Eckardt L, Kirchhof P, Loh P, Schulze-Bahr E, Johna R, Wichter T, Breithardt G, Haverkamp W, Borggrefe M
Hospital of the Westfälische Wilhelms-University, Department of Cardiology and Angiology and Institute for Arteriosclerosis Research, Münster, Germany.
J Cardiovasc Electrophysiol. 2001 Jun;12(6):680-5. doi: 10.1046/j.1540-8167.2001.00680.x.
The Brugada syndrome is a distinct form of idiopathic ventricular fibrillation characterized by a unique ECG pattern consisting of a right bundle branch block-like aspect and ST segment elevation in leads V1 to V3. As a high induction rate of ventricular tachyarrhythmias has been reported in Brugada syndrome, we hypothesized that this also may be true for supraventricular tachycardias in these patients.
Between January 1995 and December 2000, we identified 35 consecutive patients with Brugada syndrome; 26 had a history of cardiac arrest or syncope and 9 were asymptomatic. All patients underwent electrophysiologic study, including an atrial and ventricular stimulation protocol. Ten patients (29%) were found to have supraventricular tachyarrhythmias (SVT) in addition to the Brugada syndrome. These 10 patients presented with aborted sudden cardiac death (n = 3) and/or a family history of sudden cardiac death (n = 4), syncope (n = 4), or primarily with a Brugada typical ECG, a positive family history, and palpitations (n = 2). Eight of them underwent genetic testing, but only 1 had a mutation in the SCN5A gene. In 6 patients, an AV nodal reentrant tachycardia was easily and reproducibly inducible. Two patients had clinical documented and inducible episodes of an atrial tachycardia (1 in addition to an AV nodal reentrant tachycardia). One patient had paroxysmal atrial fibrillation alternating with sinus rhythm, and 2 patients with accessory pathways were identified.
This is the first description of an association of the Brugada syndrome with SVT. Thus, the arrhythmogenic substrate in Brugada syndrome may not be restricted to the ventricular level. Palpitations in this syndrome should raise the possibility of SVT. Conversely, in patients with SVT and aborted sudden cardiac death or syncope not related to SVT, the Brugada syndrome should be considered a possible additional electrophysiologic abnormality.
Brugada综合征是一种独特的特发性心室颤动形式，其特征为一种独特的心电图模式，包括V1至V3导联出现右束支传导阻滞样图形及ST段抬高。由于已有报道称Brugada综合征中心室快速性心律失常的诱发率很高，我们推测这些患者中室上性快速性心律失常的情况可能也是如此。
在1995年1月至2000年12月期间，我们连续纳入了35例Brugada综合征患者；其中26例有心脏骤停或晕厥病史，9例无症状。所有患者均接受了电生理检查，包括心房和心室刺激方案。结果发现，10例患者（29%）除Brugada综合征外还患有室上性快速性心律失常（SVT）。这10例患者表现为心脏性猝死未遂（n = 3）和/或有心脏性猝死家族史（n = 4）、晕厥（n = 4），或主要表现为典型的Brugada心电图、阳性家族史及心悸（n = 2）。其中8例接受了基因检测，但只有1例SCN5A基因发生突变。6例患者中，房室结折返性心动过速易于且可重复诱发。2例患者有临床记录且可诱发的房性心动过速发作（1例除房室结折返性心动过速外）。1例患者有阵发性心房颤动与窦性心律交替，另外还发现2例有旁路的患者。
这是首次描述Brugada综合征与SVT的关联。因此，Brugada综合征的致心律失常基质可能不限于心室层面。该综合征中的心悸应提高存在SVT的可能性。相反，在患有SVT且有心脏性猝死未遂或与SVT无关的晕厥患者中，应考虑Brugada综合征可能是一种额外的电生理异常情况。
