Eisenmenger syndrome is a three-stage syndrome that begins with a heart defect present at birth (congenital defect). Although it can sometimes be detected in newborns, it frequently does not cause any symptoms until adulthood.

Eisenmenger syndrome is the result of one or more specific congenital defects that cause high blood pressure in the lungs (pulmonary hypertension). This condition, in turn, eventually causes oxygen-poor blood to shift through a cardiac defect  into circulation. In its more advanced stages, it often causes a bluish tint (cyanosis) to the skin and other parts of the body.

Early diagnosis and treatment are important. Although symptoms frequently do not become obvious until adulthood, Eisenmenger syndrome patients often die before they turn 40. Also, because of the nature of the condition, Eisenmenger syndrome patients are counseled to avoid certain things, such as pregnancy and saunas. Treatment also involves medications to alleviate the patient’s signs and symptoms.

In severe cases, some patients may be eligible for a heart-lung transplant, in which the patient receives both a heart transplant and a lung transplant. Although heart-lung transplants are not performed very often, most people who have the surgery have a diagnosis of Eisenmenger syndrome. If possible, the physician may opt instead for a lung transplant and surgical repair of the heart defect.