Clapham Katharine R, Singh Inderjit, Capuano Isabella S, Rajagopal Sudarshan, Chun Hyung J
Section of Cardiovascular Medicine, Department of Internal Medicine, Yale Cardiovascular Research Center, Yale School of Medicine, New Haven, CT, United States.
Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, United States.
Front Cardiovasc Med. 2019 Mar 28;6:29. doi: 10.3389/fcvm.2019.00029. eCollection 2019.
Pulmonary arterial hypertension is a progressive and ultimately life-limiting disease in which survival is closely linked to right ventricular function. The right ventricle remains relatively understudied, as it is known to have key developmental and structural differences from the left ventricle. Here, we will highlight what is known about the right ventricle in normal physiology and in the disease state of pulmonary arterial hypertension. Specifically, we will explore the role of the family of MEF2 (myocyte enhancer factor 2) transcription factors in right ventricular development, its response to increased afterload, and in the endothelial dysfunction that characterizes pulmonary arterial hypertension. Finally, we will turn to review potentially novel therapeutic strategies targeting these pathways.
肺动脉高压是一种进行性且最终会危及生命的疾病，其生存率与右心室功能密切相关。右心室相对较少受到研究，因为已知它在发育和结构上与左心室存在关键差异。在此，我们将重点阐述正常生理状态以及肺动脉高压疾病状态下右心室的相关知识。具体而言，我们将探讨肌细胞增强因子2（MEF2）转录因子家族在右心室发育中的作用、其对后负荷增加的反应以及在肺动脉高压所特有的内皮功能障碍中的作用。最后，我们将转而综述针对这些途径的潜在新型治疗策略。
