Prenatal Diagnosis of Isolated Right Aortic Arch: A Collaborative Approach to Management of Postnatal Aerodigestive Symptoms.

BACKGROUND: Although prenatal imaging advancements have increased isolated right aortic arch (iRAA) detection, optimal postnatal vascular ring management remains uncertain. Whereas early postnatal imaging and surgical management may be clinically warranted for some infants, long-term data are not yet available to justify an identical approach for all prenatal iRAA diagnoses. METHODS: Children with prenatally diagnosed iRAA and left ductus arteriosus were included in a retrospective cohort study at a rural academic center. Fetal echocardiograms (2014-2024) were reviewed to exclude complex congenital heart disease. Joint postnatal cardiopulmonary care was standardized. Clinical data were ascertained using aerodigestive symptom checklists. Fisher's exact test was used to examine associations between symptoms and surgical referral. Growth was assessed by z-score rates of change from birth weight until last clinic weight. RESULTS: 3,458 fetal echocardiograms were performed yielding 31 distinct cases meeting inclusion criteria, with postnatal data available for 25. Twenty were managed medically (25% asymptomatic, 75% intermittently symptomatic) with median follow up of 14 (11.5, 17.5) months; five underwent surgery (80% progressively symptomatic) with median follow up of 36 (18, 37) months). Choking/dysphagia (p = 0.04), recurrent emesis (p = 0.03), and the combination of both digestive symptoms and noisy breathing (p = 0.01) were associated with surgical referral. Weight outcomes were similar (p = 0.98) between medical and surgical patients. CONCLUSIONS: Although aerodigestive symptoms were common, most were not progressive and weight profiles were not adversely affected. Isolated digestive symptoms or in combination with respiratory symptoms may prompt greater concern. A collaborative cardiopulmonary approach to clinical evaluation may help with short-term risk stratification.