Comparison of Inhaled Nitric Oxide and Oxygen in Participants Reactivity During Acute Pulmonary Vasodilator Testing
Condition: Idiopathic Pulmonary Arterial Hypertension; Cardiomyopathy
MeSH terms: Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension; Cardiomyopathies
Intervention: Nitric Oxide for inhalation; Oxygen; Nitric Oxide plus Oxygen
Intervention description: Nitric Oxide (NO) for inhalation, given at 80ppm over 10 minutes using an INOvent® delivery system; 100% oxygen (O2) for inhalation, given at 80ppm over 10 minutes using an INOvent® delivery system; Nitric Oxide (NO) for inhalation plus oxygen, given at 80ppm over 10 minutes using an INOvent® delivery system
Intervention MeSH terms: Nitric Oxide
Phase: Phase 3
Study type: Interventional
Primary purpose: Diagnostic
Allocation: Randomized
Intervention model: Crossover Assignment
Masking: None (Open Label)
Enrollment: 136.0
Sex: All
Minimum age: 4 Weeks
Maximum age: 18 Years
Healthy volunteers: No
Keywords: Pulmonary Vasculature; Nitric Oxide; INOmax®; Acute Lung Injury; Pulmonary Vasodilator Testing; Idiopathic Pulmonary Arterial Hypertension; Congenital Heart Disease; reversible pulmonary hypertension; vasoreactivity; Congenital Heart Disease with Pulmonary Hypertension

Summary:

      The primary purpose of this study is to compare the number of participants with reversible
      pulmonary hypertension (vasoreactivity) due to nitric oxide for inhalation and oxygen as
      compared to 100% oxygen.
    

Eligibility criteria:

        Inclusion Criteria:

          1. Must have any one of these three disease categories:

               -  Idiopathic Pulmonary Arterial Hypertension

                    -  Mean pulmonary arterial pressure (PAPm) > 25 millimeters of mercury (mmHg)
                       at rest, pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and PVRI> 3
                       u•m^2 or diagnosed clinically with no previous catheterization

               -  Congenital heart disease (CHD) with pulmonary hypertension repaired and
                  unrepaired

                    -  PAPm > 25 mmHg at rest and PVRI> 3 u•m^2 or diagnosed clinically with no
                       previous catheterization

               -  Cardiomyopathy

                    -  PAPm > 25 mmHg at rest and Pulmonary vascular resistance index (PVRI)> 3
                       u•m^2 or diagnosed clinically with no previous catheterization

          2. Scheduled to undergo right heart catheterization to assess pulmonary vasoreactivity by
             acute pulmonary vasodilation testing.

          3. Male or female, ages 4 weeks to 18 years, inclusive

          4. Signed informed consent/assent

        Exclusion Criteria:

          1. Focal pulmonary infiltrates on chest radiograph.

          2. Diagnosed with severe obstructive or restrictive pulmonary disease that is
             significantly contributing to the patient's pulmonary hypertension.

          3. Received treatment with nitric oxide for inhalation within 30 days prior to study
             initiation, are on other investigational medications, nitroglycerin, sodium
             nitroprusside, sildenafil, other Phosphodiesterase type 5 (PDE-5) inhibitors, or
             prostacyclin

          4. Pregnant [urine human chorionic gonadotropin positive (HCG +)]

          5. Baseline Pulmonary capillary wedge pressure (PCWP) > 20 mmHg