循環器ジャーナル70巻1号

2022年01月発行

特集 心筋症診療のフロントライン—概念から最新の治療まで Ⅶ章　不整脈と出会ったら

不整脈原性右室心筋症（ARVC）

著者： 大野聖子¹

所属機関： ¹国立循環器病研究センター分子生物学部

ページ範囲：P.157 - P.162

Point
・不整脈原性右室心筋症（ARVC）は心室不整脈により突然死を生じる遺伝性疾患である．
・致死性不整脈に対しては薬物・アブレーション治療や植込み型除細動器が使用される．
・ARVCは進行すると右心不全を生じ，重症例では心移植を必要とする．

参考文献

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2）Wada Y, Ohno S, Aiba T, et al : Unique genetic background and outcome of non-Caucasian Japanese probands with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Mol Genet Genomic Med 5 : 639-651, 2017

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5）Towbin JA, McKenna WJ, Abrams DJ, et al : 2019 HRS Expert Consensus Statement on Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy. Heart Rhythm 16 : e301-e372, 2019

6）Corrado D, Perazzolo Marra M, Zorzi A, et al : Diagnosis of arrhythmogenic cardiomyopathy : The Padua criteria. Int J Cardiol 319 : 106-114, 2020

7）Corrado D, Basso C, Judge DP : Arrhythmogenic Cardiomyopathy. Circ Res 121 : 784-802, 2017

8）Wang W, Orgeron G, Tichnell C, et al : Impact of Exercise Restriction on Arrhythmic Risk Among Patients With Arrhythmogenic Right Ventricular Cardiomyopathy. J Am Heart Assoc 7 : e008843, 2018

9）James CA, Bhonsale A, Tichnell C, et al : Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol 62 : 1290-1297, 2013

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11）Cappelletto C, Gregorio C, Barbati G, et al : Antiarrhythmic therapy and risk of cumulative ventricular arrhythmias in arrhythmogenic right ventricle cardiomyopathy. Int J Cardiol 334 : 58-64, 2021

12）Daimee UA, Assis FR, Murray B, et al : Clinical outcomes of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular cardiomyopathy : Insights from the Johns Hopkins ARVC Program. Heart Rhythm 18 : 1369-1376, 2021

13）日本循環器学会/日本不整脈心電学会合同ガイドライン　不整脈非薬物治療ガイドライン（2018年改訂版）．2019

14）Cadrin-Tourigny J, Bosman LP, Nozza A, et al : A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. Eur Heart J 40 : 1850-1858, 2019