Abstract

OBJECTIVES: Recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnosis of right aortic arch (RAA). We aimed to establish outcome of fetal RAA without intra-cardiac abnormalities (ICA) to guide postnatal management. METHODOLOGY: Retrospective cohort study. Outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extra-cardiac abnormalities (ECA), postnatal ICA and ECA, symptoms and surgery for vascular ring. A systematic review and meta-analysis (reference: CRD42015016097) was performed; results are reported as proportions. Kaplan Meier analysis of vascular ring cases with surgery as endpoint was performed. RESULTS: Our cohort included 86 cases; 41 had a vascular ring. Rates of chromosomal abnormalities, 22q11.2 deletion, and fetal ECA were 14.1%, 6.4% and 17.4% respectively. Sixteen studies including our cohort (312 fetuses) were included in the systematic review. Overall chromosomal abnormalities and 22q11.2 deletion rates were 9.0% (95% CI 6.0-12.5) and 6.1% (95% CI 3.6-9.3) whilst rates for cases with no ECA were 4.6% (95% CI 2.3-7.8) and 5.1% (95% CI 2.4-8.6). ECA were seen in 14.6% (95% CI 10.6-19.0) prenatally and 4.0% (95%CI 1.5-7.6) after birth. Postnatal ICA were identified in 5.0% (95% CI 2.7-7.9). Rate of symptoms (follow up ≥24 months) was 25.2% (95% CI 16.6-35.0) while 17.1% (95% CI 9.9-25.7) had surgery. Two-year freedom from surgery was 83.0% (95% CI 74.3-90.1) CONCLUSIONS: Fetal RAA without ICA is more frequently associated with ECA than chromosomal abnormalities. Most cases however, are isolated. Vascular ring symptoms occur in about 25% of cases. Postnatal surveillance is required mainly in the first 2 years of life.