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ATRIAL SEPTAL DEFECT

Etiology and Epidemiology


During the embryologic development of the heart, a septum grows toward the endocardial
cushions to divide the atria. Failure of septal growth or excessive reabsorption of tissue leads
to ASDs . ASDs represent approximately 10% of all congenital heart defects. A secundum
defect, with the hole in the region of the foramen ovale, is the most common ASD. A
primum ASD, located near the endocardial cushions, may be part of a complete
atrioventricular canal defect or may be present with an intact ventricular septum. The least
common ASD is the sinus venosus defect, which may be associated with anomalous
pulmonary venous return.
Syndromes associated with ASD

Ellis-vald- creveld
Holt-oram Syndrome
Noonan syndrome
Down Syndrome

Clinical Manifestations
Regardless of the site of the ASD, the pathophysiology and amount of shunting depend on the
size of the defect and the relative compliance of the both ventricles. Even with large ASDs
and significant shunts, infants and children are rarely symptomatic. A prominent right
ventricular impulse at the left lower sternal border (LLSB) often can be palpated. A soft
(grade I or II) systolic ejection murmur in the region of the right ventricular outflow tract
and a fixed split S2 (due to overload of the right ventricle with prolonged ejection into the
pulmonary circuit) are often audible. A larger shunt may result in a mid-diastolic murmur at
the left lower sternal border as a result of the increased volume passing across the tricuspid
valve.

Imaging Studies
ECG and chest x-ray findings reflect the increased blood flow through the right atrium, right
ventricle, pulmonary arteries, and lungs. The ECG may show right axis deviation and right
ventricular enlargement. A chest radiograph may show cardiomegaly, right atrial
enlargement, and a prominent pulmonary artery.
Treatment
Medical management is rarely indicated. If a significant shunt is still present at around 3
years of age, closure is usually recommended. Many secundum ASDs can be closed with an
ASD closure device in the catheterization laboratory. Primum and sinus venosus defects
require surgical closure.
Surgical or transcatheter device closure is advised for all symptomatic patients and also for
asymptomatic patients with a Qp:Qs ratio of at least 2:1 or those with right ventricular
enlargement. The timing for elective closure is usually after the 1st yr and before entry into
school. Closure carried out at open heart surgery is associated with a mortality rate of <1%.

Repair is preferred during early childhood because surgical mortality and morbidity are
significantly greater in adulthood; the long-term risk of arrhythmia is also greater after ASD
repair in adults. For most patients, the procedure of choice is percutaneous catheter device
closure using an atrial septal occlusion device, implanted transvenously in the cardiac
catheterization laboratory . The results are excellent and patients are discharged the following
day. With the latest generation of devices, the incidence of serious complications such as
device erosion is 0.1% and can be decreased by identifying high-risk patients such as those
with a deficient rim of septum around the device. Echocardiography can usually determine
whether a patient is a good candidate for device closure. In patients with small secundum
ASDs and minimal left-to-right shunts without right ventricular enlargement, the consensus is
that closure is not required. It is unclear at present whether the persistence of a small ASD
into adulthood increases the risk for stroke enough to warrant prophylactic closure of all these
defects.

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