...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Neuromuscular disorders: NMD >Atrio-ventricular block requiring pacemaker in patients with late onset Pompe disease
【24h】

Atrio-ventricular block requiring pacemaker in patients with late onset Pompe disease

机译：迟发性庞贝病患者需进行起搏器的房室传导阻滞

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Enzyme replacement therapy consistently improves cardiac function in infantile and juvenile onset patients with Pompe disease and cardiomyopathy, but is apparently not effective in preventing rhythm disorders, an emerging cardiac phenotype in long term survivors. In patients with late onset Pompe disease cardiomyopathy is an exceptional finding while heart rhythm disorders seem to be more frequent. We retrospectively identified, among a cohort of 131 French late onset Pompe disease patients, four patients with severe atrio-ventricular blocks requiring pacemaker implantation. These patients had no other risk factors for cardiovascular diseases or cardiomyopathy. In one patient the atrioventricular block was discovered while still asymptomatic. Cardiac conduction defects are relatively rare in late onset Pompe disease and may occur even in absence of cardiac symptoms or EKG abnormalities. However because of the possible life-threatening complications associated with these conduction defects, cardiac follow-up in patients with late onset Pompe disease should include periodical Holter-EKG monitoring.
机译：酶替代疗法可不断改善患有庞贝病和心肌病的婴幼儿发作期患者的心脏功能，但显然不能有效预防心律失常，后者是长期幸存者中出现的心脏表型。在庞贝病晚期患者中，心肌病是一个例外发现，而心律失常似乎更为频繁。我们回顾性地在131名法国晚期庞普病患者中，发现4例严重的房室传导阻滞患者需要植入起搏器。这些患者没有其他心血管疾病或心肌病的危险因素。在一名患者中，房室传导阻滞被发现而仍无症状。在晚期庞贝病中，心脏传导缺陷相对少见，甚至在没有心脏症状或EKG异常的情况下也可能发生。但是，由于与这些传导缺陷相关的可能危及生命的并发症，因此对于晚期庞贝病患者的心脏随访应包括定期的Holter-EKG监测。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号