Swiss medical weekly
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Swiss medical weekly · Oct 1994
Review[Therapy of primary pulmonary arterial hypertension: current status].
Primary pulmonary hypertension is a rare disease with a median survival of only 2.8 years after diagnosis. In this review article the minimal diagnostic workup is summarized and pathophysiological concepts are discussed. Therapeutic options today include oral anticoagulation, which has a proven survival effect. ⋯ In the majority of patients there will be no improvement, but possibly deleterious hemodynamic effects. Long term trials with both nifedipine and prostacyclin indicate a significant survival effect in those patients who initially respond to this treatment. Lung transplantation remains the only treatment option for patients with uncontrollable pulmonary hypertension.