Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death
Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death
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DOI：
10.1161/circulationaha.115.016291
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发表时间：
2015-08-18
期刊：
影响因子：
37.8
通讯作者：
Iliceto, Sabino
中科院分区：
文献类型：
--
作者：
Basso, Cristina;Marra, Martina Perazzolo;Iliceto, Sabino
背景 即使没有血流动力学损伤，二尖瓣脱垂 (MVP) 也可能出现室性心律失常和心源性猝死 (SCD)。心室电不稳定的结构基础仍不清楚。方法和结果回顾了650名年轻成人（40岁）SCD的心脏病理登记，并对以MVP为SCD唯一病因的病例进行了重新检查。确定了 43 名 MVP 患者（26 名女性；年龄范围 19-40 岁；中位年龄 32 岁）（占所有 SCD 的 7%，女性 13%）。 12例有心电图检查的病例中，10例（83%）下导联T波倒置，全部有右束支传导阻滞性室性心律失常。 70% 发现双叶受累。所有患者的组织学检查均在乳头肌水平检测到左心室纤维化，88% 的患者在基底壁下检测到纤维化。患有（n = 30）和不患有（对照受试者；n = 14）复杂室性心律失常的 MVP 的活着的患者接受了包括对比增强心脏磁共振在内的研究方案。患有右束支传导阻滞型或多形性复杂室性心律失常的患者（22 名女性；年龄范围，28-43 岁；中位年龄，41 岁），70% 的病例显示双叶受累。通过对比增强心脏磁共振，93% 的患者出现左心室晚期强化，而对照组这一比例为 14%（P
Background Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular electric instability remains elusive.Methods and Results The cardiac pathology registry of 650 young adults (40 years of age) with SCD was reviewed, and cases with MVP as the only cause of SCD were re-examined. Forty-three patients with MVP (26 females; age range, 19-40 years; median, 32 years) were identified (7% of all SCD, 13% of women). Among 12 cases with available ECG, 10 (83%) had inverted T waves on inferior leads, and all had right bundle-branch block ventricular arrhythmias. A bileaflet involvement was found in 70%. Left ventricular fibrosis was detected at histology at the level of papillary muscles in all patients, and inferobasal wall in 88%. Living patients with MVP with (n=30) and without (control subjects; n=14) complex ventricular arrhythmias underwent a study protocol including contrast-enhanced cardiac magnetic resonance. Patients with either right bundle-branch block type or polymorphic complex ventricular arrhythmias (22 females; age range, 28-43 years; median, 41 years), showed a bileaflet involvement in 70% of cases. Left ventricular late enhancement was identified by contrast-enhanced cardiac magnetic resonance in 93% of patients versus 14% of control subjects (P