Morcos George, Vashist Sudhir, Aktay Recai
Department of Diagnostic Radiology and Nuclear Medicine (G.M., R.A.) and Department of Pediatrics (S.V.), University of Maryland School of Medicine, 22 S Greene Street, Baltimore, MD 21201.
Radiol Cardiothorac Imaging. 2023 Mar 2;5(2):e220209. doi: 10.1148/ryct.220209. eCollection 2023 Apr.
Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disorder that cannot be explained by ischemic, hypertensive, or valvular heart disease and often results in sudden cardiac death. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the best-characterized ACM and can be diagnosed using the revised task force criteria. In contrast, there are no accepted clinical diagnostic criteria for arrhythmogenic left ventricular cardiomyopathy (ALVC), another subtype of ACM. Cardiac MRI aids in ARVC diagnosis by delineating biventricular structural and functional abnormalities and can be instrumental in diagnosing ALVC. This report presents a pediatric case of desmoplakin cardiomyopathy, a distinct subtype of ALVC, with findings overlapping myocarditis and LV noncompaction. Pediatrics, Heart, Cardiomyopathies Supplemental material is available for this article. © RSNA, 2023.
致心律失常性心肌病（ACM）是一种无法用缺血性、高血压性或瓣膜性心脏病解释的心肌疾病，常导致心源性猝死。致心律失常性右室心肌病（ARVC）是特征最明确的ACM，可使用修订的工作组标准进行诊断。相比之下，致心律失常性左室心肌病（ALVC）作为ACM的另一种亚型，目前尚无公认的临床诊断标准。心脏磁共振成像（MRI）通过描绘双心室结构和功能异常辅助ARVC诊断，对ALVC诊断也有帮助。本报告介绍了一名小儿桥粒斑蛋白心肌病（一种独特的ALVC亚型）病例，其表现与心肌炎和左室心肌致密化不全重叠。本文有补充材料。©RSNA，2023年。
