Interrupted Aortic Arch—Child

  • 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
    heart anatomy
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    Blood Flow Through the Heart
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  • Causes

    A direct cause is not known. The defect develops in the fifth to seventh week of fetal growth. The child is born with the condition.
  • Risk Factors

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

    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.
  • Treatment

    Talk with the doctor about the best treatment plan for your child. Treatment options include:
    Medicines, like Prostaglandin E1, 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.
    Other medicines may be given to:
    • Help the heart beat stronger
    • Get rid of extra fluid in the body
    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

    There is no way to prevent this condition. Getting appropriate prenatal care is always important.
  • RESOURCES - American Academy of Family Physicians

    American Heart Association


    Canadian Cardiovascular Society

    Heart and Stroke Foundation of Canada


    Interrupted aortic arch. Cincinnati Children’s Hospital Medical Center website. Available at: Updated November 2012. Accessed July 17, 2013.

    Interrupted aortic arch. Johns Hopkins University, Cove Point Foundation website. Available at: Updated May 16, 2011. Accessed July 17, 2013.

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