自然史和风险分层的arrhythmogenic右心室发育不良/心肌病
Jean-Sébastien Hulot1, Xavier Jouven, Jean-Philippe Empana
1INSERM Avenir & U252, Pitié-Salpêtrière University Hospital, Assistance-Publique Hôpitaux de Paris, France.
Circulation
|September 29, 2004
概括
节律失调的右心室发育不良/心肌病 (ARVD/C) 患有右或左心室功能障碍的患者面临更高的心血管死亡风险. 腹腔动脉短心结合这些因素可以识别高风险个体.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 电子生理学 电子生理学
背景情况:
- 由于自然病史数据有限和心血管死亡风险分层,心律失常性右心室张力变形/心肌病 (ARVD/C) 管理具有挑战性.
- 确定长期预后的风险因素对于有效的患者管理至关重要.
研究的目的:
- 在被诊断为ARVD/C的患者中确定与长期心血管死亡相关的风险因素.
- 为了改善风险分层,并为ARVD/C患者的临床决策提供信息.
主要方法:
- 在1977年至2000年间使用国际标准诊断的130名ARVD/C患者的回顾性分析.
- 后勤回归建模以确定心血管死亡的风险因素,平均随访时间为8.1年.
主要成果:
- 总共有24人死亡 (2.3%年死亡率),其中21人死于心血管疾病 (14人心力衰竭,7人突然死亡).
- 所有去世的患者都有心室动脉冲动史.
- 多变量分析确定了右心室衰竭和左心室功能障碍是心血管死亡的独立预测因素,特别是在与心室低心率相结合时.
结论:
- 腹腔功能障碍 (右或左) 的临床症状是ARVD/C.中心血管死亡的显著独立风险因素.
- 腹腔动脉短心症与腹腔功能障碍迹象的结合确定了高风险的患者.
- 建议对大型国际注册表进行进一步分析,以完善这些预后发现.
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