一期手术矫治主动脉弓中断及伴发畸形

Single Stage Repair of Interrupted Aortic Arch with Associated Cardiac Anomalies

目的总结主动脉弓中断（IAA）及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者（平均年龄1．1岁、平均体重7．0kg），合并畸形有室间隔缺损23例，完全性大动脉错位2例，主肺动脉窗伴右肺动脉起源于主动脉3例，第5主动脉弓残存狭窄2例，右心室双出口2例，永存动脉干2例，迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治，其中34例IAA及伴发畸形一期解剖纠治，1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月～4年，死亡2例。术后发生并发症7例．Ⅲ°房室传导阻滞和膈神经麻痹各1例，轻度吻合口梗阻5例，其余患者无残余解剖问题，心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高，围术期合适的干预是治疗成功的关键；由于伴发畸形复杂，远期生存率不容乐观。

Objective To summarize the experiences of single stage repair of interrupted aortic arch （IAA） associated with cardiac anomalies. Methods From Jan. 2000 to Dec. 2005, 48 patients admited in hospital and 35 patients were operated, the mean age at operation was 1.1 years. The associated anomalies included 23 cases of ventricular septal defect, 2 cases of transposition of great arteries, 3 cases of aortopulmonary window with aortic origin of right pulmonary artery, 2 cases of truncus arteriosus, 2 cases of double outlet right ventricle, 2 cases of stenotic fifth arch and 1 case of aberrant origin of right subclavian artery with mild hypoplastic decending aorta. Among them, 34 patients underwent single stage repair and 1 kid underwent palliative correction. Results There were 4 surgical deaths. The sequelae included one diaphragm paralysis and one 3rd degree of atrioventricular block. Only 5 kids recurred mild stenosis of aortic arch anastomosis and 2 death occurred during 3 months to 4 years of follow-up. Conclusion Though early surgical mortality for primary single stage repair is now relatively low, if appropriate interventions has been accomplished during perioperative period, but outcomes of IAA remain of concern, especially in patients with associated lesions.