Vascular rings

Overview

A vascular ring is a heart problem present at birth. That means it's a congenital heart defect. In this condition, part of the body's main artery or its branches form a ring around the windpipe, the food swallowing tube or both.

• The body's main artery is called the aorta. The condition affects the part of the aorta called the aortic arch.
• The food swallowing tube leads from the mouth to the stomach. It's called the esophagus.
• The windpipe is also called the trachea.

A vascular ring may be complete or incomplete.

• A complete vascular ring forms a ring around both the esophagus and trachea.
• An incomplete vascular ring doesn't go all the way around the esophagus or trachea.

Surgery is usually needed to treat a vascular ring.

Symptoms

Some people with a vascular ring don't have symptoms. Sometimes symptoms aren't noticed until later in life. If a vascular ring presses against the trachea and esophagus, it can lead to breathing and digestive problems.

Symptoms of a vascular ring may include:

• Frequent respiratory infections.
• Wheezing.
• Coughing.
• Trouble swallowing.
• Difficulty feeding.
• Vomiting.

Some people born with a vascular ring also may have other heart problems at birth. Specific symptoms depend on the type of heart problems present.

Diagnosis

A health care provider does a physical exam and asks questions about the symptoms. Tests done to diagnose a vascular ring may include:

• Imaging tests. A chest X-ray can show changes in the windpipe that might suggest a vascular ring. The test also can show which side of the body the aortic arch is on.

  Other imaging tests may include an echocardiogram, a CT angiogram or an MRI scan. Health care providers also may use these tests to plan treatment.

• Barium swallow. This test involves swallowing a substance called barium. X-rays are taken to see how the substance moves from the mouth to the stomach. The test can show changes in the structure of the esophagus that may be caused by vascular rings.
• Upper endoscopy. A long, flexible tube with a camera is used to examine the esophagus. The device is called an endoscope. The health care provider inserts it through the mouth and into the throat. A tiny camera at the tip sends images to a video monitor.
• Bronchoscopy. In this test, a health care provider inserts a small, flexible tube through the mouth or nose into the lungs. A light and a small camera attached to the tube allow the provider to see inside the windpipe and the lungs' airways. This test can show if a vascular ring is pushing against the trachea.

Treatment

Surgery is usually done to treat a vascular ring that presses against the trachea or esophagus. Surgery also helps prevent complications.

During surgery, the health care provider splits the vascular ring to stop the blood vessel from pressing against the windpipe and food pipe. The surgery may be done as open-heart surgery or as a minimally invasive approach.

The specific type of surgery depends on the type of heart problems present.

People born with a vascular ring need regular health checkups for life to prevent complications.