Kusano Kengo F, Taniyama Makiko, Nakamura Kazufumi, Miura Daiji, Banba Kimikazu, Nagase Satoshi, Morita Hiroshi, Nishii Nobuhiro, Watanabe Atsuyuki, Tada Takeshi, Murakami Masato, Miyaji Kohei, Hiramatsu Shigeki, Nakagawa Koji, Tanaka Masamichi, Miura Aya, Kimura Hideo, Fuke Soichiro, Sumita Wakako, Sakuragi Satoru, Urakawa Shigemi, Iwasaki Jun, Ohe Tohru
Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
J Am Coll Cardiol. 2008 Mar 25;51(12):1169-75. doi: 10.1016/j.jacc.2007.10.060.
The goal of our work was to examine the relationships of atrial fibrillation (AF) with genetic, clinical, and electrophysiological backgrounds in Brugada syndrome (BrS).
Atrial fibrillation is often observed in patients with BrS and indicates that electrical abnormality might exist in the atrium as well as in the ventricle. SCN5A, a gene encoding the cardiac sodium channel, has been reported to be causally related to BrS. However, little is known about the relationships of atrial arrhythmias with genetic, clinical, and electrophysiological backgrounds of BrS.
Seventy-three BrS patients (49 +/- 12 years of age, men/women = 72/1) were studied. The existence of SCN5A mutation and clinical variables (syncopal episode, documented ventricular fibrillation [VF], and family history of sudden death) were compared with spontaneous AF episodes. Genetic and clinical variables were also compared with electrophysiologic (EP) parameters: atrial refractory period, interatrial conduction time (CT), repetitive atrial firing, and AF induction by atrial extra-stimulus testing.
Spontaneous AF occurred in 10 (13.7%) of the BrS patients and SCN5A mutation was detected in 15 patients. Spontaneous AF was associated with higher incidence of syncopal episodes (60.0% vs. 22.2%, p < 0.03) and documented VF (40.0% vs. 14.3%, p < 0.05). SCN5A mutation was associated with prolonged CT (p < 0.03) and AF induction (p < 0.05) in EP study, but not related to the spontaneous AF episode and other clinical variables. In patients with documented VF, higher incidence of spontaneous AF (30.8% vs. 10.0%, p < 0.05), AF induction (53.8% vs. 20.0%, p < 0.03), and prolonged CT was observed.
Spontaneous AF and VF are closely linked clinically and electrophysiologically in BrS patients. Patients with spontaneous AF have more severe clinical backgrounds in BrS. SCN5A mutation is associated with electrical abnormality but not disease severity.
我们研究的目的是探讨在Brugada综合征（BrS）中，心房颤动（AF）与遗传、临床及电生理背景之间的关系。
BrS患者中常观察到心房颤动，这表明心房和心室可能均存在电活动异常。据报道，编码心脏钠通道的基因SCN5A与BrS存在因果关系。然而，关于心房颤动与BrS的遗传、临床及电生理背景之间的关系，我们知之甚少。
对73例BrS患者（年龄49±12岁，男/女=72/1）进行研究。将SCN5A突变的存在情况及临床变量（晕厥发作、记录到的室颤[VF]和猝死家族史）与自发性AF发作进行比较。还将遗传和临床变量与电生理（EP）参数进行比较：心房不应期、房间传导时间（CT）、心房重复激动以及心房额外刺激试验诱发AF。
10例（13.7%）BrS患者发生自发性AF，15例患者检测到SCN5A突变。自发性AF与晕厥发作（60.0%对22.2%，p<0.03）和记录到的VF（40.0%对14.3%，p<0.05）的较高发生率相关。在EP研究中，SCN5A突变与CT延长（p<0.03）和AF诱发（p<0.05）相关，但与自发性AF发作及其他临床变量无关。在记录到VF的患者中，观察到自发性AF（30.8%对10.0%，p<0.05）、AF诱发（53.8%对20.0%，p<0.03）的较高发生率以及CT延长。
在BrS患者中，自发性AF和VF在临床和电生理方面密切相关。发生自发性AF的BrS患者具有更严重的临床背景。SCN5A突变与电活动异常相关，但与疾病严重程度无关。
