Comparison of Inhaled Nitric Oxide and Oxygen in Participants Reactivity During Acute Pulmonary Vasodilator Testing
Idiopathic Pulmonary Arterial Hypertension, Cardiomyopathy
About this trial
This is an interventional diagnostic trial for Idiopathic Pulmonary Arterial Hypertension focused on measuring 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
Eligibility Criteria
Inclusion Criteria:
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
- Scheduled to undergo right heart catheterization to assess pulmonary vasoreactivity by acute pulmonary vasodilation testing.
- Male or female, ages 4 weeks to 18 years, inclusive
- Signed informed consent/assent
Exclusion Criteria:
- Focal pulmonary infiltrates on chest radiograph.
- Diagnosed with severe obstructive or restrictive pulmonary disease that is significantly contributing to the patient's pulmonary hypertension.
- 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
- Pregnant [urine human chorionic gonadotropin positive (HCG +)]
- Baseline Pulmonary capillary wedge pressure (PCWP) > 20 mmHg
Sites / Locations
- Lucile Salter Packard Children's Hospital at Stanford
- The Children's Hospital
- Children's Hospital Boston
- St. Louis Children's Hospital
- New York Presbyterian Hospital
- Cincinnati Children's Hospital
- Columbus Children's Hospital
- The Medical University of South Carolina
- CHU Timone - Département de cardiologie
- Hôpital d'Enfants
- Hôpital NECKER - Enfants Malades
- Beatrix Children's Hospital / University Hospital Groningen
- Hospital Sant Joan de Déu de Barcelona
- Unidad de Cardiologia Infantil - Hospital Vall d'Hebrón
- Hospital Gregorio Maranon
- Instituto Pediátrico del Corazón - Hospital Materno Infatil Doce de Octubre
- Royal Brompton Hospital
- Southampton University Hospitals Trust - Wessex Cardiothoracic Centre
Arms of the Study
Arm 1
Arm 2
Experimental
Experimental
Nitric Oxide First, Oxygen Last
Oxygen First, Nitric Oxide Last
10 minute dose of Nitric Oxide (NO) at 80 ppm, then 10 minute dose of NO plus Oxygen, then 10 minute washout, then 10 minute dose of 100% Oxygen on Day 1.
10 minute dose of 100% Oxygen, then 10 minute dose of NO plus Oxygen, then 10 minute washout, then 10 minute dose of NO at 80 ppm on Day 1.