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MRI assessment of right ventricular dysplasia

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Abstract

Right ventricular dysplasia is a new entity of unknown origin in the classification of cardiomyopathies. Also known as arrhythmogenic right ventricular cardiomyopathy (ARVC) or arrhythmogenic right ventricular dysplasia, it is a disease of the heart muscle characterised by fibroadipose atrophy mainly involving the right ventricle and responsible for severe ventricular arrhythmias and sudden death also in young people. Magnetic resonance imaging provides evidence of ventricular dilatation at the outflow tract, thinning and thickening of the wall, diastolic bulging areas (especially located at the level of the right ventricle outflow tract) and fatty substitution of the myocardium mainly at the level of the right ventricle. Many radiologists erroneously consider the previously described fatty substitution as the main sign of ARVC, even though an evaluation of fat substitution alone may be a source of error for two reasons: firstly, because isolated areas of fatty replacement are not synonymous with ARVC since small non-transmural focal fatty areas of fat are also present in the normal patients; and secondly, because the MRI detection of fat may be overestimated due to partial-volume artefacts with normal subepicardial fat. Cardiac MRI can also be employed for the diagnosis of idiopathic right ventricular outflow tract tachycardia. Considering the evolutive nature of the disease, the non-invasiveness of MRI allows the follow-up of these patients and may be considered an excellent screening modality for the diagnosis of ARVC in family members. Finally, MRI can be employed in electrophysiological studies to locate the arrhythmogenic focus and reduce sampling errors.

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References

  1. Richardson P, McKenna W, Bristow M et al. (1996) Report of the World Health Organisation International Society and Federation of Cardiology Task Force on the definition and classification of the cardiomyopathies. Circulation 93:841–42

    PubMed  Google Scholar 

  2. Thiene G, Nava A, Corrado D et al. (1988) Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318:129–133

    CAS  PubMed  Google Scholar 

  3. Corrado D, Thiene G, Nava A et al. (1990) Sudden death in young competitive athletes: clinicopathologic correlation in 22 cases. Am J Med 89:588–595

    CAS  PubMed  Google Scholar 

  4. Maron BJ, Roberts WC, McAllister HA et al. (1980) Sudden death in young athletes. Circulation 62:218–229

    CAS  PubMed  Google Scholar 

  5. Topaz O, Edwards JE (1985) Pathologic features of sudden death in children, adolescent and young adults. Chest 87:476–482

    Google Scholar 

  6. Marcus FI, Fontaine GH, Guiraudon G et al. (1982) Right ventricular dysplasia:a report of 24 adult cases. Circulation 65:384–398

    CAS  PubMed  Google Scholar 

  7. Uhl HSM. (1952) A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 91:197–209

    Google Scholar 

  8. Basso C, Thiene G, Corrado D et al. (1996) Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis? Circulation 94:983–991

    CAS  PubMed  Google Scholar 

  9. Fontaine G, Frank R, Tonet JL et al. ( 1984) Arrhythmogenic right ventricular dysplasia: a clinical model for the study of chronic ventricular tachycardia. Jpn Circ J 48:515–538

    CAS  PubMed  Google Scholar 

  10. Ananthasubramanian K, Kahaia F (1998) Arrhythmogenic right ventricular dysplasia/cardiomyopathy: review for clinician. Prog Cardiovasc Dis 50:423–427

    Google Scholar 

  11. Fontaine G, Fontaliran F, Herbert JL et al. (1999) Arrhythmogenic right ventricular dysplasia. Ann Rev Med 50:17–35

    Article  CAS  PubMed  Google Scholar 

  12. Van der Wall EE, Kayser HWM, Bootsma MM et al. (2000) Arrhythmogenic right ventricular dysplasia: MRI findings. Hertz 25:356–364

    Google Scholar 

  13. Leclerq JF, Rosengarten MD, Attuel P et al. (1981) L'extrasistolie ventriculaire idiopatique: une parasystolie ventriculaire droite protégée du rythme sinusal? Arch Mal Coeur 74:1249–1261

    Google Scholar 

  14. Kaziniererzak J, De Sutter J, Travemier R et al. (1998) Electrocardiographic end morphometric features in patients with ventricular tachycardia of right ventricular origin. 79:2049–2058

  15. Toyofuku M, Takaki H, Sunagawa H et al. (1999) Exercise-induced ST-elevation in patients with arrhythmogenic right ventricular dysplasia. J Electrocardiol 32:1–5

    CAS  Google Scholar 

  16. Chiddo A, Locuratolo N, Gaglione A et al. (1989) Right ventricular dysplasia: angiographic study: Eur Heart J 10 (Suppl D):22–26

    Google Scholar 

  17. Kisslo J (1989) Two-dimensional echocardiography in arrythmogenic right ventricular dysplasia. Eur Heart J 10 (Suppl D):26–29

    PubMed  Google Scholar 

  18. Mehta D, Odawara H, Ward DE et al. (1989) Echocardiographic and histological evaluation of the right ventricle in ventricular tachycardias of left bundle band block origin without over cardiac abnormality. Am J Cardiol 3:939–944

    Google Scholar 

  19. McKenna JW, Thiene G, Nava A et al. (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 71:215–218

    CAS  PubMed  Google Scholar 

  20. Strain J (1989) Adipose dysplasia of the right ventricle: Is endomyocardial biopsy useful? Eur Heart J 10 (Suppl D):84–88

    PubMed  Google Scholar 

  21. Casolo GC, Poggesi L, Boddi M (1987) Ecg gated magnetic resonance imaging in the right ventricular dysplasia. Am Heart J 113:1245–1248

    CAS  PubMed  Google Scholar 

  22. Ricci C, Longo R, Pagnan L et al. (1992) Magnetic resonance imaging in right ventricular dysplasia. Am J Cardiol 70:1589–1595

    CAS  PubMed  Google Scholar 

  23. Blake LM, Scheiman MM, Higgins CB (1994) MR features of arrhythmogenic right ventricular dysplasia. AJR 162:809–812

    CAS  Google Scholar 

  24. Mengetti L, Basso C, Nava A et al. (1996) Spin echo nuclear magnetic resonance for tissue characterisation in arrhythmogenic right ventricular dysplasia. Heart 76:467–470

    PubMed  Google Scholar 

  25. Midiri M, Finazzo M, Brancato M et al. (1997) Arrhythmogenic right ventricular dysplasia: MR features. Eur Radiol 7:307–312

    Article  CAS  PubMed  Google Scholar 

  26. Sommer T, Lewater T, Bierhoff E et al. (1998) MRI diagnosis of the right ventricular dysplasia. Röfo Fortschr Geb Rontgenstr Neuen Bildgeb Vefahr 169:609–615

    Google Scholar 

  27. Zandrino F, Molinari G, Smeraldi A et al. (2000) Magnetic resonance imaging of athlete's heart: myocardial mass, left ventricular function, and cross-sectional area of the coronary arteries. Eur Radiol 10:309–325

    Article  Google Scholar 

  28. Cesare E di, Catalucci A, Masciocchi C (1999) Left ventricular function: MRI assessment. Rays 24:19–32

    PubMed  Google Scholar 

  29. Cesare E di, Guglielmo A, Moscini P et al. (1995) MRI morphological and functional analysis of the right ventricle in patients with ventricular and arrhythmia. Eur Radiol 11 (Suppl 1):94

    Google Scholar 

  30. Catalucci A, Santarelli B, Cerone G et al. (1999) Arrhythmogenic right ventricular dysplasia: intra- and interobserver variability in the MRI evaluation. Eur Radiol (Suppl 1) 9:307

    Google Scholar 

  31. Cesare E di (2001) MRI of the cardiomyopathies. Eur J Radiol 38:179–184

    Article  PubMed  Google Scholar 

  32. Aufferman W, Wichter T, Breithardt G et al. (1993) Arrhythmogenic right ventricular disease: MR imaging vs angiography.

    Google Scholar 

  33. Neubauer S, Revel D, Roos A et al. de (1998) The clinical role of magnetic resonance in cardiovascular disease. Eur Heart J 19:19–39

    Article  PubMed  Google Scholar 

  34. Carlson MD, Withe RD, Troman RG et al. (1994) Right ventricular outflow tract ventricular tachycardia. J Am Coll Cardiol 24:720–727

    CAS  PubMed  Google Scholar 

  35. Proclemer A, Basadonna T, Slavich GA et al. (1997) Cardiac magnetic resonance imaging findings in patients with right ventricular outflow tract premature contractions. Eur Heart J 18:2002–2010

    CAS  PubMed  Google Scholar 

  36. Withe RD, Trohman RG, Flamm SD et al. () Right ventricular arrhythmia in the absence of arrhythmogenic dysplasia: MR of myocardial abnormalities. Radiology:743–751

  37. Gaita F, Giustetto C, Donna P di et al. (2001) Long-term follow-up of right ventricular monomorphic extrasystoles. J Am Coll Cardiol 38:364–370

    Article  CAS  PubMed  Google Scholar 

  38. Globits S, Kreiner G, Frank H et al. (1997) Significance of morphological abnormalities detected by MRI in patients undergoing successful ablation right of right ventricular outflow tract tachycardia. Circulation 96:2633–2640

    CAS  PubMed  Google Scholar 

  39. Simonetti OP, Finn JP, White RD et al. (1996) Black blood T2 weighted inversion recovery MR imaging of the heart. Radiology 199:49–57

    CAS  PubMed  Google Scholar 

  40. Erriquez D, Di Cesare E, Barile A et al. (2002) Cardiac MRI: comparison between single-shot fast spin echo and conventional spin echo sequences in the morphological evaluation of the ventricles. Radiol Med 103:34–44

    Google Scholar 

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  1. Department of Radiology, University of L'Aquila, via Vetoio 1, 67100 , L'Aquila, Italy

    Ernesto Di Cesare

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  1. Ernesto Di Cesare
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Correspondence to Ernesto Di Cesare.

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Di Cesare, E. MRI assessment of right ventricular dysplasia. Eur Radiol 13, 1387–1393 (2003). https://doi.org/10.1007/s00330-002-1771-x

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  • DOI: https://doi.org/10.1007/s00330-002-1771-x

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