Treatment The treatment of Eisenmenger syndrome should be managed by a medical team with expertise in both congenital heart disease (cardiologist) and pulmonary hypertension (cardiologist or pulmonologist), with help from other specialists, e.g. hematologists, radiologists, anesthetists etc. The goal of treatment is to minimize symptoms, and the treatment often aligns with patients who are being treated for other types of pulmonary arterial hypertension.
Currently, Eisenmenger patients are treated with pulmonary arterial hypertension therapies aimed at reducing the lung resistances and increase the amount of blood flowing through the lungs, hence delivering more oxygen to the body and reducing the load to the heart. Additional medications used include diuretics that reduce the amount of fluid in the body and, at times, medication to prevent blood clots. Beta blockers and especially calcium channel blockers are avoided, because they have a negative effect on the right ventricle. Iron supplementation may be necessary for individuals with iron deficiency anemia.
Affected individuals may have an increased risk of developing bacterial infections of the heart lining and valves (bacterial endocarditis); therefore, disease management includes the administration of appropriate antibiotics (antibiotic prophylaxis) prior to dental visits, for certain oral procedures. In addition, anesthesia and sedation carry significant risks and should be avoided. Careful monitoring during anesthesia is essential for any patients undergoing essential unavoidable surgical procedures, which should be performed in expert centers. Pregnancy should be avoided in women with pulmonary hypertension and Eisenmenger syndrome since it carries significant risks for both the mother and the developing fetus. Thus, it is essential that affected women have a thorough understanding of such risks and receive information, support, and guidance from their physicians and other members of their healthcare team concerning appropriate options to prevent pregnancy. Oxygen therapy has also been met with controversial reviews. There is no data to support its use as a mean for increasing exercise capacity or survival in adult patients. However, it has been seen to help patients with advanced disease in need of a heart-lung transplant or for nocturnal support.
Individuals with Eisenmenger syndrome should avoid dehydration, high altitudes, and activities that could cause a sudden drop in blood pressure such as saunas, steam rooms or hot tubs. Extreme physical exercise should also be limited. In severely affected patients with physical deterioration, a heart-lung transplant may be necessary.