Oral Prostacyclin Pathway Agents in PAH

Background

Key Pathways Involved in Pathogenesis of PAH

Pharmacology of Pathways Involved in Pathogenesis of PAH

Therapies Targeting the Prostacyclin Pathway

Treatment Strategy Options

Combination Pharmacotherapy in PAH Rationale and Potential Clinical Benefits

Monotherapy Recommendations for PAH

Recommendations for Initial Combination Therapy

Recommendations for Treatment Escalation in Patient on Background ERA + PDE5 Inhibitor

GRIPHON Study Design

Selexipag GRIPHON Study Titration and Dosing

GRIPHON Primary Endpoint

GRIPHON Treatment Effect by Subgroup

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

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

GRIPHON Connective Tissue Disease-Associated PAH

GRIPHON Temporary Treatment Interruptions

FREEDOM Trials Oral Treprostinil

Unresolved Issues With Using Oral vs Parenteral Routes

Patient Case

Multidisciplinary Approach to PAH Management

Abbreviations

Abbreviations (cont)