Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome
Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome
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DOI：
10.1001/jamacardio.2021.2312
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发表时间：
2021-07-07
期刊：
影响因子：
24
通讯作者：
De Backer, Julie
中科院分区：
文献类型：
--
作者：
Demolder, Anthony;Timmermans, Frank;De Backer, Julie
二尖瓣环分离(MAD)在二尖瓣脱垂、室性心动过速和心脏性猝死的患者中受到特别关注。MAD对马凡综合征(MFS)患者的临床意义在很大程度上仍未被探讨。目的定义MAD的患病率，并检查其与MFS患者心血管结局和心律失常的关系。设计、背景和参与者这项单中心的回顾性队列研究包括142名根据修订的Ghent标准确诊为MFS的患者，他们在2004年1月1日至2019年12月31日期间接受了定期随访，并接受了定期随访。患者还接受静息心电图和24小时动态心电图监测。转归包括主动脉事件(主动脉夹层或预防性主动脉手术)、心律失常事件(定义为持续性室性心动过速或心源性猝死)和二尖瓣手术。结果共评估了14 2例患者(72例女性患者(51%)，首次检查的中位年龄为25岁[范围2-])。48例患者(34%)有MAD。有MAD的患者的主动脉根部z评分高于无MAD的患者(4.1[四分位数范围，2.8-5.7]vs 3.0[四分位范围，1.8-4.0]；P
IMPORTANCE Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored.OBJECTIVE To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS.DESIGN, SETTING, AND PARTICIPANTS This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019.MAIN OUTCOMES AND MEASURES The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery.RESULTS A total of 142 patients ( 72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P