Principle of Congenital Heart Diseases

PRINCIPLE OF
CONGENITAL HEART
DISEASES (CHD)
Noormanto
Department of Pediatric
University of Gadjah Mada
Yogyakarta/ Sardjito General
Hosptal
03/05/16
03/05/16
03/05/16
Dimensions of the cardiac chambers

The RV is larger than the LV (RV handles
55%, LV handles 45%)
The pressure in the RV is identical to that
in the LV.
Fetal cardiac output

Low compliance of the heart unable to
increase stroke volume.
If the heart rate drops fall cardiac
output fetal distress (COP = SV x HR)
03/05/16
After birth:
Interupted of the umbilical cord
Lack of blood return from the placenta

Increase in systemic vascular resistance
Lung expansion
Decrease of the PVR fall PA pressure
increase pulmonary blood flow
Increase pulmonary venous return increase
of the LA pressure functional closure of the
foramen ovale
Increase of arterial O2 saturation PDA closed
03/05/16
Closure of the ductus arteriosus

Functional : 10 15 hours after birth
Anatomic closure complete 2 -3 weeks
Increase of O2 saturation constriction of the
ductal smooth muscle
The responsiveness of the ductal smooth muscle

depend on gestational age (full term more
responsiveness than preterm)
Decrease in PGE2 level after birth constriction of

the duct
Indomethacin (prostaglandine synthetase inhibitor)
can be used to close PDA in preterm infants.
03/05/16
Diagram of normal heart

SVC
110/80
02:75%
PA
AO
25/
15
LA
6
O2:75%
RA
5
RV
lung
O2:96%
LV
110/6
25/5
IVC
03/05/16
SVC/IVC= superior/inferior vena cava

AO= aorta; PA=pulmonary artery
LA=left atrium; RA=right atrium
LV=left ventricle; RV=right ventricle
Classification of CHD
NON CYANOTIC:
Increase pulmonary blood flow
ASD (Atrial Septal Defect)

VSD (Ventricular septal defect)
PDA (Patent Ductus Arteriosus)
Normal pumonary blood flow
Pulmonary stenosis (PS)
CYANOTIC:
Decrease pulmonary blood flow
ToF (Tetralogy of Fallot)

Pulmonary Atresia (PA)
TGA (Transposition of the Great Arteries)

TAPVD (Total Anomalous Pulmonary Venous Drainage)
03/05/16
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
03/05/16
TYPE OF ASD
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10
Pathophysiology
LA
RA
LV
RV
03/05/16
11
VC
PV
LA
RA
LV
RV
PA
AO
03/05/16
12
Clinical manifestations:
Usually asymtomatic (infant and children)

Recurrent lower respiratory tract infection (LRTI)
Delayed growth and development
A Widely split and fixed S2
Ejection systolic murmur
Mid diastolic rumble
Electocardigraphy:
RAD (right axis deviation)

RVH (right ventricular hypertrophy)
03/05/16
13
X-ray
Cardiomegaly
Prominent PA
Increased pulmonary vascular markings
Echocardiography
Type, position and size of the defect

Shunt direction
Enlargement of the chambers
Valves
Pressure
03/05/16
14
Chest X-Ray
Right atrial enlargement

Right ventricle hypertrophy
Prominence the MPA
segment
Increased pulmonary
vascular marking
Management
Medical :
congestive heart failure (CHF) anti failure
Surgery :
Preschool age
Non surgical closure:

Transcatheter closure using ASO (Amplatzer Septal
Occluder)
03/05/16
16
Atrial Septal De
Transcatheter closure using ASO

ASO DEVICE
Atrial Septal De
03/05/16
18
03/05/16
19
03/05/16
20
NON CYANOTIC:
CYANOTIC:


03/05/16
21
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
03/05/16
22
Ventricular Septal D
03/05/16
23
COP = SV x HR
LA
RA
110/8
25/6
RV
03/05/16
LV
24
PVR = pulmonary vascular resistant

RVP = right ventricular pressure
LVH/RVH = left/right ventricular pressure
03/05/16
25
Clinical manifestation
Asymtomatic symtomatic
Recurrent LRTI
Deleyed growth and development
Sign of CHF
Pansystolic murmur
Electrocardiography
LVH, combine LVH and RVH
03/05/16
26
X-ray
Cardiomegaly
Prominent PA
Increase pulmonary vascular marking
Echocardiography

Shunt direction
Pressure calculation
03/05/16
27
3
1. Cardiomegaly
2. Apex down ward
3. Prominence pulmonary
artery segment
4. Increased pulmonary vascu
marking
a
b
c
CTR = a+b/c
Cardiomegaly if CTR > 0.5
03/05/16
28
Management
Medical
CHF antifailure
Definitive
VSD closure
Surgery
Transcatheter closure
03/05/16
29
Transcatheter closure of Perimembranous VSD
03/05/16
30
NON CYANOTIC:

CYANOTIC:


03/05/16
31
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
03/05/16
32
Patent Dudtus Arter
110/80
COP = SV x HR
25/15
LA
RA
LV
RV
03/05/16
33
VC
PV
LA
RA
RV
LV
PA
PDA
AO
03/05/16
34
Prevalence
5 10% all CHD
Male : female = 1 : 3
Clinical manifestations
Asymtomatic - symtomatic
Recurrent LRTI
Poor weight gain
Sign of CHF
Bounding peripheral pulses (pistol shoot sign)
Continour murmur
03/05/16
35
Electrocardiography = VSD
Chest X-ray = VSD
Echocardiography

Shunt direction
Valves
Pressure
03/05/16
36
Management
Indomethacin in premature infant

Transcatheter closure/ non surgical
closure:
Coil embolization
Amplatzer ductal occluder (ADO)
Surgical closure
03/05/16
37
Transcatheter PDA
occlusions
Rashkind umbrella device

Stainless steel coils
Amplatzed ductal occluder
03/05/16
38
03/05/16
39
03/05/16
40
Patent Dudtus Arter
Gianturco coils
03/05/16
41
Patent Dudtus Arter
Amplatzer Ductal
Occluder (ADO)
03/05/16
42
Large PDA
03/05/16
Patent Dudtus Arter
43
03/05/16
44
03/05/16
45
Patent Dudtus Arter
03/05/16
46
NON CYANOTIC:


CYANOTIC:


03/05/16
47
Pulmonary Stenosis
Incidence : 8-10%
Anatomy:
Pulmonary stenosis valvular :
Bicuspid pulmonary valve
Valve leaflet thickening and adhession
Pulmonary stenosis infundibular

Hyperthropy infundibulum
03/05/16
48
Clinical findings
Valvular stenosis
Mild :
Wide 2nd HS, ejection click
Moderate:ejection systolic murmur, early systolic click
Severe : ejection systolic murmur, ejection click (-)
Stenosis infundibular
Ejection click ( - )
1st HS normal, 2nd HS weak, ejection systolic murmur
Pulmonary stenosis periphery
1st & 2nd HS normal, ejection systolic murmur
03/05/16
49
Pulmonary Sten
Mild
: ejection systolic murmur

2nd HS wide split
ejection click
Moderate: ejection systolic murmur , early ejection click
Severe : ejection systolic murmur, click ejection (-)
03/05/16
50
Pulmonary Sten
Normal or mild
cardiomegaly
Marked
pulmonary valve
post stenotic
dilatation
Normal
pulmonary
vascularity
03/05/16
51
ECG : RAD, RVH

Echocardiograhhy : confirmation diagnosis
Catheterization: increased RV pressure
without increased oxygen saturation
03/05/16
52
Management
Medicamentosa : useless
Mild stenosis: intervention (-)
Moderate stenosis: observation
Severe stenosis: balloon valvuloplasty
03/05/16
53
Pulmonary Sten
03/05/16
54
Before ballooning
03/05/16
Pulmonary Sten
55
During ballooning
03/05/16
Pulmonary Sten
56
NON CYANOTIC:

CYANOTIC:

03/05/16
57
Tetralogy of Fallot
K. Baumgartner,
Freiburg 1839
A boy with congenital
heart disease
03/05/16
58
Tetralogy of Fa
Insidence
5-8% from all CHD
Anatomy
embryology:
Cause: Left-anterior
deviation of
infundibular septum
LA
1
RA
Sindroma consist of
4 items:
1.
2.
3.
4.
pulmonary stenosis
VSD
aortic over-riding
RVH
03/05/16
LV
RV
59
Tetralogy of Fa
Hemodynamic cyanotic
Hemodynamic acyanotic
(Pink of ToF)
Infundibular PS
forces the R to L shunt across VSD
cyanosis
03/05/16
60
Clinically :
Cyanosis
Dyspnea on exertion
Squatting
History of hyper-cyanotic spell
Clubbing finger
Single 2nd HS (heart sound)
03/05/16
61
Single 2nd HS
03/05/16
62
Tetralogy of Fa
CXR :
Boot-shaped
Concave
pulmonary
segment
Apex upturned
Decreased
pulmonary
blood flow
03/05/16
63
Electrocardiography
RAD
RVH
Echocardiography
To confirm diagnosis
Prepare to surgery (exchange to
catheterization)
Pulmonary stenosis
VSD
Aortic over-riding
RVH
03/05/16
64
Management
Paliative treatment
Blalock-Taussig shunt
Definitive
total correction, the basis of
mortality and physiological
outcome optimal age for repair
3-11 months*
03/05/16
*Van Arsdell GS et al, 2000
65
Tetralogy of Fa
BT
03/05/16
66
Tetralogy of Fa
03/05/16
67
NON CYANOTIC:

CYANOTIC:

03/05/16
68
Transposition of The
Great Artery
Anatomy
Abnormality of formation of trunkal
septum that cause aorta arising from
RV and PA arising from LV
03/05/16
69
Transposition of The Great

Grea
With VSD
03/05/16
Without VSD (Simple TGA)
70
PFO
Hemodynamic normal
series
03/05/16
Hemodynamic of TGA
parallel
71
In adequate Mixing
Adequate Mixing
TGA without VSD

03/05/16
72

Grea
CXR :
Cardiomegaly
Egg-on-side
heart
Increased
pulmonary
vascular
marking
03/05/16
73
Management
Balloon atrial septostomy

Surgery: arterial switch
03/05/16
74
Balloon atrial septostomy
03/05/16
75

Grea
03/05/16
76

Principle of Congenital Heart Diseases

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Principle of Congenital Heart Diseases

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PRINCIPLE OF

Dimensions of the cardiac chambers

Fetal cardiac output

Lack of blood return from the placenta

Closure of the ductus arteriosus

The responsiveness of the ductal smooth muscle

Decrease in PGE2 level after birth constriction of

Diagram of normal heart

SVC/IVC= superior/inferior vena cava

ASD (Atrial Septal Defect)

Normal pumonary blood flow

Pulmonary stenosis (PS)

ToF (Tetralogy of Fallot)

Increase pulmonary blood flow

TGA (Transposition of the Great Arteries)

Atrial Septal Defect

Usually asymtomatic (infant and children)

RAD (right axis deviation)

Type, position and size of the defect

Right atrial enlargement

Non surgical closure:

Transcatheter closure using ASO

ASD (Atrial Septal Defect)

VSD (Ventricular septal defect)

PDA (Patent Ductus Arteriosus)

Normal pumonary blood flow

Pulmonary stenosis (PS)

ToF (Tetralogy of Fallot)

Increase pulmonary blood flow

TGA (Transposition of the Great Arteries)

Ventricular Septal Defect

PVR = pulmonary vascular resistant

Type, position and size of the defect

Transcatheter closure of Perimembranous VSD

ASD (Atrial Septal Defect)

PDA (Patent Ductus Arteriosus)

Normal pumonary blood flow

Pulmonary stenosis (PS)

ToF (Tetralogy of Fallot)

Increase pulmonary blood flow

TGA (Transposition of the Great Arteries)

Patent Ductus Arteriosus

Patent Dudtus Arter

Type, position and size of the defect

Indomethacin in premature infant

Rashkind umbrella device

Patent Dudtus Arter

Patent Dudtus Arter

Patent Dudtus Arter

Patent Dudtus Arter

ASD (Atrial Septal Defect)

Normal pumonary blood flow

Pulmonary stenosis (PS)

Decrease pulmonary blood flow

ToF (Tetralogy of Fallot)

Increase pulmonary blood flow

TGA (Transposition of the Great Arteries)

Pulmonary stenosis infundibular

: ejection systolic murmur

ECG : RAD, RVH

ASD (Atrial Septal Defect)

Normal pumonary blood flow

Pulmonary stenosis (PS)

ToF (Tetralogy of Fallot)