Comparison of Inhaled Nitric Oxide and Oxygen in Patient Reactivity During Acute Pulmonary Vasodilator Testing

• Reversible Pulmonary Hypertension (Vasoreactivity)as Defined by Hemodynamic Measurements [ Time Frame: 1 year ] [ Designated as safety issue: No ]
  Hemodynamic measurements (heart rate, systolic arterial blood pressure,diastolic arterial blood pressure, mean arterial pressure, mean central venous pressure, systolic pulmonary arterial pressure, diastolic pulmonary arterial pressure, mean pulmonary wedge pressure and cardiac output) were used to measure reversible pulmonary hypertension (vasoreactivity).
  
  

• Surgical Procedures at 1 Year [ Time Frame: 1 year after treatment ] [ Designated as safety issue: No ]
  The 1 year follow-up survival assessment consisted of a telephone call to subjects to obtain information on surgeries received pertaining to pulmonary or cardiac disease
  
  
• Adverse Events [ Time Frame: treatment 1 through treatment 3 ] [ Designated as safety issue: Yes ]
  
• Serious Adverse Events [ Time Frame: 12 hours after discontinuation of gas or dischange (whichever comes first) ] [ Designated as safety issue: Yes ]
  
• Surgical Procedures at 3 Years [ Time Frame: 3 years after treatment ] [ Designated as safety issue: No ]
  The 3 year follow-up survival assessment consisted of a telephone call to subjects to obtain information on surgeries received pertaining to pulmonary or cardiac disease
  
  

This is an open, randomized, prospective, multi-center study designed to demonstrate which diagnostic treatment is most capable of identifying patients with a reactive pulmonary vascular bed. A minimum of 100 patients will be enrolled in the study to compare the number of patients with reversible pulmonary hypertension (vasoreactivity) due to nitric oxide for inhalation and oxygen as compared to 100% oxygen. This primary objective will be obtained by measuring the decrease in mean pulmonary arterial pressure (PAPm) ≥ 20% and no decrease in cardiac index (within 5%) in patients with Idiopathic Pulmonary Arterial Hypertension (IPAH) or Congenital Heart Disease (CHD) who do not have an unrestricted shunt at the level of the ventricle or ductus arteriosis, and by measuring the decrease in PAPm ≥ 20% and no decrease in cardiac index (within 5%) and decrease in pulmonary vascular resistance index (PVRI) ≥ 25% and no decrease in cardiac index (within 5%) in patients with cardiomyopathy or CHD who have unrestricted shunt at the level of the ventricle or ductus arteriosis. Additionally, comparison of the incidence and types of drug related and serious adverse events as well as the number of patients with reversible pulmonary hypertension due to nitric oxide for inhalation alone compared to 100% oxygen and to oxygen with nitric oxide for inhalation will also be assessed.