Closed for lack of enrollment

The purpose of this program is to evaluate the logistic issues and patient requirements for chronic pulsed INOmax delivery in ambulatory, home-care patients. To understand patient needs, patients with a variety of underlying diseases will be included. Safety of chronic therapy will be monitored by serial measurements of methemoglobin, platelet function assay and reported adverse events.

This study will help develop a comprehensive home care program using INOmax delivery systems. This study will enroll a limited number of patients (5-7) from several disease categories in order to develop the experience with testing acute and chronic responsiveness to INO therapy. The following groups will be tested:

1. Primary pulmonary hypertension (PPH)
2. Severe pulmonary hypertension due to congenital (anatomic) heart disease
3. Pulmonary hypertension or severe impairment of gas exchange in infants and children with chronic lung disease
4. Pulmonary hypertension or severe impairment of gas exchange due to severe chronic lung disease in adults
5. Pulmonary hypertension or severe impairment of gas exchange in hematologic and transplant disorders - young children and adults with sickle cell disease, chronic lung disease after bone marrow transplant, cardiac transplant, or lung transplant

Inclusion criteria:

• Patients with pulmonary arterial hypertension (PAH) as defines by a mean pulmonary artery pressure (PAP) of 25 mmHg or greater by cardiac catheterization within the previous 12 months AND/OR Patients with chronic lung disease with severe impairment of gas exchange unresponsive to conventional medical management.
• If patient is receiving prostacyclin infusion treatment it must have been started at least one month prior to starting INO therapy. Patients will not be withdrawn from the study if started on other medications for treatment of pulmonary hypertension.
• Ability to establish a clinical investigator and a drug shipment site in the local area.

Exclusion Criteria:

• The Principal Investigator has determined that the patient and/or caregiver is/are unable to manage safe administration of home nitric oxide therapy and maintain appropriate drug accountability and storage.
• Women who are pregnant or nursing.