A double aortic arch is a relatively uncommon anomaly occasionally associated with congenital heart disease or the chromosome 22q11 deletion. We report a case of prenatal diagnosis of a double aortic arch in which the sonographic features in the 3-vessel and trachea view are highlighted. A PubMed-based search was made to retrieve all cases of prenatal diagnosis of double aortic arch. A total of 13 articles and 35 cases were found. The average gestational age at diagnosis was 29 weeks. Six cases had associated cardiac anomalies. Only 1 case had the 22q11 deletion, showing extracardiac anomalies without cardiac defect. The postnatal evolution was characterized by symptoms of tracheoesophageal compression in 72.4% of the cases. Detection of a double aortic arch should be followed by a thorough fetal scan and echocardiography, and a chromosomal study should be considered when the sonographic findings are consistent with the 22q11 deletion.
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