心律失常性心肌病(ACM)是一种遗传决定的心肌病,以肌细胞坏死伴纤维脂肪替代和室性心律失常为特征,甚至可导致心源性猝死。已经报道了在心内膜活检或ACM患者的自体视标本中存在炎性细胞浸润。提示炎症在该疾病的病理生理学中的可能作用。此外,已观察到伴随心电图变化和肌钙蛋白释放的胸痛发作,并将其定义为“热相”现象。这项关键的系统评价的目的是评估出现“热期”发作的ACM患者的临床特征。根据PRISMA指南,在PubMed中进行了搜索,Scopus和WebofScience电子数据库使用以下关键词:“致心律失常性心肌病”;“心肌炎”或“致心律失常性心肌病”;“肌钙蛋白”或“致心律失常性心肌病”;和“热期”。共检索到1433个标题,其中65项研究可能与该主题相关。通过适用纳入和排除标准,本综述选择了9篇报告103例经历热期发作的ACM患者的论文。在76%的病例中,发作时的年龄是可用的,报告的平均年龄为26岁±14岁(最小2-最大71岁)。总的来说,86%的患者显示左心室心外膜LGE。在热点阶段的时候,49%的患者接受了ACM(在大多数情况下,心律失常性左心室心肌病)的诊断,19%的扩张型心肌病和26%的急性心肌炎。在基因研究中,Desmoplakin(DSP)是更有代表性的疾病基因(69%),其次是Plakophillin-2(9%)和Desmoglein-2(6%)。总之,显示热期发作的ACM患者通常年轻,DSP是最常见的疾病基因,占病例的69%。目前,"热期"在疾病进展和心律失常危险分层中的作用仍有待阐明.
Arrhythmogenic cardiomyopathy (ACM) is a genetically determined myocardial disease, characterized by myocytes necrosis with fibrofatty substitution and ventricular arrhythmias that can even lead to sudden cardiac death. The presence of inflammatory cell infiltrates in endomyocardial biopsies or in autoptic specimens of ACM patients has been reported, suggesting a possible role of inflammation in the pathophysiology of the disease. Furthermore, chest pain episodes accompanied by electrocardiographic changes and troponin release have been observed and defined as the \"hot-phase\" phenomenon. The aim of this critical systematic
review was to assess the clinical features of ACM patients presenting with \"hot-phase\" episodes. According to PRISMA guidelines, a search was run in the PubMed, Scopus and Web of Science electronic databases using the following keywords: \"arrhythmogenic cardiomyopathy\"; \"myocarditis\" or \"arrhythmogenic cardiomyopathy\"; \"troponin\" or \"arrhythmogenic cardiomyopathy\"; and \"hot-phase\". A total of 1433 titles were retrieved, of which 65 studies were potentially relevant to the topic. Through the application of inclusion and exclusion criteria, 9 papers reporting 103 ACM patients who had experienced hot-phase episodes were selected for this
review. Age at time of episodes was available in 76% of cases, with the mean age reported being 26 years ± 14 years (min 2-max 71 years). Overall, 86% of patients showed left ventricular epicardial LGE. At the time of hot-phase episodes, 49% received a diagnosis of ACM (Arrhythmogenic left ventricular cardiomyopathy in the majority of cases), 19% of dilated cardiomyopathy and 26% of acute myocarditis. At the genetic study, Desmoplakin (DSP) was the more represented disease-gene (69%), followed by Plakophillin-2 (9%) and Desmoglein-2 (6%). In conclusion, ACM patients showing hot-phase episodes are usually young, and DSP is the most common disease gene, accounting for 69% of cases. Currently, the role of \"hot-phase\" episodes in disease progression and arrhythmic risk stratification remains to be clarified.