CONGENITAL HEART DISEASEDr. Poppy Roebiono, SpJP(K)Department of Cardiology and Vascular MedicineFaculty of Medicine University of Indonesia

CONGENITAL HEART DISEASEAnomalies of the heart structure and circulatory function which is present since birth due to disturbances or failure in the development of the heart during early fetal life

Incidence : 8 10 per 1000 live births

IMPORTANT

FETAL AND PERINATAL CIRCULATIONKnowledge of fetal and perinatal circulation is helpful in understanding the clinical manifestations and natural history of CHD

PULMONARY VASCULAR PRESSURE AND RESISTANCE

ELECTROCARDIOGRAMADULTNEONATEINFANTRV dominantLV dominant

HEART AUSCULTATION

HEART SOUNDS

HEART MURMURS

Left to Right Shuntsize of the defectcompliance of RV is greater than LVRA, RV and PA enlargementPulmonary Hypertensionlarge ASD large left to right shuntdevelop in the third to fourth decades of lifePulmonary Vascular Obstructive Diseasebidirectional shunt right to left shunt sianosis EISENMENGER SYNDROMEHEMODYNAMIC

Widely split and fixed S2RV volume overload prolonged RV ejection time delays the closure of the pulmonary valvelarge pulmonary venous return to RA fixed split Systolic ejection murmurnot caused by the shuntoriginates from the increased blood flow passing through the normal-sized pulmonary valve relative PSMid diastolic murmurincreased blood flow through the tricuspid valve relative TSlarge left to right shuntAccentuated P2pulmonary hypertensionAUSCULTATION

RA, RV and PA dilatationprominent pulmonary artery segmentincreased pulmonary vascular marking (plethora)CHEST X-RAY

HEMODYNAMICLeft to Right Shuntsize of the defectlevel of pulmonary vascular resistanceLA, LV and PA enlargementPulmonary Hypertensionlarge VSD large left to right shunthigh pulmonary vascular resistancePulmonary Vascular Obstructive Diseasebidirectional shunt right to left shunt sianosis EISENMENGER SYNDROME

Small VSDnormal P2 intensityholosystolic murmur produced by left to right shuntLarge VSDaccentuated P2 pulmonary hypertensionejection click (occasionally )holosystolic murmur left to right shuntmid diastolic murmur increased blood flow through the mitral valve relative MS Large VSD with Pulmonary Vascular Obstructive Diseaseloud and single S2decreased loudness of the holosystolic murmur (or disappear)AUSCULTATION

CHEST X-RAYLA, LV and PA dilatationprominent pulmonary artery segmentincreased pulmonary vascular marking (plethora)

HEMODYNAMICLeft to Right Shuntsize of the ductus diameter, length and turtuositylevel of pulmonary vascular resistanceLA, LV, ascending Ao and PA enlargementPulmonary Hypertensionlarge PDA large left to right shunthigh pulmonary vascular resistancePulmonary Vascular Obstructive Diseasebidirectional shunt right to left shunt sianosis EISENMENGER SYNDROME

Normal P2 intensitysmall PDA normal PA pressureaccentuated if pulmonary hypertension is presentContinuous (machinery) murmurleft to right shunt occurs throughout the cardiac cyclesignificant pressure gradient between Ao and PA during systole and diastoleApical mid diastolic murmurincreased blood flow through the mitral valve relative MS

Large PDA with Eisenmenger Syndromesingle and loud S2 pulmonary hypertensionno longer continuous murmur ejection systolic murmur

AUSCULTATION

CHEST X-RAYLA, LV, ascending Ao and PA dilatationprominent pulmonary artery segmentincreased pulmonary vascular marking (plethora)

ECHOCARDIOGRAPHY

ECHOCARDIOGRAPHY

CARDIAC CATHETERIZATION