Myocardial Disarray and Fibrosis across Hypertrophic Cardiomyopathy Stages Associate with ECG Markers of Arrhythmic Risk.

IF 6.7 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

European Heart Journal - Cardiovascular Imaging Pub Date : 2024-10-17 DOI:10.1093/ehjci/jeae260

Z Ashkir, A H A Samat, R Ariga, L Finnigan, S Jermy, M A Akhtar, G Sarto, P Murthy, B Wong, M B Cassar, N Beyhoff, E W Wicks, K Thomson, M Mahmod, L Tunnicliffe, S Neubauer, H Watkins, B Raman

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引用次数: 0

Abstract

Background: Myocardial disarray, an early feature of hypertrophic cardiomyopathy (HCM) and a substrate for ventricular arrhythmia, is poorly characterised in prehypertrophic sarcomeric variant carriers (SARC+LVH-).

Objectives: Using diffusion tensor cardiac magnetic resonance (DT-CMR) we assessed myocardial disarray and fibrosis in both SARC+LVH- and HCM patients and evaluated the relationship between microstructural alterations and electrocardiographic (ECG) parameters associated with arrhythmic risk.

Methods: Sixty-two individuals (24 SARC+LVH-, 24 HCM and 14 matched controls) were evaluated with multiparametric CMR including stimulated echo acquisition mode (STEAM) DT-CMR, and blinded quantitative 12-lead ECG analysis.

Results: Mean diastolic fractional anisotropy (FA) was reduced in HCM compared to SARC+LVH- and controls (0.49±0.05 vs 0.52±0.04 vs 0.53±0.04, p=0.009), even after adjustment for differences in extracellular volume (ECV) (p=0.038). Both HCM and SARC+LVH- had segments with significantly reduced FA relative to controls (54% vs 25% vs 0%, p=0.002). Multiple repolarization parameters were prolonged in HCM and SARC+LVH-, with corrected JT interval (JTc) being most significant (354±42ms vs 356±26ms vs 314±26ms, p=0.002). Among SARC+LVH-, JTc duration correlated negatively with mean FA (r=-0.6, p=0.002). In HCM, the JTc interval showed a stronger association with ECV (r=0.6 p=0.019) than FA (r=-0.1 p=0.72). JTc discriminated SARC+LVH- from controls (Area-under-the-receiver-operator-curve 0.88, CI 0.76-1.00, p<0.001), and in HCM correlated with the ESC HCM sudden cardiac death risk score (r=0.5, p=0.014).

Conclusion: Low diastolic FA, suggestive of myocardial disarray, is present in both SARC+LVH- and HCM. Low FA and raised ECV were associated with repolarization prolongation. Myocardial disarray assessment using DT-CMR and repolarization parameters such as the JTc interval demonstrate significant potential as markers of disease activity in HCM.

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肥厚型心肌病各期的心肌畸形和纤维化与心律失常风险的心电图标志物有关。

背景：心肌错乱是肥厚型心肌病（HCM）的早期特征，也是室性心律失常的基质，但肥厚前肉瘤变异携带者（SARC+LVH-）的心肌错乱特征却不明显：利用弥散张量心脏磁共振（DT-CMR），我们评估了 SARC+LVH- 和 HCM 患者的心肌混乱和纤维化情况，并评估了微结构改变与心律失常风险相关心电图（ECG）参数之间的关系：对 62 名患者（24 名 SARC+LVH-、24 名 HCM 和 14 名匹配对照）进行了多参数 CMR 评估，包括刺激回波采集模式 (STEAM) DT-CMR，以及盲法定量 12 导联心电图分析：结果：与 SARC+LVH- 和对照组相比，HCM 的平均舒张分数各向异性（FA）降低（0.49±0.05 vs 0.52±0.04 vs 0.53±0.04，p=0.009），即使调整了细胞外容积（ECV）的差异（p=0.038）。与对照组相比，HCM 和 SARC+LVH- 均有 FA 显著降低的节段（54% vs 25% vs 0%，p=0.002）。在 HCM 和 SARC+LVH- 中，多个复极化参数延长，其中校正 JT 间期（JTc）最明显（354±42ms vs 356±26ms vs 314±26ms，P=0.002）。在 SARC+LVH- 中，JTc 持续时间与平均 FA 呈负相关（r=-0.6，p=0.002）。在 HCM 中，JTc 间期与 ECV（r=0.6，p=0.019）的相关性强于 FA（r=-0.1，p=0.72）。JTc 可将 SARC+LVH- 与对照组区分开来（接收器-操作者-曲线下面积 0.88，CI 0.76-1.00，p 结论：SARC+LVH-和HCM均存在舒张期低FA，提示心肌紊乱。低FA和ECV升高与复极化延长有关。使用 DT-CMR 评估心肌错乱和 JTc 间期等复极化参数显示出作为 HCM 疾病活动性标志物的巨大潜力。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

European Heart Journal - Cardiovascular Imaging CARDIAC & CARDIOVASCULAR SYSTEMS-

CiteScore

11.60

自引率

9.70%

发文量

708

审稿时长

4-8 weeks

期刊介绍： European Heart Journal – Cardiovascular Imaging is a monthly international peer reviewed journal dealing with Cardiovascular Imaging. It is an official publication of the European Association of Cardiovascular Imaging, a branch of the European Society of Cardiology. The journal aims to publish the highest quality material, both scientific and clinical from all areas of cardiovascular imaging including echocardiography, magnetic resonance, computed tomography, nuclear and invasive imaging. A range of article types will be considered, including original research, reviews, editorials, image focus, letters and recommendation papers from relevant groups of the European Society of Cardiology. In addition it provides a forum for the exchange of information on all aspects of cardiovascular imaging.