A Randomized Double-Blind Placebo-Controlled Dose Escalation and Verification 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)

Are you eligible to participate in this study?

You may be eligible for this study if you meet the following criteria:

  • Conditions:
    Pulmonary Hypertension
  • Age: Between 18 - 79 Years
  • Gender: Male or Female
  • Other Inclusion Criteria:
    1. Able to provide written informed consent form
    2. Diagnosed with pulmonary fibrosis
    3. Historical right heart catheterization (within 3 years) or current Echocardiogram (within 3 months)
    4. Have been using oxygen therapy (at rest or only with exertion; and =10 L/min of oxygen supplementation) by nasal cannula
    5. 6 minute walking distance test (6MWD) = 100 meters and = 400 meters at screening
    6. WHO Functional Class II-IV
    7. Forced Vital Capacity = 40% predicted within last 6 months prior to the screening run-in period.
    8. 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
    9. Female study participants of childbearing potential must have a negative pre-treatment pregnancy test (serum or urine)
    10. Study participant must be clinically stable
    11. Demonstrate ability to wear the activity monitor consistently

You may not be eligible for this study if the following are true:

    1. Demonstrate symptomatic rebound defined as significant cardiopulmonary instability
    2. Episodes of disease worsening within 1 month
    3. Use of a prostacyclin analogue, guanylate cyclase stimulator, or endothelin-receptor antagonist (ERA) PAH-specific medications regardless of reason for use
    4. Acute or chronic physical impairment or adherence to therapy,
    5. Pregnant or breastfeeding females
    6. Administered L-arginine within 1 week prior to Screening
    7. 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.
    8. Use of investigational drugs or devices (including COVID-19 vaccination) within 1 month prior to Screening
    9. Any underlying medical or psychiatric condition that, in the opinion of the Investigator, makes the study participant an unsuitable candidate
    10. Any study participant who has been enrolled in any previous clinical study with inhaled NO
    11. A definitive diagnosis of a connective tissue disease
    12. The presence of emphysema unless the extent of fibrotic changes is greater than the extent of emphysema on the most recent HRCT scan
    13. 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
    14. 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
    15. 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.
    16. History of sarcoidosis
    17. History of chronic thrombo-embolic pulmonary hypertension
    18. Smoking within 3 months of Screening and/or unwilling to avoid smoking throughout the study
    19. Body mass index (BMI) >40 kg/m2 at screening
    20. Known permanent atrial fibrillation
    21. Documented pulmonary veno-occlusive disease
    22. Known severe hepatic impairment, in the opinion of the Principal Investigator
    23. Known severe renal impairment (Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) 2009 equation [Levy 2009] calculated creatinine clearance < 30 ml/min) at screening
    24. Life expectancy of < 6 months
    25. 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 prior to screening and, they are free of COVID-19 related symptoms



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