1. Pulmonary Arterial Hypertension and Connective Tissue Disease

3. Scope of the Problem CTD-PAH

4. Etiology of PAH in the United States Nearly 25% of PAH Is CTD-Related

5. Case Study: 72-Year-Old Woman Referred for Consultation Because of Abnormal Echo

6. WHO FC at PAH-SSc Diagnosis "Routine Practice" and "Screened" Patients

7. Active Screening Reveals Patients Earlier

8. Active Screening Reveals Patients Earlier Who Live Longer

9. How Best to Screen? The DETECT Study

10. DETECT Study

11. Patient Characteristics

12. Rationale for the 2 Steps of the DETECT Algorithm

13. Comparison of DETECT 2-Step Algorithm With Current Guidelines

14. Recommendations Referral for RHC

15. Screening Key Points

16. How to Screen This Patient?

17. Back to the Case…

18. Recommendations for Patients With WHO FC II PAH: Initial Monotherapy

19. Recommendations for Patients With WHO FC II PAH: Initial Combination Therapy

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

21. Initial Combination Therapy vs Pooled Monotherapy

22. Ambrisentan and Tadalafil Upfront Combination Therapy in SSc-PAH

23. Results at 36 Weeks

24. Conclusions

25. Population in the Initial Dual Oral Combination Trials of SSc Patients

26. July 16: 6 Months After Treatment Initiated

27. Reviewing the Case to Date

28. Sequential Combination Therapy Recommendations for Patients With WHO FC II PAH

29. GRIPHON Trial Design

30. Selexipag Delays Time to Clinical Worsening in Patients With CTD-Associated PAH

31. Effect of Selexipag on Primary Composite Endpoint in GRIPHON

32. Recommendations for SSc Patients Presenting With Signs/Symptoms of PAH

33. Complexities of Managing CTD-PAH

34. Take-Home Points