Pierre Groussin, Melvyn Dezecot, Donovan Decaudin, Dominique Pavin, Sandro Ninni, Cédric Klein, Pierre Ollitrault, Laure Champ Rigot, Jonaz Font, Antoine Da Costa, Jean-Marc Sellal, Néfissa Hammache, Alexandre Salaun, Charles Guenancia, Philippe Mabo, Karim Benali, Raphaël Martins
{"title":"Electrical Storm in Patients With Hypertrophic Cardiomyopathy.","authors":"Pierre Groussin, Melvyn Dezecot, Donovan Decaudin, Dominique Pavin, Sandro Ninni, Cédric Klein, Pierre Ollitrault, Laure Champ Rigot, Jonaz Font, Antoine Da Costa, Jean-Marc Sellal, Néfissa Hammache, Alexandre Salaun, Charles Guenancia, Philippe Mabo, Karim Benali, Raphaël Martins","doi":"10.1016/j.jacep.2025.06.035","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Therapeutic strategies for electrical storm (ES) in patients with hypertrophic cardiomyopathy (HCM) are not well defined, yet this population is at risk for ventricular arrhythmias (VAs).</p><p><strong>Objective: </strong>The aim of this study was to analyze acute management, in-hospital mortality, and long-term outcomes of patients with HCM experiencing ES.</p><p><strong>Methods: </strong>This multicenter study retrospectively enrolled patients with ES complicating HCM between 2017 and 2023 in 6 tertiary centers.</p><p><strong>Results: </strong>Twenty-three patients were included (mean age 61.5 ± 15.2 years, 73.9% male, left ventricular ejection fraction 50% ± 14%). Five patients (22%) had an apical aneurysm. Ten (43.4%) patients had a history of VA, including 3 with previous ES. The majority of VAs were monomorphic ventricular tachycardias (18 patients [78%]). Most patients received amiodarone (n = 20 [87%]) and beta-blockers (n = 16 [70%]). For 10 patients (44%), a radiofrequency catheter ablation was performed (including 2 endo-epicardial ablations), resulting in negative programmed ventricular stimulation in 4 patients. During their hospital management, 8 patients experienced recurrences of VA (2 [25%] previously ablated and 6 [75%] nonablated). Two (9%) patients died during the index hospitalization and 4 other patients died following discharge (median follow-up 18 months). Seven patients (30%) experienced recurrences of arrhythmias, including 4 who had undergone ablation. Radiofrequency ablation was not associated with better outcomes (recurrence rate or overall survival) in this study.</p><p><strong>Conclusions: </strong>ES in HCM frequently resolves using antiarrhythmic drugs. Many patients will present with VA recurrences. Long-term outcomes were poor, and one-fourth of the patients ended up dying at 18 months. Further studies are warranted to better analyze radiofrequency outcomes in this specific population.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":7.7000,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"JACC. Clinical electrophysiology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.jacep.2025.06.035","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Therapeutic strategies for electrical storm (ES) in patients with hypertrophic cardiomyopathy (HCM) are not well defined, yet this population is at risk for ventricular arrhythmias (VAs).
Objective: The aim of this study was to analyze acute management, in-hospital mortality, and long-term outcomes of patients with HCM experiencing ES.
Methods: This multicenter study retrospectively enrolled patients with ES complicating HCM between 2017 and 2023 in 6 tertiary centers.
Results: Twenty-three patients were included (mean age 61.5 ± 15.2 years, 73.9% male, left ventricular ejection fraction 50% ± 14%). Five patients (22%) had an apical aneurysm. Ten (43.4%) patients had a history of VA, including 3 with previous ES. The majority of VAs were monomorphic ventricular tachycardias (18 patients [78%]). Most patients received amiodarone (n = 20 [87%]) and beta-blockers (n = 16 [70%]). For 10 patients (44%), a radiofrequency catheter ablation was performed (including 2 endo-epicardial ablations), resulting in negative programmed ventricular stimulation in 4 patients. During their hospital management, 8 patients experienced recurrences of VA (2 [25%] previously ablated and 6 [75%] nonablated). Two (9%) patients died during the index hospitalization and 4 other patients died following discharge (median follow-up 18 months). Seven patients (30%) experienced recurrences of arrhythmias, including 4 who had undergone ablation. Radiofrequency ablation was not associated with better outcomes (recurrence rate or overall survival) in this study.
Conclusions: ES in HCM frequently resolves using antiarrhythmic drugs. Many patients will present with VA recurrences. Long-term outcomes were poor, and one-fourth of the patients ended up dying at 18 months. Further studies are warranted to better analyze radiofrequency outcomes in this specific population.
期刊介绍：
JACC: Clinical Electrophysiology is one of a family of specialist journals launched by the renowned Journal of the American College of Cardiology (JACC). It encompasses all aspects of the epidemiology, pathogenesis, diagnosis and treatment of cardiac arrhythmias. Submissions of original research and state-of-the-art reviews from cardiology, cardiovascular surgery, neurology, outcomes research, and related fields are encouraged. Experimental and preclinical work that directly relates to diagnostic or therapeutic interventions are also encouraged. In general, case reports will not be considered for publication.
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