neuman Y, Kotliroff A, Bental T, et al. Pulmonary artery
pressure and diastolic dysfunction in normal left ventricular
systolic function. Int J Cardiol. 2008;127(2):1748.
26. Paraskevaidis IA, Tsiapras DP, Karavolias GK, et al. Dopplerderived left ventricular end-diastolic pressure prediction
model using the combined analysis of mitral and
pulmonary A waves in patients with coronary artery
disease and preserved left ventricular systolic function.
Am J Cardiol. 2002;90(7):7204.

1133

27. Nagueh SF, Kopelen HA, Quiones MA. Assessment of left

ventricular filling pressures by Doppler in the presence of
atrial fibrillation. Circulation. 1996;94(9):213845.
28. Al-Omari MA, Finstuen J, Appleton CP, et al. Echocardi
ographic assessment of left ventricular diastolic function
and filling pressure in atrial fibrillation. Am J Cardiol.
2008;101(12):175965.
29. Bruch C, Stypmann J, Gradaus R, et al. Usefulness of
tissue Doppler imaging for estimation of filling pressures
in patients with primary or secondary pure mitral regurgi
tation. Am J Cardiol. 2004;93(3):3248.
30. Dokainish H, Nguyen JS, Sengupta R, et al. Do additional
echocardiographic variables increase the accuracy of E/e
for predicting left ventricular filling pressure in normal
ejection fraction? An echocardiographic and invasive
hemodynamic study. J Am Soc Echocardiogr. 2010;23(2):
15661.
31 Ommen SR, Nishimura RA. A clinical approach to the
assessment of left ventricular diastolic function by
Doppler echocardiography: update 2003. Heart 2003;89
(Suppl 3):1823.
32. Perk G, Tunick PA, Kronzon I. Non-Doppler two-dimen
sional strain imaging by echocardiographyfrom tech
nical considerations to clinical applications. J Am Soc
Echocardiogr. 2007;20(3):23443.
33. Wang J, Khoury DS, Yue Y, et al. Preserved left ventricular
twist and circumferential deformation, but depressed
longitudinal and radial deformation in patients with
diastolic heart failure. Eur Heart J. 2008;29(10):12839.
34. Wang J, Khoury DS, Yue Y, et al. Left ventricular untwisting
rate by speckle tracking echocardiography. Circulation.
2007;116(22):25806.
35. Dokainish H, Sengupta R, Pillai M, et al. Usefulness of new
diastolic strain and strain rate indexes for the estimation of
left ventricular filling pressure. Am J Cardiol. 2008;101(10):
15049.
36. Nguyen JS, Lakkis NM, Bobek J, et al. Systolic and diastolic
myocardial mechanics in patients with cardiac disease and
preserved ejection fraction: impact of left ventricular filling
pressure. J Am Soc Echocardiogr. 2010;23(12):127380.

CHAPTER 53
Evaluation of the Right Ventricle
Vincent L Sorrell, Steve W Leung, Brandon Fornwalt

Snapshot

General Overview

Right Ventricle Morphology

Echocardiography

M-Mode Echocardiography

Two-Dimensional Echocardiography

Doppler Echocardiography

GENERAL OVERVIEW
The assessment of the right ventricle (RV) is valuable in
many patients with heart disease. In patients with either
RV volume overload (e.g. repaired tetralogy of Fallot [TOF],
atrial septal defect [ASD], anomalous pulmonary venous
return, tricuspid regurgitation [TR] from any cause) or
RV pressure overload (e.g. pulmonary hypertension from
any cause, pulmonary stenosis), management decisions
increasingly rely on evaluation of the RV size and function.
Their trends during serial follow-up examinations
predict heart failure, arrhythmias, and death and must
be reliable.1 The noninvasive diagnostic evaluation of RV
size and function in normal and pathological conditions
is daunting due to its complex shape, nonsymmetrical
regional contraction pattern, and the lack of published
literature on normal reference values. Most physicians
practicing echocardiography are comfortable analyzing
the relatively simple circular geometry of the left ventricle
(LV), but the RV is shaped like a pyramidal banana. The
inflow and outflow portions are separated. The normal RV
shape varies depending on orientation: sagittal view (echo

Two-Dimensional Strain (Speckle Tracking)

Three-Dimensional Echocardiography

Transesophageal Echocardiography

Hemodynamics

Other Imaging Modalities

short axis) is triangular (curved); axial view (echo long axis)

is crescent-shaped; and coronal view (not possible with
two-dimensional [2D] echo) is most similar to a teapot
(Fig. 53.1). The RV myocardial wall is highly trabeculated
and barely 3 mm thin. In summary, there is no convenient
geometric model that accurately approximates the normal
or the diseased RV shape.
In addition to the variable shape, the regional contr
action pattern is also unique to the RV. The normal RV
apex is virtually immobile and tethered to the LV apex,
and therefore is dominated by the shape and function of
the adjacent left ventricular apex. The global RV systolic
function is strongly influenced by the normally concave
interventricular septum and ventricular interdependence.
Acute and chronic pathological pressure and volume
overload will greatly impact global and regional RV
performance.
Finally, global RV performance is influenced by
volume shifts that occur with normal respiration. During
inspiration, venous return increases, causing an increased
RV preload, with a slight but detectable increase in RV
stroke volume. Therefore, when quantifying RV volume

Chapter 53: Evaluation of the Right Ventricle

Fig. 53.1: Three dimensional display of complex RV geometry.

Left column demonstrates a 3D echo and the middle column is
a 3D cardiac MR (CMR) exam of a normal patient. Top row: cardiac-aligned short axis; Middle row: sagittal orientation; Bottom
row: oblique coronal or frontal plane. The two images in the right
column represent conventional long axis orientation (four-chamber view) of CMR (top) and 2D echo (bottom).

and function with echo, one should consider whether data

were acquired during inspiration, expiration, or apnea
(preferred).
These features unique to the RV result in highly
variable interpretations despite highly trained experts in
echocardiography. Most clinical studies simply use the
eye-ball qualitative assessment rather than resorting
to quantitative, or even semiquantitative, estimates of
RV size and function. Unfortunately, compared with the
reference standard (cardiac magnetic resonance imaging
[CMR]), the ability to accurately detect severely dilated RV
size or moderate to severe RV dysfunction is low and the
interobserver variability is extremely poor.2
This chapter describes the unique characteristics of
the RV and offers a comprehensive, quantitative echocar
diographic (and multimodal imaging) approach to the
investigation of the normal and pathological RV.

RIGHT VENTRICLE MORPHOLOGY

The RV can be considered to comprise three individual
and separate componentsthe apex, the inflow, and the
outflow (Fig. 53.2). Although there is significant individual
normal variability, the anterior and posterior trabecular
muscles, and the smaller medial papillary muscle are
within the RV inflow region and connected to the tricuspid
valve leaflets via the chordae tendineae. The moderator

1135

Fig. 53.2: Left Image: Three-dimensional echocardiography (3DE)

volume rendered display with color-coded regions representing
the triangle of dysplasia. The arrows in the inflow and outflow regions reflect the myofiber alignment and direction of contraction.
The larger center arrow represents the ventricular septal motion
into the cavity (toward the left ventricle). Right Image: Posterioranterior orientation of a cadaveric cast from a normal RV. Note the
complex nongeometric shape as well as the extensive trabeculation throughout the RV myocardium, although slightly less obvious
in the so-called smooth inflow region.
Courtesy: Special thanks to Frank Marcus for the image.

band is a variably prominent muscular extension that

houses the electrical apparatus of the right bundle of His as
it travels from the ventricular septum to the anterolateral
region of the RV.
These RV regions are commonly the initial sites
involved in pathology and have been termed the triangle
of dysplasia.3 These anatomical regions develop separ
ately and at distinct embryological time points and are
consequently independently subjected to congenital
malformations. Each anatomical region has been
demonstrated to have unique responses to pathology as
well as pharmacological interventions. The right ventri
cular outflow tract (RVOT) has been demonstrated to
be more reactive than the RV inflow tract to inotropic
stimulation.4 This may be important when evaluating RV
response to treatment with inotropic drugs.
The global RV systolic function is determined by the
following individual RV contraction patterns: (a) move
ment of the basal free wall toward the apex (the bellows
effect); (b) the contraction of the RVOT; and (c) the
contribution of the LV (tethering) at the interventricular
insertion sites. The influence of the ventricular septum
(interventricular dependence) is illustrated by impairment
of RV function due to the adjacent diseased LV, but not

1136

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

muscle bundles, because these may alter the symmetric

contraction of the RV free wall.7,8 Moreover, different
regions of the RV contract at different times in healthy
volunteers with the inflow region reaching peak
contraction first, followed by the outflow region and lastly
the apex approximately 100 ms later.9 Although severe focal
regional wall motion abnormalities may exist in diseases
such as arrhythmogenic RV dysplasia/cardiomyopathy
(Movie clip 53.3B), these more subtle, normal regional
variations need to be recognized as normal or else they may
inadvertently lead to misinterpretation with important
downstream consequences.

ECHOCARDIOGRAPHY
Fig. 53.3: Cardiac magnetic resonance imaging midventricular
steady-state free precession (SSFP) images of a normal (control;
top) and pathological (tetralogy; bottom) heart demonstrating the
variation in timevolume (TV) curves (right side). Note the dilated
right ventricle (RV) and delay in contraction of the surgically repaired tetralogy of Fallot patient (tetralogy). Right ventricular endocardial tracing and TV curves, green; left ventricular endocardial tracing and TV curves, yellow.

necessarily due to a specific myopathic process of the

RV myocardium.5 Given a normal pericardium, the LV
is estimated to contribute between 20% and 60% of the
function of the RV.6 Despite this knowledge, the ventricular
septum has been relatively ignored as a biventricular
muscle region. It remains unknown how best to include
this region in calculation of LV and RV function.
Some experts advocate measuring RV and LV volumes
at individual time-points of the cardiac cycle to identify the
maximal diastolic volumes, while others ignore the extreme
interventricular dependence and measure ventricular
volumes when the septum is at the midline. Results will
often be strikingly different and these differences in the
timing of minimum and maximum ventricular volumes
are particularly evident in disease states with disturbed
electrical conduction as commonly seen in left bundle
branch block or repaired TOF (Fig. 53.3). Therefore, for
serial investigations, research studies, or just clinical
consistency, these authors recommend selecting a lab
preference and being consistent. To reiterate, these authors
believe that interstudy and testretest reproducibility is
most important and, given the lack of a true gold standard,
outweighs methods aimed entirely at accuracy.
The evaluation of regional RV wall motion must
take into consideration the normal variable contraction
patterns near the moderator, parietal, and septomarginal

Echocardiography is and will likely remain a first-line

diagnostic imaging modality for evaluating the RV
structure and function because of its wide-spread availa
bility and the fact that it is a noninvasive, rapid, and
portable tool. It provides a comprehensive approach
to assess patients with suspected right heart disease.
Accurate evaluation of RV morphology and function
requires integration of multiple echocardiographic views,
including parasternal long- and short-axis, RV inflow,
apical (RV modified) four-chamber, and subcostal views.10
Although multiple quantitative methods for RV
assessment are provided, the routine assessment of RV
structure and function is mostly qualitative or semiqua
ntitative in clinical practice (Fig. 53.4). Nor
mally, the
cardiac apex is formed by the LV, but when significantly
dilated, the RV is apex-forming. Methods commonly
used to calculate the LV volume may be used to calculate
RV volumes, but are less accurate due to the complex
geometry. Due to these inherent limitations, a number of
geometry-independent parameters have been proposed.
Recently published guidelines on the echo evaluation
of the right heart recognize that there are limitations in
available published normal references and therefore, most
categories are reported as normal or abnormal, rather
than mild, moderate, or severe disease, which is common
in reporting the left heart.11

M-MODE ECHOCARDIOGRAPHY
The myofibrillar arrangement of the RV consists of mainly
subepicardial circumferential fibers and subendocardial
longitudinal fibers in the inflow region and both
subepicardial and subendocardial longitudinal fibers in
the outflow region. The majority of the RV myocardium

Chapter 53: Evaluation of the Right Ventricle

1137

Fig. 53.4: Echo windows for right ventricle (RV) assessment. Graphical illustration of the 11 recommended two-dimensional (2D) echocardiographic images from a transthoracic approach highlighting the parasternal long, parasternal short, apical, and subcostal views to
obtain a comprehensive assessment of RV size and function. For additional details, see Rudski LG, et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography. J Am Soc Echocardiogr.
2010;23:685713.

lacks the middle circumferential myofiber array that is

dominant in the LV and consequently is much more
dependent on longitudinal shortening for global ejection
than the LV.12 The longitudinal RV contraction at the base
is important in understanding and estimating RV function.
Because the predominant RV fractional shortening is
significantly greater longitudinally than circumferentially,
an evaluation of longitudinal shortening provides a rela
tively simple and reliable estimate of global RV function.
The total tricuspid annular descent or tricuspid annular
plane systolic excursion (TAPSE) is an important marker
of RV global systolic function (Fig. 53.5). Combining the
findings from 46 studies investigating this value (N = 2320),
the normal range can be reported as 2224 mm (95% CI
1531 mm).11 The TAPSE can be derived from M-mode
analysis of the lateral tricuspid annular ring or from

Doppler tissue imaging (DTI) color display. Interestingly,

for such a simple marker of RV function, the correlation
between TAPSE and right ventricular ejection fraction
(RVEF) by CMR was superior to first pass radionuclide
techniques and three-dimensional (3D) echo estimates
of RVEF in a single-center investigation of a population of
patients with ischemia or pulmonary hypertension.13
However, this population had a relatively narrow RVEF
range (58% 3%), there was significant variation in results,
and despite having the best correlation coefficient, it was
not high (r = 0.48). It is possible that the simplicity and
relative reproducibility of this displacement parameter
partially compensates for the single-dimensional nature
that lowers the accuracy when regional RV dysfunction
is present. Other clinical investigators studying more
variable patient populations have found this real-world

1138

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 53.5: Two different patients two-dimensional (2D) echocardiograms, apical four-chamber orientation (top row), diastolic (left)
and systolic (right) frames, and associated M-mode from the right
ventricle (RV) tricuspid annulus (bottom row). The patient displayed on the left has a normal RV and the patient displayed on
the right has marked RV dilation and dysfunction. Arrows represent the systolic excursion of the tricuspid annular plane systolic
excursion (TAPSE).

clinical value of TAPSE to remain when compared with

3D techniques (r = 0.64).14 Another potential consid
eration might be that the TAPSE parameter, being
single-dimensional and limited to the RV myocardial
performance, at times is superior to 3D parameters. Since
3D methods will invariably include the curved ventricular
septum, it is possible that the 3D data may contaminate
the actual RV performance by including a component
of LV contractile function. This is suggested by recent
investigations of CMR parameters of RV displacement
using a tagging sequence that approximates TAPSE.15 In
this study, the investigators used CMR to demonstrate
that the simple (and semiautomated) single-dimensional
marker of RV function out-performed the 3D traditional
parameter of RVEF (Fig. 53.6). This is an area of intense
investigation in the hope of creating a potential automated
and reliable CMR-based tool for quantifying RV function.16

TWO-DIMENSIONAL
ECHOCARDIOGRAPHY
The RV is typically smaller than the LV when normal, and
normally viewed in the apical four-chamber view using
2D echo (2DE). However, it may be difficult to confirm
the optimal alignment of these ventricles. Therefore, using
relative dimensions as the sole criterion to diagnose RV

Fig. 53.6: Graphical comparison of tricuspid annular plane systolic

excursion (TAPSE) and RVEF as measured by cardiac magnetic
resonance imaging in normal controls, repaired tetralogy of Fallot, and patients with atrial septal defect and pulmonary hypertension. These authors found a greater ability of TAPSE over RVEF
measures to separate right ventricle (RV) volume overload and
RV pressure overload clinical syndromes. The y-axis is both percentage (for RVEF; red columns) and distance in millimeters (for
TAPSE; blue columns). By multiplying the TAPSE value by 2.19,
the TAPSE result is converted to a value that equals the normal
controls RVEF (green columns) and further demonstrates the
greater ability of TAPSE compared to RVEF to demonstrate statistical differences (P < 0.05). (ASD: Atrial septal defect; ASD + PHT:
Atrial septal defect and pulmonary hypertension; TOF: repaired
tetralogy of Fallot).

dilation is subject to significant potential error. Despite

attempts to obtain orthogonal RV images necessary for
volume calculations, most commonly using the apical
four-chamber and subcostal views, it remains difficult
to validate that they are orthogonal.17 Image acquisition
should obtain the maximal diameter of the tricuspid valve
annulus to ensure appropriate relative alignment and
avoid cutting through the LV in a noncenter trajectory
(Fig. 53.7).
The RVOT is composed of a preponderance of circum
ferential myofibers and carefully evaluating the motion in
this region provides an estimate of global RV function. The
RVOT fractional shortening (RVFS%) can be calculated as
the percentage of the RVOT diastolic diameter minus the
systolic diameter divided by the diastolic diameter. Either
2DE or M-mode echocardiography of the basal parasternal
short-axis view at the level of the aortic root can be used and
has been shown to correlate with TAPSE.18 Importantly, it
closely correlates with other physiological events, such as
the shortened pulmonary acceleration time recorded at
the cusp level in patients with pulmonary hypertension.

Chapter 53: Evaluation of the Right Ventricle

Fig. 53.7: Two-dimensional (2D) echocardiogram, short-axis orientation, midventricular position (left), and accompanying schematic
(inset). Images on the right represent the apical four-chamber
cut-planes and accompanying schematic (inset). When aligned
correctly through the midcavity of the left ventricle (LV: Solid white
line), the LV/right ventricle (RV) ratio is > 1.5:1. When aligned
incorrectly superior (dashed yellow line) or inferior (dashed pink
line), this normal ratio may change and the normal RV may inadvertently appear relatively dilated. Although these lines are graphically displayed as parallel, in actual clinical practice these arise
from the same transducer location point and are more divergent.

Global, systolic RV function can also be simply assessed

quantitatively using 2DE as a percentage of change in the
RV cavity area from end-diastole to end-systole in the
apical four-chamber view. End-diastole is identified by the
onset of the R-wave, whereas end-systole is regarded as the
smallest RV cavity just before the tricuspid valve opening.
Endocardial borders of the RV free wall and septum
are traced from base to apex and the RV fractional area
change (RV FAC) is defined using the following formula:
(end-diastolic area end-systolic area)/(end-diastolic area)
100). Heavy RV trabeculation may render border tracing
difficult and requires good image quality for accuracy.
This technique incorporates the RV inflow tract and the
apex but excludes the RVOT and may overestimate RV
function if focal regional dysfunction (e.g. after surgical
repair of TOF) exists in this region. Intravenous contrast
agents designed for the LV may assist in image quality of
the RV and may unmask RV thrombus (Fig. 53.8; Movie
clip 53.6A). The percentage of RV FAC is a relatively
simple parameter that is a surrogate marker of the RVEF
and correlates well with CMR-derived RVEF (r = 0.80;
Fig. 53.9).19

1139

Fig. 53.8: See Movie clip 53.6A. Contrast-enhanced two-dimensional echocardiogram, zoom apical four-chamber orientation,
focused on the right ventricle (RV) apex. The center dark line represents the ventricular septum (myocardium). The unenhanced,
well-circumscribed, 2.0 cm 1.5 cm filling defect in the RV apex
represents a large RV thrombus. The dark region toward the base
of the RV cavity represents attenuation artifact from the dense
manufactured contrast agent.

DOPPLER ECHOCARDIOGRAPHY
Conventional Doppler
Importantly, all conventional Doppler techniques are
subject to increased error as the quality of the spectral
Doppler signal worsens and therefore, special care
should be taken during image acquisition. The rate of RV
pressure increase is derived from the continuous wave
Doppler (CWD) spectral display of the TR signal. The time
interval (dt) necessary to increase the TR velocity from
baseline to 2.0 m/s represents a change in pressure (dP) of
16 mm Hg. When the TR velocity is elevated, time intervals
from 1.0 m/s to 2.5 or 3.0 m/s (dP = 21 mm Hg and 32 mm
Hg, respectively) can alternatively be obtained (similar to
LV dP/dt estimates) and reduce error from using very low
velocities. The dP/dt value is considered normal when >400
mm Hg/s (Fig. 53.10).20 Since the dP/dt is dependent on
preload, the maximal TR velocity (TRmax) can be included
in the equation (dP/dt/TRmax) and partially compensate
for this.21 In patients with predominant RV failure, the TRderived dP/dt/TRmax, but not dP/dt alone, was shown to
be a clinically useful index of global RV contractility.

1140

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 53.9: Two-dimensional (2D) echocardiogram, apical fourchamber orientation, diastolic (left) and systolic frame (right).
The right ventricle (RV) endocardial border has been traced for
estimating the volumes (and calculating the fractional area change
[FAC]) using the Simpsons method of discs. In this patient with
severe global RV systolic dysfunction, the FAC was 13%. (FAC:
Fractional area change; RVd: RV diastolic volume; RVs: RV
systolic volume).

The pulsed wave Doppler (PWD) spectral display of

the RV inflow can be used as an estimate of RV diastolic
function. Similar to LV inflow patterns, peak early velocity
(E-wave) and its deceleration time, late velocity during
atrial contraction (A-wave) and its duration, are parameters
that reflect right-heart pathology. The hepatic and vena
cava PWD patterns also reflect RV hemodynamics and
increased right-sided heart filling pressures lead to
increased flow reversal in the hepatic vein (HV > 20%) or
superior vena cava (SVC > 10%) during apnea. Increased
flow reversals in response to inspiration or expiration
can be seen in patients with restrictive or constrictive
cardiomyopathies, respectively.22,23

Tissue Doppler
The pulmonary circulation normally has a low vascular
resistance, and consequently, a very short (or unde
tectable) isovolumic contraction time (IVCT) and isovo
lumic relaxation time (IVRT). The superficial circum
ferential fibers contract during the IVCT and the deeper
longitudinal fibers contract during ejection. The onset of
RV ejection at the outflow tract is delayed after the onset of
contraction of the inflow tract. This regional RV contractility
requires high temporal resolution to be recognized and
provides a basis for color mapping of the myocardium with
advanced tissue Doppler or speckle tracking techniques.

Fig. 53.10: Top Left: Two-dimensional (2D) echocardiogram,

short-axis orientation, midventricular level demonstrating a markedly dilated right ventricle (RV), concave septum toward the
left ventricle (LV), and RVH in a patient with severe pulmonary
hypertension and a reduced RV dP/dt of 320 mm Hg/s. Bottom
left: Continuous wave Doppler spectral display of the tricuspid
regurgitation (TR) signal confirming the elevated TR maximal
velocity (> 5 m/s; >100 mm Hg). Right panel: Zoom image of the
TR spectral continuous wave Doppler (CWD) display used to estimate the dP/dt. White arrow = TR flow at 1 m/s (4 mm Hg); Black
arrow = TR flow at 3 m/s (36 mm Hg); P = estimated change in
pressure from 1 to 3 m/s; t = measured time from 1 m/s to 3 m/s;
TRmax = maximal TR velocity.

Tissue Doppler can be used to record the peak

systolic velocity of the tricuspid annulus (S'). In healthy
individuals, the lower normal limit at the basal RV lateral
wall is 14 2 cm/s for DTI spectral displays and 10 2
cm/s for DTI color displays. This velocity has been shown
to correlate more closely with CMR-derived RVEF than
the 2D fractional area change (FAC), DTI-derived tissue
displacement, systolic strain, and strain rate.24 An S' < 9.5
cm/s identifies patients with an RVEF < 40%.25 Thresholds
of > 12, 129, and < 9 cm/s allow differentiation between
normal (> 55%), moderately reduced (3055%), and
severely reduced (< 30%) RVEF, respectively.26
Myocardial velocity of the RV free wall as measured
by DTI during the IVCT phase (IVCv) has also been used
to estimate RV contractility (Fig. 53.11). Although this
parameter appears more sensitive to loading conditions
than myocardial acceleration and other listed parameters,
this parameter demonstrated the ability to predict
outcomes in patients with pulmonary artery hypertension
(PAH).27 In 142 patients with PAH, the 6-minute walk test
( 400 M) and IVCv ( 9 cm/s) were the only clinical or
echo parameters that predicted mortality. Myocardial
acceleration during the earliest phase of IVCT is a novel

Chapter 53: Evaluation of the Right Ventricle

1141

Fig. 53.11: Two-dimensional (2D) echocardiogram, apical fourchamber orientation, tissue Doppler color map display of mean
myocardial displacement (derivative of velocity/time). The arrow
points to the basal right ventricle (RV) myocardial region, which
we have found should remain purple ( 12 mm) for approximately
50% of the length of the RV free wall in normal individuals. This
is a quick semiquantitative tool that appears to correlate with
tricuspid annular plane systolic excursion (TAPSE).

Fig. 53.12: Illustration of tissue Doppler spectral display of the

right ventricle (RV) tricuspid annulus highlighting the RV myocardial movements and representative measurements during the
entire cardiac cycle. Inset: Actual tissue Doppler spectral display
of the RV tricuspid annulus in a patient. (Ea: Early diastolic velocity of the RV annulus that occurs during early RV filling; Aa: Late
diastolic velocity of the RV annulus that occurs during the atrial
contraction; Sa: Systolic velocity of the RV annulus that occurs
during RV systolic contraction; IVCT: Isovolumic contraction time;
IVRT: Isovolumic relaxation time; IVV: Isovolumic velocity;
At: Time to reach maximal IVV.

index for the assessment of RV contractile function that is

less affected by preload and afterload changes.28 This index
is calculated by dividing myocardial velocity during IVCT
by the time interval from the onset of this wave to the time
at peak velocity.
The RV index of myocardial performance (RIMP; or Tei
index) is defined as the sum of the IVCT and IVRT divided
by the ejection time and is increased in either systolic or
diastolic RV dysfunction.29 This parameter is relatively
simple to obtain with high quality tissue Doppler or
conventional PWD, is a marker of early disease in cardiac
amyloidosis, and predicts symptoms in hypertrophic
cardiomyopathy (Figs 53.12 and 53.13).30 A value < 0.25
predicts an RVEF 0.50 (sensitivity 70%, specificity 89%)
and 0.40 predicts an RVEF < 35% (81%, 85%).31

(near the apex) myofibers.4 Unlike tissue Doppler analysis

which is subject to error from cursor angle misalignment,
2D strain (speckle tracking) is angle-independent,
allowing for the evaluation of regional function in all
myocardial segments (including the apex). Ultrasound of
the myocardium has natural small variations in decibels
(speckles) that are inherent to the characterization of the
RV wall and can be tracked throughout the cardiac cycle.
These provide a detailed regional determination of frameto-frame myocardial deformation. Peak systolic strain
and strain rate, particularly of the basal RV free wall, are
significantly impaired in patients with pulmonary arterial
hypertension and have been used as an index of global RV
function.32 The longitudinal RV strain and strain rate values
are higher and more inhomogeneous than values reported
for the LV. Longitudinal strain and strain rate values are
lowest in the RV base and increase toward the RV apex.
Strain rate imaging is independent of overall motion.
This technique has significant potential as the initial and
serial diagnostic tool to assess patients with known or
suspected RV pathology, and correlates with invasive
and noninvasive reference standards of RV performance
(Figs 53.14A and B).33,34 Moreover, strain rates are much

TWO-DIMENSIONAL STRAIN
(SPECKLE TRACKING)
The RV myocardium is normally only 34
Within this thin layer of myocardium resides
arrangement of circumferential (parallel to
valve (AV) groove and encircling the RVOT)

mm thin.
a complex
the aortic
and spiral

1142

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 53.13: Pulsed wave Doppler (PWD) spectral display of the

right ventricle (RV) inflow (upper left insert) and RV outflow (lower
right insert) and an illustrative drawing of these Doppler displays
demonstrating an alternative method from Figure 53.12 to
calculate the Tei index. (E: RV inflow early diastolic wave; A: RV
inflow late (atrial) diastolic wave; TRd: Tricuspid regurgitation
duration; ET: RV ejection time; RVOT: Right ventricular outflow tract
PWD flow; IVCT: Isovolumic contraction time; IVRT: Isovolumic
relaxation time; IMP: Index of myocardial performance.

Figs 53.14A and B: Two-dimensional (2D) echocardiogram, apical four-chamber orientation, angulated slightly rightward to visualize
the entire right ventricle (RV) apex with 2D strain color map (speckle tracking) display of the RV myocardium. A. Diastolic frame (arrow =
apical variant hypertrophic cardiomyopathy); B. Systolic frame (arrow = abnormal distal ventricular septum due to adjacent pathological
LV myocardium). For additional details, see Abdy NA, et al. Apical Hypertrophic Cardiomyopathy in an Adolescent. Congen Heart Dis.
2010;5(2):18287.

less load-dependent than strains, volumes, or ejection

fraction, which is particularly important in the RV, where
preload varies significantly with respiration.35
Recently, velocity vector imaging of the RV (longitu
dinal strain), was used to predict RV failure after left
ventricular assist device placement. In this report, a
peak strain cutoff of 9.6% predicted RV failure with 76%
specificity and 68% sensitivity.36

THREE-DIMENSIONAL
ECHOCARDIOGRAPHY
In the absence of cardiac shunting (or significant atrioven
tricular valve regurgitation), the LV and RV have the same

stroke volume, but the upper limit of normal RV volume

is greater than the LV. This explains why the lower limit
of normal RVEF is lower than the left ventricular ejection
fraction (LVEF; e.g. RV in diastole, 100 mL; RV in systole, 55
mL; RV stroke volume, 45 mL; RVEF = 45/100 = 45%; LV in
diastole, 90 mL; LV in systole, 45 mL; LV stroke volume, 45
mL; LVEF = 45/90 = 50%).
Three-dimensional echocardiography analysis of the
RV has recently been reported as a means to eliminate
the geometric intricacies of the RV. Real time 3D echo
(RT3DE) has recently become a reliable, reproducible
tool to measure the LV. Although less well reported, this
technique provides an evaluation of the RV independent
of geometric assumptions.37 The RV volumes and RVEF are

Chapter 53: Evaluation of the Right Ventricle

1143

Table 53.1: Normal Right Ventricular Volume/Body Surface Area

Volume

CMR (mL/m2)

RT3DE (mL/m2)

Mean (SD)

Mean (SD)

EDV:
All

71

71.3 (12.9)

70.0 (12.9)

Male

36

67.1 (12.1)

56.4 (13.4)

Female

35

75.6 (12.4)

74.7 (13.0)

All

71

33.5 (9.9)

33.4 (10.3)

Male

36

28.6 (8.1)

29.2 (10.7)

35

38.4 (9.1)

37.8 (7.4)

ESV:

Female

RVEF:

All

71

53.3 (8.7)

52.6 (9.9)

Male

36

57.5 (7.0)

56.2 (9.1)

Female

35

49.0 (8.8)

48.9 (9.5)

Normal right ventricular volumes (indexed to body surface area) as measured with CMR and RT3DE categorized by gender to enddiastolic and end-systolic volumes and the resulting RVEF. Table modified from Reference 38. For additional details, see Gopal AS,
et al. Normal values of right ventricular size and function by real time three-dimensional echocardiography: comparison to cardiac
magnetic resonance imaging. J Am Soc Echocardiogr. 2007;20:44555.
(CMR: Cardiac magnetic resonance imaging; EDV: End-diastolic volume; ESV: End-systolic volume; RT3DE: Real time three-dimensinal echocardiography; RVEF: Right ventricular ejection fraction).

determined by manual tracing of the endocardial borders

and require adequate image quality for this purpose.
However, due to the fact that the entire RV is acquired in
a single pyramidal data set, any acoustic window may be
used and this increases the likelihood that an adequate
image is obtained. Head-to-head comparison of 3D
techniques to 2D techniques consistently demonstrate
larger volumes and closer agreement as well as higher
reproducibility relative to CMR. Normal RT3DE values of
the RV size and function have been reported (Table 53.1).38
The technique of RT3DE has been validated in phantoms,
animals studies, adults with acquired RV pathology, and
children with congenital heart diseases.39

TRANSESOPHAGEAL
ECHOCARDIOGRAPHY
When the transthoracic ultrasound window is suboptimal
and CMR is not available, transesophageal echo (TEE)
may be performed. Most reports on TEE evaluation of
the RV are from intraoperative studies and may not be
as clinically relevant due to the frequent administration
of inotropic medications and rapid fluid shifts in this
population. In a study of 25 children operated on for ASDs,
90% had adequate 3D TEE studies.40 RV volumes with this

technique matched the direct surgical measures (r2 = 0.99)

obtained by injecting saline solution through the tricuspid
valve using a graduated syringe. In the clinical setting of
tricuspid or pulmonic valve pathology, TEE is a valuable
complementary diagnostic tool.

HEMODYNAMICS
Although not a direct estimate of RV volume or function,
the evaluation of right heart hemodynamics is exceedingly
valuable when right heart pathology is suspected. The
right atrial pressure (RAP) is readily estimated from
the inferior vena cava (IVC) dynamics and the caval
and HV flows. More recently, DTI has also been used to
evaluate this parameter. Classically, a dilated IVC, lack of
inspiratory collapse, E/e' ratio > 6, atrial septal leftward
bulge, predominant diastolic flow patterns in the SVC,
or HVs suggest an elevated RAP. However, these features
may be normal in athletes, obese individuals, congenital
narrowing of the IVCRA junction (BuddChiari), cor
triatriatum dexter, or mechanical ventilation. Importantly,
IVC imaging should be performed in the supine (not left
lateral decubitus) position. For specific RAP values, it is
somewhat reliable to use 020 mm Hg range at 5 mm Hg
intervals. If the IVC size is < 21 mm and the inspiratory

1144

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

RAP or LV systolic pressure (cuff pressure in absence of

outflow gradients), and provide an avenue to noninvasively
estimate intracardiac pressures.44

OTHER IMAGING MODALITIES

Fig. 53.15: See Movie clip 53.9. Cardiac magnetic resonance

imaging, diastolic frame from a cine image, four-chamber long-axis
orientation, demonstrating the clarity of the right ventricle (RV) that
is expected despite body habitus or lung disease that often limits
the visualization of echocardiography. Chamber size and ejection
fraction can be measured by obtaining multiple slices that covers
the entire RV and manually tracing the endocardial borders.

narrowing is > 50%, the RAP is 05 mm Hg. Use 5 mm Hg if

any other signs of elevated RAP exist. If either one of these
two parameters is abnormal, use 510 mm Hg (10 mm Hg
if other signs exist). If both size and respiratory variation
are abnormal, use 15 mm Hg (or 20 mm Hg if IVC plethora
exists). The dynamic inspiratory change > 50% is more
important than the IVC size.41
In mechanically ventilated individuals, more complex
assessment is necessary to estimate RAP, such as the HV
systolic filling fraction (HVFF = VsVTI/VdVTI) < 55%,
which has a sensitivity and specificity of 86% and 90%,
respectively, for diagnosing RAP > 8 mm Hg.42 [VsVTI:
Systolic (hepatic) venous velocity time integral; VdVTI:
Diastolic (hepatic) venous velocity time integral]. Another
formula successfully used in mechanically ventilated
patients incorporates the tricuspid inflow and DTI of the
tricuspid annulus: 1.62 E/e' + 2.13 = RAP (r = 0.7).43 This
was assessed in patients with recent cardiac surgery.
When apnea cannot be performed for these right-sided
measurements, averaging multiple Doppler velocities
appears to be an adequate alternative.
Careful scrutiny of the transvalvular (when regur
gitation exists) or trans-septal (when shunts exist)
gradients provide the means to determine pressure
gradients across cardiac chambers. These gradients are
then added or subtracted to known pressures, such as the

Extensive review of multimodal imaging of the RV is

available elsewhere, but a brief update on the capabilities of
CMR and computed tomography is worthwhile to provide
the reader with an understanding of these contemporary
diagnostic imaging capabilities.45,46 The development of
CMR has advanced significantly over the past two decades.
Since CMR is not limited by the ultrasound acquisition
window, the entire RV can be easily visualized (Fig. 53.15
and Movie clip 53.9). This allows qualitative assessment
of right ventricular wall motion and reliable quantitative
assessment of chamber size, mass, and ejection fraction,
and is considered the reference standard when assessing
new echo techniques.47 These properties have made
CMR a valuable tool in the assessment of primary RV
cardiomyopathies and cor pulmonale.48,49 CMR can also
be used to evaluate vascular anatomy and quantify blood
flow and is an important tool in the evaluation of patients
with complex congenital heart disease.50,51 Despite being
considered a reference standard due to the excellent
interstudy reproducibility and lack of a gold standard,
highly skilled technologists and interpreting physicians are
as necessary for CMR studies as they are for echo studies.
A vital aspect of CMR is the ability to evaluate tissue
characteristics. RV infarction can be easily detected with
late gadolinium contrast-enhancement imaging.52,53
This CMR sequence has also been demonstrated to be a
reliable way to assess thrombus, not uncommonly found
in the RV.5456 Yet another important strength of CMR for
quantification of RV function is the ability to quantify
regional and global RV mechanics (such as strains
and strain rates) using techniques such as myocardial
tagging.57 However, due to the thin walls of the RV, tagging
methods are mostly limited to assessment of longitudinal
RV deformation. However, newer MRI techniques such as
cine displacement encoding with stimulated echoes
(DENSE) show promise in their ability to acquire full
3D data sets for quantification of circumferential and
longitudinal strain in the RV from a single data set.9
Despite these strengths, CMR is still mostly limited
to patients without pacemakers or defibrillators and
not severely claustrophobic, although it is becoming
increasingly recognized that CMR is safe in patients with

Chapter 53: Evaluation of the Right Ventricle

Fig. 53.16: See Movie clip 53.10. Cardiac CTA displayed in the
four-chamber orientation demonstrating the clearly defined border
between the brighter blood pool and the darker right ventricular
myocardium.

these devices.58 Even when CMR is proven to be entirely

safe in patients with pacemakers, the RV lead will create
havoc for optimal imaging of the RV by producing a
ferromagnetic artifact, limiting optimal visualization
of the RV wall near the lead. Gadolinium contrast use is
limited to patients without severe renal insufficiency
[Glomerular filtration rate (GFR) > 30 mL/min/1.73 m2]
due to the increased risk of nephrogenic systemic fibrosis.
Optimal imaging is achieved when patients are in normal
sinus rhythm and able to breath-hold for 715 seconds,
which may not be possible in patients with significant
right heart pathology. Real time or single-shot imaging
can be performed in these patients in much shorter times
or during free breathing but at a cost of reduced spatial
resolution. Similar to echocardiography, it is important
to have experienced specialists acquire high quality,
comprehensive images to maximize the clinical value of
the study.
Cardiovascular computed tomography (CCT) can
also be valuable in the assessment of the RV. With high
spatial resolution (approximately 0.5 mm), the right
ventricular anatomy, chamber size, and systolic function
can be accurately determined (Fig. 53.16 and Movie clip
53.10).59 Since CCT obtains a volumetric data set with
isotropic voxels, images can be reoriented in any plane
for postacquisition evaluation. In patients with congenital
heart disease, who are unable to undergo CMR, CCT offers
an alternative noninvasive imaging option.60
Since blood has similar signal intensity as myocardium
on noncontrast CCT, injection of contrast is necessary to

1145

differentiate the myocardium from blood. Depending

on the area of interest, the site of contrast injection
(intravenous line placement), contrast injection rate
and volume, and timing of the scan need to be carefully
considered to optimize opacification of the vascular
structures or cardiac chamber of interest. This is especially
important in the evaluation of the RV since intravenous
contrast is injected via a peripheral vein, and is mixed with
noncontrast blood from other peripheral veins (e.g. IVC
flow) prior to entering the RV. This can lead to incomplete
contrast/blood mixing and limit the optimal evaluation.
Despite the high spatial resolution that can be acquired
with CCT, this technique is limited by low temporal
resolution (> 80 ms), need for ionizing radiation, and
nephrotoxic contrast agents.
Lastly, invasive RV angiography could be considered
the reference standard, but like all imaging modalities
require specialized skills at acquiring adequate orientation
and contrast filling of the RV. Some investigators have had
success at creating quantitative off-line spline models of
regional contraction patterns to help separate normal
from abnormal motion patterns.61

CONCLUSION
Continued investigation is warranted to improve our
understanding of the RV and to obtain robust noninvasive
diagnostic methods to assess this chamber, which
continues to be proven to predict clinical outcomes.
Evolving clinical settings demonstrate the importance of
evaluating the RV in an effort to predict RV failure such as
prior to left ventricular assist devices. Given the normally
complex shape, the further distortion with pathology, and
the intricate myofibrillar arrangement, it is likely that a
combination of parameters (possibly imaging and clinical)
will need to be used for optimal RV assessment rather than
a single diagnostic parameter. It may also be necessary to
combine diagnostic imaging tools, but for the foreseeable
future, echo will be the initial technique for this purpose.

DISCLOSURE
Gadolinium contrast is not Food and Drug Administration
(FDA) approved for CMR.

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1146

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

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18. Lindqvist P, Henein M, Kazzam E. Right ventricular
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22. Klein AL, Hatle LK, Burstow DJ, et al. Comprehensive
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23. Appleton CP, Jensen JL, Hatle LK, et al. Doppler evaluation
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24. Wang J, Prakasa K, Bomma C, et al. Comparison of novel
echocardiographic parameters of right ventricular function
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25. De Castro S, Cavarretta E, Milan A, et al. Usefulness
of tricuspid annular velocity in identifying global
RV dysfunction in patients with primary pulmonary
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ventricular ejection fraction. Echocardiography. 2008;25(3):
28993.
26. Tller D, Steiner M, Wahl A, et al. Systolic right ventricular
function assessment by pulsed wave tissue Doppler imaging
of the tricuspid annulus. Swiss Med Wkly. 2005;135(31
32):4618.
27. Ernande L, Cottin V, Leroux PY, et al. Right isovolumic
contraction velocity predicts survival in pulmonary
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28. Vogel M, Schmidt MR, Kristiansen SB, et al. Validation of
myocardial acceleration during isovolumic contraction as
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29. Kim WH, Otsuji Y, Yuasa T, et al. Evaluation of right vent
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31. Schwerzmann M, Samman AM, Salehian O, et al.
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in adults with repaired tetralogy of fallot. Am J Cardiol.
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32. Pirat B, McCulloch ML, Zoghbi WA. Evaluation of global
and regional right ventricular systolic function in patients
with pulmonary hypertension using a novel speckle
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33. Teske AJ, De Boeck BW, Olimulder M, et al. Echocar
diographic assessment of regional right ventricular function:
a head-to-head comparison between 2-dimensional and
tissue Doppler-derived strain analysis. J Am Soc Echo
cardiogr. 2008;21(3):27583.
34. Abdy NA, Valdes SO, Sorrell VL, et al. Apical hypertrophic
cardiomyopathy in an adolescent. Congenit Heart Dis.
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35. Weidemann F, Jamal F, Sutherland GR, et al. Myocardial
function defined by strain rate and strain during alterations
in inotropic states and heart rate. Am J Physiol Heart Circ
Physiol. 2002;283(2):H792H799.
36. Grant AD, Smedira NG, Starling RC, et al. Independent
and incremental role of quantitative right ventricular
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37. Jenkins C, Chan J, Bricknell K, Strudwick M, Marwick
TH. Reproducibility of right ventricular volumes and
ejection fraction using real-time three-dimensional
echocardiography: comparison with cardiac MRI. Chest.
2007;131(6):184451.

38. Gopal AS, Chukwu EO, Iwuchukwu CJ, et al. Normal
values of right ventricular size and function by real-time
3-dimensional echocardiography: comparison with cardiac
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39. Shiota T. 3D echocardiography: evaluation of the right
ventricle. Curr Opin Cardiol. 2009;24(5):4104.
40. Grison A, Maschietto N, Reffo E, et al. Three-dimensional
echocardiographic evaluation of right ventricular volume
and function in pediatric patients: validation of the
technique. J Am Soc Echocardiogr. 2007;20(8):9219.

41. Kircher BJ, Himelman RB, Schiller NB. Noninvasive
estimation of right atrial pressure from the inspiratory
collapse of the inferior vena cava. Am J Cardiol.
1990;66(4):4936.
42. Nagueh SF, Kopelen HA, Zoghbi WA. Relation of mean
right atrial pressure to echocardiographic and Doppler
parameters of right atrial and right ventricular function.
Circulation. 1996;93(6):11609.
43. Sade LE, Gulmez O, Eroglu S, et al. Noninvasive estimation
of right ventricular filling pressure by ratio of early tricuspid
inflow to annular diastolic velocity in patients with and
without recent cardiac surgery. J Am Soc Echocardiogr.
2007;20(8):9828.

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44. Sorrell VL, Reeves WC. Noninvasive right and left heart
catheterization: taking the echo lab beyond an image-only
laboratory. Echocardiography. 2001;18(1):3141.

45. Selton-Suty C, Juillire Y. Non-invasive investigations
of the right heart: how and why? Arch Cardiovasc Dis.
2009;102(3):21932.
46. Sorrell VL, Indik JI, Marcus FI. Right ventricular cardio
myopathies. Section III. Chapter 19. Cardiovascular
multimodal imaging in key clinical problems. In: Pahlm
O, Wagner G, editors. Multimodal Cardiovascular Imaging:
Principles and Clinical Applications. McGraw-Hill; 2011.

47. Grothues F, Moon JC, Bellenger NG, et al. Interstudy
reproducibility of right ventricular volumes, function, and
mass with cardiovascular magnetic resonance. Am Heart
J. 2004;147(2):21823.

48. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis
of arrhythmogenic right ventricular cardiomyopathy/
dysplasia: proposed modification of the task force criteria.
Circulation. 2010;121(13):153341.
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magnetic resonance in pulmonary hypertension.
J Cardiovasc Magn Reson. 2012;14:6.
50. Warnes CA, Williams RG, Bashore TM, et al.; American
College of Cardiology; American Heart Association Task
Force on Practice Guidelines (Writing Committee to Develop
Guidelines on the Management of Adults with Congenital
Heart Disease); American Society of Echo
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Heart Rhythm Society; International Society for Adult
Congenital Heart Disease; Society for Cardiovascular
Angiography and Interventions; Society of Thoracic
Surgeons. ACC/AHA 2008 guidelines for the management
of adults with congenital heart disease: a report of the
American College of Cardiology/American Heart Association
Task Force on Practice Guidelines (Writing Committee to
Develop Guidelines on the Management of Adults with
Congenital Heart Disease). Developed in Collaboration
with the American Society of Echocardiography, Heart
Rhythm Society, International Society for Adult Congenital
Heart Disease, Society for Cardiovascular Angiography and
Interventions, and Society of Thoracic Surgeons. J Am Coll
Cardiol. 2008;52(23):e143e263.
51. Sorrell VL, Altbach MI, Kudithipudi V, et al. Cardiac MRI
is an important complementary tool to Doppler echocar
diography in the management of patients with pulmonary
regurgitation. Echocardiography. 2007;24(3): 31628.
52. Kumar A, Abdel-Aty H, Kriedemann I, et al. Contrastenhanced cardiovascular magnetic resonance imaging of
right ventricular infarction. J Am Coll Cardiol. 2006;48(10):
196976.
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magnetic resonance imaging in acute coronary syndromes.
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54. Weinsaft JW, Kim RJ, Ross M, et al. Contrast-enhanced
anatomic imaging as compared to contrast-enhanced
tissue characterization for detection of left ventricular
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55. Boukantar M, Lim P, Mitchell-Heggs L. Right ventricular

thrombus and pulmonary embolism in patient with anterior
myocardial infarction. Eur Heart J. 2010;31(23):2870.
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thrombus detection and multimodality imaging using
contrast echocardiography and cardiac magnetic resonance
imaging. Heart Lung Circ. 2012;21(3):1858.
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systolic kinematics of the right ventricle. Am J Physiol
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59. Raman SV, Shah M, McCarthy B, et al. Multi-detector

row cardiac computed tomography accurately quantifies
right and left ventricular size and function compared with
cardiac magnetic resonance. Am Heart J. 2006;151(3):
73644.
60. Huang X, Pu X, Dou R, et al. Assessment of right ventricular
function with 320-slice volume cardiac CT: comparison
with cardiac magnetic resonance imaging. Int J Cardiovasc
Imaging. 2012;28(Suppl 2):8792.
61. Indik JH, Wichter T, Gear K, et al. Quantitative assess
ment of angiographic right ventricular wall motion in
arrhythmogenic right ventricular dysplasia/cardiomy
opathy (ARVD/C). J Cardiovasc Electrophysiol. 2008;19(1):
3945.

CHAPTER 54
Three-Dimensional Echocardiographic
Assessment of LV and RV Function
Aasha S Gopal

Snapshot

3D Quantaon of the Le Ventricle

INTRODUCTION
Significant progress has taken place in the last 25 years
in moving echocardiography from a two-dimensional
(2D) imaging modality to a three-dimensional (3D)
imaging modality that has found several routine clinical
applications, an important one being quantification of
cardiac structure and function. This progress has closely
paralleled the transition in transducer technology from
conventional phased-array transducers to matrix array
transducers. This chapter explores the limitations of
quantifying left and right ventricular (RV) structure and
function by conventional M-mode [one-dimensional
(1D)] and 2D echocardiography (2DE), thereby providing
the rationale for developing and adopting new methods
such as 3D echocardiography (3DE) and speckle tracking
echocardiography (STE).

3D QUANTITATION OF THE
LEFT VENTRICLE
Limitations of 2D Echocardiography
Lack of 3D Spatial Coordinates
Left ventricular ejection fraction (LVEF) is a cardinal
parameter that has been shown to have tremendous
prognostic value in a variety of clinical situations varying
from valvular heart disease, ischemic heart disease

3D Quantaon of the Right Ventricle

to cardiomyopathies. Despite its central importance,

evaluation of left ventricular (LV) structure and function
in routine clinical practice is largely subjective and
substantially relies on an expert knowledge of cardiac
anatomy and physiology. The clinician integrates this
knowledge, views moving 2D cross-sectional images,
and renders an eyeball estimation of LVEF as a first
approximation. Indeed, in the hands of experts, this
method compares quite favorably when compared to many
traditional M-mode and 2D techniques.1 However, there is
substantial interoperator variability and standardization
is difficult. More objective measures of chamber
quantification rely on detection of endocardial borders at
end-diastole and end-systole. Linear dimensions may be
obtained by M-mode echocardiography that has excellent
temporal resolution, or by 2DE. Linear dimensions are
still reported today because of simplicity of use. However,
these dimensions have high testretest variability largely
because of image positioning error, that is, the difficulty
faced by the sonographer to reproduce the same 2D
echocardiographic image with a high degree of fidelity.2
This is because, standard 2D images are prescribed by
the sonographer solely on their basis of their knowledge
of cardiac anatomy and are not based on any external 3D
spatial coordinate system.
An external 3D spatial coordinate system can be
specified by a variety of methods and the very early
prototypes of 3DE systems utilized either an acoustic

1150

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

or magnetic system of emitters and receivers that were

mounted on the transthoracic imaging transducer
(Figs 54.1 and 54.2).35 Using such a system, it was noted
that only 24% of unguided standard images are optimally
positioned within 5 mm and 15 of the ideal position
and 3DE improves positioning of standard images to
80%, a threefold improvement (p < 0.001).2 If the standard
parasternal long-axis view is positioned with a high
degree of variability, measurements obtained from these

Fig. 54.1: Freehand scanning. An external three-dimensional (3D)

spatial coordinate system is provided by an acoustic spatial locater which consists of a system of three sound emitters mounted
on the transducer (shown on the examination bed) which provides
freehand scanning. These emit sound waves that are received by
an overhead microphone array (shown above the examination
bed). The 3D spatial information is fed into a computer (shown
beside the ultrasound machine) which assigns a set of x, y, and z
Cartesian coordinates to each image.

views also exhibit a high degree of variability. A study

showed that the standard unguided 2D examination
was associated with an interobserver variability of 9.1%
for ventricular measurements. Guided 3DE significantly
reduced interobserver variability to 3.1% for the same
measurements (p < 0.005 by McNemars test).6
The lack of 3D spatial coordinates by conventional
echocardiography also means that it is not possible to
relate one cross-sectional image with another crosssectional image without making assumptions related
to image position that is, biplane images are assumed
to be orthogonal to each other but rarely ever satisfy
that assumption because each cross-sectional image
is obtained independently by the sonographer in an
unguided manner.2 An external 3D spatial coordinate
system provides a means of precisely measuring the
relationship of one cross-sectional image with respect
to another, a necessary prerequisite for accurate
quantification of the left ventricle. An external 3D spatial
coordinate system, while providing the ability to scan
freely without constraints has limitations. Limitations of
the freehand acoustical spatial locating system are that it
requires a clear line of sight between the sound emitters
and the overhead receivers. An electromagnetic spatial
locating system may be limited by interference with the
electromagnetic field by large ferromagnetic objects such
as metal examination beds and other nearby metallic
equipment that may degrade system accuracy.7
Another means of relating images to one another is
by providing a spatial coordinate system that is internal
to the heart. This approach is based on the principle that
a 3D data set can be reconstructed from a series of 2D
images in which the intervals and angles between the

Figs 54.2A to C: Freehand scanning. A modified ultrasound probe is tracked in three-dimensional (3D) space using an electromagnetic
field device; images then may be reconstructed offline to create 3D data sets. A schematic of the receiver and transmitting device and
the Cartesian coordinate system for tracking the location of the transducer is shown. (Courtesy of TomTec Imaging Systems, Munich,
Germany; with permission).

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

2D images are defined. In this method, serial 2D images

are obtained using a mechanically driven transducer that
sequentially recorded images at predefined intervals from
a fixed transducer position. The images may be acquired
in a parallel fashion or by pivoting around a fixed axis in a
rotational, fan-like manner. A variety of transthoracic and
transesophageal echocardiographic systems were devised
in which fast rotating transducers provided a series of
cross-sectional images that were all spaced relative to each
other in precise fashion (Fig. 54.3).814

Limitations of 2D Echocardiography
Geometric Assumptions
Since conventional echocardiography does not provide
cross-sectional images that are spatially related to each
other in a precise fashion, it is necessary to make certain
assumptions about ventricular geometry to arrive at a
more objective assessment of LV size and function. Linear
measurements are popular and still reported today.
However, they are difficult to reproduce when acquired
in an unguided fashion and they assume that ventricular
enlargement occurs uniformly and is reflected faithfully in
the increase in the linear dimension. For example, when

1151

the ventricle enlarges along its superiorinferior axis, that

increase is not reflected in the parasternal long-axis view
from which LV dimensions are traditionally reported. To
improve on simple linear dimensions, a plethora of models
of ventricular size and shape have been devised. Popular
among these is a prolate-ellipsoid model that utilizes two
apical views (two-chamber and four-chamber). Here the
ventricle is assumed to conform to the shape of a prolateellipse and ventricular volume is calculated based on traced
endocardial borders from these views.15 However, when
the ventricle is affected particularly by regional ischemic
heart disease, the ventricle may enlarge and remodel in
ways that deviate from a prolate-ellipsoid shape, thereby
posing a severe limitation to accurate quantification of
its size and function. 3DE overcomes these limitations
by eliminating image positioning error and geometric
assumptions (Figs 54.4 and 54.5B).16 3D reconstruction
techniques have been used extensively in vivo to validate
determination of LV volumes and EF against several
reference clinical standards such as cardiac magnetic
resonance imaging (CMR), cineventriculography, as
well as multigated radionuclide scintigraphy using the
transthoracic approach1720 and shown to be superior to
conventional 2DE methods.

Fig. 54.3: Schematic of how three-dimensional (3D) transesophageal echo (TEE) was acquired. The TEE probe is shown with its range
of rotation from 180. Typically, a two-dimensional (2D) image was acquired every 3. The relationship between the heart and the TEE
rotation is shown. (Courtesy of TomTec Imaging Systems, Munch, Germany; with permission.)

1152

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 54.4: Three-dimensional (3D) reconstructionleft ventricular

volume can be computed using a polyhedral surface reconstruction algorithm from a series of images acquired from the base of
the heart to the apex.

Limitations of 2D Echocardiography
Apical Foreshortening and Boundary
Recognition
2DE from the transthoracic approach has additional
limitations that are patient specific and inherent to the
anatomy and position of the heart as it is situated in the
rib cage. Imaging is performed through the interspaces
between the ribs and even though guided 3DE can achieve
perfect image positioning, image quality may be degraded if
there is no optimal transthoracic interspace. Inherent ways
of compensating for the lack of an adequate rib interspace is
to utilize whatever echocardiographic window is available.
This can frequently lead to apical foreshortening as was
nicely demonstrated in a simultaneous echocardiographic
and cineventriculographic study.21 Similarly, it is possible
to obtain only tangential cross-sections of the heart that
may cause inaccuracies in estimating linear dimensions
and wall thickness.

Limitations of 3D Reconstruction
Although 3D reconstruction addresses the principal
limitations to accurate quantitation of the left ventricle,
namely image position error and geometric assumptions,
this methodology has several limitations. The images
acquired for 3D reconstruction are nonsimultaneous.
Therefore, it may be inaccurate in patients with significant
intracardiac dyssynchrony. It also requires electrocardiogram (ECG) gating and is susceptible to inaccuracies due

Figs 54.5A and B: Field of view for two-dimensional (2D) echo

and real time three-dimensional (3D) echo. (A) Tomographic
image field of conventional linear phased-array transducer; (B)
Matrix array transducer used to obtain pyramidal volumetric data
set. Relation of image dissections is shown by orthogonal B-scans
and cross-sectional C-scan.

to patient motion and respiration. Therefore, to obtain a

good 3D data set free from reconstruction artifacts, data
acquisition takes longer and can last up to 5 minutes
depending on the quality of the 2D images, the patients
heart rate, and respiratory pattern. In addition, 2D images
need to be exported to offline workstations and reprocessed
manually using proprietary software. The time taken to
process the images depends on the size of the heart and
the number of images required obtaining an accurate
estimate of LV volume. The greater the number of images,
the less data interpolation is required between images
and the greater the accuracy of the method. However, the
greater the number of images obtained, the greater the
processing time. Typically, four to six images are required
for accurate determination of LV end-diastolic and
end-systolic volumes (ESVs).

Real Time 3DE

Advances in transducer technology have experienced
a revolution due to the increasing computing power of
modern electronics as well as miniaturization. These
developments allowed the first matrix array transducer to
be constructed by von Ramm et al.2224 Here the transducer
elements are arranged in a grid that allows steering in
both the azimuth and elevation directions as compared
to a conventional transducer that allows steering only in
the azimuth direction (Figs 54.5A and B). This seminal
innovation with 16:1 parallel processing allowed imaging
of a volume rather than a sector. Furthermore, real time

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

1153

Figs 54.6A and B: (A) First-generation matrix array transducer: Simultaneous display of parasternal long-axis (orthogonal B scan) and
short-axis views (C-scan); (B) First-generation matrix array transducer: Simultaneous display of apical (orthogonal B scan) and shortaxis views (C-scan). (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

scanning could be performed in a single heartbeat that did

not require ECG gating and allowed simultaneous display
of the images derived from the 3D data set. This firstgeneration matrix array transducer operated at a frequency
of 2.5 MHz and consisted of 256 nonsimultaneous firing
elements that acquired a pyramidal volume data set
measuring 60 60. A limitation of this first-generation
matrix array technology was that the image quality was
relatively poor and did not match conventional 2D image
quality. Additionally, since this was not a fully sampled
matrix but a sparse-array matrix, it did not allow for
3D rendering, but did allow simultaneous orthogonal
B-scans and cross-sectional C-scans to be displayed
(Figs 54.6A and B). Furthermore, large hearts could not be
imaged within the 60 60 volume. Nevertheless, it paved
the way for further advances to be made in matrix array
transducer technology and spurred many commercial
ultrasound manufacturers to develop it further.
Currently, real time 3DE (RT 3DE) is offered on several
commercial platforms. RT 3DE can be performed by either
switching among 2D and 3D transducers, or alternating
between 2D and 3DE modalities present within the same
all-in-one probe.25 3DE is the only imaging method that
is able to view moving structures in the beating heart in
real time. In contrast, cardiac computed tomography (CT)
and magnetic resonance imaging (MRI) provide only 3D
reconstructed images from multiple tomographic planes.
In contrast to the first-generation matrix array transducers,
current systems are fully sampled because they typically
have over 3,000 elements that make up the grid that allows
360 focusing and steering and 3D rendering. Figures
54.7A and B contrast the size of the elements compared

to a human hair within a phased-array transducer

compared to a matrix array transducer.26 Most matrix
array transducers have technology that provides wider
bandwidth with higher sensitivity. This technique now
permits tracking of the endocardial borders in real time
throughout the cardiac cycle. Whereas 3D reconstruction
methods and first-generation matrix array transducers
only permit calculation of static volumes at end-diastole
and end-systole, current matrix array transducers allow us
to tracking ventricular volume over the full cardiac cycle,
thereby allowing us to not only calculate overall EF, which
is a very important measure of cardiac performance, but
also to calculate rates of ejection and rates of filling, which
may be important as well. With further reduction in the
size of electronics, 2D arrays can now be integrated into
a volume that is small enough to fit on a transesophageal
transducer probe, thus allowing for real time 3D scanning
of the heart, thus avoiding image degradation from ribs,
lungs, and fat. As ingenious as real time three-dimensional
transesophageal echocardiography (RT 3DTEE) is, it has
not yet been feasible to perform accurate measurement of
volumes using this technique, primarily due to limitations
of the sector size, frame rate, and finding suitable boundary
tracking algorithms that will perform wall on this complex
data set.
Current RT 3DE systems offer several data acquisition
modes that have varying sector sizes. Data acquisition
is always a trade-off between sector (volume size),
line density (spatial resolution), and volumes/second
(temporal resolution). At present, two different methods
for 3D data acquisition are available: real time 3D
or live 3D mode and multibeat 3D mode. In the real
time or live mode, a thin sector (typically 30 60) of a

1154

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 54.7A and B: (A) Phased-array elements compared to the size of a human hair; (B) Matrix array elements compared to the size
of a human hair.

Fig. 54.8: Full-volume three-dimensional (3D) data set acquired

by a second generation matrix array transducer consisting of four
stitched volumes acquired over four to six heart cycles. The left
hand panel depicts static reference images with the intersecting lines showing the intersection of sagittal and coronal planes.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

Fig. 54.9: Full-volume three-dimensional (3D) data set acquired

by a second generation matrix array transducer consisting of four
stitched volumes acquired over four to six heart cycles. The left
hand panel depicts static reference images with the intersecting
lines showing the intersection of sagittal, coronal and axial planes.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

pyramidal 3D data set is obtained and visualized live, beat

after beat as during 2D scanning. In this mode, narrow
volume, zoom, wide-angle, and color Doppler modalities
are available. Since data acquisition in this mode is done
in a single heartbeat, no ECG or respiratory gating is
required and heart dynamics is shown with instantaneous
online volume rendered reconstruction. This mode also
overcomes limitations posed by rhythm disturbances
and respiratory motion. However, this mode suffers from
relatively poor spatial and temporal resolution.

To provide better spatial and temporal resolution,

multibeat acquisitions can be performed to yield a fullvolume data set. Typically a full-volume data set in secondgeneration 3D echocardiographic systems comprises four
of the small live sectors stitched together to provide a sector
size that is much larger (e.g. 101 104 depending on the
particular commercial system used and the transducer
type). This technique is not real time and therefore requires
ECG gating and is acquired over four to six consecutive
heartbeats (Figs 54.8 and 54.9). The full-volume mode is

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

1155

Figs 54.10A and B: (A) Full-volume three-dimensional (3D) data set acquired by a third-generation matrix array transducer. The data
set is displayed as a set of three image planes that are spaced 60 apart and one cross-sectional axial image; (B) Three equiangular
planes (triplane) that are spaced 60 apart can be generated selectively instead of acquiring a full-volume data set and used for calculation of 3D volumes and ejection fraction. The image quality of triplane imaging is superior to that of full-volume imaging and therefore,
may have some advantages for left ventricular quantitation.

prone to stitching artifacts if the patient moves or breathes

deeply during image acquisition. However, it has the
advantage of imaging a large volume while preserving
high spatial and relatively good temporal resolution
(volume rates). Stitching artifacts may be minimized by
performing data acquisition during suspended respiration.
Arrhythmias such as atrial fibrillation may also produce
stitching artifacts when patients with this arrhythmia are
imaged in the full-volume mode.
Third-generation 3D echocardiographic systems have
even higher processing power with fully sampled 2D
matrix arrays such that a full-volume can be obtained in
a single heartbeat (Movie clip 1A) with little sacrifice in
image quality. A zoom mode is also typically provided.
This is an enlargement of a subsegment of the thin slice live
sector that is ~30 30 that provides even greater spatial
and temporal resolution (Movie clip 1B). An x-plane mode
allows simultaneous viewing of a cross-section and the
plane orthogonal to it. Lateral tilting of this plane is also
possible by manipulating the trackball (Movie clip 2). A
mode that is particularly helpful with LV quantitation is
the triplane mode. Figure 54.10A shows a full-volume
data set that may be obtained for LV quantitation. In this
mode, the left ventricle is tracked by a border-tracking
algorithm using the full 3D data set for the full cardiac
cycle. Using this feature, dynamic LV volumes may be
obtained that are helpful in evaluating not only EF, but
also intracardiac dyssynchrony, as well as ejection rates

and filling rates. However, if only 3D EF is desired, static

volumes in end-diastole and end-systole are sufficient. In
this case, simultaneous viewing of triplane images, which
are derived from the same heartbeat and same 3D data
set but have higher resolution than the full-volume data
set, may be more practical (Fig. 54.10B and Movie clip 3).
Triplane LVEF is computed from static volumes obtained
at end-diastole and end-systole from three equidistant
planes 60 apart. Even though the phantom appears as if it
is contracting dynamically, the only true volumes that are
obtained are from end-diastole and end-systole. Computer
data interpolation is performed for all other points in the
cardiac cycle (Movie clip 3). Triplane volumes tend to
underestimate true volume since they are obtained only
from three images. However, the EFs derived from them
are quite reliable. A further advance in LV quantitation
has been to provide automatic endocardial borders
without the need for any manual operator interaction.
These sophisticated systems were designed by feeding
data generated from thousands of manually endocardial
borders by experts and teaching the computer to recognize
the type of echocardiographic view being presented and
generating an automatic border based on recognizable
features. Examples of apical two-chamber (Movie clip 4)
and four-chamber views (Movie clip 5) being automatically
processed and borders generated for EF calculation
are shown. Current 3D echocardiographic systems
also allow overlay of color information (Movie clip 6),

1156

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

conventional tissue Doppler information (Movie clip 7)

as well as color-coded strain information (Movie clip 8)
that are helpful for evaluating regurgitant jets, intracardiac
dyssynchrony, and wall motion, respectively. For these
purposes, the triplane mode is particularly helpful because
of its higher temporal resolution than the single-beat fullvolume mode.

Validation Studies of Real Time 3DE for

Left Ventricular Volumes and Ejection
Fraction
Extensive validation studies of RT 3DE against independent
reference methods have been performed. Almost all
of them confirm the earlier validation studies with 3D
reconstruction against the same reference standards. Most
show superiority over conventional 2D methods together
with improved intraobserver, interobserver, and test
retest variability.2734 Correlation coefficients against CMR
for end-diastolic volumes (EDVs) have varied from 0.92
to 0.98, and for end-systole from 0.81 to 0.98. Variable systematic underestimation of 3D EDVs from 4 mL to 14 mL
has been reported; underestimation of ESVs from 3 m L to
18 mL has also been reported. However, since both EDVs
and ESVs are underestimated, EF calculations have been
neither systematically overestimated nor underestimated.
Interobserver variability varies from 5% to 11%. Whereas
3D reconstruction methods had longer acquisition times
and image-processing times, RT 3DE has significantly cut
down on data acquisition time in many cases, to a single
heartbeat or four to six heartbeats. In addition, border
tracking has been increasingly automated, requiring only
specifying anatomic landmarks within the left ventricle
(medial and lateral mitral annular points and the apex).
While border tracking has made the reporting of ventricular
volumes more practical, the best results are obtained only
when image quality is good and when computer generated
boundaries are manually corrected.
The underestimation of ventricular volumes and
sources of error have been studied extensively by several
investigators. Using the several modes of 3D data acquisition as well as processing of 3D data, it is possible to analyze
in detail the sources of error contributing to traditional
underestimation of volumes by echocardiography. Image
positioning error includes nonorthogonal image positioning as well as apical foreshortening. This type of error
predominates in normally shaped ventricles. It can be
minimized by aligning apical images in such a way that

the long axis of the ventricle is maximized before manual

boundary tracing or border tracking is applied. However,
in patients with abnormal ventricles, the predominant
source of error is due to geometric assumptions that may
not be valid.35 This source of error can be minimized by
increasing the number of images utilized for sampling
the ventricle and carefully editing computer-generated
endocardial boundary. However, despite controlling for
image positioning error and geometric assumption error,
underestimation is still present due to differences in
boundary tracing depending on the modality chosen as
the reference modality.35 This finding was confirmed by
a multicenter study36 as well as by a recent meta-analysis
of 23 studies (1,638 echocardiograms) that compared LV
volumes and EF measured by RT 3DE and CMR examined
the overall accuracy of RT 3DE. A subset of those also
compared standard 2D methods with CMR. The pooled
biases 2 SDs for 3DE were 19.1 34.2 mL, 10.1
29.7 mL, and 0.6 11.8% for EDV, ESV, and EF, respectively.
Nine studies also included data from 2DE, where the
pooled biases were 48.2 55.9 mL, 27.7 45.7 mL, and
0.1 13.9% for EDV, ESV, and EF, respectively. In this subset,
the difference in bias between 3DE and 2D volumes was
statistically significant (p = 0.01 for both EDV and ESV).
The difference in variance was statistically significant
(p < 0.001) for all three measurements.37 Boundary-tracing
error depends largely on image quality38 and differences
in image segmentation, particularly with respect to the
handling of papillary muscles and trabeculae. In CMR
methods, these tend to be included in the cavity volume
to a greater extent, thereby resulting in larger volumes.
The larger the ventricle, the greater is the degree of
underestimation.35 The underestimation of 3DE volumes
is particularly noteworthy in subjects with heart failure.
Compared to CMR, RT 3DE is accurate for evaluation of
EF and feasible in heart failure patients, at the expense of
a significant underestimation of LV volumes, particularly
when LVEDV is above 120 mL/m2.39
Fully automated endocardial trabecular contouring
algorithms have also been used and validated to compute
volumes and EF and compared to CMR in patients in sinus
rhythm (67 subjects) as well as in atrial fibrillation (24
subjects). To correct for the underestimation of volumes,
an automated correction can be applied to track the
compacted myocardium. Among all sinus rhythm patients,
there was excellent correlation between RT 3DE and CMR
for EDV, ESV and EF (r = 0.90, 0.96, and 0.98, respectively;
p < 0.001). In patients with EF 50% (n =36), EDV and ESV
were underestimated by 10.7 17.5 mL (p = 0.001) and by

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

4.1 6.1 mL (p < 0.001), respectively. In those with EF < 50%

(n = 31), EDV and ESV were underestimated by 25.7 32.7
mL (p < 0.001) and by 16.2 24.0 mL (p = 0.001). Automated
contour correction to track the compacted myocardium
eliminated mean volume differences between RT 3DE and
CMR. In patients with atrial fibrillation, LV volumes and EF
were accurate by RT 3DE (r = 0.94, 0.94, and 0.91 for EDV,
ESV, and EF, respectively; p < 0.001). Automated 3D LV
volumes and EF were highly reproducible, as expected.40
Newer ways of addressing the systematic underestimation of RT 3DE volumes compared to CMR have
utilized contrast agents. The use of contrast agents may
improve endocardial border recognition in RT 3DE.
However, automatic and semi-automated border-tracking
algorithms for calculation of RT 3DE ventricular volumes
have not been rigorously developed for use with contrast
agents. In addition, contrast agents can sometimes cause
attenuation and difficulty in identifying the valve planes,
which may result in variable inclusion or exclusion of
the left atrium. An approach that shows some promise,
particularly in patients with poor acoustic windows, is
the use of contrast agents in conjunction with power
modulation (PM) imaging that uses low mechanical
indices and provides uniform LV opacification.41

Normal RT 3DE Values for Volumes and

Ejection Fraction
Practical and routine clinical use of 3DE volumes and
EF to detect LV remodeling and dysfunction require agespecific and gender-specific reference ranges. In 226
consecutive healthy Caucasian subjects (125 women; age
range, 1876 years), comprehensive 3DE analyses of LV
parameters were performed, and values were compared
with those obtained by conventional echocardiography.
Upper reference values (mean + 2 SDs) for 3DE LV EDVs
and ESVs were 85 and 34 mL/m2 in men and 72 and
28 mL/m2 in women, respectively. Indexing LV volumes
to body surface area did not eliminate gender differences.
Lower reference values (mean 2 SDs) for EF were 54%
in men and 57% in women and for stroke volume were
25 and 24 mL/m2, respectively. Upper reference values
for LV mass were 97 g/m2 in men and 90 g/m2 in women
and for end-diastolic sphericity index were 0.49 and 0.48,
respectively. Significant age dependency of LV parameters
was identified and reported across age groups. 3D
echocardiographic LV volumes were larger, EF was similar
and LV stroke volume and mass were significantly smaller

1157

in comparison with the corresponding values obtained by

conventional echocardiography.42
In addition to gender- and age-specific reference
values, population-specific reference values may also be
important. A study of 978 subjects from the London Life
Sciences Prospective Population (LOLIPOP) study, who
were free of clinical cardiovascular disease, hypertension,
and type 2 diabetes, showed that indexed 3DE LV volumes
were significantly smaller in female as compared with male
subjects and in Indian Asians compared with European
whites. Upper limit of normal (mean 2 SD) reference
values for the LVESV index and LVEDV index for the four
ethnicitysex subgroups were, respectively, as follows:
European white men, 29 and 67 mL/m2; Indian Asian
men, 26 mL/m2 and 59 mL/m2; European white women,
24 mL/m2 and 58 mL/m2; Indian Asian women, 23 mL/m2
and 55 mL/m2, respectively. Compared with 3DE studies,
2DE underestimated the LVESV index and LVEDV index by
an average of 2.0 and 4.7 mL/m2, respectively. LVEF was
similar between in all four groups and between 2D and
3D techniques, with a lower cutoff of 52% for the whole
cohort.43

New 3DE Parameters and RT 3DE

Left Ventricular Strain
The ability to generate timevolume curves from dynamic
RT 3DE volumes throughout the cardiac cycle have
generated new 3DE parameters of LV diastolic and systolic
performance that have shown interesting results in small
pilot studies. These parameters may be particularly
helpful in the detection of ischemia since diastolic LV
abnormalities are sensitive early signs of myocardial
ischemia and persist longer than systolic changes.44,45
Abnormalities of peak filling rate (PFR), expressed in units
of EDV/s, have been reported in ischemic patients using
Doppler echocardiography.46 A pilot study by Gopal et al.
used RT 3DE to evaluate PFR in 19 subjects with an
intermediate-high risk of computer-aided design (CAD)
together with 1-day rest/stress adenosine 99mTc-sestamibi
gated single-photon computed emission tomography
(GSPECT). Adenosine was infused at 140 mcg/kg/min
over 6 minutes. Nuclear images were acquired at 16
frames/R-R interval for 64 projections over a 180 arc at
rest and poststress. 2DE and RT 3DE were performed
at rest and at peak stress (at 26 minutes of adenosine
infusion). GSPECT and RT 3DE data were analyzed by QGS
(Cedars-Sinai) and QLab (Philips) algorithms, respectively.

1158

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 54.11: Peak filling rate (PFR) derived from RT 3DE time volume
curves fitted to third order harmonics normal response-rest and
stress. (RT 3DE: Real time, three-dimensional echocardiography).

Fig. 54.12: Peak filling rate (PFR) derived from RT 3DE time volume
curves fitted to third order harmonics-ischemic response-rest and
stress. (RT 3DE: Real time, three-dimensional echocardiography).

Nuclear and RT 3DE volume versus time curves were

Fourier-fit to third-order harmonics to compute PFR
by custom software (RSI, Inc., Boulder, CO) for rest and
stress. PFR difference (PFR) was defined as PFR stress
PFR rest. Nuclear perfusion defects were quantified by
17-segment/5-point stress and rest nuclear perfusion
scores and a summed difference score (SDS) > 2 accepted
as abnormal and indicative of stress-induced ischemia.47
A qualitative assessment of rest and stress wall motion
scores (WMS) were determined for 17 segments and 2DE
WMS difference (WMS) were computed as follows based
on systolic wall thickening48: normal wall thickening was
given a score of 1; hypokinetic wall = 2; akinetic was scored
= 3; and dyskinetic = 4. The mean age of the study group was
68.4 15 years, with 14 males. There were no significant
differences in the change in heart rate between rest and
adenosine stress studies for patients with ischemia versus
patients without ischemia (heart rate change of 17 12
beats/min vs 13 8 beats/min, p = 0.15). Of all the echo
parameters examined, only PFR exhibited significant
associations with ischemia. The response to adenosine
stress in patients without ischemia was an increase or
no change in PFR (Fig. 54.11). An abnormal response
to adenosine stress was a decrease in PFR (Fig. 54.12).

Figure 54.13 shows a representative patient with lateral

perfusion defect whose 2DE WMS was normal but whose
PFR was abnormal. Of the six patients with ischemia
(SDS > 2), four patients had abnormal PFR compared
to those without ischemia (0.12 0.77 EDV/s vs +0.66
0.44 EDV/s, p = 0.02); two patients had both a normal
WMS and a normal PFR. Two of the four patients with
abnormal PFR also had an abnormal WMS (Table 54.1).
PFR correlated inversely with ischemia [Spearmans
coefficient = 0.55, p = 0.03 with 95% confidence
interval (CI) = 0.81 to 0.08, Fisher exact test, p = 0.006;
Fig. 54.14]. An abnormal PFR predicted myocardial
ischemia with 89% accuracy, 100% specificity, 66% sensitivity, 100% positive predictive value, and 86% negative
predictive (ROC PFR threshold > 0.0).
In addition to being able to assess ejection rates and
filling rates, LV performance may be assessed by 3D strain
analysis. Strain analysis can be performed by a technique
known as STE, which identifies a pattern of speckles in
one frame and then tries to find the same pattern in the
following frames using pattern-matching algorithms. This
allows all anatomic features including the myocardium
present in the echocardiographic image to be tracked
through space and time. This technique offers advantages

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

1159

Table 54.1: Comparison of mean PFR to SDS >2.

Myocardial ischemia

Stress-rest differences
PFR > 0

PFR < 0

SDS < 2

13

SDS > 2

Fig. 54.13: Lateral wall ischemia-adenosine 99mTc-Sestamibi

GSPECT. GSPECT, gated single-photon computed emission
tomography.
Fig. 54.14: Comparison of mean PFR to SDS >2. Mean values
and error bars representing one standard deviation is plotted.

Fig. 54.15: One-dimensional Lagrangian strain. The length is the

only strain component, and thus L is measured along the only
coordinate axis, thus L = x.
Source: Reproduced with permission from: http://folk.ntnu.no/
stoylen/strainrate).

over tissue Doppler imaging (TDI), which also measures

myocardial motion, but which is angle dependent.
Therefore, motion that occurs along the ultrasound beam
can be detected by TDI. However, motion that occurs
perpendicular to the ultrasound beam is not detected.
This limitation is overcome by STE. Myocardial motion
can be expressed as myocardial displacement, velocity,
or strain. However, the former two are affected by cardiac
translation that may occur with each heartbeat or with
respiration. Strain, however, measures the deformation of
the myocardial muscle as the difference between its length
at a certain point in the cardiac cycle and its initial length
divided by its initial length. Thus, contraction is expressed
as a negative strain value whereas elongation is expressed
as a positive strain value. Limitations of 2D STE include
difficulties tracking the speckles when the speckles move

in and out of the 2D imaging plane. To overcome this

limitation, 3D STE has been developed. Thus, displacement
measured by 3D STE is larger than the corresponding 2D
STE values, indicating that through-plane motion (motion
perpendicular to the imaging plane) can be detected by 3D
STE but not 2D STE.49
Myocardial contractile motion is complex and 3D.
In addition, the complex arrangement of muscle fibers
contributes to varying extents to myocardial deformation.
Strain analysis is usually performed by using an external
Cartesian spatial coordinate system. 1D strain occurs only
along the coordinate axis (Fig. 54.15). In 2D, the strain
tensor has four components, two along the coordinate
axis and two shear strains (Fig. 54.16). In 3D, the complex
3D myocardial deformation can be decomposed into
three normal and six shear strains (Fig. 54.17). Normal
strains (longitudinal, circumferential, and radial) reflect
changes in length along a spatial coordinate system.50
Shear strains (longitudinalradial, circumferentialradial
and circumferentiallongitudinal) are forces acting in
opposite directions.50 Myocardial strain can be described
without an external spatial coordinate system using three
principal strains and three principal angles or axes of
deformation that form an internal frame of reference.50 The

1160

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 54.16: Strain in two dimensions. Above are the two normal
strains along the x and y axes, where each strain component can
be seen as Lagrangian strain along one main axis. Below are the
two shear strain components, movement of the borders relative to
each other. Here there are two strain components, characterized
by the tangent to the shear angle alpha.
Source: Reproduced with permission from http://folk.ntnu.no/
stoylen/strainrate.

Fig. 54.17: Strain in three dimensions. Only the three strain components along the x axis (one normal, two shear) are shown, but the
y and z strains will be exactly the same and can be imagined by rotating the x images.
Source: Reproduced with permission from: http://folk.ntnu.no/stoylen/strainrate.

three principal strains are oriented along three mutually

orthogonal directions and ranked from maximum
contraction at the end of systole to maximum lengthening
at the end of diastole.50,51 The principal strain is the
maximum contraction that occurs in an oblique direction
within the circumferentiallongitudinal plane and
angled to spiral counterclockwise from the apex to base
(as viewed from the apex).51 It aligns in the general
direction of the subepicardial muscle fibers.52 The benefit
of this approach of describing myocardial strain along the
axes of deformation (internal frame of reference) rather
than in terms of an external Cartesian spatial coordinate
system is that it can provide a more integrated perspective
of all the major forces experienced at the tissue level.
In addition, an internal frame of reference allows us to
eliminate shear strain since principal strain represents the
combined effect of shortening and shear.53 Rotation and

translation of the heart are no longer important with this

internal frame of reference approach53 (Fig. 54.18).
As described above, 3D STE offers an integrated
approach toward analyzing the complex motion of
the heart. In addition to providing more accurate and
reproducible volumes compared to 2D STE,54 it provides
a new methodology for analyzing regional function.
3D STE shows that all strain components are reduced
in abnormal regional segments.49 3D STE also provides
new 3D parameters to assess LV systolic function such
as global area strain (percentage of deformation in the
LV endocardial surface area defined by the longitudinal
and circumferential strains vectors). Recent studies have
shown that this parameter is more accurate and
reproducible on a regional level than the other
components.55 Global area strain is strongly correlated
with LVEF and to a lesser degree with cardiac output.56

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

Fig. 54.18: In the heart, the usual directions are longitudinal,

transmural (radial), and circumferential as shown to the left. In
systole, there is longitudinal shortening, transmural (radial) thickening and circumferential shortening. (This is an orthogonal coordinate system, but the directions of the axes are tangential to the
myocardium, and thus changes from point to point).
Source: Reproduced with permission from: http://folk.ntnu.no/
stoylen/strainrate.

A progressive decrease in global area strain is noted as

heart failure progresses from normal to stage D.57 A study
that compared a 3D strain vector (summing the radial,
circumferential and longitudinal vectors) and 3D area
strain in patients with coronary artery disease showed that
while area strain correlated with the severity of transmural
extent of necrosis, 3D strain decreased only when necrosis
extent was > 75%.58 Though many of these alternative
approaches of quantifying LV performance appear
promising, it is unclear how these indexes will correlate
diagnostically or prognostically given such constraints as
vendor variability and proprietary algorithm differences, a
sentiment reflected by a recent consensus statement by the
American and European Societies of Echocardiography.59

Left Ventricular Mass by 3DE

Several studies, including the Framingham Heart Study,
have demonstrated that elevated LV mass is an independent and strong predictor of morbid cardiac events and
death.60 Conventional 1D M-mode echo and 2DE methods
of measuring mass rely on geometric assumptions, lack
spatial registration and are associated with comparatively
high measurement variability, particularly in abnormally
shaped hearts.61 As a consequence, the testretest stability
of 1D and 2DE for the serial measurement of LV mass in
patients with hypertension may be impaired. Gottdiener

1161

Fig. 54.19: Three-dimensional (3D) line of intersection display

positioned short-axis images for left ventricular (LV) mass computation. Traced epicardial and endocardial borders are shown.

et al.62 showed that the 95% CI width of a single replicate

measurement of LV mass was 59 g. This measurement
variability exceeds the usual decrease in mass during
treatment.62 Alternative imaging tools such as MRI
and ultrafast CT not only use nonportable equipment
but also are expensive, cumbersome and not widely
available for serially following LV mass in patients. LV
mass has been calculated by 3DE reconstruction by
subtracting the endocardial volume from that of the
epicardium and multiplying the result by 1.05, the density
of myocardium (Fig. 54.19). It was calculated in vitro
in fixed animal hearts very accurately with a standard
error of the estimate (SEE) of 23 g.63 Anatomic in vivo
animal validation provided the best results for 3D echo
reconstruction (r = 0.96, SEE = 5.9 g, accuracy 6.8%)
compared with the truncated ellipsoid (r = 0.88, SEE = 10.2 g,
accuracy 12.6%) and bullet (r = 0.83, SEE = 12 g, accuracy
= 12.7%) algorithms.63 In vivo validation of LV mass by 3DE
reconstruction was carried out in normal subjects using
MRI as a standard of comparison and was shown to correlate
highly (r = 0.93, SEE = 9.2 g) with good interobserver
variability (6.3%) and statistically no different from
corresponding MRI values.64 In the same population, 3DE
reconstruction achieved a two- to threefold improvement
in the correlation with MRI over conventional M-mode
and 2DE algorithms used to compute LV mass.64 In
patients with abnormal ventricular geometry, the SEE
and limits of agreement between 3DE reconstruction
and MRI were roughly twice the values found in normal
subjects.18 LV mass determination by 3DE reconstruction

1162

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

has also been anatomically validated in vivo in humans

undergoing heart transplantation using the true weight of
the left ventricle of the explanted hearts (range 125433 g)
as the standard of reference and compared to M-mode
Penn, 2DE area length and truncated ellipsoid algorithms.
The results showed that 3DE reconstruction is a highly
accurate and reproducible method (r = 0.993, accuracy
= 4.6%, bias = 3.4 g, and interobserver variability 9.4%).
In addition, 3DE reconstruction accuracy was fourfold
superior to 2DE (r = 0.898, accuracy 19.2%, bias +21.7 g,
interobserver variability 16.7%) and ninefold superior to
M-mode echocardiography (r = 0.817, accuracy 43.4%, bias
+85.1 g, interobserver variability 18.2%).65 Studies of LV
mass with 3DE reconstruction showed that conventional
M-mode calculations of LV mass significantly overestimate
true mass.
RT 3DE has also been used to calculate the feasibility
of calculating LV mass. While 3DE reconstruction utilized
parasternal short-axis images for tracing epicardial and
endocardial borders, the RT 3DE utilizes apical views. The
use of apical views for tracing epicardial and endocardial
borders presents two problems: apical foreshortening and
off-axis longitudinal views. The first problem results in
underestimation of LV mass that may be reduced by using
a 3D-guided biplane technique.66 The second problem
occurs when the imaging plane does not pass through
the center of the left ventricle along its longitudinal axis.
This can result in tangential views of the myocardium that
show an artificially thick ventricle and may overestimate
mass. RT 3DE imaging with single-beat capture has been
used to validate LV mass measurements in 69 patients with
hypertrophic cardiomyopathy against a CMR reference
standard. RTDE and CMR values were also compared to
M-mode mass and the 2D-based truncated ellipsoid mass.
The mean time for RT 3DE analysis was 5.85 1.81 minutes.
Intraclass correlation analysis showed a close relationship
between RT 3DE and CMR LV mass (r = 0.86, p < 0.0001).
However, LV mass by the M-mode or 2DE method showed
a smaller intraclass correlation coefficient compared with
CMR-determined mass (r = 0.48, p = 0.01, and r = 0.71,
p < 0.001, respectively). BlandAltman analysis showed
reasonable limits of agreement between LV mass by RT
3DE and by CMR, with a smaller positive bias [19.5 g
(9.1%)] compared to that by the M-mode and 2D methods
[35.1 g (20.2%) and 30.6 g (17.6%), respectively]. These
results confirm the finding by 3DE reconstruction that
conventional LV mass algorithms tend to overestimate LV
mass.67 Although 3DE has shown to be more accurate than
M-mode and 2DE, a meta-analysis of 25 studies including

671 comparisons were analyzed showed that 3DE still

underestimated LV mass compared to CMR. However, the
underestimation improved with time from 5.7 g, 95% CI
11.3 to 0.2, p = 0.04 in studies before 2004 to 0.1 g, 95%
CI 2.2 to 1.9, p = 0.90 in studies published after 2008.68

Left Ventricular Remodeling, Sphericity,

and Regional Function by 3DE
3DE reconstruction has been used to analyze LV endocardial surface area, infarct subtended surface area,
infarct subtended volume and volume/mass ratio, which
may be measures that supplement measures of LV mass
in studying LV remodeling.6972 In addition, LV shape can
be characterized in terms of a sphericity index. This is
done by calculating a 3D surface area/volume ratio and
indexing it to a surface area/volume ratio of a sphere.
As the LV becomes more globular and spherical (i.e.
undergoes adverse LV remodeling), its sphericity index
approaches.1,73
LV volume can be further broken down into 16 or 17
regional segments and volume in each of these segments
can be tracked over the full cardiac cycle to generate time
volume curves (Fig. 54.20). In a normal subject without
significant intracardiac dyssynchrony, the minima of

Fig. 54.20: The left ventricle (LV) has been subdivided into 17
regional segments. The timevolume curve for each segment is
displayed. In this normal subject without significant intracardiac
dyssynchrony, the minima of the timevolume curves (shown on
the top panel) and their first derivatives (bottom panel) are all
reached at roughly the same time.

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

Fig. 54.21: The left ventricle has been subdivided into 17 regional
segments. The time-volume curve for each segment is displayed.
In this patient with significant intracardiac dyssynchrony, the minima of the timevolume curves (shown on the top panel) and their
first derivatives (bottom panel) have a wide temporal dispersion.

Fig. 54.23: Timevolume curves are generated from the entire

surface of the left ventricle (LV) and are color-coded in such a
manner that late contracting segments are color-coded in shades
of red and normally contracting segments are color-coded blue.
This is a patient with significant intracardiac dyssynchrony with
large areas of late contracting segments (shown in red).

the timevolume curves and their first derivatives are all

reached at roughly the same time. However, in a patient
with significant intracardiac dyssynchrony there is usually
widening of the QRS interval together with a temporal
dispersion of the timevolume curves (Fig. 54.21). Instead
of breaking the LV down only into 17 regional segments,
a bulls-eye plot can be made of timevolume curves that

1163

Fig. 54.22: Timevolume curves are generated from the entire

surface of the left ventricle (LV) and are color-coded in such a
manner that late contracting segments are color-coded in shades
of red and normally contracting segments are color-coded blue.
This is a normal subject without dyssynchrony (no red areas are
noted). (LA: Left atrium).

are generated from the entire LV surface. These time

volume curves can be color-coded in such a manner
as to shown late contracting segments as shades of red
and normally contracting segments in blue. A normal
subject with a perfectly synchronous heart is shown in
Figure 54.22. A patient with significant dyssynchrony is
shown in Figure 54.23 with large areas of late contracting
segments (shown in red). After receiving a biventricular
pacemaker, the areas of late contracting segments (shown
in red) have reduced in size (Fig. 54.24). Though these
tools may be very helpful in evaluating a patient for a
biventricular pacemaker, the reliability of the timevolume
curves depends critically on the 3DE image quality. In
addition, when regional volumes measured in a group
of patients were compared against CMR as a reference,
the levels of agreement were very high in basal and midventricular segments, but were considerably lower near
the apex. This difference could probably be explained by
the limited endocardial definition near the apex on both
3DE and short-axis CMR images that are affected by partial
volume artifacts at this level.74
RT 3DE also provides new ways of assessing regional
LV wall motion that may have implications for interpreting
stress echocardiograms. Instead of viewing the left
ventricle in conventional short axis, and apical images that
only display a limited portion of the myocardium, RT 3DE
can display the entire myocardial volume in a multislice
panel, allowing a comprehensive assessment of regional
wall motion (Movie clip 9).

1164

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 54.24: Timevolume curves are generated from the entire

surface of the left ventricle (LV) and are color-coded in such a
manner that late contracting segments are color-coded in shades
of red and normally contracting segments are color-coded blue.
This is the same patient after receiving a biventricular pacemaker.
Note that the areas of late contracting segments (shown in red)
have reduced in size.

Fig. 54.26: Three-dimensional echocardiography (3DE) detected

a statistically significant decrease in LV mass at both 6 weeks and
12 weeks whereas M-mode echocardiography showed a statistically significant decrease in LV mass only after 12 weeks of treatment. (LV: Left ventricle).

Serial Evaluation of Patients with 3DE

The utility of a test to assess a parameter in a serial fashion
is measured by its testretest variability. It is well known
that the testretest variability of 3DE is lower than that of
2DE. For example, testretest variability studies of 3DE
LV mass have shown that 95% of the time, a change of

Fig. 54.25: Three-dimensional (3D) echo detected a statistically

significant decrease in LV mass which paralleled a decline in BP.
(BP: Blood pressure; LV: Left ventricle).

25.8 g or greater is considered significant, that is, not due

to measurement variability alone. This is a greater than
twofold improvement over the value of 59 g reported by
Gottdiener et al. for the conventional M-mode method.
The importance of greater accuracy and reproducibility
of 3D echo in detecting biologically significant LV mass
regression is illustrated in a preliminary study of patients
with hypertension and LV hypertrophy that were treated
and imaged at baseline, after 6 weeks, and after 12 weeks
of antihypertensive therapy. Under the conditions of
this study, 3D echo detected a statistically significant
decrease in LV mass at both 6 and 12 weeks owing to its
lower measurement variability (narrower CIs) whereas
M-mode echocardiography showed a statistically significant decrease in LV mass only after 12 weeks of treatment
(Figs 54.25 and 54.26). Using the standard deviation (SD)
of the decrease in LV mass at the end of 12 weeks by each
method, it was calculated that 3D echo was capable of
detecting a 10 g reduction in LV mass at a power of 80% with
one-third the number of patients (n = 42) compared with
M-mode echocardiography (n = 148 patients; Fig. 54.27).
In addition, owing to the low intraobserver variability of
3DE, 85% of the measured change in LV mass could be
attributed to true biologic change. In contrast, since the
intraobserver variability of M-mode echocardiography
exceeded the measured change in mass, the contribution
due to true biologic change could not be determined.75
Sequential quantification of LVEF and volumes in
patients undergoing cancer chemotherapy are important
to clinical decision making. Marwick and colleagues

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

1165

Table 54.2: Misclassification rate of EF postmyocardial infarction (comparison of clinical EF, 2DE EF, and 3DE EF to CMREF)

25%

CMR EF = 40%

CMR EF = 30%

Clinical EF

42.4%

15.2%

2DE EF

21.8%

10.9%

3DE EF

14.5%

5.4%

(EF: Ejection fraction)

studied the method for EF measurement with the lowest

temporal variability. Fifty-six patients were selected for
stable function in the face of chemotherapy for breast
cancer by defining stability of global longitudinal strain
(GLS) at up to five time points (baseline, 3, 6, 9, and 12
months). In this way, changes in EF were considered
to reflect temporal variability of measurements rather
than cardiotoxicity. 2DE-biplane, 2D-triplane, and 3DE
acquisitions with and without contrast administration
was performed at each time point. Stable LV function was
defined as normal GLS ( 16.0%) at each examination.
The best temporal variability of EF 0.06 was shown by
noncontrast 3DE while other 2DE methods showed a
temporal variability of > 0.10 with 2D methods over 1 year
of follow-up.76

RT 3DE for Postmyocardial Infarct

Risk Stratification
Perhaps the greatest utility of 3DE LV quantification
occurs in risk stratifying patients with heart failure or
moderate LV dysfunction postmyocardial infarction (postMI). Decisions are made regarding offering lifesaving
therapies such as implantable defibrillator placement
and/or biventricular pacemaker based on the assessment
of the EF post-MI. Risk stratification by routine methods
(2DE, planar multigated radionuclide angiography, and
cineventriculography) were compared to 3DE and CMR
in 55 patients with MI or congestive heart failure and EF
40%. Patients were stratified by CMREF into two groups:
EF 30% and 40%. For CMREF 30%, the misclassification rates were: 42%, 22%, and 14.5% by routine methods,
2D, and 3DE; for CMREF 40%, misclassification occurred
in 15%, 11%, and 5% by routine methods, 2DE and 3DE.
Regardless of the cutoff level chosen, 3DE had the lowest
misclassification rate. 3DE also had a stronger correlation
and less bias than 2DE (Table 54.2). 3DE but not 2DE was
equivalent to CMR by analysis of variance (ANOVA). Test
retest variability of 3DE was threefold lower than 2DE.
This study shows inadequacy of routine methods and the

Fig. 54.27: Using the standard deviation of the decrease in LV

mass at the end of 12 weeks by each method, it was calculated
that three-dimensional (3D) echo was capable of detecting a 10 g
reduction in LV mass at a power of 80% with one-third the number of patients (n = 42) compared with M-mode echocardiography
(n = 148 patients).

superiority of 3DE for risk stratification by EF post-MI. The

reduced testretest variability of 3DE compared to 2DE
establishes its utility for serial monitoring.77

3D QUANTITATION OF THE RIGHT

VENTRICLE
Anatomic Considerations and Prior
Conventional Approaches
Accurate estimation of RV size and function is essential for
the management of many cardiac disorders. Estimation
of RV size and function is of central importance for the
management of various congenital diseases.78 Echocardiographic variables that reflect the severity of right
heart failure in primary pulmonary hypertension (PH)
may help identify patients appropriate for more intensive
therapy or earlier transplantation.79 Assessment of RV
function is also important in determining treatment
options for patients with pulmonary embolism, MI, and
heart failure.80,81 Therefore, an accurate, easily repeated,
noninvasive method would be ideal for the serial
evaluation of patients.
However, evaluation of RV function has been
hampered by its complex crescentic shape, large infundibulum, and trabecular nature. Its function by invasive
angiography can be characterized using area and length

1166

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 54.28: Two-dimensional echocardiography (2DE) long-axis of

RV (length) in diastole for purposes of RV volume calculation by
the area-length method. (RV: Right ventricle).

Fig. 54.29: Two-dimensional echocardiography (2DE) short-axis

of RV infundibular area in diastole for purposes of RV volume
calculation by the area-length method. (RV: Right ventricle).

measurements or Simpsons rule from single or biplane

projections.82 Single plane methods provide limited
sampling of the RV, depend on the orientation of the
imaging planes with respect to intrinsic RV axes, and
make shape assumptions. Biplane methods provide better
sampling, but are invasive and often overestimate volume.
While radionuclide ventriculography is not constrained
by geometric assumptions, results have been variable and
scanning requires the injection of radioactive agents.83
The retrosternal location of the RV as well as the
presence of ribs makes it difficult to access this chamber
fully by transthoracic echocardiography. Therefore, individual aspects of its function can be assessed separately.
Transverse shortening can be assessed by fractional
area change (FAC) in each short-axis slice. Longitudinal
contraction can be assessed by tricuspid annular plane
systolic excursion (TAPSE). TAPSE is measured as the
distance in the four-chamber plane between the lateral
aspect of the tricuspid annulus at end-diastole and endsystole. A TAPSE value of > 20 mm has been reported to be
normal. Global function is assessed by calculation of right
ventricular ejection fraction (RVEF) and several efforts
have been made to find echocardiographic methods based
on simple geometric models using single plane, biplane,
or on multiplane methods based on Simpsons rule.8493
The most common method utilizes the area and length
from an apical four-chamber view and an RV outflow tract
view93 (Figs 54.28 and 54.29). The two views are assumed

to have an orthogonal relationship to each other. However,

the transducer is moved from one position to another
based on the sonographers knowledge of cardiac anatomy
and orthogonality is assumed but not verified and
rarely satisfied. Furthermore, a prolate-ellipsoid shape
assumption is made, which also may not accurately depict
RV anatomy. While area-length methods work in vitro
and in animal models, they have wide confidence limits
in human subjects when compared to methods, which
are not subject to geometric assumptions.94 Moreover, the
geometric models used to describe the shape of the RV can
be changed unpredictably by disease.
Wide confidence limits also occur due to reliance on
anatomic visual information alone for determining image
plane orientation. Previous experience with freehand
3DE reconstruction has shown wide operator variability
in the optimal positioning of short-axis and apical image
planes.2 In addition, apical views are often foreshortened
during 2DE scanning, resulting in underestimation of the
RV length that is used in arealength formulas.21 Sheehan
et al. found that standard 2DE monoplane and biplane
RV algorithms performed better when the images were
positioned correctly using 3D electromagnetic guidance.95
Techniques such as the CT96 and CMR overcome limitations
posed by other methods in that image planes are precisely
defined and geometric assumptions are unnecessary.97
However, these imaging modalities are expensive and are
not widespread.

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

1167

Previous 3D Reconstruction Approaches

A variety of options for rapid 3DE image acquisition and
reconstruction of the right ventricle have been used.98111
Early approaches to 3DE reconstruction occurred from
fixed transducer positions (apical or subcostal) and used
rotational or fan-like scanning. This approach works
if the patients are prescreened for good image quality, a
prerequisite for this approach. However, a failure rate of 18%
has been reported in postoperative subjects due to poor
transthoracic windows.112 Therefore, 3DE reconstruction
from a fixed transducer position provides mechanical
3D spatial registration of cross-sectional images, but is
feasible only in those subjects who are echogenic enough
to permit complete visualization of the right ventricle
from a single echocardiographic position. Acoustic
and electromagnetic tracking devices were developed
to provide 3D spatial registration while scanning in a
freehand fashion, permitting the sonographer to utilize
all available echocardiographic windows.95,101,113 Apfel et
al. studied 26 patients with PH with an acoustic spatial
locating system and found a good correlation to spin-echo
CMR but with 3133% volume underestimation by 3DE.101
Since data acquisition occurs over several cardiac cycles
in the span of 810 minutes, respiratory, whole body, or
transducer motion will lead to data misregistration.

RT 3DE Approach to RV Quantification

RT 3DE uses matrix array transducer technology, pioneered
by von Ramm et al. and permits continuous acquisition
of volumetric data, thereby allowing rapid scanning and
minimizing the chance of motion artifacts. Cardiac motion
can be evaluated in a dynamic mode and the heart can
be viewed from any desired plane. Ota et al. validated RV
volume measurements using a first-generation RT 3DE
system in excised canine hearts and in 14 normal subjects.
Though their method performed accurately in vitro, their
in vivo standard of comparison was not a 3D method
but a 2D monoplanar modified Simpsons method. A
good correlation and interobserver variability (8.39.4%)
was noted between 3D right ventricular stroke volume
(RVSV) and monoplanar 2DSV.114 Shiota et al. validated
the same technology in sheep using electromagnetic flow
probes. The correlation obtained for RVSV was r = 0.8
and the BlandAltman analysis showed a mean RVSV
difference of 2.7 mL.106 First-generation RT 3DE systems
use a sparse array matrix transducer, which utilizes 256
nonsimultaneously firing elements to acquire a narrow

Fig. 54.30: Real time, three-dimensional echocardiography

(RT 3DE) data acquisition from an off-axis apical window. The top
left, top right and bottom left panels show three orthogonal multiplanar reconstructions (MPRs) of the RV. The bottom right panel
shows a partial coronal view of the three-dimensional (3D) data
set showing the intersecting sagittal and axial planes from which
the MPRs were derived. (RV: Right ventricle).

sector angle (60 60) pyramidal data set. While the 3D

data set can be captured in one heartbeat, frame rates are
low and image quality is relatively poor. Due to the narrow
sector angle, visualization of the right ventricle is difficult
since a large portion of it lies in the near field where the
sector is narrowest.
Second-generation RT 3DE systems use fully sampled
matrix array transducers utilizing 3,000 elements. This
results in improved image quality, greater contrast resolution, higher sensitivity, and penetration as well as capabilities for harmonic imaging. The full volume of the heart
can be obtained by assembling four wedges of 15 60
each over eight consecutive cardiac cycles to obtain a
pyramidal sector 90 90. Some approaches have utilized
an off-axis apical four-chamber view that highlights the
right ventricle as the initial view taken for the acquisition
of the RT 3DE data set (Fig. 54.30). Disc summation and
apical rotation algorithms have been developed to quantify
RV size and function in connection with RT 3DE. The RT
3DE-disc summation algorithm appears to be superior
to an apical rotational algorithm because it is able to
handle data from the RV inflow and outflow tracts, which

1168

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 54.31: RT 3DE-apical rotation methodThe top left, top right

and bottom left panels show three orthogonal MPRs of the RV
taken at the basal level showing discontinuity of the RV inflow and
outflow tracts. (RT 3DE: Real time, three-dimensional echocardiography; RV: Right ventricle).

Fig. 54.32: RT 3DE-apical rotation methodthe same 3D data set

shown in Figure 54.31 is now advanced to show three orthogonal
MPRs (top left, top right and bottom left panels) of the RV taken at
the mid-ventricular level. (RT 3DE: Real time, three-dimensional
echocardiography; RV: Right ventricle).

may appear to be discontinuous when viewed in a basal

short-axis cross-section (Fig. 54.31), but not from a midshort-axis section (Fig. 54.32). Whereas the apical rotation
method appears to be appropriate for the simple shape of
the left ventricle, it is unable to handle data in which the
contours appear to overlap (Fig. 54.31). The short-axis disc
summation algorithm is identical to the algorithm used for
analysis of CMR images and is able to handle discontinuous
data and overlapping contours both at basal (Fig. 54.33)
and mid-ventricular levels (Fig. 54.34). Testretest variability for RTDE by disc summation was 3.3%, 8.7%, 10%,
and 10.3%, respectively for EDV, ESV, and EF. Though
testretest variability for right ventricular end-diastolic
volume (RVEDV), RVSV, and RVEF were acceptable (8.7%,
10%, and 10.3%, respectively) and comparable to those
reported for CMR,115 these values were somewhat higher
than those noted for EDV, probably reflecting variability
in end-systolic video-frame selection.116 Normal reference
ranges of indexed volumes (mean 2 SDs) for RVEDV, ESV,
SV, and EF were 38.6 to 92.2 mL/m2, 7.8 to 50.6 mL/m2,
22.5 to 42.9 mL/m2, and 38.0 to 65.3%, respectively, for
women and 47.0 to 100 mL/m2, 23.0 to 52.6 mL/m2, 14.2
to 48.4 mL/m2, and 29.9 to 58.4%, respectively, for men.116
These values are similar to the normal referenced indexed
ranges of indexed volumes for RVEDV and RVESV by

CMR.117,118 Interstudy reproducibility of RVEDV, ESV, and

EF by CMR has been reported to be 6.2%, 14.1%, and 8.3%,
respectively, by Grothues et al.118
Despite encouraging preliminary results, there are still
many challenges associated with routine quantification
of the right ventricle. Chief among them is image quality.
RT 3DE is subject to error if the right ventricle is large and
a large portion of the infundibulum falls outside the near
field afforded by the 90 90 pyramidal sector size. Thus,
while this method works well in normals, its application in
markedly dilated right ventricles has not been established.
Additionally, if the right ventricle is large, undersampling
can occur by the apical rotation method because the
ventricular surface is usually convex and the volume lying
between the true surface and the surface approximated by
the AR algorithm is omitted, resulting in underestimation.
Underestimation may also occur because the RV
inflow and RV outflow tracts may be very large and may
appear to be discontinuous when viewed on a single
image plane and therefore not included by the volume
algorithm. This can be addressed by the short-axis disc
summation algorithm in which portions of the right
ventricle that appear discontinuous on any given plane
such as the inflow and outflow tracts can be included in
the volume by summating separate discontinuous discs.

Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function

Fig. 54.33: RT 3DE-disc summation methodthe top left, top right

and bottom left panels show three orthogonal MPRs of the RV
taken at the basal level showing discontinuity of the RV inflow and
outflow tracts. (RT 3DE: Real time, three-dimensional echocardiography; RV: Right ventricle).

Fig. 54.35: RT 3DE RV automatic boundary tracking algorithm at

the mid-ventricular level. (RT 3DE: Real time, three-dimensional
echocardiography; LV: Left ventricle; RV, Right ventricle).

In addition, the thickness of the discs can be reduced to

reduce interpolation of data between traced areas. Based
on the work of Weiss et al. significant underestimation

1169

Fig. 54.34: RT 3DE-disc summation methodthe same threedimensional (3D) data set shown in Figure 54.4A is now advanced
to show three orthogonal MPRs (top left, top right and bottom left
panels) of the RV taken at the mid-ventricular level. (RT 3DE: Real
time, three-dimensional echocardiography; RV: Right ventricle).

can be minimized by including 710 images.119 Variable

designation of end-diastolic and end-systolic frames by
RT 3DE and CMR is a source of error. Differences in image
acquisition approaches (RT 3DE long-axis rotational
approach vs CMR short-axis cross-sectional approach)
introduce different partial volume effects, which may
introduce error. Endocardial boundaries may be obscured
by tangential RT 3DE-apical slices, whereas variable
inclusion of the right atrium and RV outflow tract may
occur by CMR. Boundary tracing error remains the largest
source of error. Tracing the endocardium on the white side
of the blackwhite boundary minimizes underestimation
of RT 3DE volumes when compared to CMR. Variable
visualization of the apex can be minimized by carefully
manipulating the entire 3D data set so that the largest
long-axis is visualized and prescribing a series of short-axis
images such that they are perpendicular to the long axis.
Toggling between the traced endocardial boundaries as
displayed in the orthogonal multiplanar reconstructions
minimizes erroneous boundary tracing. Although best
results are obtained with manual boundary tracing an
automatic RV boundary recognition algorithm has been
utilized which tracks best at the mid-RV level (Fig. 54.35).
This algorithm requires manual inputs of the tricuspid
annuli and the RV apex as starting points for the boundary
tracking to take place. However, manual correction of the

1170

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

automatically generated boundaries is necessary to avoid

significant underestimation.120 In particular, the base
and the RV outflow tract is incompletely visualized 48%
of the time requires manual correction of the automatic
contours. A 3DTEE study has shown that the RV outflow
tract is not circular, but oval.121 Future developments in
automatic image segmentation, possibly with the help of
contrast agents may improve results.

RT 3DE Studies of Congenital

Heart Disease and PH
The underestimation of RV volumes is particularly marked
in patients who have massive ventricular dilation and
is not ready for clinical use in patients with congenital
heart disease.122 In PH patients, RT 3DE has shown some
promise. In patients with PH, evaluation of the RV diastolic
and systolic volume and EF by RT 3DE showed a higher
discriminating power in comparison, respectively, with 2D
RV diastolic area and the relative FACs.123 RV shape change
has been studied in PH by RT 3DE. In PH, the right ventricle
is more spherical with increased cross-sectional area at
the mid and basal ventricular segments, basal bulging
adjacent to the tricuspid valve and blunting or rounding of
the apex.124 Additionally, a RT 3DE study in these patients
showed that RV inflow and global systolic function was
impaired in inverse relationship with pulmonary artery
systolic pressure and pulmonary vascular resistance.
RV systolic synchronicity was impaired in patients with
severe PH.125 The RV remodels differently depending on
the etiology of the disease. Grapsa et al. studied 141 consecutive patients with differing etiologies of PH (idiopathic,
chronic thromboembolic disease, secondary to mitral
regurgitation). Age- and gender-matched controls were
also studied with RT 3DE. Overall, RVEDV was greater and
RVEF lower in patient with PH compared to those with
thromboembolic disease and mitral regurgitation (186.4
48.8 vs 113.5 vs 109.4 mL, p < 0.001, and 33.2% vs 36.8%
vs 66.8%, p < 0.001, respectively). Tricuspid valve mobility
was most restricted in the thromboembolic group and
least restricted in the mitral regurgitation group. Tricuspid
tenting volume was greater in the thromboembolic group
and PH group than in the mitral regurgitation group. Most
patients with PH (54.6%) had at least moderate tricuspid
regurgitation, while in the thromboembolic group, most
(59.4%) had mild and only 37.5% had moderate tricuspid
regurgitation (p < 0.01). Conversely, patients with mitral
regurgitation (85%) had only mild tricuspid regurgitation.
There was no correlation between RV systolic pressures

and the RVEF or tenting volume. Therefore, the most

adverse remodeling was noted in the patients with PH.126
The same authors studied prognostic markers in these
patients. An increase of right atrial (RA) sphericity index
> 0.24 predicted clinical deterioration with a sensitivity of
96% and a specificity of 90% [area under the curve (AUC)
= 0.97]. RV sphericity index was less sensitive (70%) and
specific (62%) in predicting clinical deterioration (AUC =
0.649). The deterioration in RVEF had a sensitivity of 91.1%
and a specificity of 35.3% (AUC = 0.479) in predicting
clinical deterioration. The dilatation of RA > 14 mL over
1 year had high sensitivity at 82.6% but low specificity at
30.8% in predicting clinical deterioration.127
In summary, the field of 3DE has made tremendous
progress over the last 25 years and is now being offered on
every clinical platform. Though its efficacy and superiority
over conventional techniques is now well established, there
is still considerable variability of products and algorithms
offered by the differing vendor platforms. This makes
standardization difficult among the various platforms
and also makes it difficult to gather a large enough patient
database to offer long-term RT 3DE prognostic parameters.
With further improvements in transducer design, image
quality, temporal resolution, and standardization, it is
anticipated that clinical guidelines regarding its routine
use will emerge.

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CHAPTER 55
Newer Aspects of Structure/Function
to Assess Cardiac Motion
Gerald Buckberg, Navin C Nanda, Julien IE Hoffman, Cecil Coghlan

Snapshot

State-of-the-Art

Composite of State-of-the-Art Reports

Novel Mechanical and Timing Interdependence

Between Torsion and Untwisng

INTRODUCTION
Cardiac motion, until recently, had been thought to follow
the observations of William Harvey, who dissected cadaver
hearts and deduced that the heart underwent constriction
for ejection and dilation for filling, acting like a water
bellows. Keith1 delivered the classic article on structure
and function during presentation of his 1918 Harveian
Lecture, and called Harvey the functional anatomist
who emphasized that we cannot claim to have mastered
the mechanism of the human heart until we have a fundamental explanation of its architecture. Keith described the
cardiac architecture to contain circumferential and helical
fibers, as he perhaps relied upon the observations of Lower2
in the 1600s describing that the cardiac apex showed helical
fibers, or Senac in the 1700s,3 who defined an internal
helix and surrounding transverse circumferential fibers, or
Krehls 1800s description4 of its powerful circumferential
fibers that cause cardiac constriction during ejection.
Physiological recordings of pressure and flow have clearly defined the impact of ventricular performance on these
variables, but their cause is determined by the function
of the underlying ventricular structure (Figs. 55.1A to C).

The Normal Heart

The Septum

The Right Ventricle

Other Consideraons

Cardiac movement had been analyzed by two-dimensional

(2D) methods like the ventriculogram and echocardiogram
that display its narrowing, shortening, lengthening, and
widening motions. Now, three-dimensional (3D) imaging
is available due to development of magnetic resonance
imaging (MRI) and speckle tracking imaging (STI;
Figs 55.2A and B), so that the natural twisting movement to
develop torsion and uncoiling to permit suction for rapid
filling becomes evident; these motions become impaired
by a spectrum of cardiac diseases. The newer 3D observations appropriately supplement the 2D measurements,
as all six movements have become accepted descriptors
of cardiac motion. Recognition of the form/function
relationship is essential in order to determine how the
interweaving circular and helical fibers cause them.
Just as the anatomist observes structure and deduces
function, those that use the echocardiogram have
observed motion and deduced structure. Solution to this
problem occurs when structure is understood, so that
function can be explained, and that is the goal of this
review. The infrastructure for explaining this macroscopic
structure/function relationship involves knowledge of
the functional anatomy of the heart. In 1942, Robb and

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1177

Robb5 summarized the findings of anatomists over five

centuries, and expressed generalized agreement that
the heart structure includes a helical configuration that
contains an apex, together with a circumferential muscle
mass that occupies upper two-thirds of the cardiac base.
Disagreement has existed as to the exact layering positions
that are occupied by the overlapping circumferential and
helical fibers. Grant,6 Lev,7 and Anderson8 have voiced
concern about how the microscopic connections between
the fiber tracts are always dislodged during manual
dissection.
Francisco Torrent-Guasp did a hand dissection of the
ventricles in his effort to define functional pathways, and
his work demonstrated that the unscrolled heart appeared
like a rope-like model when stretched from the pulmonary
artery to the aorta.9,10 His dissection demonstrated that the
intact heart contains two interconnected loops containing
a circumferential and helical muscle mass and his
configuration is called the helical ventricular myocardial
band or HVMB; this anatomy will be described in detail, as
it forms the basis of the structure/function analysis in this
review11 (Fig. 55.3).
The role of this functional analysis is to adhere to
Harveys functional anatomist requirement so that we
can integrate helical and circular fiber tracts in order
to explain reasons for the readily observed narrowing,
shortening, lengthening, widening, twisting, and uncoiling
motions. Current echocardiography movement analysis
demonstrates each of these motions, yet their conventional
state-of-the-art reports have consistently failed to
consider the circumferential muscle.1215 Conversely, the

The heart is a muscular pump that supplies blood

containing oxygen and nutrients to the body. This goal is
achieved by electrical excitation that produces sequential
ventricular emptying and filling. Figure 55.1 demonstrates
the physiological sequence of ventricular functiona
contraction phase to develop pre-ejection tension, ejection, and rapid and slow periods for filling. This report
relates the function to the underlying precisely described
muscular anatomy, thereby providing novel structural
explanations for the contractile sequence that causes the
ventricular directional motions of narrowing, shortening,
lengthening, widening, and twisting and uncoiling
(see Fig. 55.1A to C).
The observed functional patterns (see Figs 55.2A
and B) are documented by MRI and include an initial
global counterclockwise rotation and attendant narrowing
or cocking in the isovolumic contraction (IVC) phase
before ejection, followed by twisting of the cardiac
apex in a counterclockwise direction and of the base
in a clockwise direction as the ventricle longitudinally
shortens during the ejection phase, followed by a vigorous

B1

Figs 55.1A and B

functional HVMB provides an explanation for how the

interacting circular and helical fibers cause each of these
actions, as this information was gleaned from motion
studies using sonomicrometer crystals, MRI, diffusion
tensor magnetic resonance imaging (DTMRI), 2D echocardiogram, 3D STI, velocity vector imaging (VVI), and
radionuclide ventriculography.1618

BASIC HEART FUNCTION

1178

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

B2

B3

B4

Figs 55.1A to C: (A) Currently accepted time frames of systole and diastole, with measurements of intravascular pressure in the aorta,
left ventricle (LV), left atrium (LA), and LV volume, together with their impact on the mitral and aortic valves. Aortic flow occurs between
the two intervals that define ejection. The physiological phases of cardiac cycle that include isovolumic contraction, ejection, isovolumic
relaxation (to be questioned in this report), rapid and slow filling, and atrial contraction are shown; (B) Two-dimensional images of the
LV in a longitudinal view that shows the normal sequence of narrowing, shortening, lengthening, and widening of the ventricular cavity
during a normal cardiac cycle. Images were obtained by epicardial imaging in an open-chest porcine preparation. The phases of the cardiac cycle include end-diastolic state (B4), isometric phase (B1), ejection (B2), and isovolumic phase (B3). The broken-line markers are
within the ventricular cavity and define the transverse (between the midendocardial walls) and the longitudinal (from apical endocardium
to a line across the mitral annulus) dimensions. Muscle thickness is shown by the dark area adjacent to these intracavity dimensional
lines. The pale color is the cavity. The predominant changes exist with muscular thickening that narrows and widens the cavity rather
than the external wall dimensional changes. Note progressive muscular thickening (evaluated by wider distance between epicardial and
endocardial lines as myocardial mass narrows and shortens for ejection), together with maintained thickness as heart lengthens during
the rapid filling phase before substantial widening; (C) Twist of the heart: clockwise (below baseline) and counterclockwise (above baseline) motions of the base and apex, respectively, during the cardiac ejection and filling periods are represented in rotational degrees with
the use of speckle tracking with marker placed at the LV endocardial surface (Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA).
The relationships between the initial uniform and then reciprocal twisting motions of the base and apex during the pre-ejection, ejection,
and rapid and slow filling periods are explained in the text.

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1179

Figs 55.2A and B: (A) Magnetic resonance imaging (MRI) phase contrast velocity mapping (tissue phase mapping) of systolic and diastolic
cardiac frames with a temporal resolution of 13.8 ms during free breathing in a healthy volunteer. All motions are described in the text; the
arrows show the clockwise (marker to right) and counterclockwise
(marker to left) directions of transmural twisting motion during the
short-axis view and are obtained during isovolumic contraction, midsystole, isovolumic relaxation phase, and slower filling in mid-diastole;
(B) Differences in mean values for tracing radial, tangential, and longitudinal velocity motion, each 13.8 ms, for 12 volunteer subjects in whom
basal, mid, and apical segments are analyzed. Values above zero line
indicate contraction, clockwise motion, and shortening; below the zero
line, values define expansion, counterclockwise motion, and lengthening. The line expansion time is end systole (ES), with an average 320 ms
time frame. Note early radial expansion in basal segment (a), reversal of
twisting before end of systole (b), and supplemental late counterclockwise base motion during systole (c).

1180

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 55.3A and B: (A) Myocardial fiber organization. (a) Mall and MacCallums suggestion of bundles, which include deep (circular)
and oblique bulbospiral tracts. (b) Rushmers functional model, which includes the central transverse constrictor muscle and oblique
clockwise and counterclockwise layers. (c) Torrent-Guasps fiber trajectory model showing an upper transverse circumferential muscle
(or basal loop) surrounding the oblique right- and left-handed helical apical loop; (B) (a) Diffusion tensor magnetic resonance imaging
(DTMRI) studies where water is diffused parallel to fiber orientation, showing a helical positive or right-handed helix or clockwise (red)
and negative or left-handed helix or counterclockwise (yellow) muscle of myofibers reflecting circumferential or horizontal with a zero
helix angle. Note absence of circumferential or circular fibers in the septum, and how these zero angle helix fibers encircle the left and
right ventricles. (b) Dissected heart showing the circumferential or basal loop fibers encircling the left and right ventricles that are not
present in septum, and overlapping left and right helical fibers of the apical loop in septum.

global untwisting in a clockwise direction as the ventricle

lengthens and slightly widens during a phase interval
where no blood enters or leaves the ventricular chamber.
This untwisting motion continues into the rapid filling
interval, and finally a phase of relaxation exists during
diastole as heart widening continues during the slower
filling period before the atrium contracts during initiation
of the next organized beat. The helical and circumferential
muscle mass of the intact heart causes these movements,
and explaining how they cause these integrated motions
is our goal.

STATE-OF-THE-ART
The underlying myocardial muscle mass is composed
of helical and circumferential fibers, even though their
origins are uncertain.6,7,19 There is general agreement
from DTMRI studies that the basal two thirds of the left
ventricular (LV) free wall contains three layers of muscle.
These correspond to the layers defined by Streeter20 who
found that the inner 20% of the wall had subendocardial
fibers with an average angle of about +60, where the
positive sign indicates counterclockwise rotation above
the equator, the outer 25% of the wall had subepicardial

fibers with an average angle of 50 (clockwise rotation

below the equator), and the remaining 55% of midwall
muscle fibers had an approximately horizontal (equatorial
or circumferential) orientation. Streeter found that the
apical one third of the LV had no circumferential fibers,
but there is less certainty about the composition of
the septum. Many studies by DTMRI or polarized light
show three comparable layers,2124 yet others show only
two oblique layers without a circular component25,26
(Fig. 55.3B). The VVI method that will subsequently be
shown will confirm the presence of two oblique layers, as
this functional measurement provides best evidence of its
structural arrangement.17 Moreover, ultrahigh frequency
ultrasound functional studies show that these two septal
layers are separated by a thin midseptum bright echo line
partition, and that they contract independently during
thickening to develop a similar longitudinal motion.18
The thinner right ventricular (RV) side corresponds to the
subepicardial fibers of the free wall, and the thicker LV side
conforms to the free wall subendocardial fibers.
Based on studies by Streeter and previous investigators,
the noncircumferential fibers form helices16,27,28 that
are composed of oppositely wound oblique fibers
that comprise a right-handed arm within the deeper

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

(subendocardial) muscle and a left-handed arm that

occupies superficial (subepicardial) muscle. The HVMB
model of Torrent-Guasp shows that these right and left
helical arms form an apical loop; the right-handed arm is
called the descending segment and the left-handed arm
is called the ascending segment.10,29 Circular fibers with
transverse orientation19,29,30 surround the LVs and RVs, and
these are called the right and left segments of the basal loop
within the HVMB (Figs 55.4A and B). The HVMB model is
displayed in the major anatomy texts written by Clemente31
and by Moore and Dalley.32 The interaction between
the helical and circular fibers provide the mechanical
reasons for the rotational motions that are observed by
imaging studies and will be subsequently defined for
IVC, ejection, postejection isovolumic phase, and rapid
filling. Mathematic modeling by Sallin33 defined the vital
importance of fiber direction in causing function, as the
oblique helical fibers produce a 60% ejection fraction,
while the transverse orientation of the circumferential
fibers cause a 30% ejection fraction. The integrity of fiber
orientation is related to the extracellular collagen scaffold,
which governs muscle alignment, ventricular shape, and
size. The spiral fibrillar structure of endomysial collagen
supports the spatial distribution of myocytes by a weave
that ensheathes the HVMB structure described in detail34
as profound heart failure follows collagen scaffold damage
in hearts that do not have direct myocyte disease.35
The different muscular components contract asynchronously. For example, sonomicrometer crystal studies
show that the subepicardial muscle does not contract
during the isovolumic pre-ejection interval (IVC), both
subendocardial and subepicardial muscles contract
and contribute to torsion during ejection, and only
the subepicardial muscle continues to contract during
postejection isovolumic phase when untwisting or recoil
occurs.16,36 This scheme of asynchronous contraction
underscores the incorrectness of traditional thinking that
states that the ventricle contracts synchronously.
Dominance defines the governing muscular force
causing the directional and rotational motions during
each heartbeat, as the interweaving helical and circular
cardiac muscles co-contract and/or recoil during each
of the IVC, twisting, and untwisting phases. For example,
when considering the helical muscles, only the deep righthanded helical muscle contracts during IVC, whereas only
the superficial left-handed helical muscle contracts during
the isovolumic pre-filling phase. These helical muscles
are antagonistic, so that the contracting helical muscle

1181

is unopposed during each pre- and postejection phase,

yet one of them becomes the dominant muscle when the
entire helix co-contracts during ejection, whereby the
torque of the subepicardial muscle rotates the cardiac
apex counterclockwise, while the subendocardial muscle
contraction causes the basal clockwise motion that
produces shortening.16,17
Similar distinctions exist during shortening for
ejection, because the circular muscles cause compression,
yet the cardiac longitudinal dimension is reduced, thus
indicating that the helical fibers have dominant power to
oppose the elongation that would otherwise occur from
constriction, as they did during IVC. Furthermore, the
net counterclockwise and clockwise rotational directions
that exist during IVC and uncoiling (untwisting)16,17 are
governed by the most powerful component within these
overlapping circular and helical muscular components.

COMPOSITE OF STATE-OF-THE-ART
REPORTS
Prior imaging reports only address the helical component as a smooth change from a left-handed helix in
the subepicardium into a right-handed helix in the
subendocardium, without considering actions of the
anatomical circular fiber structure that remains the
centerpiece of anatomical descriptions (Fig. 55.5),4,3739
and whose presence is further confirmed by DTMRI
recordings25,26 (Fig. 55.3B). The mechanisms for twisting,
whereby the apex rotates counterclockwise and the
base rotates clockwise, has been based upon the Taber
model of a single helical layered architecture,40 where
obliquely aligned muscle fibers are embedded in an
isotropic matrix. This engineering design states that
torsion develops within each layer so that epicardial fiber
contraction rotates the apex in a counterclockwise and the
base in clockwise direction, while subendocardial region
contraction will rotate the LV apex and base in exactly the
opposite directions. In contrast, this description of torsion
within each right- and left-handed helix differs from the
current mechanistic descriptions by showing that torsion
develops between each arm of the helix, whereby there
is clockwise motion of the entire right-handed arm and a
counterclockwise motion of the entire left-handed arm16,17
(Fig. 55.6).
When both helical layers contract simultaneously
during ejection, the larger radius of rotation for the
outer epicardial layer provides a mechanical advantage

1182

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

B1

B2

B3

B4

Figs 55.4A and B: (A) Unscrolled myocardial band model of Torrent-Guasp that contains a circumferential basal loop and a helical apical loop.
Note (1) the transverse basal loop fiber orientation (be) representing circumferential fibers, and (2) the right- and left-handed apical loop helix
with predominantly oblique fibers, and (3) myocardial fold in (e) showing basal midwall twist to form the apical loop. The unfolded basal loop (d)
contains a right segment (RS) and left segment that surround the left and right ventricles. The septum does not have circumferential fibers. The
apical loop has helical fibers that form the right-handed helical arm or descending segment (DS) and left-handed arm or ascending segment
(AS). The unfolded myocardial band in (e) extends between the pulmonary artery (PA) and the aorta (Ao). Note (a) the intact heart contains
a circumferential basal loop wrap that surrounds the apical loop comprising helical fibers; (B) Architectural fiber orientation of (B1) intact heart
in upper left with circumferential fibers surrounding the inner helical fibers, (B2) detached circumferential fibers (basal loop) in upper right with
predominantly horizontal fibers compared with the conical apical loop containing right- and left-handed helical fibers in a helical design, (c) with
these segments super-imposed (top image); when the segments are separated (below), (B3) the right-handed helix or descending segment
(lower left) connected to the myocardial fold with oblique fibers aimed toward apex, and (B4) overlying left-handed helix or ascending segment
(lower right) with longer oblique fibers coursing toward aorta connection. This fiber orientation is used in all subsequent anatomical drawings.

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1183

Fig. 55.5: Conceptual cartoons of myofiber structural orientation from imaging and anatomy reports. The imaging drawing (left) separates the
left ventricle into a deep endocardium with right-handed helical clockwise fibers and a superficial epicardium with left-handed helical counterclockwise fibers. There is no circumferential or circular muscle. The anatomical drawing (right) displays similar right- and left-handed helical
arms in the deep endocardium and superficial epicardium regions but adds the prominent component of thick circular or circumferential fibers
that reflect the Triebwerkzeug described by Krehl.4 These circular fibers are considered to be constrictor fibers by anatomists.

(torque) to dominate the overall direction of rotation

toward apical counterclockwise rotation14,41,42 (Fig. 55.5).
Untwisting was ascribed to endocardial or right-handed
helix recoil in an engineering model that also predicts
(a) no difference between a cylinder and an ellipse model
(as also described by Ingels41 in intact hearts),20,30 and
(b) states that both endocardial and epicardial segments
cause systolic shortening to bring the base toward
the apex.12,15,40,43 Conversely, this analysis employs an
anatomically defined structure to define mechanical and
timing interdependence of twisting and untwisting, and
differs from current state-of-the-art reports. The right- and
left-handed helical arms will be called the descending and
ascending segments, respectively.
Imaging reports using 2D STI recordings describe
subendocardial clockwise motion and state the transmural
apical region moves clockwise, while the reciprocal
stretch of the subepicardium causes the base to move
counterclockwise during that interval. These 2D STI
observations differ from the MRI evidence of transmural
IVC apical counterclockwise motions16,44 (Figs 55.7A
to C). This disparity between different recording methods

has been ascribed to lower temporal resolution by the

same MRI modality that is simultaneously called the gold
standard of measurements. An alternate explanation is
that both methods are correct, but that this discrepancy
may be related to imaging analysis depth as MRI quantifies
transmural observations and STI focuses only upon tissue
moving in the same observation plane.16,17

DEFINITIONS
Rotation is the circular or angular movement of the
LV about its long axis, and by convention is defined as
clockwise or counterclockwise when looking up at the
heart from the foot of a supine patient. If the whole LV
rotates en masse, there is no torsion. Conversely, if apex
and base rotate in opposite directions, then torsion can
be assessed by the angular difference between them
(Fig. 55.8).
Twisting describes these differences without reference to a long-axis measurement. Untwisting expresses
the return of cardiac shape to its initial resting position.
Torsion defines the difference between the rotation of

1184

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 55.6: Structural reasons for torsion from bioengineering drawings (above) and anatomical structure (below). Comparable findings during torsion development are reported in bioengineering studies under conditions where myocardium structure is displayed
as either cylindrical (upper left) or conical (upper right), as the right-handed helical arm or deeper clockwise endocardium and is
covered by a left-handed helical or counterclockwise arm. Torsion is described as developing within each arm as shown by the
arrows in the cylinder on the right, and each arm develops clockwise and counterclockwise motion as shown on the left. The clockwise
layer is R1, counterclockwise layer is R2, and its larger torque causes apical counterclockwise rotation during torsion. Below, the anatomical structure shows a right-handed helical arm with clockwise motion (lower right) and a left-handed helical arm with counterclockwise motion (lower right) and these arms are called the descending and ascending segments of the apical loop. Torsion is described as
developing between helices, as the entire right- and left-handed helix move in different directions.

the base and apex of the LV relative to the long axis, exists
beyond the interval for systolic ejection, is measured
in degrees, and defining its duration is a vital part of its
measurement.

NOVEL MECHANICAL AND TIMING

INTERDEPENDENCE BETWEEN
TORSION AND UNTWISTING
Torsion and untwisting are transmural rotational
movements well seen by MRI.44,45 Mechanistic insight

into why these interweaving circular and helical fiber

components exert their dominant global action
becomes clearer by use of other imaging modalities such
as STI, VVI, and sonomicrometer crystals that define
regional analysis, and simultaneously characterize
their timing sequence.17,42,46 Harmonic interaction exists
during rotational movement that relates to the coiling and
uncoiling actions of responsible muscles; the uniformity
of global counterclockwise motion during IVC precedes
the apex and base differential motion during torsion,
and then recurs during untwisting as a uniform or global

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1185

C
Figs 55.7A to C: Motion of apex and base during isovolumic contraction by speckle tracking imaging (STI; upper) and magnetic resonance imaging (MRI) studies. (A) The STI study shows counterclockwise (above baseline) and clockwise motions of the base and apex,
respectively, during the isovolumic contraction. The speckle tracking with marker is placed at the left ventricular (LV) endocardial surface (Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA). Tracings from Aman Mahajan laboratory; (B) MRI studies showing global
counterclockwise motion of the apex and base during isovolumic contraction in both studies. Tagged MR images were acquired on a
1.5 T whole body MR scanner (Magnetom Sonata, Siemens, Erlangen, Germany) with a temporal resolution of 14 ms. The hatched line
following twisting (apex counterclockwise and base clockwise) marks peak apical rotation that exists just prior to the postejection isovolumic interval. Note: apex begins clockwise motion at this stage and prolongation of clockwise base rotation; global clockwise motion
occurs during this postejection isovolumic interval; (C) Velocity vector imaging (VVI) short-axis views of endocardial rotational motion of
six segments at the apex and base employing the (Sequoia 512, Siemens, Mountain View, CA, USA 4.0 MHz transducer) derived from
three-dimensional (3D) images displays counterclockwise motion of the apex and base during the isovolumic contraction (IVC) phase.
The hatched purple lines show both the end of the IVC phase where AVo is aortic valve opening, and the separated purple hatched lines
between AVc or aortic valve closure and MVo or mitral valve opening show the postejection isovolumic interval.

clockwise movement.16 Consequently, despite emphasis

upon their interplay during torsion and untwisting,14,15,47,48
understanding their interweaving muscular interactions
during the IVC interval is fundamental requirement in
order to understand mechanistic reasons for torsion and
untwisting motions. Moreover, untwisting cannot begin if
torsion is prolonged.

THE NORMAL HEART

The Left Ventricle
Isovolumic Contraction
Ventricular narrowing, elongation, and counterclockwise
net rotation characterize the pre-ejection phase.16,17

1186

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 55.8: Ventricular torsion displayed by speckle tracking imaging (STI) on left side (From Aman Mahajan laboratory) and magnetic resonance imaging (MRI) on right side (From Jurgen Hennig
laboratory), where twisting motions between the left ventricular
apex and base are displayed. STI study shows counterclockwise
(above baseline) and clockwise motions of the apex and base,
respectively, during the cardiac ejection. The speckle tracking with
marker is placed at the left ventricular (LV) endocardial surface
(Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA). MRI
phase contrast velocity mapping (tissue phase mapping) of systolic ejection cardiac frames with a temporal resolution of 13.8 ms
during free breathing in a healthy volunteer shows arrows that
demonstrate clockwise (marker to right) and counterclockwise
(marker to left) directions of transmural twisting motion during the
short-axis view.

Sonomicrometer crystal recordings during this 50-ms

interval display the contributions of individual muscle
masses by documenting that (a) the right and left sides
of the circumferential circular fibers shorten almost
simultaneously (10 ms delay between right and left
segments of basal wrap)16,49,50 with the subendocardial
descending segment helical fibers during IVC, and (b)
there is no ascending segment helical subepicardial fiber
shortening during that interval.49,50 STI measurements of
subendocardial shortening and lengthening during preejection systole provide further evidence of stiff outer
shell dominance.13 They show that the initial longitudinal
shortening is followed immediately by apical lengthening,
as the circular base compresses the inner helix; shortening
would otherwise occur if the co-contracting descending
segment was dominant. Moreover, the overlying noncontracting ascending segment fibers stretch during
ventricular elongation,42,51 so that they cannot be
responsible for global counterclockwise rotation of the
cardiac base during the pre-ejection interval. Previous

descriptions of fiber orientation of the underlying muscle

during IVC may be misleading, because only oblique
helical fibers were measured at the selected sampling;51
this analysis did not evaluate the circumferential basal
segment, whereby their horizontal orientation49,50 exerts
the compressive force that was just described.
MRI evidence of global counterclockwise motion
contradicts STI documentation of clockwise motion by
the right-handed helix or descending segment, and VVI
recordings derived from real time 3D echocardiography
in Figure 55.7C confirm this counterclockwise movement.
Global counterclockwise movement must arise from
the governing circumferential muscle fiber, because the
clockwise motion arising from descending segment is not
dominant. Anatomical analysis of circumferential muscle
mass dimensions16 shows why the thicker, left-sided basal
circular fibers exert the mechanical advantage causing
the dominant counterclockwise motion that accompanies
the ventricular narrowing that exists during LV cavity
compression (Fig. 55.9A).
Moreover, recent VVI recordings show that as the
compressed or narrowed LV chamber develops pressure,
the blood flow velocity from apex to base closes the
mitral valve,16 and there is simultaneous expansion or
rightward motion of the upper portion of noncontracting
ascending segment fibers within the septum (Fig. 55.9B).
This rightward movement, which resembles the bulging
of an aneurysm, occurs because of the presence of
noncontracting or relaxed ascending segment fibers within
this subaortic valve region that is anatomically uncovered
by the contracting right-sided deep descending segment
fibers, as shown in Figure 55.9B. Rotational motions
during IVC similarly relate to dominant interactions
because (a) the coiling of basal loops circular fibers that
causes cardiac compression, simultaneously produces a
net counterclockwise global movement that overcomes
clockwise rotation caused by the descending segment
fibers as intraventricular pressure rises before ejection,
especially because (b) the ascending segment muscle is
not shortening, so that it cannot contribute to pre-ejection
counterclockwise basal rotation. Most importantly,
the clockwise descending segment motion during IVC
produces a persistent clockwise rotational motion that
continues as torsion develops during the next phase of
ejection.

Torsion
The presystolic isovolumic interval is followed by
ventricular ejection, whereby torsion develops as the

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1187

B
Figs 55.9A and B: (A) On the left side is cranial view of the model of the helical ventricular myocardial band showing how the circular
and circumferential fibers or basal loop surrounds and embraces the conical right- and left-handed helix or apical loop. Note that (a)
circumferential fiber muscle thickness is greatest in the left component or segment, and thinner in the right component or segment and
(b) there are no circumferential fibers in the septum. On the right side are VVI images of isovolumic contraction (Sequoia, 512, Siemens,
Mountain View, CA, USA; 4.0 MHz transducer), where there is shortening of the entire circumference of basal loop, and of the righthanded helical armor descending segment. No left-handed arm or ascending segment shortening occurs, yet right-sided upper septum
motion exists in area of uncovered noncontracting left-handed helix or ascending segment as shown in B images; (B) Topographical view
of septum architecture in the wrapped heart where the right ventricle is intact (left side) and unwrapped form where the circumferential
fibers are separated. Note that the apical loop has a septum segment above the overlap of right- and left-handed helical fibers, where
the left-handed arm or ascending segment is the only muscle mass. This segment does not shorten during the isovolumic contraction,
and is the only segment that shortens during the postejection isovolumic phase (Fig. 55.8A, left panel and Fig. 55.11B, in a figure).

1188

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

LV cavity shortens and its wall thickness increases due

to deformation of all co-contracting circular and helical
fibers, as the ascending segment begins to contract.49,50
MRI and STI images13,15,16,44,46,52 document that the apex
and base twist in different directions, and VVI recordings16
show consistent inward systolic subendocardial motion
at the apex, midwall, and base to provide evidence that
contradicts theoretical concepts that counterclockwise
twisting exists within the subendocardial muscle.14,15
Compression is a central feature of the torsion
sequence as the LV shortens and twists to eject, because
such narrowing reflects the functional contribution of
circumferential or circular fibers, as well as being due to
helical fiber deformation. Conversely, shortening reflects
the principal coiling motion of oblique helical fibers
within the descending segment. The initial phase of torsion
furthers ventricular compression without imparting
substantial shortening, as the ascending segment fibers
only begin to contract at this beginning stage. VVI studies
during this phase at the onset of torsion are shown in Figure
55.1 (occupying approximately 50 ms or approximately
20% of the torsion interval at 72 beats/min), as they provide
insight into why the circular fibers remain dominant as
torsion starts. Figure 55.10 displays motion immediately
following the QRS wave on the electrocardiogram by
showing that (a) the septum twists (different motion of its
basal and apical components) as its inward velocity vector
reflects descending segment helical shortening, while the
simultaneously outward velocity vector motion is caused
by the ascending segment helical arm that is not covered by
circumferential muscle, (b) minimum shortening occurs
because the dominant circular muscle mass that occupies
the upper lateral ventricular wall becomes the governing
force of narrowing or compression, and thereby overrides
the counterclockwise motion of the underlying oblique
or ascending segment lateral LV free wall component,
whose shortening has just begun. Figure 55.1C also shows
that initiation of clockwise basal motion is delayed due to
ongoing counterclockwise motion of the circular fibers
that started during IVC, and (c), inward movement persists
within the lower lateral wall, because no circular fibers
exist in that region (Fig. 55.10).
The next phase of torsion during ejection involves
longitudinal shortening and reflects its most important
component. Helical fiber dominance governs this motion,
even though there is simultaneous compression arising
from deformation of co-contracting circular and helical
fibers, as well as from narrowing by the more horizontal

pathway achieved by the outer ascending segment helical

arm as the entire helix coils.16,17 The figure-of-eight spiraling
arms of these shortening vectors dominate (Fig. 55.11A), as
the descending segment fibers exert a downward velocity
vector direction toward the apex during their contraction,
while the ascending segment helical fibers are pulled
downward to follow a downward velocity vector toward
the apex caused by dominance of the descending segment;
the upward motion of the ascending segment only
becomes uncovered during the postejection isovolumic
phase, where the antagonistic descending segment stops
contracting. Consequently, shortening forces overcome
the predominant compressive action of the circular fibers
that existed when torsion was initiated.
There is complementary action between the reciprocally helical coiling forces that become maximal at the
apical vortex,53 because the circumferential basal fibers act
as a buttress to prevent explosion or unlimited expansion
of the vortex forces of helical fibers basal components as
they develop reciprocal outward forces to balance the
downward motion toward the apical vortex (Fig. 55.11B).
From a rotational aspect, the torque from the larger
curvature of the ascending segment helix rotates the
apex counterclockwise, while the more forceful strain in
the descending segment helical fibers16,36 continues the
clockwise rotation of the base that began during the IVC.
The extent of deformation and strain increases toward
the LV apical vortex, which is formed by the helical fibers
that exist without any surrounding circumferential muscle
at the apical region.17,54 The endocardium of the entire
septum, including its apex, midwall, and base, displays
a consistent leftward motion16 to provide evidence that
contradicts suggestions of separate torsion development
within the subendocardial layer and the subepicardial
layer.14,15,40 Instead, torsion and shearing relate to the
entire descending segment or right-handed helix rotating
clockwise and to the entire ascending segment or lefthanded helix rotating counterclockwise (see Fig. 55.6).

Postejection Isovolumic Phase

The isovolumic interval follows ejection and is characterized by untwisting, lengthening, and widening.16,17 This
interval was previously called the isovolumic relaxation
phase (IVR), but ongoing shortening of the ascending
segment continues (albeit at a reduced force so that no
ejection occurs), so that the term postejection isovolumic
interval is a more precise description. The lengthening
and widening components are 2D echocardiography

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

observations that do not define the 3D geometry required

to maintain a constant volume. Insight into this geometric
change is provided by echocardiography studies that
document how the mitral annulus becomes less oval,55
thereby explaining how an unchanged volume is
maintained as the heart lengthens and widens during this
interval.
The reciprocal relationship of contributions from
circular and helical muscles becomes revealed by comparison of their pre and postejection isovolumic volume
interactions. Before ejection, the entire circumferential
muscle and only the descending segment helical arm
shorten, without shortening of the ascending segment
helical arm. In contrast, during the postejection isovolumic
phase, shortening stops in the left and right segments of
the circular muscles and in the descending segment, but
continues in the ascending segment. There is normally
a 80- to 90-ms timing hiatus between the cessation of
the descending segment shortening and the time when
the ascending segment stops shortening (Fig. 55.13A).
Disturbance of this normal relationship during torsion
affects untwisting because apical untwisting cannot begin
if torsion is extended by prolonged descending segment
coiling.
Recoil is determined by when shortening stops.
Regional and global motion depends upon the dominance
of recoiling contributions of the interweaving circular
and descending segment arms of the helix. This recoil
process is attributed to expansion of the Titin and collagen
pathways that were compressed during ejection.56 During
the isovolumic phase, rotation during recoil reflects the
dominance within the overlapping circular and helical
muscle groups, because the ascending segment is still
shortening and thereby cannot contribute to this process.
Cardiac spatial configuration is a vital factor in the
untwisting process because the LV wall would otherwise
implode if only helical fibers caused this untwisting
motion that precedes suction during rapid filling. This
dynamic collapse is prevented by the support of the
stiff outer shell formed by circular fibers that maintain a
circumferential buttress (Fig. 55.11B). The interplaying
forces of three sets of muscle motions determine the net
or global clockwise rotation of the apex and base that
exists during untwisting. First, the ascending segment
cannot be responsible because it contributes an ongoing
counterclockwise motion as it continues to shorten, but
with diminished force during that interval.36,49,50 Second,
the descending segment arm recoils counterclockwise,
a motion that contrasts with its clockwise movement

1189

during torsion.16 Third, the circumferential or basal loop

contributes a dominant clockwise motion, which of course
contrasts to its counterclockwise movement during the
IVC phase; ventricular widening is also caused by these
recoiling circular fibers.
Lengthening is evident from the elegant studies of
Karwatowski,57 who used MRI and echocardiography
to demonstrate that isovolumic long-axis lengthening
preceded flow across the mitral valve by 46 ms. Lengthening
during untwisting is determined by both the left- and righthanded arms of the helix or the ascending and descending
segments of the apical loop. One component is the ongoing
contraction of the ascending segment that had become
more spiral during torsion. The helical coil becomes more
taut, so that this segment becomes thicker and straighter
(thus longer) when the counterforce of the right-handed
arm becomes removed after its shortening stops.16 In
nature, this resembles the mechanisms within the snake,
which elongates before striking due to differences in the
contractile sequences in paraspinal muscles (Figs 55.11A
to C). The second lengthening component involves returning the recoiling right-handed helix or descending
segment to its neutral and thus uncoiled longer helical
position (Fig. 55.11C).
The untwisting sequence during the postejection
isovolumic phase mirrors torsion by having two
components. The first phase begins just before the aortic
valve closes57,58 and is characterized by reduced torsion and
continued shortening as the ascending segment continues
to shorten (Fig. 55.12A). Moreover, VVI demonstration
that the ascending segment arms directional vector points
toward the apex does not contradict our prior suggestion
that the ascending segment exerted an upward force.
Instead, this force is overcome by the dominant righthanded arm during torsion.16
An intriguing imaging parallel exists between the initial
phases of IVC and untwisting (Figs 55.8A and 55.12B in its
left panel), whereby the same VVI image results are related
to entirely different causes. During untwisting, the early
appearance of lack of significant motion of the lateral wall
and lower septum relates to their absence of contraction,
whereas this same image reflects shortening of the circular
muscle and descending segment during IVC. The upper
septum beneath the aortic valve displays right-sided
and downward motion during untwisting that reflects
the counterclockwise motion from ongoing ascending
segment shortening. In contrast, this right-sided vector
during IVC reflects the bulging of this noncontracting

1190

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 55.10: Beginning of torsion (upper left) with co-contraction of base and entire helix. There is essentially no longitudinal ventricular
shortening at this time. Note (a) twisting septum with upper septum showing left-sided motion of the right-handed arm of the descending
segment and lower septum, and lower lateral wall has right-sided motion of left-handed arm or ascending segment, and (b) upper lateral
wall has essentially no motion as it is compressed by shortening circular basal muscle, which does not exist in the septum, and (c) the
lower lateral wall has no circumferential compression and shows leftward motion of left-handed arm or ascending segment. Slightly later
in torsion (upper right), with more twisting of septum, and increased left-sided motion of lateral wall that is occupied by the more fully
contracting left-sided arm or ascending segment. The lower images show the responsible architecture, whereby the lower left shows
the wrapped heart, where the uncovered left-handed helix displays more prominent counterclockwise motion. The lower right displays
unwrapped heart to show how the circumferential muscle covers the same upper lateral wall to exert a compressive force and limit early
rightward counterclockwise motion.

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1191

C
Figs 55.11A to C: (A) Velocity vector imaging forces during maximal torsion during ejection as longitudinal shortening occurs. Vector
angulation is directed toward the apex, as the helical left- and right-sided arms dominate to change force direction toward the apical vortex, despite ongoing circumferential or circular muscle shortening; (B) Drawing of simulated cardiac anatomy with circumferential wrap
and internal helix (lower left) with spiral motion for ejection (in center) and suction (lower right). The outward spiral forces with an apical
vortex during ejection would expand the basal wall laterally to potentially cause explosion. Lower right shows suction where inward
forces at the base would cause implosion. This circumferential wrap at the base becomes a buttress to prevent these events, and this
configuration resembles a gothic cathedral (upper center) where the buttress protects the downward forces of the dome from loss of the
base due to downward forces from the tip at the peak of the dome; (C) This drawing shows normal anatomy (upper left) with right and
left segments of basal loop [right segment (RS) and left segment (LS)], and right and left helical arms of apical loop or descending and
ascending segments [descending segment (DS) and ascending segment (AS)]. The bottom shows the coils in the right-handed helix
arms in diastole, ejection, and isovolumic phase. Note that (a) both shorten during ejection, but the descending segment is stronger,
(b) ascending segment becomes more horizontal as spiral shortens, and (c) ascending segment continues shortening during isovolumic
phase and causes elevation or lengthening. This action mirrors cobra shown in upper right that lengthens as its spiral becomes elongated in its pose before striking (Fig. 55.11A).

region due to increased intraventricular pressure

(Fig. 55.8A compared to 55.12B in its left figure).
The second untwisting component involves the
prominent left-sided vectors (Fig. 55.12B, panels b
and c) that match similar rotational motions recorded
by MRI and STI imaging methods.15,44,46,59 No flow crosses
the mitral valve during this counterclockwise movement,

even though the valve cusps may be open. Untwisting

reflects the opposite of twisting, yet global motion
shows no differential differences between the apex and
base, as both segments rotate clockwise (MRI image).
Consequently, the term untwisting is not matched by
global differential rotational action. A better term may be
either unwinding, uncoiling, or recoiling as these terms do

1192

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

B
Figs 55.12A and B: (A) On the left, tracings of endocardial and epicardial sonomicrometer crystals placed into the fiber orientation
pathways of the right- and left-handed helix, or descending and ascending segments in anterior myocardium of open-chest pig. The
solid line shows the beginning and ending of the right-handed helix shortening, and the hatched lines show the left-handed helix or
ascending segment. The postejection isovolumic phase (within yellow color overlay) shows (a) approximately 80 ms time hiatus, and
(b) lengthening of the right-handed helix as the left-handed helix or ascending (Asc.) anterior fibers are still shortening; the left ventricle
(LV) pressure and dP/dt tracings indicate the timing. On the right is unfolded myocardial architecture showing left- and right-handed
or ascending and descending segments of the helical ventricle, surrounded by the circumferential muscle of the basal loop. Note that
(1) central ventricular cavity is composed of overlapping left- and right-handed helices or ascending and descending segment fibers in
the septum region, (2) left-handed helix wraps around and overlaps the right-handed helix in septum, and (3) absence of overlap in the
lateral wall, which is composed of the left-handed helix or ascending segment. The lack of overlap in the left-handed helix or ascending
segment also occurs in the septum, below the aortic valve, as displayed in Fig. 55.9B; (B) In (a), beginning of postejection isovolumic
phase with right-sided motion of the upper septum, where there is ongoing shortening of the left-handed arm or ascending segment
without overlap of right-sided arm or descending segment. Simultaneously, there is essentially no motion of the lower septum or lateral
wall, where the circular and right-handed arm and descending segment fibers just stopped shortening. In (b), slightly later in postejection
isovolumic phase, there is left-sided motion of the septum and lateral wall due to recoil of the circumferential or circular basal muscle,
and the upper septum now shows similar left-sided movement despite the ongoing shortening of the left-sided arm or ascending segment that just showed right-sided motion before these recoiling forces became dominant. In (c), end of postejection isovolumic phase
with both septum and lateral wall showing left-sided movement as dominant recoil exists in the circumferential or circular base, which
thereby counteracts the simultaneous counterclockwise recoil of both the right-handed helical arm or descending segment and ongoing
shortening with counterclockwise motion of the left-handed helical arm or ascending segment.

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

not require the differential action that would be needed if

untwisting is used. The dominance of recoiling by circular
or circumferential fibers that cause this counterclockwise
motion parallels how these circular fibers also cause
dominant global counterclockwise rotation as they coil
during the IVC interval. Consequently, a balance becomes
apparent between fiber orientation and rotational motion
before and after torsion. The circumferentially controlled
global motions before and after torsion effectively
surround the twisting motions of the differential clockwise
and counterclockwise rotations of the base and apex
during torsion, which are principally determined by the
helical fibers.
Untwisting during elongation sets the stage for rapid
filling by creating a deceleration of ventricular pressure
and circumferential force that creates a potential vacuum
that causes suction after ventricular pressure falls below
atrial pressure.16,57 The causative mechanism of untwisting
during rapid filling differs from recoil of the noncontracting
circumferential fibers during the postejection isovolumic
phase, and relates to recoil of the left-handed arm fibers
that starts immediately after they stop shortening.
Consequently, the left-handed helix or ascending segment
cannot be the cause of the initiation of untwisting during
elongation59 because it maintains strain, continues to
shorten,16 and its counterclockwise motion is maintained
until its contraction stops. Under normal circumstances,
such ascending segment shortening is normally called
post-systolic contraction, a term that shows why the
term IVR is inaccurate.16 This early postejection time
interval for post-systolic contraction is extremely important in determining the interdependence of torsion and
untwisting, because prolonged descending segment
shortening is caused by the right-handed helical arm due
to a spectrum of causes, and will interrupt the onset of
untwisting and interfere with rapid filling patterns that are
subsequently considered under clinical implications.6063
The aforementioned temporal and mechanical factors
underlying untwisting cause a dynamic geometric change
in ventricular size and shape that result in a series
of imaging and hemodynamic changes that include
measuring the rate of untwisting48,59 as well as tau (change
in time related to change in deceleration in LV pressure)64
and development of an intraventricular pressure gradient
that becomes maximal immediately after the rapid filling
that follows the postejection isovolumic interval.65 Each
factor is a result of the functional geometric change that
produces such measurements.

1193

Figs 55.13A and B: (A) First phase of rapid filling with elongation
(note apical lengthening) and further leftward motion of the septum and lateral wall as there is recoil of the left-handed helical arm
or ascending segment, together with termination of recoil or the
circumferential or circular base and right-handed arm or descending segment whose forces interacted during the preceding postejection isovolumic phase; (B) Completion of the rapid filling phase,
whereby increasing ventricular volume is the dominant force as
the left ventricle (LV) chamber further lengthens and widens. Velocity vector imaging (VVI) displays expansion and outward velocities because filling forces overcome recoil action in septum and
lateral wall.

Rapid Filling
Untwisting has two components, as the initial unwinding
occurs during the postejection isovolumic interval and
is dissociated from the untwisting, causing rapid filling
but the causes are tightly interrelated.66 Early untwisting
during the postejection isovolumic interval is the reason
for subsequent suction, despite this temporal separation;
recoil continues from a different cause (the ascending
segment) during the subsequent phase of rapid filling
that develops after ventricular pressure falls below atrial
pressure (Figs 55.13A and B). The first phase of untwisting
is characterized by transmural clockwise motion that is
initially caused by dominant recoil of circular muscle
during this postejection isovolumic interval. The second
phase develops from elastic recoil of compressed titin coils
within the left-handed helix or ascending segment fibers.
The interaction of these dual recoiling forces is critical for
suction, because 5060% of untwisting normally occurs
before the rapid filling phase.64,67 Moreover, a fundamental
component relates to the normal 80 ms timing hiatus
between the completion of shortening of the descending
segment and the later completion of shortening in the
ascending segment. Suction increases if inotropic drugs

1194

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

enhance isovolumic phase untwisting,48,59 or diminishes

if untwisting is delayed by prolonged torsion when
shortening of the descending segment extends torsion
during this temporal hiatus interval.16,63
Unwinding of the apex to return to its original position
in order to create suction for rapid ventricular filling
requires relaxation of all muscle segments, so that the
isovolumic interval untwisting component becomes an
essential prelude to this process. Conversely, prolonged
descending segment shortening allows ongoing torsion
during the isovolumic interval, thereby diminishing
this response, retards LV pressure deceleration, reduces
ventricular compliance, and impairs the 5060% of filling
that normally occurs during this period.67 Augmented
filling pressures are then needed to achieve proper enddiastolic volume after cessation of the apical clockwise
unwinding, in order to stretch the LV satisfactorily during
the later slower or passive filling phase.

THE SEPTUM
The ventricular septum is a thick structure composed
of discrete muscular bands that separate the LV and RV.
The septum comprises approximately 40% of ventricular
muscle mass and contributes to biventricular cardiac
function.68 Analysis of this structure/function relationship
requires a full understanding of how existing normal
anatomical form translates into hemodynamic performance. Satisfactory accomplishment of this task shall
answer the 1790 supposition of Weber,19 who indicated
that actions of muscular heart would not be understood
until the muscle bundles of the septum are clarified.
Our initial experimental evaluation of septal structure/
relationships was acquired by use of sonomicrometer
crystal measurements that demonstrated how fiber
orientation determines the maximum rate of systolic
shortening. Findings validated the hypothesis that the
configuration of septum anatomy conformed to the
descending and ascending segments of the HVMB, as
described by Torrent-Guasp11 (Figs 55.3 and 55.4). This
spatial composition has been recently supported by
DTMRI recordings (Figs 55.14A to C).25,26 Oblique fibers
of the endocardial regions of the left and right sides of the
septum displayed the same functional characteristics that
exist within in the free LV wall, thereby confirming the
spatial structural configuration required for development
of twisting.

The interaction between noninvasive methods and

structure is enhanced by conventional low-resolution
ultrasound imaging of the working ventricular septum,
which has previously identified a hyperechogenic
septal line that matches the septal separation line,
which runs in a basalapical direction (Figs 55.15A
and B)18 as demonstrated by postmortem contrast
tomography studies by Lunkenheimer et al.27 Highresolution ultrasound imaging allows identification of
the structural and functional separation of the ascending
and descending septum components along the previously
observed septal line, along with temporal and sequential
movement of these muscle layers toward the respective
ventricular cavities (Figs 55.16A and B). Visualization of
different fiber orientation in the working heart using highresolution echocardiography strongly supports the helical
anatomical models displaying the muscle bands that
form the ventricular septum and free LV wall. Figure 55.17
displays similar functional contractions of the descending
and ascending segments that are similar between these
structures.
Recent animal studies in the working heart, using
higher magnification of this septum midline in porcine
and rabbit models, document that a space exists between
the edges of the septal line (Fig. 55.16B).18 The line is
approximately 100 microm (0.10 mm) wide, and its thinedge components attach to the overlying ascending
and descending segments of the septum muscle. The
septum muscle on either side of this line in the working
hearts shows a relative uniformity that depends on the
echocardiogram probe placement position in relation to
fiber orientation planes that pass along or across working
muscle (Fig. 55.16B). Further analysis of the midseptum
line demonstrates that the space between its edges (a) is
retained during systole (when intramyocardial vessels
are collapsed by the surrounding contracting muscle);
(b) is unchanged during early diastole (when flow
through vessels is greatest and would expand the space
if it was vascular); and (c) becomes nearly obliterated
when function of the overlying ascending and descending
segments is removed during cardiopulmonary bypass
by inducing ventricular fibrillation, and completely
obliterated or cardiac arrest by cardioplegia (Fig. 55.16B).
The space between this line conforms to the pathway
followed by Torrent-Guasp during his cardiac dissection as
he separated the ascending and descending loops during
postmortem analysis. Most importantly, the collapse of

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1195

Figs 55.14A to C: (A) Fiber orientation relationship of the septum,

composed of oblique fibers that arise from the descending and
ascending segments of the apical loop, surrounded by the transverse muscle orientation of the basal loop that comprises the free
right ventricular (RV) wall. Note the conical arrangement of the
septum muscle and the basal loop wrap, forming the RV cavity; (B)
(a) Diffusion tensor magnetic resonance imaging (DTMRI) studies,
where water is diffused parallel to fiber orientation, showing a helical positive or right-handed helix or clockwise (red) and negative or
left-handed helix or counterclockwise (yellow) muscle of myofibers
reflecting circumferential or horizontal with a zero helix angle. Note
absence of circumferential or circular fibers in the septum, and how
these zero angle helix fibers encircle the left and right ventricles.
(c) Dissected heart showing the circumferential or basal loop fibers
encircling the left and right ventricles that are not present in septum,
and overlapping left and right helical fibers of the apical loop in septum; (C) Diffusion tensor magnetic resonance imaging (MRI) from
the work of Zhukov and Barr26 showing the helical inner or endocardial (clockwise) and outer or epicardial (counterclockwise) fiber
orientation (in purple and blue colors) and a central left ventricular
(LV) free wall that is white to reflect a more horizontal or very small
angle pitch that does not involve the septum.

Figs 55.15A and B: (A) Cross-section images demonstrating the oblique crisscross endocardial and epicardial fibers contained within
a circumferential midseptal wall; (B) Computed tomography scans demonstrating the interweaving collagen support of the connective
tissue skeleton that is likely the scaffold for reciprocally oblique septal muscular fibers. Note the space between the two septum regions.

1196

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

B1

B2

B3

B4

Figs 55.16A and B: (A) Low- and high-resolution echocardiogram

showing the mid-hyperechogenic and midseptal line; (B) High-resolution ultrasound image of the septum at the base of the heart
acquired using high ultrasound transducer frequency (12 MHz).
Septal images showing a bilayer structure with an inner dimension
of 100 to 150 mm. B-mode or echocardiographic pattern of the
septum on either side of the septal bilayer is different, demonstrating the different directionality of the myocardial fibers on the
respective sides of the septum. The septal bilayer is recorded during a normal cardiac cycle, during ventricular fibrillation, and during
cardiac arrest.

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1197

Fig. 55.17: Comparison of ultrasonic crystal tracings of descending and ascending segments of left ventricular (LV) free wall, and
M-mode and Doppler M-mode imaging of the septum. The beginning and end of descending segment shortening and motion (solid lines), and the ascending segment (hatched lines). Strain in the right (red) and left (blue) sides of the septum is noted in systole. M-mode shows displacement of the left and right sides of the septum toward their respective ventricular chambers. Note the
delay of initiation of ascending segment and right septal motion and lengthening of descending segment during phase after ejection
and continuing displacement of the right side of the septum toward the right ventricular (RV) cavity, despite the beginning of LV cavity
expansion. (LV: Left ventricle).

this space between the border edges of this midseptal

line precisely reflects the conditions encountered by the
anatomist or pathologist in the cadaver or biopsy specimen.
Structural differences between dead vs live conditions can
lead to artifacts, but matching form to performance must
remain the goal of the functional anatomist.
We concur with problems related to cadaver dissection
limitations that were suggested by Grant,6 and Lev and
Simkins,7 and that are now supported by Anderson et al.8
An example is the incorrect posterior papillary muscle
position that accompanies the myocardial band during

Torrent-Guasps unfolding diagrams.50 Torrent-Guasp

et al. realized this error when they were made aware of
the 1971 functional studies by Armour and Randall.69
Their subsequent dissections display the proper location,
and they encouraged valid correlation of structure and
function during efforts to understand architectural reasons
for living heart motions.
The physiological implications of these observations
is that the oblique nature of the septum structure is a
vital component needed for generating the twisting
motion required for efficient ventricular ejection against

1198

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

increased peripheral vascular resistance. In contrast,

constriction or bellows action is the predominant
function of the basal loop as the result of its predominant
transverse fiber orientation, because its circular fibers
surround the LV and RV septum. Consequently, rightsided heart function may become impaired after loss of
sequential septum contraction with attendant pulmonary
hypertension, an effect that sometimes follows septum
hypokinesia or akinesia or dyskinesia in cardiac surgical
procedures with impaired myocardial protection,70 or after
temporary ischemia, or when the septum is stretched after
LV or RV volume overload. Conversely, recognizing and
using knowledge from this formfunction relationship
has resulted in developing innovative RV reconstructive
procedures that restore the septum into the midline
position, recover its twisting action, and result in favorable
clinical outcomes.71

THE RIGHT VENTRICLE

RV architecture involves two components. First, the free
wall is predominantly composed of a basal loop containing
transverse fibers, which constrict or compress the
chamber. Second, the septum contains helical fibers with
an oblique orientation that cause a twisting movement;
there is no septum transverse component (Figs 55.18A
and B). Comparison of RV and LV architecture reveals
marked differences because the RV has no global helical
configuration, even though its outflow tract free wall
contains oblique ascending segment fibers that TorrentGuasp termed aberrant fibers.11 The septum is a central
biventricular helical structure, rather than a LV structure.72
The interaction of its free walls predominant transverse
fibers and septums oblique fiber orientations determine
RV function, which was inaccurately called a bellows
action due to (a) free walls horizontal fibers that constrict
or compress the RV chamber against the septum73 and
(b) the incorrect notion that the septum was a LV
structure.72 A twisting action is needed, especially against
increased pulmonary vascular resistance (PVR), and that
movement is provided by the septums helical fibers that
becomes quantified by its shortening and lengthening
movement.
Prior concepts that RV has a bellows-like action
are related to measurement from septum to free wall
dimension by ventriculogram,73 but these 2D recordings
can only demonstrate narrowing, shortening, lengthening,
or widening movements. In contrast, 3D measurements

are needed to define the septums twisting capacity.

An example of this interface is shown by the way that
circumferential basal loop constricts the RV during IVC,
an action that precedes the septal shortening that occurs
during ejection when afterload is encountered. Wiggers
in 191474 showed that IVC compressive movements were
unaltered by RV afterloading because the pulmonary valve
did not open during this interval. In contrast, inotropic
drug stimulation directly affected circumferential free wall
muscle contraction to accentuate function.74
The interaction between septum and free wall
performance has been experimentally tested by investigators who demonstrated that RV performance was
not significantly impaired by either cauterization of the
entire RV free wall,75 its replacement by a semirigid patch
material,76 or regional ventricular fibrillation after its
isolation,77 so long as the septum was intact. Conversely,
RV failure developed if the septum was either cauterized,
made ischemic by further dye embolization after right
coronary artery occlusion, or damaged by pulmonary
hypertension;78 each intervention deteriorated performance by interrupting the natural septal wringing motion.

OTHER CONSIDERATIONS
Subendocardial Muscle; Correct
Anatomical Location But Architectural
and Functional Confusion
Subendocardial muscle mass surrounds the LV inner
surface, and drawings14,15,40,51 (Fig. 55.19) left side of the
transmural ventricle imply a circumferential line that
bisects the LV wall to separate the deeper oblique clockwise
fibers of the descending segment or right-handed helix
from the overlying counterclockwise ascending segment
or left-handed helix that occupies the outer LV shell. In
contrast, anatomical studies show that different parts of
right- and left-handed helices form the circumferential
subendocardial muscle.10,11,31,32 This difference conveys
a different functional effect, as previously described
during cardiac motions of the normal heart. The dissected
or unraveled cardiac architecture shown in Figs 55.4
and 55.9 shows that the upper septal oblique orientation
differs from reciprocally oblique fibers of the lower
septum, and that the oblique lateral ventricular wall
subendocardial fiber arrangement mirrors that of the
subepicardium, because there is no overlap of descending
and ascending segment helical fibers (Figs 55.9 and 55.10).

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

A1

A2

A3

A4

VVI recordings in Figures 55.9A and 55.12B demonstrate

dissimilar subendocardial motion during the time frames
of IVC, twisting for ejection, and untwisting before rapid
filling. Presumptions of subendocardial function that are
only based upon bioengineering models containing a
uniform or homogeneous inner shall wrap40 may provide

1199

Figs 55.18A and B: (A) Model and anatomical preparations

showing the orientation of the ventricular myocardial band of the
(A1 and A2) intact heart and (A3 and A4) after exposing the septum
by unfolding of the right ventricular (RV) free wall. Note the similar
configuration of the septum and left ventricular (LV) free wall composed of the ascending segment of the apical loop; (B) Anatomical
unwrapping of the right segment of the basal loop, which surrounds
the septum composed of the helical fibers of the descending and
ascending segments of the apical loop.

confusing conclusions. Dynamics of the anatomically

visible subendocardial muscle are linked to both an
architectural configuration that evolves from its left- and
right-handed cardiac helical form, as well as from how the
wrapped circular muscles within the upper LV influence its
motions. The global counterclockwise rotation during IVC,

1200

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 55.19: The left drawing shows a bioengineering concept of circumferentially overlapping endocardium and epicardium, whereby the
endocardium reflects the right-handed helical arm and surrounds the left ventricular (LV) inner surface. The right architectural reflection
of the anatomical endocardium shows that it is formed by both the right- and left-handed helical arms, and has fiber pathways that have
both clockwise and counterclockwise directions. This anatomy is described in Figure 55.10.

despite clockwise motion of the endocardium becomes

explained by such insight into powerful circular muscle
during normal cardiac architecture.

Torsion and Untwisting/Preload and

Afterload Relationships
Changes in torsion and untwisting within normal conical
hearts has been defined in regard to alterations in preload,
which increases them due to volume dependency, and
raised afterload that increases torsion while reducing
untwisting.15 Conversely, dilated and failing hearts have
a more spherical form and exhibit completely different
torsion and untwisting responses following similar
hemodynamic loading alterations.79 Consequently, LV
geometric alterations influence motion observations
in a manner that is independent of loading conditions,
because cardiac fiber orientation is primarily responsible
for such rotational actions.80,81 van Dalen reinforced this
observation by showing that LV sphericity index is the
strongest independent predictor of apical rotation and twist
when comparing normal subjects with an elliptical cardiac
shape against a cohort with dilated cardiomyopathy.79
Figs 55.20A and B shows such architectural changes when
the conical form becomes spherical, and implies that

changing the natural 60 angulations of the right- and lefthanded helical components toward a more horizontal82,83
configuration will geometrically alter functional twisting
and untwisting. For example, Borg recently examined
changes after increased preload and reduced afterload
in patients with mitral insufficiency (whose regurgitation
causes these intrinsic spherical ventricular shape changes)
and demonstrated decreased torsion and reduced
untwisting.84 Moreover, initiation of the untwisting
movement began 23 ms before aortic valve closure. This
reliable echocardiographic finding, thereby poses a series
of questions about responsible muscular mechanisms that
produce such reproducible changes. Untwisting normally
begins during the postejection isovolumic phase, yet mitral
valve insufficiency continues after aortic valve closure58 to
thereby support the functional role of ongoing ascending
segment contraction during an interval that previously
was called IVR.

Mitral Valve Opening Relationships

with Untwisting
Echocardiographic calculations of torsion and untwisting
are traditionally related to mitral valve opening (MVO)
alterations.15,59,85 This terminology is based upon Doppler

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1201

Figs 55.20A and B: Comparison of fiber orientation in the normal heart (above) and the dilated or spherical form (below) where there
is detachment of the circumferential or basal loop with horizontal fibers, and exposure of the normal and spherical configuration of the
right- and left-sided arms of the helical structure. Note that the normal 60 fiber orientation becomes more horizontal in the spherical
configuration and this angulation begins to resemble the more transverse fiber pathways of the circumferential or basal loop.

inflow and outflow recordings, rather than conforming

to knowledge of the separation of the mitral leaflets,
which provide the only valid confirmation of MVO. Lee
in 199058 called this MVO observation the mitral valve
artifact that correlates with the E point in the mitral
echogram, but is unrelated to actual mitral valve opening.
Moreover, concepts of untwisting existing during the IVR
interval that are based upon this MVO observation need
reevaluation, because subepicardial muscle continues to
contract and myocardial strain is maintained during this
phase.16,59 Untwisting during the postejection isovolumic
phase is due to uncoiling of the transversely oriented
circumferential muscle surrounding the cardiac base
that both rotates and houses the helix from which the
papillary muscles arise. Ventricular untwisting caused
by these horizontal fibers may simultaneously open the
mitral valve leaflets by changing papillary muscle position,
and thereby may explain Lees 1990 findings.58 Moreover,
untwisting simply cannot start if there is ongoing torsion,
as exists in a spectrum of diseases to be subsequently
discussed. Mitral valve inflow (MVI) is a more accurate
term than MVO, because this term dissociates the earlier
existing anatomical observation of leaflet separation from
a later flow effect that is only initiated during the rapid
filling phase.

Clinical Implications
Recognizing how the interdependence of torsion
and untwisting relates to underlying mechanics and

timing provides insight into how measuring torsions

peak, velocity, or rate and duration uncovers their
interconnection. Torsion duration is an essential feature
that improves a fuller understanding of untwisting,
because its knowledge integrates with the vital timing
hiatus that exists between the end of descending
and then subsequent ascending segment shortening.
Untwisting is unchanged if this interval mirrors the
approximately 80-ms interval existing at normal heart
rates. Conversely, untwisting accentuates if this interval
is extended by positive inotropic drug intervention,59 or
becomes impaired if this interval is shortened by either
negative inotropic drug intervention59 or by several other
factors described below. Extending torsion by prolonged
shortening of the right-handed helical arm or descending
segment will impair untwisting because this delay
compromises the postejection isovolumic time frame
when unwinding should start. Consequently, a unifying
influence of mechanical events during this hiatus time
frame evolves into a torsion/untwisting interdependence
that plays a major role that results in diastolic dysfunction.

Diastolic Dysfunction
The term diastolic dysfunction has been used because
heart failure occurs in patients with normal ejection
fraction, implying that the problem is diastolic in origin.
However, Tan86 has done an extensive echocardiographic
analysis and emphasized that it is not an isolated diastolic
disorder; each patient displays combined systolic and

1202

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Myocyte Factors

Fig. 55.21: The average relation between apical velocity rotation

and timing for human left ventricle (LV) in controls, and physiological (rowers) and pathological hypertrophy (aortic stenosis).
Data from magnetic resonance imaging (MRI) tagging show rapid
changes in early and late systole and early diastole, demonstrating prolongation of late systolic velocity into the early diastolic
phase in aortic stenosis. (ES: end-systole).

Myocyte causes relate to thickened LV mass due to

increased afterload from aortic stenosis, hypertension,
or hypertrophic cardiomyopathy.14,15 Stuber employed
MRI to demonstrate that aortic stenosis causes increased
peak torsion, longer interval to peak torsion,60 (Fig. 55.21)
and impaired untwisting. Similar alterations happen after
concentric hypertrophy in hypertension90 and hypertrophic cardiomyopathy. Treatment options should address
the specific cause of hypertrophy, because alleviating the
cause of LV hypertrophy allows regression of LV mass to
return twisting and untwisting capacity toward normal
by aortic valve replacement.91 Similar results likely follow
pharmacological management to reduce the increased
systemic vascular resistance, or alcohol or surgical removal
of the hypertrophied ventricular segment.92

Calcium-Related Factors
diastolic abnormalities, particularly involving ventricular
twist and deformation (strain) patterns leading to reduced
ventricular suction, delayed untwisting, and impaired
early diastolic filling.87,88 These observations emphasize
the interdependence of twisting and untwisting. Diastolic
dysfunction has clear echocardiography characteristics
relating to changes in the velocity waves during rapid
filling and atrial contraction. The role of the reported
increased untwisting during early diastolic dysfunction
is uncertain because the E-wave is reduced and impaired
filling occurs.16,89 Diastolic dysfunctions characteristic
impaired untwisting is associated with either (a) increased
torsion and preserved ejection fraction or (b) reduced
torsion from reduced systolic function.
The underlying problem is prolonged shortening of
the right-handed helical arm or descending segment
that causes extended torsion duration with resultant
compromise of the vital postejection isovolumic
phase timing hiatus. The keynote echocardiographic
observation is loss of longitudinal strain, a process caused
by the prolonged descending segment contraction causing
prolonged torsion, so that there is a delay in allowing the
noncontracting descending segment to become a fulcrum
for lengthening. Several reasons exist for this descending
segment prolongation, and the resultant treatment
options are determined by whether the causative factor
relates to (a) regional muscle anatomy, (b) physiological
calcium flux, or (c) geometric interruption of normal fiber
orientation by cardiac dilation.

Sarcolemmal calcium flux efficiency is a central underlying

event in both ischemia and aging.63,93 Kroeker studied94
twist dynamics during early ischemia and observed
that counterclockwise apical rotation was prolonged
into the isovolumic phase. A similar event occurs with
aging,95 and is also associated with prolonged shortening
of the descending segment after reperfusion following
longer ischemic intervals.94 Although prior suggestions
for defining the aging mechanism include left atrium
considerations and LV pressure deceleration changes,95,96
neither has addressed the impaired subendocardial
muscle function described by Lumens, that will prolong
inner shell shortening.96 Management options for
improving torsion and untwisting imbalance from these
causes may relate to enhancing calcium flux by reperfusion
after ischemia, or via pharmacological management
with aging; the compromised time hiatus is improved
by sodium hydrogen exchange inhibitors63 (Figs 55.22A
to D). Moreover, favorably modifying calcium efficiency by
levosimendan similarly reverses diastolic dysfunction.97

Dilated Cardiomyopathy
Geometric reasons for diminished torsion and impaired
untwisting become apparent as the normal conical
ventricular shape becomes spherical in dilated cardiomyopathy, as increased sphericity index is a primary
determinant of abnormal twisting and associated diastolic
dysfunction.79 Geometric changes thereby become the

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1203

Figs 55.22A to D: Sonomicrometer crystal tracings of hiatus between termination of right- and left-handed helix or descending and
ascending segment contraction during the isovolumic phase in the normal heart. A yellow shade defines this interval, and there is
recording of left ventricular pressure and dP/dt. (A) Normal or control intervals; (B) Bulging of both segments during ischemia or temporary coronary occlusion, without shortening; (C) 15 minutes after reperfusion shows reduced shortening, prolongation of right-handed
helix, or descending segment contraction that markedly reduces the hiatus between termination of the right- and left-handed helices
or descending and ascending shortening; (D) Diastolic dysfunction is percentage prolongation of hiatus between end of shortening
of right-handed helix (descending segment) and left-handed helix or ascending segment. Prolonged right-handed helix or descending
shortening defines this interval. The treated animals received cariporide, a sodium hydrogen ion inhibitor called HOE pretreatment.
Control values are shown below. Values expressed as mean SEM. Note return of normal hiatus following this intervention (*P < 0.05
HOE pretreatment vs no treatment).

unifying theme of torsion and untwisting dysfunction

in dilated cardiomyopathy from ischemic, valvular,
and nonischemic origin.98 Sallin33 showed that ejection
fraction diminishes as the oblique helical configuration
develops a more horizontal fiber orientation, and dilation
simultaneously stretches the fibers to also impair their
electrophysiological function.99 Moreover, diminished
untwisting is a hallmark sign of dilated cardiomyopathy,
so that this event stems from prolonged torsion to thereby
solidify the interdependence of the torsion and untwisting

relationship. Consequently, returning the helical form

with a spectrum of restoration procedures may reverse
the adverse torsion and untwisting interdependence in
dilated hearts.82

Pacing Factors
Cardiac motion studies show that normal cardiac
movement is sequential due to spread of the electrical
impulse from its earliest action within the conduction

1204

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

system toward its transition across the matrix and

into the muscle fibers.100 Heart motion after a single
transmural electrical excitation is synchronous, so that it
is not surprising why Wiggers in 1925 demonstrated that
ventricular pacing disturbed cardiac muscle movement
adversely.101 Recent studies of torsion and untwisting
during isolated ventricular pacing102,103 confirm how
a single pacing stimulus interferes with these natural
patterns. Biventricular pacing or cardiac resynchronization
therapy (CRT) also causes an inconsistent torsion and
untwisting improvement, because this fixed dual stimulus
differs from the natural spread of impulses via the His
Purkinje system that produces sequential motion. A recent
study in CRT responders demonstrated the apex and base
moving in the same direction or synchrony,52 instead
of twisting, thereby demonstrating their production of
transmural stimulation rather than sequential activation
along the His Purkinje system. Sonomicrometer crystal
studies document loss of the sequential motion following
atrium and then biventricular stimulation.104 Conversely,
there is restoration of the natural twisting and untwisting,
as sequential motion returns following atrial and then
high septal pacing;104 this pattern reflects the natural His
bundle pacing. Torsion and untwisting thereby require a
coordinated spread of impulses to the circular and helical
pathways to ensure the natural excitation contraction
coordination that does not interfere with the postejection
isovolumetric interval time hiatus.

Right Heart Failure

The septum is the lion of RV function,105 because the RV
must rely upon the requisite twisting of its helical fibers to
maintain RV cardiac output against increased PVR.105 In
contrast, paradoxical septum motion follows its stunning
during cardiac surgery70 or from its stretch following
volume overloading. The consequent bulging septal
geometric change causes its oblique fibers to become
more transverse to decrease its twisting capacity; this
infrastructure progresses to RV failure during pulmonary
hypertension. In contrast, the power of the RV free walls
compressive capacity is apparent when PVR is low; RV
failure did not occur in approximately 50% of the 3,292
consecutive surgical patients who developed paradoxical
septal motion following conventional methods of myocardial protection.70 Most importantly, the septum and
LV free wall are made of the same helical muscle, so that
LV diastolic dysfunction should occur whenever the
septum is globally stunned.

A close relationship exists between the septum and

tricuspid valve function, because its base anchors the
part of the A to V valve annulus and RV septum papillary
muscles arise from its body. Tricuspid valve regurgitation
(TR) develops when ventricular dilation stretches the
septum, a change that is caused by tethering of valve
leaflets; this mirrors the reason for MR development
occurring in patients with a wide QRS interval (Figs 55.23A
and B). Moreover, studies from our laboratory show that
acute pulmonary hypertension causes septum bowing
and resultant TR, both of which become reversed by
supplementing phenylephrine with intra-aortic balloon
pumping. This treatment restores the midline RV septum
position, while simultaneously avoiding LV vasoconstrictor
drugs induced after loading.106
RV failure treatment protocols are linked to understanding the functional HVMB causes of performance
impairment. Postoperative paradoxical septum motion is
totally avoided by use of the integrated blood cardioplegia
during open heart surgery.68 Abolition of postoperative
arrhythmias and right heart failure was achieved by
a valve ventricular approach that reconstructed the
stretched septum and replaced the pulmonary valve in
the dilated failing hearts.71 RV dysplasia was successfully
treated by normalizing the size of the aneurysmal free
wall,107 as the septum is not diseased in 80% of these
patients.108 RV failure is reversed in left ventricular assist
device (LVAD) patients by reducing LV suction to return the
bowed septum to the midline position.109 Realization that
septal twisting is further impaired by the high pulmonary
pressures in RV failure patients has resulted in avoidance
of vasoconstrictor drugs (epinephrine, dopamine), and
selection of the amrinone or milrinone agents, which
combine vasodilator and inotropic actions.
The theme of these clinical implications is that
unbalanced torsion and untwisting have a common
premise related to the impaired timing hiatus or longer
torsion duration during the postejection isovolumic
phase. Decisions about management become linked to
focusing upon how this common abnormality is altered
within different muscular, physiological, structural, and
electrical disease processes. Treatment options then
become geared toward efforts to reverse the initiating
event. Readily available torsion and untwisting monitors
may be employed to gauge their effectiveness.

CONCLUSION
Ventricular torsion is due to the twisting of the ventricle
during systole, and its subsequent untwisting is the prelude

Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion

1205

B
to subsequent diastolic filling. These interdependent
rotational events arise from the mechanical actions and
timing relationships of the hearts underlying circular and
helical muscle pathways. Explanation of the presystolic
IVC period is essential for analysis of these interactions.
Circular fibers dominate to cause pre- and post-twisting
net or global counterclockwise and clockwise movement,
whereas the helical fibers govern torsion. Normal

Figs 55.23A and B: (A) Left intraventricular view of the septum.

Note that the posterior medial papillary muscle arises from the left
ventricular (LV) wall immediately adjacent to the septum. Paradoxical or bowing septal motion causes it to bulge into the right
ventricle, so that the adjacent posterior papillary motion moves in
that direction, and results in tethering the mitral valve leaflets to
cause mitral regurgitation from this geometric reason; (B) Right
intraventricular appearance of the septum. Note attachments of the
posterior papillary muscles to the septum wall as well as tricuspid
valve leaflets. Observe that leftward bowing of septum will produce
traction upon the septum cusp leaflets and alter coaptation and
cause valve incompetence.

untwisting is related to preserving the 80-ms timing

hiatus between the end of shortening of the descending
and then the ascending arms of the helical muscle.
Central to understanding torsion and untwisting
interdependence is knowledge of the mechanics of normal
cardiac motion during the timing of the coiling and
recoiling actions of circular and helical fiber pathways.
Longer torsion duration results from prolonged right-

1206

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

handed helical arm or descending segment contraction

that compromises this timing hiatus and thereby
interferes with untwisting. Clinical implications result from
unbalanced torsion and untwisting, and longer torsion
duration becomes their common theme. Management
decisions relate to interconnected reasons for adverse
mechanical and timing factors that cause this common
abnormality within muscular, physiological, structural,
and electrical disease processes.

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of papillary muscle. Am J Physiol. 1970;218(6):171017.
70. Reynolds HR, Tunick PA, Grossi EA, et al. Paradoxical
septal motion after cardiac surgery: a review of 3,292 cases.
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71. Frigiola A, Giamberti A, Chessa M, et al.; RESTORE group.
Right ventricular restoration during pulmonary valve
implantation in adults with congenital heart disease. Eur
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72. Greenbaum RA, Ho SY, Gibson DG, et al. Left ventricular
fibre architecture in man. Br Heart J. 1981;45(3):24863.
73. Sakuma M, Ishigaki H, Komaki K, et al. Right ventricular
ejection function assessed by cineangiographyImportance
of bellows action. Circ J. 2002;66(6):6059.
74. Wiggers C. Some factors controlling the shape of the
pressure curve in the right ventricle. Am J Physiol. 1914;33:
38295.
75. Starr I, Jeffers WA, Meade RH Jr. The absence of conspicuous
increments of venous pressure after severe damage to the
right ventricle of the dog, with a discussion of the relation
between clinical congestive failure and heart disease. Am
Heart J. 1943;26:291301.
76. Sawatani S, Mandell G, Kusaba E, et al. Ventricular performance following ablation and prosthetic replacement of
right ventricular myocardium. Trans Am Soc Artif Intern
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77. Cox JL, Bardy GH, Damiano RJ Jr, et al. Right ventricular
isolation procedures for nonischemic ventricular
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78. Donald DE, Essex HE. Pressure studies after inactivation
of the major portion of the canine right ventricle. Am J
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79. van Dalen BM, Kauer F, Vletter WB, et al. Influence of
cardiac shape on left ventricular twist. J Appl Physiol. 2010;
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80. Buckberg G. Rethinking the cardiac helix: a structure/
function journey. Eur J Cardiothorac Surg. 2006;29S1:S1.
81. Buckberg GD. The structure and function of the healthy
helical and failing spherical heart. Overview: the
ventricular band and its surgical implications. Semin
Thorac Cardiovasc Surg. 2001;13(4):298300.
82. Buckberg GD; RESTORE Group. Form versus disease:
optimizing geometry during ventricular restoration. Eur J
Cardiothorac Surg. 2006;29(Suppl 1):S23844.
83. Buckberg GD, Coghlan HC, Torrent-Guasp F. The
structure and function of the helical heart and its buttress
wrapping. VI. Geometric concepts of heart failure and use

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for structural correction. Semin Thorac Cardiovasc Surg.

2001; 13(4):386401.
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torsion in primary chronic mitral regurgitation. Heart.
2008;94(5):597603.
Nishimura RA, Tajik AJ. Evaluation of diastolic filling of left
ventricle in health and disease: Doppler echocardiography
is the clinicians Rosetta Stone. J Am Coll Cardiol.
1997;30(1):818.
Tan YT, Wenzelburger FW, Sanderson JE, et al. Exerciseinduced torsional dyssynchrony relates to impaired
functional capacity in patients with heart failure and
normal ejection fraction. Heart. 2013;99(4):25966.
Tan YT, Wenzelburger F, Lee E, et al. The pathophysiology
of heart failure with normal ejection fraction: exercise
echocardiography reveals complex abnormalities of both
systolic and diastolic ventricular function involving torsion,
untwist, and longitudinal motion. J Am Coll Cardiol.
2009;54(1):3646.
Pacileo G, Baldini L, Limongelli G, et al. Prolonged
left ventricular twist in cardiomyopathies: a potential
link between systolic and diastolic dysfunction. Eur J
Echocardiogr. 2011;12(11):8419.
Park SJ, Miyazaki C, Bruce CJ, et al. Left ventricular torsion
by two-dimensional speckle tracking echocardiography in
patients with diastolic dysfunction and normal ejection
fraction. J Am Soc Echocardiogr. 2008;21(10):112937.
Mizuguchi Y, Oishi Y, Miyoshi H, et al. Concentric left
ventricular hypertrophy brings deterioration of systolic
longitudinal, circumferential, and radial myocardial
deformation in hypertensive patients with preserved left
ventricular pump function. J Cardiol. 2010;55(1):2333.
Van Der Toorn A, Barenbrug P, Snoep G, et al. Transmural
gradients of cardiac myofiber shortening in aortic valve
stenosis patients using MRI tagging. Am J Physiol Heart
Circ Physiol. 2002;283(4):H160915.
Carasso S, Woo A, Yang H, et al. Myocardial mechanics
explains the time course of benefit for septal ethanol
ablation for hypertrophic cardiomyopathy. J Am Soc
Echocardiogr. 2008;21(5):4939.
Kho C, Lee A, Hajjar RJ. Altered sarcoplasmic reticulum
calcium cyclingtargets for heart failure therapy. Nat Rev
Cardiol. 2012;9(12):71733.
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left ventricular apex rotation. An experimental study in
anesthetized dogs. Circulation. 1995;92(12):353948.
Hees PS, Fleg JL, Dong SJ, et al. MRI and echocardiographic
assessment of the diastolic dysfunction of normal aging:
altered LV pressure decline or load? Am J Physiol Heart
Circ Physiol. 2004;286(2):H7828.
Lumens J, Delhaas T, Arts T, et al. Impaired subendocardial
contractile myofiber function in asymptomatic aged
humans, as detected using MRI. Am J Physiol Heart Circ
Physiol. 2006;291(4):H15739.
Barraud D, Faivre V, Damy T, et al. Levosimendan
restores both systolic and diastolic cardiac performance
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137682.
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function journey: overview. Eur J Cardiothorac Surg.
2006;29(Suppl 1):S23.
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remodeling and ventricular arrhythmias after myocardial
infarction. Circulation. 2003;107(20):257782.
Coghlan HC, Coghlan AR, Buckberg GD, et al. The structure
and function of the helical heart and its buttress wrapping.
III. The electric spiral of the heart: The hypothesis of the
anisotropic conducting matrix. Semin Thorac Cardiovasc
Surg. 2001;13(4):33341.
Wiggers CJ. The muscular reactions of the mammalian
ventricles to artificial surface stimuli. Am J Physiol.
1925;(73):34678.
Sorger JM, Wyman BT, Faris OP, et al. Torsion of the left
ventricle during pacing with MRI tagging. J Cardiovasc
Magn Reson. 2003;5(4):52130.
Wang J, Nagueh SF, Mathuria NS, et al. Left ventricular twist
mechanics in a canine model of reversible congestive heart
failure: a pilot study. J Am Soc Echocardiogr. 2009;22(1):
958.

1209

104. Tomioka H, Liakopoulos O, Buckberg GD, et al. The effect

of ventricular sequential contraction on helical heart
during pacing. Europ J Cardiothorac Surg. 2006.
105. Saleh S, Liakopoulos OJ, Buckberg GD. The septal motor
of biventricular function. Eur J Cardiothorac Surg.
2006;29(Suppl 1):S12638.
106. Liakopoulos OJ, Ho JK, Yezbick AB, et al. Right ventricular
failure resulting from pressure overload: role of intra-aortic
balloon counterpulsation and vasopressor therapy. J Surg
Res. 2010;164(1):5866.
107. Buckberg GD, Coghlan HC, Hoffman JI, Torrent-Guasp
F. The structure and function of the helical heart and its
buttress wrapping. VII. Critical importance of septum for
right ventricular function. Semin Thorac Cardiovasc Surg.
2001;13(4):40216.
108. Basso C, Thiene G, Corrado D, et al. Arrhythmogenic right
ventricular cardiomyopathy. Dysplasia, dystrophy, or
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109. Neragi-Miandoab S, Goldstein D, Bello R, et al. Right
ventricular dysfunction following continuous flow left
ventricular assist device placement in 51 patients:
predicators and outcomes. J Cardiothorac Surg. 2012;7:60.

CHAPTER 56
Echocardiography in Assessment of
Complications Related to Permanent
Pacemakers and Intracardiac
Defibrillators
Ahmed Almomani, Khadija Siddiqui, Masood Ahmad

Snapshot

Normal Echocardiographic Findings in Permanent

Pacemakers/Implantable Cardioverter-Defibrillators

Pacemaker and Implantable Cardioverter-DefibrillatorRelated Complications

Tricuspid Regurgitation

INTRODUCTION
Over the past decades, technical advances in permanent
pacemakers (PPMs) and implantable cardioverter-defibri
llators (ICDs) have led to a tremendous increase in the
use of these medically important devices. This trend is
likely to continue due to the increased life expectancy of
the population and the increasing number of indications
for their use including placement of PPMs for cardiac
resynchronization therapy (CRT) and ICDs for primary and
secondary prevention of complications from arrhythmias
in patients with left ventricular dysfunction.13 The 11th
World Survey of Cardiac Pacing and ICDs reported that
737,840 new devices were implanted in 2009 worldwide,
with the largest number of new implants, 225,567, in the
United States. These numbers showed a huge increase
compared to a similar survey done in 2005, and the
numbers are expected to be much higher in 2013.4

Masses: Lead Infection and Thrombus

Myocardial Perforation

Deleterious Effects of Right Ventricular Apical Pacing on

Left Ventricular Function

Endocardial lead-related complications have not been

very well recognized. In recent years, there has been an
increasing awareness of device-related complications such
as tricuspid regurgitation (TR), lead infection, thrombosis,
perforation, and left ventricular dyssynchrony. We will
review the roles of transthoracic echocardiography (TTE)
and transesophageal echocardiography (TEE) in the
assessment of pacemaker/ICD endocardial lead-related
complications.

NORMAL ECHOCARDIOGRAPHIC
FINDINGS IN PERMANENT
PACEMAKERS/IMPLANTABLE
CARDIOVERTER-DEFIBRILLATORS
Transthoracic echocardiography can be used to visualize
the intracardiac portion of the PPM or ICD lead. Device

Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators

1211

Figs 56.1A to E: Two-dimensional transthoracic echocardiography (2D TTE) with pacer lead shown in modified parasternal
long-axis (A), short-axis (B), apical four-chamber (C), focused
right-sided chambers (D), and subcostal (E) views. Arrowheads
point to the lead, arrow in Figure A points to the tricuspid valve.
(AO: Aortic valve; LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).

leads may be imaged in the right atrium or right ventricle

(RV) in a number of views, including RV inflow, parasternal
short-axis at the level of the aortic valve, apical fourchamber, or subcostal view (Figs 56.1A to E). However, in
some patients, the presence of lead cannot be satisfactorily

demonstrated due to poor acoustic windows resulting

in limited visualization, and due to artifacts related
to lead reverberations. Real time transthoracic threedimensional echocardiography (RT3DE) can overcome
some of these limitations by visualizing the entire route

1212

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 56.2A to D: Real time transthoracic three-dimensional echocardiography (RT3DE) in apical four-chamber view demonstrating the
pacer route and its relation to intracardiac structures in simultaneously obtained multiple views derived from the same data set. Arrowheads point to the lead. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: right ventricle).

of the lead in multiple views from the same data set

(Figs 56.2A to D, and Movie clips 56.1 and 56.2).
In addition, in some patients with RV pacing, there may
be paradoxical septal motion due to early activation of the
RV. This, however, is not a finding isolated to the presence
of cardiac devices but can also be seen in the presence of
other conduction abnormalities, cardiac surgery, or RV
volume/pressure overload.

PACEMAKER AND IMPLANTABLE

CARDIOVERTER-DEFIBRILLATORRELATED COMPLICATIONS
Transthoracic echocardiogrphy and Transesophageal
echocardiography are useful in evaluating pacemakerrelated complications including TR, valvular or lead
vegetations, pericardial effusion, abnormal lead position,
perforation, and lead thrombus.

TRICUSPID REGURGITATION
The development of significant TR and its progression
over time is an important device-related complication.
Prospective data on TR secondary to PPM and ICD
leads are lacking. Multiple small retrospective studies
and small case series have reported this complication
(Table 56.1). TR in patients with PPMs or ICDs may not
be exclusively caused by the endocardial lead, as preexisting abnormalities such as tricuspid valve (TV) annular
dilatation or pulmonary hypertension may be present.5
A number of different mechanisms of RV intracardiac
lead-related TR have been described. In one retrospective
study, it was noted that in 41 patients with PPM or ICD
lead-induced severe TR requiring surgery, 7 patients had
perforation of the TV leaflet by the PPM or ICD lead, 4 had
lead entanglement in the TV, 16 had lead impingement
of the TV leaflets, and 14 had lead adherence to the TV.6

Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators

1213

Table 56.1: Summary of the Studies on Pacemaker-Related Tricuspid Regurgitation

Study
Sakai et al. (1987)58
Paniagua et al. (1998)

59

TR Before Lead TR After

TR Severity
Implantation
Implantation

Sample Size

Study Design

Follow-up

18

Prospective

NA

NA

TR severity not assessed

374 cases

Case control

NA

NA

27

Moderate to severe TR

NA

NA

12

(P < 0.0001)

1.2 0.7 days

10

Moderate to severe TR
(P = Not significant)

NA

NA

Out of 1,465 patients

with severe TR that
required surgery,
41 were secondary to
endocardial leads

12

Moderate to severe TR
(P = Not significant)

683 controls
Leibowitz et al. (2000)

35

Prospective

Lin et al. (2005)6

41

Retrospective

Kucukarslan et al. (2006)61

61

Prospective

Seo et al. (2008)7

87

Retrospective 36 months

NA

32

Moderate to severe
TR3D echocardiography
was used

Kim et al. (2008)62

248

Retrospective 93 days
(range,
23199)

69

NA

TTE before and after

pacer showed significant increases in TR,
moderate to severe in
24.2% and severe in 4%

Klutstein et al. (2009)63

410

Retrospective 75 days
(range,
14,367 d)

NA

NA

TTE before and after

pacer showed that 75
patients (18.3%) had
significant worsening
of TR

Vaturi et al. (2010)64

23

Prospective

48.6 32.7
months

Moderate to severe TR.

Number increased to
18 when mode changed
to active RV pacing
(P < 0.001)

Alizadeh et al. (2011)65

115

Prospective

4.1 0.8
years

10

36

Moderate to severe TR
(P < 0.001)

60

6 3 months

(RV: Right ventricle; TR: Tricuspid regurgitation; TTE: Transthoracic echocardiography).

Source: Modified with permission from Al-Mohaissen MA, Chan KL. Prevalence and mechanism of tricuspid regurgitation following
implantation of endocardial leads for pacemaker or cardioverter-defibrillator. J Am Soc Echocardiogr. 2012;25(3):24552.

In the same study, severe lead-induced TR causing rightheart failure that required TV surgery accounted for 2.8%
of all TV operations (41 of 1,465 consecutive patients)
between 1993 and 2003 at the Mayo Clinic.
The time course for TR development and progression
after endocardial lead placement in the RV is not well
defined. A large retrospective study reported an increase
in TR acutely after RV lead implantation, and progressive
worsening with time, while other studies have mainly
focused on the chronic effects of the leads on the
valve.7 Pathological studies have demonstrated major
inflammatory changes occurring within the heart only days
after lead implantation. The progression of inflammation

over weeks to months leads to fibrous tissue formation,

which encapsulates the pacemaker lead and may result
in fusion and adherence of the endocardial lead to the TV
leaflets, chordae, and papillary muscles, resulting in TR.8
Transthoracic echocardiography is often used to
assess TR after implantation of PPMs (Fig. 56.3 and
Movie clip 56.3). However, the ability to define the
precise anatomical relationship between the TV and
the pacemaker lead is quite limited using this modality.
In a study done at Mayo Clinic, the valve malfunction
due to PPM or ICD lead was diagnosed preoperatively
in only 5 of 41 patients by TTE.6 On the other hand,
RT3DE enables visualization of the entire TV, in

1214

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 56.3: Two-dimensional transthoracic echocardiography (2D

TTE) with focused view of right-sided chambers, demonstrating
the tricuspid regurgitation (TR) jet (arrow) between the right ventricle (RV) lead (arrow head) and the septal leaflet of the tricuspid
valve (TV).

particular, en-face short-axis views of the valve, which

may facilitate the enhanced ability of this technique in
assessing the route and position of the lead at the TV
and in visualizing the actual movement of the leaflets
(Figs 56.4A and B, and Movie clips 56.4 and 56.5). A
high efficacy (96%) of 3DE in complete assessment of
the TV structure has also been reported.9 Another study
reported that among the 87 patients involved in the study,
leads passing through the TV were identified in only 15
(17.2%) patients by 2D. In contrast, on 3DE examinations,
lead routes were identified in 82 (94.2%) patients. In the
remaining five patients, appropriate 3DE images for lead
route analysis could not be obtained because of artifacts
caused by the lead.7
Tricuspid regurgitation is a preventable complication of
PPM and ICD leads, and is related to the lead position, lead
route, and interaction with the valve leaflets. Prospective
studies are needed to establish the temporal relationship
between placement of the device and development/severity
of TR. Real time 3D echocardiography appears superior
to two-dimensional transthoracic echocardiography (2D
TTE) in assessing the TV in patients with PPMs and ICDs
(Figs 56.4A and B).

MASSES: LEAD INFECTION AND

THROMBUS
As there has been an increase in the number of implanted
cardiac devices over the past couple of decades, there has

also been a notable increase in the detection of masses

on these leads. Of the available imaging modalities,
magnetic resonance imaging still continues to be relati
vely contraindicated in a majority of pacemakers and
defibrillators, therefore limiting evaluation of these devices
by either computed tomography (CT) or echocardiography.
However, since the metal in the device and the movement
of the leads create artifacts on CT, the most desirable
imaging modality remains an echocardiogram. A mass
detected on an implanted lead on an echocardiogram,
almost invariably represents either a thrombus or vege
tation, and distinguishing between the two can often be
quite difficult. Since ultrasound imaging alone cannot
determine the etiology of the mass, clinical presentation
and lab data play a crucial role in the interpretation and
management of these abnormal findings.

Infection
Initial cases of pacemaker endocarditis were described
in the early 1970s.10 Reported device-related infective
endocarditis (IE) has ranged from 10% to 23% in the
literature.11,12 The incidence of infection following implan
tation of PPMs has been variably assessed, ranging from
0.13% to 19.9%,13,14 with the majority of infections repo
rted to be in the pacemaker generator pocket. Incidence
of infection in ICDs has been reported in the literature
ranging from 0.7% to 1.2%.15,16 Technical advances have
allowed transvenous implantation of ICDs, thereby
eliminating the need for thoracotomy and epicardial lead
placement. These advances have contributed to the overall
decline in infection associated with ICD.
In patients with device-related endocarditis, the
presence of vegetation is limited not only to the TV but
can be found anywhere along the course of the lead,
including the endocardium of the right atrium or RV.17
Echocardiography plays an important role by allowing
direct visualization and measurement of the mass along
with the ability to assess for cardiac involvement. On
echocardiography, vegetations have been defined as
oscillating intracardiac masses on the device leads, valve
leaflets, or endocardial surface, confirmed by imaging in
more than one echocardiographic plane. In these cases,
the valve or lead infection was confirmed by positive
blood or lead tip culture.12,18 Valvular vegetations have
been further characterized on the basis of four physical
properties, as assessed by echocardiography: vegetation
size, mobility, extent, and consistency.18

Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators

1215

Figs 56.4A and B: Real time transthoracic three-dimensional echocardiography (RT3DE) with pacer and en-face view of the tricuspid
valve from the right atrium (A). Pacer and the tricuspid valve from the right ventricle (B), the lead position (arrowhead) in relationship
to the tricuspid valve (arrow).

Although TTE has an improved sensitivity later in the

course of the disease, even then the overall sensitivity is
quite poor.19 This is in part due to the difficulty in precisely
distinguishing between abnormal masses, the TV, and the
lead itselfmainly as a result of limited visualization, poor
echogenicity in some patients, and artifacts due to lead
reverberations in others. In contrast, use of a multiplane
echocardiographic probe with TEE improves the quality
of exploration and has a much more established role as
a diagnostic technique. The ability to visualize the entire
intracardiac route of the leads, from the upper vena cava to
the RV apex, provides TEE with its much higher sensitivity
and specificity. In three of the larger studies that used
strict criteria for entry and compared results with surgical
and microbiological endpoints, the sensitivity of TTE was
2230%, while that of TEE was 9296%.1921 In addition,
TEE also has a role in defining the most appropriate
extraction technique by identifying patients with
myocardial abscess or extremely large (> 5 cm) lead
vegetations that may necessitate surgery rather than a
percutaneous method of extraction.12

Thrombus
Venous thrombosis and stenosis have been described
as the most common complications associated with
transvenous pacemaker implantation with incidence
ranging between 30% and 45%.22 Right atrial thrombus
is a rarely reported event, and can present either as an
incidental finding on echocardiogram,23 or as symptoms
of right-sided heart failure,24 obstruction, or embolization
of the pulmonary artery.25 One study described only two

cases of large right atrial thrombi that were found in a

series of 53 necropsies performed in patients with PPM.
Both patients had evidence of hemodynamic impairment
with signs of congestive heart failure. This series suggested
that a right atrial thrombus should be considered in the
setting of refractory heart failure, despite a normally
functioning pacemaker and adequate medical treatment.
Other serious complications such as superior vena cava
syndrome have also been reported but are beyond the
scope of this review.26
As stated, the diagnosis on TTE may be technically
challenging in patients with limited acoustic windows and
lead reverberations. On the other hand, TEE allows direct
visualization of the lead, the entire right atrium, interatrial
septum, superior vena cava, and inferior vena cava
(Fig. 56.5 and Movie clip 56.6). TEE has also been sugg
ested to provide information that can possibly determine
whether the thrombus is recent or long-standing.27 Longstanding thrombi tend to be sessile and sometimes contain
calcium. In contrast, fresh thrombi are highly mobile and
appear less echo-dense. The use of 3D with TTE or TEE has
an incremental value in evaluating masses attached to the
leads (Fig. 56.6 and Movie clip 56.7). As discussed above,
differences in clinical presentation and laboratory findings
play a significant role in distinguishing lead infection from
thrombus.

MYOCARDIAL PERFORATION
Myocardial perforation is a relatively rare complication
of endocardial leads of cardiac implantable devices. The
incidence of this complication is estimated to be < 1%.

1216

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 56.5: Two-dimensional transesophageal echocardiography (2D TEE) showing the pacer lead (arrowhead) and attached
mobile thrombus (arrow).

Fig. 56.7: Echocardiography (four-chamber apical view): implantable cardioverter-defibrillator (ICD) lead perforation across the
right ventricular apex. (LV: Left ventricle; PE: Pericardial effusion;
RV: Right ventricle).
Source: Reproduced with permission from Sassone B, Gabrieli L,
Boggian G, Pilato E. Management of traumatic implantable cardioverter defibrillator lead perforation of the right ventricle after car
accident: a case report. Europace. 2009;11(7):9612.

In some studies the lead perforation rates range from

0.1% to 0.8% for PPMs and 0.6% to 5.2% for ICD leads.
Lead perforation rates may depend on the lead model.28
Furthermore, lead perforation can be categorized into two
groups: acute perforation after lead placement, which is
mostly related to the procedure, and subacute or delayed

Fig. 56.6: Real time transthoracic three-dimensional echocardiography (RT3DE) of the pacer lead shown in Figure 56.5; right
ventricle (RV) lead (arrowhead) with a thrombus (arrow).

perforation defined as the perforation of the lead through

the myocardium more than 1 month after implantation.2835
In a review of 51 reported cases of delayed lead
perforation, the demographics and characteristics of this
group showed that elderly women and patients with lower
body mass are more vulnerable to this complication.36
Table 56.2 summarizes 35 cases reported in the literature.
Unlike acute lead perforation, one of the distinguishing
features of delayed lead perforation is the low risk of
cardiac tamponade and death.3739 Lead perforation can
sometimes be identified by chest X-ray showing the leads
migration outside the heart.36,40 Chest CT plays a crucial
role in the diagnosis of lead perforation when other
modalities are nondiagnostic.40,41 In one report, 15 out of
100 asymptomatic patients with pacemakers or ICDs were
found to have late lead perforation on chest CTs performed
for other reasons.40 However, CT images may be limited by
artifacts created by the leads.
Two-dimensional echocardiography is a valuable tool
for the diagnosis of lead perforation and dislodgement
(Fig. 56.7).42 This modality can also demonstrate pericardial
effusion and tamponade if present. Difficulty or failure of
2D TTE to visualize leads is not uncommon. Transthoracic
RT3DE is superior to 2D TTE in detecting lead perforation
and its route (Fig. 56.8). RT3DE is complementary to
2D TTE in clinical practice and should be used if a lead
complication is suspected.43,44

Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators

1217

Table 56.2: Summary of the Reported Cases of Lead Perforation

Characteristics

Total Number = 35

Mean Age

64.0 20.2 years

Gender:

Male

16 (45.7%)

Female

19 (54.3%)

Mean time from implant

35.9 48.8 weeks

Type of device:

Pacemaker

19 (54.3%)

ICD

15 (42.8%)

NA

1 (2.9%)

Type of lead fixation:

Active

24 (68.6%)

Passive

6 (17.1%)

NA

5 (14.3%)

Evidence of Perforation on echocardiography:

Yes

24 (68.6%)

No

4 (11.4%)

NA

7 (20.0%)

(ICD: Implantable cardioverter-defibrillators; NA: Not available).

Table includes the analysis of 35 cases reported in the listed references.29,31,32,34-37,41,44,66-84

DELETERIOUS EFFECTS OF RIGHT

VENTRICULAR APICAL PACING ON
LEFT VENTRICULAR FUNCTION

Fig. 56.8: Three-dimensional echocardiogram of an apical fourchamber view, demonstrating the pacemaker lead tip (arrow)
going through the interventricular septum (arrowhead); (LV: Left
ventricle; RV: Right ventricle).
Source: Reproduced with permission from Daher IN, Saeed M,
Schwarz ER, Agoston I, Rahman MA, Ahmad M. Live threedimensional echocardiography in diagnosis of interventricular septal perforation by pacemaker lead. Echocardiography.
2006;23(5):4289.

Left ventricular mechanical and electrical dyssynchrony

are poor prognostic factors in patients with systolic
heart failure.45,46 Abnormalities in the timing of regional
mechanical left ventricular activation, known as
intraventricular dyssynchrony, appears to be the principal
factor associated with contractile impairment that is
improved by CRT. The classic type of dyssynchrony
resulting from abnormal electrical activation is seen with
left bundle branch block (LBBB), where there is early
activation of the interventricular septum and late activation
of the posterior and lateral left ventricular walls.47 The early
septal contraction occurs before normal ejection when
pressure in the left ventricle is low, thereby generating
asynchronous stress and strain in the left ventricle, with
one wall exerting forces on the contralateral wall.48
Right ventricular apical pacing is frequently used
with implantable pacemakers, and it has been shown
to be a well-established risk factor for left ventricular

1218

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 56.9A and B: Three dimensional (3D) segmental timevelocity curves of left ventricle (LV) before pacing, left ventricular ejection fraction (LVEF) 48.5%, SDI 2.7% (A) and after right ventricle (RV) pacing, ejection fraction (EF) 45.7%, SDI 10.9% (B), showing
increased LV dyssynchrony and decrease in LVEF after pacing.

dyssynchrony leading to systolic dysfunction.49 Dyssyn

chrony with RV pacing has a similar mechanism to LBBB.
In the long run, RV pacing and dyssynchrony may trigger
ventricular remodeling by causing both systolic and
diastolic left ventricular dysfunction, increases in endsystolic volume and wall stress, leading to asymmetrical
hypertrophy and abnormal histopathology. Clinically,
these changes manifest as worsening of heart failure.48,50,51
Furthermore, one study demonstrated that RV apical pacing
can cause regional myocardial perfusion and wall motion
abnormalities near the sites of electrical stimulation,
which increase with the duration of pacing. These changes
are associated with impairment of left ventricular diastolic
function and progressive deterioration of regional left
ventricular ejection fraction over time in regions remote
from the site of electrical stimulation, resulting in a
significant reduction in global left ventricular function.52
Left ventricular dyssynchrony can be assessed by
tissue Doppler imaging and more recently by speckle
tracking echocardiography. Measurements of mechanical
dyssynchrony index guide CRT in patients with heart
failure and left ventricular dysfunction.53 Real time 3D
echocardiography provides a unique and powerful tool for
the evaluation of left ventricular dyssynchrony by allowing
comparison of the timevelocity curves of the various left
ventricular segments in the same cardiac cycle. The impact
of RV pacing on left ventricular dyssynchrony is shown in

Figures 56.9A and B.5357 Patients with RV pacing can be

evaluated for left ventricular dyssynchrony and followed
over time to detect pacing-related left ventricular systolic
dysfunction.

CONCLUSION
The increasing indications and uses for implantable
cardiac devices have led to a continuous increase in the
number of implanted devices each year. Implantation
of endocardial leads for these devices can cause many
delayed complications. Some of the complications are
mechanical and related to the presence of foreign body
and its interaction with the valves and endomyocardium,
for example, perforation, infection, and thrombosis, while
others are related to the electrical pacing of the myocardium
and conduction abnormalities, for example, dyssynchrony
and TR. It is important to have a high index of suspicion
to diagnose these complications, using the appropriate
imaging modality. Based on the preceding review, it is
clear that echocardiography plays an important role in the
diagnosis of the device-related complications. Both 2D TTE
and TEE provide extremely useful diagnostic information
that is helpful in detecting the endocardial lead-related
complications. Real time 3D echocardiography is a novel
technique that can precisely track the intracardiac route of
the lead and accurately detect most of the pacemaker/ICD
lead-related complications.

Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators

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Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

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35. Ellenbogen KA, Wood MA, Shepard RK. Delayed
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43. Stefanidis AS, Margos PN, Kotsakis AA, et al. Threedimensional echocardiographic documentation of pace
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48. Spragg DD, Kass DA. Pathobiology of left ventricular
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49. Manolis AS. The deleterious consequences of right
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58. Sakai M, Ohkawa S, Ueda K, et al. [Tricuspid regurgitation
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59. Paniagua D, Aldrich HR, Lieberman EH, et al. Increased
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60. Leibowitz DW, Rosenheck S, Pollak A, et al. Transvenous
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62. Kim JB, Spevack DM, Tunick PA, et al. The effect of
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2847.

Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators

63. Klutstein M, Balkin J, Butnaru A, et al. Tricuspid incom

petence following perm
anent pacemaker implan
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64. Vaturi M, Kusniec J, Shapira Y, et al. Right ventricular
pacing increases tricuspid regurgitation grade regardless of
the mechanical interference to the valve by the electrode.
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65. Alizadeh A, Sanati HR, Haji-Karimi M, et al. Induction and
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65. Mortensen K, Aydin MA, Goldmann B, et al. Fluoroscopy
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67. Krivan L, Kozk M, Vlasnov J, et al. Right ventricular
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69. Sakai Y, Sato Y, Matsuo S, et al. Perforation of the right
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extraction of a passive fixation lead with delayed perforation
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72. Laborderie J, Bordachar P, Reuter S, et al. Myocardial
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74. Kautzner J, Bytesnk J. Recurrent pericardial chest pain: a

case of late right ventricular perforation after implantation
of a transvenous active-fixation ICD lead. Pacing Clin
Electrophysiol. 2001;24(1):1168.
75. Amara W, Cymbalista M, Sergent J. Delayed right ventricular
perforation with a pacemaker lead into subcutaneous
tissues. Arch Cardiovasc Dis. 2010;103(1):534.
76. Haghjoo M, Alizadeh A, Fazelifar AF, et al. Delayed cardiac
perforation by one small body diameter defibrillator lead.
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77. Ferrero-de-Loma-Osorio A, Albors-Martn J, Ruiz-Granell
R, et al. Images in cardiovascular medicine: Delayed right
ventricular perforation by a transvenous active fixation
implantable cardioverter-defibrillator lead: echo
cardio
graphic diagnosis and surgical management. Circulation.
2009;119(15):211213.
78. Tziakas D, Alexoudis A, Konstantinou F, et al. A rare case
of late right ventricular perforation by a passive-fixation
permanent pacemaker lead. Europace. 2009;11(7):9689.
79. Danik SB, Mansour M, Heist EK, et al. Timing of delayed
perforation with the St. Jude Riata lead: a single-center
experience and a review of the literature. Heart Rhythm.
2008;5(12):166772.
80. Celik T, Kose S, Bugan B, et al. Hiccup as a result of late
lead perforation: report of two cases and review of the
literature. Europace. 2009;11(7):9635.
81. Haque MA, Roy S, Biswas B. Perforation by permanent
pacemaker lead: how late can they occur? Cardiol
J. 2012;19(3):3267.
82. Hrer J, Will A, Schreiber C. Delayed right-ventricular
perforation of a pacemaker lead. Pediatr Cardiol. 2011;32(5):
7089.
83. Bigdeli AK, Beiras-Fernandez A, Kaczmarek I, et al. Succ
essful management of late right ventricular perforation
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2010;6:2730.
84. Fisher JD, Fox M, Kim SG, et al. Asymptomatic anterior
perforation of an ICD lead into subcutaneous tissues.
Pacing Clin Electrophysiol. 2008;31(1):79.

CHAPTER 57
Echocardiographic Evaluation of
Ventricular Assist Devices
Peter S Rahko

Snapshot

Clinical Uses of Ventricular Assist Devices

Reverse Remodeling

Types of Devices

Preoperave Echocardiographic Evaluaon

Immediate Postsurgical Evaluaon

Serial Changes in Cardiac Structure and Funcon

INTRODUCTION
Mechanical circulatory support began in the 1960s when
Michael DeBakey and his colleagues first began conceiving of a left ventricular assist device (LVAD). Early devices
were pneumatic with an external drive system, and
the first device was implanted in 1963 in a patient who
suffered a cardiac arrest. By 1966, a second pneumatic,
pulsatile LVAD was developed and utilized in a patient.1,2
Considerable development in pulsatile LVADs, namely,
devices that had a chamber that filled and then ejected
using a pusher plate design, expanded throughout
the 1970s and into the 1980s. The first Food and Drug
Administration (FDA)-approved intracorporeal LVAD was
the Thoratec HeartMate XVE. Initially, it was pneumatically
powered but later became electrically powered. This was
the first realistic device that gave patients independence
since everything was internal except a driveline that came
out from a skin tunnel, and attached to a controller and
battery pack. For the first time, patients could be relatively
mobile and realistically go home and even go back to work
in some situations. The superiority of device therapy over
medical therapy was established in the REMATCH trial for
end-stage heart failure patients.3 However, the pulsatile
chamberpusher plate design had many moving parts,

Complicaons of Le Ventricular Assist Devices

Evidence of Underfilling of the Le Ventricle

Opmizing Le Ventricular Assist Device Sengs

Explantaon

Percutaneous Connuous Flow Devices

required heart valves at the inflow and outflow cannulas,

and thus had many possibilities for failure. Furthermore,
the device was never conceived of as anything but a bridge
to transplantation; thus, very long-term utilization of the
device was not feasible. Finally, the device was relatively
large and could only be implanted in full-sized adults.
To address the shortcomings of pulsatile devices, the
concept of a continuous flow assist device was developed.
These rotary pumps use the principle of the Archimedes
screw. Pump rotation creates force on blood and propels it
forward longitudinally through the device. The impeller of
this device must rotate at a relatively faster speed (8,000
12,000 rpm) to create a moving force on the blood. The
advantage of this design is a marked reduction in overall
size. Indeed, it was possible to miniaturize these devices
to the point that there was a marked improvement in
tolerability and also an expanded range of body size that
could benefit from this device. The first clinical use of a
device of this type occurred in 1998 with the introduction
of the MicroMed DeBakey VAD. The first approved
device by the FDA was the HeartMate II built by Thoratec
Corporation (Figs 57.1A to D).4
The third generation of assist devices is centrifugal
(Figs 57.2A to D). These devices propel blood like a
spinning top inside of a chamber. Blood enters the device

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1223

Figs 57.1A to D: (A) The Thoratec HeartMate II draws blood from the left ventricle (LV) apex through the inflow cannula into the pump
and then ejects blood through the outflow cannula to the central aorta; (B) Closer view showing the impellar design. (Used with permission of Thoratec); (C) Chest X-ray of patient with a HeartMate II showing inflow cannula position and relative position of the left ventricular assist device (LVAD) in the chest; (D) Three-dimensional reconstruction of a chest CT scan of same patient as (C). Note position of
cannula and in this case the tortuous course of the outflow cannula.

A
Figs 57.2A and B

1224

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.2A to D: Example of a centrifugal pump, the HeartWare device. (A) Relative size is small; (B) The device open shows the
rotating disk that spins blood outward from the center; (C) Diagram showing relative location of device at the left ventricle (LV) apex and
the outflow cannula to the central aorta; (D) View showing relative location in the chest of the components of the device.
Source: Used with permission from HeartWare International.

Fig. 57.3: The Abiomed Impella is moved retrograde from the

femoral artery to the central aorta and across to the left ventricle.
Blood is pulled from the left ventricle (LV) cavity by the Impella and
ejected into the central aorta.
Source: Used with permission from Abiomed.

at the center, where the spinning generates a vortex of

low pressure, with progressively higher pressures as one
reaches the outside of the spinning disk. These devices
typically rotate between 2,000 and 3,000 rpm. The first
clinical application of a third generation device occurred
in 2005 with implantation of the VentrAssist.4

CLINICAL USES OF VENTRICULAR

ASSIST DEVICES
Ventricular assist devices have multiple uses for multiple clinical circumstances. The original devices were

conceived as bridge to recovery devices to allow temporary support of patients expected to recover from a
temporary but severe clinical situation. Devices designed
for this purpose are in clinical use today and typically are
percutaneously deployed rapidly in urgent situations.
These devices provide a greater level of assist and output
response than an intra-aortic balloon pump. Devices may
be small, impeller-type systems such as the Impella series
of recovery devices (Abiomed) designed for very shortterm use, or the TandemHeart (Cardiac Assist), which is
an extracorporeal centrifugal flow pump. This device may
also be used for relatively brief periods of time (Figs 57.3
and 57.4).
Long-term assist devices are now predominantly axial
flow devices or centrifugal pumps. These devices may be
directly implanted in individuals who are severely ill or in
individuals who are chronically severely debilitated and
require emergent, urgent, or elective placement of the
device. The most detailed data on the use of these devices
comes from the Interagency Registry for Mechanically
Assisted Circulatory Support (INTERMACS).5 INTERMACS
has defined seven different profiles of patients based upon
clinical severity and is tracking these patients over time
to determine outcomes. Ongoing evaluation of patients
having these devices placed will help determine best
practices. The basic INTERMACS profiles are shown in
Table 57.1.
As assist devices have evolved, so have indications.
Bridge to recovery is predominantly confined to acute
devices, with the larger devices showing only very

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1225

Figs 57.4A and B: (A) The TandemHeart device. An inflow catheter is inserted in a femoral vein and moved up to the
right atrium and then across to the left atrium. Blood is removed from the left atrium outside to the pump and then via
the outflow cannula ejected into the central aorta; (B) Closer look at ideal position of the inflow cannula. Note there are
14 side holes and 1 end-hole.
Source: Used with permission from Cardiac Assist, Inc.

Table 57.1: Classification of Levels of Severity of Patients who are Potential Candidates for a Ventricular Assist Device: The
Interagency Registry for Mechanical Assisted Circulatory Support (INTERMACS)

Profile Percentage of Implants* Profile Name

Profile Description

16

Critical Cardiogenic Shock

Life threatening despite inotropic support, hypotensive, and

hypoperfused

38

Progressive Decline

Declining systemic function, nutrition, and renal function despite

inotropic support

28

Staple Inotropic Dependent Achieved stable blood pressure, organ perfusion, and nutrition but unable to be weaned from inotropic or other temporary
mechanical support

12

Resting Symptoms

Stable with normal volume status on oral medications. Daily

symptoms of limiting congestion at rest or with minimal activity

Exercise Intolerant

No symptoms with rest or minimal activity. All other activity

causes congestion. Frequent episodes of volume overload

Exertion Limited

No resting symptoms. No fluid overload. Minimal activity can be

performed but fatigues in a few minutes

*Percentage of implants for the year 2011.

Source: Adapted from INTERMACS, accessed at www.intermacs.org January 27, 2013.

1226

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Table 57.2: Frequency of Ventricular Assist Device Implantation by Device Strategy: The INTERMACS Registry

Device Use Strategy

Implant Year
2007

2009

2011

Bridge to Transplant

147 (42%)

505 (49%)

424 (22%)

Bridge to Candidacy

134 (38%)

432 (42%)

695 (37%)

Destination Therapy

48 (14%)

59 (6%)

742 (39%)

Bridge to Recovery

14 (4%)

13 (1%)

17 (1%)

Other

8 (2%)

11 (1%)

20 (1%)

351

1,020

1,898

Total Implants

Source: Adapted from INTERMACS, accessed at www.intermacs.org January 27, 2013.

modest use for this indication. With greater longevity

of use, indications have expanded into several other
large categories. Bridge to transplant is for patients who
are expected not to survive until a transplant becomes
available. As the demand for transplants has increased
but the availability has remained static or actually slightly
decreased, the waiting time for a heart transplant continues
to prolong. Thus, bridge to transplant now requires longterm support of these newer devices. The success of bridge
to transplantation has led to considerations for use of
these devices as an ultimate end in itself. This is called
destination therapy. Patients in this category are not
candidates for cardiac transplantation but are candidates
for prolonged assist to help improve longevity and quality
of life. A final major category is called bridge to candidacy.
These patients typically, at the time of evaluation, are
found not to be candidates for transplantation. However,
the impediment to transplantation may not be permanent
and could be reversed by ongoing therapy. Thus, there is a
possibility that these patients might be listed in the future
for transplantation. If this occurs, the patient reverts to
bridge to transplantation. If this does not ever occur, the
patient reverts to destination therapy.
Current data from INTERMACS, as of September 30,
2012, lists 7,290 patients in the registry. In 2007, during the
first full year of the registry, bridge to transplant was the
most frequent reason for placement of an LVAD. Over the
next 2 years, bridge to transplant and bridge to candidacy
were the predominant reasons for LVAD implantation.
With changes in approval of devices, 2010 saw a dramatic
increase in destination therapy, and in 2011 destination
therapy became the most frequent indication for an LVAD.
The relative frequency of these categories of implantation
is shown in Table 57.2 for selected years.5

REVERSE REMODELING
Placement of a LVAD generally causes significant reverse
remodeling. While it was hoped initially that substantial
reverse remodeling would include both volumetric
reduction and functional improvement, to the point that the
LVAD might be removed, later studies in wide varieties of
individuals have shown that the ability to explant an LVAD
is relatively uncommon.6 In an analysis of neurohormonal
blood levels in patients with LVADs, some studies have
shown a decline in levels of endothelin-1 and B-type
natriuretic peptide associated with some improvement
in function. Analysis of myocardium taken from the core
sample at the time of LVAD placement and then at the
time of either explantation or transplantation has shown
improvements in cardiac myocytes. In particular, there has
been regression of cardiac cell hypertrophy and reduction
in overall size. In addition, there has been evidence of a
reduction in total collagen back toward control levels.
Replacement fibrosis for dead myocytes generally does
not revert, but the interstitium may change favorably
with unloading from the LVAD. Unfortunately, these
positive changes in matrix and myocytes have generally
not been translated into enough improvement to allow
explantation.7,8

TYPES OF DEVICES
Short-Term Circulatory Support
Short-term devices are indicated for patients with acute
cardiogenic shock or postcardiotomy shock, which could
involve the left ventricle, right ventricle, or both. In some
circumstances, these devices may be placed prior to
performing a high-risk percutaneous intervention.

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1227

Impella Catheter-Based Assist Device

Abiomed AB5000

This device is a continuous flow system that is quite small

and can be placed percutaneously across the aortic valve
or pulmonic valve. It is a continuous flow device designed
to provide partial circulatory support for up to 6 hours.
The output of the smaller version is about 2.5 L/min. The
inlet area of the device sits in the ventricle, pulling blood
out of the ventricle and ejecting it on the other side of the
valve into the central aorta or pulmonary artery. The pump
is 12 Fr in diameter. A positive response to placement of
this device consists of (1) a reduction in filling pressure
within the ventricle, (2) a reduction in mechanical work
and wall tension that reduces oxygen demand, and (3)
an improvement in cardiac output increasing oxygen
supply to the periphery. A larger version using a 21 Fr
pump motor is the Impella 5, capable of 5 L/min of output
(see Fig. 57.3).9

This is a first generation pulsatile assist device. It is designed

for short-term support. The cannulas are surgically
implanted and the device may be used in LVAD, right
ventricular assist device (RVAD), or biventricular assist
device (BiVAD) configurations. It is a bridge to recovery or
bridge to decision device.

TandemHeart System
The TandemHeart is an extracorporeal centrifugal pump.
The inflow catheter is inserted via a femoral vein, up the
vena cava and into the right atrium, and then via transseptal puncture placed in the left atrium. The device
removes oxygenated blood from the left atrium, out of the
body to the external pump, which then returns this blood via
a femoral artery into the central aorta. The inflow catheter
has 14 side holes and an end hole. Transesophageal
echocardiography is used by some operators to help
assist in trans-septal puncture and placement of the
inflow catheter. The TandemHeart has been compared
to intra-aortic balloon pulsation in patients presenting
with cardiogenic shock. The hemodynamic effects of the
TandemHeart appear to be superior (see Figs. 57.4A and B).
However, overall mortality was not reduced.10

Thoratec CentriMag System

This device is a magnetically levitated centrifugal pump
that is extracorporeal. It comes in an adult and pediatric
version, and can be used for up to 30 days as a bridge to
decision for cardiogenic shock of either the right or left
ventricle. It has been approved for use in various formats
in several countries. This device has the capability of
producing up to 10 L/min of flow and can be configured to
provide left ventricular, right ventricular, or biventricular
support, and also can be configured to work with an
extracorporeal membrane oxygenator (ECMO) circuit.11 It
does require a sternotomy and surgical placement of the
cannulas.

Thoratec Paracorporeal Ventricular

Assist Device
This device can be used as an external or internally
implanted device. The device has been available since the
1990s, approved for patients with end-stage heart failure as
a bridge to cardiac transplantation or for postcardiotomy
support. The device is a pneumatic-driven, pulsatile
device.

Long-Term Axial Flow Devices

For all of these devices, output across the LVAD is
determined by three major factors: (1) pump rotation
speed, (2) LVAD preload, and (3) LVAD afterload. Factors
(1) and (2) are inter-related and determine the pressure
differential across the pump. For a given rotational speed,
flow across the pump increases as the pressure differential
across the pump declines. For example, consider a patient
under two circumstances: a mean arterial pressure of
55 mm Hg versus a mean pressure of 80 mm Hg. In the
former situation, particularly during diastole, the LVaorta
pressure differential is relatively small and pump flow will
be higher, giving the patient a higher systemic output.
Doppler flow velocity, both inflow and outflow, will be
higher. As aortic pressure rises, if all other things stay the
same, pump flow declines.
The most significant determinant of LVAD preload
is residual contractility of the LV. During systole, a larger
pressure rise in the LV will decrease the LVaorta pressure
difference and propel more blood forward. The patient
will demonstrate greater pulsatility and a greater degree of
flow velocity during systole in both the inflow and outflow
cannula. The degree to which the aortic valve opens shows
the relative amount of time in which the cardiac cycle LV
systolic pressure exceeds aortic pressure. This will be an
independent contributor to pulsatility.
All long-term axial devices share a relatively similar
configuration. The inflow cannula is at the left ventricular
apex and the pump is in series with the cannula. Blood flow

1228

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

12,500 rpm, and can generate flow as high as 10 L/min. A

pediatric version has also been developed.

Jarvik-2000 FlowMaker
The pump is different from other axial flow devices in that
there is no inflow cannula. The pump is directly implanted
in the apex of the left ventricle but can also be used in the
right ventricle. This pump is also relatively small at 90 g
(Fig. 57.5).

Long-Term Third Generation

Centrifugal Flow Systems
These devices represent the newest design for LVADs.
They operate at lower speeds than axial pumps and may
have a longer life expectancy than the axial flow models.
Configuration with the heart is similar to that of the
axial flow system. Current examples of these devices are
discussed below.
Fig. 57.5: Diagram of the placement of the Jarvik-2000 Flowmaker. The device has no inflow cannula; it is inserted directly into
the left ventricular apex.
Source: Used with permission from Jarvik Heart.

is boosted by the pump and returned to the aorta, usually

the ascending segment. The lifespan of these devices is
projected for several years (see Fig. 57.1). Some examples
of current devices are discussed below.

Berlin Heart INCOR Assist Device

This pump is an axial flow pump with a magnetically
levitated impeller. The pump provides flow of up to 6 L/
min at a speed of 7,500 rpm. This device is manufactured
by Berlin Heart.

HeartMate II Continuous Flow

Left Ventricular Assist Device
This system is manufactured by Thoratec and is an axial
flow pump that is electrically driven. The rotor is suspended
between the inlet and outlet sites with ball bearings. This
device has been used for bridge to transplant, bridge to
decision, and destination therapy (see Fig. 57.1).

HeartAssist System
This is an axial flow pump that is relatively small, being
only 95 g in weight. The device rotates between 7,500 and

HeartWare Ventricular System

This device is a centrifugal pump with blood flow coming
into the center of the device and exiting from the periphery.
The pump is designed to be implanted entirely in the
pericardial space, without the need for any secondary
pocket. The device is inserted directly onto the apex, with
a very short inflow cannula. The outflow cannula is placed
at the central aorta (see Fig. 57.2).

DuraHeart Magnetically Levitated

Centrifugal Assist System
This is a third generation system with no mechanical
contacts; instead, it uses magnetic levitation between
the impeller and pump housing. The device is attached
via a cannula to the left ventricle, and outflow goes to the
central aorta.

Levacor Ventricular Assist Device

This is a centrifugal device operating between 800 and
3,000 rpm, providing flows up to 9 L/min.

EvaHeart Left Ventricular System

This is a larger centrifugal pump implanted into the
left abdominal wall, weighing 420 g. The device output
depends on head pressures within the pump similar to

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1229

Table 57.3: Partial List of Ventricular Assist Devices

Name

Manufacturer

Pump Type

Inflow/Outflow
Anatomic Site

Speed Range

Current Usage

axial/
catheter based

LV/aorta RV/PA

2550 K (2.5)
1030 K (5)

Short-term up
to 6 hours

LA/femoral artery

37.5 K

Short-term up
to 30 days

Percutaneous Placement for Short-Term Support

Impella

Abiomed (Danvers, MA)

TandemHeart

Cardiac Assist, Inc. (Pitts- centrifugal/extracorporeal

burgh, PA)

Short-Term Support (surgical placement)

Abiomed AB5000

Abiomed (Danvers, MA)

Pulsatile/extracorporeal

LV apex/aorta RA/
PA

Pulsatile

Short-term
support up to
30 days

CentriMag

Thoratec Corp.
(Pleasanton, CA)

Centrifugal/extracorporeal

LV apex/aorta RA/
PA BiVAD, ECMO

05.5 K

Up to 30 days
support

Incor

Berlin Heart (Berlin,

Germany)

Axial/intracorporeal

LV apex/aorta

7.5 K

Long-term
support

HeartMate II

Thoratec Corp (Pleasanton, CA)

Axial/intracorporeal

LV apex/aorta

615 K

Long-term
support

HeartAssist 5

MicroMed Technology
(Houston, TX)

Axial/intracorporeal

LV apex/aorta

7.512.5 K

Long-term
support

Jarvik 2000

Jarvik Heart (New York,

NY)

Axial/intracorporeal

LV apex/aorta RV/
PA BiVAD

812 K

Long-term
support

HeartWare HVAD

HeartWare International
(Framingham, MA)

Centrifugal/intrapericardial LV apex/aorta

1.84 K

Long-term
support

DuraHeart

Terumo Med Corp.

(Somerset, NJ)

Centrifugal/intrapericardial LV apex/aorta

1.22.4 K

Long-term
support

Levacor

World Heart Inc. (Salt

Lake City, UT)

Centrifugal/intracorporeal

LV apex/aorta

13 K

Long-term
support

EvaHeart

Sun Medical Technology

(Naguno, Japan)

Centrifugal/intracorporeal

LV apex/aorta

12.8 K

Long-term
support

Long-Term Support

(LV: Left ventricle, PA: Pulmonary artery, BiVAD: Biventricular assist device, ECMO: Extracorporeal membrane oxygenator; RA: Right
Atrium).

axial devices. The greater the pressure differential between

LV and central aorta, the lesser the flow that goes through
the pump. On the other hand, when the LV contracts in
systole, the pressure between LV and aorta falls, facilitating
more pump flow. In this way, even though a continuous
flow device is used, the patient maintains a degree of
pulsatility.12
A summary of more commonly available devices is
listed in Table 57.3. Not all devices are universally available.

PREOPERATIVE ECHOCARDIOGRAPHIC
EVALUATION
Once clinical criteria have been established that
suggest a ventricular assist device may be necessary, a

comprehensive echocardiogram should be performed to

further refine information about the patients candidacy.
It is important that a full, comprehensive echocardiogram
be performed, with full two-dimensional (2D) views from
all standard imaging planes, along with color Doppler,
pulsed wave, and continuous wave Doppler assessment,
and appropriate dimension measurements. In some
circumstances, three-dimensional (3D) imaging may
help further define potential abnormalities, and the use
of contrast may be necessary to fully define structures and
function.13,14

Left Ventricle
The left ventricle should be carefully examined with full
long-axis and short-axis imaging. Particular attention

1230

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.6A and B: Two examples of apical thrombi. (A) Large thrombus is easily seen in the apical region of the ventricle; (B) This
thrombus was not apparent with routine imaging but was much better characterized with contrast.

Fig. 57.7: Severe dilated cardiomyopathy baseline study. Dimensions serve as a frame of reference for serial follow-up after placement of the device. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
RV: Right ventricle).

Fig. 57.8: Apical four-chamber view of the heart. The septal

contour preleft ventricular assist device (LVAD) is rightward and
left atrial volume is markedly increased. (LA: Left atrium; LV: Left
atrium; RA: Right atrium; RV: Right ventricle).

should be paid to the left ventricular apex. Care should

be taken to determine if there is any evidence of a
possible thrombus in the apex since this part of the heart
will be cored out for attachment of the inflow cannula
(Figs 57.6A and B). In addition, characterization of the
apex for any unusual shape changes or trabeculations is of
value to the surgeon. If there is a question about visibility
of endocardial ventricular function or the apex, contrast
agents should be used to enhance visualization. Careful
2D dimensions should be measured from the parasternal
long-axis view to document overall ventricular size at enddiastole and end-systole, and also wall thickness. These
values will serve as a baseline for serial evaluation of the
patient after placement of the ventricular assist device

(Fig. 57.7). The ejection fraction should be quantified;

typically, patients qualifying for ventricular assist devices
should have a severely reduced ejection fraction of < 30%.
Views should be obtained in both short-axis and apical
views to adequately define the baseline contour of the
septum so that changes in septal shape can be judged
appropriately after placement of the LVAD (Fig. 57.8). The
apex should also be assessed in ischemic heart disease
patients for the possibility of an apical aneurysm.
An evaluation of diastolic performance is also
important as a baseline. Full diastolic evaluation including
mitral inflow patterns and velocities, tissue Doppler
evaluation of the medial and lateral mitral annulus, and
pulmonary vein inflow should be recorded.

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1231

Fig. 57.9: Example of a patient with severe mitral regurgitation due to papillary muscle dysfunction. In most cases, severe
mitral insufficiency is not a contraindication for left ventricular assist
device (LVAD) placement. In many patients, decompression of the
left ventricle (LV) improves coaptation. (RV: Right ventricle).

Fig. 57.10: Aortic valve (AV) with significant calcification and on

evaluation was severely stenotic.

Left Atrium

of calcification, stenosis, or significant insufficiency could

create significant problems for this device and result in
ineffective support (Fig. 57.10).
For placement of standard apical continuous flow
devices, baseline evaluation of the aortic valve is also
critical. Careful evaluation of the leaflets should be
performed in short-axis views, and in some circumstances
might be enhanced by 3D views to determine how much
sclerosis, calcification, and leaflet motion restriction is
present. Forward flow velocities should be quantified,
and if there is any evidence of aortic stenosis, this should
be calculated and quantified. Most importantly, the
severity of aortic insufficiency should be fully quantified.
Patients with more than mild aortic insufficiency could
be at substantial risk if the valve is left alone. If significant
stenosis is present, the valve may need to be replaced,
since the patient could be put at substantial risk if the
LVAD suddenly failed.

It is important to assess the size of the left atrium. This is

best done by evaluating left atrial volume from a biplane
volume calculation (see Fig. 57.8). In some cases, it may
also be necessary to evaluate the left atrium and the left
atrial appendage with transesophageal echocardiography
if there is a strong suspicion of a thrombus in the atrial
chambers.

Mitral Valve
The mitral valve morphology should be evaluated.
LVADs are contraindicated in patients with significant
mitral stenosis. This is readily evaluated by Doppler
flows across the mitral valve. The motion of the leaflets
should be determined and the severity of mitral regurgitation evaluated (Fig. 57.9). In most circumstances,
mitral regurgitation will be expected to improve with
decompression of the left ventricle after placement of the
LVAD. Appropriate quantification of the severity of mitral
insufficiency preoperatively helps establish a baseline to
evaluate the effect of the LVAD after it is implanted.

Pericardium
The pericardium should be assessed to determine if any
significant effusion is present or if there is any evidence
that suggests the presence of constriction.

Aortic Valve
Aortic valve anatomy and function is particularly important
if the temporary impeller device is to be utilized. This
device works well if the aortic valve is normal, but presence

Atrial Septum
The atrial septum is an important structure, particularly
since relative filling pressures may change dramatically

1232

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.11A and B: (A) Dilated cardiomyopathy with small secundum atrial septal defect (ASD) with shunt shown in orange going
left to right. This was not detected in the past and may be due to stretching open of the foramen caused by chamber enlargement;
(B) Different patient with heart failure and a larger atrial septal defect. Note large orange left to right shunt jet. Both require closure during
left ventricular assist device (LVAD) surgery.

after placement of the LVAD. The patient should be

evaluated for the possibility of a patent foramen ovale
in the preoperative assessment. Careful evaluation
of the atrial septum from multiple views, particularly
the subcostal view, may detect shunting. In some
circumstances with very large hearts and persistently high
filling pressures, a previously patent foramen ovale may
stretch out, allowing a left-to-right shunt to be present
(Figs 57.11A and B, Movie clips 57.1 to 57.3). This is usually
readily quantified in the subcostal view. If no color flow
abnormalities are present, a bubble study should be
performed with injection of agitated saline intravenously.
This should be done with and without Valsalva. Careful
attention should be paid to an assessment of left atrial
filling pressures. In some circumstances, there may be
such a differential between left and right atrial pressures
that even a Valsalva maneuver may be ineffective in
detecting a patent foramen ovale. If there is a question
about the presence of a foramen ovale, a transesophageal
echo can be considered for further evaluation. Unusual
anatomy of the atrial septum, such as a large atrial septal
aneurysm or substantial lipomatous hypertrophy of the
atrial septum, could affect the trans-septal puncture
necessary for placement of the TandemHeart. If need be,
transesophageal echocardiographic guidance may be
necessary to help placement of a device.

Aorta
Examine as much of the aorta as possible. Off-axis views
may be of value to show as much of the ascending aorta as

can be demonstrated. Size of the annulus, sinus of Valsalva,

sinotubular junction, and proximal ascending aorta
should be determined and as much of the aorta examined
as possible for the possible presence of atherosclerosis that
might affect placement of the cannula. Suprasternal views
of the arch should also be performed and subcostal views
can be obtained to evaluate the upper abdominal aorta.
The presence of an untreated ascending aortic aneurysm
> 5 cm in diameter is considered a contraindication.

Tricuspid Valve
The tricuspid valve can pose particular challenges in the
perioperative period. Careful evaluation of the severity
of tricuspid valve insufficiency is essential from all
standard views. Characterization of leaflet motion and
morphology, along with measurement of the diameter of
the annulus, can be of considerable help to the surgeon in
determining whether or not tricuspid valve repair is indicated during the procedure (Figs 57.12A to D and Movie
clips 57.4 and 57.5).1517

Pulmonic Valve
The pulmonic valve should be evaluated to exclude
any unknown stenosis and also carefully evaluated to
determine if there is any significant pulmonic valve insufficiency. This is particularly important in patients who may
have underlying congenital disease. Substantial amounts
of pulmonic valve insufficiency could contribute to right
ventricular overload following placement of the LVAD.

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1233

Figs 57.12A to D: Tricuspid valve. (A) Valve morphology shows evidence of mild leaflet thickening and reduced excursion due to right
ventricle (RV) enlargement and dysfunction; (B) Severe valve insufficiency is present; (C) Coaptation is severely compromised, leaving
a visible regurgitant orifice (arrow); (D) The annulus is markedly dilated. This patient had tricuspid valve repair when the left ventricular
assist device (LVAD) was placed. (LV: Left ventricle; RA: Right atrium).

Inferior Vena Cava

The inferior vena cava should be imaged and its diameter
measured, and an evaluation of estimated central venous
pressure made. This, combined with the tricuspid
regurgitant jet, can be used to assess the severity of
pulmonary hypertension. This is particularly important if
there is disproportionate elevation of pulmonary pressures
that might indicate independent pulmonary artery disease
or reactive pulmonary hypertension.

Right Ventricle
Perhaps the most difficult and perplexing pre-LVAD
evaluation is that of the right ventricle. The concern is

always that of how the ventricle will respond during

surgery and how it will respond to placement of the LVAD
after the fact. Will the ventricle maintain good function
and not be a limiting factor on the patients overall cardiac
output and functional performance, or will it deteriorate
and become the rate limiting structure that controls rightsided congestion and cardiac output? Significant right
ventricular heart failure occurs in 2030% of post-LVAD
patients. Survival is reduced in right heart failure patients,
morbidity is increased, and length of stay prolonged.18,19
Frequently, clinical characteristics of patients do not differ
between those who do well post-LVAD and those who
develop right ventricular failure. Detailed assessment of
the right ventricle is important and frequently overlooked

1234

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

in standard echocardiographic evaluations. A special

effort should be made to characterize right ventricular
size and function from multiple views, particularly apical,
subcostal, and short-axis views. As 3D imaging improves,
better transthoracic assessment of the right ventricle may
be possible in the future.
Because the right ventricle is so difficult to evaluate,
a large number of proposed tests, measurements, ratios,
fractional changes, excursion measurements, indices,
and point score systems have been proposed (Figs 57.12
to 57.14 and Movie clip 57.6). No one measurement
is foolproof and no particular measurement is considered a gold standard. Table 57.4 summarizes only
echocardiographic-based measurements, including a
point score system based on left-sided measurements.
The point score system is a single center study that uses
simple LV measurements. This study suggests that smaller
LV size with better preserved LV systolic function but
higher levels of filling pressures (represented by the larger
LA) are more likely associated with a worse RV prognosis.26
Table 57.5 is an overall summary of the preoperative
echocardiographic assessment.

IMMEDIATE POSTSURGICAL
EVALUATION
Immediate postoperative evaluation is generally
performed by the cardiac anesthesiologist after the patient
comes off the pump.29 The immediate surgical results of
the LVAD placement should be evaluated, along with the
response of the ventricles and valves.
Before the assist device is activated, an immediate
evaluation for residual air bubbles is undertaken in the
ventricular chambers, ascending aorta, and cannulas.
Activation of the LVAD should begin appropriate
unloading and should result in a slight change in
interventricular and intra-atrial septum contours to
the left. Lack of effective decompression will result in
substantial rightward deviation of the septum, indicating
suboptimal support from the assist device or inadequate
settings to unload the left ventricle. This may require rapid
assessment of LVAD function and the device cannulas.
The opposite can also occur. Extreme leftward shift of the
septum suggests the possibility of an excessively high pump
speed that may be unloading the left side too vigorously. It
could also be caused by right ventricular dysfunction; thus,
right ventricular function should be assessed immediately
along with tricuspid valve performance, particularly for
severity of tricuspid regurgitation. An immediate concern

in this situation is whether or not right ventricular function

is adequate to sustain an adequate cardiac output in the
immediate postoperative setting (Fig. 57.15).
Another potential issue, particularly if there is leftward
deviation of the atrial septum, is the evaluation for a
patent foramen ovale. A large right-to-left shunt could
cause significant hypoxemia. Sometimes this is missed in
the preoperative assessment due to excessively high leftsided pressures. If the preoperative contrast bubble study
is abnormal and suggests a significant right-to-left shunt,
consideration needs to be given for closure of the patent
foramen ovale during the procedure.30
The inflow and outflow cannulas are inspected.
The inflow cannula should be inspected to determine
the orientation. It should be angled toward the mitral
valve and aligned with the left ventricular outflow tract.
Doppler evaluation should be performed. A normally
functioning inflow cannula generally has relatively low
velocity laminar color Doppler flow with low velocities
by pulsed wave Doppler. Flow signals deviating from
this should raise suspicion about possible obstruction
or pump malfunction. Particularly concerning would
be a regurgitant flow signal at the site of the cannula,
indicating backflow through the pump. One exception is
the Jarvik-2000. The pump is actually in the LV apex.
The outflow cannula is generally visible in high
esophageal views and should show relatively low velocities
outward from the cannula (Figs 57.16A and B).

SERIAL CHANGES IN CARDIAC

STRUCTURE AND FUNCTION
Ventricular Size and Function
The echocardiography laboratory and the heart failure
program should set up a regular schedule for evaluation of
patients with an LVAD. This allows for regular surveillance
of effectiveness of the device and for serial changes in
performance of the device. A suggested schedule could be
month 1, month 3, month 6, month 12, and every 6 months
thereafter. A comprehensive study should be performed
similar to a regular echocardiographic exam. As many
normal views as is possible should be obtained, realizing
that all views may not be available. Certain specialized
views are also necessary; some of these are outlined in
Figure 57.17.30,31
The left ventricle should be assessed particularly
for changes in size and function. The expected response
to placement of an outflow tract device would be a

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1235

Table 57.4: Evaluation of the Right Ventricle: Factors that May Impact Response to Placement of Left Ventricular Assist Device

Factors

Comment

Tricuspid Valve
Severity of regurgitation (Fig. 57.12)

May identify patients who would benefit from tricuspid valve

repair. Repair may have a survival benefit, recovery benefit, and
RV function benefit.15,16,1821

Size of annulus diameter (Fig. 57.12)

Annular diameter > 43 mm is associated with reduced survival.17

Duration of tricuspid regurgitation in systole (Fig. 57.13)

A rate corrected value of less than 461 ms indicates a worse

2-year prognosis.22

Right Ventricular Function

Semiquantitative evaluation of function

Severe systolic dysfunction associated with worse outcome.23

Fractional area change

Values < 20% are associated with increased risk of post LVAD RV
dysfunction.24

TAPSE (Fig. 57.14)

Annular motion < 7.5 mm predicts post LVAD RV failure.25

Right Ventricular Geometry

Short-axis/long-axis ratio of the RV

Ratio > 0.6 associated with worse outcome

RVEDD/LVEDD ratio (by transesophageal echocardiography) Ratio > 0.72 associated with adverse outcome.17,26
Right-sided Hemodynamics
Central vanous pressure (CVP)

Elevated pressure and ratio of CVP to PCWP > 0.64 associated

with worse outcome.19

Estimated RV systolic pressure

Conflicting findings, higher25 or lower27 RV systolic pressure

associated with worse outcome.

Left ventricle parameters point score system for predicting RV

failure after LVAD
Parameter

The point score system for detecting RV failure showed the

following performance.28

Points

LVEDD
> 78 mm

7078 mm

< 70 mm

LVEF
< 19%

1933%

> 33%

Sensitivity

Specificity

3 points

88.6%

47.4%

4 points

71.4%

67.1%

5 points

42.9%

80.3%

LAD/LVEDD
< 0.63

0.630.68

> 0.68

(CVP: Central venous pressure; LAD: Left atrial dimension; LVEDD: Left ventricular end-diastolic dimension; LVAD: Left ventricular assist device; RV: Right ventricle; RVEDD: Right ventricular end-diastolic dimension; TAPSE: Tricuspid annular plane systolic
excursion).

1236

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Table 57.5: Points of Emphasis for the Pre-Left Ventricular Assist Device Echocardiogram

Left Ventricle: Size, geometric shape, systolic function, diastolic function, and filling pressure
Left Ventricular Apex: Shape, trabeculae, and thrombus
Aortic Valve: Leaflet motion, morphology, severity of valve insufficiency, and presence of stenosis
Mitral Valve: Presence of stenosis, motion of leaflets, and severity of valve insufficiency
Tricuspid Valve: Annular dimension and severity of valve insufficiency
Atrial Septum: Patent foramen ovale and atrial septal defect
Pulmonic Valve: Severity of valve insufficiency
Inferior Vena Cava: Estimate of central venous pressure
Aorta: Ascending aortic size and atherosclerotic plaque
Pericardium: Effusion or constrictive changes
Right Ventricle: Size, geometric shape, and systolic function

Figs 57.13A to C: Two examples of calculation of tricuspid regurgitation duration. In (A) the duration is quite prolonged, giving a rate
corrected value of 631 ms. This puts the patient in a prognostically
favorable group; In (B) the rate corrected value is 458 ms, putting
the patient in the prognostically less favorable group even though
the severity of the valve regurgitation was moderate; (C) and
right ventricular systolic pressures were only mildly increased.
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle).

20 30% reduction in dimensions. These are most effectively

performed using direct 2D diameter measurements at the
tips of the mitral valve in the parasternal long-axis view
(Figs 57.18A and B and Movie clips 57.7 and 57.8). Attempts

should be made to reproduce the same views each time the

study is obtained. Measurements of ventricular ejection
fraction and function are sometimes difficult to obtain due
to the inability to get true long-axis views from the apex.

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1237

Figs 57.14A to C: Evaluation of right ventricular function.

(A) Tricuspid annular plane systolic excursion (TAPSE) in this
case is markedly reduced consistent with a reduced prognosis;
(B) Transthoracic measurement of the mid-right ventricle (RV) and
mid-left ventricle (LV) diameter, this example the ratio is 0.41, a
favorable prognostic finding; (C) Evaluation of fractional area
change of the RV in the apical four-chamber view. The end diastolic and end systolic areas are shown. The fractional area change
is only 11%, an unfavorable prognostic finding.

Fig. 57.15: Immediate postoperative transesophageal echo. The

left ventricular assist device (LVAD) has been ramped up to 8,800
rpm but the left ventricle has become suctioned down to a small
volume. In systole, the inflow cannula is now obstructed causing
flow acceleration at the inflow site that aliases the display. As soon
as systole concludes, inflow abruptly slows to normal.

Since loading conditions are no longer natural, evaluation

of an ejection fraction may not have the same meaning in
an LVAD patient.
While imaging the ventricle, it is also important to
obtain views of the inlet cannula at the apex. This should
be visible over 90% of the time and may be imaged from
low parasternal off-axis views, from distal short-axis views
in the parasternal view, and from various apical or off-axis
apical views. Cannula position and direction should be
shown as best as possible. Color flow Doppler should be
utilized in these views and under normal circumstances
should show laminar relatively low velocity flow. Pulsed
wave inflow velocities should be obtained at the origin
of the cannula. These velocities should generally be fairly
low, gently pulsatile, and average about 1.01.5 m/s, and
always less than 2.0 m/s (Figs 57.19A and B).32
In most patients, the outflow portion of the cannula
attaches to the pump in the abdomen, thorax, or
pericardium. The course of a typical cannula can be

1238

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.16A and B: (A) Transesophageal echo high esophageal view showing location of the outflow cannula in the ascending aorta
with color flow signal. The arrows show aorta landmarks for reference of location; (B) Continuous wave Doppler of outflow showing
pulsatile flow preservation. (AV: Aortic valve annular plane; ST: Sinotubular junction).

followed using off-axis modified parasternal and right

parasternal views. The relative position in the chest of
outflow cannulas is shown in Figures 57.1 and 57.2.
The transthoracic view on the cannula anastomosis is
shown in Figure 57.20A with a typical color flow signal in
Figure 57.20B and pulsed Doppler in Figure 57.20C. Off
axis images from the suprasternal notch or right parasternal imaging positions are usually needed to see the
outflow cannula and anastomotic site.
The right ventricle should be assessed for size and
function. This should be done from standard views and
apical views that emphasize the right ventricle. The septal
contour should be assessed. Generally most favorable is
a relatively neutral position between the two ventricles.
This indicates adequate but not excessive unloading
(Figs 57.21A to C and Movie clips 57.9 and 57.10). Similarly,
the septum can be assessed in the parasternal short-axis
views as one sweeps from base to apex. Under normal
circumstances, the contour of the curvature should
remain reasonably normal. The atrial chambers should be
evaluated as with normal studies and the volume of the left
atrium measured. It is expected that left atrial volume will
diminish over time. The junction of the inferior vena cava
and the right atrium should also be evaluated and central
venous pressure estimated. Hepatic vein Doppler signals
can also be assessed throughout a continuous respiratory
cycle. Valvular performance should be evaluated. The
mitral valve is expected, with appropriate unloading,
to show a reduction in regurgitation. With reduction in
left ventricular size, motion of the leaflets might also

improve some, lessening the severity of papillary muscle

dysfunction. The tricuspid valve is less predictable. The
severity of insufficiency may or may not change depending
on changes in size and function of the right ventricle.
In some situations, the tricuspid valve will have been
repaired, and so the repair should be evaluated for both
stenosis and insufficiency.
Of considerable importance is the aortic valve.
Motion of the aortic valve should be carefully evaluated
from multiple views to determine if the valve is opening
or not, and if it is opening, if it is opening every beat,
intermittently, or partially on each beat. Changes in
aortic valve morphology should also be assessed by
careful evaluation of the short-axis view. Color Doppler
examination is also important for determining if aortic
insufficiency has developed.33,34 Motion of the aortic valve
will help determine whether speed settings of the device
are optimal. At higher rotational speeds, the LVAD takes
over relatively greater amounts of output, and in many
circumstances the valve no longer opens. As speeds
are reduced or as the left ventricle starts improving,
some native contribution to outflow is again observed
(Figs 57.22A to D and Movie clips 57.11 and 57.12).
Diastolic performance of the ventricle can also be
assessed by standard indices of tissue Doppler, and
mitral valve flow and pulmonary venous inflow. In
general, successful unloading of the left ventricle results
in a reduction in left-sided filling pressures, but there is
no evidence that any intrinsic improvement in diastolic
function occurs (Figs 57.23A and B).35 Doppler assessment

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

Fig. 57.17: Specialized views obtained to help visualize structures in the patient with a left ventricular assist device (LVAD).
Source: Reproduced with permission from Elsevier.

1239

1240

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.18A and B: Example of pre- and post-left ventricular assist device (LVAD) dimensions taken at 3 months following LVAD placement. There has been significant decompression of the left ventricle (LV), with a 25% decline in LV end diastolic dimension.

Figs 57.19A and B: (A) Example of off-axis apical view showing the inflow cannula having relatively low inflow velocity (B).

of LV filling shows these changes. On the right side of the

heart, the typical response is a reduction in pulmonary
artery pressures and also central venous pressures.
Right ventricular function does not show significant
improvement over time in most patients.
Table 57.6 shows some selective data from a single
center study demonstrating some of the typical serial
changes expected after placement of an axial flow device.

COMPLICATIONS OF LEFT
VENTRICULAR ASSIST DEVICES
Evidence of Left Ventricular Over-Filling
Normally, the LVAD is expected to decompress the left
ventricle, reduce its size, and maintain the changes

over time. In some cases, there is evidence of recurrent

enlargement of the ventricular chamber. This can be
detected qualitatively or more precisely quantitatively
by noting dimension changes in the left ventricle. If the
ventricle dilates again, the septum may deviate more
rightward as filling pressures increase on the left side.
Reduced cardiac output may be associated with evidence
of stasis, causing visible spontaneous contrast in both the
left ventricle and left atrium. Mitral regurgitation may
increase in severity if the mitral valve apparatus is stretched
and the annulus increases in size. In most settings, there is
evidence of over-filling associated with a relative reduction
in cardiac output moving through the assist pump and a
relative increase in stroke volume across the aortic valve.
This typically is manifested by an increase in amplitude
and frequency of aortic valve opening from none, minimal,

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1241

Figs 57.20A to C: Transthoracic echo example of the right

parasternal view of the outflow cannula as it joins the central aorta.
(A) Note relative size of cannula; In (B), typical color flow signal of
flow in same location; In (C) pulsed Doppler at the outflow is shown
with normal velocities. (Ao: Aorta).

Figs 57.21A and B

1242

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.21A to C: (A) Apical four-chamber view showing a normal

septal contour with mild residual right ventricular dysfunction. In
(B) and (C) the patient has right ventricular dysfunction causing
an abnormal septal contour in the parasternal long- and shortaxis views. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

Figs 57.22A to D: (A) Example by M-mode of intermittent opening of the aortic valve (AV); (B) Typical central jet that can develop after
left ventricular assist device (LVAD) placement; (C) M-mode with color of same patient as (B) shows continuous nature of insufficiency
flow; (D) Continuous wave (CW) Doppler from the apical view in the same patient. Note that even though the left ventricle (LV) contracts,
it does not generate enough force to open the valve.

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1243

Figs 57.23A and B: (A) Mitral flow prior to placement of left ventricular assist device (LVAD) when the patient was in class IIIb heart failure; In
(B) the LVAD has been in place for 2 months. Note the improvement in deceleration even though the E/A ratio remains elevated.
Table 57.6: Early Serial Changes in Ventricular Size and Function after Placement of a Left Ventricular Assist Device

Pre-Op

Three Months

Six Months

LVEDD (mm)

70

61

60

LVESD (mm)

62

54

54

Biplane LVEF (%)

17

18

16

RVEDD (mm)

35

34

34

RV Function

1.5

1.6

1.4

CVP (mm Hg)

12

RVSP (mm Hg)

46

26

27

Mitral E/A Ratio

2.5

1.6

1.5

Mitral DT (ms)

146

200

195

E/e Ratio

13.3

11.8

11.4

Diastolic Grade*

2.5

2.0

2.1

LA Volume (mL)

95

73

65

*For both RV function and severity of diastolic dysfunction, the score was: 0 = normal, 1 = mildly abnormal, 2 = moderately abnormal,
3 = severely abnormal.
(CVP: Central venous pressure; DT: Deceleration time; EDD: End-diastolic dimension; EF: Ejection fraction; ESD: End-systolic
dimension; LA: Left atrium; LV: Left ventricle; RV: Right ventricle; SP: Systolic pressure).
Source: Adapted from reference 28.

or intermittent to present on every beat. There also may

be evidence of direct pump failure, which sometimes is
nonobstructive. Forward flow velocity is reduced and
LVAD regurgitation may occur (Figs 57.24A and B, Movie
clip 57.13). In some patients, the increase in flow velocity
may only occur at certain times in the cardiac cycle
(Figs 57.25A and B). If there is obstruction of the inflow
cannula, there may be a change in flow velocity, from the
normal laminar, relatively low velocity flow to an increase
in velocity that is turbulent. The cannula inflow area
should be interrogated with color, continuous wave, and

pulsed wave Doppler. It is also possible that the over-filling

and reduced flow is due to an abnormality of the outflow
from the pump; thus, the outflow cannula should also
be very carefully analyzed for evidence of turbulence or
change in velocity from one part of the cannula to another
(Figs 57.26A to C and Movie clip 57.14).
Specific potential causes of over-filling are categorized
in Table 57.7.
One specific difference must be noted for the case of
high afterload due to hypertension. In this case, the aortic
valve stays closed due to increased central aortic pressure.

1244

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.24A and B: Patient who suffered sudden power failure of his left ventricular assist device (LVAD). (A) There was a significant
change in the aortic valve M-mode that began fully opening in each beat; (B) The flow signal at the inflow cannula showed evidence of
weak forward flow, upper arrow, and regurgitant flow (lower arrow). The device did not clot in the 6 hours before surgery. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RV: Right ventricle).

Figs 57.25A and B: This patient had normal inflow cannula velocity (A). Two months later, there is a change in the inflow pattern consistent with obstruction. In this case, velocity peaks near end-systole (B). Peak velocity has increased from about 40 cm/s to almost
200 cm/s.

A
Figs 57.26A and B

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1245

Figs 57.26A to C: This patient showed evidence of reduced output.

The inflow cannula appeared normal by three-dimensional imaging
with low inflow velocity (A). The aortic valve began to open fully on
each beat (B) as shown by the arrows compared to previous intermittent opening (Fig. 57.22D). An increase of flow velocity was seen
in the outflow cannula (C) where a kink in the cannula developed.
The location of the kink was similar to that seen in Figure 57.1D
close to the anastomosis with the aorta.

Table 57.7: Causes of Left Ventricular Overfilling

Clinical Signs of Heart Failure

Possible echocardiographic
changes

Increased LVEDD
Septal contour shift rightward
Spontaneous contrast in LV, LA
Increase in mitral regurgitation
Change in mitral inflow signal, E/e suggesting increased filling pressure
Increased opening of aortic valve to every beat
Atrial septal contour shifted rightward

Cause
Pump readout changes

Specific echocardiographic
findings

Inflow obstruction

Pump failure pump thrombus

Outflow obstruction or high

afterload

Reduced flow

Reduced flow

Low pump flow

Increased power
consumption

Increased power consumption

Normal power consumption or

Spikes in power

Sudden flow reduction

High afterload: Increased

pulsatile index

Apical thrombus

Reduced inflow velocity

Thrombus in outflow cannula

Apical vegetation

Regurgitation signal from flow

reversal

Kink in outflow cannula

Adverse change in septum

contour

Doppler with different velocities

at different part of cannula

Increased inflow velocity

Anatomic obstruction of cannula

at aorta

Increased inflow turbulence

High afterload: High velocity

pulsatile LVAD flow

(LA: Left atrium; LV: Left ventricle; LVAD: Left ventricular assist device; LVEDD: Left ventricular end-diastolic dimension).

1246

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Outflow through the cannula becomes more pulsatile with

higher outflow velocity.

A second major abnormality is that of under-filling.

Under-filling may be associated with low flow but normal
overall power usage of the LVAD device. This could result
from an excessive reduction in preload. This may be a
consequence of an excessive speed setting of the device
and can be associated with set-down, in which the device
is intermittently obstructed at its inflow cannula site by
changes in contour of the interventricular septum or
apex (Figs 57.27A to C and Movie clip 57.15). The device is
typically programmed to reduce rpms to a default rate until
the obstruction is relieved. A second cause of this situation

is right ventricular failure. If the right ventricle deteriorates

after the LVAD is placed, septal contour may push from
right to left, indicating overload of the right ventricle,
reduced systolic performance of the right ventricle, and
thus a reduced cardiac output (Fig. 57.28). This situation
can be further complicated by worsening of tricuspid valve
insufficiency, which will further reduce forward flow out
of the right side of the heart and reduce volume delivered
to the left ventricle. In some situations, a third possibility
exists: right ventricular function may be preserved, but
the patient is excessively volume depleted. This could
be due to bleeding, poor intake, or excessive diuretic
use. There will be a decline in overall cardiac output due
to an excessive reduction in right ventricular preload
(Table 57.8). Restoration of normal fluid status brings
outputs back to baseline.

Figs 57.27A to C: The left ventricle (LV) cavity is small and the gap
between the cannula and septum satisfactory in systole (A)
but very small toward end-diastole (B). The inflow signal shows
increased flow velocity at end-diastole corresponding to the
findings (C). Diuretics were stopped and the left ventricular assist device (LVAD) speed reduced to 8,800 rpm. Inflow velocity
normalized.

EVIDENCE OF UNDERFILLING OF THE

LEFT VENTRICLE

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1247

Table 57.8: Causes of Left Ventricular Underfilling

Possible echocardiographic changes

Further reduction in LV end diastolic dimension

Leftward septal shift
Change in mitral inflow suggesting reduced LV filling and filling pressures
Aortic valve continuously closed
Atrial septum position variable

Potential causes

Excessive LVAD speed setting

Right ventricular failure
Increased tricuspid valve insufficiency
Dehydration (bleeding, excessive diuresis, poor intake, etc.)

Pump readouts

Reduced output
Normal power usage
Occasional set down episodes due to inflow cannula obstruction

Specific possible echocardiographic findings Low cannula inflow velocity

Change in septal contour to left or evidence of septal obstruction of inflow
Worsening RV function and high central venous pressure
Dehydration with reduced RV size, inferior vena caval signs of low or normal
central venous pressure
(LV: Left ventricle; LVAD: Left ventricular assist device; RV: Right ventricle).

Fig. 57.28: Example of right ventricle (RV) failure which causes

the septum to deviate to the left. (LV: Left ventricle).

High Left Ventricular Assist Device Flow

with Low Net Forward Cardiac Output
In most circumstances, this is associated with an
abnormality of the aortic valve, most frequently the

development of aortic insufficiency. With the position of

the pump circuit routing blood from LV to central aorta,
the presence of aortic insufficiency causes a direct loss of
forward cardiac output. Aortic insufficiency and changes
in the aortic valve have been evaluated in several serial
studies. The phenomenon of deterioration of aortic valve
leaflets, changes in coaptation, reduction in excursion of
the leaflets, and the development of continuous aortic
insufficiency have all been documented in multiple
series.32,34,36,37 Morphological changes on the valve have
been demonstrated. Changes in the valve may be related
to lack of opening motion. Some groups now suggest
that the aortic valve should be carefully observed at the
time of setup of pump speed, with pump speed adjusted
to allow at least intermittent or modest opening motion
of the valve. This may prevent deterioration of the valve
leaflets over time. More serial information is necessary
to determine more definitively the etiology and the
most appropriate settings. Aortic insufficiency is best
evaluated in the parasternal long-axis view. It may be
continuous depending on the status of LV ejection of blood
(see Figs 57.22 A to D and Movie clip 57.12).

1248

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Pericardial abnormalities may occasionally occur.

Development of a significant pericardial effusion, and
particularly cardiac tamponade physiology, may substantially change the loading conditions. The exact effect
on the heart is determined by the actual location of
the effusion (loculated or not). Hemopericardium can
complicate the early post-LVAD recovery period. Normal
diagnostic criteria for cardiac tamponade are not reliable,
particularly Doppler criteria. Chamber collapse is the best
indicator of tamponade.24,31

OPTIMIZING LEFT VENTRICULAR

ASSIST DEVICE SETTINGS
The goal of device optimization is to (1) preserve cardiac
output as much as possible, (2) eliminate congestion,
and (3) help the patient feel as well as possible. The LVAD
devices display certain pieces of information on the
controller system. For example, the HeartMate II displays
revolutions per minute (rpm; range 6,00015,000 rpm,
most commonly 8,00010,000 rpm), power consumption
in watts, flow, and the pulsatile index. The device is capable
of directly measuring rpms and power consumption. It
calculates flow and the pulsatility index based on the other
two pieces of data. The device calculations give reasonable
estimates of flow; however, at extremes of the rpm range,
for instance below 8,000, calculated flow rates become
less reliable. Flow is also determined by the pressure
gradient across the device. As speeds go up, left ventricular
systolic pressure will fall within the left ventricular
chamber relative to the central aortic pressure.
A comprehensive echocardiogram is necessary to
obtain full information in order to optimize speed settings.
In some circumstances, an interactive echocardiogram
may be performed using a ramp protocol to follow the
effect of serial pump adjustments on the echocardiogram.
From the echocardiogram, particular attention should
be made to obtain full information on the following:3840
Cannula flow velocities: Flow velocities should be
examined carefully at both the inflow site and the
outflow site. Flow velocities that are not elevated or
unusually shaped or turbulent indicate normal flow.
One should maneuver the transducer to optimize
obtaining flow in the most parallel position possible.
This will usually be from some type of an off-axis low
parasternal or apical view.
Ventricular septal contour position: The interventricular septum should be carefully evaluated. Use

of a respirometer may enhance the information by

allowing motion of the septum to be tracked with
the respiratory cycle. Under ideal circumstances, the
septum should stay at the midline. Turning device
rpms up too high may result in the septum drifting
leftward and actually collapsing the left ventricle. This
may also be observed in ramp protocols when one
is trying to quickly adjust the device to achieve an
optimal speed. On the other hand, device settings that
are too slow, that reduce flow out of the left ventricle,
may result in the septum drifting rightward to the right
ventricle. This can result in a reduced efficiency of right
ventricular performance and reduced output.
Atrial septum: Position of this structure indicates
a relative difference between LA and RA pressure.
One group40 has suggested that estimation of RA
pressure using the inferior vena cava dimension and
its response to respiration can be used to estimate LV
unloading. They assign LA pressure = RA pressure if
the atrial septum is midline, LA pressure = RA pressure
5 mm Hg if the septum is deviated to the left, and LA
pressure = RA pressure + 5 mm Hg if the atrial septum
is deviated to the right. If RA pressure is 15 mm Hg
or greater and LA pressure is equal to or greater than
RA pressure, these authors would suggest increasing
pump speed to further unload the LV.
Evaluation of LV filling characteristics may be done
directly by evaluation of the grade of diastolic filling
and estimation of filling pressures. Successful unloading should cause a reduction in diastolic grade, E/e'
ratio, and left atrial volume.35 The early mitral deceleration time can be measured and should prolong
as unloading occurs. The deceleration time can
be indexed by dividing deceleration time by peak
E velocity (e.g., 150 ms/50 cm/s = 3 ms/cm/s). A value
< 2 is associated with adverse outcomes and is another
indicator that suggests an increase in pump speed may
be beneficial (see Figs 57.23A and B).
The aortic valve should be carefully observed. There
has been much controversy about the optimal settings
with regard to aortic valve motion. It has been well
documented by multiple studies that the aortic
valve can deteriorate over time. Valve thickening
occurs, some fusion of leaflets may occur in certain
circumstances, and valvular insufficiency can develop
(see Figs 57.22A to D). It now appears that the best
policy is to set the speed so that there is intermittent
opening of the aortic valve. This has the salutary effect

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

of allowing a slight increase in pulsatility that could be

beneficial for the patient and also, more importantly
from the valve standpoint, it may reduce the likelihood
of fusion abnormalities, thickening, or development
of aortic insufficiency over time. It is no longer
recommended to set the speed so that the aortic valve
stays closed. Proof of long-term benefit of this policy is
not yet available.
Mitral valve performance: Optimal unloading of the
left ventricle should result in a reduction of mitral
valve insufficiency if it was present before placement
of the LVAD. In most settings, mitral valve insufficiency
is not due to primary valve disease; rather, it is due
to secondary valve disease from papillary muscle
dysfunction, annular enlargement, and reduced
excursion of the leaflet apparatus. In many patients,
as ventricular size is reduced, the severity of mitral
valve insufficiency may diminish. Ideally, the severity
of mitral insufficiency should be reduced to mild or
nothing with unloading.
Arterial pressure: An ideal setting would be a mean
pressure > 65 mm Hg.
Right ventricle: Optimal settings of the LVAD should
result in a reduction of filling pressures on the left side
of the heart that should be translated backward to the
right side. Optimization of the LVAD should result in
a reduction of right ventricular pressures over time.
Similarly, central venous pressure should come down
over time, particularly if there is fluid optimization that
occurs. A goal would be to lower RA pressure to 510
mm Hg through a combination of pump settings and
medical management.38
Cardiac output: Total cardiac output is best estimated
using pulsed Doppler flow at the right ventricular
outflow tract just above the pulmonic valve. Views
should be obtained to allow accurate measurement of
the diameter of the region. The flow velocity integral is
used in the formula:
RV outflow diameter/2 RV outflow TVI = stroke
volume
Stroke volume can be multiplied by heart rate to obtain
a cardiac output. An indexed value of 2.2 L/min/m2 or
greater is a reasonable goal for total outputs.
A formalized ramp test protocol for the HeartMate II
has been developed by Uriel et al.38 This protocol serially
evaluates device indices such as pulsatile index, power
output, and flow calculations from the device, along with
blood pressure and heart rate. The device is changed
through a range of 8,00012,000 rpm, if the patient

1249

tolerates this, in increments of 400 rpm. From an

echocardiographic standpoint, ventricular dimensions
are serially measured, the septal contour is observed, and
aortic valve opening and severity of aortic insufficiency are
observed, as are mitral insufficiency and right ventricular
systolic pressure estimates. From these measurements the
authors have been able to show characteristic changes in
their protocol, particularly when related to simultaneous
plotting of the pulsatility index, left ventricular enddiastolic dimension, and power output of the device.
Protocols of this nature can be used to optimize settings
by observing all the variables discussed above, and in
certain circumstances may also be used to evaluate for
the possibility of thrombosis in and around the device
because the characteristics of the ramp protocol change
when thrombus is present. A summary of optimization
goals and measurements is shown in Table 57.9.

EXPLANTATION
A small group of patients show recovery of function
sufficient to allow consideration for explantation.6 A few
disease processes are inherently self limited and have
an optimistic outlook for recovery. However, in cases of
true dilated cardiomyopathy, the chance of recovery is
very low. For instance, in one series of 1,108 patients,
20 (1.8%) had the HeartMate II explanted due to recovery
of left ventricular function. This trial and others have
also suggested that a nonischemic etiology is more likely
to recover. Of the 1,108 patients cited above, 531 were
nonischemic; they had a recovery rate of 3.4%. A group of
578 of the patients were ischemic; they had a recovery rate
of only 0.3%. Age also appears to be a significant factor.
Patients < 40 years of age have a higher rate of recovery,
and patients who have a shorter duration of heart failure
symptoms tend to have a higher rate of recovery.41,42 These
general findings have also been consistently found in
trials evaluating medical management of dilated cardiomyopathy.
Echocardiography plays a central role in identifying
patients who may be considered for explantation. Weaning
protocols were more easily performed with pulsatile
pumps since they could be temporarily slowed down to
a very low rate while patients were carefully monitored.
The transition to continuous flow devices has made
weaning more difficult. Some devices can be shut down
temporarily; others can only be partially slowed down.
Specific weaning protocols are beyond the scope of this
chapter and tend to be institution-specific.43 It should be

1250

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Table 57.9: Optimization of Left Ventricular Assist Device Performance: Echocardiographic Measurements Indicating a Favorable
Response

Cardiac Index: Cardiac Index > 2.2 L/m2/min

Ventricular Septum: Neutral at midline
Atrial Septum: Neutral at midline with an estimated right atrial pressure at 510 mm Hg
Mitral Flow Deceleration Slope Index: greater than 2 ms/cm/s
Left Atrial Volume: Reduced compared to baseline
Left Ventricular End-Diastolic Dimension: Reduced by 2030% compared to baseline
Aortic Valve Motion: Intermittent partial opening
Mitral Valve Regurgitation: Reduced compared to baseline
Tricuspid Valve Regurgitation: Reduced compared to baseline
Right Ventricle Size and Function: Stable or improved compared to baseline

noted that dobutamine stress testing has been tried in a

limited number of patients. Positive findings of enhanced
inotropic responsiveness combined with evidence of
improved cardiac output were shown to be valuable
predictors of explantation success in one study.
A more recent report from Dandel et al.44 suggested the
following echocardiographic parameters be considered,
and if met, the patient might be considered for weaning.
The left ventricle should have an end-diastolic
diameter < 55 mm and an ejection fraction 45%.
The right ventricle should not be dilated.
Valvular insufficiency of all four valves should be either
not present or only mild.
These factors are combined with right heart catheterization parameters. Recommended is a cardiac index
> 2.6 L/min/m2, a pulmonary capillary wedge pressure
< 13 mm Hg, and a right atrial pressure < 10 mm Hg.
If patients achieve this level of improvement of
performance and, importantly, show evidence of stable
maintenance of these changes over time, then patients
might be considered for a weaning protocol.

PERCUTANEOUS CONTINUOUS
FLOW DEVICES
Impella Device
This device is devised for short-term implantation. The
device is inserted generally through a large femoral
artery and retrogradely brought into position across
the aortic valve so that the distal portion of the device
suctions blood out of the left ventricle, across the
valve, and deposits the blood in the ascending aorta
(Fig. 57.3). Since this is a retrograde percutaneous device,
several different considerations before placement of the

device should be considered when reviewing either a

transthoracic or transesophageal echocardiogram. Prior to
implantation, besides the usual information obtained in a
comprehensive echocardiogram, one should pay particular attention to the presence of large atheromas in the
ascending aorta, size of the ascending aorta, abnormalities
of the aortic valve, particularly in regard to significant
stenosis that might restrict leaflet motion, increasing
the difficulty of placing the device across the valve, or
substantial aortic regurgitation, which might make the
device of little or no value. The left ventricular outflow
tract should be evaluated for possible unusual shape
or narrowing, and the mitral valve should be evaluated
for baseline insufficiency and any unusual chordal
abnormalities. During or immediately following positioning of the device, appropriate location of the device
across the aortic valve can be assessed echocardiographically by noting the location of the device relative to the
valve and also using color Doppler to verify inflow comes
from the left ventricle and outflow goes to the aorta
(Figs 57.29A to C).45 Transesophageal echocardiography
can be particularly valuable in assessing the location and
function of the device.46 For example, failure of the device
to improve patient status could be related to malposition
of the device. One case report recently described distal
positioning of the device with entrapment in the papillary
muscles, inhibiting outflow into the aorta. A 3D transesophageal echo (TEE) was demonstrated to be of particular
value in assessing the position of the device.47

TandemHeart
The TandemHeart (see Fig. 57.4) typically utilizes
a femoral vein and femoral artery with an external
pump.10 These devices are frequently placed using

Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices

1251

Figs 57.29A to C: (A) Parasternal long-axis view showing the

position of the Impella device (arrow); (B) Typical artifact generated
by the device; (C) M-mode of the device and aortic valve leaflets.

Figs 57.30A and B

1252

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 57.30A to C: Subcostal view confirming cannula position in

the inferior vena cava (IVC), arrow, (A). Apical four-chamber view
confirming correct location of the cannula tip (arrow); (B). In some
cases the cannula may migrate; in this case (C) the tip moved into
LV (arrow). (LA: Left atrium; RA: Right atrium; L: Liver).

C
echocardiographic imaging support, commonly transesophageal echocardiography. Preprocedure TEE
screening is of considerable value to evaluate not only
issues of ventricular and valvular function but also to
carefully evaluate the left and right atrial chambers for
any unusual anatomical abnormalities or thrombi that
would complicate placement of the large-bore catheter
moved retrograde up through the vena cava and then
trans-septally across the atrial septum. TEE is frequently
used for guidance of the trans-septal puncture, and
once the device is placed for correct positioning of the
retrograde catheter in the left atrium. Color Doppler can
assist in detecting flow since the device has multiple
holes at the end of its inflow catheter (see Figs 57.4A
and B). As with other assist devices, echocardiography can
serially evaluate the effect of the device. Also, if the device
appears to be working improperly, echocardiographic
imaging, particularly to evaluate the position of the inflow
cannula, may be of great value (Figs 57.30A to C and Movie
clips 57.16 to 57.17, 57.18). In some circumstances, this
cannula could migrate either further inward or backward
across the atrial septum and become malpositioned. These
catheters also can form clots and sometimes become
obstructed, which echocardiography can identify.

REFERENCES
1. Liotta D, Crawford ES, Cooley DA, et al. Prolonged partial
left ventricular bypass by means of an intrathoracic pump
implanted in the left chest. Trans Am Soc Artif Intern
Organs. 1962;8:909.
2. DeBakey ME. Left ventricular bypass pump for cardiac
assistance. Clinical experience. Am J Cardiol. 1971;27(1):
311.
3. Rose EA, Gelijns AC, Moskowitz AJ, et al.; Randomized
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Congestive Heart Failure (REMATCH) Study Group. Longterm use of a left ventricular assist device for end-stage
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Nose Y, Motomra T, Miyamoto H. History of Mechanical
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2013.
Mancini DM, Beniaminovitz A, Levin H, et al. Low incidence
of myocardial recovery after left ventricular assist device
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Bruckner BA, Stetson SJ, Farmer JA, et al. The implications
for cardiac recovery of left ventricular assist device
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180(6):498501; discussion 501.
Bruckner BA, Stetson SJ, Perez-Verdia A, et al. Regression of
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Burkhoff D, Cohen H, Brunckhorst C, et al. A randomized
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Marks J, Macedo M, Dasse K. Levitronix CentriMag and
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Nishinaka T, Miller PJ, Bearnson GB, et al. EVAHEART left
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13. Mookadam F, Kendall CB, Wong RK, et al. Left ventricular

assist devices: physiologic assessment using echocardiography for management and optimization. Ultrasound
Med Biol. 2012;38(2):33545.
14. Stout M, Ravindran R, Miller C, et al. Preimplant transthoracic echocardiographic assessment of continuous flow
left ventricular assist device. Echocardiography. 2012;
29(1):528.
15. Piacentino V 3rd, Ganapathi AM, Stafford-Smith M, et al.
Utility of concomitant tricuspid valve procedures for
patients undergoing implantation of a continuous-flow
left ventricular device. J Thorac Cardiovasc Surg. 2012;
144(5):121721.
16. Piacentino V 3rd, Williams ML, Depp T, et al. Impact
of tricuspid valve regurgitation in patients treated with
implantable left ventricular assist devices. Ann Thorac
Surg. 2011;91(5):13426; discussion 1346.
17. Kukucka M, Stepanenko A, Potapov E, et al. Impact of
tricuspid valve annulus dilation on mid-term survival after
implantation of a left ventricular assist device. J Heart Lung
Transplant. 2012;31(9):96771.
18. Slaughter MS, Rogers JG, Milano CA, et al. HeartMate
II Investigators. Advanced heart failure treated with
continuous-flow left ventricular assist device. N Engl J
Med. 2009;361(23):224151.
19. Kormos RL, Teuteberg JJ, Pagani FD, et al. HeartMate II
Clinical Investigators. Right ventricular failure in patients
with the HeartMate II continuous-flow left ventricular assist
device: incidence, risk factors, and effect on outcomes.
J Thorac Cardiovasc Surg. 2010;139(5):131624.
20. El Atrache M, Brewer R, Hassan N, et al. Tricuspid Repair
at the Time of LVAD Implantation is Associated with
Improved Survival. J Am Coll Cardiol 2012;59(13):E881.
21. Maltais S, Topilsky Y, Tchantchaleishvili V, et al. Surgical
treatment of tricuspid valve insufficiency promotes
early reverse remodeling in patients with axial-flow left
ventricular assist devices. J Thorac Cardiovasc Surg. 2012;
143(6):13706.
22. Topilsky Y, Oh JK, Shah DK, et al. Echocardiographic
predictors of adverse outcomes after continuous left ventricular assist device implantation. JACC Cardiovasc Imaging.
2011;4(3):21122.
23. Fitzpatrick JR 3rd, Frederick JR, Hsu VM, et al. Risk score
derived from pre-operative data analysis predicts the need
for biventricular mechanical circulatory support. J Heart
Lung Transplant. 2008;27(12):128692.
24. Scalia GM, McCarthy PM, Savage RM, et al. Clinical utility
of echocardiography in the management of implantable
ventricular assist devices. J Am Soc Echocardiogr. 2000;
13(8):75463.
25. Puwanant S, Hamilton KK, Klodell CT, et al. Tricuspid
annular motion as a predictor of severe right ventricular
failure after left ventricular assist device implantation.
J Heart Lung Transplant. 2008;27(10):11027.
26. Kukucka M, Stepanenko A, Potapov E, et al. Right-to-left
ventricular end-diastolic diameter ratio and prediction of
right ventricular failure with continuous-flow left ventricular
assist devices. J Heart Lung Transplant. 2011;30(1):649.

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27. Ochiai Y, McCarthy PM, Smedira NG, et al. Predictors

of severe right ventricular failure after implantable left
ventricular assist device insertion: analysis of 245 patients.
Circulation. 2002;106(12 Suppl 1):I198I202.
28. Kato TS, Farr M, Schulze PC, et al. Usefulness of twodimensional echocardiographic parameters of the left
side of the heart to predict right ventricular failure after
left ventricular assist device implantation. Am J Cardiol.
2012;109(2):24651.
29. Catena E, Tasca G. Role of echocardiography in the perioperative management of mechanical circulatory assistance.
Best Pract Res Clin Anaesthesiol. 2012;26(2):199216.
30. Liao KK, Miller L, Toher C, et al. Timing of transesophageal
echocardiography in diagnosing patent foramen ovale in
patients supported with left ventricular assist device. Ann
Thorac Surg. 2003;75(5):16246.
31. Rasalingam R, Johnson SN, Bilhorn KR, et al. Transthoracic
echocardiographic assessment of continuous-flow left
ventricular assist devices. J Am Soc Echocardiogr. 2011;
24(2):13548.
32. Estep JD, Stainback RF, Little SH, et al. The role of echocardiography and other imaging modalities in patients with
left ventricular assist devices. JACC Cardiovasc Imaging.
2010;3(10):104964.
33. Soleimani B, Haouzi A, Manoskey A, et al. Development of
aortic insufficiency in patients supported with continuous
flow left ventricular assist devices. ASAIO J. 2012;58(4):
3269.
34. Cowger J, Pagani FD, Haft JW, et al. The development of
aortic insufficiency in left ventricular assist device-supported patients. Circ Heart Fail. 2010;3(6):66874.
35. Chapman CB, Allana S, Sweitzer NK, et al. Effects of the
HeartMate II Left Ventricular Assist Device as Observed by
Serial Echocardiography. Echocardiography. 1-11-2013.
36. Toda K, Fujita T, Domae K, et al. Late aortic insufficiency
related to poor prognosis during left ventricular assist
device support. Ann Thorac Surg. 2011;92(3):92934.
37. Pak SW, Uriel N, Takayama H, et al. Prevalence of de novo
aortic insufficiency during long-term support with left
ventricular assist devices. J Heart Lung Transplant. 2010;
29(10):11726.
38. Uriel N, Morrison KA, Garan AR, et al. Development of a
novel echocardiography ramp test for speed optimization
and diagnosis of device thrombosis in continuous-flow left
ventricular assist devices: the Columbia ramp study. J Am
Coll Cardiol. 2012;60(18):176475.
39. Topilsky Y, Maltais S, Oh JK, et al. Focused review on
transthoracic echocardiographic assessment of patients
with continuous axial left ventricular assist devices. Cardiol
Res Pract. 2011;2011:187434.
40. Topilsky Y, Hasin T, Oh JK, et al. Echocardiographic
variables after left ventricular assist device implantation
associated with adverse outcome. Circ Cardiovasc Imaging.
2011;4(6):64861.
41. Goldstein DJ, Maybaum S, MacGillivray TE, et al.; HeartMate
II Clinical Investigators. Young patients with nonischemic
cardiomyopathy have higher likelihood of left ventricular
recovery during left ventricular assist device support.
J Card Fail. 2012;18(5):3925.

1254

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

42. Mano A, Nakatani T, Oda N, et al. Which factors predict

the recovery of natural heart function after insertion of
a left ventricular assist system? J Heart Lung Transplant.
2008;27(8):86974.
43. Osaki S, Sweitzer NK, Rahko PS, et al. To explant or not to
explant: an invasive and noninvasive monitoring protocol
to determine the need of continued ventricular assist
device support. Congest Heart Fail. 2009;15(2):5862.
44. Dandel M, Weng Y, Siniawski H, et al. Pre-explant stability
of unloading-promoted cardiac improvement predicts
outcome after weaning from ventricular assist devices.
Circulation. 2012;126(11 Suppl 1):S919.

45. Catena E, Milazzo F, Merli M, et al. Echocardiographic

evaluation of patients receiving a new left ventricular
assist device: the Impella recover 100. Eur J Echocardiogr.
2004;5(6):4307.
46. Patel KM, Sherwani SS, Baudo AM, et al. Echo rounds: the
use of transesophageal echocardiography for confirmation
of appropriate Impella 5.0 device placement. Anesth Analg.
2012;114(1):825.
47. Abusaid GH, Ahmad M. Transthoracic real time threedimensional echocardiography in Impella placement.
Echocardiography. 2012;29(4):E105E106.

CHAPTER 58
Echocardiographic Assessment of
Left Atrial Function
Utpal N Sagar, Hirohiko Motoki, Allan L Klein

Snapshot

Anatomy

Physiology

INTRODUCTION
As the role of echocardiography has evolved to assess
hemodynamic status and diastolic function in addition to
characterizing two-dimensional structure and function,
there has been an emphasis on improving echocardi
ographic assessment of the left atrium (LA). This has
become of increasing importance as left atrial volume
and function have been described as strong predictors
of major adverse cardiovascular events. In this chapter,
we will review the structure and multifaceted function of
the LA, the physiology of the LA, modalities of functional
assessment, and review left atrial function in the context of
various cardiovascular disease states.

ANATOMY
The LA is the most posteriorly situated chamber of the
heart and is oriented superiorly and to the left of the right
atrium. The pulmonary veins normally drain into the
posterior aspect of the LA. These vessels are covered by the
visceral or inner layer of pericardium. The serous layer is
fused with the outer fibrous pericardium.1
The LA may be divided into different regions,
including the left atrial appendage which is small, tubular,

Functional Assessment

Left Atrial Pathophysiology

and is varied in the number of lobes which comprise its

structure. The wall of the appendage also has variable
thickness, with alternating muscle bundles. The vestibule
of the LA includes the region around the mitral valve
orifice and is noted for its generally smooth endocardial
surface. Encompassing the posteroinferior wall of the
LA is the mitral isthmus, which extends between the
entrance of the left inferior pulmonary vein and the mitral
valve. Proximally, the vestibule merges with the septal
component, which joins with the posterior wall and the
roof of the LA. The fossa ovalis, which is a remnant of the
embryonic septum primum, may be seen from the left
atrial aspect of the septum as a crescent-shaped edge.
The posterior portion of the LA into which the pulmonary
veins drain is referred to as the venous component. There
are small ridge-like structures between the entrance of the
superior and inferior pulmonary veins, and a larger ridge
between the left atrial appendage and the entrance to the
left atrial appendage.1
The walls of the LA that border the regions of the LA
described previously are not of uniform thicknesses. Most
notably, the wall surrounding the venous portion of the
LA is composed of varying amounts of musculature with
differing orientations.1

1256

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 58.2: Graphical representation of the pressurevolume

relationship of left atrial (LA) function. Note the dynamic changes
in the pressure and volume that occur during the cardiac cycle.
(MVC: Mitral valve closure; MVO: Mitral valve opening; Pre-A:
Pre-atrial contraction).

Fig. 58.1: Left atrial (LA) phasic functions and their relationship
with the cardiac cycle. (TMF: Transmitral flow; PVF: Pulmonary
vein flow, ECG: Electrocardiogram). Note the changes in left atrial
volumes through the various phases of left atrial function.

PHYSIOLOGY
Although the role of the LA could be simply described as a
chamber that receives oxygenated blood, its function is far
more complex. Below, we will discuss the phasic function
of the LA and the factors that affect its function.

Phasic Left Atrial Function

Broadly, there are three phases of left atrial function,
including the reservoir, conduit, and contraction phases
(Fig. 58.1). The LA first acts as a reservoir during left
ventricular systole as the mitral valve annulus is displaced
apically, augmenting LA volume with a concomitant

decrease in pressure. The LA then receives blood that

returns via the pulmonary veins. The difference between
the passive LA emptying volume and the pulmonary blood
flow is the reservoir volume. The next phase of left atrial
function is the conduit phase in which the LA conducts
blood into the left ventricle with the opening of the mitral
valve and occurs until left atrial contraction. Essentially,
the mitral valve annulus descends toward the cardiac base,
decreasing LA volume. This volume may be determined
by the difference in LV stroke volume and the sum of the
active and passive LA volumes. In the final phase, the left
atrial pectinate muscles contract in late diastole with an
increase in LA pressure, and development of a pressure
gradient between the LA and LV, resulting in blood flow
across the mitral valve. This so-called atrial kick has a
significant contribution to the stroke volume of the left
ventricle and may be defined as the difference in the left
atrial volume at the onset of the P-wave and minimal
LA volume.2 Figure 58.2 graphically demonstrates this
relationship of left atrial pressure and volume throughout
the cardiac cycle, and its relation to left ventricular filling.
The LA also functions as a volume and pressure sensor
of diastolic function. And, through the release of natriuretic
peptides and interactions with the sympathetic nervous
system, as well as the reninangiotensinaldosterone
system, it communicates with various neurohormonal
systems.3

Chapter 58: Echocardiographic Assessment of Left Atrial Function

Physiological Effects on
Left Atrial Function
It follows that the previously described left atrial function
do not occur in isolation and are related to left ventricular
compliance. Abnormalities in left ventricular, valvular, or
atrial disease are often reflected as an increase in left atrial
filling pressures, which may be observed as enlargement
of left atrial size.4 Hence, LA afterload increases as
left ventricular (LV) filling pressures increase and as
LV diastolic dysfunction worsens. This increase in LA
afterload and LA volume results initially in an increase in
LA size and an improvement in LA function. However, LA
contractility declines once a threshold has been reached,
similar to the FrankStarling curve of the LV.2 Various
examples of the relationship between LA and LV size, and
functional assessment will be discussed in the left atrial
pathophysiology section of this chapter.

FUNCTIONAL ASSESSMENT
We have already described the significant interplay
between LA and LV function, such that events during
each phase of LA phasic function are affected by factors
from both the LA and LV. However, despite considerable
investigation, the magnitude and relative importance
of the atrial contribution to LV filling and cardiac output
remain controversial, and provide a motivation for a more
complete evaluation of the atrial cycle.
Recent advances in catheter ablation for the treat
ment of atrial fibrillation (AF), in dual- and threechamber pacemakers that maintain atrioventricular and
biventricular synchrony, and in the pathological and
clinical relevance of chamber-specific structural, elec
trical, and ionic remodeling have increased the interest
in accurately imaging the LA structure and function.
With respect to the assessment of the LA function, twodimensional echocardiography (2DE), three-dimensional
echocardiography (3DE), Doppler echocardiography,
and speckle tracking echocardiography have distinctly
different strength and weaknesses, and are complementary
in specific clinical scenarios. In this section, we discuss
the role of each modality to assess LA function with
an emphasis on the relative merits of newer imaging
techniques and how these may be applied in the various
clinical settings.

Volume
Left atrial size has been compared to the hemoglobin A1c
in diabetes as a measure of the average effect of LV filling

1257

pressures over time,5,6 making it a useful marker of both

the chronicity and severity of LV diastolic dysfunction.7,8
Left ventricle size measurement is routinely perfor
med by transthoracic echocardiography (Figs 58.3A to E).
LA antero
posterior dimension can be measured by
M-mode or B-mode in the parasternal long-axis view. This
method is convenient and has been widely adopted in
routine clinical practice. However, LA volume measured
by either the ellipsoid model or the Simpsons method
is a more reliable measure of true LA size than M-mode
LA dimension9 and is the recommended method for the
accurate assessment of LA size.10 Measuring the maxi
mum LA volume at the time of mitral valve opening
is now routinely performed with echocardiography,
although it only represents a snapshot of LA function at
a specific point of the cardiac cycle. Maximal LA volume
index is a predictor of adverse cardiovascular outcomes
such as AF, stroke, and congestive heart failure and/or
death in various conditions, including community-based
populations,1114 postmyocardial infarction,15,16 heart
failure,1719 hypertrophic cardiomyopathy,2022 AF,23 and
postcardiac surgery patients.24 However, measurement
of LA phasic volumes using 2DE is time consuming, and
errors can arise from geometric assumptions of biplane
volume calculations, as well as from difficulties with
echocardiographic window and the timing of various atrial
events.
To improve the accuracy of LA size measurement,
3DE has been studied (Fig. 58.4). The 3DE measurements
demonstrate favorable testretest variability25 and good
agreement with cardiac magnetic resonance imaging.2527
Among the newer techniques including 3DE, cardiac
computed tomography, and cardiac magnetic resonance
imaging, 3DE shows the most promise of adoption in
routine clinical practice as it is noninvasive, readily
available, and can be added onto the routinely performed
2DE examination. 3DE also offers the possibility of meas
uring LA volumes at different phases of the cardiac cycle,
yielding information on LA phasic function.

Spectral Doppler
Pulmonary vein flow, transmitral flow, and mitral annular
velocity are routinely measured. These parameters are
determined by LA function, as well as LV systolic and
diastolic performance. Peak velocity of mitral A-wave
indicates LA mechanical function, although it is also
affected by heart rate and loading conditions.28 Peak
mitral A velocity has been shown to be associated with

1258

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Figs 58.3A to E: Various methods of echocardiographic determination of left atrial size. Figure A demonstrates M-mode through the
left atrium (LA), Figures B and C demonstrate volume measurements of the LA using the biplane method of discs (modified Simpsons
rule) from apical four-chamber and two-chamber views, respectively. Figures D and E demonstrate the measurement of LA volume from
arealength method using the images from apical four-chamber and two-chamber views.

Fig. 58.4: Multiplanar imaging of the left atrium, with three-dimensional rendering and determination of the left atrial volume.

AF recurrence postcardioversion.29 Atrial ejection force,

calculated from the mitral annulus area and mitral
A-wave velocity, has been suggested to be a useful

atrial mechanical function30 and has been shown to be

prognostic for cardiovascular events in a population with a
high prevalence of hypertension and diabetes,31 although
the method is limited by the robustness and variability of
the measurements.
Mitral annular velocity during atrial contraction (a') is
another indicator of atrial contractile function, measured
by tissue Doppler imaging (TDI) of the mitral annulus to
quantify the low-velocity, high-amplitude myocardial
velocities. Its amplitude is related to both atrial contractility
and LV end-diastolic pressure.32 Increased a' is also seen
in those with LV hypertrophy, indicating increased LA
active ejection force.33 Decreased a' has been shown to
be a predictor for elevated pulmonary pressures in mitral
regurgitation, clinical deterioration in aortic stenosis, AF
postcoronary artery bypass grafting, and progression from
paroxysmal to persistent nonvalvular AF. In heart failure,

Chapter 58: Echocardiographic Assessment of Left Atrial Function

1259

of AF, left ventricular systolic dysfunction, and clinical

heart failure, the role of LA functional assessment and its
pathophysiological applications has continued to evolve.
In this section, we will discuss the role of left atrial
functional assessment in various clinical settings.

Hypertension

Fig. 58.5: An example of speckle tracking, used here to determine

left atrial strain and strain rate.

low a' is associated with poor exercise tolerance and is a

better predictor of cardiac events than E/e' and decele
ration time of transmitral E-wave.

Speckle Tracking
Measures of myocardial deformation have been
increasingly adapted to study LA mechanics. Both echo
cardiographic methods of measuring strain and SR, 2D
speckle tracking imaging, and color TDI have been adapted
to measuring LA deformation. Speckle tracking calculates
strain by tracking tissue deformation via characteristic
myocardial speckles frame-by-frame, with SR given by the
rate of such deformation (Fig. 58.5). Color TDI generates
a spatial map of myocardial velocities, from which SR of
the region of interest is derived, with strain calculated by
integrating the SR data.
The advantage of analyzing LA mechanics with strain
and SR imaging is the information that can be obtained
about each component of LA phasic function. One could
use this method to resolve the exact change in LA phasic
function with different disease states and investigate the
effect after treatment.
Thus, deformation-based parameters of LA reservoir
function could provide the prognostic information in the
population at risk for adverse cardiovascular events.

LEFT ATRIAL PATHOPHYSIOLOGY

While left atrial size has been shown to have a significant
prognostic role in the prediction of the development

The left ventricular hypertrophy and LA dilation that

is observed in patients with moderate and severe
hypertension often is not observed in patients with
mild hypertension. Earlier in this chapter, we noted
that strain and strain rate (SR) imaging may be useful in
characterizing the components of LA phasic function. In
the setting of mild hypertension, SR imaging may show a
reduction in the early diastolic LA SR, which is associated
with a decrease in LA conduit volume. These changes may
reflect early LV diastolic dysfunction, which may herald
the development of LV hypertrophy and LA dilation, and
possibly AF and clinical symptoms.34

Atrial Fibrillation
Impairments in LA structure and function may commonly
lead to AF. Various echocardiographic modalities have
been studied to evaluate the degree of LA fibrosis that
has been associated with AF. Atrial conduction time
as measured by TDI has been shown to predict the
development of AF in the general population when
prolonged greater than 190 ms.35 Recently, percutaneous
catheter ablation has been used more frequently to
successfully manage symptomatic and medically refractory
AF. In these patients, the best predictors for maintenance
of sinus rhythm following ablation were parameters of LA
reservoir function, determined by SR imaging.36,37 Studies
have shown that LA reservoir function has been related to
LA structural remodeling and fibrosis of the atrial wall.38,39
Following conversion to sinus rhythm, LA volume and
function may be monitored, with those patients who have
greater degrees of dysfunction being selected for further
therapy, such as antiarrhythmics.40
Direct current cardioversion is a widely used treatment
modality for both emergent and elective manage
ment of AF. However, there is significant risk of stroke
and thromboembolism associated with direct current
cardioversion.4144 Classically, this was thought to be
due to pre-existing left atrial thrombus or continuous
or prominent spontaneous echo contrast. Earlier, we
described the location and anatomy of the left atrial
appendage. Transesophageal echocardiography (TEE) is

1260

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

the best method of detection of thrombi in the left atrial

appendage and LA that could be embolized in the setting
of cardioversion (Figs 58.6A to C). However, significant
work in this area has described myocardial stunning that
follows cardioversion, giving rise to conditions in the LA
that may lead to thrombus, even if no LA thrombus was
present prior to cardioversion.45 Nevertheless, TEE prior
to cardioversion has become standard of care, and has
been studied extensively.45,46 Techniques have evolved
now to involve the functional assessment of the left atrial
appendage, evaluating emptying velocities with pulsed
wave Doppler at the entrance of the appendage. Peak
LA appendage emptying velocity has been shown to be
a predictor of maintenance of sinus rhythm following

cardioversion.47,48 TEE-guided cardioversion may be impo

rtant with the use of the new oral anticoagulants such as
dabigatran, rivaroxaban, and apixaban since there is no
anticoagulation monitoring in patients with AF.

Cardiomyopathies
Assessment of LA function may help to diagnose, to
differentiate, and to guide therapy of various cardio
myopathies. For example, patients with hypertrophic
cardiomyopathy have been shown to have decreased
LA longitudinal function in addition to decreased LA
reservoir function. This may be used to differentiate
hypertrophic cardiomyopathy from other forms of left
ventricular hypertrophy.49,50 Figures 58.7A to E show an

Figs 58.6A to C: The spectrum of left atrial appendage pathology. Figure A shows the presence of left atrial spontaneous echocontrast,
or smoke; Figure B demonstrates prominent, persistent spontaneous echocontrast in the left atrium and left atrial appendage (LAA),
consistent with sludge; Figure C shows the presence of a minimally mobile echodensity within the left atrial appendage, consistent
with thrombus (arrow).

Figs 58.7A to E: Patient with hypertrophic obstructive cardiomyopathy (HOCM), as seen in Figure A. Figures B and C demonstrate
severe left atrial enlargement. Mitral inflow shows decreased A-wave (Figure D) and tissue Doppler image (Figure E) shows moderate
to severe decreased atrial contraction with decreased a' velocity. (LA: Left atrium; LV: Left ventricle).

Chapter 58: Echocardiographic Assessment of Left Atrial Function

1261

Figs 58.8A to D: Echocardiogram of a patient with cardiac amyloidosis. The apical four-chamber view in Figure A demonstrates the
degree of left atrial enlargement; Figure B shows the degree of increased left ventricular wall thickness and the characteristic echotexture of a patient with cardiac amyloidosis. The transmitral flow demonstrating restrictive diastolic pattern with small A-wave is seen in
Figure C. Tissue Doppler in Figure D shows severely depressed a' velocity, consistent with restrictive cardiomyopathy. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

echo-Doppler evaluation of LA function in a patient

with HOCM. Also, in patients with cardiac amyloidosis,
we see that the LA is severely dilated and a' is reduced,
conferring a high risk for thromboembolism. Figures 58.8A
to D show an echo-Doppler evaluation of LA function in
cardiac amyloidosis. In a study of a population of patients
with ischemic and idiopathic cardiomyopathy, LA
reservoir function predicted a positive response to cardiac
resynchronization therapy, although the study did not
report strain and SR for the other phases of LA function.51

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Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

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14. Takemoto Y, Barnes ME, Seward JB, et al. Usefulness of
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prognostic value of left atrial volume in patients with
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fibrillation and congestive heart failure in patients >/=65
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19. Sabharwal N, Cemin R, Rajan K, et al. Usefulness of left
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20. Tani T, Tanabe K, Ono M, et al. Left atrial volume and
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21. Losi MA, Betocchi S, Aversa M, et al. Determinants of atrial
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cardiomyopathy. Am J Cardiol. 2004;94:895900.

22. Losi MA, Betocchi S, Barbati G, et al. Prognostic significance

of left atrial volume dilatation in patients with hypertrophic
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23. Osranek M, Bursi F, Bailey KR, et al. Left atrial volume
predicts cardiovascular events in patients originally diag
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24. Osranek M, Fatema K, Qaddoura F, et al. Left atrial volume
predicts the risk of atrial fibrillation after cardiac surgery:
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25. Jenkins C, Bricknell K, Marwick TH. Use of real-time
three-dimensional echocardiography to measure left atrial
volume: comparison with other echocardiographic techni
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26. Keller AM, Gopal AS, King DL. Left and right atrial volume
by freehand three-dimensional echocardiography: in
vivo validation using magnetic resonance imaging. Eur
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27. Artang R, Migrino RQ, Harmann L, et al. Left atrial volume
measurement with automated border detection by
3-dimensional echocardiography: comparison with
Magnetic Resonance Imaging. Cardiovasc Ultrasound.
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28. Choong CY, Herrmann HC, Weyman AE, et al. Preload
dependence of Doppler-derived indexes of left ventricular
diastolic function in humans. J Am Coll Cardiol. 1987;10:
8008.
29. Spiecker M, Bohm S, Borgel J, et al. Doppler echocar
diographic prediction of recurrent atrial fibrillation
following cardioversion. Int J Cardiol. 2006;113:1616.
30. Manning WJ, Silverman DI, Katz SE, et al. Atrial ejection
force: a noninvasive assessment of atrial systolic function.
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31. Chinali M, de Simone G, Roman MJ, et al. Left atrial systolic
force and cardiovascular outcome. The Strong Heart Study.
Am J Hypertens. 2005;18:15706; discussion 7.
32. Nagueh SF, Sun H, Kopelen HA, et al. Hemodynamic
determinants of the mitral annulus diastolic velocities by
tissue Doppler. J Am Coll Cardiol. 2001;37:27885.
33. Khankirawatana B, Khankirawatana S, Peterson B, et al.
Peak atrial systolic mitral annular velocity by Doppler
tissue reliably predicts left atrial systolic function. J Am
Soc Echocardiogr. 2004;17:35360.
34. Eshoo S, Boyd AC, Ross DL, et al. Strain rate evaluation
of phasic atrial function in hypertension. Heart. 2009;95:
118491.
35. De Vos CB, Weijs B, Crijns HJ, et al. Atrial tissue Doppler
imaging for prediction of new-onset atrial fibrillation.
Heart. 2009;95:83540.
36. Schneider C, Malisius R, Krause K, et al. Strain rate imaging
for functional quantification of the left atrium: atrial
deformation predicts the maintenance of sinus rhythm
after catheter ablation of atrial fibrillation. Eur Heart J.
2008;29:1397409.
37. Di Salvo G, Caso P, Lo Piccolo R, et al. Atrial myocardial
deformation properties predict maintenance of sinus
rhythm after external cardioversion of recent-onset lone
atrial fibrillation: a color Doppler myocardial imaging

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39.

40.

41.
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44.

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Cardiovasc Imaging. 2010;3:2319.
Cameli M, Lisi M, Righini FM, et al. Usefulness of Atrial
Deformation Analysis to Predict Left Atrial Fibrosis and
Endocardial Thickness in Patients Undergoing Mitral Valve
Operations for Severe Mitral Regurgitation Secondary to
Mitral Valve Prolapse. Am J Cardiol. 2012.
Rosca M, Lancellotti P, Popescu BA, et al. Left atrial fun
ction: pathophysiology, echocardiographic assessment,
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Lown B. Electrical reversion of cardiac arrhythmias. Br
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Bjerkelund CJ, Orning OM. The efficacy of anticoagulant
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Stein B, Halperin JL, Fuster V. Should patients with atrial
fibrillation be anticoagulated prior to and chronically
following cardioversion? Cardiovasc Clin. 1990;21:23147;
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Grimm RA, Stewart WJ, Black IW, et al. Should all patients
undergo transesophageal echocardiography before elect
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45. Grimm RA. Transesophageal echocardiography-guided

cardioversion of atrial fibrillation. Echocardiography. 2000;
17:38392.
46. Klein AL, Grimm RA, Murray RD, et al. Use of transes
ophageal echocardiography to guide cardioversion in
patients with atrial fibrillation. New Engl J Med. 2001;
344:141120.
47. Antonielli E, Pizzuti A, Palinkas A, et al. Clinical value of
left atrial appendage flow for prediction of long-term sinus
rhythm maintenance in patients with nonvalvular atrial
fibrillation. J Amer College Cardiol. 2002;39:14439.
48. Omran H, Jung W, Schimpf R, et al. Echocardiographic
parameters for predicting maintenance of sinus rhythm
after internal atrial defibrillation. J Amer College Cardiol.
1998;81:14469.
49. Paraskevaidis IA, Panou F, Papadopoulos C, et al. Evaluation
of left atrial longitudinal function in patients with
hypertrophic cardiomyopathy: a tissue Doppler imaging
and two-dimensional strain study. Heart. 2009;95:4839.

50. Rosca M, Popescu BA, Beladan CC, et al. Left atrial
dysfunction as a correlate of heart failure symptoms in
hypertrophic cardiomyopathy. J Am Soc Echocardiogr.
2010;23:10908.
51. DAndrea A, Caso P, Romano S, et al. Different effects of
cardiac resynchronization therapy on left atrial function
in patients with either idiopathic or ischaemic dilated
cardiomyopathy: a two-dimensional speckle strain study.
Eur Heart J. 2007;28:273848.

CHAPTER 59
The Use of Echocardiography to
Assess Cardiac Hemodynamics and
Guide Therapy
Roy Beigel, Robert J Siegel

Snapshot

Right Atrial Pressure/Central Venous Pressure

Addional Parameters for Esmaon of

Pulmonary Artery Hemodynamics

Le Atrial Pressure

Stroke Volume, Stroke Distance, Cardiac Output and

Systemic Pulmonary Shunts (QP/QS)

Le-Sided Filling Pressures

INTRODUCTION
Accurate noninvasive hemodynamic assessment has the
potential to greatly improve patient management with
regard to volume status, pharmacological treatment,
and clinical outcomes. Several studies have shown that
the time-honored physical examination has very
limited sensitivity and specificity for right atrial pressure
(RAP), pulmonary artery pressure (PAP), as well as
left atrial (LA) filling pressures. Since the 1970s, the
standard for hemodynamic assessment has been invasive
measurements made by pulmonary artery (PA) catheterization. However, use of the PA catheter has been subject
to criticism13 as it can increase patient morbidity.4,5
Doppler-echocardiographic measurements of right- and
left-sided filling pressures, pulmonary vascular resistance
(PVR), and cardiac output (CO) are possible to obtain in
most patients. Echocardiography can potentially provide
adequate alternative hemodynamic data, which are more
accurate than the physical examination without the risks
of invasive monitoring.

RIGHT ATRIAL PRESSURE/CENTRAL

VENOUS PRESSURE
Central venous pressure (CVP; Table 59.1) and RAP are
the same, provided that there is no obstruction of the
vena cava. Traditionally, RAP is measured with a central
venous catheter (normal range is between 1 and 7 mm Hg
for mean RAP). Elevated values have adverse prognostic
implications for morbidity and mortality,69 making
the accurate assessment of RAP an important factor in
patients assessment, management and outcome.10,11
Accurate evaluation of RAP is also a necessary for the
noninvasive estimation of the systolic and diastolic PAP
(DPAP). Table 59.1 lists various methods used for the
echocardiographic evaluation of RAP.

Inferior Vena Cava Parameters (Fig. 59.1)

The most commonly utilized method uses the inferior
vena cava (IVC) size and its respiratory variation for

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

1265

Table 59.1: The Various Methods Utilized for the Echocardiographic Evaluation of Right Atrial Pressure (RAP)

Method

Criteria Used

Strength and Limitations

IVC parameters2D or M-mode

subcostal imaging of the IVC
(Fig. 59.1)

IVC diameter, collapse:

< 2.1 cm, > 50%normal
RAP ~ 3
> 2.1 cm, > 50%intermediate* RAP ~ 8
> 2.1 cm, < 50%High
RAP ~> 15
*In cases which the IVC diameter
and collapse do not fit the normal
or high criteria.

Most validated method

Above a certain elevation of RAP, the IVC may be fully
dilated and not collapsing, making estimation above
this point difficult
Increased IVC diameter and/or decreased collapse in
the presence of RAP can be seen with:
Low respiratory compliance
Mechanically ventilated patients
Trained athletes
Prominent Eustachian valve
Narrowing of the IVCRA junction
Web or tissue present in the IVC

Systemic and hepatic venous flow

Doppler flow in the vena cava,
jugular, or hepatic veins
(Figs 59.2A and B)

Vs > Vdnormal RAP

Vs < Vdelevated RAP
(> 8 mm Hg)

Obtaining flow velocity curves from the SVC is simple,

less obtainable with hepatic veins
Severe tricuspid regurgitation can alter the venous
flow pattern without correlation to RAP
Atrial compliance and relaxation and tricuspid
annular descent can affect flow patterns and make
them less reliable
Atrial fibrillation or past cardiac surgery can cause the
hepatic vein systolic flow to be diminished regardless
of RAP

Hepatic vein filling fraction

(HVFF)pulsed Doppler of
hepatic veins

VsVTI/(VsVTI + VdVTI)
< 55High RAP > 8 mm Hg

Validated in mechanically ventilated patients

Single study24
Atrial fibrillation or past cardiac surgery can cause
the hepatic vein systolic flow to be diminished
regardless of RAP

Doppler and TDIpulsed Doppler

E/e > 6RAP > 10 mm Hg
of the tricuspid inflow and TDI of the
tricuspid valve (Figs 59. 3A and B)

Validated in mechanically ventilated patients

May not be an accurate method in patients who
have undergone cardiac surgery

Right ventricular regional

isovolumic relaxation time
(RV rIVRT)tricuspid TDI

> 59 ms correlates to
RAP > 8 mm Hg

Studied on a limited number of patients (n = 21)

in a single study25

3D RA dimensions3D
transthoracic imaging of the RA

3DE maximal RA volume

35 mL/m2 combined with IVC
diameter 2 cmcorrelates with
RAP > 10 mm Hg

Single study in a selective group of heart failure

patients with EF < 35%27

(3DE: Three-dimensional echocardiography; IVC: Inferior vena cava; RA: Right atrial; SVC: Superior vena cava; TDI: Tissue Doppler
imaging; VTI: Velocity time integral).

the echocardiographic evaluation of RAP. As the IVC is

a highly compliant vessel, its size and flow dynamics
vary with changes in CVP and volume. As shown in
Figure 59.1, during inspiration (which produces negative
intrathoracic pressure), vena cava pressure decreases and
flow increases.12,13 At low or normal RAP, there is systolic
predominance in IVC flow, such that the systolic flow
is greater than the diastolic flow. As RAP increases, it is
transmitted to the IVC, resulting in blunting of the forward

systolic flow, reduced IVC collapse with inspiration and

eventually IVC dilatation (Fig. 59.1). Current guidelines14,15
recommend using the IVC maximal diameter (IVC max)
12 cm from the RAIVC junction at end-expiration
and the IVC collapsibility index (IVCCI, which equals
[IVCmax IVCmin]/IVCmax). For RAP assessment, as noted in
Table 59.2, an IVC with a diameter < 2.1 cm and collapse
> 50% correlates with a normal RAP of 0 to 5 mm Hg. An
IVC < 2.1 cm with < 50% collapse and an IVC > 2.1 cm with

1266

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 59.1: Imaging of the inferior vena cava (IVC, marked with
asterisk) using 2D echocardiography (top, and middle images),
and M-mode echocardiography (bottom images) from the
subcostal view. The left three images show respiratory variations
of the IVC in a patient with normal right atrial pressure (RAP). The
right three images show no respiratory variation of the IVC, which
is also dilated. This patient was found to have an elevated RAP.

> 50% collapse correspond to an intermediate RAP of 5 to

10 mm Hg. An IVC > 2.1 cm with <50% collapse suggests
a high RAP of 15 mm Hg. Using midrange values of
3 mm Hg for normal and 8 mm Hg for intermediate RAP
is recommended. However, if there is minimal collapse of
the IVC (<35%) and/or secondary indices of elevated RAP
are present, upgrading to the higher pressure limit (i.e. 5
and 10 mm Hg in case of normal and intermediate RAP,
respectively) should be done. Patients should be supine
during assessment of the IVC as other positions may lead
to either under- or overestimation of IVC diameter and/
or collapsibility.16 Patients with low compliance with deep
inspiration may have a diminished IVC collapse. A sniff
maneuver that causes a sudden decrease in intrathoracic
pressure and by that accentuating the normal inspiratory
response can be used to differentiate those with normal
IVC collapsibility from those with a diminished IVC
collapsibility.
IVC size and collapsibility are helpful to identify RAP
as being high or low, but this method does not provide
precise numeric values for RAP. It should be noted that
the IVC can be dilated in individuals with a normal RAP.
Common causes of a dilated IVC in the setting of normal
RAP17,18 are listed in Table 59.1.
In order to overcome some of the limitations of RAP
estimation through IVC indices, additional Dopplerechocardiographic parameters have been evaluated and
proposed to better quantify RAP (Table 59.1).

Table 59.2: The Various Methods Utilized for the Echocardiographic Evaluation of Pulmonary Artery Pressure (PAP)

Method
Criteria Used
Systolic Pulmonary Artery Pressure (SPAP):
Using the TR jetSimplified
P = 4 V2 + RAP = SPAP
Bernoulli equation (Fig. 59.5)

Pulmonary flow acceleration

time (Fig. 59.6)

Lesser range of 100 ms indicates

elevated SPAP

Strength and Limitations

Widely validated
Simple equation
Underestimation can occur if:
RAP underestimated
Misalignment of Doppler signal
Poor TR signal
Severe TR
Overestimation can occur if:
RAP overestimated
Mistakenly using the tricuspid valve closing
spike
Not widely studied
Validated only in patients with chronic heart
failure
Measurements can be affected by extremes of
heart rate (below 60 or above 100 beats per minute)
Contd....

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

1267

Contd....
Method
Criteria Used
Diastolic Pulmonary Artery Pressure (DPAP):
Using the pulmonary
P = 4 V2 + RAP = DPAP
regurgitation (PR)
jetSimplified Bernoulli
equation (Fig. 59.9)

TR velocity at time of
pulmonic valve
openingSimplified
Bernoulli equation

P = 4 V2 + RAP = DPAP

Mean Pulmonary Artery Pressure (MPAP):

Using the peak pulmonary
P = 4 V2 + RAP = MPAP
regurgitation (PR)
jetSimplified Bernoulli
equation (Fig. 59.9)

Tracing the TR jet (Fig. 59.10)

Empirical formulas

PVR:
Formula using surrogates for
the transpulmonary pressure
gradient and transpulmonary
flow

Formulas using the pre-ejection period (PEP), AcT, and

total systolic time (TT)
Estimation of the PVR
index (PVRI)

MPAP = 0.61 SPAP + 2 mm Hg

MPAP = DPAP + 1/3 (SPAP-DPAP)

PVR (WU) = 10 TR
velocity/RVOT VTI + 0.16

PVR = 0.156 + 1.154 [(PEP/AcT)/TT]

PVRI = 1.97 + 190

[SPAP/(HR RVOT VTI)]
SPAP/(HR RVOT VTI) >0.076 correlated
with severe pulmonary vascular disease
with PVRI > 15 WU/m2

Strength and Limitations

Simple equation
Not widely validated
PR jet not always acquirable
Underestimation can occur if:
RAP underestimated
Misalignment of Doppler signal
Poor PR signal
Overestimation can occur if:
RAP overestimated
TR jet more detectable then PR
Not widely validated
Underestimation can occur if:
RAP underestimated
Misalignment of Doppler signal
Poor PR signal
Severe PR
Overestimation can occur if:
RAP overestimated
Simple equation
Not widely validated
PR jet not always acquirable
Underestimation can occur if:
RAP underestimated
Misalignment of Doppler signal
Poor PR signal
Overestimation can occur if:
RAP overestimated
Validated in a single study49
Easy to obtain
Validated only invasively51
Validated echocardiographically in a single study43
Not widely validated
Same limitations as SPAP and DPAP
Not widely validated
Underestimation can occur if:
Misalignment of Doppler signal
Poor TR signal
Severe TR
RVOT not well visualized in patients
with poor acoustic window
Single study of heart failure patients with EF
< 35%, in sinus rhythm59
Single study of patients with PH60

(PH: Pulmonary hypertension; PVR: Pulmonary vascular resistance; RAP: Right atrial pressure; RVOT: Right ventricular outflow tract;
TR: Tricuspid regurgitation; VTI: Velocity time integral).

1268

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Using pulsed wave Doppler, the hepatic vein systolic filling

fraction (HVFF), which is the ratio of the velocity time

integrals (VTIs): VsVTI/(VsVTI + VdVTI), can be obtained.

A value < 55% was found to be the most sensitive (86%)
and specific (90%) sign of an RAP > 8 mm Hg. With higher
RAP, there was a decrease in systolic filling fraction.24 In
this single study, the best model for prediction of mean
RAP was = 21.6 24 HVFF. Although IVC collapsibility
cannot be evaluated in mechanically ventilated patients,
hepatic vein flow velocities have been validated in this
situation,24 provided that the velocities are averaged over
5 consecutive beats and comprising 1 respiratory cycle.15
The maximal early filling velocity through the tricuspid
valve during diastole (E-wave) increases as the RAP rises.
The use of tissue Doppler imaging (TDI) allows recording
of myocardial and annular velocities, and can measure the
velocity of tissue relaxation of the lateral tricuspid annulus
in diastole (e'-wave) (Figs 59.3A and B). It has been shown
that there is a relationship between RAP and the E/e' ratio:
A high E velocity combined with a low e' giving an E/e' ratio
of > 6 was found to be predictive of a RAP > 10 mm Hg. This
correlation was found to also be accurate in patients on
mechanical ventilation. However, this method may not be
an accurate in patients who have undergone prior cardiac
surgery.
The RV regional isovolumic relaxation time (RV rIVRT)
is the time period between the end of systolic annular
motion and the onset of the e'-wave upon TDI of the lateral
tricuspid annulus as evaluated in the apical four-chamber
view. Using this index, it was found that an RV rIVRT of
< 59 ms corresponds to a RAP > 8 mm Hg.25

Systemic Venous Flow (Figs 59.2A and B)

The central venous Doppler flow pattern seen in the vena
cava, jugular, and hepatic veins is characterized as seen by
three distinct waveforms.19 The first is the systolic wave (Vs)
caused by RA relaxation and descent of the tricuspid ring
associated with right ventricular (RV) systole. The second
is the diastolic wave (Vd), which occurs during rapid
ventricular filling when the tricuspid valve is open. The third
is a positive A-wave, which occurs with RA contraction and
represents reverse flow. The A-wave is small and might not
be present in normal individuals. In the majority of normal
adults, inspiration increases the magnitude of Vs and Vd,
whereas the A-wave, if present, decreases in amplitude. At
low or normal RA pressures, there is systolic predominant
venous flow, such that the velocity of Vs is greater than the
velocity of Vd (Figs 59.2A and B). With elevation of the RA
pressure, the systolic flow predominance is lost, such that
Vs is substantially decreased and Vs/Vd is < 1. The higher
the RAP the lower the pressure gradient between these
veins and the RA causing diminished forward systolic flow.
This blunted gradient is present in patients with restrictive
heart disease and elevated right-sided filling pressures.2023

Doppler and Tissue Doppler

Imaging (Figs 59.3A and B)

Figs 59.2A and B: Evaluation of the central venous flow pattern using pulsed wave Doppler imaging. (A) Systolic (S) and diastolic (D)
flow in the superior vena cava of an adult with a normal biphasic pattern. The S/D > 1 is supportive of normal right atrial pressure (RAP);
(B) S and D flow velocity in the hepatic vein in a patient with elevated RAP. There is diminished S, increased D, and an S/D < 1.

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

1269

Figs 59.3A and B: Doppler and tissue Doppler imaging (TDI) of the tricuspid valve. (A) Tricuspid inflow velocity Doppler recording
(E = 36.1 cm/s); (B) Tricuspid annular velocity, e , is 5.2 cm/s. In this patient, the E/e ratio is >6, which supports that the RAP is
>10 mm Hg.

Doppler and TDI provide an alternative for RAP

evaluation when subcostal views cannot be obtained and
when there is inability to assess the IVC and hepatic indices.
They can also be used to corroborate the prediction of RAP
using HVFF in patients on mechanical ventilation where
IVCCI is inaccurate.26

RA Dimensions (Fig. 59.4)

Chronically elevated RAP generally leads to RA enlargement. RA size and volume can be assessed from many twodimensional echocardiographic (2DE) views but it is most
commonly measured in the apical four-chamber view15
(Fig. 59.4). Three-dimensional echocardiography (3DE)
provides tomographic imaging of cardiac chambers and
has the potential to be a more accurate modality for atrial
volume quantification than 2DE. In one study,27 3DE RA
volume correlated with RAP (r = 0.51, p < 0.001) in heart
failure (HF) patients. Conversely, 2DE measurements of the
RA (both size and 2DE RA volume) have not been shown
to correlate with RAP.24 Compared to the American Society
of Echocardiography (ASE) recommendations of using an
IVC diameter of 2 cm and decreased respiratory collapse
of <40%, 3DE measured maximal RA volume of 35 mL/m2
combined with an IVC diameter 2 cm improves the
sensitivity in identifying RAP > 10 mm Hg.27
Currently, there is no single ideal parameter for
noninvasive RAP estimation. Using the 2010 ASE
criteria,15 which is based on IVC parameters, RAP can
be categorized as low (05), normal (610), or elevated
(1120). A multiparameter approach from other methods

Fig. 59.4: Measuring right atrial (RA) dimensions. 2D echocardiographic images from the apical view focusing on the right heart.
On the left, the measurements of the RA major and minor axis
diameters are estimated at 4.87 and 3.07 cm, respectively. On the
right, the RA area tracing, which is 13.7 cm2.

available is to yield even more accurate estimation of

RAP. It is likely that the combination of measurements
using echocardiographic findings that measure dynamic
changes, flow, and dimensions will provide the best
noninvasive assessment of RAP.

PULMONARY ARTERY
HEMODYNAMICS (TABLE 59.2)
Pulmonary arterial hemodynamics are important for
patient diagnosis management, and prognosis. Current

1270

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

echo-Doppler modalities allow evaluation and estimation

of numerous parameters from the pulmonary vasculature:
systolic PAP (SPAP), DPAP, and mean PAP (MPAP) as well
as indirect estimation of other parameters such as PVR.

Systolic Pulmonary Artery Pressure

The SPAP is equal to the RV systolic pressure in the
absence of pulmonary valve stenosis or other RV outflow
tract (RVOT) obstruction. The normal value for the SPAP
with invasive measurements is between 15 and 30 mm Hg.
The SPAP equals the pressure gradient between the PA
and the RV plus the RAP. The pressure gradient (P) can
be calculated using the Bernoulli equation: P = 4 V2,
where V is the velocity of the tricuspid regurgitation (TR)
jet in cm/s (Fig. 59.5). In apparently healthy individuals,
the prevalence of TR upon Doppler echocardiography
varies within a range of 20 to 94% (depending on the age
of the cohort being studied).2830 Figure 59.6 shows the
pulmonary flow acceleration time (PAcT), an alternate,
less widely used method for screening patients for the
presence of pulmonary hypertension (PH). PAcT is the
interval between the onset of the forward flow in the PA
to its peak velocity. Values of <100 ms are associated with
PH.31,32 While the use of the Bernoulli equation has been
widely validated,33,34 it can be imprecise, especially in
patients with lung disease.35,36
Table 59.3 lists common causes of inaccuracy using
echo-Doppler for determination of PAP. Underestimation

Fig. 59.5: Evaluation of systolic pulmonary artery pressure (SPAP)

using Doppler imaging of the tricuspid regurgitation (TR) jet. The
maximal velocity (Vmax) is 368 cm/s. Using the Bernoulli equation:
P = 4 V2, a maximal pressure gradient (Max PG) of 54 mm Hg
between the right ventricle and atrium is calculated. When added
to the right atrial pressure, the SPAP can be estimated.

can be due to variations in Doppler angle of interrogation,

underestimation of RAP (especially when it is very
elevated; Figs 59.7A and B), the presence of severe TR, or a
poor TR signal.15,37 Overestimation of SPAP is less common
and often is due to overestimation of RAP; it can also
result from overestimating the TR signal peak velocity, as
well as mistakenly using the tricuspid valve closing spike
due to tricuspid valve closure for the tricuspid maximal
velocity. Nonetheless, Doppler estimation of SPAP is used
as a standard reliable method for screening patients for
suspected PH. A TR velocity of >2.8 m/s (corresponding to
a pressure gradient of 31 mm Hg) is regarded as the cutoff
velocity to define elevated SPAP. However, in obese38 and
elderly39 patients, the normal cutoff may be higher.
To reduce false-negative results, multiple imaging
planes and color Doppler should be used for optimal alignment with the regurgitant jet. In cases with a poor Doppler
signal, it can be enhanced with either agitated saline,
contrast, or an airbloodsaline mixture4042 (Fig. 59.8).
However, this can also lead to overestimation of the TR
velocity due to signal artifacts.

Diastolic Pulmonary Artery Pressure

The DPAP is equivalent to the LA and LV end-diastolic
pressure (LVEDP) when evaluated in individuals without
moderate or severe PH. Normal range is between 6 and
12 mm Hg. In patients with a PVR of >200 dynes/s/cm5

Fig. 59.6: Evaluation of systolic pulmonary artery pressure (SPAP)

using PA acceleration time (PAcT), which is the interval between
the onset of the forward flow in the PA to its peak velocity (yellow
line). A value < 100 ms is associated with an elevated PAP. In this
patient (the same patient in Figure 59.5), the PAcT is 63 ms, consistent with an elevated SPAP.

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

1271

Table 59.3: Causes for Inaccurate Estimation of Pulmonary Artery Pressure by Echocardiography

Cause

Resolution

Underestimation:
Variations in Doppler angle of interrogation

Multiple imaging planes to receive best Doppler signal, use of color Doppler
for optimal alignment with the regurgitant jet

Underestimation of right atrial pressure (RAP)

Adequate assessment of RAP using a multiparameter approach

Severe tricuspid regurgitation

Poor Doppler signal

Enhance signal with either, agitated saline, contrast, or an

airbloodsaline mixture.

Overestimation:
Overestimation of RAP

Adequate assessment of RAP using a multiparameter approach

Mistakenly using the tricuspid valve closing

spike for the tricuspid maximal velocity

Adequate analysis of the tricuspid Doppler signal

Contrast artifacts

Adequate use of contrast, and adequate analysis of the tricuspid Doppler signal

Figs 59.7A and B: Underestimation of systolic pulmonary artery pressure (SPAP). In this patient the estimated tricuspid regurgitation (TR)
gradient was 42 mm Hg. The estimated right atrial pressure (RAP) using inferior vena cava diameter of 2.78 cm and absence of respiratory collapse (bottom) was 15 mm Hg, giving an estimated SPAP of 57 mm Hg. However, the actual pulmonary artery catheter reading
for SPAP was 84 mm Hg. This underestimation was due to underestimation of the RAP, which was measured invasively as 40 mm Hg.

Figs 59.8A to C: Enhancing a poor tricuspid regurgitation (TR) Doppler signal (A) with saline (B) or a saline + blood mixture (C), which
gives even more enhanced results.

1272

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 59.9: Evaluation of diastolic and mean pulmonary artery pressure (DPAP and MPAP) using Doppler imaging of the pulmonic
regurgitation (PR) jet. The maximal velocity (Vmax) is 281 cm/s
(asterisk), and the velocity at end diastole is 195 cm/s (arrow).
Using the Bernoulli equation: P = 4 V2, the maximal pressure
gradient of 32 mm Hg between the pulmonary artery (PA) and the
right ventricle (RV) during diastole is calculated and corresponds
with the MPAP. When added to the right atrial pressure (RAP), this
improves accuracy. The gradient at end diastole between the PA
and the RV is also calculated and is 15 mm Hg. When added to
the RAP, the DPAP is given.

or a MPAP > 40 mm Hg, the DPAP is higher (>5 mm Hg

difference) than the mean pulmonary capillary wedge
pressure (PCWP).43 As demonstrated in Figure 59.9,
Doppler echocardiography can be used to estimate
DPAP15 by using the simplified Bernoulli equation with
the velocity of the pulmonic regurgitation (PR) jet at end
diastole providing the end-diastolic PARV gradient.
The pulmonary artery diastolic pressure (PADP) can be
estimated by adding the end-diastolic PARV to the RAP.44
This measurement correlates well with invasive measurements. The most common errors in DPAP estimation
have been attributed to inaccurate estimation of RAP.45
However, the PR jet is not always detected (even with the
use of saline).
The pulmonic valve opens when the RV and PA
pressures transiently equalize.46 The gradient between
the RA and RV can be measured using the TR velocity
and the velocity at time of pulmonary vein (PV) opening
combined with the RAP, allowing an estimate of the DPAP47
(with the use of superimposed QRS complexes from
the pulmonic flow and TR Doppler signals). However, as
these measurements are made on a steep portion of the
TR slope, any small timing error can lead to inaccurate
calculations of the DPAP.

Fig. 59.10: Estimation of the mean pulmonary artery pressure

(MPAP). The RARV mean systolic gradient is derived from tracing the tricuspid regurgitation profile and equals 42 mm Hg; adding
the RAP gives the MPAP.

Mean Pulmonary Artery Pressure

In individuals with a normal lung, the pulmonary
capillary hydrostatic pressure is equivalent to the PCWP.
However, in the presence of pulmonary venoconstriction
and PH, there can be a great difference between the lower
PCWP and the higher pulmonary capillary hydrostatic
pressure. In these situations, the DPAP does not necessarily
reflect adequate LA and LVEDP. It is then more important
to predict the MPAP, which reflects and classifies more
adequately the PAP. The peak PR jet identifies the diastolic
pressure gradient between the RV and the PA. Masuyama
et al.45 found that application of the Bernoulli equation to
the peak PR jet velocity provides an estimation of MPAP
(Fig. 59.9). Addition of the RAP improves the accuracy of
this estimate.48 Figure 59.10 demonstrates another simple
method to evaluate MPAP by adding the RAP to the RARV
mean systolic gradient, which can be derived from the TR
profile.49 As the relationship between SPAP and DPAP has
been shown to be constant,50 several empirical formulas
listed in Table 59.2 have been suggested for the estimation
of the MPAP.42,51 However, these data are derived from
invasive studies and have not been validated by Doppler
echocardiography.51

Pulmonary Vascular Resistance

The PVR is directly proportional to the pressure gradient
across the entire lungs from the PAP to the left atrial

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

pressure (LAP). PVR equals: [(MPAP mean PCWP)

80]/CO and is a hemodynamic variable, which
contributes to the management of patients with advanced
cardiovascular and pulmonary disease. Normal values
range between 20 and 130 dynes/s/m/cm5, which equals
0.25 1.6 woods units (WU). While increased SPAP may
be secondary to increased backflow from the heart, it
can also be the cause of pulmonary vascular disease.
An elevated PVR is used to define PH, and it is also
an essential component in the evaluation of patients
awaiting heart and lung transplantation52 as well as in the
determination of which patients should have closure of
their intracardiac shunt.53 Elevated values of PVR correlate
with worse clinical outcomes and prognosis in many
different patient populations.54,55 Initial studies evaluating
PVR noninvasively found only weak correlations with
invasive monitoring.56 However, using the maximal TR
velocity and the RVOT VTI has recently been shown to
correlate well with the transpulmonary pressure gradient
and transpulmonary flow, respectively (which are the
parameters used for invasive estimation of PVR). Using
the simple equation: PVR (WU) = 10 TR velocity/RVOT
VTI + 0.16.57 In patients with a ratio of <0.175, there is a
low likelihood to have a PVR > 2 WU, practically excluding

1273

pulmonary vascular disease.57 This ratio has been validated

in several studies, but in patients with a very high PVR
(>8 WU), its reliability as a quantitative measurement is
poor.58 Other additional, more complicated methods for
estimation of PVR are available5961 (Table 59.2). To date,
there have not been any comparative studies of the various
echo-Doppler methods used to assess PVR.

LEFT-SIDED FILLING PRESSURES

(TABLE 59.4)
Invasive measurements for left-sided filling pressures
include the PCWP that reflects the LAP. PCWP also reflects
LVEDP, which is the pressure within the left ventricle at the
onset of the QRS complex on electrocardiography (ECG)
aside from several conditions, which can cause overestimation (mitral stenosis) or underestimation (aortic insufficiency and a noncompliant left ventricle) of it. Noninvasive
assessment of left-sided filling pressures (LA and LV) is
done using the diastolic function parameters listed in
Table 59.4. Left-sided filling pressures are considered
elevated when the PCWP is > 12 mm Hg or the LVEDP is
> 16 mm Hg62 and elevated filling pressures are the main
physiological consequence of diastolic dysfunction.63

Table 59.4: The Various Methods Utilized for the Echocardiographic Evaluation of Left-Sided Filling Pressures (PCWP, LVEDP)

Method

Criteria Used

Strength and Limitations

Mitral inflow parameters: E-wave,

A-wave, DT, E/A ratio, IVRT.
Measured using pulse wave Doppler in the apical four-chamber
view (Fig. 59.11)

See Table 59.4 for normal values.

Obtainable in nearly all patients
Impaired LV relaxation: E/A < 1 or E/A > 2 U shape relation with LV diastolic
DT prolonged > 240 ms, IVRT prolonged
functionsimilar values for healthy and
Pseudonormal: 1< E/A < 2, 160 < DT
those with disease observed
< 240 ms
Difficult to interpret in the setting of:
Restrictive filling: E/A > 2, DT short
Sinus tachycardia
< 160 ms, IVRT short < 70 ms
Conduction system abnormalities
See also Table 59.7
Arrhythmias
Poor correlation in patients with coronary
artery disease and those with hypertrophic
cardiomyopathy with EF 50%

Pulmonary venous flow

(Figs 59.12A and B)

S > DNormal
S < Delevated LA pressure or normal in
young (<40 years) individuals.
Ar velocity < 35 cm/snormal
ArA-wave duration < 30 msnormal LA
pressure

Depth limitation
Marked cardiac enlargement
Left atrial motion artifact
Influenced by age (D dominant in young
individuals < 40 years)
Ar duration and velocity:
Not influenced by age
Applicable also in normal LVEF, MV
disease, and HCM
Hard to obtain good quality images for
interpretation
Contd...

1274

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Contd...
Method

Criteria Used

Strength and Limitations

Tissue Doppler imaging (TDI),

see Table 59.5 for normal values.
(Fig. 59.13)

,
,
E/ ratio < 8normal LV filling pressures
15 (for septal ) or > 12 (for lateral )
elevated LV filling pressures

Reduced with:
Aging
The presence of annular calcifications,
annular rings, prosthetic MV, MS
Increased with:
Moderate to severe MR
Constrictive pericarditis (lateral may be
less than septal in this situation)
May be affected by:
Preload in those with normal LVEF
LV relaxation
Systolic function
Lateral values higher than septal values
increased by:
Increased LA contractility
Decreased LVEDP
E/
Lateral ratio lower than septal
In patients with normal LVEF has low sensitivity and high specificity
In patients with mitral annular calcification, severe MR, or constrictive pericarditis
might not give an adequate estimate of
filling pressures.
Might not be valid for patients with acute
decompensated heart failure100

Propagation velocityVp

Vp > 50 cm/snormal
E/Vp > 2.5elevated PCWP > 15 mm Hg

Validated in patients with reduced LVEF

Poor reproducibility

Aortic (Fig. 59.14) and mitral

(Fig. 59.15) regurgitation jet
RA and LA shunt (Fig. 59.16)

LVEDP = diastolic BP (4 AR jet velocity2)

LAP = systolic BP (4 MR jet velocity2)
Intra atrial pressure difference = 4 V2

Validated only if no mitral stenosis is present

(DT: Deceleration time; HCM: Hypertrophic cardiomyopathy; IVRT: Isovolumic relaxation time; LA: Left atrium; LAP: Left atrial pressure; LV: Left ventricle; LVEF: Left ventricular ejection fraction; PCWP: Pulmonary capillary wedge pressure, LVEDP: Left ventricular
end diastolic pressure; MR: Mitral regurgitation; MS: Mitral stenosis; MV: Mitral valve; RA: Right atrium).

Mitral Inflow Parameters (Fig. 59.11)

Using the pulsed wave (PW) Doppler in the apical fourchamber view, assessment of the mitral inflow velocities
can be obtained, with images obtainable in nearly all
patients. The primary measurements include the peak
diastolic early filling (E-wave) and the diastolic late atrial
filling (A-wave) velocities, the ratio between these (E/A),
the peak velocity deceleration time (DT), A-wave duration,
and the IVRT (derived by placing the curser in the left
ventricular outflow tract [LVOT] to display simultaneously
the end of aortic ejection and onset of mitral inflow).

Normal values of the mitral inflow parameters vary with

aging. With increasing age, the E-wave decreases and the DT
and A-wave increases in amplitude, causing the E/A ratio to
decrease as well. The normal values as per age are shown in
Table 59.5.62 As listed in Table 59.6, heart rate and rhythm, PR
interval, CO, mitral annular size, and LA function62 as well as
other specific factors affect the mitral inflow.
It is well established that the mitral E-wave velocity
primarily reflects the LALV pressure gradient during the
early stage of diastole and is thus amenable to changes
in the preload and alterations in LV relaxation.62,64 The
mitral A-wave velocity reflects the LALV pressure

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

1275

Figs 59.11A and B: Mitral valve inflow Doppler. Primary measurements include the peak early filling (E) and the late diastolic atrial
filling (A) velocities, deceleration time (yellow line) of the E-wave. (A) patient with a normal filling pattern, the E-wave is greater than the
A wave with a deceleration time of 206 ms (>160 ms); (B) Restrictive filling pattern, the E-wave is greater than the A-wave with a very
short deceleration time of 137 ms.
Table 59.5: Normal Values by Age Groups for Doppler and Tissue Doppler Variables for Estimation of Left Heart Hemodynamics

Measurement

Age Group
1620

2140

4160

> 60

E/A ratio

1.88 0.45

1.53 0.4

1.28 0.25

0.96 0.18

DT (ms)

142 19

166 14

181 19

200 29

IVRT (ms)

50 9

67 8

74 7

87 7

Septal (cm/s)

14.9 2.4

15.5 2.7

12.2 2.3

10.4 2.1

Lateral (cm/s)

20.6 3.8

19.8 2.9

16.1 2.3

12.9 3.5

Ar duration (ms)

66 39

96 33

112 15

113 30

Source: Adopted from Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic
function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):10733.62
Table 59.6: Variables Affecting Mitral Inflow Parameters

Parameter

Variables

General parameters

E-wave

Aging
Preload
Alterations in left ventricular
(LV) relaxation

A-wave

Aging
LV compliance
Left atrial contractile function

E-wave deceleration
time (DT)

Aging
LV relaxation
LV diastolic pressure after mitral
valve opening
LV compliance

Heart rate and rhythm

PR interval
Cardiac output
Mitral annular size

gradient during the late stage of diastole, which is affected

by LV compliance and the LA contractile function.
The DT of the mitral E-wave is influenced by the LV
relaxation, LV diastolic pressure after mitral valve (MV)
opening, and the LV compliance. Alterations in the LV
end-systolic and/or end-diastolic volumes, LV elastic
recoil, and/or LV diastolic pressures directly affect the
mitral inflow velocities (E-wave) and the time intervals
(DT and IVRT).62
In patients with dilated cardiomyopathy, the mitral
inflow velocity parameters correlate better with functional
class, filling pressures, and prognosis than the calculated
left ventricular ejection fraction (LVEF).6577 However, in
patients with coronary artery disease,78 mitral regurgitation
(MR), or hypertrophic cardiomyopathy (HCM),79,80 where
the LVEF is >50%, mitral inflow variables do not correlate
as well with hemodynamic measurements.

1276

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

With the use of these parameters, especially the E/A

ratio and the DT, the echocardiographic filling pattern
can be classified to either normal (E > A, DT > 160 ms),
impaired LV relaxation (E < A, DT > 240 ms), pseudonormal
LV filling (E > A, DT > 160 ms), or restrictive LV filling
(E >> A, DT < 140 ms; Fig. 59.11).

Pulmonary venous flow also provides important information for the assessment of LV diastolic function and
LA filling pressure. In most patients, the best Doppler
recordings are obtained from the apical four-chamber
view with the pulmonary venous flow obtainable in
~approximately 90% of adult patients.81 As seen in Figures
59.12A and B, variables include the peak systolic velocity
(S), which is composed of two systolic components (S1,
S2), peak anterograde diastolic velocity (D), the S/D
ratio, and the duration and peak of the atrial reversal (Ar)
velocity waveform.
The S-wave is primarily influenced by changes in the
LA pressure, contraction, and relaxation (S1 component)
and by the stroke volume (SV) and pulse wave propagation
in the PA vasculature tree.82,83 The D-wave is influenced
by the same factors that influence the mitral E velocity.84
Ar duration and velocity are influenced by the LV late
diastolic pressure, atrial preload, and contractility.85 With

an increase in LA pressure, there is a decrease in S and

increase in D velocities resulting in an S/D ratio of <1.
When there is an increased LVEDP, the Ar velocity and
duration increase as well as the time difference between
Ar duration and the mitral A-wave duration.86,87
Normal values of pulmonary venous inflow are strongly
related to age. In young normal subjects, there is usually
prominent D velocity, reflecting their mitral E-wave. This
gradually declines (age > 40) with an increase in the S/D
ratio. The Ar velocity also usually increases with age but
normal values do not usually exceed 35 cm/s. A duration
difference of >30 ms between the Ar and the mitral inflow
A-wave (ArA duration) is the only age-independent
indication of LV A-wave pressure increase,88 which can
classify patients with abnormal LV relaxation into those
with elevated LVEDP but normal mean LA pressure,
which is the first hemodynamic abnormality seen with
diastolic dysfunction. Other variables such as maximal
LA size, E-wave DT, and a pseudonormal filling pattern,
are all indicative of an increase in the mean LA pressure
and a more advanced stage of diastolic dysfunction.62
Importantly, unlike mitral inflow parameters, the ArA
duration difference is still accurate in various patient
populations such as those with: normal ejection fraction
(EF),78 MV disease,89 and HCM.80 Yet, one of the important limitations using the Ar parameters is obtaining
high-quality images suitable for accurate reproducible
measurements.

Pulmonary Venous Flow

(Figs 59.12A and B)

Figs 59.12A and B: Doppler of the pulmonary vein flow pattern from the apical four-chamber view. (A) normal Doppler study demonstrates
the peak systolic velocity (S), which is composed of two systolic components (S1, S2), peak anterograde diastolic velocity (D), and peak
of the atrial reversal (Ar) velocity waveform; (B) An examination from a patient with an elevated left atrial filling pressure demonstrating
a decrease in S and increase in D, resulting in an S/D ratio of <1.

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

1277

TDI measurements include both systolic (S) and diastolic

velocities. The early diastolic velocities are expressed as
Ea, Em, , or , and the late diastolic velocity as Aa, Am, ,
or , with mostly used terms being and . TDI is acquired
using PW Doppler from the apical views to acquire the
mitral annular velocities.90 ASE guidelines62 recommend
acquiring and measuring TDI signals from both the septal
and lateral sides of the MV annulus. The normal values of
these parameters are influenced by age like other indices of
LV diastolic function, with a decrease in and an increase in
and the E/ ratio;91 normal values are shown in Table 59.5.
The hemodynamic determinants of the velocity include
LV relaxation, preload, systolic function, and LV minimal
pressure.62 A significant association between and LV
relaxation has been shown in several studies.92,93 While
preload has minimal effect on in the presence of LV
impaired relaxation,94,95 it increases in patients with
normal or enhanced LV relaxation.9497 Due to this, in
patients with cardiac disease, velocity can be used to
correct for the effect of LV relaxation on mitral E velocity,
and the E/ ratio can be applied for the prediction of
LV filling pressures (Figs 59.13A and B).62 The main
determinants of include LA systolic function and LVEDP
such that increased LA contractility leads to increased
velocity, whereas increased LVEDP leads to decreased
velocity.94 Once acquired, it is possible to also calculate

additional time intervals and ratios using a combination

of TDI and mitral inflow parameters such as the E velocity
to ratio (E/), which plays an important role in the
estimation of LV filling pressures. The time differential
between the QRS and both E and gives the TE- and also
provides additional information on diastolic function. TE-
is prolonged in patients with diastolic dysfunction.
The combination of TDI and mitral inflow velocities
allows for prediction of LV filling pressures. However, it is
important to take into context the clinical parameters in
order to make a reliable assessment (i.e. age, presence of
cardiovascular disease, other echocardiographic abnormalities). Unfortunately, these criteria are limited in their
accuracy for LV filling pressures.
It is recommended to use the average velocity
obtained from both the lateral and septal sides of the
mitral annulus for the prediction of LV filling pressure. The
septal is usually lower than the lateral velocity, so the
E/ ratio from the septal signal is usually higher than the
lateral . In the context of regional myocardial dysfunction,
it is recommended by the ASE to use the average
velocity, and in patient with atrial fibrillation an average
of measurements from 10 cardiac cycles is most accurate.
An E/ ratio of <8 (for either septal or lateral) is usually
associated with normal LV filling pressures. Conversely, a
ratio 15 for septal or 12 for lateral is associated with
increased LV filling pressures.62 When the value is midrange
(between 8 and 15), other indices should be used to assess
LV filling pressures. In these patients and also in those with

Tissue Doppler Annular Early and Late

Diastolic Velocities (Fig. 59.13)

Figs 59.13A and B: Evaluation of left-sided filling pressures. (A) Mitral inflow velocities are measured (E, A), showing an E > A with a
pseudonormal filling pattern (deceleration time > 160 ms); (B) Lateral tissue Doppler imaging (TDI) that includes both systolic (S) and
diastolic velocities. The early diastolic velocity is expressed as , and the late diastolic velocity as . In this patient, the E/ is elevated
and equals89/4.66 = 19, implying elevated left-sided filling pressures.

1278

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

normal EF, or with MV disease, using the TE- might provide

additional input. It has been also shown in more recent
studies that in those with normal EF, the lateral has the
best correlation with LV filling pressures and invasive
parameters of LV stiffness.98,99 It is controversial whether
in patients with acute decompensated heart failure E/ is
valid.100102

Color M-mode Flow Propagation

Velocity (Vp)
The left ventricular filing is dominated by an early wave
and an atrial-induced filling wave. In a normal ventricle,
the early filling forces are attributed to suction of blood
from the atria and the filling wave propagates rapidly
toward the apex, driven by the pressure gradient between
the LV base and the apex. Using of color flow imaging and
M-mode echocardiography placed through the center of
the LV inflow from the MV to the apex, the propagation
velocity can be estimated. A Vp > 50 cm/s is considered
normal,103,104 while in patients with myocardial ischemia
or heart failure there is slowing of the mitral to apical
flow propagation measured. In patients with reduced
LVEF (< 50%), the E/Vp ratio can be used to predict the LV
filling pressures,104 with E/Vp 2.5 predicting a PCWP of
>15 mm Hg.98

However, Vp is often not routinely evaluated due to

poor reproducibility and limited sensitivity and specificity. In addition, other signs of ventricular impairment
and filling pressure are usually apparent during the
echocardiographic evaluation, making Vp useful as
a complimentary index when there are inconsistent
findings. In patients with a normal LVEF, Vp can be falsely
normal despiteelevated filling pressures.105,106

Left Atrial Dimensions

Chronic elevation of left-sided filling pressures leads to LA
enlargement. There is a significant association between
LA dimensions and elevated left-sided filling pressures
and evaluation of LA dimensions is an important adjunct
to the echocardiographic evaluation of the left-sided filling
pressures.107 LA measurements are usually obtained most
accurately from the apical views.14 However, LA enlargement is not a specific sign for elevated filling pressures as it
can accompany also situations where the left-sided filling
pressures are not elevated and diastolic dysfunction is not
present such as in trained athletes, patients with chronic
atrial fibrillation or flutter, bradycardia, high output states
and mitral valvular disease.
Mitral inflow pattern, PV flow, TDI and Vp can serve
as useful tools for evaluation and estimation of left-sided
filling pressures. Table 59.7 lists the main differences

Table 59.7: Variables Used for the Evaluation of Filling Pressures in Those with Normal and with Decreased Ejection Fraction (EF)

Variable

Patients with Depressed EF

Patients with Normal EF

E/A

< 1 Normal FP (if E 50 cm/s)

1<2, < 1 and E > 50 cm/sindeterminate
2 elevated FP (with DT < 150 ms)

NA

E/

< 8normal FP
812indeterminate
13elevated FP

8 (Sep, Lat, Avg)normal FP

Sep 15, Lat 12, Avg 13elevated FP

Vp

> 50 cm/snormal
E/Vp > 2.5elevated FP >15 mm Hg

Can be falsely normal despite elevated FP

Ar-A

< 0 msnormal FP
029indeterminate
30 mselevated FP

< 0 msnormal FP
029indeterminate
30 mselevated FP

IVRT/TE-

> 2normal FP
< 2elevated FP

> 2normal FP
< 2elevated FP

Systolic pulmonary artery pressure

< 30 mm Hgnormal FP
> 35 mm Hgelevated FP

< 30 mm Hgnormal FP
> 35 mm Hgelevated FP

Pulmonary vein S/D

> 1normal FP
< 1Elevated FP

Limited accuracy

Left atrial volume

Some LA dilation may occur in this population < 34 mL/m2normal FP

in the presence of normal FP. Thus, this param- 34 mL/m2Elevated FP
eter should not be used in this population.

(Avg: Average; EF: Ejection fraction; FP: Filling pressure; Lat: Lateral; NA: Not applicable; Sep: Septal).

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

in the evaluation of filling pressures in patients with

either a decreased or a normal LVEF. While in patients
with impaired myocardial function the primary tool for
evaluation is the E/A ratio, in those with a normal LVEF the
estimation can be more challenging, primarily assessed
using the E/ ratio preferably with the use of other
parameters.107 A simplified diagnostic algorithm for the
evaluation of different patient populations based on the
ASE regarding the evaluation of left ventricular diastolic
function62 is shown in Figure 59.14.

ADDITIONAL PARAMETERS FOR ESTIMATION OF LEFT ATRIAL PRESSURE

Aortic and Mitral Regurgitation
Continuous Wave Doppler Signal
The LAP equals the LVEDP in the absence of mitral
stenosis. In patients with aortic regurgitation (AR), using
the continuous wave (CW) Doppler signal of the AR jet
gives the pressure gradient between the aorta and the LV
at end diastole. Peak AR velocity should be >4 m/s, and the

1279

LVEDP can be calculated as the diastolic aortic pressure

minus the pressure gradient at end diastole which, by
using the modified Bernoulli equation, is equal to 4V2
(Fig. 59.15). Diastolic blood pressure should be obtained
at the same time the AR jet is being interrogated. The
maximal pressure gradient between the LV and LA during
systole can be determined by CW Doppler in patients with
MR. LAP can be calculated as the difference between the
systolic blood pressure and the MR gradient, by using
the modified Bernoulli equation as well (Fig. 59.16). In
patients with mitral stenosis, the LA diastolic pressure is
the sum of the LVEDP and the transmitral gradient. Again,
care must be taken to obtain blood pressure at the same
time as Doppler measurements are being obtained.108

Atrial Septal Defect/Patent

Foramen Ovale Flow
As shown in Figure 59.17, by adding the estimated RAP
to the pressure gradient between the RA and the LA
as evaluated by CW Doppler, it is possible to estimate
the LAP.

Fig. 59.14: Schematic approach for the evaluation of left-sided filling pressures using the various obtained echocardiographic parameters. (DT: Deceleration time; IVRT: Isovolumic relaxation time; LA: Left atrium; PV: Pulmonary vein; SPAP: Systolic pulmonary artery
pressure).
*Refers for averaged values from both septal and lateral tissue Doppler; for specific values see Table 7.
**See text for further details. Adopted from Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left
ventricular diastolic function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):10733.62

1280

Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

Fig. 59.15: Measurement of left ventricular end diastolic pressure (LVEDP) using the aortic regurgitation (AR) signal. Using
continuous wave Doppler through the aortic valve in diastole, the
end-diastolic AR velocity gives out the pressure gradient (white arrow) which equals to 4 3.42 = 46 mm Hg. Subtracting this value
from the systolic blood pressure gives an estimate of the left atrial
pressure during systole (V-wave). The LVEDP equals the diastolic
blood pressure4V2.

Fig. 59.16: Estimation of the pressure gradient between the left

ventricle and left atrium (LA). Using continuous wave Doppler
through the mitral valve in systole, the peak MR velocity gives
out the maximal pressure gradient (white arrow), which equals to
4 4.872 = 95 mm Hg. Subtracting this value from the systolic
blood pressure gives an estimate of the LA pressure during
systole (V-wave).

STROKE VOLUME, STROKE DISTANCE,

CARDIAC OUTPUT, AND SYSTEMIC
PULMONARY SHUNTS (QP/QS)

the absence of pulmonic shunting, the pulmonic outflow

tract can be used instead of the LVOT.111
By determining the transmitral and transpulmonary
flow, it is possible to noninvasively obtain the pulmonic
to systemic flow ratio (QP/QS) in various shunt disease,
if present. However, calculation of pulmonary flow can
sometimes result in significant errors, mainly due to the
inability to adequately visualize and measure the PA
diameter or RVOT diameter. As the case in severe AR,
significant PR can lead to overestimation of the right
ventricular SV. While in patients with severe AR, Doppler
can falsely underestimate the QP/QS, in those with
significant PR it can lead to falsely overestimating it.

CO equals SV multiplied by the heart rate (CO = SV

heart rate). Using Doppler, it is possible to measure the
stroke distance, which refers to the distance traveled by a
column of blood during a fixed time (the cardiac cycle).
Multiplying the stroke distance with the cross-sectional
area through which the column moves gives the SV. This
method can be obtained at several sites, with the most
common and accurate being the LVOT109,110 (Fig. 59.18). It
is necessary that Doppler velocities be obtained parallel
to the direction of flow and that the cross-sectional area
through which the flow is occurring is obtained. As the
outflow tract is often elliptical and not circular, it may be
more appropriate to use the stroke distance rather than the
CO, as it may be miscalculated due to the aortic annulus
being elliptical. Significant AR will lead to overestimation
of the SV and consequently the CO. When present, and in

A WORD TO CONCLUDE
As noninvasive evaluation utilizes indirect indexes for
estimation of hemodynamic parameters, it is far superior
to bedside physical examination but it also has limitations.
However, in addition to providing hemodynamic data and
volume status, the Doppler data provides insight into the
pathophysiology of ventricular filling and emptying.

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

Fig. 59.17: Estimation of the left atrial (LA) pressure. On the top
image: color flow imaging demonstrating a shunt between the
right atrium (RA) and LA. On the bottom image: continuous wave
Doppler for estimation of the pressure gradient between the RA
and the LA (white arrow), which equals 4 2.742 = 30 mm Hg.
Adding this gradient to the estimated RAP gives the estimated
LA pressure.

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34(3):8029.

Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy

84. Nishimura RA, Abel MD, Hatle LK, et al. Relation of

pulmonary vein to mitral flow velocities by transesophageal
Doppler echocardiography. Effect of different loading
conditions. Circulation. 1990;81(5):148897.
85. Keren G, Bier A, Sherez J, et al. Atrial contraction is an
important determinant of pulmonary venous flow. J Am
Coll Cardiol. 1986;7(3):6935.
86. Rossvoll O, Hatle LK. Pulmonary venous flow velocities
recorded by transthoracic Doppler ultrasound: relation to
left ventricular diastolic pressures. J Am Coll Cardiol. 1993;
21(7):168796.
87. Yamamoto K, Nishimura RA, Burnett JC Jr, et al. Assessment
of left ventricular end-diastolic pressure by Doppler echocardiography: contribution of duration of pulmonary
venous versus mitral flow velocity curves at atrial contraction. J Am Soc Echocardiogr. 1997;10(1):529.
88. Klein AL, Tajik AJ. Doppler assessment of pulmonary
venous flow in healthy subjects and in patients with heart
disease. J Am Soc Echocardiogr. 1991;4(4):37992.
89. Rossi A, Cicoira M, Golia G, et al. Mitral regurgitation
and left ventricular diastolic dysfunction similarly affect
mitral and pulmonary vein flow Doppler parameters: the
advantage of end-diastolic markers. J Am Soc Echocardiogr.
2001;14(6):5628.
90. Waggoner AD, Bierig SM. Tissue Doppler imaging: a useful
echocardiographic method for the cardiac sonographer to
assess systolic and diastolic ventricular function. J Am Soc
Echocardiogr. 2001;14(12):114352.
91. De Sutter J, De Backer J, Van de Veire N, et al. Effects of age,
gender, and left ventricular mass on septal mitral annulus
velocity (E) and the ratio of transmitral early peak velocity
to E (E/E). Am J Cardiol. 2005;95(8):10203.
92. Sohn DW, Chai IH, Lee DJ, et al. Assessment of mitral
annulus velocity by Doppler tissue imaging in the
evaluation of left ventricular diastolic function. J Am Coll
Cardiol. 1997;30(2):47480.
93. Ommen SR, Nishimura RA, Appleton CP, et al. Clinical utility
of Doppler echocardiography and tissue Doppler imaging
in the estimation of left ventricular filling pressures: A
comparative simultaneous Doppler-catheterization study.
Circulation. 2000;102(15):178894.
94. Nagueh SF, Sun H, Kopelen HA, Middleton KJ, Khoury
DS. Hemodynamic determinants of the mitral annulus
diastolic velocities by tissue Doppler. J Am Coll Cardiol.
2001;37(1):27885.
95. Hasegawa H, Little WC, Ohno M, et al. Diastolic mitral
annular velocity during the development of heart failure.
J Am Coll Cardiol. 2003;41(9):159097.
96. Firstenberg MS, Levine BD, Garcia MJ, et al. Relationship of
echocardiographic indices to pulmonary capillary wedge
pressures in healthy volunteers. J Am Coll Cardiol. 2000;
36(5):16649.
97. Caiani EG, Weinert L, Takeuchi M, et al. Evaluation of
alterations on mitral annulus velocities, strain, and strain
rates due to abrupt changes in preload elicited by parabolic
flight. J Appl Physiol. 2007;103(1):807.

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98. Rivas-Gotz C, Manolios M, Thohan V, et al. Impact of

left ventricular ejection fraction on estimation of left
ventricular filling pressures using tissue Doppler and flow
propagation velocity. Am J Cardiol. 2003;91(6):7804.
99. Kasner M, Westermann D, Steendijk P, et al. Utility of
Doppler echocardiography and tissue Doppler imaging in
the estimation of diastolic function in heart failure with
normal ejection fraction: a comparative Doppler-conductance catheterization study. Circulation. 2007;116(6):
63747.
100. Mullens W, Borowski AG, Curtin RJ, et al. Tissue Doppler
imaging in the estimation of intracardiac filling pressure
in decompensated patients with advanced systolic heart
failure. Circulation. 2009;119(1):6270.
101. Nagueh SF; ASE and EAE Diastology Writing Group.
Letter by Nagueh et al. regarding article, Tissue Doppler
imaging in the estimation of intracardiac filling pressure
in decompensated patients with advanced systolic heart
failure. Circulation. 2009;120(7):e44.
102. Galderisi M, Esposito R. Letter by Galderisi and Esposito
regarding article, Tissue Doppler imaging in the estimation of intracardiac filling pressure in decompensated
patients with advanced systolic heart failure. Circulation.
2009;120(7):e46.
103. Takatsuji H, Mikami T, Urasawa K, et al. A new approach for
evaluation of left ventricular diastolic function: spatial and
temporal analysis of left ventricular filling flow propagation
by color M-mode Doppler echocardiography. J Am Coll
Cardiol. 1996;27(2):36571.
104. Garcia MJ, Ares MA, Asher C, et al. An index of early left
ventricular filling that combined with pulsed Doppler peak
E velocity may estimate capillary wedge pressure. J Am Coll
Cardiol. 1997;29(2):44854.
105. Lin SK, Hsiao SH, Lee TY, et al. Color M-mode flow
propagation velocity: is it really preload independent?
Echocardiography. 2005;22(8):63641.
106. Hsiao SH, Huang WC, Sy CL, et al. Doppler tissue imaging
and color M-mode flow propagation velocity: are they
really preload independent? J Am Soc Echocardiogr. 2005;
18(12):127784.
107. Rafique AM, Phan A, Tehrani F, et al. Transthoracic echocardiographic parameters in the estimation of pulmonary
capillary wedge pressure in patients with present or previous heart failure. Am J Cardiol. 2012;110(5):68994.
108. Ahmed SN, Syed FM, Porembka DT. Echocardiographic
evaluation of hemodynamic parameters. Crit Care Med.
2007;35(8 Suppl):S3239.
109. Lewis JF, Kuo LC, Nelson JG, et al. Pulsed Doppler echocardiographic determination of stroke volume and cardiac
output: clinical validation of two new methods using the
apical window. Circulation. 1984;70(3):42531.
110. Dubin J, Wallerson DC, Cody RJ, et al. Comparative accuracy
of Doppler echocardiographic methods for clinical stroke
volume determination. Am Heart J. 1990;120(1):11623.
111. Kirkpatrick JN, Lang RM. Heart failure: hemodynamic
assessment using echocardiography. Curr Cardiol Rep.
2008;10(3):2406.

SECTION 5
Ischemic Heart Disease,
Cardiomyopathies, Pericardial
Disorders, Tumors and Masses

Chapters
Chapter 60
Chapter 61
Chapter 62
Chapter 63
Chapter 64

Echocardiography in Ischemic Heart Disease

Stress Echocardiography
Squatting Stress Echocardiography
Three-Dimensional Stress Echocardiography
Echocardiographic Assessment of Coronary
Arteries Morphology and Coronary Flow Reserve
Chapter 65 Echocardiography in Hypertrophic Cardiomyopathy
Chapter 66 Echocardiographic Assessment of Nonobstructive
Cardiomyopathies

Chapter 67 Echocardiographic Differentiation of Ischemic and

Nonischemic Cardiomyopathy: Comparison with
Other Noninvasive Modalities
Chapter 68 Pericardial Disease
Chapter 69 Three-Dimensional Echocardiographic
Assessment in Pericardial Disorders
Chapter 70 Echocardiographic Assessment of Cardiac
Tumors and Masses

CHAPTER 60
Echocardiography in Ischemic
Heart Disease
Chetan Shenoy, Hamid Reza Salehi, Francesco F Faletra, Natesa G Pandian

Snapshot

Detecon of Ischemia

Role in Acute Coronary Syndromes

Mechanical Complicaons of Myocardial Infarcon

Role of Echocardiography in Chronic Ischemic

Novel Echocardiography Techniques in Ischemic

Heart Disease

Future

Cardiomyopathy

INTRODUCTION
Coronary artery disease (CAD) is the leading cause of death
for both men and women in the United States. Each year,
an estimated 785,000 Americans will have a new coronary
event, and nearly 470,000 will have a recurrent attack.1 It is
estimated that an additional 195,000 silent first myocardial
infarctions (MIs) occur each year.1 Approximately every
25 seconds, an American will have a coronary event, and
approximately every minute, someone will die of one.1
Noninvasive diagnosis and evaluation of the effects of
CAD are important in risk stratification and guides disease
management. Since the 1980s, echocardiography has been
the mainstay of cardiac imaging in the field of noninvasive
evaluation of CAD.2
Echocardiography has a multifaceted role in ischemic
heart disease.2 It can be used for the noninvasive detection
of chronic ischemia, acute coronary syndrome (ACS),
complications of ACS, and consequences of ischemic heart
disease. The role of echocardiography in ischemic heart
disease is well established, time-tested, and supported
by extensive literature. Advances in echocardiographic

imaging technology such as tissue harmonic imaging and

contrast echocardiography have significantly improved
the accuracy and reliability of the modality in ischemic
heart disease.
In this chapter, we will discuss established as well as
novel and emerging applications of echocardiography in
ischemic heart disease.

DETECTION OF ISCHEMIA
Stress echocardiography is commonly used for the
detection of chronic ischemia in patients with known or
suspected ischemic heart disease.36 Stress echocardiography can be performed either as an exercise test or as a
pharmacological stress test. For patients who are capable
of performing an exercise test, exercise stress rather than
pharmacological stress is recommended, as the exercise
capacity is a reliable predictor of outcomes.
While either treadmill or bicycle exercise may be
used for exercise stress, the treadmill is widely used in the
United States.37 Symptom-limited exercise according to a
standardized protocol in which the workload is gradually

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increased in stages is performed.35 The Bruce protocol is

most commonly used for treadmill exercise echocardiography in the United States. Imaging is performed at rest
and immediately after cessation of exercise.35 Exercise
stress echocardiography provides valuable information
for detection of ischemic heart disease and assessment of
valvular heart disease (Fig. 60.1).
Pharmacological stress testing is performed in
patients who cannot exercise.5,6 Commonly used agents
for pharmacological stress echocardiography include
dobutamine and vasodilators. Although vasodilators
may have advantages for assessment of myocardial
perfusion, dobutamine is preferred when the test is based
on assessment of regional wall motion.8 The standard
for dobutamine stress testing is a graded dobutamine
infusion starting at 5 g/kg/min and increasing at
3-minutes intervals to 10, 20, 30 and 40 g/kg/min.8 The
low-dose stages allow detection of viability and ischemia
in segments with abnormal function at rest, even when
assessment of viability is not the primary objective of
the test.8
Endpoints of a dobutamine stress echocardiography
study are achievement of target heart rate (defined as
85% of the age-predicted maximum heart rate), new
or worsening wall-motion abnormalities, significant
arrhythmias, hypotension, severe hypertension, and
intolerable symptoms.6,8 Atropine, in divided doses of 0.25
to 0.5 mg to a total of 2.0 mg, could be used as needed
to achieve the target heart rate.8 Atropine increases the

sensitivity of dobutamine echocardiography in patients

receiving -blockers and in those with single-vessel
disease8 (Fig. 60.2).
Both dobutamine and exercise echocardiography
result in a marked increase of heart rate. The increase in
blood pressure is much less with dobutamine compared
with exercise. With both techniques, the induction of
ischemia is related to an increase in myocardial oxygen
demand.
Vasodilator stress testing may be performed with
adenosine or dipyridamole.6 Atropine is routinely
used with vasodilator stress to enhance test sensitivity.
The addition of handgrip at peak infusion enhances
sensitivity. Vasodilator stress echocardiography usually
produces a mild-to-moderate increase in heart rate and a
mild decrease in blood pressure. While adenosine stress
is used to assess myocardial perfusion with contrast
echocardiography, it has not been widely used as a clinical
tool.6
Interpretation of stress echocardiographic images
involves visual assessment of endocardial excursion and
wall thickening.6 Either a 16- or 17-segment model of the
left ventricle (LV) may be used.6 Function in each segment
is graded at rest and with stress as normal or hyperdynamic, hypokinetic, akinetic, dyskinetic, or aneurysmal.6
Images from low or intermediate stages of dobutamine
infusion should be compared with peak stress images to
maximize the sensitivity for detection of coronary disease6
(Fig. 60.2).

Fig. 60.1: Example of an exercise stress echocardiogram using

contrast, demonstrating anterior wall ischemia (arrows). Upper
panelsrest, lower panelsstress. Left panelsdiastolic frames,
right panelssystolic frame.

Fig. 60.2: Example of a dobutamine exercise stress echocardiogram, demonstrating inferior wall ischemia (arrows). Upper
panelsrest, lower panelsstress. Left panelsdiastolic frames,
right panelssystolic frame.

Chapter 60: Echocardiography in Ischemic Heart Disease

A normal stress echocardiogram is defined as

normal LV wall motion at rest and with stress.6 Abnormal
study findings include those with fixed wall-motion
abnormalities (i.e. resting wall-motion abnormalities,
unchanged with stress, which most often represent
regions of prior infarction), or new or worsening wallmotion abnormalities indicative of ischemia6 (Figs 60.1
and 60.2). In addition to the evaluation of segmental
function, the global response of the LV to stress should be
assessed. Stress-induced changes in LV shape, cavity size,
and global contractility are also indicators of the presence
or absence of ischemia and may indicate multivessel
disease.6 The total amount of myocardium in jeopardy
predicts risk, and prolonged persistence of systolic wallthickening abnormality may also identify severe CAD.
Stress echocardiography can also predict the presence of
myocardial hibernation when wall-motion abnormalities
at rest improve or resolve with stress.6
Based on pooled data, stress echocardiography has an
average sensitivity of 88% and an average specificity of 83%
for the detection of coronary artery stenosis (generally
>50% diameter stenosis by angiography).6 Studies comparing the accuracy of nuclear perfusion imaging and
stress echocardiography in the same patient population
have shown that the tests have similar sensitivities for the
detection of CAD, but stress echocardiography has higher
specificity. In a pooled analysis of 18 studies in 1,304
patients who underwent exercise or pharmacological
stress echocardiography in conjunction with thallium- or
technetium-labeled radioisotope imaging, sensitivity and
specificity were 80 and 86% for echocardiography, and
84 and 77% for myocardial perfusion imaging, respectively.6
The relatively high specificity of stress echocardiography
contributes to its use as a cost-effective diagnostic method.
False-negative stress echocardiography studies result
primarily from suboptimal stress. They are more common
in patients with single-vessel disease or disease of the
left circumflex artery because of the smaller amount of
myocardium supplied by the left circumflex coronary
artery, in patients with a small LV cavity and increased
relative wall thickness, and in patients with significant
aortic or mitral regurgitation (MR) leading to a hyperdynamic LV.6
False-positive stress echocardiography studies can be
seen in patients with coronary artery spasm or in patients
with decreased myocardial perfusion reserve in patients
with LV hypertrophy, syndrome X, diabetes mellitus, myocarditis, and idiopathic cardiomyopathy.6 Stress-induced

1291

wall-motion abnormalities may also be seen in patients

with hypertension or underlying cardiomyopathy, in the
absence of ischemia. Finally, tethering of LV myocardium
due to patients with significant mitral annular calcification
or prior mitral valve replacement could lead to a reduction
in motion of adjacent basal inferior and basal inferoseptal
segments and result in false-positive stress studies.6
A normal stress echocardiogram is associated with an
annual mortality risk of 0.4 to 0.9%, equivalent to that of an
age- and sex-matched population, based on a total of 9,000
patients; thus, in patients with suspected CAD, a normal
stress echocardiogram confers an excellent prognosis
and coronary angiography can safely be avoided.6 With
an abnormal stress echocardiogram, the risk of future
mortality is directly related to the extent of the wall-motion
abnormalities. Patients with extensive stress-induced
abnormalities in a multivessel distribution are at a high
risk of mortality and cardiac events.
Variables on a stress echocardiogram that are
associated with adverse outcomes include baseline LV
dysfunction, wall-motion abnormalities in multivessel
distribution, extensive ischemia, location of wall-motion
abnormalities in left anterior descending coronary artery
distribution, poor ejection fraction (EF) response or
failure to reduce end-systolic volume with exercise, a low
ischemic threshold and LV hypertrophy.6

Myocardial Contrast Stress

Echocardiography
Development of ultrasound contrast agents containing
microbubbles that mimic red blood cell rheology, now
allows simultaneous assessment of both function and
perfusion via myocardial perfusion imaging, making it a
unique technique for the assessment of CAD.9,10
Contrast stress echocardiography allows enhanced
assessment of wall motion both at rest and during stress
by improving visualization of the endocardial border, by
improving the confidence in wall-motion assessment, and
by reducing the number of uninterpretable images. Thus,
by increasing the accuracy of wall-motion assessment,
contrast echocardiography enhances the diagnostic value
of stress echocardiography for the detection of CAD9,10
(Fig. 60.1).
The onset of ischemic wall-motion abnormalities
is preceded by development of regional disparities in
coronary perfusion that can be assessed by contrast agents.
Contrast echocardiography can, therefore, be employed

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Echocardiography today plays an essential role in the

diagnosis of ACSs and their related complications.11,12 In
patients presenting to the emergency room with chest
pain suggestive of an ACS but with negative biomarkers
and a nondiagnostic electrocardiogram (ECG), echocardiography plays an important role for diagnosis and
prognostication.11,12
With a critical stenosis and interruption of blood flow
in an epicardial coronary artery, the loss of myocardial
function and development of clinical signs and symptoms
proceed in a stepwise process referred to as the ischemic
cascade. It starts with a defect in perfusion and progresses
through abnormalities in left ventricular diastolic
function, decreased myocardial contractility, increased left
ventricular end-diastolic pressure, ST-segment changes,
and occasionally, chest pain.13 This ischemic cascade
forms the basis of the application of echocardiography

in the diagnosis of ACSs.13 Assessment of decreased

myocardial contractility as noted by resting wall-motion
abnormalities is the primary technique used in patients
with ACS, while other less established techniques include
assessment of diastolic dysfunction, and assessment of
myocardial perfusion using contrast echocardiography.14
Stress echocardiography is the other important technique
used in these patients.11,12
With unstable angina, the duration of ischemia
and consequently the wall-motion abnormalities may
be short-lived. Hence, assessment of wall-motion
abnormality in patients with suspected ACS should be
performed early after the onset of symptoms; normal wall
motion in a patient with a normal ECG and without chest
pain does not exclude an ACS. Conversely, if the patient
has ischemic chest pain, a normal or equivocal ECG,
and no regional wall-motion abnormalities on resting
echocardiography during or immediately after the acute
episode, the presence of acute ischemia is unlikely. The
sensitivity of echocardiography in detection of ACS via
resting wall-motion abnormalities is 90 to 95% with a
negative predictive value of 90%15,16 (Figs 60.3 to 60.5).
After an ACS has been diagnosed, echocardiographic
assessment of wall motion aids in the assessment of site
and severity of the acute CAD. The site of the myocardial
insult and consequently, the infarct-related coronary
artery, can be readily identified and the extent of the
subendocardial damage determines the degree of wallthickening that takes place in the affected segments. Mild
hypokinesis suggests only a small amount of myocardial
damage affecting a small part of the endocardium.

for the detection of myocardial perfusion and several

studies have demonstrated the clinical effectiveness of this
technique for the detection of CAD.9,10
Similar to stress echocardiography without myocardial
contrast, completely normal perfusion during myocardial
contrast stress echocardiography is very reassuring with
a <1% annual risk of adverse outcomes, while abnormal
perfusion accompanied by wall-motion abnormalities
identifies very high-risk patients with an approximately
15% annual event rate.9,10

ROLE IN ACUTE CORONARY

SYNDROMES

Figs 60.3A and B: Example of a wall motion abnormality in the mid-distal anteroseptum in a parasternal three-chamber view (arrows).
(A) Diastolic frame; (B) Systolic frame.

Chapter 60: Echocardiography in Ischemic Heart Disease

1293

Figs 60.4A and B: Example of a wall motion abnormality in the mid inferoseptum and inferior walls in a short-axis view (arrows).
(A) Diastolic frame, (B) Systolic frame.

Figs 60.5A and B: Example of a wall motion abnormality in the mid-distal lateral wall in an apical four-chamber view (arrows).
(A) Diastolic frame, (B) Systolic frame.

Severe hypokinesis is unusual in patients with small,

nontransmural infarctions. However, it could occur in
the presence of myocardial stunning overlying a small
infarction. It is not uncommon for segments adjacent to
the ischemic ones to become hypokinetic due to factors
such as tethering. Therefore, wall-motion abnormalities
following acute ischemia may be due to myocardial
necrosis, stunned or hibernating myocardium, or more
commonly, combinations of these factors.15
Echocardiography also provides useful prognostic
information for identification of patients at risk of future
cardiovascular events after an ACS. These data are based
on assessment of global LV systolic function.15

Global LV systolic function in patients with ACS

may be assessed through either wall-motion scoring or
calculation of global LV ejection fraction (LVEF). Wallmotion scoring analysis assigns a numeric value to the
degree of contractile dysfunction in each LV segment and
a wall motion score index can be derived from the sum
of individual segment scores divided by the number of
evaluated segments. The wall-motion score index been
shown to be an important prognostic indicator in patients
with CAD.
There are extensive data showing that LVEF is one of the
most powerful predictors of adverse outcomes including
mortality in patients with LV systolic dysfunction of any

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

cause including ischemic heart disease. LVEF is the single

most powerful predictor of mortality and the risk for lifethreatening ventricular arrhythmias after MI.
After resolution and management of an ACS, the
residual LVEF is important for treatment from both
medical and device therapies standpoint. Even after acute
treatment and clinical stabilization of heart failure, LVEF
has a strong and close relationship with survival in patients
with decreased LVEF.
The right coronary artery also commonly supplies the
right ventricle (RV), which may also be involved in the MI.
This has important implications in the acute management
of patients; so assessment of the RV in patients with ACS is
important to assess the degree of RV dysfunction.15
In patients with clinically suspected RV MI, echocardiography showing RV cavity enlargement and
impaired RV free wall motion confirms the diagnosis of
RV MI and helps exclude other possibilities for the clinical
presentation, such as pericardial tamponade, which may
present with the same clinical picture.
Echocardiography can be misleading for the diagnosis
of RV MI in patients with chronic pulmonary disease,
such as chronic obstructive lung disease or significant
pulmonary arterial hypertension, or acute pulmonary
disease such as pulmonary embolism.

MECHANICAL COMPLICATIONS OF
MYOCARDIAL INFARCTION
Echocardiography is the primary diagnostic tool for
detection of potentially life-threatening complications
of acute MI.17,18 Portability, immediate availability,
quick imaging, and the detailed information it provides
both on cardiac function and blood flow are critical in
the management of patients with known or suspected
mechanical complications of MI. By helping in diagnosis
and decision-making in these often critically ill patients,
echocardiography can be life-saving.17,18
After the diagnosis of a mechanical complication has
been made, echocardiography could also be used to guide the
intraoperative management (usually with transesophageal
echocardiography) and in assessing the outcome of
complicated surgeries and therapeutic procedures.17,18

Rupture of Left Ventricular Free Wall

Rupture of the LV free wall is the most common form
of myocardial rupture and the cause of death in

approximately 10% of the patients who die after an acute

MI.19,20 It occurs within the first 24 hours in about half the
patients and within a week in 85%. Any wallanterior,
lateral, or inferiorcan be involved. In up to 40% of cases,
ruptures have a subacute course with ongoing myocardial
tearing and bleeding into the pericardial space. A
pseudoaneurysm is a manifestation of a subacute tear
that is spontaneously sealed. Since; sudden death is often
a presentation of complete rupture of the LV free wall,
echocardiography has a bigger role in diagnosis of patients
with subacute free wall rupture.19,20
These patients often have pericardial effusions
secondary to hemopericardium, layered thrombus
in the pericardial space, or cardiac tamponade with
hemodynamic instability.19,20 A finding of a pericardial
effusion of 5 mm during diastole and layered thrombus
within the pericardial space are highly sensitive for the
detection of free wall rupture. The ruptured site is seldom
detected with two-dimensional (2D) echocardiography;
however, this may be possible with color-flow Doppler.19,20
Contrast echocardiography may be helpful in
establishing the diagnosis of free wall rupture in patients
with a subacute presentation.21 Passage of contrast agent
into the pericardial space after an intravenous injection
confirms a connection between the heart and the
pericardial space, while lack of contrast in the pericardial
space helps exclude a LV free wall rupture.21

Left Ventricular Pseudoaneurysm

An LV pseudoaneurysm is formed when a subacute tear
in the LV wall is spontaneously sealed and contained by
adherent pericardium or scar tissue22 (Figs 60.6A and B).
As opposed to a true LV aneurysm (Figs 60.6 and 60.7)
where the outer layer consists of thinned, necrotic, or
scarred myocardium, myocardium is not present in the
external layer of the pseudoaneurysm.22 A true aneurysm
and a pseudoaneurysm carry greatly different implications
since a true LV aneurysm rarely ruptures, while there is a
high risk of rupture with a pseudoaneurysm.22 Surgical
repair is indicated in most cases of pseudoaneurysm,
while it is not indicated in most cases of true LV aneurysm.
Therefore, echocardiography plays a crucial role not only
in the identification of a LV pseudoaneurysm, but also for
the confirmation of the absence of a true LV aneurysm.22
LV pseudoaneurysm is often detected when echocardiography is performed routinely post MI or for
indications of congestive heart failure or an embolic

Chapter 60: Echocardiography in Ischemic Heart Disease

1295

Figs 60.6A and B: Example of a left ventricular aneurysm (arrow

in Fig. A) and a left ventricular pseudoaneurysm (arrow in Fig. B).
The asterisk in the right image shows the narrow neck.

Fig. 60.7: Example of a left ventricular apical aneurysm (arrows).

event.23 A sharp discontinuity of the endocardial border at

the communication site of the pseudoaneurysm with the LV
cavity, a saccular or globular contour of the pseudoaneurysm, and a relatively narrow neck at the communication
site of the pseudoaneurysm with the LV cavity are
often seen on echocardiography23 (Figs 60.6A and B).
Careful examination and use of off-plane imaging may
sometimes be required for the identification and complete
characterization of the site of rupture. A thrombus may be
visualized within the pseudoaneurysm cavity.23
Color Doppler can also aid in the detection of LV
pseudoaneurysm and the characteristic finding is the
bidirectional flow between the cavities of the LV and
the pseudoaneurysm.23 A jet of bidirectional flow helps
distinguish a LV pseudoaneurysm from a pericardial
effusion or true aneurysm. The use of contrast agents
may also help in the diagnosis of LV pseudoaneurysm.24
Intravenous contrast agents can clearly demonstrate the
narrow neck and the communication between the cavities
of the LV and the pseudoaneurysm.24

the anterior two-third being supplied by branches of the

left anterior descending coronary artery and the posterior
third by the posterior descending artery of either the right
or circumflex coronary arteries.26 The timing of occurrence
of ventricular septal rupture varies depending on whether
the patient was reperfused or not. It occurs within the first
week after MI in patients who did not receive reperfusion
therapy, with peaks on the first day and on days 3, 4 and 5
after the MI. In patients treated with thrombolytic therapy,
most ruptures occur within the first 2 days.26
Septal ruptures can be discrete with a direct throughand-through communication at the same level on both
sides of the septum or be complex and irregular with
serpinginous tracts. In anterior MIs, septal ruptures are
usually apical and discrete, whereas in inferior MIs they
are more commonly in the basal inferoposterior septum
and more often complex.26
The ventricular septal defect can be visualized with 2D
echocardiography alone in approximately 50% of patients18
(Figs 60.8 to 60.10 and Movie clip 60.1). Multiple imaging
planes should be used and careful off-plane imaging may
be required to visualize the ventricular septal defect.
Nevertheless, 2D echocardiography may not detect small
defects and therefore, color Doppler imaging is crucial
when looking for a ventricular septal rupture. Typically
seen are turbulent transseptal flow and systolic flow
disturbance within the RV. In cases of complex defects,
color Doppler imaging may demonstrate multiple holes
or serpinginous tracts between the LV and the RV. Using

Ventricular Septal Rupture

Ventricular septal rupture most commonly occurs after
a transmural MI and is more frequent in acute anterior
infarction, extensive infarction, right ventricular infarction,
and in total occlusion of the infarct-related artery.25,26 This
complication of MI is rare (incidence of 0.20.3%), due
to the dual blood supply of the ventricular septum, with

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 60.8: Example of a postmyocardial infarction ventricular

septal rupture with flow across the defect (arrows).

Figs 60.9A and B: Example of a postmyocardial infarction ventricular septal rupture with flow across the defect (arrows).

Figs 60.10A and B: Example of a postmyocardial infarction ventricular septal rupture (asterisk); 2D image (A) and 3D image (B).

Chapter 60: Echocardiography in Ischemic Heart Disease

1297

Papillary muscle rupture is the rarest mechanical

complications of MI.17,18 The posteromedial papillary
muscle is the most affected, having its blood supply
only from the posterior descending coronary artery as
opposed to the anterolateral papillary muscle that has a
dual blood supply, both from the diagonal branch and the
left circumflex artery. Because of its single-vessel blood
supply, the posteromedial papillary muscle is 6 to 12 times
more vulnerable to rupture than the anterolateral papillary

muscle. The most frequent site of MI is, therefore, the

inferolateral wall.17,18 Often, the infarct size and expansion
are relatively small, and up to half of the patients have
single-vessel coronary disease, with many of them having
had their first MI. This complication generally occurs 2 to
7 days after an MI and is responsible for approximately 5%
of acute MI-associated mortality. Mortality is very high if
left untreated. About half die within the first day of rupture
and 90% within a week without surgical therapy. Therefore,
echocardiography is crucial for an early and accurate
diagnosis17,18 (Figs 60.11A and B and Movie clip 60.2).
Typical findings on 2D echocardiography include
a flail mitral valve leaflet and often a mobile mass seen
attached to the chordae and prolapsing into the left atrium
during systole, which is the head of the ruptured papillary
muscle. Partial rupture of a papillary muscle, defined as a
partial disconnection of the base of the papillary muscle
or rupture of one of several heads is seen more often than
complete rupture.17,18 In such cases, 2D echocardiography
shows a thin and excessively mobile papillary muscle. The
LV systolic function is often hyperdynamic.
Color Doppler imaging usually reveals severe MR, and
the direction of the regurgitation jet points to the affected
mitral leaflet: posteriorly directed regurgitation jet in flail
anterior leaflet and anteriorly directed jet in posterior
flail leaflet. If both leaflets are involved, the jet is centrally
directed. The severity of MR may be underestimated
with eccentric regurgitant jets, and a TEE may provide
incremental data in such cases.17,18

color Doppler imaging, the ventricular septal defect can be

characterized in terms of the location and the size of the
defect. The left to right jet seen on color Doppler is typically
pansystolic. In about half the patients, an aneurysm of
the ventricular septum can be seen protruding into the
RV. Similarly, in about half the patients, hyperkinetic LV
segments can be seen opposite the segment of the septal
defect. Decreased RV systolic function is not uncommon
and significant tricuspid regurgitation is frequently noted
secondary to high RV pressure.18,27
Transesophageal echocardiography (TEE) is frequently
performed to assess for mechanical complications in a
critically ill or acutely decompensated patient following
an MI.27 TEE may provide better imaging compared
to transthoracic echocardiography and may provide
improved visualization of the morphology, number,
location, and size of the ventricular septal defect(s), and
consequently better guide management decisions.27

Papillary Muscle Rupture

Figs 60.11A and B: Examples of papillary muscle rupture (arrows) in two different patients.

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Left Ventricular Aneurysm

A true LV aneurysm is a saccular protrusion or a bulge of
the LV wall during both systole and diastole, demonstrating
dyskinetic or akinetic motion28 (Fig. 60.6). As opposed to a
LV pseudoaneurysm where there is no myocardium in the
external layer of the pseudoaneurysm, the outer layer of a
true LV aneurysm consists of thinned, necrotic, or scarred
myocardium.17,18,28
LV aneurysms are frequent complications of acute MI,
and may be a cause of heart failure, LV thrombus formation
predisposing to the risks of systemic embolization, and
ventricular arrhythmias. LV aneurysms occur from
infarct expansion and adverse remodeling of the involved
segment, usually the apex, which is the thinnest segment
of the normal LV myocardium.28 Echocardiography has
excellent sensitivity and specificity for the detection of LV
aneurysms after MI.17,18,28
LV thrombus formation occurs almost exclusively in
the akinetic or dyskinetic apex with or without aneurysm
formation.17,18 Most thrombi are formed within 48 hours
to 1 week of the MI. Late formation of thrombus may be
seen in patients with severe congestive heart failure and
deteriorating LV systolic function. Chronic LV thrombus,
occurring 3 months or more after infarction, is seen with
a true LV aneurysm and nearly half of all patients with
a true LV aneurysm have a LV thrombus. In spite of its
high prevalence, chronic mural thrombi are likely to be
organized and therefore, rarely embolize. Acute thrombi
within an aneurysm are likely to be mobile and projecting

into the lumen, and therefore have a greater risk of

embolization17,18 (Figs 60.12A and B).
Echocardiography is fairly sensitive and specific for the
detection of LV thrombus.29 Reasons for false identification
of LV thrombus include trabeculations, aberrant bands,
papillary muscle, tumors, or noise and artifacts that are
mistaken for LV thrombus. Contrast agents improve
visualization of LV thrombi and the sensitivity and
specificity of echocardiography for the detection of LV
thrombus.24

ROLE OF ECHOCARDIOGRAPHY IN
CHRONIC ISCHEMIC CARDIOMYOPATHY
Chronic long-standing CAD, with or without infarction, may
result in progressive deterioration of LV function and other
accompanying pathology such as ischemic MR, increased left
atrial pressure, and so on. Echocardiography is very valuable
in the evaluation of chronic ischemic cardiomyopathy and
associated conditions30 (Figs 60.13A and B).
The assessment of LV systolic function and particularly
regional wall motion is primarily used for the diagnosis of
ischemic cardiomyopathy, determination of the severity,
and prognostication of patients with CAD.30
Ischemic cardiomyopathy is diagnosed on the basis
of LV systolic dysfunction, typically with regional wallmotion abnormalities. There are several possible reasons
for LV systolic dysfunction in patients with chronic
ischemic heart disease.

Figs 60.12A and B: Example of central mitral regurgitation due to ischemic cardiomyopathy [2D image (A) and color Doppler
image (B)].

Chapter 60: Echocardiography in Ischemic Heart Disease

1299

Figs 60.13A and B: Examples of a left ventricular apical thrombus (arrows) in two different patients, one without (A) and one
with contrast (B).

During an acute MI, the acute occlusion of blood flow

in an epicardial coronary artery leads to a rapid reduction
in resting myocardial blood flow and progressive death
of myocardium within the perfusion territory, starting
from the subendocardium and extending out to the
subepicardium in a wavefront phenomenon, as described
in 1979 by Reimer and Jennings.31 When blood flow is
restored, the myocardial necrosis is halted and some
of the acutely ischemic myocardium at risk is salvaged.
The extent of myocardium salvaged depends on the
promptness and efficacy of the revascularization therapy.
LV systolic function is affected soon after decrease in
resting myocardial blood flow and persists after the acute
MI.31
In the acute and subacute stages of a MI, the dead
myocardium is necrosed and causes segmental wallmotion abnormalities. The necrosed myocardium gradually thins and reorganizes into scar tissue or fibrosis over
weeks to months. Thus, patients with a prior MI and wallmotion abnormalities have myocardial fibrosis and scar
tissue leading to contractile dysfunction.
After an acute MI, wall motion may not return to
normal for several hours to weeks after blood flow has
been restored and the MI has completed. The LV systolic
dysfunction in an area of uninfarcted myocardium is
called myocardial stunning and is reversible.32
In patients with chronic CAD, increasing reduction
in myocardial blood flow due to the progression of
CAD may result in down-regulation of myocardial
contractile function with preserved metabolic activity.
This is also reversible and this area of the myocardium

is termed hibernating myocardium.33 When blood

flow to the hibernating myocardium is restored through
revascularization of the stenotic artery, contractile function
returns gradually. Systolic dysfunction in chronic CAD
may also occur due to myocardial stunning resulting from
repeated ischemic episodes occurring at rest (vasospasm)
or during increased myocardial oxygen demand such as
exercise.33
Therefore, wall-motion abnormalities in patients with
ischemic cardiomyopathy may be due to multiple reasonsmyocardial necrosis, myocardial fibrosis, stunned
or hibernating myocardium, and is likely due to combinations of these factors.
Determining the exact cause of wall-motion abnormalities in patients with ischemic cardiomyopathy carries
significant implications for management decisions. For
example, hibernating myocardium may warrant revascularization, as opposed to fibrotic and scarred myocardium,
which may not respond as well to revascularization.33
Determination of LV fibrosis on rest echocardiography
is based on wall thickness and echobrightness of the
segments. Segments thinner than 5 mm in diastole have
been traditionally felt to be nonviable; there has been
a long-standing thought that the likelihood of recovery
of contractility in such thinned segments is very low.34
However, recent data using cardiac magnetic resonance
imaging (CMR) have challenged this paradigm and
demonstrated that regional wall thinning could be
associated with limited scar burden in approximately 18%
of patients and associated with improved contractility and
resolution of wall thinning after revascularization.35

1300

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Myocardial contrast echocardiography permits the

assessment and quantification of myocardial blood
flow, both at rest and during hyperemia.24,36 The basic
pathophysiological principle behind this technique is
that viable myocardium has an intact microcirculation,
whereas infarcted, nonviable tissue loses this microvasculature. Myocardial contrast echocardiography allows
measurement of myocardial blood flow and blood volume,
and thus provides a direct evaluation of microvascular
integrity of the dysfunctional segments, and therefore,
viability and the likelihood of functional recovery after
revascularization.24
Contractile reserve and therefore viability can be
assessed using provocative stimuli such as exercise,
nitroglycerin, dipyridamole, postextrasystolic potentiation, and catecholamine (e.g. isoprenaline, adrenaline,
dopamine, or dobutamine) infusion in conjunction
with echocardiography. Of these options, dobutamine
stress echocardiography is the most accepted and
widely performed of these techniques. Its diagnostic and
prognostic value in the detection of myocardial viability
has been well established.8
Dobutamine at low doses of <10 g/kg/min demonstrates a relatively more potent inotropic effect than
chronotropic effect, allowing stimulation of myocardial
contractility before significant increases in heart rate,
and ischemia, occur. This is useful for the assessment
of contractile reserve. Dobutamine at higher doses
leads to both inotropic and chronotropic stimulation,
resulting in increased cardiac output, myocardial oxygen
consumption, and ischemia in the presence of significant
CAD.8,37
The protocol for assessment of viability by dobutamine
starts with two low-dose stages (5 and 10 g/kg/min), with
each stage lasting 3 minutes, and the dose is increased in
10 g/kg/min increments to a maximum dose of 50 g/kg/
min. Atropine may be given if the target heart rate is not
achieved. The test is terminated when the patient achieves
85% of the age-predicted maximum heart rate, or clinical
or echocardiographic evidence of ischemia occurs. Viable
segments will demonstrate improvement of contractility
at doses of 5 or 10 g/kg/min. Even when viability or
contractile reserve is noted at a low dose, higher doses
should be administered to observe a biphasic response,
wherein there is hypokinesis and decreased contractility at
high doses due to ischemia. The biphasic response has the
best predictive value for prediction of improvement in LV

function following revascularization, with lower chances

of functional recovery with revascularization when there
is either no contractile improvement at either low or high
dose, or sustained improvement with both low and high
doses of dobutamine.8

Ventricular Function in Chronic

Ischemic Heart Disease
Assessment of Systolic Function
Global LV systolic function in patients with ischemic cardiomyopathy may be assessed through either calculation
of global LVEF or wall-motion scoring.38
Left ventricular EF is most commonly computed
from echocardiography by using Simpsons method
of using systolic and diastolic volumes obtained from
biplane planimetry of paired orthogonal long-axis apical
views (Figs 60.14A and B). While quantitative techniques
generally are more accurate, grading based on visual
assessment is not inferior to other reference methods.
In most echocardiography laboratories, the LVEF is
determined by subjective visual assessment of a single
experienced reader.
There are extensive data showing that LVEF is one of the
most powerful predictors of adverse outcomes including
mortality in patients with LV systolic dysfunction of any
cause including ischemic heart disease. LVEF is the single
most powerful predictor of mortality and the risk for lifethreatening ventricular arrhythmias after MI.
Wall-motion scoring analysis assigns a numeric value
to the degree of contractile dysfunction in each LV segment
and a wall motion score index can be derived from the sum
of individual segment scores divided by the number of
evaluated segments.38
Doppler echocardiography can also provide useful
noninvasive estimation of left ventricular stroke volume
and cardiac output.

Assessment of Diastolic Function

By definition, all patients with systolic dysfunction have
varying degrees of diastolic dysfunction. Assessment of
diastolic function is more useful in patients with normal
systolic function but clinical features of heart failure or
those at risk for heart failure, in whom diastolic dysfunction
is clinically silent.39

Chapter 60: Echocardiography in Ischemic Heart Disease

1301

Figs 60.14A and B: Example of Simpsons method of quantification of LV ejection fraction. Diastolic frame (A) and systolic frame (B).
(LV: Left ventricle).

Details of assessment of diastolic function are beyond

the scope of this chapter and are discussed elsewhere in
this textbook. In patients with normal LVEFs, LV filling
pressures can be calculated using measures of diastolic
function. An E/e' ratio (E = peak early passive diastolic
transmitral flow recorded by pulsed wave Doppler imaging
and e' = peak mitral annular velocity recorded by tissue
Doppler imaging) of 8 identifies patients with normal LV
filling pressures, whereas a ratio 13 indicates increased
LV filling pressures.39 When the ratio is between 9 and 13,
other measurements are useful. An Ar A duration (Ar =
duration of the atrial reversal waveform on pulmonary
venous flow recorded by pulsed wave Doppler imaging
and A duration = duration of late diastolic wave across the
mitral valve recorded by pulsed wave Doppler imaging)
30 ms, a change in E/A ratio (E = peak early passive diastolic
transmitral flow recorded by pulsed wave Doppler imaging
and A = peak late diastolic transmitral flow recorded by
pulsed wave Doppler imaging) with the Valsalva maneuver
of 0.5, IVRT/TE e' [IVRT = isovolumetric relaxation
time, or the time interval between aortic valve closure
and mitral valve opening as recorded by pulsed wave
Doppler imaging and TE e' = the time interval between
the QRS complex and the onset of mitral E wave (TE) on
pulsed wave Doppler imaging subtracted from the time
interval between the QRS complex and e' onset on tissue
Doppler imaging] < 2, pulmonary artery systolic pressure
35 mm Hg (in the absence of pulmonary disease), and
maximal left atrial volume 34 mL/m2 are all indicative of
increased LV filling pressures.39

In patients with decreased LVEFs, the mitral inflow

pattern can be used to estimate filling pressures with
reasonable accuracy. Changes in the inflow pattern can
be used to track filling pressures in response to medical
therapy. In patients with impaired relaxation patterns
and peak E velocities < 50 cm/s, LV filling pressures are
usually normal. With restrictive filling, mean LA pressure
is increased.39 The use of additional Doppler parameters
is recommended in patients with E/A ratios of 1 to
< 2. A change in E/A ratio with the Valsalva maneuver of
0.5, a systolic peak velocity/diastolic peak velocity ratio
in pulmonary venous flow < 1, Ar A duration 30 ms, E/e
= (using average e) 15, IVRT/TE e' > 2, and pulmonary
artery systolic pressure 35 mm Hg (in the absence of
pulmonary disease) are suggestive of the presence of
increased filling pressures.39

NOVEL ECHOCARDIOGRAPHY
TECHNIQUES IN ISCHEMIC HEART
DISEASE
Three-Dimensional Echocardiography
While 3D echocardiography (3DE) has potential roles
in assessment of ischemia by stress testing, wallmotion abnormalities, left ventricular aneurysm, and
pseudoaneurysm, other complications of MI, the primary
application in patients with ischemic heart disease, is in
the quantification of cardiac chamber volumes, function,
and LV mass.4042

1302

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

LV chamber and mass quantification have been

studied extensively using 3DE and have been shown
to be more accurate and reproducible than 2D echocardiography techniques. A distinct advantage of 3DE
over 2D echocardiography is the ability to manipulate
the plane to align the true long axis and minor axis of the
LV, thereby avoiding foreshortening and oblique imaging
planes.4042 LV volume assessment by real time 3DE has
been demonstrated to be rapid, accurate, reproducible,
and superior to conventional 2D methods.4042 The LV
volumes can be segmented, which allows for regional LV
function assessment. LV volume and mass obtained by
real time 3DE correlate well with those obtained with CMR
or radionuclide imaging. Similarly, real time 3DE allows
improved accuracy of RV size and function assessment.
Use of real time 3DE in stress echocardiography
decreases imaging time (standard views can be obtained
with only 1 or 2 image acquisitions) with accuracy
comparable to 2D stress imaging.4042
Use of ultrasound contrast agents with 3DE potentially
improves quantification of LV volumes and EF. Another
evolving application of contrast 3DE is in the evaluation
of myocardial perfusion.43 Contrast 3DE offers the ability
to image the entire LV and to quantify the full extent of
hypoperfused myocardium.43
Thus, 3DE is complementary to 2D imaging and can be
used to improve assessment of cardiac function and morphology in patients with ischemic heart disease. Further
technological developments will lead to improvements
and will contribute to more practical applications of 3DE
in this group of patients.4043

Speckle-Tracking Echocardiography
Strain imaging potentially has incremental value in the
ability of echocardiography to detect and objectively
qualify ischemia and infarction.44,45 Speckle-tracking
echocardiography as a new noninvasive imaging technique can be helpful for an objective and quantitative
evaluation of global and regional myocardial function.
In contrast to tissue Doppler imaging, it works almost
independently from the angle of insonation and from
cardiac translational movements. This technique is
potentially a better tool to detect myocardial ischemic
segments and also differentiate active contraction from
passive motion such as passive expansion, and recoil and
tethering from adjacent segments.4446

Myocardial Ischemia
The subendocardium is the most vulnerable area to the
effects of hypoperfusion and ischemia; so, LV longitudinal
mechanics (mainly generated by subendocardium) as the
most sensitive parameter may be attenuated in patients
with CAD even at rest. Circumferential strain and twist may
remain normal or show exaggerated compensation for
preserving LV systolic performance. A lower longitudinal
strain value in asymptomatic patients without wallmotion abnormalities can be a strong predictor of stable
ischemic cardiomyopathy. Recently, postsystolic index
has been proposed as an important quantitative marker
for analysis of the ischemic myocardium. LV endocardial
area change ratio by 3D speckle tracking coupled with both
longitudinal and circumferential strain have proposed
to assess induced acute myocardial ischemia in a sheep
model.46

Myocardial Infarction
Longitudinal strains are significantly reduced in patients
with MI, proportionately within the area of infarction,
and correlate closely with peak infarct mass and EF. While
longitudinal strain has been used to detect MI, radial and
circumferential strain values have been used to distinguish
nontransmural infarction from transmural infarction47
(Figs 60.15 and 60.16). The pattern of postsystolic
shortening peak strain after aortic valve closure has been
also reported helpful. In some studies, longitudinal strain
was related to peak levels of cardiac troponin T and the
LV infarct size. It has also been shown that longitudinal
strain correlates with the global and regional extent
(transmurality) of scar tissue as evaluated by contrastenhanced MRI.47

Reperfusion
Longitudinal strain (measured immediately after reperfusion therapy) has been suggested as an excellent
predictor of LV remodeling and adverse events, such as
congestive heart failure and death.45 When combined
with wall-motion scoring, strain rate imaging offered
incremental value over wall-motion scoring alone for
prediction of functional recovery after revascularization.48

Myocardial Viability
Strain imaging as an adjunct to low-dose dobutamine
stress echocardiography has been suggested to assess

Chapter 60: Echocardiography in Ischemic Heart Disease

1303

Figs 60.15A and B: Polar map derived from speckle-tracking echocardiography showing longitudinal strain in different segments of
two cases, (A) Normal subject (homogeneous yellow) and (B) A patient with a LAD lesion with lower (orange) and positive strain (blue)
in the anteroseptal and septal segments.

Figs 60.16A and B: Speckle-tracking echocardiography demonstrating segmental longitudinal strain in a normal subject (A) and in a
patient with hypokinesis and dyskinesis due to a LAD lesion (B).

myocardial viability.49 In addition, a favorable correlation

between resting longitudinal LV strain and the extent
of scarring by CMR imaging has been reported. Both
myocardial velocity and deformation parameters have
been analyzed at rest and during dobutamine stimulation
to define the functional reserve.49
Although speckle tracking is regarded as a revolution
in echocardiography, prospective clinical trials for the
validation of this technique in large populations are still
lacking. To date, strain rate has not replaced conventional
gray-scale imaging in the assessment of regional LV
function and the implementation of these new indices into
routine clinical practice will need additional clinical and
large-scale studies.44

FUTURE
Despite the emergence of competing imaging modalities,
echocardiography remains and will continue to be the
primary imaging modality for patients with ischemic
heart disease due to its portability, ease of use,
safety, and technological advancements in terms of
techniques and instrumentation such as tissue Doppler
imaging, 3DE, speckle-tracking imaging, and contrast
echocardiography. Future advances in technology
and increased clinical data will lead to overcoming of
current limitations and further increase the value of
echocardiography in the everyday clinical use for patients
with ischemic heart disease.

1304

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

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CHAPTER 61
Stress Echocardiography
Azhar Supariwala, Siu-Sun Yao, Farooq A Chaudhry

Snapshot

Fundamentals of Stress Echocardiography

Types of Stress Echocardiography

INTRODUCTION
Stress echocardiography was first introduced by Wann
and coworkers with supine bicycle exercise,1 but the
application was limited until the development of digital
acquisition.2,3 Stress echocardiography is now routinely
used in the diagnosis, risk stratification, and prognosis of
patients with known or suspected coronary artery disease
(CAD).4,5 In recent years, there have been further advances
in the application of stress echocardiography, including
the evaluation of valvular and anatomic abnormalities
of the heart, hemodynamics, myocardial perfusion,
quantitation of wall motion abnormality with speckle
tracking, and three-dimensional (3D) imaging.

FUNDAMENTALS OF STRESS
ECHOCARDIOGRAPHY
The most common application of stress echocardiography
is the detection of flow limiting stenosis. The use of
stress echocardiography to diagnose flow-limiting CAD
is based on a sequence of events known as the ischemic

Interpretation of Stress Echocardiography

Stress Echocardiography: Future Directions

cascade6 (Fig. 61.1). Normal coronary arteries adapt

to stress by coronary vasodilatation (coronary flow
reserve) to maintain flow to meet the increased oxygen
demand. In the presence of a flow-limiting coronary
stenosis, coronary flow reserve is impaired resulting in
ischemic cascade including wall motion abnormality.
Abnormalities of regional relaxation of the left ventricle
precede systolic wall motion abnormalities. The
segmental wall motion abnormality is characterized
echocardiographically as a reduction in systolic
thickening and endocardial excu
rsion. The severity
and extent of wall motion abnormality depends on
multiple factors including severity of stenosis, level of
stress, coronary flow reserve, and collateral circulation.
Electrocardiographic changes and symptoms may or
may not occur until late in the ischemic cascade and
may not even be present in patients with mild ischemia.
Endothelial dysfunction secondary to hypertensive
heart disease and diabetes mellitus can also cause wall
motion abnormalities during stress in the absence of
flow-limiting epicardial coronary stenosis.7,8

Chapter 61: Stress Echocardiography

1307

echocardiography is that it is more physiologic and higher

peak heart rates are achieved. The disadvantage is that
images are acquired postexercise as compared to bicycle
stress where images are obtained at peak stress. However,
bicycle stress achieves lower peak heart rate, and image
acquisition is more difficult during peak exercise.

Dobutamine Stress Echocardiography

Fig. 61.1: Ischemic cascade: the sequence of events that occur

after a flow-limiting stenosis. The decrease in flow produces
biochemical metabolic abnormalities first, followed by detectable
perfusion defects by positron emission tomography (PET) or
single photon emission computed tomography (SPECT), followed
by perfusion, diastolic, and systolic abnormalities that could be
detected by stress echocardiography with microbubble and
speckle strain, eventually leading to ischemic electrocardiographic
changes and anginal symptoms.

TYPES OF STRESS
ECHOCARDIOGRAPHY
There are various ways to induce stress such as dynamic
exercise (treadmill or bicycle), pharmacologic stress
(dobutamine, dipyridamole, and adenosine), and pacing
stress.9 Hand grip and leg raise are commonly employed
as an adjunct to the above-mentioned stress modes to
further augment heart rate.10

Exercise Echocardiography
Treadmill exercise using the Bruce protocol is the most
widely used stress modality in the United States. Supine
or semi-supine bicycle stress is widely used in Europe
and in some centers in the United States.11 The images in
treadmill exercise are acquired immediately postexercise
(Figs 61.2A and B). All images are acquired within 3060
seconds, and the entire sequence is repeated to acquire a
second set of images.
Reasons for termination of stress test are symptoms of
typical angina, marked dyspnea, significant ST segment
depression or elevation, sustained arrhythmias, or hemo
dynamic compromise (hypotension or systolic blood
pressure > 220 mm Hg). The advantage of exercise stress

In patients who are unable to exercise or attain adequate

levels of workload, pharmacological stress is preferred.
With stress echocardiography, dobutamine is preferred
as it is more likely to provoke ischemia as compared to
vasodilator agents such as adenosine or dipyridamole.
Dobutamine is a sympathomimetic drug that has
both b-1 and b-2 adrenergic and a-1 activity. The affinity
of dobutamine for b-1 cardiac muscle receptors results
in positive inotropic, and, to a lesser extent, positive
chronotropic response. These actions are dose-dependent.
At lower doses, the inotropic response prevails, and
at higher doses, chronotropic activity is predominant.
Dobutamine induces myocardial ischemia in patients
with flow-limiting coronary artery stenosis by increasing
left ventricular contractility, heart rate, wall stress, and
therefore, myocardial oxygen demand.12
Dobutamine is administered as an infusion starting
at 5 or 10 g/kg/min and is increased by 510 g/kg every
3 minutes until a maximal dose of 4050 g/kg/min or until
85% of the age-predicted maximum heart rate is achieved.
Atropine is used in ~30% of patients in conjunction with
dobutamine in 0.25 mg increments up to a maximum
dose of 2 mg. Low-dose images are obtained at 5 or 10
g/kg/min or when increased contractility is observed.
Peak stress images are obtained after 85% of maximal
predicted heart rate is achieved. The images are displayed
as four digitized cine loops that show rest, low dose, peak
dose, and recovery.

Vasodilator Stress Echocardiography

Major coronary vasodilators used for stress echocardi
ography are adenosine and dipyridamole. Adenosine
is an endogenously produced substance with potent
vasodilator properties. Adenosine induces blood flow
heterogeneity by stimulating A2 receptor inducing vasodi
latation. Dipyridamole exerts its effects indirectly by
reducing the reuptake of adenosine. In the setting of a
critical coronary artery stenosis, the flow may increase
in the epicardium but falls in the subendocardium distal

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 61.2A and B: The equipment and set-up for performing a treadmill exercise stress echocardiography.

6 minutes after initiation of adenosine. Recovery images

for both dipyridamole and adenosine are obtained at
10- and 12-minute intervals from the start of infusion.
Adjunctive atropine and handgrip and leg lift exercise may
be used to further increase myocardial demand and induce
ischemia.14 The advantage of the vasodilator stress test is
that if an adverse event occurs, it resolves after termination
of infusion or after the administration of aminophylline.
The disadvantages are that it is contraindicated in patients
with severe chronic obstructive lung disease and those
taking theophylline preparations or who have recently
ingested caffeine. Vasodilators are also less likely to
precipitate ischemia compared to dobutamine.
Fig. 61.3: This is an example of a technically difficult dobutamine
stress echocardiogram. The noncontrast study had very poor
endocardial definition. Administration of contrast improved endocardial delineation. The rest images show akinesis in the apex
and apical anterior wall. The low-dose images show increased
contractility throughout the myocardium except in the apex and
apical anterior wall. At peak stress, no new wall motion abnormalities were observed with persistent akinesis, which is consistent with nonviable tissue. This study is consistent with scar in the
apex and apical anterior wall without any significant ischemia.
(LV: Left ventricle; RV: Right ventricle). (Movie clip 61.1).

to the flow-limiting stenosis, resulting in ischemia and

subsequent wall motion abnormalities.13 Dipyridamole
is administered intravenously over 6 minutes at a rate of
0.14 mg/kg/min. Peak stress images are obtained at 4- and
6-minute intervals from the start of infusion. Adenosine
is infused starting at 140 g/kg/min over a 4- to 6-minute
period. Peak stress images are obtained at 3 minutes and

Contrast Echocardiography
As many as 2030% of patients referred for stress
imaging have suboptimal visualization of the left
ventricular endocardial border echocardiographically.15
Echocardiography contrast agents significantly improve
the bloodendocardial border visualization at rest and
stress.16 Contrast-enhanced echocardiography improves
images in patients with poor acoustic windows and thus
improves inter- and intraobserver variability and diagn
ostic accuracy.15 (Fig. 61.3 and Movie clip 61.1)

Safety of Stress Echocardiography

Absolute contraindications for stress echocardiography
are: (a) unstable angina, (b) decompensated congestive
heart failure, (c) uncontrolled hypertension (> 210/110
mm Hg), (d) uncontrolled cardiac arrhythmias (causing
symptoms or hemodynamic compromise), and (e) severe

Chapter 61: Stress Echocardiography

Fig. 61.4: A baseline echocardiogram showing aortic dissection in

a patient hospitalized for chest pain. The stress echocardiogram
was cancelled and the patient underwent a computed tomography
of the chest, which was consistent with ascending aortic dissection
(Type A). The patient underwent subsequent surgery for repair
of the aortic dissection. (LA: Left atrium; LV: Left ventricle)
(Movie clip 61.2).

symptomatic aortic stenosis. The relative safety of stress

echocardiography is similar to other forms of stress testing
modalities. In a study of 85,000 patients, the event rate
was 1 in 6,574 for exercise, 1 in 557 for dobutamine, and
1 in 1,294 for dipyridamole.17,18 Minor arrhythmias such
as premature ventricular contractions, atrial arrhythmia,
nonsustained ventricular tachycardia (3%) are common
with dobutamine stress and are not necessarily indicative
of CAD. Adenosine is well tolerated in the majority
(> 80%) of patients. In the Adenoscan Multicenter Trial, the
infusion was prematurely terminated in < 0.01% of patients,
only 0.8% received aminophylline, and no sustained
episodes of atrioventricular block were observed.19

INTERPRETATION OF STRESS
ECHOCARDIOGRAPHY
Among expert readers, interpretation of stress echocar
diography has been highly reproducible.20 Interobserver
variability is mostly due to suboptimal image quality and
subtle degrees of wall motion abnormality.21 The resting
echocardiogram may reveal important diagnosis such as
aortic dissection, critical aortic stenosis, and obstructive
hypertrophic cardiomyopathy (HCM) (Fig. 61.4 and Movie
clip 61.2).
A normal response during either exercise or pharma
cological stress echocardiogram is an increase in wall
thickening and endocardial excursion with decrease in left

1309

Fig. 61.5: Interpretation of response to stress echocardiography

for each of the 17 segments. During exercise stress, the thickening and excursion of segments at rest, peak, and recovery are
compared. During dobutamine stress, segments are compared
at rest, low dose, peak dose, and recovery. Row 1/biphasic: describes an ischemic response in both exercise and dobutamine.
Row 2/monophasic: describes a normal response to either exercise or dobutamine stress with inotropic contractile reserve. Row
3/nonphasic: describes scar or nonviable myocardium.

ventricular end-systolic volume. Figure 61.5 describes the

various wall motion responses and their interpretation. The
left ventricle is divided into a 17-segment model at rest and
stress. The 17-segment model with the distribution of the
respective coronary arteries is shown in Figure 61.6. Each
segment is scored as follows: 1 = normal, 2 = hypokinesis
(reduced wall thickening and excursion), 3 = akinesis (no
wall thickening and excursion), 4 = dyskinesis (paradoxical
motion away from the center of the left ventricle during
systole) and 5 = Aneurysmal.22 It is important to remember
that the normal myocardial response postexercise is
thickening of the myocardium, marked excursion of the
endocardium, and almost complete obliteration of the left
ventricular cavity.23 With dobutamine stress in patients
with rest wall motion abnormality, ischemia is defined as
improvement of wall motion at low dose and deterioration
of wall motion at peak dose (biphasic response). Therefore,
an abnormal (ischemic) response to stress is defined as:
(a) a deterioration in left ventricle wall segment thickening
and excursion during stress (increase in wall motion of
1 grade) or (b) a biphasic response with dobutamine
stress during which wall motion abnormality improves
at baseline (viable myocardium) and then deteriorates
at peak stress (ischemia). Furthermore, an abnormal
increase in end-systolic left ventricle volume [transient
ischemic dilatation (TID)] is associated with multivessel
CAD.24,25

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 61.6: The American Society of Echocardiography (ASE) has divided the left ventricle into 17 segments. The schematic
demonstrates the relationship between the coronary artery distribution and the corresponding ASE 17 left ventricular segments.
Analyses of stress echocardiogram should provide the quantitative result according to this model. The four standard echocardiography
views provide evaluation of territories of each of the three main coronary arteries. (Ao: Aorta valve; LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle). Source: Reproduced with permission from Vidhun R Echocardiography pocketcard
set, second edition. Brm Bruckmeier Publishing; 2010.

wall segments divided by the number of visualized

segments. A normal study has a WMSI of 1.0 at both rest
and stress (Fig. 61.7 and Movie clip 61.3) A WMSI of >1.0 at
rest and stress is abnormal (Fig. 61.8 and Movie clip 61.4).

Diagnostic Accuracy to Detect CAD

Fig. 61.7: An example of a normal treadmill exercise echocardiogram demonstrating a hyperdynamic response to stress. The
standard format to display exercise stress echocardiography
images. It demonstrates side-by-side rest-stress images. The
heart rate and time of image acquisition postexercise are displayed
for each image. The resting study is on the left and postexercise
study is on the right. The patient had 1-mm ST depressions, which
are likely false-positive ECG changes (Movie clip 61.3). (ECG:
Electrocardiogram; LV: Left ventricle; RV: Right ventricle).

A semiquantitative approach for image interpretation

is done by calculation of the wall motion score index
(WMSI). WMSI is a cumulative sum score of 17 left ventricle

Using angiography as the gold standard for comparison

for a > 50% stenosis by quantitative coronary angiography
and 70% visually, the overall sensitivity for stress
echocardiography is 7585% and specificity is 8090%
(Table 61.1).2629 Sensitivity and specificity are comparable
in both men and women and among different stress
modalities (exercise, dobutamine, and vasodilator).29
Studies comparing the accuracy of single photon emission
computed tomography myocardial perfusion imaging
(SPECT-MPI) and stress echocardiography in the same
patient population have shown similar sensitivities for the
detection of CAD, with stress echocardiography having a
higher specificity (Table 61.2).30
Factors that can cause false-positive or false-negative
results are listed in Table 61.3.

Risk Stratification and Prognosis

The goal of prognostic testing is based on the premise
that patients identified as being at highest risk for adverse

Chapter 61: Stress Echocardiography

1311

Fig. 61.8: This treadmill exercise echocardiogram demonstrates ischemia. The resting study is normal while the
postexercise images reveal anteroapical wall motion abnormalities. The distribution is consistent with obstructive
coronary artery disease involving left anterior descending artery. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle) (Movie clip 61.4).

outcomes can be intervened upon to alter the natural

history of their disease process, thereby reducing future
cardiac risk.
Stress echocardiography results can risk stratify
and prognosticate patients into a low-risk group (< 1%),
intermediate (15%), and high (> 5%) risk for major cardiac
mortality.31 Table 61.4 summarizes the different variables
important in identifying risk and predicting prognosis.
Patients with mild-to-moderate wall motion abnormalities
(peak WMSI = 1.11.7) have an intermediate risk of cardiac
events and if stable, may be initially managed medically.32
Patients with peak WMSI > 1.7 and especially those
with ejection fraction 45% are at high risk of cardiac
events (Fig. 61.9). Such patients should be appropriately
referred to catheterization and consideration of coronary
revascularization in order to decrease future cardio
vascular risk.33

Extent and Severity of Myocardial Wall

Motion Abnormality as Predictors
of Prognosis
The prognostic value of stress echocardiography is
based on its ability to quantify the severity and extent of
jeopardized (ischemic) myocardium with exercise or
pharmacologic stress. The ischemic extent reflects the areas
of myocardium (number of segments) that are abnormal,
and maximal severity reflects the degree or magnitude of

wall motion abnormalities in a designated segment, both

being quantified at peak stress. Ischemic extent reflects the
number of new stress-induced wall motion abnormalities
and corresponds roughly to the number of stenosed
coronary arteries. Maximal severity reflects the magnitude
of ischemia within a designated myocardial segment and
reflects the severity of a subtending coronary stenosis
within a given coronary artery territory. Estimation of both
ischemic extent and maximal severity variables by stress
echocardiography provides a functional depiction of a
noninvasive coronary angiogram and accurate prognostic
assessment of the amount of jeopardized myocardium.
The extent and severity of wall motion abnormalities
by stress echocardiography are independent and cumu
lative predictors of prognosis.34 Thus, prognostic risk
stratification by stress echocardiography is both a separate
and combined function of the extent and severity of
wall motion abnormalities (Fig. 61.10). This proposed
model extends the use of stress echocardiography from a
simple diagnostic tool, toward establishing its utilization
in precise risk stratification, prognosis, and direction of
patient management decisions.

Prediction of Myocardial Infarction Versus

Cardiac Death by Stress Echocardiography
Identifying patients at high risk for ischemic events versus
high risk for sudden cardiac death is important in deciding

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 61.1: Studies with Diagnostic Accuracy of Treadmill, Dobutamine, and Vasodilator Stress Echocardiography Compared to
Coronary Angiographya

Studies

Study Size

Peteiro et al. 201258

116 patients

Sensitivity (%)

NPV(%)

Specificity (%)

PPV (%)

Accuracy (%)

Peak supine bicycle SE

84

63

77

Peak treadmill exercise SE

75

80

77

Post treadmill exercise SE

60

78

66

Jang et al. 201159

1,287 Treadmill Exercise SE

(Korean population)

68

Aggeli et al. 201160

60 patients dobutamine stress

61

78

83

72

Two-dimensional

80

82

Real time, three-dimensional

with perfusion

82

64

11 studies (701 hypertensive

patients)

77

89

Mahajan et al. 201027 23 studies (15 SPECT, 14 SE)

94

40

With prior myocardial infarction

83

81

Without prior myocardial

infarction

74

85

Patients without RWMA

76

88

Patients with RWMA

82

81

Dipyridamole

85

89

87

Dobutamine

86

86

84

Dipyridamole

72

82

77

Treadmill

79

92

80

Meta-analyses:
Gargiulo et al. 201161

Geleijnse et al.
200962

Picano et al. 200829

de Albuquerque
Fonseca et al. 200163

62 studies (6,881 patients)

5 studies (435 patients)

8 studies (533 patients)

Obstructive disease considered > 50% stenosis on coronary angiography.

(NPV: Negative predictive value; PPV: Positive predictive value; RWMA: Rest wall motion abnormality; SE: Stress echocardiography;
SPECT: Single photon emission positron computed tomography).
a

on the optimal clinical management strategy. Patients

at high risk for ischemic events but low risk for cardiac
death benefit more from medical therapy, but patients at
intermediate to high risk of cardiac death benefit more
from early revascularization.35 Stress echocardiography
is an effective modality at differential risk stratification
of patients for the outcome-specific end points of cardiac

death and nonfatal myocardial infarction.33 Patients with

ejection fraction < 30% are at high risk of cardiac death
(> 4%/year), and these patients should be aggressively
managed with optimal medical therapy, consideration of
revascularization, device, and cardiac resynchronization
therapy (Fig. 61.11). In patients with ejection fraction
30%, peak WMSI can further risk stratify into a low-

Chapter 61: Stress Echocardiography

1313

Table 61.2: Comparative Accuracy of Stress Echocardiography Versus Nuclear SPECT Imaging

Echocardiography
Studies

Study size

Sensitivity (%)

Specificity (%)

SPECT
Sensitivity (%) Specificity (%)

Meta-analyses:
Heijenbrok-Kal et al.28

351 patients

79

87

88

73

Imran et al. 2003a 64

10 studies (651 patients)

70

90

88

67

Fleischmann et al.30

EXSE 24 studies (2,637 patients);

EXSPECT 27 studies (3,237 patients)

85

77

87

64

OKeefe et al.65

SE 12 studies (913 patients);

SPECT 12 studies (2,626 patients)

81

89

90

72

Pharmacologic SE 14 studies
(1,049 patients);
Pharmacologic SPECT 14 studies

81

83

87a

75a

89b

83b

Dipyridamole stress echocardiography versus SPECT.

Adenosine SPECT. (EXSE: Exercise stress echocardiography; EXSPECT: Exercise SPECT; SE: Stress echocardiography; SPECT: Single
photon emission computed tomography).
a

Table 61.3: Factors that Affect Accuracy of Stress Echocardiography for Detecting Hemodynamic Obstructive Coronary Artery
Disease Compared with Coronary Angiography

False Positive

False Negative

Hypertensive response to stress

Submaximal stress (< 85% maximal predicted heart rate)

Microvascular disease: e.g. diabetes, left ventricular

hypertrophy, syndrome X, hypertrophic cardiomyopathy

Poor image quality

Cardiomyopathies

Delayed poststress image acquisition

Paradoxical septal motion, e.g. postcardiac surgery, left bundle

branch block

Very mild ischemia, circumflex coronary stenosis, branch

or distal stenosis

Coronary spasm, endothelial dysfunction

Good coronary reserve (collateral circulation), potent

endothelial function

Localized basal inferior wall motion abnormalities

Antianginal drug therapy during testing (calcium channel

blockers, b-blockers, nitrates)

Fig. 61.9: Cardiac event rate per year as a function of wall motion
score index. Worse cardiac event rate is observed with higher
peak wall motion score index.
Source: Reprinted with permission of Elsevier from Yao S, Qureshi
E, Sherrid MV, Chaudhry FA. Practical applications in stress
echocardiography: risk stratification and prognosis in patients
with known or suspected ischemic heart disease. Journal of the
American College of Cardiology. 2003;42(6):1084-90.

Fig. 61.10: Cumulative effect of ischemic extent and maximal

severity (jeopardized myocardium) of wall motion abnormalities
on event rate per year. The event rate increases as a curvilinear
function of both extent and severity combined.
Source: Reprinted with permission of Elsevier from Yao S, Qureshi
E, Syed A, Chaudhry FA. Novel stress echocardiographic model
incorporating the extent and severity of wall motion abnormality for
risk stratification and prognosis. American Journal of Cardiology.
2004;94(6):715-9.

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 61.4: Stress Echocardiography Predictors of Risk

Very Low Risk < 0.5 to 1%/year Low Risk < 2%/year Intermediate Risk
2% to 4%/year

High Risk (> 4%/year)

Incremental Risk/Other
Independent Predictors

Normal exercise stress

echocardiography

Normal pharmaco- pWMSI 1.01.7

logical stress test
Resting ejection
fraction > 50%

Extensive resting wall

motion abnormalities
(47 segments)

Age
Male gender
Prior history of CHF or MI

> 6 METs achieved

Submaximal stress
test (< 85% MPHR)

Single vessel
disease
(LCx or RCA)

Resting ejection
fraction < 45%

> 3 CAD risk factors

Left atrial size

Off anti-ischemic
therapy

Extensive ischemia
(45 segments)

Multivessel coronary artery

disease
Transient ischemic left
ventricular cavity dilatation

pWMSI > 1.7

Ischemia induced at
lower workload

Abnormal Right ventricular

wall motion
Limited exercise capacity.
Inability to exercise

On anti-ischemic
therapy

Ischemic ECG or symptoms

on stress
High pretest likelihood

Achieved > 85% MPHR

(CAD: Coronary artery disease; CHF: Congestive heart failure; ECG: Electrocardiogram; LCx: Left circumflex; METs: Metabolic equivalent of tasks; MI: Myocardial infarction; MPHR: Maximal predicted heart rate; pWMSI: Peak wall motion score index; RCA: Right
coronary artery).
Source: Modified with permission of Elsevier from Pellikka PA, Nagueh SF, Elhendy AA, et al. Echocardiography recommendations
for performance, interpretation and application of stress echocardiography. Journal of American Society of Echocardiography.
2007;20(9):1021-41.

Fig. 61.11: Schematic for the risk stratification of patients undergoing stress echocardiography. (CAD: Coronary artery disease; CD:
Cardiac death; CRT: Cardiac re-synchronization therapy; EF: Ejection fraction; WMSI: Wall motion score index).
Source: Reprinted with permission of Elsevier from Bangalore S, Yao S, Chaudhry FA. Prediction of myocardial infarction versus cardiac
death by stress echocardiography. Journal of the American Society of Echocardiography. 2009;22(3):261-7).

Chapter 61: Stress Echocardiography

1315

Fig. 61.12: This figure demonstrates an abnormal left ventricular

volume response to stress. The resting study is normal.
Postexercise, there is evidence of anteroseptal, apical, and lateral
ischemia, resulting in left ventricular dilatation or transient ischemic
dilatation (TID). Cardiac catheterization was consistent with
severe multivessel coronary artery disease. (LV: Left ventricle).
(Movie clip 61.5).

Fig. 61.13: Cardiac event rate per year as a function of stress

echocardiogram results and transient ischemic dilatation (TID). A
15-fold increase in cardiac event rate is observed with TID.
Source: Reprinted with permission of Elsevier from Yao S, Shah
A, Bangalore S, Chaudhry FA. Transient ischemic left ventricular
cavity dilation is a significant predictor of severe and extensive
coronary artery disease and adverse outcome in patients undergoing stress echocardiography. Journal of the American Society of
Echocardiography. 2007;20(4):352-8).

risk group (pWMSI = 1.0; cardiac death rate < 1.0%/year)

managed with risk factor modification alone, a low-inter
mediate risk group (pWMSI = 1.11.7; cardiac death rate
1.02.5%/year) managed with optimal medical therapy
and consideration of revascularization for symptom relief
only, and a high-intermediate risk group (WMSI >1.7;
cardiac death rate 2.54%/year) managed with optimal
medical therapy and consideration of revascularization
(to decrease future cardiac risk).36 Table 61.5 summarizes
the list of studies reporting the prognostic value of stress
echocardiography.

WMSI, and a worse prognosis than patients without TID

(Fig. 61.13). TID during stress echocardiography is a
sensitive marker of severe and extensive angiographic
CAD and is associated with a very high risk of cardiac
events (19.7%/year event rate).25 These patients should be
referred for consideration of catheterization and coronary
revascularization as the best means to modify and reduce
future cardiac risk.24,25

Transient Ischemic Left Ventricular

Cavity Dilatation
Transient ischemic left ventricular cavity dilatation (TID)
signifies an increase in left ventricular cavity size after a
wall motion abnormality induced with exercise or dobu
tamine stress. Plausible clinical explanations for TID
include: subendocardial ischemia, systolic left ventricular
dysfunction, and actual physical left ventricular dilatation
in end-diastole (Fig. 61.12 and Movie clip 61.5). TID has
a high sensitivity (100%) and moderate specificity (54%)
for the detection of severe and extensive angiographic
CAD.25 Patients with TID have a greater extent and severity
of stress-induced wall motion abnormality, higher peak

Role of Right Ventricular Wall Motion

Abnormalities in Risk Stratification
and Prognosis
The right ventricle is often termed the forgotten cham
ber. Evidence from clinical studies emphasizes the
importance of evaluating right ventricular function during
routine echocardiography. Right ventricular function has
prognostic implications in patients with CAD and heart
failure.37
With stress echocardiography, abnormal right vent
ricle function (ischemia or infarction) can further risk
stratify and add prognostic value to left ventricular
functional parameters.38 Patients with both an abnormal
right ventricle and left ventricle have a worse prognosis.
Right ventricular wall motion analysis should be routinely
assessed in patients referred for stress echocardiography
for more effective and complete risk stratification.

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 61.5: Studies Reporting the Prognostic Value of Stress Echocardiography

Studies

No. of Patients

Follow-Up Duration Normal

Abnormal

Wever Pinzon et al.66

311 HIV patients

2.9 1.9 years

0.6%/year MI/CD

11.8%/year MI/CD

Bangalore et al.

1,002 LVH patients

2.6 1.1 years

1.1%/year MI/CD

4.9%/year MI/CD

Wake et al.68

890 patients
(contrast-enhanced DSE)

30 17 months

88% 2-year (men)

74% 2-year (men)

91% 2-year (women)

80% 2-year
(women)

67

Metz et al.69

Meta-analyses
3,021 patients

33 months

0.54%/year-MI/CD

Chaowalit et al.70

3,014 patients (DSE)

Median 6.3 years

93% 5-year MI/

revascularization

78% 5-year all-cause mortality

DAndrea et al.

607 patients (supine bicycle SE) Mean 46 months

96% 5-year

84% 5-year

Biagini et al.

3,381 patients (DSE)

2.5%/year (men) MI/CD

5.9%/year (men)

1.2%/year (women) MI/CD

4.6%/year
(women)

71

72

Sozzi et al.73

401 patients (DSE)

7 3.4 years

5 1.7 years

0.8%/year (first 3 years)

MI/CD
1.7%/year (between 4th and
6th year of follow-up)

Yao et al.31

1,500 patients

2.7 1.0 years

0.9%/year

4.2%/year overall;
1.4%/year TME,
4.7% DSE

Hoque et al.74

206 patients EXSE

8.8 2.9 years

0.8%/year all-cause mortality

< 0.5%/year CD

Moderate to large
ischemia: 5.5%/
year CD

Sicari et al.75

7,333 patients Dipy or DSE

2.6 3 years

0.9%/year or
92% at ~16 years

71.2% at ~16 years

CD

Elhendy et al.76

4,347 patients EXSE

Median 3 years

97.5% at 5 years (< 1%/year)

89.7% at 5 years
CD

Marwick et al.77,78

1,581 patients DSE

5,375 patients EXSE

3.8 years 1.9 years

5.5 1.9 years

1.2%/year
1.0%/year

24%/year CD

Krivokapich et al.79

558 patients DSE

12 months

10% all cardiac events

3% hard events

34% all cardiac

events
10% hard events

Poldermans et al.80

1,734 patients DSE

Median 3 years
(range 696
months)

1.3%/year

McCully et al.81

1,325 patients EXSE

Median 2 years
(range 565
months)

0.9%/year

(ACM: All-cause mortality; CD: Cardiac death; Dipy: Dipyridamole; DSE: Dobutamine stress echocardiography; EXSE: Exercise stress
echocardiography; HIV: Human immunodeficiency virus; LVH: Left ventricular hypertrophy; MI: Myocardial infarction; TME: Treadmill exercise stress echocardiography).

Chapter 61: Stress Echocardiography

Role of Left Atrial Size in Risk

Stratification and Prognosis
An increase in left atrial dimension is a risk factor for atrial
fibrillation, stroke, and death and is closely related to
general cardiovascular mortality.39 Left atrial size reflects
the chronicity and magnitude of increased left ventricular
filling pressure. Left atrial size is a marker of the severity
and duration of left ventricular diastolic dysfunction in
patients without significant mitral valve disease or systolic
heart failure.
Left atrial size has been found to further risk stratify
patients with both normal and abnormal stress echocar
diography results.40 A normal stress echocar
diography
in the setting of a normal left atrial size confers a benign
prognosis (< 1% year). Left atrial size alone provides
independent and incremental prognostic value, indep
endent of traditional risk factors, ejection fraction, and
stress echocardiographic variables. Left atrial size should
be routinely incorporated in prognostic interpretation of
stress echocardiography.

Warranty Time of a Normal

Stress Echocardiogram
A normal stress echocardiogram confers a benign prog
nosis (< 1%/year) in most subgroups of patients. However,
it is unclear at what time a stress echocardiogram should
be repeated for risk stratification and prognosis if clinically
warranted (change in chest pain or clinical symptom
characteristics).
In patients with a normal stress echocardiogram, the
event rate at the end of 6, 12, and 18 months was <1%/year.41
After 18 months, the event rate in patients with a normal
stress echocardiogram increases to >1%/year. Thus, a
normal stress echocardiogram has a benign prognosis
(< 1%/year) for up to 18 months and may be repeated
after that if clinically warranted for effective risk
stratification.

Impact of Stress Echocardiography

on Patient Outcome
The ultimate maturity of an imaging modality is confi
rmed by patient outcome data. There has been a demo
nstrated parallel between the degree of abnormal stress
echocardiography results and referral to coronary
angio
graphy and revascularization.42 Despite physician

1317

self-referral incentives for coronary angiography, stress

echocardiography is an effective gatekeeper for an
invasive management strategy. Patients with normal stress
echocardiography studies (pWMSI = 1.0) have uniformly
low referral rates for early coronary angiography (1.7% at
30 days) and late revascularization (2.8% percutaneous
coronary intervention, 1.1% coronary artery bypass graft
surgery at 2 years). The frequency of referral to coronary
angiography and revascularization increased in proportion
to magnitude of the extent and severity of abnormal stress
echocardiography. The fact that only a minority of patients
with abnormal stress echocardiography were referred for
coronary angiography and revascularization implies that
such decisions are often complex and incorporate other
comorbidities into the decision on whether to refer for
invasive testing. These findings are also consistent with a
low referral for coronary angiography and revascularization
following abnormal nuclear scintigraphy studies.43

Cost-Effectiveness of Stress
Echocardiography in Postmyocardial
Infarction Patients
Cost-effectiveness of the four testing strategies in patients
with prior myocardial infarction was previously evaluated.44
A primary cardiac catheterization strategy (Strategy 1)
was found to be 23% more expensive, a primary stress
electrocardiogram (ECG)/exercise treadmill test strategy
(Strategy 2 and Strategy 3) was 82% more expensive
when compared to a primary stress echocardiography
strategy (Strategy 4). In patients undergoing stress
echocardiography followed by cardiac catheterization
(Strategy 4), the total cost savings was $57,293/patient
compared to a primary cardiac catheterization strategy,
which translated into cost savings of $217/patient correctly
identified. Given the high-risk nature of postmyocardial
infarction patients, it is no surprise that a primary stress
ECG/exercise treadmill test strategy was not cost-effective.
The cost-effectiveness analysis of the postmyocardial
infarction patient study showed that a strategy based on
initial stress testing and cardiac catheterization only in
patients with abnormal stress echocardiogram is ~$217/
patient correctly identified, more economical than a
primary invasive strategy. With a projected incidence of
1.1 million new, recurrent, or silent myocardial infarctions
for 2013 by American Heart Association (AHA),45 the above
data could translate into cost savings of billions of dollars
each year. This is in concordance with previous studies by

1318

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Shaw and colleagues who showed that patients with stable

angina who went directly to coronary angiography had a
much higher utilization of subsequent revascularization
but with a similar rate of death and myocardial infarction,
and much higher attendant costs compared to those who
were tested first with perfusion imaging and referred to
catheterization dependent on the findings.46 In patients
with high-risk features (ST changes, unstable angina,
elevated cardiac enzymes), a primary invasive strategy
may be more appropriate.

Doppler Hemodynamic
with Stress Echocardiography

ACC/AHA recommends stress echocardiography as a

Class I indication for symptom assessment and severity of
mitral stenosis. An increase in mean transmitral pressure
gradient > 15 mm Hg or an increase in peak pulmonary
pressure > 60 mm Hg is considered significant to warrant
invasive strategy. In asymptomatic patients with severe
mitral regurgitation, left ventricular dysfunction can be
evaluated with exercise stress echocardiography.50 In
asymptomatic patients with severe mitral regurgitation,
pulmonary artery systolic pressure > 60 mm Hg during
exercise may lower threshold for valve surgery.50

Hypertrophic Cardiomyopathy

The most important indication for surgical intervention

in patients with hemodynamically significant aortic or
mitral valve disease is the development of symptoms, as
emphasized in the recent guidelines.47 As valvular heart
disease progresses, the patient may be unaware of these
chronic changes in effort tolerance but instead adapt to
the symptoms by reducing physical activity and lifestyle
modifications. Therefore, exercise stress echocardiography
provides objective evidence in assessing symptoms,
exercise capacity, and hemodynamic changes of the valve
during stress that may be absent at rest.

Asymmetric hypertrophic cardiomyopathy (HCM) is a

common cause of dyspnea and is associated with sudden
cardiac death. HCM may be present with or without left
ventricular outflow tract (LVOT) gradient. ACC/AHA
guidelines for the diagnosis and treatment of HCM assign
exercise echocardiography as Class IIa for the detection
and quantification of exercise-induced dynamic LVOT
obstruction in patients who do not have significant
gradients at rest or provocation.51

Aortic Valve Disease

Many patients with diastolic dysfunction have symptoms

of dyspnea on exertion. The symptoms are due to rise in
left ventricular filling pressures that is needed to maintain
adequate left ventricular filling and stroke volume during
exercise. Stress echocardiography can assess latent dias
tolic dysfunction not apparent at rest but only at stress.
When relaxation is normal, the mitral annulus velocity
(e') and mitral inflow velocity (E) increase proportionally
(~25%), whereas E/e' ratio remains unchanged or is
reduced (< 8). In patients with impaired myocardial
relaxation, the increase in e' with exercise is much less than
that of mitral E velocity, such that the E/e' ratio increases
(> 15).52 Therefore, E/e' ratio increases with exercise
compared with rest in patients with latent diastolic
dysfunction.

Exercise stress echocardiography is contraindicated in

severe symptomatic aortic stenosis. In patients with asymp
tomatic aortic stenosis, stress echocardiography can provide
prognostic information by unmasking symptoms.48
An important role of stress echocardiography is in
patients with left ventricular dysfunction and low-flow or
low-gradient aortic stenosis. Low-dose dobutamine is used
to increase inotropic contractility and increasing stroke
volume differentiating pseudosevere aortic stenosis due to
left ventricular dysfunction from true aortic stenosis. The
American College of Cardiology (ACC)/American Heart
Association (ACH) recommends stress echocardiography
as Class IIa for low-flow, low-gradient aortic stenosis and
Class IIb for symptom assessment.49

Mitral Valve Disease

In some patients with severe asymptomatic mitral stenosis
and symptomatic moderate stenosis, exercise stress
echocardiography may precipitate or reproduce symptom.

Latent Diastolic Dysfunction

Dynamic Pulmonary Hypertension

In normal subjects, exercise increases stroke volume while
pulmonary vascular resistance decreases. Normal values
are defined by systolic pulmonary artery pressure < 43
mm Hg during exercise.53 Exercise-induced pulmonary

Chapter 61: Stress Echocardiography

Fig. 61.14: An example of a nonischemic treadmill exercise echo

cardiogram demonstrating dynamic pulmonary hypertension. The
resting study is on the left that shows normal right ventricular size
and function. The postexercise images on the right show right
ventricular dilatation and compression of the left ventricular cavity
with paradoxical septal motion. The abnormal septal motion is due
to right ventricular pressure overload and not due to ischemia. This
is a typical example of dynamic pulmonary hypertension during
exercise. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle). (Movie clip 61.6).

hypertension has been recognized as an early phase of

the pulmonary hypertension spectrum, especially in highrisk patients.54 The abnormal exercise-induced increase
in pulmonary pressures can be ascribed to elevated
cardiac output (e.g. in athletes) or to a normal increase
in flow but a rise in resistance due to limited capability
of the pulmonary vascular bed (e.g. chronic obstructive
pulmonary disease or advanced age) (Fig. 61.14 and
Movie clip 61.6).

STRESS ECHOCARDIOGRAPHY:
FUTURE DIRECTIONS
2D Strain, Myocardial Perfusion
and Three Dimensional (3D) Stress
Echocardiography
The interaction of ultrasound energy with the myocardium
results in unique random acoustic speckle patterns.55
Information regarding the motion and displacement
of acoustic speckle of the myocardium can be tracked
automatically throughout the cardiac cycle using sophis
ticated algorithms. The patterns can be used to obtain
strain and strain rate and thus quantification of wall
motion abnormality.55

1319

Microbubble image-enhancing agents such as

perflutren
lipid
microspheres
(Optison/Definity)
microbubbles have been used to assess myocardial
perfusion. The perfusion information in conjunction
with the corresponding regional wall motion improves
sensitivity to detect flow-limiting CAD.56
Major technological advances have led to the
development of real time, 3D stress echocardiography.
It allows for numerous tomographic interrogations,
eliminates off-axis acquisition and analysis, avoids
foreshortening of the apex, thus allowing multiple crosssectional views of the left ventricle, and more precise
comparison of similar segments to improve the detection
of localized ischemia.57 3D stress echocardiography
overcomes the difficulties in acquiring multiple views
immediately postexercise. Furthermore, multiple
tomography views can be evaluated for better assessment
of wall motion abnormality.

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73. Sozzi FB, Elhendy A, Roelandt JR, et al. Long-term prognosis
after normal dobutamine stress echocardiography. Am J
Cardiol. 2003;92(11):126770.

74. Hoque A, Maaieh M, Longaker RA, et al. Exercise
echocardiography and thallium-201 single-photon emi
ssion computed tomography stress test for 5- and 10-year
prognosis of mortality and specific cardiac events. J Am
Soc Echocardiogr. 2002;15(11):132634.
75. Sicari R, Pasanisi E, Venneri L, et al. Echo Persantine
International Cooperative (EPIC) Study Group; Echo
Dobutamine International Cooperative (EDIC) Study
Group. Stress echo results predict mortality: a large-scale
multicenter prospective international study. J Am Coll
Cardiol. 2003;41(4):58995.
76. Elhendy A, Mahoney DW, Khandheria BK, et al. Prognostic
significance of the location of wall motion abnormalities
during exercise echocardiography. J Am Coll Cardiol.
2002;40(9):16239.

77. Marwick TH, Case C, Vasey C, et al. Prediction of
mortality by exercise echocardiography: a strategy for
combination with the duke treadmill score. Circulation.
2001;103(21):256671.

78. Marwick TH, Case C, Sawada S, et al. Prediction of
mortality using dobutamine echocardiography. J Am Coll
Cardiol. 2001;37(3):75460.
79. Krivokapich J, Child JS, Walter DO, et al. Prognostic value
of dobutamine stress echocardiography in predicting
cardiac events in patients with known or suspected
coronary artery disease. J Am Coll Cardiol. 1999;33(3):
70816.
80. Poldermans D, Fioretti PM, Boersma E, et al. Long-term
prognostic value of dobutamine-atropine stress echo
cardiography in 1737 patients with known or suspected
coronary artery disease: A single-center experience.
Circulation. 1999;99(6):75762.
81. McCully RB, Roger VL, Mahoney DW, et al. Outcome
after normal exercise echocardiography and predictors
of subsequent cardiac events: follow-up of 1,325 patients.
J Am Coll Cardiol. 1998;31(1):1449.

CHAPTER 62
Squatting Stress
Echocardiography
Premindra AN Chandraratna, Dilbahar S Mohar, Peter Sidarous

Snapshot

Squatting Echocardiography

INTRODUCTION
Regional mismatch between coronary oxygen supply
and myocardial demand results in myocardial ischemia.
In such ischemic settings, wall motion abnormalities
(WMAs) detectable by echocardiography manifest early
as diastolic and subsequently systolic changes. Moreover,
functional wall abnormalities are early changes in the
well-described ischemic cascade, which concludes with
later surrogates including electrocardiography (ECG)
changes and subsequently overt chest pain.1 As such,
stress echocardiography (SE) has an established utility for
the detection of significant coronary artery disease (CAD)
with a notable accuracy of 8090%, which is superior to that
of exercise electrocardiographic testing and comparable to
that of nuclear stress imaging.2
Stress echocardiography is performed either with
exercise on a treadmill or bicycle or by infusion of a
pharmacological agent such as dobutamine, adenosine,
dipyridamole, or transes
ophageal atrial pacing. Each
of these techniques has advantages and disadvantages.
Treadmill or bicycle exercise echocardiography permits
assessment of both myocardial ischemia and functional
capacity. However, this technique may have limited
echocardiographic utility in patients in whom peak
exercise is not attainable or in patients with single vessel or

moderate stenosis.3 Furthermore, WMA in the immediate

postexercise period may rapidly resolve in some patients
resulting in false-negative studies.4 Patients with
comorbid conditions such as intermittent claudication,
chronic obstructive pulmonary disease (COPD), and
musculoskeletal or joint abnormalities may not be able to
achieve the levels of exercise sufficient for the detection
of myocardial ischemia. Occasionally, ventricular tachy
cardia, ventricular fibrillation, myocardial infarction, or
death may occur during exercise testing.5
Pharmacological stress testing is performed when exercise
testing is not feasible or it may be the preferred method of SE
in some laboratories. Moreover, the 2011 American Society
of Echocardiography (ASE) Appropriate Use Guidelines for
Echocardiography recommend dobut
amine as the firstline agent for pharmacologic SE.6 Minor side effects such as
chest pain, tremor, palpitations, and dizziness are frequently
noted.5 Less commonly, more serious complications such as
ventricular or supraventricular tachyarrhythmia, myocardial
infarction, or death may occur.7 Although both exercise and
pharmacological SE are reliable methods of detecting CAD,
the potential for serious side effects, cost, and the time factor
are disadvantages of these techniques. Thus, there is the
need for a safe, rapid, and inexpensive echocardiographic
stress test.

1324

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 62.1: End-systolic frames during standing (left) and squatting (right) in a normal subject. Note the triangular shape of the
apex during standing and squatting. (LV: Left ventricle). (Movie
clip 62.1).
Source: Reproduced with permission from Ref. 10.

SQUATTING ECHOCARDIOGRAPHY
Squatting echocardiography has emerged as a promising
modality which augments afterload and preload with little
or no change in cardiac contractility. Moreover, as afterload
and preload are major determinants of myocardial oxygen
consumption, patients with significant CAD develop acute
WMAs during squatting, which reversibly resolve upon
arising. Lewis et al. studied the effects of squatting on
hemodynamic parameters and left ventricular (LV) dime
nsions in normal subjects.8 They observed that squatting
produced increased LV cavity dimensions and increase
in mean blood pressure. There was an increase in stroke
index and cardiac index.
Chandraratna and colleagues have extended previous
observations on squatting echocardiography and demon
strated that squatting produces LV WMA in patients with
CAD.

Squatting Echocardiography Protocol

The squatting protocol included recording of the standing
(3 minutes of quiet standing) heart rate and blood pressure.
Standard parasternal long- and short-axis views and apical
two-, three-, and four-chamber views were obtained in
the standing position. The positions of the transducer
were marked and used for the squatting and dobutamine
studies. The subjects were asked to squat for 2 minutes. The
body weight was positioned over the heels and the torso
maintained in a nearly vertical position. The subjects were
instructed to maintain a normal breathing pattern, and
the blood pressure, heart rate, and echocardiogram were
recorded. The subjects were then asked to stand up and
the above parameters were repeated. A 16-segment model

Fig. 62.2: Echocardiogram of a patient with left ventricular (LV) function normal in the standing position. The LV apex had a triangular
appearance at end-systole. An extensive wall motion abnormality
developed during squatting (arrows). The distal posterior septum,
apex, and distal posterolateral wall became akinetic, and the distal
half of the LV became dilated. The wall motion abnormalities and the
acute left ventricular remodeling (AVLRM) normalized on standing.
(Movie clip 62.2).
Source: Reproduced with permission from Ref. 10.

was used for analysis of echocardiographic images. A

squatting stress echocardiogram was considered positive
if there was a new or worsening WMA during squatting. An
isolated fixed WMA was not considered a positive result.

Squatting Echocardiography:
Results/Observations
Squatting Induces LV WMA in Areas
Subtended by Stenotic Coronary Arteries
The study populations consisted of 15 normal male
subjects (Group 1) and 42 males subjects (Group 2) who
had coronary angiography.9 Each patient underwent
squatting echocardiography testing as per protocol, and
standard echocardiographic views were attained and
interpreted by an expert echocardiography reader blinded
to angiographic results.
Group 1 subjects had normal LV global and regional
function while standing. There were no WMA while
squatting (Fig. 62.1 and Movie clip 62.1). In Group 2, five
patients had a baseline WMA. New or worsening WMA
occurred during squatting in 35 patients. Twelve patients
developed a WMA in the left anterior descending coronary
artery territory, five had WMA in the circumflex coronary
artery territory, seven had WMA in the right coronary
artery territory, and seven had normal wall motion. Eleven
had WMA in multiple territories (Fig. 62.2 and Movie
clip 62.2). All WMA resolved on standing within 1 min.
None of these patients developed chest pain, arrhythmias,
or hypotension.

Chapter 62: Squatting Stress Echocardiography

1325

( 90% stenosis). Six subjects in Group 2 had LMCAS

and none had severe 3-vessel disease (p < 0.05 vs Group
1 for LMCAS and/or 3-vessel disease). In Group 3, eight
had LMCAS and none had severe 3-vessel disease
(p < 0.0001 vs Group 1).
These observations suggest that patients who develop
ALVRM during squatting have severe CAD and should be
considered for urgent revascularization therapy.

Figs 62.3A and B: (A) Apical four-chamber, end-systolic frame in

a patient with severe 3-vessel disease obtained in the standing
position. Note that the apex is triangular; (B) Apical four-chamber,
end-systolic frame during squatting. There is marked dilatation of
the distal half of the left ventricle (LV). The arrows indicate the
extent of the wall motion abnormality. (Movie clip 62.3).
Source: Reproduced with permission from Ref. 10.

The sensitivity, specificity, and accuracy of squatting

echocardiography for diagnosis of the CAD were 92%,
80%, and 91%, respectively.
In summary, the study showed that squatting induces
LV WMA in areas subtended by stenotic coronary arteries.

Patients Who Exhibit Acute LV Remodeling

(ALVRM) on Squatting Have Severe CAD
It was previously demonstrated that squatting induces LV
WMA in areas subtended by stenotic coronary arteries.9,10
In addition, it was observed that some subjects developed
acute changes in LV shape (ALVRM) during squatting.
Ninety-six subjects were divided into three groups.
Group 1 consisted of 12 subjects who developed squattinginduced ALVRM with apical and distal posterior septal
akinesis, dilation of the apex, and marked LV shape
change at end-systole (Figs 62.2 and 62.3; Movie clips 62.2
and 62.3). Group 2 consisted of 20 subjects with distal
posterior septal and apical akinesis without ALVRM,
during squatting. Group 3 consisted of 64 subjects who
developed WMA in areas other than the apex (n = 49), or
normal wall motion (n = 15) during squatting.
Coronary angiography in Group 1 revealed that six
subjects had left main coronary artery stenosis (LMCAS
50%), two had severe 3-vessel disease ( 90% stenosis),
and one had 100% left anterior descending coronary artery
occlusion. Severe CAD was defined for purpose of this
study as the presence of LMCAS, or severe 3-vessel disease

Comparison of Squatting SE and

Dobutamine SE for the Diagnosis of CAD11
Thirty-nine patients scheduled to have coronary angio
graphy for the evaluation of chest pain were included
in the study. Each patient had squatting SE followed by
dobutamine SE. For squatting SE, the echocardiogram in
standard views was recorded in the standing position. The
procedure was repeated during squatting for 2 minutes.
Dobutamine echocardiography was performed using
standard protocol.
Hemodynamic response to both squatting and dobut
amine are compared in Table 62.1.
During squatting, new or worsening WMA developed in
20 patients. Six patients developed WMA in the left anterior
descending (LAD) territory, three in the circumflex territory,
three in the RCA territory, and eight in multiple coronary
territories (Movies clips 62.4 to 62.6). The sensitivity,
specificity, and accuracy of squatting echocardiography for
the diagnosis of CAD were 95%, 94%, and 94%, respectively.
For dobutamine SE, the sensitivity, specificity, and
accuracy for the diagnosis of CAD were 85%, 94%, and 90%,
respectively. There was no significant difference between
squatting and dobutamine SE for the diagnosis of CAD
(p = .702).
These data indicate that squatting and dobutamine
echocardiography are equally useful in the diagnosis of
CAD.

Mechanism of Squatting-Induced WMA

Although the mechanism by which squatting induces WMA is
uncertain, a potential mechanism is unmasking of subclinical
segmental dysfunction in normokinetic segments subtended
by stenotic coronary arteries. Yuda et al.12 have demonstrated
subclinical LV dysfunction in patients with CAD and normal
ejection fraction. A second more likely mechanism is the
induction of myocardial ischemia as squatting increases
myocardial oxygen consumption by increasing stroke
volume via augmentation of preload and afterload.

1326

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 62.1: Heart Rate and Blood Pressure Response to Squatting versus Dobutamine11

Total Cohorts

Standing

Squatting

p-value
Standing versus
Squatting

Dobutamine

p-value
Squatting versus
Dobutamine

Heart rate (beats/min)

64 10

73 10

<0.0001

123 18

<0.0001

Systolic blood pressure (mm Hg)

121 11

136 11

<0.0001

159 23

<0.0001

Diastolic blood pressure (mm Hg)

79 6

86 7

<0.001

88 10

<0.001

Source: Reprinted with permission from Chandraratna PA, Kuznetsov VA, Mohar DS, et al. Comparison of squatting stress echocardiography and dobutamine stress echocardiography for the diagnosis of coronary artery disease. Echocardiography. 2012; 29(6):6959.

Advantages
Furthermore, there are numerous advantages to squatting
as a stress test. The absence of marked changes in heart
rate during squatting makes comparison with the baseline
echocardiogram and interpretation of stress-induced
WMA easier than when tachycardia is present. Ventricular
dilatation that occurs with squatting facilitates analysis
of wall motion. The rapid recovery of wall motion after
squatting reduces adverse sequelae. No ventricular
arrhythmias, chest pain, or hypotension were noted
during squatting. Additionally, the lack of prominent and
sustained heart rate and blood pressure increases, which
are known characteristics of other stress imaging such as
dobutamine or treadmill testing, further validates squat
echocardiography as a safer modality with fewer adverse
risks.

Limitations
There are some limitations to squatting as a stress test.
Patients with knee or hip disease or extreme obesity may
not be able to squat. The sonographer also has to squat
during the procedure, and only those with adequate
echocardiography windows are suitable for testing.
Squatting SE possesses similar limitations as com
pared to treadmill testing with respect to obesity and
orthopedics. However, unless patients have morbid obesity
or advanced orthopedic restrictions, squat imaging is still
attainable with limited effort as squatting is essentially
a static position. The same cannot be said for treadmill
testing in which certain heart rate and blood pressure end
point parameters need to be achieved for proper treadmill
assessment.

CONCLUSION
In patients with significant CAD, squatting echocar
diography has emerged as a unique stress modality, which

may be used as an adjunct to or independent of current

noninvasive stress techniques to risk stratify patients
with CAD. Moreover, squatting SE has been identified
as an inexpensive, simple, and rapid noninvasive CAD
risk assessment modality with fewer complications than
current stress imaging techniques.

REFERENCES
1. Nesto RW, Kowalchuk GJ. The ischemic cascade: temporal
sequence of hemodynamic, electrocardiographic and
symptomatic expressions of ischemia. Am J Cardiol. 1987;
59(7):23C30C.
2. Marwick TH. Stress echocardiography. Heart. 2003;89(1):
11318.
3. Marwick TH, Nemec JJ, Pashkow FJ, et al. Accuracy and
limitations of exercise echocardiography in a routine
clinical setting. J Am Coll Cardiol. 1992;19(1):7481.
4. Sicari R, Nihoyannopoulos P, Evangelista A, et al; European
Association of Echocardiography. Stress echocardiography
expert consensus statement: European Association of
Echocardiography (EAE) (a registered branch of the ESC).
Eur J Echocardiogr. 2008;9(4):41537.
5. Mertes H, Sawada SG, Ryan T, et al. Symptoms, adverse
effects, and complications associated with dobutamine
stress echocardiography. Experience in 1118 patients. Cir
culation. 1993;88(1):1519.
6. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/
AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011
Appropriate Use Criteria for Echocardiography. A Report
of the American College of Cardiology Foundation
Appropriate Use Criteria Task Force, American Society of
Echocardiography, American Heart Association, American
Society of Nuclear Cardiology, Heart Failure Society of
America, Heart Rhythm Society, Society for Cardiovascular
Angiography and Interventions, Society of Critical Care
Medicine, Society of Cardiovascular Computed Tomo
graphy, Society for Cardiovascular Magnetic Resonance
American College of Chest Physicians. J Am Soc Echo
cardiogr. 2011;24(3):22967.

Chapter 62: Squatting Stress Echocardiography

7. Picano E, Mathias W Jr, Pingitore A, et al. Safety and

tolerability of dobutamine-atropine stress echocar
diog
raphy: a prospective, multicentre study. Echo Dobutamine
International Cooperative Study Group. Lancet. 1994;
344(8931):11902.
8. Lewis BS, Lewis N, Gotsman MS. Effect of standing and
squatting on echocardiographic left ventricular function.
Eur J Cardiol. 1980;11(6):40512.
9. Chandraratna PA, Maraj R, Tabel G, et al. Left ventricular
wall motion abnormalities induced by squatting: a new
echocardiographic stress test for the diagnosis of coronary
artery disease. J Am Coll Cardiol. 2005;46(5):9313.

1327

10. Chandraratna PA, Mohar DS, Sidarous PF, et al. Implications

of acute left ventricular remodeling during squatting stress
echocardiography. Echocardiography. 2012;29(6):7005.

11. Chandraratna PA, Kuznetsov VA, Mohar DS, et al.
Comparison of squatting stress echocardiography and
dobutamine stress echocardiography for the diagnosis of
coronary artery disease. Echocardiography. 2012;29(6):
6959.
12. Yuda S, Fang ZY, Marwick TH. Association of severe coronary
stenosis with subclinical left ventricular dysfunction
in the absence of infarction. J Am Soc Echocardiogr.
2003;16(11):116370.

CHAPTER 63
Three-Dimensional Stress
Echocardiography
Rajesh Ramineni, Masood Ahmad

Snapshot

Two-Dimensional Stress Echocardiography

Three-Dimensional Transducers

Advantages of Three-Dimensional in Stress Imaging

Three-Dimensional Image Acquisition

Three-Dimensional Stress Protocol

Postacquisition Analysis

Review of Studies Comparing Three-Dimensional Stress

Differences Between 2DSE and 3DSE in Wall

Visualization

Parametric Imaging in Three-Dimensional Stress

Echocardiography

Role of Contraction Front Mapping in RT3DSE

Contrast in Three-Dimensional Stress Testing

Future Directions

Echocardiography to Current Standards

INTRODUCTION
Stress testing has been in use for a very long time in the
diagnosis of coronary artery disease (CAD), in testing
functional capacity, and in assessing prognosis. Stressinduced changes in electrocardiogram were initially
used to evaluate ischemia until the introduction of
imaging techniques, which offered higher sensitivity and
specificity. We reported the use of isometric exercise
during M-mode echocardiography in the detection of left
anterior descending (LAD) artery disease as far back as
1976.1 The value of treadmill exercise echocardiography in
the diagnosis of CAD was subsequently demonstrated.2 A
plethora of reports on the use of stress echocardiography
followed.26 Ever since, two-dimensional stress echocardi
ography (2DSE) has become an established technique
in assessing patients with CAD. Since the stress-induced
transient left ventricular (LV) wall motion abnormality
needs to be obtained rapidly,7 the technique of acquiring

two-dimensional (2D) images from multiple windows can

be challenging. The likelihood of capture at peak stress is
decreased in some patients, thereby reducing sensitivity
in the detection of ischemia.8 This creates a role for the
application of three-dimensional (3D) imaging during
stress.9 3D images can be acquired rapidly from a single
acquisition and multiple views of the myocardial segments
can be derived from the data set. Thus, three-dimensional
stress echocardiography (3DSE) can be performed with a
shorter scan time, and the technique does not require a
high level of operator skill. In this chapter, we will discuss
the evolving technology of 3DSE, describe its current
applications, and review major clinical studies to date.10

TWO-DIMENSIONAL STRESS
ECHOCARDIOGRAPHY
As an established technique in the assessment of
patients with CAD, 2DSE is commonly used in many

Chapter 63: Three-Dimensional Stress Echocardiography

Figs 63.1A and B: 3D transducers. (A) Current three-dimensional

(3D) transducer with a smaller footprint; (B) First-generation 3D
transducer with a larger footprint.

echocardiography laboratories. It has an estimated

sensitivity of 71%, a specificity of 85.7%, and a negative
predictive value of 95% for any vessel disease.11 The
sensitivity of stress echo is higher for multivessel CAD12
and lower for single vessel disease.13 However, 2DSE has
a number of limitations. In addition to the difficulties
in obtaining images in a very short time frame, the
technique has some intrinsic limitations. 2D planes are
not always aligned precisely for accurate comparison of
segmental wall motion from baseline to stress. Moreover,
apical foreshortening is a major concern, increasing false
negatives. With the advent of 3D echocardiography (3DE),
many of the limitations of two-dimensional echocardi
ography (2DE) are put to rest.1417 Simultaneous assessment
of the LV in multiple planes from one acquisition removes
temporal assumptions and improves diagnostic accuracy.
A shorter scan time means greater possibility of image
acquisition at the target heart rate. Moreover, cropping
of the full volume images provides unlimited number of
conventional and unconventional views of the LV that
cannot be obtained by the standard 2D technique.

THREE-DIMENSIONAL
TRANSDUCERS
Initial attempts to obtain 3D images were based on
reconstruction of multiple 2D images acquired by
manually tilting the transducers at various angles around a
fixed plane (Figs 63.1A and B). However, the acquisition by

1329

these transducers was extremely time consuming, thereby

precl
uding their use during stress. The introduction
of matrix array transducers revolutionized the image
acquisition in 3D. For the first time, the images could be
obtained in a true 3D format.
The first version of these transducers was designed and
described by von Ramm and Smith.18 These transducers
had 256 nonsimultaneous firing elements with an achieved
frequency of 2.5/3/5 Hz. A pyramidal volume data set was
obtained in a heartbeat with a sector angle of 60 60.
We had the opportunity to use this transducer during
stress and described, for the first time, the application of
3D during stress.8 All of the data could be obtained in a
single heartbeat and the images were cropped to display
the orthogonal and short-axis views of the LV. Due to the
small number of elements in the matrix, the resolution of
the images generated with this transducer was somewhat
low and the sector angle of 60 60 did not accommodate
dilated LVs.
The latest version of matrix array transducers uses
3,000 to 4,000 elements. This change significantly impr
oved the image resolution, penetrability, and side lobe
suppression. Moreover, the new transducers are smaller,
making it possible to fit the transducer in the rib spaces and
acquire images at wider sector angles. These transducers
can be used comprehensively for combined 2D and 3D
imaging.

ADVANTAGES OF THREEDIMENSIONAL IN STRESS IMAGING

With advances in transducer technology and the arrival
of matrix array transducers, real time 3D acquisitions
(RT3D) became possible and the role of 3DE in stress
testing surfaced (Table 63.1).9 Multiple studies have
successfully documented the feasibility of performing 3DE
during dobutamine and exercise stress testing. Although
there are variations from vendor to vendor, generally, fullvolume acquisitions of the LV are used for stress testing
that are obtained over a span of three to four cardiac
cycles. Data can be obtained with a single cardiac cycle
as well; however, the volume rate may be limited at high
heart rates. With one of the major limitations of 2DE being
the inability to obtain images in multiple planes in a very
short time period at peak stress, acquisition of full-volume
image with a maximum of four cardiac cycles places RT3D
at an advantage. In our laboratory, we demonstrated that
RT3D data during dobutamine stress echocardiography
(DSE) can be obtained in less than half the time needed

1330

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 63.1: Advantages and Limitations of RT3DSE

Advantages:
Shorter acquisition time
User-friendly and less operator dependent
Good interobserver agreement. Reproducible
Precise alignment/anatomically correct tomographic section. More accurate comparison of matched rest and stress images
Full-volume acquisition of entire true LV. Multiple sections of the LV and apical region essentially eliminate problems related to
off-axis image acquisition or LV foreshortening
Quantitative. Assessment of LV volume and EF comparable to CMR
Limitations:
Lower spatial and temporal (frame/volume rate) resolution especially at peak stress
Influenced by respiration, patient motion, and significant variation in heart rate causing image artifacts
Suboptimal anterior and lateral wall visualization related to larger transducer footprint
Longer offline data analysis time
(CMR: Cardiac magnetic resonance; EF: Ejection fraction; LV: Left ventricle).

for 2DE with greater sensitivity validated by cardiac

catheterization.8 These data are replicated in multiple
other studies.1927 The 3D data set obtained during the brief
acquisition period can be cropped and processed to assess
wall motion at rest and peak stress.
Misaligned 2D images can lead to erroneous estimation
of LV wall motion. This is overcome in RT3D by properly
aligning the LV along its long axis so that the true apex of
the LV is displayed. Even though, at this time, the image
resolution in 2DE is superior to 3DE, the current advances
in transducers make it possible to acquire full volume
data set at a high volume rate (40 volumes/s) resulting in
diagnostic images in the vast majority of patients.
3D imaging is quantitative and can assess LV volumes
as well as LV ejection fraction with accuracy comparable
to cardiac magnetic resonance imaging, which is an
additional benefit in stress imaging to identify transient
ischemic dilatation.28,29

THREE-DIMENSIONAL
IMAGE ACQUISITION
The introduction of matrix array transducers and parallel
processing allowed real time acquisition of 3D images as
opposed to reconstruction from spatially oriented and
sequentially obtained 2D images. Initially, the matrix array
had 256 elements that generated a 60 60 pyramidal
volume. Although the stress images had limited resolution,
the ability to obtain multiple views in either orthogonal

(multiplane) or in short-axis (multislice) views from a

single data set was truly impressive and obviated the need
for multiple acquisitions from multiple windows. The new
matrix array transducers have 3,000 to 4,000 elements in
the matrix, resulting in higher imaging quality.
Using Philips iE33 (Philips Medical Systems, Bothell,
WA), RT3D images can be obtained in three different
modes. They are live 3D, zoom, and wide angle with their
respective angles being 50 30, 30 30, and 90 90.
Live 3D images can be obtained within a single heartbeat.
This the most common mode of RT3D used for structural
assessment of the heart. When a more focused zone needs
further detailed exam such as valvular abnormalities,
the zoom mode is used. This mode has greater detail at
the expense of providing information about a smaller
area. A wide angle image is obtained by merging four
small pyramids. These separate pyramids are obtained in
consecutive beats by ECG gating. Due to a wider sector
angle, more volume is captured in this mode making it the
mode of choice in stress imaging. However, intrinsic to the
use of information from multiple beats, arrhythmias, stitch
artifacts, and motion artifacts pose a limitation to this
mode, some of which can be overcome by holding breath
during acquisition. In patients with arrhythmias, the
artifacts can be further reduced by decreasing the number
of beats in the full-volume acquisition. The 3D data set
can be cropped to display any number of conventional
and nonconventional views of the LV for a more thorough
assessment of wall motion. The images can be aligned

Chapter 63: Three-Dimensional Stress Echocardiography

along the true long axis of the LV for a superior assessment

of the true LV apex. With recent developments in 3D
software, high-quality, single-beat 3D acquisitions can be
obtained at good volume rates of 3040 frames per second.

THREE-DIMENSIONAL
STRESS PROTOCOL
The early versions of the 3D equipment lacked combined
2D and 3D imaging. Therefore, in order to obtain 2D and
3D images at baseline and at peak stress, the transducers
had to be switched to obtain both sets of images. With
the newer systems, the transducers have both 2D and
3D imaging capabilities. Both sets of images can also be
obtained by simply switching the acquisition modes.
Alternatively, all 2D images can be derived from the
3D data set. The protocol for imaging 3D data sets with
routinely performed 2D stress imaging remains variable
from laboratory to laboratory. Our approach is to use an
integrated 2D/3D stress protocol that includes 3D images
in the standard 2D stress imaging protocol. Essentially,
3D full-volume acquisitions are obtained at baseline after
capturing standard 2D images from multiple windows. At
this time, we obtain 3D acquisitions from the apical and
parasternal windows. These steps are repeated at peak
stress.

POSTACQUISITION ANALYSIS
A single, full-volume acquisition of RT3D can be
analyzed in various formats. A simultaneous display of
major orthogonal views can be created. This is called
the multiplane view. We are able to obtain parasternal
long- or short-axis or apical four-, two- or three-chamber
views by this technique. Multiple short-axis 2D images
(nine equidistant images in general) can also be obtained
from apex to base. This is called the multislice view.
(Movie clips 63.1 to 63.4). Studies have shown that
specificity of detection of CAD and accuracy of identifying
right coronary lesions were significantly greater with the
use of multislice view.26 This advantage was attributed to
better visualization of basal wall motion abnormalities
and the acquisition of correct short-axis views. This view
is also associated with greater reduction in interobserver
variances.26 Analysis of the LV wall motion can be done by
the 16- or 17-segment model as recommended by the ASE.
The images are analyzed in a complementary fashion;

1331

that is, if a wall motion abnormality is suspected in 2D

images but is not entirely clear, 3D images are reviewed
for confirmation or exclusion of abnormal wall motion.
This integrated approach is very useful in evaluating
questionable wall motion changes. In some patients,
when the LV is foreshortened in 2D images, 3D images are
aligned along the true long-axis of the LV and the apex is
carefully examined for wall motion abnormalities (WMA)
(Movie clips 63.5 and 63.6). Quantitative data regarding
LV volumes and LV ejection fraction are available from
the 3D images. Improvements in software used in postac
quisition analysis are making it possible to perform a sideby-side synchronized analysis of anatomically aligned 3D
DSE data sets resulting in better interobserver agreement.26
In selected patients, we have used contraction front
mapping (parametric imaging) in assessing dyssyn
chronous LV wall motion induced by ischemia.30 This
technique is still evolving and needs further study.

REVIEW OF STUDIES COMPARING

THREE-DIMENSIONAL STRESS
ECHOCARDIOGRAPHY TO CURRENT
STANDARDS
Dobutamine Stress Test
Given the concern about obtaining good images when
patients are hyperventilating postexercise, initial studies
comparing the use of 3DE over 2DE for stress testing
started with DSE (Table 63.2). Using the first-generation
matrix array transducers, we tested for feasibility of RT3DE
during DSE and compared its performance to 2DE in
253 patients.8 A subanalysis of correlation with coronary
angiograms was done in 90 patients. It was noted that
LV wall motion scores were similar with both techniques
at rest as well as peak stress, but acquisition of RT3DE
was quicker (27.4 10.7 s vs 62.4 20.1 s by 2D with a
P < 0.0001). Real time 3D echocardiography was also noted
to have higher interobserver agreement for detection of
ischemia at peak stress and higher sensitivity in detection
of CAD in patients who underwent coronary angiograms.
The next major study in this field was unveiled in
2005 when Matsumura et al., compared 2DE to RT3DE
during dobutamine stress in 56 patients who previously
underwent SPECT due to suspicion of ischemia.24 Similar
to our findings they also noted that RT3DE is significantly
quicker than 2DE with a success rate comparable to 2DE
(92% at rest and 89% at peak stress in RT3DE vs 94% and

1332

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 63.2: Diagnostic Sensitivity and Specificity of Three-Dimensional (3D) Versus Two-Dimensional (2D) in Detecting Coronary
Artery Disease (CAD)

Study (Author, Year, Ref.)

No.

Stress Test (Type)

Validation

Ahmad et al. (2008)

58

DSE

Matsumura et al. (2005)

56

Takeuchi et al. (2006)31

78

Aggeli et al. (2007)

Yoshitani et al. (2009)26

8
24

22

Jenkins et al. (2009)

2D Sen.

Sp.

3D Sen.

Sp.

Coronary angiography

79

88

DSE

Thalium201-SPECT

86

83

80

DSE

None

58

75

56

DSE

Coronary angiography

73

78

93

89

71

DSE-3D multiplane

Coronary angiography

72

72

DSE-3D multislice

Coronary angiography

77

95

Treadmill exercise-2D

Coronary angiography

83

65

Treadmill exercise-3D

Coronary angiography

40

84

Treadmill exercise-3D+
CFM

Coronary angiography

55

78

107

Dipyridamole

None

78

91

80

87

30

Adenosine 2D

Tc 99m Sestamibi
SPECT

92

75

Adenosine Live 3D

Tc 99m Sestamibi
SPECT

91

69

Adenosine full volume 3D

Tc 99m Sestamibi
SPECT

90

79

90

32

Badano et al. (2010)21

Abdelmoneim et al. (2010)

33

(CFM: Contraction front mapping; DSE: Dobutamine stress echocardiography; No.: Number of subjects; Sen.: Sensitivity;
Sp.: Specificity; SPECT: Single-photon electron-computed tomography; Tc: Technetium).

90%, respectively, in 2DE). The sensitivity, specificity, and

accuracy of these two modalities were very similar without
any statistical difference when the results of SPECT were
used as the reference standard.
In the following year (2006), Takeuchi et al. used
contrast to enhance the endocardial borders and
compared RT3DE with 2DE during DSE for the assessment
of WMA in 78 patients with known or suggested history of
CAD.31 This study was limited by the fact that it used an
earlier generation of bulky transducers with a narrow
angle. Levovist (Schering AG, Berlin, Germany) or Optison
(Mallinckrodt Inc., St. Louis, MO) /Definity (Bristol-Myers
Squibb, N. Billerica, MA) were used as contrast material in
Japan and the United States, respectively, for the included
patients. This study used the concept of worsening stressinduced increase in segmental wall motion score index
(WMSI) as being suggestive of ischemia. Moreover, they
assessed for regional wall motion in each coronary artery
territory by predefining segments into major coronary
arterial territories. Nine segments (basal anteroseptal,
basal anterior, mid-interventricular septum, midanteroseptal, mid-anterior, and four apical) were assigned
to the LAD coronary artery territory, three segments (basal

inferior, mid-inferior, and basal interventricular septum)

were assigned to the right coronary artery territory, and
four segments (basal lateral, basal posterior, mid-lateral,
and mid-posterior) were assigned to the left circumflex
coronary artery territory. It was noted that a large
number of segments were uninterpretable with 3D DSE
in comparison to 2D DSE, the majority of which were in
anterior and lateral walls. Despite a significant correlation
of WMSI between these two modalities, the concordance
rates were only moderate. The bulk of the transducer
limiting visualization of the anterolateral segments
and the low frame rate causing erroneous diagnosis of
dyssynchrony were the likely limitations of this study.
Using 2D DSE results as the gold standard in this study, the
sensitivity and specificity for detecting WMA by 3D DSE
were 58% and 75%, respectively. Sensitivity and specificity
values were 67% and 94% for the right coronary artery,
53% and 81% for the LAD, and 88% and 100% for the left
circumflex coronary artery territory, respectively.
In 2007, Aggeli et al. designed a study in close comp
arison to ours but with newer generation 3D transducers.
Real time 3D echocardiography with 2DE during DSE
were compared in 56 patients and validated with results

Chapter 63: Three-Dimensional Stress Echocardiography

from coronary angiography.22 They also showed that

acquisition time for RT3DE was significantly less (< 50%)
when compared to 2DE. Both the modalities showed
excellent agreement in WMSI at rest, but at peak stress
RT3DE showed significantly higher values. A regional wall
motion score of the apical segments (apWMS) was done
to delineate differences between the modalities, since
2DE is known to have limitations in accurate evaluation
of the apex. RT3DE showed significantly higher wall
motion scores at peak stress and higher sensitivity in the
LAD territory. The study concluded that the diagnostic
value of RT3DE is at least equivalent to 2DE during DSE
and, in addition, serves the advantage of markedly shorter
acquisition times. Better assessment of apical segments is
an added advantage.
Overall, the studies using dobutamine as the stress
agent showed that 3DSE significantly reduces the time
needed for imaging and the WMSI was higher. There was
also better interobserver agreement with 3DSE.

Dobutamine Stress Test with

Multiplane versus Multislice Imaging
Yoshitani et al., in a study which included 71 patients
with known or suspected CAD, compared the use of
multiplane versus multislice modes in the assessment of
wall motion from data obtained using RT3DE.26 Data sets
were acquired using a wide-angle (60 60) acquisition
mode, in which four wedge-shaped subvolumes (60
15 each) were obtained from four consecutive cardiac
cycles during held breath. Multiplane mode provided
simultaneous visualization of parasternal long- and shortaxis views or apical four-, two-, and three-chamber views
whereas nine equidistant 2D short-axis images from LV
base to apex were extracted and simultaneously displayed
comprising the multislice mode. Wall motion score was
obtained by visual assessment of these views, which
were then compared against the findings obtained from
a coronary angiogram performed the next day in which a
luminal narrowing of greater than 50% was considered as
significant stenosis. It was noted that, unlike the biplane
mode where there was no change in the number of
uninterpretable segments from rest to peak stress, these
numbers significantly dropped in the multislice mode. The
majority of these uninterpretable segments were noted in
the anterior and lateral walls in both modes. In contrast
to absence of differences at rest, WMSI was significantly
lower at peak stress in the multiplane mode compared

1333

with the multislice mode. On a patient basis, sensitivity

was not different, but specificity was significantly higher in
the multislice mode (95%) compared with the multiplane
mode (77%, P < 0.05). Diagnostic accuracy for detecting
right CAD was also significantly higher in the multislice
mode (93% vs 80%, P < 0.05).

Treadmill Exercise Stress Test

In 2009, Jenkins et al studied the feasibility of treadmill
exercise stress testing and compared 2DE against 3DE.32
Initially starting the study with 110 patients, the final
analysis was done on 90 patients with 20 excluded
(12 for nondiagnostic stress and 8 for poor quality 3DE).
Unlike the studies done using dobutamine, there was no
significant benefit in the acquisition time for 3DE in this
study. This study also involved an arm which uses 3DE
with contraction front mapping (CFM), which is discussed
in detail later in the chapter. The sensitivity of wall motion
assessment with 3DE (40%) was noted to be lower than
2DE (83%, P < 0.01) at comparable levels of specificity
(65% and 78%). However, the combined use of 3DE along
with CFM added to the sensitivity and overall accuracy.
This study was noted to be different on many fronts. The
average time taken to acquire 3D images was longer
than the previous studies with a possible impact on the
outcomes. 3D echocardiography images were obtained on
an average of 58 ( 25) seconds after the patient was off
the treadmill, which brings into question the ability to get
images at target heart rate. Moreover, contrast agents were
not used to enhance the endocardium. These limitations,
in addition to this being the only study available using
exercise as stress technique, make it difficult to draw
conclusions.

Adenosine Stress Test

Abdelmoneim et al., in a study involving 30 patients,
compared 3DE (full volume and live 3D) to 2DE and the
standard SPECT imaging to evaluate for CAD.33 Sensiti
vities and specificities were corelated between these three
modalities. Adenosine was administered as the stress
agent and contrast (Definity) was used for all echocardio
graphic studies. It was noted that live 3D identified
abnormalities in areas with reversible defects in SPECT
in 88% versus 63% with full-volume 3D images. This
was the first attempt comparing 3D and 2D contrast
echocardiography in the evaluation of perfusion defects
with SPECT as the diagnostic standard. However, a

1334

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

et al. the use of 3DSE was also associated with greater

sensitivity and specificity in assessing the lateral LV wall,
which plays a role in its detection of isolated left circumflex
lesions.31 However, due to the greater resolution of 2DE,
good views of the basal structures were obtained with 2DE
during stress testing.

Figs 63.2A and B: Segmental timevolume curves of a patient

who underwent three-dimensional stress echocardiography
(3DSE) and contraction front mapping (CFM). (A) At baseline,
synchronous timevolume curves are seen suggesting uniform
myocardial segmental contraction; (B) At peak stress, timevolume curves are dyssynchronous with nonuniform segmental
contraction suggesting reversible ischemia.

limitation of this study was a lack of comparison with

coronary angiography in patients with reversible perfusion
defects.

Dipyridamole Stress Test

Badano et al., with the newer high-volume rate scanners
with higher temporal resolution and the possibility of
displaying cropped images side by side, compared RT3DE
with 2DE during dipyridamole-induced stress (DipSE)
in 84 patients.21 The results of this study were consistent
with the other dobutamine-based studies reviewed earlier,
with RT3DE displaying significantly lower acquisition
times, higher WMSI at peak stress (more so in the apical
regions), and better sensitivity in the LAD territory as
noted in patients who underwent coronary angiograms.
Interestingly, the postacquisition analysis time, which
in general was assumed to be longer in RT3DE, was
also significantly shorter in RT3DE than in 2DE. However,
the temporal resolution was significantly better in
2DE (75 5 frames/s vs 41 5 volumes/s, respectively;
P < 0.0001).

DIFFERENCES BETWEEN 2DSE AND

3DSE IN WALL VISUALIZATION
One of the major limitations of 2DSE is its inability to open
up the entire LV apex (LV foreshortening), thus generating
a possibility of error in assessment of wall motion. Aggeli
et al. showed that 3DSE predicted a higher wall motion
score predominantly in the LV apex, which corresponded
with ischemia in LAD territory,22 as seen in the reference
standard (coronary angiography). As shown by Takeuchi

PARAMETRIC IMAGING IN THREEDIMENSIONAL STRESS

ECHOCARDIOGRAPHY
As an alternative to wall motion assessment, parametric
imaging can be applied in analysis of 3D stress data
(Figs 63.2A and B). Segmental timevolume curves and
contraction front maps can be generated from the
acquired 3D data sets. Temporal heterogeneity of myo
cardial contraction induced by stress can be measured
by the dyssynchrony index similar to the evaluation of
mechanical dyssynchrony in patients with LV dysfunction
for cardiac resynchronization therapy. Segmental time
to minimal systolic volume can be displayed at baseline
and at peak stress. Stress-induced ischemia will result in
delayed contraction in ischemic segments thus resulting
in dyssynchrony.

ROLE OF CONTRACTION FRONT

MAPPING IN RT3DSE
Contraction front mapping (TomTec Imaging Systems,
Munich, Germany) analyzes temporal and spatial activ
ation of LV contraction and displays a bulls-eye plot of the
contraction wave front of the myocardial segments that
reach peak contraction every 25 milliseconds (Movie clips
63.7 and 63.8). Color-coded maps show the contraction
wave in blue and the noncontracting segments in shades of
red. Thus, increasing degrees of dyssynchrony induced by
ischemia are displayed in shades of red. Contraction front
mapping also has the potential to provide quantitative
analysis of LV contraction through assessment of time
to minimal volume, aiding in the diagnosis of segm
ental ischemia. Contraction front mapping might be
applicable as a good qualitative and quantitative tool
in the assessment of ischemia as noted in its initial
application described from our laboratory.30 Jenkins
et al., in their study done on treadmill exercise stress test,
compared 2DE against 3DE as well as 3DE with CFM.32
CFM was done offline from the 3DE full-volume data.
After defining normal cutoff ranges for contraction
delay based on receiver-operating characteristics, they
noted that the concordance between angiography and

Chapter 63: Three-Dimensional Stress Echocardiography

qualitative CFM (63%) was similar to quantitative CFM

(70%). Moreover, it was noted that the combined use
of 3DE along with CFM added to the overall sensitivity
(improved from 40% to 55%), specificity (improved from
65% to 84%), and accuracy of 3DSE. The limitations of this
study were discussed earlier.

CONTRAST IN THREE-DIMENSIONAL
STRESS TESTING
Given the limited resolution of 3D when compared to 2D,
use of contrast agents to improve the visualization of the
endocardial border makes theoretical sense. Pulerwitz,
in an initial study of 14 patients, noted that ultrasound
contrast significantly increased the proportion of segments
adequately visualized during rest and peak dobutamine
infusion (91%98%, P = 0.001, and 87%99%, P = 0.001,
respectively).16 There was almost complete concordance
between observers (96.9% at rest and 98.2% at peak
stress with almost no interobserver variability), whereas
noncontrast studies had much lower agreement (84.4%
at rest and 79.9% at peak stress with kappa values less
than 0.4).
Nemes et al. in 2007, studied the use of contrast in
36 patients undergoing routine stress test for stable chest
pain.34 The images obtained with and without contrast
were compared for image quality index, and wall motion
was assessed using the standard 17-segment LV model.
It was noted that myocardial segment visualization
improved from 76% to 90% with the use of contrast, and the
image quality index improved from 2.2 to 3.1. Agreement
on coronary territory of ischemia improved from 79%
to 88%. Study agreement on myocardial ischemia also
improved from 72% to 89%. However, this study used the
larger transducer (X4 24 20 mm) with a bigger footprint
resulting in less favorable outcome with the use of
conventional RT3DE.

FUTURE DIRECTIONS
Further developments in transducer technology and
processing techniques should allow 3D acquisitions at
higher volume rates at high heart rates during stress.
Single beat acquisitions of full volumes will avoid
the potential for artifacts. Automated software for
side-by-side display of baseline and stress 3D images will
facilitate interpretation of 3DSE. Parametric imaging may
provide an entirely new approach in mapping ischemic
regions of the left ventricle. These developments should

1335

allow assessment of the LV with 3D during stress without

the need for concurrent conventional 2D imaging.
Automated fused stress image technique is another
modality which might prove to be beneficial in the future.
This method incorporates RT3D echocardiography with
myocardial perfusion SPECT imaging to create a fused
image which had excellent specificity and sensitivity in
detecting myocardial ischemia with low interobserver
variability in a study by Walimbe et al.35 They studied
20 patients with 36 angiographically evaluated coronary
arteries. This was a pilot study with a small number of
subjects. More studies with greater power are needed to
evaluate its feasibility and confirm these findings.

CONCLUSION
3DSE is an exciting technology that is rapidly evolving and
currently applied as an adjunctive technique to 2DSE.
The availability of 3D images is tremendously useful
in evaluating questionable areas of abnormality seen
in 2DSE and in more accurately assessing the changes
induced by ischemia.36 In the near future, 3DSE may be
applied independent of the traditional 2DSE resulting in a
comprehensive diagnostic stress test that is more efficient,
quantitative, and reproducible in the diagnosis of CAD.

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1. Ahmad M, Watson, LE. Application of stress echocardi
ography in the diagnosis of left anterior descending
coronary artery disease. Clin Res. 1976;515A.
2. Maurer G, Nanda NC. Two dimensional echocardiographic
evaluation of exercise-induced left and right ventricular
asynergy: correlation with thallium scanning. Am J Cardiol.
1981;48(4):7207.
3. Innocenti F, Caldi F, Tassinari I, et al. Prognostic value of
exercise stress test and dobutamine stress echo in patients
with known coronary artery disease. Echocardiography.
2009;26(1):19.
4. Olmos LI, Dakik H, Gordon R, et al. Long-term prognostic
value of exercise echocardiography compared with exercise
201Tl, ECG, and clinical variables in patients evaluated for
coronary artery disease. Circulation. 1998;98(24):26792686.
5. Marwick TH. Stress echocardiography. Heart. 2003;89(1):
1138.
6. Abreo G, Lerakis S, Ahmad M. Use of exercise echocardio
graphy to evaluate patients with chest pain. Am J Med Sci.
1998;316(5):34550.
7. Armstrong WF, Pellikka PA, Ryan T, et al. Stress
echocardiography: recommendations for performance and
interpretation of stress echocardiography. Stress Echocar
diography Task Force of the Nomenclature and Standards
Committee of the American Society of Echocardiography.
J Am Soc Echocardiogr. 1998;11(1):97104.

1336

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

8. Ahmad M, Xie T, McCulloch M, et al. Real-time threedimensional dobutamine stress echocardi

ography in
assessment stress echocardiography in assessment of
ischemia: comparison with two-dimensional dobutamine
stress echocardiography. J Am Coll Cardiol. 2001;37(5):
13039.
9. Ahmad M. Real-time three-dimensional echocardiography
in assessment of heart disease. Echocardiography. 2001;
18(1):737.
10. Abusaid GH, Ahmad M. Real time three-dimensional stress
echocardiography advantages and limitations. Echocardi
ography. 2012;29(2):2006.
11. Afridi I, Quiones MA, Zoghbi WA, et al. Dobutamine
stress echocardiography: sensitivity, specificity, and predi
ctive value for future cardiac events. Am Heart J. 1994;
127(6):151015.

12. Roger VL, Pellikka PA, Oh JK, et al. Identification of
multivessel coronary artery disease by exercise echocar
diography. J Am Coll Cardiol. 1994;24(1): 10914.
13. Marwick TH, Nemec JJ, Pashkow FJ, et al. Accuracy and
limitations of exercise echocardiography in a routine
clinical setting. J Am Coll Cardiol. 1992;19(1):7481.
14. Pandian NG, Roelandt J, Nanda NC, et al. Dynamic threedimensional echocardiography: methods and clinical
potential. Echocardiography. 1994;11(3):23759.
15. Wang XF, Deng YB, Nanda NC, et al. Live three-dimensional
echocardiography: imaging principles and clinical
application. Echocardiography. 2003;20(7):593604.
16. Pulerwitz T, Hirata K, Abe Y, et al. Feasibility of using a realtime 3-dimensional technique for contrast dobutamine
stress echocardiography. J Am Soc Echocardiogr. 2006;19(5):
5405.
17. Takuma S, Cardinale C, Homma S. Real-time three-dime
nsional stress echocardiography: a Review of current
applications. Echocardiography. 2000;17(8):7914.
18. von Ramm OT, Smith SW. Real time volumetric ultrasound
imaging system. J Digit Imaging. 1990;3(4):2616.
19. Pratali L, Molinaro S, Corciu AI, et al. Feasibility of realtime three-dimensional stress echocardiography: pharma
cological and semi-supine exercise. Cardiovasc Ultrasound.
2010;8:10.
20. Varnero S, Santagata P, Pratali L, et al. Head to head
comparison of 2D vs real time 3D dipyridamole stress
echocardiography. Cardiovasc Ultrasound. 2008;6:31.
21. Badano LP, Muraru D, Rigo F, et al. High volume-rate threedimensional stress echocardiography to assess inducible
myocardial ischemia: a feasibility study. J Am Soc Echoc
ardiogr. 2010;23(6):62835.
22. Aggeli C, Giannopoulos G, Misovoulos P, et al. Real-time
three-dimensional dobutamine stress echocardiography
for coronary artery disease diagnosis: validation with
coronary angiography. Heart. 2007;93(6):6725.

23. Nemes A, Leung KY, van Burken G, et al. Side-byside viewing of anatomically aligned left ventricular
segments in three-dimensional stress echocardiography.
Echocardiography. 2009;26(2):18995.

24. Matsumura Y, Hozumi T, Arai K, et al. Non-invasive
assessment of myocardial ischaemia using new real-time

three-dimensional dobutamine stress echocardiography:

comparison with conventional two-dimensional methods.
Eur Heart J. 2005;26(16):162532.
25. Yang HS, Pellikka PA, McCully RB, et al. Role of biplane
and biplane echocardiographically guided 3-dimensional
echocardiography during dobutamine stress echocardi
ography. J Am Soc Echocardiogr. 2006;19(9): 113643.
26. Yoshitani H, Takeuchi M, Mor-Avi V, et al. Comparative
diagnostic accuracy of multiplane and multislice threedimensional dobutamine stress echocardiography in the
diagnosis of coronary artery disease. J Am Soc Echocardiogr.
2009;22(5):43742.
27. Zwas DR, Takuma S, Mullis-Jansson S, et al. Feasibility of
real-time 3-dimensional treadmill stress echocardiography.
J Am Soc Echocardiogr. 1999;12(5):2859.
28. Qi X, Cogar B, Hsiung MC, et al. Live/real time threedimensional transthoracic echocardiographic assessment
of left ventricular volumes, ejection fraction, and mass
compared with magnetic resonance imaging. Echocardi
ography. 2007;24(2):16673.
29. Macron L, Lim P, Bensaid A, et al. Single-beat versus
multibeat real-time 3D echocardiography for assessing left
ventricular volumes and ejection fraction: a comparison
study with cardiac magnetic resonance. Circ Cardiovasc
Imaging. 2010;3(4):4505.
30. Ahmad M, Dimaano M, Xie C. Abstract 2916: Contraction
Front Mapping in Detection of Ischemia during Live
3-Dimensional Dobutamine Stress Echocardiography.
Circulation. 2006;114(II_612).
31. Takeuchi M, Otani S, Weinert L, et al. Comparison of
contrast-enhanced real-time live 3-dimensional dobuta
mine stress echocardiography with contrast 2-dimensional
echocardiography for detecting stress-induced wall-motion
abnormalities. J Am Soc Echocardiogr. 2006;19(3):2949.
32. Jenkins C, Haluska B, Marwick TH. Assessment of temporal
heterogeneity and regional motion to identify wall motion
abnormalities using treadmill exercise stress threedimensional echocardiography. J Am Soc Echocardiogr.
2009;22(3):2685.
33. Abdelmoneim SS, Bernier M, Dhoble A, et al. Assessment
of myocardial perfusion during adenosine stress using real
time three-dimensional and two-dimensional myocardial
contrast echocardiography: comparison with single-photon
emission computed tomography. Echocardiography. 2010;
27(4):4219.
34. Nemes A, Geleijnse ML, Krenning BJ, et al. Usefulness of
ultrasound contrast agent to improve image quality during
real-time three-dimensional stress echocardiography. Am
J Cardiol. 2007;99(2):2758.
35. Walimbe V, Jaber WA, Garcia MJ, et al. Multimodality
cardiac stress testing: combining real-time 3-dimensional
echocardiography and myocardial perfusion SPECT. J Nucl
Med. 2009;50(2):22630.

36. Ahmad M. Real-time three-dimensional dobutamine
stress echocardiography: a valuable adjunct or a superior
alternative to two-dimensional stress echocardiography?
J Am Soc Echocardiogr. 2009;22(5):443444.

CHAPTER 64
Echocardiographic Assessment of
Coronary ArteriesMorphology and
Coronary Flow Reserve
Karina Wierzbowska-Drabik, Jarosaw D Kasprzak

Snapshot

The Assessment of Coronary Morphology and Flow in

Distal Coronary Flow and Coronary Flow Reserve

Congenital Abnormalies of the Coronary Arteries

Transthoracic and Transesophageal Studies

Visualizaon of Coronary Arteries

INTRODUCTION
Coronary arteries are visible during standard echocardiographic examination. However, small size and vigorous
motion of epicardial coronary segments during respiratory
and cardiac cycle pose a significant challenge for all noninvasive imaging methods. Therefore, direct evaluation of
coronary arteries has not become a part of routine transthoracic echocardiographic (TTE) examination despite
significant technical improvements leading to improved
quality of imaging. These limitations are less evident in
transesophageal echocardiogram (TEE). Recent progress
in Doppler sensitivity encouraged routine use of distal
coronary flow velocity and vasodilator-induced velocity
reserve measurements.

THE ASSESSMENT OF CORONARY

MORPHOLOGY AND FLOW IN
TRANSTHORACIC AND
TRANSESOPHAGEAL STUDIES
Current TTE and TEE allows addressing the following
clinical aspects of coronary anatomy and physiology:
Identification of ostia and proximal segments of left
and right coronary arteries and diagnosis of congenital

anomalies of their origin by two-dimensional and

color Doppler studies
Assessment of flow in the main coronary arteries
(left main coronary artery (LMCA), circumflex (Cx),
left anterior descending (LAD), and right coronary
artery (RCA) with possible detection of stenotic flow
acceleration and luminal narrowing or aneurysms, for
example, in Kawasaki disease in pediatric population
Evaluation of accessible parts of mid and distal
coronary arteries and the assessment of coronary
by-pass grafts (usually internal mammary artery)
with assessment of coronary flow velocity reserve
(CFVR) based on Doppler recording at rest and under
vasodilator stress (with highest feasibility in LAD but
examined also in RCA)
Diagnosis of coronary fistulas draining to heart
chambers and pulmonary artery, which may be
often clearly visualized by TTE color Doppler study
(Fig. 64.1).

VISUALIZATION OF
CORONARY ARTERIES
Transthoracic Echocardiography
Visualization of proximal segments of coronary arteries
is possible in the majority of TTE examinations. Good-

1338

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 64.1: A typical image of proximal coronary arteries in parasternal short-axis view. (Ao: Aorta, LCA: Left coronary artery;
RCA: Right coronary artery; RV: Right ventricle).

Fig. 64.2: Additional image of the proximal right coronary artery

orifice in parasternal long-axis view. (Ao: Aorta; LV: Left ventricle;
RCA: Right coronary artery; RV: Right ventricle).

quality color flow recording in LMCA including proximal

Cx and RCA may be obtainable in approximately 55% of
cases.1 However, more recent publications report much
higher feasibility values2antegrade LAD flow may be
completely seen in >90% of patients, with lower values
for the proximal, middle, and distal segments of Cx (88%,
61%, and 3%, respectively) or RCA (40%, 28%, and 54% of
patients, respectively).
Typically, the coronary ostia are seen in parasternal
short-axis views at the level of the sinuses of Valsalva
(Fig. 64.1; Movie clip 64.1), with additional views of the
right coronary ostium seen in parasternal long-axis view
(Fig. 64.2). The perpendicular direction of LMCA in shortaxis view in TTE may cause suboptimal detection of flow
with underestimation of recorded flow velocity, thus
prompting a search for images from additional window
modified apical five-chamber view (Fig. 64.3).
The possibility of transthoracic detection of coronary
stenosis was underestimated for years. Recent studies
demonstrate that with a careful technique, many major
coronary segments can be visualized and a strategy of high
sensitivity Doppler detection of stenosis can be adopted in
experienced hands.3
Due to anatomical relationships, the easiest recording
of coronary flow is that obtained for the proximal LAD and
both interventricular branches. The most specific sign of
coronary stenosis is flow acceleration and turbulence
thus color Doppler can serve as a roadmap for more
detailed spectral flow interrogation both in TTE and
TEE studies (Movie clip 64.2). The presence of stenosis
with rapid, multidirectional flow may lighten up the

flow in the locations where normally no color of spectral

coronary flow signal could be recorded. Twofold increase
in coronary flow velocity or acceleration of diastolic flow
above 2 m/s are specific signs of a significant coronary
stenosis or restenosis.3,4
Unfortunately, there are no direct specific signs for the
color flow detection of coronary occlusion. In such cases,
reversed diastolic flow in coronary arteries depending
from retrograde filling by collateral circulation could
be recorded. This finding is very specific but offers low
sensitivity since collaterals may also provide anterograde
perfusion.5 Two-dimensional detection of coronary
luminal stenosis is usually not reliable in TTE.

Transesophageal Echocardiography
Higher ultrasound beam frequency (thus higher resolution) and anatomical proximity create opportunities for
improved imaging of proximal coronary arteries (especially
left) from the transesophageal window, including better
anatomical definition of possible lesions. TEE enables
visualization of proximal coronary arteries in more than
90% of studies and offers interpretable recordings of flow
in 85%, 65%, and 58% of LAD, Cx, and RCA , respectively.
As opposed to TTE, TEE allows the assessment of vessel
morphology including the identification of luminal
narrowing.6 Importantly, from the clinical point of
view, LMCA is evaluable in nearly in 100% for twodimensional and 88% for flow imaging by experienced
observer during TEE study.7 The technique of TEE
assessment of coronary ostia is based on the systematic

Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve

1339

Fig. 64.3: Additional image of the proximal left coronary artery in

modified apical five-chamber view; note red-coded LCA flow with
focal turbulence (stenotic sitearrow). (Ao: Aorta; LCA: Left coronary artery; RV: Right ventricle; LV: Left ventricle).

Fig. 64.4: Normal left main coronary artery with bifurcation visualized in transesophageal echocardiography (TEE). (Ao: Aorta; LA:
Left atrium; RVOT: Right ventricle outflow tract).

Fig. 64.5: Normal proximal right coronary artery visualized in twoand three-dimensional echocardiography including ostial en-face
views and flow recording. (Ao: Aorta; RA: Right atrium; RCA: Right
coronary artery; RVOT: Right ventricle outflow tract; LA: Left atrium).

Fig. 64.6: Normal left main coronary artery flow visualized in

transesophageal echocardiography (TEE). (Ao: Aorta; RVOT:
Right ventricle outflow tract; LCA: Left coronary artery; LA: Left
atrium).

visualization of left and right sinus of Valsalva in high

esophageal short-axis cross-sectional views (Movie clip
64.3) leading to the identification of LMCA dividing into
LAD and Cx (Fig. 64.4), and, usually in separate view, RCA
(Fig. 64.5). This approach allows the exclusion of coronary
artery anomalies. Usually coronary arteries visualization
is accomplished in color flow mode (Fig. 64.6; Movie
clip 64.4) with filter setting kept low to enhance low velocity
diastolic flow of the coronary arteries. Detection of focal
acceleration and aliasing is the typical, highly specific sign
of significant luminal stenosis (Figs 64.7 and 64.8). Nyquist
limit should be kept high at this stage to avoid false-positive
findings of flow acceleration coded by aliased signal. An

attempt to record flow with spectral Doppler should be

done, especially when abnormal color pattern is seen
(Movie clip 64.5), with LMCA and LAD being most feasible.
Measuring diameters is feasible mainly for LMCA and Cx
due to the perpendicular course versus TEE ultrasound
beam of LMCA to ultrasound beam in both TTE and TEE
examination allows its measurement in optimal linear
resolution.8 Beyond the search of stenosis, significant
dilatation of proximal segment of the coronary artery
may be diagnostic for the presence of fistula, aneurysm
(atherosclerotic or Kawasaki disease), or anomalous
origin of the coronary artery from the pulmonary artery
(e.g. WhiteBlandGarland syndrome). The progress in

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Figs 64.7A and B: Examples of stenosis in circumflex (Cx; A) and left anterior descending (LAD; B) detected by transesophageal echocardiography (TEE) color Doppler. (Ao: Aorta; RVOT: Right ventricle outflow tract; LCA: Left coronary artery; Cx: Circumflex artery; LAD:
Left anterior descending; LA: Left atrium).

color Doppler is close to 100% for LMCA, 95% for LAD,

75% for Cx with much less sensitivity for RCA .11

DISTAL CORONARY FLOW

AND CORONARY FLOW RESERVE

Fig. 64.8: Stenotic distal left main artery (LMA) with accelerated
flow spectrum recorded by transesophageal echocardiography
(TEE). (Ao: Aorta; RVOT: Right ventricle outflow tract; LA: Left
atrium; LCA: Left coronary artery).

three-dimensional echocardiography allows registration

of high-resolution data sets with unique ostial views
and improved tracking of the spatial course of proximal
coronary arteries9 (Figs 64.5 and 64.9). Multiplane features
of matrix probes are also valuable tools for imaging of
rapidly moving objects such as coronary arteries including
flow (Movie clip 64.3).
Twofold increase in coronary flow velocity (maximalto-prestenotic flow velocity ratio)10 or maximal flow
velocity above 1.5 to 2.0 m/s represents a specific threshold
for diagnosing significant coronary stenosis, consistent
with TTE. Specific velocity thresholds for TEE have also
been proposed (Table 64.1). The accuracy for detection of
proximal stenosis based on combined two-dimensional,

Due to improving Doppler technology, routine detection

of distal coronary flow (apical segments of major epicardial
coronary arteries) has become routinely feasible.13 Intravenous contrast agent injection may be useful to enhance
low-intensity flow signals.14
Usually, the detection of flow in distal LAD is achieved
in modified apical three-chamber view, usually in the
range of 20 to 50 cm/s (Fig. 64.10). Increased velocities
may occur if a stenotic situs is directly sampled, whereas
very low distal velocities (around 10 cm/s) with slow
deceleration were reported distally to critical stenoses
in LAD. Feasibility of distal LAD Doppler flow recording
is higher (9095%) than in RCA (6070%) or marginal
branches of Cx (main Cx flow registration is not feasible).15
Recording of coronary sinus flow allows insight in the
total coronary flow return and TEE approach has been
suggested for the diagnosis of LAD stenosis (abnormal
value < 2.0)16 or evaluation of microvascular dysfunction in
patients with diabetes with proposed cut-off values < 1.7.17
Distal post-stenotic diastolic-to-systolic velocity ratio
has been reported as an accurate approach to define
significant LAD or marginal branch stenosis (abnormal
cutoff < 1.68).18 Early postinfarction resting flow pattern in
distal LAD bed was described to predict remodeling and
transmural necrosis. Short deceleration time of diastolic

Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve

1341

Fig. 64.9: Proximal left coronary artery analyzed in three-dimensional echocardiography including ostial en-face views and multislice
imaging. (Ao: Aorta; AV: Aortic valve; RV: Right ventricle; LA: Left atrium; LCA: Left coronary artery).
Table 64.1: The Optimal Views for Proximal Segments of Coronary Arteries and Proposed Cut-Off Values for the Assessment of
Significant Stenosis

Coronary Artery

TTE-Optimal View

TEE-Optimal View

Proposed Cut-Off
Values11

Proposed Cut-Off
Values12

LMCA

High parasternal short-axis,

apical five-chamber

Short axis, 0 or 180, 12 cm

above aortic valve

> 1.23 m/s

> 1.40 m/s

LAD

High short-axis proximal and

directed toward the probe,
apical five-chamber

Short-axis distal and directed

from the probenear parallel
to Doppler beam

> 1.10 m/s

> 0.9 m/s

Cx

High short-axis distal and

directed from the probe

Short-axis proximal and

directed toward the probe

> 1.40 m/s

> 1.10 m/s

RCA

Parasternal long-axis,
parasternal short-axis

Short axis, slightly higher than

LMCA ostium and in the aortic
long-axis view

> 0.53 m/s

(Cx: Circumflex; LAD: Left anterior descending; LMCA: Left main coronary artery; RCA: Right coronary artery; TEE: Transesophageal
echocardiography; TTE: Transthoracic echocardiography).

coronary flow (600 ms) recorded 2 days after percutaneous

coronary intervention (PCI) in the distal part of LAD and
intramyocardial arteries predicted the lack of myocardial
viability after anterior wall infarction19 and worse clinical
outcomes.20 Finally, calculating distal to proximal diastolic
velocity ratio was attempted to improve the detection of
left coronary artery (LCA) stenosis (normal value < 0.5).21
Measuring resting values alone is subject to many
technical inaccuracies and thus velocity ratio approach
has become more popular. Technically, sample volume
of pulsed wave Doppler should be located distally
from the investigated stenosis (which can be localized
anywhere proximal to the measurement). The localization
of the sample volume in part proximal to stenosis may

provide false-negative results because of the presence

of normal side branches between the sampling site and
the stenosis. In clinical practice, coronary flow reserve
(CFVR) is usually assessed in LAD after flow detection
in dedicated color flow mapping protocols from apical
foreshortened three-chamber view (with probe located in
higher intercostal space). The evaluation of coronary flow
reserve (CFVR) in RCA requires recording of flow in distal
posterior interventricular artery from a modified apical
two-chamber view (Fig. 64.11).
Noninvasive evaluation of CFVR in echocardiography
allows the assessment of functional significance of the
stenosis in epicardial parts of coronary arteries and the
status of the microcirculation.22 Distal coronary flow

1342

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 64.10: Distal left coronary artery (LCA) flow recorded in color
and spectral Doppler, modified apical four-chamber view. (Ant IVS:
Anterior interventricular septum; LCA: Left coronary artery; LV: Left
ventricle).

Fig. 64.11: Distal right coronary artery (RCA) flow recorded in

color and spectral Doppler, modified apical two-chamber view.
(RCA: Right coronary artery; LA: Left atrium).

Table 64.2: The Conditions Decreasing CFR in the Absence of Significant Stenoses in Epicardial Arteries

Hypertrophic cardiomyopathy
Hypertrophy in arterial hypertension
Aortic stenosis
Aortic insufficiency
Dilated cardiomyopathy
Diabetes
Syndrome X
Increased blood viscosity: policythemia, macroglobulinemia
Hypercholesterolemia
For some of these conditions (e.g. aortic valve disease, hypertension, hypercholesterolemia) surgical or medical treatment could
reverse coronary flow reserve (CFR) impairment.10

becomes abnormal in cardiac vascular or myocardial

disease and resting values have been recently proposed.
Relationship of peak diastolic flow velocity during
vasodilatory challenge (in practiceusually intravenous
dipyridamole or adenosine, which act on precapillary
resistance vessels to maximize flow) to resting flow velocity
allows to calculate CFVR, closely corresponding with the
values provided with intracoronary Doppler measurements and useful to define physiologically meaningful
stenosis in LAD and RCA.23
The conditions impairing CFVR values despite normal
epicardial arteries in invasive angiography are listed in
Table 64.2.
CFVR is defined as the ratio of maximal (or mean)
velocity of coronary flow measured during vasodilatation
(which may be induced medically by dipirydamole,
adenosine, dobutamine, or papaverine infusion or by

exercise) to the resting or baseline flow velocity. In practice,

the assessment of CFVR is more difficult in the setting of
exercise or during dobutamine test (because of tachycardia
and increased respiratory motion), and the two methods
of choice are studies with dipirydamole (0.84 mg/kg, iv)
or adenosine (0.14 mg/kg/min, iv) infusions. Normal
values for CFVR range from 3 to 5 and values below
2 confirm the physiological significance of coronary
artery stenosis, usually with luminal narrowing > 70%
coronary artery stenosis or indicate other conditions
impairing CFVR24,25 (Figs 64.12 and 64.13). The assessment
of CFR may help in classification of intermediate stenosis
(4070%) to the invasive treatment.
CFVR decreases with age, but may exceed 5 in selected
groups of patients such as young healthy athletes.26
CFVR also show transmural dispersion, related to greater
extravascular component of microcirculatory resistance

Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve

1343

to impair CFVR34 and in long-term, an improvement in

CFVR was recorded (invasively by intracoronary Doppler)
4 months after stem cell therapy in reperfused acute
myocardial infarction comes from REPAIR-AMI trial.35 In
the group of 30 patients treated by intracoronary bone
marrow cells infusion, coronary flow reserve improved
from 2.0 0.1 to 3.8 0.2, as P < 0.001 as compared to
controls (change 1.9 0.1 to 2.8 0.2).

CONGENITAL ABNORMALITIES OF
THE CORONARY ARTERIES
The prevalence of coronary arteries anomalies ranges
from about 1 to 5.6% and the most frequent anomaly is the
Cx branch originating from the RCA or the right sinus of
Fig. 64.12: Normal coronary velocity flow reserve measured in
3639
distal left anterior descending (LAD)coronary flow velocity Valsalva, found in 0.48% of the invasive angiographies.
The clinical presentation may be silent or they may cause
reserve (CFVR) = 3.0.
angina, arrhythmia, syncope, myocardial infarction, and
40
in subendocardial layer, with higher CFVR values in sudden cardiac death. Some anomalies of coronary
subepicardial than subendocardial regions.27 The value arteries origin including the LMCA arising from the right
of adding CFVR (usually LAD) to wall motion assessment sinus of Valsalva (although less common than the origin of
lies in improved sensitivity without specificity loss.28 RCA from the left sinus of Valsalva (Fig. 64.14), may have
CFVR is recommended as a component of state-of-the- fatal consequences related to slit-like orifice narrowing,
art standard stress echo protocol with dipyridamole and sharp angulation, and risk of intra-arterial course between
is most practical when clinical questions regarding the the aorta and pulmonary artery, with threatened sudden
specific anatomical locations arise. Recently published cardiac death during exercise. The diagnosis of coronary
study has shown high accuracy of coronary flow reserve anomalies by echocardiography has been overshadowed
< 2 assessed by TTE for the detection of significant by magnetic resonance or computed echocardiography;
restenosis after stent implantation, again with cutoff value however, it remains a useful option, is radiation-free,
< 2. 0 for three major coronary arteries.29 The diagnostic and allows real-time assessment of coronary anatomy
value of TTE coronary flow reserve is high and similar to and flow. While TTE remains challenging in some cases,
TEE images usually unveil realistic coronary anatomy.
320-row computed tomography.30
The impairment of coronary flow reserve with cut- Such composite diagnostic approach, including invasive
off value < 1.7 assessed during the 24-hour period after coronary angiography or intracoronary ultrasound as
primary coronary intervention was also documented necessary, may be necessary to define the exact course of
as the predictor of left ventricular remodeling early coronary arteries, elucidate possible pathomechanisms
after anterior myocardial infarction.31 Decreased aiding in therapeutic decision-making. This may be
coronary flow reserve < 2.6 with shortened < 840 ms especially valuable when overlap of congenital and
diastolic deceleration time were also related to higher acquired atherosclerotic coronary disease comes into
incidence of cardiac events in patient after heart play.41 Identification of aberrant left coronary artery with
transplantation, defined as cardiac death, heart failure, interarterial course alone represents an indication for
and stent implantation.32 Reduced long-term survival surgical intervention, whereas in anomalous origin of the
was also described in patients with coronary flow reserve RCA more percutaneous interventions may be considered.
< 2 in dilated cardiomyopathy together with such known Finally, echocardiography may provide the valuable
predictors as increased wall motion score index (WMSI) noninvasive and radiation-free tool for the follow-up and
monitoring of coronary intervention results.42
and mitral regurgitation.33
Coronary fistulas are uncommon coronary pathology.
In the context of intracoronary stem cell therapy, early
injection in the infarct-related coronary bed does not seem Pediatric type features low-resistance connection of the

1344

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 64.13: Low coronary velocity flow reserve measured in distal left anterior descending (LAD)coronary flow velocity reserve (CFVR)
= 1.7 in a hypertensive diabetic patient free of epicardial coronary disease.

Fig. 64.14: Coronary anomalyright coronary artery (RCA) originating from the left sinus of Valsalva with interarterial course. Mild flow
abnormality (turbulence) visualized in transesophageal echocardiography (TEE). (RCA: Right coronary artery; RVOT: Right ventricle
outflow tract).

dilated coronary artery with cardiac chamber, which can

be imaged using color Doppler, usually in the right side
of the heart. TTE and TEE offer diagnosis by visualization
of marked arterial dilatation and tortuosity, with multiple
cross-sections of a winding vessel in select views with
detectable diastolic flow pattern (Fig. 64.15). In adults,
tiny fistulous connections (usually LADpulmonary
trunk) are accidentally detected in coronary angiography
and can be validated by detecting flow using color
Doppler (Fig. 64.16) The echocardiographic visualization
of coronary aneurysms and fistulas has been reported
also in iatrogenic complications related to percutaneous
coronary interventions.43

SUMMARY
The evaluation of coronary arteries by modern echocardiography enables routine noninvasive detection of
congenital anomalies and significant proximal stenosis,
especially in LMCA, which underscore the role of this
method as noninvasive and valuable screening tool in
these frequently dangerous setting. Functional assessment
of coronary stenosis by noninvasive CFVR contributes to
physiological stratification of luminal coronary narrowing,
and echocardiogram may support monitoring of coronary
artery interventional treatment, offering additional
prognostic information.

Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve

1345

Fig. 64.15: Pediatric type coronary fistula from the right coronary
artery to the right atrium. (RV: Right ventricle; RCA: Right coronary
artery; LV: Left ventricle).

Fig. 64.16: Minor left anterior descending (LAD) fistulous connection to the proximal pulmonary trunk (diastolic color flow visible) with
corresponding angiogram. (Ao: Aorta; RVOT: Right ventricle outflow tract; MPA: Major pulmonary artery; LA: Left atrium; LAD: Left
anterior descending; LCA: Left coronary artery).

Thus, TEE can detect coronary lesion in prognostically

critical proximal locations at no added cost. Incorporation
of assessment of coronary arteries in routine protocol of
TEE study can be therefore recommended, and the field of
diagnostic approaches based on transthoracic imaging is
expanding. with evidence of clinical benefits, for example,
in patients studied due to cryptogenic embolism.44

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atherosclerotic coronary disease using transthoracic
Doppler echocardiography. Arch Med Sci. 2012;8(1):1625.
42 Wierzbowska-Drabik KA, Peruga JZ, Plewka M, et al.
Nonatherosclerotic ostial stenosis of left main coronary
artery: echocardiographic assessment and follow-up after
surgical treatment. Echocardiography. 2006;23;1336.
43. Lipiec P, Peruga JZ, Krzeminska-Pakula M, et al. Right
coronary artery-to-right ventricle fistula complicating
percutaneous transluminal angioplasty: case report and
review of the literature. J Am Soc Echocardiogr. 2004;
17(3):2803.
44. Voros S, Nanda NC, Samal AK, et al. Transesophageal
echocardiography in patients with ischemic stroke
accurately detects significant coronary artery stenosis
and often changes management. Am Heart J. 2001;142(5):
91622.

CHAPTER 65
Echocardiography in Hypertrophic
Cardiomyopathy
Dan G Halpern, Mark V Sherrid

Snapshot

Defini ons and Loca ons of Hypertrophy

Le Ventricular Ou low Tract Obstruc on

Dieren al Diagnosis

INTRODUCTION
Hypertrophic cardiomyopathy (HCM) is a genetic disorder
with clinically unexplained myocardial hypertrophy (most
commonly of the interventricular septum) that occurs in
the absence of a hemodynamic cause. HCM predisposes
to symptoms, dynamic left ventricle obstruction, and
infrequently, to life-threatening arrhythmias.1 It is the most
common inherited disorder among cardiovascular diseases
(1:500) and is the leading cause of sudden cardiac death
(SCD) in young adults.1 Among its previous names are
idiopathic hypertrophic subaortic stenosis and muscular
subaortic stenosis. The preferred nomenclature is HCM,
either obstructive, or nonobstructive. HCM is inherited with
autosomal dominant transmission; currently mutations in
11 genes coding for various cardiac sarcomeric proteins are
associated with HCM.2,3 Varying phenotypic expressions
and marked heterogeneity is a hallmark of HCM (Figs 65.1A
to D). Microscopically, HCM is characterized by myocyte
hypertrophy and myocytic disarray interlaced with fibrosis.
Transthoracic echocardiography (TTE) is the most
powerful tool for the diagnosis, management, and followup of HCM.4 TTE demonstrates the site and extent of
hypertrophy, and delineates and quantifies obstruction.
Before making the diagnosis, it is imperative to rule out
more common secondary causes of concentric hypertrophy

Treatment Strategies in Hypertrophic Cardiomyopathy

Surgical Septal Myectomy

Dynamic Systolic Dysfunc on

such as uncontrolled hypertension or aortic valve stenosis.

As discussed below, elite young athletes may have mild
degrees of hypertrophy that must be distinguished from
HCM. All TTE modalities are employed: M-mode and
two-dimensional (2D) imaging, spectral, continuous
wave (CW), and tissue Doppler to evaluate: (a) location
of hypertrophy, quantitative estimation of wall thickness;
(b) detection and, if necessary, provocation of systolic
anterior motion (SAM) of the mitral valve, anatomy of
the mitral apparatus, and degree of mitral regurgitation
(MR); (c) Doppler of left ventricular outflow tract (LVOT)
velocities or mid-LV gradients at rest and after provocation;
(d) evaluation of diastolic dysfunction; and (e) pulmonary
arterial pressure.
Echo also impacts the family of an HCM patient. Since
HCM is a genetic disease, family members of diagnosed
HCM patient should be screened. Annual echocardiographic surveillance should include all first degree
relatives of the patient until the age of 21, and afterward
every 5 years. Screening under the age of 12 years is
optional unless there are suspicious symptoms of HCM or
malignant family history of premature death.1 Genotype
analysis for screening or confirmation of HCM is most
beneficial when there is a positive family history; here, 50%
have an HCM-associated mutation, whereas in sporadic
cases only 3040% have an HCM-associated gene.3

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

1349

Figs 65.1A to D: Major patterns of hypertrophy in hypertrophic cardiomyopathy (HCM). Schematic (left panel) versus echocardiographic
images (right panel). (A) Anterior septal hypertrophy; (B) Subaortic septal bulge; (C) Apical; (D) Midleft ventricular with obstruction.
Source: Reproduced in part with permission from Shah A et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy.
Echocardiography. 2009;26:92233.

Figs 65.2A to D: Spectrum of sub-basal hypertrophic

cardiomyopathy (HCM). (A) shows pure apical HCM; (B) shows
apical HCM with some extension to the mid-LV; (C) shows apical
and mid-HCM with severe encroachment of the LV cavity resulting
in a small slit-like left ventricle (LV) cavity in diastole, but no LV
obstruction and no apical akinetic chamber; (D) shows mid-LV
HCM with mid-LV obstruction and an apical akinetic chamber.
Source: Reproduced with permission from Shah A, et al. Severe
symptoms in mid and apical hypertrophic cardiomyopathy.
Echocardiography. 2009;26:92233.

DEFINITIONS AND TYPES OF

HYPERTROPHY
Hypertrophy is defined to as end diastolic wall thickness
12 mm and HCM may be considered when wall thickness

is 15 mm in the absence of hemodynamic cause for

the hypertrophy observed. There is great phenotypic
variation in the location and magnitude of hypertrophy.
Several common patterns of hypertrophy in HCM are
depicted in Figures 65.1A to D. The most common pattern
of hypertrophy is of the anterior and posterior septum,
and often of the anterior wall. Other distributions are
thickening restricted to the proximal portion of the septum
that is referred to as discrete subaortic septal bulge;
HCM that spares the base but only involves the mid and
apical segments has been referred to as sub-basal HCM.5
Varieties of sub-basal HCM are shown in Figures 65.2A
to D. Among these are apical HCM, midleft ventricular
thickening with severe encroachment of the LV cavity,
and mid-LV obstruction with an apical akinetic chamber.
Rarely, thickening is restricted to the anterior, posterior,
or lateral walls.6 On occasion, the right ventricle (RV)
may be thickened, and rarely obstructs the subvalvular
RV outflow tract.7 The pattern of hypertrophy may be
useful to predict if an individual patient will have positive
testing for a HCM-related mutation. Patients with septal
hypertrophy that extends all the way to the apex with a
resulting crescent-shaped ventricular cavity with reversal

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 65.3: Systolic anterior motion (SAM) of the mitral valve. SAM
of the mitral valve, drawn from an apical five-chamber view, as it
proceeds in early systole.
Source: Reproduced with permission from Sherrid MV, et al. An
echocardiographic study of the fluid mechanics of obstruction
in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1993:22;
81625.

of the normal apical concavity had a 79% probability of an

HCM-associated mutation, as compared to 8% of those
with a discrete subaortic septal bulge.8
The maximum wall thickness, most commonly found
in the septum, is a prognosticator for SCD in HCM, as a
septal thickness of <19 mm denotes a lower incidence of
sudden death, whereas 30 mm alone indicates nearly
a 2% per year risk of SCD.9 In patients with high risk for
SCD the physician should discuss the benefits and risks
of implantable cardioverter defibrillator implantation and
consider implantation of patients at high risk.10 Maximum
wall thickness is thus an important variable in HCM and
should be reported in every patient. The septum is best
visualized in the parasternal long-axis and short-axis views
and attention should be given on the short axis to not
include the RV moderator band or anomalous papillary
muscles. In patients with inadequate windows due to
body habitus, pulmonary disease, or atypical pattern of
distribution of hypertrophy, cardiac magnetic resonance
(CMR) imaging may aid in delineating wall thickness.11 It
is also invaluable when the magnitude of wall thickening is
ambiguous and there is doubt whether 30 mm thickening
is present or not.

Left Ventricular Outflow Obstruction and

Systolic Anterior Motion of the Mitral
Valve
While one quarter of HCM patients have LVOT obstruction
at rest, two thirds of patients exhibit obstruction at rest or

after physiological provocation, including exercise.1218

LVOT pressure gradients 30 mm Hg at rest are associated
with decreased survival.17,19 Patients who are not obstructed
at rest but who develop LVOT obstruction after provocation
are referred to as having latent obstruction.13 LVOT
dynamic obstruction causes increased LV work, decreased
diastolic aortic perfusion pressure, increased supply
demand ischemia, load-related impairment in diastolic
relaxation, and a midsystolic drop in instantaneous LV
ejection flow velocities and volumetric flow.12,18,20,21
The most common cause of LVOT obstruction is SAM
of the mitral valve with mitralseptal contact (Figs 65.3
to 65.5, Movie clips 65.1 to 65.3). The first demonstration
of SAM was made with M-mode through the mitral valve
by Shah et al. in 1969, revolutionizing understanding
of the obstruction mechanism.22 Previously, surgical
observations and cardiac catheterization suggested that
LVOT obstruction was caused by a subaortic sphincter
similar to that found in the right ventricular outflow tract
in congenital heart disease. There are several anatomical
features in HCM that permit and predispose to SAM
septal hypertrophy, anterior displacement of the mitral
papillary muscles, and elongation of the mitral leaflets
and chordae.12,16,23,24 These geometricanatomical features
allow ejection flow to get behind the mitral leaflets, and
sweep them into the septum. Flow drag, the pushing force
of flow, plays the dominant role causing SAM,12,16,2527 while
the Venturi effect, suctioning from the LVOT tunnel has
a minor contribution. The septal bulge redirects ejection
flow so that, in the apical 3-chamber view, it comes from
a relatively posterior direction and catches the anteriorly
positioned mitral leaflets (Figs 65.4 and 65.5). The anterior
mitral leaflet is most often elongated.23 In most patients,
both leaflets move anteriorly and participate in SAM.
However, in some patients it is the posterior leaflet that
extends beyond the coaptation point and obstructs.28 SAM
often begins during isovolumetric systole but may begin
later after the aortic valve opens.16,25
As systole progresses, pushing forces displace the
mitral valve anteriorly with increase in drag as the angle of
attack between the leaflet and the ejection flow increases.26
With mitralseptal contact, which may occur from early to
late systole, a dynamic gradient is created across the LVOT.
The pressure difference across the mitral valve pushes the
valve leaflets further into the septum, further decreasing
the orifice and creating higher pressure gradients.13,25
Obstruction begets more obstruction. The narrowing
orifice raises the pressure difference; the rising pressure

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

1351

Figs 65.4A and B: The pushing force of flow. (A) The dark blue, low-velocity flow behind the mitral valve pushes the leaflets
into the septum, before high-velocity flow has occurred in the outflow tract; (B) The spinnaker is pushed by the wind that strikes
its undersurface. Image of Stanley Rosenfeld reproduced with permission, The Rosenfeld Collection, Mystic Seaport Museum.
Source: Reproduced with permission from Sherrid MV, et al. Pathophysiology and treatment of hypertrophic cardiomyopathy. Prog
Cardiovasc Dis. 2006;49:12351. (LA: Left atrium; LV: Left ventricle; MV: Mitral valve; LVOT: Left ventricular outflow tract.

Figs 65.5A and B: Early in systole flow drag is the dominant

hydrodynamic force on the mitral leaflets (A). After mitralseptal
contact, the pressure difference (gradient) is the force that pushes
the mitral leaflet further into the septum (B). Source: Figure
modified and reproduced with permission from Sherrid MV, et al.
Systolic anterior motion begins at low left ventricular outflow tract
velocity in obstructive hypertrophic cardiomyopathy. J Am Coll
Cardiol. 2000;36:134454.

difference further narrows the orifice. The longer in systole

that the mitral valve touches the septum, the higher the
gradient29 (Figs 65.6 and 65.7).
Obstruction may be thought of as a tug-of-war
between the anteriorly displacing force of flow and
posterior restraint by the chordae and papillary muscles.
Pharmacological treatment with negative inotropes
decrease LV ejection acceleration and thus the pushing
force on the mitral valve.30 Flow drag is related to the square
of the flow velocity, so even small changes in acceleration
and velocity will have a large change in the pushing force.
With negative inotropes and a decrease of force on the
valve, the equilibrium is moved toward posterior restraint,
mitralseptal contact is delayed, and pressure gradients
are reduced or eliminated.
Echocardiographic evaluation of SAM and LVOT
obstruction includes M-mode 2D, and Doppler imaging.
Because of its high temporal resolution, M-mode through
the mitral valve is very useful for diagnosing and timing
of SAM and observing the duration of mitralseptal

1352

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 65.6: Four separate cardiac cycles from a patient, displaying

simultaneous echocardiographic and hemodynamic tracings during
pharmacological manipulation of the pressure gradient by increasing
doses of isoproterenol. Systolic anterior motion (SAM) without septal
contact in the first systole is not associated with a significant pressure
gradient. When SAMseptal contact first develops late in systole,
it is brief and the pressure gradient is low (second systole). When
SAMseptal contact develops early in systole, it is prolonged and the
pressure gradient is high (fourth systole with higher dose isoproterenol).
Source: Reprinted with permission from Pollick C, et al. Muscular
subaortic stenosis: the quantitative relationship between systolic
anterior motion and the pressure gradient. Circulation. 1984;69:439.

contact (see Fig. 65.6). In addition, M-mode through the

aortic valve displays midsystolic closure or notching that
correlates with the aortic bisferiens pulse. Partial closure
of the aortic valve in midsystole occurs due to a transient
fall of flow from the obstruction (Fig. 65.8).
The best 2D view to assess SAM and measure LVOT
gradients is the apical 3-chamber view where the Doppler
beam is the most parallel to the LVOT blood flow and
can be directed anteriorly and medially away from
the left atrium to avoid confusion with MR (Figs 65.7
and 65.9). The LVOT jet is narrower (of shorter duration)
than MR because it does not include isovolumetric
systole and isovulumetric diastole. Color Doppler shows
aliasing in the LVOT and CW Doppler shows typical late
peaking velocities. The contour of the Doppler CW jet is
concave to the left (dagger-shaped) because the LVOT
orifice continues to narrow in systole. This continuing
acceleration pattern is distinct from the aortic stenosis
where the jet is convex to the left with decreasing
acceleration, because the orifice is fixed in systole
(see Fig. 65.7). Careful differentiation of the LVOT jet and
the MR jet cannot be overemphasized. Contaminated jets
are frequent, and lead to overestimation of the true gradient.

Fig. 65.7: Top: Continuous wave (CW) Doppler echocardiographic

tracing through the obstructing orifice in the left ventricular outflow
tract (LVOT) of a patient with a gradient of 64 mm Hg. The contour
after the inflection point is concave to the left because of the
progressive decrease in the size of the orifice formed as the mitral
valve is pushed by the rising pressure gradient into the septum.
Reproduced with permission from Sherrid MV, et al. Reflections
of inflections in hypertrophic cardiomyopathy. J Am Coll Cardiol.
1993;54:2129. Bottom: Comparison of the Doppler velocity tracings
of the high-velocity jets of aortic stenosis, mitral regurgitation (MR),
and obstructive hypertrophic cardiomyopathy (HCM). In aortic
stenosis and MR, as velocity increases, acceleration decreases. In
contrast, in obstructive HCM, as velocity increases, acceleration also
increases. In obstructive HCM, the rising pressure difference forces
the mitral leaflet against the septum, which decreases the orifice
size and further increases the pressure difference. This amplifying
feedback loop explains the concave contour seen in obstructive
HCM. The orifice size changes as an inverse function of the pressure
difference across the stenosis, with the pressure difference itself
causing an increase in narrowing. Progressive orifice narrowing also
explains why the jet peaks late in systole in obstructive HCM.
Source: Reprinted with permission from Sherrid M. Mid-systolic
drop in left ventricular ejection velocity in obstructive hypertrophic
cardiomyopathythe lobster claw abnormality. J Am Soc
Echocardiogr. 1997;10:70712.

It is important to determine what is the mechanism

producing obstruction for accurate surgical planning.
Although the SAM of the leaflets of the mitral valve is most
commonly the obstructing culprit, the papillary muscles
may obstruct instead (Figs 65.10A to C).
In patients without resting gradients across the LVOT,
provocative maneuvers should be performed in order
to unmask obstruction because treatment of symptoms
in HCM is heavily dependent on finding a gradient
(Fig. 65.11). While there are many advanced treatments
for gradient that relieve symptoms, there are very few, if
any, treatments to improve symptoms in nonobstructed

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

Fig. 65.8: M-mode through the aortic valve depicting midsystolic

closure of the aortic valve, correlating with midsystolic fall in
ventricular ejection velocity and flow.

patients. Any decrease in preload, or afterload, or increase

in contractility increases obstruction; thus, standing,
Valsalva maneuver, the postprandial state, or imaging
after exercise may reveal latent obstruction.13,3134 Amyl
nitrite and dobutamine are nonphysiological means to
provoke gradient and thus must be viewed as suspect
since the change in load they produce may not exist in
daily life. Medications commonly used in cardiology that
decrease preload such as nitrates, high-dose diuretics, or
that decrease afterload such as angiotensin-converting
enzyme blockers and dihydropyridine calcium channel
blockers promote LVOT obstruction. These agents can be
thought of as provocative of gradient.35
Different modalities of provocation should be used
in order to ascertain LVOT obstruction. Standing and the
straining phase of the Valsalva maneuver, both decrease
venous return to the heart, reduce preload, and promote
obstruction. In one third of patients, standing produces
higher gradients than Valsalva.13 Inability to demonstrate
obstruction by these simple maneuvers warrants exercising
the patient.13-15,3638 Exercise echocardiography may provoke
SAM and obstruction, and provides functional information
including exercise tolerance, blood pressure response,
symptoms, and ischemia detection. Microvascular disease
in HCM may provoke wall motion abnormalities that are
not distinguishable from large epicardial coronary disease
without coronary angiography. Dobutamine promotes
obstruction but is nonspecific and should not be used to
diagnose obstruction in HCM. Another useful modality is
postprandial exercise testing, where the patient is imaged

1353

Fig. 65.9: Differentiating between the continuous wave (CW) left

ventricular outflow tract (LVOT) jet and mitral regurgitation (MR)
jet in obstructive hypertrophic cardiomyopathy (HCM). The panel
on the left is the outflow jet alone showing the typical concaveto-the-left contour. The panel on the far right shows the MR jet
that has a longer duration, and is continuous with the mitral inflow
velocities shown above the baseline. The MR has higher velocity
than the LVOT jet. The middle panel shows an overlapping
LVOT/MR signal obtained with beam in an intermediate position.
(Ac: Aortic closure; Ao: Aortic opening; Mc: Mitral closure).
Source: Reproduced with permission from Yock PG, et al. Patterns
and timing of Doppler-detected intracavitary and aortic flow in
hypertrophic cardiomyopathy. J Am Coll Cardiol. 1986;8:104758.

around 1 hour after a moderate meal. The mesenteric

vasodilatation creates a decrease in afterload.39
It is crucial to understand the pathophysiology of SAM
when preparing for surgical correction of the obstruction
as the operative technique should be tailored individually
from patient to patient. The extent of septal hypertrophy,
the position of the papillary muscles, and slack of the
anterior or posterior mitral valve leaflets may significantly
vary in their contribution to SAM.12,40 As mentioned
above, a hypertrophied or anomalous papillary muscle
inserting into the midanterior mitral valve leaflet without
intervening chordae may be the reason for obstruction
inadequate depth of septal resection or failure to partially
or completely resect the anomalous papillary muscle may
leave the patient with persistent obstruction (Fig. 65.10).41
Conversely, dynamic LVOT obstruction has also been
reported in conditions other than HCM such as acute
coronary syndrome (ACS) with apical ballooning due to left
anterior descending infarct, Takotsubo syndrome, postaortic valve replacement, concentric hypertrophy with
hypovolemia, mitral valve apparatus abnormalities, and
during positive inotropes use.4246 Careful history taking,

1354

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 65.10A to C: Anomalous papillary muscle inserting in the middle of the anterior mitral valve leaflet and causing left ventricular
outflow tract (LVOT) obstruction. Left panels show the obstructing papillary muscle on parasternal (A) and short-axis views (B)
and the anatomical specimen. Center (A,B,C) and right panels (A,B) show inadequate resections in patients with this pathology
(center C and right B). Because the septal resections have not been carried far enough down the septum, and because the papillary
muscle abnormalities have not been addressed, there is still severe residual obstruction shown by the arrowheads in both cases.
Source: Reproduced with permission from Klues HG, et al. Anomalous insertion of papillary muscle directly into anterior mitral leaflet in
hypertrophic cardiomyopathy. Significance in producing left ventricular outflow obstruction. Circulation. 1991;84:118897, and Maron
BJ, et al. Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction
due to anomalous papillary muscle. Circulation. 1998;98:25058.

electrocardiography, and echocardiographic assessment

are required in order to rule out these pathologies where
the treatment strategy is fundamentally different.

Mitral Apparatus and Regurgitation

In HCM patients, the papillary muscles are anteriorly
displaced in the LV and the mitral leaflets or chordae are
elongated.16,23,47 This prepositioning predisposes to SAM.

MR is commonly seen during LVOT obstruction due to

malcoaptation of the mitral valve leaflets. The MR jet is
directed posteriorly; its severity varies and is maximal
during peak SAM. The MR velocity is the highest velocity
recorded by CW Doppler and its tracing is rounded
and symmetrical; in contrast, the LVOT obstruction
Doppler contour is late peaking and concave to the left
(see Figs 65.7 and 65.9). MR velocity traces begin in
isovolumetric systole and extend into the isovolemic

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

1355

Fig. 65.11: Patient with obstructive hypertrophic cardiomyopathy and latent obstruction. There is no left ventricle (LV) outflow gradient at rest. Gradient rises to 49 mm Hg after Valsalva, to 74 mm Hg after standing, and to 144 mm Hg after treadmill
exercise. In 30% of cases, we found a higher standing than Valsalva gradient, as in this patient. Of the 56 patients (57%)
with resting gradients < 30 mm Hg, standing provoked a gradient
30 mm Hg in 23 patients (41%), thus placing them in the
domain of obstructive HCM. Standing gradient is recommended on the index echocardiogram in every patient with HCM or SAM.
Source: Reproduced with permission from Joshi S, et al. Standing and exercise Doppler echocardiography in obstructive hypertrophic
cardiomyopathy: the range of gradients with upright activity. J Am Soc Echocardiogr. 2011;24:7582.

relaxation period and thus are wider than LVOT gradient

velocities. The LVOT gradient is usually quantified directly
from the CW jet (4V2) and this modality is most often
employed.48 However, it is also possible to calculate it
from the MR velocity and the patients systolic blood
pressure (SBP) by using the modified Bernoulli equation:
LVOTgradient(mm Hg) = [4(MRvelocity)2 + 10] SBP, where the
left atrial pressure is assumed to be 10 mm Hg.
Operations or pharmacological therapy that relieve
SAM and LVOT obstruction reliably improve or completely
eliminate MR when it is due to SAM. Current surgical

techniques recognize the importance of the distortion

of the mitral valve apparatus contributing to the SAM,
LVOT obstruction, and secondary MR. Thus, to thoroughly
eliminate SAM in selected patients, in addition to septal
myectomy, further procedures are directed at the mitral
valve: (a) papillary muscle release or reposition49,50 and
(b) mitral anterior leaflet plication that shortens and stiffens
the anterior leaflet.5153 Thus, mitral valve replacement
is uncommonly necessary for pure obstructive HCM.
However, it is important to recognize that MR can be due
to structural degenerative non-HCM abnormalities of the

1356

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

valve such as calcification or prolapse, and not to SAM. A

clinical pearl is that the MR in these cases is often central
or anteriorly directed. In these patients if severe MR is
present, mitral valve replacement is usually necessary.

Diastolic Dysfunction
Impaired relaxation is widely present in HCM as well as
impaired compliance from myocardial fibrosis.54,55 Global
and segmental assessment of diastolic function should
be assessed by measurement of the transmitral inflow
velocities, tissue Doppler velocities and pulmonary vein
flow velocities, as well as left atrial size. Tissue Doppler
velocities are decreased in HCM.56 However, the E/e' ratio
has limited application in HCM and does not correlate well
with LV filling pressures.54 The thickened septum usually
has the greatest segmental diastolic dysfunction.57 The
presence of a transmitral A-wave should also be noted. Its
absence could denote atrial fibrillation or atrial stunning,
which might be an important source of symptoms in
patients with restrictive physiology. Furthermore, tissue
Doppler imaging in a gene-positive HCM relative with
normal wall thickness might denote latent subclinical
cardiomyopathy by demonstrating reduced systolic
(S') and early filling (e') velocities.58 Strain imaging may
provide additional information on segmental dysfunction
and latent myopathy.59 In patients with LVOT obstruction,
the relief of the obstruction improves diastolic relaxation.60
Otherwise, no pharmacological therapy has been shown
convincingly to directly improve diastolic function in
HCM.61,62

Apical Hypertrophic Cardiomyopathy

Apical HCM, first described by the Japanese, is more
prevalent in the Asian population and consists of significant
hypertrophy at the apex; the base of the LV is spared.5,63
This variant has a typical electrocardiogram exhibiting
deep symmetrical T-wave inversion in leads V2-6 with
apical hypertrophy and a spade-shaped LV cavity
(Figs 65.2A to D). Because of the distribution of the
hypertrophy, there is far less incidence of obstruction.
Its prognosis is better than classic septal HCM but is still
infrequently associated with arrhythmias and SCD.64
Previously, apical aneurysms were thought to accompany
apical HCM; however, aneurysms are most often a product of
mid-LV obstruction in which chronic trapping of blood
at the apex, and high systolic and diastolic pressures
there, lead to aneurysm formation via the mechanisms

of supplydemand ischemia and afterload mismatch.5

This pathology is best imaged by the administration of
intravenous echo contrast; in the United States, Definity or
Optison are used, whereas in India, Definity or Sonovue are
used. Consequently, we administer intravenous contrast
in all patients with apical or apical-mid HCM. CMR also
may aid in diagnosing apical aneurysm.65

MIDLEFT VENTRICULAR
HYPERTROPHIC CARDIOMYOPATHY
An uncommon but clinically important form of HCM has
a unique midventricular distribution of hypertrophy,
and in many patients subsequent midcavity obstruction
(see Figs 65.2A to D).5,66,67 Mid-LV hypertrophy may be
recognized when the extent of the hypertrophy is
greatest at the level of the papillary muscles. The
highest midventricular gradients are usually recorded
at the level of the hypertrophied papillary muscles.
The triad of mid-LV hypertrophy, small LV cavity, and
hypertrophic papillary muscles predispose obstruction
as the LV comes into apposition with opposite wall
and the papillary muscles.66 Mid-LV obstruction may
occur on its own without development of an apical
akinetic chamber; many investigators believe that mid-LV
obstruction leads over time to apical aneurysm through
the mechanisms of afterload mismatch and supply
demand ischemia, ballooning, scar formation, and
thinning (see Figs 65.12 and 65.13). Contrast imaging is
helpful for demonstrating the obstruction together with
the secondary apical chamber.68 An invaluable clue in
diagnosing midventricular HCM and an apical akinetic
chamber is the detection of diastolic paradoxical jet flow
(Fig. 65.13).69 The jet is paradoxical because it originates
from the apex in early diastole and travels backward
toward the LV chambernormally diastolic flow travels
toward the apex from the mitral valve. As with apical HCM,
contrast is essential for diagnosis and should be given in
every case, and CMR is also useful for the detection of the
apical chamber.

Systolic Dysfunction and Hypertrophic

CardiomyopathyChronic Irreversible
Verus Dynamic
Burnt out HCM with severe irreversible systolic dysfunction is seen in a small minority (approximately 2%)
of patients and is associated with increased morbidity

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

1357

Fig. 65.12: Two hypertrophic cardiomyopathy (HCM) patients with midleft ventricle (LV) obstruction and apical akinetic
chambers. In both cases, diastole is on the left and systole is on the right. The thin long arrows show the mid-LV hypertrophy.
The arrowheads show the mid-LV obstruction. The thicker long arrow shows the apical akinetic chambers. In the patient below,
echocardiographic contrast has been given to enhance visualization of the apical akinetic chamber. (LA: Left atrium; LV: Left ventricle).
Source: Reproduced with permission from Shah A et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy.
Echocardiography. 2009;26:92233.

Fig. 65.13: Paradoxical jet flow in midleft ventricular (LV)

obstruction with an apical akinetic chamber. Five-chamber color
Doppler view of the LV in early to mid diastole. Jet from the apex
is termed paradoxical because it courses from the apex toward the
mitral valve, at the same time as transmitral filling, below, the red
flow. There are multiple aliasing shells in the apical flow, indicating
it is high velocity. Blood is trapped in the apex during systole due
to the mid-LV obstruction, only to emerge upon relaxation of the
obstructing neck in diastole. Paradoxical jet flow should be looked
for in every patient with apical LV thickening, because it is an
important sign of a hidden apical akinetic chamber. When such flow
is detected, the routine administration of echocardiographic contrast
will readily show the apical akinetic chamber. (AC: Apical chamber;
LA: Left atrium; LV: Left ventricle. Source: Reproduced with
permission from Shah A, et al. Severe symptoms in mid and apical
hypertrophic cardiomyopathy. Echocardiography. 2009;26:92233.

1358

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 65.14A and B: (A) The Lobster Claw Abnormality is the midsystolic drop in left ventricle (LV) ejection velocities, here seen with
pulsed Doppler echocardiography at the entrance to the left ventricular outflow tract (LVOT). The midsystolic drop is due to the sudden imposition of afterload from mitralseptal contact. It is the cause of the midsystolic closure of the aortic valve and the spike and
dome pattern seen on aortic pressure tracings in patients with hypertrophic cardiomyopathy (HCM) and LVOT gradients > 60 mm Hg.
It is direct evidence of the corrosive effect of gradient in obstructive HCM; (B) The same patient after disopyramide and abolition of the
gradient. Note that the midsystolic drop in ejection velocities is no longer present. Note also the decrease in initial LV ejection acceleration, which is the mechanism of benefit of disopyramide. A decrease in ejection acceleration decreases the pushing force on the mitral
valve leaflets.

and premature death.70 Its pathophysiology is related to

the progression of ventricular fibrosis, and it may occur in
both obstructed and nonobstructed patients. The extent of
myocardial fibrosis is best visualized by CMR gadoliniumdelayed hyperenhancement.
More common than fixed LV systolic dysfunction,
LVOT obstruction may result in dynamic systolic
dysfunction due to afterload-mismatch and supply
demand ischemia.71-73 During midsystole when the flow
velocity and gradient is maximal through the LVOT, there
is a transient fall in LV ejection flow with partial closure
of the aortic valve.73 M-mode through the aortic valve
shows the classic midsystolic closure of the aortic valve
while pulsed Doppler interrogation at the entrance of the
LVOT shows a midsystolic drop in velocities in the shape
of a lobster claw (Figs 65.14 to 65.17).73-75 The midsystolic
drop in Doppler LV ejection velocities occurs due to a
premature termination of LV contraction caused by the
sudden imposition of afterload due to the obstruction.71,72
Obstruction worsens myocardial function in midsystole
on top of the inherently myopathic process. As mentioned
earlier, the lobster claw abnormality is also seen in midLV obstruction. Doppler tracing at the neck of the apical
akinetic chamber, near the beginning of the mid-LV
narrowing universally shows the lobster claw abnormality

during midsystole due to premature termination of

contraction at the apex with flow re-established in late
systole.
In contrast to the irreversible type systolic dysfunction,
alleviation of the LVOT obstruction improves systolic
function in patients with dynamic obstruction.76,77 This is best
illustrated in reported cases with sudden catastrophic LV
ballooning with severe LV systolic dysfunction following
the sudden transition from latent to overt high-resting
gradient LVOT obstruction. Patients present with obstructive
cardiogenic shock that mimics ACS. The coronary angiogram
shows no significant stenoses. As treatment strategies
are completely different, echocardiographic imaging is
crucial to show that the obstruction is the cause of the LV
dysfunction. Treatments focus on reversing precipitating
causes, intravenous fluids, intravenous beta-blockers,
and in resistant cases emergency surgical relief of LVOT
obstruction; in contrast, ACS requires coronary reperfusion.77
Positive inotropes such as dobutamine or dopamine
augment obstruction in HCM and are disastrous. Such cases
require expert echocardiography continuously. Repeat
echocardiograms the day after relief of obstruction either by
negative inotropic therapy or surgery shows improvement in
systolic function.

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

1359

DIFFERENTIAL DIAGNOSIS

Figs 65.15A to D: The midsystolic drop in left ventricle (LV) ejection

velocities. Left panel: Doppler echocardiographic tracings in a patient
with systolic anterior motion (SAM) of the mitral valve, mitralseptal
contact, and a left ventricular outflow (LVOT) gradient of > 100 mm Hg.
(A) Pulsed wave (PW) tracing with the cursor at the entrance of the
LVOT, upstream from the mitral valve. The midsystolic drop in left
ventricular ejection velocities begins at the inflection point (arrows).
It is caused by afterload-mismatch. The LV is unable to maintain
instantaneous ejection against the sudden rise in afterload; (B) CW
tracing through both the orifice and also through the entrance of the
LVOT that is apical of the mitral valve. After the inflection point (white
arrow), the contour of the jet velocity becomes concave to the left.
The superimposed midsystolic drop that occurs at the entrance of
the LVOT is shown with the yellow arrow. The midsystolic drop also
begins at the same point (white arrow). Right panel: four tracings
from a patient with LVOT systolic gradient of 120 mm Hg due to SAM
and mitralseptal contact. Tracings were obtained during the same
examination. (A) M-mode echocardiogram shows midsystolic closure
of the aortic valve leaflets. The arrow points to midsystolic closure; (B)
The midsystolic drop in left ventricular ejection velocities are shown
on pulsed Doppler tracing obtained from the apex. The pulsed cursor
is in the body of the LV, at the entrance of the left ventricular outflow
tract (LVOT). Velocity drops from 0.8 to 0.5 m/s. The arrow indicates
the nadir of the midsystolic drop; (C) Tissue Doppler echocardiogram
of the interventricular septum as measured from the apex of the LV.
Premature termination of systolic septal shortening is shown (arrow).
Scale in cm/s; (D) Continuous wave LVOT jet velocities are shown
from the left ventricular apex. LVOT gradient is 120 mm Hg. Scale
in m/s. Symmetry of events in early and midsystole is shown. The
midsystolic closure of aortic valve correlates with the midsystolic
drop in the left ventricular ejection velocities at the entrance of the
outflow tract, with premature termination of the septal shortening,
and with the peak of the gradient, afterload. Source: Reproduced
with permission from Sherrid M, et al. Reflections of inflections in
hypertrophic cardiomyopathy. J Am Coll Cardiol. 2009;54:21219.

Other etiologies that predispose to ventricular hypertrophy may be mistakenly diagnosed as HCM. This has
been termed pseudo-HCM. The most common etiology
of hypertrophy is chronic hypertension but others
include discrete subaortic membrane, athletes heart, LV
noncompaction, amyloidosis, AndersonFabry disease,
and Friedreichs ataxia.
Congenital subaortic membrane may cause high
gradients in the LVOT without the dynamic obstructive
patternthe Doppler spectra is similar to aortic stenosis
with midsystolic peak gradient and it is convex to the left.
In symptomatic patients, it is treated by surgical resection
of the membrane and replacement of the aortic valve if
there is more than mild aortic insufficiency.
LV noncompaction may superficially appear to be
hypertrophied myocardium. But left heart contrast echocardiography or CMR differentiates the compacted myocardium from the noncompacted, trabeculated myocardium.68
Thickened myocardium is the hallmark of cardiac
amyloidosis. The myocardium may have a ground glass
appearance, but this finding is not specific and can be
seen in renal failure and HCM. Moreover, with harmonic
imaging it is less prominent. A most valuable clue in making
the diagnosis of amyloidosis is the demonstration of low
voltage on the electrocardiogram, which is the reverse of any
other type of hypertrophy. Biopsy either from abdominal
fat, gastrointestinal tract, or the right ventricle makes this
diagnosis. The recent advent of transthyretin genetic analysis
is also useful in the rare patient with mutant transthyretin.
AndersonFabry disease, alpha-galactosidase A deficiency, a rare X-linked recessive lysosomal storage disease
may present with hypertrophy and infrequently with
SAM.78 It can be diagnosed with galactosidase levels in men,
and better by genetic testing in both genders. Treatment with
intravenous enzyme replacement is now available. Lastly,
Friedreichs ataxia is an autosomal recessive spinocerebellar
neuromyelopathy that may rarely present with severe hypertrophy in childhood, but more commonly presents as a
dilated cardiomyopathy.

The Athletes Heart

Highly trained athletes may develop a physiological
hypertrophic adaptation by proportionally increasing
the heart size and wall thickness resembling HCM.79 The
condition is not found with amateurs but rather with

1360

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 65.16A and B: Midsystolic drop in ejection velocities in midleft ventricle (LV) obstruction. (A) Pulsed wave (PW) spectral
Doppler with cursor located in the apical akinetic chamber at the neck of the midleft ventricular obstruction showing prominent
midsystolic drop in ejection velocity (thin arrow). There is an initial rise in velocities during the unobstructed phase (thick arrow),
followed by the marked decrease in midsystolic velocities and a second peak in early diastole. The drop in velocities corresponds
to the attenuation of the flow signal of the systolic jet in the obstructing neck during mid- and late systole; (B) Marked midsystolic
drop in LV ejection velocities in a patient with severe mid-LV obstruction. The pulsed Doppler is in the apical chamber at the
entrance of the neck of the mid-LV obstruction. Note the complete cessation of forward flow in this patient in midsystole and the
robust emptying of the chamber in late systole and early diastole. The midsystolic drop in ejection velocities is due to afterload
mismatch and provides persuasive evidence that the apical akinetic chamber is due to obstruction and supplydemand ischemia.
Source: Reproduced with permission from Sherrid M, et al. Reflections of inflections in hypertrophic cardiomyopathy. J Am Coll Cardiol.
2009;54:2129.

Fig. 65.17: Midsystolic drop in tissue Doppler velocities in

obstructive hypertrophic cardiomyopathy (HCM). The electrocardiogram, invasively measured left ventricular outflow tract
(LVOT) pressure gradient, and the tissue Doppler imaging (TDI)
velocity trace from the basal septum. In the post PVC beat, note
the simultaneous development of the LVOT gradient (red arrow)
and the midsystolic septal deceleration notch (black arrow).
Source: Reproduced with permission from Breithardt OA, et al.
Mid systolic septal deceleration in hypertrophic cardiomyopathy:
clinical value and insights into the pathophysiology of outflow
tract obstruction by tissue Doppler echocardiography. Heart.
2005;91:37980.

elite or professionals who train for more than 5 days a

week with hours of work-out a day. The physiological
adaptation of the heart to intense training depends on
the type of training. Repetitive pressure load training
such as weight lifting produces increased afterload with
subsequent concentric hypertrophy, whereas endurance
training such as jogging or cycling combines pressure and
volume load, an increase that leads to a balanced increase
in ventricular mass and chamber dilatation. Interestingly,
the largest extent of hypertrophy has been reported in
US football players, upper limit 16 mm (Fig. 65.18).80 The
gray zone between athletes heart and HCM occurs in
patients with wall-thickening between 13 and 16 mm.81
Other echocardiographic parameters that can be applied
in gray zone patients for differentiation favoring athletes
heart include: uniform hypertrophy pattern with an upper
limit of 16 mm, LV cavity size > 55 mm, and a proportional
increase in all chamber sizes with normal atria size indexed
to body surface area and normal LV diastolic function.
CMR may detect myocardial scarring in HCM that does
not occur in athletes. In ambiguous cases, athletes heart
may be distinguished from HCM by halting training for a
period of approximately 3 months to allow regression of
hypertrophy in the athlete.82

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

Fig. 65.18: Maximal wall thickness of professional football

players. The mean value was 11.2 mm ( 0.2 mm). Six percent of
players had a wall thickness > 14 mm.
Source: Reproduced with permission from Abernethy et al.
Echocardiographic characteristics of professional football players.
J Am Coll Cardiol. 2003;41:2804.

TREATMENT STRATEGIES IN
HYPERTROPHIC CARDIOMYOPATHY
Pharmacologic Therapy
Pharmacologic therapy is first-line treatment for symptomatic patients with obstructive HCM and adequate drug
trials should be administered before invasive measures
are contemplated. In patients with obstruction, the
pharmacologic treatment of HCM is based on negative
inotropes to relieve the dynamic obstruction by decreasing
anteriorly displacing drag forces on the mitral valve,
and thus potentiating normal posterior restraint of the
mitral apparatus by the papillary muscles and chordae.
Beta blockade is first-line negative inotropic therapy for
obstruction. Disopyramide in adequate dose is added
to patients who do not respond to beta blockade.83,84 The
goal of therapy in obstructed patients is improvement
in symptoms and functional status and reduction in
gradient. In patients without obstruction, beta blockers
and verapamil are used empirically for symptoms. The end
point here is improvement in functional status.

Surgical Myectomy and Resect

PlicateRelease
Septal myectomy is indicated for HCM patients who
have symptoms and gradients 50 mm Hg at rest or after

1361

physiological provocations that are refractory to medical

therapy. In the modern era, more extended myectomies
are performed that are explicitly designed to relieve
drag forces on the mitral valve.27,53,85,86 Contemporary
HCM surgical techniques also aim to both reduce septal
thickness and restore more normal mitral apparatus
architecture. TTE thus plays a pivotal role for planning
the extent of myectomy performed and to assess the
contribution of abnormal mitral anatomy to the SAM
(Figs 65.19A to D). Three planes are interrogated in order
to establish an effective septal resection: (a) Transverse
thickness of the proximal and midseptum. Adequate
myectomy redirects flow anteriorly and medially away
from the mitral valve. Over-resection may result in a
disastrous ventricular septal defect (VSD) while underresection results in the persistence of SAM and MR. (b)
Septal long-axis plane determines the length of resection
toward the apex; routinely the resection must extend 4.0
cm from the insertion of the right coronary cusp, 1.5 cm
past the point of mitral septal contact, down to the level of
the base of the papillary muscles. The most common cause
of persistent SAM is inadequate resection in this plane, just
in the subaortic area. Our surgical colleagues specifically
leave the subaortic thickening because it has nothing to do
with the cause of obstruction and because resection here
is fraught with the above mentioned complications. (c)
Mediallateral septal plane that determines the transverse
extent of the resection. While the classic Morrow
myectomy was a narrow trough 1 to 1.5 cm in diameter,
wider resection is now performed, particularly deeper
in the ventricle. It is the subaortic medial border of this
resection that is at a high risk for iatrogenic damage of the
conduction system and for VSD.
Additional corrective measures are added to the
myectomy when abnormal mitral anatomy contributes
to obstruction. Papillary muscle release is added to
myectomy in selected cases that have anterior positioning
of the papillary muscles in the LV cavity. Papillary release
was first introduced by Messmer, Schoendube, and others
and divides muscularfibrotic tissue connections between
the papillary muscles and the LV free wall.85,87 The release
allows the mitral apparatus to drop posteriorly into a
more normal position separating the inflow and outflow
tracts of the LV. Horizontal anterior leaflet plication was
first introduced by Swistel et al. It shortens and stiffens
the anterior leaflet when it is elongated and billows into
the outflow tractwhen the anterior leaflet 30 mm
(measured from mitral leaflet tip to the insertion of the

1362

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 65.19A to D: Surgical separation of left ventricular inflow from outflow in obstructive hypertrophic cardiomyopathy (HCM):
extended myectomy and papillary muscle mobilization. (A) Line drawing of outflow relative to the mitral valve in early systole. Note
the anterior position of the mitral valve coaptation. The prominent midseptal bulge redirects outflow so that it comes from a relatively
posterior direction, catching the anteriorly positioned mitral valve and pushing it into the septum; (B) After subaortic septal resection.
The subaortic septum has been resected, but only down to the tips of the mitral leaflets. Flow is still redirected by the remaining septal
bulge so that it comes from a posterior direction. It still catches the mitral valve; SAM persists, as does obstruction; (C) The septal bulge
below the mitral leaflet tips has been resected, an extended myectomy. Now, flow tracks more anteriorly and medially, away from the
mitral leaflets; (D) Mobilization and partial excision of the papillary muscles is added to extended myectomy. The mitral coaptation plane
is now more posterior, explicitly out of the flow stream. (See Movie Clips 65.3 and 65.4).
Source: Reprinted with permission from Sherrid MV. Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and
the continuing evolution of surgery for obstruction. Ann Thorac Surg. 2003;75:62032.

noncoronary aortic cusp in the 3 chamber view).52,88,89 As

with reefing of a sail, the reduction of leaflet area reduces
the billowing of the valve and stiffens it. Significant
calcifications of the leaflet are a contraindication for
the procedure. Horizontal plication is preferred over
the vertical plication introduced earlier by McIntosh
as the horizontal plication does not interfere with the
coapting surfaces.90 At St. Lukes-Roosevelt, NYC, the
combined operation is referred to as the ResectPlicate
Release operation (RPR) (Fig. 65.20 and Movie clips 65.3
and 65.4).52,53 Alternate successful approaches to mitral
valve pathology have been developed elsewhere.50,86,91 In
any given patient, preoperative planning with TTE and
transesophageal echocardiogram (TEE) determines which
aspects of the operation are applied.
The role of TEE both before and after cardiopulmonary
bypass is pivotal (Movie clips 65.3 and 65.4).27,92,93 TEE performed before decannulation allows complete assessment
of the new hemodynamics and extent of septal resection. It

should assure absence of significant SAM, more than mild

MR or VSD. 2D imaging should be used to measure the
new septal transverse thickness and length of the plicated
anterior mitral leaflet; CW and color Doppler imaging of
the LVOT and septum should rule out residual obstruction
or VSD, respectively. Then, the patient is given intravenous
dobutamine adequate to raise the heart rate to provoke
obstruction and to assess MR. If the spectral Doppler in the
LVOT persistently shows a gradient more than 30 mm Hg
or more than mild MR, the surgeon should initiate a second
pump run for additional corrective surgery.

ALCOHOL SEPTAL ABLATION

A second option for septal reduction to alleviate obstructive
symptoms in HCM patients resistant to pharmacologic
therapy is alcohol septal ablation.94,95 Ablation is recommended for patients who are not candidates for surgical septal
myectomy. At cardiac catheterization, a catheter is introduced

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

1363

Dual Chamber Pacing with Short AV Delay

Fig. 65.20: Schematic representation of the hypertrophied heart in

hypertrophic cardiomyopathy (HCM) depicting the morphological
variations leading to obstruction and the potential surgical options
for management including resection (extended myectomy),
plication (horizontal mitral plication), and release (manipulation of
the subvalvular structures). Also see Movie Clips 65.3 and 65.4.
Source: Reproduced with permission from Swistel DG, Balaram
SK. Surgical myectomy for hypertrophic cardiomyopathy in the
21st century, the evolution of the RPR repair: resection, plication,
and release. Prog Cardiovasc Dis. 2012:498502.

into the first septal branch of the left anterior descending

coronary artery. Then, careful assessment of the perforator
distribution is accomplished by injecting radiographic
contrast dye or dilute echocardiographic contrast into the
septal branch before the alcohol. The ablation procedure
creates a locally controlled myocardial infarction in the
septal region (Figs 65.21A to H). Variability in vessel
anatomy exists, so the operator must be careful not to
induce infarction of the distal septum, RV, anterior, lateral
or apical walls, or papillary muscles.94,96 If the pre-alcohol
contrast extends to any of these unexpected locales,
another septal perforator must be interrogated before
alcohol is injected. Once the operator is convinced that
the contrast distribution is limited to the proximal septum,
alcohol 100% is injected beyond an inflated balloon.
As with surgery, the echocardiogram at the end of the
procedure should evaluate the extent of the infarcted area,
presence of SAM, MR, and VSD.

Here by shortening the atrioventricular (AV) delay

complete capture of the ventricles is accomplished.
Depending on the native PR interval, AV delays of
60100 milliseconds may be required (longer for patients
with intrinsic AV delay). It is uncertain how ventricular
pacing improves obstruction, but it is most likely related
to the ventricular dyssynchrony that is produced by
ventricular pacing. Dual chamber (DDD) pacing with
short AV delay is only effective in half of patients and it is
not considered a primary therapy for LVOT obstruction.
However, guidelines support its use in patients who are
elderly or frail, or in patients who have devices for other
indications like implantable cardioverter defibrillator for
SCD prevention or for symptomatic bradycardia.1 Formal
AV optimization with echocardiography is required for all
patients who have DDD pacing for obstructive HCM.97,98
The AV delay is gradually reduced by 20 milliseconds
intervals until the QRS is maximally widened (complete
capture). At this point, duration of the diastolic transmitral
A-wave is measured, as well as its Doppler time velocity
integral (TVI), and the TVI of the whole transmitral
diastolic flow tracing. This is compared to baseline measurements before ventricular capture to assure that there is
no significant (<10%) decrement in A-duration and TVI.
Lastly, the LVOT gradient pre and post ventricular pacing
are compared to assess gradient decrease. It can sometimes
take months for the full effect of DDD pacing to take effect.
Disopyramide and DDD pacing have a synergistic effect
on LVOT gradient.99 At least yearly echocardiography is
mandatory whenever DDD pacing is used, to avoid over
therapy and marked reduction of LV systolic function due
to chronic dyssynchrony.

ENDOCARDITIS PROPHYLAXIS
Endocarditis antibiotic prophylaxis is no longer
recommended for obstructive HCM. This is a relatively
rare complication of HCM with a prevalence of <1% that
mainly affects patients with resting obstruction.100 The
mitral valve apparatus and site of septal contact are most
common locations of vegetations. TEE is the modality of
choice to diagnose endocarditis and distinguish between
vegetations and redundant leaflets from HCM.

1364

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 65.21A to H: Variety of left and right ventricular structures at risk for alcohol-induced necrosis as detected by intraprocedural echocardiography. Opacification of the medial papillary muscle of the left ventricle (LV; A, arrow), the basal segment of the posterolateral wall
(B), the entire posterolateral wall in the apical four-chamber view (C, arrows), the entire interventricular septum (D), a papillary muscle
(arrow) of the right ventricle (RV) via a moderator band in association with a small contrast depot within the interventricular septum (E),
a small segment of the basal inferior portion (arrows) of LV free wall (parasternal short-axis view) (F), the entire posterolateral wall (G)
in the apical long-axis view (same patient as C; arrows), and a larger septal portion together with a right ventricular papillary muscle
(H, arrow). (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Faber L, et al. Targeting percutaneous transluminal septal ablation for hypertrophic obstructive cardiomyopathy by intraprocedural echocardiographic monitoring. J Am Soc Echocardiogr. 2000;13:10749.

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hormonal responses to a meal in hypertrophic cardiomyopathy: a comparison of patients with and without
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1996;19(2):12935.
Cotrim CA, Adame PR, Pereira HH. Exercise echocardiography in hypertrophic cardiomyopathy-upright
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Maron BJ, Gottdiener JS, Roberts WC, Henry WL, Savage
DD, Epstein SE. Left ventricular outflow tract obstruction
due to systolic anterior motion of the anterior mitral leaflet
in patients with concentric left ventricular hypertrophy.
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Argulian E, Messerli FH, Aziz EF, et al. Antihypertensive
therapy in hypertrophic cardiomyopathy. Am J Cardiol.
2013;111(7):10405.

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36. Drinko JK, Nash PJ, Lever HM, et al. Safety of stress
testing in patients with hypertrophic cardiomyopathy. Am
J Cardiol. 2004;93(11):14434, A12.
37. Marwick TH, Nakatani S, Haluska B, et al. Provocation of
latent left ventricular outflow tract gradients with amyl
nitrite and exercise in hypertrophic cardiomyopathy. Am
J Cardiol. 1995;75(12):8059.
38. Klues HG, Leuner C, Kuhn H. Left ventricular outflow tract
obstruction in patients with hypertrophic cardiomyopathy:
increase in gradient after exercise. J Am Coll Cardiol.
1992;19(3):52733.
39. Gilligan DM, Nihoyannopoulos P, Fletcher A, et al.
Symptoms of hypertrophic cardiomyopathy, with special
emphasis on syncope and postprandial exacerbation of
symptoms. Clin Cardiol. 1996;19(5):3718.
40. Klues HG, Roberts WC, Maron BJ. Morphological
determinants of echocardiographic patterns of mitral
valve systolic anterior motion in obstructive hypertrophic
cardiomyopathy. Circulation. 1993;87(5):15709.
41. Klues HG, Roberts WC, Maron BJ. Anomalous insertion
of papillary muscle directly into anterior mitral leaflet in
hypertrophic cardiomyopathy. Significance in producing
left ventricular outflow obstruction. Circulation. 1991;
84(3):118897.
42. Haley JH, Sinak LJ, Tajik AJ, et al. Dynamic left ventricular
outflow tract obstruction in acute coronary syndromes: an
important cause of new systolic murmur and cardiogenic
shock. Mayo Clin Proc. 1999;74(9):9016.
43. Ohba Y, Takemoto M, Nakano M, et al. Takotsubo
cardiomyopathy with left ventricular outflow tract
obstruction. Int J Cardiol. 2006;107(1):1202.
44. Schwinger ME, OBrien F, Freedberg RS, et al. Dynamic
left ventricular outflow obstruction after aortic valve
replacement: a Doppler echocardiographic study. J Am
Soc Echocardiogr. 1990;3(3):2058.
45. Pearson AC, Gudipati CV, Labovitz AJ. Systolic and
diastolic flow abnormalities in elderly patients with
hypertensive hypertrophic cardiomyopathy. J Am Coll
Cardiol. 1988;12(4):98995.
46. Come PC, Bulkley BH, Goodman ZD, et al. Hypercontractile
cardiac states simulating hypertrophic cardiomyopathy.
Circulation. 1977;55(6):9018.
47. Hagge AA, Bruneval P, Levine RA, et al. The mitral valve
in hypertrophic cardiomyopathy: old versus new concepts.
J Cardiovasc Transl Res. 2011;4(6):75766.
48. Sasson Z, Yock PG, Hatle LK, et al. Doppler echocardiographic determination of the pressure gradient in
hypertrophic cardiomyopathy. J Am Coll Cardiol. 1988;
11(4):7526.
49. Schoendube FA, Klues HG, Reith S, et al. Long-term clinical
and echocardiographic follow-up after surgical correction
of hypertrophic obstructive cardiomyopathy with extended
myectomy and reconstruction of the subvalvular mitral
apparatus. Circulation. 1995;92(9 Suppl):II1227.
50. Kaple RK, Murphy RT, DiPaola LM, et al. Mitral
valve abnormalities in hypertrophic cardiomyopathy:

51.

52.

53.

54.

55.

56.

57.

58.

59.

60.

61.

62.

63.

64.

echocardiographic features and surgical outcomes. Ann

Thorac Surg. 2008;85(5):152735, 1535.e1.
Swistel DG, Balaram SK. Resection, Plication, Release
the RPR procedure for obstructive hypertrophic cardiomyopathy. Anadolu Kardiyol Derg. 2006;6 Suppl 2:316.
Balaram SK, Ross RE, Sherrid MV, et al. Role of mitral
valve plication in the surgical management of hypertrophic
cardiomyopathy. Ann Thorac Surg. 2012;94(6):19907;
discussion 1997.
Balaram SK, Tyrie L, Sherrid MV, et al. Resectionplication-release for hypertrophic cardiomyopathy: clinical
and echocardiographic follow-up. Ann Thorac Surg.
2008;86(5):153944; discussion 1544.
Nishimura RA, Appleton CP, Redfield MM, et al. Noninvasive
Doppler echocardiographic evaluation of left ventricular
filling pressures in patients with cardiomyopathies: a
simultaneous Doppler echocardiographic and cardiac
catheterization study. J Am Coll Cardiol. 1996;28(5):
122633.
Maron BJ, Spirito P, Green KJ, et al. Noninvasive
assessment of left ventricular diastolic function by pulsed
Doppler echocardiography in patients with hypertrophic
cardiomyopathy. J Am Coll Cardiol. 1987;10(4):73342.
Rajiv C, Vinereanu D, Fraser AG. Tissue Doppler
imaging for the evaluation of patients with hypertrophic
cardiomyopathy. Curr Opin Cardiol. 2004;19(5):4306.
Nagueh SF, Lakkis NM, Middleton KJ, et al. Doppler
estimation of left ventricular filling pressures in patients
with hypertrophic cardiomyopathy. Circulation. 1999;
99(2):25461.
Nagueh SF, Bachinski LL, Meyer D, et al. Tissue Doppler
imaging consistently detects myocardial abnormalities in
patients with hypertrophic cardiomyopathy and provides a
novel means for an early diagnosis before and independently
of hypertrophy. Circulation. 2001;104(2):12830.
Yang H, Sun JP, Lever HM, et al. Use of strain imaging
in detecting segmental dysfunction in patients with
hypertrophic cardiomyopathy. J Am Soc Echocardiogr.
2003;16(3):2339.
Matsubara H, Nakatani S, Nagata S, et al. Salutary effect
of disopyramide on left ventricular diastolic function
in hypertrophic obstructive cardiomyopathy. J Am Coll
Cardiol. 1995;26(3):76875.
Nishimura RA, Schwartz RS, Holmes DR Jr, et al. Failure of
calcium channel blockers to improve ventricular relaxation
in humans. J Am Coll Cardiol. 1993;21(1):1828.
Kass DA, Wolff MR, Ting CT, et al. Diastolic compliance
of hypertrophied ventricle is not acutely altered by
pharmacologic agents influencing active processes. Ann
Intern Med. 1993;119(6):46673.
Yamaguchi H, Nishiyama S, Nakanishi S, et al. Electrocardiographic, echocardiographic and ventriculographic
characterization of hypertrophic non-obstructive cardiomyopathy. Eur Heart J. 1983;4(Suppl F):10519.
Eriksson MJ, Sonnenberg B, Woo A, et al. Longterm outcome in patients with apical hypertrophic
cardiomyopathy. J Am Coll Cardiol. 2002;39(4):63845.

Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy

65. Maron MS, Finley JJ, Bos JM, et al. Prevalence, clinical
significance, and natural history of left ventricular apical
aneurysms in hypertrophic cardiomyopathy. Circulation.
2008;118(15):15419.
66. Minami Y, Kajimoto K, Terajima Y, et al. Clinical implications
of midventricular obstruction in patients with hypertrophic
cardiomyopathy. J Am Coll Cardiol. 2011;57(23):234655.
67. Cecchi F, Olivotto I, Nistri S, et al. Midventricular
obstruction and clinical decision-making in obstructive
hypertrophic cardiomyopathy. Herz. 2006; 31(9):8716.
68. Comella A, Magnacca M. Pseudo-left ventricle apical
hypertrophy: bedside diagnosis with SonoVue contrast.
Echocardiography. 2004;21(6):5634.
69. Nakamura T, Matsubara K, Furukawa K, et al. Diastolic
paradoxic jet flow in patients with hypertrophic
cardiomyopathy: evidence of concealed apical asynergy
with cavity obliteration. J Am Coll Cardiol. 1992;19(3):
51624.
70. Harris KM, Spirito P, Maron MS, et al. Prevalence, clinical
profile, and significance of left ventricular remodeling
in the end-stage phase of hypertrophic cardiomyopathy.
Circulation. 2006;114(3):21625.
71. Barac I, Upadya S, Pilchik R, et al. Effect of obstruction
on longitudinal left ventricular shortening in hypertrophic
cardiomyopathy. J Am Coll Cardiol. 2007;49(11):120311.
72. Breithardt OA, Beer G, Stolle B, et al. Mid systolic septal
deceleration in hypertrophic cardiomyopathy: clinical
value and insights into the pathophysiology of outflow
tract obstruction by tissue Doppler echocardiography.
Heart. 2005;91(3):37980.
73. Sherrid MV, Gunsburg DZ, Pearle G. Mid-systolic drop in
left ventricular ejection velocity in obstructive hypertrophic
cardiomyopathythe lobster claw abnormality. J Am Soc
Echocardiogr. 1997;10(7):70712.
74. Conklin HM, Huang X, Davies CH, et al. Biphasic left
ventricular outflow and its mechanism in hypertrophic
obstructive cardiomyopathy. J Am Soc Echocardiogr.
2004;17(4):37583.
75. Maron BJ, Gottdiener JS, Arce J, et al. Dynamic subaortic
obstruction in hypertrophic cardiomyopathy: analysis by
pulsed Doppler echocardiography. J Am Coll Cardiol. 1985;
6(1):118.
76. Kirschner E, Berger M, Goldberg E. Hypertrophic obstructive
cardiomyopathy presenting with profound hypotension.
Role of two-dimensional and Doppler echocardiography
in diagnosis and management. Chest. 1992;101(3):71114.
77. Sherrid MV, Balaran SK, Korzeniecki E, et al. Reversal
of acute systolic dysfunction and cardiogenic shock
in hypertrophic cardiomyopathy by surgical relief of
obstruction. Echocardiography. 2011;28(9):E1749.
78. Sachdev B, Takenaka T, Teraguchi H, et al. Prevalence
of Anderson-Fabry disease in male patients with
late onset hypertrophic cardiomyopathy. Circulation.
2002;105(12):140711.
79. Maron BJ, Pelliccia A. The heart of trained athletes: cardiac
remodeling and the risks of sports, including sudden death.
Circulation. 2006;114(15):163344.

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80. Abernethy WB, Choo JK, Hutter AM Jr. Echocardiographic

characteristics of professional football players. J Am Coll
Cardiol. 2003;41(2):2804.
81. Pelliccia A, Maron BJ, Spataro A, et al. The upper limit
of physiologic cardiac hypertrophy in highly trained elite
athletes. N Engl J Med. 1991;324(5):295301.
82. Maron BJ. Distinguishing hypertrophic cardiomyopathy
from athletes heart physiological remodelling: clinical
significance, diagnostic strategies and implications for
preparticipation screening. Br J Sports Med. 2009;43(9):
64956.
83. Sherrid MV, Arabadjian M. A primer of disopyramide
treatment of obstructive hypertrophic cardiomyopathy.
Prog Cardiovasc Dis. 2012;54(6):48392.
84. Sherrid MV, Barac I, McKenna WJ, et al. Multicenter study
of the efficacy and safety of disopyramide in obstructive
hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005;
45(8):12518.
85. Messmer BJ. Extended myectomy for hypertrophic
obstructive cardiomyopathy. Ann Thorac Surg. 1994;58(2):
5757.
86. Dearani JA, Ommen SR, Gersh BJ, et al. Surgery
insight: Septal myectomy for obstructive hypertrophic
cardiomyopathythe Mayo Clinic experience. Nat Clin
Pract Cardiovasc Med. 2007;4(9):50312.
87. Schoendube FA, Klues HG, Reith S, et al. Surgical
correction of hypertrophic obstructive cardiomyopathy
with combined myectomy, mobilisation and partial
excision of the papillary muscles. Eur J Cardiothorac Surg.
1994;8(11):6038.
88. Swistel DG, Balaram SK. Surgical myectomy for
hypertrophic cardiomyopathy in the 21st century, the
evolution of the RPR repair: resection, plication, and
release. Prog Cardiovasc Dis. 2012;54(6):498502.
89. Balaram SK, Sherrid MV, Derose JJ Jr, et al. Beyond
extended myectomy for hypertrophic cardiomyopathy: the
resection-plication-release (RPR) repair. Ann Thorac Surg.
2005;80(1):21723.
90. McIntosh CL, Maron BJ, Cannon RO 3rd, et al. Initial
results of combined anterior mitral leaflet plication
and ventricular septal myotomy-myectomy for relief
of left ventricular outflow tract obstruction in patients
with hypertrophic cardiomyopathy. Circulation. 1992;86
(5 Suppl):II607.
91. Minakata K, Dearani JA, Nishimura RA, et al. Extended septal
myectomy for hypertrophic obstructive cardiomyopathy
with anomalous mitral papillary muscles or chordae.
J Thorac Cardiovasc Surg. 2004;127(2):4819.
92. Grigg LE, Wigle ED, Williams WG, et al. Transesophageal
Doppler echocardiography in obstructive hypertrophic
cardiomyopathy: clarification of pathophysiology and
importance in intraoperative decision making. J Am Coll
Cardiol. 1992;20(1):4252.
93. Ommen SR, Park SH, Click RL, et al. Impact of intraoperative transesophageal echocardiography in the
surgical management of hypertrophic cardiomyopathy.
Am J Cardiol. 2002;90(9): 10224.

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94. Faber L, Ziemssen P, Seggewiss H. Targeting percutaneous

transluminal septal ablation for hypertrophic obstructive
cardiomyopathy by intraprocedural echocardiographic
monitoring. J Am Soc Echocardiogr. 2000;13(12):10749.
95. Nagueh SF, Groves BM, Schwartz L, et al. Alcohol septal
ablation for the treatment of hypertrophic obstructive
cardiomyopathy. A multicenter North American registry.
J Am Coll Cardiol. 2011;58(22):23228.
96. Singh M, Edwards WD, Holmes DR Jr, et al. Anatomy of the
first septal perforating artery: a study with implications for
ablation therapy for hypertrophic cardiomyopathy. Mayo
Clin Proc. 2001;76(8):799802.
97. Nishimura RA, Trusty JM, Hayes DL, et al. Dual-chamber
pacing for hypertrophic cardiomyopathy: a randomized,

double-blind, crossover trial. J Am Coll Cardiol. 1997;

29(2):43541.
98. Nishimura RA, Hayes DL, Ilstrup DM, et al. Effect of
dual-chamber pacing on systolic and diastolic function in
patients with hypertrophic cardiomyopathy. Acute Doppler
echocardiographic and catheterization hemodynamic
study. J Am Coll Cardiol. 1996;27(2):42130.
99. Minami Y, Kajimoto K, Kawana M, et al. Synergistic
effect of dual chamber pacing and disopyramide in
obstructive hypertrophic cardiomyopathy. Int J Cardiol.
2010;141(2):1957.
100. Spirito P, Rapezzi C, Bellone P, et al. Infective endocarditis
in hypertrophic cardiomyopathy: prevalence, incidence,
and indications for antibiotic prophylaxis. Circulation.
1999;99(16):21327.

CHAPTER 66
Echocardiographic Assessment of
Nonobstructive Cardiomyopathies
Rohit Gokhale, Manreet Basra, Victor Vacanti, Steven J Horn, Aylin Sungur, Robert P Gatewood Jr, Navin C Nanda

Snapshot

Cardiomyopathies

Dilated Cardiomyopathy (DCM)

Secondary Findings in Dilated Cardiomyopathy

The Role of Echocardiography in Opmizing Heart Failure

Echocardiography in Assessing Ventricular Remodeling

CARDIOMYOPATHIES
The diagnosis of a cardiomyopathy encompasses a wide
spectrum of cardiac diseases, all of which are characterized
by cardiac myocyte dysfunction, and therefore a myopathy.
While systolic dysfunction impairs end-organ perfusion,
diastolic dysfunction disrupts the perfusion to the cardiac
myocyte itself. This results in the propagation of a vicious
cycle whereby systolic dysfunction can ensue.
The World Health Organization (WHO) defined cardiomyopathy as a disease of the myocardium associated
with cardiac dysfunction in 1995.1 The cardiomyopathies
were classified according to anatomy and physiology
into five major types. The inclusion of arrhythmogenic
right ventricular cardiomyopathy/dysplasia (ARVC/D)
and primary restrictive cardiomyopathy (RCM) in the
classification for the first time broadened the definition,
characterizing the breadth of its presentation:
1. Dilated cardiomyopathy (DCM)
a. Idiopathic
b. Familial/genetic
c. Viral

Findings in Dilated Cardiomyopathy Based on Eology

Restricve Cardiomyopathy

Other Infiltrave Cardiomyopathies

Infecous and Metabolic Cardiomyopathies

Carcinoid Heart Disease

d. Immune-mediated
e. Toxic/alcoholic
2. Hypertrophic cardiomyopathy (HCM)
3. RCM
4. ARVC/D
5. Unclassified cardiomyopathiesfor example, left
ventricle (LV) noncompaction
It also included specific cardiomyopathies such as the
following:
Ischemic
Valvular
Hypertensive
Inflammatory: Idiopathic, autoimmune, and infectious
Metabolic: Endocrine-related; storage diseases hemochromatosis/glycogen storage
Muscular dystrophies: Duchenne, Becker, and myotonic
Peripartum
In 2006, the American Heart Association (AHA)
scientific statement proposed a more contemporary definition and classification of the cardiomyopathies.2

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The expert consensus panel proposed the following

definition:
Cardiomyopathies are a heterogeneous group of
diseases of the myocardium associated with mechanical
and/or electrical dysfunction that usually (but not invariably)
exhibit inappropriate ventricular hypertrophy or dilatation
and are due to a variety of causes that frequently are genetic.
Cardiomyopathies are either confined to the heart or are
a part of generalized systemic disorders, often leading to
cardiovascular death or progressive heart failure-related
disability.
Cardiomyopathies are categorized into two groups:
1. Primary cardiomyopathies (predominantly involving
the heart)
a. Genetic:
Hypertrophic
ARVC/D.
Left ventricular noncompaction
PRKAG2 and Danon glycogen storage diseases
Conduction defects, mitochondrial myopathies,
and ion channel disorders.
b. Mixed:
Dilated
Restrictive.
c. Acquired:
Myocarditis
Stress-induced (Takotsubo)
Peripartum
Tachycardia-induced
Infants of insulin-dependent diabetic mothers.
2. Secondary cardiomyopathies (accompanied by other
organ system involvement)
In 2008, the European Society of Cardiologys working
group on myocardial and pericardial diseases presented
an update to the WHO/ISFC classification in which
cardiomyopathy was defined as: A myocardial disorder
in which the heart muscle is structurally and functionally
abnormal in the absence of coronary artery disease (CAD),
hypertension, valvular disease, and congenital heart
disease sufficient to explain the observed myocardial
abnormality.3
In summary, the definition and classification of
cardiomyopathies continues to evolve with better understanding of the diverse disease states of the myocardium.
It is important that the sonographer and cardiologist
keep abreast of the developments in cardiomyopathies,
since it will impact the acquisition and interpretation of
echocardiographic studies.

DILATED CARDIOMYOPATHY (DCM)

The primary features of all dilated cardiomyopathies include
decreased cardiac output and left ventricular contractility
with significantly enlarged left ventricular dimensions.
The etiology of DCM is variable and encompasses a
wide spectrum of diseases. These are as noted below:
Ischemic cardiomyopathy
Idiopathic cardiomyopathy
Familial cardiomyopathy
Noncompacted myocardium
Peripartum cardiomyopathy
Hemochromatosis
Infectious
Postviral myocarditis
HIV
Legionella
Sepsis
Toxic cardiomyopathy
Alcohol
Adriamycin
Other chemotherapy-induced
Patients with DCM may initially present with
symptoms of a low cardiac output state such as fatigue or
effort intolerance. In more progressive states, they may
also note orthopnea or paroxysmal nocturnal dyspnea
and if untreated often present with acute congestive heart
failure (CHF). Echocardiography is a valuable tool in the
management of such patients and has a Class I indication
according to the ACC/AHA guidelines for the management
of CHF. Patients with a DCM have a constellation of
diagnostic features that characterize this condition.
Characterized by chamber enlargement, DCM initially
affects the LV. Measuring the left ventricular dimension by
echocardiography forms a cornerstone in the diagnosis
of DCM. According to the ASE guidelines,4 the normal
reference range for LV diastolic dimension for women is
3.95.3 cm and for men is 4.25.9 cm. When indexed to
body surface area, the normal range would be 2.43.2 cm/m2
for women and 2.23.1 cm/m2 for men. LV dimensions
in excess of 6.2 cm in women and 6.9 cm in men,
suggest severely enlarged and abnormal ventricular size
(Figs 66.1A to D; Movie clips 66.1 A to C).4

Assessment of Left Ventricular

Contractility
Visual estimation of left ventricular functional can be performed with two-dimensional (2D), three-dimensional
(3D), and M-mode echocardiography. With M-mode
measurements of left ventricular size and thickening, a

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1371

Figs 66.1A to D: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A), parasternal
short-axis (B), and apical four-chamber (C) views showing marked global left ventricle (LV) hypokinesis. Arrow points to a catheter in
the right heart. The echogenic tricuspid valve (TV) annulus probably represents fatty infiltration; (D) M-mode also showing poor LV function with increased mitral E point to interventricular septum separation. (Ao: Aorta; DA: Descending thoracic aorta; IVS: Interventricular
septum; LA: Left atrium; MV: Mitral valve; PW: Posterior wall; RV: Right ventricle) (Movie clips 66.1A to C).

reliable estimate of fractional shortening can be made,

which in turn determines contractility and ejection fraction. Measurement of the ejection fraction by Simpsons
method using estimates of the left ventricular volume during systole and diastole provides an objective assessment
of left ventricular contractility (see Chapter 41).
The decrease in systolic function can be segmental
or global depending on the underlying cause of the
cardiomyopathy. Segmental wall motion abnormalities
usually suggest an ischemic etiology, corresponding to
CAD in the distribution to the affected segments. With
time and progression of ischemic cardiomyopathy, a
global decrease in contractility may be seen. Nonischemic

cardiomyopathies usually present with a global reduction

in contractility, although regional differences may be seen.
Regional wall motion abnormalities involving the
septum are also seen in the presence of bundle branch
blocks, postoperatively after coronary artery bypass
grafting surgery and with chronic right ventricular pacing.
Bundle branch blocks lead to mechanical dyssynchrony
during ventricular systole, which further compromises
contractility and cardiac output. The presence of a
prolonged QRS duration, particularly left sided, in patients
with heart failure is associated with more advanced
myocardial disease, greater LV dysfunction, a poorer
prognosis and a higher all-cause mortality.5 In the presence

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

of bundle branch blocks, there is evidence to suggest

that cardiac resynchronization therapy (CRT) decreases
all-cause mortality, reduces heart failure hospitalizations,
and improves left ventricular ejection fraction (LVEF)
in NYHA functional Class I/II heart failure patients.6
Abnormal septal motion can also result from right ventricular
pressure or volume overload with the onset of pulmonary
hypertension and/or right ventricular failure (Figs 66.2
to 66.4; Movie clips 66.2Part 1 and 2; 66.3A to C).

The increase in left ventricular dimensions is accompanied

by a change in LV shape, with remodeling resulting in a
more spherical structure. Measurement of the sphericity

index is helpful in this regard. The sphericity index of the

LV is the ratio of the long-axis dimension to the minor-axis
dimension (diameter) of the LV. The normal ratio is > 1.6. A
sphericity index of < 1.5 implies pathological remodeling.
As the ventricle enlarges and becomes more spherical, there also occurs a displacement of the papillary
muscles apically and laterally. Consequently, there
is a change in mitral valve leaflet coaptation as the
length of the mitral apparatus is decreased, resulting in
functional mitral regurgitation (FMR). Besides mitral
regurgitation, dilatation of the LV often results in left atrial
enlargement with subsequent pulmonary hypertension
and right ventricle (RV) enlargement. Right ventricular
contractility can be primarily affected by the etiology of
the cardiomyopathy or can result from progression of left
ventricular failure.
In patients with DCM, wall thickness is variable but
is typically within normal limits. The LV mass though, is
uniformly increased.

SECONDARY FINDINGS IN DILATED

CARDIOMYOPATHY
Cardiac Dimensions

Figs 66.2A to D

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1373

Figs 66.2A to E: Dilated cardiomyopathy. Live/real time threedimensional transthoracic echocardiography (3D TTE). (A to C)
Systematic and sequential anteroposterior cropping of right ventricle
(RV) demonstrates the moderator band (arrowhead) as well as other
trabeculations and papillary muscles (arrow) in the right ventricle
(RV); (D and E) Further cropping shows only a few trabeculations in
the RV apex. There is no evidence of noncompaction. Also, there is
no evidence of a mass or clot in the RV apex, which was suspected
on the two-dimensional (2D) study. A prominent trabeculation in the
RV apex very clearly delineated by 3D TTE (arrows in the Movie
clip 66.2, Part 1) was most likely the culprit for the mass-like lesion
suspected on the 2D echocardiogram. Both ventricles are dilated and
show globally poor motion typical of dilated cardiomyopathy. Arrow in
A, B, D, and E points to a false tendon in left ventricle (LV). Movie clip
66.2, Part 2 shows another patient with dilated cardiomyopathy and
globally poor biventricular function. The arrowhead shows a large
clot in the LV which when further cropped demonstrates a central
echolucency consistent with clot lysis. A few trabeculations are also
seen. (LA: Left atrium; RA: Right atrium) (Movie clips 66.2 Parts 1
and 2). Source: Reproduced with permission from Bodiwala K, et
al. Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography. 2005;22:
61120.

Figs 66.3A and B: Dilated cardiomyopathy. (A) The left ventricle (LV) end-diastolic dimension in the parasternal long axis has decreased
in the follow-up study (right side) as compared to baseline examination (left side). Movie clips 66.3A (parasternal long-axis view), 3B
(parasternal short-axis view), and 3C (apical four-chamber view) also show improved LV function 1 year after antifailure regimen.
(Ao: Aorta; DA: Descending aorta; LA: Left atrium; PW: Posterior wall; RA: Right atrium; RV: Right ventricle; VS: Ventricular septum).

Chamber dimensions as assessed by echocardiography

also aid prognostication in patients with DCM. Significant
dilatation of the left atrium as noted when the left
atrial dimension indexed to body surface area exceeds
26 mm/m2; has been shown to predict mortality and
rehospitalization due to heart failure exacerbation.7 In
patients above 70 years of age, increased indexed left atrial
size was the single independent predictor of death from
cardiac causes and the most important indicator of cardiac
events.

With increasing right ventricular size and pressure/

volume overload, the inferior vena cava (IVC) is also
dilated. It can be imaged in the subcostal views of the heart
about 12 cm from its point of entry into the right atrium
(RA). The diameter of the IVC and the percent decrease in
the diameter during inspiration correlate with RA pressure.
Evaluation of the inspiratory response with a brief sniff
(the sniff test) is more reliable than normal inspiration
alone. The normal IVC diameter is < 1.7 cm. There is a
50% decrease in the diameter when the right atrial (RA)

1374

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 66.4A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical fourchamber; (B) views demonstrate LV and left atrial (LA) dilatation. (RA: Right atrium; RV: Right ventricle).

Fig. 66.5: Dilated cardiomyopathy. Two-dimensional transthoracic

echocardiography. Subcostal view adjusted to demonstrate the
long axis of the inferior vena cava (IVC) in a patient with dilated
cardiomyopathy and severe pulmonary hypertension. (RA: Right
atrium).

pressure is normal (05 mm Hg). A dilated IVC (> 1.7 cm)

with normal inspiratory collapse ( 50%) is suggestive of
a mildly elevated RA pressure (610 mm Hg). A dilated
IVC with an inspiratory collapse < 50% suggests that the
RA pressure is moderately elevated (1015 mm Hg).
Finally, a dilated IVC without any collapse suggests a
markedly increased RA pressure of >15 mm Hg (Fig. 66.5).8

Evidence of Effusion
Pericardial and pleural effusions are readily imaged
on echocardiography and can be signs of significant

volume overload. In particular, the presence of bilateral

right and left pleural effusions are often noted in poorly
compensated cardiomyopathies.
The presence of an isolated pericardial effusion
can provide diagnostic data regarding the etiology
of the cardiomyopathy, especially when the etiology
is postinfectious such as with viral myocarditis. The
pericardial effusion can be tapped with echocardiographic
guidance to help in the diagnostic work-up. However, in the
absence of infectious or metabolic causes such as uremia,
a circumferential effusion may also be accompanied by
anasarca.
In patients with decompensated DCM, the distribution
is typically circumferential. However, with postinfectious
DCM, the effusion may be localized. The detection of
stranding within the fluid suggests an inflammatory or
a hemorrhagic etiology of the effusion. Although rare in
cardiomyopathies, when large pericardial effusions occur,
the concern for tamponade becomes a determining factor
in the management. Evidence of tamponade physiology as
noted by right ventricular collapse, abnormal inspiratory
increase of blood flow velocity through the tricuspid
valve and abnormal inspiratory decrease of mitral valve
flow velocity, phasic variation in right ventricular outflow
tract (RVOT) or left ventricular outflow tract flow, and
exaggerated respiratory variation in IVC flow suggest
significant hemodynamic compromise. The left ventricular
function is further diminished, resulting in decreased
cardiac output. These suggest the need for emergent
evacuation of the effusion.

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1375

Figs 66.6A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Mitral valve M-mode echocardiography
showing a B bump (A) predictive of significant left ventricular end-diastolic pressure (LVEDP) and an enlarged E-point septal separation
(EPSS; B) in a patient with dilated cardiomyopathy due to a reduced stroke volume with poor systolic function. A normal EPSS should
generally be < 1 cm.

Two-Dimensional and M-Mode Findings

In the evaluation of patients with cardiomyopathy,
helpful M-mode echocardiographic findings include
E-point septal separation (EPSS) and the mitral M-mode
evaluation showing a prominent b-bump.
The distance (in millimeters) from the anterior septum
to the maximal early opening (E-point) of the mitral valve
is defined as the EPSS. The internal diameter of the LV is
proportional to diastolic LV dimension and the maximal
systolic excursion of the mitral valve is proportional to
the mitral stroke volume. Therefore, the ratio of these two
dimensions would be proportional to the ejection fraction.
The normal EPSS is approximately 6 mm. It is significantly
elevated in patients with advanced cardiomyopathy, often
exceeding 15 mm. M-mode imaging also may reveal a
b-bump at the end of the a-wave, which is also consistent
with an elevated left ventricular end diastolic pressure
(LVEDP) with advanced cardiomyopathies (Figs 66.6A
and B).

Doppler Echocardiography
Doppler echocardiography assists in assessing cardiac
output, cardiac index, stroke volume, stroke volume index
(stroke volume indexed to body surface area), ventricular
diastolic function, pulmonary arterial pressures, and right
atrial pressure. The product of the transaortic velocity time
integral with the cross sectional area of the left ventricular

outflow tract (LVOT area) gives an estimate of the stroke

volume and thus the cardiac output (stroke volume heart
rate). This is significantly reduced in patients with DCM.
The use of the mitral inflow velocity as well as mitral
annular excursion by tissue Doppler imaging provides
valuable information for the evaluation of diastolic
function. Diastolic dysfunction accompanies systolic
dysfunction and is a determinant of the prognosis of
patients with cardiomyopathy. The spectrum of diastolic
dysfunction ranges from delayed relaxation to irreversible
end-stage restrictive physiology. The diastolic function
correlates with LVEDP. The progression to restrictive
patterns of diastolic dysfunction is suggestive of volume
overload and poorly compensated heart failure.
The poor stroke volume results in elevated LV
end-diastolic pressures and decreased deceleration time.
The rapid deceleration time and an early to late diastolic
peak velocity ratio of mitral flow > 1.0 were associated with a
poorer prognosis among patients with an ejection fraction
< 40% after a recent myocardial infarction (MI).9 Patients
with reversible restrictive filling patterns have a more
favorable long-term prognosis10 with lesser hospitalizations
for heart failure. The evidence of a pseudonormal pattern of
mitral inflow velocities (earlyE-wave, and lateA-wave)
can be seen when patients progress from mild diastolic
dysfunction to more severe stages. It can be unmasked
by imaging with the use of the Valsalva maneuver. During
Valsalva, the flow into the left heart is reduced and therefore,

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ventricular remodeling and therefore, consideration of

pharmacological and nonpharmacological treatment to
correct it might be reasonable (Fig. 66.7).
Evaluation of the tricuspid valve with Doppler
echocardiography permits assessment of tricuspid
regurgitation. A measurement of the flow across the
tricuspid valve allows an estimate of the transvalvular
pressure gradient. This in turn provides a noninvasive
assessment of RV systolic and pulmonary artery pressures.
The presence of pulmonary hypertension among patients
with DCM is a poor prognostic factor and suggestive
of poorly compensated cardiomyopathy.13 Therefore,
Doppler assessment is a valuable tool in the follow-up and
prognostication of patients with DCM.
Fig. 66.7: Dilated cardiomyopathy. Two-dimensional transthoracic
echocardiography. An apical four-chamber view showing functional
mitral regurgitation (FMR). (LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle).

the left atrial and ventricular diastolic pressure is reduced

thus resulting in reduced E-wave velocities and thus an
abnormal E/A ratio of < 1.
The mitral inflow filling pattern enables assessment of
diastolic filling (by determining deceleration time of E and
evaluating for mitral annular early diastolic velocity E').
In addition, mitral annulus systolic velocity (S') and early
diastolic velocity (E') are reduced in patients with DCM.
It also provides useful indices of the volume status of
patients. Studies have shown that the E/E' ratio correlates
well with pulmonary capillary wedge pressure, which
helps in prognosticating patients with cardiomyopathy.11
However, the LV inflow pattern is a less reliable estimate
of LV diastolic function when there is moderate to severe
mitral regurgitation.
Doppler evaluation of mitral regurgitation is another
valuable tool in the assessment of patients with DCM. As
previously mentioned, with a more spherical architecture
of the LV, the papillary muscles are displaced laterally. This
in turn causes tenting of the mitral valve leaflets, which can
worsen mitral regurgitation. Recently, a study12 suggested
that the severity of FMR is an independent predictor of
adverse events in patients with heart failure due to DCM.
Severe FMR, defined as regurgitant volume (RV) > 30 mL
or effective regurgitant orifice > 0.2 cm2 or vena contracta
(VC) > 0.4 cm, was associated with a two-fold increased risk
of adverse events after adjustment for LVEF and restrictive
mitral filling pattern in patients with HF due to DCM. As
a corollary, perhaps FMR is not simply a consequence of

Additional Findings
With long-standing DCM, the mitral regurgitation and
elevated left ventricular diastolic pressures result in left
atrial enlargement. This in turn predispose patients to
atrial fibrillation, which can result in further decrement
of mechanical atrial function. With slow flow within the
atrium, spontaneous echocardiographic contrast can
be noted. Transesophageal echocardiography can often
demonstrate spontaneous echocardiographic contrast
within the left atrium indicative of poor systolic function
among patients with atrial fibrillation. Sometimes,
spontaneous echocardiographic contrast may be also
seen in the LV. Although mural thrombi in the LV are more
frequent in ischemic cardiomyopathy with significant wall
motion abnormalities, they can occur in nonischemic
DCM. They may be laminar, pedunculated, or mobile
based on the duration of onset of systolic dysfunction
(Figs 66.8 and 66.9; Movie clips 66.8A and B; 66.9Part 1
and 2).14

THE ROLE OF ECHOCARDIOGRAPHY

IN OPTIMIZING HEART FAILURE
Patients with advanced conduction system disease
necessitating pacemaker placement benefit from optimizing the pulmonary regurgitation (PR) interval with
echocardiographic guidance. Using Doppler echocardiography to assess mitral inflow, the optimal atrioventricular
delay can be identified as the interval that produces
nonfused E and A velocities without truncating the
duration of A velocity. This has been known to help
improve cardiac output and the patients symptoms.15

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1377

Figs 66.8A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Apical four- (A) and three- (B) chamber
views. Spontaneous echo contrast (arrowhead) is noted within the left ventricle (LV) cavity in B. Arrow points to a pacemaker in the right
heart. (Ao: Aorta; DA: Descending aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle) (Movie clips 66.8A and B).

Figs 66.9A and B: Multiple thrombi in a patient with poor ventricular function. Two-dimensional transthoracic echocardiography. The
movie clip on day 1 shows prominent thrombi (arrows), some of them mobile, in both atria and in the left ventricle (LV). A few show
central echolucencies of varying sizes consistent with clot lysis. Repeat examination on day 2 shows the clots to be smaller and less
prominent, most likely related to significant resorption. There was no clinical evidence for embolization. A and B are representative
frames from the movie clip. (Ao: Aorta; LA: Left atrium; RA: Right atrium; RV: Right atrium) (Movie clips 66.9 Parts 1 and 2).

Among patients with left bundle branch block (LBBB), the

degree of intraventricular dyssynchrony can be estimated
by echocardiographic evaluation. Doppler imaging, strain
imaging, tissue tracking, and tissue synchronization
imaging are useful in quantification of this dyssynchrony.
Dyssynchrony, assessed as the standard deviation of timeto-peak transverse strain, and contractile function assessed
by global longitudinal strain predicted response to CRT.16
Patients with mild to moderate dyssynchrony and good
contractile function derived significant improvement with
CRT in the presence of LBBB compared with patients with

severe dyssynchrony or poor contractile function. This

suggested that the degree of mechanical dyssynchrony
and contractile function may be important determinants
of benefit in CRT and that these measures may provide
incremental value over traditional characteristics such as
LVEF, QRS duration, and LBBB (Figs 66.10A to G; Movie
clip 66.10A to G).
More recently, Doppler tissue imaging (DTI) has been
shown to add incremental information to the clinical
exam of patients with ischemic cardiomyopathy.17 Among
patients who demonstrate a high degree of heterogeneity

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Figs 66.10A to F

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1379

Figs 66.10A to G: Biventricular pacing. Two-dimensional transthoracic echocardiography. Before removal of coronary sinus lead.
Parasternal long-axis (A), short-axis (B), apical four-chamber
(C), and subcostal (D) views. Left ventricle (LV) shows mild to
moderate dysfunction with an estimated ejection fraction of 37%.
The arrow points to a pacemaker lead in the right ventricle (RV).
RV function is normal. After removal of coronary sinus lead due to
symptomatic diaphragmatic stimulation. Parasternal long-axis (E),
short-axis (F), and apical four-chamber (G) views show the marked
deterioration of LV function with an estimated ejection fraction of
1015%. RV function is unchanged. (Ao: Aorta; DA: Descending
thoracic aorta; L: Liver; LA: Left atrium; MV: Mitral valve; RA: Right
atrium) (Movie clips 66.10A to G).

assessed on DTI, it was noted to be an independent

predictor of ischemic cardiomyopathy (ICM) and cardiac
mortality in patients with advanced LV dysfunction.

ECHOCARDIOGRAPHY IN ASSESSING
VENTRICULAR REMODELING
Association between increased LV diameters at baseline
and poor outcome after CRT implant has been described
in some studies.18,19 LV diameter measurements by
M-mode echocardiography allow acceptable estimation
of LVEF and correlate with LV volumes, but are hindered
by a wide margin of error when it comes to accurate LV
size assessment, especially for enlarged ventricles.20 The
addition of parameters such as sphericity index and endsystolic volume enable a more valid and reliable estimate
of the extent of LV dilatation.
Patients with extensive remodeling [end-systolic
volume index (ESVI) > 103 mL/m2] and only one parameter of intraventricular dyssynchrony at baseline
showed no significant changes in ejection fraction after
CRT.21 Therefore, patients with low likelihood to improve
in LV function after CRT were more likely to be those with
larger LVs. Large ESVI at the time of implant was also an
independent and strong predictor of poor outcome during
long-term follow-up (Figs 66.11A and B and Movie clip
66.11).
Furthermore, increased end-systolic volume limits
improvement in LVEF after revascularization in patients
with chronic ischemic cardiomyopathy, despite presence
of viability.22 Therefore, it has also been proposed that

extensively remodeled ventricles might be beyond

recovery with no significant benefit from CRT. Thus,
echocardiographic evaluations complement clinical and
elctrocardiographic parameters in determining the favorability of CRT. However, there remain a few limitations as
echocardiography cannot always predict response to CRT
with absolute certainty and this continues to be an area of
evolving research.
The rapid evolution of ventricular assist devices has
given patients with end stage heart failure, another avenue
of augmenting ventricular function. Echocardiography has
a useful role in evaluating the valvular function among
patients with left ventricular assist devices (LVADs). Patients with LVAD often have significant aortic regurgitation
accompanying poor systolic excursion of the aortic valve
due to the impaired cardiac output. Echocardiography
can be used to assess cavity size in the optimization of
LVAD settings to achieve clinical improvement (Figs 66.12
and 66.13; Movie clips 66.12 and 66.13).

FINDINGS IN DILATED CARDIOMYOPATHY BASED ON ETIOLOGY

Familial
It is often an unrecognized entity, with prevalence in
epidemiological studies, of 2050% among patients with
idiopathic DCM.23,24 Most familial DCM is transmitted
as an autosomal dominant condition, although all
inheritance patterns have been identified (autosomal
recessive, X-linked, and mitochondrial).

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Figs 66.11A and B: Cardiac resynchronization therapy (CRT). Biventricular pacing. Two-dimensional transthoracic echocardiography.
Apical four-chamber view. (A) Patient with cardiomyopathy with poor left ventricle (LV) ejection fraction; (B) Marked improvement in LV
function is noted following biventricular pacing. Arrowhead points to a pacing wire. (LA: Left atrium; MV: Mitral valve; RA: Right atrium;
RV, right ventricle). These are representative frames from the movie clip (Movie clip 66.11).

Figs 66.12A and B: Left ventricular assist device in a patient with cardiomyopathy. Two-dimensional transthoracic echocardiography.
Parasternal long-axis view. (A) Note very poor function of both ventricles. The aortic valve (arrow) does not open and remains in the
closed position throughout because all the ventricular flow is routed by the left ventricle (LV) assist device into the proximal ascending
aorta (Ao). There is no direct ejection of flow into the AO through the aortic valve; (B) Color Doppler examination shows significant
aortic regurgitation (AR). (LA: Left atrium; RV: Right ventricle) (Movie clips 66.12A and B).

Fig. 66.13: Left ventricular assist device. Two-dimensional

transthoracic echocardiography. Parasternal long-axis view. The
ventricular septum is dyskinetic. Arrowhead points to an assist
device in the left ventricle (LV). (Ao: Aorta; RV: Right ventricle)
(Movie clip 66.13).

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

The diagnosis requires the typical features of a DCM,

that is LV dilatation and impaired systolic function
(i.e. LVEF < 4050% or fractional shortening < 25%).
Familial cardiomyopathy is diagnosed in the setting of
a family history of two or more closely related family
members with idiopathic dilated cardiomyopathies.

1381

Peripartum cardiomyopathy is a fairly uncommon but

serious condition with an incidence of 1 in 3,000 to 4,000
live births in the United States. It is seen most commonly
in the African American population, advanced age
pregnancies, and high-risk pregnancies such as twins and
toxemia.25
Traditionally, it is defined as a cardiomyopathy
developing between the last month of pregnancy and
the first 5 months postpartum, in the absence of any
other determinable etiology for heart failure or any
other demonstrable heart disease prior to the last month
of pregnancy.26 This definition has been modified to
include the echocardiographic criteria of left ventricular
dysfunction with ejection fraction < 45%, or M-mode
fractional shortening < 30%, or both and left ventricular
end-diastolic dimension indexed to body surface area of
> 2.7 cm/m2.27,28 Various etiologies postulated include viral
infection, autoimmune mechanisms, hormonal changes,
genetic disorders, and toxemia.26
Echocardiography has been the diagnostic procedure
of choice because it involves no radiation exposure, is
noninvasive, and still provides all the required information.

After the diagnosis has been established, a follow-up

echocardiogram is recommended in the postpartum
period at 6 weeks, 6 months, and yearly thereafter.28
It has been shown that women who did not recover
ventricular function completely had higher left ventricular
end-diastolic dimension and a lower fractional shortening
at the time of diagnosis as compared to those women who
did have complete recovery.29
Most recently, TAPSE (tricuspid annular plane systolic
excursion) has been used to study RV systolic function in
peripartum cardiomyopathy patients as compared to DCM
patients.30 Mean TAPSE was significantly less in postpartum
cardiomyopathy as compared with DCM patients, and a
TAPSE 14 mm was found in the majority of postpartum
cardiomyopathy patients and in about one-third of DCM
patients. Therefore, the RV systolic function (measured
by TAPSE) is worse in peripartum cardiomyopathy
patients as compared to DCM patients. Like other dilated
cardiomyopathies, peripartum cardiomyopathy may
present with a thrombus inside the dilated LV cavity, which
can be seen on 2D or 3D echocardiography (Figs 66.14A
to R; Movie clip 66.14).
Dobutamine echocardiography has been used
to challenge the LV in women who have recovered
from peripartum cardiomyopathy. This permits the
assessment of contractile reserve, which is a measure
of the enhanced contractility with the infusion of an
inotropic agent over baseline values. Patients with
recovered peripartum cardiomyopathy, when compared
with age and parity matched controls, have a lower

Peripartum

Figs 66.14A and B

1382

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Figs 66.14C to H

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

Figs 66.14I to N

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 66.14A to R: Peripartum cardiomyopathy. Morphological assessment of left ventricular thrombus by live three-dimensional transthoracic echocardiography. (A) Arrowhead points to the thrombus attached to left ventricle (LV) apex; (B and C) Transverse plane
(TP, horizontal plane or short axis) section at the attachment point of the thrombus (arrowhead) shows it to be highly echogenic (viewed
en-face, arrow in C); (D and E) TP and longitudinal plane (LP, vertical plane or long axis) sections through the thrombus (arrowhead)
showing the echogenic attachment (arrow) and a large echolucency within the thrombus consistent with lysis; (F to H) TP and both
TP and LP sections at midthrombus level showing clot lysis. (I and J) TP section at thrombus tip level showing a solid rim (viewed
en-face); (K and L) LP section through thrombus showing clot lysis. (M and N) Frontal plane (FP) section through the thrombus viewed
en-face (M) and after tilting (N); (O) TP and LP sections through the thrombus. The data set has been tilted and rotated to show the
position of the FP; (P to R) Oblique sections through the thrombus. The arrow in R points to residual fibrin strands within the lytic area
of the thrombus. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clip 66.14). Source: Reproduced with
permission from Sinha A, et al. Morphological assessment of left ventricular thrombus by live three-dimensional transthoracic echocardiography. Echocardiography. 2004;21:64955.

contractile reserve.31 This might explain why patients with

peripartum cardiomyopathy are at greater risk for relapse
in subsequent pregnancies. This was further clarified
by Fett et al. who showed that patients with a history of
peripartum cardiomyopathy and recovery had a lower risk
of relapse when they demonstrated adequate contractile
reserve as assessed by exercise echocardiography.32 Thus,
the assessment of cardiac reserve also helps to identify

those at risk of relapse, with a higher incidence of relapse

in patients with lower cardiac reserve.

Left Ventricular Noncompaction (LVNC)

Noncompaction of the ventricular myocardium is a
cardiomyopathy thought to be caused by the arrest
of normal embryogenesis of the endocardium and

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

Fig. 66.15: Left ventricular noncompaction. Two-dimensional

transthoracic echocardiography. Contrast study using definity was
performed for better assessment of left ventricle (LV) endocardial
border. Arrows point to multiple trabeculations in the LV apex
indicative of noncompaction. Note very poor LV function often
noted in this entity. (RV: Right ventricle) (Movie clip 66.15).

myocardium. This abnormality may be associated with

other congenital cardiac defects, although it is also seen
in the absence of other cardiac anomalies. Clinical
manifestations are highly variable, ranging from patients
who remain asymptomatic to those with profound CHF,
arrhythmias, and systemic thromboemboli.
The prevalence of noncompaction cardiomyopathy is
estimated to be at 0.05% in the general population based
on echocardiographic studies.33
In normal fetal ontogenesis, the myocardium condenses and intertrabecular recesses are reduced to capillaries.
Deep intertrabecular recesses communicating with the
ventricular myocardium may evolve in some patients
because of arrest of compaction of the loosely interwoven
network of myocardial fibers during intrauterine life.34
Ventricular noncompaction affects the apical and
midventricular segments of both the inferior and lateral
wall in > 80% of the patients35 and generally spares the basal
segments. Segmental appearance is an important feature,
since a more diffuse prominent trabecular pattern may be
present in hypertrophied hearts.36 It is identified by the
presence of more than three prominent trabeculations in
the LV found at autopsy or on various imaging techniques
including echocardiography and magnetic resonance
imaging (MRI).

Echocardiographic Features of LVNC

There are two separate echocardiographic criteria for the
diagnosis of LVNCthe Jenni criteria, which stress the

1385

presence of a two-layered structure and the Chin criteria,

which focus on the depth of recesses compared to the
height of the trabeculae.35,37
The Jenni criteria include:
Ratio of thick noncompacted layer to thin compacted
> 2 and
Perfused intertrabecular recesses are supplied by
intraventricular blood on color Doppler analysis.
The Chin criterion is based on:
Ratio of distance from epicardial surface to the trough
of the trabecular recesses and distance from epicardial
surface to peak of trabeculation < 5.
In both of these, it is important that no other cardiac
structural abnormalities such as valvular obstruction or
coronary anomalies are present. The parasternal shortaxis view of the apex is particularly helpful in visualizing
the recesses (Figs 66.15 to 66.18 and Movie clips 66.15
and 66.17A and B).
Color Doppler helps to differentiate noncompacted
ventricular myocardium from prominent normal myocardial trabeculations, false tendons, aberrant bands,
cardiac tumors, and left ventricular apical thrombus by
demonstrating direct blood flow from ventricular cavity
into deep intertrabecular recesses in noncompacted
myocardium.35
The incremental value of live 3D transthoracic
echocardiography (3D TTE) has been illustrated by
Bodiwala et al. and Baker et al.38,39 This modality allows for
more detailed and accurate diagnosis as the intracavitary
echo densities suspicious for trabeculations can be
tracked in multiple directions from the base to the apex.
Besides viewing the entire trabeculation in detail, there
is a well-demonstrated distinction between prominent
noncompacted and thin compact layer of LV myocardium.
The ability to quantify the depth of penetration of the
intertrabecular recesses in addition to localizing the exact
dimensions and severity of noncompaction provides
prognostic value. Evidence of extensive left ventricular
involvement is associated with a poorer prognosis and
prompt referral for transplantation, or prophylactic
defibrillator implantation has been suggested to be useful
in this situation.40
Further, it also enables distinguishing LVNC from
other differential diagnoses such as left or right ventricular
hypertrophy, apical hypertrophic cardiomyopathy (HCM),
right or left ventricular dysplasia, endocardial fibroelastosis,
and intracardiac masses such as thrombus and tumors.
Differentiation from these entities is enhanced by full
characterization of segmental involvement, especially of

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Figs 66.16A to E: Left ventricular noncompaction. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A),
apical three-chamber (B), apical four-chamber (C and D) views
showing the trabeculations of the LV wall and deep intertrabecular
recesses (arrowheads) characteristic of left ventricle (LV)
noncompaction. (E) Apical two-chamber view with color Doppler
showing direct blood flow from the ventricular cavity into deep
intertrabecular recesses in LV noncompaction. (Ao: Aorta; LA: Left
atrium; RA: Right atrium; RV: Right ventricle).

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1387

Figs 66.17A and B: Left ventricular noncompaction. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and
apical two-chamber (B) views with Doppler evidence of flow in the recesses (B). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; MR: Mitral
regurgitation; MV: Mitral valve; RV: Right ventricle) (Movie clips 66.17A and B).

Figs 66.18A to C: Isolated left ventricular noncompaction.

Two-dimensional transthoracic echocardiography. (A) Arrow points
to prominent trabeculations involving the whole extent of left
ventricular (LV) posterolateral wall consistent with noncompaction;
(B) Myocardial perfusion study using perflutren lipid microspheres
demonstrated apical hypoperfusion (arrowhead); (C) Echo contrast
timeintensity curves show diminished rate of filling as well as peak
filling in the LV apex as compared to the ventricular septum (VS).
(LA: Left atrium; MV: Mitral valve; RA: Right atrium; RV: Right
ventricle).
Source: Reproduced with permission from Bodiwala K, et al. Live
three-dimensional transthoracic echocardiographic assessment of
ventricular noncompaction. Echocardiography. 2005;22:61120.

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

the right ventricular apex and by correct measurement

of the noncompacted/compacted ratio (N/C ratio). This
is best performed in cross-sectional views obtained
perpendicular to the hearts axis,38 and is superior with 3D
TTE.
Therefore, the full characterization of noncompaction
with 3D TTE is of clinical value. In addition, 3D TTE
may potentially offer a better modality for following
patient response to therapy, including use of -blocking
agents that have been associated with improvements in
ventricular function (Figs 66.19 to 66.25; Movie clips 66.19
[parts 1 to 5], 66.20, 66.22A to C, 66.23A to C, and 66.24
[parts 1 and 2]).
When conventional images are of poor quality or
nondiagnostic, contrast agents can be used to enhance
endocardial border delineation.41,42 The opacification
within the deep recesses can help confirm the diagnosis.
Contrast can also be used to determine the associated
presence of thrombi with and without clot lysis.

Takotsubo Cardiomyopathy/Left
Ventricle Apical Ballooning Syndrome
Takotsubo cardiomyopathy, also called stress-induced
cardiomyopathy or broken heart syndrome, is an
increasingly reported syndrome characterized by transient
systolic dysfunction of the apical and/or mid segments
of the LV that occurs in the setting of acute emotional
stress. The initial presentation often mimics that of an
acute MI with concomitant rise in cardiac biomarkers,
electrocardiographic abnormalities, and in severe
cases with features of acute CHF.43 However, coronary
angiography does not reveal a significant obstruction
that can explain the nature or magnitude of systolic
dysfunction noted. The etiology is thought to be related to
the hyperadrenergic state associated with emotional stress
or psychological trauma.
The classical finding of apical ballooning (typical
variant) and/or midventricular hypokinesis is usually
seen on left ventriculography or echocardiography.4345 In
a minority of cases, the transient left ventricular hypokinesis
is restricted to the midventricular segments (atypical
variant or apical sparing variant) without involvement
of the apex.46 As reported by Kurowski et al. the atypical
variant may account for nearly 40% of patients with
Takotsubo cardiomyopathy.46 More recently, Manzanal
et al.47 also described a case series of patients with
the atypical variant, the so-called inverted Takotsubo

cardiomyopathy, characterized by basal and midventricular

segmental akinesis. At times, right ventricular dysfunction
can accompany left ventricular dysfunction (Figs 66.26A
and B Movie clips 66.26A and B).48
The left ventricular dysfunction is reversible with
recovery of ventricular function anticipated with medical
management as outlined for DCM.46

Tachycardia-Induced Cardiomyopathy
and Other High-Output States
Tachycardia-induced cardiomyopathy is a well-recognized
entity leading to DCM, but its exact incidence is still
unknown. It should be suspected in patients with atrial as
well as ventricular arrhythmias including atrial fibrillation,
atrial tachycardias, re-entrant arrhythmias, ventricular
tachycardias, and premature ventricular contractions
(PVCs) resulting in persistently high heart rates.
It has all the features of DCM on echocardiography
with the exception that LV end-diastolic dimension (EDD)
tends to be smaller in patients with tachycardia-mediated
cardiomyopathy.49 Follow-up of the ventricular function
after a period of 36 months with satisfactory rate control
will often demonstrate significant recovery of function
(Fig. 66.27A and B; Movie clips 27A and B).

Ischemic Cardiomyopathy (ICM)

Ischemic cardiomyopathy is a form of DCM characterized
by systolic dysfunction due to CAD. The prevalence of
ischemic cardiomyopathy in the United States is nearly
two-thirds of all cases of chronic systolic dysfunction.50
Patients with CAD have concomitant risk factors such as
hypertension, which can independently cause a decline in
systolic function.
The echocardiographic features are typical of DCM.
An important finding is that of significant wall motion
abnormalities independent of conduction system abnormalities, consistent with a territory of ischemia on prior
infarction.
Revascularization can result in improvement of
ventricular function. However, with long-standing cardiomyopathy, evidence of increased end-systolic volume
limits improvement in LVEF after revascularization in
patients with chronic ischemic cardiomyopathy despite
the presence of viability. Further, secondary features
on echocardiography such as myocardial thinning with
increased echogenicity and akinesis or dyskinesia of the
left ventricular segment suggest that the systolic function

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

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Figs 66.19A to E: Combined left and right ventricular noncompaction. Live/real time three-dimensional transthoracic echocardiography. (A and B) Arrows point to prominent trabeculations in
both ventricles; (C) Arrows show multiple prominent trabeculations
in RV; (D) Transverse cropping of left ventricle (LV) apical
area shows a honeycomb-like appearance (arrow) typical of
noncompaction; (E) Echo contrast study using perflutren lipid
microspheres shows filling of intertrabecular recesses with
the contrast agent (arrows). See Movie clip 66.19, Part 1 to 5.
Systemic cropping of the three-dimensional data set demonstrates
extensive trabecular involvement of the LV (arrowheads). (AV:
Aortic valve; LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
RA: Right atrium; RV: Right ventricle) (Movie clips 66.19 Parts 1
to 5). Source: Reproduced with permission from Bodiwala K, et al.
Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography. 2005;
22:61120.

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Figs 66.20A to F

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1391

Figs 66.20A to K: Combined left and right ventricular noncompaction.

Live/real time three-dimensional transthoracic echocardiography.
(A and B) Arrows point to a cauliflower-like mass in right ventricle
(RV) mimicking a tumor. Magnetic resonance imaging in this patient
erroneously suggested a ventricular septal tumor; (C to E) Arrows
point to massive trabeculations in both left ventricle (LV) and RV
consistent with noncompaction; (F and G) Transverse (F) and
anteroposterior (G) cropping of LV apical area show a honeycomblike appearance (arrow) typical of noncompaction; (H) Arrowheads
point to multiple trabeculations occupying over 80% of RV cavity; (I
and J) Arrow points to massive trabeculations in the ventricular septal
area; (K) Arrows point to multiple trabeculations crossing the RV
cavity transversely. (Ao: Aorta; AV: Aortic valve; LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; RA: Right atrium; RCA: Right coronary
artery; RV: Right ventricle; TV: Tricuspid valve; # 1, anterior leaflet of
tricuspid valve, # 2, septal leaflet of tricuspid valve) (Movie clip 66.20).
Source: Reproduced with permission from Bodiwala K, et al. Live
three-dimensional transthoracic echocardiographic assessment of
ventricular noncompaction. Echocardiography. 2005;22:61120.

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Fig. 66.21: Isolated right ventricular noncompaction. Live/real time

three-dimensional echocardiography. In this patient, prominent
trabeculations (arrowhead) occupy over 80% of the right ventricular
(RV) cavity.

Figs 66.22A to C: Isolated right ventricular noncompaction. Twodimensional and live/real time three-dimensional transthoracic
echocardiography. (A) Two-dimensional study. Arrowheads point
to a few muscle bands in the right ventricular apex but there is
no clear-cut evidence for noncompaction; (B) Three-dimensional
study. Cropping of the image reveals a honeycomb-like appearance typical of RV noncompaction. Trabeculations (arrowhead) fill
the distal 40% of RV almost completely; (C) Intracardiac echocardiography. Shows RV noncompaction (arrowhead). (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie
clips 66.22A to C).
Source: Reproduced with permission from Reddy VK, et al.
Incremental value of live/real time three dimensional transthoracic
echocardiography in the assessment of right ventricular masses.
Echocardiography. 2009;26:598609.

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

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Figs 66.23A to C: Isolated left ventricular noncompaction and thrombi

in an adult patient. (A) Two-dimensional transthoracic echocardiography. Arrowheads show echodensities in the LV suggestive of thrombi
with areas of echolucency consistent with clot lysis. The arrow points
to adjacent trabeculations which are relatively less echogenic; (B and
C) Live/real time three-dimensional transthoracic echocardiography.
Arrows point to multiple trabeculations in left ventricle (LV). In C,
one of the echodensities (arrowhead) was cropped revealing a
prominent echolucency, typical of clot lysis (Movie clips 66.23A to C).
Source: Reproduced with permission from Yelamanchili P, Nanda NC,
Patel V, et al. Live three-dimensional echocardiographic demonstration
of left ventricular noncompaction and thrombi. Echocardiography.
2006;23:7046.

Fig. 66.24: Isolated left ventricular noncompaction and thrombus

in another patient. Live/real time three-dimensional transthoracic
echocardiography. Arrowhead points to a clot in the left ventricle
(LV). Clot hypermobility (arrowhead) is demonstrated in the Movie
clip 66.24, Part 1. Movie clip 66.24, Part 2 is from another patient
with isolated left ventricular noncompaction with thrombi in the LV
cavity. The thrombi (T) are easily differentiated from trabeculations
(arrowheads) by their much higher echogenicity. Thrombi are
denoted by arrowheads in sections of the movie containing no
trabeculations. (RV: Right ventricle) (Movie clips 66.24 Parts 1 and 2).
Source: Reproduced with permission from Yelamanchili P, Nanda NC,
Patel V, et al. Live three-dimensional echocardiographic demonstration
of left ventricular noncompaction and thrombi. Echocardiography.
2006;23:7046.

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Fig. 66.25: Left ventricular noncompaction. Shows the

technique of estimating left ventricle (LV) mass in by live/real
time three-dimensional transthoracic echocardiography.
Source: Reproduced with permission from Rajdev S, Nanda
NC, Singh A, Baysan O, Hsiung MC. Comparison of twoand three-dimensional transthoracic echocardiography
in the assessment of trabeculations and trabecular mass in
left ventricular non-compaction. Echocardiography. 2007;24:
7607.

B
Figs 66.26A and B: Takotsubo cardiomyopathy. Apical four-chamber view. Velocity vector imaging. The individual arrows point
to the direction of endocardial motion, while the lengths are proportional to velocity. (A) Compared to baseline (left figure), the
velocity of motion has significantly increased in the left ventricle (LV) mid segments, while the apex is still hypokinetic during follow
up (right figure); (B) No change is noted by visual inspection in the left atrial (LA) wall motion during follow-up (right figure) as compared to baseline (left figure). This patient belonged to a series of five patients with Takotsubo cardiomyopathy, in whom the left
atrium also appeared to be involved by velocity vector imaging with a statistical tendency toward improvement during follow-up.
(RA: Right atrium; RV: Right ventricle). These are representative frames from the movie clips. (Movie clips 66.26A and B).

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

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Figs 66.27A and B: Tachycardia-induced cardiomyopathy. Two-dimensional transthoracic echocardiography. (A) Parasternal longaxis view. The left ventricle (LV) is dilated with poor function. The heart rate was 133/min; (B) Apical four-chamber view. The patient was
placed on a -blocker, which resulted in heart rate reduction and improvement in LV function. (Ao: Aorta; DA: Descending aorta; LA: Left
atrium; MV: Mitral valve; RA: Right atrium; RV: Right ventricle) (Movie clips 66.27A and B).

Figs 66.28A and B: Ischemic cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical
four-chamber (B) views. All the chambers are markedly dilated with extremely poor function of both ventricles. The septum is thin and
echogenic, consistent with scarring and fibrosis indicating ischemic heart disease. Arrowhead points to a pacemaker wire. (Ao: Aorta;
LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right ventricle) (Movie clips 66.28A and B).

is unlikely to benefit from revascularization (Figs. 66.28A

and B; Movie clips 66.28 A and B).
Therefore, it has also been proposed that extensively
remodeled ventricles might be beyond recovery with no
significant benefit from revascularization. Thus, echocardiographic evaluations complement clinical parameters
in determining the favorability of revascularization in
ischemic cardiomyopathy.

Arrhythmogenic Right Ventricular

Cardiomyopathy/Dysplasia (ARVC/D)
ARVC/D is a genetically predetermined disorder, often
causing sudden death among young, apparently healthy

individuals. Its prevalence is estimated between 1:2,000

and 1:5,000.51 It is characterized by fibrofatty infiltration
of right ventricular myocardium, which often leads to
ventricular arrhythmias, RV dilation, and dysfunction,
ultimately leading to RV failure.52,53 Criteria for the clinical
diagnosis of ARVC/D were proposed in 1994 by the
International Task Force, based on structural, histological,
ECG, arrhythmic, and familial features of the disease.54
Various echocardiographic findings reported in
ARVC/D patients include:
1. Abnormalities of RV morphology (trabecular derangements, hyper-reflective moderator band, focal RV
aneurysms, and sacculations);55

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an abnormal trabecular pattern, a highly reflective and

irregularly shaped moderator band, or an isolated dilation
of the RVOT.55
The use of Doppler tissue imaging and 2D strainderived parameters to assess regional contractility to
identify ARVC/D has been reported,59 but this is not yet a
standard criterion. In addition, there is an increased use
of 3D echocardiographic measurements of RV volume
and ejection fraction, which have been shown to correlate
with MRI values and hence can be used in prognostication
as well as in follow-up of patients (Fig. 66.29; Movie
clip 66.29).60,61

Toxic Cardiomyopathies
Fig. 66.29: Arrhythmogenic right ventricular dysplasia. Twodimensional transthoracic echocardiography. Apical four-chamber
view shows diminished right ventricular (RV) function as well
as presence of small, localized berry-like aneurysms involving
the free wall (arrows) typical of this entity. The left ventricle (LV)
function is normal. (LA: Left atrium; MV: Mitral valve; RA: Right
atrium) (Movie clip 66.29A).

2. Abnormal RV structure (increased end-diastolic and

end-systolic RV inflow tract dimensions and RVOT
dimension in long- and short-axis views);56 and
3. Abnormal RV function (global hypokinesis or regional
wall motion abnormalities).57
It has been reported that RVOT > 30 mm is the most
common abnormality associated with Task Force Criteria
diagnosis of ARVC/D, followed by RV dysfunction.57
Over the past years, advancement in technology and
imaging including cardiac MRI, 3D echo, and contrast
echo have led to the addition of more quantitative imaging
parameters to improve diagnostic sensitivity while maintaining the diagnostic specificity.58
These include:
Major criterion:
1. Regional RV akinesia/dyskinesia/aneurysm
2. Parasternal long-axis RVOT (end-diastole) > 32 mm
or parasternal short-axis RVOT > 36 mm or fractional
area change < 33%
Minor criterion:
1. Regional akinesia/dyskinesia
2. Parasternal long-axis RVOT > 29 to < 32 mm or parasternal short-axis RVOT > 32 to < 36 mm or fractional
area change > 33% to < 40%
Echocardiographic findings reported in asymptomatic
individuals, who were family members of patients with
ARVC/D includedinferobasal localized diastolic bulge,

Chemotherapy-Induced Cardiomyopathy
Chemotherapy with adriamycin or other anthracyclines is
often adopted for a variety of hematological malignancies.
It, however, is associated with cardiotoxicity and can be
an issue among adult patients receiving chemotherapy
as well as survivors of childhood cancers.62 The toxicity is
likely due to a combination of factors including reactive
oxygen species generation and myocardial cellular
toxicity due to adriamycin itself. While acute toxicity can
result in cardiomyopathy that can in part be reversed
by discontinuation of the chemotherapy, late onset of a
cardiomyopathy is irreversible.
Late onset chronic progressive cardiomyopathy occurs
more than 1 year after anthracycline treatment.63,64 With
the loss of cardiomyocytes, cardiac function begins to
deteriorate with resultant LV wall thinning and in some
cases, progressive LV dilation.65,66
Echocardiographic abnormalities may include
decreased LV fractional shortening, end-diastolic posterior
wall thickness, and mass. Associated would be decreased
LV contractility and increased LV afterload. Left ventricular
dimension may be increased, normal, or decreased
(Figs 66.30A and B; Movie clip 66.30). Strain rate imaging
is a new technique to evaluate myocardial mechanics
and can detect early changes prior to a decrement in
the ejection fraction. This technique will enable early
detection of patients at risk of cardiomyopathy and would
be useful in managing chemotherapy regimens.
Although adults typically develop chronic DCM
after anthracycline chemotherapy, children at the end
of anthracycline treatment have a DCM, which may
then progress to a RCM.66 Afterload increases, in spite of
normal-to-reduced blood pressure caused by decreased
LV wall thickness and mass. Therefore, the elevated LV
afterload reduces the cardiac output to a greater extent

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1397

Figs 66.30A and B: Adriamycin-induced cardiomyopathy. Two-dimensional transesophageal echocardiography. Parasternal long (A)
and short; (B) axis views. Left ventricle (LV) is markedly enlarged and severely hypokinetic. The right ventricle (RV) is also very hypokinetic. Arrow points to a catheter in the right heart. (LA: Left atrium; RA: Right atrium) (Movie clip 66.30).

Fig. 66.31: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Left ventricle (LV) short-axis view at the
level of the papillary muscles shows decreased rotation of the
ventricle in systole and unrotation in diastole. This is a representative frame from the movie clip demonstrating a dilated LV.
(RV: Right ventricle). (Movie clip 66.31).

than the reduced LV systolic performance alone. While

adriamycin is among the more commonly used agents with
significant cardiotoxicity resulting in cardiomyopathy,
all chemotherapeutic agents can potentially result in
varying degrees of cardiomyopathy too due to their toxicity
profile.

Alcohol-Induced Cardiomyopathy
Alcohol-induced cardiomyopathy (ACM) is among the
leading causes of nonischemic DCM in the United States.67

Long-term heavy alcohol consumption, estimated to be

>90 g of alcohol a day for more than 5 years is associated
with a higher risk to develop ACM.
It often occurs in stages, beginning with a preclinical
or asymptomatic stage, and then progressing to a symptomatic stage. The early signs of ACM appear to be LV
dilation, with an increase in EDD and increased systolic
dimension. It also results in increased LV mass, and
modestly increased posterior and septal wall thickening.
Associated is an increase in isovolumic relaxation time and
deceleration time.68 Diastolic dysfunction thus, appears
to be an early finding. However, patients may have both
diastolic and/or systolic dysfunction with progression
of the condition. Patients may also have some degree of
wall thickening which when coupled with LV dilation will
serve to offset wall tension. Therefore, many patients may
indeed remain in a compensated and asymptomatic state
even with LV dysfunction.
As the condition progresses, more classical features of
DCM are seen with severely impaired systolic function and
progressive LV dilation (Fig. 66.31 and Movie clip 66.31).

RESTRICTIVE CARDIOMYOPATHY
The distinct morphological and hemodynamic characteristics that separate restrictive from the more common
dilated and hypertrophic cardiomyopathies are:
1. Nondilated ventricle (<40 mL/m2) with normal wall
thickness (<11.2 cm)

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

2. Rigid ventricular walls with severe LV diastolic

dysfunction with restrictive filling leading to elevated
filling pressures
3. Biatrial enlargement
4. Normal systolic function in majority of patients
5. Normal pericardium
These features are identified with 2D echocardiography as the initial diagnostic test using the Doppler
inflow velocities and tissue Doppler techniques.
RCM is classified based on the etiology69 as follows:
1. Noninfiltrative disordersidiopathic RCM, familial
cardiomyopathy, HCM, scleroderma, pseudoxanthoma
elasticum, and diabetic cardiomyopathy. Familial
disease (unrelated to amyloidosis) may overlap with
familial HCM and may be caused by some of the same
gene mutations.
2. Infiltrative disordersamyloidosis, sarcoidosis, Gaucher
disease, Hurler syndrome, and fatty infiltration
3. Storage diseaseshemochromatosis, Fabry disease
(FD), and glycogen storage disease
4. Endomyocardial diseasesfor example, endomyocardial fibrosis (EMF) and hypereosinophilic syndrome
5. Radiation and/or drug (e.g. anthracycline) toxicity can
cause an RCM or DCM.

Doppler Findings
Evaluation by Doppler echocardiography is crucial to
the diagnosis of RCM. With advanced disease, often an
elevated peak mitral inflow velocity, rapid early mitral
inflow deceleration and reduced early annular velocity
on tissue Doppler imaging is noted. Pertinent values to be
considered in the diagnosis include:
1. The E/A ratio of mitral valve inflow is pathologically
elevated typically > 2.070 and the deceleration time is
shortened to < 160 milliseconds.
2. Doppler tissue imaging of the mitral annulus also
reveals abnormally low diastolic Doppler annular
velocities typically < 10 cm/s.
3. The high mitral inflow velocities with low annular
velocity also result in a particularly high E/E' ratio > 25.
4. Since restrictive cardiomyopathies are usually global,
the RV may also be affected. Hypertrophy can be seen
in the right ventricle due to pressure overload and also
abnormal tricuspid valve inflow velocities. These can
lead to abnormal hepatic vein flow as well.
The many different etiologies of RCM also show
characteristic patterns on echocardiography.

Amyloid Cardiomyopathy
The annual incidence of systemic amyloidosis is approximately 610 cases per million of the general population.
Evidence suggests that the incidence of cardiac amyloidosis is increasing as the mean life expectancy rises. This
may also be due, in part, to improvements in diagnostic
modalities that have led to earlier identification of this
condition.71
The development of a cardiomyopathy results from the
deposition of amyloid within the myocardium. It presents
predominantly as a RCM characterized initially by diastolic
dysfunction, progressing to profound biventricular systolic
dysfunction and arrhythmias. The two most common forms
involve deposition of monoclonal light chain fragments
in the myocardium (termed primary amyloidosisAL),
or fragments of serum amyloid in secondary amyloidosis
(secondary amyloidosisAA). Endomyocardial biopsy
shows interstitial prominence with massive amyloid
deposits and varying size myocardial cells often containing
vacuoles.
In cardiac amyloidosis, echocardiography demonstrates symmetric left ventricular wall thickening typically
involving the interventricular septum, small ventricular
chambers, thickening of the atrial septum, pericardial
effusion, and dilated atria.72,73 Right ventricular diastolic
dysfunction may also be present in association with
increased right ventricular wall thickness.73 The typical
findings of biventricular wall thickness usually precede
multichamber dilation seen in the later stages. Intracardiac
thrombi may be present in up to 35% of patients with the
AL type and up to 15% in other types74 of amyloidosis
(Figs 66.32A to C; Movie clips 66.32A and B).
With progression of amyloidosis, decreased systolic
thickening of the ventricular septum and globally
decreased LVEF ensues. The ventricular walls show a
granular and brightly reflective sparkling appearance,
corresponding to amyloid particle deposition (Figs 66.33A
to H; Movie clip 66.33).
The cardiac valves typically have a thickened
appearance and although their movement is usually
normal, valvular regurgitant flow is often detected.72
Interestingly, it has been suggested that the characteristic
speckle pattern might not be as obvious when using
harmonics on later-generation echocardiography
machines.71 The use of echocardiography to differentiate
cardiac constriction from RCM, while not definitive,
is certainly helpful. The measure of the transvalvular
diastolic velocities across the atrioventricular valves is

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

1399

Figs 66.32A to C: Amyloid. Two-dimensional transthoracic

echocardiography. Parasternal long-axis (A) and apical fourchamber (B) views in a 86-year-old female patient with amyloidosis.
All chamber walls are echogenic and markedly hypokinetic due
to the infiltrative process. The ventricular walls appear thickened
because of amyloid deposition, not hypertrophy; (C) Tissue Doppler
examination shows diminished mitral E- and A-waves (arrow).
(Ao: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA:
Right atrium; RV: Right ventricle; TV: Tricuspid valve) (Movie clips
66.32A and B).

A
Figs 66.33A and B

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Figs 66.33A to H: Amyloidosis. Two-dimensional transthoracic echocardiography. Both the ventricular septum (IVS) and left ventricle
(LV) posterior wall (PW) are echogenic and markedly thickened because of amyloid deposition, not hypertrophy. These are visualized
in parasternal long- (A systole, B diastole) and short- (C systole, D diastole) axis as well as apical four-chamber (E systole, F diastole)
views. LV function is diminished; (G) M-mode examination showing similar findings; (H) Tissue Doppler imaging of the medial mitral
annulus shows a low E' velocity of 3.8 cm/s (arrow) consistent with poor longitudinal shortening of LV. (Ao: Aorta; DA: Descending aorta;
MV: Mitral valve; RA: Right atrium; RV: Right ventricle; RVW: Right ventricular wall) (Movie clips 66.33A to C).

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

particularly useful. With constriction, there is a > 25%

variation in the mitral and tricuspid velocities during
inspiration, while with amyloidosis only the tricuspid
velocity changes with little change in the mitral velocity.
In addition, in amyloidosis, the change in velocities is
concordant between both valvesthat is, they increase
and decrease at the same time, whereas in constriction the
opposite is the rule.
These findings are valuable especially in identifying
patients who can present in the advanced stages of the
disease when systolic function is depressed. These signs of
a mixed picture of dilated and restrictive cardiomyopathy
are more classical with advanced cardiac amyloidosis.

Fabry Disease
Fabry disease (FD) is a lysosomal storage disease,
caused by mutations in the gene encoding the enzyme
-galactosidase A, resulting in a deficit in enzyme activity.
It is X-linked and is characterized by the progressive
accumulation of glycosphingolipids. Multiple organs can
be affected with the heart, kidneys, and the neurological
system in particular being common targets.75
Cardiac involvement is quite frequent and is one of the
main causes of death in patients with advanced FD. In the
heart, glycosphingolipids deposition causes progressive
left ventricular hypertrophy (LVH) that can at times mimic
the morphological and clinical characteristics of HCM.76

Fig. 66.34: Fabry disease. Two-dimensional transthoracic echocardiography. Arrow shows an echolucency within the myocardium
of the ventricular septum, which has been reported in patients with
this entity. This finding reflects glycosphingolipids compartmentalization in which the subendocardial layer is spared resulting in
an echolucent area. Note that left ventricle (LV) function is poor.
Arrowhead in the Movie clip 66.34 points to an intracardiac defibrillator lead. (LA: Left atrium; RA: Right atrium; RV: Right ventricle).

1401

Males with specific gene mutations77 and heterozygous

females with low enzyme activity78 can present with a
cardiac variant of the disease. It is characterized by progressive concentric LVH, often symmetric and occasionally
asymmetric, which correlates with disease severity.79,80
The predominant finding on echocardiography is usually
wall thickening without cavity dilatation, with excessive LV
mass (up to 240 g/m2) in some male patients.78
While systolic function is usually preserved early
on, a pseudonormal filling pattern can be detected by
careful Doppler transmitral and pulmonary venous flow
analysis, which suggests increased LV filling pressures. A
prolongation of the deceleration time is often noted, and
Linhart et al. also demonstrated that up to 25% of patients
had an impaired LV relaxation pattern, indicating less
severe diastolic dysfunction.78,80
The basis of the cardiac hypertrophy in FD is different
from that seen in other infiltrative cardiomyopathies where
predominantly interstitial infiltration is encountered. The
deposits in FD are lysosomal and represent only a small
part of the increase in LV mass,77 with true ventricular
hypertrophy as a result of neurohormonal influences.
Valvular changes are seen and are likely related to the
glycosphingolipid deposits and fibrosis of valvular tissue.
In the case of the mitral valve, papillary muscle thickening
can be found accompanying ventricular hypertrophy.
These result in valvular regurgitation that is usually of mild
to moderate grade. Among patients with asymmetrical
septal hypertrophy, mimicking hypertrophic obstructive
cardiomyopathy, the typical systolic anterior motion of
the anterior mitral leaflet, can contribute to mitral valve
dysfunction. The mitral valve appears to be affected in
relatively young subjects, whereas aortic abnormalities
appear later. With progressive LVH, aortic root dilatation
can be seen. This may be accompanied by the development
of aortic valvular regurgitation, which is seldom severe
and usually mild (Fig. 66.34; Movie clip 66.34).80
With progression of the disease, myocardial fibrosis
ensues and is accompanied by left ventricular systolic
dysfunction.81 Strain imaging is particularly useful in
estimating myocardial fibrosis. The extent of myocardial
fibrosis correlates with the loss in peak systolic strain.81
Therapeutic options include the use of enzyme
replacement therapy (ERT) to prevent the extensive
deposition of glycosphingolipids. ERT is effective in
reversing the microvascular changes in FD by catabolizing
the lipid deposits and improving cardiac function in
patients with cardiac involvement.82
A recent study has also shown the benefit of emerging
echocardiographic techniques in the early diagnosis of

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cardiac involvement in FD.83 Through the use of speckle

tracking to evaluate myocardial longitudinal strain, the
study found that the presence of at least one strain value
15% demonstrates subclinical myocardial dysfunction
in patients with preclinical FD without myocardial
hypertrophy. Therefore, this provides evidence of ventricular dysfunction even with the absence of LVH and prior
to extensive deposition within the LV.
Weidemann et al. have shown that among patients
receiving ERT, the maximal benefit occurred in those
without myocardial fibrosis.81 Given the emphasis for early
diagnosis and institution of treatment, echocardiography
arguably has an important role in the management of FD.
Especially with the use of emerging techniques to guide
therapy, ERT may be able to prevent complications such as
LVH, irreversible myocardial fibrosis, lethal arrhythmias,
and coronary heart disease.

Hypereosinophilic Cardiomyopathy
The WHO published a recent update that outlined the
diagnosis of hypereosinophilic syndrome (HES).84 These
conditions are characterized by:
1. Persistent marked eosinophilia (>1,500 eosinophils/
mm3);
2. The absence of a primary cause of eosinophilia (such
as parasitic or allergic disease); and
3. Evidence of eosinophil-mediated end organ damage.
Cardiac involvement unrelated to hypertension,
atherosclerosis, or rheumatic disease is identified in 20%
of patients (only 6% at the time of initial presentation).85
Typical cardiac findings include endocardial fibrosis and
mural thrombus, which is most frequent in the apices of
both ventricles and is also characterized by progressive
heart failure.
The pathophysiology involves eosinophil infiltration
of cardiac tissue and release of toxic mediators resulting
in endocardial damage and formation of platelet thrombi.
These mural thrombi pose a high risk for embolization.
Later with disease progression, there is fibrous thickening
of the endocardial lining leading to a RCM.86,87
Hence, three stages of pathophysiologynecrosis,
thrombosis, and fibrosis are usually identified. Endomyocardial fibrosis (EMF), which was initially described in
1936 by Loeffler, is the most characteristic cardiovascular
abnormality.88 Valvular insufficiency can result from mural
endocardial thrombosis and fibrosis involving leaflets of
the mitral or tricuspid valves.89,90 In addition, entrapment

of the chordae tendineae can occur with progressive

scarring, which also results in mitral and/or tricuspid
valve regurgitation.

Findings on Transthoracic
Echocardiography
Echocardiographic evaluation during the necrosis stage
is usually normal. The hallmark echocardiographic
finding is the persistent obliteration of the apex of the
left or right ventricle, or both, by laminar thrombus.91 The
common differential diagnosis of LV apical infiltration and
obliteration include LV apical thrombus and apical HCM,
which can be differentiated by their echocardiographic
characteristics. A LV apical thrombus is associated with
underlying LV dysfunction and wall motion abnormality. In
apical cardiomyopathy, the LV apex is visualized in diastole
with obliteration in systole, thus producing the peculiar
ace of spades configuration. HES, however, shows
persistent apical obliteration, mitral valve involvement,
and progressive features of a RCM. Resolution of the
apical infiltration may sometimes be observed if treated
appropriately by medical and surgical intervention.
Tricuspid and pulmonary valvular thickening may
accompany right ventricular involvement with thrombus
formation in the RV apex. Doppler evaluation of the valves
enables the estimation of valvular regurgitation and the
determination of restrictive physiology (Figs 66.35A to H;
Movie clips 66.35A to E).

Contrast Echocardiography
Contrast echocardiography may be invaluable in the
diagnosis of hypereosinophilic cardiomyopathy enabling
it to be differentiated from a thrombus or apical HCM.
Specifically since it delineates the shape of LV, it
distinguishes between hypertrophy and complete persistent obliteration in these conditions.92

Transesophageal Echocardiography
When the transthoracic echo images are poor or limited,
transesophageal echocardiography is useful. The deep
transgastric views enable visualization of the LV apex and
can demonstrate the obliteration of the LV cavity thus
confirming the diagnosis.

Endomyocardial Fibrosis
In the third stage of HES, significant EMF ensues, resulting
in a RCM. It is considered a particularly devastating

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

Figs 66.35A to F

1403

1404

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 66.35A to H: Loeffler endocarditis. Two-dimensional transthoracic echocardiography. Baseline. (A and B) Modified four-chamber
view. (A) Arrowhead shows marked thickening of the tricuspid valve (TV). The arrow points to a large mass in the right ventricular
(RV) apex. A small pericardial effusion (PE) is also noted; (B) Color Doppler examination. Arrowhead shows moderate to severe TV
regurgitation; (C and D) Aortic (AO) short-axis view; (C) Shows marked thickening of the pulmonary valve (PV); (D) Color Dopplerguided continuous wave Doppler interrogation shows a peak gradient of 48.50 mm Hg consistent with moderate PV stenosis (arrow).
After therapy; (E and F) Modified four-chamber view. Show complete normalization of TV and RV apex. Arrowhead points to moderator
band. PE is absent; (G and H) Aortic short-axis view; (G) Thickening involving the PV has completely regressed and the valve appears
structurally normal (arrowhead); (H) Color Doppler-guided continuous wave Doppler examination shows normal flow velocities across
the PV (arrow). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium) (Movie clips 66.35A to E).
Source: Reproduced with permission from Garg A, Nanda NC, Sungur A, et al. Transthoracic echocardiographic detection of pulmonary
valve involvement in Loefflers endocarditis. Echocardiography (2013, in press).

Fig. 66.36: Endomyocardial fibrosis in a 62-year-old female with

a previous history of mansoni schistosomiasis. Two-dimensional
transthoracic echocardiography. Apical four-chamber view
shows endocardial thickening and cavity obliteration of both
ventricular apices with greater involvement of the left ventricle
(LV). The atria are enlarged, which is typical of restrictive
cardiomyopathy. The patient improved after heart failure treatment.
Source: Reproduced with permission from Carneiro Rde C et al.
Endomyocardial fibrosis associated with mansoni schistosomiasis.
Rev Soc Bras Med Trop. 2011;44(5):6445.

disease in the tropical region with an estimated 10 million

people affected by it.93 The association between this condition and parasitic infestation has been established by
multiple studies such as those of Rashwan et al. and
Mocumbi et al. 94,95
Echocardiography is particularly useful in identifying
the condition. It is characterized by obliteration of the
ventricular apices with progression of the fibrocalcific
process, spontaneous echo contrast in the ventricles
without significant systolic dysfunction, and significant
atrioventricular valve dysfunction due to adhesion of
the valve apparatus to the ventricular wall. These are
considered the major criteria used to assess the severity of
EMF. The minor criteria include severely dilated atria with
normal ventricular size, restrictive flow pattern across the
mitral or tricuspid valve, and thickening of the anterior
mitral leaflet.95 As the condition progresses, more and
more of the left ventricular cavity is obliterated, leading
to a progressively restrictive physiology.96,97 Evidence
of the severest form carries a very poor prognosis,
with an estimated survival of 2 years after diagnosis
with progressive heart failure being the predominant
presentation (Fig. 66.36).

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

Fig. 66.37: Sarcoidosis. Two-dimensional transthoracic echocardiography. Parasternal long-axis view. Both the ventricular
septum (VS) and posterior wall (PW) are echogenic, consistent
with myocardial fibrosis. (Ao: Aorta; LA: Left atrium; MV: Mitral
valve; RV: Right ventricle) (Movie clip 66.37).

OTHER INFILTRATIVE
CARDIOMYOPATHIES
Sarcoidosis
Sarcoidosis is a systemic disease characterized by the
formation of noncaseating granulomas that can infiltrate
the myocardium. Cardiac involvement only occurs in
approximately 5% of patients with systemic sarcoidosis.
Myocardial granulomas with central areas displaying
low signal intensity characteristic of fibrosis and a high
peripheral signal intensity corresponding to edema are
typically seen on cardiac MRI.98
Patients with cardiac sarcoidosis may manifest with a
variety of clinical scenarios varying from cardiomyopathy
and heart failure to conduction system abnormalities and
ventricular tachyarrhythmias.
While contemporary diagnosis often entails the use
of cardiac MRI, traditional echocardiographic findings
are useful in identifying cardiac sarcoidosis. The usual
echocardiographic appearance is that of a DCM. The
ventricle may be globally hypokinetic or the patchy nature
of sarcoid infiltration of the heart may result in regional
wall motion abnormalities. With edema or infiltration,
mild wall thickening may also be present. This is noted
on echocardiography by the presence of bright shadows
consistent with infiltration.
Typically with progression, areas of wall thinning
are seen, most commonly in the ventricular septum,

1405

associated with scarring. Specifically, the thinning of the

basal anterior septum, while relatively uncommon, is very
characteristic of cardiac sarcoidosis.99 Interestingly, the
finding of anterior septal thinning does not correlate with
conduction system abnormalities, namely varying degrees
of AV block, which are commonly seen in this condition
(Fig. 66.37; Movie clip 66.37).99
In the absence of LV thinning, ventricular wall
thickness is usually preserved. In fact, Matsumori et al.
have described a presentation similar to HCM in some
patients with sarcoidosis.100 This is, however, a relatively
rare finding.
A nonspecific, but commonly found, feature on
echocardiography is diastolic dysfunction. This may
be found early on with initial interstitial inflammation,
when systolic function may still be normal.101 Valvular
involvement is rare and might be seen as sequelae of DCM
when present.

Hemochromatosis
Hemochromatosis represents an iron overload disorder
characterized by the accumulation of iron within various
cells, including cardiac myocytes. Cardiac manifestations
of hemochromatosis are characterized by systolic
dysfunction, and cardiac MRI can detect and quantify
myocardial iron infiltration using T2 weighted imaging.
Liver biopsy is the definitive test for iron overload.102 Serum
transferrin saturation is typically > 45% and elevated serum
ferritin levels are seen, which help confirm the diagnosis
of hemochromatosis.
Although cardiac MRI is a superior imaging modality
for the diagnosis of cardiac hemochromatosis, TTE is
useful for following disease response to chelation therapy
and/or phlebotomy. The echocardiographic features
of hemochromatosis include mild LV dilatation, LV
systolic dysfunction, normal wall thickness, and biatrial
enlargement.103 The degree of iron deposition in the
myocardium correlates with the degree of LV dysfunction.

INFECTIOUS AND METABOLIC

CARDIOMYOPATHIES
Infectious Cardiomyopathy
Septic Cardiomyopathy
Acute and reversible cardiac dysfunction commonly
occurs in patients with septic shock. In the absence of
other causes of cardiomyopathy, there are two main

1406

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

factors that contribute to cardiac depression in the setting

of sepsis. Decreased RV function, presumably related
to both acute pulmonary hypertension from acute lung
injury and reduced RV contractility, act to decrease the
LV filling pressures, and hence decrease cardiac output.
In addition, a direct depression in LV contractility due
to circulating cytokines also decreases cardiac output
in sepsis. Exposure of myocardial cells to inflammatory
cytokines, mainly circulating tumor necrosis factor alpha
(TNF-) and interleukin-1B (IL-1B), have a direct negative
inotropic effect on cardiac myocytes mainly through
increases in intracellular cGMP and nitric oxide (NO).
Mitochondrial dysfunction and decreased myofilament
response to Ca2+ secondary to troponin I phosphorylation
have also been implicated.104

Figs 66.38A to C: Septicemic myocarditis. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical fourchamber (B) views. This patient with streptococcal pneumonia developed poor biventricular function consistent with myocarditis; (C) Apical
four-chamber view. Further deterioration of ventricular function was
noted on the next day and the patient succumbed in the next few days
from multiorgan failure. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
PW: Posterior wall; RA: Right atrium; RV: Right ventricle; VS: Ventricular septum) (Movie clips 66.38A to C).

Echocardiographic Features of Septic

Cardiomyopathy
Unlike cardiogenic shock, which typically manifests
as globally reduced LV function with pronounced LV
dilatation and a restrictive pattern of LV inflow (pulsed
Doppler of the mitral valve showing high E-wave velocities
and low A-wave velocities suggestive of high LV filling
pressures), septic cardiomyopathy manifests as global LV
dysfunction with minimal LV dilatation and mitral inflow
velocities suggesting near-normal LV filling pressures.
There is often pronounced yet reversible RV dysfunction
and RV dilatation (Figs 66.38A to C; Movie clips 66.38A
to C).104

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

Septic Cardiomyopathy

Cardiogenic Shock

LV function

Globally reduced

Often globally
reduced

LV dilatation

Mild

Severe

Mitral inflow
velocities

Mostly normal

Restrictive pattern
high E-wave velocities, low A-wave
velocities

RV
involvement

Often decreased
RV systolic function
and RV dilatation

Less common

Reversibility

Yes (days)

Depends on etiology

1407

of complex carbohydrate metabolism, storage disorders,

neuromuscular diseases, organic acidemias, and other
diseases such as congenital disorders of glycosylation
(CDG) and disorders of metal and pigment metabolism
(Wilson disease, hemochromatosis, DubinJohnson
syndrome).109 Children and young adults who develop
cardiomyopathy should be evaluated for underlying
inherited metabolic disorders if no obvious other sources
of cardiomyopathy are found.

Echocardiographic Features of Metabolic

Cardiomyopathies

The degree of global dysfunction in septic cardiomyopathy may be masked by severe peripheral vasodilatation causing significantly reduced afterload, thereby
appearing as if cardiac output is not significantly reduced.
However, once patients receive restoration of normal
afterload with fluid resuscitation and/or vasopressor
support, the degree of sepsis-induced LV dysfunction
is often unmasked. Once the diagnosis of septic
cardiomyopathy is made, serial bedside transthoracic
echocardiograms may be obtained to assess the degree of
LV and RV dysfunction in response to treatment.

Cardiomyopathies in pediatric patients with inborn

errors in metabolism can manifest as hypertrophic or
dilated ventricles with globally reduced systolic function.
Restrictive patterns are less common in this patient
population. However, patients with cardiomyopathies due
to disorders of metal metabolism often have a restrictive
pattern. The typical features of RCM, namely biatrial
enlargement, severe diastolic dysfunction of the LV, and
consequent right ventricular hypertrophy have been
discussed previously.

HIV-Associated Cardiomyopathy

Diabetic cardiomyopathy is diagnosed when ventricular

dysfunction develops in diabetic patients, in the absence
of coronary atherosclerosis and hypertension.110,111
There has been a reported increased risk of heart
failure in diabetic patients after matching them for age,
blood pressure, weight, cholesterol, and CAD.112 There
has also been a significant association between diabetes
and diastolic dysfunction leading to CHF with preserved
systolic function.113

Cardiac abnormalities can be found in up to 44% of

patients with HIV.105 HIV is associated with LV systolic
dysfunction in addition to DCM, although additional
cardiac abnormalities may also be present. Specifically,
pericardial effusion, pulmonary arterial hypertension,
infective endocarditis, and intracardiac masses due to
lymphoma and Kaposi sarcoma have all been described in
HIV patients. DCM and LV dysfunction are often associated
with myocarditis with various viral (including HIV), fungal,
and atypical mycobacterial organisms. Toxoplasma gondii
has also been implicated.106-108 In addition, ischemic heart
disease and the development of LV diastolic abnormalities
are associated with highly active antiretroviral therapy
(HAART).106

Metabolic Cardiomyopathy
Metabolic cardiomyopathies encompass a wide range
of inherited metabolic disorders and a wide spectrum
of other pathological conditions. Inherited metabolic
disorders often present in childhood and include defects
in mitochondrial long-chain fatty acid oxidation, carnitine
deficiency disorders, respiratory chain defects, disorders

Diabetic Cardiomyopathy

Two-Dimensional Echo
Echocardiography can be used to ascertain the systolic
and diastolic function. Diastolic dysfunction is depicted
by reduced early peak mitral inflow velocities at annular
septal and lateral levels in early diastole, as noted on
Doppler echo.
Increased myocardial reflectivity in patients with
diabetes-related heart disease has also been reported
(Figs 39A to C).114

CARCINOID HEART DISEASE

The carcinoid syndrome is a constellation of signs and
symptoms that are seen with neuroendocrine tumors,

1408

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

C
arising from the enterochromaffin cells most often in the
gastrointestinal tract. These tumors are malignant with
an incidence of around 1.22.1 per 100,000 of the general
population.115 They are characterized by the production
of vasoactive substances such as serotonin, 5-hydroxytryptamine, bradykinin, tachykinin, and prostaglandins,
which result in the typical clinical features of flushing,
diarrhea, bronchospasm, and hypotension.115,116
Carcinoid heart disease is seen in > 50% of patients with
carcinoid syndrome.117 In approximately 20% of patients,
carcinoid heart disease is the primary presentation of
metastatic carcinoid disease. Historically, the association
between carcinoid tumors and specific cardiac disease
was described in the 1950s.118 The echocardiographic
features of carcinoid heart disease were well described in
the 1980s and used reliably in its diagnosis.119,120
Cardiac involvement is predominantly right-sided as
the lungs filter the tumor products before they reach the
left atrium. It is characterized by plaque-like deposits of
fibrous tissue, typically on the endocardium of the right-

Figs 66.39A to C: Diabetic cardiomyopathy. Doppler mitral inflow

and Doppler tissue imaging in a 79-year-old female patient. (A)
Peak early mitral inflow diastolic velocity (E) measured 86.2 cm/s
in this patient; (B and C) Peak mitral annular septal (Es) and
lateral (El) velocities in early diastole measured 4.07 cm/s (B) and
4.47 cm/s (C), respectively. The ratio of E to Es and E to El
calculates to be 21.17 (normal < 15) and 19.28 (normal < 12),
respectively. The ratio of E to average of Es and El is 20.18 (normal
< 13). These findings are indicative of left ventricle (LV) diastolic
dysfunction with increased left-sided filling pressures.

sided valvular cusps and leaflets as well as the RA and

RV.121 The involvement of the tricuspid valve typically
results in hemodynamically significant regurgitation and,
less frequently, in valvular stenosis. The pulmonary valve,
which is also commonly affected, develops a combination
of stenosis and regurgitation. Hemodynamically relevant
pulmonic valve stenosis is more frequently noted than
tricuspid stenosis. This is because, the orifice of the
pulmonic valve is much smaller and consequently,
plaque deposition on the pulmonary valve and within the
pulmonic annulus and sinuses results in narrowing of the
pulmonic root. Left-sided involvement occurs in < 10%
and is characterized by valve thickening and regurgitation
without concurrent stenosis.122 Hepatic metastases are
often associated with carcinoid heart disease. Tumor
growth within the liver allows large quantities of humoral
tumor products to reach the RV without being inactivated
by the first-pass metabolism in the liver, which is thought
to be responsible for cardiac plaque formation.123,124

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

If carcinoid valvular heart disease involves the

mitral or aortic valve, a right-to-left shunt or a primary
bronchial carcinoid is frequently found. Alternatively,
left-sided valve disease may occur in the absence of these
conditions if the carcinoid syndrome is severe and poorly
controlled.125 A recent multimodality imaging study of
52 patients with carcinoid heart disease showed the
presence of a patent foramen ovale in 13 of 15 (87%)
patients with left-sided carcinoid involvement further
giving credence to the theory of right-to-left shunts being
responsible for left heart involvement.126

Echocardiographic Features
On 2D echocardiography, the tricuspid valve leaflets are
typically thickened and shortened. The leaflets become
increasingly retracted with progression of the disease,
resulting in reduced mobility. The septal and the anterior
leaflets are usually predominantly affected, whereas the
posterior leaflet may exhibit relatively preserved mobility.
Severe tricuspid regurgitation results in advanced stages
of tricuspid valve disease as the leaflets become fixed
in a semi-open position. There is also some degree of
concomitant stenosis due to a fixed orifice. Color flow
Doppler assessment of the hepatic veins may show
systolic flow reversal, consistent with severe tricuspid
regurgitation. On continuous wave Doppler tracings,
severe tricuspid regurgitation is characterized by a daggershaped profile with an early peak velocity and a rapid
decline, indicating rapid pressure equalization between
the right-sided cardiac chambers. The peak regurgitant
velocity may be increased due to coexistent pulmonic
stenosis. The presence of a prolonged pressure half-time
of the tricuspid inflow indicates associated tricuspid valve
stenosis. Increases in tricuspid inflow velocities and the
mean gradient across the tricuspid valve result from a
combination of valvular stenosis and increased blood flow
through the valve owing to the regurgitant volume.127
The pulmonary valve cusps appear thickened with
retraction and reduced mobility. The cusps may be difficult
to visualize if they are severely retracted. Constriction of
the pulmonary annulus as a result of plaque deposition
may also be observed. Doppler echocardiographic
assessment of the pulmonary valve is particularly helpful
because demonstration of the anatomical changes may be
challenging. Increased systolic velocities on continuous
wave Doppler examination are consistent with pulmonary
stenosis, whereas a dense regurgitant spectral profile with
a short deceleration time is typical for severe regurgitation.

1409

Chronic tricuspid and pulmonic valve regurgitation

results in progressive volume overload of the right-sided
chambers. The hemodynamic situation can be further
compromised by pressure overload due to pulmonic
stenosis. As a consequence, the RA and ventricle become
increasingly dilated. Furthermore, hypokinesis of the RV
may be apparent in advanced stages of the disease. Consequently, patients present with right ventricular failure and
signs of volume overload characterized by pedal edema,
pulsatile liver, and effort intolerance. While isolated left
ventricular dysfunction is not seen per se, the presence
of right ventricular failure results in a low systemic output
state with diminution of the cardiac output.
Left-sided valvular involvement is infrequent. It is
characterized by diffuse valve thickening and retraction
with reduced mobility and regurgitation, but without
significant stenosis (Figs 66.40A to C, Movies 66.40A to C).
Dumaswala et al have elegantly illustrated the
incremental value of 3D echocardiography in carcinoid
heart disease.128 Three-dimensional echocardiography
can allow for better assessment of (a) valvular structure,
(b) severity of valvular disease, (c) extent of cardiac
involvement, and (d) metastatic lesions. It is particularly
useful as it permits evaluation of all three leaflets of the
tricuspid and pulmonary valves. This is not so with 2D
echocardiography wherein only the anterior and septal
leaflets of the tricuspid valve and the anterior (left anterior)
and left (posterior) leaflets of the pulmonary valve can be
visualized. Estimation of the effective regurgitant orifice
area is made using the proximal isovelocity surface area
(PISA) method by 2D transthoracic echocardiography
(2D TTE), which depends on the assumption that flow
convergence is hemispheric, although this does not
hold true at most times.129 The vena contracta method,
which is also used to find the area of regurgitation by
2D TTE, depends on the assumption that the area of
regurgitation is circular or elliptical, which is also not
the case in most circumstances. The measurement of the
vena contracta is also more accurate with 3D imaging
since it permits visualization of the entire regurgitant jet.
Thus, the quantification of valvular regurgitation,
particularly tricuspid and pulmonary, which are more
common in carcinoid heart disease, is more reliable with
3D echocardiography.
Further, the visualization of endocardial deposits is
superior with 3D echocardiography as is the evaluation
of hepatic metastasis in the subcostal views. These help
improve the diagnostic accuracy while also identifying
the anatomical extent of endocardial deposits (Figs 66.41A
to K; Movie clips 66.41A to F).

1410

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

The pulmonary valve appeared thickened, retracted,

and immobile in 36 patients (49%) and could not be
visualized in an additional 29 patients (39%). Pulmonic
stenosis was identified in 25 patients (53%) by Doppler
echocardiography.

Enlargement of the RA and the RV was seen in

67 (91%) and 65 (88%) patients, respectively. Four patients
(5%) exhibited impaired right ventricular systolic function.
Left-sided valve lesions were only found in five patients
(7%). Presence of myocardial carcinoid metastases was

Figs 66.40A to C: Carcinoid disease. Two-dimensional transthoracic echocardiography. (A) Apical four-chamber view shows
systolic noncoaptation of thickened anterior (1) and septal
(2) leaflets of tricuspid valve (TV); (B) Color Doppler examination
shows severe tricuspid regurgitation (arrowhead) resulting from
systolic noncoaptation of TV leaflets. Tricuspid regurgitation (TR)
jet practically fills the right atrium (RA); (C) Aortic (Ao) short-axis
view shows severe pulmonary regurgitation (PR) with the PR jet
extending all the way to the TV level. (LA: Left atrium; LV: Left
ventricle; PA: Pulmonary artery; RV: Right ventricle) (Movie clips
66.40A to C).
Source: Reproduced with permission from Ref. 128. 66.40A
to C).

Figs 66.41A and B

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

Figs 66.41C to H

1411

1412

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

K
Figs 66.41A to K: Carcinoid disease. Live/real time three-dimensional transthoracic echocardiography. (A) 1, 2, and 3 represent
thickened anterior, septal, and inferior (posterior) leaflets of the tricuspid valve (TV), respectively, showing a very large area of
noncoaptation in systole (Movie clip 66.41A, part 1). Movie clip 66.41A part 2 shows QLAB images. Movie clip 66.41A part 3 shows color
Doppler assessment of TV regurgitation (TR). The vena contracta (VC; arrowhead) is very large, measuring 2.51 cm2, consistent with
torrential TR; (B) 1, 2, and 3 represent thickened anterior (left anterior), left (posterior), and right (right anterior) leaflets of the pulmonary
valve (PV), respectively, (Movie clip 66.41B part 1). In Movie clip 66.41B part 2 the arrowhead points to noncoaptation of the PV leaflets
in diastole. Movie clip 66.41B part 3 shows color Doppler assessment of pulmonary regurgitation (PR). The VC (arrowhead) is large,
measuring 0.7 cm2, consistent with severe PR; (C to E) Arrowhead shows a prominent, localized echogenic carcinoid deposit involving
the interatrial septum (IAS); (F) The arrowhead demonstrates a carcinoid deposit lining the wall of the inferior vena cava (IVC); (G and H)
QLAB. Upper arrowheads point to the carcinoid deposit involving the right atrium (RA) superior wall. Lower arrowhead views the same
plaque en-face. It measured 2.02 0.85 cm, area = 1.36 cm2; (I to K) Subcostal examination; (I) A large carcinoid metastasis, bounded
by dots, is noted in the liver (L). QLAB sections taken at two different levels show the extent of hemorrhage/necrosis (arrowheads) in
the lesion; (J) Another liver metastasis (M, dots) is seen encroaching the RA and IVC; (K) Examination of another patient with known
simple L cysts. Arrowheads point to some of the cysts, which are generally completely echolucent with thin, well-defined borders. These
features distinguish them from a carcinoid lesion, where the echolucencies do not involve the whole cyst and the borders are less well
defined and are thickened. (Ao: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; PA: Pulmonary artery; RV: Right ventricle)
(Movie clips 66.41A parts 1 to 3, 66.41B parts 1 to 3, 66.41C to F, 66.41I parts 1 and 2, 66.41J and 66.41K).

Pellikka et al. have described the spectrum of the

echocardiographic changes and the frequency of their
detection in an elegant series of 74 patients with carcinoid
heart disease treated at the Mayo Clinic.122 Tricuspid

regurgitation was the predominant finding present in

72 patients (97%) by 2D criteria and all 69 patients who
underwent Doppler examination. In 62 patients (90%),
the degree of regurgitation was moderate or severe.

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

demonstrated in three patients (4%) and small pericardial

effusions without hemodynamic significance were seen in
10 patients (14%).
Thus, carcinoid heart disease can have varied
features that are well defined and diagnosed through
echocardiography. While left-sided involvement is rare,
the presence of metastatic masses in the LV and mitral
valve disease are concerning features that warrant
aggressive therapy. Further, cardiac output is affected due
to right ventricular dysfunction rather than left ventricular
involvement.

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pattern and left ventricular diameter predict response and
prognosis after cardiac resynchronisation therapy. Heart.
2008;94(8):102631.

1414

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

20. Dujardin KS, Enriquez-Sarano M, Rossi A, et al.

Echocardiographic assessment of left ventricular
remodeling: are left ventricular diameters suitable tools?
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22. Bax JJ, Schinkel AF, Boersma E, et al. Extensive left
ventricular remodeling does not allow viable myocardium
to improve in left ventricular ejection fraction after
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23. Burkett EL, Hershberger RE. Clinical and genetic issues
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24. Codd MB, Sugrue DD, Gersh BJ, et al. Epidemiology of
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25. Mielniczuk LM, Williams K, Davis DR, et al. Frequency of
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27. Hibbard JU, Lindheimer M, Lang RM. A modified definition
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36. Boi I, Fabijani D, Carevi V, et al. Echocardiography in

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Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

53. Marcus FI, Fontaine GH, Guiraudon G, et al. Right

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61. Prakasa KR, Dalal D, Wang J, et al. Feasibility and variability
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62. Lipshultz SE, Alvarez JA, Scully RE. Anthracycline
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63. Grenier MA, Lipshultz SE. Epidemiology of anthracycline
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1415

67. Piano MR. Alcoholic cardiomyopathy: incidence, clinical

characteristics, and pathophysiology. Chest. 2002;121(5):
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68. Lazarevi, AM, Nakatani, S, Nekovi, AN, et al. Early
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69. Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. 1997;336(4):26776.
70. Armstrong WF, Ryan T. Feigenbaums Echocardiography.
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71. Sharma N, Howlett J. Current state of cardiac amyloidosis.
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72. Siqueira-Filho AG, Cunha CL, Tajik AJ, et al. M-mode and
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73. Klein AL, Hatle LK, Burstow DJ, et al. Comprehensive
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74. Feng D, Syed IS, Martinez M, et al. Intracardiac thrombosis
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76. Tanaka H, Adachi K, Yamashita Y, et al. Four cases of
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77. von Scheidt W, Eng CM, Fitzmaurice TF, et al. An atypical
variant of Fabrys disease with manifestations confined to
the myocardium. N Engl J Med. 1991;324(6):3959.
78. Whybra C, Kampmann C, Willers I, et al. AndersonFabry disease: clinical manifestations of disease in female
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79. MacDermot KD, Holmes A, Miners AH. Anderson-Fabry
disease: clinical manifestations and impact of disease
in a cohort of 98 hemizygous males. J Med Genet. 2001;
38(11):75060.
80. Linhart A, Palecek T, Bultas J, et al. New insights in cardiac
structural changes in patients with Fabrys disease. Am
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81. Weidemann F, Niemann M, Breunig F, et al. Longterm effects of enzyme replacement therapy on fabry
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treatment. Circulation. 2009;119(4):5249.
82. Weidemann F, Breunig F, Beer M, et al. Improvement of
cardiac function during enzyme replacement therapy
in patients with Fabry disease: a prospective strain rate
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83. Saccheri MC, Cianciulli TF, Lax JA, et al.; AADELFA. TwoDimensional Speckle Tracking Echocardiography for Early
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84. Gotlib J. World Health Organization-defined eosinophilic
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management. Am J Hematol. 2012;87(9):90314.

1416

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

85. Ogbogu PU, Bochner BS, Butterfield JH, et al. Hypereosinophilic syndrome: a multicenter, retrospective
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86. Fauci AS, Harley JB, Roberts WC, et al. NIH conference.
The idiopathic hypereosinophilic syndrome. Clinical,
pathophysiologic, and therapeutic considerations. Ann
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87. Weller PF, Bubley GJ. The idiopathic hypereosinophilic
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88. Loeffler W. Endocarditis parietalis fibroplastica mit
Bluteosinophilic. Schweiz Me Wochenschr. 1936;66:817.
89. Tanino M, Kitamura K, Ohta G, et al. Hypereosinophilic
syndrome with extensive myocardial involvement and
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90. Ommen SR, Seward JB, Tajik AJ. Clinical and echocardiographic features of hypereosinophilic syndromes.
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91. Feigenbaum H, Armstrong WF, Ryan T. Feigenbaums
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92. Shah R, Ananthasubramaniam K. Evaluation of cardiac involvement in hypereosinophilic syndrome:
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93. Yacoub S, Kotit S, Mocumbi AO, et al. Neglected diseases
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94. Rashwan MA, Ayman M, Ashour S, et al. Endomyocardial
fibrosis in Egypt: an illustrated review. Br Heart J.
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95. Mocumbi AO, Ferreira MB, Sidi D, et al. A population study
of endomyocardial fibrosis in a rural area of Mozambique.
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96. Acquatella H, Schiller NB. Echocardiographic recognition
of Chagas disease and endomyocardial fibrosis. J Am Soc
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97. Acquatella H, Schiller NB, Puigb JJ, et al. Value of twodimensional echocardiography in endomyocardial disease
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98. Vignaux O. Cardiac sarcoidosis: spectrum of MRI features.
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100. Matsumori A, Hara M, Nagai S, et al. Hypertrophic
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102. Seward JB, Casaclang-Verzosa G. Infiltrative cardiovascular

diseases: cardiomyopathies that look alike. J Am Coll
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103. Ptaszek LM, Price ET, Hu MY, et al. Early diagnosis
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104. Vieillard-Baron A. Septic cardiomyopathy. Ann Intensive
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105. Blanchard DG, Hagenhoff C, Chow LC, et al. Reversibility of
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106. Velasquez EM, Glancy DL, Helmcke F, et al. Echocardiographic findings in HIV Disease and AIDS. Echocardiography. 2005;22(10):8616.
107. Rerkpattanapipat P, Wongpraparut N, Jacobs LE, et al.
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108. dAmati G, di Gioia CR, Gallo P. Pathological findings of
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109. Gilbert-Barness E. Review: Metabolic cardiomyopathy and
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110. Francis GS. Diabetic cardiomyopathy: fact or fiction? Heart.
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111. Picano E. Diabetic cardiomyopathy. the importance of
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112. Kannel WB, Hjortland M, Castelli WP. Role of diabetes
in congestive heart failure: the Framingham study. Am J
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113. Kitzman DW, Gardin JM, Gottdiener JS, et al. Cardiovascular
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114. Fang ZY, Yuda S, Anderson V, et al. Echocardiographic
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115. Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid
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116. Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med.
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117. Lundin L, Norheim I, Landelius J, et al. Carcinoid heart
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118. Thorson A, Biorck G, Bjorkman G, et al. Malignant
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syndrome. Am Heart J. 1954;47(5):795817.

Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies

119. Callahan JA, Wroblewski EM, Reeder GS, et al.

Echocardiographic features of carcinoid heart disease. Am
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120. Howard RJ, Drobac M, Rider WD, et al. Carcinoid heart
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125. Connolly HM, Schaff HV, Mullany CJ, et al. Surgical

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128. Dumaswala B, Bicer EI, Dumaswala K, et al. Live/Real
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129. Khanna D, Miller AP, Nanda NC, et al. Transthoracic
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22(9):74869.

CHAPTER 67
Echocardiographic Differentiation
of Ischemic and Nonischemic
Cardiomyopathy: Comparison with
Other Noninvasive Modalities
Sula Mazimba, Arshad Kamel, Navin C Nanda, Maximiliano German Amado Escanuela, Kunal Bhagatwala, Nidhi M Karia

Snapshot

Echocardiographic Assessment of Ischemic and

Echocardiographic Distinction between Ischemic

Nonischemic Cardiomyopathy

M-Mode Echocardiography

Two-Dimensional/Three-Dimensional/Doppler
Echocardiography

Cardiomyopathy and Nonischemic Dilated

Cardiomyopathy

Other Noninvasive Imaging Modalities

INTRODUCTION
The left ventricle (LV) may be enlarged and show
dysfunction in both ischemic heart disease and dilated
cardiomyopathy. It is therefore important to distinguish
ischemic cardiomyopathy (ICM) from nonischemic dilated
cardiomyopathy (NICM) as management may be different.
The distinction between the two types of conditions may
have therapeutic and prognostic implications. Patients
with ICM may benefit from a revascularization treatment
strategy.13 Furthermore, patients with ICM have worse
prognosis than NICM patients.4 In clinical practice,
distinguishing between the two types of conditions can
be very challenging. In some situations, a diagnosis
can be inferred from history and physical examination
(e.g. postpartum or chemotherapy-induced NICM). In
general, distinction of the two cardiomyopathies lies in

the identification of significant coronary artery disease

(CAD) as the primary mechanism for the LV dysfunction
(< 45%). Definition of ICM requires the identification of
significant CAD in the presence of depressed LV ejection
fraction (EF). Significant CAD for the ICM has been
proposed as stenosis of any of the epicardial vessels
> 75%, or a history of myocardial infarction or previous
revascularization of the coronaries.5 Coronary angiography
remains the gold standard method of evaluating CAD.59
Nonetheless, coronary angiography is not without risks.
It is invasive, operator-dependent, and may be associated
with adverse events.9,10 A noninvasive imaging modality,
particularly in patients with low to intermediate pretest
probability for CAD is often recommended. Coronary
angiography is reserved for patients with a high pretest
probability for CAD, who may also benefit from revascul
arization.9

Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy

1419

Fig. 67.1: Parasternal long-axis view showing a severely

dilated left ventricular cavity. There is malcoaptation of the anterior and posterior mitral valve leaflets. (AO: Ascending aorta; DA:
Descending aorta; LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).

Fig. 67.2: Nonischemic cardiomyopathy. M-mode echocardiography shows a large mitralseptal separation, E-point septal
separation (EPSS) of 45 mm is noted on the M-mode. (AO: Aorta;
LA: Left atrium; LV: Left ventricle).

ECHOCARDIOGRAPHIC ASSESSMENT
OF ISCHEMIC AND NONISCHEMIC
CARDIOMYOPATHY

LV systolic function is strongly related to functional status

and prognosis in patients with cardio
myopathy.22 The
predictive impact of LVEF on mortality is much more
pronounced in ICM than NICM.23
Doppler measures of contractility are reduced in both
ICM and NICM. The left ventricular outflow tract flow
velocity or velocity time integral is usually decreased to
<18 cm. Another measure of ventricular contractility is
the dP/dt (i.e. the change in left ventricular pressure over
time). This is measured from the mitral regurgitant jet.
A value of <600 indicates significant impairment of the
LV contractility. A low dP/dt is associated with adverse
cardiovascular outcomes and mortality.24,25 The myo
cardial performance index (MPI), or the Tei index is
another Doppler-derived measure of systolic and diastolic
ventricular function.26 A calculated MPI value of > 0.60 is
correlated with adverse outcomes in both patients with
nonischemic and ischemic cardiomyopathies.2729

Two-dimensional (2D) echocardiography is a low-cost and

widely available diagnostic tool used in the initial evaluation
of patients with left ventricular systolic dysfunction.11 Left
ventricular dilatation has been implicated in the initial
development and progression of systolic dysfunction with
or without CAD (Figs 67.1 to 67.3. Also Figs 66.1C and 66.2B
in Chapter 66)1214 it is thought that ventricular dilatation
is the initial adaptive response of a failing heart.15,16 As
the LV chamber further enlarges, a remodeling process
that is regulated by mechanical, neurohormonal, and
genetic factors ensue.12,1719 The end result is a change in
the geometric configuration of the heart muscle from an
ellipsoid to a spherical shape.20 A spherically shaped heart
has ineffectual muscle contractions, larger ventricular
volumes, and conformational changes in the mitral
annular apparatus. NICM on echo is characterized by an
increase in ventricular chamber sizes with reduced indices
of systolic function (left ventricular ejection fraction
[LVEF] < 45%, or fractional shortening < 25% and reduced
fractional area change). The enlarged LV chambers occur
in the setting of normal or reduced LV thickness.21
Systolic function is often reduced in dilated cardio
myopathy (Fig. 67.1). The degree of reduction of the LVEF
is well correlated to the severity of the cardiomyopathy.4

M-MODE ECHOCARDIOGRAPHY
The sphericity index (SI) is a surrogate marker of LV
remodeling. It is the ratio of left ventricular long-axis
internal dimension to the LV diameter at end-systole.30,31
A SI of <1.5 underscores a very severely dilated LV cavity.
Another M-mode parameter, which is related to LV cavity
dilatation and reduced LV systolic excursion, is the E-point
septal separation (EPSS). This is measured as the distance
from the tip of the open anterior mitral leaflet in diastole

1420

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 67.3A to F

Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy

1421

Figs 67.3A to G: Ischemic cardiomyopathy in a 38-year-old female

patient. M-mode/two-dimensional transthoracic echocardiography.
(A and B) Parasternal long-axis views demonstrating a huge left
ventricle (LV) measuring 90 mm and severe mitral regurgitation
(arrow); (C and D) M-mode examination showing a very large
LV with severely reduced function. Also note markedly increased
E-point septal separation (EPSS) measuring 32 mm in D; (E to G)
Apical views; (E) The coaptation point of the mitral leaflets is displaced into the LV and directed eccentrically toward the LV free wall,
suggesting ischemic origin of cardiomyopathy; (F) Shows dyskinesis (arrow) of the distal LV inferior wall and septum; (G) Severe
mitral regurgitation (arrow) resulting from reduced coaptation of the
displaced mitral leaflets. Movie clip H shows hypokinesis (arrow) of
the distal right ventricular (RV) free wall. (AO: Aorta; LA: Left atrium;
RA: Right atrium) (Movie clips 67.3A to 67.3H).

to the ventricular septal wall. A normal value is < 6 mm.32

EPSS of >10 mm reflects severe LV dilatation and systolic
dysfunction (Fig. 67.2).3234 The utility of EPSS is limited in
aortic regurgitation and mitral stenosis. The aortic valve
may demonstrate reduced leaflet excursions coupled
with early closure. Both these features are a reflection
of a diminished cardiac output. Another feature on
M-mode that signifies low cardiac output is the reduced
wall motion of the aortic root. One of the advantages
of M-Mode echo is its high temporal resolution (1000
3000 Hz compared to 20129 Hz with 2D echo). Twodimensional echocardiography, on the other hand, has
a higher spatial resolution compared to M-mode. Thus,
combining the two modalities can be helpful in guiding
accurate alignment of the M-mode beam for reliable
quantification of the chamber sizes.35 Severe NICM is
suggested by left ventricular end-diastolic dimension
> 56 mm in males and 53 mm in females. A dilated LV
chamber in the setting of normal or reduced LV wall
thickness (end-diastolic wall thickness of <6 mm) that is
global in nature is highly indicative of NICM. In ICM, there
may be regional variation in thickness of the myocardium.
Myocardial thickness of <6 mm at end diastole is highly
suggestive of nonviable myocardium.36

TWO-DIMENSIONAL/THREEDIMENSIONAL/DOPPLER
ECHOCARDIOGRAPHY

(DCM). Even though linearly derived measurements are

used to calculate chamber sizes, these measurements
are based on geometric assumptions of the LV cavity in
the form of a truncated ellipsoid.21 These measurements,
however, may grossly underestimate the true LV sizes
and volumes in CAD patients with distorted LV cavities.
This is because of the regional wall motion abnormalities
(WMAs) often present in CAD (presence of aneurysms,
WMAs, asymmetrical and foreshortened ventricles).
Therefore, volumetric measurements for accurate cardiac
chamber size quantification are preferred to linear
measurements. More recently, three-dimensional (3D)
echo has been shown to have an advantage over 2D echoderived measurements. Three-dimensional transthoracic
echocardiography (3D TTE) can overcome the limitation
of quantifying volumes in ICM patients with distorted
LVs. Volumetric measurements by 3D echo yield more
accurate and reproducible measurements [comparable
to radionuclide or cardiac magnetic resonance imaging
(CMR)].3740 One of the challenges of obtaining volumetric
measurements in 3D echo arises when the cardiac
chambers are foreshortened. This situation can lead
to an underestimation of LV volumes.41 Automated
endocardial tracings have been recommended as one way
of overcoming this limitation.42 In the difficult situation
with poor endocardial visualization, the use of contrast
agents can enhance the accuracy and reproducibility of
measurements.43

Left Ventricular Volumes

Left Ventricular End-Systolic

Volume Index

Correct quantification of the cardiac chambers is necessary

in assessing the severity of the dilated cardiomyopathy

End-systolic volume index (ESVI) provides important

information regarding the severity and prognosis of

1422

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

NICM.4446 ESVI increases progressively with the advan

cement of disease. It has been shown conclusively that
adverse outcomes increase with an ESVI of > 25 mL/m2
(normal value, < 20 mL/m2).45 An ESVI > 60 mL/m2 is
associated with increased adverse outcomes in patients
with ICM undergoing surgical ventricular reconstruction.47

Two-dimensional Echo/Doppler-Derived
Left Ventricular Diastolic Dysfunction
Diastolic function assessment in NICM and ICM provides
useful information regarding the severity and overall
prognosis. Patients with restrictive diastolic physiology
on two-dimensional transthoracic echocardio
graphy
48,49
(2D TTE) have increased adverse outcomes.
A short
deceleration E time is associated with severe symptoms
and is also a powerful and independent prognostic
indicator of poor outcomes in patients with NICM.22,49
51
Grade II diastolic dysfunction has also been shown
to predict adverse outcomes and increased hospital
re-admissions in patients with NICM.52

Right Ventricle
Right ventricular (RV) dysfunction is a powerful predictor
for exercise capacity as well as mortality in patients with
NICM.5355 Assessment of RV function is often challenging
because of the complexity of the 3D structure that is
less amenable to geometric assumptions like the LV. In
addition, the RV is in a substernal position, which renders
imaging more challenging. The numerous trabeculations
also renders volumetric assessment of the RV function
challenging.56,57 3D TTE, however, compares favorably
with CMR in the assessment of RV function.58 A 2D TTE
parameter that has been validated in the assessment of RV
function is the tricuspid annular plane systolic excursion
(TAPSE).59,60 Right ventricular function is measured by the
degree of excursion of the tricuspid annulus from the base
to the apex. A TAPSE of <15 mm is associated with poor
RV systolic function. Decreased RV systolic function as
estimated by TAPSE is associated with increased mortality
in patients admitted for heart failure. Among patients with
LV systolic dysfunction, the involvement of RV dilatation is
often suggestive of a NICM. The RV is often spared in ICM
unless there is accompanying right ventricular infarction
in which case the right ventricle will be affected. RV
involvement portends worse adverse outcomes in both
NICM and ICM.55,6163

Left Atrium
Left atrial (LA) size has been shown to have prognostic
value in patients with NICM. LA volume is determined by
the degree of LV dilatation, diastolic dysfunction as well
as the severity of mitral regurgitation (MR).64,65 LA size
correlates well with the severity of diastolic dysfunction,
which in turn has prognostic information for patients
with both ICM and NICM.6672 In patients with NICM with
functional MR, LA size may reflect the duration of the MR.73
The LA size was a more powerful predictive variable than
the severity of MR jet in determining adverse outcomes
in NICM patients.65 The current American Society of
Echocardiography guidelines recommends obtaining
LA volumes rather than linear dimensions, which do
not take into account the asymmetric remodeling of
the chamber.21,74,75 The upper limit of normal LA-ESVI is
28 mL/m2.21

Mitral Regurgitation: Mechanisms

Functional MR is a common feature in patients with
NICM or ICM. The presence of MR is associated with
poor prognosis even in patients with prior repair of the
mitral valve (MV).7679 Functional MR is characterized
by malcoaptation of the two mitral leaflets despite a
structurally normal MV (Figs 67.3B, 67.3G and 67.4). In
an occasional patient with ICM, the seagull sign may be
visualized as explained in Figure 67.4.80 The mechanism
related to the malcoaptation of the leaflets is thought
to be due to the remodeling of the LV cavity.8183 Factors
thought to contribute to MR include dilatation of the
mitral annulus, tethering of the leaflets (due to apical or
posterior displacement of the papillary muscles), and
reduced LV function (geometric changes occurring in a
remodeled ventricle).8494 The degree of conformational
changes that occur in the MV apparatus in functional MR
can be quantitatively assessed. These geometric variables
for MV deformation are discussed in the following section.

Tenting Area
This is the area enclosed by the annular plane and the two
mitral leaflets (Fig. 67.5). It has been shown to accurately
reflect the degree of functional mitral regurgitation
(FMR). It is also an independent predictor of mortality
and hospitalization in patients with NICM.95 Patients
with a tenting area > 3.4 cm2 had higher brain naturetic
peptide (BNP) levels, worse functional status, more
hospitalizations, and higher death rates.

Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy

1423

Fig. 67.5: The shaded region illustrates the tenting area of the
mitral valve. (H: Tenting height; LA: Left atrium; LV: Left ventricle;
MV: Mitral valve).
Fig. 67.4: Transesophageal apical four-chamber view showing
severe mitral regurgitation by color Doppler flow imaging
(arrow). Arrowhead shows a kink in the middle of the anterior leaflet
mimicking a seagull. This seagull sign results from tethering
produced by a strut chord and is considered indicative of ischemic origin of cardiomyopathy. (LA: Left atrium; LV: Left ventricle;
RV: Right ventricle) (Movie clip 67.4).

Fig. 67.6: A section of mitral valve from apical four-chamber view.

1 = anterior leaflet angle (AL) which measured 21. 2 = posterior
leaflet angle (PL) which measured 62 (LA: Left atrium; LV: Left
ventricle).

Coaptation depth, otherwise known as MV tenting

height, is the shortest distance between the leaflet coap
tation point and the mitral annular plane (Fig. 67.5).96
The degree of leaflet tethering is measured by the angle
at which each of the two mitral leaflets joins the mitral
annular plane. These are the anterior leaflet angle (ALa)
and the posterior leaflet angle (PLa; Fig. 67.6). Because
there is differential insertion of fibers on the mitral leaflets
(fine marginal chordae fibers insert at the leaflet tips and
the thicker chordae insert at the leaflet base), further
categorization of the AL angles can be made. Tethering of
the anterior MV leaflet at the base (ALa base) is measured
by obtaining the angle at AL base as it intersects with the

annular plane. On the other hand, tethering at the distal AL

tip (ALa tip) is calculated by measuring the angle between
the annular plane and a line that joins the anterior annulus
and the coaptation point of the two leaflets.94,97 Lee et al.
have proposed a classification of AL tethering into three
subtypes based on the morphology and site of maximal AL
tenting.97
Type I AL: Tethering involves minimally tethered AL
along its long-axis dimension.
Type II AL: Tethering is characterized by posteriorly
directed tethering by the basal chordae. The
morphology of AL has a very prominent bend
on 2D echo imaging.
Type III AL: Consists of a pronounced apical tethering of
both the base and the distal tip of the AL. It
is often recognized on echo by a large AL tip
angle.
It has been proposed that this classification should
help guide treatment decisions in patients with functional
MR. For instance, mitral annuloplasty is more likely to be
successful with type I and II subtypes.97
Both the PLa and ALa have been shown to offer
diagnostic and prognostic information in patients with
ischemic MR.98101 One of the distinguishing features of
ICM from NICM is in the pattern of MV deformation
(measured as a change in the AL and PL angles). The
MV deformation is geometrically asymmetrical in ICM
compared to NICM.101,102 In the NICM, the conformational
changes of the MV are the same in all the planes. It appears
that regional LV remodeling from ischemia results in
asymmetrical papillary muscle displacement, which then
leads to asymmetrical leaflet tethering, thereby affecting
the coaptation of the two mitral leaflets. Two separate
regurgitant jets often characterize ICM MR.101,103,104 These

1424

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

jets correspond to the deformational changes in the

MV leaflets. There is a funnel-shaped conformational
deformity in the medial side of the MV and a prolapsed
deformity in the lateral portion of the valve. The medial
deformity is responsible for the centrally directed jet,
while the lateral prolapsed deformity is responsible for the
eccentric jet. Interestingly, it is the asymmetrical apically
oriented displacement of the papillary muscles rather
than the asymmetrical medial lateral orientation of the
papillary muscle displacement that accounts for the MV
deformational changes seen in ICM.102
The coaptation depth and tenting area both correlate
with the severity of MR in patients with systolic dysfu
nction.93 Furthermore, these parameters have been shown
to confer prognostic information in patients undergoing
functional MV intervention. Magne et al noted that in
ICM patients undergoing functional MV annuloplasty, the
presence of a PLa of more than 45 had increased rates
of recurrent MR and other adverse events.100 There is a
growing body of evidence to suggest that the geometric
differences in the patterns of tethering seen in ICM
and NICM can help guide the choice of therapy for
functional MR.92,97,101,105

Stress Echocardiography
Stress echocardiography has emerged as a useful tool
for the evaluation of selected patients with suspected or
known ischemic heart disease.106 The high specificity of
stress echocardiography compared to other modalities
contributes to its utility as a cost-effective diagnostic tool
for CAD. The sensitivity and specificity of new WMAs
induced by dobutamine for detection of CAD is 89% and
85%, respectively. The sensitivity in those with multivessel
or left main disease is 100% compared to 81% in those
with a single vessel disease.107 Dobutamine stress echo
can help differentiate between ICM and NICM. In the
absence of CAD, the normal response to dobutamine
stress test is augmentation of systolic function and
contractility with increasing doses of dobutamine. In the
presence of significant disease, an initial augmentation
of LVEF and contractility is followed by a decline in
LVEF and/or contractility or emergence of new WMA
at higher doses of dobutamine.106,108 The segmental wall
motions are graded by dividing the heart into 16 segments
(17 segments if echo is being compared with another
imaging modality such as nuclear perfusion) and each
segment is then graded on a scale of 14 on the basis of
wall motion (1 = normal, 2 = hypokinesis, 3 = akinesis,
4 = dyskinesis).109 A cumulative score is obtained at

baseline and at end of the study. A cumulative score

of all the segments is then divided by the number of
segments. A numerical score of 1 is normal while that
above 1 is abnormal. Higher scores predict higher severity
of obstructive disease or the presence of more extensive
disease. Dobutamine stress echo can also help identify
patients with ICM that can benefit from revascularization.
Patients who have an initial augmentation of LVEF and
contractility at low dose dobutamine followed by a decline
with high dose (biphasic response) are candidates for
revascularization. A biphasic response indicates viable
myocardium that could be salvaged with revascularization.

Coronary Echocardiography
2D TTE has been studied as a means of detecting CAD
and by extension, potentially differentiating ICM from
NICM.110 2D TTE was able to detect proximal CAD in
93% of the study patients. Interestingly, visualization of
the coronaries in dilated LV was much easier in patients
with NICM than ICM. This is primarily because NICM
patients generally have much larger chamber sizes than
ICM patients and, therefore, the curvature of the coronary
arteries is less steep and easily visualized. Although the
success rate of visualizing CAD lesions using 2D TTE was
high in this study, other researchers have recorded much
lower success rates.111113

Myocardial Contrast Echocardiography

There is an emerging role of myocardial contrast echocar
diography (MCE) in assessing myocardial perfusion status
in patients suspected of having CAD.114117 MCE uses
contrast agents to improve the visualization of blood
endocardial interface. This enables the assessment
of ventricular wall motion, wall thickness, LVEF, and
qualitative and quantitative evaluation of myocardial
and coronary blood flow.43 One of the advantages of MCE
is that it not only provides microcirculatory blood flow
information, but also transmural blood flow information.
Using a vasodilator pharmacological stress test, myocardial
perfusion may be assessed both at rest and stress. With this
information, myocardial blood flow reserve is measured
as the difference between the peak flow during stress and
baseline flow at rest. Endomyocardial flow impairment has
been shown to be directly associated with the progression
of LV dysfunction. 7,118 The application of MCE in clinical
practice is still limited and remains to be standardized.

Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy

ECHOCARDIOGRAPHIC DISTINCTION
BETWEEN ISCHEMIC CARDIOMYOPATHY AND NONISCHEMIC DILATED
CARDIOMYOPATHY
There are several features on 2D TTE that help distinguish
ICM from dilated NICM (Table 67.1). Traditionally, WMAs
have been used to discriminate between NICM and ICM.
In ICM, the WMA tend to be regional and correspond to
specific coronary artery distribution.119,120 The presence
of WMA has more diagnostic value in normal sized
ventricles.121125 Medina et al.121 studied 60 patients with
dilated LV and LV dysfunction using 2D TTE for the
detection of regional WMA so as to differentiate between
ICM and NICM. They reported a sensitivity, specificity, and
predictive accuracy of 83%, 57%, and 77%, respectively, in
differentiating ICM from NICM based on the presence of
WMA. In patients with normal LV size but LV dysfunction,
the sensitivity, specificity, and predictive accuracy
were found to be 95%, 100%, and 95%, respectively, in
detecting ICM.120 Besides WMA, identification of regional
thinning of myocardial wall (< 6 mm), or aneurysmal
myocardial segment corresponding to coronary blood
flow area increases the likelihood of the diagnosis of ICM.
Aneurysms and scar on the LV surface were found in fewer
than 15% of NICM on necropsies conducted by Roberts
et al.126 Chen et al. used semiquantitative echocardiographic
segmental wall motion scoring to predict CAD. Myocardial
wall motion was scored according to the scoring described
by Heger et al.127 (i.e. hyperkinesia = 1, normal = 0, hypoki
nesia = 1, akinesia = 2, and dyskinesis = 3). Patients with a
LVEF < 50% had a mean score of 6.9, while those with LVEF
above 50% had a mean score of 1.1.122
The above criteria are, however, not entirely exhaustive
in defining ICM because WMA may also be seen in up to
two-thirds of patients with NICM.107,128130 These WMA in
patients with NICM have been attributed to abnormal
microcirculatory perfusion despite normal epicardial
blood vessels.131,132 Wallis et al. showed that up to 64% of
patient with NICM had WMAs especially when conduction
abnormalities were present (i.e. left bundle branch block
[LBBB]). When they excluded patients with LBBB, WMA
were found in 59% of the study population with NICM.128
In the same study, Wallis et al. found that WMA were more
commonly associated with older age. The younger patients
with NICM had more diffuse involvement, were more
symptomatic, and generally had a poor prognosis. Because
resting WMA is not sensitive at discriminating CAD, the

1425

situation is made worse if there is a concomitant LBBB.

Duncan et al used quantified stress echocardiography
to assess changes in the long-axis systolic amplitude to
discriminate between ICM and NICM. In the presence
of LBBB, inability to increase septal systolic amplitude
by >1.5 mm was highly indicative of CAD. This method
was better than visual assessment of wall motion score
index (WMSI; sensitivity and specificity of 94% and
100%, respectively).133 RV dilatation can be suggestive
of NICM. The right ventricle is often spared in ICM
unless, as mentioned previously, there is accompanying
RV infarction in which case one would expect to see a
hypocontractile, dilated RV.62,63 Involvement of the RV
is indicative of an advanced disease stage and is a poor
prognostic sign in either ICM or NICM.134 Because of the
complex 3D structure, quantification of the RV is rendered
difficult with 2D TTE, but can be assessed more accurately
with 3D TTE.135

OTHER NONINVASIVE
IMAGING MODALITIES
Single-Photon Emission
Computed Tomography
Myocardial perfusion imaging (MPI) using singlephoton emission computed tomography (SPECT) is
a well-validated, noninvasive imaging modality used
in the diagnosis, treatment and prognostication of
CAD.136139 SPECT MPI has high sensitivity and specificity
that approaches 90% in detecting significant CAD (with
at least 50% luminal diameter stenosis).140142 In broad
terms, SPECT MPI classifies patients in three categories
normal study (normal perfusion defects at stress and
rest), reversible ischemia (perfusion defects with stress
and normal perfusion at rest), and a fixed scar (perfusion
abnormality at rest and stress). It is known that on SPECT
MPI, NICM is characterized by homogenous tracer
uptake, whereas ICM has extensive perfusion defects
that are often regional.138,143145 Patients with reversible
perfusion defects usually benefit from revascularization
treatment strategies. The limitation of SPECT with
thallium is that patients with NICM can still present
with perfusion defects even in the absence of significant
CAD.146 The combination of perfusion abnormalities that
occur in myocardial territories consistent with areas of
WMA overcomes the problem of WMA alone in NICM
undergoing thallium imaging alone.147 Large perfusion
defects, however, are more predictive of significant ICM

1426

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Table 67.1: Comparison of Noninvasive Findings in Ischemic Cardiomyopathy and Nonischemic Dilated Cardiomyopathy

Modality

ICM with Dilated LV

NICM with Dilated LV

Clinical evaluation

Older patients

May be younger

H
 istory of ischemic heart disease such as angina
and myocardial infarction

May not have history of ischemic heart disease

S
 egmental WMA/thinning (<6 mm in end diastole)
in coronary artery distribution. Sensitivity 65% to
81%, and specificity 56% to 99%

Less common. Defects when present may not

relate to coronary artery distribution

E
 ndocardial brightening/scarring more common
due to infarction

Less common

B
 iphasic response with dobutamine stress test
indicative of hibernating viable myocardium is
more common

Less common

P
 erfusion defects with contrast echo more
common

Less common

2 D TTE/Doppler may show stenosis in proximal or

other coronary arteries, which may be small

Coronary arteries may show no stenosis, may

be larger

Spherical LV less common

Spherical LV more common

M
 ay show prominent plaques in larger vessels
such as ascending aorta, arch, and abdominal
aorta.

May be normal

A
 symmetric closure of mitral valve leaflets with
eccentric regurgitation jet(s) more common.
Seagull sign (Fig. 67.4) may be present

Symmetric closure with central jets more common. Seagull sign absent

LV dilatation less severe

Severe spherical LV dilatation with malcoaptation of mitral valve leaflets

R
 ight ventricle usually not enlarged and function
normal except when RCA is stenotic/occluded or
RV infarction present

RV enlargement and/or dysfunction more

frequent

Single-photon
emission computed
tomography (SPECT)

P
 erfusion defects in coronary artery distribution.
More accurate if combined with WMA
Normal sized RV with good function more
common

Perfusion defects less common and if present

do not relate to coronary artery distribution
Large RV with reduced function more common

Coronary computed
tomographic angiogram (CCTA)

C
 oronary artery calcification more common.Coronary stenosis, both obstructive and
nonobstructive may be identified

Less common

Cardiac magnetic
resonance (CMR)

I ncreased gadolinium uptake or enhancement indicating scar formation in the previously infarcted
areas more common
Regional WMA/thinning and perfusion defects
similar to echocardiography above

Less common

Large mismatch defects more common

Less common

Echocardiography

Positron emission
tomography (PET)

May have history of viral infections, septicemia,

alcoholism, metabolic, or infiltrative diseases

Regional WMA/thinning and perfusion defects

similar to echocardiography above

(2D TTE: Two-dimensional transthoracic echocardiography; ICM: Ischemic cardiomyopathy; LV: Left ventricle; NICM: Nonischemic
cardiomyopathy; RV: Right ventricle; WMAs: Wall motion abnormalities).

Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy

than smaller defects. When a large perfusion defect is

present, the sensitivity of SPECT to accurately identify
ICM was as high as 97%. Conversely, in the absence of a
large perfusion defect, SPECT MPI was able to accurately
discriminate NICM in 94% of the study group.144 More
recently, an approach that incorporates the severity of
the defect with the extent of WMA was shown to increase
the likelihood of detecting ICM. Defect severity ratio was
computed by measuring the ratio of the count density of
the most severe perfusion defect over the count density
of the most normal area of myocardium. A stress defect
ratio < 45% was predictive of ICM (with a sensitivity of
60% and specificity of 91%). Most patients with NICM had
higher stress defect ratio.148 Electrocardiographically gated
radionuclide ventriculography (RNV) has been shown to
have incremental value in differentiating between NICM
and ICM. Given that WMA are not confined to ICM, the
EF information provided by RNV can further serve as a
pointer to the diagnosis. NICM patients have lower LVEF,
which often involves the RV as well.149

Coronary Computed
Tomographic Angiogram
Coronary computed tomographic angiogram (CCTA)
has emerged to be an important noninvasive diagnostic
modality in the evaluation of CAD.150152 The diagnostic
utility of CCTA is in patients with low to intermediate risk
for CAD. There are several published studies that have
looked at the role of CCTA in differentiating between
NICM and ICM.153157 Two primary avenues by which CCTA
stratifies patients for the underlying CAD is the calculation
of calcium score and by anatomical definition of coronary
arteries for presence of a plaque or stenosis. Calcium score
is calculated using electron-beam computed tomography
(EBCT) and multislice computed tomography (MSCT). A
high burden of coronary calcium (calcium score of > 80)
assessed by EBCT was associated with a 99% sensitivity
and an 83% specificity in accurately identifying patients
with ICM.154 Presence of calcium is a known marker
for atherosclerosis. In another imaging modality using
ultrasound of the carotids, carotid calcification in patients
with dilated cardiomyopathy had a sensitivity of 96% and
specificity of 89% of accurately identifying ICM.158 CCTA
is more sensitive in identifying patients with ICM than
2D TTE (sensitivity of 68% and specificity of 73%).159,160
However, the sensitivity of CCTA to accurately diagnose
ICM decreases in patients with a high calcium burden
(to 73%).156

1427

Cardiac Magnetic Resonance Imaging

CMR has become an important noninvasive diagnostic
tool in the evaluation of various cardiac conditions.161 It
has become the reference, if not the gold standard, for
evaluation of cardiac chamber sizes, mass, volumetric
measurements, and EF. In clinical situations that require
serial cardiac measurements, CMR has an added
advantage of being highly reproducible with low interand intra-study variability. Other advantages of CMR
include absence of ionizing radiation and relatively
high spatial and temporal resolution.161,162 The clinical
applications of CMR continue to evolve and expand,
and now include noninvasive evaluation of the proximal
coronaries.163,164 The sensitivity and specificity of CMR
for the detection of CAD is 91% and 81%, respectively.165
CMR has been studied in the differentiation of ICM from
NICM. Furthermore, the use of gadolinium enhancement
helps with characterizing scar and other secondary
causes of NICM (i.e. myocarditis, sarcoidosis).164,166,167
In patients with ICM, CMR identifies subendocardial or
transmural enhancement indicative of scar formation in
virtually 100% of the patients.167,168 In the NICM patients,
three patterns are identified. The first pattern shows no
enhancement with gadolinium, the second subset shows
subendocardial/transmural enhancement similar to the
ICM, and the third subset shows longitudinal patchy
midwall enhancement. Enhancement with gadolinium
highlights areas of the myocardium with previous
infarction.169 The most common type of CMR pattern (58%)
is no enhancement, a finding consistent with a clinical
picture of no previous areas of infarction. The second
subtype shows enhancement similar to that of ICM (13%)
but without obstructive coronary lesions on angiography.
These findings represent myocardial infarction from
an embolic plaque or a coronary artery source that has
recanalized. The third subtype (28%) involves patchy
midwall enhancement that has been attributed to
fibrosis of the midventricular wall. The pathogenesis
of this type of fibrosis has been linked to a whole host
of potential etiologies such as genetic factors, toxins,
infections, microvascular ischemia, neurohormonal, and
immunological factors.170173 The pattern of enhancement
of the scar has prognostic information. Improvement of
contractility after revascularization is low in patients with
extensive transmural extent of the scar; for instance, if there
is >50% delayed enhancement on CMR, the likelihood of
response to revascularization was <10%.164 More recently,

1428

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Assomull et al. showed that in NICM patients with patchy

midwall fibrosis on CMR, midwall fibrosis was a significant
predictor of death or hospitalization. Midwall fibrosis also
predicted secondary outcome measures of sudden cardiac
death or ventricular tachycardia.171

Positron Emission Tomography

Positron emission tomography (PET) imaging is an ideal
noninvasive diagnostic tool for assessing myocardial
viability. The advantage of PET is that, not only does it
detail coronary artery blood flow, but it also provides
valuable information on the metabolic activity of the
myocardium.174,175 PET has the advantage of having higher
temporal and spatial resolution than SPECT imaging. PET
imaging has been shown to accurately predict ICM patients
who would benefit from surgical revascularization after
viability testing.176 The commonly used perfusion agents
are ammonium-13 and rubidium 82, while a commonly
used metabolic agent is F-18 FDG. Carbon-11 palmitate
has also been used as a marker of metabolism (a marker
of free fatty acid utilization).177 Ordinarily myocardial cells
utilize free fatty acids for energy metabolism. Ischemic
myocardial cells, on the other, hand utilize glucose instead
of free fatty acids. Thus, with PET imaging, radiolabeled
glucose (F-18 FDG) can be visualized when it is taken up
by myocardial cells. Reduced perfusion and increased
glucose uptakes indicate presence of ischemia with viable
myocardium. On the other hand, reduced uptake of
perfusion and metabolic agent indicates the presence of
nonviable myocardium or scar tissue. ICM patients show
large mismatched defects on PET imaging than NICM
patients.177 The sensitivity and specificity of PET imaging
for differentiating between ICM and NICM are 85% and
80%, respectively.174,177

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132. Chen JW, Ting CT, Chen YH, et al. Differential coronary
microvascular function in patients with left ventricular
dysfunction of unknown causeimplication for possible
mechanism of myocardial ischemia in early stage of
cardiomyopathy. Int J Cardiol. 1999;69(3):25161.

Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy

133. Duncan AM, Francis DP, Gibson DG, et al. Differentiation

of ischemic from nonischemic cardiomyopathy during
dobutamine stress by left ventricular long-axis function:
additional effect of left bundle-branch block. Circulation.
2003;108(10):121420.
134. Lewis JF, Webber JD, Sutton LL, et al. Discordance in degree
of right and left ventricular dilation in patients with dilated
cardiomyopathy: recognition and clinical implications.
J Am Coll Cardiol. 1993;21(3):64954.
135. Nanda NC, Hsiung MC, Miller AP, et al. Left ventricular
and right ventricular and function assessment, in live/real
time 3D echocardiography. 2010.
136. Lima RS, Watson DD, Goode AR, et al. Incremental value of
combined perfusion and function over perfusion alone by
gated SPECT myocardial perfusion imaging for detection
of severe three-vessel coronary artery disease. J Am Coll
Cardiol. 2003;42(1):6470.
137. Salerno M, Kramer CM. Advances in cardiovascular MRI
for diagnostics: applications in coronary artery disease
and cardiomyopathies. Expert Opin Med Diagn. 2009;3(6):
67387.
138. Schinkel AF, Elhendy A, van Domburg RT, et al. Incremental
value of exercise technetium-99m tetrofosmin myocardial
perfusion single-photon emission computed tomography
for the prediction of cardiac events. Am J Cardiol.
2003;91(4):40811.
139. Cannon RO 3rd, Cunnion RE, Parrillo JE, et al. Dynamic
limitation of coronary vasodilator reserve in patients with
dilated cardiomyopathy and chest pain. J Am Coll Cardiol.
1987;10(6):1190200.
140. Klocke FJ, Baird MG, Lorell BH, et al. ACC/AHA/ASNC
guidelines for the clinical use of cardiac radionuclide
imagingexecutive summary: a report of the American
College of Cardiology/American Heart Association Task
Force on Practice Guidelines (ACC/AHA/ASNC Comm
ittee to revise the 1995 guidelines for the clinical use of
cardiac radionuclide imaging). Circulation. Sep 16 2003;
108(11):140418.
141. Shaw LJ, Iskandrian AE. Prognostic value of gated myocardial
perfusion SPECT. J Nucl Cardiol. 2004;11(2):17185.
142. Hakeem A, Bhatti S, Dillie KS, et al. Predictive value of
myocardial perfusion single-photon emission computed
tomography and the impact of renal function on cardiac
death. Circulation. 2008;118(24):25409.
143. Eichhorn EJ, Kosinski EJ, Lewis SM, et al. Usefulness of
dipyridamole-thallium-201 perfusion scanning for disting
uishing ischemic from nonischemic cardiomyopathy. Am J
Cardiol. 1988;62(13):94551.
144. Tauberg SG, Orie JE, Bartlett BE, et al. Usefulness of

thallium-201 for distinction of ischemic from idiopathic
dilated cardiomyopathy. Am J Cardiol. 1993;71(8):67480.
145. Shiotani H, Yamabe H, Fukuzaki H. The clinical and

prognostic significance of dipyridamole Tl-201 emission
computed tomography in patients with dilated cardio
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146. Dunn RF, Uren RF, Sadick N, et al. Comparison of

thallium-201 scanning in idiopathic dilated cardiomyopathy
and severe coronary artery disease. Circulation. 1982;66(4):
80410.
147. Danias PG, Ahlberg AW, Clark BA 3rd, et al. Combined
assessment of myocardial perfusion and left ventricular
function with exercise technetium-99m sestamibi gated
single-photon emission computed tomography can
differentiate between ischemic and nonischemic dilated
cardiomyopathy. Am J Cardiol. 1998;82(10):12538.
148. Yao SS, Qureshi E, Nichols K, et al. Prospective validation
of a quantitative method for differentiating ischemic versus
nonischemic cardiomyopathy by technetium-99m sesta
mibi myocardial perfusion single-photon emission comp
uted tomography. Clin Cardiol. 2004;27(11):61520.
149. Schuijf JD, Shaw LJ, Wijns W, et al. Cardiac imaging

in coronary artery disease: differing modalities. Heart.
2005;91(8):11107.
150. Raff GL, Gallagher MJ, ONeill WW, et al. Diagnostic

accuracy of noninvasive coronary angiography using
64-slice spiral computed tomography. J Am Coll Cardiol.
2005;46(3):5527.
151. Janowitz WR, Agatston AS, Viamonte M Jr. Comparison of
serial quantitative evaluation of calcified coronary artery
plaque by ultrafast computed tomography in persons
with and without obstructive coronary artery disease. Am
J Cardiol. 1991;68(1):16.
152. Leber AW, Knez A, von Ziegler F, et al. Quantification

of obstructive and nonobstructive coronary lesions by
64-slice computed tomography: a comparative study
with quantitative coronary angiography and intravascular
ultrasound. J Am Coll Cardiol. 2005;46(1):14754.
153. Andreini D, Pontone G, Pepi M, et al. Diagnostic accuracy
of multidetector computed tomography coronary angio
graphy in patients with dilated cardiomyopathy. J Am Coll
Cardiol. 2007;49(20):204450.
154. Budoff MJ, Shavelle DM, Lamont DH, et al. Usefulness
of electron beam computed tomography scanning for
distinguishing ischemic from nonischemic cardiom
yopathy. J Am Coll Cardiol. 1998;32(5):11738.
155. Andreini D, Pontone G, Bartorelli AL, et al. Sixty-four-slice
multidetector computed tomography: an accurate imaging
modality for the evaluation of coronary arteries in dilated
cardiomyopathy of unknown etiology. Circ Cardiovasc
Imaging. 2009;2(3):199205.
156. Ghostine S, Caussin C, Habis M, et al. Non-invasive

diagnosis of ischaemic heart failure using 64-slice comp
uted tomography. Eur Heart J. 2008;29(17):213340.
157. Lima JA, Hare J. Visualizing the coronaries in patients

presenting with heart failure of unknown etiology. J Am
Coll Cardiol. 2007;49(20):20512.
158. Androulakis AE, Andrikopoulos GK, Richter DJ, et al. The
role of carotid atherosclerosis in the distinction between
ischaemic and non-ischaemic cardiomyopathy. Eur Heart
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159. Le T, Ko JY, Kim HT, Akinwale P, et al. Comparison of

echocardiography and electron beam tomography in
differentiating the etiology of heart failure. Clin Cardiol.
2000;23(6):41720.
160. Shemesh J, Tenenbaum A, Fisman EZ, et al. Coronary

calcium as a reliable tool for differentiating ischemic from
nonischemic cardiomyopathy. Am J Cardiol. 1996;77(2):
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161. Rajappan K, Bellenger NG, Anderson L, et al. The role of
cardiovascular magnetic resonance in heart failure. Eur J
Heart Fail. 2000;2(3):24152.
162. Doherty NE 3rd, Seelos KC, Suzuki J, et al. Application of
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evaluation of response to angiotensin-converting enzyme
inhibitor therapy in dilated cardiomyopathy. J Am Coll
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163. Danias PG, Roussakis A, Ioannidis JP. Diagnostic perfor
mance of coronary magnetic resonance angiography as
compared against conventional X-ray angiography: a
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164. Kim RJ, Wu E, Rafael A, et al. The use of contrast-enhanced
magnetic resonance imaging to identify reversible
myocardial dysfunction. N Engl J Med. 2000;343(20):
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165. Nandalur KR, Dwamena BA, Choudhri AF, et al. Diagnostic
performance of stress cardiac magnetic resonance imaging
in the detection of coronary artery disease: a meta-analysis.
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166. Simonetti OP, Kim RJ, Fieno DS, et al. An improved MR
imaging technique for the visualization of myocardial
infarction. Radiology. 2001;218(1):21523.
167. McCrohon JA, Moon JC, Prasad SK, et al. Differentiation of
heart failure related to dilated cardiomyopathy and coronary
artery disease using gadolinium-enhanced cardiovascular
magnetic resonance. Circulation. 2003;108(1):549.

168. Soriano CJ, Ridocci F, Estornell J, et al. Noninvasive diag

nosis of coronary artery disease in patients with heart
failure and systolic dysfunction of uncertain etiology,
using late gadolinium-enhanced cardiovascular magnetic
resonance. J Am Coll Cardiol. 2005;45(5):7438.
169. Wu E, Judd RM, Vargas JD, et al. Visualisation of presence,
location, and transmural extent of healed Q-wave and nonQ-wave myocardial infarction. Lancet. 2001;357(9249):
218.
170. Izawa H, Murohara T, Nagata K, et al. Mineralocorticoid
receptor antagonism ameliorates left ventricular diastolic
dysfunction and myocardial fibrosis in mildly symptomatic
patients with idiopathic dilated cardiomyopathy: a pilot
study. Circulation. 2005;112(19):29405.
171. Assomull RG, Prasad SK, Lyne J, et al. Cardiovascular

magnetic resonance, fibrosis, and prognosis in dilated
cardiomyopathy. J Am Coll Cardiol. 2006;48(10):197785.
172. de Leeuw N, Ruiter DJ, Balk AH, et al. Histopathologic
findings in explanted heart tissue from patients with endstage idiopathic dilated cardiomyopathy. Transpl Int. 2001;
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173. Knaapen P, Boellaard R, Gtte MJ, et al. Perfusable tissue
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pathologic correlation with PET in ischemic and non
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175. Geltman EM. Metabolic imaging of patients with cardio
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176. Bengel FM, Higuchi T, Javadi MS, et al. Cardiac positron
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177. Eisenberg JD, Sobel BE, Geltman EM. Differentiation of
ischemic from nonischemic cardiomyopathy with positron
emission tomography. Am J Cardiol. 1987;59(15):141014.

CHAPTER 68
Pericardial Disease
Trevor Jenkins, Brian D Hoit

Snapshot

Acute Pericardi s

Pericardial Eusion

M-Mode and Two-Dimensional Echocardiography

Pericardial Tamponade

INTRODUCTION
Pericardial heart disease represents a spectrum of
conditions with significant mortality and morbidity
commonly encountered in cardiovascular medicine.
Normal pericardial anatomy was first described in
antiquity, while descriptions of pericardial pathology
appeared during the 17th to 18th centuries.1 Ultrasound
visualization of the pericardium has advanced rapidly
during recent decades and has become an essential
tool for diagnosis and management of pericardial heart
disease. The value of echocardiography in this regard is
the ability to display both structure and physiology at
high spatial and temporal resolution. Efforts to visualize
pericardial disease originate with the early days of cardiac
ultrasound. Inge Edler first published images of an
anterior pericardial effusion in 1961.2 Harvey Feigenbaum
published several early manuscripts documenting the
power of echocardiography to display pericardial effusions
and published the relation between the swinging heart
in cardiac tamponade by M-mode with electrical alternans
by electrocardiography (ECG).3 Such early observations
helped expand the scope of echocardiography beyond
detection of mitral stenosis.4

Constric ve Pericardi s

Eusive-Constric ve Pericardi s

Congenital Anomalies

Mul modality Imaging of the Pericardium

Anatomy and Echocardiographic

Appearance
The pericardium is an avascular tissue comprising two
histologically distinct layersthe visceral pericardium, a
serosal single cell layer adherent to the epicardium and
great vessels; and the parietal pericardium, a thick fibrous
outer layer. A potential space is enclosed between these
two layers, which normally contains 1535 mL of serous
fluid distributed mostly over the atrialventricular and
interventricular grooves.5 This fluid serves as a lubricant
during cardiac motion. Pericardial reflections around the
proximal ascending aorta, central pulmonary arteries,
pulmonary veins, and venae cavae form the oblique and
transverse sinuses. The fibrous parietal pericardium is less
distensible than myocardium, a property that functionally
limits myocardial chamber distention.6,7
The normal pericardium is usually not well visualized
by two-dimensional (2D) echocardiography. The typical
appearance is that of a thin, bright, highly echogenic line
denoting the intersection of parietal pericardium and lung
tissue. The normal pericardium by computed tomography
(CT) is < 2 mm in diameter. Thickening of the pericardium
(> 5 mm diameter) may allow for direct visualization.

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Normally, a trace amount of pericardial fluid may be seen

at end-systole.

Physiology and Pathophysiology

The pericardium is not essential for life as no adverse
consequences follow congenital absence or surgical removal of the pericardium. However, the pericardium serves
many important functions (Table 68.1). Most relevant
to echocardiographic evaluation, it limits distension of
the cardiac chambers, and facilitates interaction and
coupling of the ventricles and atria.8 Pericardial restraint
of ventricular filling becomes significant when the
pericardial reserve volume (the normally small difference
between unstressed pericardial volume and cardiac
volume) is exceeded. This may occur with rapid increases
in blood volume and in disease states characterized by
rapid increases in heart size, such as acute mitral and
tricuspid regurgitation. In contrast, chronic stretching of
the pericardium results in stress relaxation and creep
(decreased pericardial pressure and increased in volume
with constant stretch, respectively, owing to viscoelastic
properties of the pericardium) and cellular hypertrophy,
which explains why large but slowly developing effusions
do not produce tamponade.8
In view of the pericardiums simple structure, clinicopathological processes involving it are understandably
few and includes only pericarditis and its complications,
tamponade and constriction, and congenital lesions.
However, the pericardium is affected by virtually every
category of disease, including infectious, neoplastic,
immune-inflammatory, metabolic, iatrogenic, traumatic,
Table 68.1: Functions of Pericardium

Mechanical
Effects on individual chambers:

Constrains chamber distention during cardiac cycle

Modulates cardiac chamber interaction and coupling

Maintains left ventricular geometric shape
Preserves pressurevolume relation of the cardiac
chambers
Effects on entire heart:
Lubrication and friction reduction

and congenital etiologies. Thus, pericardial disease

may present either as an isolated phenomenon or as a
complication of a variety of systemic disorders, trauma, or
certain drugs. In these settings, pericardial involvement
may be overshadowed by extracardiac manifestations
and difficult to recognize.8 This chapter will review the
echocardiographic findings in each of these conditions.

ACUTE PERICARDITIS
Acute pericarditis may be isolated or present as a
manifestation of a systemic process. Although the etiology
is highly variable, most cases of acute pericarditis are
idiopathic or viral. Inflammation of the pericardium
is usually silent echocardiographically, as echogenic
brightness of the pericardium lacks sufficient diagnostic
sensitivity and specificity.
Echocardiography is recommended as the initial
noninvasive imaging test for acute pericarditis, because
it accurately detects pericardial effusion and tamponade,
and ventricular dysfunction due to myopericarditis.9
Echocardiography estimates the volume of pericardial
fluid, identifies cardiac tamponade, suggests the basis of
pericarditis, and documents associated acute myocarditis.
In addition, the presence of adhesions, fibrous strands,
hemorrhage, and loculations may aid in the diagnosis of
morbid conditions such as purulent bacterial pericarditis
that may require pericardiocentesis. Although patients
with purely fibrinous acute pericarditis have a normal
echocardiogram, the presence of a pericardial effusion is
consistent with acute pericarditis and is one of the criteria
for its diagnosis. A transthoracic echocardiogram (TTE) is
particularly critical in the setting of high-risk features of
acute pericarditis associated with worse outcome including
fever > 38C, subacute onset, an immunosupressed state,
trauma, or evidence of hemodynamic compromise.10
The use of echocardiography for the evaluation of all
patients with suspected pericardial disease was given
a Class I recommendation by a 2003 task force of the
American College of Cardiology (ACC), the American
Heart Association (AHA), and the American Society of
Echocardiography (ASE).11 Additional imaging modalities
may be necessary if the TTE is negative or inconclusive in
a patient with complex or atypical clinical presentations.

Mechanical barrier to infection

Balances inertial hydrostatic and gravitational forces
Miscellaneous (vasomotor, immunological, fibrinolytic, regulation of localized gene and protein expression)

PERICARDIAL EFFUSION
Accumulation of transudative or exudative fluid in excess
of 50 mL is abnormal and may be seen with pericarditis

Chapter 68: Pericardial Disease

Table 68.2: Etiology of Pericarditis

Idiopathic:
Infectious (viral, bacterial, mycobacterial, fungal, or AIDS/HIV)
Autoimmune (systemic lupus erythematosus, rheumatoid
arthritis, systemic sclerosis, or ankylosing spondylitis)
Neoplastic [primary (mesothelioma), secondary (breast,
lung, melanoma, lymphoma)]
Radiotherapy:
Nephrogenic (uremic, dialytic)
Cardiac injury (surgery, interventional, trauma)
[acute

or

chronic

of cases. In the remainder, patients with evidence of an

inflammatory process were most likely to have acute
idiopathic pericarditis, while those without inflammatory
signs or tamponade were more likely to have a chronic
idiopathic effusion, and those with cardiac tamponade
but without inflammatory signs most commonly had a
malignant effusion.14

M-MODE AND TWO-DIMENSIONAL

ECHOCARDIOGRAPHY

Metabolic (drugs, myxedema, amyloidosis)

Myocardial infarction
syndrome)]

1437

(Dresslers

of any etiology (Table 68.2). Pericardial effusions are

very common after cardiac surgery. In 122 consecutive
patients studied before and serially after cardiac surgery,
effusions were present in 103 patients; the majority
appeared by postoperative day 2, reached their maximum
size by postoperative day 10, and usually resolved without
sequelae within the first postoperative month.12 However,
large effusions or effusions causing pericardial tamponade
are uncommon following cardiothoracic surgery. In one
retrospective survey of more than 4,500 postoperative
patients, only 48 were found to have moderate or large
effusions by echocardiography; of those, 36 met diagnostic
criteria for tamponade.13
Effusion should be suspected and an echocardiogram
obtained in all patients who present with chest pain
consistent with pericarditis or aortic dissection, an
enlarged (typically flask shaped) cardiac silhouette
seen on chest radiogram, systemic disease associated
with pericardial effusion accompanied by jugular venous
distension, after a myocardial infarction, or in patients
who develop hypotension or hemodynamic instability in
the setting of interventional cardiac procedures. However,
asymptomatic pericardial effusions are often discovered
during the evaluation of an unrelated medical complaint
or disorder.
Chronic effusive pericarditis is an entity of unknown
etiology that may be associated with large, asymptomatic
effusions. Many conditions that cause pericarditis (e.g.
uremia, tuberculosis, neoplasia, connective tissue
disease) produce chronic pericardial effusions. In a series
of 322 patients admitted to a tertiary care hospital with
at least a moderate-sized pericardial effusion, the cause
was attributed to a preexisting medical condition in 60%

Echo is the initial procedure of choice to detect the

presence of a pericardial effusion because it is portable,
noninvasive, can be performed with minimal delay, and
attention to technical detail results in excellent sensitivity
and specificity. The diagnostic feature on M-mode
echocardiography is the persistence of an echo-free space
between parietal and visceral pericardium throughout the
cardiac cycle. Separations that are observed only in systole
represent clinically insignificant accumulations. The
superior spatial orientation of 2D echo allows delineation
of the size and distribution of pericardial effusion, as well
as detection of loculated fluid. As the amount of pericardial
fluid increases, fluid distributes from the posterobasilar
left ventricle (LV) apically and anteriorly, and then laterally
and posteriorly to the left atrium (LA). Fluid adjacent to the
right atrium (RA) is an early sign of pericardial effusion. A
left pleural effusion may mimic a pericardial effusion on
M-mode, in which cases fluid anterior to the descending
aortic on a 2D echo parasternal long axis establishes the
fluid as pericardial rather than pleural, which is posterior
to the aorta15 (Figs 68.1 and 68.2).
The size of a pericardial effusion on 2D echo is
qualitatively described by the end-diastolic distance of
the echo-free space between the parietal and visceral
pericardium: trivial (seen only in systole), small (< 10
mm), moderate (1020 mm), large (> 20 mm), or very
large (> 2.5 cm); an even distribution of the effusion
sampled from multiple 2D transducer positions increases
the predictive accuracy of the estimate. 2D echo also
allows for the detection of fluid that may be loculated, or
an echodensity more consistent with an exudate or clot
rather than a transudate. Exudative pericardial effusions
may show features such as stranding, adhesions, or an
uneven distribution reflecting a more inflammatory
composition; finding these frond-like, band-like, or
shaggy intrapericardial echoes should alert one to the
possibility of a difficult and potentially less therapeutic

1438

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 68.1A to D: Two-dimensional (2D) echocardiography of pericardial effusions of varying size. Anterior (*) and posterior (arrow) are
seen as echo-free spaces of increasing size including small, posterior (A), moderate circumferential (B), and large circumferential (C).
Figure D shows a large pleural effusion (curved arrow) posterior to a small pericardial effusion (arrow) with the pericardium visualized as
an echogenic linearity between both. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

pericardiocentesis and a more complicated course, but

have little value in identifying the cause of the effusion
(Figs 68.3A and B). Pericardial effusions that contain clots
(e.g. after cardiac surgery), may be missed on a TTE and
may require transesophageal echo (TEE), CT, or cardiac
magnetic resonance imaging (CMR).
Distinguishing epicardial fat from (particularly
anterior) pericardial effusion may be difficult, but
epicardial fat is slightly echogenic and moves in concert
with the heart, whereas pericardial effusion is generally
echolucent and motionless. Epicardial fat may appear
circumferentially (fat envelope) and be difficult to discern.
In addition to its mimicry, pericardial fat accumulation is
a source of bioactive molecules, is significantly associated

with obesity-related insulin resistance, and is a coronary

risk factor16 (Fig. 68.4).

PERICARDIAL TAMPONADE
Cardiac tamponade is a life-threatening condition
caused by fluid accumulation in the pericardial sac and
is characterized by elevation and equalization of cardiac
diastolic and pericardial pressures, a reduced cardiac
output, and an exaggerated inspiratory decrease in arterial
systolic pressure (>10 mm Hg) referred to as pulsus
paradoxus. Cardiac tamponade is poorly related to the size
of the effusion, as it is the rapidity of fluid accumulation in
the pericardial space and the eclipse of pericardial reserve

Chapter 68: Pericardial Disease

1439

Figs 68.2A to D: M-mode echocardiography of pericardial effusions of varying size. Anterior (*) and posterior (arrow) are seen as
echo-free spaces of increasing size including small, posterior (A), moderate circumferential (B), and large circumferential (C). Concurrent pleural (curved arrow) and pericardial effusion are shown in Figure D. Note that parietal pericardium displays relatively flat motion
throughout the cardiac cycle best visualized in Figure C. (LV: Left ventricle; RV: Right ventricle).

Figs 68.3A and B: Exudative pericardial effusions as seen by two-dimensional (2D) echocardiography apical views. Frond-like (A) and
band-like (B) adhesions are seen bridging a large pericardial effusion fluid, indicating an inflammatory component to the effusion. Note
the thickened pericardium in Figure A. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

1440

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 68.4: An echo-clear space (arrow) is seen anterior to the

pericardium due to epicardial fat. (AO: Aorta; LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).

volume that elevates pericardial pressure and interferes

with cardiac filling.
Cardiac tamponade may be acute or subacutechronic
and should be viewed hemodynamically as a continuum
ranging from mild (pericardial pressure lower than 10 mm
Hg) to severe (pericardial pressure higher than 1520 mm
Hg). Mild cardiac tamponade may cause few symptoms,
whereas moderate tamponade and especially severe
tamponade produce precordial discomfort, dyspnea, and
a sense of doom.
Patients who are severely hypovolemic due to
hemorrhage, dialysis, or overdiuresis may have low
pressure tamponade in which the intracardiac and pericardial diastolic pressures are < 10 mm Hg. In a series of
279 patients who underwent combined pericardiocentesis
and cardiac catheterization, 143 patients (51%)
were diagnosed with cardiac tamponade defined as
intrapericardial pressures equal to RA pressure prior to
pericardiocentesis.17 Low pressure cardiac tamponade
was diagnosed in 29 patients (10%) who had an initial
intrapericardial pressure of < 7 mm Hg and a RA pressure
after pericardiocentesis of < 4 mm Hg. Clinical findings
commonly associated with cardiac tamponade, such
as sinus tachycardia, jugular venous distention, and
pulsus paradoxus were less common in the low pressure
group. However, the hemodynamic significance of these
effusions could be demonstrated on echo by right heart
chamber collapse and respiratory variations in Doppler
transvalvular flow velocities, despite the absence of
vena caval plethora. In the absence of clinical signs of

tamponade, urgent pericardiocentesis is usually not

necessary, but careful monitoring is warranted.
Tamponade may also be regional. A loculated,
eccentric effusion, or localized hematoma can produce
regional tamponade in which only selected chambers
are compressed. As a result, the typical physical, hemodynamic, and echocardiographic signs of tamponade are
often absent. Regional tamponade is most often seen after
pericardiotomy or myocardial infarction; clinical suspicion
should be heightened in these settings. Establishing
the diagnosis is challenging and may require additional
echocardiographic views (e.g. transesophageal) and other
advanced imaging techniques (e.g. CT, CMR).
When cardiac tamponade is suspected, a 2D echo with
Doppler should be obtained emergently unless a delay
might prove life-threatening. CT and CMR are used only
for complicated cases such as postoperative or loculated
effusions. While there are many echo signs of tamponade,
the most important ones are the presence of a pericardial
effusion, dilated (plethoric) inferior vena cava (IVC)
and hepatic veins (which indicate that systemic venous
pressures are elevated), and a LV with reduced enddiastolic and systolic dimensions with Doppler evidence
of reduced stroke volume and cardiac output.
In most cases of cardiac tamponade, other classic
echo-Doppler findings are also present and confirm
the diagnosis. These signs include right heart diastolic
chamber collapses (when pericardial pressures exceed
intracardiac pressure), an inspiratory bulge or bounce of
the interventricular septum into the LV, and characteristic
abnormal respiratory changes in Doppler flow velocity
recordings.

M-Mode and Two-Dimensional Echo

Two-dimensional echo imaging from standard transducer
positions establishes the qualitative size of the effusion, its
distribution, and to an extent, the nature of the effusion.
When cardiac tamponade is present with a moderate
or large effusion, the LV cavity dimensions are reduced,
and because mass is conserved, the wall thickness is
increased (pseudohypertrophy)18 (Figs 68.5A and B).
When the effusion is massive, the heart swings freely in
the pericardial space and displays a pendular motion
that is associated with electrical alternans (Movie clip 1).
Right ventricular end-diastolic diameter increases
during inspiration while reciprocally, LV end-diastolic
diameter decreases; opposite changes are seen during
expiration (see below). An important sign of tamponade

Chapter 68: Pericardial Disease

1441

Figs 68.5A and B: Pseudohypertrophy (A) is demonstrated in the setting of a large pericardial effusion with tamponade physiology with
normalization; (B) of left ventricular wall thickness after pericardiocentesis.

Fig. 68.6: Plethora (dilation) of the inferior vena cava (IVC) indicative of elevated central venous filling pressure in the setting of
tamponade. (L: Liver).

that should be carefully sought is IVC plethora, defined as

dilation of the IVC and hepatic veins with < 50% reduction
in diameter during inspiration. In one series, IVC plethora
was present in 92% of pericardial effusions that were
associated with pulsus paradoxus and which required
pericardial drainage.19 Although IVC dilation is highly
sensitive for cardiac tamponade, it is a nonspecific sign
indicating elevated right heart pressures and therefore is
seen in heart diseases in which a pericardial effusion is
absent (Fig. 68.6).
Diastolic RA and right ventricle (RV) chamber
invagination or collapses on 2D echo are usually seen

in cardiac tamponade and are particularly valuable in the

diagnosis of low pressure tamponade when IVC dilation
is minimal or absent. The subcostal view is often the best
to visualize RA and RV chamber collapse. The superior
temporal resolution of M-mode echo makes it ideal for
judging the timing and duration of collapse. Occasionally,
LA and LV chamber collapses are observed (Figs 68.7A
and B).
Chamber collapses indicate transient negative
transmural pressure and occur during their respective
relaxation phase when intracavitary pressure reaches its
nadir.20 Thus, atrial collapse begins at end-diastole near
the peak of the R-wave, while ventricular collapse begins
in early diastole after the end of the T-wave. In general,
specificity of collapses is greater and tamponade more
severe, the longer the duration of compression. Brief RA
collapse is sensitive but not specific, whereas RA collapse
that exceeds one-third of the cardiac cycle is nearly 100%
sensitive and specific for clinical cardiac tamponade.21
RV diastolic collapse generally occurs when cardiac
output has decreased about 20% from baseline but before
systemic blood pressure has fallen; initially, it is seen
only during inspiration, but as tamponade becomes
more severe it occurs throughout the respiratory cycle22
(Movie clip 2). Experimental studies indicate that right
heart chamber collapse occurs earlier than pulsus
paradoxus, and that the sensitivity and specificity of
chamber collapse improves as the severity of tamponade
increases. These studies also suggest that RA chamber
collapse may have a higher predictive value than RV
collapse.23

1442

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 68.7A and B: Two-dimensional (2D) echocardiograms in the apical four-chamber view demonstrating chamber collapse due to
tamponade. During late diastole, there is inversion of the right atrial lateral wall (A), and right ventricular free wall (B). (AO: Aorta; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).

Table 68.3: Sensitivity and Specificity of Right Heart

Chamber Collapses in Cardiac Tamponade

Sensitivity (%) Specificity (%)

Any chamber

90

65

Right atrium

68

66

Right ventricle

60

90

Simultaneous right atrium/

right ventricle (RA/RV)

45

92

Source: Modified from Reference 24.

Although the sensitivity and specificity of collapses are

variable (Table 68.3), the absence of any cardiac chamber
collapse has > 90% negative predictive value for clinical
cardiac tamponade.24 However, right heart diastolic
collapse may occur only at higher levels of pericardial
pressure, or may be absent in conditions in which right
heart chamber pressures are elevated before the effusion
accumulated, as may be seen with RV hypertrophy and
severe pulmonary hypertension. Conversely, collapse of
the right heart chamber may occur earlier than normal
when intracardiac pressures are low owing to hypovolemia
or with coexisting severe LV dysfunction.25,26 Posterior
loculated effusions after cardiac surgery and severe
pulmonary arterial hypertension may produce LA and LV
diastolic collapse. As indicated earlier, establishing the
diagnosis of regional tamponade is challenging and may
require nontraditional echo views, TEE, CT, or CMR.

In cardiac tamponade, inspiration lowers right heart

pressures and augments systemic venous return as it does
in normal individuals. However, unlike the minimal (< 5%)
change in left-sided filling during normal inspiration in
normal individuals, left heart filling decreases abnormally
in cardiac tamponade, resulting in a reduced stroke volume
and the appearance of pulsus paradoxus. This phenomenon
is due to enhanced ventricular interdependence, wherein an
increase in filling on one side of the heart is associated with
a decrease on the opposite side. Thus, during inspiration
there is an increase in RV dimension and a decrease in LV
dimension that is due to septal movement toward the LV free
wall (and a decrease in the pulmonary venous to LA pressure
gradient). The result is a characteristic inspiratory bulge or
bounce of the interventricular septum into the LV. It should
be recognized that an inspiratory septal bulge or bounce is
not specific for cardiac tamponade but may be seen in other
conditions associated with pulsus paradoxus, such as chronic
obstructive pulmonary disease and pulmonary embolism.
In these instances, the clinical context and the absence of a
pericardial effusion rule out cardiac tamponade as causal. On
the other hand, an inspiratory septal bulge may be absent in
cardiac tamponade when there is LV hypertrophy or marked
preexisting elevated LV filling pressures.27

Doppler Flow Velocity Recordings

Analogous to the changes seen on M-mode and 2D
echo, characteristic respiratory changes occur in pulsed

Chapter 68: Pericardial Disease

1443

Figs 68.8A and B: Pulsed wave Doppler in a patient with cardiac tamponade. Note the increased expiratory flow velocity of the mitral
valve (A) and increased inspiratory flow velocity of the tricuspid valve (B).

Fig. 68.9: Hepatic vein flow reversals (arrow) during expiration.

Note systolic forward venous flow predominates in moderatesevere tamponade with systolic inspiratory augmentation (*).

wave Doppler transvalvular velocities when compared

with normal controls and patients with asymptomatic
effusions, namely tricuspid and pulmonary flow velocities
increase with inspiration while simultaneously mitral
and aortic valve flow velocities decrease. The changes are
greatest on the first beat of inspiration and expiration (a
point which helps differentiate the respiratory variation
seen in obstructive lung disease). Respiratory variation
in the isovolumic relaxation and ejection times are also
seen28,29 (Figs 68.8A and B).
Normal hepatic venous flow is biphasic, with systolic
velocity greater than diastolic velocity, and reduced

forward velocity or small reversals at atrial contraction

and end systole (venous reversals); with inspiration, both
peak systolic and diastolic flow velocities increase. In
mild cardiac tamponade, forward flow velocities decrease
and venous flow during systole predominates because
intrapericardial pressure decreases significantly only
during ventricular ejection. In moderate tamponade,
diastolic flow velocity is markedly reduced but still
augments with inspiration. When tamponade is severe,
forward flow occurs only during systole, and when hepatic
forward flow is observed only during inspiration, systemic
venous and intracardiac pressures are markedly elevated
and equalized at which time cardiac arrest is imminent
(Fig. 68.9).
Patients with cardiac tamponade also display characteristic expiratory changes of hepatic venous flows. On the
first beat of expiration, diastolic flow velocity decreases
or reverses. High positive and negative predictive values
for cardiac tamponade are reported using hepatic venous
recordings (82% and 88%, respectively), but they are not
evaluable in about one-third of patients.24

Echo-Guided Pericardiocentesis
Unless the situation is immediately life-threatening,
experienced staff should perform pericardiocentesis in a
facility equipped with monitoring to optimize the success
and safety of the procedure. Monitoring the cardiac rhythm
and systemic blood pressure is a minimum requirement.
The advantages of needle pericardiocentesis include
the ability to perform careful hemodynamic measurements

1444

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 68.10A and B: Two-dimensional (2D) echocardiography-assisted pericardiocentesis from the apical four-chamber view. Note the
appearance of agitated saline bubbles (Figure A arrow) after successful needle entry into the pericardial space. Catheter advancement
(Figure B arrow) is visualized as the pericardial drain is passed into the effusion. (LV: Left ventricle; RV: Right ventricle).

and relatively simple logistic and personnel requirements.

The safety of the procedure has been increased by using
2D echo guidance with a 1.2% major complication rate in
1,127 cases over 21 years.30 Echocardiographic guidance
assists pericardial drainage using alternative sites on
the chest wall. Injection of agitated saline and imaging
can confirm that the pericardial space was entered
(Figs 68.10A and B).

CONSTRICTIVE PERICARDITIS
Constrictive pericarditis (CP) is a condition in which
a thickened, scarred, inelastic, noncompliant, and
often calcified pericardium limits diastolic filling of the
ventricles. The etiologies of CP are wide-ranging and
include viral and idiopathic pericarditis, cardiac surgery
(the most common antecedent in developed countries),
tuberculosis (common in underdeveloped countries),
collagen vascular disease, trauma, and chest radiation.
CP may have a long latency period after the initial (and
sometimes unrecognized) pericardial injury as might
occur in post-irradiation and post-traumatic pericarditis,
becoming apparent only decades later. Although it is
commonly thought that a normal pericardial thickness
excludes the diagnosis of CP, 28% of 143 surgically
confirmed cases had normal pericardial thickness on CT
scan, and 18% had normal thickness on histopathological
examination.31
Classic chronic CP is encountered less frequently
than it was in the past, whereas subacute CP, occurring
weeks to months after the inciting injury, is becoming

more common. Postoperative CP is an important cause of

constriction, with a reported incidence of 0.2%.8
Transient (acute) constriction may occur in
approximately 15% of patients with acute effusive
pericarditis. Doppler-detected constrictive physiology
resolved without pericardiectomy in 36 of 212 patients
studied retrospectively after an average of approximately
8 weeks at Mayo Clinic.32 The most common cause of
transient CP was caused by pericardial inflammation
after pericardiotomy in nine cases; infection, idiopathic,
collagen vascular disease, trauma, and malignancy
accounted for the remaining cases. Treatment included
anti-inflammatory agents, antibiotics, chemotherapy, and
angiotensin-converting enzyme inhibitors plus diuretics.
Five patients had resolution of constriction without any
specific therapy.
Localized CP is rare, but occasionally a localized band
constricts the inflow or outflow region of one or more of
the cardiac chambers. The clinical picture then simulates
valve disease or venous obstruction.
The suspicion for CP is based on clinical history
and examination, which require subsequent evaluation
and confirmation by imaging and hemodynamic data.
Most patients with CP are referred to evaluate cardiac
function, right heart failure, ascites, or edema. Typical
2D and Doppler echo findings often arrive at the correct
diagnosis and serve to differentiate CP from restrictive
cardiomyopathy and other conditions (e.g. severe
tricuspid regurgitation, chronic liver disease) mimicking
constriction.

Chapter 68: Pericardial Disease

1445

Table 68.4: Echocardiographic Findings in Constrictive

Pericarditis

M-Mode:

Thickened pericardium (dual pericardial echos)

Septal shudder and bounce

Atrial septal notch (immediately after atrial systole)

Flattening of the posterior left ventricle (LV) wall in
mid-late diastole
Premature opening of the pulmonic valve
Two-dimensional (2D) echo:

Increased pericardial thickness (best with transesophageal echo)

Septal shudder and bounce

Tubular appearance to ventricles with dilated atria

DCruz sign [abnormal angle formed by LV and left atrium

(LA) posterior walls]

Vena caval plethora

Sharp diastolic filling halt

Fig. 68.11: M-mode echocardiogram from a patient with constrictive pericarditis. Note the echo-bright thickened posterior
pericardium and flat posterior left ventricular (LV) wall during mid
to late diastole (arrow). An atrial systolic notch (*) is visualized
after atrial systole.

Doppler:
Restrictive diastolic filling pattern on mitral and tricuspid
inflow
E/A velocity ratio > 1.5 with shortened deceleration time
(< 150 ms)
Tricuspid inflow velocity increases on first beat after
inspiration

Mitral inflow velocity decreases on first beat after inspiration

Expiratory increase in pulmonary vein velocities

Diastolic hepatic vein expiratory reversals

Increased mitral propagation velocity (color M-mode)

Tissue Doppler:

Elevated e' velocity

Annulus paradoxus
Annulus reversus

M-Mode and Two-Dimensional Echo

Echo is usually the initial diagnostic procedure in patients
with suspected CP (Table 68.4). Pericardial thickening and
calcification, and abnormal ventricular filling produce
characteristic changes on the M-mode echo. Increased
pericardial thickness is suggested by parallel motion of the
visceral and parietal pericardium, which is separated by a
relatively echo-free space. Echocardiographic correlates
of the hemodynamic abnormalities of CP include diastolic

flattening of the LV posterior wall endocardium, abrupt

posterior motion of the ventricular septum in early
diastole with inspiration (septal shudder and bounce),
and occasionally, premature opening of the pulmonary
valve. These findings, which reflect abnormal filling of the
ventricles, are insensitive and subtle and lack the specificity
to be clinically useful. Although no sign or combination
of signs on M-mode echocardiography is diagnostic of
CP, a normal study virtually rules out the diagnosis33
(Fig. 68.11).
Two-dimensional echocardiography echo reveals
dilation and absent or diminished collapse of the IVC
and hepatic veins indicative of elevated RA pressure,
moderate biatrial enlargement, a sharp halt in ventricular diastolic filling, and abnormal ventricular septal
motion that results from interventricular dependence
(Movie clip 3). LV systolic function as judged by the
ejection fraction is typically normal but may be impaired
in mixed constrictiverestrictive disease, which may occur
with radiation-induced disease or after cardiac surgery.
Measurement of pericardial thickness by TEE correlates
strongly with that obtained by CT and has deserved an
ACC/AHA/ASE Class IIb recommendation.34 However,
it is important to remember that demonstration of the
characteristic constrictive hemodynamics is required to
establish a firm diagnosis (Figs 68.12A and B).

1446

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 68.12A and B: Two-dimensional (2D) echocardiography findings in a patient with constrictive pericarditis. Note the tubular geometry
of left ventricle in parasternal long-axis (A) and apical four-chamber (B) views. Figure A demonstrates DCruz sign, an abnormal contour
of the posterior left ventricle and posterior left atrial walls giving rise to an angle < 150. (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).

Doppler Flow Velocity Recordings

Doppler echo is essential for diagnosis and usually shows
restrictive LV and RV diastolic filling patterns, which
are characterized by high early (E) velocity, shortened
deceleration time (< 150 ms), and a reduced atrial (A)
wave. Mitral inflow velocity usually, but not always, falls
as much as 2540% and tricuspid velocity greatly increases
in the first beat after inspiration35,36 (Fig. 68.13). These
respiration-induced phenomena are manifestations of
enhanced ventricular interaction and are not present
in either normal subjects or patients with restrictive
cardiomyopathy. Increased respiratory variation of mitral
inflow may be missing in patients with markedly elevated
left atrial pressure but can sometimes be brought out in
such patients by preload reduction with a head-up tilt
or diuretic administration.37 Similar to mitral flow, the
respiratory variation in pulmonary venous (particularly
diastolic) flow is often pronounced. Hepatic vein diastolic
flow reversal increases with expiration, reflecting the
ventricular interaction and the dissociation of intracardiac
and intrathoracic pressures, which is essential in the
diagnosis of constriction38,39 (Fig. 68.14). In contrast,
inspiratory hepatic vein diastolic flow reversals suggest
restrictive cardiomyopathy. The propagation velocity of
early diastolic transmitral flow on color M-mode is normal
or increased and is often >100 cm/s.

Fig. 68.13: Transvalvular Doppler flow of the mitral valve in a

patient with constrictive pericarditis. Mitral early inflow velocity falls
by 40% in the first cardiac cycle after inspiration (arrow). There is
inversely, a simultaneous increase in tricuspid inflow velocity (not
shown).

Tissue Doppler imaging is particularly useful in

differentiating between CP and restrictive cardiomyopathy.4043 Tissue Doppler shows a prominent early
diastolic velocity (e') from the medial mitral annulus,
whereas the transmitral E is tall and narrow but the tissue
e' is reduced (< 7 cm/s) in restrictive cardiomyopathy.
The usually positive linear relation between E/e' and left
atrial pressure is reversed in many patients with CP since

Chapter 68: Pericardial Disease

1447

Fig. 68.14: Doppler echocardiography of the hepatic vein in a

patient with constrictive pericarditis. Hepatic vein diastolic flow
reversal increases with expiration (arrows), due to ventricular interaction and the dissociation of intracardiac and intrathoracic pressures.

Figs 68.15A and B: Tissue Doppler velocities in a patient with constrictive pericarditis. There are normal lateral (A) and supranormal
septal (B) early diastolic tissue velocities. Note the annulus reversus sign with higher septal than lateral early diastolic velocity. This
sign is likely due to tethering of the lateral atrioventricular groove to the thickened pericardium.

medial e' increases progressively as the severity of

constriction becomes worse; this has been called annulus
paradoxus.44 In addition, lateral mitral annulus e is
usually lower than e' from the medial annulus in patients
with CP; this annulus reversus is thought to be due to
the tethering of the lateral atrioventricular groove to the
thickened pericardium45 (Figs 68.15A and B). Differences
in longitudinal and circumferential deformation may
distinguish CP from restrictive cardiomyopathy. Thus,
circumferential strain, torsion, and early diastolic untwisting
are reduced, and longitudinal strain, displacement, and early
diastolic tissue velocities are unchanged in constriction,
whereas circumferential strain and early diastolic untwisting

are preserved and longitudinal mechanics are reduced in

restrictive cardiomyopathy. However, there could be reduced
regional longitudinal mechanics in CP due to tethering of the
involved pericardium.46
In those situations where echo findings are equivocal,
additional imaging testing (CT or MR) is needed to make
the diagnosis with greater confidence. In some patients,
hemodynamic cardiac catheterization may be necessary
to establish the diagnosis. Even when the diagnosis of CP is
certain after an echo, other imaging tests are often necessary
to evaluate pericardial inflammation, coexisting myocardial
disease, or comprehensive pericardial as well as cardiovascular anatomy for subsequent management decisions.47

1448

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 68.16A and B: Two-dimensional (2D) echocardiogram (A) and M-mode of effusive constrictive pericarditis from the parasternal
view. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).

EFFUSIVE-CONSTRICTIVE
PERICARDITIS
EffusiveCP is an uncommon syndrome of pericardial
constraint with clinical and pathophysiological features
of both cardiac tamponade and chronic CP. Idiopathic
pericarditis, radiation, and neoplasia are the most
frequent antecedents. Unlike the predominant parietal
involvement in chronic CP, involvement of the visceral as
well as the parietal pericardium (epicarditis) is involved in
effusiveCP with inflammation, fibrotic thickening, and
variable degrees of myocardial adherence.48 In a series of
190 patients undergoing pericardiocentesis for cardiac
tamponade, the disorder was diagnosed in 15 (8%).49
The diagnosis was defined by a failure of the right atrial
pressure to fall by 50% or to a level below 10 mm Hg after
pericardiocentesis. In these patients, constriction was
clinically suspect in 7; symptoms were usually present
for less than 3 months, right-heart failure was evident in
all, evidence of acute pericarditis was noted in 7, pulsus
paradoxus was noted in 10, and pericardial calcification
was present in none.
Noninvasive imaging is not very useful in the diagnosis
of effusiveCP.50 The echo findings of effusiveCP depend
on the stage of the disease, although most often the
M-mode, 2D, and Doppler features are consistent with
a moderate or large pericardial effusion and cardiac
tamponade. The pericardial effusion may become
organized; echogenic and fibrinous strands may result in
regions of loculation (Figs 68.16A and B).

Metastatic neoplasia is the leading cause of pericardial

disease in hospitalized patients, most often in patients with
lung or breast cancer, melanoma, lymphoma, and acute
leukemia. Many cases are asymptomatic and are found only
incidentally at autopsy, but others cause symptoms and may
progress to cardiac tamponade. Primary cardiac tumors may
invade the pericardium directly51,52 (Figs 68.17A and B).
Primary mesothelioma of the pericardium is a rare and
highly lethal tumor. Signs and symptoms are nonspecific,
and echocardiography is insensitive for its detection; CT
and magnetic resonance imaging (MRI) are the most
promising diagnostic tests. Other primary tumors of the
pericardium are quite rare.
Pericardial tumors may appear as nodular echodensities
or diffuse pericardial thickening, the latter more common
with malignant tumors. They are usually nonmobile
although they may contain mobile elements. Benign lesions
(teratomas, hemangiomas, and lymphangiomas) can appear
as cystic masses with septations. The pericardium may be
thickened and cause constriction; less commonly, effusive
CP occurs. Echocardiography rapidly and accurately
detects pericardial effusion, identifies metastatic lesions,
and provides evidence for cardiac compression. Delayedenhancement contrast MRI is particularly useful in evaluating
pericardial mass lesions.

CONGENITAL ANOMALIES
Pericardial cysts are usually rare remnants of defective
embryological development of the pericardium. In

Chapter 68: Pericardial Disease

1449

Figs 68.17A and B: A pericardial metastasis near the right ventricle (A) is seen in patient with widely metastatic lymphoma in the
subcostal window. Definity contrast enhancement (B) opacifies the right ventricle (RV) blood pool. (L: Liver).

Figs 68.18A and B: A pericardial cyst (arrow) is seen adjacent to the right ventricle. Color Doppler flow (B) within the right atrium
demonstrates normal vena caval flow but a lack of flow within the pericardial cyst. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

contrast, hydatid cysts are infectious in nature. Cysts

vary greatly in size and are most commonly found in the
right cardiophrenic angle. Cysts are benign and produce
no local or general symptoms; their importance lies in
differentiation from neoplasm. Although they can be
demonstrated echocardiographically as echolucent
spaces that do not communicate with the pericardial sac
on echocardiography, the nature of the lesion usually is
confirmed by CT (Figs 68.18A and B).
Pericardial diverticula are very rare and can be
congenital or acquired malformations found most often
at the costophrenic angles. Unlike pericardial cysts,

pericardial diverticula may communicate with the

pericardial space and change in size depending on body
position and respiration.
Congenital absence of the pericardium is a rare
condition that may be isolated or associated with other
cardiac and noncardiac abnormalities, and pericardium
may be completely or partially absent. Complete absence
of the left pericardium is the most common variant.
Echocardiographic and Doppler features of congenital
absence of the pericardium include unusual imaging
windows, enlargement of the RV, excessive cardiac motion,
and abnormal motion of the interventricular septum.53

1450

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 68.19: MRI-delayed enhancement sequence with gadolinium

contrast demonstrates a diffusely enhancing (arrows), thickened
pericardium.

MULTIMODALITY IMAGING OF THE

PERICARDIUM
Although echo remains as usually the initial imaging
test for pericardial disorders because of its ease of use,
wide availability, bedside availability, cost-effectiveness,
and versatility, additional imaging modalities may be
necessary. In general, CMR provides excellent anatomical,
tissue characteristics and some physiological data.
Compared to echo, CMR provides superior anatomical
assessment with better spatial resolution and a larger field
of view, but physiological assessment is more limited.
Specifically, CMR provides diagnostic identification of
pericardial edema from short tau inversion [T2 double
inversion recovery (DIR) sequence or short inversion time
inversion recovery (STIR) sequence] and inflammation
from late gadolinium enhancement (LGE) sequences,
which are not available with other imaging modalities54
(Fig. 68.19) Cardiac CT is a predominantly anatomical
modality with superior definition of pericardial
anatomy relative to surrounding chest structures and
pericardial calcification. It is, therefore, used in situations
where anatomy is incompletely defined by echo or a
contraindication for CMRfor example, in a patient
with suspected absence of the pericardium or a patient
with a pacemaker. CT may also be useful for preoperative
planning once pericardectomy is indicated especially in
patients with a previous sternotomy.47
See Movie clips 13 under Chapter 68 in DVD.

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34. Ling LH, Oh JK, Tei C, et al. Pericardial thickness measured
with transesophageal echocardiography: feasibility and
potential clinical usefulness. J Am Coll Cardiol. 1997;
29(6):131723.
35. Hatle LK, Appleton CP, Popp RL. Differentiation of
constrictive pericarditis and restrictive cardiomyopathy
by Doppler echocardiography. Circulation. 1989;79(2):
35770.
36. Oh JK, Hatle LK, Seward JB, et al. Diagnostic role of
Doppler echocardiography in constrictive pericarditis. J
Am Coll Cardiol. 1994;23(1):15462.
37. Oh JK, Tajik AJ, Appleton CP, et al. Preload reduction to
unmask the characteristic Doppler features of constrictive
pericarditis. A new observation. Circulation. 1997;95(4):
7969.

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38. Klein AL, Cohen GI, Pietrolungo JF, et al. Differentiation

of constrictive pericarditis from restrictive cardiomyopathy
by Doppler transesophageal echocardiographic measurements of respiratory variations in pulmonary venous flow.
J Am Coll Cardiol. 1993;22(7):193543.
39. Sun JP, Abdalla IA, Yang XS, et al. Respiratory variation of
mitral and pulmonary venous Doppler flow velocities in
constrictive pericarditis before and after pericardiectomy.
J Am Soc Echocardiogr. 2001;14(11):111926.
40. Garcia MJ, Rodriguez L, Ares M, et al. Differentiation of
constrictive pericarditis from restrictive cardiomyopathy:
assessment of left ventricular diastolic velocities in
longitudinal axis by Doppler tissue imaging. J Am Coll
Cardiol. 1996;27(1):10814.
41. Ha JW, Ommen SR, Tajik AJ, et al. Differentiation of
constrictive pericarditis from restrictive cardiomyopathy
using mitral annular velocity by tissue Doppler
echocardiography. Am J Cardiol. 2004;94(3):31619.
42. Sohn DW, Kim YJ, Kim HS, et al. Unique features of early
diastolic mitral annulus velocity in constrictive pericarditis.
J Am Soc Echocardiogr. 2004;17(3):2226.
43. Ha JW, Oh JK, Ommen SR, et al. Diagnostic value of mitral
annular velocity for constrictive pericarditis in the absence
of respiratory variation in mitral inflow velocity. J Am Soc
Echocardiogr. 2002;15(12):146871.
44. Ha JW, Oh JK, Ling LH, et al. Annulus paradoxus: transmitral
flow velocity to mitral annular velocity ratio is inversely
proportional to pulmonary capillary wedge pressure in
patients with constrictive pericarditis. Circulation. 2001;
104(9):9768.
45. Veress G, Ling LH, Kim KH, et al. Mitral and tricuspid
annular velocities before and after pericardiectomy in
patients with constrictive pericarditis. Circ Cardiovasc
Imaging. 2011;4(4):399407.
46. Sengupta PP, Mohan JC, Mehta V, et al. Accuracy and
pitfalls of early diastolic motion of the mitral annulus
for diagnosing constrictive pericarditis by tissue Doppler
imaging. Am J Cardiol. 2004;93(7):88690.
47. Verhaert D, Gabriel RS, Johnston D, et al. The role of
multimodality imaging in the management of pericardial
disease. Circ Cardiovasc Imaging. 2010;3(3):33343.
48. Dahiya A, Lytle BW, Klein AL. Constrictive epicarditis. J Am
Coll Cardiol. 2011;58(6):e11.
49. Sagrist-Sauleda J, Angel J, Snchez A, et al. Effusiveconstrictive pericarditis. N Engl J Med. 2004;350(5):46975.
50. Hancock EW. A clearer view of effusive-constrictive
pericarditis. N Engl J Med. 2004;350(5):4357.
51. Wilkes JD, Fidias P, Vaickus L, et al. Malignancy-related
pericardial effusion. 127 cases from the Roswell Park
Cancer Institute. Cancer. 1995;76(8):137787.
52. Luk A, Ahn E, Vaideeswar P, et al. Pericardial tumors.
Semin Diagn Pathol. 2008;25(1):4753.
53. Connolly HM, Click RL, Schattenberg TT, et al. Congenital
absence of the pericardium: echocardiography as a
diagnostic tool. J Am Soc Echocardiogr. 1995;8(1):8792.
54. Bogaert J, Francone M. Cardiovascular magnetic resonance
in pericardial diseases. J Cardiovasc Magn Reson. 2009;
11:14.

CHAPTER 69
Three-Dimensional Echocardiographic
Assessment in Pericardial Disorders
O Julian Booker, Navin C Nanda

Snapshot

Two-Dimensional Transthoracic Echocardiography

Versus Three-Dimensional Transthoracic

Echocardiography in Pericardial Eusion

Two-Dimensional Transthoracic Echocardiography

Versus Three-Dimensional Transthoracic
Echocardiography in Constric on

INTRODUCTION
The pericardium is a relatively avascular fibroserous sac.
It is created by invagination of the heart within the serous
pericardium and surrounded by the fibrous pericardium.
The serous pericardium is composed of a single layer
of mesothelial cells. The layer adherent to the fibrous
pericardium is termed the parietal layer. The layer deep to
the fibrous pericardium is called the visceral pericardium.
Those portions of the visceral pericardium that are
adherent to the myocardium are specifically called the
epicardium. The fibrous pericardium is much sturdier
than the serous pericardium but is still relatively thin
(< 2 mm) and surrounds the heart and part of the great
vessels.1 Between the serous layers, there is a potential
space that contains 15 to 50 mL of fluid that serves
as lubrication. Most of the fluid is located within the
interventricular and atrioventricular grooves with a
predilection for more dependent areas.
The pericardium has a unique interaction with heart.
The fibrous pericardium attaches to the diaphragm,
anterior mediastinum, and sternum. The pericardium
surrounds the proximal portions of the great vessels, which
serve as additional anchor points. These attachments serve

Two-Dimensional Transthoracic Echocardiography

Versus Three-Dimensional Transthoracic

Echocardiography in Pericardial Masses

to fix the heart within the chest. The nature and position of
the fibrous pericardium also make it an important barrier
to infection. Pericardial constraint allows for greater
isovolumic pressures at any given volume for both the
left and the right ventricles. These benefits of constraint
are curbed by the absence of the pericardium.24 Further
possible roles of pericardial constraint include preventing
acute increases in chamber volume as a response to
elevations in filling pressures. It also helps modulate
the physical and hydrodynamic interaction between
the four cardiac chambers5,6 and creates ventricular
interdependence.7,8
There are anatomical and hemodynamic consequences
to pericardial disease. The cardiac chambers are physically
connected within a confined space within the pericardium.
There is also hydrodynamic interdependence related to
blood flow. Changes in the filling dynamics of one chamber
will impact the others. Tamponade and constriction are
primarily hemodynamic entities resulting from abnormal
pericardial constraint. Echocardiography, with its ability
to not only evaluate structure but also hemodynamic
assessment, is the cornerstone of the evaluation of
pericardial disorders. The standard evaluation involves
Doppler interrogation and two-dimensional (2D) imaging.

Chapter 69: Three-Dimensional Echocardiographic Assessment in Pericardial Disorders

Over the past decade, more powerful computer processors have resulted in larger quantities of data that can
be processed quickly. Development of a full matrix array
has evolved echocardiography from an approximately
5 mm slice to a nearly full volumetric acquisition of the
heart and its immediately surrounding structures using a
gated, multiple-beat acquisition. Three-dimensional (3D)
technology provides the ability to manipulate and crop the
data set in an almost infinite number of orientations and
provides a more complete assessment of cardiac anatomy
including the pericardium. With high-resolution images,
the surfaces of the two layers can be viewed en face.9 This
approach allows for a more complete structural assessment
than two-dimensional transthoracic echocardiography
(2D TTE) alone.
Publications regarding three-dimensional transthoracic echocardiography (3D TTE) in the evaluation of pericardial disease have been limited. Three-dimensional echocardiographys more complete anatomical visualization

1453

can help define potential structural abnormalities. Given

the importance of echocardiography in the evaluation of
pericardial diseases, the potential incremental benefit of
additional 3D images cannot be overlooked. Some of the
potential diagnostic advantages of 3D echocardiography
can be seen in Table 69.1 and Figures 69.1 to 69.11.

TWO-DIMENSIONAL TRANSTHORACIC ECHOCARDIOGRAPHY VERSUS

THREE-DIMENSIONAL TRANSTHORACIC ECHOCARDIOGRAPHY IN
PERICARDIAL EFFUSION
2D TTE is a sensitive, but not very specific method of
identifying pericardial effusion.10 On 2D and M-mode
imaging, pericardial fluid will appear as a hypoechoic
space with hyperechoic structures, the epicardium and
pericardium, respectively, on either side. The planar

Table 69.1: Advantages of 3D TTE* in Evaluating Pericardial Diseases

Anatomy of pericardial layers

Parietal and visceral layers can be visualized en-face
Identification of extent of fibrin deposits, exudative coating over pericardial layers
Better visualization of fibrin strandsextension, attachments, mobility, and consistency
Pericardial effusion
Better identification of the echo reflectors within the fluid
Ability to comprehensively assess effusion behind the atria and right ventricle
More comprehensive identification of loculated effusions
Increased accuracy of size estimation
Pericardial hematomas
Easy identification and differentiation from uncomplicated effusion
Granulomatous disease
Ability to identify the central necrosis and hence the pathological characterization as granuloma
Ability to identify tethering of pericardium
Tumor/mass
More accurate identification of pericardial deposits
Provides clue to identification of malignant nature by identifying the inhomogeneous nature
Identification of extracardiac extension
Constrictive pericarditis
More comprehensive evaluation of extent and severity
Identification of morphological type
Contd...

1454

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Contd...
Differentiation of pericardial effusion from pleural effusion and ascites
En-face viewing of the falciform ligament to better identify ascites
Pericardiocentesis
Better needle visualization with its pyramidal imaging plane
Pericardial cyst
Identification of loculations and trabeculations
*3D TTE, live/real time three-dimensional transthoracic echocardiography.
Source: Reproduced with permission from Sudhakar S, Nanda NC. Role of live/real time three-dimensional transthoracic echocardiography in pericardial disease. Echocardiography. 2012;29:98102.

Figs 69.1A to D: Live/real-time three-dimensional transthoracic echocardiography in pericardial effusion in a 64-year-old female with
renal failure. (A) The arrowhead points to fibrin deposition over the right ventricular pericardium giving a rugged appearance. Pericardial
effusion extends behind both atrial (see also Movie clip 69.1A). (B) Apical four-chamber view. Cropping from bottom displays a smooth
visceral pericardium over the basal left atrial (LA) wall (arrowhead; see also Movie clip 69.1B). (C) Subcostal examination. Arrowheads
show multiple fibrin deposits on the right atrial (RA) visceral pericardium resulting in a rugged appearance (see also Movie clip 69.1C).
(D) Arrowhead shows a flap-like fibrin mass (see Movie clip 69.1D parts 1 and 2). (IVC: Inferior vena cava; LV: Lleft ventricle; PE: Pericardial effusion; TV: Tricuspid valve).6
Source: Reproduced with permission from Ref. 10.

Chapter 69: Three-Dimensional Echocardiographic Assessment in Pericardial Disorders

1455

nature of standard 2D imaging can make distinguishing

pericardial effusions from ascites or pleural effusion
difficult. The falciform ligament, which appears as a linear
band stretching from the liver to the abdominal wall, can
be used to identify ascites. However, the linear nature of the
ligament can make it difficult to distinguish from fibrinous
strands that can sometimes be seen within the pericardial
space. With 3D TTE, the falciform ligament more closely
approximates its appearance in vivo and can be seen as a
sheet-like structure (Fig. 69.2C).
2D TTEs ability to delineate pleural effusion can be
equally difficult. The parasternal long axis view is the most
frequently used to identify pleural effusion. The oblique
sinus represents the reflection of the pericardium. The

result is that pericardial effusions will be found anterior to

the descending aorta. 3D TTE can be used better to identify
the pericardial space in relation to the aorta. Similarly,
visualization of atelectatic lung can help differentiate
pleural and pericardial effusions (Fig. 69.3).
2D TTEs ability to quantify pericardial effusions is
unreliable. Estimation of pericardial volumes using the
posterior free space11 does not adequately account for
loculated effusions or asymmetry related to postural
shifts. 3D TTE can acquire the entire pericardial space in a
single acquisition. By identifying the visceral and parietal
borders, pericardial fluid volumes can be quantified
without geometric assumptions. 3D TTE has proven
itself to be more accurate and reproducible than 2D TTE

Figs 69.2A to C: Live/real time three-dimensional transthoracic

echocardiography in pericardial effusion in an 85-year-old female
with congestive heart failure and ascites. (A) Upper and lower
arrowheads point to visceral and parietal layers of the pericardium,
respectively. Both the pericardial layers are smooth without fibrin
deposition (see also Movie clip 69.2A); (B) Right parasternal
examination. The arrowhead points to a smooth visceral pericardium
overlying the right (RA) and left (LA) atrium (see also Movie clip
2B); (C) The arrowhead shows a localized collection of fibrin over
the visceral pericardium of the left ventricular (LV) free wall (see
also Movie clip 69.2C). Movie clip 69.2D shows pericardial effusion
(arrowhead) extending behind the left atrium. The arrowhead
in Movie clip 69.2E points to three-dimensional images of the
falciform ligament which is a useful landmark in distinguishing
ascites from associated pericardial effusion. It appears as a sheet
of tissue connected to the liver rather than a linear echo mimicking
a fibrin strand seen with conventional two-dimensional imaging.
The arrowhead in Movie clip 69.2F points to the falciform ligament
viewed en-face in another patient with ascites. (D: Diaphragm; DA:
Descending thoracic aorta; IVC: Inferior vena cava; L: Liver; RV:
Right ventricle).
Source: Reproduced with permission from Ref. 10.

1456

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 69.3: Live/real time three-dimensional transthoracic echocardiography in an 84-year-old male with renal failure, and
pericardial and left pleural effusions. Cropping of the threedimensional data set using Qlab software analysis package
revealed a rugged visceral (1, 2, arrowhead) and parietal
(3) pericardium as well as a rugged visceral (4) and parietal
(5) pleura, from fibrin deposits (see also Movie clip 69.3A). Movie
clips 69.3B and C show similar findings using regular cropping in
the same patient. Movie clips 69.3D and E are from a different
patient with pleural effusion, shown for comparison. These show
cropping to more comprehensively assess the extent of pleural
effusion (PLE) and the collapsed lung (arrowhead) and their
relation to the heart (H). Movie clip 69.3F from another patient
with chronic renal failure shows the attachment of collapsed lung
lobes (horizontal arrowheads) to the hilum (vertical arrowheads)
and its relationship to the heart (H). This patient had previously
undergone pericardiectomy for constriction. Both these patients
were studied from the back in the sitting position. (DA: Descending
thoracic aorta; PP: Parietal pericardium; VPL: Visceral pleura;
VP: Visceral pericardium).
Source: Reproduced with permission from Ref. 10.

in quantifying asymmetric pericardial effusions.12 The

potential benefit for serial evaluations of effusion size
cannot be overstated.
3D TTE has improved visualization of the inferior vena
cava, right ventricle, and right atrium. Classic changes
often found in tamponade such as a plethoric inferior
vena cava diastolic collapse of the right ventricle as well
as inappropriate collapse of the atria can be seen by both
2D and 3D TTE. However, these findings can be better
appreciated on 3D TEE providing incremental benefit in
the assessment of tamponade.13
Fibrin deposits have been documented in virtually
every type of pericardial disease. Echogenic material seen
within the pericardial space has been associated with
worse clinical outcomes like recurrence of pericardial
effusions and constrictive pericarditis.14,15 As such,
identifying the presence of these strands becomes

Fig. 69.4: Live/real time three-dimensional transthoracic echocardiography in a 62-year-old male with pericardial effusion
developing following mitral valve replacement. Arrowheads point
to fibrinous strands connecting the visceral and parietal portions of
the pericardium. The accompanying Movie clips 69.4A and B from
the same patient show both mobile and nonmobile fibrin strands. In
Movie clip 69.4A, artifacts appeared when the instrument gain was
decreased and disappeared with increase in gain, emphasizing
the importance of optimizing gain settings when assessing threedimensional images. Movie clip 69.4B shows cropping done using
the Qlab software analysis package. Abbreviations as in previous
figures.
Source: Reproduced with permission from Ref. 10.

critically important in the evaluation of pericardial

effusions. 3D TTE has shown to be superior to 2D TTE in
identifying and characterizing the consistency, location,
and mobility of fibrinous material within the pericardial
space (Figs 69.1 and 69.4). Fibrin stranding and other
echogenic pericardial materials are most often associated
with inflammatory processes. 3D TTEs ability to define
these echo reflectors can help identify more complicated
effusions such as hematomas and purulent effusions
(Figs 69.5 and 69.6).9,16 Should pericardiocentesis be
required, the 3D TTE provides superior anatomical
delineation to guide pericardiocentesis.17

TWO-DIMENSIONAL TRANSTHORACIC ECHOCARDIOGRAPHY VERSUS

THREE-DIMENSIONAL TRANSTHORACIC ECHOCARDIOGRAPHY IN
CONSTRICTION
Fibrinous adhesions are a result of the inflammatory
process within the pericardial space. Fibrinous adhesions
of the visceral and parietal pericardia can be found at the

Chapter 69: Three-Dimensional Echocardiographic Assessment in Pericardial Disorders

1457

Fig. 69.5: Live/real time three-dimensional transthoracic echocardiography in a 50-year-old male status post cardiac transplantation and pericardial effusion compressing the right atrium.
The arrowhead points to the compressed right atrium (RA). Cropping reveals no significant echo reflectors (solid tissue) in the
effusion suggesting that it mainly comprises fluid or represents a
hematoma that is of uniform, homogenous consistency (see also
Movie clip 69.5). The patient improved after drainage of 1,000 cc
of yellowish fluid from the pericardial cavity using a pigtail catheter.
(Abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.

Fig. 69.6: Live/real time three-dimensional transthoracic echocardiography in a 17-year-old male with a bullet injury and subsequent development of pericardial hematoma. The red dots outline
a loculated component of a very large pericardial hematoma (see
also Movie clip 69.6A). Movie clip 69.6B shows a huge pericardial
hematoma (arrowhead) with large multiple echolucencies consistent with fluid collections. These were not well seen on two-dimensional imaging. (Abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.

time of necropsy without any history of overt pericardial

disease.18 Constrictive pericarditis is the condition where
the parietal and/or visceral pericardium become thickened
and sometimes calcified to the point where it inhibits
filling of the cardiac chambers. Constrictive pericarditis is
a difficult-to-diagnose condition. The primary diagnosis of
constriction is made by hemodynamic assessment, either
by echocardiography or simultaneous right and left heart
catheterizations.
Unfortunately, there are no good medical treatments
for chronic constrictive pericarditis as the mechanism
of the constrictive physiology is structural. Because
pericardiectomy is difficult and associated with significant
morbidity, it is paramount that the diagnosis is correct
before proceeding. However, many clinicians prefer
additional evidence in the form of imaging, such as
thickening or calcification of the pericardium to support
the diagnosis before committing the patient to surgery.
Historically, the imaging modalities of choice for
morphological assessment of the pericardium have
been magnetic resonance imaging (MRI) or computed
tomography scan (CT). They have been shown to

accurately delineate the thickness and calcification of the

involved pericardium.
Determining the extent of pericardial involvement
by echocardiography can be unreliable with 2D imaging.
3D TTE has shown the ability to identify pericardial
involvement along the entire extent of both ventricles.9,19
An excellent example can be found in a woman with
recurrent heart failure symptoms. Her 2D TTE showed
limited calcification of the posterior wall of the left ventricle
and anterior free wall of the right ventricle. 3D TTE showed
extensive involvement of the regions in question and was
suggestive of a severe constrictive process (Fig. 69.11).
These findings were corroborated with cardiac MRI and
later confirmed at the time of surgery.10 Similar to MRI,
3D echocardiography can demonstrate tethering of the
affected pericardium to the adjacent myocardium. These
characteristics of 3D TTE may make it an ideal third option
for morphological assessment of pericardium in surgical
planning. The fact that the 3D imaging can be acquired at
the time of the standard 2D echocardiographic evaluation
of constriction may someday make 3D TTE the preferred
choice over MRI and CT.

1458

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Perhaps the greatest benefit of 3D TTE over 2D TTE in the

evaluation of pericardial disease lies in the evaluation of

mass lesions. Manipulation of the 3D data set allows a

more complete visualization of pericardial-based masses.
Systematic cropping can provide information about the
character of a mass. Identifying inhomogeneity within
a mass can provide clues to its nature. Conversely, an
echolucent lesion without flow on Doppler is likely to be a
benign pericardial cyst (Fig. 69.10).
3D TTE has been used successfully in a case series
to provide vital clues as to the pathology of several

TWO-DIMENSIONAL TRANSTHORACIC ECHOCARDIOGRAPHY VERSUS

THREE-DIMENSIONAL TRANSTHORACIC ECHOCARDIOGRAPHY IN
PERICARDIAL MASSES

Figs 69.7A to D: Live/real time three-dimensional transthoracic echocardiography in a 26-year-old male with tuberculous pericardial
effusion. (A) Cropping of the apical four-chamber data set shows no inward motion of the proximal right ventricular free wall (arrowhead)
during systole, probably due to fibrinous adhesions. The distal wall contracts well; (B) Examination of visceral pericardium (VP) over
the ventricles demonstrates a mildly rugged appearance. Movie clips 69.7A and B Parts 1 to 3 show the cropping technique used to
demonstrate the visceral pericardium of both ventricles; (C) A large mass (arrowhead) is seen involving the visceral pericardium of the
left ventricle (LV). The etiology is not clear but it could possibly represent a tuberculous granuloma; (D) The arrowhead points to a large
highly echogenic mass involving the right ventricular visceral pericardium consistent with a calcified granuloma in another patient with
tuberculosis. Movie clip 69.7C from the same patient shows a granuloma (arrowhead) involving the parietal pericardium. Movie clips
69.7D and E are from a different patient with purulent pericardial effusion due to methicillin-resistant Staphylococcus aureus. Movie clip
69.7D shows markedly thickened and echogenic parietal (upper arrowhead) and visceral (lower arrowhead) pericardium. Movie clip
69.7E shows an abnormal loculated appearance of the visceral pericardium (arrowhead) when viewed en-face by cropping from the
apex. (Abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.

Chapter 69: Three-Dimensional Echocardiographic Assessment in Pericardial Disorders

1459

Figs 69.8A and B: Live/real time three-dimensional transthoracic echocardiography in a 66-year-old male with pericardial metastasis
from a malignant thymoma. (A) The arrowhead shows a huge pericardial mass (bounded by red dots) measuring 9.2 6.3 cm. Movie
clips 69.8A to C show the full extent of this huge mass with three-dimensional imaging. Cutting open the tumor in its mid portion (arrowhead in Movie clip 69.8B) revealed solid inhomogenous tissue. Arrowheads in Movie clip 69.8C show parietal involvement of the tumor
by multiple band-like extensions. The two-dimensional study in this patient (Movie clip 69.8D) shows a much smaller mass (arrowhead)
measuring 3.8 1.5 cm attached to the right ventricular outflow tract visceral pericardium; (B) Surgical specimen. (Other abbreviations
as in previous figures).
Source: Reproduced with permission from Ref. 10.

Figs 69.9A and B: Live/real time three-dimensional transthoracic echocardiography in a 68-year-old male with lung carcinoma. (A and B)
The arrowhead points to an irregular mass in the parietal pericardium (see also Movie clips 69.9A and B). The etiology is not clear,
but it could possibly represent a pericardial metastasis. Movie clip 69.9C shows another mass (arrowhead) located over the visceral
pericardium (VP) in this patient. This type of mass (arrowhead) was also seen by two-dimensional imaging (Movie clip 69.9D). However,
the other mass seen involving parietal pericardium (PP) was not detected by this modality. Movie clip 69.9E is from a different patient
with a poorly differentiated lung adenocarcinoma. Arrowhead points to a mass in the parietal pericardium consistent with metastasis.
This was not visualized on two-dimensional imaging. Examination of pericardial fluid in this patient showed the presence of malignant
cells. (Other abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.

masses. In one case, the echolucent core was suggestive

of granuloma in a patient with confirmed tuberculosis
(Fig. 69.7C). A second case of tuberculosis was found to
have a highly echogenic pericardial mass consistent with

a calcified granuloma (Fig. 69.7D). The utility of 3D TTE

is never more apparent than when evaluating masses in
the setting of metastatic disease. In a patient with a known
thymoma, a 3.8- 1.5-cm was found to be adherent to the

1460

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 69.10A and B: Live/real time three-dimensional transthoracic echocardiography in a 36-year-old male with a pericardial cyst. (A)
The arrowhead points to a pericardial cyst confirmed by a CT scan of the chest (see Movie clip 69.10A). (B) Cropping of the threedimensional data set demonstrates multiple band-like tissue (arrowhead) within the cyst (see Movie clip 69.10B). Movie clips 69.10C
and D represent two-dimensional images which do not show multiple bands criss-crossing the cyst (arrowhead). (L: Liver). (Other
abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.

Figs 69.11A to C: Two-dimensional and live/real time threedimensional transthoracic echocardiography in a 53-yearold female with constrictive pericarditis. (A) Two-dimensional
study. Arrowheads in the parasternal long-axis view point to
an echogenic left ventricular posterior wall consistent with
calcification (see also Movie clip 69.11A); (B) Represents a threedimensional study using Qlab software analysis package. The
arrow demonstrates a highly echogenic posterior pericardium
consistent with calcification. When this was cropped transversely
using an oblique cropping plane, widespread involvement of the
left ventricular posterior wall with calcification (arrowhead) was
evident. Highly echogenic calcification is visualized anteriorly also.
Top and bottom arrowheads in the left upper quadrant in Movie clip
69.11B point to anterior and posterior calcification, respectively.
The patient underwent pericardiectomy. (AO: Aorta; MV: Mitral
valve). (Other abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.

Chapter 69: Three-Dimensional Echocardiographic Assessment in Pericardial Disorders

visceral pericardium. 3D TTE later demonstrated that the

mass was actually much larger, measuring 9.2 6.3 cm
(Fig. 69.8). Moreover, the lesion was both solid and
inhomogeneous, consistent with tumor. These dimensions
correlated closely with the pathological specimen at the
time of surgery.9

CONCLUSION
Pericardial diseases vary widely in their nature and
consequences. Although 2D TTE is the imaging modality of
choice to evaluate pericardial conditions, its planar nature
can lead to incomplete visualization of the pericardium.
3D TTE has shown benefit in both the diagnosis of
pericardial disease and guiding therapeutic intervention.
Including a 3D data set should become part of the routine
echocardiographic evaluation of pericardial disease where
the diagnosis is in question or if intervention is required.

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and cardiac function. Prog Cardiovasc Dis. 1979;22:
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4. Glantz SA, Misbach GA, Moores WY, et al. The pericardium
substantially affects the left ventricular diastolic pressurevolume relationship in the dog. Circ Res. 1978;42:43341.
5. Maruyama Y, Ashikawa K, Isoyama S, et al. Mechanical
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6. Spodick DH. Threshold of pericardial constraint: the

pericardial reserve volume and auxiliary pericardial
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7. Bove AA, Santamore WP. Ventricular interdependence.
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8. Santamore WP, Shaffer T, Papa L. Theoretical model of
ventricular interdependence: pericardial effects. Am J
Physiol. 1990:259.
9. DCruz IA, Khouzam RN, Minderman D. Three-dimensional
echocardiographic appearances of pericardial effusion
with tamponade. Echocardiography. 2007;24(2):1625.
10. Hernandez CM, Singh P, Hage FG, et al. Live/real time three
dimensional transthoracic echocardiographic assessment
of pericardial disease. Echocardiography. 2009;26:125063.
11. Horowitz MS, Schultz CS, Stinson EB, et al. Sensitivity and
specificity of echocardiographic diagnosis of pericardial
effusion. Circulation. 1974;50:23947.
12. Hsu FL, keefe D, Desiderio D, et al. Echocardiographic
and surgical correlation of pericardial effusion in patients
with malignant disease. J Thoracic Cardiovasc Surgery.
1998:121516.
13. Kim SH, Song JM, Jung IH, et al. Initial echocardiographic
characteristics of pericardial effusion determine the
pericardial complications. Int J Cardiol. 2009;136(2):1515.
14. Lee SH, Kim WH, Ko JK. Fibrinous pericardial effusion in
a three-dimensional echocarcardiography. QJM. 2013.
15. Lewinter, M. Pericardial diseases. In: Libby, editor.
Braunwalds Heart Disease. Philadelphia: Saunders; 2008.
16. Nanda CN, Hsiung MC, Miller AP, et al. Live/Real Time 3D
Echocardiography. Wiley-Blackwell, 2010.
17. Vazquez de Prada JA, Jiang L, Handschumacher MD,
et al. Quantification of pericardial effusions by threedimensional echocardiography. J Am College Cardiol.
1994;24(1):2549.
18. Waller BF, Taliercio CP, Howard J, et al. Morphologic
aspects of pericardial heart disease; Part 1. Clin Cardiol.
1992;15(3):2039.
19. Zagol B, Minderman D, Munir A, et al. Effusive constrictive
pericarditis: 2D, 3D echocardiography and MRI imaging.
Echocardiography. 2007;24:111014.

CHAPTER 70
Echocardiographic Assessment of
Cardiac Tumors and Masses
Leon Varjabedian, Jennifer K Lang, Abdallah Kamouh, Steven J Horn, Tuba Kemalolu z, Aylin Sungur, Kruti Jayesh
Mehta, Kunal Bhagatwala, Nidhi M Karia, Maximiliano German Amado Escauela, Robert P Gatewood Jr, Navin C Nanda

Snapshot

Echocardiographic Assessment of Cardiac Tumors and

Masses

Primary Benign Cardiac Tumors

Malignant Primary Cardiac Tumors

ECHOCARDIOGRAPHIC ASSESSMENT
OF CARDIAC TUMORS AND MASSES
Standard two-dimensional (2D) transthoracic echocardiography (TTE) with Doppler is the initial diagnostic
modality of choice in a patient with a cardiac tumor or
mass. Transesophageal echocardiography (TEE) is often
complementary to TTE in the full assessment of patients
with a known or suspected cardiac mass.
A complete echocardiographic evaluation of a cardiac
tumor or mass should include: (a) Characterization of
the shape, dimensions, and volume of the mass (e.g.
round vs lobulated, small vs large).1 The size of an
intracardiac mass has important clinical implications
in predicting embolic events, congestive heart failure,
and death, and as an efficacy assessment after treatment
(anticoagulation, antibiotics, and chemotherapy).2 Nanda
et al. reported that 2D measurements from a transthoracic
or a transesophageal study underestimate the true
maximum diameter of irregularly shaped structures. (b)
Identification of the location of the tumor and type of
attachment to the heart (e.g. pedunculated vs sessile).
(c) Description of the echogenicity (e.g. echolucent vs
echodense, homogenous vs heterogeneous, and presence of calcification). (d) assessment of mobility (e.g.

Secondary Cardiac Tumors

Normal Variants and Other Masses

MICE

Extracardiac Masses

sessile, prolapsing, shimmering). (e) Description of the

relationship to adjacent structures (intra- or extracardiac).
(f ) Identification of route of access to the heart [e.g. primary
vs secondary through the wall, pulmonary veins, inferior
vena cava (IVC)]. (g) Quantification of the hemodynamic
consequences of the mass (e.g. flow or outflow tract
obstruction, valvular stenosis or insufficiency, chamber
filling or ventricular function). (h) Calculation of cardiac
chamber dimensions, left ventricular volumes, and
ejection fraction.
The differential diagnosis of cardiac masses includes
tumors, thrombi, nonbacterial thrombotic endocarditis,
infective endocarditis, or normal variant intracardiac
or extracardiac structures. Imaging features that favor a
diagnosis of tumor are a mobile, pedunculated appearance
and an associated pericardial effusion. Masses that cross
anatomical planes, from myocardium to pericardium or
endocardium, are likely to be tumors. The clinical setting
and associated echocardiographic findings are also crucial
in guiding the diagnosis of a cardiac mass. For example,
left atrial thrombi are associated with mitral valve
stenosis, enlarged left atrium, and atrial fibrillation, while
ventricular thrombi are associated with cardiomyopathies
or regional wall-motion abnormalities. Right atrial (RA)
thrombi are seen in the setting of indwelling catheters or

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

pacemaker wires. Nonbacterial thrombotic endocarditis

is found in patients with malignancy or systemic lupus
erythematosus.
The sensitivities of transthoracic and Transesophageal
echocardiogram for detection of a cardiac mass are
93% and 97%, respectively.3 In general, the sensitivity
of both the TTE and TEE is highest for endocardial
lesions because the mass is easily distinguished from the
echolucent chamber, while the sensitivity is slightly lower
for intramyocardial lesions and lowest for pericardial
tumors. TTE offers a superior acoustic window for the left
ventricle (LV) and, therefore, is more sensitive in detecting
a left ventricular tumor. On the other hand, TEE has the
advantage of providing better resolution for valvular and
posterior structures that are distant from the anterior chest
wall, such as the left and right atria, superior vena cava
(SVC), and the descending thoracic aorta. Because of its
superior image resolution and transesophageal approach,
intraoperative TEE has proven extremely useful to the
surgeon for the guidance of tumor resection, particularly
in cardiac sarcoma. Increasingly, TEE is also used to aid
cardiac biopsy and guide surgical intervention, helping to
ensure that there is no residual tumor and that the repaired
structures are free of defects.
A concerning limitation of assessing cardiac masses
with 2D echocardiography (2DE) is the possibility of
actually missing the mass during the evaluation due
to complex geometric shapes. This can sometimes be
overcome by employing nonstandard views. Threedimensional echocardiography (3DE) has an important
advantage over 2DE in its ability to show 3D structures
rather than the need to conceptualize these complex
shapes from multiple cross-sectional 2D images.4
3DE has an additional advantage in evaluating
cardiac tumors because of its ability to locate the precise
attachment of a mass to the myocardium, to section the
mass and view it from any plane, to calculate the volume of
a mass and follow this volume over time, and to accurately
define the spatial relationship of the mass to other
structures.2,5,6
Images obtained by 3DE can be sectioned and viewed
from any angle, providing the examiner a more detailed
assessment of the mass even from within. Certain masses
have distinctive and characteristic appearances that can
aid in making the diagnosis. For example, fibromas and
lipomas typically are dense, very bright homogenous
masses, which relate to the presence of fibrous
tissue. Myxomas demonstrate localized areas of large
echolucencies consistent with necrosis or hemorrhage

1463

as well as very bright reflective echodense areas with or

without acoustic shadowing due to calcifications pointing
to their chronicity.7 Hemangiomas are highly vascular
tumors that demonstrate more extensive and closely
packed vascular (echolucent) areas that extend all the
way to the periphery with little solid tissue as compared
to myxomas.8 High-grade sarcomas may demonstrate
areas of necrosis with dilated vasculature (echolucencies)
surrounded by dense hyperechoic band-like tissue
consistent with fibrosis, thus giving the appearance of a
doughnut.9
On 3DE, papillary fibroelastomas are characterized
by a central echodensity that corresponds to their
fibrocollagenous core and finger-like projections, which
represent multiple fronds.10 The stalk by which this tumor
attaches to the valve can also be well seen. They may be
difficult to differentiate from Lambls excrescences, which
are also attached to the tip of the valve leaflets. Lambls
excrescences, however, usually present as multiple strands
on multiple valves and tend not to be discreet rounded
lesions.
When TTE and TEE cannot provide definitive answers
regarding the presence or absence of vegetations, a
three-dimensional transthoracic echocardiography (3D
TTE) might prove useful.11 3DE is particularly helpful
in visualizing all three leaflets of the tricuspid valve, the
right atrium, the right ventricle (RV), and any indwelling
catheters to assess for the presence of vegetations.
Three acquisition modes are used with real time 3D
echocardiography (RT3DE) in the evaluation of cardiac
tumors: (a) full volume, (b) live 3D, and (c) 3D zoom (a
smaller, magnified pyramidal data at a higher resolution).12
Full-volume acquisitions can be obtained from the
parasternal, apical four-chamber, apical two-chamber,
and subcostal views. However, the availability of more
data in these large pyramids of information comes at
the expense of lower image resolution. Hence, imaging
with narrow angles (live 3D) is recommended if highresolution images of the cardiac mass are desired. Fullvolume acquisition allows the echocardiographer to slice
and crop the heart in as many ways as required to obtain a
comprehensive tomographic evaluation of the mass. Fullvolume and live 3D acquisitions in the bicaval view are
useful in characterizing masses in the SVC, IVC, interatrial
septum, and right atrium.1
RT3DE provides a more comprehensive assessment of
the interior structure of the mass that correlates better with
pathological findings (necrosis, hemorrhage, cystic areas,
or fibrotic bands).9,13 RT3DE can also enhance the ability of

1464

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

the echocardiographer to detect associated abnormalities

and conditions that predispose to the development of a
mass, such as an LV apical aneurysm or rheumatic mitral
valve disease.14,15 It helps to characterize masses in the
right atrium, where the differential diagnosis includes
a normal structure (prominent IVC ridge or a crista
terminalis), embryonic remnants (prominent Eustachian
valve or a Chiari network), thrombus, or a tumor arising
from the IVC.16,17 Therefore, full assessment of patients
with a cardiac mass or tumor requires a comprehensive
TTE that may need to be supplemented with TEE and or
3DE as well as cardiac computed tomography (CT) and
magnetic resonance imaging (MRI).
3D TTE can assess the size of cardiac masses and
describe the complex anatomy of the heart.18 If a cardiac
lesion is identified, chest CT with contrast enhancement
and cardiac magnetic resonance imaging (CMR) with
contrast are superior modalities for characterization
of the lesions and delineation of the extent of tumor
involvement. They can also help exclude the possibility
of direct cardiac extension of a tumor that originates
from adjacent mediastinal structures. CT and CMR are
particularly good at depicting the pericardium and great
vessels and evaluating the extent of disease, and CT
can also detect calcification, which is important in the
differential diagnosis.19
Echocardiographic contrast agents can also help
correctly classify intracardiac masses, differentiating
tumors from thrombi.20 Tissue characterization of the
mass with perfusion assessment can differentiate between
a contrast hyperenhanced (highly vascular or malignant
tumors), contrast hypoenhanced (with poor vascularity
such as myxomas), or with no enhancement (such as
avascular thrombi). Use of myocardial contrast has been
recommended by American Society of Echocardiography
Consensus statement to characterize masses.21 Other
methods including MRI can be used as complementary
techniques to evaluate intracardiac tumors.

majority of the remainder originating in the right atrium.22

Other less common locations are the ventricles (RV more
than LV), valves [usually tricuspid valve (Figs 70.2A and B;
Movie clip 70.2) more than mitral valve], and IVC.23,24
On gross examination, myxomas have a soft, gelatinous
appearance with some lobulation.25 They may contain
areas of hemorrhage (Figs 70.3 to 70.5; Movie clip 70.3)5
and calcification (Figs 70.6A to C) and generally range
between 5 and 6 cm in dimension. Figure 70.6D explains
why, unlike 3DE, the size of a cardiac mass including
myxomas can be frequently underestimated by twodimensional TTE as well as multiplanar two-dimensional
TEE.
The classic triad of symptoms relates to the obstructive,
embolic, and constitutional effects of the tumor.26,27
However, myxomas are often asymptomatic or associated
with no specific symptoms or clinical findings (e.g.
incidental findings of a murmur on physical examination).
Myxomas have female gender preponderance. No significant difference exists between male and female patients
age (32 22.39 years, 43.75 18.73 years, P = 0.1692).28
The most common origin of atrial myxoma is the
interatrial septum (Figs 70.7A to F); Movie clips 70.7A and B)
near the fossa ovalis, accounting for 85% of the cases.29
Other less common anatomical origins within the atria in
descending order of frequency are posterior and anterior
walls and the atrial appendage.30,31 Atrial myxoma tends to
be larger in size (Figs 70.8A and B),32 thereby obstructing

PRIMARY BENIGN CARDIAC TUMORS

Cardiac Myxoma
Cardiac tumors are rare with an estimated incidence
of 1/100,000 per year. Albeit myxoma being the most
frequently encountered benign cardiac tumor. Myxomas
commonly arise on the endocardial surface of all cardiac
chambers and rarely the heart valves. Seventy-five percent
of myxomas occur in the left atrium (Fig. 70.1), with the

Fig. 70.1: Transthoracic two-dimensional (2D) echocardiogram

(apical four-chamber view) of a patient with a 5.0 5.6 cm
spherical mass seen attached to the posterior roof of the left
atrium representative of an atrial myxoma. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Source: Dr Sachin Wadhawan, Buffalo Heart Group, Buffalo NY.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1465

Figs 70.2A and B: Transesophageal echocardiogram of a patient with a tricuspid valve myxoma. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle) (Movie clip 70.2.).

Figs 70.3A to C

1466

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.3A to G: Live/real time three-dimensional (3D) transthoracic echocardiographic assessment of left atrial myxoma. (A
to C) Frontal plane sections taken at three different sequential
levels in the 3D data set demonstrate echolucencies (arrowhead)
within the tumor consistent with hemorrhage, which is largest in
the middle section (B); (D) Transverse plane sections of the tumor
viewed en face, also demonstrating hemorrhage (arrowhead);
(E) Frontal plane, transverse plane, and vertical plane sections of
the tumor showing extensive hemorrhage (arrowhead); (F) The
arrow points to the tumor stalk; (G) Resected specimen showing
a large hemorrhage. Arrows in the Movie clip 70.3 point to large
areas of hemorrhage that correspond with the surgical specimen.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral
valve; RA: Right atrium; RV: Right ventricle) (Movie clip 70.3).
Source: Reproduced with permission from Mehmood F, Nanda
NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography.
2005;22(2):13743.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1467

Figs 70.4A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of left atrial myxoma. (A) Arrowhead
points to a large echolucency consistent with a large hemorrhage. (B) Resected specimen showing a large hemorrhage. (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Mehmood F, Nanda NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography. 2005;22(2):13743.

Figs 70.5A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of left atrial myxoma. (A) Arrowhead
points to a large echolucency, which corresponds closely to the hemorrhage seen in the resected specimen (B). (LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Mehmood F, Nanda NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography. 2005;22(2):13743.

1468

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.6A to D: Two-dimensional (A) and live/real time three-dimensional (3D; B and C) transesophageal echocardiography.
(A) Arrow points to a myxoma in the left atrium with a broad attachment on the atrial septum. The dense areas represent calcification,
while the echolucent areas indicate the presence of hemorrhages. (B and C) The attachment of the tumor is much better delineated by
the 3D technique. (IAS: Interatrial septum; LA: Left atrium; RA: Right atrium); (D) Schematic diagram demonstrating that the maximum
dimension of an object (in this case, a cylinder) can be obtained only if the ultrasound beam cuts through its longest dimension (true
long axis) when using a multiplane probe. However, when the two-dimensional planes (dotted lines) are stacked together to obtain a 3D
image, the object (cylinder), including its long axis, can be viewed completely, even though it is not oriented parallel to the ultrasound
beam as it is rotated from 0 to 180 (Movie clips 70.6A to C).
Source: Reproduced with permission from Nanda NC et al. Incremental value of three-dimensional echocardiography over
transesophageal multiplane two-dimensional echocardiography in qualitative and quantitative assessment of cardiac masses and
defects. Echocardiography. 1995;12:61928.

the valvular orifice. Myxomas originating from heart valves

are uncommon and tend to be small in size.33
Atrial myxoma originating from the fossa ovalis
protrude into the atrial cavity but remain attached to the
septum by a stalk (Figs 70.9 and 70.10; Movie clip 70.9).
This stalk can be short, allowing little motion, or long,
therefore explaining the motion that can be seen on real

time 2DE. Some atrial myxomas can prolapse into the

mitral or tricuspid valve orifice and even into the ventricle
during diastole.
Ventricular myxomas (Figs 70.11A to D; Movie clips
70.11A, C Parts 1 to 3, D and E;34 Figs 70.12A to D; Movie
clips 70.12BD)7 may originate on the ventricular free
wall or interventricular septum and may be sessile or

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1469

Figs 70.7A to F: Transesophageal echocardiogram of a patient with a large left atrial septal myxoma (A to F) causing significant mitral
regurgitation (E and F). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clips 70.7A and B).

1470

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.8A and B: Two-dimensional transesophageal echocardiography in a patient with a large biatrial myxoma presenting for
operation. (A) Arrowheads point to areas of calcification within the tumor. (B) Arrowheads point to areas of hemorrhage within the tumor.
(AS: Atrial septum; AV: Aortic valve; H: Hemorrhage; LA: Left atrium; LVO: Left ventricular outflow tract; M1: Myxoma in right atrium; M2:
Myxoma in left atrium; RA: Right atrium; RVO: Right ventricular outflow tract).
Source: Reproduced with permission from Srivastava R, Hsiung MC, Fadel A, Nanda NC. Transesophageal echocardiographic
demonstration of biatrial myxoma. Echocardiography. 2004;21(2):1878.

Figs 70.9A and B: (A) Parasternal long-axis and (B) apical four-chamber views demonstrating a large right atrial myxoma attached
to the atrial wall by a stalk prolapsing through the tricuspid valve into the right ventricle. (RA: Right atrium; RV: Right ventricle) (Movie
clip 70.9).

pedunculated.35,36 Valvular myxomas are defined as

a myxoma arising from different parts of the valvular
apparatus (valve leaflets, annulus, commissure, junction
area, or subvalvular apparatus). A mitral valve myxoma, for
example, has variable characteristics by echocardiography:
pedunculated,37 sessile,38 nonpedunculated,39 hetero-

geneous,40 homogeneous echogenic,38 multilobulated

mobile,41,42 well-circumscribed,42 or an irregular adherent
mass.
Cardiac myxomas are generally first diagnosed by TTE,
and are often an unexpected finding. TEE is usually complementary by confirming and further defining the features.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1471

Fig. 70.10: Live/real time three-dimensional transesophageal

echocardiography. Arrow shows a myxoma attached to the middle
portion of the atrial septum viewed from the left side. (AV: Aortic valve;
LA: Left atrium; RV: Right ventricle) (Movie clips 70.10).

C
Figs 70.11A to C

1472

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Despite the individual variability of myxomas, TTE

typically reveals a homogenous echogenic masses arising
within a cardiac chamber or from the valve leaflet with
areas of echolucency correlating with hemorrhage or echo
bright areas correlating with necrosis and calcification
(Fig. 70.13, Movie clip 70.13).
On M-mode, the tumor fills the left atrium in systole.
During diastole, the tumor prolapses into the mitral valve
orifice. Because the tumor partially obstructs the mitral
valve orifice, the mitral E-F slope is decreased, suggesting
a persistent gradient across the valve and impaired left
ventricular filling. The pattern can mimic M-mode pattern
of mitral stenosis except the valves are not thickened.
Left atrial myxomas on 2D echocardiogram generally
appear as mobile, rounded, or ovoid echogenic masses
that lie completely within the atrial cavity during systole
but may prolapse into or through the mitral valve during
diastole (Fig. 70.14). These most commonly arise from
the interatrial septum. Their point of attachment is best
demonstrated in an apical or subcostal four-chamber
view. Large prolapsing myxomas generally interfere with
left ventricular inflow and mitral valve diastolic motion.43
The portion of the tumor protruding into the LV in
diastole is very mobile, and because of this it has a greater
potential to embolize as compared to the remaining of
the tumor (Movie clip 70.13). Myxomas typically have
multiple internal reflective interfaces that give the tumor
a finely speckled appearance and make the body of the
tumor as reflective as its margins.44 Bright echoes can be
due to localized calcifications. Visualization of echo-free
region in a left atrial mass due to areas of hemorrhage
or necrosis may be a useful feature in differentiating

Figs 70.11A to D: Two-dimensional (A and B) and live/real time

three-dimensional (C and D) transesophageal echocardiography
of right ventricular myxoma. (A) The tumor (T) is visualized in the
right ventricular (RV) outflow tract beneath the aortic valve (AV).
(B) Doppler studies. Color Doppler examination shows turbulent
signals (arrow in the upper panel) in RV and pulmonary artery (PA).
Continuous wave Doppler interrogation shows high velocity flow
signals consistent with obstruction. (C) Arrowheads show large
echolucencies consistent with hemorrhages in the tumor (T). Arrow
points to a linear area of calcification. (D) The dotted line shows
the echogenic area of attachment of the tumor viewed en face. It
measured 1.47 1.44 cm, area 1.04 cm2. The aortic (AO) wall
adjacent to the tumor attachment area is also echogenic. As shown
in the movie clips 70.11C (Parts 13), the three-dimensional data set
was cropped from the bottom to the tumor attachment just beneath
the AV. Then, the data set was rotated to fully delineate en face the
echogenic area of tumor attachment. In Movie clips 70.11C Part 3,
the AO wall was further cropped to reveal the vessel lumen and the
mobile tumor tissue (red arrow) at the AO root level. Two other movie
clips 70.11D and E also show tumor attachment (arrows) beneath the
AO root. (RA: Right atrium; TV: Tricuspid valve) (Movie clips 70.11A,
C parts 1 to 3, D and E).
Source: Reproduced with permission from Khairnar P, Hsiung MC,
Mishra S, et al. The ability of live three-dimensional transesophageal
echocardiography to evaluate the attachment site of intracardiac
tumors. Echocardiography. 2011;28(9):10415.

myxoma from thrombus or vegetation.44 Transducer

angulations are a key in recognizing the tumor. Doppler
echocardiography can reveal mitral valve regurgitation
caused by tumor interfering with normal valve closure.
and also demonstrate an increased transvalvular gradient
produced by mitral orifice obstruction.
Myxomas must be differentiated from vegetations,
atrial thrombi, and other tumors or tumor-like structures
(Fig. 70.15).31,45 The characteristic appearance, motion, and
site of origin of most myxomas distinguish them from the
majority of atrial and valvular masses. Myxomas arising
from locations other than the atria tend to have unusual
shapes and be nonprolapsing, which make them more
difficult to diagnose correctly. Valvular vegetations usually
can be differentiated from left atrial or valvular myxomas
by the clinical setting. The presence of valve disruption
is a very strong evidence against a myxoma. A cardiac
mass in association with rheumatic mitral stenosis, atrial
fibrillation, or dilated cardiomyopathy is much more
suggestive for a thrombus than a myxoma. Atrial thrombi
tend to be located more in the atrial appendage compared
to atrial myxoma, which tend to originate from the septum
and occupy the atrial chamber. Thrombi are laminated,
immobile, and have broad base attachment. Myxomas
are usually smooth, symmetrical, and frequently calcified.
Metastatic tumors generally have extension to other
structures (e.g. pulmonary veins in lung malignancies).

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1473

Figs 70.12A to D: Real time two- (A) and three-dimensional (B to D) transthoracic echocardiography in right ventricular myxoma. (A)
The arrowhead points to a large myxoma in the right ventricle (RV) visualized in the apical four-chamber view. Tumor attachment is not
visualized. (B to D) The arrowhead in B points to the large myxoma. The arrowheads in C show attachments of the myxoma to the RV
inferior wall; D shows one of the attachments (arrow) of the tumor to the tricuspid valve using the QLab on the system. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium) (Movie clips 70.12 BD Parts 1 and 2).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.

RA myxomas can be visualized using subcostal and

apical four-chamber views. Prior to the advent of 2DE,
RA myxoma were difficult to diagnose with M-mode
unless when present behind the tricuspid valve only when
protruding to the RV. As with left atrium, RA myxomas
on TTE are globular in shape with defined borders.
Obstruction of the tricuspid valve can alter the leaflet
motion causing tricuspid stenosis or regurgitation. They
may be associated with RA and ventricular dilation, and
cause paradoxical motion of the interventricular septum.
RA myxomas should be differentiated from normal

congenital variants, such as Chiari network or persistent

Eustachian valve, which appear as linear serpiginous
echoes in the right atrium and lack the globular shape
and bulk typical of myxoma.46 RA myxomas should also
be differentiated from foreign bodies including right heart
catheters, and pacemaker wires that can mimic cardiac
masses, particularly when superimposed thrombus is
present.47 Malignant tumors can be differentiated from
myxomas when extension from the IVC is detected.
Right ventricular myxomas originate from the free
wall or the interventricular septum. They appear as a

1474

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 70.13: Two-dimensional transesophageal echocardiography.

A huge myxoma is seen occupying almost the whole of the left
atrium (LA) with a broad attachment to the atrial septum. Highly
reflectile component of the tumor represents calcification. The portion of the tumor protruding into the left ventricle (LV) in diastole
is very mobile and because of this, it has a greater potential to
embolize as compared to the remaining of the tumor. (RA: Right
atrium; RV: Right ventricle) (Movie clip 70.13).

Fig. 70.15: Two-dimensional transthoracic echocardiography.

Fat in the tricuspid valve annulus. Arrow points to a prominent
echodensity in the anterior tricuspid valve annulus indicative of
fibrofatty tissue. This should not be mistaken for a mass lesion.
(RA: Right atrium; RV: Right ventricle) (Movie clip 70.15).

mass that lie within the body of the RV during diastole

and extend toward the right ventricular outflow tract or
prolapse through the pulmonic valve during systole.48
The differential diagnosis may include: thrombus, tumors

Fig. 70.14: Parasternal long-axis view showing a left atrial

myxoma prolapsing into the left ventricle. (Ao: Aorta; LA: Left
atrium; LV: Left ventricle).
Source: Dr Stephen Downing, Erie County Medical Center,
Buffalo, NY.

of other origin, vegetation, prominent moderator band,

and foreign bodies such as SwanGanz catheter and
transvenous pacemaker.
Left ventricular myxomas are extremely rare49 and
must be differentiated from other left ventricular tumors,
thrombi, false tendons, and prominent or calcified
papillary muscles. Left ventricular thrombi are generally
distinguished by associated LV wall akinesis or dyskinesis.
3DTTE may provide additional information in
assessing myxomas in different locations. It has the
capacity to section the mass and view it from different
angles, demonstrating maximum dimension using
multiplane probes or stacked 2D planes (Movie clips
70.11A to F), giving the examiner a more comprehensive
assessment of the mass and possibly additional
information about echolucency (Figs 70.16A to C; Movie
clips 70.16A and BD).5,7,9 The attachment of the tumor
is much better delineated by the 3D technique (see Fig.
70.6B and C). Contrast echo is another useful technique.
With contrast echo, the appearance of hyperenhancement
of a suspected myxoma is supportive evidence.
After diagnosis, surgery should be performed urgently,
in order to prevent complications such as embolic events
or obstruction of the mitral orifice. Follow-up examination,
including echocardiography, should be performed
regularly.50 Recurrence rates reported for cardiac myxomas
are 47% for sporadic cases and 1021% for familial cases.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

Although recurrence rates are high, second recurrences

are rare.51
Follow-up echocardiography is recommended after
surgical tumor resection. Particularly in large size myxoma,
where there is a significant recurrence rate of 5%.

Papillary Fibroelastoma
Papillary fibroelastomas (PFEs) are the second most
common primary cardiac neoplasms, accounting for 5% of
all tumors of the heart52 and 7.9% of benign primary cardiac
tumors. However, they are the most common valvular
tumors.53,54 The true incidence may be underestimated55
because PFEs are often asymptomatic. It is frequently
observed between the fourth and the eighth decade of
life.56 The pathophysiology of this tumor origin is variable.
It can be seen in patients with long-standing heart disease

1475

Figs 70.16A to C: Three-dimensional (3D) echocardiographic

images cropped to show a left atrial myxoma attached to the
interatrial septum (A to C). 3D images demonstrate the finely
speckled appearance typical of myxomas.
Source: Dr Fredrich Albrecht, Suburban Cardiology, Buffalo, NY
(Movie clips 70.16A and B).

suggesting a post-traumatic tumor or as a degenerative

process.55,57 Other etiologic hypothesis include relation to
organized thrombi,58 or a latent development postradiation
exposure.59 Gross pathological specimen resembles a sea
anemone.
Because of the papillary configuration and soft, fragile
nature, PFEs are a potential cause of ostial coronary obstruction, leading to myocardial ischemia or infarction.60,61
The multiple fonds with recesses may also act as a substrate
for fibrin and platelet aggregation, with subsequent
peripheral or central embolization, depending on their
cardiac location.62 The risk of embolization is higher in
PFE compared to myxomas (34% compared to 24%)54
because PFE commonly arise in the high flow and high
pressure systemic outflow tract of the heart (e.g. aortic valve
or LV).63

1476

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

B1

B2

Figs 70.17A and B: (A) Two-dimensional transthoracic echocardiography (parasternal long-axis view) of a patient with a papillary
fibroelastoma presenting as an 0.8 0.8 cm mobile echogenic
mass at the level of the sinotubular junction attached to the aortic surface of one of the aortic valve leaflets. Source: Dr Hashmat
Ashraf, Chief of Cardiac Surgery, Buffalo General Medical Center,
Buffalo, NY; (B) Two-dimensional transesophageal echocardiography showing a papillary fibroelastoma attached to the aortic valve
causing aortic insufficiency (AI) as shown by color Doppler (right
panel). (Ao: Aorta; LA: Left atrium; LV: Left ventricle).
Source: Dr. Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY (Movie clip 70.17). Transesophageal echo movie clip showing a papillary fibroelastoma attached to the
aortic valve.
Source: Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY.

Aortic (Figs 70.17 and 70.18; Movie clips 70.17 and

70.18A and B) and mitral leaflets are the most frequent
sites of origin, representing 6090% of the diagnosed
cases, followed by tricuspid (Figs 70.19 and 70.20)64 and
pulmonary valves [Figs 70.21A to F; Movie clips 70.21B,
C(Parts 1and 2) and E, BE].10,53,56,65 Approximately 77% of
these tumors originate on the valves, and the other 23% in
the endocardial nonvalvular surface.53
Echocardiography remains the modality of choice to
identify and characterize PFEs.66 PFEs normally appear as
mobile, small, pedunculated, and echodense formations.67
The rounded, centrally radiolucent tumor is outlined
with a refractile linear echo.68 They are well demarcated
and homogeneously textured in appearance, having a
speckled interior with stippling near the edges, which
correlates with the papillary projections on the surface of
the tumor (Figs 70.22A and B; Movie clip 70.22; Figs 70.23A
and B; Movie clips 70.23A and B).65 Half of PFEs appear

pedicled65 with a short stalk attached to the valve. Fingerlike projection on the fibroelastoma produce a prominent
fluttering appearance of the tumor surface, which is a
distinguishing feature on real time 2D TTE.
These tumors rarely exceed 1 cm in diameter and are
usually attached by a small pedicle to one of the cardiac
valve leaflet edges. Other less common anatomical sites
for PFE origin include: right atrium, RV, left atrium, LV,
left ventricular outflow tract (LVOT), atrial or ventricular
septum, and coronary ostia.69 Doppler echocardiography
usually demonstrates either minimal or mild regurgitation
but rarely significant regurgitation or stenosis.70,71
TTE has a sensitivity and specificity of 88.9% and 87.8%,
respectively, with an overall accuracy of 88.4% for the detection
of PFE 20 mm. An overall TTE sensitivity of 61.9% is reported
in case of tumor dimension 20 mm.65 A higher sensitivity is
reached with TEE, particularly for smaller PFEs.65 3D TTE has
also been successfully employed in PFE diagnosis.10,72,73

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1477

Figs 70.18A to C: (A) Enlarged two-dimensional (2D) echocardiogram apical four-chamber view; (B) two-dimensional transesophageal echocardiogram (2D TEE), and (C) three-dimensional
transesophageal echocardiogram (3D TEE) showing a papillary
fibroelastoma attached to the chordal apparatus of the mitral valve.
(LA: Left atrium; LV: Left ventricle; RV: Right ventricle). Source:
Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo General
Medical Center, Buffalo, NY (Movie clip 70.18A). 2D echo movie
clip showing a papillary fibroelastoma attached to a chordal
apparatus of the mitral valve.
Source: Dr. Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY (Movie clips 70.18A and
70.18B). 2D TEE movie clip showing a papillary fibroelastoma
attached to a chordal apparatus of the mitral valve. Source: Dr
Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo General Medical
Center, Buffalo, NY.

Fig. 70.19: Transesophageal echocardiogram of a patient with

a papillary fibroelastoma on the atrial surface of the anterior leaflet of the tricuspid valve. The mass presents as a large (1.56
1.2 cm), sessile, heterogeneous, mobile density with shimmering at
the bloodtissue interface. (RA: Right atrium; RV: Right ventricle).
Source: Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY.

Fig. 70.20: Live/real time three-dimensional transthoracic echocardiographic evaluation of tricuspid valve fibroelastoma. The arrow shows a
fibroelastoma attached to the septal leaflet (S) of the tricuspid valve. (AO:
Aorta; LA: Left atrium; RV: Right ventricle) (Movie clip 70.20).
Source: Reproduced with permission from Pothineni KR,
Duncan K, Yelamanchili P, et al. Live/real time three-dimensional
transthoracic echocardiographic assessment of tricuspid valve
pathology: incremental value over the two-dimensional technique.
Echocardiography. 2007;24(5):54152.

1478

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.21A to F: Pulmonary valve fibroelastoma. (A) Two-dimensional transthoracic echocardiography. Arrowhead points to fingerlike projections on the fibroelastoma; (B to E) Live/real time three-dimensional transthoracic echocardiography; (B) Arrowhead points to
fibroelastoma and the arrow to the echogenic stalk attaching it to the pulmonary valve (PV); (C) Cropping and sectioning the tumor and
viewing it en face shows no evidence of echolucency; (D) Arrowhead points to finger-like projections (fronds) on the fibroelastoma; (E)
Ex vivo imaging of the resected fibroelastoma shows a dense central core and multiple finger-like projections (arrowheads); (F) Gross
pathological specimen of fibroelastoma showing multiple frond-like structures resembling a sea anemone (left) and histopathology of the
surgical specimen demonstrating a central core of collagen and elastin covered by a single layer of endothelial cells (right). (AO: Aorta;
AV: Aortic valve; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle; RVO: Right ventricle outflow tract) (Movie clips 70.21B,
70.21C Parts 1 and 2, and 70.21E). (Source: Reproduced with permission from Singh A, Miller AP, Nanda NC, Rajdev S, Mehmood F,
Duncan K. Papillary fibroelastoma of the pulmonary valve: assessment by live/real time three-dimensional transthoracic echocardiography. Echocardiography. 2006;23(10):8803.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1479

Figs 70.22A and B: Aortic valve fibroelastoma. Two-dimensional transesophageal echocardiography. Parasternal long-axis (A) and
short-axis (B) views. Arrowhead in A points to a mobile rounded mass attached to the right coronary cusp (arrow in B). The mass has
prominent, short, discrete projections on the surface, resembling a sea urchin or fronds of a curtain, and an echogenic central area from
collagen deposition. These findings are typical of a fibroelastoma. (AO: Aorta; LA: Left atrium; LAA: Left atrial appendage; RA: Right
atrium; RV: Right ventricle) (Movie clip 70.22).

Figs 70.23A and B: Tricuspid valve fibroelastoma. Two-dimensional transthoracic echocardiography. Right ventricular inflow (A) and
parasternal short-axis; (B) views show an echodensity with small, multiple spicule-like structures typical of a fibroelastoma (arrowhead).
(AV: Aortic valve; RA: Right atrium; RV: Right ventricle) (Movie clips 70.23A and B).

PFEs are easily distinguished from myxomas, by size

and location. Myxomas commonly arise in the left atrium
with a pedicle attached to the region of the fossa ovalis.22
Myxomas are seen less frequently in the right atrium or
ventricles and rarely involve the mitral valve. PFEs may be
differentiated from fibromas, which are nonencapsulated
and highly refractile, ovoid, and usually solitary masses
that occur primarily in children and commonly involve
the LV, interventricular septum, and RV.74,75 PFE may

be difficult to distinguish from valvular myxoma, giant

Lambls excrescences (Figs 70.24A and B; Movie clip
70.24), and valvular vegetations. PFEs usually attach on
the downstream side of the valve, whereas vegetations
occur on the upstream side and are usually associated
with clinical signs of sepsis or symptoms of endocarditis.
Primary surgical excision is the recommended therapy
for symptomatic PFEs, with left heart side location and
with a specific clinical picture suggestive for possible

1480

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.24A and B: Two-dimensional transesophageal echocardiography. Lambls excrescence. Arrowhead points to a Lambls
excrescence on an aortic (AO) valve cusp. (LA: Left atrium; LV: Left ventricle; RV: Right ventricle) (Movie clip 70.24).

embolization.53,65 Asymptomatic patients with large mobile

masses ( 1 cm) have an increased risk of cardiovascular
complications from embolization and sudden cardiac
death and should undergo a curative surgical excision.65
Asymptomatic patients with small nonmobile lesions (< 1
cm) should be closely followed up with echocardiography
until symptoms develop or tumors enlarge and become
mobile.7678 TEE performed before and during surgery can
demonstrate the exact location of the tumor, influence
surgical approach, and guide complete tumor excision.61

Cardiac Fibroma
Benign cardiac fibromas, when diagnosed, are seen
almost exclusively in the pediatric population.79 Adult
cases have been reported but are exceedingly rare.80,81 It is
the second most common primary ventricular neoplasm
after rhabdomyomas. Grossly, these tumors are firm and
nonencapsulated, intramural lesions ranging in size from
3 cm to >10 cm in diameter.22
Clinically, cardiac fibromas may present as shortness
of breath, dizziness, syncope, and heart failure. Symptoms
occur when the tumor increases in size causing obstructive
disease. Cardiac fibromas may also be responsible for
asymptomatic murmurs since childhood, or be an origin
for arrhythmias. Fifty percent are asymptomatic. Cardiac
fibromas can be found incidentally as calcification in
lower mediastinum on thoracic spine radiograph, or on
CT of the chest. They most commonly occur in the RV
(Figs 70.25A to C; Movie clips 70.25AB, CBC)7 and are

usually associated with the interventricular septum. On

occasions, they can occur on the lateral wall or in the apex
of the RV.
Echocardiography helps in confirming the presence of
a fibroma that is found primary within the interventricular
septum or anterior free wall of the RV.82 Central calcification
is frequent. It can obstruct the mitral orifice and produce
significant obstruction to inflow. It can also present as
massive tumor causing right ventricular outflow obstruction
or displace the septum against the free wall of the LV, or shift
the tricuspid valve backward toward the atrium.
Fibromas are difficult to differentiate from rhabdomyomas, as both can present as a solitary, large mass,
particularly if intracavitary. However, their characteristic
location within the interventricular septum83 or within
the ventricular free wall84 often aids in the appropriate
diagnosis.
Cardiac magnetic resonance (CMR) is very helpful in
showing anatomical details, and differentiating primary
cardiac tumors from thrombus.85 The heterogeneity of
the tissue component presents as hypointense T1 signal
with absent early vascular enhancement on perfusion
imaging, and avid delayed enhancement suggesting a
benign etiology and fibrous tissue.86 These tumors require
surveillance imaging due to the potential for recurrence.

Cardiac Rhabdomyoma
Cardiac rhabdomyoma is the most common primary
cardiac tumor in pediatric age group.87,88 This tumor is

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

C
multicentric and can occur in the ventricular free wall and
interventricular septum.8890 It occurs in equal frequency
in the right and left ventricles.
Diagnosis can occur in the prenatal period; however,
the majority are diagnosed in infancy. The incidence of
tuberous sclerosis in patients with cardiac rhabdomyoma
has been reported to be between 30 and 50%.91 Clinically,
rhabdomyoma can lead to life-threatening ventricular
arrhythmia or obstruction of ventricular filling or outflow
with intracavitary extension seen in 50% of patients.
Echocardiography is an ideal tool to diagnose
rhabdomyomas. These tumors tend to appear as multiple
homogenous, well-circumscribed, echogenic ventricular
masses. They may be intracavitary or intramural masses in
RV and LV. They have been described as having a speckled
appearance. A pedunculated rhabdomyoma occupying or
protruding into the ventricular cavity and aortic valve with
significant LVOT obstruction has been reported.92

1481

Figs 70.25A to C: Right ventricular fibroma. Two-dimensional

(A) and live/real time three-dimensional (B and C) transthoracic
echocardiography. (A) Arrowhead points to a single mass in the
right ventricle (RV); (B and C) The arrowhead (B) points to a highly
echogenic mass occupying right ventricle (RV) cavity and outflow
tract. Sectioning the mass and viewing it en face demonstrates two
separate tumors (arrowheads in C). (AO: Aorta; LA: Left atrium;
LV: Left ventricle; RA: Right atrium) (Movie clips 70.25BC Parts
1 to 3).
Source: Reproduced with permission from Reddy VK, Faulkner M,
Bandarupalli N, et al. Incremental value of live/real time three-dimensional transthoracic echocardiography in the assessment of right
ventricular masses. Echocardiography. 2009;26(5):598609.

When outflow obstruction is present, pulsed and

continuous wave Doppler can determine the degree
of outflow gradient and help guide management. The
presence of multiple nodular masses in several chambers
of the heart helps differentiate rhabdomyoma from other
primary cardiac tumors (e.g. myxoma, fibroma, or other
benign tumors).
2D echocardiogram is also a useful method of screening
asymptomatic infants and children with tuberous sclerosis
for the presence of cardiac tumors.91 Surgical intervention
may be required for children if a rhabdomyoma causes
severe clinical symptoms either due to life-threatening
dysrhythmia or outflow obstruction refractory to medical
treatment.93 Spontaneous regression has been reported.94

Cardiac Lipomas
Lipomas of the heart are most frequently located in the
LV (Figs 70.26A to C; Movie clips 70.26ABCAC), RV

1482

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

C
(Figs 70.27A and B; Movie clips 70.27 (Parts 1 to 3),7
and right atrium (Figs 70.28A to C; Movie clip 70.28).22
Clinically, these tumors are silent, although conduction
disturbances have been reported.95
Subepicardial tumors are large, often pedunculated,
whereas subendocardial lipomas are typically sessile.
About 25% are completely intramyocardial.22 Valvular
lipomas are rare, and have been described on the mitral
and tricuspid valves.96
Lipomas are differentiated from left ventricular
myxomas in that lipomas are less mobile and generally
more echodense. These tumors need to be differentiated
from lipomatous hypertrophy of the interatrial septum
(Figs 70.29A and B), which is characterized by accumulation
of adipose tissue in the atrial septum.97 In the subcostal views,
globular thickening of the interatrial septum with central
sparing gives what has been described as a dumbbell
shape to the atrial septum with lipomatous hypertrophy.

Figs 70.26A to C: Left ventricular lipoma. Live/real time threedimensional transthoracic echocardiography. The arrowheads
in A and B point to a highly echogenic mass in the left ventricle
(LV). Further cropping of the three-dimensional data set shows
the presence of two lipomas (arrowheads in C) in the left ventricle.
Arrow in the movie clips 70.26A and B points to the lipoma that
is highly echogenic and shows no echolucencies on cropping.
Movie clip 70.26C shows another patient with a left ventricular
lipoma (arrow) together with the surgically resected specimen.
A few trabeculations are also present adjoining the lipoma in
the left ventricular apex. (RV: Right ventricle). (Movie clips
70.26AB, C).

Valvular lipomas are not easy to differentiate from

other valvular tumors such as myxomas, PFEs or fibromas,
or valvular vegetations. MRI may aid confirming the fatty
nature of the tumor.98

Cardiac Hemangiomas
Hemangiomas are the most common benign vascular
tumors of the heart (Figs 70.30A to D; Movie clip 70.30 B).8
These tumors are usually solitary but may be multiple.99
They may occur anywhere in the heart including the
epicardium, often in association with pericardial effusion.99
They may be either intracavitary or intramural. Intramural
hemangiomas occur most commonly on the right side of
the heart (Figs 70.31A to D; Movie clips 70.31A, B, and D
Parts 12),34 particularly in the interventricular septum.99
Clinical signs depend on anatomical location.
Congestive heart failure occurs with large intracavitary

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1483

Figs 70.27A and B: Right ventricular lipoma. Live/real time three-dimensional transthoracic echocardiography. (A) The arrow points to
a large markedly echogenic lipoma interdigitating and infiltrating the right ventricle (RV) free wall. (B) Surgical specimen. (LA: Left atrium;
LV: Left ventricle) (Movie clips 70.27 Parts 1 to 3).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.

Figs 70.28A to C: Two-dimensional echocardiography showing

a cardiac lipoma attached to the apicoseptal left ventricular wall
(A). CT with (B) and without (C) IV contrast showing a hypodense
LV lipoma. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
Source: Dr Steven Horn, Medical Director Noninvasive Cardiology
Lab, Kalieda Health, Buffalo, NY (Movie clip 70.28).

1484

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.29A and B: Two dimensional transesophageal echocardiography. Lipomatous hypertrophy of the atrial septum. (A) Arrow
points to thickening of atrial septum that spares foramen ovale. This is caused by fatty infiltration of the septum and usually has no
clinical sequelae. (B) Massive lipomatous hypertrophy (arrowheads) affects the entire atrial septum and occupies most of the right
atrium (RA) in another patient. (LA: Left atrium; RA: Right atrium; RPA: Right pulmonary artery; SVC: Superior vena cava). (Courtesy
of Dr Allan Schwadron, Dothan, AL).

tumors restricting filling of right heart chambers.100 The

tumor may simulate infundibular pulmonic stenosis when
they involve the upper portion of the interventricular
septum.97 Recurrent pericardial effusions are frequent
presentation.101,102
2D echocardiographic imaging shows echo-free
areas within the mass, representing vascular channels
(Figs 70.32A and B; Movie clip 70.32)5 and cavernous
lakes. These tumors are generally nonpedunculated,
nonhomogenous, solitary masses. When seen in the
right heart and associated with a pericardial effusion,
they are more suggestive of hemangioma than myxoma,
rhabdomyoma, or other primary intracardiac tumors.
Spontaneous regression of cavernous hemangiomas has
been documented.103

MALIGNANT PRIMARY CARDIAC

TUMORS
Sarcomas
Malignant primary tumors of the heart account for onequarter of all primary cardiac tumors, the vast majority
of which are sarcomas (95%; Fig. 70.33; Movie clips
70.33A and B).7 The most common primary malignant
cardiac neoplasms in the adult are angiosarcomas (33%)
and rhabdomyosarcomas (21%), followed by pericardial

mesotheliomas (16%), fibrosarcomas (11%), primary

cardiac lymphomas (PCLs; 6%), osteosarcomas (4%),
neurogenic sarcomas (2%), leiomyosarcomas (<1%; Figs
70.34A to C; Movie clip 70.34)9, liposarcomas (<1%), and
synovial sarcomas (<1%).104 In addition to identifying the
presence of a cardiac tumor, echocardiography provides
valuable information regarding the anatomical location
(right vs left heart chambers, intracavitary vs intramural),
tumor extension (Figs 70.35A to H; Movie clip 70.35;
valves, arteries, veins, and pericardium), physiological
consequences such as valvular stenosis and regurgitation,
ventricular inflow or outflow obstruction, chamber
obliteration and ventricular function, and associated
findings such as recurrent pericardial effusion. All of these
features taken together help to establish a differential
diagnosis of the type of tumor in question and assess
its hemodynamic effects on cardiac function.105107 3D
TTE has more recently become a helpful adjunct in the
detection and characterization of intracardiac masses,
secondary to its improved imaging over 2DE.108 In addition
to 2D, 3D, and M mode echocardiography, contrast
echocardiographic perfusion imaging has become an
important supplement to help classify cardiac masses.
Compared with the adjacent myocardium, malignant and
vascular tumors, such as sarcomas, hyperenhance with
the use of contrast imaging, whereas stromal tumors and
thrombi hypoenhance.20,109,110

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1485

Figs 70.30A to D: Hemangioma involving the mitral valve. (A) Two-dimensional transthoracic echocardiography. Parasternal long-axis
view. The arrow points to the mass attached to the anterior mitral leaflet (AML), with a few echolucencies within it; (B) Live/real time
three-dimensional transthoracic echocardiography. Apical four-chamber view. The arrow points to a mass attached to the mitral valve.
The presence of multiple echolucencies (arrowheads, inset A) within the mass is consistent with a hemangioma; (C) Two-dimensional
transesophageal echocardiography. Low esophageal modified five-chamber view. The arrow points to the mass on the AML, with a few
scattered echolucencies within it; (D) Histopathology. H&E stain 10x. In addition to the sheet-like areas of epithelioid endothelial cells
with small vascular spaces typical of epithelioid hemangioma (arrows), this lesion also contains larger, more cavernous vascular spaces
(arrowheads). (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle). (Movie clip 70.30B).
Source: Reproduced with permission from Dod HS, Burri MV, Hooda D, et al. Two- and three-dimensional transthoracic and transesophageal echocardiographic findings in epithelioid hemangioma involving the mitral valve. Echocardiography. 2008;25(4):4435.

Angiosarcoma is the most frequent type of sarcoma

in the adult population and the most common primary
malignant cardiac tumor. It occurs more frequently in
middle-aged males,104 is predominately right-sided,111
and carries a dismal prognosis.107 Typically, the tumor
completely replaces the RA wall, fills the entire cardiac
chamber, and invades surrounding structures including
the tricuspid valve, free wall of the LV, interventricular
septum, right coronary artery, and great veins.112,113 On

pathological examination, cardiac angiosarcoma looks

like a lobulated mass with a necrotic and hemorrhagic
appearance and is composed of malignant cells forming
vascular channels.114 On echocardiogram it often appears
as a bulky, mobile, heterogeneous broad-based RA
mass near the IVC, extending intracavitary and into the
pericardium, occasionally invading the caval veins or
tricuspid valve.115 Because of its tendency to invade valves,
sarcomas of the tricuspid and mitral valves may mimic

1486

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.31A to D: Right ventricular hemangioma. (A) Two-dimensional transesophageal echocardiography. The tumor (T) is located
in right ventricle (RV); (B to D) Live/real-time three-dimensional transesophageal echocardiography; (B) The tumor (arrow) is located
in RV; (C) Arrow points to multiple small echolucencies involving the whole tumor including the periphery, typical of hemangioma;
(D) The three-dimensional data set was cropped from bottom and rotated to view the echogenic area of tumor attachment (dotted lines).
It measured 2.76 1.40 cm, area 2.01 cm2. (AV: Aortic valve; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium; TV: Tricuspid
valve) (Movie clips 70.31A, B, D Parts 1 and 2).
Source: Reproduced with permission from Khairnar P, Hsiung MC, Mishra S, et al. The ability of live three-dimensional transesophageal
echocardiography to evaluate the attachment site of intracardiac tumors. Echocardiography. 2011;28(9):10415.

cardiac angiosarcomas. Angiosarcoma that replaces

the atrial wall can also lead to RA rupture, as diagnosed
by color Doppler echocardiography and contrast echocardiography.116 On CT imaging, angiosarcoma can be
identified as a low-attenuation RA mass, which may be
irregular or nodular. On MRI, it shows moderate intensity
on T1- and T2-weighted images with local nodular areas of
increased signal intensity interspersed to give a cauliflowerlike appearance.117 Clinical presentation is usually
nonspecific; however, recurrent pericardial effusions are
a common manifestation and should raise a high index

of suspicion.118 There may also be a possible etiological

correlation between the HIV infection or Kaposi sarcoma
and cardiac angiosarcomas.119 Angiosarcoma is usually
associated with complications including pulmonary
embolism, right heart failure, cardiac tamponade, SVC
syndrome, cardiac chamber and coronary artery fistulas,
or cardiac perforation.
Rhabdomyosarcoma is the second most common
primary cardiac sarcoma, but the most common cardiac
tumor in children and adolescents. This tumor arises
from embryonic cells in the septum, which may explain

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1487

Figs 70.32A and B: Left atrial hemangioma. Live/real time threedimensional transthoracic echocardiography. (A) Arrowheads
(arrow in Movie clip 70.32) point to two of the large number of
closely packed echolucencies in the tumor mass with sparse solid
tissue; (B) Resected specimen showing multiple vascular areas.
(LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA: Right
atrium; RV: Right ventricle) (Movie clip 70.32).
Source: Reproduced with permission from Mehmood F, Nanda
NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography.
2005;22(2):13743.

Fig. 70.33: Right ventricular sarcoma. Live/real time threedimensional transthoracic echocardiography. Arrow points to a
mass in the right ventricle (RV) showing large echolucencies
surrounded by echogenic band-like tissue giving a doughnut
appearance. Movie clip 70.33B shows, in a different patient, renal cell
carcinoma invading the inferior vena cava (IVC) and the proximal right
atrium (RA). Cut section demonstrates a solid tumor with no evidence
of necrosis. Surgically resected specimen is also shown. (LA: Left
atrium; LV: Left ventricle) (Movie clips 70.33A and B).
Source: Reproduced with permission from Reddy VK, Faulkner M,
Bandarupalli N, et al. Incremental value of live/real time three-dimensional transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.

1488

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

its high prevalence in children younger than 1 year.

Rhabdomyosarcomas most commonly arise from either
the left or right ventricular wall and frequently invade
cardiac valves or interfere with valve function secondary
to their mass effect.120 On echocardiogram, it appears as an
echodense, multilobular pedunculated mass with irregular
borders. While it is most commonly seen originating from
the ventricles, it has also been described on 2D echo as a
lobulated mobile mass inside the left atrium projecting
into the left ventricle during diastole and provoking
turbulence.121 Additional case reports have also described
an echodense mass that originates from the pulmonary
valve and obliterates the right ventricular outflow tract
with associated high peak transpulmonary gradients.122
On contrast CT, it shows as a hypodense mass, while on
MRI it appears isointense to myocardium on T1-weighted
images and demonstrates more or less homogeneous

B
Figs 70.34A to C: Left atrial leiomyosarcoma. (A) Live/real time
three-dimensional transthoracic echocardiography. The arrows
point to a doughnut-like appearance of the tumor mass located
in the left atrium; (B) Intermediate and high-power microscopic
appearances of the leiomyosarcoma. The tumor is composed
of pleomorphic spindle cells arranged in a fascicular pattern.
Abnormal mitotic activity (inset) was easily identified; (C) Lowpower microscopic appearance of necrotic tumor. The periphery
of a necrotic area within the tumor showed acellular stroma
and dilated/ectatic blood vessels (arrows) consistent with the
echocardiographic findings. (AO: Aorta; LA: Left atrium; LV: Left
ventricle) (Movie clip 70.34).
Source: Reproduced with permission from Suwanjutah T, Singh H,
Plaisance BR, Hameed O, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic findings in primary left atrial
leiomyosarcoma. Echocardiography. 2008;25(3):3379.

enhancement after contrast, although there may be areas

of low signal intensity due to central necrosis within the
tumor.123,124 Systemic metastases typically develop in the
lung, bone, and brain. In most patients, survival time is
limited to <12 months.
Fibrosarcomas are primarily fibroblastic in origin
and appear as firm, graywhite nodules with areas of
hemorrhage and necrosis. They can occur on the right and
left sides of the heart and invade the cardiac chambers and
the pericardium. Echocardiographically, they are most
often seen as a mobile, heterogeneous left atrial mass.
Because most occur on the left side of the heart, associated
signs and symptoms are related to pulmonary congestion,
mitral stenosis, and pulmonary vein obstruction. Similar
to angiosarcomas and rhabdomyosarcomas, survival is
poor.105

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

Figs 70.35A to F

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.35A to H: Aortic leiomyosarcoma in a 43-year-old male. (A to F) A mass (arrowheads) in the descending thoracic aorta (DA).
Color Doppler examination shows prominent flow signals in the unobstructed portion of the aorta (AO). This makes thrombus unlikely,
because associated spontaneous contrast echoes caused by a low-flow state usually are present. Also, no dissection flap is identified.
The arrows in A to C and E to H show a large echogenic mass outside the AO, consistent with hematoma; (G and H) A hematoma
(arrow, arrowheads) is seen extending on both sides of the descending aorta (DA, AO), even where the tumor mass is not present. At
surgery, the mass was found to be a leiomyosarcoma that involved the aortic wall, resulting in perforation that caused the hematoma
(Movie clip 70.35).
Source: Reproduced with permission from Suwanjutah T, Singh H, Plaisance BR, Hameed O, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic findings in primary left atrial leiomyosarcoma. Echocardiography. 2008;25(3):3379.

Primary Cardiac Lymphoma

Primary cardiac lymphoma (PCL) is defined as a nonHodgkin lymphoma (NHL) exclusively located in the heart
or the pericardium.125127 The accepted definition of PCL
also includes a lymphoma presenting as cardiac disease,
especially if the bulk of the tumor is intrapericardial.128
Cardiac 99NHL is commonly associated with HIV
infection.129131
The most typical locations for cardiac lymphomas are
the right atrium and RV, and less often the left atrium and
LV.132,133 PCL is rare, comprising only 0.5% of all lymphomas
and 1.32% of all heart tumors.127,128 More than 80% of PCLs
are diffuse B-cell NHLs, the majority of which are a large
cell type and less often T-cell lymphomas.
The two most common manifestations are pericardial
effusion and heart failure.134 SVC syndrome, stroke,
pulmonary hypertension, aortic valvular obstruction,127
and myocardial infarction have also been reported.128
Atrioventricular and bundle branch blocks, low
voltages, and negative T-waves have been reported on
electrocardiography (ECG).135
Echocardiography is a good noninvasive diagnostic
tool to detect pericardial effusion and the presence of a
lymphoma mass (Figs 70.36A and B). Cardiac lymphoma

can appear as a frank multilobulated mass mostly located

in the right atrium,136 occasionally in the RV, and rarely in
the left cavities.137 Other echocardiographic findings seen
in clinical cases were limited thickening of the pericardium
or the cardiac wall, decrease of the cardiac kinetics with or
without pericardial effusion, and cardiac tamponade.138
ECG-gated MRI helps determine tissue characteristics,
location, mobility, and its relationship with surrounding
tissues.139

Cardiac Plasmacytoma
Primary plasmacytoma of the heart is extremely rare.
These tumors, when present in the heart, are usually
secondary to metastatic disease or direct extension from
the mediastinum. The right atrium is the most common
site of cardiac plasmacytoma.140 They also can originate in
the atrioventricular groove with a short stalk perforating
into the right atrium near the tricuspid valve annulus.
Heart failure is the most common clinical presentation.
Plasma immunoelectrophoresis detection of monoclonal
immunoglobulin G paraproteinemia and k light chains
increases the probability for this disease entity. Bence
Jones proteinuria may or may not be present. On gross

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1491

Figs 70.36A and B: Two-dimensional transesophageal echocardiography. Hodgkin lymphoma involving the atrial septum. (A) Fourchamber view demonstrating thickening of the atrial septum (arrowhead) produced by the tumor. Note sparing of the fossa ovalis;
(B) Arrowhead shows a large circumscribed echolucent area in the atrial septum containing multiple, bright echo densities (coin lesion).
(LA: Left atrium; MV: Mitral valve; RA: Right atrium; SVC: Superior vena cava; TV: Tricuspid valve).
Source: Reproduced with permission from Miller A, Mukhtar O, et al. Two- and three-dimensional TEE differentiation of lymphoma
involving the atrial septum from lipomatous hypertrophy. Echocardiography. 2001;18(3):2059.

examination, these tumors usually present as a large,

friable mass. Definitive diagnostic confirmation is made
through postresection histological and histochemical
examination demonstrating the classic features of a
plasmacytoma. A standard 2D echocardiogram reveals the
presence of a large mass in the right atrium (Figs 70.37A
to C; Movie clips 70.37A to C), often producing a subtotal
obstruction of the diastolic inflow through the tricuspid
valve.141 Treatment is usually palliative and consists of
resection followed by chemotherapy.

Hydatid Cyst
Cardiac hydatid cyst is a rare parasitic disease caused by
larvae of Echinococcus granulosus, which is still endemic
in many sheep-raising countries. Although the LV is mostly
involved in this condition, all cardiac chambers and rarely
the pericardium can be affected (Figs 70.38A to Z; Movie
clips 70.38A and B; Figs 70.39A to F). Patients can be
asymptomatic, have nonspecific symptoms such as chest
pain, shortness of breath, or palpitations or present as an
acute complication of cyst rupture.142 Echocardiogram
usually shows a unique finding of multiple cysts inside the
cardiac chambers or the pericardium.

Pericardial Mesotheliomas
Primary pericardial mesothelioma is a very rare tumor,
with a reported prevalence of <0.002%.143 In addition to

asbestos exposure, other suspected risk factors include

radiation exposure, infection (including SV40 and TB),
dietary factors, and recurrent serosal inflammation.144
Clinical manifestations include constrictive pericarditis,
pericardial effusion, cardiac tamponade, and heart
failure. Diagnosis can be challenging and often requires
a multimodal imaging approach including echocardiography, MRI, CT, and FDG-PET scans. TTE can show
a pericardial effusion comprising a partially organized
fibrinous structure and a thickened pericardium. 2DE can
be misleading; however, as it can suggest the presence of
an abnormal amount of pericardial fluid with a surgical
plane for resection. As later pathological findings will
reveal, the low-density echogenic region surrounding
the heart will be tumor rather than fluid.145 FDG-PET/CT
scan demonstrates an intrapericardial accumulation of
the tracer, seen with local infection or tumor.146 MRI is
emerging as the best modality for demonstrating the extent
and nature of the constrictive process, and will show a softtissue mass within the pericardial space that is enhanced
with gadolinium DTPA.147 Pericardial fluid cytology
is often negative for malignant cells and a diagnosis
usually requires tissue for histological evaluation.143,144
The prognosis of primary pericardial mesothelioma is
extremely poor due to its late presentation, inability for
complete tumor eradication by surgery, and poor response
to radiotherapy and chemotherapy.

1492

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.37A to C: Two-dimensional (2D) transesophageal

echocardiogram showing a periaortic right atrial mass representative of plasmacytoma (A to C). (A: Aorta; AV: Aortic valve; LAL Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
(Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
Buffalo, NY (Movie clip 70.37A). 2D echocardiogram showing a
periaortic right atrial plasmacytoma. Source: Dr Edward Spangenthal, Roswell Park Cancer Institute, Buffalo, NY. Movie clip
70.37B.Transesophageal echocardiogram showing a periaortic
right atrial plasmacytoma seen anterior to the aortic valve. Source:
Dr Edward Spangenthal, Roswell Park Cancer Institute, Buffalo,
NY (Movie clip 70.37C). Transesophageal echocardiogram showing a pariaortic right atrial plasmacytoma seen on the right atrial
side of the interatrial septum.
Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
Buffalo, NY.

Secondary Cardiac Tumors

While primary cardiac tumors are very rare, secondary or
metastatic heart tumors are the most common tumors of
the heart. In clinical practice, secondary cardiac tumors
are rarely seen. The frequency of cardiac metastases
is generally underestimatedvarying from series to
series, cardiac metastases were found in up to 25% of
postmortem patients who had died of malignancies.148152
Because of absent or nonspecific signs and symptoms of
cardiac involvement, most metastases are diagnosed post
mortem. Cardiac metastases mostly appear in patients
with disseminated tumor disease; solitary metastases to
the heart are very rare. Despite their frequency, metastatic
heart tumors only rarely gain clinical attention. Signs
of cardiac involvement are often overlooked, since the
symptoms of disseminated tumor disease prevail. Like

primary tumors of the heart, metastases may imitate

valvular heart disease or cause cardiac failure, ventricular
or supraventricular heart rhythm disturbances, con
duction defects, syncope, embolism, or, quite often,
pericardial effusion (Fig. 70.40). Not infrequently, cardiac
tumor invasion contributes to the mechanism of death in
affected patients.148150
In principle, every malignant tumor can metastasize
to the heart. The most common tumors with cardiac
metastatic potential are carcinomas of the breast, the
lung (see Fig. 70.40), the esophagus, leukemia, malignant
melanoma (Figs 70.41 and 70.42), and lymphoma (Fig.
70.43).153155 Of tumors that metastasize to the heart,
melanoma has the highest rate of cardiac metastasis,
although lung and breast carcinomas are more commonly
encountered because of the higher prevalence of these
cancers.153155

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

Figs 70.38A to F

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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.38G to L

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

Figs 70.38M to R

1495

1496

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.38S to X

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1497

Figs 70.38A to Z: Live/real time three-dimensional transthoracic echocardiography. Hydatid cyst. Arrow in the apical four-chamber (A)
and short-axis (B) views in a young Asian Indian policeman shows a large mass in the left ventricle (LV), which represents a hydatid cyst
that had become smaller since the previous study done a few years ago. This collapse of the hydatid cyst could have been spontaneous or may be related to antihelminthic albendazole therapy, which had been given to this patient. (LA: Left atrium; RV: Right ventricle)
(Movie clips 70.38A and B). (C to Z) Live three-dimensional transthoracic echocardiographic assessment of hydatid cyst in the left ventricle in another patient a 37-year-old Asian Indian male. (C) The arrow points to the large hydatid cyst in the left ventricle (LV) seen on
the two-dimensional transthoracic echocardiogram. (B to I) Sequential transverse plane (TP) sections taken at various levels (# 15) of
the three-dimensional data set. The cyst is not visualized at the level of the body of the mitral valve(C, *), but its basal tip (arrow) comes
into view when the section is taken further down at the mitral valve leaflet tips (D). In H and I, the large arrowhead points to a tertiary or
grand-daughter cyst located within the secondary or daughter cyst. The small arrowhead in H shows a small greatgrand-daughter cyst
budding from the tertiary cyst. The arrow points to the parent hydatid cyst. (J to M) Longitudinal plane (LP) sections (# 1 and 2) through
the hydatid cyst (arrow). (N) Combined TP and LP sections through the hydatid cyst. (O to T) Only FP (O), combined FP and TP (P and
Q), combined FP and LP (R), and combined FP, TP, and LP (S and T) sections through the hydatid cyst. (U to Y) Oblique plane (OP)
sections through the hydatid cyst. In X, the OP section is rotated to view the cyst en face. A tertiary or grand-daughter cyst is shown
attached to the secondary or daughter cyst by a stalk (small double arrowheads). (Z) Schematic of hydatid cyst. (LA: Left atrium; RA:
Right atrium; RV: Right ventricle; S: Stalk). (Movie clips 70.38CZ).
(Source: Reproduced with permission from Sinha A, Nanda NC, Panwar R, et al. Live three-dimensional transthoracic echocardiographic assessment of left ventricular hydatid cyst. Echocardiography. 2004;21(8):699705.

A
Figs 70.39A and B

1498

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.39A to F: (A) Transthoracic echocardiography shows a large pericardial hydatid cyst (MS) compressing the left ventricular (LV)
lateral wall. It measures approximately 8 cm in diameter and appears to contain layered solid tissue. The patient was an 18-year-old
Asian Indian male who had emigrated to South Alabama at the age of 5 from India. He presented with a recent syncopal episode and the
electrocardiography (ECG) showed deeply inverted T-waves in anterolateral leads consistent with myocardial ischemia. Initially, the twodimensional echocardiogram appeared to show poor LV function but it soon became apparent that this was artifactual and resulted from
the ultrasonic beam passing directly from the aortic root into the cyst; (B and C) The arrows point to multiple intramyocardial coronary
vessels imaged within the compressed LV free wall. These were visualized using a high-resolution color Doppler system. Note that the
Nyquist limit is set at a very low velocity of 0.16 m/s; (D) The arrows demonstrate a high velocity of 1.2 m/s obtained by color Dopplerguided pulsed Doppler interrogation of an intramyocardial coronary vessel (normal velocity < 0.6 m/s). Thus, there was compression
of the LV lateral wall by the cyst that served to explain the ECG changes. A coronary arteriogram also showed systolic emptying of the
first marginal branch of the circumflex artery. The cyst was surgically resected and the patient is doing well; (E) Left: Three-dimensional
reconstruction demonstrates membrane-like solid tissue in the cyst. Right: Three-dimensional reconstruction of intramyocardial coronary vessels (arrows) in the compressed LV wall; (F) Left upper panel: Hydatid cyst wall and surrounding soft tissue. Left lower panel:
Close-up of hydatid scolex with double rows of hooklets (100x). Right panel: Laminated membrane. (C: Pericardial cyst; LA: Left atrium;
RA: Right atrium; RV: Right ventricle) (Movie clip 70.39).
Source: Reproduced with kind permission from Kluwer Academic Publishers, Advances in Echo Imaging using Contrast Enhancement, 2nd edition, 1997, Ch. 41 Echocardiographic detection of intramyocardial coronary obstruction produced by pericardial hydatid
CPT, Fig. 2B. Jamil F, Nanda NC, et al. Echocardiographic Detection of Intramyocardial Coronary Obstruction Produced by Pericardial
Hydatid Cyst. Echocardiography. 1997;14(5):45960.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

Fig. 70.40: Apical four-chamber view showing a left ventricular

intracardiac metastasis of a ureteral sarcomatoid tumor (blue).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle).
Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
Buffalo, NY.

1499

Fig. 70.41: Apical four-chamber view showing metastatic melanoma with metastasis to the papillary muscle. (LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).

Figs 70.42A and B: Two-dimensional transesophageal echocardiography. Metastatic melanoma involving the right ventricle. (A and
B) A huge tumor mass (M, T) is noted in the right ventricle (RV) reaching up to the pulmonary valve. (AO: Aorta; LV: Left ventricle; PA:
Pulmonary artery).
Source: Reproduced with permission from Nanda NC, Domanski MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2007:519.

2DE plays an essential role in the detection of cardiac

metastases and their complications (Figs 70.44 and 70.40).
It can generally be used to determine the location, size,
shape, attachment, and mobility of the cardiac tumor.
It has a good sensitivity to detect intracardiac tumors,
but a lower detection rate for pericardial or paracardial

lesions.155,156 In cases of peri- or paracardial lesions, the

transesophageal approach is superior to the transthoracic
approach (see Figs 70.42A and B).157,158
Tumors can spread to the heart through four
alternative pathways: (a) by direct extension, (b) through
hematogenous spread, (c) through the lymphatic system,

1500

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Fig. 70.43: Transthoracic two-dimensional (2D) echocardiogram

(parasternal long-axis view) of a patient with lymphoma and
evidence of a very large and uniform echodense mass anterior
and spanning the entire heart with possible extension into the
pericardial space. (AV: Aortic valve; LA: Left atrium; LV: Left
ventricle; L: Lymphoma).

Fig. 70.44: Subcostal view showing pericardial metastasis (P)

and circumferential pericardial effusion (PE) in a patient with metastatic lung cancer. (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).
Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
Buffalo, NY.

and (d) by intraluminal venous extension. About twothirds of all cardiac metastases involve the pericardium,
one-third the epicardium or the myocardium, and only 5%
the endocardium.148
Tumors such as the bronchial, breast, and esophageal
carcinoma, which develop near the heart, may expand by
direct extension into the heart, but predominantly by the
lymphatics. All preferentially affect the pericardium.148153
In the case of pericardial involvement, echocardiography
can show dense pericardial bands reflecting thickening
by inflammation or tumor infiltration. Pericardial effusion
can be proven quickly, with high sensitivity. When the
pericardium is involved, echocardiography is usually
used both as a guidance for pericardiocentesis and for the
follow-up of effusions. Tumor cells within the pericardial
fluid may verify diagnosis of metastatic pericardial
involvement (see Figs 70.43 and 70.44).
Malignant melanoma (see Figs 70.41 and 70.42),
lymphoma (see Fig. 70.43), leukemia, soft tissue, and bone
sarcoma usually spread hematogenously. Myocardial
metastases can involve any one of the heart chambers
(Figs 70.45A to E, Movie clips 70.45B, C and DE; Figs
70.46A to E; Movie clips 70.46A and B).13,159 Intracavitary
cardiac metastases are the least common variety. Echocardiography cannot distinguish intracavitary metastatic
tumor from primary cardiac tumors.

Some tumors diffuse along the IVC reaching the right

atrium and producing an intracavitary lesion, leading
occasionally to obstruction. These include carcinoma of
the kidney, hepatocellular carcinoma, leiomyoma of the
uterus, and carcinoma of the adrenal cortex. This type of
lesion can become so obstructive that it fills the chamber
completely and blocks tricuspid movement, resulting
in a clinical pattern similar to pericardial constriction
or myocardial restrictive disease. Invasion of the heart
through the SVC into the atrium can occur in the case of
carcinoma of the lung and thyroid gland.148
Carcinoid heart disease is also another entity, which
can be diagnosed by echocardiography. Metastatic
carcinoid tissue in the liver produces biologically active
substances, including serotonin, which cause abnormalities
of the right-sided cardiac valves and endocardium. Typical
changes include thickening, retraction, and increased
rigidity of the tricuspid and pulmonic valve leaflets,
resulting in valvular regurgitation or, less often, valvular
stenosis. Left-sided valvular involvement is rarely seen.160
Cardiac involvement is present in one-half of the patients
with carcinoid tumors. Heart failure resulting from severe
tricuspid regurgitation is a common cause of mortality in
these patients. Although metastatic carcinoid disease is
rare, the echocardiographic findings are pathognomonic
and may lead to the diagnosis in a patient in whom it was
not considered previously.160

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1501

Figs 70.45A to E: (A) Microscopic examination of a biopsy specimen

showing malignant tumor cells with marked nuclear pleomorphism,
prominent nucleoli, and high mitotic rate. No glandular or squamous
differentiation was noted; (B to E) Two-dimensional transthoracic
contrast echocardiography; (B) Precontrast study showing two
masses (arrow heads) in the left ventricle (LV) attached to the
ventricular septum; (C to E) Postcontrast study. Initial frames
showed contrast filling LV cavity and outlining the two masses,
but there was no significant tumor enhancement (C). Subsequent
beats (D and E) demonstrate prominent enhancement of both
metastatic tumor masses consistent with high vascularity seen in
a poorly differentiated malignant tumor. RV, right ventricle. (Movie
clips 70.45B, C and DE).
Source: Reproduced with permission from Yelamanchili P,
Wanat FE, Knezevic D, Nanda NC, Patel V. Two-dimensional
transthoracic contrast echocardiographic assessment of metastatic
left ventricular tumors. Echocardiography. 2006;23(3):24850.

1502

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.46A to E: Metastatic chordoma. (A) Real-time twodimensional transthoracic echocardiography. The arrow points to
the chordoma in the right ventricle (RV); (B and C) Live/real time
three-dimensional transthoracic echocardiography. Arrows point
to echolucencies consistent with hemorrhages and cystic areas,
and the arrow heads denote echodense areas produced by fibrotic bands. The less intense areas represent the myxoid stroma;
B and C represent in-vivo and ex-vivo studies, respectively;
(D and E) Pathological specimen showing the lobulated resected
tumor (D); The cut surface (E) shows hemorrhagic and cystic areas
(arrows) and dense fibrotic bands (arrow heads). (LA: Left atrium;
RA: Right atrium; TV: Tricuspid valve) (Movie clips 70.46A and
70.46B).
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Bell WC, Post JD. Live/real time three-dimensional
transthoracic echocardiographic description of chordoma metastatic to the heart. Echocardiography. 2008;25(4):4402.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

Normal Variants and Other Masses

There are several normal variant cardiac structures that
may mimic a cardiac mass. In conjunction with clinical
presentation, evaluation of their size, shape, location,
mobility, and site of attachment by echocardiography
helps to differentiate them from malignant pathology.

Chiari Network
The Chiari network is an embryological remnant that results
from incomplete reabsorption of the right valve of the sinus
venosus. It appears as a web-like structure with thread-like
components and is present in 2% of the population. Its widely
mobile and serpiginous appearance within the right atrium
can be confused with other highly mobile pathological
structures including vegetations, flail tricuspid leaflet,
ruptured chordae tendinae, thrombus, or right heart tumor.46
The key differential points by echocardiogram include (a)
identification of two, and ideally three, normal appearing
tricuspid valve leaflets; (b) presence of a bright, rotatory,
highly mobile echocardiographic target that does not move
into the right ventricular inflow tract or RV in diastole as
would be typical of a tricuspid leaflet vegetation; and (c)
in the four-chamber apical or subcostal view, the typical
posterolateral orientation and anteroinferior and medial
course of this structure across the right atrium. The use of
intravenous bolus contrast material to outline the course of
the IVC, right atrium, tricuspid valve, and RV can also be of
additional benefit in excluding the presence of RA mass and
tricuspid leaflet disruption.46

Eustachian Valve
The Eustachian valve, an embryological remnant of the
valve of the IVC, commonly appears as a thin flap arising
from the anterior rim of the IVC orifice. However, it can also
persist as a mobile, elongated structure that projects several
centimeters into the RA cavity and has been misinterpreted
as an intra-atrial thrombus. Eustachian valves move in a
more restricted manner than Chiari networks, and should be
imaged from the apical four-chamber view, right inflow tract,
and subcostal views to distinguish them from vegetation,
tumor, or thrombus in the right atrium. On echocardiogram,
it appears as a linear, echodense object.

Thebesian Valve
The thebesian valve, also known as the valve of the
coronary sinus, is a semicircular fold of the lining

1503

membrane of the right atrium. It can be mistaken for a

RA mass on echocardiogram, and varies anatomically
more than the Eustachian valve. On echocardiogram, it
has been described as an echodense, crescentic fold that
resembles bars and bands or threads and networks. It can
have fenestrations or present as multiple fine strands at the
coronary sinus.161 Clinical practice suggests that the valve
may present difficulties for cannulation of the coronary
sinus. In autopsy cases, thebesian valves are present in
over 70% of hearts.162,163 Over 15% of hearts have a valve
that covers more than 75% of the ostium and can be devoid
of any fenestrations.162

Crista Terminalis
The crista terminalis is a well-defined fibromuscular
structure formed by the regression of the septum
spurium as the sinus venosus is incorporated into the
RA wall. Depending on the amount of regression, the
crista terminalis can also be very prominent and mimic
a RA mass. On TTE, the crista terminalis is seen as an
echodense linear ridge in the posterior RA wall, extending
laterally from the atrial septum. On 3DE, it can be more
clearly defined as a thick and tapering linear structure in
the posterior wall of the right atrium.16

Moderator Band
The moderator band, or septomarginal trabeculation,
is a muscular trabeculation extending from the lower
interventricular septum to the anterior RV wall. It is easily
identified because of its location in the RV, carrying the
right bundle branch from the bundle of His. It is best seen
in the apical four-chamber view and subcostal views, and
in the apical view, can be seen as it transverses the RV
cavity at midventricular level, connecting the free wall to
the interventricular septum. In the parasternal long-axis
view of the RV, it can be seen as a large muscular band that
crosses the apex of the RV obliquely.

Coumadin Ridge
The Q-tip sign, also known as the Coumadin ridge, is
a prominent muscular structure formed between the left
atrial appendage (LAA) and the atrial insertion of the left
upper pulmonary vein. It was often misdiagnosed as a
thrombus until it became well described in the literature.
It can be differentiated from a thrombus by its lack of
mobility and characteristic location. This tissue may

1504

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

accumulate fat, creating a mass-like appearance, usually

with a thin, proximal part and a thicker, more bulbous,
distal part seen on echocardiogram.

Thrombus
Cardiac thrombi can be located within the atrial (Figs
70.47A and B, Movie clips 70.47, Figs 70.48A to E)7 and
ventricular chambers (Figs 70.49 and 70.50),7 and it can
extend to the heart from the vena cava (Figs 70.51A to
C) or from the heart to the pulmonary arteries (Figs 70.51A
to C and 70.52). They may develop as a consequence of
multiple underlying cardiac disorders affecting the valves
and myocardium.
Echocardiography serves as a cornerstone in the
evaluation and diagnosis of these patients. It is considered
to be the first-line imaging modality in such patients.

Atrial Thrombus
Within the atria, thrombi can be found in the atrial appendages, within the body of the atrium, or in a combination
of these areas. They are usually the consequence of poor
atrial emptying and blood stasis in conditions such as atrial
fibrillation, atrial flutter, and mitral stenosis. They may also
be associated with indwelling catheters (see Figs 70.51A to
C). The most common location for thrombi in the atrium
is the LAA, which cannot be regularly seen by TTE. In this

setting, TEE is the gold-standard technique, with a great

sensitivity and specificity to detect LAA thrombi.164 These
thrombi are seen as echo-reflecting masses in the atrial
body or in the apex of the LAA, distinct from the underlying
endocardium, observed in more than one imaging plane,
and not related to pectinate muscles. This should be
differentiated from the mass-like effect, which is a normal
variant rarely seen in the LAA on TEE (Figs 70.53A to D;
Movie clip 70.53). Other signs that are usually associated
with thrombosis in the atrium are low LAA emptying
velocities, dilated (area > 6 cm2) and multilobulated LAA,
and spontaneous echo contrast.

Left Ventricular Thrombus

In the ventricles, thrombi are usually located in the
apex. They usually occur in the setting of significant left
ventricular dysfunction and after acute anterior and/or
apical myocardial infarction. It is extremely rare to have a
LV thrombus on the top of a normally contracting LV wall; it
is usually adjacent to a hypokinetic and akinetic area of the
LV wall. These thrombi have to be seen in at least two views
(usually apical and short axis). LV thrombus is frequently
suspected. Beside ultrasound artifacts, normal structures
and normal variants can be mistaken as thrombus, such
as normal or aberrant trabeculae or false tendon, muscle
tendon such as moderator band, and papillary muscles.

Figs 70.47A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of right atrial thrombus. (A)
Arrowhead points to a large mobile serpiginous thrombus in the right atrium (RA) prolapsing into the right ventricle (RV); (B) Cropped
segments of the thrombus (arrowheads) demonstrate a homogenous appearance with no echolucencies, indicating absence of clot lysis
(Movie clip 70.47).
Source: Reproduced with permission from Nanda NC, Hsiung MC, Miller AP, Hage FG: Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1505

E
Figs 70.48A to E: Live/real-time three-dimensional transthoracic echocardiography. (A and B) Apical (A) and parasternal short-axis
(B) views show a large mass (arrow) consistent with thrombus in the right atrium (RA) with possible attachment to the RA free wall;
(C and D) Right parasternal views showing the attachment of the thrombus to the RA free wall in the vicinity of the inferior vena cava
(IVC) and tricuspid valve (TV) directly opposite the superior vena cava (SVC) entrance into the RA. (LA: Left atrium; LV: Left ventricle;
RV: Right ventricle); (E) Schematics of (a) clot, (b) myxoma, (c) sarcoma/chordoma, and (d) hemangioma. The horizontal arrowhead
in (a) points to central lysis in a clot, in (b) it points to an area of hemorrhage/necrosis in a myxoma, and in (c) to an area of necrosis
surrounded by thick, band-like tissue containing collagen, giving a doughnut-like appearance seen with chordoma and sarcoma. The
vertical arrowhead in (b) points to dense calcification in the myxoma. (d) Demonstrates a hemangioma that is completely vascular and
the echolucencies involve the whole tumor, including periphery (Movie clips 70.48AD).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.

1506

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.49A to D: Two-dimensional (A) and live/real-time three-dimensional (B to D) transthoracic echocardiography in right ventricular
thrombus. (A) The arrowhead points to one bifid or possibly two clots in the right ventricle (RV). (B and C) Cropping the three-dimensional (3D) data set demonstrates three separate clots in the RV (arrowheads). (D) Another patient with clots in RV showing central lysis in
a cropped 3D image. (LA: Left atrium; LV: Left ventricle; RA: Right atrium) (Movie clips 70.49A, BC parts 1 to 4, D).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time
three-dimensional transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598
609.

LV thrombus may be protruding within the LV

cavity or flat (mural) lying along the LV wall. It may be
homogeneously echogenic, or have a heterogeneous
texture often with central lucency. Thrombi may be fixed
along LV wall or present an independent motion to a
variable extent. Motion commonly involves a portion of
the thrombus but might involve the entire thrombus. What
differentiates LV thrombus from an artifact is that the
motion of the thrombus is independent of the underlying
myocardium. Color Doppler tissue imaging may further
facilitate this differential diagnosis.
Although TTE has high sensitivity and specificity in
diagnosing LV thrombus, very often the LV apex cannot be

clearly defined and the presence or absence of a thrombus

may be very difficult to establish. In such cases, the use
of contrast ultrasound agent injected intravenously can
clearly identify the presence of a thrombus and decrease
both intraobserver and interobserver variability.165

Vegetation
Cardiac vegetation presents as an oscillating or nonoscillating intracardiac mass attached to the valves, other
endocardial structures, or implanted on intracardiac
material. It is considered to be the pathological hallmark
of endocarditis. Endocarditis can involve any of the heart

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1507

Figs 70.50A to D: Live/real-time three-dimensional transthoracic echocardiogram. (A) Four-chamber view showing no thrombus or
aneurysm in the left ventricle (LV); (B) Anteriorposterior cropping displays the large aneurysm containing thrombus (arrow); (C and D)
Sectioning of the thrombus (C) and viewing it en face (D) shows no evidence of lysis or liquefaction. (RV: Right ventricle) (Movie clip
70.50).
Source: Reproduced with permission from Duncan K, Nanda NC, Foster WA, Mehmood F, Patel V, Singh A. Incremental value of live/
real time three-dimensional echocardiography in the assessment of left ventricular thrombi. Echocardiography 2006;23(1):6872.

valves, with predilection to prosthetic valves. Diseased

valves are more prone to be affected during infectious
states. Vegetations are typically attached to the lowpressure side of the valve structure, but may be located
anywhere on the components of the valvular and the
subvalvular apparatus, as well as the mural endocardium
of the cardiac chambers or the ascending aorta. When
large and mobile, vegetations are prone to embolism and
less frequently to valve or prosthetic obstruction.
Two-dimensional and transoesophageal echocardiography serves as the cornerstone in noninvasive detection
of vegetation. In the setting of endocarditis, the role
of echocardiography is not just the identification of

vegetation; it provides clinically important information on

the presence and degree of valvular destruction and their
hemodynamic consequences, as well as on the existence
of perivalvular infection.
Overall, the detection rate for vegetations by TTE
in patients with a clinical suspicion of endocarditis
averages around 50 to 75%.166,167 The diagnostic yield of
the technique in the detection of vegetations is influenced
by several factorsimage quality; echogenicity and
vegetation size; vegetation location; presence of previous
valvular disease or valvular prosthesis; experience
and skill of the examiner; and pre-test probability of
endocarditis.

1508

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

TEE enhances the sensitivity to 8590% for the diagnosis

of vegetations, while more than 90% specificity has been
reported for both TTE and TEE.167169 Both TTE and TEE
do not permit differentiation between septic and other
aseptic vegetations present in conditions such as Libman
Sacks endocarditis in systemic lupus erythematosus,
antiphospholipid syndrome, and marantic endocarditis.
Vegetations on prosthetic valves are more difficult
to detect by TTE than those involving native valves.
Therefore, TEE should always be used if the diagnosis
of prosthetic endocarditis is suspected. The sewing ring
and support structures of mechanical and bioprosthetic
valves are strongly echogenic and may prevent vegetation
detection within the valve apparatus or its shadow.
Thrombus, pannus, and strands can be mistaken as
vegetative material given their similar appearance
(Figs 70.54A to C; Movie clips 70.54A to C). In large

Figs 70.51A to C: Two-dimensional transesophageal echocardiography. Thrombus in the pulmonary artery and the superior
vena cava. (A to C) Large thrombi (M,C) are noted in the superior vena cava (SVC) and the right pulmonary artery (RPA) in this
patient with an infusion catheter, which acted as the nidus for
thrombus. (AO: Aorta; ASC AO: Ascending aorta; LA: Left atrium).
Source: Reproduced with permission from Nanda NC, Domanski
MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia: Lippincott Williams & Wilkins; 2007:521.

series of prosthetic endocarditis, TEE has shown an 86

to 94% sensitivity and 88100% specificity for vegetation
diagnosis, while TTE sensitivity was only 3669%.169
In the setting of tricuspid vegetations, TTE allows
an easy and correct diagnosis, probably because the
majority of patients with tricuspid endocarditis are young
intravenous drug abusers with large vegetations. The
vegetations are located on the atrial side of the tricuspid
valve, in the way of the regurgitant jet. TEE did not increase
the accuracy of TTE in the detection of vegetations in
tricuspid endocarditis in one study.168,170
Infection or endocarditis on a pacemaker lead is
difficult to diagnose by TTE, since pacemaker leads
produce reverberations and artifacts that may mask or
make difficult the recognition of vegetations close to these
structures. In addition, when vegetations were visualized,
it was difficult to determine whether tricuspid valve

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1509

D
Figs 70.52A to E: Two-dimensional transesophageal echocardiography. A 78-year-old black female. (A and B) Thrombic obstruction
of distal left and right pulmonary arteries. Arrowhead points to a
large thrombus in the distal left pulmonary artery (LPA, A) and in
the distal right pulmonary artery (RPA, B); (C to E) Thrombotic
occlusion of LPA descending lobar branches; (C) Large arrowhead
points to the thrombus (TH) and small arrowhead points to an
echolucent area of clot lysis. Arrow shows extension of the TH into
a descending lobar branch of the LPA; (D) Turbulent flow signals
with prominent flow acceleration are noted in a descending lobar
branch (labeled 2). Arrow points to the left pulmonary vein (LPV),
and arrowhead shows the TH; (E) Color Doppler-guided continuous
wave Doppler interrogation (arrow) shows a high systolic velocity
of 2.2 m/s and a high diastolic velocity of 1.0 m/s indicative of
significant obstruction. (AO: Aorta; T: Transverse plane) (Movie
clips 70.52A to E).
Source: Reproduced with permission from Kang SW, Nekkanti R,
Nanda NC, et al. Transesophageal echocardiographic identification of thrombus producing obstruction of left pulmonary artery descending lobar branches and bronchial artery dilatation. Echocardiography. 2002;19:838.

1510

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.53A to D: Transesophageal echocardiographic finding of LAA lobe mimicking a mass lesion. (A) The arrowhead points to what
appears to be a mass in or adjacent to LAA; (B) Schematic; (C) Keeping the mass-like lesion in the middle of the monitor screen and
rotating the transducer from 0 to 180 shows that the mass-like effect is produced by a lobe (#3) of the LAA; (D) Schematic. Numbers
1 and 2 denote the other two lobes of the LAA. (AO: Aorta; LA: Left atrium; LAA: Left atrial appendage; LV: Left ventricle; M: Mass; MV:
Mitral valve; PA: Pulmonary artery; PE: Fluid in the transverse sinus of pericardium) (Movie clip 70.53).
Source: Reproduced with permission from Giove GC, Singla I, Mishra J, Nanda NC. Transesophageal echocardiographic finding of left
atrial appendage lobe mimicking a mass lesion. Echocardiography. 2011;28:6845.

endocarditis, lead infection, or both were present. TEE was

clearly superior to TTE in this clinical setting (sensitivity
23% vs 94%).171
Regurgitation of the infected valve is present in
most cases resulting from a variety of mechanisms. They
include prevention of proper leaflet or cusp coaptation by
the vegetation and valvular destruction that can result in
a small perforation in a cusp, or lead to a complete flail
leaflet. Valvular perforation is a frequent complication that
may cause severe insufficiency with an acute onset and
precipitate heart failure. This is more common in aortic

valves than mitral valves. This carries a poor prognosis in

most cases. TTE appears more useful in detecting mitral
than aortic perforations. Color flow Doppler imaging
allows location of abnormal flows at the areas of anatomical
interruption and, therefore, it helps to differentiate mitral
cusp perforation from true mitral regurgitation. TEE is
recommended if a valve perforation is suspected and TTE
is negative or equivocal.
In the case of mechanical mitral prosthesis, TEE with
color flow mapping is of importance in the diagnosis
of paravalvar regurgitation. The presence of a new or

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

C
increasing paravalvar regurgitation or valve dehiscence is
a major criterion for the diagnosis of endocarditis.
Extension of the infection to the perivalvar tissues is
a sign of poor prognosis in the evolution of the disease.
Extravalvar extension may lead to endothelial erosion,
perivalvar abscess, mycotic aneurysm, and intracardiac
fistulae.

Perivalvular Abscess
Perivalvar cavities are formed when annular infections
break through and spread into contiguous tissue. In native
aortic valve endocarditis, they generally occur through
the weakest portion of the annulus, which is near the
membranous septum. Perivalvar abscesses are particularly
common in prosthetic valve endocarditis, since the
annulus is the usual primary site of infection. A perivalvar

1511

Figs 70.54A to C: Two-dimensional (A and B) and three-dimensional

(C) transthoracic echocardiography. Tumor mimic following mitral
valve replacement. Arrowhead points to subvalvular apparatus
(chordae and papillary muscle) remaining in the left ventricle
following resection of the native mitral valve and replacement
with a prosthetic valve. This could be misdiagnosed as tumor or
vegetations. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle) (Movie clips 70.54A to C).

abscess is considered to be present when a definite region

of reduced echodensity is found on the echocardiogram,
or when echolucent cavities within the valvular annulus
or adjacent myocardial structures are found in the setting
of valvular infection. Sensitivity and specificity of TTE
for abscess detection were 28% and 99%, respectively,
compared with 87% and 95%, respectively, with TEE. TEE is
especially useful in prosthetic endocarditis. The diagnosis
of aortic abscesses were easier than mitral abscesses, both
with TTE (42% vs 9%) and TEE (86% vs 57%).8

MICE
Mesothelial/macrophage incidental cardiac excrescences,
or MICE, are made up of mesothelial cells, macrophages,
scattered inflammatory cells and fibrin, and lack a vascular
network or supporting stroma.172 Lack of vascularity is an

1512

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.55A and B: Real time two-dimensional transthoracic echocardiography. Arrowhead points to a large echogenic mass involving
the posterior mitral annulus, consistent with calcification. An echolucent area consistent with liquefaction is seen in B. (AO: Aorta; LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clips 55A and B).
(Source: Reproduced with permission from Assudani J, Singh B, Samar A, et al. Echocardiography. 2010;27:114750).

important feature distinguishing them from localized

mesothelial hyperplasia, which can also form incidentally
detected excrescences and contain sheets of mesothelial
cells, macrophages, and fibrin. They are more commonly
found in the left heart chambers and on valve surfaces
especially during aortic and mitral valve surgery or in
endomyocardial biopsy specimens. On echocardiography,
it can appear as a free-floating mass or loosely adherent.
It has been described as isointense on MRI compared to
myocardium on T1-weighted images and hyperintense
on T2-weighted images, without enhancement with
contrast.173 While they are benign, they may be potentially
mistaken for a primary or metastatic malignancy.

Mitral Annular Calcification

Mitral annular calcification (MAC) is a common echocardiographic finding seen mainly in older patients and
those with chronic renal failure. Caseous calcification
is a rare variant seen as a large, round, echodense
mass with smooth borders situated in the periannular
region, with no acoustic shadowing artifacts and
containing central areas of echolucencies resembling
liquefaction.174 The diagnosis is made by TTE in most
cases but 2D or 3D transesophageal echocardiogram
(Figs 70.55 to 70.59; Movie clips 70.55A and B to 70.58A
Parts 1 and 2, B, CD Parts1 and 2-D) are helpful in some
cases where the diagnosis is in question to make more

Fig. 70.56: Live/real time three-dimensional transthoracic echocardiography. Cropped three-dimensional data set. The lower
arrowhead points to a highly echogenic calcification while the upper
arrowhead shows a less echogenic area of uniform appearance.
(MV: Mitral valve; TV: Tricuspid valve) (Movie clip 70.56).
Source: Reproduced with permission from Assudani J, Singh B,
Samar A, et al. Echocardiography. 2010;27:114750.

definitive diagnosis and prevent more invasive testing

and even exploratory cardiotomy.

Cardiac Calcified Amorphous Tumor

Cardiac calcified amorphous tumor (CAT) is another
rare benign cardiac mass that can mimic malignancy and

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1513

Fig. 70.57: Real time two-dimensional transesophageal echocardiography. The arrowhead denotes posterior mitral annular
calcification. Note shadowing and reverberations beneath the
calcification. (LA: Left atrium; LV: Left ventricle) (Movie clip 70.57).
Source: Reproduced with permission from Assudani J, Singh B,
Samar A, et al. Echocardiography. 2010;27:114750.

Figs 70.58A to D: Live/real time three-dimensional transesophageal echocardiography in caseous mitral annular calcification.
(A and B) Four-chamber views. The arrowheads in A and B point to a large echogenic mass involving the posterior mitral annulus,
consistent with calcification. In B, the mass shows multiple, discrete, band-like, and punctuate echodensities surrounded by a highly
echogenic border. (C and D) Cropping of the four-chamber data set shows both a highly echogenic (lower arrowhead) and a relatively
less echogenic (upper arrowhead) components of the mass. Transverse cropping (D) also reveals a pattern of highly echogenic
component as well as a relatively less echogenic area with multiple, small, discrete band-like, and punctate echodensities. (AML:
Anterior mitral leaflet; AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). [Movie clips 70.58A (Parts 1 and 2), B to D
(Parts 1 and 2)].
Source: Reproduced with permission from Assudani J, Singh B, Samar A, et al. Echocardiography. 2010;27:114750.

1514

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.59A and B: Two-dimensional transesophageal echocardiography. Extracardiac tumor. (A and B) Tumor (T, arrow) is seen posterior to the aortic root bulging into the left atrium (LA). (AO: Aorta; LV: Left atrium; RV: Right ventricle).
Source: Reproduced with permission from Nanda NC, Domanski MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2007:524.

cause symptoms due to obstruction or embolization of

calcific fragments.175,176 Typical findings for CAT include
calcium nodules within amorphous fibrinous materials
often displaying characteristic histological findings that
include nodular calcium deposits, chronic inflammatory
cell infiltration, hyalinization, and degenerating blood
elements.175 Intracardiac echocardiogram will demonstrate
curvy linear densities representative of endomyocardial
calcifications.177

Extracardiac Masses
In addition to normal cardiac variants, certain extracardiac
masses can also mimic cardiac tumors by compressing
the cardiac chambers from the outside of the heart and
creating a mass effect. Examples include tumors or diseases
of the mediastinum (Figs 70.59 to 70.63), hematomas
(Fig. 70.64), coronary aneurysms, and pseudoaneurysms.
Although not uncommon, pericardial fat deposition
when prominent can masquerade as extracardiac tumor
(Figs 70.65A and B; Movie clips 70.65A and B).
Sclerosing mediastinitis is a rare disorder characterized
by the invasive proliferation of fibrous tissue within the
mediastinum and frequently results in the compression
of vital mediastinal structures including the heart. It can
cause compression of the atrial or ventricular cavities
and can cause obstruction of the SVC and the pulmonary
arteries (Figs 70.60 to 70.63).

Hiatal hernias can also be seen on TTE and TEE

as an extracardiac mass compressing the left atrium
with associated mild left ventricular hypertrophy with
normal function and no thrombus in the LA. If a hiatal
hernia is suspected, use of oral contrast or soda during
TTE may be diagnostic, but during TEE it carries the
risk of aspiration.178 Juxtacardiac pulmonary atelectasis
or lobar collapse can also simulate pericardial tumor
implants on echocardiographic examination (pericardial
pseudotumor) from surrounding compressive effusive
fluid.179 Echocardiographic delineation of pericardial and
pleural anatomy can be used to delineate the atelectatic
nature of these masses, combined with ancillary
radiographic and CT studies. In addition, drainage of
pleural fluid will lead to the disappearance of the masses
on echocardiographic examination.

Intracardiac Hardware
In addition to intrathoracic masses mimicking cardiac
tumors, intracardiac hardware such as Impella percutaneous left ventricular assist device catheters, pacing
leads, SwanGanz catheters, and central line catheters
are also commonly seen left atrial foreign bodies on
echocardiography. Catheters and pacemaker leads are
usually seen on echocardiography as two parallel thin
linear echodensities separated by an echolucent slit that
produce typical reverberations and side lobe artifacts

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

A
Figs 70.61A and B

1515

Figs 70.60A to C: Two-dimensional transesophageal echocardiography. Sclerosing mediastinitis. This 43-year-old man presented
with respiratory failure. (A) A mass (M) surrounds the left atrium
(LA), right pulmonary artery (RPA), and superior vena cava (SVC);
(B and C) The mass appears to infiltrate and invaginate into the LA
and extends up to the base of the left atrial appendage (LAA). This
resembles an intracardiac tumor. (AO: Aorta; AV: Aortic valve; PA:
Pulmonary artery; RA: Right atrium; RVO: Right ventricular outflow).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in sclerosing mediastinitis. Echocardiography. 1996;13:1038.

1516

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.61A to C: Two-dimensional transesophageal echocardiography. Sclerosing mediastinitis. Same patient as in Figures
70.60 A and B. Both the right lower pulmonary vein (RLPV) and
right upper pulmonary vein (RUPV) demonstrate obstruction near
their entrance in the left atrium (LA). The exact sites of obstruction
in the lower and upper pulmonary veins, shown by the arrow and
the arrowhead, respectively, mark the transition from laminar (red)
to disturbed (mosaic) flow; (C) Pulsed Doppler interrogation of
the mosaic flow reveals a high velocity of 2.58 m/s, indicative of
obstruction. (LV: Left ventricle; M: Mass; RVO: Right ventricular
outflow; SVC: Superior vena cava).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:1038.

Figs 70.62A to C: Two-dimensional transesophageal echocardiography. Sclerosing mediastinitis. Same patient as in Figures 70.60
and 70.61. (A and B). The arrow points to the site of obstruction in
the superior vena cava (SVC) near its junction with the right atrium (RA). Color Doppler examination shows a thin mosaic flow jet
in (B), indicative of obstruction; (C) Pulsed Doppler interrogation
reveals a high velocity of at least 1.61 m/s. (LA: Left atrium; M:
Mass; RPA: Right pulmonary artery).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:1038.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

1517

Figs 70.63A and B: Sclerosing mediastinitis. Histology of mediastinal biopsy tissue from the same patient shown in Figures 70.60 to
70.62. (A) Photomicrograph of sclerosing process impinging on mediastinal adipose tissue (congo red, original magnification 125);
(B) Photomicrograph of collagenization of blood vessel wall with narrowing of the lumen (center), a region of cellular fibrosis (above),
and a region of acellular fibrosis (below; hematoxylin and eosin, original magnification 125).
Source: Reproduced with permission from Kovach TA, Nanda NC, Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:103108.

Fig. 70.64: Two-dimensional transesophageal echocardiography.

Hematoma following cardiac surgery. Arrowheads show hematoma that developed around the aortic root following aortic valve
replacement. It had a benign course. (AO: Aorta; LA: Left atrium;
LAA: Left atrial appendage).
Source: Reproduced with permission from Nanda NC and
Domanski MJ, editors. Atlas of Transesophageal Echocardiography.
2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:535.

shadowing other structures. Clinical correlation is important

in assessing the echocardiographic presence of such objects.
In summary, the integration of the echocardiographic
findings with the patients medical history and clinical
scenario is critical to establishing a differential diagnosis
and to determining the hemodynamic consequences

of a cardiac mass or tumor. Serial studies can provide

useful information regarding progression, regression, and
results of treatment of the mass or tumor. In some cases,
additional imaging modalities such as cardiac CT and MRI
can provide complementary information that enhances
the echocardiographic findings.

1518

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses

Figs 70.65A and B: Two-dimensional transthoracic echocardiography. Epicardial fat pad. Arrows point to prominent epicardial fat deposition both anteriorly and posteriorly visualized in parasternal long-axis view and around the cardiac apex in the apical four-chamber
view (Movie clips 70.65A and B). (LA: Left atrium; LV: Left ventricle; RV: Right ventricle).

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patients. Circulation. 1993;87(4):118896.
161. Hellerstein HK, Orbison JL. Anatomic variations of
the orifice of the human coronary sinus. Circulation.
1951;3(4):51423.
162. Mak GS, Hill AJ, Moisiuc F, et al. Variations in Thebesian
valve anatomy and coronary sinus ostium: implications
for invasive electrophysiology procedures. Europace.
2009;11(9):118892.
163. Pejkovic B, Krajnc I, Anderhuber F, et al. Anatomical
variations of the coronary sinus ostium area of the human
heart. J Int Med Res. 2008;36(2):31421.
164. Pearson AC, Labovitz AJ, Tatineni S, et al. Superiority of
transesophageal echocardiography in detecting cardiac
source of embolism in patients with cerebral ischemia of
uncertain etiology. J Am Coll Cardiol. 1991;17(1):6672.
165. Kurt M, Shaikh KA, Peterson L, et al. Impact of contrast
echocardiography on evaluation of ventricular function
and clinical management in a large prospective cohort. J
Am Coll Cardiol. 2009;53(9):80210.
166. Mgge A, Daniel WG, Frank G, et al. Echocardiography
in infective endocarditis: reassessment of prognostic
implications of vegetation size determined by the transthoracic and the transesophageal approach. J Am Coll
Cardiol. 1989;14(3):6318.

Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses

167. Evangelista A, Gonzalez-Alujas MT. Echocardiography in

infective endocarditis. Heart. 2004;90(6):6147.
168. Shapiro SM, Young E, De Guzman S, et al. Transesophageal
echocardiography in diagnosis of infective endocarditis.
Chest. 1994;105(2):37782.
169. Shively BK, Gurule FT, Roldan CA, et al. Diagnostic
value of transesophageal compared with transthoracic
echocardiography in infective endocarditis. J Am Coll
Cardiol. 1991;18(2):3917.
170. San Romn JA, Vilacosta I, Zamorano JL, et al. Transesophageal echocardiography in right-sided endocarditis.
J Am Coll Cardiol. 1993;21(5):122630.
171. Klug D, Lacroix D, Savoye C, et al. Systemic infection related
to endocarditis on pacemaker leads: clinical presentation
and management. Circulation. 1997;95(8):2098107.
172. Veinot JP, Tazelaar HD, Edwards WD, et al. Mesothelial/
monocytic incidental cardiac excrescences: cardiac MICE.
Mod Pathol. 1994;7(1):916.
173. Censi S, DellAmore A, Conti R, et al. Cardiac mesothelial/
monocytic-incidental-excrescence: more than an artifactual
lesion? Interact Cardiovasc Thorac Surg. 2008;7(6):12013.

1523

174. Deluca G, Correale M, Ieva R, et al. The incidence and

clinical course of caseous calcification of the mitral
annulus: a prospective echocardiographic study. J Am Soc
Echocardiogr. 2008; 21(7):82833.
175. Reynolds C, Tazelaar HD, Edwards WD. Calcified amorphous tumor of the heart (cardiac CAT). Hum Pathol.
1997;28(5):6016.
176. Lewin M, Nazarian S, Marine JE, et al. Fatal outcome of
a calcified amorphous tumor of the heart (cardiac CAT).
Cardiovasc Pathol. 2006; 15(5):299302.
177. Habib A, Friedman PA, Cooper LT, et al. Cardiac calcified
amorphous tumor in a patient presenting for ventricular
tachycardia ablation: intracardiac echocardiogram diagnosis and management. J Interv Card Electrophysiol.
2010;29(3):1758.
178. Chan J, Manning WJ, Appelbaum E, et al. Large hiatal hernia
mimicking left atrial mass: a multimodality diagnosis. J Am
Coll Cardiol. 2009;54(6):569.
179. Plehn J, Sager J, Foster E, et al. Pericardial pseudotumor.
Echocardiographic observation of juxtacardiac pulmonary
collapse. Chest. 1988;94(4):83741.

SECTION 6
Congenital Heart Disease

Chapters
Chapter 71 Fetal Cardiac Imaging
Chapter 72 M-Mode and Two-Dimensional Echocardiography
in Congenital Heart Disease
Chapter 73 Real time 3D Echocardiography for
Quantification of Ventricular Volumes, Mass and
Function in Children with Congenital and Acquired
Heart Diseases

Chapter 74 Three-Dimensional Echocardiography in

Congenital Heart Disease
Chapter 75 Echocardiography in the Evaluation of
Adults with Congenital Heart Disease
Chapter 76 Echocardiographic Evaluation for Acquired Heart
Diseases in Childhood

1527

CHAPTER 71
Fetal Cardiac Imaging
Aarti H Bhat

Snapshot

Scope of Fetal Cardiology

Indicaons for Fetal Cardiac Evaluaon

Fetal Physiology

Indicaons for Fetal Echocardiography

INTRODUCTION
The field of fetal cardiology has made significant advances
along with all aspects of prenatal evaluation over the last
three decades. Almost all structural fetal heart disease is
amenable to a detailed in utero diagnosis that can then
be used to develop plans for fetal as well as postnatal
management of a pregnancy. Fetal ultrasound and twodimensional imaging are central to this diagnostic process.
Improvements in ultrasound techniques as well as our
enhanced understanding of fetal cardiac anatomy and
physiology have facilitated and broadened the scope of
this specialty. The purpose of this chapter is to familiarize
the reader with the basic framework of fetal cardiology
using a basic description of echocardiographic anatomy.
Liberal use of illustrative labeled screen images as well as
movie clips (on-line version) are intended to orient the
reader as well as provide an example of the anatomical
defect or finding being discussed.

SCOPE OF FETAL CARDIOLOGY

Cardiac malformations occur in 4 to 8/1,000 live births
and account for one-third of perinatal mortality due to
congenital anomalies.13 In the low-risk general population, an obstetric scan using the four-chamber and

Extracardiac Reasons and Associaons for Fetal Heart

Disease

Fundamentals of Fetal Cardiac Imaging

Case Studies

outflow views yields a sensitivity of 52 to 92% in detecting

congenital heart defects. The range is similar in highrisk populations. A fetal echocardiogram (traditionally
acquired if the screening ultrasound is positive and/or in
high-risk pregnancies) has a sensitivity of 42 to 100% for
detection of congenital heart disease.411 Over the years,
many studies have proven aspects of improved survival
or decreased morbidity in groups of newborns that have
received a prenatal cardiac diagnosis that translates into
a specific birth plan and postnatal management including
emergent neonatal intervention.1217 Identification of
critical heart disease can allow in utero surveillance
culminating in therapy in the form of maternal medications,
fetal cardiac intervention, and a heightened awareness
of immediate requirements in the delivery room or soon
after.18,19 Despite the presence of the need for prenatal
diagnosis and the presence of robust tools to achieve this,
the overall rate of prenatal detection by routine obstetric
scan is unfortunately low.2022
Heart defects in a fetus may be isolated if there is no
other organ or genetic abnormalities or associated with
other organ anomalies or genetic problems. Determining
a possible abnormality in fetal life allows for a more
detailed evaluation of the other organ systems, fetal
growth and well-being, and chromosomal disorders. Such

1528

Section 6: Congenital Heart Disease

a process may lead to an overall complete fetal diagnosis,

allowing the involved teams to plan a process of pregnancy
management. Practice care models are unique to each
institution and vary greatly depending on the setting
(primary vs. tertiary), specialty (obstetric, pediatric, or
combined) or skill and resource level (screening center
or referral center). There are potentially many good and
appropriate ways to achieve the endpoints of prenatal
cardiac care, which are (a) as accurate and complete a
diagnosis as possible; (b) a complete, fact-driven, and
compassionate counseling; and (c) securing a safe birth
and transition plan for the maternalfetal dyad and the
newborn. This chapter is aimed at providing the reader with
a good understanding of the first necessary undertaking of
anatomical diagnosis.
Prenatal counseling is very stressful on the parents
for whom the hope and prospect of a normal baby is
now changed. A genuine compassionate and honest
approach to the interview session needs to secure their
understanding and trust, as the whole team establishes
common goals. Parental grief, stress, and depression need
to be acknowledged as these are known to continue from
the prenatal to the postnatal periods.23

INDICATIONS FOR FETAL CARDIAC

EVALUATION
A screening obstetric ultrasound at about 18 to 20 weeks
is usually performed in most obstetric programs. The
primary aim of this scan is to acquire all pertinent fetal and
uteroplacental information in a particular pregnancy. An
abnormal fetal cardiac or extracardiac scan at this gestation
as well as a suspected or proven chromosomal anomaly
prompts a fetal cardiac evaluation and is the single most
robust source for fetal cardiac pathology. The vast majority
of these patients do not have any of the risk factors typically
considered as high risk. Pregnancies considered at high
risk for fetal cardiac and noncardiac pathologies are
the dominant cause for referral to the fetal cardiologist,
but, the relative yield of abnormal fetal cardiac exams is
relatively lower from this indication group. This brings
up an interesting aspect while planning a fetal cardiac
program aiming to increase prenatal diagnosis rates in
that most of the abnormal fetal heart diagnoses actually
come from a low-risk group. This fact implies that any
increase in fetal cardiac diagnosis over a population
can only come from an improvement in the obstetric
screening scan. Improving the awareness of obstetric
sonographers, close attention to and low threshold for

referral if there are concerning or unusual findings, and

improved access to fetal cardiologists are each a key step
in overall improvement in the rates of prenatal diagnosis
of fetal heart defects. Critical congenital heart disease such
as atresias of the atrioventricular valve with or without
accompanying hypoplastic ventricles, cardiac dysfunction,
and pericardial effusion are amenable to detection from a
four-chamber view. However, other critical diseases such
as transposition of the great arteries and semilunar valve
hypoplasia/atresia can only be detected if biventricular
outflow view can be obtained. The parallel orientation of
the great arteries can be easily determined in the outflow
view even if the four-chamber view indicated no obvious
abnormality [for example, in a dextro Transposition of great
arteries (dTGA) without ventricular septal defect (VSD)].
Nuchal translucency (NT) detects the echo-free space at
the back of the neck, typically measures at 11 to 14 weeks
of gestation. Increased NT (typically > 4 mm) is strongly
associated with abnormal chromosomes, with or without
congenital heart disease.24,25 Often times, at this stage,
with or without a confirmed diagnosis of fetal aneuploidy
depending on when and if the parents and providers are
proceeding with an amniocentesis or chorionic villus
biopsy (CVS), the pregnancy is referred for a fetal cardiac
evaluation. Even in the absence of confirmed aneuploidy,
elevated fetal NT in the first trimester and other soft
markers such as intracardiac echogenic focus (IEF) and
intrauterine growth retardation (IUGR) or extracardiac
pathology may be indications for fetal echocardiography.

FETAL PHYSIOLOGY
Fetal circulation appears uniquely adapted to maximize
efficiency and oxygen delivery. Some of the most
exhaustive information on fetal physiology was originally
derived from fetal sheep.26 Similar data from the human
fetus is incomplete. Oxygenated blood is delivered to
the fetus through the umbilical vein. This vein enters the
porta hepatis and gives several branches to the left lobe
of the liver, distal to these is the ductus venosus. The
umbilical veins then arches toward the right lobe of the
liver, is joined there by the portal vein, and gives branches
to the right lobe of the liver. The ductus venosus then
continues on and connects to the inferior vena cava, as
do the left hepatic veins. Flow from the ductus venosus is
preferentially directed by the Eustachian valve, across the
foramen ovale to the left atrium. Nearly the entire return
from the superior vena cava enters the right ventricle
through the tricuspid valve.2731 Right ventricular output

Chapter 71: Fetal Cardiac Imaging

[55% of the combined biventricular output (CVVO)] is

directed across the pulmonary valve, most of this enters
the ductus arteriosus, and joins the aortic arch at the
isthmus. Just under 20% of the combined cardiac output
enters the pulmonary bed through the pulmonary arteries
and returns to the left atrium. Left ventricular output
(45% of the CVVO) is directed across the aortic valve to the
ascending aorta. Most of this amount flows to the coronary
arteries, and the head and neck vessels, so that only 10% of
the CVVO actually crosses the isthmus, the region of the
distal aortic arch between the origins of the left subclavian
and the insertion of the ductus arteriosus. The aortic and
ductal arches merge, and the descending aorta carries
supply to the abdominal organs and then to the umbilical
artery, headed to the placenta. In this manner, the entire
umbilical venous flow is returned to the umbilical arterial
side.
The CVVO in the fetus is about 450 mL/min/kg of
estimated fetal weight and the right:left ventricular output
ratio is about 1.2 to 1.3.32,33 CVVO is determined by heart
rate and stroke volume, and the latter is determined by
preload and afterload, very similar to the physiological
inter-relationships seen in children and adults. However,
fetal myocardium generates less tension at similar muscle
lengths as compared to adult myocardium and there may
be differences in the sarcoplasmic reticulum as well as
calcium uptake function.34,35
In the fetus, the two natural rightleft communications
at the level of the foramen ovale and the ductus arteriosus
allow equalization of the right and left pressures at the
atrial and great arterial levels. The left ventricularaortic
output upto and including the aortic isthmus is likely a
higher afterload as compared to the right ventricular
pulmonary trunk output that has a higher compliance
placental bed distally.

INDICATIONS FOR FETAL

ECHOCARDIOGRAPHY
Maternal Indications
Family History of Congenital Heart Disease
Family history of transmissible genetic malformations
that have known cardiac abnormality such as 22q11
deletion syndromes, Marfan or Noonan syndrome as well
as multifactorial-etiology type defects such as ventricular
septal defect or bicuspid aortic valve-hypoplastic left heart
syndrome spectrum in first order relatives is an indication

1529

for fetal echocardiography. Maternal metabolic disorders

such as diabetes and phenylketonuria are known to
increase the risk for congenital cardiac defects. Maternal
diabetes in particular has been the cause of much
controversy in terms of screening recommendations.3640
The incidence of fetal cardiac defects is noted to be
as much as five time higher than euglycemic pregnancies
and worsens with HbA1c levels > 6.3% in the first trimester.
Existing strategies are based on resource utilization
models (comprehensive fetal obstetric ultrasound in
the pregestational diabetic population, followed by fetal
cardiac referral in the presence of concerning findings)
or risk-based models (risk increases with elevated HbA1c
levels).41 Maternal autoimmune disease such as Sjogren
syndrome or systemic lupus erythematosus has an
increased incidence of fetal congenital complete heart
block (CCHB) that can be detected as well as monitored
by fetal echo and for which therapeutic options exist.42
Maternal teratogen exposure, for example, alcohol, lithium,
retinoic acid anticonvulsants, and SSRI antidepressant
have noted increase in fetal cardiac and extracardiac
malformations. Maternal and intrauterine infections such
as congenital rubella and coxsackie virus can be seen with
cardiac defects. The use of in vitro fertilization or other
reproductive assist techniques are being increasingly
recognized with elevated rate of congenital cardiac
malformations.
Fetal indications such as a fetal heart abnormality
suspected on a screening obstetric ultrasound, extracardiac structural abnormalities, single umbilical artery,
chromosomal abnormalitiesconfirmed or suspected
fetal arrhythmia, multiple gestations, and fetal nonimmune hydrops.

EXTRACARDIAC REASONS AND

ASSOCIATIONS FOR FETAL
HEART DISEASE
Twin gestations can have unique cardiovascular findings,
more common in monochorionic gestations.
Twintwin transfusion syndrome (TTTS) can occur due
to abnormal placental vascular connections in about 15%
of monochorionic twin pregnancies, increasingly seen due
to more widespread use of reproductive technologies.43
There is discordance in fetal size. The recipient twin is
larger, has polyhydramnios, cardiomegaly, biventricular
hypertrophy and dysfunction, and atrioventricular valve
incompetence. In approximately 10%, there is acquired

1530

Section 6: Congenital Heart Disease

right ventricular outflow tract obstruction, pulmonary

stenosis, and even reversal of ductal flow.44,45 Eventually,
recipient cardiomyopathy from systolic as well as diastolic
dysfunction can lead to hydrops and fetal demise. The
donor twin, on the other hand, is likely to have a structurally
and functionally normal heart, evidence of elevated
placental vascular resistance, relative hypovolemia, and
oligohydramnios. Scoring systems are used to gauge
disease severity.46,47 Laser photocoagulation of the
placental vascular connections is currently the treatment
of choice for TTTS.48 The abnormal cardiovascular findings
can improve after this therapy, although complete normalization is unlikely in the subgroup that has developed
anatomical pulmonary atresia.49
Twin reverse arterial perfusion (TRAP) occurs in 1% of
monochorionic pregnancies, in which one twin is acardiac
with a primitive or nonfunctional heart. The normal twin
heart pumps blood through superficial arterialarterial
placental anastomosis to this acardiac twin. As a result, the
direction of flow in the umbilical artery of the acardiac
acephalic twin is from placenta to fetus (reversed). Serially
evaluating the combined cardiac output of the normal twin
can monitor for the increased cardiovascular workload to
this heart, which can culminate in heart failure, hydrops,
and demise.50
Congenital cystic adenomatoid malformation (CCAM)
is a rare developmental abnormality of the lung due
to overgrowth of the terminal bronchioles, causing a
dysplastic lung mass. This mass can cause compression
of the developing fetal heart, alter its position within the
thorax, alter the angulation of the inferior vena cava as it
enters the displaced heart, decrease cardiac filling, and
potentially lead to fetal hydrops. Due to the increased
intrathoracic pressure from the lung mass, hydrops is not
typically accompanied by pleural or pericardial effusions.51
Congenital diaphragmatic hernia (CDH) is a defect
in the muscular diaphragm between the chest and
abdominal cavities. Abdominal contents can herniate
through this defect and occupy space in the chest,
causing varying degrees of displacement and extrinsic
compression to the heart and lungs. Since this process
happens in early gestation, the presence of herniated
contents can adversely and proportionately impact the
development of lung parenchyma as well as vasculature
causing pulmonary hypoplasia. CDH can occur along with
structural heart disease as part of a syndrome (e.g. Wolf
Hirschhorn syndrome, trisomy 18, vitamin A exposure),
the commonest being ventricular septal defect. Curiously,
a decrease in left ventricular wall as well as cavity

dimensions is also noted, possibly due to decreased filling,

but may also eventuate in obstructive left-sided lesions.
Sacrococcygeal teratoma is a pluripotential tumor
arising from the sacrococcygeal region. This is the
commonest tumor seen in the fetus, albeit rare and
its major cardiac impact is to cause high-output heart
failure and hydrops in the fetus. Serial evaluation of the
CVVO is indicated to decide on need and timing for fetal
intervention in the form of a cyst aspiration, laparoscopic
laser ablation, surgical debulking, or early delivery.

FUNDAMENTALS OF FETAL
CARDIAC IMAGING
Two-dimensional imaging is the principal imaging
modality for fetal heart disease detection. Basic ultrasound
principles apply to fetal imaging, although with many
additional levels of technical challenges as well as
opportunities. In general, a combination of obstetric and
pediatric cardiology skills is required. Flexibility, familiarity
as well as perseverance are essential to get as complete
and accurate a fetal cardiac examination as possible. The
small size of fetal cardiac structures as well as the relatively
faster fetal heart rate can challenge the spatial as well as
temporal resolution capabilities of the ultrasound system.
Accordingly, a higher frequency transducer, minimal
distance between the fetal heart and the transducer on
the maternal abdomen, appropriate depth and width
of the region of interest, and dynamic focusing are all
important considerations and need to be optimized. Since
axial resolution typically exceeds radial resolution, the
transducer can be moved on the maternal abdomen so as
to insonate the region of interest at minimal angulation.
Similar to the pediatric cardiac exam, each region of
interest needs to be seen in multiple views to get an accurate
impression. Again, similar to a pediatric cardiac exam,
a segmental completeness is necessary. Fetal position,
movement, and variable transducer positions make this a
literal moving target and while a protocolized progression
of sweeps and views cannot be realistically applied,
every attempt should be made to demonstrate as many
anatomical and functional components of each segment.
A lower frequency transducer, even a nonobstetric one
may be occasionally helpful in a later trimester gestation,
where more penetration is required. This strategy may also
be required in the rare event of an aliasing Doppler signal
with the usual obstetric transducer. Harmonic imaging
may enhance the bloodtissue interface and permit
better assessment in some challenging cases. In early

Chapter 71: Fetal Cardiac Imaging

gestation and while the pregnancy is still pelvic, the use

of endovaginal transducers can permit the best resolution
studies in the first trimester and may add information to
transabdominal scans.52,53 Guidelines have been published
for the basic and extended basic fetal cardiac scan and
the reader intending to formalize a fetal echo protocol is
encouraged to read them.54,55
By convention, the transducer notch is always pointing
toward the maternal left side. Even as multiple sweeps
will require clockwise and anticlockwise rotation, it is
important to keep in mind that the notch must be kept in
the left two quadrants to avoid confusion. The left/right
invert button may allow better pattern recognition in some
situations and readers, but, overall, inadvertent switching
can cause tremendous errors in situs assessments and
should be avoided or vigilantly supervised. A fetal cardiac
exam must begin with knowledge of the fetal indication,
obstetric findings, and other concerning extracardiac
anatomical findings in the fetus. The key elements are
quite comprehensive and include assessment of the
fetal number and position in the uterus. Determining
the abdominal and cardiac situs needs to be established
at the very beginning of the study. This can be done by
establishing the fetal presentation followed by the fetal left
and right sidethe use of a doll to illustrate the relative
location of a fetus in the uterus is often helpful to interpret
a uniplanar display. Another method that has been used
requires the fetal scan to demonstrate the fetal head to the
right of the screen. From this position, a 90 rotation of the
transducer will bring the fetal heart into a cross-sectional
view. Levocardia is interpreted from a fetal heart and apex
that are located and directed to the right of the fetal spine
in this position.56 Ideally, the fetal cardiologist should be
able to interpret both methods and even use both in each
case as a mechanism for cross checking. As part of this situs
sweep, the location of the heart and stomach on the left,
and inferior vena cava on the right establishes abdominal
as well as cardiac situs. The position of the heart within the
fetal chest may itself indicate potential abnormality if it is
located in the right chest, apex is pointed to the right side of
the fetus, or if the cardiac axis is abnormal. The fetal cardiac
axis is the angle between the plane of the interventricular
septum and the presumed midline and has been noted to
be 43 7.57 The cardiothoracic area should be under 40%
a relatively larger heart occurs in cardiac lesions such as
Ebsteins anomaly and a relatively larger lung component
is noted in severe pulmonary cystic lesions. The following
views comprise a complete exam, although of course they
may not be acquired in this order and may be incomplete

1531

Fig. 71.1: Cardiac situs, cardiothoracic ratio, and cardiac axis

determination. Fetal cardiology consult was requested due to
older sibling with tricuspid atresia. Gestational age is 28 3/7 weeks.
In the Cordes technique demonstrated, the fetal position within the
uterus is determined and a short axis of the fetal chest is obtained
with the fetal head on the right of the screen. Fetal heart and
cardiac apex to the right of the fetal spine in this technique indicate levocardia. Situs can also be cross-checked with the second
technique demonstrated in Figure 71.2. The cardiothoracic ratio
is determined as a ratio of the fetal heart compared to the thoracic area. In this example, it is 8.27/35.31 cm2 = 23% and normal.
The cardiac axis is the imaginary axis between the line joining the
fetal spine with the middle of the fetal frontal chest and the line
drawn along the plane of the interventricular septum. It is 30
in this example and normal. This Figure correlates with Movie
clip 71.1.

and need to be pursued in a fractionated manner

(Figs 71.1 to 71.37, Movie clips 71.1 to 71.26). It is helpful to
keep a mental checklist that allows a complete anatomical
and functional three-dimensional (3D) picture to form
while the study is being conducted or reviewed.

Four-Chamber View
This view is part of the obstetric standard for anatomy
scan. Some very important and critical cardiac lesions are
readily picked up in this view such as hypoplastic left or
right heart, mitral or tricuspid atresia, atrioventricular (AV)
canal defects balanced or unbalanced, double inlet left
ventricle, single ventricle type situations, and large septal
defects. While such a diagnosis may be striking at the very
onset of the examination, this view alone is inadequate
for a complete assessment. In more subtle situations of
ventricular or atrioventricular valve discrepancy, each of
these structures needs to be measured in the cardiac cycle
that reaches its maximal dimension and compared to
gestational age-based norms.5860 This view demonstrates

1532

Section 6: Congenital Heart Disease

Fig. 71.2: Situs determination and ventricular morphology. The

fetus is in vertex position. With the transducer notch toward the left
of the maternal abdomen, this image indicates the heart is within the
left chest. Situs can also be cross-checked with the second technique demonstrated in Figure 71.1. The descending aorta (DAo) is
seen in cross section to the left of the fetal spine. The left atrium is
the left-sided upper chamber, identified by the presence of a right
and a left-sided pulmonary vein entering it posteriorly (marked *).
The redundant foraminal membrane is seen to separate the right
and left atria and is usually bulging into the left atrium secondary to
right-to-left shunting at this level across the patent foramen ovale
(PFO). The primum component of the interatrial membrane is noted
to be intact. The morphologically right ventricle (RV) is noted with a
trabeculated apex. The left ventricle (LV) has a more rounded apex
with less trabeculations. The tricuspid valve (TV) and mitral valve
(MV) are seen on the right and left sides, respectively. This Figure
correlates with Movie clip 71.2.

Fig. 71.3: Inferior vena cava (IVC), hepatic veins (Hep V), and
ductus venosus (DV). The inferior vena cava is right-sided in this
fetus with levocardia. It is joined by the right and left hepatic veins
as well as the ductus venosus, which can be identified as a turbulent region on color Doppler along the course of the umbilical vein
to the IVC. These venous structures connect to the right atrium
(RA). This Figure correlates with Movie clip 71.3.

Fig. 71.4: Two-dimensional (2D) bicaval view. In this transverse

view, the fetal head is to the left of the screen and the fetal abdomen is toward the right. The inferior vena cava (IVC) receives the
hepatic veins (**) before joining the right atrium (RA). The right
superior vena cava (RSVC) is seen entering the RA from the cranial aspect of the fetus. The diaphragm separates the liver and
abdominal contents from the lung and the heart in the thorax. The
azygous vein (*) is seen joining the RSVC. (Movie clip 71.4).

Fig. 71.5: Color Doppler across interatrial membrane. The interatrial membrane region is marked * and indicates right-to-left shunting from the right atrium (RA) to the left atrium (LA) in this normal
fetus. (Movie clip 71.5).

Chapter 71: Fetal Cardiac Imaging

1533

Fig. 71.6: There is laminar color Doppler flow across the tricuspid
(TV) and mitral (MV) valves from the right (RA) and left atria (LA)
to their respective ventricles (RV, LV). There is no obvious shunting at the interventricular septal level. (Movie clip 71.6).

Fig. 71.7: Pulsed wave Doppler across the tricuspid valve showing
a wave dominance in this 28-week fetus. (Movie clip 71.7).

Fig. 71.8: Pulsed wave Doppler across the mitral valve showing in
this 28-week fetus. (Movie clip 71.8).

Fig. 71.9: Color Doppler of pulmonary veins. The pulmonary

venous connections are elicited by applying color Doppler in views
demonstrating the back of the left atrium and lowering scales until
they can be easily visualized and interrogated with pulse Doppler.
This Figure correlates with Movie clip 71.7. (DAo: Descending
aorta; LLPV: Left lower pulmonary vein; LUPV: Left upper pulmonary vein; PFO: Patent foramen ovale; RUPV: Right upper pulmonary vein). (Movie clip 71.9).

Fig. 71.10: Pulse Doppler of pulmonary vein. In this image, the right
upper pulmonary vein is being interrogated after it was identified on
color Doppler. Note that there is some contamination of the signal
with the right pulmonary artery signalseen as the sharp systolic
wave below the baseline. This coincidentally demonstrated the swave of the pulmonary venous Doppler, followed by the d-wave.
(Movie clip 71.10).

1534

Section 6: Congenital Heart Disease

Fig. 71.11: Short-axis mitral valve, interventricular septum, moderator band attachment. The left ventricle (LV) is identified by its
circular contour, presence of two papillary muscles and mitral valve
(MV), and smooth interventricular septum. The right ventricle is
the anterior chamber and its outflow is seen wrapping around the
left ventricle in a relatively oblong manner. The septal attachment
of the moderator band is identified. (Movie clip 71.11).

Fig. 71.12: Short axis of interventricular septum (IVS). Multiple

sweeps up and down of the interventricular septum need to be
performed to determine the ventricular morphology as well as look
for any obvious drop-outs suggestive of ventricular septal defects.
This Figure correlates with Movie clip 71.12.

Fig. 71.13: Color Doppler ventricular septum short axis. Color

Doppler is applied to images in Figure 71.12. The interventricular
septum is intact in this view. This Fig. correlates with Movie clip
71.13.

Fig. 71.14: Two-dimensional (2D) biventricular outflow views show

crisscrossing. From the four-chamberequivalent view of the
fetal heartsweeping anteriorly in the fetal chest brings out the left
ventricular outflow tract (marked in cross hatched line) and then
the right ventricular outflow tract (marked by continuous line) as
they arise from their respective ventricles. The outflow tracts are
noted to crisscross and this aspect is important to identify normal
outflow anatomy. This Figure correlates with Movie clip 71.14.

atrial as well as ventricular morphology and atrioventricular

concordance. A posterior sweep to the coronary sinus and
anterior sweep to the pulmonary valve will show critical
intervening cardiac structures such as septum primum,
inlet, membranous and muscular interventricular septum,

and bilateral AV valve morphology and function. The left

atrium is the most posterior structure and at least one rightand one left-sided pulmonary vein can be seen entering it
in this view, confirmed on color and spectral Doppler. The
coronary sinus can be seen as a small structure parallel

Chapter 71: Fetal Cardiac Imaging

1535

Fig. 71.15: Two-dimensional (2D) right ventricular outflow, pulmonary valve (PV). The right ventricle (RV) is the anterior chamber
and its outflow is seen wrapping around the left ventricle in a relatively oblong manner. The PV and main pulmonary artery (MPA)
are seen in this image. The aortic valve is seen at the crux of the
heart at the same level as the PV and is marked as X in this image.
This image correlates with Movie clip 71.15.

Fig. 71.16: Two-dimensional (2D) left ventricular outflow tract. The

left ventricle is identified. Sweeping slightly anteriorly in the fetal
chest and with some angulation to bring out the left ventricular
outflow tract, which is seen to be unobstructed and open in this
view. The aortic valve (AoV) and ascending aorta (Ao) are noted.
The right pulmonary artery is seen to traverse behind the ascending aorta. The membranous region of the interventricular septum
between the aortic valve and the tricuspid valve is additionally
seen in this particular image (*). This image correlates with Movie
clip 71.16.

Fig. 71.17: Color Doppler laminar flow across pulmonary

valve (X). (Movie clip 71.17).

Fig. 71.18: Color Doppler laminar flow across aortic valve ( )

and aortic arch (AoAr). (Movie clip 71.18).

to the posterior left atrioventricular groove, entering into

the inferior aspect of the right atriumobvious dilatation
can be seen in conditions where it receives a left-sided
superior vena cava (LSVC). An intact septum primum
can be critical to the diagnosis of an endocardial cushion
defect or atrioventricular canal defect (AVCD). A large
drop out in the interatrial membrane is readily obvious

and the bulging of thin foraminal rims into the left atrium
indicates the direction of shunting at this level from right
atrium to left atriumthis can be confirmed on color
Doppler in orthogonal views of the interatrial membrane.
The tricuspid valve and the right ventricle are mildly
dominant toward latter gestation, although both sides
appear relatively symmetric in earlier gestation. Defects

1536

Section 6: Congenital Heart Disease

Fig. 71.19: Pulse Doppler across pulmonary valve. (Movie clip

71.19).

Fig. 71.20: Pulse Doppler across proximal transverse aortic arch.

(Movie clip 71.20).

Fig. 71.21: Short axis of aortic valve (marked as X). The symmetric and trileaflet anatomy of the normal aortic valve is seen in this
image. This view is particularly useful to illustrate the morphology
of an abnormal aortic valve as annular hypoplasia, valve thickening, and asymmetry. The tricuspid valve (TV) leading to the right
ventricle and right ventricular outflow tract (RVOT) is noted. The
patent foramen ovale (PFO) is well profiled in this view. (Movie
clip 71.21).

Fig. 71.22: Two-dimensional (2D) short axis both semilunar

valves and right pulmonary artery (RPA). The pulmonary valve (P)
is more anterior and slightly larger. The main pulmonary artery and
its bifurcation into the right and left pulmonary arteries are noted
by cranial angulation from this view or by opening these vessels
out from the three-vessel view. The right pulmonary artery traverses behind the ascending aorta. (Movie clip 71.22).

in the inlet, membranous, muscular, and apical regions of

the interventricular septum may be evident in this view,
but all components of the interventricular septum should
be assessed in multiple views and specifically in views
perpendicular to the plane of the defect so as to minimize
drop out artifact due to parallel beam orientation.
Deviation 2 standard deviations or z score 2 compared
to gestation-based norms as well as serial evaluation of
ventricular major and minor dimensions are very helpful
in determining if one side is larger or smaller by itself or
merely appears to be so due to a smaller or larger other

side. This comparison has been found to be very useful

in situations of ventricular hypoplasia, unbalanced AV
canal defects, severe semilunar valve obstructions, fetal
coarctation and heart failure, or high-output situations.
Fractional area of shortening may be used to assess systolic
function. Color Doppler in this view demonstrates lack of
antegrade flow in AV valve atresia, valve incompetence,
VSD flow, and outflow directions. Spectral Doppler
should be used to ascertain the inflow pattern of the right
as well as left side as well as incompetence jets. Tissue
Doppler has been used in an attempt to analyze systolic

Chapter 71: Fetal Cardiac Imaging

1537

Fig. 71.23: Two-dimensional (2D) of ductal and aortic arches, isthmus (*). The ductal arch (DuAr) is anterior and describes a gradual
curve slightly rightward and posteriorly where it is met at the isthmus (*) by the more posterior and acutely angulated aortic arch
(AoAr). (Movie clip 71.23).

Fig. 71.24: Transverse aortic arch, and head and neck vessels.
The fetal head is to the left of this screen image. The transverse
aorta (TrAo) has been opened out to demonstrate each of the
head and neck vessels, the last one being the left subclavian
artery (LSCA). This image set is important to demonstrate head
and neck vessels as well as indicators or aortic arch anomalies.
The aortic isthmus is distal to the LSCA and needs to be clearly
visualized to rule out aortic coarctation. (Movie clip 71.24).

Fig. 71.25: Two-dimensional (2D) of three-vessel view and pulmonary artery (PA) bifurcation. The orientation of the fetal chest
is marked. The fetal spine (Sp) marks the posterior aspect. The
descending aorta (DAo) is seen just to the left and anterior of the
spine. The three vessels noted from left to right as well as anterior
to posterior are the PA, aorta (Ao), and right-sided superior vena
cava (RSVC) in that order of location as well as decreasing size.
Identification of the location as well as relative sizes of the great
vessels in this view is critical in detecting and confirming presence
of obstruction, size discrepancy as well as arch anomalies. The
right pulmonary artery (RPA) is seen to arise from the main PA and
traverses posterior to the aorta as well as RSVC as it heads to the
right lung hilum. (Movie clip 71.25).

Fig. 71.26: Two-dimensional (2D) features of the ductal arch.

The ductal arch is the continuation of the pulmonary artery after
it arises from the right ventricle (RV) and pulmonary valve (PV),
and after this vessel has given rise to the right pulmonary artery
(*) and left pulmonary artery (**). The ductal arch is the larger
of the two arches and dives posteriorly to join the aortic arch at
the aortic isthmus. In this Figure, the isthmus is not well profiled,
and that view will need to be developed along with the aortic arch
to establish anatomy as well as flow in the isthmic region. This
is important to predict fetal aortic coarctation. (Movie clip
71.26).

1538

Section 6: Congenital Heart Disease

Fig. 71.27: Two-dimensional (2D) features of the aortic arch.

The ascending aorta (AscAo) gives rise to the right innominate
(*), left common carotid artery (**), and the left subclavian artery
(***) before it joins the ductal arch at the aortic isthmus. (Moive
clip 71.27).

Fig. 71.28: Two-dimensional (2D) three-vessel view. In this additional example of a normal three-vessel view, the orientation of
the fetal chest is marked. The fetal spine (Sp) marks the posterior
aspect. The descending aorta (DAo) is seen just to the left and
anterior of the spine. The three vessels noted from left to right as
well as anterior to posterior are the pulmonary artery (PA), aorta
(Ao), and right-sided superior vena cava (RSVC) in that order of
location as well as decreasing size. Identification of the location
as well as relative sizes of the great vessels in this view is critical
in detecting and confirming presence of obstruction, size discrepancy as well as arch anomalies. The right pulmonary artery (RPA)
is seen to arise from the main PA and traverses posterior to the
aorta as well as RSVC as it heads to the right lung hilum. (Movie
clip 71.28).

Fig. 71.29: Two-dimensional (2D) evaluation of the aortic isthmus.

The aortic outflow in the crux of the heart is marked (**) and gives
rise to the aortic arch. The head and neck vessels are seen arising from the top of this arch. The isthmus is seen as the region
after the left subclavian opposite the insertion of the ductus
arteriosus (*).

Fig. 71.30: Pulse Doppler across ductus venosus.

Chapter 71: Fetal Cardiac Imaging

Fig. 71.31: Pulse Doppler across inferior vena cava (IVC).

Fig. 71.32: Pulsed wave Doppler of hepatic vein.

Fig. 71.33: Pulse Doppler across umbilical artery.

Fig. 71.34: Pulse Doppler across umbilical vein.

Fig. 71.35: Umbilical cord vessels. The umbilical vein (*) is relatively thin walled and of larger caliber. There are two umbilical
arteries (-) that are thicker walled and of smaller lumen.

1539

and diastolic function of both ventricles,61,62 although

this tool has not been fully explored secondary to the
numerous unknown factors that can impact it and also
lack of robust interobserver variability. From this nodal
four-chamber view, a more anterior tilt will bring out the
left ventricular outflow. The aortic valve can be well seen
and measurements made in this view. Outflow tract as
well as aortic valve issues can be seen in this view and
further interrogated with color as well as spectral Doppler.
The left ventricular cardiac output can be calculated by
determining the systolic dimension of the aortic annulus
and the velocity time integral of the transaortic pulsed
wave Doppler. On further tilting toward the anterior fetal
chest, the right ventricular infundibulum is seen to lead
to the pulmonary valve. Crisscrossing of the great arteries
with the pulmonary artery being the more anterior and
leftward is reassuring for normal great artery relationship.

1540

Section 6: Congenital Heart Disease

Fig. 71.36: Situs sweep in this 24-year-old Gravida 2 referred due

to maternal history of aortic stenosis. This echo was performed at
31 1/7 week gestation. The reader is basically oriented with the
use of labels as the Cordes technique is used to determine situs.
The horizontal bar (/) indicates that the more apically displaced
tricuspid valve is located on the right sidethis is the normal tricuspid valve leading to the morphological right ventricle. (Movie
clip 71.36).

Fig. 71.37: Two-dimensional (2D) ductal and aortic arches. The

ductal arch (DuAr) is anterior and describes a gradual curve
slightly rightward and posteriorly where it is met at the isthmus ( )
by the more posterior and acutely angulated aortic arch (AoAr).
(Movie clip 71.37).

Loss of this relationship will be obvious in other views

and allows diagnosis of malposition syndromes. However,
semilunar valve function may not be easily separated in
this view.
Short-axis views are possible in multiple planes
perpendicular to the long-axis view. A central reference
image is of the right ventricular outflow tract as it wraps
around the left ventricle. Ventricular septal defects with
outlet or conal extension can be seen in this scan-plane
and deviation of the conal septum can be appreciated in
this view. Presence of conal deviation, even in the absence
of significant outflow obstruction, merits serial follow-up
to monitor for in utero progression to a potential ductaldependent situation. Such progression of intracardiac
findings has been shown in tetralogy of Fallot (TOF) as
well as hypoplastic left heart syndrome (HLHS).6368
A common underpinning of such in utero progression
is a commonly believed concept of antegrade flow being a
stimulus for growth of valves and cavities. Slight leftward
angulation from this view will bring the pulmonary valve,
main pulmonary artery, right and left branch pulmonary
arteries as well as ductal arch into view. A more cephalad
sweep with some rightward angulation will develop the
three-vessel view in the superior mediastinum of the
fetus. The main pulmonary artery, aorta, and right-sided
superior vena cava are lined as in decreasing order of
size from the leftward and anterior most location (PA) to

the most rightward and posterior location (RSVC). This

view can provide insight into flow discrepancies in the
subpulmonic and subaortic ventricle, in the respective
semilunar valves, or in the great arteries.69 For example,
the three-vessel (3V) view in TOF will demonstrate a
smaller and mildly posteriorly (and cranially) displaced
pulmonary artery. A smaller aortic dimension is seen with
HLHS and its variants. Bilateral SVC is suspected if there is
another circular signal to the left of the pulmonary artery
and at the same anteroposterior level as the RSVC. Various
aortic arch anomalies including coarctation, interruption,
double aortic arch, and vascular slings can also be
appreciated in this view. If the PA and aorta are opened out
in this view, these vessels will be seen to dive posteriorly as
they transit into the ductal and aortic arches, respectively.
Once again, this view is a visual demonstration of expected
size proportion. The ductal and aortic arches meet in
the aortic isthmus and measurements as well as color
and Doppler here can raise concern for coarctation if
the isthmus is narrowed or if there is flow turbulence or
increased velocity across this region. Also, if there is an
abnormal flow pattern in either arch, it will be readily
noted on color Doppler, although the size discrepancy
in severe stenosis situations can make it difficult to open
these two arches in single view consistently. Focusing on
the aortic arch, the sidedness can be determined based on
the direction of a left-sided arch diving from a rightward

Chapter 71: Fetal Cardiac Imaging

anterior location to its leftward posterior location to the

left of the trachea. In most of these cephalad planes, the
thymus can be seen in the anterior mediastinumits
hypoplasia along with a conotruncal lesion may indicate
22q11 deletion spectrum.
Ventricular long-axis view is obtained by aligning the
transducer along the left ventricular outflow. Ventricular
septal defects, aortic override (pathognomonic of TOF),
aortomitral continuity, goose-neck deformity (pathognomonic of AVCD), mitral and aortic valve sizes as well as
ventricular morphology can be well seen in this view. Fetal
noncompaction of the myocardium can be a controversial
diagnosis in early gestation but may be remarkable enough
to appear convincing in later gestation. Left ventricular
basal wall function as well as overall systolic function
can be assessed. Unusual morphologies such as left
ventricular diverticulum and fetal rhabdomyomas may be
well delineated in this view. Spectral Doppler in this or the
four-chamber view with the pulsed wave Doppler gates
across the mitral as well as aortic valves can be optimized
to show the inflowoutflow Doppler to calculate. This
is an accepted method to determine the mechanical
PR interval in maternal systemic lupus erythematosus
or Sjogrens syndrome, where there is risk for fetal heart
block, to calculate isovolumic relaxation time and the fetal
Tei index.61
Caval long-axis view lines up the inferior vena cava
(IVC) as well as SVC entering the right atrium. From
this view, the right pulmonary artery passing behind the
RSVC and sometimes a prominent azygous vein entering
the RSVC can be appreciated. A dilated RSVC may be an
indicator of an arteriovenous malformation in the head
and neck region, commonly in the cerebral territory.
Such a finding may also raise suspicion for anomalous
pulmonary venous drainage and should be followed by
attempts to delineate at least three of the pulmonary veins.
At this level, the IVC as well as the hepatic vein should be
interrogated by spectral Doppler and as the IVC is traced
further to ensure that it is uninterrupted. An interrupted
IVC is a marker for heterotaxy syndrome, usually of the
left atrial isomerism type. From this view, color Doppler
over the liver region can identify the ductus venosus as a
narrowing in the course of the umbilical vein to the IVC.
Once identified on color Doppler, spectral Doppler is used
to show the flow pattern in this structure.
Ductal and aortic arch views are best developed
perpendicular to the three-vessel view. The aortic arch is
higher and describes a wider arch, likened to the candy
cane. It is identified by the origin of the head and neck

1541

vessels from the top of the curve. The ductal arch describes
a tighter curve and is described as a hockey stick as it
joins the aortic arch. Sweeping back and forth amidst
these two arches allows separation of these arches. A
proportionate size, as well as laminar antegrade flow on
color Doppler are reassuring for preserved fetal physiology
and right-to-left shunting at the ductal level. Resistance
and pulsatility indices can be calculated and are valuable
indicators to differentiate increased flow across the
ductus (increased right ventricular output) from ductal
constriction (due to maternal nonsteroidal ingestion).70,71
A size discrepancy needs further assessment. In ductaldependent systemic circulations, there is antegrade flow in
the ductal arch, possible abnormal flow in the isthmus, and
potentially retrograde flow in the transverse aortic arch. In
ductal-dependent pulmonary circulation, the ductus is
abnormally angulated and arises more vertically from
the undersurface of the aortic arch, and has retrograde
flow indicating that even in the fetal stage, pulmonary
circulation is dependent on a retrograde ductus. Identifying
both these entities is critical as it has ramifications on fetal
counseling, preparing a more detailed birth plan including
initiation of prostaglandins soon after birth.

Fetal Cardiac Function

Left ventricular shortening fraction can be calculated
using either two-dimensional images or M-mode
interrogation at the level of the papillary muscles on the
left side and at midlevel in the right side. Normal left and
right ventricular shortening fraction is 34% 3% after 17
weeks gestation.72 Poor reproducibility of modified biplane
Simpson technique-based ventricular ejection fractions
contrasts with the better reproducibility and variability
in three- and four-dimensional (4D) measurements of
ventricular volumes and mass.7375 A cardiovascular profile
score has been widely used to reflect fetal cardiac status.
This composite score gives 2 points each for presence or
absence of hydrops, Doppler pattern in the umbilical vein
and ductus venosus, heart size based on cardiothoracic
ratio, cardiac function based on inflow pattern, AV valve
incompetence and shortening fraction, and finally
Doppler pattern in the umbilical artery.76
Across both mitral and tricuspid valve, the E-wave is
smaller and the A-wave is dominant and the E/A ratio is
usually <1, presumably due to the reduced compliance
of the ventricular myocardium toward the end of the first
trimester. As pregnancy progresses, improved ventricular
compliance is manifested as increased E-wave and
increasing E/A ratio across both valves.7781 Similar to

1542

Section 6: Congenital Heart Disease

the postnatal and adult experience, a fetal myocardial

performance index can be derived based on inflow and
outflow Dopplers. A higher myocardial performance
index indicates a greater amount of dysfunction, although
it may not separate out systolic from diastolic dysfunction.
This Tei index can be compared with recently published
norms and is shown to be relatively stable across gestation
(mean MPI = 0.36, range 0.280.44).61,8284 The fetal
isovolumic relaxation time is the interval between closure
of the semilunar valves and opening of atrioventricular
valves with a mean value of 34 ms (range is 2641 ms).82,85
The greatest utility of these parameters may be in the
longitudinal follow-up in a particular fetus with cardiac or
extracardiac pathology affecting this parameter.

Core and Cord Dopplers

This term describes the spectral Doppler pattern in
the umbilical artery, umbilical vein, ductus venosus,
IVC, hepatic vein, and middle cerebral artery. Venous
structures closest to the heart are the first to manifest
changes secondary to increased filling pressures. Both the
hepatic vein and the IVC show a triphasic pattern similar
to the s, d, and a wave pattern seen in mature venous
systems. Prominent a-wave reversal indicates elevated
filling pressures, low velocity s-waves indicate presence
of important tricuspid incompetence. It is recommended
to acquire umbilical artery (UA) and umbilical vein (UV)
signals in a free loop of umbilical cord midway between the
placenta and the fetus. The umbilical venous flow carries
blood from the placenta to the fetus and is a low velocity,
continuous, nonphasic flow. Appearance of notching
with decrease in centripetal flow during the cardiac cycle is
abnormal. The umbilical artery carries fetal deoxygenated
blood from the fetus to the placenta. The low placental
vascular resistance allows systolic as well as diastolic flow,
which can be quantified using the pulsatility index. This
index is elevated in intrauterine growth retardation and in
the donor of TTTS. Middle cerebral artery is interrogated
midway after its origin from the circle of Willis and the
lateral cranium. Unlike the UA, there is minimal diastolic
flow in this vessel because of elevated cerebrovascular
resistancein situations where the fetus is adapting to
a poor circulatory situation by decreasing its vascular
resistance; there is more diastolic flow in this artery.86
Accompanying case studies illustrate all the above
principles, multiple imaging views, and diagnostic information in a variety of anatomical situations. (See Figs

71.38 to 71.93 and the corresponding movie clips, and also

Movie clip 71.94).

Fetal Cardiac Rhythm Assessment

Rhythm assessment is integral to a screening echocardiogram. Although the primitive heart tube demonstrates
organized contractions by 12 days postconception, the
conduction system is developed by 16 weeks of gestation,
later than the fetal cardiac structures at 12 weeks.87 Fetal
heart rate varies through gestation, ranges about 110 to
180 beats/minute (bpm) in the first trimester, maximum
rate achieved at 9 weeks gestation, and averaging about
135 bpm (110150 bpm) by later trimesters.88,89
Pulse Doppler or M-mode techniques can be used
to detect the rate of cardiac events. To determine the
atrioventricular synchrony, simultaneous inflowoutflow
Dopplers such as SVC and aorta, mitral valve and aorta,
pulmonary artery and pulmonary vein, and M mode
through atrial as well as ventricular structures can to be
analyzed.90,91 Unfortunately, all these techniques require
persistence and skill due to challenges from fetal position,
fetal movement, and image resolution. Fetal arrhythmias
and premature beats can be quite striking at the time of an
obstetric scan, but the vast majority of referrals for reasons
of fetal arrhythmia reveal premature atrial beats or no
rhythm issues.92 Premature atrial contractions are 10 times
more common than premature ventricular contractions;
both have a combined occurrence of about 1.7% in third
trimester studies.93 The relationship between atrial and
ventricular contractions and their respective rates usually
allows identification of supraventricular tachycardia
(SVT), atrial flutter, junctional ectopic tachycardia, and
ventricular tachycardia. Fetal SVT with 1:1 atrial (A):
ventricular (V) contraction is the commonest at 6690% of
all fetal tachyarrhythmia and is the cause of nonimmune
hydrops in many. These can be further divided into short
and long V-A types.91 The ventricular rate is typically 250
bpm, and there is 1:1 A:V relationship. The latter feature
separates fetal SVT from the second commonest fetal
tachyarrhythmia, which is atrial flutter.
In atrial flutter, atrial rates may range from 350
500 bpm, eventual ventricular rate depending on AV
conduction. Rapid ventricular rates without V-A synchrony
suggest ventricular and junctional ectopic tachycardia.
Besides meticulous rhythm studies in affected fetuses,
the structure of the fetal heart must also be assessed in
detail since structural defects can coexist in an important
number. Tachycardias are described as sustained if

Chapter 71: Fetal Cardiac Imaging

1543

Case VSD 1. Fig. 71.38: Ventricular septal defect in four-chamber

view. The right and left atria (RA, LA) and ventricles (RV, LV) as
well as respective atrioventricular valves are labeled (TV, MV).
A drop-out (*) is noted at the crest of the interventricular septum in
this image and is suspicious for a ventricular septal defect. (Movie
clip 71.38).

Case VSD 1. Fig. 71.39: Two-dimensional (2D) ventricular long

axis with ventricular septal dropout in high muscular region.
(Movie clip 71.39).

Case VSD 1. Fig. 71.40: Color Doppler ventricular long axis with
ventricular septal dropout in high muscular region confirms the
presence of a septal defect in this region. (Movie clip 71.40).

Case VSD 1. Fig. 71.41: Three-vessel view (3VV) with Bilateral

superior vena cava (BLSVC). The ductal arch (DuAr) and aortic
arch (AoAr) are appropriate in their relative orientation as well as
size. However, there are two good-sized bilateral superior vena
cava [right-sided superior vena cava (RSVC) and left-sided superior vena cava (LSVC)] noted in this view. (Movie clip 71.41).

Case VSD 1. Fig. 71.42: Postnatal confirmation of high muscular

ventricular septal defect (VSD). (Movie clip 71.42).

1544

Section 6: Congenital Heart Disease

Case VSD2. Fig. 71.43: Midmuscular ventricular septal defect

(VSD; *). This defect is suspected in the muscular septum just
proximal to the moderator band in the right ventricle. Note that in
this instance, the angle of insonation is parallel to the drop-out and
suspected defect.

Case VSD 2. Fig. 71.44: Orthogonal view of midmuscular ventricular septal defect (VSD). This is the same patient and imaging
session as in Figure 71.1. In this image, the previously suspected
ventricular septal defect is confirmed on color Doppler as well as a
nearly perpendicular angle of insonation.

Case AVCD. Fig. 71.45: Four-chamber identification of atrioventricular canal components. A fetal echocardiogram was performed
for this 38-year-old G6P4 with Marfan syndrome and a fetus at 28
5/7 weeks gestation with trisomy 21 confirmed on amniocentesis.
The left ventricle is left-sided and on the left of the screen. The
right ventricle is identified with its apical trabeculations. A large
atrial septal defect including the septum primum (**) is seen along
with a large ventricular septal defect (*). The common atrioventricular valve is seen to sit astride this large defect in the crux of
the heart.

Case AVCD. Fig. 71.46: Atrial morphology and atrioventricular

canal defect (AVCD). The right atrial appendage is broad-based
triangular and with dense pectinate musclesthis identifies
the right atrium and is seen in this image as a. The left atrial
appendage is smaller, thin, and finger-like and has fewer pectinate
musclesseen in this image on the left side and labeled l. The
pulmonary veins are seen to enter into the back of the left atrium.
The large common atrium communicates via a common atrioventricular valve with the ventricles distally. The ventricular septal
defect (VSD) is noted (*).

they occur for >50% of the study period, otherwise they

are considered nonsustained. Functional parameters
including ventricular dimensions, cardiothoracic ratios,
presence and severity of AV valve incompetence, output,
cord and core Dopplers, and presence of hydrops need to
be serially collected and interpretedthese are invaluable

to decide on when to start maternal medications and also

to follow up the effect of the rhythm disturbance as well
as medication. Most premature beats and nonsustained
tachycardias can be monitored without intervention while
fetal well-being is monitored. Oral maternal medications
in th e form of digoxin, flecainide, sotalol, terbutaline, and

Chapter 71: Fetal Cardiac Imaging

Case AVCD. Fig. 71.47: Short-axis view of common atrioventricular (AV) valve demonstrates the single valve, and its anterior and
posterior leaflets as well as right and left components. The anterior
leaflet has connections to the crest of the interventricular septum
in this example, indicating that it is likely to be Rastelli type 1. The
pulmonary valve (PV) is noted anteriorly and is of a good size.

Case AVCD. Fig. 71.49: Three-vessel viewdiscrepancy and

left-sided superior vena cava (LSVC). The reader is encouraged
to orient the structures and note that the three-vessel view is abnormal. Firstly, the aorta (Ao) is much smaller than the pulmonary
artery (P). Secondly, there is a left-sided structure in the same
plane as the right-sided superior vena cava and of equivalent size
on the left of the pulmonary arterythis indicates the presence of
the left SVC as also demonstrated in Figure 71.4. The relative disproportion is concerning for left ventricular outflow tract or aortic
abnormalities or for smaller aortic arch. The ascending as well as
transverse arch measured small, and the last head and neck vessel seemed to be directed toward the fetal neck with no continuity
with the remaining ductal arch. This anatomy was suspicious for
interrupted aortic arch.

1545

Case AVCD. Fig. 71.48: Left-sided superior vena cava (LSVC)

to CS, smaller aorta. The LSVC is noted to drain into the right
atrium and the roof of the coronary sinus appears to be intact on
this two-dimensional (2D) image. This was confirmed on color
Doppler. The aorta (X) is seen in the crux of this view and appears
to be smaller than the anterior pulmonary valve.

amiodarone are important decisions that allow rate as well

as rhythm control but have important implications for the
mother as well as fetus.9498
Fetal bradycardia is diagnosed when the ventricular
rates are < 100 to 110 bpm. These low heart rates may
occur transiently and with no adverse outcome secondary
to vagal stimulus. More persistent bradycardia is an
indicator of a compromised fetus. CCHB with overall
ventricular rates of 30 to 120 bpm can arise due to
maternal autoimmune illnesses such as systemic lupus
erythematosus (SLE) and Sjgrens syndrome and also
from fetal structural heart disease such as heterotaxy
syndromes (left atrial isomerism), l-looped ventricles,
rarely idiopathic. CCHB due to maternal autoantibodies
anti-Ro (anti-SSA), and anti-La (anti-SSB) is presumably
due to affinity of these transplacentally transmitted
antibodies to the fetal conduction system and rarely to
the myocardium. First and second degree heart block
are amenable to diagnosis and treatment with maternal
steroids; third degree heart block is generally thought to
be irreversible. Stabilization and even reversal of first and
second degree AV block with maternal fluorinated steroids
has resulted in this being the treatment of choice. Others
have reported additional improvement in outcome with
maternal use of intravenous immunoglobulins, especially
if the myocardium is also affected.99103 Fetal bradycardia
can also be seen in long QT syndrome.104

1546

Section 6: Congenital Heart Disease

Case Dextrocardia. Fig. 71.50: In this situs sweep, the cardiac

apex is directed toward the right of the fetal chest. This fetus was
referred due to abnormal obstetric scan and this study was performed at 26 3/7 weeks gestation. The fetus is in breech position.

Case Dextrocardia. Fig. 71.51: Situs sweep with dextrocardia and

right-sided stomach. This indicates that the heart as well as the
stomach are located in the right side of the body, that is, cardiac as
well as abdominal situs inversus. (Movie clip 71.51).

Case Dextrocardia. Fig. 71.52: Asymmetric four-chamber view,

atrioventricular canal defect (AVCD). A common atrioventricular valve is suspected accompanied by an atrial and ventricular
septal defect (*). Left-sided atrium appears to have the right atrial
appendage (RAAp) and hence the right atrium. The left atrium is
right-sided and receives the pulmonary veins. The right-sided left
ventricle appears to be smaller than the other ventricle. (Movie
clip 71.52).

Case Dextrocardia. Fig. 71.53: Pulmonary veins to right-sided left

atrium. The right and left pulmonary veins are seen to join the back
of the right-sided left atrium in this color Doppler image. (Movie
clip 71.53).

Safety of Fetal Ultrasound

ultrasound energy can be theoretically secondary to its

thermal effect relating to increase in temperature in the
region of insonation, or mechanical, relating primarily to
cavitation. Most ultrasound systems allow for display of
potential increase in temperature in the field by displaying
the thermal index for either soft-tissue (TIS), or bone
(TIB). The TI represents an estimate of the temperature
rise in the field and is approximately proportional to

Prolonged and/or repeated fetal ultrasound is sometimes

required in fetal diagnosis. Despite theoretical concerns,
no confirmed harmful effects have been detected over
the years. The use of each imaging modality is associated
with ultrasound energy expenditure, most with the use of
color Doppler in a small region of interest. Bioeffects of

Chapter 71: Fetal Cardiac Imaging

1547

Case Dextrocardia. Fig. 71.54: Double outlet right ventricle. In this

image, the left-sided right ventricle gives rise to both the great
arteries. There is some deviation of the conal septum, so that
this protrudes below the pulmonary valve. The pulmonary valve
appears to be smaller than the aortic valve (identified as the nondividing vessel). The pulmonary artery (PA) is identified as the
vessel that bifurcates distally. The aorta is anterior and larger than
the pulmonary artery. This great artery relationship should be
confirmed in other views. (Movie clip 71.54).

Case Dextrocardia. Fig. 71.55: Smaller pulmonary artery and bifurcation. The right pulmonary artery (RPA) is red on color Doppler
as it moves toward the transducer; the left pulmonary artery (LPA)
is blue as it moves away. The main pulmonary artery (MPA) is of
smaller caliber than the aorta (Ao) that is directed leftward from its
anterior position. (Movie clip 71.55).

Case Dextrocardia. Fig. 71.56: En face view of unbalanced CA

valve. The left-sided right ventricle (RV) is more dominant. The
smaller right-sided left ventricle (LV) is seen posterior and right of
the RV. The common atrioventricular valve is seen on short axis in
this view. (Movie clip 71.56).

Case Dextrocardia. Fig. 71.57: Abnormal three-vessel view with

aorta anterior and leftward. The aorta is most anterior (**); a
smaller pulmonary artery is seen to its right and more posteriorly.
Bilateral superior vena cava (SVC) are once again suspected in
the same plane (*).

the temperature increase in degree Celsius. The risk of

mechanically induced ultrasound damage is displayed by
the mechanical index (MI), which is defined as the ratio
of maximal peak rarefactional pressure to the square root
of the ultrasound frequency. The risk of mechanical injury
rises with increasing MI.105107 As newer modalities such

as Doppler applications assessing tissue motion and realtime 3D imaging continue to develop, bioeffects on the
fetus will need to continue to be monitored. As there are
no strictly defined limits established, use of ultrasound
energy in fetal echocardiography is best expressed by the
ALARA principleas low as reasonably achievable.108

1548

Section 6: Congenital Heart Disease

Case TA. Fig. 71.58: Four-chamber asymmetry. This four-chamber equivalent view demonstrates asymmetry in ventricular chamber size, the right-sided right ventricle (RV) is smaller, and its apex
does not reach up to the cardiac apex. The tricuspid valve above
it seems to have plate-like atresia between the right atrium (RA)
and RV. A large ventricular septal defect (*) is seen separating
the smaller right ventricle from the larger and apex-forming left
ventricle (LV). The mitral valve (MV) is seen in its open diastolic
position. The interatrial foraminal membrane is seen bulging into
the left atrium (LA), suggesting that there is right-to-left flow at this
level. (Movie clip 71.58).

Case TA. Fig. 71.59: d-malposition of the aorta. The aorta (Ao)
is identified due to its relationship with head and neck vessels.
This vessel arises from the anterior and rightward bulboventricular chamber (BVC). Blood enters the BVC through a currently
unrestrictive ventricular septal defect (VSD) from the left ventricle
(LV). There is no aortomitral continuity and the aorta is of good
size. (Movie clip 71.59).

Case TA. Fig. 71.60: d-malposition of the great arteries. The

pulmonary valve (PV) leads to a good-sized pulmonary artery
that is seen to bifurcate distally. This great vessel is seen to arise
from the left ventricle (LV). The ventricular septal defect (VSD; *)
connecting this left ventricle to the bulboventricular chamber is not
well profiled in this view. (Movie clip 71.60).

Case TA. Fig. 71.61: Origin of malposed great arteries, subpulmonic ventricular septal defect (VSD). The aorta (Ao) is anterior,
smaller, and arises from the anterior diminutive right ventricle
(RV). The pulmonary artery (P) arises posteriorly from the larger
and dominant left ventricle (LV). This indicates ventriculoarterial
discordance. A subpulmonic VSD (*) and its relationship to the
great arteries is seen clearly. Note that the subpulmonic conus
is minimally thickened and deviated into the subpulmonic region.
(Movie clip 71.61).

Chapter 71: Fetal Cardiac Imaging

1549

Case TA. Fig. 71.62: Abnormal 3VV in DTGA. The fetal spine (Sp)
and anterior aspects are marked. The aorta is rightward and anterior to the pulmonary artery (d-malposed). The right pulmonary
artery (RPA) origin from the pulmonary artery (PA) is seen before
this vessel continues as the ductal arch (DuAr) to the descending
aorta (DAo). (Movie clip 71.62).

Case TA. Fig. 71.63: DTGA, anterior aorta bulboventricular chamber. The reader is encouraged to use the previous images to determine the anatomy in this labeled image. (Movie clip 71.63).

Case HLHS. Fig. 71.64: Asymmetry of the ventricles. A significant

fetal structural heart defect is suspected in the early part of this
scan due to the obvious asymmetry of the ventricles. The right
atrium (RA) and right ventricle (RV) are identified to be of adequate size. The left ventricle (LV) cannot be easily opened out and
the atrioventricular valve above it appears plate-like and does not
open well. A hypoplastic left heart variant is suspected in this image. (Movie clip 71.64).

Case HLHS. Fig. 71.65: Bicaval view showing foraminal flap. The
superior (SVC) and inferior (IVC) vena cavae are seen to enter
the right atrium in this bicaval view. The foraminal membrane is
seen with the crest of the flap directed toward the right atrium (*),
indicating left-to-right flow at this level. Normal atrial level shunting
in the fetus is always from the right to the left and presence of an
opposite direction of flow indicated impaired left atrial drainage
from anatomical or functional causes. (Movie clip 71.65).

Three-Dimensional Imaging

two planes, a 3D data point is called a voxel. This comprises

information in all three dimensions. An immense
advantage of 3D fetal scanning is that postprocessing
can allow navigation within this data set to yield virtual
and customizable scan planes that can better depict

3D and 4D (3D + fourth dimension of time) fetal echocardiography requires acquisition of a volume of imaging
data. Just as two-dimensional data is defined as a pixel in

1550

Section 6: Congenital Heart Disease

Case HLHS. Fig. 71.66: Color Doppler showing left-to-right (LA

to RA) shunting at the atrial level (*). This image correlates with
Movie clip 71.66.

Case HLHS. Fig. 71.67: Asymmetric three-vessel view. The

pulmonary artery is dominant and anterior. A smaller aorta is seen
to its right, followed by a right-sided superior vena cava. The
pulmonary artery goes on to bifurcate and the right pulmonary
artery (RPA) continues behind the aorta. The transverse aorta is
suspected to be the diminutive vessel that is headed from the
ascending aorta (Ao) toward the leftward aspect of the fetus.
(Movie clip 71.67).

Case HLHS. Fig. 71.68: Color Doppler of both arches. Further

cranial sweep from the location in Figure 71.4 reveals the aortic
and ductal arches. On applying color Doppler, there is antegrade
flow through the ductal arch. The point (*) is the isthmus and there
is retrograde filling of the aortic arch from this region (red flow
toward the transducer). Both arches should be antegrade and
symmetric. Presence of asymmetry is a subtle clue to possible hypoplasia, but retrograde flow indicates severe obstruction of one
of these arches and is a ductal-dependent pulmonary or systemic
circulation at birth. (Movie cilip 71.68).

Case HLHS. Fig. 71.69: Color Doppler showing retrograde transverse aortic arch flow. This image is derived by rotation of the
transducer from the position that demonstrated Figure 71.5. Here
the aortic arch is identified by the head and neck vessels, and the
transverse aorta (AoAr) is seen to fill retrograde from the isthmus
and the antegrade ductal arch (DuAr). (Movie clip 71.69).

anatomy as well as relationships between intracardiac

and extracardiac structures. When acquired over time
and multiple events in one or more cardiac cycles (STIC
= spatiotemporal image correlation and other 4D imaging

terminology) it is considered 4D ultrasound. This modality

acquires a series of B-mode images and stacks them
up to result in a data volume across a cardiac cycle.
Such a volume may potentially contain all the imaging

Chapter 71: Fetal Cardiac Imaging

1551

Case HLHS. Fig. 71.70: Spectral Doppler of retrograde aortic arch

flow shows that the flow is coming anteriorly and toward the aortic
root rather than the normal flow posteriorly and away from the
aortic root.

Case HLHS. Fig. 71.71: Retrograde arch flow on power color

Doppler. Power color Doppler can be used to bring out color flow
phenomenon to advantage. (Movie clip 71.71).

Case HLHS 2. Fig. 71.72: Four-chamber viewsmaller left ventricle (LV) and mitral atresia. In this case, a large and hypertrabeculated right ventricle (RV) contrasts with the smaller, non-apexforming LV. The smaller left atrium (LA) is separated from the
larger right atrium (RA) by a slightly thickened interatrial septum
with a central defect; its edges are bowing into the right atrium.
(Movie clip 71.72).

Case HLHS 2. Fig. 71.73: Four-chamber view with no color flow

across mitral valve. In this image, while there is antegrade flow
across the tricuspid valve into the right ventricle, there is no flow
across the mitral valve into the left ventricle in the presence of
plate-like mitral atresia. (Movie clip 71.73).

information that is required for fetal cardiac screening.109,110

Volume reconstruction and rendering are postprocessing
applications that allow manipulation of the data set to extract
relevant information and display. For example, volume
rendering of a data set with a fetal ventricular septal defect
will demonstrate the location, rims, and extent of the defect.
Color flow Doppler has been used as a way of mapping
flow in the fetal heart and a combination of 3D and 4D

technologies with color Doppler may be a promising tool

for multiplanar display of flow phenomenon.111,112
With the recent availability of live 3D scanning, this
technology has been utilized in assessing the fetal heart.113
B-Flow is a nonDoppler-based imaging technology
by digital encoding of emitted ultrasound beams into
two sub-beams. One of these beams is a high frame rate
B-mode display of color flow that is enhanced to increase

1552

Section 6: Congenital Heart Disease

Case HLHS 2. Fig. 71.74: Color flow across large apical ventricular septal defect (VSD). This is the only source of blood flow into
the left ventricle from the right ventricle. (Movie clip 71.74).

Case HLHS 2. Fig. 71.75: Color Doppler, mitral atresia, and

left-to-right flow at patent foramen ovale (PFO). (Movie clip
71.75).

Case HLHS 2. Fig. 71.76: Color contrast in pulmonary veins and

patent foramen ovale (PFO). This image illustrates the complexity as well as variable directionality of multiple flows in a small
region of interest. Careful attention will allow interpretation and the
information thus gathered should appear to be internally consistent and unifying toward the anatomy and physiology. (Movie clip
71.76).

Case HLHS 2. Fig. 71.77: Asymmetric 3VV in mitral atresiaclue

for arch hypoplasia. The large ductal arch is a stark contrast to the
much smaller and tortuous aortic arch. (Movie clip 71.77).

signal return from normal flow phenomenon. As a result,

the lumen dimensions as well as overall cardiac flow
phenomenon can be easily visualized. Some investigators
have found it to be useful to measure vessel dimensions,
others have found it to be highly sensitive to vessel
anomalies such as transposition of the great arteries.114116
Power Doppler and high definition power Doppler
are used in conjunction with 3D static imaging or STIC
imaging, respectively. They operate at lower velocities and
enhance visualization of flow phenomenon with color.117

They have been found to be particularly useful in first and

early second trimester scans.118
Fetal cardiac interventions are intended to normalize
the physiological disturbance that has come about in the
developing fetus because of a structural problem. This
stems from the observation that improved flow across
valves and structures improves their size and function
as also the observation that these techniques are both
feasible and bear good results. Theoretically at least, it is
possible to improve the anatomy of the developing heart

Chapter 71: Fetal Cardiac Imaging

1553

Case HLHS 2. Fig. 71.78: Aortic arch deceptively normal in long

axis. On this static image, it almost appears as though the aortic
arch is being demonstrated and appears to be of good size. In
fact, the left subclavian artery is arising from the top of the ductal
arch where it meets the diminutive aortic arch. The aortic arch is
antegrade due to the flow coming into it from the apical ventricular
septal defect (VSD). However, often, a diminutive aortic arch is difficult to trace out along its entire distance and multiple views and
clues need to be used. (Movie clip 71.78).

Case HLHS 2. Fig. 71.79: Pulmonary venous Doppler showing

consistent but low velocity a-wave reversal. This feature will need
to be serially followed in this fetus as pregnancy advances, since
the natural history of such restrictive atrial septums is to further
decrease with time. Presence of atrial level restriction to leftto-right flow adds significant morbidity as well as mortality and
may be an indication for fetal intervention or emergent postnatal
intervention.

Case PA. Fig. 71.80: Asymmetric four chamber showing tricuspid and right heart hypoplasia. The tricuspid annulus is small and
appears to be dysplastic. The right ventricle is smaller and non
apex-forming. (Movie clip 71.80).

Case PA. Fig. 71.81: Longitudinal view showing aortic outflow and
ventricular septal defect (VSD; *). The left atrium (LA) and left ventricle (LV) are noted here, with presence of aortomitral continuity.
The aortic outflow (Ao) is astride a ventricular septal defect (*).
(Movie clip 71.81).

or even temporize it enough to allow for survival, and

diminish postnatal mortality and morbidity. Thus, balloon
dilatation of the aortic valve in critical aortic stenosis
and HLHS, pulmonary valve balloon dilatation in critical
pulmonary stenosis or pulmonary atresia with an intact
interventricular septum, balloon dilatation of the interatrial
septum in cases with HLHS with restrictive interatrial

septum or in d-transposition of the great arteries, and

intact interventricular septum all bear potential interest.
The greatest constraint facing this field is the substantial
ethical issues as well as logistics around it. Encouraging
results have been noted in the hands of experienced
centers performing interventions at the extreme end of the
spectrum of congenital heart disease.119123

1554

Section 6: Congenital Heart Disease

Case PA. Fig. 71.82: Retrograde ductal arch (DuAr) in fetus with
pulmonary atresia. (Moive clip 71.82).

Case PR. Fig. 71.83: Four chamber with biventricular apical

hypertrophy (*), hyperechogenic ventricular walls (~), and right
ventricular chamber dilatation. Accompanying biventricular
systolic dysfunction is better seen in the accompanying movie
(Movie clip 71.83).

Case PR. Fig. 71.84: Dysplastic pulmonary valve. The pulmonary

annulus itself is of a normal size (--), seen here distal to a normal
appearing right ventricular outflow tract (RVOT). However, the pulmonary valve leaflets are not discerniblerolled up and thickened
leaflet tissue is seen in this location. The main pulmonary artery
(MPA) is dilated. (Moive clip 71.84).

Case PR. Fig. 71.85: Color Doppler right ventricular outflow showing free pulmonary incompetence and ductal reversal. A wide
regurgitant jet is seen to enter the right ventricle (RV) from across
the plane of the previously noted pulmonary annulus. In fact, this
reversal is seen to extend into and include the main pulmonary artery (MPA) and the ductal arch (DuAr). (Movie clip 71.85).

Chapter 71: Fetal Cardiac Imaging

1555

Case PR. Fig. 71.86: 3VV dilated pulmonary valve and branch
pulmonary artery (PA). (Movie clip 71.86).

Case PR. Fig. 71.87: Color Doppler both arches flow reversal in
ductal arch.

Case PR. Fig. 71.88: Pulse Doppler in ductal arch showing diastolic reversal or tofro flow.

Case PR. Fig. 71.89: Pulse Doppler across ductus venosus showing return to baseline.

Case PR. Fig. 71.90: Pulse Doppler of umbilical artery showing

loss of antegrade diastolic flow.

Case PR. Fig. 71.91: Pulse Doppler of umbilical vein with no obvious or significant venous notching.

1556

Section 6: Congenital Heart Disease

Case PR follow-up. Fig. 71.92: Two-dimensional (2D) dilated right

ventricular outflow tract (RVOT), main pulmonary artery (MPA),
and minimal pulmonary valve tissue. There has been marked enlargement of the pulmonary annulus (----) even accounting for the
advancing gestation. (Movie clip 71.92).

CASE STUDIES
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.

Case
Case
Case
Case
Case
Case
Case
Case
Case
Case

VSD 1 (ventricular septal defect)

VSD 2 (ventricular septal defect)
AVCD (atrioventricular canal defect)
Dextrocardia
TA (tricuspid atresia)
HLHS 1 (hypoplastic left heart syndrome)
HLHS 2 (hypoplastic left heart syndrome)
PA (pulmonay atresia)
IEF
PR (pulmonary regurgitation).

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Section 6: Congenital Heart Disease

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58. Sharland GK, Allan LD. Normal fetal cardiac measurements
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59. Tan J, Silverman NH, Hoffman JI, et al. Cardiac dimensions
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64. Hornberger LK, Sanders SP, Rein AJ, et al. Left heart obstructive lesions and left ventricular growth in the midtrimester
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67. Mkikallio K, McElhinney DB, Levine JC, et al. Fetal
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69. Yoo SJ, Lee YH, Kim ES, et al. Three-vessel view of the
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70. Maulik D, Saini VD, Nanda NC, et al. Doppler evaluation
of fetal hemodynamics. Ultrasound Med Biol. 1982;8(6):
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71. Tulzer G, Gudmundsson S, Sharkey AM, et al. Doppler
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72. Schmidt KG, Birk E, Silverman NH, et al. Echocardiographic
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73. Simpson JM, Cook A. Repeatability of echocardiographic
measurements in the human fetus. Ultrasound Obstet
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74. Gonalves LF, Espinoza J, Romero R, et al. Four-dimensional fetal echocardiography with spatiotemporal image
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75. Bhat AH, Corbett V, Carpenter N, et al. Fetal ventricular mass
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76. Huhta JC. Fetal congestive heart failure. Semin Fetal Neonatal Med. 2005;10(6):54252.
77. Rizzo G, Arduini D, Romanini C. Doppler echocardiographic
assessment of fetal cardiac function. Ultrasound Obstet
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78. Carceller-Blanchard AM, Fouron JC. Determinants of the
Doppler flow velocity profile through the mitral valve of
the human fetus. Br Heart J. 1993;70(5):45760.
80. Tulzer G, Khowsathit P, Gudmundsson S, et al. Diastolic
function of the fetal heart during second and third trimester:
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study. Eur J Pediatr. 1994;153(3):1514.

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81. Mkikallio K, Vuolteenaho O, Jouppila P, et al. Ultrasonographic and biochemical markers of human fetal cardiac
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82. Hernandez-Andrade E, Figueroa-Diesel H, Kottman C,
et al. Gestational-age-adjusted reference values for the
modified myocardial performance index for evaluation
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2007;29(3):3215.
83. Friedman D, Buyon J, Kim M, et al. Fetal cardiac function
assessed by Doppler myocardial performance index
(Tei Index). Ultrasound Obstet Gynecol. 2003; 21(1):336.
84. Meriki N, Welsh AW. Technical considerations for measurement of the fetal left modified myocardial performance
index. Fetal Diagn Ther. 2012;31(1):7680.
85. Van Mieghem T, Gucciardo L, Lewi P, et al. Validation of
the fetal myocardial performance index in the second and
third trimesters of gestation. Ultrasound Obstet Gynecol.
2009;33(1):5863.
86. Donofrio MT, Bremer YA, Schieken RM, et al. Autoregulation of cerebral blood flow in fetuses with congenital
heart disease: the brain sparing effect. Pediatr Cardiol.
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87. Clark JM, Case CL. Fetal arrhythmias. In: Gillete PC,
Garson A, editors. Clinical Pediatric Arrhythmias. 2nd ed.
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88. Matias A, Montenegro N, Areias JC, et al. Haemodynamic
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89. Wheeler T, Murrills A. Patterns of fetal heart rate during
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pulmonary artery and vein. Heart. 2007;93(11):144853.
91. Fouron JC. Fetal arrhythmias: the Saint-Justine hospital
experience. Prenat Diagn. 2004;24(13):106880.
92. Kleinman CS, Nehgme RA. Cardiac arrhythmias in the
human fetus. Pediatr Cardiol. 2004;25(3):23451.
93. Southall DP, Richards J, Hardwick RA, et al. Prospective
study of fetal heart rate and rhythm patterns. Arch Dis
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94. Krapp M, Kohl T, Simpson JM, et al. Review of diagnosis,
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95. Krapp M, Baschat AA, Gembruch U, Geipel A, Germer U.
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96. Oudijk MA, Ruskamp JM, Ververs FF, et al. Treatment of fetal
tachycardia with sotalol: transplacental pharmacokinetics
and pharmacodynamics. J Am Coll Cardiol. 2003;42(4):
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97. Merriman JB, Gonzalez JM, Rychik J, et al. Can digoxin
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98. Strasburger JF, Cuneo BF, Michon MM, et al. Amiodarone

therapy for drug-refractory fetal tachycardia. Circulation.
2004;109(3):3759.
99. Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated
congenital heart block: demographics, mortality, morbidity
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100. Jaeggi ET, Hornberger LK, Smallhorn JF, et al. Prenatal
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101. Friedman DM, Kim MY, Copel JA, et al. PRIDE Investigators. Utility of cardiac monitoring in fetuses at risk for
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102. Jaeggi ET, Fouron JC, Silverman ED, et al. Transplacental
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104. Beinder E, Grancay T, Menndez T, et al. Fetal sinus bradycardia and the long QT syndrome. Am J Obstet Gynecol.
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105. Abramowicz JS, Kossoff G, Marsl K, et al. Literature review
by the ISUOG Bioeffects and Safety Committee. Ultrasound
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106. Miller MW, Brayman AA, Abramowicz JS. Obstetric
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107. Kurjak A. Are color and pulsed Doppler sonography safe
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Obstet Gynecol. 2000;16:5946.
109. Yagel S, Cohen SM, Achiron R. Examination of the fetal heart
by five short-axis views: a proposed screening method
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110. Chaoui R, Heling KS. New developments in fetal heart
scanning: three- and four-dimensional fetal echocardiography. Semin Fetal Neonatal Med. 2005;10(6):56777.
111. Maulik D, Nanda NC, Hsiung MC, Youngblood JP. Doppler
color flow mapping of the fetal heart. Angiology. 1986;
37(9):62832.
112. Chaoui R, Hoffmann J, Heling KS. Three-dimensional (3D)
and 4D color Doppler fetal echocardiography using spatiotemporal image correlation (STIC). Ultrasound Obstet
Gynecol. 2004;23(6):53545.
113. Maulik D, Nanda NC, Singh V, et al. Live three-dimensional
echocardiography of the human fetus. Echocardiography.
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114. Volpe P, Campobasso G, Stanziano A, et al. Novel application

of 4D sonography with B-flow imaging and spatio-temporal
image correlation (STIC) in the assessment of the anatomy
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2006;28(1):406.
115. Bord A, Vlasky DV, Rosenak D, et al. B-flow modality
combined with STIC in the evaluation of malalignment of
the great vessels. Ultrasound Obstet Gynecol. 2006; 28:447.
116. Bord A, Rosenak D, Vlasky DV, et al. B-flow modality
combined with STIC in the evaluation of fetal venous
anomalies. Ultrasound Obstet Gynecol. 2006;28:5567.
117. Yagel S, Cohen SM, Shapiro I, et al. 3D and 4D ultrasound
in fetal cardiac scanning: a new look at the fetal heart.
Ultrasound Obstet Gynecol. 2007;29(1):8195.
118. Carvalho JS, Moscoso G, Tekay A, et al. Clinical impact of
first and early second trimester fetal echocardiography on
high risk pregnancies. Heart. 2004;90(8):9216.

119. Marshall AC, van der Velde ME, Tworetzky W, et al.

Creation of an atrial septal defect in utero for fetuses with
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restrictive atrial septum. Circulation. 2004;110(3):2538.
120. Tworetzky W, Wilkins-Haug L, Jennings RW, et al. Balloon
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selection, technique, and results of successful intervention.
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121. Tulzer G, Arzt W, Franklin RC, et al. Fetal pulmonary
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123. Gardiner HM. In-utero intervention for severe congenital
heart disease. Best Pract Res Clin Obstet Gynaecol.
2008;22(1):4961.

1561

CHAPTER 72
M-Mode and Two-Dimensional
Echocardiography in Congenital
Heart Disease
Neeraj Awasthy, Savitri Shrivastava

Snapshot

Paent Preparaon

Imaging

Dextrocardia

Principles of Sequenal Chamber Analysis

General Features: Shunt Lesions

Atrial Septal Defects

Ventricular Septal Defect

Patent Ductus Arteriosus

Aortopulmonary Window

Gerbode Defect

Atrioventricular Septal Defects

Congenital Anomalies of Mitral Valve

Congenital Abnormalies of Tricuspid Valve

Valvular Aorc Stenosis

Subvalvular Aorc Stenosis

Supravalvular Aorc Stenosis

Aorc Regurgitaon

Sinus of Valsalva Aneurysm

Aortocameral Communicaons

Aorc Override

Double Outlet Right Ventricle

Truncus Arteriosus

Transposion of Great Vessels (TGA)

Atrioventricular and Ventricoarterial Discordance

Normal Flow Paern of Pulmonary Veins

Anomalies of Pulmonary Veins

Total Anomalous Pulmonary Venous Connecon

Anomalies of Systemic Veins

Coronary Artery Anomalies

Coronary Arteriovenous Fistula

Coronary Aneurysms

Abnormal Formaon of Arch

Coarctaon of Aorta (CoA)

Interrupon of Aorc Arch

Aorc Aneurysm

Univentricular Atrioventricular Connecons

Tricuspid Atresia

Mitral Atresia and Hypoplasc Le Heart Syndrome

Heterotaxy Syndrome

1562

Section 6: Congenital Heart Disease

PART 1: BASICS OF IMAGING AND SEQUENTIAL SEGMENTAL ANALYSIS

Echocardiography is the most useful modality of imaging
in the diagnosis of congenital heart disease (CHD). The
following special issues should be remembered while
performing pediatric echocardiography:

PATIENT PREPARATION14
Prior to taking the patient for an echocardiographic
study, all the available clinical data should be carefully
perused and the oxygen saturation checked. This helps in
total evaluation of lesions. Pediatric echocardiographic
examination should be performed when the child is quiet
and cooperative or else one may miss certain important
observations. In a crying baby alter intrathoracic pressures
resulting in fallacious gradients and other vital findings.
Most of the neonates and infants can be quietened
by a feed, handling by an experienced nurse, using a
pacifier, and keeping them adequately warm. Wrapping
a small child is in itself not only a proper positioning
method for small children and infants but a cuddled
up wrapped infant can be easily quietened for a proper
echocardiographic examination (Fig. 72.1). Certain
children can also remain quiet by familiarizing them with
the surroundings and a friendly environment with some

toys and music. A cheerful environment, interaction

with the patient, allowing the parents to be around,
and familiarizing the patient with equipment are a few
factors that help in achieving the childs cooperation.
Sedation may be appropriate if the above methods fail.
Sedation should be given in the presence of a parent or a
relative with whom the child is familiar. This helps in the
acceptance of the drug and gives a better sedative effect.
Also, a familiar parent is able to detect changes in the
sensorium of the patient. Drugs commonly used are oral
chloral hydrate and nasal midazolam. Chloral hydrate is
given in a dose of 50 to 100 mg/kg weight (the total dose
should not exceed 1.5 g).1 Children may become restless
and agitated with chloral hydrate. This usually occurs
when the medicine is given when they have just woken
from sleep or are hungry. Midazolam nasal drops can also
be used in infants and young children (up to 3 years), with
a dose of 0.2 mg/kg bodyweight in the nostrils.2,5,6 If the
patient does not go to sleep or becomes uncooperative,
the same dose can be repeated. Many patients do not fall
asleep with midazolam but become passive, cooperative,
and drowsy; thus, the study can be performed. The other
important aspect is patience. It is rewarding to patiently
wait till the child quietens down and then proceed with

Fig. 72.1: The steps for appropriately wrapping up an infant for proper cardiac evaluation. Cuddled up neonate is well suited and cooperative for any echocardiographic examination. Step1: Laying the sheet with single fold on a flat surface (bed); Step 2: Demonstrates
the end, which is picked up and wrapped on the babys opposite feet; Step 3: Demonstrates step 2 on the baby; Step 4: Wrapping the
left over upper end of the sheet on the upper limb; Step 5: Repeating the step of wrapping the upper arm for the other arm; Step 6: Child
with restrained both upper limbs; Step 7 and Step 8: The lower part of the sheet is wrapped around the lower limbs to cuddle up the child.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

the study. In sedated babies, oxygen saturation should be

monitored during the study. There is a wide variation in
the age of the patients with CHD, as such transducers of
different sizes are needed. In cardiac imaging the sound
waves travel through the chest wall, pericardium, and
cardiac structures. A portion of the energy is absorbed
and the rest is transmitted. The absorption increases with
frequency; therefore, a high frequency ultrasound beam,
that is, 10 MHz will not penetrate as far as a low frequency
transducer like a 2.0 MHz frequency will. This is one of the
reasons why high frequency transducers are needed for
the new born and infants while low frequency transducers
are used for grown up children and adults.3
For small and premature infants, a 12 MHz transducer
is used. It has excellent near-field axial and lateral
resolution. However, its ability to penetrate the far field is
limited. For optimal resolution of the image, the highest
frequency transducer should be tried first. If penetration is
not adequate, progressively lower frequency transducers
can be used till an optimal image is obtained. Multiple
transducers may be used in the same patient for optimal
imaging. For subcostal imaging generally a transducer with
a lower frequency than used for transthoracic imaging gives
better results. The probe transducers which are commonly
used for pediatric echocardiographic are generally as per
the age group of the patient. While a 12 MHz probe
transducer is used for small children, a transducer with a
frequency range from 5 MHz to 8 MHz is used for grownup children and adolescents. In adults, a lower frequency
probe transducer (45 MHz) is generally used.

pulsed and continuous wave Doppler, velocities across all

valves are recorded. If any unusual turbulence on CFI is
noted, it should be carefully interrogated using pulsed and
continuous wave Doppler.
Conventional views for echocardiography are detailed
below.1,2,4-8
Because of the limitations imposed by the air-filled
lungs and bony thoracic structures, the echocardiographic
planes for the heart are obtained from only four areas of
the body (Table 72.1):
Subcostal.
The cardiac apexapical four-chamber (4C) views.
Parasternal regionadjacent to sternumsecond,
third, and fourth intercostal spaces.
Suprasternal notch.

Subcostal Window7
Evaluation of congenital heart lesions generally starts with
the subcostal view. This provides information regarding
the situs, which is a crucial step in the diagnosis of CHD.
Imaging the inferior vena cava (IVC) and abdominal aorta
in cross section gives this information (Fig. 72.2). In small
infants if the transducer is pressed too deep, impairment
of respiration may occur. Intensive pressure may also
compress on the IVC impairing the venous return.
At times, it may lead to nonvisualization of an otherwise
patent IVC. The pulsations, color flow mapping, and pulse
Doppler interrogation identifies the IVC and the aorta.
Table 72.1: Conventional Views of Echocardiography

IMAGING
The anatomical reference for the echocardiographic
planes is the major axis of the heart and not the major
axis of the body. Displaying images in an anatomically
correct fashion allows better understanding of anatomy,
particularly in cases of complex CHD. For an anatomically
correct display, the apex of the imaging sector is placed at
the bottom of the screen in the subcostal and the apical
views, and for the rest of the views it is placed at the top of
the screen. The aim of the study is to image all structures
of the heart and great vessels in all possible planes so that
one can get a three-dimensional image from the twodimensional (2D) study. The study should be performed
in a systematic sequence in all patients so as to minimize
errors in diagnosis. In each plane, the transducer should
be swept across the heart and great vessels in a specific
direction with and without color flow imaging (CFI). Using

1563

Subcostal
Sagittal (B1 caval)
Coronal
Paracoronal view
Apical
4C view
5C view with aorta
5C view with pulmonary artery
Parasternal
Parasternal long-axis view
Psax ( parasternal short-axis view)
High parasternal short-axis view (ductal view)
Suprasternal windows
Long-axis view
Short-axis view

1564

Section 6: Congenital Heart Disease

The position of the heart in the thorax is best appreciated

in the subcostal views, which is helpful in planning the
whole study. This view also shows the movements of both
domes of the diaphragm, any pleural collections, and the
position of the liver.

Fig. 72.2: Two-dimensional echocardiography. Subcostal shortaxis at the level of abdominal vessels. Inferior vena cava and aorta
are shown in cross section with aorta (red) to left of spine and
inferior vena cava (blue) to right of spine (*).

D1
Figs 72.3A to E

Following this, one proceeds to image other cardiac

structures from the subcostal view (Figs 72.3A to F).
The interatrial septum is viewed in the sagittal plane
(Bicaval view) with the transducer placed below the
xiphisternum. This approach helps to view the flap of
foramen ovale, superior vena cava (SVC), IVC, and both
right upper and lower pulmonary veins. Their connection
can be identified; color flow mapping and pulse Doppler
interrogation of these veins are done to rule out any
obstruction. In venous obstruction, the first to get altered
is the loss of phasic character of venous flow; later, the total
velocity also increases. The atrial septum should also be
imaged in coronal plane so as to profile the atrial septum
completely. Then sweep in the sagittal plane at the level of
the ventricular septum, atrioventricular (AV) valves, and
great vessels. The sweeps will profile the right ventricle
(RV), RV out flow and pulmonary arteries (with a straight
anterior tilt), interventricular septum, left ventricle (LV)
and left ventricular out flow tract from right to left, AV
valves and the great vessels, then the operator should
proceed with the coronal section of the ventricle and the
out flow tract by tilting the plane of transducer posterior
to anterior. By giving a counterclockwise rotation to the
transducer, one can obtain a paracoronal view showing RV

D2

E1

E2

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

F1

F2

F3

F4

1565

Figs 72.3A to F: Two-dimensional echocardiography subcostal window. (A) Subcostal coronal (four-chamber equivalent) view showing
interatrial septum, connection of right upper pulmonary vein (RUPV) to left atrium. Descending aorta ( ) in short axis is imaged behind
the left atrium; (B) Subcostal coronal view with posterior-to-anterior sweep showing left ventricular outflow tract and aorta with color flow
mapping (blue); (C) Far anterior tilting shows right ventricular outflow tract; (D1 and D2) Subcostal paracoronal view with right anterior
tilt showing right ventricle (RV) and right ventricular outflow tract. (E1 and E2) Subcostal paracoronal view with color compare showing
right ventricular outflow (*) tract, main pulmonary artery, and right pulmonary artery; (F1 to F4) Subcostal sagittal view of the muscular
septum with right-to-left sweep. This view demonstrates the short axis of the left ventricle (LV) at the level of the mitral valve with sweep
up to the apex of the heart. The color comparison at the scale of 3536 is especially important to look for a small muscular ventricular
septal defect (VSD), especially in the setting of the increased pulmonary artery pressures (not shown in the illustration).

outflow and right pulmonary artery (PA). This view is also

very useful to profile perimembranous and subpulmonary
areas of interventricular septum. In coronal section,
movement of both domes of the diaphragm can be
visualized during respiration.

Subsequently, one should proceed with the

parasternal apical, long-axis, short-axis, and suprasternal views. These will be detailed later. The sequence
may need to be altered in a few children till they are well
sedated.

1566

Section 6: Congenital Heart Disease

Sagittal view is similar to the left anterior oblique

view and paracoronal view is similar to the right anterior
oblique view in the angiographic studies.
In the sagittal view, one should sweep the transducer
from right to left and reverse, and in the coronal view,
the sweep should be from the posterior to anterior and
reverse, in order to obtain various sections of the heart.

Apical View (4C View; Figs 72.4A to D)

Apical view is commonly called the four-chamber view
or 4C view. The apical impulse corresponds to the site of
optimal placement of the probe for 4C view. After obtaining
an adequate window from the apex, the transducer should
be swept from the posterior to anterior plane and reverse.

Figs 72.4A to D: Two-dimensional transthoracic echocardiography. (A) Apical four-chamber view in posterior plane showing coronary
sinus (CS) coursing from left to right in atrioventricular groove; (B) Apical four-chamber view showing all four cardiac chambers, the
interatrial septum, and interventricular septum. Offsetting of atrioventricular valves (attachment of tricuspid valve more toward apex than
mitral valvearrow) is seen; (C) Apical four-chamber view in anterior plane (apical five-chamber view) showing left ventricular outflow
tract and aorta; (D) Same view in far anterior tilt visualizes right ventricular outflow tract and main pulmonary artery. (Ao: Aorta; CS:
Coronary sinus; LA: Left atrium; LV: Left ventricle; MPA: Main pulmonary artery; RA: Right atrium; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1567

In the most posterior plane, the coronary sinus can be

imaged coursing at the base of the left atrium (LA) and
opening into right atrium (RA; Fig. 72.4A). The classical 4C
view shows all four chambers, AV valves, and the offsetting
of AV valves (Fig. 72.4B). With the anterior tilt, the aorta can
be seen and on a further anterior tilt the PA can be profiled
if the great arteries are normally related (Figs 72.4C and D).
The apical five-chamber and two-chamber views are
commonly used in adult practice to image various
segments of the LV. As the transducer is swept anteriorly
from the apical view, image quality deteriorates because
of rib shadowing.

Parasternal Long-Axis View (Figs 72.5A to D)

This view is obtained with the marker of the probe

directed to the right shoulder in the long axis of the
heart. The parasternal long-axis (PLAX) view visualizes
the RV, the interventricular septum, and LV; by
sweeping from left to right, one visualizes the aorta
and LA and the LV (Fig. 72.5A). The sweep toward the
left and superiorly allows visualization of the RV out
flow tract (Fig. 72.5B). The rightward and inferior tilt
profiles the tricuspid valve and the adjacent RV inflow
(Figs 72.5C and D).

Figs 72.5A to D: Two-dimensional transthoracic echocardiography. (A) Parasternal long-axis view of left ventricle showing the left
ventricular inflow and outflow tract with anteriorly placed RV cavity; (B) Parasternal long-axis view with anterior tilt showing right
ventricular outflow tract. (C and D) Parasternal long axis with posterior tilt and color compare showing right ventricular inflow. (Ao: Aorta;
LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right atrium; RVOT: Right ventricular outflow tract).

1568

Section 6: Congenital Heart Disease

Parasternal short-axis (PSAX) view is obtained with

the transducer pointing to the left and probe marker

perpendicular to the PLAX view. Serial cross sections of

the heart are obtained, demonstrating various structures
by sweeping superiorly and inferiorly. The superior sweeps
show the great vessels and the inferior sweeps show serial
cross sections of the ventricles from base to apex.

Parasternal Short-Axis View

(Figs 72.6A to D)

Figs 72.6A to D: Two-dimensional transthoracic echocardiography. (A) Parasternal short-axis view at the base of the heart showing
aorta (Ao), right ventricular outflow tract, pulmonary valve, main pulmonary artery, bifurcation of main pulmonary artery, and right and
left pulmonary arteries; (B) Parasternal short-axis view of left ventricle at the level of the mitral valve showing the anterior mitral leaflet
(arrow) and the posterior mitral leaflet (arrow); (C) Parasternal short-axis view of left ventricle at the level of papillary muscle showing
the location of papillary muscles, anterolateral at 4 oclock and posteriomedial at 8 oclock, trabecular part of interventricular septum,
and right ventricular cavity; (D) Parasternal short-axis view toward apical part of ventricular septum. (LPA: Left pulmonary artery; LV: Left
ventricle; MPA: Main pulmonary artery; RPA: Right pulmonary artery; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Parasternal Short-Axis View for

Coronary Arteries (Fig. 72.7)1,2,8
The coronary arteries are better visualized with higher
frequency transducers in PSAX views at the level of aortic
cusps. Using zoom and low color flow gain, one should
check the color flow in both coronary arteries. The right
coronary artery (RCA) is at a more superior plane, hence
the transducer needs to be rotated counterclockwise, and
for the left coronary artery (LCA) a clockwise rotation of
the transducer is required. The origin of both the coronary

1569

arteries can usually be visualized. The coronary arteries

can also be visualized from a subcostal window in the
coronal view of left ventricular out flow.

High Parasternal or
Ductal View (Fig. 72.8)
The classic short-axis view visualizing the aorta and PA is
initially obtained from just below the clavicle, a level higher
than the usual parasternal views. From this location, the
transducer is rotated anticlockwise. During this process,
the right PA gradually goes away from view. The main PA
is then seen continuing as the left PA. The descending
thoracic aorta then is in view. The duct is located at the
junction of left PA with the descending aorta. This is called
ductal view.

Suprasternal Views (Figs 72.9 and 72.10)

This view is profiled by placing the probe in the suprasternal
notch. It is helpful to place a small pillow below the neck
to extend the neck.

Long-Axis View (Fig. 72.9)

Fig. 72.7: Two-dimensional echocardiography. Parasternal shortaxis view showing origin of both the right and left coronary arteries.
(LCA: Left coronary artery; RCA: Right coronary artery; Ao: Aorta).

Fig. 72.8: Two-dimensional echocardiography. High parasternal

short-axis view with anticlockwise tilt to profile ductal area with
color flow imaging shows Desc. aorta, main pulmonary artery, and
left pulmonary artery in short axis. (AO: Aorta; LPA: Left pulmonary
artery; MPA: Main pulmonary artery; Desc. Ao: Desending aorta).

The direction of the marker of the probe is toward the

left shoulder. This oblique view profiles the arch and
descending aorta lengthwise. In case of right aortic arch,
the transducer needs to be rotated anticlockwise to profile
the arch and descending aorta.

Fig. 72.9: Two-dimensional echocardiography. Suprasternal

long-axis view with color flow mapping showing arch of aorta and
descending aorta with arch branches. (A: Transverse arch; Desc.
Aorta: Descending aorta).

1570

Section 6: Congenital Heart Disease

Fig. 72.10: Two-dimensional echocardiography with color flow compare. Suprasternal short-axis view showing aorta in short axis, right
pulmonary artery in long axis and innominate vein joining superior vena cava, all four pulmonary veins joining left atrium. (LA: Left
atrium; RPA: Right pulmonary artery; SVC: Superior vena cava; AO: aorta; Inn V: Innominate vein).

Short-Axis View (Fig. 72.10)

The direction of probe is parallel to the body plane. This
short-axis view profiles the aorta in its short axis and
opens up the right PA below the aorta in its entire length
till the first branch. Below the right PA, one can see the LA
receiving all four pulmonary veins, also called the crab
view. By tilting the transducer to left and anteriorly, left PA
can be profiled. By tilting the transducer superiorly and to
the right, one can profile the innominate vein and the SVC.
In the suprasternal notch view try to look for the branching
of the arch vessels. If the first branch courses to right and
divides it, this indicates a left aortic arch, and if the first
branch divides and courses to the left side, it is diagnostic
of the right aortic arch.

DEXTROCARDIA
For patients with dextrocardia, attempts should be made
to perform the same views keeping the transducer on
the right side of the chest (Fig. 72.11). The marker on the
transducer should point in the same overall direction as
for patients without dextrocardia. This enables consistency
in image orientation and display.

Fig. 72.11: Two-dimensional echocardiography. Subcostal view

showing situs solitus with dextrocardia and L-looped ventricles.
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle).

Color Flow Doppler

Color is superimposed on 2D images to demonstrate
the flow information. While using color flow mapping
the 2D image should be idealized and 2D gain reduced.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1571

Table 72.2: Pitfalls in Imaging of Congenital Heart Disease

Chest wall deformities: These are common in patients with congenital heart disease. Thus, pectus excavatum or carinatum produces
poor parasternal images. Also, scoliosis causes the heart to shift to the ipsilateral side and produces difficulty in imaging
Diaphragmatic hernia: In this condition, the bowel loops can come in front of the chest wall. Since air is a poor conductor of
ultrasound, it can cause unexplained difficulties in imaging. The plain X-ray of the chest easily diagnoses the condition
The postoperative state: Chest wall edema, bandages, and chest wall drains in the immediate postoperative period and
deformity of the sternum in the late postoperative state frequently cause problems in imaging. This is especially true for midline
structures. Following the arterial switch and the LeCompte maneuver, the pulmonary artery frequently lies behind the sternum.
Similarly, midline structures like conduits may be poorly visualized.
Grown up congenital heart disease: It is frequently difficult to get good images in a grown up patient with congenital heart disease.
This is especially true for structures like the pulmonary artery, pulmonary and systemic veins, descending thoracic aorta, ductal
area, etc. even with use of adult transducer. Associated with the poor window is the inability to get good color flow images or
continuous wave Doppler envelopes

Figs 72.12A and B: Nonoptimal scales can result in overestimation of tricuspid valve regurgitation. (A) Apical four-chamber view
showing tricuspid valve regurgitation with splaying of the colors (at color scale of 32); (B) Apical four-chamber view in the same patient
showing tricuspid valve regurgitation with no splaying of the colors at color scale of 72.

Red color indicates flow toward the transducer and blue

indicates away from the transducer. Color Doppler allows
rapid display of a great deal of information. One can easily
visualize small disturbed jets and study them in depth.
CFI is an extremely useful modality that can considerably
improve the speed and accuracy of an echocardiographic
examination. If used incorrectly, however, it can result
in an erroneous interpretation of information. The
echocardiographer needs to be aware of the following
pitfalls of CFI to make optimal use of color Doppler
(Table 72.2).
Failure to optimize 2D images before using color
Doppler results in poor quality color flow images:
The echocardiographer should endeavour to obtain
maximum possible information from 2D images before
using color. This allows CFI to be specifically targeted
to areas of interest.

Size of color sector: If the color sector is very large,

the frame rate is reduced considerably. This results
in poorly defined color flow images with potential for
both overestimation and underestimation of lesion
severity. The size of color sector should be kept as
small as possible. The use of the zoom switch allows
for improved visualization with the smallest possible
size of the color sector.
Failure to optimize gain and scale settings (Figs 72.12A
and B): Excessive gains can result in overestimation of
regurgitant lesions such as mitral, tricuspid and aortic
regurgitation. The color Doppler gain should be adjusted in a manner that no color is seen in areas where
blood doesnot flow.
Failure to optimize color scales (Figs 72.13A and B):
Low velocity flows (venous flows, flows in the atria,
etc.) are best demonstrated by low color scale settings.

1572

Section 6: Congenital Heart Disease

Figs 72.13A and B: Optimal color gain settings are essential to prevent graining in the field of imaging. (A) Four-chamber (inverted
view) showing graining in the field of interest with gain settings of 90%; (B) The same patient with trace regurgitation and minimal graining after optimizing color gains.

The same settings will overestimate the severity of high

velocity regurgitant jets such as aortic regurgitation
and mitral regurgitation. Wherever high velocity flows
are anticipated, the color scale settings should be
appropriately increased.
Multiple color jets: Multiple color flow jets require
careful interpretation. Examples include multiple
ventricular septal defects (VSDs) or a combination
of VSD with infundibular pulmonary stenosis. In
this situation, some lesions may be missed. Careful
attention to 2D images can often resolve the individual
lesions.

Interpreting Color Flow

Information in Isolation
A number of criteria are available for quantification of
regurgitation jets by CFI. However, using them in isolation
may result in incorrect interpretation of lesion severity.
The information obtained on CFI should be combined
with the 2D information to improve accuracy. Examples
include incorporation of left atrial size, left ventricular
size and function while estimating severity of mitral
regurgitation, and left ventricular size and function for
estimation of severity of aortic regurgitation.

Pulsed Doppler (Figs 72.14A and B)

A single ultrasound crystal alternatively transmits and
receives the ultrasound signal. The vessels or valve
being interrogated is imaged by 2D echocardiography.

The Doppler cursor should be placed in the direction of

the flow being interrogated as seen on color flow mapping.
There are limits to the maximum frequency shifts that
can be unambiguously displaced at any given point; the
maximum detectable frequency shift in one direction is
one half of the sampling rate. The maximum detectable
frequency is called the Nyquist limit. The pulsed Doppler
mode selectively provides the flow velocities at the area
being interrogated.

Continuous Wave Doppler

The continuous wave Doppler transducer has two crystals,
one continuously transmits an ultrasound signal whereas
the other continuously receives the back-scattered ultrasound; therefore, Doppler signals from all blood flow traversed by the ultrasound beam are received and displayed.
The main advantage of plural wave of continuous wave
Doppler is that there is no limit to the maximum limits that
can be measured. Therefore, very high velocity jets can be
correctly interrogated.

Cautions in Using Pulsed and Continuous

Wave Doppler Evaluation
Poor Echo Windows
If the images are suboptimal, Doppler signals are
suboptimal as well. The nonimaging continuous wave
Doppler probe may be used in selected circumstances,
keeping in mind the potential pitfalls of using the
nonimaging probe.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1573

Figs 72.14A and B: (A) Doppler signal with inappropriate gain setting resulting in falsely increased gradients. Note excessive splaying
with a gradient of 85 mm Hg; (B) The same patient with optimized gains with a gradient of 51 mm Hg (+).

Inappropriate Transducer Frequency

High quality Doppler signal with clear display of high
velocity signals requires lower transducer frequencies
than those required for optimal images. Serious
underestimation of stenotic lesions may result from use
of high frequency transducers for obtaining Doppler
gradients. This is particularly true for Doppler interrogation
of deeper lesions such as coarctation. Whenever possible,
gradients obtained during imaging with high frequency
transducers should be confirmed by using lower frequency
transducers.

Inappropriate Doppler Gain and Filter

Settings (Figs 72.12 and 72.13)
High velocity signals require high filter settings. For low
velocity signals such as venous signals, low filter settings
are appropriate. The Doppler gain should be adjusted to
minimize signal-to-noise ratio. Increased gain settings
result in noisy signals with poorly defined spectral margins.
This can result in overestimation of the gradients.

Inappropriate Doppler Scales

(Figs 72.14A and B)
Aliasing of the signal results when the Doppler scales
are inappropriately low. Once aliasing occurs, the signal
cannot be accurately quantified. Increasing the scales of
the Doppler signals can minimize aliasing. For pulsed
Doppler, this would require increasing in the pulse

repetition frequency (PRF). High velocity signals alias

despite maximum PRF. For measuring their velocity,
continuous wave Doppler will have to be used.

Inappropriate Signal Alignment

(Figs 72.15A and B)
It is the most common cause of underestimation of
Doppler gradients. The correct use of CFI and interrogation
in multiple views considerably minimizes the likelihood
of poor alignment. For example, in a patient with valvular
aortic stenosis the gradient should be obtained in the
apical, the right parasternal, and the suprasternal views.
The maximum value obtained represents the true Doppler
gradient. However, the gain settings should be optimized to
get a clear envelope. For a patient with a perimembranous
VSD, Doppler signals may have to be recorded in the
subcostal, the apical, and the parasternal views to obtain
the best aligned signal.

Pitfalls Relating to the Use of the Nonimaging

Continuous Wave Doppler Probe
There is a likelihood of mistaking one signal for another
if the nonimaging probe alone is used for gradient
estimation. For example, while interrogating from the
apex, the systolic gradient of mitral regurgitation may be
mistaken for aortic stenosis. Again, interrogation from
multiple views can help reduce the chances of errors.

1574

Section 6: Congenital Heart Disease

Figs 72.15A and B: (A) High frequency signal not getting aligned in view of the pulsed wave Doppler exceeding the Nyquist limit;
(B) The same patient by appropriately increasing the scale of the measurements, and changing from pulsed wave to continuous wave
Doppler.

Failure to Appreciate the Limitations of the

Bernoulli Equation
Simply stated, the Bernoulli equation is as follows:
Pressure difference between two points = convective
acceleration + flow acceleration + viscous friction. Of these
three determinants of pressure difference, convective
acceleration is considered the most significant. Convective
acceleration is defined by the formula 1/2p (V22 V12);
p represents the fluid density of blood; V1 and V2 are
the velocity of blood at the two points across which the
pressure difference is being measured. After converting
blood density by using the conversion factor to mm Hg
and velocity to m/s, the coefficient calculates out as 3.98.
This is rounded to 4. The assumptions that are made while
applying the Bernoulli equation to clinical situations
are as follows: (a) Flow acceleration and viscous friction
are considered negligible; (b) The velocity proximal to
the obstruction is negligible as compared to the distal
velocity. After making these assumptions, the formula is
simplified to: pressure difference = 4 peak instantaneous
velocity. (c) The pressure gradients are not calculated in
tubular uniform structures. Although this formula has
been validated for a number of situations, the assumptions
made introduce important limitations to its applications
in specific situations. These include serial obstructions
where it cannot be assumed that proximal velocity (VI)
is negligible. For measuring pressure differences across
discrete obstruction within a tubular structure (such
as coarctation of the aorta), the VI should be measured
and VI should be subtracted from V2. Other examples
where V1 may be increased include combined stenotic

and regurgitant lesions, stenotic lesion combined with a

shunt lesion [atrial septal defect (ASD) with pulmonary
stenosis], and high output states such as anemia. In
patients with polycythemia, the viscous friction may be
significant and blood density (p) cannot be assumed to be
the same as for normal, and in such a situation significant
underestimation of Doppler gradients may result.

Pressure Gradients Across Trivial Lesions

When the amount of blood flow is very small, such as in
tiny muscular VSDs or in trivial tricuspid regurgitation,
the pressure gradient may be underestimated by Doppler
despite optimal gain setting. This is possibly because the
sample volume is too small.

Comparison of Echo Gradients with Those

Obtained During Cardiac Catheterization
(Fig. 72.16)
Peak-to-peak versus peak instantaneous pressure
gradients: While interpreting Doppler flow gradients, it
is important to remember that Doppler measures the
peak instantaneous pressure difference, which is higher
than the peak-to-peak pressure difference measured by
cardiac catheterization. The relationship between peak
instantaneous pressure difference and peak-to-peak
pressure difference is complex, possibly because it is
determined by a number of influences (such as heart rate,
severity of obstruction, dP/dt, etc.). Doppler gradients can
exceed gradients recorded by cardiac catheterization by as
much as 30 to 40 mm Hg.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Fig. 72.16: The graph demonstrates the comparison of peak

instantaneous gradient with peak-to-peak gradient in left ventricular
and aortic pressure tracing. The instantaneous pressure gradient
is higher than the peak-to-peak gradient.

Failure to Relate the Doppler

Gradients to Flows
Pressure gradients are influenced by flows. While interpreting gradients recorded by Doppler, the physiological
state of the patient needs to taken into consideration.
High output states, fever, anxiety, agitation, anemia can
all exaggerate gradients. For this reason, it is imperative
to record systemic blood pressure (BP) while trying
to predict PA pressures using gradients across VSDs.
Similarly, while interpreting the severity of stenotic lesions
by pressure gradients, the flow across the orifice needs to
be considered. Other pathophysiological states that can
reduce blood flow include hypovolemia, hypotension, and
ventricular dysfunction.

PRINCIPLES OF SEQUENTIAL
CHAMBER ANALYSIS913
The cardinal principle of sequential chamber analysis states
that the morphology of a chamber should be determined
on the basis of its most constant component. Thus, one
should not use a component to identify a structure if
that component itself is variable. The evaluation of any
congenital heart involves a systemic sequential analysis
that involves the following steps:
Identification of abdominal situs.
Identification of atria and atrial arrangement.

1575

Fig. 72.17: Two-dimensional echocardiography in a patient with

situs inversus. Subcostal short-axis view at the level of abdominal
vessels showing inferior vena cava and aorta in cross section with
aorta (red) to the right of spine, and inferior vena cava (blue) to the
left of spine (), reversed position of the liver (toward the left of
the spine) and gastric bubble (toward the right of the spine). (Ao:
Aorta; V: IVC; Li: Liver; GB: Gastric bubble).

Identification of ventricular morphology and AV

connection.
Identification of great vessels and ventricular arterial
connection.
Identification of associated abnormalities and
assessing their severity.
The initial step of evaluation starts with the determination of abdominal situs with IVC to the right of the
spine and aorta to the left in situs solitus (Fig. 72.3) and
vice versa in situs inversus (Fig. 72.17). The atrial situs
generally corresponds with the abdominal situs. If the
IVC and aorta are on same side of the spine then possibly
we are dealing with isomeric heart (Fig. 72.18). The IVC
may be interrupted, which will suggest left isomerism
(Fig. 72.18).

Identification of Atria and Their

Arrangement
The next step is to identify the atrial arrangement. Four
types of atrial arrangements can exist:
Normal atrial arrangement (Situs solitus)the RA is
right-sided and the LA is left-sided.
Mirror image atrial arrangement (Situs inversus)the
RA is left-sided and the LA is right-sided.
Isomerism of the left atrial appendage.
Isomerism of the right atrial appendage.

1576

Section 6: Congenital Heart Disease

Fig. 72.18: Two-dimensional echocardiography. Subcostal shortaxis view at the level of abdominal vessels. Inferior vena cava and
aorta are shown in cross-section with aorta (red) and inferior vena
cava (blue) to right of spine. Spine (*). (Ao: Aorta; V: IVC).

Figs 72.19A and B: Two-dimensional echocardiography in subcostal coronal view with superior tilt showing (A) broad-based right atrial
appendage (RAA); (B) narrow, finger-like left atrial appendage (LAA).

Identification of Atrium
The atrial appendage: It is the most constant feature for
atrial identification. The right atrial appendage is a broad
triangular structure with a broad junction with the rest
of the atrium (Figs 72.19A and B). It has coarse pectinate
muscle within the appendage, which extend all around
the vestibule of the appendage and atrial junction. The
morphological left atrial appendage is tubular with a
narrow junction with the rest of the atrium (Fig. 72.20).
The posterior wall is smooth, and the pectinate muscle is
confined only to the anterior quadrants of the vestibule.
Echocardiographically, the shape of atrial appendages is
best visualized by a combination of the subcostal coronal
view (right and left atrial appendages) and parasternal
short axis at great vessel level (left atrial appendage). The
pectinate muscles, however, cannot be well identified by

echocardiography. Unfortunately, altered hemodynamics

in complex CHD may not allow the appendage to have a
classical appearance.
The venous drainage: The opening of the suprahepatic
portion of the IVC to the RA is the most important marker in
identifying morphological RA and can be easily identified
on 2D echocardiography in the subcostal long-axis view.
The pulmonary venous connection is too variable to be
used as a marker for LA.
The atrial septum: The septum primum overlaps the
septum secundum from the left atrial side and if present,
is an important feature in identifying the LA, best
visualized in the subcostal sagittal view. Unfortunately,
in some complex forms of CHD the atrial septum may
be absent and thus this feature cannot be used as
a marker.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1577

left- or right-sided atrium or sometimes in the midline

(for those who believe in the isomerism concept, both
atria are of same morphology, hence called the right or left
isomerism).
The atrial appendage morphology would be the most
specific marker for atrial identification. Unfortunately, the
shape of the appendage (as discussed previously) rarely
helps in complex CHD and identification of the atrial
pectinate muscle by 2D echocardiography is not usually
possible.

Fig. 72.20: Two-dimensional echocardiography. Subcostal sagittal

viewbicaval view showing the attachment of suprahepatic
portion of inferior vena cava to right atrium. Right pulmonary
artery is shown in short axis posterior to superior vena cava (*).
(IVC: Inferior vena cava; LA: Left atrium; RA: Right atrium; SVC:
Superior vena cava).

The coronary sinus (Fig. 72.5A): The coronary sinus always

traverses the floor of the LA and opens into RA. It is a useful
marker if present. The apical four-chamber view angled
posteriorly profiles this feature well.
Aortainferior vena cava relation in the abdomen
(Fig. 72.3): This indirect method of inferring the atrial
morphology is the most popular and easily performed
technique by 2D echocardiography. It is very sensitive
and specific in patients of normal or mirror image atrial
arrangement but less so in the group with isomerism. It
is based on the facts that, in patients of normal or mirror
image arrangements, the viscera and great vessels in the
abdomen are lateralized. The subcostal short-axis scan at
the level of diaphragm shows the relation of great vessels
and the spine to each other. Thus, in normally arranged
atria, the aorta is always to the left of the spine and the IVC
to the right, at the level of the diaphragm. This relation is
reversed in mirror image arrangement.

Echocardiographic Identification of
Isomerism
Right and left isomerism should be strongly suspected on
echocardiography when the following findings are present.
Right Isomerism: Also called the asplenia syndrome, this
is strongly suspected on the abdominal scan of the great
vessels. The aorta and IVC lie on the same side of the
spine (left or right) instead of on either side. The aorta
lies posterior to the IVC. The IVC opens into either the

Left isomerism: Also called the polysplenia syndrome,

this can also be suspected on the abdominal scan of great
vessel. The aorta and a venous channel again lie on the
same side of the spine (left or right). The aorta is anterior
and the venous channel lies posterior to it. This venous
channel actually represents the azygos/hemiazygos vein,
which then continues posteriorly through the diaphragm
and joins the left (hemiazygos) or right (azygos) SVC.
The IVC is interrupted and the hepatic veins connect to
the atrium directly. IVC interruption is seen in 85% cases of
left isomerism. For those who do not believe in the concept
of isomerism, the chamber that receives the coronary
sinus/hepatic vein would become the morphological RA.
There are other echocardiographic features by which
isomerism should be suspected although not diagnostic.
Bilateral SVC.
Absent coronary sinus (in right isomerism or asplenia).
Single atrium.
Total anomalous pulmonary venous connection
(TAPVC)The pattern of TAPVC is different in right
and left isomerism.
Total anomalous systemic venous drainage.
Common AV valve.
Discordance between abdominal and atrial situs.

Ventricular Morphology and

Atrioventricular Connection
The connection of the atrial myocardium to the ventricular
mass is called the AV connection. The following types of
connections are recognized (Table 72.3):
Concordant AV connection (Fig. 72.21).
Discordant AV connection (Fig. 72.22).
Univentricular atrioventricular connection (Figs
72.23A and B).
Isomeric AV connection.
Uniatrial and biventricular connection.
Criss-cross AV connection.
To describe AV connection, one should identify the
ventricular morphology.

1578

Section 6: Congenital Heart Disease

Table 72.3: Echocardiographic Identification of Atrioventricular Connection

The offsetting sign and moderator band are best identified by the apical four-chamber view
The chordal attachment of the morphological tricuspid valve to the interventricular septum is best seen in the subcostal
coronal views
The two discrete papillary muscles of left ventricle and the apical trabeculations of right ventricle are best appreciated in parasternal
short-axis views. The transducer is focused inferiorly starting at the base of the heart. The papillary muscles are first seen followed
by the apical trabeculations
Absence of an atrioventricular valve, common atrioventricular valve, and straddling are best seen in the apical views. The common
atrioventricular orifice is also well appreciated in the subcostal sagittal views

Fig. 72.21: Two-dimensional echocardiography. Apical four-chamber view showing all four cardiac chambers and offsetting of atrioventricular valves (attachment of tricuspid valve more toward apex
than mitral valvearrow). (LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle).

Fig. 72.22: Two-dimensional echocardiography. Apical fourchamber view showing discordant atrioventricular connection, right
atrium to left ventricle, and left atrium to right ventricle. Moderator
band on left side (anatomical right ventricle) and reverse offsetting
of atrioventricular valves are seen (arrow).

Figs 72.23A and B: Two-dimensional echocardiography. Apical four-chamber view showing univentricular atrioventricular (AV)
connection. (A) Tricuspid atresia (arrow), hypoplastic right ventricle; (B) Mitral atresia (arrow), with hypoplastic left ventricle (LV).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Echocardiographic Identification of
Ventricular Morphology
The offsetting sign of atrioventricular valves: This sign,
if present, forms the single most important feature in
identification of ventricular morphology. It takes into
account that the AV valve always follows its ventricle.
The tricuspid valve is attached more apically compared
to the mitral valve. Thus, a ventricle which has its AV
valves attached more apically will be the morphological
RV. The disadvantage with this sign is that when a VSD
extends to the inlet septum or in univentricular hearts, this
offsetting sign is lost and therefore cannot be used as a
feature for ventricular morphological identification.
The ventricular septal and apical trabeculation: The
morphologists consider this as the most constant feature of
ventricular morphology. The RV apical trabeculations are
coarse. The moderator band contributes to this coarseness.
The RV side of the septum is seen to be coarser compared
to the LV side of the septum. Echocardiographically, this
feature becomes difficult to identify if one of the ventricles
is severely hypoplastic or there is a true single ventricle.
Attachment of the chordae and papillary muscle to the
septum: The RV side of the septum gains attachment of the
chordae and papillary muscle. The left ventricular side of
the septum is always free of chordal attachment.
Papillary muscles: The LV has two discrete papillary
muscles. The RV has three or more papillary muscles.

Echocardiographic Identification of
Atrioventricular Connection

Concordant AV connection: When the LA (whether

right- or left-sided) connects to the morphological
LV and the RA (right- or left-sided) connects to the
morphological RV.
Discordant AV connection: When the LA (whether
right- or left-sided) connects to the RV and the
morphological RA (right- or left-sided) connects to the
morphologic LV.

Identification of Great Vessels and

Ventricular Arterial Connection
Echocardiographic identification of great vessel connection to the ventricular mass is evaluated in the following
steps.

1579

Identification of Great Vessels

The PA is identified by its immediate bifurcation into left
and right branches. This is best appreciated in the PSAX
view at great vessel level. Other clues are: (a) In the PLAX
view if the posterior great vessel is seen coursing posteriorly
after its origin the vessel is the PA. The aorta always takes
an anterior course. (b) Annular hypoplasia is often the
rule in patients with pulmonary stenosis physiology (like
tetralogy etc.). Then the vessel with a smaller annulus
and with turbulent flow (on color or pulsed Doppler) is
often the PA. (c) The aorta does not give rise to any branch
before the carotids across the coronaries and it gives rise to
the arch vessels. The coronary arteries are useful pointers
to identify aorta, but because of their variable origin (e.g.
the PA) they cannot be used as a criterion for a great vessel
to be labeled as the aorta.

Identification of Connection of Great Vessels

A combination of views are used to identify great vessels
and their connection.
Starting from the subcostal coronal scan, the
transducer is tilted superiorly in a gradual fashion. The
great vessels appear sequentially as the transducer is
tilted superiorly, the posterior great vessel is seen first and
then the anterior great vessel. During this process, the
great vessel connections with the ventricles can also be
identified.
Apical viewAs the transducer is tilted superiorly from
the level of AV valves to the great vessel, the connection
with the ventricles can be identified.
PLAX viewIn this view if the two vessels can be clearly
seen in the same plane and have a side-by-side relation,
the two great vessels are likely to be transposed. Not
only the continuity of the ventricle with the great vessels
is identified in this view, but also the degree of override
[to classify the anatomy, for example, double outlet right
ventricle (DORV)] can be assessed in this view.
Univentricular atrioventricular connection: When both
the atria connect predominantly to one ventricle it is
called the univentricular connection. Most often the other
ventricle can be identified echocardiographically and is
rudimentary. Rarely one can have true absence of another
ventricle (true single ventricle). The atria can connect
to one ventricle by two valves (double inlet ventricle,
common AV valve, or a single AV valve, the other valve
being absent like in mitral or tricuspid atresia). When two
AV valves are committed to one main ventricular mass
there can be a wide spectrum of commitment. At one end,

1580

Section 6: Congenital Heart Disease

both AV valves are completely committed to one ventricle,

the other ventricle being devoid of an inlet. Then there
can be a varying degree of commitment of one AV valve
to the dominant ventricle. In such cases, the 50% rule is
used, wherein, if one AV valve is committed more than
50% to a ventricle, then it is ascribed to that ventricle. The
reason for commitment is due to straddling/overriding of
a valve through the VSD. Overriding refers to the AV valve
annulus and straddling occurs when the tensor apparatus
crosses over to the contralateral ventricle. Usually both
exist together but each can be independent of the other.
Tricuspid valve always straddles through an inlet VSD and
the mitral valve through an outlet VSD.
Isomeric atrioventricular connection: This terminology
is used by the group, which believes in the concept of
isomerism. In such cases, since both atria are isomeric
(right or left), the connection cannot be concordant or
discordant as per definition. Subsequent description of
the ventricles is done by using the loop method (discussed
later).
Uniatrial and biventricular connection: In these cases one
AV junction is absent but the other AV junction overrides
the ventricular septum with the valve of this junction
straddling both ventricles, thereby creating a biventricular
connection.

Atrioventricular Connections and the

Loop Rule
In the presence of concordant AV connection in normally
arranged atria, there will always be a D-loop (or right
hand topology), and in the presence of discordant AV
connection in normally arranged atria, it will always be
an L-loop (or left hand topology). The reverse applies in
patients of mirror image atrial arrangement (L-loop in
concordant AV connection and D-loop with discordant
AV connection). There are descriptions of hearts with
concordant AV connections with normally arranged atria
but with an L-loop and also vice versa. Thus, although in
the majority of cases the loop rule holds true and can be
inferred, it was felt necessary to mention the loop in the
sequential description of congenitally malformed heart
because of these rare examples. Also, in criss-cross AV
connections, there may be normally arranged atria and AV
concordance with L-looped ventricles.
D-Loop (right hand topology): It implies that the RV is
to the right of the LV in normally arranged atria and vice
versa in mirror-imaged atria.

L-Loop (left hand topology): It implies that the RV is to the

left of the LV in normally arranged atria and vice versa in
mirror imaged atria.
These features can be easily made out by 2D echocardiography.

Ventriculoarterial Connection
The ventriculoarterial connections can be divided into the
following types:
Concordant ventriculoarterial connection.
Discordant ventriculoarterial connection.
Double outlet ventricle (right or left).
Single outletpulmonary atresia, aortic atresia.
Common outlet.

Concordant Ventriculoarterial Connection

The LV is connected to the aorta and the RV to the PA.
Discordant ventriculoarterial connection (Fig. 72.24): The
LV is connected to the PA and the RV to the aorta. It is also
called transposition of great arteries.
Double outlet ventricle (Fig. 72.25): (1) Double Outlet Right
VentricleWhen the PA and > 50% of the aorta [the VSD
type] or the aorta and > 50% of the PA (the transposition
type) arises from the RV, it is called DORV. Some authors
would define DORV only when > 90% of a vessel is
committed to the ventricle.
The present definition of DORV is based on connections
of the great vessel to the ventricle. Some groups of authors
feel that double outlet ventricle should be defined only

Fig. 72.24: Two-dimensional echocardiography. Parasternal longaxis view showing ventriculoarterial discordance. (AO: Aorta; LV:
Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

in terms of the conal morphology. Thus, these groups of

workers would define DORV only when there is bilateral
coni, that is, there is aortomitral and tricuspid pulmonary
or mitral pulmonary and tricuspid aortic discontinuity.
(2) Double Outlet Left Ventricle (Fig. 72.26)When the
aorta and >50% of the PA or PA and >50% of aorta arise
from the LV, it is called double outlet left ventricle (DOLV).
In this condition, there is bilateral absence of the conus,
that is, both the semilunar valves are in continuity with the
AV valves.

1581

ventricle and the other great vessel is atretic and cannot

be traced to the ventricular mass. The commonest in
this group is pulmonary atresia. The echocardiographer
needs to distinguish between an absent connection and
an imperforate valve. In the latter, there is a potential
communication between the great vessel and the ventricle
and therefore can be designated as concordant or
discordant connection.

Single outlet (Figs 72.27A and B): This is recognized

when there is only one great vessel arising from the

Common outlet (Figs 72.28A and B): In this condition, a

common trunk arises from the ventricles and gives rise to
aorta, PA, and the coronaries. It is also called the truncus
arteriosus.

Fig. 72.25: Two-dimensional echocardiography. Apical four-chamber view with anterior tilt showing double outlet right ventricle with
nonrestrictive inlet ventricular septal defect (VSD; *). (AO: Aorta;
LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

Fig. 72.26: Two-dimensional echocardiography in subcostal coronal

view with anterior tilt in a case of double outlet left ventricle showing both the great arteries coming off from the left ventricle. (AO:
Aorta; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

Figs 72.27A and B: A case of ventricular septal defect and pulmonary atresia. Subcostal paracoronal view with color compare showing
the atretic right ventricular outflow tract (arrow) and ventricular septal defect (*). (AO: Aorta; PA: Pulmonary artery.

1582

Section 6: Congenital Heart Disease

Figs 72.28A and B: Two-dimensional echocardiography of an infant with truncus arteriosus. (A) Subcostal coronal view with anterior
tilt showing the single outflow-truncus arising from the ventricles; (B) Parasternal short-axis view showing the pulmonary arteries arising
from the common trunk and bifurcating into left and right pulmonary arteries. (LPA: Left pulmonary artery; LV: Left ventricle; MPA: Main
pulmonary artery; RPA: Right pulmonary artery; T: Truncus).

Connections and Spatial Relationship

One has to differentiate between connections and
spatial relationship of great vessels. Connections refer to
the way the great arteries are aligned to the ventricular

mass, whereas spatial relationship refers to the way the

great vessels are related to each other. The two features
are independent of each other and should be separately
defined echocardiographically.

PART 2: LEFT-TO-RIGHT SHUNTS: ATRIAL SEPTAL DEFECT, VENTRICULAR SEPTAL

DEFECT, PATENT DUCTUS ARTERIOSUS, AND AORTOPULMONARY WINDOW
In this section, echocardiographic findings of common
shunt lesions are discussed. The addition of Doppler and
color flow mapping also gives physiological information
about flow and pressures and enables the pediatric
cardiologist to refer patients for surgical treatment without
cardiac catheterization, especially in neonates and infants.
The commonly seen shunt lesions include ASD, VSD,
patent ductus arteriosus (PDA), and aortopulmonary
window (APW).

GENERAL FEATURES: SHUNT LESIONS

There are a few salient features of all the shunt lesions.
The shunt would lead to volume overload of the
chambers it feeds, generally described in relation
to the tricuspid valve. If a shunt is proximal to the
tricuspid valve, it would lead to volume overloading of
the RA and RV, often referred to as pre-tricuspid shunt

(Fig. 72.29). If a lesion is beyond the tricuspid valve, it

would lead to the volume overloading of the LA and LV,
often referred to as post-tricuspid shunts (Fig. 72.30).
The magnitude of the chamber enlargement depends
upon the magnitude of the shunt (in the absence of
anemia). Thus, significant RA and RV enlargement
would be a feature of pre-tricuspid shunt, while a
significant LA and LV enlargement would be a feature
of post-tricuspid shunt (Fig. 72.30). The dimension
of the chambers can be compared with standardized
values to ascertain chamber dilation (e.g. by using
z-scores).
The pressure of the investigated chamber can rise
on account of distal obstruction (obstruction of the
outflow of the chamber) or it would be because of
the transmitted pressure from the high pressure
communicating chamber.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1583

Fig. 72.29: M-mode echocardiography of a pretricuspid shunt

showing enlarged right ventricle and volume overloading of the
right ventricle with flattened interventricular septal motion in an
infant.

Fig. 72.30: M-mode echocardiography at the ventricular level

(post-tricuspid shunt) showing dilated left ventricle (LV) with
normal septal motion in a neonate.

Increase in RV pressures may be because of infundibular or valvular pulmonary stenosis, obstruction in branch pulmonary arteries, or obstruction
in pulmonary vascular bed (as in elevated pulmonary vascular resistance).
Increase in pressures in RV will also be seen in large
VSD; similarly, a large PDA would lead to significant
increase in PA pressures due to direct transmission
of pressures.
The magnitude of the gradient from a chamber outflow
would also be dependent on the magnitude of shunt
into the chamber (Fig. 72.29).
This may lead to exaggerated gradients even in
hemodynamically insignificant lesions, namely
exaggerated pulmonary gradients in pre-tricuspid
shunts (like ASD), exaggerated mitral and aortic
valve gradients in post-tricuspid shunts like VSD or
PDA.
The secondary manifestations of the shunt lesions may
themselves lead to exaggerated secondary effects, for
example, dilatation of LV leading to the mitral annular
dilatation on account of post-tricuspid shunt may lead
to mitral regurgitation and this may further lead to
mitral annular dilatation and LV dilatation.
Since a shunt lesion is a communication between two
chambers, the gradient between the two chambers will
be dependent on the size of the defect (Figs 72.31A and B).
The size of the defect in two dimensions may be a
useful guide in deciding the degree of shunt.

The size of the VSD may be compared to the size of

the aortic root for classifying the size of the VSD as
large, moderate, or small.
The size of defects like ASD, VSD, would help in
deciding the size of the device, which may be used
to close these defects.
Echocardiography should focus not only on the
characteristics of the primary lesion, but also on
the structures adjacent of the defect, for example,
distances from the adjoining valves.
VSDIt is important to note the distances from the
aortic valve and tricuspid valve when considering
for device closure.
For ASD, the rims are seen not only for their
adequacy to hold the device but also the adjoining
structures which may be encroached whenever
contemplating a device closure (Figs 72.32A to C).
For APW, the distance of the defect from coronaries
and valves becomes important.
The post-tricuspid shunt is known to mask
the manifestations of an anomalous LCA from
pulmonary artery (ALCAPA), and thus, one should
carefully look at the 2D anatomy and color flow
mapping to define the origin of the coronary
arteries whenever investigating a post tricuspid
shunt lesion.
Whenever investigating a shunt at multiple sites or an
associated lesions, one must remember that the shunt
flow may get exaggerated by the presence of distal
obstruction and also by the associated shunt.

1584

Section 6: Congenital Heart Disease

Figs 72.31A and B: (A) Continuous wave Doppler signal of a patent ductus arteriosus, showing peak gradient of 89 mm Hg, systemic
pressures were 100 mm Hgestimated pulmonary artery (PA) pressures will be 11 mm Hg; (B) Shows the peak systolic gradient across
the ventricular septal defect (VSD) of 98 mm Hg, systemic pressures were 120 mm Hg. By these observations, the predicted right
ventricular (RV) systolic pressure would be 22 mm Hg.

Figs 72.32A to C: Two-dimensional transesophageal echocardiography. (A) Four-chamber view showing atrial and atrioventricular
valve rims (arrows); (B) Basal short-axis view showing the atrial and
aortic rims (arrows); (C) Modified basal long-axis view, showing the
superior vena caval and inferior vena caval rims (arrows).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

The associated post-tricuspid shunt may lead to

exaggerated manifestations of pre-tricuspid shunt
lesions (e.g. ASD). Thus, RA and RV may be unduly
dilated even in the presence of small ASD, with the
associated presence of post-tricuspid shunt (VSD
or PDA) or associated presence of mitral stenosis.
The associated aortic stenosis or coarctation of
aorta (CoA) may exaggerate the shunt across the
VSD.
The associated obstructive lesions distal to the shunt
lesions may become masked and may manifest
themselves only after the shunt lesion is closed.
Mitral stenosis may not manifest itself in the
presence of ASD (although it may exaggerate the
shunt flow across it).
The manifestations of significant mitral regurgitation may get unmasked after ASD closure.
High left ventricular end-diastolic pressure
(LVEDP) may not only exaggerate ASD shunt, but
also lead to pulmonary edema after ASD closure.
VSD or PDA may mask the gradients across aortic
stenosis or CoA, which may manifest after the
treatment of the underlying shunt lesion.
Certain systemic disorders and conditions may exaggerate or confound the echocardiographic features of
a shunt lesion.
Anemia may exaggerate the gradients across any
shunt lesion or across valves. Anemia may lead to
LV dilatation, thus confounding the assessment of
post-tricuspid shunt lesion.
Systemic hypertension may not only lead to
exaggerated shunt gradients, but also result in
secondary ventricular hypertrophy, thus leading to
high LVEDPs and exaggerating ASD shunt.
Hyperdynamic states such as fever and thyroid
disorders may exaggerate the shunt gradients.
Thus, an assessment of a patient with a shunt lesion
does not mean an isolated evaluation by echocardiography;
it refers to complete clinical evaluation of the patient.
Now we will discuss individually the assessment of shunt
lesions seen commonly.

1585

Determining the volume overload, pulmonary arterial

pressures, associated lesion, and complications.
Defining the lesions operability.
Deciding the relevant modality of treatment.
The essential approach to any lesion should not
be directed at the shunt lesion, rather it should be a
standardized sequential analysis, as it is not the shunt
lesion in isolation that exists and one needs to evaluate all
the structural heart defects. A few salient features of the
important shunt lesionsASD, VSD, PDA, and APWare
discussed below.

ATRIAL SEPTAL DEFECTS

Objectives (see Also Table 72.4)
To diagnose ASD, assess the following:
Its anatomical site and size.
The direction and quantum of flow.
The degree of pulmonary arterial hypertension.
AV valve anomalies, pulmonary veins, and pulmonary
valve stenosis.

Atrial Septal Defect Types1,2,14,15

Echocardiography plays a major role in the evaluation of
ASD. Defects of atrial septum are classified as follows.

Patent Foramen Ovale

Foramen ovale is a passage between septum secundum
on right side and septum primum on left side. Its patency

Step-wise Approach
(On Echocardiography)
Step-wise approach (on echocardiography) for evaluation
of any shunt lesion involves:
Determining the presence of shunt lesion (Fig. 72.33).
Defining its location and size of defect (Figs 72.32A to C).

Fig. 72.33: Two-dimensional transesophageal echocardiography.

Basal long-axis view with color flow mapping showing left-to-right
shunt flow. No additional defects are seen. (LA: Left atrium; RA:
Right atrium).

1586

Section 6: Congenital Heart Disease

Table 72.4: Stepwise Evaluation for an Atrial Septal Defect

Initial indication is the volume overload of the chambers [right atrium (RA) and right ventricle (RV)] in 4C view
Visualize the defect from subcostal view (sagittal and coronal views)
Determine the site of defect: fossa ovalis or other
Determine the direction of shunting
Assess associated structures, particularly pulmonary veins and atrioventricular (AV) valves
Assess pulmonary artery pressures: tricuspid regurgitation (TR) and pulmonary regurgitation (PR) gradients
Determine suitability for device closure

These limbic bands separate the defect from atrial wall.

These defects could be single, fenestrated mesh-like, or
multiple.

Sinus Venosus Atrial Septal Defect

(Figs 72.36A and B)
The hallmark of all of these defects is that the border of
the fossa ovalis should be intact, the defect is overlapped
by either SVC in SVC type and by IVC in IVC type sinus
venosus defect. There may be an associated anomalous
drainage of pulmonary veins. Defects extending from the
fossa ovalis superiorly or inferiorly should not be classified
as a venosus defect.
Fig. 72.34: Two-dimensional echocardiography. Subcostal bicaval
view with color view mapping showing fossa ovalis atrial septal
defect with left-to-right shunt. (SVC: Superior vena cava; RA:
Right atrium; LA: Left atrium).

is a must for fetal survival and for normal growth of the

fetal heart. After birth, the foramen ovale closes as left
atrial pressure rises due to increased pulmonary venous
return with fall in PVR. If pressure on either side of atria
rises, stretching of flap of foramen ovale occurs and leads
to shunting across foramen ovale. In most individuals, the
foramen ovale is functionally closed shortly after birth;
however, patency of a competent foramen ovale has been
found in 25% of normal hearts on autopsy.

Fossa Ovalis Atrial Septal Defect

(Figs 72.34 and 72.35)
It is the commonest of the ASDs (69%) with varied sizes.
This type of defect occupies the central part of atrial
septum involving in part or whole flap valve of the foramen
ovale. Septum ovale defects are bounded on either side by
the limbic bands (superior and inferior limbic bands).

Coronary Sinus Atrial Septal Defect

(Fig. 72.37)
Coronary sinus ASD is a rare anomaly. It is located in the
inferior most part of the atrial septum at the anticipated
site of the coronary sinus ostium. The clue to the diagnosis
of such a defect is the presence of a persistent left superior
vena cava (LSVC) with evidence of RV volume overload.

Ostium Primum Atrial Septal Defect

(Fig. 72.38)
This defect is present in the lower most part of the atrial
septum. This type of defect may be part of atrioventricular
septal defect (AVSD) and may be characterized by the
absence, in part or whole, of the atrioventricular septum.

Echocardiographic Imaging in
Atrial Septal Defects
Enlarged RA, RV, and paradoxical septal motion of
interventricular septal motion are indirect evidence of

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1587

Figs 72.35A and B: Two-dimensional echocardiography. Subcostal bicaval view with color comparison showing fossa ovalis atrial
septal defect with adequate superior vena cava (SVC) rim (upper
arrow) and deficient inferior vena cava (IVC) rim (lower arrows) and
left-to-right shunt. (LA: Left atrium; RA: Right atrium; SVC: Superior
vena cava).

Figs 72.36A and B: Two-dimensional echocardiography. Subcostal bicaval view with color comparison showing the superior vena cava
(SVC) type of sinus venosus atrial septal defect. SVC is overriding the defect with partial anomalous pulmonary venous drainage of
right upper pulmonary vein to SVC. (LA: Left atrium; RA: Right atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena cava).

left-to-right shunt at atrial level. The best views to directly

visualize the ASDs are subcostal coronal and sagittal views.
ASD can be diagnosed by a drop out in the interatrial
septum with flow across the defect on color flow mapping.
When the defect is visualized, its relationship to the SVC
and IVC should be evaluated. If the SVC forms the roof of
the defect, it is SVC sinus venous type. If the IVC straddles

the defect, it is IVC sinus venosus type. The defects in the

centre of the atrial septum involving the fossa ovalis area
are fossa ovalis defects. The defect in the lower most part of
the interatrial septum with atrioventricular valves attached
at the same level are designated as ostium primum defects.
The imaging should also be done from apical fourchamber and short-axis views. The four-chamber view will

1588

Section 6: Congenital Heart Disease

Fig. 72.37: Two-dimensional echocardiography. Subcostal coronal

view with posterior tilt showing coronary sinus type of atrial septal
defect (arrow). (LA: Left atrium; RA: Right atrium).

Fig. 72.38: Two-dimensional echocardiography. Apical fourchamber view showing a large ostium primum atrial septal defect
(ASD) defect in lowermost part of the interatrial septum (arrow) with
absence of offsetting of the atrioventricular (AV) valves (arrow). (LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

also show the attachments of the AV valves. These may be

at the same level in ostium primum defect. The color flow
mapping across the defect will show the direction of flow
and also presence or absence of any atrioventricular valve
regurgitation. Doppler velocities across all valves should
be taken, in particular the pulmonary valve to look for any
pulmonary stenosis.
The inflow velocities of the AV valves should be seen
to rule out any mitral valve obstruction. The associated
mitral obstruction may be missed unless specifically seen
on 2D echocardiography, as there may not be a significant
gradient across the mitral valve even with significant
obstruction because of an associated ASD (true for all
Lutembacher cases). All pulmonary veins should be
specifically imaged to see if they are abnormally connected
or not and to look for any pulmonary vein stenosis. The
pulmonary veins are best seen in subcostal coronal and
sagittal, apical four-chamber, PSAX, and suprasternal
short-axis views.

than right atria. The second wave of left-to-right shunt

occurs with atrial contraction. A small right-to-left shunt
occurs during early systole when right atrial pressure
exceeds left atrial pressure because of unequal activation
time of the two atria. A second wave of right-to-left shunt
occurs with rapid filling phase in early diastole. During that
time, IVC flow tends to flow toward the LA. Respiration
has some effect on direction of shunting; with inspiration,
the left-to-right shunt decreases, while the reverse occurs
during expiration.
For the estimation of pulmonary arterial pressure, the
peak gradient of tricuspid regurgitation gives the systolic
pressure of the RV in absence of RV outflow obstruction. If
pulmonary regurgitation is present, the pressure derived
from the peak diastolic velocity will reflect the pulmonary
arterial mean pressure.
Doppler echocardiography accurately depicts the
direction of shunting.
Pulsed Doppler: Pulsed Doppler shows the typical flow
pattern as discussed earlier.
Color flow mapping (Figs 72.33 and 72.39):
With 2D imaging, color flow mapping clearly shows
the net direction of flow.
M-modeThis is seldom used in daily practice but
depicts best the direction of shunting during the
various phases of a cardiac cycle.

Direction of Shunt1416
With an isolated uncomplicated ASD, pressure between
the two atria is similar with a variation up to 5 mm Hg, with
cardiac cycle and phases of respiration. Dominant shunt
occurs from left to right. Left-to-right shunt occurs mainly
during mid to late systole as the v-wave of LA is larger

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1589

Figs 72.39A and B: A case of total anomalous pulmonary venous drainage in a 2-year-old child. Two-dimensional echocardiography
with color compare in subcostal sagittal view showing right-to-left shunt across atrial septal defect (arrow). (LA: Left atrium; RA: Right
atrium).

Doppler and CFI should always be performed for the

following reasons:
To confirm the presence of an echo dropout seen on
2D imaging. The characteristic pulse Doppler tracing
and/or CFI confirms the echo dropout as a true defect.
Interrogation of the AV and semilunar valves for
regurgitation and stenosis. In this regard, the presence
of trivial to mild tricuspid or pulmonary regurgitation
is a universal finding in large ASDs and is related
to RV dilatation leading to tricuspid annulus and
PA dilatation due to large pulmonary blood flow.
Increased forward velocity across the pulmonary
valves up to 3 to 4 m/s may be seen with large ASDs
and does not always indicate pulmonary stenosis. The
pulmonary valves in these cases are morphologically
normal and there is no doming of the valve.
The tricuspid regurgitation velocity should always be
obtained to predict the RV systolic pressure and hence
indirectly the PA pressure.
Interrogation of the pulmonary veins should be done
to rule out the association of pulmonary venous
obstruction.

Objective Calculation of Qp/Qs Ratios

Pulmonary and systemic flows can be calculated by the
2D and Doppler echocardiogram. The following formula is
being used to calculate the flows:
SV =
SV :
V :
CSA :
RR :

V CSA RR
1000 mL /1

stroke volume.
mean velocity (velocity time integral).
Cross-sectional area of flow (cm2).
R-to-R interval (s/beat).

Cardiac output = stroke volume multiplied by heart rate

and the (heart rate equals 60,000/RR interval), then the
cardiac output will be
V CSA 60s /min
1000 mL /1
To calculate pulmonary blood flow (Qp), PA mean
velocity and diameter of pulmonary outflow can be
measured from PLAX view of right ventricular outflow
tract (RVOT) or PSAX view.

1590

Section 6: Congenital Heart Disease

To calculate the systemic blood flow (Qs), mean

velocity across left ventricular outflow and the diameter of
left ventricular outflow can be measured. To calculate the
aortic mean velocity, apical five-camber view is used; the
sample volume is kept just above the aortic valve leaflets.
Other views that can be used to measure the aortic mean
velocity are subcostal and suprasternal views.
To measure the left ventricular outflow diameter,
PLAX view is the best view. Other views that can be used to
measure the left ventricular outflow diameter are subcostal
and suprasternal.
While taking the mean velocity, the Doppler beam
should be positioned as parallel as possible to the flow, so
that no correction for intercept angle needs to be made.
To calculate the systemic and pulmonary blood flow,
in place of left and RV outflow, we can use mitral and
tricuspid mean velocity and annulus diameter in apical
four-chamber view assuming no regurgitation in patients
without shunt lesions. In patients with atrial shunt, the
tricuspid flow will represent pulmonary blood flow and
mitral flow will represent systemic flow. But in ventricular
and aortopulmonary shunts, the mitral flow will represent
the pulmonary blood flow + the systemic blood flow and
aortic flow will represent the systemic flow.
After calculating the systemic and pulmonary blood
flow, left-to-right shunt can be calculated by subtracting
systemic blood flow from pulmonary blood flow or the
ratio of pulmonary and systemic blood flows, Qp/Qs can
be calculated.

Limitations of Technique of Estimation of

Degree of Left-to-Right Shunt
Various Doppler methods to calculate Qp/Qs have been
described, but there are several possible sources of error
while Doppler is being used to calculate the flows; so we
do not use this routinely.
Errors in the measurement of mean velocity due to:
Errors in determining the intercept angle.
The lack of a uniform velocity profile across the
vessel lumen.
Variation caused by respiration or other physiological factors. During respiration, the variability in the
velocity time integral at mitral and tricuspid valve
are 14.5% and 13.2%, respectively.
Errors in measurement of cross-sectional area due to
Inaccurate gain settings.
Due to pressure, flow, and elasticity of vessel, the
cross-sectional area of vessel changes throughout

the cardiac cycle and in various phases of respiration, particularly in the PA .

Measurement in the direction of lateral resolution.

Indication of Cardiac Catheterization

Diagnostic cardiac catheterization in ASDs is indicated in
those instances where correct evaluation of PA pressure
is not possible by Doppler echocardiogram, pulmonary
hypertension is suspected and information on PVR is
required in decision making. This is because, although PA
pressure may be reliably predicted by Doppler calculation,
calculation of flow data is fallacious and can introduce
error in the calculation of PVRs. In cases with elevated
PVR, the reactivity of the pulmonary vascular bed can also
be tested during cardiac catheterization.

Evaluation of Atrial Septum by

Transesophageal Echocardiography1,2,16
In pediatric age group, transthoracic echocardiography
using subcostal view provides a complete diagnosis and
good assessment of ASD for interventional or surgical
closure. In adolescents and adults, as subcostal windows
do not provide adequate penetration, transesophageal
echocardiography (TEE) is required for detailed profilation
of the ASD.
Views that are most useful for evaluation of ASD on
TEE are:
Basal short-axis view.
Bicaval or basal long-axis view.
Four-chamber view.

Basal Short-Axis View

This view can be obtained by keeping the endoscope in the
middle part of esophagus. This view provides imaging of
aortic valve and atrial septum. The aortic and atrial rims
can be best seen in this view. The fossa ovalis defect is seen
in middle part of atrial septum, sinus venosus defect SVC
type is seen in the upper part of the septum adjacent to
SVC, and the sinus venosus IVC type of ASD is seen in the
lowermost part of septum adjacent to IVC.

Basal Long-Axis or Bicaval View

This view can be obtained by keeping the endoscope at the
same level and rotating the icon to 80 to 100. This profiles
the ASD in relation to superior and IVC, fossa ovalis defect
is seen in middle part of septum. Sinus venosus defect

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1591

Table 72.5: Stepwise Evaluation for Ventricular Septal Defect

Initial indication is the volume overload of the chambers [left atrium (LA) and left ventricle (LV)]
Visualize the defect from all possible windows
Determine the presence of additional defects (screening the septumseptal sweep in subcostal, apical four-chamber, parasternal
short- and long-axis views in color and B-mode)
Determine the direction of shunting through the defect
Assess associated defects, particularly outflow obstructions and adjacent structures
Assess pulmonary artery pressures: ventricular septal defect (VSD) gradients, tricuspid regurgitation (TR), and pulmonary regurgitation (PR) velocities
Determine the volume overload of chambers (z-scores of LV, particularly in M-mode)
Determine suitability for device closure or surgery

is seen in relation to SVC with SVC type of defect or in

relation to IVC with IVC type of defect along with partial
anomalous venous drainage of pulmonary vein. In case
of fossa ovalis ASD, superior vena caval and inferior vena
caval rims can be accurately assessed in this view.

view and apical four-chamber views. The aortic rim is best

seen in transthoracic echocardiography PSAX view. The
superior vena caval and inferior vena caval rims are best
visualized on transthoracic echocardiography in subcostal
sagittal (bicaval view).

VENTRICULAR SEPTAL DEFECT

Four-chamber View
Four-chamber view can be obtained by keeping the
endoscope at the lower part of esophagus. This view
profiles the atrial septum with fossa ovalis defect in the
middle of septum, atrial and AV valve rims, and volume
overloaded RA and RV. By rotating the endoscope, we
can see the attachment of right and left pulmonary veins
draining into LA. In this view mitral valve morphology and
mitral regurgitation can also be assessed.

Objectives (Table 72.5)

Confirmation of VSD.
Determination of the size and morphological location
of VSDs.
Ruling out associated lesions.
Assessment of chamber size and wall thickness.
Estimation of shunt size (pulmonary/systemic flow
ratio).
Estimation of RV and pulmonary arterial pressures.

Assessment of Fossa Ovalis Atrial Septal

Defect for Percutaneous Device Closure

With the availability of various devices for the closure

of ASD, the importance of detailed anatomy of the ASD
including rims around the defect and the relationship
with the surrounding structures has become of great
importance. 2D echocardiography, both transthoracic
and transesophageal, clearly show the anatomy and is
used for selecting the cases for device closure of the ASD.
During the procedure of device closure, TEE serves as the
most important landmark for the proper placement of the
device. We have proposed that for clarity, uniformity, and
mutual communication, the rims should be designated
according to the structure they are related, for example,
superior vena caval, inferior vena caval, AV septal (rim at
the crux), aortic, and atrial rims (rim of the superior wall of
atrium near the right upper pulmonary vein).
The AV septal and atrial rims are best visualized on
transthoracic echocardiography in subcostal coronal

VSDs should be imaged from several planes. Artifactual

dropouts may confuse the viewer using single plane
imaging about the presence or absence of a VSD,
particularly if it is small. The addition of color Doppler flow
imaging is useful to reconfirm the presence of VSDs. CFI
of VSDs has also radically improved the ability to detect
unusually located and/or very small VSDs.
Morphological location of VSD is described as
viewed from the RV. VSDs can be classified into following
types1,2,1822 (Fig. 72.40):
Perimembranous VSD
Muscular VSD
Muscular inlet
Muscular outlet
Trabecular defect
Doubly committed VSD
Inlet VSD

Morphological Location17,18

1592

Section 6: Congenital Heart Disease

Perimembranous (Figs 72.41A and B)

To identify a VSD as perimembranous, it is essential to
demonstrate involvement of the membranous septal
area. Cross-sectional echocardiography shows a discrete
area of septal dropout in the area of the interventricular
membranous septum adjacent to posterior semilunar

valve and tricuspid valve, with the membranous septum

completely excavated away. However, perimembranous
defects are not restricted to the membranous septum
but always extend into one or more of the neighboring
subunits of the muscular septum. Such defects are then
subdivided into three distinct groups on the basis of the
major extension of the defect.
Perimembranous outlet defect: These defects excavate
anteriorly from the area of interventricular membranous
septum into the subarterial portion of the muscular outlet
septum. They are called perimembranous subaortic
defects. Such defects are not seen in a scan through the
four-chamber plane and will only be seen when scanning
the four-chamber plus aortic root plane, that is, fivechamber view or long-axis views. The defects are clearly
roofed by the roof of the posterior great artery, with the
trabecular septum and tricuspid valve forming their
inferior rim. Large perimembranous outlet defects, which
extend anteriorly below the whole great vessel root, are
consistently visualized in the precordial long-axis plane,
and are also visualized using serial short-axis scans.

Fig. 72.40: Schematic diagram of the interventricular septum with

removed right ventricular (RV) cavity from the RV side. Various
parts of the interventricular septum are shown: bluemuscular
septum, dark yellowperimembranous region, purplethe inlet
septum, and light brownoutlet septum. (Ao: Aorta; IVC: Inferior
vena cava; Mod.: Moderator band; PB: Parietal band; RA: Right
atrium; SB: Septal band; SVC: Superior vena cava).

Perimembranous inlet defects: In these defects, the area

of septal dropout associated with the defect extends
posteriorly into the four-chamber plane as the defect
excavates through the muscular inlet septum toward the
crux of the heart. The roof of this posterior extension is
formed by the atrioventricular junction tissue enclosed by
the septal aspects of the mitral and tricuspid valve annuli.
Anteriorly, this roof is formed by central fibrous body and
AV muscular septum and posteriorly by the AV fibrous
plane. Perimembranous inlet defects will be consistently

Figs 72.41A and B: Two-dimensional echocardiography. (A) Apical four-chamber view with anterior tilt showing the perimembranous
ventricular septal defect (VSD) (arrow) getting restricted by septal leaflet of the tricuspid valve in two dimensions; (B) Color flow mapping of the same patient showing the turbulent jet of the restricted VSD with small left ventricle (LV)-to-right atrium (RA) shunt (arrow).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1593

visualized in a four-chamber plane roofed by AV valve

septal leaflets, which insert into the central fibrous body
at a common level. Scanning anteriorly to the junction
of the four-chamber plane with the four chambers plus
aortic root plane will demonstrate the involvement of
the membranous septum by the defects. An isolated
perimembranous inlet defect will not be visualized in any
of the other standard echocardiographic planes recorded.

body or an AV or arterial valve ring. Since it is impossible

to differentiate the subunits of the muscular septum
accurately using cross-sectional echocardiography,
correct classification of any muscular defect depends on
accurate knowledge of where each subunit of the muscular
septum is visualized within the various planes available to
the echocardiographer. With this knowledge, it is possible
to differentiate the muscular defects into three types.

Perimembranous trabecular defects: In the normal heart,

the inferior border of the membranous septum merges
imperceptibly into the trabecular septum. So it is not
surprising that cross-sectional echocardiography can
neither determine the precise junction of these two
subunits, nor can it demonstrate involvement of the
trabecular septum in a perimembranous defect. However,
a correlation of echocardiography with morphological
findings suggest that two echocardiographic features will
indicate inferior extension of a perimembranous defect
into the trabecular septum: (a) a broad blunt upper
end of the interventricular septum forming floor of a
perimembranous defect at the posterior aspect of a scan
through the four-chamber plus aortic root plane, and (b)
where any such defect is seen to extend inferiorly for more
than half the aortic root diameter.

Muscular inlet defects: These defects lie within the boundaries of the smooth inlet septum and will be visualized by
scanning through the four-chamber plane. They are not
visualized in any other of the echocardiographic planes,
and their characteristic feature, which allows differentiation from the perimembranous inlet defects, is that the
upper muscular rim of the defect is separated from the AV
junction by a muscle bar. Thus, in muscular inlet defects,
the AV junction morphology is normal compared to the
abnormal morphology in perimembranous inlet defects.

The morphological feature diagnostic of a muscular defect

is that its rims are formed entirely by muscle and does
not include the fibrous tissue of either the central fibrous

Muscular outlet defects: These defects have entirely

muscular rims and are located in the smooth muscular
outlet septum below the anterior portion of the aortic root
and the subpulmonic infundibulum. This septal subunit is
a small and extremely elusive structure. It is only profiled
accurately in one echocardiographic view, subcostal RV
outflow plane, but it can also be seen in PSAX view. In
these planes, the defects are seen to be located mainly
in the anterior muscular outlet-septum separated from
the pulmonary valve by the muscular infundibulum. By
definition, they never extend posteriorly to involve the
membranous septum. Muscular outlet defects may occur
in isolation or may present part of a complex lesion.

Muscular Ventricular Septal Defects

(Figs 72.42 and 72.43)

Figs 72.42A and B: Two-dimensional echocardiography in subcostal coronal view (A) with anterior tilt and color compare; (B) Showing
outlet muscular ventricular septal defect (VSD; arrow). (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

1594

Section 6: Congenital Heart Disease

Figs 72.43A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view showing the apical muscular
ventricular septal defect (VSD) with left-to-right shunt.

Figs 72.44A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view showing the doubly committed
ventricular septal defect (VSD) with left-to-right shunt.

Muscular trabecular defect: These defects are divided into:

(a) Single trabecular defectThese are best visualized
on short-axis scanning from the apex of the heart to the
great artery roots or long-axis scanning of septum in
different planes. Defect size is closely related to the ability
of the cross-sectional system to identify the defect. (b)
Multiple trabecular defects (Swiss cheese defects)These
defects may not be directly visualized by cross-sectional
echocardiography. Multiple defects burrow through
the septum obliquely and may not produce a complete
echocardiographic window across the width of the
trabecular septum, so that the septum may appear intact

on cross-sectional echocardiography. CFI is helpful also in

defining multiple muscular defects.

Doubly Committed Subarterial Defects1,23,24

(Figs 72.44A and B)
Doubly committed subarterial defect is roofed by conjoint
aortic and pulmonary valve rings that appear to lie at
the same level. In these defects, the ventricular septum
will appear intact throughout the four-chamber plane.
Doubly committed subarterial defects are seen from the
four-chamber view with anterior tilt, paracoronal view,

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

parasternal long-axis, and PSAX views. Scanning anteriorly

into the four chambers plus aortic root plane, the defects
will be visualized below the aortic and pulmonary trunks,
which take origin at the same level and are in a side-byside relationship. The conjoint arterial valves roof the
defect, whose inferior margin is formed by the crest of the
trabecular septum.

Fig. 72.45: Two-dimensional echocardiography in apical fourchamber view showing the large inlet ventricular septal defect
(VSD) getting partially restricted by septal leaflet of tricuspid valve
(arrow).

1595

Inlet Ventricular Septal Defect

(Figs 72.45 and 72.46)
The features of the inlet VSD are essentially the same as
that of the muscular inlet VSD (described above) except for
the absence of the muscular par below the inlet valves and
there is continuity of both the inlet AV valves established
by the presence of the VSD; therefore, off setting of the AV
valve may not be present.
Size of VSD: The size of any defect is important to
comment but in practice, the judgment of size of defect
is generally made on hemodynamic grounds (degree of
left-to-right shunt, presence of volume overload, and
PA pressure). According to some authors, a VSD size is
defined in relation to aortic root size, a defect is defined
as a small VSD if it is less than one third of aortic root
diameter, one third to two thirds of aortic root diameter
is considered as a moderate-sized defect, and defects
that are more than two thirds of the aortic root size are
defined as large VSDs. With isolated defects, when there
is equalization of pressure between two ventricles in the
absence of pulmonary stenosis, they are then termed
large or nonrestrictive defects. Since right and LVs do not
contract exactly simultaneously, there is always some
inequality in the ventricular pressures. A restrictive defect
is one in which RV and PA pressures are lower than LV
with a pressure gradient of > 60 mm Hg (VSD peak velocity

Figs 72.46A and B: Two-dimensional echocardiography. (A) Subcostal coronal view with anterior tilt showing the perimembranous
ventricular septal defect (VSD); (B) Parasternal long-axis view with posterior tilt toward the tricuspid valve and color compare showing
the perimembranous VSD.

1596

Section 6: Congenital Heart Disease

> 4 m/s). Moderately restrictive VSDs have a pressure

difference of 25 to 60 mm Hg (VSD peak velocity 2.54 m/s).
In all patients, the aortic valve should be carefully profiled
in relation to VSD, to rule out any prolapse of aortic cusps
through the VSD making it appear artifactually small
(Fig. 72.47). These patients will need surgery if one third or
more of the aortic cusp is prolapsing through the VSD, or if
it is associated with aortic regurgitation. There may be large
malaligned VSDs (Figs 72.48A to C) that are associated with
anterior or posterior malalignment of the outlet septum.
These are generally very large and associated with either

Fig. 72.47: Two-dimensional echocardiography with parasternal

long-axis view showing the doubly committed ventricular septal
defect (VSD) getting partially restricted by the prolapse of the right
coronary cusp (arrow). (LA: Left atrium; LV: Left ventricle; RV:
Right ventricle).

pulmonary stenosis, in case of anterior malalignment

[as in tetralogy of Fallot (TOF)] or may be associated with
posterior malalignment leading to subaortic obstruction.
The latter may be associated with other left ventricular
outflow tract (LVOT) obstructive lesions such as subaortic
membrane, bicuspid aortic valve, or even CoA.

Doppler Evaluation of
Ventricular Septal Defect
Color Doppler
Use of color Doppler has proved a valuable addition to the
diagnosis of VSDs in several ways.
The location of VSDs especially smaller ones and
multiple muscular ones can easily be determined
using Doppler color flow mapping technique.
Size of VSD.
Determination of shunt direction across the VSD.
Color flow mapping is also useful for proper alignment
of jet flow for Doppler interrogation.
Color flow mapping has a major role in rapid detection
and localization of VSDs (Fig. 72.49).2528 Small ventricular
septal defects, especially muscular, can be missed by
2D echocardiography. Color flow mapping is sensitive
in detecting small and multiple VSDs. The sensitivity of
color flow mapping is more with restrictive defects than
nonrestrictive ones, probably because of early detection of
a turbulent jet with a restrictive VSD.
Color flow mapping has been used to define the
direction of shunt across a VSD whether it is left-to-right

Figs 72.48A to C: Two-dimensional echocardiography. (A) Parasternal long-axis view showing perimembranous ventricular septal defect
(VSD) with anterior malalignment of the septum (arrow); (B) Subcostal coronal view with anterior tilt showing the anterior malalignment
of the septum (arrow); (C) Parasternal long-axis view showing perimembranous VSD with posterior malalignment of the septum (arrow).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1597

Continuous and Pulsed Wave

Doppler Examination2528
Pulsed and continuous wave Doppler examination is used
to assess the direction of shunt across a VSD, pressure
gradient across it (difference of LVRV systolic pressure),
RV pressure (by VSD gradient and peak gradient of
tricuspid regurgitation jet), and diastolic function of both
ventricles.

Direction of Shunt (Fig. 72.49)

Fig. 72.49: A 25-year-old man with Eisenmenger syndrome. Twodimensional echocardiography with parasternal long-axis view
showing doubly committed ventricular septal defect with right-toleft shunt. (LV: Left ventricle; RV: Right ventricle).

or right-to-left, and whether it is laminar (high RV systolic

pressure) or turbulent (low RV systolic pressure). With
isolated restrictive VSD, the shunt is seen as a turbulent
left-to-right jet during systole. With small or moderate size
defects, because the LV diastolic pressure is higher than RV
diastolic pressure, left-to-right shunt may persist during
diastole also. With a nonrestrictive VSD with low PVR,
left-to-right shunt occurs during systole with a small rightto-left shunt occurring during the isovolumic relaxation
period. If in addition there is RV volume overload
(associated significant tricuspid regurgitation, ASD, or
anomalous pulmonary venous connection), right-to-left
shunt occurs throughout diastole. In a child with isolated
nonrestrictive VSD with high PVR, direction of flow can
be bidirectional or dominantly right to left depending
upon the severity of pulmonary vascular obstructive
disease. With associated pulmonary stenosis, there may be
isolated right-to-left shunt depending upon the severity of
pulmonary stenosis. With severe RV outflow obstruction,
a turbulent jet of right-to-left shunt may be seen in a small
VSD due to suprasystemic RV systolic pressure.
With the use of color flow mapping with careful
interrogation, one should also identify if there is any LV to
right atrial shunt as the high velocity of LV to right atrial
jet can be misinterpreted as elevated RV pressure. This can
happen particularly with VSDs, which are decreasing in
size because of ingrowth of tissue from the septal leaflet of
tricuspid valve.

With isolated uncomplicated nonrestrictive VSD, pressure

between the two ventricles is similar. In patients with
low PVR, dominant shunt occurs from left to right during
systole, and with increase in LV, end-diastolic pressure
left-to-right shunt will persist during diastole also. A
typical M-shaped flow pattern is seen in patients
with nonrestrictive VSD, explanation for which is: as LV
contraction starts early and lasts longer than RV, so with
onset of systole flow occur from left to right, with decrease
in degree of shunt during midsystole as pressure between
two ventricles equalize, and in later part of systole as RV
relaxes, left-to-right shunt dominates. With restrictive
VSD, left-to-right shunting occurs throughout systole. In
some small muscular VSDs, left-to-right shunt occurs only
during the early phase of systole, presumably because
of closure of the defect in midsystole with ventricular
contraction. Bidirectional or right-to-left shunting can
occur with both restrictive and nonrestrictive VSDs as
described earlier.

Pressure Gradient across

Ventricular Septal Defect
While taking continuous wave Doppler across a VSD,
the cursor should be well aligned with the defect jet on
color flow mapping. The velocity of the VSD shunt can
be determined using the Bernoullis equation, p = 4V2,
where p is the pressure difference and V is the maximum
recorded velocity. This gives the difference between the
left and RV systolic pressures. The left ventricular systolic
pressure is derived from the systolic BP (provided there is
no left ventricular out flow obstruction), which should be
recorded at the time of Doppler study using appropriatesized BP cuffs.
Right ventricular pressure = Systolic blood pressureVSD
jet peak gradient (4V2)

1598

Section 6: Congenital Heart Disease

Fig. 72.50: M-mode echocardiography in a case of ventricular

septal defect showing a dilated left ventricle (LV) in a 2-year-old
child.

This equation has been found to have good correlation

with cardiac catheterization-derived RV systolic pressure.
However, sometimes the jet velocity may not reflect the
interventricular pressure gradient accurately because
proper alignment of the Doppler beam with the jet is
not possible, and that if the defect has some length to
it, the viscous frictional forces make the application of
the modified Bernoullis equation inappropriate. Also,
because the dP/dt of the RV is lower than that of the LV, a
falsely high jet velocity can be recorded in some cases with
an anatomical large VSD. Determining the RV pressure
from tricuspid insufficiency jet velocity (which may be
found in some cases) is more accurate in such instances.

M-mode Echocardiography (Fig. 72.50)

M-mode echocardiography is used to assess left atrial and
left ventricular size, to provide an estimation of the degree
of shunt across the VSD and to evaluate RV size and wall
thickness, which will reflect elevated RV systolic pressure.
For direction of shunt, color M-mode is rarely used in daily
practice but depicts best the direction of shunting during
the various phases of a cardiac cycle.

Interrogation of the Atrioventricular and

Semilunar Valves for Regurgitation and
Stenosis (Fig. 72.51)
Tricuspid regurgitation velocity should always be obtained
to predict the RV systolic pressure and hence indirectly the

Fig. 72.51: Two-dimensional echocardiography in apical fourchamber view with color compare showing the well-opened mitral
valve (arrow) and turbulence across mitral valve on color flow
mapping, because of increased flow across the mitral valve in
a patient with nonrestricted perimembranous ventricular septal
defect. (LA: Left atrium; LV: Left ventricle).

PA pressure if there is no pulmonary stenosis. Presence

of pulmonary stenosis and aortic regurgitation should
be evaluated. The velocities of flow across both AV valves
and semilunar valves should be measured to rule out any
associated abnormality.

Pulmonary Blood Flow to Systemic

Blood Flow Ratio
With regards to quantifying pulmonary and systemic
shunt flow using Doppler echocardiography, several
methods currently exist, although none is widely used due
to variable results.

Assessment of Suitability for

Device Closure
Percutaneous closure for midmuscular VSDs and some
perimembranous VSDs can be done. While assessing
the child for device closure, the defect should be at least
5 mm away from AV valves and semilunar valves and other
defects requiring cardiopulmonary bypass should not
be present. The softer variety of Amptazer duct occluder
II (ADO II) series can be used to close defects in the
perimembranous region (particularly a defect with an
aneurysm of the septal leaflet of the tricuspid valve) and
muscular VSDs.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Utility of Transesophageal
Echocardiography
TEE has helped in better visualization of VSDs when the
transthoracic window is poor, especially in adolescents
and adults. Straddling and overriding of the AV defect can
be better detected by TEE. TEE is extremely useful during
percutaneous closure of these defects.

PATENT DUCTUS ARTERIOSUS

Anatomy
The ductus arteriosus is a remnant portion of the sixth
aortic arch that connects the left PA with the descending
portion of the aortic arch. The PA end of the PDA is
usually immediately to the left of the PA bifurcation.
The aortic connection is just distal to the origin of the
left subclavian artery. The pressure differences across
the PDA is estimated by the Doppler velocity, which
will predict the pulmonary arterial pressures, systolic
as well as diastolic, by its difference with the BP of the
child. The flow across the PDA is estimated by the size
of LA and LV. Spontaneous closure of the PDA usually
begins at the pulmonary end within 24 hours of birth.
Persistence of this fetal structure beyond 10 days of life in
a term baby is considered abnormal. The ductus is funnelshaped in configuration in approximately two thirds
of patients.27

Echocardiography1,2,2931
Objectives (Table 72.6)

Demonstrate the presence of a duct.

Detailed definition of ductus.
Size of the duct.
Type of duct.
The hemodynamic significance of ductus.
Direction of shunt.

1599

Pulmonary arterial pressure.

Quantification of shunt.
Associated defects.

Echocardiographic Views
The following echocardiographic views profile accurately
the morphology of the duct.
Ductal view (Fig. 72.52): The transducer is placed in the
high parasternal window just beneath the left clavicle.
After obtaining a short-axis cut of the great vessels
visualizing the PA bifurcation, the transducer is rotated
anticlockwise in gradual motion. At one point, the right PA
goes away from the view and the duct with the adjacent
descending aorta opens. This view in neonates and infants
also visualizes the origin of the left subclavian artery. In
patients with associated coarctation, the posterior shelf
and coarctation can also be well visualized in this view.
Suprasternal view: There are three views to visualize the
duct from the suprasternal view.
(1) Suprasternal long-axis viewThis is the best view for
visualizing the vertical duct arising from the undersurface
of the transverse arch in patients with pulmonary atresia.
The origin of such ducti is well seen, but the insertion point
at the PA requires further anterior tilt. This is because of
the tortuous nature of such ducti.
In patients with discordant ventriculoarterial
connection (e.g. transposition of the great vessels), the
duct can be visualized well in its entire length in this view.
(2) Suprasternal short-axis viewThis is the classical
short-axis arch view and can visualize those ducti which
arise from the base of the left subclavian artery and
descend straight down to insert into the left PA. If the aortic
arch is right-sided and the patient has pulmonary stenosis
physiology, the entire length of the duct can be seen in this
view because unlike in patients with a vertical duct, it does
not follow a tortuous course.

Table 72.6: Stepwise Evaluation for a Patent Ductus Arteriosus

Visualize the ductus ostium and aortic isthmus from the parasternal short-axis, high parasternal short-axis, and suprasternal views
Determine the direction of shunt by color flow mapping and Doppler
Take the peak velocity of the patent ductus arteriosus (PDA) signal, which will give the pressure difference between the aorta and
pulmonary artery, and obtain the aortic, right ventricular outflow tract (RVOT), and pulmonary artery (PA) velocities
Perform measurements of the left ventricle and left atrium as these will reflect the volume of the left-to-right shunt
Specifically look for associated defects like coarctation of aorta (suprasternal view), subaortic membrane, bicuspid aortic valve,
aortic interruption, and aortopulmonary window (communication between the ascending aorta and pulmonary artery)

1600

Section 6: Congenital Heart Disease

Fig. 72.52: Two-dimensional echocardiography in high parasternal

view (ductal view) with color flow mapping showing patent ductus
arteriosus (PDA; arrow) with left-to-right shunt. (Ao: Aorta; LPA:
Left pulmonary artery; PDA: Patent ductus arteriosus).

The length of the duct needs to be measured to

determine the choice of coil/device/stent.
This view is particularly important for Amplatzer duct
occluder II (ADO II) devices as it may give a fair estimate of
the waist of the duct to decide for the device size. It is again
best determined by the modified ductal view.
In patients (older child, adolescent, and adults) where
it is not possible to evaluate detailed anatomy of the duct
because of inadequate windows, the size of the duct can be
determined by the narrowest width of the color flow across
the duct. This, however, always overestimates the ductal
size and gives only a rough estimate. In all cases where
nonsurgical intervention is planned, these measurements
performed by echocardiography will need to be confirmed
by the gold standard, that is, angiography.

Duct Morphology
Usual Ductus

(3) Modified suprasternal long-axis view (Ductal view)

This is a less well-described view to visualize the usual
duct. It has the advantage of visualizing the duct in its entire
length and most closely mimics the lateral angiogram
performed during cardiac catheterization. From the usual
suprasternal long-axis view, the transducer is rotated
anticlockwise. A slight anterior tilt then shows the duct
from its ampullary part to its insertion and accurate
measurements of the ductus size can be made.

Characteristics of the Ductus

Size of Duct
Because of frequent interventions being performed on the
duct (closure/stenting), it has become important to make
various measurements of the duct by echocardiography.
These include the following.

Size of the Narrowest Part of the Duct

This should be done at the narrowest point (as this site
would be hemodynamically most restrictive). In the
majority of cases, this would be at the site of PA insertion.
In the usual duct, this can be accurately measured in the
ductal view or the modified arch view.

Size of the Ampulla of Duct

To the interventionist, the size of the ampulla is important
to determine the possibilities of coil placement. The ampulla can be best measured in the modified ductal view.

The usual ductus arises from the descending aorta just

below the origin of the third branch of the aortic arch (left
subclavian artery with left aortic arch) and inserts into
the PA immediately to the left of PA bifurcation. This type
of ductus is best defined from the ductal and modified
suprasternal views. It narrows at the PA end and is of
funnel shape, has a straighter course, and the Doppler
signals can be very well aligned from the ductal view. For
catheter interventions like ductal coiling/device closure
or ductal stenting, these ducti are easy to cannulate by the
femoral artery route. Other uncommon configurations of
ductus include the following:
Short duct with narrow aortic end
Tubular connection with no narrowing
Tubular connection with multiple narrowings
Calcified PDA
Aneurysm of the aortic end of the PDA.
Aneurysm of ductus arteriosus at the aortic end is a
rare complication in adult patients with ductus arteriosus
and can be profiled from the ductal view.

Vertical Duct
A vertical duct arises from the undersurface of the aortic
arch, has a tortuous course, and is commonly seen in
patients with VSD and pulmonary atresia because of
in utero flow from the aorta to the pulmonary arteries.
The best view to define a vertical duct is suprasternal
long-axis view. This view shows the duct arising from the
undersurface of arch and having a S curve. Because

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

of the double curve, it is not possible to visualize the

aortic and pulmonary ends in the same view. Anterior
angulation of the transducer will show the PA insertion
of the duct. These ducti are difficult to cannulate from the
femoral artery route and may have to be accessed from the
ascending aorta or from the upper limb arteries.

1601

appears as predominantly red flow with minimal aliasing.

In patients with severe pulmonary arterial hypertension,
a bidirectional shunt is visualized on CFI. With suprasystemic PA pressure as in duct-dependent systemic blood
flow, a turbulent high velocity right-to-left flow in systole
and diastole in the descending aorta is observed. This can
mimic coarctation of the aorta.

Subclavian Origin
Subclavian origin of ductus occurs with duct-dependent
pulmonary blood flow with right aortic arch. The
suprasternal view defines best this ductus. It has a
straighter course; hence the Doppler alignment is good.
For catheter intervention, it is easy to cannulate this type
of ductus arteriosus from the femoral arterial route.

Hemodynamic Significance
Hemodynamic significance of ductus arteriosus can be
assessed by evidence of volume overload of LA and LV,
direction of shunt, and pulmonary arterial pressure.

Chamber Dimensions
Left atrial enlargement signifies increased pulmonary
venous return because of left-to-right ductal shunting. The
ratio of the LA to aorta (Ao) is measured at the level of the
aortic valve (the LA: Ao ratio) by M-mode echocardiography
in PLAX view. The aortic root does not enlarge significantly
with even an extremely large PDA. In general, LA: Ao ratio
> 1.3:1 indicates a significant shunt. The LV will enlarge
as cardiac output increases with increased pulmonary
venous return. The best method to determine the presence
of volume overload of the LV is M-mode measurement of
left ventricular diastolic dimension and comparing it with
normal values for the patients age and weight.

Direction of Shunt and Pulmonary

Arterial Pressure3133
Color Doppler Imaging of Duct
Color flow mapping increases the sensitivity of detection
of a ductus. This includes a tiny duct, which may not be
seen on 2D imaging and adolescent or adult patients
where absence of good windows (especially ductal view)
prevents visualization of the duct on 2D imaging. On color
flow mapping, a small duct with normal PA pressure is
displayed as a mosaic flow from descending aorta to PA.
With a large duct, and low PVR, the duct jet generally

Continuous Wave Doppler Examination of

Ductus Arteriosus
With the use of continuous wave Doppler, the direction of
shunt in relation to cardiac cycle and pulmonary arterial
pressure (systolic BP minus peak pressure gradient
across duct = systolic pulmonary arterial pressure) can be
assessed. Systemic diastolic pressure minus the pressure
derived from the diastolic velocity of PDA signal gives the
diastolic pressure in the PA. With an isolated left-to-right
shunt, and a small to moderate-sized duct normal or mildly
elevated PA pressures are seen. Doppler examination of
such a duct shows continuous flow toward the transducer
with the peak in late systole. A large duct with pulmonary
arterial hypertension will show bidirectional shunting
on Doppler imaging of the duct, right to left in systole
and left to right in diastole. With increasing PVR and no
step-up in oxygen saturation in the PA, the peak of rightto-left shunt appears early in systole. With further rise
in PVR, right-to-left shunt begins in systole extending to
diastole. With duct-dependent systemic circulation and
severe pulmonary arterial hypertension, only right-to-left
shunting across the duct is visualized.

Evidence of Aortic Runoff

(Figs 72.53A and B)
Presence of aortic run off in a patient with a ductus is
indicative of low pulmonary diastolic pressure and blood
flow from aorta to PA occurs in diastole, which can be
detected by M-mode color flow mapping.
Thus, color flow mapping shows flow reversal in the
descending aorta in diastole up to the level of the duct. On
continuous wave Doppler examination of the descending
aorta from the suprasternal long-axis view below the
ductus, forward flow signals in systole (below the baseline)
and reverse signals in diastole (above the baseline) are
noted. The reverse signals indicate flow from descending
aorta to PA. With the Doppler sample volume placed above
the level of the duct, flow will be in a forward direction in
both systole and diastole.

1602

Section 6: Congenital Heart Disease

Figs 72.53A and B: (A) Two-dimensional echocardiography in suprasternal view showing diastolic flow reversal in the arch of aorta; (B)
Same view with cursor across the flow on M-mode showing pan-diastolic flow reversal. (D. Ao: Descending aorta; TA: Transverse arch).

Limitations of Echocardiographic
Imaging of the Duct
There are several limitations of profilation of a duct by 2D
imaging.
Limited acoustic windows: The duct is profiled in the
direction of lateral resolution of the transducer, hence
it is difficult to visualize with certainty a very small
duct in small babies. A high frequency probe with an
excellent lateral resolution is helpful.
If the duct is long and tortuous, it may be difficult to
profile the whole length of the duct.
Poor acoustic windows in adolescent and adult
patients, deformity, hyperinflated lungs, very obese
children.

Limitations in Hemodynamics
The long length of a PDA results in underestimation of
pressure gradients across it.

AORTOPULMONARY WINDOW
(FIGS 72.54A AND B)
Aortopulmonary window (APW) or aortopulmonary septal
defect accounts for 0.2 to 0.6% of patients with congenital
heart defects.34 Nearly half of all patients have associated
cardiac lesions, including aortic origin of the right PA,3539

type A interruption of the aortic arch,36,3941 TOF,3942 and

anomalous origin of the right or LCA from the PA and right
aortic arch.39,43,44 More rarely, it is associated with VSD,4446
pulmonary39 or aortic atresia, d-transposition,47 and
tricuspid atresia.48

Types
APW is classified into three types:37
Type I: Defect between semilunar valves and PA bifurcation.
Type II: Distal type defect involving origin of right PA.
Type III: Large defect involving both type I and type II.
Echocardiography: Objectives are:
Diagnosis
Type of APW
Associated heart defects
Operability
2D echocardiography usually can accurately diagnose
the aortopulmonary septal defect. Views most useful
for profiling an APW are subcostal coronal view, fivechamber view, PSAX plane at the level of great vessels,
and the suprasternal views. In all these views, the wall
separating the aorta and PA is aligned in the direction of
lateral resolution, so great care is needed to differentiate
a true defect from an artifactual dropout. A T artifact at
the edges of the defect will distinguish it from a normal
dropout and CFI will confirm the defect.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1603

Figs 72.54A and B: Two-dimensional echocardiography with color comparison in parasternal short-axis view showing a large aortopulmonary window (arrow). (Ao: Aorta; PA: Pulmonary artery).

Figs 72.55A and B: Two-dimensional echocardiography with color mapping showing the Gerbode defect (arrow) with left-to-right shunt.
(Ao: Aorta; RA: Right artium; LV: Left ventricle).

Color Flow Mapping

Color flow mapping is used to demonstrate flow through
the defect. With large defects, which usually is the case,
the flow appears laminar, low velocity, and bidirectional
across the defect, and with smaller defects, a continuous
high velocity left-to-right jet is usually present. With low
PVR, evidence of aortic runoff can be detected in both
ascending and descending aorta in contrast to PDA in
which normal aortic flow is seen in ascending aorta and
the arch. The operability will depend on the degree of
left-to-right shunt as assessed on color flow mapping,
and the presence of left atrial and left ventricular
enlargement.

GERBODE DEFECT
(FIGS 72.55 AND 72.56)
In this entity, the shunt occurs from LV to RA through a
defect in the membranous ventricular septum and essentially leading to volume overload of the RA and the RV.
In these cases, RA dimensions and features of right
atrial and ventricular volume overload need to be
evaluated. The features are essentially same as ASD.
The point to remember is that the pressure difference
across the tricuspid valve taken in these circumstances
may be fallacious as one may mistake the high velocity LV
to RA jet for the tricuspid regurgitation velocity resulting in
an erroneous diagnosis of severe PA hypertension.

1604

Section 6: Congenital Heart Disease

Figs 72.56A and B: Two-dimensional echocardiography with color comparison in parasternal short-axis view showing Gerbode defect
(arrow) with left-to-right shunt (arrow). (Ao: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle).

PART 3: ATRIOVENTRICULAR SEPTAL DEFECTS

ATRIOVENTRICULAR SEPTAL DEFECTS
Atrioventricular septal defects (AVSDs) account for 4 to
5% of CHD and an estimated occurrence of 0.19 in 1,000
live births.49,50 About 40 to 45% of children with Down
syndrome have CHD, and among these, approximately
40% have an AVSD, usually the complete form.49 Complete
AVSD also occurs in patients with heterotaxy syndromes
(more common with asplenia than with polysplenia).
Common atrium has been associated with Ellisvan
Creveld Syndrome. The assessment of the AV junction is
readily achieved by 2D echocardiography and since AVSDs
are primarily an abnormality of this region, delineation of
detailed morphology is possible by this technique. Color
flow Doppler interrogation complements by demonstrating the sites of intracardiac shunting and AV regurgitation,
as well as defining any obstruction in the LVOT if present.
Pulsed and continuous wave Doppler are used to assess
PA pressure and severity of LVOT obstruction.5158
The following basic views can define these anatomical
features (Table 72.7).
Subcostal coronal view shows the common AV junction, loss of offsetting of AV valves, scooped out inlet
septum, and inferior bridging leaflet of AV valve (Fig. 72.57).
Subcostal sagittal view shows the common AV junction,
in addition to both superior and inferior bridging leaflets
and anterior unwedged position of aorta. Subcostal

long-axis view of LVOT defines the goose neck deformity

of LVOT (long LVOT with anterior position of the aorta;
Fig. 72.60). Apical four-chamber view will display the inlet
VSD with loss of offsetting (Fig. 72.59). PSAX view shows
the trileaflet left AV valve, presence of cleft; presence
of common AV junction, and abnormal position of the
papillary muscles in the LV. PLAX view shows discrepancy
in the left ventricular inflow and outflow measurements
and presence of LVOT obstruction (Fig. 72.58).

Types Of Atrioventricular
Septal Defect
Partial Atrioventricular
Septal Defect (Fig. 72.61A)5156,59,60
Subcostal coronal and sagittal views supplemented with
apical four-chamber, PLAX and short-axis views can
define the anatomy with great accuracy. In addition, AV
valves need to be profiled in subcostal en face view by
rotating the transducer 30 to 45 clockwise from subcostal
four-chamber view. From this view, with tilting the plane
from anterior to posterior, all five leaflets, separate AV
valve orifices, attachment of anterosuperior bridging
leaflet to anterior muscular septum, and posteroinferior
septum to inlet septum can be defined. The opening of
AV valves is seen as two separate openings created by the

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1605

Table 72.7: Common Features on Echocardiography in a Case of Atrioventricular Septal Defect

Loss of offsetting of atrioventricular valves

Deficiency of inlet portion of ventricular septum
Presence of common atrioventricular valve junction
Abnormal morphology of atrioventricular valve leaflets
Abnormal position of papillary muscles of left ventricle
Longer left ventricular outflow, and anterior unwedged position of aorta

Fig. 72.57: Two-dimensional echocardiography. Subcostal paracoronal view with anterior tilt showing the components of the common atrioventricular (AV) valve. (Ao: Aorta; IBL: Inferior bridging
leaflet; LV: Left ventricle; RV: Right ventricle; SBL: Superior bridging leaflet).

Fig. 72.58: Two-dimensional echocardiography. Parasternal

long-axis view from an infant with complete atrioventricular septal
defect showing discrepancy in left ventricular inflow (line-a) and
outflow (line-b) measurements. (Ao: Aorta; LA: Left atrium; LV: Left
ventricle; RV: Right ventricle).

Fig. 72.59: Two-dimensional echocardiography in apical fourchamber view showing large inlet ventricular septal defect (VSD;
arrow) in a case of atrioventricular septal defect. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Fig. 72.60: Subcostal apical four-chamber view with anterior tilt in

diastole showing goose neck deformity of left ventricular outflow
tract, that is, left ventricular outflow tract is elongated with anterior
unwedged position of aorta (arrow). (Ao: Aorta; LV: Left ventricle;
RV: Right ventricle).

1606

Section 6: Congenital Heart Disease

Figs 72.61A and B: Two-dimensional echocardiography. (A) Apical four-chamber view showing a large ostium primum atrial septal
defect (ASD; arrow), no ventricular septal defect (VSD), lack of offsetting of the atrioventricular (AV) valves (arrow); (B) Apical fourchamber view showing a large ostium primum ASD and a large inlet VSD. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).

presence of a bridging tongue of tissue. The characteristic

features of partial AVSD are two separate AV orifices within
the common AV junction, abnormal valve leaflets with
or without cleft, and usually presence of ASD between
lower part of atrial septum and the crest of the ventricular
septum. Leaflets are attached to the crest of ventricular
septum, so there will be loss of offsetting and usually there
is no VSD or it is restrictive, although this is not a universal
finding. Rarely, only inlet VSD and intact interatrial septum
is present when the bridging leaflets are attached to the
lower part of the interatrial septum. VSD can be profiled
from subcostal coronal and sagittal, apical four-chamber,
and PLAX with posterior tilt and short-axis views. Here the
trileaflet left AV valve guarding the left component of the
common AV junction, seen in PSAX and subcostal en face
views, will be the hallmark feature to differentiate it from
an isolated inlet muscular VSD. Rarely, a left AV valve with
three leaflets may be the only manifestation of an AVSD
with intact atrial and ventricular septum. There may be a
cleft of only left or right AV valve (more commonly of left
side). The cleft can be profiled in the subcostal en face view
and PLAX and PSAX views. In PSAX view, the cleft (zone of

apposition between superior and inferior bridging leaflets)

is seen toward the ventricular septum to differentiate this
anomaly from an isolated cleft of mitral valve, where the
cleft will be oriented toward the LVOT (Fig. 72.66). Also,
other features of AVSD such as a longer LVOT, unwedged
and anterior position of aorta as described earlier will be
present. Less common variant is common atrium (virtual
absence of atrial septum), usually found in the setting of
left or right isomerism. Associated anomaly of AV valves
such as a dual orifice AV valve with or without stenosis
and Ebsteins anomaly of right AV valve can sometimes be
present and should be looked for on 2D echocardiography
(Table 72.8).

Complete Atrioventricular Septal Defect

(Atrioventricular Septal Defect with Common
Valvular Orifice
(Fig. 72.61B)5356,6163
The typical echocardiographic features of complete
AVSD seen in subcostal coronal and sagittal, and apical

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1607

Table 72.8: While Evaluating a Patient with Atrioventricular Septal Defect, the Following Needs to be Addressed

Type of defectpartial or complete atrioventricular septal defect

Extent of atrial shunting
Extent of ventricular shunting
Presence and degree of atrioventricular valve regurgitation
Commitment of atrioventricular valves to respective ventricles, is there balanced atrioventricular connection or unbalanced
atrioventricular connection, degree of ventricular hypoplasia if present
Presence of straddling
Potential for left or right ventricular outflow obstruction
Pulmonary artery pressures
Associated lesions

four-chamber views are ostium primum ASD, common

AV valve guarding the common junction, and an inlet
interventricular communication of variable size. The VSD
can be small or large depending upon the attachment of the
bridging leaflets. As described earlier, the inferior bridging
leaflet is seen best in subcostal coronal view, and it may
be firmly attached by a midline raphae to the septum; as
a result, there will be no interventricular communication
close to crux. The superior bridging leaflet is seen in
apical four-chamber view with anterior tilt and most of
the variations in ventricular component of shunting are
seen beneath the superior bridging leaflet. The subcostal
en face view shows both superior and inferior bridging
leaflets. When the superior bridging leaflet is attached
firmly to the septal crest, there is no defect beneath it;
more commonly, this leaflet is attached to a normally
positioned medial papillary muscle and is attached by
multiple cords to the crest of the septum. There are then
multiple interventricular communications through the
intercordal spaces, and the flow can be recognized on color
flow mapping. This type of defect is called as Rastelli type
A under Rastelli classification. In the so-called Rastelli
type B, the RV medial papillary muscle is positioned in
midseptal position, the degree of bridging is greater, and
the bridging leaflet is less well attached to the ventricular
septal crest, and becomes free-floating. When the papillary
muscle is located still further in the RV, a so-called Rastelli
type C defect is produced. In this situation, almost always a
large VSD is present and is particularly frequent in Downs
syndrome.
Rarely with complete AVSD, there will be absence of
any interatrial shunt when the superior bridging leaflet is
attached to the lower end of atrial septum, and absence of
VSD when it is firmly attached to the ventricular septum as
described with a partial AVSD.

Commitment of atrioventricular valve to ventricle and

relationship of atrioventricular valve leaflets to the septal
structurebalanced or unbalanced atrioventricular
septal defect: Apical four-chamber, subcostal coronal, and
subcostal en face views are required to profile commitment
of AV valves to respective ventricles, and to look for presence
of overriding. These views allow simultaneous visualization
of all four chambers, AV valves, and atrial and ventricular
septa. If the AV junction is shared equally, then there is a
balanced AV connection. When there is overriding of AV
valve to one of the ventricles and malalignment between
atrial and ventricular septum, then the condition is termed
as unbalanced AV connection leading to hypoplasia of left
or RV depending upon the degree of overriding (Figs 72.62
and 72.63). One of the AV valves can be atretic, causing
hypoplasia of the respective ventricle. Abnormal relation
between atria and ventricles can also occur when the
common AV junction is not equally shared between both
the ventricles but is committed exclusively to one or the
other atrium. This condition is termed as double outlet
atrium or uniatrial biventricular AV connection and can
be defined in subcostal and apical four-chamber views.6466
Straddling of AV valves is also an issue that needs to
be defined. Straddling of left AV valve is profiled in PLAX
view while for right AV valve, the four-chamber view and
subcostal en face view are required to profile the chordal
attachments.
Color flow mapping is required to define presence and
direction of shunting across interatrial or interventricular
septum, presence of AV valve regurgitation or stenosis, and
presence of left or RVOT obstruction. Direction of shunt
across ASD or VSD can be profiled from views used to define
the defects. Subcostal, coronal, apical four-chamber, and
PSAX views are required to look for presence of AV valve

1608

Section 6: Congenital Heart Disease

Figs 72.62A and B: Two-dimensional echocardiography. Apical four-chamber view showing unbalanced atrioventricular canal defect
with left ventricle (LV) dominance (A) and right ventricle (RV) dominance (B). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).

regurgitation, presence of LVright atrial shunt, or RV

left atrial shunt. RVleft atrial shunt could be a cause of
cyanosis in a child with partial AV canal defect with normal
PA pressure. The quantitative assessment of valvular
stenosis is not accurate by Doppler echocardiography
when there is a large ASD. So it is important to evaluate
valve anatomy by 2D echocardiography and look especially
for dysplasia, tethering of leaflets, and valve orifice. In such
cases, valve stenosis may manifest after closure of the ASD
if not addressed at surgery.
The outflow tract of both LV and RV should be assessed,
as subvalvular obstruction of any of the outflows can occur.
Left ventricular outflow tract obstruction (Table 72.9):6268
LVOT is longer and narrower than normal in AVSD,
although in most cases there is no overt stenosis. Any
factor that causes further narrowing of LVOT causes LVOT
obstruction. Causes of LVOT obstruction are highlighted
in Table 72.9.
Right ventricular outflow tract obstruction: Pulmonary
stenosis can occur at subvalvular (malalignment of outlet

septum, infundibular hypertrophy), valvular, or supravalvular level.

Complex atrioventricular septal defect: A complex AVSD
can be defined as an AVSD morphology that precludes
two-ventricle correction.66-69 The following conditions can
be the cause of such a situation:
The most frequent is the association of AVSD with heterotaxy/isomerism. Anomalous systemic/pulmonary
venous connection and hypoplasia of ventricles frequently precludes biventricular correction.
Abnormalities of ventricular arterial connections.
AVSD can be associated with DORV, making it difficult
or impossible to route the LV to aorta as the VSD, thus
precluding a two-ventricle repair.
Right/left ventricular dominant AVSD.
Because of extreme straddling/overriding of the
common AV valves across the VSD, one of the ventricles
may be hypoplastic. This will prevent two-ventricle repair.
In most cases, quantification of hypoplasia is subjective,
based on echocardiography (Figs 72.62A and B).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1609

Table 72.9: Left Ventricular Outflow Tract Obstruction in Atrioventricular Septal Defect

Tight adherence of superior bridging leaflet to septal crest causing left ventricular outflow tract to be longer and narrower. Left
ventricular outflow tract obstruction is more common in partial atrioventricular septal defect and with primum ASD
Discrete subaortic membrane
Ventricular septal hypertrophy
Abnormal chordal attachment of superior bridging leaflet
Prominent anterolateral muscle bundle
Left ventricular outflow tract can be profiled from subcostal coronal view with anterior tilt, subcostal sagittal view, and parasternal long-axis view. Color flow mapping shows turbulence beginning in subaortic area
Careful Doppler interrogation shows site of obstruction and severity of obstruction of left ventricle outflow tract

Figs 72.63A to D: (A) Subcostal en face view profiling the common atrioventricular (AV) valve completely; (B) Tracing the common
AV valve orifice during end diastole, averaged over three cardiac cycles (area B); (C) This circumference is then divided by a line drawn
over the interventricular septum from the tip of the infundibular septum to the crest of the muscular septum, thus dividing the AV valve
into left and right components (D) Take the area of the left component. For a balanced atrioventricular septal defect, AVVI (A area/B
area in figure) should be > 0.67.

1610

Section 6: Congenital Heart Disease

The area of the common AV valve and left component can

be measured and the ratio calculated as shown in Figs
72.63 A to D to quantitate the hypoplasia.
Associated defects: TOF and DORV with malposed great
vessels are frequently associated with complete AVSD,
especially in the setting of isomerism.69 With unbalanced
AV connection leading to hypoplastic LV, aortic arch should
be carefully profiled in the suprasternal long-axis view to
rule out arch anomalies like CoA and arch interruption.
With hypoplastic RV, pulmonary stenosis and pulmonary
atresia may be associated and need to be profiled carefully.

Hemodynamic Assessment of
Atrioventricular Septal Defect
Without Pulmonary Stenosis
Partial atrioventricular septal defect: These defects
behave like an ASD. In the absence of significant AV
valve regurgitation and normal PA pressure, the lesion
is well tolerated and patients may present late like fossa
ovalis ASDs. Significant AV valve regurgitation, however,
may cause early congestive heart failure. Accurate
assessment of PA pressure and AV valve regurgitation is
critical in decision-making for timing of surgery. Doppler
assessment of PA pressure is usually performed by
assessing tricuspid regurgitation velocity. Care should be
taken in not confusing LVright atrial shunt for tricuspid
regurgitation as the former (LVright atrial shunt) will
invariably produce high velocity signals which will not be
reflecting pulmonary arterial pressures.

Complete atrioventricular septal defect: This is associated with large VSD and pulmonary arterial hypertension. Thus, congestive heart failure develops in
the first few months of life. Also, rapid progression
(6 months of life) of pulmonary vascular disease occurs
in this condition. Thus, there is an urgent need to correct
these lesions early in life. If correction is performed at
the appropriate age, then echocardiography alone is
enough for assessment of this lesion as the morphology
is well delineated by this technique and there is no need
for invasive determination of PA pressure and vascular
resistance. Late presentation, however, may need more
detailed evaluation with cardiac catheterization.

With Pulmonary Stenosis

Patients with pulmonary stenosis present as TOF.
However, the morphology is much more complicated. The
VSD, which is predominantly of the inlet type, also extends
into the outlet septum. Anterior malalignment of the
outlet septum causes RV outflow obstruction and other
morphological abnormalities associated with TOF.

Mechanism of Atrioventricular
Valve Regurgitation
In the majority of cases, AV valve regurgitation occurs
through the cleft in the left AV valve. This is well-appreciated in the PSAX views and subcostal paracoronal
(en face) view. Regurgitation can also occur through the
commissures of the left AV valve or through the right
AV valve.

PART 4: CONGENITAL LEFT VENTRICULAR AND RIGHT

VENTRICULAR INFLOW ANOMALIES
CONGENITAL ANOMALIES OF MITRAL
VALVE (TABLE 72.10)70
Mitral stenosis or mitral regurgitation forms the predominant manifestation of mitral valve anomalies. Other associated cardiac anomalies are present in 90% of them.
Echocardiography can assess the degree of severity of the
lesion and define the varied anatomy preoperatively for
planning the surgery. It is useful intraoperatively to guide
the surgery and postoperatively to assess the outcome.7074

Echocardiographic Views to Define

Mitral Valve Lesions
Mitral valve is best visualized in parasternal long-axis,
apical four-chamber and two-chamber views, and in PSAX
view. Addition of color Doppler further helps to diagnose
the abnormalities. PLAX view shows the motion of mitral
valve leaflets and any evidence of doming or prolapse can
be noted. The chordal length, chordal thickening, and
chordal insertion are also well seen in this view. Additional

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1611

Table 72.10: Common Mitral Valve Abnormalities

Supramitral membrane or ring

Hypoplasia of the mitral apparatus
Dysplasia of the mitral valve
Parachute mitral valve
Cleft mitral leaflet (anterior or posterior)
Abnormal mitral arcade
Double orifice mitral valve
Accessory mitral valve tissue/orifice
Ebsteins anomaly of the mitral valve
Mitral valve prolapse
Mitral regurgitation secondary to other causes, some of which
are congenital

Infective
Myocarditis

Kawasaki disease

Infective endocarditis

Rheumatic heart disease

Papillary muscle dysfunction

Mitral regurgitation and mitral stenosis

Ischemia
Anomalous origin of the left coronary artery from the
pulmonary artery
Cardiomyopathy
Dilated cardiomyopathy
Hypertrophic cardiomyopathy

Storage disease/infiltration

Hurler disease

Amyloidosis

Connective tissue disease

Marfan's syndrome

Fig. 72.64: Two-dimensional echocardiography in subcostal

short-axis view with color flow mapping from a child with partial
atrioventricular septal defect showing left atrioventricular valve
regurgitation through the cleft (arrow). (LV: Left ventricle; RV:
Right ventricle).

with stenosis, to see the level of stenosis. Doppler is also

useful in estimating gradients and valve area. Gradients
by Doppler may be underestimated due to associated
interatrial defect or due to poor alignment secondary to
multiple levels of obstruction to left ventricular inflow.
Pressure half-time method is often not reliable in this
setting. Planimetry of the mitral valve orifice in PSAX view
may be the best method for assessment of mitral valve
area.
The normal mitral valve area is 2.4 to 3.6 cm2/m2. In
mild mitral stenosis, the valve area is reduced to 1.2 to 2.4
cm2/m2; in moderate mitral stenosis, mitral valve area is
0.6 to 1.2 cm2/m2; and in severe mitral stenosis, the valve
area is < 0.6 cm2/m2.

EhlersDanlos syndrome, etc.

abnormalities of LVOT like subaortic membrane, tubular

narrowing, and so on can be assessed. Apical four-chamber
view shows ventricular inflow region and any obstructive
membrane or ring in the atrium, like a supramitral ring.
The valve annuli can be measured. Apical four-chamber
view is good for showing dilatation of atria, which may
occur secondary to atrioventricular valve stenosis
and/or regurgitation. PSAX view shows orientation of
commissures, chordae, and papillary muscles including
the number of papillary muscles. Cleft mitral valve
(Fig. 72.64) and double orifice mitral valve are also best
diagnosed in this view. Addition of color flow mapping is
necessary for quantifying the regurgitation and in cases

The Echocardiographic Evaluation of

Individual Mitral Lesions
Supramitral Ring or Membrane75,76
(Figs 72.68 and 72.69)
It is a circumferential ridge of fibrous tissue on the atrial
surface of mitral valve attached to the base of the atrial
surface of mitral leaflets. True incidence, although not
well described, varies from 9 to 20% of reported cases of
congenital mitral stenosis. In approximately 4% of cases, it
is an isolated anomaly. The opening of the central orifice
of the shelf-like projection decides the severity of mitral
obstruction.

1612

Section 6: Congenital Heart Disease

Echocardiographically, supramitral ring (Figs 72.65A

and B) is well seen in parasternal long- and short-axis
views, and apical and subcostal four-chamber views. There
are two variants of supramitral ring:
Small separation between mitral annulus and the ring
during diastolesupra-annular variant.
Membrane firmly adhered to the mitral leaflets
annular variant.

Figs 72.65A and B: (A) Two-dimensional echocardiography in

apical four-chamber view showing annular type of supramitral
membrane; (B) Color flow mapping of the same showing turbulence starting from the supravalvular membrane. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Fig. 72.66: Two-dimensional (2D) echocardiography from an infant with cor triatriatum. Zoomed up apical four-chamber view on
2D echocardiography, showing a shelf in left atrium stretching
from atrial septum on the right side to lateral wall of left atrium on
the left with a narrow communication (arrow). (LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).

The second variety is difficult to image in real time

and is best visualized by slow frame-by-frame playback
examination of a held image. Majority of supramitral
rings are nonobstructive. The obstruction is produced
when their central lumen is small or when associated
with a small mitral annulus, fusion of valve commissures,
parachute mitral valve, or accessory mitral tissue.
Color Doppler examination shows the site of actual
obstruction by turbulent flow and spectral Doppler
interrogation will provide the gradient across the mitral
valve. Supramitral ring needs to be differentiated from
cor triatriatum sinistrum (Figs 72.66 and 72.67). In cor
triatriatum sinistrum, LA is divided into two chambers
superior and inferior by an abnormal diaphragm. The
superior chamber receives pulmonary veins and inferior
chamber communicates with left atrial appendage and
mitral inflow. With supramitral ring, the stenosing ring
is located much closer to mitral valve, lying between
the mitral valve and left atrial appendage; another
differentiating feature between the two is the movement of
the diaphragm during systole and diastole. The best views
to detect cor triatriatum are apical four-chamber, subcostal
coronal, and PLAX views. In apical four-chamber view,
the shelf is seen horizontally. The side attachment is to
atrial septum and on left side to lateral wall of LA above
the left atrial appendage. In PLAX view, the shelf stretches
superiorly to the posterior aortic root and inferiorly to
posterior left atrial wall. Color flow mapping shows that

Fig. 72.67: Two-dimensional echocardiography. Apical four-chamber view with color flow mapping showing turbulence (arrow) in
a case of cor triatriatum. (LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

with cor triatriatum, turbulence starts in mid left atrial

cavity, but with supramitral ring, mosaic jet forms at just
above the mitral valve.
Supramitral ring is known to be associated with CoA,
VSD, double-outlet RV, TOF, subaortic stenosis, bicuspid
aortic valve, valvular aortic stenosis, complete AVSD,
PDA, bicuspid pulmonary valve, ASD, abnormal tricuspid
valve, persistent LSVC draining into coronary sinus, partial
anomalous pulmonary venous connection (PAPVC),
endocardial fibroelastosis, double aortic arch, hypoplastic
LV, and coronary anomalies.

Hypoplastic Mitral Valve7784

A hypoplastic mitral valve is nearly always associated with
hypoplastic left heart syndrome (HLHS) or its variant. LA
may be small in size. Mitral valve is often dysplastic with
a small annulus, thickened leaflets, and short chordae,
attaching directly into the left ventricular wall. The papillary
muscles are poorly developed or rudimentary, with a very
small left ventricular cavity. The LVOT including aortic
arch is very small or atretic. An ASD is mostly present
and LA is decompressed via the ASD. With restrictive
ASD, left atrial pressure remains very high leading to
severe pulmonary venous and arterial hypertension.
During echocardiography in patients with hypoplastic left
heart, mitral atresia, or severe mitral stenosis, interatrial
communication should be carefully assessed for adequacy
from subcostal views.
Echocardiographic measurements suggesting the
diagnosis of HLHS include an aortic valve annulus
< 5 mm, mitral valve annulus < 9 mm, left ventricular
inflow diameter < 21 mm, and end-diastolic left ventricular
volume of < 20 mL/m2.

Dysplasia of Mitral Valve (Typical Congenital

Mitral Stenosis)77,84
This condition is characterized by thickening of leaflets
with rolled edges, fused commissures, short chordae
with reduced interchordal spaces and poorly developed
papillary muscles. The leaflets are thickened with limited
mobility and show typical doming during diastole. They
may also show nodularity on both atrial and ventricular
aspects of the valve. Echocardiography from a combination
of views, apical four-chamber, PLAX and short-axis views,
reveal the detailed anatomy of mitral valve.
Severity of the obstruction can be assessed by
cross-sectional echocardiography along with Doppler
interrogation of the gradient across the stenotic valve.

1613

Parachute Mitral Valve87,89

In this anomaly, all chordae tendineae of the mitral leaflets
are attached to a single papillary muscle. The anterolateral
papillary muscle is usually absent. Sometimes, there may
be two papillary muscles adjacent to each other, producing
a functional single papillary muscle.
Echocardiographically, papillary muscles are best
evaluated in PSAX view. Parachute mitral valve results
in mitral stenosis due to reduced interchordal spaces.
Chordae may also be short and thickened. Severity of
stenosis is best evaluated by measurement of gradients
across the mitral valve. This condition is often seen as part
of the Shones complex. Other associations include VSD,
double-outlet RV, and ASD.

Cleft Mitral Leaflet (Fig. 72.66)8993

Isolated cleft in mitral valve is rare with a reported
incidence of 15% of all congenital anomalies of the mitral
valve. This is one of the few lesions of mitral valve, which is
readily amenable to successful surgery. Generally, the cleft
is in the anterior mitral leaflet, but a cleft in the posterior
mitral leaflet has been reported. This condition should be
differentiated from cleft in mitral valve associated with
partial or complete AVSD.
The cleft extends from the free margin to the annulus
for a variable length and divides the leaflet into two
equal parts. The leaflets may be normal or may be mildly
dysplastic or thickened, producing variable degree of
mitral regurgitation. The parasternal short-axis of the cleft
is directed toward the LVOT. Cleft in mitral leaflet produces
mitral regurgitation and color flow clearly shows the
regurgitation originating from the cleft. The abnormality
is best visualized in PSAX, apical four-chamber, and
subcostal short-axis views.
Cleft in anterior mitral leaflet is very commonly
associated with AVSDs. However, it is not a true cleft but
a commissure between the anterior and the posterior
bridging leaflets. The major axis of this so-called cleft in
AVSD is directed toward the interventricular septum.

Abnormal Mitral Arcade (Hammock

Mitral Valve)8789 (Figs 72.68 and 72.69)
This is best seen on echocardiography from the atrial
side of the mitral valve in PSAX, subcostal short-axis, and
PLAX views. The valve has the shape of a funnel without
commissures with a central orifice of variable size. Chordae
are seen to cross the orifice, giving the appearance of a
hammock. Thickened papillary muscles of LV may also

1614

Section 6: Congenital Heart Disease

Fig. 72.68: Two-dimensional echocardiography in a child with a

hammock mitral valve showing thickened leaflets attached directly
to the papillary muscle without intervening chordae with left atrium
(LA) enlargement. (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).

Fig. 72.69: Two-dimensional echocardiography in a child with

hammock mitral valve. Parasternal long-axis view showing
the hammock mitral valve with mild mitral regurgitation and
turbulence in left ventricular outflow tract (LVOT). (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Figs 72.70A and B: Two-dimensional echocardiography in apical four-chamber view (A) showing double orifice mitral valve with separate subvalvular apparatus; (B) Parasternal short-axis view at the level of the mitral valve showing the two separate orifices of equal
size. (LA: Left atrium; LV: Left ventricle).

result in partial obstruction to left ventricular inflow.

When examined from the left ventricular side in apical
or subcostal four-chamber view or apical two-chamber
views, one can see either direct insertion of the leaflets
into the papillary muscles or insertion through short, thick
chordae. Bridge of fibrous tissue adherent to the inferior
aspect of anterior mitral leaflet may also be seen.
This abnormality usually results in both mitral stenosis
and mitral regurgitation. Abnormal mitral arcade may be
associated with ASD, PDA, valvular and subvalvular aortic
stenosis, and CoA.

Double Orifice Mitral Valve9496

(Figs 72.70A and B)
In this condition, there are two mitral orifices with separate
leaflets, chordae tendineae, and papillary muscles. In
85% of cases, the orifices are of unequal size with smaller
orifice situated close to the anterolateral commissure
in 41% and close to the posteromedial commissure in
44%. Less commonly, there is a bridge of fibrous tissue
between the two leaflets making two openings. The
number of papillary muscles may vary from two to four.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Three varieties of double orifice mitral valve have been

described: an incomplete bridge type, in which a small
strand of tissue connects the anterior and the posterior
leaflets at the leaflet edge level; a complete bridge type, in
which a fibrous bridge divides the atrioventricular orifice
completely into equal or unequal parts; and a hole type,
in which an additional orifice with subvalvular apparatus
is present in the posterior commissure of the mitral valve.
They could be distinguished by sweeping the transducer
in cross-sectional view from the apex toward the base of
the heart.
Variable extent of mitral stenosis and regurgitation is
often present. The type of the defect does not predict the
presence or severity of stenosis or regurgitation. Double
orifice mitral valve is commonly associated with partial or
complete AVSD, CoA, aortic stenosis, PDA, VSD, and ASD.

Accessory Mitral Orifice

This abnormality results from a circular deficiency of
mitral leaflet tissue. The size of the orifice can vary and the
border of the accessory orifice is usually devoid of chordae
tendineae. In some cases, chordae may insert into an
independent papillary muscle. Accessory mitral orifice is
best visualized in PSAX and subcostal four-chamber view
with color Doppler interrogation. Apical four-chamber
view may show the abnormality of the subvalvular
apparatus. An abnormal position and orientation of a
mitral regurgitant jet may help suspect this condition
and warrants further evaluation in different views. This
condition is sometimes associated with transposition of
great arteries, partial AVSD, and interrupted IVC.

Ebsteins Anomaly of Mitral Valve

This is a rare anomaly with very few published case
reports.97 Here, the LA is dilated and the posterior leaflet
of mitral valve, which is dysplastic, is displaced downward
with normal insertion of anterior mitral leaflet into the
ventricular septum (above the septal tricuspid leaflet). Few
case reports have shown associated thin left ventricular
wall. This abnormality is best visualized in apical, subcostal
four-chamber views and in PLAX view. The severity of
mitral regurgitation can be assessed by color and spectral
Doppler interrogation.
Ebsteins anomaly of mitral valve should not be
confused with Ebsteins anomaly of left atrioventricular
valve in association with corrected transposition, where
septal leaflet of morphological tricuspid valve is apically
displaced.

1615

The reported associations include Ebsteins anomaly of

tricuspid valve, Marfan syndrome, double-outlet RV, ASD,
PDA, CoA, hypoplasia of ascending aorta, and valvular
aortic stenosis.

Mitral Valve Prolapse98101

Mitral valve prolapse (MVP) results from myxomatous
degeneration of the mitral valve, more commonly affecting
the posterior leaflet; however, anterior or both leaflets can
be effected, which balloons into the LA during systole,
resulting in noncoaptation of mitral leaflets producing
the typical click and murmur. The mitral leaflets show
degenerative changes with elongated chordae. The
chordae may sometimes get ruptured, producing severe
mitral regurgitation.
Diagnostic criteria are as follows:
Perloff et al. set the stage for accurately diagnosing
MVP by expanding the diagnostic standards to include
clinical and echocardiographic criteria98,99
In a Framingham Heart Study, Freed et al. historically
described echocardiographic criteria for MVP as
classic versus nonclassic (see below)100
Use of the PLAX view increases the diagnostic accuracy
of MVP.101
Findings are as follows:
Classic MVPThe PLAX view shows > 2 mm superior
displacement of the mitral leaflets into the LA during
systole, with a leaflet thickness of at least 5 mm
Nonclassic MVPDisplacement is > 2 mm, with a
maximal leaflet thickness of < 5 mm
Other echocardiographic findings that should be considered as criteria are leaflet thickening, redundancy,
annular dilatation, and chordal elongation.
Izumo et al. describes the superiority of using
three-dimensional (3D) TEE (en face view) versus 2D
TEE (commissural view) in patients with severe mitral
regurgitation due to prolapse or flail mitral valve to assess
the etiology with respect to quantification of prolapse
segment and width. Based on the complex mitral valve
anatomy, 2D TEE could not detect the largest prolapse gap
and width, thus concluding 3D TEE superiority.101
MVP may be associated with connective tissue
disorders or be idiopathic. The prolapse is best visualized
in PLAX view, apical and subcostal four-chamber views,
and apical two-chamber view. Severity of the prolapse
can be graded by cross-sectional echocardiography and
severity of regurgitation can be assessed by color Doppler.
MVP is often associated with ASD secundum and rarely
with VSD.

1616

Section 6: Congenital Heart Disease

Ebsteins anomaly is characterized by apparent apical

displacement of septal and posterior tricuspid valve leaflet
insertion. The anterior leaflet is attached normally, but
is elongated and is sail-like. The displacement of septal
and posterior leaflets is caused by partial or complete
adherence of these leaflets to the underlying myocardium.
The incidence of this malformation varies from 0.03 to 0.6%
of all CHDs. The disease comprises a spectrum of severity

from a mild displacement to a severe one and produces

varying degree of low-pressure tricuspid regurgitation
and rarely stenosis of the tricuspid valve. The commissure
between the septal and posterior leaflet is the point of
maximal displacement. These two leaflets are mostly
dysplastic and of variable size. The RA dilates to a variable
extent depending on the severity of tricuspid regurgitation.
Tricuspid valve annulus is also enlarged. Because of the
displacement of the leaflets, the RV is divided into two
partsthe inlet portion of atrialized ventricle, which is
thin-walled and often aneurysmal, and the trabecular and
outlet portions, called functional RV.
Few cases of Ebsteins anomaly have an imperforate
tricuspid valve with a muscular partition between the
inlet and the trabecular portion of the RV. Presence of
an ASD is seen in 65 to 93% of cases and in majority, it is
either a stretched patent foramen ovale or a small ostium
secundum type of defect.
Apical four-chamber view shows sail-like anterior
leaflet of tricuspid valve and is the view of choice for
assessing the degree of septal leaflet displacement, and
the apical four-chamber view with posterior tilt will
profile displacement of posterior leaflet. The PLAX view
when tilted toward RV inflow also shows the abnormally
placed tricuspid valve. A septal leaflet displacement of >
8 mm/m2 has been found to be a sensitive indicator of the
diagnosis. Also, an absolute value of displacement of > 15
mm in children of < 14 years, or > 20 mm in adults helps
in echocardiographic diagnosis of Ebsteins anomaly,
discriminating it from the normal variations and position
of tricuspid valve with marked right atrial enlargement.

Table 72.11: Various Congenital Lesions of the Tricuspid Valve

Ebsteins anomaly
Tricuspid valve dysplasia
Tricuspid valve prolapse
Double orifice tricuspid valve
Parachute deformity
Congenitally unguarded tricuspid orifice
Tricuspid atresia

CONGENITAL ABNORMALITIES OF
TRICUSPID VALVE (TABLE 72.11)
Apart from Ebsteins anomaly, other congenital anomalies
of the tricuspid valve apparatus (valve annulus, valve
leaflets, chordae tendineae, and papillary muscles) are not
very common. Dysplastic valve with varied abnormalities
can occasionally be seen.

Ebsteins Anomaly of the Tricuspid

Valve102111 (Figs 72.71A and B)

Figs 72.71A and B: Two-dimensional echocardiography in a patient with Ebsteins anomaly of tricuspid valve. (A) Apical four-chamber
view with slight leftward tilt showing the apical displacement of the septal leaflet of tricuspid valve (arrow), (+) shows the normal site
of attachment of TV, enlarged right atrium, atrialized right ventricle, and reduced size of the functional right ventricle; (B) Apical fourchamber view with posterior tilt (at the plane of coronary sinus) showing displaced posterior leaflet of tricuspid valve. (ARV: Atrialized
right ventricle; CS: Coronary sinus; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Anterior tricuspid leaflet, although normally attached

at the tricuspid annulus, is rarely completely normal.
Because of its sail-like nature and abnormal attachment to
the ventricular wall and papillary muscle, it may cause RV
inflow obstruction. The anterior leaflet may be joined to
the posterior or septal leaflet as a hammock-like structure
producing functional tricuspid stenosis.
Ebsteins anomaly may be associated with significant
RVOT dilatation and RV dysfunction. It may also be
associated with left ventricular dysfunction and variable
degree for left ventricular fibrosis and hypertrophy.
Adequacy of functional RV determines the treatment
strategy of future single or two-ventricle repair. In a subset
of patients with Ebsteins anomaly with small functional
RV and significant desaturation, a Glenn or Fontan type of
surgery is indicated.
The usual Ebsteins anomaly is always associated with
situs solitus and atrioventricular and ventriculoarterial
concordance. However, Ebsteins anomaly may involve
the left atrioventricular valve in atrioventricular and
ventriculoarterial discordance. Here, the nature of the
displacement and formation of the septal and posterior
leaflets are similar, but the anterior leaflet is smaller and
not elongated or sail-like. Also, the left atrioventricular
valve regurgitation is of high pressure in comparison to
low-pressure tricuspid regurgitation in Ebsteins anomaly
of usual type.
Associated defects in Ebsteins anomaly are rare and
include VSD, PDA, partial AVSD, pulmonary stenosis,
pulmonary atresia, and MVP.
In order to grade the severity of the Ebstein deformity
using echocardiography, Celermajer et al.107 described

1617

the following ratio: RA + aRV/RV + LV + LA (RV = area of

the RV, RA = area of the RA, aRV = atrialized portion of the
RV at end diastole, LA = area of the LA, LV = area of the
LV); all the measurements are made in end diastole. These
are graded as Grade I (ratio < 0.5), Grade II (ratio from
0.50.99), Grade III (ratio from 1.01.49), and Grade IV
(ratio > 1.5), with Grade I having the best prognosis while
Grade IV, the worst prognosis.

Tricuspid Valve Prolapse111

As an isolated anomaly, tricuspid valve prolapse is rare. It
is more commonly seen in association with MVP. There is
similar myxomatous degeneration of the tricuspid valve
leaflets with thinned leaflets and elongated chordae. PLAX
view permits evaluation of septal and posterior leaflets.
The anterior and septal leaflets are best visualized from the
PSAX and from apical and subcostal four-chamber views.
In approximately 40 to 48% of cases, there is associated
MVP.

Congenitally Unguarded
Tricuspid Orifice1,2,112
Here, the orifice between the RA and the RV is normal,
but there is no tricuspid valve apparatus. There is either
complete absence of valve or only remnants of valvular
tissue are present. The close differential includes dysplastic
tricuspid valve (Figs 72.72A and B). Usually, the RA is
dilated and the RV is hypoplastic.
This anomaly is best visualized in apical and subcostal
four-chamber views as well as in long-axis parasternal
inflow view. Severe low-pressure tricuspid regurgitation
is invariably present. Associations include pulmonary

Figs 72.72A and B: Two-dimensional transthoracic echocardiography. Apical four-chamber view with color compare in a case of
severely dysplastic tricuspid valve showing markedly enlarged right atrium and severe tricuspid regurgitation. (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).

1618

Section 6: Congenital Heart Disease

Figs 72.73A to C: Two-dimensional transthoracic echocardiography in a case of Uhls anomaly. (A) Apical four-chamber view
showing dilated thin walled RV (arrow) and dilated tricuspid orifice
with tricuspid valve (arrow). (B) Parasternal short-axis view with
Uhls anomaly showing dilated right atrium with thinned out tricuspid valve (arrow). (C) Subcostal sagittal view of the same patient
showing thin tricuspid valve and the lack of apical trabeculations of
right ventricle (arrow).

atresia with intact ventricular septum, ASD, PDA, doublechambered RV, cor triatriatum, VSD, Uhls anomaly113
(Figs 72.73A to C), and LSVC draining into RA.

Uhl's Anomaly113
Though not a disease of the tricuspid valve it is discussed
here because it mimics the presentation of Ebstein
anomaly of TV or dysplastic TV closely. Uhls anomaly

is characterized by the apposition of the epicardium

and endocardium essentially because of the absence
of the myocardial layer. Closest differential diagnosis is
arrythmogenic right ventricular dysplasia but Uhl's is
not familial unlike latter. Echocardiography would show
thinned out parchment-like appearance of the ventricular
wall (Figs 72.73A to C). Diastolic opening of the pulmonary
valve may be seen in some cases. MRI remains the
investigative modality of choice.

PART 5: LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION

LVOT obstruction can occur at various levels:
Valvular aortic stenosis
Subvalvular aortic stenosis
Supravalvular aortic stenosis.

VALVULAR AORTIC STENOSIS

Congenital bicuspid aortic valve occurs in 1.3% of the
population114118 and, therefore, is one of the most common

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

congenital heart malformations. Valvar aortic stenosis is

the most common type of LVOT obstruction accounting
for 70 to 91% of aortic obstructions. It is caused by cusp
deformities, either with or without narrowing of the
annulus. It may manifest in the neonate or progressive
obstruction may develop in an inherently abnormal
valve.
The echocardiographic study of valvular aortic stenosis
should include the following:114-119
Morphology of the stenotic valve
Dimensions of the aortic root
Severity of valvular obstruction
Left ventricular hypertrophy and function, and
Associated anomalies.

1619

The PSAX view at the base of the heart and long-axis view
are best views to determine the morphology and number
of cusps.
Normally, the tricuspid aortic valve has three cusps
of nearly equal size. Three commissures form a Y-shaped
pattern in diastole. In systole, the leaflets open along these
commissures to create a wide-open triangular orifice. The
congenital anomalies of aortic valve comprise a spectrum
of deformities, which include a decrease or increase in
the number of valve cusps, their form, and size. Normal
aortic valve leaflets are thin with unrestricted mobility.
In stenotic valves, leaflets are thickened and domed in
systole. Doming of valve leaflets during systole occurs due
to limited cusp separation leading to restricted mobility
of valve cusps. Subcostal coronal with anterior tilt, apical
four-chamber with anterior tilt, and PLAX views are

particularly useful to define the valve motion. PSAX view

is the best view to define the number of cusps and cusp
morphology.
Following variations occur in the aortic valve
morphology:
Unicuspid aortic valve is separated pathologically
into two typesacommissural and unicommissural.
The acommissural valve is a rare anomaly and has a
single membrane-like leaflet with a central circular
orifice. The orifice is typically eccentric and circular
in systole. In diastole, an eccentrically located valve
closure is seen with raphae. Unicommissural valve is
frequently seen in symptomatic neonates with aortic
stenosis.In systole, the opening of the valve is eccentic
and circular while in diastole raphe is seen and valve
closes eccentrically. The unicuspid valve is generally
stenotic in neonatal period, although occasionally it
has sufficient redundancy and may be the cause of
obstruction in later life.
Congenital bicuspid aortic valve (Figs 72.74A and B)
occurs in about 2% of the general population. It is
formed by the fusion of two cusps. The fusion of left
and right coronary cusps results in a bicuspid valve
with two cusps positioned anteroposteriorly with
the commissures to the right and left. The fusion of
right and noncoronary cusps results in two cusps
positioned right and left, and the two commissures
have an anteroposterior orientation. In some
cases, fused commissures called raphe are seen on
echocardiography and in the closed position may
give the appearance of a tricuspid aortic valve. It is
only in systole that the valve does not open along the
fused commissure. The development of aortic valve

Morphology of Stenotic Aortic Valve

Figs 72.74A and B: Two-dimensional transthoracic echocardiography. (A) Parasternal short-axis view showing bicuspid aortic valve in
diastole with single closure line of fusion; (B) The same patient in systole showing the fused right and noncoronary cusps of the aortic
valve (arrows). (LA: Left atrium; RA: Right atrium; RV: Right ventricle).

1620

Section 6: Congenital Heart Disease

Figs 72.75A and B: (A) M-mode cut across the aortic valve and left atrium showing eccentric closure of the thickened aortic valve; (B)
Showing the same across the normal valve for comparison.

Fig. 72.76: Two-dimensional echocardiography with parasternal

short-axis view showing a quadricuspid aortic valve. (Ao: Aorta;
LA: Left atrium; RA: Right atrium; RV: Right ventricle).

stenosis is variable and may be related to valvular

characteristics. Patients with anteroposteriorly (as
opposed to right-left) and eccentric (vs symmetric)
valve leaflets have faster rate of progression of aortic
obstruction. Patients with fusion of right coronary and
noncoronary leaflets are more likely to have aortic
regurgitation. Combination of bicuspid aortic valve
and aortic coarctation is usually associated with milder
aortic valve disease.
The echocardiographic diagnosis of a bicuspid
aortic valve is based on demonstration of two cusps
and two commissures on PSAX view. Additional
features that support the diagnosis include leaflet
redundancy, infolding, and eccentric valve closure.

In PLAX view, an abnormal eccentric coaptation

line (best seen on M-mode) with systolic leaflet
doming and an abnormal pattern of systolic
opening is seen (Figs 72.75A and B).
Although there is no fixed pattern of coronary
artery origin with a bicuspid aortic valve, usually
the coronary arteries emerge from the anterior
sinus in case of anterior and posterior cusps with
normally related great vessels. In presence of right
and left cusps, LCA arises from anterior part of left
sinus, and right coronary from anterior part of right
sinus.
Congenitally stenotic tricuspid aortic valve has three
aortic cusps. The edges of the cusps are rolled or
gnarled with varying degrees of commissural fusion.
This abnormality is often associated with a narrowed
aortic annulus.
Quadricuspid aortic valve (Fig. 72.76) is a very rare
(0.013%) congenital anomaly. No correlation has been
found between anatomical variation in the size of cusps
and functional status. Although aortic stenosis is rare,
approximately 50% of cases have aortic insufficiency.
Aortic regurgitation is more common with a small
accessory cusp.
In the PSAX view, four diastolic closure lines are
present forming a characteristic X pattern, and
in systole, four cusps open and form a rectangular
configuration. Color flow mapping will show
presence of aortic regurgitation if present.
Pentacuspid aortic valve and hexacuspid aortic valves
have been described in case reports.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1621

Sinotubular Junction
Sinotubular junction is the point of the union of the aortic
sinuses and the tubular portion of the ascending aorta.
The apex of the aortic valve commissures correspond to
the sinotubular junction.
PLAX view is the best view to measure the aortic
valve annulus, the thickness and mobility of the leaflets,
the plane of the valvular orifice, the sinuses of Valsalva,
the sinotubular junction, and the proximal portion of the
ascending aorta.

Severity of Aortic Stenosis119121

Fig. 72.77: Two-dimensional echocardiography with parasternal
long-axis view of left ventricular outflow showing measurements.
(A: Annulus; B: Aortic sinuses; C: Sinotubular junction; LA: Left
atrium; LV: Left ventricle; RV: Right ventricle).

Aortic Root (Fig. 72.77)

The aortic root is the portion of the ventricular outflow
tract that supports the leaflets of the aortic valve delineated
superiorly by the sinotubular junction and inferiorly by the
ventricular junction. The aortic root acts as an individual
hemodynamic entity; integrity of all its components is
essential for normal function. Aortic root dimensions are
assessed at four levels: the annulus, the sinuses of Valsalva,
sinotubular junction, and the proximal ascending aorta.
The aortic root dimensions should be routinely measured,
as it is often dilated in the presence of bicuspid aortic valve,
irrespective of associated hemodynamic disturbances.
This progressive dilatation of aortic root is not prevented
even after aortic valve replacement; as such cardiologists
recommend reconstruction and remodeling of dilated
aorta at surgery for bicuspid aortic valve patients.

Aortic Valve Annulus

Ventriculoarterial junction anatomically corresponds to
the insertion of the arterial trunk into the ventricular mass
that supports it. In the LV, this insertion acquires the shape
of a fibromuscular ring and is described as aortic annulus.
The muscular portion corresponds to the left ventricular
myocardium, which supports the valve, and the fibrous
portion corresponds to the insertion at the level of the
fibrous continuity between the aortic and mitral valve
leaflets.

Direct quantitative assessment of the severity of aortic

valvular stenosis can be obtained using Doppler
echocardiography. At the same time, one should also
look for associated left ventricular hypertrophy and left
ventricular diastolic and systolic dysfunction.
Normal aortic valve blood flow is laminar and peak
systolic velocity of blood flow across the aortic valve rarely
exceeds 1.5 m/s. In aortic valve stenosis, the LV generates
high pressures to overcome the obstruction, resulting
in both turbulent flow and increased velocity across the
valve. The pulsed wave (PW) Doppler helps in localizing
the site of obstruction by demonstrating low velocity in
the LV outflow and increased velocity across the aortic
valve. However, continuous wave Doppler is required to
quantitate the valvular obstruction.
The jet velocities distal to the stenotic aortic valve orifice
are recorded from multiple viewssubcostal, apical, right
parasternal, and suprasternal views. The velocity of the
aortic stenosis jet is defined as the highest continuous
wave Doppler signal obtained from any window. Only
well-defined envelopes should be used for quantification
of velocities to obviate significant errors. The ultrasound
beam must be aligned parallel to the flow for accurate
velocity recording guided by 2D image and color flow.
Angle correction should be avoided. Underestimation of
stenosis severity can occur due to a nonparallel intercept
angle. At higher velocities, a small error may lead to
significant errors of gradients because of the quadratic
relation between velocity and pressure gradient.
The usual cause of overestimation of aortic stenosis
is if one interrogates the mitral regurgitation signal
mistakenly. Both jets occur in systole and in the same
direction. A difference in timing may be helpful as the
mitral regurgitation signal velocity starts during isovolumic
contraction and continues through isovolumic relaxation,

1622

Section 6: Congenital Heart Disease

and the aortic stenosis signal start after the isovolumic

contraction during aortic ejection.
The velocity determination across the aortic valve is
flow-related. Hence, conditions causing increased flow
such as aortic regurgitation and elevated cardiac output
as seen in anemia, anxiety, pregnancy, and exercise will
increase the flow velocity across the aortic valve. Hence, it
is also necessary to determine the velocity proximal to the
aortic valve and do the necessary correction in Bernoullis
equation. Conditions associated with low cardiac output
such as left ventricular failure commonly seen in neonatal
or elderly aortic stenosis preclude the use of valve gradient
as an indicator of severity of valvular stenosis. Another
physiological issue that needs to be considered in the
Doppler assessment of pressure gradients in patients
with aortic stenosis is the phenomenon of distal pressure
recovery. The fluid dynamics of valvular aortic stenosis
are characterized by a laminar high-velocity jet in the
narrowed orifice, with the narrowed segment of the flow
stream (the vena contracta) occurring downstream from
the anatomical valve orifice. As the jet expands and
decelerates beyond the vena contracta, the associated
turbulence results in an increase in aortic pressure
pressure recovery such that when aortic pressure is
measured in the distal ascending aorta, the left ventricular
to aortic pressure difference is less than if aortic pressure is
measured in the vena contracta.

Doppler mean gradient is comparable to mean pressure

gradient measured at the cardiac catheterization
As Doppler mean gradient is the average of all the peak
instantaneous gradients throughout the systole, and
not on single peak velocity, it can be obtained with
greater accuracy and reproducibility
Mean gradient is less affected by transvalvular flow.
Mean gradient is the basis of calculation of valve area
using the Gorlin equation.

Aortic Valve Area

The calculation of aortic valve area is a useful method for
determining the severity of the stenosis independent of
transvalvular flow in contrast to pressure gradient across
the valve. In children where the decision regarding severity
of aortic stenosis remains unanswered in patients with
intermediate pressure gradients, determination of aortic
valve area should also be performed. Aortic valve area can
be measured by the following methods:
PlanimetryThe aortic valve area can be measured
by direct tracing from PSAX view at the level of great
vessels on 2D echocardiography. There are some
limitations in pediatric patients as
Fast heart rate leading to limitation of frame rates.
Error in measurement of small orifice.
Irregular valve opening that is difficult to trace.
Aortic valve area by continuity equation:

122128

Pressure Gradients

Transvalvular pressure gradients are usually calculated

from Doppler aortic velocity profiles. The peak gradient
and the mean gradient are measured. The peak gradient
is determined from the peak velocity using the modified
Bernoulli equation (p = 4V2), and mean gradient by
averaging all the peak gradients in a systolic ejection
period. In general, the Doppler-measured peak gradient
may not correspond to the catheter measured peakto-peak pressure gradient, because Doppler measures
instantaneous peak-to-peak gradient, which is
fundamentally different from the peak-to-peak catheter
gradient usually calculated in the cardiac catheterization
laboratory. In some children, especially with moderate
degree of stenosis, two measurements can differ by
as much as 30 mm Hg. Mean gradients, measured by
averaging the instantaneous catheter or Doppler gradients
over the systolic ejection period, correspond more closely
to each other. The Doppler mean gradient has several
advantages over the Doppler peak instantaneous gradient.

CSAav =

CSALvot VTILvot
VTIav

CSA = Cross sectional area of aortic valve.

V TI = Velocity time integral.
av = Aortic valve.
Lvot = Left ventricular outflow tract.

Critical Neonatal Aortic Stenosis129139

In infants presenting with signs of aortic stenosis in the
first few months of life, echocardiography provides a rapid
noninvasive diagnostic method.
The aortic valve leaflets are thickened and domed.
In many cases, the leaflets are immobile and a clear
systolic opening may not be visualized. The annulus
usually measures 58 mm
Usually, there is post-stenotic dilatation of the
ascending aorta and the ratio of the ascending aorta
to the annulus is more than 1.0. This phenomenon

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

characterizes a LV, generating a pressure gradient

across the aortic valve leading to release of energy in
the ascending aorta and post-stenotic dilatation
The LV is thickened and hypertrophied
Increased echogenicity of the mitral valve and papillary
muscles is seen in PSAX, parasternal long-axis, and
apical four-chamber views
Redirection of fetal flow patterns and delayed
regression of PVR results in the RV and the main PA
being enlarged.
If the ventricular function is normal, the peak velocity
across the valve is increased. More often, neonates present
with severe left ventricular dysfunction, and differentiation
from dilated cardiomyopathy becomes important.
In the latter case, the aortic valve is normal with no
evidence of post-stenotic dilatation. However, associated
cardiomyopathy is difficult to rule out in some cases
and is only diagnosed retrospectively if the ventricular
contractility fails to improve after relief of aortic stenosis.

Aortic Stenosis Versus Hypoplastic

Left Ventricle
In some infants dilated RV may dwarf the LV, which
appears small or even hypoplastic. The following features
help to differentiate and indicate small LV:
The evaluation of shape of the LV may be useful in these
cases. Normal LV is usually ellipsoid and extends to the
cardiac apex in four-chamber view. But the hypoplastic
LV is globular and does not extend to cardiac apex
LV inflow dimension (hinge point of posterior mitral
leaflet to cardiac apex of < 25 mm)
Mitral valve annulus diameter of < 9 mm
Ventriculoaortic junction of < 5 mm, (all measured
from apical four-chamber or long-axis view at enddiastole will indicate hypoplastic LV)
Left ventricular cross-sectional area measured in
the PLAX view that included the mitral valve, aortic
valve, and left ventricular apex at end diastole of
< 2 cm, usually predicts hypoplastic of LV and
nonsurvival after balloon aortic valvotomy
Predominant or total antegrade flow in the ascending
aorta and transverse arch is indicative of an
adequate LV
A new discriminant analysis was found to more
accurately predict survival with a biventricular
circulation than with the model using the traditional
criteria. The new criteria first emphasized the need

1623

for having an adequate mitral valve by acquiring

the mitral valve area (calculated by assuming the
morphology was that of an ellipse with radii measured
from the PLAX and apical four-chamber views) to
have a z-score of > 2. Only then can the new criteria
be applied. These criteria consist of (a) aortic annulus
z-score measured from the PLAX view, (b) ratio of
the long axis of the LV to the long axis of the heart, (c)
endocardial fibroelastosis grade: none, mild (affecting
papillary muscles only), moderate (affecting papillary
muscles and some of the endocardium), and severe
(affecting papillary muscles and extensive portions
of the endocardium). A new regression equation was
developed:
Score = 10.98 (BSA) + 0.56 (Aortic annulus z score) +
5.89 (LAR) 0.79 (EFEgrade) 6.78
where
EFE grade is 0 for none or mild, and 1 for moderate or
severe.
The threshold score for survival to be better with
biventricular versus univentricular repair is > 0.65.

Hemodynamics
The LV becomes hypertrophied with increasing left ventricular outflow obstruction. Severe unrelieved obstruction may lead to an oxygen demand/supply mismatch
leading to subendocardial ischemia and fibrosis.
Ventricular systolic function is assessed by the
conventional methods of calculating shortening fraction
and ejection fraction. This is an important parameter in
the echocardiographic assessment of an aortic stenosis
patient as the assessment of severity by pressure gradients
depends upon ventricular function. A decrease in function
decreases the transvalvular flow and the gradients no
longer reflect the severity of obstruction.
Diastolic ventricular function is assessed by the filling
abnormalities of the LV. From the mitral valvular Doppler
recording peak flow velocities, filling rates and proportion
of flow in various phases of diastole may be assessed.
Comparative studies of these subjects with normal controls
have revealed higher E-velocity, a much higher A-velocity,
therefore an inverse E/A ratio. The percentage of total
Doppler area in the first third of diastole was significantly
lower and the percentage of the total Doppler area under
the A-wave was higher. M-mode tracing in PLAX view at
the mitral valve may show a b-bump or a c-interruption if
the LVEDP is elevated.

1624

Section 6: Congenital Heart Disease

Associated Anomalies129-135
A PDA is seen in 20 to 65% cases of valvular aortic stenosis.
CoA is found in 11 to 53% cases and stenosis of the mitral
valve in 25% cases. Other anomalies like VSD and mitral
valve abnormalities are also common and should be
looked for.

SUBVALVULAR AORTIC STENOSIS

Subvalvular aortic stenosis is responsible for 8 to 30% of
cases of LVOT obstruction and corresponds to 1.2% of
all cardiac anomalies.135-140 Classically, the subvalvular
aortic stenosis has been divided into fixed and dynamic

Fig. 72.78: Two-dimensional echocardiography in parasternal

long-axis view showing subvalvular aortic stenosis with accessory
tissue of mitral valve (arrow). (Ao: Aorta; LV: Left ventricle; RV:
Right ventricle).

Fig. 72.79: Two-dimensional echocardiography in parasternal

long-axis view showing the ventricular septal defect (VSD; star)
with posterior malalignment of the outlet septum (arrow). (Ao:
Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).

obstruction. Dynamic obstruction is part of hypertrophic

cardiomyopathy and systolic anterior motion of mitral
valve, not being discussed in this chapter.
Echocardiographic evaluation of fixed subvalvular
aortic stenosis includes:
Cause of subvalvular stenosis.
Presence of aortic regurgitation.
Associated anomalies.
Severity of obstruction.
Cause of subvalvular aortic stenosis.140150 There are
several types of fixed subaortic obstructions:
Discrete fibrous membrane (Fig. 72.78).
Fibromuscular collar.
Tunnel subaortic stenosis.
Posterior displacement of infundibular septum with
discrete narrowing of the LVOT (Fig. 72.79).
Other less common causes are accessory mitral
valve tissue or tissue arising from membranous
septum protruding into LVOT, systolic anterior
motion of anterior leaflet of mitral valve as in cases of
hypertrophic cardiomyopathy (Figs 72.80 to 72.82).
Most commonly, a discrete fibrous membrane
or fibromuscular shelf encircles the LVOT. Rarely, it
extends for a longer distance (more than one third of
aortic diameter) and forms a tunnel-shaped obstruction.
Abnormal tissue may extend and tether the aortic valve or
the anterior mitral leaflet.
Fixed subaortic obstruction usually occurs in
association with other defects in 6470% of the cases and
is often diagnosed when the child is investigated for them.

Fig. 72.80: Two-dimensional echocardiography. Parasternal longaxis view in a child with discrete subaortic membrane showing
anterior insertion of the membrane to the ventricular septum below
the right aortic cusp (arrow). (Ao: Aorta; LA: Left atrium; LV: Left
ventricle; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1625

Fig. 72.81: Two-dimensional echocardiography. Parasternal longaxis view in a 2-year-old child with discrete circumferential subaortic membrane (arrows) close to aortic valve. (Ao: Aorta; LA: Left
atrium, LV: Left ventricle; RV: Right ventricle).

Fig. 72.82: Two-dimensional echocardiography. Parasternal longaxis view in a child with hypertrophic obstructive cardiomyopathy
showing the severely hypertrophied interventricular septum and
systolic anterior motion of the anterior leaflet of the mitral valve
(arrow). (LA: Left atrium; LV: Left ventricle; RV: Right ventricle
Ao: Aorta).

Echocardiographically, subaortic stenosis can be

studied from the parasternal long-axis, the apical fourchamber, and subcostal coronal views with anterior tilt.
Membranous subaortic stenosis appears as a discrete
shelf adherent to the interventricular septum beneath
the aortic valve or up to 2.5 cm below it. The PLAX view
is highly sensitive in detecting discrete subaortic stenosis;
however, in most cases, the anterior insertion alone is
visualized because the membranous diaphragm being
directed posteriorly toward the left ventricular posterior
wall and mitral valve is aligned parallel to the ultrasound
beam in the PLAX view. In some cases, however, with
careful interrogation, anterior insertion to ventricular
septum below the right aortic cusp and posterior insertion
to the base of anterior mitral leaflet insertion can both
be seen. In four-chamber subcostal coronal views with
anterior tilt, the ultrasound beam is perpendicular to the
subaortic ridge and profiles the anatomy of membrane
better. Careful PSAX scans of the LVOT are more likely to
visualize the extent of attachment of the membrane. The
membrane may come in and out of the plane of PSAX
section very quickly because of rapid cardiac motion and,
frame-by-frame analysis is required to assess it adequately.
In the majority of cases, it has the shape of a horseshoe;
rarely is it a complete ring and in most cases only one
insertion is visualized at a given time. Luminal diameter
is not a criterion for severity of obstruction. With discrete
subaortic stenosis, the jet of stenosis often damages
the aortic valve leading to thickened aortic valve on 2D
echocardiography. Color flow mapping reveals presence

of aortic regurgitation. In contrast to bicuspid aortic valve,

there is no post-stenotic dilatation of the ascending aorta.
The fibromuscular collar is a thick muscular ring that
forms at a lower level in LVOT than the subaortic membrane
and is seen easily in parasternal long-axis, four-chamber
(apical and subcostal coronal) with anterior tilt as a thick
fibrous shelf projecting into LVOT. A fibromuscular tunnel
is diagnosed by the same views and gives the appearance
of a long narrow tract of subaortic narrowing.
Posterior displacement of outlet septum: Subaortic
stenosis due to posterior malalignment of outlet septum
occurs with a nonrestrictive perimembranous VSD.
This subgroup usually has associated arch anomalies
such as CoA or arch interruption. Best view to profile
malalignment of outlet septum is parasternal long-axis,
although four-chamber views with anterior tilt also define
the posterior malalignment of outlet septum leading to
subaortic narrowing. Arch anomalies should be defined in
detail from suprasternal views in these cases.
With subaortic stenosis, on M-mode trace the aortic
valve leaflets shows a slight flutter and early to mid
systolic closure along with other evidence of significant
left ventricular outflow obstruction such as left ventricular
hypertrophy. Doppler echocardiography quantitates
the degree and site of obstruction. Color Doppler
illustrates the flow acceleration at the site of stenosis and
aliasing velocities beyond that. It helps in positioning
the ultrasound beam parallel to the left ventricular
outflow in various views. The total gradient across the left
ventricular outflow is quantitated by continuous wave

1626

Section 6: Congenital Heart Disease

Doppler using the modified Bernoullis equation. The right

infraclavicular view with the patient turned toward the
right usually records the highest velocities. PW Doppler is
used to localize the site of outflow obstruction by placing
the pulsed Doppler sequentially and noticing the site of
increase in outflow velocity.
In diffuse tunnel obstruction, the maximal velocity
is produced inside the tunnel and this can be missed
altogether by Doppler and catheter techniques, at times.
In these latter cases, the pressure drop caused by viscous
friction along the tunnel may cause a lower Doppler
velocity to be recorded, resulting in an underestimation
of the true gradient. The tunnel subaortic stenosis may be
associated with a narrow aortic annulus and often causes
significant concentric left ventricular hypertrophy. In
infants with CoA or arch interruption, reduced aortic valve
diameter and increased mitralaortic separation could
be precursors to subaortic obstruction. The extension
of subaortic fibroelastic tissue to involve the aortic
root at the site of insertion of the aortic valve cusps and
increasing fibrosis of the aortic root may be responsible
for this discrepancy. The size of the aortic root has a
marked effect on the optimal relief of the fixed subaortic
stenosis. In patients with fixed subaortic stenosis, the
aortic root can be small (25%) and should be measured
preoperatively because in the presence of small aortic
root, a special surgical technique is required. There is a
13% incidence of left ventricular outflow abnormalities in
immediate family members. Hence, their screening is an
essential part of patient evaluation. In 65 to 70% of cases of
subaortic obstruction, associated defects are present and

include VSD, CoA or arch interruption, bicuspid aortic

valve, supravalvular mitral stenosis, and persistence of the
LSVC with dilated coronary sinus causing restrictive left
ventricular filling.
Discrete subvalvular aortic stenosis is a progressive
lesion. Fixed subaortic stenosis has been noticed to
progress more rapidly in the presence of associated lesions
than isolated subaortic stenosis. This implies that careful
screening should be done for associated lesions in all
cases of discrete subaortic stenosis. Tethering of mitral
and aortic valves is also known to develop subsequently in
serial echocardiograms.
Even after adequate surgical relief, this lesion is known
to recur and patients require long-term follow-up.
Aortic insufficiency can also progress and should be
carefully evaluated in follow-up of cases of discrete subaortic
stenosis. The distance between the diaphragm and the aortic
valve should be noted in the initial echocardiogram. The
mechanism responsible for aortic regurgitation is believed to
be repetitive trauma caused by the high velocity jet through
the subvalvular stenosis as well as extension of the fibroelastic
tissue of discrete subaortic stenosis toward the base of one or
more aortic cusps.

Fig. 72.83: Two-dimensional echocardiography. Apical fourchamber view with anterior tilt and color flow mapping in a case
of supravalvular aortic stenosis. The turbulence (arrow) begins
above the level of the aortic valve (arrow). (AA: Ascending aorta;
LV: Left ventricle).

Fig. 72.84: Two-dimensional echocardiography. Parasternal

long-axis view of the left ventricular outflow tract showing severe
stenosis at the level of the sinotubular junction (arrow) in a patient
with supravalvular aortic stenosis. (Ao: Aorta; LA: Left atrium; LV:
Left ventricle; RV: Right ventricle).

SUPRAVALVULAR AORTIC STENOSIS

(FIGS 72.83 AND 72.84)151154
Supravalvular aortic stenosis is the rarest of left ventricular
outflow obstructions (211%). It occurs at the sinotubular
junction and produces a localized or diffuse narrowing.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

This is a group of lesions with varying anatomy and is

characterized into three types:
Membranous type (<25%)
Hourglass type (5075%)
Uniform hypoplasia of the ascending aorta (<25%).
During an echocardiographic diagnostic study, the
following anatomical features need to be defined:
Morphology and severity of supravalvular aortic
stenosis
Anatomy of the aortic valve
The coronary anatomy
Associated anomalies.

Morphology
The obstruction is best imaged in parasternal long-axis,
apical five-chamber and subcostal views of the LVOT, or
suprasternal long- and short-axis views and high right
parasternal views. In the parasternal and apical fivechamber views in the normal heart, the aortic diameter
increases at the level of the sinuses of Valsalva and then
decreases at the superior border of the aortic sinuses. The
diameter of the ascending aorta above the aortic sinuses is,
however, the same as the diameter of the ventriculoaortic
junction or aortic root in the normal heart.
The detection of obstruction in hourglass deformity
depends upon visualization of an obvious decrease
in the caliber of the vessel relative to the surrounding
normal areas, namely at sinotubular junction, then
some dilation, and narrowing again. The following
measurements are taken in the PLAX view: (a) aortic
annulus, (b) maximal diameter at sinuses of Valsalva, (c)
sinotubular junction, (d) narrowest part, and (e) aorta
distal to the obstruction.
Measurements are taken from the inner aspect of
the aortic root echo to the inner aspect of the posterior
root echo. Careful attention should be paid to aligning
the probe so that long axis of the vessel and adequate
visualization of the lumen both proximal and distal to the
area of obstruction is obtained. Oblique angulation of the
cross-sectional scan plane to vascular lumen may give
the appearance of the aorta being cut-off as beam passes
obliquely through the lateral wall of the vessel.
Normally, the diameter of the sinotubular junction is
either equal or slightly (12.5%) more than the diameter
of the aortic annulus. In supravalvular aortic stenosis, a
percentage decrease of > 25% from the annulus is noted.
There is a rough correlation in the percentage decrease

1627

in diameter with catheter peak gradients. In the diffuse

type of supravalvular aortic stenosis, the aortic hypoplasia
extends from the sinotubular junction to the innominate
artery. Usually, the aortic arch and arch vessels are also
involved. A severely hypoplastic and narrowed segment
may have retrograde flow from PDA supplying the arch
vessels and the descending aorta. When the aorta is
diffusely hypoplastic, the annulus is frequently also
involved and can no longer be used as a reference for
estimation of severity.
The membranous lesion generally appears as a discrete,
linear echo extending inward from the walls of the aorta
and encroaching on the vascular lumen. The membrane
is typically located at the sinotubular junction just above
the insertion of the superior LA wall into the posterior
aortic root. The separation between the inner margins of
an obstructive membrane is less than the diameter of the
outflow tract at the annulus.
Doppler interrogation aids in confirmation of diagnosis.
The site of stenosis is confirmed both by color flow and
pulsed Doppler, while maximal velocity is obtained by
continuous wave Doppler. The jet flow of supravalvular
aortic stenosis is often directed toward the innominate
artery; consequently, the highest value of the peak velocity
is often obtained by aligning the Doppler beam parallel
with the innominate artery in the suprasternal views.
Pressure gradient across the aortic root is calculated
from the modified Bernoullis equation and correlates
with catheter-measured peak-to-peak gradient across
the fibrous diaphragm. However, in hourglass deformity,
gradual re-expansion of the aorta distal to stenosis leads to
pressure recovery and discrepancy between Doppler and
catheter-measured gradients.

Aortic Valve Anomalies

Aortic valve abnormalities have been reported in 25 to 45%
of the cases. It is important to assess the dynamics of aortic
valve motion throughout the cardiac cycle. In diastole,
the valve may function normally; however, in systole its
motion can be limited and the opening incomplete due to
spatial restriction; this has been called pseudovalvular
stenosis. The presence of bicuspid aortic valve, valve
thickening, and commissural fusion have been reported
in a high percentage of cases. Aortic valve regurgitation
has been reported in 13 to 66% cases of supravalvular
aortic stenosis and needs careful evaluation. Anomalies of
aortic valve can be seen in the same view as described for
valvular stenosis.

1628

Section 6: Congenital Heart Disease

Coronaries
Coronary artery abnormalities are invariably associated
and may be:
Dilated and tortuous coronary arteries due to
exposure to high systolic pressures proximal to the site
of obstruction (rarely coronary artery aneurysms have
been reported)
Coronary orifice can be narrowed by the overhanging,
fibrous bridge.
Rarely, the orifice of coronary artery can be completely
obstructed as the valve cusps become adherent to the
aortic wall.
With the patient in the left lateral position, the
transducer is placed in the left parasternal position
to obtain a short-axis view of the great vessels. Slight
adjustment of the imaging plane, so that it traverses the
heart below the pulmonary trunk, allows for visualization
of the origin and proximal portion of coronary arteries.
The ostia and the coronaries should be carefully assessed
for dilatation, tortuosity, or narrowing. The apical fourchamber view demonstrates RCA coursing along the right
AV groove and aiming the transducer superiorly toward
the left ventricular outflow, the branching of left anterior
descending (LAD), and left circumflex may be seen
besides their profiling in the PSAX view. In severe cases,
myocardial ischemia and infarction have been reported
as such regional wall motion abnormalities should be
assessed at echocardiography.

Presence of Stenosis of the Central or

Branch Pulmonary Arteries (Fig. 72.85)
Stenosis of pulmonary arteries proximally at the bifurcation
or its branches can be associated with supravalvular
aortic stenosis, particularly with Williams syndrome. It
can be multiple sites along the branches or a generalized
hypoplasia. Central pulmonary arteries can be evaluated
by echocardiography, but if peripheral branches seems to
be involved, other modalities such as spiral CT/MRI will
be needed.

Serial Echocardiograms
The condition is usually progressive in nature and cases
with mild narrowing need careful long-term follow-up.

AORTIC REGURGITATION
Congenital aortic insufficiency is a rare entity as an
isolated lesion. It frequently occurs in association with

Fig. 72.85: Two-dimensional echocardiography high parasternal

short-axis view with color flow mapping, in a 2-year-old child with
Williams syndrome, showing bilateral pulmonary artery stenosis
with turbulent flow. (LPA: Left pulmonary artery; MPA: Main pulmonary artery; RPA: Right pulmonary artery).

other congenital heart defects, aortic root dilatation,

and infectious processes of the aorta. Causes of aortic
regurgitation are highlighted in Table 72.12.
The primary cardiac abnormalities are the commonest
cause of congenital aortic regurgitation such as a
bicuspid or quadricuspid aortic valve. Significant aortic
regurgitation presenting in neonatal period can be due to
two causes:
Aorticleft ventricular tunnel.
Unguarded aortic valve.
While evaluating the patient with aortic regurgitation,
the following things should be assessed on echocardiography:
Left ventricular outflow abnormality-cause of aortic
regurgitation.
Severity of regurgitation.
Left ventricular dimensionsend-systolic and enddiastolic.
Left ventricular systolic and diastolic function.

Left Ventricular Outflow AbnormalityCause of Aortic Regurgitation

PLAX view and PSAX view at the level of great vessels are the
best views to define the LVOT abnormalities as described

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Table 72.12: Causes of Aortic Regurgitation

Primary congenital cardiac abnormality

Aortic valve abnormality
Bicuspid aortic valve
Quadricuspid aortic valve
Absence of aortic valve cusps (unguarded aortic orifice)

Aortico-left ventricular tunnel (Figs 72.86A and B)

Annulo-aortic ectasia (Figs 72.87 A and B).

Connective tissue disorders with aortic root dilatation

Marfan syndrome, EhlersDanlos syndrome

Turner syndrome with aortic ectasia (Figs 72.87A and B)

Association with other forms of congenital heart defects

Aortic valve prolapse into ventricular septal defect (doubly
committed, outlet muscular, or perimembranous)

Dilatation of aortic root as in tetralogy of Fallot physiology

Truncus arteriosus

1629

severity of regurgitation, ventricular dimensions, and

ventricular function is very important before taking the
decision about management, whether the patient will
require medical follow-up, valve repair, or will need aortic
valve replacement. Aortic valve annulus and aortic root
size should be measured in patients undergoing aortic
valve replacement in PLAX view. If a patient is planned
for the Ross procedure, then pulmonary root should be
measured in addition to aortic root measurement.
Etiology of aortic regurgitation present in other heart
defects such as VSD with aortic valve prolapse (commonly
doubly committed, outlet muscular, or perimembranous),
subaortic stenosis, and truncus arteriosus has been
discussed in respective sections.

Aortic Root Dilatation

in an earlier section. Aortic valve cusp number and

anatomy, rolled, gnarled, and inadequate, or redundant
and prolapsing should be defined. Determination of

Aortic root dilatation occurs in connective tissue disorders

like Marfan syndrome, EhlersDanlos syndrome,
Turner syndrome, and multivalvular heart diseases.
With connective tissue disorders, aortic root dilatation
is progressive; initially it involves sinuses of Valsalva,
ascending aorta, then dilatation progresses to involve
aortic annulus leading to distortion of aortic valve, and

Rheumatic fever (mostly associated with mitral valve disease)

Infectious processes of the aortic valve
Bacterial endocarditis

Figs 72.86A and B: Two-dimensional echocardiography. Parasternal long-axis view with slight anterior tilt and color comparison
in a 7-day-old neonate with aortico-left ventricular tunnel (arrow). Color flow mapping shows aortic regurgitation. (Ao: Aorta; RV: Right
ventricle; LV: Left ventricle; T: Tunnel).

1630

Section 6: Congenital Heart Disease

Figs 72.87A and B: Two-dimensional echocardiography a case of annulo-aortic ectasia in a 2-year-old girl. (A) Parasternal long-axis
view showing the dilated (ectatic) aortic root; (B) Subcostal coronal view with anterior tilt showing the left ventricular outflow tract with
ectatic aortic root in the same patient. (Ao: Aorta; RV: Right ventricle; LV: Left ventricle; LA: Left atrium).

Table 72.13: Checklist of Aortic Insufficiency in Pediatric Patients

Look for the etiology of aortic valve insufficiency and assess

the need for surgical intervention
Assess for the following:

Thickening of the aortic valve cusps

Morphology of the commissures, presence of a raphe

Cusp prolapse

Detached or flail valve cusps

Vegetations

Aortic root dilatation

Left ventricle (LV) wall thickness
LV end-diastolic dimension
LV end-systolic dimension

LV shortening fraction and LV ejection fraction

aortic regurgitation. While evaluating the patient with

connective tissue disorders such as Marfan syndrome,
aortic root measurements should be taken at four levels
aortic annulus, sinus of Valsalva, sinotubular junction,
and ascending aorta 1 cm above the sinotubular junction,
compared with age-related norms and should be followed
up serially. Undue dilatation or rapid increase in these
parameters will identify the patient who is at a risk of
development of aortic dissection and needs elective aortic
root replacement procedure.

Severity of Regurgitation
With the use of 2D echocardiography, left ventricular
dilatation and function can be assessed, and on color flow

mapping and pulsed Doppler interrogation, severity of

aortic regurgitation can be evaluated. Parameters of AR
are not discussed in detail as these are covered in chapters
on acquired heart diseases. A reference checklist is given
in Table 72.13.

SINUS OF VALSALVA ANEURYSM

(FIGS 72.88A AND B)155167
Sinus of Valsalva aneurysm is a rare cardiac anomaly. It
is thought to result from absence of normal elastic tissue
and abnormal development of the bulbus cordis leading
to a separation between the aortic media and the annulus
fibrosus, which in turn leads to thinning of the wall of
the aortic sinus. Other diseases that involves the aortic
root may also lead secondarily to the sinus of Valsalva
aneurysm. This malformation occurs more frequently in
males than females (3:1) and in patients of Asian (India in
Asia) origin. It can be congenital or occurs as an acquired
lesion in patients with connective tissue disorders such as
Marfan syndrome, EhlersDanlos syndrome, annuloaortic
ectasia, ankylosing spondylitis, or with endocarditis. The
weakened wall of sinuses of Valsalva may progressively
dilate under systemic pressure and eventually rupture into
a low-pressure chamber.
Echocardiographic evaluation of aneurysm of sinus of
Valsalva includes:
Sinus involved showing characteristic of aneurysm
Presence of obstruction caused by the aneurysm or
rupture of the aneurysm

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1631

Figs 72.88A and B: Ruptured sinus of Valsalva in a 30-year-old male patient. (A) Two-dimensional echocardiography with color compare in parasternal short-axis view showing aneurysm of noncoronary sinus rupturing into the right atrium (arrow). (Ao: Aorta; LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Cavity where the aneurysm protrudes or ruptures

Aortic regurgitation
Associated anomalies.
Any of the aortic sinuses may be involved, and more
than one sinus may be rarely involved (0.6%). Right
coronary sinus is most commonly involved (73%),
followed by the noncoronary sinus (20%); left coronary
sinus (6%) is the least commonly involved. Enlargement
of the aneurysm is in the direction of least resistance, so
most commonly it bulges into the right-sided chambers.
Unruptured aneurysm can cause obstruction of the
surrounding structures like the RVOT, right PA and,
descending aorta. Tricuspid valve distortion leading
to tricuspid regurgitation can occur with aneurysm
bulging into RV inflow. Rarely it can burrow into the
interventricular septum resulting in complete heart
block. The aneurysm can also get calcified. The aneurysm
may rupture into a neighboring chamber, leading to
development of symptoms acutely. Bulging or rupture of
the right sinus can occur into RV (commonest), RA, PA, LV,
or into the pericardium. Aneurysm of noncoronary sinus
can bulge or rupture into RA (most commonly), RV, LA,
LV, or pericardium. Aneurysm of left coronary sinus can
extend into LA, RA, LV, RV, PA, or pericardium.

Aortic Regurgitation
Aortic regurgitation is a frequent occurrence in patients
with an aneurysm of sinus of Valsalva with or without
rupture. Aortic regurgitation occurs due to loss of support
of the aortic sinus and its annulus and its distortion. In

addition, the runoff produces a Bernoullis effect, due to

which the cusp is pulled away from its line of apposition
leading to increased incompetence. Aortic regurgitation
is more common when aneurysm of sinus of Valsalva
is associated with VSD (43.3%) as compared to intact
ventricular septum (25.9%).

Associated Anomalies
Deformation and rupture of the sinus of Valsalva is
commonly associated with VSD, so it has to be looked
for carefully (Fig. 72.91). The right sinus may prolapse
through a doubly committed VSD towards RVOT, causing
RVOT obstruction and subsequent rupture. Both the right
coronary and noncoronary sinuses may prolapse through
a perimembranous VSD into RV inflow, which may lead to
distortion of tricuspid valve and tricuspid regurgitation.
Other associated anomalies are pulmonary stenosis, TOF,
ASD, bicuspid aortic valve, CoA, and LSVC, which needs to
be scanned carefully.
Aneurysm of sinus of Valsalva can be best defined
from parasternal long-axis, subcostal coronal and apical
four-chamber views with tilting the transducer toward RV
inflow and outflow. PSAX view also defines the anatomy
with the sinus protruding into RV inflow, outflow, RA,
LA, or into the PA. Combination of 2D echocardiography
with color flow mapping and pulsed/continuous wave
Doppler interrogation is helpful in detailed profilation
of the lesion. With rupture of aneurysm into right-sided
chambers, there will be dilatation of right-sided chambers
with large left-to-right shunt and rise of pressures. With

1632

Section 6: Congenital Heart Disease

rupture of aneurysm into LA or LV, these chambers will be

volume-loaded with rise in left atrial and LVEDPs. Color
flow mapping demonstrates rupture of aneurysm and
the cavity into which it opens. Rupture into RA, LA, RV, or
PA will result in continuous flow from aortic sinus to the
receiving cavity, while with rupture into LV, there will be
only diastolic flow. Color flow mapping also defines the
aortic regurgitation. M-mode echocardiography is the
best way to demonstrate direction of shunt and its relation
to cardiac cycle, with ruptured sinus of Valsalva (RSOV),
a continuous flow from aorta to receiving low-pressure
chamber can be demonstrated.
The measurements of aortic annulus, sinotubular
junction, and ascending aorta should be made. Also, the
exact sinus involved by the aneurysm and size of its final
opening and distance from the coronary orifice should be
measured when contemplating RSOV device closure.
RSOV aneurysm should be differentiated from
aorticocameral tunnel, the most important feature being
the origin of tunnel will be above the sinus of Valsalva.

AORTOCAMERAL
COMMUNICATIONS168174
Aortocameral communications are abnormal communications between the root of aorta and one of the cardiac
chambers, the commonest being aortico-left ventricular
tunnel, followed by aortico-right atrial tunnel, aortico-RV
tunnel, and least in frequency is aortico-LA tunnel.

Aortico-Left Ventricular Tunnel

(Figs 72.86A and B)
Aortico-left ventricular tunnel is a rare congenital
malformation characterized by abnormal paravalvular
communication between anterior aspect of aorta and LV. It
causes progressive left ventricular failure and aneurysmal
dilation of aorta.
Incidence of aortico-left ventricular tunnel has been
estimated to be around 0.1% of congenitally malformed
hearts from review of clinical and pathological material
and 0.46% of cardiac malformation identified on fetal
echocardiography. About twice as many cases have been
reported in males as in females, but it is rarely seen in
patients of Asian, Oriental, and African descent.
Echocardiography in neonate and young infant
helps demonstrate the entire course of tunnel as well
as its relationship to aortic root, sinuses, and coronary
ostia. The PLAX view shows a septal dropout at the
anterosuperior part of interventricular septum, with a free

communication noted anteriorly with the RVOT ending

distal to right coronary sinus of aorta. In the PSAX view, an
echo dropout can be seen at the level of aortic valve. There
is a crescent-shaped structure wrapping around the right
coronary cusp anteriorly, clearly distinct from aortic root.
The aortic origin can be shown to be above the sinus and
separate from the origin of both coronary arteries enabling
differentiation of tunnel from sinus of Valsalva fistula.
Occasionally, a coronary artery may arise from the tunnel.
In neonates and infants, it is possible to demonstrate the
coronary artery origin on 2D echocardiography; however,
in older patients it may be difficult.
In apical four-chamber and subcostal coronal views
with anterior tilt, the tunnel is seen to protrude into RVOT.
Subcostal sagittal view also shows bulging of tunnel into
RVOT , which can cause subpulmonary stenosis.
Both pulsed Doppler and CFI show systolic antegrade
flow and diastolic retrograde flow within the tunnel. CFI
is particularly useful in assessing coexisting central aortic
regurgitation. Presence of subpulmonary stenosis should
also be defined with the use of color flow mapping and
pulsed Doppler interrogation. Associated anomalies have
been described in several studies and include bicuspid
aortic valve, aortic stenosis, PDA, pulmonary stenosis,
VSD, and sinus of Valsalva aneurysm. These anomalies can
also be demonstrated on 2D echocardiography.

Aortico-Right Ventricle Tunnel

Aortico-RV tunnel is another rare congenital malformation
in which the tunnel connects aorta above the level of sinus
of Valsalva to the RV. PLAX view on 2D echocardiography
defines the tunnel as a communication starting above
the sinus of Valsalva, and with anterior tilt from this view,
opening of this tunnel into RV can be defined. Color flow
mapping will reveal high velocity flow into the RV during
systole as well as diastole. This needs to be differentiated
from RSOV into RV. In RSOV, the aortic end of tunnel is
situated below the coronary arteries. RV is usually dilated
and hypertrophied, as it is volume- and pressure-loaded.

Aortico-Right Atrial Tunnel

Aortico-right atrial tunnel, which connects aorta above the
level of sinus of Valsalva to the RA, is very rare. The tunnel,
more commonly from left sinus, runs posterior to the aorta
and then opens into the RA. PSAX view at the level of great
vessels defines the length of tunnel joining RA and aorta.
Color flow mapping will show high-velocity, continuous
flow from aorta to the RA.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1633

PART 6: ECHOCARDIOGRAPHIC ANATOMY OF TETRALOGY OF

FALLOT WITH PULMONARY STENOSIS
Prevalence of TOF (regardless of pulmonary valve morphology) ranges from 0.26 to 0.48 per 1,000 live births.
TOF consists of anterior and cephalad deviation of the
outlet septum leading to a large malaligned (mostly perimembranous type) VSD with narrowing of the RVOT.175179
Echocardiography is the most important investigation for
any such case and has replaced invasive angiography for
preoperative evaluation of TOF patients. The evaluation
of TOF has to be done in sequential analysis as for any
other case of CHD. It is extremely rare to have TOF with
isomeric hearts. PLAX view (Figs 72.89A and B) shows
the defect well. Subcostal paracoronal view (Fig. 72.90)
also identifies the anatomy of the tetralogy well, showing
the VSD, infundibular septum, RVOT, and even branch
pulmonary arteries, particularly right pulmonary artery
(RPA). The important salient features of the lesion are
discussed below.

Ventricular Septal Defect

The presence of the large malaligned VSD and dilatation of

the aortic root can result in aortic override. This can also be
seen with an isolated large VSD without anterocephalad
deviation of the outlet septum. Echocardiographically,
aortic override is best documented in the PLAX view
(Fig. 72.91). The aortic mitral continuity is also best seen
in this view.

Commonest VSD seen in 74% of cases of TOF is perimembranous outlet VSD, since the tricuspid valve forms
one of the margins of the defect. Echocardiographically,
the subcostal sagittal and coronal views show tricuspid
aorticmitral continuity, which is a differentiating feature
from DORV. The aortic valve forms the roof of the defect;
the posterior-inferior margin is the area of tricuspid
aorticmitral continuity and the anterior and anteriorinferior margins are muscular. The PSAX view at the level of
the aortic valve shows that the VSD extends from the area
of the tricuspid valve anteriorly to the area of the muscular
septum (Fig. 72.91). The outlet septum itself separates the
aortic valve from the pulmonary valve.
Muscular outlet VSD is seen in 20% of cases of TOF. A
muscle bar is present in the posterior-inferior margin of
the defect and this separates the tricuspid valve from the
aortic valve. Thus, except for the superior margin, which is
formed by the aortic valve, the VSD in rest of the margins
is entirely muscular. Echocardiographically, this feature is
well seen in the subcostal coronal view as a bar of muscle separating the aortic valve from the tricuspid valve. In
the PSAX view also the aortic valve is separated from the
tricuspid valve by a muscle bar. The importance of recognizing this entity is that this bar of tissue separates the conduction tissue from the margins of the defect making the
chances of heart block unlikely when the VSD is closed.

AORTIC OVERRIDE

Figs 72.89A and B: Two-dimensional echocardiography in parasternal long-axis view with color comparison in a 2-year-old child with
tetralogy of Fallot showing a large perimembranous ventricular septal defect (VSD), aortic override ( ), and aortic mitral continuity
(arrow). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

1634

Section 6: Congenital Heart Disease

Fig. 72.90: A 2-year-old child with tetralogy of Fallot. Two-dimensional echocardiography with subcostal paracoronal view showing
the large perimembranous ventricular septal defect (VSD; arrow)
with anterior malalignment of the septum, causing subvalvular pulmonary stenosis. (Ao: Aorta; RVOT: Right ventricular outflow tract;
RV: Right ventricle).

Fig. 72.91: Two-dimensional echocardiography. Parasternal

short-axis view showing a large ventricular septal defect with
anterior malalignment of the septum (arrow) leading to subvalvular
pulmonary stenosis. (Ao: Aorta; PA: Pulmonary artery; RA: Right
atrium; RV: Right ventricle).

In 2 to 3% of the case of TOF, the VSD is juxta-arterial or

doubly committed VSD (Figs 72.92 and 72.93).180 The outlet
septum is absent and the roof of the VSD is formed by the
aortic and pulmonary valve in continuity. The inferior
margins are muscular unless the VSD is large enough
to extend to the area of tricuspid aortic continuity (i.e.
perimembranous). The aortopulmonary continuity in this
type of VSD is best visualized in the subcostal paracoronal
and PSAX views.
Inlet extension of VSD is recognized on echocardiography from the apical four-chamber view. The important
echocardiographic features in this case will be dropped
out at the level of the AV valve. TOF can also be associated
with a complete AVSD. This is differentiated from the
inlet extension of a perimembranous VSD by (a) absence
of offsetting, (b) presence of a common AV orifice (best
visualized in the subcostal sagittal scan, and (c) ASD of the
ostium primum variety.
Additional VSDs have to be evaluated in any case
of TOF (Fig. 72.94). The most common site is in the
trabecular septum. Because of the large proximal
unrestricted perimembranous VSD and equal right and
left ventricular pressures, these smaller defects can be
missed even on the color scan unless great care is taken
to scan the interventricular septum in detail. The best
views to visualize these defects on echocardiography are
the subcostal sagittal and the apical four-chamber views.
Use of the zoom feature in inverted view of the septum and

low scale of color flow mapping to scan the entire part of

the interventricular septum in more detail increases the
chances of detecting additional VSDs more reliably.
Restrictive VSD (Hoffmanns variant; Figs 72.95, 72.96,
and 72.97): This occurs in 1.5% of the cases of TOF. The
hallmark of the VSD in TOF is its unrestrictive nature
causing equalization of the right and left ventricular
pressures irrespective of the degree of pulmonary stenosis.
Very rarely, however, the VSD can become restrictive.
The mechanism of the restriction is best recognized
echocardiographically. The most common mechanism
is the presence of accessory tricuspid valve tissue or the
normal tricuspid valve, prolapsing through the VSD, which
narrows the interventricular communication.

Pulmonary Stenosis181185
RVOT narrowing in a case of TOF is a consequence of
the anterior and cephalad deviation of the infundibular
septum. The hallmark in TOF is infundibular stenosis
with variable degree of valvular stenosis (Figs 72.97
and 72.98). Thus, patients with large VSD and isolated
valvular stenosis should not be classified under TOF.
Anterocephalad deviation of the outlet septum is
seen best echocardiographically in the subcostal
sagittal, coronal views and PSAX views at the level of
the great arteries. In patients of TOF and pulmonary
atresia, the deviation is so extreme so as to produce

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1635

Fig. 72.92: Two-dimensional echocardiography. Parasternal shortaxis view in a 3-year-old child with tetralogy of Fallot and doubly
committed ventricular septal defect showing the point of continuity
between aortic and pulmonary valves (arrow). (Ao: Aorta; PA:
Pulmonary artery; RV: Right ventricle).

Fig. 72.93: Two-dimensional echocardiography. Parasternal

short-axis view in a patient of tetralogy of Fallot with color flow
mapping showing doubly committed ventricular septal defect. (Ao:
Aorta; PA: Pulmonary artery; RV: Right ventricle).

Fig. 72.94: Two-dimensional echocardiography. Modified parasternal short-axis view in a case of tetralogy of Fallot showing the
additional muscular ventricular septal defect (VSD) tract (arrow)
with left right shunt. (LV: Left ventricle; RV: Right ventricle).

Fig. 72.95: Tetralogy of FallotHoffmans variant. Two-dimensional

transthoracic parasternal long-axis view with color comparison in
a case of tetralogy of Fallot VSD getting partially restricted by the
septal leaflet of the tricuspid valve (arrow) leading to suprasystemic
right ventricle (RV) pressures with turbulent right-to-left shunt. (Ao:
Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).

1636

Section 6: Congenital Heart Disease

Fig. 72.96: Tetralogy of FallotHoffmans variant. Parasternal

short-axis view with color compare showing large ventricular
septal defect getting partially restricted by tricuspid valve leaflet (arrow) with severe infundibular pulmonary stenosis (arrow).
(Ao: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle;
RVOT: Right ventricular outflow tract).

Fig. 72.97: Two-dimensional echocardiography. Parasternal

short-axis view with color compare showing severe infundibular
pulmonary stenosis (arrow) in a patient of TOF. (Ao: Aorta; PA:
Pulmonary artery; RV: Right ventricle).

Figs 72.98A and B: Two-dimensional echocardiography in subcostal paracoronal view with color compare showing severe
infundibular pulmonary stenosis (arrow) with turbulent flow in
right ventricular outflow tract (RVOT), in a patient with tetralogy of
Fallot. (PA: Pulmonary artery; RV: Right ventricle).

Fig. 72.99: Two-dimensional echocardiography in parasternal

short-axis view showing short segment pulmonary atresia (arrow)
in a patient of TOF. Branch pulmonary arteries are confluent. (Ao:
Aorta; LPA: Left pulmonary artery; RPA: Right pulmonary artery;
RV: Right ventricle).

atresia (Fig. 72.99). Progression of the obstruction occurs

due to (a) failure of the subpulmonary infundibulum to
grow with increase in somatic growth, (b) progressive
acquired infundibular septal hypertrophy, and (c)
progressive acquired RV free wall hypertrophy. All these
features can be documented echocardiographically.
Pulmonary valve is bicuspid in 51% of cases of TOF.2
Valvular stenosis is almost always present in TOF and is

demonstrable echocardiographically as doming of the

pulmonary valve. It is important to measure the annular
size in all cases and compare to normal expected for the
patient size (Fig. 72.100). Normal pulmonary valve annulus
has surgical implications, because the surgeon can correct
the abnormality without using a transannular patch. The
annular size is best measured in PSAX views or anteriorly
tilted long-axis view.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Fig. 72.100: Two-dimensional echocardiography. Parasternal

short-axis view showing the measurement of the pulmonary
annulus from the hinge points (+) and RCA. (Ao: Aorta; PA: Pulmonary artery; RCA: Right coronary artery (arrow); RV: Right ventricle).

1637

Fig. 72.101: Two-dimensional echocardiography. Modified parasternal short-axis view with color compare in a patient with
tetralogy of Fallot showing absence of pulmonary valve (arrow)
with hypoplastic pulmonary artery annulus and free pulmonary
regurgitation (on color mapping) across the pulmonary annulus.
(PA: Pulmonary artery; RV: Right ventricle).

arteries may be dilated to variable degrees. The physiology

is essentially same as that of TOF. The RV obstruction is
usually at the level of pulmonary annulus; associated
infundibular stenosis may sometimes be present. Very
rarely, there may be an absence of pulmonary valve in the
absence of VSD.

The Pulmonary Arteries

(Figs 72.102 to 72.105)

Fig. 72.102: Two-dimensional echocardiography in high parasternal short-axis view showing a narrow origin (arrow) of left pulmonary artery in a case of TOF. (Ao: Aorta; LPA: Left pulmonary artery; MPA: Main pulmonary artery).

Tetralogy of Fallot with Absent

Pulmonary Valve (Fig. 72.101)
This is a rare variant of TOF seen in 2% of cases. The
pulmonary valve is absent and replaced by nubs of
fibrous tissue, which is best seen echocardiographically in
short-axis views as bright echoes. This is associated with
significant pulmonary regurgitation clearly seen on color
flow mapping. The main/right/left proximal pulmonary

Echocardiographically, the pulmonary arteries need to

be visualized in multiple views. The paracoronal view not
only identifies the RVOT, but also well visualizes the right
PA. The subcostal coronal view shows the entire length of
the right PA from its origin to its branching at the hilum.
The PSAX view shows the pulmonary trunk, confluence,
and the origins of the right and left pulmonary arteries and
a considerable length of the right PA. The left PA, which
is most difficult to visualize, is occasionally seen in its
entire length from the high left parasternal view and the
suprasternal oblique long-axis view with anterior tilt. The
suprasternal short-axis view opens the entire length of
the right PA. Systematic delineation of the PA anatomy is
crucial in the management of TOF. The size of pulmonary
arteries is measured at the hila, that is, before the first
division. The optimum size of the pulmonary arteries may
be seen from the standardized charts for the weight of the
baby. Various ratios used for this include: Mc Goons ratio,

1638

Section 6: Congenital Heart Disease

Figs 72.103A and B: Two-dimensional echocardiography with color compare in high parasternal short-axis view showing confluent
branch pulmonary arteries with diffuse narrowing in left pulmonary artery (LPA, arrow). (MPA: Main pulmonary artery; RPA: Right
pulmonary artery).

Fig. 72.104: Two-dimensional echocardiography in suprasternal

short-axis view with anterior tilt showing both the branches
of pulmonary artery. (LPA: Left pulmonary artery; RPA: Right
pulmonary artery).

Fig. 72.105: Two-dimensional echocardiography in suprasternal

short-axis view showing the right pulmonary artery and the adjacent structures. (AO: Aorta; Innom: Innominate vein; LUPV: Left
upper pulmonary vein; LA: Left atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena cava).

wherein the ratio of the sum of the size of branch pulmonary

arteries to the descending aorta is measured. This should
exceed 1.5 for optimum outcome. The pulmonary arteries
in TOF frequently show abnormalities of size, confluence,
or obstruction. The most important step is to determine
whether the pulmonary arteries are confluent. Once this
is done, the individual pulmonary arteries are scanned to
look for narrowing. Narrowing can be of two varieties: (a)
discrete stenosis and (b) diffuse hypoplasia. The common
sites for discrete stenosis are (a) the point of bifurcation
of the main PA, thus involving the origin of the right

and left PA, (b) the site of insertion of the patent ductus
arteriosus into the left PA, and (c) supravalvular narrowing
of the main PA. Diffuse hypoplasia can involve the left or
right pulmonary arteries or both. Multiple, peripheral PA
narrowing within the lung parenchyma is very rare and
cannot be detected by echocardiographic scans.

Aortic Arch
The suprasternal view is used to differentiate left and right
aortic arches. Right aortic arch is seen in 25% of patients

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

with TOF (Fig. 72.106). Two methods are used for their
differentiation: (a) In the suprasternal short-axis view,
the transducer is tilted posteriorly. In right aortic arch,
the descending aorta mostly falls to the right and in left
aortic arch the descending aorta mostly falls to the left.
(b) The branching pattern of the head and neck vessels is
most important. In left aortic arch, the normal branching
pattern of the first branch of the aortic arch (innominate
artery) can be seen dividing into two branches to the
right. The opposite is true in patients with right aortic arch
with mirror image branching. The fallacy of the second
method is when patients of right arch have an aberrant
left subclavian (3%) artery and vice versa (<1%). (c) The

Fig. 72.106: Two-dimensional echocardiogram in suprasternal

view showing right aortic arch in a patient with tetralogy of Fallot
showing the division of the first branch toward left side. (LSA: Left
subclavian artery; LICA: Left internal carotid artery).

1639

relationship of trachea and the ascending aorta in this view

also helps in deciding the side of the arch. The ascending
aorta is to the left of the trachea in left-sided and on the
right side in right-sided aortic arch.

Patent Arterial Duct and

Aortopulmonary Collaterals
(Figs 72.107A and B)
Patent arterial duct is easily defined with color Doppler
flow as continuous flow in the left PA at its origin. The
duct originates from its usual position in patients with
TOF with pulmonary stenosis. In patients with pulmonary
atresia, however, the duct is vertical, that is, it arises from
the under surface of the arch and descends down, and
then takes a tortuous S-shaped course before joining the
left PA. Echocardiographically, patent arterial ducts in
TOF with pulmonary stenosis are best seen from the high
parasternal views (ductal view), whereas the vertical ducts
are best seen in the suprasternal long-axis views.
Right-sided duct can rarely be seen in patients with
TOF. In such cases, the continuous flow on color flow
mapping is seen in the right PA.
Aortopulmonary collaterals are most often present in
cases of TOF with pulmonary atresia, although they can be
present in patients with TOF with pulmonary stenosis. The
suprasternal views show the collaterals arising from aortic
arch or descending aorta on color Doppler mapping using
a low scale as continuous turbulant signal. The presence
of collaterals can also be suspected by profiling the
descending thoracic aorta in its long axis from subcostal

Figs 72.107A and B: Two-dimensional echocardiography. (A) Suprasternal long-axis view showing the origin of a collateral from the
ductal area (arrow); (B) Subcostal sagittal view showing collaterals arising from the descending aorta (arrow). (Des Ao: Descending
aorta; TA: Transverse arch).

1640

Section 6: Congenital Heart Disease

window on color flow mapping with a low scale. It is

usually not possible to further delineate the course of these
collaterals in the lung parenchyma by echocardiography.

Coronary Artery Anomalies186

Surgically important coronary artery anomalies are seen in
3 to 15% of patients with TOF. The PSAX view is the best to
detect these anomalies. Because of the clockwise rotation
of the aortic root, the right coronary sinus occupies a more
leftward and anterior location, and left sinus becomes
more posterior. The origin of the right and left coronary
arteries from these sinuses can be seen clearly in PSAX
view. Thus, in patients with normal origin of coronary
arteries, the left main stem is seen to course from a more
left/posterior location and the right main stem from a more
leftward position. The most common anomalies seen are:
(a) origin of the LAD coronary artery from the RCA. In the
PSAX view, this is seen as a large branch arising from the
RCA and coursing leftward and anterior toward the RVOT.
A large coronal branch can also be mistaken for the LAD.
However, in such cases the LAD is seen arising from the
left main branch. (b) Single coronary artery arises from
the right sinus or left sinus, and (c) dual LADs arise from
the left and the right sinuses. In this condition, it may be
difficult echocardiographically to differentiate the branch
arising from the right main stem from a large coronal
branch.

Echocardiographic Measurements in
Tetralogy of Fallot1,2,187

left to right (pink tetralogy). However, the major advantage

of the color Doppler is in the evaluation of small additional
muscular VSD. In nearly all patients of TOF, the VSD does
not reveal any gradients because of equal right and left
ventricular pressures. The only exception is in patients
in whom the VSD becomes restrictive. The gradients
then will be determined by the severity of the pulmonary
stenosis. If the pulmonary stenosis is severe, then there
would be reverse gradients (from RV to LV) because of
suprasystemic RV pressures.
Evaluation of pulmonary stenosis (Fig. 72.108): It is difficult
to differentiate by Doppler, the relative contribution of
the infundibular, valvular, supravalvular, and peripheral
pulmonary component of the obstruction. It is also
difficult to align the Doppler signal in order to obtain
the total gradient across the RVOT and pulmonary valve
because of their different spatial orientation. Subcostal
sagittal view of RV outflow is the only view that opens both
the RVOT and pulmonary valve, and in some patients one
may be able to align the Doppler signal accurately. It is
important to determine whether the pulmonary circulation
is protected from the high RV pressures.
Color flow mapping shows that the turbulence starts at
the subvalvular level and continues to the PA level. Again,
color flow fails to differentiate the relative contribution of
the various levels of obstruction in TOF.
Poor echocardiographic window, very severe infundibular obstruction, and severe polycythemia are some
causes of inability to document antegrade flow across the
RV outflow by color or pulsed Doppler accurately.

The following measurements are routinely performed and

compared to a nomogram based on weight of the patient.
The pulmonary annulusThis is measured at the site
of insertion of the pulmonary valve leaflets.
The right and left PA at the hilum just before their
first branchingThe suprasternal short-axis view
is selected for measuring the right PA and the high
parasternal or the oblique suprasternal long-axis views
for the left PA.

The Utility of Doppler in Evaluation of

Patients of Tetralogy of Fallot
Evaluation of ventricular septal defect: Ventricular septal
defect (VSD) is usually seen to shunt right to left. If the
pulmonary stenosis is mild to moderate, the VSD shunts

Fig. 72.108: Continuous wave Doppler signal showing right ventricular outflow gradient in a case of tetralogy of Fallot (TOF). Note
the sickle-shaped signal of the infundibulum stenosis (arrow).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Patent arterial duct and aortopulmonary collaterals:

These are seen as continuous signals in the PA on color
flow mapping. Pulsed Doppler shows continuous high
velocity signals on the spectral waveform. The PDA is seen
opening at the junction of the main PA, left or rarely right
PA. Aortopulmonary collaterals usually open more distally
beyond the hilum into the PA.
Aortic regurgitation (Fig. 72.109): It is common to see mild
aortic regurgitation in TOF, especially in adolescents,
adults, and patients who had undergone shunt surgery
or have had endocarditis. Associated anomalies include
presence of ASD in 38% and LSVC in 9% of cases of
TOF. One should keenly look for pulmonary venous
anomalies particularly on 2D echocardiography, because
with decreased pulmonary blood flow conditions, the
pulmonary veins may not be well visualized on color flow
mapping. Aortopulmonary window (APW) (Fig. 72.110),
LVOT obstruction, mitral valve anomalies (Fig. 72.111),
have been reported, but these left-sided lesions are very
rare in TOF patients.

1641

example, anomalous origin of one of the PAS from the

ascending aorta may also need additional imaging
modalities.
In older children, adolescents, and adults with TOF,
inability to delineate anatomical details is an indication
for using other imaging modalities like angiography
MRI/CT. They are also indicated in evaluation of patients
for complete repair of TOF following shunt surgery for
accurate delineation of the PA anatomy and determination
of PA pressure. It is difficult to predict the PA pressure
accurately by Doppler even if the signals are good.
In the immediate postoperative state, cardiac
catheterization is indicated for accurate delineation of the
severity and extent of peripheral PA stenosis, residual VSD,
and for coil occlusion of aortopulmonary collaterals.

Postoperative Evaluation of
Tetralogy of Fallot

In neonates and infants, the suspicion of major

aortopulmonary collaterals (MAPCAs) and discontinuous
pulmonary arteries forms the most important indication
for cardiac catheterization. Rarer variations of TOF, for

The postoperative evaluation of a case of TOF includes

evaluation for the residual lesions such as residual RVOT
obstruction (Fig. 72.112), branch PA stenosis, and residual
VSD (Fig. 72.113). Branch PA stenosis is particularly
common at the site of ductal insertion or at the site
of previous BT shunt. It is important to take tricuspid
regurgitation jet velocities to determine the RV pressure,
as it would reflect any obstructive component in both
RVOT and branch pulmonary arteries.

Fig. 72.109: Two-dimensional echocardiography in parasternal

long-axis view with color flow mapping showing moderate aortic
regurgitation and dilated aortic root in a case of grown up tetralogy
of Fallot. (AO: Aorta; LV: Left ventricle; RV: Right ventricle). (*),
perimembranous ventricular septal defect (VSD).

Fig. 72.110: Two-dimensional echocardiography in parasternal

short-axis view at the level of the aorta, profiling a large aortopulmonary window (arrow) in a neonate with pulmonary atresia
and ventricular septal defect. (Ao: Aorta; PA: Pulmonary artery;
valvular pulmonary atresia).

Indications for Cardiac Catheterization in

Patients with Tetralogy of Fallot

1642

Section 6: Congenital Heart Disease

Fig. 72.111: Two-dimensional echocardiography in parasternal

long-axis view in a case of tetralogy of Fallot showing a large ventricular septal defect with overriding aorta. Mitral valve is thickened
and doming is present consistent with mitral stenosis. Also note
the presence of short mitral chordae. (Ao: Aorta; LA: Left atrium;
LV: Left ventricle; RV: Right ventricle).

Fig. 72.112: Two-dimensional echocardiography with color flow

mapping in a patient with tetralogy of Fallot following surgery.
Parasternal long-axis view with anterior tilt shows significant
turbulence in right ventricular outflow tract. (LV: Left ventricle; PA:
Pulmonary artery; RV: Right ventricle).

Figs 72.113A and B: Two-dimensional echocardiography in parasternal long-axis view with color flow mapping in cases of tetralogy of
Fallot after total correction showing significant residual ventricular septal defect (VSD) from the upper end of the VSD patch (VSD patch
marked by the arrow). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).

Small residual VSD may be seen in the region of the

patch at the site where the sutures are placed far apart to
avoid damage to the AV node and the conduction system.
As endothelium grows over the patch, these shunts usually
disappear generally by the third month, and rarely they
may be significant (Figs 72.113A and B). Other types of
shunt that may be seen in postoperative patients include
small insignificant coronary artery fistulas. Significant
pulmonary regurgitation may be present in a subset of
patients where a transannular patch has been sutured.
This leads to progressive RV dilatation and in some cases,
aneurysm of RVOT (Figs 72.114 and 72.115). This needs

to be carefully evaluated in the follow-up of this subset

of patients. Another most important aspect to evaluate is
the RV function, both systolic and diastolic (Figs 72.116 to
72.119), which needs to be monitored at each follow-up
visit. The presence of pulmonary regurgitation leads to
progressive dilatation of the right sided structures: RVOT, right
ventricle and right atrium, hence one needs to look keenly at
the RV function parameters (Figs 72.114 and 72.115).
Systolic function may be measured by TAPSE (tricuspid
annular systolic excursion (Fig. 72.117), RV fractional area
change (Figs 72.118A and B) and systolic indices on tissue
Doppler (Fig. 72.116).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1643

Fig. 72.114: Two-dimensional echocardiography. Apical fourchamber view in a patient with tetralogy of Fallot with transannular
patch showing dilatation of the right atrial (RA) and right ventricular (RV) cavity. (LA: Left atrium; LV: Left ventricle).

Fig. 72.115: Two-dimensional echocardiography with parasternal

short-axis view in an operated patient with tetralogy of Fallot. A
transannular patch is placed across the right ventricular outflow
tract. There is dilatation of the right ventricular outflow tract, right.
(PA: Pulmonary artery; RVOT: Right ventricular outflow tract; RA:
Right atrium; RV: Right ventricle).

Fig. 72.116: Tissue Doppler velocity of the annulus of the tricuspid

valve showing normal systolic velocity.

Fig. 72.117: TAPSE: tricuspid annular systolic excursion is a

measurement of the systolic function of the right ventricle. The
M-mode cursor is placed at the annular attachment of the anterior
septal leaflet.

Evaluation of the diastolic dysfunction must be

done in any postoperative patient of TOF as the diastolic
dysfunction may be present even in the absence of
systolic dysfunction. Various parameters to assess the
right ventricular diastolic dysfunction include: (A) Inflow
parameters: Diastolic dysfunction can be measured by
measuring isovolumic relaxation time, deceleration time,
E and A wave velocities and EA ratio; (B) Inferior vena cava
(IVC) : Normally there should be more than 50% collapse
of IVC with inspiration. With diastolic dysfunction IVC
becomes dilated with decreased respiratory variation.

With diastolic dysfunction there is resistance to right

ventricular filling which exceeds pulmonary vascular
resistance, this leads to transmission of the transtricuspid
atrial flow to pulmonary artery, superior, inferior vena
cava and hepatic veins; (C) Flow reversal in hepatic veins
and SVC can be documented with Doppler signal.Reversal
in SVC is best interrogated from subxiphoid sagittal view
or suprasternal short-axis view; (D) Transmitted a wave
in main pulmonary artery: In parasternal short-axis view
the Doppler cursor is placed in main pulmonary artery.
The presence of a wave following p wave on ECG and

1644

Section 6: Congenital Heart Disease

Figs 72.118A and B: Right ventricle (RV) fractional area and fractional area change. Measuring the right ventricular area in diastole
(RVD) (A) and subtracting from it the right ventricular area in systole (RVS); (B) The difference divided by the right ventricle area in
diastole gives the fractional area change, a measure of the right ventricular systolic function.

Figs 72.119A and B: Right ventricular diastolic dysfunction. Showing the profiling of inferior vena cava (IVC) from subcostal window:
(A) IVC is dilated; (B) M-mode cursor across the IVC shows no respiratory phasic variation.

present during all phases of respiration characterizes

atrial flow transmission to pulmonary artery; (E) Tissue
Doppler diastolic parameters with cursor placed across
the tricuspid valve.

DOUBLE OUTLET RIGHT VENTRICLE

Double outlet right ventricle (DORV) encompasses
features of various entities, ranging from simple VSD to
TOF to transposition of the great arteries (TGA). This is a
rare anomaly. Its frequency is approximately 0.09 cases
per 1,000 births, and it represents 1 to 1.5% of patients with

CHD. Like the varied spectrum of DORV, the definition is

also variable.188,189 The commonest definition by Neufield
et al.188 is as follows: Both great arteries and arterial trunks
arise exclusively from the morphological RV, neither
semilunar valve is in fibrous continuity with either AV
valve, and usually, a VSD is present and represents the
only outlet from the LV. Pulmonary valve or subpulmonary
stenosis may be present or absent. Echocardiography has
a very important role in delineation of cardiac anatomy to
enable planning of surgical strategy for DORV. DORV often
occurs in association with a variety of complex situations
that include atresia of one of the atrioventricular valves and

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1645

Classification and Terminology190195

The determinants of individual subtypes of DORV are
based upon the location of the VSD and great artery
relationship.

Location of the Ventricular Septal Defect

in Relation to the Great Arteries
The location of the VSD determines whether it can be
routed to either of the two great vessels.

Subaortic (Fig. 72.120)

Fig. 72.120: A 5-year-old case of double outlet right ventricle
(DORV) with ventricular septal defect (VSD) and pulmonary
stenosis. Two-dimensional echocardiography in parasternal longaxis view shows the mitral aortic large subaortic VSD, more than
50% aortic over ride discontinuity. (Ao: Aorta; LV: Left ventricle;
RV: Right ventricle).

discordant atrioventricular connection. For this section,

we will confine ourselves to DORV in association with two
functioning atrioventricular valves and atrioventricular
concordance.

Definition
The diagnosis of DORV (Fig. 72.120) requires the following
criteria to be fulfilled.188193
DORV infers that both great arteries arise from the RV
as the name implies. There is a spectrum of the degree of
override of one great vessel and herein lies the controversy
as to when to classify an origin as double outlet versus
concordant ventriculoarterial connection. Some authors
follow the 50% rule, which means that if a vessel is 50% or
more committed to a chamber, it is considered originating
from that chamber. However, it is difficult to determine
when exactly an override is 50% or more. To avoid this
difficulty, some authors diagnose DORV if the override is
90% or more.
Some consider absence of fibrous continuity between
the posterior semilunar valve and the mitral valve
(Fig. 72.120A). According to some, this is not mandatory.
One can have fibrous continuity of the mitral and one
semilunar valve with criteria of origin of great vessel being
fulfilled. We believe that both features are important for
the diagnosis of DORV, that is, more than 50% override and
mitral aortic discontinuity.

The VSD is located just below the aortic conus and thus
can be routed to aorta. In this case, saturations are better
because saturated blood from LV is directed to the aorta. In
the event that the great artery relationship is normal with
no pulmonary stenosis, the condition may be corrected
like a perimembranous VSD. In presence of pulmonary
stenosis, it can be repaired like a TOF. In the majority of
cases in this group great vessels are normally related. In
rare cases, the aorta may be L-malposed.

Subpulmonic (Fig. 72.121)

The VSD is located just below the subpulmonic conus
and thus can be routed to PA. In the majority of cases in
this group the aorta is malposed (i.e. anterior to the PA).
Pulmonary arteries are often not protected by stenosis of
the valve or subvalvular region. These patients present like
classical transposition-Taussing Bing Anomaly. However,
pulmonic stenosis may occur if the conal septum between
the aortic and pulmonary roots is deviated posteriorly
and/or to the left.

Doubly committed
Here, the conal septum between the aortic and pulmonary
outflow tract is deficient. As a result, the VSD is located
below both outflow tracts and can be routed to either great
vessel.

Remote
Remote VSDs are typically located in the inlet septum
(Fig. 72.122). The tricuspid valve tensor apparatus comes
in the path between the VSD and either of the outflow
tracts. Overriding/straddling of tricuspid valve can occur

1646

Section 6: Congenital Heart Disease

Fig. 72.121: Two-dimensional echocardiography. Subcostal view

with anterior tilt showing both great vessels arising from right
ventricle (RV). Subpulmonary ventricular septal defect (VSD)
is routable to the pulmonary artery (arrow). (Ao: Aorta; LV: Left
ventricle; PA: Pulmonary artery; RV: Right ventricle).

Fig. 72.122: Two-dimensional echocardiography. Apical fourchamber view in a case of double outlet right ventricle (DORV)
showing large inlet ventricular septal defect (VSD). (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Figs 72.123A and B: Two-dimensional echocardiography in a subxiphoid sagittal view in two cases, showing a ventricular septal defect
located in the inlet septum (arrow) and not related to either great artery origin. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
RV": Right ventricle).

in this type of VSD. This group also includes muscular VSDs

that cannot be routed to either great vessel (Figs 72.123A
and B).

Great Artery Relationship to one Another

The following four possibilities exist in DORV:
Normal great artery relation
Side-by-side great artery relationship

D-malposition of the aorta

L-malposition of the aorta.
Various combinations with the above give rise to 16
individual subtypes with 9 being commonly reported.
Additional descriptive features should include the state
of the outflow tracts to each great artery and associated
anomalies. The echocardiographic description should
include a statement on whether or not a two-ventricle
repair is feasible.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Establishing a Diagnosis of
Double Outlet Right Ventricle by
Echocardiography194
The following needs to be specifically evaluated:
VSD
Subpulmonary outflow tract
Subaortic outflow tract and arch
Coronary arteries.

Subaortic Ventricular Septal Defect

In the subcostal sagittal view, the aorta will be seen to arise
predominantly from RV. In this view, the tricuspid valve is
seen to form the posteroinferior margin. Its attachments
can be variable. In some cases, some chordae can gain
attachment to the outlet septum. The subpulmonary
outflow tract will not be seen in this view as it is more
anterior. This view also shows the size of the VSD (discussed
later). In the subcostal coronal view, both great vessels
can be seen arising from the RV, with the subpulmonary
outflow tract anterior and to the left of the aortic outflow
like in TOF.
The PLAX and apical five-chamber views also clearly
demonstrate the degree of override of aorta like in tetralogy
as well as aortic mitral discontinuity.

1647

pulmonary annulus is commonly associated, and to

a varying degree, hypoplasia of the pulmonary trunk.
Echocardiographically, malalignment of outlet septum is
recognized by subcostal sagittal or coronal views. Apical
five-chamber and PLAX views show malalignment of outlet
septum very well in the group of patients with transposed
great vessels. By Doppler echocardiography, the severity
of pulmonary stenosis is best assessed in the subcostal
coronal view in the patients with normally related vessels
and in the apical views in those with d-malposed great
vessels.

Aortic Outflow Obstruction

Stenosis of the subaortic region may result from anterior
malalignment of the conal septum. This is seen in the
group of patients with malposed great vessels. They rarely
produce any Doppler gradients. Typically, the aortic
annulus, ascending and transverse arches are smaller than
normal, and coarctation is a common association. The
following measurements are important: aortic annulus,
ascending aorta, transverse arch, distal transverse arch,
aortic isthmus, and the narrowest dimension of the
coarctation site if present.

Subpulmonary Ventricular
Septal Defect (TaussigBing Anomaly)
In this condition, the aorta is anterior (malposed) in
majority of cases. In the subcostal sagittal view, the great
vessel overriding the septum is the PA. The aorta will not be
seen or will come under view in a more rightward sweep.
In the coronal section, both great vessels will be seen
having a parallel origin with the aorta to the right and PA
to the left. Pulmonary valve and mitral valve discontinuity
will also be obvious.

Stenosis of the Pulmonary Outflow

The commonest cause of obstruction is malalignment
of outlet septum. In normally related great vessels the
outlet septum is anteriorly malaligned and in transposed
great vessels the outlet septum is posteriorly malaligned,
resulting in subpulmonary stenosis. Hypoplasia of

Fig. 72.124: Two-dimensional echocardiogram from subcostal

short-axis view with color compare showing restricted subaortic
ventricular septal defect (VSD) in a case of double outlet right ventricle (DORV). VSD size is smaller (small white arrows) than the
aortic annulus (block yellow arrows) and routing will require VSD
enlargement. Tricuspid valve () does not come in the pathway
from the left ventricle to aorta.

1648

Section 6: Congenital Heart Disease

Table 72.14: The Complete Checklist for Preoperative Assessment of Double Outlet Right Ventricle

Visceral and atrial situs, sequential chamber relationship

Systemic and pulmonary veins, atria and atrial septum
Atrioventricular valve morphology, attachments of the valve tissue and function
The ventricles: size and function
The ventricular septum: location of ventricular septal defects, size of the ventricular septal defect as compared to the aortic annulus,
routability of ventricular septal defect, presence or absence of atrioventricular valve tissue in the way between the ventricular
septal defect and the great artery (at the site of the prospective patch), presence or absence of conal tissue that may obstruct the
prospective patch (which may therefore require resection)
The conotruncus: This includes the outflow tracts that lead to the great vessels and the great vessel origins including the semilunar
valves; the presence or absence of obstruction in either of the outflow tracts, the nature of obstruction if present, the severity of
obstruction; and the sizes of the aortic and pulmonary valve annuli
The status of the branch pulmonary arteries
The presence or absence of patent ductus arteriosus
The aortic arch: side, branching, and the presence or absence of coarctation
Feasibility of a two-ventricle repair (with reasons)
Likelihood for requirement of a conduit
Coronary artery anatomy

Restriction of the Ventricular

Septal Defect (Fig. 72.124)
Mild restriction in size of VSD is quite common but rarely
severe to produce a Doppler gradient across the VSD
(between left and RV). VSD is defined as small when its
size is smaller than the aortic annulus.

Remote Ventricular Septal Defect

One should suspect a remote VSD if while visualizing the
great vessels, the VSD is not seen. Inlet VSDs are best seen
in the subcostal views in a more posterior plane along with
the tricuspid valve. In the apical 4C view, the VSD is seen
in the plane that visualizes the mitral and tricuspid valves
(crux). In this view, as one sweeps anteriorly toward the
great vessels, the VSD disappears from view. Overriding
and straddling of tricuspid valve can be well visualized in
this view.

Role of Echocardiography in Planning

Surgical Strategy (Table 72.14)195198
The aim of evaluation is to ensure that a two-ventricle repair
is carried out if and when possible. Both the long-term
survival and functional status after two-ventricle repair
are superior to patients who undergo the single ventricle
repair.196198 Two good-sized ventricles: Hypoplasia of
either of the ventricles clearly rules out a two-ventricle
repair (Fig. 72.130). In borderline situations, the size of the

mitral or tricuspid valve annulus should be determined

and compared to normal. A size of < 2 standard deviations
of either of the annuli merits concern.
Straddling of either of the AV valves (Fig. 72.125).
Typically, straddling of the tricuspid valve is associated
with an inlet defect and/or hypoplasia of the RV.
Straddling of the mitral valve may occur in patients
with outlet VSD
Confluent adequate-sized pulmonary arteries: Measurements of pulmonary annulus, pulmonary trunk, and
branch pulmonary arteries should be made like in TOF
The VSD should be suitably located for intraventricular
re-routing. If pulmonic stenosis is present, the VSD will
be routable to the aortic root without AV valve tissue in
the way. In the absence of pulmonic stenosis, the VSD
should be routable to either the aorta or the PA. This is
where echocardiography provides critical information
that is often not available even on angiography
Atrioventricular valve tissue in the way between VSD
and the aorta (Figs 72.126 and 72.127; or PA in the case
of a TaussigBing anomaly). Careful subxiphoid and
PSAX sweeps help in evaluating the location of the
prospective baffle and whether the AV valves will come
in the way
Inlet VSD is not an absolute contraindication for twoventricle repair. It is possible to route remotely located
VSDs with tricuspid valve tissue in the way using
innovative surgical methods such as the multiple
patch technique.
If the patient is a candidate for two-ventricle repair,
additional specific issues need to be addressed. They include:

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Fig. 72.125: Two-dimensional echocardiography. Subcostal sagittal view in a case of situs inversus with mesocardia showing a
large ventricular septal defect with hypoplasia of the right-sided
left ventricle. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
RV: Right ventricle).

1649

Fig. 72.126: Two-dimensional echocardiography in subcostal

coronal view with anterior tilt in a case of double outlet right
ventricle, ventricular septal defect (VSD), and pulmonary stenosis.
Note the presence of tricuspid valve tissue in the pathway from left
ventricle (LV) to aorta (arrow). (Ao: Aorta; LV: Left ventricle; RV:
Right ventricle).

Fig. 72.127: Two-dimensional echocardiogram. Subcostal short-axis view with anterior tilt and color compare in a case of double outlet
right ventricle (DORV) with a large subaortic ventricular septal defect (VSD) and pulmonary stenosis. Presence of tricuspid valve (arrow) in the pathway from left ventricle (LV) to aorta resulting in restriction of the VSD. (Ao: Aorta; LV: Left ventricle; RV: Right ventricle).

Origin of LAD coronary artery in the TOF type of

DORV (with subaortic VSD and normally related great
arteries). If the LAD coronary artery is seen to cross in
front of the RVOT, a conduit or a modified technique
for relieving RV obstruction may be required.
The origin of coronary arteries in the TaussigBing
anomaly prior to an arterial switch operation.
The incidence of atypical coronary artery origin is
higher in the TausigBing anomaly as compared with
classical transposition
Presence or absence of additional muscular VSDs that
may require to be closed at the time of surgery should
be looked for

Other associated anomalies that may need to be

addressed need proper evaluation.
The postoperative evaluation of such cases is essentially the same as that described under TOF repair. Postoperative evaluation involves:
Checking for residual lesions: residual VSD, additional
VSD
Checking for pulmonary stenosis and RVOT gradient
(or RVPA conduit gradient; Fig. 72.128). The gradient
across RVOT may be aligned in the subcostal sagittal
view with anterior tilt and in paracoronal and PSAX
views. Tricuspid regurgitation velocity may give a good
indication of the presence of distal obstruction

1650

Section 6: Congenital Heart Disease

Evaluation of branch pulmonary arteries

Looking for RV systolic and diastolic function (as
explained earlier)
Looking for the presence of LVOT gradients (generally
across the VSD)
In case of BT shunt, evaluate the patency of the BT
shunt (Figs 72.129 and 72.130) and look for distortion
of the pulmonary arteries
In case of a PA band, estimate the band gradients to
decide the adequacy of the band and PA pressures.
One should also look for migration of PA band and
distortion of pulmonary artery branches.

Fig. 72.128: Two-dimensional echocardiogram in parasternal

short-axis view with color flow mapping in an operated patient with
pulmonary atresia and ventricular septal defect (VSD) showing
conduit obstruction (arrow). (Ao: Aorta; RV: Right ventricle).

TRUNCUS ARTERIOSUS
(FIGS 72.128, 72.131 TO 72.134)
A common arterial trunk arising from the base of the
heart, and giving origin to aorta, pulmonary arteries, and
coronaries is referred to as truncus arteriosus. A single
semilunar valve is found in truncus arteriosus, and this
valve differentiates truncus arteriosus from aortic and
pulmonary valve atresia, conditions in which a single
arterial vessel also receives the entire output of both
ventricles but in which a second atretic semilunar valve
is present. Truncus arteriosus is usually associated with a
large VSD resulting from absent or deficient outlet septum.
In 25% of cases, the defect may extend to the membranous/
perimembranous area. Rarely, the VSD may be restrictive
or even absent. According to the initial classification given
by Collet and Edwards199 in 1948, truncus may be classified
as following:
Type I: Origin of a main pulmonary trunk from the
lateral aspect of the common trunk.
Type II: Left and right pulmonary arteries originate
separately from the common trunk. The origin is
close to each other, or from a common orifice,
usually located on the posterior aspect of the
common trunk.
Type III: Origin of only one PA from the common trunk,
the other PA can originate from the ductus
arteriosus or directly from ascending aorta
(MAPCA artery).
Type IV: It is defined not by the pattern of origin of
pulmonary arterial branch but rather by the
coexistence of an interrupted aortic arch or
aortic hypoplasia or preductal aortic coarctation.

Figs 72.129A and B: Two-dimensional echocardiography. Suprasternal short-axis view showing a patent left BT shunt (arrow) to left
pulmonary artery. (LPA: Left pulmonary artery); (B) Color Doppler flow mapping in the same patient showing flow through the left BT
shunt indicating its patency.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1651

Fig. 72.130: Two-dimensional echocardiography with color flow

mapping suprasternal short-axis view showing the right BT shunt
(arrow) to right pulmonary artery. (RPA: Right pulmonary artery).

Figs 72.131A to C: Two-dimensional echocardiography. (A) Subcostal coronal view with anterior tilt in a child with truncus arteriosus Type I, giving rise to aorta and pulmonary artery (arrow)
showing the presence of single outflow truncus (Tr) overriding the
ventricular septal defect (marked by star); (B) Parasternal long axis
view showing the presence of single outflow truncus (Tr) with main
pulmonary artery stump arising from the truncus ( arrow); (C) Parasternal short axis view with color flow mapping in the same patient
showing the common trunk (MPA) giving rise to both the pulmonary
arteries). (RV: Right ventricle; LV: Left ventricle; Tr: Single outflow
truncus; Ao : Aorta).

In these cases, a well-documented correlation

with 22q11 deletion syndrome is noted.
Van Praagh and van Praagh have proposed an
expanded classification system that also includes two
commonly associated abnormalities of the great arteries.

Type A signified the presence of VSD while type B signified

the absence of VSD. Their type A1 corresponds to Type I
of Collett and Edwards, and type A2 encompasses Types
II and III. Type A3 includes cases with absence of truncal
origin of one PA, with blood supply to that lung from the

1652

Section 6: Congenital Heart Disease

Fig. 72.132: Two-dimensional echocardiography. Parasternal

short-axis view from an infant with persistent truncus arteriosus
showing tricuspid truncal valve. (LA: Left atrium; RA: Right atrium;
T: Truncal valve).

Fig. 72.133: Two-dimensional echocardiography. Subcostal coronal

view with anterior tilt showing the origin of the main pulmonary artery
from the lateral aspect of the common trunk (arrow). There is flow
acceleration in the main pulmonary artery consistent with flow restriction. (PA: Main pulmonary artery trunk; TR: Truncus; Ao: Aorta).

Figs 72.134A and B: Two-dimensional echocardiography in high parasternal short-axis view with color compare in a case of persistent
truncus arteriosus type I with confluent branch pulmonary arteries. (LPA: Left pulmonary artery; RPA: Right pulmonary artery; T: Truncal
valve).

ductus arteriosus or from a collateral artery. Lastly, type

A4 is associated with underdevelopment of the aortic
arch, including tubular hypoplasia, discrete coarctation,
or complete interruption.200 Modified Van Praaghs
classification was accepted by Congenital Heart Surgery
Nomenclature Database Project 2000 and classified into
type A1-2, type A3( hemitruncus) and type A4. Dr Anderson
et al. proposed a simplified classification classifying truncus
into Aortic dominant type and pulmonary dominant type.

Echocardiography
(Figs 72.131 to 72.134)
Intracardiac anatomy usually reveals situs solitus and AV
concordance. Two balanced ventricles are usually present
and separated by a large VSD. Truncus arteriosus has
been described with tricuspid atresia, hypoplastic double
inlet ventricle, and a very rare form with discordant AV
connection.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Two-Dimensional Echocardiography201
Subcostal coronal view with anterior tilt shows a common
trunk arising from the heart, and overriding the VSD.
Subcostal paracoronal view can profile the pulmonary
artery arising from the trunk and bifurcating into right
pulmonary artery and left pulmonary artery from lateral
aspect of the trunk. Apical four-chamber view with
anterior tilt will profile the common trunk arising from the
heart and overriding the VSD. PLAX view is the best view
to profile truncal anatomy. This view shows the common
trunk arising from the heart committed to both ventricles
and overriding the large VSD. Slight posterior tilt from a
standard PLAX view shows the origin of main PA from the
posterolateral aspect of the common trunk. PSAX view at
the base of heart shows the truncal valve in cross section,
large outlet VSD, and pulmonary arteries as they arise

1653

from the trunk. Truncal valve can be tricuspid (6067%),

quadricuspid (2531%), or bicuspid (8%). Valve leaflets
are usually thickened. The truncal valve is in continuity
with the anterior mitral valve leaflet in the PLAX view. The
truncal valve may be normal, stenotic, and/or regurgitant.
Rarely, there can be absence of the pulmonary arteries.
From suprasternal view, the aortic arch anatomy
should be properly defined to ascertain the side of arch
(right aortic arch is reported in 2536% of cases), branching
pattern, and associated coarctation or arch interruption.

Doppler Imaging
Color flow Doppler mapping shows regurgitation or
stenosis of truncal valve and any stenosis at the origin
of pulmonary arteries. With continuous wave Doppler,
gradient across the truncal valve and pulmonary arteries
can be recorded.

PART 7: COMPLETE TRANSPOSITION OF GREAT ARTERIES

TRANSPOSITION OF GREAT VESSELS (TGA)

Anatomy

TGA means discordant ventriculoarterial connection,

that is, origin of great vessels from inappropriate
ventricles. TGA accounts for 57% of all congenital
cardiac malformations. Aorta arises from morphological
RV and pulmonary arteries from the morphological LV.
It is not based on the spatial interrelationship of the
great arteries.202204

Among the characteristic features generally recognized

on echocardiography is the parallel orientation of ventricular outflows (both the PA and the aorta) unlike normal
hearts where they cross (Figs 72.135A and B). The
pulmonary valve is not wedged as deeply between the two
atrioventricular valves as is the aortic valve in the normal
heart. The area of offsetting of atrioventricular valves and

Figs 72.135A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view with anterior tilt in a neonate
with complete transposition of great vessels (TGA) showing ventriculoarterial discordance and the presence of parallel great vessels.
There is acute posterior angulation of the pulmonary artery as it arises from the left ventricle. (LV: Left ventricle; RV: Right ventricle; Ao:
Aorta; PA: Pulmonary artery).

1654

Section 6: Congenital Heart Disease

perimembranous septum is less marked. LVOT is longer

than in a normal heart but not to the extent of AV canal
defect. In about 40 to 45% of cases there is an associated
VSD. The entire ventricular septum is a straight structure
rather than curved as in the normal heart.204,205
In the normal heart, aortic root is right and posterior to
pulmonary trunk with situs solitus and left and posterior
to pulmonary trunk with situs inversus. The most obvious
external abnormality is relation of great vessels with aorta
being generally anterior. The commonest relation is aortic
root right and anterior to pulmonary trunk (>80% of cases)
with situs solitus and left and anterior to pulmonary trunk
in situs inversus.202,203 Aortic valve is usually superior
to pulmonary trunk as it is supported by a muscular
infundibulum and the muscular infundibulum on the
left side is mostly absorbed leading to mitralpulmonary
continuity. However, conal anatomy can vary as a there
can be bilateral subarterial conus, subpulmonary conus
with absent subaortic conus, and rarely bilaterally absent
conus. Variations in great vessel relationship are reported
more commonly with a VSD, anterior-posteriorly related
great vessels, aortic root left and anterior, right and
posterior or left and posterior to pulmonary trunk.

Associated Lesions202-205
Nearly half of the patients with d-transposition of great
vessels have no other associated anomaly except a
persistent foramen ovale or ASD and PDA (Fig. 72.136).
VSD is the commonest associated anomaly (4045%);
combination of VSD and LVOT obstruction (pulmonary
stenosis) are observed in 10%, and an isolated LVOT
obstruction in approximately 5% of cases. Less commonly
encountered anomalies are AV valve abnormalities, aortic
obstructions, arch anomalies, and anomalies of systemic
and pulmonary venous connections.

Echocardiographic Evaluation
Two-Dimensional Echocardiography
The usual segmental analysis is essential as for any other
congenital cardiac anomaly. After defining the situs,
subcostal coronal and apical four-chamber views provide
the AV connection. Anterior tilt from subcostal coronal
and apical four-chamber views profiles first the posteriorly
placed great vessel that bifurcates into two, that is, PA arising
from LV with mitralpulmonary continuity (Fig. 72.135).
Further angulation profiles the anteriorly placed great
vessel with muscular infundibulum (subaortic conus),

Fig. 72.136: Two-dimensional echocardiography in subcostal

view in a case of complete transposition of great vessel (TGA) with
color flow mapping, showing a restricted patent foramen ovale
(PFO; arrow). (LA: Left atrium; RA: Right atrium).

arising from RV. Subcostal sagittal view in different planes

profiles the discordant ventriculoarterial connection,
parallel course of great vessels, and conal anatomy.
In patients with bilateral conus, fibrous discontinuity
between both semilunar valves and AV valves is present,
usually there is associated VSD and variation in great
vessel relationship, and coronary pattern may coexist. This
conal anatomy can be well profiled in subcostal sagittal,
coronal, and PLAX views. PLAX view demonstrates
parallel relation of great vessels and abrupt posterior turn
of pulmonary trunk immediately after origin (Fig. 72.135).
The parallel arrangement of great vessels is suggestive
of d-transposition of great vessels, but is not diagnostic,
as it can be found in other ventriculoarterial connections
such as DORV with malposed great vessels. High PSAX
and modified suprasternal long-axis views also profile the
origin of aorta from the morphological RV.
In a normal heart, great vessels have a wrap around
relationship, that is, pulmonary trunk arises from RV and
takes a leftward turn while aorta after arising from the LV
turns to the right (circle sausage appearance; Figs 72.137A
and B). With normally related great vessels, in PSAX view,
aorta is right and posterior to pulmonary trunk. With
complete transposition of great vessels, both outflows
are in parallel as seen in PLAX (Figs 72.138A and B) and
subcostal sagittal views. PSAX view profiles the spatial
relationship of great vessels, as double circles, aorta right
and anteriorly or directly anterior and less commonly
aorta left and anterior and posteriorly placed pulmonary
trunk. As both outflows are at different levels, usually both

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1655

Figs 72.137A and B: Two-dimensional echocardiography in parasternal long-axis view (Figure A) and parasternal short-axis view
(Figure B) at the level of the great vessels from an infant with transposition of great vessel (TGA) showing right and anterior position
of the aorta as compared to the pulmonary artery. Arrow shows the origin of the left main coronary artery. (Aorta: Ascending aorta; PA:
Pulmonary artery). (RV: Right ventricle; LV: Left ventricle).

Figs 72.138A and B: Two-dimensional echocardiogram in subcostal coronal view with anterior tilt. (A) Shows the origin of the aorta from
the right ventricle; (B) Shows the origin of the pulmonary artery (and pulmonary artery branching) from the left ventricle. (Ao: Aorta; LV:
Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

semilunar valves cannot be profiled in the same plane.

Main PA and its branches are seen at level of a cross section
across aortic valve, and a cross section across pulmonary
valve provides an image of RVOT. Apical four-chamber
view may demonstrate the initial outflow tract dividing
into branch pulmonary arteries followed by opening of the
aorta in a further anterior tilt (Figs 72.139A and B).

Chamber Size206210
After birth in simple (or complete) transposition of great
vessels with intact ventricular septum, with the fall in PVR
during first few weeks of life, left ventricular mass does not

increase and left ventricular posterior wall thickness starts

regressing as the LV is supporting the pulmonary circulation
(Figs 72.140A and B). The RV is systemic ventricle and LV
is pulmonary ventricle in complete transposition of great
vessels with intact ventricular septum. Four-chamber and
subcostal coronal views show dilated RA and RV and the
interventricular septum bulges toward the LV, giving the
configuration of a banana to the LV (banana-shaped LV).
The motion of the upper part of the ventricular septum is
normal in 50% of patients, that is, moving posteriorly in
systole while in the remainder, septal motion is abnormal,
remaining flat or moving anteriorly during systole and this
may also create a dynamic form of subpulmonary stenosis

1656

Section 6: Congenital Heart Disease

Figs 72.139A and B: Two-dimensional echocardiography in apical four-chamber view with anterior tilt showing the origin of pulmonary artery
(PA) from the left ventricle (LV). Further anterior tilt shows the origin of aorta (Ao) from right ventricle the (RV). Ventricular arterial discordance.

Figs 72.140A and B: Two-dimensional echocardiography in parasternal long-axis view in a 1-year-old patient with transposition of great
vessel (TGA) with intact ventricular septum. Shows the thinned out posterior wall of the left ventricle (2.7 mm). M-mode echocardiography of the same showing the regressed left ventricle (LV).

often associated with systolic anterior motion of the mitral

valve. These findings may be seen in apical four-chamber
view with anterior tilt and PLAX views. On M-mode,
systolic anterior motion of the anterior leaflet of mitral
valve is easily recognized.
Features to be assessed by echocardiography while
evaluating a child with complete transposition of great
vessels with intact ventricular septum for primary arterial
switch operation include:211,212
LV geometry
LV mass index (Table 72.15)
LV posterior wall thickness index (<0.3 mm taken as
regressed)

LV volume index
LV mass/volume ratio.
With intact interventricular septum, RA and RV
are always dilated as seen in apical four-chamber and
subcostal coronal views. In subcostal sagittal and PSAX
views, RV dominates with a circular configuration while
the LV takes on a crescentic shape.
In case of elevated left ventricular pressure as with
a nonrestrictive VSD, large PDA, large aortopulmonary
collaterals, significant left ventricular outflow obstruction,
or pulmonary arterial hypertension, the circular shape of
LV will be maintained with normal posterior wall thickness
and LV mass.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1657

Table 72.15: Left Ventricular Mass and Volume Indexed to Body Surface Area in m2.204

Newborns
Infants

Volume (mL) Enddiastole

Two-dimensional (2D) Mass

(g) End-diastole

M-mode (g)
End-diastole

Mass/Volume Index
(g/mL)

31.3 6.1

47.7 12.7

59.8 21

1.57 0.4

38.7 5

48.8 8

56.6 15

1.27 0.2

Children (14 years)

54.6 7.6

58.6 10.3

61.9 11.5

1.08 0.2

Children (48 years)

59.8 6.9

61.9 7.1

69 24

1.05 0.1

Children (812 years)

Children > 12 years

64 6

66.9 7.6

88.2 20

1.04 0.1

66.4 7.8

74.1 9.8

91.2 40

1.1 0.1

Figs 72.141A and B: Two-dimensional echocardiography showing balloon atrial septostomy with echocardiographic imaging in a case
of transposition of great vessels (TGA). (A) Subcostal sagittal view showing the catheter in inferior vena cava (IVC). (B) Subcostal coronal view showing the inflated balloon in left atrium. (B: Balloon; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

Associated Defects
Septal Defects
Interatrial Communication: Most commonly it is a patent
foramen ovale. Fossa ovalis ASD occurs only in 5% of cases
with complete transposition of the great vessels. Interatrial
communication as a site of bidirectional shunting results
in good intracardiac mixing if ASD is of adequate size.
Subcostal sagittal and coronal views are used to profile the
atrial communication and color flow mapping is used to
assess the direction of shunt, which is usually bidirectional
(left to right during systole, i.e. with an atrial v-wave,
and right to left during diastole, i.e. with atrial a-wave).
M-mode with color flow mapping across the ASD is the
best method to profile the direction of shunting. It is very
important to assess adequacy of interatrial communication
with intact ventricular septum or small VSD for better

mixing, and with large VSD or PDA to decrease left

atrial pressure and provide good mixing at the level of
atria. In case of restrictive interatrial communication,
echocardiography shows a dilated LA, interatrial septum
bowing toward the RA, and turbulent flow across the
interatrial communication with Doppler color flow
mapping. PW Doppler interrogation reveals gradient
across the interatrial communication. Echocardiographyguided balloon atrial septostomy to enlarge the interatrial
communication can be done in the intensive care setting
in neonates if the interatrial communication is restrictive
and arterial switch operation is not planned immediately
(Figs 72.141A and B). 2D echocardiogram from subcostal
sagittal view is used to profile the course of balloon catheter
from IVC to RA and then to LA. Subcostal coronal view is
used to profile the catheter course during septostomy and
to determine the size of ASD and flow across ASD after the
procedure.

1658

Section 6: Congenital Heart Disease

Ventricular septal defect: Features of VSD to be assessed

include:
Location of VSD.
Size of VSD.
Direction of shunt.
Relation of AV valves to VSD.
Relation of outflow tracts to VSD.
The most significant and frequently occurring defect in
complete transposition is a VSD, which can be small, large,
single, or multiple (Fig. 72.142). The most characteristic
defects are those that open beneath the ventricular outlets
with the muscular outlet septum malaligned with the rest
of the ventricular septum with overriding of one of the great
vessels, most commonly the PA (posterior great vessel).
These defects can have a posteroinferior muscular rim or
may extend to perimembranous area. Frequently, these
defects are crossed by the tension apparatus of tricuspid
valve, which often inserts to a papillary muscle arising from
the outlet septum. The outlet septum can be malaligned
to LV causing pulmonary stenosis or anteriorly causing
subaortic narrowing. In cases of anterior malalignment,
arch anomalies such as CoA or arch interruption may be
associated. The latter anomalies are more commonly seen
in DORV with subpulmonary VSD, a close differential
diagnosis of TGA. The VSD can extend to the inlet septum.
These defects are hidden beneath the septal leaflet of
tricuspid valve. When the defect extends into inlet septum,
there is potential for straddling and overriding of tricuspid
valve. With perimembranous VSD extending to inlet

Fig. 72.142: Two-dimensional echocardiography from a one and

half-month-old child with transposition of great vessels (TGA).
Parasternal long-axis view showing the presence of a large perimembranous ventricular septal defect () and posterior malalignment of the septum (arrow). (AO: Aorta; LA: Left atrium; LV: Left
ventricle; RV: Right ventricle PA: Pulmonary artery).

septum, there is potential for septal leaflet of tricuspid

valve to get prolapsed into the subpulmonary area and
cause subpulmonary stenosis. Other types of defects that
can be found are of AV canal type, multiple muscular,
large apical muscular, and doubly committed VSDs. With
a doubly committed defect, aorta is usually left-sided and
there are increased chances of the presence of coronary
anomalies.
On echocardiographic evaluation, the following need
to be assessed in patients with TGA:
VSD
Anomalies of AV valves
Attachment of tricuspid valve chordae to outlet
septum or to the crest of ventricular septum
Overriding of AV valve
Straddling of AV valves
Any evidence of a cleft in an AV valve
Outflow tract abnormalities
Outflow tract obstruction.
Outlet trabecular defect: Scanning from subcostal sagittal,
subcostal coronal, and PLAX views would visualize a
defect in outlet septum with or without extension to
perimembranous area, intact subaortic infundibulum,
and overriding PA. In addition, malalignment of outlet
septum should be looked for. Outlet VSD with posterior
malalignment (subpulmonary narrowing) can be profiled
from subcostal coronal view with anterior tilt, subcostal
sagittal view of left ventricular outflow, and PLAX view.
Because of the posterior deviation of outlet septum, there is
a direct route from LV to aorta making these children good
candidates for intraventricular re-routing from LV to aorta.
While evaluating for intraventricular re-routing of LV to
aorta, size of VSD in relation to aortic valve annulus needs
to be assessed in addition to relation of AV valves to VSD. If
the VSD is smaller in relation to aortic valve annulus, then
it needs enlargement during intraventricular repair. In the
presence of straddling of AV valve, biventricular repair will
not be possible and the patient would need to undergo a
Fontan procedure.
Anterior malalignment of outlet septum (subaortic
narrowing) can also be profiled from subcostal sagittal
view, subcostal coronal view of RV outflow, and PLAX
view. The anterior malalignment of outlet septum causes
subaortic narrowing and produces a long, oblique course
from the LV to aorta. This makes intraventricular repair
extremely difficult. With anterior malalignment of outlet
septum, chances of arch anomalies are higher so a careful
evaluation of aortic arch from suprasternal long-axis view
is mandatory to rule out a hypoplastic transverse arch,
coarctation, or arch interruption.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1659

With outlet VSD having perimembranous extension,

septal leaflet of tricuspid valve can prolapse across the
VSD into subpulmonary outflow leading to subpulmonary
narrowing, as mentioned previously. This can be profiled
from subcostal coronal and apical four-chamber views
with anterior tilt.
Inlet ventricular septal defect: Inlet VSD can be best
profiled from four-chamber views (apical or subcostal
coronal). Four-chamber (subcostal coronal and apical)
views are also useful to profile overriding of AV valves
and straddling of tricuspid valve if present. In addition, a
modified subcostal sagittal view at the level of AV valves
(en face view) can also define the presence of overriding
or straddling. Mitral valve straddling can also be profiled
from PLAX view as the mitral valve chordae cross the VSD
and insert on the opposite side of septum.
Doubly committed ventricular septal defect: Doubly
committed VSD is best profiled from a combination of
subcostal coronal and sagittal views, and PLAX and PSAX
view. These views show the defect in outlet septum and
presence of aortic and pulmonary valve continuity.
Scanning from subcostal (coronal and sagittal), apical,
and parasternal (long-axis and short-axis) views are used
to define muscular VSDs.
Color flow mapping helps in defining the direction of
shunt. With nonrestrictive VSD, direction of shunting is
left to right during diastole and right to left during systole.
With restrictive VSD, a turbulent jet from right to LV will
be found. Continuous wave Doppler interrogation shows a
pressure gradient across the VSD as well as the direction of
shunt.
Patent ductus arteriosus (Fig. 72.143): Suspicion of
presence of ductus arteriosus in complete transposition
occurs when on color flow mapping, reverse flow is seen in
PA from subcostal coronal with anterior tilt and subcostal
sagittal views. In complete transposition of great vessels,
because of the altered spatial relationship of great vessels,
the total length and anatomy of ductus is easily profiled
from suprasternal long-axis view. Color flow mapping and
pulsed Doppler interrogation is used to define direction
of shunting and magnitude of shunt across the ductus.
With a large PDA, as PA pressures are systemic, direction
of shunting is bidirectional, PA to aorta during systole
and aorta to PA during diastole if the PVR is lower than
systemic. With the development of pulmonary vascular
obstructive disease, direction of shunt becomes PA to
aorta during diastole too.

Fig. 72.143: Two-dimensional echocardiography. Suprasternal

view in a 6-day-old child with complete transposition of great vessels showing a large patent ductus arteriosus (PDA; arrow). (Des.
Ao: Descending aorta; PA: Pulmonary artery).

With restrictive PDA, color flow mapping reveals

turbulent flow from aorta to PA. With continuous wave
Doppler interrogation of ductus, a high velocity continuous
signal peaking in late systole is obtained.

Left Ventricular Outflow Tract Obstruction213

Left ventricular outflow obstruction can occur with an
intact ventricular septum as well as in the presence of a
VSD. Such lesions can be found at the level of valve or can
be subvalvular. Isolated valvular stenosis is rare and usually
occurs in combination with subvalvular obstruction,
which can be dynamic, fixed, or both.
Dynamic left ventricular outflow obstruction (Fig. 72.144):
With intact ventricular septum or small VSD, the RV has
systemic pressure; interventricular septum may bulge
toward LV in systole, causing dynamic subpulmonary
obstruction. In addition, this dynamic subpulmonary
obstruction causes systolic anterior motion of anterior
leaflet of mitral valve, and midsystolic closure of
pulmonary valve. 2D echocardiography in apical fourchamber, subcostal coronal, and PLAX views shows
bulging of interventricular septum toward LV during
systole. M-mode through the mitral valve clearly reveals
systolic anterior motion of anterior leaflet of mitral valve
and across the pulmonary valve shows midsystolic valve
closure. Color flow mapping reveals mild turbulence
across LVOT, and continuous wave Doppler usually
reveals pressure gradients. In its severest form it behaves

1660

Section 6: Congenital Heart Disease

Fig. 72.144: Two-dimensional echocardiography. Parasternal

long-axis view in a case of complete transposition of great vessels
(TGA) with intact interventricular septum showing systolic anterior
motion of the mitral valve (arrow). This creates a dynamic left ventricular outflow tract obstruction. (Ao: Aorta; IVS: Interventricular
septum; LV: Left ventricle; PA: Pulmonary artery).

Fig. 72.145: Two-dimensional echocardiography with parasternal

long-axis view showing posterior malalignment of the ventricular
septum leading to subvalvular left ventricular outflow tract obstruction (arrow) in a case of transposition of great vessels (TGA) with
ventricular septal defect (VSD) and pulmonary stenosis. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

like hypertrophic cardiomyopathy. The obstructive lesion

associated with septal bulging is more likely to occur with
anteroposteriorly related great vessels than side-by-side
related great vessels. Milder form of septal bulging can be
exaggerated by presence of fibrous ridge located on the
septal bulge.
Fixed anatomical obstruction: Fixed LVOT obstruction,
if significant, results in LV becoming spherical and thick
walled.
Fixed obstruction occurs because of:
Subpulmonary fibrous ridge/fibrous subpulmonary
membrane or ring. 2D echocardiography in fourchamber (subcostal coronal and apical) views with
anterior tilt and PLAX view reveals presence of discrete
ridge/membrane in subpulmonary area. This fibrous
ridge can be elongated giving a tunnel type lesion.
On color flow mapping, turbulence starts below the
pulmonary valve, and on pulsed or continuous wave
Doppler, severity of stenosis can be graded.
Valvar pulmonary stenosis is usually due to a bicuspid
pulmonary valve. 2D echocardiography in PSAX view
can demonstrate the bicuspid pulmonary valve. Color
flow mapping reveals that turbulence starts at the level
of pulmonary valve.
Anomalous attachment of the tension apparatus of
mitral valve across the outflow tract or accessory mitral
valve tissue can also cause LV outflow obstruction.
This can be profiled best in a five-chamber view
(Fig. 72.146).

Fig. 72.146: Two-dimensional echocardiography using subcostal

coronal view with anterior tilt in a child with complete transposition
of great vessels (TGA) showing prolapse of accessory mitral valve
tissue into the left ventricular outflow tract (LVOT, arrow), causing
obstruction. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
RV: Right ventricle).

All these lesions can occur with intact ventricular

septum as well as with VSD.
Malalignment of outlet septum: With nonrestrictive
outlet VSD, posterior malalignment of outlet septum
may narrow the subpulmonary outflow tract. Subcostal
coronal view with anterior tilt and PLAX view can
profile the posterior malalignment of outlet septum
(Figs 72.145 and 72.146).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Prolapse of septal leaflet of tricuspid valve or accessory

tricuspid valve tissue across VSD: In this condition, the
effective size of VSD becomes smaller due to prolapse
of accessory tissue or septal leaflet of tricuspid
valve. 2D echocardiography from subcostal coronal,
subcostal sagittal, and PLAX views can profile the
VSD and prolapse of tricuspid tissue across the defect
causing left ventricular outflow obstruction. Color
flow mapping is used to define level of obstruction and
direction of shunt across the VSD.

Coronary Arteries
The arterial switch operation for repair of complete
transposition requires the mobilization and reimplantation
of the coronary arteries. Whenever the position of the
aortic root is abnormal, the origin of the coronary arteries
deviates from those found in the normal heart. Thus,
identification of coronary artery anatomy becomes an
integral part of the preoperative echocardiographic
examination of a child with complete transposition
of great vessels. Coronary anomalies can be correctly
diagnosed preoperatively with echocardiography in 95%
of the patients. Sinuses are described from PSAX view and
labeled as left-facing sinus (left side-sinus I) and rightfacing sinus (right side-sinus II). In most cases, the orifice
of the coronary artery is situated approximately in the
middle of the sinus of Valsalva just below the sinotubular
junction; minor deviations from this central position are
found frequently.
In general, the origin and proximal course of the
coronary arteries can be profiled from modified PSAX view
at the level of great vessels and apical four-chamber and
subcostal coronal views. From the PSAX view at the level
of great vessels, relationship of great vessels and sinuses (I
or left-facing, II or right-facing, and noncoronary) should
be defined.

Coronary Artery Patterns in

Transposition of the Great Arteries214219
Left Anterior Descending and Circumflex
Coronary Arteries from Sinus I, and Right
Coronary Artery from Sinus II

Usual coronary pattern in complete transposition

Demonstration of separate origin of coronaries from
respective sinuses in PSAX view at the level of great
vessels and subcostal coronal view with anterior tilt

1661

Imaging the bifurcation of LCA into LAD and circumflex

vessels in PSAX view, PLAX view with anterior tilt, and
subcostal coronal view with anterior tilt
Absence of retropulmonary course of a coronary artery
(between mitral valve and the pulmonary trunk) in
apical four-chamber, subcostal coronal, and PSAX
views.

Circumflex Coronary Artery from Right

Coronary Artery (from Sinus II), Left Anterior
Descending Coronary Artery from Sinus I

Second most common pattern in complete transposition

Demonstration of separate origin of coronaries from
respective sinuses in PSAX view at the level of great
vessels and subcostal coronal view with anterior tilt
Demonstration of origin of circumflex coronary
artery from RCA and then retropulmonary course
of circumflex vessel (between mitral valve and the
pulmonary trunk) in apical four-chamber, subcostal
coronal, and PSAX views
Absence of bifurcation of LCA into LAD and circumflex
in PSAX view and subcostal coronal view with anterior
tilt.

Single Right Coronary Artery

Demonstration of single coronary ostium arising from

sinus II (right-facing sinus) in PSAX view
Imaging origin of LCA from RCA and then its retropulmonary course between mitral valve and pulmonary
trunk. This can be defined from modified PSAX (by
moving the transducer superior and rightward), apical
four-chamber, and subcostal coronal views
Demonstration of bifurcation of left main coronary
artery into LAD and circumflex branches
Very rarely, LAD and circumflex coronary arteries have
separate origins from the right sinus. In this scenario,
LAD coronary artery courses anterior to aorta and
the circumflex artery has a retropulmonary course
to reach the left AV groove. PSAX view at the level of
great vessels profiles origin of coronary artery from
sinus II, retropulmonary course of circumflex, and
course of LAD artery anterior to aorta. Four-chamber
views (subcostal coronal and apical) in posterior
plane profile retropulmonary course of circumflex and
anterior tilt from this view profiles LAD artery coursing
anterior to aorta.

1662

Section 6: Congenital Heart Disease

from left-facing sinus (sinus I). In this scenario, the

circumflex artery after arising from right courses
retropulmonary to reach the left AV groove, and RCA
courses anterior to aorta after its origin from left.

Single Left Coronary Artery

(Figs 72.147 and 72.148)
Less common than single RCA: Demonstration of single
coronary ostium arising from sinus I (left-facing sinus) in
PSAX view and origin of RCA from left. Its course anterior
to aorta can be profiled in PSAX, apical four-chamber, and
subcostal coronal view with anterior tilt. The coronaries
can arise from a single stump, all three coronaries separately or two coronaries with separate origin.
Rarely, circumflex artery arises from RCA and takes a
retropulmonary course to reach left AV groove. Subcostal
coronal view, apical four-chamber view, and PSAX view at
the level of great vessels shows retropulmonary course of
circumflex artery, while these views in anterior plane show
the RCA anterior to aorta.

Inverted Origin of Coronaries

Demonstration of separate origin of coronaries in

PSAX view.
Imaging the retropulmonary course of LCA arising
from right-facing sinus (sinus II) and its bifurcation
into LAD and circumflex branches. This can be profiled
in PSAX, apical four-chamber, and subcostal coronal
views.
Demonstrating origin of RCA from left-facing sinus
(sinus I), its course anterior to aorta in PSAX, and fourchamber views with anterior tilt.
Less commonly there can be inverted origin of
circumflex only and origin of RCA and LAD artery

Intramural Coronary Artery

An intramural coronary is a potentially significant risk
factor for transfer of the coronaries as part of the arterial
switch operation for transposition of great arteries.
Preoperative diagnosis is advantageous because it helps
to prevent accidental injury to the intramural coronary
artery during transection of the aortic root and excision of
the coronary artery ostium from the aorta.
Origin of coronary artery from contralateral sinus, as
in case of left main or LAD artery origin from rightfacing sinus or RCA from left-facing sinus profiled in
PSAX view. Their orifices are placed adjacent to the
commissure (usually intercoronary).
Identification of major coronary artery coursing between
the two arterial roots in PSAX, apical four-chamber, and
subcostal coronal views with posterior to anterior tilt.
After origin, the proximal portion of coronary artery
runs parallel to the aortic wall and perpendicular to
the intercoronary commissure, giving a double border
appearance to the posterior aortic wall.
In approximately 10% of cases, the orifice of a coronary
artery will arise close to one of the valve commissures that
can be defined in PSAX view at the level of great vessels.
With coronary ostia near the commissure, chances of

Figs 72.147A and B: Two-dimensional echocardiography in parasternal short-axis views in two cases of complete transposition of great vessels
(TGA) showing the origin of the coronary arteries. (A) In case A, the coronaries arise from a single stump. In case B, all the three coronary arteries
arise separately. (Ao: Aorta; LAD: Left anterior descending artery; RCA: Right coronary artery; LCx: Left circumflex artery).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1663

Fig. 72.148: Two-dimensional echocardiography with parasternal

short-axis view in an infant with TGA showing both the coronary
artery systems arising from a common sinus (sinus I with two separate origin). (Ao: Aorta; LMCA: Left main coronary artery; RCA:
Right coronary artery).

Figs 72.149A to C: (A) Two-dimensional echocardiography in parasternal short-axis view in an infant with TGA showing the origin
of dual right coronary artery (RCA). (A) RCA from sinus II; (B) Left
main and RCA from sinus I; (C) Apical four-chamber view with tilt
showing the presence of the left circumflex artery in the left atrioventricular (AV) groove. (Ao: Aorta; LA: Left atrium; LAD: Left anterior descending artery; LCT: Left common trunk; LCx: Left circumflex artery; LV: Left ventricle; PA: Pulmonary artery; RA: Right
atrium; RCA: Right coronary artery; RV: Right ventricle).

neopulmonary regurgitation are higher after arterial switch

operation. Less commonly, ostia can also arise above
the commissures in the tubular portion of the aorta

(higher origin of coronary). Occasionally, two ostia will

arise from the same sinus (usually sinus II; Fig. 72.148).
Rarely there may be a dual RCA219 (Figs 72.149A to C).

1664

Section 6: Congenital Heart Disease

PART 8: ATRIOVENTRICULAR AND VENTRICOARTERIAL DISCORDANCE

ATRIOVENTRICULAR AND
VENTRICOARTERIAL DISCORDANCE
Congenitally corrected transposition of great vessels
(CTGA) or L-transposition of great vessels is characterized
by discordant AV connections combined with discordant
ventriculoarterial connection. Thus, with situs solitus,
morphological RA on right side is connected to
morphological LV on right side (L-looped ventricles),
which is in turn connected to PA.220 On the other hand,
morphological LA is connected to morphological RV on
left side, which gives rise to aorta (Figs 72.150 and 72.151).
This anomaly can be encountered with situs solitus or
with situs inversus. Anomalies having univentricular AV
connection such as atresia of one of the AV valves, and
double inlet LV with hypoplastic RV on left side should be
excluded.
The aorta usually arises left and anterior to pulmonary
valve (L-transposed aorta), but rarely aorta can be right
and anterior to pulmonary trunk. There will be fibrous
continuity between mitral leaflet and the pulmonary valve.
On the other side, aorta is separated from tricuspid valve
by the muscular infundibulum.

Fig. 72.150: Two-dimensional echocardiography. Subcostal coronal view in a patient of corrected transposition of great vessels
(CTGA) showing atrioventricular discordance. (LA: Left atrium; LV:
Left ventricle; PA: Pulmonary artery; RA: Right atrium; RV: Right
ventricle).

Associated Defects221232
The commonest associated anomaly is left AV valve
(tricuspid valve) abnormalities.220 The other lesions are
VSD and pulmonary stenosis. However, any kind of
congenital cardiac malformation can be encountered.

Coronary Artery Anatomy233237

Usually coronary arteries arise from two aortic sinuses,
which are adjacent to the pulmonary trunk. The anatomy
is mirror image relative to the arrangement seen in the
normal heart. Right side coronary artery exhibits the
pattern of morphological LCA and left-sided coronary
artery exhibits the pattern of morphological RCA. The
most frequent coronary anomaly is single coronary artery.
Segmental analysis is crucial for the diagnosis of
corrected transposition and 5% of corrected transposition
cases occur in the setting of situs inversus. The
discordance between the atrial (abdominal) situs and
the cardiac position is itself a pointer to possibility of
CTGA, for example, dextrocardia with situs solitus and
levocardia with situs inversus, and 25% of patients with
corrected transposition have cardiac malposition, either

Fig. 72.151: Two-dimensional echocardiography. Subcostal coronal view with anterior tilt in a child of corrected transposition of
great vessels (CTGA) and ventricular septal defect (VSD; star)
showing the L-looped ventricles with aorta arising from morphological left-sided right ventricle and pulmonary artery arising from
morphological left ventricle (right-sided). Mitral and pulmonary
valves show continuity (arrow). (Ao: Aorta; LV: Left ventricle; PA:
Pulmonary artery; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1665

dextrocardia or mesocardia. As with d-transposition,

echocardiographic diagnosis of corrected transposition
of great vessels is based on sequential chamber analysis
and demonstrating abnormal AV and ventriculoarterial
connections. After defining situs from subcostal shortaxis view, both atria should be defined from subcostal
coronal, and sagittal and apical four-chamber views, to
profile discordant AV connection, that is, morphological
RA connects to morphological LV. The presence of atrial
situs (which generally goes along with the abdominal
situs) opposite to the cardiac position, that is, dextrocardia
with situs solitus and levocardia with situs inversus, is in
itself generally a setting of corrected transposition of great
arteries.
In situs solitus and levocardia: From the subxiphoid
view, as the transducer is angled superiorly from the
coronal view, the LV, which lies inferiorly and to the right is
viewed. Further superior tilt brings the RV in view, which
lies to the left of the morphological LV.
In situs inversus and dextrocardia: The morphological
RA is left-sided. On angulating the transducer superiorly
in the subxiphoid coronal view, the LV first comes under
view. It lies inferiorly and to the left. On further superior
tilt, the RV is viewed, lying to the right of the LV.
Four-chamber views show malalignment between
interatrial septum and inlet part of interventricular
septum. This is seen as a rightward curve of atrial septum
in situs solitus. After defining AV connections, ventri-

culoarterial connections should be defined from subcostal

coronal and apical four-chamber views (Fig. 72.151). To
demonstrate ventriculoarterial connections, it is important
to trace ventricular outflow tracts to the great arteries.
In the subcostal view, after the ventricles are identified,
continuing superior angulation first shows the PA arising
from the LV. This artery is deeply wedged between the
mitral and tricuspid valves and the pulmonary valve is
seen to be continuous with the mitral valve. This view also
profiles the LVOT and its abnormalities. Continuing the
superior angulation shows the aorta arising from the RV
(Figs 72.151 and 72.152). It is seen to lie furthest to the left
and follows a straight course upward. It has no continuity
with the tricuspid valve. The two great vessels are seen to
have a parallel course. In those with suboptimal subcostal
windows the aorta may not always be visualized in this view
because the ribs prevent further anterior tilt. The next view
is the apical four-chamber view. This may be suboptimal
in cases with mesocardia or dextrocardia. In these cases,
the transducer may have to be placed very close to the
sternum or some times over it. This view, however, shows
the features of discordant AV connection very well. In situs
solitus and levocardia, the left-sided AV valve (tricuspid
valve) is seen to attach more apically compared to the
right-sided AV valve (mitral valve; Figs 72.153 and 72.154).
This view also shows the moderator band of the leftsided morphological RV very clearly. The apex of the RV
(left-sided) frequently appears foreshortened in this view.

Fig. 72.152: A case of corrected transposition of great vessels

(CTGA). Subcostal paracoronal view with slight tilt to the right
showing the pulmonary artery arising from the right-sided left
ventricle and its division. (LA: Left atrium; LPA: Left pulmonary
artery; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle;
RPA: Right pulmonary artery).

Fig. 72.153: Two-dimensional echocardiography. Apical fourchamber view from a 4-year-old child with corrected transposition
of great arteries showing atrioventricular discordance, reverse
offsetting of atrioventricular (AV) valves (arrow), and moderator
band in morphological right ventricle on left side (arrow). (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

1666

Section 6: Congenital Heart Disease

The membranous septum is clearly seen in this view

situated between the morphological LV (right-sided) and
LA (left-sided).
Anterior tilt from the apical four-chamber view first
shows the PA and LVOT (Fig. 72.155). On further anterior
tilt, the aorta should be seen to the left of the PA. This
view may not always be optimal, because on continuing
anterior tilt, transducer contact with the chest wall is
frequently lost. Loss of offsetting remains a characteristic
feature. (Figs. 72.156A and B).

Subcostal sagittal view also defines ventriculoarterial

connections, origin of aorta from RV, and PA from LV
and parallelly arranged great vessels. Parasternal long
axis is oriented in a much more vertical direction than
usual. As ventricular septum in corrected, transposition
is sagittally oriented, the long-axis view may be confusing,
particularly in the presence of large perimembranous
VSD with inlet extension giving the impression of single
ventricle. This occurs because the ventricles are side-byside with sagittally arranged interventricular septum, and

Fig. 72.154: Two-dimensional echocardiography with zoomed up

view of the crux of the heart showing atrioventricular discordance
and reverse offsetting of the atrioventricular (AV) valves (arrow).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle).

Fig. 72.155: Two-dimensional echocardiography with fourchamber view with anterior tilt in a case of corrected transposition
of great vessels (CTGA) showing a regressed left ventricle; there is
no left ventricular (LV) outflow tract obstruction. (LV: Left ventricle;
PA: Pulmonary artery; RV: Right ventricle).

Figs 72.156A and B: Two-dimensional echocardiography. Apical four-chamber view in a case of corrected transposition of great vessels
(CTGA) showing the comparison of the normal heart with normal offsetting (arrow) (Figure A) of atrioventricular valves as compared to
the CTGA with reverse offsetting and the trabeculated right ventricle (RV) with a moderator band (Figure B). (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

the plane of ultrasound passes through an AV valve and

semilunar valve without passing through any portion of
the interventricular septum.
PLAX view profiles parallel-arranged great vessels
and multiple chords attaching to interventricular septum
with tricuspid valve and aortic discontinuity. Because the
ventricles and great vessels are side-by-side, in long-axis
view of LV and PA the adjacent RV and aorta may also be
visualized. Minor posterior tilting from PLAX view shows
the posterior AV valve continuity with the posterior arterial
valve. The PSAX view at the level of great vessels defines
spatial relationship of great vessels. As great vessels have
parallel arrangement, these are seen as a double circle in
PSAX view, usually aorta to left and anterior to PA. However,
in corrected transposition, any spatial orientation of great
vessels is possible, as in d-transposition of great vessels.
As both semilunar valves are not at the same level, cross
section through the aortic valve profiles main PA and its
bifurcation and cross section through pulmonary valve
profiles RVOT in long axis. PSAX view is oriented more
horizontally in corrected transposition and ventricular
septum is aligned in anteroposterior direction.
Coronary artery can be defined from PSAX view;
origin of RCA (anatomical left) can be profiled from left
coronary cusp, although it is more difficult to profile the
left coronary cusp than in d-transposition of great vessels.
As aorta is left and anterior, ascending aorta ascends on
left border of the heart straight up on the left and then
arches, and descending aorta descends on the left behind
the ascending aorta. High left parasternal view with
anticlockwise rotation (ductal view) profiles the ductus
and also the whole length of arch because of its leftward
and anterior position. Usually, the aortic arch is leftsided with normal arch branching; demonstration of arch
branches is important as right aortic arch occurs in 18% of
cases with corrected transposition.

Associated Malformations
Ventricular Septal Defect
It is reported in 6070% of patients with CTGA. The
commonest is perimembranous defect (Fig. 72.151), which
is subpulmonary and extends posteriorly toward the crux of
the heart and erodes significantly the inlet septum. Fibrous
continuity between mitral, tricuspid, and pulmonary valve
is present. It is important to define VSD relation to AV valves
and document if there is any overriding or straddling of AV
valves, more commonly of tricuspid valve. In cases with

1667

Fig. 72.157: Two-dimensional echocardiography. Modified parasternal long-axis view showing the origin of the pulmonary artery
from the left ventricle (LV) with prolapse of the mitral valve into
the left ventricular outflow tract (LVOT; arrow) causing obstruction.
(LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

VSD extending to inlet septum, offsetting of AV valves is

lost. Septal leaflet of tricuspid valve may prolapse through
VSD, causing it to become hemodynamically smaller, or it
may prolapse into subpulmonary area through the defect
causing subpulmonary stenosis (Fig. 72.157). Muscular
VSD can be profiled from a combination of views (fourchamber, parasternal long- and short-axis). These views
define muscular boundaries of the defect and its extension
(Figs 72.158 and 72.159). Doubly committed VSD is best
profiled from subcostal coronal view and is roofed by the
continuity between aortic and pulmonary valve leaflets.
Color flow mapping shows direction of shunt, turbulent
(restrictive) or laminar flow across the VSD (Fig. 72.159).
Pressure gradient (difference in left and RV pressure) can
be determined by use of continuous wave Doppler.

Tricuspid Value Abnormalities232 (Fig. 72.160)

These are almost an essential part of CTGA and are
seen in 90% of cases in autopsy series, although they
are hemodynamically significant in 30% of cases only.
The most common anomaly is valvular dysplasia, with
or without apical displacement of the septal and or
mural leaflet. Apical four-chamber, parasternal longaxis, and modified short-axis views are the best to define
tricuspid valve anomalies, including thickening of valve
leaflets and downward displacement of septal and mural
leaflets. Atrialization, with thinning and dilatation of RV
as seen in Ebsteins anomaly with concordant AV and
ventriculoarterial connections is rare with corrected

1668

Section 6: Congenital Heart Disease

Figs 72.158A and B: Two-dimensional echocardiography. Apical four-chamber view with color compare with anterior tilt showing the
origin of the pulmonary artery from the left ventricle and the presence of supravalvular pulmonary stenosis (white arrow). A ventricular
septal defect is noted (yellow arrow) in the anterior muscular plane. (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).

Fig. 72.159: Two-dimensional echocardiography with color comparison showing a small anterior muscular ventricular septal
defect (arrow) in a case of corrected transposition of great vessels
(CTGA). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery;
RV: Right ventricle; RA: Right atrium).

Fig. 72.160: Two-dimensional echocardiography. Apical fourchamber view with color flow mapping showing severe tricuspid
regurgitation (TR) in Ebsteins anomaly of the left-sided atrioventricular (AV) valve in a case of corrected transposition of great vessels (CTGA). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

transposition. With significant Ebsteins malformation,

hypoplasia of RV and occasionally subaortic obstruction
can occur and should be properly defined.
Other abnormalities can be short and thickened
chordae that insert directly into ventricular wall, deformed and abnormal papillary muscles, dilatation of
tricuspid valve annulus, and straddling of the left AV valve
(Fig. 72.161). By color flow mapping, valvular regurgitation
(more commonly) or stenosis of tricuspid valve should
be defined. Less commonly, tricuspid valve stenosis
can occur in congenitally corrected transposition due

to supratricuspid ring or membrane. From apical fourchamber and PLAX view, this membrane is seen as thin,
linear echoes just above the tricuspid valve, attaching
laterally to free wall of LA and medially to left atrial
surface just above the crux. Color flow mapping shows
turbulence beginning above the level of valve, and on
pulsed or continuous wave Doppler interrogation, severity
of obstruction can be defined. Rarely cor triatriatum
sinister may be the cause of tricuspid inflow obstruction.
Best views to profile cor triatriatum are PLAX and fourchamber views. In PLAX view, the membrane extends

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1669

from posterior wall of LA toward interatrial septum. In

apical four-chamber view, the membrane extends from
lateral wall of LA above the left atrial appendage toward
the interatrial septum. Cor triatriatum can be obstructive
or nonobstructive. Color flow mapping and pulsed
Doppler imaging should be used to define any obstruction
to tricuspid inflow.
Mitral valve abnormality with CTGA: Dysplastic mitral
valve can occur, but is not common.

is obtained with the use of pulsed and continuous wave

Doppler interrogation. Often a PA band is placed in the
restricted VSD or intact septum subgroup as a measure to
prepare the LV for the subsequent double switch surgery
(Fig. 72.164). Care should be taken to separate the jet of
VSD from pulmonary stenosis.

Left Ventricular Outflow Tract

Obstruction
Pulmonary stenosis occurs in approximately 30 to 50% of
patients, with 85% of these in association with a VSD. The
anatomical nature of pulmonary stenosis varies. Valvular
stenosis is usually accompanied by one or the other
variety of subpulmonary obstruction. Subpulmonary
obstruction may take the form of fibrous diaphragm or
an aneurysm of fibrous tissue that protrudes into LVOT
(see Fig. 72.157). The fibrous tissue tags may originate
from perimembranous septum, tricuspid or mitral valve,
or even a leaflet of pulmonary valve (see Fig. 72.157).
Posterior malalignment of outlet septum associated with
VSD can also cause LVOT obstruction. The anatomical
features of LVOT are best defined from subcostal coronal
and apical four-chamber views with anterior tilt, and
PLAX view (Figs 72.162 to 72.164). Peak pressure gradient

Abnormalities of RVOT are somewhat uncommon,

occurring in about 10% of patients. Obstruction or severe
regurgitation of tricuspid valve is commonly associated
with outflow tract obstruction. In the setting of severe
left AV valve regurgitation, one must be certain that the
obstruction is not simply functional. As aorta is supported by
the muscular infundibulum, muscular outflow obstruction
may be dynamic. Other causes of obstruction can be
anterior malalignment of outlet septum with VSD, subaortic
membrane, valvular stenosis, and accessory mitral or
tricuspid valve tissue. The RVOT can be best imaged from
parasternal long-axis, subcostal coronal with anterior tilt,
and subcostal sagittal views. Doppler interrogation can be
difficult due to improper alignment, and a high parasternal
or subcostal coronal with anterior tilt views are used in an
attempt to align the Doppler beam.
Coarctation of aorta is often associated with severe
tricuspid regurgitation, VSD, hypoplasia of RV, and subaortic obstruction.

Fig. 72.161: Two-dimensional echocardiography in apical fourchamber view in a case of corrected transposition of great vessels
(CTGA) showing Grade I straddling of the left atrioventricular
valve (tricuspid) across the large inlet ventricular septal defect
(arrow). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).

Fig. 72.162: Two-dimensional echocardiography in subcostal view

with anterior tilt and color flow mapping in corrected transposition of
great vessels showing turbulence across left ventricular outflow tract
(LVOT) (arrow) because of hypoplastic pulmonary annulus and main
pulmonary artery (MPA). (LV: Left ventricle; PA: Pulmonary artery;
RV: Right ventricle; RA: Right atrium).

Abnormalities of Right Ventricular

Outflow

1670

Section 6: Congenital Heart Disease

Fig. 72.163: Two-dimensional echocardiography in subcostal view

with anterior tilt and color flow mapping in corrected transposition
of great vessels (CTGA) showing turbulence across left ventricular
outflow tract (LVOT) because of supravalvular pulmonary stenosis.
(LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle)
(arrow).

Fig. 72.164: Two-dimensional echocardiography in apical fourchamber view with anterior tilt showing a pulmonary artery (PA)
band in situ with flow acceleration across it (arrow). (LV: Left
ventricle; RV: Right ventricle; PA: Pulmonary artery).

PART 9: PULMONARY VEINS

Evaluation of the pulmonary veins forms an essential
component of any segmental analysis of the congenital
heart. The indicators for the abnormal connection include
dilated chambers to which the veins drain, right-to-left atrial
shunting, nonvisualization of the veins to the LA (bald LA),
and a small LA. In such a scenario it is equally essential to
screen the systemic veins (SVC, IVC, innominate veins) for
abnormal dilatation or increased flow. Echocardiographic
views for demonstration of pulmonary venous connection
to LA and their flow patterns are:238241
The subcostal view (Figs 72.165A and B): The coronal
section clearly demonstrates right upper and left
upper pulmonary venous connection to LA, while
the sagittal section identifies the right upper and right
lower pulmonary veins.
Apical four-chamber view (Figs 72.166 and 72.167):
This view gives a clear visualization of the left-sided
pulmonary veins and right upper pulmonary vein.
Posterior tilt in apical four-chamber view at level of
IVC shows the right lower pulmonary vein connection
to LA.
Parasternal short- and long-axis views: PSAX and
PLAX views show both left and right upper pulmonary
veins. The descending aorta in short axis separates
right and left pulmonary veins.

Suprasternal short-axis view (Crab view): This is the

best view in the pediatric age group to demonstrate all
four pulmonary veins that are located below the right
PA (Figs 72.168A and B).

NORMAL FLOW PATTERN OF

PULMONARY VEINS
All pulmonary veins should be interrogated by color
flow mapping followed by pulsed and continuous wave
Doppler with attention to intercept angle, wall filter, and
velocity scale. Pulsed Doppler sample volume should
be placed distal to the orifice of the pulmonary vein as it
enters the LA, so that Doppler velocity reflects the events
occurring in pulmonary vein and not the LA. In normal
subjects, the pulmonary vein Doppler recording consists
of biphasic forward flow, S-wave in systole due to atrial
relaxation and descent of floor of LA, D-wave in diastole
due to fall in LA pressures with opening of mitral valve and
rapid ventricular filling, and a small reversed A-wave
occurring during LA contraction.
The normal values for the S-, D-, and A-wave in
subjects under the age of 50 years are nearly constant,
except in newborns who have nearly equal S- and D-waves.
Continuous high velocity pattern present in pulmonary
veins in the first few days of life may be due to relatively

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1671

Figs 72.165A and B: Two-dimensional echocardiography in subcostal coronal view with color flow mapping showing right upper and
left upper pulmonary veins draining into left atrium (LA; arrows). LA: Left atrium; RA: Right atrium).

Figs 72.166A and B: Two-dimensional echocardiography in sagittal subcostal view showing right upper and right lower pulmonary veins
draining to left atrium (LA; arrows). (LA: Left atrium; RA: Right atrium).

Figs 72.167A and B: Two-dimensional echocardiography in apical

four-chamber view showing the right upper and left lower pulmonary
veins connecting to left atrium (LA; arrows). (LLPV: Left lower
pulmonary vein; RUPV: Right upper pulmonary vein).

1672

Section 6: Congenital Heart Disease

Figs 72.168A and B: Two-dimensional echocardiography in

suprasternal short-axis view (crab view) with color compare
showing all four pulmonary veins connecting to the left atrium
(arrows). (LLPV: Left lower pulmonary vein; LUPV: Left upper
pulmonary vein; RLPV: Right lower pulmonary vein; RUPV: Right
upper pulmonary vein).

Figs 72.169A and B: Two-dimensional echocardiography in

suprasternal short-axis view with color compare showing leftsided pulmonary veins (arrows) draining to the innominate vein.
(Innom: Innominate vein).

hypoplastic and underperfused pulmonary veins during

fetal life, which are suddenly exposed to increased
pulmonary blood flow after birth with fall in PVR.

on the other hand has been reported. Usually, left-sided

pulmonary veins connect anomalously to the derivatives
of left cardinal system, that is, coronary sinus and the left
innominate vein. Right-sided pulmonary veins usually
connect to derivatives of right cardinal system, that is, SVC
or IVC. However, as the developing splanchnic plexus is
a midline structure, the possibility of cross connection
exists.
Most cases of PAPVC associated with an ASD with
only 20% of cases having intact interatrial septum. Other
associated heart defects can be VSD, LSVC, and complex
congenital heart defects associated with isomerism. After
sinus venosus ASD, the commonest type of PAPVC is left
upper lobe pulmonary vein to left innominate vein followed
by right pulmonary veins to IVC (Scimitar syndrome).
The latter group is associated with hypoplasia of right PA
and right lung, anomalies of bronchial system, horseshoe lung, pulmonary sequestration, and dextroposition
of heart along with, on the chest X-ray, a Turkish swordlike shadow on the right heart border produced by the
descending pulmonary venous channel formed by rightsided pulmonary veins connecting to IVC.
While doing echocardiography, a high index of
suspicion is required to diagnose PAPVC. In any patient
with right atrial and RV dilation, all pulmonary veins
should be defined on 2D echocardiography and color flow
mapping connecting to LA, and all possible drainage sites
should be interrogated with color flow, because in the
case of supernumerary pulmonary veins, one can profile

ANOMALIES OF PULMONARY VEINS

Abnormal Number of Pulmonary Veins
Normally there are two right and two left pulmonary veins.
The most common variation is the presence of a single
pulmonary vein on either left or right side. There may
be increased number of pulmonary veins. The number
of pulmonary veins on one side could vary from three to
five. The variation in number of pulmonary veins is of no
clinical significance if they are connected normally to LA.

Anomalous Pulmonary Venous Return

Partial anomalous pulmonary venous connection
(PAPVC) is used to describe the condition where one or
more but not all pulmonary veins connect abnormally
(Figs 72.169A and B) and total anomalous pulmonary
venous connection (TAPVC) is a term used when all
four pulmonary veins connect abnormally to systemic veins
or RA. When pulmonary veins are connected normally to
posterior wall of LA but drain anomalously because of the
orientation of the atrial septum, the condition is termed
as partial or total anomalous pulmonary venous drainage
(PAPVD/TAPVD).
Almost every possible connection between pulmonary
vein on one hand and various systemic venous tributaries

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

four pulmonary veins connecting to LA but an accessory

pulmonary vein or a tributary of pulmonary vein may be
draining anomalously.
In majority of cases with PAPVC, there will be evidence
of right-sided volume overload in the form of dilated RA
and RV with paradoxical septal motion except in the case
of PAPVC of a single pulmonary vein or PAPVC associated
with stenosis (small shunt).

TOTAL ANOMALOUS PULMONARY

VENOUS CONNECTION242246
All pulmonary veins form a confluence and that confluence
drains to systemic venous system. The commonest site of
drainage is left innominate vein followed by portal system,
coronary sinus, SVC, RA, and rarely mixed type, that is,
more than one site of abnormal connection.
The goals of echocardiography are:
To define individual pulmonary veins
To ascertain that all pulmonary veins are draining
to pulmonary venous confluence (PVC) or draining
directly
Relation of PVC to LA, PA, and airways
Site of drainage of PVC/individual veins
Any evidence of obstruction at the site or during the
course of the pulmonary venous channel
Adequacy of interatrial communication
PA pressures
Any associated heart defect.

Fig. 72.170: Two-dimensional echocardiography. Apical fourchamber view in a case of total anomalous pulmonary venous
connection (TAPVC) showing markedly dilated right atrium and
right ventricle. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

1673

Steps in Diagnosis of Total Anomalous

Pulmonary Venous Connection
Features Common to all Forms of Total
Anomalous Pulmonary Venous Connection
Dilated right-sided structures (RA, RV, and PA; Fig. 72.170).
LA and LV (may appear smaller)
Interatrial septum bows toward left, with right-to-left
shunt at atrial level (Fig. 72.171)
RV appears to compress the LV and on M-mode,
evidence of paradoxical ventricular septal motion
suggestive of RV volume overload is seen
Echocardiographic evidence of pulmonary hypertension may exist, in the setting of obstruction or flowrelated PA hypertension
Inability to image the pulmonary vein entrance to LA is
the first echocardiographic suspicion that one may be
dealing with TAPVC.

Pulmonary Venous Confluence (PVC)

The common chamber to which all pulmonary veins
connect before finally draining to systemic venous channel
is referred to as PVC. PVC is profiled as an echo-free space,
which lies in a plane posterior to LA but is separated from
it. It is well seen in suprasternal short-axis and subaortic
coronal views. In the subcostal sagittal view in its cross
section below the right PA and posterior to the SVC, giving
the appearance of a double circle. The size and orientation
(horizontal and vertical) of PVC and its relation to LA is
important when planning for surgery.

Fig. 72.171: Two-dimensional echocardiography with color

compare in subcostal coronal view in a 6-month child with total
anomalous pulmonary venous connection (TAPVC) showing rightto-left shunt at the atrial level (arrow). (LA: Left atrium; RA: Right
atrium).

1674

Section 6: Congenital Heart Disease

After identifying the PVC, the next step is to profile

individual pulmonary veins for their course and diameter
because obstruction can occur at any level. This is the
strong independent predictor of survival in TAPVC.

In case of supracardiac TAPVC (to vertical vein/SVC/

azygos vein), PVC lies horizontally oriented posterior
and superior to LA (Figs 72.172A and B). This can be best
visualized in suprasternal short-axis and subcostal sagittal
views. In supracardiac TAPVC to left innominate vein, the
ascending channel connecting to left innominate vein can
be shown in suprasternal short-axis view (Fig. 72.173).
In this view, the anomalous pulmonary venous connection gives the appearance of a large vascular collar
surrounding the transverse arch. Color flow mapping
in a vertical vein shows continuous low velocity flow
directed away from the heart. There will be evidence of
dilated innominate vein and SVC along with dilated rightsided chambers. In TAPVC to SVC or azygos vein, the
innominate vein will be of normal caliber and in TAPVC
to azygos or to SVC, the SVC will be grossly dilated. In a
rare variety of TAPVC, that is, to azygos vein, the channel
ascends posterior to right PA and joins the azygos vein
(Fig. 72.174). The best view to define this anatomy is
subcostal sagittal, high PSAX, and suprasternal short axis
views. In case of intracardiac TAPVC (to RA/coronary

sinus), the PVC lies directly posterior to LA and all four

pulmonary veins join ipsilaterally to that confluence at
the same level (Fig. 72.175). In intracardiac type of TAPVC,
with connection to RA, the PVC can be imaged draining
directly to RA. Rarely the pulmonary veins can drain
individually to RA. The views most commonly used are
subcostal coronal, and sagittal and PSAX views. In TAPVC
to coronary sinus, the dilated coronary sinus receiving
all pulmonary veins bulges anterosuperiorly into LA and
can be imaged easily in subcostal coronal, apical fourchamber, and PLAX views.
In infracardiac TAPVC (to IVC/hepatoportal system),
the pulmonary veins connect to vertical vein at a different
level and the repair is more challenging (Fig. 72.176).
The PVC is often small, inferior and posterior to LA, the
descending venous channel (Des. PVC) passes through the
diaphragm usually anterior to aorta, and joins a systemic
vein or the hepatoportal system (portal vein, ductus
venosus, left hepatic vein, and IVC). Three channels, two
descending (aorta and the Des. PVC) and one ascending
(IVC, which is dilated) can be defined in subcostal views.
The short-axis view shows these three vessels in short axis.
Subcostal sagittal view is the best view to define the origin
of the PVC, its course, and connection to hepatoportal
system. Pulse Doppler examination shows continuous low
velocity flow signals or high velocity disturbed flow if there
is obstruction, which is usually present in these cases. The
flow in the descending pulmonary venous channel is away
from the heart and continuous low velocity flow signals in
IVC are directed toward the heart.

Orientation and Site of Drainage of

Pulmonary Venous Confluence

Figs 72.172A and B: Suprasternal short-axis view with color flow mapping in a case of supracardiac total anomalous pulmonary venous
connection (TAPVC). (A) Shows right- and left-sided pulmonary veins forming a pulmonary venous confluence; (B) Shows pulmonary
venous confluence continuing as vertical vein. (PVC: Pulmonary venous confluence; RPA: Right pulmonary artery; VV: Vertical vein).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1675

Fig. 72.173: Two-dimensional echocardiography with color flow

mapping. Suprasternal short-axis view in a patient with supracardiac total anomalous pulmonary venous connection (TAPVC) with
color flow mapping showing the ascending vertical vein (VV) from
the pulmonary venous confluence draining into the innominate vein,
which together with the superior vena cava (SVC) is dilated. (Innom: Innominate vein; SVC: Superior vena cava; VV: Vertical vein).

Fig. 72.174: Two-dimensional echocardiography with colour flow

mapping in suprasternal view in a case of supracardiac total
anomalous pulmonary venous connection (TAPVC) showing the
pulmonary veins from left and right sides ascending and draining
to the azygos vein (arrows). (AZ: Azygos vein).

Fig. 72.175: Two-dimensional echocardiography with color flow

mapping. Subcostal coronal view showing the pulmonary venous confluence draining to the coronary sinus (CS), right-to-left shunt across
the patent foramen ovale (PFO; arrow), dilated right atrium (RA), and
normal-sized superior vena cava (SVC). (CS: Coronary sinus; RA:
Right atrium; PFO: Patent foramen ovale; SVC: Superior vena cava).

Fig. 72.176: Two-dimensional echocardiography with color

compare. Subcostal sagittal bicaval view (modified) in a case of
infradiaphragmatic total anomalous pulmonary venous connection
(TAPVC) showing the pulmonary venous confluence continuing as
the descending vertical vein (desc. VV) and then draining into the
inferior vena cava through hepatic sinusoids (RA: Right atrium).

Rarely, a mixed form of TAPVC occurs in which

pulmonary veins drain to two or more separate systemic
venous sites. In this subgroup, multiple windows using
2D and color flow mapping must be used to image the
connection of all four individual pulmonary veins. The
most common type of mixed drainage connection is to
coronary sinus and via vertical vein to left innominate
vein.

Defining the Obstruction247

Color flow mapping and PW Doppler should be used to
diagnose the presence of obstruction. Normal pattern
in a pulmonary vein is laminar flow on color Doppler
mapping and biphasic low velocity flow on pulse
Doppler. If there is obstruction at any level, there will
be turbulent, aliased flow on color flow mapping and

1676

Section 6: Congenital Heart Disease

high velocity disturbed flow on pulsed Doppler, which will

not touch the baseline, and with more severe obstruction
it will be continuous. The level of obstruction can be the
following:
Long channel itself offers some resistance
Restrictive interatrial communication (Fig. 72.177)
In supracardiac TAPVC to left innominate vein, as the
ascending channel passes between left PA and left
bronchus (vascular ring), it gets compressed (so-called
hemodynamic vise; Figs 72.178A and B)

At junction of venous confluence to the site of drainage

(Fig. 72.179)
In infradiaphragmatic TAPVC
As channel passes through the diaphragm
Resistance offered by hepatoportal system.
The tricuspid regurgitation velocity helps to predict the
PA pressures. In obstructed TAPVC if a ductus is present,
there may be right-to-left shunt indicating suprasystemic
pressures in the pulmonary circuit.

Anomalous Drainage of Pulmonary

Veins with Normal Connection
Anomalous pulmonary venous drainage may be partial
(PAPVD) or complete (TAPVD).248 Normally connected
pulmonary veins may have flow directed to RA in the
following conditions:
Sinus venosus ASD.
Large fossa ovalis ASD extending to posterior wall
of LA.
Septum primum malposition defect.

Fig. 72.177: Two-dimensional echocardiography with color flow

mapping in subcostal sagittal view showing a restrictive interatrial
communication (arrow) with the turbulence across a patent
foramen ovale in a case of total anomalous pulmonary venous
connection (TAPVC). (LA: Left atrium; RA: Right atrium).

Septum Primum Malposition Defect

Causing Partial or Total Anomalous
Pulmonary Venous Drainage
(Figs 72.180 and 72.181)
This condition is usually associated with visceral
heterotaxy, commonly with left isomerism and rarely with
right isomerism.

Figs 72.178A and B: (A) Two-dimensional echocardiography with color flow mapping. Modified suprasternal short-axis view showing
turbulence in the ascending vertical vein in a case of supracardiac total anomalous pulmonary venous connection; (B) Continuous
wave Doppler tracing showing the continuous flow throughout the cardiac cycle with a mean gradient of 5 mm Hg. (Ao: Aorta; Innom:
Innominate vein; VV: Vertical vein).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1677

Fig. 72.179: Two-dimensional echocardiography using subcostal

coronal view with anterior tilt and color flow mapping showing the
pulmonary venous confluence (PVC) draining to the coronary sinus.
Patent foramen ovale (PFO) shunting is right to left (white arrow).
There is flow acceleration at the junction of the pulmonary venous
confluence and the coronary sinus (black arrow). (CS: Coronary
sinus; RA: Right atrium).

Figs 72.180A and B: Two-dimensional echocardiography. Subcostal coronal view with color compare in a case of total anomalous
pulmonary venous drainage showing a malaligned interatrial septum (arrow) resulting in the drainage of the pulmonary veins occurs into
the right atrium (arrows). (LA: Left atrium; RA: Right atrium).

Fig. 72.181: Two-dimensional echocardiography. Apical fourchamber view showing malaligned interatrial septum (arrow) leading to total anomalous pulmonary venous drainage with normally
connected pulmonary veins. RA is markedly dilated. (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

1678

Section 6: Congenital Heart Disease

Echocardiographic pointers to the diagnosis of septum

primum malposition defect are:
Deviation of septum primum toward anatomical LA
Absence of septum secundum
Normally attached pulmonary veins to anatomical LA
Interatrial communication in most posterior plane
between posterior wall of LA and displaced septum
primum
On color flow mapping, drainage of pulmonary
veins to anatomical RA can be visualized. Number of
pulmonary veins draining anomalously depends upon
degree of malposition of septum primum.
The views that can profile these anomalies are
subcostal coronal, apical four-chamber, and parasternal
long-axis.

or situs inversus). In contrast, the incidence of systemic

venous anomalies in patients with heterotaxy syndrome
exceeds 90%. Systemic venous anomalies are usually
associated with other CHD.

Pulmonary Vein Stenosis

Persistent Left Superior Vena Cava

Pulmonary vein stenosis may be seen with TAPVC and

needs to be keenly looked at but as an isolated case is a
rare anomaly. It is usually associated with other lesions.
Echocardiographic diagnosis is based upon a high index of
suspicion in patients who present with features of primary
pulmonary hypertension or pulmonary hypertension out
of proportion to the heart defect.
After 2D demonstration of pulmonary venous connection, color flow mapping must be done. On CFI, turbulent
or mosaic flow pattern is the first indication of pulmonary
vein stenosis in contrast to laminar flow in nonobstructed
pulmonary veins. Pulsed and continuous wave Doppler
interrogation reveals loss of phasic flow and/or increased
velocity. The change in flow pattern depends upon the
length of the obstructed channel and severity of stenosis.
The condition has to be differentiated from increased
pulmonary venous flow velocity associated with a large
left-to-right shunt. In that case, there will be increased
pulmonary vein velocity with preservation of phasic
pattern, which touches the baseline. On 2D measurement,
the stenosed pulmonary vein will be found to be of smaller
caliber than normal.

ANOMALIES OF SYSTEMIC VEINS

Systemic venous anomalies are rarely symptomatic in
isolation, but are brought to notice on evaluation for
other associated lesions. Their visualization may have
a significant impact on the management of various
lesions either directly or indirectly. Clinically significant
abnormalities of the systemic veins are infrequent when
the visceroatrial situs is lateralized (either situs solitus

Abnormalities of systemic veins may be classified into

abnormalities involving:249252
SVC.
Retroaortic innominate vein
IVC and hepatic veins
Abnormalities of coronary sinus
Persistence of valves of embryonic venous sinus
Total anomalous systemic venous connection
Decompressive veins like levoatriocardinal vein.

Superior Vena Cava

The commonest abnormality of SVC is persistence of
LSVC draining to coronary sinus and then to RA. The
prevalence of LSVC is 0.3% in general population, and 2
to 10% with congenital heart defects.253 LSVC exists with
right superior vena cava (RSVC) in > 84% of cases and in
>75% cases, a bridging innominate vein (H connection)
is present. LSVC is located anterior to aortic arch and left
PA, passes inferiorly, accepts hemiazygos vein, and then
joins coronary sinus in the posterior AV groove.
Hemodynamic significance: Although in isolation, it is
of no hemodynamic significance, it poses difficulty with
transvenous insertion of pacemaker leads; during an open
heart procedure, separate venous cannulation is needed
if the bridging vein is absent or very small and in patients
undergoing univentricular correction. It has been noted
that with persistent LSVC, the incidence of arrhythmias
is higher and chances of preexcitation are ten times
more than the general population, possibly because of
abnormality of AV node and SA node. Congenital cardiac
malformations that show a high frequency of persistent
LSVC are juxtaposition of right atrial appendage (34%), AV
canal defect (19%), mitral atresia (17%), and TOF (11%).
With persistent LSVC, there is increased association
of left-sided obstructive lesions and hypoplastic LV as
the dilated coronary sinus impinges on mitral inflow
during fetal life. An increase in the magnitude of left-toright shunt at the atrial level was found in patients with
associated ASD.
The presence of LSVC can be suspected on a chest
radiogram based on a shadow along the left upper border
of mediastinum.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1679

Echocardiography: 2D echocardiography in subcostal

coronal, apical four-chamber, and PLAX views show the
dilated coronary sinus as an oval structure, while subcostal
coronal, and apical four-chamber views in posterior
plane show the dilated coronary sinus in its whole length
(Figs 72.182A to C). Other causes of dilated coronary sinus
should be looked for if one does not find a LSVC, such as
anomalous pulmonary venous drainage to coronary sinus,
high right atrial pressure leading to passive congestion, and
increased LCA flow leading to increased coronary sinus
return. A dilated coronary sinus should be differentiated
from pericardial effusion, which is not continuous with RA
and inferior to the level of anterior mitral leaflet.
Suprasternal short-axis view with left tilt shows LSVC,
with or without the bridging innominate vein (Figs 72.183A
and B). Coronary sinus in this view is seen as a crescentshaped structure receiving LSVC. On color flow mapping,

flow is seen away from the transducer in contrast to the

vertical vein receiving pulmonary veins where flow is seen
toward the transducer.
In cases of poor echo windows, injection of agitated
saline in left arm delineates the LSVC and coronary sinus.

Figs 72.182A to C: 2D echocardiography in a case of left superior

vena cava (LSVC) to coronary sinus. (A) Apical four-chamber view
with posterior tilt shows a dilated coronary sinus; (B) Apical fourchamber view shows a dilated coronary sinus in short axis; (C)
Parasternal long-axis view showing the dilated coronary sinus
(CS). (CS: Coronary sinus; LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle).

Left Superior Vena Cava to Coronary Sinus

with Interatrial Communication and Defect in
Wall of Coronary Sinus (see Fig. 72.34)
This is a rare anomaly, known as Raghib syndrome. If a
localized defect is present in the wall of coronary sinus,
it is called partial unroofing of coronary sinus. More
marked deficiency leads to LSVC draining to LA, also
known as coronary sinus type of ASD. Here, the interatrial
communication is coronary sinus ostium, which is located
below and posterior to fossa ovalis. Rarely there may be

1680

Section 6: Congenital Heart Disease

Figs 72.183A and B: Two-dimensional echocardiography in suprasternal short-axis view with color compare showing a large left
superior vena cava (SVC) draining to coronary sinus. (CS: Coronary sinus; LSVC: Left superior vena cava).

associated fossa ovalis or ostium primum ASD. Depending

upon pressure difference between two atria, left-to-right
or right-to-left shunt occurs. The patient may present with
complication of right-to-left shunt with no significant
murmur, such as cyanosis, paradoxical embolism, brain
abscess, and stroke. Rarely, the ostium of coronary sinus
may be atretic, causing whole of the LSVC flow into the
LA. Rare cases of this in association with Lutembacher
syndrome have been reported.254
Echocardiography: 2D echocardiographic findings are
similar to LSVC drainage to coronary sinus. Special caution
should be taken to define integrity of the wall of coronary
sinus in case of cyanosis, because localized fenestrations
are difficult to detect. In these circumstances, contrast
echocardiography with saline injection in left arm shows
filling of coronary sinus and LA almost simultaneously.

Left Superior Vena Cava to Left Atrium

With completely unroofed coronary sinus, LSVC terminates
in LA generally between left atrial appendage anteriorly
and left upper pulmonary vein posteriorly. This anomaly
rarely occurs in isolation, and is usually associated with
heart defects like right isomerism.
Echocardiography: Subcostal coronal, PSAX, and
suprasternal short-axis views show connection of LSVC to
LA. On color flow mapping, flow is seen toward the heart
and away from the transducer. Contrast echo with saline
injection in left arm demonstrates connection of LSVC
to LA.

Absent Right Superior Vena Cava253,254

It is a rare anomaly of systemic veins and has been reported
in 0.1% of patients undergoing cardiac catheterization.
Either the right SVC is completely absent or represented by
a fibrous vestigial cord. The right innominate vein drains
to LSVC and then to coronary sinus. Embryologically, it is
due to complete involution of right cardinal vein.

Clinical Significance
Sinoatrial junction is poorly developed in these cases and
patients, usually adults, may develop sick sinus syndrome
requiring a pacemaker. Issues that make the diagnosis of
absent right SVC important include: (a) implantation of
transvenous pacemaker, (b) placement of transvenous
pulmonary catheter for monitoring particularly without
the usage of fluoroscopy, (c) systemic venous cannulation
for extracorporeal membrane oxygenation (ECMO),
(d) systemic venous cannulation for cardiopulmonary
bypass, (e) partial or total cavopulmonary anastomosis,
(f ) orthotopic heart transplantation, (g) endomyocardial
biopsies, and (h) cardiac catheterization particularly for
interventricular procedures.
Special precaution should be taken at the time of openheart surgery to avoid damage to area around coronary
sinus, and ligation of coronary sinus must be avoided.
Echocardiography: Suprasternal short-axis view is
especially important for determining whether right SVC is
present or not, in addition to profiling of LSVC to coronary

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

sinus. In cases with absent right SVC, systemic veins

appear to be mirror image of normal in suprasternal shortaxis view. Subcostal sagittal view shows absence of right
SVC connecting to RA.
Rarely, there may be absence of both the right and
LSVC, and blood from arms, head, and upper torso is
returned to the RA through the azygos vein and IVC
mimicking chronic SVC obstruction.

1681

Anomalous drainage of the SVC to the LA is an extremely

rare congenital cardiac malformation scantily reported
in the English literature. Patients with this condition

usually present with cyanosis, clubbing, easy fatigability,

features of polycythemia, and generally have no significant
findings on cardiac examination. Some patients present
with cerebrovascular accident. A common association
is anomalous drainage of right upper pulmonary veins
to the SVC1an anomaly that should be actively looked
for once anomalous RSVC drainage is diagnosed.
Echocardiography as described above and contrast
echocardiography are good modalities for the diagnosis;
rarely, when in doubt, one may consider CT scan for the
diagnosis. Surgical correction is warranted to prevent the
risks of chronic polycythemia and systemic embolization.
Surgery has had excellent short- and long-term outcomes.
About 23 cases have been reported since the date of this
anomaly.

Right Superior Vena Cava to Left Atrium

(Figs 72.184 and 72.185)

Figs 72.184A and B: Two-dimensional echocardiography. Subcostal sagittal view with color compare showing the drainage of right
superior vena cava (SVC; arrow) to left atrium. (LA: Left atrium; RA: Right atrium; SVC: Superior vena cava).

Fig. 72.185: Saline contrast study. Saline was injected into the
right brachial vein and contrast echoes appeared in the left atrium
followed by left ventricle. No contrast appeared in the right atrium
and right ventricle, clearly showing communication of superior
vena cava (SVC) to left atrium. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle; SVC: Superior vena cava).

1682

Section 6: Congenital Heart Disease

Aneurysm of Superior Vena Cava

It occurs due to inherent weakness in the structure of
venous wall. 2D echocardiography in subcostal sagittal
and suprasternal short-axis views can demonstrate the
aneurysmal dilatation of SVC.
It is important to exclude the causes of undue dilatation
of the SVC in cases like anomalous drainage of pulmonary
veins (TAPVC/PAPVC to SVC) or abnormal fistulous
communication to SVC.255

Retroaortic Innominate Vein (Fig. 72.186)

Abnormal course of left innominate vein beneath the
arch is named as retroaortic innominate vein and
embryologically it is due to persistence of lower venous
plexus between both anterior cardinal veins. Retroaortic
innominate vein joins right SVC below the insertion of
azygos vein.
Clinical importance: This anomaly is of no hemodynamic consequences but during surgery, mobilization of
right SVC is difficult. It should be especially visualized and
differentiated from right PA on echocardiography. Most
patients of retroaortic innominate vein have associated
congenital cardiac malformations including TOF, truncus
arteriosus, AVSD, heterotaxy syndrome, HLHS, pulmonary
atresia with intact ventricular septum, and CoA.
Echocardiography: Suprasternal short-axis view is
best to demonstrate retroaortic innominate vein. On 2D

Fig. 72.186: Two-dimensional echocardiography. Suprasternal

short-axis view with color flow mapping in a child with tetralogy
of Fallot showing laminar flow in retroaortic innominate vein. (Ao:
Decesnding Aorta; As Ao: Ascending aorta; Innom V: Innominate
vein; RPA: Right pulmonary artery).

echocardiography and color flow mapping, this is seen as

a horizontal channel crossing from left to right beneath
the transverse arch superior to right PA. At times it can be
mistaken for right PA. But on pulsed Doppler interrogation,
the flow pattern in innominate vein will be venous and
right PA will be arterial.

Abnormalities of Inferior Vena Cava

Inferior Vena Cava Interruption
Embryologically, it occurs due to failure of right subcardinal
vein to develop properly and to anastomose with vitelline
vein, resulting in enlargement of supracardinal system.
Incidence of IVC abnormalities is 0.6% in patients with
CHD, with 86% left isomerism, and it rarely occurs in
isolation or with right isomerism.
Clinical significance: This anomaly is of no physiological value but complicates cardiac catheterization,
cardiac interventions, and single ventricular repair.
Interrupted inferior vena cava with azygos continuation
(Fig. 72.187):256 There will be absence of intrahepatic segment of IVC. Hepatic veins connect directly to RA or form
a common hepatic vein to connect to RA. Venous return
from lower body is via azygos vein, which connects to right
SVC or hemiazygos which connects to innominate vein.
Echocardiography: Subcostal sagittal view at the level
of diaphragm shows anterior aorta, and a venous channel
posterior and on right side of the spine. Subcostal sagittal

Fig. 72.187: Two-dimensional echocardiogram. Subcostal sagittal

view with leftward tilt and color flow mapping showing an ascending venous channel (azygos continuation) posterior to aorta.
(Ao: Aorta; AZ: Azygos; RA: Right atrium).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

view shows ascending venous channel posterior to aorta

(see Fig. 72.187) and hepatic veins entering RA, and no
continuity is seen between hepatic vein and IVC.
Interrupted inferior vena cava with hemiazygos continuation: In this defect, the abdominal or hepatic portion of
IVC is absent, and hepatic veins connect to RA. Systemic
venous return from lower body returns via hemiazygos
vein, which connects to LSVC or coronary sinus or directly
to RA.
Echocardiography: Subcostal short-axis view at level
of diaphragm shows aorta anterior to spine, and venous
channel (hemiazygos vein) on left and posterior to the
spine.
Rarely, venous return from lower body is to both SVC
via azygos vein on right side and hemiazygos vein on left
side, and only hepatic vein continues to drain to the RA.

Inferior Vena Cava to Left Atrium

Embryologically, it is impossible for IVC to connect to the
morphological LA. It is the persistence of Eustachian valve
directing the IVC blood to LA through foramen ovale or
ASD. Clinically, the patient presents with features of rightto-left shunt. Rarely, with IVC type of sinus venosus ASD,
right atrial connection of IVC may get atretic leading to a
condition mimicking IVC connecting to LA.
Echocardiography: Subcostal sagittal view shows
inferior vena caval blood directed to LA or IVC directly
connecting to LA. Saline contrast injection in femoral vein
demonstrates filling of LA from IVC.
Clinical importance: Clinical importance of hepatic
veins connecting to LA lies in recognizing the condition
prior to Fontan or Kawashima procedure, as these may
lead to arterial hypoxemia and pulmonary arteriovenous
fistulas. The visualization can be done by echocardiography, angiography, CT scan, or MRI.

Bilateral Inferior Vena Cava

Bilateral venous systems contribute to the formation of the
normal IVC and thus can explain the possibility of bilateral
IVCs. Bilateral suprahepatic IVC are a frequent finding
in cases of visceral heterotaxy syndrome with asplenia.
These can rarely occur in normal visceral situs in which
case left-sided hepatic veins drain into a normal coronary
sinus. Bilateral infrarenal IVCs can occur in normal or
abnormal visceral situs. Their visualization is particularly
essential whenever contemplating the Fontan completion
or interventional procedures.

1683

Abnormalities of Coronary Sinus257,258

Partial or Completely Unroofed
Coronary Sinus
It manifests itself as LSVC to LA. Rarely there is no LSVC.

Atretic Coronary Sinus Ostium

Thin membrane-like tissue causes atresia of coronary
sinus ostium. There is an alternate exit for coronary sinus
blood to flow via LSVC, or at times an abberant connection
with IVC. If there is no alternate exit, then the condition
will be fatal because of myocardial infarction.
Echocardiography on color flow mapping in suprasternal short-axis and PSAX views shows retrograde flow in
LSVC (away from the heart and toward the transducer) if
LSVC exists with normal connecting pulmonary veins.

Anomalous Connection of Hepatic Vein to

Coronary Sinus
This rare anomaly is of no hemodynamic significance. On
2D echocardiography with color flow mapping, hepatic
venous flow to coronary sinus can be demonstrated in
subcostal coronal view in the posterior plane.
Clinical significance: It is characterized by progressive
and severe cyanosis after Fontan completion if not
recognized preoperatively.

Coronary Sinus Aneurysm/Diverticulum

This rare condition has been reported in up to 8 to 10%
of cases presenting with supraventricular tachycardia,
undergoing radiofrequency ablation. Gold standard for
the diagnosis is angiography, but diagnosis can be made
by echocardiography if aneurysm is not very small.
Echocardiography: Subcostal coronal and apical fourchamber views with posterior tilt show out pouching with
a distinct neck arising from coronary sinus.

Prominent Venous Valves

Venous Valves
Venous valves such as Eustachian and Thebesian are
prominent during fetal life and tend to involute after
birth. Persistence can be in the form of small remnants.
Pathological persistence of venous valves can cause partial
or complete division of RA, that is, venous sinus-accepting

1684

Section 6: Congenital Heart Disease

systemic veins and true RA (right atrial appendage and

vestibule of tricuspid valve), known as cor triatriatum
dexter. Right-to-left shunt may occur across foramen ovale.
There is high association with RV and PA hypoplasia in
these cases. The incomplete regression of the embryonic
right valve of sinus venosus may leave a fenestrated
or unfenestrated membrane in the RA that should be
considered a normal benign variant of the so-called Chiari
network.
Echocardiography in subcostal sagittal and coronal
views shows persistence of an echodense structure in
RA and on color flow mapping, right-to-left shunt across
foramen ovale may be seen. This anomaly needs to be
differentiated from right atrial myxoma. Sometimes a very
prominent Chiari network may cause difficulty during
atrial septal device closure.

Total Anomalous Systemic

Venous Drainage
It is an exceptional form of CHD scantily described in
the literature. All systemic venous flow, including the
RSVC, persistent LSVC, IVC, and coronary sinus drain
abnormally into the LA. This disorder is usually associated
with AV canal defects, common atrium, ASD, VSD, and
heterotaxy. It is a very rare disorder and a total of only
11 cases have been reported so far including three of our
unpublished cases. Surgical correction is often difficult
and complicated. Total anomalous systemic venous
drainage can be classified into two types; accordingly,
the type of vena cava cannulation will vary. In type I, the
IVC is not interrupted and conventional cardiopulmonary

bypass can be created. In type II, the IVC is interrupted,

and single cannulation of the SVC and conventional
cardiopulmonary bypass can be achieved. In this case,
hepatic veins can be cannulated with a small cannula after
excision of the atrial septum or an intracardiac extractor
is used to manage the blood flow. Most of these cases
including the three seen by us have been associated with
heterotaxy syndromes.

Decompressive Venous Channels

Levoatrial cardinal vein: This is a persistence of connection
of pulmonary veins to systemic veins, which occurs due
to severe left-sided obstruction and restrictive interatrial
communication; rarely does it occur in isolation. The
levoatriocardinal vein arises directly from LA or from a
pulmonary vein and connects to right SVC, innominate
vein, or jugular vein.
Echocardiography: Subcostal coronal PSAX, and suprasternal views can demonstrate the levoatriocardinal vein.
On color flow mapping, flow is seen away from the heart and
toward the transducer with normally connecting pulmonary
veins.
Decompressive venous channels are especially
common after Glenn shunt surgery for a univentricular
pathway. These can be suspected on echocardiography
and well profiled either by angiography, CT, or MRI. They
can be tackled either by catheterization (vascular plugs or
coils) or surgically. It is important to occlude the channel
before determining the pulmonary pressures in preFontan catheterization.

PART 10: IMAGING OF CORONARY AND PULMONARY ARTERIES

CORONARY ARTERY ANOMALIES
Coronary anomalies cause 10 to 19% of sports-related
deaths in athletes and 1.2% of nonsports-related deaths in
young individuals.259264 In 69% of the population, there is a
dominant RCA system, while in 11%, the LCA is dominant,
thereby giving rise to the posterior descending coronary
artery, and in 20% there is codominance.263 Interestingly, a
significant number of patients with bicuspid aortic valves
or aortic stenosis (2057%) have left-dominant systems
and a short left main coronary artery.265267 Also, separate
origins of the LAD and left circumflex coronary arteries
occurs in about 1% of population and is more frequent

with bicuspid aortic valves. Even though various coronary

anomalies cannot always be identified by echocardiogram,
it is imperative to identify the origin of coronaries on every
echocardiogram. Failure to identify normal coronary
origins from the mid-portions of the left and right coronary
sinuses will alert one to the possibility of an anomalous
coronary origin.
The origin and proximal course of the coronaries are
identified in the PSAX views recorded from a high left
parasternal window using a high frequency transducer
(Figs 72.188A to D). The origin of left main coronary
artery from the left coronary sinus, bifurcation of left main
coronary artery to LAD, and left circumflex and origin

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1685

Figs 72.188A to D: Two-dimensional echocardiography with color flow mapping showing normal coronaries. (A) Parasternal short-axis
view showing the right coronary artery (RCA) and the left anterior descending artery (LAD, arrow); (B) Color flow mapping showing the
course of LAD; (C) Parasternal short-axis view showing the origin of the right coronary artery (arrow); (D) Color flow mapping showing
right coronary artery (RCA). (Ao: Aorta; PA: Pulmonary artery; RVOT: Right ventricular outflow tract).

of the RCA from the right coronary sinus will be noted

in this view. On gentle clockwise rotation of the probe,
a longer length of the LAD artery and its branches will
be noted. Similarly, a gentle counterclockwise rotation
of the probe should be able to show a longer length of
the RCA.
Numerous coronary artery abnormalities are described
in normal as well as abnormal hearts. We will only focus
on clinically and significantly relevant abnormalities.

Anomalous Pulmonary Origin of

Coronary Artery268273
Anomalous origin of the left main coronary artery (the
LAD, circumflex branch) from PA (ALCAPA) or RCA

(ARCAPA) from the proximal PA or more distally from the

proximal right PA is a rare congenital anomaly with serious
consequences. In such cases, symptoms appear within a
few days of birth, and death may follow within a few weeks;
it is compatible with life only if associated with pulmonary
hypertension or significant collaterals from the other
coronary artery system.
The heart develops normally in fetal life. After birth, as
long as the pulmonary arterial pressure remains at or near
systemic levels, the left ventricular myocardium is supplied
by the anomalous artery and remains well perfused. As
pressure in the PA falls postnatally, perfusion of the LV
becomes inadequate, as the coronary artery pressure falls
below the left ventricle end-diastolic pressure (LVEDP).
The consequence of this circulatory handicap is a decrease

1686

Section 6: Congenital Heart Disease

of left ventricular function, due to myocardial ischemia.

At the same time, the ischemic myocardium is being
perfused increasingly by a developing set of collateral
vessels from the RCA, which are present normally. Those
with perfectly distributed collateral arteries before the PA
pressure falls escape with minor ischemic damage of the
left ventricular myocardium, which is well perfused, and
normal in form and function; this type is often diagnosed
in adolescence or adulthood. Any case of LV dysfunction
should be evaluated for this anomaly. It is important to
remember that any condition wherein the PA pressures
are high such as high pressure shunt lesions (VSD or

Fig. 72.189: Parasternal short-axis view showing a dilated right

coronary artery in a case of anomalous left coronary artery origin from the pulmonary artery. (Ao: Aorta; RCA: Right coronary
artery).

ductus) may not lead to the above picture, and in fact

may even show a normal coronary flow pattern on color
Doppler echocardiography. Thus, the diagnosis can be
easily missed if the origin of the coronary arteries is not
profiled properly leading to a catastrophic event after
closure of these defects.274,275 It is, therefore, imperative to
recognize the entity by imaging the coronary arteries on
2D echocardiography. Echocardiographic features of the
anomaly include:
Dilated RCA and absence of LCA from left sinus
(Fig. 72.189).
Anatomical delineation of the anomalous origin of LCA
from PA is done by 2D echocardiography in PSAX view
at the level of great vessels. The common site of origin
of LCA from PA is from the posterior-facing sinus. Very
rarely, the anomalous LCA may have its origin from the
other nonfacing anterior sinus of PA. Rare instances
of origin of LCA from main PA trunk or right PA have
been reported, often missed on echocardiogram
and diagnosed by other means. Echocardiographic
identification of the origin has a major relevance to
surgical strategy, since establishment of dual coronary
supply is easily achieved only if the coronary artery can
be mobilized to the aorta (Figs 72.190A and B).
Detection of left-to-right shunt in PA.
Color Doppler demonstration of reversal of flow from
LCA (Figs 72.190A and B) to PA.
Left ventricular dilatation and reduced systolic
function; surgical risk stratification depends on left
ventricular function.

Figs 72.190A and B: Two-dimensional echocardiography. (A) Shows the origin of the left main coronary artery (marked by the arrow)
from the pulmonary artery; (B) Color flow mapping shows flow reversal in the coronary artery with left anterior descending artery (LAD)
showing blue color flow signals indicative of flow toward the pulmonary artery in a case of anomalous origin of left coronary artery from
pulmonary artery (ALCAPA). (Ao: Aorta; PA: Pulmonary artery).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1687

Figs 72.191A and B: Two-dimensional echocardiography. Apical four-chamber view showing the sclerosed papillary muscle (arrow)
in a case of anomalous left coronary artery from pulmonary artery; (B) Color flow mapping showing mild mitral regurgitation. (LA: Left
atrium; LV: Left ventricle).

Ischemic mitral regurgitation (Figs 72.191A and B). In all

infants and children with unexplained left ventricular
dilatation and mitral regurgitation, anomalous origin
of LCA from PA should be considered as a possible
etiology and actively excluded.
Scarring of papillary muscles of the LV as a result of
myocardial ischemia, which acts as a very strong clue
in infants with severe left ventricular dysfunction to
the etiology for the myocardial dysfunction.
Color Doppler demonstration of myocardial collateral
flow in the interventricular septum through septal
collaterals from RCA to LAD artery (Fig. 72.192).

Anomalous Origin of Right Coronary

Artery from Pulmonary Artery276278
Right coronary artery (RCA) origin from the pulmonary
trunk (ARCAPA) is rarer than anomalous origin of
LCA from the PA. These patients can present with left
ventricular dysfunction at a later age as compared to
patients of ALCAPA (Fig. 72.193). The coronary flow to
the interventricular septum is compromised. In infants
and children presenting with left ventricular dysfunction,
the origin of RCA should also be looked for carefully. If
it is not seen from the aortic sinuses, its origin from the
PA is a possibility. The other clue could be the presence
of collateral flow in the interventricular septum with
normal LCA.
Various coronary abnormalities of coronary origin
have been described.279-287 Few important ones are discussed here.

Fig. 72.192: Two-dimensional echocardiography. Modified parasternal short-axis view with color flow mapping in a case of
anomalous left coronary from pulmonary artery showing a dilated
right coronary artery (RCA; arrow) with multiple collaterals from
right coronary artery (arrow). (Ao: Aorta).

Tangential Origin of Coronary Artery

Normal coronary artery origin is from the mid-portions
of the left and right coronary sinuses of the aorta. The
coronaries are given off perpendicular to the aortic wall. In
some cases, coronary arteries can arise from other areas,
namely high origin or commissural origin, arising from
the noncoronary sinus, or arising tangentially instead
of perpendicularly. The tangential origin is associated
with a slit-like coronary orifice. This can be suspected
on careful echocardiographic profiling of coronary
origin in PSAX view using a high frequency transducer.

1688

Section 6: Congenital Heart Disease

Fig. 72.193: Two-dimensional echocardiography. Parasternal

short-axis view showing anomalous origin of the right coronary
artery from the pulmonary artery (arrow). (Ao: Aorta; PA: Pulmonary artery).

Pathologists consistently observe a fibrous ridge at the

ostium of tangentially oriented ectopic coronary arteries.
Such ridges are often said to have potentially catastrophic
consequences. Nevertheless, plaque activation or
rupture is seldom documented, even on examination of
the histological anatomy. High origin and commissural
origin of coronary arteries are also sometimes associated
with the same anatomy. In these anomalies especially
high origin of coronary arteries above the aortic sinuses,
abnormal fluid dynamics have been documented to cause
significantly attenuated blood flows.

CORONARY ARTERIOVENOUS
FISTULA (FIGS 72.194A TO C)
Coronary arteriovenous fistulas are present in 1 of 50,000
live births (0.002% of the general population) and are
visualized in 1 of 500 patients undergoing catheterization
(0.20.25%).288,289 The etiology of these lesions may be
congenital or acquired; the latter may be broken down
into infectious, traumatic, and iatrogenic. Iatrogenic
causes may be further subdivided as after surgery,
catheterization, angioplasty, or endomyocardial biopsy.
We will be discussing congenital coronary arteriovenous
fistulae only.
Coronary arteriovenous fistula involves RCA in 60%,
LAD artery in 25%, and left circumflex artery in 15% of
patients. Rarely it involves more than one coronary artery.

The common sites of drainage are RV in 30%, RA in 25%,

and PA in about 20%, rarely into LA, LV, coronary sinus, or
SVC. The termination may be a single or multiple orifices.
Echocardiographic features of coronary arteriovenous
fistula include (a) dilated proximal coronary artery, (b)
continuous color Doppler flows with high velocity rather
than normal velocity diastolic flows seen in normal
coronary arteries, (c) delineation of the course of the
fistula to its termination, unless it is tortuous when it may
not be possible to track it fully, (d) high-velocity turbulent
flow at the site of drainage, and (e) volume overload of
cardiac chambers depending on the magnitude of left-toright shunt through the fistula. Rarely, the fistula opens
into a large sac (fifth cardiac chamber), which opens into
one of the cardiac chambers. Echocardiography provides
good details of fistula in many patients. Angiographic
classification of surgical relevance is proposed by
Sakakibara.288 Type A (proximal type)proximal coronary
segment dilated to the origin of fistula, distal end normal;
and type B (distal type)coronary artery dilated over
its entire length, terminating as a fistula in the right side
of the heart.

CORONARY ANEURYSMS
Aneurysms are defined as dilations of a coronary vessel
1.5 times the adjacent normal coronaries. The aneurysm
can be saccular or fusiform, fusiform being the most
common. Aneurysms may be congenital or acquired,
the latter may be further subdivided into atherosclerotic,
Kawasaki disease, traumatic, iatrogenic (surgical, after
angioplasty, catheterization, or endomyocardial biopsy),
infectious, and systemic diseases (polyarteritis nodosa,
syphilis, EhlersDanlos syndrome, Marfan disease, and
scleroderma).

Kawasaki Disease
Kawasaki disease is the commonest disease associated
with coronary aneurysms in the pediatric age group.
Moderate coronary involvement with aneurysms is present
in 12.8 to 25% of patients with untreated Kawasaki disease;
the incidence of coronary involvement reduces to one
fifth after timely intravenous gamma globulin therapy. The
following definitions pertaining to Kawasaki disease are
generally accepted in the literature: segmental stenosisa
braid-like lesion with multiple tortuosities of the vessel,

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1689

Figs 72.194A to C: A 2-year-old child with coronary arteriovenous

(AV) fistulae from right coronary artery (RCA) to right atrium (RA).
(A) Parasternal short-axis view showing a dilated right coronary
artery (arrow) at its origin; (B) Apical four-chamber view with posterior tilt showing the dilated and aneurysmal right coronary artery
in the atrioventricular (AV) groove (arrow); (C) Apical four-chamber
view showing the aneurysmal RCA with small opening (arrow)
in the right atrium. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
PA: Pulmonary artery; RA: Right atrium; RV: Right ventricle).

which represents recanalization of an occlusion; localized

stenosisa discrete, wedge-like narrowing at the inlet
or outlet of an aneurysm; ectasia1.5 times larger than
normal adjacent coronaries; dilation/small aneurysm
up to 3 mm ( irregular lumen); aneurysms4 to 8 mm;
and giant aneurysmsgreater than 8 mm.
On echocardiography, proximal parts of left main,
LAD artery, left circumflex, and RCA can be defined from
parasternal short- and long-axis view at the level of great
vessels as described earlier. Apical four-chamber view
with posterior tilt to the right side defines the distal part of
RCA in right AV groove and to left side profiles circumflex

artery in the left AV groove. The circumflex artery can also

be profiled from long-axis view with posterior tilt and
subcostal five-chamber view.

Atresia of Left Main Coronary Artery290

Atresia of left main coronary artery with lack of luminal
continuity from aortic root to LCA is associated with
collaterals from the RCA through septal branches. This
mimics the septal collaterals of anomalous origin of LCA
from PA on echocardiography, but there will be no reverse
flow pattern seen in LCA and no left-to-right shunt in PA .

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Section 6: Congenital Heart Disease

PART 11: ECHOCARDIOGRAPHIC EVALUATION OF AORTIC ARCH AND ITS ANOMALIES

Aortic arch anomalies constitute an important subgroup
of cardiac lesions, which may occur in isolation or in
association with other intracardiac defects. The thrust in
the present century is on precise, noninvasive imaging of
the aortic arch and its principal branches in order to plan
timely appropriate treatment.
Echocardiography is an important noninvasive diagnostic modality in diagnosing, monitoring the progression
of lesions, and planning timely intervention and followup after intervention in these lesions. The suprasternal
notch291 views are the most important echocardiographic
views in diagnosis of aortic arch anomalies. In neonates
and infants, subcostal, high parasternal and apical views
can also provide imaging of the aortic arch, but these
views permit imaging only up to the origin of first branch
and these windows are inadequate in older children and
adults. PLAX and short-axis views can image only the
proximal aorta and hence have limited usefulness.
2D echocardiographic imaging of the arch: The
arch of aorta is best imaged from suprasternal view.291
For the suprasternal views, the transducer is placed in
the suprasternal notch and aligned as closely parallel
as possible with the sternum. In order to gain access to
the suprasternal notch, the patient is positioned supine
with a pillow beneath the shoulders to extend the neck
without producing tension on the sternocleidomastoid
muscles. The patients head is turned to the left or right
so that the chin does not prevent adequate placement of
the transducer in the suprasternal notch. The ascending
aorta, transverse, and descending thoracic aorta are
best visualized in the suprasternal long-axis view. To
obtain good suprasternal long-axis view, the transducer
is placed in the suprasternal notch with the plane of
ultrasound beam oriented between the right nipple and
the left scapular tip. Suprasternal short-axis view is used
to define the branching pattern and side of arch. To obtain
suprasternal short-axis view, the transducer is placed in
horizontal position in suprasternal notch and should be
turned to right to define bifurcation of first arch vessel with
left aortic arch, and to left with right aortic arch.
Aortic arch anomalies can be classified as follows:
Abnormal formation of aortic arch
Coarctation and its most severest form, that is,
interrupted aortic arch
Aortic aneurysms.

ABNORMAL FORMATION OF ARCH

The subgroup of aortic arch abnormities resulting from
abnormal formation of aortic arch includes:
Mirror image right-sided aortic arch
Vascular rings
Cervical aortic arch
Double aortic arch.

Right Aortic Arch292297

Left and right aortic arch refer to which bronchus is
crossed by the arch, not to which side of the midline the
aortic root ascends. Practically, the sidedness of the aortic
arch is determined indirectly with echocardiography
or angiography by the branching pattern of the
brachiocephalic vessels. As a rule, the first arch vessel
gives rise to the carotid artery opposite the side of the
arch. The very rare cases of retroesophageal or isolated
innominate artery are exceptions to this rule. But by
far the more common source of error in the use of this
rule is the difficulty in deciding which of the two carotid
arteries the first one is. A more reliable rule but one that
may be difficult to apply with ultrasound imaging is that
retroesophageal vessels or isolated vessels, that is, arising
only from a ductus or ligamentum (without connection to
the aorta), are always opposite the side of the aortic arch.
The incidence of right aortic arch among patients
with tetralogy of Fallot has been reported to be anywhere
from 13 to 34%.292 The incidence in truncus arteriosus is
generally higher than in tetralogy. An overall incidence of
8% in patients with d-TGA with intact septum, and 16% in
those with transposition, VSD and pulmonary stenosis has
been reported.293
The normal left-sided arch is viewed in suprasternal
long-axis view with the plane of ultrasound beam directed
between the right nipple and left scapular tip with the first
branch being the right innominate artery, which bifurcates
into right subclavian and right carotid artery. The diagnosis
of right-sided arch is suspected when the transducer has to
be rotated counterclockwise between the left nipple and
right scapular tip in order to visualize the arch. Tilting the
transducer anteriorly and posteriorly from suprasternal
long-axis view images the relation of transverse aorta with
the tracheal rings and tracheal air column, that is, in right
aortic arch the transverse aortic arch is seen to the right

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

of trachea. Right-sided aortic arch is further confirmed

if the first vessel courses leftward and then bifurcates,
which is seen in suprasternal short-axis view. If the first
arch vessel courses to left but does not bifurcate, then
there are chances of more complex malformations being
present such as an aberrant left subclavian artery as well
as increased chances of vascular ring formation.

1691

A vascular ring is an aortic arch anomaly in which the

trachea and esophagus are completely surrounded by
vascular structures. The vascular structures need not be
patent. Vascular rings are formed when an aortic arch
abnormality forms a ring of tissue encircling the trachea
and esophagus. The presence of vascular ring anomaly
is highly probable if the suprasternal views demonstrate
arch anatomy other than a left arch with a normal right
innominate artery or a right arch with mirror image
branching. These rings are recognizable by the presence
of one of three ds opposite the side of the aortic arch:
diverticulum, dimple, or descending aorta. A diverticulum
is a large vessel arising from the descending aorta that
gives rise to a smaller caliber vessel with a sudden taper.
A dimple is a tapered, blindly ending out pouching from
the aorta. Descending aorta opposite the side of the aortic
arch refers to its location in the upper thorax. These three
ds form the vascular ring only when connected by a

ligamentum arteriosum or an atretic segment of aortic

arch which cannot be profiled by 2D echocardiography.
The common type of vascular ring anomalies include:
Left aortic arch with aberrant right subclavian artery
(Fig. 72.195) and right duct or ductus ligament
Right aortic arch with aberrant left subclavian artery
and left duct or ductus ligament
Right aortic arch with retroesophageal segment and
left descending aorta, and
Double aortic arch.
On echocardiography, diagnosis of left aortic arch with
aberrant right subclavian or right aortic arch with aberrant
left subclavian is possible if there is failure to demonstrate
bifurcation of the first arch vessel. Demonstration of origin
of fourth arch vessel, that is, aberrant subclavian is difficult
by echocardiography, and definition of detailed anatomy
requires spiral CT or MRI. Double aortic arch can be
directly visualized by performing a standard long-axis view
and rotating the transducer 30 to 45 counterclockwise
(Fig. 72.196). In suprasternal short-axis view, evidence of
a double circle with tracheal ring in center is suggestive of
double aortic arch. In some cases, even subcostal views
may demonstrate aorta bifurcating into two arches a few
centimeters above the aortic valve. Demonstration of both
arches requires use of views for left and right aortic arch.
Usually, one arch dominates and the other is hypoplastic
or atretic. Origin of arch vessels should be defined from
suprasternal views.

Fig. 72.195: Two-dimensional echocardiography in suprasternal

long-axis view showing the aberrant right subclavian artery
(arrow) arising distal to the origin of the left subclavian artery. (Ds
Ao: Descending aorta; TA: Transverse arch).

Fig. 72.196: Two-dimensional echocardiography. Suprasternal

short axis in a case of double aortic arch showing the right and left
components of the arch. (R: Right component of the double aortic
arch; L: Left component of the double aortic arch).

Vascular Rings

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Section 6: Congenital Heart Disease

Cervical Aortic Arch298301

In this anomaly, the arch is found above the level of the
clavicle. There are two main subcategories of cervical arch:
Those with anomalous subclavian artery and vascular
ring, with either descending aorta contralateral to the
arch or retroesophageal diverticulum.
Those with a virtual normal branching pattern.
Cervical aortic arch presents as a pulsatile mass and
may be identified from the suprasternal long-axis view. It
requires placement of transducer onto the neck over the
pulsatile mass, and suprasternal long-axis view will then
show a long ascending aorta.

of coarctation anatomy in most patients. High quality ultrasound images can be obtained in infants but may be somewhat difficult to obtain in larger children and adolescents.

CoA is narrowing of the aorta, most commonly at the

junction (isthmus) of the arch of aorta and descending
thoracic aorta. CoA occurs in approximately 6 to 8% of
patients with CHD.301 True CoA results from a localized
thickening of aortic media, which protrudes into the
lumen of the aorta from the posterior and lateral walls
and obstructs blood flow (Figs 72.197A and B). Although
usually a discrete lesion, coarctation may consist of a
long stenotic segment or tubular hypoplasia. Very rarely,
the narrowing may be located in the abdominal aorta
(Fig. 72.198).
2D echocardiography and Doppler studies (Figs
72.199A and B) provide an accurate, noninvasive assessment

Echocardiographic objectives:
Is there coarctation?
Is there discrete stenosis or long segment stenosis?
Is there arch hypoplasia?
Branching pattern of arch.
Are there associated cardiac anomalies, in particular,
bicuspid aortic valve, left-sided obstructive lesions
such as aortic stenosis, subaortic membrane, mitral
valve disease, or hypoplastic left heart?
Left ventricular hypertrophy.
Ventricular function.
The echocardiographic examination using the suprasternal long-axis view provides an image of the entire arch,
with the area of coarctation seen near the origin of left
subclavian artery, that is, juxtaductal CoA (Figs 72.200A
and B). On 2D echocardiography, it is seen as a prominent
posterior shelf with significant coarctation. When imaging
the arch, one must be absolutely certain that the entire arch
is imaged, particularly in the region of the left subclavian
artery. The diagnosis can be missed with inadequate
imaging in this area.
Type of coarctation may either be a long segment
narrowing or, more commonly short segment obstruction
caused by posterior endothelial shelf projecting into
the aorta (Figs 72.197A and B). There may be associated

COARCTATION OF AORTA (CoA)302,303

Figs 72.197A and B: Two-dimensional echocardiography with color compare. (A) Suprasternal long-axis view with color compare in a
child of coarctation of aorta showing tiny posterior (arrow) shelf with flow acceleration; (B) Suprasternal long-axis view with color compare in another case of coarctation of aorta showing a prominent posterior shelf (arrow) with significant turbulence in the arch starting
from the coarctation region. (As Ao: Ascending aorta; Ds Ao: Descending aorta; TA: Transverse arch).

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1693

Fig. 72.198: Arch measurements. Suprasternal long-axis view of

the ascending aorta and aortic arch showing the measurements to
be made. (A: Ascending aorta; B: Transverse arch; C: Isthmus; D:
Narrowest segment; E: Descending aorta).

Figs 72.199A and B: Continuous wave Doppler signals across the coarctation of aorta. (A) Demonstrates mild coarctation of aorta
with early diastolic spill and a gradient of 31 mm Hg; (B) Shows severe coarctation of aorta with pan-diastolic flow and a gradient of
61 mm Hg.

Figs 72.200A and B: Two-dimensional echocardiography. Suprasternal long-axis view showing interruption of the aortic arch distal to
the left subclavian artery with arch hypoplasia (arrow); (B) Represents suprasternal long-axis view showing interruption of the arch of
aorta (arrow) between the carotid arteries (type C). (As Ao: Ascending aorta; Ds Ao: Descending aorta; TA: Transverse arch).

1694

Section 6: Congenital Heart Disease

hypoplasia of transverse arch. In the region of the ductus

arteriosus, an anterior shelf may normally exist just
proximal to the aortic origin of ductus. It is important not
to mistake this for the coarctation shelf. The measurements
which should be taken in a case of coarctation are shown
in Figure 72.198.
Certain intracardiac findings suggest the possibility
that a CoA may exist before the arch is imaged. Left
ventricular outflow obstructive lesions, significant right or
left ventricular hypertrophy without obvious explanation,
and the absence or reduced pulsation in descending
aorta as imaged from the subcostal window suggest the
possibility of CoA.

Doppler Feature of Aortic

Coarctation303311
Doppler echocardiography can assist in determining the
hemodynamic severity of coarctation. First, color flow
imaging of the descending aorta is performed, looking
for a high-velocity (turbulent flow) jet in the region of
coarctation. Once such a jet is imaged on color Doppler
examination, the continuous wave Doppler beam is
directed into the area of turbulent flow (Figs 72.199A and
B). The Doppler recording usually shows a high velocity
jet with antegrade flow extending well into diastole. This
flow pattern is characteristic of severe obstruction in a
vessel with a pressure gradient extending into diastole.
As the severity of obstruction increases, the pressure
above the coarctation remains elevated for a longer
period of the cardiac cycle, and thus, the jet extends into
the entire diastole. From the peak velocity of the jet (V2)
and the simplified Bernoulli equation (P = 4V22), the peak
instantaneous pressure gradient across the coarctation
can be calculated. Many patients with CoA have multiple
left heart obstructive lesions (e.g. bicuspid aortic valve,
valvular aortic stenosis, subaortic membrane, etc.) that
can lead to an increased peak flow velocity proximal to
the coarctation jet. If the peak velocity proximal to the
coarctation (V1) exceeds 1 m/s recorded by pulsed Doppler
echocardiography from suprasternal long-axis view, then
the expanded Bernoulli equation (P = 4[V22 V12]) should
be used to avoid a significant overestimation of the peak
gradient. Even then the gradient across the coarctation
may be unreliable. The gradient depends on the shape and
length of the coarctation segment, the patients cardiac
output, and the presence and extent of collateral flow.
Thus, the gradient can over- or underestimate the severity
of coarctation. If the left ventricular ejection fraction is low,
there can be underestimation of severity of coarctation
by Doppler. In such cases, a slow dP/dt of the systolic

signal, diastolic spill, lumen size at the coarctation site, left

ventricular hypertrophy, and PA pressures help in the final
diagnosis.
The coarctation area can also be profiled from high
PSAX view with counterclockwise tilt. This view opens up
the coarctation area and descending aorta below the left
subclavian artery.
In patients with inadequate suprasternal widow, pulsed
Doppler echocardiographic recordings from the proximal
abdominal aorta can be useful. It shows a continuous
antegrade flow signal with no evidence of flow reversal or
cessation, the time of peak velocity is prolonged, and the
mean acceleration rate is decreased.

INTERRUPTION OF AORTIC ARCH

Arch interruption represents the most severe form of
coarctation, which can be demonstrated from suprasternal
views.312,313 Interruption of arch of aorta is generally
associated with complex CHDs like AV canal defect,
d-transposition of great arteries with or without tricuspid
atresia, TaussigBing anomaly, and congenitally corrected
transposition apart from VSD, PDA, AP window, subaortic
stenosis, bicuspid aortic valve, and mitral stenosis. The
interruption of arch of aorta may involve only a short
segment or a long segment with a long distance between
the proximal and distal segments of the aorta. Celoria
and Patton312 classified them as type A if the interruption
was distal to the left subclavian artery, type B if between
carotid and subclavian arteries, and type C if between
carotid arteries. However, these types may be further
subcategorized313 and definitions generalized to include
both right and left arch patterns as in Table 72.16.
Type B arch interruption is the most common form
and is usually associated with conotruncal anomalies
and normally aligned great arteries in which there is a
large malalignment type of VSD associated with posterior
displacement of the infundibular septum and subaortic
obstruction. Type B interruption is also commonly seen in
association with DiGeorge syndrome. Type A interruption
tends to occur with aorticopulmonary septal defect and
intact ventricular septum and in a large group of patients
with transposition of great arteries.
On 2D echocardiography, suprasternal views show
ascending aorta continuing to at least one of the arch
vessel. In type A arch interruption, all three branches are
seen proximal to interruption; in type B interruption, first
and second branches are seen proximal to interruption;
and in type C interruption, only the first branch, that is,
innominate artery is seen arising proximal to interruption.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1695

Table 72.16: Classification of Interrupted Aortic Arch

Type A: Interruption distal to the left subclavian artery

Without retroesophageal or isolated subclavian artery

With retroesophageal subclavian artery

With isolated subclavian artery

Type B: Interruption between second carotid and ipsilateral subclavian artery

Without retroesophageal or isolated subclavian artery

With retroesophageal subclavian artery (i.e. both carotid arteries proximal, both subclavians distal)

With isolated subclavian artery

Type C: Interruption between carotid arteries

Without retroesophageal or isolated subclavian artery

With retroesophageal subclavian artery

With isolated subclavian artery

Descending aorta is relatively dilated with ductal continuation to descending aorta, which is well profiled in
suprasternal long-axis and high PSAX views. Care should
be taken to ensure that the ductus arteriosus connecting the
main PA to descending aorta (ductal arch) is not mistaken
for the true arch. Color flow mapping confirms the findings
and as systemic flow is duct-dependant, patency of duct
or any restriction of duct should be defined by color flow
mapping. Pulsed Doppler is used to assess the gradient
across the ductus. Sometimes, restriction of ductus
arteriosus can mimic CoA, and that should be clearly
defined. In patients with suprasystemic PA pressures, there
can be turbulent right to left flow across the ductus.

AORTIC ANEURYSM
The third important subgroup of aortic arch anomalies
includes aneurysms of the aorta. Aneurysm of a vessel
is defined as a dilated segment > 50% in diameter as
compared to the proximal segment. In pediatric age
group, in contrast to adults, aneurysm of ascending aorta
is more common than descending aorta. Annuloaortic
ectasia is defined as aneurysmal involvement of annulus
and aortic root, in addition to ascending aorta. In children,
aneurysmal dilatation of proximal aorta can be caused by
conditions associated with medial degeneration of the
aorta such as Marfan syndrome, EhlersDanlos syndrome,
Turner syndrome, in association with bicuspid aortic valve,
and idiopathic; however, some types of infectious disease
like bacterial endocarditis can also result in aneurysm
formation. Aneurysm of proximal aorta is usually easily

recognized from the parasternal long-axis, PSAX, subcostal

coronal, and suprasternal long-axis views. Aneurysmal
involvement of descending aorta usually occurs secondary
to previous surgical or catheter intervention such as
surgical repair of CoA or balloon dilatation of coarctation.
Chances of aneurysmal formation are much less after stent
deployment for CoA. Descending aorta aneurysm can be
defined from suprasternal long-axis, suprasternal shortaxis (aneurysm from lateral wall), and subcostal sagittal
views in infants and children. The echocardiographic
measurements are helpful in diagnosing and assessing
the progression of the disease. Besides visualization of the
aneurysm, echocardiographic examination should also
focus on detecting complications of aneurysms such as
compression of adjacent structures and fistula formation.
Transthoracic echocardiography is a useful modality
in diagnosis, monitoring, and planning appropriate nonsurgical/surgical management and in postintervention
follow-up of these patients. Transthoracic echocardiography has limitations in grown-up children because of
suboptimal acoustic windows and in the postintervention
period. In such patients, transesophageal echocardiography spiral CT, or MRI can demonstrate these lesions.

Anomalous Origin of Branch

Pulmonary Artery from Ascending
Aorta (Fig. 72.201)314,315
Anomalous origin of one PA from ascending aorta used to be
known as hemitruncus (This terminology is not used now).

1696

Section 6: Congenital Heart Disease

Echocardiographic diagnosis of origin of PA from

ascending aorta depends upon demonstration of:
Absence of normal bifurcation of main PA
RVOT continuing as one PA
Origin of one PA from ascending aorta.
Subcostal coronal view with anterior tilt, PLAX view,
and suprasternal long-axis and short-axis views show
origin of a branch pulmonary artery (PA) from ascending
aorta. PSAX view profiles main PA continuing as one PA
and with change in the plane of ultrasound, origin of
another PA from ascending aorta can be profiled. This is
differentiated from truncus arteriosus, by demonstrating
two separate semilunar valves, and from APW by
demonstrating PA bifurcation in the latter defect.
Fig. 72.201: Two-dimensional echocardiography in modified
suprasternal short-axis view showing the origin of the left
pulmonary artery from the ascending aorta. (Ao: Aorta; LPA: Left
pulmonary artery; RPA: Right pulmonary artery).

This anomaly needs to be differentiated from APW, truncus

arteriosus, and discontinuous pulmonary arteries with one
PA supplied from ductus or a collateral, as in the setting of
VSD with pulmonary atresia. By far, the more common form
is anomalous origin of the right PA, seen in 82% of 108 cases
excellently reviewed by Kutsche and Van Mierop.314 The
anomalous right PA usually arises from the posterior aspect of
the ascending aorta close to the aortic valve. Less commonly,
it originates from the lateral ascending aorta just proximal
to the innominate artery. PDA and aorticopulmonary septal
defect are commonly associated with anomalous origin
of the right PA; other cardiovascular anomalies are rare. In
contrast, TOF and aortic arch anomalies, for example, right
aortic arch and anomalous origin of the subclavian artery, are
common in anomalous origin of the left PA. An association
with DiGeorge syndrome, frequently noted with persistent
truncus arteriosus, is not seen with anomalous origin of a PA
from the ascending aorta.

Pulmonary Artery Sling316318

Pulmonary artery sling is a rare anomaly in which the left PA
arises from right PA and passes posteriorly between trachea
and esophagus to the left side. Due to this course, left PA
forms a sling, encircling trachea, anteriorly main PA, right
and posteriorly left PA, and on the left ductus arteriosus or
ductus ligament. PA sling compresses the right bronchus
and trachea from the posterior aspect as it courses to left and
posterior after its origin from right PA. The child presents with
respiratory distress and stridor in infancy.
This anomaly should be looked for in infants presenting
with stridor and respiratory distress. The anomaly could
first be suspected if PSAX view does not show normal
main PA bifurcation, main PA seems to continue as right
PA , and on tilting the transducer inferiorly and to left,
left PA can be profiled arising from right PA . After origin,
left PA passes posterior to echogenic shadow of trachea.
This finding can also be defined from high PSAX view
and modified suprasternal short-axis view. With the use
of color flow mapping, it becomes easier to define the
anomaly. Associated defects such as VSD, ASD, and TOF
can occur in 40% of cases of PA sling and should be looked
for on echocardiography.

PART 12: UNIVENTRICULAR HEART AND HETEROTAXY SYNDROME

INTRODUCTION
Univentricular heart means a cardiac malformation in
which a biventricular repair is not feasible and hence these
hearts will be subjected to the surgical univentricular
pathway, where the systemic venous return will be

directed to the pulmonary circulation either partially

(bidirectional Glenn shunt) or totally (modified Fontan
surgery). As per the unified reporting system of the Society
of Thoracic Surgeons-Congenital Heart Surgery Database
Committee, this includes the following.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Hearts unsuitable for biventricular correction are:

Univentricular AV connections
Tricuspid atresia
Mitral atresia and HLHS
Pulmonary atresia with intact ventricular septum
Heterotaxy syndromes
Cardiac malpositions
DORV with nonroutable VSD.

UNIVENTRICULAR ATRIOVENTRICULAR CONNECTIONS319-324

The unifying feature of hearts previously described
as single, common, or univentricular is that the AV
connection is completely or predominantly to a single
ventricular chamber. Two basic situations may be present
in univentricular physiology:
Both AV valves are committed to one ventricular
chamber.
There may be only one AV valve permitting the access
of only one atrium to the dominant ventricle (with the
second ventricle being rudimentary).
The literature has varying names for these hearts,
namely cor triloculare biatriaum, cor biloculare, single
ventricle, common ventricle, and univentricular heart.
This group is subclassified into three groups based on the
AV valve connections:
Double inlet ventricle (Fig. 72.202): Common form of
single ventricle where there are two patent AV valves.

Fig. 72.202: Two-dimensional echocardiography. Apical fourchamber view showing the two inlets into a ventricle with left ventricular morphology in a case of double inlet left ventricle (DILV).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium).

1697

Single inlet ventricle: One of the AV valves is atretic

and not patent. In this entity, either the left or the right
AV valve is atretic and it is better to use the term left
AV valve atresia or right AV valve atresia rather than
tricuspid and mitral atresia (Fig. 72.203).
Common inlet ventricle: In this case, the AV connection is through a common AV valve; this condition is
commonly seen in heterotaxy syndromes (Figs 72.204A
and B). The AV valve has free-floating leaflets and is
classified as Rastelli type C AVSD.

Double Inlet Ventricle

The classic form of single or common ventricle described
as double inlet ventricle has been classified by Van Praagh
into four types:
Double inlet LV
Double inlet RV
Double inlet ventricle of mixed morphology
Double inlet ventricle of indeterminate or undifferentiated morphology.
The echocardiographic recognition of a double
inlet ventricular with left or right morphology is based
on two well-defined morphological principles. The
first morphological principle states that left ventricular
myocardium typically has a relatively smooth appearance
with numerous fine oblique trabeculations, whereas
RV myocardium has an irregular surface with relatively
coarse straight trabeculations. The second principle states

Fig. 72.203: Two-dimensional echocardiography. Apical fourchamber view showing in a case of tricuspid atresia with hypoplastic right ventricle (RV), a large atrial septal defect (arrow) and
a small muscular ventricular septal defect (VSD; star). (LA: Left
atrium; LV: Left ventricle; RV: Right ventricle; RA: Right atrium).

1698

Section 6: Congenital Heart Disease

Figs 72.204A and B: Two-dimensional echocardiography. (A) Apical four-chamber view showing complete atrioventricular septal defect
(AVSD) with a nonapex-forming small left ventricular (LV) cavity not suitable for biventricular pathway; (B) Shows a common AVSD
(unbalanced type) with hypoplastic right ventricle (RV) on a single ventricular pathway. (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).

that the ventricular chamber that has an infundibulum

giving rise to a great artery whether it represents the main
ventricle or the hypoplastic outflow chamber represents
the morphological RV. As a corollary, the ventricular
chamber having a direct arterial connection without an
intervening infundibulum represents a morphological LV.
A good echocardiographic view of the foramen
(bulboventricular foramen) separating the main ventricle
into which both the AV valves connect with the outlet
chamber should be obtained by using subcostal and
parasternal windows. The differentiation of ventricular
morphology as smooth surface versus irregular surface
is also best made on these views. A careful look at either
side of the bulboventricular foramen, (one side of which is
main ventricle and other side is the rudimentary chamber)
will show which of the sides has rough trabeculations and
which side has a relatively smooth surface and this will
define the main chamber and the rudimentary chamber
as either the morphological right or the morphological
left.
Identifying on echo the infundibular chamber, which
always goes with morphological RV, is important. A
patent infundibulum always gives off a great artery and
echocardiographic identification of an infundibulum is
done by noting the separation from the AV valves, namely
the AV valvesemilunar valve fibrous discontinuity. So a
hypoplastic subarterial outlet chamber noted on echocardiography is indicative of morphological RV. If the
rudimentary chamber is a blind chamber located inferiorly

and not giving off any great arteries, it is morphologically

the LV.
To restate the above echocardiographic principles for
differentiation between the morphological right and LV
again, if there is a univentricular heart with a rudimentary
chamber located posteroinferiorly, which does not give
off a great artery, and remains a blind pouch, then it is
the left ventricular rudimentary chamber and so the main
ventricle gets described as double inlet ventricle of RV
morphology. Van Praagh describes such a morphological
double inlet ventricle of RV morphology as a hip pocket
rudimentary left ventricular chamber, to indicate that
it is always posteriorly located. To summarize, double
inlet ventricle is classified as double inlet ventricle of left
ventricular morphology (accounts for 80% of double inlet
ventricles) if the rudimentary outflow chamber is located
anteriorly and gives off a great artery, and the rudimentary
chamber has relatively coarser trabeculations on its
surface near bulboventricular foramen. In double inlet
ventricles of RV morphology (accounts for < 10% of double
inlet ventricles), the rudimentary chamber is a blind
posteroinferior hip pocket pouch; it does not give off
any great artery and the main ventricle will have coarser
walls than the rudimentary ventricle. The other two
varieties of double inlet ventricle described by Van Praagh
namely double inlet ventricle of mixed morphology and
indeterminate morphology (account for < 10% of cases and
are very rare) have echocardiographically no rudimentary
chamber and both great arteries arise from the same main
ventricular cavity.

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1699

Double Inlet Left Ventricle

Assessment of the Systemic Veins

This is the commonest type of double inlet ventricle

accounting for almost 80% of the double inlet ventricles.
This group is subdivided into three subgroups based on
the great artery relationship:
Left and anterior Aorta (L-malposed aorta): In 55%
of double inlet left ventricle (DILV), the main LV
gives off the PA , there is pulmonary valveAV valve
fibrous continuity, AV discordance, the rudimentary
chamber is located on the left and anterior side of the
morphological main LV, and the anterior rudimentary
RV infundibular chamber gives off the aorta and the
aorta is left and anterior to the PA .
Right and anterior Aorta (D-malposed aorta): In 30% of
DILV, the main LV gives off the PA, there is pulmonary
valveAV valve fibrous continuity, the anterior
rudimentary RV infundibular chamber gives off the
aorta, and the aorta is right and anterior to the PA.
Normally related great arteries (Holmes Heart): In 15%
of DILV, the main ventricular chamber gives off the
aorta, there is aorticAV valve fibrous continuity, the
rudimentary anterior infundibular chamber gives off the
PA, the PA is located left and anterior to the aorta, and
the aortaPA relation resembles that of a normal heart.
Apical view shows double inlet ventricle and PSAX view
shows normally related aortaPA relationship; there is
narrowing of the subpulmonary infundibulum causing
subvalvular pulmonary stenosis.
Rare variations from this schema include double outlet
from the rudimentary chamber, atresia of one semilunar
valve often the pulmonary valve, and truncus arteriosus.

In every patient with univentricular AV connection, since

the path of palliation is univentricular, it is mandatory
to be precise about the systemic and pulmonary venous
anatomy before planning surgery. Systemic venous
anomaly commonly present is bilateral SVC. With left
isomerism, interrupted IVC should be looked for (seen in
85% of patients).

Double Inlet Right Ventricle

The main RV gives off both great arteries, the semilunar
valves of both aorta and PA are not in fibrous continuity
to the AV valves, and a well-discerned rudimentary hip
pocket (chamber) located posteriorly is of left ventricular
morphology. Depending on the location of the posterior
rudimentary LV, the segmental approach will vary as
D-loop (when the rudimentary chamber is posterior and
to the left), and L-loop (when the rudimentary chamber is
posterior and to the right of the main RV). Since the longterm outcome after Fontan surgery for single ventricles of
RV morphology is suboptimal compared to the single LVs,
echocardiographic identification of the precise ventricular
morphology as right and left is not just of academic interest
but has prognostic significance.

Assessment of the Pulmonary Veins

Anomalies of pulmonary venous return are also common
in single ventricle patients, especially in the setting of
heterotaxy syndromes. In single ventricular physiology
with reduced pulmonary flows, the anomalous pulmonary
venous drainage and associated obstruction of pulmonary
veins may not show up clearly since the pulmonary venous
flow will be of low velocity. Unidentified anomalous
pulmonary venous drainage into the azygos vein may
inadvertently be ligated/clipped as a part of Glenn shunt
with serious implications. The echocardiographer should
make serious attempts to trace all the pulmonary veins
meticulously and ensure that no individual pulmonary vein
drains anomalously into a chamber other than the atria and
there is no obstruction in the pulmonary venous pathway.

Assessment of the Atrioventricular Valves

It is imperative to assess the function of the AV valves
precisely, since the AV valve function has an important
bearing on the univentricular repair.
Stenosis of the AV valves is common and may manifest with minimal gradients. Morphological features of
these valves may be supramitral ring, hypoplastic AV valve,
double orifice AV valve, parachute mitral valve or dysplastic AV valve. These have major effects on the univentricular
pathway. Significant regurgitation of the AV valves too poses problems in univentricular surgical approach by increasing the atrial pressures and thereby impeding pulmonary
venous return. Such valves may need annuloplasty and
repair to minimize the degree of AV valve insufficiency.
In patients with volume-overloaded heart, with increased
pulmonary blood flow, the ventricular dilatation and
associated AV valve annular dilatation results in regurgitation. Once the volume overload is arrested by surgical
intervention like PA banding, ventricular volume
reduces immediately and the AV valve regurgitation of such
patients will reduce in severity.

1700

Section 6: Congenital Heart Disease

Bulboventricular Foramen
The bulboventricular foramen is the orifice through
which the main ventricular chamber feeds blood to the
rudimentary outflow chamber. If the chamber supports
the pulmonary flows like in Holmes heart, then restriction
of the bulboventricular foramen results in restricted
pulmonary blood flow. If the rudimentary outflow
chamber supports the aortic circulation, restriction of
bulboventricular foramen results in subaortic obstruction.
To calculate the area of bulboventricular foramen, measure
the maximum diameter of the bulboventricular foramen
in two orthogonal planes. If the dimensions are a and b,
then the area of the bulboventricular foramen is calculated
assuming it to be an ellipse using the formula:
Area = a + b/4
When the area of the bulboventricular foramen is
> 2.0 cm2/m2, the foramen is considered nonrestrictive.
In patients with area < 2.0 cm2/m2, during the initial
palliation, the restrictive bulboventricular foramen may
need to be addressed using one of the surgical strategies
like DamusKayeStansel procedure (end-to-side anastomosis of the main PA to the ascending aorta) or
enlargement of the bulboventricular foramen. In patients
in whom the bulboventricular foramen is anatomically
smaller though nonrestrictive by Doppler recordings, a
close echocardiographic watch is justified. The progression
of the hemodynamic narrowing of the bulboventricular
foramen is explained as follows: initially with acute volume
unloading of the ventricle after PA banding, there is an
acute reduction of the ventricular volumes and reduction
of the bulboventricular foramen area. Over long-term
follow-up, there is progressive ventricular hypertrophy,
which too results in further restriction of bulboventricular
foramen size. In neonates who are too young and small to
tolerate an enlargement of bulboventricular foramen or
DamusKayeStansel procedure, alternative approaches
followed include palliative arterial switch operation to
shift the restrictive rudimentary outflow chamber from
subaortic location to subpulmonary location. Alternative
method is the measurement of the VSD size and comparing
it with the aortic annulus size. If the 2D measurement of
the VSD is less than aorta, it requires enlargement.

Single Inlet Ventricle

This subgroup needs to be echocardiographically
differentiated from morphological tricuspid atresia

and mitral atresia with hypoplastic right or left heart.

Morphological definition for a single ventricle with one
AV valve atresia is that the atretic or imperforate plate of
the valve annulus overrides the ventricular septum and is
predominantly (>50%) committed to the main ventricle.
This means there is malalignment of the atrial and
ventricular septa produced by AV valve annular override.
Echocardiographic recognition of this feature will be done
by demonstrating malalignment of the atrial and ventricular
septum in a four-chamber, subcostal coronal and long-axis
view with anterior tilt. In tricuspid atresia and mitral atresia,
there will be alignment of the interatrial and ventricular
septum and the atretic plate of the AV valve will be related to the
hypoplastic RV or hypoplastic LV and will not override the
ventricular septum.

TRICUSPID ATRESIA
Tricuspid atresia is defined as complete agenesis of the
tricuspid valve with no direct communication between the
RA and RV. Morphologically, this entity is classified as in
Table 72.17.
Echocardiographic differentiation between tricuspid
atresia and single ventricle with right AV valve atresia has
already been highlighted in the previous discussion. We
will now discuss the important echocardiographic features
of this entity.

Tricuspid Atresia Type I

Tricuspid atresia is associated with normally related great
arteries in almost 75% of the cases. Rare instances are
absence of interventricular communications and these
patients have pulmonary atresia and do not have an
echocardiographically demonstrable RV at all (Type Ia).
However, in many instances, the VSD is restrictive and
this restrictive VSD is the site of significant subpulmonary
stenosis (Type Ib). Type Ia and Ib may require BT shunt
prior to the planned Glenn shunt depending upon the
systemic saturations. In patients with normally related
great arteries, a large VSD (Type Ic) is often associated
with unrestricted pulmonary blood flow and these
patients present early with cyanosis and congestive
heart failure and need PA banding. Echocardiographic
evaluation of tricuspid atresia for a presurgical evaluation
is mostly similar to the issues discussed under double inlet
ventricles.

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1701

Table 72.17: Classification of Tricuspid Atresia

Type 1: Normally related great arteries (75% of cases)

Type 1a with pulmonary atresia
Type 1b with pulmonary hypoplasia, small VSD
Type 1c with no pulmonary hypoplasia, large VSD
Type 2: d-Transposition of great arteries (25% of cases)
Type 2a with pulmonary atresia
Type 2b with pulmonary or subpulmonary stenosis
Type 2c with no pulmonary hypoplasia and large VSD
Type 3: l-Malposition of great arteries (very rare)
Type 3a, pulmonary or subpulmonary stenosis
Type 3b, subaortic stenosis
(VSD: Ventricular septal defect)

Tricuspid Atresia Type II

In patients with tricuspid atresia and d-transposition of
great arteries, the size of VSD decides on the development of subaortic stenosis and hence it needs assessment
similar to the bulboventricular foramen of univentricular
heart. Another echocardiographically significant lesion
frequently coexisting with tricuspid atresia and transposition of great vessels is aortic arch anomalies including arch
and isthmic hypoplasia. In some patients with tricuspid
atresia with transposition of great vessels, there will be left
juxtaposition of atrial appendages. This condition is recognized echocardiographically by noting absence of right
atrial appendage normally demonstrated in subcostal
bicaval view as an anterior projection from the RA
immediately below the SVCright atrial junction. In juxtaposition of atrial appendages, the appendage is missing
in this location, but instead noted on left side of the great
arteries.

MITRAL ATRESIA AND HYPOPLASTIC

LEFT HEART SYNDROME
(FIGS 72.204 AND 72.205)
This condition is included in this discussion, since it
also needs a univentricular type of repair. This syndrome
embraces a continuum of congenital cardiac anomalies
characterized by underdevelopment of the aorta, aortic
valve, LV, mitral valve, and LA to a varying extent.
Echocardiographic diagnosis of this entity will help in
quick recognition of the duct dependency of the systemic
circulation. The aortic arch color Doppler flow imaging will
show typical flow reversal in arch of aorta from the level of

the duct, since the arch and ascending aorta get retrograde
flows from the patent ductus. Prompt recognition of
such flow reversal in aortic arch and duct dependence
of systemic circulation will help in early institution of
prostaglandin E1 therapy and transfer to an advanced
cardiac surgical center.
Another important echocardiographic decision-making issue is identification of adequacy of interatrial septal
communication. Since ASD is the only outlet for pulmonary venous blood, inadequate atrial communication will
need urgent enlargement either in cardiac catheterization
suites or operation rooms. In some cases of mitral atresia,
decompression of the LA through a small unroofing of the
coronary sinus may be present.

Hypoplastic Left Heart Syndrome

Initial echocardiographic assessment of the HLHS (before
Stage 1 palliation, i.e. before Norwood procedure): The
goal of the assessment is to provide an accurate diagnosis
and complete hemodynamic information keeping in mind
that the parameters for future univentricular pathway
need to be looked at.
Objectives of echocardiography are to assess:
Anatomical and physiological components of HLHS:
LA, mitral valve, LV, LVOT, aortic valve, ascending
aorta, aortic arch, and isthmus
Adequacy of interatrial communication
Anatomy of the ductus and determine its adequacy
and physiology
Anatomy of the RV, RV outflow, tricuspid valve, and
pulmonary valve
RV function, AV regurgitation if any.

1702

Section 6: Congenital Heart Disease

Figs 72.205A to D: Two-dimensional echocardiography with color flow mapping in a case of left ventricular (LV) hypoplasia. (A) Fourchamber view with color flow mapping showing a restrictive interatrial communication; (B and C) Apical four-chamber view with color
flow compare showing a small LV cavity with thickened endocardium and flow acceleration across the mitral valve; (D) Suprasternal
view showing a hypoplastic ascending aorta and transverse arch. (As Ao: Ascending aorta; Ds Ao: Descending aorta; LA: Left atrium;
LV: Left ventricle; RA: Right atrium; TA: Transverse arch).

Associated Systemic Venous Anomalies

The evaluation is as for all CHD entities with sequential
analysis. Certain characteristic features that may be looked
at are enumerated below.

Subcostal View
As the transducer is tilted toward the cardiac base, the
coronary sinus, RA, and RV are visualized. A dilated
coronary sinus should alert one for the presence of
LSVC or anomalous pulmonary venous connection to
the coronary sinus. The RA in typical scenario is dilated
and hypertrophied from obligatory left-to-right shunting
through the patent foramen ovale. The RV also is dilated

as compared to the small LV. From the subcostal views,

a careful assessment of the adequacy of the interatrial
communication is done. The other feature that needs to
be looked at includes pulmonary venous connections, size
and function of the ventricular chambers, and evaluation
of the AV valves.
Atrial septum: Frequently, there is a leftward deviation
of the superior attachment of the atrial septum. The severity
of the restriction at the atrial septal communication can
be assessed by using color or spectral Doppler. The mean
gradient is obtained over three cardiac cycles.
Pulmonary veins: Anomalous pulmonary venous
connection/drainage occur in 5 to 10% of the cases of HLHS.
In cases of restricted interatrial septal communication,

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

a decompressing vein may be visualized from the LA

draining to variable locations. These may be stenosed and,
therefore, a full view of the course of the drainage of these
venous channels is needed. This could be assisted by use
of PW Doppler interrogation and color flow mapping.
Anterior angulation demonstrates the dilated RV
and RVOT. This needs to be carefully evaluated as this
will be the sole outflow tract from the cardiac chamber
in the near future. Associated pulmonary stenosis and
pulmonary valve regurgitation represent a poor subset.
The hypoplastic (tiny) ascending aorta may at times be
better visualized in the subcostal views.

Apical Views
Apical four-chamber view provides comparison of the
relative right and left sides of the heart. The RV is generally
large and hypertrophied, and the LV is small, musclebound, and nonapex forming. The endocardial surface
of the LV is often echogenic because of endocardial
fibroelastosis. This in itself is a very important prognostic
marker (particularly in borderline situations where one is
contemplating a two-ventricle repair). The morphology of
the mitral and the tricuspid valve can also be evaluated
in this view. The annulus of the mitral valve is typically
hypoplastic, severely stenosed, or may be atretic. This
may be evaluated using color flow mapping and Doppler
assessment. In case of stenosis of the mitral valve without
mitral regurgitation, one should be alerted to the likelihood
of ventriculocoronary sinusoids.
Tricuspid valve morphology and function needs to be
assessed also. It is important to identify and quantify the
tricuspid regurgitation, an important prognostic feature.
RV function is also assessed in this view.
Anterior tilt will show the aortic valve, ascending aorta,
and pulmonary trunk. Doppler assessment of the aortic
valve and color flow mapping will show the patency of the
aortic valve. The pulmonary valve should also be similarly
investigated.

Parasternal Views
Parasternal views are important to assess ventricular
function and size and function of the AV valves. The
dilated RV is seen anteriorly and the small LV, posteriorly.
The ventricular septum is usually intact. The aortic valve
is either stenosed or atretic and LVOT may have subaortic
obstruction, which can also be seen in this view. The
morphology of the mitral valve should be studied and
the dimensions of the mitral and aortic valve annulus

1703

measured in this view. Angling the transducer inferiorly

allows assessment of the tricuspid valve and tricuspid
regurgitation. Angling the transducer anteriorly shows the
pulmonary valve and the RVOT. This needs to be seen for
the presence of any obstruction. Pulmonary regurgitation
needs to be evaluated.
Short-axis sweeps allow assessment of the mitral valve
and subvalvular pathology. It also allows determining
the morphology of the aortic valve and the coronaries.
The RCA is usually prominent because of its supply to the
dominant RV. In the presence of LV to coronary fistulous
connections, the LCA may become prominent. High
PSAX view allows visualization of PA branches and the
arterial ductus. This view will also show the length of the
decompressing venous channel by tilting the transducer
posteriorly. Slight counterclockwise rotation may show
the large ductus; this may be larger than the arch and the
pulmonary arteries.

Suprasternal Notch View

This provides full assessment of the aortic arch. The
sidedness of the arch should be determined; the aortic
arch has a more acute curve than normal in these patients.
The sizes of the ascending aorta, transverse arch, and
isthmus should be measured and the posterior shelf of
coarctation of the aorta should be evaluated. Doppler
sampling of the distal segment of the aortic arch reveals
retrograde flow in systole into the transverse arch and
antegrade flow in diastole into the ductus arteriosus and
pulmonary vascular bed.

Echocardiographic Assessment
After the Stage 1 Palliation
The Norwood procedure consists of surgical
reconstruction and augmentation of the ascending aorta
and aortic arch with aortopulmonary amalgamation,
an atrial septectomy, and systemic to pulmonary shunt,
which can be either a BlalockTaussig shunt or a RV to PA
conduit (Sanos modification and PA banding). Interatrial
septal assessment is important as restriction may occur
mostly due to inadequate resection, an important cause
of cyanosis. The right BT shunt may be visualized from
the suprasternal short- or long-axis views with angulation
of the transducer toward the right side. Postoperative
complications of the shunt including pulmonary over
circulation, shunt stenosis, and hence PA distortion
may occur and need evaluation. Sanos shunt can be
best visualized by subcostal and modified apical views.

1704

Section 6: Congenital Heart Disease

Modified apical view is obtained by moving the transducer

anteriorly. Sliding the transducer up toward midsternum
will permit viewing the distal aspect of the conduit and
PA branches. Narrowing of the shunt at any point along its
course or at its insertion into the PA should be identified.
Color flow mapping and Doppler interrogation of the
conduit and proximal branch pulmonary arteries should
demonstrate a typical to and fro pattern of flow.
Evaluation of RV function and tricuspid regurgitation
should be performed from multiple imaging planes.
Echocardiographic evaluation of the neoaorta and arch is
also important with the distal aortic arch deserving special
attention. The ongoing evaluation after the first stage
Norwood is same as for Glenn and Fontan pathway.

characterized by bilateral right atrial appendages and

left isomerism with both appendages of left morphology.
Although there are several cardiac anomalies that are
common to both forms, certain unique features help to
differentiate the two forms. Right isomerism is associated
with absence of spleen in majority of cases and hence it is
also referred to as asplenia syndrome.328 Left isomerism
is characterized by presence of multiple, albeit abnormal,
spleens and is hence called polysplenia syndrome.
Several extracardiac anomalies other than those that
involve the spleen are associated with these syndromes.
Tables 72.18 and 72.19 compare cardiac and extracardiac
anomalies found in these two syndromes.

Initial Echocardiogram (Table 72.20)

HETEROTAXY SYNDROME
Introduction
The term visceral heterotaxy originates from the Greek word
heteros other than and taxis arrangement. It basically
refers to any arrangement of body organs in patterns other
than the usual (situs solitus) or its mirror image variant (situs
inversus).319325 Very often, these conditions are associated
with an abnormal number and arrangement of the spleen,
and hence the term splenic syndromes has often been
given to these conditions. Cardiac malformations associated
with these syndromes are often complex with abnormalities
at various levels of the cardiac axis (visceroatrial situs,
ventricular loop, and ventriculoarterial connections). The
characteristic cardiac abnormality found in the heart is
referred to as isomerism of atrial appendages, whereby
both the atrial appendages have either right or left
morphology.326,327 Current echocardiographic techniques
allow a complete structural evaluation of the entire cardiac
anomalies in these patients, so that cardiac catheterization
is required only for hemodynamic evaluation prior to
consideration of single ventricle palliation in selected
cases. In this chapter, we discuss the steps involved in
the systematic evaluation of the heart and great vessels in
patients with visceral heterotaxy.

Anatomical Background:
Characterization of Two Distinct
Heterotaxy Syndromes
Two distinct patterns emerge when we consider cardiac
abnormalities in these complex syndromesright
isomerism and left isomerism. Right isomerism is

In view of the complex nature of associated cardiac

abnormalities, most of the patients will come to clinical
attention in the neonatal period or early infancy itself.
The basic aim of the initial echocardiogram is to assess
the entire spectrum of the cardiac anatomy with special
emphasis on evaluation of pulmonary blood flow. A
segmental approach starting from the determination of
the visceral and atrial situs, and then proceeding to each
segment in the heart and great arteries is recommended.329
In most cases, initial palliation is possible on the basis of
echocardiographic evaluation alone without resorting to
cardiac catheterization.
The steps involved in the initial echocardiographic
evaluation are as follows:

Determination of Abdominal Situs and

Cardiac Position
This is achieved using the subcostal short-axis view. In
situs solitus, the liver will be found to occupy the right side
with the stomach on the left side of the spine. The aorta
will occupy the left side of the spine and IVC on the right
side. In situs inversus, this arrangement is reversed with
aorta occupying the right anterior aspect of the spine. The
position of the heart can be determined by cranial tilt of
the transducer and determining which way the apex of
the heart points (leftlevocardia; rightdextrocardia;
middlemesocardia). The position of the heart (levo or
dextrocardia) does not help in differentiation of right and
left isomerism.
In patients with visceral heterotaxy, the position of
the liver can be to the right, to the left, or transverse.
The stomach can be on either side or midline. These

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

1705

Table 72.18: Cardiac and Extracardiac Anomalies in Isomerism Syndromes

Right Isomerism

Left Isomerism

Dextrocardia

40%

40%

Right pulmonary isomerism

70%

10%

Left pulmonary isomerism

Rare

60%

Bilateral superior vena cava (SVC)

50%

40%

Absent inferior vena cava (IVC)

Rare

70%

Total anomalous pulmonary venous connection (TAPVC)

70%

Rare

Partial anomalous pulmonary venous connection

Rare

40%

Atrial septal defect (ASD)/common atrium

90%

80%

Atrioventricular (AV) septal defect

85%

40%

Single ventricle

50%

10%

Transposed great arteries

80%

30%

PS/pulmonary atresia

80%

30%

LVOTO

Rare

40

Extracardiac anomalies

CNS, gastrointestinal,
skeletal and genitourinary

Biliary atresia
Intestinal malrotation
Agenesis of gall bladder

Source: Gutgesell HP. Cardiac Malposition and Heterotaxy. In: Garson Jr, Bricker JT, Fischer DJ, Neish SR, editors. The Science and
Practice of Pediatric Cardiology. Baltimore, MD: Williams & Wilkins; 1998:153961.

relationships do not help differentiate between right and

left isomerism. However, the characteristic arrangement
of the aorta and IVC in relation to the spine will give a clue
to the diagnosis and help to differentiate between the two
types. In right isomerism, the aorta and IVC will be found
to occupy the same side of the spine (right or left), with the
IVC occupying a position more anterior to the spine than
the aorta. In left isomerism, the IVC is often interrupted
with the abdominal portion of the IVC continuing as
azygos or hemiazygos veins.330 This venous channel is
distinguished by location on the same side of the spine as
the aorta, but in a posterior position. All these relations are
best demonstrated using the subcostal sagittal scan and
short-axis scan.

Venoatrial ConnectionsSystemic Veins

Abnormalities of the systemic veins are very common
in both syndromes. Knowledge of the systemic venous
anatomy is very important since a vast majority of these
patients eventually require palliation via the univentricular
pathway. The patterns and frequencies of these anomalies
are summarized in Table 72.18.
Superior vena cava: Right isomerism is characterized by
presence of bilateral SVC, which typically drains to the roof

of atrium on both sides. The frequency of bilateral SVC is

lower in left isomerism. SVC drainage to the atrium can be
best demonstrated using the subcostal sagittal coronal or
four-chamber and suprasternal views. LSVC drainage is
best noted by suprasternal long-axis and short-axis views
with leftward tilt of the transducer.332 In these views, the
LSVC is seen descending in front and to left of the aortic
arch and pulmonary hilum toward the atrioventricular
groove. In the suprasternal short-axis views, the presence
of bilateral SVC can be demonstrated and the presence
and size of the bridging vein can be assessed. In patients
with right isomerism, LSVC will be seen draining to the
roof of the atrium between the left upper pulmonary
vein and left-sided atrial appendage. In patients with left
isomerism, LSVC may drain to the coronary sinus. Color
flow mapping should be used to determine the exact
point of insertion of the SVC into the atrium. PW Doppler
is useful in differentiating LSVC from similar appearing
structures (left levocardinal vein) by demonstrating flow
toward the heart. In cases with bilateral SVC, the actual
sizes of both SVC and the presence and size of the bridging
vein should be noted.
Inferior vena cava: Most patients with right isomerism have
no abnormalities of the IVC drainage with the IVC receiving
the hepatic veins and draining to the RA. However, a vast

1706

Section 6: Congenital Heart Disease

majority of patients with left isomerism have abnormal

IVC drainage, with interruption in IVC being the most
common anomaly. The subcostal short-axis view shows
the azygos vein as a large vessel on posterior and rightward
Table 72.19: Venoatrial Connections in Isomerism Syndromes

Right Isomerism

Left Isomerism

Superior vena cava (SVC)

Unilateral to right

29%

22%

Unilateral to left

20%

16%

Bilateral to roof

51%

38%

Bilateral, one via

coronary sinus

24%

To right-sided atrium

48%

12%

To left-sided atrium

52%

12%

Interrupted on right

34%

Interrupted on left

42%

Inferior vena cava (IVC)

Hepatic veins
Confluence to IVC

76%

14%

Confluence to atrium

43%

Unilateral to atrium

6%

8%

Bilateral to atrium

18%

35%

To right-sided atrium

19%

26%

To left-sided atrium

19%

14%

Bilaterally to atriums

60%

Centrally via confluence

3%

To extracardiac site

59%

Pulmonary veins

Source: Uemura et al. Annals of Thoracic Surgery. 1995;60:5619.

aspect of the spine. In the subcostal sagittal view, this large

vessel can be traced to the posterior aspect of the aorta
draining to the posterior aspect of the right SVC.
In hemiazygos continuation of the IVC, the abdominal
portion of the right IVC is absent. Blood from the lower
body returns to the heart by way of a left-sided continuation
of a venous channel (hemiazygos continuation), which
drains to LSVC or coronary sinus. The subcostal short-axis
view of the abdomen shows a large venous channel on
the left posterior aspect of the spine. Using the subcostal
sagittal view with cranial angulation and suprasternal
view, the drainage of this vessel to the heart can be imaged.
Hepatic veins: Evaluation of the hepatic vein drainage
is vital in evaluation of patients with interrupted IVC,
especially before consideration of the bidirectional
cavopulmonary shunt (BCPS; Kawashima Operation).
Anomalies in the hepatic venous drainage have been
implicated in the genesis of pulmonary arteriovenous
fistulae after BCPS in these patients, leading to systemic
desaturation.7 This will necessitate re-routing the hepatic
veins to the pulmonary circulation. In patients with left
isomerism, different patterns of hepatic venous drainage
have been demonstrated (Table 72.19). The veins can
drain unilaterally or bilaterally to the atrium or may form a
confluence and then drain to the atrium. The presence of
bilateral drainage to both atria often makes a subsequent
Fontan conversion more difficult technically. In patients
with right isomerism, hepatic venous drainage is normal
with all veins forming a confluence and draining into the
suprahepatic portion of the IVC. The abnormalities of the
hepatic venous drainage are best demonstrated using the
subcostal short-axis view with cranial tilt and subcostal
sagittal view.333

Table 72.20: The Echocardiographic Checklist for the Univentricular Pathway Patient

Look at the electrocardiography (ECG) for any rhythm abnormality

Evaluation of the pulmonary artery (PA) pressures: these may be done from shunt lesions such as patent ductus arteriosus (PDA)
or pulmonary outflow gradients
Ventricular dysfunction
Systemic atrioventricular (AV) valve regurgitation
Aorta and subaortic obstruction if any
Presence of interatrial communication may be essential in certain circumstances
Pulmonary venous drainage
Systemic venous drainage and to look at systemic veins
Pulmonary artery anatomy: (a) size of the pulmonary arteries, (b) distortion of the pulmonary arteries

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

Abnormalities of coronary sinus: A characteristic feature

of right isomerism is the absence of the coronary sinus
with the cardiac veins draining directly to the atrium. The
absence of the coronary sinus can be demonstrated in the
apical four-chamber view and subcostal sagittal view. The
LSVC, if present, will be directly draining to the roof of the
atrium between the left upper pulmonary vein and left
atrial appendage. Drainage of LSVC to coronary sinus can
be seen in patients with left isomerism.

Venoatrial Connections: Pulmonary Veins

Abnormal pulmonary venous drainage is more common
in patients with right isomerism. A majority (>80%) of
these cases have TAPVCs to right-sided atrium or systemic
veins. TAPVC in the setting of right isomerism can occur
to supracardiac, intracardiac, or infracardiac locations.
When pulmonary veins enter the atrium directly (without
confluence or common pulmonary vein), they tend to
drain to the smooth intercaval portion of the atria. Forty
percent of patients with left isomerism have PAPVC
with right veins entering the right-sided atrium and left
veins to left-sided atrium. This may result from leftward
malalignment of atrial septum. Obstruction to pulmonary
venous flow is also more common in the setting of right
isomerism. This is particularly important to identify before
consideration of placement of a systemicPA shunt to
increase pulmonary blood flow. Failure to identify an
obstructed TAPVC will lead to development of severe,
often fatal, pulmonary edema after placement of the
shunt. Pulmonary venous drainage can be imaged from
the subcostal coronal, apical four-chamber, PSAX, and
suprasternal views. The presence of a confluence, size
of individual veins, drainage of all veins to confluence,
presence of additional directly draining veins, and
adequacy of communication of the confluence to atrium/
systemic veins should be identified. Color flow mapping
and PW Doppler will help identify obstruction in the
pulmonary venous drainage. Obstruction to pulmonary
venous flow will produce characteristic high velocity (>2
m/s), continuous flow signals on PW Doppler. Obstruction
to pulmonary venous drainage can occur at the site of
insertion of the veins to SVC, at the junction of vertical
vein to brachiocephalic vein, at the level of infracardiac
drainage of the veins, or by external compression when the
vertical vein runs a retrobronchial course (specific to right
isomerism).334,335

1707

Atria and Appendages

In some patients with heterotaxy, discordance between
atrial and abdominal situs has been described. Drainage
of systemic veins, presence of tendinous insertion of the
Eustachian valve, and limbus of fossa ovalis on septal
surface characterizes RA, while the flap valve of the
fossa ovalis on the other side is generally an indicator of
LA. However, in patients with heterotaxy, the venoatrial
connections, vestibular morphology, and atrial septal
structure are all variable. Identification of the atrial situs is
often best done by evaluating the morphology of the atrial
appendages. The characteristic features of the right atrial
appendage are larger size, triangular appearance, broad
base, and presence of pectinate muscle extending till the
crux of the heart. The left atrial appendage, in contrast, is
smaller, has a tubular appearance with narrow base, and
has pectinate muscle at the vestibule only. However,
pectinate muscles cannot be profiled on echocardiography. Using these features, the atrial situs can be determined in the vast majority of patients with heterotaxy. The
term situs ambiguous should be reserved to the very few
patients where the atrial situs cannot be determined even
after evaluating all the above mentioned features.
The atrial appendages are best visualized using TEE.
Using transthoracic echocardiography, the right atrial
appendage is well visualized in the PLAX view through
the RV inflow tract and subcostal sagittal view. The left
atrial appendage is well seen in PSAX and subcostal fourchamber views.336,337

Atrial Septum
Common atrium with both atria having right morphology
is often associated with right isomerism. In patients
with left isomerism, 50% of cases have common atrium.
The morphology of the atrial septum does not help in
differentiating between right and left isomerism. The
ASD is best imaged using the subcostal coronal and
sagittal views. The adequacy of the size of the interatrial
communication is important before consideration of a
single ventricle repair.

Atrioventricular Junction
The atrioventricular connections in patients with
isomerism can be biventricular or univentricular. The
ventricular topology can be either D-loop or L-loop,

1708

Section 6: Congenital Heart Disease

with D-Loop patterns dominating. Left isomerism has

a higher prevalence of biventricular atrioventricular
connection compared with right isomerism (74% vs 46%).
Univentricular connections are much more common in
right isomerism. Irrespective of the nature of atrioventricular connection, most patients with isomerism (93% in
right and 67% in left) have a common atrioventricular valve.
It is very important to assess the degree of regurgitation
of the common valve, its mechanisms, and possibility of
repair before consideration of single ventricle palliation,
since it is a major risk factor for surgery. The anatomy of
the valve is best assessed by subcostal coronal view with
anteroposterior sweeps, subcostal en face view, and apical
four-chamber views.338

Ventricles and Ventricular Septum

A univentricular heart is more common in patients
with right isomerism than left. Within the category of
isomeric hearts with single ventricle, morphological
RV type of single ventricle is more common. In patients
with univentricular heart and double inlet connection
via a common atrioventricular valve, the position of
the ventricular septum relative to the common valve
will help to identify the ventricular morphology. If the
septum is found anterior to the valve, the ventricular
morphology will be that of LV; if it is posterior, it suggests
RV. Failure to identify a ventricular septum indicates
single ventricle of indeterminate morphology. In cases
of RV type of single ventricle, the rudimentary LV cavity
is located posterior or on the diaphragmatic surface of
the heart with no arterial outlets. If the single ventricle is
of the LV type, a rudimentary RV/outflow chamber will
be present with at least one of the great arteries typically
arising from that chamber. In patients with biventricular
connections and common AV valve (more common in left
isomerism), the ventricular morphology can be identified
by the nature of trabeculations, papillary muscles, and
site of septal attachment of inferior bridging leaflets.
Associated anomalies of the ventricle include presence of
noncompaction of the ventricular myocardium.
A thorough evaluation of the ventricular systolic
and diastolic function should be undertaken in the
initial evaluation as well as in evaluation prior to single
ventricle repair. End-systolic and end-diastolic volumes
and ejection fraction are calculated. The ventricular
mass can be calculated by subtracting the endocardial
volume from the epicardial volume and multiplying the
resultant myocardial volume by 1.05 (specific gravity

of myocardium). The diastolic function of the ventricle

can be assessed by Doppler evaluation of the AV valve
and pulmonary veins. The application of newer imaging
modalities like tissue Doppler imaging, harmonics, and
strain rate imaging are yet to be standardized for use in
these patients.
In patients with biventricular connections (more
common in left isomerism), an attempt should be
made to assess for possibility of biventricular repair.
Factors that decide this include characteristics of the AV
valve (balanced vs. unbalanced, subvalvular apparatus
straddling) and characteristics of the VSD (location,
routability, presence of multiple VSDs, etc.). The
relationship of the conducting system with respect to the
VSD also is an important consideration in view of risk of
heart block when biventricular repair is attempted.
Evaluation of the ventricles and ventricular septum is
done using a combination of subcostal long- and shortaxis views, four-chamber view with cranial angulation,
and parasternal long- and short-axis views.

Ventriculoarterial Connections
In patients with right isomerism, the common abnormalities of the ventriculoarterial connections are DORV
with pulmonic stenosis/atresia and single outlet with no
pulmonary outflow. Discordant ventriculoarterial connections may also be seen. The assessment of severity of
pulmonary stenosis is very important. The outflow tracts
can be imaged using subcostal coronal, four-chamber,
and PSAX views. Doppler evaluation of the gradient
across the pulmonary outflow (subvalvular and valve)
will provide an estimate of PA pressures. However, in
presence of pulmonary venous obstruction, Doppler
estimation of the gradient may underestimate the severity
of the obstruction of outflow. In such cases, attention
should focus on morphology of the outflow tract. In left
isomerism, concordant ventriculoarterial connections are
more common and obstruction to pulmonary outflow is
less common.

Branch Pulmonary Arteries and Sources of

Pulmonary Blood Flow
Evaluation of branch pulmonary arteries constitutes one of
the most essential aspects of evaluation prior to any form
of surgical repair. This is best done from the PSAX view,
the ductal (high parasternal view), and suprasternal views.
The presence of the confluence of pulmonary arteries is

Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease

first determined. The diameter of the branch PAs is then

measured at the level of the hilum. Adequacy of the sizes
of the branch PAs can be determined by calculating the
z-scores. The presence of narrowing at any point along the
PA (origin, site of previous shunt, etc.) is documented. It is
important to discriminate between focal areas of stenosis
(repairable) and more diffuse stenosis of the entire PA. The
sources of pulmonary blood flow (antegrade, ductus, or
aortopulmonary collateral) need to be determined. The
presence of bilateral ductus supplying the right and left
pulmonary arteries is common in right isomerism. This is
best demonstrated by suprasternal views.
The presence of MAPCAs can be evaluated in the
suprasternal and subcostal long-axis views, this may not be
possible to assess by echocardiography of the descending
aorta. Large MAPCAs will cause flow reversal in the
descending aorta. MAPCA can be identified by presence of
continuous flow in the vessel, tortuous course, and direct
termination to the lung (instead of PA).

Aortic Arch and Branches

Obstruction to aortic outflow is more common in patients
with left isomerism with common atrioventricular valve.
From the four-chamber view with cranial angulation (fivechamber view) and PLAX view, the LVOT can be visualized
and presence of any obstruction across the aortic valve
is assessed. If there is obstruction, the gradients can be
calculated. The morphology of the aortic valve is best
assessed in the PSAX view. The evaluation of the arch is
best done from the suprasternal views. Measurements
of the aorta at levels of ascending aorta, transverse arch,
isthmus, and descending aorta are taken in all patients. The
presence of localized/diffuse constriction and gradients
across the points of obstructions are also measured.

ACKNOWLEDGMENTS
We are indebted to our colleagues for their cooperation.
We wish to express our heartfelt thanks and gratitude to
our secretary Ms Poonam Toppo for her secretarial help
and Dr Kunal Bhagatwala for the correction of the proof of
the chapter and helping with the movie clips.

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1718

Section 6: Congenital Heart Disease

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284. Virmani R, Chun PK, Goldstein RE, et al. Acute takeoffs of

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286. Akagi T, Rose V, Benson LN, et al. Outcome of coronary
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287. Kato H, Koike S, Yamamoto M, et al. Coronary
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288. Sakakibara S, Yokoyama M, Takao A, et al. Coronary
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Arch Anomalies
291. Allen HD, Goldberg SJ, Sahn DJ, et al. Suprasternal notch
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292. Hastreiter AR, DCruz IA, Cantez T, et al. Right-sided
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293. Mathew R, Rosenthal A, Fellows K. The significance of
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294. Knight L, Edwards JE. Right aortic arch. Types and associated
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295. Garti IJ, Aygen MM, Vidne B, et al. Right aortic arch with
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296. Drucker MH, Symbas PN. Right aortic arch with aberrant
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297. Knight WB. Hypoplastic right retro-oesophageal aortic
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298. Haughton VM, Fellows KE, Rosenbaum AE. The cervical
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300. Mullins CE, Gillette PC, McNamara DG. The complex of
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301. Tiraboschi R, Crupi G, Locatelli G, et al. Cervical aortic

arch with aortic obstruction: report of two cases. Thorax.
1980;35(1):2630.
302. Fyler DC, Buckley LP, Hellenbrand WE, et al. Report of the
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303. Carvalho JS, Redington AN, Shinebourne EA, et al.
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of juxta-ductal aorta and rapid progression of obstruction
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305. Morris JH, McNamara DG. Coarctation of the aorta
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306. Smallhorn JF, Huhta JC, Adams PA, et al. Cross-sectional
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309. Sanders SP, MacPherson D, Yeager SB. Temporal flow
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310. Shady RE, et al. Pulsed Doppler findings in-patients with
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311. Simpson IA, et al. Color Doppler flow mapping
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314. Kutsche LM, Van Mierop LH. Anomalous origin of a
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1719

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Section 6: Congenital Heart Disease

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1992;20(3):67884.

CHAPTER 73
Real Time 3D Echocardiography for
Quantification of Ventricular Volumes,
Mass and Function in Children with
Congenital and Acquired
Heart Diseases
Shuping Ge, Jie Sun, Lindsay Rogers, Rula Balluz

Snapshot

Left Ventricular Volumes, Ejection Fraction, and Mass

Right Ventricular Volumes, Ejection Fraction, and Mass

Single Ventricular Volumes, Ejection Fraction, and Mass

INTRODUCTION
The advent of real time three-dimensional echocar
diography (RT3DE) is a major milestone in the evolution
of echocardiography. RT3DE has been used to generate
three-dimensional (3D) views of cardiac structures in an
intuitive and object format, obviating the need to mentally
create 3D images based on sweeps by two-dimensional
echocardiography (2DE). Furthermore, RT3DE provides
an opportunity to quantitatively measure distances, areas,
volumes to quantify the cardiac structures, flows, and
function in 3D that may not possible by 2DE. A query of
3D echocardiography yields over 4,500 publications
in PubMed database. Therefore, the focus of this chapter
is the use of RT3DE to quantitatively assess ventricular
volumes, mass, and function in children with congenital
and acquired heart diseases. Other related topics can be
found in other chapters of this book.

Three-Dimensional Analysis of Regional Wall Motion,

Synchrony, and Strain

Future Perspectives

Quantitative measurement of left ventricular (LV)

volumes, mass, and function is one of the most common
and important indications for echocardiography. These
measurements are among the most powerful tools for
diagnosis and prognosis of congenital and acquired heart
diseases and for assessment of medical, percutaneous,
and surgical interventions. Awareness is also growing of
the importance of right ventricular (RV) volume, mass, and
function in many cardiopulmonary diseases. Furthermore,
there are challenges and opportunities to measure the
volume, mass, and function of complex chambers such
as the left atrium, right atrium, and the univentricular
heart. As echocardiography continues to be the imaging
modality of choice for these measurements, the strengths
and limitations of M-mode, two-dimensional (2D), and
recently 3D echocardiographic methodologies for accurate
and reproducible measurement of these indices have been
extensively investigated for congenital and acquired heart
diseases.

1722

Section 6: Congenital Heart Disease

Evidence suggests that 3DE provides improved

accuracy and reproducibility over 2D methods for measu
rement of LV volume and function calculation in adults13
and in children.410 Data have accumulated on the utility of
3DE for measuring chamber volumes and function for the
RV, and for the single ventricle,2,1114 which may become
more widely used in clinical and research arenas in the
future. Finally, new advanced modes of analysis such as
3D strain and synchrony analysis by 3DE are promising
methodologies that warrant further investigation.

LEFT VENTRICULAR VOLUMES,

EJECTION FRACTION, AND MASS
The utilization of echocardiography to assess LV chamber
size and function dates to the advent of this technology.
Popp et al.15 reported on the change of cardiac dimensions
using M-mode echocardiography. Feigenbaum et al.16
used these changes to assess LV function and correlated it
to angiography. The correlation appeared good except in
cases with dilatation and regional wall abnormalities. The
development of 2DE allowed the utilization of LV area and
the LV long axis to estimate volume. Wyatt et al.17,18 showed
that this technique was superior to M-mode, especially in
asymmetrical hearts. This technique is limited by the fact
that 3D measurements are extrapolated from 2D-acquired
data using geometrical assumptions. Also, on-axis crosssectional images may not be accurate because the whole
surface of the heart is not visualized. Finally, there may
be problems with foreshortening the long axis of the
ventricle. The logical next step is the development of 3D
constructions of the LV to assess volumes and function.
Dekker et al.19 first attempted to create 3D images with
a mechanical arm, which resulted in creating a 3D model
of the LV. Many investigators also published measurements
of LV volumes using similar techniques with in vivo
validation using animal and human models.2025 The
ability of 3D technology to overcome geometric assum
ptions, avoid image positioning errors, and provide
a surface reconstruction algorithm was the basis for
this improvement, as confirmed by many subsequent
studies.2629
The advent of RT3DE generated a renewed enthusiasm
for potential clinical use in the adult population. RT3DE
has been shown to have excellent correlations with
cardiac magnetic resonance imaging (CMR) for assessing
chamber volumes and ejection fraction (EF) in adults,

Fig. 73.1: Semiautomated three-dimensional echocardiography

(3DE) algorithm for measurement of LVEDV, LVESV, and left ventricular ejection fraction (LVEF). (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle).
Source: Adapted from Ref. 96.

but it underestimates LV volumes with insignificant

difference for the EF between the two modalities. Most
studies found the differences in volume calculations to be
more significant with dilated and abnormal ventricles.30
Some experts propose including the endocardial surface
with the trabeculations to improve the correlation with
CMR.3133 Others suggest increasing the number of
planes to better delineate the endocardial surface in an
attempt to eliminate geometric assumptions, especially
in abnormal ventricles.34,35 Others have had more success
using direct volumetric measurement through the use of
semiautomated border detection (Fig. 73.1).
RT3DE has also been applied in adults with congenital
heart disease for measurement of LV volumes. van den
Bosch et al.36 studied 32 patients ranging in age from
1951 years with complex congenital heart disease,
including tetralogy of Fallot, RV obstructive lesions,
and transposition of the great vessels after atrial switch
surgery. Compared with CMR, RT3DE underestimated LV
volume, but the difference was not statistically significant.
These investigators found good correlations in LVEDV,
LVESV, and left ventricular ejection fraction (LVEF)
between RT3DE by manual border detection and CMR,
with correlation coefficients (r) = 0.98, 0.97, and 0.94,
respectively. Correlations in LVEDV, LVESV, and EF were
modest between CMR and RT3DE using automatic border
detection in this study of ventricles with altered geometries
(r = 0.79, 0.83, and 0.54, respectively).

Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children

Left Ventricular Mass

RT3DE has also been used for the assessment of LV mass.
The technique requires identification of both endocardial
and epicardial borders. This has made estimation of LV
mass more challenging in adult studies. A major challenge
is the difficulty in visualizing the apex for mass calculation.
However, validation studies showed better correlation
between RT3DE and CMR compared with estimating LV
mass estimation using M-mode or 2D methods, which
have been reproduced in many adult studies.37,38

Meta-Analysis
Shimada and Shiota39 conducted a meta-analysis to assess
the sources of errors in evaluation of the LV by 3DE. Their
analysis included 3,055 subjects in 95 studies. The study
showed significant underestimation bias of both LVEDV
and LVESV by RT3DE compared with CMR. The bias
for estimation of LVEF was not statistically significant.
Sources of error included female sex and presence of
congenital heart disease, which were associated with
more underestimation in the analysis. Semiautomatic
border detection and the use of matrix-array transducer
were associated with less underestimation. Despite the
differences between RT3DE volumetric estimation of the
LV and CMR, the literature supports the role of RT3DE as
both accurate and reproducible in assessing LV volume
and LVEF, although it is not interchangeable with other
radiological modalities.
Shimada and Shiota40 published another meta-analysis
evaluating the use of RT3DE to assess LV mass. This analysis
assessed 25 studies including 671 comparisons. Studies
published in 2004 or earlier showed high heterogeneity
[I(2) = 69%)] and significant underestimation of LV mass
by 3DE (5.7 g, 95% confidence interval 11.3 to 0.2,
P = 0.04). Studies published between 2005 and 2007
were still heterogeneous [(I(2) = 60%)] but showed less
systematic bias (0.5 g, 95% confidence interval 2.5 to 1.5,
P = 0.63). In contrast, studies published in 2008 or later were
highly homogeneous [(I(2) = 3%)] and showed excellent
accuracy (0.1 g, 95% confidence interval 2.2 to 1.9,
P = 0.90). Investigation of factors affecting the bias revealed
that evaluation of cardiac patients compared with healthy
volunteers led to greater bias (P < 0.05). In conclusion,
this meta-analysis elucidates the underestimation of LV
mass by 3DE, improvement of the technique over the past
decade, and factors affecting the degree of bias. These data

1723

provide a detailed basis for improving the accuracy of 3DE,

an indispensable step toward further clinical application.
Currently, for transthoracic and transesophageal
assessment of LV volumes and EF, 3DE is recommended
over the use of 2DE, as 3DE has been clearly demonstrated
to provide more accurate and reproducible measurements.2

Left Ventricle Analysis in Children

Although M-mode and 2DE recordings are widely used
to assess LV size, mass, and global systolic function in
children with congenital and acquired heart disease,
in many defects LV or ventricular septal geometric
or functional alterations diminish the accuracy and
reproducibility of these measurements. Many validation
studies have demonstrated that 3DE compares favorably
with other independent reference standards such as
CMR and is superior for assessing LV volumes, mass, and
EF in children as well as adults (Table 73.1). Since our
first validation of RT3DE by CMR,4 seven RT3DE studies
have been published comparing measurements of LV
volumes, mass, and EF with CMR measurements.410 These
reports showed excellent correlation, agreement, and
reproducibility for volumes and mass between the two
modalities but only modest advantages in measurement of
EF. We have also found that RT3DE using a semiautomated
method is more accurate and reproducible than M-mode
or Simpsons 2D biplane method and is as efficient as 2DE
in children.6 These findings suggest that the American
Society of Echocardiographys recommendation that in
adults LV should be quantified using RT3DE may also be
applied to children.1,2
Studies of LV mass assessment in children are very
limited. However, the available studies report excellent
correlations between RT3DE and CMR, although RT3DE
overestimated LV mass in comparison to CMR in most
of these studies. Inter- and intraobserver variability in
this assessment are excellent.4,68 These data suggest that
RT3DE quantification of LV mass is feasible for clinical
applications, although the normal ranges of LV volumes,
mass, and EF in various age groups must still be defined.

RIGHT VENTRICULAR VOLUMES,

EJECTION FRACTION, AND MASS
Because of the complex geometry of the right ventricle,
clinical assessment of its size, mass, and EF has largely
been qualitative. However, the importance of RV
chamber size and systolic function has increasingly been

1724

Section 6: Congenital Heart Disease

Table 73.1: Real Time Three-Dimensional Echocardiography for Left Ventricle Volumes, Mass, and Function in Children

RT3DE Compared With

Other Method

Methods

LV Indices

Correlation

19

CMR

Long-axis, eight
planes

LVEDV, LVESV,
LV mass, LVEF,
SV

r = 0.860.97

2007

25

LV angiography

Semiautomated

LVEDV and
LVES

r = 0.9790.996

Lu et al.6

2008

20

CMR

Four-plane, eightplane semiautomated

LVEDV, LVESV,
LV mass, LVEF,
SV

r = 0.850.9

Riehle et al.7

2008

12

CMR

Semiautomated

LVEDV, LVESV,
LV mass, LVEF,
SV

r = 0.930.99, 0.69 for EF

Friedberg
et al.8

2010

35

CMR

Disc summation

LVEDV, LVESV,
LVEF

r = 0.900.96

Hascot et al.9

2010

50

RT3DE

Semiautomated
(Qlab vs. TomTec)

LVEDV, LVESV,
LV mass, LVEF,
SV

r > 0.97 but 0.79 for EF

Laser et al.10

2010

49

CMR

2 Ultrasound systems/
semiautomated

LVEDV and
LVESV

r = 0.910.95

Reference

Year

Number of
Patients

Bu et al.4

2005

Iino et al.5

(CMR: Cardiac magnetic resonance imaging; EF: Ejection fraction; LV: Left ventricle; LVEDV: Left ventricular end-diastolic volume
LVEF: Left ventricular ejection fraction; LVESV: Left ventricular end-systolic volume; RT3DE: Real time three-dimensional echocardiography; SV: Stroke volume).

recognized in both pediatric and adult heart disease.

Many volume and pressure overloading conditions can
cause remodeling and dysfunction of the RV. Studies in
children who have undergone repair of tetralogy of Fallot
have shown that longitudinal follow-up of right and left
ventricular remodeling and dysfunction are crucial to
guide timely intervention to replace the pulmonary valve
to avoid failure of the RV, arrhythmia, and sudden cardiac
death. Although CMR has been established as a clinical
standard for quantitative analysis of the RV, quantitative
RT3DE may be useful in many instances, for example,
as a potential screening tool and in those in whom CMR
is contraindicated or not available. Because of the cost,
portability, and availability of RT3DE and the growing
evidence of the utility of RV3DE, the application of RV3DE
for quantitative analysis of the RV may increase in the
future.
Unlike the LV, there is no established geometric model
that can be used for quantitative analysis of RV volumes,
mass, and function. Clinical evaluation of RV size and
function is largely qualitative for both adults and children.
Quantitative analysis of the RV becomes a major potential
application for 3DE, especially since RT3DE hardware and
software have become available. Initial validation studies
of RV volumes and EF quantification by 3DE were based

on in vitro and in vivo models.4147 Multiple clinical studies

in adults followed, comparing RT3DE volumes with
CMR.4853

Right Ventricle Analysis in Children

and in Patients with Congenital Heart
Disease
Our group11 used a disc-summation method (Figs 73.2A
and B) and studied 20 normal children between the ages
of 6 and 18 years comparing RV volumes and function
obtained with RT3DE versus CMR. We found excellent
correlation between RT3DE and CMR, with correlation
coefficients of 0.96, 0.98, and 0.89 for RVESV, RVEDV,
and RVEF, respectively. We also found good inter- and
intraobserver variability. There was small but significant
underestimation of RV volume, especially RVEDV, by
RT3DE compared with CMR. The differences were not
statistically significant for RVESV and RVEF. Niemann
et al.12 used a Beutel semiautomated method
(Fig. 73.2C) to study 17 adults and 16 children with
congenital heart disease, including tetralogy of Fallot,
transposition of the great arteries, truncus arteriosus, atrial
septal defect, ventricular septal defect (VSD), coarctation

Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children

1725

Figs 73.2A to C: (A) The disc summation method for measurement of right ventricular (RV) volumes and ejection fraction (EF)
in children. Manual tracing of the RV endocardial contours was
performed on a stack of short-axis images. The four-chamber
views and coronal views are used as reference images; (B) Simpsons principle is used to derive RVEDV, RVESV, and right ventricular ejection fraction (RVEF); (C) The semiautomated method
for measurement of the RV indices. The method is based on the
Beutel model with manual tracing of the RV endocardial land
marks, including a set of coronal, sagittal, and four-chamber views.
(LV: Left ventricle; RV: Right ventricle).
Source: Adapted from Ref. 96.

of the aorta, and atrioventricular septal defect. They used

a semiautomated border detection method in comparison
with CMR. Excellent correlation was obtained, with corre
lation coefficients of 0.99 for RVESV, 0.95 for RVEDV, and
0.97 for EF.
Therefore, as seen in adults with cardiovascular
disease, including congenital heart disease,5459 quanti
tative analysis of RV volumes and EF using RT3DE is also
feasible in children. The limitations of RT3DE for quanti
tative analysis of the RV are related to image quality, border
clarity, and the difficulty imaging the entire RV, especially
in patients with an enlarged RV, such as those who have
undergone repair of tetralogy of Fallot.

Meta-Analysis
Shimada et al.60 performed a meta-analysis of published
studies comparing assessment of RV volumes and EF by
RT3DE versus CMR. The analysis included 23 studies and
807 adults and children. They concluded that compared
with CMR, 3DE significantly underestimated RVESV,

RVEDV, and EF, particularly in patients with larger volumes

(> 200 mL) and in younger patients (18 years). There was
no improvement for the analysis with time. The use of
matrix-array transducer did not improve the correlation
either. There was no statistical difference with the use of
automated compared with manual border detection.
Although RT3DE is highly promising for the evaluation of
RV indices, further investigation is warranted to address
the limited feasibility and accuracy before widespread
clinical application.2

SINGLE VENTRICULAR VOLUMES,

EJECTION FRACTION, AND MASS
One of the most complex types of congenital heart
disease in children is the functional single ventricle. These
patients often require two to three stages of palliation,
ultimately resulting in a Fontan type of palliation, and they
often have long-term structural and functional compli
cations, arrhythmias, exercise intolerance, and limited

1726

Section 6: Congenital Heart Disease

neurodevelopmental outcomes. RT3DE requires no geo

metric assumptions and is ideally suited for quantification
of volumes and EF for this type of congenital heart disease.
Altmann et al.61 first used acoustic spatial locator 3D
reconstructive technology to assess the volume and EF
of single ventricles with LV morphology in comparison
with 2DE and CMR. They studied 12 patients and showed
superior agreement with less data scatter in ESV, EDV,
mass, and EF between 3DE (bias 3.4 5.5 mL, 14.2 8.3
mL, 5.8 8.4 g, and 4.4% 5.3%, respectively) and CMR
than between 2DE and CMR (2.9 8.1 mL, 2.9 10.4 mL,
8.3 12.0 g, and 8.5% 10.3%, respectively). The interand intraobserver variabilities were more favorable for
3DE (5.77.1%) compared with CMR than for 2D compared
with CMR (1024%). Altmann et al. concluded that 3DE
provides estimates of ventricular volumes, EF, and mass
that are comparable to CMR in this select group of patients
with single ventricles of LV morphology.
Soriano et al.62 applied RT3DE for the estimation of
volumes, mass, and EF in patients with single ventricles
using a disc-summation technique. RT3DE was feasible
in 27 of 29 patients (93%). The RT3DE EDV correlated well
but was smaller than with CMR, and 3DE EF was smaller
than with CMR. There was no significant difference in
measurements of ESV or mass. The 3DE interobserver
differences for mass and volumes were not significant
except for measurement of EF. Intraobserver differences
were not significant. They speculated that the modest
correlation in EF between RT3DE and CMR was related
in part to the small range of EFs in this single-ventricle
population. This study suggested that RT3DE quanti
fication of EDV, ESV, mass, and EF in patients with single
ventricles is feasible, reproducible, and reasonably
accurate, except in measurement of EF, and warrants
further validation.
We showed that a long-axis, eight-plane Simpsons
technique is also feasible to assess volumes and EF for the
functional single ventricle. Furthermore, our study showed
that EF values are an independent predictor of transplantfree survival for this group. EF < 40% is associated with
significantly lower freedom of transplant-free survival in
this group of patients.14

THREE-DIMENSIONAL ANALYSIS
OF REGIONAL WALL MOTION,
SYNCHRONY, AND STRAIN
Regional Wall Motion and Synchrony
The capability of RT3DE to capture the entire LV in three
dimensions offers the opportunity not only to assess

global LV function more accurately and reproducibly

but also to quantify the regional function of the 16 LV
segments and to assess LV intraventricular synchrony.
To analyze LV regional function and synchrony, the cyclic
changes of the volumes of each of the 16 LV segments
are plotted against time over the course of the cardiac
cycle. The volume curves and plots allow measurement of
volume changes of each segment (i.e. segmental motion)
and temporal differences of each segment to minimum
volume (i.e. synchrony). Regional minimal volume (i.e.
maximal contraction) normally occurs at the same time
in ventricular systole for all segments. The systolic dyssyn
chrony index (SDI) is calculated as the standard deviation
of time to regional minimal volume of the segments.
Studies in the pediatric literature have validated
use of this new technology to assess LV synchrony in
volume and pressure loading conditions, the effect of
ventricular septal patch repair to LV synchrony, and
outcome of resynchronization therapy in patients with
a single ventricle with LV morphology. Kobayashi et al.63
prospectively studied 27 children with end-stage renal
disease (13 on peritoneal dialysis and 14 on hemodialysis)
and 29 normal controls. SDI was normalized to cardiac
cycle duration (SDIp). SDIp (16 segments) and LV mass
were significantly greater in the hemodialysis group. SDI
and SDIp (16 segments) improved after a hemodialysis
session (P < 0.05); LV mass and LV mass index remained
unchanged. LV dyssynchrony was significantly greater
in patients with LV hypertrophy compared with those
without. Their study demonstrated an association between
LV volume load and LV hypertrophy with significant LV
dyssynchrony in end-stage renal disease. SDI may provide
a sensitive marker for mechanistic and early detection of
LV dysfunction.
Veeram et al.64 studied patients with large VSDs who
underwent surgical patch closure during infancy to
investigate the long-term effects of the presence of akinetic
patch in the ventricular septum and postoperative right
bundle branch block (RBBB) on LV mechanical synchrony
and global systolic function. Their study showed that
pediatric patients 510 years after VSD patch closure have
normal LV function. The presence of the RBBB was associated
with mechanical dyssynchrony and tendency toward LV
dilatation in this group of patients. They demonstrated
that RT3DE is a sensitive technique for assessing LV
synchrony in postoperative VSD patients, especially
those with RBBB, who require long-term follow-up
for potential complications.
Finally, Bacha et al.65 performed multisite pacing
studies in 26 patients undergoing stepwise single-ventricle

Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children

1727

Figs 73.3A and B: Three-dimensional speckle tracking echocardiography (3DSTE) offline analysis. The endocardial borders were
manually traced at left ventricle (LV) end-diastole and end-systole from the apical four-chamber, two-chamber, and LV long-axis views,
respectively. The LVEDV, LVESV, left ventricular ejection fraction (LVEF), systolic dyssynchrony index (SDI), LV three-dimensional (3D)
systolic peak global (A) and segmental strain (B) were automatically calculated by the software. The mean systolic 3D peak strain of the
16 segments represented LV global strain. The mean basal, mid, and apical segmental systolic 3D peak strain represented the LV segmental peak systolic strain, respectively. The 3D strain indices derived included global systolic strain (GSS); global longitudinal systolic
strain (GLSS); systolic strain of basal, middle, and apical segments.
Source: Adapted from Ref. 96.

palliation. RT3DE was used in 10 subjects. They observed

significant response to multisite pacing, including
(a) improvement in QRS duration in 24 of 26 patients
(93.9 17.5 ms vs 71.7 10.8 ms; P < 0.001); (b) increase
in systolic blood pressure in 25 of 26 patients (86.3 20.0
mm Hg vs 93.8 20.2 mm Hg; P < 0.001); (c) increase in
cardiac index in 21 of 22 patients (3.2 0.8 vs 3.7 1.0 L
min1 m2; P < 0.001); and (c) improvement in index
of asynchrony in 8 of 10 patients (10.3 4.8 vs 6.0 1.4;
P < 0.04). The study suggests that RT3DE is potentially
useful in assessing cardiac resynchronization therapy even
in cases with complex geometries.

Strain Analysis by Real time

Three-Dimensional Echocardiography
Conventional indices of regional and global ventricular
function, defined by endocardial excursion, such as
fractional shortening and EF, are considered to be loaddependent.66,67 Myocardial velocities by tissue Doppler
imaging (TDI) do not rely on geometric assumptions but
are inherently unidimensional, angle-dependent, variable
with aging, and influenced by anthropometrics and heart
rate.68,69 Myocardial strain has been shown to be more
robust for assessment of regional ventricular myocardial
function using both echocardiography and CMR.7074 TDIbased strain analysis has been extensively validated and
has been shown to be limited as a one-dimensional and

angle-dependent method.70 Recently, three-dimensional

speckle tracking echocardiography (2DSTE) has been
introduced as a new method to quantify myocardial strain.
This technique measures myocardial deformation by
means of frame-by-frame tracking and motion analysis
of speckles within B-mode images. Validation studies
with tagged CMR and sonomicrometry in adults70,71 and
children75,76 have provided evidence that 2DSTE is a reliable
method for determining ventricular myocardial function.
2DSTE has many limitations, however. Firstly, myocardial
deformation measurement by 2DSTE is affected by loss
of speckles due to motion outside the imaging plane.77
Secondly, 2DSTE has limited reproducibility, likely owing
to the variabilities in the choice of image planes and lack
of standardization in image analysis.78 Finally, the analysis
is cumbersome and six planes are needed for complete
analysis, which is a major limitation for automation and
potential clinical use.
More recently, the advent of three-dimensional speckle
tracking echocardiography (3DSTE) (Figs 73.3A and B)
has shown the potential to overcome the limitations
of 2DSTE imaging for the assessment of LV global
and regional systolic function. This method tracks the
motion of speckles within the scan volume, allowing
more complete and accurate assessment of myocardial
deformation in 3D space7982 by avoiding the loss of
speckles due to out-of-plane motion. 3DSTE has been
validated for the quantification of LV volumes82 and LV wall

1728

Section 6: Congenital Heart Disease

motion in ischemic heart disease83 in adults. Nonetheless,

data are scarce and incomplete for children with or
without congenital heart disease.75,76,84 Thus, feasibility,
reproducibility, maturational changes, and normal values
in this population must be shown before these indices can
be used for assessing LV function for diagnosis, prognosis,
and risk stratification of various congenital and acquired
heart diseases in the young before and after medical,
percutaneous, and surgical intervention.
We used RT3DE to study 256 consecutive healthy
subjects using full-volume 3D data acquisition with a 3D
matrix-array transducer.85 Study subjects were divided
into five age groups: group 1 (birth to age 1 year); group
2 (15 years); group 3 (69 years); group 4 (1013 years);
and group 5 (1418 years). The 3D LV global strain (GS),
global longitudinal peak systolic strain (GLS), global radial
peak systolic strain (GRS), and global circumferential
peak systolic strain (GCS) values were determined using
modality-independent software. A total of 228 cases
(89%) were adequate for analysis and 28 cases (11%) were
excluded, including 10 cases excluded owing to the low
frame rates as determined by the system and 18 cases
excluded owing to poor 3D images. We found statistically
significant differences among the five age groups for GLS
and GCS, but no statistical difference between the age
groups for GRS and GS values. Multiple linear regression
analyses showed that LV and RV dimension, mass, volume,
and EF were significantly associated with GLS, GS, GRS,
and GCS. The interobserver variability of 3D systolic strain
parameters ranged from 1.2% to 9.5%, and intraobserver
variability ranged from 0.5% to 3.8%. Our study concluded
that global 3D systolic strain measurement using the new
3D RT STE is feasible and reproducible in children. GLS
and GCS are higher in early puberty and lower in late
puberty compared with infancy and early childhood. GRS
and GS have no maturational changes. Future work will
define the z scores for these measurements and in various
congenital and acquired heart diseases for potential
clinical application and research.
To use this new methodology to evaluate LV strain
and function, Saltijeral et al.86 evaluated 30 consecutive,
nonselected obese children and 42 healthy volunteer
children using a similar RT3DE wall motion tracking
method. They found statistically significant differences
in interventricular septum thickness, LV posterior wall
thickness, LVEDV, LVESV, left atrium volume, LV mass, and
lateral annulus peak velocity between the groups as well as
in all the 3D wall motion tracking variables. By multivariate
logistic regression analysis, the strongest relationship with

obesity was found for LV average circumferential strain

(b coefficient = 0.74; r2 = 0.55; P = 0.003). Their data
suggested that obesity-related cardiomyopathy is
associated not only with structural cardiac changes but also
with myocardial deformation changes in the childhood.
Assessment of LV circumferential strain using 3D wall
motion tracking is a marker with promising sensitivity for
identifying obesity-related cardiomyopathy.
The limitations of this technology include limited
spatial and temporal resolution of the 3DE data sets and
the variability in the measurements secondary to the
hardware, software, and algorithms provided by various
vendors. Further validation, improvement, and standar
dization are warranted for clinical application.

FUTURE PERSPECTIVES
Much evidence suggests that in the presence of adequate
image quality, LV volumes, mass, and EF measurements
by 3DE agree more closely with CMR measurements
and have better reproducibility than does 2DE, making
3DE the modality of choice for the everyday clinical
evaluation of LV volumes and EF. Quantitative analysis
of volumes, mass, and EF for the RV and for single
ventricles holds great promise and warrants future
investigation. New modes of regional wall motion, strain,
and LV dyssynchrony assessment using RT3DE are in
the forefront of active research. Future advancements in
hardware are expected to allow acquisition of wide-angle
3D data from the entire heart in a single cardiac cycle,
with higher spatial and temporal resolution. We anticipate
that further development in automatic quantitative
analysis algorithms and software will make RT3DE an
integral clinical methodology for diagnosis, prognosis,
and assessment of medical, percutaneous, and surgical
intervention for congenital heart disease.87

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Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children

54. Grewal J, Majdalany D, Syed I, et al. Three-dimensional

echocardiographic assessment of right ventricular volume
and function in adult patients with congenital heart
disease: comparison with magnetic resonance imaging.
J Am Soc Echocardiogr. 2010;23(2):12733.
55. Grapsa J, ORegan DP, Pavlopoulos H, et al. Right ventricular
remodelling in pulmonary arterial hypertension with
three-dimensional echocardiography: comparison with
cardiac magnetic resonance imaging. Eur J Echocardiogr.
2010;11(1):6473.
56. Grison A, Maschietto N, Reffo E, et al. Three-dimensional
echocardiographic evaluation of right ventricular volume
and function in pediatric patients: validation of the
technique. J Am Soc Echocardiogr. 2007;20(8):9219.
57. Khoo NS, Young A, Occleshaw C, et al. Assessments of right
ventricular volume and function using three-dimensional
echocardiography in older children and adults with
congenital heart disease: comparison with cardiac
magnetic resonance imaging. J Am Soc Echocardiogr.
2009;22(11):127988.
58. van der Hulst AE, Roest AA, Holman ER, et al. Real-time
three-dimensional echocardiography: segmental analysis
of the right ventricle in patients with repaired tetralogy of
Fallot. J Am Soc Echocardiogr. 2011;24(11):118390.

59. van der Zwaan HB, Geleijnse ML, McGhie JS, et al.
Right ventricular quantification in clinical practice: twodimensional vs. three-dimensional echocardiography
compared with cardiac magnetic resonance imaging. Eur
J Echocardiogr. 2011;12(9):65664.
60. Shimada YJ, Shiota M, Siegel RJ, et al. Accuracy of right
ventricular volumes and function determined by threedimensional echocardiography in comparison with
magnetic resonance imaging: a meta-analysis study. J Am
Soc Echocardiogr. 2010;23(9):94353.
61. Altmann K, Shen Z, Boxt LM, et al. Comparison of threedimensional echocardiographic assessment of volume,
mass, and function in children with functionally single
left ventricles with two-dimensional echocardiography
and magnetic resonance imaging. Am J Cardiol. 1997;
80(8):10605.

62. Soriano BD, Hoch M, Ithuralde A, et al. Matrix-array
3-dimensional echocardiographic assessment of volumes,
mass, and ejection fraction in young pediatric patients with a
functional single ventricle: a comparison study with cardiac
magnetic resonance. Circulation. 2008;117(14):18428.
63. Kobayashi D, Patel SR, Mattoo TK, et al. The impact of
change in volume and left-ventricular hypertrophy on leftventricular mechanical dyssynchrony in children with endstage renal disease. Pediatr Cardiol. 2012 Mar 23. [Epub
ahead of print].
64. Veeram Reddy SR, Du W, Zilberman MV. Left ventricular
mechanical synchrony and global systolic function in
pediatric patients late after ventricular septal defect patch
closure: a three-dimensional echocardiographic study.
Congenit Heart Dis. 2009;4(6):4548.
65. Bacha EA, Zimmerman FJ, Mor-Avi V, et al. Ventricular
resynchronization by multisite pacing improves myocardial

1731

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66. Dragulescu A, Mertens LL. Developments in echocar
diographic techniques for the evaluation of ventricular
function in children. Arch Cardiovasc Dis. 2010;103
(11-12):60314.
67. Pacileo G, Di Salvo G, Limongelli G, et al. Echocardiography
in congenital heart disease: usefulness, limits and new
techniques. J Cardiovasc Med (Hagerstown). 2007;8(1):
1722.
68. Gorscan J III, Strum DP, Mandarino WA, et al. Quanti
tative assessment of alterations in regional left ventricular
contractility with color-coded tissue Doppler echocar
diography. Circulation. 1997;95:242333.
69. Eidem BW, McMahon CJ, Cohen RR, et al. Impact of cardiac
growth on Doppler tissue imaging velocities: a study
in healthy children. J Am Soc Echocardiogr. 2004;17(3):
21221.
70. Korinek J, Wang J, Sengupta PP, et al. Two-dimensional
straina Doppler-independent ultrasound method for
quantitation of regional deformation: validation in vitro
and in vivo. J Am Soc Echocardiogr. 2005;18(12):124753.
71. Amundsen BH, Helle-Valle T, Edvardsen T, et al. Noninvasive
myocardial strain measurement by speckle tracking
echocardiography: validation against sonomicrometry and
tagged magnetic resonance imaging. J Am Coll Cardiol.
2006;47(4):78993.
72. Young AA, Axel L, Dougherty L, et al. Validation of tagging
with MR imaging to estimate material deformation.
Radiology. 1993;188(1):1018.
73. Amundsen BH, Helle-Valle T, Edvardsen T, et al. Noninvasive
myocardial strain measurement by speckle tracking
echocardiography: validation against sonomicrometry and
tagged magnetic resonance imaging. J Am Coll Cardiol.
2006;47(4):78993.
74. Moore CC, Lugo-Olivieri CH, McVeigh ER, et al. Threedimensional systolic strain patterns in the normal human
left ventricle: characterization with tagged MR imaging.
Radiology. 2000;214(2):45366.

75. Marcus KA, Mavinkurve-Groothuis AM, Barends M,
et al. Referen\nics using speckle tracking echocardi
ography: fundamentals and clinical applications. J Am Soc
Echocardiogr. 2010;23(4):35169; quiz 453.
78. Bansal M, Cho GY, Chan J, et al. Feasibility and accuracy
of different techniques of two-dimensional speckle based
strain and validation with harmonic phase magnetic
resonance imaging. J Am Soc Echocardiogr. 2008;21(12):
131825.
79. Dhooge J, Konofagou E, Jamal F, et al. Two-dimensional
ultrasonic strain rate measurement of the human heart
in vivo. IEEE Trans Ultrason Ferroelectr Freq Control.
2002;49(2):2816.
80. Perez de Isla L, Balcones DV, Fernandez-Golfin C, et al.
Three-dimensional-wall motion tracking: a new and faster
tool for myocardial strain assessment: comparison with two
dimensional wall motion tracking. J Am Soc Echocardiogr.
2009; 22:32530.

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Section 6: Congenital Heart Disease

81. Gayat E, Ahmad H, Weinert L, et al. Reproducibility and

inter-vendor variability of left ventricular deformation
measurements by three-dimensional speckle-tracking
echocardiography. J Am Soc Echocardiogr. 2011;24(8):
87885.
82. Nesser HJ, Mor-Avi V, Gorissen W, et al. Quantification of
left ventricular volumes using three-dimensional echocar
diographic speckle tracking: comparison with MRI. Eur
Heart J. 2009;30(13):156573.
83. Maffessanti F, Nesser HJ, Weinert L, et al. Quantitative
evaluation of regional left ventricular function using
three-dimensional speckle tracking echocardiography in
patients with and without heart disease. Am J Cardiol.
2009;104(12):175562.
84. Bussadori C, Moreo A, Di Donato M, et al. A new 2D-based
method for myocardial velocity strain and strain rate
quantification in a normal adult and paediatric population:
assessment of reference values. Cardiovasc Ultrasound.
2009;7:8.

85. Zhang L, Gao J, Xie M, et al. Left ventricular threedimensional global systolic strain by real-time threedimensional speckle-tracking in children: feasibility,
reproducibility, maturational changes, and normal ranges.
J Am Soc Echocardiogr. 2013; 26(8):853-9.

86. Saltijeral A, Isla LP, Prez-Rodrguez O, et al. Early
myocardial deformation changes associated to isolated
obesity: a study based on 3D-wall motion tracking analysis.
Obesity (Silver Spring). 2011;19(11):226873.
87. Zhang L, Xie M, Balluz R, Ge S. Real time three-dimensional
echocardiography for evaluation of congenital heart
defects: state of the art. Echocardiography. 2012;29(2):
23241.

88. Caiani EG, Corsi C, Zamorano J, et al. Improved
semiautomated quantification of left ventricular volumes
and ejection fraction using 3-dimensional echocar
diog
raphy with a full matrix-array transducer: comparison
with magnetic resonance imaging. J Am Soc Echocardiogr.
2005;18(8):77988.

89. Jacobs LD, Salgo IS, Goonewardena S, et al. Rapid online

quantification of left ventricular volume from real-time
three-dimensional echocardiographic data. Eur Heart J.
2006;27(4):4608.
90. Nikitin NP, Constantin C, Loh PH, et al. New generation
3-dimensional echocardiography for left ventricular
volumetric and functional measurements: comparison
with cardiac magnetic resonance. Eur J Echocardiogr.
2006;7(5):36572.
91. Krenning BJ, Kirschbaum SW, Soliman OI, et al. Comparison
of contrast agent-enhanced versus non-contrast agentenhanced real-time three-dimensional echocardiography
for analysis of left ventricular systolic function. Am J
Cardiol. 2007;100(9):14859.
92. Qi X, Cogar B, Hsiung MC, et al. Live/real time threedimensional transthoracic echocardiographic assessment
of left ventricular volumes, ejection fraction, and mass
compared with magnetic resonance imaging. Echocar
diography. 2007;24(2):16673.
93. Macron L, Lim P, Bensaid A, et al. Single-beat versus
multibeat real-time 3D echocardiography for assessing left
ventricular volumes and ejection fraction: a comparison
study with cardiac magnetic resonance. Circ Cardiovasc
Imaging. 2010;3(4):4505.
94. Chang SA, Lee SC, Kim EY, et al. Feasibility of singlebeat full-volume capture real-time three-dimensional
echocardiography and auto-contouring algorithm for
quantification of left ventricular volume: validation
with cardiac magnetic resonance imaging. J Am Soc
Echocardiogr. 2011;24(8):8539.
95. Iriart X, Montaudon M, Lafitte S, et al. Right ventricle
three-dimensional echography in corrected tetralogy
of Fallot: accuracy and variability. Eur J Echocardiogr.
2009;10(6):78492.
96. Balluz R, Liu L, Zhou X, et al. Real time three-dimensional
echocardiography for quantification of ventricular volumes,
mass, and function in children with congenital and acquired
hear diseases. Nanda NC (Editor). Echocardiography.
2013;30(4):47282.

CHAPTER 74
Three-Dimensional Echocardiography
in Congenital Heart Disease
Steven Bleich, Gerald R Marx, Navin C Nanda, Fadi G Hage

Snapshot

Shunt Lesions/Septal Defects

Common Atrium

Aortopulmonary Window

Patent Ductus Arteriosus (PDA)

Conotruncal Anomalies

Oulow Tract Obstrucon

INTRODUCTION
Congenital heart disease (CHD) includes an extensive
group of diagnoses affecting both pediatric and
adult patients. The complexity of CHD has motivated
cardiologists to better understand each individual defect
in order to maximize management of these patients
and improve treatment options. Two-dimensional (2D)
echocardiography has been the gold standard imaging
modality used because of its convenience, low cost, and lack
of harmful side effects as compared to magnetic resonance
imaging (MRI) and multidetector computed tomography
(CT).1,2 However, identifying and understanding CHD
often requires a real life, three-dimensional (3D) image
of the defect.35 With 2D echocardiography, the reader
often expends effort to mentally reconstruct the heart and
great vessels in three dimensions, sometimes, preventing
visualization of relevant details. The emergence of 3D
echocardiography (3DE) provides a more comprehensive
view of the cardiac anatomy which offers incremental
value in the diagnosis of CHD.
3DE has gained popularity in the field of CHD because
it can portray a dynamic and realistic picture of the

Aorc Arch Anomalies

Atrial and Atrioventricular Valve Abnormalies

Other Abnormalies

Double Outlet Right Ventricle

Sinus of Valsalva Aneurysm

structural defect and nearby cardiac structures.6 Views

previously unobtainable by 2D echocardiography are
captured readily by 3D imaging offering further insight
regarding morphology, location, specific dimensions, and
dynamic changes of specific heart structures during the
cardiac cycle.4 3DE enhances cardiologists understanding
of CHD by providing unique and valuable data that is
useful in determining the appropriate treatment option for
each specific defect.5

SHUNT LESIONS/SEPTAL DEFECTS

Longstanding congenital heart defects causing elevated
pulmonary artery pressure can cause significant strain on
the right ventricle, ultimately resulting in right ventricular
remodeling and dysfunction. The ability to measure right
ventricular size and function can guide medical and
surgical management before deterioration in right
ventricular function. For example, in patients following
surgical repair of Tetralogy of Fallot (ToF), follow-up
imaging to detect ventricular dysfunction and remodeling
can help to determine the appropriate time for placement
of a competent pulmonary valve to avoid further

1734

Section 6: Congenital Heart Disease

complications such as right ventricular failure, arrhythmia,

and sudden cardiac death.7 Unfortunately, based on
the intricate shape of the right ventricle, measurement
of its size and function pose a challenge to traditional
2D imaging.7 Cardiac MRI has previously been utilized
to measure this data, but due to its cost, extensive time
necessary to obtain and interpret images, and restrictive
accessibility, its use is limited.7,8 3D-transthoracic
echocardiography (TTE) has considerable advantage over
2D TTE because of its ability to accurately assess volume
and function of the right ventricle based on the unique
views it provides.9 Strong correlations between 3DE and
cardiac MRI for the quantitative analysis of the right
ventricle have been reported, but limitations including
underestimation of right ventricular volume and the
visualization of the entire right ventricle in patients with
an enlarged right ventricle in a single imaging window
must be recognized.7 With regard to 3DE, the American
Society of Echocardiography (ASE) suggests that capturing
higher quality images may facilitate increased utilization
of this modality.10
Equally important in CHD is the assessment of single
ventricular size and function. Studies comparing 3D
echocardiographic measurements of single ventricle
volumes, mass, and ejection fraction compare favorably
to MRI in young infants.11 The ability to measure left
ventricular volumes in patients with borderline left hearts
being considered for either single ventricle palliative
surgeries or biventricular repair is also significant. Often
the ventricles are spherical and thus 2D imaging relies on
mathematical assumptions that often do not apply. Threedimensional imaging has been shown to again provide
measurements of left ventricular size and function in small
young infants as compared to MRI.12

Atrial Septal Defects (ASDs)

Atrial septal defects represent 710% of all congenital heart
defects, often diagnosed incidentally in asymptomatic
adults.13,14 An ASD can consist of one or multiple defects
and understanding the extent of the defects is key in
planning for the appropriate corrective procedure
(Figs 74.1A to D). This is especially germane when
considering defects for catheterization closure. There are
several different types of ASDs depending on their location
in the atrial septum and they are discussed below.

Secundum ASD
Secundum ASDs and patent foramen ovale (PFO) occupy
the middle portion of the atrial septum. Although 2D
echocardiography has historically been the imaging
modality of choice in diagnosing these ASDs, images
obtained via this method can lack optimal viewing of the
defect and therefore distort the measurements obtained.15
In the setting of multiple fenestrations such as the Swiss
cheese pattern or multiple hole pattern, the limitations of
2D TTE are even more obvious. The key to any successful
echocardiographic image relies on a clear imaging
window with the patient in a position that provides the
best view for the reader. 3D TTE performed using the
apical, para-apical, right parasternal and subcostal views
provides adequate en face visualization of the defect in
most patients (Figs 74.2 and 74.3). The interatrial septum
is often best observed in the right parasternal view with
the patient in the right lateral decubitus position.15
Information regarding exact dimensions and location of
an ASD along with relative surrounding cardiac anatomy
and rim size can be obtained more easily by 3D TTE than
2D transesophageal echocardiography (TEE).15,16 With
the advent of real time 3D TTE, a PFO can be visualized
as well as the motion of the valve covering it (Figs 74.4
and 74.5).17 Recognizing the development of worsening
dyspnea and hypoxia with change in position from the
supine to sitting position suggests the possibility of
platypnea-orthodeoxia syndrome. 3D TEE has been used
to document an open PFO with shunting of blood in the
sitting position and closed PFO in the supine position
(Figs 74.6 and 74.7).18 One study done by Morgan et al.
compared the results obtained by 2D TEE to 3D TTE
with respect to maximum defect diameter, area and
circumference of an ASD.19 Data obtained from these two
modalities were not statistically different, however, 3D
TTE compared to 2D TEE provided clinically significantly
information. 3D was able to recognize appropriate
candidates for percutaneous closure of an ASD while
eliminating the need for more invasive imaging with TEE.
Repair of ASDs have traditionally been performed
with surgery, however, recent evidence recommends the
use of more percutaneous closure devices. The decision
to proceed with surgery versus percutaneous approach
depends on the location of the ASD, its shape and size,
and the surrounding tissue.4,15 In order to proceed with
percutaneous repair, the cutoff with regard to rim size has

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1735

Figs 74.1A to D: Live three-dimensional right parasternal transthoracic echocardiographic examination of atrial septum and superior and inferior vena cavae. (A and B) Color Doppler examination in another patient showing four (numbered 1 through 4) separate
secundum defects at different levels of the atrial septum; (C and D) Two of these defects are viewed en face. In this 25-year-old female,
four atrial septal defects at different levels of the atrial septum could be demonstrated by sequential cropping of the three-dimensional
dataset. Only two of these defects could be visualized by two-dimensional transthoracic echocardiography. (LA: Left atrium; RA: Right
atrium). (Movie clip 74.1).
Source: Reproduced with permission from Patel V, Nanda NC, Upendram S, et al. Live three-dimensional right parasternal and supraclavicular transthoracic echocardiographic examination. Echocardiography. 2005;22:34960.

been set at 5 mm. Obtaining a comprehensive view of the

defect and nearby cardiac structures is feasible with 3D
TTE compared to 2D echocardiography, which helps in
determining the appropriate therapeutic intervention.20
In patients with secundum ASD, the most common type
of ASD encountered in clinical practice, 3D TTE can
view the defect and surrounding rims of tissue which is
valuable when planning a transcatheter approach.15,21 The
ability of 3D TTE to calculate accurate measurements of
the defect size has been validated by recording similar
measurements during surgical and percutaneous
repair.15,22 Knowing the exact dimensions of the defect is

important when selecting the size of the occluder device

which can prevent postprocedure complications. Placing
of an inappropriately sized occluder device can lead
to persistence of the shunt, invasion of nearby cardiac
anatomy, breakdown of the device, device embolization
and even perforation of the heart.23 Understanding the
serious nature of these complications substantiates the
need to precisely document the defect, its size, and all
relevant cardiac anatomy. Furthermore, in the setting of an
above mentioned complication, 3DE has been life saving
in visualizing the abnormality and allowing immediate
repair.24 Color Doppler 3D TTE can identify a small residual

1736

Section 6: Congenital Heart Disease

Figs 74.2A and B: Live three-dimensional transthoracic echocardiographic (3D TTE) assessment of atrial septal defect. Arrowhead
points to a large secundum atrial septal defect visualized from both right (RA, A) and left atrial (LA, B) aspects. Note the large rim of
tissue surrounding the defect. (AS: Atrial septum). Movie clip 74.2, Part 1 shows en face viewing of a small atrial septal defect from both
the left and right atrial aspects. There is an ample rim of tissue surrounding the defect. Movie clip 74.2, Part 2 from another adult patient
with a secundum atrial septal defect. Regular and QLab (Philips Medical Systems, Andover, MA) cropping of the 3D data set views the
large defect en face (two arrowheads). Reasonable amount of atrial septal tissue surrounds the defect. The defect measures 3.2 3.1
cm, area 9.2 cm2. Movie clip 74.2, Part 3 from another patient with a secundum atrial septal defect. The two-dimensional study shows a
large defect (arrowhead) in the apical 4-chamber and subcostal views. Regular and QLab croppings view the large defect en face (two
arrowheads/arrows). Although the defect is similar in size to the previous patient, there is hardly any rim of the tissue adjacent to the
aorta making it hazardous to close it in the catheterization laboratory. Movie clip 74.2, Parts 4 and 5. In this large patient, a secundum
defect in the atrial septum (AS) was suspected during subcostal examination but a definitive diagnosis could not be made. When the
images were acquired using a three-dimensional transducer and cropped, the defect (arrow) was clearly visualized with left and right
shunting. (LA: Left atrium; RA: Right atrium). [Movie clip 74.2, (Parts 1 to 5)].
Source: Reproduced with permission from Mehmood F, Vengala S, Nanda NC, et al. Usefulness of live three-dimensional transthoracic
echocardiography in the characterization of atrial septal defects in adults. Echocardiography J. 2004;21:70713.

Fig. 74.3: Three-dimensional image of atrial septal defect: en

face view of the atrial septum, from the right atrium. Accurate
description of the surrounding rims and their measurements
provided by TomTec software, in the preoperative assessment of
this young patient. (AV: Atrioventricular; IVC: Inferior vena cava;
SVC: Superior vena cava; RV: Right ventricle; RA: Right atrium).
Source: Reproduced with permission from De Castro S, Caselli
S, Papetti F, et al. Feasibility and clinical impact of live threedimensional echocardiography in the management of congenital
heart disease. Echocardiography. 2006;23:55361.

shunt after the placement of an ASD occluder device that

often occurs and usually resolves over time.25 3D TTE is
useful in evaluating the morphology and effectiveness of
percutaneous closure devices used for closure of an ASD
and PFO (Figs 74.8 to 74.10).

Current ASE guidelines advocate for the utilization of

2D TEE during a percutaneous closure repair because of
precise measurements of the ASDs maximum dimension
and bordering septal rims, however, its limitations must
be understood.24 The success of 2D TEE relies on the

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1737

Figs 74.4A and B: Live three-dimensional transthoracic echocardiography assessment of patent foramen ovale (PFO). (A and B)
Arrow shows the PFO while the arrowheads point to contrast signals moving through the defect into the left atrium (LA) following an
intravenous agitated saline injection. 3D TTE, three-dimensional transthoracic echocardiographic; (LV: Left ventricle).
Source: Reproduced with permission from Mehmood F, Vengala S, Nanda NC et al. Usefulness of live three dimensional transthoracic
echocardiography in the characterization of atrial septal defects in adults. Echocardiography J. 2004;21:70713.

Figs 74.5A to D

1738

Section 6: Congenital Heart Disease

Figs 74.5A to E: Real time/three-dimensional transthoracic

echocardiographic visualization of the valve of foramen ovale. Right
parasternal approach. (A) Arrowhead points to the atrial septum; (B
and C) En face view of the atrial septum from the left atrial (LA) side
shows the valve of foramen of ovale in the closed (B) and open (C)
positions. In the closed position it completely covers the foramen
ovale; (D) Arrowhead points to a mobile flap of tissue at the junction
of superior vena cava (SVC) and right atrium (RA) representing a
remnant of right-sided sinus venosus valve; (E) Arrowhead shows
the Eustachian valve. (IVC: Inferior vena cava). [Movie clip 74.5,
(Parts 1 to 8)].
Source: Reproduced with permission from Panwar SR, Perrien
JL, Nanda NC, Singh A, Rajdev S. Real time/three-dimensional
transthoracic echocardiographic visualization of the valve of
foramen ovale. Echocardiography. 2007;24:11057.

Fig. 74.6: Transesophageal echocardiogram in the supine position. Echogram taken in the supine position showing the foramen
ovale is closed (right figure), and no apparent right-to-left shunt by
Doppler color flow (left figure). (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Sasaki T, Miyasaka Y,
Suwa Y, et al. Real time three-dimensional transesophageal echocardiographic images of platypnea-orthodeoxia due to patent foramen ovale. Echocardiography. 2013;30:E11617.

Fig. 74.7: Transesophageal echocardiogram in the sitting position. Echogram taken in the sitting position showing the foramen
ovale is wide open (right figure), with a massive right-to-left shunt
across the patent foramen ovale by Doppler color flow (left figure).
(LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Sasaki T, Miyasaka Y,
Suwa Y, et al. Real time three-dimensional transesophageal echocardiographic images of platypnea-orthodeoxia due to patent foramen ovale. Echocardiography. 2013;30:E11617.

observers ability to obtain unlimited angles and views

of the ASD and mentally recreate these images into 3D.
Undoubtedly, this task is difficult and may preclude a
comprehensive evaluation of the defect before, during
and after the procedure. 3D TEE offers a distinct en face
view of the defect compared to 2D TEE, providing accurate
measurements as well as confirmation of device placement
in its appropriate position.
3D TEE is effective in measuring the defect size, rim
size, left atrial (LA) and right atrial (RA) occluder disc

dimensions, and distance between the LA and the aorta in

patients with secundum ASD determined to be appropriate
candidates for a percutaneous closure procedure.24 There
have been reports of encroachment of the LA occluder disc
on the aorta observed by 3D TEE, putting patients at risk
of aortic erosion and even cardiac tamponade (Fig. 74.11).
Insufficient rim size and placement of a wrong sized
occluder device are likely contributing factors in causing
aortic erosions. Bhaya et al. found a correlation between
the maximum dimension of the ASD, the length of the LA

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1739

Figs 74.8A to F: Live three-dimensional transthoracic echocardiographic assessment of transcatheter closure of atrial septal defect.
(A) Arrow points to the waist of the atrial septal defect (ASD) transcatheter closure device; (B and C) Shows the device viewed from the
top (B) and obliquely (C). Note that the left atrial disc (# 1) is larger than the right atrial disc (# 2); (D) Arrowhead points to the stainless
steel screw thread located in the center of the right atrial disc viewed enface; (E) Arrow points to the small residual shunt seen one day
after the device was positioned; (F) Amplatzer device used for transcatheter closure of ASD. Arrowhead points to the metallic cap and
the arrow points to the waist of the device. (LA: Left atrium; LV: Left ventricle; MC: Metallic cap; NW: Nitinol winding; RA: Right atrium;
RV: Right ventricle; ST: Screw thread). Movie clip 74.8 shows 3D TTE examination of the atrial septal defect closure device 6 weeks
after implantation. The device is well seated. (Movie clip 74.8).
Source: Reproduced with permission from Sinha A, Nanda NC, Misra V, et al. Live three-dimensional transthoracic echocardiographic
assessment of transcatheter closure of atrial septal defect and patent foramen ovale. Echocardiography. 2004;21:74953.

disc, the waist size of the device, and the distance between
the LA disc and the aorta.24 3D TEE measurements of LA
and RA disc lengths have been nearly identical to the
manufacturers assigned size, validating its calculations.
3D TEE is capable of not only selecting proper patients for
the percutaneous procedure, but also identifying patients
at higher risk of complications prompting more regular
follow-up.
Device embolization is a serious complication of
percutaneous repair that can be minimized by accurate
measurements of the ASD and placement of an appropriate
sized occluder device. Wei et al. described an unfortunate

case of a patient with a complex Swiss cheese type secundum

ASD resulting in device embolization to the left atrium and
then right iliac artery (Figs 74.12A to G).26 Initial imaging
with 2D TTE was inaccurate compared to follow-up imaging
with 3D TTE in evaluating the complexity of the ASD based
on the absence of en face views of the entire atrial septum.
When defects include multiple fenestrations, the risk
of complications from a percutaneous repair increases
tremendously and alternative treatment options must be
considered.26 3D TTE can help to select good candidates for
percutaneous repair of ASD and to inform both patients and
physicians of the risk of possible complications.

1740

Section 6: Congenital Heart Disease

Fig. 74.9: Live three-dimensional transthoracic echocardiographic

assessment of transcatheter closure of patent foramen ovale (PFO).
Represent the left atrial and right atrial discs of the transcatheter
device used to close the PFO. (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Sinha A, Nanda NC, Misra V, et al. Live three-dimensional transthoracic echocardiographic
assessment of transcatheter closure of atrial septal defect and patent foramen ovale. Echocardiography. 2004;21:74953.

Figs 74.10A to G: Live three-dimensional transthoracic echocardiographic assessment of

transcatheter closure of atrial septal defect. In vitro studies. (A and B) Arrowhead shows the stainless
steel screw thread. # 1 and # 2 represent the left atrial and right atrial discs seen enface (A) and
from the side (B); (C) Arrowhead points to the metallic cap seen in the middle of the left atrial disc.
This is where the nitinol windings come together; (D to G) Real time, 2-dimensional transthoracic
echocardiography in embolization of atrial septal defect occlusion device; (D) Arrowhead points
to the device located at the bifurcation of the main pulmonary artery; (E) Color Doppler-guided
continuous-wave Doppler interrogation demonstrates absence of significant obstruction. Peak
velocity is only 1.47 m/s; (F and G) Live/real time, three-dimensional transthoracic echocardiography
in embolization of atrial septal defect occlusion device; (F) Arrowhead points to the device located
at the bifurcation of the main pulmonary artery; (G) Atrial septal defect viewed en face from the
right atrial side (dotted line). (AO: Aorta; LPA: Left pulmonary artery; RPA: Right pulmonary artery).
Source: Figs 10A to C Reproduced with permission from Sinha A, Nanda NC, Misra V, et al. Live
three-dimensional transthoracic echocardiographic assessment of transcatheter closure of atrial
septal defect and patent foramen ovale. Echocardiography. 2004;21:74953. Source: Figs 10D to
G Reproduced with permission from Dod HS, Reddy VK, Bhardwaj R, et al. Embolization of atrial
septal occluder device to the pulmonary artery: A rare complication and usefulness of live/real time
three-dimensional transthoracic echocardiography. Echocardiography. 2009;26:6.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1741

Fig. 74.11: Multiplanar rendering mode: Upper panels. Top

arrowheads point to the left atrial disc, lower arrowheads to the
right atrial disc. D1 represents the measurement of the radius of
the left atrial disc from the middle of the central marker band to
the outer edge of the disc. D2 represents the measurement of the
radius of the right atrial disc from the middle of the end screw to
the outer edge of the disc. The arrow in the top right panel points to
the area of contact of the left atrial disc with the aorta (AO) Lower
panels. Arrowhead in the right lower panel points to the left atrial
disc covering the atrial septal defect. Movie clip 74.11 shows the
left atrial disc in contact with the aorta throughout the cardiac cycle.
Source: Reproduced with permission from Bhaya M, Mutluer
FO, Mahan III EF, et al. Incremental utility of live/real time threedimensional transesophageal echocardiography in percutaneous
closure of atrial septal defects. Echocardiography. 2013;30:
34553.

Figs 74.12A to D

1742

Section 6: Congenital Heart Disease

Sinus Venosus ASD

Sinus venosus ASD (SVASD) represents ~4% to 11% of
ASDs.24 Some experts believe that a sinus venosus defect
is not a true ASD, but rather the failure of interposition
of the wall between the superior vena cava and the right
upper pulmonary veins of superior sinus venosus defects
or lack of the wall separating the right lower pulmonary
veins from the back wall of the right atrium in patients
with inferior sinus venosus defects. Hence, the veins
connect normally but drain abnormally due to this lack of
interposing walls and therefore, a sinus venosus defect
is often not characterized as simply a hole in the wall
between the atria.
The capabilities of 3DE have been shown to be
effective in assessing a sinus venosus defect, 3D TEE
reconstruction images confidently visualize the defect in

F
Figs 74.12A to G: Live/real time, three-dimensional transesophageal echocardiographic assessment of device embolization
during percutaneous atrial septal defect closure. (A) The arrowheads
point to multiple secundum atrial septal defects (ASD, swiss cheese
appearance) viewed en face from the left atrium (LA); (B) Color
Doppler assessment showing flow signals within the defects viewed en
face (left panel). QLAB examination (right panel) showing four defects
numbered 1, 2, 3, and 4; (C) QLAB examination demonstrating en
face view of one of the defects (1) using Color Doppler. In the upper
left panel the cropping plane is positioned exactly parallel to the defect
which resulted in en face viewing of the defect in the lower left panel.
Subsequently the area was measured by planimetry; (D) Demonstrates
the first ASD closure device (D1) in position (viewed from left atrium
and anatomically correct). Arrowhead shows a large residual defect
viewed en face. The arrow points to the device placement catheter;
(E) Shows the second ASD closure device (D2) in position, partially
overlapping D1 (viewed from left atrium and anatomically correct).
Arrowhead shows the presence of one of the two significant residual
defects; (F) Shows embolization of one of the closure devices (D) to
the LA; (G) Shows the device (D) in the proximal descending thoracic
aorta (DA) after percutaneous manipulation from the iliac artery. 1 and
2 denote the right and the left atrial sides of the device, which are
viewed en face in the left lower and the right upper panels. (MV: Mitral
valve; RA: Right atrium).
Source: Reproduced with permission from Wei J, Hsiung MC,
Tsai SK, et al. Atrial septal occluder device embolization to an
iliac artery: A case highlighting the utility of three-dimensional
transesophageal echocardiography during percutaneous closure.
Echocardiography. 2012;29:112831.

relation to the superior vena cava (SVC) and right superior

pulmonary vein offering significant advantage over 2D TEE
(Figs 74.13A to C).27 Both modalities are successful in
sizing the defect comparatively to actual surgical measurements, but 3D TEE was also capable of calculating the area
of the defect with the extra dimensions it obtained.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1743

Figs 74.13A to C: Multiplane transesophageal 3D reconstruction of sinus venosus ASD. (A) The arrowhead points to the large defect in
the superior portion of the atrial septum. The arrow shows the right superior PV entering the SVC-atrial junction at the site of the defect.
(B and C) Orthogonal views demonstrating the size of the defect (ASD), which measured 3.69 cm2 in area. The maximal dimension of
the defect was 2.15 cm, which corresponded to the diameter of 2 cm measured at surgery. The top arrowhead in B points to the right
superior PV, and the bottom arrowhead points to the defect. (ASD: Atrial septal defect; SVC: Superior vena cava; LA: Left atrium; RA:
Right atrium).
Source: Reproduced with permission from Nanda NC, Ansingkar K, Espinal M, et al. Transesophageal three-dimensional echo assessment of sinus venosus atrial septal defect. Echocardiography. 1999;16:8357.

Unroofed Coronary Sinus

Ventricular Septal Defects

Unroofed coronary sinus is an uncommon type of ASD

which involves a communication between the roof
of the coronary sinus and the left atrium and is often
diagnosed in combination with a persistent left superior
vena cava.28 Unroofing of the coronary sinus can also
occur intentionally with surgical repair when there is total
anomalous return of the pulmonary veins to the coronary
sinus.29 The diagnosis of unroofed coronary sinus can be
difficult to ascertain with 2D imaging, and when very large
can be mistaken for a primum ASD. Importantly, when
an unroofed coronary sinus defect is associated with a
left superior vena cava, right-to-left shunting can occur,
leading to serious side effects such as a cerebral emboli or
a brain abscess.28
An acquired unroofed coronary sinus was reported in
an adult patient after there was breakdown of the surgical
patch that was placed over the coronary sinus ostium to
repair total anomalous pulmonary venous return (TAPVR)
during infancy (Figs 74.14A to E).29 With its technological
advanced cropping capabilities and the views captured
from unique angles, 3D TTE clearly visualized the
communication between the coronary sinus and the left
atrium confirming the diagnosing suggested by coronary
angiography. Furthermore, 3D color Doppler imaging
confirmed the left-to-right shunting of blood from left
atrium through the defect in the roof of the coronary sinus
into the right atrium.

Ventricular septal defects (VSD) represent a commonly

diagnosed congenital heart defect in the newborns.
The defect is most often located within the muscular or
perimembranous portion of the interventricular septum.
The diagnosis of a VSD can be made by 2D echocardiography, however, the accuracy of its measurements are
often inconsistent because of the typical oval shape of
a VSD.30 As a result of its uneven contour, 2D views are
unable to accurately quantify the true dimensions of the
defect, often resulting in underestimation.4,30 The location,
dimensions and shape of the VSD are more accurately
assessed by live/real time 3D TTE (Figs 74.15A and B).22
With a more complex VSD, the capabilities of 3DE are even
more apparent when compared to 2D echocardiography.
3D TTE presents an en face view of the defect to provide
more reliable measurements of its area and circumference
to better assist the cardiologist in planning for treatment
(Figs 74.16A and B).31
Similar to ASDs, there has been some transition
in the treatment of muscular, and to some extent
perimembranous VSDs, from the traditional surgical
approach to percutaneous repair because of reported
decreased mortality rate and fewer complications compared to surgery.31 On the other hand, many medical
centers and expert pediatric cardiologists prefer not to
perform percutaneous closure of membranous VSDs
because of high incidence of heart block, damage to

1744

Section 6: Congenital Heart Disease

Figs 74.14A to E: Real time/live three-dimensional transthoracic

echocardiographic findings in coronary sinus atrial septal defect.
(A and B) Arrowhead points to unroofed coronary sinus (CS)
which resulted in left atrial (LA) to right atrial (RA) shunting; (C)
Shows opening (arrow) of CS into RA; (D) Parasternal long-axis
view. Arrowhead points to a defect in the roof of CS resulting
in communication with LA; (E) Two-dimensional transthoracic
echocardiography in the same patient. Arrowhead points to a
questionable defect in lower portion of atrial septum. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle). (Movie clip 74.14).
Source: Reproduced with permission from Singh A, Nanda NC,
Romp RL, Kirklin JK. Assessment of surgically unroofed coronary
sinus by real time/live time three-dimensional transthoracic
echocardiography. Echocardiography. 2007;24:746.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1745

Figs 74.15A and B: (A) The arrowhead points to a perimembraneous ventricular septal (VS) defect viewed en face; (B) Shows color
Doppler flow signals in the defect. (LA: Left atrium; LVOT: Left ventricular outflow tract; MV: Mitral valve; RV: Right ventricle). [Movie clip
74.15, (Parts 1 to 4)].
Source: Reproduced with permission from Mehmood F, Miller AP, Nanda NC, et al. Usefulness of live/three-dimensional transthoracic
echocardiography in the characterization of ventricular septal defects in adults. Echocardiography. 2006;23:4217.

Figs 74.16A and B: (A and B) Ventriuclar septal defect. The arrow in A points to a large defect in the trabecular ventricular system
visualized in the apical four-chamber view. The arrow in B shows the same defect viewed en face by cropping of the three-dimensional
data set. Note the generous margins of the defect. Color Doppler examination shows flow signals moving through the defect. (Movie
clip 74.16).
Source: Reproduced with permission from Nanda NC, Hsiung MC, Miller AP, et al. Live/real time 3D echocardiography. Chichester, West
Sussex: Wiley, Blackwell, 2010.

the aortic valve, and hemolysis. With the percutaneous

procedure, obtaining precise dimensions of the defect
and visualizing nearby cardiac structures becomes
even more important. Compared to 2D TTE, 3D TTE
provides a surgeons view of the cardiac anatomy to the

interventionalist prior to the start of the procedure which

can be utilized in the preprocedural planning.30
The use of 3D TEE for comprehensive assessment of
VSDs has become more accepted because of the unique
angles and views obtained of the interventricular septum

1746

Section 6: Congenital Heart Disease

and the accurate data with regard to size and shape of

the defect. 3D TTE can be used in clinical situations
that previously relied on 3D TEE because of comparable
information provided by both and can be performed in less
invasive manner. Another advantage of 3D TTE compared
to 2D echocardiography is its ability to more accurately
depict the distance from the defect to the tricuspid valve, a
very important measurement in preventing complications
during repair of the defect.30 Identification of the exact
location of the atrioventricular valves and the extent of the
anatomical rim in relation to the VSD, plays a significant
role in determining whether a patient would benefit
more from a surgical or a percutaneous intervention
(Figs 74.17 and 74.18). Fortunately, 3D TTE is capable of
capturing such images clearly. The effectiveness of 3D TTE
in measuring maximum dimension, circumference, and
area of the VSD was validated with comparable surgical
and intraoperative findings by 3D TEE.30 By visualizing the
VSD en face with 3D TTE, an added dimension of depth is
incorporated into the surgical decision making process.32

Atrioventricular Septal Defects

Atrioventricular septal defect (AVSD) is a type of CHD
that occurs as a result of incomplete fusion of the superior
and inferior endocardial cushions. Absence of fusion
of these structures during fetal development results
in abnormal development of both the atrioventricular
septum as well as the corresponding valves.33 There are
three types of ASVDs: complete, partial, and intermediate.
Complete ASVDs are distinguished by a large AVSD
(primum ASD and inlet VSD) in combination with a single
atrioventricular valve (Fig. 74.19A to G).33 Modified Rastelli

types A, B, and C are used to categorize complete ASVDs

based on the superior bridging leaflet and its attachments
to the crest of the ventricular septum and right ventricle.34
Partial ASVDs, on the other hand, have variable defects
affecting the atrial septum, ventricular septum or both
and is characterized by the presence of separate right and
left atrioventricular valves (Fig. 74.20A to E). Recognizing
the narrow and elongated left ventricular outflow tract (the
so-called goose neck deformity seen on the angiogram)
and the lack of wedging of the aorta between the mitral
and tricuspid valves is diagnostic in patients with ASVD.
These findings are presumed to be secondary to the
displacement of the left ventricular outflow tract and aortic
valve in the anterior and superior direction.33,34
Investigation of 3D TTE in patients with ASVDs noted
obvious advantages over 2D TTE in measuring the size
and magnitude of the defect and valves, as well as nearby
cardiac structures.33 The ability of 3D TTE to provide an
en face view of a complete ASVD and furthermore a clear
delineation of the characteristic five leaflets in complete
defects, sets this modality apart from the capabilities
of 2D TTE (Figs 74.21A to D).34 In addition, 3D TTE can
identify the superior bridging leaflet and its attachment
which is responsible for categorizing ASVDs into modified
Rastelli types.33,34 Singh et al. described a patient with a
presumed diagnosis of Rastelli type C ASVD based on
imaging with 2D TTE, however, follow-up imaging with 3D
TTE demonstrated that the defect was actually a Rastelli
type A ASVD.33 Similarly, 2D TTE provided visualization
of a right and left atrioventricular valve annuli, suggesting
a partial ASVD, but an en face view of 3D TTE also
identified features of a complete ASVD confirming the

Fig. 74.17: Shows the relationship and the distances of pulmonary

valve and tricuspid valve from the ventricular septal defect. (PV:
Pulmonary valve; TV: Tricuspid valve; RA: Right atrium; RV: Right
ventricle).
Source: Reproduced with permission from Chen FL, Hsiung
NC, Nanda NC, Hsieh KS, Chou MC. Real time 3-dimensional
echocardiography in assessing ventricular septal defects: An
echocardiographic-surgical correlative study. Echocardiography.
2006;23:5628.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1747

Figs 74.18A to G: Perimembranous ventricular septal defect (inlet) (arrow). (A) Real time 3D echocardiography (RT3DE) volume-rendered image of the right ventricle (RV) displaying the right aspect of the ventricular septal defect. The location of the defect in relation to
the tricuspid valve (TV) is shown; (B) Surgical view of VSD from the right aspect; (C) RT3DE volume-rendered image of the left ventricle
(LV) displaying the left aspect of the VSD. The location of the defect in relation to the left ventricular outflow tract (LVOT) is shown; (D)
2DE parasternal long-axis views showed the VSD in relation to aortic valve and right ventricle; (E to G) Live three-dimensional transthoracic echocardiography in a patient with tetralogy of Fallot; (E) Note the wide aortic root (AO) and narrow pulmonary artery (PA); (F) The
AO overrides the interventricular septum (IVS); (G) Four-chamber view. The ventricular septal defect (VSD) is located at the crux. (AO:
ZAorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle);
Source: Figures 18A to D reproduced with permission from Chen FL, Hsiung NC, Nanda NC, Hsieh KS, Chou MC. Real time 3-dimensional echocardiography in assessing ventricular septal defects: An echocardiographic-surgical correlative study. Echocardiography.
2006;23:5628.
Source: Figures 18E to G reproduced with permission from Wang et al. Echocardiography. 2003;20:593604.

diagnosis of intermediate ASVD.33 3D echocardiography

can easily recognize the characteristic ASVD findings of
an elongated and narrowed left ventricular outflow tract.
Additionally, 3DE can identify the so called cleft within the
left atrioventricular valve and evaluate its length, width,
and rim size which is often a limitation of 2D TTE.
After repair of an ASVD, left atrioventricular valve
(LAVV) regurgitation is a common complication affecting
greater than 10% of patients. These findings are likely
related to postsurgical changes leading to increased valve
diameter and consequent valve insufficiency.35,36 Knowing
about this potential complication substantiates the need
for a reliable imaging modality to diagnose and monitor
progression of valvular disease.35 Live/real time 3D TTE is
preferable to 2D TEE in evaluating the LAAV after ASVD

repair because of its ability to visualize the valve and its

surrounding anatomy, in addition to the color Doppler
capabilities. 3D TTE is also able to better evaluate valve
morphology and function. 3D TEE is effective in measuring
the size, shape, and location of the ASVD which correlates
well with the actual surgical measurements.37 3D TTE can
confirm data already obtained by 2D TTE, but the above
mentioned cases indicate the advanced capabilities of 3D
TTE in diagnosing complex CHD defects which 2D TTE
often misses.

COMMON ATRIUM
Common atrium is a type of ASVD that occurs as a
result of absent atrial septal tissue and affixing of the

1748

Section 6: Congenital Heart Disease

Figs 74.19A to F

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

Figs 74.19A to G: Live/real time, three-dimensional transthoracic

echocardiography in complete atrioventricular septal defects. (A)
Arrow shows attachment of common atrioventricular valve to a
papillary muscle in the right ventricle (Rastelli type B). No attachments
were seen to the crest of the ventricular septum (arrowhead); (B) En
face view shows all five leaflets of common atrioventricular valve;
(C) En face view of the defect viewed from top and sides (arrows).
Arrowhead points to atrial septum; (D) Arrowhead points to elongated
and narrowed left ventricular outflow tract; (E) Shows absence
of wedging of aorta (AO) in relation to common atrioventricular
valve (CAV) annulus; (F) Arrowhead points to the vena contracta
of CAV regurgitation jet. Its area measured 0.1 cm2. Color Dopplerguided continuous-wave Doppler interrogation of regurgitant
jet showed a velocity time integral (VTI) of 79 cm. Regurgitant
volume was calculated as 7.9 cm3; (G) Bicuspid aortic valve (AV).
(AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA:
Left atrium; LV: Left ventricle; MI: Mural inferior leaflet; ML: Mural
lateral leaflet; PA: Pulmonary artery; PV: Pulmonary valve; RA: Right
atrium; RV: Right ventricle; RAV: Right atrioventricular valve; RVO:
Right ventricular outflow tract; SB: Superior bridging leaflet). [Movie
clips 74.19A, B, C, E, (Parts 1 to 3)].
Source: Reproduced with permission from Singh A, Romp RL, Nanda
NC, et al. Usefulness of live/real time three-dimensional transthoracic
echocardiography in the assessment of atrioventricular septal defects.
Echocardiography. 2006;23:598606.

Figs 74.20A to D

1749

1750

Section 6: Congenital Heart Disease

Figs 74.20A to E: Live/real time, three-dimensional transthoracic echocardiography in partial atrioventricular septal defects.
(A) Arrowhead points to a prominent cleft in the anterior leaflet
of the left atrioventricular valve (LAV); (B) Arrows point to two
left ventricular papillary muscles located close to each other; (C)
Arrowhead points to a widened anteroseptal commissure (cleft)
of the right atrioventricular valve (RAV); (D) Arrowhead points to an
accessory LAV orifice; (E) Arrows point to the presence of only two
scallops in the posterior leaflet of LAV. The black arrowhead points
to the anterior leaflet of LAV. (AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA: Left atrium; LV: Left ventricle; MI:
Mural inferior leaflet; ML: Mural lateral leaflet; PA: Pulmonary
artery; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle;
RAV: Right atrioventricular valve; RVO: Right ventricular outflow
tract; SB: Superior bridging leaflet). Movie clip 74.20C.
Source: Reproduced with permission from Singh A, Romp RL,
Nanda NC, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in the assessment of atrioventricular septal defects. Echocardiography. 2006;23:598606.

Figs 74.21A to D: Live/real time, three-dimensional transthoracic echocardiography (3DTTE) in atrioventricular septal defects (AVSDs).
(A) Complete AVSD. Arrowhead shows attachment of CAV to the crest of the ventricular septum (Rastelli type A). Arrow points to atrial
component of the defect; (B) Complete AVSD. Arrowhead points to an anomalous papillary muscle projecting into the left ventricular
outflow tract causing subaortic obstruction; (C and D) Intermediate AVSD; (C) En face view of CAV shows superior bridging (SB) leaflet crossing over into the RV; (D) Both the AO and the PA are seen arising from the RV consistent with double outlet right ventricle.
(AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA: Left atrium; LV: Left ventricle; MI: Mural inferior leaflet; ML: Mural
lateral leaflet; PA: Pulmonary artery; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle; RAV: Right atrioventricular valve; RVO:
Right ventricular outflow tract; SB: Superior bridging leaflet). (Movie clips 74.21B and C).
Source: Reproduced with permission from Singh A, Romp RL, Nanda NC, et al. Usefulness of live/real time three-dimensional transthoracic echocardiography in the assessment of atrioventricular septal defects. Echocardiography 2006;23:598606.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

atrioventricular valves to the interventricular septum.38

3D TEE has been used to diagnose a common atrium with
a cor triatriatum membrane and an associated cleft mitral
valve leaflet (typically seen in common atrium) (Figs
74.22A to I). 2D TEE evaluated a prominence of the LA wall,
but was unable to identify it as a cor triatriatum membrane
and misdiagnosed these findings as a partial ASVD.

AORTOPULMONARY WINDOW
An aortopulmonary window is a rare congenital anomaly
involving a communication between the ascending
aorta and the pulmonary artery. Echocardiography is the
imaging modality of choice in diagnosing aortopulmonary
window.39 The typical echocardiographic finding in
patients with an aortopulmonary window is dilation of the

left atrium and ventricle secondary to chronic left-to-right

shunting. 2D TTE has been the imaging modality of
choice; however, there are obvious limitations that should
be recognized. False positive results can occur in 2D TTE
from artifactual dropouts designating 3D TTE as a better
modality in diagnosing aortopulmonary window.40 The
ability of 3D TTE to combine different data sets from various
angles offers a better understanding of the defect and
enhances the confidence of the diagnosis. The en face view
of the defect offered by 3D TTE also allows for more accurate
measurement of its dimensions (Figs 74.23 and 74.24).40

PATENT DUCTUS ARTERIOSUS (PDA)

The ductus arteriosus is a normal connection during fetal
life between the pulmonary artery and the descending

Figs 74.22A to D

1751

1752

Section 6: Congenital Heart Disease

Figs 74.22A to I: Three-dimensional transesophageal echocardiographic examination in cor triatriatum with common atrium.
(A and B) The arrow points to the cor triatriatum membrane while
the arrowheads (black) outline a large nonobstructive opening
present within the membrane; (C) The common atrium (CA) with
no atrial septum and the cor triatriatum membrane are well seen;
(D to H) Show left and right upper pulmonary veins (LPV and RPV)
located superior to the cor triatriatum membrane (arrows). 1 and
2 represent jets of right and left atrioventricular valve (RAV and
LAV) regurgitation, respectively. Portions of both jets appear to
move into the common atrium through the large opening in the cor
triatriatum membrane. Short-axis view of left atrioventricular valve
showing a cleft (C) in the anterior leaflet. (AV: Aortic valve; LVO:
Left ventricular outflow; PL: Posterior left atrioventricular valve
leaflet. RV: Right ventricle; LV: Left ventricle).
Source: Reproduced with permission from Baweja G, Nanda NC,
Kirklin JK. Definitive diagnosis of cor triatriatum with common
atrium by three-dimensional transesophageal echocardiography
in an adult. Echocardiography. 2004;21:3036.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1753

Figs 74.23A to F: Live/real time, three-dimensional transthoracic echocardiographic assessment of aortopulmonary window.
(A) Aortic short-axis view showing no evidence of an aortopulmonary window at this level. Movie clip 74.23, Part 2. The arrow points to
the aortopulmonary window; (B) When the three-dimensional dataset was cropped posteroanteriorly, a large communication between
the aorta (AO) and pulmonary artery (PA) was revealed (arrowhead); (C to E) The aortopulmonary window (arrowhead) could be viewed
en face by cropping the 3D dataset from the side (C) and rotating it (D and E); (F) Color Doppler exam shows mild pulmonic regurgitation
(PR). The arrowhead points to the aortopulmonary window. (AV: Aortic valve; LA: Left atrium; LPA: Left pulmonary artery; RPA: Right
pulmonary artery). [Movie clip 74.23, (Parts 1 and 2)].
Source: Reproduced with permission from Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:969.

1754

Section 6: Congenital Heart Disease

Figs 74.24A to D: Live/real time, three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. (A and B)
Arrowhead points to the aortopulmonary window. Movie clip 74.24, Parts 1 to 2. The arrow points to the aortopulmonary window. The
asterisk in Movie clip 74.24, Part 3 denotes the descending thoracic aorta; (C) Arrow points to the interrupted aortic arch. Arrowhead
points to the aortopulmonary window; (D) Arrow points to a patent foramen ovale. (BCT: Brachiocephalic trunk; LCC: Left common
carotid artery; LSA: Left subclavian artery; MV: Mitral valve; RA: Right atrium; TV: Tricuspid valve). [Movie clip 74.24, (Parts 1 to 3)].
Source: Reproduced with permission from Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:969.

thoracic aorta (Figs 74.25 to 74.28).41 Shortly after birth,

the ductus arteriosus is expected to close, but in some
patients it remains open and can persist into adulthood.
Patent ductus arteriosus can be diagnosed by a variety of
imaging modalities including 2D echocardiography (TTE,
TEE), CT, and MRI. A study by Sinha et al. in 2004 sought
to understand the usefulness of 3D TTE in the evaluation
of PDA.42 2D TTE can diagnose a PDA, however, the
assessment of the defect is often limited without the use
of 3D TTE as well. 3D TTE can provide a realistic image
of the PDA including its location, shape, length and width
as well as en face visualization of its connections with the

pulmonary artery and descending thoracic aorta.42 3D TTE

can determine the type of PDA which is useful in selecting
optimal candidates for percutaneous intervention, the
optimal anatomical approach for the procedure, and any
expected risks or complications.

CONOTRUNCAL ANOMALIES
Transposition of the Great Arteries
Transposition of the great arteries (TGA) is organized
into two distinct types: dextro (D-TGA) and levo (L-TGA;

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1755

Figs 74.25A to C: Two-dimensional transthoracic echocardiography in an adult with patent ductus arteriosus. (A and B) Color
Doppler examination demonstrates flow signals (arrowhead)
moving between the main pulmonary artery (PA) and the descending
thoracic aorta (DA) indicative of patent ductus arteriosus; (C)
Color Doppler-guided continuous-wave Doppler examination
demonstrates flow signals moving from PA to DA in systole and
back into PA in diastole (arrowheads). The arrow points to the
continuous-wave Doppler cursor line. (AO: Aorta; DA: Descending
aorta; PA: Pulmonary artery; RPA: Right pulmonary artery).
Source: Reproduced with permission from Sinha A, Nanda
NC, Khanna D, et al. Live three-dimensional transthoracic
echocardiographic delineation of patent ductus arteriosus. Echocardiography. 2004;21:4438.

A
Figs 74.26A and B

1756

Section 6: Congenital Heart Disease

Figs 74.26A to C: Live three-dimensional transthoracic echocardiography in an adult with patent ductus arteriosus. B mode images.
(A) The pyramidal section has been cropped to show the full extent
of the PDA (arrowhead) which connects the PA to DA. The arrow
points to the left atrial appendage; (B and C) The pyramidal section
has been cropped from the top to show the opening of the PDA
(arrowhead) into the pulmonary artery and its close location to the
origin of the left pulmonary artery (arrow). (LPA: Left pulmonary
artery; LV: Left ventricle; PV: Pulmonary valve).
Source: Reproduced with permission from Sinha A, Nanda NC,
Khanna D, et al. Live three-dimensional transthoracic echocardiographic delineation of patent ductus arteriosus. Echocardiography. 2004;21:4438.

Figs 74.27A to D

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1757

F
Figs 74.27A to G: Live three-dimensional transthoracic echocardiography in an adult with patent ductus arteriosus (PDA). Color Doppler
images. The pyramidal section has been cropped to visualize flow
signals in PA, PDA (arrowhead), and DA. (A) Shows the length of the
PDA (arrowhead) connecting the PA and DA. The arrow points to an
intercostal artery arising from DA; (B and C) Color Doppler images have
been isolated by completely suppressing B mode images. The isolated
color Doppler images could be rotated from 0 to 176, thus enabling
comprehensive visualization of flows in PA, PDA (arrowhead), and DA in
three-dimensions. Images at 0 (top left), 45 (top right), 90 (lower left),
and 176 (lower right) rotation are shown; (D) Frontal sections showing
color Doppler signals moving from PA into DA in systole (left) and back
into PA in diastole (right); (E and F) Tilted (top half) and enface (lower half)
views of PDA (arrowheads) at aortic (E) and pulmonary (F) connections.
On the right, B mode signals have been suppressed resulting in only
flow visualization; (G) Schematic L, length of the ampulla, defined as the
distance between the mid-portion of narrowest diameter of PDA and the
mid-portion of the aortic end. This measured 0.94 cm in our patient. D,
PDA diameter at aortic insertion (ampulla diameter). This measured 1.31
cm in our patient. (DA: Descending thoracic aorta; LPA: Left pulmonary
artery; LV: Left ventricle; PV: Pulmonary valve; RV: Right ventricle).
(Movie clip 74.27).
Source: Reproduced with permission from Sinha A, Nanda NC, Khanna
D, et al. Live three-dimensional transthoracic echocardiographic
delineation of patent ductus arteriosus. Echocardiography. 2004;21:
4438.

Figs 74.29 to 74.37). D-TGA is characterized by ventriculoarterial discordance whereby the right ventricle
pumps blood to the aorta and the left ventricle pumps
blood to the pulmonary artery.43 A shunt lesion (ASD,
VSD, or PDA) between the left and right side of the
heart must be present to ensure appropriate mixing of
pulmonary and systemic return to enable perfusion of
oxygenated blood to the bodys vital organs. In an atrial
switch surgery, deoxygenated blood is diverted from the
inferior and superior vena cavae to the left side of the heart
and from there into the pulmonary artery through the
creation of an intra-atrial baffle.44 Senning and Mustard
atrial switch procedures were historically the standard of

care for repair of TGA, however, long-term follow-up of

these patients revealed significant morbidity associated
with postprocedural complications. These complications
include baffle leaks, tricuspid valve regurgitation,
obstruction, arrhythmias, systolic dysfunction and even
sudden death.45 The current recommended surgical
approach is an arterial switch operation, which involves
rearranging the aorta and pulmonary artery to the
appropriate anatomical ventricle.46
L-TGA (or congenitally corrected TGA) is characterized
most often by situs solitus with atrioventricular and
ventriculoarterial discordance. In these patients, the
right atrium connects to the left ventricle which pumps

1758

Section 6: Congenital Heart Disease

Fig. 74.28: Thoracic magnetic resonance angiogram with Gadolinium in the same patient as Figures 25 to 27. The arrowhead
points to patent ductus arteriosus (PDA) viewed in the left lateral
position. (DA: Descending thoracic aorta; PA: Pulmonary artery;
RV: Right ventricle).
Source: Reproduced with permission from Sinha A, Nanda
NC, Khanna D, et al. Live three-dimensional transthoracic
echocardiographic delineation of patent ductus arteriosus.
Echocardiography. 2004;21:4438.

Figs 74.29A and B: Dextro-transposition of the great arteries. (A) The aorta (AO) arises from the right ventricle (RV); (B) The pulmonary
artery (PA) originates from the left ventricle (LV). The arrows point to left and right branches of the main PA. (Movie clips 74.29A and B).
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095104.

deoxygenated blood to the pulmonary artery. The left

atrium connects to the right ventricle which pumps
oxygenated blood to the aorta.44 Patients with L-TGA often
remain undiagnosed into adulthood unless they have
coexisting congenital defects which prompt corrective
repair in childhood. Signs and symptoms of right
ventricular heart failure can develop in adulthood due to
chronic pumping of the right ventricle against the higher
systemic pressure often prompting further workup and
eventual surgical repair.43

2D TTE is used to diagnose and monitor progression

of disease in L-TGA and to assess for complications
after corrective surgery of D-TGA, but 3D TTE has been
shown to have valuable advantages over 2D imaging.44 A
comprehensive view of the aortic and pulmonary valves
with corresponding valve function is more easily obtained
by 3D TTE compared to 2D TTE. Specifically, the tricuspid
valve is most often affected in TGA and 3D TTE with its
en face view and unique angles is capable of identifying
each of three leaflets of the valve. The posterior or inferior

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1759

Figs 74.30A and B: Levo-transposition (corrected transposition) of the great arteries. (A) The arrows point to two vena contractas of
tricuspid regurgitation (TR) jets; (B) En face view of the two TR vena contractas (arrows). The movie clips show cropping of the tricuspid
regurgitation jet (arrow) to view the vena contracta (arrowhead) en face in another patient with levo-transposition (corrected transposition) of the great vessels. (RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve). [Movie clip 74.30, (Parts 1 to 3)].
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095104.

Figs 74.31A to C: Dextro-transposition of the great arteries. In (A)

the aortic valve (AV) and aorta are located anterior and to the right
of the pulmonary valve (PV) and pulmonary artery. All four cardiac
valves are visualized; In (B) the AV and aorta are located directly
anterior to the PV and pulmonary artery. The arrow points to the
intra-atrial baffle; C shows normal relationship of the semilunar
valves and the great arteries in a patient without transposition of
the great arteries. The PV and the pulmonary artery are located
anterior and to the left of the aorta and the AV. All four cardiac
valves are visualized. (MV: Mitral valve; TV: Tricuspid valve).
(Movie clips 74.31A to C).
Source: Reproduced with permission from Enar S, Singh P,
Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries
in the adult. Echocardiography. 2009;26:1095104.

1760

Section 6: Congenital Heart Disease

Fig. 74.32: Dextro-transposition of the great arteries. Multiple

anatomic defects are present in the tricuspid valve (TV) which is
viewed en face in the closed position in systole.
Source: Reproduced with permission from Enar S, Singh P,
Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries
in the adult. Echocardiography. 2009;26:1095104.

Figs 74.33A to C: Dextro-transposition of the great arteries.

(A and B) The arrow points to a cleft in the anterior mitral valve (MV)
leaflet; (C) The arrow points to a narrow left ventricular outflow
tract. Systolic anterior movement of the anterior mitral leaflet (AML)
is also seen. The data set was cropped from the top. (RV: Right
ventricle; VS: Ventricular septum).
Source: Reproduced with permission from Enar S, Singh P,
Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries
in the adult. Echocardiography. 2009;26:1095104.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1761

Figs 74.34A to D: Dextro-transposition of the great arteries. (A and B) The intra-atrial baffle (B) appears as a shelf when viewed en
face by cropping from the bottom. A defect in the baffle is not well seen in the illustration but it is clearly visualized (arrowhead) in the
accompanying movie clips which view the baffle en face; (C) Shows the relationship of the baffle to the inferior vena cava (IVC); (D)
Color Doppler study shows systemic venous flow signals (arrow) moving through the left ventricle (LV) toward the pulmonary artery (PA).
(SVA: Systemic venous atrium). [Movie clip 74.34, (Parts 1 and 2)].
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095104.

tricuspid leaflet is frequently visualized poorly by 2D TTE

requiring assistance from 3D imaging. With regard to
function, 3D TTE reveals deficiencies in leaflet coaptation,
prolapse of leaflet components, and provides specific
details on the defect within the valve itself. Over time, the
severity of valvular regurgitation should be monitored
and progressively worsening function can be evaluated by
3D TTE by visualizing the shape and measuring the area
of the vena contracta, a function lacking in 2D TTE. The
vena contracta basically relates to the hole in a valve
through which regurgitation occurs and hence accurate

measurement of its size is important in determining

regurgitation severity. The product of the area of vena
contracta assessed by 3D TTE and the velocity time
integral of the regurgitation jet obtained by continuouswave Doppler provides an accurate estimate of the volume
of regurgitation.44 In addition to the tricuspid valve, 3D
TTE is unique in offering multiple views of the pulmonic
valve and can detect systolic anterior motion of the
mitral valve and left ventricular outflow tract obstruction
(affecting up to one-third of patients with TGA who have
undergone an atrial switch operation) with significant

1762

Section 6: Congenital Heart Disease

Fig. 74.35: Dextro-transposition of the great arteries. The arrow

points to a shelf-like intra-atrial baffle located behind the pulmonary
artery (PA) in another patient. Note the anterior and leftward position
of the aortic valve and aorta (AO) in relation to the pulmonary valve
and pulmonary artery (PA). (TV: Tricuspid valve).
Source: Reproduced with permission from Enar S, Singh P,
Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great
arteries in the adult. Echocardiography. 2009;26:1095104.

Figs 74.36A to C: Dextro-transposition of the great arteries.

(A) Color Doppler examination. The arrow points to a defect
in the intra-atrial baffle (B); (B) En face view of the leak (arrow)
at the origin (vena contracta); (C) Arrow points to the defect in
the baffle seen after suppression of color Doppler flow signals.
(PVA: Pulmonary venous atrium; SVA: Systemic venous atrium).
(Movie clip 74.36).
Source: Reproduced with permission from Enar S, Singh P,
Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great
arteries in the adult. Echocardiography. 2009;26:1095104.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1763

difficulty in performing such a comprehensive evaluation.36

3D TTE can supplement 2D TTE with precise measurement
of the size and shape of a VSD. Based on the usefulness
of 3D TTE in monitoring pulmonary regurgitation and its
severity, cardiologists can recognize high risk patients and
monitor progression of disease closely.

Coronary Artery Anomalies

Fig. 74.37: Dextro-transposition of the great arteries. Arrow

points to a leak in the intra-atrial baffle (B) in another patient. (IVS:
interventricular septum).
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in
the adult. Echocardiography. 2009;26:1095104.

anatomical detail.44 Follow-up of patients who have had an

atrial switch surgery relies heavily on the ability of 3D TTE
to evaluate for postprocedure complications such as baffle
leaks (up to 40% of patients after Mustard procedure) or
obstruction, functions that are often limited by 2D TTE.44,47
Ahmed et al. described an intra-atrial baffle obstruction
years after surgical repair of a TGA diagnosed by 3D TTE
based on its ability to view the defect en face in the short
axis.

Tetralogy of Fallot
ToF is a complex cyanotic CHD defect that includes VSD,
overriding aorta, pulmonary outflow tract obstruction and
right ventricular hypertrophy. Surgical repair is necessary
early in life and these patients require close followup to monitor for possible complications. Postsurgical
ToF patients can experience severe pulmonary valve
regurgitation or right ventricular outflow obstruction.
Very rarely, patients may have residual VSDs.48 3D TTE
has demonstrated its superiority compared to 2D imaging
in assessing pulmonary regurgitation severity based on
visualization of the vena contracta.49 Accurate assessment
of the size and shape of residual defects is crucial for
determining if repeat surgery is necessary and 2D TTE,
especially in the setting of other congenital anomalies, has

Anomalous coronary artery (ACA) is not an uncommon

diagnosis and may be underestimated due to the fact that
only 20% of patients with this structural abnormality will
present with symptoms.50 Common presentations include
myocardial infarction, arrhythmias, angina, syncope, and
even sudden death.50,51 Complications from ACA are more
likely to occur when coronary arteries are anatomically
located between the aorta and the pulmonary arteries.
Obstruction of blood flow likely occurs secondary to
compression between the great vessels or acute angulations in the coronary arteries.51
Coronary angiography remains the gold standard for
diagnosing anomalous coronary arteries, however, less
invasive imaging with echocardiography can provide useful
information that angiography often misses.50 Multiplane
2D TEE and 3D reconstruction can often provide similar
findings in ACA, but the 3D images offer supplemental
data used to increase the confidence of the diagnosis.51 3D
TTE has previously been used to assess proximal coronary
arteries; however, Vengala et al. in 2003 demonstrated
additional capabilities of 3D TTE in evaluation of coronary
anatomy.52 ACAs first detected by 3D TTE were later
confirmed with angiography, validating its use. The en
face view and unique maneuvering in multiple geometric
planes offered by live/real time 3D TTE depicts the origin
and course of the coronary artery. Coronary angiography
often has difficulty in visualizing the anomalous vessel
between the aorta and the pulmonary artery, which as
described above carries a greater risk of complications.51
Both 2D and 3DE can diagnose an anomalous origin
of the left coronary artery from the pulmonary artery,
but 3D TTE can also visualize the opening of the ACA
en face and its connections to the aorta and pulmonary
artery (Figs 74.38 and 74.39).53 This presentation requires
immediate surgical correction that previously consisted
of creating a tunnel between the aorta and the pulmonary
artery reversing blood flow into the aorta. 3D TTE was
capable of tracking the tunnel between the aorta and

1764

Section 6: Congenital Heart Disease

D
Figs 74.38A to E: Two-dimensional transesophageal and transthoracic echocardiography in an adult with anomalous origin of the left
coronary artery from the pulmonary artery. (A and B) Intraoperative
transesophageal study shows a dilated right coronary artery (RCA, A)
and the surgically created tunnel (T, B); (C to E) Transthoracic study
(performed 13 years after surgery). The arrow in C shows a small
defect in the tunnel (T) near the aorta through which shunting occurs
into the pulmonary artery (arrowhead in D). Color Doppler-guided
continuous-wave Doppler examination (E) shows shunting occurring
practically throughout the cardiac cycle (arrowheads). The arrow in
E points to the Doppler cursor line. (AO: Aorta; LA: Left atrium; LPA:
Left pulmonary artery; PR: Pulmonary regurgitation; PV: Pulmonary
valve; RA: Right atrium; RPA: Right pulmonary artery; RVOT: Right
ventricular outflow tract). Source: Reproduced with permission from
Ilgenli TF, Nanda NC, Sinha A, Khanna D. Live three-dimensional
transthoracic echocardiographic demonstration of anomalous origin
of left coronary artery from the pulmonary artery. Echocardiography.
2004;21:55962.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

Figs 74.39A to F

1765

1766

Section 6: Congenital Heart Disease

pulmonary artery and Doppler functions were used to

confirm the tunnel-pulmonary artery shunting.53 Based
on the 3D views, the area of the defect was measured and
shunt volume into the pulmonary artery was calculated.
Nowadays, surgeons just reimplant the coronary artery
between the pulmonary artery and the aorta. Diagnosis of
the left main coronary artery fistula to the left ventricle has
also been made by 3D TTE (Figs 74.40A and B).54

OUTFLOW TRACT OBSTRUCTION

Congenital Aortic Stenosis/Bicuspid
Aortic Valve
The most common cause of aortic stenosis (AS) in adults
is acquired, related to calcific degeneration of the valve,
but congenital AS, most notably from bicuspid aortic valve
(BAV), is a clinically relevant abnormality. When a patient
presents with symptoms of AS prior to the seventh decade
of life, the diagnosis of BAV should be strongly considered.
In addition to AS, aortic regurgitation (AR), infective
endocarditis, and aortic dissection have all been linked to
BAV.55
Compared to 2D TTE, 3D TTE offers better images of the
aortic valve, especially the visualization of the individual
leaflets.56 The identification of redundant aortic valve
leaflets are relevant in distinguishing the development of
AS versus AR based on the BAV closure down the midline.1
3D TTE is able to recognize redundancy of aortic valve
leaflets and offer prognostic information, a feature 2D

Figs 74.39A to G: Live three-dimensional transthoracic echocardiographic demonstration of anomalous origin of left coronary
artery from the pulmonary artery. (A) The arrowhead points to the
orifice of the anomalous coronary artery while the arrow shows
the defect in the tunnel; (B and C) Color Doppler examination.
In B, flow signals (arrowhead) are seen moving into the orifice
of the anomalous coronary artery from the tunnel (T). The arrow
points to the tunnel-pulmonary artery shunt. In C, the arrow points
to color Doppler shunt flow signals visualized in three dimensions;
(D) Coronary angiogram. Arrowhead points to fistula originating
from the left anterior descending coronary artery (LAD) and
draining into the pulmonary artery (PA) which is partially opacified.
(E to G) Live three-dimensional transthoracic echocardiography; E.
Arrowhead points to a small localized area of abnormal flow signals
in the pulmonary artery (PA) just distal to the pulmonary valve (PV)
in both diastole (F) and systole (G) consistent with entrance of
the fistula into the PA. The arrow in F points to mild pulmonary
regurgitation. (AV: aortic valve; CX: Circumflex coronary artery;
LM: Left main coronary artery; RVO: Right ventricular outflow
tract). (PA: Main pulmonary artery; AO: Aorta; LA: Left atrium;
LPA: Left pulmonary artery; PR: Pulmonary regurgitation; PV:
Pulmonary valve; RA: Right atrium; RPA: Right pulmonary artery;
RVOT: Right ventricular outflow tract). Source: Figure 74.39A to C
Reproduced with permission from Ilgenli TF, Nanda NC, Sinha A,
Khanna D. Live three-dimensional transthoracic echocardiographic
demonstration of anomalous origin of left coronary artery from the
pulmonary artery. Echocardiography. 2004;21:559562. Source:
Figure 74.39D Reproduced with permission from Mehta D, Nanda
NC, Vengala S, Mehmood F, Taylor J. Live three dimensional
transthoracic echocardiographic demonstration of coronary artery
to pulmonary artery fistula. Am J Geriatric Cardiol. 2005;14:424.
Source: Figures 74.39E to G Reproduced with permission from
Mehta D, Nanda NC, Vengala S, Mehmood F, Taylor J. Live three
dimensional transthoracic echocardiographic demonstration of
coronary artery to pulmonary artery fistula. Am J Geriatric Cardiol.
2005;14:424.

TTE is unable to perform (Figs 74.41 to 74.43).56 Leaflet

perforation is an unfortunate complication that can result
in significant AR which has been visualized by 3DE.
Quadricuspid aortic valves, although rare, have been
diagnosed by 3D TTE. Burri et al. described a young
female patient scheduled to undergo aortic valve surgery
for AR who was misdiagnosed with BAV based on 2D
echocardiographic findings (Figs 74.44A to C).57 On further
investigation of the valve with preoperative 3D TTE and
intraoperative multiplane TEE, images were consistent
with a quadricuspid aortic valve (Figs 74.45A and B).
Understanding the capabilities of 3D TTE and analyzing
the valve carefully from each echocardiographic view can
be instrumental in making a diagnosis that was previously
missed. In this case, a quadricuspid aortic valve was
visualized in the parasternal short-axis view, but not in the
parasternal long-axis view.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1767

Figs 74.40A and B: Live/real time, three-dimensional transthoracic echocardiographic detection of left main coronary artery fistula into
the left ventricle. (A and B) The arrowhead in A points to the enlarged left main coronary artery (LM). Its entrance into the left ventricle
(LV) is denoted by an arrowhead in (B). (AO: Aorta). (Movie clips 74.40A and B).

Figs 74.41A and B: Two-dimensional transthoracic echocardiography in an adult patient with bicuspid aortic valve and severe aortic
regurgitation. (A) The bicuspid aortic valve (AV) is shown in systole in the open position with no evidence of stenosis; (B) Color Doppler
examination shows an eccentric jet of aortic regurgitation originating posteriorly (horizontal arrow). The vertical arrow points to mild
pulmonic regurgitation. (PV: Pulmonic valve; RA: Right atrium; RV: Right ventricle). (Movie clips 41A and B).
Source: Reproduced with permission from Singh P, Dutta R, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic assessment of bicuspid aortic valve morphology. Echocardiography. 26.:4:47880.

It is important to recognize that 2D echocardiography

with Doppler tends to overestimate the severity of stenotic
lesions due to the pressure recovery phenomenon and the
presence of localized high velocities (and gradients) which
may not reflect the true gradient across the stenotic valve
or subvalvar membrane. Also, 3DE is particularly useful in
characterizing adjacent stenotic lesions in tandem such as

aortic valve stenosis and supravalvar stenosis in the same

patient. This feature cannot be done in 2D Doppler which
will simply give the maximum gradient obtained from both
areas but cannot identify the severity of each individual
lesion. A major advantage of 3D over 2D echocardiography
is the ability to systematically crop the 3D data sets in a
sequential manner such that an en face view of the valve

1768

Section 6: Congenital Heart Disease

Figs 74.42A and B: Live/real time, three-dimensional transthoracic echocardiography in the same patient. (A) Note the presence of
several folds in the bicuspid aortic valve in the closed position viewed from the ventricular side; (B) The arrow points to a well circumscribed perforation in the posterior cusp of the aortic valve. (LA: Left atrium; PV: Pulmonic valve; RA: Right atrium; RV: Right ventricle).
(Movie clips 74.42A and B).
Source: Reproduced with permission from Singh P, Dutta R, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic assessment of bicuspid aortic valve morphology. Echocardiography. 26;4:47880.

Figs 74.43A and B: Two-dimensional transthoracic echocardiography. (A and B) The aortic valve (AV) appears bicuspid. (LA: Left
atrium; RV: Right ventricle). (Movie clip 74.43).
Source: Reproduced with permission from Burri MV, Nanda NC, Singh A, Panwar SR. Live/real time three-dimensional transthoracic
echocardiographic identification of quadricuspid aortic valve. Echocardiography. 2007;24:6535.

orifice is obtained at the level of the valve tip where the

stenosis is most significant. This can then be planimetered
providing an accurate estimate of the stenosis severity.
3DE is also useful in making sure that the cutting plane
in the 3D data set is exactly parallel to the plane of the
valve orifice preventing inaccuracies introduced by

oblique planes. With 2D echocardiography, it is not always

possible to be certain that one is visualizing the valve at
its tip and it is difficult to determine whether the plane is
parallel or oblique in relation to the valve orifice. This is
because, at any given time, 2D echocardiography provides
only a thin slice-like view of a cardiac structure such as the

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1769

Figs 74.44A to C: Multiplane two-dimensional transesophageal

echocardiography. (A and B) Four aortic valve (AV) leaflets
(numbered in B) are well seen. The arrow in A points to diastolic
noncoaptation of AV leaflets which resulted in significant aortic
regurgitation. C. The arrow points to severe aortic regurgitation.
(AO: Aorta, RA: Right atrium). (Movie clip 74.44A and B).
Source: Reproduced with permission from Burri MV, Nanda NC,
Singh A, Panwar SR. Live/real time three-dimensional transthoracic echocardiographic identification of quadricuspid aortic valve.
Echocardiography. 2007;24:6535.

Figs 74.45A and B: Live/real time, three-dimensional transthoracic echocardiography. (A and B) Shows a quadricuspid aortic valve
with four numbered leaflets clearly visualized. (AO: Aorta, RA: Right atrium; TV: Tricuspid valve.). [Movie clip 74.45, (Parts 1 and 2)].
Source: Reproduced with permission from Burri MV, Nanda NC, Singh A, Panwar SR. Live/real time three-dimensional transthoracic
echocardiographic identification of quadricuspid aortic valve. Echocardiography. 2007;24:6535.

1770

Section 6: Congenital Heart Disease

aortic valve. This is unlike a 3DE data set that includes the
whole valve which can then be studied at any time using
any desired plane and angulation.

Subaortic Stenosis
Stenosis can occur not only at the level of the valve but
above the valve (supravalvular) as well as below the valve
(subvalvular or subaortic). When subaortic stenosis is
diagnosed it is important to distinguish the fixed from
the dynamic type. Fixed subaortic stenosis can be due to
a focal, fibromuscular membrane or a diffuse tunnel-type
lesion. On the other hand, dynamic subaortic stenosis can
develop as a result of systolic anterior motion of the mitral
valve, a common echocardiographic finding in patients
with hypertrophic cardiomyopathy.1 Interestingly, there is
a strong association between patients previously diagnosed
with a VSD and subaortic stenosis which appears to be
related to fibrous tissue formation from turbulent blood
flow at the prior surgical site.58
With 3D TTE, the aortic valve, subaortic membrane,
and surrounding cardiac anatomy is clearly visualized
which is beneficial in measuring the severity of stenosis
(Figs 74.46 and 74.47).1,59,60 2D TTE can be helpful
in diagnosing subaortic stenosis based on Doppler
measurement of gradient across the membrane, however,
these calculations are often inaccurate. In a study by

Bandarupalli et al. the measurement of valve gradient and

orifice area by 2D TTE was indicative of severe obstructive
disease, but follow-up calculations by 3D TTE with the
subaortic membrane en face from the left ventricular
outflow tract were indicative of insignificant stenosis.60
Left heart catherization was performed to confirm the
echocardiographic findings, and in fact was consistent
with those obtained by 3D TTE (Fig. 74.48). 3D TTE is more
reliable because it evaluates the subaortic membrane
in its entirety and locates the defect in the membrane
(Figs 74.49 to 74.51).59 The defect was viewed en face by
rotation and cropping of data sets, a technology unique to
3DE. Furthermore, 3D TTE images can identify the hole
in a hole which is diagnostic of subaortic stenosis.

AORTIC ARCH ANOMALIES

Coarctation of the Aorta
The diagnosis of coarctation of the aorta is often missed
prenatally and immediately after birth manifesting as
shock, once closure of the ductus arteriosus occurs.61 Later
in life, patients with coarctation of the aorta present with
significant differences in blood pressure readings between
the upper and lower extremities. If not repaired, patients
are at risk of developing symptoms of congestive heart
failure, aortic rupture, and endocarditis.58 Percutaneous

Figs 74.46A and B: Two-dimensional transthoracic echocardiogram in discrete subaortic membranous stenosis. (A) The arrowhead
points to the subaortic membrane; (B) Using color Doppler-guided continuous-wave Doppler, a peak gradient (PG) of 134 mm Hg
(arrow) was obtained across the left ventricular (LV) outflow tract. (AO: Aorta; LA: Left atrium; RV: Right ventricle; RVO: Right ventricular
outflow tract).
Source: Reproduced with permission from Agrawal GG, Nanda NC, Htay T, Dod HS. Live three-dimensional transthoracic echocardiographic identification of discrete subaortic membranous stenosis. Echocardiography. 2003;20:6179.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1771

Figs 74.47A to C: Live three-dimensional transthoracic echocardiography in discrete subaortic membranous stenosis.
(A and B) The arrowhead points to a narrow opening in the
membrane imaged in short axis; (C) Membrane (arrowhead)
viewed from the top showing its attachment to the ventricular
septum (VS) and the anterior leaflet of the mitral valve. (AO: Aorta;
RA: Right atrium). The arrowhead in the Movie clip 74.47 points to
the obstructing membrane. (Movie clip 74.47).
Source: Reproduced with permission from Agrawal GG, Nanda
NC, Htay T, Dod HS. Live three-dimensional transthoracic echocardiographic identification of discrete subaortic membranous
stenosis. Echocardiography. 2003;20:6179.

Fig. 74.48: Discrete subaortic membrane. Cardiac catheterization

pressure tracings showing no significant gradient across the LV
outflow tract.
Source: Reproduced with permission from Bandarupalli N,
Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis
of significant obstruction by Doppler in a patient with discrete
subaortic membrane: Correct diagnosis by 3D-transthoracic
echocardiography. Echocardiography. 2008;25:10046.

1772

Section 6: Congenital Heart Disease

Figs 74.49A and B: Discrete subaortic membrane. Two-dimensional transthoracic echocardiography. (A) Arrowhead points to the
membrane imaged in the parasternal long-axis view; (B) Shows peak and mean pressure gradients of 64 mm Hg and 31 mm Hg across
the left ventricular outflow tract by continuous-wave Doppler. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). (Movie
clip 74.49).
Source: Reproduced with permission from Bandarupalli N, Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of significant
obstruction by Doppler in a patient with discrete subaortic membrane: Correct diagnosis by 3D-transthoracic echocardiography. Echocardiography. 2008;25:10046.

Figs 74.50A and B: Discrete subaortic membrane. Live/real time, three-dimensional transthoracic echocardiography. (A and B) Subaortic membrane (arrowhead) and orifice viewed en face. The orifice measured 2.29 cm2 by planimetry. (LA: Left atrium; PV: Pulmonic
valve; RA: Right atrium; RV: Right ventricle). (Movie clip 74.50).
Source: Reproduced with permission from Bandarupalli N, Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of significant
obstruction by Doppler in a patient with discrete subaortic membrane: Correct diagnosis by 3D-transthoracic echocardiography. Echocardiography. 2008;25:10046.

balloon angioplasty with or without stent placement is

becoming more popular as the treatment option for young
adult patients with coarctation of the aorta, while surgery
remains an option for those not candidates for percutaneous

approach. Evaluating a patient for coarctation of the aorta

includes 2D TTE with continuous-wave Doppler focusing
on the identification of diminishing anterograde flow during
diastole to confirm hemodynamically significant stenosis.58

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1773

Fig. 74.51: Discrete subaortic membrane. Live/real time, threedimensional transthoracic echocardiography. Aortic valve orifice
viewed en face. It measured 2.94 cm2 by planimetry. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
Source: Reproduced with permission from Bandarupalli N,
Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of
significant obstruction by Doppler in a patient with discrete
subaortic membrane: Correct diagnosis by 3D-transthoracic
echocardiography. Echocardiography. 2008;25:10046.

Fig. 74.52: Real time, two-dimensional transesophageal echocardiogram in aortic arch vasum vasi to pulmonary artery fistula. The
arrowhead points to flow signals moving from the wall of the aortic
arch (AO) into the main PA. (Movie clip 74.52).
Source: Reproduced with permission from Sadat K, Pradhan
M, Nanda NC, Joshi D, Diddi HP. Two- and three-dimensional
transthoracic echocardiography in the assessment of aortic
arch vasum vasi to pulmonary artery fistula. Echocardiography.
2013;30:21924.

2D TTE has traditionally been used to make the

diagnosis of aortic arch abnormalities, but understanding
its limitations allows for the use of more advanced
technology.62 For example, postsurgical repair of coarcation
of the aorta results in a tortuous aortic arch which makes
evaluation by 2D TTE difficult. 3D TTE can create an
echocardiographic angiogram and rotate images in
various angles and planes providing the cardiologist with
a detailed understanding of the extravascular cardiac
anatomy.63

correct diagnosis. The diagnosis was confirmed based on

calculations of flow acceleration within the aortic wall, the
pressure within the fistula itself, and the gradient between
the aorta and the pulmonary artery.64

Aortic Arch to Pulmonary Artery Fistula

Aortic lumen to pulmonary artery fistulas (APAF) is
extremely rare, and APAF forming from the vasum vasi
in the posterior wall of the aortic arch is even more rare.64
2D TEE demonstrated turbulent blood flow in the main
pulmonary artery moving from a direction not consistent
with the lumen as the site of origin. Further evaluation
with 3D imaging from various angles actually identified
the fistula beginning in the posterior wall of the aortic
arch likely from a vasum vasi (Figs 74.52 and 74.53). 3D
TTE confidently assessed the fistula at its origin with its
en face view and aided 2D TTE in confidently making the

ATRIAL AND ATRIOVENTRICULAR

VALVE ABNORMALITIES
Cor Triatriatum Sinister
Cor triatriatum sinister is a rare CHD characterized by
a fibromuscular membrane located in the left atrium
superior to the LA appendage.65 The location of the
membrane distinguishes this entity from a mitral
supravalvular membrane which is located below the LA
appendage. The size of the defect in the membrane is
indirectly proportional to the degree of obstruction of
blood flow and subsequent hemodynamic significance.
2D echocardiography has traditionally been the
imaging modality of choice for the diagnosis of cor
triatriatum sinister, however 3D imaging offers greater
precision in assessing the size and shape of the defect
within the membrane.65 3D TTE visualizes the
membrane en face leaving the cardiologist with a better

1774

Section 6: Congenital Heart Disease

Figs 74.53A and B: Live/real time, three-dimensional transthoracic echocardiogram in aortic arch vasum vasi to pulmonary artery
fistula. (A) The arrowhead points to abnormal flow signals originating within the posterior wall of the aortic arch. A small area of flow
acceleration is also noted. Careful cropping revealed no connection of this abnormal flow signals with the aortic lumen; (B) En face view of
the fistula vena contracta. It is very small measuring 1.7 mm in diameter, area 3.5 mm2. (LA: Left atrium). (Movie clips 74.53A and B).

Figs 74.54A and B: Live/real time, three-dimensional transthoracic echocardiography in cor triatriatum sinister. (A) Arrowhead points
to cor triatriatum membrane (M), which is located superior to left atrial appendage (LAA); (B) Arrowhead (arrow in movie) points to a
large opening in cor triatriatum membrane visualized en face. The dimensions were 3.06 1.03 cm and area 2.3 cm2. (LA: Left atrium;
LV: Left ventricle; LVO: Left ventricular outflow tract; RA: Right atrium; RV: Right ventricle). (Movie clip 74.54).
Source: Reproduced with permission from Patel V, Nanda NC, Arellano I, Yelamanchili P, Rajdev S, Baysan O. Cor triatriatum sinister:
Assessment by live/real time three-dimensional transthoracic echocardiography. Echocardiography. 2006;23:8012.

understanding of the defect (Figs 74.54A and B). 3D TTE

can accurately diagnose cor triatriatum sinister based on
the echocardiographic characteristics described above
and quantify the size of the defect which is valuable in
understanding its clinical significance.65 Conversely,
2D TTE was able to make the diagnosis, but could not
comment on the size and shape of the defect and thus could

not comment on the clinical relevance of the defect. In the

late 1990s, Samal et al. described offline 3D reconstruction of
2D TTE images for enhanced visualization of cor triatriatum
sinister along with other atrial membrane defects.66 With the
creation of 3D images, the exact size, shape, and location
of the membrane defect was known to the cardiologist and
surgeon which was valuable in planning for surgical repair.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1775

B
Figs 74.55A to C: Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve. The
arrowhead points to the cleft in the anterior mitral valve leaflet
seen in the open (A and B) and closed (C) positions. (LV: Left
ventricle; PML: Posterior mitral leaflet; RV: Right ventricle). The
cleft is directed toward the left ventricular outflow tract unlike
the atrioventricular septal defect where the cleft points medially.
Movie clip 74.55, Parts 1 and 2 from another patient shows an
isolated cleft (arrow) in the anterior mitral leaflet in a 28-year-old
female. Color Doppler examination shows prominent regurgitation
(arrowhead) into the left atrium through the cleft. [Movie clip 74.55,
(Parts 1 and 2)].
Source: Reproduced with permission from Sinha A, Kasliwal
RR, Nanda NC, et al. Live three-dimensional transthoracic
echocardiographic assessment of isolated cleft mitral valve.
Echocardiography. 2004;21:65761.

Isolated Mitral Valve Cleft

Ebsteins Anomaly

Isolated mitral valve cleft is a rare congenital defect that

may cause mitral regurgitation. The direction the cleft
faces helps to distinguish an AVSD (toward the ventricular
inlet septum) from isolated mitral valve cleft (toward the
left ventricular outflow tract), a feature easily performed
by 3D TTE.67,68 The diagnosis of isolated mitral valve
cleft is often missed by 2D TTE making 3D TTE a more
enticing imaging modality. In patients with mitral valve
prolapse, the diagnosis of isolated mitral valve cleft by
2D TTE is especially difficult because of the redundant
leaflets. Compared to 2D TTE, 3D TTE visualizes the
cleft in the anterior mitral valve leaflet from multiple
viewpoints ensuring greater confidence in the diagnosis.
In addition, it provides accurate dimensions of the defect
and the mitral rim and vena contracta measurements give
reliable assessment of the severity of the resultant mitral
regurgitation (Figs 74.55 and 74.56).67

Ebsteins anomaly is a rare congenital defect affecting

less than 1% of patients with CHD that is characterized by
the attachment of tricuspid valve leaflets, often the septal
and posterior leaflets, to the walls of the right ventricle
and ventricular septum.69 The tethering of the septal
leaflet to the ventricular septal wall causes an apparent
displacement of these structures in the direction of the
right ventricular apex that is characteristically seen on
2D TTE. Despite these findings, 2D TTE has difficulty
identifying other important morphological features of
Ebsteins anomaly.
Due to the limitations of the 2D TTE, 3D TTE can
be used in patients with Ebsteins anomaly because it
can visualize each individual leaflet and the tethering
to the right ventricular free wall or ventricular septum
(Fig. 74.57).70 Based on the comprehensive view of the
leaflets offered by 3D TTE, the size and location of the

1776

Section 6: Congenital Heart Disease

Figs 74.56A to D: Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve. (A to C) The
arrowhead points to the cleft in the anterior mitral valve leaflet. Note thickened mitral leaflets with a narrow orifice indicative of associated
mitral stenosis; (D) Isolated cleft mitral valve in another child. This en-face transthoracic three-dimensional view shows the cleft (arrow)
dividing the superior and inferior components of the anterior mitral leaflet. Note the perception of the depth of the cleft with threedimensional imaging. (MV: Mitral valve; LV: Left ventricle; PML: Posterior mitral leaflet; RV: Right ventricle). (Movie clip 74.56D).
Source: Figures 74.56A to C reproduced with permission from Sinha A, Kasliwal RR, Nanda NC, et al. Live three-dimensional
transthoracic echocardiographic assessment of isolated cleft mitral valve. Echocardiography. 2004;21:65761.

tethered and nontethered parts of the leaflets can be better

evaluated, which is relevant when the patient is evaluated
for tricuspid valve repair. A surgeons understanding of the
amount of nontethered leaflet tissue is crucial in selecting
appropriate candidates for valve repair, a function that
3DE has nearly mastered. The nontethered leaflets of the
tricuspid valve are known to extend outward creating a
bubble-like appearance on 3D imaging that is diagnostic of
Ebsteins anomaly (Figs 74.58 and 74.59).70 It is important
to recognize that not all tethered areas can be viewed
en face by 3D TTE, one obvious limitation preventing a
complete understanding of the severity of this anomaly.

OTHER ABNORMALITIES
Chiari Network
Chiari network is often clinically insignificant, but some
reports suggest increased risk of thrombus formation,
paradoxical embolization, or endocarditis.71 Chiari
network is composed of a large fenestrated membrane
in the right atrium with wide attachments at the crista
terminalis and interatrial septum as a result of persistence
of the right-sided sinus venosus valve. 3D TTE is valuable
in distinguishing Chiari network from Eustachian and
Thebesian valves based on their smaller size and location,

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

A
Figs 74.58A and B

1777

B
Figs 74.57A to C: Live/real time, three-dimensional transthoracic
echocardiography in Ebsteins anomaly associated with transposition of the great vessels. (A) Four-chamber view shows apparent
displacement of the attachment of the septal leaflet of the tricuspid
valve (TV) toward the apex; (B) Tethering of the septal leaflet of the
TV results in a bubble-like appearance (yellow arrowhead) in the
middle portion of the ventricular septum as the nontethered portion
moves toward closure during systole. This transverse section
was taken at a level denoted by #1 in (A); (C) Transverse section
taken at a more inferior level (#2 in A) demonstrates bubble-like
appearance of both septal (yellow arrowhead) and posterior (black
arrowheads) TV leaflets produced by tethering. (a: Anterior TV
leaflet; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA:
Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Patel V, Nanda NC,
Rajdev S, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of Ebsteins Anomaly. Echocardiography. 2005;22:84754.

1778

Section 6: Congenital Heart Disease

Figs 74.58C to H

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

I
and cor triatriatum dexter network based on its thickness
with few or absent fenestrations (Figs 74.60 and 74.61).
Other clinical clues suggesting the diagnosis of cor
triatriatum dexter are obstruction of blood flow and its
frequent association with other congenital defects. 2D TTE
has diagnosed a defect as a Eustachian valve but followup imaging with 3D TTE confirmed the diagnosis of Chiari
network.71

DOUBLE OUTLET RIGHT VENTRICLE

Double outlet right ventricle is a congenital anomaly
whereby the aorta and main pulmonary artery are
connected (entirely or partly) to the morphologic
right ventricle. The presence of a VSD almost always
accompanies this diagnosis (Fig. 74.62). Some experts
consider double outlet right ventricle on a spectrum with
other CHDs including ToF in the presence of a VSD and/or
pulmonary stenosis as well as TGA.72 The recommended
surgical repair includes creation of a baffle that connects the
aorta to the left ventricle through the VSD. Other surgical
options include arterial switch procedures. In planning for
the proper repair procedure, a comprehensive evaluation
of the location and size of the VSD and its proximity to the
semilunar valves as well as the relationship of the aorta
and main pulmonary artery to each other is essential.72,73
Surgeons have long relied on 2D echocardiography,
however, 3DE has become a feasible imaging option and
offers significant advantage in the visualization of cardiac
anatomy and in obtaining the necessary measurements
related to double outlet right ventricle. Specifically,
3D imaging can crop images to better visualize the
atrioventricular valves, the VSD, and the great vessels in
a single echocardiographic window. This information

1779

Figs 74.58A to I: Live/real time, three-dimensional transthoracic

echocardiography in isolated Ebsteins anomaly. (A) Transverse
section taken at the apex of TV shows a large area of noncoaptation (N) as well as tethering and bubble-like appearance of anterior (yellow arrows) and posterior (black arrowhead) TV leaflets;
(B to D) Transverse sections taken more basally demonstrate
multiple bubbles in the septal (yellow arrowheads) and posterior
(black arrowheads) TV leaflets. Inset in D shows all three leaflets of the tricuspid valve in the open position; (E) Oblique section shows multiple bubbles (black arrowheads) in the posterior
(p) TV leaflet produced by tethering to RV inferior wall. Inset in E
shows a long snake-like posterior (p) TV leaflet; (F) The oblique
section shown in E has been rotated to more optimally view
the attachment of posterior (p) TV leaflet to the RV inferior wall;
(G) The arrowhead in another patient with Ebsteins anomaly
shows a bubble-like appearance resulting from tethering of the
septal leaflet (s) of the tricuspid valve to the ventricular septum; (H
and I) The arrowhead points to a large defect in the anterior leaflet
of the tricuspid valve in a different patient with Ebsteins anomaly.
Note also small, discrete nodular areas of thickening on the anterior tricuspid leaflet. Asterisks represent loss of tricuspid valve
tissue which is considerable in this patient. The septal leaflet of the
tricuspid valve was tethered to the ventricular septum. (a: Anterior
tricuspid valve leaflet; AV: Aortic valve; LV: Left ventricle; RV: Right
ventricle; P: Posterior tricuspid valve leaflet). [Movie clips 74.58B,
58D, 58D (inset), 58E (inset), and 58F, 58G, 58H].
Source: Figure 58A to F reproduced with permission from Patel
V, Nanda NC, Rajdev S, et al. Live/real time three-dimensional
transthoracic echocardiographic assessment of Ebsteins Anomaly.
Echocardiography. 2005;22:84754.
Source: Figure 58G to I Reproduced with permission from Pothineni K,
et al. Live/real time three-dimensional transthoracic echocardiographic
assessment of tricuspid valve pathology: incremental value over
the two-dimensional technique. Echocardiography. 2007;24:
54152.

improves understanding of the defect and helps members

of the medical team to plan for surgical repair.73 3D imaging
can predict if a repaired baffle between the left ventricle
and aorta will eventually cause tricuspid valve obstruction
or right ventricular outflow obstruction. Furthermore,
3D observation from the right ventricle elucidates the
proximity of the VSD to the aorta, pulmonary artery,
and tricuspid valve preparing the surgeon for potential
right ventricular outflow tract obstruction after a baffle
procedure and the need for a right ventricle to pulmonary
artery conduit.

Left Ventricular-RA Communication

Left ventricular-RA communication is often congenital
in nature, however this defect can also be acquired
secondary to endocarditis, mitral or aortic valve
replacement, chest trauma, or myocardial infarction.74
Hansalia et al. described a patient with acquired left
ventricular-RA communication related to a previous
aortic valve replacement that was erroneously diagnosed

1780

Section 6: Congenital Heart Disease

Figs 74.59A to E: Isolated Ebsteins anomaly. (A to C) Shows

tethering of all three TV leaflets. The resulting bubbles are
denoted by arrowheads for the septal (yellow) and posterior (black)
TV leaflets and an arrow for the anterior (a) TV leaflet; (D) A threedimensional view from a child showing the large atrialized portion of
the right ventricle (RV); (E) Short-axis three-dimensional view from
the same child shows a large region of noncoaptation (arrow) of
the tricuspid valve when viewed en-face from RV apex. (A: Anterior
tricuspid valve leaflet; LA: Left atrium; LV: Left ventricle; RA: Right
atrium; S: Septal leaflet of tricuspid valve. AV: Aortic valve; LV: Left
ventricle; RV: Right ventricle; P: Posterior tricuspid valve leaflet).
(Movie clips 74.59D and E).
Source: Reproduced with permission from Patel V, Nanda NC,
Rajdev S, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of Ebsteins Anomaly. Echocardiography. 2005;22:84754.

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1781

Fig. 74.60: Real time, two-dimensional transesophageal echocardiography. Arrowhead points to a linear structure in the right
atrium (RA) consistent with a Eustachian valve. Movie clip 74.60,
Part 2, arrow shows the tumor. [Movie clip 74.60, (Parts 1 and 2)].
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Singh A, Panwar SR, Gandhari S. Live/real time
three-dimensional transthoracic echocardiographic visualization of
Chiari Network. Echocardiography. 2007;24:9957.

Figs 74.61A to C: Live/real time, three-dimensional transthoracic

echocardiography. (A and B) Chiari network. Small arrowheads in
A point to some of the multiple openings in the large membrane,
outlined by red dots. Attachment of the Chiari membrane
(arrowhead) to the interatrial septum (*) is shown in (B). Movie
clip 74.61A arrowhead points to the Chiari membrane; (C) Renal
cell carcinoma. Arrow points to the tumor in the inferior vena cava
(IVC) at the right atrial (RA) junction. (L: Liver; LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; TV: Tricuspid valve). (Movie clips
74.61A and B).
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Singh A, Panwar SR, Gandhari S. Live/real time
three-dimensional transthoracic echocardiographic visualization of
Chiari Network. Echocardiography. 2007;24:9957.

1782

Section 6: Congenital Heart Disease

as pulmonary hypertension by 2D TTE.74 The diagnosis

was likely missed by 2D TTE because of the limited
views it offered, preventing complete visualization of the
defect. The diagnosis in this case was also complicated by
simultaneous severe tricuspid regurgitation. Follow-up
imaging with 3D and its volumetric data demonstrated the
defect and shunting of blood from the left ventricle to the
right atrium.

Right Coronary Artery Fistula

A coronary artery fistula is a rare congenital abnormality
that empties blood into one of the cardiac chambers or
into blood vessels through one or multiple fenestrations.75
These fistulas can also occur secondary to coronary

artery trauma. While coronary angiography has been

the recommended imaging modality in the diagnosis
of coronary artery fistula, it is limited in its ability to
delineate intricate coronary anatomy and its proximity
to other relevant structures. 2D TEE has been shown to
demonstrate a fistula between the right atrium and right
coronary artery with the use of color Doppler, however, it
has difficulty visualizing the fistula tract in its entirety and
therefore, often miscalculates its dimensions. On the other
hand, 3D TEE is capable of obtaining an en face view of
the right coronary artery to right atrium fistula and has
been able to track the fistula along its course (Fig. 74.63).
Importantly, 3D TEE was able to clearly identify the origin
and termination sites of the fistula and therefore make
accurate measurements, which can be used to plan for
treatment.75

Fig. 74.62: Double outlet right ventricle (DORV). This subcostal

three-dimensional 3 D acquisition in a child shows the relationship
of the aorta (AO, right) and pulmonary artery (PA, left). Both arise
from the right ventricle (RV) and the ventricular septal defect
(arrow) can be extended to the aorta. (LV: Left ventricle). (Movie
clip 74.62).

A
Figs 74.63A and B

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1783

Figs 74.63A to F: Live/real time, three-dimensional transesophageal echocardiography of right coronary artery to right atrium fistula.
(A) Qlab study. Top arrows and the bottom right arrow point to the origin of the dilated RCA. The RCA opening viewed en face (arrow
in bottom left panel) measured 1.70 cm 1.55 cm, area 1.96 cm2; (B) Vertical arrowhead shows the continuity of the descending and
the ascending (transverse arrowhead) limbs of the fistula. Arrow shows origin of the dilated RCA; (C) Qlab cropping also shows the
continuity (lower arrowhead) of the descending and ascending (upper right arrowhead) limbs of the fistula. Arrow in upper left and lower
right panels show the origin of dilated RCA; (D) The arrow shows flow signals moving from the fistula (arrowheads) into RA; (E and F)
Represent regular (E) and Qlab (F) en face views (arrow) of the opening of fistula into RA. This measured 1.83 cm 1.43 cm, area 3.94
cm2. Arrowheads show segments of the fistula. (AO: Aorta; AV: Aortic valve; RA: Right atrium). (Movie clips 74.63B to 74.63F).
Source: Reproduced with permission from Mishra J, Puri HP, Hsiung MC, et al. Incremental value of live/real time three-dimensional
over two-dimensional transesophageal echocardiography in the evaluation of right coronary artery fistula. Echocardiography. 2011;28:
8058.

1784

Section 6: Congenital Heart Disease

SINUS OF VALSALVA ANEURYSM

A sinus of Valsalva aneurysm is an unusual cardiac
anomaly that can be congenital or acquired and often
involves the right coronary sinus.76 Patients with sinus of
Valsalva aneurysm are frequently asymptomatic, therefore,
the diagnosis is either made incidentally on imaging or if
the aneurysm ruptures. 2D TEE has value in identifying
a sinus of Valsalva aneurysm, but has limitations in the
diagnosis of a rupture. Color Doppler can be helpful in
this situation, however, in the setting of hypovolemic
shock after a rupture, the findings are often limited. 3D
TEE incorporates multiple geometric planes and various
angulations with cropping of images to localize a sinus of
Valsalva aneurysm and its exact site of rupture which may
not be evident on 2D TEE (Figs 74.64 and 74.65). Due to
the severity of a ruptured aneurysm, urgent identification
and intervention must take place providing 3D TTE with
diagnostic utility.

Ventricular Septal Aneurysm Causing

Right Ventricular Outflow Obstruction

obstruction referred for surgical intervention.77 Using 3D

reconstruction capabilities, the ventricular septal aneurysm,
the right ventricular outflow tract, and the pulmonary valve
could be seen en face (Figs 74.66 and 74.67). With the shortaxis view, the aneurysm could be visualized extending almost
throughout the entire right ventricular outflow tract near
the level of the pulmonary valve during systole consistent
with severe obstruction, a feature 2D TEE was unable to
perform. 2D TEE located the VSD, but calculations made
by continuous-wave Doppler were indicative of less severe
outflow obstruction likely related to the intricacy of lining
up the Doppler wave in the appropriate direction along
the right ventricular outflow tract.

Hypoplastic Left Heart Syndrome

In this entity the left sided structures including the left
ventricle, the left atrium, mitral valve, aortic valve, and the
aorta are small and under developed to a varying degree. The
right heart is very prominent (Fig. 74.68). These newborns
need urgent surgery to maintain the systemic circulation.

CONCLUSION

Aneurysm of the ventricular septum can result in right

ventricular outflow tract obstruction and represents
an unusual congenital cardiovascular abnormality.
Baweja et al. described a patient with a large VSD and
aneurysm with suspected right ventricular outflow tract

The use of 3DE alone or along with 2D echocardiography

helps to increase the confidence in the diagnosis of
CHD.78,79 As technology continues to improve, 3DE has
the potential to expand and impact the management and
treatment of this growing patient population.

Figs 74.64A and B

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1785

Figs 74.64A to D: Live/real time, three- dimensional transesophageal echocardiography in ruptured right sinus of Valsalva aneurysm.
(A and B) Arrow points to site of rupture of the aneurysm (AN); (C and D) Arrow points to the mouth of the aneurysm viewed en face.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). (Movie clips 74.64A to 74.64D).
Source: Reproduced with permission from Raslan S, Nanda NC, Lloyd L, Khairnar P, Reilly SD, Homan WL. Incremental value of live/
real time three-dimensional transesophageal echocardiography over the two-dimensional technique in the assessment of sinus of
Valsalva aneurysm rupture. Echocardiography. 2011;28:10415.

Fig. 74.65: Autopsy in ruptured right sinus of Valsalva aneurysm.

Arrow points to the rupture. (AO: Aorta, LV: Left ventricle).
Source: Reproduced with permission from Raslan S, Nanda NC,
Lloyd L, Khairnar P, Reilly SD, Homan WL. Incremental value of live/
real time three-dimensional transesophageal echocardiography
over the two-dimensional technique in the assessment of sinus of
valsalva aneurysm rupture. Echocardiography. 2011;28:10415.

1786

Section 6: Congenital Heart Disease

Figs 74.66A and B: Transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow
obstruction in an adult. (A) The arrow points to the ventricular septal aneurysm bulging into right ventricular outflow tract. The ventricular
septal defect is delineated by the arrowhead; (B) Color Doppler examination shows a narrow turbulent jet (black arrowheads) indicative
of significant obstruction produced by the aneurysm (arrow). (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery;
RA: Right atrium; TV: Tricuspid valve).
Source: Reproduced with permission from Baweja G, Nanda NC, Nekkanti R, Dod H, Ravi B, Fadel A. Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow obstruction in an adult. Echocardiography. 2004;21:957.

A
Figs 74.67A and B

Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease

1787

Figs 74.67A to D: Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing
ventricular outflow obstruction in the same patient as in previous figure. (A) Short-axis (en-face) view just below the level of the
pulmonary valve (PV) showing the aneurysm (arrow) practically occupying the entire right ventricular outflow tract (RVO) indicative of
severe obstruction; (B) Long-axis view also shows the aneurysm (arrow) bulging into the RVO; (C and D) Short-axis view of the PV
(black arrow); (C) Normal opening of the PV during systole indicative of absence of any obstruction at this level; (D) The PV in closed
position in diastole. VS, ventricular septum. (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium;
TV: tricuspid valve).

Fig. 74.68: Right ventricle in hypoplastic left heart syndrome. This

is a subcostal acquisition of the entire right ventricle (RV) in an
infant with hypoplastic left heart syndrome (HLHS). (PA: Pulmonary
artery). (Movie clip 74.68).

ACKNOWLEDGMENT
We thank Dr Maximiliano German Amado Escauela for
his help.

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1788

Section 6: Congenital Heart Disease

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echocardiography. Echocardiography. 2009;26(2):2247.
75. Mishra J, Puri HP, Hsiung MC, et al. Incremental value of
live/real time three-dimensional over two-dimensional
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echocardiography over the two-dimensional technique
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CHAPTER 75
Echocardiography in the Evaluation of
Adults with Congenital Heart Disease
Reema Chugh

Snapshot

Key Concepts of Echocardiography in Adults with

Congenital Heart Disease

Simple Congenital Heart Defects in Adults

Valvular Disease

Complex Congenital Heart Defects

Most of the fundamental ideas of science are essentially simple, and may,
as a rule, be expressed in a language comprehensible to everyone.
Albert Einstein

INTRODUCTION
Advancements in pediatric, medical care, surgical, and
interventional techniques have improved survival into
adulthood for over 85% of those born with congenital
heart defects (CHDs) in developed countries. Depending
upon the type of defect, presenting symptoms, and the
availability of medical resources, many are diagnosed in
infancy and childhood, while in others, the diagnosis is not
made until adulthood. Transthoracic echocardiography is
the primary diagnostic imaging test, while other modalities
such as cardiac catheterization, magnetic resonance
imaging (MRI), or computed tomographic angiography
(CTA) are used as confirmatory or complementary tests
for complete assessment and evaluation of extracardiac
structures in adults with congenital heart disease (ACHD).
While coronary angiography remains the gold standard for
assessment of coronary artery disease, MRI is the reference
standard for quantification of the right ventricular volume

and function. Adults with devices that are not MRI

compatible may undergo CTA at the expense of exposure
to radiation.
This chapter is specifically written for an adult
cardiologist and may appear very basic to a pediatric
cardiologist. However, the knowledge of residua and
sequelae resulting from long-term survivorship of our
patients into adulthood, who are trending into geriatric
years may be beneficial to all, since both pediatric and
adult cardiologists are vested in favorable outcomes
for all our patients. While two-dimensional (2D) echocardiogram with Doppler and color flow imaging are an
essential part of routine diagnostic assessment, threedimensional (3D) echocardiography adds more details.
Transesophageal echocardiography (TEE) aids in viewing
posterior structures, interatrial septum, pulmonary
veins, pulmonary artery, and the aorta. Intraoperative
TEE helps in directing and confirming the results of the
surgery. A concise and adapted version of the American

1792

Section 6: Congenital Heart Disease

Society of Echocardiography guidelines for performance

of transthoracic and transesophageal echocardiography
applicable to ACHD are listed in Tables 75.1 and 75.2,
respectively.1,2 Exercise stress echocardiography assesses
functional capacity, systemic ventricular contractile
reserve, the impact of exercise on pulmonary hypertension,
severity of valve diseases, and arrhythmias.
All adults with CHD need routine lifelong followup for early identification of problems. Many will need
reoperations and interventions due to residual shunts,
prosthetic valve dysfunction, conduit stenosis, baffle
leaks, or may require heart transplantation. Common
clinical issues faced by these individuals are impaired left
ventricular function resulting in heart failure, pulmonary
hypertension, progression of valvular disease (regurgitation and stenosis), bradyarrhythmias/tachyarrhythmias
(atrial and ventricular), endocarditis, aortic/pulmonary
artery dilatation/aneurysms, shunt problems, and conduit
failures. It is essential to make every effort to acquire prior
medical records, especially the operative notes and results
of previous imaging studies in order to understand each
individuals unique anatomy and gauge follow-up.
In addition, women with CHD in their reproductive
years need echocardiography to aid in preconception
counseling, assessment of underlying defects to determine
the maternalfetal risk, guide follow-up during pregnancy,
labor, delivery, and postpartum.
An echocardiographer also holds the responsibility of
identifying lesions that may need medical management,
Table 75.1: Indications for Performing Transthoracic Echocardiography (TTE) for Adults with Congenital Heart Diseases
(ACHD)

interventional procedures, or surgeries in a

manner. The American College of Cardiology
American Heart Association (AHA) guidelines
management of adults with congenital heart

timely
(ACC)/
for the
disease

Table 75.2: Indications for Performing Transesophageal Echocardiography (TEE) for Adults with Congenital Heart Diseases
(ACHD)

Diagnostic indications
Identification of defects in adults with poor acoustic transthoracic windows
Identification of defects that have limited visibility on TTE
(such as a sinus venosus defect, fenestrated ASD, origin of
the coronaries)
Rule out cardioembolic source in an adult with stroke,
including assessment of possible right-to-left shunt due to
a PFO with an agitated saline contrast study; examination
of the left atrial appendage for a thrombus, atherosclerotic
plaques in the proximal aorta
Examination of intra- or extracardiac baffles following the
Fontan, Senning, or Mustard procedure
Evaluation of vegetation, perforation, endarteritis, or suspected abscess
Ruling out an intracardiac thrombus prior to cardioversion
for atrial flutter/fibrillation
Status of prosthetic valves
Guiding catheter-based interventions
Directing placement of ASD or VSD occlusion devices
accurate assessment of size, shape, location of the defect,
and identifying associated defects
Delineation of structures not clearly visible on transthoracic
echocardiography (such as pulmonary veins)
Catheter tip placement for dilation and stent implantantion
for conduit/baffle stenoses in catheterization laboratory

Initial evaluation in an adult suspected of having CHD

Guidance during radiofrequency ablation procedures

Follow-up of ACHD when there is a change in clinical status

or cardiac examination

Perioperative indications

Re-evaluation to guide therapy in adults diagnosed with CHD

Routine surveillance (<1 year) of adults with CHD following
incomplete or palliative repair with residual structural or
hemodynamic abnormality but without a change in clinical
status or cardiac examination
Routine surveillance (<2 years) of adults with stable CHD
following complete repair, without a change in clinical status/
examination, hemodynamic abnormality, or known residual
structural defects.
Source: Adapted from 2011 American Society of Echocardiography guidelines for appropriate use criteria for echocardiography.1
(Free download of the detailed document is available at:
http://www.asecho.org/files/AUCEcho.pdf )

Immediate preoperative definition of cardiac anatomy and

function
Assessment of postoperative myocardial function
Rule out postoperative surgical results, residual shunts, or
valvular regurgitation
Assessment of the results of a minimally invasive surgical
incision or a video-assisted cardiac procedure
Evaluation and monitoring of a postoperative patient with
an open sternum or poor acoustic windows
Source: Adapted from American Society of Echocardiography
2005 guidelines for ASE Indications and Guidelines for Performance of Transesophageal Echocardiography in the Patient with
Pediatric Acquired or Congenital Heart Disease2 (Free download
available: http://www.asefiles.org/pediatrictee.pdf)

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

determined Class I recommendations, for which there

are sufficient data to clearly support the benefits of
interventions/surgeries over the potential risks.3 On the
other hand, with the Class III recommendations, the risks
outweigh the benefits and the treatment or procedure
should not be performed because it may even be harmful.
The Class IIa and IIb recommendations lie in a gray
zone where limited benefits outweigh the risks, making
the treatment/procedures reasonable on an individual
basis. In this chapter, the Class I and III recommendations
identifiable by echocardiography are discussed, allowing
the echocardiographer to play an active role in appropriate
management of this population.

KEY CONCEPTS OF ECHOCARDIOGRAPHY IN ADULTS WITH

CONGENITAL HEART DISEASE
A Simplified Segmental Approach
Unlike echocardiography in infants and children, the
echocardiographic windows are far more limited in adults,
primarily due to body habitus and rib spacing. In addition,
adult cardiologists have a different approach to diagnosis

1793

and management of structural heart diseases. They are also

not entrenched in the numerous classification systems
or detailed embryological understanding of congenital
heart defects. Often the terminology, vocabulary words,
and terms are unfamiliar and baffling. This can make
echocardiography in ACHD for the adult cardiologist quite
overwhelming and intimidating. Table 75.3 lists some of
the commonly used terms and Table 75.4 lists some names
of the common surgeries for CHD.
There is a shift from a wide spectrum of defects
ranging from the undiagnosed simple to complex in
children versus simple undiagnosed defects to previously
diagnosed/operated defects with residua and sequelae
in adults. Traditionally, most centers perform adult
echocardiograms with the orientation of the images
upside down unlike the images seen in conventional
pediatric echocardiography. A basic, practical, and yet
systematic approach is, therefore, necessary to allow an
adult cardiologist to assess this population with clarity,
accuracy, and confidence. As pointed out by Dr John
Child, guru of echocardiography in adults with CHD,
Always perform a complete examination! This step-bystep approach entails the following.

Table 75.3: Common Terminology in Adult Congenital Heart Disease (ACHD)

Atrioventricular (AV) concordanceright atrium empties into the right ventricle, left atrium empties into the left ventricle
Atrioventricular (AV) discordanceright atrium empties into the left ventricle, left atrium empties into the right ventricle
Dextrocardiaapex of the heart points to the right (old term dextroversion)failure of pivoting of the cardiac apex to the left
Levocardiaapex of the heart points to the left
Mesocardiaapex of the heart points to the middle
Dextropositiondisplacement of the heart in the right chest due to a space occupying structure
Eisenmenger physiologydevelopment of irreversible pulmonary hypertension with equalization of pulmonary and systemic
pressures, with pulmonary vascular resistance exceeding systemic vascular resistance leading to flow reversal (right to left) through
an intracardiac shunt
Right isomerismbilateral right handedness (two morphological right atria), asplenia, both the aorta (anterior) and inferior vena
cava (posterior) are on the same side of the spine
Left isomerismbilateral left handedness (two morphological left atria), polysplenia, both the aorta (posterior) and inferior vena
cava (anterior) are on the same side of the spine
Situs inversus totalisheart is located in the mirror-image position of normal
Ventriculoarterial concordancethe morphological right ventricle pumps into the pulmonary artery, while the morphological left
ventricle pumps into the aorta
Ventriculoarterial discordancethe morphological right ventricle pumps into the aorta, while the morphological left ventricle
pumps into the pulmonary artery (transposition of the great arteries)
Restrictive VSDusually small in size with a left-to-right shunt without causing significant hemodynamic derangement
Nonrestrictive VSDlarge VSD at risk for reversal of the shunt (right-to-left). Hemodynamically, the pulmonary arterial and aortic
pressures are equal so the magnitude and direction of the shunt is determined by the pulmonary vascular resistance (PVR).

1794

Section 6: Congenital Heart Disease

Table 75.4: Common Surgical Terms in Adult Congenital Heart Disease (ACHD)

Classic BlalockTaussigThomas shuntsubclavian to pulmonary artery anastomosis to increase pulmonary blood flow
Modified BlalockTaussigThomas shuntsubclavian to pulmonary artery anastomosis, using a Gore-tex graft, to increase pulmonary blood flow
Potts shuntdescending aorta to left pulmonary artery anastomosis to increase pulmonary blood flow
Glenn shuntsuperior vena cava to right pulmonary artery anastomosis to provide low pressure pulmonary blood flow
Atrial switch repairsin both types of atrial switch repairs, the deoxygenated blood from the vena cavae goes through a baffle into
the left ventricle, which pumps the blood into the pulmonary artery. The oxygenated blood returning from the lungs passes through
the pulmonary veins via another baffle into the right ventricle that pumps the blood through the aorta into the systemic circulation.
Thus, the morphological right ventricle becomes a systemic ventricle and the morphological left ventricle, a subpulmonic ventricle.
Senning atrial switch repair for DTGAbaffles are created from the patients tissues (right atrial wall and part of the atrial septum)
Mustard atrial switch repair for DTGAbaffles are created from the pericardium and synthetic material
Waterston shuntascending aorta to right pulmonary artery anastomosis to increase pulmonary blood flow
Rastelli procedureright ventricle-to-pulmonary artery conduit to allow blood flow from the right ventricle to reach the branch
pulmonary arteries, usually in patients with right ventricular outflow tract obstruction
Rashkind procedurecatheter-based procedure for balloon atrial septostomy to allow intermixing of the deoxygenated right atrial
blood with the oxygenated left atrial blood in DTGA
Fontan (classic)right atrial to pulmonary artery anastomosis in tricuspid atresia
Jatenearterial switch for DTGA (anatomical correction with reimplantation of the coronaries)
Biventricular repairoperation to establish two functioning ventricles without the mixing of the venous and systemic circulations,
by closing the shunt defects and creating a connection between the right ventricle and the pulmonary circulation. Usually performed for tetralogy of Fallot, pulmonary atresia with VSD, transposition of the great arteries, and double-outlet ventricles.

Position of the Apex

The position of the apex can be determined from the
standard subcostal views. In dextrocardia, the apex points
to the right, while in levocardia it points to the left. The
middle position is described as mesocardia. Congenitally
corrected transposition of the great arteries (CCTGAs)
should be considered in the presence of dextrocardia,
especially when the gastric bubble is on the left and the
cardiac apex is on the right.

Determination of Atrial Situs

In atrial situs solitus, the morphological right atrium is
located on the right side and the morphological left atrium
is located on the left side. The most specific identifying
feature of the right atrium is the eustachian valve (Fig.
75.1). In the subcostal views or the apical four-chamber
views (with posteriorly tilted probe), it can often be traced
from the orifice of the inferior vena cava, across the floor,
to the lower portion of the atrial septum. The right atrium
can usually be identified by the drainage of the inferior
vena cava except in situs ambiguous (isomerism). The
superior vena caval drainage is not as predictable, since

Fig. 75.1: Transthoracic echocardiogram (apical four-chamber

view) showing an echodensity in the right atrium due to the remanent of a eustachian valve. (EV: Eustachian valve; LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).

it can drain into either atria or into both as in the case of

persistent left superior vena cava. The left atrium usually
has pulmonary veins draining into it, other than in the
case of an anomalous pulmonary venous drainage. In situs
inversus, this relationship is reversed.

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1795

Determination of Atrioventricular
Relationship (the Location of Ventricles by
Bulboventricular Loop)
In the D-loop, the right ventricle is on the right while the
left ventricle is on the left. In the L-loop, there is ventricular
inversion with the morphological right ventricle on the
left and the morphological left ventricle on the right side.
Since the atrioventricular (AV) valves always follow their
respective ventricles, the tricuspid valve is attached to
the morphological right ventricle and the mitral valve is
attached to the morphological left ventricle. Accordingly,
the positions of the tricuspid and mitral valves are also
transposed in the L-loop. Other identifying features of the
AV valves are their positions and morphology. In the apical
four-chamber view, the position of the trileaflet tricuspid
valve is closer to the apex as compared with the mitral
valve. In the parasternal short-axis view, the mitral valve is
shaped like a fish mouth and has chordal attachments to
the two papillary muscles.
The crescent-shaped right ventricle is heavily
trabeculated as compared with the ellipsoid left ventricle.
The left ventricle usually has a smooth endocardial border
except in adults with left ventricular noncompaction or
in those with long-standing hypertension. In the right
ventricle, the presence of a moderator band (a prominent
muscle bundle crossing from the septum to the free wall),
and septal attachment of the tricuspid valve positioned
more apically that the mitral valve (since the AV valves are
always attached to their respective ventricles) provides
further confirmatory evidence.

Ventriculoarterial (VA) Connections

In a normal/concordant connection, the right ventricle
pumps into the pulmonary artery while the left ventricle
pumps into the aorta, with the position of the aorta
being posterior and to the right of the pulmonary valve.
In transposition of the great arteries/discordant VA
connection, the morphological right ventricle pumps into
the aorta while the morphological left ventricle pumps into
the pulmonary artery. The pulmonary artery is identified
by its right and left branches in the high parasternal shortaxis view. The aorta is identified by its candy cane or
hockey-stick appearance in the suprasternal views. The
cranial branches (brachiocephalic, common carotid, and
subclavian) may also be delineated in this view (Fig. 75.2).
The origin of the coronary arteries may be seen in the high

Fig. 75.2: Diagrammatic representation of the aortic arch (AoA)

in the suprasternal view, displaying the branches. (BC: Brachiocephalic; C: Carotid; SC: Subclavian).

parasternal short-axis view. The aortic and pulmonary

valves remain loyal to their respective great arteries.

Abdominal Situs
Abdominal situs is usually concordant with atrial situs.
Hence, it is often used to determine the atrial situs,
although the two follow each other around 70% of the
time. In atrial situs solitus, this relationship can be revealed
in the subcostal views by tracing the path of the inferior
vena cava, which is nonpulsatile (blue on color Doppler)
and located on the right of the spine as it passes through
the liver. The descending thoracic aorta is pulsatile (red on
color Doppler) and courses on the left of the spine. Gastric
folds and stomach bubble are also seen on the left side.
However, in atrial situs inversus, all these positions are
reversed with aorta to the right of the spine and the inferior
vena cava to the left of the spine. The subcostal view also
allows determination of direction along which the major
axis of the heart is aligned.

Assessment of the Shunts

Adults with CHD may have congenital shunt defects or
surgically created shunts for palliative repairs. In addition,
patch leaks or suture dehiscence cause iatrogenic shunt
lesions. While color Doppler is the initial method of
choice to detect a shunt, pulsed wave and continuous
wave Doppler allow evaluation of the gradient across the
shunt.

1796

Section 6: Congenital Heart Disease

An agitated saline contrast study, popularly referred to

as the bubble study, is useful for the right heart lesions.
On the other hand, contrast echocardiography using a
defined agent that lasts long enough to make its way to the
left heart allows further delineation of both right and left
defects. An agitated saline contrast study can be performed
by promptly injecting 10 mL of agitated saline contrast (8
mL of saline mixed with 1 mL of blood and 1 mL of air)
through a peripheral intravenous catheter. Its accuracy in
detecting a right-to-left shunt increases significantly by
performing an adequate Valsalva maneuver. The images
are recorded for 5 to 12 beats to assess for an intracardiac
shunt (with prompt appearance of microbubbles) versus
a pulmonary arteriopulmonary shunt (with delayed
appearance of microbubbles).4,5 It should be avoided in
patients with a large intracardiac shunt because of the risk
of cerebral microemboli.
Contrast echocardiography using the perflutren
lipid microsphere injectable suspension should not be
used in adults with suspected right-to-left, bidirectional,
or transient right-to-left cardiac shunts. Based on the
manufacturers approved protocol, it may be used in those
without shunts to opacify the left ventricular chamber
for better visualization of endocardial borders when
echocardiograms are suboptimal. It has also been useful
in defining intracardiac masses, diagnosing left ventricular
noncompaction, left ventricular aneurysm/rupture, and
aortic pathologies including dissection.6
In most adults with major shunt defects, like the atrial/
ventricular septal defects or patent ductus arteriosus,
chamber enlargement serves as a valuable surrogate
for gauging the impact of a long-standing shunt on the
receiving chambers. The right heart is enlarged in atrial
septal defects, while the left heart is enlarged in ventricular
septal defects and patent ductus arteriosus due to increased
pulmonary blood flow directed eventually to the left heart.
A shunt volume calculation based on information derived
from a TTE and TEE plays a limited role for more accurate
assessment of pulmonary to systemic flow (Qp/Qs) in
some adults.

Echocardiography in Pregnancy
The hemodynamic and physiological effects of pregnancy
in women with CHD pose an extra workload on the
cardiovascular system. As the pregnancy progresses into
the second and third trimesters, there is a 30% to 40%
increase in blood volume causing an 18% to 25% increase
in stroke volume. The heart rate rises by 20% and the

combined effect results in a 30% to 50% increase in cardiac

output. The pulmonary as well as the systemic vascular
resistances fall, leading to a mild drop in both the systolic
and diastolic blood pressures.7
An echocardiogram during pregnancy also reflects
these changes.8,9 During the second trimester, there is
increase in sizes of the left and right ventricles and the
left atrium. There is expansion of the left ventricular (LV)
volume in systole and diastole. There is hypertrophy of
the left ventricular walls. By the third trimester, the heart
appears more globular. Biventricular reductions in global
and segmental longitudinal deformations have been noted
as compensatory changes to accommodate the increase in
cardiac dimensions during pregnancy. Ejection fraction
does not change in women with anatomically normal
hearts. However, in women with CHD, the systemic
and pulmonic ventricular size and function should be
followed up during pregnancy and in postpartum,
since a change is likely to occur in those with marginal
contractile reserves. In addition, evaluation of residual
or recurrent lesions, severity of pulmonary hypertension,
and measurement of aortic root size should be performed.
While stress echocardiography using treadmill
exercise testing should be avoided during pregnancy,
this test is very valuable before conception for assessing
the baseline functional capacity, exercise duration,
and workload metabolic equivalents of task (METs).
Attention should be paid to exercise-induced symptoms,
arrhythmias, or ischemia, since these are likely to recur
with the hemodynamic stress of pregnancy. Imaging
provides information about the contractile reserve with
response of systemic and pulmonic ventricle to exercise.
Changes in pre- and postexercise gradients across stenotic
valves, left or right ventricular outflow tract gradients, and
estimated right ventricular systolic pressures (RVSP) are
also reflective of the potential changes during pregnancy,
labor, and delivery.
When strongly indicated, a submaximal treadmill
stress test without exceeding 70% of the maximum agepredicted heart rate on Bruce protocol may be performed
during pregnancy. Unless it is absolutely necessary, any
exposure to radiation should be avoided during pregnancy.
The risk of radiation exposure to the fetus should be
discussed with the mother. If possible, the procedures
should be postponed, until late second or third trimesters
of pregnancy. Abdominal shielding for protection of the
fetus is always necessary.
These women need individualized preconception
counseling. Maternal and fetal outcomes depend

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

upon the severity of underlying defects and maternal

functional status prior to pregnancy. Echocardiography
allows preconception assessment and follow-up during
pregnancy. Other complimentary imaging modalities may
need to be performed in select cases for identification and
correction of hemodynamically significant underlying
defects before pregnancy. Women with severe pulmonary
hypertension, ascending aorta diameter > 4.5 cm, or
lesions that cannot be repaired before pregnancy should
be counseled to avoid pregnancy due to high maternal
morbidity and mortality.10

Aortic Root in Adults with Congenital

Heart Disease
Structural abnormalities of the great arterial walls and
additive hemodynamic stress lead to dilatation of the
aorta and the pulmonary arteries in many congenital
heart defects.11 Progressive dilatation has been noted with
bicuspid aortic valve, coarctation of aorta, large ventricular
septal defect, tetralogy of Fallot/pulmonary atresia
with ventricular septal defect, and in truncus arteriosus11
(Table 75.5).
Echocardiographic assessment of the aortic root
dimension should be routinely performed. Roman et al
described a method of measuring the aortic root at end
diastole by the leading-edge convention in the parasternal
or occasionally, apical long-axis view that displayed the
maximum diameter parallel to the aortic annular plane.
The aortic annular diameter was measured between the
hinging points of the right and noncoronary cusps of the
aortic valve in systole.12 Normal limits in relation to age,
body size, and gender of 2D echocardiographic aortic root
dimensions have been described in patients aged 15 years
or older by Devereux et al.13 In absolute terms, aortic root
dilatation is defined as diameter at the level of a sinus of
Valsalva 4 cm.

1797

In our practice, we make all measurements after

freezing the most suitable systolic frame that allows:
(a) proper identification of the hinging points of the
right and noncoronary cusps of the aortic valve; and (b)
measurements recorded at reproducible anatomical
landmarks perpendicular to the axis of blood flow. These
measurements are taken at the level of the aortic annulus,
midsinuses, sinotubular junction, and about 2 cm distal to
the sinotubular junction as shown in a parasternal longaxis view.14 Linear measurements are then recorded from
inner edge to inner edge (Fig. 75.3). It should also be noted
that in radiographic imaging, aortic root measurements
are made from outer edge to outer edge.
Aortic aneurysms have been known to cause dissection
in association with some of these defects. Operative repair
is indicated in symptomatic patients with aortic diameter
over 4.4 to 5 cm and/or growth >0.5 cm/year, for ascending
aortic aneurysms associated with bicuspid aortic valve or
other genetically mediated disorders that are at high risk
for dissection, according to the clinical practice guidelines
for thoracic aortic disease.15 The aortic root should be
assessed annually if the diameter ranges from 3.5 to 4.4 cm
and semiannually if it is 4.5 to 5.5 cm. A debate about more
specific guidelines for a variety of CHDs continues.

Table 75.5: Congenital Heart Defects in Adults Commonly

Associated with a Dilated Aortic Root

Bicuspid aortic valve

Ventricular septal defect
Coarctation of aorta
Tetralogy of Fallot
Truncus arteriosus
Transposition of the great arteries
Single ventricle (univentricular heart) with pulmonary
stenosis

Fig. 75.3: Diagram showing the aortic root in the parasternal

long-axis view. Measurements are taken (leading edge to leading
edge) at the level of the (1) aortic annulus at the hinge points of
the leaflets, (2) midsinuses, (3) sinotubular junction, and (4) about
2 cm distal to the sinotubular junction.

1798

Section 6: Congenital Heart Disease

SIMPLE CONGENITAL HEART DEFECTS

IN ADULTS
Shunt Lesions
Patent Foramen Ovale
Ruling out a cardiac source of emboli in an adult with a
stroke or transient ischemic attack may be one of the
most common indications for performing a TEE in most
hospitals around the world. Although a TEE can visualize
various causes of cerebrovascular events such as systemic
ventricular/left atrial appendage thrombi, atheromatous
plaques in the ascending aorta/arch, the possibility of
a paradoxical embolus through a patent foramen ovale
(PFO) also needs to be ruled out.
Color Doppler with TTE in the subcostal view or on
multiplane TEE views may alert us of its existence in many
cases. An agitated saline contrast study during TTE or
TEE requires an adequate Valsalva maneuver in an adult
to prove a right-to-left shunt. Transcranial Dopplers are
currently the reference standard for ruling out paradoxical
microemboli.16,17 Three-dimensional echocardiography is
more likely to demonstrate PFO as a slit-shaped tunnellike defect in the atrial septum by TTE in adults with good
acoustic windows or by TEE in all the adults.
Persistent patency of the foramen ovale in adults
makes it the most common CHD, with an autopsy-derived
incidence of around 27% for a probe patent PFO.18 TEE with
saline contrast has detected the association between rightto-left shunting at rest and high membrane mobility, in
patients with cerebrovascular ischemic events who have a
PFO, and are at higher risk for recurrent brain embolism.19
The atrial septum (fossa ovalis membrane) may
demonstrate thinning and marked redundancy. An atrial
septal aneurysm (ASA) is characterized by a redundant,
undulating, interatrial membrane in the region of the fossa
ovalis (Fig. 75.4). The diameter of the base can exceed
15 mm and the amplitude of the interatrial septum
excursion is above 10 to 15 mm. ASA may be associated
with one or multiple PFOs on an average in 70% of the
cases.20 Besides PFO, the ASA may be associated with
mitral valve prolapse, dilated atria, and intracardiac
thrombi, which may explain the increased frequency
of embolic stroke in this population.21 ASA and PFO
synergistically potentiate the risk for cryptogenic stroke
in adults <55 years of age.20,22,23 There is an increased
likelihood of thrombus formation on the left atrial side
of the ASA. Other echocardiographic findings associated

Fig. 75.4: Transesophageal echocardiogram showing an atrial

septal aneurysm (ASA), which can be described as a redundant,
undulating, interatrial membrane in the region of the fossa ovalis.
(LA: Left atrium; RA: Right atrium).

with a potentially higher risk for cerebral embolic events

are eustachian valve anatomy or Chiari network favoring
right-to-left shunting.24
Device closure for PFO in adults with cryptogenic
stroke remains controversial. Information from two
prospective clinical trials, CLOSURE I (Evaluation of the
STARFlex Septal Closure System in Patients with a Stroke
and/or Transient Ischemic Attack due to Presumed
Paradoxical Embolism through a Patent Foramen Ovale)
study25 and Randomized Evaluation of Recurrent Stroke
Comparing PFO Closure to Established Current Standard
of Care Treatment (RESPECT)26 show that between
medical management and PFO device closure, there is
no significant benefit in the outcomes. These studies
evaluated the incidence of recurrent ischemic strokes
in the primary intention-to-treat analysis associated
with closure of a PFO in adults who had a cryptogenic
ischemic stroke. However, in a subset of this population,
device closure was superior to medical therapy alone (in
the prespecified per-protocol and as-treated analyses),
with a low rate of associated risks.26 Another prospective
study conducted by the PC trial investigators reports that
closure of a PFO for secondary prevention of cryptogenic
embolism did not result in a significant reduction in the
risk of recurrent embolic events or death as compared with
medical therapy.27
Currently, the decisions to close or not to close the
PFO in young adults with cryptogenic stroke are being

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

made on humanitarian grounds on a case-by-case basis

after weighing individual risk benefit ratio and providing
an informed consent.
Intracardiac echocardiography (ICE) has played a
major role in device closure.28 More recently, 3D TEE
has taken its place in many centers.29 However, there is a
significant disagreement between TEE and rotational ICE
in measuring fossa ovalis and selecting the device for PFO
closure, particularly in patients with ASA.30

Atrial Septal Defects

Adults with undiagnosed atrial septal defects (ASD)
usually present with dyspnea, fatigue, and palpitations
due to atrial arrhythmias. The various types of atrial septal
defects are secundum, primum, sinus venous, and the rare
coronary sinus defect.31

Echocardiography
Primarily, the subcostal, followed by the high parasternal
and apical four-chamber views are the best views in
assessment of the atrial septum. There is a likelihood
of a false-positive reading of an ASD due to midseptal
dropout in the apical four-chamber view. Pulsed and
color flow Doppler define the direction of the shunt most
accurately at a lower Nyquist level. The direction of the
low-velocity shunt depends on ventricular compliance.
Other low venous flows from the superior and the inferior
vena cava or the coronary sinus can cause a false-positive
signal suggestive of an interatrial communication by color
Doppler. A tricuspid regurgitant jet with the flow directed
toward the interatrial septum can also be misleading.

1799

Right heart enlargement with diastolic flattening

and paradoxical motion of the interventricular septum
indicate right ventricular volume overload in the presence
of a significant left-to-right shunt. On transthoracic
echocardiography, right atrial/ventricular enlargement,
a D-shaped septum (on the short-axis view in diastole)
with paradoxical motion of the interventricular septum,
and varying degrees of pulmonary hypertension occur
due to long-standing left-to-right shunt. When pulmonary
stenosis is present, it may prevent the development of
secondary pulmonary hypertension.
In the absence of pulmonary stenosis, the estimated
right ventricular systolic pressure (RVSP) is a surrogate for
pulmonary artery pressure. As with acquired heart disease, it
is assessed by applying the simplified Bernoulli equation:
RVSP = 4 (tricuspid regurgitation (TR) jet velocity)2 +
right atrial pressure
Right heart enlargement and dilatation of the
pulmonary artery are signs of a significant shunt volume
and are sufficient to warrant closure of the atrial septal
defect. A Qp/Qs calculation is unnecessary in these cases.
Other concomitant defects that contribute to left heart
volume overload are complete atrioventricular septal
defects associated with a cleft mitral valve and mitral
regurgitation.

Types of Atrial Septal Defect

(Figs 75.5A to C)
The secundum ASD is the most common type and
comprises nearly 75% of the ASDs. It is best seen in the
subcostal view. It can be seen in the apical four-chamber

Figs 75.5A to C: Diagram showing locations of the three common types of atrial septal defects (ASD): (A) Secundum ASD;
(B) Primum ASD; (C) Sinus venosus ASD. (LA: Left atrium; RA: Right atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena
cava).

1800

Section 6: Congenital Heart Disease

view but there may be some false-positive dropouts of

the midseptum. Besides right heart enlargement that
may progress to right or biventricular heart failure, these
patients are at risk for paradoxical embolism, progressive
pulmonary hypertension, and have an increased incidence
of atrial arrhythmias.
The ostium primum ASD (20%) that rarely presents as
an isolated finding is best seen in the apical four-chamber
view as an echo dropout in the lower part of the interatrial
septum. It is usually associated with a cleft anterior mitral
valve, which is best seen in the parasternal short-axis view.
Its association with atrioventricular septal defect is later
discussed in this chapter.
The sinus venous defect (5%) is usually associated
with anomalous pulmonary venous drainage and often
missed on a transthoracic echocardiogram. The entire
atrial septum from the orifice of the superior vena cava to
the orifice of the inferior vena cava should be displayed to
identify echo dropout. It is usually best seen in the subcostal
short-axis view, and occurs between the junction of the
superior vena cava and the interatrial septum. It rarely
presents in adults as a dropout between the inferior vena
cava and the interatrial septum.
A rare case of coronary sinus defect (1%) is suspected
especially in the presence left superior vena cava draining
into the roof of the left atrium. There may be partial or
complete absence of the coronary sinus. It is best seen in
a modified apical four-chamber view. A large coronary
sinus orifice shows evidence of left-to-right atrial shunting
due to defect in the roof of the coronary sinus (sinoseptal
defects). The right ventricular systolic pressure needs to
be determined, since pulmonary hypertension is likely
to occur in adults with significant shunts. If there is a
significant right-to-left shunt due to another defect, the
orifice of the coronary sinus may not be so enlarged and
go unrecognized. With the development of pulmonary
hypertension, the low velocity of the shunt flow across
the coronary sinoseptal defect may be confused with
other low-velocity flow states within the atria. Anomalous
pulmonary venous drainage is more commonly associated
with sinus venosus ASD but can also occur in this setting.
Tricuspid atresia has also been reported with a coronary
sinus defect.
Common atrium is a rare condition in which the
atrial septum is nearly or completely absent, causing an
intermixing of the oxygenated and deoxygenated blood.
The right and left sides of the common atrium retain
morphological features of the right and left atria. It may

be associated with an atrioventricular septal defect, cleft

mitral valve, partial anomalous pulmonary venous return,
and left superior vena cava. Pulmonary hypertension may
be present due to increased pulmonary arterial flow. The
best views are the apical four-chamber and the subcostal
views for detecting the absence of the interatrial septum,
defect in the membranous ventricular septum, right
heart enlargement with 2D echocardiography and color
Doppler. The parasternal short-axis views at the aortic level
show dilatation of the pulmonary artery due to pulmonary
hypertension.

Transesophageal Echocardiography
A TEE is necessary in nearly all adults for localizing/sizing
the secundum ASD, measuring septal rims (superior and
inferior), entry of the superior vena cava, connection of all
pulmonary veins, and for ruling out concomitant defects
before planning device closure or surgery. Color Doppler
can determine the major direction of the shunt (Fig. 75.6).
Measurements are made in orthogonal planes to allow
selection for appropriate candidates for device closures.
Three-dimensional TEE allows real time visualization of
the defect. It also allows more defined measurements of the
rims, size, and location with respect to adjacent structures,
and assessment of concomitant defects. In the current
era, it plays a major role in transcatheter implantation of
the ASD closure device.3236 TEE also helps in detecting
procedural complications such as device embolization
into the pulmonary artery or into the iliac artery.37,38 Early
and late complications such as erosions caused by devices
can also be demonstrated by TEE.39

Fig. 75.6: Transesophageal echocardiogram with color Doppler

showing the left-to-right shunt due to a secundum atrial septal
defect (ASD). (AoV: Aortic valve; RA: Right atrium).

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

The presence of right heart enlargement and/or mild

pulmonary hypertension without a visible defect on TTE
should be investigated by a TEE with the probe angled to
around 90 for delineating a sinus venosus defect, and with
posterior angulation of the probe to demonstrate a coronary
sinus defect. TEE plays a pivotal role in assessment of the
pulmonary veins.40,41 It is very useful in diagnoses of these
two types of defects that are often missed by transthoracic

1801

echocardiography.42,43 A synopsis of echocardiographic

assessment of ASDs is reviewed in Table 75.6.

Contrast Echocardiography
An agitated saline contrast study is usually unnecessary
in adults with ASDs but when performed, it may reveal
a bidirectional shunt with the major component of a

Table 75.6: Atrial Septal Defects

Types
Secundummost common type of ASD
Primum ASDoften associated with an endocardial cushion defect/partial AV canal defect (inlet VSD) and cleft anterior mitral valve
Sinus venous ASDusually associated with partial anomalous pulmonary venous return
Coronary sinus ASDcommonly seen with persistent left superior vena cava
Common atrium
Associated defects
Occurs mostly as an isolated anomaly
Partial anomalous pulmonary venous return (sinus venosus defect)
May occur in association with the following defects:
Mitral valve prolapse
Ventricular septal defect
Patent ductus arteriosus
Pulmonary stenosis/right ventricular outflow tract obstruction
Tetralogy of Fallot
D-transposition of the great arteries
Congenitally corrected transposition of the great arteries
Truncus arteriosus
Tricuspid atresia
Ebsteins anomaly
Echocardiographic assessment
Identification of the type of defect
Estimation of the size of the defect in orthogonal views to get the largest dimension with 2D echocardiography or by planimetry
with 3D echocardiography
Color Doppler to assess to direction of the shunt (using low Nyquist limit)
Pulsed wave Dopplercharacteristic flow pattern begins in early systole, although most of the cardiac cycle with a broad peak in
late systole/early diastole, with peak velocity usually < 2 m/s (combination of pulmonary and systemic venous flow)
Right heart sizeright atrium and ventricle are enlarged when the shunt is significant with Qp/Qs over 1.5 (may avoid the need
to calculate Qp/Qs)
Estimation of right ventricular systolic pressure/degree of pulmonary hypertension
Postoperative
Rule out residual shunt
Device closure
Confirm alignment of the device along the interatrial septum
Rule out impingent on surrounding structuresmitral valve, aortic valve, and pulmonary veinsor erosion of the device

1802

Section 6: Congenital Heart Disease

left-to-right shunt. A dense bolus from an intravenous

agitated saline injection shows negative contrast jet
near the defect with passage of a few bubbles into the
left atrium during transient periods of higher right atrial
pressure. Contrast echocardiography is contraindicated
in patients with Eisenmenger physiology, since a large
bolus of microemboli entering the systemic circulation may
potentially cause cerebral ischemia.

hypertension, right heart failure, and the likelihood of

atrial arrhythmias. Adults with large secundum ASD/septal
aneurysm, primum, sinus venosus, or coronary sinus
defects need surgery for closure of the defect and repair
of concomitant defects. Although adults with small ASDs
(< 5 mm) are at low risk of having these complications,
closure may be considered to reduce the risk of paradoxical
embolism.

Exercise Testing

The Postoperative Adult

Exercise testing is useful for objective assessment of

functional capacity and for revealing exercise-induced
arrhythmias, and changes in oxygen saturation in adults
with pulmonary arterial hypertension. Maximal exercise
testing should be avoided and effort should be restricted to
<70% of maximum age-predicted heart rate, in those with
severe pulmonary arterial hypertension.

Following repair of all ASDs or device closure of the

secundum ASD, echocardiography plays an important role
in ruling out residual shunts or pulmonary hypertension.
Intraoperative TEE checks the adequacy of the repair by
confirming the absence of residual shunts in all types of
ASDs after device or surgical repair. It is especially useful
after pericardial patch repair of the sinus venosus defect
in the superior vena cava, with baffle redirecting the
anomalous pulmonary veins to the left atrium. Narrowing
or stenosis of the superior vena cava or pulmonary veins
will manifest as flow acceleration on color Doppler at the
site of the obstruction.
Postoperatively, an urgent TTE is necessary in a
surgical patient presenting with fever, pleuritic chest
pains, and progressive dyspnea with or without abdominal
symptoms to rule out pericardial effusion and possible
tamponade, since there is a likelihood of pericarditis for
several weeks postoperatively.
Device malalignment or impingement on surrounding
structures (pulmonary veins, aortic, and mitral valves)
should be ruled out following device closure. Other
potential complications following ASD device closure are
pericardial effusion, erosion of the atrial wall or aorta,
device thrombosis, or endocarditis within first 6 months
of implantation. TTE should be performed on the day
following device closure, at 1 month, 6 months, at 1 year,
and then annually for a few years post device closure.

Cardiac Catheterization
Diagnostic cardiac catheterization is not indicated in
younger adults with uncomplicated ASD if they have
had adequate noninvasive imaging. Shunt runs are of
value when the site of the defect is unclear, such as in
case of sinus venosus defect or a coronary sinus defect.
Cardiac catheterization is essential for assessment of
pulmonary vasoreactivity in adults with severe pulmonary
hypertension prior to ASD closure. In older adults,
coronary artery disease also needs to be ruled out before
surgical closure. Currently, cardiac catheterization is
mostly performed in conjunction with device closure of an
ASD.

Magnetic Resonance Imaging/Computed

Tomographic Angiography
MRI/CTA delineates the course of the anomalous
pulmonary veins into the right atrium, persistent left
superior vena cava, and allows visualization of other
extracardiac structures.

Ventricular Septal Defects

ASD Closure
The Class I ACC/AHA recommendations for closure of an
atrial septal defect are visual or quantifiable evidence of
right heart overload (right heart enlargement or Qp/Qs
over 1.5).3 Early closure of moderate to large defects lowers
the risk of long-term complications including pulmonary

Although ventricular septal defects (VSDs) are the most

common defects in infancy, they are less prevalent in
adulthood due to spontaneous closure in early life. The
clinical presentation of an isolated VSD through life will
depend largely on defect size and pulmonary vascular
resistance.31,4447

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1803

Fig. 75.7: Transthoracic echocardiogram (apical four-chamber

view) showing that the tricuspid septal leaflet prolapses in an
attempt to close the ventricular septal defect (VSD) leading to
the development of a membranous septal aneurysm. (ASD: Atrial
septal defect; RA: Right atrium; RV: Right ventricle; TV: Tricuspid
valve).

Fig. 75.8: Diagrammatic representation of the venturi effect

causing prolapse of aortic valve (AoV) cusp in an attempt to close
the ventricular septal defect (VSD), leading to aortic regurgitation
(AR) as seen with a transesophageal echocardiogram.

Most individuals are diagnosed in childhood due to a

loud murmur. Device or surgical closure is not indicated
in adults with small VSDs in the absence of pulmonary
hypertension. These adults are at a higher risk of acquiring
endocarditis, where the VSD jet strikes the endocardial
surface close to the tricuspid valve causing tricuspid valve
vegetations that may predispose to pulmonary embolism.
The tricuspid septal tissue may prolapse in an attempt to
close the VSD leading to the development of membranous
septal aneurysm (Fig. 75.7). Large membranous septal
aneurysms can also cause obstruction to the right
ventricular outflow tract.
Similarly, aortic regurgitation can occur when the
aortic cusps (usually right or left) attempt to spontaneously
close a membranous VSD due to venturi effecta
suction force that is created by a gradient across the VSD
that draws the aortic valve leaflet to prolapse and close the
VSD in a flap-like manner (Fig. 75.8).
Moderate to large VSDs may present with impaired left
ventricular function and heart failure. Progressive tricuspid
regurgitation and increasing severity of pulmonary
hypertension are also potential long-term issues. In
some unfortunate cases, a large VSD may predispose
to irreversible pulmonary hypertension (Eisenmenger
physiology) that is no longer amenable to surgical closure.
The VSDs are classified according to their position in
the interventricular septum (Figs 75.9A to C). They have

also been numerically divided into four types, correlating

with their position as noted below.48

Type 1: Supracristal (Also Known as Outlet,

Subpulmonic, Infundibular) VSD
The parasternal short-axis view is the best view to
document a left-to-right shunt in the 2 oclock position at
the level of the aortic valve. It may present as a low-velocity
diastolic shunt flow preceding a left-to-right shunt during
systole, and can be mistaken as other defects such a sinus
of Valsalva aneurysm or a coronary artery fistula, since
they also similarly appear in the parasternal long-axis
view.

Type 2: Membranous (or Perimembranous)

VSD
This is the most common type of VSD in adults and it is
best seen in the parasternal long-axis and short-axis views
with the help of color Doppler that reveals a turbulent
left-to-right shunt. Two-Dimensional echocardiographic
images may show a dropout in the membranous septum
just below the aortic valve in the five-chamber view. Other
suitable views for visualizing this defect are the apical and
subcostal views.

1804

Section 6: Congenital Heart Disease

Figs 75.9A to C: Diagram showing locations of the four types

of ventricular septal defects (VSD). (AoV: Aortic valve; LA: Left
atrium; LV: Left ventricle; PA: Main pulmonary artery; RA: Right
atrium; RV: Right ventricle).

Type 3: Inlet (or AV Canal Type) VSD

This type of VSD is usually associated with a primum ASD
and involves the area around the crux of the heart. It may
present as an AV septal defect with associated AV valve
abnormalities as seen in individuals with Down syndrome.
It is best seen in the apical four-chamber view.

Type 4: Muscular
These VSDs may be multiple or large enough to persist
into adulthood. They are located in the trabecular septum

with a rim of muscle around the defects. They are best

seen in the parasternal long-axis view or in the apical fourchamber view with the help of color Doppler.

Echocardiography
Transthoracic echocardiography with color Doppler
displays location, size, and number of the VSD. For
calculation of pressure gradient across the VSD with
continuous wave Doppler, the cursor should be aligned
along the direction of the VSD for accurate assessment
of the shunt. Heart chamber dimensions, presence of

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

right or left ventricular outflow obstruction, and left

ventricular size and function should be estimated on serial
echocardiograms. A significant volume of blood flow is
directed from the left to the right shunt into the pulmonary
artery leading to an increased left heart volume. Over
time, this causes left heart enlargement and decreases
left ventricular function. The aortic valve morphology
and function should be evaluated to rule out prolapse
and regurgitation. Estimation of right ventricular systolic
pressure (RVSP) is usually performed using the pulmonary
regurgitation jet, since contamination of the tricuspid jet
signal by a VSD jet, through the left-to-right shunt, may
overestimate RVSP. Associated defects that need to be
ruled out are listed in Table 75.7.

Magnetic Resonance Imaging/

Computed Tomography
These imaging modalities are useful for assessment of
apical and supracristal VSDs in unusual locations, and for
evaluation of extracardiac structures such as the aorta.

Cardiac Catheterization
It is usually performed in conjunction with device
closure of the defect. The major role of diagnostic
cardiac catheterization is in assessment of pulmonary
artery pressures and pulmonary vasoreactivity prior to
closure, when pulmonary hypertension is suspected on
echocardiography. In addition, coronary arteriography is
performed in older adults prior to surgical closure of the
defect.

VSD Closure
The Class I ACC/AHA recommendations for closure of a
VSD are the evidence of left ventricular volume overload
(Qp/Qs 2.0) or a history of infective endocarditis. VSD
closure is contraindicated in adults with severe irreversible
pulmonary vascular disease.
At other times, VSDs are closed during surgery for
aortic regurgitation and other associated defects.49
Although there is no Class I indication for catheterbased device closure of a VSD in an adult, the Class IIa
indications for device closure include high surgical risk
with hemodynamically significant residual shunt or
history of infective endocarditis. In these cases, the VSD
should not be located proximal to the aorta or the tricuspid

1805

valve, because otherwise there will be device impingement

on these structures.
Intraoperative TEE with color Doppler allows detection
of multiple VSDs, especially muscular or supracristal
preoperatively, since these may not be directly visible to
the surgeon.
After the operation, TEE confirms patch closure
and rules out residual defects. Patch closure and use of
intraoperative TEE have been shown to improve surgical
outcomes.49
A comprehensive assessment of concomitant defects
requiring surgery, such as aortic valve repair/replacement
for regurgitation, resection of subaortic membrane in
case of left ventricular outflow tract obstruction, and
relief of right ventricular outflow obstruction by adequate
resection and patch should be performed.

The Postoperative Adult

Postoperatively, TTE is useful for assessment of left
ventricular function, ruling out residual shunts or
pulmonary hypertension. Associated defects such as
aortic root dilatation and aortic regurgitation need longterm follow-up.

Patent Ductus Arteriosus

Survival into the ninth and tenth decades of life is possible
in an adult with an unoperated patent ductus arteriosus
(PDA). The PDA is a vascular structure that connects the
inferior curvature of the proximal descending aorta to
the roof of the main pulmonary artery near the origin
of the left pulmonary artery in fetal life, and is designed
for spontaneous closure in early infancy. Persistent
ductus arteriosus may occur in adulthood when spontaneous, surgical, or device closure has failed to occur in
childhood.31

Echocardiography
Echocardiography is sensitive and very specific in
diagnosing PDA. Tiny PDAs may sometimes be missed
and there is low likelihood of a false-positive PDA.50,51
Two-dimensional TTE is able to demonstrate a
persistent anatomical connection between the descending
aorta and the pulmonary artery only in some adults. This is
due to limited acoustic windows because of body habitus
or secondary to lower ultrasonic frequencies hampering

1806

Section 6: Congenital Heart Disease

Table 75.7: Ventricular Septal Defects

Types
Perimembranousmost common
Supracristal/infundibular/subpulmonic
Muscular
Atrioventricular (AV) septal defect [inlet/atrioventricular (AV) canal defect]
Associated defects
Atrial septal defect
Patent ductus arteriosus
Coarctation of aorta
Aortic root dilatation
Aortic regurgitation
Tricuspid regurgitation
Echocardiographic assessment
Identification of the type of defect
2D and color Doppler evaluation of the size of defect in systole
Color and continuous wave Doppler to determine the direction and gradient of the shunt
Is it a restrictive or nonrestrictive VSD?

Impact on chamber size

Left atrial dilation, left ventricular dilatation (due to excess blood going through the lungs because of the left-to-right shunt)
Right heart enlargement/right ventricular hypertrophy occurs in adults with Eisenmenger physiology

Ventricular functionleft ventricular systolic function

Rule out associated defects
Examination of the aortic valve (size in systole, degree of aortic regurgitation in diastole)aortic regurgitation is usually associated with supracristal or perimembranous VSD
Estimation of right ventricular systolic pressure/degree of pulmonary hypertension
Postoperative
Rule out residual ventricular septal defect
Estimation of right ventricular systolic pressure/degree of pulmonary hypertension
Assessment of associated defects
Ventricular function

with the visualization of the PDA, variations in position,

alignment, and shape and length of the ductus. In order
to see the entire ductus connect to the inferior curvature
of the descending aorta, the probe should be rotated
clockwise.
Color Doppler can diagnose whether the flow is due to
a PDA or not, by freezing or slowly moving frames in order

to trace the path of the jet of color Doppler flow. Although

the jet appears to be arising from the bifurcation of the
pulmonary artery, the actual origin of the shunt is in the
descending thoracic aorta.
The best view is the high left parasternal short-axis
view above the level of the aortic valve, or a modified
suprasternal view that achieves a long-axis view of the

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

right ventricular outflow tract and the main pulmonary

artery. Color Doppler displays the bifurcation of the
pulmonary artery and its branches, by angling the probe
leftward and superiorly in the high parasternal short-axis
view. The bifurcation of the main pulmonary artery may
appear to trifurcate with the ductus arising along with
left and right pulmonary arteries (Fig. 75.10).52
In a typical PDA with a left-to-right shunt, a color
Doppler jet may be seen flowing through the left pulmonary
artery into the main pulmonary artery with marked
spectral dispersion in systole and diastole (throughout the
cardiac cycle in adults). The maximal velocity is recorded
by placing the Doppler sample volume just proximal to the
bifurcation. The systolic and diastolic flow can be recorded
from the high parasternal window, with an ultrasound
beam aligned directly into the orifice of the PDA. Color
Doppler will display diastolic flow from PDA along the
lateral wall of the pulmonary artery, while pulsed wave
Doppler will demonstrate flow reversal along the anterior
wall of the pulmonary artery. The machinery murmur
auscultation correlates with the continuous variation of the
flow between systole and diastole on echocardiography.
In the suprasternal view, pulsed wave Doppler may
demonstrate holodiastolic flow reversal in the descending
aorta due to antegrade flow into the ductus in diastole. Flow

Fig. 75.10: Diagrammatic representation of the high parasternal

short-axis view on a transthoracic echocardiogram showing the bifurcation of the main pulmonary artery that may appear trifurcate
with the ductus arising along with left and right pulmonary arteries.
(Desc Ao: Descending aorta; LA: Left atrium; LPA: Left pulmonary
artery; MPA: Main pulmonary artery; PDA: Patent ductus arteriosus; RA: Right atrium; RPA: Right pulmonary artery).

1807

across the ductus causes pressure equalization between

the aorta and the pulmonary artery first in diastole but
with increasing severity of pulmonary hypertension, the
pressure difference between aorta and the pulmonary
artery will decrease in systole. To improve the accuracy of
estimated right ventricular systolic pressure calculated by
using the modified Bernoullis equation (4V2), a bolus of
saline is injected via peripheral intravenous catheter.53 This
enhances the profile of the maximum tricuspid regurgitant
jet velocity obtained on pulsed Doppler. The left heart
chambers size depends upon the size of the PDA. Adults
with a small PDA may have normal cardiac dimensions
and no echocardiographic evidence of pulmonary
hypertension, while those with moderate to large PDA may
have left heart enlargement with impaired left ventricular
function and varying degrees of pulmonary hypertension.
A dilated, aneurysmal, and calcified main pulmonary
artery may be seen in adults with a long-standing shunt
past the third decade of life. In a dilated pulmonary artery,
low velocity retrograde flow can be demonstrated in late
systole. With rising pulmonary vascular resistance, there
is a bidirectional shunt with right-to-left flow seen in early
systole and a left-to-right flow seen in late systole/diastole.
This may further deteriorate to irreversible pulmonary
hypertension and Eisenmenger physiology. Although
most PDAs occur in isolation, associated defects such as
VSD, coarctation of aorta, and complex CHD should be
evaluated.
Coronary artery fistula, anomalous coronary artery,
aortopulmonary collateral, ruptured sinus of Valsalva,
and pulmonary regurgitation may appear like ductal flow,
since they produce turbulence in the main pulmonary
artery with color Doppler examination.
Three-dimensional transthoracic echocardiography
may be helpful in differentiating vascular structures
from artifacts and echo dropouts. For a comprehensive
assessment, one must acquire 3D color Doppler flow
signals from the PDA jet, main pulmonary artery, and the
descending thoracic aorta followed by rotation to view
the flow signals from all sides at any desired angulation.
By rotating these isolated color Doppler images from 0 to
180, one can visualize flow in pulmonary arteries, PDA,
and the descending aorta in three dimensions. Color
Doppler 3D transthoracic echocardiography can also
demonstrate flow signals moving from the pulmonary
artery into the descending thoracic aorta in systole and
back into the pulmonary artery in diastole.5456

1808

Section 6: Congenital Heart Disease

Transesophageal Echocardiography
In adults with limited acoustic windows, TEE is helpful in
assessment of the PDA and associated defects. It may reveal
more details such as the anatomy of ductus arteriosus and
its relationship with the descending aorta and pulmonary
artery. Real time 3D TEE helps in imaging of the PDA,
before and after Amplatzer device closure. However, the
cranial part of the aortic arch cannot be visualized because
of the near-field artifact.54
Live 3D TEE plays a major role in the perioperative
monitoring during transcatheter device closure by cutting
down the fluoroscopy time. It is useful in defining the

anatomical details, confirming the position of the device

after closure, and ruling out a residual shunt.56
A synopsis of echocardiographic assessment of the
PDA is reviewed in Table 75.8.

Cardiac Catheterization
Cardiac catheterization is primarily performed at the time
of device closure to determine the anatomy of the ductus,
degree of shunt, pulmonary vascular resistance (PVR),
pulmonary vasoreactivity, associated defects, and coronary
artery disease in older adults. Catheter-based device closure
is mainly guided by fluoroscopy and partially by TEE.

Table 75.8: Patent Ductus Arteriosus

Associated defects
Usually occurs as an isolated anomaly
May be associated with the following defects:
Ventricular septal defect
Coarctation of aorta
Valvular defectsaortic or mitral valves
Complex CHDsin association with complex defects such as congenitally corrected transposition of the great arteries or tetralogy of Fallot.
Echocardiographic assessment
Identification of the defectwhen visible, the bifurcation of the main pulmonary artery may appear trifurcate with the ductus arising along with left and right pulmonary arteriesmeasure length and width when possible
Color Dopplerjet seen through the left pulmonary artery into the main pulmonary artery with marked spectral dispersion in
systole and diastole (throughout the cardiac cycle in adults) in a typical PDA with a left-to-right shunt
Assessment of pressure gradient across the PDA
Measurement of left ventricular dimensions and quantitative assessment of left ventricular functionleft heart enlargement is
common with moderate to large PDA
Estimation of right ventricular systolic pressures/pulmonary hypertension (right ventricular hypertrophy occurs in adults with
Eisenmenger physiology)
Rule out pulmonary artery dilatation, aneurysm, or calcification (often noted by the fourth decade of life)
Assessment of the aortic archrule out flow acceleration/obstruction, diastolic flow reversal
Postoperative
Left ventricular size and function, left atrial enlargement
Rule out residual shunts
Ensure proper device placement (a well-seated device can be visualized between the pulmonary artery bifurcation and the
inferior margin of the aorta. It should not protrude into the aortic lumen or the pulmonary artery)

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

Magnetic Resonance Imaging/Computed

Tomography
These modalities are rarely required for imaging an
isolated PDA for determining suitability for device closure.

1809

AV valve between the atria and ventricles. The cleft in the

left-sided AV valve leaflet results from incomplete fusion
of the superior and inferior bridging leaflets of the AV
valve.

Echocardiography
PDA Closure
The ACC/AHA Class I recommendations3 for device or
surgical closure of PDA in adults are:
Left heart enlargement (atrial and/or ventricular) due
to volume overload or in the presence of pulmonary
hypertension due to a left-to-right shunt
History of endarteritis.
Surgical or device closure of PDA is contraindicated in
adults with severe pulmonary hypertension due to reversal
of the shunt (right to left). Calcification and tissue friability
in adults can make surgical closure challenging and
therefore, it is performed in limited cases. Surgery instead
of device closure should be considered in adults who have
calcified PDA, ductal aneurysm, history of endarteritis,
and very large PDAs.

The AV septal defects are best seen in the apical-four

chamber views. The complete AV is classified into three
Rastelli types based on the extent of chordal attachments of
the superior bridging leaflet. This defect may be associated
with Down syndrome.31
Two-dimensional echocardiography defines the
size of the AV septal defect, AV valve attachments, and
determines the presence of a common AV valve or two
separate AV valves. A distinctive feature of AV septal defect
is the bridging leaflets of the AV valves that are viewed in
the same horizontal plane, on an apical four-chamber view
(Fig. 75.11). A cleft anterior mitral valve is identified in the
parasternal short-axis view (Fig. 75.12).
Color Doppler is used to estimate the severity of the AV
valve regurgitation, and the level, size, direction of atrial
and ventricular shunts.

The Postoperative Adult

Transthoracic echocardiogram and color Doppler are used
in long-term follow-up after device closure or surgery.
Postoperatively, proper device placement is confirmed by
visualizing a well-seated device between the pulmonary
artery bifurcation and the inferior margin of the aorta,
without any protrusion into the pulmonary artery or
aortic lumen. In the immediate postoperative period,
residual shunts should also be ruled out. On subsequent
echocardiograms, smaller shunts are likely to resolve due
to endothelialization over time.

Atrioventricular Septal Defect

Atrioventricular septum is the partition between the left
ventricular outflow tract and facing right atrium in normal
hearts.31 Morphological variations in the anomalies of the
AV septum and the AV valves present as various types of
AV septal defects.57 In partial AV septal defects, there is
primum ASD, with two separate AV valves, a cleft mitral
valve, and no VSD; in intermediate AV septal defect, in
addition to these findings, there is a small restrictive
membranous VSD; while in complete AV septal defect, there
is a large septal defect extending from the primum atrial to
the perimembranous ventricular septum with a common

Fig. 75.11: Diagrammatic representation of the transthoracic apical four-chamber view showing the distinctive feature of AV septal
defect with the bridging leaflets of the AV valves viewed in the
same horizontal plane (as shown with the dotted line). (LA: Left
atrium; LV: Left ventricle; MV: Mitral valve; PV: Pulmonary vein;
RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).

1810

Section 6: Congenital Heart Disease

Fig. 75.12: Transthoracic echocardiogram showing a cleft anterior mitral valve (MV) in the parasternal short-axis view. (AMVL:
Anterior mitral valve leaflet; PMVL: Posterior mitral valve leaflet).

Live/real time 3D TTE shows incremental value over

2D TTE in the evaluation of an adult with a complete AV
septal defect. Most importantly, it provides an en face view
of all the five leaflets of the common AV valve. It allows
more detailed evaluation of shunts (especially from the
left ventricle to right atrium), quantitative assessment of
regurgitant lesions, and classification into one of the three
Rastelli types.58,59

The Postoperative Adult

Postoperative long-term issues that are assessed by
echocardiography include a search for a residual shunt
lesion (post-AV patch repair), degree of mitral (left AV
valve) or tricuspid regurgitation, right ventricular systolic
pressure, degree of mitral stenosis following surgery of
cleft mitral valve, or subaortic stenosis due to the long left
ventricular outflow tract that manifests as a gooseneck
deformity on cardiac catheterization. A synopsis of the
echocardiographic assessment of AV septal defects is
reviewed in Table 75.9.

Persistent Left Superior Vena Cava

Left superior vena cava (LSVC) is the most common thoracic
venous abnormality.60 It is the embryological remnant of
the persistent left anterior cardinal vein. Its prevalence
varies from around 0.3% in the general population to 10%
in patients with CHD. The LSVC usually drains via the

coronary sinus into the right atrium in the rarest type of

ASD called the unroofed coronary sinus defect. It may
be associated with other defects such as a bicuspid aortic
valve, coarctation of the aorta, or cor triatriatum. In 20% of
those who have a persistent LVNC, the right superior vena
cava may be absent, leading to marked dilatation of the
coronary sinus because of increased blood flow.31 The right
superior vena cava may be absent in approximately 15% to
30% of the individuals born with a persistent LSVC. These
individuals have a high likelihood of having an enlarged
coronary sinus because of markedly increased blood flow
into the LSVC.60
The LSVC is often an incidental finding in asymptomatic
adults. It may be of clinical significance during pacemaker/
defibrillator implantation, central venous catheter
placement, and in patients undergoing cardiopulmonary
bypass surgery during retrograde cardioplegia, since the
solution can perfuse retrograde into the left persistent
superior vena cava, thereby decreasing the effectiveness of
cardioplegia.

Echocardiography
TTE with an agitated saline contrast study helps in
making the diagnosis. The agitated saline contrast
injection (bubble study) in the left arm via a peripheral
intravenous catheter flows into the left brachiocephalic
vein with appearance of microbubbles in the left atrium in
case of an unroofed coronary sinus. If the LSVC is draining
into an intact enlarged coronary sinus, an injection
into either the right or left arms (through a peripheral
intravenous catheter) will demonstrate the microbubbles
in the enlarged coronary sinus and then in the dilated
right heart.61 If the right superior vena cava is absent, the
injection of agitated saline contrast into the right arm will
first appear in the enlarged coronary sinus followed by the
right atrium.62

Magnetic Resonance Imaging/Computed

Tomography
Often the LSVC and all the pulmonary veins cannot be
easily identified by TTE or TEE. Like other extracardiac
structures, the LSCV can clearly be identified on MRI or
CTA with 3D reconstruction.60,61

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1811

Table 75.9: AV Septal Defect

Associated defectsUsually occurs in patients with Down syndrome

Patent ductus arteriosusmost common
AV valve regurgitation
Coarctation of the aorta
Left ventricular outflow tract obstruction
Double orifice mitral valve
Muscular ventricular septal defects
Right ventricular outflow tract obstruction
Pulmonary stenosis
Left ventricle-to-right atrial shunt
Gerbode defectright ventricle-to-left atrial shunt
Echocardiographic assessment
Identification of the defect
Primum atrial septal defect
Inlet ventricular septal defect
Cleft anterior mitral valve
Tricuspid commissurewidened (anteroseptal)
Estimation of right ventricular systolic pressures/pulmonary hypertension (right ventricular hypertrophy occurs in adults with
Eisenmenger physiology)
Left ventricular size and function
Right ventricular size and function
Assessment of the size and the gradient across the atrial and ventricular shunt
Degree of AV valve regurgitation
Severity of the associated defects
Postoperative
Biventricular size and function
Rule out residual shunts
Degree of AV valve regurgitationespecially mitral regurgitation due to cleft mitral valve

The Postoperative Adult

Adults with LSVC associated with coronary sinus defect
and partial anomalous pulmonary venous return
require patch closure of the coronary sinus type of ASD
and an intracardiac baffle to redirect the anomalous
pulmonary venous flow into the left atrium. Follow-up
echocardiography focuses on ruling out residual shunts
and persistent pulmonary hypertension from previously
long-standing left-to-right shunts.

Sinus of Valsalva Aneurysm

Sinus of Valsalva aneurysm may be congenital or acquired
with clinical presentation ranging from an asymptomatic

murmur to acute cardiogenic shock. Aneurysm formation

in the aortic sinus can occur at the junction of the aorta and
the annular fibrosa, due to structural wall abnormalities. It
may extend to surrounding structures, causing obstruction
or fistulae. An unruptured aneurysm may go undiagnosed
until it causes symptoms due to right ventricular outflow
obstruction.63 A small perforation may come to our attention
on physical examination because of a continuous murmur
on auscultation. An acute rupture may result in high output
decompensated heart failure. Coronary artery compression
may present as chest pain due to ischemia. Since the sinus
of Valsalva aneurysms originate more commonly from the
right coronary or the noncoronary cusp, they are more likely
to cause right coronary artery compression.

1812

Section 6: Congenital Heart Disease

The differential diagnoses include an acquired

aneurysm from complicated infective endocarditis, a
coronary artery fistula, an aneurysm of the membranous
ventricular septum, or a PDA. A normal coronary origin
with normal coronary lumen favors the diagnosis of sinus
of Valsalva aneurysm. Its location in aortic sinus above
the plane of the origin of the coronaries distinguishes it
from aneurysm of the membranous ventricular septum.
In adults with acquired aneurysm following complicated
infective endocarditis, the fistula does not have an
extended finger-like aneurysmal extension from the base
to the apex as seen in the sinus of Valsalva aneurysm.

Echocardiography

chamber or vascular structure. The morphology can be

complex with single or multiple communications that may
form a maze of fine intramural channels communicating
with each other. It most commonly affects the right side of
the heart.65
The clinical presentation depends upon the size of the
shunt and the structures involved. Most coronary fistulae
drain into the right heart structures. Adults with a large
shunt may present with heart failure or angina due to
coronary steal phenomenon caused by diversion of blood
flow away from the territory supplied by the coronary,
into the site of drainage (right heart, pulmonary artery, or
superior vena cava).

Two-dimensional echocardiography shows an aneurysmal sac, which can present as an abnormal, circular, thinwalled structure protruding into a heart chamber. Ruptured
aneurysms will lead to chamber enlargement over time.
Color Doppler with transthoracic echocardiogram will
show flow originating in the aorta and flowing into a
heart chamber (usually the right ventricle) in a ruptured
aneurysm. Multiple views, starting with the parasternal
long-axis and short-axis views at the level of the aortic
root, are required to locate the sinus from which the
aneurysm originated. The transducer is then angulated to
obtain modified views and locate the aneurysm (usually
above the aortic cusps). Contrast echocardiography allows
more precise definition of the aneurysmal sac. It aids in
the diagnosis of a left-to-right shunt by demonstrating
a negative contrast image in the right chambers with a
ruptured aneurysm and no negative contrast image with
an unruptured aneurysm.64 TEE delineates the anatomical
details such as the site of origin by displaying the aortic
root and sinuses more clearly. Three-dimensional
echocardiography adds incremental value by further
defining the spatial relationships between the aneurysm
and the cavity into which it drains.
Associated echocardiographic findings include aortic
regurgitation resulting from altered aortic root anatomy.
However, if there is severe aortic regurgitation, one
must rule out extensive damage to the aortic leaflets
by endocarditis or acute rupture of sinus of Valsalva
aneurysm. Chronic small fistulae with a left-to-right shunt
cause left heart enlargement or right atrial enlargement
when the fistula drains into the right atrium.

Echocardiography

Coronary Artery Fistula

Fig. 75.13: Transthoracic echocardiogram (high parasternal

short-axis view) showing the site of drainage of a coronary artery
fistula. (LA: Left atrium; PA: Pulmonary artery; RVOT: Right ventricular outflow tract).

Coronary artery fistula is defined as an abnormal

communication between a coronary artery and a cardiac

On a transthoracic echocardiogram, a markedly enlarged

proximal part of the involved coronary artery alerts the
echocardiographer of its presence. The structure or the
cardiac chamber to which the coronary artery fistula
drains is also enlarged in those with significant longstanding shunts. The parasternal long-axis views with
color Doppler focusing on the aortic root may also bring
it to our attention. A high parasternal short-axis view
and its modifications help in identifying its origin and
site of drainage (Fig. 75.13). However, 2D transthoracic
echocardiography is limited in its ability to reliably trace
the course of the fistula as well as the site of drainage in
many adults.66

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

TEE offers better spatial resolution and delineates

the course of coronary artery fistula from its origin to
the chambers into which it is draining in multiplane
views.67
Three-dimensional TEE with its ability to provide
en face views has been reported to reveal morphology
and dimensions of the coronary artery fistulae more
comprehensively.68,69

Cardiac Catheterization
Cardiac catheterization is best tool for diagnosing and
tracing the entire course of the coronary artery. Coronary
angiography also allows accurate assessment of obstructive
coronary atherosclerosis is adults who are at higher
cardiovascular risk. While small coronary fistulae do not
require closure, the larger ones may require percutaneous
closure or surgery to avoid the risk for developing heart
failure or angina.

Magnetic Resonance Imaging/Computed

Tomography
MRI/CTA are the best noninvasive imaging modalities
for defining the course of the coronary arteries and the
enlargement of the chambers. The number of incidentally
detected coronary artery fistulae in the era of multidetector
CT scanning has increased.65

Surgery for Sinus of Valsalva Aneurysms and

Coronary Artery Fistulae
Surgical repair, ligation, bypass, or percutaneous closure
should be considered before the development of potential
complications such as cardiac failure, infectious endocarditis,
embolization, pulmonary hypertension, arrhythmia, or
ischemia related to myocardial hypoperfusion, aortic regurgitation, dissection, rupture, or death.70

The Postoperative Adult

Postoperatively, adults need follow-up echocardiography
to rule out residual patch leaks, compression of surrounding
structures, and resolution of chamber enlargement and
assessment of ventricular function.

1813

VALVULAR DISEASE
Tricuspid Valve
Ebsteins Anomaly
First described by Wilhelm Ebstein in 1866, this rare
cyanotic CHD occurs in <1% of CHD with varying degree
of apparent apical displacement of the point of attachment
of the basal insertions of the septal and posterior leaflets of
the tricuspid valve.71 Due to failure of delamination, there
is adherence of tricuspid valve leaflets to the underlying
myocardium. In addition, there is apical displacement
of the functional tricuspid valve annulus with the septal
leaflets more apically displaced than the posterior,
followed by the anterior leaflets.72
The malformed tricuspid valve may be incompetent,
stenotic, or rarely, imperforate. If the right ventricle is
subdivided into the inlet, trabecular, and outlet portions,
then the displacement of the tricuspid orifice to the
junction of the inlet and trabecular ventricular zones
describes the essence of this anomaly.73
Due to atrialization of the right ventricle, by the
downward displacement of the functional tricuspid
annulus, the right atrium appears larger than its actual size.
The right ventricle, therefore, appears to be smaller and
may have impaired function over time. The left ventricular
function may also decrease in adulthood. The downward
displacement of the septal tricuspid valve leaflet is
associated with discontinuity of the central fibrous body
and septal atrioventricular ring, thus creating a potential
substrate for accessory atrioventricular connections and
ventricular pre-excitation, making the patient at risk for
sudden death. Right heart catheterization, therefore,
carries a risk of inducing ventricular arrhythmias leading to
sudden cardiac death. On angiography, morphofunctional
abnormalities of the left ventricle have been demonstrated
in many patients, which may be explained by increased
fibrosis in the left ventricular wall and the ventricular
septum as demonstrated by histological studies.74
The anterior tricuspid leaflet may be fenestrated
and redundant, giving it a sail-like appearance. The
septal leaflet may be tethered. The severity of tricuspid
regurgitation, presence of a PFO, or ASD determines
clinical outcomes. This defect is often associated with
pulmonary stenosis/atresia and VSDs, congenitally
corrected transposition of the great arteries, and tetralogy
of Fallot.31

1814

Section 6: Congenital Heart Disease

The term Ebstenoid or Ebstein-like may be used to

describe the broad spectrum of variations in morphology
of the typical Ebsteins valve. Although it is usually
diagnosed in childhood, milder variations may be noted
on echocardiography in adulthood when the individual
presents with dyspnea, decreasing exercise tolerance,
right heart failure, and worsening atrial arrhythmias.
The common arrhythmias are atrial fibrillation/flutter or
supraventricular tachycardia due to WolffParkinson
White (WPW) syndrome in 15% to 20% adults. Cyanosis
occurs in the presence of an atrial shunt (PFO or ASD)
that may also increase the risk of paradoxical embolism
leading to transient ischemic attack or stroke.

Echocardiography
In the current era, Ebsteins anomaly is most commonly
diagnosed by echocardiography.72 On transthoracic
echocardiography, the Ebsteins tricuspid valve is best
seen in the apical four-chamber view with its characteristic
sail-like elongated anterior leaflet and the downward
displacement of the septal leaflet caused by the tethering
to the septum, making it appear as though it is attached
apically in relation to the point of attachment of the anterior
mitral leaflet (Fig. 75.14). The diagnostic feature of Ebsteins
anomaly is an apical displacement of both the septal
and the posterior tricuspid leaflets, exceeding 20 mm or
8 mm/m2 in adults.75 In an apical four-chamber view, this
distance is measured from the point of attachment of the

Fig. 75.14: Transthoracic echocardiogram showing Ebsteins

valve in an apical four-chamber view with its characteristic saillike elongated anterior leaflet and the downward displacement
of the septal leaflet. (LA: Left atrium; LV: Left ventricle; MV: Mitral
valve; RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).

anterior mitral valve leaflet to the point of attachment

of the septal tricuspid valve leaflet. This view also shows
severity of tricuspid regurgitation, its impact on the right
atrial enlargement, and biventricular ventricular function
(Fig. 75.15).
The parasternal short-axis view may also help define
the tricuspid valve anatomy. Tricuspid valve features
associated with decreased functional capacity are absence
of the septal leaflet, pronounced tethering, restricted
leaflet motion, and displacement of the anterior leaflet.76
Associated defects such as pulmonary stenosis and shunts
(PFO/ASD/VSD) are also to be followed-up on serial
echocardiograms. TEE with agitated saline contrast is
used to indentify a patent foramen ovale in adults with
poor acoustic windows. A synopsis of echocardiographic
assessment is reviewed in Table 75.10.

Stress Echocardiography
Stress echocardiography with treadmill testing offers an
objective assessment of functional capacity, ventricular
contractile reserve, impact of exercise on arrhythmias, and
cyanosis in adults with Ebsteins anomaly.

Magnetic Resonance Imaging/Computed

Tomography
MRI/CTA allows quantification of biventricular function
and assessment of tricuspid valve morphology in adults

Fig. 75.15: Transthoracic echocardiogram showing Ebsteins

valve in the apical four-chamber view associated with tricuspid
regurgitation. (LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1815

Table 75.10: Ebsteins Anomaly

Associated defects

Patent foramen ovale

Atrial septal defect

Ventricle septal defect

Patent ductus arteriosus (PDA)

Pulmonary stenosis or atresia

Congenitally corrected transposition of the great arteries (CCTGA)

Left ventricular noncompaction (LVNC)40%

Tetralogy of Fallot

Echocardiographic assessment

Identify the large sail-like anterior tricuspid leaflet

Degree of tricuspid valve displacement (septal leaflet attachment)

Right ventricular size and function

Degree of tricuspid regurgitation

Right atrial enlargementusually marked

Left ventricular size and function

Postoperative

Biventricular size and function

Degree of tricuspid regurgitation

Right atrial enlargement

Rule out residual shunts

with limited acoustic windows, who are unable to have

a TEE. MRI allows better visualization of the posterior
tricuspid valve leaflet, tricuspid valve fenestrations,
quantification of right heart size, and right ventricular
ejection fraction, while echocardiography is more
accurate in assessment of valve morphology and detecting
small shunt lesions. Echocardiography and MRI provide
complementary data for appropriate risk stratification
prior to surgery.72

Cardiac Catheterization
Coronary angiography is performed before surgical
intervention in patients at risk for coronary artery disease
or for assessment of pulmonary vasoreactivity in adults
with severe pulmonary hypertension.

Tricuspid valve repair or replacement with PFO/ASD

closure is performed in:
A symptomatic adult with right heart failure, declining
exercise capacity, or progressive right ventricular
dilation/impaired right ventricular systolic function
with worsening atrial and/or ventricular arrhythmias
Worsening cyanosis with oxygen saturation < 90%
Transient ischemic attack or stroke attributed to
paradoxical embolism
Increased cardiac silhouette on imaging.
At the time of surgery for the tricuspid valve and
associated defects, the Maze procedure is performed for
atrial arrhythmias, since catheter-based ablation is far
more challenging in adults with Ebsteins anomaly due
to multiple accessory pathways. For those with previous
tricuspid valve surgery, reoperation is indicated for
impaired valvular function.

Tricuspid Valve Surgery

The ACC/AHA Class I recommendations for surgical
treatment for adults with Ebsteins anomaly are the
following:3

The Postoperative Adult

The postoperative adult with Ebsteins anomaly needs follow-up for tricuspid regurgitation postrepair, assessment

1816

Section 6: Congenital Heart Disease

of prosthetic valve function, biventricular function, and

residual shunts.

Pulmonary Valve
Pulmonary Stenosis
While the majority of the individuals have narrowing of the
pulmonary valve at the valvular level, stenosis may occur
at the subvalvular or supravalvular levels. Among the three
morphological types, the commonest one is the domeshaped pulmonary valvea mobile valve with narrow central
opening and systolic doming seen on echocardiography.
It is usually associated with main pulmonary artery
dilatation that results from structural abnormalities.11 The
three rudimentary raphe fuse to cause valve stenosis that
may increase in severity due to calcification. Over time,
pulmonary regurgitation may develop and the jet usually
directed toward the left pulmonary artery. Other associated
defects are ASD, VSD, and infundibular or right ventricular
hypertrophy leading to dynamic obstruction of the right
ventricular outflow tract.31
Dysplastic pulmonary valve has significant myxomatous thickening of the valve leaflets that reduces leaflet
mobility. Associated commonly with Noonan syndrome,
it may also manifest with narrowing of the pulmonary
annulus and the right ventricular outflow tract.
Unicuspid or bicuspid pulmonary valve may rarely be
seen in adults with tetralogy of Fallot. The quadricuspid
pulmonary valve has been reported as a rare finding in
literature, with a rudimentary accessory cusp interposed
between three cusps of same size in most cases. It may
present with stenosis/regurgitation and pulmonary artery
dilatation/aneurysm. The most common CHDs associated
with it are ASD, VSD, PDA, and bicuspid aortic valve.78

Echocardiography
The era of echocardiographic detection of the pulmonary
valve was heralded by Dr Nanda who published his
findings in 1972.77
On 2D transthoracic echocardiography, the parasternal
short-axis view at the level of the aorta is the best view for
assessment of the pulmonary valve morphology and
assessment of regurgitation or stenosis. The parasternal
long-axis view displays the right ventricular outflow
tract. The level and severity of the stenoses are important
in determining the timing for intervention or surgery.
Standard grading ranges from mild (peak valvular gradient

< 30 mm Hg), moderate (peak valvular gradient between

30 and 50 mm Hg), and severe (peak valvular gradient >
50 mm Hg). Silvilairat et al. raised a possibility that the
mean Doppler gradient may be the preferred method for
accurately estimating the severity of the pulmonary valve
stenosis, since it correlates best with the peak-to-peak
gradient obtained by cardiac catheterization. According
to their study, estimation of only the peak (maximum)
valvular gradient may lead to overestimation of the severity
of pulmonary stenosis.78 Further studies are required to
figure out why exactly Doppler mean pressure gradients
and catheterization peak-to-peak pressure gradients seem
to show good correlation.79
Long-standing unoperated severe pulmonary stenosis
leads to right ventricular hypertrophy, and systolic
flattening of the interventricular septum due to increased
pressure overload on the right heart. Spectral Doppler
examination may reveal a dagger-shaped, late-peaking
systolic signal characteristic of significant dynamic
obstruction of the right ventricular outflow tract.
The right ventricular size, mass, and systolic function
along with pulmonary regurgitation and pulmonary artery
dimensions should be assessed on echocardiography. The
pulmonary artery may be considered as dilated when: (a)
a ratio of maximum pulmonary artery diameter (usually
measured at the point of bifurcation into right and left
branches) to the aortic diameter (measured at the level of
the aortic valve or 2 cm beyond the aortic valve) is 1.4; and
(b) a ratio of pulmonary ring to the aortic ring diameters
is 1.5.80 Nearly half the pulmonary artery aneurysms
are associated with CHD. Besides pulmonary valve
stenosis, they may occur in individuals with pulmonary
regurgitation, ASD, PDA, VSD, tetralogy of Fallot, and
Eisenmenger syndrome. The presence of a thrombus
should be ruled out.81
TEE is very important for examining the cusp anatomy
of the pulmonary valve in the short-axis view. The best
view to visualize this valve is a modified view, with the
TEE probe anteroflexed between 135 and 145, so that
it aligns the pulmonary valve into the short axis and the
aortic valve into long axis.78 In individuals with limited
transthoracic windows, TEE also helps in delineating the
right ventricular outflow tract and lesions in the branch
pulmonary arteries.
A synopsis of echocardiographic assessment is
reviewed in Table 75.11.

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1817

Table 75.11: Pulmonary Stenosis

Associated defects

Patent foramen ovale

Atrial septal defect

Ventricular septal defect

Peripheral pulmonary stenosis

Patent ductus arteriosus

Echocardiographic assessment

Type of valvedome-shaped, dysplastic or quadricuspid

Valve annulus

Thickness of the leaflets

Gradientdegree of stenosis

Concomitant pulmonary regurgitation

Pulmonary artery dimensionsdilatation, aneurysm or narrowing

Subvalvular areainfundibular obstruction/hypertrophy

Right ventricular size and function

Postoperative

Valve gradientstenosis

Concomitant pulmonary regurgitation

Pulmonary artery dimensionsdilatation, aneurysm or narrowing

Cardiac Catheterization
Cardiac catheterization is rarely necessary for diagnosis
and is usually performed when catheter-based intervention
is indicated. For confirmation, the gradients should be
obtained at the valvular, sub-, and supravalvular levels.
The presence of obstruction in the infundibulum as well
as main, branch, or peripheral pulmonary arteries should
be ruled out. It is important to note again that the peakto-peak gradient by cardiac catheterization correlates
best with the mean Doppler rather than with the peak
instantaneous Doppler gradient.78 Right ventriculogram is
performed for assessment of the right ventricular function
and degree of pulmonary regurgitation.

Magnetic Resonance Imaging/Computed

Tomography
MRI/CTA are indicated for accurate imaging of the main,
branch, and peripheral pulmonary arteries, quantification
of right ventricular systolic function and degree of pulmonary regurgitation.

Pulmonary Valve Intervention (Valvotomy)

and Surgery (Replacement)
The Class I ACC/AHA recommendations for interventional
and surgical treatment for adults with valvular pulmonary
stenosis are the following:3
Catheter-based intervention with balloon valvotomy in:
Asymptomatic adults with a domed pulmonary valve
with a mean Doppler gradient > 40 mm Hg (peak
instantaneous Doppler gradient > 60 mm Hg) if the
degree of pulmonic regurgitation is less than moderate
Symptomatic adults with a domed pulmonary valve
with a mean Doppler gradient > 30 mm Hg (a peak
instantaneous Doppler gradient > 50 mm Hg) if the
degree of pulmonic valve regurgitation is less than
moderate.
Surgery is performed for the following indications:
Adults with severe pulmonary stenosis and associated
defects such ASD, severe pulmonary regurgitation,
or stenosis at other levels (subvalvular/supravalvular
pulmonary stenosis or hypoplastic pulmonary
annulus)

1818

Section 6: Congenital Heart Disease

Dysplastic thickened pulmonary valve

Concomitant surgery is indicated for associated lesions
such as severe tricuspid regurgitation, a surgical Maze
for atrial fibrillation, or when the main pulmonary
artery is markedly dilated (usually over 4 cm) and is
causing symptoms by compressing on contiguous
structures.
Of note, there are no clear cut guidelines for surgery
in an adult who has a dilated main pulmonary artery
that is associated with a dome-shaped pulmonary
valve. There is less likelihood of rupture of these lowpressure aneurysms. Hence, pulmonary arterioplasty or
main pulmonary artery replacement with a tube graft/
valved tube graft is performed when there are significant
symptoms due to compression on contiguous structures
or when concomitant surgery is otherwise indicated.
Bioprosthetic pulmonary valves are chosen because,
despite anticoagulation, the low pulmonary artery
pressure and slow blood flow puts these individuals at a
high risk of valve thrombosis with mechanical prosthetic
valves.

The Postoperative Adult

Besides the assessment of native/prosthetic valve
function, the main pulmonary artery should be evaluated
for dilatation. On long-term follow-up after surgery,
increasing severity of pulmonary regurgitation has been
reported. Following percutaneous balloon angioplasty,
reinterventions may be required for recurrence of
pulmonary stenosis, and mild pulmonary regurgitation is
common.82

Mitral Valve
Cor Triatriatum
Also known as cor triatriatum sinister, this is a very rare
cardiac defect that may go undiagnosed into adulthood
especially when it presents as an isolated anomaly. It
is characterized by a perforated membrane (partial or
complete) dividing the left atrium into two chambers
the proximal (pulmonary venous chamber) receives the
pulmonary veins, and the distal true chamber comprises
the left atrium with the fossa ovalis and left atrial
appendage.31 Associated defects include patent foramen
ovale, secundum ASD, and rarely a common atrium.
Adults usually present with symptoms similar to mitral
stenosis due to obstruction to flow through the perforated

membrane because of fibrosis or calcification. Others

may develop atrial fibrillation or mitral regurgitation.
The differential diagnosis includes a supravalvular mitral
ring or left atrial dissection. Hemodynamically, it may
mimic pulmonary vein stenosis or left atrial mass (tumor,
thrombus, or cyst).8385

Echocardiography
The best view is the apical four-chamber view that shows
a thin undulating membrane moving toward the mitral
valve in diastole and away from it in systole. The proximal
chamber is usually dilated due to a back up of flow
caused by obstruction through the perforated membrane
into the low pressure distal chamber. The left atrial
appendage is usually normal in size. Color flow Doppler
shows flow acceleration at the sites of perforations in the
membrane. The right ventricular systolic pressure should
be estimated from the tricuspid regurgitant jet to rule out
pulmonary hypertension.
It is important to establish the position of the left atrial
appendage in the distal chamber in order to confirm cor
triatriatum and exclude a supravalvular mitral ring.86
Transesophageal echocardiography is useful in
confirming the diagnosis by clearly delineating the
position of the left atrial appendage. It also allows complete
assessment of any associated intracardiac defects such as
the patent foramen ovale.83 Contrast echocardiography is
an important adjuvant technique where the perforation in
the membrane cannot be delineated by color Doppler. It
shows a differential opacification of the proximal versus
the distal chamber with delayed emptying of contrast into
the distal chamber through the communication between
the chambers.85 Three-dimensional reconstruction is
useful in diagnosing cor triatriatum by defining atrial
membranes and the associated defects.87
Although these echocardiographic modalities may
suffice, in some cases a left and right heart catheterization
with simultaneous pulmonary capillary wedge and left
ventricular end-diastolic pressure measurements may be
required to reveal a significant mean gradient, prior to
definitive surgical resection of the membrane.

Cleft Mitral Valve

A cleft mitral valve can occur as an isolated defect or
in association with other CHD. The cleft can be in the
anterior leaflet usually in association with a primum ASD
or may occur in the posterior leaflet.88,89 Other defects

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

associated with a cleft mitral valve are atrioventricular

septal defect, secundum ASD, VSD, coarctation of aorta,
double outlet right ventricle, and tricuspid atresia. The
long-term sequelae are left atrial enlargement and left
ventricular enlargement with impaired function due to
progressive mitral regurgitation. These adults are also at
risk for developing endocarditis, atrial arrhythmias, and
pulmonary hypertension.
It is best seen in the parasternal short-axis view
(Fig. 75.12). The direction of mitral regurgitation jet and
its severity can be assessed based on the data compiled
from the parasternal long-axis, apical two-chamber, fourchamber, and subcostal views. The 3D echocardiography
en face view shows the location of the cleft in relation to the
scallops of the mitral leaflet most clearly.90
The indications for surgery are similar to those for
acquired mitral regurgitation. Surgical treatment involves
reconstruction with ring annuloplasty or mitral valve
replacement.

1819

coarctation of aorta, VSD, and subaortic stenosis. It may

occur in association with more complex defects such as
Shones syndrome, transposition of the great arteries, truncus
arteriosus, tricuspid atresia, Ebsteins anomaly, and double
outlet right ventricle.91
The clinical presentation of DOMV is variable and
may go undiagnosed until adulthood unless there
are significant mitral regurgitation, mitral stenosis, or
associated cardiac defects. Mitral regurgitation appears to
occur more frequently than mitral stenosis.

Echocardiography

In this rare congenital malformation, abnormal fusion of the

endocardial cushion leads to two equal or unequal mitral
valve orifices. Double orifice mitral valve (DOMV) with
intact atrioventricular septum can present as reduplication
of the orifice caused by two separate valve orifices supported
by their own separate subvalvular apparatus. On the other
hand, it can occur in association with an atrioventricular (AV)
septal defect with a fibrous bridge across the mitral orifice.
There may be abnormal papillary muscle rotation, fusion,
or a single papillary muscle. Besides partial or complete
AVSD, other associated defects include atrial septal defect,

It is usually best seen in the parasternal short-axis view.

Other views are the subcostal short-axis view at the level
of the mitral valve and the apical four-chamber view.
Das et al. described the transthoracic echocardiographic
views that help in determining various morphology of
the DOMV; for the DOMV with complete bridging, the
best views are the parasternal or subcostal short-axis
with a sweeping of the transducer from base to apex; for
DOMV with incomplete bridging, a single opening of the
mitral valve appears on basal cuts in the short-axis views
and then the double orifice becomes apparent only when
the transducer is carefully swept toward the apex; for the
hole-type DOMV, an apical four-chamber is the preferred
view. In their study, they reported that a smaller, accessory
orifice was located in the anterior leaflet of the mitral valve
in all cases.92
The mitral valve may rarely be normal functioning
and more often be stenotic or regurgitant. The mitral valve
area is calculated as the sum of the area of the two mitral
orifices (Figs 75.16A and B). Isolated DOMV with normally

Double Orifice Mitral Valve

Figs 75.16A and B: Transthoracic echocardiogram showing that the mitral valve area in an adult with a double orifice mitral valve (DOMV) is
calculated as the sum of the area of the two separate mitral orifices (A and B), in the parasternal short-axis views by planimetry.

1820

Section 6: Congenital Heart Disease

functioning valves may go unrecognized until later in

life and then only discovered as an incidental finding.93
DOMVs presenting in association with other defects, such
as atrioventricular (AV) septal defect, VSD, ASD, Ebsteins
anomaly, tetralogy of Fallot, left heart obstructive lesions,
and coarctation of aorta, are more likely to be diagnosed in
childhood and undergo surgical repair or replacement of
the mitral valve.

Parachute Mitral Valve

The parachute mitral valve is a variant of abnormal
attachment of the mitral valve chordae to a solitary or fused
papillary muscle. This can result in mitral stenosis due to
a restrictive valve opening, as subvalvular obstruction
due to fused chordae, or it can rarely present with mitral
regurgitation because of prolapsed mitral valve. It can
also present as an isolated anomaly or part of the Shones
complex, which is characterized by a supravalvular
membrane or ring, parachute mitral valve, subaortic
stenosis, and coarctation of aorta.94 It may occur with
other lesions such as an ASD or bicuspid aortic valve with
stenosis. Most cases present in infancy and are operated
early.95,96 In rare cases, parachute mitral valve with
Shones complex may go undiagnosed into adulthood.97
The majority present with dyspnea, decreasing exercise
tolerance, or atrial fibrillation due to stenosis or
regurgitation, while in others the finding is incidental.

Transesophageal Echocardiography
TEE offers a more detailed analysis of the defect and
isolated defects in multiplane views. It often allows
differentiation between a true parachute and parachutelike mitral valve. In a parachute-like mitral valve, most
of the chordae tendinae are attached to a main papillary
muscle while the other papillary muscle is hypoplastic and
close to the main one.98 TEE should also be considered
when the severity of stenosis or regurgitation indicates
surgery in accordance with the ACC/AHA guidelines for
valvular disease.99
The ACC/AHA Class I recommendations for surgical
treatment for young adults with mitral valve disease are
the following:99
Congenital Mitral Stenosis:
Symptomatic young adult patients with congenital
mitral stenosis and NYHA functional Class III or IV,
who have a mean mitral valve gradient > 10 mm Hg on
Doppler echocardiography.
Other Class I indications that apply to all mitral valve
disorders include:
Symptomatic adults with NYHA functional Class
IIIIV, who have moderate or severe mitral stenosis
measured by other methods when percutaneous
mitral balloon valvotomy is either not available;
contraindicated because of concomitant moderate
to severe mitral regurgitation; persistent left atrial
thrombus despite anticoagulation; or unfavorable
mitral valve morphology.

Echocardiography
The transthoracic parasternal long-axis and shortaxis views (at the level of the papillary muscle), apical
four-chamber and two-chamber views are valuable
in assessment of the morphology of the mitral valve,
subvalvular apparatus, papillary muscle, and associated
left heart lesions. The parasternal long-axis view may show
chordae tendinae converging to the single papillary muscle
either as short, thickened, underdeveloped, and adherent
causing stenosis, versus elongated and floppy without
adequate coaptation of the mitral valve leaflets/prolapse
resulting in mitral regurgitation. In the parasternal shortaxis view at the midpapillary level, a single papillary is
visualized posteromedially and the mitral orifice may be
eccentric. In the parasternal short-axis view at the basal
level, parachute leaflets described as typical for this
condition are often seen.

Mitral Regurgitation
Mitral valve repair is preferred over MV replacement in
most cases:
Symptomatic adult with severe congenital mitral
regurgitation with NYHA functional Class III or IV
symptoms
Asymptomatic adult with severe congenital mitral
regurgitation and left ventricular ejection fraction
60%
Severe mitral regurgitation with NYHA functional
Class II, III, or IV symptoms in the absence of severely
reduced left ventricular function (ejection fraction
< 30%) and/or end-systolic dimension > 55 mm
Asymptomatic adult with chronic severe MR with
mildly to moderately reduced left ventricular function
(ejection fraction between 30% and 60%, and/or endsystolic dimension 40 mm).

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

Aortic Valve
Bicuspid Aortic Valve
Biscuspid aortic valve (BAV) is defined by the presence of
complete or partial fusion of two aortic leaflets, often manifesting as right and left leaflet fusion, otherwise as right
and noncoronary cusp fusion, or as left and noncoronary
cusp fusion.31 While right and left leaflet fusion is more
often associated with stenosis/regurgitation requiring
early intervention, the right leaflet and noncoronary
cusp fusion is more likely to be associated with aortic
root dilatation.100102 The cusp size may be unequal due to
fusion of two cusps leading to one larger cusp. A central
raphe may divide the larger of the two cusps at the site of
congenital fusion of two parts of the conjoined cusps.
Although many adults are previously diagnosed with a
bicuspid aortic valve because of a heart murmur, in others
the diagnosis is first made when they acquire endocarditis
and present with acute severe aortic regurgitation due
to aortic leaflet destruction or perforation, leading to
heart failure. Secondary infection of the mitral valve due
to contiguous spread of the infection is known to occur.
Destruction of the infected valves causes regurgitation
and the vegetations increase the risk of embolic stroke.
Sepsis is common in this scenario. Siniawski et al reported
high mortality (approximately 29%) in adults who have
endocarditis with aortic ring abscess and secondary
infection of the mitral valve requiring double valve surgery.
According to their study, the most potent independent risk
factors for mortality were septic shock and severe aortic
root destruction.103
First-degree relatives of patients with bicuspid
aortic valve should be screened by echocardiography
for the presence of a bicuspid aortic valve, since familial
reoccurrence of bicuspid aortic valve is approximately 9%,
mostly likely due to an autosomal dominant pattern of
inheritance with reduced penetrance.104
Severe calcification or regurgitation can be present in
third or fourth decade, often accelerated by risk factors
such as hyperlipidemia. The calcification and fibrosis
primarily occurs at the raphe and base of the cusps.105
Aortic regurgitation may result from inability of the
leaflets to coapt due to aortic root dilatation and loss of the
sinotubular junction, retraction of the leaflets caused by
fibrosis, prolapse of aortic cusps, aortic valve attempting
to close a perimembranous VSD (venturi effect), or
following endocarditis due to extensive valve destruction.

1821

Aortic root dilatation is commonly seen in adults with

bicuspid aortic valve and they are at least a ninefold greater
risk for aortic dissection.106

Echocardiography
In the transthoracic parasternal long-axis view, doming
of the bicuspid aortic valve leaflets in systole can be
appreciated in most young adults who are unlikely to
have distorted dysplastic valve or restricted mobility due
to calcification of the valve. The parasternal short-axis
view (at the aortic valve level), is the best view for defining
valve anatomy by showing fusion between two cusps (one
smaller and one larger) or a variation of it. The extent of
commissural fusion determines degree of stenosis or
obstruction. In this view, the open bicuspid aortic valve
appears to look like an elliptical American football or
fish-mouth opening (Fig. 75.17).
Two-dimensional echocardiography and continuous
wave Doppler are routinely used to determine the severity
of valve stenosis using the same criteria as in the general
population, defined by the ACC/AHA valvular heart disease
guidelines.99 The peak aortic velocity should be assessed
by continuous wave by Doppler echocardiography in
multiple views and the highest values should be recorded
(usually best captured in the suprasternal views). The peak
and mean gradients are derived accordingly. Since the

Fig. 75.17: Diagrammatic representation of the echocardiographic

parasternal short-axis views showing morphological features of
the (1) closed normal trileaflet (Mercedes Benz sign) aortic valve
as seen on a transesophageal echocardiogram, and (2) an open
bicuspid (American foot ball), (3) open unicuspid (tear drop) (a)
unicommissural, (b) acommissural (4) a closed quadricuspid (X-sign)
aortic valve as seen on transthoracic parasternal short-axis views.

1822

Section 6: Congenital Heart Disease

peak instantaneous aortic valve gradient may overestimate

the severity of stenosis, the mean gradient is preferred.
It correlates more closely with the peak-to-peak gradient
traditionally measured during cardiac catheterization. The
more universally accepted grading system is the following:
mildvalve area > 1.5 cm2, mean gradient < 25 mm Hg,
or jet velocity < 3.0 m/s; moderatevalve area 1.01.5 cm2,
mean gradient 2540 mm Hg, or jet velocity 3.04.0 m/s; or
severevalve area < 1.0 cm2, mean gradient > 40 mm Hg, or
jet velocity > 4.0 m/s.
Aortic regurgitation is quantified as mild, moderate,
or severe, according to the American Society of
Echocardiography criteria using standard methods as
in acquired valve disease.107 Aortic regurgitation may be
evaluated by color Doppler in the parasternal long-axis,
short-axis, apical four-chamber, and five-chamber views.
The rate of deceleration of the velocity signal of aortic
regurgitation, the presence of retrograde flow (diastolic
flow reversal) in the proximal descending/abdominal
aorta on continuous wave Doppler echocardiography,
and the ratio of proximal jet area to left ventricular outflow
tract area in combination with the ratio of jet height to left
ventricular outflow tract height allow assessment of the
severity of aortic regurgitation.
The left ventricular size, function, and mass are
assessed on serial echocardiograms and are important
criteria in determining the need for surgery and long-term
clinical outcomes. Associated defects include coarctation
of the aorta, VSD, PDA, subaortic stenosis, and parachute
mitral valve.31
Intrinsic structural abnormalities of the aortic wall
can lead to aortic root dilatation that can in turn progress
to an aneurysm that carries risk of dissection five- to
ninefold higher than in the general population.106 Other
contributory factors for increase in aortic diameters are
worsening aortic regurgitation, high body surface area, and
advancing age.108,109 In the parasternal long-axis view, the
aortic root should be routinely measured in systole, from
leading edge to leading edge, at the levels of the annulus,
midsinus level, sinotubular junction, and the proximal
aorta.108 It is important to report these measurements in
all the cases, since progressive aortic dilatation may occur
in this population even in the absence of significant aortic
stenosis and regurgitation. Depending upon the size of
the aorta, imaging should be performed every other year
if the maximum dimensions are < 40 mm, and performed
annually if they are 40 mm. Unfortunately, replacement
of the bicuspid aortic valve has not been shown to reduce
the risk of further dilatation.

Three-dimensional echocardiography is useful in

defining the morphology of the aortic valve. In adults
with good quality transthoracic images, it may define
thickening or redundancy of the aortic valve leaflets with
multiple folds. It also helps in identifying vegetations and
localizing perforations.110
Another variant of congenital aortic valve defects is the
unicuspid aortic valve, described as a single commissure
with two poorly developed cusps. The short-axis view of the
aortic valve clinches the diagnosis on TTE or TEE. During
diastole, the raphae may look like true commissures giving
it the Mercedes Benz sign appearance of a trileaflet
aortic valve. During systole, there is no cusp separation,
and the unicuspid valve looks like a teardrop with an
eccentric opening (Fig. 75.17). The more common type of
unicuspid aortic valve is unicommissural and the other
one, acommissural (with a lateral attachment to the aorta
at the level of the orifice). The origin of the coronaries
should be normal due to normal development of the
sinuses of Valsalva in these individuals.111,112
In even rarer cases, there is development of an
extra commissure during valvulogenesis, resulting
in a quadricuspid aortic valve. On transthoracic
echocardiography and more confidently with a TEE, it is
best identified in the short-axis view at the level of the aortic
valve during diastole, when it appears like an X sign (Fig.
75.17). The 3D echocardiogram defines the anatomy of the
four cusps more clearly.113
Like the BAV, all other congenital malformations of the
aortic valve are prone to an increased rate of calcification and
fibrosis leading to significant stenosis and/or regurgitation,
thereby requiring aortic valve replacement.114 They are also
associated with aortic root dilation in majority of the cases.
The risk of aortic dissection is 18-fold more in individuals
with a unicuspid aortic valve.106
A synopsis of echocardiographic assessment of the
bicuspid aortic valve is reviewed in Table 75.12.

Cardiac Catheterization
Cardiac catheterization is recommended for assessment
of coronary arteries before aortic valve surgery in
adults who are at risk for coronary artery disease. It is
also recommended when a pulmonary autograft (Ross
operation) is being considered, so that the origin of the
coronary arteries can be defined in case CTA/MRI are not
being performed. It is of incremental value when there is

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1823

Table 75.12: Bicuspid Aortic Valve

Associated defects

Coarctation of aorta

Aortic root dilatation/aneurysm or dissection

Subaortic and supravalvular (aortic) stenosis

Ventricular septal defect

Echocardiographic assessment

Type of valvefusion of raphe

Valve annulus

Thickness of the leaflets

Gradientdegree of stenosis

Concomitant aortic regurgitation

Aortic root dimensionsdilatation or aneurysm

Subaortic obstruction/hypertrophyleft ventricular outflow tract gradient

Left ventricular size mass and function

Postoperative

Assessment of valve gradient

Grading aortic valve regurgitation/paravalvular leak

Aortic root dimensions to rule out dilatation/aneurysm or dissection

a discrepancy between symptoms and echocardiographic

findings.
Most adults will require aortic valve replacement.
Catheter-based percutaneous balloon valvuloplasty is a
possibility in a young adult with an isolated severe aortic
stenosis and noncalcified pliable valves, or it may offer
temporary relief in a markedly symptomatic pregnant
woman with severe stenosis refractory to medical
management during late second or third trimesters.
The ACC/AHA Class I indications for aortic valvotomy
in adolescents and young adults are the following:99
Symptoms such as angina, syncope, or dyspnea on
exertion with a left ventricular peak-to-peak gradient
across the aortic valve of 50 mm Hg or higher in
the absence of heavy calcification, on cardiac
catheterization
Asymptomatic patient with a left ventricular peak-topeak gradient across the aortic valve > 60 mm Hg on
cardiac catheterization
Asymptomatic patient with ECG changes (ST or
T-wave changes over the left precordium) at rest or
with exercise and left ventricular peak-to-aortic valve
gradient > 50 mm Hg.

Magnetic Resonance Imaging/Computed

Tomography
The aortic root, arch, and the descending thoracic
aorta should be evaluated by either of these imaging
modalities prior to aortic valve replacement or when
echocardiography is suspicious for an enlarging aneurysm.

Stress Testing
Although stress testing with exercise may be performed
cautiously to assess functional capacity and blood
pressure response in asymptomatic adults who do not
have critical stenosis, it is contraindicated in symptomatic
adults, in those with repolarization abnormalities on ECG,
or systolic dysfunction on echocardiography.
Dobutamine stress testing can be performed
judiciously in adults with low-gradient aortic stenosis in
the setting of low left ventricular ejection fraction, since
the low cardiac output may mask a higher grade of
stenosis.
The ACC/AHA Class I indications for aortic valve
replacement are the following:3

1824

Section 6: Congenital Heart Disease

Aortic Stenosis:
Severe aortic stenosis or chronic severe regurgitation
in an adult undergoing coronary artery bypass graft
surgery or any other cardiac surgery of the aorta or
concomitant defect
Severe aortic stenosis with left ventricular function
< 50%
Symptomatic severe aortic regurgitation with left
ventricular ejection fraction < 50% and left ventricular
dilatation
Aortic root surgery is indicated when the ascending
aorta diameter is 5.0 cm or when it is increasing by
5 mm or more per year.
Aortic Regurgitation:
Symptomatic (angina, syncope, or dyspnea on
exertion) chronic severe aortic regurgitation
Asymptomatic adult with chronic severe aortic
regurgitation with reduced systolic function (ejection
fraction < 50% confirmed on two echocardiograms
(13 months apart)
Asymptomatic chronic severe aortic regurgitation with
progressive left ventricular enlargement (end-diastolic
dimension > 4 standard deviations above normal)
should receive aortic valve repair or replacement.

The Postoperative Adult

Echocardiography is useful for follow-up of prosthetic
valve function, left ventricular size and function, aortic root
dimensions, and for estimating right ventricular systolic
pressures postoperatively. The aortic root dimensions need
long-term follow-up, since significant enlargement of the
ascending aorta with aneurysm formation and dissection
continues even after valve replacement.104 When aortic root
replacement with coronary reimplantation is performed,
there is a risk of coronary ostial obstruction. Potential
prosthetic valve complications include periprosthetic
regurgitation with or without hemolysis, obstruction
related to pannus or thrombosis, and endocarditis.

Subaortic Stenosis
Subaortic stenosis is a congenital obstruction below the
aortic valve that may present as a discrete shelf-like
fibrous ring or as a fibromuscular tunnel-type defect
causing a fixed left ventricular outflow tract obstruction
below the aortic valve.31

Although it usually presents as an isolated defect, the

subaortic fibrous ring may extend onto the anterior mitral
leaflet, accessory mitral tissue, or anomalous chords
causing subaortic aortic stenosis. It is often associated
with a perimembranous VSD or an atrioventricular
ventricular septal defect. Also, it may become more visible
after ventricular septal patch closure.
Long-term residua and sequelae include progressive
aortic valve damage and aortic regurgitation due to
increased turbulence in the subaortic left ventricular
outflow tract, in more than half the cases. Increasing
ventricular hypertrophy due to pressure overload
eventually leads to impaired left ventricular function.
These adults are also at risk for infective endocarditis and
sudden cardiac death.115,116

Echocardiography
Subaortic stenosis is best seen in the parasternal long-axis
view that delineates the left ventricular out flow tract with
the transducer positions perpendicular to the membrane.
The left ventricular outflow tract (LVOT) obstruction
is examined carefully with color flow Doppler, since
the obstruction may present at multiple levels. Aortic
regurgitation may occur due to long-standing subaortic
flow disturbance.
In most adults, 2D transthoracic echocardiography
defines the anatomy of the subaortic stenosis, the
dimensions of the ascending aorta, adjacent mitral valve
involvement, degree of left ventricular hypertrophy, and
function (both systolic and diastolic). Sometimes a thin,
wispy, discrete fibrous subaortic ring may not be clearly
visible on transthoracic echocardiography. Doppler helps
in estimating the degree of aortic valve regurgitation. The
severity of the gradient across the left ventricular outflow
tract and the aortic valve can be determined by continuous
wave Doppler (Fig. 75.18).
The location of a VSD has an impact in the estimating
of the severity of subaortic stenosis. The degree of
subaortic stenosis may be underestimated when the VSD
is proximal to subaortic obstruction and overestimated
when it is distal to it. As in the case of aortic stenosis,
reduced left ventricular systolic function will also lead
to underestimation of the gradient. Among the factors
affecting progression of the left ventricular outflow tract
obstruction are the position of the membrane adjacent to
the aortic valve and whether it extends toward the mitral
valve.

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1825

End-hole or micromanometer-tipped catheters are

required for confirming the left ventricular outflow tract
gradient accurately. Timely surgery is important, since it
may be able to reduce the progression of left ventricular
hypertrophy and aortic regurgitation.

Surgical Resection of Subaortic Stenosis

Fig. 75.18: Continuous wave Doppler showing the severity of

the gradient across the left ventricular outflow tract with Valsalva
maneuver in a patient with subaortic stenosis.

Transesophageal Echocardiography
TEE is useful preoperatively and intraoperatively, in adults
with limited acoustic windows, to define the morphology
of the subaortic stenosis, and assess associated defects.
Intraoperative TEE evaluates adequate resection of the
subaortic membrane or ridge and rules out residual aortic
regurgitation. It also confirms the absence of iatrogenic
damage to the anterior mitral valve leaflet or creation of a
VSD by aggressive resection of the membrane. Similarly,
3D echocardiography may further define left ventricular
outflow anatomy.

Exercise Stress Testing

Stress echocardiography is used to determine any increase
in gradient with exercise in symptomatic adults with a
peak gradient < 50 mm Hg.

Magnetic Resonance Imaging/Computed

Tomography
In adults with limited acoustic windows who are unable to
undergo a TEE, an MRI or CTA plays an important role in
defining the anatomy and associated defects.

Cardiac Catheterization
Preoperatively, cardiac catheterization is performed in
adults who are at high risk for coronary artery disease.

The ACC/AHA Class I indications for surgical intervention

in adults with subaortic stenosis are the following:3
Severe obstruction with peak instantaneous gradient
> 50 mm Hg or a mean gradient > 30 mm Hg
Progressive aortic regurgitation with a left ventricular
end-systolic diameter of 50 mm or more or a left
ventricular ejection fraction < 55%.

The Postoperative Adult

The left ventricular outflow tract should to be assessed
with 2D transthoracic echocardiography and Doppler in
the parasternal long-axis and apical four-chamber views,
to rule out recurrence of subaortic stenosis or progression
of aortic regurgitation. The postoperative residua and
sequelae in an adult with discrete shelf-like fibrous ring
are iatrogenic VSD, aortic and mitral regurgitation due to
extensive resection of interventricular septum, and injury
to the mitral valve. Pacemaker wires may be seen in the
right heart chambers of those who suffered a conduction
system injury during surgery.

Supravalvular Aortic Stenosis

In adults, supravalvular stenosis may present with
hypertension or symptoms suggestive of ischemia. In this
defect, there is a fixed obstruction extending from below
the sinotubular junction distally into the aortic root. Even
though the obstruction usually occurs distal to the origin
of the coronary arteries, there may be ischemia due to
limited diastolic flow in adults with high systolic pressures
that also cause left ventricular hypertrophy. In some
adults, there may be coronary ostial obstruction (partial
or complete) or abnormalities of the coronaries such as
ectasia.117
In children with Williams syndrome, supravalvular
stenosis is the most common congenital heart defect.
Associated defects include peripheral pulmonary artery
stenosis and hypoplastic aorta.31

1826

Section 6: Congenital Heart Disease

Echocardiography
The left ventricular size, mass, systolic, and diastolic
function should be assessed on serial echocardiograms in
adults with supravalvular aortic stenosis. In most adults, the
parasternal long-axis view demonstrates the morphology
of the aortic root and allows measurement of the diameter
at the levels of the annulus, midsinus, sinotubular junction,
and the ascending aorta 2 cm distal to the sinotubular
junction. In others, the tubular portion of the aorta is not
well visualized and the best views are the suprasternal or
high right parasternal views. Increased continuous wave
Doppler gradient across an anatomically normal aortic
valve should alert the echocardiographer about a possible
obstruction. Pressure recovery phenomenon may be seen
in adults with long-segment or tubular stenosis.
Transesophageal echocardiography is useful in
obtaining this information in adults with limited acoustic
windows on transthoracic echocardiography. It also
defines the origins of the coronary arteries.

Stress Echocardiography
In adults presenting with symptoms and signs suggestive
of ischemia, stress echocardiography can be performed
to assess for coronary involvement and evaluation of the
gradient across the stenosis before and after exercise.

Magnetic Resonance Imaging/Computed

Tomography
Extracardiac structuresaorta, pulmonary artery, and
its branches, coronaries as well as the left ventricular
outflow tract are best delineated by MRI/CTA. In Williams
syndrome, the entire aorta and the renal arteries should be
imaged to look for peripheral stenosis.

Cardiac Catheterization
For definitive diagnosis of coronary involvement and for
accurately measuring the gradient across the supravalvular
stenosis, angiography is the preferred diagnostic imaging
modality.

Surgery for Supravalvular Stenosis

The ACC/AHA Class I recommendations for surgical
treatment for adults with supravalvular stenosis are the
following:

Symptomatic supravalvular stenosis with Doppler

echocardiography demonstrating a peak (instantaneous) gradient > 70 mm Hg and a mean gradient
over 50 mm Hg
Significant symptoms such as angina, dyspnea, or
syncope in the setting of left ventricular hypertrophy
and/or impaired left ventricular function.

The Postoperative Adult

Long-term follow-up is required to assess left ventricular
outflow tract obstruction, left ventricular mass/function,
and aortic and mitral regurgitation. There is a lifelong risk
of coronary involvement, left ventricular hypertrophy, and
diastolic dysfunction due to hypertension. Adults with
previous patch repair of the hypoplastic aorta are at risk of
aneurysm formation at the site of patch repair.

COMPLEX CONGENITAL HEART

DEFECTS
Complex defects are those that usually present as a group
of anomalies centered around a major condition.

Coarctation of Aorta
Coarctation of aorta (COA) is discrete or segmental
narrowing of the aorta below the origin of the left
subclavian artery, at the junction of the distal aortic arch,
and the descending aorta (in most cases) or its variation.31
It is appears more likely to be a diffuse arteriopathy with
associated structural abnormalities of the great arterial
walls that are not just limited to focal stenosis. Hence,
it is no longer considered as a simple defect.118 The
commonest associated defects are a bicuspid aortic valve
followed by mitral valve disease. These individuals are also
prone to aortic root dilatation.
Prior to the era of surgeries or interventions, the
survival was only 50% by 32 years of age.119 While many
individuals are operated in childhood in the present
era, some are still diagnosed in adulthood during an
evaluation for secondary hypertension.120 Despite relief of
stenosis by surgery or catheter-based intervention, adults
have long-term residua, sequelae, increased morbidity,
and reduced life spans. The most common issue is
ambulatory hypertension and significant left ventricular
hypertrophy seen on an ECG or an echocardiogram.
Despite successful COA repair, recurrence at the site of

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

stenosis (recoarctation) or aneurysmal dilatation at the site

of repair are not uncommon. If left uncorrected, these are
associated with high morbidity and mortality. Aneurysms
are most often seen at the site of coarctation repair but
may involve the descending thoracic aorta and the distal
aortic arch.121 Predictors of aneurysmal formation at the
site of coarctation repair include the use of the patch
graft technique and late correction of coarctation. Endto-end repair of COA is associated with the lowest risk of
aneurysmal formation at the site of repair.
Adults with a bicuspid aortic valve who have high
preoperative pressure gradients are at higher risk of
having an aneurysm of the ascending aorta, especially
following late repair of COA. Aortic root aneurysms are
also associated with morbidity and mortality due to a high
prevalence of severe aortic regurgitation, dissection, and
rupture.122 Another site for aneurysm rupture is in the
circle of Willis. Adults with COA have been noted to have
a higher prevalence of premature coronary artery disease.
A recent study points out that COA lesion alone does
not predispose to premature coronary artery disease.123
Hypertension, vascular, and endothelial factors are more
likely the associated culprits.

Echocardiography
Two-dimensional echocardiography is highly specific
in diagnosing aortic arch obstruction.124 The aortic
arch should be first interrogated carefully by 2D
echocardiography with pulsed Doppler recording, under
direct visualization. On transthoracic echocardiography,
the coarctation is best seen in the suprasternal or high right
parasternal views, also allowing visualization of the aortic
arch and proximal descending aorta. Presence of color
flow Doppler turbulence in the aortic arch brings to our
attention the maximum site of narrowing. Continuous
wave spectral Doppler interrogation is used to assess
the gradient. It shows characteristic flow profile with an
increased systolic flow velocity and continuous gradient
of forward diastolic flow. The more accurate formula
for estimating the pressure gradient is 4(V2-V1)2 with
V1 being the velocity proximal to the coarctation and V2
being the peak velocity at the site of maximal narrowing.
This formula avoids inaccurate reports of high pressure
gradients obtained by the simplified Bernoulli equation
(4V2) in patients with long tubular narrowing of the aortic
arch.125 A peak gradient of over 20 mm Hg indicates a
significant obstruction. However, the gradient may be
low or negligible if there are collaterals or ductal flow.

1827

Marx et al. had noted that the major pitfall of

echocardiography in COA was due to the significant
collateral flow resulting in the failure to record an accurate
Doppler gradient.125 In the absence of collateral flow,
Therrien et al. reported that echocardiography was a
suitable screening test with a sensitivity of 87%.126 Limited
visualization of the aortic arch has been another issue with
transthoracic echocardiography.
An abnormal Doppler flow pattern may also be noted
in the abdominal aorta, with decreased pulsatility and
absence of early diastolic flow reversal. The abdominal
aortic Doppler profile will not show diastolic flow reversal
in such patients without a discrete obstructive lesion.
Conversely, it is a useful technique for screening an obscure
obstruction of significance that will manifest by delayed
systolic upstroke, diastolic flow reversal (antegrade flow),
and turbulence during systole on color/spectral Doppler
examination of the upper abdominal aorta. Abnormal
flow in collateral vessels can be detected by color flow and
pulsed wave Doppler.
Adults with coarctation often have a bicuspid aortic
valve and aortic root dilatation. Therefore, it is important
to measure the transverse dimensions at the level of the
aortic annulus, aortic sinuses, sinotubular junction, and
approximately 2 cm distally in the ascending aorta.
Due to long-standing hypertension and multiple
factors, many adults are prone to hypertensive heart
disease and need assessment of left ventricular size,
mass (left ventricular hypertrophy), systolic, and diastolic
function. Other associated defects are ventricular septal
defects, subaortic stenosis, patent ductus arteriosus, and
mitral valve disorder (supramitral ring).
A synopsis of the echocardiographic assessment of
coarctation of aorta is reviewed in Table 75.13.

Transesophageal Echocardiography
Agrawal et al. showed the incremental value of TEE in
delineating the aortic arch branches. Their protocol can
be adapted for the multiplane TEE probe.127 The short-axis
view of the proximal descending aorta is first identified as
a circular cavity in the transverse plane in the midthoracic
level. Further withdrawal of the probe brings the aortic
arch into view with the loss of the circular configuration
transitioning into a horizontal tube-like structure.
Switching from the transverse to the longitudinal plane (90)
demonstrates all three vessels (Fig. 75.1). The longitudinal
cut of the aortic arch makes it appear circular or ovoid.
Small movements of the probe (rotation, movement,

1828

Section 6: Congenital Heart Disease

Table 75.13: Coarctation of the Aorta

Associated defects

Bicuspid aortic valve

Ventricular septal defect

Patent ductus arteriosus

Left heart obstructive lesionssubaortic stenosis, mitral stenosis

Aortic root/arch dilatation or aneurysm

Echocardiographic assessment

Aortic arch anatomyrule out aneurysm

Aortic arch gradient

Assessment of associated defects

Left ventricular hypertrophy

Left ventricular function

Stress echocardiography for evaluation of ambulatory hypertension and coronary ischemia

Postoperative

Rule out recoarctation or aneurysm at the site of the surgery (especially with patch repair)

Left ventricular hypertrophy (hypertension usually persists despite surgical relief of the coarctation)

Left ventricular size and function

Stress echocardiography for evaluation of ambulatory hypertension and coronary ischemia

and angulation) allow visualization of the aortic arch.

Clockwise rotation of the probe moves the vertical plane
to the right bringing the innominate artery into view as
the most anterior vessel, while counterclockwise rotation
moves it to the left, and reveals the left carotid and the left
subclavian arteries more posteriorly.127 The X-plane view
(Philips Echocardiography System) allows simultaneous
views of the transverse and longitudinal planes.

Treadmill Stress Testing

Treadmill stress testing plays a very important role
in determining the maximum exercise systolic blood
pressure, which is a predictor for chronic hypertension
in adults with COA.128 Antihypertensive therapy can then
be started in a timely manner. Adults with COA may have
normal blood pressure during office visits and treadmill
stress testing unmasks the ambulatory hypertension in
response to exercise that they may be experiencing in their
daily lives.
It also allows assessment of functional capacity,
exercise-induced arrhythmias, and ischemia.
Depending upon its availability, cardiopulmonary
exercise testing (CPET) may hold a promise by using
exercise parameters that may assist in early identification

of nonhypertensive patients with COA who are at increased

risk of developing of arterial hypertension.129

Stress Echocardiography
Stress echocardiography with color and continuous wave
Doppler from the suprasternal notch allows assessment
of the coarctation gradient at rest and following exercise,
in addition to providing information obtained by the
treadmill stress testing. Coronary artery disease occurs
prematurely in this population, especially in those with
uncontrolled ambulatory hypertension.

Magnetic Resonance Imaging/Computed

Tomography
MRI is the diagnostic test of choice for complete
evaluation of the coarctation of aorta due to limitations
of echocardiography, especially TEE (because of the
perpendicular plane of the Doppler in relation to the
obstructive lesion).126
In patients undergoing surgery, preoperative MRI/
MRA or CT angiography with 3D reconstruction allows
precise identification of the location, anatomy of the
coarctation, and the entire aorta along with the collaterals.

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

MRI is useful for defining the morphology, delineating

aneurysms in postoperative cases, site of the stenosis,
extent and degree of narrowing, aortic arch anatomy,
and aortopulmonary collaterals. Most importantly, MRI
provides hemodynamic data with calculation of the
pressure gradient across the stenosis.
CTA is another preferred imaging modality for imaging
the area of coarctation and for obtaining extracardiac
information in an adult with a pacemaker or defibrillator.
Within a short acquisition time, simultaneous visualization
of the ascending aorta, aortic arch, descending aorta, aortic
valve, course of the coronary arteries, coronary anomalies
and calcium risk score can be obtained.130
Magnetic resonance angiography (MRA) is performed
every 5 years to search for aneurysms of the intracranial
arteries, since intracranial aneurysms may occur even
in normotensive adults.131 MRA screening has reported
intracranial aneurysms in approximately 10% of the
patients with COA.132 Martin et al. reported a case of a young
woman with severe COA presenting and subarachnoid
hemorrhage.133
Evaluation of the repair site by MRI/CT should be
performed at intervals of 5 years or less, depending on the
specific anatomical findings before and after repair.

Cardiac Catheterization
Coronary artery disease should be ruled out in adults prior
to surgery. Stent implantation is the preferred intervention
compared to balloon angioplasty alone for coarctation of
aorta in adults, with persistent relief of stenosis and lower
incidence of aneurysm formation.

Surgery or Intervention for

Coarctation of Aorta
The ACC/AHA Class I recommendations for interventional
and surgical treatment of coarctation of the aorta in adults
are the following:3
Peak-to-peak coarctation gradient 20 mm Hg
Peak-to-peak coarctation gradient < 20 mm Hg when
an imaging study shows direct evidence of significant
coarctation with radiological evidence of significant
collateral flow.
In addition, the presence of a large aortic root, arch
aneurysm, or severe degree of associated defects may also
prompt early intervention/surgery.
Catheter-based intervention is the preferred
alternative to surgery for recurrent aortic coarctation

1829

following surgical repair in the absence of aneurysm/

pseudoaneurysm formation, complex coarctation
affecting the adjoining arch arterial branches, or need for
concomitant surgery (for aortic valve or aortic root).

The Postoperative Adult

Recurrence of narrowing at the coarctation site can occur
in adulthood. Aneurysm formation at the repair site
occurs in some adults after Dacron patch aortoplasty or
following resection of the coarctation. Some individuals
many have pseudoaneurysms while in others, there is a
risk of dissection around the site of repair due to intrinsic
structural abnormalities of the aorta.
Doppler echocardiography, guided by transthoracic
2D images, is useful for follow-up after successful stenting
of the coarctation site reflected by a significant decrease
in peak systolic pressure gradient. Other promising
parameters that may find more widespread use in the
future include diastolic velocity, diastolic velocity halftime index, and diastolic pressure half-time index.134

Tetralogy of Fallot
Four defects make the tetralogy of Fallot (TOF)a VSD, an
aorta that overrides the large ventricular septal defect (by <
50% of its diameter), subpulmonary infundibular stenosis,
and right ventricular hypertrophy. The presence of an ASD
would make it the pentalogy of Fallot!31
The clinical presentation is affected by the variable
degree of right ventricular outflow tract obstruction. The
most extreme form is pulmonary atresia with a VSD.
Associated defects are common and include abnormal
or absent pulmonary valve, pulmonary artery anomalies
(dilated or hypoplastic), aortic root dilation, aortic
regurgitation, right aortic arch (approximately 2030%),
and abnormal origin of the coronary (left anterior
descending coronary artery arising from the right coronary
artery and crossing the right ventricular outflow tract).
Most adults have had previous palliative shunts or
complete intracardiac repair but continue to present
with multiple residua and sequelae. The intracardiac
repair comprises closure of the VSD with redirection of
the aorta toward the left ventricle, and resection of the
right ventricular outflow tract obstruction with relief of
hypertrophied muscle that is contributing to infundibular
stenosis. Following an intracardiac repair of TOF, the most
common long-term cardiac issue is chronic pulmonary
valve regurgitation that requires reoperations (pulmonary
valve replacement), approximately every 10 to 20 years.

1830

Section 6: Congenital Heart Disease

Adults with pulmonary regurgitation (due to an absent

pulmonary valve or other pulmonary valve abnormalities)
who did not undergo pulmonary valve replacement
during their growing years, also present with progressive
pulmonary regurgitation. Those with absent pulmonary
valve also have massive enlargement of the pulmonary
arteries with severe pulmonary regurgitation. Some other
factors exacerbating pulmonary regurgitation in this
population that can be assessed echocardiographically
are the following: pulmonary annulus size, peripheral
pulmonary artery stenosis, residual shunts (atrial and
ventricular septal defects), pulmonary hypertension, and
right ventricular diastolic dysfunction.
Progressive pulmonary regurgitation leads to right
ventricular enlargement, impaired right ventricular
function (systolic and diastolic), and increasing tricuspid
regurgitation and right atrial enlargement. The clinical
impact of these is variable and different patients will
present with varying degrees of decreased exercise
tolerance, dyspnea on exertion, and predisposition to
atrial/ventricular arrhythmias. In a multicenter study, we
reported that symptoms related to pulmonary regurgitation
may be well tolerated over a long period; progressively
increasing from the beginning of the fourth decade with
more than half the adults becoming symptomatic in the
following two decades.135
Besides pulmonary regurgitation, other factors
contributing to right ventricular enlargement and
impaired function are the degree of tricuspid regurgitation,
pulmonary hypertension, residual shunts, and distortion
of the shape of the right ventricle. The dilated right ventricle
may exhibit improved ejection fraction temporarily in
response to volume overload before spiraling down with
further decrease in myocardial contractility.
Left ventricular dilatation, decreased contractility,
increased myocardial stiffness, and impaired function
occurs over time following intracardiac repair of TOF.136
The left ventricular size and function is impacted
specifically by the severity of aortic regurgitation due to
inadequate coaptation of the aortic leaflets as a result of
aortic root dilatation. Due to interventricular dependence,
the deteriorating right ventricular function adversely
impacts the left ventricular performance.
For both the ventricles, the timing of palliative shunts
and definitive intracardiac repair affects myocardial
contractility. Prolonged cyanosis, inadequate myocardial
preservation during surgeries involving cardiopulmonary
bypass, and pressure and volume overload have deleterious
effects on the myocardium predisposing it to fibrosis.

Pulmonary regurgitation and right ventricular outflow

aneurysm/akinesia are independently associated with
right ventricular dilatation, leading to hypertrophy and
reduced function over time. It is, therefore, mandatory
to restore pulmonary valve function and avoid RVOT
aneurysm/akinesia in order to preserve biventricular
function.137
Concomitant defects such as atrial or ventricular septal
defect may present with residual patch leaks on Doppler
interrogation.
Although aortic root dilation is fairly common in adults
with tetralogy of Fallot, aortic dissection has only been
reported in rare instances in the published literature. In
these reports, it invariably occurred with markedly severe
aortic root dilatation, with aortic dimensions varying
between 7 and 9.3 cm.138140 So far, the aortic arch sidedness
has not been proven to have an independent association
with the degree of aortic root dilatation.
In addition to the hemodynamic effects, severe
pulmonary regurgitation exacerbates the scar tissue at the
site of the RVOT patch aneurysm, making it a nidus for
generation of monomorphic ventricular tachycardia.141
Pulmonary atresia with ventricular septal defect
represents the severest form of right ventricular outflow
tract obstruction. Surgical repairs are accordingly more
complex, involving unifocalization of the multiple
aortopulmonary collaterals into right and left conduit that
are then connected to the right ventricle to pulmonary
artery (RV to PA) conduit, to mimic the main pulmonary
artery and its branches (Fig. 75.19).

Echocardiography
A comprehensive transthoracic echocardiographic evaluation in an adult with tetralogy of Fallot postintracardiac
repair begins with the assessment of the right and left
ventricular size and function. Left ventricular function
is estimated by standard methods. Besides the standard
measures for assessing left ventricular function, a
Doppler-derived index of LV filling pressure (the ratio
of early transmitral flow velocity to early diastolic mitral
annular velocity) is a powerful predictor of ventricular
arrhythmias.142
Right ventricular enlargement and declining systolic/
diastolic function are common especially in the setting
of progressive pulmonary regurgitation. The enlarging
right ventricle stretches the tricuspid annulus causing
progressive tricuspid regurgitation, followed by right atrial

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1831

independent parameter for right ventricular function is

myocardial acceleration during isovolumetric contraction,
a Doppler-based index, described by Frigiola et al.148 The
isovolumic acceleration index is calculated by dividing
the myocardial velocity during isovolumic contraction by
the time interval from the onset of the acceleration to the
time at peak velocity. It allows early detection of impaired
ventricular function due to the detrimental effect of
pulmonary regurgitation before the onset of symptoms.148
While qualitative assessment by experienced readers
continues to be a practical solution for serial followups, 3D quantification of right ventricular function with
speckle tracking is gaining popularity. In a study by Gopal
et al, 3D echocardiographic techniques have been shown
to correlate closely with MRI-derived right ventricular
end-diastolic volume and could reduce the need for MRI
in some patients.149

Fig. 75.19: Diagrammatic representation of the surgical repair

in pulmonary atresia with ventricular septal defect. Unifocalization
of the multiple aortopulmonary collateral arteries (MAPCAs) into
right and left conduits is performed, that are then connected to
the main right ventricle to pulmonary artery (RV to PA) conduit, to
mimic the main pulmonary artery and its branches. (LV: Left ventricle; RV: Right ventricle; VSD: Ventricular septal defect). (Inspired
by an operation note sketch by Dr Hillel Laks).

enlargement. The right ventricular systolic pressure is

estimated from the tricuspid regurgitant jet velocity. Some
adults may have a residual right ventricular outflow tract
obstruction contributing to right ventricular hypertrophy,
while others may have an aneurysm of the right ventricular
outflow tract.
Right ventricular size and systolic function: The shape of
the right ventricle, trabeculations, and distortion due to
previous surgeries involving the outflow tract have made
accurate quantification very challenging. Transthoracic
echocardiography is limited in visualizing the right
ventricular outflow tract for identifying aneurysm that
along with right ventricular dilatation have a major impact
on the clinical status of those operated for tetralogy of
Fallot in the presence of pulmonary regurgitation. In
addition, changes in loading conditions may affect some of
the measurements obtained by using echocardiographic
parameters such as the Tei index,143,144 stress and strain rate
imaging,145 and instantaneous rate of pressure increase
(dP/dt) by Doppler assessment.146,147 A relatively load-

Right ventricular diastolic function: Isolated right ventricular restriction late after tetralogy of Fallot repair is fairly
common. Methods for assessment of right ventricular
diastolic function and restrictive physiology have been of
major research interest. The clinical applications of these
time-consuming protocols have been limited so far due
to difficulty in accurately using them on the distorted
right ventricular anatomy. Gatzoulis et al. showed that
antegrade diastolic flow in the main pulmonary artery,
coinciding with atrial systole (A-wave) throughout the
respiratory cycle was indicative of right ventricular
restriction. On pulsed Doppler sampling (at the midpoint
between the pulmonary valve leaflets and bifurcation of
the main pulmonary artery), the duration of pulmonary
regurgitation was noted to be shorter in those with right
ventricular restriction. This finding was thought to be
reflective of reduced right ventricular diastolic compliance,
with restrictive physiology at end-diastole, thereby making
the right ventricle a passive conduit between right atrium
and pulmonary artery during atrial systole.150 However, it
can also be present in normal hearts during inspiration.
Therefore, it should be recorded for at least five consecutive
beats to be of diagnostic value in identifying reduced right
ventricular diastolic compliance.
Other features on pulsed Doppler that support restrictive
physiology include: Superior vena caval flow reversal
with atrial systole (measured 12 cm proximal to the
right atrium), and shorter inspiratory and expiratory
transtricuspid E-wave deceleration times (measured at the
level of the tricuspid valve leaflets).

1832

Section 6: Congenital Heart Disease

Pulmonary valve regurgitation/stenosis: While some adults

may have residual pulmonary stenosis that needs attention
for its etiology, location, and severity, the most common
issue in this population is pulmonary regurgitation.
The best view for assessment of pulmonary regurgitation
is the parasternal short-axis view at the level of the great
arteries. This view can be modified to evaluate the proximal
conduit, the main and branch pulmonary arteries. Color
and pulsed Doppler are used to evaluate: (a) width of
the regurgitant jet at the level of the pulmonary valve or
proximal right ventricular outflow tract (RVOT) conduit/
prosthetic valve; and (b) the degree of flow reversal in the
main and branch pulmonary arteries. For assessment of
the RVOT conduit, continuous wave Doppler is used to
assess maximum velocity and mean gradient across the
conduit or a prosthetic valve in the parasternal long-axis
views, apical, or subcostal views. Pulmonary regurgitation
is graded as trace, mild, moderate, or severe as shown in
Table 75.14.
Although many other methods have been proposed in
recent times for the assessment of pulmonary regurgitation,
color Doppler continues to be the first one to bring it to our
attention. The regurgitant jet flow can be seen in the right
ventricular outflow tract. Retrograde diastolic flow into the
main pulmonary artery and its branches may be seen in
severe pulmonary regurgitation. Spectral Doppler sample
at the level of the pulmonary valve demonstrates normal
forward flow in systole and holodiastolic flow reversal due
to severe pulmonary regurgitation. A pressure half-time
of <100 milliseconds is also a good indicator of severe
pulmonary regurgitation.151
However, color Doppler may not be a reliable indicator
in low-pressure severe pulmonary regurgitation. In these
cases, the pulsations in the right ventricular outflow tract/
main pulmonary artery that often extend into the branches
are the surrogate marker.
The degree of pulmonary stenosis or residual obstruction at the level of pulmonary valve, infundibulum, or
pulmonary artery branches should be estimated using the
standard continuous wave Doppler technique.
Echocardiographic assessment of palliative shunts: Although
most of the adults have undergone intracardiac repair with
takedown of the shunt, a few may still have long-standing
palliative shunts. The most common shunts are:
Classic BlalockTaussigThomas (BTT) shuntsubclavian to pulmonary artery anastomosis to increase
pulmonary blood flow
Modified BTT shuntsubclavian to pulmonary
artery anastomosis, using a Gore-tex graft to increase
pulmonary blood flow

Potts shuntdescending aorta to left pulmonary artery

anastomosis to increase pulmonary blood flow
Waterston shuntascending aorta to right pulmonary
artery anastomosis to increase pulmonary blood flow.
These palliative shunts were performed to enhance
pulmonary flow in patients with severe right ventricular
outflow tract obstruction prior to complete intracardiac
repair. Continuous wave Doppler shows nonlaminar
blood flow directed from the aorta to pulmonary artery
both in systole and diastole. With the larger shunts,
pulmonary hypertension may develop over time. When
the pulmonary hypertension becomes severe, the diastolic
flow ceases; and when pulmonary artery systolic pressure
exceeds systemic values, the systolic flow ceases.152 While
the orifice of the BTT shunt between the subclavian and
pulmonary artery shunt may be seen on a transthoracic
echocardiogram, transesophageal echocardiography is
required to see the side-to-side connections in Potts and
Waterston shunts.152
Aortic root dilation (with loss of the sinotubular
junction) is common in tetralogy of Fallot and may
contribute to progressive aortic regurgitation due to
inadequate coaptation of the aortic leaflets. Aortic root
dimensions should, therefore, be measured on serial
echocardiograms as discussed in the section on aortic root
in adults with congenital heart disease. Color Doppler
assists in ruling out residual shunts (ventricular or atrial
septal defects).
A synopsis of echocardiographic assessment of
tetralogy of Fallot is reviewed in Table 75.15.

Stress Echocardiography
Exercise testing is required for objective assessment
of functional capacity and to unveil exercise-induced
arrhythmias such as atrial fibrillation/flutter, premature
ventricular complexes, and nonsustained ventricular
tachycardia in the postoperative adult with tetralogy of
Fallot. Monomorphic ventricular tachycardia most often
occurs in the presence of an old ventriculotomy scar. Poor
heart rate response to exercise unmasks chronotropic
incompetence that may lead to pacemaker implantation.
Stress echocardiography provides additional information regarding the severity of pulmonary hypertension
postexercise and the impact of pulmonary regurgitation
on the right ventricular contractile reserve, by gauging
its hyperdynamic response to exercise. Meijboom et al.
showed that exercise capacity inversely correlates with right
ventricular dilation in patients with repaired tetralogy.153

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1833

Table 75.14: Tetralogy of Fallot

Associated defects

Right aortic arch20% to 30%

Pulmonary stenosis

Hypoplastic pulmonary arteries

Absent pulmonary valve

Pulmonary atresia

Pulmonary artery dilatation/aneurysm

Patent foramen ovale

Atrial septal defect

AV septal defects

Muscular ventricular septal defects

Anomalous origin of the coronary arteries (left anterior descending arising from right coronary cusp and courses across the
right ventricular outflow tract)

Aortic root dilatation

Aortic regurgitation

Subaortic stenosis

Aortopulmonary window

Aortopulmonary collaterals

Absent left pulmonary artery

Origin of a pulmonary artery from the aorta

Ebsteins anomaly of the tricuspid valve

Anomalous coronaries

Echocardiographic assessment

Right and left ventricular size and function

Right ventricular outflow tract (infundibular) obstruction

Pulmonary regurgitation

Pulmonary stenosisat different levels, usually branch stenosis

Aortic regurgitation

Aortic root dilation (loss of the curvature of the sinotubular junction)

Shunt lesionsventricular septal defects, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava

Aortic arch anatomy (right aortic arch seen in 2025%)

Origins of the coronary arteries

Postoperative

Residual VSD or patch leak

Pulmonary regurgitation

Pulmonary stenosis

Right ventricular out flow tract size/aneurysm

Aortic root size

Aortic regurgitation

Right ventricular size and function

Left ventricular size and function

1834

Section 6: Congenital Heart Disease

Table 75.15: Echocardiographic Grading of Pulmonary Regurgitaiton

A. Based on the Color Doppler width of the jet on the ventricular aspect of pulmonary valve leaflets
Tracea flash of color
Mildjet width < 20% of the annular width of the valve/conduit
Moderatejet width between 20% and 40% of the annular width of the valve/conduit
Severejet width > 40% of the valve/conduit annular width
B.

Based on diastolic flow reversal

TraceDiastolic color flow reversal limited to the beginning at valve/conduit
MildDiastolic color flow reversal limited to the proximal half of the main pulmonary artery
ModerateDiastolic color flow reversal extending into distal half of the main pulmonary artery
SevereDiastolic flow reversal extending into proximal branch pulmonary arteries

Source: Adapted from Brown DW et al. J Am Soc Echocardiogr 2012;25:38392.

It also plays an adjunctive role in risk stratification of

patients who are more likely to have sudden cardiac death
by determining the impact of hemodynamic burden of
pulmonary regurgitation and right ventricular impairment
on the electrophysiological substrates.
In patients undergoing cardiopulmonary exercise
testing, a decline in functional aerobic capacity (maximum
VO2) to <70% of genderage-predicted maximum value
or a decline over 20% compared with serial testing is
considered significant.
Adults with right ventricle to pulmonary artery (RV
to PA) conduit should undergo evaluation for abnormal
ventricular function and conduit obstruction at peak
exercise. Besides tetralogy of Fallot, others with RV to
PA conduit include patients with pulmonary atresia and
multiple aortopulmonary collaterals, truncus arteriosus,
or d-transposition of great arteries. A RV-to-PA conduit
is considered to be stenotic when the mean continuous
wave Doppler gradient across the conduit is over 20 mm Hg
on echocardiography. Hasan et al. reported a protocol
with mean instantaneous systolic gradient and maximum
instantaneous systolic gradient across the RV-to-PA
conduit obtained at rest and immediately postexercise,
using continuous wave Doppler through the conduit in the
parasternal short-axis view.154

Cardiac Catheterization
Angiography plays a limited role in the current era for
the postoperative adult with tetralogy of Fallot. It is not a
reliable test for assessment of pulmonary regurgitation,
since the angiographic severity may be influenced by
catheter position across the pulmonary artery. It accurately

assesses coronary artery disease, pulmonary artery or

branch stenosis, and pulmonary vascular resistance
in adults with pulmonary hypertension due to longstanding shunts. It is routinely performed in conjunction
with potential catheter-based interventions such as
percutaneous pulmonary valve implantation.
The Melody transcatheter pulmonary valve
(Medtronic, Inc., Minneapolis, MN) is approved by the US
Food and Drug Administration for treatment of patients
with regurgitant or stenosed right ventricular outflow
tract (RVOT) conduits. The indications for implantation
can vary from isolated pulmonary regurgitation to relief
of RVOT obstruction, from a bridging procedure to a more
definitive therapy.155
Echocardiography complements MRI in determining
the suitability for percutaneous pulmonary valve
implantation by assessing the following parameters: (a)
right ventricular outflow tract diameter between 16 and
24 mm, (b) right ventricular outflow gradient of >30 mm
Hg, (c) the presence of discrete waist or calcification for
implantation, and (d) the absence of significant pulsatility.
MRI provides additional information about any limitations
in access to the right ventricle due to occlusion of the
central vein.
Echocardiography also identifies procedural complications resulting from implantation of the percutaneous
pulmonary valve, including rupture of the RVOT conduit,
embolization or migration of the device, and perforation of
a heart chamber that may manifest as a pericardial effusion.
Other short- and long-term complications include stent
fracture resulting in recurrent obstruction, endocarditis,
valvular stenosis/regurgitation, paravalvular leak, and
valvular thrombosis. The most common complication

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

noted during follow-up of the Melody valves are stent

fractures with an incidence of approximately 20%.156
Other interventional procedures include ASD closures,
angioplasty/stent implantation of obstructed pulmonary
arteries/branches, and deployment of coils in the collateral
vessels or systemicpulmonary artery shunts.

Magnetic Resonance Imaging/Computed

Tomography
MRI is the current reference standard for quantification of
right ventricular volume and the systolic function.157,158 It
provides accurate measurements of the aorta, pulmonary
artery, and its branches. Areas of myocardial fibrosis due
to previous surgeries can be identified by gadoliniumenhanced MRI.
For preoperative evaluation in adults who are unable
to have an MRI (due to devices such as pacemakers/
defibrillators), CTA is an alternative. In addition, it allows
us to identify anomalous coronaries and calcium score.

Surgery in a Postoperative Adult with

Tetralogy of Fallot
The ACC/AHA Class I indication for surgery in a
postoperative adult with tetralogy of Fallot is:3
Pulmonary valve replacement for symptomatic severe
pulmonary regurgitation with declining exercise
tolerance
Since bioprosthetic valves have a limited life expectancy, surgery should be performed only when it is clearly
indicated. The porcine bioprosthetic valves have shown
better durability than the cryopreserved homografts. Many
studies have proposed additional criteria in symptomatic
adults presenting with exertional dyspnea, heart failure,
and ventricular arrhythmias. These include progressive
right ventricular dilatation.
When the pulmonary valve replacement is performed in
a timely fashion, it may lead to improvement in symptoms,
reduction in right ventricular size, and recovery of systolic
function. The timing of surgery in asymptomatic adults with
pulmonary regurgitation remains controversial. It may be
considered when there is an objective evidence of a decline
in exercise capacity on stress testing and progressive right
ventricular enlargement confirmed on MRI. Based on the
data from two studies, in order to expect improvement
in right ventricular function following pulmonary valve
replacement, the surgery should be considered when the
right ventricular end-diastolic volume is between 150 and

1835

170 cc/m2; and/or when the right ventricular end-systolic

volume reaches 85 cc/m2.159,160
Other criteria include widened QRS over 180
milliseconds or an annual increase in QRS width over 3.5
milliseconds in the setting of ventricular arrhythmias and
worsening tricuspid regurgitation.
Concomitant surgical removal of the right ventriculotomy scar, right ventricular outflow tract aneurysm,
pulmonary arterioplasty, aortic root, aortic valve repair/
replacement, and closure of residual shunts are performed
to improve the hemodynamic status. Surgical relief of
RVOT obstruction should be considered when RVSP is
over two thirds of the systemic pressure.
Intraoperative transesophageal echocardiography
plays an important role, ensuring the adequacy of repair
in these patients.

The Postoperative Adult

Although patients may demonstrate some symptomatic
improvement with medical therapy, pulmonary valve
replacement is the only treatment modality with proven
long-term benefit. Serial echocardiograms may show a
reduction in right ventricular size, an improvement or at
least stabilization of right ventricular function.
In later years, following bioprosthetic pulmonary
valve replacement or placement of an extracardiac
conduit (from the right ventricle to pulmonary artery),
stenosis or regurgitation may develop and require further
reoperations to maintain functional improvement.161 An
overall review of multiple studies shows that pulmonary
valve replacement in tetralogy of Fallot postintracardiac
repair shows an improvement in clinical outcomes.
Symptoms resolve and right ventricular function often
improves. Several retrospective reviews have reported
reduction in right ventricular end-diastolic and systolic
volumes along with an increased right ventricular stroke
volume.162
Meijboom et al. reported that surgery for pulmonary
valve replacement should be offered to symptomatic
patients for best outcomes in the majority of adults, who
have undergone intracardiac repair in childhood and
present with severe pulmonary regurgitation and right
ventricular dilatation.163

D-Transposition of the Great Arteries

In d-transposition of the great arteries (d-TGA), there is
ventriculoarterial discordance with abnormal origins of

1836

Section 6: Congenital Heart Disease

the aorta and pulmonary artery. This implies that the aorta
arises from the systemic morphological right ventricle
and lies rightward and anterior to the pulmonary artery,
which in turn arises from the pulmonic morphological left
ventricle.
Common associated defects include a shunt lesion, a
VSD (45%), an ASD, or a PDA that allows intermixing of the
deoxygenated and oxygenated blood to permit survival,
since this circulation is a parallel circuit and will not be
compatible with life without any communication. Most
individuals are diagnosed early in life with this form of
cyanotic congenital heart disease and have undergone one
of the types of repairs. Other associated defects include left
ventricular outflow tract (LVOT) obstruction, coarctation
of the aorta, and anomalies of the coronary ostia due to
transposition.31

Postatrial Switch Repair

Many older adults seen in our practice have had an atrial
switch repair. In this type of surgery, the deoxygenated
blood from the superior and inferior vena cava is directed
via a baffle into the left ventricle, and then pumped into
the pulmonary artery. The left ventricle becomes the
subpulmonic ventricle. The oxygenated blood returns
through another baffle from the pulmonary veins into
the right ventricle and gets pumped into the aorta. This
makes the morphological right ventricle the subaortic
or the systemic ventricle. The two types of atrial switch
operations are the Senning and the Mustard repairs. They
differ primarily in terms of the material used to create the
baffles. In the Senning operation, the baffle is created from
the patients tissues (right atrial wall and part of the atrial
septum).164 The Mustard operation uses pericardium and
synthetic material to make the baffle.165 The interatrial
baffles can deteriorate over time leading to obstruction or
leaks. The scar tissue along atrial incisions and the foreign
material may become substrates for atrial arrhythmias.
Surgical damage of the sinus node and conduction system
makes pacemaker surgery more likely in adulthood.

Echocardiography
Serial echocardiography is performed in adults who have
undergone atrial switch repair to assess the morphological
right ventricular size and function. Most adults have a
globular-shaped, hypertrophied morphological right

ventricle, which is functioning as a systemic ventricle

by pumping into the aorta. In the parasternal short-axis
views, the left ventricle appears compressed due to a
D-shaped septum or bowing of the septum toward the
morphological left ventricle. The aorta and the pulmonary
artery can be seen parallel to each other in the parasternal
long-axis view and in the same plane in the parasternal
short-axis view (Figs 75.20A and B).
The systemic (morphological right) ventricular function
is impacted by the degree of systemic AV (tricuspid) valve
regurgitation in a way quite similar to the impact of the
mitral regurgitation on the left ventricle in normal hearts.
Progressive aortic regurgitation increases the workload on
the systemic (morphological right) ventricular function.
Subpulmonic stenosis increases pressure load on the left
ventricle.
Patients with baffle obstructions or leaks may
present with dyspnea or worsening atrial arrhythmias.
Flow acceleration, turbulent flow on color Doppler, and
increased Doppler gradients indicate baffle narrowing
or obstruction. Baffle issues are more commonly seen
following a Mustard rather than a Senning repair and
usually involve the superior limb.166 Bottega et al. reported
obstruction of the superior limb in 44% of the adults postMustard repair.167
The velocities in the superior limb of the systemic
venous baffle can be measured from a parasternal or
tilted apical four-chamber view. Baffle obstruction by
Doppler echocardiography is considered when there is
a pulsed wave Doppler peak velocity of 1.5 m/s with a

Figs 75.20A and B: Diagrammatic representation of the

transthoracic echocardiogram in d-transposition of the great
arteries (DTGA). The aorta and the pulmonary artery (PA) are
seen in parallel to each other in the parasternal long-axis view (A)
and in the same plane (rather than criss-cross) in the parasternal
short-axis view (B). (LA: Left atrium; LV: Left ventricle; RV: Right
ventricle).

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

characteristic loss of the biphasic Doppler profile.167,168

Pulmonary venous baffles are easier to image by
echocardiography and should be suspected when there is
an increase in the left ventricular systolic pressure.169
Baffle leaks usually result in right heart enlargement
as seen with long-standing atrial septal defects. In these
adults, the subpulmonary left ventricle can appear more
D-shaped with a compressed appearance when there is
a hemodynamically relevant baffle leak, subpulmonary
obstruction, or pulmonary hypertension.169 Color Doppler
may reveal the site of the shunt and an agitated saline
contrast study may confirm it.
TEE may be required when there is limited visualization
by a transthoracic echocardiogram. TEE also allows direct
examination of caval anastomosis.170 Hepatic venous flow

1837

and superior vena caval patterns showing presystolic flow

reversal with atrial contraction indirectly indicate patency
of the caval anatomosis. Residual ventricular septal
defects or patch leaks should be examined with color
Doppler. A synopsis of echocardiographic assessment
of the postoperative adult with d-TGA is reviewed in
Table 75.16.
Three-dimensional echocardiography may add
incremental value to visualization of the baffle and
tricuspid valve anatomy in the post-Mustards adults. It
may provide a more comprehensive assessment of baffle
obstruction by allowing it to be viewed in multiple planes
and projections.171 In addition to the en face viewing
of the intra-atrial baffle and measurements of vena
contracta from the baffle leaks, it provides complementary

Table 75.16: D-Transposition of the Great Arteries

Associated defects

Shunt lesionsventricular septal defect (usually perimembranous), atrial septal defect and patent ductus arteriosus

Left ventricular outflow tract (LVOT) obstructionpulmonary stenosis, usually valvular but may be subvalvular

Coarctation of the aorta

Coronary artery anomalies

Right ventricular outflow tract (RVOT) obstruction (subaortic narrowing)

Echocardiographic assessment
Most of the adults have had surgeries in their childhood

Great arteriesaorta and pulmonary arteryare seen parallel to each other in the parasternal long-axis view.

In the short-axis view they are seen next to each other with the aorta being anterior and rightward.

Postoperative
Atrial switch repair

Assessment right (systemic) ventricular size systemic function

Right ventricular hypertrophy

Baffle leak

Baffle obstruction

Estimation of right ventricular systolic pressure/degree of pulmonary hypertension

Arterial switch repair

Assessment of left ventricular size and systolic function

Coronary artery fibrosis may lead to ischemia/infarctionstress echocardiography is performed to assess wall motion abnormalities

Neoaortic valve regurgitation

Mild aortic root dilatation

Pulmonary artery stenosis at the valvar supravalvar or peripheral level

Estimation of right ventricular systolic pressure/degree of pulmonary hypertension

Post-Rastelli repair

Assessment of biventricular ventricular size and systolic function

Conduit obstruction

1838

Section 6: Congenital Heart Disease

anatomical details of the pulmonary valve and the left

ventricular outflow tract.172

Stress Echocardiography
Exercise capacity may be limited postatrial switch
repair due to stiffness and noncompliance of the baffles,
deterioration in systemic ventricular function, sick
sinus syndrome, or advanced conduction disease. Baffle
problems appear to have the most effect on exercise ability
because of impaired ventricular filling and limited ability
to augment stroke volume during exercise.173 Hence,
functional capacity assessed objectively by exercise stress
echocardiography should be followed by a search for causes
of hemodynamic impairment such as baffle obstruction,
progressive tricuspid regurgitation, subpulmonic obstruction, impaired systemic ventricular function, or
chronotropic incompetence. Routine repetition of exercise
studies every 3 years is recommended even in the absence
of symptoms, since gradual declines in function often go
unnoticed by patients.169

Cardiac Catheterization
Systemic or pulmonary venous baffle obstructions are
diagnosed by angiography and can be often be relieved by
balloon-expanded stent implantation. Some of the baffle
leaks can be eliminated by implanting smaller devices.
Intracardiac echocardiography plays a role in guiding
percutaneous closure of atrial baffle defects.175

Magnetic Resonance Imaging/Computed

Tomography
Baffle stenosis or leak may be more clearly defined by MRI/
CTA.167 Baffle patency should be evaluated by either of
these modalities before transvenous device implantation.
MRI is also the gold standard for accurate assessment of
systemic right ventricular volumes and function, since
most echocardiographic parameters have reduced
sensitivity and marked intraobserver variability.174

Reoperation for D-Transposition of the Great

Arteries Postatrial Switch Repair
The Class I ACC/AHA indications for surgery in d-TGA
postatrial switch repair (Mustard or Senning) are the
following:3
Moderate to severe systemic (morphological tricuspid)
AV valve regurgitation without significant ventricular

dysfunction. Baffle leak with left-to-right shunt

> 1.5:1, right-to-left shunt with arterial desaturation
at rest or with exercise, symptoms, and progressive
ventricular enlargement that is not amenable to device
intervention
Superior vena cava or inferior vena cava obstruction
not amenable to percutaneous treatment
Pulmonary venous pathway obstruction not amenable
to percutaneous intervention
Symptomatic severe subpulmonary stenosis.

Postarterial Switch Repair

Dr A Jatene first described the arterial switch repair in
which the great arteries are translocated to achieve their
anatomically correct positions and the coronary arteries
are reimplanted into the neoaorta.176 The most common
long-term issues postarterial switch repair in a young adult
with d-TGA are neoaortic root dilatation and neoaortic
valve regurgitation, since the current aortic valve, which
was originally a pulmonary valve, is now placed in a
high pressure circulation. Supravalvular pulmonary
stenosis often occurs at the arterial anastomotic sites.
Due to reimplantation of the coronary arteries into the
neoaorta, fibrosis and scarring at the anastomotic sites
of the coronaries may cause ischemia in young adults.
Coronary angiography or CTA is indicated when ischemia
is likely. Adults with intramural or single coronary arteries
have a higher mortality than those with a typical coronary
pattern. Among the long-term issues are supravalvular
stenosis and aortic aneurysm.

Echocardiography
Neoaortic dilatation and aortic regurgitation may develop
over time. Neoaortic constriction can occur at the site of
anastomosis. Patients with previous pulmonary artery
banding (although it is now rarely performed) are more
likely to have neoaortic root dilatation but it may not
necessarily progress on late follow-up. Risk factors for
aortic regurgitation include older age at the time of
presentation, presence of a VSD, and previous pulmonary
artery banding.177

Stress Echocardiography
Following atrial switch repair, stress echocardiography is
performed to screen for exercise-induced ischemia.

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

Cardiac Catheterization
Coronary ostial fibrosis leading to coronary ischemia
prompts early catheterization in those adults who have
undergone reimplantation of the coronaries into the base
of the neoaorta.

Magnetic Resonance Imaging/Computed

Tomography
These imaging modalities are helpful in assessment of
the neoaortic diameters, coronary artery ostial fibrosis
(that can lead to myocardial ischemia), and extracardiac
structures such as pulmonary artery stenosis.

Reoperation for D-Transposition of the Great

Arteries Postarterial Switch Repair
The Class I indications for surgery in d-TGA postarterial
switch repair include:
Symptomatic severe right ventricular outflow tract
obstruction (conduit obstruction) not amenable to
catheter-based intervention with a peak-to-peak
gradient > 50 mm Hg, or with concomitant severe
pulmonary regurgitation
Symptomatic myocardial ischemia that cannot be
relieved by catheter-based intervention in an adult
with a coronary artery abnormality
Severe symptomatic neoaortic valve regurgitation
Severe neoaortic root dilatation 55 mm.

Post-Rastelli Repair
The Rastelli procedure is performed in d-TGA that is
associated with a large VSD and significant pulmonary
stenosis. In this surgical repair, a right ventricle to
pulmonary artery conduit allows blood flow from the right
ventricle to reach the branch pulmonary arteries, usually in
patients with right ventricular outflow tract obstruction.178

Echocardiography
Echocardiography plays an important role in evaluation
of long-term complications including right ventricular
outflow tract (RVOT) obstruction, pulmonary conduit
obstruction with degeneration and calcification of the
pulmonary valve, residual shunts, and degree of pulmonary
and tricuspid regurgitation. The tricuspid regurgitant jet
velocity helps in estimation of the right ventricular systolic

1839

pressure, which is usually elevated in the setting of conduit

obstruction. Progressive tricuspid regurgitation with right
ventricular enlargement, hypertrophy, and failure are
bad prognostic signs indicating high risk of mortality. Left
ventricular outflow tract (LVOT) obstruction can occur at
any level in the long intraventricular baffle through the
VSD to aorta. Structural abnormalities of the great arterial
walls make these adults prone to aortic root dilatation and
aortic valve regurgitation.

Cardiac Catheterization
Cardiac catheterization determines the conduit gradient
accurately. Catheter-based interventions such as intraconduit percutaneous pulmonary valve implantation, and
dilation with or without stent implantation for relief of
branch pulmonary artery stenosis can be performed when
indicated.

Magnetic Resonance Imaging/Computed

Tomography
Prior to surgery, MRI/CTA may be helpful in assessment
of adults with significant conduit obstruction/leaks or
branch pulmonary stenosis.

Reoperation for D-Transposition of the Great

Arteries Post-Rastelli Repair
The ACC/AHA Class I indications for reoperation after
Rastelli procedure in the following settings are:3
Prior Conduit and/or Valve Replacement
Symptomatic adult with conduit obstruction with a
peak-to-peak gradient > 50 mm Hg
Subaortic (baffle) obstruction with a mean gradient
> 50 mm Hg and left ventricular hypertrophy
Severe symptomatic aortic regurgitation requiring
concomitant surgery.
Conduit Regurgitation
Symptomatic/decreased exercise tolerance
Severe right ventricular enlargement with severely
reduced right ventricular function
Severe tricuspid regurgitation.
Residual Ventricular Septal Defect
Increased left ventricular size from volume overload
Reduced right ventricular function from pressure
overload
Systolic pulmonary artery pressure > 50 mm Hg

1840

Section 6: Congenital Heart Disease

Right ventricular outflow tract obstruction with peak

instantaneous gradient > 50 mm Hg
Pulmonary artery pressure less than two thirds of
systemic pressure and responsive to pulmonary
vasodilators.

The Postoperative Adult

In addition to the residua and sequelae specific to the type
of surgery (atrial or arterial switch or Rastelli repairs) as
detailed above, the postoperative adult with d-TGA may
require the following surgeries:
Pacemaker Surgery with Epicardial Leads
In adults with significant residual intracardiac shunts
or systemic venous obstruction, epicardial leads are
the safest option.
Pulmonary Artery Stenosis or Branch Pulmonary Stenosis
Surgery is performed when interventional procedures
are not feasible in adults with severe pulmonary artery
stenosis or branch pulmonary stenosis.

Congenitally Corrected Transposition of

the Great Arteries (CCTGA)
Although far less common than d-TGA, congenitally
corrected transposition of the great arteries (CCTGA) can
sometimes go undiagnosed into adulthood, especially
in the absence of any associated defects. Adults with
unoperated CCTGA are often misdiagnosed in adulthood
and referred late to ACHD specialists for appropriate
management, despite having symptomatic systemic AV
valve regurgitation and impaired morphological right
ventricular (systemic) function.179
In addition to the ventriculoarterial discordance (the
great arteriesaorta and pulmonary arising from opposite
ventricles), there is atrioventricular discordance (the right
atrium drains into left ventricle through the mitral valve,
left atrium drains into right ventricle through the tricuspid
valve) due to ventricular inversion. The term corrected
refers to the physiologically normal direction of blood flow
due to double discordance, with two wrongs try to make
a right resulting in the morphological right ventricle
pumping into the aorta and the morphological left ventricle
into the pulmonary artery. It has been sometimes referred
to as l-transposition, because the morphological right
ventricle is to the left of the morphological left ventricle.
Levocardia with the apex of the heart in the left side of
the chest is most common, but some cases may have

mesocardia (apex in midline) or dextrocardia (apex in the

right side of the chest). The diagnosis of CCTGA should be
highly considered in adults with dextrocardia.
Heart failure is common by the fourth and fifth
decades and has been reported in 51% of adults with
associated defects versus in 34% without any associated
defects.180 The systemic right ventricular function is usually
impaired due to long-standing tricuspid (systemic AV
valve) regurgitation and influences the timing of surgery
in CCTGA with or without associated lesions.181 The
most common associated defect is an abnormality of the
systemic AV valve that may occur in up to 90% of the cases
(Ebstein-like, dysplastic).182,183 Other associated defects
include a perimembranous VSD in approximately 70% and
subvalvular pulmonary stenosis in approximately 40%. In
a multi-institutional study, Graham et al. reported aortic
regurgitation in 25% to 36% of adults with CCTGA.180 The
presence of a VSD and significant pulmonary stenosis may
contribute to cyanosis. A higher incidence of endocarditis
(11%) has been reported in these adults.184
Most adults are likely to get a pacemaker implantation,
since conduction abnormalities are so common in this
population with complete heart block occurring at the rate
of 2% per year.185

Echocardiography
Unfortunately, this diagnosis may be missed on echocardiography because of failure to identify the abnormal
position of the AV valves associated with ventricular
inversion. In addition, the 1% of adults with CCTGA without
associated defects are even more likely to go undiagnosed
into adulthood. Identifying the relationship of the great
arteries and defining the position of the morphological
tricuspid valve are essential for clinching the diagnosis on
echocardiography.
As seen in d-TGA, the two great arteries are parallel to
each other in the modified parasternal long-axis view. They
are seen in the same cross-sectional plane in the parasternal
short-axis views. The position of aorta is anterior, leftward,
and superior, while the pulmonary artery is rightward,
posterior, and inferior. Identification of the ventricular
morphology and positions of the AV valve that are best seen
in the apical four-chamber view remains critical in making
the diagnosis (Fig. 75.18). The AV valves always follow
their ventricles and, therefore, the tricuspid valve (referred
to as the systemic AV valve) is on the left side with the
morphological right (systemic) ventricle posing as the left
AV valve, while the mitral valve (referred to as the pulmonic

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

1841

Fig. 75.21: Transthoracic echocardiogram in congenitally corrected transposition of the great arteries (CCTGA) showing ventricular
inversion and tricuspid valve on the left side (apically placed in
relation to the mitral valve). (LA: Left atrium; LV: Left ventricle;
MB: Moderator band; MV: Mitral valve; RA: Right atrium; RV: Right
ventricle; TV: Tricuspid valve).
Source: With permission from Vijayalakshmi IB, Rao PS, Chugh
R, editors. A Comprehensive Approach to Congenital Heart Diseases. 1st ed. New Delhi: Jaypee Brothers Medical; 2013: 767.

Fig. 75.22: Transthoracic echocardiogram in congenitally corrected transposition of the great arteries (CCTGAs) showing that the
left ventricle pumps into the transposed pulmonary artery and
its branches (arrows) in a modified four-chamber view. (LV: Left
ventricle; RV: Right ventricle).

AV valve) is on the right side with the morphological

left (pulmonic) ventricle (Fig. 75.21). The systemic right
ventricle pumps into the aorta that is identified by its
candy cane appearance. The left ventricle pumps into
the pulmonary artery, which is identified by its right and
left branches in a modified apical four-chamber view
(Fig. 75.22). The complex shape of the morphological right
ventricle makes quantification of systemic ventricular
function by echocardiography more challenging.
Echocardiographic assessment of the systemic
ventricular function and degree of systemic AV valve
(morphological tricuspid) regurgitation are extremely
important since long-term outcomes including heart
failure and mortality are impacted by the inter-relationship
between the severity of regurgitation and the declining
systemic ventricular function. The morphology of the
systemic AV valve (morphological tricuspid), the dilatation
of its annulus (due to enlarging systemic ventricle), and the
presence of pacemaker wire may all contribute to failure of
leaflet coaptation and further progression of regurgitation.
Long-standing tricuspid regurgitation is a major risk factor
for deterioration of right ventricular function.181

the systemic ventricular contractile reserve can be made

by stress echocardiography that also allows assessment of
chronotropic insufficiency.

Exercise Testing with Stress Echocardiography

While treadmill stress testing provides an objective
assessment of functional capacity, a good estimation of

Magnetic Resonance Imaging

The best method for quantification of systemic ventricular
function is an MRI, which is currently the reference
standard. Since many adults with CCTGA have devices
(pacemakers or defibrillators), CTA is the next best option
when MRI cannot be performed.

Cardiac Catheterization
The role of cardiac catheterization is mainly for delineating
the coronary anomalies or atherosclerosis prior to surgery.
It also allows evaluation of pulmonary vascular reactivity
in adults with significant pulmonary hypertension.

Surgery for CCTGA

Referral to cardiac surgery should be sought for early
systemic AV valve replacement in symptomatic adults or
before the systemic ventricular ejection fraction declines
below 45%.186 It is more likely to occur in adults with
tricuspid valve abnormalities with progressive tricuspid
regurgitation. Other echocardiographic signs that should
alert the clinician are an enlarging right ventricle, left atrial

1842

Section 6: Congenital Heart Disease

enlargement, progressive pulmonary hypertension, and

the appearance of atrial arrhythmias.
The key ACC/AHA Class I indications for surgery in
adults with CCTGA are the following:3
Unrepaired CCTGA or post-tricuspid valve repair with
severe AV valve regurgitation
Left ventricle is functioning at systemic pressures
postbiventricular repair
Progressive moderate to severe aortic regurgitation
leading to ventricular dilatation and impaired function
Conduit obstruction with
Markedly high right ventricular pressures and/or
impaired morphological right ventricular function
after anatomical repair
Markedly elevated left ventricular pressures in an
adult with a nonanatomical correction.

of the tricuspid valve. Following surgery, the prosthetic

systemic AV valve (that replaced the tricuspid valve) should
be monitored by echocardiography along with periodic
assessment of systemic ventricular function, pulmonary
conduit function (post-Rastelli repair), degree of aortic
regurgitation, progression of pulmonary hypertension,
and evaluation of residual septal defects.
After pacemaker implantation, pacing has been shown
to cause a septal shift that may aggravate dilatation of the
systemic ventricle and precipitate worsening of systemic
AV valve regurgitation. Hence, these should be watched
more closely after pacemaker implantation. A synopsis of
echocardiographic assessment of CCTGA is reviewed in
Table 75.17.

The Postoperative Adult

Truncus arteriosus is also known as truncus arteriosus

communis and common aorticopulmonary trunk.
Approximately 80% of the infants survive at 1 year without
surgical intervention.187,188 Since mortality without surgery

Tricuspid valve repair is less likely to be a long-term

solution in many adults due to abnormal morphology

Truncus Arteriosus

Table 75.17: Congenitally Corrected Transposition of the Great Arteries

Associated defects

Ventricular septal defectperimembranous (6080%)

Pulmonary, subpulmonary or supravalvular stenosis

Tricuspid (left AV valve) anomalyEbsteins anomaly, Ebstein-like or dysplastic morphological tricuspid valve

Tricuspid (left AV valve) regurgitation

Patent foramen ovale

Atrial septal defect

Aortic or subaortic stenosis

Aortic regurgitation

Coarctation of aorta

Conduction abnormalitiescomplete heart block

Dextrocardia

Coronary artery anomalies (inverted coronaries due to ventricular inversion)

Echocardiographic assessment

Systemic ventricular (morphological right ventricle) size and function

Degree of systemic AV (tricuspid) valve regurgitation

Identification of associated defects

Postoperative

Systemic ventricular (morphological right ventricle) size and function

Degree of systemic AV (tricuspid) valve regurgitation

Rule out residual shunt lesions

Conduit stenosis (in Rastelli repair)

Aortic regurgitation

Thromboembolic risk

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

is very high in infancy, most adult survivors have had

previous repairs. This defect is characterized by a single
great artery with a single semilunar valve. The single great
arterial vessel arises from the base of the heart giving
rise to the coronary, pulmonary, and systemic arteries.
It is larger than the aorta with poorly developed sinuses
of Valsalva, and has a single semilunar valve that is also
known as the truncal valve. A large nonrestrictive,
subarterial VSD is required for intermixing of blood
between the two ventricles. While the VSD is usually
described as membranous, it has also been thought to
result from absence of the distal pulmonary infundibulum
(both septal and free wall).189 Since the VSD is roofed
by the truncal valve, there is poor truncal support, and
truncal valve regurgitation occurs.31 The truncal valve may
be trileaflet in the majority (nearly 70%), quadricuspid in
around 20% to 25%, and bicuspid in around 7% to 9%.189
When the truncal valve is quadricuspid, it is a combination
of the three leaflets of the aortic valve and pulmonary
leaflet tissue.189 The pulmonary arteries arise from the
posterolateral aspect of the common arterial trunk with
separate origins for the right and left branches. A right
aortic arch is present in 20% to 30% of the cases.31,189

Table 75.18: Truncus Arteriosus

Associated defects

Right aortic arch30%

Interrupted aortic arch

Abnormal truncal valve

Trileaflet (most common)
Quadricuspid
Bicuspid

Truncal valve stenosis/regurgitation

Left superior vena cava

Secundum atrial septal defect

Absence of branches of the pulmonary artery

Anomalous origin of the coronary artery

Echocardiographic assessmentpostoperative

Biventricular size and function

Aortic root size

Aortic (truncal) valve regurgitation

Rule out residual shunts

Assessment of any associated defects

Conduit patency or regurgitation

1843

The origins and epicardial course of the coronaries are

variable and often anomalous. The left coronary usually
arises from the left posterior aspect of the truncus while
the right coronary often arises from the right anterior
aspect of the truncus.31

Echocardiography
On transthoracic echocardiography, the parasternal longaxis view shows the truncal root, valve, and sometimes the
origins of the pulmonary arteries. The apical four-chamber
view is helpful in demonstrating a VSD and an overriding
truncus. The suprasternal views allow identification of a right
aortic arch and coarctation of the aorta. Color Doppler is
useful in identifying truncal valve stenosis or regurgitation,
presence of pulmonary stenosis at the origin of the branches,
and for determining the direction of the shunt. An agitated
saline contrast study is performed to rule out right-to-left
shunts and to define the right ventricular outflow tract in
the parasternal short-axis views or the subcostal views.190,191
Associated defects are coarctation of aorta, interrupted aortic
arch, right aortic arch, patent ductus arteriosus, ASD, and
tricuspid stenosis. In the absence of significant pulmonary
stenosis, pulmonary hypertension develops. A synopsis
of echocardiographic assessment of truncus arteriosus is
reviewed in Table 75.18.

1844

Section 6: Congenital Heart Disease

Magnetic Resonance Imaging/Computed

Tomography
Since extracardiac malformations occur in up to 40% of these
individuals, MRI or CTA are very useful for assessment of
aortic arch anomalies and branches pulmonary stenosis.

Cardiac Catheterization
Besides evaluation of the coronaries, cardiac catheterization is performed in conjunction with stent placement
when there is conduit obstruction.

Surgery for Truncus Arteriosus

Surgery is indicated for conduit failure not amenable to
intervention, severe truncal regurgitation, and enlarging
truncal dimensions.

The Postoperative Adult

Improvements in early diagnosis and primary neonatal
repair have allowed better survival.192 Palliation with
pulmonary artery banding (which delays primary surgery
to a later age) is no longer believed to offer any benefit and
is associated with high mortality rates.
McGoon, Rastelli, and Ongley performed the first
successful surgical repair of truncus arteriosus in 1967
using an aortic homograft with an aortic valve to establish
continuity from the right ventricle to the pulmonary
artery.193 The surgical approach has been modified over
the past four decades. In these operations, reconstruction
of the right ventricular outflow tract is necessary to
establish a separate pulmonary circulation. However,
there continues to be a need for reoperations. In a recent
study, the actuarial survival at 30 years was reported as
approximately 83% with a reoperation rate of 76% among
survivors, and truncal valve replacement was required in
20% during long-term follow-up.194
Most adults seen in our practice have undergone a
homograft or xenograft repair. Homografts are preferred
because of lower rates of stenosis and longer durability
with long-term freedom from reoperations. Smaller
conduit size at the time of the initial surgery also leads
to early reoperation because the child outgrows it during
developing years. Other reasons for graft failure are
conduit calcification, stenosis, and infective endocarditis.
Catheter-based interventions for conduit valve obstruction
may reduce some of the reoperations by allowing the

conduits to function for a longer duration. Another

major indication for reoperation that impacts longterm outcomes/mortality is truncal valve regurgitation
requiring replacement or repair.195197
The long-term residua and sequelae assessed
by echocardiography are progressive homograft or
conduit obstruction/regurgitation, truncal or prosthetic
valve regurgitation/stenosis, residual VSD, impaired
biventricular function, truncal root dilatation, and
pulmonary hypertension. These adults may have limitations in functional capacity, and are at risk of developing
heart failure, cardiac arrhythmias, and infective endocarditis.

Double Outlet Right Ventricle

A key feature in this heterogenous group of defects
described as the double outlet right ventricle (DORV) is that
the aorta and the pulmonary artery arise primarily (>50%)
from the right ventricle.198 Dr Joseph Perloff eloquently
states, It has been argued that the malformation is
virtually unclassifiable because of its excessively complex
and diverse anatomy.31
The clinical patterns in this spectrum of defects
vary based on the size/location of the VSD, presence or
absence pulmonary stenosis, ventricular size, status of the
atrioventricular valves (AV), and the degree of pulmonary
vascular resistance. Dr Perloff describes the two most
common clinical types: (a) subaortic VSD without
pulmonary stenosis and (b) subpulmonary VSD without
pulmonary stenosis, with each one having either low or
high pulmonary vascular resistance.31

Echocardiography
Most individuals with DORV are operated in childhood.
Echocardiography in an unoperated adult focuses on the
relation of the aorta and pulmonary artery, the position
and relation of the VSD, any obstruction (subpulmonic
or subaortic), presence of pulmonary hypertension, and
identification of a constellation of associated defects.
Irreversible pulmonary vascular disease with Eisenmenger
physiology will preclude surgical repair in adulthood, as
seen in adults with large subaortic or subpulmonic VSDs
without pulmonary stenosis.199 The best views are the
parasternal and subcostal views for identification of the
origin of both great arteries from the morphological right
ventricle.

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

Surgery for Double Outlet Right Ventricle

Depending upon the type, morphology, and circulation,
in most cases a Rastelli repair is performed. A conduit
from right ventricle (RV) to pulmonary artery (PA), and
another conduit from left ventricle to aorta restores
the physiological circulation. Other types of repairs are
palliative single ventricle (Fontan) surgery, arterial switch
type repair (in subpulmonary VSD with transpositionlike physiology), or intracardiac repair as in tetralogy of
Fallot (for subaortic VSD with pulmonary/subpulmonary
stenosis). Concomitant surgical procedures are performed
for associated defects.200,201

The Postoperative Adult

The postoperative adult with a Rastelli repair has an
external conduit directing blood from the right ventricle
into the pulmonary artery and an internal connection
between the left ventricle and aorta through the VSD.
Follow-up echocardiograms are performed to rule out
residual VSD, assess degree of AV valve regurgitation,
conduit obstruction (right ventricle to pulmonary artery
valve conduit), kinking of the left ventricle to aorta
conduit, and status of associated defects (such as COA).
The indications for conduit replacement post-Rastelli
repair are discussed previously in the section on d-TGA.

Stress Echocardiography
Stress echocardiography with treadmill stress testing
is valuable in assessing exercise intolerance related to
residual hemodynamically significant lesions, exerciseinduced arrhythmias, and chronotropic incompetency.

Cardiac Catheterization
In limited cases, cardiac catheterization is performed to
assess the pulmonary vascular resistance, and hemodynamic
status prior to repair and before transcatheter pulmonary
valve implantation in the patients with RV to PA conduits.

Magnetic Resonance Imaging/Computed

Tomography
MRI/CTA is performed when transthoracic or transesophageal echocardiography are inadequate for assessment of the gradient across the conduits. These imaging
modalities allow delineation of the course of the coronaries,
aortic root (dilatation/aneurysm), arch (COA), pulmonary

1845

arteries, and the relationship between the chambers, great

vessels, and AV valves.202,203

Univentricular Heart
The spectrum of univentricular hearts comprises multiple
defects that are not amenable to biventricular repair and
rely on the dominant ventricle to support both the systemic
and pulmonary circulations. CHDs that present with the
single ventricle physiology are tricuspid atresia, mitral
atresia, hypoplastic right or left ventricle, and double inlet
left ventricle. The clinical manifestations and prognosis for
an adult with any form of a univentricular heart are related
to the pulmonary artery blood flow/pressure, which is
affected by the presence and degree of pulmonary stenosis,
and the level of pulmonary vascular resistance. The
majority of the adult survivors have undergone palliative
repairs: systemic to pulmonary artery shunt and/or Glenn
procedure, or Fontan procedure.

Tricupsid Atresia and the Post-Fontan Adult

The most common presentation seen in the adult congenital heart disease clinics is tricuspid atresia (imperforate
tricuspid valve) with a large dominant morphological
left ventricle and a hypoplastic right ventricle. The great
arteries may exhibit ventriculoarterial concordance or
discordance (complete transposition of the great arteries).
Both arteries may arise from the same chamber or the
pulmonary artery may arise from the rudimentary right
ventricle. There may be an atrial and/or ventricular septal
defect. The majority of these people have undergone
Fontan surgery in childhood.
The classic Fontan is a right atrial-to-pulmonary
artery connection in a patient with tricuspid atresia.204
Over the years, there have been many variations of the
Fontan repair (as shown in Figs 75.23A to C) resulting in
complete or near-complete separation of the pulmonary
and systemic circuits (in those who are not candidates
for biventricular repair). In these patients, the pulmonary
blood flow requires unobstructed pulmonary arteries, low
filling pressure of the single ventricle, normal ventricular
function, low pulmonary vascular resistance, and absence
of aortic valve regurgitation. Modifications of the surgical
techniques have been associated with more favorable
outcomes. There are several variables contributing to
overall early and late failure of the Fontan. Hypoplastic left
heart syndrome with a right-sided tricuspid valve (as the
predominant atrioventricular valve) has been shown to
increase the early risk more than threefold.205

1846

Section 6: Congenital Heart Disease

Figs 75.23A to C: The Fontan procedure and its common

modifications. (A) Classic right atrial to pulmonary artery (RA to
PA) connection, (B) lateral tunnel Fontan, (C) extra cardiac conduit
(ECC) Fontan. (HRV: Hypoplastic right ventricle; IVC: Inferior vena
cava; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA:
Right atrium; SVC: Superior vena cava).

Echocardiography
Echocardiographic assessment of the post-Fontan adult
looks into the important determinants that have a longterm effect on the systemic ventricular size and function.
The tricuspid atresia is best seen in the apical fourchamber view (Fig. 75.24). These include systemic AV
valve morphology, degree of valvular regurgitation, and
the presence of subaortic stenosis.
The cavoatrial, atrial septal, or ventricular septal
defects should be identified, since they contribute to the
persistence of cyanosis. In some individuals, fenestrated
atrial septum or an adjustable ASD are a necessity. In these
cases, the gradient across the fenestration or shunt should

be measured. The best views are the apical four-chamber

or the subcostal views. For visualizing the atriopulmonary
connection (right atrium to pulmonary artery), one must
obtain modified short-axis suprasternal views with right
angulation to expose the superior vena cava. Color Doppler
and pulsed Doppler interrogation of the flow are necessary
to rule out an obstruction. In case of an obstruction, color
Doppler will demonstrate flow turbulence, and pulsed
wave Doppler will show high velocity flow over 1 m/s with
loss of respiratory variation (normal flow is low velocity
with respiratory variation giving it a biphasic pattern).206
Spontaneous contrast seen in the Fontan circuit
represents slow blood flow in the pathway. Right atrial

Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease

Fig. 75.24: Transthoracic echocardiogram showing hypoplastic

right ventricle (RV), imperforate tricuspid valve in an adult with
tricuspid atresia, as seen in an apical four-chamber view. (ASD:
Atrial septal defect; LA: Left atrium; LV: Left ventricle; RA: Right
atrium).

thrombus formation is common with a varying incidence

depending upon the type of surgical technique and the
number of postoperative years. It carries a significant risk
of death, especially in the setting of pulmonary embolism
that is capable of causing clinical instability. Tsang et al.
reported a right atrial thrombus in 12% of adults with the
Fontan circulation, and an associated overall mortality
rate of 18%.207 The diagnosis is made incidentally in some
adults who are asymptomatic. In others, the thrombus
may persist despite anticoagulation. There have been
various studies addressing antiplatelet therapy versus
anticoagulation in this population. Even in patients
with extracardiac conduit (ECC) Fontans, antiplatelet
therapy alone failed to prevent the rate of early or late
thromboembolic events. Their bleeding risk was similar
to that when anticoagulation therapy was given alone or
in association with antiplatelet drugs.208 Although the
debate continues, the current recommendation is that
adults with atrial arrhythmias or those with residual ASDs/
fenestrations should be given anticoagulation therapy.

Transesophageal Echocardiography
The onset of heart failure symptoms with hepatic
congestion and jugular venous distension (in those
without a Glenn shunt) or the development of new atrial
intractable arrhythmias prompts a search for conduit
stenosis. Conduit obstruction may be partially assessable

1847

by transthoracic echocardiography. Therefore, for conduit

stenosis (other than in right atrium to right ventricular
connection), TEE is the procedure of choice for examining
the entire Fontan circuit.209 It allows more detailed
examination for potential complications that involve
the right atrial portion of the Fontan such as cavoatrial
shunting, atrial septal shunting, and atrial to pulmonary
artery obstruction. TEE is also indicated to rule out a
thrombus in an adult with a thromboembolic event and
for assessment of anatomical abnormalities of the Fontan
pathway. TEE is more likely to detect atrial and pulmonary
thrombi that could not be seen by transthoracic imaging in
patients who have undergone the Fontan operation.209,210
Three-dimensional echocardiography may augment the
detection or exclusion of thrombi in the Fontan conduit.211
A synopsis of echocardiographic assessment of the
post-Fontan adult is reviewed in Table 75.19.

Magnetic Resonance Imaging/Computed

Tomography
MRI is useful for investigating extracardiac structures such
as the patency of the conduits/cavopulmonary pathways,
and ventricular and valvular functions.212 MRI and CTA
provides information about the systemic and pulmonary
arterial/venous anatomy in addition to intracardiac
complex defects, ventricular volumes, mass, ejection
fraction, and regurgitant fractions.

Cardiac Catheterization
The etiology of oxygen-unresponsive hypoxemia in adults
with Fontan is determined by cardiac catheterization that
can identify potential causes including persistent Fontan
fenestration, systemic venous-to-pulmonary venous
collaterals, and pulmonary arteriovenous malformations.
In heart failure patients, the cause of volume retention
may be worsening ventricular function, increasing Fontan
circuit pressure, and resistance that exaggerate the rightto-left shunts. Catheter-based closure of residual shunts by
coils or ASD devices may relieve the symptoms.
Cardiac catheterization plays an important role in
assessment of protein-losing enteropathy (PLE) in the postFontan adult. Causes of increased resistance to effective
pulmonary flow, such as obstruction to pulmonary
flow at the pulmonary artery or venous levels, AV valve
stenosis or regurgitation may contribute to progressive
PLE. The aortic-pulmonary collaterals can be also be

1848

Section 6: Congenital Heart Disease

Table 75.19: Univentricular Heart Post-Fontan Tricuspid Atresia

Associated defects

Univentricular heart/single ventricle physiology with a hypoplastic right ventricle

Atrial septal defect

Ventricular septal defect

Transposition of the great arteries

Echocardiographic assessmentpostoperative

Systemic (usually left) ventricular dysfunction

Right atrial enlargement

Thrombus in the right atrium

Atrioventricular (AV) valve regurgitation

Conduit obstructionobstruction of the Fontan connectionusually due to right pulmonary vein compression from an
enlarged right atrium or from conduit obstruction.

delineated by aortography. For these adults, creation or

enlargement of an atrial septal communication (ASD or
fenestration) may decrease the central venous pressure.
It is also a prerequisite to pulmonary vasodilator therapy
for pulmonary hypertension or heart transplantation
evaluation.

CONCLUSIONS
Echocardiography plays a vital role in diagnosis and
follow-up of adults with congenital heart defects. Every
effort should be made to acquire the previous medical
records, especially operation notes and imaging studies,
in order to understand the blueprint of each individuals
unique anatomy and physiology. It is essential to perform
a complete examination and be flexible in performing
modified views to acquire the desired images. Equally
important is our understanding about the indications for
applying the appropriate imaging modalities. Since our
eyes see what the mind knows, an echocardiographer
must have knowledge of the underlying anatomy, types
of surgeries, associated defects, long-term residua, and
sequelae in order to provide a comprehensive assessment
that will direct timely and appropriate treatment.

ACKNOWLEDGMENTS
My deepest gratitude to Dr John S Child and Dr Navin C
Nanda for their inspiration and the gift of knowledge; to
Robert Reber for his expertise as an audiovisual engineer;
to Ms Hovey Lee for the extensive literature searches;
to the patients and sonographers (Paul Junkel, Terri

McAnallen, Albert Amoranto, and Janae Johnson) at

the Echocardiography Laboratory, Kaiser Foundation
Hospital, Panorama City, California, for their innumerable
clinical contributions and support; and to my dear
daughter Tanisha for her unconditional love, patience, and
understanding.

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CHAPTER 76
Echocardiographic Evaluation for
Acquired Heart Diseases in Childhood
Jie Sun, Rula Balluz, Lindsay Rogers, Shuping Ge

Snapshot

Infective Endocarditis

Modified Duke Criteria for the Diagnosis of Infective

Endocarditis

Echocardiographic Findings

Complications of Infective Endocarditis

INTRODUCTION
As with congenital heart disease, acquired heart diseases are
also common in children. The spectrum includes infective
endocarditis, rheumatic heart disease (RHD), Kawasaki
disease, and other cardiovascular involvement related
to other systems or organs, such as hypertension, sickle
cell disease, chronic renal disease, and cancer survivors.
Echocardiographic diagnosis and assessment have become
a routine practice to evaluate these patients initially and
possibly longitudinally to facilitate diagnosis, prognosis, and
guidance for treatment. In this chapter, we will discuss three
of the most common acquired cardiovascular diseases in
children, namely, infective endocarditis, RHD, and Kawasaki
disease, and further in-depth discussion can be found in
comprehensive texts and other sources.

INFECTIVE ENDOCARDITIS
Infective endocarditis (IE) is an uncommon but lifethreatening infection. Despite advances in diagnosis,
antimicrobial therapy, surgical techniques, and manage
ment of complications, patients with IE still have high
morbidity and mortality rates related to this condition.

Rheumatic Heart Disease

Jones Criteria, Updated 1992

Kawasaki Disease

Coronary Ectasia and Aneurysms by Echocardiography

IE can happen on normal heart or abnormal heart.

The patients with certain conditions are at increased risk
to develop IE, such as congenital heart disease, acquired
heart disease, indwelling catheters, intracardiac devices,
prostheses, immunodeficiency, and intravenous drug
abuse. The true incidence of IE in pediatric population
or adults is unknown. IE accounts for about 1 in 1,280
pediatric admissions per year.1 The vegetation can occur
on valve leaflets, the walls, chordae, paraprosthetic tissue,
shunts, or conduits. There is an increased proportion
in repaired congenital heart disease due to increased
population in this group. The clinical diagnosis of IE is
based upon a combination of clinical features, positive
blood culture, and evidence of vegetation by imaging or
pathology.
Turbulent blood flow produced by congenital or
acquired heart disease, such as flow from high- to
low-pressure chambers or across a narrowed orifice,
traumatizes the endothelium. This creates a predisposition
for deposition of platelets and fibrin on the surface of the
endothelium, which results in nonbacterial thrombotic
endocarditis. Invasion of the bloodstream with a microbial

Chapter 76: Echocardiographic Evaluation for Acquired Heart Diseases in Childhood

species that has the pathogenic potential to colonize this

site can then result in IE. Bacteria can be entrapped and
colonize to initiate focus of infection; platelets and fibrin
are deposited forming vegetation. It almost always involves
a valve and can cause significant valve insufficiency or
perforation. Its complications include congestive heart
failure, embolization, abscess, heart block, mycotic
aneurysm, pericarditis, or myocarditis.
Subacute presentation of IE is the more common
type. Its presentation can be insidious, nontoxic, and
with immune phenomena. The most common pathogen
is viridans strep; other pathogens such as fungal, HACEK
group, and coagulase-negative Staphylococcus aureus
can also be the cause of subacute cases. The extracardiac
manifestations can be splenomegaly, hematuria, immune
phenomena including Roth spot, splinter hemorrhages,
Janeway lesion, and Osler node. The extracardiac
manifestation in children with subacute IE is less common
than in adults. The patients with acute presentation of IE
can present with high fever and appear very toxic. The
acute IE mostly happens in postoperative patients or
patients with an indwelling catheter. The most common
cause of acute IE is Staphylococcus aureus.
The clinical diagnosis of IE is established based on the
modified Duke criteria.2 Two major or one major and three
minor criteria or five minors are required to establish a
diagnosis of IE.
The pathological criteria for IE include micro-organism
demonstrated by culture or histology of a vegetation, or
in a vegetation that has embolized, or in an intracardiac
abscess or pathological lesionsvegetation or intracardiac
abscessconfirmed by histology showing active IE.

MODIFIED DUKE CRITERIA FOR

THE DIAGNOSIS OF INFECTIVE
ENDOCARDITIS
Major Criteria
Positive Blood Culture for Infective
Endocarditis

Typical micro-organism for infective endocarditis from

two separate blood cultures: Viridans streptococci,
Staphylococcus aureus, Streptococcus bovis, HACEK
group (Hemophilus spp. Actinobacillus actinomycete
mcomitans, Cardiobacterium hominis, Eikenella spp.,
and Kingella kingae) or

1857

Community-acquired enterococci, in the absence of a

primary focus
Persistently positive blood culture, defined as
recovery of a micro-organism consistent with infective
endocarditis from blood cultures drawn more than
12 hours apart or all of three or a majority of four or
more separate blood cultures, with first and last drawn
at least 1 hour apart
Single positive blood culture for Coxiella burnetti or
phase I antibody titer > 1:800.

Evidence of Endocardial Involvement

Positive Echocardiogram for Infective Endocarditis:
Transesophageal echocardiography (TEE) recom
mended as first test in the following patients: (a)
prosthetic valve endocarditis; or (b) those with at
least possible IE by clinical criteria; or (c) those
with suspected complicated IE such as paravalvular
abscess. Transthoracic echocardiography (TTE)
recommended as first test in all other patients.
Definition of positive findings: Oscillating intracardiac
mass, on valve or supporting structures, or in the
path of regurgitant jets, or on implanted material, in
the absence of an alternative anatomical explanation
or myocardial abscess or new partial dehiscence of
prosthetic valve.
New valvular regurgitation: Increase or change in preexisting murmur not sufficient.
Minor Criteria:
Predisposing heart condition or intravenous drug use
Fever 38.0C (100.4F)
Vascular phenomena: Major arterial emboli, septic
pulmonary infarcts, mycotic aneurysm, intracranial
hemorrhage, conjunctival hemorrhage, and Janeway
lesions
Immunological phenomena: Glomerulonephritis,
Oslers nodes, Roth spots, and rheumatoid factor
Positive blood culture not meeting major criterion as
noted previously (excluding single positive cultures
for coagulase-negative Staphylococci and organisms
that do not cause endocarditis) or serological evidence
of active infection with organism consistent with
infective endocarditis.

ECHOCARDIOGRAPHIC FINDINGS
Echocardiography should be performed in any patient sus
pected of having IE to allow earlier diagnosis, treatment,

1858

Section 6: Congenital Heart Disease

and prevent complications. The echocardiographic appear

ance of vegetations, noninfectious thrombi, and other
intracardiac masses may be indistinguishable. The incidental
echocardiographic finding of a mass in a patient who has
no associated clinical suspicion of IE likely warrants further
investigation and follow-up but should not be considered a
vegetation without supporting clinical features.
The accuracy of TTE for detecting vegetations in IE has
been evaluated in a variety of studies. Studies show that
TTE had a mean sensitivity of 79% for the detection of
vegetations.3 There is generally good agreement between
the location and identity of large vegetations on TTE and
pathological findings from autopsy or surgery.4 The role of
the more invasive and expensive TEE in the diagnosis of
IE stems from its high sensitivity in detecting and defining
valve vegetations. The sensitivity of TEE is substantially
higher (above 90%) than the values achieved with the
transthoracic approach.5

Vegetation Location
The morphology of IE vegetations is dependent on the
location of the endothelial lesion and always follows the
pathological bloodstream. When IE occurs in association
with ventricular septal defect, the vegetation is typically
visualized on the right ventricular aspect of the septum
and/or on the site where the high-velocity jet strikes the
right ventricular free wall. In the case of patent ductus
arteriosus, the vegetation may float through the pulmonary
artery. In patients with regurgitation of atrioventricular
valves, the vegetation is located on the atrial side

Fig. 76.1: Mitral valve vegetation. A vegetation involves the posterior

leaflet of mitral valve (white arrow). A perforation with color flow signals moving through it is noted (red arrow). Mitral valve insufficiency
is present (yellow arrow). This patient had cerebral embolism.

(Fig. 76.1). When the aortic valve is affected, perforation

through the annulus into the myocardium or into either
atria is possible. Newly acquired AV block together with
clinical suspicion of IE may be a strong indicator for the
presence of para-aortic ring abscess. Heart conditions
most associated with IE were unrepaired ventricular septal
defect, mitral regurgitation, and bicuspid aortic valve.6 In
patients who undergo palliative surgery, infected aorta to
pulmonary artery shunts predominate. In those who have
corrective surgery, the most common sites for developing
IE include right ventricle to pulmonary artery valved
conduits, prosthetic valves, and ventricular septal defect
patch closure. Patients with indwelling catheters such as
central lines can also develop line infection or associated
vegetation (Fig. 76.2).

Vegetation Size and Embolic Risk

In general, a larger vegetation size appears to be predictive
of embolic risk. In a study of 105 patients with IE, patients
with a vegetation diameter above 10 mm had a significantly
higher incidence of embolic events than did those with
smaller vegetations (47 vs 19%, P < 0.01).7 The association
was particularly strong in patients with mitral valve
endocarditis. Vegetation size did not appear to predict
other complications such as severe heart failure and death
and was not related to the location of endocarditis or type
of organism.
A series of 211 patients with 28 embolic events found
that vegetation size was associated with embolic risk for
staphylococcus and mitral valve vegetations, but was not

Fig. 76.2: Inferior vena cava (IVC) vegetation. IVC vegetation

(black arrow) in a patient who had an indwelling central line causing direct trauma of endothelium.

Chapter 76: Echocardiographic Evaluation for Acquired Heart Diseases in Childhood

associated with embolic risk for streptococcus or aortic

vegetations.8
Vegetation mobility confers incremental risk beyond
vegetation size. In a series of 178 patients with definite
IE who underwent TEE, the incidence of embolism, as
diagnosed by cerebral and thoracoabdominal CT scans,
was higher when vegetation size was 15 mm and when
the vegetation was moderately or severely mobile (62 vs
20% for low mobility).9 Embolic events were particularly
frequent when both severely mobile and very large
vegetations were present. Embolic risk generally falls with
time after institution of appropriate antibiotic therapy.

COMPLICATIONS OF INFECTIVE
ENDOCARDITIS
In addition to its role in diagnosing IE, echocardiography is
important for recognizing the intracardiac complications
associated with IE including regurgitant valve lesions,
chordal rupture, valve perforation, prosthetic dehiscence,
and paravalvular leak. Vegetations may also extend to
the outside of valve into surrounding structures to cause
abscess, fistula, or pseudoaneurysm formation.

Negative Result
The implications of an initial TEE examination that fails
to show vegetations in a patient with suspected IE should
be carefully considered. Although TEE cannot definitively
rule out IE, its high diagnostic sensitivity results in a
low probability of the disease when negative results are
obtained in a patient with an intermediate likelihood of
the disease. However, repeat examination is important,
particularly in patients at high risk for IE. Although the rate
of false-negative TEE examinations in patients with IE is
low, negative results confer an obligation of careful followup with exercise of sound clinical judgment.

Infective Endocarditis Prophylaxis

Recommended for Invasive Dental
Procedure10

Prosthetic cardiac valve or prosthetic material used

for cardiac valve repair
Previous IE
Unrepaired cyanotic congenital heart defect (CHD),
including palliative shunts and conduits
Completely repaired congenital heart defect with
prosthetic material or device, whether placed by
surgery or by catheter intervention, during the first
6 months after the procedure
Repaired CHD with residual defects at the site or
adjacent to the site of a prosthetic patch or prosthetic
device (which inhibit endothelialization)
Cardiac transplantation recipients who develop
cardiac valvulopathy
Except for the conditions listed above, antibiotic
prophylaxis is no longer recommended for any other form
of CHD.

RHEUMATIC HEART DISEASE

Rheumatic fever is an acute inflammatory illness that
occurs following an upper respiratory infection with
group A b-hemolytic streptococci (GAS). Rheumatic fever
particularly affects children 515 years old. It is the most
common form of acquired heart disease in children and
young adults throughout the world. It is a rare disease
in the United States. However, several outbreaks were
reported in certain geographical areas.11 The treatment
of strep pharyngitis shifts to nonrheumatogenic strains
of GAS. Its presentation is less classic. The significance
of rheumatic fever is chronic rheumatic valvar disease
including mitral regurgitation, aortic regurgitation, mitral
stenosis, or aortic stenosis. It usually takes over decades
from valvar regurgitation to valvar stenosis.

JONES CRITERIA, UPDATED 199212

Infective Endocarditis Prophylaxis

In patients with underlying cardiac conditions associated
with the highest risk of adverse outcome from IE, IE
prophylaxis for dental procedure is reasonable. In patients
with conditions associated with the highest risk of adverse
outcome from endocarditis, giving prophylaxis for dental
procedures is reasonable.

1859

Major Criteria

Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules

Prophylaxis is reasonable because endothelialization of prosthetic material occurs within 6 months after the procedure.

1860

Section 6: Congenital Heart Disease

Minor Criteria
Arthralgia
Fever
Elevated acute phase reactants
Erythrocyte sedimentation rate
C-reactive protein
Prolonged PR interval
Plus supporting evidence of an antecedent group A
streptococcal infection:
Positive throat culture or rapid strep test
Elevated or rising streptococcal antibody titers

Aortic regurgitation occurs in approximately 2025%

of patients with acute rheumatic carditis, usually in
combination with mitral regurgitation. Isolated aortic
regurgitation occurs in approximately 5% of patients with
acute rheumatic carditis14 (Fig. 76.4).

Chronic Rheumatic Heart Disease

The mitral valve is the predominant valve affected during

acute carditis.13 Characteristic changes include annular
dilation and chordal elongation, leading to prolapse
of the anterior leaflet. As a result, the leaflets no longer
coapt appropriately and there is a regurgitant orifice.
In rheumatic carditis, the regurgitant jet is typically
directed toward the posterolateral wall of the left atrium,
causing thickening and calcification of the endocardium
(Fig. 76.3). It is important to differentiate rheumatic mitral
prolapse from mitral prolapse seen in Barlows syndrome.
In rheumatic fever mitral carditis, only the coapting
portion of the anterior leaflet prolapses and there is no
billowing of the medial portion. In contrast, billowing of
the posterior or both leaflets of the mitral valve occurs
in Barlows syndrome (mitral valve prolapse). Another
differentiating feature is that chordal rupture frequently
occurs with Barlows syndrome, but rarely in patients with
rheumatic mitral valve disease.

Chronic mitral regurgitation is the most common form of

RHD in children and adults.
Mitral valve stenosis usually occurs in the third to
fifth decade of life.15 The natural history of rheumatic
mitral valve disease can begin with regurgitation, to
complete resolution of regurgitation without evidence
of heart disease, and then on to later development of
clinically significant mitral stenosis, and/or regurgitation
when patients reach adulthood.16 Characteristic changes
occur to the mitral valve apparatus in rheumatic fever,
including thickening and the scarring of the valve leaflets,
commissures, and chordae tendineae. Fusion of the mitral
cusps and chordae tendineae leads to thickening and
shortening of these structures, resulting in a mitral orifice
that is restricted in size and shaped like a funnel. Women
are more likely than men to develop rheumatic mitral
stenosis.17
On echocardiography, patients with rheumatic mitral
stenosis have thickened echodense leaflets, commissural
and/or chordal fusion, and abnormal diastolic leaflet
excursion resulting in a bent knee or hockey stick
appearance of the anterior leaflet. Mitral stenosis and
regurgitation may coexist.

Fig. 76.3: Mitral valve insufficiency. Mitral valve insufficiency

(white arrow) in a patient with acute rheumatic heart disease.

Fig. 76.4: Aortic insufficiency. Aortic insufficiency (white arrow) in

a patient with acute rheumatic heart disease.

Acute Valvulitis

Chapter 76: Echocardiographic Evaluation for Acquired Heart Diseases in Childhood

Like mitral valve stenosis, aortic valve stenosis is a

form of chronic RHD and occurs about 2040 years after
the initial acute illness. On echocardiography, imaging
of chronic rheumatic aortic valve stenosis demonstrates
thickened leaflets with variable degrees of commissural
fusion and leaflet retraction. Dooming of the leaflets,
increased echogenicity, and restricted motion present as
aortic valve stenosis progresses.

KAWASAKI DISEASE
Kawasaki disease is an acute, self-limited vasculitis that
occurs predominantly in infants and young children. It
was first described in Japan in l967 by Tomisaku Kawasaki.
The disease is now known to occur in both endemic and
community-wide epidemic forms in the Americas, Europe,
and Asia in all races. Kawasaki disease is characterized
by fever, bilateral nonexudative conjunctivitis, erythema
of the lips and oral mucosa, changes in the extremities,
rash, and cervical lymphadenopathy. Coronary artery
aneurysms or ectasia develop in approximately 1525%
of untreated children with the disease and may lead to
myocardial infarction, sudden death, or ischemic heart
disease.1820
In the United States, Kawasaki disease is the leading
cause of acquired heart disease in children. Treatment of
Kawasaki disease in the acute phase is directed at reducing
inflammation in the coronary artery wall and preventing
coronary thrombosis. The long-term therapy in individuals
who develop coronary aneurysms is aimed at preventing
myocardial ischemia or infarction.
The etiology of Kawasaki disease remains unknown,
although clinical and epidemiological features strongly
suggest an infectious cause. It also is possible that Kawasaki
disease results from an immunological response that is
triggered by any of several different microbial agents.
Kawasaki disease is a generalized systemic vasculitis
involving blood vessels throughout the body. Aneurysms
may occur in other extraparenchymal muscular arteries.
The early stages in the formation and development of
arteritis in Kawasaki disease have been well studied
morphologically in relatively large muscular arteries.21
Cardiovascular manifestations can be prominent in the
acute phase of Kawasaki disease and are the leading cause
of long-term morbidity and mortality in these patients.
During this acute phase, the pericardium, myocardium,
endocardium, valves, and coronary arteries may all be
involved.

1861

CORONARY ECTASIA AND ANEURYSMS BY ECHOCARDIOGRAPHY

The major sequelae of Kawasaki disease are related to
the coronary arterial system. Cardiac imaging is critical
for the evaluation of all patients with suspected Kawasaki
disease. It requires serial echocardiograms. The initial
echocardiogram should be performed as soon as the
diagnosis is suspected, but initiation of treatment should
not be delayed by the timing of the study. This initial
study establishes a baseline for longitudinal follow-up
of coronary artery morphology, cardiac function, and
the evolution and resolution of pericardial effusion
when present. Two-dimensional (2D) imaging should be
performed with the highest frequency transducer possible.
These probes allow for higher-resolution and detailed
evaluation of the coronary arteries. The focus should be
on imaging the left main coronary artery (LMCA), left
anterior descending coronary artery (LAD), left circumflex
coronary artery (LCX), and right coronary artery (RCA).
Common sites of coronary aneurysms include the proximal
LAD and proximal RCA, followed by the LMCA, then
LCX, and finally the distal RCA and the junction between
the RCA and posterior descending coronary artery22
(Figs 76.5 to 76.7). In addition to measuring coronary artery
dimensions, imaging the coronary arteries also may reveal
the lack of normal tapering and perivascular echogenicity
or brightness.23

Fig. 76.5: Left main coronary artery (LMCA; white arrow) and left
anterior descending artery (LAD; red arrow) ectasia in a patient
with Kawasaki disease without aneurysm.

1862

Section 6: Congenital Heart Disease

Table 76.1: Echocardiographic Views of Coronary Arteries in Patients with Kawasaki Disease

Left main coronary artery: Precordial short-axis at level of aortic valve; precordial long-axis of left ventricle (superior tangential);
subcostal left ventricular long-axis
Left anterior descending coronary artery: Precordial short-axis at level of aortic valve; precordial superior tangential long-axis of
left ventricle; precordial short-axis of left ventricle
Left circumflex: Precordial short-axis at level of aortic valve; apical four-chamber
Right coronary artery, proximal segment: Precordial short-axis at level of aortic valve; precordial long-axis (inferior tangential) of
left ventricle; subcostal coronal projection of right ventricular outflow tract; subcostal short-axis at level of atrioventricular groove
Right coronary artery, middle segment: Precordial long-axis of left ventricle (inferior tangential); apical four-chamber; subcostal
left ventricular long axis; subcostal short-axis at level of atrioventricular groove
Right coronary artery, distal segment: Apical four-chamber (inferior); subcostal atrial long-axis (inferior)
Posterior descending coronary artery: Apical four-chamber (inferior); subcostal atrial long-axis (inferior); precordial long-axis
(inferior tangential) imaging posterior interventricular groove

Fig. 76.6: Left main coronary artery (LMCA; white arrow) and left
anterior descending artery (LAD; yellow arrow) ectasia and early
aneurysm near the bifurcation (red arrow) in a patient with Kawasaki disease.

Fig. 76.7: Right coronary artery aneurysm (white arrow) in a

patient with history of Kawasaki disease.

The most commonly used view is the parasternal shortaxis at the level of the aortic root. However, a compre
hensive study of both the left and right coronary arteries
and their branches should be performed from parasternal,
four-chamber, and subcostal views to ascertain full
evaluation of the coronary system (Table 76.1).
The coronary arteries should be measured by 2D
echocardiography from inner edge to inner edge excluding
points of branching. Particular attention should be paid
to describe coronary abnormalities, including ectasia,
aneurysm, or intraluminal thrombi. When a coronary
artery is larger than normal (dilated) without a segmental
aneurysm, the vessel is considered ectatic. The use of z
scores are available for the LMCA, proximal LAD, and
proximal RCA. Aneurysms are classified as saccular if

axial and lateral diameters are nearly equal or as fusiform

if symmetric dilatation with gradual proximal and distal
tapering is seen. In the United States, aneurysms were
classified as small (< 5 mm internal diameter), medium
(58 mm internal diameter), or giant (> 8 mm internal
diameter). The Japanese Ministry of Health criteria classify
coronary arteries as abnormal if the internal lumen
diameter is > 3 mm in children < 5 years old or > 4 mm in
children 5 years old; if the internal diameter of a segment
measures 1.5-times that of an adjacent segment; or if the
coronary lumen is clearly irregular.22
In addition, assessment of left ventricle (LV) function
should be a part of the echocardiographic evaluation
of all patients with suspected Kawasaki disease. LV enddiastolic and end-systolic dimensions and a shortening

Chapter 76: Echocardiographic Evaluation for Acquired Heart Diseases in Childhood

fraction should be measured from standard M-mode

tracings. Apical imaging allows the estimation of LV
end-diastolic and end-systolic volumes and an ejection
fraction. Evaluating regional wall motion may be useful,
especially in children with coronary artery abnormalities
using routine as well as stress.
Standard pulsed and color flow Doppler interrogation
should be performed to assess the presence and degree of
valvular regurgitation (in particular for mitral and aortic
valves). Finally, the presence of pericardial effusion should
be evaluated as a part of routine evaluation.
For follow-up studies in patients with Kawasaki disease,
echocardiographic evaluation should be routinely per
formed at the time of diagnosis, at 2 weeks, and at 68 weeks
after onset of the disease. It is debatable if repeat echocardio
graphy should be performed 1 year after the onset of the
illness in patients in whom the echocardiographic findings
are normal at 48 weeks. Follow-up echocardiograms
should identify the progression or regression of coronary
abnormalities, evaluate ventricular and valvular function,
and assess the presence or evolution of pericardial effusions.
It is important to recognize the limitations of echo
cardiography in the evaluation and follow-up of patients
with Kawasaki disease. Although echocardiographic
detection of thrombi and coronary artery stenosis
has been reported, the sensitivity and specificity of
echocardiography for identifying these abnormalities is
unclear. In addition, the visualization of coronary arteries
becomes progressively more difficult as a child grows and
body size increases. Angiography, intravascular ultrasound
(IVUS), TEE, and other modalities including magnetic
resonance angiography (MRA) and ultrafast computed
tomography (CT) may be of value in the assessment of
selected patients.22

REFERENCES
1. Van Hare GF, Ben-Shachar G, Liebman J, et al. Infective
endocarditis in infants and children during the past
10 years: a decade of change. Am Heart J. 1984;107(6):
123540.
2. Li JS, Sexton DJ, Mick N, et al. Proposed modifications to
the Duke criteria for the diagnosis of infective endocarditis.
Clin Infect Dis. 2000;30(4):6338.
3. OBrien JT, Geiser EA. Infective endocarditis and echo
cardiography. Am Heart J. 1984;108(2):38694.
4. Gilbert BW, Haney RS, Crawford F, et al. Two-dimensional
echocardiographic assessment of vegetative endocarditis.
Circulation. 1977;55(2):34653.

1863

5. Shively BK, Gurule FT, Roldan CA, et al. Diagnostic

value of transesophageal compared with transthoracic
echocardiography in infective endocarditis. J Am Coll
Cardiol. 1991;18(2):3917.
6. Di Filippo S, Delahaye F, Semiond B, et al. Current patterns
of infective endocarditis in congenital heart disease. Heart.
2006;92(10):14905.
7. Mgge A, Daniel WG, Frank G, et al. Echocardiography
in infective endocarditis: reassessment of prognostic
implications of vegetation size determined by the
transthoracic and the transesophageal approach. J Am Coll
Cardiol. 1989;14(3):6318.
8. Vilacosta I, Graupner C, San Romn JA, et al. Risk of
embolization after institution of antibiotic therapy for
infective endocarditis. J Am Coll Cardiol. 2002;39(9):
148995.
9. Di Salvo G, Habib G, Pergola V, et al. Echocardiography
predicts embolic events in infective endocarditis. J Am Coll
Cardiol. 2001;37(4):106976.

10. Walter Wilson, Kathryn A Taubert, Michael Gewitz,
et al. Prevention of Infective Endocarditis. Circulation.
2007;116:173654.
11. Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic
fever in the intermountain area of the United States.
J Pediatr. 1994;124(1):916.
12. Dajani AS, Ayoub E, Burman FZ, et al. Secial Writing Group
of the Committee on Rheumatic fever, Endicarditis, and
Kawasaki of the American Heart Association. Guidelines
for the diagnosis of rheumatic fever. Jones Criteria. 1992
update. JAMA. 1992:268:206973.
13. Zhou LY, Lu K. Inflammatory valvular prolapse produced
by acute rheumatic carditis: echocardiographic analysis
of 66 cases of acute rheumatic carditis. Int J Cardiol.
1997;58(2):1758.
14. Arora R, Subramanyam G, Khalilullah M, et al. Clinical
profile of rheumatic fever and rheumatic heart disease: a
study of 2,500 cases. Indian Heart J. 1981;33(6):2649.
15. Horstkotte D, Niehues R, Strauer BE. Pathomorphological
aspects, aetiology and natural history of acquired mitral
valve stenosis. Eur Heart J. 1991;12 Suppl B:5560.
16. Virmani R, Farb A, Burke AP, et al. Pathology of acute
rheumatic carditis. In: Narula J, Virmani R, Reddy KS,
Tandon R, editors. Rheumatic Fever. Washington, DC:
American Registry of Pathology Press;1999:221.
17. Stollerman GH. Rheumatic fever in the 21st century. Clin
Infect Dis. 2001;33(6):80614.
18. Kato H, Sugimura T, Akagi T, et al. Long-term consequences
of Kawasaki disease. A 10- to 21-year follow-up study of
594 patients. Circulation. 1996;94:137985.
19. Dajani AS, Taubert KA, Gerber MA, et al. Diagnosis and
therapy of Kawasaki disease in children. Circulation.
1993;87:177680.
20. Taubert KA, Rowley AH, Shulman ST. Nationwide survey
of Kawasaki disease and acute rheumatic fever. J Pediatr.
1991;119:27982.

1864

Section 6: Congenital Heart Disease

21. Naoe S, Takahashi K, Masuda H, et al. Kawasaki disease.

With particular emphasis on arterial lesions. Acta Pathol
Jpn. 1991;41:78597.
22. Newburger JW, Takahashi M, Gerber MA, et al.; Committee
on Rheumatic Fever, Endocarditis and Kawasaki Disease;
Council on Cardiovascular Disease in the Young; American
Heart Association; American Academy of Pediatrics.
Diagnosis, treatment, and long-term management of

Kawasaki disease: a statement for health professionals

from the Committee on Rheumatic Fever, Endocarditis
and Kawasaki Disease, Council on Cardiovascular Disease
in the Young, American Heart Association. Circulation.
2004;110(17):274771.
23. Newburger JW, Taubert KA, Shulman ST, et al. Summary
and abstracts of the Seventh International Kawasaki
Disease Symposium: December 47, 2001.

SECTION 7
Miscellaneous and Other
Noninvasive Techniques

Chapters
Chapter 77
Chapter 78
Chapter 79
Chapter 80
Chapter 81

Echocardiography in Systemic Diseases

Echocardiography in Women
Echocardiography in the Elderly
How to do Echo for the Electrophysiologist
Echocardiography in Life-Threatening Conditions

Chapter 82 Lung Ultrasound in Cardiology

Chapter 83 The Future of Echocardiography and Ultrasound
Chapter 84 A Primer on Cardiac MRI for the
Echocardiographer
Chapter 85 Cardiac CT Imaging

1867

CHAPTER 77
Echocardiography in
Systemic Diseases
Mahdi Veillet-Chowdhury, Smadar Kort

Snapshot

Systemic Lupus Erythematosus

Rheumatoid Arthris

Hypereosinophilic Syndrome

Systemic Sclerosis

Renal Disease

Amyloidosis

INTRODUCTION
The presence of cardiac involvement in patients who suffer
various systemic diseases is relatively common and is often
associated with worse prognosis; therefore, early diagnosis
is critical. Echocardiography is a simple, noninvasive
imaging modality that is valuable in the evaluation of
patients with suspected cardiac manifestations of certain
disease processes, from autoimmune syndromes to
inflammatory conditions to various infections. In this
chapter, we will discuss the role of echocardiography in
the assessment of these patients and illustrate how newer
features of echocardiography such as tissue Doppler
imaging, three-dimensional (3D) echocardiography, and
speckle-tracking can be used to better assess specific
pathologies.

SYSTEMIC LUPUS ERYTHEMATOSUS

Systemic lupus erythematosus (SLE) is an autoimmune
disorder involving antinuclear autoantibodies that causes
a systemic inflammatory state in patients. Cardiovascular
involvement is common, which includes accelerated

Carcinoid

Chagas Disease

Sarcoidosis

Thyroid Disorders

Nutrional Deficiency

atherosclerosis, valvular disease, pericardial disease, and

myocardial disease.1,2 Echocardiography plays a vital role
in the detection of involvement of cardiac structures,
such as detection of pericardial effusions or diastolic
dysfunction. In fact, the presence of pericarditis is one
of the classifying criteria established by the American
College of Rheumatology for diagnosing SLE. Studies
have shown the prevalence of pericardial effusions in
patients with SLE ranging from 20% to 30% in some
populations.3,4 The chronic inflammatory state can
lead to early atherosclerosis, vasculitis, and ultimately
myocardial involvement, leading to left ventricular
(LV) diastolic dysfunction. A study of 85 SLE patients
described abnormally prolonged isovolumetric relaxation
times (IVRTs) and significantly greater thickness of the
interventricular septum and posterior wall, indicating
worsened diastolic function.5
Another frequent finding in patients with SLE is the
detection of LibmanSacks endocarditis. LibmanSacks
endocarditis is a nonbacterial verrucous valvular lesion
that often involves the mitral valve (MV) (Fig. 77.1).6 It is
believed that the deposition of fibrin-platelet thrombi on
the affected valve leads to the vegetations. As patients

1868

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 77.1: LibmanSacks endocarditis in a 58-year-old woman

with systemic lupus erythematosus (SLE). The transesophageal
echocardiogram (TEE) demonstrates a verrucous lesion on the
anterior mitral valve leaflet (arrow). (LA: Left atrium; LV: Left
ventricle).

are usually asymptomatic, LibmanSacks endocarditis is

often identified at autopsy, resulting in underestimation
of the true prevalence of this entity by initial studies.7
However, with increased awareness of this lesion and
the development of more advanced echocardiographic
modalities, a prevalence of up to 43% has been reported.6,8
The vegetations seen on transthoracic echocardiography
(TTE) and transesophageal echocardiogram (TEE) are
usually irregular in shape, firmly attached to the surface
at the valve ring and commissures, and vary in size and
echodensity.911

RHEUMATOID ARTHRITIS
Rheumatoid arthritis (RA) is a systemic autoimmune
disease of unknown etiology that can affect several organs,
including the heart. Cardiac manifestations include
coronary artery disease, conduction disease, valvular heart
disease, pericardial disease, and myocardial disease.12
Both systolic and diastolic LV dysfunction can occur
due to a combination of accelerated cardiovascular disease,
chronic inflammation, and use of cardiotoxic medications
causing nodules and fibrosis in the myocardium.13 Since
the myocardial performance index (MPI) is independent of
heart rate, preload, and afterload, it has been used to assess
global LV dysfunction in RA patients. A study involving
40 patients with active RA revealed that the MPI of the LV
was significantly higher than those of control patients,
indicative of abnormal global LV function, while the right
ventricular (RV) MPI was preserved.14 In combination with

the MPI, the transmitral flow propagation velocity was

shown to identify diastolic dysfunction in patients with
long-standing history of RA.15 In addition, patients with
RA have prolonged left and right ventricular deceleration
times (DTs) and IVRTs compared to control subjects.16
A study involving 60 patients with long-standing RA
demonstrated that the ratio of the early diastolic velocity
of the mitral annulus (E') to the late diastolic velocity of the
mitral annulus (A'), as well peak A and the ratio between
the early diastolic filling (E) to E' (E/E') parameters were
significantly impaired. Tissue Doppler imaging (TDI)
can add incremental value to conventional Doppler
echocardiography in RA patients, especially with RV
dysfunction, as RV diastolic impairment can be a predictor
of subclinical myocardial and pulmonary disease. A study
evaluating RV diastolic function in 35 RA patients revealed
that the prevalence of RV diastolic abnormalities defined
as a ratio of (E) to atrial filling (A) of <1, was higher in RA
patients compared to control subjects.17
The most common cardiac structure involved in RA
is the pericardium, with evidence of pericarditis reported
to be 3050% in these patients.18 Although <10% of the
patients with pericarditis are symptomatic, constrictive
pericarditis or rapidly developing exudative effusions
portend a poor prognosis.18,19
Valvular disease is also common, with a reported
prevalence of approximately 39% in patients with RA.20
The inflammatory state leads to the formation of nodules
and fibrosis throughout the entire valvular apparatus,
most frequently involving the mitral and aortic valves
(Movie clip 77.1; Figs 77.2A to C).2022 Mitral regurgitation
is the most common valvular abnormality, while aortic
regurgitation and stenosis can also occur.23

HYPEREOSINOPHILIC SYNDROME
Hypereosinophilic syndrome (HES), or Loefflers syndrome,
is characterized by a persistently elevated eosinophil
count (1.5 109/L) for at least 6 months as well as
eosinophil-related end organ damage with no identifiable
cause. HES tends to occur more commonly in males
between the third and sixth decades of life. Cardiac
involvement has been reported in >50% of patients and
suggests a poor prognosis.24 Cardiac damage classically
occurs in two stages. In the first stage, acute myonecrosis
occurs due to eosinophilic infiltration of the myocardium
and subsequent release of toxic proteins. The later
stage is described by the formation of mural thrombi,
endomyocardial fibrosis, valvular disease, and restrictive
cardiomyopathy.25,26

Chapter 77: Echocardiography in Systemic Diseases

1869

Figs 77.2A to C: (A) A three-dimensional (3D) echocardiogram of a

56-year-old gentleman with rheumatoid arthritis (RA). A parasternal
long-axis view of a thickened and fibrotic mitral valve (MV) leaflets and chordae (arrow); (B) A 3D parasternal short-axis view of
the thickened and fibrotic MV (arrow); (C) A two-dimensional (2D)
parasternal short-axis view of a thickened aortic valve (AV) with
a small nodular density on the left coronary cusp (curved arrow).
(LV: Left ventricle).

On echocardiography, there can be evidence of

endomyocardial thickening with plaques larger than
2 mm. In addition, valvular regurgitation due to adhesion
of the valvular apparatus can become clinically significant. Progressive scarring causes restriction of the
chordae tendinae, which often affects the posterior
mitral leaflet.27,28 In addition, there can be evidence of
fibrothrombotic growth on both the ventricular apices due
to blood stasis and denuded myocardium, occasionally
causing obliteration of the cavities (Figs 77.3 and 77.4).
Studies have also reported a predilection for pericardial
involvement, with one study demonstrating pericardial
effusions in almost one-third of subjects with HES.25
As mentioned earlier, later stages of HES can cause
restrictive cardiomyopathy. As the endomyocardial
thickening and fibrosis leads to diastolic dysfunction,
Doppler echocardiography and TDI measurements of
diastolic parameters can help in evaluating the full scope

of cardiac involvement, such as decreases in transmitral

E-wave DT and an E/A ratio consistent with restrictive
physiology.

SYSTEMIC SCLEROSIS
Systemic sclerosis (SSc) is a connective tissue disorder
characterized by vascular lesions and widespread fibrosis
of the skin and other organs, frequently the heart, with
one study showing the prevalence of cardiac involvement
to be as high as 32% in patients with diffuse SSc.29 A
range of cardiac manifestations can be seen, including
conduction system disease, coronary artery disease,
pericardial involvement, RV dysfunction due to pulmonary
hypertension, and both LV systolic and diastolic
dysfunction. As studies have shown that patients with
cardiac involvement confer a poor prognosis, screening
for subclinical disease with echocardiography is vital.30

1870

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 77.3: A 74-year-old gentleman with hypereosinophilic syndrome (HES). A two-dimensional (2D) apical four-chamber view
demonstrating thickening and infiltration of the right ventricle
(RV; arrow). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

Figs 77.4A to D: (A) A 67-year-old gentleman with asthma and hypereosinophilic syndrome (HES) with an eosinophil count of
5.6 109/L. A two-dimensional (2D) parasternal long-axis view demonstrating thickening and infiltration of the left ventricle (LV); (B) A
parasternal short-axis view demonstrating thickening and infiltration of the LV (arrow); (C) An apical four-chamber view demonstrating
thickening and infiltration of the LV (arrow); (D) An apical two-chamber view demonstrating thickening and infiltration of the LV. (LA: Left
atrium; LV: Left ventricle; RV: Right ventricle).

Chapter 77: Echocardiography in Systemic Diseases

Myocardial fibrosis is a hallmark cardiac manifestation

of SSc and can lead to increased ventricular mass, ventricular wall motion abnormalities, and impaired diastolic
relaxation.31,32 TDI can be used to measure systolic
longitudinal velocity (S') and mitral annular velocity (E')
to identify patients with systolic and diastolic dysfunction,
respectively.33 A study with 100 consecutive patients
with SSc showed that using TDI to evaluate mitral and
tricuspid annular velocities, the presence of subtle RV and
LV dysfunction was able unmasked.34 Speckle-tracking
echocardiography can also provide insight toward
subclinical LV and RV systolic dysfunction. Another study
showed that in SSc subjects with a normal radionuclide
ejection fraction, speckle imaging was able to detect
lower systolic and diastolic strain rates.35 Speckle-tracking
imaging of the RV revealed that while TDI indices were
similar in subjects with SSc compared with controls, the
SSc subjects were found to have reduced strain.36
Due to pulmonary involvement, the presence of
secondary RV dysfunction needs to be fully excluded.
Therefore, validated measurements of RV function should
be incorporated into the routine assessment in SSc, and
should include echocardiographic parameters such as
tricuspid annular plane systolic excursion, RV free wall
TDI-derived S', and pulmonary artery pressures.37,38
Pericardial disease in SSc can range from asymptomatic
pericardial effusions to constrictive pericarditis. Although
up to 78% of patients with SSc have been reported to have
some pericardial involvement, the prevalence of clinically
symptomatic disease is low at 516%.3941 Pericardial
effusions in the setting of pulmonary hypertension is a
prognostic indicator in patients with SSc, as the presence
of pericardial effusions together with increased right
atrial (RA) size and abnormal interventricular septal
displacement during diastole predicts poor outcomes.42
Valvular disease has also been described, with nodular
thickening of the MV reported in up to 38% of patients
with SSc.43

RENAL DISEASE
Cardiovascular mortality is significantly increased in
patients with chronic kidney disease (CKD), leading to
earlier atherosclerosis, valvular and pericardial disease,
arrhythmias, and heart failure. Use of echocardiography
plays a crucial role in the evaluation of these patients as
the structural and functional abnormalities can alter the
management and prognosis of this patient population.
Over time, a combination of CKD and other medical

1871

conditions such as hypertension and diabetes leads to

myocardial fibrosis and the development of left ventricular
hypertrophy (LVH). However, due to the high tendency
for pressure and volume overload to occur, the cardiac
structure can display varying types of hypertrophy.44,45
This cardiac alteration leads to dysfunction in both
diastolic and systolic properties. Even though LVH is fairly
common in this population, the accurate assessment of
LV mass index is especially difficult due to variations in
patients volume status.44,46 Similarly, diastolic assessment
in these patients could be challenging. Elevated LV filling
pressures signify worsening relaxation and compliance of
the LV; however, in the CKD patient, the wide variations in
volume status makes mitral inflow velocity measurements
challenging.47 Therefore, other assessments can further
aid in evaluation of diastolic dysfunction. In hemodialysis
(HD) patients, left atrial (LA) enlargement, assessed by
calculating the LA volume index, has been found to be a
predictor of mortality.48,49 Furthermore, the ratio of early
mitral flow velocity to early mitral annulus velocity (E/E')
has been proven to be a reliable measure of LV filling
pressures in end-stage renal disease (ESRD) patients.50
Uremic cardiomyopathy is characterized by LV enlargement, hypertrophy, and systolic dysfunction. Fractional shortening, a measurement of global LV systolic
function in the absence of regional abnormalities, could
overestimate contractility in patients with concentric
LVH and, therefore, should not be used in these patients.
In dialysis patients, tissue velocity and strain imaging
can detect changes in LV function and are less affected
by the volume status of the patient.51 The utility of stress
echocardiography for excluding coronary atherosclerotic
disease in these patients is uncertain, given the relatively
lower specificity of this modality in the presence of LVH, a
common finding in this patient population.52
Another common finding in HD patients is myocardial
stunning due to acute myocardial ischemia during dialysis
sessions, which over time increases the risk of heart failure
and arrhythmias.53 A study examining 70 HD patients
found a significant reduction in systolic function in 64% of
subjects.54
Given the abnormalities in calcium and phosphorus
metabolism and inflammation associated with renal
disease, valvular and vascular calcifications are common
in patients with CKD, particularly mitral annular
calcifications (MACs), which has been reported to occur
in > 40% of ESRD patients (Movie clip 77.2; Figs 77.5A
and B).5557 Of great importance are the findings of mobile
components associated with MAC, as these lesions can
become sources of emboli.5860

1872

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 77.5A and B: (A) A 59-year-old woman with endstage renal disease (ESRD). A transesophageal echocardiogram (TEE) demonstrating a large echodensity (arrows) on the atrial surface of the posterior mitral valve (MV) annulus and leaflet consistent with mobile
mitral annular calcification (MAC); (B) A TEE zoomed in view demonstrating a large echodensity (arrows) on the atrial surface of the
posterior MV annulus and leaflet consistent with mobile mitral annular calcification (MAC). (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).

Uremic pericarditis is used to describe ESRD patients

who develop clinical manifestations of pericarditis. Studies
have reported a wide range of prevalences, but generally
occur in < 20% of patients with ESRD.6163 Similar to the
development of pericarditis, pericardial effusions can also
develop, with studies showing prevalences of 1127% in
ESRD patients.62,6468

AMYLOIDOSIS
Amyloidosis is a systemic disorder that is caused by
extracellular deposits of insoluble aggregated proteins
with a -pleated sheath configuration. The incidence
of light chain (AL) amyloidosis or primary amyloidosis
is more than 10 per million person-years in the US
population and has a predilection for men in the sixth
decade of life.69 Amyloid can affect numerous organ
systems but infiltration of the heart confers an extremely
poor prognosis, as amyloid patients with congestive heart
failure (CHF) have a median survival of about 6 months.70
In cardiac amyloidosis, amyloid deposits infiltrate the
myocardium with progression to myocyte necrosis and
local interstitial fibrosis.
As the heart is thickened, diastolic dysfunction progresses, eventually leading to restrictive cardiomyopathy.71
Due to the reduced compliance of the LV, the chamber
diameters remain normal, but the free wall and septum
thicken.72 While amyloid deposits are rare in the epicardial

vessels, they can involve the intramural vasculature and

lead to microvascular ischemia. In addition, although
relatively rare, amyloid can accumulate in the pericardium
and lead to constrictive physiology.72
As there is no single test with a great sensitivity to
diagnose cardiac amyloid, when evaluating a patient with
suspected cardiac amyloid it is important to correlate the
clinical picture with the diagnostic findings. Echocardiography can be a valuable tool to support establishing the
diagnosis. Although not specific, one of the characteristic
two-dimensional (2D) echocardiographic features is a
granular appearance of the myocardium (Movie clip 77.3;
Figs 77.6 to 77.8). The most common finding is thickening
of the LV wall in the absence of other contributing factors
such as hypertension or aortic stenosis.73 A study showed
that the presence of low voltage on the electrocardiogram
(ECG) associated with an interventricular septal thickness of >1.98 cm has a sensitivity of 72% and specificity of
91% for the diagnosis of cardiac amyloidosis.74 It has been
shown that this finding has a strong inverse relationship
with survival in patients with heart failure due to amyloid.75
A recent study examining seven consecutive patients with
endomyocardial biopsy-proven cardiac amyloid found
that an echocardiographic pattern of preserved motion
of the LV apex and hypokinesis of the basal to mid
segments was consistently identified in all patients.76
Other striking features include biatrial enlargement and
thickened valves.

Chapter 77: Echocardiography in Systemic Diseases

1873

Fig. 77.6: An 89-year-old woman with systemic amyloidosis. Threedimensional (3D) apical four-chamber view showing right ventricle
(RV) infiltration, can be used to differentiate thickening of the free
wall from a moderator band (asterisk). (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).

Figs 77.7A to C: (A) An 85-year-old gentleman with systemic

amyloidosis. A two-dimensional (2D) apical four-chamber view
demonstrating a starry-sky speckled appearance and thickening
of the left ventricular (LV) myocardium; (B) A pulsed wave Doppler
echocardiography of the mitral valve (MV) demonstrating a transmitral E-velocity (arrow): A-velocity (curved arrow) ratio > 2:1 and
a short deceleration time, consistent with restrictive physiology;
(C) A tissue-Doppler imaging (TDI) echocardiography of the lateral
mitral annulus demonstrating a decreased E'-velocity (asterisk),
consistent with abnormal diastolic function. (LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).

Early amyloid deposition does impair isovolumetric

relaxation leading to mild diastolic dysfunction with
reversal of the transmitral E/A velocity ratio initially, but

eventually leading to restrictive cardiomyopathy with

E/A ratio > 2:1. TDI can further assist in the diagnosis
as it can show decreased mitral annular velocities and

1874

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 77.8A to C: (A) An 80-year-old gentleman with systemic

amyloidosis and congestive heart failure. A two-dimensional (2D)
apical four-chamber view demonstrating thickening of the left ventricular (LV) myocardium; (B) A pulsed wave Doppler echocardiography of the mitral valve (MV) demonstrating a transmitral E-velocity
(arrow): A-velocity (curved arrow) ratio > 2:1, consistent with
restrictive physiology; (C) A tissue Doppler imaging (TDI) echocardiography of the lateral mitral annulus demonstrating a decreased
E'-velocity (asterisk), consistent with abnormal diastolic function.
(LV: Left ventricle).

elevated filling pressures. Investigators have shown that

the myocardial velocity profiles of the LV septum and
posterior wall show a distinctive serrated pattern not
present in patients with hypertension or hypertrophic
cardiomyopathy.77
Another useful technique that can provide prognostic
information is tissue Doppler-based strain and strain rate
imaging (SRI), which can allow accurate assessment of
regional myocardial deformation with high spatial and
temporal resolution.78 Studies have shown that Dopplerderived longitudinal systolic strain averaged for the
16 segments of the LV can differentiate biopsy-proven
cardiac amyloid patients from healthy control patients.79,80
A related newer technique is speckle and derived SRI,
which was shown to differentiate cardiac amyloidosis
and hypertrophic cardiomyopathy from other causes
of increased LV wall thickness.81 The authors found that

global myocardial deformation was significantly lower in

patients with cardiac amyloid compared to those with LV
hypertrophy due to other causes. Other helpful techniques
include the use of contrast echocardiography to evaluate
for coronary flow reserve and 3D echocardiography to
assess for the presence of intraventricular dyssynchrony.
Although these findings are not specific, their presence
could help support the diagnosis of this rare condition.82,83

CARCINOID
Carcinoid tumors are rare neuroendocrine malignancies
derived primarily from enterochromaffin cells, with the
primary site usually being in the gastrointestinal tract. The
usual presentation occurs in the fifth to seventh decade of
life with the classic symptom triad of flushing, secretory
diarrhea, and bronchospasm.84 Carcinoid heart disease
has been reported to be present in up to 5060% of patients

Chapter 77: Echocardiography in Systemic Diseases

with carcinoid syndrome, and should therefore be

excluded in these patients.85 Cardiac involvement portends a poor prognosis, as one study has shown reduced
mean survival in patients with carcinoid heart disease
(life expectancy of 1.6 years compared to 4.6 years in those
without cardiac manifestations).86 Cardiac involvement
occurs through paraneoplastic effects when tumor growth
within the liver allows the secretory products, such as
serotonin, to reach the right side of the heart without having
gone through inactivation in the hepatic or pulmonary
circulatory bed. However, left-sided heart disease can also
occur, although the incidence is much less due to the low
levels of serotonin present after passing through the lung.86
This disease process manifests in plaque-like endocardial
deposits of fibrous tissue on the ventricular and arterial
aspects of the tricuspid and pulmonic valves (PVs),
respectively.87 The clinical symptoms usually encompass
that of right-sided heart failure, although conduction
disease, coronary vasospasm, constrictive pericarditis,
and restrictive cardiomyopathy have all been reported.8891
The presence of right-sided heart failure is an independent
predictor of mortality in this population.92
Echocardiography is a key tool in aiding the diagnosis and management of carcinoid heart disease.
Typical features include thickening of the valve leaflets
and cusps, causing them to become retracted, fixed,
and noncoapting, resulting in tricuspid and pulmonic
stenosis and regurgitation (Movie clip 77.4; Figs 77.9 and
77.10).86 Most commonly, thickening involves the septal
and anterior leaflets of the tricuspid valve (TV), as well
as the chordae and papillary muscles.93 This results in
echocardiographic signs of severe tricuspid regurgitation,
such as enlarged vena contracta width and regurgitant
volume, dilated inferior vena cava (IVC), and systolic flow
reversal in the hepatic veins. Typically, continuous wave
Doppler echocardiography shows a dagger-shaped profile,
with an early peak pressure and rapid decline.86 If the PV
is involved, the predominant location of the plaques are
at the pulmonic root, causing constriction of the root and
orifice.94 However, concomitant pulmonic regurgitation
is not uncommon as demonstrated by prior studies.86 If a
patent foramen ovale (PFO) is present, the increased RA
pressure due to tricuspid and pulmonic valvular disease
can result in a significant right-to-left shunt, promoting
the likelihood of developing left heart involvement with
carcinoid, and therefore the presence of a PFO should
routinely be excluded.95

1875

TDI also can play an important role in evaluating

the progression of carcinoid heart disease. A study of 41
patients with carcinoid syndrome demonstrated that E/E'
ratio 8 was an independent marker of death.96 Myocardial
strain echocardiography can also help in the evaluation
of this population. Aside from valvular disease, carcinoid
can also lead to the development of mural endocardial
fibrosis of the ventricles. A study involving 89 patients
with carcinoid disease revealed reduced RV function
using strain compared with healthy control subjects.
This reduced RV function was independent of valvular
involvement, further supporting the role of tissue Doppler
when assessing patients with carcinoid.97

CHAGAS DISEASE
Chagas disease is a tropical disease endemic in Mexico,
Central, and South America caused by the parasite
Trypanosoma cruzi. There is usually an acute or early
phase and a chronic or late phase. Although infrequent,
fulminant myocarditis can occur in 15% of patients in the
acute phase.98 More commonly, about 30% of seropositive
individuals develop cardiomyopathy several years after the
acute phase.99 This usually occurs through a combination
of cardiac dysautonomia, microvascular disease, parasite,
and immune-mediated myocardial injury. A recent
systematic review demonstrated that positive serology for
Chagas disease is associated with a higher risk of death in
patients with heart failure.100
Echocardiography can provide valuable information
regarding the extent of myocardial damage and, therefore,
help determine the prognosis of patients with Chagas heart
disease. Apart from acute myocarditis, the early phase
of Chagas disease can lead to the rapid development of
pericardial effusions, with one study noting the presence
of pericardial effusions in 42% of subjects.101 Most of the
structural heart changes take place slowly over years
after the initial infection goes unnoticed (Movie clip 77.5;
Figs 77.11A to F). Studies have shown that > 50% of
patients with Chagas disease develop apical aneurysms,
and become at higher risk of forming a mural thrombus.102
Both TTE and TEE were shown to recognize potential
cardiac source of emboli with high accuracy.103
More advanced cardiac disease include global cardiac
dilatation and diffuse hypokinesis; however, early in the
disease process, segmental wall motion abnormalities
can also be identified on 2D echocardiograms, with the
apical and posteroinferior walls being the most commonly

1876

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 77.9A to D: A 53-year-old gentleman with metastatic carcinoid disease to bones and liver. A two-dimensional (2D) echocardiography
showing a thickened, retracted, and noncoapting tricuspid valve (arrow); (B) A color flow Doppler echocardiography showing severe
tricuspid regurgitation with a large regurgitant jet; (C) A continuous wave (CW) Doppler echocardiography showing an increased
E-velocity across the tricuspid valve (TV), consistent with severe tricuspid stenosis (curved arrow). However, the mean pressure gradient
of 5 mm Hg across the valve excludes the presence of significant stenosis as the etiology; (D) A three-dimensional (3D) short-axis view
demonstrating a thickened, fixed, noncoapting tricuspid valve (arrow). (RA: Right atrium; RV: Right ventricle).

affected.104106 Dobutamine stress echocardiography has

a role when evaluating these patients, as a blunted heart
and contractile response during dobutamine infusion can
be found, even in subjects without baseline regional wall
motion abnormalities.107 Interestingly, in this study, few
patients demonstrated a biphasic response to dobutamine,
with improvement in contractility at a low dose, and
deterioration in contractile function at a higher dose.
Over time, patients infected with Chagas are at high
risk of developing both systolic and diastolic dysfunction.
A study of 169 patients with Chagas cardiomyopathy
reported that a reduced TDI septal E'-wave of 11 cm/s
and a septal E/E' ratio > 7.2 were both highly sensitive and

moderately specific for detection of any stage of diastolic

dysfunction.105 In addition, in symptomatic patients, the
MPI is markedly higher for both the LV and RV, consistent
with combined severe systolic and diastolic myocardial
dysfunction.108

SARCOIDOSIS
Sarcoidosis is a multisystem disorder that is characterized
by the presence of noncaseating granulomas that can
affect numerous organs in the body. The etiology remains
unknown, although environmental, occupational, infectious, and genetic causes have all been proposed. While

Chapter 77: Echocardiography in Systemic Diseases

1877

Figs 77.10A and B: (A) A 53-year-old gentleman with carcinoid heart disease. A two-dimensional (2D) short-axis view showing a thickened and fixed pulmonic valve (PV; arrow); (B) A continuous wave (CW) Doppler echocardiography showing mild pulmonic stenosis with
a mean transvalvular pressure gradient of 20 mm Hg (curved arrow).

Figs 77.11A to D

1878

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 77.11A to F: A 28-year-old gentleman emigrated from El Salvador and presented with Chagas heart disease. A two-dimensional
(2D) parasternal long-axis view showing a dilated left ventricle (LV; asterisk); (B) A 2D echocardiogram short-axis view showing a dilated
LV (asterisk); (C) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow); (D) A zoomed-in 2D apical fourchamber view showing mitral regurgitation (MR); (E) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow);
(F) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow) (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

it can affect people across all ethnic groups and ages,

studies have shown that there is a predilection among
African Americans and Northern Europeans.109 While
the disease can remain asymptomatic for many years,
the most common presentation involves the pulmonary
system, causing cough and dyspnea. However, cardiac
involvement is not uncommon, with a prevalence in
the United States reported to be about 25% in patients
with systemic sarcoidosis, and should, therefore, be
excluded in these patients.110 All cardiac structures can be
involvedinfiltration of the granulomas in the conduction
system, myocardial involvement of all chambers, and
the development of pericardial effusions have all been
reported.111
Although considered the gold standard for establishing the diagnoses, endomyocardial biopsy often is
unable to demonstrate pathological evidence of cardiac
sarcoidosis.112 Therefore, noninvasive modalities, such as
echocardiography, are a valuable tool in the diagnoses
and management of sarcoid heart disease, as it can
demonstrate a range of structural abnormalities, and can
be repeated as needed (Movie clips 77.6 to 77.8; Figs 77.12
and 77.13).
Given that pulmonary disease is the most common
manifestation of sarcoidosis, right-sided heart failure
due to pulmonary hypertension is commonly observed;
therefore, evidence of elevated RV pressures should be

evaluated using echocardiography.112 The LV free wall

and interventricular septum are the most commonly
affected sites.111 LV dilatation and wall thinning can
occur leading to aneurysm formation.113 Granuloma
penetration into the myocardium can cause myocardial
fibrosis and lead to systolic and diastolic dysfunction with
wall motion abnormalities, usually at the mid and basal
levels.114 In addition, both thickening and thinning of
the interventricular septum with dyskinetic segments as
well as papillary muscle dysfunction leading to valvular
insufficiency have also been reported.114,115 It is important
to identify these echocardiographic abnormalities as
studies have shown that the severity of LV dysfunction
and end-diastolic diameter are independent predictors of
mortality in this patient population.116
Other echocardiographic abnormalities have been
noted in patients with sarcoidosis. Pericardial effusions
have been reported in up to 19% of patients with cardiac
sarcoidosis.117 In addition, constrictive pericarditis has
been reported, although this is infrequent.118 One study
looking at 69 patients with chronic sarcoidosis found
that patients with sarcoid had lower midwall fractional
shortening compared with controls.119 Furthermore,
echocardiography can be used to detect cyclic variation of
integrated backscatter to estimate the myocardial acoustic
properties to recognize cardiac sarcoidosis.120

Chapter 77: Echocardiography in Systemic Diseases

Fig. 77.12: A 41-year-old gentleman with sarcoidosis and

severe segmental left ventricular (LV) systolic dysfunction, who had
normal coronary arteries on cardiac catheterization. A two-dimensional (2D) parasternal short-axis view showing a dilated LV.
(LV: Left ventricle).

THYROID DISORDERS
Hyperthyroidism is characterized by elevated peripheral
free thyroid hormone levels combined with decreased
thyroid stimulating hormone (TSH) levels. Thyrotoxicosis
can lead to significant changes in the cardiac structure
and function, causing hypertension, heart failure, and
arrhythmias. Echocardiography can play an important
role in the evaluation of patients with thyroid disorders,
as even subclinical hyperthyroidism can lead to cardiac
abnormalities. Elevated thyroid hormones leads to a high
cardiac output by up to 50300% as well as increased
preload, leading to an increase in LV mass and LA
size.121124 Due to the increased cardiac contractility and
heart rate, shortened interventricular conduction time
and pre-ejection period have been demonstrated.123 SRI
has shown enhancement of systolic stain rate in patients
with subclinical hyperthyroidism due to the greater
early systolic phase deformation.125 However, stress
echocardiography has demonstrated a blunted increase
in LV ejection fraction and cardiac output.126 In regards
to RV function, TDI has demonstrated enhanced systolic
function in subjects with overt hyperthyroidism.127
Mild diastolic dysfunction has also been demonstrated
by Doppler echocardiography, with reduced peak E-wave
velocity and a significantly higher peak A-wave velocity.125
TDI reveals impairment of the mitral annular velocity and

1879

Fig. 77.13: A 74-year-old woman with sarcoidosis. A twodimensional (2D) apical four-chamber view demonstrates
right ventricular (RV) hypertrophy, mildly dilated RV, and right
atrium (RA). LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle.

SRI reveals a significant decrease in diastolic function. In

patients with Graves disease, valvular abnormalities have
been reported, such as tricuspid regurgitation and MV
prolapse.128
In contrast to hyperthyroidism, hypothyroid is characterized by decreased lower peripheral thyroid hormone
levels and elevated TSH levels. Physiologically, there is
evidence of decreased cardiac output and contractility.
As compared to hyperthyroidism, there is a predilection for arrhythmias due to prolonged QT interval and
the prevalence of heart failure is much less. Again,
echocardiographic data plays an important role in the
evaluation of these patients. Numerous studies have
shown abnormalities in systolic function indices, such as
reduced ejection fraction and fractional shortening, and
increased pre-ejection period (Movie clip 77.6; Figs 77.14A
and B).129132 In patients with subclinical hypothyroidism,
TDI was shown to be able to demonstrate systolic
dysfunction during exercise.133,134 LV diastolic dysfunction
can easily be diagnosed on echocardiograms by demonstrating the presence of significant impairment of LV
filling, prolonged IVRT, and prolongation of the MPI.135138
Although cardiac tamponade physiology is rare, pericardial effusions can also be found frequently, with studies
showing a prevalence of up to 25% in patients with overt
hypothyroidism.139

1880

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 77.14A and B: (A) A 59-year-old gentleman with hypothyroidism. A two-dimensional (2D) parasternal long-axis view demonstrating a dilated left ventricle (LV); (B) An apical four-chamber view demonstrating dilated atria and LV. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle).

Figs 77.15A and B: (A) A 46-year-old gentleman with a history of alcohol abuse and thiamine deficiency. A two-dimensional (2D) apical
two-chamber view showing a dilated left ventricle (LV) with reduced systolic function; (B) A 2D apical two-chamber view showing small
LV diameter and significantly improved systolic function after thiamine replacement. (LA: Left atrium; LV: Left ventricle).

NUTRITIONAL DEFICIENCY
Nutritional deficiencies in patients can adversely affect
myocardial performance and increase cardiovascular
morbidity and mortality, particularly thiamine deficiency
due to either dietary factors or alcohol abuse (or sometimes
a combination of both). Thiamine, or Vitamin B1, is a watersoluble B complex vitamin, and its deficiency has been
shown to cause heart disease, most notably beriberi heart
disease. Beriberi is characterized by heart failure with
biventricular dysfunction with significant hemodynamic
abnormalities, most particularly RV failure and elevated
LV end-diastolic pressures.140 Echocardiographic features
include biventricular enlargement and decreased systolic
function of both chambers (Figs 77.15A and B).

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patients with different causes of hyperthyroidism. Nat Rev
Endocrinol. 2010;6(8):43143.
Sgarbi JA, Villaa FG, Garbeline B, et al. The effects of
early antithyroid therapy for endogenous subclinical
hyperthyroidism in clinical and heart abnormalities. J Clin
Endocrinol Metab. 2003;88(4):16727.
Biondi B, Palmieri EA, Lombardi G, et al. Effects of thyroid
hormone on cardiac function: the relative importance of
heart rate, loading conditions, and myocardial contractility
in the regulation of cardiac performance in human
hyperthyroidism. J Clin Endocrinol Metab. 2002;87(3):
96874.
Smit JW, Eustatia-Rutten CF, Corssmit EP, et al. Reversible
diastolic dysfunction after long-term exogenous subclinical
hyperthyroidism: a randomized, placebo-controlled study.
J Clin Endocrinol Metab. 2005;90(11):60417.
Di Bello V, Aghini-Lombardi F, Monzani F, et al. Early
abnormalities of left ventricular myocardial characteristics
associated with subclinical hyperthyroidism. J Endocrinol
Invest. 2007;30(7):56471.
Kahaly GJ, Wagner S, Nieswandt J, et al. Stress echocardiography in hyperthyroidism. J Clin Endocrinol Metab.
1999;84(7):230813.
Arinc H, Gunduz H, Tamer A, et al. Evaluation of right
ventricular function in patients with thyroid dysfunction.
Cardiology. 2006;105(2):8994.
Kage K, Kira Y, Sekine I, et al. High incidence of mitral
and tricuspid regurgitation in patients with Graves disease
detected by two-dimensional color Doppler echocardiography. Intern Med. 1993;32(5):3746.
Crowley WF Jr, Ridgway EC, Bough EW, et al. Noninvasive
evaluation of cardiac function in hypothyroidism.
Response to gradual thyroxine replacement. N Engl J Med.
1977;296(1):16.
Forfar JC, Muir AL, Toft AD. Left ventricular function in
hypothyroidism. Responses to exercise and beta adrenoceptor blockade. Br Heart J. 1982;48(3):27884.
Lee RT, Plappert M, Sutton MG. Depressed left ventricular
systolic ejection force in hypothyroidism. Am J Cardiol.
1990;65(7):5267.
Vitale G, Galderisi M, Lupoli GA, et al. Left ventricular
myocardial impairment in subclinical hypothyroidism
assessed by a new ultrasound tool: pulsed tissue Doppler.
J Clin Endocrinol Metab. 2002;87(9):43505.

Chapter 77: Echocardiography in Systemic Diseases

133. Arinc H, Gunduz H, Tamer A, et al. Tissue Doppler echocardiography in evaluation of cardiac effects of subclinical
hypothyroidism. Int J Cardiovasc Imaging. 2006;22(2):
17786.
134. Kosar F, Sahin I, Turan N, et al. Evaluation of right and
left ventricular function using pulsed-wave tissue Doppler
echocardiography in patients with subclinical hypothyroidism. J Endocrinol Invest. 2005;28(8):70410.
135. Doin FL, Borges Mda R, Campos O, et al. Effect of central
hypothyroidism on Doppler-derived myocardial performance index. J Am Soc Echocardiogr. 2004;17(6):6229.
136. Biondi B, Klein I. Hypothyroidism as a risk factor for
cardiovascular disease. Endocrine. 2004;24(1):113.

1885

137. Fldes J, Istvnfy M, Halmgyi M, et al. Hypothyroidism

and the heart. Examination of left ventricular function in
subclinical hypothyroidism. Acta Med Hung. 1987; 44(4):
33747.
138. Aghini-Lombardi F, Fabrizio AL, Di Bello V, et al. Early
textural and functional alterations of left ventricular
myocardium in mild hypothyroidism. Eur J Endocrinol.
2006;155(1):39.
139. Kahaly GJ, Dillmann WH. Thyroid hormone action in the
heart. Endocr Rev. 2005;26(5):70428.
140. Ayzenberg O, Silber MH, Bortz D. Beriberi heart disease.
A case report describing the haemodynamic features. S Afr
Med J. 1985;68(4):2635.

CHAPTER 78
Echocardiography in Women
Jennifer Kiessling, Navin C Nanda, Tugba Kemaloglu z, Aylin Sungur, Kunal Bhagatwala, Nidhi M Karia

Snapshot

Dierences in Echocardiographic Measurements and

Technical Considera ons

Structural Heart Disease: MVP, Mitral Stenosis, and

Mitral Annular Calcifica on

Ischemic Heart Disease/Stress

Echocardiography/Polycys c Ovarian Syndrome

INTRODUCTION
In terms of cardiac disease, it is often thought that men
are more affected, while women are somehow protected.
This has proven to be untrue. This chapter will illustrate
the use of the echocardiogram in women. Furthermore,
it will highlight some differences in the standardization
of echocardiographic measurements as well as some
technical challenges in women. Echocardiography is often
the primary diagnostic tool to diagnose patients with
structural heart disease. Structural heart diseases such
as atrial septal defects, mitral valve prolapse (MVP), and
pulmonary arterial hypertension are more commonly seen
in women. Also, the utilization of stress echocardiography
in women to diagnose underlying coronary artery disease
(CAD) is discussed and compared to other current
imaging modalities. The usage of the echocardiogram in
pregnant women is discussed in detail. Finally, the role of
fetal echocardiography is highly useful to diagnose and
manage fetal cardiac anomalies.

DIFFERENCES IN ECHOCARDIOGRAPHIC MEASUREMENTS AND

TECHNICAL CONSIDERATIONS
Reference values for chamber sizes, vessel diameters, and
left ventricle (LV) mass differ between men and women

Takotsubo Cardiomyopathy

Congenital Heart Disease

Echocardiography in Pregnancy, Peripartum

Cardiomyopathy, Fetal Echocardiography

(Table 78.1). The aortic root size, LV end systolic, and

LV end diastolic dimensions are larger in men than in
women.15 There are also differences in Doppler velocities
and mitral annular velocities. In women, the diastolic
early mitral annular velocities are higher using pulsed
wave tissue Doppler imaging (TDI) compared to men. For
example, in healthy women, the normal early diastolic
early mitral annular velocities were 11.8 3.2 cm/s in
women and 10.8 3.0 cm/s in men.5 When comparing
the E-wave deceleration time in men versus women, it is
shorter in women. Furthermore, it has been reported that
the left ventricular end-diastolic diameter (LVEDD) and
left ventricular end-systolic diameter (LVESD) volumes
decrease with age in both men and women, but the
changes are more pronounced in women. Also, the muscle
mass and systolic function, assessed by left ventricular
ejection fraction (LVEF), increase with age, but more so in
women than men.
In women who are obese or have large breasts, this
poses a challenge to the sonographer. Furthermore, if it is
technically difficult to obtain the ultrasound images due
to the patients body size and/or pendulous breasts, then
it proves to be even more difficult to interpret the images.
Breast implants can also cause significant technical
challenges. In 1992, silicone implants were removed from
the market, however; there were some silicone implants
being implanted and studied for investigative purposes.

Chapter 78: Echocardiography in Women

1887

Table 78.1: Reference Limits and Partition Values of Left Ventricular Mass and Geometry

Women

Men

Reference
Range

Mildly
Abnormal

Moderately
Abnormal

Severely
Abnormal

Reference
Range

Mildly
Abnormal

Moderately
Abnormal

Severely
Abnormal

67162

163186

187210

211

88224

225258

259292

293

4395

96108

109121

122

49115

116131

132148

149

Linear Method
LV mass, g
2

LV mass/BSA, g/m

LV mass/height, g/m

4199

100115

116128

129

52126

127144

145162

163

LV mass/height2,7, g/m2,7

1844

4551

5258

59

2048

4955

5663

64

Relative wall thickness, cm

0.220.42

0.430.47

0.480.52

0.53

0.240.42

0.430.46

0.470.51

0.52

Septal thickness, cm

0.60.9

1.01.2

1.31.5

1.6

0.61.0

1.11.3

1.41.6

1.7

Posterior wall thickness, cm 0.60.9

1.01.2

1.31.5

1.6

0.61.0

1.11.3

1.41.6

1.7

66150

151171

172182

> 193

96200

201227

228254

> 255

4488

89100

101112

113

50102

103116

117130

131

2D Method
LV mass, g
2

LV mass/BSA, g/m

Source: Reproduced from Roberto M. Lang, Michelle Bierig, Richard B. Devereux, et al. Recommendations for Chamber Quantification:
A Report from the American Society of Echocardiographys Guidelines and Standards Committee and the Chamber Quantification
Writing Group, Developed in Conjunction with the European Association of Echocardiography, a Branch of the European Society of
Cardiology. J Am Soc Echocardiogr. 2005;18:144063.
(BSA: Body surface area; LV: Left ventricular; 2D: 2-dimensional).
Bold italic values: Recommended and best validated.

Figs 78.1A and B: Breast implant. Two-dimensional transthoracic echocardiography. (A) Arrows point to images produced by a saline
breast implant; (B) Apical four-chamber view. Arrow points to a breast implant and the arrowhead to a pacing wire in the region of the
tricuspid valve, which shows fatty infiltration (F). A prominent moderator band (M) is seen in the right ventricle. (LA: Left atrium; LV: Left
ventricle; RA: Right atrium). (Movie clips 78.1A and B).

Consequently, the majority of implants in the late 1990s

and early 2000s are saline. Then, in 2006, the Food and
Drug Administration (FDA) approved the first silicone
filled implant for the use of breast augmentation. Even
more recently, there have been approvals by the FDA for
a new silicone gel-filled implant. This newer implant is

reported to have a more cohesive gel than the previous

silicone implants.6 As the number of women having
silicone breast implants is expected to increase, there will
continue to be limitations in diagnostic cardiac testing
such as echocardiography. Saline and silicone breast
implants interfere with the penetration of ultrasound

1888

Section 7: Miscellaneous and Other Noninvasive Techniques

beams and produce artifacts6 (Figs 78.1A and B). Despite

attempts from the sonographer to increase the gain or
change the ultrasound waves frequency, the images are
still of poor quality and cluttered with artifacts. These
make interpretation of echo findings more difficult. The
artifacts which are produced from the breast implants are
similar in some ways to the artifacts caused by air in the
lung. To obtain interpretable echocardiographic views, the
echocardiographer often needs to find alternative windows
such as the subcostal approach which avoids passage of
the ultrasound beam through the implants.6 Sometimes,
depending on the size of the breast implant, a sonographer
can modify the standard echocardiographic views in order
to prevent the ultrasound beam from crossing the silicone
implant.6 However, even with attempts to reposition the
probe in order to obtain better quality echocardiographic
images, the images still remain suboptimal. Furthermore,
there is significant limitation in the ability to interpret the
study.

STRUCTURAL HEART DISEASE: MVP,

MITRAL STENOSIS, AND MITRAL
ANNULAR CALCIFICATION
Mitral Valve Prolapse

between 5% and 15%. Using the more modern criteria,

the prevalence of MVP in the general population is
estimated at 25%.7 Comparing the two genders, women
tend to have less posterior prolapse, less flail, but more
leaflet thickening.8 Also, compared to men, women
tend to have less frequent severe regurgitation. M-mode
echocardiography allows for the diagnosis of MVP with
obvious leaflet thickening and posterior bowing of the
mitral valve apparatus during systole.79 On the twodimensional (2D) echocardiogram MVP is diagnosed
by noting localized displacement of one or both leaflets
into the left atrium with or without mitral regurgitation.
Chordal rupture may complicate MVP (Figs 78.2 and 78.3).
Women with MVP tend to have both cardiac as well as
noncardiac abnormalities. Examples of this include skeletal
abnormalities such as pectus excavatum and scoliosis.9
Other associations with MVP include atypical chest pain,
easy fatiguability, abnormal electrocardiographic (EKG)
response to exercise (ST-T changes), and a variety of
atrial and ventricular arrhythmias. The collection of these
symptoms in the setting of MVP is often termed the MVP
syndrome (MVPS) or dysautonomia. It has been reported
that the clinical features are due to autonomic dysfunction.
Both the sympathetic and parasympathetic nervous
systems are likely involved, but not well understood

Mitral valve prolapse (MVP) and regurgitation is seen

more commonly in women. Some studies report that MVP
affects ~6% of women. MVP was overdiagnosed during the
1970s and 1980s due to lack of strict echocardiographic
criteria. Earlier studies estimated the prevalence of MVP

Mitral Valve Stenosis in Women

Epidemiologic studies conducted in Western countries

have demonstrated a higher prevalence of mitral stenosis
in women. The largest study conducted to date included

Figs 78.2A and B: Mitral valve prolapse in a 42-year-old patient. (A) Two-dimensional transthoracic echocardiography. Arrowhead
points to prolapse of both mitral leaflets. (B) M-mode study was useful in demonstrating prolapse in mid to late systole (arrow).
(AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RV: Right ventricle; SVC: Superior vena cava). (Movie clip 78.2).

Chapter 78: Echocardiography in Women

1889

Figs 78.3A and B: Ruptured chordae tendinae and mitral valve prolapse in a 71-year-old female. Live/real time three-dimensional
transesophageal echocardiography. (A) The arrowhead shows a ruptured chord of a severely prolapsing A2 segment of the anterior
mitral valve leaflet (AML); (B) Color Doppler imaging. Numbers 1 and 2 point to two jets of severe MR. The arrowhead points to the ruptured chord. (LA: Left atrium; MV: Mitral valve; MR: Mitral regurgitation; PML: Posterior mitral valve leaflet). (Movie clips 78.3A and B).
Source: Reproduced with permission from Nanda et al. Comparison of real time two-dimensional with live/real time three-dimensional
transesophageal echocardiography in the evaluation of mitral valve prolapse and chordae rupture. Echocardiography. 2008;25:11317.

echocardiograms performed on 12,926 women and 11,339

males to evaluate for mitral stenosis.10 The prevalence for
women is estimated at 1.8%. It is not clearly understood
why the association with the female sex exists.10 Furthermore, it has been shown that women, in addition to
being more prone to the development of rheumatic heart
disease/mitral stenosis, are at higher risk of death in the
setting of rheumatic heart disease.11 Figures 78.4 to 78.10
demonstrate various aspects of echocardiographic findings in rheumatic mitral valve disease. The color Doppler
examination in Figures 78.4A to F nicely illustrates a turbulent jet in the LV which originates from the mitral valve
as well as the prominent flow acceleration, characteristic
of mitral stenosis. The continuous wave Doppler further
illustrates the severity of mitral stenosis by demonstrating
a significant mean gradient.

Mitral Valve Calcification

Although mitral annular calcification is a chronic degenerative process and progresses with increasing age, it is
more commonly seen in women, especially those over
the age of 70 (Figs 78.11A to C). Consequences of mitral
annular calcification may include mitral stenosis, mitral
regurgitation, infective endocarditis, atrial arrhythmias,
and heart block. It has been shown that the presence of
mitral annular calcification on echocardiography may be

a marker of obstructive CAD, especially in the setting of

anginal symptoms.12 Furthermore, in women, the absence
of mitral annular calcification was a marker for the absence
of obstructive CAD. However, this did not hold true for
men.

ISCHEMIC HEART DISEASE/STRESS

ECHOCARDIOGRAPHY/POLYCYSTIC
OVARIAN SYNDROME
Ischemic heart disease remains the leading killer in the
United States. Although there have been advances in
treating ischemic heart disease (IHD), there still remains
a significant growth in prevalence of IHD. It has been
shown in prior studies that 38% of deaths in women were
related to CAD. This will only likely continue to increase
as the population continues to age.13 Furthermore, more
women than men, annually, have died from CAD, refuting
the old belief that heart disease was a mans disease. It
has been shown that women who are critically at risk for
CAD are often missed by the traditional approaches to
disease management, partly because of atypical clinical
presentation and low suspicion of index on the part of the
examining physician.
Women represent a unique and challenging patient
population to the clinician. Women tend to have greater
symptom burden and a lower prevalence of obstructive

1890

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.4A to F: Rheumatic mitral stenosis. Two-dimensional transthoracic echocardiography in a 45-year-old female with
a history of rheumatic fever several years ago. (A) Arrow points to thickened mitral valve leaflets with a typical hockey-stick
appearance visualized in the parasternal long axis view; (B) Short-axis view of the mitral valve (MV) at the leaflet tips. MV area
measured 1.10 cm2 suggestive of fairly severe stenosis; (C) Color Doppler examination shows a turbulent jet (arrowhead) in the
left ventricle (LV) originating from the MV. Note the prominent flow acceleration (arrow); (D) Color Doppler guided continuouswave Doppler show peak and mean gradients of 34 and 19 mm Hg, respectively; (E) Pressure half time assessment also
showed severe MV stenosis with a valve area of 0.87 cm2; (F) Live/real time three-dimensional study confirmed the presence of
severe stenosis with no calcification of commissures. There was only mild mitral regurgitation. The patient is on the wait list for
percutaneous mitral valvuloplasty (Movie clips 78.4A, C, and F). (AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
RV: Right ventricle).

Chapter 78: Echocardiography in Women

1891

Figs 78.5A to E: Rheumatic mitral stenosis in 34-year-old female

patient with history of paroxysmal nocturnal dyspnea. Two-dimensional transthoracic echocardiography. (A and B) Parasternal long
axis (A) and apical four-chamber (B) views show thickened mitral
valve leaflets with doming in diastole indicative of mitral stenosis.
The subvalvular apparatus is also thickened; (C) Color Doppler
examination shows mild mitral regurgitation and moderate aortic
regurgitation; (D) Live/real time three-dimensional transesophageal
echocardiography demonstrates a very small mitral orifice (arrow)
indicative of severe stenosis; (E) Severe mitral regurgitation
developed following percutaneous mitral valvuloplasty (Movie
clips 78.5A to E). (PA: Pulmonary artery; RA: Right atrium). Other
abbreviations as in previous figure.

CAD on coronary angiography, compared to men. Interestingly, women often times have a more adverse outcome
despite having lower angiographic disease burden.

In women, the prevalence of obstructive CAD is low

before menopause, the average age of menopause being
~51 years.13 After the age of menopause, the prevalence

1892

Section 7: Miscellaneous and Other Noninvasive Techniques

of CAD in women increases to become almost equal with

men at the age of 70 years (Figs 78.12 and 78.13). While
women may have overall lower rates of hypertension and
smoking, both elderly hypertensive women and young
female smokers are very prominent at risk subgroups.
Population studies have also demonstrated that women

have lower total cholesterol measurements until about the

age of 50 years. Above the age of 50 years, women tend to
have greater values. The risk factors for CAD seen in post
menopausal women consist of obesity, hypertension, and
dyslipidemia. The clustering of the risk factors commonly
termed the metabolic syndrome consists of the following

Figs 78.6A and B: Female patient with rheumatic involvement of mitral and tricuspid valves. Transthoracic three-dimensional echocardiography. (A) Apical four-chamber view showing thickened mitral (MV) and tricuspid (TV) valves. (B) Cropping of the three-dimensional
data set and en face viewing shows a very small mitral orifice in diastole indicative of severe stenosis. Note absence of calcification in
the commissures. In comparison, the TV shows a much larger opening consistent with absence of significant stenosis. The movie clip
shows fairly preserved motion of anterior (A) and posterior (P) leaflets but marked restriction of the septal (S) leaflet of the tricuspid
valve. The septal leaflet is identified by its close proximity to the ventricular septum. The anterior and posterior leaflets are recognized
by their anterior and posterior locations in relation to the left ventricular outflow (LVO) tract. The three-dimensional technique is considered the gold standard for assessing the mitral orifice area because, unlike two-dimensional echocardiography, the cropping plane can
be positioned exactly parallel to the flow limiting orifice tip. Also, three-dimensional echocardiography easily assesses all three leaflets
of the TV en face which is difficult with two-dimensional imaging (Movie clip 78.6). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).

A
Figs 78.7A and B

Chapter 78: Echocardiography in Women

1893

Figs 78.7A to D: Mitral stenosis and regurgitation in a female patient. Transthoracic three-dimensional echocardiography. (A) Apical
four-chamber view shows restricted mobility of both mitral valve (MV) leaflets which are thickened; (B) Cropping of the three-dimensional
data set was performed to view the mitral orifice (arrow) at its tip. Planimetry was consistent with significant stenosis. Note presence of
calcification in the body of the posterior leaflet but the anterior leaflet and both commissures are free of calcium; (C) Mosaic color signals
in the left atrium with a prominent flow acceleration point to severe mitral regurgitation; (D) Meticulous cropping of the three-dimensional
data set was done to view en face the vena contracta of the mitral regurgitation jet. This was performed practically at the level of the
mitral leaflets between the flow acceleration and the regurgitant jet. It measured more than 0.6 cm2 indicative of severe regurgitation
(Movie clip 78.7). Other abbreviations as in previous figures.

Figs 78.8A and B: (A) Rheumatic mitral stenosis and regurgitation in a female patient. Transesophageal two-dimensional echocardiography. Four-chamber view shows thickening of both mitral leaflets with restricted motion. Note thickening of chordal apparatus also;
(B) Color Doppler examination demonstrates significant mitral regurgitation (MR) (Movie clips 78.8A and B). Other abbreviations as in
previous figures.

conditions: insulin resistance, dyslipidemia [elevated

triglycerides, low high-density lipoprotein (HDL)], hypertension, and abdominal obesity. The chest pain symptoms
which women experience may be explained by the
metabolic alterations which occur. This may result in

shifting energy substrates toward myocardial and peripheral glucose metabolism. Therefore, the traditional
stress testing which is based on demand ischemia may fail
to detect obstructive CAD in subsets of women without a
significant coronary stenosis.13

1894

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.9A and B: Rheumatic mitral stenosis and regurgitation in a female patient. Transesophageal three-dimensional echocardiography. (A) En face view of the mitral valve (MV) shows mild narrowing of the thickened MV consistent with mild to moderate
stenosis; (B) Systolic view shows noncoaptation of the mitral leaflets indicative of significant mitral regurgitation. (AV: Aortic valve).
(Movie clip 78.9).

Figs 78.10A and B: Rheumatic mitral stenosis in another female patient. Transesophageal three-dimensional echocardiography.
(A) The left atrium is viewed from the top and shows a large clot attached to the supero-posterior wall. The mitral valve (MV) is heavily
calcified; (B) Three-dimensional two-chamber view demonstrates clots (arrowheads) in the body and appendage of the left atrium
(Movie clips 78.10A and B). Abbreviations as in previous figures.

Using Stress Echocardiography in

Women
In some patients with obstructive CAD, resting echocardiograms may demonstrate tell-tale wall motion abnormalities or an akinetic or dyskinetic area with fibrotic scar
formation may reveal the presence of an old myocardial
infarction which may have been silent or undiagnosed.
However, in most patients the diagnosis of obstructive CAD

is made by stress echocardiography. Treadmill exercise

echocardiography is most commonly used because it
results in greater patient oxygen consumption and therefore a higher sensitivity in detecting exercise induced wall
motion abnormalities and diagnosing CAD as compared
to pharmacologic agents such as dobutamine. The usage
of pharmacologic agents (dobutamine or dipyridamole)
to perform stress echocardiography helps to overcome
the challenges of women who are incapable of maximal

Chapter 78: Echocardiography in Women

1895

Figs 78.11A to C: Mitral annulus calcification in an 80-year-old

female. Two-dimensional transthoracic echocardiography. (A to C)
Arrowhead points to a heavy mitral annular calcification while the
arrows show aortic annular calcification. The aortic valve leaflets
are also calcified with restrictive opening and continuous-wave
Doppler demonstrated high velocity consistent with significant
stenosis (Movie clips 78.11A and B). (AO: Aorta; LA: Left atrium;
LV: Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right
ventricle).

exercise. The advantages of stress echocardiography

include the lower cost, absent radiation exposure, and the
ability to image both ventricular function as well as cardiac
structures. However, the utility of echocardiography can
be limited if factors such as obesity exist which will limit
acoustic windows. Furthermore, it is known that as women
progress through menopause, they seem to have a greater
loss in physical functioning when compared with men.
Thus, if there is reduced exercise tolerance, the utility of
stress echocardiography is markedly reduced. Acquisition
of peak stress echocardiography images is limited by
the experience of the sonographer and the clinician.
Even with the limitations, exercise echocardiography is
highly accurate at detecting CAD in women. Both the
sensitivity and specificity of stress echocardiography are
comparable to radionuclide techniques with the added
advantage that one does not have to cope with breast
shadows which may interfere with accurate interpretation
when reading radionuclide studies in women. Stress

echocardiography is very useful in diagnosing 2 and 3

vessel disease with accuracy exceeding 80% to 85% but,
like radionuclide testing, is less useful in diagnosing
1 vessel disease especially involving the circumflex artery.
Contrast echocardiography serves as a valuable adjunct
to stress echocardiography in patients with suboptimal
acoustic windows. Intravenous injections of commercially
available contrast agents produce full opacification of
both right and left ventricular cavities resulting in complete delineation of the endocardial borders facilitating
accurate assessment of wall motion abnormalities. Echo
contrast agents consist of microbubbles of a nontoxic
gas smaller or same size as the red blood cells and they
travel with them into the coronary circulation and hence
they opacify the ventricular myocardium also. This
property has been used to evaluate myocardial perfusion
abnormalities developing during stress echocardiography
by demonstrating areas of reduced or absent opacification
providing corroborative evidence of the presence of

1896

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.12A and B: (A) Aortic atherosclerosis in a 72-year-old

female with coronary artery disease, systemic hypertension and type
2 diabetes mellitus presenting with chest pain. Two-dimensional
transthoracic echocardiography. Suprasternal examination. Arrowhead points to a mobile plaque in the proximal descending
thoracic aorta (DA); (B) Aortic atherosclerosis in the same patient
as above. Two-dimensional transesophageal echocardiography
performed to rule out aortic dissection. Arrowhead in the left panel
points to a large mobile plaque in the anterior portion of the DA,
arrowhead in the right panel demonstrates a large ulcer in the
plaque posteriorly. Ischemic heart disease in a 75-year-old female.
Two-dimensional transthoracic echocardiography. The parasternal
long-axis view shows dyskinesis of the proximal left ventricular
posterior wall (PW). (Movie clips 78.12A to C). (ACH: Aortic arch;
IA: Innominate artery; LA: Left atrium; LCC: Left common carotid
artery; PA: Pulmonary artery; AO: Aorta; LA: Left atrium; RV: Right
ventricle; SVC: Superior vena cava; VS: Ventricular septum).

B
obstructive CAD. More recently, live/real time threedimensional (3D) echocardiography is increasingly used
together with contrast echocardiography in an attempt to
further enhance the accuracy of stress echocardiography
in assessing CAD. With 3D echocardiography, it is
possible to capture the whole LV in the pyramidal shaped
3D data set which can then be cropped in a systematic
and sequential manner to examine all ventricular walls
and segments for stress induced motion abnormalities.
This obviates the limitations of the 2D technique which
produces only thin slice-like sections of the LV at any
given time precluding comprehensive assessment of
all segments. Apical foreshortening, common with 2D
echocardiography, is avoided or reduced with the 3D
approach which also has been shown to have much less
intra- and interobserver variability in the assessment of
left ventricular function. Another advantage is the 3D
data can be stored in the equipment or offline and can

be recropped at will any time by the same or different

cardiologist to double check the findings. A significant
disadvantage relates to the quality of 3D images which is
lower than 2D images and hence the technique is useful
only in patients with good acoustic windows and in those
in whom the image quality has been enhanced by contrast
echocardiography.
Although not commonly used, there are reported
cases where transesophageal echocardiogram has actually
revealed the diagnosis of CAD. It should be standard
protocol to interrogate the left and right coronary arteries
when they are visualized during a routine transesophageal echocardiogram exam. Figures 78.13A to M nicely
demonstrate images of CAD. Notice the utilization of
pulsed Doppler interrogation which reveals high diastolic
velocities, consistent with severe stenosis. The coronary
arteriograms included confirm the severity of the stenosis
suspected by TEE.

Chapter 78: Echocardiography in Women

Figs 78.13A to F

1897

1898

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.13G to L

Chapter 78: Echocardiography in Women

1899

Figs 78.13A to M: Coronary stenosis in a 61-year-old female. Transesophageal echocardiographic examination. (A) Top arrow points
to dilatation of the proximal left anterior descending coronary artery
(LAD), whereas the bottom arrow demonstrates turbulent flow in
mid-LAD. Pulsed Doppler interrogation of this area revealed a high
diastolic velocity of 1.3 m/s (arrowhead in the inset), consistent
with significant stenosis; (B) Top arrow points to turbulent flow in
mid-LAD, whereas the bottom arrow shows turbulent flow in the
more distal portion of LAD; (C) Arrow demonstrates narrowing in
the first diagonal branch (arrowhead) of LAD. Continuous-wave
Doppler interrogation reveals a very high diastolic velocity of 3.0
m/s (arrowhead in the inset) indicative of very severe stenosis; (D)
Arrows show large segments of proximal, mid and distal segments
of LAD visualized in this view; (E) Arrow demonstrates the presence
of turbulent flow in the proximal right coronary artery; (F) Pulsed
Doppler interrogation (arrowhead) of right coronary artery (RCA)
M
demonstrates a high velocity of 1.3 m/s (arrowhead in the inset)
indicative of significant stenosis; (G) Arrow points to the presence of turbulent flow in the left circumflex coronary artery; (H) Arrow points
to the origin of the left circumflex coronary, which appears normal. However, reversed flow (blue) with turbulent flow signals (arrowhead)
is noted in the mid and distal portions of the circumflex vessel, consistent with filling from collaterals and significant stenosis. Doppler
interrogation of this area shows a high diastolic velocity of 1.0 m/s (arrowheads in the inset); (I) Arrowhead demonstrates turbulent flow
signals in the intramyocardial coronary arteries consistent with stenosis; (J) The interventricular vein (V) is imaged next to the dilated
LAD (arrow). Note that the flow in the interventricular vein is in the opposite direction of LAD flow; (K) Coronary angiogram. The top
arrowhead demonstrates 90% stenosis at the origin of the diagonal branch. The proximal LAD is dilated, and beyond the dilatation,
the mid-LAD shows 50% stenosis (just beyond the bottom arrowhead). Note multiple areas of significant stenosis in the more distal
segments of LAD; (L) Arrow points to total occlusion of proximal circumflex coronary artery. Retrograde filling of more distal portions of
the circumflex vessel from collaterals was noted; (M) Coronary angiogram. The arrow points to significant stenosis in the proximal right
coronary artery. Note significant stenosis in the mid and distal portions. (AO: Aorta; LA: Left atrium; LM: Left main coronary artery; LV:
Left ventricle; MV: Mitral valve; PA: Pulmonary artery; RVO: Right ventricular outflow tract). Reproduced with permission from Nanda
et al. Transesophageal echocardiographic diagnosis of coronary stenosis in a stroke patient. Echocardiography. 1999;16:58992.
In Movie clip 78.13, 1, Left anterior descending coronary artery; 2, Diagonal branch; 3, Proximal right coronary artery; 4, circumflex
artery; 5, Intramyocardial coronary arteries (cannot be visualized by angiogram). V, coronary vein. Upper arrow shows proximal LAD.
Lower arrow shows distal LAD. Arrowhead points to post-stenotic dilatation of the diagonal branch.

Polycystic Ovarian Syndrome/Syndrome

X in Women
Women with polycystic ovarian syndrome (PCOS) have
been shown to be at higher risk for CAD compared to
women without PCOS. Women with PCOS have risk factors
which mirror a risk factor profile for that of a man. These
risk factors include: anovulation, hyperandrogenism, and
insulin resistance. PCOS could be thought of as the best
example of syndrome X. If the cluster of risk factors known
as the metabolic syndrome/syndrome X are indeed risk
factors of atherosclerosis and CAD, then women with
PCOS should have more atherosclerosis than women
without PCOS, especially at younger ages.14 Therefore,
these women with PCOS would need to be followed as the
risk of developing obstructive CAD would be higher.

TAKOTSUBO CARDIOMYOPATHY
Takotsubo cardiomyopathy is a very common clinical
entity which occurs far more often in women than in

men (9:1 female/male ratio). The echocardiographic

features include transient dyskinesia/akinesia usually
localized to the apex of the LV (see Movie clips 78.26A and
78.26B in chapter on Nonobstructive Cardiomyopathy).
Associated involvement of the left atrium has also been
reported.11 In some patients only the middle portion of
the ventricle is involved. The clinical presentation is very
similar to that of acute coronary syndrome. The clinical
history often reveals that the person has been under
extreme emotional or physical stress which precipitates
the abrupt onset of symptoms, including chest pain and
shortness of breath. Examples of emotional stressors
include grief, fear, anger, relationship conflicts, and
financial problems. Examples of physical stressors include
acute asthma exacerbation, surgery, chemotherapy, or
stroke. This form of cardiomyopathy has a predilection
for women, specifically those over the age of 50.15,16 Only
10% of cases have been reported to occur in men. Usually,
patients present to the emergency department with
concerns of having a myocardial infarction. Initially, both

1900

Section 7: Miscellaneous and Other Noninvasive Techniques

Takotsubo and acute coronary syndromes share a similar

presentation, with abnormalities in the electrocardiogram
(ECG) as well as biomarkers. In fact, this syndrome has
been reported to represent 1.5% to 2.2% of acute coronary
syndrome that presents with ST elevation or Q-waves on
EKG.16,17 As a result, most patients are taken to the cardiac
catheterization laboratory and are found to have normal
coronary arteries in addition to the unique shape of the LV
which is seen during the LV gram. This unusual shape of
the LV, which resembles a Japanese octopus pot, is what
has given Takotsubo its name.
The classic case of stress induced cardiomyopathy
is characterized by the presence of apical ballooning
involving all left ventricular walls with a hyperdynamic
base. These abnormalities are not limited to single
coronary artery territory. There are theories to explain
why the apex is affected and the base remains unaffected.
This could be due to the fact that the apex is considered
to be more responsive to adrenergic stimulation.11,1519 This
assumes that the catecholamine surge is the mechanism
of Takotsubo.15 Echocardiography can prove to be very
useful in the early diagnosis of this disease, and could
actually prevent patients from undergoing unnecessary
cardiac catheterizations given the detection of wall
motion abnormalities not confined to typical coronary
artery distribution in association with recent emotional
or physical stressors. Echocardiography is also useful
in assessing complications of Takotsubo such as clot
formation in the LV apex and in the follow-up of these
patients. Some of these patients show full recovery with
complete normalization of wall motion abnormalities
while in others residual wall motion abnormalities persist
for a long time.

Vector velocity imaging (VVI) has been used to demonstrate that in patients with Takotsubo cardiomyopathy,
there is both LV systolic and diastolic longitudinal dysfunction, not just systolic radial dysfunction.11 Traditional
2D echocardiography is typically used to assess radial dysfunction. VVI uses a tracking algorithm which incorporates both velocities of set points (i.e. mitral annulus and
tissue-cavity border) as well as speckle tracking. The result
is segmental quantitative velocity, strain, and strain rate.
VVI has demonstrated that there are definitive reductions
in both longitudinal systolic and diastolic dysfunction in
Takotsubo cardiomyopathy.11 It has also been shown that
there is improvement in the longitudinal function with
time. Furthermore, there has been evidence to suggest that
the left atrium is also affected in Takotsubo cardiomyopathy.11 Specifically, the left atriums systolic strain and strain
rate and the diastolic velocity and strain rate are reduced.
However, there is some data to suggest that there is improvement in the involved walls of the left atrium.11

Figs 78.14A and B

CONGENITAL HEART DISEASE

Atrial Septal Defects
Congenital heart disease seems to have an association with
gender. Defects which involve the inflow tract such as the
atrial septal defect and Ebsteins (see Figures 57, 58, and
59 in chapter on Three-Dimensional Echocardiography
in Congenital Heart Disease) are seen most commonly in
females (Figs 78.14 and 78.15). Of patients with secundum
atrial septal defects (ASDs), 6575% are female. In
contrast, there is equal gender distribution for both sinus
venosus and ostium primum ASDs.2024 Outflow tract
abnormalities such as aortic stenosis and transposition

Chapter 78: Echocardiography in Women

1901

Figs 78.14A to C: Secundum atrial septal defect in a 53-year-old

female. Two-dimensional transthoracic echocardiography. (A and B).
Apical four-chamber views show an atrial septal defect (arrows) with
flow signals moving from the left atrium (LA) into the right atrium
(RA). The right ventricle (RV) is enlarged; (C) is a suprasternal
view in the same patient showing continuity of flow signals (arrow)
between the pulmonary artery (PA) and the descending thoracic
aorta (DA) indicative of an associated patent ductus arteriosus.
(ACH: Aortic arch, LV: Left ventricle). Movie clips 78.14A to C.

Fig. 78.15: Secundum atrial septal defect in a 37-year-old female. Two-dimensional transesophageal echocardiography. Arrow points
to flow signals moving from the left atrium into the right atrium through a secundum defect. Abbreviations as in previous figure. Movie
clip 78.15 is from another female patient demonstrating en face visualization of a secundum atrial septal defect (upper arrow) using
live/real three-dimensional tranesophageal echocardiography. Note a large rim in relation to the aorta (AO), tricuspid valve (lower left
arrow), and mitral valve (MV). Lower right arrow points to the pulmonary valve. (PA: Pulmonary artery). Other abbreviations as in previous figure.

of the great arteries are more commonly seen in men.

Atrial and ventricular septal defects also have a significant
association with pulmonary hypertension. There has been
some thought that the secundum ASD begins the cascade
of vascular injury ultimately resulting in the pulmonary
vascular remodeling and finally the development of
pulmonary arterial hypertension. This could explain the
gender disparities in pulmonary arterial hypertension.23

Pulmonary Hypertension
Pulmonary arterial hypertension is typically defined as
a measured mean pulmonary artery pressure (mPAP)

greater than 25 mm Hg, in conjunction with a normal

cardiac output and normal pulmonary capillary wedge
pressure (Figs 78.16 to 78.18).25 The association with the
female sex is quite interesting. In fact, women have a 35%
higher risk of developing pulmonary hypertension.25,26
Idiopathic pulmonary arterial hypertension is thought to
be rare, with an incidence of 2 to 5/million/year. However,
it is much more common in women compared to men with
a reported ratio of 2.5:1.25,26 Various genetic mutations and
polymorphisms have been reported to be associated with
idiopathic pulmonary arterial hypertension, and none
appear to be sex-linked.

1902

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.16A to D: Severe systemic level pulmonary artery pressure in a 37-year-old female with primary pulmonary hypertension.
Two-dimensional transthoracic echocardiography. Apical views. (A) Note the marked enlargement of both the right ventricle (RV) and
the right atrium (RA). The atrial septum bulges prominently into the left atrium (LA). Movie clip 78.8A shows diastolic bulging of the ventricular septum into the left ventricle (LV) indicative of right-sided volume overload; (B) Color Doppler examination shows the presence
of severe tricuspid regurgitation (TR); (C) Continuous-wave Doppler examination reveals a very high pulmonary artery systolic pressure
of 118 mm Hg (arrow); (D) Bubble study shows delayed appearance (after 4 beats) of the micro bubbles (arrowhead) in the left heart
consistent with intrapulmonary shunting. This is related to dilatation of the pulmonary arterioles. (LV: Left ventricle; TV: Tricuspid valve).
(Movie clips 78.16A to C).

In the included figures, specifically Figure 78.16, there

is an excellent example of severe pulmonary hypertension
in a young woman. The classic features of pulmonary
hypertension include the marked enlargement of both
the right ventricle and right atrium. Furthermore, there
is diastolic bulging of the interventricular septum from
right to left indicating the significant right-sided volume
overload. Color Doppler highlights the severe tricuspid
regurgitation seen in cases of pulmonary hypertension.
A bubble study should always be performed in a case of
pulmonary hypertension as it will reveal the presence of

intrapulmonary shunting. Intrapulmonary shunting is

seen when there is a delayed appearance of microbubbles
in the left side of the heart.

ECHOCARDIOGRAPHY IN PREGNANCY,
PERIPARTUM CARDIOMYOPATHY,
FETAL ECHOCARDIOGRAPHY
Many physiological changes develop in many organ
systems during the course of pregnancy. Cardiac output
increases by 30% to 40% during the first trimester of

Chapter 78: Echocardiography in Women

1903

Figs 78.17A and B: Severe pulmonary hypertension with right-sided involvement. Two-dimensional transthoracic echocardiography.
(A and B) The tricuspid valve (TV) annulus is dilated with systolic noncoaptation (arrow) of the leaflets leading to severe tricuspid regurgitation (arrowhead). The pulmonary annulus is also dilated resulting in severe pulmonic valve regurgitation (PR). (CS: Coronary sinus;
DA: Descending aorta; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle). (Movie clips 78.17A and B).

Figs 78.18A to C: Pulmonary artery aneurysm in a 21-year-old

female patient with familial primary pulmonary hypertension. Twodimensional transthoracic echocardiography. (A) The pulmonary
artery (PA) is markedly enlarged at 4.89 cm; (B) The right ventricle
(RV) is larger than the left ventricle (LV) in the apical four-chamber
view. Note displacement of the ventricular septum into the LV
during systole; (C) Bubble study was negative for intracardiac
or intrapulmonary shunting. (LA: Left atrium; MV: Mitral valve;
PV: Pulmonary valve; RA: Right atrium; TV: Tricuspid valve).
(Movie clips 78.18A to C).

1904

Section 7: Miscellaneous and Other Noninvasive Techniques

pregnancy. These changes can be seen on Doppler echocardiography with increased blood flow velocities and
more prominent color Doppler flow signals in the cardiac
cavities. Increased color Doppler flow signals in the region
of the atrial septum may mimic an atrial septal defect
especially in the presence of septal dropouts common in
the apical four-chamber view. In this instance, continuity
of color flow signals between the two atria in the region
of the septal dropout may result in an erroneous diagnosis
of a secundum atrial septal defect. At 8 to 11 weeks,
an average cardiac output of 6.7 L/min will increase to
8.7 L/min at 36 to 39 weeks. This increase is mostly due to
an increase in stroke volume, but also a more rapid
heart rate. Also, there is a reduction in systemic vascular
resistance. It is these changes which characterize the
hyperdynamic circulation seen in pregnancy. On the
echocardiogram, hyperkinetic ventricular wall motion is
evident during this period of pregnancy.

Peripartum Cardiomyopathy

Figs 78.19A to D

In some women, the extra workload of the heart results in

ventricular failure in the peripartum period (Figs 78.19A
to G). Peripartum cardiomyopathy is characterized by
systolic dysfunction, ventricular dilatation, and secondary
mitral regurgitation, all well assessed by echocardiography.
Thrombus formation especially in the left ventricular apex
may be noted. This is a worrisome complication because
of the potential for embolization and anticoagulants are
essential. Anticoagulants like warfarin which facilitate
the natural clot lytic mechanisms present in the blood
stream work by first initiating a small area of liquefaction
within the middle portion of a thrombus and this gradually
spreads all the way to the periphery eventually resulting
in complete resolution of the thrombus. Thus, on serial
follow-up of 2D transthoracic echocardiograms the size
of a thrombus may appear unchanged and anticoagulant

Chapter 78: Echocardiography in Women

1905

Figs 78.19A to G: Peripartum cardiomyopathy. Live/real time three-dimensional transthoracic echocardiography in a 29-year-old
female. (A) Arrow points to a large clot in the left ventricular (LV) apex. Both ventricles showed poor function; (B to D) A transverse plane
(TP) section through the clot (B) shows a large area of echolucency (arrow) consistent with clot lysis (C and D). This indicated that the
clot had practically completely dissolved and only a thin rim remained; (E) Schematic showing coagulation cascade and thrombus lysis.
Plasminogen-activating factor (PAF) is secreted by endothelialized mesenchymal cells lining the microscopic crypts which develops
within the thrombus. PAF activates plasminogen to plasmin which digests fibrin leading to thrombus lysis. (F and G) A TP section taken
at the attachment point of the clot shows it to be highly echogenic consistent with collagen. This patient had been on anticoagulant
therapy for a long time but on the two-dimensional study the clot did not appear to regress. However, the three-dimensional technique
was useful in assuring us the effectiveness of anticoagulant therapy with almost complete clot resolution. The remainder of the clot
regressed completely with no clinical or laboratory evidence of embolization (Movie clips 78.19). (vWF: von Willebrand factor; TF: Tissue
factor; WBC: White blood cell; RBC: Red blood cell; CF: Clotting factors). Other abbreviations as in previous figures.

therapy ineffective although the thrombus in fact may be

undergoing progressive liquefaction and resolution from
within. In these cases, it is important to perform live/real
time 3D echocardiography which can provide multiple
short-axis sections of the thrombus and comprehensively
assess the presence and extent of clot lysis. The technique
can also assess the cross-sectional size and nature of the
area of the attachment of the thrombus to the ventricular
wall and this may provide information regarding its
potential for embolization. For example, a mobile thro-

mbus with a small tenuous cross-sectional attachment

site may be more prone to embolization than one with
a much larger attachment area. Also, a bright echogenic
attachment area suggests increased collagen content and
fibrosis providing a more firm and stable attachment of the
clot to the ventricular wall.
Women with peripartum cardiomyopathy will usually
present shortly after childbirth, but there are some women
who will have the clinical presentation consistent with
peripartum cardiomyopathy late in the third trimester.

1906

Section 7: Miscellaneous and Other Noninvasive Techniques

In high risk pregnancies, fetal echocardiography plays a

very important role in evaluating normal and abnormal

fetal cardiac anatomy and physiology (Figs 78.20 to 78.22).

M-mode studies of the mitral valve and Doppler tracings
of mitral and tricuspid inflow have been found useful in
evaluating fetal arrhythmias.27 Using both 2D and 3D fetal

Fetal Echocardiography

Figs 78.20A to F

Chapter 78: Echocardiography in Women

1907

Figs 78.20A to G: Normal fetus. Live/real time three-dimensional

echocardiographic study. (A) 31-week-old fetus. Arrowhead
points to the foramen ovale. Arrowhead in (B) denotes the ductus
arteriosus connecting aorta (AO) to pulmonary artery (PA); (C to F)
19-week-old fetus. Arrowhead points to foramen ovale visualized
in a four-chamber view (C) and from top (D). Color Doppler
image shows physiological right to left shunting (arrowhead, E).
Arrowhead in F points to a normal tricuspid valve. Both atrial
septum (AS) and ventricular septum (VS) are viewed en face in
this image; (G) 22-week-old fetus. Color Doppler image shows
ascending aorta (AA), aortic arch (ACH) and descending thoracic
aorta (DA). (IVC: Inferior vena cava; LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Maulik et al. Live
Three-dimensional echocardiography of the human fetus. Echocardiography. 2003; 20:71521.

Figs 78.21A to D

1908

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.21E to J

Chapter 78: Echocardiography in Women

1909

Figs 78.21A to N: Complete atrioventricular septal defect in 36-week-old fetus. Live/real time three-dimensional echocardiographic
study. (A to D) Four-chamber views cropped to show the common atrioventricular valve (V) and the defect (asterisks). Arrowhead in C
points to the atrial septum. (E to F) The pyramidal section has been cropped from the top and rotated toward the examiner to display all
five leaflets of V: posterior (P), left lateral (L1), left anterior (A1), right anterior (A2), and right lateral (L2). Small portions of the ventricular
septum (S) and atrial septum (AS) have been retained to show their relationship to V. V is open in E and closed in F. (G) Arrowhead
demonstrates multiple chordal attachments of V to S; (H to J) En face viewing of the defect (asterisk) from above (H), from the inferior
aspect (I) and from the right side (J); (K to M) Five-chamber view shows the aorta (AO) arising from LV. In M, the pyramidal section has
been cropped to show regurgitation (R) from the right-sided component of V. (N) Arrowhead shows the ductus arteriosus. (AV: Aortic
valve; S: Ventricular septum). (Movie clip 78.21).
Source: Reproduced with permission from Maulik et al. Live three-dimensional echocardiography of the human fetus. Echocardiography
2003;20:71521.

echocardiography allows diagnosis of many congenital

cardiac lesions. This information is crucial to the
obstetrician and the pediatric cardiologist who can discuss
the prognosis with the parents and also aids them in the
management of these conditions in the neonatal period. For
example, in complete atrioventricular septal defects, valve

attachments to papillary muscles in the opposite ventricle

are not amenable to surgical repair and hence these
babies carry a dismal prognosis. Also, cyanotic babies with
dextro transposition of the great vessels need intervention
immediately after birth. In some specialized centers in
the country, percutaneous intrauterine interventions

1910

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 78.22: Thickened tricuspid valve in a 20-week-old fetus.

Live/real time three-dimensional echocardiographic study. The
arrowhead points to a markedly thickened tricuspid valve. (LA:
Left atrium; RA: Right atrium).
Source: Reproduced with permission from Maulik et al. Live
three-dimensional echocardiography of the human fetus. Echocardiography. 2003; 20:71521.

have been successfully performed to correct aortic and

pulmonary valve stenosis.2730 This is done in an attempt
to prevent the development of hypoplastic left and right
heart syndromes in the newborn. 3D echocardiography
actually provides more information in assessing the
severity. Both valves are enclosed in the 3D data sets, and
they can be cropped to view the maximum flow limiting
orifice area. It is the direct en face visualization coupled
with the quantification of the stenotic semilunar valve
orifice which makes 3D echocardiography more reliable
than 2D.2730 Furthermore, there is added information
obtained from 3D echocardiography due to the ability to
view cardiac chambers, valve leaflets, and great vessels in
three dimensions. This added information can prove to
be useful in guiding catheter based interventions in the
intrauterine setting after birth.
Other pathologic conditions seen in women are
found in Figures 78.23 to 78.37 with corresponding movie
clips.

Figs 78.23A to D: Systemic hypertension. The patient is a 37-year-old female with very severe hypertension. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A), short-axis (B), and apical four-chamber (C) views. Note the marked concentric
hypertrophy of both the ventricular septum (VS) and the posterior wall (PW). The left atrial appendage (LAA) is clearly delineated and
is free of any clot. (D) M-mode study shows left ventricular hypertrophy. (AO: Aorta; LA: Left atrium; MV: Mitral valve; RA: Right atrium;
RV: Right ventricle). (Movie clips 78.23A to C).

Chapter 78: Echocardiography in Women

Fig. 78.24: Left ventricular hypertrophy in a 30-year-old patient

with chronic renal disease. Two-dimensional transthoracic echocardiography. Both the ventricular septum (VS) and the posterior
wall (PW) are hypertrophied and contain echo densities related
to fibrosis. (AO: Aorta; LA: Left atrium; RV: Right ventricle).
(Movie clip 78.24).

1911

Fig. 78.25: Mitral valve vegetations in a 43-year-old female with infective endocarditis. Live/real time three-dimensional transesophageal echocardiography. The black arrow (horizontal arrowhead
in the video clip) points to a mitral valve vegetation involving the
A1 segment and commissure. Note the presence of central perforation. Two other vegetations are also seen involving A2 and A3
segments of the anterior mitral leaflet. (AO: Aorta; PV: Pulmonary
valve; TV: Tricuspid valve). (Movie clip 78.25).
Source: Reproduced with permission from Hansalia et al. The
value of live/real time three-dimensional transesophageal echocardiography in the assessment of valvular vegetations. Echocardiography. 2009;26:126473.

Figs 78.26A and B: Mitral valve vegetation in a 32-year-old female with infective endocarditis. Live/real time three-dimensional
transesophageal echocardiography. (A) The arrowhead points to a large mitral valve vegetation with a central perforation; (B) The
lower arrowhead points to an annular abscess and the upper arrowhead points to the mitral valve. (AO: Aorta; PV: Pulmonary valve).
(Movie clips 78.26A and 78.28B).
Source: Reproduced with permission from Hansalia et al. The value of live/real time three-dimensional transesophageal echocardiography in the assessment of valvular vegetations. Echocardiography. 2009;26:126473.

1912

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.27A and B: Right-sided heart failure resulting from severe tricuspid regurgitation due to infective endocarditis. Two-dimensional
transthoracic echocardiography. (A) Arrowheads denote vegetations on the tricuspid valve leaflets; (B) Color Doppler examination
shows severe tricuspid regurgitation (TR). (AO: Aorta; CS: Coronary sinus; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
(Movie clips 78.27A part 1, 78.27A part 2 and 78.27B).

Figs 78.28A and B: Systemic lupus erythematosus in a young female patient. Two-dimensional transthoracic echocardiogram. (A and B).
Parasternal long-axis view shows the presence of pericardial effusion (PE, A) which resolved during follow-up (B). There are no valvular
abnormalities. (AO: Aorta; CS: Coronary sinus; DA: Descending aorta; LA: Left atrium; LV: Left ventricle; PW: Posterior wall; RV: Right
ventricle; SVC: Superior vena cava; VS: Ventricular septum). (Movie clips 78.28A and B).

Chapter 78: Echocardiography in Women

1913

Figs 78.29A and B: Metastatic left pleural effusion in a 53-year-old female with chronic myeloid leukemia. Two-dimensional transthoracic echocardiography. (A) In the parasternal long-axis view, the pleural effusion (PLE) is diagnosed by noting an echo free-space posteriorly extending beyond the descending thoracic aorta (DA); (B) PLE is readily diagnosed by placing the ultrasound transducer in the
posterior intercostal spaces with the patient sitting up. (LA: Left atrium; LB: Left back; LU: Lung; LV: Left ventricle; RV: Right ventricle).
(Movie clips 78.29A and B).

Figs 78.30A and B: Bicuspid aortic valve in a young female. (A and B) Both two-dimensional (A: Left panel, systole; right panel,
diastole) and three-dimensional (B) transthoracic echocardiography show the presence of a bicuspid aortic valve with horizontal cusp
orientation. (AO: Aorta; LA: Left atrium; PV: Pulmonary valve; RA: Right atrium, RV: Right ventricle). (Movie clips 78.30A and B).

1914

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.31A and B: Bicuspid aortic valve. Two-dimensional transesophageal echocardiogram showing a bicuspid aortic valve imaged
in long (A) and short (B) axis views. The arrow in A points to valve redundancy and prolapse into the left ventricular (LV) outflow tract.
In B, the cusps are vertically oriented and appear to be equal in size. (AO: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle;
TV: Tricuspid valve). (Movie clips 78.31A and B).

Figs 78.32A to D

Chapter 78: Echocardiography in Women

1915

Figs 78.32A to G: Quadricuspid aortic valve in a 54-year-old

female. (A and B) Two-dimensional transthoracic echocardiography. The aortic valve (AV) appears bicuspid. (C to E) Multiplane twodimensional transesophageal echocardiography. (C and D) AV leaflets (numbered in D) are well seen. The arrow in C points to diastolic noncoaptation of AV leaflets which resulted in significant aortic
regurgitation. (E) The arrow points to severe aortic regurgitation.
(F and G) Live/real time three-dimensional transthoracic echocardiography show a quadricuspid AV with four numbered leaflets
clearly visualized. (AO: Aorta; LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve). (Movie
clips 78.32A to D, 78.32F part 1 and part 2).
Source: Reproduced with permission from Burri et al. Live/real time
three-dimensional transthoracic echocardiographic identification of
quadricuspid aortic valve. Echocardiography. 2007;24:6535.

Fig. 78.33: Muscular outflow ventricular septal defects. Twodimensional transthoracic echocardiography. Short-axis view
at the level of the LV outflow tract demonstrates two adjacent
ventricular septal defects (1 and 2). Note the two defects are
not related to the tricuspid or pulmonary valve and hence are
not in the perimembranous or supracristal location. (AO: Aorta;
PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle; TV:
Tricuspid valve). (Movie clip 78.33).

1916

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.34A to D: Levo (corrected) transposition of the great vessels with a ventricular septal defect and pulmonary artery banding in
a 25-year-old female. Parasternal long-axis views (A and B) show a large ventricular septal defect with flow signals moving from the
pulmonary ventricle (morphological LV) into the aorta (AO). The arrow points to a band in the pulmonary artery (PA) surgically placed
in an attempt to protect the patient from pulmonary hypertension. Two-dimensional (C) and three-dimensional (D) transthoracic shortaxis views show the aortic valve and the AO located directly anterior to pulmonary valve (PV) and PA indicative of transposition of the
great vessels. (CS: Coronary sinus; LV: Left ventricle; MV: Mitral valve; RA: Right atrium). (Movie clips 78.34A to D).

A
Figs 78.35A and B

Chapter 78: Echocardiography in Women

1917

Figs 78.35A to C: Embolization of a left atrial appendage clot

into the systemic circulation in a 67-year-old female undergoing
coronary artery bypass surgery. Two-dimensional transesophageal
echocardiography. This was initially performed to rule out the presence of mitral regurgitation because an apical systolic murmur was
heard pre-operatively. No significant mitral regurgitation was noted
but a clot was demonstrated in the left atrial appendage (LAA) (A).
The patient had two brief episodes of atrial fibrillation in the past
which probably accounted for the formation of the clot. Before the
surgeon could make any decision, the clot was noted to embolize
from the appendage into the systemic circulation (B and C). In the
immediate post bypass period, the left femoral pulsation was found
to be absent pointing to the location of the clot which was successfully removed. The patient had an uneventful recovery without any
stroke or other complication. (AO: Aorta; LA: Left atrium; LV: Left
ventricle). (Movie clip 78.35).

Figs 78.36A to D

1918

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 78.36A to F: Combined valvar and supravalvar aortic stenosis. Live/real time three-dimensional transthoracic echocardiography.
(A) Horizontal arrowhead points to supravalvar aortic stenosis produced by calcification at the sinotubular junction. The vertical arrowhead shows heavy mitral annular calcification; (B) Supravalvar stenotic orifice viewed in short axis (arrowhead); (C) Short-axis view at
the level of the aortic valve (AV) leaflets demonstrating mild valvar stenosis. Live/real time three-dimensional transthoracic echocardiography in a patient with calcification at the aortic sinotubular junction but no stenosis; (D) The arrowheads point to prominent calcifications
at the sinotubular junction viewed in long axis; (E) Short-axis view at the sinotubular junction shows a large orifice (arrowhead) that
measured 2.5 cm2; (F) Short-axis view at the level of the AV (left) and immediately above it (right). The AV orifice measured 1.7 cm2 by
planimetry, consistent with mild aortic stenosis. The arrowhead in the right panel points to sinotubular calcification protruding into the
aortic lumen imaged just beyond the AV leaflets. (AO: Ascending aorta; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA:
Right atrium; RV: Right ventricle; RVO: Right ventricular outflow tract; TV: Tricuspid valve). (Movie clip 78.36).
Source: Reproduced with permission from Rajdev et al. Live/real time three-dimensional transthoracic echocardiographic assessment
of combined valvar and supravalvar aortic stenosis. Am J Geriatr Cardiol. 2006;15:18890.

A
Figs 78.37A and B

Chapter 78: Echocardiography in Women

1919

Figs 78.37A to D: Paravalvular mitral regurgitation in a 69-year-old female. Paravalvular mitral regurgitation (arrowhead) is noted by
transthoracic (A) and transesophageal (B) echocardiography. (C and D) Represent live/real time three-dimensional images showing two
plugs (arrowheads) used to successfully close the leak percutaneously in the cardiac catheterization laboratory. Small arrows in D point
to intact sutures. (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; MR: Mitral regurgitation; MVR: Mitral valve replacement; RA: Right
atrium; RV: Right ventricle). (Movie clips 78.37A to D).

CONCLUSION
Echocardiography proves to be a very useful diagnostic
tool for women, but it is not without its challenges. There
are structural heart diseases that occur more commonly in
women, and it is important to remember that women can
often have atypical presentations, which sometimes delays
the accurate diagnosis. Often, the diagnosis is delayed in
women because it is thought that the likelihood of a certain
disease process is rather low. Hopefully, this discussion
has helped to illustrate and highlight the structural heart
disease processes that are actually seen more frequently in
women.

REFERENCES
1. Dalen H, Thorstensen A, Vatten LJ, et al. Reference values and distribution of conventional echocardiographic
Doppler measures and longitudinal tissue Doppler velocities in a population free from cardiovascular disease. Circ
Cardiovasc Imaging. 2010;3;61422.
2. Lang RM, Bierig M, Devereux RB, et al. Recommendations
for Chamber Quantification: A Report from the American
Society of Echocardiographys Guidelines and Standards
Committee and the Chamber Quantification Writing
Group, Developed in Conjunction with the European
Association of Echocardiography, a Branch of the
European Society of Cardiology. J Am Soc Echocardiogr.
2005;18:144063.

3. Lauer MS, Larson MG, Levy DL. Sex-specific reference

M-mode values in adults: population-derived values with
consideration of the impact of height. J Am Coll Cardiol.
1995;26:103946.
4. Sadaniantz A, Hadi BJ, Saint Laurent L. Gender Differences
in Mitral Inflow Parameters of Doppler Echocardiography.
Echocardiography. 1997;14(5):43540.
5. Vasan RS, Larson MG, Benjamin EJ, et al. Echocardiographic
reference values for aortic root size: the Framingham Heart
Study. J Am Soc Echocardiogr. 1995;8(6):793800.
6. Movahed MR. Impairment of echocardiographic acoustic
window caused by breast implants. Eur J Echocardiogr.
2008;9(2):2967.
7. Avierinos JF, Inamo J, Grigioni F, et al. Sex differences in
morphology and outcomes of mitral valve prolapse. Ann
Intern Med. 2008;149(11):78795.
8. St John Sutton M, Weyman AE. Mitral valve prolapse prevalence and complications: an ongoing dialogue. Circulation.
2002;106(11):13057.
9. Gaffney FA, Karlsson ES, Campbell W, et al. Autonomic
dysfunction in women with mitral valve prolapse syndrome.
Circulation. 1979;59(5):894901.
10. Movahed MR, Ahmadi-Kashani M, Kasravi B, Saito Y.
Increased prevalence of mitral stenosis in women. J Am
Soc Echocardiogr. 2006;19(7):9113.
11. Burri MV, Nanda NC, Lloyd SG, et al. Assessment of systolic
and diastolic left ventricular and left atrial function using
vector velocity imaging in Takotsubo cardiomyopathy.
Echocardiography. 2008;25(10):113844.
12. Atar S, Jeon DS, Luo H, et al. Mitral annular calcification: a
marker of severe coronary artery disease in patients under
65 years old. Heart. 2003;89(2):1614.

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Section 7: Miscellaneous and Other Noninvasive Techniques

13. Shaw LJ, Bairey Merz CN, Pepine CJ, et al. WISE Investigators.
Insights from the NHLBI-Sponsored Womens Ischemia
Syndrome Evaluation (WISE) Study: Part I: gender
differences in traditional and novel risk factors, symptom
evaluation, and gender-optimized diagnostic strategies.
J Am Coll Cardiol. 2006;47(3 Suppl):S4S20.
14. Talbott E, Guzick D, Clerici A, et al. Coronary heart
disease risk factors in women with polycystic ovary
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8216.
15. Citro R, Caso I, Provenza G, et al. Right ventricular
involvement and pulmonary hypertension in an elderly
woman with tako-tsubo cardiomyopathy. Chest. 2010;137
(4):9735.
16. Donohue D, Movahed MR. Clinical characteristics,
demographics and prognosis of transient left ventricular
apical ballooning syndrome. Heart Fail Rev. 2005;10(4):
3116.
17. Donohue D, Ahsan C, Sanaei-Ardekani M, et al. Early
diagnosis of stress-induced apical ballooning syndrome
based on classic echocardiographic findings and correlation
with cardiac catheterization, J Am Soc Echocardiogr. 2005;
18:1423.
18. Hurst RT, Prasad A, Askew JW 3rd, et al. Takotsubo
cardiomyopathy: a unique cardiomyopathy with variable
ventricular morphology. JACC Cardiovasc Imaging. 2010;
3(6):6419.
19. Haghi D, Athanasiadis A, Papavassiliu T, et al. Right
ventricular involvement in Takotsubo cardiomyopathy. Eur
Heart J. 2006;27(20):24339.
20. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Gender
and outcome in adult congenital heart disease. Circulation.
2008;118(1):2632.

21. Warnes CA. Sex differences in congenital heart disease:

should a woman be more like a man? Circulation. 2008;
118(1):35.
22. Webb G, Gatzoulis MA. Atrial septal defects in the adult:
recent progress and overview. Circulation. 2006;114(15):
164553.
23. Rothman KJ, Fyler DC. Sex, birth order, and maternal age
characteristics of infants with congenital heart defects. Am
J Epidemiol. 1976;104(5):52734.
24. Samnek M. Boy:girl ratio in children born with different
forms of cardiac malformation: a population-based study.
Pediatr Cardiol. 1994;15(2):537.
25. McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114(13):141731.
26. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary
hypertension. A national prospective study. Ann Intern
Med. 1987;107(2):21623.
27. Maulik D, Nanda NC, Hsiung MC, et al. Doppler color flow
mapping of the fetal heart. Angiology. 1986;37(9):62832.
28. Nanda, NC, Sudhakar S, Joshi D, et al. Role of three
dimensional echocardiography in prevention and early
detection of cardiac diseases. In: Chopra, HK, editor. Heart
Protection Book. Delhi, India: ASSOCHAM and Ministry
of Health and Family Welfare, Govt. of India, 2011 and
ASSOCHAM released on September 2011: 437.
29. Singh A, Mehmood F, Romp RL, et al. Live/Real time threedimensional transthoracic echocardiographic assessment
of aortopulmonary window. Echocardiography. 2008;25(1):
969.
30. Singh A, Romp RL, Nanda NC, et al. Usefulness of live/real
time three-dimensional transthoracic echocardiography
in the assessment of atrioventricular septal defects. Echocardiography. 2006;23(7):598608.

1921

CHAPTER 79
Echocardiography in the Elderly
Gopal Ghimire, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia

Snapshot

Aorc Atherosclerosis and Penetrang Aorc Ulcer

Aorc Valve Sclerosis

Aorc Stenosis

Aorc Aneurysm

Aorc Dissecon

INTRODUCTION
Aging induces significant alteration in the structure and
function of the cardiovascular (CV) system. Although these
adaptive changes are physiological and asymptomatic,
they are at times difficult to distinguish from preclinical
disease. Furthermore, presence of coexisting CV diseases
can modulate these adaptive responses and complicate
the clinical picture. In this chapter, we aim to discuss the
CV peculiarities in elderly patients.

AORTIC ATHEROSCLEROSIS AND

PENETRATING AORTIC ULCER
(FIGS 79.1A TO C)
Atherosclerosis is a systemic disease with a strong
predilection to involve the aorta especially in the elderly.
A study revealed that the average age of those with
large/complex plaques (measuring 4 mm in size) in
the aorta on transesophageal echocardiography (TEE)
was 70 years.1 In the Stroke Prevention: Assessment of
Risk in a Community (SPARC) study,2 of 588 patients
(average age, 66.9 years) undergoing TEE, the overall
prevalence of aortic plaque in any location was 43.7%,
of which complex plaque (defined as 4 mm or mobile)

Le Ventricular Mass, Dimensions and Funcon

Echocardiography in Stroke Paents: Assessment of

Coronary Stenosis

Mitral Annular Calcificaon

Prosthec Valves

was 7.6%. The prevalence of aortic plaques increased

progressively from 8.4% in the ascending aorta to 31% in
aortic arch and 44.9% in the descending aorta. Similarly,
the prevalence of the complex plaques in the respective
locations was 0.2%, 2.2%, and 6.0%. The atherosclerotic
plaque on the aortic arch is now considered as the third
leading cause of embolic stroke.1 The prevalence of 4 mm
aortic plaque in stroke patients (1421%) is on the same
order of magnitude as that of the other two important
causes of embolic strokecarotid artery disease
(1013%) and atrial fibrillation (1830%).3,4 Although the
aortic plaque can be construed as a surrogate marker for
generalized atherosclerosis and stroke risk, the French
Aortic Plaque in Stroke (FAPS) study established causality
between the stroke and complex aortic arch atheroma as
the odds ratio (OR) for stroke in patients with plaques in
the descending aorta (unlikely to embolize upstream to
the cerebral vessels) was only 1.5 for the plaques 4 mm
when compared with an OR of 13.8 for those in the aortic
arch.4 Furthermore, this study also established a gradient
between increasing plaque thickness and incremental
stroke risk with OR significantly greater in those with a
plaque thickness 4 mm: the OR for stroke in patients
with submillimeter plaques was 1.0 (no increased risk), in
contrast to 3.9 for 13.9 mm plaques, and 13.8 for plaques

1922

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 79.1A to C: Aortic atherosclerosis in three elderly patients.

(A and B) Two-dimensional (2D) transesophageal echocardiography (A) Upper arrow points to a mobile atherosclerotic plaque
and the lower to a fixed one in the descending thoracic aorta (AO).
Arrowhead shows prominent ulceration in the plaque; (B) Arrow,
in another patient, shows a mobile plaque in the ascending aorta
(AA); (C) 2D transthoracic echocardiography. Arrow, in a different patient, shows a fixed plaque in the proximal abdominal aorta
(AB) imaged using the subcostal approach (Movie clips 79.1A to C).
(L: Liver).

4 mm. Although transthoracic echocardiography (TTE)

can visualize the aortic root and proximal ascending aorta,
the aortic arch, and the descending thoracic aorta from
various windows, the resolution of the TTE is not optimal
enough for accurate evaluation of the atherosclerotic
plaque. In contrast, the TEE probe is closer to the aorta
and can be used at a higher frequency, thus allowing for
higher resolution than on TTE. An accurate and detailed
evaluation of the aorta, including the origin of the great
vessels, is possible,5 and there is excellent interobserver
and intraobserver variability.6
Very uncommonly, the atherosclerotic lesion penetrates the internal elastic lamina into the media, leading
to formation of a penetrating aortic ulcer (PAU). The
natural history of this entity may involve progression to a
variable degree of intramural hematoma (IMH) formation,
pseudoaneurysm formation, aortic rupture, or late aneurysm.7 Although PAUs can occur throughout the aorta, it

has predilection for the thoracic and abdominal aorta

more than in the arch or ascending aorta. PAU typically
mimics presentation of the classic aortic dissection with
acute chest or back pain, and indeed PAUs constitute
up to 28% of acute aortic syndrome.7 However, 25% of
patients with PAU are asymptomatic and the lesions were
identified during axial imaging for other indications.8 The
patients are typically older and hypertensive individuals
with multiple coronary risk factors and coexisting vascular
disease. Imaging techniques for PAU include aortography,
computed tomography (CT), magnetic resonance imaging
(MRI), and TEE with TEE showing aortic atherosclerotic
plaque with focal ulceration of the intima. The management
of the patient with PAU must be individualized depending
on the location and disease progression. The general
consensus is that a large or unstable PAU or a PAU with
progression should have surgical intervention. The predictors of disease progression are refractory or recurrent

Chapter 79: Echocardiography in the Elderly

Fig. 79.2: Aortic valve sclerosis. Three-dimensional transesophageal echocardiography. Short axis cropping at the tip of the aortic
valve (AV) obtained from a parasternal long-axis data set shows
a large aortic orifice (arrowhead in the Movie clip 79.2) with no
evidence of stenosis. The AV leaflets are mildly thickened consistent with sclerosis. (LA: Left atrium; TV: Tricuspid valve). (Movie
clip 79.2).
Source: Reproduced with permission from Nanda NC, Hsiung MC,
Miller AP, Hage FG. Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.

1923

Fig. 79.3: Aortic valve sclerosis. Three-dimensional transesophageal echocardiographic reconstruction. Shows a thickened tricuspid aortic valve (AV) with no significant pressure gradient. (LA: Left
atrium; RA: Right atrium; RV: Right ventricle) (Movie clip 79.3).

pain, ulcer craters > 20 mm in diameter or 10 mm in

depth, interval worsening of aortic hematoma, periaortic hematoma, expanding pseudoaneurysm, increasing
pleural effusion, and rupture. Due to segmental nature
of this entity, endovascular intervention is an attractive
alternative, especially since these patients tend to be
elderly and have multiple comorbidities.913

AORTIC VALVE SCLEROSIS

(FIGS 79.2 TO 79.4)
Murmur
A systolic murmur in the aortic area is present in
approximately 50% of elderly population.14,15 The reliability
of clinical examination alone in the assessment of aortic
sclerosis or stenosis (AS) in the elderly is poor.16 In one
study, 17% of patients who were deemed to have AS only
on physical exam were found to have mitral regurgitation
(MR) as the only significant finding.17 Most of the murmurs
in the elderly subjects are related to sclerotic aortic valve
(AV) leaflets, flow into tortuous, noncompliant great
vessels, or a combination of these. Such murmurs must
be distinguished from murmurs caused by mild to severe
valvular AS, or atrioventricular valve regurgitation, which
are prevalent in this age group. The ACC/AHA recommends

Fig. 79.4: Aortic valve sclerosis. Three-dimensional transesophageal echocardiographic reconstruction demonstrates a mildly
thickened, obliquely oriented bicuspid aortic valve (AV) with raphe
and no significant gradient consistent with AV sclerosis. (LA: Left
atrium; RA: Right atrium; RV: Right ventricle). (Movie clip 79.4).

that echocardiography should not be done for patients who

have a Grade II or softer midsystolic murmur identified as
innocent or functional by an experienced observer.18

Prevalence, Pathophysiology, and

Echocardiographic Findings
AV sclerosis is focal thickening of the AV leaflets with commissural sparing, normal leaflet mobility, and no evidence
of left ventricular outflow tract (LVOT) obstruction with

1924

Section 7: Miscellaneous and Other Noninvasive Techniques

transaortic gradient of <2.5 m/s. The degenerative process

may not be confined to the leaflets alone; it can also extend to the aortic root. Furthermore, 50% of patients with
AV sclerosis also have associated mitral valve sclerosis.19
Approximately, one in four subjects more than 65 years
may have AV sclerosis and the prevalence increases with
advancing age.20 However, AV sclerosis is not an inevitable consequence of aging as 2545% of octogenarians
have no evidence of AV calcification.21,22 The relationship
between AV sclerosis and age is nonlinear with a sharp
increase in prevalence at age about 65 years in men and at
age about 75 years in women.
Despite normal hemodynamics, AV sclerosis is not
benign; it is independently associated with poor CV
outcome, for example, with a relative risk of death of
1.66 [95% confidence interval (CI) 1.232.23]23 or of a
new coronary event of 1.76 (95% CI 1.522.03).24 In LIFE
study (Losartan Intervention For End point reduction in
hypertension), composite CV outcome occurred in 15%
of hypertensive patients with AV sclerosis, compared with
8% patients with normal AV.25 Patients admitted with chest
pain and AV sclerosis had a higher incidence of CV events
(16.8% vs 7.1%) and worse event-free survival than did
those without AV sclerosis.26 In the CV health study, the
rate of death from any cause or death from CV cause of
patients with AV sclerosis was twice that of patients with
a normal AV.23 Two-dimensional (2D) echocardiography
is the best noninvasive modality to diagnose AV sclerosis.
The mobility of AV leaflets and the severity and extent
of calcification on the cusps and in the aortic annulus
along with peak and mean pressure gradients across the
AV should be measured. The absence of high gradients
distinguishes it from significant AS. Three-dimensional
(3D) echocardiography can supplement the 2D technique
by providing a more comprehensive evaluation of AV
anatomy, leaflet motion, and exact sites and degree of
calcification.
AV sclerosis may progress to AS. Of 2,131 patients with
AV sclerosis, 15.9% developed AS over a mean follow-up
of 7.4 years with 5.4% developing clinically significant
(moderate to severe) AS.27 In another study of 400 patients
with AV sclerosis followed for a mean duration of 44 30
months, 32.75% developed some degree of AS with 2.5%
developing severe AS defined as a peak jet velocity 4 m/s,
5.25% of patients progressed to moderate AS, which was
defined as a peak jet velocity between 3.1 and 3.9 m/s,
and 25% developed mild AS, which was defined as a peak
jet velocity between >2 and 3 m/s.28

AORTIC STENOSIS
Prevalence and Pathophysiology
AS in adults is caused by a calcific process involving a
normal trileaflet or congenital bicuspid aortic valve (BAV).
This calcific process can progress from the base of the
cusps to the leaflets, eventually causing a reduction in
leaflet motion and effective valve area without leading to
commissural fusion.29 The AV is more likely to be tricuspid
in patients in their eighth, ninth, and tenth decades of life,
and bicuspid in younger patients.3032 However, a study
revealed that three out of nine nonagenarians undergoing
surgical AV replacement had bicuspid valves.33 In the
Cardiovascular Health Study, AS was present in 2% of
the entire study cohort (adults 65 years), 2.6% of those
75 years or older, and 4% of those 85 years or older.
Changing demographic pattern in the western society and
changing epidemiology of rheumatic heart disease have
rendered calcific AS as one of the most prevalent valvular
diseases. AS currently is considered the most frequent
indication for valve replacement surgery, and the second
most common indication for cardiac surgery in older
adults, which is surpassed only by coronary artery bypass
grafting (CABG).34

Two-Dimensional Echocardiography
(Figs 79.5 to 79.7; Also see Fig. 78.11
in Chapter 78)
Echocardiography is an integral part of the evaluation of
AS in order to confirm the diagnosis, and assess its severity
and evaluate its upstream consequences on the left
ventricle (LV). Clinical signs and symptoms are of limited
use in distinguishing critical from noncritical AS due to
unsatisfactory sensitivity and specificity in the aged.35
Although cardiac catheterization is regarded as the gold
standard for evaluation of valvular dysfunction, it is invasive
and is associated with a higher risk of complications in the
elderly. In a single center study, 22 of 101 patients with
valvular AS who underwent retrograde catheterization
of the AV had focal diffusion-imaging abnormalities in
cranial MRI, a pattern consistent with acute cerebral
embolic events after the procedure; three of these patients
(3%) had clinically apparent neurological deficits.36 Thus,
cardiac catheterization in the elderly portends substantial
risk of clinically apparent cerebral embolism and frequent
silent ischemic brain lesions. Cardiac catheterization is

Chapter 79: Echocardiography in the Elderly

1925

Figs 79.5A to D: A 79-year-old man with exertional angina and syncope. Two-dimensional transthoracic echocardiography. On the
parasternal long-axis view (A) a calcified aortic valve with restricted opening is shown at the arrow. The maximal velocity across the
valve on continuous wave (CW) Doppler is 4.1 m/s (B) with a velocity time integral (VTI) of 84 cm, which correspond to a peak gradient
of 67 mm Hg and mean gradient of 35 mm Hg. The PW across the left ventricular outflow tract (LVOT) measured a velocity of 88.4 cm/s
across the LVOT (C); (D) The aortic valve area and the dimensionless index are calculated, both corresponding to severe aortic stenosis.
Notice that small errors in the measurement of the LVOT diameter will result in large errors in the calculated aortic valve area, while
the dimensionless index is unaffected by this measurement. Adapted from Adegunsoye A, et al. Echocardiography. 2011;28:11729.

currently recommended for assessment of severity of

AS only in symptomatic patients when noninvasive tests
are inconclusive or when there is a discrepancy between
noninvasive tests and clinical findings regarding severity
of AS.18
During echocardiography, it is of utmost importance
to check for the consistency of different echocardiographic
findings and with clinical assessment. In patients with
severe AS, two-dimensional transthoracic echocardiography (2D TTE) shows augmented reflectance of the
valvular cusps along with significant thickening and
deformity. The valve leaflets are domed, less mobile, and
with restricted leaflet excursion. In intermediate cases, the

free edge remains mobile despite immobility of the base,

and fusion of the commissures does not occur primarily. If
a discrete orifice can be demonstrated, then the valve area
can be measured with planimetry, but direct planimetric
measurements of the aortic orifice are of insufficient
sensitivity and specificity as a result of the uncertainty that
the plane of imaging is at the leaflet tips (where maximum
stenosis commonly occurs) and is exactly parallel to the
orifice.
The determinants of pressure gradient across a stenotic
valve are the valve orifice area and the transvalvular flow.37
Thus, in the presence of depressed cardiac output, relatively
low pressure gradients may be obtained in patients with

1926

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 79.6: Schematic. Because a stenotic jet consists of a small

central region or core of high velocity and a larger outer region
of lower velocity, the continuous wave Doppler cursor must be
positioned in the jet core in addition to being aligned parallel to
the jet direction to measure the maximum jet velocity. The threedimensional structure of the jet may cause the continuous wave
cursor to appear to be correctly positioned in the jet while, in reality,
the cursor may not be properly placed in the core. Therefore,
after the initial alignment of the continuous wave cursor in the
visualized jet, minimal transducer angulations are still required
to interrogate the jet core, which may be in the azimuthal plane.
Failure to interrogate the core results in an underestimation of the
peak transvalvular velocity and thus the severity of the stenotic
lesion. In this illustration, the aortic stenosis (AS) jet is shown to
consist of a central core, which has the highest velocity, and an
outer region of lower velocity flow surrounds this central core. The
highest velocity is thus obtained if the continuous wave Doppler
cursor is aligned parallel to the core of the jet (cursor 2), while
lower velocities are recorded if the cursor is positioned outside the
core (cursors 1, 3, 4, and 5).
Source: Reproduced with permission from Nanda NC. Textbook of
Color Doppler Echocardiography. Philadelphia, PA: Lea and Febiger, Inc; 1989:17890.40

severe AS. Conversely, during exercise or other high-flow

states, significant pressure gradients can be measured
in minimally stenotic valves. Therefore, comprehensive
assessment of AS requires measurement of transvalvular
flow, mean transvalvular pressure gradient using Doppler
interrogation, and calculation of the effective valve orifice
area.
Doppler echocardiography utilizes ultrasound reflecting off moving red blood cells to measure the velocity
of blood flow and allows the noninvasive assessment of
normal and abnormal blood flow patterns. Continuous
wave Doppler echocardiography measures the highest
velocities along the plane of interrogation, but for
reliable velocity measurements, the cursor must be
aligned parallel to the flow jet. The accuracy of this task is

facilitated by using color Doppler as a guide that enables

easy visual identification of the aortic jet.38 The peak and
mean velocities across the AV can be translated into
peak and mean pressure gradients by using the modified
Bernoullis equation.39 An important caveat is that even
with color Doppler guidance, optimization in the cursor
position is paramount to obtain the maximum velocity.
This is because the AS jet has an outer region of relatively
low velocity flow and a central core of higher velocity that
can be appreciated only when viewed in three dimensions
(Fig. 79.6).40 Malalignment of the beam with the flow
across the valve will lead to underestimation of the velocity
and, therefore, of the severity of AS. In order to minimize
this source of error, the velocities have to be measured
using several echocardiographic windows, the apical and
right parasternal views, and the highest value assumed to
correspond to the proper alignment.41 Since transvalvular
velocity is squared in the equation, a small change in
transvalvular velocity can result in a significant change
in the transvalvular gradient. Estimation of the aortic
valve area (AVA) can also be performed with the use of
the continuity equation, which depends on the principle
of the law of continuity of flow.42 This has been shown to
be a reliable index of AS severity that correlates highly
with cardiac catheterization measurements and surgical
findings.43 This calculation entails measuring the LVOT
diameter, the time velocity integral at the LVOT using
pulsed wave Doppler, and the time velocity integral at the
AV (using continuous wave Doppler, Fig. 79.2). LVOT crosssectional area is computed from the diameter assuming
that the outflow tract has a circular configuration. The
outflow tract diameter is measured immediately proximal
to the attachment of the aortic leaflets during systole from
the standard parasternal long-axis plane, and the largest
inner diameter is selected.44 The peak LVOT velocity is
estimated with the pulsed Doppler technique in the apical
transducer position, with the sample volume placed
1.01.5 cm proximal to the AV to avoid the area of AS jet
flow acceleration. The continuity equation is independent
of transaortic flow and the presence of aortic or mitral
insufficiency, and the equation has been validated in the
presence of severe aortic incompetence.45 The continuity
equation assumes that the LVOT diameter and the flow
across the LVOT are measured at the same level, which
is technically difficult since they are assessed in different
echocardiographic views. Also, since continuous wave
Doppler is used to measure the highest velocity across a
straight path, it also assumes that there is no other form
of LVOT obstruction other than AS such as supravalvular
or subaortic stenosis. Nevertheless, the most frequent

Chapter 79: Echocardiography in the Elderly

1927

Figs 79.7A to F: Mitral and aortic annular calcification in a 99-year-old patient. Two-dimensional transthoracic echocardiography.
(A and B) Parasternal long-axis view (A) and parasternal short-axis view (B) at the level of the aortic valve show heavy calcification
involving the aortic valve, and aortic and mitral annuli (1), mitral valve (2), and mitral subvalvular apparatus (3, 4); (C) Parasternal
short-axis view at the level of the mitral valve shows calcification involving the posterior mitral leaflet and mitral annulus (2); (D)
Apical four-chamber view showing calcification involving the ventricular septal muscle (arrow). Arrowhead points to a pacemaker in
the right atrium (RA); (E) Apical two-chamber view showing calcification involving the mitral valve and annulus (2). R represents a
reverberatory artifact deep in the left atrium from the calcified mitral valve. (F) Color Doppler-guided continuous wave Doppler interrogation of the aortic valve demonstrates peak and mean gradients of 30 and 18 mm Hg, respectively (arrow), consistent with mild
aortic stenosis. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle) (Movie clips 79.7A to E).

1928

Section 7: Miscellaneous and Other Noninvasive Techniques

Table 79.1: Limitations of Two-Dimensional Transthoracic/Color Doppler Echocardiography

The continuity equation provides an indirect rather than direct estimation of the AVA
True LVOT velocity can be difficult to determine from the area of increased flow acceleration
Accurately measuring LVOT diameter may be difficult (e.g. in patients with aortic or mitral annular calcification)
Doppler cursor may not be in the jet core even with color Doppler guidance, resulting in AS severity underestimation
Localized high gradients may be present in the region of the aortic valve; these do not reflect the true gradient across the AV,
resulting in overestimation of AS severity
AS severity cannot be correctly assessed in the presence of coexisting subaortic or supravalvular stenosis
Source: Adapted from Vengala et al.52
(AS: Aortic stenosis; AV: Aortic valve; AVA: Aortic valve area; LVOT: Left ventricular outflow tract).

source of error in measuring the AV area is due to the

inaccuracy of LVOT diameter measurement. This is
especially difficult in older adults with accumulation of
calcium at the annulus. Furthermore, since the LVOT is
often elliptical rather than circular in nature if measured
proximal to the LVaortic junction, 3D modalities of
imaging may be more accurate in measuring the LVOT
than 2D TTE.46 The age-related alteration in the LVOT
geometry with reduction in the septoaortic angle with
advancing age might render the outflow tract narrow in
the parasternal long-axis view.47 Since the LVOT diameter
is squared in the calculation of AVA, even minor errors in
measurement will amplify the inaccuracy of estimation
of AVA. This is particularly pertinent during evaluation
of the progression of severity of stenosis in an individual
patient, since large factitious variations in the calculated
valve area can be due to erroneous measurements of the
LVOT diameter. This limitation can be obviated by usage
of the dimensionless index, which is simply the ratio of
the velocity across the LVOT to that across the AV and
completely eliminates the area of the LVOT from the
equation.42 A ratio of 0.91.0 is considered as normal and
a ratio of <0.25 is regarded as severe stenosis. Another
potential source of error that is particularly relevant in
older population is the overestimation of the severity of AS
due to the phenomenon of pressure recovery, which leads
to the overestimation of AS severity when measured by
Doppler due to the conversion of kinetic energy upstream
to potential energy downstream across the stenotic valve.
This effect is further exaggerated in individuals with
domed and tubular stenoses, a narrow ascending aorta
(<3 cm), or a narrow LVOT (2 cm).4851 Other situations
in which Doppler echocardiography can potentially
overestimate the severity of AS includes confounding
of the true AS jet with localized high velocity gradients

presumably related to calcific areas in the AV, mistaken

identification of a jet (the jet of MR can be mistaken for
AS or the smaller velocity aortic jet may be contaminated
with the much higher velocity signals from the associated
MR jet), concomitant stenotic lesions present in tandem
(such as supravalvular stenosis or discrete subaortic
stenosis), and nonrepresentative jet selection (e.g. a post
extrasystolic beat). Table 79.1 depicts the limitations of
the transthoracic/color Doppler echocardiography for
evaluation of AS.
Multiplane transesophageal echocardiography (2D TEE)
permits detailed evaluation of the morphology of the
stenosed AV that may provide useful information about
the etiology of AS (degenerative, rheumatic, or bicuspid)
independent of the transthoracic acoustic window. By
gradually advancing the transesophageal probe to detect
the flow-limiting tip of the valve that is stenotic allows
direct planimetric quantification of the anatomical AVA.
The accuracy of this semi-invasive method may be limited
by difficulties in obtaining the optimal imaging plane
orientation at the level of the tip of the stenosed AV cusps.
Prior studies found clinically important overestimation
of effective AVA by TEE planimetry compared with flowderived methods with a variable reproducibility and
accuracy.5356 In geriatric population, precise planimetric
quantification of AVA by 2D TEE poses unique challenges
due to the altered aortoseptal angle with an obliquely
placed aorta, prior valve replacement, or severely calcified
valvular cusps, which cause acoustic shadowing and
interfere with visualization. In clinical echocardiographic
practice, AS is considered severe when the AVA is < 1.0 cm2,
indexed AVA57 is < 0.6 cm2/m2, and the mean transvalvular
gradient is > 40 mm Hg.18,57 Severe AS is unlikely if stroke
volume (that translates into transvalvular flow) is normal
and there is a mean pressure gradient of < 40 mm Hg.

Chapter 79: Echocardiography in the Elderly

Low Flow, Low Gradient Aortic Stenosis

with Low Ejection Fraction (Fig. 79.8)
In the presence of low transvalvular flow, lower pressure
gradients may be encountered even in presence of severe
AS; since the gradients are a squared function of flow,
even a modest decrease in flow may lead to a significant
reduction in gradient. A low-flow, low-gradient (LF-LG)
state is encountered in 5 to 10% of patients with severe
AS.58 Typically, these patients have severely depressed
LV systolic function5962 and portend a dismal prognosis
irrespective of modality of treatment with survival rates of
50% at 3-year follow-up if treated medically, and 6 to 33%
if treated surgically.58,6163
LF-LG severe AS is defined as EOA 1.0 cm2 or 0.6 cm2/m2
when indexed for BSA, mean transvalvular gradient
< 40 mm Hg, and left ventricular ejection fraction (LVEF)
< 40% in the presence of a low-flow state that is defined
as a cardiac index < 3.0 L/min/m2 and a stroke volume
index < 35 mL/m2.57,64,65 Stroke volume can be measured by
Doppler echocardiography,6668 biplane Simpson method
(2D TTE), or cardiac catheterization.69 However, the mere
presence mean gradient of <40 mm Hg and small AVA and
low LVEF does not definitely confirm LG LF severe AS,
since mild-to-moderately diseased valves may not open
fully due to low-flow state, resulting in a functionally small
valve area (pseudosevere AS). In these cases, dobutamine

1929

stress echo (DSE) up to a maximum dosage of 20 g/kg/min

is useful to discriminate severe from pseudosevere AS and
evaluate the LV contractile reserve.59,61 This test should
be performed with great caution in the elderly because
of the increased potential for ventricular arrhythmia
especially in the presence of coexisting coronary artery
disease (CAD). Typically, pseudosevere AS in the elderly
shows a significant increase in AV opening and AVA with
relatively little increase in gradient, whereas true severe
AS is characterized by no significant increase in AVA
even though there is significant change in LV ejection
fraction and stroke volume as reflected by an increase in
the LVOT velocity time integral (V TI). No change in AVA
with <20% change in stroke volume or LVOT V TI points
to poor LV contractile reserve. Patients with low-flow
reserve constitute approximately 3040% of patients with
low LVEF, LF-LG AS, and its presence portends a higher
operative mortality (2233%) than those with normal
flow reserve (58%).5962 However, the postoperative LVEF
recovery and the late survival rate in patients who survived
surgery were equivalent to those with normal flow reserve70
and much better than in those with no flow reserve treated
medically.66 In summary, the assessment of LV flow
reserve by DSE is useful to estimate the operative risk but
the absence of flow reserve is not predictive of recovery of
LV function, improvement in symptomatic status, and late
survival after surgery.61,62,70 The absence of LV flow reserve,
therefore, should not preclude consideration of AVR in
these patients.61,66 However, since the operative risk for
surgical AVR is generally very high in the absence of flow
reserve, transcatheter aortic valve replacement (TAVR)
may be a valuable alternative in these patients.71

Paradoxical Low Flow, Low Gradient

Aortic Stenosis with Preserved Ejection
Fraction

Fig. 79.8: Dobutamine stress echocardiography to evaluate the

left ventricular flow reserve and to distinguish the true severe
aortic stenosis from pseudosevere aortic stenosis. (EOA: Effective
orifice area (in square centimeters); EOAProj, projected EOA at
normal flow rate (in square centimeters); P: Mean transvalvular
gradient (in mm Hg); SV: Stroke volume).

As many as one third of patients with severe AS on the basis

of AVA calculation have unequivocally low transvalvular
gradients (mean gradient < 40 mm Hg) despite a preserved
LV ejection fraction of >50%.72 These patients tend to
be in the older age group, have small, concentrically
hypertrophied LV with a lower LV diastolic volume index
(52 12 mL/m2), a higher level of LV global afterload
reflected by a higher valvuloarterial impedance, and a
lower overall 3-year survival a poor outcome if managed
medically.7274 The diagnosis is suggested on echo/Doppler
studies by noting paradoxically low flows with a calculated

1930

Section 7: Miscellaneous and Other Noninvasive Techniques

stroke volume index of 35 mL/m2 or less in the presence

of normal LV ejection fraction as well as decreased LV
midwall shortening. Currently, paradoxically low-flow,
low-gradient severe AS is considered a distinct clinical
entity. The distinctive features of this entity are robust
LV concentric remodeling and myocardial fibrosis, both
contributing to the restrictive physiology. The fibrosis is
mainly subendocardial and is responsible for marked
attenuation of intrinsic LV systolic function, not evidenced
by the LVEF, but rather by other more sensitive parameters
directly measuring LV midwall or longitudinal axis
shortening.7477

Live/real time three-dimensional transthoracic echocardiography (3D TTE) with a full matrixarray transducer has

transformed the complex technique of 3D imaging into an

efficient, cost-effective, and clinically viable procedure.78,79
Rotating the 3D data set and cropping from the aorta to
the LVOT and stopping at the tips of the trileaflet or BAV
allows for the direct planimetry of the stenotic valve and
calculates AVA with increased confidence level.8083
The inherent ability to present data in 2D cut-planes
from a 3D data set permits assessment of angulated
orifices and domed AVs as well as localizes and evaluates
the exact individual sites of obstruction in patients
with associated subvalvular, supravalvular, or tandem
obstruction (see Figs 79.3 to 79.5).8386 In low-flow states
as in patients with severe AS and low cardiac output, 3D
TTE may also supplement 2D TTE; the AVA can be directly
inspected and measured following dobutamine infusion,
which obviates reliance on 2D TTE/Doppler gradients
that provide only indirect estimations of AS severity.
Although 3D TTE can be performed in all patients with
AS, it is especially important to perform 3D TTE when

Figs 79.9A to C: Aortic valve stenosis. Live/real time threedimensional transthoracic echocardiography. (A to C) Careful
cropping of the parasternal long-axis data set at the flow-limiting
tips of the aortic valve (AV) leaflets demonstrated a bicuspid
morphology and severe stenosis. (LA: Left atrium; LV: Left
ventricle). (Movie clips 79.9A to C Parts 1 to 4).
Source: Reproduced with permission from Nanda NC, Hsiung MC,
Miller AP, Hage FG. Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.

Live/Real Time Three-Dimensional

Transthoracic Echocardiography
(Figs 79.9 to 79.16)

Chapter 79: Echocardiography in the Elderly

1931

Figs 79.10A to C: Another adult patient with bicuspid aortic valve

stenosis. Live/real time three-dimensional transthoracic echocardiography. (A and B) The arrowhead in A points to thickened aortic
valve (AV) leaflets with restricted opening motion viewed in parasternal long axis. Short-axis cropping at the AV tip demonstrates
a severely stenotic bicuspid valve; (C) QLab cropping shows a
small orifice consistent with significant AV stenosis. Despite the
presence of a motion artifact, it was possible to assess the orifice
size in this patient. (LA: Left atrium; LV: Left ventricle). (Movie clips
79.10A to C Parts 1 to 4).
Source: Reproduced with permission from Nanda NC, Hsiung MC,
Miller AP, Hage FG. Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.

Fig. 79.11: Mild aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction showing a mildly calcified
tricuspid aortic valve (AV) with minimal stenosis. (LA: Left atrium;
RA: Right atrium; RV: Right ventricle) (Movie clip 79.11).

Fig. 79.12: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. Demonstrates a heavily
calcified, vertically oriented bicuspid aortic valve (AV) with a very
small orifice indicative of severe stenosis. (LA: Left atrium; RA:
Right atrium; RV: Right ventricle) (Movie clip 79.12).

1932

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 79.13: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. Demonstrates a heavily
calcified, horizontally oriented bicuspid aortic valve (AV) with a very
small orifice denoting severe stenosis. (LA: Left atrium; RA: Right
atrium; RV: Right ventricle) (Movie clip 79.13).

Figs 79.14A and B: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. (A) Shows a heavily
calcified tricuspid aortic valve (AV) with small a very small orifice consistent with severe stenosis; (B) In addition, a perforation
(arrowhead) is visualized in diastole in one of the cusps. (LA: Left atrium; RA: Right atrium; RV: Right ventricle) (Movie clip 79.14).

Fig. 79.15: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. Demonstrates severe
tricuspid aortic valve (AV) stenosis with a very small orifice but
only mild calcification. (LA: Left atrium; RA: Right atrium; RV: Right
ventricle) (Movie clip 79.15).

Chapter 79: Echocardiography in the Elderly

1933

Table 79.2: Indications for Performing Three-Dimensional Echocardiography in Older Adults with Suspected Aortic Stenosis

Increased likelihood of significant pressure recovery:

Aortic valve doming on two-dimensional echocardiography (in both bicuspid and tricuspid aortic valves)
Tubular, rather than discrete aortic stenosis
Narrow or borderline-narrow left ventricular outflow tract diameter ( 2.0 cm)
Small ascending aortic diameter (< 3.0 cm measured at or just beyond the sinotubular junction)
Discrepancy between two-dimensional echocardiography and Doppler findings
Discrepancy between two-dimensional echocardiography/Doppler and clinical findings
A case can be made for performing three-dimensional echocardiography in all patients with suspected aortic stenosis because
of the additional data provided and to avoid the dependence on gradients obtained by technicians; however, this may not be
practical or cost-effective

Figs 79.16A and B: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. (A) Demonstrates
a horizontally oriented severely stenotic bicuspid aortic valve (AV) with a valve area of 0.9 cm2; (B) Diastolic frame shows a perforation
(arrowhead) in the AV. (LA: Left atrium; RA: Right atrium; RV: Right ventricle; RVO: Right ventricular outflow tract) (Movie clip 79.16).

there is reason to believe that 2D TTE/Doppler may not

provide an accurate assessment of AS severity (Table 79.2).
However, it is important to recognize that in patients with
poor acoustic windows or those with very heavily calcified
valves, which are more commonly seen in the elderly, 3D
TTE images may not be adequate quality to planimeter
the aortic orifice. Some investigators have reported that
this may occur in as many as 20% of patients, although
in our experience it is possible to measure the AVA by 3D
TTE planimetry in a much greater proportion of patients
with AS. 3D TEE reconstruction or live/real time 3D TEE
may supplement 3D TTE in patients with poor acoustic
windows.

Ventricular Response to Aortic Stenosis

Concentric hypertrophy is a compensatory response to
chronic elevation of afterload and increased intracavitary

pressures due to AS. The increased muscle mass normalizes

wall stresses by an increase in left ventricular wall thickness
relative to the chamber size by operation of Laplaces law.87
Unfortunately, this compensatory response is maladaptive
because the hypertrophied heart may have reduced
coronary blood flow per gram of muscle88 and also exhibit
an attenuated coronary vasodilator reserve, even in the
absence of epicardial CAD.89 The hemodynamic stress
of exercise or tachycardia can elicit a maldistribution
of coronary blood flow and subendocardial ischemia,
which can contribute to systolic or diastolic dysfunction
of the LV. Pressure overload left ventricular hypertrophy
(LVH) has been shown to increase sensitivity to ischemic
injury, with larger infarcts and higher mortality rates than
in the absence of hypertrophy.90,91 In geriatric population
especially in the female, an excessive or inappropriate
degree of hypertrophy has been observed with wall
thickness greater than is necessary to counterbalance

1934

Section 7: Miscellaneous and Other Noninvasive Techniques

the high intracavitary pressures. This response maintains

the systolic wall stress relatively low and ejection fraction
relatively high, but has been associated with high
perioperative morbidity and mortality.9294
In the geriatric patient with AS, the hemodynamic
load to the LV is summation of the resistance offered
by the stenotic AV and the attenuated systemic arterial
compliance. Hence, description of the severity of the AS in
elderly is rather simplistic if described merely by AVA and
transvalvular gradients as it does not take into consideration
the influence of altered systemic arterial compliance and
systemic vascular resistance. The global LV hemodynamic
load can be quantified by valvuloarterial impedance
(Zva), which incorporates the degree of valve stenosis and
the systemic arterial compliance.95,96 Zva has been shown
to be superior to the conventional indices of AS severity in
predicting LV dysfunction and patient outcome.73,95

Transcatheter Aortic Valve

Replacement (Figs 79.17A to E)
Standard surgical AV replacement in the elderly carries
a high mortality in the immediate postoperative period,
but subsequent to this the mortality in the long term
approximates that in the younger patients. However, the
presence of serious comorbidities and other contraindications often precludes surgery in these patients. In
this regard, the advent of TAVR has been a boon for
the elderly and is now considered a standard of care for
patients with severe symptomatic AS who have a high
risk or contraindications to conventional surgical AV
replacement. This is a catheter-based procedure in which
a prosthetic valve mounted on a catheter tip is implanted
inside the preexisting stenosed AV with angiographic
and echocardiographic guidance. Based on data from
prospective randomized clinical trials, this procedure
has been shown to confer improvement in survival in
nonoperative candidates when compared to conventional
medical treatment97 and confers equivalent survival benefit
compared to open heart surgery in high-risk patients.98
During the procedure, accurate measurement of the AV
annulus or ring is pivotal to select the most appropriate
prosthetic valve size to avoid complications such as
prosthesis migration, paravalvular aortic regurgitation,99
or aortic annulus rupture. Some paravalvular regurgitation
leading to postoperative aortic insufficiency (AI) is
common post TAVR and is known to occur in 2240%
of patients.100,101 Moderate or severe paravalvular aortic

regurgitation (AR) was seen in 12.2% of TAVR patients in

the seminal PARTNER (Placement of Aortic Transcatheter
Valves) trial, a significantly higher figure than seen in the
surgical group (0.9%).98 AR, when more than just trace,
has been shown to be a predictor of both in-hospital and
long-term mortality.101 One of the important predictors
of paravalvular AR and postprocedure AR is prosthesis
annular mismatch. Conventionally, the manufacturers
recommendations on size selection had been based on
2D echocardiographic measurement of the AV annulus.
However, the 2D echocardiographic measurement has
been shown to significantly underestimate the AV annular
dimensions.102104 Furthermore, there is significant interobserver and intraobserver variability in measurement
because of uncertainty about where in the dense calcium
to place the cursor and also the frequent need for guessing
where the leaflet hinge point is. 3D echocardiographic
measurements may mitigate some of these problems105
but it still underestimates the annular dimension when
compared to multidetector computerized tomographic
(MDCT) measurements. MDCT is considered as the
reference standard for measuring the aortic annulus in
most centers. In the majority of patients, the AV ring is an
oval structure with sagittal diameter being the minimum
diameter and the coronal diameter being the maximum
diameter of the annulus. Therefore, averaging the
minimum and maximum diameter to get a mean diameter
is a pragmatic way of sizing the annulus. However, in one
third of the patients, the true minimum and maximum
diameter will be at oblique angles and not in the true
coronal and sagittal plane, respectively, and this leads to
an imperfect and imprecise measurement in up to 33% of
the patients.106 These shortcomings have prompted many
TAVR centers to use a multimodality imaging approach in
which all patients receive MDCT along with 2D TTE, and
2D and 3D TEE. Some of the complications of TAVR in the
elderly such as stroke and paravalvular aortic regurgitation
may be reduced with further impending improvements in
the technique and development of improved valve design.

AORTIC ANEURYSM (FIGS 79.18A AND B)

An aortic aneurysm represents a pathologically dilated
segment of the aorta that has the propensity to expand
and rupture. The Olmsted county study estimated the
incidence of thoracic aortic aneurysm (TAA) to be 5.9
per 100,000 person-years compared with 350 cases
for abdominal aortic aneurysms (AAA).107 However,

Chapter 79: Echocardiography in the Elderly

1935

Figs 79.17A to E: Transcatheter aortic valve replacement (TAVR)

in an 85-year-old patient. Two-dimensional (2D) and live/real time
three-dimensional (3D) transesophageal echocardiography. (A)
Pre procedure. 3D short-axis view shows a calcified aortic valve
(arrow) with a very small irregular orifice consistent with severe
aortic stenosis; (B) During procedure. Arrow shows a catheter
in the region of the aortic root and valve imaged using the 3D
approach; (C to E) Post procedure. Arrow in C and D points to a
thin aortic valve leaflet seen in both 2D and 3D long-axis views.
The upper arrow in E points to significant paravalvular aortic
regurgitation clearly seen beyond the confines of the prosthetic
elements visualized in the 2D short-axis view. Lower arrow points
to mild valvular regurgitation located within the ring. (AO: Aorta; LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle)
(Movie clips 79.17A to D).

more contemporaneous estimates in the era of CT and

echocardiography indicate the incidence to be at least 10
per 100,000.108110 The increment is largely due to improved
diagnostics and case ascertainment.111 Of the TAA, the

ascending aorta is affected in 50 to 60% of cases, the aortic

arch in < 10%, and the descending thoracic aorta in 30 to
40%. The mean age of diagnosis is 5969 years with a male
predominance of 2:1 to 4:1.

1936

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 79.18A and B: Aortic arch aneurysm with rupture. Live/real time three-dimensional transesophageal echocardiography. (A) The
top arrowhead points to the aneurysm in a 76-year-old male with a bioprosthetic aortic valve, which contains thrombus (T), and the bottom arrowhead denotes the site of rupture of this aneurysm into the mediastinum. Movie clips 79.18A Parts 1 and 2. In Movie clip 79.18A
Part 2, arrowheads in the right upper panel and in the left lower panel point to en face views of the aneurysm rupture site and mouth of
the aneurysm, respectively. Note the presence of spontaneous echo contrast suggestive of low blood flow state in the aneurysm and
aortic arch (ACH); (B) Ascending aortic aneurysm in a 67-year-old male with rupture into the mediastinum. Arrowhead points to a large
rupture visualized en face measuring 1.75 2.34 cm, area 3.01 cm2. It was not possible to visualize the rupture site en face by twodimensional echocardiography. Movie clip 79.18B.
Source: Reproduced with permission from Joshi D, Bicer E, Donmez C, et al. Incremental value of live/real time three-dimensional
transesophageal echocardiography over the two-dimensional technique in the assessment of aortic aneurysm and dissection. Echocardiography. 2012;29:62030.

The etiology of aneurysms can be degenerative, related to cystic medial degeneration (CMD), genetically triggered, atherosclerotic, inflammatory, traumatic, or mycotic. CMD is a common denominator in many genetically
triggered TAA including Marfan syndrome (MFS). In addition, normal aging is associated with some degree of CMD
and this process is accentuated by hypertension. These
changes cause progressive weakening of the aortic wall,
leading to dilation and aneurysm formation. The genetically triggered TAA can be syndromic with multisystem
manifestations [MFS, LoeysDietz syndrome (LDS), vascular EhlersDanlos syndrome (vEDS), Turner syndrome
(TS)] or nonsyndromic [familial TAA and dissection syndrome (FTAA/D), aortopathy associated with BAV] that
manifests with thoracic aortic disease alone.
The TAA in MFS involves the sinuses of Valsalva
with the ascending aorta above the sinotubular junction
usually being normal in dimension. TTE provides excellent
imaging of the aortic root and the sinuses of Valsalva and
is therefore an adequate imaging tool for evaluation and
surveillance of TAA size in MFS. In contrast, in LDS aortic
root, involvement is less common, with the descending and
abdominal aorta and aortic branch vessels more frequently involved. TTE alone may, therefore, not be sufficient
enough for adequate evaluation as well as surveillance

of TAA in LDS. TEE can image almost the entire thoracic

aorta well and is an attractive alternative to computerized
tomography or MRI. FTAA/D accounts for 20% of TAA
and exhibits autosomal dominance inheritance with
reduced penetrance, variable age of onset, and variable
expression with regards to location of aneurysm112 with the
pedigree showing TAAs in 66%, AAAs in 25%, and cerebral
aneurysms in 8%. Imaging of the aorta in family members
often reveals asymptomatic aneurysms, and the incidence
of aortic disease increases with advancing age. BAV affects
approximately 1% of the population and the aortopathy
associated with BAV is one of the most common causes of
ascending aortic aneurysm. The TAA associated with BAV
often arises in the proximal to mid-ascending aorta, and
is not a mere passive post-stenotic dilation, but a direct
consequence of abnormalities of the aortic media. It may
be present in absence of AS or AR and may occur late after
AVR. Due to its predilection to form an aneurysm in the
proximal to the mid-ascending aorta, it is imperative that
the entire ascending aorta be visualized to evaluate for
aneurysms above the sinotubular junction. This implies
that imaging with TTE may not be optimal enough and
may require evaluation with TEE or CT or MRI. The
atherosclerotic TAA have predilection for the descending
aorta and generally originate just distal to the origin of

Chapter 79: Echocardiography in the Elderly

1937

the left subclavian artery. Morphologically, they are either

fusiform or saccular and may extend into the abdominal
aorta (thoracoabdominal aneurysm) or coexist with AAA.
TEE is excellent for evaluation of the atherosclerotic TAA
but may need additional imaging to evaluate the distal
end of the thoracoabdominal aneurysm or evaluate the
presence of synchronous AAA.
Many factors influence the natural history of TAA;
the most robust determinants of likelihood of rupture
or dissection are the underlying causes of TAA, location
of TAA, pre-existing diameter of the TAA, and growth
velocity. Intervention is generally recommended when
the TAA reaches a certain size threshold in an appropriate
candidate. In general, prophylactic surgical intervention is
recommended when the ascending TAA reaches 5.5 cm,
5.0 cm in the setting of BAV, 4.55 cm in setting of MFS and
FTAA/D, or 4 cm in LDS patients, as they are phenotypically
more aggressive.113116 In TS, prophylactic surgery has

been recommended when the ascending aorta is 3.5 cm

or larger or 2.5 cm/m2 or larger.117 Other modifiers that
determine the timing of intervention include the velocity
of aneurysm growth > 0.5 cm/year, coexisting valvular
disease and indications for concomitant cardiac surgery,
and body size; surgery is recommended if the maximal
cross-sectional area (in cm2) of the ascending aorta or root
divided by the patients height in meters exceeds a ratio of
10.115 Prophylactic intervention is indicated in descending
TAAs with a diameter > 5.5 cm or thoracoabdominal aortic
aneurysm > 6 cm.115

Figs 79.19A to D

AORTIC DISSECTION
(FIGS 79.19 TO 79.22)
Although aortic dissection may occur in genetically
predisposed young adults such as those with MFS, LDS,
vEDS, FTAA/D, BAV, or TS, but it is mainly a disorder of the

1938

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 79.19A to G: Aortic dissection in an elderly patient. Twodimensional transthoracic and transesophageal echocardiography.
(A to G) Transthoracic parasternal long-axis (A) and apical fivechamber (B) views demonstrate irregularly moving linear echoes
(arrows) in the aorta (AO), resembling a wiggling worm typical of
aortic dissection. Thus, in some cases, a definitive diagnosis of
aortic dissection can be made by transthoracic echocardiography
precluding a transesophageal study, which can subsequently be
done in the operating room. This prevents undue delay in taking the
patient for surgery. In this patient, the dissection flap can be clearly
seen protruding into the left ventricular outflow tract in diastole and
this resulted in severe aortic regurgitation. Transesophageal long(C) and short-axis (D) views of the aortic root and ascending aorta
in another elderly patient show the dissection flap (arrow) extending into the right coronary artery (arrowhead). It is also seen in the
vicinity of the origin of left main coronary artery (LMCA) but does
not extend into it. Examination of the descending thoracic aorta in
short- (E and F) and long-axis (G) views show the perfusing lumen
(PL) surrounded by the nonperfusing lumen (NPL, arrow). Movie
clips 79.19E shows the dissection flap mimicking opening and closing motion of a persons mouth as if the patient was begging for
help, the so-called Help sign. (LA: Left atrium; LV: Left ventricle;
RV: Rright ventricle). (Movie clips 79.19A to E).

Figs 79.20A and B

Chapter 79: Echocardiography in the Elderly

Figs 79.20C to H

1939

1940

Section 7: Miscellaneous and Other Noninvasive Techniques

I
elderly with ascending aortic dissection, most prevalent
between 50 and 60 years of age, and descending aortic
dissection peaking at 6070 years of age. Although the
classical presentation of the patient to the emergency
room is severe tearing chest pain radiating to the back
along with symptoms of autonomic activation, this
typical presentation may be modified by the location of
dissection and coexisting complications including acute
coronary syndrome (ACS). Aortic dissection and ACS can
present synchronously as the aortic dissection can cause
impairment to coronary flow due to a variety of reasons:
the dissection flap may mechanically obstruct the orifice of
the left or right coronary artery, the dissection process may
extend along the walls of a coronary artery significantly
narrowing the vessel lumen, subadventitial hematomas
(commonly present and alerts the surgeon to the presence
of dissection as soon as he opens the chest wall) may
compress the coronary arteries, localized pericardial
effusion either due to heart failure or partial rupture of the
dissected aorta into the pericardium may also compress
the coronaries, and finally, hypotension resulting from
dissection in an elderly patient with preexisting significant
CAD may precipitate severe myocardial ischemia and/or
myocardial infarction. It is important that the diagnosis
of acute aortic dissection be made emergently and if it
involves the ascending aorta and/or aortic arch (DeBakey
type I or type II dissection or Stanford type A dissection),
urgent surgical intervention is necessary. Any delay in
doing this substantially increases the mortality rate,
which in acute dissection is very high, up to 12% per
hour reported in the first several hours after dissection.118
This has led to the recommendation that even a coronary
arteriogram should not be performed in an elderly patient

Figs 79.20A to I: Aortic dissection rupture into the right ventricular

outflow tract. (A to C) Two-dimensional transthoracic echocardiography. (A) Parasternal long-axis view. The arrowhead points to the
site of rupture of the false lumen (FL) into the right ventricular outflow tract (RVOT); (B) Color Doppler examination. The arrowhead
on the right points to a communication between the true lumen (TL)
and FL. The arrowhead on the left shows flow signals moving from
the FL to the RVOT. Moderate aortic regurgitation (AR) is also displayed; (C) Continuous wave spectral Doppler interrogation of the
rupture site showing continuous flow throughout the cardiac cycle;
(D to G) Live/real time three-dimensional transthoracic echocardiography; (D) The arrowhead points to the en face view of the
rupture site upon cropping of the data set. It is roughly elliptical in
shape and measured 0.51 cm2 in area (A) by planimetry; (E) QLab
image of the same data set with the orifice (arrow head) planimeterized; (F) The arrowhead points to compression of the main pulmonary artery (PA) by the FL; (G) Extension of the dissection (arrowhead) into the left common carotid artery (LCC) is shown; (H and I)
Computed tomography angiogram. (H) Left anterior oblique (LAO)
view demonstrating the communication (arrowhead) between the
FL and RVOT; (I) Cut slab volume rendered image in oblique LAO
view demonstrating PA compression by the dilated FL. The arrow
in figures H and I points to the dissection flap extending into the
aortic arch and the brachiocephalic artery (BR). (A: Anterior; AO:
Aaorta; F: Foot; H: Head; L: Left; LA: Left atrium; LV: Left ventricle; P: Posterior; PV: Pulmonary valve; R: Right; RA: Right atrium).
(Movie clips 79.20AI).
Source: Reproduced with permission from Hansalia S, Nanda NC,
et al. Live/real time three-dimensional transthoracic echocardiographic assessment of aortic dissection rupture into right ventricular outflow tract: a case report and review of literature. Echocardiography. 2009;26(1):1006.

because that would further incur delay and catheter

manipulation in the aorta may aggravate the dissection
if the catheter passes into the nonperfusing lumen. It
is also important to differentiate aortic dissection from
an ACS to avert an immediate disastrous outcome from
administration of anticoagulants or antiplatelet agents to
the patient. In addition, it is paramount to be aware, as
mentioned above, that both may coexist and the ACS may
in fact be related to aortic dissection.
CT scan is often used to detect or rule out aortic
dissection because of its easy availability in the emergency
room but occasionally gives false-positive results. In
many centers, echo machines are also available in the
emergency department and the emergency physicians
are trained to perform transthoracic studies. A linear
echo moving in an irregular, chaotic manner in the aortic
lumen (a worm wiggling in the aorta) is highly specific
for aortic dissection. However, a transthoracic echo may
be negative in many instances and most often one has
to resort to TEE to make the diagnosis. In addition to
finding an irregular flap-like echo in the aortic lumen,

Chapter 79: Echocardiography in the Elderly

1941

Figs 79.21A to C: Aortic dissection. Transesophageal threedimensional echocardiographic reconstruction. (A and B) The
descending thoracic aorta was examined using multiple cut sections
and various viewing angles. Both the true (TL) and the false (FL)
lumens are well visualized, and the dissection flap (F) presents as
a sheet-like structure along the aortic length. The communication
(arrows) between the true and false lumens is viewed en face using
a transverse section in C. (H: Mediastinal hematoma that resulted
from rupture of dissection).
Source: Reproduced with permission from Nanda NC, Khatri G,
et al. Three-dimensional echocardiographic assessment of aortic
dissection. Echocardiography. 1998;15(8):74554.

Figs 79.22A and B: Aortic dissection. Two-dimensional (A) and live/real time three-dimensional (3D) transesophageal; (B) echocardiography. Arrowhead points to a linear echo in ascending aorta (AO) consistent with dissection. However, instrument artifacts may present
as linear echoes in aortic lumen mimicking dissection. Therefore, in cases where a doubt exists as to the origin of the linear echo, it is
best to do a live/real time 3D study. The dissection flap will then appear as a sheet with finite width when viewed en face reflecting splitting of the aortic wall by the dissection process. Thus, aortic dissection can be confidently diagnosed or ruled out. (RPA: Right pulmonary
artery) (Movie clip 79.22).

1942

Section 7: Miscellaneous and Other Noninvasive Techniques

differential color Doppler flow signals in the nonperfusing

and perfusing lumens. and detection of a communication
between the perfusing and nonperfusing lumens by
color Doppler further add to the confidence level of the
diagnosis. Visualization of spontaneous echo contrast
signals in the vicinity of the aorta should alert one to the
presence of dissection rupture and calls for immediate
surgery. In these instances, rupture has occurred but has
been temporarily closed by a plug of fibrin preventing
exsanguination.
Color Doppler is also useful in not only detecting
the presence of aortic regurgitation but also assessing
its severity. Severe aortic regurgitation resulting from
the dissection flap interfering with aortic cusp motion
and/or prolapsing into the LVOT can be diagnosed and
the surgeon informed sparing the patient concomitant
AV replacement. On the other hand, severe aortic
regurgitation due to a dilated or distorted aorta in an
elderly patient or due to thickened or retracted valve
cusps found on the echocardiogram would require AV
replacement. Over all, TTE has a sensitivity of 78100%
for type A aortic dissection, but only 3155% in type B
dissection; therefore, a negative TTE does not exclude
acute aortic dissection. However, TEE is highly accurate
for the evaluation and diagnosis of acute aortic dissection
and boasts a sensitivity of >98% and specificity of 9497%.
During a transesophageal study, it is important to examine
the proximal coronaries for obstruction produced by the
dissection flap impinging on the orifices or narrowing
produced by extension of dissection along their walls.
In the older patient, coronary lumen narrowing and
obstruction produced by atherosclerotic plaques can also
be visualized by echocardiography and this is important
information for the surgeon, especially if a preoperative
coronary angiogram was not performed. Depending on
the clinical status of the patient, the surgeon may consider
placing bypass grafts. The perfusing lumen (which may
occasionally be the false lumen) can be differentiated
from the nonperfusing lumen by its expansion during
systole. Also, color Doppler flow signals are usually more
prominent in the perfusing lumen as compared to the
nonperfusing lumen. Artifacts are not uncommonly seen
in the aorta during transesophageal examination and have
been mistaken for dissection in inexperienced hands with
near-disastrous results. These artifacts are linear in nature
and their motion tends to be parallel to the movement of
the aortic walls. Also, they may extend beyond the aortic
wall, which easily identifies them as artifacts and not

dissection. However, some dissections present as virtually

nonmobile linear echoes and distinguishing them from
an artifact becomes very difficult. In these instances, 3D
TTE and TEE are invaluable in making a diagnosis with
certainty. Cropping the 3D data set will demonstrate the
dissection flap as a sheet of tissue that clearly differentiates
it from the linearly presenting artifactual echo. Basically,
aortic dissection represents splitting of the wall of the
aorta and should, therefore, present as a tissue with finite
width rather than a linear echo seen on 2D imaging, which
provides only thin slice-like sections of the aorta at any
given time.
Dissection involving only the descending thoracic aorta
(DeBakey type III or Stanford type B) may be diagnosed by
2D TTE using the suprasternal approach and by examining
the descending thoracic aorta imaged behind the LV/left
atrium in the parasternal long-axis view. Dissection may
also be visualized in the proximal abdominal aorta using
the subcostal approach. The diagnosis is made by noting
echocardiographic findings similar to those mentioned
above for dissection involving the ascending aorta/aortic
arch. Unlike ascending aorta/aortic arch dissection, these
patients are generally managed conservatively unless
there is evidence of aortic enlargement and other signs of
progression or there is compromise of blood supply to a
visceral organ or spinal cord with impending paraplegia.
In 1520% of patients, aortic dissection does not
communicate with the aortic lumen forming an IMH. This
presents as a thickened aortic wall and is considered a
precursor of aortic dissection. This is especially common
in the hypertensive and older patient and most commonly
involves the ascending aorta. Echocardiography is useful
to follow its course over time and any increase in size or
progression to dissection with communication with the
vessel lumen warrants consideration for surgery. IMH
has been found to progress to aortic dissection or rupture
in up to 45%, regress completely in 34%, or evolve into
pseudoaneurysm in 24%.119 Small, localized IMH are
generally conservatively managed.

LEFT VENTRICULAR MASS,

DIMENSIONS, AND FUNCTION
LV mass increased monotonically with age in the whole
Framingham study cohort, but not in a subgroup of normal
individuals.120 Other studies have also corroborated
that after excluding the influence of coexisting disease,
LV hypertrophy is not an inevitable consequence of

Chapter 79: Echocardiography in the Elderly

aging.121,122 However, LV wall remodels with normal aging

primarily with an increase in relative wall thickness (ratio
of wall thickness to chamber radius) but with little or no
increase in overall LV mass.123 This concentric remodeling
parallels the age-related stiffening of the arterial tree,
while hypertension induces concentric hypertrophy of the
myocardium with an increase in LV mass.
Aging induces significant alteration in LVOT geometry124 primarily due to reduction in the angle between
the aorta and the interventricular septum. The age-related
dilation and lengthening of the aorta may push the septum downward and kink its upper portion, accentuating
the septoaortic angle. This shifts the position of the interventricular septum relative to the chest wall and leads to
systematic errors in echocardiographic M-mode measurements across the LV. This anatomical alteration also induces a septal bulge in >10% of the geriatric population but
is not associated with higher LVOT velocity or increased
LV mass index. LV systolic function remains relatively well
preserved and there are no significant alterations in LV
ejection fraction with normal aging.125
The Doppler transmitral inflow velocities are used as
surrogate indices for echocardiographic assessment of
ventricular diastolic function. Both in the Framingham
study126 and the Cardiovascular Health study,127 age was
the predominant determinant of Doppler indices of LV
diastolic function in normal subjects with decrease in
peak early velocity (E-wave) and increase in peak late
velocity (A-wave) with increasing age. These indices
change gradually and progressively128,129 with decrement
in E-wave velocity of approximately 50%126,130 together
with a 40% increment of A-wave velocity between 30 and
70 years of age.131 The ratio of the peak E and A is shown
to range from a mean of 2.08 0.55 for subjects in their
third decade to 0.84 0.29 for those in their eighth decade.
A peak velocity E/A ratio < 1 is abnormal in subjects aged
< 40 years, but occurs in most subjects aged 70 years.126
These indices are accompanied by a prolongation of the
deceleration time of the E-wave,132 and an increase in left
atrial size127 and are present in more than 85% of healthy
people over the age of 70 years.129 However, mitral inflow
velocities profiles are affected by loading conditions,133 left
atrial pressure,131 are reflective of flow patterns, and are
not synonymous with function. It is therefore desirable
to have additional variables to complement mitral
inflow Doppler velocity in evaluating diastolic function.
Pulsed wave tissue Doppler imaging (TDI) measures low
amplitude myocardial velocities with a high sampling rate

1943

and can provide a measurement of the rate of change of

longitudinal dimension and volume.134 The mitral annulus
velocity profile by TDI during diastole consists of early
diastolic movement (E) that commences simultaneously
with the onset of mitral inflow and late diastolic velocity
(A) that corresponds to the late transmitral flow A. The
mitral annular TDI velocities are relatively less preloadsensitive and fall progressively with increasing age.134 In
addition, significant decrement in the ratio of early to late
myocardial velocities, E/A with increasing age is observed
mimicking the pattern of normal mitral inflow. However,
although reversal of the transmitral E/A ratio occurs in the
60s, reversal of the E to A ratio occurs in the 40s.134,135

ECHOCARDIOGRAPHY IN STROKE
PATIENTS: ASSESSMENT OF
CORONARY STENOSIS
(FIGS 79.23 TO 79.25; ALSO SEE
FIG. 78.13 IN THE CHAPTER 78)
Ischemic stroke constitutes 7080% of all strokes and
is caused by embolic or thrombotic occlusions of the
cerebral vessels and accounts for major morbidity and
mortality in the elderly.136 Embolic occlusions can be of
arterial or cardiac origin, with 1530% attributable to
cardioembolism (CE). Identification of the specific etiology
is crucial for risk stratification and in order to tailor the most
optimal preventive strategy. CE strokes portend a poor
prognosis with increased short- and long-term recurrence,
higher in-hospital mortality, and a higher index of fatal
recurrence versus other causes of stroke.136138 There is
no clear consensus on the indication and the optimal
echocardiographic approach in the cardiac evaluation
of ischemic stroke. The European Stroke Organization
guidelines recommend the use of echocardiography in
selected patients, while the American Stroke Association
guidelines do not make any clear recommendation on
its use..139,140 In real life practice, evaluation for cardiac
source for embolism is one of the most common requests
for the performance of a TEE (>25% of all studies at most
institutions).141 TEE identifies possible sources of CE
including the presence of a patent foramen ovale in >50%
of patients without clinically known heart disease, in
comparison, the diagnostic yield with TTE with agitated
saline-injection is only 25%, which drops to 10% without
saline-injection.142 However, the diagnostic superiority
of TEE does not necessarily translate into altered
therapeutic decisions. While the diagnosis of thrombus,

1944

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 79.23A to D: Ostial left main (LM) and mid-left anterior descending coronary artery (LAD) stenosis in a 72-year-old white male.
Two-dimensional transesophageal echocardiography. (A) Shows an area of flow disturbance in the ostium of the LM (1), preceded by
prominent flow convergence; (B) Color Doppler-guided continuous wave Doppler interrogation shows a very high diastolic velocity of
2.0 m/s (arrows) indicative of severe LM stenosis. Note also the high systolic flow velocity; (C) Demonstrates an area of aliased flow in
the region of mid-LAD (2) with a high diastolic flow velocity indicating significant stenosis; (D) Coronary angiogram showing 95% ostial
LM stenosis and 50% mid-LAD stenosis (arrows). (Ao: Aorta; Cx: Left circumflex coronary artery; LA: Left atrium; PA: Pulmonary artery)
(Movie clip 79.23).
Source: Reproduced with permission from Thakur A, Voros A, Nanda NC, et al. Transesophageal echocardiographic diagnosis of proximal coronary artery stenosis in patients with ischemic stroke. Echocardiography. 1999;16(2):15966.

infective endocarditis, and cardiac tumors will have major

therapeutic implications, entities like patent foramen
ovale, atrial septal aneurysm, complex aortic atheroma,
and spontaneous echo contrast may not always change
patient management despite their robust epidemiological
association with stroke recurrence. Although age has been
used to select the echocardiographic approach in stroke
patients, several studies have concluded that it should not
be used as an exclusion criterion in the selection of patients
for TEE.143 Presence of preexisting heart disease may

increase the probability of finding the source of CE with

TTE alone and may obviate the need for TEE.122 In a study
of 441 unselected ischemic stroke patients, TEE detected a
source of CE in 56% of the patients. However, in the cohort
of patients in sinus rhythm and without apparent heart
disease, TEE altered the therapeutic strategy in only 8%
of patients.144 In another study, the therapeutic impact of
TEE was highest only in the cohort of patients in whom
the cause of ischemic stroke remained cryptogenic despite
routine diagnostics.145

Chapter 79: Echocardiography in the Elderly

Figs 79.24A to F

1945

1946

Section 7: Miscellaneous and Other Noninvasive Techniques

G
Figs 79.24A to G: Detection of left main (LM), mid-left anterior
descending coronary artery (LAD), and left internal carotid artery
stenosis and demonstration of atherosclerotic plaque in the left
subclavian artery in a 59-year-old black female. Two-dimensional
transesophageal echocardiography. (A) The black arrowheads
demonstrate prominent atherosclerotic plaques in the LM, producing 90% stenosis; (B) The arrow shows flow turbulence corresponding to the stenosis seen in A; (C) Color Doppler-guided continuous wave Doppler (arrow) demonstrates a very high diastolic
flow velocity of 3 m/s consistent with severe stenosis. The systolic
flow velocity is also high at 2 m/s; (D) The lower arrowheads point
to a prominent shadowing effect produced by the heavily calcified
plaque in the LM viewed in short axis (top arrowhead); (E) Color
Doppler-guided pulsed wave Doppler interrogation of the mid-LAD
(arrow) demonstrates a high peak diastolic flow velocity exceeding
1 m/s, indicating significant stenosis; (F) Withdrawal of the probe
into the upper esophagus and laryngopharynx demonstrates
marked narrowing of the proximal left internal carotid artery (LICA,
arrow), indicative of severe stenosis; (G) The arrow shows a large
soft plaque occupying 50% of the proximal left subclavian artery
(LSCA) viewed in an oblique axis. (Ao: Aorta; Cx: Left circumflex
coronary artery; LA: Left atrium; LCC: Left common carotid artery;
LEC: Left external carotid artery; LV: Left ventricle; PA: Pulmonary
artery; RVO: Right ventricular outflow tract).
Source: Reproduced with permission from Thakur A, Voros A,
Nanda NC, et al. Transesophageal echocardiographic diagnosis of proximal coronary artery stenosis in patients with ischemic
stroke. Echocardiography. 1999;16(2):15966.

The prevalence of asymptomatic CAD in patients

with cerebrovascular disease is very high146148 and
predominantly accounts for the morbidity and mortality
in patients with stroke or a transient ischemic attack
(TIA).149 Although recurrent strokes occur more frequently
than cardiac events over the long term after stroke,
cardiac events still account for a greater proportionate
mortality.150,151 It may, therefore, be pertinent to identify
asymptomatic coronary artery stenosis that might benefit
from specific additional therapeutic measures to prevent

a first coronary event. The American Heart Association/

American Stroke Association recommends that patients
with stroke/TIA who have Framingham Risk Scorepredicted 10-year CHD risk 20% should be considered
for noninvasive testing for asymptomatic CAD.149 TEE
evaluation of stroke patients provides a unique opportunity
in which the presence of coexisting disease of a major
epicardial artery can be evaluated.152 Direct visualization
of the origins of the left and right coronary arteries is
possible in the transverse imaging plane, basal short-axis
view, just above AV leaflets. With anteflexion and slight
leftward tilting, the left main ostium and the entire length
of the left main coronary artery can be imaged between
the 1 and 2 oclock positions. Continued tracking allows
visualization until the left anterior descending (LAD) and
left circumflex coronary arteries bifurcate. Further inferior
tilting or slight probe angulation allows longer segments of
the LAD to be visualized. The ostium of the right coronary
artery (RCA) can be imaged in the basal short-axis,
transverse image plane, and interrogating between the 6
and 7 oclock positions.153
Coronary stenosis can be diagnosed by Doppler
interrogation of the diastolic flow in the coronary arteries.
The TEE pulsed wave Doppler waveform in the normal
LAD generally consists of a peak velocity of 40 20 cm/s,154
which is fairly consistent with normal maximal diastolic
velocity of 49 20 cm/s and 37 12 cm/s in the LAD and
RCA, respectively.155 In patients with significant coronary
stenosis, pulsed wave Doppler velocities are significantly
increased to >100 cm/s, and the color-flow pattern changes
from a low-velocity, laminar pattern to the characteristic
mosaic color flow pattern seen with turbulent flow and
higher velocities that exceed the Nyquist limit. However,
patients with elevated cardiac output, moderate to
severe aortic regurgitation, or hypertrophic obstructive
cardiomyopathy normally have augmented coronary
artery velocities exceeding 100 cm/s.156,157 Conversely,
extremely severe luminal narrowing (subtotal or highgrade stenosis) may limit coronary flow so severely that
the velocity within the vessel is actually diminished.

MITRAL ANNULAR CALCIFICATION

(FIG. 79.7; ALSO SEE FIG. 70.58 IN
THE CHAPTER 70)
Mitral annular calcification (MAC) is a chronic degenerative process of the mitral valve ring that was first
described in 1908 by Bonninger as associated with

Chapter 79: Echocardiography in the Elderly

1947

Figs 79.25A to E: Anomalous coronary artery in a 69-year-old

black female. Two-dimensional transesophageal echocardiography. The patient presented with pulmonary edema and stroke with
left sided residual weakness. (A and B) Show both, left main (right
arrowhead) and right coronary arteries (left arrowhead) with color
flow in B, arising from a common ostium in the right sinus with the
left main coursing between aorta (AO) and right ventricular outflow
tract (RVO); (C) The arrowhead points to intramyocardial course of
the left anterior descending coronary artery (arrowhead) within the
ventricular septum; (D) Demonstrates the course of right coronary
artery (arrowhead) in the right atrioventricular groove; (E) Coronary
angiogram showing the common origin of the left main (LM) and
right coronary (RCA) arteries. The patient underwent percutaneous
transluminal coronary angioplasty with stent placement in mid RCA
because of 90% stenosis. (Movie clip 79.25) (LVO: Left ventricular
outflow tract; MV: Mitral valve; RV: Right ventricle).
Source: Reproduced with permission from Nanda NC, Bhambore
M, Jindal A, et al. Transesophageal Three-Dimensional Echocardiographic Assessment of Anomalous Coronary Arteries. Echocardiography. 2000;17(1):5360.

1948

Section 7: Miscellaneous and Other Noninvasive Techniques

complete heart block.158 In the cardiovascular health

study, MAC was found in 42% of patients with a mean age
of 76 years.159 In the Atherosclerotic Risk in Communities
(ARIC) study,160 MAC was much less common in younger
subjects with a mean age of 59 and a prevalence of 4.6% in
females and 5.6% in males.
The calcific process in MAC initiates at the attachment
points of the annulus and the calcification may extend to
involve the base and body of both mitral leaflets. Mitral
regurgitation (MR) is commonly associated with MAC,
with a reported incidence of up to 63%.161 The putative
mechanism for MR is accentuated rigidity of the annulus.
Typically, MR is mild to moderate by color Doppler and
not severe enough to require surgical intervention.
However, severe annular calcification may result in
poor penetration of the ultrasonic beam resulting in
underestimation of the severity of mitral regurgitation. In
these cases, it is important to perform a 3D transthoracic
study to assess the regurgitant vena contracta, which
can provide a more quantitative estimate of the severity
of mitral regurgitation. Transesophageal echo may also
need to be done in difficult cases. Typically, leaflet tips
are spared and this distinguishes it from rheumatic mitral
disease, where calcification commonly involves the tips
of mitral leaflets and commissures producing a typical
hockey stick appearance. In the elderly, calcification
may occur in the chordae and papillary muscles but, unlike
rheumatic disease, does not produce chordal shortening
and fusion. MAC in the elderly may occasionally become
severe enough to produce significant mitral stenosis
requiring mitral valve replacement. Calcification may
also involve the aortic annulus and rarely the tricuspid
annulus where fatty deposits are more common. The latter
may mimic a tumor mass. Occasionally, calcification may
involve the whole cardiac skeleton as well as the proximal
ventricular walls in the elderly, resulting in various types
of heart block and other conduction abnormalities. 2D
TTE represents the best noninvasive technique to not only
diagnose but also assess the extent and severity of mitral
annulus calcification as well as calcification affecting
other areas of the heart. The diagnosis is made by noting
the presence of highly echogenic areas in and surrounding
the mitral annulus best seen in the parasternal and apical
planes. A short-axis view at the level of the mitral valve may
show crescent-shaped calcification posteriorly similar to
fluoroscopy and chest X-ray. One particular abnormality
one has to be particularly aware of in the elderly is caseous
calcification. With the passage of time, a severely calcified
area of the mitral annulus may undergo liquefaction

simulating tumor necrosis and may show some mobility.

This is essentially a benign condition and does not need
any surgical intervention. However, some of these patients
have been referred for surgical resection in the mistaken
belief that the lesion is an annular tumor. We have found
live/real time 3D TTE useful in these cases to make a more
confident diagnosis of caseous calcification.162 Careful
cropping of the 3D data set shows the extent and severity
of echolucencies and in one particular elderly patient
studied by us intraoperatively, a typical telltale tooth paste
like appearance was noted when the mass was sectioned
and viewed en face on the 3D data set. This finding was
confirmed at surgery.
MAC is associated with traditional CV risk factors
and calcific aortic disease, coronary atherosclerosis
and chronic kidney disease, and is now considered the
surrogate marker of atherosclerosis. Several trials have
evaluated the association between MAC and CV outcomes.
Data from the Framingham study suggested that each
1-mm increment in MAC increased the composite risk of
CVD, CVD death, and all-cause death by approximately
10%.163 Similarly, MAC was found to incur a significant risk
for coronary events in the ARIC study160 and increased CV
morbidity, CV mortality, and all-cause mortality of patients
with atrial fibrillation in the Belgrade Atrial Fibrillation
Study.164

PROSTHETIC VALVES
(FIGS 79.26 AND 79.27)
Prosthetic valves are common in geriatric population. A
suggested estimate of the prevalence of valve prostheses
ranged from 0.2 per 1,000 in those aged 44 and under
to 5.3 per 1,000 in those 75 years of age and older.165 3D
TTE has been shown to be superior to 2D TTE in the
evaluation of prosthetic valves, especially the mechanical
prostheses, since it allows for the visualization of both
leaflets simultaneously, which increases the confidence
in excluding significant abnormalities.166 Although the
current guidelines continue to be based on the unreliable
Doppler-derived pressure gradients for the assessment
of prosthetic valve dysfunction, 3D TTE is emerging as a
more robust tool.167,168
In conclusion, echo/Doppler techniques represent
the most useful and most cost-effective noninvasive
modalities in the assessment of CV disease entities in the
geriatric patient. In addition, these techniques are also
useful in monitoring various structural and physiological
changes that occur in the CV system with aging.

Chapter 79: Echocardiography in the Elderly

1949

Figs 79.26A to C: St. Jude mitral prosthesis in a 70-year-old female.

Live/real time three-dimensional transthoracic echocardiography.
(A) Arrow points to a large thrombus on the atrial aspect of mitral
valve replacement (MVR); (B) Arrows point to two thrombi on the
ventricular aspect; (C) Arrow points to a thrombus in the left atrial
appendage (LAA; Movie clips 79.26 Parts 1 to 3). The arrowheads
in Part 1 show thrombi on the atrial aspect of the prosthesis. The
arrowhead in Part 2 denotes an irregular thrombus with some
mobility in the left atrial appendage. Part 3 of the movie clip shows
virtually no motion of the prosthetic valve, only ring motion. (AO:
aorta; LA: Left atrium; LV: Left ventricle; R: Reverberations from
MVR; RV: Right ventricle).
Source: Reproduced with permission from Singh P, Inamdar V,
Hage FG, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in evaluation of prosthetic valve
function. Echocardiography. 2009;26:123649.

Fig. 79.27: Tissue mitral prosthesis in a 85-year-old female patient.

Live/real time three-dimensional transthoracic echocardiography.
Arrow points to a tear in one of the leaflets of mitral valve replacement (MVR) that is prolapsing into left atrium (LA; Movie clips 79.27
Parts 1 to 3). (AV: Aortic valve). Other abbreviations as in previous
figure.
Source: Reproduced with permission from Singh P, Inamdar V,
Hage FG, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in evaluation of prosthetic valve
function. Echocardiography. 2009;26:123649.

1950

Section 7: Miscellaneous and Other Noninvasive Techniques

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134. Sohn DW, Chai IH, Lee DJ, et al. Assessment of mitral
annulus velocity by Doppler tissue imaging in the
evaluation of left ventricular diastolic function. J Am Coll
Cardiol. 1997;30(2):47480.
135. Rodriguez L, Garcia M, Ares M, et al. Assessment of
mitral annular dynamics during diastole by Doppler
tissue imaging: comparison with mitral Doppler inflow
in subjects without heart disease and in patients with left
ventricular hypertrophy. Am Heart J. 1996;131(5):9827.
136. Kolominsky-Rabas PL, Weber M, Gefeller O, et al. Epidemiology of ischemic stroke subtypes according to TOAST
criteria: incidence, recurrence, and long-term survival
in ischemic stroke subtypes: a population-based study.
Stroke. 2001;32(12):273540.
137. Eriksson SE, Olsson JE. Survival and recurrent strokes in
patients with different subtypes of stroke: a fourteen-year
follow-up study. Cerebrovasc Dis. 2001;12(3):17180.
138. Arboix A, Garca-Eroles L, Massons J, et al. Predictive clinical
factors of in-hospital mortality in 231 consecutive patients
with cardioembolic cerebral infarction. Cerebrovasc Dis.
1998;8(1):813.
139. European Stroke Organisation (ESO) Executive Committee;
ESO Writing Committee. Guidelines for the management
of ischaemic stroke and transient ischaemic attack 2008.
Cerebrovasc Dis. 2008;25:457507.
140. Adams HP Jr, del Zoppo G, Alberts MJ, et al. American Heart
Association; American Stroke Association Stroke Council;
Clinical Cardiology Council; Cardiovascular Radiology and
Intervention Council; Atherosclerotic Peripheral Vascular
Disease and Quality of Care Outcomes in Research
Interdisciplinary Working Groups. Guidelines for the early
management of adults with ischemic stroke: a guideline
from the American Heart Association/American Stroke
Association Stroke Council, Clinical Cardiology Council,
Cardiovascular Radiology and Intervention Council,
and the Atherosclerotic Peripheral Vascular Disease and
Quality of Care Outcomes in Research Interdisciplinary
Working Groups: the American Academy of Neurology
affirms the value of this guideline as an educational tool
for neurologists. Stroke. 2007;38(5):1655711.
141. Dressler FA, Labovitz AJ. Arterial emboli and cardiac
masses: Role of transesophageal echocardiography. In:
Maurer G, editor. Transesophageal Echocardiography. New
York, NY: McGraw-Hill; 1994:111.
142. Rahmatullah AF, Rahko PS, Stein JH. Transesophageal
echocardiography for the evaluation and management of
patients with cerebral ischemia. Clin Cardiol. 1999;22(6):
3916.

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143. de Bruijn SF, Agema WR, Lammers GJ, et al. Transesophageal

echocardiography is superior to transthoracic echocardiography in management of patients of any age with transient
ischemic attack or stroke. Stroke. 2006;37(10):25314.
144. Strandberg M, Marttila RJ, Helenius H, et al. Transoesophageal echocardiography in selecting patients for anticoagulation after ischaemic stroke or transient ischaemic
attack. J Neurol Neurosurg Psychiatr. 2002;73(1):2933.
145. Harloff A, Handke M, Reinhard M, et al. Therapeutic
strategies after examination by transesophageal echocardiography in 503 patients with ischemic stroke. Stroke.
2006;37(3):85964.
146. Touz E, Varenne O, Calvet D, et al. Coronary risk stratification in patients with ischemic stroke or transient ischemic
stroke attack. Int J Stroke. 2007;2(3):17783.
147. Hertzer NR, Young JR, Beven EG, et al. Coronary angiography
in 506 patients with extracranial cerebrovascular disease.
Arch Intern Med. 1985;145(5):84952.
148. Hoshino A, Nakamura T, Enomoto S, et al. Prevalence of
coronary artery disease in Japanese patients with cerebral
infarction: impact of metabolic syndrome and intracranial
large artery atherosclerosis. Circ J. 2008;72(3):4048.
149. Adams RJ, Chimowitz MI, Alpert JS, et al. American Heart
Association/American Stroke Association. Coronary risk
evaluation in patients with transient ischemic attack and
ischemic stroke: a scientific statement for healthcare
professionals from the Stroke Council and the Council on
Clinical Cardiology of the American Heart Association/
American Stroke Association. Stroke. 2003;34(9):231022.
150. Dhamoon MS, Sciacca RR, Rundek T, et al. Recurrent stroke
and cardiac risks after first ischemic stroke: the Northern
Manhattan Study. Neurology. 2006;66(5):6416.
151. Touz E, Varenne O, Chatellier G, et al. Risk of myocardial
infarction and vascular death after transient ischemic
attack and ischemic stroke: a systematic review and metaanalysis. Stroke. 2005;36(12):274855.
152. Sorrell VL, Nanda NC. Transesophageal echocardiographic
evaluation of coronary arteries for stenosis in the elderly
patient. Am J Geriatr Cardiol. 2001;10(1):3041, 49.
153. Samdarshi TE, Nanda NC, Gatewood RP Jr, et al. Usefulness
and limitations of transesophageal echocardiography in
the assessment of proximal coronary artery stenosis. J Am
Coll Cardiol. 1992;19(3):57280.
154. Yamagishi M, Miyatake K, Beppu S, et al. Assessment of
coronary blood flow by transesophageal two-dimensional
pulsed Doppler echocardiography. Am J Cardiol. 1988;62
(9):6414.
155. Ofili EO, Labovitz AJ, Kern MJ. Coronary flow velocity
dynamics in normal and diseased arteries. Am J Cardiol.
1993;71(14):3D9D.
156. Fusejima K. Noninvasive measurement of coronary artery
blood flow using combined two-dimensional and Doppler
echocardiography. J Am Coll Cardiol. 1987;10(5):102431.
157. Kisanuki A, Murayama T, Matsushita R, et al. Transesophageal Doppler echocardiographic assessment of left
coronary blood flow velocity in chronic aortic regurgitation. Am Heart J. 1996;131(1):1016.

1955

158. Bonninger, M. (a) Blut transfusion bei pernizioser anamie:

(b) Zwei Falle von Herz block. (34). 1908:2292.
159. Barasch E, Gottdiener JS, Larsen EK, et al. Clinical significance of calcification of the fibrous skeleton of the heart
and aortosclerosis in community dwelling elderly. The
Cardiovascular Health Study (CHS). Am Heart J. 2006;151
(1):3947.
160. Fox E, Harkins D, Taylor H, et al. Epidemiology of mitral
annular calcification and its predictive value for coronary
events in African Americans: the Jackson Cohort of the
Atherosclerotic Risk in Communities Study. Am Heart J.
2004;148(6):97984.
161. Osterberger LE, Goldstein S, Khaja F, et al. Functional
mitral stenosis in patients with massive mitral annular
calcification. Circulation. 1981;64(3):4726.
162. Assudani J, Singh B, Samar A , et al. Live/real time threedimensional transesophageal echocardiographic findings
in caseous mitral annular calcification. Echocardiography.
2010;27(9):114750.
163. Fox CS, Vasan RS, Parise H, et al. Framingham Heart
Study. Mitral annular calcification predicts cardiovascular
morbidity and mortality: the Framingham Heart Study.
Circulation. 2003;107(11):14926.
164. Potpara TS, Vasiljevic ZM, Vujisic-Tesic BD, et al. Mitral
annular calcification predicts cardiovascular morbidity
and mortality in middle-aged patients with atrial fibrillation: the Belgrade Atrial Fibrillation Study. Chest. 2011;
140(4):90210.
165. Kaczmarek RG, Silverman BG, Gross TP, et al. The
epidemiology of prosthetic heart valves in the United
States. Tex Heart Inst J. 1995;22:8691.
166. Singh P, Inamdar V, Hage FG, et al. Usefulness of live/real
time three-dimensional transthoracic echocardiography in
evaluation of prosthetic valve function. Echocardiography.
2009;26(10):123649.
167. Hage FG, Nanda NC; American Society of Echocardiographys Guidelines and Standards Committee; American
College of Cardiology; American Heart Association;
European Association of Echocardiography; Japanese
Society of Echocardiography; Canadian Society of Echocardiography. Guidelines for the evaluation of prosthetic
valves with echocardiography and Doppler ultrasound:
value and limitations. Echocardiography. 2010;27(1):913.
168. Zoghbi WA, Chambers JB, Dumesnil JG, et al.
American Society of Echocardiographys Guidelines
and Standards Committee; Task Force on Prosthetic
Valves; American College of Cardiology Cardiovascular
Imaging Committee; Cardiac Imaging Committee of
the American Heart Association; European Association
of Echocardiography; European Society of Cardiology;
Japanese Society of Echocardiography; Canadian Society
of Echocardiography; American College of Cardiology
Foundation; American Heart Association; European
Association of Echocardiography; European Society
of Cardiology; Japanese Society of Echocardiography;
Canadian Society of Echocardiography. Recommendations
for evaluation of prosthetic valves with echocardiography

1956

Section 7: Miscellaneous and Other Noninvasive Techniques

and doppler ultrasound: a report From the American

Society of Echocardiographys Guidelines and Standards
Committee and the Task Force on Prosthetic Valves,
developed in conjunction with the American College of
Cardiology Cardiovascular Imaging Committee, Cardiac
Imaging Committee of the American Heart Association,
the European Association of Echocardiography, a registered branch of the European Society of Cardiology, the
Japanese Society of Echocardiography and the Canadian
Society of Echocardiography, endorsed by the American
College of Cardiology Foundation, American Heart
Association, European Association of Echocardiography, a

registered branch of the European Society of Cardiology,

the Japanese Society of Echocardiography, and Canadian
Society of Echocardiography. J Am Soc Echocardiogr.
2009;22(9):9751014; quiz 1082.
169. Quere JP, Monin JL, Levy F, et al. Influence of preoperative
left ventricular contractile reserve on postoperative ejection
fraction in low-gradient aortic stenosis. Circulation. 2006;
113(14):173844.
170. Doufekias E, Segal AZ, Kizer JR. Cardiogenic and aortogenic
brain embolism. J Am Coll Cardiol. 2008;51(11):104959.
171. Nanda NC. Textbook of Color Doppler Echocardiography.
Philadelphia, PA: Lea and Febiger, Inc; 1989:17890.

CHAPTER 80
How to do Echo for the
Electrophysiologist
Chittur A Sivaram

Snapshot

Echocardiography in Supraventricular Tachycardia

Left Atrium

Atrial Septum

Pulmonary Veins

INTRODUCTION
The sub-subspecialty of cardiac electrophysiology (EP) has
made tremendous strides and remarkable progress in the
last three decades. Several major therapeutic advances in
EP techniques have resulted in new treatment options,
capable of providing favorable clinical outcomes in patients
with recurrent arrhythmias. Such innovative therapies
in EP include ablation treatment of atrioventricular (AV)
nodal reentrant tachycardia, accessory pathways, and atrial
fibrillation (AF) as well as device therapy for primary and
secondary prevention in ventricular tachyarrhythmias.
Echocardiography is frequently performed in
rhythm disorders for the initial assessment as well as
during follow-up. The appropriate use of transthoracic
echocardiogram (TTE), transesophageal echocardiogram
(TEE), and intracardiac echocardiography (ICE) is
an important responsibility of cardiologists involved
in the ordering, performance, and interpretation of
echocardiographic modalities.1 The following description
will attempt at delineating steps for maximizing the yield
of echocardiography in patients with rhythm disorders
through a focused approach to echo imaging relevant to
the EP diagnosis under consideration. This chapter will
be predominantly centered on the use of TTE and TEE.

Inferior Vena Cava

Echocardiography in Ventricular Tachycardia

Echocardiography in Cardiac Implantable Electronic

Devices

Discussion of ICE will not be included in this chapter since

ICE is by and large limited to the EP laboratory and performed
by the electrophysiologist during EP procedures. The reader
is also encouraged to cross-reference to other chapters in the
book with overlapping information.

ECHOCARDIOGRAPHY IN SUPRA
VENTRICULAR TACHYCARDIA
Often the typical patient with supraventricular tachycardia
(SVT) has a structurally normal heart and consequently,
a completely normal echocardiogram. This is particularly
true in AV nodal re-entrant tachycardia (AVNRT).
However, several aspects of TTE have special relevance
to the preablation assessment in SVT. The preablation
TTE should carefully document chamber dimensions
including left atrial (LA) size and volume. It will be helpful
to the electrophysiologist to have the dimensions of
coronary sinus (CS) to facilitate catheters placement in
the CS during ablation. Dimension of CS may be obtained
from the apical four-chamber view of TTE with posterior
angulation of the transducer or with TEE in the lower
midesophageal location.

1958

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 80.1:Parasternal long-axis view of transthoracic echocardiogram (TTE) showing dilated coronary sinus (CS) in the left
AV groove region (arrow). The descending thoracic aorta is seen
outside the pericardial layer while CS is intrapericardial. Dilated
CS can be the result of raised right heart pressures or persistent
superior vena cava (SVC) drainage into CS.

Fig. 80.2: Apical four-chamber view of transthoracic echocardiogram (TTE) with posterior angulation of transducer demonstrates
the dilated coronary sinus (CS).

CS imaging during TEE is done by advancing the TEE

probe slightly from the midesophageal four-chamber
view. This view will demonstrate the ostium of CS, CSRA
junction as well the terminal part of the CS. A slight further
advancement of the TEE probe can often reveal the middle
cardiac vein as it drains into the CS. Rarely, ablation of
accessory pathways in preexcitation is performed in the
middle cardiac vein and assessment of the middle cardiac
vein becomes highly relevant to the electrophysiologist.
Cannulation of CS is routinely performed in all patients
during cardiac ablation. As such, the dimension of CS is an
important piece of information to the electrophysiologist.
Normal size of the CS is approximately 6 mm.
Dilatation of the CS should raise the suspicion of
persistent left superior vena cava (PLSVC) drainage to
CS. The additional volume of blood drained from the
left upper limb through PLSVC causes an increase in CS
size. CS is easily imaged by TTE in the parasternal longaxis view as well as the apical four-chamber views with
posterior angulation of the probe (Figs 80.1 and 80.2).
Differentiation from descending thoracic aorta is aided by
the fact the CS is within the pericardium, while descending
aorta is extrapericardial. Saline contrast injection from
the left arm would confirm the diagnosis further since
contrast appears in the CS prior to appearance in the right
heart chambers (Fig. 80.3). The approach to EP procedures
and device implantation from left subclavian route is
significantly influenced by presence of PLSVC.

Two important anatomical landmarks in the CS

the Thebesian valve (located at the junction of CS and
right atrium) and the valve of Vieussens (located more
proximally within the CS)are visible during TEE
(Fig. 80.4). The valve of Vieussens frequently causes difficulty
in catheter advancement within CS,2 and as such might be
helpful information in the preablation assessment.
Imaging in patients with preexcitation requires
additional attention to features of Ebsteins anomaly due
to its association with posteroseptal accessory pathways.
Careful demonstration of attachment of the septal leaflet of
tricuspid valve relative to anterior mitral leaflet is indicated
in all patients with pre-excitation. An apparent apical
displacement of tricuspid valve leaflet insertion relative to
mitral leaflet insertion in excess of 8 mm/m2 Body surface
area (BSA) is consistent with Ebsteins anomaly (Figs 80.5
and 80.6). The posterior leaflet of the tricuspid valve needs
special attention and needs to be carefully looked at to
exclude Ebsteins anomaly; this can be done using TTE in
the parasternal right atriumright ventricle (RARV) view
with additional posterior angulation of the transducer or
using TEE in the transgastric long-axis RARV view.
A rare morphological association between preexcitation and diverticulae of CS has been described
(Fig. 80.7). While cardiac CT and contrast angiography of
CS are superior to echocardiography in the detection of
CS diverticulae, occasionally TTE or TEE might provide
clues toward the diagnosis in the preablation patient with
pre-excitation.

Chapter 80: How to do Echo for the Electrophysiologist

1959

Fig. 80.3:Apical four-chamber view of transthoracic echocardiogram (TTE). Saline contrast injection from the left arm shows
opacification of coronary sinus (CS) and right heart chambers. The
appearance of contrast in CS prior to right heart chambers is diagnostic of persistent left superior vena cava (PLSVC) to CS.

Fig. 80.4:Thebesian valve seen with transesophageal echo

cardiogram (TEE) at the junction of coronary sinus (CS) to right
atrium.

Fig. 80.5:Parasternal long-axis view transthoracic echocardiogram (TTE), showing dilated right heart chambers in a patient with
Ebsteins anomaly. The anterior right heart was composed of right
atrium (RA) with its atrialized portion of right ventricle (RV).

Fig. 80.6:Apical four-chamber view of the same patient as

before. Marked apparent apical displacement of the septal and
anterior leaflets of tricuspid valve is seen, resulting in a severely
dilated right atrium (RA); there is a large atrialized part of the right
ventricle (RV).

Scanning Tips

leaflets brought to focus by changing the degree of

posterior angulation of transducer.
3. Scan carefully to look for CS diverticulae, a rare
morphological association in pre-excitation.
4. In presence of dilated CS, perform left arm saline
contrast injection to exclude persistent left SVC
drainage to CS.

1. TTE is usually completely normal in SVT.

2. Look for evidence of Ebsteins anomaly; need to
demonstrate septal and posterior leaflet insertion to
the annulus; RARV view from parasternal window
should be used to delineate septal and posterior

1960

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 80.7:Coronary sinus contrast angiography showing pre

sence of a diverticulum in pre-excitation (photograph courtesy of
Dr Sunny Po, University of Oklahoma).

LEFT ATRIUM
In patients with arrhythmias, imaging of the left
atrium provides very valuable information to the
electrophysiologist, particularly if ablation therapies are
being considered. Dilatation of left atrium is frequently
seen in patients with atrial arrhythmias such as AF,
atrial flutter, and atrial tachycardia. Careful assessment
of LA size is critical. Traditionally, the anteroposterior
dimension of LA in systole measured using M-mode
echocardiography from the parasternal long-axis view of
TTE is the standard method for reporting LA size. However,
in many patients there is an obvious discordance between
LA size measured with M-mode and LA dimension in the
superior-inferior axis seen in the apical four-chamber
view of TTE. Recent studies have demonstrated a strong
correlation of both LA volume and LA volume index with
cardiovascular outcomes.3,4 Most of the commercially
available echocardiography machines have measurement
packages that permit calculation of LA volume using the
Simpsons biplane method during TTE. Care should be
taken to obtain stop frame images in systole that clearly
demonstrates the LA outlines in the apical four-chamber
and two-chamber views, as well as exclusion of LA
appendage and pulmonary veins (PVs) from the trace
contours.
In patients in atrial arrhythmias [AF, A flutter and
atrial tachycardias], a significant risk of thromboembolic
complications exists. Several findings on TEE correlated to
increased thromboembolic events have been described.

Presence of spontaneous echo contrast (SEC; smoke)

in left atrial appendage (LAA) has been well recognized
as a precursor for development of LAA thrombus and
embolism.4 SEC can be distinguished from high gain
artifact based on the swirling appearance seen only in
SEC. LAA thrombus is often present in patients with AF
and its confirmation is facilitated by demonstration of the
mass abnormality in orthogonal views. Prominent ridges
in the LAA (pectinate lines) should not be confused with
LAA thrombus.
Careful Doppler interrogation of LAA during TEE for
assessing emptying velocities is required as part of the
assessment of LAA function. A pulsed Doppler sample
volume should be placed close to the ostium of LAA.
Normal LAA Doppler signal is quadriphasic, with an
emptying and filling signal seen in mid-diastolic and late
diastole. The mid-diastolic signals frequently have small
velocity and might not be easily apparent. Normal LAA
emptying velocities are above 0.4 m/s. Reduced emptying
velocities are seen in patients with AF, atrial stunning, and
in low cardiac output states.
An under-recognized application of LAA Doppler is the
fact that it serves as a surrogate for atrial contraction and
atrial activity, and thus could help in analysis of rhythm. In
AF and A Flutter, LAA Doppler signal shows characteristic
emptying and filling signals at a rapid rate. The rate and
frequency (cycle length) of atrial emptying signal can be
measured from the LAA Doppler, thus providing clues to
rhythm analysis.
The nongeometric shape of LAA poses unique
challen ges to the echocardiographer in performing a
comprehensive scanning that evaluates the entire LAA for
thrombotic masses (Fig. 80.8). To overcome this challenge,
at least two orthogonal views of LAA are required to ensure
adequate visualization. This can be achieved by sequential
dialing up of the scan plane angle or by preset simultaneous
orthogonal views offered in some of the newer equipment
(Figs 80.9A and B).
Diagnostic accuracy of combined two-dimensional
(2D TTE) and three-dimensional TTE (3D TTE) when
compared with TEE in experienced hands has been
excellent. The differentiation of pectinate lines in LAA from
thrombus is also aided by 3D TTE (Figs 80.10A to D).5,6
LAA exclusion is recommended in conjunction
with mitral valve surgery as well as surgical ablation
of AF for maximal protection against thromboembolic
risk in both valvular and nonvalvular AF. Exclusion of
LAA by clips or suturing offers lesser protection against

Chapter 80: How to do Echo for the Electrophysiologist

1961

Fig. 80.8:Transesophageal echocardiogram (TEE) showing a

thrombus (arrow) in the left atrial appendage (LAA) in a patient with
atrial fibrillation.

Figs 80.9A and B: (A) Transesophageal echocardiogram (TEE) showing left atrial appendage (LAA; midesophageal probe position,
scan plane angle 54). Left upper pulmonary vein is seen adjacent to LAA, and it appears that a good demonstration of LAA has been
obtained; (B) With additional scan plane angle without any change in probe position, a large additional segment of LAA is now visualized. This underscores the importance of careful scanning of LAA with different scan plane angles to assess the complex shape of LAA.

thromboembolism compared to excision of LAA. Two

well-known complications after LAA exclusion are
recanalization of LAA and persistence of a LAA remnant.
The presence of LAA remnant is accompanied by a greater
risk of thromboembolism compared to LAA excision.7
Recanalized LAA often is associated with presence of an
emptying and a filling signal seen with color flow Doppler
as well as pulsed Doppler (Figs 80.11A and B).
Rarely other abnormalities of the LAA might be
present, for example, nonobstructive valves, masses such
as lipoma.

In patients with AF and A Flutter, worsening of

pre-existing SEC might be seen immediately after
cardioversion. This is caused by atrial stunning, which
results in a reduced atrial transport function even after
sinus rhythm has been re-established. Atrial stunning has
been known to persist for several weeks postcardioversion.

Scanning Tips
1. Obtain M-mode measurements of LA size.
2. Obtain LA volumes from apical four- and twochamber views.

1962

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 80.10A to D:(A) Two-dimensional transesophageal echocardiogram. Arrowhead points to an echodense mass within the left atrial appendage (LAA) consistent with a thrombus; (B to D) Three-dimensional transthoracic echocardiogram. Within the LAA there are two echo densities noted. Sequential cropping shows both to be parts of pectinate muscles, which traverse
the LAA. The upper echo density (upper arrowhead) is larger because it represents a short-axis cut through two pectinate muscles virtually
in contact with each other. This most likely represents the thrombus seen on the transesophageal echocardiogram. The second
echo density (bottom arrowhead) is smaller because only one pectinate muscle is involved. (LUPV: Left upper pulmonary vein).
Source: Reproduced with permission from Karakus G, Kodali V, Inamdar V, Nanda NC, Suwanjutah T, Pothineni KR. Comparative
assessment of left atrial appendage by transesophageal and combined two and three-dimensional transthoracic echocardiography.
Echocardiography. 2008;25:91824.

3. Scan LAA in multiple views to demonstrate all areas of

the nongeometric LAA.
4. Emptying velocity signal of LAA is a surrogate for atrial
activity.
5. Adjust gain appropriately to optimal level for
demonstration of SEC.

ATRIAL SEPTUM
Abnormalities of atrial septum are occasionally seen in
patients undergoing ablation. Preprocedural assessment

of the atrial septum provides important information for

procedures requiring trans-septal puncture. The fossa
ovalis region is the thinnest part of the atrial septum. The
septum secundum is slightly thicker and is seen to the
right of the thinner septum primum. Presence of a patent
foramen ovale is confirmed by demonstration of a space
between the septum primum and secundum along the
superior aspect and presence of flow between the two
layers of atrial septum using color flow Doppler and saline
contrast study.

Chapter 80: How to do Echo for the Electrophysiologist

1963

Figs 80.11A and B: (A) Transesophageal echocardiogram (TEE) showing a recanalized left atrial appendage (LAA) after surgical
exclusion; (B) Peak velocity by continuous wave (CW) Doppler was 3.4 m/s with a peak gradient of 46 mm Hg across the recanalized
LAA.

Procedures
requiring
trans-septal
puncture
(e.g. PV isolation for AF) often produce a left-to-right shunt
at the fossa ovalis region immediately postprocedure.
These shunts are invariably small and the defects close
spontaneously over time. ICE guidance is used in most
ablation laboratories for trans-septal puncture. If the
puncture enters the upper part of atrial septum, significant
hematoma might result due to entry into the Waterstons
groove (an extracardiac space between the two atria due to
in-folding of the atrial walls).
A frequent abnormality of the atrial septum in patients
undergoing AF ablation is lipomatous atrial septum.
Significant thickening of the atrial septum can occur due
to deposition of fat and this might be mistaken for an atrial
tumor. Typically lipomatous atrial septum spares the fossa
ovalis region. Lipomatous atrial septum is more often seen
in patients with obesity.

Scanning Tip
Immediately after AF ablation, there is often a left-toright shunt at the site of trans-septal puncture.

PULMONARY VEINS
The critical role played by firing from PV in the genesis of AF
has now conclusively been recognized. This mechanism is
the basis for ablative therapies in AF (PV isolation). Many
patients undergoing PV isolation for AF require additional
procedures including additional ablation for AF, A Flutter,

and macro-reentrant tachycardia. Thus, the importance

of morphological features of PV for the ablation planning
has been recognized in such patients undergoing preand postablation assessment. TTE has limited ability to
demonstrate PV anatomy. Occasionally, one might be
able to see the ostia of right and left upper PV in the apical
four-chamber view of TTE. Rarely the left inferior PV might
be seen in the parasternal long-axis view. Other imaging
techniques are therefore needed for PV imaging. While
CT angiography and cardiac MR imaging have been well
recognized for their ability in delineating PV anatomy,
the role of TEE has been less well recognized. Experience
from centers with high volume of TEE have shown that
all four PVs can be imaged in an overwhelming number
of patients; ostial size, Doppler velocity profile, and other
abnormalities can be easily demonstrated.8 A systematic
review of published studies shows a diagnostic accuracy
rate of TEE for PV abnormalities comparable to CT
angiography and cardiac MR imaging.9
PV imaging during TEE is generally performed at the
midesophageal level. Left upper PV is adjacent to LAA,
while right upper PV is adjacent to SVC. Left lower PV is
anatomically close to the descending thoracic aorta. These
adjacent anatomical landmarks are helpful in locating
PVs. Each PV ostium has an oval shape with a larger and
a smaller orthogonal dimension. From the midesophageal
probe position during TEE, progressive scan plane
angulation combined with counterclockwise torque of the
scope will bring up both left upper and lower PVs in a single

1964

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 80.12: Transesophageal echocardiogram (TEE) from midesophageal level, employing 120 scan plane angle as well as torque
shows left upper and lower pulmonary veins (PVs).

Fig. 80.13: Transesophageal echocardiogram (TEE) from midesophageal level showing right upper, middle, and lower pulmonary
veins (PVs). Right upper PV is adjacent to superior vena cava.

view (Fig. 80.12). Right upper PV can be demonstrated in

the caval view by additional dialing of scan plane angle
or clockwise torque of the scope. Right lower PV can be
imaged by slight additional downward movement of the
TEE probe (Fig. 80.13). The ostial dimensions of both lower
PVs are smaller compared to upper PVs. The lower PVs are
more difficult to image by and large.
PV flow should be imaged using pulsed Doppler with
the sample volume placed about 1 cm from the ostial site.
Occasionally, the localization of PV ostium can be difficult
due to a degree of flaring of the PV as they enter LA with
the resultant absence of a distinct PVLA junction. The
typical PV flow pattern consists of a systolic forward flow,
a diastolic forward flow, and atrial reversal. These patterns
are influenced by diastolic dysfunction, LA pressures, and
presence of significant mitral regurgitation (MR). It will
be essential to have careful assessment of PV velocities
preablation for accurate assessment of PV stenosis
development. PV stenosis has become a rare complication
of ablation therapy for AF since focal ablation within PV
is not currently used for AF ablation, but PV isolation by
ablation within LA is employed. However, PV stenosis
still does occur after AF ablation and it can be readily
diagnosed by TEE through assessment of PV dimensions
post procedure as well as increased PV velocities
(Figs 80.14 and 80.15). Often color flow Doppler shows
characteristic focal aliasing within PVs; sampling pulsed
Doppler at the point of aliasing reveals increased velocity
(usually >1 m/s) and loss of the typical spectral pattern

of low velocity flow signals. Localized stenosis involving

only one PV is frequently asymptomatic while stenosis
of all four PVs will lead to development of symptomatic
pulmonary hypertension.10 The presence of high velocities
in PVs immediately after ablative therapies is explained by
edema caused by the procedure; this frequently resolves
over time. Persistent elevation of PV velocities would
be consistent with development of actual scarring and
stenosis of PV.

INFERIOR VENA CAVA

Postablation complications involving inferior vena cava
(IVC) have been described. Routine scanning of IVC is
indicated pre- and postablation. IVC can be easily visualized by TTE from the subcostal window. More angulation
to bring right-sided structures in view will often demonstrate long segments of the IVC easily. Size and inspiratory
collapse of IVC allow prediction of right heart filling
pressures. IVC can also be demonstrated using TEE; this
requires advancing the TEE probe from the midesophageal
four-chamber view to close to the transgastric level. With
clockwise torque of the scope, the IVCRA junction is
brought into view. With additional advancement of probe
and dialing about 20 to 40 of scan plane angle, both the
IVC and hepatic vein are demonstrated. Ablation might
be complicated by development of mobile masses in
the IVC, close to the IVCRA junction (Fig. 80.16), very
likely representing the development of thrombi from the

Chapter 80: How to do Echo for the Electrophysiologist

1965

Figs 80.14A and B: (A) Stenosis of the right middle pulmonary vein (PV) after atrial tachycardia ablation. Significant aliasing of color
flow is seen at the ostial site of right middle PV; (B) Pulsed Doppler sampling from right middle PV shows marked increase in velocities
at the site of aliasing (peak diastolic velocity 1.3 m/s).

Figs 80.15A and B: (A) Transesophageal echocardiogram (TEE) showing focal aliasing of the left upper pulmonary vein (PV) ostium
after atrial fibrillation (AF) ablation; (B) Pulsed Doppler from the left upper PV shows a peak velocity of approximately 1.5 m/s in diastole
consistent with PV stenosis.

Fig. 80.16:Transthoracic echocardiogram (TTE) subcostal view

showing a floating thrombus in the inferior vena cava (IVC) after an
ablative procedure. Patient did not manifest pulmonary embolism.

1966

Section 7: Miscellaneous and Other Noninvasive Techniques

procedure. Clinically significant pulmonary embolism

is infrequent in these patients. No correlation between
number of sheaths used and duration of procedure have
been demonstrated in patients developing IVC thrombi
after ablation.

ECHOCARDIOGRAPHY IN
VENTRICULAR TACHYCARDIA
Echo imaging with TTE provides highly meaningful
information in patients with ventricular tachyarrhythmias.
As such, imaging with specific diagnostic possibilities in
mind is called for in a patient with ventricular ectopy. Most
patients with ventricular arrhythmias have an anatomical
substrate (i.e. underlying structural heart disease).
Common substrates of ventricular arrhythmias
include:11
Coronary artery disease with scar from prior myoc
ardial infarction

Dilated cardiomyopathy
a. Noncompaction
Hypertrophic cardiomyopathy
Infiltrative diseases

a. Sarcoid heart disease
Arrhythmogenic RV dysplasia (ARVD)
Since ventricular tachycardia (VT) ablations require
placement of catheters in left ventricle (LV), the presence
of LV apical thrombi should be evaluated. Off-axis
scanning of the LV apex and the use of Definity contrast
are very helpful techniques for the demonstration of LV
apical thrombi. Some EP laboratories are also interested
in obtaining information about the degree of aortic arch
atherosclerotic burden since ablation of VT requires
manipulation of catheters across the arch of aorta.
The Table 80.1 lists the common conditions associated
with ventricular ectopy and the potential findings that
should be carefully looked at during echo imaging.

Table 80.1: Common Conditions Associated with Ventricular Arrhythmias and Associated Echocardiographic Findings

Disease

Characteristic Findings

Coronary artery disease

Increased LV dimensions
Reduced fractional shortening
Reduced left ventricular ejection fraction
(LVEF; Simpsons biplane)
Localized scar (thinning, abnormal wall motion)
LV mural thrombi

Dilated cardiomyopathy

Increased LV dimension
Reduced fractional shortening
Reduced LVEF (Simpsons biplane)
Diffuse hypokinesis
LV mural thrombi

Noncompaction12

Increased LV trabeculations
Ratio of noncompacted to compacted segments > 2

Hypertrophic cardiomyopathy

Small LV cavity
Hyperdynamic LV function
Asymmetrical septal hypertrophy
Systolic anterior motion of mitral valve
Mitral regurgitation
Aortic valve midsystolic closure
LV outflow gradient (at rest and/or during provocation, e.g.
Valsalva maneuver)

Sarcoid heart disease

Arrhythmogenic RV dysplasia13

Localized basal septal scars

Contd...
Increased right ventricular outflow tract (RVOT) dimensions
( 32 mm in the parasternal long-axis view, 36 mm in the basal
short-axis view)
Increased echogenicity of moderator band
Prominent branching pattern
Focal outpouching of RV free wall

Chapter 80: How to do Echo for the Electrophysiologist

1967

Contd...
Disease

Characteristic Findings

Sarcoid heart disease

Localized basal septal scars

Arrhythmogenic RV dysplasia

13

Increased right ventricular outflow tract (RVOT) dimensions

( 32 mm in the parasternal long-axis view, 36 mm in the basal
short-axis view)
Increased echogenicity of moderator band
Prominent branching pattern
Focal outpouching of RV free wall

The presence of fibromuscular bands in LV has

been reported in patients with idiopathic LV ventricular
tachycardia (Belhassen VT). Fibromuscular bands in
this disease typically run across the LV cavity from the
posterolateral free wall to the basal part of the septum.14

ECHOCARDIOGRAPHY IN CARDIAC
IMPLANTABLE ELECTRONIC DEVICES
Cardiac implantable electronic devices (CIEDs) are indi
cated in a broad array of conditions including symptomatic
bradycardia (pacing), ventricular arrhy
thmias and
sudden cardiac arrest (defibrillators for primary and
secondary prevention), and severe heart failure [cardiac
resynchronization therapy (CRT)]. Echocardiography
provides significant information in patients undergoing
evaluation for CIEDs as well as when infection and
bacteremia complicate CIED therapy.
In the preprocedural selection of CIEDs, the task
for the echocardiographer is to demonstrate the major
features of the underlying structural heart disease and
to provide relevant quantitative measurements dictated
by the guidelines applicable in CIED therapy. Patients in
need of CIED therapy for primary or secondary prevention
of VT have underlying structural heart disease (coronary
artery disease, hypertrophic cardiomyopathy, or dilated
cardiomyopathy) and reduced ejection fraction (EF).
Thus, the preprocedural assessment should include
careful estimation of EF by Simpsons method. Selection of
patients for CRT is based on symptom level of heart failure
and QRS duration. The role of echocardiography in patient
selection for CRT is highly controversial and not supported
by strong evidence.
All patients with CIED infections require echo
cardiography to rule out infective endocarditis.15 This
is due to the fact that infection in CIED patients may be
restricted to the device pocket in which case the duration
of antibiotic therapy is shorter. Thus, TEE is required

in patients with CIED and bacteremia prior to device

explantation. The presence of ghostscasts comprising
inflammatory masses over the leads persisting after lead
extractionhas been demonstrated to be associated with
worse prognosis in CIED infections.16
Lead extraction is required in CIED infections and one
of the complications of this procedure is hemopericardium
and cardiac tamponade.
In summary, in patients undergoing EP procedures,
a unique set of knowledge and skills is required for us to
provide the relevant information required by the EP team.
Close collaboration between the EP and echocardiography
teams along with a process of continuing learning through
clinical correlation is required.

REFERENCES
1. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/
AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011.
Appropriate Use Criteria for Echocardiography. J Am Coll
Cardiol. 2011;57(9):112666.
2. Corcoran SJ, Lawrence C, McGuire MA. The valve of
Vieussens: an important cause of difficulty in advancing
catheters into the cardiac veins. J Cardiovasc Electrophysiol.
1999;10(6):8048.
3. Abhayaratna WP, Seward JB, Appleton CP, et al. Left atrial
size: physiologic determinants and clinical applications.
J Am Coll Cardiol. 2006;47(12):235763.
4. Gabriel RS, Klein AL. Managing catheter ablation for
atrial fibrillation: the role of echocardiography. Europace.
2008;10 Suppl 3:iii813.
5. Karakus G, Kodali V, Inamdar V, et al. Comparative
assessment of left atrial appendage by transesophageal
and combined two- and three-dimensional transthoracic
echocardiography. Echocardiography. 2008;25(8):91824.
6. Kumar V, Nanda NC. Is it time to move on from twodimensional transesophageal to three-dimensional
transthoracic echocardiography for assessment of left atrial
appendage? Review of existing literature. Echocardiography.
2012;29(1):11216.

1968

Section 7: Miscellaneous and Other Noninvasive Techniques

7. Kanderian AS, Gillinov AM, Pettersson GB, et al. Success

of surgical left atrial appendage closure: assessment by
transesophageal echocardiography. J Am Coll Cardiol.
2008;52(11):9249.
8. Stavrakis S, Madden G, Pokharel D, et al. Transesophageal
echocardiographic assessment of pulmonary veins and left
atrium in patients undergoing atrial fibrillation ablation.
Echocardiography. 2011;28(7):77581.
9. Stavrakis S, Madden GW, Stoner JA, et al. Transesophageal
echocardiography for the diagnosis of pulmonary vein
stenosis after catheter ablation of atrial fibrillation: a
systematic review. Echocardiography. 2010; 27(9):11416.
10. Saad EB, Marrouche NF, Saad CP, et al. Pulmonary vein
stenosis after catheter ablation of atrial fibrillation:
emergence of a new clinical syndrome. Ann Intern Med.
2003;138(8):6348.
11. Stevenson WG, Soejima K. Catheter ablation for ventricular
tachycardia. Circulation. 2007;115(21):275060.

12. Stanton C, Bruce C, Connolly H, et al. Isolated left
ventricular noncompaction syndrome. Am J Cardiol. 2009;
104(8):11358.

13. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis
of arrhythmogenic right ventricular cardiomyopathy/
dysplasia: proposed modification of the task force criteria.
Circulation. 2010;121(13):153341.
14. Thakur RK, Klein GJ, Sivaram CA, et al. Anatomic substrate
for idiopathic left ventricular tachycardia. Circulation.
1996;93(3):497501.
15. Sohail MR, Uslan DZ, Khan AH, et al. Management and
outcome of permanent pacemaker and implantable
cardioverter-defibrillator infections. J Am Coll Cardiol.
2007;49(18):18519.
16. Le Dolley Y, Thuny F, Mancini J, et al. Diagnosis of cardiac
device-related infective endocarditis after device removal.
JACC Cardiovasc Imaging. 2010;3(7):67381.

CHAPTER 81
Echocardiography in
Life-Threatening Conditions
Rachel Harris, Elizabeth Ofili

Snapshot

Chest Trauma

Blunt Chest Trauma

Penetrang Chest Trauma

Acute Mitral Regurgitaon

Acute Severe Aorc Regurgitaon

Aorc Dissecon

INTRODUCTION
The uses of two-dimensional transthoracic and transesophageal echocardiography have proven vital in risk
stratification of patients who present with life-threatening
conditions.1 In evaluating patients with hemodynamic
instability, new or worsening heart murmur, or a recent
history of blunt or penetrating trauma, echocardiography
has become the mainstay in identifying those with
critical injuries in need of surgical or immediate invasive
intervention. Transthoracic echocardiography is both
sensitive and specific for identification of pericardial
effusion, pericardial injury, and peritoneal fluid in the
setting of anterior chest trauma.2 The advantages of
transthoracic echo are its bedside availability, noninvasive
characteristics, and ability to provide rapid information
regarding cardiac structure and function. Transesophageal
echo has greater sensitivity and specificity in evaluating
valvular and aortic pathology, prosthetic valves, interatrial
shunts, and cardiac sources of emboli but is more invasive
(Table 81.1).3

Debakey Classificaon

The Stanford Classificaon

Pulmonary Thromboembolic Disease

Air Embolism

Hypovolemia

Large Intracardiac Thrombus

CHEST TRAUMA
In 2010, among US persons aged 144, unintentional injury
was the number one cause of death.5 Cardiac injuries
are among the most lethal of thoracic trauma patients,
especially in penetrating injuries.6,7 In this same age
group, the majority of nonfatal hospital injury visits were
secondary to blunt force trauma.8 Although the majority of
these patients die in the field from their injuries, among
the survivors who present to the emergency department,
meticulous detail and a high index of suspicion must be
held for the hemodynamically stable patient who may
rapidly deteriorate post presentation.

BLUNT CHEST TRAUMA

This type of injury is more commonly the direct result of
assault, motor vehicle collisions, falls, or fallen objects
more commonly. There are numerous injuries that should
be suspected depending on the mode of injury. In the case
of blunt objects whose force may decrease the chest walls

1970

Section 7: Miscellaneous and Other Noninvasive Techniques

Table 81.1: Perioperative Indications for use of Transesophageal Echocardiogram in Trauma Patients

Category 1

Acute hemodynamic instability in which ventricular function and its determinants are uncertain and have
not responded to treatment
Unstable patient with unexplained hemodynamic disturbances, suspected valvular pathology, or thromboembolism
Immediate evaluation of patient with suspected thoracic aortic pathology: aneurysm, dissection, or disruption
Pericardial window procedures

Category 2

At risk for myocardial ischemia or infarction or hemodynamic disturbances

Detection of air embolism or foreign body
Suspected cardiac trauma
Evaluation of pericardial effusion

Category 3

Evaluation of thoracic trauma in patient with low-suspicion of injury

Intraoperative assessment of repair of thoracic aortic injury

Category 1: Supported by the strongest evidence or expert opinion

Category 2: Supported by weaker evidence and expert consensus
Category 3: Little current scientific or expert support
Source: Adapted from the 1999 ASE/SCA guidelines for performing a comprehensive intraoperative multiplane transesophageal
echocardiography examination: recommendations of the American Society of Echocardiography Council for Intraoperative
Echocardiography and the Society of Cardiovascular Anesthesiologists Task Force for Certification in Perioperative Transesophageal
Echocardiography.3,4

anteroposterior diameter, puncture injuries from ribs

may result. Moreover, right atrial and ventricular free wall
rupture, massive hemothorax, and cardiac tamponade
have also been documented.9

Cardiac Tamponade
Echocardiography results in the diagnosis of cardiac
tamponade as well as assists in rapid management and
treatment, that is, echocardiographically guided pericardiocentesis with catheter drainage.10 Patients presenting
with hemodynamic instability post penetrating or blunt
force chest trauma should be emergently evaluated for
cardiac involvement, namely, myocardial contusion or
pericardial effusion.11,12
The clinical presentation of diaphoresis, dyspnea,
muffled heart sounds, and hypotension should warrant
further evaluation for pericardial effusion.11,13,14 Careful
evaluation using parasternal long- and short-axis,
apical four-chamber, and subcostal windows should be
undertaken as well as the use of M-mode.15 Evaluation of
diastolic right ventricular collapse can be demonstrated
as well as right atrial collapse/inversion. Diastolic right
ventricular collapse is more specific but less sensitive than
right atrial diastolic collapse.
While most cases of cardiac tamponade are immediate,
there are rare reported cases of delayed tamponade up to
70 days post injury (Figs 81.1 to 81.4).16

Fig. 81.1: Parasternal long-axis with large circumferential

pericardial effusion (PE). (AO: Aorta; DA: Descending aorta;
IVS: Interventricular septum; LA: Left atrium; PW: Posterior wall)
(Movie clip 81.1).

With Doppler, there may be evidence of exaggerated

respiratory variation in inflow velocity. The transtricuspid
and transmitral inflow velocities should not vary by more
than 25% or 15%, respectively (Fig. 81.5).10
When evaluating the pulmonary and aortic inflow
velocities, these should not vary by >10%.10 There may
also be evidence of phasic variation in the right and
left ventricular outflow tract (LVOT) and exaggerated

Chapter 81: Echocardiography in Life-Threatening Conditions

1971

Fig. 81.2: Subcostal view of anterior pericardial fluid. Arrowhead

points to diastolic right ventricular collapse. (L: Liver; LV: Left ventricle; PE: Pericardial effusion) (Movie clip 81.2).

Fig. 81.3: Parasternal short-axis of right ventricular (RV) diastolic

collapse (arrowhead) and pericardial effusion (PE). (LV: Left ventricle) (Movie clip 81.3).

Fig. 81.4: M-mode demonstrating right ventricular wall (RVW)

diastolic collapse (arrow). (CW: Chest wall).

Fig. 81.5: Tricuspid valve (TV) respiratory flow variation. (LV: Left
ventricle; RV: Right ventricle).

respiratory variation in inferior vena cava (IVC) flow.17 The

operator will also likely see a plethoric (distended) IVC (in
the absence of hypovolemia).

biochemical cardiac markers.18 Cardiac contusion can

occur as a direct result of pressure on the myocardium
caused by deceleration forces that affect the chest wall
or indirectly secondary to shear stresses and increased
intrathoracic pressures.1921 Noninvasive attempts to
characterize cardiac contusion have aimed at evaluating
the combination of ECG, cardiac enzymes, use of telemetry,
and echocardiogram (notably transthoracic echo). ECG
changes may be associated with ST depression, but

Cardiac Contusion
The definition of cardiac contusion is a histological
diagnosis. However, the clinical diagnosis relies on the
sum of echo findings, electrocardiogram (ECG), and

1972

Section 7: Miscellaneous and Other Noninvasive Techniques

these findings are not specific for myocardial ischemia.

However, should a patient have ECG changes, they should
be monitored at least 24 hours for arrhythmias.20,21
Both CPK Mb and troponin levels have been evaluated
in terms of their sensitivity and specificity, and troponin is
more specific. Post trauma, the CPK Mb has been shown to
be elevated in noncardiac injuries.20,21
Using bedside transthoracic echocardiogram (TTE),
the operator can examine for possible focal wall motion
abnormalities, valvular involvement, focal wall rupture,
pericardial effusion, or great vessel injury (Figs 81.6A and B).

PENETRATING CHEST TRAUMA

Penetrating chest injury may be caused by a knife, gun, rifle,
or any sharp or impaling object through the skin. The path of
entry to exit should be closely examined with consideration of
possible cardiac structures and/or vessels involved. Wounds
to the inferolateral left parasternal regions call for close
evaluation of the LV, whereas lower right and left parasternal
wounds call for right ventricular evaluation.22 Involvement
of the interventricular septum is also common as well as
free wall rupture.47 Doppler evaluation of suspected areas
of involvement should also be performed.23 If intracardiac
missiles are visualized, they may distally embolize and their
location should be reported.24
In the setting of chest wall emphysema or hemothorax,
the sensitivity of transthoracic echo is decreased and
transesophageal echocardiogram (TEE) should be considered if the patient remains clinically stable and passage
of the TEE probe can be safely conducted (Figs 81.7A to D).

ACUTE MITRAL REGURGITATION

Postpenetrating or blunt injury, myocardial infarction
or endocarditis, acute mitral regurgitation (MR) from
possible valve, chord or papillary muscle involvement
must be considered. Careful evaluation of the plane of
the penetrating injury should be performed. Standard
parasternal long-axis, parasternal short-axis, apical fourand two-chamber as well as subcostal images should be
obtained during transthoracic echo. For transesophageal
echocardiography, transgastric short- and long-axis and
midesophageal four- and two-chamber, and long-axis
views are optimal. M-mode assessment can assist in further
evaluation of the possible mechanism of the regurgitation
as well as give useful information regarding volume status
(i.e. M-mode evidence of B-bump on the AC shoulder in
increased LV end-diastolic pressure states). If possible,
a determination of the mechanism (i.e. perforation of
valve leaflet, pap muscle dysfunction vs. rupture, chordal
involvement), severity and repairability of the MR should
be performed. This may involve transesophageal echo
evaluation if the patient remains hemodynamically stable
(Figs 81.8 to 81.10 and Table 81.2).

ACUTE SEVERE AORTIC

REGURGITATION
Acute aortic regurgitation can occur commonly with
trauma, aortic dissection, and infective endocarditis.
Close attention must be paid to several clinical factors in
considering a patient with acute aortic regurgitation. The

Figs 81.6A and B: Parasternal short-axis view (apical level) in end diastole (A) and end-systole (B). Note the focal inferior wall akinesis
(arrows) and small pericardial effusion (PE) in a patient post trauma. (LV: Left ventricle) (Movie clip 81.6A and B).

Chapter 81: Echocardiography in Life-Threatening Conditions

1973

Table 81.2: Classification of Severe Mitral Regurgitation

Color Doppler jet area

Vena contracta width > 0.7 cm with large central mitral regurgitation (MR) jet [area > 40% of
left atrium (LA) area] or with a wall-impinging jet of any size, swirling in LA

Doppler vena contracta width (cm)

0.70

Regurgitant volume (mL/beat)

60

Regurgitant fraction (%)

50

Regurgitant orifice area (cm2)

0.40

Source: Adapted from Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 Practice Guidelines for the Management of
Patients With Valvular Heart Disease: Executive Summary. J Am Coll Cardiol. 2006;48(3):598675.25

Figs 81.7A to D: Short-axis at the level of the mitral valve showing a small ventricular septal defect (VSD) without (A) and with (B)
color (arrow). Three months later, the VSD was noted to be significantly larger, measuring 1.5 cm in diameter shown without (C) and
with (D) color (arrows). (LV: Left ventricle; RV: Right ventricle). Courtesy of Dr Robert Chisholm, St Michaels Hospital, Toronto, ON, with
permission.47

1974

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 81.8: Color Doppler evidence of severe mitral regurgitation

(MR). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery)
(Movie clip 81.8).

Fig. 81.9: Prolapse of P3 scallop (arrow in Movie clip 81.9A).

(LA: Left atrium; LV: Left ventricle; MR: Mitral regurgitation) (Movie
clips 81.9A and 81.9B).

M-mode is also useful in assessing for premature closure

of the mitral valve (prior to QRS onset) and diastolic
fluttering of the anterior mitral valve leaflet (specific but
not sensitive).25 Doppler may also reveal evidence of
diastolic MR (Figs 81.11 to 81.14 and Table 81.3).

AORTIC DISSECTION
TTE and TEE are both are indicated (Class I) in diagnostic
imaging of suspected aortic dissection.26 The type and
extent of the dissection should be determined.

DEBAKEY CLASSIFICATION
Fig. 81.10: Doppler evidence of severe mitral regurgitation (arrow;
Vmax, 4.67 m/s). (LA: Left atrium; LV: Left ventricle).

pulse pressure in acute regurgitation may be normal to only

mildly increased, given rapid equalization of pressures
versus in a chronic, compensated state [LV dilatation,
eccentric left ventricular hypertrophy (LVH), and a large
stroke volume] the pulse pressure can be very wide. The LV
and left atrial dimensions should be assessed with normal
values indicating an acute decompensation. The standard
transthoracic views to assess regurgitation severity are
the apical three- and five-chamber, suprasternal notch,
and right parasternal windows. For assessment of the
LVOT diameter, the parasternal long-axis view is optimal.

Type I: Originates in ascending aorta, propagates at least

to the aortic arch, and often beyond it distally.
Type II: Originates in and is confined to the ascending
aorta.
Type III: Originates in descending aorta, rarely extends
proximally but will extend distally.

THE STANFORD CLASSIFICATION

Type A: All dissections involving the ascending aorta,
regardless of the site of origin (DeBakey types I
and II).
Type B: All dissection not involving the ascending aorta
(DeBakey type III; Table 81.4).
The diagnosis of aortic dissection is visualization
of two wall lumina separated by an intimal flap

Chapter 81: Echocardiography in Life-Threatening Conditions

1975

Table 81.3: Classification of Severe Aortic Regurgitation

Color Doppler jet width

Central jet, width > 65% left ventricular outflow tract (LVOT)

Doppler vena contracta width (cm)

Regurgitant volume (mL/beat)
Regurgitant fraction (%)

> 0.60
60
50

Regurgitant orifice area (cm2)

0.30

Pressure Half-time (ms)

< 200

Color M-mode propagation velocity (cm/sec)

80

Source: Adapted from Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 Practice Guidelines for the Management of
Patients With Valvular Heart Disease: Executive Summary. J Am Coll Cardiol. 2006;48(3):598675.25

Fig. 81.11: Aortic valve rupture. Long-axis view reveals flail right
coronary cusp (arrow) resulting in severe acute aortic regurgitation. (AO: Aorta; LA: Left atrium; LV: Left ventricle) (Movie clip
81.11).

Fig. 81.12: Color Doppler examination showing severe aortic

regurgitation (AR). (AO: Aorta; LA: Left atrium; RV: Right ventricle)
(Movie clip 81.12).

Fig. 81.13: M-mode of mitral valve (MV) revealing early diastolic

closure (arrow) due to severe aortic regurgitation. (AO: Aorta; LA:
Left atrium; LV: Left ventricle).

Fig. 81.14: Color Doppler superimposed on M-mode tracing.

Evidence of pan-diastolic regurgitation (arrow). (LVO: Left ventricle
outflow).

1976

Section 7: Miscellaneous and Other Noninvasive Techniques

Table 81.4: Standard Two-Dimensional Transthoracic Views

in Evaluating Aortic Dissection2729

Left Parasternal

Ascending aorta

Apical

Ascending aorta

Subcostal

Ascending aorta

Right parasternal

Ascending aorta

Suprasternal Notch

Aortic arch

Paraspinal

Descending aorta

causes difficulty in visualizing small dissection areas in the

distal ascending aorta and anterior aortic arch that may be
encountered if not using a multiplane transesophageal
probe.31,32

PULMONARY THROMBOEMBOLIC
DISEASE

(Figs 81.15A and B).30 Evidence of tears may lead to

propagation of the dissection either proximally or distally.
Characteristics of the true lumen are systolic expansion
and diastolic collapse, the absence or low intensity of
spontaneous echocardiographic contrast, systolic jets
directed away from the lumen, and systolic forward flow.
Characteristics of the false lumen are diastolic diameter
increase, spontaneous echocardiographic contrast,
and reversed, delayed, or absent flow and thrombus
formation.27,29,30 Flow signals within the false lumen
represent signs of communication (Figs 81.16 and 81.17).
There are a few special considerations when performing
transesophageal imaging. The number of tears should be
documented as well as the location of each (distance from
teeth to tip of probe).26 Moreover, there is a blind zone
caused by overlap of the trachea and the left main stem
bronchus between the esophagus and the aorta, which

Right ventricular dysfunction and dilatation may be evident

in massive or submassive pulmonary thrombo-embolic
(PTE) disease. Not all patients presenting with PTE need
urgent echocardiographic evaluation but it may be useful in
determining prognosis as well as the need for thrombolysis
(Fig. 81.18). Mortality rates for acute PTE can exceed
15% in the first 3 months post diagnosis.3335 A common
specific finding on echo is evidence of pressure overload,
(Figs 81.19A to D) described by Nazeyrollas et al. as right
ventricle/left ventricle End-diastolic dimension (RV/LVEDD)
> 0.5 (parasternal M-mode echo) and tricuspid insufficiency
(TI) with a jet velocity > 2.5 m/s.36 Furthermore, a
RV/LVEDD ratio of 0.9 or greater (left parasternal long-axis
or subcostal view) is an independent predictor of hospital
mortality.37 The findings of akinesia of the mid right
ventricular free wall but normal motion of the apex are
referred to as the McConnells Sign.38 This phenomenon
has a reported 77% sensitivity and a 94% specificity for the
diagnosis of acute pulmonary thromboembolism in the
setting of right ventricular dysfunction.36,38

Two-dimensional echo Doppler and M-mode standard views

reveal key portions of the aorta

Figs 81.15A and B: Descending aortic dissection with intimal flap (arrows). (FL: False lumen; TL: True lumen). Courtesy of Dr Anekwe
Onwuanyi, Morehouse School of Medicine, Atlanta, GA (Movie clip 81.15).

Chapter 81: Echocardiography in Life-Threatening Conditions

Fig. 81.16: Color Doppler with evidence of flow signals in the

false lumen (FL) consistent with communication. (TL: True lumen)
(Movie clip 81.16).

Fig. 81.18: Echogenic structure (arrowhead) in right atrium (RA)

consistent with thrombus in transit in a patient subsequently diagnosed with acute pulmonary thromboembolism. (LA: Left atrium;
LV: Left ventricle; MV: Mitral valve; RV: Right ventricle) (Movie clip
81.18).

AIR EMBOLISM
Air embolism can result post multiple injuries including
blunt and penetrating chest trauma (Fig. 81.20). It may
result in air in the coronary arteries and Doppler findings
on echo consistent with air in cardiac chambers or major
arteries.39 When a patent foramen ovale is present, a very
small amount of air can result in paradoxical emboli.
Patients may present with hemodynamic instability,
respiratory distress, or postcardiac arrest. Physical exam
findings may be the appearance of cutaneous petechiae.

1977

Fig. 81.17: Spontaneous contrast in false lumen (FL). (TL: True

lumen) (Movie clip 81.17).

Figs 81.19A to D: (A) Patient who presented with submassive

acute pulmonary thromboembolism and right heart strain. Interatrial
septum bows into the left atrium (LA) consistent with elevated right
atrial (RA) pressures. (B) D sign (arrow) revealing right ventricular
(RV) pressure overload and pulmonary hypertension. (C) Tricuspid
regurgitant (TR) velocity > 4 m/s; (D) Dilated inferior vena cava
(IVC) with no inspiratory collapse. (LV: Left ventricle) (Movie clip
81.19B).

Prompt identification is needed to assist in definitive

treatment.

HYPOVOLEMIA
Consideration of hypovolemia should be part of the
differential in patients with hypotension. The size of the left and
right ventricles are not reliable indicators of hypo-volemia;

1978

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 81.20: Air (small echogenic shructures) noted in left

atrium (LA) and left atrial appendage (LAA; Arrows) (Movie clip
81.20).

Figs 81.21A and B: Normal sized left ventricle (LV) with hyperdynamic state in hypotensive patient. End diastole (A) and end systole
(B). (Movie clip 81.21).

however, in severe hypovolemia, the left and right ventricles

are small and hyperkinetic, with evidence of systolic
collapse of the LV (Figs 81.21 and 81.22).4042 A small IVC
(< 1.2 cm) has a 100% specificity (low sensitivity) for a RA
pressure of <10 mm Hg.43

LARGE INTRACARDIAC THROMBUS

Thrombus is defined as a discrete echodense mass with
defined margins that are distinct from the endocardium
and seen throughout systole and diastole. They can
occur in the setting of low flow or stasis. Cardiac thrombi
may predispose the patient to significant morbidity and
mortality. They may be formed de novo or identified as
right heart embolism while in transit.
There are several causes including atrial fibrillation,
atrial flutter, atrial appendage, LV aneurysm formation,

Fig. 81.22: Transesophageal echocardiogram (TEE). Parasternal

long-axis view reveals small left ventricular (LV) diastolic dimensions indicating severe hypovolemia. (AO: Aorta; LA: Left atrium;
RV: Right ventricle) (Movie clip 81.22).

Chapter 81: Echocardiography in Life-Threatening Conditions

1979

Figs 81.23A and B: Apical views. Left ventricular (LV) apical mobile thrombus (arrow) in newly diagnosed congestive heart failure
(CHF) in a patient who suffered a stroke 2 weeks later on anticoagulation therapy. (MV: Mitral valve; RV: Right ventricle) (Movie
clips 81.23A and B)

make a formal diagnosis. Transesophageal images are not

superior over transthoracic images for identification of LV
thrombi as the apex is usually foreshortened but is 100%
sensitive and 99% specific in identifying left atrial and left
atrial appendage thrombus (Fig. 81.24).44
The appearance of thrombi can vary from a heterogeneous, echolucent, protruding masses (in newly formed
thrombus) to a homogenous, smooth mass in chronic
states.46

SUMMARY
In life-threatening conditions, both two-dimensional
TTE and TEE are essential in rapid risk stratification, via
evaluation of cardiac structures and function, as well as
Fig. 81.24: Left atrial thrombus (LA: arrow) extending into left atrial remain a current noninvasive and more cost-effective
appendage (not shown) in a patient with severe mitral stenosis.
mainstay in diagnosis and treatment strategies.
(AV: Aortic valve) (Movie clip 81.24).

REFERENCES
valvular disease, intracardiac devices, postmyocardial
infarction, deep venous thrombosis, and malignancy more
commonly.
Transthoracic echo has a sensitivity of 9095% and a
specificity of 8590% for detection of LV thrombi where the
presence of thrombus was confirmed at surgery or autopsy
(Figs 81.23A and B).43,44 LV mural thrombi are more difficult
to diagnose when contrast agents are not used.45
Optimal views for imaging LV thrombi are apical
images that position the ventricular apex in the near field.
The mass should be visualized in at least two views to

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trauma: evaluation by two-dimensional echocardiography.
Am Heart J. 1987;113(4):9716.
2. Tayal VS, Beatty MA, Marx JA, et al. FAST (focused
assessment with sonography in trauma) accurate for
cardiac and intraperitoneal injury in penetrating anterior
chest trauma. J Ultrasound Med. 2004;23(4):46772.
3. Marciniak D, Smith CE. Pros and cons of transesophageal
echocardiography in trauma care. Internet J Anesth.
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Krner M, Krtz MM, Degenhart C, et al. Current
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Adams A, Fotiadis N, Chin JY, et al. A pictorial review
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Tsang TS, Oh JK, Seward JB, et al. Diagnostic value of
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25(8):73440.
Thakur RK, Aufderheide TP, Boughner DR. Emergency
echocardiographic evaluation of penetrating chest trauma.
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Jimenez E, Martin M, Krukenkamp I, et al. Subxiphoid
pericardiotomy versus echocardiography: a prospective
evaluation of the diagnosis of occult penetrating cardiac
injury. Surgery. 1990;108(4):6769; discussion 679.
Porter JM, Page R, Wood AE, et al. Ventricular perforation
associated with central venous introducer-dilator systems.
Can J Anaesth. 1997;44(3):31720.
Rogers FB, Leavitt BJ. Upper torso cyanosis: a marker for
blunt cardiac rupture. Am J Emerg Med. 1997;15(3):2756.
Martin M, et al. 2-D echocardiography: emergent use in
the evaluation of penetrating precordial trauma. J Trauma.
1991;31(7):9025.
Rendn F, Gmez Dans LH, Castro M. Delayed cardiac
tamponade after penetrating thoracic trauma. Asian
Cardiovasc Thorac Ann. 2004;12(2):13942.
Feigenbaum H, Amstrong WF, et al. Feigenbaums Echocardiography. 6th ed. Lippincott Williams & Wilkins; 2005:
256.
Sybrandy KC, Cramer MJ, Burgersdijk C. Diagnosing
cardiac contusion: old wisdom and new insights. Heart.
2003;89(5):4859.
Wisner DH, Reed WH, Riddick RS. Suspected myocardial
contusion. Triage and indications for monitoring. Ann
Surg. 1990;212(1):826.
Tenzer ML. The spectrum of myocardial contusion: a
review. J Trauma. 1985;25(7):6207.
Kaye P, OSullivan I. Myocardial contusion: emergency
investigation and diagnosis. Emerg Med J. 2002;19:810.

22. Wilson RF, Bassett JS. Penetrating wounds of the pericardium or its contents. JAMA. 1966;195(7):51318.
23. Moore EE. Traumatic ventricular septal defect. Surgery.
2007;142(5):7767.
24. Nguyen R, Ouedraogo A, Deneuville M. Gunshot wounds
to the chest with arterial bullet embolization. Ann Vasc
Surg. 2006;20(6):7803.
25. Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA
2006 Practice Guidelines for the Management of Patients
With Valvular Heart Disease: Executive Summary. J Am
Coll Cardiol. 2006;48(3):598675.
26. Clouse WD, Hallett JW Jr, Schaff HV, et al. Acute aortic
dissection: population-based incidence compared with
degenerative aortic aneurysm rupture. Mayo Clin Proc.
2004;79(2):17680.
27. Mintz GS, Kotler MN, Segal BL, et al. Two dimensional
echocardiographic recognition of the descending thoracic
aorta. Am J Cardiol. 1979;44(2):2328.
28. Khandheria BK, Tajik AJ, Taylor CL, et al. Aortic dissection:
review of value and limitations of two-dimensional
echocardiography in a six-year experience. J Am Soc
Echocardiogr. 1989;2(1):1724.
29. Erbel R, Alfanso F, Boileau C, et al. Diagnosis and
management of aortic dissection. Recommendations of
the Task Force on Aortic Dissection, European Society of
Cardiology. Eur. Heart J. 2001;22:164281.
30. Erbel R, Oelert H, Meyer J, et al. Influence of medical
and surgical therapy on aortic dissection evaluated by
transesophageal echocardiography. Circulation. 1993;
87:160415.
31. Erbel R, Engberding R, Daniel W, et al. Echocardiography
in diagnosis of aortic dissection. Lancet. 1989;1(8636):
45761.
32. Kanojia A, Kasliwal RR. Recent advances in echocardiography of aortic disorders. Asian Cardiovasc Thorac
Ann. 1998;6:1537.
33. Lankeit M, Jimnez D, Kostrubiec M, et al. Predictive
value of the high-sensitivity troponin T assay and the
simplified Pulmonary Embolism Severity Index in
hemodynamically stable patients with acute pulmonary
embolism: a prospective validation study. Circulation.
2011;124(24):271624.
34. Torbicki A. Echocardiographic diagnosis of pulmonary
embolism: a rise and fall of McConnell sign? Eur J
Echocardiogr. 2005;6(1):23.
35. Goldhaber SZ, Visani L, De Rosa M. Acute pulmonary
embolism: clinical outcomes in the International
Cooperative Pulmonary Embolism Registry (ICOPER).
Lancet. 1999;353(9162):13869.
36. Nazeyrollas P, Metz D, Jolly D, et al. Use of transthoracic
Doppler echocardiography combined with clinical and
electrocardiographic data to predict acute pulmonary
embolism. Eur Heart J. 1996;17(5):77986.
37. Frmont B, Pacouret G, Jacobi D, et al. Prognostic value
of echocardiographic right/left ventricular end-diastolic
diameter ratio in patients with acute pulmonary embolism:

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results from a monocenter registry of 1,416 patients. Chest.

2008;133(2):35862.
McConnell MV, Solomon SD, Rayan ME, et al. Regional
right ventricular dysfunction detected by echocardiography
in acute pulmonary embolism. Am J Cardiol. 1996;78(4):
46973.
Yee ES, Verrier ED, Thomas AN. Management of air
embolism in blunt and penetrating thoracic trauma. J
Thorac Cardiovasc Surg. 1983;85(5):6618.
Charron C, Caille V, Jardin F, et al. Echocardiographic
measurement of fluid responsiveness. Curr Opin Crit Care.
2006;12(3):24954.
Feissel M, Michard F, Faller JP, et al. The respiratory
variation in inferior vena cava diameter as a guide to fluid
therapy. Intensive Care Med. 2004;30(9):18347.
Barbier C, Loubires Y, Schmit C, et al. Respiratory changes
in inferior vena cava diameter are helpful in predicting
fluid responsiveness in ventilated septic patients. Intensive
Care Med. 2004;30(9):17406.
Jue J, Chung W, Schiller NB. Does inferior vena cava
size predict right atrial pressures in patients receiving

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mechanical ventilation? J Am Soc Echocardiogr. 1992;

5(6):61319.
Weinsaft JW, Kim RJ, Ross M, et al. Contrast-enhanced
anatomic imaging as compared to contrast-enhanced
tissue characterization for detection of left ventricular
thrombus. JACC Cardiovasc Imaging. 2009;2:96979.
Weinsaft JW, Kim HW, Crowley AL, et al. LV Thrombus
Detection by Routine Echocardiography. Insights Into
Performance Characteristics Using Delayed Enhancement
CMR. JACC: Cardiovascular Imaging. July 2011;4(7):
70212.
Pepi M, Evangelista A, Nihoyannopoulos P, et al.; European
Association of Echocardiography. Recommendations for
echocardiography use in the diagnosis and management
of cardiac sources of embolism: European Association of
Echocardiography (EAE) (a registered branch of the ESC).
Eur J Echocardiogr. 2010;11(6):46176.
Dehghani P, Ibrahim R, Collins N, et al. Post-traumatic
ventricular septal defectsreview of the literature and
a novel technique for percutaneous closure. J Invasive
Cardiol. 2009;21(9):4837.

CHAPTER 82
Lung Ultrasound in Cardiology
Luna Gargani, Eugenio Picano

Snapshot

Physical and Physiological Basis of Lung Ultrasound

Methodology

Pulmonary Interstitial Edema

Pleural Effusion

Pulmonary Embolism

INTRODUCTION
Assessment of the lung has always been considered
off-limits for ultrasound, since it is standard textbook
knowledge that because ultrasound energy is rapidly
dissipated by air, ultrasound imaging is not useful for the
evaluation of the pulmonary parenchyma.1 However, in
recent years, lung ultrasound scan (LUS) has proved to be a
useful tool for evaluating many different acute and chronic
heart and lung disease conditions.2 It is especially valuable
because it is a very easy application of echography, far less
technically demanding than echocardiography,3 rapid to
perform and interpret, portable, repeatable, non-ionizing,
and independent of the cardiac acoustic window. Thus, it
is suitable for a quick but meaningful evaluation for both
in-patients and out-patients.

PHYSICAL AND PHYSIOLOGICAL

BASIS OF LUNG ULTRASOUND
All diagnostic ultrasound methods are based on the
principle that ultrasound is reflected by an interface
between media with different acoustic impedance. In
normal conditions, with aerated lungs, the ultrasound
beam finds the lung air and no image can be depicted
because the ultrasound beam is rapidly dissipated by the
air.4 The only detectable structure is the pleura, visualized

Acute Respiratory Distress Syndrome

Pneumothorax

Cardiopulmonary Ultrasound: An Integrated Approach

Limitations

as a hyperechoic horizontal line, moving synchronously

with respiration (Fig. 82.1, Movie clip 82.1). This dynamic
horizontal movement synchronized with respiration is
called lung sliding. In an aerated lung, several hyperechoic
horizontal lines, arising at regular intervals from the pleural
line, can be seenthese are called A-lines and are not
pathological. When the air content decreases, the acoustic
mismatch needed to reflect the ultrasound beam is
created, and some images appear. In the presence of
extravascular lung water (EVLW), the ultrasound beam
finds subpleural interlobular septa thickened by edema.
The reflection of the beam creates some comet-tail
reverberation artifacts, called B-lines or ultrasound lung
comets (Fig. 82.2; Movie clip 82.2). B-lines are defined
as discrete laser-like vertical hyperechoic reverberation
artifacts that arise from the pleural line, extend to
the bottom of the screen without fading, and move
synchronously with lung sliding.5
The physical basis of the cardiogenic watery B-lines
also explains the source of pneumogenic fibrotic B-lines,
which are found in the presence of interstitial pulmonary
fibrosis.6 The physical scatterer is represented by waterthickened interlobular septa with cardiogenic B-lines, and
by connective tissue-thickened interlobular septa with
pneumogenic B-lines. The two types of B-lines can pose
a challenge to differential diagnosis, although several
clues may help distinguish the two entities. Cardiogenic

Chapter 82: Lung Ultrasound in Cardiology

Fig. 82.1: Sonographic appearance of a normal lung.

1983

Fig. 82.2: Sonographic appearance of multiple B-lines (indicated

by the white arrows).

extravascular lung water very early in the course of lung

injury in pigs, even at a stage when no changes in hemoga
sanalytic parameters can be observed.9

METHODOLOGY

Fig. 82.3: Sonographic appearance of a consolidated lung. The

echotexture becomes similar to that of the liver.

B-lines are always bilateral and are generally more diffuse

in the right lung than the left lung, with a hot zone of
higher density along the axillary lines (in lying patients,
as decubitant regions).7 Moreover, cardiogenic B-lines
can be dissolved in a few hours by an acute diuretic load.8
For a further reduction in the air content, when the lung
is consolidated, it is possible to visualize the pulmonary
parenchyma as a solid parenchyma, just like the liver or
the spleen (Fig. 82.3).
B-lines are a very early event in the cascade, leading to
pulmonary edema, as has been shown in an experimental
model of oleic acid-induced lung injury, which mimics
human acute respiratory distress syndrome (ARDS).
B-lines unmasked accumulation of histologically verified

The lung ultrasound examination can be performed

using any commercially available two-dimensional (2D)
scanner (cardiac, convex, microconvex, or linear probe),
also portable, with any transducer frequency. Higher
frequencies, as in linear probes, are useful for the
evaluation of the pleura, but provide a worse definition of
the pulmonary parenchyma. There is no need for a second
harmonic or Doppler imaging mode. The examinations are
performed with patients in a near-supine, supine, sitting,
or even standing position, as clinically indicated. Two
main approaches to LUS should be consideredone for
diagnosis and one for semiquantification and follow-up.
In patients admitted to the emergency department, for a
prompt diagnosis, LUS can be focused on eight areas, two
anterior and two lateral per side.5 When the assessment
is focused on the evaluation of pulmonary congestion,
in order to achieve a semiquantification that may help
for follow-up and prognostic stratification, it would be
appropriate to use the scheme shown in Figure 82.4.7
Ultrasound scanning of the anterior and lateral chest is
obtained on the right and left hemithorax, from the second
to the fourth (on the right side to the fifth) intercostal
spaces, and from the parasternal line to the axillary line.
The sum of the B-lines found on each scanning site yields
a score denoting the extent of extravascular fluid in the

1984

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 82.4: Thoracic scanning areas for semiquantitative assessment of B-lines.

Source: Modified from Jambrik et al. 2004.

lung. Zero is defined as a complete absence of B-lines in

the investigated area. For clinical purposes, B-lines may be
semiquantified from mild to severe degree, similar to what
is done for most echocardiographic parameters.10 B-lines
have a very satisfactory intraobserver and interobserver
variability, consistently < 10%.7

PULMONARY INTERSTITIAL EDEMA

Diagnosis
The possibility of detecting pulmonary edema before it
becomes clinically apparent is so inherently attractive
that the effort to develop and validate such a technique
still continues after many years of tireless and relatively
unrewarding attempts.11 Chest X-ray remains by far the
best and most frequently used screening test for the
detection of pulmonary edema, but is difficult to interpret
and is imprecise, with high interobserver variability.12
The absence of chest X-ray findings does not exclude the
presence of a high pulmonary capillary wedge pressure
(PCWP) > 30 mm Hg. According to American Heart
Association/American College of Cardiology guidelines,
serial chest radiographs are not recommended in the
assessment of pulmonary congestion in chronic heart
failure (HF), since they are too insensitive to detect all
but the most extreme changes in the fluid status. Direct
measurement of PCWP via catheterization is the gold
standard to evaluate hemodynamic congestion, but its
invasive nature limits clinical utility. Thus, because current

technology for measuring lung edema can be inaccurate

(chest X-ray), cumbersome (nuclear medicine and radio
logy techniques), or invasive (indicator dilution), there is
great potential for a technology that could quantify lung
edema noninvasively in real time with a radiation-free and
portable method.
Being a sign of pulmonary congestion, B-lines are
useful for the identification of cardiogenic versus noncar
diogenic dyspnea in an acute setting,13 with an accuracy
comparable to that of natriuretic peptides.1416 B-lines could
be a plausible alternative, especially in emergency depart
ments where natriuretic peptide analysis is not available,
and could aid diagnosis in cases where natriuretic peptide
levels are in the gray zone, and positive predictive value
is not very high, especially in patients with renal failure.

Treatment
The recognition, quantification, and monitoring of pulm
onary congestion is important for clinicians at all stages of
care of the HF patient. Accurate assessment of effectiveness
of medical treatment in reducing pulmonary congestion
is mandatory in these patients. Chest X-ray remains by
far the best and most frequently used screening test for
the detection and in-hospital follow-up of pulmonary
congestion, although showing the previously mentioned
limitations. Another way to monitor congestion is through
monitoring body weight. However, this has limited
reliability as a predictor of congestion status, since body

Chapter 82: Lung Ultrasound in Cardiology

weight does not take into account systemic water, weight

gain may reflect normal fluctuations in time, and weight
loss due to loss of muscle/fat (cardiac cachexia) may
obscure increased fluid retention.17
B-lines have already been proposed as a bedside, easyto-use alternative diagnostic tool for clinically monitoring
pulmonary congestion in patients with HF.18 The sign is
very dynamic, as shown by rapid increase after exercise,
in patients with and without left ventricular dysfunction.19
B-lines are well-related to the degree of dyspnea: the
number of B-lines increases, as the degree of dyspnea
worsens.20 Since B-lines can be dissolved in a few minutes
by an acute diuretic load, they may be a useful bedside tool
for monitoring diuretic therapy response, in a real time
fashion.8,18
The simplicity and low-tech nature of this examination
also makes it appealing for out-of-hospital office monit
oring of HF patients; there would be the possibility of
tailoring pharmacological therapy as soon as the patient,
although asymptomatic, shows echographic signs of
pulmonary congestion, in order to avoid new hospita
lizations for worsening dyspnea, which would likely
appear with some days of delay.21 The possibility of
assessing B-lines with light, portable, hand-held devices
could also allow a cardiologist to evaluate the degree of
decompensation directly at the patients home.3

Prognosis
Persistent hemodynamic congestion that is not adequately
recognized and treated before discharge is associated with
adverse clinical outcome in HF patients; on the other
hand, postdischarge freedom from pulmonary congestion
is associated with a better prognosis.22 It has been shown
that in patients admitted to the hospital with dyspnea and/
or chest pain, the presence of B-lines identifies a subgroup
at a higher risk of experiencing eventsthe higher the
number of B-lines, the worse the outcome. The 16-month
event-free survival showed a significantly better outcome
for those patients without B-lines, whereas a worse outcome
was observed in patients with a severe grade of B-lines
(total number > 30). In regard to future HF hospitalization
alone, and not as part of the combined endpoint, the rate
of new hospitalization for progression of HF was higher in
patients with a severe B-line score and lower in patients
with no B-lines (log rank = 24.4, P < 0.0001).23 These data
have also been confirmed in a multicentric study on a
large number of patients on hemodialysis.24

1985

PLEURAL EFFUSION
Detection of pleural effusion (PE) is the more established
application of LUS.1 PE can be easily detected directly
through the intercostal spaces with either a cardiac or
convex probe. The effusion should first be sought in
dependent zones, that is, lateral and posterior chest. It
rules out other etiologies such as atelectasis, consolidation,
mass, or an elevated hemidiaphragm. It takes less time
than radiographic methods and can be repeated serially
at the bedside. Ultrasound images PE as an anechoic or
hypoechoic space between two pleural layers. The lung
behind a PE appears either aerated or consolidated in the
case of large PE. In critically ill patients, LUS is especially
valuable, showing better sensitivity and reliability than
bedside chest X-ray for the diagnosis of PE.25,26 Bedside
chest X-ray rarely detects small effusions and can also miss
effusions of up to 500 mL.27 For the detection of PE, with
computed tomography (CT) as a gold standard, sensitivity
and specificity of ultrasound are 93%.27 Minimal effusions
can be detected using ultrasound, provided the probe is
applied over an adequate area of the chest. Ultrasound
can detect the effusion, evaluate its volume, provide
information on its nature, and indicate the appropriate
area for an eventual thoracocentesis. Ultrasound is
acknowledged as the method of choice for detecting an
effusion in a supine patient.

PULMONARY EMBOLISM
The diagnosis of pulmonary embolism has always been a
considerable challenge and requires a high index of clinical
suspicion from the emergency physician. In addition,
diagnosing pulmonary embolism may require the use of
one or more direct and indirect diagnostic methods. Most
lesions related to pulmonary embolism are localized in
the lower lobes of the lung and are often associated with
an area of pleuritic chest pain. These processes essentially
involve the terminal air spaces and result in the evacuation
of air from the affected parenchymal area, creating an
ultrasonic window and allowing ultrasound waves to
travel further into the lung. The characteristic sonographic
findings in pulmonary embolism are multiple, hypoechoic,
pleural-based parenchymal lesions which adopt a wedge
shape.28 These lesions can be visualized at least in three
fourths of cases.29 The accuracy of LUS for the detection of
pulmonary embolism was found to be 84% at a prevalence
of 55% in a large multicenter study series comprising
352 patients with suspected pulmonary embolism.30

1986

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 82.5: Small subpleural consolidations and multiple B-lines in

a typical sonographic pattern of ALI/ARDS.

In combination with echocardiography and leg vein

compression sonography, the accuracy of LUS is >90%.31 In
order to be detectable by LUS, the lesions need to extend to
the pleural surface, which is usually the case. In addition,
mechanical alterations associated with consolidated lung
tissue, increased capillary pressure, and permeability,
due to the release of inflammatory mediators, also cause
increased exudation of fluid,28 leading to localized pleural
fluid collection adjacent to the affected pulmonary region
or presence of localized multiple B-lines. Exploration of
the lesions by color Doppler imaging may provide addi
tional diagnostic information. In pulmonary infarction,
areas of pulmonary arterial flow cannot be detected on
color Doppler ultrasound, a phenomenon referred to
as consolidation with little perfusion.31 On the other
hand, recanalization of incomplete infarction resulting
from anticoagulation treatment or intrinsic lysis can be
demonstrated by the reappearance of a blood flow signal
on follow-up. It is important to note that a normal LUS
does not exclude the presence of PE.29

ACUTE RESPIRATORY
DISTRESS SYNDROME
ARDS is a common syndrome of diffuse lung injury
and has a high mortality rate.32 In ARDS, LUS provides a
sensitivity of 98% and a specificity of 88% in diagnosing
the presence of the alveolar-interstitial syndrome as seen
at CT, performing better than either auscultation or chest

X-ray.33 Differential diagnosis between acute cardiogenic

pulmonary edema and ARDS may often be difficult. The
sonographic pattern of multiple diffuse B-lines is present
in both conditions. However, there are some clues that
may help in the differential diagnosispleural line
abnormalities, presence of small subpleural consolidations
or coarse appearance of the pleural line (Fig. 82.5), spared
areas defined as areas of normal lung pattern surrounded
by areas of multiple B-lines, and lung consolidations are
often found in ARDS, but are not present in cardiogenic
pulmonary edema.34
B-lines can also identify subclinical pulmonary edema
in situations of extreme physiology such as strenuous effort
in ordinary conditions (elite apnea divers or triathletes)35,36
or ordinary effort in extreme conditions such as high
altitude pulmonary edema in recreational climbers,37
again suggesting that clinical symptoms are only the tip of
the iceberg of pulmonary edema, even outside the acute
HF syndrome.

PNEUMOTHORAX
Pneumothorax (PTX) is a frequent, life-threatening
complication in patients who have been admitted to the
emergency department. Bedside chest X-ray may mis
diagnose up to 30% of cases.38 Radiographically occult
PTX may rapidly progress to tension PTX if its diagnosis
is missed or delayed, especially in patients receiving
mechanical ventilation.39 Being a nondependent
condition, PTX should be sought at first on the anterior
and lower areas. Absence of lung sliding is a basic and
initial step for the diagnosis, and a striking absence of
motion arising from the pleural line is observed instead
of the lung sliding.40,41 The presence of lung sliding allows
PTX to be confidently discounted because the negative
predictive value is 100%.40 The abolition of lung sliding
can be objectified in M-mode, which gives a characteristic
pattern, the stratosphere sign. However, absent lung sliding
does not necessarily mean PTX. Many other situations yield
abolished lung sliding, such as high-frequency ventilation,
massive atelectasis, pleural adherences, severe fibrosis,
and so on.5 The other conditions needed to diagnose PTX
by LUS are absence of B-linesthe slightest B-line allows
prompt ruling out of PTX.40 The pathognomonic LUS sign
of PTX is the lung point, which allows PTX to be confirmed,
with a specificity of 100% and sensitivity of about 65%, a
percentage that falls with major PTX with complete lung
retraction.42

Chapter 82: Lung Ultrasound in Cardiology

1987

Table 82.1: The Three B-Line Scenarios: Heart Failure, ALI/ARDS, and Interstitial Lung Disease

Clinical Examples

Heart Failure

ALI/ARDS

Pulmonary Fibrosis

EF

Abnormal (decreased)

Normal

Normal

PASP

Abnormal (increased)

Normal

Normal/Increased

Effect of diuresis/dialysis
on B-lines

Reduction in minuteshours

No effect

No effect

Other LUS sign

Pleural effusion

Subpleural alterations

Pleural thickening

Theater

ER/Cardiology/Internal Medicine

Intensive Care

Pulmonology/Internal Medicine

(ALI: Acute lung injury; ARDS: Acute respiratory distress syndrome; EF: Ejection fraction; LUS: Lung ultrasound scan; PASP:
Pulmonary artery systolic pressure).

CARDIOPULMONARY ULTRASOUND:
AN INTEGRATED APPROACH
Echocardiography is an essential tool for the management
of patients with heart disease, providing an enormous
amount of information on both acute and chronic condi
tions. The addition of LUS to echocardiography provides
an additive insight into the presence of EVLW or any other
pulmonary involvement. Presence of multiple, diffuse,
bilateral B-lines associated with left ventricular systolic
and/or diastolic dysfunction or valvular heart disease is
highly suggestive of cardiogenic pulmonary congestion.
Moreover, for any given level of cardiac dysfunction,
the response of the pulmonary vascular bed may be
variable. LUS helps identify those patients who, although
asymptomatic, are going to decompensate and require
more aggressive treatment.43,44 Presence of multiple,
diffuse, bilateral B-lines, associated with a normal heart,
indicates a noncardiac cause of pulmonary edema such as
acute lung injury (ALI)/ARDS or interstitial pneumonia.
Alternatively, especially in a chronic setting, it should
prompt the suspicion of pulmonary fibrosis. It is important
to distinguish the multiple, diffuse, bilateral B-line pattern
from focal multiple B-lines, which can be present in
normal lungs or may be seen in the context of many
pathological conditions such as lobar pneumonia, pulmo
nary contusion, pulmonary infarction, pleural disease,
and neoplasia. This further underlines the importance of
integrating LUS findings with the patients history, clinical
presentation, and other instrumental data. An example
of the integration between clinical, echocardiography,
and lung ultrasound information for the differentiation of
the three main scenarios of B-lines in HF, ALI/ARDS, and
interstitial lung disease is reported in Table 82.1.

LUS is one of the easiest applications of echography,

much easier than echocardiography. Image patterns
are readily teachable, and minimal didactic and image
recognition skill sessions are needed.3,45 The learning
curve for B-line evaluation and grading is very short. The
complement of LUS to echocardiography would require
only a few minutes in addition to the time needed for a
resting echocardiogram.

LIMITATIONS
LUS limitations are essentially patient-dependent. Obese
patients may be more difficult to examine due to the
thickness of their rib cage and soft tissues. The presence
of subcutaneous emphysema or large thoracic dressings
alters or precludes the propagation of the ultrasound
beams to the subpleural lung parenchyma. The main
limitation of B-line interpretation is the lack of specificity.
As previously mentioned, they are a sign of interstitial
syndrome; therefore, they are a very sensitive but not
specific sign of cardiogenic pulmonary edema. How to
distinguish the different etiologies of B-lines has been
discussed in this chapter. However, it must be emphasized
that LUS does not rule out pulmonary abnormalities
(especially consolidations) that do not reach the pleura.
In the next few years, LUS is likely to become an
extension of physical examination in many different
clinical settings, from the emergency department to the
intensive care unit, from cardiology to pulmonology, and
nephrology wards and dialysis units.

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1988

Section 7: Miscellaneous and Other Noninvasive Techniques

2. Moore CL, Copel JA. Point-of-care ultrasonography. N Engl

J Med. 2011;364(8):74957.
3. Bedetti G, Gargani L, Corbisiero A, et al. Evaluation of
ultrasound lung comets by hand-held echocardiography.
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4. Soldati G, Copetti R, Sher S. Sonographic interstitial
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5. Volpicelli G, Elbarbary M, Blaivas M, et al; International
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6. Gargani L, Doveri M, DErrico L, et al. Ultrasound lung
comets in systemic sclerosis: a chest sonography hallmark
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7. Jambrik Z, Monti S, Coppola V, et al. Usefulness of ultrasound
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8. Picano E, Gargani L. Ultrasound lung comets: the shape of
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10. Picano E, Frassi F, Agricola E, et al. Ultrasound lung comets:
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11. Lange NR, Schuster DP. The measurement of lung water.
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12. Nieminen MS, Bhm M, Cowie MR, et al; ESC Committee
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guidelines on the diagnosis and treatment of acute heart
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13. Lichtenstein D, Mezire G. A lung ultrasound sign allo
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14. Gargani L, Frassi F, Soldati G, et al. Ultrasound lung
comets for the differential diagnosis of acute cardiogenic
dyspnoea: a comparison with natriuretic peptides. Eur J
Heart Fail. 2008;10(1):707.
15. Liteplo AS, Marill KA, Villen T, et al. Emergency thoracic
ultrasound in the differentiation of the etiology of shortness
of breath (ETUDES): sonographic B-lines and N-terminal
pro-brain-type natriuretic peptide in diagnosing congestive
heart failure. Acad Emerg Med. 2009;16(3):20110.
16. Prosen G, Klemen P, trnad M, et al. Combination of lung
ultrasound (a comet-tail sign) and N-terminal pro-brain
natriuretic peptide in differentiating acute heart failure
from chronic obstructive pulmonary disease and asthma
as cause of acute dyspnea in prehospital emergency
setting. Crit Care. 2011;15(2):R114.

17. Chaudhry SI, Wang Y, Concato J, et al. Patterns of weight

change preceding hospitalization for heart failure.
Circulation. 2007;116(14):154954.
18. Volpicelli G, Mussa A, Garofalo G, et al. Bedside lung
ultrasound in the assessment of alveolar-interstitial
syndrome. Am J Emerg Med. 2006;24(6):68996.
19. Agricola E, Picano E, Oppizzi M, et al. Assessment of stressinduced pulmonary interstitial edema by chest ultrasound
during exercise echocardiography and its correlation
with left ventricular function. J Am Soc Echocardiogr.
2006;19(4):45763.
20. Frassi F, Gargani L, Gligorova S, et al. Clinical and echocar
diographic determinants of ultrasound lung comets. Eur J
Echocardiogr. 2007;8(6):4749.
21. Yu CM, Wang L, Chau E, et al. Intrathoracic impedance
monitoring in patients with heart failure: correlation with
fluid status and feasibility of early warning preceding
hospitalization. Circulation. 2005;112(6):8418.
22. Gheorghiade M, Filippatos G, De Luca L, et al. Congestion
in acute heart failure syndromes: an essential target of
evaluation and treatment. Am J Med. 2006;119(12 Suppl 1):
S310.
23. Frassi F, Gargani L, Tesorio P, et al. Prognostic value of
extravascular lung water assessed with ultrasound lung
comets by chest sonography in patients with dyspnea and/
or chest pain. J Card Fail. 2007;13(10):8305.
24. Zoccali C, Torino C, Tripepi R, et al.; Lung US in CKD
Working Group. Pulmonary congestion predicts cardiac
events and mortality in ESRD. J Am Soc Nephrol. 2013;
24(4):63946.
25. Eibenberger KL, Dock WI, Ammann ME, et al. Quantification
of pleural effusions: sonography versus radiography.
Radiology. 1994;191(3):6814.
26. Balik M, Plasil P, Waldauf P, et al. Ultrasound estimation of
volume of pleural fluid in mechanically ventilated patients.
Intensive Care Med. 2006;32(2):31821.
27. Roch A, Bojan M, Michelet P, et al. Usefulness of ultrasono
graphy in predicting pleural effusions > 500 mL in patients
receiving mechanical ventilation. Chest. 2005;127(1):
22432.

28. Reissig A, Heyne JP, Kroegel C. Sonography of lung
and pleura in pulmonary embolism: sonomorphologic
characterization and comparison with spiral CT scanning.
Chest. 2001;120(6):197783.
29. Mathis G. Chest Sonography. 2nd ed. Berlin: Springer
Verlag; 2008.
30. Mathis G, Blank W, Reissig A, et al. Thoracic ultrasound
for diagnosing pulmonary embolism: a prospective multi
center study of 352 patients. Chest. 2005;128(3):153138.
31. Yang PC. Color Doppler ultrasound of pulmonary consoli
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32. Ware LB, Matthay MA. The acute respiratory distress synd
rome. N Engl J Med. 2000;342(18):133449.
33. Lichtenstein D, Goldstein I, Mourgeon E, et al. Comparative
diagnostic performances of auscultation, chest radiography,
and lung ultrasonography in acute respiratory distress
syndrome. Anesthesiology. 2004;100(1):915.

Chapter 82: Lung Ultrasound in Cardiology

34. Copetti R, Soldati G, Copetti P. Chest sonography: a useful

tool to differentiate acute cardiogenic pulmonary edema
from acute respiratory distress syndrome. Cardiovasc
Ultrasound. 2008;6:16.
35. Frassi F, Pingitore A, Cialoni D, et al. Chest sonography
detects lung water accumulation in healthy elite apnea
divers. J Am Soc Echocardiogr. 2008;21(10):11505.
36. Pingitore A, Garbella E, Piaggi P, et al. Early subclinical
increase in pulmonary water content in athletes performing
sustained heavy exercise at sea level: ultrasound lung
comet-tail evidence. Am J Physiol Heart Circ Physiol.
2011;301(5):H21617.
37. Pratali L, Cavana M, Sicari R, et al. Frequent subclinical
high-altitude pulmonary edema detected by chest
sonography as ultrasound lung comets in recreational
climbers. Crit Care Med. 2010;38(9):181823.
38. Chiles C, Ravin CE. Radiographic recognition of pneum
othorax in the intensive care unit. Crit Care Med.
1986;14(8):67780.
39. Bridges KG, Welch G, Silver M, et al. CT detection of occult
pneumothorax in multiple trauma patients. J Emerg Med.
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1989

40. Lichtenstein DA, Menu Y. A bedside ultrasound sign ruling

out pneumothorax in the critically ill. Lung sliding. Chest.
1995;108(5):13458.
41. Kirkpatrick AW, Sirois M, Laupland KB, et al. Hand-held
thoracic sonography for detecting post-traumatic pne
umothoraces: the Extended Focused Assessment with Sono
graphy for Trauma (EFAST). J Trauma. 2004;57(2):28895.
42. Lichtenstein D, Mezire G, Biderman P, et al. The lung
point: an ultrasound sign specific to pneumothorax. Inte
nsive Care Med. 2000;26(10):143440.
43. Picano E, Gargani L, Gheorghiade M. Why, when, and how
to assess pulmonary congestion in heart failure: pathophy
siological, clinical, and methodological implications. Heart
Fail Rev. 2010;15(1):6372.
44. Gargani L. Lung ultrasound: a new tool for the cardiologist.
Cardiovasc Ultrasound. 2011;9:6.
45. Noble VE, Lamhaut L, Capp R, et al. Evaluation of a tho
racic ultrasound training module for the detection of
pneumothorax and pulmonary edema by prehospital
physician care providers. BMC Med Educ. 2009;9:3.

CHAPTER 83
The Future of
Echocardiography and Ultrasound
David Cosgrove

Snapshot

Plane Wave Ultrafast Imaging

Trends in Scanners

Doppler

Microbubbles

Elastography

Light and Sound

Therapeutic Applications of Ultrasound

INTRODUCTION
Ultrasound has shown a remarkable ability to evolve
and even revolutionize, thanks to the efforts of research
laboratories and commercial companies around the
world. Unlike some industries, ideas developed in one
quarter find their way into clinical scanners surprisingly
rapidly, to the benefit of our patients. Here, some of the
more exciting and intriguing developments and prospects
are briefly covered, but it should be borne in mind that the
most important innovations may well be unpredictable:
expect the unexpected!

PLANE WAVE ULTRAFAST IMAGING

A major trend in ultrasound imaging is likely to be the
implementation of plane wave imaging1 (Fig. 83.1). This
approach, first mooted in the 1980s, radically changed the
way scanning is performed.2 Instead of transmitting a series
of focused ultrasound beams one by one, the transmitted
ultrasound is unfocused, all the elements being activated
at once. All the focusing is performed on receive, ideally
using software-driven systems. The potential penalty
in spatial resolution is more than made for by the huge
increase in frame rate (up to 20,000 fps, depending on
the required depth) so that multiple pulses can be sent,
for example, for compounding, while still retaining very

Fig. 83.1: Ultrafast plane wave imaging. The left image shows a
transverse section of a rat brain, measuring about 1 cm across,
imaged with the conventional pulse-echo technique. Spatial resolution is greatly increased by using plane wave imaging, shown
on the right, in which multiple interrogation at a different angle of
insonation (compounding) has been used.
Source: Redrawn with permission from Mace E, Montaldo G,
Cohen I, et al. Functional ultrasound imaging of the brain. Nat
Methods. 2011;8(8):6624.

high frame rates. The images are crisper because the

transmission is so rapid that any motion-induced blurring
is frozen; this is likely to be especially important in
cardiology and particularly for the fetal heart. Doppler

Chapter 83: The Future of Echocardiography and Ultrasound

1991

Fig. 83.2: Ultrafast Doppler. A series of transverse sections of a

rat brain using directional power Doppler at an acquisition rate
of 20,000/s. The method allows changes in cerebral blood flow
to be displayed after brain stimulation in the same way as
functional MRI.
Source: Redrawn with permission from Tanter M.

Fig. 83.3: Smartphone ultrasound system. This USB-based scanner from Mobisante uses a Smartphone to create ultrasound
images. The implications for usage and training are major. http://
www.mobisante.com/product-overview/

could also benefit3 (Fig. 83.2) as could elastography4

and contrast studies could take advantage of the overall
lower peak power (since there is no transmit focusing)
which could reduce bubble destruction. In addition, the
possibility of using extended multipulse methods could
improve tissue suppression.
The reason for the slow implementation of what
seems like an obvious way to exploit one of the strengths
of ultrasound, its speed, has been computing limitations.
Handling the huge amount of data and capturing and
processing the radio frequency (RF) data from over 100
elements at such high data rates was impossible when
the approach was first suggested. With the startling speed
bumps that the industry has experienced and with the
addition of fast graphics chips (stimulated by the computer
gaming industry), this limitation has been rolled back
and partial implementations have appeared in clinical
scanners. Much further development can be expected.

training and embodies a cruel conundrum: less skilled

users need the best image quality, and this is denied
them. They raise concerns for the quality of diagnoses and
possible legal consequences. They could also exacerbate
the great harm that ultrasound has delivered in some
developing countries: informing on the gender of the
fetus so that females can be selectively aborted, a human
disaster of unprecedented scale. Indeed, it could be argued
that, on account of this, ultrasound has done more harm
than good worldwide.6
Increasingly, software-driven scanners can be expected to predominate, at least at the mid- and high end, with
fast computing and graphics cards replacing fixed function
chips for greater flexibility, upgradability and advantages
in servicing. This could result in price reductions, though
there has been no sign of this to date. They are also expected
to form the platform for plane wave (ultrafast) imaging.
Always the key component of an ultrasound system,
transducers are being improved, chiefly with the intro
duction of single crystal piezoelectric materials.7 These
are grown rather like a silicon chip, rather than being a
ceramic that requires firing, as is used in conventional
piezo materials. Single crystals have better sensitivity on
both transmit and receive, and wider bandwidths, so that
they can operate over wider frequency ranges, meaning
that one probe can work over a wider range of applications.
This could be especially important in contrast-enhanced

TRENDS IN SCANNERS
Miniaturization is well under way, with laptops and
even smart phones being used, if only for display5
(Fig. 83.3). These devices are likely to find their way into
every clinicians white coat pocket, supplementing or
even replacing their stethoscopes. This degree of miniatu
rization comes at a cost: their image quality cannot rival
that of high-end systems. This puts an extra burden on

1992

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 83.4: Live/real time three- and four-dimensional transesophageal echocardiography. En face view of paravalvular mitral prosthetic regurgitation, oriented to be identical to the surgical view.
The paravalvular (P) defect is localized at 5 oclock position in
this patient with a metallic prosthesis. (AO: Aorta; LAA: Left atrial
appendage; MVR: Mitral valve replacement).
Source: Reproduced with permission from Singh P, Manda J, Hsiung MC, et al. Live/real time three-dimensional transesophageal
echocardiographic evaluation of mitral and aortic valve prosthetic
paravalvular regurgitation. Echocardiography 2009;8:9807.

Fig. 83.5: Acoustic structure quantification of the liver. This form of

tissue characterization looks at the B-mode structure, working in
the radio frequency (RF) domain, to assess the image texture. The
result is a moving image in which uniform structures are displayed
in blue or green, and heterogeneous structures, here exemplified
by the walls of blood vessels, are depicted as orange and red. It is
likely to be of most value in the liver for assessing steatosis (very
uniform) or severe fibrosis and cirrhosis.

ultrasound (CEUS) where higher harmonics need to be

detected. Their efficiency is also key to the development
of cable-free transducer systems: adequate battery life
depends on highly efficient transduction. There is also a
move to replace the lead in Lead zirconate titrate (PZT)
transducers by less toxic elements such as barium, driven
by concerns over the potential toxicity of lead in discarded
transducers.8
One transducer trend that, sadly, seems to have stalled
is the capacitance micro-machined ultrasound transducer
(cMUT); these tiny electrostatic transducers are formed
by silicon etching using a photographic mask, just as for a
semiconductor chip, but are built with an air gap between
two electrodes.9 When an electric charge is placed across
the gap, the plates are attracted (or repelled, depending on
the charge direction) and the plates move closer together
(or further apart), exactly as in an electrostatic loudspeaker.
The symmetrical effect on receipt of an acoustic signal
allows them to act as both receivers and transmitters. Each
element is only a millimeter or so in size and so a matrix of
many thousands is needed. An advantage is that once the
photo mask has been created, they can be made in large
numbers in the same way as silicon chips, and they could
therefore become cheap and even disposable (of interest
for intravascular ultrasound). It is also possible to build

semiconductors onto the back of the cMUT, operating as

preamplifiers, for example. Prototypes have been built, but
the field seems to have gone into abeyance.
Ways to cut conventional PZT arrays into two-dimen
sional matrices containing thousands of elements have
been developed.10 These engineering wonders pose a major
challenge in cabling and in handling the huge amount
of data they generate, but they make possible threedimensional (3D) and four-dimensional (4D) transducers
that have been especially promising in echocardiography
(Fig. 83.4) and may eventually develop into the standard
way to perform ultrasound examinations by replacing the
bulky hybrid electromechanical probes that are widely
available.
Analysis of the B-mode signal content historically
formed the basis for ultrasound tissue characterization, a
subject that had a vogue but lost its momentum. Interest
has now been rekindled because the capacity to work with
RF or raw data has arisen with continuing improvements in
computing power and data storage and the annual Tissue
Characterization Conference has regained popularity.11
There are also commercial implementations that either
map tissue texture [e.g. Toshibas Acoustic Structure
Quantification, ASQ12 (Fig. 83.5)] or look at aspects of the
frequency spectrum of the echoes (e.g. Histoscanning).13

Chapter 83: The Future of Echocardiography and Ultrasound

1993

interrogated from several angles. The computer can

then correct the result for the beam-to-vessel angle. This
approach can be done with ultrafast imaging, the rapidly
acquired data being reviewed in slow motion so that
spectral measurements can be made postacquisition.16 In
a smart innovation, another approach is to make use of the
lateral modulation that is conventionally ignored to gather
data about both inline and across-line Doppler shifts.17
The resulting color Doppler real time display is direction
independent, with vector arrows to indicate the flow
direction. It is particularly useful for tortuous vasculature,
though the method works less well for deeper vessels.

MICROBUBBLES
Fig. 83.6: Fusion imaging. In order to biopsy this liver lesion, a
previously acquired computed tomography (CT) scan has been
entered into the scanners memory and the two image sets aligned
using a position sensor attached to the ultrasound probe. The
lesion is highlighted in a green circle. This allows the complete
cross-sectional images of CT to be synchronized with the ultrasound images and the latter used for improved targeting of needles for biopsy and interstitial therapy.

An important trend, especially for interventional work,

is fusion imaging, which combines the advantages of the
complete cross-sectional display of computed tomography
(CT) and magnetic resonance (MR) with the real time
interactiveness of ultrasound14 (see Fig. 83.6). In this
approach, a 3D data set from a CT or MR scan is imported
into the ultrasound system; the probe is attached to an
electromagnetic position sensor and its position in space
is linked to the cross-sectional imaging, which is then
resliced and adjusted to match the ultrasound images.
This allows a needle or ablation antenna to be positioned
using ultrasound together with the CT or MR data.
A redesign of the scanner user interfaces to improve
cleanliness by replacing the current button and knobdriven panels with flat wipe-down control surface would
be especially important in sensitive environments where
microbial contamination is to be avoided (e.g. intrao
perative ultrasound).15 It would necessitate a radical
rethink but the benefits could be wide ranging.

DOPPLER
Doppler has lagged behind until recently. However, the
development of methods to mitigate its angle depen
dence should simplify its routine use. One method
uses compounding, so that each pixel or range gate is

The available microbubbles for ultrasound contrast have

excellent properties and, with microbubble-specific
multipulse techniques at low mechanical index (MI), give
good enhancement of both the macro- and microvascular
systems; it has become routine for characterization of
focal liver lesions (Figs. 83.7A to C), for endocardial border
detection, and is also widely used for assessing myocardial
perfusion.1820 Many innovative applications are being
developed, including administration into other body
cavities and even intradermal injection for ultrasound
lymphangiography (Fig. 83.8).
However, there remains a huge potential for further
development, both for diagnostic and for therapeutic
purposes. There is much preclinical work on targeted
microbubbles, mainly directed to the endothelium, the first
cell membrane encountered after intravenous injection.21
Three main directions have emerged: targeting activated
endothelium as a means to image acute inflammation,
targeting neovascularization as a means to image the new
vessels in tumors, atheroma and chronic inflammation
and targeting thrombus, all forms of molecular imaging.22
The first human studies with a microbubble targeted
to VEG-F2 have begun addressing the need for better
imaging of prostate cancer, with promising results.23
One of the difficulties has been the choice of ligand: the
conventional streptavidin-biotin linker is highly effective
but is antigenic, so not suitable for human use. For the
VEG-F2 study, Bracco developed a peptide linker that
seems to be well tolerated, and there are also maleamide
linkers that may prove clinically useful.
This target is restricted to the vascular space and
the endothelium because microbubbles are too large
to penetrate into the interstitium, even when it is leaky,
as occurs in tumors. So there has been much interest in

1994

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 83.7A to C: Liver metastasis. This lesion in the inferior

border of the liver is indeterminate on B-mode ultrasound (arrow
in A) despite the presence of scanty power Doppler signals.
Following intravenous (IV) administration of a 1.2-mL dose of the
microbubble SonoVue, there is enhancement in the arterial phase
at 39 s (B) followed shortly by washout in the late phase at 1 min
07 s (C), seen as an increase and then a reduction in the goldcolored signals in the left pane. These hemodynamic features are
characteristic of malignancy, in this case, metastatic disease.

Fig. 83.8: Sentinel lymph nodemicrobubble lymphangiography.

In this patient with an invasive breast carcinoma, a small amount
(0.4 mL) of SonoVue was injected intradermally in the periareaolar
tissue and the draining lymphatic (seen here as a green curved
line in the left-hand panel) traced to the sentinel lymph node
(arrow), which was then biopsied. The method could improve
preoperative staging in these patients and avoid the need for
frozen section microscopy in theater, thus reducing the need for
repeat surgery when this is falsely negative, as occurs in up to 25%
of cases.

developing particles24 (actually condensed microbubbles

with no gas) that are both small enough to do so and can
be re-activated to form microbubbles by sonication once
they have reached the desired tissue space or target.

ELASTOGRAPHY
A special opportunity to examine the mechanical
properties of human tissue has been the development

Chapter 83: The Future of Echocardiography and Ultrasound

1995

Fig. 83.9: Strain elastography. This breast carcinoma shows as

a predominantly blue (indicating hard) region in the elastogram,
shown in the left panel. Regions of interest can be placed over the
surrounding fat and over the lesion to derive a strain ratio, here
calculated at 3.37, indicating a stiff lesion.

Fig. 83.10: Shear wave elastography. A scirrhous breast cancer

shows as a shadowing mass on B-mode in the lower panel. The
shear wave elastogram in the upper panel shows it to be very stiff
(red colors) especially in the periphery where values of 225 kPa
were measured. This system uses ultrafast plane wave imaging to
capture the shear wave at sufficient speed that real time imaging
is possible.

of elastography.2527 Tissue stiffness derives from the

interlinks between tissue structures, especially connective
tissues (and ultimately, bone, though this is not amenable
to ultrasonic interrogation), and this is quite different
to the structures that support the longitudinal waves of
conventional ultrasound. The attraction of elastography is
the huge range of tissue stiffness and the marked changes
that occur with pathology, malignancies, for example,
being stiffer than the host tissue. Two ways to image the
elastic properties of tissue have been developed: strain and
shear wave approaches. In strain elastography, the longer
established of the two, the tissue is displaced, usually by
gentle probe pressure, and the way it deforms is measured
by tracking the changes in the speckle pattern using
conventional ultrasound (Fig. 83.9). Softer tissues displace
more, harder tissues less, and a color overlay indicating
their relative stiffness can be created.
Shear waves are transverse, slow-moving waves that
are generated by any body movements. Their speed is
proportional to the tissues stiffness. The commonest way
to generate shear waves is to use acoustic radiation force
impulses (ARFIs) with high MI ultrasound push pulses
that are akin to those used in Doppler. The resulting
shear waves travel at right angles to the push beam
and their speed can be measured using conventional
or plane wave ultrasound (Fig. 83.10). The shear wave
method has advantages of being applicable in any organ
that is ultrasound-accessible (strain elastography is hard

to use for the heart or abdominal organs) and of being

quantitative, with readouts in meters per second (m/s)
that can be converted to kilopascals (kPa), the standard
unit for elasticity.
Much work has been done in refining the classification
of malignancies in the breast, thyroid, and prostate and for
assessing liver fibrosis; in this application, it is beginning
to reduce the need for liver biopsies.
The mechanical properties of body tissues are extre
mely complex and elasticity also includes a viscosity
component (tissue is stiffer when distorted rapidly) as
well as porosity (exemplified by pitting edema). Some of
these might be amenable to elastography approaches. The
viscosity component results in a spread of the frequencies
of shear waves and measuring this is feasible and could
provide a key to evaluating steatosis of the liver.28

LIGHT AND SOUND

If a laser pulse is focused into soft tissue, the local heating
causes expansion which releases an ultrasound pulse that
can be detected at the body surface, a process known as
photoacoustic imaging.29 Tissue strongly attenuates most
light wavelengths (it is opaque to most colors of light)
but infrared and near-infrared light can penetrate for a
centimeter or so. It is strongly absorbed by hemoglobin
and melanin (endogenous absorbers), and injected
dyes can extend these effects. The precision of the laser

1996

Section 7: Miscellaneous and Other Noninvasive Techniques

pulses gives excellent spatial resolution and both single

ultrasound transducers (with CT-like repositioning) and
arrays have been used to image blood vessels, melanomas,
and myocardial infarctions in small animals. Extension to
superficial tissues in man seems possible.
The complementary approach, acousto-optical ima
ging, relies on the fact that the beam of a laser light passing
through a semi-opaque medium such as tissue is diffracted
when the material is agitated by a traversing ultrasound
beam. A classic implementation is the Schlieren tank used
to visualize the beam from an ultrasound transducer and
the same principle can be used to image tissues and has
been applied to small animals.30 In an extension of this
method, the arrival time of a shear wave can be measured,
and if this is performed at more than one location
(e.g. using an array to generate shear waves from different
locations), the shear wave speed can be measured with
great precision.31

THERAPEUTIC APPLICATIONS
OF ULTRASOUND
Diagnostic ultrasound is used at low powers, partly with
the intention of avoiding heating tissue, but if the power
is increased, heating occurs and this has been exploited in
physiotherapy. It is also effective for promoting healing of
bone fractures and skin ulcers.32
If much higher powers are used, tissue temperatures
can be raised to above 55C and the tissue coagulated.33,34
In this approach, a large transducer with tight focusing
concentrates the beam into a volume of about 2 10
mm; heating takes a few seconds and then the beam is
repositioned to the neighboring location and the steps
repeated across the desired volume that needs to be
ablated. The process is known as highly focused ultrasound
(HIFU) and is widely used, especially in China, to ablate
tumors, both benign and malignant as well as for pain
control in cancer of the pancreas.35 In many countries,
HIFU is officially recommended for prostate disease,
both benign hypertrophy and localized cancer. It has the
advantage of being noninvasive and very precise: the heat
coagulation completely spares tissue only a few cells away
from the coagulated region. Its disadvantage is that it is
slow, though ways to speed it up are being investigated.
Ultrasound is being developed as a means to allow
drugs to cross the skin barrier for transdermal drug
delivery.36 Low-frequency ultrasound transiently loosens
the squamous layer of the epidermis (probably by
cavitation) so that even large molecules such as insulin

and heparin can be delivered. This has the potential for

continuous administration of these drugs that otherwise
must be injected, with the expectation of a bettercontrolled therapeutic effect. The process also works in
the opposite direction such that interstitial fluid samples
can be obtained for analysis, for example, of glucose levels.
Ultimately, the combination could facilitate a feedback
loop to maintain excellent round-the-clock blood sugar
control.
Microbubbles and nanodroplets can have ligands
attached and can carry payloads such as nucleic acids37
and chemotherapeutic drugs for targeted delivery, with the
expectation that tissue side effects would be minimized.
Short of this development, the coadministration of
conventional microbubbles and thrombolytic drugs
such as tissue plasminogen activator (tPA) and plasmin
has been used with sonication of the relevant cerebral
artery (usually the middle cerebral, but alternatively the
coronaries). Diagnostic ultrasound alone accelerates
thrombolysis, but the combination is more effective and
clinical trials are underway.38 Remaining problems are
the risk of hemorrhagic stroke and the persistence of
ischemic damage to the downstream microvasculature.
In myocardial infarction, this manifests itself as poor
myocardial perfusion at the microvascular level despite
restoration of flow in the main coronary arteries.
The therapeutic uses of microbubbles and derivatives
seem likely to become more important clinically than their
diagnostic value.

CONCLUSION
The prospects for continued clinically significant develo
pments in ultrasonography are strong with many inno
vations in the offing. The area continues to surprise us with
innovations in diagnostics while therapeutic opportunities
are also on the horizon.

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compounding for very high frame rate ultrasonography and
transient elastography. IEEE Trans Ultrason Ferroelectr
Freq Control. 2009;56(3):489506.
2. Shattuck DP, Weinshenker MD, Smith SW, et al. Explo
soscan: a parallel processing technique for high speed
ultrasound imaging with linear phased arrays. J Acoust
Soc Am. 1984;75(4):127382.
3. Tanter M, Bercoff J, Sandrin L, et al. Ultrafast compound
imaging for 2-D motion vector estimation: application to
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4. Tanter M, Bercoff J, Athanasiou A, et al. Quantitative

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5. Kawai J, Tanabe K, Matsuzaki M, et al. [Validation of a new
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7. Chen, J, R Panda, and B Savord. PureWave crystal techno
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Ultrasound/Solutions/technologies/Philips_PureWave_
crystal_technology.pdf.
8. Shrout T, Zhang S. Lead-free piezoelectric ceramics: alter
natives for PZT? J Electroceramics. 2007;19(1):11326.
9. Oralkan O, Ergun AS, Johnson JA, et al. Capacitive micro
machined ultrasonic transducers: next-generation arrays
for acoustic imaging? IEEE Trans Ultrason Ferroelectr Freq
Control. 2002;49(11):1596610.
10. Yu Z, Blaak S, Chang ZY, et al. Front-end receiver electr
onics for a matrix transducer for 3-D transesophageal
echocardiography. IEEE Trans Ultrason Ferroelectr Freq
Control. 2012;59(7):150012.
11. Linzer, M. UITC Symposium. 2013; Available from: http://
uitc-symposium.org/index.html.
12. Kuroda H, Kakisaka K, Kamiyama N, et al. Non-invasive
determination of hepatic steatosis by acoustic structure
quantification from ultrasound echo amplitude. World J
Gastroenterol. 2012;18(29):388995.
13. Braeckman J, Autier P, Garbar C. Computer-aided ultraso
nography (HistoScanning): a novel technology for locating
and characterizing prostate cancer. BJU Int. 2007.
14. Sandulescu DL, Dumitrescu D, Rogoveanu I, et al. Hybrid
ultrasound imaging techniques (fusion imaging). World J
Gastroenterol. 2011;17(1):4952.
15. Sykes A, Appleby M, Perry J, et al. An investigation of the
bacteriological contamination of ultrasound equipment.
Br L Infect Control. 2006;7(4):1620.
16. Osmanski BF, Pernot M, Montaldo G, Bel A, Messas E,
Tanter M. Ultrafast Doppler imaging of blood flow dyna
mics in the myocardium. IEEE Trans Med Imaging. 2012;
31(8):16618.

17. Evans DH, Jensen JA, Nielsen MB. Ultrasonic colour
Doppler imaging. Interface Focus. 2011;1(4):490502.
18. Piscaglia F, Nolse C, Dietrich CF, et al. The EFSUMB
Guidelines and Recommendations on the Clinical Practice
of Contrast Enhanced Ultrasound (CEUS): update 2011
on non-hepatic applications. Ultraschall Med. 2012;33(1):
3359.
19. Claudon M, Dietrich CF, Choi BI, et al; World Federation for
Ultrasound in Medicine; European Federation of Societies
for Ultrasound. Guidelines and good clinical practice
recommendations for Contrast Enhanced Ultrasound
(CEUS) in the liverupdate 2012: A WFUMB-EFSUMB

1997

initiative in cooperation with representatives of AFSUMB,

AIUM, ASUM, FLAUS and ICUS. Ultrasound Med Biol.
2013;39(2):187210.
20. NICE, dg. SonoVue (sulphur hexafluoride microbubbles)
contrast agent for contrast- enhanced ultrasound imaging
of the liver. 2012.
21. Pochon S, Tardy I, Bussat P, et al. BR55: a lipopeptide-based
VEGFR2-targeted ultrasound contrast agent for molecular
imaging of angiogenesis. Invest Radiol. 2010;45(2):8995.
22. Kiessling F. Science to practice: exploring new indications
for molecular US imaging. Radiology. 2013;267(3):6612.

23. Smeenge M, Mischi M, Laguna Pes MP, et al. Novel
contrast-enhanced ultrasound imaging in prostate cancer.
World J Urol. 2011;29(5):5817.

24. Dayton PA, Zhao S, Bloch SH, et al. Application of
ultrasound to selectively localize nanodroplets for targeted
imaging and therapy. Mol Imaging. 2006;5(3):16074.

25. Bamber J, Cosgrove D, Dietrich CF, et al. EFSUMB
guidelines and recommendations on the clinical use of
ultrasound elastography. Part 1: Basic principles and
technology. Ultraschall Med. 2013;34(2):16984.
26. Cosgrove D, Piscaglia F, Bamber J, et al. EFSUMB guidelines
and recommendations on the clinical use of ultrasound
elastography. Part 2: Clinical applications. Ultraschall Med.
2013;34(3):23853.
27. Gennisson JL, Deffieux T, Fink M, et al. Ultrasound elasto
graphy: principles and techniques. Diagn Interv Imaging.
2013;94(5):48795.
28. Song P, Urban M, Manduca A, et al. Comb-push ultrasound
shear elastography (CUSE) with various ultrasound push
beams. IEEE Trans Med Imaging. 2013.;32(8):143547.
29. Lai P, Xu X, Wang LV. Ultrasound-modulated optical tomo
graphy at new depth. J Biomed Opt. 2012;17(6):066006.
30. Lerosey G, Fink M. Acousto-optic imaging: merging the
best of two worlds. Nature Photonics. 2013;7:2657.
31. Cheng Y, Li R, Li S, et al. Shear wave elasticity imaging
based on acoustic radiation force and optical detection.
Ultrasound Med Biol. 2012;38(9):163745.

32. Patrick MK. Ultrasound in physiotherapy. Ultrasonics.
1966;4:1014.
33. Barkin J. HIFU: Definitely ready for prime time. Can Urol
Assoc J. 2011;5(6):4223.
34. Dubinsky TJ, Cuevas C, Dighe MK, et al. High-intensity
focused ultrasound: current potential and oncologic
applications. AJR Am J Roentgenol. 2008;190(1):1919.
35. Jang HJ, Lee JY, Lee DH, et al. Current and Future Clinical
Applications of High-Intensity Focused Ultrasound (HIFU)
for Pancreatic Cancer. Gut Liver. 2010;4 Suppl 1:S5761.
36. Polat BE, Hart D, Langer R, et al. Ultrasound-mediated
transdermal drug delivery: mechanisms, scope, and
emerging trends. J Control Release. 2011;152(3):33048.
37. Koike H, Tomita N, Azuma H, et al. An efficient gene
transfer method mediated by ultrasound and microbubbles
into the kidney. J Gene Med. 2005;7(1):10816.
38. Barlinn K, Barreto AD, Sisson A, et al. CLOTBUST-hands
free: initial safety testing of a novel operator-independent
ultrasound device in stroke-free volunteers. Stroke.
2013;44(6):16416.

CHAPTER 84
A Primer on Cardiac MRI for the
Echocardiographer
Madhavi Kadiyala, Aasha S Gopal

Snapshot

Quantave Le and Right Ventricular Assessment

Strain Assessment

Le Ventricular Structure

Myocardis and Sarcoidosis

Cardiac Hypertrophy

Cardiomyopathies

INTRODUCTION
Significant progress has been made in the area of
cardiac imaging since the days of M-mode and B-mode
echocardiography. Advances in computed tomography (CT)
and magnetic resonance imaging (MRI) technology, along
with three-dimensional (3D) echocardiography enable the
modern cardiologist to make clinical diagnoses with high
accuracy and precision. Until recently, myocarditis was a
presumptive diagnosis in a patient presenting with elevated
cardiac troponins and normal coronary arteries. Today,
accurate diagnosis of myocarditis and the extent of myocardial
involvement can be made without invasive procedures using
cardiac MRI. This chapter discusses the practical applications
and limitations of cardiac MRI in various cardiac conditions.
A glossary of common cardiac MRI terms is included at the
end of this chapter.
While echocardiographic imaging is the first line of
diagnostic testing for most cardiac conditions, cardiac
MRI is valuable, when it is important to assess:
1. Tissue characterization: myocardial infarction, inflammation, and fibrosis
2. Accurate quantitation of cardiac chambers

Velocity Mapping, Flow and Shunt Assessment

Valvular Heart Disease and Prosthec Valves

Pericardial Disease

Normal Variants and Masses

Limitaons of Cardiac MRI and CT

3. Cardiac masses and normal structural variants

4. Intracardiac shunts
5. Pericardial pathology.
While cardiac MRI can be very useful in many
conditions, its value is limited in some situations, for
example, patent foramen ovale, small mobile masses such
as vegetations, calcium, etc. Flow quantitation in valvular
heart disease is also possible. However, only imagers
with expertise in this area should interpret this useful
technique due to the many limitations of the methodology.
Additionally, there are technical and patient-related
factors that influence the image quality, thus affecting the
overall utility of cardiac MRI. In most situations, since it is
the echocardiographer who recommends MRI imaging, it
is important to understand the advantages and limitations
of cardiac MRI.

QUANTITATIVE LEFT AND RIGHT

VENTRICULAR ASSESSMENT
Accuracy of echocardiographic quantitation of the left
ventricle is highly dependent on the image quality and
can be highly variable due to uncertainty of the location of

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

the imaging plane in 3D space. Cardiac MRI is considered

the gold standard for quantitative assessment of the
ventricles due to high accuracy and reproducibility in
measuring ventricular volumes and ejection fraction.
The most common method uses a stack of short-axis
images acquired through the body of the ventricles
(Fig. 84.1). Analysis is performed using the Simpsons
method, where the endocardial area from each slice is
measured and multiplied by the interslice distance. The
volumes are traced in end-diastole and end-systole, and
ejection fraction is calculated. Automated software packages
are available that assist the image analyst in computing
accurate volumes and function in 10 to 15 minutes.
Recent advances in 3D echocardiographic methods
enable quantitative assessment with less variability compared to two-dimensional (2D)-based methods. However,
the accuracy of 3D echo quantitation is highly dependent
on the image quality. In one study, only 22% of clinical
patients had the image quality required for accurate
analysis.1 When compared to MRI, 3D echocardiographic
methods can significantly underestimate left ventricular
volumes, as much as by 67 45 mL in end-diastole and 41
46 mL in end-systole.2 Technical advances continue to
improve the spatial and temporal resolution of 3D echo.
However at the present time, resolving trabeculations
by 3D echo is challenging and can lead to inaccuracies,
particularly when image quality is not optimal. Additionally, most MRI labs trace the endocardial borders
including the papillary muscles in the left ventricular
pool, exacerbating the difference with 3D echo methods.
In evaluation of patients, it is important to use the same
method of quantitation and not use the different imaging
methods interchangeably.

1999

Quantitative assessment of the right ventricle continues to be a challenge with echocardiographic methods.
Due to the complex morphology of the right ventricle,
no single view or imaging plane can provide adequate
information for accurate assessment of the right ventricle.
Although quantitative assessment by 3D echocardiography
is better that 2D-based methods,3 it is not widely available
and the technique needs further refinement before routine
use. Accurate right ventricular quantitation is particularly
important in arrhythmogenic right ventricular dysplasia,
atrial septal defects, anomalous pulmonary veins, etc.
It is standard practice to quantitatively determine RV
volumetrics by cardiac MRI. The inter- and intraobserver
variability by cardiac magnetic resonance (CMR) are very
low4 and allows for serial monitoring of chamber volumes.
Similar to the left ventricle, a short-axis stack of the right
ventricle is used for analysis (Fig. 84.1).

STRAIN ASSESSMENT
The complex myofiber arrangement in the heart allows it
to be a highly efficient pump, such that only 10% to 15%
single fiber shortening is required to generate an ejection
fraction of 65% to 70%. In the midwall, the myocardial
fibers generally have a circumferential layout. The myofibers become obliquely oriented in the endocardium and
epicardium, but in opposite directions, resulting in a perpendicular arrangement of the endocardial and epicardial
fibers. This arrangement leads to a finely orchestrated
deformation sequence in circumferential, longitudinal,
and radial directions. Circumferential shortening is primarily due to midwall fiber shortening. Longitudinal left
ventricular shortening is the result of contraction of the

Fig. 84.1: Epicardial and endocardial contours are semiautomatically traced on a stack of short-axis slices of the left ventricle in
end-diastole (shown) and end-systole. Endocardial area from each slice is measured and multiplied by the interslice distance. Thus,
accurate end-diastolic and end-systolic volumes are computed and ejection fraction is calculated (EDV ESV/EDV) and expressed as
a percentage. To obtain a time volume curve of the cardiac cycle, the endocardial contours need to be traced in all phases of cardiac
cycle.

2000

Section 7: Miscellaneous and Other Noninvasive Techniques

oblique epicardial and endocardial fibers. In general, the

epicardial fiber contraction is the dominant force and the
endocardial fibers shorten passively in the same direction
as the epicardial fibers. Even though the greatest amount
of myocardial deformation occurs in the subendocardium,
this is driven by the subepicardial fibers. In the setting
of ischemia, which initially affects the subendocardium, longitudinal shortening would be affected before
circumferential shortening and radial thickening and
therefore be a more sensitive marker.5,6
Accurate assessment of myocardial deformation has
potential for clinical use; however, reliable measurements
and standardization of the various echocardiographic
methods has been challenging.7 MRI-based tagging was
the first noninvasive technique to reliably assess myocardial strain8,9 and is considered the gold standard.
Tagging involves selective destruction of magnetization in
vertical, horizontal, or grid patterns along the myocardium
and assessing the deformation between these tag lines
(Movie clip 84.1). In general, tag lines do not persist
through the entire cardiac cycle and assessment of strain
in late diastole can be difficult.
The most common method of tagging is spatial modulation of magnetization (SPAMM) and software packages
are available for semiquantitative analysis. The technique can be time consuming and requires acquisition of

specialized sequences, making it difficult to implement

in routine clinical practice. More recently, techniques
such as feature tracking have been developed (Fig. 84.2)
that can assess strain on routine cine MRI images.10 Other
MRI techniques such as strain-encoded (SENC) MRI and
displacement-encoded (DENSE) MRI are being evaluated,
and discussion of these techniques is beyond the scope of
this chapter.

LEFT VENTRICULAR STRUCTURE

When compared to echocardiography, cardiac MRI
provides higher definition images of the left ventricular
cavity and its structure. The papillary muscles, chordal
attachments, and apical anatomy are better appreciated.
This has resulted in increased diagnosis of conditions like
left ventricular noncompaction (LVNC).

Left Ventricular Noncompaction

LVNC is an unclassified cardiomyopathy characterized
by abnormal myocardium with two distinct layers:
noncompacted and compacted layers. The natural
history and the prognosis of this condition are not well
established. Several echocardiographic criteria have
been proposed to diagnose LVNC. Commonly used
echocardiographic methods involve assessing the end-

Fig. 84.2: Strain imaging by CMR. The left panel depicts regional myocardial deformation by harmonic phase analysis (HARP) of
tagged images. The green curve corresponds to the septum. The red curve corresponds to the lateral wall. The right panel depicts
regional myocardial deformation by feature tracking analysis of steady-state free precession (SSFP) cine images. Strain analysis of
seven segments in the four-chamber view is shown.

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2001

Fig. 84.3: Left ventricular noncompaction (LVNC) in a young patient with recurrent episodes of syncope and history of atrial septal
defect repair as a child. 2D echocardiogram showed mildly reduced left ventricular function but missed the increased trabeculation, due
to suboptimal endocardial resolution. Contrast echocardiography was able to identify the abnormal myocardium, although in less detail
than CT or MRI. CT angiography demonstrated normal coronary arteries and increased trabeculations. Cardiac MRI was diagnostic
of LVNC. Pathological LVNC is often associated with congenital heart disease as in this patient; (CT: Computed tomography;
MRI: Magnetic resonance imaging).

systolic ratio of noncompacted to compacted myocardium

(ratio > 2:1 is significant), and presence of color flow in the
deep intertrabecular recesses.11 The number and location
of the trabeculations are taken into account in other
echocardiographic criteria.12 These criteria are considered
too sensitive by some,13,14 and others suggest that the
echocardiographic criteria may be too strict. The sensitivity of cardiac MRI is higher than echocardiography1517
(Fig. 84.3). The cardiac MRI criteria for LVNC use end-

diastolic ratio > 2.3:1 rather than the systolic ratio, as

the myocardium is relaxed in diastole and it is easier to
discern noncompacted myocardium from compacted
myocardium (Figs 84.4A to C).18 MRI studies reveal that
some degree of noncompaction in the left ventricular
apex is very common and does not necessarily indicate
pathology. Due to higher contrast resolution and better
apical resolution of cardiac MRI, it is not uncommon to
find apical trabeculation(Fig. 84.4C). Care should be taken

2002

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 84.4A to C: LVNC: in addition to prominent trabeculations (A), deep recesses (B) are often seen in LVNC; (C) Physiological
hypertrabeculation: note the prominent apical trabeculation in C, which can be seen in healthy subjects. This does not indicate pathology.

to avoid over diagnosis. Petersen et al. found that up to

6 3 myocardial segments can be noncompacted in normal
subjects.19 Patients with pathological noncompaction
(10 3) and dilated cardiomyopathy (DCM) (7 3) often
have a greater number of noncompacted segments,
compared to patients with hypertension, aortic stenosis,
or hypertrophic cardiomyopathy (HCM).

Tissue Characterization
The ability to characterize tissues by using different
imaging sequences is a feature unique to MR imaging. The
sequences that are most commonly used are T1 weighted,
T2 weighted, fat suppression, and gadolinium-enhanced
sequences. Using various MRI sequences (Table 84.1), it

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2003

Table 84.1: Signal Intensity (Brightness on the Images) of Various Tissues in the Common Tissue Characterization Sequences
Used in Cardiac MRI

SSFP

T1w

T2w

Fat Suppression First Pass Gadolinium

(Perfusion)

Gadolinium
Enhancement PSIR

Adipose

Suppressed

Fluid transudate

No

Fluid exudate

Int

Int

Int

No

Hemorrhagic
(acute) extra
cellular
methemoglobin

Hemorrhagic
chronic (deoxyhemoglobin,
intracellular
methemoglobin)

(low in
sub acute)

on early and
late imaging

Thrombus
(chronic)

Patchy fibrosis

Multiple patchy
foci in hypertrophic
segments (HCM > AS)

T1 mapping
techniques

Diffusely increased
SI irrelative to normal
myocardium

T1 mapping
techniques

Thrombus acute/
subacute

Diffuse myocardial fibrosis

Edema (inflammation/
ischemia)

Infarction/
necrosis

None

Patchy Midmyocardial Subepicardial

Non coronary

No

Subendocardial/
Transmural Coronary
distribution

Collagen/amyloid
Calcification

TI scout

No

Iron
Vascularity

Other
Sequences

T2* sequence

(AS: Aortic stenosis HCM: Hypertrophic cardiomyopathy; PSIR: Phase sensitive inversion recovery; SSFP: Steady-state free precession).
Arrows indicate the signal intensity (SI) relative to myocardium. Details of the sequences are found in the text and the glossary.

is possible to reliably identify fat, fluid, thrombus, edema,

infarction, and inflammation in most situations. First
pass gadolinium imaging is used to assess vascularity and
perfusion. Late gadolinium enhancement (LGE) is perhaps
the most useful sequence for tissue characterization.
The location, pattern, and intensity of LGE are helpful in
assessing inflammation, fibrosis, infarction, and infiltrative
patterns.

Myocardial Infarction and

Viability Assessment
MRI with late gadolinium imaging is the gold standard
in the diagnosis and characterization of myocardial
infarction. Increased interstitial space due to myocardial
necrosis and fibrosis in the infarcted segment results in
delayed gadolinium uptake and slow wash out compared

2004

Section 7: Miscellaneous and Other Noninvasive Techniques

to the healthy myocardium.18 This results in increased

signal intensity of infarcted segments on late gadolinium
images (1020 min postgadolinium contrast). MRI is highly
sensitive and small subendocardial infarcts (Fig. 84.5)
can be easily detected on MRI due to the higher spatial
and contrast resolution, even when echocardiography
and SPECT imaging are normal. Histologically confirmed
subendocardial infarcts were detected by LGE-MRI in
92%, whereas SPECT imaging detected only 28%.20
Infarction typically begins in the endocardium and
progresses toward the epicardium. Subendocardial or
transmural involvement in a coronary artery distribution
is characteristic of myocardial infarction (Fig. 84.5).
Microvascular obstruction (Fig. 84.6) in acute myocardial
infarction (MI) is an exception, where there are areas of
hypoenhancement in the subendocardium surrounded by
hyperenhancement. This is due to inadequate penetration
of contrast into the center of the infarction from plugging
of small vessels by thrombus and debris. The transmural
extent of enhancement correlates with the thickness of
the nonviable scar tissue and is related to the extent of
functional recovery after revascularization. In general,
when there is less than 50% transmural enhancement,
the myocardial segment is considered viable suggesting
high likelihood of functional recovery.21 There is excellent

correlation of quantitative assessment of infarct mass by

LGE with positron emission tomography (PET) infarct
size.22

Fig. 84.5: Small recent subendocardial infarction in the circumflex

territory (day 3). Increased signal on T2w images is consistent with
edema. Edema is more extensive than the areas of necrosis seen
on LGE. Echocardiography shows normal regional wall motion.
(LGE; Late gadolinium enhancement).

Fig. 84.6: Anteroseptal myocardial infarction (blue arrows) on late

gadolinium imaging. The red arrowheads point to a large area of
microvascular obstruction.

MYOCARDITIS AND SARCOIDOSIS

Cardiac MRI is considered the gold standard for the
diagnosis of myocarditis (Fig. 84.7). Tissue characterization
is useful in determining the presence and duration of
myocardial inflammation. The location is generally in a
noncoronary distribution and can be patchy or diffuse.
The pattern is often mid-myocardial or subepicardial and
can involve the basal lateral wall or septum. In contrast,
ischemic pathology usually begins in subendocardium
and progresses to the epicardium. Presence of edema and
increased capillary permeability is consistent with the
acute inflammatory process.23
Myocardial edema associated with inflammation can
be seen as global or regional increase in signal intensity
(SI) on T2w images. The sensitivity of detecting edema in
acute phase of myocarditis is high (84%) with a specificity of 75%.24 Increased capillary permeability is recognized by increased contrast uptake on early gadolinium
enhancement (EGE) imaging, which is quantified as at
least 45% increase in signal intensity on postcontrast

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2005

Fig. 84.7: Tissue characterization in acute

myocarditis. Bright areas in T2w image
indicate edema. Increased signal intensity
in postcontrast T1 images (>45% relative
to precontrast) and EGE images indicate
increased capillary permeability. The area
of enhancement in LGE indicates necrosis/
fibrosis (less extensive than extent of edema).
(EGE: Early gadolinium enhancement;
LGE: late gadolinium enhancement).

Fig. 84.8: Myopericarditis: early and late gadolinium enhancement images both show multiple foci of hyperenhancement (green arrows)
in the myocardium, consistent with myocarditis. There is evidence of pericardial enhancement (blue arrows) indicating pericarditis. This
appears to be limited to the basal pericardium. The pericardium in the mid-sax images does not show enhancement. A moderate sized
pericardial effusion is present (star).

T1 images compared to the precontrast images. Higher

signal intensity in myocardium relative to skeletal muscle,
postcontrast (ratio > 4) is also indicative of increased
capillary permeability. Abnormal enhancement on
delayed gadolinium imaging is indicative of irreversible
myocardial damage due to either necrosis or fibrosis.
Delayed gadolinium enhancement occurs when there
is expansion of interstitial space, due to necrosis of
myocardial cells or infiltration. Small areas of necrosis
seen in the acute phase of myocarditis may no longer

be seen over time due to fibrosis and scar contraction

that is smaller than the threshold of the MRI sequences.
Pericardial effusion and associated pericarditis can also be
present (Fig. 84.8).
In sarcoidosis, myocardial inflammation is characterized by noncaseating granulomas. Cardiac involvement can be seen in up to 30% of patients25 with pulmonary sarcoidosis and when present, requires aggressive
treatment. The presence of multiple foci of intense hyperenhancement (Fig. 84.9) in a noncoronary distribution

2006

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 84.9: Cardiac sarcoidosis: intense hyperenhancement in the basal inferolateral wall on late gadolinium enhancement images in
this patient with history of pulmonary sarcoidosis and arrhythmias is suggestive of cardiac sarcoidosis.

(midwall, subepicardial, or transmural) in a patient with

sarcoidosis is consistent with cardiac involvement. It is,
however, difficult to differentiate sarcoid granulomas
from other forms of myocarditis and definitive diagnosis
requires tissue biopsy.

CARDIAC HYPERTROPHY
Cardiac amyloidosis, hypertensive cardiomyopathy,
HCM, storage diseases such as Fabrys disease can all
cause cardiac hypertrophy and may be indistinguishable from one another by echocardiography. Tissue
characterization by MRI is useful in further evaluation
of these conditions.
Echocardiography is usually the reason to suspect
HCM with or without obstruction. Morphological features
of HCM, particularly the distribution of hypertrophy
(septal, midwall, or apical) are well characterized on
routine cine MRI images. Apical HCM may be missed
on echocardiography due to limited apical resolution.
Contrast-enhanced cardiac MRI images often, but not
always, show patchy hyperenhancement in areas of

hypertrophy (Figs 84.10 and 84.11), indicating areas

of fibrosis.26 Myocardial fibrosis or scarring detected
by cardiac MRI occurs in up to 33% to 86% of patients
with HCM. It is suggested that these abnormal areas of
enhancement increase the risk for arrhythmias and sudden
cardiac death.27 Whether patients with extensive areas of
enhancement need prophylactic defibrillators is an area of
active investigation. Abnormal myocardial deformation in
the hypertrophied myocardium can be detected on tagged
imaging (Movie clip 84.2).
While there is no specific pattern of hyperenhancement
in Fabrys disease, midwall hyperenhancement in the basal
inferolateral wall has been described in some series.28
It is uncommon to find areas of intense hyperenhancement in hypertrophy from pressure overload. A
mildly increased heterogeneous signal on LGE imaging is
often noted in hypertensive heart disease, aortic stenosis,
etc. Pressure overload hypertrophy in these conditions
generally results in diffuse myocardial fibrosis, which is
difficult to detect visually. Quantitative methods such as
T1 mapping are being investigated to quantitate diffuse
fibrosis in these conditions.

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2007

Fig. 84.10: Hypertrophic cardiomyopathy: severe hypertrophy of the septal myocardium with markedly abnormal LGE. Patchy areas
of enhancement with multiple foci in the thickened septum are indicative of extensive fibrosis. (LGE: Late gadolinium enhancement).

Fig. 84.11: Apical variant of hypertrophic cardiomyopathy: focal bright enhancement (blue arrows) in the apex on contrast-enhanced
image in a patient with apical HCM. Apical infarctions are also frequently observed due to coexisting microvascular disease. (HCM:
Hypertrophic cardiomyopathy).

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Section 7: Miscellaneous and Other Noninvasive Techniques

Amyloid heart disease has certain unique features by

cardiac MRI, allowing for a noninvasive diagnosis without
biopsy. There is marked expansion of the interstitial space
due to amyloid deposition resulting in hyperenhancement
especially on early gadolinium imaging. The pattern is
frequently diffuse subendocardial, as amyloid deposits
tends to be endocardial initially, but can be more diffuse
as the disease progresses (Fig. 84.12). Biventricular and
atrial involvement are often present. The kinetics of
gadolinium in amyloidosis is unusual due to early wash
out of gadolinium from the blood pool, as a result of its
binding with the amyloid protein in the blood. Anatomic
features such as biatrial enlargement, thickened valves,
and pericardial effusion are evident on the cine MRI
images. Amyloid patients have restrictive diastolic
filling, which is best detected by pulsed wave and tissue
Doppler echocardiography. Echocardiography and tissue
characterization by MRI are usually adequate to make a
reliable diagnosis or to exclude cardiac amyloidosis in
most patients.

CARDIOMYOPATHIES
MRI offers additional information in the diagnosis of
cardiomyopathy beyond size and systolic function. The
role of MRI in dilated and other forms of cardiomyopathies
is discussed below.
DCM may occur in many conditions including viral
myocarditis, alcoholic cardiomyopathy, hemochromatosis,
thyroid disease, familial cardiomyopathy, etc. Most
commonly, DCM is idiopathic and is a diagnosis of
exclusion. It is believed that mechanisms specific to viral

myocarditis and chronic inflammation can eventually

lead to DCM.29 Accurate chamber quantitation is useful
in the initial diagnosis and monitoring of the progression
of DCM. Late gadolinium images can have a variable
appearance in DCM, and in general, is helpful in excluding
ischemic cardiomyopathy. A frequently observed finding
in DCM is a mid-myocardial stripe pattern30 (Figs 84.13A
and B) of enhancement, which is not specific for a particular
etiology. A subepicardial enhancement pattern may also
be present in patients with DCM and myocarditis.31 LGE is
noted in half of patients with biopsy proven myocarditis;
however, an absence of LGE does not indicate absence
of previous myocarditis.32 Cardiac MRI can be helpful
in guiding the endomyocardial biopsy,33 by directing it
toward the pathological segments.
Myocarditis associated with eosinophilia can occur
in a variety of conditions such as eosinophilic myocardial
fibrosis, Loefflers syndrome, Churg-Strauss syndrome,
etc. The pathophysiology involves eosinophilic infiltration
of the endomyocardium, followed by necrosis and fibrosis
along with thrombus formation. Any form of eosinophilic
myocarditis can eventually result in fibrotic contraction
and restrictive cardiomyopathy. Normal ventricular and
apical contraction with characteristic subendocardial
enhancement pattern and apical thrombus on late
gadolinium imaging are the hallmarks of this condition34
(Fig. 84.14). While restrictive diastolic filling of the
ventricles can be appreciated on cine imaging in advanced
cases, diastolic abnormalities are best detected on pulsed
wave and tissue Doppler echocardiography.
Myocardial iron overload is an occasional cause of
cardiomyopathy and can occur in conditions such as
thalassemia, myelodysplasia, and hemochromatosis.
Iron overload-induced cardiomyopathy is reversible
if intensive chelation therapy is instituted in time. T2*
imaging is a specialized gradient echo sequence that can
be used to estimate myocardial and hepatic iron load.35
MRI T2* times correlate well with myocardial iron levels.

VELOCITY MAPPING, FLOW AND

SHUNT ASSESSMENT

Fig. 84.12: Cardiac amyloidosis: diffuse enhancement in both

the ventricles and atria (blue arrows) in a patient with cardiac
amyloidosis. Also present are pericardial and pleural effusions
(orange arrows).

Phase velocity imaging or velocity mapping is an MRI

sequence that is used to assess velocity and flow. In this
sequence, each point of the imaging plane is encoded
with a phase shift, which is directly related to the velocity
in that pixel. Velocity encoding can be applied in plane
(in the direction of flow) or through plane to the flow
(perpendicular to the flow). Through plane images are

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2009

Figs 84.13A and B: Mid myocardial stripe pattern of enhancement on late gadolinium images is seen in this patient with dilated
cardiomyopathy.

Fig. 84.14: Diffuse subendocardial enhancement (green arrows)

in the left and right ventricles along with a thrombus (yellow arrow)
in the left ventricular apex in a patient with Loefflers syndrome.

typically used for quantitative analysis. Similar to the

Nyquist limit, a velocity-encoding window is specified,
which determines when aliasing will occur. Generally,
the velocity-encoding window is set as close as possible
to the estimated peak velocity. This allows for accurate
flow analysis while avoiding aliasing. Instantaneous flow
volume can be calculated by measuring the velocity of
all pixels in an area of interest. Integrating the flow of all
phases of the cardiac cycle yields the flow volume per beat.
Pulmonic (Qp) and systemic flow (Qs) can be calculated
by measuring through plane flow in the proximal pulmonary artery and the ascending aorta (Fig. 84.15).
Echocardiographic estimation of Qp/Qs is often prone to
error due to errors in measuring the diameter of pulmonary

outflow. Error due to inaccurate estimation of area is

unlikely with MRI as the through plane area is mapped
and traced in each frame of the cardiac cycle. Pulmonic
and systemic flow should be equal in the absence of a
shunt and are generally within 10% of each other.
In general, the measurement of Qp/Qs by MRI is
accurate and reliable.36,37 When estimating flow, it is
important that velocity mapping is performed as close
to the center of the magnetic field as possible. The great
vessels can almost always be positioned in the center. Any
errors in flow calculation tend to cancel out when deriving
a ratio; therefore, the estimation of Qp/Qs is generally very
accurate. This is, however, not the case when using velocity
mapping to assess valvular flow. There are several technical
issues that may limit the accuracy of flow quantitation
across the valves. Some of these technical issues can
be addressed by paying attention to proper positioning
of imaging planes, applying background correction
techniques, and rigorous internal validation with flow
phantoms. It is however important to realize that there are
limitations of phase-velocity imaging at the present time
and the data should be interpreted only by experienced
users, who understand the limitations of the method.
Technical improvements in velocity mapping methods are
underway which will allow for reliable routine assessment
flow quantitation.

VALVULAR HEART DISEASE AND

PROSTHETIC VALVES
Cardiac MRI allows for comprehensive assessment of
valve disease, including valvular anatomy, function, flow

2010

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 84.15: Forward flow through the aorta (Qs) and pulmonary artery (Qp). In velocity- mapped images, pixels with velocity in either
direction are represented in grades of black or white (note the opposing colors in the ascending and descending aorta), whereas
stationary objects are represented in gray. The area under the curve is integrated to obtain the flow in either direction.

quantitation, as well as provides accurate quantitative

analysis of the left and right ventricles. The assessment
of the ventricles is important to understand the effect of
the valvular pathology on the heart and to detect
progression of pathology in serial evaluation of a patient.
The role of MRI in the more common valvular conditions
is discussed below.

Aortic Stenosis
In general, direct planimetry of stenotic valve area is the
preferred method of assessing severity of aortic stenosis
by MRI. Valve area can be measured in either cine images
or in through plane velocity-mapped images and has been
shown to correlate well with Doppler echocardiography
and catheterization measures of severity.38,39 A crosssectional image through the leaflet tips in systole obtained
by carefully aligning two perpendicular left ventricular
outflow tract (LVOT) views is used to calculate the valve
area (Fig. 84.16). Peak and mean velocities can be obtained
by velocity mapping. Through planes that are perfectly
perpendicular to the stenotic jet at the tips of aortic leaflets
can be obtained, even in the setting of angulated aortic
roots.
It is also feasible to calculate aortic valve area by MRI
using the continuity method similar to echocardiography.
Through plane velocity maps of the LVOT and planimetered
area of LVOT are obtained by obtaining a perpendicular
plane through the LVOT and peak velocity obtained at the
leaflet tips is used to calculate the aortic valve area.

It has to be noted that the temporal resolution

of velocity mapping sequences (2545 ms) is much
lower than that of Doppler echocardiography (10-fold
higher temporal resolution) and generally results in
underestimation of peak velocities. In addition, magnetic
field inhomogeneities and eddy currents can result
in variability in measurements. For this reason, direct
planimetry of aortic valve area by is preferred over the
continuity method by MRI.

Aortic Regurgitation
Regurgitant jets are seen as flow void on steady-state free
precession (SSFP) images due to turbulence. There is a
modest correlation between the width of the jet and the
size of flow void with severity of regurgitation. However,
severity is often underestimated when using SSFP
sequences. It is often difficult to detect small amounts
of aortic regurgitation and may be missed by SSFP
imaging. Quantitative assessment by velocity mapping
is recommended for assessment of significant aortic
regurgitation.
Quantitative echocardiographic assessment of aortic
regurgitation (AR) can be challenging due to difficulty in
visualizing the vena contracta and proximal convergence
of AR jet. Assumptions involving mitral annular area
calculation limit the use of the continuity equation in
assessing AR. In contrast quantitative analysis by cardiac
MRI, velocity mapping is relatively straightforward.
Through plane flow is measured (Fig. 84.17) above the

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2011

Fig. 84.16: Systolic frames of the aortic valve in long- and short-axis images are represented. The flow is in plane in long axis and
through plane in short axis. Aortic valve area can be traced by planimetry in either standard cine (SSFP) images or phase image
(as shown). In this example, it is easier to trace the contours on the phase image compared to the SSFP image. Respiratory artifacts
and dark signal from calcification render the SSFP image technically difficult to trace. (SSFP: Steady-state free precession).

aortic valve and below the level of the coronary artery ostia,
after carefully selecting the plane using two perpendicular
longitudinal views of the ascending aorta. Forward and
reverse flows are quantitated and regurgitant fraction is
calculated as follows.
Regurgitant fraction (%) = Aortic retrograde flow
(mL/beat) 100 Aortic forward flow (mL/beat).
The regurgitant volume may be underestimated by
velocity mapping due to the motion of the valve plane
during the cardiac cycle; however, interstudy reproducibility is high and the method is useful for long-term patient
follow-up.40 Quantitative assessment of left ventricular
volumes and function are equally important in long-term
follow-up. In the absence of other valvular regurgitation,
the difference between left and right ventricular stroke
volumes should equal the amount of aortic regurgitation
and this can be used to quickly corroborate the velocity
mapping analysis.

Fig. 84.17: Quantitation of aortic regurgitation: flow below the

baseline is forward flow in systole. Flow above the baseline represents reverse flow. In this example, there is holodiastolic reverse
flow consistent with significant aortic regurgitation. The calculated
regurgitant fraction is 39%.

2012

Section 7: Miscellaneous and Other Noninvasive Techniques

Mitral Valve
When assessing mitral valve with cardiac MRI, standard
long-axis SSFP views are generally adequate for assessing
the motion and function of mitral valve. Systematic
evaluation of individual segments of mitral valve can
be done to identify prolapsed segments, when there is a
specific question of an anatomical abnormality (Fig. 84.18).
The resolution is, however, inferior to transesophageal
echocardiography, such that the anatomic detail is less
well appreciated. This is generally true with cardiac MRI
sequences, when assessing any thin structures that move
rapidly like valve leaflets.

Mitral Regurgitation
The major contribution of cardiac MRI in the setting of
mitral regurgitation is quantitative assessment of regur-

gitation, ventricular volumes, and function. Quantitative

analysis of mitral regurgitation is performed as follows:
In the presence of single valve regurgitation:
Regurgitant volume is the extra stroke volume (SV) the
affected ventricle has to pump.41 Stroke volumes are
calculated by quantitative analysis of the left and right
ventricles at end-diastole and end-systole
(SV = EDV ESV)
Mitral regurgitant volume = LVSV RVSV (LVSV: Left
ventricular stroke volume; RVSV: Right ventricular stroke
volume)
In the setting of mitral regurgitation, the left ventricle has
to eject a higher stroke volume as it is now pumping into
both the aorta and the left atrium in systole.
In the setting of other coexisting valve regurgitation:
Mitral regurgitant volume = LVSV Aortic forward flow

Fig. 84.18: Multiplane cine MRI images in a patient with posterior mitral valve prolapse and anterior regurgitant jet. By prescribing
multiple planes through a short-axis image of the mitral valve (last frame), it is possible to accurately localize the pathology.
(MRI: Magnetic resonance imaging).

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

In systole, the left ventricle ejects into the aorta and

the left atrium. Aortic forward flow is calculated by the
velocity mapping method. Subtracting this forward
volume from the left ventricular stroke volume yields the
mitral regurgitant volume. This method holds true even in
the setting of coexisting aortic and tricuspid regurgitation.
It is also possible to directly measure the mitral inflow
by through plane velocity mapping of the mitral annulus
(Fig. 84.19). Subtracting the aortic forward volume from
the mitral inflow volume yields the mitral regurgitant
volume. However, the excessive motion of the mitral
annulus during the cardiac cycle results in variable
inflow quantitation and is not the preferred method of
quantitation. Although not routinely performed, it is
feasible to evaluate the pulmonary venous flow pattern to
further assess severity of MR. As each additional sequence
adds to the imaging time, this is not routinely performed.

Mitral Stenosis
In general, echocardiography is the preferred method
of assessing the anatomy and function of the mitral
valve in mitral stenosis. By prescribing through planes
perpendicular to the mitral leaflets it is possible to perform
direct planimetry of the mitral valve orifice. While velocity

2013

and flow calculations are technically feasible, they are

routinely not performed.

Tricuspid Regurgitation
Tricuspid valve anatomy can be assessed in the standard
long-axis views. Qualitative assessment of the tricuspid
regurgitant (TR) jet can be difficult because of less
prominent signal void in the setting of lower velocity
in the right ventricle (Figs 84.20A and B). Quantitative
analysis is useful and is performed using the same
principles as that of mitral regurgitation. The most
common method is
Tricuspid regurgitant volume = RV stroke volume
Pulmonary forward flow (mL/beat).
Quantitation of right ventricular volumes and function
is important in the evaluation of the severity of TR and for
long-term follow-up.

Prosthetic Valves
Despite the prevalent misconception that prosthetic valves
are a contraindication to cardiac MR imaging, almost
all prosthetic valves can be safely imaged at strengths of
1.5 tesla. It should be remembered that the mechanical

Fig. 84.19: Top panel demonstrates estimation of mitral inflow at the level of the mitral annulus. The area under the curve in diastole
yields the mitral inflow. Bottom panel depicts the flow pattern in the left pulmonary vein. The flow is systolic dominant.

2014

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 84.20A and B: Tricuspid regurgitation. The severity of valvular regurgitation is usually underestimated by SSFP sequences,
particularly in the case of right ventricle, where the regurgitant jets are of lower velocity. (SSFP: Steady-state free precession).

forces of the cardiac pump have a much stronger effect on

the prosthetic valve compared to the weak magnetic forces
generated by MRI. Visualization of the actual prosthesis is
often limited by the metallic artifact (Fig. 84.21); however,
this is variable and sometimes excellent imaging is
possible. Evaluation of the prosthetic valves is otherwise
similar to native valves and quantitative flow using velocity
mapping and volumetric analysis of the ventricle is valid in
assessing prosthetic valve function.

PERICARDIAL DISEASE
Echocardiography is the primary imaging modality to
evaluate pericardial disease; however, MR imaging offers
additional information that can help in clarification
of pathology and management. Cardiac MRI methods
allow for excellent tissue characterization, understanding
pericardial function, as well as presence of adhesions.
Normal pericardium is 1 to 2 mm thick (Figs 84.22A
and B) and is generally visualized as a layer of low signal
intensity on dark blood and SSFP images and is often
better visualized along the right ventricle, where there is
usually adipose between right ventricular free wall and the
pericardium. Tagged imaging can be helpful in assessing
slippage of the pericardium. In normal subjects, as
the myocardium contracts and relaxes, it slides past the
adjacent pericardium and slippage is clearly visualized
(Movie clip 84.3). In the setting of adherent pericardium,
the slippage sign is absent (Movie clip 84.4).
Differentiation of pericardial effusion from epicardial
fat can be difficult at times on echocardiography,
particularly if the adipose is extensive and has an atypical

Fig. 84.21: Normally functioning bioprosthetic aortic valve.

Artifacts from the bioprosthetic struts are noted. The bioprosthetic
valve is noted to open normally in systole. Phase velocity images
can be done in plane (three-chamber) and through plane (sax) to
assess flow. Peak velocity can be calculated from through plane
phase velocity images.

distribution. Clear distinction is possible by MR imaging

owing to the different signal characteristics of fat and fluid
(Fig. 84.23). Additionally, when assessing for pericardial
effusion, MR imaging allows accurate determination of the
size and extent of the effusion. The type of effusion can be
reasonably determined by assessing the signal intensity of

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2015

Figs 84.22A and B: Normal pericardium is identified by a layer of low signal between the layers of epicardial fat which has bright signal
on cine and LGE images. Pericardium along the right ventricle is better visualized.

Fig. 84.23: Pericardial cyst is noted adjacent to the left ventricle (). Fluid has bright signal on SSFP images and has a higher signal
on T2w (T2 > T1) images and short tau-inversion recovery (STIR) sequences. On phase sensitive inversion recovery LGE sequence,
fluid appears dark. In contrast, pericardial fat (blue arrows) has bright signal on SSFP, T1 weighted, and LGE images. Additionally,
signal from fat is suppressed on fat suppression and STIR sequences. (LGE: Late gadolinium enhancement; SSFP: Steady-state free
precession).

2016

Section 7: Miscellaneous and Other Noninvasive Techniques

the effusion in various sequences. Moreover, tagged images

may be helpful in assessing the fluidity and composition.
For example, in the setting of free-flowing fluid, tag lines
fade away quickly within the effusion (Movie clip 84.5).
On the other hand, when the effusion is organized or is
less fluid, the tag lines tend to persist longer. The effects
of the effusion on the cardiac chambers such as chamber
collapse and abnormal septal motion can be appreciated
on cine images similar to echocardiography. Importantly,
the presence or absence of pericardial inflammation
can be determined (see Fig. 84.8) using cardiac MRI
sequences. In the setting of active inflammation, there is
increased signal on T2w images and hyperenhancement
on gadolinium-enhanced images. The presence of any
concurrent myocarditis can also be assessed. Pericardial
thickening may or may not be present in pericarditis and if
thickened, may have an irregular appearance.
Chronic inflammation of the pericardium results in
pericardial fibrosis, thickening, and occasional calcification eventually leading to constriction and impaired
diastolic filling of the left ventricle (Figs 84.24A and B,
Movie clip 84.6). Typical features of constrictive pericarditis such as tubular elongated appearance of the left
ventricle and diastolic flattening of the septum can be
easily appreciated on SSFP imaging. Paradoxical septal
motion and variation with respiration can be visualized on
real time imaging sequences. However, it should be kept
in mind that diastolic function and respiratory variation
of inflow are easier to determine with Doppler and
tissue Doppler echocardiography than with cardiac MRI.
Echocardiographic assessment is essential to confirm
constrictive physiology and it is not advised to make a

definitive diagnosis of constrictive pericarditis based on

cardiac MRI findings alone. However, the demonstration
of thickened irregular pericardium and/or adhesions on
tagged imaging by cardiac MRI can corroborate the clinical
and echocardiographic findings to make an accurate
diagnosis. It should be remembered that constriction can
also occur in the absence of pericardial thickening. In this
situation, cardiac MRI is of little value.

NORMAL VARIANTS AND MASSES

Due to its superior spatial resolution and tissue
characterization, cardiac MRI is often used to evaluate
cardiac masses. A full description of cardiac tumors and
their tissue characteristics is beyond the scope of this
chapter. Besides tumors, normal cardiac structures can also
produce mass like appearance on echocardiographic
images. Cardiac MRI is helpful in these situations to
confirm the benign nature of these masses avoiding
further invasive procedures. Extracardiac adipose tissue is a
frequent cause of a cardiac mass on echocardiography and
is clearly discerned by cardiac MRI (Fig. 84.25). Epicardial
fat can be difficult to differentiate from pericardial effusion
by echocardiography, particularly in postoperative
setting. Using tissue characterization techniques such
as fat suppression, the true nature of the masses can
be identified. Occasionally, right atrial masses seen on
echocardiography are due to hypertrophic Eustachian
valves and crista terminalis and their benign nature can be
readily identified with MR imaging (Fig. 84.25 and 84.26).
In addition to fat suppression sequences, which can
detect the presence of adipose tissue, perfusion sequences
are helpful to determine the vascularity of a mass.

Figs 84.24A and B: Pericardial thickening can be clearly seen on dark blood images along the left ventricle in this patient who had
features of constrictive pericarditis (Movie clip 84.6).

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2017

Fig. 84.25: This patient was referred for cardiac MRI for evaluation of multiple right atrial masses on an echocardiogram. There is
extensive amount of epicardial fat that wraps around the right atrium along with lipomatous hypertrophy (blue arrows). Epicardial fat
in the tricuspid annulus often appears as right atrial mass. In addition, there is a prominent eustachian valve (orange arrows). The
eustachian valve is a normal atrial structure, but can be mistaken for a mass, when it is enlarged. A clue for identifying this correctly is
its location at the ostium of the inferior vena cava.

Cardiac thrombi occur more commonly than

tumors. Contrast cardiac MRI is particularly useful in
identifying thrombus. Thrombi are characterized by lack
of enhancement on first pass imaging, early, and LGE
(Fig. 84.27). Intracardiac thrombi generally occur in
relation to infarcted segments and atrial appendages
(atrial fibrillation patients).
The morphological features, location, presence of
adipose tissue, vascularity, and appearance on LGE
images are helpful in characterizing cardiac masses
(Fig. 84.28). While there are no specific diagnostic features
of malignancy, the presence of multiple lesions in multiple
chambers, pericardial involvement, signal heterogeneity,
areas of hemorrhage, necrosis, hemorrhagic pericardial
effusion, or infiltration into other tissue layers indicate

a higher likelihood of malignancy. There is significant

overlap of tissue characteristics of the various types of
tumors, such that specific tissue diagnosis is not made
based on cardiac MRI findings alone. Cardiac MRI is
extremely helpful to evaluate the initial tumor burden and
extent, as well as in the follow-up of tumors.

LIMITATIONS OF CARDIAC MRI AND CT

Cardiac MRI is a powerful diagnostic tool in the cardiologists armamentarium. However, it is important to
recognize the limitations of this modality. Patients with
pacemakers and defibrillators cannot generally have an
MRI. Cardiac MRI studies are usually long studies requiring
patients to be supine and hold their breath. It is difficult to

2018

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 84.26: The mass noted in the right atrium on the echocardiographic images was demonstrated to be hypertrophic crista-terminalis
(blue arrows) and eustachian valve (orange arrows). Notice the thickened crista along the posterior wall of the right atrium that extends
from the inferior to the superior vena cava. This is well characterized on the serial cine planes through the atria.

Fig. 84.27: An unusually large thrombus seen in a young female patient with diabetes, peripheral vasculopathy, nephrotic syndrome
admitted with sepsis. Echocardiographic images in the upper panel demonstrate a large mass attached to the anterior wall, suspicious
for thrombus. There was severe biventricular dilation and systolic dysfunction. The absence of enhancement on perfusion and late gadolinium enhancement is consistent with thrombus. Additionally, there was diffuse hyperenhancement of the myocardium and abnormal
gadolinium kinetics consistent with cardiac amyloidosis.

Chapter 84: A Primer on Cardiac MRI for the Echocardiographer

2019

Fig. 84.28: Papillary fibroelastoma on the tricuspid valve. Note the low signal of the mass on the cine MRI (SSFP) images due to partial
volume effects. There is no evidence of fat suppression or perfusion. (MRI: Magnetic resonance imaging; SSFP: Steady-state free
precession).

obtain satisfactory studies in patients who are unable to

lie supine, are claustrophobic, or uncooperative. While it
is possible to sedate patients, the images are generally less
than optimal in this situation. Most cardiac MRI images
are segmented, that is, averaged over a few cardiac cycles.
In the setting of arrhythmias with irregular heart rhythms
(e.g. atrial fibrillation, frequent ectopy), the image quality
is suboptimal.
While the image contrast and tissue characterization of
MR imaging is better than echocardiography, the temporal
resolution is much lower (most MR sequences have 2040
frames/s compared to echocardiography, which can
image in the range of 30100 frames/s). The slice thickness
of MR images is usually 5 to 8 mm and the images have
partial volume effects (Fig. 84.28). Generally, slices thinner
than 5 mm do not have adequate signal and are not used.
These limitations may be overcome by improvement in
MR technology and development of newer sequences.
Cardiac MRI is extremely helpful in many conditions;
however, technical considerations may limit its value
in some situations. For instance, neither SSFP nor flow
sequences are sensitive or specific enough for detecting

low-velocity flow across a small patent foramen ovale

(PFO) and cardiac MRI should not be used for the
diagnosis of PFO. Color Doppler and agitated saline
contrast echocardiography is the preferred diagnostic
test. Similarly, when imaging valvular vegetations and
small mobile masses by cardiac MRI, it should be kept
in mind that highly mobile small masses are not well
imaged due to the lower temporal resolution and partial
volume effects. Transesophageal echocardiography is
the preferred method. Another consideration is imaging
calcific structures. In general, calcium has no signal on MR
sequences due to lack of water content and is always dark
on all sequences. Calcification is best imaged on X-ray or
CT imaging.

CONCLUSION
Cardiac MRI has become a standard diagnostic modality
in todays cardiology practice, although availability of this
technique is still limited to a few major centers. It is the
gold standard in the determination of cardiac volumes
and evaluation of right ventricle, particularly when

2020

Section 7: Miscellaneous and Other Noninvasive Techniques

echocardiographic visualization is not adequate. Tissue

characterization by cardiac MRI is extremely useful in
the diagnosis of myocardial infarction, myocarditis,
cardiomyopathies, and cardiac masses. It is important for
the cardiologist and the cardiac imager to understand the
unique advantages and limitations of cardiac MR imaging.

Flow Imaging (Phase Velocity/Velocity Mapping)

This sequence is used to assess velocity and flow. Each point of
the imaging plane is encoded with a phase shift, which is directly
related to the velocity in that pixel. Velocity encoding can be
applied in plane (in the direction of flow) or through plane to the
flow (perpendicular to the flow). This is helpful in determining
Qp/Qs and also in assessing valvular function.

GLOSSARY OF CARDIAC MRI SEQUENCES

Cine Imaging
Steady-State Free Precession (SSFP)
This is the work horse cardiac MRI sequence. Images have a very
high signal-to-noise ratio and excellent contrast between the
myocardium and blood. This is a gated sequence acquired over
several cardiac cycles with final display being an average of the
collected data. Respiratory motion and arrhythmia can degrade
the image quality.

Real Time imaging

This is a nongated imaging technique used in the setting of
arrhythmia or respiratory artifacts; however, image quality is
inferior compared to SSFP images.

Dark Blood Imaging (Spin Echo)

Spin echo sequences yield high quality images at a given
time point in cardiac cycle and are generally obtained as
dark blood images. These sequences are very useful for tissue
characterization.
T1 weighted: In this sequence, tissues with short T1 relaxation
time appear bright: Example: adipose tissue (TI ~ 200 ms). Signal
from tissues with long T1 (water > 2000 ms) is suppressed.
T2 weighted: Tissues with long T2 have higher signal in this
sequence. Example: free water has long T2 and therefore will
have high signal.
Fat suppression: In this sequence, signal from fat is suppressed
by applying an additional excitation pulse at the frequency of fat
resonance. This sequence is helpful in tissue characterization.
Short-tau-inversion-recovery (STIR): This is another tissue
characterization sequence with an inversion recovery prepulse
that suppresses signal from fat and has higher signal from water.

T2* (Star) Imaging

This is a special sequence based on signal decay, which is
influenced by the tissue iron content. Tissues with high iron
content have short T2*. This sequence is useful for assessing iron
overload.

Contrast CMR (Gadolinium)

First pass imaging (perfusion): Images are acquired in rapid
succession during the first pass of intravenously injected
gadolinium. This sequence can be used to assess qualitative and
quantitative myocardial perfusion. First pass imaging is also
useful in assessing vascularity of cardiac masses.
Late gadolinium enhancement (LGE)/phase sensitive inversion
recovery (PSIR): This is a T1-dependent inversion recovery
sequence that highlights increased interstitial space, where
gadolinium accumulates and has a delayed washout. Gadolinium
decreases T1 relaxation time and when delayed images are
obtained 10 to 20 minutes after gadolinium, the contrast between
the healthy myocardium and abnormal myocardium (e.g.
infarction, inflammation, fibrosis, infiltration) is at its maximum.
The PSIR sequence further increases the contrast of the LGE
sequence.
Early gadolinium enhancement (EGE): EGE is performed
using the same T1 inversion recovery sequence as in LGE,
but performed earlier (<10 min after gadolinium). It is useful
for imaging increased capillary permeability in myocarditis,
amyloidosis, and thrombus imaging.

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16. Borreguero LJ, Corti R, de Soria RF, et al. Images in
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21. Kim RJ, Hillenbrand HB, Judd RM. Evaluation of myocardial

viability by MRI. Herz. 2000;25(4):41730.
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Cardiol. 2009;53(17):147587.
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27. Moon JC, McKenna WJ, McCrohon JA, et al. Toward
clinical risk assessment in hypertrophic cardiomyopathy
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to dilated cardiomyopathy and coronary artery disease
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31. De Cobelli F, Pieroni M, Esposito A, et al. Delayed
gadolinium-enhanced cardiac magnetic resonance in
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magnetic resonance assessment of human myocarditis:
a comparison to histology and molecular pathology.
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Section 7: Miscellaneous and Other Noninvasive Techniques

35. Anderson LJ, et al. Magnetic resonance for the early

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CHAPTER 85
Cardiac CT Imaging
Satinder P Singh, Sushilkumar K Sonavane

Snapshot

Challenges for Cardiac Computed Tomography

Radiaon Dose

Paent Selecon

Technique

Image Postprocessing

Image Analysis

Pialls and Arfacts

Diagnosc Accuracy of Coronary Computed

Coronary Plaque

Prognosc Informaon from Coronary Computed

Tomography Angiogram

Cardiac Funcon

Myocardial Perfusion

How to Improve Accuracy of Computed Tomography

Angiogram in Determining Flow Liming Disease

Clinical Indicaons

Tomography Angiogram

INTRODUCTION
In the 1980s, the electron beam computed tomography
(EBCT) was introduced and was able to freeze cardiac
motion with a temporal resolution (TR) of 50 ms. EBCT
was used extensively for early coronary artery calcium
(CAC) evaluation. However, its role for CT angiography was
limited due to excessive noise and poor spatial resolution
(SR) from 3 mm to 4 mm thick images. Multidetector
computed tomography (MDCT), which was introduced
in the late 1990s, has rapidly evolved over the past few
years. A 64-MDCT is capable of providing TR of 165 ms
(85 ms with dual source scanner), SR of 0.4, slice thickness
of 0.6, and gantry rotation time of 330 ms or even less
(Table 85.1). MDCT can provide information about
coronary artery patency, coronary calcium, left ventricular
(LV) function, and to some extent even myocardial
perfusion. In addition, extracardiac sources of chest pain

such as esophagitis, pneumonia, pulmonary embolism,

aortic dissection, as well as chest wall abnormalities can
also be evaluated in the same setting. In the last 45 years,
we have also seen dramatic improvement in postprocessing
and segmentation capabilities due to the availability of
powerful computers and their supercomputing powers.
Coronary artery disease (CAD) is a leading cause of
mortality and morbidity in industrialized nations. The
management decisions often depend upon accurate
evaluation of coronary artery lumen. Catheter coronary
angiography is considered the gold standard for coronary
arterial evaluation due to its superior temporal and SR.
Although not insignificant, there is a complication rate
of about 3.6% and a mortality of 0.1% associated with
this procedure.1 It gives accurate luminal information
but lacks information about vessel wall and presence and
type of plaque. Coronary computed tomography (CCT)
angiography not only provides anatomical information

2024

Section 7: Miscellaneous and Other Noninvasive Techniques

Table 85.1: Comparison of Spatial Resolution (SR) and Temporal Resolution (TR) for Different Modalities

Modality

Spatial Resolution (SR) [mm]

Electron beam computed tomography (EBCT)

Temporal Resolution (TR) [ms]

> 0.6

50100

16-Multidetector computed tomography (MDCT)

0.5

250400

64-MDCT

0.4

165250

Dual Source

0.4

83

Catheter Angio

0.2

520

Cardiac magnetic resonance imaging (CMRI)

0.7

20

regarding the coronary artery lumen and presence of

stenosis but also information regarding the vessel wall
and remodeling not seen on conventional catheter
angiography. Due to its near-isotropic image resolution,
cardiac CT is well suited to evaluate complex congenital
heart diseases (CHDs). It provides information about
cardiac chambers, valves, pulmonary and coronary veins,
and any extracardiac pathology in the mediastinum/hilum
or visualized lungs.
Advantages of cardiac CT include its noninvasive
nature, excellent SR, good TR, excellent patient acceptance, wide availability, simple and fast technique, and no
contraindication to existing hardware.

CHALLENGES FOR CARDIAC

COMPUTED TOMOGRAPHY
Small size of coronary arteries, complex cardiac anatomy,
and cardiac motion are the three main challenges for CT
evaluation of heart and coronary arteries. Several factors
defining CT performance include: (a) Volume coverage
per second, defined by detector coverage, pitch, and
gantry rotation time; (b) SR defined by detector size in
longitudinal direction, scan field of view (FOV) and image
matrix in axial plane, and slice reconstruction increment;
(c) TR defined by gantry rotation time and reconstruction
method.

Spatial Resolution
It refers to the ability to resolve as separate forms, small
objects that are very close together. Submillimeter SR with
isotropic imaging (i.e. equal resolution in all three planes)
is desirable to delineate small coronary artery branches.
The SR in CT depends on the size of the three-dimensional
pixels (voxel) in the image as seen on the monitor. The
smaller the size of voxel the less partial volume averaging
and better SR. The voxel size depends on the resolution of
the X-ray sensors and the focal spot size. It may also depend

on the type of material used in the detectors. The SR of

CT is excellent at around 0.40.5 mm and is the primary
strength of CT (Table 85.1). For most practical purposes,
this resolution currently is good enough to evaluate larger
epicardial coronary arteries and their branches. However,
to optimally evaluate calcified smaller coronary artery
branches or patency of coronary artery stents, a SR of
0.2 mm or less is required. Higher SR also increases image
noise and to maintain sufficient signal to noice ratio
(SNR), radiation exposure needs to be increased. Flat
panel volume CT scanners have much higher SR but their
contrast and TR are much inferior to current MDCT.

Temporal Resolution
TR of a CT scanner is determined by the speed of rotation
of the gantry. Since images may be reconstructed from
a 180 rotation rather 360 rotation, the TR is equal to
half the gantry rotation speed. The current generation of
scanners has a very fast gantry rotation time of 0.270.30
ms. Excessive mechanical forces and G forces are the
limiting factors in further increasing the gantry rotation.
Two possible ways to improve TR are multisegment
reconstruction and use of dual source CT scanner.
Multisegment reconstruction selects small portions of
projection data from various heart cycles and combines
all projections to obtain sufficient data for image
reconstruction (TRmax = TR/2 M). However, variable
heart rhythm can cause image degradation due to
misregistration. Dual source CT design uses two X-ray
tubes and two detectors mounted on the gantry with a 90
angular offset and leads to high TR of 83 ms (one-fourth
rotation time; Table 85.1).

Cardiac Gating
Electrocardiography (ECG) gating of the tomographic
images is commonly performed in cardiac CT. Gating
not only minimizes cardiac motion artifacts and allows

Chapter 85: Cardiac CT Imaging

2025

localization of an image to a particular phase of cardiac

cycle but also improves visualization of smaller structures
(Figs 85.1A and B). Both prospective and retrospective
electrocardiography (ECG) gating are available. In
prospective gating, imaging is done in axial scan mode at
a predetermined interval from the preceding R-wave, and
usually image acquisition occurs in late diastole when the
cardiac motion is minimal. Images are obtained every
other heart beat with table moves in between; therefore,
such an acquisition is also known as step and shoot mode.
Since radiation exposure occurs only for a short period in
diastole, this reduces the radiation exposure to the patient.
Prospective gating can only be done in patients with low
and regular heart rates, and since no systolic information
is gathered, cardiac functional analysis cannot be
performed. In retrospective gating, imaging is done in
helical mode and continues throughout the cardiac
cycle. Therefore, the radiation exposure to the patient
is significantly higher. Since both diastolic and systolic
data are captured, functional analysis of the CT data can
be performed to determine ejection fraction (EF), stroke
volume, and ventricular volumes. The accuracy of left and
right ventricular function by MDCT has been validated
with echocardiography and magnetic resonance (MR).24
In addition, qualitative assessment of regional LV function
is performed by visualizing changes in wall thickness
using a cine loop display of multiple cardiac phases
(1090%). Regional wall-motion abnormalities are also
shown to correlate with MR and echocardiography.58

X-Rays have been classified as carcinogens by WHO and

CDC. Per-capita radiation dose from clinical imaging
exams in the United States increased almost 600% from
1980 to 2006. Globally, 93 million CTs per year are done at
16/1,000 persons rate, which includes 58 million CTs yearly
in the United States and 3 million CTs performed annually
in children < 15 years. CT scans deliver almost half of the
estimated collective radiation dose in the United States.9,10
In an article published in the New England Journal of
Medicine, the authors Brenner and Hall predicted that in
a few decades, 1.52% of all cancers in the United States
may be due to the radiation exposure from CT scans
being done now.11 They used dose-response information
from the atomic bomb survivors to calculate the risk for
patients undergoing CT procedures. This claim has been
disputed by American College of Radiology (ACR) using
the following arguments: (a) CT exams result in limited
radiation exposure to the body parts, whereas the atomic
bomb survivors experienced instantaneous radiation
exposure to the whole body; (b) CT exams expose patients
solely to X-rays, while atomic blast survivors were exposed
to X-rays, particulate radiations, neutrons, and other
radioactive nuclei. Therefore, the known biological effects
are very different for these two scenarios. Nonetheless,
ACR supports the as low as reasonably achievable
(ALARA) concept and urges providers to use the minimum
level of radiation needed in such exams to achieve the

RADIATION DOSE

Figs 85.1A and B: (A) Nongated versus (B) Gated axial computed tomography (CT) image. Gated image showed minimal cardiac
motion related artifact, which helps in visualization of small size structures such as coronary arteries. (AO: Aorta; LAA: Left atrial
appendage; PA: Pulmonary artery).

2026

Section 7: Miscellaneous and Other Noninvasive Techniques

necessary results. The typical radiation dose exposure

from commonly performed procedures is show in
Table 85.2.
Two radiation parameters are available from the
CT studythe CT dose index volume (CTDIvol) and
the dose length product (DLP). The DLP is obtained by
CTDIvol multiplied by the total scan length. Effective
dose, measured in milliSieverts (mSv), is calculated by
the DLP multiplied by tissue weighing factor (k) that takes
into account the relative sensitivity of a particular body
region. For cardiac imaging, the most commonly used
k-factor is 0.014. It reflects the relative risk from exposure
to ionizing radiation and accounts for the characteristics
of exposed tissues. The effective radiation dose in mSv
allows us to compare different forms of radiation such as
X-rays and isotope exposures in nuclear medicine studies
(Table 85.3).

Methods to Reduce Radiation

Exposure (Table 85.4)

Individualized protocols: The technique of computed

tomography coronary angiography (CTCA) is
particularly amenable to dose optimization by
tailoring the scan protocol to the individual patient.
Adjustments are made to the tube current (mAs)
and tube voltage (kVp) according to patient size and
anatomical shape. The range of mAs (400800) and
kVp (80120) are utilized in our practice.
The CT radiation dose changes in proportion of
square of the tube voltage. Thus, small changes in
tube voltage have more impact on the radiation
dose. In the PROTECTION I study, the use of the
100-kV tube voltage protocol was associated with
53% reduction in radiation dose, when compared

Table 85.2: Typical Effective Doses of Common Examinations

Modality

Effective Radiation Dose (mSv)

Chest radiograph (CxR) PA/Lateral

0.020.04

Mammogram

0.4

Head computed tomography (CT)

13

Chest computed tomography angiogram (CTA) for (pulmonary

thromboembolism (PTE)

810

Abdomen CT routine

1012

Multiphase abdomen/pelvis CT

2234

Catheter Angio

37

SPECT

(79) Rest or Stress only

(1823) Rest and Stress

Coronary CTA

613 (retrospective)
15 (prospective), < 1 (High Pitch)

Thallium 201

2123

Trans Atlantic Flight

3.0

Average US background

3.64.5

Table 85.3: Computed Tomography Radiation Dose Descriptors

Volume computed tomography (CT) dose index

CT dose index volume (CTDIvol) [mGy]

Average dose within the scan volume (CTDI/Slice)

Dose length product

Dose length product (DLP) [mGy.cm]

Integrates CTDI vol over the scan length (DLP/Scan)
Reflect biological effects attributable to a complete scan acquisition

Effective dose

E (mSv)

Chapter 85: Cardiac CT Imaging

Table 85.4: Methods to Reduce Radiation Dose in Cardiac

Computed Tomography

Choose correct protocol for a given question

kVp: 80, 100, 120, 140
mA: Lower the better
Dose modulation
Iterative reconstruction (ASIR, iDose, SAPHIRE, VEO)
High pitch computed tomography angiogram (CTA; only
applicable with dual source scanners)

with the conventional 120-kV scan protocol.12

It is important to reduce both tube current and
more so the tube voltage whenever possible while
maintaining the image quality. At our institution
we try to apply 100 kVp to patients with body mass
index (BMI) < 26.
FOV: Since the radiation dose is directly proportional
to the scan length, restriction of FOV in Z-axis to the
heart, just above origin of coronary arteries to just
below the inferior wall of the left ventricle is of vital
importance. Preceding calcium scan if done can be
used as a reference to optimize FOV for computed
tomography angiogram (CTA). In cases where there are
no calcium scans done, we start just below the carina.
This limits excessive exposure and further application
of proper filters limit X-ray scatter toward detectors.
Hausleiter demonstrated that a 1% increase in scan
length was associated with 5% increase in radiation.13
Prospective versus retrospective gating: The current
trend is more in favor of using prospective gating due to
tremendous radiation dose savings. PROTECTION I, a
large international multicenter study in 2009 involving
more than 50 sites reported a mean effective dose of
12 mSv where only 6% of studies were prospectively
gated.13 Whereas a study by Freeman et al. showed
a mean effective radiation dose of 3.39 mSv using
prospective gating.14
High pitch protocols: Several recent studies done on a
dual source scanner using a high pitch of 3.4 (compared
to standard 0.2 in conventional scanners) have reported coronary computed tomography angiogram
(CCTA) acquisition with extermely low radiation dose
of around 1 mSv.1519 This technique is only applicable
for dual source scanners and requires a low heart rate
of < 60 bpm to avoid motion artifacts.

2027

PATIENT SELECTION
The revised 2010 appropriate use criteria for cardiac CT
were published in 2010.20 Use of noncontrast CCT for
calcium scoring is rated as appropriate in intermediate
and selected low-risk patients. CCTA is considered appropriate in: (a) Patients with suspected anomalous coronary
artery, symptomatic patients with low to intermediate
pretest probability for CAD who have normal ECG and
cardiac biomarkers, uninterpretable or nondiagnostic
ECG or equivocal biomarkers; (b) New onset heart failure
with reduced left ventricular ejection fraction (LVEF) in
low to intermediate probability for CAD and preoperative
coronary assessment prior to noncoronary cardiac surgery
and intermediate pretest probability; (c) Patients with
prior equivocal stress testing or when there is discordance
between the stress test and clinical suspicion for CAD;
(d) Evaluation for bypass patency after coronary artery
bypass graft (CABG) and prior left main (LM) coronary
stenting in asymptomatic patients; (e) Cardiac structure
and function category, with appropriate indications
including coronary anomalies, CHD, evaluation of right
ventricle (RV) function, evaluation of LV function when
imaging from other modalities is inadequate or evaluation
of prosthetic heart valves; (f ) Preablation pulmonary valve
(PV) mapping or prior to redo sternotomy in reoperative
cardiac surgery.

Contraindication for Computed

Tomography Angiogram
Some of the contraindications for CCTA include: (a) renal
insufficiency; (b) IV contrast allergy; (c) inability to hold
breath or follow verbal commands; (d) persistently elevated
heart rate, frequent premature ventricular contractions
(PVCs) or premature atrial contractions (PACs). Presence
of atrial fibrillation (AF) is a relative contraindication
depending on the ventricular rate (VR).

TECHNIQUE
Important steps to prepare patients for cardiac CT/
CTE are listed in Table 85.5. A radiologist or cardiologist
should monitor and supervise all CCTA studies for
optimization of protocol and best results. The type of
gating and technical parameters should be chosen based
on the clinical question asked, patient BMI, and heart rate
(Tables 85.6 to 85.11).

2028

Section 7: Miscellaneous and Other Noninvasive Techniques

Table 85.5: Patient Prep for Computed Tomography Angiogram

Labs and screening

For any C/I (renal failure, contrast allergy), note any pacemakers, details of bypass surgery

Oral -blockade

3 days preferred

Patient education and instructions

Explain what to expect

No solid food 4 hours
No coffee or stimulants
Arrive at least 3060 minutes before exam

IV access

Prefer anticubital fossa, at least 20 ga, test flush with saline

Electrocardiography (ECG) electrodes Proper placement

Heart rate

If > 70 bpm, give oral or iv -blocker; ideal close to 60 bpm

Practice breath hold

When patient is on the table

Table 85.6: Coronary Calcium Score Protocol

Scanning Mode

Axial

kV

120

mAs

140240

Rotation time

0.5

Collimation

Table 85.7: Coronary Computed Tomography Angiogram

Protocol on 64 Detector Scanner

Scanning Mode

Helical

kV

80120

mAs

>600

Rotation time

0.4

64 .65

Collimation

64 .625

Pitch

0.2

Pitch

0.2

Field of view (FOV)

220

Field of view (FOV)

220

Filter

CB (standard cardiac)

Filter

CB (standard cardiac), sharper (stent)

Slice thickness

2.5 mm

Slice thickness

0.67 mm

Increment

2.5 mm

Increment

0.33 mm

Acquisition

Helical

Acquisition

Axial

IV contrast

yes, 80100 cc, 45 cc/s

Gating

Prospective

Saline chaser

40 cc at 45 cc/s

IV contrast

none

Gating

Retrospective

Effective Dose

13 mSv

Effective Dose

1015 mSv

Table 85.8: Preablation Pulmonary Valve Mapping

(Prospective Gating)

Slice Thickness

1.4 mm

Increment

0.7 mm

Region of interest (ROI)

Left atrium

Contrast

4080 cc at 4 cc/s*

*Depends on body mass index (BMI) and if patient is getting

ablation the same day.

necessitate evaluation of CCTA on a workstation capable

of two- and three-dimensional (2D and 3D) display.
The interpreting physician must know how to do the
postprocessing and not just rely on processed images by
the technologist. The common reformation methods used
are: multiplanar reformation (MPR), maximum-intensity
projection (MIP), shaded surface display (SSD), and direct
volume rendering (DVR).

Transaxial Images
IMAGE POSTPROCESSING
Complexity of coronary anatomy, cardiac motion, calciumrelated artifacts, and the subtle nature of coronary lesions

Series of 2D images stacked in the longitudinal (Z-axis)

direction in which they are acquired provide minimum
distortion or errors and maximum resolution and
gray-scale rendering. Since tortuous vessels will move

Chapter 85: Cardiac CT Imaging

2029

Table 85.9: Transcatheter Aortic Valve Replacement (Retrospective Gated Chest and Nongated Abdominal/Pelvis Using Same
Contrast Bolus)

Contrast

60100 cc at 33.8 cc/s [depending on renal function and body mass index (BMI)]

Saline chaser

70 cc at same rate as contrast

Bolus technique

With threshold set for 100 H.U. in the ascending aorta

Table 85.10: Contrast Media and Injection

Concentration

300, 320, 370

Flow rate 47 cc/s

Weight/ body mass index (BMI) based verus fixed rate

Phases

Two phase (contrast + saline)

Three phase (contrast + contrast/saline + saline)

Bolus technique

Based on predetermined threshold (usually 150 H.U.)

ROI in ascending or descending aorta

Time bolus

Inject a 15- to 20-cc test bolus and calculate time to peak

Table 85.11: Optimization of Cardiac Computed Tomography

Angiogram

Before

Decrease heart rate (most crucial)

During

Tailor exam according to indication

Breath hold
Do not use dose modulation if large patient or
variable heart rate
Optimum contrast opacification

After

Electrocardiography (ECG) editing

Use optimal W/L
Proper phase selection and postprocessing
method

in and out of plane as the slice thickness is not variable,

it requires the reader to mentally reconstruct the 3D
anatomical relationships of the vessels and other structures
(Movie clip 85.1).

Multiplanar Reconstruction
A plane is defined inside the 3D volume, and only data
in this plane is displayed. It is performed by using either
straight or curved planes; thickness is set to zero as a
default to optimize image quality, but can be changed
(slab MPR). Its advantages include ease of use and speed,
provision of images containing all available information
(all Hounsfield unit values retained), usefulness in
delineating the morphology of the plaque, and its effect on
the lumen and adjacent vessel wall. Several disadvantages
include its operator dependence, prone to introduce false-

positive and -negative stenosis, viewing from multiple

different view points is required (double oblique method),
and only one branch of a vessel is displayed at a given time
(Figs 85.2A and B).

Curved Multiplanar Reformation

This allows to follow the course of a tortuous vessel for
longer distances as it changes direction. It requires the
centerline to be tracked correctly (done manually or
automatically) and, therefore, allows visualization of
the entire course of the vessel in one image (Fig. 85.3).
However, inaccurate centerline tracking may lead to
pseudo lesions (Figs 85.4A and B)

Maximum-Intensity Projection
This involves projection of highest-attenuation voxels
within volumetric data (all points below this value are
ignored). It uses thicker sections to include vessel lumen
and its wall (usually 45 mm for coronary), optimizes
visualization and tracking of contrasted structures and is
especially useful for blood vessel depiction (Fig. 85.5). The
advantages include: visualization of a longer segment of
a vessels course, reducing perceived image noise, good
differentiation between vessels and background, and
finally is useful when metal is present because of decreased
artifacts with this rendering. Loss of lesion information
within the slab volume (as MIP does not provide in-depth
information) and interference from overlapping structures
are its main limitations.

2030

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.2A and B: Double oblique method. A 54-year-old male with chest pain and abnormal stress MPT: Mixed plaque is seen in the
proximal left anterior descending (LAD) and more noncalcified plaque in mid-LAD after the origin of D1 (red arrow). Using the double
oblique method, the area of narrowing can be validated in three different planes.

Volume Rendering
This used to be a very tedious and time-consuming
process, but now with advanced fast computing powers
of modern processors this process is done with one or
few clicks. Due to its capacity to display multiple tissues
and their relationships to one another, VR is useful for
visualizing spatial relationships such as defining the
course of coronary anomalies, presence and course of
the bypass grafts as well as in the analysis of thoracic
CV structures and complex CHD (Figs 85.6A and B). VR
images are impressive and often used for teaching and

illustrations for patients (Movie clip 85.2). VR images still

are somewhat operator-dependent and should not be used
for assessing vessel narrowing, which can be misleading
(Movie clip 85.3).

Virtual Endoscopy
In this technique, the dense contrast within the vessel is
made transparent while the wall of the vessel is opaque,
viewed from the point of view of an observer positioned
within the vessel. It creates dramatic images but its clinical
utility is limited at this time.

Chapter 85: Cardiac CT Imaging

2031

Fig. 85.3: Curved multiplanar reformation (MPR). Centerline tracking allows visualization of the entire opacified vessel in one image.
Most current vendors provide semiautomatic centerline placement with one or two points of seeding.

Figs 85.4A and B: The centerline point shown in green is outside the vessel in image (A) causing an area of false stenosis on the curved
multiplanar reformation (MPR). After correctly placing the seed in the lumen of the coronary artery, the pseudostenosis disappears.

2032

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 85.5: Maximum-intensity projection (MIP) images optimize

visualization and tracking of contrasted structures such as coronary
arteries and aorta. (AO: Aorta).

Figs 85.6A and B: (A) Normal coronary artery. The frontal view demonstrates the most anterior chamber as the right ventricle and right
coronary artery coursing in the right artrioventricular (AV) groove (arrow). The broad pyramidal-shaped right atrial appendage (RAA) is
nicely seen. The aorta (AO) is to the right of the pulmonary artery (PA), which is more anterior and to the left of the aorta. (AO: Aorta;
PA: Pulmonary artery; RA: Right atrium; RV: Right ventricle); (B) The axial volume rendered image shows the anomalous origin of the
right coronary artery from the left cusp (red arrow) with interarterial course (yellow arrow). (L: Left cusp; LA: Left atrium; NC: Noncoronary;
PA: Pulmonary artery; R: Right cusp).

IMAGE ANALYSIS
Coronary artery evaluation should be done using the
standard AHA 17-segment evaluation. The coronary
arteries originate from superior portions of the sinuses
just below the sinotubular junction (Fig. 85.7). The normal
coronary artery origin is often situated at a right angle to
the aortic root wall, whereas anomalous coronary artery
origins are often at acute angles. The left coronary artery
originates from the left sinus of Valsalva and courses
between the right ventricular outflow tract (RVOT) and
the left atrial appendage (LAA). It typically divides into

two major branches, the left anterior descending (LAD)

and the left circumflex (Cx) artery (Fig. 85.8). Sometimes
there is an additional third branch known as ramus
intermedius, which courses between the diagonals
and the obtuse marginal (OM) branches and supply a
territory of the LV that might otherwise be supplied by
a diagonal or OM branch. When the ramus branch is a
large branch, the corresponding diagonal or OM branches
are often small in size. LAD courses in the epicardial
space along the anterior interventricular groove between
the right and left ventricles and supplies blood flow to
the interventricular septum via perforators and to the

Chapter 85: Cardiac CT Imaging

2033

Fig. 85.7: Normal coronary artery origin volume rendering (VR).

Normal coronary arteries originate from coronary sinuses just
below the sinotubular junction at near-right angle. (L: Left cusp;
R: Right cusp).

Fig. 85.8: Volume rendered computed tomography (CT) image of

aortic root and coronary arteries in left anterior oblique (LAO) projection view show the RCA, (LM: Left anterior descending (LAD),
and Cx arteries with their major branches. (AO: Aorta; LV: Left
ventricle).

anterior and anterolateral walls of the LV through diagonal

branches. The branching pattern of LAD is quite variable.
The left circumflex (LCx) courses in the left atrioventricular
groove between the left atrium (LA) and LV, and provides
flow to the lateral and posterior lateral walls of the LV
through OM branches. The right coronary artery (RCA)
arises from the right sinus and courses within the right
atrioventricular groove between the right atrium (RA)
and RV. It supplies acute marginal branches to the free
RV wall. The conus artery typically arises as a first branch
from the proximal RCA and courses around the RVOT and
terminates on the anterior aspect of the heart. In 5060%
of patients, the sinonodal artery arises from the RCA and
courses posteriorly between the aortic root and RA toward
the cavoatrial junction. The RCA continues to the crux
of the heart and gives rise to the posterior descending
artery (PDA), which supplies the posterior aspect of the
interventricular septum. RCA then continues to supply the
posterior LV via Posterolateral (PL) LV branches. Coronary
dominance refers to the supply of the PDA and PL LV
branches. The most common coronary system is right
dominance (> 85%) where both PDA and PL LV branches
are supplied by RCA (Fig. 85.9A). In a left dominant system
(810%), the LCx supplies the posterior LV branches
(Fig. 85.9B). In a codominant or balanced system (510%),
the RCA supplies the PDA and LCx supplies the PL LV
branches (Fig. 85.9C). Myocardial bridging is a congenital
variation where a segment of the epicardial coronary
artery is tunneled through the myocardium (Fig. 85.10).

This is most commonly seen in the LAD and is more

often visualized on CTA studies in comparison to catheter
angiograms. The clinical significance of this variation is
not clear but according to some it may have a protective
effect on the tunneled portion.21 Coronary artery stenosis
should be described in detail and should include its
location, degree, number, quantification, factors affecting
evaluation, any positive (Figs 85.11A and B) or negative
(Figs 85.12A and B) remodeling, and features of plaque
including its size, density, and presence of ulceration.

Proper Phase Selection

In retrospective gated studies, it is important to always
scroll through all phases to select the best phase with the
least motion. All coronary vessels are usually best seen in
diastolic phase image (6080% of RR interval), whereas
RCA sometimes is better seen in the systolic phase
especially in patients with higher heart rates (Figs 85.13
and 85.14).

Extracardiac Findings
It is important for the imager to look at the entire FOV
for any given patient. In our experience we have found
unexpected lung nodules, lymph nodes, pulmonary
embolism, pneumonia, esophagitis, and aortic dissection
(Figs 85.15 to 85.18). In a study of 6,920 patients who
underwent diagnostic CCTA, the authors found presence
of extracardiac findings in almost one fourth of all patients.

2034

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.9A to C: Coronary dominance (A) Right dominance. Right

dominance, seen in the majority of the population is shown in the axial
maximum-intensity projection (MIP) image. Both posterior descending artery (PDA) and PL LV branches are arising from RCA; (B) Leftsided dominance. Left-sided dominant circulation, where the PDA
and PL LV branches arise from the left circulation; (C) Codominance
pattern of coronary circulation. Inferior volumerendered view of
the heart shows the codominance pattern of coronary circulation.
The PDA (yellow arrow) is supplied by the RCA while the PL LV
(white arrow) territory is supplied by the Cx. The codominance
pattern is the second most common circulation pattern after the
most common right circulation pattern.

In their study, several serious diagnosis were missed when

reviewing only the limited FOV images and use of broad
FOV led to more workup and follow-up.22

PITFALLS AND ARTIFACTS

Cardiac Motion Artifact
Lower and steady heart rate is very critical for obtaining a
good quality CTA. Even with the newer 256, 320 detector
as well as dual source scanners, the emerging consensus
is to have heart rate as low as possible preferably close to
60 bpm to optimize image quality. The RCA shows
maximum motion during the cardiac cycle followed by
LAD and Cx (Movie clips 85.4A and B). The TR of current
Fig. 85.10: Myocardial bridge. The proximal left anterior descend- scanners is getting better but is not closer to 50 ms required
ing (LAD) courses through the myocardium (red arrow) for a short to achieve motion-free coronary artery imaging. Misalidistance and then follows the normal epicardial course.
gnment or slab artifact results from a high heart rate,

Chapter 85: Cardiac CT Imaging

2035

Figs 85.11A and B: Coronary computed tomography angiogram (CCTA) 4 mm thick axial maximum-intensity projection (MIP) image
shows tight narrowing in the mid-left anterior descending (LAD; red circle), which was confirmed at catheter angiography. The outer wall
to outer wall size of the vessel at the site of noncalcified plaque is larger as compared to vessel proximal to stenosis suggesting positive
remodeling, which is one of the features of vulnerable plaque.

Figs 85.12A and B: In this young 47-year-old male with acute chest pain, the coronary computed tomography angiogram (CCTA)
demonstrates significant stenosis of the mid-left anterior descending (LAD) near the origin of the septal branch. The outer to outer size
of the vessel in this region is actually decreased in comparison to the proximal vessel suggesting the presence of negative remodeling.
Catheter angiogram confirmed the presence of fibrotic narrowing in the LAD.

2036

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.13A and B: Proper phase selection. In studies done using retrospective gating, the entire cardiac cycle images are available
from 0% to 90% RR intervals and can be reconstructed at 510% increment. Since cardiac motion is minimal in the mid to late diastole,
the left coronary circulation is best visualized during the diastolic phase as shown in these images. The right coronary artery is also seen
best in diastole except in a few instances where it is actually better seen at end-systole.

Figs 85.14A to C: Proper phase selection. RCA in 30%, 40%, and 80% phase. The last image also shows misregistration artifact
(arrow).

irregular heart rate, or presence of arrhythmia and is often

best seen on coronal or sagittal reconstructed images
(Figs 85.19A and B).

preferably exercised to hold her/his breath before image

acquisition. Unlike ECG editing, which can improve image
quality in patients with mild arrhythmia, one cannot do
much with respiratory motion artifacts (Figs 85.20A and B).

Respiration Motion Artifact

With the current scanners, very short 410 seconds breath
hold is required and is easily tolerated by majority
of patients. The patient still needs to be instructed and

Blooming Artifacts
These occur with high attenuating structures such as
coronary stents or calcification due to partial volume

Chapter 85: Cardiac CT Imaging

2037

Figs 85.15A and B: Extracardiac findings. In limited field of view (FOV) computed tomography angiogram (CTA), the right hilar and
subcarinal nodes (arrows) are barely visible at the edge, but become obvious with wide FOV image (B).

Figs 85.16A and B: The right lower lobe pulmonary embolisms (arrow) seen in wide field of view (FOV) image (B) can be missed
altogether if only limited FOV images (A) are reviewed.

Figs 85.17A and B: The nodule in the left upper lobe is difficult to appreciate in image (A) and can mimic an end on vessel. It is easy
to find on the wide field of view (FOV) image (B). The nodule in this patient was proven to be metastasis from renal cell carcinoma.

2038

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.18A and B: Extracardiac findings. A 45-year-old female presented with severe chest pain. A gated cardiac computed tomography (CT) was performed and showed normal coronary arteries but there was severe thickening of the entire thoracic esophagus
(arrows), which is better appreciated in wide field of view (FOV) image (B).

Figs 85.19A and B: Misregistration artifact due to variability in heart rate is better appreciated on coronal image (B, arrow) than the
axial image (A).

averaging and cause excessive blooming and overestimation of coronary luminal narrowing (Figs 85.21A
to C). A dual energy technique can provide images
without calcifications and may prove to be useful in better
assessment of coronary luminal narrowing by enhancing
vessel visualization. Similarly, monochromatic imaging at
different keV has been shown to decrease blooming from
calcium at 140 keV in comparison to lower keV.23 Coronary
stent-related artifacts can be improved by using a sharper
kernel for reconstruction as well as with thinner images,
although at the cost of slightly increased image noise
(Figs 85.22A and B).

Beam-Hardening Artifacts
An X-ray beam passing through a high-density structure
gets attenuated with most of its low energy photons
absorbed. In locations near such high attenuating
material, the X-ray beam maintains its low as well as high
energy photons, resulting in an area of low density in the
image. A common location for such an artifact is LV apex
and posterolateral wall of LV near the descending thoracic
aorta. It is important to recognize this artifact to avoid
misinterpretation (Figs 85.23A and B). Few vendors now
provide beam-hardening correction algorithms.

Chapter 85: Cardiac CT Imaging

2039

Figs 85.20A and B: Respiration artifact. Breathing-related artifacts can degrade computed tomography angiogram (CTA) studies and
cannot be reversed with editing. These images are from the first patient scanned as gated computed tomography (CT) study on 40
detector scanner at our facility. The first image (A) showed a sudden cutoff of the mid-left anterior descending (LAD) which, of course,
was not seen at the subsequent cath. When you look at the lung window images at the same level, respiration motion-related artifact is
clearly visible as a ghost shadow in the pulmonary vasculature (arrow).

Figs 85.21A to C: Blooming artifacts. Calcium-related blooming

artifact (A) remains a problem and is the common cause for calling
overstenosis on computed tomography angiogram (CTA). Some
newer scanners have different detector material, which helps in
suppressing this artifact as shown in image (B). It is not possible
to determine stent patency due to severe metal blooming artifact
in image (A).

2040

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.22A and B: Stent evaluation. Sharper filter or kernel (B) often are very useful to look at the stent lumen and restenosis. The
images are very sharp and show the lumen, although at the cost of slightly increased noise in comparison to standard smooth filter
use (A).

Figs 85.23A and B: Beam-hardening artifact. The hypodensity noted near the LV apex (arrow) in this patient was not visualized on the
coronal images. This artifact can easily be misinterpreted for myocardial hypoperfusion.

DIAGNOSTIC ACCURACY OF
CORONARY COMPUTED
TOMOGRAPHY ANGIOGRAM
CCTA has become a robust and accurate clinical tool for
the noninvasive evaluation of the coronary arteries due

to the high spatial and TR of the current MDCT scanners.

The overall performance of the 64-detector CCTA for
detecting coronary artery stenosis in comparison to
catheter angiography on a patient-based analysis results
in sensitivity, specificity, positive predictive values, and
negative predictive values of 85100%, 64100%, 64100%,

Chapter 85: Cardiac CT Imaging

and 83100%, respectively.2431 Improved TR of the dual

source scanners has increased the number of evaluable
coronary artery segments even in patients with high heart
rates.3238

2041

An acute coronary event often results from plaque rupture

and subsequent luminal thrombosis. It is also now
suggested that most acute myocardial infarctions result
from rupture of nonstenotic vulnerable plaques.41 A
vulnerable plaque is often large in size, is lipid rich,
has an ulcerated irregular surface with areas of spotty
calcifications, and often shows positive remodeling and
active inflammation. Many of the features of vulnerable
plaque can be determined from CCTA (Figs 85.24A and B).
Sato et al. evaluated plaques with CTA in patients with
acute coronary syndrome (ACS) and those with stable

angina, and found that in the ACS group the mean density
of the plaque was 25 15 H.U. whereas it was 71 16 H.U.
in patients with stable angina.42
In their study of plaque evaluation with CT attenuation
values, Sun et al. showed clear accurate characterization
of calcified plaques, but there was a significant overlap
between CT attenuation values of lipid rich and fibrous
plaques.43 The authors concluded that at this time, the
CT attenuation value is not able to accurately distinguish
between lipid-rich and fibrous plaques.
The relationship between coronary artery remodeling
and plaque composition has been studied and reported
by Varnava et al.44 These authors studied 88 male subjects
who died with CAD and examined 108 plaques postmortem; 59% had no or positive remodeling and 41% had
negative remodeling. They found that there was higher
lipid content and macrophage count in plaques with
positive remodeling (a marker of plaque vulnerability).
In another study, Reilly et al. looked at the effect of
statins on human coronary atherosclerotic plaque morphology.45 The authors retrospectively reviewed the arterial sections from native hearts of patients with end-stage
ischemic heart disease who received cardiac transplantation (Tx). Thirty-three of 44 study group patients received
pre-Tx statins, and 11 did not (which served as control).
Both groups were similar in total and low-density lipid
(LDL) cholesterol levels, and available number of arterial
sections per patient. The prevalence of low-grade fibrous

CORONARY PLAQUE
CCTA is excellent at detecting the presence of atherosclerotic plaques and shows good correlation with
intravascular ultrasound (IVUS).39 However, CTs tend
to underestimate the volume of a plaque. Leber et al.
compared plaque assessment by CTA to IVUS and found
that the vessel plaque volume is underestimated by
64-MDCT and its correlation with IVUS was only
moderate.40

Plaque Characterization

Figs 85.24A and B: Plaque characterization. Plaque characterization has been investigated and although lower computed tomography (CT) attenuation plaques are presumed to be lipid rich and those with CT attenuation between 40 and 120 are fibrous, there is
considerable overlap. At this time it is best to use terms calcified, noncalcified, or mixed plaque for CT descriptions. The CT images in
this Figure. belong to two different patients; (A) one with a positive remodeling has noncalcified plaque of CT values of 43, and (B) the
other has noncalcified plaque of CT value of 157 and associated negative remodeling.

2042

Section 7: Miscellaneous and Other Noninvasive Techniques

plaques was much higher (45.7%) in patients receiving

statins than those who were not on statins (11.3%). The
high-grade lesions were found more frequently in the
control group (66.3%) than the study group (34.6%). The
authors concluded that statin therapy substantially
enhances plaque stabilization due to reduction in plaque
inflammation.

PROGNOSTIC INFORMATION
FROM CORONARY COMPUTED
TOMOGRAPHY ANGIOGRAM
The prognostic value of coronary calcification by CT has
been well described but the prognostic value of CCTA
is less well reported and remains a hot topic.46 Several
smaller studies have reported variable outcome from CTA
data.4756 In a recent meta-analysis of 18 studies evaluating
9,592 patients with a mean follow-up of 20 months, the
authors concluded that major adverse cardiovascular
events (MACE) among patients with normal CTA
are rare and there is incrementally increasing future
MACE with increasing CAD by CTA.46 In another study,
Russo et al. compared the prognostic value of CCTA with
that of coronary calcium scoring and clinical risk factors.57
The authors evaluated hard cardiac events in 441 patients
who underwent CCTA and calcium scoring for about
32 months. Patients with normal CCTA had a very low
hard annualized event rate of < 0.89%, in comparison
to 3.89% in patients with any CAD. Obstructive CAD
(P < 0.003) and presence of noncalcified or mixed plaque
(P < 0.0001) were independent predictors of hard events
on multivariate analysis.
In another study of 432 patients followed for 24 months,
van Werkhoven et al. looked at the incremental prognostic
value of CCTA compared with coronary calcium score
alone.58 In this study, multivariate analysis demonstrated
that the extent of disease and plaque characterization were
predictive of cardiac events, specifically plaque burden
and plaque composition provided incremental prognostic
value over clinical variables and coronary calcium scoring.
They also found that 20% of patients with a zero coronary
calcium score had noncalcified plaques and 4% patients
with no coronary calcium had significant CAD stenosis
(> 50%) by CTA, therefore suggesting that coronary
calcification alone may not be adequate to accurately
assess prognosis. James Min et al. looked at the prognostic
value of CCTA for prediction of all-cause mortality in
more than 1,127 patients older than 45 years.55 The patient

population included symptomatic patients, those with

abnormal stress or rest test as well as asymptomatic
ones with peripheral vascular disease (PVD) or multiple
risk factors for CAD. The authors identified several
prognostically valuable CCTA indices based on visual
estimate, modified Duke prognostic CA score, and clinical
coronary plaque score.

CARDIAC FUNCTION
LV function as reflected by the EF is one of the most
important prognostic parameters in patients with CAD. In
patients getting retrospective gating, LV assessment can be
easily performed with CT data available throughout the
cardiac cycle (Fig. 85.25).
In a study comparing cardiac function analysis from
MDCT with echo, single-photon emission computed
tomography (SPECT), and magnetic resonance imaging
(MRI), the authors found that there was agreement and
similar correlation between CT and MR for EF, enddiastolic volume (EDV), ESV, and LV mass parameters.59
The standard deviation of EF difference between CT and
MR was significantly less than that between echo and MR
or between SPECT and MR. The evaluation of RV function
is more challenging not only because of the shape of RV
but also often less than optimal contrast enhancement of
the RV when using CCTA protocol.

MYOCARDIAL PERFUSION
Although CT is currently not the method of choice to
evaluate myocardial perfusion, one can sometimes see
perfusion abnormalities in the myocardium in the resting
state (Figs 85.26A and B).

Stress Myocardial Imaging Using

Computed Tomography
In standard current clinical practice, patients with significant coronary artery stenosis detected on MDCT often
require additional functional assessment to determine
the significance of coronary stenosis, thereby adding more
tests, cost, radiation, and patient inconvenience. With
improved spatial and TR along with decreased scan time
and less radiation exposure, the role of MDCT in evaluating
myocardial perfusion is emerging. Several research cardiac
CT perfusion studies in animals and human subjects have
been published and are proposing MDCT as a useful
alternative modality in the evaluation of myocardial

Chapter 85: Cardiac CT Imaging

2043

Fig. 85.25: Cardiac functions. In retrospective gated CTA, cardiac function can be derived using CT threshold method and can be
displayed in bulls-eye or graphic representation.

perfusion.6065 Since X-rays are attenuated proportional

to contrast concentration in the area of interest, in the
absence of beam-hardening artifacts the hypoattenuated
myocardial area on CT represents decreased perfusion.
Similar to SPECT, one can also use stress induced by
adenosine, ragadenoson, or dipyridamole to accentuate
the differential myocardial blood flow (MBF) between
normal and ischemic areas.
Computed tomographic perfusion (CTP) imaging can
be performed qualitatively with the more widely available
64-detector scanners or more quantitative dynamic
imaging can be done now with 256- and 320-detector
scanners. With the latter, time attenuation curves (TACs)
of the aorta, LV, and myocardium can be constructed
to provide information regarding MBF and myocardial
blood volume (MBV) using mathematical models.63 The
second generation dual source scanners can also be used

in shuttle and high-pitch mode to derive myocardial

perfusion imaging (MPI).64
In a study of 35 patients with a high suspicion of CAD,
Rocha-Filho and his group evaluated CTA alone and in
combination with adenosine stress perfusion CT for the
diagnosis of obstructive CAD on catheter angiography.66
The initial diagnostic performance of CCTA to detect
50% stenosis had a sensitivity of 83%, specificity of 71%, a
positive predictive value of 66%, and a negative predictive
value of 87%. After including the CTP information,
each of these indices improved to 91%, 91%, 86%, and
93%, respectively. Findings were also similar in the 70%
coronary stenosis group. The same group also compared
the CTP data to SPECT after including a delayed 7-minute
prospective CT to determine delayed enhancement and
found similar comparative diagnostic accuracy for 70%
stenotic lesions.67

2044

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.26A and B: (A) Resting myocardial perfusion abnormality. This 45-year-old-male came to the emergency department after
being involved in a motor vehicle collision. He had a routine chest/abdominal/pelvis computed tomography (CT). The representative
image of the chest CT shows a clear area of hypodensity in the mid to distal septum and apex with CT attenuation values of 33 in comparison to remote lateral wall LV myocardium with a CT value of 68. The wall thickness was maintained. These findings are suggestive
of acute myocardial ischemia/infarction and follow-up serial cardiac biomarkers and the electrocardiography (ECG) showed STEMI in
the left anterior descending (LAD) distribution; (B) In chronic myocardial infarction there is a subendocardial hypodense defect (arrows)
in a vascular territory associated with wall thinning and remodeling as shown in image (B).

HOW TO IMPROVE ACCURACY OF

COMPUTED TOMOGRAPHY
ANGIOGRAM IN DETERMINING
FLOW LIMITING DISEASE
In a recent study done in 381 patients using a 320-detector
scanner, Lima et al. have shown the added value of CT
perfusion data to routine CCTA to increase the diagnostic
accuracy of the CTA to define flow-limiting CAD at a lower
radiation dose when compared with conventional invasive
angiogram and MPI combined.68
CT-based fractional flow reserve (FFR-CT) calculation
is another upcoming promising method to determine
noninvasively the significance of any coronary artery
stenosis. Computational fluid dynamics (CFDs) is used
to calculate pressure gradients across stenotic areas on
standard CCTA studies. In a multicenter DeFACTO study,
the authors compared FFR-CT with invasive FFR in 252
patients and found that there was improved diagnostic
accuracy and ability to determine functionally significant
stenosis by using FFR-CT in conjunction with CCTA over
CTA alone (AUC: 0.81 vs 0.68 respectively, P < 0.001).69 This
promising method is currently limited by the long time (up
to several days) taken to compute huge data but hopefully
will be available one day soon for it to be applicable in
clinical practice.

CLINICAL INDICATIONS
Coronary Calcium Scoring
Quantification of CAC burden on CT is shown to be
an independent, noninvasive marker and predictor of
adverse cardiovascular (CV) events in asymptomatic
individuals.70 The risk assessment on CT CAC extends
beyond that provided by the Framingham risk score to a
population with a wide ethnic background.70 Thus, CAC
has the potential to restratify intermediate risk groups into
lower or higher groups.
Prospective ECG-gated cardiac images are acquired
with a slice thickness of 2.53 mm without intravenous
contrast. The FOV extends from just below the carina to
cardiac apex. Coronary calcium is defined as an area of at
least three adjacent pixels in the axial plane in the course
of a coronary artery, with an attenuation threshold value
of 130 H.U. or greater. Three in-axial-plane face-connected
pixels correspond to a minimum lesion area > 1 mm2,
which is used as a reference value in calcium scoring.71
There are currently two CT calcium scoring systems widely
used: the original Agatston method and the volume scoring
method developed by Callister et al.71,72 These images
are postprocessed on a dedicated 3D workstation with
appropriate software that automatically detects calcium
on all CT slices (Fig. 85.27).

Chapter 85: Cardiac CT Imaging

2045

consensus regarding the calcium score beyond which

CCTA should not be performed. Some authors suggest
a value of 600 while others, 1,000. In our opinion what
is more important is the distribution of the coronary
calcium, since a CCTA can still be useful if the calcium is
concentrated in a few coronary segments leaving many
other segments evaluable as shown in Figure 85.28.

Acute Chest Pain Evaluation with

Multidetector Computed Tomography
in the Emergency Department Setting
Fig. 85.27: Coronary calcium score. Calcium scores are calculated by the computer based on selected H.U. threshold calcifications and the numbers tabulated in different vascular distribution
as shown here.

In Agatstons scoring method, the calcium area is

multiplied by a number related to CT attenuation. Partial
volume effects lead to higher peak values for small calcific
lesions but not for large ones. Whereas, the volume
scoring method appears to somewhat resolve the issue of a
section dependent on minor changes in section thickness.
However, excellent correlation has been demonstrated
between the two scoring methods.73 Both methods
calculate lesion-specific scores within the left main, LAD,
left circumflex, and right coronary arteries and provide
total scores for an individual artery as well as total score
across all four arteries.
When CAC scan is performed just preceding the CCTA,
it can serve as a gatekeeper to exclude cases with heavy
calcification because of low diagnostic yield. However,
there is no established absolute number of CAC score
above which CCTA should not be performed. Secondly, it
also serves as a reference scan to tighten the CCTA FOV in
craniocaudal direction.
The expert consensus document by the American
College of Cardiology Foundation and the American Heart
Association regarding the current role of CAC testing
in clinical practice among asymptomatic individuals
states, It may be reasonable to consider use of CAC
measurement in asymptomatic individuals who are at
intermediate risk.74 However, the committee did not find
enough evidence regarding the utility of CAC testing in
further risk-stratifying in those considered at high risk
of developing CAD in the next 10 years.74 There is no

Despite recent advances in medical sciences, the evaluation of acute chest pain presenting in the emergency
department (ED) is difficult and remains a diagnostic
challenge. In a study of more than 10,000 chest pain
ED patients, 2.1% with acute MI and 2.3% with acute
unstable angina were inappropriately discharged.75 Due
to medicolegal issues, many of these patients with chest
pain get admitted unnecessarily for further tests and
investigations leading to billions of dollars of excessive
cost each year.76 In this scenario, a test with a very high
negative predictive value, such as CCT angiography, can
be very useful in effectively triaging this cohort of patients.
A major limitation, especially in acutely ill patients,
is the quality of the images especially if the patients have
tachycardia. Administration of -blockers, which is a
standard practice for CCT angiography, is time consuming,
is limited by contraindications, and may not be effective
in many acutely ill patients. Initial studies of dual source
CT have shown a very good diagnostic accuracy of CCTA,
even with a high heart rate.33,77
Improved assessment of coronary arteries with CT
and easy accessibility and close proximity of ED to such
scanners has increased the interest in MDCT evaluation
of chest pain. With 64-detector CT, sensitivity and
specificity rates of > 90% have been reported with few
nonevaluable segments than with earlier generation
MDCT scanners.28,30,78
A recent blinded prospective study of 103 patients
with chest pain who presented to the ED showed
absence of significant coronary stenosis and presence of
nonsignificant atherosclerotic plaque by MDCT, suggesting
absence of ACS, giving a negative predictive value of
100%.79 In another study, MDCT compared favorably
with radionuclide perfusion imaging for detecting ACS in
92 low-risk patients with chest pain in the ED.75

2046

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 85.28: Coronary calcification. The cutoff coronary calcium is reported to be between 600 and 1,200. However, computed tomography angiogram (CTA) still could be useful if the calcification score for not performing CTA is limited to a few segments and it can
also show noncalcified lesions elsewhere as in these images. This patient had a calcium score of 1,060 causing mostly nonobstructive
disease, but there was additional more significant stenosis in the distal RCA due to mostly noncalcified plaque (red circle).

Meijboom et al. evaluated the role of 64-MDCT in

254 symptomatic patients with an estimated pre-test probability of CAD to be high (N = 105) in 87%, intermediate
(N = 83) in 53%, and low (N = 66) in 13%, using the
Duke Clinical Scoring System.80 The estimated posttest probability of the presence of significant CAD after
a negative CT scan was 17%, 0%, and 0%, respectively,
and after a positive CT scan was 96%, 88%, and 68%,
respectively. Based on these findings, the authors
suggested the usefulness of MDCT in symptomatic
patients with low or intermediate estimated pre-test
probability of having significant CAD. CT did not provide
additional relevant diagnostic information in high pre-test
probability symptomatic patients.
In a prospective randomized trial of 197 patients with
acute chest pain at low risk for ACS presenting to the ED,

Goldstein et al. compared the safety, diagnostic efficiency,

and cost effectiveness of 64-detector CT with standardof-care diagnostic evaluation.81 In 75% of patients,
physicians were able to rapidly triage by immediately
excluding or identifying coronary disease using MDCT.
In the remaining 25% of patients, further nuclear stress
testing was performed due to either nondiagnostic MDCT
scans or the presence of intermediate severity lesions. No
adverse coronary events were identified for 6 months in
patients who underwent MDCT comparable to the other
group with the standard-of-care approach. The average
time in the diagnostic evaluation of patients was 3.4 hours
in the MDCT group compared to 15 hours in the standardof-care evaluation group. The average cost of patients
was modestly lowered $1,872 to $1,586, when the MDCT
algorithm was used.

Chapter 85: Cardiac CT Imaging

Several authors recently have investigated the value

of calcium scoring, especially with EBCT to triage ED
chest pain patients.8284 In one such study of 105 patients
with possible ACS with normal cardiac enzymes and a
nondiagnostic ECG result, Laudon et al. found the overall
sensitivity, specificity, and negative predictive value for
a positive finding (non-zero calcium score) at EBCT to
be 100%, 63%, and 100%, respectively.82 No patient with
a negative EBCT had any cardiac event at 4-month
follow-up.

Techniques of Multidetector Computed

Tomography for Acute Chest Pain
Evaluation in Emergency Department
Two different protocols are described. First is a dedicated
standard CCT angiography protocol, which includes
prospecitve gating and small FOV. The advantage of this
protocol is improved SR to evaluate coronary arteries
with less contrast and radiation dose. The disadvantages
include nonvisualization of other potential causes of chest
pain in the lungs and mediastinum not included in the
FOV.
In the second triple rule out protocol, the entire
chest is scanned using a gated technique, but with a large
FOV leading to increased contrast dose, and increased
scan time. The advantage of this protocol is evaluation of
other potential causes of chest pain as described above.
There is no consensus regarding use of these protocols or
regarding a preference to use these protocols. However, in
a given patient, if there is a strong suspicion for CAD or
the main issue is excluding CAD, a dedicated CTA protocol
is preferred. On the other hand, in patients with atypical
chest pain, without definitive CAD suspicion, a triple rule
out may suffice.
Two recently published large trials ACRIN-PA and
ROMICAT II collectively recruited 2,370 low- to intermediate-risk patients presenting with suspected ACS
to the ED in the United States and compared protocols
including an early CT with traditional care group.85,86 The
authors concluded that there was a significant reduction
in the length of stay and increased discharge rate from the
ED in the CT group without any adverse outcome.
In CT-STAT trial, 699 patients at 16 EDs were
randomized to either CCTA or MPI.87 All of the patients
had symptoms of ischemia but a normal or nondiagnostic
rest ECG, no previous known coronary disease, a low

2047

TIMI score, and no other obvious indicators of ACS such

as elevated biomarkers or arrhythmia. Over 6 months of
follow-up, the patients imaged with CCTA were diagnosed
54% faster than those imaged with MPI (median 2.9 hours
vs 6.3 hours, P < 0.0001). The total costs of care were
38% lower with the CCTA group (median $2,137 vs $3,458,
P < 0.0001), even though the cost of each MPI test itself
was only a little more than the cost of each CCTA ($538
vs $507). The diagnostic strategies made no difference
in MACE rate (0.8% in the CCTA arm vs. 0.4% in the MPI
arm, P = 0.29). The CCTA patients were also exposed to
less radiation than the MPI patients (11.5 mSv vs 12.8 mSv,
P = 0.02).

Anomalous Coronary Artery Evaluation

Coronary artery anomalies are rare (0.31%) and are
often asymptomatic. Malignant form of anomalies can be
symptomatic presenting with syncope, arrhythmia, chest
pain, or sudden death.
MDCT is now considered the gold standard method to
evaluate patients with suspected coronary artery anomalies
with an appropriate use criteria score of A 9.20 Anomalies
of coronary arteries can be classified as anomalous origin,
anomalous proximal course, or anomalous termination
(Figs 85.29 to 85.32). Among the abnormal courses, the
interarterial course is most dangerous and has been
implicated as one of the causes of sudden death in young

Fig. 85.29: Coronary artery anomaly. The most common

anomaly is high origin of the right coronary artery above the sinotubular junction as shown in this image (arrow). (L: Left cusp;
R: Right cusp).

2048

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 85.30: Single coronary artery. In this patient presenting with

atypical chest pain, coronary computed tomography angiogram
(CCTA) showed a large single coronary artery (arrow) arising from
the right cusp and supplying all territories of right and left circulation. Whenever one of the coronary artery is significantly larger
than normal, one should look for obstructive disease in other vessels, coronary artery fistula, or anomalous coronary artery origin
from a low pressure system such as a pulmonary artery.

Fig. 85.31: RCA from left cusp. Sagittal oblique CT image shows
anomalous origin of the right coronary artery from the left cusp
and has interarterial course between aorta and pulmonary artery
(arrows). (L: Left cusp; NC: Noncoronary; PA: Pulmonary artery;
R: Right cusp).

Fig. 85.32: Volume rendering (VR) image clearly shows the empty
right cusp and anomalous origin of the right coronary artery from
left cusp. Due to the proximity of the anomalous vessel to commissure and proximal intramural course unroofing procedure can be
done in such cases with good results. (L: Left cusp; NC: Noncoronary; R: Right cusp).

Fig. 85.33: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA). A 25-year-old female with increasing shortness of breath. Oblique volumerendered image demonstrates
the anomalous origin of the left coronary artery from the pulmonary artery (red arrow). The RCA (white arrow) is much larger.

patients. The prepulmonic course is often seen in patients

with tetralogy of Fallot (TOF) and should be identified
preoperatively so as to avoid directly cutting into it on the
operating table.
Anomalous origin of left main coronary artery from the
pulmonary artery (ALCAPA) is a rare anomaly and often

presents early in life with chest pain or dilated ischemic

cardiomyopathy (Fig. 85.33). Table 85.12 describes the
methods to optimize evaluation of a suspected coronary
artery anomaly.
Coronary artery fistula is rare, commonly congenital,
and can be coronary-cameral fistulas (between coronary

Chapter 85: Cardiac CT Imaging

2049

Table 85.12: Optimization of Anomalous Coronary Artery

Computed Tomography Angiogram Protocol

In a younger age, restrict field of view (FOV)

For interarterial course, show pulmonary valve relation (good
to have some contrast in right circulation)
Volume rendering (VR) images show spatial relationship nicely
Multiplanar reformation (MPR)/thin maximum-intensity
projection (MIP) images needed to document intramural
course and oblique narrow takeoff of the anomalous vessel

artery and cardiac chambers), coronary arteriovenous (AV)

malformation (between coronary artery and low pressure
veins), drainage often to RV, RA, or pulmonary arteries,
and less frequently to superior vena cava (SVC), coronary
sinus, or LA (Fig. 85.34). Rarely, coronary artery fistula
develops from acquired causes such as trauma or invasive
procedures (pacemaker placement, endomyocardial
biopsy) or CABG. Most are small and the patient, asymptomatic. On examination, a continuous murmur can be
heard at the left lower sternal border and the CA branches
proximal to the shunt become enlarged. Smaller fistulas
in children tend to grow with age. If left untreated, they
can cause symptoms in 19% of patients under the age of
20 years and in over 60% of older patients. Small fistulas
in an asymptomatic patient should be followed clinically
for signs of growth and increasing flow. The larger hemodynamically significant fistulas should be closed either
surgically or via transcatheter occlusion techniques
especially if the fistula is short and less tortuous.
Sequelae of coronary artery fistula may include chronic
angina, congestive heart failure (CHF), cardiomyopathy,
myocardial infarction (MI), hypertension (HTN), endocarditis, and rarely fistula rupture.

Preoperative Computed
Tomography Angiogram
When catheter angiography is considered dangerous,
difficult, or contraindicated, such as patients with aortic
vegetations, ascending aortic dissection and those with
massive aortic dilatation making it very challenging to
canulate coronary ostia (Figs 85.35A to C).

Re-do Coronary Artery

Bypass Graft
This is another common indication and can be performed
with or without intravenous contrast depending on the
clinical question (Figs 85.36A and B).

Fig. 85.34: Coronary artery (CA) fistula. LV to OM branch fistula

in a patient after MI is seen on the axial computed tomography
angiogram (CTA) image. (LV: Left ventricle; RV: Right ventricle).

Role in Asymptomatic
Intermediate Risk Patient
Currently, CTA is not indicated for asymptomatic individuals and this is a topic of hot debate. One school of
thought leans toward getting CTA in patients with
significant risk factors including family history of premature atherosclerosis.
In a large multicenter registry study, Chinnaiyan
et al. presented their research data from the advanced CV
imaging consortium (ACIC) in Michigan looking at the
CCTA in asymptomatic individuals.88 A total of 21,573
patients without known CAD participating in ACIC at
43 institutions in Michigan were included and of these
11.8% were asymptomatic and 88.2% were symptomatic.89
The asymptomatic group patients were older, had more
males with higher Framingham risk scores, and higher
frequency of abnormal stress tests (38% vs 21%). CCTA
in these patients showed a lower frequency of normal
coronary arteries (38% vs 51.2%) and higher frequency
of both < 50% and > 50% luminal stenosis. The authors
concluded that the asymptomatic patients had worse risk
profiles and a higher CAD burden on CCTA. These findings
have broad implication on identification of candidates for
aggressive secondary prevention.
The main hurdle in our view has been the issue of
radiation exposure, which is understandable. But with
the recent advances and availability of very low dose CTA
protocols it seems logical and appropriate to use CTA
selectively in such high-risk populations with radiation
exposure close to or < 1 mSv. In fact, with such low-dose
scanning capability, it is possible that in the near future

2050

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.35A to C: Preoperative computed tomography angiogram

(CTA). Preoperative CTA is done when catheter angio is difficult
or dangerous; (A) ascending aortic aneurysm and dissection in
a patient with previous coronary artery bypass graft (CABG);
(B) ascending aortic dissection narrowing the LM ostium (arrow);
(C) aortic valve endocarditis as seen on echo and computed
tomography (CT) images (arrow).

CTA might play an important role as a screening modality

for CAD somewhat similar to where we stand now for
screening of lung cancer using low-dose CT.

Coronary Artery Bypass Graft

Gated cardiac CT is well suited to evaluate patency of
bypass grafts noninvasively, since these grafts are usually
larger diameter structures and there is less cardiac motionrelated artifacts. The reported sensitivity and specificity
for detecting stenosis or occlusion is 95100%.90 With the
newer scanners, the anastomosis can be well evaluated
with less motion, less metal clip hardware artifact, and
better vascular opacification (Figs 85.37A to C). The CTA
protocol needs to include the entire chest, especially
the subclavian arteries if the patient has had internal
mammary grafts. More contrast is often needed and the
breath hold is slightly longer (Figs 85.38A and B).

Coronary Stent Evaluation

The population needing follow-up of PCI is growing
and follow-up of stents with catheter angio is expensive
and carries risks. Currently, clinical symptoms and/or
provocative tests are noninvasive tools with no direct stent
imaging capability available. In a study comparing the
diagnostic accuracy of coronary in-stent stenosis using a
64-detector CT with invasive coronary angiography, the
authors reported high sensitivity, specificity, and negative
predictive values of 92% and 90%, 81% and 79%, and 98%
and 96% per segment/per patient basis, respectively.91 The
positive predictive value, however, was only modest at 54%
and 58%, respectively. Several steps that can be taken to
improve in-stent visualization are the use of higher mAs
to improve contrast resolution, use of high intravascular
attenuation, use of sharper kernel, and lowering of the
heart rate (Figs 85.39A to C).

Chapter 85: Cardiac CT Imaging

2051

Figs 85.36A and B: Redo sternotomy. Performing computed tomography (CT) is an appropriate indication for a patient being evaluated
for redo CABG or noncoronary cardiac surgery. CT can accurately detect the distance of vital structures near the sternum as well as the
presence of any adhesions. In the images here, this patient had developed a pseudoaneurysm (PSA) at the site of aortic cannulation
(arrows) done for cardiopulmonary bypass. Computed tomography angiogram (CTA) clearly showed the close proximity of the PSA to
the sternum, which is useful information for the surgeons in planning a safe surgical approach.

Cardiac Masses
Primary cardiac tumors are rare but metastases to the
heart from other locations are much more common.
Primary benign tumors are much more common than
primary malignant neoplasms. Myxoma is the most
common primary benign cardiac neoplasm in adults.
Most myxomas arise in the LA near the fossa ovalis and
less commonly from the atrial free wall or atrioventricular
valve. Less commonly they can arise in the RA or RV. On
CT, myxomas are round, smooth, and lobulated masses
often with a pedicle and demonstrate patchy postcontrast
enhancement. Often these masses have areas of calcifications as well as low attenuation (Fig. 85.40). On a
retrospective gated CT, the pedunculated myxoma can be
seen to prolapse through the mitral valve. Symptoms from
myxoma are usually due to hemodynamic obstruction,
embolism, or rarely constitutional symptoms. Multiple
cardiac myxomas can be associated with pigmented skin
lesions, extracardiac tumors (schwannoma, pituitary adenomas), and endocrine abnormalities (Carneys complex).
Lipoma is the second most common primary benign
cardiac tumor. It can either arise from the subendocardial
layer or subepicardial/myocardial layer. The endocardial

lipomas are usually small and most commonly seen in

the LV or along the septal wall or roof of the RA. Usually,
these are asymptomatic and detected incidentally on
cross-sectional imaging or rarely can cause symptoms if
large in size, usually by obstructing a cavity, extrinsic mass
effect, or causing arrhythmia. On CT, these tumors are well
defined, noncalcified, and nonenhancing masses with fat
CT attenuation values (Figs 85.41A and B).
Fibroma is the second most common primary benign
tumor of the heart in children after rhabdomyoma. These
tumors are less commonly seen in adults and more
commonly arise from LV than RV in the interventricular
septum or anterior ventricular wall. On CT, they are
homogenous intramyocardial masses, often calcified with
little or minimal enhancement after contrast administration
(Fig. 85.42). Patients can present with dysarrhthymia,
chest pain, or sudden death. Several syndromes are associated with cardiac fibromas including Gardner, Gorlin,
and familial adenomatous polyposis.
Fibroelastomas account for approximately 10% of
cardiac tumors and 75% of valvular neoplasms.92 Most
arise from the endocardium of aortic, mitral, tricuspid,
and pulmonary valves (in decreasing order) and are often
solitary, small nodular masses, mimicking vegetation,

2052

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.37A to C: Coronary artery bypass graft (CABG) volumerendering (VR) image. (A and B) Proximal and distal anastomosis
(circle) with good distal flow from LIMA to posterior descending
artery (PDA) graft; (C) Multiple grafts are seen including left
internal mammary artery (LIMA) and two venous grafts. Metal clips
are actually not causing much metal streak artifact. (LSC: Left
subclavian artery).

Figs 85.38A and B: Coronal oblique computed tomography angiogram (CTA) image (A) shows the opacified LIMA with patent distal
anastomosis with diagonal branch (circle) and distal flow. The corresponding cath angio view (B) confirms these findings.

Chapter 85: Cardiac CT Imaging

2053

Figs 85.39A to C: (A) Mild in-stent narrowing in mid-left anterior

descending (LAD) stent (arrows); (B and C) Stent occlusion in
saphenous venous graft (SVG) graft as shown in two orthogonal
images.

thrombus, and myxoma on imaging. Due to their small

size, these tumors are difficult to visualize on CT. In
comparison to a vegetation, fibroelastoma usually spares
the valve-free margin. They can be detected incidentally
in an asymptomatic patient or rarely can cause symptoms
due to embolization or rarely coronary ostial obstruction.
Metastasis is the most common malignancy of the
heart occurring in approximately 1012% of patients
with extracardiac malignancy.93 Several tumors have
the propensity to metastasize to the heart including
melanoma, lung, breast, thyroid, renal, and esophageal
cancers (Figs 85.43A and B). Tumors can spread to heart
via hematogenous, direct, transvenous, or translymphatic
invasion. Direct invasion occurs usually with lung, breast,
Fig. 85.40: Left atrium (LA) myxoma. Axial computed tomography or other mediastinal tumors and usually manifests as
(CT) image shows a homogenous noncalcified mass (arrow) in pericardial effusion and thickening. Venous extension of
the left atrium attached to the interventricular septum. (AO: Aorta; the renal and adrenal carcinoma occurs via the inferior
LV: Left ventricle RV: Right ventricle).
vena cava (IVC), whereas lung cancer can extend into the

2054

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.41A and B: Lipoma. A large fat-computed tomography (CT) density mass (arrow) is seen in the right atrium extending up to
the superior vena cava (SVC) to right atrium (RA) junction superiorly as seen in image (B). (AO: Aorta; LV: Left ventricle; PA: Pulmonary
artery).

sarcoma occurring more commonly in females between

20 and 50 years of age. The most common site of cardiac
angiosarcoma is RA, while other sarcoma types tend to
occur more on the left side (Figs 85.44A and B). These
tumors appear as irregular nodular soft tissue masses
with infiltration into the pericardium with postcontrast
heterogenous enhancement. They also tend to involve
more than one chamber by direct infiltration. Often there
is associated pulmonary metastasis in approximately
two-thirds of patients.
Pericardial cysts are the result of blind-ended ventral
parietal pericardial recesses. Most patients are asymptomatic and only rarely cause chest pain or dyspnea. The
most common location is cardiophrenic angle, 80% on
the right side. On CT, they appear as well-defined fluid
Fig. 85.42: Cardiac fibroma. There is a calcified intramyocardial
mass arising in the left ventricular lateral wall (arrow). Cardiac density smooth masses, which show no enhancement
after contrast (Figs 85.45A and B).
fibromas are common in pediatric age groups.
Lipomatous hypertrophy of interatrial septum
occurs usually in older obese patients and are mostly
heart via pulmonary veins. Solitary cardiac metastasis
asymptomatic. On CT, the interatrial septum is thickened
is rare and hematogenous metastasis often is associated
> 20 mm with fat and is dumbbell-shaped with sparing of
with other organ metastasis. The masses are often irregular
the FOV (Figs 85.46A and B).
and demonstrate heterogenous enhancement.
Lymphoma of the heart is often secondary rather than
a primary cardiac tumor. Primary cardiac lymphoma is Pulmonary Vein Ablation
often the more aggressive non-Hodgkin B-cell, tends to Atrial fibrillation (AF), the most common sustained
occur at an older age and in patients with HIV infection. cardiac arrhythmia, is a major cause of stroke and the most
Common sites of involvement include pericardium, RA, common cardiac arrhythmia requiring hospitalization.
and AV groove.
AF usually begins as paroxysmal AF, with approximately
Sarcoma is the most common primary cardiac 60% of patients converting spontaneously to normal sinus
malignancy. Angiosarcoma is the most common type of rhythm. Approximately 40% of patients develop persistent

Chapter 85: Cardiac CT Imaging

2055

Figs 85.43A and B: Metastasis. Metastasis from melanoma involving the pericardium (star) aortic root and interventricular septum
(arrow).

Figs 85.44A and B: Angiosarcoma. Angiosarcoma of the heart (star) involving right ventricle (RV) and extending across the right
atrioventricular (AV) to involve the right atrium (RA).

Figs 85.45A and B: Pericardial cyst. A well-circumscribed water density nonenhancing mass is seen along the right atrium (RA).

2056

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.46A and B: Lipomatous hypertrophy of the interatrial septum. Lipomatous hypertrophy of the interatrial septum often spares the
fossa ovalis and produces a dumbbell-shaped appearance as shown in image (red circle). The fatty component can extend into the right
atrial wall to a variable degree and can sometimes encroach on the cavoatrial junction (arrow), although usually without any hemodynamic
consequences. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium: RV: Right ventricle; RVOT: Right ventricle outflow tract).

AF requiring medical or procedural intervention to restore

normal sinus rhythm. Up to 50% of patients develop
recurrent AF within the first year of initial onset.
Radiofrequency catheter ablation of the pulmonary vein
ostia has become the procedure of choice for treating AF.
CT plays an important role in the evaluation of these
patients including pulmonary venous anatomy and any
variation before the procedure, and detecting postablation
complications. Prospective gating is suitable for the
majority of patients except those with a very high heart
rate or obese patients, where either retrospective gating
with tube current modulation or a nongated chest CTA is
an option since motion-related artifacts are not an issue
for pulmonary veins and LA.
The most common normal pulmonary venous pattern
(7580%) is two right and two left pulmonary veins
(superior and inferior) with a separate ostia for each vein.
The pulmonary ostium is defined as the junction point
of the pulmonary vein and LA at the point of parietal
pericardium reflection over the LA. The pulmonary vein
segment from the ostia to its first branch is called pulmonary vein trunk and the region of left atrial wall in between
the two separate ipsilateral pulmonary veins is called the
intervenous saddle. The pulmonary vein ostia and the
intervenous saddle together are known as pulmonary
venous vestibule. Pulmonary venous branching is more
variable than pulmonary artery (PA) branching variations
and includes variation in number, branching pattern, and

length of the pulmonary trunk. MDCT is useful in detecting

these pulmonary vein variations, knowledge of which is
vital for successful and complete ablation procedure.
Paroxysms of AF are initiated by trains of spontaneous
activity originating from the pulmonary veins (9096%),
with almost half arising in the left superior pulmonary
vein.
In most individuals, sleeves of the left atrial myocardium extend into the pulmonary veins for a distance of
217 mm.
The myocardial sleeve of the LA surrounding the
proximal pulmonary venous segment and venoatrial
junction are believed to be the source of initiation and
maintenance of AF.94 Since the myocardial sleeve is
longest around the left superior pulmonary vein, this
vein alone contributes to > 50% of all ectopic beats for AF.
Radiofrequency ablation is performed at or within 5 mm
of the pulmonary vein ostia; therefore, it is important to
know the number of pulmonary veins and branching
pattern to ensure ablation of all ostia. Successful ablation
of all electric connections to these veins can eliminate
paroxysmal AF.
Because radiofrequency energy is preferably applied
at the venoatrial junction of all the pulmonary veins to
avoid stenoses and eliminate ostial remnants that may
contribute to recurrent AF, knowledge of how many
pulmonary veins are present, and their ostial locations, is
important to ensure that all the ostia are ablated. However,

Chapter 85: Cardiac CT Imaging

it is difficult and time-consuming to locate the ostia with

conventional angiography at the time of ablation. It is
important to know ostial orientation and the distance from
each ostium to the bifurcation of each pulmonary vein,
because pulmonary vein narrowing seems to be critically
dependent on catheter position and not on duration of
radiofrequency energy application.
Planning CT is done to determine the number of PVs
and ostia, PV diameter and length to its first order branch,
accessory veins and the presence of any LAA thrombus
(Figs 85.47 to 85.49).

Fig. 85.47: Segmented left atrium posterior view shows the

draining pulmonary veins. (LLL: Left lower lobe; LUL: Left upper
lobe; RLL: Right lower lobe; RUL: Right upper lobe).

2057

Transcatheter Aortic
Valve Replacement
Transcatheter aortic valve replacement (TAVR) is rapidly
becoming a popular alternative procedure for older
patients with severe symptomatic aortic stenosis, who are
at high risk for open surgical repair. Several prosthetic
valves are available including the balloon expandable
Edwards Sapien Valve (Edwards Lifesciences Inc, Irvine,
CA, USA) in 23, 26, and 28 mm sizes and self-expanding
CoreValve (Medtronics Inc, Minneapolis, MN, USA)
available in 23, 26, 29, and 31 mm sizes. In the United
States, only the 23- and 26-mm Edwards valves are
currently approved. The most common implantation route
is transfemoral. If this is not feasible, the Edwards Sapien
valve can be implanted via direct transapical approach or
rarely via a transaortic approach. Currently, this system is
not approved for subclavian artery approach.
Imaging is necessary before the procedure to determine annulus size, annulus area, degree of aortic calcification, access route, and suitable fluoroscopic projection
angles that permit exact orthogonal views onto the valve.
Preprocedural assessment of the aortic root for TAVR
requires knowledge of the orientation of the aortic root
relative to the body axis. For correct deployment of a
percutanous valve prosthesis, the root orientation is
usually assessed by repeated X-ray aortograms in one
or two orthogonal planes. To simplify the procedure, 3D
data sets obtained by MDCT may allow the prediction of
angiographic projections orthogonal to the valve plane
(Figs 85.50A to C).

Figs 85.48A and B: PV ablation. Computed tomography angiogram (CTA) images are loaded into PV ablation software in the cath lab
and are fused with cath images to show the catheter location in relation to the left atrium (LA) and its veins. The electrical map is then
generated and shows as red dots (B), which need to be ablated. (LIPV: Left inferior pulmonary vein; LSPV: Left superior pulmonary vein;
RIPV: Right inferior pulmonary vein; RSPV: Right superior pulmonary vein).

2058

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 85.49: Left atrial appendage (LAA) thrombus. This image

shows a patient with LAA thrombus (*) with chronic atrial fibrillation. Presence of thrombus is a contraindication for an ablation
procedure. (AO: Aorta; PA: Pulmonary artery).

Figs 85.50A to C: Computed tomography angiogram (CTA) for

transcatheter aortic valve replacement (TAVR). CT plays an important role in preoperative planning of TAVR patients, annulus size
(A) and area can be determined along with distance of LM and right
upper lobe (RUL) to annulus (B) and (C).

Chapter 85: Cardiac CT Imaging

CT imaging is the preferred method to evaluate the

aortic root, entire aorta, and iliac as well as common
femoral arteries preferably with single contrast injection.
Since most such patients are older and have some renal
impairment, it is preferable to do complete analysis with a
single contrast bolus. At our institution, we use 70100 cc
of contrast for a retrospective gated chest from arch till
diaphragm and then a nongated CTA of the abdomen
and pelvis, (Table 85.9). Given the advanced age of the
patient, radiation exposure is of lesser concern after some
modifications for minimum recommended vessel lumen
size for different delivery systems.95
Accurate annulus size measurement is very important
in choosing the correct size of the prosthetic valve. A valve
that is too large can cause rupture of the annulus, which
is often fatal and if the valve is too small, there is a risk of
embolization and paravalvular leak.9698

Congenital Heart Disease

CT plays an important role in the evaluation of patients with
known or suspected CHD. With the advances in surgical
techniques and CV anesthesia along with perioperative
care, many patients with complex CHD now survive
into adulthood. Majority of these patients need lifelong
care including reoperations from underlying anatomy or
previous surgical palliative procedures.
Careful preparation is important for CT evaluation
of pediatric and adult CHD. The CT protocol should
be selected on a case by case basis depending on the
suspected anomaly. In neonates/infants, cardiac gating
is a challenge due to higher heart rates. To minimize
other artifacts, we routinely intubate them and suspend
their respiration during the few seconds of nongated CT
scanning. To avoid physical movement, the child is also
given some form of sedation or muscle paralytics. We use
approximately 1 cc/lb of contrast and dilute it with 50%
saline, and the injection is made with hand. A central
umbilical venous catheter (UVC) catheter positioned
just below the cavoatrial junction is preferred, although
a peripheral access also suffices the need for vascular
opacification. In adults, the protocol includes a gated CTA
preferably with controlled heart rate.
A systematic segmental approach is the best method
to evaluate complex CHD by CTA as follows: (a) determine
atrial situs derived from bronchial branching pattern
(b) locate the three segments (the atrial chambers,
ventricular chambers, and the great arteries), (c) identify
the cardiac connections (venoatrial, atrioventricular, and
ventriculoarterial), (d) assess associated malformations,

2059

and (e) determine the cardiac position (position of the

heart within the chest, orientation of the apex). Due to
complexity of CHD, CT images often require evaluation on
a dedicated 3D workstation.

Atrial Septal Defect

Atrial septal defect (ASD) accounts for about one-third of
cases of CHD detected in adults.99 Ostium secundum atrial
septal defects are the most common type of interatrial
communication located at the fossa ovalis (Figs 85.51A
to D) and ostium primum atrial septal defects are the next
most common type, the least common type of interatrial
communication is a sinus venosus defect, which is located
at the junction of either the superior or the inferior vena
cava with the RA. In patients with a superior sinus venosus
defect, there is frequently an abnormal connection of one
or all of the right pulmonary veins (Figs 85.52A and B).100

Anomalous Pulmonary Venous Connection

Patients with total anomalous pulmonary venous connection are often evaluated in early childhood, especially
the infracardiac variety, which is often associated with
obstruction of the vertical vein at the diaphragm leading
to pulmonary venous HTN and edema. In partial anomalous pulmonary venous connection, one or more (but
not all) pulmonary veins are connected to the vena cava
or RA (Fig. 85.53). Abnormal connection of the right
upper pulmonary vein to the SVC occurs frequently
in the presence of a sinus venosus defect. Anomalous
connection of the right pulmonary vein or veins to the
IVC, often seen in association with hypoplasia of the right
lung and anomalous systemic arterial supply through
aortopulmonary collateral vessels, is called scimitar
syndrome.

Transposition of Great Arteries

The majority of adult patients with this condition have
undergone corrective surgery either atrial or arterial
switch operations. In the atrial switch procedure, an atrial
baffle redirects systemic venous blood to the anatomical
left pulmonary ventricle and pulmonary venous blood to
the anatomical right systemic ventricle, with a resultant
functional atrial switch. The Mustard operation usually
is performed with pericardial tissue or a tissue graft used
for the baffle, whereas in the Senning procedure, the atrial
septum is reconstructed to form the baffle (Figs 85.54A
to D).101 In the arterial switch operation, the aorta and the

2060

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.51A to D: Atrial septal defect (ASD) and Eisenmenger syndrome. Chest radiograph (A) shows an enlarged heart, dilated pulmonary arteries, and shunt vascularity. Axial computed tomography (CT) image (B) shows a large septum secundum defect (circle) along
with a dilated right atrium. The main pulmonary artery is dilated (C) and there is mosaic perfusion in both lungs (D). Findings are suggestive of secondary pulmonary HTN. (LA: Left atrium; LV: Left ventricle; RA: Left atrium; RV: Right ventricle; MPA: Main pulmonary artery).

Figs 85.52A and B: Sinus venosus atrial septal defect (ASD). A connection is seen between distal superior vena cava (SVC) and roof
of the left atrium (A, red arrow) along with anomalous right upper lobe (RUL) pulmonary vein drainage into the SVC (B, yellow arrow).
(AO: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle; RAA: Right atrial appendage; PA: Pulmonary artery; PV: Pumonary
valve).

Chapter 85: Cardiac CT Imaging

2061

Fig. 85.53: Coronal computed tomography (CT) angio view.

Partial anomalous pulmonary venous return (PAPVR) of right lung
into inferior vena cava (IVC; arrow) is responsible for secondary
pulmonary artery hypertension (PAHTN) in this 48-year-old
female presenting with progressive shortness of breath and was
responsible for secondary pulmonary HTN. This patient was
operated successfully. (RA: Right atrium).

Figs 85.54A to D: Transposition of great arteries (TGA) status post (s/p) Senning operation. The pulmonary artery (PA) is to the left and
slightly posterior to the aorta (AO). It is connected to the morphological left ventricle (LV). An intra-atrial baffle (B) is connected to the
LV. The aorta is connected to the morphological right ventricle (RV), which is hypertrophied and dilated. The morphological left atrium
is surgically connected to the RV, thereby correcting the blood flow: systemic venous blood flows via the baffle (star) into the LV then
into PA and lungs. The oxygenated blood returns via the pulmonary veins into the LA and is directed into the RV and then into the aorta.

2062

Section 7: Miscellaneous and Other Noninvasive Techniques

PA are transected above the semilunar valves and moved

to the correct circulatory position. The coronary arteries
are excised from the right side with a button-like margin
of tissue around each artery and are implanted just above
the valve on the left side of the heart; the areas from which
the coronary arteries were excised are then patched with
pericardium. Postoperative evaluation after an atrial
switch procedure may be difficult with echocardiography
because of the retrosternal position of the pulmonary
trunk and pulmonary arteries.

Congenitally Corrected Transposition of the

Great Arteries
In this condition, there is discordant atrioventricular and
ventriculoarterial connections, such that the morphological RA is connected via the mitral valve to the
morphological right-sided LV and then to the PA. The
morphological LA is connected via the tricuspid valve
to the morphological left-sided RV and then to aorta
(Figs 85.55A and B). In patients with congenitally corrected
transposition, the great arteries are parallel (side-by-side
arrangement). In the absence of associated anomalies
(e.g. ventricular septal defect, pulmonary outflow tract
obstruction), congenitally corrected transposition of the
great arteries is often asymptomatic until adulthood.

Tetralogy of Fallot
Because the surgical repair is usually performed during
early childhood, the role of CT in diagnosing TOF is
minimal. In postsurgical evaluation, the main purpose
of CT is to visualize extracardiac complications, to depict
the morphological characteristics of the main PA and its
branches (to identify any obstruction, distortion after
previous palliative shunt creation, or aneurysm), and to
detect any right ventricular enlargement due to chronic
volume overload in the presence of severe pulmonary
regurgitation (Figs 85.56A and B).

Coarctation of the Aorta Congenital

Heart Disease
The most common type of coarctation seen in adults is
postductal, where the narrowing is located just distal
to the left subclavian artery. The aorta just distal to the
coarctation is typically dilated and there is often dilatation
of the left subclavian artery (Fig. 85.57). There is slightly
higher incidence of associated bicuspid AVs in patients with
Turner syndrome. Preoperatively, CT can easily establish
the diagnosis of coarctation and provide additional
information about collateral flow. Postoperatively, CT can
be used to evaluate restenosis, patch dilatation, or patency
of endo-stent depending on the type of treatment used.

Figs 85.55A and B: Corrected transposition. In congenitally corrected transposition, there is atrioventricular and ventriculoarterial discordance such that the left atrium (LA) is connected to right ventricle (RV), which is connected to the aorta. The right atrium (RA) is connected
to left ventricle (LV) and is then connected to the pulmonary artery (PA). The circulation circuit in these patients is: systemic blood return
morphological RA
morphological LV
pulmonary arteries. Oxygenated blood from lungs return via pulmonary veins
morphological LA morphological R V aorta.

Chapter 85: Cardiac CT Imaging

2063

Figs 85.56A and B: Tetralogy of Fallot (TOF) after palliation treatment. A 64-year-old female with prior BlalockTaussig palliation for
tetralogy of Fallot. Oblique coronal three-dimensional (3D) volume-rendered reconstruction demonstrating right ventricular (RV) hypertrophy, overriding aorta (AO): and subaortic ventricular septal defect (VSD; asterisk). 3D volume-rendered slab reconstruction illustrating
the right ventricular outflow tract obstruction (arrow). (PA: Pulmonary artery).

Fig. 85.57: Coarctation of aorta. Volume-rendered sagittal oblique

view of the thoracic aorta demonstrates focal narrowing of the
descending aorta (circle) beyond the origin of the left subclavian
artery (LSA). A few of the dilated collateral vessels are also seen
in this image (arrows).

Fig. 85.58: Left ventricular assist device (LVAD). Typical location of inflow cannula (A) in left ventricle (LV) midcavity, pointed
forward the mitral valve. The outflow cannula (B) is connected to
the ascending aorta.

Left Ventricular Assist Device

worsening right heart failure, arrhythmias, hypotension,

and hemolysis can be indicators of suboptimal LVAD function or related complications such as tamponade or aortic
regurgitation. Accurate early recognition and management of these symptoms is important. CT has been used
successfully to diagnose several common LVAD complications. CTA can delineate inflow and outflow cannula
position, abnormal angulation, cannula thrombi, pericannula fluid collection, pericardial effusion, and driveline
position (Figs 85.58 to 85.60). 3D reconstruction, a wide

There has been a steady increase in the number of continuous flow left ventricular assist devices (LVAD) implanted
annually, since the Heartmate II LVAD was approved for
bridge to transplant in 2008 and destination therapy in
2010. Given the persistent donor shortage and improvements in LVAD technology, patients are increasingly likely
to undergo LVAD placement and remain on LVAD therapy
for prolonged periods of time. Persistent left heart failure,

2064

Section 7: Miscellaneous and Other Noninvasive Techniques

Figs 85.59A and B: Outflow cannula thrombosis (B) in this patient with chronic heart failure and post-left ventricular assist device
(LVAD) complication. This was also seen in the aortic root injection angiogram done in an attempt to remove the thrombus.

contains 1535 mL of serous fluid.105 The normal pericardial thickness on CT/MR is < 2 mm.106,107 On CT, it is a
linear isodense structure between the epicardial and
pericardial fat.

Pericardial Duplication Cyst

Fig. 85.60: Left ventricular assist device (LVAD) obstruction.

Abnormal position of inflow cannula (A), which is abutting the left
ventricle (LV) lateral (arrow) wall causing obstruction. (RV: Right
ventricle).

FOV, and reproducibility of measurements are advantages

of CTA over echocardiographic techniques. In addition,
gated and dynamic CTA can provide functional information, including right ventricular function and cardiac
output.102104

Pericardial Diseases
Pericardium is a dual layer fibrous sac surrounding the
heart with inner visceral layer adherent to the epicardium
and the outer parietal layer. The potential space between
these two layers is called the pericardial cavity that

These are congenital encapsulated cysts in close proximity

to the pericardium that usually do not communicate
with the parent cavity. The incidence is rare, about 1 in
100,000.108 They can occur anywhere around the heart, but
the right cardiophrenic angle is the commonest site.109 On
imaging, these are seen as round or elliptical, thin-walled
cystic lesions with low-density fluid within (H.U. 010;
Figs 85.45A and B). However, higher attenuation contents
could be seen with infection or hemorrhage.

Pericardial Defect
It is a rare anomaly with the spectrum ranging from a
small defect to complete absence of the pericardium.110
It is commonly seen along the left side of the heart and
may have associated congenital abnormalities such as
ASD, PDA, or a bicuspid AV.111
The lack of visualization of normal pericardium is a
direct sign. However, it is difficult to visualize the normal
pericardium along the left side of the heart. The indirect
signs include levorotation of the heart, and interposition
of lung tissue between the aorta and main PA or between
inferior border of heart and diaphragm. Excessive motion
of the cardiac apex is seen on cine MR images.112 Complete

Chapter 85: Cardiac CT Imaging

absence of pericardium is usually asymptomatic. Partial

absence may cause complications such as herniation with
entrapment of a cardiac chamber or structure.

2065

further assessment with clinical history, echocardiography, or cardiac MR is indicated.

Constrictive Pericarditis
Pericardial Effusion
Pericardial effusion may be transudate, exudates, or
hemorrhage. The role of imaging is to determine its
presence, pericardial thickening, and mediastinal or
thoracic abnormalities to explain the cause. There are no
specific criteria for distinguishing physiological versus
pathological pericardial fluid. Visual evaluation and
categorization as mild, moderate, or severe is routinely
performed (Fig. 85.61). Pericardial thickness of > 4 mm is
considered abnormal.113 Mediastinal lymphadenopathy or
presence of thoracic malignancy may raise the suspicion
for malignant effusion. Hemorrhagic effusions are seen
in the setting of trauma, dissection, postsurgery, and
will show CT attenuation value between 40 and 80 H.U.
(Fig. 85.62).

Pericardial Calcification
Calcification of pericardium is secondary to a prior insult.
Postcardiac surgery, hemopericardium, exudative effusions
such as tuberculous, postradiation are some of the common
causes of pericardial calcifications (Fig. 85.63). Pericardial
calcification may result in constrictive physiology and

Fig. 85.61: Pericardial effusion and tamponade. Large amount

of pericardial effusion (stars) producing a mass effect leading to
compression of right ventricle (RV) and dilation of inferior vena
cava (IVC), hepatic veins, and coronary sinus (CS) (not shown),
supports the presence of tamponade physiology. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium).

Thickening and fibrosis of pericardium from inflammatory

conditions mentioned above can lead to constrictive
pericarditis. Pericardial calcification may be seen in up to
50% of these cases.114
Physiologically, there is impaired diastolic filling of
the ventricles that leads to equalization of right and left
ventricular diastolic pressures.115 The patient may present
with right heart failure. The treatment is surgical stripping
of the pericardium. It is important to distinguish this from
restrictive cardiomyopathy, since they may have the same
physiology although the treatment is completely different.
Flattening of the RV free wall and tubular configuration of
ventricles are other features that may be seen.

Pericardial Tumors
Primary pericardial tumors are extremely rare and
metastases are far more common (see Fig. 85.43).116 Focal
or generalized pericardial thickening, enhancement,
nodularity, and hemorrhagic pericardial effusions are
some of the features that may be seen on CT. The common
primary malignancies that metastasize to the pericardium
are lung, breast, melanoma, and lymphoma.117

Fig. 85.62: Hemopericardium. High attenuating fluid (45 H.U.) in

pericardial space is suggestive of blood. The cause of hemopericardium in this patient was uremia.

2066

Section 7: Miscellaneous and Other Noninvasive Techniques

Fig. 85.63: Pericardial calcification. The pericardium is thickened

measuring 4 mm and shows areas of spotty calcification (arrows).
This patient did not have constrictive physiology.

Primary pericardial tumors include sarcoma (see Fig.

85.44), mesothelioma, and lymphoma. Lipoma is the most
common benign tumor of the pericardium. Fibroma and
teratoma may also occur in relation to the pericardium.
Both lipoma and teratoma have a low malignant potential.

CONCLUSION
MDCT has evolved rapidly over the last decade into a
powerful cardiac imaging tool that allows the physician to
visualize the coronary artery anatomy along with cardiac
morphology and function in a single noninvasive study.
Advances in technology now enable us to scan patients
with higher heart rates, decreased radiation dose, and
ability to overcome calcium artifacts. CTA compares
well to catheter angiography in detecting stenosis and,
in addition is superior in detecting regional wall-motion
abnormalities. Myocardial perfusion information can be
derived from rest and stress MDCT, although its wider
practical application is limited at this time. CT-FFR is
in early investigational state but could become a game
changer once it can be performed easily and quickly.

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37. Tsiflikas I, Brodoefel H, Reimann AJ, et al. Coronary CT
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39. Voros S, Rinehart S, Qian Z, et al. Prospective validation
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43. Sun Z, Ng KH. Prospective versus retrospective ECG-gated
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Section 7: Miscellaneous and Other Noninvasive Techniques

46. Hulten EA, Carbonaro S, Petrillo SP, et al. Prognostic

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48. Cademartiri F, Seitun S, Romano M, et al. Prognostic value
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59. Yamamuro M, Tadamura E, Kubo S, et al. Cardiac

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66. Rocha-Filho JA, Blankstein R, Shturman LD, et al. Incremental value of adenosine-induced stress myocardial
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68. Lima JA. CORE320: Diagnostic performance of combined
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71. Agatston AS, Janowitz WR, Hildner FJ, et al. Quantification
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72. Callister TQ, Cooil B, Raya SP, et al. Coronary artery
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73. Nasir K, Raggi P, Rumberger JA, et al. Coronary artery
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9. PMID:15110208.

Chapter 85: Cardiac CT Imaging

74. Greenland P, Bonow RO, Brundage BH, et al. ACCF/AHA

2007 clinical expert consensus document on coronary
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75. Pope JH, Aufderheide TP, Ruthazer R, et al. Missed
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76. Swap CJ, Nagurney JT. Value and limitations of chest pain
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77. Scheffel H, Alkadhi H, Plass A, et al. Accuracy of dualsource CT coronary angiography: First experience in a high
pre-test probability population without heart rate control.
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78. Leschka S, Alkadhi H, Plass A, et al. Accuracy of MSCT
coronary angiography with 64-slice technology: first
experience. Eur Heart J. 2005;26(15):14827.
79. Hoffmann U, Nagurney JT, Moselewski F, et al. Coronary
multidetector computed tomography in the assessment of
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225160.
80. Meijboom WB, van Mieghem CA, Mollet NR, et al. 64-slice
computed tomography coronary angiography in patients
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81. Goldstein JA, Gallagher MJ, ONeill WW, et al. A randomized controlled trial of multi-slice coronary computed
tomography for evaluation of acute chest pain. J Am Coll
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82. Laudon DA, Vukov LF, Breen JF, et al. Use of electron-beam
computed tomography in the evaluation of chest pain
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83. Georgiou D, Budoff MJ, Kaufer E, et al. Screening patients
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electron beam tomography: a follow-up study. J Am Coll
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84. McLaughlin VV, Balogh T, Rich S. Utility of electron beam
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1999;84(3):3278, A8.
85. Hoffmann U, Truong QA, Schoenfeld DA, et al.
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86. Litt HI, Gatsonis C, Snyder B, et al. CT angiography for safe
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2069

87. Goldstein JA, Chinnaiyan KM, Abidov A, et al. CT-STAT

Investigators. The CT-STAT (Coronary Computed Tomographic Angiography for Systematic Triage of Acute Chest
Pain Patients to Treatment) trial. J Am Coll Cardiol.
2011;58(14):141422.
88. Chinnaiyan KM, Raff GL, Goraya T, et al. Coronary computed
tomography angiography after stress testing: results
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89. Chinnaiyan KM, Depetris AM, Al-Mallah M, et al. Rationale,
design, and goals of the Advanced Cardiovascular Imaging
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90. Hamon M, Lepage O, Malagutti P, et al. Diagnostic
performance of 16- and 64-section spiral CT for coronary
artery bypass graft assessment: meta-analysis. Radiology.
2008;247(3):67986.
91. Ehara M, Kawai M, Surmely JF, et al. Diagnostic accuracy
of coronary in-stent restenosis using 64-slice computed
tomography: comparison with invasive coronary
angiography. J Am Coll Cardiol. 2007;49(9):9519.
92. Araoz PA, Mulvagh SL, Tazelaar HD, et al. CT and MR
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93. Sparrow PJ, Kurian JB, Jones TR, et al. MR imaging of
cardiac tumors. Radiographics. 2005;25(5):125576.
94. Shah DC, Hassaguerre M, Jas P. Toward a mechanismbased understanding of atrial fibrillation. J Cardiovasc
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95. Achenbach S, Delgado V, Hausleiter J, et al. SCCT expert
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96. Gilard M, Eltchaninoff H, Iung B, et al. FRANCE 2 Investigators. Registry of transcatheter aortic-valve implantation in high-risk patients. N Engl J Med. 2012; 366(18):
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97. Kodali SK, Williams MR, Smith CR, et al. PARTNER Trial
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surgical aortic-valve replacement. N Engl J Med. 2012;
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98. Sinning JM, Hammerstingl C, Vasa-Nicotera M, et al. Aortic
regurgitation index defines severity of peri-prosthetic
regurgitation and predicts outcome in patients after
transcatheter aortic valve implantation. J Am Coll Cardiol.
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100. Otsuka M, Itoh A, Haze K. Sinus venosus type of atrial
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101. Siegel MJ, Bhalla S, Gutierrez FR, et al. MDCT of postoperative anatomy and complications in adults with
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Index
Page number followed by f and t indicate figure and table respectively.

A
Abdominal aortic aneurysm, ultrasound
stethoscope for screening of, 294
295
Aberration, 81
Abiomed Impella, 1224f
Abnormal mitral arcade, 16131614
Absorption, 5657
ACC. See American College of Cardiology
(ACC)
Accessory mitral orifice, 1615
ACCF. See American College of
Cardiology
Foundation (ACCF)
Accreditation Council of Graduate
Medical Education (ACGME), 754
Acetylcholine (Ach), 450, 451452, 451f,
455
Ach. See Acetylcholine (Ach)
ACM. See Alcohol-induced
cardiomyopathy (ACM)
Acoustic energy safety, 111
Acoustic enhancement, 734, 734f
Acoustic radiation force impulses (ARFI),
1995
Acoustic shadowing, 61, 62f, 733734
Acquired heart diseases, in childhood,
18561864

aortic insufficiency, 1860f

complications of, 1859

coronary ectasia and aneurysms,
18611863

Duke criteria for, 1857

echocardiographic findings, 1857
1859

infective endocarditis, 18561857

infective endocarditis prophylaxis,
1859

Jones criteria for, 18591861

acute valvulitis, 1860

chronic RHD, 18601861

minor criteria, 1860

Kawasaki disease, 1861, 1862t

mitral valve insufficiency, 1860f

overview, 1856

rheumatic heart disease, 1859

vegetations in, 18581859

location for, 1958

size and embolic risk, 19581959
ACS. See Acute coronary syndromes
(ACS)
AcuNav Diagnostic Ultrasound
Catheter, 644, 645f. See also
Intracardiac echocardiography
(ICE)
Acute aortic regurgitation, 1972

acute decompensation, 1974

acute regurgitation, 1974

aortic dissection, 1972

diastolic fluttering, 1974

infective endocarditis, 1972

left ventricular hypertrophy, 1974

post trauma, 1972t

suprasternal notch, 1974

transthoracic views, 1974

ventricular septal defect, 1973f
Acute chest pain, CT scan for, 20452047
Acute coronary syndromes (ACS)

contrast echocardiography for, 443

echocardiography role in, 12921294

evaluation of, by CONTISCAN

transducer, 226
Acute heart failure syndrome, 1986
Acute LV remodeling (ALVRM), 1325
Acute myocardial infarction, three
dimensional speckle tracking and,
376t
Acute pericarditis, 1436
Acute respiratory distress syndrome
(ARDS), 1983, 1986

acute heart failure syndrome, 1986

chest X-ray, 1986

differential diagnosis, 1986

diffuse lung injury, 1986

lung ultrasound scan, 1986

sonographic pattern

multiple diffuse B-lines, 1986, 1986f

small subpleural consolidations,
1986, 1986f

spared areas, 1986

subclinical pulmonary edema, 1986
Acute rheumatic fever (ARF), 931

diagnosis of carditis in, 765775

echo and Doppler studies, 768770

echocardiography vs. clinical
examination, 767768

echo interrogation, 767, 769t770t

M-mode echo in, 770

myocarditis, 766

pericarditis, 766

pitfalls in Jones criteria, 766767

regurgitation, 766

rheumatic vs. nonrheumatic
regurgitation, 768

secondary prophylaxis, fidelity of, 775

two-dimensional echo, 770772

overview, 765

physiological vs pathological
regurgitation, 772773

Vijaya's echo criteria, 771, 771t
Adenosine stress test, 13331334
ADMA (asymmetrical dimethyl arginine),
454
Adriamycin-induced cardiomyopathy,
1397f
Adult congenital heart disease (ACHD),
17911855

aortic root in, 1797, 1797f

complex congenital heart defects,
18261848

echocardiography, key concepts of,
17931797

abdominal situs, 1795

assessment of shunts, 17951796

atrial situs solitus, 1794

atrioventricular relationship, 1795

position of apex, 1794

pregnancy and, 17961797

simplified segmental approach,
17931795

VA connections, 1795

indications for TEE, 1792t

overview of, 17911793

I-II

Comprehensive Textbook of Echocardiography

simple congenital heart defects,
17981813

surgical terms in, 1794t

terminology in, 1793t

valvular disease, 18131826
Aging, effect of
on HV Doppler, 303

on pulmonary venous flow, 329330,
330f
Air embolism, 1977

coronary arteries, 1977

hemodynamic instability, 1977

paradoxical emboli, 1977

postcardiac arrest, 1977

post multiple injuries, 1977

respiratory distress, 1977
Air microbubble contrast, 436
AIUM. See American Institute of
Ultrasound in Medicine (AIUM)
ALa. See Anterior leaflet angle (ALa)
Alcohol-induced cardiomyopathy (ACM),
1397
Alcohol-induced necrosis, 1364f
Alcohol septal ablation
in HCM, 13621363

for hypertrophic obstructive
cardiomyopathy, 571
Alfieri stitch, 539
Aliasing, 70, 137, 736. See also Artifacts
Alveolar-interstitial syndrome, 1986
ALVRM. See Acute LV remodeling
(ALVRM)
Ambulatory echocardiography, 227229,
229t
American College of Cardiology (ACC),
750, 751752, 757, 758
American College of Cardiology
Foundation (ACCF), 758, 2045
American correction, 580
American Heart Association (AHA), 6, 758
American Institute of Ultrasound in
Medicine (AIUM), 110
American Registry of Diagnostic Medical
Sonography (ARDMS), 754
American Society of Anesthesiologists
(ASA), 638
American Society of Echocardiography,
907
American Society of Echocardiography
(ASE), 91, 132, 296, 516, 638, 753,
1115, 1323
A-mode echocardiography, 57, 58f
Amplatzer PFO Occluder, 557
AmplatzerTM atrial septal occluder,
552553, 554f

AmplatzerTM multifenestrated septal

occluder, 553, 554f
AmplatzerTM ventricular septal occluder,
558
Amplifier, 60
Amplitude Doppler. See Power Doppler
Amptazer duct occlude II (ADO II) series,
1598
AMVL. See Anterior mitral valve leaflet
(AMVL)
Amyloid cardiomyopathy, 13981401,
1399f1400f
Amyloidosis, 18721874, 1873f1874f

velocity vector imaging in, 399
Analogue-to-digital converter, 67
Angiosarcomas, 14851486, 2055f
Angle of insonation, 66
Annuloaortic ectasia, 974, 1695
Annuloplasty ring dehiscence, 608
Annulus paradoxus, 14461447
Anomalous coronary artery (ACA),
17631766

computed tomography for, 2047
2049, 2047f

in elderly, 1947f
Anomalous origin of left main coronary
artery from pulmonary artery
(ALCAPA), 2048, 2048f
Anomalous papillary muscle, 1354f
Anomalous pulmonary venous drainage,
1676
Antegrade velocity of mitral inflow, 869
Anterior circulation, 665668, 667f669f
Anterior leaflet angle (ALa), 1423
Anterior mitral valve leaflet (AMVL), 777
Anterior pericardial effusion, 170
Anthracycline exposure, 395
Aorta

echocardiographic evaluation,
945951

basic principles, 945

normal aortic size, 951

PLAX view, 945

PSAX view, 946

transesophageal echocardiography,
949950, 950f, 951f

transthoracic echocardiography,
945949, 946f, 947f949f

and esophagus, 969

genetic syndromes affecting, 958959

bicuspid aortopathy, 959

Marfan syndrome, 958959

transesophageal imaging of, 967982,
969f970f

Index

acute aortic diseases, 976982

aortic aneurysm, 974976

aortography, 980

atherosclerotic disease, 971974

celiac artery, 970

descending thoracic aorta, 970

human anatomic cross-section, 968f

intramural hematoma, 978

left carotid and subclavian artery,
970, 971f
magnetic resonance imaging and,
979

orientation, 968970

real time 3D, 981

transgastric level, 969
Aortic abscess

in native valve, 1045f

transesophageal approach, 1053f
Aortic aneurysms, 951954, 16951696,
1797

anomalous origin of PA from
ascending aorta, 16951696,
1696f

echocardiographic evaluation of,
951954, 952f954f

in elderly, 19341937

cystic medial degeneration and, 1936

etiology of, 1936

natural history of, 1937

with rupture, 1936f

epidemiology of, 951

pathophysiology of, 951

pulmonary artery sling, 1696

transesophageal imaging of, 974976
Aortic arch (AoA), 664665, 665f, 666f,
1538f, 1795f

abnormities, 16901692

cervical aortic arch, 1691

right aortic arch, 16901691

vascular rings, 1691

anomalies, 665

coarctation of aorta, 16921694

interruption of, 16941695, 1695t

types of, 664665, 665f
Aortic arch anomalies, 17701773

aortic arch to pulmonary artery
fistula, 1773

coarctation of aorta, 17701773
Aortic arch scanning protocol, carotid
ultrasound, 676f
Aortic atheroma, 959961

appearance on TTE and TEE, 959,
960f

grading, 961t

penetrating aortic ulceration, 961,
962f

TEE characterization of, 960961
Aortic atherosclerosis

in elderly, 19211923, 1922f

epiaortic ultrasonography in,
640641, 641f

in female, 1896f
Aortic balloon valvuloplasty (ABV), 541
Aortic coarctation. See Coarctation of
aorta (CoA)
Aortic dissection, 954958, 1974

aorta distal to left subclavian artery,
954

in ascending aorta, 954

diagnostic imaging, 1974

dissection, 1974

in elderly, 19371942, 1937f1938f,
1941f

acute coronary syndrome and, 1940

color Doppler flow in, 1942

descending thoracic aorta, 1942

perfusing lumen, 1942

rupture, 1938f1940f

epidemiology, 954

intramural hematoma, 957958

TEE diagnosis of, 955957

TTE evaluation, 955

Type A dissection, 954, 955957

Type B dissection, 954
Aortic dissection, epiaortic
ultrasonography for detection of,
641
Aortic insufficiency, 1860f

and carotid ultrasound findings, 693
Aortic isthmus, 1538f
Aortic leiomyosarcoma, 1489f1490f
Aortic lumen to pulmonary artery fistulas
(APAF), 1773
Aortico-left ventricular tunnel, 1632
Aortico-right atrial tunnel, 1632
Aortico-right ventricle tunnel, 1632
Aortic override, 16331644

aortic arch, 16381639

aortopulmonary collaterals, 1639
1640

coronary artery anomalies, 1640

patent arterial duct, 16391640

pulmonary arteries, 16371638, 1638f

pulmonary stenosis, 16341637

tetralogy of Fallot with absent
pulmonary valve, 1637, 1637f

cardiac catheterization, indications
for, 1641

echocardiographic measurements in,
16401641, 1640f

postoperative evaluation of, 1641
1644, 1642f

ventricular septal defect, 16331634,
1633f1634f
Aortic regurgitation (AR), 806812,
930944, 16281630, 1630f

in adults, 1824

aortic root dilatation, 16291630

aortic valve surgery, timing of,
941942

cardiac MRI for, 20102011, 2011f

causes of, 16281629, 1629t

continuous wave Doppler, 810811

signal of, 1279

3DE VCA measurement of, 280

diastolic fluttering of AML, 807808
echocardiography

exercise, 811

indications for, 807t

role of transthoracic, 811

three-dimensional, 812

etiology of, 930935

acute rheumatic fever, 931

aortic root and aortic annulus,
diseases of, 935, 936f

aortic valve cusps, diseases of,
930935, 932f935f

Behet's disease, 935

congenital cardiac defects and aorta
abnormalities, 930931

infective endocarditis, 931

systemic illnesses, 931

flow convergence, 809

follow-up in, 812

grading of, 810f

left ventricle adaptation to, 941

left ventricular outflow abnormality,
16281629

pulsed wave Doppler, 810

regurgitant jet size, 808809

severity, quantification of, 936941

color Doppler methods, 936939

continuous wave Doppler methods,
940941, 941f

mitral valve findings, 941

M-mode and 2D findings, 936, 937f

parameters to grade, 937t

pulsed wave Doppler methods,
939940, 940f

severity of, 808, 808t, 812, 1630

sinus of Valsalva aneurysm and, 1631

vena contracta imaging in, 938
Aortic root, 1621

I-III

ACHD and, 1797

anatomy of, 897

echocardiographic assessment of
dimension, 1797

parasternal long-axis view of, 1797f
Aortic sclerosis, echocardiography of,
899900
Aortic stenosis, 802806, 896918

in adults, 1824

aortic jet velocity, 803804

aortic valve Doppler examination,
904912

appropriate examination, 904905

mean and peak gradients, 906

pressure recovery, 912

valve area measurement, 906907

aortic valve replacement for, 540546

associated conditions, 806

asymptomatic patients with normal
LV function, 912923

cardiac MRI for, 2010, 2011f

and carotid ultrasound findings, 693

with coexistent subvalvular stenosis,
903904

critical neonatal, 16221623

3DE assessment of, 279

echocardiography in, 898904

aortic measurements, 902903

aortic sclerosis, 899900

bicuspid aortic valve, 902

criteria for, 914t916t

indications and appropriateness for,
913, 914t

M-mode, 898899, 899f

recommendations for, 802t

stress, 805806, 912913

three-dimensional, 806, 900902

two-dimensional, 899

in elderly, 19241934

aortic valve area, estimation of, 1926

2D echocardiography, 19241928,
1925f, 1927f

3D echocardiography, indications for,
1933t

LFLG-AS with low EF, 1929f

live/real time 3D TTE, 19301933

mild, 1921f

PLFLG-AS with preserved EF,
19291930

prevalence and pathophysiology,
1924

severe, 1931f1932f, 1933f

transcatheter aortic valve
replacement, 1934, 1935f

ventricular response to, 19331934

I-IV

Comprehensive Textbook of Echocardiography

etiology of, 897898

low-gradient, low stroke volume,
severe, 908911

aortic valve calcium score, 911

dobutamine infusion, 910

hypertension and, 911

low transvalvular gradients and, 913

mean transaortic pressure gradient,
804805

normal aortic valve anatomy, 896897

aortic root, 897

aortic valve, 896897

oveview, 896

pseudosevere group of, 911

sclerosis to stenosis, 897898

severity, estimation of, 803, 907908,
907t

definition of, 907, 907t

methodological limitations in, 908,
908t, 909t

pitfalls in estimation of, 908

technical errors in, 908

strain in evaluation of, 913

subvalvular, 16241626

supravalvular, 16261628

valve area, 805

valvular, 16181624

vs. hypoplastic left ventricle, 1623
Aortic trauma, and free rupture, 961963
Aortic valve (AV), 582584

anatomy of, 896897

3DE assessment of, 278f, 279, 279f

3D echo of, 520522, 520f

intermittent opening of, 1242f

short axis of, 1536f

ventricular assist devices, 1231, 1231f
Aortic valve, in adults, 18211826

aortic regurgitation, 1824

aortic stenosis, 1824

bicuspid, 18211824

cardiac catheterization, 18221823

echocardiography, 18211822

MRI/CT for, 1823

postoperative adult, 1824

stress testing, 18231824

subaortic stenosis, 18241825

supravalvular aortic stenosis,
18251826
Aortic valve annulus, 1621
Aortic valve area (AVA), 279, 1622

calculation of, 543, 585586
Aortic valve calcium score:, 911
Aortic valve diseases, 802816, 1318

aortic regurgitation, 806812

continuous wave Doppler, 810811

3D echocardiography, 812

diastolic fluttering of AML, 807808

exercise echocardiography, 811

flow convergence, 809

follow-up in, 812

grading of, 810f

pulsed wave Doppler, 810

regurgitant jet size, 808809

role of TEE in, 811

severity of, 808, 808t, 812

aortic stenosis, 802806

aortic jet velocity, 803804

associated conditions, 806

3D echocardiography, 806

mean transaortic pressure gradient,
804805

severity of, 803

stress echocardiography in, 805806

valve area, 805

role of 3D TEE in operating room in,
582588, 594f600f

stress echocardiography in, 1318
Aortic valve Doppler examination,
904912
Aortic valve fibroelastoma, 1479f
Aortic valve pseudoaneurysm, 1046f
Aortic valve sclerosis, 1923f

in elderly, 19231924, 1923f

bicuspid, 1931f

echocardiographic findings for,
19231924, 1930f

normal hemodynamics in, 1924

pathophysiology of, 19231924

prevalence of, 19231924

systolic murmur, 1923
Aortic valve stenosis (AS)

in adults, 919

calcific, 919

dobutamine stress echocardiography,
response to, 921

invasive evaluation of, 923

invasive vs. noninvasive evaluation
of, 922924

SAVR role in, 926927

severity grade, 919

three main entities of severe, 926t
Aortocameral communications, 1632

aortico-left ventricular tunnel and,
1632

aortico-right atrial tunnel and, 1632

aortico-right ventricle tunnel and,
1632
Aortopulmonary window (APW), 1751,
1753f1754f

Index

color flow mapping, 1603

types of, 1602
APAF. See Aortic lumen to pulmonary
artery fistulas (APAF)
Aperture

3D ultrasound imaging and, 80

and TEE image quality, 104, 106f
Apical approach, of three-dimensional
echocardiography, 244, 254f, 255f
Apical hypertrophic cardiomyopathy,
1356
Apical window, TTE, 146, 148

five-chamber plane, 153154, 153f,
154f, 154t

Doppler imaging, 153154, 154f, 154t
technique, 153, 153f

four-chamber plane, 148153,
148f153f, 149t

chamber quantification, 150151,
151f, 152f

diastolic function assessment,
149150, 150ff, 151f

Doppler imaging, 149, 150f

intracardiac shunts assessment, 153,
153f

right heart assessment, 151152, 152f

technique, 148, 148f, 149t

two-dimensional anatomic imaging,
148149, 149f

long-axis or three-chamber plane,
155, 156f

two-chamber plane, 154155, 154f,
155f

chamber quantification, 154155

Doppler imaging, 155

technique, 154, 154f

two-dimensional anatomic imaging,
154, 154f, 155f
Application specific integrated circuits
(ASICs), 77, 77f, 78
ARDMS. See American Registry of
Diagnostic Medical Sonography
(ARDMS)
Area under the curve (AUC), 452, 452f
ARFI. See Acoustic radiation force
impulses (ARFI)
ARIC. See Atherosclerotic Risk in
Communities (ARIC) study
Arrhythmogenic right ventricular
cardiomyopathy (ARVC), 1395
1396
Arrhythmogenic right ventricular
dysplasia (ARVD), 395396, 1395
1396, 1396f

Arterialventricular coupling, 11211122

Artifacts, 61, 732749

acoustic shadowing and
enhancement, 61, 62f, 733734,
734f

aliasing, 736

from assumptions made by
ultrasound equipment
software, 749t

attenuation and enhancement, 61

categorization of, 733

double image, 736

3D ultrasound imaging and, 8081

effects of tissues in ultrasonic beam,
732, 733f

encountered in day-to-day clinical
practice, 738f748f

identification and elimination of, 737
mirror image, 735736

on pulmonary venous Doppler,
343345, 344f345f

range ambiguity, 736

recognition and interpretation of, 732

reflection, 62, 62f

reverberation, 62, 62f, 734735, 735f

side lobe artifacts, 6263, 63f

in three-dimensional
echocardiography, 736737

transesophageal echocardiography
and, 107110, 113116, 114f
ASD. See Atrial septal defect (ASD)
ASE. See American Society of
Echocardiography (ASE)
Asymptomatic patients, with normal LV
function, 912923
Atherosclerosis, 959961

grading, 961t

transesophageal imaging of, 971974
Atherosclerotic plaque, mild, 960f
Atherosclerotic Risk in Communities
(ARIC) study, 1948
Atresia, of left main coronary artery, 1689
Atretic coronary sinus ostium, 1683
Atrial and atrioventricular valve

abnormalities, 17731776

cor triatriatum sinister, 17731775

Ebsteins anomaly, 17751776

isolated mitral valve cleft, 1775
Atrial balloon septostomy, 531
Atrial ball valve thrombus, 845
Atrial fibrillation (AF), 12591260

computed tomography for, 20542057

myocardial sleeve of LA, 2056

paroxysms of, 2056

planning CT, 2057

radiofrequency catheter ablation,
2056

and HV Doppler, 309, 309f

and PV Doppler, 333, 333f

TD imaging in, 353
Atrial flutter, and PV Doppler, 333, 333f
Atrial myxoma, 1468
Atrial septal defects (ASD), 1134, 1279,
15851591, 1734

in adults, 17991802, 1801t

cardiac catheterization, 1802

closure of, 1802

contrast echocardiography, 1801
1802

echocardiography, 1799

exercise testing, 1802

MRI/CTA for, 1802

postoperative adult in, 1802

transesophageal echocardiography,
18001801, 1800f

types of, 17991800, 1799f

atrial septum, evaluation of, 1590

basal long-axis or bicaval view,
15901591

basal short-axis view, 1590

four-chamber view, 1591

cardiac catheterization in, 1590

closure of
Amplatzer ASD occluder, 557f
ASD closure using intracardiac
echocardiography, 559f
ASD sizing ballon, 556f

closure devices, 552553, 555f

3D TEE diagnosis of ASD, 551, 553,
553f

3D TEE monitoring of, 554555, 557f,
558f

3D TEE sizing of ASDs, 556f

3D TEE visualization of ASD rim, 557f

ICE imaging during, 648, 650, 650f

indications for, 551

secundum ASDs, 552, 554f

computed tomography for, 2059,
2060f

coronary sinus, 1586

degree of left-to-right shunt in, 1590

direction of shunt, 15881589

3D TEE for, 513

echocardiographic imaging in,
15861588

fossa ovalis, 1586

for percutaneous device closure, 1591

objectives, 1585

ostium primum, 1586

patent foramen ovale, 15851586

I-V

and PV Doppler, 342, 342f

Qp/Qs ratios in, 15891590

secundum, 17341742

sinus venosus, 1586, 17421743

stepwise evaluation for, 1586t

three-dimensional speckle tracking
and, 376t

types of, 15851586

in women, 19001901, 1900f1901f
Atrial septum, 1248, 1962

ablation,patients, 1962

abnormalities, 1962

frequent, 1963

atrial tumor, 1963

fossa ovalis region, 1962

lipomatous, 1963

obesity, patients, 1963

saline contrast study, 1962

septum primum, 1962

septum secundum, 1962

trans-septal puncture, 1962

Waterstons groove, 1963
Atrioventricular and ventricoarterial
discordance, 16641670

abnormalities of RVOT, 1669

associated defects, 1664

associated malformations, 16671669

tricuspid value abnormalities,
16671669, 1668f

ventricular septal defect, 1667, 1668f

coarctation of aorta, 1669

coronary artery anatomy in, 1664
1667, 1665f1666f

four-chamber views, 16651666

PLAX view, 1667

segmental analysis, 16641665

subcostal sagittal view, 16661667

left ventricular outflow tract
obstruction, 1669
Atrioventricular canal defect (AVCD),
1535, 1544f, 1546f
Atrioventricular connection,
echocardiography of, 1558t,
15771579, 1578f

concordant, 1579

discordant, 1579

isomeric, 1580

and loop rule, 1580

uniatrial and biventricular
connection, 1580

univentricular, 15791580
Atrioventricular dissociation

and HV Doppler, 308, 308f

and PV Doppler, 331332, 332f

I-VI

Comprehensive Textbook of Echocardiography

Atrioventricular septal defects (AVSD),

16041610, 17461747

in adults, 18091810

echocardiography, 18091810

postoperative adult, 1810
AV valve regurgitation, 1610

with common valvular orifice,
16061610

complete, 16061610

complex, 1608

3D TTE for, 17461747

echocardiography in, features of,
1605t

hemodynamic assessment of, 1610

LAVV regurgitation and, 1747

LVOT obstruction in, 1608, 1609t

needs for evaluation of, 1607

overview, 1604

partial, 16041606, 1610

pulmonary stenosis and, 1610

right ventricular outflow tract
obstruction in, 1608

types of, 16041610, 17471751
Atrium, identification of, 15761577

aorta-IVC and abdomen, 1577

atrial appendage, 1576

atrial septum, 1576

coronary sinus, 1577

venous drainage, 1576
Attenuation artifacts, 56, 61, 431433,
432f, 433f
Auenbrugger, Leopold, 4
Augmentation index (AIx), 465, 467, 467f
Aureus Staphylococci, 1043
Autocorrelation detector, 71
AVCD. See Atrioventricular canal defect
(AVCD)
AV fistulas, 699700
Axial resolution, 59, 59f, 733, 733f

B
Balloon mitral valvotomy, in mitral
stenosis, 839t, 842f

complications of, 840841

echocardiography in patients for,
838840

evaluation of patient, 838840, 839t

mitral regurgitation in, 840841

post, 841f, 843f, 844f
Balloon valvuloplasty, monitoring of, by
CONTISCAN transducer, 233, 233f,
234f
Barlows syndrome, 1860

BART (blue away red toward), 64

Basilar artery, 668
Battery-powered ultrasound imagers,
291292, 292f
Beam width artifact, 733, 734f
Behet's disease, 935
Benign cardiac fibromas, 1480
Berlin Heart INCOR Assist Device, 1228
Bernoulli equation, limitations of, 1574
Biatrial myxoma, 1470f
Bicuspid aortic valve (BAV), 898, 1766
1770. See also Aortic valve

in adults, 18211824

cardiac catheterization, 18221823

echocardiography, 18211822

MRI/CT for, 1823

postoperative adult, 1824

stress testing, 18231824

aortopathy or aortic enlargement
in, 902

echocardiography of, 902

in female, 1913f1914f

M-mode echo for, 7, 7f

progressive degenerative changes in,
902
Bicuspid aortopathy, 959
Bicycle stress echocardiography, 269
Bilateral superior vena cava (BLSVC),
1543f, 1683
Bioprosthetic aortic valve
with anterior paravalvular aortic

regurgitation, 1088f

flow velocity across, 1091f

with posterior mass, 1089f

severe stenosis and, 1090f
Bioprosthetic mitral valve

transesophageal echocardiogram of,
1088f

vegetations on leaflets, 1089f
Bioprosthetic tricuspid valve
with severe stenosis, 1091f

struts and, 1085f

transesophageal echocardiogram of,
1089f
Bioprosthetic valves, 10811082

aortic, 1088f

mitral, 1088f

stented porcine, 1081t

unstented porcine, 1082t
Biplane method of discs, 11181119
Biplane transesophageal echo (TEE)
probe, 100f
Biventricular apical hypertrophy, 1554f
Biventricular pacing, 234, 1378f1379f

Index

Block matching, 275

Blood velocity, and Doppler signal, 66
Blooming, 433, 434f
BLSVC. See Bilateral superior vena cava
(BLSVC)
Blunt chest trauma, 1969
assault, 1969

blunt objects, 1969

cardiac trauma, 1970t

hemodynamic disturbances, 1970t

hemodynamic instability, 1970t

infarction, 1970t

myocardial ischemia, 1970t

intraoperative assessment, 1970t

massive hemothorax, 1970

pericardial effusion, 1970t

puncture injuries, 1970

thoracic aortic pathology, 1970t

thoracic trauma, 1970t

vehicle collisions, 1969
B-mode echocardiography, 57, 58f
BNP. See Brain natriuretic peptide (BNP)
Bolus infusions, 421
Bom, Nicolaas, 8
Bovine arch, 665, 666f
Bovis Streptococci, 1043
Brachial/radial artery occlusion and
release, 453
Bradykinin, 455
Brain natriuretic peptide (BNP), 295, 1422
British Society of Echocardiography, 750
Brockenbrough needle, 533
Budd-Chiari syndrome, 319

C
Calcific aortic stenosis. See also Aortic
stenosis

2D echocardiography in, 900

effective orifice area in, 900

geometric orifice area in, 900
Calcium channel blockers (CCB), 1073t
Calcium-related factors, in cardiac
motion, 1202
Cannon wave, 308, 308f
Cannula flow velocities, 1248
Capacitance micro-machined ultrasound
transducer (cMUT), 1992
Capacitive micromachined ultrasonic
transducer (CMUT), 115f, 116, 116f
Capillary blood volume (CBV), 421422
Capillary wedge pressure (CWP), 1063
Carcinoid heart disease, 1006, 14071413,
1500

with carcinoid syndrome, 1408

cardiac involvement in, 1408

2D transthoracic echocardiography,
1007f

echocardiographic features, 1409
1413

endocardial deposits, 1409,
1410f1412f

left-sided valvular involvement, 1409,
1410f

pulmonary valve cusps, 1409

tricuspid regurgitation, 1412
Carcinoid tumors, 18741875, 1876f
Cardiac amyloidosis, 352, 373, 1261
Cardiac MRI for, 2008f
Cardiac calcified amorphous tumor
(CAT), 15121514
Cardiac catheterization
in atrial septal defects, 1590

of bicuspid aortic valve in adults,
18221823

in CCTGA, 1841

in coarctation of aorta, 1829

double outlet right ventricle, 1845

in D-transposition of great arteries,
1838

echo gradients during, 1574, 1575f

indications for, tetralogy of Fallot and,
1641

truncus arteriosus, 1844

univentricular hearts, 18471848
Cardiac computed tomography, 2023
2070

advantages of, 2024

anomalous pulmonary venous
connection, 2059

aorta congenital heart disease,

coarctation of, 2062, 2063f

atrial septal defect, 2059, 2060f

cardiac function, 2042, 2043f
CCTA, contraindications for, 2027

challenges for, 20242025

cardiac gating, 20242025

spatial resolution, 2024, 2024t

temporal resolution, 2024, 2024t

clinical indications for, 20442066

acute chest pain, 20452047

anomalous coronary artery, 2047
2049, 2047f

asymptomatic intermediate risk
patient, 20492050

cardiac masses, 20512054

congenital heart disease, 20592063

coronary artery bypass graft, 2050,
2052f

coronary calcium scoring, 20442045,
2045f, 2046f

coronary stent evaluation, 2050

left ventricular assist device, 2063
2064

pericardial diseases, 20642066

pulmonary vein ablation, 20542057

re-do coronary artery bypass graft,
2049

constrictive pericarditis, 2065

contrast media and injection and,
2029t

coronary calcium score protocol,
2028t

FOV for, 2027

image analysis, 20322034

extracardiac findings in, 20332034,
2037f

proper phase selection, 2033

image postprocessing, 20282032

curved multiplanar reformation, 2029

maximum-intensity projection, 2029,
2032f

multiplanar reconstruction, 2029,
2301f

transaxial images, 20282029

virtual endoscopy, 2030

volume rendering, 2030

major adverse cardiovascular events
in, 2042

in myocardial perfusion, 20422043

overview, 20232024

patient preparation for, 2028t

patient selection for, 2027

pericardial calcification, 2065, 2066f

pericardial defects, 20642065

pericardial duplication cyst, 2064

pericardial effusion, 2065, 2065f

pericardial tumors, 20652066

pitfalls and artifacts, 20342040

beam-hardening artifact, 2038, 2040f

blooming artifacts, 20362038, 2039f

cardiac motion artifact, 20342036

misregistration artifact, 2038f

respiration motion artifact, 2036,
2039f

plaque characterization, 20412042,
2041f

preablation pulmonary valve
mapping, 2028t

radiation dose, 20252027

of common examinations, 2026t

descriptors, 2026t

methods to reduce, 20262027, 2027t

I-VII

stress myocardial imaging using,
20422043

technique, 2027, 2028t, 2029t

tetralogy of fallot, 2062, 2063f

transcatheter aortic valve
replacement, 2029t, 20572059

transposition of great arteries,
20592062, 2061f

congenitally corrected, 2062, 2062f
Cardiac contusion, 1971

biochemical cardiac markers, 1971

echocardiogram, 1971

echo findings sum, 1971

electrocardiogram, 1971

myocardium, 1971

post trauma, 1972
Cardiac fibroma, 2054f
Cardiac hemangiomas, 14821484
Cardiac hypertrophy, cardiac MRI in,
20062008
Cardiac imaging, 34. See also
Echocardiography
Cardiac implantable electronic devices
(CIED), 1967

cardiac arrest, 1967

cardiac resynchronization therapy,
1967

echocardiography, 1967

infective endocarditis, 1967

heart disease, 1967

coronary artery disease, 1967

dilated cardiomyopathy, 1967

hypertrophic cardiomyopathy, 1967

lead extraction, 1967

preprocedural selection, 1967

symptomatic bradycardia, pacing,
1967

ventricular arrhythmias, 1967
Cardiac lipomas, 14811482, 1483f
Cardiac magnetic resonance imaging, in
ICM/NICM, 14271428
Cardiac masses, role of 3D TEE in
operating room in, 617627,
625f634f
Cardiac motion, 11761209

calcium-related factors in, 1202

clinical implications, 1201

definitions, 11831184

diastolic dysfunction, 12011202

dilated cardiomyopathy and,
12021203

heart function, basic, 11771180,
1177f1180f

MRI phase contrast velocity mapping
of, 1179f

I-VIII

Comprehensive Textbook of Echocardiography

myocardial fiber organization, 1180f

time frames of systole and diastole,
1177f1178f

during isovolumic contraction, 1185f

myocyte factors in, 1202

of normal heart, 11851194

overview, 11761177

pacing factors in, 12031204

right heart failure and, 1204

right ventricle, 1198

rotation, 1183

state-of-the-art, 11801181

composite of, 11811183

HVMB model, 1181

muscle contraction, asynchronous,
1181

subendocardial muscle, 11981200,
1199f, 1200f

torsion, 11831184

twisting, 1183

untwisting, 1183

mitral valve opening and, 12001201

torsion and, 11841185, 1200

ventricular septum, 11941198

ventricular structure, function of,
1177f1178f
Cardiac MRI (CMR), 381

cardiac amyloidosis, 2008f

for cardiac hypertrophy, 20062008,
2007f

cardiomyopathies, 2008

for left and right ventricle, 19981999,
1999f

for left ventricular structure, 2000
2004

LVNC, 20002002, 2001f, 2002f

tissue characterization, 20022003,
2003t

limitations of, 20172019

for mitral valve, 2012

for myocardial infarction, 20032004,
2004f

for myocarditis and sarcoidosis,
20042006, 2005f, 2006f

for normal variants and masses,
20162017

overview, 1998

pericardial disease, 20142016, 2015f

of prosthetic valves, 20132014

strain assessment, 19992000, 2000f

for valvular heart disease, 20092013

aortic regurgitation, 20102011, 2011f

aortic stenosis, 2010, 2011f

mitral regurgitation, 20122013

mitral stenosis, 2013

tricuspid regurgitation, 2013, 2014f

velocity mapping, flow and shunt
assessment, 20082009

viability assessment, 20032004
Cardiac myxoma, 14641475
Cardiac output (CO), 230, 231f, 1280, 1281
Cardiac Performance Analysis (CPA), 380
Cardiac plasmacytoma, 14901491, 1492f
Cardiac resynchronization therapy (CRT),
233234, 365, 1372

for heart failure, 369, 370f

LBBB and, 1377
Cardiac rhabdomyoma, 14801481
Cardiac sarcoidosis, 3D speckle tracking
and, 376t
Cardiac shunts, 171
Cardiac situs, 1531f
Cardiac tamponade, 14381440, 1970
blunt force, 1970

cardiac involvement, 1970

clinical presentation, 1970

and HV Doppler, 317, 317f

left ventricular outflow tract

diameter, 1974

myocardial contusion, 1970

pericardial effusion, 1970

pulsed wave Doppler, 1443f

and PV Doppler, 341

right heart chamber collapses in,
1442t

right ventricular wall, 1971t

transmitral inflow velocities, 1970

transtricuspid velocities, 1970

tricuspid valve, 1971t
Cardiac transplantation
2D STE and, 371

velocity vector imaging in, 399400,
399f
Cardiac tumors and masses, 14621523

atrial thrombus, 1504

cardiac thrombi, 1504

cardiac vegetation, 15061511
Chiari network, 1503

computed tomography for, 20512054
Coumadin ridge, 15031504

crista terminalis, 1503

differential diagnosis of, 14621463

echocardiographic assessment of,
14621464

3D TTE, 1464

full-volume acquisitions, 1463

limitation of, 1463

papillary fibroelastomas, 1463,
1476f1477f

Eustachian valve, 1503

Index

left ventricular thrombus, 15041506

malignant primary cardiac tumors,
14841511

angiosarcoma, 14851486

cardiac plasmacytoma, 14901491,
1492f

fibrosarcomas, 1488

hydatid cyst, 1491, 1493f1498f

pericardial mesotheliomas, 1491

primary cardiac lymphoma, 1490

rhabdomyosarcoma, 14861488

sarcomas, 14841488

mesothelial/macrophage incidental
cardiac excrescences, 1511
1518

cardiac calcified amorphous tumor,
15121514

extracardiac masses, 1514, 1514f

intracardiac hardware, 15141517

mitral annular calcification, 1512,
1512f

moderator band, 1503

perivalvular abscess, 1511

primary benign cardiac tumors,
14641484

benign cardiac fibromas, 1480

cardiac hemangiomas, 14821484

cardiac lipomas, 14811482, 1483f

cardiac myxoma, 14641475

cardiac rhabdomyoma, 14801481

papillary fibroelastomas, 14751480

secondary cardiac tumors, 14921500

carcinoid heart disease, 1500

malignant melanoma, 1500

thebesian valve, 1503
Cardiomyopathies. See also specific
Cardiomyopathy

diabetic, 1407, 1408

dilated. See Dilated cardiomyopathy
(DCM)

2D STE and, 369371

hemochromatosis, 1405

infectious, 14051407

HIV-associated cardiomyopathy,
1407

septic cardiomyopathy, 14051407

infiltrative, 1405

left ventricular noncompaction,
13841388

echocardiographic features of,
13851388, 1386f1387f

isolated, 1387f

normal fetal ontogenesis, 1385

metabolic, 1407

in neuromuscular disorders, 396

peripartum, 13811384, 1381f1384f

primary, 1370

sarcoidosis, 1405, 1405f

secondary, 1370

tachycardia-induced, 1388, 1395f

Takotsubo cardiomyopathy, 1388,
1394f

toxic, 13961397
adriamycin-induced
cardiomyopathy, 1397f

alcohol-induced cardiomyopathy,
1397
chemotherapy-induced
cardiomyopathy, 13961397

two-dimensional echo, 1407
Cardiopulmonary bypass, and HV
Doppler, 317, 318f
Cardiopulmonary ultrasound, 1987

acute lung injury/ARDS, 1987

B-lines, 1987

evaluation, 1987

cardiogenic pulmonary congestion,
1987

echocardiography, 1987

echography, 1987

lung ultrasound scan, 1987

addition of, 1987

findings, 1987

helps in, 1987

pathological conditions, 1987

pulmonary fibrosis, 1987

three B-line scenarios, 1987, 1987t

valvular heart disease, 1987
Cardiothoracic ratio, 1531f
Cardiovascular Credentialing
International (CCI), 754
Carditis, diagnosis of, 765775

autopsy, 771f

echo and Doppler studies, 768770
echocardiography

superiority of, 773774

two-dimensional, 770772

vs. clinical examination, 767768

echo interrogation, 767, 769t770t

M-mode in, 770

myocarditis, 766

pericarditis, 766

pitfalls in Jones criteria, 766767

regurgitation, 766

rheumatic vs. nonrheumatic
regurgitation, 768

secondary prophylaxis, fidelity of, 775
Carotid artery, 673f
Carotid bulb, 667, 668f

Carotid intima-media thickness (CIMT),

471, 676677, 678f
Carotid ultrasound examination. See
Peripheral vascular ultrasound
Carpentier, Alain, 580
Catheter-based LAA closure, 512
Catheter-based transcutaneous
interventional procedures,
531532

for device closure of cardiac shunts,
548

atrial septal defects (ASDs), 550555

closure of PFOs, 555, 557

patent ductus arteriosus (PDA),
548550

ventricular septal defects (VSDs),
557559

fluoroscopy versus echocardiography
in, 532

guidance of electrophysiology
ablation procedures, 566,
568569

miscellaneous procedures, 569

alcohol septal ablation, 571

left ventricular pseudoaneurysm
closure, 569, 571, 571f

right ventricular endomyocardial
biopsy, 571

occlusion of left atrial appendage
(LAA), 559561

epicardial suturing of LAA, 563, 566

intracardiac device closure of LAA,
561, 563

transseptal puncture, 532533

for valvular disease

aortic stenosis, 540546

mitral regurgitation, 538540, 541f,
542f

mitral stenosis, 533537, 537f539f

paravalvular prosthetic leaks, closure
of, 546548, 550f, 551f
CCAM. See Congenital cystic

adenomatoid malformation (CCAM)
CCB. See Calcium channel blockers
(CCB)
CCTA. See Coronary computed
tomographic angiogram (CCTA)
CCTGA. See Congenitally corrected
transposition of great arteries
(CCTGA)
CDG. See Congenital disorders of
glycosylation (CDG)
CDH. See Congenital diaphragmatic
hernia (CDH)

I-IX

Celiac artery, transesophageal imaging

of, 970
Central processing unit (CPU), 60
Certified Cardiographic Technician
(CCT), 754
Cervical aortic arch, 1691
Cervical arteries Doppler spectral
waveforms, 674f
CFR. See Coronary flow reserve (CFR)
CFVR. See Coronary flow velocity reserve
(CFVR)
Chagas disease, 18751876, 1877f1878f
Chemotherapy cardiotoxicity, 2D STE
and, 371372
Chemotherapy-induced cardiomyopathy,
13961397
Chest trauma, 1969

cardiac injuries, 1969

penetrating injuries, 1969

perioperative indications, 1970t

unintentional injury, 1969
Chiari network, 1503, 17761779
Chordae rupture, 1015

2D transthoracic echocardiography,
1012f, 1013f
Chordal rupture, 857f
Chordoma, in right ventricle, 1502f
Chorionic villus biopsy (CVS), 1528
Christian Dopplers principle, 26, 26f
Chronic aortic stenosis, myocardial
response to, 920
Chronic effusive pericarditis, 1437
Chronic irreversible verus dynamics,
13561358
Chronic ischemic heart disease
echocardiography role in, 1298
1301

ventricular function in, 13001301

diastolic function assessment,
13001301

systolic function assessment, 1300
Chronic kidney disease (CKD). See Renal
disease
Chronic obstructive pulmonary disease
(COPD)

and HV Doppler, 306, 307f

squatting stress echocardiography in,
1323
Chronic thromboembolic pulmonary

hypertension (CTEPH), 1073t, 1074
Circle of Willis, 669, 670f
Circulation, 13
Circumferential strain, 362, 363f, 389
Cleft mitral leaflet, 1613

I-X

Comprehensive Textbook of Echocardiography

Clinical cardiac ultrasound, development

of, 47
Clipping, 89
Clutter, 81
CoA. See Coarctation of aorta (CoA)
Coaptation depth, 1423, 1424
Coarctation of aorta (CoA), 963, 964f,
16921694, 17701773

in adults, 18261829

cardiac catheterization, 1829

echocardiography, 18271828

MRI/CT for, 18281829

postoperative adult, 1829

surgical intervention for, 1829

treadmill stress testing, 1828

Doppler feature of, 1694
Coherent contrast imaging (CCI), 420
Collateral pathways, 669671, 670f
Color Doppler, 64, 113, 113ff, 114f
Color Doppler imaging, three-

dimensional echocardiography and,
248, 254255, 266f
Color Doppler ultrasound

history of development of, 11
Color flow Doppler

advantages and disadvantages of, 71

methodology, 71, 71f

transthoracic echocardiogram, 137
Color maps, 71. See also Color flow
Doppler
Color processor, 71
Combined biventricular output (CVVO),
1529
Comet tail artifact, 734
Common atrium, 17471751, 1751f1752f
atrial septum and, 1800
Common carotid artery (CCA), 665
Complex congenital heart defects,
18261848
Complex congenital heart defects, in
adults, 18261848
CCTGA, 18401842, 1842t

cardiac catheterization, 1841

echocardiography, 18401841

exercise testing with stress
echocardiography, 1841

magnetic resonance imaging, 1841

postoperative adult, 1842

surgery for, 18411842

coarctation of aorta, 18261829, 1828t

cardiac catheterization, 1829

echocardiography, 1827

MRI/CT for, 18281829

postoperative adult, 1829

stress echocardiography, 1828

surgical intervention for, 1829

transesophageal echocardiography,
18271828

treadmill stress testing, 1828

double outlet right ventricle, 1824,
1825

D-transposition of great arteries,
18351840, 1837t

cardiac catheterization, 1838

echocardiography, 18361838

MRI/CT for, 1838

postarterial switch repair, 1838

postatrial switch repair, 1836

reoperation for, 18381840

stress echocardiography, 1838

tetralogy of Fallot, 18291835, 1833t

cardiac catheterization, 18341835

echocardiography, 18301832

MRI/CT for, 1835

postoperative adult, 1835

postoperative adult, surgery in, 1835

stress echocardiography, 18321834

truncus arteriosus, 18421844, 1843t

cardiac catheterization, 1844

echocardiography, 1843

MRI/CT for, 1844

postoperative adult, 1844

surgery for, 1844

univentricular hearts, 18451848

cardiac catheterization, 18471848

echocardiography, 18461847

MRI /CTA for, 1847

transesophageal echocardiography,
1847

tricupsid atresia and post-fontan
adult, 1845, 1846f
Computational fluid mechanics, 67
Computed tomography angiogram (CTA)
flow limiting disease, accuracy in,
2044

preoperative, 2049, 2050f

retrospective gated, 2043f
Congenital anomalies
of coronary arteries, 13431344

of mitral valve, 16101615

individual mitral lesions, evaluation
of, 16111615

mitral valve lesions,
echocardiographic views,
16101611

types of, 1611t

of tricuspid valve, 16161618

Index

congenitally unguarded

tricuspid orifice, 16171618, 1617f

Ebsteins anomaly of, 16161617,
1616f

tricuspid valve prolapse, 1617
Congenital aortic stenosis, 17661770
Congenital bicuspid aortic valve,
16191620, 1619f
Congenital complete heart block (CCHB)
fetal, 1529, 1545
Congenital cystic adenomatoid
malformation (CCAM), 1530
Congenital diaphragmatic hernia (CDH),
1530
Congenital disorders of glycosylation
(CDG), 1407
Congenital double orifice mitral valve, in
mitral stenosis, 844845
Congenital heart defects, simple, in
adults, 17981813

shunt lesions, 17981813

atrial septal defects, 17991802, 1801t

atrioventricular septal defect,
18091811

coronary artery fistula, 18121813

patent ductus arteriosus, 18051809

patent foramen ovale, 17981799

persistent left superior vena cava,
18101811

sinus of Valsalva aneurysm, 1811
1812

ventricular septal defects, 18021805
Congenital heart disease (CHD), 1733
1790, 19001902

aortic arch anomalies, 17701773

aortic arch to pulmonary artery
fistula, 1773

coarctation of aorta, 17701773

aortopulmonary window, 1751

atrial and atrioventricular valve
abnormalities, 17731776

cor triatriatum sinister, 17731775

Ebsteins anomaly, 17751776

isolated mitral valve cleft, 1775

atrial septal defects, 19001901
Chiari network, 17761779

common atrium, 17471751,
1751f1752f

computed tomography for, 20592063

conotruncal anomalies, 17541766

anomalous coronary artery, 1763
1766

tetralogy of fallot, 1763

transposition of great arteries,
17541763

dextrocardia, 15701575, 1570f

Bernoulli equation, limitations of,
1574

color flow Doppler, 15701572

color flow information, 1572

continuous wave Doppler, 1572

Doppler gain and filter settings,
inappropriate, 1571f, 1572f,
1573

Doppler scales, inappropriate, 1573,
1573f

echo gradients during cardiac
catheterization, 1574, 1575f

nonimaging continuous wave

Doppler probe, pitfalls of, 1573

poor echo windows, 1572

pulsed Doppler, 1572

signal alignment, inappropriate,
1573, 1574f

transducer frequency, inappropriate,
1573

trivial lesions, pressure gradients
across, 1574

double outlet right ventricle, 1779
1783

left ventricular-RA communication,
17791782

right coronary artery fistula, 1782
1783

echocardiography (imaging) of,
15631570

apical view, 15661567, 1566f

conventional views of, 1563t

high parasternal or ductal view, 1569,
1569f

long-axis view, 1569, 1569f

parasternal long-axis view, 1567,
1567f

parasternal short-axis view, 1568
1569, 1568f, 1569f

pitfalls in, 15711572, 1571t

short-axis view, 1570, 1570f

subcostal window, 15631566,
1564f1565f

suprasternal views, 1569, 1569f, 1570f

and HV Doppler, 317318, 318f

other abnormalities, 17761779

outflow tract obstruction, 17661770

congenital aortic stenosis/bicuspid
aortic valve, 17661770

subaortic stenosis, 1770

overview, 1733

patent ductus arteriosus, 17511754,
1755f1757f

patient preparation, 15621563, 1562f

pulmonary hypertension, 19011902

RT 3DE approach to, 1170

sequential chamber analysis,
principle of, 15751582

atrial arrangement, identification of,
15751577

atrioventricular connection, 1577
1579

ventricular arterial connection,
15791582

shunt lesions/septal defects, 1733
1747

atrial septal defects, 1734

atrioventricular septal defectsts
17461747

secundum ASD, 17341742

sinus venosus ASD, 17421743

unroofed coronary sinus, 1743

ventricular septal defects, 17431746

sinus of Valsalva aneurysm, 1784

hypoplastic left heart syndrome, 1784

right ventricular outflow obstruction
and, 1784
Congenitally corrected transposition of
great arteries (CCTGA), 18401842

cardiac catheterization, 1841

echocardiography, 18401841

exercise testing with stress
echocardiography, 1841

magnetic resonance imaging, 1841

postoperative adult, 1842

surgery for, 18411842

velocity vector imaging in, 392
Congenitally corrected transposition of

great vessels (CTGA), 1664, 1664f
Congenitally stenotic tricuspid aortic
valve, 1620
Congenitally unguarded tricuspid orifice,
16171618, 1617f
Congenital mitral stenosis, 844
Congenital parachute mitral valve, 845,
846f
Congenital subaortic membrane, 1359
Conotruncal anomalies, 17541766

anomalous coronary artery, 1763
1766

tetralogy of fallot, 1763

transposition of great arteries,
17541763
Constrictive pericarditis (CP), 14441448

Doppler flow velocity records,
14461448, 1447f

3D TTE vs. 2D TTE, 14561457

I-XI

echocardiographic findings in, 1445t
effusive, 1448f

hepatic vein and, 1447f

and HV Doppler, 315317, 316f, 317f

M-mode and 2D echo, 1444, 1445f,
1446f

and PV Doppler, 340341, 340f, 341f
Continuity equation, 923

for aortic valve area, 906907

for MVOA, 789
Continuous infusions, 421
Continuous wave Doppler, 6364. See
also Spectral Doppler

disadvantage of, 6869

methodology, 6869, 69f
Continuous-wave jet intensity, MR and,
869
CONTISCAN transducer, 224237
use of, 224225, 225f

acute coronary syndromes,
evaluation of, 226

ambulatory echocardiography,
227229

balloon valvuloplasty, monitoring of,
233

cannulation of coronary sinus,
monitoring of, 233236

exercise echocardiography, 226227

intraoperative monitoring, 236237

noninvasive hemodynamic
monitoring, 229230

pericardiocentesis, monitoring of,
230233
Contractile reserve, assessment of, 276
Contrast CMR (gadolinium), 2020
Contrast defect area (CDA), 423
Contrast defect length (CDL), 423424
Contrast echocardiography, 416436,
737, 1308. See also Myocardial
contrast echocardiography (MCE)
contrast administration

bolus injection, 421

continuous infusion, 421

contrast agents, 416417, 417f

commercially available, 417, 418t

idle, properties of, 417

safety of, 434435, 435t

ultrasound and, interaction between,
418419, 419f

use of, 428431

contrast imaging, physics of, 417418,
418f

history of development of, 1113

imaging modalities, 420, 420f

I-XII

Comprehensive Textbook of Echocardiography

intermittent/triggered imaging,
420421

real time imaging, 420

indications for, 426

enhanced endocardial border
delineation (EBD), 426, 426f

left ventricular opacification (LVO),
426427, 427f429f

myocardial perfusion, evaluation of,
427428, 429f

myocardial perfusion, principles of
assessment of, 421422, 422f

contrast modality during MCE,
422423

qualitative MCE, 423, 424f, 425f

quantitative MCE, 424, 426

semiquantitative MCE, 423424, 425f

and problems, 431

attenuation artifacts, 431433, 432f,
433f

blooming, 433, 434f

swirling, 433, 434f

thoracic cage artifacts, 433434, 434f

saline, 435, 436f

training in, 757
Contrast microbubbles, 416417
Contrast score index, 441
COPD. See Chronic obstructive
pulmonary disease (COPD)
CoreValve, self-expandable, 541542, 543
Coronary aneurysms, 16881689

atresia of left main coronary artery,
1689

Kawasaki disease, 16881689
Coronary anomaly, 1344f
Coronary arteries, 13371347

anatomy and physiology, 1337

congenital abnormalities of, 1343
1344

coronary flow reserve, 13401343

coronary ostia, 1338

coronary stenosis, 1338

distal coronary flow, 13401343

normal left main, 1339f

overview, 1337

parasternal short-axis view for, 1569,
1569f

proximal, 1338f, 1339f, 1341f, 1341t

visualization of, 13371340

transesophageal echocardiography,
13381340

transthoracic echocardiography,
13371338
Coronary arteriovenous fistulas, 1688,
1689f

Coronary artery anomalies, 16841688

anomalous pulmonary origin
of coronary artery, 16851687

of right coronary artery, 1687

tangential origin of coronary artery,
16871688
Coronary artery disease (CAD)
ALVRM on squatting and, 1325

2D STE and, 367

evaluation of, 226

squatting SE vs. dobutamine SE for,
1325

squatting stress echocardiography in,
1323

three-dimensional vs. two dimensional in, 1332t

velocity vector imaging in, 396398
Coronary artery distribution, 1116f
Coronary artery fistula, 20482049

in adults, 18121813

sequelae of, 2049
Coronary calcium scoringm, in computed
tomography, 20442045, 2045f,
2046f
Coronary computed tomography

angiogram (CCTA), 2035f. See also

Cardiac computed tomography

contraindications for, 2027

coronary plaque, 20412042

diagnostic accuracy of, 20402041

flow limiting disease, accuracy in,
2044

in ICM/NICM, 1427

optimization of, 2029t

optimization of anomalous, 2049t

prognostic information from, 2042

protocol on 64 detector scanner,
2028t
Coronary fistulas, 13431344

pediatric type, 1345f
Coronary flow reserve (CFR), 13401343

conditions decreasing, 1342t

impairment of, 1343
Coronary flow velocity reserve (CFVR),
446, 1337, 13411343, 1344

conditions impairing, 1342t

noninvasive evaluation of, 13411342

normal, 1343f
Coronary ostia, 1338
Coronary sinus

defect in wall of, 16791680

partial or completely unroofed, 1683
Coronary sinus aneurysm/diverticulum,
1683

Index

Coronary sinus atrial septal defect, 1586

Coronary sinus cannulation, monitoring
of, by CONTISCAN transducer,
233236, 235f, 236f, 236t
Coronary stenosis, 1338, 1340f

cut-off values for, 1341t

in elderly, 19431946, 1944f, 1946f

in elderly female, 1897f1899f
Cor triatriatum sinister, 17731775
Cosine correction, 67f
Coumadin ridge, 15031504
Crab view, 184, 185f
Crista terminalis, 188, 1503
Critical neonatal aortic stenosis, 1622
1623
Cropping, 241
Cropping, 3D echocardiography and,
269270, 269f
CRT. See Cardiac resynchronization
therapy (CRT)
CT-based fractional flow reserve (FFR CT), 2044
CT dose index volume (CTDIvol), 2026
CTEPH. See Chronic thromboembolic
pulmonary hypertension (CTEPH)
Curie, Pierre, 4
Curvilinear probe, 58, 58f
CVS. See Chorionic villus biopsy (CVS)
CVVO. See Combined biventricular
output (CVVO)
CWP. See Capillary wedge pressure
(CWP)
Cystic medial degeneration (CMD), 1936

D
Dark blood imaging (spin echo), 2020
Data acquisition methods, 3D
echocardiography

cropping, 269270, 269f

3DE color flow Doppler imaging, 269

image display, 269270

multiplane mode, 268269

multiple-beat 3DE imaging, 269

real time 3DE, 269

tomographic slices, 270, 270f

3D beamformer, 7778

DDD pacing, for obstructive HCM,
1363
Debakey classification, 1974

aortic arch, 1974

ascending aorta, 1974

proximally, 1974
Deceleration time (DT), 313

Decompressive venous channels, 1684

Definity, 417, 418t. See also Contrast
echocardiography
Degenerative disease, 882f
Degenerative mitral regurgitation, 863
Degenerative mitral valve stenosis, 844
DENSE. See Displacement encoding with
stimulated echoes (DENSE)
Depth, and frame rate, 59, 60f, 61
Dextro Transposition of great arteries
(dTGA), 1528, 1549f, 1759f1763f
Dextrotransposition of great vessels, 6
DHF. See Diastolic heart failure (DHF)
Diabetes, velocity vector imaging in, 398
Diabetic cardiomyopathy, 1407, 1408
Diastolic dysfunction

cardiac motion and, 12011202

grade I, 1128

grade II, 1128

HCM and, 1356

identification of, 11261130

left ventricular hypertrophy patient
with, 1125f
Diastolic heart failure (DHF), 11241125
Diastolic pulmonary artery pressure
(DPAP), 1065t, 1066, 12701272,
1272f
DICOM format, 90, 95
Diet drug-induced valvulopathy, 1006
Digital Imaging and Communication
(DICOM), 391
Dilated cardiomyopathy (DCM), 394395,
445446, 447f, 12021203, 1370
1372, 1371f

cardiac MRI for, 2008f

chamber enlargement in, 1370

decompensated, 1374

etiology of, 1370, 13791397

familial, 13791381

ischemic cardiomyopathy, 1388
1395, 1395f

left ventricular contractility, 1370
1372

mitral inflow filling pattern, 1376

mitral regurgitation, 1376

optimizing heart failure,
echocardiography role in,
13761379

secondary findings in, 13721376

atrial fibrillation, 1376

cardiac dimensions, 13721374, 1373f
Doppler echocardiography, 13751376

pericardial and pleural effusion, 1374

two-dimensional and M-mode
findings, 1375, 1375f

systolic function in, 1371

ventricular remodeling, 1379

wall motion abnormalities in,
13711372
Dilated pulmonary valve, 1555f
Dipyridamole stress test, 1334
Discrete subaortic membrane,
1771f1772f
Displacement encoding with stimulated
echoes (DENSE), 1144
Distal coronary flow, 13401343
D-malposition

of aorta, 1548f

of great arteries, 1548f
Dobutamine stress echocardiography
(DSE), 274, 1307
Dobutamine stress test, 13311333
Dominance, 1181
Doppler, 1993

across-line Doppler shifts, 1993

beam-to-vessel angle, 1993

color Doppler, 1993

postacquisition, 1993
Doppler, Christian, 9
Doppler angiography. See Power Doppler
Doppler equation, 65, 66
Doppler hemodynamics, with stress
echocardiography, 13181319

aortic valve disease, 1318

dynamic pulmonary hypertension,
13181319, 1319f

hypertrophic cardiomyopathy, 1318

latent diastolic dysfunction, 1318

mitral valve disease, 1318
Doppler principle, 349
Doppler shift, 65
Doppler spectral display, 6768
Doppler tissue imaging. See Tissue
Doppler
Doppler ultrasound, 911, 6364, 6573

color Doppler, 64, 71

continuous wave Doppler, 6364,
6869
Doppler velocity determination, 7273

history of development of, 911

information from, 73

instrumentation, 6668

physical principles, 63

power Doppler, 7172

pulsed wave Doppler, 6364, 6970

tissue Doppler, 64, 72
Doppler velocity determination, 7273

errors in, 72
DORV. See Double outlet right ventricle
(DORV)

I-XIII

Dose length product (DLP), 2026

Double-balloon catheter
commissurotomy (CBC), 233
Double image artifacts, 736. See also
Artifacts
Double orifice mitral valve, 16141615,
1614f
Double outlet right ventricle, 17791783,
18441845

cardiac catheterization, 1845

echocardiography, 1844

left ventricular-RA communication,
17791782

MRI/CT for, 1845

postoperative adult, 1845

right coronary artery fistula, 1782
1783

stress echocardiography, 1845

surgery for, 1845
Double outlet right ventricle (DORV),
1580, 16441650

definition, 1645

echocardiography of, 1647

planning surgical strategy, role in,
16481650

great arteries and, 16451646

preoperative assessment of, checklist
for, 1648t

stenosis of pulmonary outflow,
16471648

aortic outflow obstruction, 1647

remote ventricular septal defect, 1648

ventricular septal defect, restriction
of, 1647f, 1648

subaortic ventricular septal defect in,
1647

subpulmonary ventricular septal
defect in, 1647

TaussigBing anomaly, 1647

ventricular septal defect, location of,
16451646

doubly committed, 1645

remote, 16451646, 1646f

subaortic, 1645, 1645f

subpulmonic, 1645, 1646f
Doubly committed ventricular septal
defect, TGA and, 1659
DPAP. See Diastolic pulmonary artery
pressure (DPAP)

3DSTE. See Three-dimensional
speckle tracking
echocardiography (3DSTE)
dTGA. See dextro Transposition of great
arteries (dTGA)
D-transposition of great arteries,
18351840

I-XIV

Comprehensive Textbook of Echocardiography

cardiac catheterization, 1838

echocardiography, 18361838

MRI/CT for, 1838

postarterial switch repair, 1838

postatrial switch repair, 1836

stress echocardiography, 1838

velocity vector imaging in, 392
Dual plane transesophageal echo (TEE)
probe, 99100, 100f
Duchenne muscular dystrophy (DMD),
396
Ductal arch (DuAr), 1537f
Ductus arteriosus, 548
Ductus venosus (DV), 1532f, 1538f
Duplex scanning protocol, carotid
ultrasound, 672f
DuraHeart magnetically levitated
centrifugal assist system, 1228
Dynamic pulmonary hypertension, stress
echocardiography in, 13181319,
1319f
Dysplasia of mitral valve, 1613
Dysplastic pulmonary valve, 1554f

E
Early cardiac flow Doppler era, 2449

color Doppler, development of,
3738, 41f44f

diastology, 4748

directional Doppler flowmetry, 2930

Doppler presentation, controversy
on, 3031

flow concept, emergence of, 2728

heart cavities, exploring of, 33, 34f

intracardiac Doppler flow velocity
traces changes, interpretation
from, 3334

mature flow Doppler era, 44, 4647

nondirectional flow Doppler
technique, 2829

peripheral artery recordings, lessons
from, 31, 33

preflow Doppler era

invasive procedures in, 25

noninvasive procedures in, 2526,
26f, 27f

pulsed Doppler with 2D
echocardiography,
combination of, 3637, 38f40f

transcutaneous approaches, return
to, 3436

Early gadolinium enhancement
(EGE), 2020

EBCT. See Electron-beam computed

tomography (EBCT)
Ebsteins anomaly, 986f, 17751776

associated with transposition of great
vessels, 1777f

2D transesophageal
echocardiography, 1025f1028f

of mitral valve, 1615

M-mode echocardiography, 986f

RT 3DTTE for, 1777f1779f

transthoracic echocardiography

three-dimensional, 1029f

two-dimensional, 1024f1025f

tricuspid valve, 16161617, 1616f
Echocardiography, 1957, 1969

ablation treatment, 1957

accessory pathways, 1957

angiography, 1958

coronary sinus contrast, 1958

arrhythmias, 1957

atrial fibrillation, 1957

atrioventricular, 1957

body surface area, 1958

cannulation, 1958

cardiac ablation, 1958

coronary sinus, 1957

Doppler in, 6566. See also Doppler
ultrasound

echocardiogram, 1957

transthoracic, 1962f

echocardiographic modalities, 1957

electrophysiologist, 1957

electrophysiology, 1957

history of, 319

clinical cardiac ultrasound, 47

color Doppler ultrasound, 11

contrast echocardiography, 1113

2D echocardiography, 89

3D echocardiography, 1418

Doppler ultrasound, 911

perspective on, 19

tissue Doppler and speckle tracking
imaging, 14

transesophageal echocardiography,
13

ultrasound, 4

immediate invasive intervention,
1969

intracardiac echocardiography, 1957

midesophageal location, 1957

nodal re-entrant tachycardia, 1957

one-dimensional, 3f

ostium, CS,CS-RA, 1958

parasternal, 1958f

Index

pediatric, 6

persistent left superior vena cava,
1958

progress in, 5f

right atriumright ventricle, 1958

risk stratification, 1969

superior vena cava, 1958f

supraventricular tachycardia, 1957

supraventricular tachycardia, 1957

Thebesian valve, 1958

therapeutic, 1957

thoracic aorta, 1958f

transesophageal echocardiogram,
1957

transthoracic, 1969

transthoracic echocardiogram, 1957

tricuspid valve, 1958

valve of Vieussens, 1958
Echocardiography, in elderly, 19211956

aortic aneurysm, 19341937

cystic medial degeneration and, 1936

etiology of, 1936

natural history of, 1937

with rupture, 1936f

aortic atherosclerosis, 19211923,
1922f

aortic dissection, 19371942,
1937f1938f, 1941f

acute coronary syndrome and, 1940

color Doppler flow in, 1942

descending thoracic aorta, 1942

perfusing lumen, 1942

rupture, 1938f1940f

aortic stenosis, 19241934

aortic valve area, estimation of, 1926

2D echocardiography, 19241928,
1925f, 1927f

LFLG-AS with low EF, 1929f

live/real time 3D TTE, 19301933

mild, 1921f

PLFLG-AS with preserved EF,
19291930

prevalence and pathophysiology,
1924

severe, 1931f1932f

transcatheter aortic valve
replacement, 1934, 1935f

ventricular response to, 19331934

aortic valve sclerosis, 19231924,
1923f

bicuspid, 1931f

echocardiographic findings for,
19231924, 1930f

normal hemodynamics in, 1924

pathophysiology of, 19231924

prevalence of, 19231924

systolic murmur, 1923

coronary stenosis, 19431946, 1944f,
1946f

left ventricular mass, 19421943

mitral annular calcification, 1946
1948

overview, 1921

penetrating aortic ulcer, 19211923

prosthetic valves, 19481949, 1949f
Echocardiography, in women, 18861920

breast implant, 1887f

congenital heart disease, 19001902

atrial septal defects, 19001901,
1900f1901f

pulmonary hypertension, 19011092

echocardiographic measurements

vs. technical considerations, 1886
1888

ischemic heart disease, 18891893

overview, 1886

polycystic ovarian syndrome, 1899

stress echocardiography, 18941899

structural heart disease, 18881889

mitral valve calcification, 1889, 1895f

mitral valve prolapse, 1888,
1888f1889f

mitral valve stenosis, 18881889

Takotsubo cardiomyopathy, 1899
1900
Echocardiography training, 750760. See
also Training, in echocardiography
Echocardiography transducer, 55
Echo-clear space, 1440f
Echo-guided pericardiocentesis, 1443
1444
EchoPAC, 385
ECMO circuit. See Extracorporeal
membrane oxygenator (ECMO)
circuit
Edge-to-edge repair, 539
Edler, Inge, 45
Edwards Sapien Valve, 2057
Effective orifice area (EOA)

in calcific aortic stenosis, 900

patient prosthetic mismatch and,
10911092

of prosthetic valves, 10841087
Effective regurgitant orifice area (EROA),
518519, 992, 1038
Effusive-constrictive pericarditis, 1448
Eisenmenger syndrome, 1597f
Ejection fraction postamyocardial
infarction, 1165t

Elastography, 1994, 1995

acoustic radiation force impulses,
1995

attraction of, 1995

breast malignancies, 1995

human tissue, 1994

mechanical properties, 1994

shear wave, 1995, 1995f

advantage, 1995

strain elastography, 1995

tissues elastic properties

two ways to image, 1995

tissue stiffness, 1995
Electric safety, TEE probe equipment
and, 110111, 111f, 111t
Electromagnetic compatibility (EMC),
110
Electromagnetic interference (EMI), 112
Electron-beam computed tomography
(EBCT), 2023

in ICM/NICM, 1427
Emergency department setting, MDCT
in, 20452047
EMF. See Endomyocardial fibrosis (EMF)
End-diastolic volume (EDV), 11561157
Endocardial border delineation (EBD),
421, 426
Endocardial cushion defect, 1535
Endocardial trabecular contouring
algorithms, 11561157
Endocarditis

pacemaker associated, 1214

of prosthetic valves, 10991100
Endocarditis prophylaxis, HCM and,
13631364
Endomyocardial fibrosis (EMF), 1402
1404
Endothelial cells, 449450, 449f

dysfunction, 451. See also
Endothelial dysfunction

function of, 450, 450ff, 451t
Endothelial dysfunction, 449474. See
also Flow-mediated dilatation
(FMD)

assessment of, methods for, 455457,
456f, 456t

and carotid intimal medial thickness,
471, 474f

concept of FMD

case studies on, 459462

limitations of, 458459, 663

conduit arteries, ultrasound imaging
of, 456

cuff inflation pressure, 457

duration of occlusion, 457

I-XV

measurement of flow-mediated
dilatation, 457, 457f

proximal and distal occlusions,
456457

quantification of shear stress, 457

technique of FMD in brachial artery,
458t

factors affecting, 451, 451t

future directions, 471, 474

NO release and, 455, 455f

other methods for endothelial
function assessment, 465

laser Doppler flowmetry, 469470

low flow-mediated vasoconstriction,
470471, 470f474f

peripheral arterial tonometry,
467469, 468f, 469f

pulse wave velocity analysis, 466467,
466f, 467f

shear stress and flow-mediated
dilatation, 452454, 452f

shear stress and FMD response,
analysis of, 457458, 457f

vasoactive molecules in
vasoregulation, 454

endothelins, 454
endothelium-dependent
hyperpolarizing factor, 454

nitric oxide, 454

prostacyclin, 454
Endothelial progenitor cells (EPCs), 450,
450f
Endothelin (ET), 454
Endothelium-dependent hyperpolarizing
factor (EDHF), 454
Endovascular Valve Edge-to-Edge Repair
Study (EVEREST II) trial, 539
End-stage renal disease (ESRD), 1871
1872, 1872f
End-systolic volume index (ESVI), 180,
1379

ICM/NICM and, 14211422
Energy Doppler. See Power Doppler
Enhancement, 61
eNOS (type-III NO-synthase), 454
EOA. See Effective orifice area (EOA)
Epiaortic ultrasonography (EAU),
638641

in aortic pathology

aortic atherosclerosis, 640641, 641f

aortic dissection, 641

epiaortic probe and preparation,
638639, 639f, 640f

imaging views/planes, 639640, 640f

indications for, 638

three-dimensional, 641

I-XVI

Comprehensive Textbook of Echocardiography

Epicardial fat pad, 1518f

E-point septal separation (EPSS), 169,
1117, 1375, 1375f
EPSS. See E-point septal separation
(EPSS)
EROA. See Effective regurgitant orifice
area (EROA)
Esophagus, 967
ESVI. See End-systolic volume index
(ESVI)
Eulerian strain, 389
European Association of Cardiovascular
Imaging (EACI), 91, 750, 751, 757,
758
European Association of
Echocardiography (EAE), 132, 296, 907
European Society of Cardiology (ESC),
751
European Union (EU), 110
Eustachian valve, 1503
EvaHeart left ventricular system, 1228
1129, 12281229
Exercise echocardiography, 226227,
227f, 228f, 1307
External carotid artery (ECA), 665666
Extracardiac masses, 1514, 1514f
Extracorporeal membrane oxygenator
(ECMO) circuit, 1127
Extravascular lung water (EVLW), 1982

F
Fabry disease, 352, 14011402, 1401f
False lumen (FL), 1977f
FAPS. See French Aortic Plaque in Stroke
(FAPS) study
Fast Fourier transform (FFT), 67, 113
Fat pad, 170
Fatty infiltration of liver, 318
FDA 510 (K) Track 3 regulations, 111
Feigenbaum, Harvey, 6
Feinstein, Steve, 13
Fetal bradycardia, 1545
Fetal cardiac function, assessment of, by
velocity vector imaging, 390391
Fetal cardiac imaging, 15271560

congenital heart disease, family
history of, 1529

fetal bradycardia, 1545

fetal cardiac evaluation, indications
for, 1528

fetal cardiology, scope of, 15271528

fetal echocardiography, indications
for, 1529

fetal heart disease, 15291530

congenital cystic adenomatoid
malformation, 1530

congenital diaphragmatic hernia,
1530

sacrococcygeal teratoma, 1530

twin reverse arterial perfusion, 1530

twintwin transfusion syndrome,
15291530

fundamentals of, 15301531

caval long-axis view, 1541

core and cord Dopplers, 1542

ductal and aortic arch views, 1541

fetal cardiac function, 15411542

fetal ultrasound, safety of, 15461547

four-chamber view, 15311541

rhythm assessment, 15421545

short-axis views, 15401541

three-dimensional imaging, 1549
1553

ventricular long-axis view, 1541

overview, 15271560

physiology, 15281529

prenatal counseling in, 1528

reasons and associations for, 1529
1530
Fetal echocardiography, in women,
19061919, 1906f1909f
Fetal heart abnormality, 1529
Fetal heart disease, fetal imaging,
15291530

congenital cystic adenomatoid
malformation, 1530

congenital diaphragmatic hernia,
1530

sacrococcygeal teratoma, 1530

twin reverse arterial perfusion, 1530

twintwin transfusion syndrome,
15291530
FFT analyzer, 67
Fibrinous adhesions, 14561457
Fibroma, 2051
Fibromuscular collar, 1625
Fibrosarcomas, 1488
First pass imaging (perfusion), 2020
Fixed anatomical obstruction, TGA and,
16601661
Fixed subaortic obstruction, 16241625
Flail tricuspid valve, 10071029

3d transthoracic echocardiography,
1012f

in male with dyspnea, 1012f
Flial posterior mitral valve leaflet, 853f
Flow convergence method, MR severity
and, 870871

Index

Flow image, 71
Flow-mediated dilatation (FMD),
452454, 452f454f. See also
Endothelial dysfunction
clinical utility of, 465, 466t

factors affecting

ACE-inhibitors, 465

age and sex, 463

coronary risk factors, 463464

diet, antioxidants, and supplements,
465

exercise, 465, 465f

hyperglycemia, 464

infections, 465

smoking, 464, 464f

sympathetic over activity, 465

unstable angina, 464465, 465f

weight loss, 465
FMD response, 452
Focus, and TEE image quality, 106, 106f
Fontan procedure, and HV Doppler, 318,
318f
Food and Drug Administration (FDA),
13, 110
Fossa ovalis

anatomy of, 533, 535f

2D transesophageal examination,
988f
Fossa ovalis atrial septal defect, 1586

for percutaneous device closure, 1591
Four-chamber asymmetry, 1548f
Fourier, Joseph, 68
Fractional area change (FAC), 1166
Frame, 59
Frame rate, 59

depth and, 59, 60f

image line density and, 59

sector angle and, 59, 60f
Framingham Heart Study, 1161
French Aortic Plaque in Stroke (FAPS)

study, 1921
Frequency, 55

in clinical use, 57

and TEE image quality, 104105, 105f
Fully sampled matrix-array transducers,
77, 268
Functional mitral regurgitation (FMR),
1372

DCM and, 1376, 1376f
Fundamental imaging, 57

apical four-chamber view, 57f

G
Gall stones, 220
Gating, 80

Gelatin-encapsulated nitrogen bubbles,

13
Gender,effect of, on HV Doppler, 303
Geometric orifice area (GOA), 900
Gerbode defect, 1603, 1603f, 1604f
Geroulakos classification, of ultrasonic
plaque, 678679, 681f
Ghost image, 61
Global circumferential peak systolic
strain (GCS), 1728
Global circumferential strain (GCS),
9192
Global longitudinal peak systolic strain
(GLS), 1728
Global longitudinal strain (GLS), 91, 91f,
273, 1165
Global radial peak systolic strain (GRS),
1728
Global strain, 712714, 712f715f
GLS. See Global longitudinal strain (GLS)
Goose neck deformity, 1746
Gore-Helex atrial septal occluder, 553,
554f
Gorlin equation, 923
Great vessels, identification of, 15791580

apical view, 1579

connection of, 15791580

PLAX view, 1579
GSPECT. See 99mTc-sestamibi gated
single-photon computed emission
tomography (GSPECT)

H
Haemophylus influenzae, 1043
Hand-carried ultrasound device (HCD),
752
Harmonic imaging, 57

apical four-chamber view, 57f
HeartAssist system, 1228
Heart chamber segmentation algorithms,
706707, 707f
Heart defects, in fetus, 15271528
Heart failure with preserved ejection
fraction (HFnlEF), 1122
HeartMate II, 694, 1228
HeartWare ventricular system, 1224f,
1228
Heating safety, TEE probe equipment
and, 111
Hemangioma

involving mitral valve, 1485f

left atrial, 1487f

right ventricular, 1486f

Hematoma, 1517f

after femoral artery access, 696, 697f
Hemochromatosis, 1405
Hemodynamometer, 67
Hepatic veins (Hep V), 1532f

anatomy of, 299300, 300f

versus biliary ducts, 305, 305f

blood flow in, 300, 301f

aging and, 303

Doppler V wave, 300, 302f

exercise and, 303

factors affecting, 302303, 303f

gender and, 303
HV Doppler measurements, 302, 302f

normal, 300, 301f

physiology of, 300302, 301f, 302f

pregnancy and, 303

respiration and, 302303, 303f

two components of systolic flow, 300,
301f

challenge to use of HV Doppler,
319321, 320f

disease states, and HV flow pattern,
305

atrial fibrillation, 309, 309f

atrioventricular dissociation, 308,
308f

cardiac tamponade, 317, 317f

cardiopulmonary bypass, 317, 318f

chronic obstructive lung disease, 306,
307f

congenital heart disease, 317318,
318f

constrictive pericarditis, 315317,
316f, 317f

liver disease, 318319, 319f

premature ventricular contractions,
309, 309f

prolonged PR interval, 308, 308f

pulmonary hypertension, 311, 313,
313f

restrictive cardiomyopathy, 315, 315f

right ventricular diastolic
dysfunction, 313315, 315f

right ventricular end-diastolic
pressure, assessment of,
309310, 310f

right ventricular systolic dysfunction,
313, 314f

short PR interval, 307, 308f

sinus bradycardia, 306, 307f

sinus tachycardia, 306, 307f

tricuspid regurgitation, 310311, 311f,
312f

I-XVII

tricuspid stenosis, 311, 312f

heterotaxy syndrome and, 1706
HV Doppler flow patterns, schematic
drawing of, 321f

imaging of, 299302

pulse Doppler across, 1539f

spectral Doppler of, 319321

versus superior vena cava, Doppler
pattern of, 304, 304f

transesophageal echocardiography,
305

transthoracic echocardiography, 304,
305f

visualization and recording of HV

Doppler, technical considerations in,
305, 305f, 306t
Hepatic vein systolic filling fraction
(HVFF), 1268
Hertz, Hellmuth, 45
Heterotaxy syndrome, 17041709

anatomy, 1704

cardiac and extracardiac anomalies
in, 1705t

initial echocardiogram of, 17041709

abdominal situs and cardiac position,
17041705

aortic arch and branches, 1709

atria and appendages, 1707

atrial septum, 1707

atrioventricular junction, 17071708

branch pulmonary arteries, 1708
1709

pulmonary blood flow, sources of,
17081709

venoatrial connections, 17051707

ventricles and ventricular septum,
1708

ventriculoarterial connections, 1708

overview, 1704

venoatrial connections in, 1706t
Hewlett Packard SONOS 5500, 227
HFnlEF. See Heart failure with preserved
ejection fraction (HFnlEF)
High-flow, high-gradient aortic stenosis,
920. See also Aortic stenosis
Highly focused ultrasound (HIFU), 1996
High MI imaging, 420421
High-pass filtering, 6667
High pulse repetition frequency (HPRF),
64
HIV-associated cardiomyopathy, 1407
HLHS. See Hypoplastic left heart
syndrome (HLHS)
HOCM. See Hypertrophic obstructive
cardiomyopathy (HOCM)
Hodgkin lymphoma, 1491

I-XVIII

Comprehensive Textbook of Echocardiography

Homograft aortic prosthesis, 1100f

Human hearing, 55
HVFF. See Hepatic vein systolic filling
fraction (HVFF); HV systolic filling
fraction (HVFF)
HV systolic filling fraction (HVFF), 1144
Hydatid cyst, 1491, 1493f1497f,
1493f1498f

pericardial, 1497f1498f
Hypereosinophilic cardiomyopathy, 1402
Hypereosinophilic syndrome, 18681869,
1870f
Hypertension, three-dimensional speckle
tracking and, 376t
Hyperthyroidism, 1879, 1880f
Hypertrophic cardiomyopathy, 395

three-dimensional speckle tracking
and, 376t
Hypertrophic cardiomyopathy (HCM),
13481368

alcohol septal ablation, 13621363

apical, 1356

definitions and types of, 13491356

diastolic dysfunction, 1356

differential diagnosis, 13591360

athletes heart, 13591360, 1361f

LV noncompaction, 1359

dual chamber pacing for, 1363

endocarditis prophylaxis, 13631364

hypertrophied heart in, 1363f

and latent obstruction, 1355f

with midleft ventricle (LV)
obstruction, 1357f

mid-left ventricular, 13561358

mitral apparatus and regurgitation,
13541356

obstructive, 1355f, 1360f

overview, 1348

patterns of hypertrophy in, 1349f

risk of SCD and, 1350

stress echocardiography in, 1318

sub-basal, 1349f

systolic anterior motion of mitral
valve, 13501354, 1350f
systolic dysfunction and, 1356
1358

treatment of, 13611362

pharmacologic therapy, 1361

resect-plicate-release operation,
1362

surgical myectomy, 13611362, 1362f
Hypertrophic cardiomyopathy (HCM)
mutations, TD imaging in, 351
Hypertrophic obstructive

cardiomyopathy (HOCM), 1260

Hypertrophied heart, in HCM, 1363f

Hypertrophy

definition, 1349

patterns in HCM, 1349f
Hypoplastic left heart syndrome (HLHS),
1540, 1549f, 1550f1551f, 1701,
1784

velocity vector imaging in, 393394
Hypoplastic mitral valve, 1613
Hypovolemia, 1977, 1978f

hyperkinetic, 1978

hypotension, 1977

hypotensive, patient, 1978f

hypo-volemia, 1977

I
ICE. See Intracardiac echocardiography
(ICE)
ICM. See Ischemic cardiomyopathy
(ICM)
IEC 60601-1 document, on safety of
medical electronic devices, 110
Image line density, and frame rate, 59
Image optimization and equipment, 60

depth and, 61

focus and, 61

gain and, 61

sector width and, 61

zoom and, 61
Image rendering, in 3D echocardiography
2D tomographic slices, 7980

surface rendering, 79

volume rendering, 7879, 79f
Imaging artifact, 3D reconstruction
imaging and, 17
IMH. See Intramural hematoma (IMH)
Impella catheter-based assist device, 1227
Impella device, 1250, 1251f
Individual mitral lesions, evaluation of,
16111615

abnormal mitral arcade, 16131614

accessory mitral orifice, 1615

cleft mitral leaflet, 1613

double orifice mitral valve, 1614
1615, 1614f

dysplasia of mitral valve, 1613
Ebsteins anomaly of mitral valve, 1615

hypoplastic mitral valve, 1613

mitral valve prolapse, 1615

parachute mitral valve, 1613

supramitral ring or membrane,
16111613
Indocyanine green, 11

Index

Infectious cardiomyopathy, 14051407

HIV-associated cardiomyopathy,
1407

septic cardiomyopathy, 14051407
Infective endocarditis

aortic abscess

in native valve, 1045f

transesophageal approach, 1053f

aortic insufficiency, massive, 1046f

Candida albicans, 1044f

in childhood, 18561857

diagnosis of, 1059

Duke criteria for, 1042, 1043t

false-positive results in, 1045t

echocardiography in

findings, 10431047

goals of, 10421043

indications of, 10581059, 1059f

metallic aortic valve prosthesis,
infected, 1057f

prognostic stratification, role in,
10501058

sequential transesophageal,
1051f1052f

three-dimensional, 10491050,
1050f1058f

overview, 10421043

periannular extension of, 10441045

pericardial effusion, 10451047

prosthetic valve, 1047

abscess in patient with mechanical,
1047f

dehiscence, 1047

prosthetic pitch, 1047
pseudoaneurysm

in aortic annulus, 1046f

aortic valve, 1046f

right-sided, 10471049

transesophageal approach, 1048f

type of patient with, 10481049

special considerations in, 10471050

tricuspid valve endocarditis, 1058f

valve perforation, 1045, 1046f

bicuspid aortic, 1046f

native mitral, 1046f, 1056f

vegetations, 10431044, 1044f

on bicuspid aortic valve, 1044f

fungus infection, 1054f

large aortic, 1044f, 1053f

on mitral native valve, 1046f, 1050f

transesophageal approach, 1053f
Infective endocarditis prophylaxis, 1859
Inferior vena cava (IVC), 1063, 11431144,
1532f, 1964

abnormalities of, 16821683

bilateral, 1683

inferior vena cava interruption,
16821683

inferior vena cava to left atrium, 1683

cannula position in, 1252f

dilated, 1066f

in dilated cardiomyopathy, 1373
1374, 1374f

hepatic vein, 1964

heterotaxy syndrome and, 17051706

inspiratory collapse, 1964

plethora (dilation) of, 1441f

pulse Doppler across, 1539f

for RAP, 12641266, 1266f

size, 1964

subcostal window, 1964

thrombi, 1966

transgastric level, 1964

ventricular assist devices and, 1233
Inferior vena cava interruption, 1682
1683
Ingenious balloon, 535
Inkjet technology, 6
Inlet VSD, 1659
Instrumentation, Doppler, 6668
Interagency Registry for Mechanically
Assisted Circulatory Support
(INTERMACS), 1224, 1226
Interatrial communication, TGA and,
1657
Interatrial membrane, color Doppler
across, 1532f
INTERMACS. See Interagency Registry

for Mechanically Assisted
Circulatory Support
(INTERMACS)
Internal carotid artery (ICA), 665668
International Electrotechnical
Commission (IEC), 110
International Registry of Acute Aortic
Dissection study, 980
International Society of Cardiovascular
Ultrasound (ISCU), 753, 758
Interrupted aortic arch, 16941695, 1695f
classification of, 1695t

type B arch interruption, 1694
Intersocietal Commission for the

Accreditation of Echocardiography
Laboratories (ICAEL), 132
Interventricular septum (IVS), 1534f
Intra-aortic balloon pump, and carotid
ultrasound findings, 693
Intracardiac echocardiography (ICE),
643653

advantages of, 652

clinical applications, 644

during electrophysiology intervention
atrial septal puncture, 644645,
646f

EP procedures and, 647648

pulmonary vein isolation for atrial
fibrillation ablation, 645, 647,
647f

right ventricular outflow tract
tachycardia, 647, 648f

ventricular tachycardia from left
ventricle, 647, 649f

VT foci from great vessels, 647,
648f649f

equipment and catheters, 643

mechanical system, 643

phased array ultrasound system, 644,
644t

extracardiac use of, 651, 652f

imaging specifications, 644, 645f

limitations of, 652

during structural intervention

Melody valves, 651

patent foramen ovale/atrial septal
defect closure, 648, 650, 650f

periprosthetic valve, 650651, 651f

ventricular septal defect closure, 650,
651f

three-dimensional, 652, 653f
Intracardiac hardware, 15141517
Intramural hematoma (IMH), 957958,
958f

transesophageal imaging of, 978
Intrauterine growth retardation (IUGR),
1528
Intravascular ultrasound (IVUS), 643,
655661

examination procedure, 656657

future perspectives, 661

image acquisition, 655656

electronically switched multielement
array system, 656

mechanically rotating transducer,
655656

image interpretation, 657659, 658f,
659f

safety of, 661

technology, 655

utility of, 659660, 660f
Ischemic and nonischemic
cardiomyopathy

cardiac magnetic resonance imaging,
14271428

cronary computed tomographic
angiogram, 1427

I-XIX

doppler echocardiography, 1421
1424

coronary echocardiography, 1424

left atrial size, 1422

left ventricular diastolic dysfunction,
1422

left ventricular end-systolic volume
index, 14211422

left ventricular volumes, 1421

mitral regurgitation, mechanism,
14221424, 1423f

myocardial contrast
echocardiography, 1424

right ventricle dysfunction, 1422

stress echocardiography, 1424

tenting area, 1422, 1423f

echocardiographic assessment, 1419

echocardiographic differentiation of,
14181434

large mitralseptal separation, 1419

M-mode echocardiography, 1419
1421

overview, 1418

positron emission tomography, 1428

severely dilated left ventricular cavity,
1419

single-photon emission computed
tomography, 14251427
Ischemic cardiomyopathy (ICM), 1388
1395, 1395f, 1418, 1420ff1421f

vs. NICM, 1425, 1426t
Ischemic cascade, 1307f
Ischemic handgrip, 453
Ischemic heart disease, 12891305

central mitral regurgitation and,
1298f

chronic. See Chronic ischemic heart
disease detection of, 12891292

pharmacological stress testing, 1290

stress echocardiography for, 1289
1291, 1290f

vasodilator stress testing, 1290

echocardiography in

myocardial contrast stress, 12911292

speckle-tracking, 13011303, 1303f

three-dimensional, 13011302

overview, 1289
Ischemic LV dysfunction, 3D speckle

tracking and, 376t
Ischemic mitral regurgitation, 858863
Isolated carditis, 774775
Isolated mitral valve cleft, 1775
Isomerism, echocardiography of, 1577
Isomerism syndromes. See also
Heterotaxy syndrome

I-XX

Comprehensive Textbook of Echocardiography

cardiac and extracardiac anomalies
in, 1705f

venoatrial connections in, 1706t
Isovolumic contraction (IVC), 11851186
Isovolumic contraction time (IVCT), 1140
Isovolumic relaxation time (IVRT), 313,
1069t, 1140
IUGR. See Intrauterine growth
retardation (IUGR)
IVC. See Inferior vena cava (IVC)
IVCCI. See IVC collapsibility index
(IVCCI)
IVC collapsibility index (IVCCI), 1265
IVCT. See Isovolumic contraction time
(IVCT)
IVRT. See Isovolumic relaxation time
(IVRT)
IVUS. See Intravascular ultrasound
(IVUS)
IVUS with virtual histology (IVUS-VH),
657

J
Jarvik-2000 Flowmaker, 1228, 1228f
Joyner, Claude, 6
Junctional rhythm, and PV Doppler, 333,
334f

K
Kaul, Sanjiv, 13
Kawasaki disease (KD), 16881689
in childhood, 1861

coronary arteries with, 1862t

velocity vector imaging in, 398

L
LAD. See Left anterior descending (LAD)
LaGrangian strain, 389
Lambls excrescences, 1015, 1480f
LAP. See Left atrial pressure (LAP)
Large intracardiac thrombus, 1978

atrial appendage, 1978

atrial fibrillation, 1978

atrial flutter, 1978

autopsy, 1979

deep venous thrombosis, 1979

disystole, 1978

echolucent, 1979

endocardium, 1978

heart embolism, 1978

intracardiac devices, 1979

systole, 1978

transthoracic echo, 1979

transthoracic images, 1979

ventricular apex, 1979
Lariat procedure, 563, 566, 566f568f
Laser Doppler flowmetry (LDF), 469470
Laser Doppler perfusion monitoring
(LDPM). See Laser Doppler
flowmetry (LDF)
Late gadolinium enhancement (LGE),
2003, 2020
Latent diastolic dysfunction, stress
echocardiography in, 1318
Lateral resolution, 59, 59f, 733, 734f
Lavengin, Paul, 4
LBBB. See Left bundle branch block
(LBBB)
Lead infection, associated with
pacemaker, 11141115
Lead perforation, 12151217

chest CT for, 1216

echocardiography in, 1216f

reported cases of, 1217t
Lead zirconate titrate (PZT), 1992
Leakage current., 110111, 111f, 111t
Left anterior descending (LAD), 1337

artery disease, 1328

distal post-stenotic diastolic-to systolic velocity ratio, 13401341
Left atrial appendage (LAA), 1960

abnormalities, 1960

adequate visualization, 1960

clot detection 3D TEE for, 512, 619

continuous wave, 1963f

3DE assessment of, 277, 277f

doppler interrogation, 1960

embolism, 1960

endocardial device closure of,
561563, 562f565f

epicardial suturing of. See Lariat
procedure

exclusion, 1961

percutaneous, 561

surgical, 560561

mid-diastolic signals, 1960

mitral valve, surgery, 1960

orthogonal mass abnormality, 1960

pectinate lines, 1960

persistence, 1961

prominent ridges, 1960

recanalized, 1963f

thrombus, 1960

traverse, 1962f
Left atrial appendage clot, embolization
of, 1917f
Left atrial appendage thrombus, 2058f

Index

Left atrial function, 12551263

anatomy of, 1255

cardiac cycle and, 1256f

functional assessment, 12571259
LA volume, 1257, 1258f

speckle tracking, 1259, 1259f

spectral Doppler, 12571259

left atrial size, 1257, 1258f

overview, 1255

overview of, 1255

pathophysiology, 12591261, 1260f

atrial fibrillation, 12591260

cardiac amyloidosis, 1261f

cardiomyopathies, 1260, 1260f

hypertension, 1259

physiology of, 1256

phasic left atrial function, 1256

physiological effects on, 1257

pressure-volume relationship of,
1256f
Left atrial hemangioma, 1487f
Left atrial leiomyosarcoma, 1488f
Left atrial myxomas, 845, 1467f, 1472,
1474f, 1475f
Left atrial pressure (LAP), 1279, 1281f
Left atrial septal myxoma, 1469f
Left atrium, 1960

ablation therapies, 1960

anteroposterior dimension, 1960

arrhythmias patients, 1960

atrial arrhythmias patients, 1960

atrial flutter, 1960

doppler, 1961

color flow, 1961

pulsed, 1961

echocardiographer, 1960

echodense mass, 1962f

index, 1960

latrial tachycardia, 1960

left atrial appendage, 1960

abnormalities, 1960

adequate visualization, 1960

continuous wave, 1963f

doppler interrogation, 1960

embolism, 1960

exclusion, 1961

mid-diastolic signals, 1960

mitral valve, surgery, 1960

orthogonal mass abnormality, 1960

pectinate lines, 1960

persistence, 1961

prominent ridges, 1960

recanalized, 1963f

thrombus, 1960

traverse, 1962f

left upper pulmonary vein, 1962f

M-mode echocardiography, 1960

pectinate muscles, 1962f

pulmonary veins, 1960, 1961f

rhythm analysis clues, 1960

spontaneous echo contrast, 1960

swirling appearance, 1960

thromboembolic, 1960

complications, 1960

event, 1960

nonvalvular, 1960

risk, 1960

valvular, 1960

transesophageal echocardiogram,
1961f

volume, 1960

Simpsons biplane method, 1960
Left atrium myxoma, 2053f
Left bundle branch block (LBBB), 1217,
1377
Left heart hemodynamics, age groups for,
1275t
Left isomerism, 1577
Left lower pulmonary vein (LLPV), 327,
328f
Left main coronary artery (LMCA)

flow in distal, detection of, 1340

transesophageal echocardiography,
13381340, 1339f

transthoracic echocardiography,
13371338, 1339f
Left main coronary artery ostium, 647
Left main coronary artery stenosis
(LMCAS), 1325
Left parasternal approach, of three dimensional echocardiography,
244, 245f254f
Left-sided filling pressures, 12731279

color M-mode flow propagation
velocity, 1278

echocardiographic methods for,
1273t1274t

evaluation of, 1277f

variables used for, 1278t

left atrial dimensions, 12781279

mitral inflow parameters in, 1274
1276, 1275f, 1275t

pulmonary venous flow and, 1276,
1276f

tissue Doppler annular diastolic
velocities, 12771278
Left-sided superior vena cava (LSVC),
1535, 1545f
Left upper pulmonary vein (LUPV), 327,
328f, 1962f

Left ventricle, 11851194, 12461248

apical velocity rotation and timing
for, 1202f

isovolumic contraction, 11851186

lengthening, 1189

postejection isovolumic phase,
11881193, 1192f

rapid filling, 11931194, 1193f

recoiling, 1189

torsion, 11861188

ventricular assist devices and,
12291230
Left ventricle, 3D quantitation of,
11491165

2D echocardiography, limitations of,
11491152

apical foreshortening, 1152

boundary recognition, 1152

3D spatial coordinates, lack of,
11491151

geometric assumptions, 1151

3D reconstruction, 1152f

limitations of, 1152

freehand scanning, 1150f

real time 3D ecocardiography,
11521156, 1152f

data acquisition, 11531155, 1154f,
1155f

first-generation matrix array
transducer, 1153

left ventricular strain, 11571161,
1159f, 1160f

left volumes and EF, validation of,
11561157

peak filling rate from, 1158f

for postmyocardial infarct risk
stratification, 1165

third-generation systems in, 1155
1156

regional segments of, 1162f, 1163f

serial evaluation of patients with
3DE, 11641165
Left ventricle apical ballooning
syndrome, 1388
Left ventricle twist, 276
Left ventricle underfilling, 1246f

causes, 1247t

evidence of, 12461248
Left ventricular aneurysm, 1295f, 1298
Left ventricular apical thrombus, 1299f
Left ventricular assist devices (LVAD),
1204

and carotid ultrasound findings,
693694

complications of, 12401246

I-XXI

computed tomography and, 2063
2064

left ventricular overfilling, 12401246

causes, 1245t

mitral flow prior to placement of,
1243f

net forward cardiac output and,
12471248

optimizing settings, 12481249, 1250f

placement response of, factors affect,
1235f

power failure of, 1244f

structures in patient with, 1239f

transesophageal echo, postoperative,
1237f
Left ventricular diastolic dysfunction

grades of, 1127f

ICM/NICM and, 1422

left atrial dilation in patient with,
1125f
Left ventricular diastolic function

2D echocardiography, 11241125

flow propagation velocity, 1127

color Doppler, 1129f

future directions for, 11311132

imaging techniques for, 11311132

integrating echocardiographic
variables, 11301131, 1130f1131f

left atrial size, 11241125

left ventricular mass, 11241125

in nonsinus rhythm, 11291130

overview, 1124

pulmonary artery pressure, 1129

pulmonary artery systolic pressure
and, 1130f

pulmonary venous flow, 11271128

tissue Doppler imaging for, 1127,
1128f

Valsalva maneuver, 11261127

wall function, 11241125
Left ventricular dimensions, 19421943
Left ventricular dysfunction, ultrasound
stethoscope for screening of, 295
Left ventricular dyssynchrony, 1218

three-dimensional speckle tracking
and, 376t
Left ventricular ejection fraction (LVEF),
381, 1116, 1117, 11241125

in LFLG-AS, 1929

pacemaker and, 1218f

systolic myocardial dysfunction and,
1131f

Left ventricular end-diastolic
diameter (LVEDD), 1886

I-XXII

Comprehensive Textbook of Echocardiography

Left ventricular end diastolic pressure

(LVEDP), 1277, 1279, 1280f, 1375,
1375f
Left ventricular end-diastolic pressure
(LVEDP)

assessment of, 334335, 334f, 335f
Left ventricular end-diastolic volume
(LVEDV), 271
Left ventricular end-systolic diameter
(LVESD), 1886
Left ventricular end-systolic volume
(LVESV), 271
Left ventricular filling pressure

demonstration of elevated, 1126
1130

restrictive transmitral filling pattern,
1126f

transmitral diastolic inflow for, 1126f
Left ventricular free wall, rupture of, 1294
Left ventricular geometry, partitions
value of, 1887f
Left ventricular hypertrophy (LVH), 294,
351, 352, 1125f, 1871, 19331934,
1974

in female, 1911f
Left ventricular internal dimension in
diastole (LVIDd), 1118
Left ventricular internal dimension in
systole (LVIDs), 1118
Left ventricular intracardiac metastasis,
1499f
Left ventricular lipoma, 1482f
Left ventricular mass, 11241125,
19421943

BlandAltman analysis, 1162

3D echocardiography, 11611162

epicardial and endocardial borders,
1161f

partitions value of, 1887t

RT 3DE for, 1162
Left ventricular myxomas, 1474
Left ventricular noncompaction (LVNC),
395396, 13841388, 1385f,
1389f1391f, 1394f

cardiac MRI for, 20002002, 2001f,
2002f

echocardiographic features of,
13851388, 1386f1387f

isolated, 1387f, 1393f

normal fetal ontogenesis, 1385
Left ventricular opacification (LVO), 421,
426
Left ventricular outflow tract (LVOT)

forms of, 897t

in prosthetic valves, 1084

Left ventricular outflow tract (LVOT)

obstruction, 13501354

anomalous papillary muscle and,
1354f

aortic regurgitation, 16281630

aortocameral communications, 1632

conversely, dynamic, 13531354

echocardiographic evaluation of,
13511352, 1352f

sinus of Valsalva aneurysm, 1630
1632

subvalvular aortic stenosis and,
16241626

supravalvular aortic stenosis and,
16261628

systolic anterior motion and, 1350

TGA and, 16591661

valvular aortic stenosis and, 1618
1624

vs. mitral regurgitation, 1353f
Left ventricular overfilling, 12401246
Left ventricular pseudoaneurysm, 569,
12941295

closure of, 571, 571f
Left ventricular quantification, 3D
echocardiography and, 8285, 84f
Left ventricular-RA communication,
17791782
Left ventricular remodeling, 3D
echocardiography, 11621164
Left ventricular systolic function,
11151123

arterialventricular coupling,
11211122

assessment of, 11161119

biplane method of discs, 11181119

blood supply from particular
coronary artery, 1116

contrast echocardiography of, 1121,
1121f

Doppler echocardiography of, 1119

2D speckle tracking
echocardiography, 1120

fractional shortening at
endocardium, 1118

longitudinal fiber shortening, 1119

M-mode echocardiography, 1116
1119

E point septal separation, 1117

left ventricular ejection fraction,
1116, 1117

parasternal long-axis, 1116

modified cylinder-ellipse formula,
1118f

myocardial contractile velocity and,
1119

Index

myocardial performance index, 1120

overview, 1115

Quinones method, 1117

seventeen-segment model, 1116f

sphericity index, calculation of, 1119

Tei's index, 1120, 1120f

tissue Doppler imaging, 1119

transthoracic echocardiography

three-dimensional, 1122

two-dimensional, 11161119

velocity vector imaging, 1120

visual estimation of, 11151116
Left ventricular thrombus, 15041506
Left ventricular volumes

assessment of, 81

in ICM/NICM, 1421
Leiomyosarcoma, 627

aortic, 1489f1490f

left atrial, 1488f
Levacor ventricular assist device, 1228
Levo-transposition of great arterie, 1759f
Levo transposition of great vessels, in
female, 1916f
LFLG-AS. See Low-flow, low-gradient
aortic stenosis (LFLG-AS)
LibmanSacks endocarditis, 18671868,
1868f
Libman-Sacks vegetations, 931
Lidocaine, 508
Light and sound, 1995, 1996

acousto-optical imaging, 1996

complementary approach, 1996

endogenous absorbers, 1995

laser pulses, 1995, 1996

precision, 1995

photoacoustic imaging, 1995

Schlieren tank, 1996

classic implementation, 1996
Linear array system, 8
Linear/phase array transducers, 76, 76f
Linear probe, 58, 58f
Line density, 60
Lipoma, 2054f
Lipomatous hypertrophy

of atrial septum, 1484f

of interatrial septum, 182, 182f, 533,
536f, 2054, 2056f

Live 3DE, 269
Liver cirrhosis, and HV Doppler, 318, 319f
Liver disease, and HV Doppler, 318319,
319f
LMCA. See Left main coronary artery
(LMCA)
LMCAS. See Left main coronary artery
stenosis (LMCAS)

Lobster claw abnormality, 1352f, 1358,

1358f
Loeffler endocarditis, 1403f1404f
Loefflers syndrome, 10061007, 2009f

2d transthoracic echocardiography,
1008f, 1009f
Loeys-Dietz syndrome, 958
LOLIPOP. See London Life Sciences
Prospective Population
(LOLIPOP) study
London Life Sciences Prospective
Population (LOLIPOP) study, 272,
1157
Longitudinal fiber shortening, 1119
Longitudinal peak systolic strain (LPSS),
1069t
Longitudinal strain, 362, 363f, 389
Long-term axial flow devices, 12271228

Berlin heart incor assist device, 1228

HeartAssist system, 1228

HeartMate II continuous flow LV
assist device, 1228

Jarvik-2000 Flowmaker, 1228, 1228f
Long-term third generation centrifugal
flow systems, 12281229

DuraHeart magnetically levitated
centrifugal assist system, 1228

EvaHeart left ventricular system,
12281129, 12281229

HeartWare ventricular system, 1224f,
1228

Levacor ventricular assist device,
1228
Lower bound velocity (LBV), 88
Low-flow, low-gradient aortic stenosis
(LFLG-AS)

with ejection fraction, 1929

with low ejection fraction, 920921

SAVR in, 927

with normal ejection fraction,
921924
Low flow-mediated vasoconstriction
(LFMC), 470471, 470f474f
Low MI imaging, 420
Low transvalvular gradients, aortic
stenosis with, 913
LPSS. See Longitudinal peak systolic
strain (LPSS)
LSVC. See Left-sided superior vena cava
(LSVC)
Lung artifact, 227
Lung carcinoma, 1459f
Lung ultrasound scan (LUS), 1982, 1983

aerated lung, 1982

cardiogenic watery B-lines, 1982,
1983

differential diagnosis

challenge, 1982

extravascular lung water, 1982

human acute respiratory distress
syndrome, 1983

limitations, 1987

B-line interpretation, 1987

obese patients, 1987

patient-dependent, 1987

lung sliding, 1982

main approaches, 1983

methodology, 1983, 1984

pulmonary parenchyma, 1983

thoracic scanning areas, B-lines
semiquantitative assessment,
1983, 1984f

two-dimensional scanner, 1983

physical basis, 1982, 1983

physical scatterer, 1982

physiological basis, 1982, 1983

pulmonary edema, 1983

sonographic appearance, 1982, 1983f

consolidated lung, 1983f

multiple B-lines, 1982, 1983f

normal lung, 1983f
LUPV. See Left upper pulmonary vein
(LUPV)
LVAD. See Left ventricular assist device
(LVAD)
LV concentric hypertrophy, 920
LV contractility, reduction in, 920
LV diastolic function, assessment of, by
TD imaging, 352353, 355f, 356f
LV dyssynchrony, TD Imaging for, 353,
354f
LVEDD. See Left ventricular end-diastolic
diameter (LVEDD)
LVEDP. See Left ventricular end diastolic
pressure (LVEDP)
LVEDV index, 1157
LVEF. See Left ventricular ejection
fraction (LVEF)
LV ejection fraction (LVEF), 270

in acute coronary syndromes,
12931294
LVESD. See Left ventricular end-systolic
diameter (LVESD)
LVESV index, 1157
LV filling pressures, estimation of, by TD
imaging, 352353
LVIDd. See Left ventricular internal
dimension in diastole (LVIDd)

I-XXIII

LVIDs. See Left ventricular internal

dimension in systole (LVIDs)
LV noncompaction, three-dimensional
speckle tracking and, 376t
LVOT. See Left ventricular outflow tract
(LVOT)
LVOT geometry, aging and, 1943
LVOT velocity time integral, in LFLG-AS,
1929
LV regional function, 3DE assessment of,
271272, 276

aortic stenosis, 279

aortic valve assessment, 278f, 279,
279f

contractile reserve, 276

left and right atria, 276277, 277f

left ventricle twist, 276

right ventricle, 277278

valvular assessment, 278, 278f
LV systolic dysfunction, 3D speckle
tracking and, 376t
LV volume assessment, 3D speckle
tracking and, 376t
LV wall motion abnormalities

three-dimensional speckle tracking
and, 376t
Lymphomas, 1500f, 2054

primary cardiac, 1490

M
MAIN-COMPARE trial, 659
Major adverse cardiovascular events
(MACE), 2042
Malignant melanoma, 1500
Malignant primary cardiac tumors,
14841511

angiosarcoma, 14851486

cardiac plasmacytoma, 14901491,
1492f

fibrosarcomas, 1488

hydatid cyst, 1491, 1493f1498f

pericardial mesotheliomas, 1491

primary cardiac lymphoma, 1490

rhabdomyosarcoma, 14861488

sarcomas, 14841488
Manometers, 27
Marfan syndrome (MFS), 958959

TAA and, 1936
Matrix biplane transesophageal echo
(TEE) probe, 99100, 100f
MDCT. See Multidetector computed
tomography (MDCT)

I-XXIV

Comprehensive Textbook of Echocardiography

Mean left atrial pressure, assessment of,

335, 335f
Mean pulmonary artery pressure (MPAP),
1063, 1064t, 1065t, 1066, 1272, 1272f, 1901
Mechanical index (MI), 57, 111, 418419

high, 422423

low, 423
Mechanical safety, TEE probe
examination and, 111112
Mechanical valves, 1082

bileaflet, 1083t, 1085f

single disc, 1083t
Melody valves, 651
Membranous subaortic stenosis, 1625
Mesothelial/macrophage incidental
cardiac excrescences, 15111518

cardiac calcified amorphous tumor,
15121514

extracardiac masses, 1514, 1514f

intracardiac hardware, 15141517

mitral annular calcification, 1512,
1512f
Metabolic cardiomyopathy, 1407
Metastasis, 20532054

from melanoma, 2055f
Metastatic left pleural effusion, in female,
1913f
Metastatic melanoma, 1499f

involving right ventricle, 1499f
Micro-beam forming, 78
Microbubbles, 1993, 1994

first human studies, 1993

future potential, 1993

liver metastasis, 1993, 1994f

macro/micro- vascular systems, 1993

mechanical index, 1993

microbubble lymphangiography

sentinel lymph node, 1993, 1994f

preclinical work, 1993

ultrasound contrast, 1993

VEG-F2 study, 1993
Mid-left ventricular hypertrophic
cardiomyopathy, 13561358
Midmuscular ventricular septal defect,
1544f
Midwall fractional shortening (MWFS),
1118
Miniaturization technology, 102103,
103f, 104f
MinivisorTM, 291, 292f
Mirror image artifacts, 735736. See also
Artifacts
MitraClip device, 539
Mitral annular calcification (MAC), 1512,
1512f, 1895f, 19461948

in elderly, 19461948

Mitral atresia, 1552f, 17011704

associated systemic venous
anomalies, 17021703

apical four-chamber view, 1703

parasternal views, 1703

subcostal view, 17021703

suprasternal notch view, 1703

echocardiography after stage 1
palliation, 17031704
Mitral prosthetic regurgitation, 1106f,
1107f1108f

paravalvular, 1102f1104f
Mitral regurgitation (MR), 791801,
880895, 1972, 1974f

acute, 1972

acute vs. chronic, 885

cardiac MRI for, 20122013

chronic asymptomatic, sequential
evaluation of, 890892

BNP levels, 891

Doppler echocardiography, 891892

follow-up in, 890891

chronic functional, 884f

classifications, 1973t

color Doppler, 1973t, 1974f, 1975f,
1975t

Doppler vena contracta, 1973t, 1975t

regurgitant fraction, 1973t, 1975t

regurgitant orifice area, 1973t

regurgitant volume, 1973t, 1975t

color flow imaging, 885

continuous wave Doppler, 886

continuous wave Doppler signal of,
1279

3DE assessment of, 282283, 282f,
283f

echocardiographic criteria for, 889t

endocarditis, 1972

etiology of, 880884

exercise echocardiography, role of,
892893

hemodynamically stable, 1972

hemodynamic consequences of,
889890

left atrium, 890

left ventricle, 889890

pulmonary arterial pressure, 890

right ventricle, 890

ICM/NICM and, 14221424, 1423f

mechanism of, 884885

functional classification, 884

jet direction, 884885

mitral valve repair, feasibility of, 892

overview, 880

papillary muscle, 1972

Index

pap muscle dysfunction, 1972

primary, 880883

leaflets, 880883

mitral annulus, 883

subvalvular apparatus, 883

pulmonary venous flow, 886

and PV Doppler, 336338, 337f, 338f

rupture, 1972

secondary, 883884

left ventricular remodeling, 883884

mitral annulus, 883

mitral valve distortion, 883884

severity of, 885889

grading of, 888

qualitative assessment of, 885886

quantitative assessment of, 886888,
888f

semiquantitative assessment of, 886

surgical correction of, 538540, 541f,
542f

transesophageal echocardiography,
1972

vena contracta, 886
Mitral regurgitation, echocardiographic
assessment of, 847863

causes of, 848t

echocardiography, role of, 848t

etiology and mechanism of, 848849

hemodynamic consequences,
874876

left atrial size and pulmonary
pressures, 876

left ventricle size and function,
874876

key notes, 847849

mitral valve prolapse, 849

severity of, 863876

antegrade velocity of mitral inflow,
869

color Doppler jet area, 863868

continuous-wave jet intensity, 869

echocardiographic assessment of,
864t

PISA method, 870871

pulmonary venous flow, 869

quantitative echo/Doppler methods,
869874

semiquantitative echo/Doppler
methods, 863869

vena contracta width, 868869

surgical considerations in, 876
Mitral stenosis, 776791

anterior mitral valve leaflet, 777, 780f

associated lesions, 783784

cardiac MRI for, 2013

continuity equation, 789

3DE assessment of, 280, 281f, 282
echocardiography

exercise, 790

pitfalls of using, 790791

recommendations for, 778t

three-dimensional, 791

two-dimensional, 778779, 779f

mitral valve, assessment of, 779783

abnormal motion of leaflets, 779781

commissural fusion, 781783

PISA method, 789

planimetry, 785

pressure gradient, 785787

pressure half-time, 787789

and PV Doppler, 338339, 339f
severity

classification of, 784t

determination of, 784785

indices of, 789790

methods for quantification of, 785t
Mitral stenosis, echocardiographic
assessment, 826847, 827f

anatomic considerations, 826827

atrial septal defects, 842

balloon mitral valvotomy, 839t, 842f

complications of, 840841

echocardiography in patients for,
838840

evaluation of patient, 838840, 839t

mitral regurgitation in, 840841

post, 841f, 843f, 844f

cardiac perforation, 841842

causes of, 846847

conditions clinically mimicking
rheumatic, 844846

atrial ball valve thrombus, 845

congenital double orifice mitral
valve, 844845

congenital mitral stenosis, 844

congenital parachute mitral valve,
845, 846f

degenerative mitral valve stenosis,
844

left atrial myxoma, 845

diseases of other valves, 834836
echocardiography

in balloon mitral valvotomy, 838840,
839t

exercise, 844

role of three-dimensional, 844

two-dimensional, parameters of, 827

indices of severity, 829834

continuity equation, 833

diastolic pressure gradient, 829831

mitral valve area planimetry, 831

PISA method, 834

pressure half-time, 831833, 832t,
833f

Wilkins Score, 830t

left atrium, 834

left atrium appendage, 834

long-term outcome, 842843

mitral regurgitation and, 834836

mitral valve morphology, 827829

leaflet motion, 828

subvalvular pathology, 828

valve calcification, 828829

valve thickening, 827828

normal mitral valve area, 827

pulmonary hypertension, 836838

mitral valve echocardiographic
scoring system, 836

Wilkins scoring system, 836837

rheumatic, 827

RT 3DE scoring system, 838t
Mitral valve (MV), 577579, 578f, 579f

in adults, 18181820

cleft, 18181819

cor triatriatum, 1818

double orifice mitral valve, 18191820

echocardiography, 1818

mitral regurgitation, 1820

parachute, 1820

anatomy, assessment of

Cormier score, 782t

Wilkins score, 782t

anatomy of, Cormier Score, 837t

3DE assessment of, 278, 278f, 280,
281f

3D echo of, 516520, 517f, 518f

pulsed wave Doppler across, 1533f

real time 3D echocardiographic score
of, 783t

short-axis, 1533f
Mitral valve, congenital anomalies of,
16101615

individual mitral lesions, evaluation
of, 16111615

abnormal mitral arcade, 16131614

accessory mitral orifice, 1615

cleft mitral leaflet, 1613

double orifice mitral valve, 1614
1615, 1614f

dysplasia of mitral valve, 1613

Ebsteins anomaly of mitral valve,
1615

hypoplastic mitral valve, 1613

mitral valve prolapse, 1615

parachute mitral valve, 1613

I-XXV

supramitral ring or membrane,
16111613

mitral valve lesions,
echocardiographic views,
16101611

types of, 1611t
Mitral valve area (MVA), 282
Mitral valve calcification, 1889

in women, 1889
Mitral valve diseases, 775776, 826879,
1318

anatomy of mitral valve, 775776

mitral regurgitation, 791801

assessment of, 793

color Doppler flow, 793796

continuous wave Doppler, 796797

exercise echocardiography, 800

follow-up in, 801

pulmonary vein flow, 798

pulsed Doppler, 797798

severity of, 793

integrative approach for, 800801

organic, 801t

role of TEE in assessing, 788800,
799t

supportive signs, 798
three-dimensional
echocardiography, 800

two-dimensional echocardiography,
792793

mitral stenosis, 776791

overview of, 826

role of 3D TEE in operating room in,
577582, 578f593f

stress echocardiography in, 1318
Mitral valve insufficiency, 1860f
Mitral valve lesions, echocardiographic
views, 16101611

Mitral valve morphology, 827829

leaflet motion, 828

subvalvular pathology, 828

valve calcification, 828829

valve thickening, 827828
Mitral valve opening, and untwisting,
12001201
Mitral valve orifice area (MVOA), 777,
790791

continuity equation, 789

mitral PHT and, 787789
Mitral valve performance, 1249
Mitral valve prolapse, 169

3D TTE and, 282, 282f

ultrasound stethoscope for screening
of, 295
Mitral valve prolapse (MVP), 849863,
1615

I-XXVI

Comprehensive Textbook of Echocardiography

echocardiographic assessment of,
850855
M-mode, 850

three-dimensional, 851855

two-dimensional transesophageal,
851, 855f, 856f

two-dimensional transthoracic, 851,
852f855f

surgical methods and indicators in,
855863

degenerative mitral regurgitation, 863

functional mitral regurgitation,
857858

infective endocarditis, 863

ischemic mitral regurgitation,
858863

rheumatic mitral regurgitation, 863

tricuspid and pulmonary valve
prolapse with, 855f

in women, 1888, 1888f1889f
Mitral valve quantification

three-dimensional echocardiography
and, 8182, 82f, 83f
Mitral valve segmentation analysis, 881f
Mitral valve stenosis, in female, 1888
1889
Mitral valve vegetation, in female, 1911f
Mitral valvular verrucous nodules, 773f
M-mode echocardiography, 3, 3f, 58, 58f,
119130

development of, 47

in left ventricular systolic function,
11161119

E point septal separation, 1117

left ventricular ejection fraction,
1116, 1117

parasternal long-axis, 1116

in right ventricle, 11361138

transthoracic echocardiogram, 136,
136f

of tricuspid valve, 984986

Ebsteins anomaly, 986f

functional events, demonstrating,
984f

septal leaflet, identification, 985f

systolic abnormalities, 986f

using contrast injections,
identification, 985f
Moderator band, 1503
MPAP. See Mean pulmonary artery
pressure (MPAP)

mPAP. See mean pulmonary artery
pressure (mPAP)
MSCT. See Multislice computed
tomography (MSCT)

99mTc-sestamibi gated single photon computed emission
tomography (GSPECT), 1157,
1159f
Mucopolysaccharidosis, 931
MullinsTM catheter, 533
Multi-beat acquisition, in 3D
echocardiography, 7475, 75f
Multicenter Aneurysm Screen Study
(MASS), 294
Multidetector computed tomography
(MDCT), 2023

acute chest pain evaluation with,
20452047

for coronary artery anomalies,
20472049

techniques of, 2047
Multigate pulsed Doppler, 70
Multipath reflection, 81
Multiplane mode, 268269
Multiple-beat 3DE imaging, 269
Multiple thrombi, with poor ventricular
function, 1377f
Multislice computed tomography
(MSCT), 1427
Muscular inlet defect, 1593
Muscular outlet defect, 1593
Muscular trabecular defect, 1594
Muscular ventricular septal defects,
557558, 15931594, 1593f, 1594f.
See also Ventricular septal defects
(VSDs), closure of

muscular inlet defect, 1593

muscular outlet defect, 1593

muscular trabecular defect, 1594
MV clipping, 538540, 541f, 542f

3D TEE guidance of, 540

MitraClip device for, 539

patient selection for, 540
MVOA. See Mitral valve orifice area
(MVOA)
MVP syndrome (MVPS), 1888
MWFS. See Midwall fractional shortening
(MWFS)
Myocardial blood flow (MBF), 442
Myocardial blood volume (MBV), 422
Myocardial contractile motion, 1159
1160
Myocardial contractile velocity, 1119
Myocardial contrast echocardiography
(MCE), 416, 422, 430. See also
Contrast echocardiography

for CAD detection, 430431

coronary flow reserve by, 431

for myocardial perfusion assessment,
427428, 429f

Index

for myocardial viability detection, 431
Myocardial contrast stress
echocardiography, 12911292
Myocardial deformation imaging, 360.
See also Strain imaging
Myocardial disease, detection of, by TD
imaging, 350352
Myocardial fiber organization, 1180f
Myocardial infarction

mechanical complications of,
12941298

left ventricular aneurysm, 12981300

left ventricular free wall, rupture of,
1294

left ventricular pseudoaneurysm,
12941295

papillary muscle rupture, 1297, 1297f

ventricular septal rupture, 1295
1297, 1296f

speckle-tracking echocardiography,
1302

vs. cardiac death, 13111315
Myocardial ischemia, speckle-tracking
echocardiography, 1302
Myocardial perforation, pacemaker
associated, 12151217

2D transesophageal
echocardiography in, 1216,
1216f

reported cases of, 1217t

RT3DE in, 1216f
Myocardial performance index (MPI),
1120

ICM/NICM and, 1419

in rheumatoid arthritis, 1868
Myocardial perfusion

echocardiography, 441447

acute coronary syndromes, 443

chronic coronary artery disease,
443445

myocardial perfusion evaluation, by
CE, 441443

nonischemic dilated
cardiomyopathy, assessment
of, 445446, 447f

and stress echocardiography, 1319
Myocardial tagging, 405
Myocardial viability, speckle-tracking
echocardiography, 13021303
Myocardial viability assessment, contrast
echocardiography for, 443
Myocardial wall motion abnormality,
1311
Myocarditis, velocity vector imaging in,
398399

Myofiber structural orientation, 1183f

Myxomas, 622623, 1474f, 2051

atrial, 1468

attached to atrial septum, 1471

biatrial, 1470f

left atrial, 1467f

left atrial septal, 1469f

right atrial, 1470f

right ventricular, 1471f1472f, 1473f

ventricular, 14681470

N
Narrow-angled display, 241
National Board of Echocardiography
(NBE), 754
National Electrical Manufacturers
Association (NEMA), 110
National Institutes of Health, 8
Native valve endocarditis, role of 3D TEE
in operating room in, 597604,
609f612f
Nitric oxide (NO), 454

and endothelial function, 450, 451
Nodal re-entrant tachycardia (NRT), 1957
Noninvasive hemodynamic monitoring,
229230, 230f, 231f
Nonischemic dilated cardiomyopathy
(NICM), 1418

mitralseptal separation in, 1419f

vs. ICM, 1425, 1426t
Nonsinus rhythm, 11291130
Nonstandard echocardiographic
examination, 188223

abdominal examination, 220

examination from back, 216, 218, 220

two-dimensional transthoracic
echocardiography, 221f

left atrial appendage examination,
212, 214, 216, 217f219f

live/real-time three-dimensional
transthoracic echocardiography,
217f219f

two-dimensional transesophageal
echocardiogram, 219f

two-dimensional transthoracic
echocardiography, 217f

left parasternal and apical planes for
coronary arteries, 190, 204, 207,
213f216f

live/real-time three-dimensional
transthoracic echocardio
graphy, 214f216f

two-dimensional transthoracic
echocardiography, 213f214f

right and left supraclavicular
examination, 189190,
205f212f

live/real-time three-dimensional
transthoracic echocardio graphy, 209f212f

two-dimensional transthoracic
echocardiography, 205f209f

right parasternal examination planes,
188189, 189f204f

live/real-time, three-dimensional
transthoracic echocardio graphy, 196f204f

two-dimensional transthoracic
echocardiography, 189f196f
Non-ST elevation myocardial infarction
(NSTEMI), evaluation of, 226
No-reflow phenomenon, 443
Normal heart, 11851194
left ventricle, 11851194

isovolumic contraction, 11851186

lengthening, 1189

postejection isovolumic phase,
11881193, 1192f

rapid filling, 11931194, 1193f

recoiling, 1189

torsion, 11861188

sonomicrometer crystal tracings,
1203f

ventricular narrowing, 1187f
Nutritional deficiency, 1880
Nyquist, Harry, 70
Nyquist limit, 69, 70, 736

O
Obesity, and HV Doppler, 318319, 319f
Off-axis imaging, 164
One-dimensional (1D)
echocardiography, 3f, 25, 25f
Optigo, 291, 292f
Optison, 417, 418t. See also Contrast
echocardiography
Ostium primum atrial septal defect, 1586
Outflow tract obstruction, 17661770

congenital aortic stenosis/bicuspid
aortic valve, 17661770

subaortic stenosis, 1770

P
Paced rhythm, and PV Doppler, 334, 334f
PAcT. See Pulmonary flow acceleration
time (PAcT)
PA diastolic pressure (PADP), 230, 231f

I-XXVII

PAH. See Pulmonary arterial

hypertension (PAH)
Papillary fibroelastoma, 624,1023f1024f,
1476f1477f

on tricuspid valve, 2019f
Papillary muscle rupture, 862f, 1297,
1297f
PAPS. See Pulmonary hypertension
(PAPS)
PAPVC. See Partial anomalous pulmonary
venous connection (PAPVC)
Parachute mitral valve, 1613
Paradoxical low-flow, low-gradient aortic
stenosis (PLFLG-AS), 921922

cardiac catheterization and, 923

diagnosis of, 924

invasive vs. noninvasive evaluation of
AS, 922924

LVEF and, 921922

mechanisms for, 924925

cardiac output, 924

impaired myocardial function, 924

pseudostenosis, 925

with preserved ejection fraction,
19291930

SAVR in, 927
Parametric display, 708711, 709f711f
Parasternal long-axis (PLAX) view, 1116,
1117

of aortic root, 1797f
Parasternal window, TTE

linear measurements, 139141,
139f141f

ascending aorta, 140, 141f

Doppler imaging, 141, 141f

parasternal long-axis (PLAX) plane,
137139, 138f, 139f, 139t

M-mode imaging, 138139, 139f

technique, 137138

two-dimensional anatomic imaging,
138, 138f, 139f, 139t

parasternal short-axis plane, 144146,
145f147f, 145t

aortic valve level, 145, 146f

coronary artery imaging, 145

Doppler imaging, 146, 147f

left ventricular apex level, 146, 147f

linear measurements, 146

mitral valve level, 145, 146f

papillary muscle level, 145, 146f

technique, 144145, 145f, 145t

right parasternal long axis view,
141142

Doppler imaging, 142

technique, 141142

I-XXVIII

Comprehensive Textbook of Echocardiography

two-dimensional anatomic imaging,
142

right ventricle inflow view, 142, 142f,
142t, 143f

Doppler imaging, 142, 143f

technique, 142, 142f

two-dimensional anatomic imaging,
142, 143f

right ventricular outflow tract view,
142144, 143f, 143t, 144f

Doppler imaging, 143144, 144f

technique, 142, 143f, 143t

two-dimensional anatomic imaging,
143, 144f
Paravalvular aortic prosthetic
regurgitation, 1105f
Paravalvular mitral prosthetic
regurgitation, 1102f1104f
Paravalvular mitral regurgitation, in
female, 1918f1919f
Paravalvular prosthetic leaks
closure of, 546548, 549f, 550f

3D TEE monitoring of, 548, 550f, 551f

3D TEE and, 512, 513f
Paravalvular regurgitation (PVR), 608612
of prosthetic valves, 1099

vs. transvalvular regurgitation, 1106
Partial anomalous pulmonary venous
connection (PAPVC), 1672
PASP. See Pulmonary artery systolic
pressure (PASP)
Patches, 78
Patent ductus arteriosus (PDA), 1599
1602, 17511754, 1755f1757f,
18051809, 1808t

anatomy, 1599

aortic runoff, 1601

cardiac catheterization, 1808

chamber dimensions, 1601

closure of, 548550, 551f552f, 1809

direction of shunt, 1601

duct morphology, 16001601

subclavian origin, 1601

usual ductus, 1600

vertical duct, 16001601

ductus, characteristics of, 1600

echocardiography, 15991600

ductal view, 1599

echocardiographic views, 15991600

objectives, 1599

suprasternal view, 15991600

echocardiography for, 18051807

hemodynamic significance of, 1601

with left-to-right shunt, 1807

MRI/CTA, 1809

postoperative adult, 1809

pulmonary arterial pressure, 1601

stepwise evaluation for, 1599t

TGA and, 1659

transesophageal echocardiography,
1808
Patent foramen ovale, 15851586
Patent foramen ovale (PFO), 7

closure of, 555, 557, 558f

ICE imaging during, 648, 650, 650f

color contrast in, 1552f
Patient prosthetic mismatch (PPM),
10911092
Pattern correlation (PC), 88
PAU. See Penetrating aortic ulceration
(PAU)
PA wedge pressure (PAW), 230, 231f
PCWP. See Pulmonary capillary wedge
pressure (PCWP)
Peak filling rate (PFR), 11571158

RT 3DE in, 1158f
Pediatric echocardiography, birth and
development of, 6
Pediatric hearts, imaging of, 285286
Penetrating aortic ulcer (PAU), 961, 962f

in elderly, 19211923
Penetrating chest trauma, 1972

close evaluation, 1972

hemothorax, 1972

impaling object, 1972

transesophageal echocardiogram
(TEE), 1972

wounds, 1972

lower left parasternal, 1972

lower right parasternal, 1972
Percutaneous continuous flow devices,
12501252

Impella device, 1250, 1251f

TandemHeart, 12501251
Percutaneous mitral balloon
valvuloplasty (PMBV), 533537,
537f539f
Percutaneous transvenous mitral
commissurotomy (PTMC), 777,
790
Perfluorocarbon gases, 13
Perfusion Score Index (PSI), 423
Pericardial cysts, 1460f, 2054, 2055f
Pericardial diseases, 14351451

acute pericarditis, 1436

anatomy of, 14351436

computed tomography for, 2064
2066

Index

congenital anomalies, 14481449

constrictive pericarditis, 1444

Doppler flow velocity records,
14441448

M-mode and 2D echo, 1444

3D echocardiography, 14521461

echocardiographic appearance,
14351436

effusive-constrictive pericarditis,
1448

fibrin deposits and, 1456

M-mode and 2D echocardiography,
14371438

multimodality imaging of
pericardium, 1450

overview, 1435

pathophysiology of, 1436

pericardial effusion, 14361437

pericardial tamponade, 14381440

Doppler flow velocity recordings,
14421443

echo-guided pericardiocentesis,
14431444

two-dimensional echo, 14401442

physiology of, 1436
Pericardial duplication cyst, computed
tomography for, 2064
Pericardial effusion, 10451047, 1436
1437

circumferential, 1500f

computed tomography for, 2065,
2065f

3D transthoracic echocardiography
advantages of, 1453t1454t

live/real-time, 1454f, 1455f1456f

vs. 2D transthoracic
echocardiography, 14531456

echocardiography of
M-mode, 1439f

two-dimensional, 1438f

epicardial fat and, 1438

exudative, 1439f

tuberculous, 1458f
Pericardial hydatid cyst, 1497f1498f
Pericardial masses, 14581461
Pericardial mesotheliomas, 1491
Pericardial metastasis, 1500f

from malignant thymoma, 1459f
Pericardial tamponade, 14381440

Doppler flow velocity records,
14421443

echo-guided pericardiocentesis,
14431444

two-dimensional echo, 14401442

Pericardial tumors, computed

tomography for, 20652066
Pericardiocentesis, monitoring of, by
CONTISCAN transducer, 230233,
232f
Pericarditis. See also Pericardial disease
acute, 1436

chronic effusive, 1437

constrictive. See Constrictive
pericarditis (CP)

effusive-constrictive, 1448

etiology of, 1437t
Pericardium. See also Pericardial disease
functions of, 1436t

multimodality imaging of, 1450

normal, 1435
Perimembranous, 15921593, 1592f,
1595f, 1596f

inlet defect, 15921593

outlet defect, 1592

trabecular defect, 1593
Perimembranous inlet defect, 15921593
Perimembranous outlet defect, 1592
Perimembranous trabecular defect, 1593
Perimembranous ventricular septal
defect, 1747f
Perimembranous VSDs, 557. See also
Ventricular septal defects (VSDs),
closure of
Peripartum cardiomyopathy, 13811384,
1381f1384f

definition, 1381

dobutamine echocardiography in,
13811384

TAPSE and, 1381

vs. DCM, 1381

in women, 19041905, 1904f1905f
Peripheral arterial tonometry (PAT),
467469, 468f, 469f
Peripheral artery/intra-coronary infusion
of Ach, 453
Peripheral hand warming, 453
Peripheral vascular ultrasound, 663701

carotid artery diseases and, 663664

anterior circulation, 665668,
667f669f

aortic arch, 664665, 665f, 666f

assessment after carotid artery
endarterectomy and stenting,
688691, 692f

cardiac pathology and ultrasound
findings, 693694

2003 carotid duplex SRU consensus
criteria, 684685

cerebrovascular anatomy, 664

collateral pathways, 669671, 670f

grading carotid stenosis, 679,
682683, 683f

grading internal carotid artery
stenosis, 683684, 684t,
685f688f

intima-media thickness and carotid
plaque assessment, 676679,
678f681f

near occlusion and total occlusion of
ICA, 685688, 690f

posterior circulation, 668669, 670f

scanning protocol, 671676,
672f675f

technical aspects of carotid studies,
671

vertebral arteries, assessment of,
691693, 694f

femoral access complications by,
694701

arterial dissection, 700

AV fistula, 699700, 701f

bleeding and hematoma, 696, 697f

patient-related risk factors, 696

procedure-related risk factors, 696

pseudoaneurysm, 696699, 698f

retroperitoneal bleeding, 696
Perivalvular abscess, 1511
Permanent pacemakers/implantable
cardioverter-defibrillators

complications in, 1212

deleterious effects of, 12171218

echocardiographic findings in,
12101212

endocarditis and, 1214

myocardial perforation, 12151217

2D transesophageal
echocardiography in, 1216, 1216f

reported cases of, 1217t

RT3DE in, 1216f

overview, 1210

RV apical pacing with LV
dyssynchrony, 12171218

thrombosis and stenosis associated
with, 1215

tricuspid regurgitation, 12121214

lead infection associated with,
11141115

time course for, 1213

transthoracic echocardiography for,
12131214, 1213f
Persistent left superior vena cava
(PLSVC), 1958

I-XXIX

PET. See Positron emission tomography

(PET)
PFRa. See PFR difference (PFRa)
PFR difference (PFRa), 1158
Phantom tumors, 737
Phased array technology, 58
Phase sensitive inversion recovery (PSIR),
2020
Phonocardiography, 25, 26f
Physio ring, 582, 591f593f
Piezoelectric crystal, 5556
PISA. See Proximal isovelocity surface
area (PISA); Proximal isovelocity
surface area (PISA) method
PISA method, 282283
PISA (proximal isovelocity surface area)
method, 70
PLAATO device, 561563
Plane wave ultrafast imaging, 1990, 1990f,
1991

elastography, 1991

fast graphic chips, 1991

radio frequency data, 1991

software-driven systems, 1990

spatial resolution, 1990

potential penalty, 1990

ultrafast doppler, 1990, 1991, 1991f

ultrasound imaging, 1990

Plaques, 677678, 680f, 681f
Plasmacytoma, 1492f

cardiac, 14901491, 1492f
PLAX. See Parasternal long-axis (PLAX)
Pleural effusion (PE), 1985

chest X-ray, 1985

computed tomography, 1985

detection, 1985

effusion, 1985

etiologies, 1985

lung appearance, 1985

minimal effusions

detection, 1985

ultrasound, 1985

ultrasound images, 1985
PLFLG-AS. See Paradoxical low-flow,

low-gradient aortic stenosis
(PLFLG-AS)
Pneumothorax (PTX), 1986

diagnosis, 1986

conditions needed, 1986

lung sliding

abolition, 1986

absence, 1986

presence, 1986

nondependent condition, 1986

I-XXX

Comprehensive Textbook of Echocardiography

pathognomonic LUS sign, 1986

radiography, 1986
Point-of-care diagnosis, 291296

acute care environment and, 294

battery-powered ultrasound imagers
and, 291292, 292f

cardiac disorders, screening and
identification of, 294

abdominal aortic aneurysm, 294295

left ventricular dysfunction, 295

mitral valve prolapse, 295

and future directions, 296

new physical examination

follow-up echocardiography, 293294

ultrasound stethoscope, applications
of, 293, 293f

preparticipation screening of
athletes, 295

remote areas and developing

countries, imaging in, 295

traditional physical examination,
292293, 292f

training requirements, 295296
Poiseuille, Jean, 67
Poiseuilles law, 66, 67
Polycystic ovarian syndrome, in women,
1899
Positron emission tomography (PET),
1429
Posterior circulation, 668669, 670f
Posterior displacement of outlet septum,
1625
Posterior wall thickness (PWTd), 1118
Postsystolic contraction (PSC), 367
Posttreadmill exercise echocardiography,
8
Power Doppler, 113, 114f

advantages and disadvantages of, 72

methodology, 7172
Power Doppler harmonic imaging
(PDHI), 421
Power modulation, 420
Power pulse inversion, 421
PPM. See Patient prosthetic mismatch
(PPM)
Pregnancy

echocardiography in, 19021904

effect of, on HV Doppler, 303
Pre-left ventricular assist device
echocardiogram, 1236t
Premature ventricular contractions, and
HV Doppler, 309, 309f
Primary benign cardiac tumors, 1464
1484

benign cardiac fibromas, 1480

cardiac hemangiomas, 14821484

cardiac lipomas, 14811482, 1483f

cardiac myxoma, 14641475

cardiac rhabdomyoma, 14801481
Primary cardiac lymphoma, 1490
Private tags, 95
Processing technology, TEE, 103, 104f,
105f
Prolonged PR interval

and HV Doppler, 308, 308f

and PV Doppler, 331, 331f
Propagation velocity, 55, 63

in different materials, 56
PROSPECT trial, 369
Prostacyclin (PGI-2), 454
Prosthetic aortic valve abscess,
1109f1110f, 1111f
Prosthetic dehiscence, 1108f1109f
Prosthetic pitch, 1047
Prosthetic regurgitation
paravalvular aortic, 1105f

paravalvular mitral, 1102f1104f
Prosthetic valve dysfunction, 10871092

endocarditis, 10881090

obstruction, 10901092

patient prosthetic mismatch,
10911092

role of 3D TEE in operating room in,
605617, 613f624f

regurgitation, 1087
Prosthetic valve infective endocarditis,
1047

abscess in patient with mechanical,
1047f

dehiscence, 1047

prosthetic pitch, 1047
Prosthetic valves, 1015, 1021f1022f,
10801093, 10921093, 10941112,
19481949

aortic position, failed homograft in,
1087f

aortic prosthetic valve stenosis, 1100

aortic valve and ascending aorta, 1096

assessment of, 10821087

cardiac catheterization, 10921093

cardiac magnetic resonance imaging,
1093, 1093f

cinefluoroscopy, 1092

clinical data for, 1083t

computed tomography scan, 1092,
1092f

3D transesophageal
echocardiography, 11001107

Index

color Doppler reconstruction, 1106f,
1107f1108f

paravalvular aortic prosthetic
regurgitation, 1105f

paravalvular mitral prosthetic
regurgitation, 1102f1104f

prosthetic dehiscence, 1108f1109f
right atrial lipoma, 1096f

3D transthoracic echocardiography,
10951100

of homograft aortic prosthesis, 1100f,
1101f

of St. Jude aortic prosthesis, 1097f,
1101f

of St. Jude mitral prosthesis, 1097f,
1098f1099f

of tissue mitral prosthesis, 1100f

3D visualization, 10941095

2D visualization, 1111t

echocardiographic evaluation of,
1084t

effective orifice area of, 10841087

in elderly, 19481949, 1949f

endocarditis of, 10991100

identification of thrombus, 1098

mitral, 1095

overview, 1080, 1094

paravalvular regurgitation, 1099

prosthetic valve dysfunction,
10871092

endocarditis, 10881090, 1089f

obstruction, 10901092

patient prosthetic mismatch,
10911092

regurgitation, 1087, 1088f

Ross procedure, 1086f, 1090f

surgical mitral and aortic clocks, 1096

types of, 10801082

bioprosthetic valves, 10811082,
1081t, 1082t, 1088f

mechanical valves, 1082, 1083t

true biological valves, 10801081

valved canduit, 1085f1086f
Proximal isovelocity surface area (PISA)
method, 82, 785, 992

for AR severity, 938939

in mitral regurgitation, 887888

MR severity and, 870871
Proximal transverse aortic arch, pulse
Doppler across, 1536f
Pseudoaneurysms
in aortic annulus, 1046f

aortic valve, 1046f

femoral, 696699, 698f

Pseudosevere aortic valve stenosis, 910f

PTMC. See Percutaneous transvenous
mitral commissurotomy (PTMC)
Pulmonary arterial hypertension (PAH),
1063, 1129

2D STE and, 372

echocardiographic prognostic
predictors in, 1073t1074t

idiopathic, 1068f1069f, 1070f

pressure response to exercise,
1071t1072t
Pulmonary artery (PA)

bifurcation, 1537f

bifurcation and smaller, 1547f

catheterization, 229230
Pulmonary artery aneurysm, in women,
1903
Pulmonary artery hemodynamics,
12691273

diastolic pulmonary artery pressure,
12701272, 1272f

mean pulmonary artery pressure,
1272, 1272f

pulmonary vascular resistance,
12721273

systolic pulmonary artery pressure,
1270, 1270f, 1271f
Pulmonary artery pressure (PAP), 1129
echocardiographic methods for,
1266t1267t
Pulmonary artery sling, 1696
Pulmonary artery systolic pressure
(PASP), 1034, 1129, 1130f, 1987t
Pulmonary atresia, 1033, 1581f
Pulmonary capillary wedge pressure
(PCWP), 1064t, 1065t, 1984
Pulmonary embolism (PE), 1067, 1985,
1986

anticoagulation treatment, 1986

color Doppler imaging, 1986

consolidated lung tissue, 1986

associated mechanical alterations,
1986

disgnosis, 1985

localization, 1985

localized multiple B-lines, 1986

lung ultrasound scan

accuracy, 1985

echocardiography, 1986

leg vein compression sonography,
1986

pleuritic chest pain, 1985

pulmonary arterial flow area, 1986

sonographic findings, 1985

characteristics, 1985

ultrasonic window, 1985
Pulmonary flow acceleration time (PAcT),
1270
Pulmonary hypertension (PH), 171, 890,
10631079

clinical classification of, 1064t1065t

conventional echocardiography,
10631070

2d echocardiographic features, 1067

pulmonary hemodynamics, 1063
1067

stress echocardiography, 10671070

transesophageal echocardiography,
1067

2d echocardiographic signs, indirect,
1069t

diagnosis of
echocardiography role in, 1075f

ESC guidelines for, 1067

diagnostic algorithm in, 10731074

Doppler echocardiographic indices
of, 1065t

hemodynamic definitions of, 1064t

and HV Doppler, 311, 313, 313f

mitral stenosis and, 836838

mitral valve echocardiographic
scoring system, 836

M-mode echo for, 7, 7f

nonconventional echocardiography,
10701073

real time, 3d echocardiography, 1073

strain imaging, 10721073

tissue Doppler imaging, 10701072

overview, 1063

RT 3DE of, 1170

RV systolic function in, impaired,
1069t, 1070f

velocity vector imaging in, 399

Wilkins scoring system, 836837

in women, 19011092

severe, 1903f
Pulmonary interstitial edema, 1984, 1985

diagnosis, 1984

American College of Cardiology

guidelines, 1984

B-lines, 1984

chest X-ray, 1984

chronic heart failure (HF), 1984

gray zone, 1984

limitations, 1984

pulmonary capillary wedge pressure,
1984

prognosis, 1985

I-XXXI

B-lines significance, 1985

heart failure hospitalization, 1985

persistent hemodynamic congestion,
1985

treatment, 1984, 1985

B-lines, 1985

degree of dyspnea, related to, 1985

pharmacological therapy, 1985

tailoring, 1985

pulmonary congestion, reducing of,
1984

medicines effectiveness assessment,
1984

monitoring body weight, 1984

ventricular dysfunction, 1985
Pulmonary regurgitation, 1036, 1038f,
1039f

causes of, 1037f

severe, 1037f
Pulmonary stenosis, 171, 10321036

acquired causes of, 1033f

congenital causes of, 1032f

echocardiographic assessment of,
10341036, 1034f, 1035f1036f

continuous wave Doppler signal in,
1034f

enlarged systolic frame, 1036f

midtransesophageal view, 1036f

patient with tetralogy of fallout, 1035f

transpulmonary valve velocity, 1035f

etiology, 1032

grades of, 1034

normal pulmonary valve area in,
1034

pathology, 1032

pulmonary atresia, 1033

secondary anatomic changes in, 1033
types of, 1033
Pulmonary thrombo disease, 1976

arotic dissection, 1976f

dilatation, 1976

echocardiographic evaluation, 1976

Echogenic structure, 1977f

false lumen, 1977f

post diagnosis, 1976

pulmonary hypertension, 1977f

pulmonary thrombo-embolic, 1976

pulmonary thromboembolism, 1976,
1976f

right ventricle/left ventricle end diastolic dimension, 1976

true lumen, 1977f

ventricular dysfunction, 1976
Pulmonary valve, 10311041

I-XXXII

Comprehensive Textbook of Echocardiography

in adults, 18161818

cardiac catheterization, 1817

MRI/CTA for, 1817

postoperative adult, 1818

surgical treatment for, 18171818

cardiac CT scans, 1040

cardiac MRI, 1040

catheterization, 1040

congenital diseases in newborns,
incidence of, 1032t

echocardiographic evaluation,
10371038

effective regurgitant orifice area, 1038

epidemiology, 10311032

parasternal short-axis view of, 1034f

postpulmonary valve surgery,
10391040

allograft replacement, 1040

pulmonary regurgitation, 1036, 1038f

causes of, 1037f

severe, 1037f

pulmonary stenosis, 10321036

acquired causes of, 1033f

congenital causes of, 1032f

echocardiographic assessment of,
10341036, 1034f, 1035f1036f

etiology, 1032

grades of, 1034

normal pulmonary valve area in,
1034

pathology, 1032

pulmonary atresia, 1033

secondary anatomic changes in, 1033

types of, 1033

pulse Doppler across, 1536f

Ross procedure, 10381039

artificial conduits in, 1039

pulmonary homograft, 1038, 1039f

right ventricular outflow tract, 1038
Pulmonary valve fibroelastoma, 1478f
Pulmonary vascular resistance (PVR),
1063, 1065t, 1066, 12721273
Pulmonary vein ablation
cardiac computed tomography for,
20542057

computed tomography for, 2054
2057
Pulmonary vein isolation, for atrial
fibrillation, 566, 568569, 569f,
570f
Pulmonary veins (PV), 16701684, 1963

ablative therapies, 1963

abnormalities, 1963

additional ablation, 1963

anatomy, 1963

delineating, 1963

anatomy of, 325

anomalies of, 16721673

abnormal number of pulmonary
veins, 1672

anomalous pulmonary venous
return, 16721673

blood flow in

aging and, 329330, 330f

factors affecting, 329331

loading conditions and, 330331

physiology of, 325327, 326, 328f

respiration and, 329

cardiac MR, 1963

color contrast in, 1552f

color Doppler of, 1533f

CT angiography, 1963

disease states, and PV blood flow

atrial fibrillation, 333, 333f

atrial flutter, 333, 333f

atrial septal defect (ASD), 342, 342f

atrioventricular dissociation,
331332, 332f

cardiac tamponade, 341

conduction disorders, 331

constrictive pericarditis, 340341,
340f, 341f

junctional rhythm, 333, 334f

left ventricular end-diastolic
pressure, assessment of,
334335, 334f, 335f

mean left atrial pressure, assessment
of, 335, 335f

mitral regurgitation, 336338, 337f,
338f

mitral stenosis, 338339, 339f

myocardial relaxation pattern,
impaired, 335336

paced rhythm, 334, 334f

premature ventricular contractions,
332, 332f

prolonged PR interval, 331, 331f

pseudonormal filling pattern, 336

pulmonary vein stenosis, 342, 342f

rate and rhythm disorders, 332334

restrictive cardiomyopathy, 339340,
340f

restrictive filling pattern, 336, 337f

short PR interval, 331, 331f

sinus bradycardia, 332, 333f

sinus tachycardia, 332, 332f

flaring degree, 1964

floating thrombus, 1965f

Index

flow pattern, 1964

atrial reversal, 1964

diastolic dysfunction, 1964

diastolic forward flow, 1964

mitral regurgitation, 1964

systolic forward flow, 1964

A Flutter, 1963

imaging, 1963

imaging of, 325329

technical considerations in, 327, 329,
329t

transesophageal echocardiography,
327

transthoracic echocardiography, 327

isolation, 1963

left inferior, 1963

left upper, 1963f

limitations and technical pitfalls, 342,
342f

localization, 1964

lower, 1963f

macro-reentrant tachycardia, 1963

midesophageal, 1963

level, 1963f

probe, 1963

morphological features, 1963

normal flow pattern of, 16701672

ostium, 1964

peak diastolic velocity, 1965f

pulmonary hypertension, 1964

pulse Doppler of, 1533f

right middle, 1965f

to right-sided left atrium, 1546f

right upper, 1963f

caval view, 1964

spectral Doppler of, 325345

artifacts, 343345, 344f345f

differential diagnosis for abnormal
PV flow patterns, 345, 345f

limitations and technical pitfalls,
342343, 344f

stenosis, 1965f

development, 1964

systemic venous anomalies, 1678
1684

systolic forward flow, 1964

thoracic aorta,descending, 1963

total anomalous pulmonary
venous connection, 16731678,
1673f, 1674f1675f

anomalous drainage of, 1676

diagnosis steps in, 16731676

echocardiographic goals, 1673

features of, 1673

pulmonary vein stenosis and, 1678

pulmonary venous confluence,
16731675

septum primum malposition defect
and, 16761678, 1677f
Pulmonary vein stenosis
and PV Doppler, 342, 342f

with TAPVC, 1678
Pulmonary venous confluence (PVC),
16731675, 1674f

orientation and site of drainage of,
16741675
Pulmonary venous flow, MR severity and,
869
Pulmonic valve

3D echo of, 522523, 522f, 523f

ventricular assist devices and, 1232
Pulmonic valve disease, 3DE assessment
of, 284, 284f, 285f
Pulsed Doppler, 6364. See also Spectral
Doppler
Pulsed wave Doppler

advantages and disadvantages of, 69

and aliasing, 70, 70f

methodology, 6970, 69f
Pulse inversion, 420
Pulse pressure (PP), 467
Pulse repetition frequency (PRF), 63, 69,
736
Pulse wave velocity (PWV) analysis,
466467, 466f, 467f
PVR. See Pulmonary vascular resistance
(PVR)
PWTd. See Posterior wall thickness
(PWTd)
Pyramidal imaging, 241
PZT (lead zirconate titanate) ceramics,
102, 103f

Q
QLab, 385,514
Quadrature detector, 66
Quadricuspid aortic valve, 1620, 1620f
in female, 1915f
Quantification techniques in
echocardiography, noninvasive,
705729

aortic valve assessment, 727728,
728f

3DE quantification tools, clinical
applications of, 721723

left ventricle global and regional
function, 722

left ventricle mass, 722, 722f

left ventricle shape analysis, 722723,
723f

fluid mechanics algorithms and
particle imaging, 720721, 721f

future outlook, 728729, 729f

global strain, 712714, 712f715f

goal of, 706

heart chamber segmentation
algorithms, 706707, 707f

limitations of speckle tracking
echocardiography, 720

mitral valve assessment, 725727,
726f728f

parametric display, 708711,
709f711f

regional strain, 714715, 715f, 716f

right ventricular quantification,
723725, 723f726f

rotation, twist, and torsion, 717720,
719f

segmental/regional analysis of left
ventricle, 707708, 708f, 709f

strain quantification and speckle
tracking algorithms, 711712

three-dimensional speckle tracking
and strain, 715, 717, 717f719f
Quantitative gated single-photon
emission computed tomography
(QGSPECT), 271

R
RABT (red away blue toward), 64
Radial strain, 362, 363f, 389
Range ambiguity, 736. See also Artifacts
RAP. See Right atrial pressure (RAP)
Rashkind procedure, 531
RBBB. See Right bundle branch block
(RBBB)
RCM. See Restrictive cardiomyopathy
(RCM)
Reactive hyperemic index (RHI), 467468
Reactive oxygen species (ROS), 454
Real time, three-dimensional TEE
(RT3DTEE) probe, 101102, 101f,
102f
Real time 3D echo (RT3DE), 11421143
Real time (RT) 3D mode, 269
Real time (RT) 3D TTE, 74
Real time/live 3D echocardiography, 241
Real time myocardial perfusion
echocardiography (RTMPE),
444445

I-XXXIII

Real time three-dimensional

echocardiography (RT3DE)

future perspectives of, 1728

left ventricular ejection fraction,
17221723

analysis in children, 1723, 1724t

meta-analysis, 1723

for left ventricular mass, 1723

left ventricular volumes, 17221723

in adults with congenital heart
disease, 1722

analysis in children, 1723, 1724t

correlation with CMR, 1722

meta-analysis, 1723

overview, 17211722

regional wall motion and synchrony,
17261727

right ventricular ejection fraction,
17231725

analysis in children, 17241725

meta-analysis of, 1725

right ventricular volumes, 17231725

analysis in children, 17241725

meta-analysis of, 1725

single ventricular ejection fraction,
17251726

single ventricular mass, 17251726

single ventricular volumes, 1725
1726

strain analysis by, 17271728
Receiver, 60
Red blood cells (RBCs), 416
Reflection artifacts, 62, 62f
Regional strain, 714715, 715f, 716f
Regional wall motion and synchrony,
RT3DE of, 17261727
Region of interest, 71
Registered Cardiac Sonographer (RCS),
754
Registered Cardiovascular Invasive
Specialist (RCIS), 754
Registered Diagnostic Cardiac
Sonographer (RDCS), 754
Registered Vascular Specialist (RVS), 754
Registered Vascular Technologist (RVT),
754
Regurgitant jet, spectral strength of, 811t
Regurgitant volume (RV), 1376
Reid, John, 6
Remote ventricular septal defect, 1648
Renal disease, 18701872
Reperfusion, speckle-tracking
echocardiography, 1302
Resolution, 58, 655

I-XXXIV Comprehensive Textbook of Echocardiography

axial, 59, 59f

frame rate, 59, 60f

lateral, 59, 59f

line density, 60
Respiration, effect of

on hepatic venous flow, 302303,
303f, 306, 307f

on pulmonary venous flow, 329
Resting myocardial perfusion
abnormality, 2044f
Restrictive cardiomyopathy (RCM), 395,
1369, 13971404

amyloid cardiomyopathy, 13981401,
1399f1400f

contrast echocardiography, 1402

Doppler echocardiography, 1398

endomyocardial fibrosis, 14021404,
1404f

Fabry disease, 14011402, 1401f

and HV Doppler, 315, 315f

hypereosinophilic cardiomyopathy,
1402

and PV Doppler, 339340, 340f

transesophageal echocardiography,
1402

transthoracic echocardiography,
1402
Retroaortic innominate vein, 1682, 1682f
Retrograde aortic arch flow, 1551f
Retrograde ductal arch (DuAr), 1554f
Retroperitoneal bleeding, 696
Reverberation artifacts, 62, 62f, 734735,
735f. See also Artifacts

transesophageal echocardiography
and, 107, 109110, 109f
Reynolds, Osborne, 67
Reynolds number, 66, 67
Rhabdomyosarcomas, 14861488
Rheumatic heart disease (RHD), 1859

ARF and, 774

chronic, 775

morphological features of, 777t

tricuspid stenosis and, 1006f

WHF echocardiographic criteria for,
775, 776t
Rheumatic mitral regurgitation, 863
Rheumatic mitral stenosis, 533537,
537f539f

in female, 1890f1891f, 1893f1984f
Rheumatic valvular heart disease, 898
Rheumatoid arthritis (RA), 1868
Right aortic arch, 16901691
Right atrial lipoma, 1096f
Right atrial myxomas, 1470f, 1473

Right atrial pressure (RAP), 1063, 1065t,

11431144, 12641269

Doppler and tissue Doppler imaging,
12681269, 1269f

echocardiographic methods for,
1265t

estimation of, 1066t

IVC parameters, 12641266, 1266f

size and collapsibility, 1266
RA dimensions, 1269, 1269f

systemic venous flow, 1268, 1268f

Right atrial thrombus, 1504f
Right atriumright ventricle (RARV),
1958
Right atrium thrombus, 1505f
Right bundle branch block (RBBB), 1726
Right coronary artery fistula, 17821783
Right heart failure, and cardiac motion,
1204
Right isomerism, 1577
Right parasternal approach, of three dimensional echocardiography,
246, 248, 259f266f
Right pulmonary artery (RPA), short axis
of, 1536f
Right-sided infective endocarditis,
10471049

transesophageal approach, 1048f

type of patient with, 10481049
Right upper pulmonary vein (RUPV), 327,
328f
Right ventricle, 1198

cardiac motion and, 1198

double outlet, 1547f

evaluation of, 1235t

failure, 1247f

ventricular assist devices and,
12331234
Right ventricle, 3D quantitation of,
11651170

anatomic considerations, 11651166

conventional approaches, 11651166

2D echocardiography, 1166f

3D reconstruction approaches,
previous, 1167

global function, 1166

longitudinal contraction, 1166

retrosternal location of, 1166
RT 3DE approach to, 11671170

apical rotation method, 1168f

automatic boundary tracking
algorithm, 1169f

of congenital heart disease, 1170

data acquisition, 1167f

Index

disc summation method, 1169f

of pulmonary hypertension, 1170

second-generation, 11671168

transverse shortening and, 1166
Right ventricle, evaluation of, 11341148

apex-forming, 1136

cardiac magnetic resonance imaging,
1136f, 11441145, 1144f

cardiovascular computed
tomography, 1145

contractile function, 11401141

Doppler echocardiography, 1139
1141

continuous wave Doppler spectral
display, 1139

conventional doppler, 11391140

pulsed wave Doppler spectral
display, 1140, 1142f

tissue doppler, 11401141, 1141f

echocardiography, 1136

M-mode, 11361138

three-dimensional, 11421143

transesophageal, 1143

two-dimensional, 11381139, 1139f,
1140f, 1141f

echo windows for, 1137f

gadolinium contrast in, 1145

geometry of, 1135f

hemodynamics, 11431144

HV systolic filling fraction, 1144

right atrial pressure, 11431144

morphology, 11351136

regional RV wall motion, 1136

right ventricular outflow tract, 1135
RV systolic function, 11351136

myofibrillar arrangement of, 1136
1137

overview, 11341135

evaluation of, 11341148

real time 3D echo, 11421143

Simpsons method of discs for, 1140f

triangle of dysplasia, 1135, 1135f

two-dimensional strain (speckle
tracking), 11411142, 1142f

volume/body surface area, 1143t
Right ventricle fractional area change
(RVFAC), 1071f
Right ventricular diastolic dysfunction,
1644f

and HV Doppler, 313315, 315f

Right ventricular dysfunction

ICM/NICM and, 1422

mild residual, 1242f

Right ventricular ejection fraction

(RVEF), 381, 11371138

global function of RV, 1166
Right ventricular end-diastolic pressure
(RVEDP), 309310, 310f
Right ventricular end-diastolic volume
(RVEDV), 1168
Right ventricular endomyocardial biopsy,
571
Right ventricular fibroma, 1481f
Right ventricular function, TD
assessment of, 354, 356f357f
Right ventricular hemangioma, 1486f
Right ventricular lipoma, 1483f
Right ventricular myxomas, 1471f1472f,
14731474, 1473f
Right ventricular noncompaction,
1389f1391f

isolated, 1392f
Right ventricular outflow tract (RVOT),
1135, 1138

flow velocity envelope, 1068f

outcomes of allograft conduits for,
1039

pulmonary hypertension and,
10661067, 1067f
Ross procedure and, 1038
Right ventricular quantification, 723725,
723f726f
Right ventricular sarcoma, 1487f
Right ventricular stroke volume (RVSV),
1167
Right ventricular systolic dysfunction,
313, 314f
Right ventricular thrombus, 1506f
Right ventricular wall (RVW), 1971t
RIMP. See RV index of myocardial
performance (RIMP)
Ring dehiscence, 605606
Rotatable transesophageal echo (TEE)
probe, 100, 100f, 101f
Rotation (cardiac motion), 1183
RSOV. See Ruptured sinus of Valsalva
(RSOV)
RT3DE. See Real time 3D echo (RT3DE)
Ruptured sinus of Valsalva (RSOV), 1632
RVEDV. See Right ventricular end diastolic volume (RVEDV)
RVEF. See Right ventricular ejection
fraction (RVEF)
RVFAC. See Right ventricle fractional area
change (RVFAC)
RV FAC. See RV fractional area change
(RV FAC)

RV fractional area change (RV FAC), 1139

RVFS. See RVOT fractional shortening
(RVFS)
RV index of myocardial performance
(RIMP), 1141
RVOT. See Right ventricular outflow tract
(RVOT)
RVOT fractional shortening (RVFS), 1138
RV regional isovolumic relaxation time
(RVrIVRT), 1268
RVrIVRT. See RV regional isovolumic
relaxation time (RVrIVRT)
RVSV. See Right ventricular stroke volume
(RVSV)

S
Sacrococcygeal teratoma, 1530
Saline contrast, 1213
Saline contrast chocardiography, 435,
436f
SAM. See Systolic anterior motion (SAM)
Sapien valve, balloon expandable,
541543
Sarcoidosis, 1405, 1405f, 18761879
Sarcomas, 14841488, 2054

right ventricular, 1487f
SAVR. See Surgical aortic valve
replacement (SAVR)
Scan line, 77
Scanners, trends, 19911993

acoustic structure quantification,
liver, 1992, 1992f

advantage, 1992

B-mode signal

analysis, 1992

cable-free transducer systems, 1992

development of, 1992

capacitance micro-machined
ultrasound transducer, 1992

complete cross-sectional display

computed tomography, 1993

magnetic resonance, advantages of,
1993

contrast-enhanced ultrasound, 1991,
1992

crystal piezoelectric materials, 1991

electrostatic loudspeaker, 1992

fusion imaging, 1993f

Lead zirconate titrate, 1992

miniaturization, 1991

scanner user interfaces, 1993

semiconductors, 1992

smartphone ultrasound system, 1991,
1991f

I-XXXV

software-driven scanners, 1991

symmetrical effect, 1992

tissue characterization conference,
1992

transesophageal echocardiography

live/real time three/four dimensional, 1992, 1992f

ultrasound system, 1991
SCD. See Sudden cardiac death (SCD)
Sclerosing mediastinitis, 1515f1517f
Screen, 60
Secondary cardiac tumors, 14921500

carcinoid heart disease, 1500

malignant melanoma, 1500
Sector angle, and frame rate, 59, 60f
Sector probe, 58, 58f
Secundum ASD, 17341742

device embolization in, 1739

3D TEE in, 17381739, 1739f,
1740f1742f

repair of, 17341736
Semilunar valves, short axis of, 1536f
Septal aneurysms, 182
Septal wall thickness at diastole (SWTd),
1118
Septic cardiomyopathy, 14051407, 1406f

echocardiographic features of,
11406407
Septum (ventricular), 11941198, 1205f

echocardiographic pattern of, 1196f

endocardial and epicardial fibers,
1195f

fiber orientation of, 1195f

septal line, 1194

ultrasonic crystal tracings, 1197f
Septum primum malposition defect

TAPVC and, 16761678, 1677f
Serial evaluation, of patients with 3DE,
11641165
Shah, Pravin, 11
Shear rate, 452
Shear stress, 452454, 452f454f, 455. See
also Endothelial dysfunction
Short PR interval

and HV Doppler, 307, 308f

and PV Doppler, 331, 331f
Short-tau-inversion-recovery (STIR),
2020
Short-term circulatory support devices,
12261227

Abiomed AB5000, 1227

Impella catheter-based assist device,
1227

TandemHeart system, 1227

I-XXXVI Comprehensive Textbook of Echocardiography

Thoratec CentriMag system, 1227

Thoratec paracorporeal ventricular
assist device, 1227
Shunt lesions

aortopulmonary window, 16021603

atrial septal defects, 15851591

features of, 15821585

Gerbode defect, 1603, 1603f, 1604f

ventricular septal defect, 15911599
Shunt lesions, in adults, 17981813

atrial septal defects, 17991802, 1801t

cardiac catheterization, 1802

closure of, 1802

contrast echocardiography, 1801
1802

echocardiography, 1799

exercise testing, 1802

MRI/CTA for, 1802

postoperative adult in, 1802

transesophageal echocardiography,
18001801, 1800f

types of, 17991800, 1799f

atrioventricular septal defect,
18091810

echocardiography, 18091810

postoperative adult, 1810

coronary artery fistula, 18121813

patent ductus arteriosus, 18051809,
1808t

cardiac catheterization, 1808

closure of, 1809

echocardiography for, 18051807

with left-to-right shunt, 1807

MRI/CTA, 1809

postoperative adult, 1809

transesophageal echocardiography,
1808

patent foramen ovale, 17981799

persistent left superior vena cava,
18101811

echocardiography, 1810

MRI/CTA, 18101811

postoperative adult, 1811

sinus of Valsalva aneurysm, 1811
1812

ventricular septal defects, 18021805,
1806t

cardiac catheterization, 1805

closure of, 1805

echocardiography, 18041805

inlet, 1804

locations of, 1804f

membranous, 1803

MRI/CTA for, 1805

muscular, 1804

postoperative adult, 1805

supracristal, 1803

venturi effect, 1803f
Shunt lesions/septal defects, 17331747

atrial septal defects, 1734

atrioventricular septal defectsts,
17461747

secundum ASD, 17341742

sinus venosus ASD, 17421743

unroofed coronary sinus, 1743

ventricular septal defects, 17431746
Sickle cell disease, three-dimensional
speckle tracking and, 376t
Side lobe artifacts, 6263, 63f, 736. See
also Artifacts

transesophageal echocardiography
and, 107, 108f
Sigmoid septum, of elderly, 167
Single element transducers, 76, 76f
Single-photon emission computed
tomography (SPECT), 14251427
Single plane transesophageal echo (TEE)
probe, 99, 99f
Single ventricle, velocity vector imaging
in, 392394
Sinotubular (ST) junction, 168, 1621
Sinus bradycardia

and HV Doppler, 306, 307f

and PV Doppler, 332, 333f
Sinus of Valsalva aneurysm, 16301632,
1631f, 1784

in adults, 18111812

aortic regurgitation and, 1631

associated anomalies, 16311632

echocardiographic evaluation of,
16301631

hypoplastic left heart syndrome, 1784

right ventricular outflow obstruction
and, 1784

ruptured right, 1784f1785f

VSD and, 1631
Sinus tachycardia

and HV Doppler, 306, 307f

and PV Doppler, 332, 332f
Sinus venosus ASD (SVASD), 1586,
17421743, 1743f
Sinus venous defect, 1800
Sjogrens syndrome, 1545
SLE. See Systemic lupus erythematosus
(SLE)
Slicing methods, 79
Smearing, 59
Society of Cardiovascular
Anesthesiologists (SCA), 638

Index

Sonar system, development of, 4

Sonic reflector, good, properties of, 65
SonoHeart, 292f
Sonovue, 417, 418t. See also Contrast
echocardiography
Sound velocity, effect of, 107, 107f, 107t,
108t
Sound waves, 5556, 56f
SPAMM. See Spatial modulation of
magnetization (SPAMM)
SPAP. See Systolic pulmonary arterial
pressure (SPAP); Systolic
pulmonary artery pressure (SPAP)
SPARC. See Stroke Prevention:
Assessment of Risk in a
Community (SPARC) study
Spark gap position-locating approach, 14
Spatial and temporal resolution, 3D
imaging and, 80, 80f
Spatial modulation of magnetization
(SPAMM), 2000
Spatial resolution, 55
Spatiotemporal image correlation (STIC),
285286, 1550
Speckle tracking acquisition, 8798

arrhythmias and, 98

2D speckle tracking, limitation of, 92

gain setting, 97

M-mode, 8788, 88f

multiview monitoring during live
acquisition, 97, 97f

multiview orientation, 97, 98f

patient breath-hold, 98

RR interval, 91

standardization, 9192

standard views, 91

three-dimensional acquisition, 92,
94f95f

frequency, 95

modes, 96, 97f

one-beat acquisition, 96

raw data storage, 95

scan range/volume width, 96

triggered full volume, 96, 97f

volume rate, 96

wall motion tracking, 92, 95

versus tissue doppler imaging, 92

two-dimensional speckle tracking,
88, 88f

acquisition considerations, 88

depth, 89

dynamic range, 90

frame rate, 8889, 89f

gain level, 89, 89f

high frame rate, 89

imaging mode, 90, 90f

lateral gain, 8990

raw data format, 90

scan range, 89

two-dimensional frequency, 90
Speckle-tracking echocardiography
(STE), 360376, 382, 13021304.
See also Velocity vector imaging
(VVI)

cardiac muscular anatomy, 360361,
361f

image acquisition and processing,
367

limitations of, 374 375

myocardial infarction, 1302

myocardial ischemia, 1302

myocardial viability, 13021303

reperfusion, 1302

strain and, 362365, 362t, 363f

three-dimensional, 372373, 373f,
374f

area strain measurement and, 373,
375, 375f, 375t

clinical applications of, 373374, 376t

and tissue Doppler imaging, 360, 361t

two-dimensional, 365367, 365t, 366t

cardiac transplantation and, 371

cardiomyopathies and, 369371

chemotherapy cardiotoxicity and,
371372

clinical application of, 367372, 368t

congenital heart disease and, 372

coronary artery disease and, 367

CRT for heart failure and, 369

right ventricular function and, 372

rotational dynamics, role of, 372, 372f

valvular heart diseases and, 371
SPECT. See Single-photon emission
computed tomography (SPECT)
Spectral broadening, 68
Spectral Doppler, 66, 112113, 113f

transthoracic echocardiogram,
136137, 137f
Splenic syndromes, 1704
Squatting stress echocardiography,
13231327, 13241326

acute LV remodeling on, 1325

advantages of, 1326

chronic obstructive pulmonary
disease, 1323

coronary artery disease, 1323

electrocardiography (ECG), 1323

end-systolic frames in, 1324f

limitations of, 1326

LV function and, 1324f

mechanism of, 1325

overview, 1323

protocol, 1324

results/observations, 13241325

vs. dobutamine stress
echocardiography, 1325, 1326t

wall motion abnormalities, 1323

left ventricular, 13241325

mechanism of, 1325
St. Jude aortic valve, 1085f
St. Jude bileaflet mitral valve, 1092f
Stanfard classification, 1974

apical, 1976t

ascending aorta, 1974

diastolic collapse, 1976

dissections, 1974

esophagus, 1976

left parasternal, 1976t

lumen, 1976

mitral valve, 1975f

pressure Half-time, 1975t

systolic expansion, 1976

transesophageal probe, 1976

transthoracic views, 1976t

visualization, 1974
Stanford type A aortic dissection, 935,
936f
Staphylococcus aureus, 1048
STARFlex occluder, 557
StarrEdward valve, 1085f
State Food and Drug Administration
(SFDA),China, 110
State-of-the-art, 11801181

composite of, 11811183

HVMB model, 1181

muscle contraction, asynchronous,
1181
Statins, 450
Steady-state free precession (SSFP), 2020

for aortic regurgitation, 2010
Stenosis of pulmonary outflow, 1647
1648

aortic outflow obstruction, 1647

remote ventricular septal defect, 1648

ventricular septal defect, restriction
of, 1647f, 1648
Stenotic aortic valve, morphology,
16191620
STIC. See Spatiotemporal image
correlation (STIC)
Stiffness index (SI), 465, 467

I-XXXVII

Strain

calculation of, 362

imaging, 360

normal, 385, 389

normal rotation and torsion values,
364t

normal values for, in normal adults,
364t

peak, 365

physics of, 385, 389, 389f

rate, 362

shear, 385, 389

types of, 362, 362t, 363f
Streptococcus viridans, 1054
Stress (Takotsubo) cardiomyopathy

three-dimensional speckle tracking
and, 376t
Stress echocardiography, 226227,
13061322

cardiac event rate as function of,
1315f

cost-effectiveness of, 13171318

Doppler hemodynamics with,
13181319

aortic valve disease, 1318

dynamic pulmonary hypertension,
13181319, 1319f

hypertrophic cardiomyopathy, 1318

latent diastolic dysfunction, 1318

mitral valve disease, 1318

fundamentals of, 13061307

future directions for, 1319

impact on patient outcome, 1317

interpretation of, 13091319

diagnostic accuracy to detect CAD,
1310, 1313t

transient ischemic LV cavity
dilatation, 1315

warranty time, 1317

myocardial infarction vs. cardiac
death by, prediction of,
13111315

myocardial perfusion and, 1319

overview, 1306

predictors of risk, 1314t

prognostic value of, 1316t

risk stratification and prognosis,
13101315, 1314f

left atrial size in, role of, 1317

myocardial wall motion abnormality,
1311

RV wall motion abnormalities in, role
of, 1315

safety of, 13081309

I-XXXVIII Comprehensive Textbook of Echocardiography

three dimensional, 1319

training in, 757

types of, 13071309

contrast echocardiography, 1308

dobutamine stress echocardiography,
1307, 1308f

exercise echocardiography, 1307,
1308f

vasodilator stress echocardiography,
13071308

vs. nuclear SPECT imaging, 1313t
Stress testing, three-dimensional
echocardiography for, 274, 274f
Stroke distance (SD), 1280, 1281f
Stroke Prevention: Assessment of Risk in
a Community (SPARC) study, 1921
Stroke volume (SV), 1280, 1281f

in DCM, 1375

in dilated cardiomyopathy, 1375
Structural heart disease, in women,
18881889

mitral valve calcification, 1889

mitral valve prolapse, 1888,
1888f1889f

mitral valve stenosis, 18881889
Subaortic stenosis, 1770

in adults, 18241825
Subcostal approach, of three dimensional echocardiography,
244, 256f255f
Subcostal window, TTE

color and spectral Doppler imaging,
158159

four-chamber view, 155, 156f, 156t,
157f

great vessel imaging, 157158, 158f,
159f

abdominal aorta, 158, 159f

hepatic vein, 158, 158f

inferior vena cava, 158, 158f

short-axis views, 157, 157f
Subendocardial muscle, 11981200,
1199f, 1200f
Subvalvular aortic stenosis, 16241626,
1624f

aortic insufficiency and, 1626

cause of, 1624

diffuse tunnel obstruction and, 1626

discrete subvalvular, 1626

echocardiographic evaluation of,
1624

fibromuscular collar in, 1625

fixed subaortic obstruction, 1624
1625

membranous subaortic stenosis,
1625

posterior displacement of outlet
septum in, 1625
Subvalvular infundibular stenosis, 1033,
1035f, 1036f
Sudden cardiac death (SCD), 1348
Summed difference score (SDS), 1158

vs. WMS difference, 1159f, 1159t
Summing, 77
Superior vena cava (SVC), 1140

anomalies of, 16781682

absent right, 1680

aneurysm of, 1682

clinical significance of, 16801681

defect in wall of coronary sinus,
16791680

left superior vena cava to coronary
sinus, 16791680

left superior vena cava to left atrium,
1680

persistent left, 16781679

right superior vena cava to left
atrium, 1681, 1681f

heterotaxy syndrome and, 1705

normal spectral Doppler of, 304, 304f
Supraclavicular approach, of three dimensional echocardiography,
244, 258f259f
Suprasternal approach, of three dimensional echocardiography,
244, 257f
Supravalvular aortic stenosis, 16261628,
1626f

in adults, 18251826

aortic valve anomalies, 1627

branch pulmonary arteries in, 1628,
1628f

coronary artery abnormalities, 1628

morphology, 1627
Supravalvular stenosis, 1033
Supraventricular tachycardia (SVT), 1542,
1957
Surface rendering, 79, 79f
Surgical aortic valve replacement (SAVR),
540541

in aortic stenosis, 926927

in LFLG-AS with low ejection
fraction, 927

PLFLG-AS and, 922

in PLFLG-AS with normal ejection
fraction, 927
Suture-less valves, 606
SVC. See Superior vena cava (SVC)

Index

Swirling, 433, 434f

Swiss cheese defects, 1594
SWTd. See Septal wall thickness at
diastole (SWTd)
Systemic diseases, 18671885

amyloidosis, 18721874, 1873f1874f

carcinoid tumors, 18741875

Chagas disease, 18751876,
1877f1878f

echocardiography in, 18671885

hypereosinophilic syndrome,
18681869, 1870

nutritional deficiency, 1880

overview, 1867

renal disease, 18701872

rheumatoid arthritis, 1868

sarcoidosis, 18761879

systemic lupus erythematosus,
18671868

systemic sclerosis, 18691870

thyroid disorders, 18791880
Systemic hypertension, in female, 1910f
Systemic lupus erythematosus (SLE),
1545, 18671868

in female, 1912f
Systemic pulmonary shunts (QP/QS),
1280
Systemic sclerosis, 18691870
Systemic veins, anomalies of, 16781684

classification of, 1678

coronary sinus, abnormalities of,
1683

decompressive venous channels,
1684

inferior vena cava, abnormalities of,
16821683

bilateral, 1683

inferior vena cava interruption,
16821683

inferior vena cava to left atrium, 1683

retroaortic innominate vein, 1682,
1682f

superior vena cava, 16781682

absent right, 1680

aneurysm of, 1682

clinical significance of, 16801681

defect in wall of coronary sinus,
16791680

left superior vena cava to coronary
sinus, 16791680

left superior vena cava to left atrium,
1680

persistent left, 16781679

right superior vena cava to left
atrium, 1681, 1681f

total anomalous systemic venous
drainage, 1684

venous valves, 16831684
Systolic abnormalities, of tricuspid valve,
986
Systolic anterior motion (SAM), 1348

echocardiographic evaluation of,
13511352, 1352f

mitral-septal contact, 1359f

of mitral valve, 13501354, 1350f,
1359f

pathophysiology of, 1353
Systolic blood pressure (BP),
pseudonormalization of, 911
Systolic dysfunction, and HCM, 1356
1358
Systolic dyssynchrony index (SDI), 373,
1726
Systolic function

in dilated cardiomyopathy, 1371

in ICM/NICM, 1419
Systolic myocardial dysfunction, 1131f
Systolic pulmonary artery pressure
(SPAP), 1063, 1065t, 1066, 1067,
1270, 1270f, 1271f

T
TAA. See Thoracic aortic aneurysm (TAA)
Tachycardia-induced cardiomyopathy,
1388, 1395f
Takotsubo cardiomyopathy, 1388, 1394f

in women, 18991900

vector velocity imaging, 1900
TandemHeart device, 1225f, 1227,
12501251
TAPSE. See Tricuspid annular plane
systolic excursion (TAPSE)

in peripartum cardiomyopathy, 1381
TAPVC. See Total anomalous pulmonary
venous connection (TAPVC)
TAPVR. See Total anomalous pulmonary
venous return (TAPVR)
TaussigBing anomaly, 1647
Tei's index, 1120, 1120f, 1419
Temporal tap, 674, 675f
Tenting area, 1422, 1423f
Tetralogy of fallot (TOF), 1763

with absent pulmonary valve, 1637,
1637f

in adults, 18291835

cardiac catheterization, 18341835

echocardiography, 18301832

MRI/CT for, 1835

postoperative adult, 1835

postoperative adult, surgery in, 1835

stress echocardiography, 18321834

aortic regurgitation in, 1641, 1641f

cardiac catheterization, indications
for, 1641

computed tomography for, 2062,
2063f

echocardiographic measurements in,
16401641, 1640f

Hoffman's variant, 1635f, 1636f

postoperative evaluation of, 1641
1644, 1642f

with pulmonary stenosis, 1633

velocity vector imaging in, 391392
Thebesian valve, 1503
Thermal index (TI), 111
Thoracic aortic aneurysm (TAA),
19341937

in Marfan syndrome, 1936

natural history of, factors impacts,
1937
Thoracic cage artifacts, 433434, 434f
Thoratec CentriMag system, 1127
Thoratec HeartMate II, 1223f
Thoratec paracorporeal ventricular assist
device, 1227
Three-dimensional echocardiographic
guidance of percutaneous
procedures, 531571. See
also Catheter-based
transcutaneous interventional
procedures
Three-dimensional echocardiography
(3DE), 1418, 240267, 705

advantages/disadvantages of, 262,
267

apical approach, 244, 254f, 255f

artifacts in, 736737. See also
Artifacts

basics of, 7485

beam forming, 7778

color Doppler imaging, 248, 254255,
266f

evolution of, 7475, 75f

examination protocol, 241244,
242f243f

image quality, limitations in

aperture, 80

artifacts, 8081

gating, 80

spatial and temporal resolution, 80,
80f

left parasternal approach, 244,
245f254f

I-XXXIX

multibeat acquisition in, 7475, 75f

in operating room, 577634

aortic valve disease, 582588

cardiac masses, 617627

limitations of 3D TEE and future
directions, 628629

mitral valve disease, 577582

native valve endocarditis, 597604

prosthetic valve dysfunction, 605617

tricuspid valve disease, 589590,
593597

quantification, 8185

left ventricular, 8285, 84f

mitral valve, 8182, 82f, 83f

rendering in, 78, 79f

2D tomographic slices, 7980

surface rendering, 79

volume rendering, 7879, 79f

right parasternal approach, 246, 248,
259f266f

strengths of, 81

subcostal approach, 244, 256f255f

supraclavicular approach, 244,
258f259f

suprasternal approach, 244, 257f

technology related to, 240241,
240f241f

transducer technology and, 76

3D matrix array transducers, 7677,
77f78f

linear/phase array transducers, 76,
76f

single element transducers, 76, 76f

for valvular heart disease, 515528

aortic valve, 520522, 520f

case examples of, 525528

data acquisition, 515516, 516f, 517f

image optimization, 516

mitral valve, 516520, 517f, 518f

pulmonic valve, 522523, 522f, 523f

tricuspid valve, 523525, 524f
Three-dimensional epiaortic
ultrasonography, 641
Three-dimensional intracardiac
echocardiography, 652, 653f
Three-dimensional matrix array
transducers, 7677
Three-dimensional (3D) speckle tracking,
92, 94f95f

frequency, 95

modes, 96, 97f

for morphological study, 94f

one-beat acquisition, 96

raw data storage, 95

I-XL

Comprehensive Textbook of Echocardiography

scan range/volume width, 96

triggered full volume, 96, 97f

volume rate, 96

wall motion tracking, 92, 95, 97f
Three-dimensional speckle tracking and
strain, 715, 717, 717f719f
Three-dimensional speckle tracking
echocardiography (3DSTE),
372373, 373f, 374f, 17271728,
1727f

area strain measurement and, 373,
375, 375f, 375T

clinical applications of, 373374, 376t

use of, 404405, 405f
Three-dimensional stress
echocardiography, 13281336

advantages of, 13291330, 1330t

contraction front mapping in, role of,
13341335

contrast in, 1335

current standards vs., 13311334

adenosine stress test, 13331334

dipyridamole stress test, 1334

dobutamine stress test, 13311333

treadmill exercise stress test, 1333

future directions, 1335

image acquisition in, 13301331

overview, 1328

parametric imaging in, 1334

postacquisition analysis, 1331

stress protocol in, 1331

three-dimensional transducers, 1329,
1329f

vs. 2DSE, in wall visualization, 1334
Three-dimensional transducers, 1329
Three-dimensional transesophageal
echocardiography (3D TEE), 18,
507514

aortic valve display in short-axis view
by, 511f512f

biplane (X-plane) image of left atrial
appendage, 509f

image display recommendations,
511, 511t

image optimization, 510

colors, 510

compression, 510

cropping and rotation, 510

gain, 510

smoothing, 510

imaging modalities in, 509t

full volume, 509

full-volume and live 3D color flow,
509

real time (RT) 3D, 508

simultaneous biplane mode, 509

zoom view, 508

imaging protocol for, 510t

indications for, 508t

procedure for

image acquisition, 508509

imaging sequence, 509510

preprocedural planning, 508

RT3D TEE imaging, advances in,
511512

technology for, 507

uses of, 512

catheter-based LAA closure, 512

congenital heart disease, 513

LAA clot, detection of, 512

left ventricular function and
dyssynchrony assessment, 514

mitral prosthesis paravalvular leak
closure, 512

mitral stenosis and balloon
valvotomy, 513514

mitral valve assessment, 513

transcatheter aortic valve
replacement, 512513

trans-septal puncture, 512
Three-dimensional transthoracic
echocardiography examination,
268286

aortic annulus, 280

aortic regurgitation, 280

data acquisition, methods for

cropping, 269270

3DE color flow Doppler imaging, 269

image display, 269270

multiplane mode, 268269

multiple-beat 3DE imaging, 269

real time 3DE, 269

tomographic slices, 270, 270f

left ventricular assessment

image acquisition methods, 270, 270f

LV regional function, 271272

normal values, 272

volume and systolic function
assessment, 271

mitral regurgitation, 282283, 282f,
283f

mitral stenosis, 280282, 281f

mitral valve assessment, 280, 281f

pediatric and fetal cardiac
pathologies and, 285286

pulmonic valve disease, 284, 284f,
285f

regional LV function, 276

Index

aortic stenosis, 279

aortic valve assessment, 278f, 279,
279f

contractile reserve, 276

left and right atria, 276277, 277f

left ventricle twist, 276

right ventricle, 277278

valvular assessment, 278, 278f

reproducibility, 272273

3D speckle-tracking applications,
275, 275f

3D stress echocardiography, 274275,
274f

global LV function, evaluation of, 276

LV mass, 273274, 273f

methods of validation, 275276

tricuspid valve disease, 283284, 283f,
284f
Three-dimensional TTE, 159161, 161f
Thyroid disorders, 18791880
T2* (star) imaging, 2020
Timeacoustic intensity curve, 442
Time gain compensation (TGC), 61
Tissue Doppler (TD) imaging, 14, 64, 72,
349357, 360, 382

in atrial fibrillation, 353

color TD imaging, 349

development of, 350

diastolic function, assessment of,
352353, 355f, 356f

for LV dyssynchrony, 353, 354f

LV filling pressures, estimation of,
352353

myocardial disease and, 350352

and prognosis, 354355

RV function, assessment of, 354,
356f357f

spectral TD imaging, 349

and technical considerations,
349350, 350f

transthoracic echocardiogram, 137

uses of, 350357
Tissue plasminogen activator (tPA), 1996
Tissue velocity imaging. See Tissue
Doppler
TomTec Cardiac Performance Analysis,
380
Torsion (cardiac motion)

with co-contraction of base and helix,
1190f

compression, 1188

definitions, 11831184

during ejection, 1191f

left ventricle, 11861188, 1186f

longitudinal shortening, 1188

structural reasons for, 1184f

and untwisting, 1200
Total anomalous pulmonary venous
connection (TAPVC), 1577, 1672,
16731678, 1673f, 1674f1675f. See
also Pulmonary veins

anomalous drainage of, 1676

diagnosis steps in, 16731676

echocardiographic goals, 1673

features of, 1673

infracardiac, 1674

pulmonary vein stenosis and, 1678

pulmonary venous confluence,
16731675

septum primum malposition defect
and, 16761678, 1677f
Total anomalous pulmonary venous
return (TAPVR), 1743
Toxic cardiomyopathies, 13961397
adriamycin-induced
cardiomyopathy, 1397f

alcohol-induced cardiomyopathy,
1397
chemotherapy-induced
cardiomyopathy, 13961397
Training, in echocardiography, 750751,
759f, 760f

appropriate use criteria, 758

cardiac sonographers, training of,
753754

certification and maintenance of
proficiency, 758, 759f, 760f

contrast echocardiography and, 757

CT and MRI, training in, 755

duration and sites, 755

fellowship training, 751752, 752t

content of, 754755

level 1 training, 751

level 2 training, 751

level 3 training, 752

intraoperative TEE and, 756757

intravascular and intracardiac
ultrasound and, 757

noncardiologists, training of, 752753

in pediatric echocardiography and
congenital heart disease, 753

special echocardiographic
procedures and, 755

stress echocardiography and, 757

three- and four-dimensional TTE and
TEE and, 757

tissue Doppler and speckle tracking
echocardiography, 757758

training requirements, 751t

transesophageal echocardiography
and, 755, 756t
Transcatheter aortic valve implantation
(TAVI). See Transcatheter aortic
valve replacement (TAVR)
Transcatheter aortic valve replacement
(TAVR), 512513, 540546

AVA calculation, 543

CTA for, 2058f

CT and, 2029t, 20572059

in elderly, 1935f

intra- and postprocedural monitoring
by 2D/3D TEE, 543, 546, 546f

patient seletion for, 543
TAVR valves, 541543, 543f
three-dimensional speckle tracking and,
376t
Transducer probe, 60
Transducers, 58
Transducer technology, 102, 103f
Transesophageal echocardiography
(TEE), 13, 99116, 487

acoustic shadowing, 61, 62f
artifacts

reverberation, 107, 109110, 109f

side lobes, 107, 108f

sound velocity, effect of, 107, 107f,
107t, 108t

of coronary arteries, 13381340

current/future technologies, 112116

and artifacts, 113116, 114f

CMUT technique, 115f, 116, 116f

color Doppler, 113, 113f, 114f

power Doppler, 113, 114f

spectral Doppler, 112113, 113f

image quality

aperture and, 104, 106f

focus and, 106, 106f

frequency and, 104105, 105f

kinds of

dual plane probe, 99100, 100f

matrix array probe, 101102, 101f

miniaturization technology, 102103,
103f, 104f

processing technology, 103, 104f, 105f

real time, three-dimensional TEE,
102

rotary plane probe, 100, 100f, 101f

single plane probe, 99, 99f

transducer technology, 102, 103f

variable plane probe, 100, 100f

pulmonary veins, imaging of, 327

safety considerations, 110

I-XLI

acoustic energy safety, 111

electric safety, 110111

electromagnetic compatibility, 112

heating safety, 111

mechanical safety, 111112

three-chamber view, 60f

training in, 755, 756t
Transesophageal echocardiography,
tricuspid valve

in inferior myocardial infarction,
1014f

three-dimensional, 988990

two-dimensional, 988, 988f, 989f990f

bicaval view, 988f

longitudinal plane examination, 989f

mid-esophageal four-chamber view,
988f

normal tricuspid valve anatomy,
989f990f
Transjugular intrahepatic portosystemic
shunt (TIPS), 319
Transmitral diastolic inflow, 1126f
Transmitter, 60
Transposition of great arteries (TGA),
16531663, 17541763

anatomy, 16531654, 1653f

associated defects, 16571661

coronary arteries, 1661

fixed anatomical obstruction,
16601661

inlet VSD, 1659

left ventricular outflow obstruction,
16591661

septal defects, 16571659

VSD, 16581659

associated lesions, 1653

computed tomography for, 2059
2062, 2061f

coronary artery patterns in, 1661
1663

intramural coronary, 16621663

inverted origin of coronaries, 1662

single left coronary artery, 1662,
1662f1663f

single right coronary artery, 1661

echocardiographic evaluation,
16541656

chamber size, 16551656, 1656f

two-dimensional, 16541655, 1654f,
1655f
Transseptal cardiac catheterization, 233
Transseptal puncture, 512, 532533, 534f
Transthoracic echocardiography (TTE),
132162

I-XLII

Comprehensive Textbook of Echocardiography

apical window, 146, 148

five-chamber plane, 153154, 153f,
154f, 154t

four-chamber plane, 148153,
148f153f, 149t

long-axis or three-chamber plane,
155, 156f

two-chamber plane, 154155, 154f,
155f

beginning of, 132, 135

cardiac structures assessed in,
133t134t

apical window, 133t134t

parasternal window, 133t

of coronary arteries, 13371338

four-chamber view, 60f

imaging modalities

color flow Doppler, 137

M-mode echocardiography, 136, 136f

spectral Doppler, 136137, 137f

tissue Doppler imaging, 137

two-dimensional echocardiography,
135136, 135f

imaging windows and planes, 135

left ventricle and wall motion
determination, 161162, 162f

parasternal window

linear measurements, 139141,
139f141f

parasternal long-axis plane, 137139,
138f, 139f, 139t

parasternal short-axis plane, 144146,
145f147f, 145t

right parasternal long axis view,
141142

right ventricle inflow view, 142, 142f,
142t, 143f

right ventricular outflow tract view,
142144, 143f, 143t, 144f

patient positioning for, 135

pulmonary veins, imaging of, 327,
328f

subcostal window

color and spectral Doppler imaging,
158159

four-chamber view, 155, 156f, 156t,
157f

great vessel imaging, 157158, 158f,
159f

short-axis views, 157, 157f

suprasternal notch window, 159, 160f

three-dimensional, 159161, 161f

of tricuspid valve

three-dimensional, 988990

two-dimensional, 986988, 987f

Transthoracic examination, 164186

apical views

color and spectral Doppler, 176177,
176f

five-chamber view, 178179, 178f,
179f

four-chamber view, 175, 175f178f

left ventricle, 175

long-axis view, 180, 181f, 182

LV and atrium quantitation, 180, 180f,
181f

mitral and tricuspid valves, 175176

two-chamber view, 179, 179f

variations on four-chamber view, 176

parasternal long-axis view, 166171,
167f

aortic valve, aortic root and
ascending aorta, 167168, 168f

calcification, 170

chamber size and function, 167, 168f

Doppler imaging, 170

extracardiac structures, 170

mitral valve, 168169, 169f

M-mode measurements, 169170,
169f, 170f

pulmonary artery long axis, 171

right ventricular inflow view, 171,
171f

parasternal short axis, 172175

aortic valve, 172, 172f

atria, 172173

left ventricle, 174175, 174f

mitral valve, 174, 174f

right ventricular outflow tract,
173174, 173f, 174f

set-up and patient positioning, 164,
166

standard recommended protocol,
165t166t

subcostal views

abdominal aorta and inferior vena
cava, 183, 183f, 184f

four-chamber view, 182, 182f

short-axis views, 182, 183f

suprasternal views, 184185, 185f,
186f
Transvalvular pressure gradients, 1622
Transvalvular regurgitation, 1106
Transverse aortic arch, 1537f
Transverse strain, 362, 363f
TRAP. See Twin reverse arterial perfusion
(TRAP)
Traumatic tricuspid papillary muscle

2d transthoracic echocardiography,
1012f, 1013f

Index

Treadmill exercise stress test, 1333

Treadmill stress echocardiography

equipment and set-up for, 1308f

hyperdynamic response to stress,
1310f

in ischemia, 1311f

vs. coronary angiography, 1312t
Triangle of dysplasia, 1135, 1135f
Tricuspid annular plane systolic
excursion (TAPSE), 177, 178f,
381382, 1069t

longitudinal contraction of RV, 1166

right ventricular ejection fraction
and, 11371138, 1138f

RV systolic function and, 11371138
Tricuspid atresia, 17001701

classification of, 1701t

type I, 1700

type II, 1701
Tricuspid regurgitation (TR), 813816,
9901004

cardiac MRI for, 2013, 2014f

color flow imaging, 814

continuous wave Doppler, 815, 815f

2d transthoracic echocardiography,
992f, 993f996f, 1002f

effect of Nyquist limit, 997f

echocardiography in

three-dimensional, 816

transesophageal, 816

two-dimensional, 814

flow convergence method, 814815

hepatic veins in, 999f

and HV Doppler, 310311, 311f, 312f

measurement of VC, 814

pacemaker associated, 12121214

lead infection associated with,
11141115

time course for, 1213

transthoracic echocardiography for,
12131214, 1213f

parasternal approach for, 999f1000f

PISA method for, 992

pulmonary artery pressure, 815

pulmonary hypertension, systemic
level, 995f

pulsed wave Doppler, 815

rheumatic, 1002f

right heart failure and, 1204

RV dimensions and function,
815816

severity, echocardiography criteria,
993t

severity of, 816, 816t

torrential, 996f

transesophageal echocardiography,
1000f1002f

before and after annuloplasty, 1001f

coronary sinus, 1000f

two jets of, 1001f
Tricuspid regurgitation velocity (TRV),
1063, 1066, 1066f, 1067
Tricuspid stenosis, 812813, 10041007

carcinoid heart disease and, 1006

diet drug-induced valvulopathy, 1006

in elderly female with rheumatic
heart disease, 1006f

grading scales, 1004t

hemodynamically significant,
findings, 1004t

and HV Doppler, 311, 312f

Loefflers syndrome, 10061007

severity of, 813

transthoracic echocardiography

three-dimensional, 1006f

two-dimensional, 1005f1006f
Tricuspid valve (TV), 589590, 1233

in adults

cardiac catheterization, 1815

Ebsteins anomaly, 18131814, 1815t

echocardiography, 1814

MRI/CT for, 18141815

postoperative adult, 18151816

stress echocardiography, 1814

tricuspid valve surgery, 1815

anatomy of, 984, 989f990f

congenital anomalies of, 1022,1616
1618

congenital lesions of, 1616t

congenitally unguarded tricuspid
orifice, 16171618, 1617f

Ebsteins anomaly of, 16161617,
1616f

tricuspid valve prolapse, 1617

3DE assessment of, 278, 278f

3D echo of, 523525, 524f

and dilated cardiomyopathy, 1376

dilated cardiomyopathy and, 1376

flail, 10071029

M-mode echocardiography, 984986

Ebsteins anomaly, 986f

functional events, demonstrating,
984f

septal leaflet, identification, 985f

systolic abnormalities, 986f

using contrast injections,
identification, 985f

pacer and, 1215f

pulsed wave Doppler across, 1533f

three leaflets of, 991f

transesophageal examination

three-dimensional, 988990

two-dimensional, 988, 988f, 989f990f

transthoracic examination

three-dimensional, 988990

two-dimensional, 986988, 987f

ventricular assist devices and, 1232,
1233f
Tricuspid valve annulus, 1474f
Tricuspid valve diseases

anatomy of tricuspid valve, 812

3DE assessment of, 283284, 283f,
284f

tricuspid regurgitation, 813816

color flow imaging, 814

continuous wave Doppler, 815, 815f

3D echocardiography, 816

flow convergence method, 814815

measurement of VC, 814

pulmonary artery pressure, 815

pulsed wave Doppler, 815

role of 3D TEE in operating room in,
589590, 593597, 601f608f

RV dimensions and function,
815816

severity of, 816, 816t

transesophageal echocardiography
in, 816

two-dimensional echocardiography,
814

tricuspid stenosis, 812813

severity of, 813
Tricuspid valve endocarditis, 10081015,
1058f

in adult female, 1017f

MRSA positive, 1020f
Tricuspid valve fibroelastoma, 1023f,
1477f, 1479f
Tricuspid valve myxoma, 1465f
Tricuspid valve prolapse, 10071029, 1617

2d transesophageal
echocardiography, 1010f1011f

in elderly male with dyspnea, 1011f

myxomatous degeneration, 1011f

prosthetic valves and, 1015,
1021f1022f

transthoracic echocardiography

three-dimensional, 1011f

two-dimensional, 1009f1010f
TV endocarditis, 10081015
TV tumors, 1015

I-XLIII

Tricuspid valve prosthesis

bovine pericardial, 1022f

normal functioning porcine, 1021f
Tricuspid valve vegetation

2d transthoracic echocardiography,
1016f, 1018f1019f

in intravenous drug abuser

adult female, 1017f1018f

adult male, 1020f1021f

transesophageal echocardiography

three-dimensional, 1019f

two-dimensional, 1017f1018f, 1019f
Trivial lesions, pressure gradients across,
1574
True biological valves, 10801081
True lumen (TL), 1977f
Truncus arteriosus, 16501653,
1650f1652f

in adults, 18421844

cardiac catheterization, 1844

echocardiography, 1843

MRI/CT for, 1844

postoperative adult, 1844

surgery for, 1844

classification of, 16501652

Doppler imaging, 1653

echocardiography in, 1651f1652f,
1652

two-dimensional, 1653

infant with, 1582f
TRV. See Tricuspid regurgitation velocity
(TRV)
Trypanosoma cruzi, 1875
TTTS. See Twintwin transfusion
syndrome (TTTS)
TUPLE maneuver, 551, 553f
Turbulence, detection of, 66
Twin reverse arterial perfusion (TRAP),
1530
Twintwin transfusion syndrome (TTTS),
15291530
Twisting (cardiac motion), 1183
Two-dimensional echocardiography
(2DE), 89, 705

transthoracic echocardiogram,
135136, 135f

weaknesses of, 81
Two-dimensional (2D) scanning concept,
4f
Two-dimensional (2D) speckle tracking,
88, 88f

acquisition considerations, 88

depth, 89

dynamic range, 90

I-XLIV

Comprehensive Textbook of Echocardiography

frame rate, 8889, 89f

frequency, 90

gain level, 89, 89f

high frame rate, 89

imaging mode, 90, 90f

lateral gain, 8990

raw data format, 90

scan range, 89
Two-dimensional speckle tracking
echocardiography (2D STE),
365367, 365f, 366f

clinical application of, 367, 368t

cardiac transplantation, 371

cardiomyopathies, 369371

chemotherapy cardiotoxicity,
371372

congenital heart disease, 372

coronary artery disease, 367

CRT for heart failure, 369

right ventricular function, 372

rotational dynamics, role of, 372, 372f

valvular heart diseases, 371
Two-dimensional stress
echocardiography, 13281329

vs. 2DSE, in wall visualization, 1334
Two-dimensional subcostal
echocardiography, abdominal
aorta, 222f
Two-dimensional (2D) TEE examination,
480485

esophageal intubation, 481

informed consent for, 480

patient selection for, 480

preparation and conscious sedation,
480

procedure, 481485, 481f485f

bicaval view, 482f

color Doppler interrogation of aortic
valve in long axis, 483f

color Doppler interrogation of aortic
valve in short axis, 484f

color Doppler interrogation of atrial
septum, 482f

color Doppler interrogation of LAA,
482f

color Doppler interrogation of mitral
valve, 483f

color Doppler interrogation of
tricuspid valve, 484f

continuous wave Doppler
interrogation of tricuspid valve,
484f

descending aorta (DA) imaged at 0
and 90, 485f

left atrial appendage, 481f

left ventricular outflow tract view and
aortic valve, 483f

lower esophageal four-chamber view,
484f

mid-esophagus four-chamber view at
0, 481f

mitral valve view at 0 and 90, 483f

mitral valve view at 45 and 135, 483f

pulmonic valve and right ventricular
outflow tract, 484f

pulsed wave Doppler interrogation of
LAA, 482f

pulsed wave Doppler interrogation of
pulmonary vein, 482f

short-axis view of aortic valve, 483f

transgastric long-axis view of left
ventricle, 485f

transgastric short-axis view of left
ventricle, 485f

transgastric short-axis view of right
ventricle, 485f
Two-dimensional tomographic slices,
7980
Two-dimensional transthoracic
echocardiography

subcostal approach

abdominal aorta, 222f223f
Type B arch interruption, 1694
Type II tricuspid atresia, 1701
Type I tricuspid atresia, 1700

U
UCAs. See Ultrasound contrast agents
(UCAs)
Uhls anomaly, 1618, 1618f
Ultraharmonics, 421
Ultrasound

artifacts, 6163

basics of, 5564

definition of, 4

highly focused ultrasound, 1996

history of, 4

imaging by, 5760

A-mode, 57, 58f

B-mode, 57, 58f

M-mode, 58, 58f

resolution, 5860, 60f

transducers and probes, 58, 58f

2D ultrasound, 58, 58f

low-frequency ultrasound, 1996

microbubbles, 1996

nanodroplets, 1996

Index

problems, 1996

therapeutic applications, 1996

diagnostic ultrasound, 1996

tissue plasminogen activator, 1996
Ultrasound contrast agents (UCAs),
416417, 417f, 441. See also
Contrast echocardiography

commercially available, 417, 418t

idle, properties of, 417

safety of, 434435, 435t

ultrasound and, interaction between,
418419, 419f

use of, 428431
Ultrasound frequency, and Doppler
signal, 66
Ultrasound-guided compression therapy
of pseudoaneurysms, 699, 700f
Ultrasound-guided thrombin injection,
699
Ultrasound image, 87
Ultrasound stethoscope, 291. See also
Point-of-care diagnosis
Umbilical artery, pulse Doppler across,
1539f
Umbilical cord vessels, 1539f
Umbilical vein, pulse Doppler across,
1539f
Unicuspid aortic valve, 1619
Univentricular atrioventricular
connections, 16971700
Univentricular heart, in adults, 18451848

cardiac catheterization, 18471848

echocardiography, 18461847

transesophageal, 1847

MRI /CTA for, 1847

tricupsid atresia and post-fontan
adult, 1845, 1846f
Univentricular heart post-fontan
tricuspid atresia, 1848t
Unroofed coronary sinus, 1743
Unscrolled myocardial band model, 1182f
Untwisting (cardiac motion), 1183

during elongation, 1193

mitral valve opening and, 12001201

during postejection isovolumic phase
mirrors torsion, 1189

prominent left-sided vectors in,
11911193

torsion and, 11841185, 1200
Upper transesophageal and
transpharyngeal examination,
487506

arch vessels, identifications of, 488f

bilateral ostial vertebral artery
stenosis, 501f502f

carotid body paraganglioma,
detection of, 500f

Doppler signal of vessels on
transpharyngeal ultrasound, 488t

internal carotid artery stent,
detection of, 499f

left- and right-sided carotid arteries,
494f

left carotid artery stent, detection of,
498f

left carotid bulb and internal carotid
artery stenosis, 496f497f

left internal mammary artery, 493f

left-sided carotid arteries, 489f490f

left subclavian artery, 491f492f

left subclavian artery stenosis and
steal syndrome, 502f503f

left vertebral artery, 490f491f

left vertebral artery origin stenosis,
501f

pan-diastolic backflow in aortic arch
branches and neck vessels,
504f505f

right-sided carotid arteries, 495f, 496f

right subclavian artery, 494f

V
Valsalva aneurysm, sinus of, 16301632
Valsalva maneuver, 11261127
Valve perforation, 1045, 1046f

bicuspid aortic, 1046f

native mitral, 1046f, 1056f
Valvular aortic stenosis, 16181624

aortic root, 1621

aortic valve annulus, 1621

aortic valve area, 1622

associated anomalies in, 1624

critical neonatal aortic stenosis,
16221623

hemodynamics, 1623

severity of, 16211622

sinotubular junction, 1621

stenotic aortic valve, morphology,
16191620

transvalvular pressure gradients,
1622

vs. hypoplastic left ventricle, 1623
Valvular disease, in adults, 18131826

aortic valve, 18211826

mitral valve, 18181820

pulmonary valve, 18161818

tricuspid valve, 18131816

Valvular heart disease, cardiac MRI,

20092013

aortic regurgitation, 20102011, 2011f

aortic stenosis, 2010, 2011f

mitral regurgitation, 20122013

mitral stenosis, 2013

tricuspid regurgitation, 2013, 2014f
Valvular heart disease, 3D echo in,
515528

aortic valve, 520522, 520f

case examples of, 525528

data acquisition, 515516, 516f, 517f

image optimization, 516

mitral valve, 516520, 517f, 518f

pulmonic valve, 522523, 522f, 523f

tricuspid valve, 523525, 524f
Valvular heart diseases, 2D STE and, 371
Valvular regurgitation, 278
Valvular stenosis, 1033
Valvuloarterial impedance (Zva), 1934
Valvuloarterial impedance formula, 911
Variable plane probe, 100, 100f
Vasa vasorum, 450
Vascular rings, 1691
Vasoconstrictors, 451
Vasodilators, 451
Vasodilator stress echocardiography,
13071308
Velocity vector imaging (VVI), 365, 365f,
380406

analysis, 400

for cardiac resynchronization therapy
response, 400401

dyssynchrony in pediatric and
congenital heart disease and, 401

application of

amyloidosis, 399

cardiomyopathy, 394396

congenital heart disease, 391394

congenitally corrected transposition
of great arteries, 392

coronary artery disease, 396398

D transposition of great arteries, 392

exercise and, 400

fetal cardiac function, 390391

heart transplantation, 399400, 399f

Kawasaki disease (KD), 398

myocarditis and, 398399

in patients with diabetes, 398

pulmonary artery hypertension, 399

single ventricle, 392394

systemic right ventricle and, 392

tetralogy of fallot, 391392

cardiac anatomy and, 380381

I-XLV

future directions

LV twist, evaluation of, 406

MRI tagging versus MRI velocity
vector imaging, 405406

three-dimensional STE, 404405, 405f

physics of strain and, 385, 389

and reproducibility and correlation
between vendors, 401404

Vena contracta area (VCA), 278

MR severity and, 869870
Vena contracta technique, 82
Vena contracta width, MR and, 868869
Ventricular arterial connection,
identification of, 15791582

concordant, 15801581

spatial relationship, 1582
Ventricular assist devices, 12221254

apical thrombi, 1230f

cardiac structure and function,
changes in, 12341240

clinical uses of, 12241226

dilated cardiomyopathy

baseline study, 1230f

with small secundum atrial septal
defect, 1232f

echocardiographic evaluation of,
12221254

explantation, 12491250

implantation by device strategy,
1226t

left ventricular over-filling, evidence
of, 12401246

levels of severity, 1225t

list of, 1229t

overview, 12221224

percutaneous continuous flow
devices, 12501252

Impella device, 1250, 1251f

TandemHeart, 12501251

postsurgical evaluation, immediate,
1234

preoperative echocardiographic
evaluation, 12291234

aorta, 1232

aortic valve, 1231, 1231f

atrial septum, 12311232

inferior vena cava, 1233

left atrium, 1231

left ventricle, 12291230

mitral valve, 1231

pericardium, 1231

pulmonic valve, 1232

right ventricle, 12331234

tricuspid valve, 1232, 1233f

I-XLVI

Comprehensive Textbook of Echocardiography

reverse remodeling, 1226

septal contour preleft, 1230f

Thoratec HeartMate II, 1223f

types of, 12261229

long-term axial flow devices, 1227
1228

long-term third generation

centrifugal flow systems, 12281229

short-term circulatory support,
12261227

ventricular size and function,
12341240

diastolic performance of ventricle,
12381240

inlet cannula, 1237, 1240f

left ventricle size and function,
12341237

motion of aortic valve, 1238

right ventricle size and function,
1237f, 1238
Ventricular deptal defect, 1581f
Ventricular morphology,
echocardiography of, 1579
Ventricular myxomas, 14681470
Ventricular papillary muscle rupture,
right

in female with dyspnea, 1014f1015f

transesophageal echocardiogram,
1013f1014f
Ventricular septal contour position, 1248
Ventricular septal defect (VSD), 1361,
1543f, 15911599, 17431746,
18021805

closure of, 557559, 560f

direction of shunt, 1597

Doppler evaluation of, 15961597

color Doppler, 15961597

pulsed and continuous wave Doppler
examination, 1597

3D TEE for, 513

midmuscular, 1544f

M-mode echocardiography, 1598

morphological location, 15911596

doubly committed subarterial
defects, 15941595, 1594f, 1596f

inlet ventricular septal defect,
15951596, 1595f

muscular ventricular septal defects,
15931594, 1593f, 1594f

perimembranous, 15921593, 1592f,
1595f, 1596f

objectives, 1591

pressure gradient across, 15971598

Qp/Qs ratios in, 1598

regurgitation and stenosis, 1598

restriction of, 1647f, 1648

stepwise evaluation for, 1591t

suitability for device closure, 1598
TGA and, 1658

transesophageal echocardiography
in, 1599
Ventricular septal defect closure, ICE
imaging during, 650, 651f
Ventricular septal defects, in adults,
18021805, 1806t

cardiac catheterization, 1805

closure of, 1805

echocardiography, 18041805

inlet, 1804

locations of, 1804f

membranous, 1803

MRI/CTA for, 1805

muscular, 1804

postoperative adult, 1805

supracristal, 1803

venturi effect, 1803f
Ventricular septal dropout, 1543f
Ventricular septal muscle rupture,
12951297

postmyocardial infarction, 1296f

transesophageal echocardiogram,
1013f1014f
Ventricular systolic function, assessment
of, traditional echocardiographic
limitations in, 381382, 381f
Ventricular tachycardia (VT), 1966

apical thrombi, 1966

coronary artery disease, 1966t

dilated cardiomyopathy, 1966t

echocardiography, 1966

hypertrophic cardiomyopathy, 1966t

left ventricle (LV), 1966

noncompaction, 1966t

sarcoid heart disease, 1966t

ventricular arrhythmias, 1966t

arrhythmogenic RV dysplasia
(ARVD), 1966

coronary artery disease, 1966

dilated cardiomyopathy, 1966

hypertrophic cardiomyopathy, 1966

infiltrative diseases, 1966

substrates, 1966

ventricular ectopy, 1966

common conditions, 1966t
Ventriculoarterial discordance, 1580f
Vertebral arteries, assessment of,
691693, 694f
Vijaya's echo criteria, 771, 771t
Visualization ,of received ultrasound
energy, 56, 56f
Volume rendering, of 3DE data set, 7879,
79f
V-ScanTM, 292, 292f
VSD. See Ventricular septal defect (VSD)
VVI. See Velocity vector imaging (VVI)

W
Wall motion abnormalities (WMA)
in DCM, 13711372

detection of, on echocardiogram, 226

in dilated cardiomyopathy, 1371
1372

ischemic, 12911292, 1292f1293f

squatting stress echocardiography in

left ventricular, 13241325

mechanism of, 1325
Wall motion score index (WMSI), 1313f,
1343
Wall motion scores (WMS), 1158
Wall visualization, 3DSE vs. 2DSE in, 1334
Watchman, 563
Wavelength, 55
Wide-angled display, 241
Wilkinss score, 536
William's syndrome, 1628f
WMA. See Wall motion abnormalities
(WMA)
WMS. See Wall motion scores (WMS)
WMSa. See WMS difference (WMSa)
WMS difference (WMSa), 1158

vs. summed difference score, 1159f,
1159t
WMSI. See Wall motion score index
(WMSI)
World Health OrganizationInternational
Society of Hypertension (WHO ISH), 294

Z
Zoom mode, 269