Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.
Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.
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DOI：
10.1093/eurheartj/ehv162
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发表时间：
2015-12-07
影响因子：
39.3
通讯作者：
Calkins H
中科院分区：
文献类型：
--
作者：
Corrado D;Wichter T;Link MS;Hauer R;Marchlinski F;Anastasakis A;Bauce B;Basso C;Brunckhorst C;Tsatsopoulou A;Tandri H;Paul M;Schmied C;Pelliccia A;Duru F;Protonotarios N;Estes NA 3rd;McKenna WJ;Thiene G;Marcus FI;Calkins H
心律失常性右室心肌病/发育不良（ARVC/D）是一种遗传性心肌疾病，主要影响右心室（RV），易导致室性心律失常和心源性猝死（SCD）。在过去的三十年中，已经有大量的研究定义了该病的发病机制、遗传方面和临床表现（参见“病因学、发病机制、诊断和自然史”作为在线补充资料）。1994年和2010年，一个国际工作组（ITF）文件提出了基于心电图（ECG）、心律失常、形态学、组织病理学和临床遗传因素的ARVC/D标准化诊断指南。18,19随着对心律失常结局、危险因素和挽救生命的治疗干预措施的了解不断增加，批判性地解决和正确看待与ARVC/D患者临床管理相关的问题显得尤为及时。目前的ITF共识声明是对目前使用的风险分层算法和药物或非药物治疗方法的全面概述，这通常对心血管专家和其他从业人员，特别是那些不经常参与ARVC/D管理的从业人员构成临床挑战。
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inheritable heart muscle disease that predominantly affects the right ventricle (RV) and predisposes to ventricular arrhythmias and sudden cardiac death (SCD). 1–17 In the last three decades, there have been a significant number of studies defining the pathogenesis, genetic aspects, and clinical manifestations of the disease (See ‘Etiology, pathogenesis, diagnosis and natural history’as Supplementary material online). In 1994 and 2010, an International Task Force (ITF) document proposed guidelines for the standardized diagnosis of ARVC/D based on electrocardiographic (ECG), arrhythmic, morphological, histopathological, and clinico-genetic factors. 18, 19The growing knowledge regarding arrhythmic outcome, risk factors, and life-saving therapeutic interventions, make it particularly timely to critically address and place into perspective the issues relevant to the clinical management of ARVC/D patients. The present ITF consensus statement is a comprehensive overview of currently used risk stratification algorithms and approaches to therapy, either pharmacological or non-pharmacological, which often poses a clinical challenge to cardiovascular specialists and other practitioners, particularly those infrequently engaged in the management of ARVC/D.