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Brugada综合征与Brugada综合征拟表型。鉴别诊断的重要性。

Brugada syndrome and Brugada phenocopy. The importance of a differential diagnosis.

作者信息

Dendramis Gregory

机构信息

Cardiovascular Division, Department of Internal Medicine and Cardiovascular Diseases, University Hospital "Paolo Giaccone", Palermo, Italy.

出版信息

Int J Cardiol. 2016 May 1;210:25-7. doi: 10.1016/j.ijcard.2016.02.097. Epub 2016 Feb 17.

Abstract

To date Brugada syndrome (BrS) is considered a primary electrical heart disease and the diagnosis is based on precise clinical and electrocardiographic features. Many other diseases and conditions can lead to a Brugada-like ECG pattern but the vast majority of patients with BrS possess a structurally normal heart, which is consistent with the notion that this is a primary electrical heart disease. Presently, the terminology used in the literature to describe Brugada type 1 ECG pattern induced in patients without BrS is diverse and variable. Brugada phenocopies (BrP) are clinical entities that present with identical ECG patterns to those of true BrS but are elicited by various other clinical circumstances. They form a group of heterogeneous conditions that are perhaps the most difficult to differentiate from true congenital BrS due to identical ECG patterns and recently has been proposed an updated classification of conditions that may induce BrP and many criteria useful to differentiate BrP from BrS. A systematic diagnostic approach is crucial to avoid diagnostic errors that involve expenditure of time and resources, but above all it is useful to avoid to send patients without a real BrS to inopportune diagnostic and therapeutic paths that are sometimes burdened by considerable risks.

摘要

迄今为止, Brugada综合征(BrS)被认为是一种原发性心脏电疾病,其诊断基于精确的临床和心电图特征。许多其他疾病和情况可导致类似Brugada的心电图模式,但绝大多数BrS患者的心脏结构正常,这与该病是原发性心脏电疾病的观点一致。目前,文献中用于描述非BrS患者诱发的1型Brugada心电图模式的术语多样且多变。Brugada拟表型(BrP)是指临床实体,其心电图模式与真正的BrS相同,但由各种其他临床情况诱发。它们构成一组异质性疾病,由于心电图模式相同,可能是最难与真正的先天性BrS区分的,最近有人提出了可能诱发BrP的疾病的更新分类以及许多有助于区分BrP与BrS的标准。系统的诊断方法对于避免涉及时间和资源消耗的诊断错误至关重要,但最重要的是,避免将没有真正BrS的患者送去进行有时负担着相当大风险的不适当的诊断和治疗途径是有帮助的。

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