分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

Staged total cavopulmonary connection for complex congenital heart diseases

目的总结分期全腔静脉-肺动脉连接术（TCPC）治疗复杂先天性心脏病的经验。方法1998年6月至2008年3月，22例先天性心脏病复杂畸形患者接受分期TCPC。本组中单心室合并肺动脉狭窄9例，合并肺动脉闭锁3例；完全性大动脉转位，十字交叉心，肺动脉瓣狭窄1例；完全性房室通道，左心室发育不良，肺动脉闭锁，房室瓣少-中量反流1例；完全性房室通道，动脉导管未闭，肺动脉狭窄，双向Glenn术后伴吻合口狭窄房室瓣大量反流1例；镜面右位心，功能性单心室，肺动脉闭锁，动脉导管未闭，体肺侧支，双侧双向Glenn术后2年，右下肺动-静脉瘘1例；三尖瓣闭锁并肺动脉狭窄4例；三尖瓣闭锁伴肺动脉闭锁，动脉导管未闭1例；镜面右位心，右心室双出口，室间隔缺损，左心室发育不良，功能性单心房，肺动脉瓣狭窄，三尖瓣少量反流，粗大体肺侧支1例。第一期体肺分流术后，二期双向Glenn术5例，第一期单侧或双侧双向Glenn手术17例。行双向Glenn手术的年龄为（5．9±4．4）岁，Glenn术前肺动脉压为17～20mmHg（1mmHg=0．133kPa）；房室瓣膜关闭不全3例；一期手术前Nakata指数小于200mm^2／m^2者4例。接受TCPC术平均年龄为（9．6±4．9）岁，与双向Glenn术间隔时间（3．7±1．2）年，术前经上腔静脉置管测得肺动脉压力均小于15mmHg。全组22例均采用心外管道TCPC，房室瓣成形1例。结果全组住院死亡1例，病死率为4．5％。死亡病例为单心室，肺动脉闭锁，第一期体肺分流术后，二期左肺动脉成形双向Glenn术后5年第三期行TCPC，术后左肺反复多次大出血死亡。余患者均顺利出院，术后中心静脉压12～18mmHg。出院前经皮测血氧饱和度为90％～96％，活动量明显增加，发绀及气促症状消失，心功能均为NYHA分级Ⅰ～Ⅱ级。结论对于高危患者根据病情选择分期TCPC，可以使更多有高危因素不能行Fontan手术的患者得到救治的机会，扩大手术适应证，并取得满意疗效。

Objective To review the experience of staged total cavopulmonary connection （TCPC） in complex congenital heart diseases. Methods From June 1998 to March 2008, 22 patients underwent staged TCPC for complex congenital heart diseases. Among them, 9 were univentricle and pulmonary artery valve stenosis; 3 were univentricle and pulmonary artery atresia; 1 was transposition of great arteries, crisscross heart and pulmonary artery valve stenosis; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery atresia and atrioventrieular valvular regurgitation; 1 was complete atrioventricular canal defects, left ventricular hypoplasia , pulmonary artery valve stenosis and atrioventricular valvular regurgitation after Glenn procedure; 1 was mirror image dextrocardia, single ventricle, pulmonary artery atresia, major aortopulmonary collateral arteries （MAPCAs） and right pulmonary arteriovenous fistula after Glenn procedure; 4 were tricuspid atresia and pulmonary, artery valve stenosis; 1 was tricuspid atresia and pulmonary atresia; 1 was mirror image dextroeardia, double-outlet of right ventricle, left ventrieular hypoplasia, pulmonary artery valve stenosis, tricuspid incompetence, and MAPCAs. Among them, 5 patients received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC. Seventeen patients received bidirectional Glenn procedure, the mean age was （5.9 ±4. 4）years old. Pulmonary artery pressure pre-Glenn procedure was 17 to 20 mm Hg （1 mm Hg = 0. 133 kPa）. Atrioventricular valve incompetence in 3 patients. Nakata index was less than 200 mm^2/m^2 in 4 patients before the first stage operation. The age of TCPC procedure was （ 9. 6 ± 4. 9） years old, the interval time was （ 3.7± 1.2 ） years. Results There was one in-hospital death, the mortality was 4. 5%. The patient with univentricle and pulmonary atresia, received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC and died of pneumonorrhagia. Other patients were recovered well, postoperative central venous pressure was 12 to 18 mm Hg, percutaneous oxygen saturation was 90% to 96%. The cardiac function were in NYHA class Ⅰ to Ⅱ. Conclusions The staged TCPC was a good procedure in high-risk Fortran candidates. The results were satisfactory for those patients. This staged strategy may extend the operative indications for the Fontan procedure.