Vascular ring

Overview

A vascular ring occurs when the aorta grows incorrectly during fetal development. It is a congenital heart defect. The deformity strains the trachea, esophagus, or both, causing breathing or swallowing difficulties.

The aorta is the primary artery that transports oxygenated blood from the heart to the rest of the body. In a typical scenario, the aorta originates from the left ventricle of a baby’s heart and travels upward alongside the trachea, forming the aortic arch. From there, it descends on the side of the trachea, which is known as the descending aorta. With a vascular ring, aorta or its branches partially or completely form a ring around your baby’s trachea, esophagus, or both.

A vascular ring can be complete or incomplete. A complete vascular ring surrounds both the esophagus and the trachea. An incomplete vascular ring fails to surround the esophagus or trachea. A vascular ring may also develop in a variety of ways. The three most prevalent are:

• Double aortic arch: The aorta begins properly, but soon separates into two arches and on the other side, the arches rejoin as one blood vessel. This wraps and squeezes the infant’s trachea and esophagus.
• Right aortic arch: In this case, the aorta of the baby originates to the right of their trachea instead of the left. One of its branches, which develops unusually, passes behind the baby’s esophagus and gives rise to another artery that goes to their left arm (known as the left subclavian artery). The ring is completed by the presence of a remnant of a blood vessel that was open during fetal development, which is now known as the ligamentum arteriosum.

These blood arteries have the potential to restrict your baby’s trachea and/or esophagus.

• Aberrant (abnormal) right subclavian artery (RSA): Also known as arteria lusoria. This artery usually arises from the brachiocephalic trunk, one of the three arteries that arise from the aortic arch. But an abnormal RSA develops directly from the arch. This form of vascular ring is not a complete ring and may not cause symptoms.

Since the trachea is the tube that transports air to and from the lungs, a vascular ring might cause breathing problems. It might also create feeding and digestion problems when the esophagus cannot efficiently transport food as it should be. In most cases, a vascular ring may be treated with surgery.

Symptoms

Depending on the type and degree, vascular ring symptoms may develop during childhood, later in life, or never. Some people who are born with a vascular ring may also have additional heart abnormalities. The type of heart issues present determines the specific symptoms.

Several symptoms are associated with vascular ring, such as:

• Cough
• Respiratory infections
• Wheezing or noisy breathing
• Swallowing difficulties
• Having difficulty eating
• Vomiting
• Inability to gain weight normally

Cause

The cause of vascular ring, a medical condition, is unknown and it develops during fetal growth. Usually, certain portions of the aorta that are meant to disappear during fetal development do not undergo regression.

Risk factor

Vascular rings are a rare occurrence, affecting fewer than 1% of infants and representing only 1% to 3% of all cases of congenital heart defects. Although anyone can develop a vascular ring, it appears to be more common in male newborns compared to females.