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节律失常的右心室发育不良:美国的经验
Darshan Dalal1, Khurram Nasir, Chandra Bomma
1Division of Cardiology, The Johns Hopins University School of Medicine, Baltimore, MD, USA.
Circulation
|December 14, 2005
概括
节律失调的右心室发育不良 (ARVD) 在年轻人中经常出现心或昏迷. 早期诊断和植入式心脏转换器-除器 (ICD) 治疗显著降低ARVD患者的死亡率.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 遗传性疾病是一种遗传性疾病.
背景情况:
- 节律失调的右心室发育不良 (ARVD) 是一种遗传性心肌病.
- 它的特征是渐进的右心室功能障碍和心室心律不整.
- 遗传异质性有助于疾病呈现和进展的变化.
研究的目的:
- 描述ARVD的临床表现,特征,存活率和自然史.
- 分析美国大型患者队列的结果.
- 为ARVD的临床管理和遗传咨询提供信息.
主要方法:
- 美国100名ARVD患者的回顾性分析.
- 对患者人口统计学,表现症状,诊断时间表和治疗方式的审查.
- 卡普兰-梅尔生存分析和评估结果,包括突然心脏死亡 (SCD) 和心脏移植.
主要成果:
- 呈现时的平均年龄为26岁,在32%的病例中具有家族性模式.
- 常见的症状包括心 (27%),昏迷 (26%),心脏突然死亡 (SCD) (23%) 等.
- 平均存活时间为60年; 随着及时诊断和植入式心脏转换器-除器 (ICD) 植入,死亡率较低.
结论:
- ARVD通常在生命的第二到第五十年之间表现出来.
- 延迟诊断是常见的,但ICD治疗可以降低死亡率.
- 在ARVD呈现和过程中显著的变异性强调了遗传因素的重要性.
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