Double Outlet Right Ventricle (DORV)

Double outlet right ventricle (DORV)Double outlet right ventricle (DORV) encompasses a whole family of lesions characterised by both great arteries arising predominantly from the right ventricleVentricles in the presence of a ventricular septal defect (VSDVentricular septal defect (VSD)). The aorta often overrides the VSDVentricular septal defect (VSD) but at least 50% of the aorta must be committed to the RVBorderline right ventricle (RV) in order to classify as a DORVDouble outlet right ventricle (DORV). Instead of forming a distinct cardiac lesion, DORV is a large group of congenital defects with highly variable anatomy and physiology. The spectrum ranges from VSDVentricular septal defect (VSD)-like or TOFTetralogy of Fallot (TOF)-type physiology to TGA/VSDVentricular septal defect (VSD) or complex single ventricleSingle ventricle disease. Driven by the respective physiology, anatomic characteristics, and associated defects, clinical management and surgical strategy can include primary repair, staged procedures, complex biventricular reconstructions, and single ventricleSingle ventricle pathways. Outcome across the spectrum is good, but worse in subgroups with outflow tract obstructions, associated arch hypoplasia, and in single ventricleSingle ventricle patients.