Congenital Variants and Anomalies of the Aortic Arch

Abstract

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. ^©RSNA, 2016.