National Organization for Rare Disorders, Inc.
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.
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.
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
American Heart Association
7272 Greenville Avenue
Dallas, TX 75231-4596
Congenital Heart Anomalies, Support, Education, & Resources, Inc. (CHASER, Inc.)
2112 North Wilkins Road
Swanton, OH 43558
National Transplant Assistance Fund (NTAF)
150 N. Radnor Chester Road
Radnor, PA 19087
National Foundation For Transplants
5350 Poplar Ave
Memphis, TN 38119
NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
American Organ Transplant Association
PO Box 667566
Houston, TX 77266-7566
Second Wind Lung Transplant Association, Inc.
St Clair Shores, MI 48082
Children's Heart Association for Support and Education
c/o The Cardiac Clinic, Division of Cardiology
The Hospital For Sick Children
555 University Avenue
Ontario, M5G 1X8
Kids With Heart National Association for Children's Heart Disorders, Inc.
P.O Box 12504
Green Bay, WI 54307-2504
Little Hearts, Inc.
P.O. Box 171
110 Court Street, Suite 3A
Cromwell, CT 06416
Congenital Heart Information Network (C.H.I.N.)
101 N Washington Ave, Suite 1A 101 N Washington Ave, Suite 1A
Margate City, NJ 08402-1195
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
For a Complete Report
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