dr.

Indriwanto S Atmosudigdo, SpJP (K)

Pediatric Cardiology and Congenital Heart Disease
Department of Cardiology and Vascular Medicine
Faculty of Medicine University of Indonesia

CONGENITAL HEART
DISEASE
Anomalies of the heart structure and

circulatory function which is present since

birth due to disturbances or failure in the
development of the heart during early fetal
life
Incidence : 8 10 per 1000 live births

History
Physical Examination
ECG
Chest X-Ray
Echocardiography
Cardiac Catheterization

UNDERSTAND
CARDIOVASCULAR
ANATOMY
PHYSIOLOGY
PATHOPHYSIOLOGY

NON
NON SURGICAL
SURGICAL
AND
AND
SURGICAL
SURGICAL
INTERVENTION
INTERVENTION

Knowledge of fetal and perinatal circulation is helpful in

understanding the clinical manifestations and natural history of CHD

CHANGES IN CIRCULATION
AFTER BIRTH
Shift of blood flow for gas exchange from placenta to the lungs
1. Interruption of the umbilical cord
Increase of SVR
Closure of ductus venosus
2. Lung expansion
Reduction of PVR
Functional closure of PFO
Closure of PDA

Fetal
Circulation
Shunts:
1. Placenta
2. Ductus Venosus
3. Foramen Ovale
4. Dustus
Arteriosus

Normal
Post-Natal
Circulation

PULMONARY VASCULAR PRESSURE

AND RESISTANCE

ELECTROCARDIOGR
AM
NEONATE

RV dominant

INFANT

ADULT

LV dominant

HEART AUSCULTATION
HEART SOUNDS AND MURMURS
vibrations produced by cessation or
propulsion of blood within the heart
radiates through the thorax skin
stethoscope
evaluation of heart disease

HEART SOUNDS
First Heart Sound (S1)
closure of the Mitral and Tricuspid valves

Second Heart Sound (S2)

closure of the Aortic and Pulmonary Valves
splitting of the S2 A2 procedes P2
splits with respiration

Third Heart Sound (S3)

early in diastole
rapid filling phase of the ventricle
heard in normal children

Fourth Heart Sound (S4)

atrial contraction
rare in children

HEART MURMURS
Turbulent blood flow
produces vibrations
stenosis
regurgitation
shunt

Classification
timing during cardiac cycle
- systolic murmur
- diastolic murmur
- continuous murmur

ECHOCARDIOGRAPHY

ECHOCARDIOGRAPH
Y

CARDIAC
CATHETERIZATION

Congenital Heart Disease

Acyanotic/noncyanotic

cyanotic

Non Cyanotic
Left to Right Shunt
Atrial Septal Defect
Ventricle Septal Defect
Patent Ductus

Arteriosus
Outflow tract Obstruction
Pulmonal stenosis
Aorta stenosis

oligaemic
oligaemic lung
lung

RVH
RVH

ToF
ToF
PA
PA +
+ VSD
VSD
DORV
DORV +
+ PS
PS
ASD/VSD
ASD/VSD +
+
PS
PS

LVH
LVH

TA
TA +
+ PS
PS
PA
PA +
+ RV
RV

hipoplastik
hipoplastik

plethoric
plethoric lung
lung

RVH
RVH

TGA
TGA -- IVS
IVS
DORV
DORV +
+ VSD
VSD
TAPVD
TAPVD

BVH
BVH

Truncus
Truncus Art
Art
TGA
TGA +
+ VSD
VSD

Non Cyanotic

HEMODYNAMIC
Left to Right Shunt
- size of the defect
- compliance of RV is greater than LV

RA, RV and PA enlargement

Pulmonary Hypertension
- large ASD large left to right shunt
- develop in the third to fourth decades of life
- Pulmonary Vascular Obstructive Disease
- bidirectional shunt right to left shunt

sianosis
EISENMENGER SYNDROME

AUSCULTATION
Widely split and fixed S2

RV volume overload prolonged RV ejection time

delays the closure of the pulmonary valve

large pulmonary venous return to RA fixed split

Systolic ejection murmur

not caused by the shunt

originates from the increased blood flow passing

through the normal-sized pulmonary valve relative

PS

Mid diastolic murmur

increased blood flow through the tricuspid valve

relative TS
large left to right shunt

Accentuated P2
pulmonary hypertension

CHEST X-RAY
RA, RV and PA dilatation
prominent pulmonary artery
segment
increased pulmonary
vascular marking (plethora)

HEMODYNAMIC
Left to Right Shunt
- size of the defect
- level of pulmonary vascular resistance

LA, LV and PA enlargement

Pulmonary Hypertension
-

large VSD large left to right shunt

high pulmonary vascular resistance
Pulmonary Vascular Obstructive Disease
bidirectional shunt right to left shunt sianosis
EISENMENGER SYNDROME

AUSCULTATION

Small VSD

Small VSD

normal P2 intensity
holosystolic murmur
produced by left to right shunt

Large VSD

Large VSD

accentuated P2 pulmonary hypertension

ejection click (occasionally )
holosystolic murmur left to right shunt
mid diastolic murmur increased blood flow through the mitral
valve relative MS

Large VSD with Pulmonary Vascular Obstructive Disease

loud and single S2
decreased loudness of the holosystolic murmur (or disappear)

CHEST X-RAY
LA, LV and PA dilatation
prominent pulmonary artery
segment
increased pulmonary
vascular marking (plethora)

HEMODYNAMIC
Left to Right Shunt
- size of the ductus diameter, length and turtuosity
- level of pulmonary vascular resistance

LA, LV, ascending Ao and PA enlargement

Pulmonary Hypertension
-

large PDA large left to right shunt

high pulmonary vascular resistance
Pulmonary Vascular Obstructive Disease
bidirectional shunt right to left shunt sianosis
EISENMENGER SYNDROME

AUSCULTATION
Normal P2 intensity

small PDA normal PA pressure

accentuated if pulmonary hypertension is present

Continuous (machinery) murmur

left to right shunt occurs throughout the cardiac cycle

significant pressure gradient between Ao and PA during systole
and diastole

Apical mid diastolic murmur

increased blood flow through the mitral valve relative MS

Large PDA with Eisenmenger Syndrome

single and loud S2 pulmonary hypertension

no longer continuous murmur ejection systolic murmur

CHEST X-RAY
LA, LV, ascending Ao and PA
dilatation
prominent pulmonary artery
segment
increased pulmonary vascular
marking (plethora)

NONCYANOTIC CHD
OUTFLOW TRACT OBSTRUCTION

VENTRICLE OUTFLOW TRACT OBSTRUCTION

WITHOUT SHUNT

Left ventricle outflow tract

obstruction

 narrow split S2
ejection systolic click
harsh ejection systolic murmur

AUSCULTATION

 asymptomatic
symptomatic
depend of
severity of lesion
myocardial function

dyspneu
dyspneu
Feeding
Feeding difficulty
difficulty
Failure
Failure to
to thrive
thrive
Heart
Heart Failure
Failure
Syncope
Syncope pain
pain
chest
chest
Sudden
Sudden death
death

NEONATUS
duct dependent systemic circulation
Closed duktus arteriosus
deteriorate systemic
circulation
hypoperfusion

BABY AND CHILD

asymptomatic mild lesion
symptomatic :
headache
epitasis
Pulsless

Right ventricle outflow tract

obstruction

valvar

infundibular

supravalvar

RV pressure overload
RVH

NEONATUS critical PS
duct dependent pulmonary circulation
closed duktus arteriosus
severe cyanosis acidosis
BABY and CHILD
asymptomatic mild lesion
symptomatic :
Right Heart failure
oedema
hepatomegaly
acites

Cyanosis
bila ada PFO

 S2 weak
ejection systolic click

AUSCULTATION

harsh ejection systolic murmur

LESI OBSTRUKTIF ALUR KELUAR

VENTRIKEL KIRI DAN KANAN

Neonatus Duct Dependent

PGE1
sementara dipersiapkan intervensi
non-bedah / bedah)

INTERVENSI NON BEDAH

Gradien tekanan > 40 50 mmHg

Balloon Aortic Valvyuloplasty (AS valv)
Balloon Pulmonal Valvuloplasty (PS valv)
Balloon Angioplasty (CoA)
INTERVENSI BEDAH

Valvotomy (PS / AS valvar)

Reseksi otot (PS / AS subvalvar)
Rekonstruksi (PS / AS Supravalvar)

Cyanotic

FKUI International

Oligemic
Pulmonary Stenosis
or Atresia

PFO / ASD / VSD

( R L SHUNT )

Tetralogi Fallot
PS + PFO / ASD
PA + VSD

cyanosis
spell hypoxia
squatting

Spell cyanotic

Less than1 year ( 2 4 month )

minute - hour

Emergrncy
Serious complication
CVD
KEMATIAN

knee-chest position

Oxigen

Sedasion : diazepam

or morfin
acidosis correction : \
Bic Nat
Propranolol
BT Shunt/ surgery

 deviasion
deviasion of
of infundibulum
infundibulum septum
septum
to
to anterior
anterior
malrotasi
malrotasi bulbus
bulbus

VSD
VSDperimembranus
perimembranus
Ao
Aooverriding
overriding
PS
PSvalvular-infundibular
valvular-infundibular
RV
RVhipertrof
hipertrof

PALIATIF operation
BT SHUNT
spell hypoxia
< 6 month
small PA size

TOTAL CORRECTION
> 6 month
good size of PA

Plethora
TGA
TGA
COMMON
COMMONMIXING
MIXING

Common Mixing:
TAPVD
Univentricular Connection
Trunkus Arteriosus

Increase Pulmonary blood flow

feeding difficulty
Failure to Thrive
reccurence RT infection
CHF
pulmonary Hypertention

Pulmonary
Pulmonary
vascular resistence
resistence
vascular

Paralel circulation

atrial : PFO, ASD

ventricle : VSD
Geart of Arteries: PDA

www.schneiderchildrenhospital.org

Intervension non surgery

For
Congenital Heart Diseases

FKUI International

Occlusion of Intracardiac and Vascular

Shunts
Coil embolization of PDA

Left, top: Catheter crosses the PDA

from the aortic side and delivers a coil.
Left, bottom: Withdrawal of catheter,
leaving coil in PDA

Occlusion of Intracardiac and Vascular

Shunts
Amplatzer Ductal Occluders

Amplatzer ductal occluder

Illustration courtesy AGA Medical Group

Aorta angiogram with device

occlusion of PDA, lateral view

Amplatzer Duct Occluder

Amplatzer Duct Occluder

Occlusion of Intracardiac and Vascular

Shunts
Amplatzer occlusion of atrial septal defect

Clockwise from above:

Transcatheter delivery of
Amplatzer device, which is
positioned across the
atrial septal defect
Left: Amplatzer device in
place

Occlusion of Intracardiac and Vascular

Shunts
Devices for occlusion of the PFO and ASD

Above:

Gore Helex septal occluder

Illustration courtesy W. L. Gore and Associates

Upper left:

CardioSEAL occluder
Illustration courtesy NMT Medical

Lower left:

Amplatzer PFO occluder

Illustration courtesy AGA Medical Group

Amplatzer septal
occluder

Amplatzer septal
occluder

Occlusion of Intracardiac and Vascular Shunts

Ventricular Septal Defect Occlusion

Above: Echocardiogram of muscular VSD

Upper right: Fluoro image of CardioSEAL
device occlusion of a VSD. Transesophageal
echo probe (TEE) and pigtail catheter in place.
Lower right: Amplatzer muscular ventricular
septal occluder Illustration courtesy AGA Medical Group

Occlusion of Intracardiac and Vascular Shunts

VSD Occlusion with CardioSEAL Device

Balloon Pulmonary
valvuloplasty

Angioplasty
Aortic Coarctation Angioplasty

Angiograms showing (left) post-surgical coarctation of the aorta

and (right) angioplasty balloon inflated across coarctation site

Angioplasty
Aortic Coarctation Angioplasty

Illustrations showing (left) uninflated and (right) inflated angioplasty

balloon positioned within coarctation of the descending aorta

Intravascular Stents
Coarctation of the Aorta

Left: uninflated angioplasty balloon and stent within coarctation

Middle: expansion of balloon and stent
Right: deflation of balloon leaving stent wide open