It 3 - Penyakit Jantung Bawaan - Ria

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  • Structures of the heart

  • Normal Heart

  • Atrial Septal defect( ASD )Insidence : + 10 % : ratio = 1,5 to 2 : 1Anatomy : Defect on foramen ovale : Secundum ASD Defect at SVC and RA junction: sinus venosus ASD Defect at ostium primum : primum ASD

  • ANATOMY

  • ASD

  • Atrial Septal DefectDiagram of ASD

  • Clinical FeaturesSymptomsMost infants : asymptomatic ..undetectedThe first present at age 6 to 8 weeks with a soft murmur and possibly a fixed and somewhat widely split S2Infant with large ASD may present with poor growth, recurrent lower respiratory tract infection and heart failure

  • LALVRVRAPAAOSystemicLungsQp > QsAtrial septal defect

  • RARVLALVRARVLALVAtrial septal Defect

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

  • 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(other tipe of ASD)ConservativeAnti failureFailSuccessPH (-)PH (+)PVD (-)PVD (+)HyperoxiaReac-tiveNonreactiveSurgicalClosure

  • Transchateter closure of ASD

  • Atrial septal defect

  • 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

  • VSD

  • SystemicLungsQp > QsVentricular Septal defect

  • LA

    LV

    RV

    RA

    PA

    AO

  • RARVRALALARVLVLVVentricular 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
  • Patent Ductus Arteriosus Anatomy

    Fetus: ductus arteriosus connects PA and aorta

    If ductus does not closs Patent Ductus arteriosus

  • PDA

  • RARVLALVRALARVLVPatent Ductus Arteriosus

  • LALVRVRAPAAOSystemicLungsQp > QsPatent Ductus Arteriosus

  • PDA is more common in : Premature infants BW < 1750 g : 45% BW < 1200 g : 80% Genetic abnormalities Infants whose mother had German measles (Rubella)PDA in preterm haemodynamic instability co-morbidity & mortality EARLY DIAGNOSIS

  • Patent Ductus ArteriosusClinical findings

    Small defect: Symptom (-) Growth and development normalModerate and large defect:Decreased exercise tolerantWeigh gained not goodFrequent URTI

  • DIAGNOSIS

  • Patent Ductus ArteriosusAuscultation : continuosus murmur at upper LSB 2

  • Chest X-RayLarge PDA:Prominence of the left atrium,left ventricle, ascending aorta,Pulmonary vascular marking

  • ECGSmall PDA : normalModerate PDA : LVHLarge PDA : BVHPDA with PVOD : RVH

  • Patent Ductus ArteriosusDiagnosis DifferentialAP-windowArterio-venous fistulae

    Management premature: ibuprofenPDA closure : surgery transcatheter closure

  • MANAGEMENT

    Medical treatment : prostaglandin synthesis inhibitorPreterm neonates : usefullAterm neonates : useless

    Transcatheter closure : mostly choice treatment

    Surgical closure :Infant < 5 kg with large PDAPreterm neonates : medical treatment unsuccessful or contraindicated

  • PDA IN PRETERM NEONATESSpecial problem : haemodynamic instability

    Treatment should be started as soon as PDA suspected Once a significant shunt is present increased pulmonary blood flow damage to premature lungs

    PDA can be closed with prostaglandin synthesis inhibitors

  • TRANSCATHETER CLOSURE *Transcatheter occlusion is effective with a high rate of complete occlusion

    *Complication rare

  • Tetralogy FallotIncidence5-8% from all CHD

    AnatomyCause: Left-anterior deviation of infundibular septum

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

  • Tetralogy Fallot

  • Central cyanosis

  • Central cyanosis

  • PathophysiologyCyanosis is a bluish discoloration of the skin and mucous membranes resulting from an increased concentration of reduced hemoglobinClinical cyanosis occurs when the amount of reduced hemoglobin in the cutaneous vein may result 5 g/100mlThe critical level of reduced hemoglobin in the cutaneous vein may result from either desaturation of arterial blood or increased extraction of oxygen by peripheral tissue

  • Cardiac causes of cyanosisInadequate pulmonary blood flow (severe cyanosis)Tricuspid atresiaPulmonary atresiaTetralogy of Fallot

    Independent pulmonary and systemic circulation (severe cyanosis)Tranpose great artery

    Mixing (moderate cyanosis)Truncus arteriosus

  • Diagnosis

    Clinically : cyanosis Single 2nd HS, ejection systolic murmur

    X Ray : Boot ShapedECG: RAD, RVH

  • Tetralogy FallotSingle 2nd HS, ejection systolic murmur

  • CXR : Boot-shapedConcave pulmonary segmentApex upturnedDecreased pulmonary blood flow

  • Tetralogy FallotECG : RAD, RVHEchocardiography : 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