A Clinical Study to Assess the Safety and Efficacy of Pulsed, Inhaled Nitric Oxide (iNO) in Subjects At Risk of Pulmonary Hypertension Associated With Pulmonary Fibrosis on Long Term Oxygen Therapy (Part 1 and Part 2) (REBUILD)

General Information Age Group Adults Status Active Protocol Number PULSE-PHPF-001 Background Information The purpose of this study is to determine if the INOpulse® delivery system, that includes the INOpulse® device, inhaled nitric oxide study drug as delivered via triple lumen nasal cannula can help treat patients with fibrotic interstitial lung disease (fILD) who are on oxygen therapy and who are at risk of having or developing pulmonary hypertension associated with pulmonary fibrosis (PH-PF). Specifically, the study will determine if the use of the INOpulse® delivery system can help improve daily levels of moderate to vigorous types of physical activity (for example walking, climbing stairs, household chores and cleaning and, exercise and hobbies), as measured by an activity monitor (a non-invasive way to monitor rest/activity). Offered At Inova Fairfax Hospital 3300 Gallows Rd. Falls Church, VA 22042 Principal Investigator Christopher King, MD Eligibility Information Forced Vital Capacity ≥ 40% predicted within last in the 60 day Screening period. Willingness to use INOpulse delivery device for as long as possible every day but no less than 12 hours per day and willingness to wear the activity monitor for as long as possible every day but no less than 10 hours per day while awake. Have been using oxygen therapy (including use limited to exertion) at ≤ 10 L/min of oxygen supplementation by nasal cannula for at least 4 weeks prior to the Screening Run-In period. Study participants must have completed at least 3 weeks (+/- 3 days) of activity monitoring at time of the Baseline/Randomization visit. During the Screening Run-In period prior to Baseline/Randomization, study participants must demonstrate the ability to consistently use the INOpulse device greater than 12 hrs/day in the opinion of the investigator. Age between 18 and 80 years (inclusive) at screening. Study participant must be clinically stable for at least 4 weeks prior to Baseline/Randomization in the opinion of the Principal Investigator. Demonstrate ability to wear the activity monitor consistently. Wear compliance will be assessed during the 14 days prior to Baseline/Randomization visit and is defined as at least 8 compliant days, that includes 2 weekend days, with at least 10 hours of wear time while awake per day. Ineligibility Information Episodes of disease worsening within 1 month (4 weeks) prior to Baseline/Randomization in the opinion of the investigator. Use of a prostacyclin, prostacyclin analogue, or prostacyclin receptor agonist, guanylate cyclase stimulator, or endothelin-receptor antagonist (ERA) PAH-specific medications regardless of reason for use, except for phospho-diesterase type-5 (PDE5) inhibitors. (The use of PDE-5 inhibitors regardless of reason for use is allowed as long as the dose has been stable for at least 3 months prior to screening and there are no plans to adjust the dose during the study) Acute or chronic physical impairment (other than dyspnea due to ILD +/- pulmonary hypertension) that would limit the ability to comply with study procedures (i.e., 6MWT) or adherence to therapy, including carrying and wearing the pulsed delivery INOpulse device per study protocol, or medical problem(s) likely to preclude completion of the study. For women with child-bearing potential: Pregnant or breastfeeding females at Screening, or planning to get pregnant, or unwilling to use appropriate contraception if sexually active to avoid pregnancy during the study and for at least 30 days after discontinuation of the study drug. The concurrent use of the INOpulse device with a continuous positive airway pressure (CPAP), Bilevel positive airway pressure (BiPAP), or any other positive pressure device. Use of investigational drugs or devices within 1 month prior to Screening. Any underlying medical or psychiatric condition that, in the opinion of the Investigator, makes the study participant an unsuitable candidate for the study including unable to complete 6MWT. Any study participant who has been enrolled in any previous clinical study with inhaled NO • Diagnosis of pulmonary arterial hypertension (PAH) or PH for reasons other than WHO Group 3 PH-ILD. The presence of emphysema unless the extent of fibrotic changes is greater than the extent of emphysema on the most recent HRCT scan in the opinion of the Investigator. A study participant must not start a rehabilitation program during the study, not have started within 3 months of screening or change the nature of their rehabilitation program at any time during the blinded portion of the study. Chronic use of a nitric oxide donor agent such as nitroglycerin or drugs known to increase methemoglobin such as lidocaine, prilocaine, benzocaine, nitroprusside, isosorbide, or dapsone at screening. Heart failure with reduced ejection fraction (HFrEF; systolic heart failure) with an ejection fraction of < 40%; or severe HF with preserved EF (HFpEF; diastolic HF) as assessed by the Principal Investigator. History of sarcoidosis. History of chronic thrombo-embolic pulmonary hypertension (CTEPH; WHO group 4 PH). Smoking within 3 months of Screening and/or unwilling to avoid smoking throughout the study. Body mass index (BMI) >40 kg/m2 at screening. Intermittent uncontrolled atrial fibrillation, in the opinion of the Principal Investigator. Documented pulmonary veno-occlusive disease. Known severe hepatic impairment, in the opinion of the Principal Investigator. Known severe renal impairment (Chronic Kidney Disease Epidemiology Collaboration (CKDEPI) 2009 equation [Levy 2009] calculated creatinine clearance < 30 ml/min) at screening. Life expectancy of < 6 months, in the opinion of the Principal Investigator. Positive COVID-19 infection (positive test or high clinical suspicion) in the 2 months prior to screening unless the potential study participant has had a negative COVID-19 test at least 6 weeks More Information: https://clinicaltrials.gov/ct2/show/NCT03267108 Contact Information Contact Name Priscila Dauphin, CCRC Contact Phone 703-776-7128 Contact Email Send Email