摘要:目的:回顾性研究大动脉转换术同时进行主动脉弓矫治的I期手术治疗完全性大动脉错位或TaussigBing合并主动脉弓畸形的早中期效果。方法:2000年1月至2008年12月,连续对26例存在主动脉弓畸形的完全性大动脉错位或TaussigBing畸形的小婴儿进行了I期手术矫治,其中完全性大动脉错位13例(TGA/VSD 11例,TGA/IVS 2例),TaussigBing 13例;主动脉弓畸形中主动脉弓中断(A型)7例,CoA19例,6例伴有冠状动脉异常类型。平均手术年龄(28±35) d,lt;2个月占62％,手术平均体重为(4.19±1.15) kg。在深低温停循环或深低温低流量下进行主动脉弓畸形矫治,采用自身组织直接吻合扩大或重建弓,伴有弓部发育不良者补片扩大成形。伴有冠状动脉畸形者在大动脉转换手术中冠状动脉移植方法予改良处理。〖HTH〗结果〖HTSS〗:手术住院死亡3例(11.5%),死因与冠脉移植无关。平均插管时间102 h,监护室时间平均8 d。术后早期生存者主动脉瓣上压力阶差gt;30 mm Hg有2例,主动脉瓣反流轻度2例。单因素分析中伴有冠状动脉异常类型者与术后早期死亡或并发症的风险相关,多因素分析示其与手术年龄、肺动脉高压、术前FS、主动脉阻断时间、术后血清乳酸水平相关。随访期3个月~7年,无死亡,术后5年实际生存率为88.5％(95％ 可信度范围CI 76％～96％),术后1年、5年无需介入干预或手术分别为91.4％、87％。结论:TGA和TaussigBing伴有主动脉弓畸形者I 期进行大动脉转换术和主动脉弓畸形矫治早中期效果良好,早期手术并发症和死亡的风险因素为年龄偏大,肺高压严重,把握手术时机是手术成功要则之一。Abstract: Objective: The study was to evaluate earlymid term results after onestage arterial switch operation (ASO) associated with aortic arch repair for D Transposition of the great arteries (DTGA) and TaussigBing Anomaly with arch abnormally in infant. 〖WTHZ〗Methods〖WTBZ〗: Between January 2000 and December 2008, a primary operation including aortic arch repair through a midline sternotomy was performed in 26 patients, 13 patients with DTGA and 13 TaussigBing. Most patients (62%) underwent operation during the first two months. The repair of arch was accomplished under deep hypothermic circulatory arrest or low flow, employing a wide pericardial patch to reconstruction of arch in some patients or direct ananstomosis. Results: There were 3 (11.5%) hospital deaths. The high risk factors for early mortality and morbidity were unsuitable reconstructed arch, higher age, severe pulmonary hypertension and longer aortic crossclamp time. There were no late deaths. Actuarial 5year survival was 88.5% (95% CI 70% to 96%). Actuarial freedom from overall reintervention, reoperation among operative survivors was 91.4% at 1 year and 87% at 5 years, respectively. Conclusion: the singlestage repair for DTGA and TaussigBing with aortic arch abnormally is suitable choice for infant, and followup of operative survivors is favorable. Optimal operative time was as sooner as possible.