Heart Rhythm
Volume 6, Issue 7 , Pages 984-992 , July 2009

Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: Results from the North American Multidisciplinary Study

Received 3 November 2008 ,Accepted 6 March 2009.

References 

  1. Marcus FI, Fontaine G. Arrhythmogenic right ventricular dysplasia/cardiomyopathy, a review. Pacing Clin Electrophysiol. 1995;18:1298–1314
  2. Kies P, Bootsma M, Bax J, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Screening, diagnosis, and treatment. Heart Rhythm. 2006;3:225–234
  3. Sen-Chowdhry S, Lowe MD, Sporton SC, et al. Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. Am J Med. 2004;117:685–695
  4. Thiene G, Nava A, Corrado D, et al. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988;318:129–133
  5. Maron BJ. Cardiovascular risks to young persons on the athletic field. Ann Intern Med. 1998;129:379–386
  6. Marcus FI. Right ventricular dysplasia: evaluation and management in relation to sports activities. In:  Estes NAM,  Salem DN,  Wang PJ editor. Sudden Cardiac Death in the Athlete. Armonk, NY: Futura Publishing Co; 1998;p. 277–284
  7. Corrado D, Basso C, Rizzoli G, et al. Does sports activity enhance the risk of sudden death in adolescents and young adults?. J Am Coll Cardiol. 2003;42:1959–1963
  8. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65:384–399
  9. Bomma C, Rutberg J, Tandri H, et al. Misdiagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Cardiovasc Electrophysiol. 2004;15:300–306
  10. McKenna WJ, Thiene G, Nava A, et al. Diagnosis of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology). Br Heart J. 1994;71:215–218
  11. Marcus F, Towbin J, Zareba W, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) a multidisciplinary study: Design and protocol. Circulation. 2003;107:2975–2978
  12. Nava AF, Folino F, Bauce B, et al. Signal-averaged electrocardiogram in patients with arrhythmogenic right ventricular cardiomyopathy and ventricular arrhythmias. Eur Heart J. 2000;21:58–65
  13. Buxton AE, Fisher JD, Josephson ME, et al. Prevention of sudden death in patients with coronary artery disease: the Multicenter Unsustained Tachycardia Trial (MUSTT). Prog Cardiovasc Dis. 1993;36:215–226
  14. Yoerger DM, Marcus F, Sherrill D, et al. Echocardiographic findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia. J Am Coll Cardiol. 2005;45:860–865
  15. Tandri H, Friedrich MG, Calkins H, et al. MRI of arrhythmogenic right ventricular cardiomyopathy/dysplasia. J Cardiovasc Magn Res. 2004;6:557–563
  16. Basso C, Thiene G. Autopsy and endomyocardial biopsy findings. In:  Marcus F,  Nava A,  Thiene G editor. Arrhythmogenic RV Cardiomyopathy/Dysplasia. Milan: Springer-Verlag Italia; 2007;p. 29–44
  17. Basso C, Ronco F, Marcus F, et al. Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria. Eur Heart J. 2008;29:2760–2771
  18. Dalal D, Nasir K, Bomma C, et al. Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation. 2005;112:3823–3832
  19. Heidbuchel H, Hoogsteen J, Fagard R, et al. High prevalence of right ventricular involvement in endurance athletes with ventricular arrhythmias (Role of an electrophysiologic study in risk stratification). Eur Heart J. 2003;24:1473–1480
  20. Marcus F, Towbin JA. The mystery of arrhythmogenic right ventricular dysplasia/cardiomyopathy: from observation to mechanistic explanation. Circulation. 2006;114:1794–1795
  21. 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;113:1799–1806
  22. Peters S, Trummel M. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy: value of standard ECG revisited. Ann Noninvasive Electrocardiol. 2003;8:238–245
  23. Nasir K, Bomma C, Tandri H, et al. Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity (A need to broaden diagnostic criteria). Circulation. 2004;110:1527–1534
  24. Cox MGPJ, Nelen MR, Wilde AAM, et al. Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria. J Cardiovasc Electrophysiol. 2008;19:775–781
  25. Bauce B, Basso C, Rampazzo A, et al. Clinical profile of four families with arrhythmogenic right ventricular cardiomyopathy caused by dominant desmoplakin mutations. Eur Heart J. 2005;26:1666–1675
  26. Sen-Chowdhry S, Syrris P, Ward D, et al. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression. Circulation. 2007;115:1710–1720
  27. Sen-Chowdhry S, Syrris P, Prasad SK, et al. Left-dominant arrhythmogenic cardiomyopathy: an under-recognized clinical entity. J Am Coll Cardiol. 2008;52:2175–2187
  28. Sievers B, Addo M, Franken U, et al. Right ventricular wall motion abnormalities found in healthy subjects by cardiovascular magnetic resonance imaging and characterized with a new segmental model. J Cardiovasc Magn Res. 2004;6:601–608
  29. Bluemke DA, Krupinski EA, Ovitt T, et al. MR Imaging of arrhythmogenic right ventricular cardiomyopathy: Morphologic findings and interobserver reliability. Cardiology. 2003;99:153–162
  30. Burke AP, Farb A, Tashko G, et al. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases?. Circulation. 1998;97:1571–1580
  31. Fontaine G, Fontaliran F, Zenati O, et al. Fat in the heart (A feature unique to the human species? Observational reflections on an unsolved problem). Acta Cardiol. 1999;54:189–194
  32. Maceira AM, Prasad SK, Khan M, et al. Reference right ventricular systolic and diastolic function normalized to age, gender and body surface area from steady-state free precession cardiovascular magnetic resonance. Eur Heart J. 2006;27:2879–2888
  33. Tandri H, Daya SK, Nasir K, et al. Normal reference values for the adult right ventricle by magnetic resonance imaging. Am J Cardiol. 2006;98:1660–1664
  34. Indik JH, Dallas WJ, Ovitt T, et al. Do patients with right ventricular outflow ventricular arrhythmias have normal right ventricular wall motion? (A quantitative analysis compared to normal subjects). Cardiology. 2005;104:10–15
  35. Bowles N, Ni J, Marcus F, et al. The detection of cardiotropic viruses in the myocardium of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2002;39:892–895
  36. Calabrese F, Angelini A, Thiene G, et al. No detection of enteroviral genome in the myocardium of patients with arrhythmogenic right ventricular cardiomyopathy. J Clin Pathol. 2000;53:382–387
  37. Fontaliran F, Fontaine G, Brestescher C, et al. Signification del infiltrats lymphoplasmocytaires dans la dyplasia ventriculare droite arhythmogene. Arch Mal Coeur. 1995;88:1021–1028
  38. Basso C, Thiene G, Corrado D, et al. Arrhythmogenic right ventricular cardiomyopathy: dysplasia, dystrophy or myocarditis?. Circulation. 1996;94:983–991
  39. Chimenti C, Pieroni M, Maseri A, et al. Histologic findings in patients with clinical and instrumental diagnosis of sporadic arrhythmogenic right ventricular dysplasia. J Am Coll Cardiol. 2004;43:2305–2313
  40. Wichter T, Hindricks G, Lerch H, et al. Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy (An analysis using I-meta-iodobenzylguanidine scintigraphy). Circulation. 1994;89:667–683
  41. Ott P, Marcus F, Sobonya RE, et al. Cardiac sarcoidosis masquerading as right ventricular dysplasia. Pacing Clin Electrophysiol. 2003;1498–1503
  42. Kies P, Bootsma M, Bax JJ, et al. Serial reevaluation for ARVD/C is indicated in patients presenting with left bundle branch block ventricular tachycardia and minor ECG abnormalities. J Cardiovasc Electrophysiol. 2006;17:586–593

 This study was supported by Grant NIH UO1-HL65594.

 Author affiliations are listed in the Appendix.

PII: S1547-5271(09)00288-4

doi: 10.1016/j.hrthm.2009.03.013

Heart Rhythm
Volume 6, Issue 7 , Pages 984-992 , July 2009