PAH Clinical Scenarios: Advances in Diagnostic Assessment, Guideline Recommended Care, and Emerging Treatments – Clinical Brief #2

Current Recommendations for PAH Treatment


Pulmonary arterial hypertension (PAH) is a rare, life-threatening disorder characterized by elevated pressure in the pulmonary arteries that quickly progresses to right ventricular failure and premature death. Expert consensus recommends early diagnosis and immediate treatment to improve survival in this mostly younger adult patient population. In addition to knowing how to recognize and diagnose PAH, physicians must also be able to initiate appropriate therapeutic strategies (generally combination therapies using multiple drug classes) and be aware of emerging treatment modalities to optimize outcomes.

In 2022, updates to the European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines included recommending combination therapy based on World Health Organization Functional Class (WHO-FC) and other factors, outlined below, in making decisions about treating PAH.

In treating patients with PAH, most should be initiated on an endothelin receptor antagonist (ERA) and phosphodiesterase-5 inhibitor (PDE-5i) therapy and some might need triple therapy.

Listen to insights from experts Dr. John Ryan and Dr. Vallerie McLaughlin about the decision-making process for treating patients with PAH.