Interrupted Aortic Arch—Child
by Cynthia M. Johnson, MA
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.
IAA is present at birth. The cause is not known. It happens in the fifth to seventh week of fetal growth.
The risk of this problem is higher in children who have DiGeorge syndrome. This is a problem with the chromosomes.
Symptoms often appear within the first day or two after birth. Problems may be:
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).
The goal of treatment is to get blood to flow as it should.
Medicines may be used to:
Surgery will also be done to form a connection between the 2 parts of the aortic arch. Any hole in the heart between the ventricles will also be closed. The ductus arteriosus is then closed.
Lifelong monitoring by a doctor who treats the heart will also be needed.
There are no known guidelines to prevent IAA.
American Heart Association
Healthy Children—American Academy of Pediatrics
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
Evaluation of the infant for congenital heart disease (CHD). EBSCO DynaMed website. Available at: https://www.dynamed.com/evaluation/evaluation-of-the-infant-for-congenital-heart-disease-chd. Accessed November 5, 2020.
Interrupted aortic arch. Cincinnati Children’s Hospital Medical Center website. Available at:
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Accessed November 5, 2020.
Silberbach M, Hannon D. Presentation of congenital heart disease in the neonate and young infant. Pediatr Rev. 2007 Apr;28(4):123-131.
Last reviewed September 2020 by EBSCO Medical Review Board Kari Kassir, MD
Last Updated: 5/11/2021