Prenatal double aortic arch presenting with a right aortic arch and an anomalous artery arising from the ascending aorta

Abstract

Double aortic arch is a rare anomaly of the aortic arch and this may cause tracheal damage by compressing the trachea during the first year of life. We report here on three fetal cases of double aortic arch and their outcomes. These malformations presented as an isolated right aortic arch or as a typical vascular ring form on the prenatal echocardiography that was obtained between 22 and 24 weeks gestation. In two of the three patients, the major intracardiac anomaly was a ventricular septal defect, and this was associated with the aortic malformation. A more detailed evaluation of the branching pattern of the aorta, and particularly whether the aorta gives off a left-sided limb of the double arch, could suggest the diagnosis of a double aortic arch in utero. Two patients underwent successful surgical correction and they showed good clinical outcomes. Fetal double aortic arch can be prenatally suspected after performing fetal echocardiography for those fetuses that show a right side aortic arch, and this can help to avoid delaying the postnatal management of this rare anomaly.