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Double Aortic Arch—Child by Deanna M. Neff, MPH

Definition

Double aortic arch is a type of vascular ring heart defect. In a normal heart, the blood flows in from the body to the right atrium, goes into the right ventricle, and then goes to the lungs to pick up fresh oxygen. The blood returns to the left atrium, goes into the left ventricle, and goes out to the rest of the body through a very large artery called the aorta.

With a double aortic arch, the aorta branches into right and left tubes, instead of just being one large tube. The two tubes can circle around and compress the airways and/or esophagus.

While this condition may be detected in infancy, it is often found later.

Heart Chambers and Valves

Blood Flow Through the Heart

Causes

Double aortic arch is a congenital defect. This means that the baby is born with the condition. It is not known exactly why the heart develops abnormally in some babies.

Risk Factors

Risk factors for developing double aortic arch are not well known.

Symptoms

Symptoms may include:

Trouble breathing (wheezing and cough)
Lung infections
Poor feeding (eg, vomiting, choking)
Trouble swallowing
Acid reflux

These symptoms may be caused by other conditions. If your child has any of these, talk to the doctor right away.

Diagnosis

Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:

Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
CT scan —a type of x-ray that uses a computer to make pictures of structures inside the chest
MRI scan —a test that uses magnetic waves to make pictures of structures inside the body
Chest x-ray —an imaging test that uses low amounts of radiation to create an image of the chest
Electrocardiogram —a test that measures the electrical activity of the heart
Upper gastrointestinal tests —a series of x-rays of the esophagus, stomach, and duodenum (the first part of the small intestine)
Bronchoscopy —a visual exam of the air passages leading into the lungs

Treatment

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

Surgery

If your child is having symptoms like difficulty breathing, surgery will be done. The goal of surgery is to tie off and close one of these extra branches. Once this is done, symptoms may improve right away or gradually over time.

Lifelong Monitoring

Your child will have regular exams from a heart specialist.

Prevention

There is no known way to prevent double aortic arch. Getting appropriate prenatal care is always important.

RESOURCES:

American Academy of Pediatrics http://www.aap.org/
American Heart Association http://www.heart.org/HEARTORG/

CANADIAN RESOURCES:

Canadian Cardiovascular Society http://www.ccs.ca/
Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

References:

Children’s Hospital Boston. Vascular ring. Children’s Hospital Boston website. Available at: http://www.childrenshospital.org/az/Site1942/mainpageS1942P0.html . Accessed July 12, 2010.
Johns Hopkins University, Cove Point Foundation. Double aortic arch. Johns Hopkins University, Cove Point Foundation website. Available at: http://www.pted.org/?id=doubleaortic1 . Updated April 3, 2009. Accessed July 23, 2010.
Mayo Clinic. Atrioventricular canal defect. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/atrioventricular-canal-defect/DS00745/DSECTION=risk-factors . Updated July 8, 2010. Accessed July 7, 2010.

Last reviewed September 2012 by Michael Woods, MD

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