CONGENITAL HEART .

DISEASES
ASD VSD TOF

- Christina M Joseph
MBBS - 2007

ACYANOTIC
WITHOUT SHUNT

CHD

CYANOTIC

INCREASED PULMONARY BLOOD FLOW - TGV ; TAPVC ; TA with VSD

-TGV, TAPVC , TA with VSD

- MS, MR, AS, AR - Coarctation of aorta - Endocardial fibroelastosis - PS, PR, PHT
WITH SHUNT ASD VSD PDA

DECREASED PULMONARY BLOOD FLOW

Dominant Left Ventricle
TA ; PA/IVS Ebstein anomaly -

Dominant Right Ventricle

with PHT - ASD ; VSD ; ASD ; VSD ; PDA without PHT

TOF
- Dominant RV with PS - TGV with PS - Single ventricle with PS

LEFT TO RIGHT SHUNTS

ASD

VSD
PDA

ATRIAL SEPTAL DEFECT

Abnormal communication between the atria at the

septal level
Usually sufficiently large enough to allow free

communication between the atria , subdivided according to the anatomic location

1. Defect at fossa ovalis ( ostium secundum) Most common 70% ; Atrial septal tissue seperates inferior edge of the defect from the AV valves. Single or multiple Can be large >2cms Females > males 3:1 PAPVC may be associated lesion

2. Partial atrioventricular canal defects 20% of ASDs defects of the AV septum lying inferior to the fossa ovalis ( ostium primum )
Situated in lower part of interatrial septum

Part of AV septal defects

Can have clefts in Mitral and tricuspid valves and minor VSDs

3. Sinus venosus defects 10%

4. Coronary sinus defects

HEMODYNAMICS OF ASD

Leaking of oxygenated blood from L to R atrium

Right atrial enlargement

Tricuspid - delayed diastolic murmur

RV enlargement Prolonged ejection phase Pulmonary ejection murmur ; delayed P2 S2 widely split and fixed ; Cardiac apex formed by enlarged RV ; accentuated P2 also audible

History
Majority considered asymptomatic , Mild diminution

of stamina ; exercise intolerance frequent chest infections in childhood Mild fatigue or dyspnea in late adolescent eartly adult stage 3/4th patients symptomatic as adults CCF rare in childhood ; common 4th-5th decades of life ; associated with onset of arrhythmias

Clinical picture - ASD

Parasternal impulse Systolic thrill may be palbable in 2nd LIS 10% patients

Cardiomegaly- mild- moderate

HEART SOUNDS S1 - Normal or slightly accentuated at left lower sternal border S2 - Widely split and fixed P2 - accentuated and widely transmitted along the left sternal border upto the apex MURMURS Shunt murmur absent Flow murmurs a) TRICUSPID delayed diastolic b) PULMONARY- ejection systolic

INVESTIGATIONS
1. Chest Xray Cardiomegaly Pulmonary plethora ; associated PAPVC
2. ECG Right axis deviation ostium secundum defect rsR in the right precordial leads 90% patients

3. ECHO 4. Cardiac catheterization

RA enlargement Small aorta Pulmonary trunk enlarged with plethora Least likely to lead to Eisenmenger reaction

Natural history
Spontaneous closure

100% closure if <3mm before one and half years 40% before 4 yrs if 3-8mm >8mm unlikely to close Asymptomatic Eisenmengers syndrome at 20-30 years CCF Atrial fibrillation and flutter CNS - paradoxical emboli Infective endocarditis - Rare

TREATMENT
MEDICAL
Few infants CCF- anticongestive theapy ,
infective endocarditis rare antibiotic coverage at

times of possible bacteremcia recommended only if associated mitral valve diseae suspected. SURGICAL

ECG ASD with PAH

Surgery
CLOSURE OF THE ASD All symptomatic patients ; Asymptomatic if Qp:Qs >2:1 Surgery delayed till 3-4 years for spontaneous closure. Mortality <1% with open surgical closure Morbity and mortality increase if sugery is performed late

VENTRICULAR SEPTAL DEFECT

TYPES OF VSD
Most common cardiac malformation (25%)

TYPES
Membranous(80%)

Muscular Trabecular (5-20%) Outlet (supracristal) (5-7%) Inlet ( uncommon )

HEMODYNAMICS of VSD
L to R shunt in systole; pansystolic murmur masks A2

Functional ejection systolic murmur at pulmonary valve

Large volume of blood to lungs Plethora on CXR ; Left atrial enlargement. Mitral delayed diastolic murmur A2- early ; P2 late ; S2 widely split varies with respiration

CLINICAL FEATURES
SMALL VSDS
Asymptomatic
Harsh, loud blowing pansystolic murmur with

thrill 3-4 ICS close to sternum P2 normal

LARGE VSDS
symptomatic from infancy Recurrent respiratory tract infections

Feeding difficulty
Growth failure Profuse perspiration

Features of CCF

EXAMINATION
Cardiomegaly

Precordial bulge with parasternal heave

Precordium hyperkinetic Systolic thrill left sternal border

HEART SOUNDS S1 Masked by the murmur S2 Masked by murmur at LLSB At 2nd LIS widely split but mobile S3 With small LR Shunts MURMURS Shunt murmur - PANSYSTOLIC Flow murmurs a) Pulmonary- EJECTION SYSTOLIC ( drowned ) b) Mitral DELAYED DIASTOLIC

Investigations
Chest Xray
Normal to enlarged heart Increased pulmonary vasculature

ECG

LVH Biventricular hypertrophy Notched peaked p waves 2D Echocardiography with Doppler Cardiac catheterization and angiography

ECG - VSD

VSD
LA - enlargement
Cardiac contour Biventricular enlargement Pulmonary trunk enlarged with plethora CCF often if large

Natural course
Spontaneous closure (30-50%) Small muscular (80%) Perimembranous (35%) Maximum in first year - 40%, 8yrs 90%

Asymptomatic Infective endocarditis (<2%) Large defects recurrent LRIs, CCF PAH leading to Eisenmenger syndrome Infundibular stenosis Protects effects of PAH Shunt decreases

Management
Small VSD No regular treatment Infective endocarditis prophylaxis
Large VSD Control heart failure Prevent development of pulm. Hypertension Prevent growth failure

TREATMENT
Medical Surgical Indicated if

1) CCF in infancy does not respond to medication 2) L R shunt is large (Pulmonar flow more than twice systemic) 3)Associated PS , PAH , AR

Surgery
Pulmonary arterial banding

Complicated VSD Multiple VSDs Very premature infants

Transcatheter closure

Major complications of VSD . surgery

Complete heart block Bifascicular heart block

Reopened or residual VSD

TETRALOGY OF FALLOT
RVOT obstruction (Infundibular stenosis)

VSD (perimembranous, non restrictive)

Septal over-ride of aorta Right ventricular hypertrophy

HEMODYNAMICS of TOF
Degree of RVOT obstruction determines -onset -

cyanosis ,RVH
Severity of cyanosis directly proportional to the severity

of PS ; but the intensity of the systolic murmur is inversely related to the severity of the PS

EXAMINATION
Cyanosis

Not seen in pink TOF ( Acyanotic ) Clubbing Prominent a waves in JVP Normal sized heart Systolic thrill - < 30% patients

HEART SOUNDS S1 Normal S2 Loud Single Only A2 heard P2 soft and inaudible MURMURS Shunt murmur ABSENT Flow murmurs a) Pulmonary- EJECTION SYSTOLIC b) Aortic EJECTION click Continuous murmur Due to collaterals

CLINICAL PICTURE
Dyspnoea on exertion Cyanosis usually starts in 1st year of life

Hypercyanotic spells
Squatting episodes in older children Growth retardation Clubbing in older children

Pathophysiology of cyanotic spell

Increase in R-L shunt due to sudden infundibular . spasm/ decreased systemic vascular resistance Decrease in pO2, Ph ,Increase in pCo2 Hyperapnea Increased cardiac output and heart rate Increase in systemic venous return.Increase in R-L

TOF Course and complications

Failure to thrive Neurological complications

Anoxic infarction in CNS Venous thrombosis hemiplegia Brain abscess Eye congested retina papilloedema Infective Endocarditis CCF - rare

Investigations
Haemoglobin Chest Xray coeur en sabot

ECG

Right axis deviation and RVH Tall peaked p wave ECHO ; Cardiac catheterization

Tetrad
RVOT obstruction VSD RVH Aorta over-riding the interventricular septum Normal sized heart

Management of anoxic spells

Knee chest position Humified Oxygen Morphine 0.2mg/kg Sodium bicarbonate to correct acidosis Propranolol 0.1-0.2mg/kg IV slowly Phenylephrine Infusion Correct anemia Glucose

MEDICAL TREATMENT
PGE1 in newborn period

Prevent and promptly treat dehydration

Treat iron deficiency Oral propranolol 0.5- 1mg/kg every 6 hours

OPERATIVE TREATMENT

PALLIATIVE
Blalock-Taussig shunt Potts shunt Waterstonss shunt

DEFINITIVE
Under CPB Closing the VSD Resecting the infundibular obstruction

SHUNT PROCEDURES
Blalock Taussig subclavian to ipsilateral

pulmonary artery
Waterston ascending aorta to PA
Potts descending Aorta to left branch of PA

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