致心律失常性右室心肌病(ARVC)旧称致心律失常性右室发育不良,又称为右心室扩张型心肌病、右心室壁瘤、羊皮纸样心脏,是一种遗传性心肌病,以右心室功能障碍和室性心律失常为特征,右心室心肌细胞常被脂肪或纤维脂肪组织替代。ARVC主要为常染色体显性遗传,外显率不一,临床表现轻重悬殊,多数病例死亡时年龄<40岁,有些发生于儿童,本病是青年期猝死的重要原因。虽然儿童ARVC的猝死和严重心力衰竭发生并不多见,但早期诊断ARVC有利于对患儿进行风险评估,指导早期治疗,提高生活质量,预防猝死,降低病死率。 To arrhythmogenic right ventricular cardiomyopathy (ARVC) Formerly known arrhythmogenic right ventricular dysplasia, also known as right ventricular dilated cardiomyopathy, right ventricular aneurysm, parchment-like heart, is a hereditary cardiomyopathy, right Ventricular dysfunction and ventricular arrhythmias are characterized by right ventricular myocardial cells often replaced by fat or fibrous adipose tissue. ARVC mainly autosomal dominant inheritance, different penetrance, clinical manifestations of severe disparity, the majority of cases of death <40 years old, and some occur in children, the disease is an important cause of sudden death in adolescence. Although the incidence of sudden death and severe heart failure is rare in children with ARVC, early diagnosis of ARVC is helpful for assessing risk in children, guiding early treatment, improving quality of life, preventing sudden death and reducing mortality.