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.

abstractsObjective To investigate the value of clinical follow‐up in prenatal diagnosis of isolated double aortic arch ( DAA ) . Methods T he 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 ,M RI , 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 ,w ho 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 .