Postnatal Outcomes in Prenatally Detected Vascular Rings

Abstract

Vascular rings are rare congenital defects that can cause tracheal and/or esophageal compression. Prenatal detection is increasing due to advances in screening and fetal echocardiography. Postnatal outcomes remain variable. We describe our single-center experience of postnatal outcomes in prenatally detected vascular rings and evaluate factors associated with surgery. We performed a retrospective review of all fetal diagnoses of possible vascular ring evaluated between 2016 and 2023. Patients with significant intracardiac abnormalities, without postnatal data, or without confirmed postnatal diagnosis were excluded from postnatal analysis. Outcome variables included symptoms, tracheal/esophageal compression, and surgical repair. The prenatal cohort included 109 patients with 80% right aortic arch (RAA) and 20% double aortic arch (DAA). Of 72 patients in the postnatal cohort, 85% underwent computed tomography angiography (CTA) at median age of 2 months with 0.84 ± 0.34 mSv of radiation. On CTA, 69% had a diverticulum. Of those with RAA, 95% had an aberrant left subclavian. Most (79%) had airway abnormalities on CTA. Fifteen (21%) developed symptoms at median age of 5 months. Fifty-eight percent of patients underwent surgery at median age of 10 months, of which 33% were due to symptoms. On univariate analysis, DAA was associated with airway abnormalities on CTA and surgical repair. At 30-month follow up, 99% of patients remained asymptomatic. Prenatal diagnosis of vascular rings is associated with high rates of airway abnormalities, even in those without symptoms. Surgical repair was pursued in asymptomatic patients with DAA and airway abnormalities in our institution. As DAA is associated with airway abnormalities, these patients may require closer monitoring for future symptom development.

Keywords: Cardiac computed tomography angiography; Congenital heart disease; Fetal echocardiography; Vascular ring.