This defect may go undiagnosed as the symptoms are often mild. A Barium Swallow Study may be used to help identify the problem. If there is significant interference with breathing or swallowing, surgery may be performed.

Surgical treatment involves the tying off and separation of one of the branches of the double aortic arch. Sometimes, one of the branches is already closed (atretic), with no blood flowing through it. In this case, it is simply clipped or tied off and separated from the other branch.

In cases where blood flows through both branches, the smaller vessel is tied off and then severed at a point between the subclavian artery and where the smaller branch joins the larger branch to form the descending aorta. The ends of the remaining sections of the aorta are then closed with sutures.