CONGENITAL HEART DEFECT(CHD)Dr. Eka Gunawijaya, Sp.A(K) - Bagian Ilmu Kesehatan Anak FK UNUD - Pelayanan Jantung Terpadu RSUP Sanglah Dps

Eka Gunawijaya

Classification of CHD ACYANOTICNormal pulmonary blood flowTransposition of Great Artery (TGA) tanpa PS

Increase pulmonary blood flowTGA dengan VSDTruncus arteriosusTotal anomaly pulmonary vein return (TAPVR)

Decrease pulmonary blood flowTetralogy of Fallot (ToF)Pulmonary atresia (PA)Tricuspid atresia (TA)Normal pulmonary blood flowAortic Stenosis (AS)Coarctatio Aorta (CoA)

Increase pulmonary blood flowPatent Ductus Arteriosus (PDA)Atrial Septal Defect (ASD)Ventricular Septal Defect (VSD)

CYANOTIC*Eka GunawijayaFrequent case !

Eka Gunawijaya

ANAMNESIS any cyanosis?ACYANOTICTetralogy of Fallot (ToF)Pulmonal atresia (PA)Tricuspid atresia (TA)Transposition of Great Artery (TGA)Atrio Ventricular Septal Defect (AVSD)Total Anomaly Pulmonary Veins Return (PAPVR)Ventricular Septal Defect (VSD)Patent Ductus Arteriosus (PDA)Atrial Septal Defect (ASD)Coarctatio Aorta (CoA) Aortic Stenosis (AS)

SIANOSIS*Eka Gunawijaya Most frequent Most frequent

Eka Gunawijaya

CYANOSIS (mild to severe) central cyanosis tongue, gums, oral mucosalDo not see only in lips*Eka Gunawijaya

Eka Gunawijaya

With clubbing fingers*CYANOSIS in fingersMild : SpO2 85-94%Moderate : SpO2 65-84%Severe : SpO2 < 65%

Clubbing fingersClubbingNormalNormalClubbing look at the nail bed*Eka Gunawijaya

Eka Gunawijaya

Conjunctiva injection without secretion*Geographic tongueOther then cyanosis.

When cyanosis?Since birth with severe cyanosis :Pulmonary atresia (PA)Tricuspid atresia (TA)Transposition of Great Artery (TGA)

Few months after birth :Tetralogy of Fallot (ToF)Atrio Ventricular Septal Defect (AVSD)Truncus arteriosus (TrAo)

After infant with mild cyanosis :Total anomaly pulmonary vein return (TAPVR)

*Eka Gunawijaya

Eka Gunawijaya

Cyanosis with heart failure?Since birth with severe cyanosis :Pulmonary atresia (PA)Tricuspid atresia (TA)Transposition of Great Artery (TGA)

Few months after birth :Tetralogy of Fallot (ToF)Atrio Ventricular Septal Defect (AVSD)Truncus arteriosus (TrAo)

After infant with mild cyanosis :Total anomaly pulmonary vein return (TAPVR)

*Eka Gunawijaya Heart failure Heart failure Heart failure Heart failure Heart failure Heart failure

Eka Gunawijaya

Heart failure in Acyanosis CHDVentricular Septal Defect (VSD)- Small defect asymptomatic, no heart failure- Moderate defect heart failure since 6 months old- Large defect heart failure since neonate to 3 months old

Patent Ductus Arteriosus (PDA)- Small defect asymptomatic, no heart failure- Moderate defect heart failure since 6 months old- Large defect heart failure since neonate to 3 months old

Atrial Septal Defect (ASD)- Small defect asymptomatic, no heart failure- Moderate defect heart failure when adult- Large defect heart failure when teen*Eka Gunawijaya

Eka Gunawijaya

HEART FAILUREDecrease cardiac performance

Decrease blood perfusion

Boy9 moBW 5.2 kg

TreatmentMinimalize the body need for blood perfusion decreasing the metabolism:- Total bed rest- No pain- No feverManage the systemic problems :- Treat anemia, infection, electrolyte imbalanceManage lung problems :- Oxygenation, manage lung infectionDrugs for heart :- Anti heart failure

Drugs for heart failureRight atrium Right ventricle

Left ventricle AFTERLOAD PRESSUREAorta pressure ACE inhibitorPulmonal pressure SildenafilPRELOAD VOLUMESystemic vein volume DiureticsPulmonary vein volumeLeft atrium CONTRACTILITY OF MIOCARDEjection fraction Inotropics

Anti heart failureCombine diuretics (furosemide + spironolactone)Potassium (if only use furosemide)ACE inhibitor (captopril)Inotropics (dopamine, dobutamine, digoxin)*Eka Gunawijaya

Eka Gunawijaya

4 most frequent- ToF- VSD, PDA, ASD

Eka Gunawijaya

ToF2. Overriding Aorta4. Right ventricle hypertrophy (RVH)1. Pulmonary stenosis (PS) : - valvular - infundibular (infravalvular)3. Ventricular septal defect (VSD)4 PATOLOGY RVRALALVAortaPulmonal arteryLeft pulmonal veinSVCIVC*ToF is the most frequent of cyanotic CHD (10% of total CHD)

ASDSuperior vena cavaInferior vena cava Right atriumRight ventricleNORMAL HEARTAscenden aortaRight pulmonal arteryRight pulmonal veinPulmonal valveASD sinus venosusASD secundum (ASD-II)ASD primum (ASD-I)Tricuspid valveDescenden aorta

VSD sub arterial doubly committed (SADC) or VSD supra cristalVSD perimembran inletVSD perimembran outlet(Mostly found)VSD mid muscularVSD muscular anteriorVSD muscular apicalVSD muscular posteriorVSD

Patent Ductus Arteriosus (PDA)Ductus arteriosus (DA) not spontaneously closed at birth be a PDA :Blood flow from Aorta descenden PDA Pulmonal artery(Left to Right shunt)*

Precordial bulging*Eka GunawijayaRV hypertrophyInspection from cranial or caudalLV hypertrophyToFASDVSDPDA

Eka Gunawijaya

RV heave*Eka GunawijayaRV hypertrophyToFASDRV hypertrophy

Eka Gunawijaya

*Eka GunawijayaCause by hard activity of Left ventricle (LV)LV impulseVSDPDALV hypertrophyLV hypertrophy

Eka Gunawijaya

*Eka GunawijayaCause by hard activity of Left ventricle (LV)LV liftingVSDPDALV hypertrophyLV hypertrophy

Eka Gunawijaya

Systolic Thrill on Left Sternal Border*Eka GunawijayaCause by murmur grade IV/6 of Pulmonal stenosis (in ToF)No thrill in ASDCause by murmur grade IV/6 of VSDCause by murmur grade IV/6 of PDA

Eka Gunawijaya

AUSCULTATIONUpper left sternal border (ULSB)

Mid left sternal border (MLSB)ToFUpper left sternal border (ULSB) or Pulmonal areaASD

Murmur systolic ejection in Upper left sternal border (ULSB) + Mid left sternal border (MLSB)More severe the Pulmonal Stenosis (PS) more mild the murmur gradationMurmur systolic ejection (Pulmonal stenosis relative) in ULSB

Heart sound 2 (S2) single (without P2 sound), or P2 sound is weak (A2 > P2)Wide Fixed Splitting of heart sound 2 (S2 sound)Ejection click of severe PS in ULSBNo click

Upper left sternal border (ULSB)

Mid left sternal border (MLSB)ToFUpper left sternal border (ULSB) or Pulmonal areaASD

Murmur systolic ejection in Upper left sternal border (ULSB) + Mid left sternal border (MLSB)More severe the Pulmonal Stenosis (PS) more mild the murmur gradationMurmur systolic ejection (Pulmonal stenosis relative) in ULSB

Heart sound 2 (S2) single (without P2 sound), or P2 sound is weak (A2 > P2)Wide Fixed Splitting of heart sound 2 (S2 sound)

AUSCULTATIONUpper left sternal border (ULSB) or Pulmonal areaVSDLower left sternal border (LLSB) or Tricuspid areaPDAApical (Apex)Apical (Apex)

Murmur Hollow systolic or Pan systolic in LLSB, cause by turbulence blood flow at VSDMurmur continues (Machinery murmur) in ULSB, cause by PDA flow along systolic & diastolicAccentuate P2 sound (A2 < P2) Accentuate P2 sound (A2 < P2) Ejection click in ULSBNo clickMurmur Carey Coombs (Mid diastolic apical murmur) in Apical areaMurmur Carey Coombs (Mid diastolic apical murmur) in Apical area

Upper left sternal border (ULSB) or Pulmonal areaVSDLower left sternal border (LLSB) or Tricuspid areaPDAApical (Apex)Apical (Apex)

Murmur Hollow systolic or Pan systolic in LLSB, cause by turbulence blood flow at VSDMurmur continues (Machinery murmur) in ULSB, cause by PDA flow along systolic & diastolicAccentuate P2 sound (A2 < P2) Accentuate P2 sound (A2 < P2) Ejection click in ULSBNo clickMurmur Carey Coombs (Mid diastolic apical murmur) in Apical areaMurmur Carey Coombs (Mid diastolic apical murmur) in Apical area

12Chest x-ray Anteroposterior of ToF :1. Concave pulmonal segment2. Lift rounded apex3 Decrease pulmonal blood flow (oligemic lungs)Boot-shaped heart(Couer en sabot)33Boot-shaped heartRVHPSChest x-ray ToF*

Rontgen toraks PJB asianotikCTR increaseIncrease pulmonal blood flow (pletora lungs)Convex Pulmonal segmentLA appendage enlarge (Left atrial enlarge)LVH *Prominent to rightRAERVHCardio Thoracic ratio (CTR) increaseCTR increaseVSDPDAASDVSDPDAASDLVH

Emergency in cyanotic CHD

Cyanotic spell, or Hypoxic spell, or Tet spell (commonly found in ToF)Chronic Hipoxia erithropoisis Policytemia or increasing haematocrite (HCT) increase blood viscosity slow blood flow Thrombosis Brain abscess (sterile or infective)Thrombosis decreasing clothing factor & thrombocyte consumptive coagulopathy Massive bleeding*Eka Gunawijaya

Eka Gunawijaya

Monitor the Haemoglobine (Hb) & Packed cell volume (PCV) or HCT level

Maintain Haematocrite (HCT) at level 55-65%If HCT > 65% do PhlebotomyIf Hb < 15 g% : anemia absolute do transfussion with Packed red cell (PRC)If HCT > 3x Hb : anemia relative give hematinic drug (iron supplement)*Eka Gunawijaya

Eka Gunawijaya

Patomechanism of Cyanotik spell Right to left shunt (cyanosis )Tachypneu Venous return pO2 pCO2 pHSpasme infundibulum right ventricle Systemic vascular pressureLoud cry Lung pressureWake up*Clinical manifestations :- become more cyanosis- panic/irritable- tachypneu- letargy convulsion DEADSpO2 < 65%Triggered by : wake up, shock, loud cry, hard activicty

Hard activityShock

Right to left shunt (cyanosis )Tachypneu Venous return pO2 pCO2 pHSpasme infundibulum right ventricle Systemic vascular pressureLoud cry Lung pressureWake up*Hard activityShockManagement Cyanotik spellMorphinFentanilSodium bicarbonateKnee-chest positionEpinefrinPropranololKnee-chest positionSquatting in child

FINAL MANAGEMNT*Eka GunawijayaTRANS-CATHETERSURGERYVSDPDAASDToF VSD ASD PDA

Eka Gunawijaya

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