心外管道全腔肺分流手术的临床应用

Clinical application of extracardia.c conduit Fontan procedure

目的探讨心外管道全腔肺分流手术在小儿复杂青紫型先天性心脏病中的应用价值。方法1998年1月至2008年10月48例患儿接受了心外管道全腔肺分流手术，其中男37例，女11例；年龄3．1～14．5岁，平均（5．6±3．0）岁；体重13～56kg，平均（21．1±10．3）kg；三尖瓣闭锁23例，单心室20例，右心室双出口（远离大动脉型）2例，室间隔完整的肺动脉闭锁2例，法乐四联症伴完全性房室间隔缺损1例；术前SPO2 70％～93％，平均（80．2±6．1）％。38例患儿在行心外管道全腔肺分流手术前接受多次手术，包括改良BT分流5例次，肺动脉环缩4例次，双向腔肺分流手术36例次。12例患儿在同一次手术中完成全腔肺分流手术。结果术后早期死亡3例；乳糜胸7例，气胸2例，胸腔金葡菌感染1例，短暂的交界性心动过速2例，均治愈；术后胸腔引流放置3．5～48d。随访1个月至12年，晚期死亡2例，其余35例心功能恢复至Ⅰ级，6例心功能Ⅱ级，2例心功能Ⅲ级，随访心电图均为窦性心律。统计分析I期Fontan和分期Fontan在术后机械通气时间、监护时间、引流时间，病死率上无统计学差异。结论心外管道全腔肺分流手术术后心律失常的发生率低，开窗术有助于围术期恢复，Ⅰ期Fontan在部分患儿中是可行的。

Objective To evaluate the therapeutic effect of extracardiae conduit Fontan procedure for the patients with complex and cyanosed congenital heart diseases. Methods From Jan 1998 to Oct 2008, 48 patients underwent extracardiac conduit Fontan procedure （37 boys and 11 girls）, aging from 3.1 years to 14. 5 years （mean 5.6 ± 3.0 years）. The weight ranged from 13 kg to 56 kg （mean 21.1 ± 10. 3 kg）. Twenty-three patients were diagnosed as tricuspid atresia, 20 single ventricle, 2 double outlet right ventricle （DORV）, 2 pulmonary artery atresia with complete intraventricular septurn （PA/IVS） and 1 tetralogy of Fallot with complete atrioventricular septal defect. Preoperative SpO2 ranged from 70% to 93% （mean 80. 2% ± 6. 1%）. Before the extracardiac conduit Fontan procedure, 38 patients underwent two or more operations including BT, shunt （5 eases）, pulmonary artery banding （4 cases） and bidirectional cavopulmonary connection （36 cases）. Twelve cases underwent completed total cavopulmonary connection in the same operation. Results Three patients died at early postoperative stage and 2 died at late stage. All postoperative complications were cured, including chylothorax in 7 cases, pneumatothorax in 2, intrathoracic staphylococcus aureus infection in Ⅰ and temporal junction tachycardia in 1. Postoperative pleural drainage was performed on all patients. All patients were followed up from one month to twelve years. Thirty-five patients were evaluated as NYHA class Ⅰ , 6 NYHA class Ⅱ , 2 NYHA classⅢ. ECG showed all patients got sinus rhythm at follow-up. No significant difference was noted in mortality, duration of mechanical support, ICU stay or pleural drainage between one stage Fontan and staged Fontan procedures. Conclusions The incidence of arrhythmia is low in patients undergoing extracardiac conduit Fontan procedure. Fenestration is necessary for patients＂ recovery. One stage Fontan procedure is feasible for a certain group of patients.