[Hopes and experiences in the treatment of severe pulmonary hypertension].

20 scribed prolonged benefits of this therapy in hemodynamic parameters, functional capacity and survival. Other 2002 and 2003 studies, such as Pombo et al in the current issue of REVISTA ESPAÑOLA DE CARDIOLOGÍA, have collected between 8 and 10 years of experience in treating pulmonary arterial hypertension with intravenous epoprostenol.4-6 They all agree in the essential message: intravenous epoprostenol therapy will improve long-term survival and functional capacity in severe pulmonary hypertension. However, survival to 5 years of patients that underwent therapy was only 55% in the best case. The benefits of intravenous epoprostenol therapy is also counterbalanced by complications related to the administration system (infections, catheter thrombosis, rebound pulmonary hypertension due to pump failure). These difficulties have lead to finding new drugs for treating pulmonary hypertension, of which the new prostaglandin analogues administered subcutaneously (treprostinil), orally (beraprost) or inhaled (iloprost)1,7-9 are the most promising. These new drugs not only have demonstrated a benefit in exercise tolerance and hemodynamic parameters in uncontrolled studies, but also have shown positive results in double-blind and placebo-controlled randomized clinical trials. The results could be similar as to those shown with epoprostenol. Another group of drugs for treating severe pulmonary hypertension are the endothelin receptors antagonists 1 (ET-1). Endothelins are vasoconstriction peptides secreted by the endothelian cells. Endothelin plasma levels are directly correlated to pulmonary arterial pressure and pulmonary vascular resistance. An ET-1 receptor antagonist, bosentan, was approved by the Food and Drug Administration (FDA) in November, 2001, for treating patients with NYHA functional class III-IV pulmonary hypertension. These drugs improve functional capacity and hemodynamic parameters, although their use is frequently limited by secondary effects, such as potentially severe hepatoxicity and teratogenicity, and by multiple pharmacological interactions.1 A short-term and long-term benefit has been recently communicated in severe pulmonary hypertension ED I TO R I A L S