When you hear that your child was born with a vascular ring, you may be concerned. A vascular ring is a birth defect where the blood vessels in the chest can press on and narrow your child’s feeding tube or windpipe.

At Mount Sinai Kravis Children’s Heart Center our pediatric cardiologists are highly trained, extremely skilled, and very experienced. We diagnose and treat all types of heart conditions in children of all ages. This includes all types of vascular rings.

About Vascular Rings

The healthy normal aorta—the main blood vessel that supplies the body with blood—leads from the heart, arching up towards the head, continuing to arch to the left of the windpipe (trachea) and feeding tube (esophagus) before turning down to lead towards the lower half of the body.

However, when the aorta does not form normally, the blood vessels in your child’s chest can form a ring around the trachea and esophagus. This condition is known as a vascular ring. The rings that press against the trachea can cause your child breathing problems. The rings that push on the esophagus can make it difficult for your child to swallow. Both affect your child’s health.

There are various ways blood vessels form vascular rings: a double aortic arch, a right aortic arch with an aberrant left subclavian artery, and a pulmonary artery sling.

• Double aortic arch: With this condition, the aorta divides abnormally into two parts. One part arches to the left, and the other part to the right. Together, they form a ring around the trachea and esophagus.
• Right aortic arch with an aberrant left ligamentum arteriosum: In this condition, the aortic arch goes to the right instead of the left as it should. The blood vessel that goes to the left (left subclavian artery), comes from behind the esophagus and trachea. Sometimes this condition also means that a certain blood vessel (ductus arteriosus) that normally closes after birth, passes to the left of the esophagus and trachea.
• Pulmonary artery sling: This condition happens when the artery that goes to the left lung (left pulmonary artery) forms too far down on the main artery that carries blood from the heart to the lungs (main pulmonary artery).  The left pulmonary artery then passes between the esophagus and trachea, putting pressure on them and blocking blood flow to the left lung. Children with this condition may also have complete tracheal rings, an abnormality that causes the trachea to become narrow.

Symptoms

If your child has a vascular ring, the symptoms may include:

• Difficulty swallowing
• Labored or noisy breathing, especially when excited or active
• Persistent cough
• Repeated pneumonias or respiratory infections

Some children may have no symptoms at all. We might make the diagnosis incidentally, as part of an imaging evaluation for another issue. If your child has no symptoms, we may not need to treat the condition.

Diagnosis

To diagnose a vascular ring, we may use some these tests.

• Bronchoscopy involves inserting a narrow tube in the trachea to see if the airways are affected
• Cardiac magnetic resonance imaging (MRI) uses safe, powerful magnets and radio waves to create pictures of the heart
• Chest computed tomography (CT) scan uses X-rays to create detailed images of the blood vessels and airway
• Chest X-ray takes pictures of the heart, lungs, and blood vessels, and can show if the heart is enlarged
• Echocardiogram is non-invasive ultrasound that uses sound waves to take an image the heart
• Other gastrointestinal tests allow us to see if the swallowing tube (esophagus) is affected

Treatments We Offer

If your child is having symptoms, or if we are worried about narrowing of the airway, we may need to perform surgery. If your child has no symptoms, we may not need to, though we may continue to monitor them. Often, when performing surgery, we can use a minimally invasive procedure which means fewer complications and quicker recovery for your little one.

Treatment depends on the type of vascular ring. The most common types of conditions and treatments are:

• Double aortic arches require closing and dividing one of the aortic arches, usually the left. This relieves pressure of the aorta on the trachea and esophagus.
• Right aortic arches with left ligamentum arteriosum necessitate cutting a piece of the ligament (ligamentum arteriosum) to keep it from pressing on the trachea and esophagus.
• Pulmonary artery slings require repositioning the left pulmonary artery to its proper spot on the main pulmonary artery. If there are abnormalities of the trachea, these may need to be addressed as well.

Your child’s cardiologist and surgeon will explain your child’s specific situation and the treatment needed.

We monitor your child after the procedure to make sure recovery is complete and quick. Most children don’t need additional procedures after surgical treatment.

Why Choose Children’s Heart Center?

Children’s Heart Center’s pediatric cardiologists use an integrated team approach and provide compassionate care to all our patients and support to their families. Our surgeons, physicians, and other staff highly experienced in diagnosing, treating, monitoring, and providing follow-up care for all types of heart conditions. At Children’s Heart Center, your child will have a team of specialists dedicated to your child’s wellbeing. Thanks to the Mount Sinai Health System network of specialists, we can easily consult with experts in pulmonology, gastroenterology or radiology if necessary.

We get to know you and your child. Be assured that your little one is in good hands with us.