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心电图表现与肥厚型心肌病患者表型表达的关系:一项心脏磁共振研究。

Relationship of ECG findings to phenotypic expression in patients with hypertrophic cardiomyopathy: a cardiac magnetic resonance study.

机构信息

Division of Cardiology, Cardinal Massaia Hospital of Asti and Faculty of Medicine University of Turin, Asti, Italy.

出版信息

Int J Cardiol. 2013 Aug 10;167(3):1038-45. doi: 10.1016/j.ijcard.2012.03.074. Epub 2012 Mar 30.

DOI:10.1016/j.ijcard.2012.03.074
PMID:22464482
Abstract

BACKGROUND

The 12-lead electrocardiogram (ECG) is considered an essential screening tool for hypertrophic cardiomyopathy (HCM). A vast array of ECG abnormalities has been described in HCM, although their relationship to left ventricle (LV) morphology and degree of hypertrophy appears elusive. Aim of this study was to assess the relationship of ECG patterns with the HCM phenotype assessed according to the novel opportunities offered by cardiac magnetic imaging (CMR).

METHODS

CMR and 12-lead ECG were performed in 257 HCM patients. Severity of ECG abnormalities was defined by the sum of 9 criteria: abnormal cardiac rhythm, QRS duration ≥ 100 ms, Romhilt-Estes score ≥ 5, fascicular block (LAHB) and/or bundle-branch block (LBBB or RBBB), ST-T abnormalities, ST-T segment elevation ≥ 0.2 mV, prolonged QTc interval, pathological Q waves, absence of normal Q wave. Four ECG groups were identified: normal (0 criteria); mildly abnormal (1-3 criteria); moderately abnormal (4-6 criteria); markedly abnormal (7-9 criteria).

RESULTS

There was a direct relationship between severity of ECG abnormalities and HCM phenotype. LV mass index was normal in most patients with normal ECG and progressively increased with each class of ECG score, from 70.9 ± 18.6g/m(2) in patients with normal ECG to 107.1 ± 55.1g/m(2) among those with markedly abnormal ECG (p=<0.0001). Likewise, the prevalence and extent of late gadolinium enhancement (LGE) increased significantly with the ECG score, from 37% in patients with normal ECG to 93% in patients with markedly abnormal ECG (overall p=0.0012). A normal ECG had a negative predictive accuracy of 96% for markedly increased LV mass (>91 g/m(2) for men and >69 g/m(2) for women), and of 100% for maximum LV thickness ≥ 30 mm.

CONCLUSIONS

In a large HCM cohort, the number and severity of ECG abnormalities were directly related to phenotypic expression as revealed by CMR. Although false negative ECG findings remain a challenge in population screenings for HCM, a normal ECG proved effective in ruling out severe LV hypertrophy, suggesting potential implications for long-term follow-up of HCM patients and family members. A simple score for quantification of ECG abnormalities in HCM patients is proposed.

摘要

背景

12 导联心电图(ECG)被认为是肥厚型心肌病(HCM)的基本筛查工具。尽管已经描述了大量 HCM 中的 ECG 异常,但它们与左心室(LV)形态和肥厚程度的关系似乎难以捉摸。本研究旨在评估根据心脏磁共振成像(CMR)提供的新机会评估的 ECG 模式与 HCM 表型之间的关系。

方法

对 257 例 HCM 患者进行了 CMR 和 12 导联心电图检查。通过 9 项标准总和来定义 ECG 异常的严重程度:异常心律、QRS 持续时间≥100ms、Romhilt-Estes 评分≥5、束支阻滞(LAHB 和/或 LBBB 或 RBBB)、ST-T 异常、ST-T 段抬高≥0.2mV、QTc 间期延长、病理性 Q 波、正常 Q 波缺失。确定了 4 种 ECG 组:正常(0 项标准);轻度异常(1-3 项标准);中度异常(4-6 项标准);明显异常(7-9 项标准)。

结果

随着 ECG 异常严重程度的增加,ECG 异常和 HCM 表型之间存在直接关系。大多数心电图正常的患者 LV 质量指数正常,并且随着 ECG 评分的增加而逐渐增加,从心电图正常患者的 70.9±18.6g/m2增加到明显异常心电图患者的 107.1±55.1g/m2(p<0.0001)。同样,随着 ECG 评分的增加,晚期钆增强(LGE)的发生率和范围显著增加,从心电图正常患者的 37%增加到明显异常心电图患者的 93%(总体 p=0.0012)。正常心电图对明显增加的 LV 质量(男性>91g/m2,女性>69g/m2)和最大 LV 厚度≥30mm 的预测准确率为 96%,阴性预测准确率为 100%。

结论

在大型 HCM 队列中,ECG 异常的数量和严重程度与 CMR 显示的表型表达直接相关。尽管在 HCM 的人群筛查中仍然存在假阴性 ECG 发现的挑战,但正常心电图在排除严重 LV 肥厚方面是有效的,这表明对 HCM 患者和家庭成员的长期随访可能具有潜在意义。提出了一种用于量化 HCM 患者 ECG 异常的简单评分。

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