Brugada P, Brugada J
Cardiovascular Center, OLV Hospital, Aalst, Belgium.
J Am Coll Cardiol. 1992 Nov 15;20(6):1391-6. doi: 10.1016/0735-1097(92)90253-j.
The objectives of this study were to present data on eight patients with recurrent episodes of aborted sudden death unexplainable by currently known diseases whose common clinical and electrocardiographic (ECG) features define them as having a distinct syndrome different from idiopathic ventricular fibrillation.
Among patients with ventricular arrhythmias who have no structural heart disease, several subgroups have been defined. The present patients constitute an additional subgroup with these findings.
The study group consisted of eight patients, six male and two female, with recurrent episodes of aborted sudden death. Clinical and laboratory data and results of electrocardiography, electrophysiology, echocardiography, angiography, histologic study and exercise testing were available in most cases.
The ECG during sinus rhythm showed right bundle branch block, normal QT interval and persistent ST segment elevation in precordial leads V1 to V2-V3 not explainable by electrolyte disturbances, ischemia or structural heart disease. No histologic abnormalities were found in the four patients in whom ventricular biopsies were performed. The arrhythmia leading to (aborted) sudden death was a rapid polymorphic ventricular tachycardia initiating after a short coupled ventricular extrasystole. A similar arrhythmia was initiated by two to three ventricular extrastimuli in four of the seven patients studied by programmed electrical stimulation. Four patients had a prolonged HV interval during sinus rhythm. One patient receiving amiodarone died suddenly during implantation of a demand ventricular pacemaker. The arrhythmia of two patients was controlled with a beta-adrenergic blocking agent. Four patients received an implantable defibrillator that was subsequently used by one of them, and all four are alive. The remaining patient received a demand ventricular pacemaker and his arrhythmia is controlled with amiodarone and diphenylhydantoin.
Common clinical and ECG features define a distinct syndrome in this group of patients. Its causes remain unknown.
本研究的目的是呈现8例反复发生心脏骤停但无法用目前已知疾病解释的患者的数据，其共同的临床和心电图特征将他们定义为患有与特发性室颤不同的独特综合征。
在无结构性心脏病的室性心律失常患者中，已定义了几个亚组。本研究中的患者构成了具有这些表现的另一个亚组。
研究组由8例患者组成，6例男性，2例女性，均反复发生心脏骤停。大多数病例可获得临床和实验室数据以及心电图、电生理、超声心动图、血管造影、组织学研究和运动试验的结果。
窦性心律时的心电图显示右束支传导阻滞、QT间期正常，胸前导联V1至V2-V3导联持续ST段抬高，无法用电解质紊乱、缺血或结构性心脏病解释。在接受心室活检的4例患者中未发现组织学异常。导致（心脏骤停）猝死的心律失常是一种快速多形性室性心动过速，由短联律间期室性早搏诱发。在接受程控电刺激研究中的7例患者中，有4例通过2至3次心室期前刺激诱发了类似的心律失常。4例患者在窦性心律时HV间期延长。1例接受胺碘酮治疗的患者在植入按需心室起搏器时突然死亡。2例患者的心律失常用β-肾上腺素能阻滞剂得到控制。4例患者接受了植入式除颤器，其中1例随后使用了该除颤器，4例患者均存活。其余1例患者接受了按需心室起搏器，其心律失常用胺碘酮和苯妥英钠控制。
共同的临床和心电图特征在这组患者中定义了一种独特的综合征。其病因仍然未知。
