Double-Outlet Right Ventricle in an Adult With a Univentricular Heart and Total Situs Inversus

Double-outlet right ventricle is a complex conotruncal anomaly in which both great arteries arise predominantly from the right ventricle. The coexistence of total situs inversus and univentricular physiology is exceedingly rare.

A 38-year-old man presented with severe chronic cyanosis (baseline oxygen saturation 65%), NYHA functional class III dyspnea, and right-sided heart failure. Multimodal imaging (echocardiography, CT, and cardiac magnetic resonance) confirmed total situs inversus, a markedly hypoplastic left ventricle anatomically isolated by a muscular ridge, and double-outlet right ventricle with moderate subpulmonary stenosis. His condition was stabilized medically, but surgical risk was deemed prohibitive owing to his univentricular physiology.

This case underscores the critical importance of comprehensive imaging for delineating complex intracardiac anatomy and illustrates how moderate subpulmonary stenosis can temper pulmonary overcirculation, thereby prolonging survival.

Meticulous anatomical definition is essential for managing rare adult congenital heart disease.