49例复杂降主动脉弓及降主动脉发育不良合并心内畸形的一期矫正

One-stage correction of 49 cases of complex descending aortic arch and descending aortic dysplasia complicated with intracardiac malformation

目的:先天性主动脉弓和降主动脉发育不良合并心内异常是一种罕见的心血管畸形,目前其手术方式仍存在争议。本研究主要探讨胸骨正中开胸治疗的有效性和安全性。方法:回顾性分析49例复杂主动脉弓和降主动脉发育不良患者的临床资料。其中加宽组(31例)行主动脉弓和降主动脉加宽,旁路组(18例)行升主动脉-降主动脉旁路。随访1年,收集两组患者术后资料。结果:旁路组和加宽组各有1例早期死亡。加宽组2例早期声音嘶哑,2例乳糜胸。随访1年内所有患者的上下肢收缩压差均小于10 mmHg(1 mmHg=0.133 kPa)。结论:胸骨正中开胸治疗主动脉弓及降主动脉发育不良是安全可行的,血管移植物旁路是治疗主动脉弓离断、主动脉脉弓远端发育不良或血管明显钙化患者的良好选择。

Objective:Congenital aortic arch and descending aorta dysplasia combined with intracardiac abnormalities are rare cardiovascular malformations.At present,the surgical method is still controversial.This study mainly discusses the efficacy and safety of median sternotomy.Methods:The clinical data of 49 patients with complex aortic arch and descending aortic dysplasia were analyzed retrospectively.The patients in the widening group(n=31)underwent widening of aortic arch and descending aorta,while the patients in the bypass group(n=18)underwent ascending aorta-descending aorta bypass.All patients were followed up for 1 year,and the postoperative data were collected.Results:There was one early death each in the bypass group and the widening group.There were 2 patients with early hoarseness and 2 patients with chylothorax in the widening group.The change in the systolic pressure gradients between the upper and lower limbs was less than 10 mmHg(1 mmHg=0.133 kPa)within one year of follow-up in all patients.Conclusion:Median sternotomy is safe and feasible in treating aortic arch and descending aortic dysplasia,vascular graft bypass is a good choice for patients with interrupted aortic arch,distal aortic arch dysplasia,or obvious vascular calcification.