Current treatment of pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare, progressive, life-threatening disease that leads to the right ventricular heart failure and premature death. Available specific therapies act primarily as vasodilators and have a limited effect on the biological cause of the disease, that is, the uncontrolled proliferation of vascular endothelial and smooth muscle cells. Currently used drugs for the treatment of PAH, as well as potential drugs that are in various phases of clinical trials are reviewed in the article. The authors discuss also the issue of drug provision for patients with PAH.