Interstitial lung disease with pulmonary hypertension and the potential for inhaled Treprostinil: A single centre perspective

Aim: As a large tertiary single ILD centre we evaluated the number of patients under our care who would potentially be eligible for inhaled Treprostinil. We also wanted to confirm the ILD phenotypes of this group as well as the effect of smoking history, co-morbidities, lung function and echocardiographic findings on mortality.

Methods: There are approximately 1800 patients under our care with over 300 new referrals a year. Between March 2019- March 2020, 250 echocardiograms were performed looking for evidence of pulmonary hypertension. Patients with non-invasive evidence of PH were included as per the INCREASE protocol.

Results: Approximately 15% of ILD patients had evidence of PH on echocardiography during this period. Of this cohort, 12 patients met criteria for consideration of inhaled Treprostinil if haemodynamic criteria were confirmed upon invasive measurement. These patients were predominately those with IPF and CHP. This group had a propensity for lower DLco and Kco with a previous smoking history. Of all patients with evidence of PH upon echocardiogram mortality was significant as 70% of patients had died within a 24-month period.

Conclusion: It is evident from a reviewing a single year that PH is evident in a substantial proportion of our ILD patients, however only a small proportion would fit the criteria for consideration of inhaled Treprostinil.