心外管道全腔静脉-肺动脉连接术的疗效

目的总结心外管道全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)的经验,探讨手术适应证、手术设计、手术方法以及治疗效果.方法回顾分析29例心外管道TCPC患者的临床资料,平均年龄10岁,病种包括三尖瓣闭锁9例,左室型心室双入口9例,二尖瓣闭锁3例,右心室双出口合并左心室发育不良5例,矫正型大动脉转位解剖右心室发育不良3例.全部在体外循环下手术,12例采用心脏停搏,17例在并行转流心脏不停跳下手术.结果早期22例中死亡5例,后7例无死亡;随访3个月～10年,无晚期死亡.发生低心排血量综合征12例,乳糜胸11例.结论心外管道TCPC用于较大儿童或成人患者与其他Fontan类手术比较,临床长期效果较好.但应严格掌握手术适应证,合理的手术设计和术中细致操作,有危险因素时增用开窗术以及围术期严密观察和处理是提高手术近、远期治疗效果的关键.

Therapeutic efficacy of extracardiac conduit total cavopulmonary connection

Objective To summary the experience of extracardiac conduit total cavopulmonary connection (TCPC) and study the operative indication, design, method, and therapeutic efficacy. Methods 29 patients of extracardiac conduit TCPC were reviewed:the average age was 10 years. Of them, there were 9 cases of tricuspid atresia, 9 double inlet ventricle with left ventricular type, 3 mitral atresia, 3 corrected transposition of the great arteries with anatomically right ventricular hypoplasia and 5 double outlet of right ventricle with left ventricular hypoplasia. All patients underwent cardiopulmonary bypass, 12 cases with heart arrested, and 17 without heart arrested. In them, 20 cases' superior vena cava were anastomosed directly to the upper margin of right pulmonary artery, 9 cases deviated to the left side of right pulmonary artery to enlarge the stoma. For the inferior vena cava stoma, 22 cases' anterior walls of right atrium were partially incised, and sutured to the posterior wall, then anastomosed with Gore-Tex blood vessel prostheses and connected to pulmonary trunk, and the other 7 cases' bottom of right atrium was totally incised, the proximal was closed, and the distal was anastomosised with Gore-Tex blood vessel prostheses and connected to the lower margin of right pulmonary artery, deviated to the right sidedness. Results 5 died in the first 22 cases, and the next 7 cases all survive. All patients were followed up for 3 months to 10 years with no late death. Of them, 12 cases had low cardiac output syndrome, and 11 cases of chylothorax. Conclusions Compared with other types of Fontan operation, the extracardiac conduit TCPC has better long-term effects in older or grown-up children. Nevertheless, strict operative adoption, reasonable operative design, refined procedures, carefully observation and treatment are the key points of improving therapeutic efficacy.