Isolated vascular rings in the era of high prenatal detection rates: Demographics, diagnosis, risk factors, and outcome.

ABSTRACT

OBJECTIVE: We reviewed our center's isolated vascular ring data. METHODS: Inclusion criteria were patients born in Nevada between June 2015 and July 2020 with situs solitus, levocardia, atrioventricular and ventriculoarterial concordance, and no significant intracardiac malformations. RESULTS: We identified 95 patients. Of the 95, 56 (59%) were female (p = .033). For the study period, there were approximately 180,000 live births, for a prevalence of 5.3 isolated vascular rings per 10,000 live births. Of the 95, 78 (82%) were prenatally diagnosed. Of the 95, 63 (66%) were products of high-risk pregnancies (p = .0001). Additionally, we found advanced maternal age was an isolated vascular ring risk factor (relative risk ratio, 2.7; 95% confidence interval, 1.8, 4.1; p < .00001). CONCLUSIONS: Isolated vascular rings are relatively common cardiovascular malformations and more common in females. High prenatal detection rates are achievable. Further, the majority with isolated vascular rings are products of high-risk pregnancies, and advanced maternal age is a statistically significant occurrence risk factor.