摘要目的 探讨双主动脉弓合并心内畸形的临床特点、诊断和手术方法.方法 回顾性分析5例双主动脉弓合并心内畸形患者,男4例,女1例,年龄41天～19岁,体质量4.3～56.0kg.合并法洛四联症2例,右心室双出口2例(1例左肺动脉异常起源于左主动脉弓,1例右冠状动脉异常起源于左前降支),完全型大动脉转位(TGA)并室间隔缺损1例.4例术前经多排螺旋CT确诊并经正中开胸同期手术矫治心内畸形及双主动脉弓,1例合并TGA者大动脉调转术后因顽固肺部感染不能脱离呼吸机,CT检查确诊双主动脉弓后再次经原正中切口手术切断左侧主动脉弓.结果 1例术后因呕吐误吸导致病情恶化死亡,余4例治愈出院,未出现与双弓矫治相关的并发症.结论 双主动脉弓手术治疗可取得良好效果,对于合并心内畸形的双主动脉弓患者,可经胸骨正中切口同期矫治.有顽固呼吸道症状的患者,应及时进行CT检查,尽早发现并手术治疗.

abstractsObjective To evaluate the clinical characteristics,diagnosis and surgical repair methods of double aortic arch (DAA) associated with complex cardiac anomalies.Methods Retrospectively analyzed the clinical data of the 5 DAA associated with complex cardiac anomalies patients,4 males and 1 female,age from 41 days to 19 years old,weight 4.3-56.0 kg.Accompanied cardiac malformites including 2 cases with TOF,2 cases with DORV,and 1 case with d-TGA.4 cases were diagnosed DAA via MSCT and were surgical treated combined with cardiac malformations at same stage.1 case of DAA associated with d-TGA experienced stubborn pulmonary infection with increased airway resistance and could not tolerate ventilator weaning after aterial switch operation.Then he was diagnosed DAA with left arch atresia through MSCT.So,the patient underwent another surgery to excise the left aortic arch.Results One case worsened postoperatively by the cause of vomiting and aspiration and then died.The other four were survival and discharged with no DAA repair relating complication.Conclusion Double aortic arch can be cured by surgical repair and DAA with cardiac defects can be operated in one stage via median sternotomy.Congenital vascular ring should be considered in patients with stubborn and unexplained respiratory symptoms,who should receive timely imaging examinations for accurate diagnosis and surgical treatment at the early stage.