Macitentan in incident and prevalent pulmonary arterial hypertension (PAH): OPUS/OrPHeUS real-world data

Introduction: Patients (pts) newly diagnosed with PAH typically have worse prognosis than pts with longer disease durations. The OPUS drug registry and OrPHeUS medical chart review provide real-world data in US PAH pts newly treated with macitentan. Aims and objectives: To describe characteristics and outcomes in incident and prevalent PAH pts in OPUS/OrPHeUS. Methods: OPUS is a prospective, multicentre, long-term, observational drug registry (Apr 2014–present; NCT02126943). OrPHeUS was a multicentre medical chart review (Oct 2013–Mar 2017; NCT03197688). Pts initiating macitentan ≤6 months after diagnosis were defined as incident, >6 months as prevalent. Results: By Sept 2019, OPUS/OrPHeUS included 1955 incident and 2347 prevalent pts. At macitentan initiation, demographics were similar between the groups. Key parameters are shown in the table. Conclusions: Incident pts had greater disease severity at macitentan initiation, yet 41% were on monotherapy at 6 months. In addition, discontinuations, hospitalisations and survival at 1 year were similar in both groups.