Vascular rings

Date Updated: 12/30/2020

Overview

Vascular rings are malformations of the aortic arch in the main blood vessel that leads from the heart. Because of the malformation, the aortic arch and its branches partly or completely encircle the windpipe (trachea), the esophagus or both. The esophagus is the tube that leads from your mouth to your stomach.

These defects are present at birth (congenital), but symptoms can occur in infancy or later in life. Symptoms are caused by some portion of the ring pressing against the trachea or esophagus or both.

Some people with vascular rings don't have symptoms, but vascular rings that press against the trachea and esophagus can lead to breathing and digestive problems. Respiratory signs and symptoms may include:

Frequent respiratory infections
Wheezing
Coughing

Digestive signs and symptoms may include:

Trouble swallowing
Difficulty feeding
Vomiting

People with vascular rings may also have other congenital heart defects.

Diagnosis

To diagnose vascular rings and rule out other conditions that may be causing the symptoms, your doctor may review your child's signs and symptoms, do a physical exam, and order tests that may include:

Imaging tests. A chest X-ray can show which side of the body the aortic arch is on, and any changes in the trachea that may suggest that a vascular ring is present.

Other imaging tests may be done to diagnose vascular rings, such as an echocardiogram or a CT angiogram or MRI. Doctors may also use these tests to plan treatment.
Barium swallow. In this test, your child swallows a substance called barium. X-rays are used to see how the barium moves inside of the esophagus. The barium allows the doctor to see whether or not there's an indentation that may be caused by vascular rings.
Upper endoscopy. A long, flexible tube with a camera allows your child's doctor to examine the esophagus. The endoscope is inserted through the mouth into the throat as a tiny camera at the tip of the tube sends images to a video monitor.
Bronchoscopy. A bronchoscopy may be done to evaluate the cause of symptoms and to determine the location and severity of compression against the trachea. In this test, a doctor inserts a small, flexible tube through the mouth or nose into the lungs. A light and a small camera attached to the bronchoscope allow the doctor to look inside the trachea and the lungs' airways.

Treatment

Surgery is usually needed to treat vascular rings that are pressing against the trachea or esophagus and to avoid complications. Surgeons divide the vascular rings to release the vessels pressing against the trachea and esophagus. The procedure is often conducted during open surgery, but in some cases a minimally invasive approach may be used.

Different surgical approaches may be used, depending on the specific malformation.

Children and adults with vascular rings may need lifelong care and regular follow-up appointments with specialists to monitor their conditions. Multiple specialists, including congenital cardiologists, doctors trained in lung conditions (pulmonologists), doctors trained in digestive conditions (gastroenterologists), and doctors trained in ear, nose and throat conditions may be involved in their care.