Evaluation of impact of inhaled nitric oxide on pulmonary vessel blood volume measured by high resolution computed tomography (HRCT) in subjects with World Health Organization (WHO) Group 3 Pulmonary Hypertension (PH) Associated with Chronic Obstructive Pulmonary Disease (COPD) on Long-Term Oxygen Therapy (LTOT) (Part 1) and in subjects with WHO Group 3 Pulmonary Hypertension (PH) Associated with Idiopathic pulmonary fibrosis (IPF) on Long-Term Oxygen Therapy (LTOT) (Part 2).

Phase 1

• Conditions: Part 1: Pulmonary Hypertension (PH) Associated with Chronic Obstructive Pulmonary Disease (COPD) Part 2: Pulmonary Hypertension (PH) Associated with Idiopathic pulmonary fibrosis (IPF); MedDRA version: 18.1Level: PTClassification code 10021240Term: Idiopathic pulmonary fibrosisSystem Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; MedDRA version: 18.1Level: LLTClassification code 10010952Term: COPDSystem Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; MedDRA version: 18.1Level: PTClassification code 10009033Term: Chronic obstructive pulmonary diseaseSystem Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; MedDRA version: 18.1Level: PTClassification code 10037400Term: Pulmonary hypertensionSystem Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; MedDRA version: 18.1Level: HLTClassification code 10037401Term: Pulmonary hypertensionsSystem Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]

• Registration Number: EUCTR2014-003423-21-BE
• Lead Sponsor: Bellerophon Pulse Technologies LLC

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2014-08-29
• Last Update Posted: 10 July 2017

Recruitment & Eligibility

• Status: ot Recruiting
• Sex: All
• Target Recruitment: Not specified

Inclusion Criteria

Part 1
1. A confirmed diagnosis of COPD by the Global initiative for chronic Obstructive Lung Disease (GOLD) criteria
2. Pulmonary hypertension determined by 1 of the following within the past 12 months
a. A right heart catherization (not obtained within ± 7 days of an exacerbation) with an mPAP = 25 mmHg, or
b. An echocardiogram (not obtained within ± 7 days of an exacerbation) with a TRV = 2.9 meters per second (m/s), or a systolic PAP = 38 mmHg by 2-D echocardiogram.
(Note: a subject with a TRV < 2.9 m/s will meet this inclusion criteria if there is an acceptable mPAP = 25 mmHg determined by right heart catherization)
3. Current or former smokers with at least 10 pack-years of tobacco cigarette smoking history before study entry
4. Age = 40 years, = 80 years
5. A post-bronchodilatory FEV1/FVC < 0.7 and a FEV1 < 60% predicted (values obtained within 6 months prior to screening can be used unless obtained within ± 7 days of an exacerbation; otherwise, the test must be performed during screening)
6. Receiving LTOT for = 3 months and = 10 hours per day as determined by history
7. Females of childbearing potential must have a negative pre-treatment urine pregnancy test
8. Signed informed consent prior to the initiation of any study mandated procedures or assessments
Part 2:
1. Patients will have a diagnosis of IPF as determined by a responsible and experienced Respiratory physician and based on;
• HCRT: usual interstitial pneumonia
• FVC: 50-90% of predicted FVC
2. PH defined as sPAP = 50 mm Hg by echocardiogram
3. Age = 40 years
4. Receiving LTOT for = 3 months
5. Females of childbearing potential must have a negative pre-treatment urine pregnancy test
6. Signed informed consent prior to the initiation of any study mandated procedures or assessments

Are the trial subjects under 18? no
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 5
F.1.3 Elderly (>=65 years) yes
F.1.3.1 Number of subjects for this age range 5

Exclusion Criteria

Part 1:
1. A diagnosis of asthma or other non-COPD respiratory disease, in the opinion of the Investigator
2. Lack of patency of nares upon physical examination
3. Experienced during the last month an exacerbation requiring:
a) start of or increase in systemic oral corticosteroid therapy, and/or
b) hospitalization
4. Left ventricular dysfunction as measured by:
Screening echocardiographic evidence of left ventricular systolic dysfunction (left ventricular ejection fraction [LVEF] < 40%), or
Screening echocardiographic evidence of left ventricular diastolic dysfunction greater than moderate (i.e., > Grade 2), or
Any history of pulmonary capillary wedge pressure (PCWP), left atrial pressure (LAP) or left ventricular end diastolic pressure (LVEDP) > 18 mm Hg as measured during cardiac catheterization within the past 6 months unless documented to have resolved by a subsequent cardiac catheterization
5. Renal impairment (i.e., an estimated GFRMDRD < 30 ml/min/1.73 m2) or history of renal failure using the equation (Levey et al., 2007):
estimated GFRMDRD = 175×Scr-1.154×Age-0.203 ×1.212 (if black) ×0.742 (if female)
where Scr = Standardized serum creatinine
Subjects with possible compromised kidney function (eGFRMDRD between 30 and 60 ml/min/1.73 m2) may be enrolled provided the Radiology Department and Principal Investigator review the medical records of subjects with an eGFRMDRD between 30 and 60 ml/min/1.73 m2 in order to confirm the contrast agent can be safely administered to these subjects and approval by both the Radiology Department and Principal Investigator must be obtained before enrolling these subjects.
6. Known allergy to contrast media.
7. Clinically significant valvular heart disease that may contribute to PH, including mild or greater aortic valvular disease (aortic stenosis or regurgitation) and/or moderate or greater mitral valve disease (mitral stenosis or regurgitation), or status post mitral valve replacement
8. Use within 30 days of screening or current use of approved PH medications such as sildenafil or bosentan (use of Cialis® or Viagra® for erectile dysfunction is permitted)
9. Use of investigational drugs or devices within 30 days prior to enrollment into the study
10. Any underlying medical or psychiatric condition that, in the opinion of the Investigator, makes the subject an unsuitable candidate for the study
Part 2:
1. Patients with a current IPF exacerbation or exacerbation within the past 30 days.
2. Lack of patency of nares upon physical examination
3. Left ventricular dysfunction as measured by:
a) Screening echocardiographic evidence of left ventricular systolic dysfunction (left ventricular ejection fraction [LVEF] < 40%), or
b) Screening echocardiographic evidence of left ventricular diastolic dysfunction > moderate (i.e., > Grade 3), or
c) Any history of pulmonary capillary wedge pressure (PCWP), left atrial pressure (LAP) or left ventricular end diastolic pressure (LVEDP) > 18 mmHg as measured during cardiac catheterization within the past 6 months unless documented to have resolved by a subsequent cardiac catheterization
4. Renal impairment (i.e., an estimated GFRMDRD < 30 ml/min/1.73 m2) or history of renal failure using the equation (Levey et al., 2007): estimated GFRMDRD = 175 × Scr -1.154 × Age-0.203 × 1.212 (if black) × 0.742 (if female) where Scr = Standardized serum creatinine
Subjects with possible compromised kidney function (eGFRMD

Study & Design

• Study Type: Interventional clinical trial of medicinal product
• Study Design: Not specified