A phase 2b, open label study to assess the safety and efficacy of increasing doses of pulsed, inhaled nitric oxide (iNO) in subjects with pulmonary fibrosis and sarcoidosis on long term oxygen therapy followed by a long term extension study
A phase 2b, open label study to assess the hemodynamic effects of increasing doses of pulsed, inhaled nitric oxide (iNO) in subjects with pulmonary hypertension associated with pulmonary fibrosis or sarcoidosis on long term oxygen therapy followed by an open label extension study
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
17
inhaled nitric oxide
University of Miami
Miami, Florida, United States
University of Cincinnati
Cincinnati, Ohio, United States
Temple University
Philadelphia, Pennsylvania, United States
Vanderbilt University Medical Center
Nashville, Tennessee, United States
Measurement of mean PAP
Mean pulmonary arterial pressure (mPAP) will be measured at iNO 30, 45, 75 and 125 mcg/kg IBW/hr
Time frame: During a single right heart catheterization procedure
Measurement of PCWP
Pulmonary capillary wedge pressure (PCWP) will be measured at iNO 30, 45, 75 and 125 mcg/kg IBW/hr
Time frame: During a single right heart catheterization procedure
Measurement of PVR
Pulmonary vascular resistance (PVR) will be measured at iNO 30, 45, 75 and 125 mcg/kg IBW/hr
Time frame: During a single right heart catheterization procedure
Measurement of CO
Cardiac output (CO) will be measured at iNO 30, 45, 75 and 125 mcg/kg IBW/hr
Time frame: During a single right heart catheterization procedure
Change in 6MWD from Baseline to 16 Weeks
Change in 6 minute walk distance
Time frame: 16 weeks
Incidence and Severity of Treatment Emergent Adverse Events
Including adverse events related to device deficiency
Time frame: During a single right heart catheterization procedure
Pulmonary Rebound
Symptoms associated with acute withdrawal of iNO: systemic arterial oxygen desaturation, hypoxemia, bradycardia, tachycardia, systemic hypotension, shortness of breath, near-syncope and syncope
Time frame: During a single right heart catheterization procedure
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Inova Heart and Lung Vascular Institute
Falls Church, Virginia, United States
University of Washington Medical Center
Seattle, Washington, United States
Distance Saturation Product (DSP)
Difference in DSP from baseline to Week 16 Difference in DSP from baseline to 16 weeks
Time frame: 16 weeks
Dyspnea
Difference in dyspnea as measured by UCSD Medical Center Pulmonary Rehabilitation Program Shortness of Breath Questionnaire on a scale from 0 (none at all) to 5 (maximal or unable to do because of breathlessness) from baseline to Week 16
Time frame: 16 weeks
Quality of Life Assessment
Difference in disease specific Quality of Life as measured by St. George's Respiratory Questionnaire
Time frame: 16 weeks
Incidence of Adverse Events and Serious Averse Events
Evaluation of adverse events and serious adverse events
Time frame: Through study completion; an average of 1 year
Integral Distance Saturation Product (IDSP)
Difference in IDSP from baseline to Week 16
Time frame: 16 weeks