Bellerophon Announces First Patient Enrolled in Phase 2b Study Evaluating INOpulse® for Treatment of Pulmonary Hypertension Associated with Interstitial Lung Disease

WARREN, N.J., Jan. 03, 2018 -- Bellerophon Therapeutics, Inc. (Nasdaq:BLPH), a clinical-stage biotherapeutics company, today announced that the first patient has been randomized into a Phase 2b study evaluating INOpulse® in patients with Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD).  The first patient was treated at The Lung Research Center in Chesterfield, MO, by Neil A. Ettinger, M.D.

The iNO-PF Phase 2b, randomized, double-blind, placebo-controlled clinical study will assess the safety and efficacy of pulsed, inhaled nitric oxide (iNO) versus placebo in patients with PH-ILD, including patients with idiopathic pulmonary fibrosis (PH-IPF).  A total of 40 subjects will be enrolled in this clinical trial.  The study design includes a 1-week run-in period, followed by an 8-week double-blinded treatment period.  The primary endpoint of the study is the change in 6 Minute Walk Distance from baseline to week 8.  The trial will include several additional endpoints, including the change in Right Ventricular Function.

“The initiation of our iNO-PF study represents an important milestone in our INOpulse development program,” said Fabian Tenenbaum, Chief Executive Officer of Bellerophon.  “PH associated with ILD, including PH-IPF, are devastating conditions with no approved therapies.  Patients suffering from these conditions represent an orphan population with a mean survival of only two years from diagnosis and for whom systemic vasodilators have proven to be ineffective.  The proprietary and targeted INOpulse mechanism of action, and the positive data generated from our previously completed Phase 2a study, suggest that INOpulse has the potential to become the first approved therapy to address this critical treatment void.  We look forward to working with our Steering Committee and investigators to continue the development of INOpulse for PH-ILD.”

Top-line results from this study are expected to be available by the end of 2018.  Additional details of the trial can be found at www.clinicaltrials.gov (NCT 03267108).

About Bellerophon

Bellerophon Therapeutics is a clinical-stage biotherapeutics company focused on developing innovative therapies at the intersection of drugs and devices that address significant unmet medical needs in the treatment of cardiopulmonary diseases. The Company is currently developing three product candidates under its INOpulse program, a proprietary pulsatile nitric oxide delivery system. The first is for the treatment of pulmonary arterial hypertension (PAH), for which the Company has commenced Phase 3 clinical trials. The second is for the treatment of pulmonary hypertension associated with chronic obstructive pulmonary disease (PH-COPD) and the third candidate is for the treatment of pulmonary hypertension associated with Interstitial Lung Disease (PH-ILD), both of which are in Phase 2 development.  For more information, please visit www.bellerophon.com.

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