PDF(Pubmed)
Abstract：
UNASSIGNED: The Brugada syndrome (BrS) is an inherited disorder associated with the risk of ventricular fibrillation and sudden cardiac death (SCD). The current main therapy is an implantable cardioverter-defibrillator (ICD). However, the risk stratification and management of patients remain challenging. Here, we present a case of BrS representative of the pitfalls that clinicians may encounter in the management of Brugada patients in routine clinical practice.
UNASSIGNED: A 39-year-old man with BrS and recurring syncope was implanted with a subcutaneous ICD (S-ICD) (EMBLEM MRI S-ICD, Boston Scientific). Syncope recurred some months later. Subcutaneous ICD interrogation showed no arrhythmic events, but SMART Pass (high-pass filter) deactivation was noted. A query was sent to Boston Scientific clinical service, unveiling an extremely long asystolic pause as syncope determinant. Subcutaneous ICD was explanted and replaced by conventional single chamber ICD in the pre-pectoral region.
UNASSIGNED: Brugada syndrome patients with high-risk features are candidates for ICD implantation to prevent SCD. Recent evidence highlighted that symptomatic patients carry a substantially higher risk compared with asymptomatic ones. Syncope may represent a pivotal symptom in BrS patients, but young patients with Type 1 Brugada pattern may experience syncope other than from tachyarrhythmias. Subcutaneous ICD is an advisable option in young ICD recipients to avoid lifetime complication related to standard transvenous systems. However, S-ICD lacks pacing capabilities and, therefore, is not indicated when an anti-bradycardia system is needed. The diagnostic workup of syncope in Brugada patients may be ineffective in elucidating the underlying aetiology whose understanding is essential to offer a personalized therapeutic approach.
UNASSIGNED: A 39-year-old man with BrS and recurring syncope was implanted with a subcutaneous ICD (S-ICD) (EMBLEM MRI S-ICD, Boston Scientific). Syncope recurred some months later. Subcutaneous ICD interrogation showed no arrhythmic events, but SMART Pass (high-pass filter) deactivation was noted. A query was sent to Boston Scientific clinical service, unveiling an extremely long asystolic pause as syncope determinant. Subcutaneous ICD was explanted and replaced by conventional single chamber ICD in the pre-pectoral region.
UNASSIGNED: Brugada syndrome patients with high-risk features are candidates for ICD implantation to prevent SCD. Recent evidence highlighted that symptomatic patients carry a substantially higher risk compared with asymptomatic ones. Syncope may represent a pivotal symptom in BrS patients, but young patients with Type 1 Brugada pattern may experience syncope other than from tachyarrhythmias. Subcutaneous ICD is an advisable option in young ICD recipients to avoid lifetime complication related to standard transvenous systems. However, S-ICD lacks pacing capabilities and, therefore, is not indicated when an anti-bradycardia system is needed. The diagnostic workup of syncope in Brugada patients may be ineffective in elucidating the underlying aetiology whose understanding is essential to offer a personalized therapeutic approach.
摘要：
■Brugada综合征（BrS）是一种与心室纤颤和心源性猝死（SCD）风险相关的遗传性疾病。当前的主要治疗方法是植入式心脏复律除颤器（ICD）。然而,患者的风险分层和管理仍然具有挑战性.这里,我们介绍了一例BrS病例,说明临床医生在常规临床实践中处理Brugada患者时可能遇到的缺陷.
■一名39岁的患有BrS和复发性晕厥的男子被植入皮下ICD（S-ICD）（EMBLEMMRIS-ICD，波士顿科学公司）。几个月后晕厥复发。皮下ICD询问显示无心律失常事件,但注意到SMARTPass（高通滤波器）停用。向波士顿科学临床服务公司发送了一个查询，揭示了一个非常长的心搏暂停作为晕厥的决定因素。植入皮下ICD，并在胸前区域用常规单腔ICD代替。
■具有高风险特征的Brugada综合征患者是ICD植入以预防SCD的候选人。最近的证据强调，与无症状患者相比，有症状患者的风险要高得多。晕厥可能是BrS患者的关键症状，但患有1型Brugada模式的年轻患者可能会出现除快速性心律失常以外的晕厥。皮下ICD是年轻ICD接受者的明智选择，以避免与标准经静脉系统相关的终生并发症。然而,S-ICD缺乏起搏能力，因此,当需要抗心动过缓系统时，不指示。Brugada患者晕厥的诊断检查可能无法阐明潜在的病因，而这些病因的理解对于提供个性化的治疗方法至关重要。
■一名39岁的患有BrS和复发性晕厥的男子被植入皮下ICD（S-ICD）（EMBLEMMRIS-ICD，波士顿科学公司）。几个月后晕厥复发。皮下ICD询问显示无心律失常事件,但注意到SMARTPass（高通滤波器）停用。向波士顿科学临床服务公司发送了一个查询，揭示了一个非常长的心搏暂停作为晕厥的决定因素。植入皮下ICD，并在胸前区域用常规单腔ICD代替。
■具有高风险特征的Brugada综合征患者是ICD植入以预防SCD的候选人。最近的证据强调，与无症状患者相比，有症状患者的风险要高得多。晕厥可能是BrS患者的关键症状，但患有1型Brugada模式的年轻患者可能会出现除快速性心律失常以外的晕厥。皮下ICD是年轻ICD接受者的明智选择，以避免与标准经静脉系统相关的终生并发症。然而,S-ICD缺乏起搏能力，因此,当需要抗心动过缓系统时，不指示。Brugada患者晕厥的诊断检查可能无法阐明潜在的病因，而这些病因的理解对于提供个性化的治疗方法至关重要。
