Definition

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

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Blood Flow Through the Heart

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Causes

IAA is present at birth. The cause is not known. It happens in the fifth to seventh week of fetal growth.

Risk Factors

The risk of this problem is higher in children who have DiGeorge syndrome. This is a problem with the chromosomes.

Symptoms

Symptoms often appear within the first day or two after birth. Problems may be:

  • Lack of energy
  • Poor feeding
  • Rapid breathing
  • Pale, blue, or cool skin
  • Few wet diapers

Diagnosis

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).

Treatment

The goal of treatment is to get blood to flow as it should.

Medicines may be used to:

  • Keep some blood flowing through another blood vessel called the ductus arteriosus
  • Help the heart beat stronger
  • Remove extra fluid in the body

Surgery will also be done to form a connection between the two 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.

Prevention

There are no known guidelines to prevent IAA.

RESOURCES:

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

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

CANADIAN RESOURCES:

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

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

REFERENCES:

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: http://www.cincinnatichildrens.org/health/i/iaa. 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: 11/5/2020