Skip to main content
Log in

The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy

  • Originals
  • Published:
Heart and Vessels Aims and scope Submit manuscript

    We’re sorry, something doesn't seem to be working properly.

    Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Summary

Twenty-four patients presenting with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ventricular tachycardia of right ventricular origin associated with structural abnormalities of the right ventricle) were divided into two groups with left ventricular ejection fraction (LVEF) above or below 45%. The distribution of LVEF in the group with LVEF below 45% was comparable with the distribution in 6 patients with idiopathic dilated cardiomyopathy who had ventricular tachycardia originating in the left ventricle (P = 0.2). They also had the same unfavorable long-term prognosis. Therefore, it is suggested that the term, arrhythmogenic right ventricular cardiomyopathy (ARVC), be restricted to patients with a LVEF below 45%. Histological data obtained in the ARVC group showed signs of acute or chronic myocarditis (in the right and left ventricles). It can be hypothesized that patients with arrhythmogenic right ventricular dysplasia (ARVD) may be prone to develop infectious myocarditis. In patients in whom an abnormal host immune response had been seen, progressive deterioration of right and left ventricular function could be observed. This pattern may be superimposed on the genetically determined background of ARVD. This could explain the wide spectrum of clinical presentation observed in patients with tachycardia originating in an abnormal right ventricle.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Fontaine G, Guiraudon G, Frank R, Vedel J, Grosgogeat Y, Cabrol C, Facquet J (1977) Stimulation studies and epicardial mapping in ventricular tachycardia: Study of mechanisms and selection for surgery. In: Kulbertus HE (ed) Reentrant arrhythmias. MTP, Lancaster, pp 334–350

    Google Scholar 

  2. Marcus FI, Fontaine G, Guiraudon G, Frank R, Laurenceau JL, Malergue MC, Grosgogeat Y (1982) Right ventricular dysplasia: A report fo 24 cases. Circulation 65:384–399

    PubMed  Google Scholar 

  3. Manyari DE, Klein GJ, Gulamhusein SS, Kostuk WJ, Boughner DR, Guiraudon G, Wyse DG, Mitchell LB (1983) Arrhythmogenic right ventricular dysplasia: A generalized cardiomyopathy. Circulation 68:251–257

    PubMed  Google Scholar 

  4. Miani D, Pinamonti B, Bussani R, Silvestri F, Sinagra G, Camerini F (1993) Right ventricular dysplasia: A clinical and pathological study of two families with left ventricular involvement. Br Heart J 69:151–157

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  6. Boffa GM, Thiene G, Nava A, Dallavolta S (1991) Cardiomyopathy: A necessary revision of the WHO classification. Int J Cardiol 30:1–7

    PubMed  Google Scholar 

  7. Camerini F, Pinamonti B, Sinagra G, Di Lenarda A, Salvi A, Morgera T, Silvestri F, Bussani R (1990) Left ventricular involvement in right ventricular cardiomyopathy. In: Olsen EGJ, Sekiguchi M (eds) Restrictive cardiomyopathy and arrhythmias. Cardiomyopathy update 3. University of Tokyo Press, Tokyo, pp 255–274

    Google Scholar 

  8. Marcus FI, Fontaine G (1995) Arrhythmogenic right ventricular dysplasia. In: Kowey PR, Podrid PJ (eds) Cardiac arrhythmias: Mechanism, diagnosis and management. Williams and Wilkins, Baltimore, pp 1121–1130

    Google Scholar 

  9. McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, Camerini F (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy Br Heart J 71:215–218

    PubMed  Google Scholar 

  10. Fontaine G, Cansell A, Frank R, Tonet JL, Aaddaj S, Aldakar M, Grosgogeat Y (1991) Catheter ablation techniques for ventricular tachycardia. In: El-Sherif N, Samet Ph (eds). Cardiac pacing and electrophysiology. WB Saunders, Philadelphia, pp 471–483

    Google Scholar 

  11. Aouate P, Fontaliran F, Fontaine G, Frank R, Benassar A, Lascault G, Tonet J, Himbert C, Guerot C (1993) Holter et mort subite. Interet dans un cas de dysplasie ventriculaire droite arythmogene. Arch Mal Coeur 86:363–367

    PubMed  Google Scholar 

  12. Virmani R, Robinowitz M, Clark MA, McAllister HA (1982) Sudden death and partial absence of the right venticular myocardium. Arch Pathol Lab Med 106:163–167

    PubMed  Google Scholar 

  13. Fontaine G, Brestescher C, Fontaliran F, Himbert C, Tonet J, Frank R (1995) Modalites evolutives de la dysplasie ventriculaire droite arythmogene. A propos de 4 observations. Arch Mal Coeur (in press)

  14. Halphen C, Beaufils Ph, Azancot I, Baudouy Ph, Manne B, Slama R (1981) Tachycardies ventriculaires recidivantes par dysplasie ventriculaire droite. Association a des anomalies du ventricule gauche. Arch Mal Coeu 74:1113–1118

    Google Scholar 

  15. Fitchett DH, Sugrue DD, Mac Arthur CG, Oakley CM (1984) Right ventricular dilated cardiomyopathy. Br Heart J 51:25–30

    PubMed  Google Scholar 

  16. Higuchi S, Caglar NM, Shimada R, Yamada A, Takeshita A, Nakamura M (1984) Sixteen-year follow up of arrhythmogenic right ventricular dysplasia. Am Heart J 108:1363–1365

    PubMed  Google Scholar 

  17. Webb JG, Kerr CR, Huckell VF, Mizgala HF, Ricci DR (1986) Left ventricular abnormalities in arrhythmogenic right ventricular dysplasia. Am J Cardiol 58:568–570

    PubMed  Google Scholar 

  18. Daubert JC, Descaves C, Foulgoc JL, Bourdonnec C, Laurent M, Gouffault J (1988) Critical Analysis of Cineangiographic Criteria for Diagnosis of Arrhythmogenic Right Ventricular Dysplasia. Am Heart J 115:448–459

    PubMed  Google Scholar 

  19. Pinamonti B, Sinagra G, Salvi A, Di Lenarda A, Morgera T, Silvestri F, Bussani R, Camerini F (1992) Left ventricular involvement in right ventricular dysplasia. Am Heart J 123:711–724

    PubMed  Google Scholar 

  20. Letac B, Tayot J, Barthes P (1977) Infiltration graisseuse du coeur et maladie de Uhl. (A propos d'une observation de lipomatose cardiaque). Arch Mal Coeur 70:107–113

    PubMed  Google Scholar 

  21. Chomette G, Koulibali M, Linares-Cruz E, Fontaine G, Grosgogeat Y, Cabrol C (1986) Dysplasie arythmogene. Parentes nosologiques avec le syndrome de Uhl et la lipomatose. A propos de trois observations anatomocliniques. Arch Anat Cytol Pathol 34:46–50

    PubMed  Google Scholar 

  22. Gutierrez PS, Ferreira SMF, Lopes EA, Ebaid M (1989) Intramural coronary vessels in partial absence of the myocardium of the right ventricle. Am J Cardiol 63:1152–1154

    PubMed  Google Scholar 

  23. Bharati S, Ciraulo DA, Bilitch M, Rosen KM, Lev M (1978) Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease. Circulation 57:636–644

    PubMed  Google Scholar 

  24. Camerini F, Di Lenarda A, Perkan A, Pinamonti B, Sinagra G (1994) Right ventricular dysplasia/cardiomyopathy: What is the evolution of the disease? In: Sekiguchi M, Richardson PJ (eds) Prognosis and treatment of cardiomyopathies and myocarditis. Cardiomyopathy update 5. University of Tokyo Press, Tokyo, pp 281–294

    Google Scholar 

  25. Ibsen HHW, Baandrup U, Simonsen EE (1985) Familial right ventricular dilated cardiomyopathy. Br Heart J 54:156–159

    PubMed  Google Scholar 

  26. Horimoto M, Funayama N, Satoh M, Igarashi T, Sekiguchi M (1989) Histological evidence of left ventricular involvement in arrhythmogenic right ventricular dysplasia. Jpn Circ J 53:1530–1534

    PubMed  Google Scholar 

  27. Hisaoka T, Kawai S, Ohi H, Ishijima M, Okada R, Hayashida N, Saiki S, Kobayashi H, Yoshimura H (1990) Two cases of chronic myocarditis mimicking arrhythmogenic right ventricular dysplasia. Heart Vessels (Suppl 5):51–54

    Google Scholar 

  28. Saito K, Kuroda A, Arima S, Kawataki M, Tanaka H (1990) Arrhythmogenic right ventricular dysplasia with left ventricular involvement: Report of a case. Heart Vessels (Suppl 5):62–64

    Google Scholar 

  29. Gallo P, D'Amati G, Pelliccia F (1992) Pathologic evidence of extensive left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy. Hum Pathol 23:948–952

    PubMed  Google Scholar 

  30. Bharati S, Feld AW, Bauernfeind RA, Kattus AA, Lev M (1983) Hypoplasia of the right ventricular myocardium with ventricular tachycardia. Arch Pathol Lab Med 107:249–253

    PubMed  Google Scholar 

  31. Goodin JC, Farb A, Smialek JE, Field F, Virmani R (1991) Right ventricular dysplasia associated with sudden death in young adults. Modern Pathol 4:702–706

    Google Scholar 

  32. Sugrue DD, Edwards WD, Olney BA (1985) Histologic abnormalities of the left ventricle in a patient with arrhythmogenic right ventricular dysplasia. Heart Vessels 1:179–181

    PubMed  Google Scholar 

  33. Fontaliran F, Fontaine G, Brestescher C, Vilde F, Labrousse J (1995) Signification des infiltrats lymphoplasmocytaires dans la dysplasie ventriculaire droite arythmogene. Arch Mal Coeur (in press)

  34. Barry WH (1994) Mechanisms of immune-mediated myocyte injury. Circulation 89:2421–2432

    PubMed  Google Scholar 

  35. Matsumori A, Kawai C (1982) An animal model of congestive (dilated) cardiomyopathy: Dilatation and hypertrophy of the heart in the chronic stage in DBA/2 mice with myocarditis caused by encephalomyocarditis virus. Circulation 66:355–360

    PubMed  Google Scholar 

  36. Matsumori A, Kishimoto C, Kawai C, Sawada S (1983) Right ventricular aneurysms complicating encephalomyocarditis virus myocarditis in mice. Jpn Circ J 47:1322–1324

    PubMed  Google Scholar 

  37. Rampazzo A, Nava A, Danieli GA, Buja G, Daliento L, Fasoli G, Scognamiglio R, Corrado D, Thiene G (1994) The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23–q24 Hum Mol Genet 3:959–962

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Fontaine, G., Fontaliran, F., Rosas Andrade, F. et al. The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy. Heart Vessels 10, 227–235 (1995). https://doi.org/10.1007/BF01744901

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01744901

Key words

Navigation