Efficacy and determinants of response to inhaled treprostinil in pulmonary hypertension - interstitial lung disease

Inhaled treprostinil has shown to improve exercise capacity in patients with pulmonary hypertension-interstitial lung disease (PH-ILD). We evaluated the efficacy and determinants of favorable response to inhaled treprostinil at six months. Methods: Of the 106 patients screened, 42 were eligible for this retrospective single-center study. Assessments included rate of patients who achieved >= 30m improvement on 6-min walk test (6MWT), and death or transplantation rates at 6 months of treatment initiation. Results: Patients were predominantly female (n = 26, 62 %) with autoimmune PH-ILD (n = 23, 55 %), and a median age of 68 (61, 75) years. Ten (38.5 %) patients achieved a distance increase >= 30 m in 6MWT. No statistically significant determinants of walking >= 30 m were noted on univariate analysis; however, responders had a lower right ventricular (RV) tissue Doppler S' velocity (9.2 [7.0, 11.0] vs. 11.9 [10.0, 14.4], p = 0.018) cm/s and evidence of pericardial effusion on baseline echocardiogram (82 % vs. 26 %, p = 0.003). PH-ILD patients who died or underwent transplantation were more likely to have progressive pulmonary fibrosis (PPF) (95 % vs 50 %, p < 0.001) Conclusions: In real-world setting, treatment with inhaled treprostinil for six months increased the 6MWT by >= 30 m in about a third of PH-ILD patients. Lower RV tissue Doppler S' velocity and presence of pericardial effusion at baseline were associated with favorable response to inhaled treprostinil. PPF portends a poor survival in PH-ILD.