Right ventricle remodeling in chronic thromboembolic pulmonary hypertension

Journal of Translational Internal Medicine

article

Jixiang Liu¹  Peiran Yang⁶  Han Tian¹  Kaiyuan Zhen¹  Colm McCabe⁸  Lan Zhao⁸  Zhenguo Zhai¹ 

[1] Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital;National Center for Respiratory Medicine;National Clinical Research Center for Respiratory Disease;Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, Peking Union Medical College;Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences and Peking Union Medical College;Department of Physiology, State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, School of Basic Medicine Peking Union Medical College;Peking University China–Japan Friendship School of Clinical Medicine;National Heart and Lung Institute ,(NHLI), Imperial College London

关键词: chronic thromboembolic pulmonary hypertension;    right ventricular failure;    maladaptive remodeling;    pathophysiology;    targeted medicine;   

DOI  :  10.2478/jtim-2022-0027

来源: Walter de Gruyter GmbH

【 摘 要 】

Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed, butpotentially curable pulmonary vascular disease. The increased pulmonary vascular resistancein CTEPH is caused by unresolved proximal thrombus and secondary microvasculopathy in thepulmonary vasculature, leading to adaptive and maladaptive remodeling of the right ventricle(RV), eventual right heart failure, and death. Knowledge on the RV remodeling process inCTEPH is limited. The progression to RV failure in CTEPH is a markedly slower process. Adetailed understanding of the pathophysiology and underlying mechanisms of RV remodelingmay facilitate early diagnosis and the development of targeted therapy. While ultrasound,magnetic resonance imaging, right heart catheterization, and serum biomarkers have beenused to assess cardiac function, the current treatment strategies reduce the afterload of theright heart, but are less effective in improving the maladaptive remodeling of the right heart.This review systematically summarizes the current knowledge on adaptive and maladaptiveremodeling of the right heart in CTEPH from molecular mechanisms to clinical practice.

【 授权许可】

Unknown