Kataoka Shohei, Serizawa Naoki, Kitamura Kazutaka, Suzuki Atsushi, Suzuki Tsuyoshi, Shiga Tsuyoshi, Shoda Morio, Hagiwara Nobuhisa
Department of Cardiology, Tokyo Women׳s Medical University, 8-1 Kawadacho, Shinjuku-ku, Tokyo 162-8666, Japan.
J Arrhythm. 2016 Feb;32(1):70-3. doi: 10.1016/j.joa.2015.10.007. Epub 2015 Nov 30.
Overlapping characteristics of Brugada syndrome (BrS) and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) have been reported in recent studies, but little is known about the overlapping disease state of BrS and ARVC/D. A 36-year-old man, hospitalized at our institution for syncope, presented with this overlapping disease state. The electrocardiogram showed spontaneous coved-type ST-segment elevation, and ventricular fibrillation was induced by right ventricular outflow tract stimulation in an electrophysiological study. BrS was subsequently diagnosed; additionally, the presence of epsilon-like waves and right ventricular structural abnormalities met with the 2010 revised task force criteria for ARVC/D. After careful investigation for both BrS and ARVC/D, an implantable cardioverter defibrillator was inserted in the patient. This case revealed 2 important clinical findings: (1) BrS and ARVC/D clinical features can coexist in a single patient, and EPS might be useful for determining the phenotype of overlapping disease (e.g., BrS-like or ARVC/D-like). (2) An overlapping disease state of BrS and ARVC/D can change phenotypically during its clinical course. Therefore, careful examination and attentive follow-up are required for patients with BrS or ARVC/D.
近期研究报道了布加综合征（BrS）与致心律失常性右室心肌病/发育不良（ARVC/D）的重叠特征，但对于BrS与ARVC/D的重叠疾病状态知之甚少。一名36岁男性因晕厥入住我院，呈现出这种重叠疾病状态。心电图显示自发性穹窿型ST段抬高，电生理研究中右室流出道刺激诱发了室颤。随后诊断为BrS；此外，存在类epsilon波和右室结构异常符合2010年修订的ARVC/D工作组标准。在对BrS和ARVC/D进行仔细检查后，为该患者植入了植入式心律转复除颤器。该病例揭示了两个重要的临床发现：（1）BrS和ARVC/D的临床特征可在同一患者中共存，电生理检查可能有助于确定重叠疾病的表型（如BrS样或ARVC/D样）。（2）BrS和ARVC/D的重叠疾病状态在其临床过程中可能发生表型改变。因此，对于BrS或ARVC/D患者需要进行仔细检查和密切随访。
