Arenas-Fabbri Vincenzo, Andrade-Cuellar Elias Noel, Guillot-Castillo Saúl Yair, Monroy-Jiménez Maria Alejandra, Maldonado-Tenesaca Andrea Paulina, Aceves-Millan Rocio, Solis-Gómez Juan Carlos, Robledo-Nolasco Rogelio, de Jesús Encinos-Méndez Diego, Elizalde-Uribe Ivan Alejandro
Department of Cardiovascular Imaging, National Medical Center "November 20th," Institute of Social Security and Services for State Workers, Mexico City, Mexico.
Clinical Cardiology, National Medical Center "November 20th," Institute of Social Security and Services for State Workers, Mexico City, Mexico; Cardiac Electrophysiology, National Medical Center "November 20th," Institute of Social Security and Services for State Workers, Mexico City, Mexico.
JACC Case Rep. 2025 Jul 9;30(18):103958. doi: 10.1016/j.jaccas.2025.103958.
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.
TAKE-HOME MESSAGE: Meticulous anatomical definition is essential for managing rare adult congenital heart disease.
