In addition to combination treatment of pulmonary artery hypertension (PHT) with currently available drug options (endothelin-receptor inhibitors, phosphodiesterase-5 inhibitors, prostanoids). Numerous new drugs of other substance groups are being tested in preclinical and clinical studies. Most of the newly tried drugs use previously identified mechanisms of action that are involved in the development and progression of the underlying vascular disease. This has led to a certain paradigm shift which, in addition to vasodilatation and anti-platelet aggregation treatment, increasingly focuses on antiproliferative effects with the aim of preventing or regressing vascular remodelling. Of particular interest in this connection are tyrosine kinase inhibitors, which inhibit the action of such peptide growth factors as platelet-derived growth factor. Imatinib, one of this class of action, has given promising results in experimental studies and several case reports. Another greatly promising approach in the treatment of PHT are activators and simulators of soluble guanylyl cyclase, which are also currently being investigated in clinical trials.