Abstract
Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.