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Successful Withdrawal of Long-term Epoprostenol Therapy for Pulmonary Arterial Hypertension^*

^* From the Pulmonary Vascular Center, University of California, San Diego, CA.

Correspondence to: Lewis J. Rubin, MD, FCCP, University of California, San Diego, 9300 Campus Point Dr, La Jolla, CA 92037; e-mail: ljrubin{at}ucsd.edu

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.

Key Words: epoprostenol • Flolan • pulmonary hypertension

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