Effects of Pulmonary Hypertension Therapy in Atypical Pulmonary Arterial Hypertension

Other illness

• Significant chronic obstructive pulmonary disease that is a primary contributor to symptoms in the opinion of the investigator
• Complex congenital heart disease
• Intrinsic (prolapse, rheumatic) valve disease with more than moderate mitral, tricuspid or aortic regurgitation
• Terminal illness (other than HF) with expected survival of less than 1 year

Lab values

• Acute or chronic severe liver disease as evidenced by any of the following: encephalopathy, variceal bleeding, INR \> 1.7 in the absence of anticoagulation treatment

Other

• Ischemia thought to contribute to dyspnea in the opinion of the investigator
• Obstructive hypertrophic cardiomyopathy
• Known infiltrative cardiomyopathy (amyloid)
• Constrictive pericarditis or tamponade
• Active myocarditis
• More than mild aortic or mitral stenosis
• Enrollment or planned enrollment in another therapeutic clinical trial in next 3 months.
• Inability to comply with planned study procedures
• Pregnancy or breastfeeding mothers

• Age ≥ 18 years
• Pulmonary hypertension with mean PA pressure \>20 mmHg and a planned initiation of pulmonary arterial hypertension therapy
• No active treatment for precapillary pulmonary hypertension
• Ambulatory (not wheelchair/scooter dependent)
• Presence of any risk factor for left heart disease will qualify for inclusion (either atrial fibrillation, body mass index\>30 kg/m2, arterial hypertension, diabetes, coronary artery disease or age\>60 years)