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CONGENITAL HEART

DISEASE
R Rukma Juslim,SpJP,FIHA
Subdep Jantung & Pembuluh
Darah
RSAL DR. Ramelan
Surabaya

EPIDEMIOLOGY
1. 8 in 1000 live births
2. Cyanotic & Acyanotic
3. Grown-up Congenital Heart Disease
(GUCH) :
a. < 20% before surgical
correction
b. Now : 85%

CYANOTIC
Neonate :
1.TGA (Transposition of the Great Arteries)
2.Tricuspid atresia
3.Obstructed TAPVD ( Total Anomalous
Pulmonary Venous Drainage )
4.Severe PS
5.Pulmonary atresia
6.Severe Ebsteins anomaly with ASD
7.Hypoplastic left heart

CYANOTIC
Infant and Older Child :
1.TGA
2.TOF

ACYANOTIC
Neonate :
1.Congenital Aortic Stenosis
2.Coarctation + VSD/PDA
Infant & Older child :
VSD, ASD, PDA, Congenital AS,
Coartaction, PS.

SPESIFIC CARDIAC DEFFECT


Atrial Septal Defect
Ventricular Septal Defect
Atrioventricular Septal Defect
Patent Ductus Arteriosus
Bicuspid Aortic Valve
Coarctation of the Aorta
Ebstein Anomaly
Tetralogy of Fallot
Eisenmenger Syndrome

ATRIAL SEPTAL DEFECT


Anatomy : (Type of ASD)
1. Patent Foramen Ovale (PFO)
2. Ostium primum
3. Ostium Secundum
4. Sinus Venosus Defect
5. Coronary sinus
6. AV canal
7. IVC defect

ASD
Secundum
ASD

Primum ASD

AV canal

Presentation

Child or Adult

childhood

Infancy

Appearance

Normal

Normal

Down syndrome

Color

Normal

Normal

Cyanosis

Signs

Secundum ASD

As ASD II MR

As VSD

Ventricular
Septum

Intact

Intact

VSD component

ECG

RBBB + RAD

RBBB + LAD

RBBB, LAD,
Long PR or
worse

Pulmonary
Hypertension
Mitral Valve

No
Usually normal

No

Yes

Varying degrees Severe MR


of MR

ASD Secundum
Clinical manifestation :
Right Heart dominant
RV failure sign
Auscultation :
Pulmonary systolic ejection murmur
Fixed splitting S2 & P2 on any phase
respiration

ATRIAL SEPTAL DEFECT


Diagnostic testing :
1. ECG : RBBB, RAD.
2. Thorax Photo : Cardiomegaly (RA &
RV >>), Dilated central pulmonary
artery.
3. Echocardiography :Defect diameter,
Echo drop, RA & RV dilatation; Qp:Qs
4. Catheterization

ATRIAL SEPTAL DEFECT


INTERVENTIONAL OPTION :
1. Device Closure
2. Surgical Closure

VENTRICULAR SEPTAL DEFECT


Anatomy : (Type of VSD )
1. Muscular
2. Membranous /Infracristal /Inlet VSD
3. Posterior (AV Defect) Doubly
Committed Sub arterial / outlet VSD
4. Infundibular (Supracristal)

Grades of VSD
1.
2.
3.
4.
5.

Very small
Small
Moderate
Large
Eisenmenger syndrome

CLINICAL MANIFESTATION
VERY
SMALL

SMALL

MODERATE

LARGE

EISENME
NGER

Murmur & Early


Site
ejection
systolic
LSE only

Loud
Pansystoli
c. LSE to
Apex & PA

=Small +
diastolic
murmur
at apex

Pansystoli
c
decresend
o to S2.
Pulmonar
y ejection
systolic

None at
LSE.
Ejection
systolic
PA & PR

Apex

Normal

N / LV >
slight

LV & RV > LV & RV > RV >> PA


slight
palpable

ECG

Normal

Normal

LA&LV >
LAD

LA,LV,RV
>

RA& RV >
RAD

Thorax
foto

Normal

Normal

Slight
cardiome
galy

Cardiome
galy,
Large PA

Large PA

VENTRICULAR SEPTAL DEFECT


Clinical Manifestation :
Small~ Asymptomatic
Moderate~ dyspnea in adult life
Auscultation : Holosystolic murmur
Diagnostic Testing :
1. ECG : Normal; LAA, LVH
2. Thorax Photo : Cardiomegaly
3. Echocardiography: Defect diameter, Echo
drop, LV dilatation.

VSD Treatment
1. Very small & small : Spontaneous
closure
2. Moderate : Surgery
3. Large : Surgery
4. Eisenmenger syndrome : Medical
treatment.

PATENT DUCTUS ARTERIOSUS


Anatomy :
Ductus fail to close at birth &
presents clinical problem.
Clinical Manifestation :
Silent PDA = Asymptomatic
Auscultation :
Very small :Continuous machinery
murmur ICS II.

PATENT DUCTUS
ARTERIOSUS
Moderate PDA : Collapsing pulse with
wide pulse pressure; Thrill systolic
and or diastolic at ICS II.
The S2 sound is usually Inaudible

PATENT DUCTUS ARTERIOSUS


Diagnostic testing :
1. ECG : Normal, RVH (Large PDA)
2. Thorax Photo : Normal, Cardiomegaly
3. Echocardiography : Shunting
Intervention :
1. Ductal device
2. Surgery

TETRALOGY FALLOT
Anatomy :
1. Non restrictive VSD
2. Overriding Aorta (>50%)
3. Right Ventricular Hypertrophy
4. Right Ventricular Outflow Tract
(RVOT) obstruction (+ PS )

TETRALOGY FALLOT
Clinical Manifestation :
1. Unoperated :
a. Severe RVOT obstruction : central
cyanosis & clubbing ; Hypoxic spells
b. S1 normal, S2 inaudible
2. Palliated : Pulmonal hypertension
3. Complete/Total Repaired :
Asymptomatic patients, late symptom :
DOE, RV failure.

TETRALOGY FALLOT
Diagnostic testing :
ECG
1. Unoperated : RVH,RAD, RBBB.
2. Palliated : LVH
3. Repaired : CRBBB after repair

TETRALOGY FALLOT
Thorax Photo :
1. Unoperated :Boot shaped heart
2. Palliated : Blalock Taussig
(increased pulmonary blood flow);
Waterston (unilateral pulmonary
hypertension)
3. Repaired : cardiomegaly from RV
dilatation

TETRALOGY FALLOT
Interventional Options :
Surgery
Resection infundibular stenosis,
Transannular patch
Interventional
Balloon dilatation, stent insertion

Thank you
for your kind
attention