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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Taussig-Bing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Taussig-Bing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),Taussig-Bing 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,<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。结果: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>30 mmHg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Taussig-Bing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。
OBJECTIVE: To retrospectively study the early-to-middle-term effect of stage Ⅰ surgery for aortic arch replacement or Taussig-Bing combined with aortic arch deformity treated with aortic valve replacement. Methods: From January 2000 to December 2008, a total of 26 consecutive infants with complete aortic arch malformation or Taussig-Bing deformity underwent primary surgical correction. Among them, 13 cases of complete aortic dislocation (TGA / VSD 11 cases, TGA / IVS in 2 cases) and Taussig-Bing in 13 cases. There were 7 cases of aortic arch dislocation (type A), 19 cases of CoA and 6 cases of coronary artery abnormalities. The mean operative age was 28 ± 35 days, 62% in 2 months and 4.19 ± 1.15 kg in operation. In the deep hypothermic circulatory arrest or deep hypothermia low flow correction of aortic arch deformity, direct anastomosis with their own organizations to expand or reconstruct the bow, accompanied by bow hypoplasia patch expansion shape. Coronary artery malformations associated with coronary artery transplantation in the conversion of aortic surgery to be modified. Results: Three hospitalized patients died of surgery (11.5%). The cause of death was unrelated to coronary artery transplantation. The average intubation time was 102 h, and the average duration of care room was 8 d. Postoperative early survival of the aortic valve pressure gradient> 30 mmHg in 2 cases, mild aortic regurgitation in 2 cases. In the univariate analysis, the type of coronary artery abnormalities was associated with the risk of early postoperative death or complications. Multivariate analysis showed that it was not related to age of surgery, pulmonary hypertension, preoperative FS, aortic cross-clamping time, postoperative serum lactate level Related. The follow-up period ranged from 3 months to 7 years with no death. The 5-year postoperative 5-year actual survival rate was 88.5% (95% confidence interval CI 76% to 96%). No intervention or surgery was required after 1 year and 5 years 91.4%, 87%. Conclusion: TGA and Taussig-Bing associated with aortic arch deformity Ⅰ period of aortic and aortic arch anomaly correction early and mid-term good effect, early surgical complications and mortality risk factors are older, severe pulmonary hypertension, to grasp the timing of surgery is surgery One of the keys to success.