We have changed our visitation policy for the safety of our patients and staff. Click here for the updated visitation policy and click here for information about COVID-19.

Lahey Health is now part of Beth Israel Lahey Health

by Kassir K


An interrupted aortic arch (IAA) is a rare heart defect. The aortic arch is part of the major blood vessel that helps move blood from the heart to the rest of the body. With IAA, the aortic arch is interrupted or incomplete. Blood cannot flow the way it should. Children with IAA may also have a hole in the wall between the right and left chambers in the heart.

Heart Chambers and Valves
heart anatomy
Copyright © Nucleus Medical Media, Inc.
Blood Flow Through the Heart
Copyright © Nucleus Medical Media, Inc.


IAA is present at birth. A direct cause isn’t known. It happens in the fifth to seventh week of fetal growth.

Risk Factors

There is an higher risk for IAA if your child also has DiGeorge syndrome . This is a problem with the chromosomes.


Symptoms often appear within the first day or two after birth. Many times, the baby will show symptoms soon after birth. Tell your doctor if you notice:

  • Lack of energy
  • Poor feeding
  • Rapid breathing
  • Pale, blue, or cool skin
  • Decreased urine output

IAA can lead to shock and heart failure . Your child will need emergency care.

During the exam, the doctor may detect:

  • Fast heart rate
  • Weak pulse
  • Low oxygen levels

These symptoms may be due to other health problems.


You will be asked about your child's symptoms and health history. A physical exam will be done.

Pictures may be taken of your child's chest. This can be done with:

Your child's heart activity may be measured. This can be done with electrocardiogram (EKG).


Talk with the doctor about the best plan for your child. Your child may have:


Certain medicines will often be given to keep some blood flowing through another blood vessel called the ductus arteriosus. This lets some blood get around the interruption in the aorta. This is a short term treatment.

Medications may also:

  • Help the heart beat stronger
  • Remove extra fluid in the body


Surgery is needed to fix IAA. Surgery aims to form a connection between the two parts of the aortic arch. The hole in the heart between the ventricles is also closed. The ductus arteriosus is then closed.

Lifelong Monitoring

Your child will need to see a heart specialist regularly.


IAA can’t be prevented.


American Heart Association  http://www.heart.org 

Healthy Children—American Academy of Pediatrics  http://www.healthychildren.org 


Canadian Cardiovascular Society  http://www.ccs.ca 

Heart and Stroke Foundation of Canada  http://www.heartandstroke.com 


Evaluation of the infant for congenital heart disease (CHD). EBSCO DynaMed Plus website. Available at:  https://www.dynamed.com/topics/dmp~AN~T566082/Evaluation-of-the-infant-for-congenital-heart-disease-CHD  . Updated April 30, 2018. Accessed June 29, 2018.

Interrupted aortic arch. Cincinnati Children’s Hospital Medical Center website. Available at: http://www.cincinnatichildrens.org/health/i/iaa. Updated July 2015. Accessed June 29, 2018.

Revision Information