Tu Bin, Wu Lingmin, Zheng Lihui, Liu Shangyu, Sheng Lishui, Liu Limin, Zhu Zhenghui, Yao Yan
National Key Laboratory, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Front Cardiovasc Med. 2021 Nov 12;8:769138. doi: 10.3389/fcvm.2021.769138. eCollection 2021.
Current treatment guidelines for arrhythmogenic right ventricular cardiomyopathy (ARVC) mainly emphasize on prevention of ventricular arrhythmic events. Despite the progressive nature of ARVC, therapeutic options focusing on decelerating disease progression are scarce. This retrospective observational cohort study included 311 patients [age, 39.1 ± 14.4 years; male, 233 (74.9%)] with a definite diagnosis of ARVC as determined by the 2010 Task Force Diagnostic Criteria. Among them, 113 patients (36.3%) received ACEI/ARB treatment. Disease progression was evaluated according to repeat transthoracic echocardiograms with a linear mixed model. Patients receiving ACEI/ARB treatment were associated with slower disease progression reflected by a gradual decrease in tricuspid annular plane systolic excursion than those not receiving ACEI/ARB treatment (0.37 vs. 0.61 mm per year decrease, < 0.001) and slower dilation of right ventricular outflow tract (0.57 vs. 1.06 mm per year increased, = 0.003). Cox proportional hazard regression models were used to evaluate the association between life-threatening ventricular tachycardia events and ACEI/ARB treatment. A reduced risk of life-threatening ventricular arrhythmia was associated with ACEI/ARB treatment compared to that without ACEI/ARB treatment (adjusted HR: 0.71, 95% CI: 0.52-0.96, = 0.031). ACEI/ARB treatment is associated with slower disease progression and lower risk of life-threatening ventricular arrhythmia in patients with ARVC. Delaying disease progression may pave way for reducing life-threatening ventricular arrhythmia risk.
致心律失常性右室心肌病(ARVC)的现行治疗指南主要强调预防室性心律失常事件。尽管ARVC具有进展性,但专注于减缓疾病进展的治疗选择却很少。这项回顾性观察性队列研究纳入了311例患者[年龄,39.1±14.4岁;男性,233例(74.9%)],这些患者根据2010年工作组诊断标准被确诊为ARVC。其中,113例患者(36.3%)接受了ACEI/ARB治疗。采用线性混合模型根据重复的经胸超声心动图评估疾病进展。与未接受ACEI/ARB治疗的患者相比,接受ACEI/ARB治疗的患者疾病进展较慢,表现为三尖瓣环平面收缩期位移逐渐减小(每年减小0.37 vs. 0.61 mm,<0.001),右室流出道扩张较慢(每年增加0.57 vs. 1.06 mm,=0.003)。采用Cox比例风险回归模型评估危及生命的室性心动过速事件与ACEI/ARB治疗之间的关联。与未接受ACEI/ARB治疗相比,接受ACEI/ARB治疗可降低危及生命的室性心律失常风险(校正HR:0.71,95%CI:0.52 - 0.96,=0.031)。ACEI/ARB治疗与ARVC患者较慢的疾病进展和较低的危及生命的室性心律失常风险相关。延缓疾病进展可能为降低危及生命的室性心律失常风险铺平道路。