Vascular Ring: A Guide to This Congenital Heart Irregularity

Medically Reviewed By John A. Moawad, MD, FACS
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A vascular ring is a rare vascular irregularity when the aortic arch presses on the trachea and esophagus. It causes respiratory and gastrointestinal symptoms. There are several types of vascular ring. Surgery can treat vascular ring when symptoms make surgery necessary.

This article explains what a vascular ring is, the types of vascular ring, symptoms, and how it is treated.

What is a vascular ring?

A doctor is listening to a persons heart and breathing.
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A vascular ring is a condition when vascular structures encircle and compress the trachea and esophagus. It is usually a result of a congenital defect present at birth.

The compression of the trachea and esophagus causes respiratory and gastrointestinal symptoms. The aorta and its branches are vascular structures that usually form the vascular ring.

The aorta arches up to the left of the trachea and then down. At the top of the aortic arch are arteries that deliver blood to the head, arms, and other upper body parts. The descending aorta contains arteries that supply blood to the abdomen, legs, and parts of the lower body.

What are the types of vascular ring?

There are three types of vascular ring:

  • Double aortic arch. This type contains two aortic arches. One of the arches is large, and the other is small and underdeveloped. The branches of the two aortic arches surround the esophagus and trachea.
  • Right aortic arch. In this type, the aortic arch curves right, and the left subclavian artery passes behind the esophagus and trachea instead of in front.
  • Left aortic arch. In this type, the right subclavian artery appears behind the esophagus and trachea, compressing these structures.

What are the symptoms of a vascular ring?

Signs and symptoms of vascular ring usually affect the respiratory and gastrointestinal systems. Common symptoms include:

  • noisy breathing, especially with eating
  • difficulty eating or swallowing
  • regurgitating or choking on food
  • the feeling of something stuck in the throat
  • acid reflux
  • persistent cough
  • respiratory infections

What causes a vascular ring?

A vascular ring is formed in utero. Usually, by the end of the second month of fetal development, the branches of the aortic arches have formed into arteries. However, when a vascular ring forms, the branches remain in place and form a ring.

What are the risk factors for a vascular ring?

Vascular ring remains a rare congenital irregularity. However, people assigned male at birth have a 1.4 to 2 times greater risk of developing the condition than females.

Vascular ring is not genetic and does not appear to run in families.

How do you prevent a vascular ring?

There is no way to prevent a vascular ring, but early recognition of symptoms can help prompt the right form of treatment.

Vascular ring is usually not visible on prenatal ultrasound. Some people may not show any signs until they are into adulthood.

If your child is showing symptoms of a vascular ring, it’s important to have them seen by their pediatrician for diagnosis and early treatment.

How do doctors diagnose a vascular ring?

Most cases of vascular ring are mild. Some symptoms can appear at several weeks or months of age.

A doctor will perform a physical exam on your child, paying attention to their respiratory and gastrointestinal systems.

The doctor may order several tests to help diagnose a vascular ring, including:

How do you treat a vascular ring?

Surgery is usually the only way to treat a vascular ring. For children with no symptoms, surgery is unnecessary, and their doctor will continue monitoring their condition.

During surgery for vascular ring, the surgeon makes an incision on the left side of the chest in between the ribs to access the area. The surgery is not an open heart procedure, and risks are typically minimal. Hospitalization after surgery is usually only 1–2 days.

How does a vascular ring affect quality of life?

Most people, up to 70%, become asymptomatic within a year after surgery. However, respiratory symptoms may persist for 4–6 months after surgery.

In children with a vascular ring, respiratory symptoms can cause distress and prevent them from performing their usual activities or participating in sports. Gastrointestinal symptoms can make eating difficult.

You can help your child cope with the condition by talking with their doctor about ways to manage persistent coughing or other respiratory symptoms. If your child has trouble eating, you can try serving them smaller meals with foods that are easy to swallow.

What are the potential complications of a vascular ring?

Complications of a vascular ring usually affect the trachea and esophagus due to the compression of these structures. Examples of complications include:

  • laryngeal nerve injury
  • vocal cord paralysis
  • thoracic duct injury
  • pleural effusion
  • narrowing of blood vessels, bleeding, and clotting

Frequently asked questions

Here are some frequently asked questions about vascular ring.

How serious is vascular ring?

Most cases of vascular ring are mild and rarely result in serious symptoms, such as respiratory arrest or apnea. However, symptoms can be distressing and interfere with quality of life. It’s important to talk with your doctor if your child is experiencing symptoms of a vascular ring.

Are vascular rings genetic?

Vascular rings are not genetic and do not appear to run in families. Vascular rings usually form early on in fetal development.

Do all vascular rings need surgery?

Not all vascular rings will require surgery. If your child has no symptoms, they do not need surgery, and their condition will continue to be monitored by their doctor. Most people who have surgery report relief of symptoms.

Summary

A vascular ring is a rare congenital defect that occurs when vascular structures surround and compress the trachea and esophagus. It usually forms in the 4th or 5th week of gestation when the branches of the aorta form into arteries and blood vessels.

A vascular ring causes respiratory and gastrointestinal symptoms such as noisy breathing and trouble eating and swallowing. Surgery is often the only way to correct a vascular ring. In most cases, surgery will help relieve the symptoms associated with a vascular ring.

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Medical Reviewer: John A. Moawad, MD, FACS
Last Review Date: 2022 Oct 28
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