Congenital Heart Disease
Congenital Heart Disease
Congenital Heart Disease
Normal Heart
ASD
Diagram of ASD
Lungs
LV
LA
AO
PA
RV
RA
Qp > Qs
Systemic
RA
RV
RA
LA
RV
LV
LA
LV
Clinical findings
Asymptomatic
Auscultation :
Normal 1st HS or loud Widely split and
fixed
2nd HS
Ejection systolic murmur
Diagnosis Differential
Primary Atrial Septal Defect
ECG : LAD
Partial Anomalous Pulmonary Vein
Drainage
Pulmonary Stenosis
Innocent Murmur
Management
Surgery : Preschool age
Recent treatment: transcatheter closure using
ASO (Amplatzer septal occluder)
ASD
Large Shunt
Small Shunt
Observation
Evaluation
At age 5-8 yrs
Cath
FR<1.5
Heart
Failure (-)
Children/Adults
PH (-)
Heart
Failure (+)
PVD
(-)
Anti failure
Success
FR>1.5
Conservative
Infants
Age >1yrs
W >10kg
PH (+)
PVD
(+)
Hyperoxia
Fail
Surgical
Closure
Reactive
Non
reactive
Conservative
During balloon
sizing
VSD
Lungs
LV
LA
AO
PA
RA
RV
Qp > Qs
Systemic
RA
RV
LA
LV
RA
LA
RV
LV
Clinical findings
Day 1st 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
Murmur: pansystolic
grade 3/6 or higher at
LSB 3
Small VSD
Large VSD
Cardiomegaly
Apex down ward
Prominence pulmonary
artery segment
Increased pulmonary vascular
marking
Diagnosis Differential
PDA with PH
Tetralogy Fallot non cyanotic
Inoscent murmur
Management:
Definitive : VSD closure
Surgery
Transcatheter closure
DSV
Heart failure (+)
Anti failure
Aortic valve
prolaps
Fail
Infundibular
stenosis
PVD(-)
Success
PAB
Evaluate
in 6 mths
PH Spontaneous
closure
Cath
PVD(+)
Cath
Reactive
Smaller
Cath
FR<1.5 FR>1.5
Nonreactive
Conservative
Anatomy
Fetus: ductus arteriosus connects PA and aorta.
If ductus does not closs Patent Ductus arteriosus
PDA
Lungs
LV
LA
AO
PA
RA
RV
Qp > Qs
Systemic
RA
RV
LA
LV
RA
LA
RV
LV
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 4th extremities
Diagnosis
Pulsus seler and continuous murmur heard
Chest X- Ray
Similar to VSD
Diagnosis Differential
AP-window
Arterio-venous fistulae
Management
premature: indometasin
PDA closure : surgery
transcatheter closure
PDA
Neonates/Infants
Heart failure (+)
Premature
Anti failure
Indometacin
Success
Children/Adults
Full term
Anti failure
Fail
Fail
Surgical
ligation
PH (+)
RL
LR
Hyperoxia
Success
Age >12wks
W >4kg
Spontaneous
closure
PH (-)
Reactive
Transcatheter closure
Non
reactive
Conservative
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 2nd HS
ejectiin click
Moderate: ejection systolic, early systolic click
Severe : ejecstion systolic, ejection click (-)
Stenosis infundibular
Ejection click ( - )
1st HS normal, 2nd HS weak, ejection systolic
Pulmonary stenosis periphery
1st & 2nd HS normal, ejection systolic
Pulmonary Stenosis
Mild
: ejection systolic
2nd 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 2nd HS vary with respiration
Poulmonary Stenosis
Pulmonary Stenosis
ECG : RAD
Echocardiograhhy : confirmation diagnosis
Catheterization: increased RV pressure
without increased oxygen saturation
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
Coarctation Aorta
Clinical findings
Severe coarctation in neonates period can cause
heart failure in 1st 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, 1st and 2nd 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
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 2nd HS, ejection systolic murmur
Tetralogy Fallot
Tetralogi Fallot
Tetralogy Fallot
CXR :
Boot-shaped
Concave pulmonary
segment
Apex upturned
Decreased pulmonary
blood flow
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
clinically
ECG
Tetralogy of Fallot
< 1 yr
> 1 yr
spell (+)
propranolol
spell (-)
age 1 yr
failed
CXR
echo
succeed
cath
BTS
evaluation
cath
BTS/
PDA Stent
small PA
total correction
good sized PA
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
Hemodynamic normal
series
Hemodynamic of TGA
parallel
In adequate Mixing
Adequate Mixing
Clinical aspects
Diagnosis
Clinically :
Suspicious if neonates presents with cyanotic
with birth weight normal or bigger
Murmur (-)
Single 2nd HS and loud
Murmur (-)
Single 2nd HS and loud
CXR :
Cardiomegaly
Egg-on-side heart
Increased
pulmonary
vascular
marking
ECG :
RAD
RVH
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
BAS/Blallock Hanlon
VSD(-)
1mth
VSD(+)
LVOTO(-)
> 1mth
Cath
LV2/3 syst
3 mths
LV<2/3 syst
Senning
LVOTO(+)
PAB
Arterial Switch
Cath
Arterial Switch and
Perforated VSD
Rastelli
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
Truncus Arteriosus
Diagnosis
Clinically suspected if:
neonates present with cyanotic and single 2nd 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
Tricuspid Atresia
Incidence
1 % from all CHD
Embriology
Valve formed at 5th weeks
Fussion of part of endocardial
cushion, ventricular septum and
miocardium
Tricuspid Atresia
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
Manifestasi klinis
Cyanosis early after birth
Increased RV activity
Increased LV activity
Auscultation
Single 1st 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
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
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