Right aortic arch with left aberrant subclavian artery and azygos continuation of IVC

Case contributed by Jose Ignacio Aragon
Diagnosis certain

Presentation

Further evaluation of a right aortic arch previously seen on a CXR

Patient Data

Age: 10 years
Gender: Female

The aortic arch is on the right side in relation with the trachea. 

The aberrant left subclavian artery is the branch that arises from Kommerell diverticulum at the distal end of the aortic arch. These findings are related to a right aortic arch with aberrant left subclavian artery. 

Also, there is dilatation of the azygos vein that demonstrates azygos continuation of the IVC.  The azygos vein drains in the superior vena cava. 

On the sagital MIP projection outlined with arrows is the suprahepatic IVC and the arch of azygos vein draining into the SVC. 

Case Discussion

The right aortic arch and aberrant left subclavian artery occurred as the result of an interruption of the dorsal segment of the left arch between the left common carotid and left subclavian arteries with regression of the right ductus arteriosus in the hypothetical double aortic arch.

Kommerell diverticulum occurs in some anomalies of the aortic arch system. It refers to the bulbous configuration of the origin of an aberrant left subclavian artery in the setting of a right sided-aortic arch. The prevalence of a right sided-aortic arch is <1%. 

Continuation of the suprarenal IVC as the azygos or hemiazygos vein is attributable to embryonic failure to form the right subcardinal–hepatic anastomosis. The continuation of the azygos as the IVC has a prevalence of 0.6%.

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