Eisenmenger Syndrome

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Eisenmenger Syndrome is not the name you expected.

Disorder Subdivisions

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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.

Supporting Organizations

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
Website: http://www.heart.org

American Organ Transplant Association

P. O. Box 418
Stilwell, KS 66085
Tel: 832-930-AOTA (2682)
Email: aotaonline@gmail.com
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
Email: mb@tchin.org
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: michelle@kidswithheart.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
Email: info@littlehearts.org
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 preguntas@nacersano.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: nhlbiinfo@rover.nhlbi.nih.gov
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: info@transplants.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: barlows9303@gmail.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.