JACC. Case reports · 2025
Double-Outlet Right Ventricle in an Adult With a Univentricular Heart and Total Situs Inversus.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
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
Аннотация
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 physiology.
DISCUSSION: 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.
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 physiology.
DISCUSSION: 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.