[无症状渠道病:风险分层和初级预防]。

Assem Aweimer, Andreas Mügge, Ibrahim Akin, Ibrahim El-Battrawy
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引用次数: 0

摘要

一般来说,无症状的通道病患者心源性猝死(SCD)的风险增加,这是由于编码离子通道的基因的致病性变异导致病理性离子电流。通道病变包括长qt综合征(LQTS)、Brugada综合征(BrS)、儿茶酚胺能多形性室性心动过速(CPVT)和短qt综合征(SQTS)。除了患者的临床表现、病史和临床检查外,主要的诊断工具是心电图和基因检测,以确定已知的基因突变。早期和正确的诊断以及进一步的患病个体及其亲属的风险分层对预后至关重要。最近可用的LQTS和BrS风险评分计算器可以准确估计SCD风险。目前尚不清楚这些技术在多大程度上改善了患者对植入式心律转复除颤器(ICD)系统治疗的选择。在大多数情况下,以避免触发因素(通常是药物或压力情况)的形式对无症状患者开始基本治疗就足够了,并有助于降低风险。此外,还有其他降低风险的预防措施,如长期使用非选择性β受体阻滞剂(用于LQTS和CPVT)或美西汀用于LQTS3。患者及其家属应转诊到专科门诊进行初级预防意义上的个体风险分层。
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[Asymptomatic channelopathies : Risk stratification and primary prophylaxis].

In general, asymptomatic patients with channelopathies are at increased risk of sudden cardiac death (SCD), due to pathogenic variants in genes encoding ion channels that result in pathological ion currents. Channelopathies include long-QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and short-QT syndrome (SQTS). In addition to the patient's clinical presentation, history and clinical tests, the main diagnostic tools are electrocardiography and genetic testing to identify known gene mutations. Early and correct diagnosis as well as further risk stratification of affected individuals and their relatives are paramount for prognosis. The recent availability of risk score calculators for LQTS and BrS allows SCD risk to be accurately estimated. The extent to which these improve patient selection for treatment with an implantable cardioverter-defibrillator (ICD) system is currently unknown. In most cases, initiation of basic therapy in asymptomatic patients in the form of avoidance of triggers, which are usually medication or stressful situations, is sufficient and contributes to risk reduction. In addition, there are other risk-reducing prophylactic measures, such as permanent medication with nonselective β‑ blockers (for LQTS and CPVT) or mexiletine for LQTS3. Patients and their family members should be referred to specialized outpatient clinics for individual risk stratification in the sense of primary prophylaxis.

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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
期刊最新文献
[Controversies in rhythmology]. [Atrial fibrillation in combination with severe mitral regurgitation : Which should be treated first, the atrial fibrillation or the mitral valve?] [Initial ablation of atrial fibrillation-Is pulmonary vein isolation sufficient? : Pro and contra]. [(LV)EF is a poor predictor of sudden cardiac death : Pro/Contra]. Erratum zu: Herzrhythmusstörungen bei Erwachsenen mit angeborenen Herzfehlern.
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