Congenital Heart Disease= CHD

~ an Overviewmtsdarmawandept anak fk uii

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CHD Abbreviations VSD ventricular septal defect ASD atrial septal defect PS pulmonary stenosis AS aortic stenosis HLHS hypoplastic left heart syndrome TAPVC/D totally anomalous pulmonary venous

connection/drainage TGA transposed great arteries PFO patent foramen ovale PDA patent ductus arteriosus CAVC- complete Atrioventricular canal defect (PAPVC-

partial= ostium primum ASD) AV valve- atrioventricular valve (usually mitral or tricuspid)

Congenital Heart Disease

Commonest group of life threatening anomalies

8/1000 live births VSD 30-50% PDA 10% ASD 7%. PS 7% Coarctation 6%, AS 5% Tetralogy 5%, TGA 5% AV canal defects 3%

Penyakit Jantung Bawaan

Pertanyaan penitng :Apakah sianotik ?Apakah ada bising ?Aliran darah paru : normal, ↑ atau ↓ ?Bagian jantung mana saja yang terlibat ?

Blood VesselsBlood Vessels

Angiogenesis Angiogenesis

Proyeksi jantung pd dinding dada

VSD

RHD

ASD

PDA

Proyeksi Jantung

Jenis bising : PDA : kontinue ASD : sistolik VSD : sistolik RHD : pansistolik

RHDVSD

ASD

PDA

Anatomi Jantung

Listrik Jantung

Listrik JantungListrik Jantung

Listrik Jantung

Listrik Jantung

Atrial Flutter

Aktivitas Listrik JantungAktivitas Listrik Jantung

How Does Heart Work ?How Does Heart Work ?

Blood FlowBlood Flow

Jenis KelainanJenis Kelainan

Structural “mal~alignment”Structural “mal~alignment” LubangLubang

Jenis KelainanJenis Kelainan

Structural “mal~alignment”Structural “mal~alignment” Transposition of great arteriesTransposition of great arteries Tetralogy of FallotTetralogy of Fallot Truncus arteriosusTruncus arteriosus Total anomalous pulmonary venous Total anomalous pulmonary venous

returnreturn Atrioventricular canalAtrioventricular canal

Jenis KelainanJenis Kelainan

LubangLubang Atrial septal defectAtrial septal defect Ventricular septal defectVentricular septal defect

Jenis KelainanJenis Kelainan CoarctationCoarctation Patent ductus arteriosus (PDA)Patent ductus arteriosus (PDA) ValvesValves

StenosisStenosis RegurgitationRegurgitation Absence/atresiaAbsence/atresia

Tricuspid (hypoplastic right heart)Tricuspid (hypoplastic right heart) Mitral +/- aortic (hypoplastic left heart)Mitral +/- aortic (hypoplastic left heart)

ArrhythmiasArrhythmias Heart blockHeart block Supraventricular tachycardiaSupraventricular tachycardia

Transposition of great Transposition of great arteriesarteries

Transposition of great Transposition of great arteriesarteries

Aortic Stenosis

Tetralogy of Fallot ~ TOFTetralogy of Fallot ~ TOF

Clubbing Finger

Hemodinamik

Mengukur JVP

Mengukur JVP

Alfred Blalock Helen Taussig

Blalock-Taussig Shunt

Modified Blalock-Taussig Modified Blalock-Taussig ShuntShunt

“Hi, I Have a Tetralogy of Fallot”

Truncus arteriosusTruncus arteriosus

Total anomalous pulmonary venous Total anomalous pulmonary venous returnreturn

Septal Defect

Atrial septal defect ~ Atrial septal defect ~ ASDASD

ASD

Occluder ~ Amplatzer®

Occluder pada ASD

ASD Occluder

1 2

2

34

4

ASD Occluder

ASD Occluder

ASD OccluderASD Occluder

Ventricular septal defect ~ Ventricular septal defect ~ VSDVSD

VSD

Atrioventricular canalAtrioventricular canal

Single Ventricle

Coarctation of aortaCoarctation of aorta

Patent ductus arteriosus ~ Patent ductus arteriosus ~ PDA PDA

Patent ductus arteriosus ~ Patent ductus arteriosus ~ PDAPDA

Normal

Penutupan PDA Normal

Blood Flow pd PDA

Blood Flow pd PDA

Occluder pada PDA

Patent Ductus Arteriosus

-Failure of the ductus to close in the face of increased O2 saturation and drop in PG-abnormal smooth muscle composition

Patent Ductus Arteriosus

Usually causes a left to right shunt

Volume overload of the left heart

Rarely causes severe pulmonary hypertension

Cyanosis, chronic hypoxia, polycythemia

Ao

LV

RV

PA

PDA

ManagementManagement

• Indomethacin in premature infant

• Transcatheter closure/ non surgical closure:– Coil embolization– Amplatzer ductal occluder (ADO)

• Surgical closure

PDA

Neonates/Infants Children/Adults

Heart failure (+) Heart failure (-)

Premature Full term

Anti failureIndometacin

Success Fail

Spontaneous closure

Anti failure

SuccessFail

Surgical ligation

Transcatheter closure

PH (-) PH (+)

LR RL

Hyperoxia

Reactive Nonreactive

Conservative

Age >12wksW >4kg

Prostaglandin E1Prostaglandin E1

Helps maintain PDAHelps maintain PDA Reasons for PGE1Reasons for PGE1

To increase pulmonary blood flowTo increase pulmonary blood flow To allow for mixing (allowing better To allow for mixing (allowing better

oxygen delivery)oxygen delivery) To maintain systemic flow (even though To maintain systemic flow (even though

deoxygenated blood)deoxygenated blood)

Prostaglandin cont’dProstaglandin cont’d

Usual dose is 0.03 mcg/kg/min - 0.05 Usual dose is 0.03 mcg/kg/min - 0.05 mcg/kg/minmcg/kg/min

Apnea is common - intubation often Apnea is common - intubation often necessarynecessary

Fever is side effectFever is side effect

Transcatheter PDA occlusionsTranscatheter PDA occlusions

Rashkind umbrella deviceRashkind umbrella device Stainless steel coilsStainless steel coils Amplatzed ductal occluderAmplatzed ductal occluder

Gianturco coilsGianturco coils

Amplatzer ductal Amplatzer ductal occluderoccluder

ProcedureProcedure

PDA

AO

ADO

ADO

AO

Aortogram of PDA

During deploying coil

After deployed of ADO

Tricuspid atresiaTricuspid atresia

Hypoplastic left heart syndrome

Cyanosis vs acyanoticCyanosis vs acyanotic

Cyanosis requires Cyanosis requires shunting or mixing shunting or mixing (except…?)(except…?)

Cyanosis vs acyanoticCyanosis vs acyanotic

Must haveMust have Septal defect (atrial or Septal defect (atrial or

ventricular)ventricular) PDAPDA Driving force for right to Driving force for right to

left shuntingleft shunting

Cyanotic Spell ~ Serangan Sianosis

Evaluation possible congenital Evaluation possible congenital heartheart

ABC’sABC’s Periksa : frekuensi, irama, impulse, Periksa : frekuensi, irama, impulse,

bising, nadi (brachial and femoral)bising, nadi (brachial and femoral) Satura O2 (pre and postductal)Satura O2 (pre and postductal) BGABGA Chest xrayChest xray EchocardiogramEchocardiogram

Initial management Initial management considerationsconsiderations

Too much pulmonary blood flow?Too much pulmonary blood flow? Too little pulmonary blood flow?Too little pulmonary blood flow? Too little systemic flow?Too little systemic flow? Mixing?Mixing?

Increased pulmonary flow Increased pulmonary flow (Qp)(Qp)

ASDASD VSDVSD TGATGA TruncusTruncus Double outlet right ventricleDouble outlet right ventricle Atrioventricular canal ~ single Atrioventricular canal ~ single

ventricleventricle PDAPDA

Decreased pulmonary Decreased pulmonary flowflow

Right sided obstructionRight sided obstruction Pulmonary atresia/stenosisPulmonary atresia/stenosis

Tetralogy of FallotTetralogy of Fallot Tricuspid atresiaTricuspid atresia

Decreased systemic flow Decreased systemic flow (Qs)(Qs)

Left sided obstructionLeft sided obstruction TAPVR with or without obstructionTAPVR with or without obstruction Hypoplastic left heart Hypoplastic left heart

Mitral atresia, aortic atresia, Mitral atresia, aortic atresia, interrupted aortic arch, coarctation of interrupted aortic arch, coarctation of aortaaorta

Bentuk Anomali Dada

Pectus excavatum

Pigeon Chest

Pigeon Chest

Pigeon Chest

Pigeon Chest

CTR = AB : normal < 0,5 CD

Edema paru pdGagal jantung

Edema paru

Edema paru

Tell me, I forget…Show me, I remember…

Involve me, I understand… -Chinese proverb

Tell me, I forget…Show me, I remember…

Involve me, I understand… -Chinese proverb

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