Pulmonary hypertension is a multietiological disease. Other forms have to be excluded to establish this diagnosis. Pulmonary hypertension is classified into five categories. One of these forms is pulmonary arterial hypertension with special treatment options. There are three main pathophysiological pathways and corresponding treatment includes the administration phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostacyclin derivates. Novel therapeutic options are under development. Patients with pulmonary hypertension need regular care and their treatment has to be conducted in specified centres.
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