Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan

Exclusion Criteria

• •1. Resting PAH (mPAP \> 25mmHg) on right heart catheterization
• •2. Other known causes of PAH including prior venous thromboembolism, HIV infection, chronic liver disease with portal hypertension, left ventricular systolic dysfunction (e.g. LVEF \< 40%), and congenital causes of PAH
• •3. Severe hepatic disease precluding the use of ambrisentan (AST/ALT ≥3x ULN).
• •4. Women who are pregnant or breastfeeding.
• •5. Concurrent therapy with a prostanoid or prostanoid analogue, PDE5 inhibitors, or enrolled in another active clinical study.
• •6. Use of any prostacyclin or endothelial receptor antagonist (ERA) within 30 days before study entry.
• •7. Bed or wheel chair bound or a baseline 6-Minute Walk distance (6MWD) less than 150 meters.
• •8. Childbearing capable women who are unwilling or unable to use an acceptable method to avoid pregnancy for the entire study period.
• •9. New York Heart Association (NYHA) Classification: Class IV
• •10. Renal dysfunction (serum creatinine \>2.5mg/dL).
• •11. Uncontrolled sleep apnea.