Vascular Rings as a Cause of Respiratory Difficulties

Abstract

Background: Vascular rings are rare congenital developmental anomalies of the cardiovascular system that present with variable respiratory and gastrointestinal symptoms often refractory to what is considered a standard treatment. The aim of our study was to describe the clinical presentation, diagnostic pathway, and management of children referred to a tertiary pediatric department for unexplained respiratory symptoms in whom a vascular ring was confirmed, with particular attention to the role of echocardiography in raising the initial suspicion, and to compare our findings with published cohorts. Methods: We performed a retrospective descriptive case series of patients (n = 7) diagnosed at the Department of Pediatrics and Adolescent Medicine, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, University Hospital Martin, between 2021 and 2025. Results: The median age at onset of clinical symptoms was 30 days (interquartile range [IQR] 1–120 days). The median age at definitive diagnosis was 14.5 months (IQR 4.3–94.0 months). A genetic abnormality was observed in 3 patients (42.9%), and a positive family history of congenital heart defects was present in 4 (57.1%). All patients (n = 7) were symptomatic at the time of definitive diagnosis. We observed double aortic arch (DAA) in 2 patients (28.6%), right aortic arch with aberrant left subclavian artery (RAA + ALSA) in 4 (57.1%), and aberrant right subclavian artery (ARSA) in 1 (14.3%). One patient with RAA + ALSA had a concurrent pulmonary artery sling (PAS). In 6 patients (85.7%), the initial suspicion was raised by transthoracic or prenatal echocardiography. Six patients (85.7%) underwent surgical repair at a median age of 10.5 months (IQR 4.1–20.1 months), while in one case the risk–benefit ratio of surgery is being evaluated. Conclusions: In this series, echocardiography was a useful non-invasive modality for raising the initial suspicion, whereas CT angiography was required to define the vascular anatomy; the majority of patients were definitively diagnosed and treated within the first two years of life. In our cohort, we also identified a rare combination of a vascular ring (RAA + ALSA) and a pulmonary artery sling (PAS) in a single patient.