National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Eisenmenger Syndrome is not the name you expected.
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.
American Heart Association
- 7272 Greenville Avenue
- Dallas, TX 75231
- Tel: (214)784-7212
- Fax: (214)784-1307
- Tel: (800)242-8721
- Email: Review.email@example.com
- Website: http://www.heart.org
American Organ Transplant Association
- P. O. Box 418
- Stilwell, KS 66085
- Tel: 832-930-AOTA (2682)
- Email: firstname.lastname@example.org
- Website: http://www.aotaonline.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
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- Website: 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
- Website: http://rarediseases.info.nih.gov/GARD/
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: firstname.lastname@example.org
- Website: http://www.kidswithheart.org
Little Hearts, Inc.
- P.O. Box 171
- 110 Court Street, Suite 3A
- Cromwell, CT 6416
- Tel: (860)635-0006
- Fax: (860)635-0006
- Tel: (866)435-4673
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- Website: http://www.littlehearts.org
March of Dimes
- 1275 Mamaroneck Avenue
- White Plains, NY 10605
- Tel: (914)997-4488
- Fax: (914)997-4763
- Email: AskUs@marchofdimes.org or firstname.lastname@example.org
- Website: http://www.marchofdimes.org and nacersano.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: email@example.com
- Website: http://www.nhlbi.nih.gov/
National Foundation For Transplants
- 5350 Poplar Ave
- Suite 430
- Memphis, TN 38119
- Tel: (901)684-1697
- Fax: (901)684-1128
- Tel: (800)489-3863
- Email: firstname.lastname@example.org
- Website: http://www.transplants.org
National Transplant Assistance Fund
- 150 N. Radnor Chester Road
- Suite F-120
- Radnor, PA 19087
- Tel: (610)353-9684
- Fax: (610)535-6106
- Tel: (800)642-8399
- Email: NTAF@transplantfund.org
- Website: http://www.transplantfund.org
Second Wind Lung Transplant Association, Inc.
- P.O. Box 1657
- Wimberley, TX 78676-1657
- Tel: (512)847-9303
- Tel: (888)855-9463
- Email: email@example.com
- Website: http://www.2ndwind.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, 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.
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.
Last Updated: 4/5/2008
Copyright 2006 National Organization for Rare Disorders, Inc.
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