Long-Term Arrhythmic and Nonarrhythmic Outcomes of Lamin A/C Mutation Carriers
Long-Term Arrhythmic and Nonarrhythmic Outcomes of Lamin A/C Mutation Carriers
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DOI：
10.1016/j.jacc.2016.08.058
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发表时间：
2016-11-29
影响因子：
24
通讯作者：
Lakdawala, Neal K.
中科院分区：
文献类型：
--
作者：
Kumar, Saurabh;Baldinger, Samuel H.;Lakdawala, Neal K.
背景：LMNA突变是可变表达的，可能导致心肌病、房室传导阻滞（AVB）或心房心律失常（AAs）和室性心律失常（VA）。关于lmna相关的心律失常和非心律失常结果的详细自然史研究是有限的，并且心脏表型指标的预后意义仍然不确定。目的：本研究旨在描述LMNA突变携带者的心律失常和非心律失常的结果，并评估心脏表型指数的预后意义。方法AVB、AA、持续性VA、左室收缩功能障碍（LVD）发生率（-左室射血分数）
BACKGROUND Mutations in LMNA are variably expressed and may cause cardiomyopathy, atrioventricular block (AVB), or atrial arrhythmias (AAs) and ventricular arrhythmias (VA). Detailed natural history studies of LMNA-associated arrhythmic and nonarrhythmic outcomes are limited, and the prognostic significance of the index cardiac phenotype remains uncertain.OBJECTIVES This study sought to describe the arrhythmic and nonarrhythmic outcomes of LMNA mutation carriers and to assess the prognostic significance of the index cardiac phenotype.METHODS The incidence of AVB, AA, sustained VA, left ventricular systolic dysfunction (LVD) (- left ventricular ejection fraction