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

Abstract

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.