Eisenmenger's syndrome

Treatment of Eisenmenger's syndrome focuses on controlling the symptoms and preventing complications that may occur from the combination of pulmonary hypertension with the abnormal blood circulation patterns in the heart. In pulmonary hypertension, elevated blood pressures develop only in the blood vessels of the lungs. Once Eisenmenger's syndrome develops, corrective surgery is not recommended and may even be detrimental. Repairing the hole in the heart would likely result in dangerously high blood pressure in the heart, due to the elevated pressures in the lungs. These high pressures in the heart can cause it to enlarge and eventually fail.

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The primary treatment options for patients with Eisenmenger's syndrome are medications used to control rhythm disturbances, optimize heart-muscle contraction, and reduce fluid buildup in the legs, abdomen and lungs. Occasionally, pulmonary vasodilators, medications that dilate the arteries in the lungs, have been successful at reducing the symptoms of Eisenmenger's syndrome. However, many of these medications can also affect blood pressure, fluid and electrolyte values, and heart rate. For a patient with Eisenmenger's syndrome, rapid changes in blood pressure, fluid volume, or pulse rate can exacerbate the condition and result in dangerously low levels of oxygen in the blood. Any medication that could lower blood pressure needs to be carefully reviewed and monitored by a congenital cardiologist.

Heart and lung transplantation or lung transplant with repair of the heart defect are options for some people with Eisenmenger's syndrome. Heart and lung transplants are rare--fewer than 50 are performed in the United States each year. As donor availability increases and surgical technology and drug research continue to advance, long-term results will also improve.

Complications from Eisenmenger's syndrome can be deadly. Even a simple operation requiring a general anesthetic (such as an appendectomy) is potentially life-threatening. Surgery should be performed at a medical center where the congenital cardiologist, surgeon, cardiac anesthesiologist, and staff have experience with Eisenmenger's syndrome. In addition, patients with Eisenmenger's syndrome need to discuss the use of vitamin supplements with their physician. Iron changes the chemistry of blood and should be used only if the blood iron level is low. Over-the-counter multivitamins often contain supplemental iron and should not be used without approval of the congenital cardiologist.