Objective To summarize the outcomes of reconstructing right ventricular outflow tract with conical perucardial conduit in type Ⅲ pulmonary atresia with ventricular septal defect (PA/VSD).Methods We retrospectively analyzed the clinical data of 7 patients with type Ⅲ PA/VSD who underwent surgical repair in the Department of Cadiovascular Surgery,Guangdong Cardivascular Institute from January 2012 to August 2014.There were 3 males and 4 females at a median age of 2.5 years (range,1.4 to 10.8 years) and a mean weight of(11.4 ± 3.4) kg.All patients were underwent right ventricular outflow tract reconstruction with conical pericardial conduit.Results The mean bypass time was (132.7 ± 32.5) min,the mean aorta cross-clamping time was(71.9 ± 15.1) min.There was 1 patient with diaphragmatic paralysis and 1 patient with chylothorax,both of whom were underwent surgical intervention.There was 1 patient with postoperative pneumonia.The ventilation time was 17.8-356.9 hours.There was no in-hospital death.The mean ICU stays was 2.8-21.5 days and the mean hospital stays was 13-74 days.All patients were alive and no severe anoxia during follow-up.Conclusion The early outcomes of reconstructing right ventricular outflow tract with conical pericardial conduit in type Ⅲ PA/VSD was good.Preoperative evaluation of the pulmonary development and MAPCAs were helpful for making rational choice.