Developmental disorders

III.4.1.: Double outlet right ventricle (DORV, double outlet right ventricle) – Introduction

Double outlet right ventricle (DORV) is a heterogeneous malformation, a unified definition for this kind of complex heart defect. According to a definition one of the great vessels and the other great vessel at least 50 % in its diameter originates from the right ventricle.

According to other definitions DORV must present with bilateral conus in between the great vessels and the atrioventricular annulus. The muscle mass of the sub arterial cone show high degree of variability. The label has been introduced by Witham for partial transpositions in which both arteries originate from the right ventricle. Its cases have been divided into various sub categories by Eseteit Fallot and Eisenmenger according to the presence or absence of associated pulmonary stenosis. The former clinically manifest in cyanosis. According to Van Praagh unilateral conus may also appear. Neufeld et al. classified DORV by not only based on the absence or presence of pulmonary stenosis but also according to the localization of VSD.