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

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

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.

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).

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.