Inhaled Treprostinil treatment in pulmonary hypertension associated with interstitial lung disease in an outermost region of Europe.

Background. According to the European clinical practice guidelines inhaled treprostinil may be considered in patients with pulmonary hypertension (PH) associated with interstitial lung disease (ILD). Methods. Multicentre, observational study in PH associated with ILD patients, diagnosed with right heart catheterisation and treated with inhaled treprostinil. The main objective is to describe the experience in Canary Islands with inhaled treprostinil in PH associated with ILD. Patients included had a precapillary haemodynamic profile and ILD with >20% involvement in the chest CT scan. Clinical, haemodynamic and survival characteristics were collected. Results. Eighteen patients were included. Mean age was 74± 8 years (50% female) and BMI was 26± 6 kg/m². The most frequent ILD was fibrosis-emphysema syndrome (38.9%). Patients presented haemodynamic severity (PAPm 38± 10mmHg and PVR 6.2± 2WU). 55.6% of patients received inhaled treprostinil monotherapy, the rest received different combination therapy. Three patients discontinued treatment in the first 6 months (two due to handling problems with the device and one due to cough). After treatment, patients significantly improved functional class and NT-proBNP levels (2040± 2235pg/mL vs 869± 985pg/mL, p= 0.045). Moreover, we observed a non-significant improvement in the 6-minute walk test (TM6M) from 310± 138m to 328± 147m (p= 0.436). Median survival was 56 months, with survival rates of 75% at 1 year, 40% at 3 years, and 25% at 5 years. Conclusions. Inhaled treprostinil in PH associated with ILD was well tolerated. In our series of patients, inhaled treprostinil improved functional class and NT-proBNP levels.