Dr. dr. Indriwanto S Atmosudigdo, SpJP (K). MARS

Pediatric Cardiology and Congenital Heart Disease Department of Cardiology and Vascular MedicineFaculty of Medicine University of Indonesia

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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

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Knowledge of fetal and perinatal circulation is helpful in understanding the clinical manifestations and natural history of CHD

Fetal Circulation Shunts: 1. Placenta 2. Ductus Venosus 3. Foramen Ovale 4. Dustus Arteriosus

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PULMONARY VASCULAR PRESSURE AND RESISTANCE

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ELECTROCARDIOGRAMADULTNEONATEINFANTRV dominantLV dominant

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HEART AUSCULTATION

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HEART SOUNDS

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HEART MURMURS

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ECHOCARDIOGRAPHY

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ECHOCARDIOGRAPHY

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CARDIAC CATHETERIZATION

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Congenital Heart DiseaseAcyanotic/noncyanoticcyanotic

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Non Cyanotic Left to Right ShuntAtrial Septal DefectVentricle Septal DefectPatent Ductus Arteriosus

Outflow tract Obstruction Pulmonal stenosisAorta stenosis

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Non Cyanotic

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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

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AUSCULTATIONWidely 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 hypertension

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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)

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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)

NONCYANOTIC CHDOUTFLOW TRACT OBSTRUCTIONVENTRICLE OUTFLOW TRACT OBSTRUCTIONWITHOUT SHUNT

Left ventricle outflow tract obstruction

narrow split S2 ejection systolic click harsh ejection systolic murmurAUSCULTATION

asymptomatic symptomatic depend of severity of lesion myocardial function

dyspneuFeeding difficultyFailure to thriveHeart Failure

Syncope painchestSudden death

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NEONATUSduct dependent systemic circulationClosed duktus arteriosus deteriorate systemic circulationhypoperfusion

BABY AND CHILD asymptomatic mild lesion symptomatic : headacheepitasisPulsless

Right ventricle outflow tract obstruction

NEONATUS critical PS duct dependent pulmonary circulationclosed duktus arteriosus severe cyanosis acidosisBABY and CHILD asymptomatic mild lesion symptomatic : Right Heart failureoedemahepatomegalyacitesCyanosis bila ada PFO

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S2 weak ejection systolic click harsh ejection systolic murmurAUSCULTATION

LESI OBSTRUKTIF ALUR KELUAR VENTRIKEL KIRI DAN KANANNeonatus Duct DependentPGE1 sementara dipersiapkan intervensi non-bedah / bedah)

INTERVENSI NON BEDAHGradien tekanan > 40 50 mmHgBalloon Aortic Valvyuloplasty (AS valv)Balloon Pulmonal Valvuloplasty (PS valv)Balloon Angioplasty (CoA)

INTERVENSI BEDAHValvotomy (PS / AS valvar)Reseksi otot (PS / AS subvalvar)Rekonstruksi (PS / AS Supravalvar)

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Oligemic cyanosis spell hypoxia squattingPulmonary Stenosis or Atresia+PFO / ASD / VSD( R L SHUNT ) Tetralogi Fallot PS + PFO / ASD PA + VSD

Less than1 year ( 2 4 month ) minute - hourSpell cyanoticEmergrncySerious complication CVD KEMATIAN knee-chest position Oxigen Sedasion : diazepam or morfin acidosis correction : \ Bic Nat Propranolol BT Shunt/ surgery

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deviasion of infundibulum septum to anterior malrotasi bulbusVSD perimembranusAo overridingPS valvular-infundibularRV hipertrofi

TOTAL CORRECTION > 6 month good size of PA PALIATIF operationBT SHUNT spell hypoxia < 6 month small PA size

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Plethora feeding difficulty Failure to Thrive reccurence RT infection CHFpulmonary Hypertention Increase Pulmonary blood flowTGACOMMON MIXINGPulmonary vascular resistenceCommon Mixing: TAPVD Univentricular Connection Trunkus Arteriosus

atrial : PFO, ASD ventricle : VSD Geart of Arteries: PDAwww.schneiderchildrenhospital.org

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Intervension non surgeryForCongenital Heart Diseases

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Occlusion of Intracardiac and Vascular ShuntsCoil embolization of PDALeft, top: Catheter crosses the PDA from the aortic side and delivers a coil.

Left, bottom: Withdrawal of catheter, leaving coil in PDA

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Occlusion of Intracardiac and Vascular ShuntsAmplatzer Ductal OccludersAmplatzer ductal occluderIllustration courtesy AGA Medical Group Aorta angiogram with device occlusion of PDA, lateral view

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Amplatzer Duct Occluder

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Amplatzer Duct Occluder

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Occlusion of Intracardiac and Vascular ShuntsAmplatzer occlusion of atrial septal defectClockwise from above: Transcatheter delivery of Amplatzer device, which is positioned across the atrial septal defect

Left: Amplatzer device in place

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Occlusion of Intracardiac and Vascular ShuntsDevices for occlusion of the PFO and ASDAbove: Gore Helex septal occluder Illustration courtesy W. L. Gore and Associates Upper left: CardioSEAL occluder Illustration courtesy NMT Medical Lower left: Amplatzer PFO occluder Illustration courtesy AGA Medical Group

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Amplatzer septal occluder

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Amplatzer septal occluder

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Occlusion of Intracardiac and Vascular ShuntsVentricular Septal Defect Occlusion

Above: Echocardiogram of muscular VSD

Upper right: Fluoro image of CardioSEAL device occlusion of a VSD. Transesophageal echo probe (TEE) and pigtail catheter in place.

Lower right: Amplatzer muscular ventricular septal occluder Illustration courtesy AGA Medical Group

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Occlusion of Intracardiac and Vascular ShuntsVSD Occlusion with CardioSEAL Device

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Balloon Pulmonary valvuloplasty

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AngioplastyAortic Coarctation Angioplasty Angiograms showing (left) post-surgical coarctation of the aorta and (right) angioplasty balloon inflated across coarctation site

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AngioplastyAortic Coarctation Angioplasty Illustrations showing (left) uninflated and (right) inflated angioplasty balloon positioned within coarctation of the descending aorta

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Intravascular StentsCoarctation of the AortaLeft: uninflated angioplasty balloon and stent within coarctation Middle: expansion of balloon and stent Right: deflation of balloon leaving stent wide open

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