Idiopathic pulmonary arterial hypertension: current approach to diagnosis and pharmacological treatment

Current aspects of diagnosis, classification and therapy of pulmonary hypertension (PH) were reviewed in the article. A new classification, including all known clinical variants of PH, and functional classification were presented. A diagnostic algorithm with routine diagnostic methods and highly sensitive innovative techniques (CT-angiography of the lungs, perfusion scintigraphy, the right heart catheterization, etc.) was shown. The author discussed prognostic factors of 1-year survival, general pathogenic mechanisms including endothelin and prostacyclin pathways and a role of nitric oxide and soluble guanylate cyclase stimulators. Assessment criteria of acute vasoreactivity test results were reviewed. Typical therapeutic approaches including calcium channel blockers, oxygen, anticoagulants and diuretics were described. Main classes of specific drugs were listed such as endothelin receptor antagonists, prostacyclins, soluble guanylate cyclase stimulators, phosphodiesterase 5 inhibitors, selective prostacyclin receptor agonists. Current investigations are directed to searching new effective and safe drugs that could improve outcomes, slow down progression of the disease and improve quality of the patient’s life.