Pilot Study Evaluating the Safety and Performance of the GeNO NITROsyl Delivery System for Inhaled Nitric Oxide

TREATMENT/FOLLOW-UP:

Participants meeting eligibility criteria will receive open label nitric oxide at 80 ppm via a nasal cannula. Hemodynamic clinical laboratory and clinical assessment data will be collected at baseline, after 15 minutes of inhaled nitric oxide administration, post RHC procedure and at hospital discharge. Day 5 +/- 3 post RHC, telephone contact to assess general health status.

Inclusion criteria

• Have a confirmed diagnosis of PAH, WHO Group 1.
• WHO Functional Class II or III equivalent, PAH.
• Have been clinically stable with regard to signs and symptoms of PAH for at least 30 days prior to RHC.
• May be receiving approved mono therapies or combination PAH therapies.
• Females that are surgically sterile or post-menopausal. Females of chil-bearing potential must have negative pregnancy test and must be practicing adequate birth control.

Exclusion criteria

• Have had a new type of chronic therapy (including but not limited to oxygen, a different category of vasodilator, a diuretic, digoxin) for PAH added within (1) month of RHC.
• Have any PAH medication except for anticoagulants discontinued within the week prior to RHC.
• Have evidence of significant parenchymal lung disease as evidenced by pulmonary function tests within the last six months
• CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) syndrome
• Have a history of uncontrolled sleep apnea within three months of RHC.
• Have a history of hemodynamically significant left-sided heart disease
• Have evidence of left-sided heart disease
• Have any other disease that is associated with pulmonary hypertension (e.g. congenital systemic-to-pulmonary shunt, sickle cell anemia, schistosomiasis).
• Documented uncontrolled systemic hypertension as evidenced by systolic blood pressure greater than 160 mmHg or diastolic blood pressure greater than 100 mmHg.
• Have used prescription appetite suppressants within 3 months prior to wean/transition.
• Have chronic kidney disease stage IV or worse or the requirement for dialysis.
• Be receiving an investigational drug, have in place an investigational device, or have participated in an investigational drug study within the past 30 days.
• Have had an atrial septostomy.
• Have anemia (hemoglobin <10 g/dL), active infection or any other ongoing condition that would interfere with the interpretation of study assessments.
• Have any serious or life-threatening disease other than conditions associated with PAH (e.g. malignancy requiring aggressive chemotherapy, renal dialysis, etc.).
• Have unstable psychiatric status or be mentally incapable of understanding the objectives, nature or consequences of the trial
• Participant is pregnant or lactating
• Significant, ongoing alcohol or drug abuse.