Group 3 Pulmonary Hypertension: From Bench to Bedside

• Published in: Circulation research Vol. 130; no. 9; pp. 1404 - 1422
• Main Authors: Singh, Navneet, Dorfmüller, Peter, Shlobin, Oksana A., Ventetuolo, Corey E.
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 29.04.2022
• Subjects: Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - epidemiology; Lung; Lung Diseases, Interstitial - complications; Lung Diseases, Interstitial - drug therapy; Vasodilator Agents; Ventricular Dysfunction, Right - drug therapy; hypertension, pulmonary; lung diseases, interstitial; ventricular dysfunction, right; hypoxia; prevalence
• ISSN: 0009-7330; 1524-4571; 1524-4571
• DOI: 10.1161/CIRCRESAHA.121.319970

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.