Important
It is possible that the main title of the report Eisenmenger Syndromeis not the name you expected.
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)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com
American Heart Association
National Center
7272 Greenville Avenue
Dallas, TX 75231-4596
Tel: (214)373-6300
Fax: (214)373-0268
Tel: (800)242-8721
Email: inquire@heart.org
Internet: http://www.americanheart.org
Congenital Heart Anomalies, Support, Education, & Resources
2112 North Wilkins Road
Swanton, OH 43558
Tel: (419)825-5575
Fax: (419)825-2880
Email: chaser@compuserve.com
Internet: http://www.csun.edu/~hcmth011/chaser/chaser-news.html
National Transplant Assistance Fund (NTAF)
Suite 230
3475 West Chester Pike
Newtown Square, PA 19073
USA
Tel: 6103539684
Fax: 6103531616
Tel: 8006428399
Email: NTAF@transplantfund.org
Internet: http://www.transplantfund.org
National Foundation For Transplants
1102 Brookfield Road
Suite 200
Memphis, TN 38119
USA
Tel: 9016841697
Fax: 9016841128
Tel: 8004893863
Email: info@transplants.org
Internet: http://www.transplants.org
NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
Tel: (301)592-8573
Fax: (301)251-1223
Email: nhlbiinfo@rover.nhlbi.nih.gov
American Organ Transplant Association
PO Box 667566
Houston, TX 77266-7566
Tel: (281)996-8799
Fax: (413)803-9178
Internet: http://www.aotaonline.org
Second Wind Lung Transplant Association, Inc.
23609 Talbot
St Clair Shores, MI 48082
USA
Tel: 5862943162
Fax: 7273973609
Tel: 8888559463
Email: beanpahoun@aol.com
Internet: http://www.2ndwind.org
Children's Heart Association for Support and Education
c/o The Cardiac Clinic, Division of Cardiology
The Hospital For Sick Children
555 University Avenue
Toronto
Ontario, M5G 1X8
Canada
Tel: 4164102427
Email: kidheart@angelfire.com
Internet: http://www.angelfire.com/on/chase/
Kids With Heart National Association for Children's Heart Disorders, Inc.
1578 Careful Dr
Green Bay, WI 54304-2941
Tel: (920)498-0058
Fax: (920)498-0058
Tel: (800)538-5390
Email: kidswithheart@greenbaynet.com
Internet: http://www.kidswithheart.org
Little Hearts, Inc.
P.O. Box 171
Cromwell, CT 06416
USA
Tel: 8606350006
Fax: 8606350006
Tel: 8664354673
Email: info@littlehearts.org
Internet: http://www.littlehearts.org
Congenital Heart Information Network
1561 Clark Dr
Yardley, PA 19067
Tel: (215)493-3068
Fax: (215)493-3068
Email: mb@tchin.org
Internet: http://www.tchin.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
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, 2006National Organization for Rare Disorders, Inc.
