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Management and outcome of atrioventricular (AV) canal defects

Craig E Fleishman, MD
Aykut Tugertimur, MD, FACC
Section Editors
John K Triedman, MD
Heidi M Connolly, MD, FASE
Deputy Editor
Carrie Armsby, MD, MPH


Atrioventricular (AV) canal defects are a group of congenital cardiac defects involving, to varying extent, the AV septum and AV valves (ie, mitral and tricuspid valves). They are also referred to as AV septal defects, endocardial cushion defects, or persistent AV ostium.

The management and outcome of patients with AV canal defects will be reviewed here. The anatomy, pathophysiology, clinical features, and diagnosis of AV canal defects are discussed separately. (See "Clinical manifestations, pathophysiology, and diagnosis of atrioventricular (AV) canal defects".)


Failure of the superior and inferior endocardial cushions to fuse correctly during cardiac development results in a broad range of AV septal and valve defects as follows (figure 1 and figure 2):

Complete AV canal defect is a result of complete failure of fusion between endocardial cushions. It is characterized by a primum atrial septal defect (ASD) that is contiguous with a ventricular septal defect (VSD) and a common AV valve.

Partial AV canal defect is due to incomplete fusion of the endocardial cushions and consists of a primum ASD and a common valvular annulus with two separate valve orifices. Due to abnormal fusion of the left tubercle of the superior and inferior cushions, the anterior leaflet of the mitral valve is cleft, which is often associated with mitral valve regurgitation.

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Literature review current through: Nov 2017. | This topic last updated: Feb 09, 2016.
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