Long term treatment of pulmonary arterial hypertension with beraprost, an oral prostacyclin analogue

Objective-To evaluate the effects of one year's treatment with beraprost, a n orally active prostacyclin analogue, in patients with severe pulmonary hy pertension. Patients-13 patients with severe pulmonary hypertension. This was primary i n nine, thromboembolic in three, and caused by Eisenmenger syndrome in one. Methods-All patients underwent right heart catheterisation. Mean (SD) right atrial pressure was 5 (3) min Hg, mean pulmonary artery pressure was 48 (1 2) mm Hg, cardiac index was 2.6 (0.8) 1/min/m(2), and mixed venous oxygen s aturation was 68 (7)%. Beraprost was started at the dose of 20 mug three to four times a day (1 mug/kg/day), increasing after one month to 40 mug thre e to four times a day (2 mug/kg/day), with further increases of 20 tg three to four times a day in case of clinical deterioration. Main outcome measures-New York Heart Association (NYHA) functional class, e xercise capacity measured by distance walked in six minutes, and systolic p ulmonary pressure (by echocardiography) were evaluated at baseline, after o ne month's treatment, and then every three months for a year. Results-After the first month of treatment,NYHA class decreased from 3.4 (0 .7) to 2.9 (0.7) (p < 0.05), the six minute walking distance increased from 213 (64) to 276 (101) m (p < 0.05), and systolic pulmonary artery pressure decreased from 93 (15) to 85 (18) mm Hg (NS). One patient died after 40 da ys from refractory right heart failure, and another was lost for follow up at six months. The 11 remaining patients had persistent improvements in fun ctional class and exercise capacity and a significant decrease in systolic pulmonary artery pressure in the period from 1-12 months. Side effects were minor. Conclusions-Oral administration of beraprost may result in long lasting cli nical and haemodynamic improvements in patients with severe pulmonary hyper tension.