室间隔完整型肺动脉闭锁的临床治疗策略  

Strategy of surgical management for pulmonary atresia with intact ventrieular septum

作　　者：宓亚平[1] 贾兵[1] 李炘[1] 叶明[1] 陈张根[1] 

机构地区：[1]复旦大学附属儿科医院心血管中心,上海200032

出　　处：《中华小儿外科杂志》2009年第1期20-24,共5页Chinese Journal of Pediatric Surgery

基　　金：基金项目：上海市重点学科建设资助项目（编号：B116）

摘　　要：目的总结室间隔完整型肺动脉闭锁的治疗策略。方法1994年1月至2008年1月，31例室间隔完整型肺动脉闭锁行于术治疗，男23例，女8例，年龄20h至3岁，体重2．9～12．5kg。手术包括体肺分流5例，闭式肺动脉瓣切开4例（联合体肺分流2例），跨瓣补片18例（联合体肺分流12例），双向腔肺分流术4例。根据手术时间分为A组（1994至2003年，13例）和B组（2014年至今，18例）。结果住院死亡8例（8／31，25．81％），B组病死率明显低于A组（2／18、vs 6／13，P〈0．01）。不同手术阶段（r=0．40，P=0．03）、手术前已机械通气（r=0．56，P=0．001）等因素与手术病死率有关。结论室间隔完整型肺动脉闭锁治疗方案需个体化，加强心功能支持和随访以获得满意生存率。Objective To evaluate the clinical treatment courses, outcomes and follow-up for the pulmonary atresia with intact ventricular septum （PAWS） in our center, and to delineate strate gies for the oplimal management of PAIVS. Methods From January 1994 to January 2008, 31 con secutive infants （23 males and 8 females） with PAIVS underwent surgical treatment. Mean age at operation was 106.88 days （ranged from 20 hours to 3 years）. Mean weight was 4.71kg （ranged from 2.9kg to 12.5kg）. Six cases were complicated with severe maldeveiopment of right ventricle, 5 with patency of sinusoid, 2 with Ebstein malformation, 1 with atresia of tricuspid valve（TV）, 3 with stenosis of TV, and 3 with maldevelopment of TV. Initial surgical treatment included： five Blalock-Taussig shunt （BT shunt）, 2 closed pulmonary valvotomy （CPV）, 2 CPV and BT shunt, 6 right ventricu lar outlet tract reconstruction （RVOTR）, 12 RVOTR and BT shunt and 4 Bidirectional cavopulmonary connection. The patients were divided into two groups based on the timing of operation： group A （1994 to 2003, 13 cases）and group B （2004 to now, 18 cases）. Results Mean duration of mechanical ventilation and intensive care unit stay were 1.98 ± 1.78 days and 6.42±5.98 days , and mean length of stay was 16. 12 ±9.27 days, respectively. There were totally eight patients died during hospitaliza tion（8/31,25. 81%）. Mortality of group B was significantly lower than that of group A（2/18 vs 6/13, P〈0.01 ）. Multivariable analyses demonstrated that the timing of operation（r=0. 40, P = 0. 03） and mechanical ventilation before surgical treatment（r= 0.56, P = 0.001） could be the risk factors for in hospital mortality. Twenty one cases were followed up at the average period of 20 months （ranged from 2 months to 7 years）. Conclusions Patients with PAIVS should undergo appropriate operations in time based on individual conditions, such as the size of tricuspid valve, the shape of right ventricle and malformation of coronary

关 键 词：肺动脉瓣闭锁 心脏外科手术 心脏病 先天性 

分 类 号：R686[医药卫生—骨科学]