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Classification of atrial septal defects (ASDs), and clinical features and diagnosis of isolated ASDs in children

G Wesley Vick, III, MD
Louis I Bezold, MD
Section Editor
John K Triedman, MD
Deputy Editor
Carrie Armsby, MD, MPH


Atrial septal defects (ASDs) are common, accounting for approximately 13 percent of congenital heart disorders. The clinical consequences of an ASD are related to the anatomic location of the defect, its size, and the presence or absence of other cardiac anomalies.

The classification of ASDs, and the clinical features and diagnosis of isolated ASDs in children will be reviewed here. The management and prognosis of children with isolated ASDs are discussed separately. (See "Management and outcome of isolated atrial septal defects in children".)


Congenital defects of the atrial septum are common, accounting for approximately 13 percent of congenital heart disorders, with a reported birth prevalence of approximately 2 per 1000 live births [1-4].


Normal development — The septation of the atria begins as early as the fifth week of gestation and involves three structures: septum primum, septum secundum, and the atrioventricular (AV) canal septum, which is made up in part by the superior and inferior endocardial cushion.

The septum primum arises from the superior portion of the common atrium and grows caudally towards the AV canal septum (eg, the endocardial cushions) located between the atria and ventricles. The fusion between the septum primum and the endocardial cushions closes the orifice (ostium primum) separating the right and left atria (figure 1).

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Literature review current through: Nov 2017. | This topic last updated: Oct 26, 2017.
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