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

Phase 2

Completed

Lead Sponsor: Allegheny Singer Research Institute (also known as Allegheny Health Network Research Institute)

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.

Recruitment & Eligibility

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.

Arm && Interventions

Group	Intervention	Description
Intervention	Nitric Oxide	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

Primary Outcome Measures

Name	Time	Method
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	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)

Secondary Outcome Measures

Name	Time	Method
Change in Stroke Volume	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)
Change in O2 Requirements	2 hours	Number of patients that required more or less oxygen by nasal prongs during the course of the study.
Change in CardioMems Cardiac Output	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)
Change in 6 Minute Walk Distance (6MWD)	2 hours	Difference (in distance walked between 2 set points over 6 minutes) at baseline (t=0) to post-intervention (t=2h).
Change in O2 Saturations	2 hours
Change in Modified Borg Dyspnea Scale	2 hours	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).
Change in RV Stroke Work Index	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)
Change in Mean Arterial Pressure	2 hours	Difference between baseline (t=0) and post-intervention (t=2h).
Change in Heart Rate	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)
Change in Rate Pressure Product	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)
Change in Right Ventricular (RV) Power	2 hours	Difference between baseline (t=0) and post-intervention (t=2h)