TReatment Of Pulmonary HYpertension 1 Study

Inclusion Criteria

• •Patient with known pulmonary arterial hypertension (PAH), which has been diagnosed as idiopathic PAH, connective tissue disease PAH, anorexigen induced or familial PAH
• •PAH diagnosis confirmed by hemodynamic evaluation performed prior to screening and showing all of the following: Mean pulmonary artery pressure (mPAP) ≥25 mmHg at rest; Pulmonary capillary wedge pressure (PCWP) or left ventricular end diastolic pressure (LVEDP) ≤15 mmHg; Pulmonary vascular resistance (PVR) at rest \>3 Wood units; Not meeting the criteria for a positive vasodilator response (fall in mPAP ≥ 10 mmHg to ≤ 40 mmHg).
• •Patient with a current diagnosis of WHO functional class III
• •Patient should be on two pulmonary arterial hypertension specific medications other than parenteral prostanoids
• •Patient is adhering to a stable drug regimen (i.e., with no changes of dose or medication for a minimum of 3 months prior to enrollment)
• •Patient with eGFR levels of ≥ 30 ml/min/1.73m2or serum creatinine levels of ˂ 150µmol/l