Prenatal Diagnosis of a Double Aortic Arch in the Current Era: A Change in Trajectory?

All articles of this category (opens in new window) Background: Double aortic arch (DAA) is a rare congenital vascular anomaly resulting in a complete vascular ring that encircles the trachea and esophagus, potentially leading to respiratory and gastrointestinal symptoms. Before the introduction of the three-vessel and trachea view in 2012 into the current prenatal screening guidelines, children with DAA presented in childhood with respective symptoms. After 2012, the prenatal detection rate of vascular rings increased to 70%. This study retrospectively analyzed prenatal diagnosis and perioperative management in DAA in the current era in our institution. Methods: A retrospective cohort study enrolled patients with a fetal diagnosis of DAA from 2019 to 2025. Data were obtained from reviewing medical files. Prenatal data, if available, were collected, as well as postnatal data from neonatal and cardiological reports, and surgical files were analyzed. The search identified 18 fetuses in the fetal database of the Pediatric Heart Center Vienna. All children were liveborn. None of the children presented with symptoms in the immediate postnatal period. Two children were symptomatic in the first 2 months; one presented with stridor, one with recurrent vomiting. After 2023, we introduced a computed tomography scan at the age of 4 to 6 months in all children with DAA regardless of symptoms. It revealed an indentation of the moderate esophagus ± and the mild trachea in five children. Four-eighteenth (22%) children underwent surgery at the age of 1 month. One child is awaiting surgery. In all symptomatic patients, symptoms were relieved successfully. Conclusion: Prenatal detection of a DAA is feasible and has dramatically increased in recent years. Thus, we are seeing a lot more clinically asymptomatic children who might have abnormal findings on different imaging modalities. Due to the lack of consensus on the best clinical and surgical management, multicenter studies to report on long-term outcomes, including surgical and nonsurgical cases, are needed for decision-making. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany