P3560Real-world experience with concomitant macitentan and riociguat treatment in patients with pulmonary hypertension (PH) in the OPsumit USers (OPUS) registry

Abstract

Introduction: Data on the use of the endothelin receptor antagonist macitentan and the soluble guanylate cyclase stimulator riociguat to treat pulmonary arterial hypertension (PAH) in combination are limited. Purpose: OPUS (NCT02126943) provides experience in a real-world setting of the safety of macitentan and riociguat therapy in combination in PH patients. Methods: OPUS is an ongoing, long-term, prospective, multicentre, observational, drug registry of patients newly treated with macitentan in the US. This analysis includes enrolled patients who received concomitant treatment with macitentan and riociguat. Patient characteristics are described at macitentan initiation. Safety data are described from the time when patients were receiving both macitentan and riociguat and are descriptively compared with data in the overall PH population with follow-up. Results: As of October 2017, OPUS included 1512 PH patients newly treated with macitentan and with follow-up data; of these, 125 patients were treated concomitantly with riociguat. The reason for macitentan prescription included PAH (n=96, 77%) and chronic thromboembolic PH (CTEPH; n=23, 18%). At macitentan initiation, the median (range) age of the 125 patients was 62.0 (18-88) years and 93 (74%) patients were female. The median (Q1, Q3) exposure to concomitant macitentan and riociguat was 6.3 (2.5, 14.3) months; 34% and 17% of patients had exposures >12 and >18 months. Macitentan was initiated before riociguat in 59 (47%) patients. Adverse events (AEs) experienced during the exposure period are shown in the table. Conclusion: OPUS is the largest cohort of patients newly treated with macitentan and provides insight into real-world treatment, including combination therapy with macitentan and riociguat. These data show that there are no unexpected safety findings with this combination in patients with PH.