Diagnosis and Pathophysiological Mechanisms of Group 3 Hypoxia-Induced Pulmonary Hypertension

Group 3 hypoxia-induced pulmonary hypertension (PH) is an important and increasingly diagnosed condition in both the pediatric and adult population. The majority of pulmonary hypertension studies to date and all three classes of drug therapies were designed to focus on group 1 PH. There is a clear unmet medical need for understanding the molecular mechanisms of group 3 PH and a need for novel non-invasive methods of assessing PH in neonates.

Several growth factors are expressed in patients and in animal models of group 3 PH and are thought to contribute to the pathophysiology of this disease. Here, we review some of the findings on the roles of vascular endothelial growth factor A (VEGFA), platelet-derived growth factor B (PDGFB), transforming growth factor-beta (TGFB1), and fibroblast growth factors (FGF) in PH. Additionally, we discuss novel uses of echocardiographic parameters in assessing right ventricular form and function. FGF2, TGFB, PDGFB, and VEGFA may serve as biomarkers in group 3 PH along with echocardiographic methods to diagnose and follow right ventricle function. FGFs and VEGFs may also function in the pathophysiology of group 3 PH.