Eisenmenger's syndrome is a progressive congenital heart condition named after Dr. Victor Eisenmenger, who first described it in 1897. People who have the condition are usually born with a hole in the partition separating the ventricles of the heart (ventricular septal defect). This leads to increased blood flow to the lungs and an elevation of blood pressure in the lungs. When the high blood pressure becomes an irreversible problem on its own, the direction of blood flow through the hole in the heart is reversed, and the condition is called Eisenmenger's syndrome. It may develop early childhood, or it can develop slowly over many years, depending on the severity of the defect. Once Eisenmenger's develops, repairing the defect is not longer an option.

Adults with congenital heart disease require expert care for continued survival and quality of life. Patients need advice about many issues such as exercise, noncardiac surgery, preventing infections and potential future problems. Mayo Clinic cardiologists have expertise in congenital heart disease and work closely with cardiothoracic surgeons and other specialists to develop treatment plans unique to each patient's needs.

Treatment Options

Treatment for people with Eisenmenger's syndrome is aimed at controlling symptoms and preventing complications.

Medication

Medication is the primary treatment option. Medication is used to control rhythm disturbances and strengthen heart muscle contraction. Medication is also used to treat the symptoms of heart failure such as fluid buildup in the legs, abdomen and lungs.

Many medications used to treat the symptoms of Eisenmenger's syndrome will affect blood pressure, fluid and electrolyte (sodium, potassium, etc.) values and heart rate. These medications require close monitoring. A rapid change in blood pressure, fluid volume or pulse rate could decrease oxygen levels in the patient's blood to a critical level. Any medication that could cause blood pressure to fall should be carefully reviewed with the doctor.

Anticoagulants (medication that "thins" the blood or increases the time it takes to clot are occasionally used in this condition. Patients receiving anticoagulants such as aspirin or Coumadin must be monitored closely. Severe bleeding may cause a loss in blood volume and result in very low blood pressure, which could be disastrous for a patient who has Eisenmenger's syndrome.

A therapeutic phlebotomy (removal of some blood) may be considered to "thin" the blood. This must be done very carefully while replacing the removed blood with an equal volume of fluid such as sugar water to avoid a fall in blood pressure. Therapeutic phlebotomy should not be done routinely more than three times a year. More frequent phlebotomies could deplete the body of iron and cause iron-deficiency anemia. This can reduce oxygen-carrying capacity and increase the risk of stroke.

Surgery

After Eisenmenger's syndrome has developed, surgery to repair the defect is no longer possible. The lung's resistance to blood flow and the increased pressure needed to push blood through the lung would rapidly cause the repaired heart to enlarge and fail.

Heart and lung transplantation or lung transplant with repair of the shunt defect are options for some people with Eisenmenger's syndrome. Heart and lung transplantation is a very serious undertaking and fewer than 50 are done in the United States every year. As donor availability, surgical technology and drug research continue to advance, long-term results will also improve.