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Virtual Pediatric Hospital: Paediapaedia: Right Aortic Arch with Aberrant Left Subclavian Artery Paediapaedia: Cardiovascular Diseases

Right Aortic Arch with Aberrant Left Subclavian Artery

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


Clinical Presentation:
Usually an incidental finding on a CXR since the ring is rarely tight enough to cause symptoms.

Etiology/Pathophysiology:
This is the most common malformation of the aortic arch resulting in a complete vascular ring around the trachea and esophagus.

Two types exist:

The most common type of right aortic arch is right aortic arch with an aberrant left subclavian artery and it is 2-3 times more common than right arch with mirror image branching of the brachiocephalic vessels. Right arch and aberrant left subclavian artery has a 5-12% incidence of associated congenital heart disease while right arch with mirror branching of the brachiocephalic vessels has a 98% incidence of associated congenital heart disease.

Pathology:
Not applicable

Imaging Findings:
The chest film shows a right sided aortic arch. The esophagram shows a posterior indentation on the esophagus.

DDX:

References:

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