Anomalies of the Aortic Arch

Developmental anomalies of the aortic arch are a heterogeneous group of disorders with a broad spectrum of clinical manifestations ranging from being an incident finding on diagnostic imaging studies to severe symptomatic compression of the airways and digestive tract. Insight into the multidimensional anatomic relations of arch vessels, trachea, esophagus, and their embryologic precursors are key to understanding aortic arch anomalies and its variants. Anomalies of the arch either can manifest as an isolated lesion or associated with other congenital heart diseases. 22q11.2 deletion syndrome has been observed in many cases when intracardiac anomalies coexist with aortic arch anomalies. The prevalence of aortic arch anomalies is often underestimated as they may be frequently asymptomatic, particularly in the absence of a complete vascular ring. CT angiography and MRI are increasingly used for accurate diagnosis and to describe three-dimensional (3D) anatomical relationships to neighboring structures. Though echocardiography is often performed mainly to diagnose underlying heart disease, it can accurately depict the course and branching pattern of the aortic arch aiding in the planning of subsequent cross-sectional imaging studies. For symptomatic patients, surgical release of a constricting band often provides symptomatic relief; in some cases, reimplantation of an aberrant vessel may be required, persistence of residual symptoms may be seen in some cases. High-risk patients may require long-term follow-up. Although coarctation of the aorta is associated with anomalies of the aortic arch; Coarctation, as the primary leision,is discussed in chapter 66 (coarctation of the aorta) of this textbook.