摘要目的 主动脉弓中断是一类复杂的先天性心脏病,其标准Aristotle复杂性评分高达10.8分.本文通过回顾性分析总结主动脉弓中断一期矫治术的疗效、并发症及常见失误及预防.方法 自2008年2月至2013年10月,我院心脏外科共接收并手术治疗主动脉弓中断患儿12例.男9例,女3例;合并室间隔缺损的患儿8例,其中7例为A型弓中断,1例为B型;合并主肺动脉间隔缺损的患儿1例,为A型弓中断;合并Taussig-Bing的患儿2例,1例为A型,1例为B型弓中断;无合并其他心血管畸形的患儿1例,为A型弓中断.总计A型主动脉弓中断10例,占83.3％,B型主动脉弓中断2例,占16.7％,无C型主动脉弓中断.患者年龄为28 d～90个月,中位年龄为4个月;体质量为3.1～18.5 kg,中位体质量为4.1kg.术前常规行超声心动图及螺旋CT检查,全部患儿均行一期矫治术,并根据所合并的心内畸形调整手术入路及手术方法.术毕当天及出院前常规行心脏超声检查,并术后3个月、半年及每年行常规随访.结果 全组无早期死亡.术后反复呼吸道感染1例,为主动脉弓中断合并室间隔缺损,支气管纤维镜检查提示左主支气管重度狭窄,术后11d经左后外侧切口行降主动脉松解、悬吊术,并抗感染治疗后治愈出院,但在术后50d时因再次肺部感染死亡.全组病儿围术期未出现神经系统并发症及肾功能损害.随访1～68个月,除1例压差大于20 mmHg外,尚未发现残余主动脉弓梗阻、左室流出道梗阻及左主支气管梗阻发生.结论 主动脉弓中断一经诊断即需尽早手术.一期矫治是安全、有效的.充分切除动脉导管组织,广泛彻底游离松解胸部各血管进行无张力吻合是主动脉弓降部成形手术成功及减少残余主动脉弓梗阻、左室流出道梗阻及左主支气管梗阻的关键.

abstractsObjective To review the perioperative management of interrupted aoritic arch (IAA) so as to decrease its mortality and morbidity rates.Methods During the period of February 2008 and October 2013,a total of 12 IAA patients underwent one-stage corrections at our hospital.There were 9 boys and 3 girls.Eight patients were diagnosed with concurrent ventricular septal defect (VSD),including type A (n =7) and type B (n =1).One patient of type A had concurrent aorticopulmonary window.Two cases of either type had concurrent Tuassig-Bing.One case was free of other cardiac malformations.There were type A (n =10,83.3％) and type B (n =2,16.7％).Their operative age was 28 days to 90 months and their weight 3.1-18.5 kg.Echocardiography and computed tomography were routine preoperative diagnostic tools.All patients underwent one-stage surgical corrections for their particular cardiac malformations.Routine postoperative follow-ups were set at discharge,3,6 and 12 months.Results There was no early death.One case of concurrent VSD with early refectory pneumonia had severe left bronchial stenosis.After descending aorta suspension,this patient was discharged and died from pneumonia at Day 50 postoperation.No neurological or renal complications occurred.During follow-ups,except for 1 patient of over 20 mmHg pressure gradient across anastomosis,there was no other case of residual aortic arch stenosis,left ventricular obstruction or bronchus stenosis.Conclusions Prompt surgery is indicated for confirmed IAA.One-stage correction is a safe and effective procedure.The key point for a successful surgery is tension-free anastomosis.