9/9/2014 Ostium Secundum Atrial Septal Defects

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Ostium Secundum Atrial Septal Defects
Author: Ira H Gessner, MD; Chief Editor: Steven R Neish, MD, SM more...
Updated: Jun 17, 2013
Background
An ostium secundum atrial septal defect is an abnormally large opening in the atrial septum at the site of the foramen
ovale and the ostium secundum.
Embryology
In the early embryo heart, the atria comprise a common chamber. As the atria enlarge, the septum primum forms
and grows toward the developing atrioventricular canal area, which is later divided by the superior and inferior
endocardial cushions. These cushions fuse and bend with their convexity toward the atria, thereby approaching the
down-growing septum primum. This process continually narrows the passageway between the atria, which is then
defined as the ostium primum.
[1]
The ostium primum completely closes; however, before this occurs, a central perforation appears in septum primum,
allowing continuous unrestricted flow from the right atrium to the left atrium. This perforation, the second opening in
the septum primum, is called ostium secundum. As the atria expand to either side of the truncus arteriosus, a fold is
produced within the atria just to the right of septum primum. This passively formed fold is septum secundum. The
leading edge of septum secundum is concave in shape and is called the foramen ovale. It comes to overlay the
ostium secundum but does not interfere with blood flow from right to left through ostium secundum. After birth, with
onset of pulmonary blood flow and elevation of left atrial pressure, the septum primum is pushed against the septum
secundum, effectively closing the ostium secundum.
Fusion of the septum primum and the septum secundum closes the foramen ovale. Complete closure occurs in most
individuals. In 25-30% of normal adult hearts, however, a probe can be passed from the right atrium to the left atrium
via the foramen ovale and ostium secundum. This patent foramen ovale allows a tiny left-to-right shunt that can be
detected by sensitive techniques, such as color Doppler echocardiography, in 15-20% of adults.
Anatomy
A secundum atrial septal defect is usually bordered by the edge of the fossa ovalis and the exposed circumference
of ostium secundum. The shape of the defect varies from circular to oval. Less often, strands of tissue cross the
defect creating a fenestrated appearance that suggests multiple defects. Rarely, a defect can extend posteriorly and
inferiorly, approaching the site of inferior vena cava entrance into the right atrium.
[2]
Pathophysiology
A secundum atrial septal defect can result from inadequate formation of the septum secundum so that it does not
completely cover the ostium secundum. More often, the ostium secundum is excessively large because of increased
resorption so that septum secundum cannot cover it.
Natural history
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Although heart failure from secundum atrial septal defect rarely occurs in children, this complication can often occur
in adults. Adults also demonstrate a propensity for atrial dysrhythmias, including atrial flutter and fibrillation,
presumably caused by chronic right atrial dilation. These complications may not be reversible if closure is delayed.
Data indicate that closure in persons older than 40 years does not reduce the risk of atrial dysrhythmia. Spontaneous
closure of a small secundum atrial septal defect does occur and is usually documented when the initial diagnosis is
made early in infancy.
[3, 4]
A moderate atrial septal defect may also decrease significantly in size or even close when
the defect is diagnosed early in life.
[5]
However, defects diagnosed later in childhood seldom decrease in size and
many significantly increase in size.
[6]
The foramen ovale mechanism remains patent in at least 15% of adults (echocardiography can identify clinically
insignificant shunts with this frequency). Some of these individuals could be classified as having a small secundum
atrial septal defect. The presence of this phenomenon has been identified as a potential risk factor for stroke due to
embolization into the systemic arterial circulation.
[7]
Although unusual, obstructive pulmonary vascular disease may occur in adults with an atrial septal defect.
[8]
Epidemiology
Frequency
International
Isolated secundum atrial septal defects account for approximately 7% of congenital cardiac defects. Congenital heart
defects of significance occur in approximately 8 per 1000 live births. Therefore, 5-6 cases of secundum atrial septal
defect occur per 10,000 live births. This number refers only to defects that are large enough to come to clinical
attention. Many small defects that remain undetected occur in addition to numerous cases of patent foramen ovale,
as mentioned above.
Mortality/Morbidity
An isolated secundum atrial septal defect very seldom causes significant symptoms in pediatric patients, regardless
of defect size.
Rarely, an infant may develop congestive heart failure in the presence of a secundum atrial septal defect.
Whether the defect alone is responsible for causing heart failure is not well established, although it certainly
adds to the patient's hemodynamic difficulties. Failure to thrive caused only by a secundum atrial septal
defect is, similarly, a rare occurrence.
Bacterial endocarditis is not a risk with this lesion and the American Heart Association does not advise use of
antibiotic prophylaxis.
No data indicate that an uncomplicated secundum atrial septal defect can cause pulmonary vascular disease
in pediatric patients.
A patient with primary pulmonary hypertension may demonstrate a secundum atrial septal defect, but no
cause-and-effect relationship can be established.
Patients with a significant secundum atrial septal defect who live at an altitude higher than 4000 feet may
incur a small risk of developing pulmonary vascular disease.
Death from an isolated secundum atrial septal defect does not occur in pediatric patients.
Race
No significant racial influences have been identified.
Sex
Secundum atrial septal defect occurs more commonly in females than males, with a female-to-male ratio of 2:1.
Age
Secundum atrial septal defect is a congenital abnormality and, therefore, is present at birth. It may be diagnosed at
any age, including early infancy, although the characteristic findings on physical examination often escape detection
9/9/2014 Ostium Secundum Atrial Septal Defects
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until the patient reaches a few years of age. An acquired defect occurs only iatrogenically.
Contributor Information and Disclosures
Author
Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine
Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics , American
College of Cardiology , American Heart Association , American Pediatric Society , and Society for Pediatric
Research
Disclosure: Nothing to disclose.
Specialty Editor Board
Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical
Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas
Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics , American
College of Cardiology , American Heart Association , Arkansas Medical Society , International Society for Heart
and Lung Transplantation , and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of
Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Alvin J Chin, MD Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician,
Cardiology Division, Children's Hospital of Philadelphia
Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of
Science , American Heart Association , and Society for Developmental Biology
Disclosure: Nothing to disclose.
Gilbert Z Herzberg, MD Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New
York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.
Chief Editor
Steven R Neish, MD, SM Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department
of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics ,
American College of Cardiology , and American Heart Association
Disclosure: Nothing to disclose.
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