Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers A European Cohort Study
Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers A European Cohort Study
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DOI：
10.1016/j.jacc.2011.08.078
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发表时间：
2012-01-31
影响因子：
24
通讯作者：
Pinto, Yigal M.
中科院分区：
文献类型：
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作者：
van Rijsingen, Ingrid A. W.;Arbustini, Eloisa;Pinto, Yigal M.
目的探讨Lamin A/C （LMNA）突变携带者发生恶性室性心律失常（MVA）的危险因素。背景LMNA突变引起多种临床表型，包括扩张型心肌病和传导疾病。许多LMNA突变携带者预后不良，因为MVA的频率高，进展到终末期心力衰竭。然而，目前尚不清楚如何识别有MVA风险的突变携带者。方法在这个包含269名LMNA突变携带者的多中心队列中，我们评估了MVA的危险因素，定义为心源性猝死、复苏和适当的植入式心律转复除颤器（ICD）治疗。结果中位随访43个月（四分位数范围17 ~ 101个月），48例（18%）患者出现MVA首次发作，11例患者心肺复苏成功，25例患者接受适当的ICD治疗，12例患者突然死亡。非持续性室性心动过速、左室射血分数是MVA的独立危险因素
Objectives The purpose of this study was to determine risk factors that predict malignant ventricular arrhythmias (MVA) in Lamin A/C (LMNA) mutation carriers.Background LMNA mutations cause a variety of clinical phenotypes, including dilated cardiomyopathy and conduction disease. Many LMNA mutation carriers have a poor prognosis, because of a high frequency of MVA and progression to end-stage heart failure. However, it is unclear how to identify mutation carriers that are at risk for MVA.Methods In this multicenter cohort of 269 LMNA mutation carriers, we evaluated risk factors for MVA, defined as sudden cardiac death, resuscitation, and appropriate implantable cardioverter-defibrillator (ICD) treatment.Results In a median follow-up period of 43 months (interquartile range: 17 to 101 months), 48 (18%) persons experienced a first episode of MVA: 11 persons received successful cardiopulmonary resuscitation, 25 received appropriate ICD treatment, and 12 persons died suddenly. Independent risk factors for MVA were nonsustained ventricular tachycardia, left ventricular ejection fraction