Right aortic arch with aberrant left subclavian artery
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.
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Right sided aortic arch. There is an associated aberrant left retro-oesophageal subclavian artery which results in moderate compression of the oesophagus, 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 calibre of the ascending and descending thoracic aorta.
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-oesophageal 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 oesophageal compression1.
- 1. Türkvatan A, Büyükbayraktar FG, Olçer T et-al. Congenital anomalies of the aortic arch: evaluation with the use of multidetector computed tomography. Korean J Radiol. 2009;10 (2): 176-84. doi:10.3348/kjr.2009.10.2.176 - Free text at pubmed - Pubmed citation
- 2. Kimura-Hayama ET, MeléNdez G, MendizáBal AL et-al. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 2010;30 (1): 79-98. Radiographics (full text) - doi:10.1148/rg.301095061 - Pubmed citation
- 3. Chai OH, Han EH, Kim HT et-al. Right-sided aortic arch with the retroesophageal left subclavian artery as the fourth branch. Anat Cell Biol. 2013;46 (2): 167-70. doi:10.5115/acb.2013.46.2.167 - Free text at pubmed - Pubmed citation