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.

Aortic arch anomalies are present in 1% to 2% of the general population and are commonly associated with congenital heart disease, chromosomal defects, and tracheaesophageal compression in postnatal life. The sonographically based detection of aortic arch anomalies lies in the 3-vessel and trachea view. Although highly sensitive, this view alone does not allow identification of the aortic arch branching pattern, which prevents an accurate diagnosis. The systematic addition of a subclavian artery view as part of a standardized procedure may be useful in the differential diagnosis of these conditions. We describe the sonographic assessment of fetal aortic arch anomalies by combining 2 fetal transverse views: the 3-vessel and trachea view and the subclavian artery view, which are included in the cardiovascular system sonographic evaluation protocol. We also review the sonographic findings and the clinical implications of fetal aortic arch anomalies.

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