双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

目的 分析和评估纠正型大血管错位伴肺动脉狭窄病儿采用双调转(Double-Switch)手术的治疗效果.方法 2001年8月至2008年12月采用Double-Switch手术行纠正型大血管错位伴肺动脉狭窄21例.其中男11例,女10例;年龄3.5个月至6.3岁,平均(31±18)个月;体重6～28 kg,平均(13.1±6.5)kg.室间隔缺损(VSD)为膜周型15例,远离大动脉1例,肺动脉下5例.轻度二尖瓣反流2例,中度反流2例.采用Senning+Rastelli手术方法 .13例右心室与肺总动脉的连接采用牛颈静脉管道.结果 全组均生存.体外循环转流156～287 min,平均(202.0±35.8)min;主动脉阻断93～161min,平均(138.0±19.8)min.均无完全性房室传导阻滞.随防2～5年,上腔静脉流速增快3例(1.8～2.2)m/s,其中1例术后2.6年再次手术解除上腔静脉梗阻,余2例在随防中;肺静脉回流均无明显梗阻;牛颈静脉管道通畅,解剖右室流出道连接处梗阻2例,压力阶差在30～45 mm Hg(1 mm Hg=0.133kPa),尚在随防中.结论 Double-Switch手术纠治纠正型大血管错位伴肺动脉狭窄的复杂型先心病,取得了较好效果,但仍需进一步随访,以观察这类复杂手术方法 的长期疗效.

Double-switch operation for corrected transposition of great arteries with pulmonary stenosis

Objective To compare the results of surgical repair for congenitally corrected transposition of the great arter-ies(ccTGA) with pulmonary stenosis(PS), and to analyze the risk factors that may affect early results and surgical technique. Methods From Aug. 2001 to Dec. 2008, 21 patients with ccTGA and PS were treated in our hospital. They aged 3.5 months to 6.3 years [(31 ± 18) months], weighted 6.28 kg [(13.1± 6.5) kg]. Fifteen cases had paramembranous ventricular sep-tal defect (VSD), 1 noncommitted VSD and 5 subpulmonary VSD. The repairs comprised of Senning and Rastelli operation. RV-PA reconstruction was done by xenopericardial conduit in 13 patients. RV-PA direct anastomosis plus pericardial patch in 6 patients and homograft patch with autopulmonary valve in 2 patients. Results All the patients were alive. During 2 - 5 years follow-up, blood flow rate in superior vena cava increased to 1.8 - 2.2 m/s in 3 cases. One of them needed reoperation to re-lease the stenosis and the rest 2 were in follow-up. Tow patients had right ventricular outlet obstruction with a pressure gradient of 30 -45 mmHg were in follow-up. There was no other stenosis and valve insufficiency. Conclusion Double-Switch opera-tion is practical and impressive in treating of CCTGA with PS. It is important to evaluate the size of VSD and the reconstruction of RV-PA. But it still need more cases and longer time to evaluate the long-term effects.