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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 200mg/day for 50months with a sustained clinical response.