Clinical Progress of a Patient Treated with Prostacyclin Infusion for 5 Years and Developments in the Treament of Primary Pulmonary Hypertension

A tweutyeiglıt year old ma/e patient first preselli ed in !995 w !ten !te was 21 years old w it/ı symptoms of !temoptysis and dyspnea. T!te diagnosis of primary pulmonary !typertension was m ade after excluding ot/ter etiologies of pulmonary arterial !typertemion.T!te pat i em w/to !tad a history of familial primary pulmonary lıyperlension was tremed wit!t ca/ci um c/ıamıel blackers and warfarin for 2 years. Because of tlı e increase in t!te patient's symptoms t!te tlı erap/ıy was clıanged to intravenous prostacyc/in. Wit!t this t!teraplıy tlıe symptoms of tlıe patient recovered and lıis survival was prolonged. However. most of tlı e side e.ffects and complications related to iutravenous prostacycline infiısionwere also seen. After recieving prostacyc/infor 5 years. t!te symptoms started agailı. We decided to add endot/ıelin receptar mıtagonists(bosentan) and/or plıop/ıodiesterase in!tibitors(sidenafi/) to t!te treatmelli reg inıeu. We are reporting tftis case to s ftm·e our experience with long temı epoprosteuol treatment. Au overview of the modem treatment of primary pulmouary lypertension is also given.