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目的评价主动脉弓中断(interrupted aortic arch,IAA)合并其他心内畸形一期根治手术的治疗效果。方法回顾性分析2006年11月至2016年4月行一期双心室根治的48例IAA合并其他心内畸形患儿的临床资料,其中男25例、女23例,中位年龄29(8~91)d,平均体质量(3.80±0.67)kg。所有患儿均行端端吻合加补片扩大术。11例患儿行左室流出道梗阻(left ventricular outflow tract obstruction,LVOTO)解除术。结果 39例(81.3%)IAA患儿合并非复杂性畸形,9例(18.8%)合并复杂性畸形。平均随访(72.1±19.7)个月。38例(79.1%)患儿完成随访,6例住院期间死亡,3例晚期死亡。早期生存率为87.5%,5年生存率83.3%,10年生存率81.3%。10例患儿需要再手术,包括8例继发LVOTO和2例吻合口狭窄。结论端端吻合加前壁补片扩大是一期根治IAA患儿的有效治疗方法。
Objective To evaluate the therapeutic effect of interrupted aortic arch (IAA) combined with other radical anastomosis surgery. Methods The clinical data of 48 cases with IAA and other intracardiac malformations underwent biventricular radiotherapy from November 2006 to April 2016 were retrospectively analyzed. There were 25 males and 23 females with a median age of 29 (range, 91) d, average body weight (3.80 ± 0.67) kg. All patients underwent end-to-end anastomosis plus patch expansion. Eleven patients underwent left ventricular outflow tract obstruction (LVOTO). Results 39 cases (81.3%) of IAA children with non-complex deformity, 9 cases (18.8%) with complicated deformity. The average follow-up (72.1 ± 19.7) months. Thirty-eight (79.1%) children were followed up, 6 died during hospitalization and 3 died late. The early survival rate was 87.5%, the 5-year survival rate was 83.3%, and the 10-year survival rate was 81.3%. Ten children underwent further surgery, including eight cases of secondary LVOTO and two cases of anastomotic stenosis. Conclusion Anastomosis plus anterior wall patch expansion is an effective treatment for children with IAA.