VP14.10: Prenatal diagnosis of vascular rings and its clinical significance in postnatal life

Abstract

Prenatal detection of vascular rings has increased over the past two decades. These lesions may be asymptomatic or cause respiratory and/or gastrointestinal (GI) symptoms from tracheal or esophageal compression. Symptomatology dictates surgical correction. We present 12 cases of prenatally diagnosed vascular rings with their postnatal course. We reviewed all fetal aortic arch anomalies diagnosed from 2016–2019 at our institution. We collected prenatal and postnatal diagnosis, cardiac and non-cardiac anomalies, genetics, postnatal clinical course, symptoms and surgical outcomes. 32 fetal echocardiograms revealed arch sidedness and branching anomalies; 25 isolated right aortic arch (RAA) with mirror image branching, 6 isolated aberrant right subclavian artery, 1 isolated aberrant left subclavian, 11 RAA with aberrant left subclavian and 1 double aortic arch. Out of the 12 vascular ring patients, no genetic syndromes were identified prenatally or postnatally despite the risk for 22q11 deletion with RAA. Postnatal imaging confirmed the prenatal diagnosis in all 12 patients. Associated congenital heart disease included ventricular septal defect in one patient. Associated extracardiac disease included pelvic kidney and tracheobronchomalacia. 7 (58%) remained asymptomatic and 5 (42%) reported respiratory and/or GI symptoms. Average age of symptom onset was 67 days. 3 (25%) patients required surgery for respiratory and GI symptoms at mean age of 7.3 months. Mean age of clinical follow-up was 6.4 months. We found that the majority of prenatally diagnosed vascular rings remain asymptomatic and do not have concomitant intracardiac or extracardiac disease or genetic syndromes. Presently, there are no prenatal markers to predict postnatal surgery. Further research may identify prenatal markers of respiratory or gastrointestinal compromise.