Objective To retrospectively analyze surgical cases of pulmonary atresia combined with ventricular septal defect and to discuss the surgical strategy, surgical risk factors, and early and mid-term outcomes. Methods From March 2007 to June 2012, 43 patients underwent surgery for this type of disease, including 22 males and 21 females; ages ranged from 3 months to 19 years; combined malformations included 5 cases of permanent left superior vena cava, 3 cases of coronary artery malformation, 1 case of subcardiac complete pulmonary vein ectopic drainage, 2 cases of transposition of the great arteries, and 1 case of coronary artery fistula. Clinicopathological typing (typing according to the International Nomenclature Scheme for Premature Heart Disease): 28 cases of type A, 14 cases of type B, and 1 case of type C. Twenty-five cases (58.1%) in the stage I radical surgery group underwent stage I radical surgery, including 18 cases of type A and 7 cases of type B. The right ventricular connection of the pulmonary artery was reconstructed with a valved bullous jugular vein conduit in 19 cases, and the right ventricular outflow tract was reconstructed with a valved bullous jugular vein vessel piece in 6 cases. 5 patients had their ventricular septal defects repaired with a living valve patch. In the palliative surgery group, 18 patients (41.9%) underwent palliative surgery, including 10 type A, 7 type B and 1 type C. Among them, 5 had modified BT shunts, 5 had central shunts, 6 had right ventricular pulmonary artery connections, and 2 had Greene shunts. 4 patients (22%) underwent stage II radical surgery. Large corporal pulmonary collateral vessels were managed: 14 intraoperative ligations in 8 patients, 8 interventional blockages in 4 patients, and 7 vessel fusions in 3 patients. Results Four (16%) of the primary radical surgery deaths occurred. All four patients had B-type combined with multiple collateral vessels, one with subcardiac pulmonary vein ectopic drainage, and one with coronary artery fistula, all with preoperative McGoon values of 1.2±0.15. All four patients had incomplete ligation of collateral vessels, and the postoperative right ventricular to left ventricular peak pressure ratio was greater than 0.75. The postoperative right ventricular to left ventricular peak pressure ratio was greater than 0.75. The other 18 patients were discharged with preoperative McGoon values of 1.5±0.25 and postoperative right ventricular to left ventricular peak pressure ratios of 0.58±0.21. The patients were followed up for 2 months to 5 years with cardiac function class I-II (NYHA classification), and the follow-up ultrasound showed low pulmonary regurgitation and good valve preservation. 1 case was re-piped 3 years after surgery due to anastomotic stenosis at the pulmonary artery end. In the palliative surgery group, there was one death (5.5%) due to severe pulmonary infection and multi-organ failure; one case was lost to follow-up, and the follow-up period ranged from 1 month to 5 years, with a significant increase in oxygen saturation and improved mobility and McGoon values. The interval was 12 to 48 months. The surgical treatment of pulmonary atresia combined with ventricular septal defect is still challenging, and the appropriate surgical plan should be selected according to the pathological staging and the developmental status of the pulmonary artery. Treatment of large somatic pulmonary collateral vessels is an important step in the procedure. A postoperative right ventricular to left ventricular peak pressure ratio greater than 0.75 suggests poor healing. Palliative right ventricle to pulmonary artery connection appears to be more effective in promoting pulmonary artery development than other bypass procedures. The decellularized combined with photo-oxidation technique prepared bovine jugular vein with a valved conduit has good early to mid-term results as a pulmonary artery replacement material.