Brugada syndrome and neurally mediated susceptibility.

Nicolino Patruno, Daniele Pontillo, Renato Anastasi, Lorenzo Sunseri, Luigi Giamundo, Giovanni Ruggeri
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Abstract

The risk of sudden death in patients with Brugada syndrome (BS) is still unclear. Moreover, particular clinical conditions may have a confounding effect on the diagnostic and therapeutic approach. We report the case of a 27-year-old man with a clinical history of suspected neurally mediated syncope and typical ECG features of BS. The tilt table test showed a type I, mixed, positive response. The electrophysiological study (EPS) disclosed a peculiar ventricular irritability with the induction of a life-threatening arrhythmia. After the implantation of a cardioverter-defibrillator an episode of ventricular fibrillation during sleep at night was correctly identified and treated by the device. The association between neurally mediated susceptibility and the typical ECG abnormalities of BS is not an unexpected event in young subjects. The misjudgment of the pathophysiological mechanism of syncopal episodes may lead, on one hand, to overlook the risk of sudden death and, on the other, to pursue inappropriate therapeutic measures. The application of a tailored diagnostic work-up based on currently available guidelines may be useful to overcome the clinical and therapeutic dilemma.

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Brugada综合征与神经介导的易感性。
Brugada综合征(BS)患者猝死的风险尚不清楚。此外,特殊的临床条件可能对诊断和治疗方法产生混淆作用。我们报告的情况下,27岁的男子临床病史怀疑神经介导晕厥和典型的心电图特征的BS。倾斜台试验显示I型混合阳性反应。电生理研究(EPS)揭示了一种特殊的心室激惹与诱发危及生命的心律失常。在植入心律转复除颤器后,该装置可正确识别并治疗夜间睡眠时发生的心室颤动。神经介导的易感性与BS的典型ECG异常之间的关联在年轻受试者中并非意外事件。对晕厥发作病理生理机制的错误判断,一方面可能导致忽视猝死的危险,另一方面可能导致采取不适当的治疗措施。根据目前可用的指导方针,应用量身定制的诊断检查可能有助于克服临床和治疗困境。
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