Arrhythmias and cardiac MRI associations in patients with established cardiac dystrophinopathy

IF 2.8 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Open Heart Pub Date : 2024-04-01 DOI:10.1136/openhrt-2023-002590

John Bourke, Margaret Tynan, Hannah Stevenson, Leslie Bremner, Oscar Gonzalez-Fernandez, Adam K McDiarmid

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引用次数: 0

Abstract

Aims Some patients with cardiac dystrophinopathy die suddenly. Whether such deaths are preventable by specific antiarrhythmic management or simply indicate heart failure overwhelming medical therapies is uncertain. The aim of this prospective, cohort study was to describe the occurrence and nature of cardiac arrhythmias recorded during prolonged continuous ECG rhythm surveillance in patients with established cardiac dystrophinopathy and relate them to abnormalities on cardiac MRI. Methods and results A cohort of 10 patients (36.3 years; 3 female) with LVEF<40% due to Duchenne (3) or Becker muscular (4) dystrophy or Duchenne muscular dystrophy-gene carrying effects in females (3) were recruited, had cardiac MRI, ECG signal-averaging and ECG loop-recorder implants. All were on standard of care heart medications and none had prior history of arrhythmias. No deaths or brady arrhythmias occurred during median follow-up 30 months (range 13–35). Self-limiting episodes of asymptomatic tachyarrhythmia (range 1–29) were confirmed in 8 (80%) patients (ventricular only 2; ventricular and atrial 6). Higher ventricular arrhythmia burden correlated with extent of myocardial fibrosis (extracellular volume%, p=0.029; native T1, p=0.49; late gadolinium enhancement, p=0.49), but not with LVEF% (p=1.0) on MRI and atrial arrhythmias with left atrial dilatation. Features of VT episodes suggested various underlying arrhythmia mechanisms. Conclusions The overall prevalence of arrhythmias was low. Even in such a small sample size, higher arrhythmia counts occurred in those with larger scar burden and greater ventricular volume, suggesting key roles for myocardial stretch as well as disease progression in arrhythmogenesis. These features overlap with the stage of left ventricular dysfunction when heart failure also becomes overt. The findings of this pilot study should help inform the design of a definitive study of specific antiarrhythmic management in dystrophinopathy. Trial registration number [ISRCTN15622536][1]. All data relevant to the study are included in the article or uploaded as online supplemental information. [1]: /external-ref?link_type=ISRCTN&access_num=ISRCTN15622536

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已确诊的心脏肌营养不良症患者的心律失常与心脏磁共振成像的关系

目的一些心脏营养不良症患者会突然死亡。这种死亡是可以通过特定的抗心律失常治疗来预防，还是仅仅表明心脏衰竭无法承受药物治疗，目前尚不确定。这项前瞻性队列研究旨在描述已确诊的心肌营养不良症患者在长时间连续心电图节律监测期间记录到的心律失常的发生率和性质，并将其与心脏磁共振成像的异常情况联系起来。方法和结果共招募了 10 例因杜氏肌营养不良（3 例）或贝克尔肌营养不良（4 例）或女性杜氏肌营养不良基因携带效应（3 例）导致 LVEF<40% 的患者（36.3 岁；3 例女性），他们都接受了心脏核磁共振成像、心电图信号平均化和心电图环形记录器植入。所有患者均服用标准的心脏药物，无心律失常病史。中位随访时间为 30 个月（13-35 个月），无死亡或缓慢性心律失常发生。8例（80%）患者（仅室性 2例；室性和房性 6例）被确诊为无症状快速性心律失常的自限性发作（范围 1-29）。较高的室性心律失常负荷与心肌纤维化程度相关（细胞外体积%，p=0.029；原生 T1，p=0.49；晚期钆增强，p=0.49），但与 MRI 上的 LVEF% 无关（p=1.0），与左心房扩张的房性心律失常无关。VT 发作的特征提示了各种潜在的心律失常机制。结论心律失常的总体发病率较低。即使样本量很小，瘢痕负荷较大和心室容积较大的患者心律失常发生率也较高，这表明心肌伸展和疾病进展在心律失常发生中起着关键作用。这些特征与左心室功能障碍阶段重叠，此时心力衰竭也变得明显。这项试验性研究的结果将有助于为肌营养不良症特定抗心律失常治疗的最终研究设计提供参考。试验注册号[ISRCTN15622536][1]。与该研究相关的所有数据均包含在文章中或作为在线补充信息上传。[1]:/external-ref?link_type=ISRCTN&access_num=ISRCTN15622536

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来源期刊

Open Heart CARDIAC & CARDIOVASCULAR SYSTEMS-

CiteScore

4.60

自引率

3.70%

发文量

145

审稿时长

20 weeks

期刊介绍： Open Heart is an online-only, open access cardiology journal that aims to be “open” in many ways: open access (free access for all readers), open peer review (unblinded peer review) and open data (data sharing is encouraged). The goal is to ensure maximum transparency and maximum impact on research progress and patient care. The journal is dedicated to publishing high quality, peer reviewed medical research in all disciplines and therapeutic areas of cardiovascular medicine. Research is published across all study phases and designs, from study protocols to phase I trials to meta-analyses, including small or specialist studies. Opinionated discussions on controversial topics are welcomed. Open Heart aims to operate a fast submission and review process with continuous publication online, to ensure timely, up-to-date research is available worldwide. The journal adheres to a rigorous and transparent peer review process, and all articles go through a statistical assessment to ensure robustness of the analyses. Open Heart is an official journal of the British Cardiovascular Society.