Fetuses with right aortic arch: a multicenter cohort study and meta‐analysis

ABSTRACT Objectives Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of fetal RAA without intracardiac abnormalities (ICA) to guide postnatal management. Methods In the retrospective cohort part of our study, outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extracardiac abnormalities (ECA), postnatal ICA and ECA, and symptoms of and surgery for vascular ring. A systematic review and meta‐analysis was also 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 rates of chromosomal abnormalities and 22q11.2 deletion were 9.0% (95% CI, 6.0–12.5%) and 6.1% (95% CI, 3.6–9.3%), whilst the respective 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 in 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 of vascular rings (follow‐up ≥ 24 months postpartum) was 25.2% (95% CI, 16.6–35.0%), and 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 with 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 after delivery. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. RESUMEN Objetivos El uso reciente de protocolos de cribado prenatal de anomalías cardíacas ha aumentado los diagnósticos de fetos con arco aórtico derecho (AAD). El objetivo de este estudio fue tratar de establecer los resultados perinatales en fetos con AAD sin anomalías intracardíacas (ICA, por sus siglas en inglés) como guía para el cuidado postnatal. Métodos En la parte de cohorte retrospectiva de nuestro estudio, los parámetros estudiados fueron las tasas de anomalías cromosómicas, la deleción 22q11.2, las anomalías fetales extracardíacas (ECA, por sus siglas en inglés), las ICA y ECA postnatales, y los sí