Real-life use of inhaled treprostinil in pulmonary hypertension associated with interstitial lung disease

Background: In the INCREASE trial, inhaled treprostinil improved 6-min walk distance (6MWD), time to clinical worsening, and decreased NT-pro-BNP levels in patients with pulmonary hypertension associated with interstitial lung disease (ILD-PH). However, hemodynamic data and real-world experience have not been sufficiently investigated. Methods: This multicenter, observational, prospective study assessed the clinical and hemodynamic outcomes of patients receiving inhaled treprostinil as compassionate use for severe ILD-PH (mPAP >20 mmHg, PAWP ≤15 mmHg, PVR >5 WU), between 2023/03 and 2024/10 in France. Data were extracted from the French PH registry. Results: 102 patients were treated, with a median age of 72 yrs (including 77 males; 31 IPF). Nine patients died, 3 underwent transplantation, and 5 discontinued treatment before follow-up, 5 were too clinically unstable to be reassessed. Follow-up data (Table) showed improvement in functional class in 19/80 patients (23.8%), increase in 6MWD >30 meters in 27/58 patients (46.6%), and decrease in PVR >20% in 30/79 patients (38%). Overall, no significant improvement was found in hemodynamic parameters, NT-pro-BNP levels, or lung function tests. Conclusion: Inhaled treprostinil led to hemodynamic improvement in only 38% of reassessed patients. This study found no significant hemodynamic difference in the overall cohort. erj;66/suppl_69/PA1914/TB1 T1 TB1 mPAP (mmHg) CI (l/min/m2) PVR (WU) FVC (%) DLCO (%) 6MWD (m) NT proBNP (ng/l) V0 38.5 [33-45] 2.20 [1.9-3.0] 6.80 [5.88-9.65] 77 [59.5-90] 23 [19.5-28.5] 246 [180-332] 460 [148-1614] V1 37.5 [30.5-43] 2.35 [2.00-2.63] 6.70 [5.04-8.05] 71 [57-87] 23 [18-27] 278 [178-345] 305.5 [111-1190] p 0.30 0.35 0.09 0.44 0.41 0.95 0.32