Vascular Rings

A vascular ring happens when blood vessels surround your trachea (airway) and esophagus (food pipe), forming a ring. The ring can press on your trachea, esophagus or both. This can cause problems with breathing and/or eating.

It’s a rare birth defect (congenital condition) that can occur when the aorta (the largest artery) forms differently during fetal development. Symptoms of vascular rings may appear during infancy, later in life or not at all. It depends on the underlying cause and severity. The condition can be life-threatening, but surgery is often successful.

Symptoms of vascular rings

If the vascular ring affects your trachea (airway), symptoms may include:

• Cough
• Frequent upper respiratory infections, which can lead to pneumonia
• Nasal flaring (nostrils widen during breathing)
• Noisy breathing (wheezing or stridor)
• Respiratory cyanosis (bluish skin)

If the vascular ring affects your esophagus (food pipe), symptoms may include:

• Acid reflux
• Feeling like something is stuck in your throat
• Growth faltering in infants
• Trouble eating or frequent choking when eating
• Trouble swallowing (dysphagia), especially solid foods
• Vomiting

If you have a vascular ring, you may have other congenital heart diseases. They could cause symptoms like heart murmur and heart palpitations.

Vascular ring causes

Vascular rings form due to aortic arch anomalies (differences). These differences come about during fetal development for unknown reasons. Often, parts of the aorta that should disappear during fetal development don’t go away. Healthcare providers call these remnants.

The two most common anomalies that lead to vascular rings are double aortic arch and right aortic arch:

• Double aortic arch (DAA): Your baby’s aorta starts typically, but then divides into two arches (instead of one). They surround your baby’s trachea and esophagus. The arches come back together as one blood vessel on the other side.
• Right aortic arch (RAA): Your baby’s aorta starts to the right of their trachea instead of the left. It gives off a blood vessel that arises abnormally and passes behind their esophagus. This branch then gives rise to another artery. A remnant of a blood vessel completes the ring.

Complications of this condition

Some vascular rings exist into adulthood without detection or complications. In other cases, vascular rings may cause problems like:

• Repeated laryngeal nerve injury and vocal cord paralysis
• Thoracic duct injury and chyle leak
• Tracheomalacia (collapsed airway)
• Vascular complications, like narrowing (stenosis), bleeding and blood clots (thrombosis)

How doctors diagnose this condition

Healthcare providers may detect vascular rings during a pregnancy ultrasound or fetal echocardiogram. At any point after birth, your provider may recommend some of the following imaging tests to help diagnose a vascular ring:

• Chest X-ray
• CT scan
• Echocardiogram

The gold standard for looking at vascular rings is a CT chest angiogram. It shows how your blood flows through your heart and blood vessels. This allows your provider to see your vascular anatomy in detail.

You may need other tests to further evaluate how the vascular ring is affecting your trachea or esophagus, like:

• Barium swallow (esophagogram): X-rays show how barium (a liquid) moves through your esophagus. It can identify an indentation caused by a vascular ring.
• Bronchoscopy: A provider places a lighted tube through your mouth or nose and into your lungs so they can see inside your airways.
• Upper endoscopy: A provider places a lighted tube through your mouth and into your throat to see inside your esophagus.

How are vascular rings treated?

The only treatment for vascular rings is surgery. Most healthcare providers only recommend surgery if you have symptoms.

The surgery is often an open procedure (thoracotomy). This means you’ll need an incision in your chest wall. But in some cases, you may be able to get a less invasive type of surgery. These include endoscopic robotic-assisted surgery and video-assisted thoracoscopic surgery (VATS).

The type of surgery depends on the aortic arch anomaly and how much it’s squeezing your trachea and/or esophagus. In general, vascular ring surgery usually involves:

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

Your healthcare team will go over the procedure in detail. This includes its steps, risks and recovery.

What should I ask my healthcare provider?

If you or your child has a vascular ring, consider asking your 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?

What can I expect if I have this condition?

Surgery to correct a vascular ring is safe overall and often successful. You may have symptoms for a few months afterward. But most people are symptom-free within a year of surgery.

After treatment, you may need to follow up with specialists for many years, including a:

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

What is the life expectancy of someone with a vascular ring?

According to studies, most people who get vascular ring surgery have a typical life expectancy. Without treatment, some cases of vascular rings can be fatal. They can damage your trachea, making it very difficult to breathe.