Right ventricle remodeling in chronic thromboembolic pulmonary hypertension

[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.

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