[Eisenmenger syndrome. Conventional treatments and general management]

Eisenmenger syndrome is a multisystem disorder, which evolves to severe complications in adult patients. It is recommended that patients be referred to a center with expertise in the fields of pulmonary hypertension and congenital heart disease. General measures include education of patients, avoidance of strenuous physical activity, pregnancy and infections, and careful intraoperative monitoring during noncardiac surgery. Conventional therapies for these patients are based on clinical experience rather than evidence based data. Phlebotomies should be reserved for patients with moderate-to severe hyperviscosity symptoms (usually when haematocrit is > 65 %). The use of oxygen therapy is controversial, but sometimes might improve symptoms. Oral anticoagulants are also controversial and can be used in patients with pulmonary thrombosis and without haemoptysis. Transplantation is restricted to highly symptomatic patients with poor prognosis. Novel disease targeting therapy may delay the need for transplantation for these patients.