SEE PAGE 148 B rugada syndrome (BrS) is an inherited ion channelopathy characterized by a coved-type ST-segment in the right precordial leads predisposing to ventricular arrhythmias and sudden death (1). Clinical expressivity varies ranging from a complete lack of symptoms in 72% of patients to sudden death as an initial presentation in 7% (2). Followup results from the earliest patient experience exposed dismal results wherein 62% of patients who presented with sudden cardiac death had recurrence of ventricular arrhythmias (3). With the 25th anniversary of the original description of BrS upon us, it is appropriate to revisit progress made, particularly as it pertains to patient outcomes and the prevention of sudden cardiac death. Despite significant advances in our understanding of BrS from a cellular and mechanistic standpoint, as practitioners we continue to struggle with identifying the asymptomatic patient at risk for sudden cardiac death, specifically as it pertains to primary prevention implantable cardioverter-defibrillator (ICD). The case for risk stratification, particularly in the pediatric patient, is demonstrated by the fact that most pediatric patients diagnosed with BrS are asymptomatic at presentation. In a study by Harris et al. (4), the most common initial presentation of BrS was a family history (47%), followed by an incidental electrocardiographic finding (25%), syncope (14%), arrhythmia (13%), and aborted sudden cardiac death (1%); therefore, 72% of pediatric patients diagnosed with BrS were asymptomatic at presentation. As with many cardiovascular diseases in the young, the paucity of robust clinical research has forced the

中文翻译：

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小儿 Brugada 综合征

参见第 148 页 B rugada 综合征 (BrS) 是一种遗传性离子通道病，其特征是右心前导联中的凹型 ST 段，易导致室性心律失常和猝死 (1)。临床表现各不相同，从 72% 的患者完全没有症状到 7% 的患者以猝死为初始表现 (2)。最早患者经历的随访结果暴露了令人沮丧的结果，其中 62% 的心脏性猝死患者出现室性心律失常复发 (3)。随着对 B​​rS 的原始描述出现 25 周年，重新审视已取得的进展是适当的，特别是因为它与患者结果和预防心源性猝死有关。尽管我们从细胞和机械的角度对 BrS 的理解取得了重大进展，作为从业者，我们继续努力识别有心脏猝死风险的无症状患者，特别是因为它与一级预防植入式心律转复除颤器 (ICD) 有关。大多数诊断为 BrS 的儿科患者在就诊时没有症状，这证明了风险分层的情况，特别是在儿科患者中。在哈里斯等人的一项研究中。(4) BrS 最常见的初始表现是家族史 (47%)，其次是偶然的心电图发现 (25%)、晕厥 (14%)、心律失常 (13%) 和流产的心源性猝死 (1) %); 因此，72% 诊断为 BrS 的儿科患者在就诊时没有症状。与年轻人的许多心血管疾病一样，缺乏强有力的临床研究迫使