Congenital Disease of the Aortic Arch

Congenital aortic arch anomalies are the result of errors in the progression of division of the conotruncus and regression and development of the embryologic aortic arches during weeks 4 through 7 of gestation. These errors occur in 1–2% of the population and may be an isolated finding or associated with a variety of genetic chromosomal defects or congenital heart disease [1]. Several hypothetical variations in regression or maintained patency of the paired embryologic aortic arches have been well-described, including aortic arch sidedness as determined by arch position to the left or right of the trachea, the branching order of brachiocephalic arteries from the aortic arch, and the position of the aortic arch in the mediastinum or cervical location [2–4]. Anomalies of the aortic arch are symptomatic because of obstructed blood flow or disruption of adjacent normal anatomic relationships. Chromosomal deletion of 22q11 is an important factor; 75% of children with this deletion have congenital heart disease, and up to 24% of patients with this deletion have aortic arch anomalies [5, 6] (Table 1).