Vascular Rings

A vascular ring occurs when the aorta forms abnormally during fetal development. The aorta is the main artery that carries oxygenated blood away from the heart to the rest of the body. The defect presses on the trachea, esophagus or both, causing problems with breathing or eating.

A vascular ring is a congenital defect, meaning it’s present at birth. The condition can be life-threatening, but surgery is usually successful.

Normally, the aorta starts in the left ventricle (lower left chamber) of your baby’s heart. It travels up the side of their trachea (windpipe) and forms an arch (called the aortic arch). It then goes back down the side of their trachea (the descending aorta). The aorta branches off several times at the aortic arch and descending aorta. This creates arteries that deliver blood to various parts of your baby’s body.

But with a vascular ring, the aorta or its branches partially or completely wrap around your baby’s trachea, esophagus or both. Because the trachea is the tube that delivers air to and from their lungs, a vascular ring can cause breathing problems. The esophagus is a tube that carries food and liquids from your baby’s mouth to your stomach. So a vascular ring can also cause feeding/digestive issues.

What are the different kinds of vascular rings?

A vascular ring can form in many different ways. The three most common are:

• Double aortic arch: The aorta starts normally but then divides into two arches, surrounding and compressing your baby’s trachea and esophagus. The arches come back together as one blood vessel on the other side.
• Right aortic arch: The aorta starts abnormally, to the right of your baby’s trachea instead of the left. It gives off a normal blood vessel that arises abnormally and passes behind their esophagus. This branch then gives rise to another artery that travels to your baby’s left arm (left subclavian artery). The ring is completed by the remnant of a blood vessel that’s open in fetal development (ligamentum arteriosum). These blood vessels can constrict your baby’s trachea and/or esophagus.
• Aberrant (abnormal) right subclavian artery (RSA): This artery normally branches off the brachiocephalic trunk, one of the three arteries that grow from the aortic arch. But an aberrant RSA grows directly from the arch. Also called arteria lusoria, this type of vascular ring is not a complete ring and may not produce symptoms.

How common are vascular rings?

Vascular rings are rare, occurring in less than 1% of babies. They represent only about 1% to 3% of all congenital heart defects.

Who might get a vascular ring?

The condition can affect any ethnicity. For unknown reasons, it’s about twice as common in babies assigned male at birth as in babies assigned female at birth.