新生儿期根治主动脉弓中断伴合并畸形

目的介绍新生儿围术期主动脉弓中断伴合并畸形的治疗经验。方法全组13例,年龄3～28d,平均体重3．5kg,伴发畸形有主肺动脉窗、有心室双出口Taussig—Bing畸形、右肺动脉起源于升主动脉、室间隔缺损、房间隔缺损、左室流出道梗阻、动脉导管未闭等。均一期行主动脉弓中断及伴发畸形根治术。结果手术死亡1例。随访中轻度吻合口梗阻1例。结论主动脉弓中断是一种少见的先天性心脏病,新生儿期死亡率高,一经发现须及时采取一期解剖根治手术。

Single stage repair of neonatal interrupted aortic arch with associated cardiac anomalies

Objective To introducing the experiences of single stage repair of neonatal interrupted aortic arch （IAA） with associated cardiac anomalies. Methods From Jan. 2005 to May. 2008 ,there 13 neonate patients age varied from 3 to 28 days and mean body weight was 3.5 kg with IAA were hospitalized and underwent corrective repair. The associated anomalies included： aortopulmonary window,double outlet fight ventricle,anomalous origin of fight pulmonary artery from the ascending aorta,ventrieular septal defect,atrial septal defect, mild left ventricular outflow tract obstruction and patent ductus arteriosus. Results Single stage corrective operations were carried in all patients, 1 kid died from operation and 1 kid recurred mild stenosis of aortic arch anastomosis at 2 year follow-up. Conclusions Interrupted aortic arch is a rare congenital cardiac anomaly that accounts for high mortality in neonate if haven＇t appropriate interventions during perioperative period. Though early surgical mortality for primary one-stage repair is now relatively low,while improving, outcomes of interrupted aortic arch remain of concern, especially in patients with associated lesions.