Pulmonary Hypertension: Expert Didactics and Discussion (Master Class)-How to Diagnose and Treat Pulmonary Hypertension complicating Lung Disease

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Video Summary

The presentation provides an in-depth overview of pulmonary hypertension (PH) associated with lung diseases (Group 3 PH), particularly in COPD, interstitial lung disease (ILD), and other related conditions. PH in lung disease is common, especially mild forms, and significantly worsens survival regardless of lung disease severity. Diagnosis is challenging since symptoms like dyspnea overlap with lung disease symptoms; echocardiography and DLCO tests help but are imperfect, and right heart catheterization remains the gold standard.<br /><br />Treatment decisions depend on distinguishing whether symptoms arise mainly from vascular disease or lung fibrosis. Vasodilators like sildenafil have mixed results, often improving hemodynamics but not function due to ventilation-perfusion mismatching. Inhaled treprostinil shows promise by targeting well-ventilated lung areas, improving 6-minute walk distance and reducing clinical worsening in ILD-PH, but with side effects like cough. COPD patients generally see limited benefit from PH-targeted therapies, except a subgroup with severe PH and less lung damage.<br /><br />Management emphasizes optimizing underlying lung disease, treating comorbidities (especially sleep apnea), supplemental oxygen, pulmonary rehab, and early transplant evaluation. Future directions include better phenotyping patients, refining risk criteria, combining antifibrotic and vasodilator therapies, and exploring new agents targeting common fibrotic and vascular pathways. Overall, personalized treatment balancing disease burden and therapy side effects is crucial.

Keywords

pulmonary hypertension

PH

Group 3 PH

COPD

Chronic Obstructive Pulmonary Disease

interstitial lung disease

diagnosis challenges

vasodilator therapy

inhaled treprostinil

personalized treatment