ObjectiveThe aim of this study was to analyze the mid- and long-term outcome of single cusp replacement in patients with ventricular septal defect and aortic insufficiency. MethodsFrom September 2005 through March 2014, 172 patients underwent single cusp replacement and ventricular septal defect (VSD) closure. The median age was 19.5 years (range, 9 months to 67 years). Additional techniques were used to repair associated anomalies including sinus of Valsalva aneurysm, patent ductus arteriosus, patent foramen ovale, subaortic membrane, and intramural coronary artery. ResultsOne patient with large left ventricle (preoperative end-diastolic diameter: 9.8cm) died after the procedure from incurable heart failure. Four patients required a second pump run for residual aortic insufficiency (AI) (two patients, 1.16%) and residual VSD (two patients, 1.16%). Four patients required re-exploration for postoperative bleeding or cardiac tamponade. Mean follow-up was 53.2725.37 months (median, 56.5 months; range, 3 to 104 months). Redo aortic valve surgery was performed in three patients. Three patients had moderate-severe AI during the following period without reoperation. There was no post-operative endocarditis. ConclusionSingle cusp replacement can be safely used in patients with VSD-AI.