胎儿单纯双主动脉弓的产前诊断与临床随访分析

摘要

目的 探讨产前诊断为单纯双主动脉弓(double aortic arch,DAA)胎儿临床随访的价值.方法 回顾性分析我院产前诊断DAA胎儿的临床结局,产前诊断单纯DAA的复查由CT、MRI、尸检或超声心动图完成,分析临床结局的意义.结果 收集产前单纯DAA病例17例,孕妇年龄19～44(28±6)岁,诊断孕周23～32(27±3)周.产前误诊1例DAA-type A型,产前诊断准确性94％.15例孕妇选择生产,2例患儿出现相关临床症状,接受手术治疗;13例患儿在随访期间内未出现相关临床表现,累计临床症状静默率86％.另外2例终止妊娠,1例合并有室间隔缺损,1例合并先天性高位气道闭锁综合征.17例DAA中,右弓优势15例,左弓优势1例,左右弓均衡1例.15例染色体核型分析未发现异常.结论 产前多切面的超声扫查可明确单纯DAA的诊断,也应结合其他影像学资料,拓展思路,以免患儿出生后延误诊治,预防明显致命性事件的发生,必要时及时实施产时宫外治疗.产前单纯DAA短期内总体预后良好.%Objective To investigate the value of clinical follow-up in prenatal diagnosis of isolated double aortic arch (DAA) . Methods The clinical follow-up materials were retrospectively reviewed in 17 fetuses . Of all the isolated DAA fetuses ,the accuracy rate of prenatal diagnosis was confirmed by CT ,MRI , autopsy or echocardiography ,and pregnant outcomes were summarized . Results A total of 17 fetuses had a sonographic diagnosis of isolated DAA in our centers at a mean gestational age of 23 -32(27 ± 3) weeks , with mother mean age 19 -44 (28 ± 6) years old . One case of DAA type-A was misdiagnosed ,15 cases were delivered with 2 cases occurred respiratory distress or mild dysphagia ,who received surgical treatment , and 13 cases clinical findings were unremarkable at the fellow-up of 24 months ,the silent-rate of clinical symptoms was 86％ . Termination of pregnancy happened in 2 cases ,with 1 (6％ ) had additional ventricular septal defects ,another ( 6％ ) had additional anomalies of congenital high airway obstruction syndrome . In 17 cases of fetuses with isolated DAA ,there were 15 ( 88％ ) cases with dominant right-sided arch ,1 case (6％ ) with dominant left arch ,and 1 case (6％ ) with equal arches in size .Karyotyping prenatal testing was offered to 15 parents with normal results . Conclusions Prenatal ultrasound can accurately diagnose isolated DAA by multiple sections scan ,expanding diagnostic ideas by combination with other medical imaging data to prevent apparent life-threatening event ,or to guide for ex-utero intrapartum treatment . Isolated DAA clinical follow-up results in good outcome .