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肥厚型心肌病患者中段室间隔梗阻的临床意义。

Clinical implications of midventricular obstruction in patients with hypertrophic cardiomyopathy.

机构信息

Department of Cardiology, Tokyo Women's Medical University, Tokyo, Japan.

出版信息

J Am Coll Cardiol. 2011 Jun 7;57(23):2346-55. doi: 10.1016/j.jacc.2011.02.033.

DOI:10.1016/j.jacc.2011.02.033
PMID:21636036
Abstract

OBJECTIVES

We investigated the prevalence, clinical characteristics, and prognosis of hypertrophic cardiomyopathy (HCM) patients with midventricular obstruction (MVO).

BACKGROUND

Previous descriptions of patients with MVO have been confined to case reports or small patient series, and this subgroup of HCM patients has therefore remained underrecognized.

METHODS

The study population included 490 HCM patients. Left ventricular MVO was diagnosed when the peak midcavitary gradient was estimated to be ≥30 mm Hg.

RESULTS

MVO was identified in 46 patients (9.4%). Patients with MVO were more likely to be symptomatic than those without. MVO was found to be an independent determinant of HCM-related death in multivariate models (hazard ratio [HR]: 2.23, p = 0.016), and this trend was especially pronounced for the combined endpoint of sudden death and potentially lethal arrhythmic events (HR: 3.19, p < 0.001). Apical aneurysm formation was identified in 28.3% of patients with MVO and strongly predicted HCM-related death (HR: 3.47, p = 0.008) and the combined endpoint of sudden death and potentially lethal arrhythmic events (HR: 5.08, p < 0.001). In addition, MVO without apical aneurysm was also identified as an independent determinant of the combined endpoint of sudden death and potentially lethal arrhythmic events (HR: 2.43, p = 0.045).

CONCLUSIONS

This analysis identified MVO as an independent predictor of adverse outcomes, especially the combined endpoint of sudden death and potentially lethal arrhythmic events. Our results suggest that longer periods of exposure to MVO might lead to unfavorable consequences. They also support the principle that the presence of MVO in patients with HCM has important pathophysiological implications.

摘要

目的

我们研究了伴有中室间隔梗阻(MVO)的肥厚型心肌病(HCM)患者的患病率、临床特征和预后。

背景

此前对伴有 MVO 的患者的描述仅限于病例报告或小系列病例,因此这一 HCM 患者亚组仍未被充分认识。

方法

研究人群包括 490 例 HCM 患者。当估计左心室中腔梯度≥30mmHg 时,诊断为左心室 MVO。

结果

46 例(9.4%)患者存在 MVO。与无 MVO 者相比,MVO 患者更可能出现症状。在多变量模型中,MVO 是 HCM 相关性死亡的独立决定因素(风险比[HR]:2.23,p=0.016),对于猝死和潜在致命性心律失常事件的联合终点,这一趋势更为明显(HR:3.19,p<0.001)。MVO 患者中 28.3%存在心尖动脉瘤形成,强烈预测 HCM 相关性死亡(HR:3.47,p=0.008)和猝死和潜在致命性心律失常事件的联合终点(HR:5.08,p<0.001)。此外,无心尖动脉瘤的 MVO 也被确定为猝死和潜在致命性心律失常事件联合终点的独立决定因素(HR:2.43,p=0.045)。

结论

本分析确定 MVO 是不良结局的独立预测因素,特别是猝死和潜在致命性心律失常事件的联合终点。我们的结果表明,较长时间的 MVO 暴露可能会导致不良后果。它们还支持这样一个原则,即 HCM 患者中存在 MVO 具有重要的病理生理意义。

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