Effects and problems of continuous infusion of epoprostenol for patients with primary pulmonary hypertension

Abstract

Objective We examined the usefulness and the problems of epoprostenol (Epo) therapy in adult Japanese with primary pulmonary hypertension (PPH). Subjects and Methods In eleven cases with PPH, both acute and chronic effects, and clinical effects of Epo were assessed. Results In the acute challenge test (n=6), Epo reduced both systemic and pulmonary vascular resistance and increased the cardiac output, but did not change the ratio of pulmonary to systemic vascular resistance, while the systemic and pulmonary blood pressure also did not change. In the chronic study (n=9), Epo decreased the pulmonary blood pressure without changes in systemic blood pressure, and increased the cardiac output. Both systemic and pulmonary vascular resistance decreased with a decrease in the ratio of the pulmonary to systemic vascular resistance. The level of brain natriuretic peptide and atrial natriuretic peptide, NYHA functional class and 6-minute walking distance were improved by Epo therapy. In spite of Epo therapy, two patients did not improve and died. Another patient improved in terms of symptoms but then died suddenly from massive lung bleeding. Two patients who improved physically could not be discharged because of psychiatric problems. One patient underwent lung transplantation. Five out-patients have been continuing Epo therapy. Conclusion We demonstrated that the chronic effect of Epo treatment is sufficient even at the dose used in our hospital. However, it was shown that there was resistance to this therapy in some of the cases and that we should pay attention to the severe adverse effects of Epo.