Liu Jixiang, Yang Peiran, Tian Han, Zhen Kaiyuan, McCabe Colm, Zhao Lan, Zhai Zhenguo
Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing 100029, China.
National Center for Respiratory Medicine, Beijing 100029, China.
J Transl Int Med. 2022 Jul 2;10(2):125-133. doi: 10.2478/jtim-2022-0027. eCollection 2022 Jun.
Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed, but potentially curable pulmonary vascular disease. The increased pulmonary vascular resistance in CTEPH is caused by unresolved proximal thrombus and secondary microvasculopathy in the pulmonary vasculature, leading to adaptive and maladaptive remodeling of the right ventricle (RV), eventual right heart failure, and death. Knowledge on the RV remodeling process in CTEPH is limited. The progression to RV failure in CTEPH is a markedly slower process. A detailed understanding of the pathophysiology and underlying mechanisms of RV remodeling may facilitate early diagnosis and the development of targeted therapy. While ultrasound, magnetic resonance imaging, right heart catheterization, and serum biomarkers have been used to assess cardiac function, the current treatment strategies reduce the afterload of the right heart, but are less effective in improving the maladaptive remodeling of the right heart. This review systematically summarizes the current knowledge on adaptive and maladaptive remodeling of the right heart in CTEPH from molecular mechanisms to clinical practice.
慢性血栓栓塞性肺动脉高压（CTEPH）是一种诊断不足但有潜在治愈可能的肺血管疾病。CTEPH中肺血管阻力增加是由未溶解的近端血栓和肺血管继发性微血管病变引起的，导致右心室（RV）发生适应性和 maladaptive 重塑，最终导致右心衰竭和死亡。关于CTEPH中右心室重塑过程的知识有限。CTEPH进展为右心衰竭的过程明显较慢。对右心室重塑的病理生理学和潜在机制的详细了解可能有助于早期诊断和靶向治疗的发展。虽然超声、磁共振成像、右心导管检查和血清生物标志物已被用于评估心脏功能，但目前的治疗策略降低了右心的后负荷，但在改善右心的 maladaptive 重塑方面效果较差。本综述系统地总结了目前关于CTEPH中右心适应性和 maladaptive 重塑从分子机制到临床实践的知识。
