Double-outlet right ventricle (DORV) is a group of complex ventriculoarterial connections. In the literature, there are reports of trans-aortic correction of tetralogy of Fallot, as well as ventricular septal defects and complex DORV without right ventricular outflow tract obstruction. However, a pure trans-aortic approach for DORV ventricular septal defect (VSD) right ventricular outflow tract obstruction repair is not reported in the literature. We present a case of pure trans-aortic repair of DORV VSD pulmonary stenosis (PS) in an adult. A 20-year-old male with a known case of DORV, VSD, and PS presented with cyanosis. A pure trans-aortic repair of DORV was done. Complete trans-aortic DORV VSD PS repair is yet to be reported in the literature. The trans-aortic approach avoids a right atriotomy, right ventriculotomy, and injury to coronary arteries in cases of complicated tetralogy of Fallot and avoids injury to the tricuspid valve. This approach can be used in selected patients for intracardiac repair. The advantages are excellent visualization of the defects and avoidance of injury to the aortic cusps and bundle of His. In addition, placement of the VSD patch on the left ventricular outflow side may prevent residual shunts after repair.
