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    Eisenmenger Syndrome

    Important
    It is possible that the main title of the report Eisenmenger 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

    • Eisenmenger Complex
    • Eisenmenger Disease
    • Eisenmenger Reaction
    • Eisenmenger Physiology

    Disorder Subdivisions

    • None

    General Discussion

    Eisenmenger syndrome is a rare progressive heart condition that develops in some individuals with structural malformations of the heart that are present from birth (congenital heart defects). The disorder is characterized by increased blood pressure in the main blood vessel (pulmonary artery) connecting the heart to the lungs (pulmonary hypertension) and improper blood flow within the heart.

    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.

    The most common congenital heart defect associated with Eisenmenger syndrome is a ventricular septal defect (VSD) or a "hole in the heart" between the two lower chambers of the heart (left ventricle and right ventricle. This defect allows blood to flow from the left ventricle into the right ventricle (left-to-right shunt). The shunt causes increased blood flow into the lungs eventually resulting in pulmonary hypertension, which causes progressive damage to the small blood vessels in lungs (pulmonary vascular disease). As the damage continues, pulmonary hypertension increases and the small blood vessels become thickened or blocked hampering the flow of blood. Ultimately, blood flow is reversed back through the shunt so that blood flows from the right ventricle into the left ventricle (right-to-left shunt) bypassing the lungs completely. A variety of symptoms including life-threatening complications may occur.

    Eisenmenger syndrome specifically refers to the combination of pulmonary hypertension and right-to-left shunting of the blood within the heart.

    Resources

    March of Dimes Birth Defects Foundation
    1275 Mamaroneck Avenue
    White Plains, NY 10605
    Tel: (914)997-4488
    Fax: (914)997-4763
    Tel: (888)663-4637
    Email: Askus@marchofdimes.com
    Internet: http://www.marchofdimes.com

    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

    National Transplant Assistance Fund
    150 N. Radnor Chester Road
    Suite F-120
    Radnor, PA 19087
    USA
    Tel: (610)353-9684
    Fax: (610)535-6106
    Tel: (800)642-8399
    Email: NTAF@transplantfund.org
    Internet: http://www.transplantfund.org

    National Foundation For Transplants
    5350 Poplar Ave
    Suite 430
    Memphis, TN 38119
    USA
    Tel: (901)684-1697
    Fax: (901)684-1128
    Tel: (800)489-3863
    Email: info@transplants.org
    Internet: http://www.transplants.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/

    American Organ Transplant Association
    21175 Tomball Parkway
    194
    Houston, TX 77070
    Tel: (713)344-2402
    Fax: (713)344-9422
    Email: aotaonline@gmail.com
    Internet: http://www.aotaonline.org

    Second Wind Lung Transplant Association, Inc.
    P.O. Box 1657
    Wimberley, TX 78676-1657
    USA
    Tel: (512)847-9303
    Tel: (888)855-9463
    Email: barlows9303@gmail.com
    Internet: http://www.2ndwind.org

    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

    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/

    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: 4/5/2008
    Copyright 1988, 1989, 2001, 2002, 2003, 2006 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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