• Hypoplastic Left Heart Syndrome

    (HLHS)

    Definition

    Hypoplastic left heart syndrome is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood then moves out to the rest of the body.
    With this syndrome, structures on the left side of the heart, which includes the aorta, aortic valve, left ventricle, and mitral valve, may be:
    • Too small
    • Absent
    • Abnormally developed
    Since the heart cannot function properly, oxygen-rich blood flow to the body is limited. This condition requires immediate care from a doctor.
    Heart Chambers and Valves
    heart anatomy
    Copyright © Nucleus Medical Media, Inc.
    Blood Flow Through the Heart
    IMAGE
    Copyright © Nucleus Medical Media, Inc.

    Causes

    Hypoplastic left heart syndrome is present at birth. It is not known exactly why the heart does not develop normally.

    Risk Factors

    Factors that may increase your chance of having a child with hypoplastic left heart syndrome include:
    • Previous pregnancy with fetal heart abnormalities or fetal loss
    • Family history of congenital heart defects

    Symptoms

    Symptoms usually appear within days after birth. Tell the doctor if you notice the following in your infant or child:
    • Blue/gray skin color
    • Cool skin
    • Rapid or difficult breathing
    • High heart rate
    • Sweaty, clammy skin
    • Poor feeding

    Diagnosis

    You will be asked 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:
    Electrocardiogram (EKG) can monitor the heart's electrical activity.

    Treatment

    Talk with your doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:

    Medications

    Medications are necessary to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and the aorta, which is the largest artery in the body. It usually closes within a few days after birth. Keeping this passage open is a temporary treatment. Other medications may be used as well.

    Surgery

    Surgery may be done to improve blood flow. This can be done through a variety of reconstructive and shunting procedures. Surgeries are usually done in stages:
    • After birth
    • 4-6 months of age
    • 2-4 years of age

    Lifelong Monitoring

    Your child will need to see a heart specialist regularly. Heart medication will be needed throughout your child's life.

    Prevention

    There are no current guidelines to prevent hypoplastic left heart syndrome because the cause is unknown. Getting proper prenatal care is always important.

    RESOURCES

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

    Family Doctor—American Academy of Family Physicians http://familydoctor.org

    CANADIAN RESOURCES

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

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

    References

    Hypoplastic left heart syndrome. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T114438/Hypoplastic-left-heart-syndrome. Updated January 25, 2016. Accessed June 6, 2016.

    Hypoplastic left heart syndrome. Johns Hopkins Children's Center website. Available at: http://www.hopkinschildrens.org/Hypoplastic-Left-Heart-Syndrome.aspx. Updated May 16, 2011. Accessed June 6, 2016.

    Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects%5FUCM%5F307037%5FArticle.jsp. Updated October 21, 2015. Accessed June 6, 2016.

    Revision Information

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