Exercise in Genetic Cardiovascular Conditions

 描述(申请人提供)：锻炼是数百万美国人生活中不可或缺的一部分。锻炼的好处是众所周知的--提高寿命和生活质量(QOL)。然而，尽管关于患有遗传性心血管疾病(如长QT综合征(LQTS)和肥厚型心肌病(HCM))的运动安全性的数据很少，但这些患者因激烈的竞争或娱乐活动而导致心源性猝死的风险被认为高得令人望而却步，这些患者受到限制 不仅是参与竞技体育，而且还参与大多数休闲运动，这是美国和欧洲目前达成的共识声明。然而，初步数据表明，第一，许多患有这些疾病的患者尽管受到限制，但仍在锻炼，第二，风险可能比假设的低，这些患者限制运动可能会产生有害的影响。对于患有LQT和HCM的患者来说，运动的风险是否超过了医疗和生活质量的好处，从来没有进行过前瞻性的评估。这项研究将前瞻性地调查运动对LQTS患者的风险和好处 和HCM在平行观测队列中。这项研究将评估这样一种假设，即适度或剧烈锻炼的人死亡、心脏骤停、晕厥或适当的ICD治疗的风险不会高于久坐不动的人，而锻炼的人的生活质量将更高。选择继续锻炼的HCM和LQT患者将通过传统的招募网站进行识别和登记，并通过与患者倡导组织和不太活跃的患者合作，将被登记为对照小组。然后，这些受试者将被前瞻性地跟踪三年。这项研究的结果将会有 对病人护理产生直接和直接的影响。如果这些假设是正确的，那么将有更多的人享受到锻炼的好处。如果这些假设被证明是不正确的，那么目前 运动需要了解，探索性目标将调查哪些表型、临床、基因和运动相关因素可能会缓和运动对心律失常结果的影响，并将确定可能缓和运动限制对生活质量影响的临床和心理变量。

 DESCRIPTION (provided by applicant): Exercise is an integral part of life for millions of Americans. The benefits of exercise are well- known-improvement in longevity and quality of life (QOL). However, while data on safety of exercise for patients with genetic cardiovascular conditions, such as the long QT syndrome (LQTS), and hypertrophic cardiomyopathy (HCM,) are sparse, the risk of sudden cardiac death due to vigorous competitive or recreational activities for these patients is thought to be prohibitively high and these patients are restricted from participation in not just competitive, but most recreational sports as well, by current consensus statements in both the US and Europe. However, preliminary data suggest that first, many patients with these conditions are exercising despite the restrictions and second, that the risks may be lower than postulated, and that there may be detrimental effects of exercise restriction in these patients. Whether the risks of exercise outweigh the medical and quality-of-life benefits for patients with these LQTS and HCM has never been evaluated prospectively. This study will prospectively investigate the risks and benefits of exercise for patients with LQTS and HCM in parallel observational cohorts. The study will evaluate the hypothesis that risks of death, cardiac arrest, syncope, or appropriate ICD therapy will not be higher in those exercising moderately or vigorously than the sedentary, and that quality of life will be greater in those exercising. Individuals with HCM and LQT who have chosen to continue to exercise will be identified and enrolled both through traditional recruiting sites, and through partnering with patient-advocacy organizations and less active patients will be enrolled as a comparator group. These subjects will then be followed prospectively for three years. Results of this study will have an immediate and direct impact on patient care. If the hypotheses are correct, many more individuals will enjoy the benefits of exercise. If the hypotheses prove incorrect, those currently exercising need to know, and exploratory aims will investigate what phenotypic, clinical, genotypic, and sports-related factors may moderate the impact of exercise on arrhythmic outcomes, and will identify clinical and psychological variables which may moderate the impact of exercise restriction on quality of life.