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.
- Singh, N.
- Dorfmüller, P.
- Shlobin, O. A.
- Ventetuolo, C. E.
Keywords
- Humans
- *Hypertension, Pulmonary/drug therapy/epidemiology
- Lung
- *Lung Diseases, Interstitial/complications/drug therapy
- Vasodilator Agents
- *Ventricular Dysfunction, Right/drug therapy
- hypertension, pulmonary
- hypoxia
- lung diseases, interstitial
- prevalence
- ventricular dysfunction, right
