Conditions

Interrupted Aortic Arch

  • 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 is interrupted aortic arch?

Interrupted aortic arch is a birth defect in which a small section of the aorta is missing.

The aorta carries oxygen-rich (red) blood from the left ventricle of the heart to all parts of the body. Normally, the aorta has a curved shape. Just above the heart, small arteries branch off the aorta and go to the head and arms. Then the aorta curves down toward the belly, lower body and legs. The part that curves down is called the descending aorta.

Heart Illustration.jpg

This is a normal heart. The aorta leaves the heart near the top and then curves down toward the lower body. In interrupted aortic arch, the first part of this curve, or arch, is not connected to the rest.

In interrupted aortic arch, the first part of the aorta (which supplies blood to the head and arms) is not connected to the rest (which supplies blood to the lower body and legs).

This means oxygen-rich blood from the heart cannot reach the lower body and legs in the normal way. Instead, the descending aorta connects to the pulmonary artery by a blood vessel called the ductus arteriosus.

Most babies with interrupted aortic arch also have a hole in the wall between the right and left ventricles called a ventricular septal defect (VSD).

  • It is normal for babies to have a ductus arteriosus. Before a baby is born, this vessel shifts blood away from their lungs into their aorta. This works fine because babies do not breathe on their own before birth, so they do not need blood to get to their lungs to pick up oxygen.

    Normally, the ductus arteriosus closes soon after birth. In babies with a normal aorta, this closure does not cause problems. But in babies with interrupted aortic arch, the closure means blood cannot get to the descending aorta.

    Before the ductus arteriosus closes, babies born with this defect do get blood to their lower body. But it is not oxygen-rich blood coming from their left ventricle. For most babies, it is a mix of oxygen-rich and oxygen-poor blood.

    This happens because most babies with interrupted aortic arch also have a ventricular septal defect. This hole allows some oxygen-rich blood to flow from the left ventricle to the right ventricle and then to the ductus arteriosus.

Interrupted Aortic Arch at Seattle Children’s

    • Our team of more than 40 pediatric cardiologists has diagnosed and treated this condition in many babies — and we provide any ongoing care patients need as they grow into young adults.
    • Our pediatric cardiac surgeons perform more than 500 procedures yearly. Each has experience correcting interrupted aortic arch and related heart conditions in newborns and babies.
    • We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery.
  • Consistently ranked one of the nation's best cardiology and cardiac surgery programs by U.S. News and World Report.

    Our surgical outcomes are among the best in the nation year after year, including outcomes for VSDs.

    • If your developing baby is diagnosed with interrupted aortic arch 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. We plan and carry out their surgery based on the specific details of their heart defect, like if they have VSD along with interrupted aortic arch. 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.
    • At Seattle Children’s, we set goals to reduce the number of procedures your child is likely to need for interrupted aortic arch.
    • After surgery for this condition, some patients develop a narrow spot where their aorta was repaired. This puts extra stress on their heart. It may mean they need a cardiac catheterization procedure or another surgery to open the aorta again.
    • By carefully studying the steps in our surgical approach and the results for our patients, we designed a standard way to repair the aorta with lower risk of getting a narrow spot in the future.
    • 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 Interrupted Aortic Arch

Your baby will likely not have symptoms at birth. When their ductus arteriosus starts to close, within the first week of life, they will start to have symptoms because their blood is not flowing the way it should.

They may have symptoms like these: 

  • Weakness
  • Trouble feeding
  • Getting tired easily
  • Working hard to breathe
  • Fast heartbeat
  • Blue or purple-tinged skin, mouth, lips or nail beds (cyanosis)

Diagnosing Interrupted Aortic Arch

Sometimes doctors can diagnose this condition in a developing baby before birth. They might first see something different about your baby’s aorta 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 baby’s doctor will examine your baby and ask for details about any symptoms your baby has, their health history and your family health history.

Your child will need tests that provide more information about how their heart and nearby blood vessels look and work. These include: 

They may also need cardiac catheterization.

Treating Interrupted Aortic Arch

Babies with interrupted aortic arch need surgery during the first week of life.

Before surgery takes place, your baby will need medicine (prostaglandin) that keeps the ductus arteriosus open so blood can get to the rest of their body.

Surgeons will connect the 2 separate parts of the aorta, close your baby’s ventricular septal defect (VSD) if needed and close the patent (open) ductus arteriosus. Often, surgeons can do all these steps during the same operation. However, your baby may need more than 1 surgery based on their condition, such as the size and position of their VSD and how much blood flows out of their left ventricle.

After your child recovers from surgery, it will be important for them to keep seeing their cardiologist on a regular schedule to check their heart health. People with a repaired aorta sometimes develop a narrow spot where the repair was done or below the valve that leads out of their left ventricle (subaortic stenosis). With regular visits, your child’s team can check for early signs of conditions like these and make sure your child gets the care they need.

Contact Us 

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

Providers, see how to refer a patient.

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.