Journal of Electrocardiology
Volume 42, Issue 2 , Pages 137.e1-137.e10 , March 2009

Cardiac imaging in right ventricular cardiomyopathy/dysplasia—how does cardiac imaging assist in understanding the morphologic, functional, and electrical changes of the heart in this disease?

  • Vincent L. Sorrell, MD, FACC, FACP, FASE

      Affiliations

    • Corresponding Author InformationCorresponding author. Clinical Medicine (Cardiology), Radiology, Pediatrics, The Allan C Hudson and Helen Lovaas Endowed Chair of Cardiac Imaging, University of Arizona and the Sarver Heart Center, Tucson, AZ 85724-5037, USA. Tel.: +1 520 626 1416; fax: +1 520 626 4333.
  • ,
  • Sachin Kumar, MD
  • ,
  • Nishant Kalra, MD

Received 19 November 2008

References 

  1. Marcus FI, Fontaine GH, Guiradon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65:384
  2. Theine G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988;318:129
  3. Nava A, Thiene G, Canciani B, et al. Familial occurrence of right ventricular dysplasia; a study involving 9 families. J Am Coll Cardiol. 1988;12:1222
  4. McKenna WJ, Thiene G, Canciani B, 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 Jr. 1994;71:215
  5. Yang Z, Bowles NE, Shcerer SE, et al. Desmosomal dysfunction due to mutations in desmoplakin causes arrythmogenic right ventricular dysplasia/cardiomyopathy. Circ Res. 2006;99:646
  6. Awad MM, Calkins H, Judge DP. Mechanisms of disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Nat Clin Pract Cardiovasc Med. 2008;5:258
  7. Daliento L, Rizzoli G, Thiene G, et al. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol. 1990;66:741
  8. Corrado D, Basso C, Leoni L, et al. Three-dimensional electroanatomic voltage mapping increases accuracy of diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation. 2005;111:3042
  9. Nava A, Canciani B, Buja G, et al. Electrovectorcardiographic study of negative T waves on precordial leads in arrhythmogenic right ventricular dysplasia: relationship with right ventricular volumes. J Electrocardiol. 1988;21:239
  10. Corrado D, Thiene G. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: clinical impact of molecular genetic studies. Circulation. 2006;113:1634
  11. Basso C, Czarnowska E, Barbera MD, et al. Ultrastructural evidence of intercalated disc remodeling in arrhythmogenic right ventricular cardiomyopathy: an electron microscopy investigation on endomyocardial biopsies. Eur Heart J. 2006;27:1847
  12. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30:1512
  13. Tabib A, Loire R, Chalabreysse L, et al. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia. Circulation. 2003;108:3000
  14. Altemose G, Buxton A. Idiopathic ventricular tachycardia. Annu Rev Med. 1999;50:159
  15. 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
  16. Abou Jaoude S, Leclercq JF, Coumel P. Progressive ECG changes in arrhythmogenic right ventricular disease—evidence for an evolving disease. Eur Heart J. 1996;17:1717
  17. Nava A, Thiene G, Canciani B, et al. Familial occurrence of right ventricular dysplasia: a study involving nine families. J Am Coll Cardiol. 1988;12:1222
  18. Mulvagh SL, Rakowski H, Vannan MA, et al. American Society of Echocardiography consensus statement on the clinical applications of ultrasonic contrast agents in echocardiography. J Am Soc Echocardiogr. 2008;21:1179
  19. Scognamiglio R, Fasoli G, Nava A, et al. Contribution of cross-sectional echocardiography to the diagnosis of right ventricular dysplasia. Eur Heart J. 1989;10:538
  20. Scognamiglio R, Fasoli G, Nava A. Relevance of subtle echocardiographic findings in the early diagnosis of the concealed form of right ventricular dysplasia. Eur Heart J. 1989;(Suppl D):27
  21. Marcus F, Towbin JA, Zareba W, et al. A multidisciplinary study: design and protocol. Circulation. 2003;107:2975
  22. Yoerger DM, Marcus FI, Sherill D, et al. Echocardiographic findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia. J Am Coll Cardiol. 2005;45:860
  23. Teske AJ, De Boeck BWL, Melman PG, et al. Echocardiographic quantification of myocardial function using tissue deformation imaging, a guide to image acquisition and analysis using tissue Doppler and speckle tracking. Cardiovascular Ultrasound. 2007;5:27
  24. Prakasa KR, Wang J, Tandri H, et al. Utility of tissue Doppler and strain echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Cardiol. 2007;100:507
  25. Lindström L, Wilkenshoff UM, Larsson H, Wranne B. Echocardiographic assessment of arrhythmogenic right ventricular cardiomyopathy. Heart. 2001;86:31
  26. Kjaergaard J, Svendsen JH, Sogaard P, et al. Advanced quantitative echocardiography in arrythmogenic right ventricular cardiomyopathy. J Am Soc Echocardiogr. 2007;20:27
  27. Prakasa K, Dalal D, Wang J, et al. Feasibility and variability of three dimensional echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Cardiol. 1997;5:703
  28. Bellenger NG, Francis JM, Davies CL, Coats AJ, Pennell DJ. Establishment and performance of a magnetic resonance cardiac function clinic. J Cardiovasc Magn Reson. 2000;2:15
  29. Tandri H, Bomma C, Calkins H, et al. Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia. J Magn Reson Imaging. 2004;6:848
  30. Kiès P, Bootsma M, Bax J, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: screening, diagnosis, and treatment. Heart Rhythm. 2006;3:225
  31. Tandri H, Calkins H, Nasir K, et al. Magnetic resonance imaging findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia. J Cardiovasc Electrophysiol. 2003;14:476
  32. Tandri H, Macedo R, Marcus F, et al. Role of magnetic resonance imaging in arrhythmogenic right ventricular dysplasia. Insights from the North American arrhythmogenic right ventricular dysplasia. Am Heart J. 2008;155:289
  33. 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 Mag Reson. 2004;6:601
  34. Roux JF, Dubuc M, Pressacco J, et al. Concordance between an electroanatomic mapping system and cardiac MRI in arrhythmogenic right ventricular cardiomyopathy. Pacing Clin Electrophysiol. 2006;29:109
  35. Zafar HM, Litt HI, Torigian DA. CT imaging features and frequency of left ventricular myocardial fat in patients with CT findings of chronic left ventricular myocardial infarction. Clin Radiol. 2008;63:256
  36. Boxt LM. Radiology of the right ventricle. Radiol Clin North Am. 1999;37:379
  37. Midiri M, Finazzo M, Brancato M, et al. Arrythmogenic right ventricular dysplasia: MR features. Eur Radiol. 1997;7:307
  38. Castillo E, Tandri H, Rodriguez ER, et al. Arrhythmogenic right ventricular dysplasia: ex vivo and in vivo fat detection with black-blood MR imaging. Radiology. 2004;232:38
  39. Abbara S, Migrino RQ, Sosnovik DE, Leichter JA, Brady TJ, Holmvang G. Value of fat suppression in the MRI evaluation of suspected arrhythmogenic right ventricular dysplasia. AJR Am J Roentgenol. 2004;182:587
  40. Macedo R, Prakasa K, Tichnell C, et al. Marked lipomatous infiltration of the right ventricle: MRI findings in relation to arrhythmogenic right ventricular dysplasia. AJR Am J Roentgenol. 2007;188:423
  41. Burke AP, Farb A, Tashko G, Virmani R. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium. Circulation. 1998;97:1571
  42. Jain A, Tandri H, Calkins H, et al. Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia. J Cardiovasc Magn Reson. 2008;10:32
  43. Bluemke DA, Krupinski EA, Ovitt T, et al. MR imaging of arrhythmogenic right ventricular cardiomyopathy: morphologic findings and interobserver reliability. Cardiology. 2003;99:153
  44. Tandri H, Castillo E, Ferrari V, et al. Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia—sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle. J Am Coll Cardiol. 2006;48:2277
  45. Aufferman W, Wichter T, Breithardt G, et al. Arrhythmogenic right ventricular disease: MR imaging versus vs. angiography. Am J Radiol. 1993;161:549
  46. Wichter T, et al. MRI. In:  Nava A,  Rossi L,  Thiene G editor. ARVC/D. Amsterdam: Elsevier; 1997;p. 269
  47. Altbach MI, Li Z, Bilgin A, et al. Interleaved acquisition of lipid and water images of the heart using a double-inversion fast spin-echo method. Magn Reson Med. 2005;54:1562
  48. Pinamonti B, Sinagra G, Salvi A, et al. Left ventricular involvement in right ventricular dysplasia. Am Heart J. 1992;123:711
  49. Higuchi S, Caglar NM, Shimada R, Yamada A, Takeshita A, Nakamura M. 16-year follow-up of arrhythmogenic right ventricular dysplasia. Am Heart J. 1984;108:1363
  50. Becker CR, Ohnesorge BM, Schoepf UJ, et al. Current development of cardiac imaging with multidetector row CT. Eur J Radiol. 2000;36:97
  51. Kim RJ, Fieno DS, Parrish TB, et al. Relationship of MRI delayed contrast enhancement to irreversible injury, infarct age, and contractile function. Circulation. 1999;100:1992
  52. Nazarian S, Bluemke DA, Lardo AC, et al. Magnetic resonance assessment of the substrate for inducible ventricular tachycardia in nonischemic cardiomyopathy. Circulation. 2005;112:2821
  53. Nemeth MA, Muthupillai R, Wilson JM, Awasthi M, Flamm SD. Cardiac sarcoidosis detected by delayed-hyperenhancement magnetic resonance imaging. Tex Heart Inst J. 2004;31:99
  54. Babu-Narayan SV, Kilner PJ, Li W, et al. Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome. Circulation. 2006;113:405
  55. Tandri H, Saranathan M, Rodriguez E, et al. Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging. J Am Coll Cardiol. 2005;45:98
  56. Turrini P, Angelini A, Theine G, et al. Late potentials and ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol. 1999;83:1214
  57. Bomma C, Rutberg J, Tandri H, et al. Misdiagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Cardiovasc Electrophysiol. 2004;15:300
  58. Dery R, Lipton MJ, Garrett JS, et al. Cine-computed tomography of arrhythmogenic right ventricular dysplasia. J Comput Assist Tomogr. 1986;10:120
  59. Tada H, Shimizu W, Ohe T, et al. Usefulness of electron beam computed tomography in arrhythmogenic right ventricular dysplasia. Circulation. 1996;94:437
  60. Hamada S, Takamiya M, Ohe T, et al. Arrhythmogenic right ventricular dysplasia: evaluation with electron beam CT. Radiology. 1993;187:723
  61. Tandri H, Bomma C, Calkins H, et al. Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia. J Magn Reson Imaging. 2004;19:848
  62. Bomma C, Tandri H, Nasir K, et al. Role of helical CT in qualitative and quantitative evaluation of arrhythmogenic right ventricular dysplasia. Pacing Clin Electrophysiol. 2003;26(suppl):96
  63. Sisson JC, Shapiro B, Meyers L, et al. Metaiodobenzylguanidine to map scintigraphically the adrenergic nervous system in man. J Nucl Med. 1987;28:1625
  64. Agostini D, Verberne HJ, Burchert W, et al. I-123-mIBG myocardial imaging for assessment of risk for a major cardiac event in heart failure patients: insights from a retrospective European multicenter study. Eur J Nucl Med Mol Imaging. 2008;35:535
  65. Paul M, Schafers M, Kies P, et al. Impact of sympathetic innervation on recurrent life-threatening arrhythmias in the follow-up of patients with idiopathic ventricular fibrillation. Eur J Nucl Med Mol Imaging. 2006;33:866
  66. Takahashi N, Ishida Y, Maeno M, et al. Noninvasive identification of left ventricular involvements in arrhythmogenic right ventricular dysplasia: comparison of 123I-MIBG, 201TlCl, magnetic resonance imaging and ultrafast computed tomography. Ann Nucl Med. 1997;11:233
  67. Wichter T, Hindricks G, Lerch H, et al. Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy: an analysis using 123I-meta-iodobenzylguanidine scintigraphy. Circulation. 1994;89:667
  68. Mariano-Goulart D, Dechaux L, Rouzet F, et al. Diagnosis of diffuse and localized ARVD by gated blood-pool SPECT. J Nucl Med. 2007;48:1416

PII: S0022-0736(08)00511-6

doi: 10.1016/j.jelectrocard.2008.12.019

Journal of Electrocardiology
Volume 42, Issue 2 , Pages 137.e1-137.e10 , March 2009