Right aortic arch with aberrant left subclavian artery

Case contributed by Julian Maingard

Presentation

History of 22q11.2 deletion syndrome (DiGeorge syndrome) and perimembranous VSD presents with 3 months of dysphagia and 20kg LOW.

Patient Data

Age: 30 years
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 5-10 mm 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 diseases 1.

The above case is 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 population 1. Right-sided aortic arches are classified into 3 different types. Their classification is dependent on the branching pattern of the aortic arch vessels 1,2. This case is a type II right-sided 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 compression 1.

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