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多中心 Brugada 综合征心律失常事件研究中的发热相关心律失常事件。

Fever-related arrhythmic events in the multicenter Survey on Arrhythmic Events in Brugada Syndrome.

机构信息

Department of Cardiology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARD-Heart); Pediatric Arrhythmias, Electrophysiology and Sudden Death Unit, Cardiology Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.

出版信息

Heart Rhythm. 2018 Sep;15(9):1394-1401. doi: 10.1016/j.hrthm.2018.04.007. Epub 2018 Apr 9.

Abstract

BACKGROUND

The literature on fever-related arrhythmic events (AEs) in Brugada syndrome (BrS) is currently limited to few case reports and small series.

OBJECTIVE

The present study aimed to describe the characteristics of fever-related AE in a large cohort of patients with BrS.

METHODS

The Survey on Arrhythmic Events in Brugada Syndrome is a multicenter study on 678 patients with BrS with first AE documented at the time of aborted cardiac arrest (n = 426) or after prophylactic implantable cardioverter-defibrillator implantation (n = 252).

RESULTS

In 35 of 588 patients (6%) with available information, the AE occurred during a febrile illness. Most of the 35 patients were male (80%), Caucasian (83%), and proband (70%). The mean age at the time of AE was 29 ± 24 years (range 0.3-76 years). Most patients (80%) presented with aborted cardiac arrest and 6 (17%) with arrhythmic storm. Family history of sudden death, history of syncope, and spontaneous type 1 Brugada electrocardiogram were noted in 17%, 40%, and 71% of patients, respectively. Ventricular fibrillation was induced at electrophysiology study in 9 of 19 patients (47%). An SCN5A mutation was found in 14 of 28 patients (50%). The highest proportion of fever-related AE was observed in the pediatric population (age <16 years), with a disproportionally higher event rate in the very young (age 0-5 years) (65%). Males were involved in all age groups and females only in the pediatric and elderly groups. Fever-related AE affected 17 Caucasians aged <24 years, but no Asians aged <24 years.

CONCLUSION

The risk of fever-related AE in BrS markedly varies according to age group, sex, and ethnicity. Taking these factors into account could help the clinical management of patients with BrS with fever.

摘要

背景

目前有关发热相关心律失常事件(AEs)在Brugada 综合征(BrS)中的文献仅限于少数病例报告和小系列研究。

目的

本研究旨在描述发热相关 AE 在 BrS 大患者队列中的特征。

方法

心律失常事件在 Brugada 综合征中的调查是一项针对 678 例 BrS 患者的多中心研究,这些患者在心脏骤停发作时(n = 426)或在预防性植入式心脏复律除颤器植入后(n = 252)首次出现 AE。

结果

在 588 例可提供信息的患者中,有 35 例(6%)的 AE 发生在发热性疾病期间。35 例患者中大多数为男性(80%)、白种人(83%)和先证者(70%)。AE 发生时的平均年龄为 29 ± 24 岁(范围 0.3-76 岁)。大多数患者(80%)表现为心脏骤停发作,6 例(17%)为心律失常风暴。17%、40%和 71%的患者分别有家族性猝死史、晕厥史和自发性 1 型 Brugada 心电图。在 19 例患者中的 9 例(47%)行电生理研究时可诱发室颤。在 28 例患者中发现 14 例(50%)存在 SCN5A 突变。发热相关 AE 的最高比例发生在儿科人群(年龄 <16 岁),其中年龄很小的(0-5 岁)的发生率更高(65%)。男性参与了所有年龄组,而女性仅参与了儿科和老年组。发热相关 AE 影响了 17 名年龄 <24 岁的白种人,但没有亚洲人年龄 <24 岁。

结论

发热相关 AE 在 BrS 中的风险明显随年龄组、性别和种族而异。考虑这些因素可以帮助管理发热的 BrS 患者。

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