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

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

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.

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.