Conditions

Vascular Rings

  • If this is a medical emergency, call 911.

     

     

    • If you are a provider, fax a New Appointment Request Form (NARF) (PDF) (DOC) to 206-985-3121 or 866-985-3121 (toll-free).
    • No pre-referral work-up is required for most conditions. If you have already done testing such as an EKG, Holter monitor or echocardiogram, please fax this information as well as relevant clinic notes and the NARF to 206-985-3121 or 866-985-3121 (toll-free).
    • Your patient will be seen as quickly as possible by the provider who is the best match for managing the current problem.
    • View our complete Heart Center referral information.

What are vascular rings?

Vascular rings are abnormal arteries around the trachea and esophagus. The arteries can press on the trachea and esophagus, causing problems with breathing and feeding.

Before birth, it is normal for babies to have arches of tissue near their trachea and esophagus. As your baby develops, these arches should either turn into normal arteries or disappear. This leaves a single arch called the aortic arch (a left bend in the aorta, just above the heart). Rarely, some of the arches of tissue do not turn into 1 aortic arch or disappear. Instead, they turn into vascular rings.

Sometimes, the rings wrap all the way around the trachea and esophagus. Sometimes, they wrap partway around. Either way, the rings can cause pressure and symptoms.

  • There are several types of rings, named for where and how they formed. Your child’s doctor can explain the type your child has.

    These are the most common types: 

    • Double aortic arch. In this type, the aorta divides into 2 arches. One arch goes around the left side of the trachea and esophagus, and the other goes around the right side.
    • Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. In this type, the aorta bends toward the right instead of the left as it leaves the heart. A ligament connects the pulmonary artery to an artery that branches off the aorta. This traps the trachea and esophagus inside.

Vascular Rings at Seattle Children’s

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    • If your developing baby is diagnosed with vascular rings before birth, our Prenatal Diagnosis and Treatment team works closely with you and your family to plan and prepare for the care your baby will need.
    • Your child’s treatment plan is custom-made. If they have no symptoms, they likely do not need surgery, but it is still important for them to see a Heart Center doctor on a regular schedule to check their health. If they do have symptoms, we plan and perform surgery based on the details of their condition. We closely check your child’s needs to make sure they get the care that is right for them at every age.
    • We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.
    • We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
    • Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
    • Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
    • Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make the journey as smooth as possible.
    • Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
    • Read more about the supportive care we offer.

Symptoms of Vascular Rings

Most babies with vascular rings have symptoms because the rings put pressure on their trachea and esophagus. Some children have no symptoms as babies. They may develop symptoms as they get older.

Pressure on the trachea can cause breathing problems, such as these: 

  • Loud breathing (stridor)
  • Working hard to breathe
  • Wheezing or high-pitched cough
  • Infections in the lungs (respiratory infections) or repeated pneumonia 

Breathing problems may get worse for babies when they try to feed or for older children when they eat.

Pressure on the esophagus can cause feeding problems, such as these: 

Diagnosing Vascular Rings

Sometimes doctors can diagnose this condition in a developing baby before birth. They might first see something different about the arteries near your baby’s heart on standard prenatal ultrasound. To learn more about your baby’s condition, you might have a fetal echocardiogram (an ultrasound done to check the heart more closely). Seattle Children’s Prenatal Diagnosis and Treatment team can care for you when you are pregnant if your developing baby has a known or suspected problem.

To diagnose this condition after birth, your child’s doctor will examine your child and use a stethoscope to listen to their heart.

The doctor will ask for details about any symptoms your child has, their health history and your family health history.

Your child will need tests that provide more information. These may include: 

  • Chest X-ray
  • A CT (computed tomography) scan or MRI (magnetic resonance imaging) of the heart and blood vessels
  • Echocardiography
  • Angiography
  • Bronchoscopy
  • X-ray  of the esophagus

Treating Vascular Rings

Some children with vascular rings need treatment, and some do not.

Children who have symptoms need surgery. During surgery, your child’s surgeon cuts the rings so they do not press on the trachea or esophagus. Usually, surgeons do this surgery through a single small cut (incision) in the chest.

Our heart team works closely with Seattle Children’s general pediatric surgeons to treat vascular rings with as little surgery as possible. We developed a team that can treat some children with vascular rings using thoracoscopic surgery. This means using a small camera and microsurgical tools placed into the chest through tiny holes. This may reduce the recovery time after surgery.

Your child may need medicine to help with their symptoms until they can have surgery.

If your child has vascular rings but no symptoms, your child’s doctor will want to check your child on a regular basis to see if any symptoms start to develop.

Contact Us

Contact the Heart Center at 206-987-2515 for an appointment, second opinion or more information.

Related Links

Paying for Care

Learn about paying for care at Seattle Children’s, including coverage, billing and financial assistance.

For Healthcare Professionals

  • If this is a medical emergency, call 911.

     

     

    • If you are a provider, fax a New Appointment Request Form (NARF) (PDF) (DOC) to 206-985-3121 or 866-985-3121 (toll-free).
    • No pre-referral work-up is required for most conditions. If you have already done testing such as an EKG, Holter monitor or echocardiogram, please fax this information as well as relevant clinic notes and the NARF to 206-985-3121 or 866-985-3121 (toll-free).
    • Your patient will be seen as quickly as possible by the provider who is the best match for managing the current problem.
    • View our complete Heart Center referral information.