Double Outlet Right Ventricle (DORV)

In DORV, the PA and at least 50% of the aorta arising from the RV.  There is almost always a VSD.  Four groups exist, based on the relationship of the VSD to the great arteries.  The VSD can be subaortic, subpulmonary, doubly committed, or noncommitted.  Therefore, DORV encompasses a spectrum of disease that ranges anatomically and physiologically from TOF to TGA.

Repair requires careful consideration of many anatomic variations and a surgical plan must be developed on an individual basis.