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两种疾病的故事:长 QT 综合征和 Brugada 综合征的历史。

A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome.

机构信息

Tel Aviv Sourasky Medical Center and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.

Tel Aviv Sourasky Medical Center and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.

出版信息

J Am Coll Cardiol. 2016 Jan 5;67(1):100-8. doi: 10.1016/j.jacc.2015.10.020.

DOI:10.1016/j.jacc.2015.10.020
PMID:26764071
Abstract

The Brugada syndrome (BrS) and long-QT syndrome (LQTS) present as congenital or acquired disorders with diagnostic electrocardiograms (ST-segment elevation and prolonged QT interval, respectively) and increased risk for malignant arrhythmias. Our understanding of the 2 disease forms (congenital vs. acquired) differs. A female patient on quinidine for atrial fibrillation who develops ventricular fibrillation is diagnosed with "acquired LQTS" and is discharged with no therapy other than instructions to avoid QT-prolonging medications. In contrast, an asymptomatic male patient who develops a Brugada electrocardiogram on flecainide is diagnosed with "asymptomatic BrS" and could be referred for an electrophysiological evaluation that could result in defibrillator implantation. The typical patient undergoing defibrillator implantation for BrS is asymptomatic but has a Brugada electrocardiogram provoked by a drug. The authors describe how the histories of LQTS and BrS went through the same stages, but in different sequences, leading to different conclusions.

摘要

Brugada 综合征(BrS)和长 QT 综合征(LQTS)分别表现为先天性或获得性疾病,其诊断性心电图分别为 ST 段抬高和 QT 间期延长,并伴有恶性心律失常的风险增加。我们对这两种疾病形式(先天性与获得性)的理解存在差异。一名因心房颤动而服用奎尼丁的女性患者发生心室颤动,被诊断为“获得性 LQTS”,除了避免使用延长 QT 间期的药物外,无需其他治疗即可出院。相比之下,一名无症状的男性患者在服用氟卡尼时出现 Brugada 心电图,被诊断为“无症状 BrS”,可能需要进行电生理评估,这可能导致植入除颤器。典型的因 BrS 而植入除颤器的患者无症状,但因药物引起 Brugada 心电图。作者描述了 LQTS 和 BrS 的病史是如何经历相同的阶段,但顺序不同,导致不同的结论。

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