A vascular ring is a malformation of the aortic arch anatomy, where vessels partly or completely encircle the trachea and esophagus. A normal heart with a normal aortic arch is shown on the left. An example of a vascular ring — a double aortic arch — is shown on the right.

What causes a vascular ring?

A vascular ring occurs when the aorta (the large artery that carries oxygenated blood out of the heart) or its branches form a complete ring around the trachea (the tube that carries air to the lungs) and the esophagus (the tube that carries food to the stomach).

How rare is a vascular ring?

Vascular ring is rare. It accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem.