Pulmonary Hypertension Research Group, Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Québec, QC G1V 4G5, Canada.
Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Cells. 2021 Jun 11;10(6):1473. doi: 10.3390/cells10061473.
Pulmonary arterial hypertension is a debilitating chronic disorder marked by the progressive obliteration of the pre-capillary arterioles. This imposes a pressure overload on the right ventricle (RV) pushing the latter to undergo structural and mechanical adaptations that inexorably culminate in RV failure and death. Thanks to the advances in molecular biology, it has been proposed that some aspects of the RV and pulmonary vascular remodeling processes are orchestrated by a subversion of developmental regulatory mechanisms with an upregulation of a suite of genes responsible for the embryo's early growth and normally repressed in adults. In this review, we present relevant background regarding the close relationship between overactivation of fetal genes and cardiopulmonary remodeling, exploring whether the reawakening of developmental factors plays a causative role or constitutes a protective mechanism in the setting of PAH.
肺动脉高压是一种使人虚弱的慢性疾病，其特征是毛细血管前小动脉的进行性闭塞。这会给右心室（RV）造成压力负荷，迫使后者进行结构和机械适应，最终不可避免地导致 RV 衰竭和死亡。由于分子生物学的进步，有人提出 RV 和肺血管重塑过程的某些方面是由发育调节机制的颠覆来协调的，其中上调了一整套负责胚胎早期生长的基因，而这些基因在成人中通常受到抑制。在这篇综述中，我们介绍了关于胎儿基因过度激活与心肺重塑之间密切关系的相关背景，探讨了发育因子的重新激活是否在 PAH 中起因果作用或构成保护机制。
