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Switching to macitentan in pulmonary arterial hypertension

Background: Macitentan, an oral dual endothelin receptor antagonist (ERA), has demonstrated improved morbidity and mortality in patients with pulmonary arterial hypertension (PAH) in a long-term event-driven study. Aims: To provide data on clinical efficacy and safety of switching to macitentan, in adult PAH patients. Methods: PAH patients were included in this single center restrospective study, provided they were switched from another ERA to macitentan. Patients were managed following the currently held guidelines, and other PAH-specific treatment with phosphodiesterase-5 inhibitors and prostanoids were prescribed accordingly. Functional class, 6 minute walk distance, NT-proBNP levels and hemodynamic measurements were assessed. The last available measurements pre and the first available measurements post initiation of macitentan were compared. Safety end-points focused on liver enzymes and hemoglobin levels, as well as peripheral edema. Results: 10 PAH patients (3 males), aged 59±13 years, with a PAH history of 5±2years, were studied. They did not demonstrate any difference in functional class after switching to macitentan. They had an increase in 6 minute walk distance (from 407±81 m to 416±85, p=0.06), and a significant improvement in NT-proBNP levels (1044±878 ng/L to 832±901, p=0.03). A non-significant increase in cardiac output (from 4.9±0.6 L/min to 5±0.6, p=0.7) and decrease in pulmonary vascular resistance (from 5.9±2.5 Wood Units to 5.5±2.6, p=0.08) were observed. No episodes of increased liver enzymes, anemia or peripheral edema were observed. Conclusion: Switching to macitentan was well tolerated and associated with improved natriuretic peptide levels in our patient cohort.