Department of Obstetrics and Gynecology, Amsterdam UMC Location University of Amsterdam, Amsterdam, The Netherlands.
Amsterdam Reproduction and Development, Amsterdam, The Netherlands.
Prenat Diagn. 2023 May;43(5):629-638. doi: 10.1002/pd.6326. Epub 2023 Feb 17.
To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA).
Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR.
In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor.
Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.
确定需要在生命的第一年及之后进行手术的儿童比例，以改善对患有右主动脉弓（RAA）胎儿的父母的咨询。
从前瞻性登记 PRECOR 中提取 2007 年至 2021 年间诊断为孤立性 RAA 的胎儿，定义为无心脏内或心脏外异常。
共纳入 110 例胎儿，92 例产前诊断为 RAA，18 例为双主动脉弓（DAA）。22q11 缺失综合征的患病率为 5.5%。6 例妊娠终止，5 例为假阳性；因此，随访包括 99 例新生儿。在生命的第一年，10 例婴儿（10%）接受了手术。共有 25 例（25%）儿童在平均 17 个月时接受了手术。这 25 例中有 8 例（32%）为 DAA。只有 1 例 DAA 婴儿因阻塞性喘鸣而在生命的第一周需要手术。
产前诊断为 RAA 的儿童发生新生儿期急性呼吸问题的风险较低。仅在怀疑 DAA 的情况下，似乎才需要在具有新生儿重症监护和儿科心胸设施的医院分娩。应告知期待中的父母，目前有 25%的儿童需要择期手术，而且仅因急性呼吸窘迫而偶然需要手术。
