Successful withdrawal of long-term epoprostenol therapy for pulmonary arterial hypertension

Background: IV epoprostenol treatment of pulmonary arterial hypertension (PAH) has been believed to require an indefinite duration of therapy. Objective: To describe the successful discontinuation of long-term epoprostenol therapy in four patients. Design: Case reports. Setting: Outpatient clinic, tertiary-care hospital. Patients: Four patients with acutely nonvasoreactive, World Health Organization (WHO) functional class IV PAH received long-term epoprostenol therapy. All patients subsequently demonstrated normalization of pulmonary arterial pressures on epoprostenol. treatment. These patients were selected for epoprostenol withdrawal. Intervention: Down-titration and discontinuation of epoprostenol. Results: All four patients were safely transitioned from epoprostenol to oral therapies and have maintained WHO functional class I-II for a mean of 11 months (range, 8 to 16 months). The duration of epoprostenol therapy prior to discontinuation averaged 5.7 years (range, 2.4 to 13.5 years). Conclusion: Epoprostenol may sufficiently reverse the pathogenic process in select patients with PAH to allow a transition to less complex and less invasive treatment modalities.