1. Date of download: 7/9/2016 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary Arterial Hypertension J Am Coll Cardiol. 2008;51(16):1527-1538. doi:10.1016/j.jacc.2008.01.024 Survival Rates Among Patients With CHF With Systolic Dysfunction Survival rates without urgent heart transplantation among patients with congestive heart failure (CHF) and left ventricular ejection fraction less than 35% are lower among patients with high pulmonary arterial pressures (PAPs) and low right ventricular ejection fraction (RVEF). Group 1: normal PAP/preserved RVEF (n = 73). Group 2: normal PAP/low RVEF (n = 68). Group 3: high PAP/preserved RVEF (n = 21). Group 4: high PAP/low RVEF (n = 215). Reproduced, with permission, from Ghio et al. (29). Figure Legend:

2. Date of download: 7/9/2016 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary Arterial Hypertension J Am Coll Cardiol. 2008;51(16):1527-1538. doi:10.1016/j.jacc.2008.01.024 Treatment Algorithm for PAH Adapted From 2007 ACCP Guidelines Letters following recommendations are based on a combination of level of evidence and perceived benefit: A = strong recommendation; B = moderate recommendation; C = weak recommendation. Recommendations with an E are based on expert opinion rather than clinical trial evidence. Ambrisentan was approved for functional class II and III pulmonary arterial hypertension (PAH) after these guidelines were published. ACCP = American College of Chest Physicians; CCB = calcium-channel blocker; FC = functional class; INH = inhaled; IPAH = idiopathic pulmonary arterial hypertension; IV = intravenous; SC = subcutaneous. Reproduced, with permission, from Badesch et al. (49). Figure Legend:

3. Date of download: 7/9/2016 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary Arterial Hypertension J Am Coll Cardiol. 2008;51(16):1527-1538. doi:10.1016/j.jacc.2008.01.024 Survival in Patients With Idiopathic PAH After Treatment With Epoprostenol Among patients who were FC III or IV prior to treatment with epoprostenol, survival among patients improving to FC I or II (solid line) was substantially better than patients who were FC III or IV (dashed line) after treatment; p < 0.001. NYHA = New York Heart Association; other abbreviations as in Figure 2. Reproduced, with permission, from Sitbon et al. (63). Figure Legend:

4. Date of download: 7/9/2016 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary Arterial Hypertension J Am Coll Cardiol. 2008;51(16):1527-1538. doi:10.1016/j.jacc.2008.01.024 Cardiac MRI in Advanced PAH Images of idiopathic PAH in a 32-year-old subject. The “D-shaped” left ventricle (LV) seen in panels A and B is due to elevated pulmonary pressures leading to dilation of the right ventricle (RV). The right atrium (RA) is also markedly dilated. The RV end- diastolic volume is 363 ml, and the RV ejection fraction is 20%. LA = left atrium; MRI = magnetic resonance imaging; other abbreviation as in Figure 2. Figure Legend: