Central defect in the atrio-ventricular septum. Typically one defect. The portion above the atrio-ventricular valves is often referred to as the "primum ASD" and the portion below the valves as the VSD.
There is a single, complex atrio-ventricular valve spanning the area of the defect rather than the two separate valves guarding two separate atrio-ventricular openings.
In addition to rebuilding the central septum of the heart with a patch, the single atrio-ventricular orifice and its valve are separated into right and left components and valve reconstruction involves attaching these valves to the patch and typically closing the cleft of the left-sided atrio-ventricular valve to reduce regurgitation or leakage.