Prenatal Diagnosis of Isolated Right Aortic Arch: A Collaborative Approach to Management of Postnatal Aerodigestive
Jonathan N Flyer1,2,3, Kelly J Knight2, Erika M Edwards1,4,5
1Department of Pediatrics, University of Vermont, Burlington, Vermont, USA.
Insights
Prenatal diagnosis of isolated right aortic arch (iRAA) is increasing, but optimal management is unclear. Most infants with iRAA can be managed medically, with surgery reserved for those with progressive aerodigestive symptoms.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Vascular Anomalies
Background:
- Prenatal imaging advances improve detection of isolated right aortic arch (iRAA).
- Optimal postnatal management strategies for infants with iRAA remain uncertain.
- Long-term data are insufficient to guide universal management approaches for all prenatal iRAA diagnoses.
Purpose of the Study:
- To evaluate the clinical course and management outcomes of infants diagnosed with isolated right aortic arch (iRAA) prenatally.
- To identify factors associated with surgical referral and outcomes in this cohort.
Main Methods:
- Retrospective cohort study of infants with prenatally diagnosed iRAA and left ductus arteriosus.
- Review of fetal echocardiograms to exclude complex congenital heart disease.
- Standardized joint postnatal cardiopulmonary care and clinical data ascertainment via aerodigestive symptom checklists.
- Growth assessment using z-score rates of change.
Main Results:
- Twenty of 25 infants with iRAA were managed medically, with 75% experiencing intermittent symptoms.
- Five infants underwent surgery due to progressively symptomatic disease.
- Choking, dysphagia, recurrent emesis, and combined digestive/respiratory symptoms were associated with surgical referral.
- Weight outcomes were similar between medically and surgically managed groups.
Conclusions:
- Most infants with prenatally diagnosed iRAA exhibit manageable aerodigestive symptoms without adverse weight impact.
- Isolated digestive symptoms or combined digestive and respiratory symptoms warrant closer monitoring and may indicate need for surgical intervention.
- A collaborative cardiopulmonary approach aids in short-term risk stratification for iRAA management.
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.
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