Pulmonary Hypertension: Expert Didactics and Discussion (Master Class)-Understand the Pathophysiology of Pulmonary Arterial Hypertension and Treatment

Video Transcription

Video Summary

The presentation by Jean Elwing from the University of Cincinnati provides an in-depth overview of pulmonary arterial hypertension (PAH), focusing on its complex pathophysiology and evolving treatment algorithms. PAH is characterized by progressive pulmonary vasculopathy involving dysregulation of growth factors, inflammation, genetic predispositions (such as BMPR2 mutations), and vascular remodeling. Despite diverse triggers like HIV or lupus, PAH pathophysiology is similar across patients, allowing a common treatment approach.<br /><br />Diagnosis relies heavily on right heart catheterization and thorough clinical assessment, with updated hemodynamic definitions lowering the mean pulmonary artery pressure threshold to >20 mmHg and PVR >2 Woods units for PAH. Echocardiography is a useful screening tool but often requires further invasive testing.<br /><br />Treatment has evolved from solely vasodilators (targeting endothelin, nitric oxide, prostacyclin pathways) to include novel therapies addressing molecular pathways, such as cetatercept targeting BMPR2 signaling. Current guidelines recommend risk stratification models guiding initial combination therapy and subsequent escalation, including addition of oral prostacyclins or IV therapies in high-risk cases. Early aggressive treatment can improve long-term survival and functional status.<br /><br />Emphasis is placed on comprehensive evaluation, regular risk reassessment, and adapting treatment to achieve low-risk status, improving survival rates markedly compared to historical data. The talk highlights ongoing research and hope for more personalized therapies in PAH management.

Keywords

pulmonary arterial hypertension

PAH pathophysiology

BMPR2 mutations

right heart catheterization

hemodynamic definitions

vasodilator therapies

cetatercept

risk stratification

personalized PAH treatment