Double-Outlet Left Ventricle With Leftward Anterior Aorta in Infants

Among the conotruncal abnormalities, double-outlet left ventricle (DOLV) has been considered an embryologic conundrum, and no more than 200 cases have been described since 1975. DOLV refers to anomalous ventriculoarterial connections with the great arteries arising entirely or predominantly from the left ventricle. It represents a heterogenous group of heart defects considering embryology, morphology, and hemodynamics. DOLV can be detected prenatally or postnatally by transthoracic echocardiography.

This study presents a case series of 3 newborns with DOLV and a leftward anterior aorta. The neonates showed symptoms of hypoxia and cardiac compromise after birth and required emergency interventional and surgical procedures.

The presented cases contribute interesting insights into the variability of DOLV, and they show the importance of multimodality imaging with echocardiography, computed tomography, and cardiac catheterization.

Complex heart defects coexisting with DOLV require precise visualization before surgical or interventional procedures (eg, a single ventricular vs a biventricular solution).