关键词: Arrhythmogenic cardiomyopathy Arrhythmogenic left ventricular cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy Cascade family screening Catheter ablation Diagnosis of arrhythmogenic cardiomyopathy Disease mechanisms Electrophysiology Exercise restriction Genetic testing Genetic variants ICD decisions Left ventricular noncompaction Risk stratification Treatment of arrhythmogenic cardiomyopathy

Mesh : Arrhythmogenic Right Ventricular Dysplasia / diagnosis therapy Consensus Humans Risk Assessment

来  源:   DOI:10.1016/j.hrthm.2019.09.019   PDF(Sci-hub)

Abstract:
Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
摘要:
致心律失常性心肌病(ACM)是一种不继发于缺血性,高血压,或者心脏瓣膜病.ACM包含了广泛的遗传,系统性,传染性,和炎症性疾病。此名称包括,但不限于,致心律失常右心室/左心室心肌病,心脏淀粉样变性,结节病,查加斯病,和左心室不紧密。ACM表型与其他心肌病重叠,尤其是伴有心律失常的扩张型心肌病,可能与心室扩张和/或收缩功能受损有关。该专家共识声明为临床医生提供了有关ACM评估和管理的指导,并包括有关遗传学和疾病机制的临床相关信息。PICO问题被用来评估当代证据,并提供与致心律失常性右心室心肌病运动相关的临床指导。建议由专家编写小组制定和批准,在用证据表进行系统的文献检索后,讨论他们自己的临床经验,介绍该领域的最新知识。每个建议都是使用美国心脏病学会和美国心脏协会制定的建议类别和证据水平系统提出的,并附有参考文献和解释性文本,以提供必要的上下文。对ACM遗传基础的持续认识为检查疾病和心律失常发展的多种触发因素和潜在的共同途径提供了机会。
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