Congenital Heart Disease

Mashuri
 Foetal Circulation
Arterial blood leaves the
placenta via the umbilical
vein
This branches and delivers
blood to the IVC by way of
the ductus venosus
Blood then goes into the right
atrium, 30% goes across the
foramen ovale, the rest to the
RV then to PA
Instead of going to the
lungs, 85% goes through the
PDA to the aorta
 Defintion
Structural or functional heart disease that
present at birth. It is not static, there is always a
continuous anatomical or physiological change
Con (together) genitus (birth)
Estimate in live birth range from 4-10.2 per
1000live birth.
 Incidence
The incidence of specific type of CHD varies
from one country to another
Specific aetiology only known 10%
8% genetic
2% environmental (rubella, foetal-alcohol
syndrome
90% Multifactorial inheritance
 Classification

Acynotic Cyanosis
Shunts ( L to R) : TOF
ASD TGA
VSD
Tricuspid atresia
PDA
Truncus
AVSD
Stenosis: TAPVR
AS Ebsteins
PS Single ventricle eg.
Coarctation HLHS[hypoplastic left heart synd]
 Classification
Acyanotic Cyanosis
Increased pulmonary Increased pulmonary blood
blood flow flow
ASD
TAPVR
VSD
PDA TGA
AVSD TAP
ECD
PAPVR Decreased pulmonary blood
Decreased pulmonary flow
blood flow
TOF
AS
PS Tricuspid atresia
Coarctation Truncus
Ebsteins
Single ventricle eg.
HLHS[hypoplastic left heart synd]
 Atrial septal defect
Communication between
atria as a result of the lack of
closure of the foramen ovale
after birth or improper
closure during gestation.
Shunt from LARA
RA, RV, PA, PV
LV normal
 Atrial septal defect
Classification:
Simple
Primum (primary
septa)
Secundum (ovale
fossa)
Sinus venosus

Complex
Atrioventricular
septal defect (AVSD)
 Atrial septal defect

CXR: cardiomegaly (enlarged right ventricle,

right atrium) and pulmonary outflow tract.
 Ventricular Septal Defect
Most common CHD
Incidence of 1/3000
Can be single or multiple
Can be associated with other
congenital heart diseases
(Down syndrome)
Shunt from LVRV
LV, RV, LA, PA, PV
RA is normal
Classification:
70% membranous close to
pulmonary valve and
Pulmonary artery
20% muscular
5% Aortic valve (sub
aortic)
5% near junction of Mitral
and tricuspid valve (A-V
canal defect)
 Chest X-Ray (VSD)

Cardiomegally
(LA, LV,RV,PA)

Increased vascular
marking
 Patent Ductus Arteriosus
During foetal life blood from PA
shunted through the DA in to the
Aorta.
After birth closed functionally
Prematurity and hypoxia
predispose for patency
Female are more affected than
males Commonly associated with
rubella of the mother
Isolated PDAs are common in
high altitude
 X-ray finding in PDA: enlarged
pulmonary artery, left ventricle,
pulmonary plethore. The aortic
arch prominent
 Pulmonary Stenosis
Congenital fusion of pulmonary valve cups, I.e. congenital
valvular pulmonary stenosis.
Congenital excessive hypertrophy of the muscles
surrounding the outflow tract of the right ventricle (I.e. the
infundibulum) below the level of the pulmonary valve, i.e.
congenital infundibular pulmonary stenosis.
 X-ray

Pulmonary oligemia
occurs in moderate and
severe cases and results
in reduced pulmonary
vascular markings.
Right ventricular
enlargement is
proportional to the
severity of the stenosis.
Right atrial enlargement
may also occur.
 If the stenosis is
valvular, the jet of blood
coming out of the
narrow orifice hits
against the pulmonary
artery wall and causes
its weakening and
dilatation. This is post-
stenotic dilatation of
the pulmonary artery.
 Coarctation of The Aorta

Coarctation of the aorta is narrowing of

the aorta usually at the junction of the
arch with the descending aorta just
distal to the left subclavian artery. B
Because of the narrowing, pressure
rises in the ascending aorta and the
aortic arch and its branches.
 Anastomosis forms between the branches of he
aorta proximal and distal to the obstruction. The
most important of these connect the subclavian
artery through its internal mammary branch to the
intercostal arteries which arise from descending
aorta.
The intercostal arteries become enlarged and
tortuous and erode the lower border of the fourth to
ninth ribs causing rib notching. Appreciable
anastomosis develops gradually by time. That is why
rib notching is not detectable except after the age of
10.
 Other
anastomosis
develops around
the scapula and
another connects
the superior and
inferior epigasric
arteries
1. Signs of left ventricular
hypertrophy.

2. Large and high aortic arch.

On the left cardiac border
two convexities are
present: The first is due to
dilated aortic arch and is
followed by another due to
post-stenotic dilatation of
the aorta. This results in
the characteristic 3 sign
(Fig. 97).
 Rib notching is the most
specific sign (Figs. 98 & 99)
and it occurs in the fourth to
the tenth ribs and appears
by the age of 10.
Fig. (98): X-ray of coarctation of aorta showing Fig. (99): Enlarged view of the
rib notching starting from the 4th rib. The left ribs showing notching of their
border of the heart shows the 3 sign. lower borders
 Tetralogy of Fallot

Common cyanotic
congenital cardiac anomaly
Four anatomical
components of TOF:
VSD
Overriding Aorta
Right ventricular outflow
obstruction
Right ventricular
hypertrophy
 1. Severe pulmonary stenosis which causes right
ventricular hypertrophy. The pulmonary stenosis is
usually infundibular but sometimes it is both valvular and
infundibular.
2. Large ventricular septal defect which makes the pressure
equal in both ventricles.
3. The origin of the aorta is abnormally deviated to the right,
i.e. dextroposed (dextro = right) so that it lies partly over
the right ventricle, i.e. the aorta over-rides both ventricles.
Due to the severe pulmonary stenosis and the large
ventricular septal defect, the pressure in both ventricles is
equal. There is no left to right shunt. On the contrary, part
of the blood pumped by the right ventricle passes into the
aorta causing its enlargement and central cyanosis.
 Chest x ray (TOF)

Normal sized, boot

shaped heart.

Reduced pulmonary
vascular marking
(oligemic depend on the
degree of RVOT
obstruction.
 1. Right ventricular hypertrophy causes the apex to be
displaced outwards and becomes separated from the
diaphragm.

2. The aorta receives blood from both ventricles and the

aortic arch is enlarged. Sometimes the aortic arch is
directed to the right i.e. right-sided aortic arch (Fig. 102).

3. The pulmonary artery and its branches are diminished in

size due to the pulmonary stenosis (pulmonary oligemia).
There is a concavity in the region of the underdeveloped
RV outflow tract.

All the above factors result in a characteristic cardiac

shadow, i.e. coeur en sabot (Sabot = wooden shoe).
 Fig. (100): Two cases of Fallots tetrology

Fig. (101): Fallots tetrology with near Fig. (102): Fallots tetrology with right
pulmonary atresia. The aorta is sided aortic arch
markedly enlarged