Interní Med. 2010; 12(4): 182-185

Current status and perspectives of pharmacotherapy of pulmonary arterial hypertension

MUDr.Pavel Jansa

II. interní klinika kardiologie a angiologie,

Centrum pro plicní hypertenzi VFN a 1. lékařská fakulta UK, Praha

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 conventional (treatment of heart failure, anticoagulation

treatment, calcium channel blockers) and specific (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.

phosphodiesterase-5 inhibitors, experimental treatment options.

Keywords: pulmonary arterial hypertension, conventional treatment, specific treatment, prostanoids, endothelin receptor antagonists,

Published: May 1, 2010  Show citation