Seminar congenital cardiac disorders (pda,TA and AP Window)
Patent dustus arteriosus is a congenitalo
disorder in the heart wherein a neonates
ductus arteriosus (blood vessel
connecting pulmonary artery to proximal
descending aorta) fails to close after
DUCTUS ARTERIOSUS is the blood vessel
connecting pulmonary artery to proximal
descending aorta in the fetal circulation
Normally,soon after the birth the ductus
arterteriosus gets closed as a result of
constriction of smooth muscles in its
vessel wall by release of bradykinin.
When it remains open,it results in patent
pulmonary artery pressure drops and
blood with higher pressure from aorta is
shunted to the pulmonary artery
Chest x ray (cardiomegaly)
Pda murmer is heard on physical
NSAIDS such as indomethacin
ibuprofen is given which helps to close
PDA(Pgs are responsible for ductus
patency,NSAIDS act as inhibitors for pgs)
Surgical correction by division and
ligation of patent vessels and done at 12years of age.
Window is the rare(0.1%
of all congenital defects)
congenital defect in which
there is a hole connecting
the a major artery(aorta)
and pulmonary artery.
Aortopulmonary window represents a failure
of the conotruncus to differentiate into the
aorta and pulmonary artery.
No genetic associations or environmental risk
factors are known.
The 2 competing embryologic theories are
› aortopulmonary window is part of a spectrum of
conotruncal abnormalities, which
truncus arteriosus at one end
aortopulmonary window is unrelated to
truncus arteriosus because the lesions
associated with each defect are so dissimilar
Minimal cyanosis present
Symptoms of heart failure appear
during early infancy
The defect is usually large, and the
cardiac murmur is systolic with a middiastolic rumble as a result of increased
blood flow across the mitral valve.
Normally blood flows through PA into lungs
where it picks up oxygen.then blood
travels back to heart by PV
With AP window blood from aorta flows
into pulmonary artery
Large amount of blood flow to pulmonary
artery results in pulmonary hypertension
and heart failure
Electrocardiogram- shows either left ventricular or
Radiographic- shows cardiac enlargement and
prominence of the pulmonary artery and
Echocardiography- shows enlarged left-sided heart
Magnetic resonance angiography (MRA)- can also
be utilized to visualize the defect.
Cardiac catheterization- reveals a left-right shunt at
the level of the pulmonary artery, as well as
hyperkinetic pulmonary hypertension, because the
defect is almost always large.
Selective aortography- injection of contrast medium
into ascending aorta demonstrates the lesion, and
manipulation of the catheter from the main
pulmonary artery directly to the ascending aorta is
Medical therapy is focused on preoperative stabilization.
Surgical correction is the only effective treatment for
aortopulmonary window (APW).
Intravenous prostaglandins (e.g., alprostadil) may be
required to maintain patency of the ductus arteriosus in
patients with interrupted aortic arch in order to provide blood
flow to the lower half of the body.
Digoxin and furosemide are frequently administered to treat
the heart failure and volume overload associated with this
Inotropic agents (e.g., dopamine, dobutamine) may also be
required for infants with significant heart failure and low
cardiac output associated with myocardial dysfunction.
arteriosus is the rare
congenital heart disease in
structure known as truncus
arteriosus fails to properly
divide pulmonary trunk and
truncus arteriosus large
blood vessel leads out of heart
Type I : one pulmonary artery and two
lateral pulmonary arteries
TYPE II :Two posterior or posteriolateral
Vary with age and depend on the level of
pulmonary vascular resistance.
In immediate new born period,
› signs of heart failure usually absent
› murmur and minimal cyanosis
In older infants,
Pulmonary blood flow is torrential
Clinical picture dominated by heart failure
Cyanosis is minimal
Wide pulse pressure and bounding pulses- runoff
blood from the truncus to the pulmonary circulation
› Enlarged heart and the precordium is hyperdynamic
› S2 is loud and single
› A systolic ejection murmur, accompanied by a thrill,
generally audible along the left sterna border
Electrocardiogram- shows right, left or combined
ventricular hypertrophy, cardiac enlargement,
prominent shadow that follows the normal course
of the ascending aorta and aortic knob; the aortic
arch is to the right in 50% of patients.
Echocardiography- shows the large truncal artery
overriding the VSD and the pattern of origin of the
branch pulmonary arteries.
Pulsed and colour Doppler- used to evaluate
truncal valve regurgitation.
Cardiac catheterization- shows left to right shunt at
the ventricular level, with right-left shunting into the
truncus. Angiography reveals the large truncus
arteriosus and more precisely defines the origin of
the pulmonary arteries.
Medical care before surgical repair depends
on clinical presentation.
Most neonates with truncus arteriosus display
some evidence of congestive heart failure;
they are usually treated with digitalis and
Intravenous prostaglandin is often administered
in patients with truncus arteriosus upon
presentation because the differential diagnosis
includes numerous anomalies with ductdependent systemic or pulmonary blood flow.
However, it is beneficial only in patients with
associated interruption of the aortic arch or
Surgical repair shud be done with in 2
months as it is fatal.