Neuilly Orlane, Lisa Le Ngoc Anh, Khairy Paul, Hiram Roddy
Montreal Heart Institute, Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada.
CJC Open. 2025 Jun 23;7(9):1170-1189. doi: 10.1016/j.cjco.2025.06.013. eCollection 2025 Sep.
Conditions provoking the electrical and structural-functional remodelling of the myocardium can lead to the development of heart rhythm disorders, including atrial fibrillation (AF) and ventricular tachyarrhythmias that can cause sudden cardiac death. Right heart disease (RHD) causes progressive structural and functional remodelling of the right heart responsible for right ventricular and atrial dysfunction and arrhyhmias. Conditions contributing to the development of RHD include left heart disease, pulmonary arterial hypertension, congenital heart disease, right-sided myocardial infarction due to coronary artery occlusion, and amyloidosis. In adult patients with RHD associated with pulmonary arterial hypertension, the prevalence of AF is about 20%, and in adult patients with arrhythmogenic right ventricular cardiomyopathy, it is 14%. A study has suggested that compared to non patients without congenital heart disease, AF appears 30 years earlier in adult patients with congenital heart disease, with a 10-20-fold-higher incidence. This narrative review article aims to review knowledge about the pathophysiology of RHD associated with cardiac arrhythmia. Evidence is reported about the mechanisms underlying the initiation and maintenance of the arrhythmogenic substrate in RHD. We summarize the available experimental approaches to study RHD associated with cardiac arrhythmia, including models (isolated cardiomyocytes, fibroblasts) and models (monocrotaline, pulmonary artery banding, Sugen/hypoxia). In addition, we discuss potential future strategies targeting myocardial inflammation and fibrosis in the prevention of cardiac arrhythmia in RHD.
引发心肌电重构和结构 - 功能重构的情况可导致心律失常的发生，包括心房颤动（AF）和室性快速心律失常，这些心律失常可导致心源性猝死。右心疾病（RHD）会导致右心进行性结构和功能重构，进而导致右心室和心房功能障碍及心律失常。促成RHD发生的情况包括左心疾病、肺动脉高压、先天性心脏病、冠状动脉闭塞导致的右心室心肌梗死以及淀粉样变性。在患有与肺动脉高压相关的RHD的成年患者中，AF的患病率约为20%，而在患有致心律失常性右心室心肌病的成年患者中，这一比例为14%。一项研究表明，与无先天性心脏病的患者相比，先天性心脏病成年患者发生AF的时间要早30年，发病率高出10 - 20倍。这篇叙述性综述文章旨在回顾有关与心律失常相关的RHD病理生理学的知识。报告了有关RHD中致心律失常底物起始和维持的潜在机制的证据。我们总结了研究与心律失常相关的RHD的现有实验方法，包括 模型（分离的心肌细胞、成纤维细胞）和 模型（野百合碱、肺动脉环扎、Sugen/低氧）。此外，我们还讨论了未来针对心肌炎症和纤维化预防RHD中心律失常的潜在策略。
