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 describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and review other malformations of the aortic arch, including interrupted aortic arch (IAA), hypoplastic aortic arch, and coarctation of aorta. I have also described few anomalies of pulmonary artery (PA) origin and bifurcation like PA sling which has clinical presentation similar to vascular ring and anomalous origin of PA from aorta which present as large aorto-pulmonary communication with congestive heart failure. 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.

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