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

ABSTRACT:

Background

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.

Case summary

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 physiolo

SUBMITTER: Arenas-Fabbri V 

PROVIDER: S-EPMC12441456 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature