Are all rings created equal? A single centre experience of fetal and paediatric vascular rings.

Abstract

Background: Vascular rings cause highly variable clinical presentations. This study assesses the impact of prenatal versus postnatal diagnosis on clinical outcomes.

Methods: We conducted a single centre retrospective review of isolated vascular ring patients (without significant CHD) from 2011 to 2022 and compared clinical and operative data between patients with prenatal and postnatal diagnoses.

Results: Of 177 patients, 45% (N = 80) had prenatal diagnosis. Between 2018 and 2022, 78% had prenatal diagnosis compared to 41% from 2013 to 2018 and 4% before 2013 (p < 0.001). 76.3% (N = 135) had a right aortic arch with left ligamentum arteriosum, 22.6% (N = 40) had a double aortic arch, and 1.1% (N = 2) had a left aortic arch with right ligamentum arteriosum. Postnatal diagnosis patients were more likely to have preoperative respiratory symptoms (55.7%), medications (34.0%), or admissions (24.7%) (versus 32.5%, 10.0%, and 11.3% of the prenatal diagnosis patients, p < 0.05) and require surgical repair (68.0% versus 38.8% of prenatal diagnosis patients, p < 0.0001). 54.8% of patients had surgical repair; prenatal diagnosis patients were younger at surgery, 7.5 (3-11) months compared to 16.0 (5-18) months in the postnatal diagnosis patients (p = .0014). Double aortic arch patients were more likely to require surgical repair (90.0%, compared to 44.5% with right aortic arch, p < 1e^-4). Postnatal diagnosis patients had more residual postoperative symptoms (40.9% versus 16.1% in prenatal diagnosis patients, p = 0.01).

Conclusion: Prenatal diagnosis of vascular rings improves clinical surveillance, resulting in earlier surgical repair in symptomatic patients and diminished morbidity. Higher risk double aortic arch patients should have a tailored evaluation pathway.