Vaskulárna medicína 3/2011

Current status and perspectives of pharmacotherapy of pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a primary disease of pulmonary arterioles resulting in progressive precapillary pulmonary hypertension and, if untreated, it relatively rapidly leads to right ventricular failure and death. PAH either occurs due to unknown causes or is associated with a known cause (connective tissue disease, liver disease, congenital heart disease, HIV infection or abuse of some anorectic drugs). Current pharmacotherapy of PAH can be divided into supportive (treatment of heart failure, anticoagulation treatment, O2 therapy) and specific (calcium channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors). The method of treatment is determined by the use of acute pulmonary vasodilation testing. Treatment with high doses of calcium channel blockers is only indicated in patients with a positive test. Those who test negative receive, in addition to long-term anticoagulation treatment, a specific pharmacotherapy as monotherapy or combination therapy. Newly investigated experimental treatment options in PAH include prostacyclin receptor agonists, soluble guanylate cyclase activators and stimulators, statins, serotonin receptor antagonists and serotonin transporter blockers, Rho kinase inhibitors, vasoactive intestinal peptide or tyrosine kinase inhibitors.

Keywords: pulmonary arterial hypertension, supportive therapy, specific treatment, prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, experimental treatment options