Background: Epoprostenol significantly improves function and survival in patients with pulmonary arterial hypertension (PAH) but is associated with many risks and side effects. Furthermore, effective oral therapy is now available. We report our long-term experience with 13 patients from among 118 treated with epoprostenol who were able to be weaned to oral therapy, including 6 with persistently abnormal hemodynamics (mean pulmonary artery pressure > or = 35 mm Hg).
Methods: Oral therapy with bosentan (n = 11) or sildenafil (n =2) was started before weaning epoprostenol in all but 1 patient. Right heart catheterization was performed when patients reached a dose of 2 ng/kg/min, and epoprostenol was discontinued with hemodynamic monitoring. Functional class and 6-minute walk test were assessed at regular intervals. Repeat right heart catheterization was performed 1 year after discontinuation of epoprostenol.
Results: Nine patients remained on oral therapy alone for up to 46 months. Four patients deteriorated in functional class, and 2 of them resumed epoprostenol therapy. Inhaled iloprost was started in another patient. One additional patient died, unrelated to PAH. Twelve patients underwent right heart catheterization at the time of epoprostenol discontinuation. Hemodynamic evaluation 13.2 +/- 0.9 months later showed that the 5 patients with normal or nearly normal hemodynamics at the time of discontinuation of epoprostenol had no deterioration, whereas 4 of the 7 patients with abnormal hemodynamics had worsened. The 6-minute walk test at last follow-up was not significantly changed from maximal distance on epoprostenol (420 +/- 94 vs 412 +/- 95 meters).
Conclusion: Weaning from epoprostenol to sildenafil or bosentan with sustained clinical improvement is possible, even with persistent pulmonary hypertension; however, patients with persistently abnormal hemodynamics are at risk for hemodynamic and clinical deterioration and require close follow-up.