Vascular rings in a tertiary care center: A retrospective review of clinical presentations and interventions

Abstract

BACKGROUND

Vascular rings are rare congenital anomalies of the aortic arch system that encircle and compress the trachea and esophagus, leading to respiratory and feeding symptoms that may mimic more common pediatric conditions. Delayed diagnosis remains a challenge, particularly in patients presenting beyond the neonatal period.

AIM

To describe the clinical presentation, diagnostic approach, anatomical characteristics, surgical management, and postoperative outcomes of pediatric patients diagnosed with complete and incomplete vascular rings at a tertiary referral center.

METHODS

We conducted a retrospective study of patients diagnosed with vascular rings at the Children’s Heart Center of the American University of Beirut Medical Center between January 2010 and December 2025. All patients underwent transthoracic echocardiography and computed tomography angiography for diagnostic confirmation and surgical planning.

RESULTS

Eight patients were identified, of whom three (37.5%) were male. The mean age at time of procedure was 17.5 months (range: 8 days to 38 months). Feeding difficulties and chronic respiratory symptoms were the most common presenting features (both 75%). Three patients (37.5%) had associated intracardiac anomalies. Double aortic arch was the most frequent anatomical subtype (50%), followed by right aortic arch with aberrant left subclavian artery (37.5%). All patients underwent surgical repair, with cardiopulmonary bypass required in two cases. There were no operative or in-hospital deaths. Two neonates developed postoperative tracheomalacia. During a mean follow-up of 32 months, complete symptom resolution was observed in 50% of patients, while the remainder demonstrated significant clinical improvement.

CONCLUSION

Vascular rings remain an important but often underrecognized cause of airway and esophageal compression in infants and young children. Delayed presentation was common in this cohort, underscoring the need for heightened clinical suspicion in patients with persistent respiratory or feeding symptoms. Multimodal imaging and timely surgical intervention result in excellent survival and favorable clinical outcomes, although residual symptoms may persist, particularly in patients with severe preoperative airway compromise.

Keywords: Vascular rings; Aortic arch anomalies; Double aortic arch; Right aortic arch; Tracheoesophageal compression; Congenital heart disease; Pediatric airway obstruction

Core Tip: Vascular rings are rare congenital aortic arch anomalies that frequently present with nonspecific respiratory and feeding symptoms, leading to delayed diagnosis. This single-center retrospective study provides a comprehensive clinical and surgical overview of pediatric vascular rings, distinguished by extensive multimodal imaging. High-quality transthoracic echocardiography, three-dimensional computed tomography angiography, and selective angiography clearly demonstrate anatomical subtypes and airway relationships. These detailed visual findings, combined with favorable surgical outcomes, emphasize the diagnostic value of advanced imaging and reinforce the importance of early recognition and timely intervention.