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Fetuses with right aortic arch: a multicenter cohort study and meta-analysis
F D'Antonio1, A Khalil1, V Zidere2
1Fetal Medicine Unit, St George's University Hospital NHS Foundation Trust and Institute of Cardiovascular and Cell Sciences, St George's, University of London, London, UK.
Summary
Fetal right aortic arch (RAA) without heart defects is often linked to extracardiac abnormalities, not chromosomal issues. Most cases are isolated, but about 25% develop vascular ring symptoms, requiring postnatal monitoring.
Area of Science:
- Cardiology
- Prenatal Diagnosis
- Genetics
Background:
- Antenatal screening for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA).
- Understanding the outcomes of isolated fetal RAA is crucial for guiding postnatal management.
Purpose of the Study:
- To determine the outcomes of fetuses diagnosed with right aortic arch (RAA) but without intracardiac abnormalities (ICA).
- To provide guidance for the postnatal management of isolated fetal RAA.
Main Methods:
- Retrospective cohort study analyzing rates of chromosomal abnormalities, 22q11.2 deletion, extracardiac abnormalities (ECA), and postnatal ICA/ECA.
- Systematic review and meta-analysis of 16 studies (312 fetuses).
- Kaplan-Meier analysis for vascular ring surgery as an endpoint.
Main Results:
- In the cohort, 14.1% had chromosomal abnormalities, 6.4% had 22q11.2 deletion, and 17.4% had fetal ECA.
- Systematic review found overall rates of 9.0% for chromosomal abnormalities and 6.1% for 22q11.2 deletion.
- Vascular ring symptoms occurred in 25.2% of cases, with 17.1% requiring surgery; 2-year freedom from surgery was 83.0%.
Conclusions:
- Isolated fetal RAA is more commonly associated with extracardiac abnormalities than chromosomal abnormalities.
- The majority of isolated fetal RAA cases are isolated.
- Postnatal surveillance is recommended, particularly within the first two years after birth, due to the potential for vascular ring symptoms.
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