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Arrhythmogenic right ventricular dysplasia/cardiomyopathy

Published online by Cambridge University Press:  13 January 2017

Gabriela M. Orgeron  and
Jane E. Crosson
Gabriela M. Orgeron
Affiliation:
Division of Cardiology, Bloomberg Children’s Center, Johns Hopkins University, Baltimore, Maryland, United States of America
Jane E. Crosson*
Affiliation:
Division of Pediatric Cardiology, Bloomberg Children’s Center, Johns Hopkins University, Baltimore, Maryland, United States of America
*
Correspondence to: Dr J. E. Crosson, Johns Hopkins Children’s Center, 1800 Orleans Street, Room 2319, Baltimore, MD 21287, United States of America. Tel: 410 955 5987; Fax: 410-614-9942; E-mail: jcrosso@jhmi.edu
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Abstract

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited cardiomyopathy characterised by ventricular arrhythmias and an increased risk of sudden cardiac death. Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnosis is based on criteria that take into account electrical and structural cardiac abnormalities, as well as mutation analysis. Appropriate pharmacological therapy and the prevention of sudden death with implantable defibrillators are important in the management of these patients. Exercise is considered an important environmental factor for the development and progression of the disease.

Keywords

Arrhythmogenic right ventricular dysplasia/cardiomyopathyexercisechildrenyoung athletes
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Supplement S1: Sudden Cardiac Arrest in the Young: A Contemporary Perspective , January 2017 , pp. S57 - S61
Copyright
© Cambridge University Press 2017 

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References

1. Marcus, FI, Fontaine, GH, Guiraudon, G, et al. Right ventricular dysplasia: report of 24 adult cases. Circulation 1982; 65: 384–398.Google Scholar
2. Corrado, D, Thiene, G, Nava, A, Rossi, L, Pennelli, N. Sudden death in young competitive athletes: clinicopathologic correlations in 22 cases. Am J Med 1990; 89: 588–596.Google Scholar
3. Dalal, D, Nasir, K, Bomma, C, et al. Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation 2005; 112: 3823–3832.Google Scholar
4. Peters, S, Trummel, M, Meyners, W. Prevalence of right ventricular dysplasia: cardiomyopathy in a non-referral hospital. Int J Cardiol 2004; 97: 499–501.Google Scholar
5. Groeneweg, J, Bhonsale, A, James, C, et al. Clinical presentation, long-term follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members. Circ Cardiovasc Genet 2015; 8: 437–446.CrossRefGoogle ScholarPubMed
6. Den Haan, AD, Tan, BY, Zikusoka, MN, et al. Comprehensive desmosome mutation analysis in North Americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Cardiovasc Genet 2009; 2: 428–435.CrossRefGoogle ScholarPubMed
7. Bauce, B, Rampazzo, A, Basso, C, et al. Clinical phenotype and diagnosis of arrhythmogenic right ventricular cardiomyopathy in pediatric patients carrying desmosomal gene mutations. Heart Rhythm 2011; 8: 1686–1695.CrossRefGoogle ScholarPubMed
8. Te Riele, AS, Sawant, AC, James, C, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy in the pediatric population: clinical characterization and comparison with adult-onset disease. JACC Clin Electrophysiol 2015; 1: 551–560.CrossRefGoogle ScholarPubMed
9. Marcus, FI, McKenna, WJ, Sherrill, D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation 2010; 121: 1533–1541.Google Scholar
10. Calkins, H. Arrhythmogenic right ventriculardysplasia/cardiomyopathy-three decades of progress. Circ J 2015; 79: 901–913.CrossRefGoogle Scholar
11. Corrado, D, Basso, C, Rizzoli, G, Schiavon, M, Thiene, G. Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol 2003; 42: 1959–1963.Google Scholar
12. Saberniak, J, Hasselberg, NE, Borgquist, R, et al. Vigorous physical activity impairs myocardial function in patients with arrhythmogenic right ventricular cardiomyopathy and in mutation positive family members. Eur J Heart Fail 2014; 16: 1337–1344.Google Scholar
13. Ruwald, AC, Marcus, F, Estes, NA 3rd, et al. Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy: results from the North American multidisciplinary study of arrhythmogenic right ventricular cardiomyopathy. Eur Heart J 2015; 36: 1735–1743.Google Scholar
14. 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 2013; 62: 1290–1297.Google Scholar
15. Sawant, AC, Te Riele, AS, Tichnell, C, et al. Safety of American Heart Association-recommended minimum exercise for desmosomal mutation carriers. Heart Rhythm 2016; 13: 199–207.Google Scholar
16. Kirchhof, P, Fabritz, L, Zwiener, M, et al. Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice. Circulation 2006; 114: 1799–1806.CrossRefGoogle ScholarPubMed
17. Chelko, S, Asimaki, A, Wei, A, et al. Exercise and mitochondrial respiration in mice with arrhythmogenic cardiomyopathy. Circulation 2014; 130: A20589.CrossRefGoogle Scholar
18. Fabritz, L, Hoogendijk, MG, Scicluna, BP, et al. Load-reducing therapy prevents development of arrhythmogenic right ventricular cardiomyopathy in plakoglobin-deficient mice. J Am Coll Cardiol 2011; 57: 740–750.CrossRefGoogle ScholarPubMed
19. Piccini, JP, Dalal, D, Roguin, A, et al. Predictors of appropriate implantable defibrillator therapies in patients with arrhythmogenic right ventricular dysplasia. Heart Rhythm 2005; 2: 1188–1194.CrossRefGoogle ScholarPubMed
20. Bhonsale, A, James, CA, Tichnell, C, et al. Incidence and predictors of implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergoing implantable cardioverter-defibrillator implantation for primary prevention. J Am Coll Cardiol 2011; 58: 1485–1496.CrossRefGoogle ScholarPubMed
21. Corrado, D, Leoni, L, Link, MS, et al. Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 2003; 108: 3084–3091.CrossRefGoogle ScholarPubMed
22. Corrado, D, Wichter, T, Link, MS, et al. Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement. Eur Heart J 2015; 36: 3227–3237.Google ScholarPubMed
23. Philips, B, Madhavan, S, James, C, et al. Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Arrhythm Electrophysiol 2012; 5: 499–505.CrossRefGoogle ScholarPubMed
24. Philips, B, te Riele, AS, Sawant, A, et al. Outcomes and VT recurrence characteristics after epicardial ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Heart Rhythm 2014; 12: 716–725.CrossRefGoogle ScholarPubMed
25. Maron, BJ, Udelson, JE, Bonow, RO, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from the American Heart Association and American College of Cardiology. Circulation 2015; 132: e273–e280.Google Scholar
26. Tedford, RJ, James, C, Judge, DP, et al. Cardiac transplantation in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol 2012; 59: 289–290.Google Scholar