Advance in

Diagnostic and Noormanto

Departemen Ilmu
Management for Kesehatan Anak FK KMK
UGM/ RS Dr. Sardjito

Congenital Heart Yogyakarta

Diseases
 Anatomy-Physiology

Classification of Pediatric Heart

Outline Disease

Pathophysiology, clinical
presentation and management of
VSD, PDA, ASD and ToF
Anatomy- Physiology
Systolic-Diastolic
Ao

AP
PARU2
LA
RA

RV LV
Diastolic
/80

/16

8
6

/8
/6
Systolic

120 /80

24/16

8
6

120/ 8
24/ 6
 Acquisitions

• ARF – CRF/ RHD

• SBE, etc.

Congenital
Classification
of Pediatric • Non cyanotic heart disease:
• VSD
Heart Disease • ASD
• PDA
• Cyanotic heart disease:
• Tetralogy of Fallot (ToF)
• Pulmonary atresia
• Transposition of the Great Arteries
Ventricular Septal Defect
(VSD)
• Prevalence
• 15 – 20 % all CHD
• Anatomy
• Subarterial defect : below pulmonary and aortic
valve
• Perimembranous defect: below aortic valve at pars
membranous septum
• Muscular defect
Pathophysiology of VSD
• Defect  L-R shunt
• Systolic Pressure gradient (LV and RV) 120 / 80
 Pan-systolic murmur
• Impact on left heart  SV↓ 24 / 16
(COP = SV x HR)  tachycardia 
tachypnea  volume overload (Frank 8
Starling effect) pulmonary edema 6
 dyspnea 120 / 8

Symptom: 24 / 6
• Tachycardia  palpitation, sweating
• Tachypnea, fatigue, feeding difficulty
• Dyspnea
• Failure to grow
Pathophysiology of VSD
• Defect  L-R shunt
120 / 80
• Impact on right heart:
pulmonary overload  dilatation 24 / 16
pulmonary arteria and capillary
vasoconstriction  pulmonary 8
hypertensions
6
120 / 8
Symptom:
• Recurrent pneumonia 24 / 6
• Shortness breath
Clinical sign:
• S2 ↑ (if pulmonary hypertensions)
 • Asymptomatic - symptomatic
• Tachycardia  palpitation, sweating
• Tachypnea, fatigue, feeding difficulty
Clinical • Dyspnea
manifestations • Shortness breath
• Recurrent pneumonia
• Failure to grow
• Pan-systolic murmur  LLSB
 Small VSD  normal

Moderate VSD

• LA enlargement
Electrocardiography • LVH

Large VSD

• LA enlargement
• LVH and RVH
• RVH
 • Cardiomegaly
• Apex downward
Chest x-ray • Prominent PA
• Increase pulmonary vascular marking
 3

a
b
c 2

CTR = a+b/c

Cardiomegaly if CTR > 0.5

12/21/2019 17
• Echocardiography
• Type, position and size of the
defect
• Shunt direction
• Enlargement of the chambers
• Pressure calculation
 Management

General  supportive

Medical

• Congestive Heart Failure

• Vasodilator (captopril, bisoprolol)
• Diuretic (furosemide, spironolactone)
• Pulmonary hypertension
• Sildenafil
Principle medical treatment

Reduce Stroke Volume

Reduce renal blood flow Baroreceptor

Activation of RAS Activation of SNS

Renin
Bisoprolol

Angiotensin I Heart rate 

ACi

Angiotensin II Peripheral vasoconstriction

Aldosteron secretion

Na and water retension Diuretic

Preload  Afterload 
 VSD closure
Management
Definitive
•Transcatheter closure
•Surgery
Transcatheter VSD closure
 PDA

Patent Ductus
Arteriosus (PDA)
• Pathology
• Persistent patency of ductus
arteriosus ( a normal fetal
structure between the LPA
and the descending aorta)
• Risk Factor
• Premature
• Asphyxia
• Lung diseases
• Infusion of PGE1
 • 5 – 10% all CHD
Prevalence • Male : female = 1 : 3
Pathophysiology of PDA
• Defect  L-R shunt
• Continuous murmur depend on 120 / 80
diameter, location?
• Impact on left heart  SV↓ 24 / 16
(COP = SV x HR)  tachycardia 
tachypnea  volume overload (Frank 8
Starling effect) pulmonary edema 6
 dyspnea 120 / 8

Symptom: 24 / 6
• palpitation
• shortness of breath
• Feeding difficulty
• sweating
PDA
• Defect  L-R shunt
120 / 80
• Impact on right heart:
pulmonary overload  dilatation 24 / 16
pulmonary arteria and capillary
vasoconstriction  pulmonary 8
hypertensions
6
120 / 8
Symptom:
• Recurrent pneumonia 24 / 6
• Shortness breath
Clinical sign:
• S2 ↑ (if pulmonary hypertensions)
 • Asymtomatic - symtomatic
• Recurrent pneumonia
• Poor weight gain
• Tachycardia  palpitation, sweating
Clinical • Tachypnea, fatigue, feeding difficulty
manifestations • Dyspnea
• Shortness breath
• Bounding peripheral pulses (pistol shoot sign)
• Continuous murmur
 Small PDA  normal

Moderate PDA  LA
Echocardiography enlargement and LVH

Large PDA  RVH and LVH

Chest X-ray ∾ VSD

• Cardiomegaly
• Apex downward
• Prominent PA
• Increase pulmonary vascular marking
• Echocardiography
• Type, position and size of the defect
• Shunt direction
• Enlargement of the chambers
• Valves
• Pressure
Management

Indomethacin in premature infant

Transcatheter closure/ non surgical closure:

• Coil embolization
• Amplatzer ductal occluder (ADO)

Surgical closure
Transcatheter PDA closure
Atrial Septal Defect
(ASD)
• Prevalence
•5 – 10 % all CHD
•Male : female = 1 : 2
• Anatomy :
 Secundum ASD: defect on foramen ovale
Sinus venosus ASD: defect at SVC and RA
junction
Primum ASD: defect at ostium primum
Pathophysiology
• Defect  R-L shunt
• LA – RA shunt  gradient 2 mmHg
no heart murmur heard on IAS defect
• Volume overload in RV  relative
stenosis in PA  murmur, late closing
of S2  splitting fixed S2 8
• pulmonary overload  dilatation
pulmonary arteria and capillary
vasoconstriction  pulmonary 6
hypertensions

Clinical symptom:
• Recurrent lower respiratory tract
infection
• Delayed growth
 Usually asymtomatic (infant and children)

Recurrent lower respiratory tract infection (LRTI)

Delayed growth and development

Clinical
manifestations A Widely split and fixed S2

Ejection systolic murmur

Mid diastolic rumble

Electrocardiography

• Small ASD  normal

• Moderate- large ASD
• RAD
• RVH - RBBB
Chest X-Ray
• Cardiomegaly
• Prominent PA
• Increased pulmonary vascular
markings
Trans-thorax Echocardiography
• Type, position and size of the
defect
• Shunt direction
• Enlargement of the chambers
• Valves
• Pressure calculations
Trans-esophagus Echocardiography
• Type, position and size of the
defect
• Shunt direction
• Enlargement of the chambers
• Valves
• Pressure calculations
 Medical :
• Congestive heart failure (CHF)
• Pulmonary hypertension
Management Non surgical closure:
• Transcatheter closure using
device (ASO, lifetech,
Memopart, occlutect)
• Timing
Secundum ASD
Transcatheter ASD closure
Tetralogy of Fallot (ToF)

Pulmonary stenosis

Right ventricle hypertrophy

VSD
1
Overriding Ao
4
3

K. Baumgartner, Freiburg 1839

A boy with congenital heart disease
 Insidence
5-8% from all CHD

ToF Anatomy embryology:

Cause: Left-anterior deviation of infundibular
septum
Pathophysiology
• Pulmonary stenosis  ejection
systolic murmur  audible at birth SpO2 <95%

• Shunt R to L :
• No heart murmur (on IVS defect)
• Aortic desaturation
• Cyanosis

• Clinical symptom:
• Dyspnea on exertions 120/80
• Clubbing finger 120/8
• Squatting 120/6
SpO2 >95%

• History of hyper-cyanotic spell SpO2 70%

• Single heart sound (S2)

 Cyanosis
Dyspnea on exertion
Clubbing finger
Clinical
Squatting
manifestations
History of hyper-cyanotic spell
Single 2nd HS (heart sound)
Ejection systolic murmur
Electrocardiography

• RAD
• RVH
Chest x-ray
• Boot-shaped
• Concave pulmonary segment
• Apex upturned
• Decreased pulmonary blood flow
Echocardiography
Long axis view

• Type, position and size of

the defect
• Shunt direction
• Enlargement of the
chambers
• Pulmonary Stenosis (PS),
dimension PAs branch
• Pressure calculations
 • Medical
• Propranolol
• Paliative treatment
• Blalock-Taussig shunt

Management • Definitive
• Total correction, the basis of mortality and physiological
outcome  optimal age for repair 3-11 months*

*Van Arsdell GS et al, 2000

BT shunt
Difinitive
Pulmonary atresia
• Cyanotic at birth  degree of cyanosis
depend on PDA/collateral  discharges
• Duct dependent  heart murmur cannot
be heard  continuous murmur 
discharges
• To maintain PDA still open 
Prostaglandin/ PDA stenting /BT shunt
before surgical procedure
Transposition of the
Great Arteries (TGA)
• History of cyanosis from birth
• Dyspnea and feeding difficulties
• Moderate - severe hypoxia
• No heart murmur
• Parallel circulation  need adequate interatrial
communication  BAS/ PDA stenting