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    Hypoplastic Left Heart Syndrome

    Important
    It is possible that the main title of the report Hypoplastic Left Heart Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • Aortic and Mitral Atresia with Hypoplasic Left Heart Syndrome
    • HLHS

    Disorder Subdivisions

    • None

    General Discussion

    Hypoplastic left heart syndrome is a term used to describe a group of closely related rare heart defects that are present at birth (congenital). The normal heart has four chambers. The two upper chambers, known as atria, are separated from each other by a fibrous partition known as the atrial septum. The two lower chambers are known as ventricles and are separated from each other by the ventricular septum. Valves connect the atria (left and right) to their respective ventricles. The valves allow for blood to be pumped through the chambers. Blood travels from the right ventricle through the pulmonary artery to the lungs where it receives oxygen. The blood returns to the heart through pulmonary veins and enters the left ventricle. The left ventricle sends the now oxygen-filled blood into the main artery of the body (aorta). The aorta sends the blood throughout the body.

    Hypoplastic left heart syndrome is characterized by the underdevelopment (hypoplasia) of the chambers on the left side of the heart (i.e., left atrium and ventricle). In addition, the mitral valve, which connects these chambers to each other, is usually abnormally narrow (stenosis) or closed (atresia) and the aortic valve, which connects the heart to the major vessels that lead from the lungs (ascending aorta), may also be narrow or closed. Infants with hypoplastic left heart syndrome also have an abnormally narrow ascending aorta.

    Resources

    American Heart Association
    7272 Greenville Avenue
    Dallas, TX 75231
    Tel: (214)784-7212
    Fax: (214)784-1307
    Tel: (800)242-8721
    Email: Review.personal.info@heart.org
    Internet: http://www.heart.org

    NIH/National Heart, Lung and Blood Institute
    P.O. Box 30105
    Bethesda, MD 20892-0105
    Tel: (301)592-8573
    Fax: (301)251-1223
    Email: nhlbiinfo@rover.nhlbi.nih.gov
    Internet: http://www.nhlbi.nih.gov/

    Kids With Heart ~ National Association for Children's Heart Disorders, Inc.
    1578 Careful Dr.
    Green Bay, WI 54304
    Tel: (920)498-0058
    Fax: (920)498-0058
    Tel: (800)538-5390
    Email: michelle@kidswithheart.org
    Internet: http://www.kidswithheart.org

    Little Hearts, Inc.
    P.O. Box 171
    110 Court Street, Suite 3A
    Cromwell, CT 06416
    USA
    Tel: (860)635-0006
    Fax: (860)635-0006
    Tel: (866)435-4673
    Email: info@littlehearts.org
    Internet: http://www.littlehearts.org

    Birth Defect Research for Children, Inc.
    976 Lake Baldwin Lane
    Orlando, FL 32814
    USA
    Tel: (407)895-0802
    Email: staff@birthdefects.org
    Internet: http://www.birthdefects.org

    Congenital Heart Information Network (C.H.I.N.)
    P.O. Box 3397
    Margate City, NJ 08402-0397
    Tel: (609)823-4507
    Fax: (609)822-1574
    Email: mb@tchin.org
    Internet: http://www.tchin.org

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    Madisons Foundation
    PO Box 241956
    Los Angeles, CA 90024
    Tel: (310)264-0826
    Fax: (310)264-4766
    Email: getinfo@madisonsfoundation.org
    Internet: http://www.madisonsfoundation.org

    Fetal Hope Foundation
    9786 South Holland Street
    Littleton, CO 80127
    USA
    Tel: (303)932-0553
    Tel: (877)789-4673
    Email: info@fetalhope.org
    Internet: http://www.fetalhope.org

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 9/17/2007
    Copyright 1986, 1993, 1994, 1996, 2003, 2007 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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