ABC | Volume 112, Nº1, January 2019

Elias Nero et al ARVC/D - Diagnosis and treatment Arq Bras Cardiol. 2019; 112(1):91-103 Review Article 1. Fontaine G, Chen HS. Arrhythmogenic right ventricular dysplasia back in force. Am J Cardiol. 2014;113(10):1735-9. 2. Elias J, Tonet J, Frank R, Fontaine G. Arrhythmogenic right ventricular dysplasia. Arq Bras Cardiol. 1998;70(6):449-56. 3. Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65(2):384-98. 4. Wang W, James CA, Calkins H. Diagnostic and therapeutic strategies for arrhythmogenic right ventricular dysplasia/cardiomyopathy patient. Europace. 2018 Apr 23; [Epub ahead of print]. Disponível em: https:// academic.oup.com/europace/advance-article 5. Corrado D, Link MS, Calkins H. Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 2017;376(1):61-72. 6. Calkins H. Arrhythmogenic right ventricular dysplasia/cardiomyopathy - three decades of progress. Circ J. 2015;79(5):901-13. 7. Philips B, Cheng A. 2015 update on the diagnosis and management of arrhythmogenic right ventricular cardiomyopathy. Curr Opin Cardiol. 2016;31(1):46-56. 8. Asimaki A, Kleber AG, Saffitz JE. Pathogenesis of arrhythmogenic cardiomyopathy. Can J Cardiol. 2015;31(11):1313-24. 9. Zhang L, Liu L, Kowey PR, Fontaine GH. The electrocardiographic manifestations of arrhythmogenic right ventricular dysplasia. Curr Cardiol Rev. 2014;10(3):237-45. 10. Marcus F, Edson S, Towbin JA. Genetics of arrhythmogenic right ventricular cardiomyopathy: a practical guide for physicians. J Am Coll Cardiol. 2013;61(19):1945-8. References Author contributions Conception and design of the research and acquisition of data: Elias Neto J, Tonet J, Fontaine G; analysis and interpretation of the data and critical revision of the manuscript for intellectual content: Elias Neto J, Tonet J, Frank R; statistical analysis, obtaining funding and writing of the manuscript: Elias Neto J. Potential Conflict of Interest No potential conflict of interest relevant to this article was reported. Figure 4 – Flowchart of indications for implantation of ICD in ARVC/D. The flowchart is based on available data on annual mortality rates associated with specific risk factors. High risk of major arrhythmic events: > 10%/year; intermediate risk: 1% to 10%/year and low risk: < 1%/year. The indications for ICD implantation were determined by consensus, taking not only the statistical risk into account, but also the general health status, socioeconomic factors, psychological impact and adverse effects of the device. SCD: sudden cardiac death; VF: ventricular fibrillation; VT: ventricular tachycardia; RV: right ventricle; LV: left ventricle. *See the text for the distinction between major and minor risk factors. Adapted from Corrado et al., 2017. 22 High risk – SAD aborted due to VF – Sustained VT – Severe dysfunction (RV and/or LV) ICD indicated (Class I) ICD should be indicated (Class IIa) ICD can be indicated (Class IIb) ICD not indicated (Class III) Intermediate risk Low risk ≥ 1 minor risk factor* ≥ Major risk factors: – Syncope – NSVT – Moderate dysfunction (RV and/or LV) No risk factors Gene carrier - asymptomatic Flowchart for indication of ICD implant in ARVC/D Sources of Funding There were no external funding sources for this study. Study Association This study is not associatedwith any thesis or dissertationwork. 11