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Eisenmenger Syndrome
Condition Overview: Eisenmenger syndrome is a progressive heart condition that develops as a complication of certain structural heart malformations present at birth (congenital heart defects). Due to such defects, there may be abnormal blood flow between certain heart regions (e.g., from the left to the right lower chamber [ventricle] of the heart [left-to-right shunt]) or between major blood vessels (aorta and pulmonary artery) and a gradual rise in blood pressure within the pulmonary artery, which carries oxygen-poor (deoxygenated) blood to the lungs (i.e., pulmonary hypertension). Without early surgical correction of the underlying defect, such changes may cause progressive damage to and increased resistance to blood flow within small blood vessels in lung tissue (pulmonary vascular disease) and associated complications (e.g., reversed [right-to-left] ventricular shunt, etc.).The symptoms and signs of Eisenmenger syndrome often do not occur until about the second or third decade of life; however, they may develop more rapidly in some cases. Such findings may include bluish discoloration of the skin and mucous membranes (cyanosis), increasing difficulties breathing (dyspnea) with exertion, fainting episodes (syncope), and other abnormalities due to insufficient oxygen supply to body cells (hypoxia), leading to potentially life-threatening complications. Eisenmenger syndrome most frequently occurs as a complication of ventricular septal defects (VSDs). A VSD is characterized by an abnormal opening in the fibrous partition (septum) that normally separates the two lower heart chambers (ventricles), allowing abnormal blood flow between the two ventricles. Changes associated with Eisenmenger syndrome may also occur due to other septal defects of the heart, abnormal persistence of the fetal opening between the pulmonary artery and aorta (patent ductus arteriosus), or other congenital defects in which there is abnormal communication between the aorta and pulmonary artery. . |
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