Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some people who were born with structural problems of the heart. Eisenmenger syndrome is a condition that results from abnormal blood circulation caused by a defect in the heart. Most often, people with this condition are born with a hole between the two pumping chambers the left and right ventricles of the heart.
Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.Patients in underdeveloped countries are more likely to present late with uncorrected congenital cardiac lesions and a markedly elevated pulmonary vascular resistance (PVR). They are more likely to be inoperable secondary to Eisenmenger physiology.
Eisenmenger syndrome develops over time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs because of congenital heart defects that cause blood to flow from the left side of the heart to the right side of the heart (left-to-right shunt). Congenital heart defects of this type include:
• Patent ductus arteriosus (PDA). A connection between the aorta and the pulmonary artery that allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.
• Atrial septal defect (ASD). An opening in the atrial septum, or dividing wall between the two upper chambers of the heart known as the right and left atria.
• Ventricular septal defect (VSD). An opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles.
• Atrioventricular canal defect (AV canal). A heart problem that involves several abnormalities of structures inside the heart, including an ASD, VSD, and improperly formed mitral and/or tricuspid valves.
The goals of treatment for Eisenmenger syndrome are aimed at decreasing the pulmonary artery pressure, improving oxygenation, and decreasing degree of cyanosis and erythrocytosis. Treatment methods may include, but are not limited to, the following: