年长儿童及成人患者肺动脉闭锁合并室间隔缺损的外科治疗

Surgical Treatment of Pulmonary Atresia with Ventricular Septal Defect in Elder Children and Adults

目的探讨年长儿童和成人患者肺动脉闭锁伴室间隔缺损(PA-VSD)外科手术治疗的方法、术式和手术时机,以提高手术疗效。方法 1996年10月至2008年10月共有39例8岁以上PA-VSD患者在北京阜外心血管病医院行一期或分期根治术,其中男21例,女18例;年龄8～27岁,平均年龄13.43岁。A型14例,B型11例,C型14例。行一期根治术23例,采用同种带瓣血管或带瓣牛颈静脉行肺动脉-右心室流出道连接术,对合并粗大体动脉肺动脉侧枝血管的3例患者行肺动脉融合术(UF);分期手术16例,其中体动脉-肺动脉分流术9例,分期根治术7例。结果围术期共死亡6例,总病死率15.38%(6/39),其中一期根治术院内死亡4例(17.39%),均为C型患者,死亡原因为术后肺动脉压增高引起的低心排血量综合征;分期手术院内死亡2例(12.50%),其中1例B型再次分流患者,1例C型分期根治患者,均为再次手术中损伤主动脉,引起出血死亡。随访28例,随访率84.85%,随访时间14.0个月～9.2年,失访5例。随访期间无死亡患者,心功能I级9例,Ⅱ级13例,Ⅲ级5例,Ⅳ级1例。3例患者主动脉瓣有少量至中量反流,给予口服利尿补钾药物治疗,定期随访。结论年长儿童和成人PA-VSD患者肺血管损伤程度较重,且常合并粗大体动脉肺动脉侧枝血管,病情复杂和重症患者一期根治手术的标准应当在现有标准的基础上更加严格界定。

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.