INSERM, UMR, Nantes, France.
Circulation. 2010 Feb 9;121(5):635-43. doi: 10.1161/CIRCULATIONAHA.109.887026. Epub 2010 Jan 25.
Brugada syndrome is characterized by ST-segment elevation in the right precordial leads and an increased risk of sudden cardiac death (SCD). Fundamental questions remain on the best strategy for assessing the real disease-associated arrhythmic risk, especially in asymptomatic patients. The aim of the present study was to evaluate the prognosis and risk factors of SCD in Brugada syndrome patients in the FINGER (France, Italy, Netherlands, Germany) Brugada syndrome registry.
Patients were recruited in 11 tertiary centers in 4 European countries. Inclusion criteria consisted of a type 1 ECG present either at baseline or after drug challenge, after exclusion of diseases that mimic Brugada syndrome. The registry included 1029 consecutive individuals (745 men; 72%) with a median age of 45 (35 to 55) years. Diagnosis was based on (1) aborted SCD (6%); (2) syncope, otherwise unexplained (30%); and (3) asymptomatic patients (64%). During a median follow-up of 31.9 (14 to 54.4) months, 51 cardiac events (5%) occurred (44 patients experienced appropriate implantable cardioverter-defibrillator shocks, and 7 died suddenly). The cardiac event rate per year was 7.7% in patients with aborted SCD, 1.9% in patients with syncope, and 0.5% in asymptomatic patients. Symptoms and spontaneous type 1 ECG were predictors of arrhythmic events, whereas gender, familial history of SCD, inducibility of ventricular tachyarrhythmias during electrophysiological study, and the presence of an SCN5A mutation were not predictive of arrhythmic events.
In the largest series of Brugada syndrome patients thus far, event rates in asymptomatic patients were low. Inducibility of ventricular tachyarrhythmia and family history of SCD were not predictors of cardiac events.
Brugada 综合征的特征是右侧胸前导联 ST 段抬高和发生心源性猝死（SCD）的风险增加。目前仍存在一些基本问题，即评估真正与疾病相关的心律失常风险的最佳策略，尤其是在无症状患者中。本研究的目的是评估 FINGER（法国、意大利、荷兰、德国）Brugada 综合征注册研究中 Brugada 综合征患者的 SCD 预后和危险因素。
患者在欧洲 4 个国家的 11 个三级中心招募。纳入标准包括排除可引起 Brugada 综合征样表现的疾病后，基线或药物激发后出现 1 型心电图。该注册研究共纳入 1029 例连续患者（745 例男性，72%），中位年龄为 45（35 至 55）岁。诊断依据为：（1）心源性猝死中止（6%）；（2）不明原因晕厥（30%）；和（3）无症状患者（64%）。中位随访 31.9（14 至 54.4）个月期间，51 例患者发生心脏事件（5%）（44 例患者接受了适当的植入式心脏复律除颤器电击，7 例患者猝死）。心源性猝死中止、不明原因晕厥和无症状患者的年心脏事件发生率分别为 7.7%、1.9%和 0.5%。症状和自发性 1 型心电图是心律失常事件的预测因素，而性别、SCD 家族史、电生理研究中诱发的室性心动过速/心室颤动、SCN5A 基因突变的存在不能预测心律失常事件。
在迄今最大的 Brugada 综合征患者系列研究中，无症状患者的事件发生率较低。室性心动过速/心室颤动的诱发性和 SCD 家族史不能预测心脏事件。
