Right aortic arch with aberrant left subclavian artery

Case contributed by Dr Julian Maingard


30 yo male with a history of 22q11.2 deletion syndrome (DiGeorge Syndrome) and perimembranous VSD presents with 3 months of dysphagia and 20kg LOW.

Patient Data

Age: 30
Gender: Male

Right sided aortic arch. There is an associated aberrant left retro-esophageal subclavian artery which results in moderate compression of the esophagus, just inferior to the thoracic inlet. There is also mild narrowing of the trachea, approximately 5-10mm above the carina, secondary to this vascular impression. Normal caliber of the ascending and descending thoracic aorta.

Case Discussion

​Congenital anomalies of the heart are uncommon and commonly associated with other congenital cardiac diseases1.

The above case if that of a right sided aortic arch with an aberrant left retro-esophageal subclavian artery. Right sided aortic arch occurs in 0.05% of the population1. Right sided aortic arch's are classified into 3 different types. Their classification is dependent on the branching pattern of the aortic arch vessels1,2. This case is a type II aortic arch which is relatively common accounting for 39.5% of cases 3.

While often an incidental finding on imaging symptomatic patients commonly present with dysphagia due to extrinsic esophageal compression1.

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Case information

rID: 31501
Published: 14th Oct 2014
Last edited: 14th Aug 2019
System: Vascular, Cardiac
Inclusion in quiz mode: Included

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