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Brugada综合征先证者的长期随访

Long-Term Follow-Up of Probands With Brugada Syndrome.

作者信息

de Asmundis Carlo, Mugnai Giacomo, Chierchia Gian-Battista, Sieira Juan, Conte Giulio, Rodriguez-Mañero Moises, Pappaert Gudrun, Czapla Jens, Nijs Jan, La Meir Mark, Casado Ruben, Ströker Erwin, De Regibus Valentina, Brugada Pedro

机构信息

Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.

Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.

出版信息

Am J Cardiol. 2017 May 1;119(9):1392-1400. doi: 10.1016/j.amjcard.2017.01.039. Epub 2017 Feb 10.

DOI:10.1016/j.amjcard.2017.01.039
PMID:28274576
Abstract

This study analyzes the natural history of a large cohort of probands with Brugada syndrome (BrS) to assess the predictive value of different clinical and electrocardiographic parameters for the development of ventricular fibrillation (VF) or sudden cardiac death (SCD) during a long-term follow-up. Baseline characteristics of 289 consecutive probands (203 men; mean age 45 ± 16 years) with a Brugada type 1 electrocardiogram were analyzed. After a mean follow-up of 10.1 ± 4.6 years, 29 malignant arrhythmias occurred. On multivariate analysis, a history of VF and syncopal episodes, fragmented QRS (f-QRS), spontaneous type 1 electrocardiogram, and early repolarization pattern were significantly associated with later occurrence of VF/SCD. In patients with drug-induced BrS, the accentuation or de novo appearance of f-QRS in other leads was always associated with VF/SCD. Cerebrovascular events occurred in 8 patients with atrial fibrillation (15.1%), most of them (75%) presenting as the first clinical manifestation. The time-to-diagnosis was found to be significantly shorter in those patients who directly came to our center than in those who referred to our center for a second opinion. In conclusion, systematic use of the pharmacologic challenge in patients with unexplained cardiovascular symptoms and/or atrial fibrillation might significantly improve the identification of BrS with a shortening of the time-to-diagnosis. The CHADSVASc score might be inappropriate for predicting transient ischemic attack or stroke in BrS. This study confirms the independent predictive value of previous VF and syncopal episodes, f-QRS, type 1 electrocardiogram, and early repolarization pattern. In BrS a sufficiently long follow-up is necessary before conclusions on prognosis are apparent.

摘要

本研究分析了一大群 Brugada 综合征(BrS)先证者的自然病史,以评估不同临床和心电图参数对长期随访期间室颤(VF)或心源性猝死(SCD)发生的预测价值。分析了 289 例连续的具有 1 型 Brugada 心电图的先证者(203 例男性;平均年龄 45±16 岁)的基线特征。平均随访 10.1±4.6 年后,发生了 29 次恶性心律失常。多因素分析显示,VF 和晕厥发作史、碎裂 QRS(f-QRS)、自发 1 型心电图及早期复极模式与 VF/SCD 的后期发生显著相关。在药物诱导的 BrS 患者中,其他导联 f-QRS 的加重或新发总是与 VF/SCD 相关。8 例房颤患者发生了脑血管事件(15.1%),其中大多数(75%)为首发临床表现。发现直接到我们中心就诊的患者诊断时间明显短于转诊至我们中心寻求二次意见的患者。总之,对不明原因心血管症状和/或房颤患者系统使用药物激发试验可能会显著改善 BrS 的识别,并缩短诊断时间。CHADSVASc 评分可能不适用于预测 BrS 患者的短暂性脑缺血发作或中风。本研究证实了既往 VF 和晕厥发作、f-QRS、1 型心电图及早期复极模式的独立预测价值。在 BrS 中,在得出明显的预后结论之前,需要进行足够长时间的随访。

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