ABSTRACT
Introduction It has been said that a student with a complete understanding of double-outlet right ventricle (DORV) will understand a significant proportion of congenital heart disease. Knowledge of the embryological origins of DORV provides insight into other outflow tract disorders, and DORV physiology can bear resemblance to most other forms of congenital heart disease. This chapter approaches the specific challenges of fetal DORV, with a focus on correct diagnosis of the cardiac and noncardiac features in order to provide effective and accurate counseling regarding the possible surgical pathways, outcome, and appropriate perinatal management strategy.
