Double Outlet Right Ventricle With Laevomalposition Of Aorta

Abstract

Introduction:
Double ouliet right ventricle (DORV) is an uncommon congenital anomaly having several subtypes’. One rare subtype described is its assoc iation with laevomalposition of the aorta. We describe one such case encountered.

Discussion:
DORV (SDL) laevomalposition of the aorta is a rare variety of DORy, thought to result due to the ventricular loop folding normally to the right (d4oop), but the conotruncus twisting to the left (L-malposition). Both subaortic and subpulmonary coni persist and the subpulmonary conus remains poorly expanded resulting in infundibular stenosis. This type of malformation was first described by Birmingham (1893)2 and since then many case reports have appeared in subsequent literature.