Sustained long-term benefit of sildenafil in primary pulmonary hypertension.

Primary pulmonary hypertension is a rare disease with an ominous prognosis for which new therapeutic options are being developed. Recently, there have been case reports indicating that sildenafil may be of benefit in short- to midterm follow-up. However, long-term clinical data of sildenafil in primary pulmonary hypertensive patients is lacking. We report a patient with primary pulmonary hypertension treated with sildenafil 200 mg/day for 50 months with a sustained clinical response.