Isolated Right Aortic Arch Vascular Ring Tracheal Compression Can Significantly Increase in the Short Term

ObjectiveOur principal objective was to evaluate the progression of tracheal compression over time in patients with isolated right aortic arch vascular rings (RAA VRs) who underwent serial computed tomographic (CT) scans. Our secondary objective was to characterize the outcomes of our comprehensive prenatal screening program for VRs.MethodsWe identified all infants born in Nevada with an isolated VR diagnosed prenatally or in infancy between January 2018 and December 2025.ResultsWe identified 168 children with an isolated vascular ring. Of the 168 children, 34/168 (20%) were CT-scan-confirmed double aortic arches, and 134/168 (80%) were RAA VRs, of which 104 were CT-scan-confirmed. Thirty infants only had an initial fetal echocardiogram diagnosis (12 pending CT scan, and 18 lost to follow-up). Of the 104 patients with a CT-scan-confirmed RAA VR, 88 underwent 1 preoperative CT scan, and 16 underwent 2 preoperative CT scans. Of the 16 infants with 2 scans, all with right aortic arch, aberrant left subclavian artery-left ductus arteriosus/left ligamentum (RAA ALSCA-LDA/LL) anatomy: the initial scan average tracheal compression was 29 ± 11% at a median age of 2 weeks. The subsequent average tracheal compression was 50 ± 10% (P = .00001) at a median time of 14.4 months from the first to the second CT scan.ConclusionsNevada has a high prenatal detection rate for isolated vascular rings. In addition, isolated vascular rings are not rare. Furthermore, initial neonatal CT-confirmed mild tracheal compression from a RAA ALSCA-LDA/LL can increase significantly in the short term, supporting the need for close follow-up and the potential value of infant repair.