Pulmonary Arterial Hypertension and Connective Tissue Disease

Scope of the Problem CTD-PAH

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

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

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

Active Screening Reveals Patients Earlier

Active Screening Reveals Patients Earlier Who Live Longer

How Best to Screen? The DETECT Study

DETECT Study

Patient Characteristics

Rationale for the 2 Steps of the DETECT Algorithm

Comparison of DETECT 2-Step Algorithm With Current Guidelines

Recommendations Referral for RHC

Screening Key Points

How to Screen This Patient?

Back to the Case…

Recommendations for Patients With WHO FC II PAH: Initial Monotherapy

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

Initial Use of Ambrisentan + Tadalafil in PAH The AMBITION Trial

Initial Combination Therapy vs Pooled Monotherapy

Ambrisentan and Tadalafil Upfront Combination Therapy in SSc-PAH

Results at 36 Weeks

Conclusions

Population in the Initial Dual Oral Combination Trials of SSc Patients

July 16: 6 Months After Treatment Initiated

Reviewing the Case to Date

Sequential Combination Therapy Recommendations for Patients With WHO FC II PAH

GRIPHON Trial Design

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

Effect of Selexipag on Primary Composite Endpoint in GRIPHON

Recommendations for SSc Patients Presenting With Signs/Symptoms of PAH

Complexities of Managing CTD-PAH

Take-Home Points