ATRIAL SEPTAL DEFECT

DEFINITION

Atrial septal defect is an abnormal communication between the atria due to defect
in the interatrial septum.it allowing the blood from high pressure left atrium to low
pressure right atrium .

INCIDENCE

 ASDs constitute 8% to 10% of congenital heart defects in children.

 The recent incidence of ASDs has been estimated to be 100 per 100,000
live births.
 The female :male ratio for secundum ASDs is 2:1, but for the sinus venosus
ASDs it is 1:1
 About 30% to 50% of children with congenital heart defects have ASDs as
part of the cardiac defect.

EPIDEMIOLOGY

The majority of cases of ASDs are sporadic.

Autosomal dominant inheritance does as part of the

 Holt-oram syndrome (hypoplastic or absent thumbs, radii, triphalangism,

phocomelia, 1st-degree heart block. ASDs)or in
 Families with secundum ASD and heart block

TYPES OF ASD

1. Ostium secundum ASD(75%)

2. Ostium primum ASD(20%)
3. Sinus venosus ASD(5%)

1. Ostium secundum ASD-

Location –
 This defect is present at the site of fossa ovalis.
  Secundum ASDs may be single or multiple ( fenestrated atrial
septum)
 This is the most common defect and around seventy percent of the
children who are diagnosed with atrial septal defect, suffer from this
particular condition. This type of defect arises during the pregnancy
period, when the atrial septum is unable to close at the time of
development of the heart. It leads to the formation of an opening in
the septum

2.Ostium Primum Atrial Septal Defect: It is a type of atrioventricular canal defect

and hence it is linked with the presence of a hole in one of the leaflets present in
the mitral valve.

3.Sinus Venosus Atrial Septal Defect: This type of defect arises due to the
presence of a hole at the junction of superior vena-cava and the right atria, at the
point where the pulmonary veins enters into the heart. As a consequence of which,
blood drains out of the pulmonary vein from the right atria towards the left atria.

PATHOPHYSIOLOGY

In moderate to large defect a considerable shunt of oxygenation blood flows

from the left to right atrium

Volume overload and dilation of the right atrium and ventricle

The tricuspid and pulmonary annuli may dilate and become incompetent

Increased flow into lungs

 Pulmonary arteries, capillaries and veins are dilated

Flow – related pulmonary artery hypertension

Medial hypertrophy of pulmonary arteries and muscularization of the

arterioles resulting in pulmonary vascular obstructive disease

Reversal of the shunt

Eisenmerger syndrome

CAUSES

Down syndrome – patients with Down syndrome have higher rates of ASDs,
especially a particular type that involves the ventricular wall. As many as one half
of Down syndrome patients have some type of septal defect.

Ebstein's anomaly – about 50% of individuals with Ebstein anomaly have an

associated shunt between the right and left atria, either an atrial septal defect or a
patent foramen ovale.

Fetal alcohol syndrome – about one in four patients with fetal alcohol syndrome
has either an ASD or a ventricular septal defect.

Holt–Oram syndrome – both the osteium secundum and osteum primum types of
ASD are associated with Holt–Oram syndrome. Holt-Oram syndrome is
characterized by skeletal abnormalities of the hands and arms (upper limbs) and
heart problems. People with Holt-Oram syndrome have abnormally developed
bones in their upper limbs. At least one abnormality in the bones of the wrist
(carpal bones) is present in affected individuals .
Lutembacher's syndrome – the presence of a congenital ASD along with
acquired mitral stenosis.