Pulmonary hypertension · Pulmonary hypertension in lung disease
Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease
| Design | Randomised, double-blind, placebo-controlled trial |
|---|---|
| Treatment | Inhaled treprostinil four times daily |
| Control | Placebo |
| Population | 326 patients with pulmonary hypertension due to interstitial lung disease |
| Follow-up | 16 weeks |
| Primary endpoint | Change from baseline in six-minute walk distance at week 16 |
| Result | Least-squares mean difference in six-minute walk distance 31.12 m (95% CI 16.85 to 45.39, p<0.001) favouring treprostinil. NT-proBNP fell by 15% with treprostinil while rising in the placebo group. |
| Secondary endpoints | NT-proBNP, clinical worsening, FVC, exacerbations of underlying lung disease |
In patients with pulmonary hypertension due to interstitial lung disease, inhaled treprostinil improved exercise capacity from baseline, assessed with the use of a 6-minute walk test, as compared with placebo.
The trial authors, in the published abstract
The first positive trial in group 3 pulmonary hypertension, a group in which systemic vasodilators had repeatedly failed or caused harm by worsening ventilation-perfusion matching. Delivering the drug by inhalation to ventilated lung is the mechanistic argument, and it worked. It also motivated TETON-2, testing treprostinil in IPF without requiring pulmonary hypertension at all.
Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med 2021 Jan 28;384(4):325-334.