目的:右主动脉弓(RAA)是一种罕见的异常,在成年人群和低风险胎儿中的发生率为0.1%。我们在这项研究中的目的是根据亚型评估相关的异常和条件。
方法:这是一项回顾性研究,调查了2018年至2022年在我院诊断为RAA的连续妊娠。将患有RAA的胎儿分为三组,RAA伴右侧动脉导管(RAA-RDA),RAA伴左侧动脉导管(RAA-LDA),和RAA双主动脉弓(RAA-DAA)。
结果:在研究期间共有81例胎儿被诊断为患有RAA。RAA-RDA组的心脏异常发生率(82.8%)高于RAA-LDA(17.6%)和RAA-DAA(22.2%)组(p<0.001)。两组产妇年龄差异无统计学意义,诊断周,妊娠结局,心外异常,和遗传异常。36例导致活产的孤立RAA胎儿中有3例(8%)出现了与血管环相关的症状,1例(2.7%)RAA-DAA新生儿接受手术治疗。
结论:使用RAA-RDA的胎儿心脏异常发生率高。对于心脏异常和其他结构异常,应进行超声检查。由于对气管和食道的压迫风险,血管环形成是一种罕见但重要的并发症。
OBJECTIVE: Right aortic arch (RAA) is a rare anomaly with an incidence of 0.1 % in the adult population and low-risk fetuses. Our aim in this study was to evaluate associated anomalies and conditions according to subtypes.
METHODS: This was a retrospective study examining consecutive pregnancies diagnosed with RAA in our hospital between 2018 and 2022. Fetuses with RAA were divided into three groups, RAA with right-sided ductus arteriosus (RAA-RDA), RAA with left-sided ductus arteriosus (RAA-LDA), and RAA with a double aortic arch (RAA-DAA).
RESULTS: A total of 81 fetuses were diagnosed as having RAA during the study period. The rate of cardiac anomalies (82.8 %) in the RAA-RDA group was higher than in the RAA-LDA (17.6 %) and RAA-DAA (22.2 %) groups (p<0.001). No statistically significant difference was found between the groups in terms of maternal age, diagnosis week, pregnancy outcome, extracardiac anomalies, and genetic anomalies. Three (8 %) of 36 fetuses with isolated RAA who resulted in live birth developed symptoms related to the vascular ring, and one (2.7 %) newborn with RAA-DAA underwent surgery.
CONCLUSIONS: The incidence of cardiac anomalies is high in fetuses with RAA-RDA. Ultrasound examinations should be performed for cardiac anomalies and additional structural anomalies. Vascular ring formation is a rare but important complication due to compression risk to the trachea and esophagus.