Endocardial cushion defect
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2] Synonyms and keywords: Atrioventricular septal defect; atrioventricular canal defect; AV canal defect; AV septal defects; canalis atrioventricularis communis; persistent atrioventricular ostium; abnormal development of endocardial cushions
Overview
Historical Perspective
Classification
Based on the anatomical features and their impact on physiology, endocardial cushion defect may be classified into complete, partial, intermediate, transitional, and intermediate forms.
Pathophysiology
AV canal connects the atria to the ventricles. At four to five weeks of gestation, the superior and inferior endocardial cushions of the common AV canal fuse. Results in the formation of the mitral and tricuspid valve and the AV septum. Any failure of fusion results in endocardial cushion defect. Pathophysiology of endocardial cushion defects depends upon level of left to right shunting and degree of shunting. Incompetent AV valves in endocardial cushion defect results in regurgitation. There is a strong association between AV canal defects and Down syndrome