Before you begin reading about vascular rings in children, please read the explanation of how the normal heart works for a basic understanding of the heart's structure and function.
A vascular ring occurs when the aorta (the large artery that carries oxygenated blood out of the heart) or its branches form a complete ring around the trachea (the tube that carries air to the lungs) and the esophagus (the tube that carries food to the stomach). (All of these structures are very close to one another in this crowded area of the chest.) This happens when certain parts of the aorta, which normally disappear during fetal development, persist abnormally.
“Vascular” means relating to blood vessels (veins and arteries). The condition is called “vascular ring” because the aorta — or other, related arteries — encircles the trachea and the esophagus.
Normally, the aorta starts at the left ventricle of the heart as one large vessel: it arches up (the aortic arch) to the left of the trachea and then down (the descending aorta). Arteries that deliver blood to the head, arms and other parts of the upper body branch off at the top of the arch. Arteries that deliver blood to the abdomen, legs and other parts of the lower body branch off from the descending aorta.
There are different types of vascular rings. The most common are:
The structure of the vascular ring and its relationship to the trachea and esophagus varies from child to child. The Cardiac Center team will explain your child’s condition in detail.
The following symptoms may be present in children with vascular rings:
In most cases, the symptoms of a vascular ring are mild and the condition can go undiagnosed until a child is older, or even an adult. If the condition is severe, and the child has trouble breathing, a vascular ring may be diagnosed at several weeks or months of age.
Diagnosis of a vascular ring may require some or all of these tests:
Vascular rings require surgery (not open-heart) if there are symptoms. In most cases, the surgeon makes an incision on the left side of the chest and goes through the ribs to access the area.
The Cardiac Center team will explain your child’s surgery in more detail.
In some cases, the patient has no symptoms of vascular ring and the condition is discovered during an unrelated medical test, procedure or evaluation. In some of these cases, instead of surgery, a Cardiac Center team will monitor the child to make sure symptoms don’t appear and the condition isn’t harming the child’s health.
The procedure to correct vascular ring is considered low-risk, so in most cases, surgery is recommended.
In most cases, children with vascular rings recover completely after surgery and won’t need additional procedures. It is not uncommon for noisy breathing to continue for several weeks or months after surgery as the trachea slowly takes on a more normal shape. Rarely, symptoms such as coughing or labored breathing continue after surgery. We will monitor your child to make sure recovery is as complete and rapid as possible.
Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physician. Patients will need to carefully follow doctors’ advice. In rare cases, we will suggest your child limits certain types of exercise.
Children born with vascular rings who do not require surgery should follow up with a cardiologist. We will help patients transition care to an adult cardiologist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children’s Hospital of Philadelphia and Penn Medicine, meets the unique needs of adults who were born with heart defects.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Paul M. Weinberg, MD
Date: July 2009
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