Abnormalities of the Aortic Arch

Congenital abnormalities of the aortic arch, which take many forms, result from aberrant development of one or more components of the embryonic pharyngeal arch system and represent less than 1% of all congenital cardiac defects. They occur with about equal frequency in both sexes with no geographical or racial predominance. There are five primary groups of anatomic arch anomalies: double aortic arch, right aortic arch with mirror-image (i.e., normal) branching, right aortic arch with abnormal branching, left aortic arch with abnormal branching, and cervical aortic arch. Aortic arch anomalies are associated with a chromosome 22q11 deletion in approximately 20% of patients. Clinically, aortic arch anomalies can be divided into those that cause (or are likely to cause) physiologic abnormalities, and those that do not. Physiologic abnormalities resulting from aortic arch anomalies include tracheobronchial compression, esophageal compression, and abnormal blood flow patterns. The location and severity of compression varies with the configuration of the lesion. Prompt diagnosis and treatment of these congenital abnormalities can be lifesaving. Surgical division of the vascular ring is indicated in any patient with symptoms of airway or esophageal compression.