Pilot Study Evaluating the Safety and Performance of the GeNO NITROsyl Delivery System for Inhaled Nitric Oxide
Condition: Pulmonary Arterial Hypertension
MeSH terms: Pulmonary Arterial Hypertension
Intervention: Nitric Oxide generated by the GeNO nitrosyl delivery system
Intervention description: single short-term exposure to inhaled nitric oxide using the GeNO nitrosyl delivery system.
Intervention MeSH terms: Nitric Oxide
Phase: Phase 2
Study type: Interventional
Primary purpose: Diagnostic
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 10.0
Sex: All
Minimum age: 18 Years
Healthy volunteers: No
Keywords: Diagnostic Techniques and Procedures

Summary:

      This is an open label phase 2 pilot study designed to evaluate the safety, tolerability and
      device performance of the GeNO nitrosyl delivery system during right heart catheterization
      (RHC) in participants with pulmonary arterial hypertension (PAH). All participants will
      receive inhaled nitric oxide in oxygen or nitric oxide in air delivered by nasal cannula.
      Hemodynamics, clinical laboratory and clinical assessment data will be collected on all
      participants to evaluate safety.
    

Eligibility criteria:

        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.