Journal
CIRCULATION RESEARCH
Volume 130, Issue 9, Pages 1404-1422Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1161/CIRCRESAHA.121.319970
Keywords
hypertension; pulmonary; hypoxia; lung diseases; interstitial; prevalence; ventricular dysfunction; right
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Pulmonary hypertension (PH) due to chronic lung disease is a common type of PH that requires effective treatment options. The recent approval of a therapy for pulmonary arterial hypertension related to interstitial lung disease is a positive advancement. This review examines the molecular mechanisms contributing to pulmonary vasculopathy in chronic hypoxia, the pathology and epidemiology of Group 3 PH, right ventricular dysfunction in this population, and clinical trial data on the use of pulmonary vasodilators in Group 3 PH.
Pulmonary hypertension (PH) because of chronic lung disease is categorized as Group 3 PH in the most recent classification system. Prevalence of these diseases is increasing over time, creating a growing need for effective therapeutic options. Recent approval of the first pulmonary arterial hypertension therapy for the treatment of Group 3 PH related to interstitial lung disease represents an encouraging advancement. This review focuses on molecular mechanisms contributing to pulmonary vasculopathy in chronic hypoxia, the pathology and epidemiology of Group 3 PH, the right ventricular dysfunction observed in this population and clinical trial data that inform the use of pulmonary vasodilators in Group 3 PH.
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