马芬综合症. 马芬综合症. 大动脉动脉瘤修复后的长期生存和并发症
R Finkbohner1, D Johnston, E S Crawford
1Department of Internal Medicine, University of Texas Medical School at Houston 77036.
Circulation
|February 1, 1995
概括
大动脉动脉瘤的手术修复显著改善了马尔凡综合征患者的预期寿命. 然而,由于疾病的全身性质,许多人需要进一步的大动脉手术.
科学领域:
- 心血管外科心血管外科
- 遗传学 是一个遗传学.
- 血管医学 血管医学
背景情况:
- 马凡综合征 (MFS) 与危及生命的大动脉动脉瘤和剖析有关.
- 现在手术的进步允许在MFS患者中治疗这些心血管并发症.
研究的目的:
- 评估外科手术修复大动脉动脉瘤对马凡综合征患者预期寿命的影响.
- 分析这一群体中大动脉修复后的长期临床过程和重新手术率.
主要方法:
- 对192名马方综合征患者的医疗记录的回顾性审查,这些患者在26年内接受了大动脉动脉瘤修复.
- 使用卡普兰-梅尔曲线和统计数据分析进行生存分析.
- 采访患者以获得全面的术前和术后数据.
主要成果:
- 平均存活时间增加到61年,比之前报告的47年显著改善 (P < .0006).
- 53%的患者因随后的动脉瘤或解剖而需要第二次手术,主要涉及大动脉.
- 上升大动脉动脉瘤修复是最常见的初始手术;初始手术时的剖析预测了重新手术.
结论:
- 动脉动脉瘤的手术修复已明显改善了马尔凡综合征患者的生存率.
- 重复手术的高率凸显了马尔凡综合征会影响整个大动脉,需要终身监测.
- 与接受预防性修复的患者相比,初始主动脉解剖患者面临更高的风险,需要随后的主动脉手术.
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