Objective To investigate the surgical treatment methods and effects for pulmonary atresia with ventricular septal defect (PAVSD) in elder children and adults in order to promote the treatment effects. Methods From October 1996 to October 2008, we performed stage1 or staged biventricular repair on 39 PAVSD patients including 21 males and 18 females, ranging from 8 to 27 years old with an average age of 13.43 years. There were 14 cases of type A, 11 cases of type B, and 14 cases of type C. Among them, 23 patients underwent stage1 radical repair in which either human blood vessel with valves or bovine jugular vein with valves were used to connect the pulmonary artery and the right ventricular outflow tract. In these 23 patients, 3 patients complicated with major aortopulmonary collaterals(MAPCAs) underwent unifocalization (UF) operation. The other 16 patients received staged repair, including 9 cases of systemic to pulmonary artery shunt and 7 of staged radical cure. Results There were 6 perioperative deaths with a total mortality of 15.38%(6/39), including 4 (17.39%) stage1 radical repair cases and 2 (12.50%) staged radical repair cases. The former 4 were all type C patients, dying from low cardiac output due to increased pulmonary arterial pressure. In the latter 2 deaths, 1 was a type B secondary shunt patient, and the other was a type C staged radical repair case, both of whom died of bleeding caused by aortic injury in the succeeding operations. Followup was done on 28 cases with a followup rate of 84.85%. The followup time ranged from 14.0 months to 9.2 years with 5 cases missing. No patient died during the followup, and 9 patients maintained their cardiac function at class Ⅰ, 13 at class Ⅱ, 5 at class Ⅲ and 1 at class Ⅳ. Three patients had aortic valve regurgitation of small to medium volume, the treatment of which included an administration of oral potassium diuretic medication and regular follow-up. Conclusion Pulmonary vessels of elder children and adults with PAVSD are usually injured severely and oftentimes it is complicated with MAPCAs. Standard for stage1 radical repair should be defined more strictly based on the present one.

Citation： SHI Yi,XU Jianping,YAN Jun,et al .. Surgical Treatment of Pulmonary Atresia with Ventricular Septal Defect in Elder Children and Adults. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2010, 17(3): 177-181. doi: Copy