Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers.
Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers.
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运动增加了与年龄相关的外观和心律不齐的风险，在心律不齐的右心室发育不良/心肌病相关的脱发突变携带者中。
DOI：
10.1016/j.jacc.2013.06.033
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发表时间：
2013-10-01
影响因子：
24
通讯作者：
Calkins, Hugh
中科院分区：
文献类型：
--
作者：
James, Cynthia A.;Bhonsale, Aditya;Tichnell, Crystal;Murray, Brittney;Russell, Stuart D.;Tandri, Harikrishna;Tedford, Ryan J.;Judge, Daniel P.;Calkins, Hugh
关键词：
确定运动如何影响桥粒突变患者致心律失常性右心室发育不良/心肌病（ARVD/C）的外显率。虽然动物模型和轶事证据表明运动是ARVD/C的风险因素，但尚未进行系统的人体研究。87名携带者（46名男性，平均年龄44±18岁）从10岁开始接受定期体力活动的采访。研究了运动与以下因素的关系：1）持续性室性心律失常（VT/VF），2）C期心力衰竭（HF），3）符合ARVD/C（TFC）诊断标准。耐力运动员（n=56）在较年轻时出现症状（30.1±13.0 vs. 40.6±21.1岁，p=0.05），在末次随访时更有可能遇到TFC（82% vs. 35%，p<0.001），无VT/VF（p=0.013）和HF（p=0.004）的终身生存率较低。与那些在就诊前每年运动最少（最低四分位数）的人相比，第二（OR=6.64，p=0.013），第三（OR=16.7，p=0.001）和最高（OR=25.3，p<0.0001）四分位数的人更有可能达到TFC。在61例未出现VT/VF的受试者中，在平均8.4±6.7年随访期间发生首次VT/VF事件的13例受试者均为耐力运动员（p=0.002）。在临床表现之前（p=0.036）和之后（p=0.005），运动最多（前四分位数）的患者首次VT/VF的生存率最低。在前四分位数的个体中，减少运动可降低VT/VF风险（p=0.04）。耐力运动和频繁运动增加桥粒突变携带者VT/VF、HF和ARVD/C的风险。这些发现支持这些患者的运动限制。
To determine how exercise influences penetrance of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) among patients with desmosomal mutations. While animal models and anecdotal evidence suggest exercise is a risk factor for ARVD/C, there have been no systematic human studies. Eighty-seven carriers (46 male, mean age 44±18) were interviewed about regular physical activity from age ten. The relationship of exercise with; 1) sustained ventricular arrhythmia (VT/VF), 2) stage C heart failure (HF), and 3) meeting diagnostic criteria for ARVD/C (TFC) was studied. Endurance athletes (n=56) developed symptoms at a younger age (30.1±13.0 vs. 40.6±21.1 years, p=0.05), were more likely to meet TFC at last follow-up (82% vs. 35%, p<0.001), and had a lower lifetime survival free from VT/VF (p=0.013) and HF (p=0.004). Compared to those who did the least (lowest quartile) exercise per year prior to presentation, those in the second (OR=6.64, p=0.013), third (OR=16.7, p=0.001), and top (OR=25.3, p<0.0001) quartiles were increasingly likely to meet TFC. Among 61 who did not present with VT/VF, the 13 subjects experiencing a first VT/VF event over a mean 8.4±6.7 year follow-up were all endurance athletes (p=0.002). Survival from first VT/VF was lowest among those who exercised most (top quartile) both prior to (p=0.036) and after (p=0.005) clinical presentation. Among individuals in the top quartile, a reduction in exercise decreased VT/VF risk (p=0.04). Endurance exercise and frequent exercise increase risk of VT/VF, HF, and ARVD/C in desmosomal mutation carriers. These findings support exercise restriction for these patients.
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影响因子：
37.8
作者：
Dalal, D;Nasir, K;Calkins, H
通讯作者：
Calkins, H
影响因子：
37.8
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作者：
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通讯作者：
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作者：
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