A normal heart during development develops a septum and two valves that separate the upper atrium from the lower ventricle and also separates the heart into four chambers but with atrioventricular septal defect this does not occur because the septum has not developed properly. Hence, the primary defect being the failure of formation of the embryonic structure known as the endocardial cushions. These are responsible for separating the central parts of the heart near the tricuspid and mitral valves (AV valves), which separate the atria from the ventricles. Corrective surgery is the primary treatment. Photo by @quinnhanses