Primary pulmonary hypertension is a progressive disease, which carries a poor prognosis. To date, the efficacy of pulmonary vasodilator therapy has been limited. Despite the limitations of pulmonary vasodilator therapy, it has seen significant advances in recent years. One of these drugs, sildenafil, appears to be promising. Here, a 27-year-old young man with the diagnosis of primary pulmonary hypertension is presented. We added sildenafil 200 mg/day (qid) to the ongoing therapy of iloprost. At follow up one day and one month later, his exercise capacity was greatly improved and he enjoys a good quality of life without obvious side effects. Pulmonary artery pressure decreased from 112 mmHg to 73 mmHg one day later. Sildenafil needs to be evaluated in depth prospectively in the treatment of pulmonary hypertension.
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