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