马尔芬人群中的大动脉事件率:一个队列研究
Guillaume Jondeau1, Delphine Detaint, Florence Tubach
1Centre de Référence pour le Syndrome de Marfan et Apparentés, Hôpital Bichat, 75018 Paris, France. guillaume.jondeau@bch.aphp.fr
Circulation
|December 3, 2011
概括
马凡综合征 (MFS) 的最佳管理表明大动脉剖析风险低于50mm以下. 50毫米的预防性手术对于MFS患者来说是一个合理的门.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 血管外科 血管外科
背景情况:
- 对于马凡综合征的管理,特别是手术时间,缺乏关于大动脉风险的可靠数据.
- 大动脉并发症是马尔凡综合征发病率和死亡率的主要原因.
研究的目的:
- 根据标准化护理协议,评估马方综合征患者的大动脉风险.
- 确定在马方综合征中预防性大动脉手术的最佳门.
主要方法:
- 对732名符合国际标准的马方综合征患者的回顾性分析.
- 标准化护理包括每两年进行一次心声回声检查,贝塔阻塞和活动辅导.
- 建议在最大的大动脉直径为50毫米时进行预防性手术.
主要成果:
- 死亡或大动脉解剖的整体事件率为每年0.17%.
- 大动脉剖析风险随着大动脉直径显著增加,特别是在≥50毫米时.
- 对直径小于40毫米的风险低 (0.09%/年),对于45-49毫米的风险为0.3%/年.
结论:
- 在马尔凡综合征患者中,大动脉剖析风险仍然很低,大动脉直径在45至49毫米之间.
- 大动脉直径为50毫米是考虑预防性大动脉手术的合理门.
- 标准化的医疗和外科管理可以有效地减轻马尔凡综合征中的大动脉风险.
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