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Eisenmenger Syndrome

National Organization for Rare Disorders, Inc.

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.

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