主动脉弓中断伴室间隔缺损的一期纠治

One-stage repair of interrupted aortic arch with ventricular septal defect

目的探讨主动脉弓中断伴室间隔缺损纠治手术的经验。方法36例主动脉弓中断伴室间隔缺损的患儿，其中1例伴轻度升主动脉发育不良和左室流出道狭窄，1例伴第5弓残存，2例伴主肺动脉窗，1例伴右肺动脉异常起源于主动脉。所有患者均行一期解剖纠治手术。结果手术死亡2例，5例延迟关胸。术后6月～5年随访，1例死亡，2例左支气管轻度受压，2例存在心室水平残余分流，6例降主动脉流速稍快，全组心肺功能均良好。结论主动脉弓中断伴室间隔缺损一经发现须及时手术治疗，一期纠治手术成功率较高，需密切关注主动脉吻合口狭窄及左支气管受压对患儿远期生存造成的影响。

Objective To explore the experience of one-stage repair of interrupted aortic arch with ventricular septal defect. Methods Thirty-six cases of interrupted aortic arch with ventricular septal defect were included. Among them, one was complicated with mild stenosis of left ventricnlar outflow tract, one with stenotic persistent fifth arch, two with aortopulmonary window and one with anomalous origin of right pulmonary artery from ascending aorta. All the patients underwent one-stage repair. Results Two cases died from operation, and 5 experienced delayed sternum closure. The patients were followed up for 6 months to 5 years. Death occurred in one case, mild compression of left bronchus occurred in two cases, ventricular residual shunt in two cases and slight fast flow rate of descending aorta in 6 cases. The eardiorespiratory function of all the cases remains well during the follow up. Conclusion Timely operation should be prefomed for interrupted aortic arch with ventricular septal defect, and one-stage repair may yield lower surgical mortality. However, long-term outcomes remain of concern due to aorta anastomotic stenosis and compression of left bronchus.