Double aortic arch is the most common vascular ring due to persistence of the fourth aortic arches bilaterally. The ascending aorta is split into right and left aortic arches with the former is usually the dominant, higher and larger. The aortic arches join posteriorly to form the descending aorta. 

The double aortic arches forms a complete symptomatic vascular ring that encircles the esophagus and trachea giving the symptoms of dyspnea and stridor (usually in the infancy and childhood) and dysphagia (more common in the adulthood).

Association of other congenital heart disease is rare.