Vascular Rings

What is a vascular ring?

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.

What causes a vascular ring anomaly?

For reasons scientists don’t understand yet, vascular rings occur during fetal development. Often, parts of the aorta that should disappear during fetal development don’t go away.

What are the symptoms of a vascular ring?

Depending on the type and severity, vascular ring symptoms may appear during infancy, later in life or not at all. People with vascular rings may have visited a pulmonologist (lung specialist) or gastroenterologist (digestion specialist) for long-lasting symptoms. But they may not have gotten a diagnosis.

If the vascular ring affects your (or your baby's) trachea, you may experience symptoms such as:

• Cough.
• Frequent respiratory infections.
• Nasal flaring (nostrils widen during breathing).
• Noisy breathing (wheezing or stridor).
• Respiratory cyanosis (bluish skin).

If the vascular ring affects your (or your baby's) esophagus, you may experience symptoms including:

• Acid reflux.
• Failure to thrive (failure to gain weight at a normal pace).
• Feeling like something is stuck in your throat.
• Trouble eating or frequent choking when eating.
• Trouble swallowing (dysphagia), especially solid foods.
• Vomiting.

People with vascular rings may have other congenital heart defects, which may lead to symptoms such as:

• Heart murmur.
• Heart palpitations.
• Shortness of breath (dyspnea).

How is a vascular ring diagnosed?

A healthcare provider may order some of the following tests to diagnose a vascular ring:

• Chest X-ray: A chest X-ray produces pictures of the inside of your baby's body. It’s often one of the first tests performed during the diagnosis process.
• CT scan: A CT scan takes pictures of internal structures, including your baby's heart, trachea and esophagus.
• Echocardiogram: Also called an echo, an echocardiogram creates moving pictures of your baby's heart and aorta.

The healthcare provider may order other tests to evaluate your (or your baby's) trachea or esophagus, such as:

• Barium swallow (esophagogram): This test involves swallowing a substance called barium. X-rays show how the barium moves through your esophagus. The test can identify an indentation caused by a vascular ring.
• Bronchoscopy: For a bronchoscopy, a healthcare provider will place a tube through your mouth or nose into your lungs. The tube has a light and small camera so the healthcare provider can see inside your airways.
• Upper endoscopy: During an upper endoscopy, a tube goes through your mouth and into your throat. The small light and camera allow the healthcare provider to see inside your esophagus.

How are vascular rings treated?

Vascular rings that are pressing on your trachea or esophagus require surgery. Often, these don't need to be repaired in early infancy, except for double aortic arches.

The procedure performed depends on the type and severity of your baby's condition. Vascular ring surgery usually involves:

• Dividing part of the vascular ring to reduce pressure.
• Removing a piece of the aorta or artery.

Surgery is often an open procedure through the side of your baby's chest (thoracotomy), requiring larger incisions. Rarely in larger children, a minimally invasive procedure is possible.

Can I reduce the risk of a vascular ring?

Scientists don’t understand what causes vascular rings to form as a fetus develops, so there are no strategies to prevent them.

What can I expect if I have this condition?

Surgery to correct a vascular ring is quite safe and often successful. Although you or your child may have symptoms for a few months afterward, most people are symptom-free within a year of surgery. Most people don’t need follow-up surgeries, but it's possible.

You may need to follow up with specialists for many years, including:

• Cardiologist (heart specialist).
• Gastroenterologist (digestive specialist).
• Pulmonologist (respiratory specialist).

What else should I ask my healthcare provider about a vascular ring?

If you or your child has a vascular ring, consider asking your healthcare provider the following questions:

• What type of vascular ring is it?
• Is it wrapped around my trachea, esophagus or both?
• Do you recommend surgery?
• If so, will the surgery be open or minimally invasive?
• How long will it take to recover?
• Will my symptoms go away after surgery?
• Should I see any other specialists?
• Do I have any other heart abnormalities?
• If so, can they be treated surgically at the same time?

A note from Cleveland Clinic

A vascular ring is an abnormal formation of the aorta that presses on the trachea, esophagus or both. The congenital defect can cause problems with breathing or eating. If you or your child has a vascular ring, surgery can correct the condition and eliminate symptoms.