More than 30 years of Brugada syndrome: a critical appraisal of achievements and open issues.

Q4 Medicine Herzschrittmachertherapie und Elektrophysiologie Pub Date : 2024-03-01 Epub Date: 2023-12-12 DOI:10.1007/s00399-023-00983-y
Lars Eckardt, Christian Veltmann
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Abstract

Over the last three decades, what is referred to as Brugada syndrome (BrS) has developed from a clinical observation of initially a few cases of sudden cardiac death (SCD) in the absence of structural heart disease with ECG signs of "atypical right bundle brunch block" to a predominantly electrocardiographic, and to a lesser extent genetic, diagnosis. Today, BrS is diagnosed in patients without overt structural heart disease and a spontaneous Brugada type 1 ECG pattern regardless of symptoms. The diagnosis of BrS is less clear in those with an only transient or drug-induced type 1 Brugada pattern, but should be considered in the presence of an arrhythmic syncope, family history of BrS, or family history of sudden death. In addition to survived cardiac arrest, syncope is probably the single most decisive risk marker for future arrhythmias. For asymptomatic BrS, risk stratification remains challenging. General recommendations to lower the risk in BrS include avoidance of drugs/agents known to induce and/or increase right precordial ST-segment elevation, including treatment of fever with antipyretic drugs. Several ECG markers that have been associated with an increased risk of SCD have been incorporated into a recently published risk score for BrS. The aim of this article is to provide an overview of the status of risk stratification and to illustrate open issues und gaps in evidence in BrS.

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Brugada 综合征 30 多年:对成就和未决问题的批判性评估。
在过去的三十年中,所谓的 Brugada 综合征(BrS)已经从最初临床观察到的几例无结构性心脏病、心电图显示 "非典型右束束状传导阻滞 "的心脏性猝死(SCD)病例,发展为主要由心电图诊断,其次是遗传学诊断。如今,无明显结构性心脏病和自发性 Brugada 1 型心电图模式的患者,无论有无症状,均可诊断为 BrS。对于仅有一过性或药物诱发 1 型 Brugada 模式的患者,BrS 的诊断不太明确,但如果出现心律失常性晕厥、BrS 家族史或猝死家族史,则应考虑诊断 BrS。除了幸存的心脏骤停患者外,晕厥可能是未来发生心律失常的唯一最具决定性的风险标志。对于无症状的 BrS 患者,风险分层仍然具有挑战性。降低 BrS 风险的一般建议包括避免使用已知会诱发和/或增加右心前区 ST 段抬高的药物/制剂,包括使用退烧药治疗发热。一些与 SCD 风险增加相关的心电图指标已被纳入最近公布的 BrS 风险评分中。本文旨在概述风险分层的现状,并说明 BrS 的未决问题和证据差距。
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来源期刊
Herzschrittmachertherapie und Elektrophysiologie
Herzschrittmachertherapie und Elektrophysiologie Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.10
自引率
0.00%
发文量
76
期刊介绍: Mit wissenschaftlichen Original- und Übersichtsarbeiten, Berichten über moderne Operationstechniken und experimentelle Methoden ist die Zeitschrift Herzschrittmachertherapie + Elektrophysiologie ein Diskussionsforum für Themen wie: - Zelluläre Elektrophysiologie - Theoretische Elektrophysiologie - Klinische Elektrophysiologie - Angewandte Herzschrittmachertherapie - Bradykarde und tachykarde Herzrhythmusstörungen - Plötzlicher Herztod und Risikostratifikation - Elektrokardiographie - Elektromedizinische Technologie - Experimentelle und klinische Pharmakologie - Herzchirurgie bei Herzrhythmusstörungen Mitteilungen der Arbeitsgruppen Herzschrittmacher und Arrhythmie der Deutschen Gesellschaft für Kardiologie - Herz und Kreislaufforschung e.V. (DGK) sowie Stellungnahmen und praktische Hinweise runden das breite Spektrum dieser Zeitschrift ab. Interessensgebiete: Kardiologie, Herzschrittmachertherapie, Herzschrittmachertechnologie, klinische Elektrophysiologie
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