1. Updates in Pulmonary Arterial Hypertension

3. Case Study: 37-Year-Old Man

4. Case Study: Diagnostics

5. Diagnostics (cont): Echocardiogram

6. Case Study: Tests to Rule Out Secondary Cause

7. Case Study: Noninvasive Summary

8. Case Study: Hemodynamics

9. Case Study: Risk Assessment

10. Case Study: Summary

11. Background

12. Determinants of Prognosis in PAH

13. Treatment Strategy Options

14. Monotherapy Recommendations for PAH

15. Recommendations for Initial Combination Therapy

16. Initial Use of Ambrisentan + Tadalafil in PAH The AMBITION Trial

17. AMBITION Study Primary Endpoint* Results

18. Actual Treatment Plan

19. Why Not Simply “Stay the Course?”

20. French Registry

21. French Registry (cont)

22. French Registry: Survival Based on Number of Low-Risk Criteria Achieved at First Evaluation

23. French Registry BNP Subset Analysis

24. COMPERA Registry

25. SWEDISH Register (SPAHR)

26. Comprehensive Risk Stratification at Early Follow-Up Determines Prognosis

27. Summary of 3 European Studies

28. Sequential Combination Therapy Recommendations for WHO FC III PAH

29. Key Pathways Involved in Pathogenesis of PAH

30. Classes of Agents That Target Different Pathways in PAH

31. The PGI2 Pathway

32. Therapies Targeting the Prostacyclin Pathway

33. TRIUMPH Inhaled Treprostinil Added to Oral Monotherapy

34. FREEDOM Trials Oral Treprostinil

35. Recommendations for Treatment Escalation in Patient on Background ERA + PDE5I

36. GRIPHON Study Design

37. GRIPHON Primary Endpoint

38. GRIPHON Treatment Effect by Subgroup

39. Case Follow-Up

40. Primary Composite Endpoint in Patients Grouped by Prespecified Selexipag Individual Maintenance Dose vs Placebo

41. Combination Pharmacotherapy in PAH Rationale and Potential Clinical Benefits

42. Take-Home Points

43. Abbreviations

44. Abbreviations (cont)

45. Abbreviations (cont)