Volume 3, Issue 1

Revised PH Classifications and New Treatment Developments

In this issue:

The updated (2022) ESC/ERS (European Society of Cardiology/European Respiratory Society) guidelines provide new definitions of pulmonary hypertension (PH) with lowered diagnostic thresholds, as well as revised treatment algorithms based on risk stratification at diagnosis and follow-up. Findings from the multicenter INCREASE study of inhaled treprostinil, and the phase 3 STELLAR trial of sotatercept, provide new evidence for the use of these agents in particular patients with PH. How actionable are the data supporting these changes? How is implementing them expected to improve patient outcomes? And all in all, what do these developments mean to the practicing pulmonologist?

These are some of the questions addressed by Catherine Simpson MD, MHS, and Darin Rosen, MD, from the Division of Pulmonary and Critical Care at Johns Hopkins School of Medicine, in this issue of ePulmonology Review.

Learning objectives:

  • Explain how the revised 2022 ESC/ERS guidelines, including the lowered thresholds indicating PH, may change clinical practice.
  • Describe the current research regarding the expanded indication for inhaled treprostinil for individuals with PH associated with interstitial lung disease.
  • Discuss the current clinical trial evidence in support of sotatercept, a novel fusion protein that alters TGF-beta signaling, as a potential new therapy for PAH.


Catherine E. Simpson, MD, MHS
Catherine E. Simpson, MD, MHS

Assistant Professor of Medicine,
Division of Pulmonary and Critical Care,
Johns Hopkins School of Medicine
Baltimore, Maryland

Darin Rosen, MD
Darin Rosen, MD

Pulmonary Fellow
Johns Hopkins University
Baltimore, Maryland

Program Directors:

Gregory B. Diette, MD

Professor of Medicine, Epidemiology and Environmental Health Sciences
Johns Hopkins University School of Medicine
Baltimore, Maryland

Christine Smith, RN, MSN

Senior Clinic Nurse Coordinator
Johns Hopkins Medicine
Baltimore, Maryland

Length of activity:

1.0 hour Physicians
1.0 contact hour Nurses

Launch date: January 31, 2024
Expiration date: January 30, 2026