Double outlet right ventricle

Double outlet right ventricle (DORV) is a congenital heart defect in which both the aorta and the pulmonary artery arise predominantly or entirely from the right ventricle, rather than the normal arrangement where the aorta arises from the left ventricle. This condition is also known as origin of both great vessels from the right ventricle or Taussig-Bing anomaly (when the ventricular septal defect is subpulmonary). DORV accounts for approximately 1–3% of all congenital heart defects and represents a spectrum of malformations with variable anatomy. The condition primarily affects the cardiovascular system. Because both great arteries connect to the right ventricle, oxygen-poor and oxygen-rich blood mix, leading to reduced oxygen delivery to the body. A ventricular septal defect (VSD) is almost always present and is essential for blood to exit the left ventricle. Key symptoms depend on the specific anatomy and include cyanosis (bluish discoloration of the skin), difficulty feeding, poor weight gain, rapid breathing, and heart failure in infancy. The position of the VSD relative to the great arteries, the presence of pulmonary stenosis, and other associated anomalies (such as coarctation of the aorta or atrioventricular canal defects) significantly influence the clinical presentation. Some patients present similarly to tetralogy of Fallot, while others resemble transposition of the great arteries. Treatment for DORV is surgical and depends on the specific anatomical subtype. Options range from biventricular repair (such as an intraventricular tunnel or arterial switch operation) to univentricular palliation (Fontan pathway) when biventricular repair is not feasible. Early surgical intervention, often in the neonatal period or infancy, is typically required. Long-term follow-up with a cardiologist specializing in congenital heart disease is essential, as patients may require additional surgeries or catheter-based interventions over their lifetime. Outcomes have improved significantly with advances in surgical techniques, though prognosis varies based on the complexity of the anatomy and associated defects.

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