Burhanuddin Iskandar

Pediatric Cardiology
Pediatric Department,Medical Faculty,
Hasanuddin University/ WS Hospital Makassar
Telur TGA
Manusia salju/angka 8 TAPVD
Karpet Ebstein anomali
Sepatu both TF
Structures of the heart
Normal Heart

Atrial Septal defect
( ASD )
Insidence : + 10 %
: ratio = 2 : 1
Anatomy :
Defect on foramen ovale : Secundum ASD
Defect at SVC and RA junction: sinus
venosus ASD
Defect at ostium primum : primum ASD

ASD
Atrial Septal Defect
LA
LV
RV RA
PA
AO
Systemic
Lungs
Qp > Qs
Atrial septal defect
Atrial Septal Defect
Diagram of ASD
RA
RV
LA
LV
RA
RV
LA
LV
Atrial septal Defect
Clinical findings
Asymptomatic
Auscultation :
Normal 1st HS or loud
Widely split and fixed
2
nd
HS
Ejection systolic murmur

Atrial septal Defect
Atrial Septal Defect
Auscultation :1
st
HS N or loud
widely split and fixed 2
nd
HS
Ejection Sistolic Murmur
ECG : IRBB , right ventricular hypertrophy

Atrial Septal Defect
Right atrial enlargement
Prominence the MPA
segment
Increased pulmonary
vascular marking
Atrial Septal Defect
Chest X-Ray
Atrial Septal Defect
Diagnosis Differential

Primary Atrial Septal Defect
ECG : LAD
Partial Anomalous Pulmonary Vein
Drainage
Pulmonary Stenosis
Innocent Murmur

Atrial Septal defect

Management
Surgery : Preschool age
Recent treatment: transcatheter closure using
ASO (Amplatzer septal occluder)

ASD
Small Shunt
Large Shunt
Observation
Evaluation
At age 5-8 yrs
Cath
FR<1.5 FR>1.5
Conservative
Infants
Children/Adults
Heart
Failure (-)
Heart
Failure (+)
Age >1yrs
W >10kg
Transcatheter closure (Secundum ASD) /
Surgical Closure(others)
Conservative
Anti failure
Fail Success
PH (-) PH (+)
PVD
(-)
PVD
(+)
Hyperoxia
Reac-
tive
Non
reactive
Surgical
Closure
Atrial septal defect
Atrial septal defect
ASD before occlusion
During balloon
sizing
Atrial septal defect
Atrial septal defect
ASD after occluded
using ASO
Ventricular septal defect
Insidence
20 % of all CHD
No sex influenced
Anatomy
Subarterial defect : below pulmonary and
aortic valve
Perimembranous defect: below aortic valve at pars
membranous septum
Muscular defect
VSD
Ventricular Septal Defect
LA
LV
RV
RA
PA
AO
Systemic
Lungs
Qp > Qs
Ventricular Septal defect
RA
RV
RA
LA
LA
RV LV LV
Ventricular septal defect
Ventricular Septal Defect
Ventricular Septal Defect
Clinical findings
Day 1
st
after birth: murmur (-)
After 2-6 weeks : murmur (+)
Murmur : pansystolic grade 3/6 or higher
at LSB 3
Small muscular defect: early systolic murmur
Significant defect: Mid diastolic murmur at apex
Small VSD
Large VSD
Ventricular Septal Defect
Murmur: pansystolic
grade 3/6 or higher at
LSB 3

Ventricular Septal Defect
Cardiomegaly
Apex down ward
Prominence pulmonary
artery segment
Increased pulmonary vascular
marking

Ventricular septal Defect
Diagnosis Differential

PDA with PH
Tetralogy Fallot non cyanotic
Inoscent murmur


Ventricular septal defect
Management:

Definitive : VSD closure
Surgery
Transcatheter closure

DSV
Heart failure (+) Heart failure (-)
Anti failure
Fail Success
PAB
Evaluate
in 6 mths
Surgical closure/Transcatheter closure
Aortic valve
prolaps
Infundibular
stenosis
PH Smaller Spontaneous
closure
Cath
PVD(-) PVD(+) Cath
Cath
Reactive Non-
reactive
Conservative
FR>1.5 FR<1.5
Ventricular septal defect
VSD before occlusion
Ventricular septal defect
VSD during deploying
the device
Ventricular septal defect
VSD after occluded
using ASO
Patent Ductus Arteriosus
Insidence
+ 10%
Female : Male = 1.2 to 1.5 : 1
Premature and LBW higher

Anatomy
Fetus: ductus arteriosus connects PA and aorta.
If ductus does not closs Patent Ductus arteriosus

PDA

LA
LV
RV
RA
PA
AO
Systemic
Lungs
Qp > Qs
Patent Ductus Arteriosus
RA
RV
LA
LV
RA
LA
RV LV
Patent Ductus Arteriosus
Patent Ductus Arteriosus
Clinical findings

Small defect:
Symptom (-)
Growth and development normal
Significant defect:
Decreased exercise tolerant
Weigh gained not good
Frequent URTI
Specific case: pulsus seler at 4
th
extremities

Patent Ductus Arteriosus
Diagnosis
Pulsus seler and continuous murmur heard

Patent Ductus Arteriosus
Chest X- Ray
Similar to VSD
Patent Ductus Arteriosus
Auscultation : continuosus murmur
at upper LSB 2

Diagnosis Differential
AP-window
Arterio-venous fistulae

Management
premature: indometasin
PDA closure : surgery
transcatheter closure
Patent Ductus Arteriosus
PDA
Neonates/Infants Children/Adults
Heart failure (+) Heart failure (-)
Premature
Full term
Anti failure
Indometacin
Success Fail
Spontaneous
closure
Anti failure
Success Fail
Surgical
ligation
Transcatheter closure
PH (-) PH (+)
LR RL
Hyperoxia
Reactive
Non
reactive
Conservative
Age >12wks
W >4kg
Patent Ductus Arteriosus
Patent Ductus Arteriosus
Patent Ductus Arteriosus
PDA before occluded
using ADO
Patent Ductus Arteriosus
PDA after occluded
using ADO
Patent Ductus Arteriosus
PDA before occluded
using coil
Patent Ductus Arteriosus
PDA after occluded
using coil
Pulmonary Stenosis
Incidence : 8-10%

Anatomy:
Pulmonary stenosis valvular :
Bicuspid pulmonary valve
Valve leaflet thickening and adhession
Pulmonary stenosis infundibular :
Hyperthropy infundibulum

Pulmonary Stenosis
Clinical findings
Valvular stenosis
Mild : Ejection systolic
Wide 2
nd
HS
ejectiin click
Moderate: ejection systolic, early systolic click
Severe : ejecstion systolic, ejection click (-)
Stenosis infundibular
Ejection click ( - )
1
st
HS normal, 2
nd
HS weak, ejection systolic
Pulmonary stenosis periphery
1
st
& 2
nd
HS normal, ejection systolic
Pulmonary Stenosis
Mild : ejection systolic
2
nd
HS wide split
ejection click
Moderate: ejecsi systolic , early ejection click
Severe : ejection systolic, click ejection (-)
Poulmonary Stenosis
Diagnosis
Asymptomatic patient:
click systolic (stenosis valvular)
systolic murmur
wide split 2
nd
HS vary with respiration

Poulmonary Stenosis
Normal or mild cardiomegaly
Marked pulmonary valve
post stenotic dilatation
Normal pulmonary
vascularity
ECG : RAD
Echocardiograhhy : confirmation diagnosis
Catheterization: increased RV pressure
without increased oxygen saturation

Pulmonary Stenosis
Pulmonary Stenosis
Management

Medicamentosa : useless
Mild stenosis: intervention (-)
Moderate stenosis: observation
Severe stenosis: balloon valvuloplasty
Pulmonary Stenosis
Pulmonary Stenosis
Before ballooning
Pulmonary Stenosis
During ballooning
Pulmonary Stenosis
After ballooning
Coarctation Aorta
Incidence
In Western country 5 % of all CHD
In Asian Country incidence lower
underdiagnosis ?

Anatomy
Stenosis at any where in the aorta
(from aortic valve to abdominalis aorta)
More frequent at ductus arteriosus Botalli and
pulmonary artery junction
Coarctation Aorta
Clinical findings
Severe coarctation in neonates period can cause
heart failure in 1
st
weeks of life

Clinical manifestation in children:
arterial hypertension
commonly asymptomatic
Different pulses felt at upper and lower
extremities

Examination : increased left ventricular activity, thrill
systolic, 1
st
and 2
nd
HS normal, ejection systolic
murmur
Coarctation Aorta
Diagnosis
Clinically : lower extremities pulses are weak
CXR : Mild cardiomegaly
Prominence of aortic knob
Normal pulmonary blood flow
ECG : normal or LVH
Echocardiography: a discrete shelf-like membrane
Cardiac catheterization and angiography: to confime
diagnosis
Coarctation Aorta
Management

Neonates :
PGE1 to maintain PDA
Diuretic
Correction acid-base imbalance
Prepared to undergo surgery

Big children:
Surgery should be done as soon as diagnosis
made
Balloon angioplasty
Coarctation Aorta
Coarctation Aorta
Coarctation Aorta
Coarctation Aorta
Coarctation Aorta
Before ballooning
Coarctation Aorta
During ballooning
Coarctation Aorta
After ballooning
Tetralogy Fallot
Insidence
5-8% from all CHD

Anatomy
Cause: Left-anterior deviation of infundibular septum

Sindroma consist of 4 items:
VSD
pulmonary stenosis
aortic over-riding
RVH
Tetralogy Fallot
Tetralogy Fallot
Hemodynamic acyanotic
Hemodynamic cyanotic
Tetralogy Fallot
Diagnosis

Clinically : cyanosis
Single 2
nd
HS, ejection systolic murmur

Tetralogy Fallot
Single 2
nd
HS, ejection systolic murmur
Tetralogi Fallot
CXR :
Boot-shaped
Concave pulmonary
segment
Apex upturned
Decreased pulmonary
blood flow

Tetralogy Fallot
Tetralogy Fallot
ECG : RAD
Echocardiography : to confirm diagnosis

Tetralogy Fallot
Diagnosis Differential
Pulmonary Atresia
Double outlet right ventricle and pulmonary stenosis
Transposisi of great arteri and pulmonary stenosis

Management
Paliative treatment: Blalock-Taussig shunt
Definitive: total correction


Tetralogy of Fallot
< 1 yr > 1 yr
spell (+)
spell (-)
propranolol
failed
succeed
BTS
total correction
cath
small PA good sized PA
clinically
ECG
CXR
echo
age 1 yr
cath
BTS/
PDA Stent
evaluation
Tetralogy Fallot
Tetralogy Fallot
Transposition of Great
Artery

Insidence
5% of CHD

Anatomy
Abnormality of formation of trunkal septum that cause
aorta arising from RV and PA arising from LV

Transposition of Great artery
Hemodynamic normal
Hemodynamic of TGA
series parallel
Transposition of Great artery
TGA without VSD
In adequate Mixing Adequate Mixing
Transposition of Great artery
TGA with large VSD TGA with VSD and PS
Transposition of Great artery
Clinical aspects

More frequent in male
Birth weight usually normal or bigger
Cyanotic vary from mild to severe
Auscultation : single 2
nd
HS and loud
Murmur vary from silent to pansystolic murmur or
continuous murmur
Transposition of Great artery
Diagnosis
Clinically :
Suspicious if neonates presents with cyanotic
with birth weight normal or bigger
Murmur (-)
Single 2
nd
HS and loud

Transposition of Great artery
Murmur (-)
Single 2
nd
HS and loud

Transposition of Great artery
Transposition of Great artery
CXR :
Cardiomegaly
Egg-on-side heart
Increased
pulmonary
vascular
marking
Transposition of Great artery
ECG :
RAD
RVH
BVH
Echocardiography : to confirm diagnosis
Cardiac catheterization: usually is not needed

Diagnosis Differential

trunkus arteriosus
trikuspid atresia
pulmonary atresia

Management

Surgery: arterial switch
Paliative : Blalock-Taussig shunt
Transposition of Great artery
Transposition of Great Artery
PGE1
VSD(-) VSD(+)
1mth > 1mth
Cath
LV2/3 syst LV<2/3 syst
PAB
Arterial Switch
LVOTO(-)
LVOTO(+)
>3 mths 3 mths
Cath
PARI<8 PARI8
Arterial Switch and
Perforated VSD
Dynamic &
resectable
Un-
resectable
BTS/
PDA stent
Cath
Rastelli
Senning
BAS/Blallock Hanlon
Transposition of Great artery
Truncus Arteriosus
Insidence
around 1 % of CHD
Anatomy
Failure of septation of truncus arteriosus form aorta and
pulmonary artery
There are 3 type:
Type 1 : MPA arises from the truncus and then divides into
the RPA and LPA
Tipe 2 : The PAs arise from the posterior aspect of the
truncus
Tipe 3 : The PAs arise from the lateral aspects of the truncus
Tipe 4: Arteries arising from the descending aorta supply the
lungs
Truncus Arteriosus
Truncus Arteriosus
Truncus Arteriosus
Diagnosis
Clinically suspected if:
neonates present with cyanotic and single 2
nd
HS
murmur vary
CXR: cardiomegaly
increased pulmonary vascular marking
ECG: biventricular hypertrophy
Echocardiografhy: to confirm diagnosis
Catheterization: decreased oxygen saturation at right
heart and aorta

Truncus Arteriosus
Diagnosis Differential
Transposisi of great artery
Total anomalus pulmonary vein drainage

Management

Medicamentosa : temporary
Surgery: Rastelli
Palliative: pulmonary artery banding
Truncus Arteriosus
Truncus Arteriosus
Tricuspid Atresia
Incidence
1 % from all CHD
Embriology
Valve formed at 5
th
weeks
Fussion of part of endocardial
cushion, ventricular septum and
miocardium
Anatomy
Valve leaflet adhession one to another,
difficult to open
ASD essentially required to drain blood
from RA to LA
Classified into 2 group
Normal related great artery
Transposed grat artery
Tricuspid Atresia
Tricuspid Atresia with normal
related great artery

Tricuspid atresia with
transposed geat artery

Tricuspid Atresia
Manifestasi klinis
Cyanosis early after birth
Increased RV activity
Increased LV activity
Auscultation
Single 1
st
and 2 nd HS

Tricuspid Atresia
Clinical manifestation
In almost all patients murmur is silent
If murmur present
Diastolic murmur due to relative MS
Pansystolic murmur due to VSD

Tricuspid Atresia
Tricuspid Atresia
Diagnosis and diagnosis differential
Clinically: Cyanosis with or without murmur



Tricuspid Atresia
CXR:
Heart minimally
Enlarged
The PVMs are
Decreased
The MPA segment is
concave
Tricuspid Atresia
ECG:
LAD
Left ventricular hypertrophy
With or without LAE
Tricuspid Atresia
Echocardiography: Essential to make
diagnosis
Catheterization
Catheter can not be passed from RA to RV
Increased RA and LA pressure
Decreased oxygen saturation in LA
Angiography: definitive diagnosis
Tricuspid Atresia
Diagnosis differential
Transposition of great artery
Truncus arteriosus
Tetralogy of Fallot
Total Anomalous pulmonary vein
drainage

Tricuspid Atresia
Management
Fontan operation
Tricuspid Atresia
Tricuspid Atresia
Tricuspid Atresia
Tricuspid Atresia
Tricuspid Atresia
Tricuspid Atresia
Modification of Fontan operation
Tricuspid Atresia