New horizons for the use of the second generation of endothelin receptor antagonist macitentan in patients with pulmonary hypertension

Since 2015, macitentan, the new oral dual endothelin receptors antagonist (ERA), has been successfully introduced into clinical practice in the Russian Federation for the treatment of pulmonary arterial hypertension (PAH) patients. It has improved physicochemical properties, which provides tissue specificity. Among ERA and other drugs of specific therapy, macitentan is the only one with indication-prevention of PAH progression. In the randomized, placebo-controlled study SERAPHIN in 742 PAH patients aged >12 years, macitentan 10 mg compared with placebo reduced the risk of morbidity and mortality by 45%. By month 6, macitentan with favorable tolerability profile provided a significant increase in the 6-minute walk test distance, the improvement of the functional class (FC) and hemodynamic parameters - pulmonary vascular resistance (PVR) and cardiac index. Macitentan significantly reduced the need for hospitalization for all reasons, including associated with PAH worsening. This review presents a modern view on the possibility of using macitentan in the clinical practice. It is shown that the drug is an important choice among ERA for the treatment of PAH patients. In the study MERIT-1 macitentan significantly improved PVR (geometric mean ratio 0.84, 95% CI 0.70-0,99, p=0.041) in inoperable CTEPH patients. The evidence base for the macitantan use in various PAH subgroups, including portopulmonary hypertension, in children as well as beyond group 1 - CTEPH, left heart diseases - is supplementing with the new data, which will expand the possibilities of its clinical use.