Double outlet right ventricle

Abstract

Double-outlet right ventricle is a complex congenital heart defect consisting of anomalous ventriculo-arterial connections with several “phenotypes” and associations. It is a conotruncal anomaly with a frequency of 0.5-1.5 % among all congenital cardiac defects and with a wide spectrum of anatomic forms depending on the location of the ventricular septal defect, the relationship between the great vessels and the ventricular cavities, and the presence of pulmonary outflow obstruction. This chapter discusses anatomical and functional classifications, pathophysiology, clinical presentation, and medical and surgical management of the double-outlet right ventricle.