Vascular Ring

A vascular ring happens when the aorta or its branches form a complete ring around the trachea (windpipe) and the esophagus, the tube that leads from the throat to the stomach.

A vascular ring is a birth defect that can cause issues with breathing and eating. Children with a vascular ring may have symptoms as a baby or during early childhood.

It’s normal for a fetus in the womb to have some arches of tissue near the trachea and esophagus. As the baby develops, these arches should either change to become arteries or completely disappear. Rarely, some of these arches do not disappear and turn into vascular rings.

There are several types of rings, named for where and how they formed. These are the most common types:

  • Double aortic arch: The aorta divides into two arches, with one going around the left side of the trachea and esophagus and one going around the right side.
  • Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum: In this type of vascular ring, the aorta bends toward the right instead of the left as it leaves the heart. A ligament (the ligamentum arteriosum) connects the pulmonary artery and one of the arteries that branch off the aorta. This traps the trachea and esophagus inside the ring.

The specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine — the leading providers of pediatric heart care in Louisville and Southern Indiana — can help your child with a vascular ring.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a precise diagnosis and determine what treatment, if any, is needed.

Norton Children’s has a network of outreach diagnostic and treatment services throughout Kentucky and Southern Indiana.

Vascular Ring Symptoms

Most babies with a vascular ring will have symptoms caused by the pressure the rings put on their trachea and esophagus. Some children with a vascular ring have no symptoms as babies, but develop symptoms as they get older.

Pressure on the trachea can cause breathing issues such as:

  • Loud or labored breathing
  • Wheezing or high-pitched cough
  • Infections in the lungs or repeated pneumonia
  • Breathing issues that get worse when a baby tries to feed or when an older child eats

Pressure on the esophagus can cause issues such as:

  • Acid reflux
  • Choking
  • Eating slowly
  • Trouble swallowing
  • Trouble eating solid food
  • Vomiting

A vascular ring can form when the baby’s aorta does not develop the way it should during pregnancy. Doctors don’t know why this happens.

Diagnosing Vascular Ring

A pediatric cardiologist will examine your child, listen to his or her heart, and ask about your child’s symptoms and health history, as well as your family’s health history. The following tests may provide more details:

  • Bronchoscopy: For this test, a camera is guided down the throat so doctors can see the child’s airways.
  • Chest X-ray: This shows pictures of the heart and lungs, and can show heart and lung issues, extra blood flow or fluid in the lungs caused by defects.
  • CT angiography: Uses a computer tomography (CT) scanner to produce detailed images of the blood vessels following a dye injection.
  • Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show the structure of the heart and ductus arteriosus as well as the function of the heart. Norton Children’s Heart Institute has 28 tele-echo locations throughout Kentucky and Southern Indiana.
  • Heart catheterization: This procedure provides direct pressure measurements and angiograms, X-ray images of the heart chambers and vessels, that can show the vascular ring.
  • Upper GI: This procedure takes a video of the upper gastrointestinal (GI) system using dye that is swallowed. It allows doctors to see the shape of the esophagus and an area compressed by the vascular ring.

Vascular Ring Treatment

If your child has vascular rings but doesn’t have symptoms, your doctor will want to see your child regularly to check whether any symptoms develop.

Children who have symptoms from vascular rings will need surgery. The procedure is not open heart surgery but does involve surgery inside the chest. In many cases, the surgeon makes a small incision on the left side of the chest and goes between the ribs to make the repair.

DOUBLE AORTIC ARCH SURGERY

The surgeon will cut and stitch closed one of the arches, usually the left, so the aorta no longer encircles or puts pressure on the trachea or esophagus.

RIGHT AORTIC ARCH WITH LEFT LIGAMENTUM ARTERIOSUM SURGERY

The surgeon divides the ligamentum arteriosum so that it no longer puts pressure on the trachea or esophagus. The right aortic arch remains, as a person can live with this type of defect.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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