CONGENITAL HEART DISEASER Rukma Juslim,SpJP,FIHASubdep Jantung & Pembuluh DarahRSAL DR. RamelanSurabaya

EPIDEMIOLOGY8 in 1000 live birthsCyanotic & AcyanoticGrown-up Congenital Heart Disease (GUCH) :a. < 20% before surgical correctionb. Now : 85%

CYANOTIC Neonate :TGA (Transposition of the Great Arteries)Tricuspid atresiaObstructed TAPVD ( Total Anomalous Pulmonary Venous Drainage )Severe PSPulmonary atresiaSevere Ebsteins anomaly with ASDHypoplastic left heart

CYANOTICInfant and Older Child :TGATOF

ACYANOTICNeonate :Congenital Aortic StenosisCoarctation + VSD/PDAInfant & Older child :VSD, ASD, PDA, Congenital AS, Coartaction, PS.

SPESIFIC CARDIAC DEFFECTAtrial Septal DefectVentricular Septal DefectAtrioventricular Septal DefectPatent Ductus ArteriosusBicuspid Aortic ValveCoarctation of the AortaEbstein AnomalyTetralogy of FallotEisenmenger Syndrome

ATRIAL SEPTAL DEFECTAnatomy : (Type of ASD)Patent Foramen Ovale (PFO)Ostium primumOstium SecundumSinus Venosus DefectCoronary sinusAV canalIVC defect

ASD

Secundum ASDPrimum ASDAV canalPresentationChild or AdultchildhoodInfancyAppearanceNormalNormalDown syndromeColorNormalNormalCyanosisSignsSecundum ASDAs ASD II MRAs VSDVentricular SeptumIntactIntactVSD componentECGRBBB + RADRBBB + LADRBBB, LAD, Long PR or worsePulmonary Hypertension No No YesMitral ValveUsually normalVarying degrees of MRSevere MR

ASD SecundumClinical manifestation :Right Heart dominantRV failure signAuscultation :Pulmonary systolic ejection murmurFixed splitting S2 & P2 on any phase respiration

ATRIAL SEPTAL DEFECTDiagnostic testing :ECG : RBBB, RAD.Thorax Photo : Cardiomegaly (RA & RV >>), Dilated central pulmonary artery.Echocardiography :Defect diameter, Echo drop, RA & RV dilatation; Qp:QsCatheterization

ATRIAL SEPTAL DEFECTINTERVENTIONAL OPTION :

Device Closure

Surgical Closure

VENTRICULAR SEPTAL DEFECTAnatomy : (Type of VSD )MuscularMembranous /Infracristal /Inlet VSDPosterior (AV Defect) Doubly Committed Sub arterial / outlet VSDInfundibular (Supracristal)

Grades of VSDVery smallSmallModerateLargeEisenmenger syndrome

CLINICAL MANIFESTATION

VERY SMALLSMALLMODE-RATELARGEEISENMENGERMurmur & SiteEarly ejection systolic LSE onlyLoud Pansystolic. LSE to Apex & PA=Small + diastolic murmur at apexPansystolic decresendo to S2. Pulmonary ejection systolicNone at LSE. Ejection systolic PA & PRApexNormalN / LV > slightLV & RV > slightLV & RV >RV >> PA palpableECGNormalNormalLA&LV >LADLA,LV,RV >RA& RV >RADThorax fotoNormalNormalSlight cardiomegalyCardiomegaly, Large PALarge PA

VENTRICULAR SEPTAL DEFECTClinical Manifestation :Small~ AsymptomaticModerate~ dyspnea in adult lifeAuscultation : Holosystolic murmurDiagnostic Testing :ECG : Normal; LAA, LVHThorax Photo : CardiomegalyEchocardiography: Defect diameter, Echo drop, LV dilatation.

VSD Treatment Very small & small : Spontaneous closureModerate : SurgeryLarge : SurgeryEisenmenger syndrome : Medical treatment.

PATENT DUCTUS ARTERIOSUSAnatomy :Ductus fail to close at birth & presents clinical problem.Clinical Manifestation :Silent PDA = AsymptomaticAuscultation :Very small :Continuous machinery murmur ICS II.

PATENT DUCTUS ARTERIOSUSModerate PDA : Collapsing pulse with wide pulse pressure; Thrill systolic and or diastolic at ICS II.

The S2 sound is usually Inaudible

PATENT DUCTUS ARTERIOSUSDiagnostic testing :ECG : Normal, RVH (Large PDA)Thorax Photo : Normal, CardiomegalyEchocardiography : ShuntingIntervention :Ductal deviceSurgery

TETRALOGY FALLOTAnatomy :Non restrictive VSDOverriding Aorta (>50%)Right Ventricular HypertrophyRight Ventricular Outflow Tract (RVOT) obstruction (+ PS )

TETRALOGY FALLOTClinical Manifestation :1.Unoperated : a. Severe RVOT obstruction : central cyanosis & clubbing ; Hypoxic spells b. S1 normal, S2 inaudible2.Palliated : Pulmonal hypertension 3.Complete/Total Repaired : Asymptomatic patients, late symptom : DOE, RV failure.

TETRALOGY FALLOTDiagnostic testing :ECGUnoperated : RVH,RAD, RBBB.Palliated : LVHRepaired : CRBBB after repair

TETRALOGY FALLOTThorax Photo :Unoperated :Boot shaped heartPalliated : Blalock Taussig (increased pulmonary blood flow); Waterston (unilateral pulmonary hypertension)Repaired : cardiomegaly from RV dilatation

TETRALOGY FALLOTInterventional Options :SurgeryResection infundibular stenosis, Transannular patch

InterventionalBalloon dilatation, stent insertion

Thank you for your kind attention

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