Interrupted Aortic Arch—Child

Definition

Definition

An interrupted aortic arch 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 this defect, the aortic arch is interrupted or incomplete. Blood cannot flow through it normally. This makes blood flow to the body less efficient. Children with this defect may also have a hole in the wall between the right and left chambers in the heart.

Heart Chambers and Valves

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Blood Flow Through the Heart

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Causes

Causes

An interrupted aortic arch is present at birth. A direct cause is not known. The defect develops in the fifth to seventh week of fetal growth.

Risk Factors

Risk Factors

There is an increased risk for this condition if your child also has DiGeorge syndrome. This is a chromosomal abnormality.

Symptoms

Symptoms

Symptoms typically 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 the following in your infant or child:

  • Weakness
  • Poor feeding
  • Rapid breathing
  • Pale, blue, or cool skin
  • Decreased urine output

This condition 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 conditions.

Diagnosis

Diagnosis

The doctor will ask about your child’s symptoms and medical history. A physical exam will be done.

Images 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

Treatment

Talk with the doctor about the best treatment plan for your child. Treatment options include:

Medications

Certain medications will usually

be given to keep some blood flowing through another blood vessel called the ductus arteriosus. This allows some blood to get around the interruption in the aorta. This is a temporary treatment.

Medications may also:

  • Help the heart beat stronger
  • Get rid of extra fluid in the body

Surgery

Surgery is needed to correct the defect. 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.

Prevention

Prevention

There are no current guidelines to prevent interrupted aortic arch because the cause is unknown. Getting appropriate prenatal care is always important.

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.ca

References:

Interrupted aortic arch. Cincinnati Children’s Hospital Medical Center website. Available at:
http://www.cincinnatichildrens.org/health/i/iaa
Updated November 2012. Accessed July 17, 2013.

Interrupted aortic arch. Johns Hopkins Children’s Center website. Available at:
http://www.hopkinschildrens.org/Interrupted-Aortic-Arch.aspx
Updated May 16, 2011. Accessed July 17, 2013.

Last reviewed June 2015 by Michael Woods, MD
Last Updated: 5/11/2013

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