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Structures of the heart

Normal Heart
Sign and Symptom of CHD
• Dyspneu
• Cyanosis
• Feeding problem
– Intermittent feeding
– Prolonged feeding
• Dyspneu de effort
• Orthpneu
• Recurrent respiratory tract infection
• Poor weight gain
• Asymptomatic murmur
• The presence of murmur does not mean that
there is CHD
• No murmur does not exclude CHD
Classification of CHD
– Acyanosis
• PDA
• ASD
• VSD
• PS
– Cyanosis
• ToF
• TGA
• Pulmonary atresia
• Truncus arteriosus
• Total anomaly pulmonary vein drainage
• Ticuspid atresia
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
Patent Ductus Arteriosus

LA LV
Lungs

PA AO

Systemic
RV RA

Qp > Qs
Patent Ductus Arteriosus

RA LA
RA LA

RV LV
RV LV
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 4th 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
Patent Ductus Arteriosus

Diagnosis Differential
AP-window
Arterio-venous fistulae

Management
premature: indometasin
PDA closure : surgery
transcatheter closure
PDA

Neonates/Infants Children/Adults

Heart failure (+) Heart failure (-) PH (-) PH (+)

Premature Full term


LR RL

Anti failure Anti failure


Indometacin Hyperoxia

Fail Success
Success Fail Reactive Non
Age >12wks reactive
W >4kg

Spontaneous Surgical Transcatheter closure Conservative


closure ligation
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
Atrial Septal defect
( ASD )

• Insidence : + 10 %
• ♂: ♀ ratio = 1,5 to 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
Atrial Septal Defect

Diagram of ASD
Atrial septal defect

LA LV
Lungs

PA AO

Systemic
RV RA

Qp > Qs
Atrial septal Defect

RA LA

RA LA

RV LV
RV LV
Atrial septal Defect

 Clinical findings
Asymptomatic
Auscultation :
Normal 1st HS or loud
Widely split and fixed
2nd HS
Ejection systolic murmur
Atrial Septal Defect

Auscultation :1st HS N or loud


widely split and fixed 2nd HS
Ejection Sistolic Murmur
Atrial Septal Defect

ECG : IRBB , right ventricular hypertrophy


Interpretasi foto dada
Atrial Septal Defect
Chest X-Ray

Right atrial enlargement


Prominence the MPA
segment
Increased pulmonary
vascular marking
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

Infants Children/Adults
Observation
Heart Heart PH (-) PH (+)
Evaluation Failure (-) Failure (+)
At age 5-8 yrs PVD PVD
Anti failure (-) (+)
Cath Hyperoxia
Success Fail

FR<1.5 FR>1.5 Age >1yrs Surgical Reac- Non


W >10kg Closure tive reactive

Conservative Transcatheter closure (Secundum ASD) / Conservative


Surgical Closure(other tipe of ASD)
Atrial septal defect
Atrial septal defect

ASD before occlusion


Atrial septal defect

During balloon
sizing
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
Ventricular Septal defect

LA LV
Lungs

PA AO

RV RA Systemic

Qp > Qs
Ventricular septal defect

RA
LA
RA LA

RV LV RV LV
Ventricular Septal Defect
Ventricular Septal Defect

• 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
Ventricular Septal Defect

Murmur: pansystolic
grade 3/6 or higher at
LSB 3 Small VSD

Large VSD
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
Aortic valve Infundibular PH Spontaneous Smaller
prolaps stenosis closure

Fail Success PVD(-) PVD(+) Cath

PAB Cath FR<1.5 FR>1.5


Cath

Evaluate Reactive Non-


in 6 mths reactive

Conservative
Surgical closure/Transcatheter closure
Ventricular septal defect

VSD before occlusion


Ventricular septal defect

VSD during deploying


the device
Ventricular septal defect

VSD after occluded


using ASO
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

Normal or mild cardiomegaly


Marked pulmonary valve
post stenotic dilatation
Normal pulmonary
vascularity
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

Single 2nd HS, ejection systolic murmur


Tetralogi Fallot
Tetralogy Fallot

CXR :
Boot-shaped
Concave pulmonary
segment
Apex upturned
Decreased pulmonary
blood flow
“ Boot-shaped” appearance
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 • CXR
• ECG Tetralogy of Fallot • echo

< 1 yr > 1 yr

spell (+) spell (-)


propranolol
age 1 yr
cath
failed succeed

BTS
evaluation

cath BTS/ small PA good sized PA


PDA Stent

total correction
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
Transposition of Great artery

Hemodynamic normal Hemodynamic of TGA


“series” “parallel”
Transposition of Great artery

In adequate Mixing Adequate Mixing

TGA without VSD


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 normal or bigger
Cyanotic vary from mild to severe
Auscultation : single 2nd 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 2nd HS and loud
Transposition of Great artery

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

CXR :
Cardiomegaly
Egg-on-side heart
Increased
pulmonary
vascular
marking
“Egg on side” appearance
Transposition of Great artery

ECG :
RAD
RVH
Echocardiography : to confirm diagnosis
Cardiac catheterization: usually is not
needed
Transposition of Great artery

Diagnosis Differential

 trunkus arteriosus
 trikuspid atresia
 pulmonary atresia

Management
Palliative: BAS
Surgery: arterial switch
Paliative : Blalock-Taussig shunt
Transposition of Great Artery

PGE1 BAS/Blallock Hanlon

VSD(-) VSD(+)

LVOTO(-) LVOTO(+)
 1mth > 1mth
3 mths >3 mths Dynamic & Un-
Cath resectable resectable
Cath
LV2/3 syst LV<2/3 syst BTS/
PARI<8 PARI8 PDA stent
Senning PAB
Cath
Arterial Switch Arterial Switch and Rastelli
Perforated VSD
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
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 with normal Tricuspid atresia with


related great artery transposed geat artery
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

• 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


Disadvantageous surgery
TERIMA KASIH

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