Ventricular stimulation in patients with myotonic dystrophy type 1 may not predict future ventricular arrhythmias

IF 2.5 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
Lukasz Cerbin MD , Amneet Sandhu MD, MSc , Michael Rosenberg MD , Christopher Barrett MD , Rafay Sabzwari MD , Lohit Garg MD , Alexis Tumolo MD , Wendy Tzou MD , Paul Varosy MD , Johannes Von Alvensleben MD , Matthew Zipse MD , Ryan Aleong MD
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引用次数: 0

Abstract

Background

Myotonic dystrophy type 1 (DM1) is associated with progressive conduction disease. Furthermore, DM1 patients are at risk ventricular arrhythmias (VAs), although prediction remains difficult. The 2022 Heart Rhythm Expert Consensus Statement gives a IIb recommendation to the use of electrophysiology study (EPS) to risk-stratify patients for VAs. The utility of EPS in predicting the development of VAs, however, has not been explored in this patient population.

Objective

The study sought to examine the natural history of DM1 patients with positive and negative ventricular stimulation (v-stim) during EPS.

Methods

Patients with a history of DM1 undergoing EPS with associated v-stim from 2008 to present were retrospectively identified.

Results

From 2008 to 2022, 26 consecutive DM1 patients presented for EPS with v-stim. Four v-stim protocols were positive for sustained or hemodynamically significant ventricular tachycardia (VT), one of which was induced with 600 doubles, the others with triple extrastimuli. A total of 22 of 26 subjects received a device implant, with 18 receiving permanent pacemakers and 4 implantable cardioverter-defibrillators. All 4 of the patients with positive v-stims underwent ICD implantation. After a mean of 5.7 years of follow-up, 7 patients had sustained VT, 6 of whom had negative v-stims. Of the 4 patients with positive v-stims, only 1 developed sustained VT in follow-up. Other than baseline QT interval at time of EPS, no baseline characteristics were significantly different between patients with and without subsequent VT.

Conclusion

In this single center, v-stim in DM1 patients did not predict clinical VAs, as a vast majority of DM1 patients who developed VAs had negative v-stims.
1 型肌营养不良症患者的心室刺激可能无法预测未来的室性心律失常
背景1型肌营养不良症(DM1)与进行性传导疾病有关。此外,DM1 患者还存在室性心律失常(VAs)的风险,但预测仍很困难。2022 年《心脏节律专家共识声明》(The 2022 Heart Rhythm Expert Consensus Statement)建议使用电生理学研究(EPS)对室性心律失常患者进行风险分级,建议等级为 IIb。方法回顾性鉴定 2008 年至今接受 EPS 并伴有 v-stim 的 DM1 患者。结果从 2008 年到 2022 年,连续有 26 例 DM1 患者接受 EPS 并伴有 v-stim。四项v-stim方案对持续性或血流动力学显著性室性心动过速(VT)呈阳性反应,其中一项用600倍频诱导,其他则用三倍外刺激诱导。26 名受试者中共有 22 人接受了设备植入,其中 18 人接受了永久起搏器,4 人接受了植入式心律转复除颤器。所有 4 名 V-stims 阳性患者都接受了 ICD 植入术。在平均 5.7 年的随访后,7 名患者出现持续 VT,其中 6 名患者的 v-stims 呈阴性。在 4 名 v-stims 呈阳性的患者中,只有 1 人在随访期间出现持续性 VT。除 EPS 时的基线 QT 间期外,随后出现 VT 和未出现 VT 的患者的基线特征无显著差异。
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来源期刊
Heart Rhythm O2
Heart Rhythm O2 Cardiology and Cardiovascular Medicine
CiteScore
3.30
自引率
0.00%
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0
审稿时长
52 days
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