Double outlet right ventricle is a relatively uncommon anomaly among the congenital heart diseases. It is a form of abnormal ventriculoarterial connection. Great arteries originate from the morphologically right ventricle. The differential clinical signs and symptoms depend on the ventricular septal defect type and also the additional cardiac anomalies. Various anatomic subtypes are present. Many different types of either palliative or corrective surgical procedures have been developed. The clinical findings like cyanosis or congestive heart failure become apparent in the first two months of life. Right ventricular failure and low cardiac output are important predictors of postoperative early mortality for the patients who are not operated on in time. The surgical results depend on the ventricular septal defect localization. Types of double outlet right ventricle, additional cardiac anomalies, clinical signs and symptoms, diagnosis and surgical procedures are discussed in this literately reviewed article.

Keywords : Double outlet right ventricle, intraventricular tunneling, right ventricular outflow tract reconstruction