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.
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References
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
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
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
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
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
Boffa GM, Thiene G, Nava A, Dallavolta S (1991) Cardiomyopathy: A necessary revision of the WHO classification. Int J Cardiol 30:1–7
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
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
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
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
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
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
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)
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
Fitchett DH, Sugrue DD, Mac Arthur CG, Oakley CM (1984) Right ventricular dilated cardiomyopathy. Br Heart J 51:25–30
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
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
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
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
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
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
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
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
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
Ibsen HHW, Baandrup U, Simonsen EE (1985) Familial right ventricular dilated cardiomyopathy. Br Heart J 54:156–159
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
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
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
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
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
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
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
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)
Barry WH (1994) Mechanisms of immune-mediated myocyte injury. Circulation 89:2421–2432
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
Matsumori A, Kishimoto C, Kawai C, Sawada S (1983) Right ventricular aneurysms complicating encephalomyocarditis virus myocarditis in mice. Jpn Circ J 47:1322–1324
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
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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
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DOI: https://doi.org/10.1007/BF01744901