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Virtual Pediatric Hospital: Paediapaedia: Double Aortic Arch Paediapaedia: Cardiovascular Diseases

Double Aortic Arch

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Symptoms are related to tracheal compression (respiratory distress, wheezing, tachypnea) or esophageal compression (dysphagia, choking, aspiration, vomiting). A vascular ring is more likely to be symptomatic if it is complete.

Etiology/Pathophysiology:
The ascending aorta arises anterior to the trachea and divides into 2 arches which pass posterior and to the right and anterior and to the left of the trachea and esophagus, creating the vascular ring. The right arch gives off the right common carotid and subclavian arteries and the left arch gives off the left common carotid and subclavian arteries.The two arches rejoin posteriorly to form the descending aorta.

There are two types:

Pathology:
Not applicable

Imaging Findings:
The posterior right arch is more promient and higher than the left arch. The esophagram on the lateral view shows variable anterior compression of the trachea and a large posterior indentation of the esophagus by the right arch. The esophagram on the AP view shows bilateral compression of the esophagus forming a reverse S sign with the superior curve due to compression from the higher right arch and the inferior curve due to compression from the left arch.

DDX:

References:

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