Cadrin-Tourigny Julia, Tadros Rafik, Talajic Mario, Rivard Lena, Abadir Sylvia, Khairy Paul
From the Electrophysiology Service, Montreal Heart Institute and Department of Medicine, Université de Montréal, 5000 Bélanger St. E., Montreal, Quebec, H1T 1C8, Canada.
Expert Rev Cardiovasc Ther. 2015 Jun;13(6):653-64. doi: 10.1586/14779072.2015.1043891. Epub 2015 May 6.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is an uncommon but increasingly recognized inherited cardiomyopathy that is associated with malignant ventricular arrhythmias and sudden cardiac death, particularly in young individuals. The implantable cardioverter-defibrillator (ICD) is widely regarded as the only treatment modality with evidence to support improved survival in patients with ARVC and secondary prevention indications. In contrast, there is no universally accepted risk stratification scheme to guide ICD therapy for primary prevention against sudden cardiac death. Potential benefits must be weighed against the considerable risks of complications and inappropriate shocks in this young patient population. This article tackles the challenges of risk stratification for sudden cardiac death in ARVC and critically appraises available evidence for various proposed risk factors. The authors' over-arching objective is to provide the clinician with evidence-based guidance to inform decisions regarding the selection of appropriate candidates with ARVC for ICD therapy.
致心律失常性右室心肌病/发育异常（ARVC）是一种罕见但日益被认识的遗传性心肌病，与恶性室性心律失常和心源性猝死相关，尤其是在年轻人中。植入式心律转复除颤器（ICD）被广泛认为是唯一有证据支持可提高ARVC患者生存率及用于二级预防的治疗方式。相比之下，尚无普遍接受的风险分层方案来指导针对心源性猝死一级预防的ICD治疗。对于这一年轻患者群体，必须权衡潜在益处与并发症及不适当电击的重大风险。本文探讨了ARVC心源性猝死风险分层的挑战，并批判性地评估了各种提出的风险因素的现有证据。作者的总体目标是为临床医生提供基于证据的指导，以帮助其做出关于选择合适的ARVC患者接受ICD治疗的决策。
