Double-outlet right ventricle (S,D,D), VSD related to pulmonary artery, and pulmonic stenosis absent. Correction with an intraventricular conduit in infancy.

The case history of a 5-month-old infant with double-outlet right ventricle (S,D,D) ventricular septal defect (VSD) related to the pulmonary artery, and absence of pulmonic stenosis is reported. The anomaly was repaired with an internal intraventricular conduit. This is the youngest infant with this subset of DORV to survive complete repair and the youngest in whom an intraventricular conduit was used. The diagnosis was not suspected preoperatively, and it is fortunate that the intracardiac anatomy permitted this type of repair at this age. The optimal management program for this subset of DORV should be initial pulmonary artery banding with later complete repair.