Is Atrial Vulnerability Increased In Arrhythmogenic Right Ventricular Cardiomyopathy?

  • D. Corrado
  • G. Buja
  • C. Basso
  • A. Nava
  • G. Thiene
Conference paper


Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle dis- ease, often familial, that is characterized pathologically by fibro-fatty replacement of the right ventricular (RV) myocardium and clinically by ventricular arrhyth- mias of RV origin leading to sudden death, mostly in young people and athletes [1–8]. The progressive loss of the RV myocardium has been considered the result of a genetically determined dystrophic process, an inflammatory myocardial injury and death, or a programmed cell death (“apoptosis”) [1–6,9]. The most fre- quent clinical features of ARVC consist of ventricular arrhythmias with left bun- dle branch block morphology ranging from isolated premature ventricular beats to sustained ventricular tachycardia or ventricular fibrillation [1–7, 10]. Other common clinical manifestations of the disease include global and/or regional dysfunction and structural alterations of the right ventricle or both ventricles, and ECG depolarization/repolarization changes, mostly localized to right precor- dial leads [11]; supraventricular arrhythmias (SVA) -mostly atrial fibrillation and flutter- represent a less frequent clinical feature [12–15]. Whatever the etiopatho- genetic mechanisms involved, there is definitive clinico-pathological evidence that ARVC is a progressive heart muscle disease [6]. Clinico-pathological investi- gations and long-term follow-up data from clinical studies indicate that ARVC with time may lead to more diffuse RV changes and left ventricular involvement culminating in heart failure [6,16,17]. Therefore, the natural history of ARVC is a function of both the electrical instability of diseased ventricular myocardium, which can precipitate “arrhythmic” cardiac arrest any time during the disease course, and the progressive myocardial loss that results in right or biventricular dysfunction and heart failure.


Right Ventricular Atrial Flutter Atrial Tachycardia Arrhythmogenic Right Ventricular Cardiomyopathy Ventricular Cardiomyopathy 
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  1. 1.
    Marcus FI, Fontaine G, Guiraudon G et al (1982) Right ventricular dysplasia. A report of 24 adult cases. Circulation 65:384–398PubMedCrossRefGoogle Scholar
  2. 2.
    Thiene G, Nava A, Corrado D, Rossi L, Pennelli N (1988) Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318:129–133PubMedCrossRefGoogle Scholar
  3. 3.
    Nava A, Rossi L, Thiene G (eds) (1997) Arrhythmogenic right ventricular cardiomyopathy- dysplasia. Elsevier, AmsterdamGoogle Scholar
  4. 4.
    Thiene G, Nava A, Angelini A, Daliento L, Scognamiglio R, Corrado D (1988) Anatomoclinical aspects of arrhythmogenic right ventricular cardiomyopathy. In: Baroldi G, Camerini F, Goodwin JF (eds) Advances in cardiomyopathy. Springer Milan, pp 397–408Google Scholar
  5. 5.
    Basso C, Thiene G, Corrado D, Angelini A, Nava A, Valente M (1996) Arrhythmogenic right ventricular cardiomyopathy: dysplasia, dystrophy, or myocarditis? Circulation 94:983–991PubMedCrossRefGoogle Scholar
  6. 6.
    Corrado D, Basso C, Thiene G et al (1997) Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol 30:1512–1520PubMedCrossRefGoogle Scholar
  7. 7.
    Corrado D, Thiene G, Nava A, Rossi L, Pennelli N (1990) Sudden death in young competitive athletes: clinicopathologic correlation in 22 cases. Am J Med 89:588–596PubMedCrossRefGoogle Scholar
  8. 8.
    Corrado D, Basso C, Schiavon M, Thiene G (1998) Screening for hypertrophic cardiomyopathy in young athletes. New Engl J Med 339:364–369PubMedCrossRefGoogle Scholar
  9. 9.
    Valente M, Calabrese F, Angelini A, Basso C, Thiene G (1998) In vivo evidence of apoptosis in arrhythmogenic right ventricular cardiomyopathy. Am J Pathol 152:479–484PubMedGoogle Scholar
  10. 10.
    Fontaine G, Frank R, Fontaliran F, Lascault G, Tonet J (1992) Right ventricular tachycardias. In: Parmley WW, Chatteryce K (eds) Cardiology. Lippincott, New York 1:1–17Google Scholar
  11. 11.
    McKenna WJ, Thiene G, Nava A et al (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 71:215–218PubMedCrossRefGoogle Scholar
  12. 12.
    Fichett DH, Sugrue DD, Mac Arthur CG, Oakley CM (1984) Right ventricular dilated cardiomyopathy. Br Heart J 51:25–30CrossRefGoogle Scholar
  13. 13.
    Klein LW, Horowitz LN (1988) Familial right ventricular dilated cardiomyopathy associated with supraventricular arrhythmias. Am J Cardiol 62:482–483PubMedCrossRefGoogle Scholar
  14. 14.
    Tonet JL, Castro-Miranda R, Iwa T, Poulain F, Frank R, Fontaine G (1991) Frequency of supraventricular tachyarrhythmias in arrhythmogenic right ventricular dysplasia. Am J Cardiol 67:1153PubMedCrossRefGoogle Scholar
  15. 15.
    Brembilla-Perrot B, Jacquemin L, Houplon P et al (1998) Increased atrial vulnerability in arrhythmogenic right ventricular cardiomyopathy. Am Heart J 135:748–754PubMedCrossRefGoogle Scholar
  16. 16.
    Blomström-Lundqvist C, Säbel CG, Olsson SB (1987) A long term follow up of 15 patients with arrhythmogenic right ventricular dysplasia. Br Heart J 58:477–488PubMedCrossRefGoogle Scholar
  17. 17.
    Pinamonti B, Sinagra G, Salvi A et al (1992) Left ventricular involvement in right ventricular dysplasia. Am Heart J 123:711–724PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2000

Authors and Affiliations

  • D. Corrado
    • 1
  • G. Buja
    • 1
  • C. Basso
    • 1
  • A. Nava
    • 1
  • G. Thiene
    • 1
  1. 1.Dipartimenti di Cardiologia e PatologiaUniversità di PadovaPaduaItaly

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