Atrial septal defect ostium primum
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [[2]]; Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [[4]]
Overview
During fetal development, the septal wall may fail to fuse causing an atrial septal defect to arise. An ostium primum atrial septal defect is one such type of malformation arising from the irregular development of the septum and tricuspid and mitral valves.
Ostium Primum Atrial Septal Defect
The ostium primum atrial septal defect (also known as an endocardial cushion defect) is a defect in the atrial septum at the level of the tricuspid and mitral valves. This is sometimes known as an endocardial cushion defect because it often involves the endocardial cushion, which is the portion of the heart where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid valve.
Endocardial cushion defects are associated with abnormalities of the atrioventricular valves (the mitral valve and the tricuspid valve). These include the cleft mitral valve, and the single atrioventricular valve (a single large, deformed valve that flows into both the right ventricle and the left ventricle).
Endocardial cushion defects are the most common congenital heart defect that is associated with Down's syndrome.