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Interrupted Aortic Arch—Child

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    TOP

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

Risk Factors    TOP

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

Symptoms    TOP

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.

Diagnosis    TOP

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 withelectrocardiogram (EKG).

Treatment    TOP

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

Medications

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

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    TOP

Your child will need to see a heart specialist regularly.

Prevention    TOP

IAA can’t be prevented.

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 Plus website. Available at: http://www.dynamed.... Updated April 30, 2018. Accessed June 29, 2018.
Interrupted aortic arch. Cincinnati Children’s Hospital Medical Center website. Available at:
...(Click grey area to select URL)
Updated July 2015. Accessed June 29, 2018.
Last reviewed June 2018 by Kari Kassir, MD
Last Updated: 6/29/2018

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This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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