Congenital Heart Disease = CHD

~ an Overview
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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 Vessels

Angiogenesis

Proyeksi jantung pd dinding dada

PDA

ASD VSD
RHD

Proyeksi Jantung
Jenis bising :

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

PDA

ASD
VSD RHD

Anatomi Jantung

Listrik Jantung

Listrik Jantung

Listrik Jantung

Listrik Jantung

Atrial Flutter

Aktivitas Listrik Jantung

How Does Heart Work ?

Blood Flow

Jenis Kelainan

Structural mal~alignment Lubang

Jenis Kelainan

Structural mal~alignment
Transposition of great arteries Tetralogy of Fallot Truncus arteriosus Total anomalous pulmonary venous return Atrioventricular canal

Jenis Kelainan

Lubang
Atrial septal defect Ventricular septal defect

Jenis Kelainan

Coarctation Patent ductus arteriosus (PDA) Valves

Stenosis Regurgitation Absence/atresia

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

Arrhythmias

Heart block Supraventricular tachycardia

Transposition of great arteries

Transposition of great arteries

Aortic Stenosis

Tetralogy of Fallot ~ TOF

Clubbing Finger

Hemodinamik

Mengukur JVP

Mengukur JVP

Alfred Blalock

Helen Taussig

Blalock-Taussig Shunt

Modified Blalock-Taussig Shunt

Hi, I Have a Tetralogy of Fallot

Truncus arteriosus

Total anomalous pulmonary venous return

Septal Defect

Atrial septal defect ~ ASD

ASD

Occluder ~ Amplatzer

Occluder pada ASD

ASD Occluder

2 2
3 4

ASD Occluder

ASD Occluder

ASD Occluder

Ventricular septal defect ~ VSD

VSD

Atrioventricular canal

Single Ventricle

Coarctation of aorta

Patent ductus arteriosus ~ PDA

Patent ductus arteriosus ~ PDA

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

PDA
Ao

PA

RV
LV

Management
Indomethacin in premature infant Transcatheter closure/ non surgical closure:
Coil embolization Amplatzer ductal occluder (ADO)

Surgical closure

PDA Neonates/Infants Heart failure (+) Premature Anti failure Indometacin Success Children/Adults Heart failure (-) PH (-) Full term Anti failure Fail Success LR PH (+) RL

Hyperoxia
Non reactive

Fail

Reactive
Age >12wks W >4kg

Spontaneous closure

Surgical ligation

Transcatheter closure

Conservative

Prostaglandin E1

Helps maintain PDA Reasons for PGE1

To increase pulmonary blood flow To allow for mixing (allowing better oxygen delivery) To maintain systemic flow (even though deoxygenated blood)

Prostaglandin contd

Usual dose is 0.03 mcg/kg/min - 0.05 mcg/kg/min Apnea is common - intubation often necessary Fever is side effect

Transcatheter PDA occlusions

Rashkind umbrella device Stainless steel coils Amplatzed ductal occluder

Gianturco coils

Amplatzer ductal occluder

Procedure
AO

PDA
AO

ADO

ADO

Aortogram of PDA

During deploying coil

After deployed of ADO

Tricuspid atresia

Hypoplastic left heart syndrome

Cyanosis vs acyanotic

Cyanosis requires shunting or mixing (except?)

Cyanosis vs acyanotic

Must have

Septal defect (atrial or ventricular) PDA Driving force for right to left shunting

Cyanotic Spell ~ Serangan Sianosis

Evaluation possible congenital heart

ABCs Periksa : frekuensi, irama, impulse, bising, nadi (brachial and femoral) Satura O2 (pre and postductal) BGA Chest xray Echocardiogram

Initial management considerations

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

Increased pulmonary flow (Qp)

ASD VSD TGA Truncus Double outlet right ventricle Atrioventricular canal ~ single ventricle PDA

Decreased pulmonary flow

Right sided obstruction

Pulmonary atresia/stenosis

Tetralogy of Fallot Tricuspid atresia

Decreased systemic flow (Qs)

Left sided obstruction TAPVR with or without obstruction Hypoplastic left heart

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

Bentuk Anomali Dada

Pectus excavatum

Pigeon Chest

Pigeon Chest

Pigeon Chest

Pigeon Chest

Edema paru

Edema paru pd Gagal jantung

CTR = AB : normal < 0,5 CD

Edema paru

Tell me, I forget Show me, I remember Involve me, I understand

-Chinese proverb

Terima Kasih