CONGENITAL

HEART DEFECT

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

Classification of CHD
ACYANOTIC

Normal pulmonary blood flow

Aortic Stenosis (AS)
Coarctatio Aorta (CoA)
Increase pulmonary blood flow
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)

Frequent case !

CYANOTIC
Normal pulmonary blood flow
Transposition of Great Artery (TGA)
tanpa PS
Increase pulmonary blood flow
TGA dengan VSD
Truncus arteriosus
Total anomaly pulmonary vein return
(TAPVR)
Decrease pulmonary blood flow
Tetralogy of Fallot (ToF)
Pulmonary atresia (PA)
Tricuspid atresia (TA)
Eka Gunawijaya

ANAMNESIS any cyanosis?

ACYANOTIC

Ventricular Septal Defect (VSD)

Patent Ductus Arteriosus (PDA)
Atrial Septal Defect (ASD)
Coarctatio Aorta (CoA)
Aortic Stenosis (AS)

Most frequent

SIANOSIS
Tetralogy of Fallot (ToF)
Most frequent
Pulmonal atresia (PA)
Tricuspid atresia (TA)
Transposition of Great Artery (TGA)
Atrio Ventricular Septal Defect (AVSD)
Total Anomaly Pulmonary Veins Return (PAPVR)

Eka Gunawijaya

CYANOSIS (mild to severe) central cyanosis

tongue, gums, oral mucosal
Do not see only in lips

Eka Gunawijaya

CYANOSIS
in fingers

With clubbing fingers

Mild
: SpO2 85-94%
Moderate : SpO2 65-84%
Severe : SpO2 < 65%

Clubbing fingers
look at the nail bed

Normal

Clubbing

Normal

Eka Gunawijaya

Clubbing

Other then cyanosis.

Conjunctiva injection
without secretion

Geographic tongue

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

Cyanosis with heart failure?

Since birth with severe cyanosis :
Pulmonary atresia (PA) Heart failure
Tricuspid atresia (TA) Heart failure
Transposition of Great Artery (TGA) Heart failure
Few months after birth :
Tetralogy of Fallot (ToF)
Atrio Ventricular Septal Defect (AVSD) Heart failure
Truncus arteriosus (TrAo) Heart failure
After infant with mild cyanosis :
Total anomaly pulmonary vein return (TAPVR)

Eka Gunawijaya

Heart failure

Heart failure in Acyanosis CHD

Ventricular 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

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HEART FAILURE
Decrease cardiac performance
Boy
9 mo
BW 5.2 kg

Decrease blood perfusion

Treatment

Minimalize the body need for blood perfusion

decreasing the metabolism:
- Total bed rest
- No pain
- No fever
Manage the systemic problems :
- Treat anemia, infection, electrolyte imbalance
Manage lung problems :
- Oxygenation, manage lung infection
Drugs for heart :
- Anti heart failure

Drugs for heart failure

AFTERLOAD PRESSURE
Aorta pressure
ACE inhibitor
Pulmonal pressure
Sildenafil

PRELOAD VOLUME
Systemic vein
volume
Diuretics
Pulmonary
vein volume
Left
atrium

Right
atrium

Right
ventricle

Left
ventricle

CONTRACTILITY OF
MIOCARD
Ejection fraction
Inotropics

Anti heart failure

Combine diuretics (furosemide + spironolactone)
Potassium (if only use furosemide)
ACE inhibitor (captopril)
Inotropics (dopamine, dobutamine, digoxin)

Eka Gunawijaya

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4 most frequent
- ToF
- VSD, PDA, ASD

4 PATOLOGY

ToF

Pulmonal
artery

Aorta

Left
pulmonal
vein

SVC

LA

1. Pulmonary stenosis (PS) :

- valvular
- infundibular (infravalvular)

2.
Overriding
Aorta

RA

LV
RV

4. Right ventricle
hypertrophy (RVH)

3. Ventricular
septal defect
(VSD)

IVC

ToF is the most frequent of cyanotic CHD (10% of total CHD)

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ASD
Superior
vena cava

NORMAL HEART
Right pulmonal artery

ASD sinus
venosus
ASD secundum
(ASD-II)

Ascenden aorta
Pulmonal
valve

Right
atrium

Right
pulmonal
vein

Right
ventricle

Inferior
vena cava
Descenden aorta

Tricuspid valve

ASD primum
(ASD-I)

VSD

VSD sub arterial doubly

committed (SADC) or
VSD supra cristal

VSD perimembran
inlet

VSD perimembran outlet

(Mostly found)

VSD muscular
posterior

VSD mid muscular

VSD muscular anterior

VSD muscular apical

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)

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Precordial bulging
ToF

ASD

RV hypertrophy

VSD

PDA

Inspection from cranial or caudal

LV hypertrophy
Eka Gunawijaya

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ToF

RV heave

RV hypertrophy

ASD

RV hypertrophy

Eka Gunawijaya

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Cause by hard activity of

Left ventricle (LV)

LV impulse

VSD

LV hypertrophy

PDA

LV hypertrophy
Eka Gunawijaya

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Cause by hard activity of

Left ventricle (LV)

LV lifting

VSD

LV hypertrophy

PDA

LV hypertrophy
Eka Gunawijaya

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Systolic Thrill on Left Sternal Border

Cause by murmur grade IV/6 of

Pulmonal stenosis (in ToF)

No thrill in ASD

Cause by
murmur grade
IV/6 of PDA
Cause by
murmur grade
IV/6 of VSD

Eka Gunawijaya

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AUSCULTATION
ToF

ASD
Upper left sternal border
(ULSB)
or Pulmonal area

Upper left sternal border (ULSB)

Mid left sternal border (MLSB)

Murmur systolic ejection in Upper left sternal border

(ULSB) + Mid left sternal border (MLSB)
More severe the Pulmonal Stenosis (PS) more mild
the murmur gradation

Murmur 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 ULSB

No click

ToF
Upper left sternal border (ULSB)

ASD
Upper left sternal border
(ULSB)
or Pulmonal area

Mid left sternal border (MLSB)

Murmur systolic ejection in Upper left sternal border

(ULSB) + Mid left sternal border (MLSB)
More severe the Pulmonal Stenosis (PS) more mild
the murmur gradation

Murmur 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)

AUSCULTATION
PDA

VSD

Upper left sternal border

(ULSB)
or Pulmonal area
Lower left sternal border
(LLSB)
or Tricuspid area

Apical
(Apex)

Apical
(Apex)

Murmur Hollow systolic or Pan systolic in LLSB,

cause by turbulence blood flow at VSD

Murmur continues (Machinery murmur) in ULSB,

cause by PDA flow along systolic & diastolic

Accentuate P2 sound (A2 < P2)

Accentuate P2 sound (A2 < P2)

Ejection click in ULSB

No click

Murmur Carey Coombs (Mid diastolic apical

murmur) in Apical area

Murmur Carey Coombs (Mid diastolic apical murmur)

in Apical area

PDA

VSD

Upper left sternal border

(ULSB)
or Pulmonal area
Lower left sternal border
(LLSB)
or Tricuspid area

Apical
(Apex)

Apical
(Apex)

Murmur Hollow systolic or Pan systolic in LLSB,

cause by turbulence blood flow at VSD

Murmur continues (Machinery murmur) in ULSB,

cause by PDA flow along systolic & diastolic

Accentuate P2 sound (A2 < P2)

Accentuate P2 sound (A2 < P2)

Ejection click in ULSB

No click

Murmur Carey Coombs (Mid diastolic apical

murmur) in Apical area

Murmur Carey Coombs (Mid diastolic apical murmur)

in Apical area

Chest x-ray ToF

3
PS

RVH

2
Boot-shaped heart
(Couer en sabot)

Chest x-ray Anteroposterior of ToF :

1. Concave pulmonal segment
Boot-shaped heart
2. Lift rounded apex
3 Decrease pulmonal blood flow (oligemic lungs)

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Rontgen toraks PJB asianotik

Convex Pulmonal segment

Increase pulmonal blood flow (pletora lungs)

LA appendage
enlarge (Left
atrial enlarge)

VSD

ASD

PDA
Prominent to right

Cardio Thoracic ratio (CTR)

increase

VSD

CTR increase

CTR increase

ASD

PDA
RAE

LVH

LVH

RVH
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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

31

Monitor the Haemoglobine (Hb) &

Packed cell volume (PCV) or HCT
level

Maintain Haematocrite (HCT) at level 55-65%

If HCT > 65% do Phlebotomy
If 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

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Patomechanism of Cyanotik spell

Shock

Wake up

Spasme
infundibulum
right ventricle

Clinical manifestations :
- become more cyanosis
- panic/irritable
- tachypneu
- letargy convulsion DEAD
SpO2 < 65%

Systemic
vascular pressure

Loud cry

Lung pressure

Right to left
shunt
(cyanosis )
pO2
pCO2
pH

Venous
return

Triggered by : wake up, shock,

loud cry, hard activicty

Tachypneu
Hard activity
33

Knee-chest
position

Management
Cyanotik spell
Shock
Epinefrin

Wake up

Spasme
infundibulum
right ventricle

Propranolol

Venous
return

Systemic
vascular pressure

Lung pressure

Right to left
shunt
(cyanosis )

Morphin
Fentanil

Knee-chest
position

Loud cry

Tachypneu

pO2
pCO2
pH
Sodium
bicarbonate
Hard activity
34

Squatting in child

FINAL MANAGEMNT
SURGERY

TRANS-CATHETER

VSD

PDA

ASD

Eka Gunawijaya

ToF
VSD
ASD
PDA

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