Geraghty Lucy, Santangeli Pasquale, Tedrow Usha B, Shivkumar Kalyanam, Kumar Saurabh
Department of Cardiology, Westmead Hospital, Sydney, NSW, Australia.
Electrophysiology Section, Cardiovascular Division, Hospital of the University of Pennsylvania, PA, USA.
Heart Lung Circ. 2019 Jan;28(1):123-133. doi: 10.1016/j.hlc.2018.10.005. Epub 2018 Oct 15.
Cardiac electrical storm (ES) is characterised by three or more discrete episodes of ventricular arrhythmia within 24hours, or incessant ventricular arrhythmia for more than 12hours. ES is a distinct medical emergency that portends a significant increase in mortality risk and often presages progressive heart failure. ES is also associated with psychological morbidity from multiple implanted cardioverter defibrillator (ICD) shocks and exponential health resource utilisation. Up to 30% of ICD recipients may experience storm in follow-up, with the risk higher in patients with a secondary prevention ICD indication. Storm recurs in a high proportion of patients after an initial episode, and multiple storm clusters may occur in follow-up. The mechanism of storm remains elusive but is likely influenced by a complex interplay of inciting triggers (e.g., ischaemia, electrolyte disturbances), with autonomic perturbations acting on a vulnerable structural and electrophysiologic substrate. Triggers can be identified only in a minority of patients. An emergent treatment approach is warranted, if possible with emergent transfer to a high-volume centre for ventricular arrhythmia management with a multi-modality approach including ICD reprogramming, sympathetic blockade (sedation, intubation, ventilation, beta blockers), and anti-arrhythmic drugs, and adjunctive intervention techniques, such as catheter ablation and neuraxial modulation (e.g., thoracic epidural anaesthesia, stellate ganglion block). Outcomes of catheter ablation of ES are excellent with resolution of storm in over 90% of patients at 1year with a low complication rate (∼2%). ES may occur in the absence of structural heart disease in the context of channelopathies, Brugada syndrome, early repolarisation and premature ventricular contraction-induced ventricular fibrillation. There are unique treatment approaches to these conditions that must be recognised. This state-of-the-art review will summarise the incidence, mechanism, and multi-modality treatment of ES in the contemporary era.
心脏电风暴（ES）的特征是24小时内出现三次或更多次离散的室性心律失常发作，或持续性室性心律失常超过12小时。ES是一种独特的医疗急症，预示着死亡风险显著增加，且常预示着进行性心力衰竭。ES还与多次植入式心律转复除颤器（ICD）电击导致的心理疾病以及指数级增长的医疗资源利用相关。高达30%的ICD植入者在随访中可能经历电风暴，二级预防ICD适应症患者的风险更高。初次发作后，很大一部分患者会复发电风暴，随访中可能会出现多个电风暴群集。电风暴的机制仍然难以捉摸，但可能受到诱发触发因素（如缺血、电解质紊乱）复杂相互作用的影响，自主神经紊乱作用于易损的结构和电生理基质。只有少数患者能识别出触发因素。如果可能，应采用紧急治疗方法，紧急转诊至大容量中心，采用多模式方法管理室性心律失常，包括ICD重新编程、交感神经阻滞（镇静、插管、通气、β受体阻滞剂）、抗心律失常药物以及辅助干预技术，如导管消融和神经轴调制（如胸段硬膜外麻醉、星状神经节阻滞）。ES导管消融的效果极佳，超过90%的患者在1年时电风暴得到缓解，并发症发生率较低（约2%）。在通道病、Brugada综合征、早期复极和室性早搏诱发的室颤背景下，ES可能在无结构性心脏病的情况下发生。对于这些情况，必须认识到有独特的治疗方法。这篇最新综述将总结当代ES的发病率、机制和多模式治疗。
