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Arrhythmogenic right ventricular cardiomyopathy: evaluation of the current diagnostic criteria and differential diagnosis
European Heart Journal ( IF 37.6 ) Pub Date : 2019-10-21 , DOI: 10.1093/eurheartj/ehz669 Domenico Corrado 1 , Peter J van Tintelen 2, 3 , William J McKenna 4, 5 , Richard N W Hauer 6 , Aris Anastastakis 7 , Angeliki Asimaki 8 , Cristina Basso 1 , Barbara Bauce 1 , Corinna Brunckhorst 9 , Chiara Bucciarelli-Ducci 10 , Firat Duru 9 , Perry Elliott 5 , Robert M Hamilton 11 , Kristina H Haugaa 12, 13 , Cynthia A James 14 , Daniel Judge 15 , Mark S Link 16 , Francis E Marchlinski 17 , Andrea Mazzanti 18 , Luisa Mestroni 19 , Antonis Pantazis 20 , Antonio Pelliccia 21 , Martina Perazzolo Marra 1 , Kalliopi Pilichou 1 , Pyotr G A Platonov 22 , Alexandros Protonotarios 23 , Alessandra Rampazzo 24 , Jeffry E Saffitz 25 , Ardan M Saguner 9 , Christian Schmied 9 , Sanjay Sharma 26 , Hari Tandri 14 , Anneline S J M Te Riele 27, 28 , Gaetano Thiene 1 , Adalena Tsatsopoulou 29 , Wojciech Zareba 30 , Alessandro Zorzi 1 , Thomas Wichter 31 , Frank I Marcus 32 , Hugh Calkins 14 , Aris Anastastakis , Angeliki Asimaki , Cristina Basso , Barbara Bauce , Corinna Brunckhorst , Chiara Bucciarelli-Ducci , Hugh Calkins , Domenico Corrado , Firat Duru , Perry Elliott , Robert M Hamilton , Richard N W Hauer , Kristina H Haugaa , Cynthia A James , Daniel Judge , Mark S Link , Francis E Marchlinski , Frank I Marcus , William J McKenna , Andrea Mazzanti , Luisa Mestroni , Antonis Pantazis , Antonio Pelliccia , Martina Perazzolo Marra , Kalliopi Pilichou , Pyotr G A Platonov , Alexandros Protonotarios , Alessandra Rampazzo , Jeffry E Saffitz , Ardan Saguner , Christian Schmied , Sanjay Sharma , Hari Tandri , Anneline S J M Te Riele , Gaetano Thiene , Adalena Tsatsopoulou , Peter J van Tintelen , Thomas Wichter , Wojciech Zareba , Alessandro Zorzi ,
European Heart Journal ( IF 37.6 ) Pub Date : 2019-10-21 , DOI: 10.1093/eurheartj/ehz669 Domenico Corrado 1 , Peter J van Tintelen 2, 3 , William J McKenna 4, 5 , Richard N W Hauer 6 , Aris Anastastakis 7 , Angeliki Asimaki 8 , Cristina Basso 1 , Barbara Bauce 1 , Corinna Brunckhorst 9 , Chiara Bucciarelli-Ducci 10 , Firat Duru 9 , Perry Elliott 5 , Robert M Hamilton 11 , Kristina H Haugaa 12, 13 , Cynthia A James 14 , Daniel Judge 15 , Mark S Link 16 , Francis E Marchlinski 17 , Andrea Mazzanti 18 , Luisa Mestroni 19 , Antonis Pantazis 20 , Antonio Pelliccia 21 , Martina Perazzolo Marra 1 , Kalliopi Pilichou 1 , Pyotr G A Platonov 22 , Alexandros Protonotarios 23 , Alessandra Rampazzo 24 , Jeffry E Saffitz 25 , Ardan M Saguner 9 , Christian Schmied 9 , Sanjay Sharma 26 , Hari Tandri 14 , Anneline S J M Te Riele 27, 28 , Gaetano Thiene 1 , Adalena Tsatsopoulou 29 , Wojciech Zareba 30 , Alessandro Zorzi 1 , Thomas Wichter 31 , Frank I Marcus 32 , Hugh Calkins 14 , Aris Anastastakis , Angeliki Asimaki , Cristina Basso , Barbara Bauce , Corinna Brunckhorst , Chiara Bucciarelli-Ducci , Hugh Calkins , Domenico Corrado , Firat Duru , Perry Elliott , Robert M Hamilton , Richard N W Hauer , Kristina H Haugaa , Cynthia A James , Daniel Judge , Mark S Link , Francis E Marchlinski , Frank I Marcus , William J McKenna , Andrea Mazzanti , Luisa Mestroni , Antonis Pantazis , Antonio Pelliccia , Martina Perazzolo Marra , Kalliopi Pilichou , Pyotr G A Platonov , Alexandros Protonotarios , Alessandra Rampazzo , Jeffry E Saffitz , Ardan Saguner , Christian Schmied , Sanjay Sharma , Hari Tandri , Anneline S J M Te Riele , Gaetano Thiene , Adalena Tsatsopoulou , Peter J van Tintelen , Thomas Wichter , Wojciech Zareba , Alessandro Zorzi ,
Affiliation
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease characterized by progressive fibrofatty replacement of the right ventricular (RV) myocardium which may act as a substrate for ventricular arrhythmias and sudden cardiac death (SCD).1,2 The classic form of ARVC is a genetically determined cardiomyopathy caused by heterozygous or compound mutations in genes encoding proteins of desmosomes, which are specialized intercellular structures providing mechanical attachment of myocytes.3 However, there are other genetic (non-desmosomal) and non-genetic causes of the disease. Biventricular and left-dominant disease variants have been identified and have led some to use the term ‘arrhythmogenic cardiomyopathy’ (ACM) to define the broader spectrum of the disease phenotypic expressions.2–8
中文翻译：
心律失常性右室心肌病：当前诊断标准的评估和鉴别诊断
心律失常性右心室心肌病（ARVC）是一种遗传性心肌病，其特征是右心室（RV）心肌逐渐进行纤维脂肪替代，其可能充当室性心律不齐和心源性猝死（SCD）的底物。1 ，2 ARVC的典型形式是编码桥粒，这是专门提供心肌细胞的机械连接间结构的蛋白质的基因引起的杂合或化合物突变遗传决定的心肌病。3但是，该疾病还有其他遗传（非桥粒）和非遗传原因。已经确定了双心室和左优势疾病变体，并导致一些人使用术语“心律失常性心肌病”（ACM）来定义疾病表型表达的更广泛范围。2–8
更新日期：2020-04-08
中文翻译：
心律失常性右室心肌病：当前诊断标准的评估和鉴别诊断
心律失常性右心室心肌病（ARVC）是一种遗传性心肌病，其特征是右心室（RV）心肌逐渐进行纤维脂肪替代，其可能充当室性心律不齐和心源性猝死（SCD）的底物。1 ，2 ARVC的典型形式是编码桥粒，这是专门提供心肌细胞的机械连接间结构的蛋白质的基因引起的杂合或化合物突变遗传决定的心肌病。3但是，该疾病还有其他遗传（非桥粒）和非遗传原因。已经确定了双心室和左优势疾病变体，并导致一些人使用术语“心律失常性心肌病”（ACM）来定义疾病表型表达的更广泛范围。2–8
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