Updates in Pulmonary Arterial Hypertension

Case Study: 37-Year-Old Man

Case Study: Diagnostics

Diagnostics (cont): Echocardiogram

Case Study: Tests to Rule Out Secondary Cause

Case Study: Noninvasive Summary

Case Study: Hemodynamics

Case Study: Risk Assessment

Case Study: Summary

Background

Determinants of Prognosis in PAH

Treatment Strategy Options

Monotherapy Recommendations for PAH

Recommendations for Initial Combination Therapy

Initial Use of Ambrisentan + Tadalafil in PAH The AMBITION Trial

AMBITION Study Primary Endpoint* Results

Actual Treatment Plan

Why Not Simply “Stay the Course?”

French Registry

French Registry (cont)

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

French Registry BNP Subset Analysis

COMPERA Registry

SWEDISH Register (SPAHR)

Comprehensive Risk Stratification at Early Follow-Up Determines Prognosis

Summary of 3 European Studies

Sequential Combination Therapy Recommendations for WHO FC III PAH

Key Pathways Involved in Pathogenesis of PAH

Classes of Agents That Target Different Pathways in PAH

The PGI2 Pathway

Therapies Targeting the Prostacyclin Pathway

TRIUMPH Inhaled Treprostinil Added to Oral Monotherapy

FREEDOM Trials Oral Treprostinil

Recommendations for Treatment Escalation in Patient on Background ERA + PDE5I

GRIPHON Study Design

GRIPHON Primary Endpoint

GRIPHON Treatment Effect by Subgroup

Case Follow-Up

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

Combination Pharmacotherapy in PAH Rationale and Potential Clinical Benefits

Take-Home Points

Abbreviations

Abbreviations (cont)

Abbreviations (cont)