[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. Furthermore, in a selected subgroup of patients, vasodilators may be effective in lowering pulmonary vascular resistance and pulmonary arterial pressure. Among these, prostacyclin and the calcium antagonists nifedipine and diltiazem are the most promising treatments. It is mandatory to perform an acute drug trial under close monitoring of pulmonary and systemic pressures as well as cardiac output before starting vasodilator treatment. 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.