Vascular ring

A vascular ring is a rare congenital heart condition that may make breathing and eating difficult for your baby. No one knows what causes these birth defects so there’s no way to prevent them.

How serious is a vascular ring?

Having a vascular ring can be mild or serious. As blood vessels develop in your baby’s chest during pregnancy, some vessels may form abnormally so that they wrap around the trachea (windpipe) or esophagus. The trachea lets air flow to your baby’s lungs and the esophagus carries food to your baby’s stomach. If the vascular ring puts pressure on either the trachea or the esophagus, it can make it hard for your baby to breathe or swallow.

Vascular ring symptoms

Symptoms may show up when your baby is very young, although some children don’t have symptoms. When a baby or child has vascular ring symptoms, they may have some of the following problems with breathing:

• Loud breathing (stridor)
• Wheezing
• Cough
• Repeated pneumonias or respiratory infections
• Respiratory distress

More rarely, infants and children have vascular ring symptoms related to digestion such as:

• Difficulty swallowing (dysphagia)
• Slow breast or bottle feeding
• Choking
• Difficulty eating solid foods
• Gastroesophageal reflux disease (GERD)
• Spitting up (vomiting)

Complications from an untreated vascular ring may include damage to the trachea.

What’s one common type of vascular ring?

Each vascular ring that forms is unique. One common type of vascular ring is called a double aortic arch. When a double aortic arch forms, the aorta forms abnormally and causes a vascular ring.

A normal aorta passes on one side of the windpipe and esophagus. In a double aortic arch, the aorta forms a second branch that passes on the other side of the windpipe and esophagus. The abnormal branches of the aorta rejoin on the other side to form a vascular ring.

Complete vascular ring

A complete vascular ring is just what it sounds like. Abnormal tissue connects in a way that forms a complete ring around the trachea and esophagus. A double aortic arch is a complete vascular ring.

 Incomplete vascular ring

An incomplete vascular ring happens when the abnormal tissue doesn’t form a complete ring around the trachea, the esophagus or both. Incomplete vascular rings can still cause symptoms when they put pressure on the trachea, esophagus or both

How does a doctor diagnose a vascular ring?

When your child has a vascular ring, symptoms may appear in the first few months of life. Their doctor will perform a full physical exam, including listening to their heart.

Several cardiac imaging tests may be suggested to confirm a diagnosis and help plan for potential surgery. Imaging lets your child’s doctor evaluate the shape, size and position of the vascular ring. Some of the tests they may recommend are:

• Echocardiogram
• Chest X-ray
• CT scan of the heart, blood vessels and surrounding tissues
• MRI of heart and blood vessels
• Angiography (X-ray of blood vessels)

Your child’s doctor and health care team will clearly explain what is going on with your child’s heart and blood vessels.

Vascular ring treatment

If your child has no symptoms, their doctor may recommend they be monitored regularly for changes or complications.

Surgery will be recommended when a vascular ring causes symptoms. Vascular ring surgery to relieve the abnormal formation of the blood vessels lessens pressure, alleviates symptoms and lets your child lead a normal life. Your child’s symptoms may resolve quickly after surgery.

Whether your child has surgery or not, they’ll need to be monitored. Depending on their condition, your child may see specialists in cardiology, gastroenterology or pulmonology.

Contact us with your questions about heart care for your child.

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