Effects of continuous intravenous epoprostenol therapy on advanced primary pulmonary hypertension in Taiwanese patients

Primary pulmonary hypertension (PPH) is a progressive disease for which there is no cure. Continuous intravenous infusion of epoprostenol in selected patients with advanced PPH improves symptoms and survival, but the long-term impact has not been reported in Taiwanese patients. Four patients with advanced PPH treated with epoprostenol therapy were enrolled in this study from July 2000 to September 2001. The basic hemodynamic status, cardiothoracic ratio, 6-minute-walk test results, maximum oxygen consumption, New York Heart Association functional class and survival were re-evaluated before and after 12 months of epoprostenol treatment. One of the patients died after 10 months of epoprostenol therapy due to heart failure, and the other 3 were still alive after a mean of 28 months. The surviving patients had pronounced symptomatic, hemodynamic, functional and survival benefit from epoprostenol therapy. Our results suggest that chronic infusion of epoprostenol can improve functional status and survival of Taiwanese patients with advanced PPH disease.