Comparison of Inhaled Nitric Oxide and Oxygen in Participants Reactivity During Acute Pulmonary Vasodilator Testing

Study Overview
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Brief Summary
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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.

Official Title
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Comparison of Supplemental Oxygen and Nitric Oxide for Inhalation Plus Oxygen in the Evaluation of the Reactivity of the Pulmonary Vasculature During Acute Pulmonary Vasodilator Testing

Conditions
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Idiopathic Pulmonary Arterial Hypertension, Cardiomyopathy

Intervention / Treatment
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* Drug: Nitric Oxide for inhalation
* Drug: Oxygen
* Drug: Nitric Oxide plus Oxygen


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 4 Weeks
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Allocation: Randomized
Intervention Model: Crossover Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Nitric Oxide First, Oxygen Last<br>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. | Drug: Nitric Oxide for inhalation<br>* Nitric Oxide (NO) for inhalation, given at 80ppm over 10 minutes using an INOvent® delivery system<br>* Other names: INOmax®;Drug: Oxygen<br>* 100% oxygen (O2) for inhalation, given at 80ppm over 10 minutes using an INOvent® delivery system<br>Drug: Nitric Oxide plus Oxygen<br>* Nitric Oxide (NO) for inhalation plus oxygen, given at 80ppm over 10 minutes using an INOvent® delivery system<br>* Other names: Inhaled Nitric Oxide, Oxygen;|
| Experimental: Oxygen First, Nitric Oxide Last<br>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. | Drug: Nitric Oxide for inhalation<br>* Nitric Oxide (NO) for inhalation, given at 80ppm over 10 minutes using an INOvent® delivery system<br>* Other names: INOmax®;Drug: Oxygen<br>* 100% oxygen (O2) for inhalation, given at 80ppm over 10 minutes using an INOvent® delivery system<br>Drug: Nitric Oxide plus Oxygen<br>* Nitric Oxide (NO) for inhalation plus oxygen, given at 80ppm over 10 minutes using an INOvent® delivery system<br>* Other names: Inhaled Nitric Oxide, Oxygen;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of Participants With Reversible Pulmonary Hypertension (Vasoreactivity) | A composite of hemodynamic measurements were used to identify reversible pulmonary hypertension (vasoreactivity) | on Day 1 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of Participants With Related Surgical Procedures Within 1 Year | Number of participants who received surgery for pulmonary or cardiac disease within 1 year after treatment. | within 1 year | 
| Number of Participants With Adverse Events (AEs) | An AE was defined as any untoward medical occurrence. An AE need not have a causal relationship with treatment and included any event that was not seen at baseline or, if present at baseline, increased in severity. | on Day 1 | 
| Number of Participants With Serious Adverse Events (SAEs) | SAEs were collected during the 12 hours after discontinuation of gas or discharge (whichever came first). An SAE was defined as any event that resulted in death, was life threatening, resulted in permanent disability or incapacity, required or prolonged inpatient hospitalization, or was a congenital anomaly. Important medical events that, without medical or surgical intervention, would also have resulted in one of the outcomes listed above were also considered as SAEs. | within 12 hours | 
| Number of Participants With Related Surgical Procedures Within 3 Years | Number of participants who received surgery related to pulmonary or cardiac disease within 3 years | within 3 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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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