Contemporary treatment patterns in patients with pulmonary arterial hypertension: insight from the observational exposure study

SESSION TITLE: Pulmonary Vascular Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Proceedings from the World Symposium on Pulmonary Hypertension recommend initial double combination therapy with an endothelin receptor antagonist (ERA) and a phosphodiesterase 5 inhibitor (PDE5i) for treatment of newly-diagnosed pulmonary arterial hypertension (PAH) patients with a low/intermediate risk status. Here we use real-world data from the EXPOSURE study to analyse treatment patterns in the subset of patients initiating PAH therapy with an ERA, a PDE5i or both. METHODS: EXPOSURE (EUPAS19085) is an ongoing, multicentre, prospective, observational study conducted in Europe and Canada which recruits PAH patients initiating either selexipag (Selexipag Cohort) or another PAH therapy (Other Cohort). Patients in the Other Cohort are included here. The analysis includes first-time initiators of ERA monotherapy (new ERA subgroup), PDE5i monotherapy (new PDE5i subgroup), or ERA and PDE5i double combination therapy (new ERA+PDE5i subgroup; both drugs must have been initiated within 30 days of each other) from September 2017 to June 2019. Patients initiating any other PAH therapy within 30 days after baseline were excluded from this analysis. RESULTS: Of the 291 patients included, 81 (27.8%) were in the new ERA group, 123 (42.3%) in the new PDE5i group and 87 (29.9%) in the new ERA+PDE5i group. Most patients had newly-diagnosed PAH (75% of the new ERA group diagnosed for < 1 year and 75% of the new PDE5i and new ERA+PDE5i groups diagnosed within 3 months prior to treatment initiation) and were in WHO functional class III. Patients in the new PDE5i group tended to be older (mean age 68 years) with a greater proportion (63.1%) having idiopathic PAH (IPAH) versus the new ERA (61 years; 42.5% IPAH) and new ERA+PDE5i (59 years; 54.0% IPAH) groups. After IPAH, the most frequently reported etiologies were PAH-associated with connective tissue disease (range: 17.2% to 31.3% of patients per subgroup) and PAH-associated with congenital heart disease (range: 5.7% to 22.5% of patients per subgroup). Patients in each subgroup were predominantly at intermediate risk for 1-year mortality (determined using COMPERA risk assessment): 45 (59.2%) in the new ERA group, 73 (63.5%) in the new PDE5i group and 62 (73.8%) in the new ERA+PDE5i group. CONCLUSIONS: EXPOSURE provides real-world, contemporary data on treatment patterns in PAH patients. In this analysis, the majority of patients, despite being newly diagnosed with PAH and at intermediate risk, were initiated on monotherapy rather than upfront double combination therapy. CLINICAL IMPLICATIONS: EXPOSURE provides contemporary real-world data and insights into treatment patterns in PAH. DISCLOSURES: Employee relationship with Janssen Pharmaceutical Company Please note: >$100000 Added 06/02/2020 by Patricia Biedermann, source=Web Response, value=Salary No relevant relationships by PILAR ESCRIBANO-SUBIAS, source=Web Response Scientific Medical Advisor relationship with Actelion Please note: $5001 - $20000 Added 06/09/2020 by Sean Gaine, source=Web Response, value=Honoraria Scientific Medical Advisor relationship with United Therapeutics Please note: $1001 - $5000 Added 06/09/2020 by Sean Gaine, source=Web Response, value=Honoraria Scientific Medical Advisor relationship with Novartis Please note: $1-$1000 Added 06/09/2020 by Sean Gaine, source=Web Response, value=Honoraria Scientific Medical Advisor relationship with Daichio Sanyko Please note: $1001 - $5000 Added 06/09/2020 by Sean Gaine, source=Web Response, value=Honoraria Consultant relationship with Menerinni Please note: $1001 - $5000 Added 06/09/2020 by Sean Gaine, source=Web Response, value=Travel Advisory Committee Member relationship with Actelion Please note: $1001 - $5000 Added 05/25/2020 by Tobias Lange, source=Web Response, value=Honoraria Advisory Committee Member relationship with Acceleron Pharma Please note: $1001 - $5000 Added 05/25/2020 by Tobias Lange, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with BMS Please note: $1-$1000 Added 05/25/2020 by Tobias Lange, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Janssen-Cilag Please note: $1001 - $5000 Added 05/25/2020 by Tobias Lange, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with MSD Please note: $1001 - $5000 Added 05/25/2020 by Tobias Lange, source=Web Response, value=Honoraria Employee relationship with janssen pharmaceutical company Please note: $5001 - $20000 Added 06/02/2020 by Audrey Muller, source=Web Response, value=Salary Advisory relationship with Actelion Please note: $1001 - $5000 Added 05/21/2020 by Stefan Söderberg, source=Web Response, value=Honoraria Advisory Committee Member relationship with Actelion Please note: $1001 - $5000 Added 05/21/2020 by Stefan Söderberg, source=Web Response, value=Honoraria Employee relationship with Janssen Pharmaceutical Compamy Please note: >$100000 Added 06/02/2020 by Hong Sun, source=Web Response, value=Salary