Pulmonary arterial hypertension (PAH) is a rare and rapidly progressing disease characterized by an increase in pulmonary resistance, which, if untreated, inevitably progresses to right ventricular heart failure and death. New treatment options were developed in the last 10 years. Endothelin receptor antagonists (ERAs) and prostanoids are an effective treatment with a better survival. Phosphodiesterase inhibitors (PDIs) are of a great interest, and first results in the treatment of patients are promising. PDIs are different in pulmonary selectivity and half-life.Right heart catheterization is the "gold standard" in the diagnosis of pulmonary hypertension. Direct classification of the pulmonary hypertension is feasible, and the effects of the drugs are directly measurable. The results of right heart catheterization are comparable for inhalative and oral drugs. New generations of drugs resulted in special testing protocols. Furthermore, individual concepts of therapy can be developed.