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复发性特发性室颤的管理:一例从诊断到成功消融的病例报告

Management of recurrent idiopathic ventricular fibrillation: a case report from diagnosis to successful ablation.

作者信息

Adamek Jan, Jiravsky Otakar, Ranic Ivan, Chovancik Jan, Kautzner Josef

机构信息

Department of Cardiology, Cardiocentre, Agel Hospital Trinec-Podlesi, Konska 453, 739 61 Trinec, Czech Republic.

Faculty of Medicine, University of Ostrava, Syllabova 19, 703 00 Ostrava, Czech Republic.

出版信息

Eur Heart J Case Rep. 2025 May 16;9(6):ytaf247. doi: 10.1093/ehjcr/ytaf247. eCollection 2025 Jun.

Abstract

BACKGROUND

Idiopathic ventricular fibrillation (IVF) is a rare cause of sudden cardiac death and is a diagnosis of exclusion. With the availability of genetic testing, this condition is becoming increasingly rare. Nevertheless, in some cases, no identifiable cause is found. Management of recurrent IVF episodes poses a significant clinical challenge, often requiring advanced interventional approaches.

CASE SUMMARY

We present a 43-year-old male with a history of out-of-hospital cardiac arrest due to VF in 2015. Despite extensive examinations, including normal coronary angiography, cardiac MRI, and genetic testing, no underlying aetiology was identified. The patient received an implantable cardioverter-defibrillator (ICD) for secondary prevention. After an 8-year arrhythmia-free period, he experienced recurrent ICD shocks in 2023. Repeated diagnostics, including MRI and genetic testing, yielded inconclusive results. An electrophysiological study revealed abnormalities in the Purkinje fibre network, including a focal source within the conduction system and a localized scar in the lower mid-left ventricular septum. Radiofrequency ablation targeting these areas successfully terminated the electrical storm.

DISCUSSION

This case highlights the complexities in diagnosing and managing IVF, demonstrating a strong association between the Purkinje fibre network abnormalities in arrhythmogenesis. It underscores the importance of electrophysiological studies and catheter ablation in refractory cases, even when advanced imaging and genetic testing fail to reveal a clear aetiology.

CONCLUSION

In patients with recurrent IVF refractory to conventional management, targeted ablation of Purkinje-related triggers not only terminates the storm, but provides durable rhythm control, as illustrated by our 8-month follow-up.

摘要

背景

特发性室颤(IVF)是心脏性猝死的罕见原因,是一种排除性诊断。随着基因检测技术的应用,这种疾病越来越少见。然而,在某些情况下,仍找不到明确病因。复发性IVF发作的管理面临重大临床挑战,通常需要先进的介入方法。

病例摘要

我们报告一名43岁男性,有2015年因室颤导致院外心脏骤停的病史。尽管进行了广泛检查,包括冠状动脉造影正常、心脏磁共振成像(MRI)和基因检测,但未发现潜在病因。该患者接受了植入式心脏复律除颤器(ICD)用于二级预防。在经历了8年无心律失常期后,他于2023年再次出现ICD电击。包括MRI和基因检测在内的反复诊断结果均不明确。电生理研究显示浦肯野纤维网络存在异常,包括传导系统内的一个局灶性起源点和左心室中下部间隔的一个局限性瘢痕。针对这些区域的射频消融成功终止了电风暴。

讨论

本病例突出了IVF诊断和管理的复杂性,表明浦肯野纤维网络异常与心律失常发生之间存在密切关联。它强调了在难治性病例中电生理研究和导管消融的重要性,即使先进的影像学检查和基因检测未能揭示明确病因。

结论

对于常规治疗难治的复发性IVF患者,如我们8个月的随访所示,靶向消融与浦肯野相关的触发因素不仅能终止电风暴,还能提供持久的节律控制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e0c/12142309/f4fa611e6e4f/ytaf247f1.jpg

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