Heart Rhythm Management Center, UZ Brussel-VUB, Laarbeeklaan 101, Brussels, Belgium.
Eur Heart J. 2011 Sep;32(17):2153-60. doi: 10.1093/eurheartj/ehr129. Epub 2011 Jul 4.
We sought to investigate the value of a family history of sudden death (SD) in Brugada syndrome (BS).
Two hundred and eighty consecutive patients (mean age: 41 ± 18 years, 168 males) with diagnostic type I Brugada ECG pattern were included. Sudden death occurred in 69 (43%) of 157 families. One hundred and ten SDs were analysed. During follow-up VF (ventricular fibrillation) or SD-free survival rate was not different between patients with or without a family history of SD of a first-degree relative, between patients with or without a family history of multiple SD of a first-degree relative at any age and between patients with or without a family history of SD in first-degree relatives ≤35 years. One patient had family history of SD of two first-degree relative ≤35 years with arrhythmic event during follow-up. In univariate analysis male gender (P = 0.01), aborted SD (P < 0.001), syncope (P = 0.04), spontaneous type I ECG (P < 0.001), and inducibility during electrophysiological (EP) study (P < 0.001) were associated with worse prognosis. The absence of syncope, aborted SD, spontaneous type I ECG, and inducibility during EP study was associated with a significantly better prognosis (P < 0.001).
Family history of SD is not predictive for future arrhythmic events even if considering only SD in first-degree relatives or SD in first-degree relatives at a young age. The absence of syncope, aborted SD, spontaneous type I ECG, and inducibility during EP study is associated with a good five-year prognosis.
我们旨在探讨家族性猝死(SD)病史在Brugada 综合征(BS)中的价值。
共纳入 280 例连续诊断为 Brugada 心电图 I 型的患者(平均年龄:41±18 岁,168 例男性)。69 例(43%)患者发生猝死。分析了 110 例猝死事件。在随访期间,具有一级亲属 SD 家族史或无家族史、任何年龄具有一级亲属多发 SD 家族史或无家族史、一级亲属≤35 岁 SD 家族史或无家族史的患者,VF(心室颤动)或 SD 无事件生存率无差异。随访期间,1 例患者有 2 名≤35 岁一级亲属 SD 家族史和心律失常事件。单因素分析显示,男性(P=0.01)、既往 SD(P<0.001)、晕厥(P=0.04)、自发性 I 型心电图(P<0.001)和电生理(EP)研究中的诱发性(P<0.001)与预后不良相关。无晕厥、既往 SD、自发性 I 型心电图和 EP 研究中的诱发性与显著更好的预后相关(P<0.001)。
即使仅考虑一级亲属中的 SD 或年轻一级亲属中的 SD,家族性 SD 病史也不能预测未来的心律失常事件。无晕厥、既往 SD、自发性 I 型心电图和 EP 研究中的诱发性与良好的五年预后相关。