Woo Kel Vin, Ornitz David M, Singh Gautam K
Department of Pediatrics, Washington University School of Medicine, One Children's place, Campus Box 8116-NWT, St. Louis, MO, 63132, USA.
Department of Developmental Biology, Washington University School of Medicine, Saint Louis, MO, USA.
Curr Treat Options Cardiovasc Med. 2019 Mar 22;21(3):16. doi: 10.1007/s11936-019-0718-3.
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.
3型缺氧诱导的肺动脉高压（PH）在儿科和成人人群中都是一种重要且诊断率日益增加的病症。迄今为止，大多数肺动脉高压研究以及所有三类药物疗法都旨在聚焦于1型PH。对于了解3型PH的分子机制以及新生儿非侵入性评估PH的新方法，存在明确未满足的医疗需求。
几种生长因子在3型PH患者和动物模型中表达，并且被认为对该疾病的病理生理学有贡献。在此，我们综述一些关于血管内皮生长因子A（VEGFA）、血小板衍生生长因子B（PDGFB）、转化生长因子-β（TGFB1）和成纤维细胞生长因子（FGF）在PH中作用的发现。此外，我们讨论超声心动图参数在评估右心室形态和功能方面的新用途。FGF2、TGFB、PDGFB和VEGFA以及超声心动图方法可作为3型PH的生物标志物，用于诊断和跟踪右心室功能。FGF和VEGF也可能在3型PH的病理生理学中发挥作用。
