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. 2023 May;43(5):629-638.
doi: 10.1002/pd.6326. Epub 2023 Feb 17.

Short-term outcome after the prenatal diagnosis of right aortic arch

Bo B Bet  1   2 , Maartje C Snoep  3 , Elisabeth van Leeuwen  1   2 , Ingeborg H Linskens  2   4 , Monique C Haak  3 , Lieke Rozendaal  5 , Ingmar Knobbe  6 , Joost van Schuppen  7 , Carlijn E L Hoekstra  8 , David R Koolbergen  9 , Sally-Ann Clur  2   10 , Eva Pajkrt  1   2
Affiliations

Short-term outcome after the prenatal diagnosis of right aortic arch

Bo B Bet et al. Prenat Diagn. 2023 May.

Abstract

Objectives: 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).

Methods: 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.

Results: 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.

Conclusions: 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.

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