Objective Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare complicated congenital heart disease.The intracardiac structure is also complicated.We sum up the corporal surgical procedure of PA/IVS.Methods Between May 2004 and May 2010,we performed emergency or sub-emergency operation in 72 newborns,infants and children [age,1 day to 5.2 years; mean age,(1.3 ±0.6) years] with favorable anatomy.The surgical procedures were divided into three kinds:1.16 cases were performed with Blalock-Tassuing (B-T) shunt operation for A group; 2.33 cases were performed with transannular patch from RVOT-MPA with or without B-T shunt operation for B group.3.22 cases were performed a hybrid procedure with or without B-T shunt operation for C group.From all the patients,15 cases had been finished the two staged operation.Results 10 patients has been died in which 6 patients were in B group with B-T shunt.Only 2 patients were for the hybrid procedure.The ECHO showed the gradient across the pulmonary annular was 15-39 mm Hg [(23 ± 5) mm Hg (1 mm Hg =0.133 kPa)] after operation.The tricuspid insufficient had been siginificantly improved.There were 58 cases were preoperatively serious tricuspid insufficient.There were 11 cases and 13 cases were respectively for middle and mild to middle tricuspid insufficient postoperatively.Follow-up with 2-5 years,there were radical operation for 8 cases,one and half ventrical operation for 5 cases and BDG and Fontan operation for 2 cases.Conclusions.Conclusion BT shunt in common procedure and using a hybrid approach is more safe and feasible than traditional procedure for the patients with PA/IVS.Z vale and right ventricular growth were importment for procedure in the future including radical 、one and half ventrical and Fon tan operation.