Department of Cardiovascular Medicine and Diagnostic Radiology, Faculty of Life Sciences, Graduate School of Medical Sciences, Kumamoto University, 1-1-1, Honjo, Chuo-ku, Kumamoto, 860-8556, Japan.
Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.
ESC Heart Fail. 2020 Oct;7(5):3174-3178. doi: 10.1002/ehf2.12790. Epub 2020 Jun 27.
A 74-year-old man had abnormal left ventricular (LV) function according to a perioperative test at a local hospital and was transferred to our institution for further evaluation and treatment. His electrocardiogram demonstrated the presence of premature ventricular contraction with a QRS complex of the right bundle branch block type and superior axis. His echocardiography showed systolic dysfunction of the LV (LV ejection fraction, 44.6%). Cardiac computed tomography imaging revealed banded and patchy densities observed frequently from the middle to epicardial layer of the LV wall. Cardiac magnetic resonance imaging showed fat signals on fat-selective images and late gadolinium enhancement in the mid-wall to subepicardial layers in the LV myocardium. Endomyocardial biopsy revealed the histological presence of fibrofatty replacement. A genetic analysis revealed a nonsense mutation in the desmoplakin gene. Thus, he was diagnosed with left-dominant arrhythmogenic cardiomyopathy. To prevent fatal ventricular arrhythmias, an implantable cardioverter defibrillator was successfully implanted.
一位 74 岁男性在当地医院的一项围手术期检查中发现左心室（LV）功能异常，随后被转至我院进行进一步评估和治疗。他的心电图显示存在室性早搏，QRS 复合波呈右束支传导阻滞型，电轴向上。他的超声心动图显示 LV 收缩功能障碍（LV 射血分数为 44.6%）。心脏计算机断层扫描成像显示 LV 壁中层至心外膜层常见的带状和斑片状密度。心脏磁共振成像显示在 LV 心肌的中壁至心外膜层有脂肪选择性图像上的脂肪信号和晚期钆增强。心肌内膜活检显示存在纤维脂肪替代的组织学特征。基因分析显示桥粒蛋白基因突变。因此，他被诊断为左优势型致心律失常性右室心肌病。为预防致命性室性心律失常，成功植入了植入式心脏复律除颤器。
