婴幼儿先天性双主动脉弓的临床特征和超声诊断

Clinical features and ultrasonic diagnosis of congenital double aortic arch in infants and toddlers

目的总结婴幼儿先天性双主动脉弓(congenital double aortic arch,DAA)的临床特征和超声图像特点,提高超声对双主动脉弓的诊断率。方法回顾性分析2015年1月至2019年12月湖南省儿童医院14例诊断为双主动脉弓患儿的临床资料和超声图像特征。14例中男8例,女6例;年龄1天至2岁7个月,中位年龄7个月;体质量3.2~12.5 kg,中位体质量7 kg。行心脏常规切面检查后重点探查胸骨上窝主动脉弓长轴短轴结合及连续切面,重点观察主动脉弓位置、数目、形态、内径、分支形态和结构。14例均经心脏平扫/增强CT或手术确诊。结果14例中通过超声诊断13例,漏诊1例,超声与CT的诊断符合率为92.8%;右弓优势型9例(9/14,64.2%),左弓优势型3例(3/14,21.4%),左右弓均衡型2例(2/14,14.2%);9例为单纯双主动脉弓,2例合并室间隔缺损,1例合并法洛四联症,2例合并房间隔缺损。12例行手术治疗,2例追踪随访中。结论双主动脉弓可引起婴幼儿严重的呼吸道症状。超声心动图应注重胸骨上窝主动脉弓长短轴多切面连续扫查,并结合CT检查,提高对双主动脉弓的诊断率,尽量早期诊断和治疗,改善双主动脉弓患儿的预后。

Objective To summarize the clinical features and ultrasonic imaging characteristics of congenital double aortic arch(DAA)in infants and toddlers and explore the diagnostic techniques of improving the diagnostic rate of DAA by ultrasound.Methods The clinical data and ultrasonic imaging characteristics were retrospectively analyzed for 14 DAA infants and toddlers from January 2015 to December 2019.There were 8 boys and 6 girls with a median age of 7 months(1 day to 2 years)and a median body weight of 7(3.2-12.5)kg.After conventional cardiac section,special attention was paid to exploring the long axis and short axis of aortic arch of upper sternal fossa,continuous sections and position,number,shape,inner diameter,branch shape and structure of aortic arch.A definite diagnosis was made by plain/enhanced CT scan or surgery.Results The diagnosis was made by ultrasound(n=13)or missed(n=1).The diagnostic coincidence rate between ultrasound and CT was 92.8%.And the clinical types were right arch dominant(9/14,64.2%),left arch dominant(3/14,21.4%)and double bow balance type(2/14,14.2%).The etiologies were isolated DAA(n=9),ventricular septal defect(n=2),tetralogy of Fallot(TOF,n=1)and atrial septum defect(n=2).Twelve children were operated while two cases were followed up.Conclusion DAA may cause severe respiratory symptoms in infants and toddlers.Continuous ultrasonic scans of long/short axes of aortic arch of upper sternal fossa should be performed along CT examination to boost the diagnosis rate of DAA and improve the prognosis by making an early diagnosis and offering proper treatments.