Effect of iNO on Invasively Derived Pulmonary Pressures in Patients With PAH

Study Overview
=================
Brief Summary
-----------------
Pulmonary hypertension is characterized by an increase in the pressures in the blood supply to the lungs greater than a mean pressure of 25mmHg and a concomitant increase in overall pulmonary vascular resistance (PVR). In patients who have remodeling of their pulmonary vasculature, PVR will increase with exercise instead of decreasing as it would in normal patients. Based on published evidence, the investigators intend to investigate the effects of inhaled nitric oxide (iNO) on patients undergoing standard exercise techniques who have separately and previously had an implanted pulmonary artery monitoring device (CardioMems by St Jude Medical, Inc.) placed.

Official Title
-----------------
Effects of iNO on Invasively Derived Pulmonary Vascular Parameters in Patients With Pulmonary Arterial Hypertension

Conditions
-----------------
Pulmonary Arterial Hypertension

Intervention / Treatment
-----------------
* Drug: Nitric Oxide


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Males or females age 18 to 80 years with a diagnosis of pulmonary arterial hypertension (WHO Group I pulmonary hypertension). Must be able to read and understand English and consent for themselves Previously implanted CardioMems continuous PA pressure Ambulatory and able to complete 6MWD test. Exclusion Criteria: Pregnant or lactating females; negative pregnancy test as confirmed by evaluation of data from pre-enrolled Risk Evaluation and Mitigation Strategies (REMS) program.

Ages Eligible for Study
-----------------
Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Intervention<br>Determination of non-invasive pulmonary pressures by CardioMems device interrogation pre- and post-administration of inhaled nitric oxide at 0.075 mg/kg IBW/hr | Drug: Nitric Oxide<br>* Ambulatory inhaled nitric oxide delivery system and 6 minute walk distance test<br>* Other names: iNOPulse DS;|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Difference in Ambulatory 6 Minute Walk Distance (6MWD) Pulmonary Artery (PA) Pressures (Systolic, Diastolic, Mean) Between the Post-intervention (iNO+) and Pre-intervention (iNO-) Condition as Measured by the CardioMems Device | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in CardioMems Cardiac Output | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 
| Change in 6 Minute Walk Distance (6MWD) | Difference (in distance walked between 2 set points over 6 minutes) at baseline (t=0) to post-intervention (t=2h). | 2 hours | 
| Change in O2 Saturations |  | 2 hours | 
| Change in Modified Borg Dyspnea Scale | The Modified Borg Dyspnea scale measures patient's subjective analysis of dyspnea from 0 (nothing at all) to 10 (maximal) during the 6MWD test with the highest value recorded and difference calculated between baseline (t=0) and post-intervention (t=2h). | 2 hours | 
| Change in O2 Requirements | Number of patients that required more or less oxygen by nasal prongs during the course of the study. | 2 hours | 
| Change in Mean Arterial Pressure | Difference between baseline (t=0) and post-intervention (t=2h). | 2 hours | 
| Change in Heart Rate | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 
| Change in Rate Pressure Product | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 
| Change in Right Ventricular (RV) Power | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 
| Change in Stroke Volume | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 
| Change in RV Stroke Work Index | Difference between baseline (t=0) and post-intervention (t=2h) | 2 hours | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Exercise, Inhaled nitric oxide, 6 minute walk test, CardioMems