Post on 28-Dec-2015
Burhanuddin IskandarPediatric Cardiology
Pediatric Department,Medical Faculty, Hasanuddin University/ WS Hospital Makassar
Telur TGAManusia salju/angka 8 TAPVDKarpet Ebstein anomaliSepatu both TF
Structures of the heart
Normal Heart
Atrial Septal defect( ASD )
• Insidence : + 10 %• ♀: δ 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 findingsAsymptomaticAuscultation :
Normal 1st HS or loudWidely split and
fixed 2nd HSEjection systolic murmur
Atrial septal Defect
Atrial Septal Defect
Auscultation :1st HS N or loud
widely split and fixed 2nd HS
Ejection Sistolic Murmur
ECG : IRBB , right ventricular hypertrophy
Atrial Septal Defect
Right atrial enlargementProminence the MPA segmentIncreased pulmonary vascular marking
Atrial Septal DefectChest 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
EvaluationAt age 5-8 yrs
Cath
FR<1.5 FR>1.5
Conservative
Infants Children/Adults
Heart Failure (-)
Heart Failure (+)
Age >1yrsW >10kg
Transcatheter closure (Secundum ASD) /Surgical Closure(others)
Conservative
Anti failure
FailSuccess
PH (-) PH (+)
PVD (-)
PVD (+)
Hyperoxia
Reac-tive
Nonreactive
SurgicalClosure
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 LALA
RV LVLV
Ventricular septal defect
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
Small VSD
Large VSD
Ventricular Septal Defect
Murmur: pansystolic grade 3/6 or higher at LSB 3
Ventricular Septal Defect
CardiomegalyApex down wardProminence pulmonary artery segmentIncreased 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 SmallerSpontaneousclosure
Cath
PVD(-) PVD(+) Cath
Cath
Reactive Non-reactive
Conservative
FR>1.5FR<1.5
Ventricular septal defect
VSD before occlusion
Ventricular septal defect
VSD during deploying the device
Ventricular septal defect
VSD after occludedusing ASO
Patent Ductus Arteriosus
Insidence+ 10%Female : Male = 1.2 to 1.5 : 1Premature and LBW higher
AnatomyFetus: 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 tolerantWeigh gained not goodFrequent URTI
Specific case: pulsus seler at 4th extremities
Patent Ductus Arteriosus
DiagnosisPulsus 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 failureIndometacin
Success Fail
Spontaneous closure
Anti failure
SuccessFail
Surgical ligation
Transcatheter closure
PH (-) PH (+)
LR RL
Hyperoxia
Reactive Nonreactive
Conservative
Age >12wksW >4kg
Patent Ductus Arteriosus
Patent Ductus Arteriosus
Patent Ductus Arteriosus
PDA before occludedusing ADO
Patent Ductus Arteriosus
PDA after occludedusing ADO
Patent Ductus Arteriosus
PDA before occludedusing coil
Patent Ductus Arteriosus
PDA after occludedusing 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 findingsValvular stenosis
Mild : Ejection systolic Wide 2nd HS ejectiin click
Moderate: ejection systolic, early systolic clickSevere : ejecstion systolic, ejection click (-)
Stenosis infundibular Ejection click ( - )1st HS normal, 2nd HS weak, ejection systolic
Pulmonary stenosis periphery1st & 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
• DiagnosisAsymptomatic patient:
click systolic (stenosis valvular)systolic murmurwide split 2nd HS vary with respiration
Poulmonary Stenosis
Normal or mild cardiomegaly Marked pulmonary valve post stenotic dilatationNormal 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 AortaIncidence• In Western country 5 % of all CHD• In Asian Country incidence lower
underdiagnosis ?
AnatomyStenosis 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 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
madeBalloon angioplasty
Coarctation Aorta
Coarctation Aorta
Coarctation Aorta
Coarctation Aorta
Coarctation Aorta
Before ballooning
Coarctation Aorta
During ballooning
Coarctation Aorta
After ballooning
Tetralogy FallotInsidence
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
CXR : Boot-shapedConcave pulmonary
segmentApex upturnedDecreased 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 maleBirth weight usually normal or biggerCyanotic vary from mild to severeAuscultation : single 2nd HS and loudMurmur vary from silent to pansystolic murmur or continuous murmur
Transposition of Great artery
• DiagnosisClinically :
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
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 mths3 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 ArteriosusInsidence
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 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
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
• 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 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 EnlargedThe PVMs are DecreasedThe 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