Kuliah CHD

Burhanuddin IskandarPediatric CardiologyPediatric Department,Medical Faculty, Hasanuddin University/ WS Hospital Makassar

Telur TGAManusia salju/angka 8 TAPVDKarpet Ebstein anomaliSepatu both TF

Structures of the heart

Normal Heart

Atrial Septal defect( ASD )Insidence : + 10 % : ratio = 2 : 1Anatomy : Defect on foramen ovale : Secundum ASD Defect at SVC and RA junction: sinus venosus ASD Defect at ostium primum : primum ASD

Atrial Septal Defect

LALVRVRAPAAOSystemicLungsQp > QsAtrial septal defect

Atrial Septal DefectDiagram of ASD

RARVLALVRARVLALVAtrial septal Defect

Clinical findingsAsymptomaticAuscultation : Normal 1st HS or loudWidely split and fixed 2nd HSEjection systolic murmur Atrial septal Defect

Atrial Septal DefectAuscultation :1st HS N or loudwidely split and fixed 2nd HS Ejection Sistolic Murmur

ECG : IRBB , right ventricular hypertrophyAtrial Septal Defect

Right atrial enlargementProminence the MPA segmentIncreased pulmonary vascular marking Atrial Septal DefectChest X-Ray

Atrial Septal DefectDiagnosis Differential

Primary Atrial Septal DefectECG : LADPartial Anomalous Pulmonary Vein DrainagePulmonary StenosisInnocent Murmur

Atrial Septal defect

ManagementSurgery : Preschool ageRecent treatment: transcatheter closure using ASO (Amplatzer septal occluder)

ASDSmall ShuntLarge ShuntObservationEvaluationAt age 5-8 yrsCathFR1.5ConservativeInfantsChildren/AdultsHeart Failure (-)Heart Failure (+)Age >1yrsW >10kgTranscatheter closure (Secundum ASD) /Surgical Closure(others)ConservativeAnti failureFailSuccessPH (-)PH (+)PVD (-)PVD (+)HyperoxiaReac-tiveNonreactiveSurgicalClosure

Atrial septal defect

Atrial septal defectASD before occlusion

During balloon sizingAtrial septal defect

Atrial septal defectASD after occluded using ASO

Ventricular septal defectInsidence 20 % of all CHD No sex influencedAnatomy Subarterial defect : below pulmonary andaortic valve Perimembranous defect: below aortic valve at pars membranous septum Muscular defect

Ventricular Septal Defect

SystemicLungsQp > QsVentricular Septal defect

LA

LV

RV

RA

PA

AO

RARVRALALARVLVLVVentricular septal defect

Ventricular Septal Defect

Ventricular Septal DefectClinical findingsDay 1st after birth: murmur (-)After 2-6 weeks : murmur (+)Murmur : pansystolic grade 3/6 or higher at LSB 3 Small muscular defect: early systolic murmurSignificant defect: Mid diastolic murmur at apex

Small VSD Large VSD Ventricular Septal DefectMurmur: pansystolic grade 3/6 or higher at LSB 3

Ventricular Septal DefectCardiomegalyApex down wardProminence pulmonary artery segmentIncreased pulmonary vascular marking

Ventricular septal DefectDiagnosis Differential

PDA with PHTetralogy Fallot non cyanoticInoscent murmur

Ventricular septal defectManagement:

Definitive : VSD closure Surgery Transcatheter closure

DSVHeart failure (+)Heart failure (-)Anti failureFailSuccessPABEvaluate in 6 mthsSurgical closure/Transcatheter closureAortic valve prolapsInfundibular stenosisPHSmallerSpontaneousclosureCathPVD(-)PVD(+)CathCathReactiveNon-reactiveConservativeFR>1.5FR

Ventricular septal defectVSD before occlusion

Ventricular septal defectVSD during deploying the device

Ventricular septal defectVSD after occludedusing ASO

Patent Ductus Arteriosus Insidence+ 10%Female : Male = 1.2 to 1.5 : 1Premature and LBW higherAnatomyFetus: ductus arteriosus connects PA and aorta. If ductus does not closs Patent Ductus arteriosus

LALVRVRAPAAOSystemicLungsQp > QsPatent Ductus Arteriosus

RARVLALVRALARVLVPatent Ductus Arteriosus

Patent Ductus ArteriosusClinical findings

Small defect: Symptom (-) Growth and development normalSignificant defect:Decreased exercise tolerantWeigh gained not goodFrequent URTISpecific case: pulsus seler at 4th extremities

Patent Ductus Arteriosus DiagnosisPulsus seler and continuous murmur heard

Patent Ductus ArteriosusChest X- RaySimilar to VSD

Patent Ductus ArteriosusAuscultation : continuosus murmur at upper LSB 2

Diagnosis DifferentialAP-windowArterio-venous fistulae

Management premature: indometasinPDA closure : surgery transcatheter closurePatent Ductus Arteriosus

PDANeonates/InfantsChildren/AdultsHeart failure (+)Heart failure (-)PrematureFull termAnti failureIndometacinSuccessFailSpontaneous closureAnti failureSuccessFailSurgical ligationTranscatheter closurePH (-)PH (+)LRRLHyperoxiaReactiveNonreactiveConservativeAge >12wksW >4kg

Patent Ductus Arteriosus

Patent Ductus ArteriosusPDA before occludedusing ADO

Patent Ductus ArteriosusPDA after occludedusing ADO

Patent Ductus ArteriosusPDA before occludedusing coil

Patent Ductus ArteriosusPDA after occludedusing coil

Pulmonary Stenosis Incidence : 8-10%

Anatomy:Pulmonary stenosis valvular : Bicuspid pulmonary valve Valve leaflet thickening and adhession Pulmonary stenosis infundibular : Hyperthropy infundibulum

Pulmonary Stenosis Clinical findingsValvular stenosis Mild : Ejection systolic Wide 2nd HS ejectiin clickModerate: ejection systolic, early systolic clickSevere : ejecstion systolic, ejection click (-) Stenosis infundibular Ejection click ( - )1st HS normal, 2nd HS weak, ejection systolic Pulmonary stenosis periphery1st & 2nd HS normal, ejection systolic

Pulmonary StenosisMild : ejection systolic 2nd HS wide split ejection clickModerate: ejecsi systolic , early ejection click Severe : ejection systolic, click ejection (-)

Poulmonary StenosisDiagnosisAsymptomatic patient:click systolic (stenosis valvular)systolic murmurwide split 2nd HS vary with respiration

Poulmonary StenosisNormal or mild cardiomegaly Marked pulmonary valve post stenotic dilatationNormal pulmonary vascularity

ECG : RADEchocardiograhhy : confirmation diagnosisCatheterization: increased RV pressure without increased oxygen saturation

Pulmonary Stenosis

Pulmonary StenosisManagement

Medicamentosa : uselessMild stenosis: intervention (-)Moderate stenosis: observationSevere stenosis: balloon valvuloplasty

Pulmonary Stenosis

Pulmonary StenosisBefore ballooning

Pulmonary StenosisDuring ballooning

Pulmonary StenosisAfter ballooning

Coarctation AortaIncidenceIn Western country 5 % of all CHDIn Asian Country incidence lower underdiagnosis ?

AnatomyStenosis at any where in the aorta (from aortic valve to abdominalis aorta)More frequent at ductus arteriosus Botalli and pulmonary artery junction

Coarctation Aorta

Clinical findingsSevere coarctation in neonates period can cause heart failure in 1st weeks of life

Clinical manifestation in children: arterial hypertensioncommonly asymptomatic Different pulses felt at upper and lower extremities

Examination : increased left ventricular activity, thrill systolic, 1st and 2nd HS normal, ejection systolic murmurCoarctation Aorta

Diagnosis Clinically : lower extremities pulses are weakCXR : Mild cardiomegalyProminence of aortic knob Normal pulmonary blood flowECG : normal or LVHEchocardiography: a discrete shelf-like membraneCardiac catheterization and angiography: to confime diagnosisCoarctation Aorta

Management

Neonates : PGE1 to maintain PDA Diuretic Correction acid-base imbalance Prepared to undergo surgery

Big children:Surgery should be done as soon as diagnosis madeBalloon angioplastyCoarctation Aorta

Coarctation Aorta

Coarctation AortaBefore ballooning

Coarctation AortaDuring ballooning

Coarctation AortaAfter ballooning

Tetralogy FallotInsidence5-8% from all CHD

AnatomyCause: Left-anterior deviation of infundibular septum

Sindroma consist of 4 items: VSD pulmonary stenosis aortic over-riding RVH

Tetralogy Fallot

Tetralogy FallotHemodynamic acyanoticHemodynamic cyanotic

Tetralogy FallotDiagnosis

Clinically : cyanosis Single 2nd HS, ejection systolic murmur

Tetralogy FallotSingle 2nd HS, ejection systolic murmur

Tetralogi Fallot

CXR : Boot-shapedConcave pulmonary segmentApex upturnedDecreased pulmonary blood flowTetralogy Fallot

Tetralogy FallotECG : RADEchocardiography : to confirm diagnosis

Tetralogy FallotDiagnosis Differential Pulmonary Atresia Double outlet right ventricle and pulmonary stenosis Transposisi of great arteri and pulmonary stenosis

Management Paliative treatment: Blalock-Taussig shunt Definitive: total correction

Tetralogy of Fallot< 1 yr> 1 yrspell (+)spell (-)propranololfailedsucceedBTStotal correction cathsmall PAgood sized PA clinically ECG CXR echoage 1 yrcathBTS/PDA Stentevaluation

Tetralogy Fallot

Transposition of Great ArteryInsidence5% of CHDAnatomyAbnormality of formation of trunkal septum that cause aorta arising from RV and PA arising from LV

Transposition of Great artery

Fig. 7

Transposition of the great arteries.

Hemodynamic normalHemodynamic of TGAseriesparallelTransposition of Great artery

TGA without VSDIn adequate MixingAdequate Mixing Transposition of Great artery

TGA with large VSDTGA with VSD and PSTransposition of Great artery

Clinical aspects

More frequent in maleBirth weight usually normal or biggerCyanotic vary from mild to severeAuscultation : single 2nd HS and loudMurmur vary from silent to pansystolic murmur or continuous murmurTransposition of Great artery

DiagnosisClinically : Suspicious if neonates presents with cyanotic with birth weight normal or biggerMurmur (-)Single 2nd HS and loudTransposition of Great artery

Murmur (-)Single 2nd HS and loud


Transposition of Great arteryCXR :CardiomegalyEgg-on-side heartIncreased pulmonary vascular marking

Transposition of Great arteryECG :RADRVHBVH Echocardiography : to confirm diagnosisCardiac catheterization: usually is not needed

Diagnosis Differential

trunkus arteriosus trikuspid atresia pulmonary atresia

Management

Surgery: arterial switchPaliative : Blalock-Taussig shuntTransposition of Great artery

Transposition of Great ArteryPGE1VSD(-)VSD(+) 1mth> 1mthCathLV2/3 systLV3 mths3 mthsCathPARI

Truncus ArteriosusInsidencearound 1 % of CHDAnatomy Failure of septation of truncus arteriosus form aorta and pulmonary artery There are 3 type:Type 1 : MPA arises from the truncus and then divides into the RPA and LPATipe 2 : The PAs arise from the posterior aspect of the truncusTipe 3 : The PAs arise from the lateral aspects of the truncusTipe 4: Arteries arising from the descending aorta supply the lungs

Truncus Arteriosus

DiagnosisClinically suspected if:neonates present with cyanotic and single 2nd HSmurmur vary CXR:cardiomegaly increased pulmonary vascular markingECG: biventricular hypertrophyEchocardiografhy: to confirm diagnosisCatheterization: decreased oxygen saturation at right heart and aortaTruncus Arteriosus

Diagnosis Differential Transposisi of great artery Total anomalus pulmonary vein drainage

Management

Medicamentosa : temporarySurgery: Rastelli Palliative: pulmonary artery bandingTruncus Arteriosus

Truncus Arteriosus

Tricuspid AtresiaIncidence1 % from all CHDEmbriologyValve formed at 5th weeksFussion of part of endocardial cushion, ventricular septum and miocardium

AnatomyValve leaflet adhession one to another, difficult to openASD essentially required to drain blood from RA to LA Classified into 2 groupNormal related great arteryTransposed grat arteryTricuspid Atresia

Tricuspid Atresia with normal related great artery

Tricuspid atresia with transposed geat artery

Tricuspid Atresia

Manifestasi klinisCyanosis early after birthIncreased RV activityIncreased LV activityAuscultationSingle 1st and 2 nd HS

Tricuspid Atresia

Clinical manifestationIn almost all patients murmur is silentIf murmur presentDiastolic murmur due to relative MSPansystolic murmur due to VSD

Tricuspid Atresia

Tricuspid Atresia

Diagnosis and diagnosis differentialClinically: Cyanosis with or without murmur

Tricuspid Atresia

CXR: Heart minimally EnlargedThe PVMs are DecreasedThe MPA segment is concaveTricuspid Atresia

ECG: LADLeft ventricular hypertrophyWith or without LAETricuspid Atresia

Echocardiography: Essential to make diagnosisCatheterizationCatheter can not be passed from RA to RVIncreased RA and LA pressureDecreased oxygen saturation in LAAngiography: definitive diagnosisTricuspid Atresia

Diagnosis differentialTransposition of great arteryTruncus arteriosusTetralogy of FallotTotal Anomalous pulmonary vein drainage

Tricuspid Atresia

ManagementFontan operationTricuspid Atresia

Tricuspid Atresia

Modification of Fontan operationTricuspid Atresia

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Kuliah CHD