Contemporary Approach to Vascular Rings

Vascular rings are rare congenital malformations causing tracheal or esophageal compression. Advances in imaging have increased prenatal and postnatal diagnoses, but contemporary surgical outcomes remain underreported. This study reviews the early and midterm results of vascular ring repairs at a single institution over 7 years and examines the impact of diagnosis timing on outcomes.

A retrospective review of pediatric patients diagnosed with vascular rings between 2018 and 2025 was conducted at a single center. Patients included were those who underwent surgical repair with data collected on demographics, diagnostic modality, operative techniques, and complications. Analyses included descriptive statistics and survival analyses for reoperation-free intervals.

We included 77 consecutive patients (median age, 10.1 months; median weight, 9 kg) who underwent 78 procedures, most commonly for right aortic arch with left ligamentum with Kommerell diverticulum (n = 40, 51%) or double aortic arch (n = 34, 44%). Fifty-seven patients were diagnosed prenatally (73%), resulting in earlier surgical intervention (9.1 months, interquartile range [IQR], 6.3-17.9 vs 32.6 months [IQR, 9.2 months-10.4 years], P = .003), especially for right aortic arch with left ligamentum with Kommerell diverticulum (12 [IQR, 6.3-30.9] months vs 4.4 [IQR, 2.4-10.4] years, P < .001). Most surgical repairs were performed via thoracotomy (69, 88%), with low complication rates, including hospital mortality of 0%, chylothorax rate of 7.7%, and reoperation rate of 5.1%. Three-year freedom from reoperation was 98.3% (95% CI, 95%-100%).

Contemporary vascular ring repairs are safe and effective, including at young age in patients diagnosed prenatally. Early intervention, particularly for right aortic arch with left ligamentum, is feasible and associated with favorable outcomes.