Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed. It is a cell-to-cell junction cardiomyopathy, typically caused by genetically determined abnormalities of cardiac desmosomes, which leads to detachment of myocytes and alteration of intracellular signal transduction. The diagnosis of arrhythmogenic cardiomyopathy does not rely on a single gold standard test but is achieved using a scoring system, which encompasses familial and genetic factors, ECG abnormalities, arrhythmias, and structural/functional ventricular alterations. The main goal of treatment is the prevention of sudden cardiac death. Implantable cardioverter defibrillator is the only proven lifesaving therapy; however, it is associated with significant morbidity because of device-related complications and inappropriate implantable cardioverter defibrillator interventions. Selection of patients who are the best candidates for implantable cardioverter defibrillator implantation is one of the most challenging issues in the clinical management.

心律失常性心肌病是一种遗传性心肌疾病，易导致心源性猝死，尤其是在年轻患者和运动员中。病理特征包括心肌细胞丢失和右心室心肌的纤维脂肪置换。经常观察到双心室受累。它是一种细胞间连接型心肌病，通常是由基因确定的心脏桥粒异常所致，导致心肌细胞脱离和细胞内信号转导的改变。心律失常性心肌病的诊断不依靠单一的金标准测试，而是使用评分系统实现的，该评分系统包括家族和遗传因素，ECG异常，心律不齐以及结构/功能性心室改变。治疗的主要目标是预防心源性猝死。植入式心脏复律除颤器是唯一经证实可挽救生命的疗法。然而，由于与设备有关的并发症和不适当的植入式心脏复律除颤器干预，它与高发病率相关。选择最适合植入式心脏复律除颤器植入的患者是临床管理中最具挑战性的问题之一。