Ivan Varga, Eva Gonçalvesová
Pulmonary arterial hypertension (PAH) is a rare disease of pulmonary circulation with an unfavourable prognosis. Remodelling of pulmonary arteries in PAH results in the right heart failure. Diagnosis of the disease is complex with ultimate importance of right heart catheterization. PAH treatment consists of conventional and specific therapy as well as of interventional and surgical approaches. Specific pharmacotherapy includes calcium channel blockers, phosphodiesterase 5 inhibitors, endothelin receptor antagonists and prostanoids. Drugs lead to improvement of symptoms, hemodynamic parameters, and long-term survival. Their proper administration requires periodic reevaluation of treatment success in specialized centres.