Diseases & Conditions


Eisenmenger Syndrome

Synonyms of Eisenmenger Syndrome
  • Eisenmenger Complex
  • Eisenmenger Disease
  • Eisenmenger Physiology
  • Eisenmenger Reaction

Disorder Subdivisions

    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.

    Organizations related to Eisenmenger Syndrome
    • American Heart Association
      National Center
      Dallas TX 75231-4596
      Phone #: 214-373-6300
      800 #: 800-242-8721
      e-mail: [email protected]
      Home page: http://www.americanheart.org
    • American Organ Transplant Association
      PO Box 667566
      Houston TX 77266-7566
      Phone #: 281-996-8799
      800 #: --
      e-mail: N/A
      Home page: http://www.aotaonline.org
    • Children's Heart Association for Support and Education
      c/o The Cardiac Clinic, Division of Cardiology
      Ontario None M5G 1X8
      Phone #: 416-410-2427
      800 #: --
      e-mail: [email protected]
      Home page: http://www.angelfire.com/on/chase/
    • Congenital Heart Anomalies, Support, Education, & Resources
      2112 North Wilkins Road
      Swanton OH 43558
      Phone #: 419-825-5575
      800 #: --
      e-mail: [email protected]
      Home page: http://www.csun.edu/~hcmth011/chaser/chaser-news.html
    • Congenital Heart Information Network
      1561 Clark Dr
      Yardley PA 19067
      Phone #: 215-493-3068
      800 #: --
      e-mail: [email protected]
      Home page: http://www.tchin.org
    • Kids With Heart National Association for Children's Heart Disorders, Inc.
      1578 Careful Dr
      Green Bay WI 54304-2941
      Phone #: 920-498-0058
      800 #: 800-538-5390
      e-mail: [email protected]
      Home page: http://www.kidswithheart.org
    • Little Hearts, Inc.
      P.O. Box 171
      Cromwell CT 06416
      Phone #: 860-635-0006
      800 #: 866-435-4673
      e-mail: [email protected]
      Home page: http://www.littlehearts.org
    • March of Dimes Birth Defects Foundation
      1275 Mamaroneck Avenue
      White Plains NY 10605
      Phone #: 914-428-7100
      800 #: 888-663-4637
      e-mail: [email protected]
      Home page: http://www.marchofdimes.com
    • NIH/National Heart, Lung and Blood Institute Information Center
      P.O. Box 30105
      Bethesda MD 20824-0105
      Phone #: 301-592-8573
      800 #: --
      e-mail: [email protected]
      Home page: N/A
    • National Foundation For Transplants
      1102 Brookfield Road
      Memphis TN 38119
      Phone #: 901-684-1697
      800 #: 800-489-3863
      e-mail: [email protected]
      Home page: http://www.transplants.org
    • National Transplant Assistance Fund (NTAF)
      Suite 230
      Newtown Square PA 19073
      Phone #: 610-353-9684
      800 #: 800-642-8399
      e-mail: [email protected]
      Home page: http://www.transplantfund.org
    • Second Wind Lung Transplant Association, Inc.
      23609 Talbot
      St Clair Shores MI 48082
      Phone #: 586-294-3162
      800 #: 888-855-9463
      e-mail: [email protected]
      Home page: http://www.2ndwind.org

    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html