全腔静脉肺动脉连接术后近、中期效果分析

Analysis of the short and midterm results for total cavopulmonary connection.

目的总结全腔静脉肺动脉连接术（TCPC）的临床经验，探讨该术式的应用范围及近、中期效果。方法回顾性总结了自1999年至2007年31例TCPC患者的临床资料，全部病例分为儿童组（≤16岁，21例）和成人组（10例），全部病例中单心室15例，完全性心内膜膜垫缺损并右心室双出口8例，完全性大动脉转位并右心室双出口3例，三尖瓣闭锁3例，右心室双出口并左心室发育不良1例，矫正型大动脉转位并法洛四联症1例。其中合并3房心1例，永存左上腔10例，二尖瓣中、重度返流1例，共同瓣中度返流3例。所有病例均合并肺窄且肺动脉发育良好。行TCPC，其中心内隧道10例，心外管道21例；28例在体外循环下手术，3例为非体外循环手术。结果儿童组无围术期死亡，无顽固性胸腔积液；成人组死亡2例，3例出现顽固性胸腔积液，1例出现下腔静脉淤滞症状，经治疗后消失。随访6个月至8年，心功能Ⅰ级26例，Ⅱ级2例，Ⅲ级1例。结论TCPC手术是功能性矫治先天性心脏病复杂畸形的有效方法，成人术后并发症较多．近、中期效果主要与房室瓣返流有关。

Objective To summarize the clinical experiences of total cavopulmonary connection （TCPC）, investigate the applications and short and mid term effects. Methods From 1999 to 2007,31 patients with complex congenital heart disease underwent TCPC. All patients were divided into child group（ ≤ 16 years old , n = 21 ） and a- dult group （n = 10）, including 15 cases of functional univentriele, 8 cases of complete atrioventricular septal defect with double outlet of right ventricle, 3 cases of transposition with double outlet right ventricle, 3 cases of tricuspid atresia, 1 case of double outlet of right ventricle with hypoplastie left heart. 1 case of corrected transposition of the great arteries with TOF. Among them, 1 patient had cortriatriatum, 10 patients had left superior vena cave, moderate or severe mitral valve insufficiency occurred in 1, moderate atrioventricular valve insufficiency occurred in 3.28 patients underwent TCPC with cardiopulmonary bypass （CPB）and 3 patients without CPB;Of them, 21 patients were treated with extracardiac conduit and 10 cases with intratrial lateral tunnel. Results There was no early death, postoperative death or large quantities of pleural effusion in child group;but two deaths and three patients with large quantities of pleural effusion in adult group. One case caught up in inferior venous stasis and was cured. Follow- up was carried out from 6 months to 6 years. 26 patients were in NYHA class Ⅰ,2 patients in NYHA class Ⅱand only one patient in NYHA class Ⅲ. Condusions TCPC is effectively used for complex congenital heart disease which cart not be corrected in anatomy ;serious postoperative complications often occur in adults. Atrioventricular valve insufficiency decisively affects the short and midterm effects, even the long-term effects.