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.

Isolated Vascular Rings Are Common Cardiovascular Malformations

“…14 A previous publication of ours reported our experience with vascular ring types, risk factors, and outcomes. 2 In that study, we noted the preponderance of right aortic arch vascular rings, higher occurrence in females, and a statistically significant association with maternal comorbidities, especially advanced maternal age.…”

“…We previously reported a Nevada general population prevalence for isolated vascular rings of 5.3 per 10,000 live births. 2 Recently, Vigneswaran and colleagues, from the United Kingdom, reported an isolated vascular ring prevalence of 6.9 per 10,000 live births from prenatal detection data generated from 33,000 pregnancies in a general population. 3 Our past investigations have reported the demographics, associated maternal comorbidities, and outcomes for vascular rings cared for at our center.…”

“…3 Our past investigations have reported the demographics, associated maternal comorbidities, and outcomes for vascular rings cared for at our center. 2,4,5 This study specifically investigated prevalence data for isolated vascular rings in the Southern Nevada general population over the past 8 years.…”

Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: The nevada experience

“…We previously demonstrated an association between advanced maternal age or other maternal comorbidities and some forms of critical congenital heart disease, isomeric situs, and vascular rings 17–20 . Others have also shown associations with advanced maternal or maternal comorbidities and congenital heart disease 21–24 .…”

“…We previously demonstrated an association between advanced maternal age or other maternal comorbidities and some forms of critical congenital heart disease, isomeric situs, and vascular rings. [17][18][19][20] Others have also shown associations with advanced maternal or maternal comorbidities and congenital heart disease. [21][22][23][24] Embedding our fetal cardiologists in all the state's maternal-fetal-medicine clinics provides a population of high-risk pregnancies comprised of both those with maternal comorbidities and abnormal routine obstetric ultrasounds or both.…”