马尔凡综合征中关节分离:多中心心血管磁共振研究
Kenan Kaya1, Jonathan Kottlors1, Thorsten W Gietzen2
1Institute for Diagnostic and Interventional Radiology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Straße 62, 50937 Cologne, Germany.
概括
中心环状断裂 (MAD) 在马凡综合征 (MFS) 中非常普遍,通常会影响所有环状部位. 虽然与心血管事件没有直接联系,但MAD程度与左心室功能障碍和大动脉扩大有关.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 医疗成像医学成像
背景情况:
- 在马凡综合征 (MFS) 中,心脏环状断裂 (MAD) 的患病率在使用心血管磁共振 (CMR) 时尚不明.
- 肌痛复杂症是一种影响连接组织的遗传性疾病,经常导致心血管并发症.
研究的目的:
- 确定使用CMR.在MFS患者中MAD的患病率,程度和分布.
- 调查MAD与左心参数,大动脉尺寸和MFS心血管事件之间的关联.
主要方法:
- 从91名患有纤维素-1基因变异的MFS患者中对CMR研究的回顾性分析.
- 经验丰富的放射科医生对MAD,心肌脱落 (MVP),左心室 (LV) 功能和大动脉尺寸的评估.
- 追踪心血管事件,包括大动脉,心律失常和 mitra事件,平均随访时间为4.0年.
主要成果:
- 在MFS患者中观察到MAD的高患病率 (81.3%),主要在下环和常常全环环.
- MAD的程度和局部性与LV扩张,减少LV喷射率和MVP有显著的相关性.
- 7.1mm的MAD程度最好地预测了MVP患病率 (敏感度为77.1%,特异性为89.3%).
结论:
- 在MFS中MAD的广泛性表明一种全身环状病理.
- 虽然整体MAD存在与心血管事件无关,但其程度和局部性与LV功能障碍,重塑和大动脉扩大有关.
- MAD可以作为MFS疾病进展的标志物,特别是在MVP和大动脉尺寸方面.
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