CLINICAL CASE OF SUCCESSFUL 10-YEAR AMBRIZENTAN TREATMENT OF THE PATIENT WITH IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION

Abstract

Idiopathic pulmonary arterial hypertension (IPAH) is a rare cardiovascular disease of unknown etiology, characterized by pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) increase due to pronounced remodeling of small pulmonary arteries and arterioles, with progressive deterioration and the rapid development of right ventricle failure. The key pathogenetic feature is the endothelial dysfunction, which leads to the development of vasoconstriction, thrombosis in situ, proliferative and inflammatory changes in the lung microvasculature. Optimization of drug therapy is associated with the clinical implementation of highly effective pathogenetic drugs which act on the main disease targets - activation of endothelin-1 (ET-1) system, the lack of endogenous prostacyclin and nitric oxide. The role of ET-1 as a powerful vasoconstrictor that induces cell proliferation and differentiation, production of cytokines, growth factors, biologically active substances. Endothelin receptor antagonists (ERA) represents the important class of PAH-specific therapy, including two non-selective ERAs-nonselective bosentan and selective ambrizentan. Presented clinical case demonstrates the long-term reliable efficacy and safety of ambrizentan for 10 years in the IPAH patient with the baseline functional class III (WHO).