婴幼儿主动脉弓中断的一期纠治

One-stage repair of interrupted aortic arch in infants

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 outcome of surgical repair of interrupted aortic arch （IAA） combined with anomalies. Methods We retrospectively analyzed the clinical data of 48 patients with IAA combined with anomalies undergoing one-stage biventricular repair in Shanghai Children＇s Medical Center from November 2006 to April 2016. There were 25 males and 23 females with a median age of 29 d （range, 8 to 91 d） and a mean weight of 3.80±0.67 kg. All patients underwent end-to-end anastomosis with patch augmentation, and relief of left ventricular outflow tract obstruction （LVOTO） was performed in 11 patients. Results In IAA children with anomalies, 39 （81.3%） suffered noncomplex lesions and 9 （18.8%） complex lesions. Mean follow-up was 72.1±19.7 months for 38 patients. There were 6 in-hospital deaths and 3 patients died during follow-up. The early survival rate was 87.5%, 5-year rate 83.3% and 10-year rate 81.3%. Reintervention was required in 10 patients, including 8 with subsequent LVOTO and 2 with anastomotic stenosis. Conclusion End-to-end anastomosis with patch augmentation is effective for IAA.