Skip to main content
SpringerLink
Log in

Arrhythmogene rechtsventrikuläre Kardiomyopathie

Arrhythmogenic right ventricular cardiomyopathy: Etiology, diagnosis, and treatment

Ätiologie, Diagnostik und Therapie

  • Übersicht
  • Published:
Medizinische Klinik Aims and scope Submit manuscript

Zusammenfassung

□ Die arrhythmogene rechtsventrikuläre Kardiomyopathie (ARVC) ist charakterisiert durch regional betonte Degeneration und Atrophie des rechtsventrikulären Myokards mit nachfolgendem Ersatz durch Fett- und Bindewebe. Die Erkrankung manifestiert sich im Jugend- oder jungen Erwachsenenalter, Männer sind häufiger betroffen. Leitsymptome der ARVC sind ventrikuläre Tachyarrhythmien mit linksschenkelblockartiger Konfiguration. Auch plötzliche Herztodesfälle können die primäre Manifestation der ARVC darstellen.

□ Charakteristische Befunde sind Repolarisationsstörungen und eine Verlängerung des QRS-Komplexes in den rechtspräkordialen Ableitungen des Oberflächen-EKG sowie regional betonte Störungen der Struktur und Funktion des Rechten Ventrikels. Eine linksventrikuläre Beteiligung wird erst in fortgeschrittenen Krankheitsstadien beobachtet und kann in seltenen Fällen zu einer biventrikulären Herzinsuffizienz führen.

□ Im Vordergrund der Therapie stehen die Behandlung der Kammertachykardien und die Prophylaxe des plötzlichen Herztodes. Der individuelle Einsatz von antiarrhythmischer Pharmakotherapie, Katheterablation und Defibrillatorimplantation kann zur Verbesserung der Langzeitprognose von Patienten mit ARVC beitragen.

Summary

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by regional atrophy of right ventricular myocardium and subsequent replacement by fatty and fibrous tissue. The disease manifests in young adult-hood with a predominance of males. Hallmarks of ARVC are ventricular tachyarrhythmias of left bundle branch block pattern which frequently occur during exercise. However, sudden death may also be the first manifestation of the disease.

□ Characteristic findings are repolarization abnormalities and QRS prolongation in the right precordial leads of the surface ECG and regional abnormalities of right ventricular structure and wall motion. Left ventricular involvement may occur in later stages of the disease but rarely leads to progressive biventricular heart failure.

□ Therapeutic efforts are mainly directed to the treatment of ventricular tachyarrhythmias and the prevention of sudden death. A tailored treatment strategy including antiarrhythmic drug therapy, catheter ablation and implantation of cardioverter-defibrillators may be used to improve the long-term prognosis of patients with ARVC.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Literatur

  1. Angelini A, Basso C, Nava A, Thiene G. Endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy. Am Heart J 1996;132:203–6.

    Article  PubMed  CAS  Google Scholar 

  2. Auffermann W, Wichter T, Breithardt G, Joachimsen K, Peters PE. Arrhythmogenic right ventricular diasease: MR imaging versus angiography. Am J Radiology 1993;161:549–55.

    CAS  Google Scholar 

  3. Basso C, Thiene G, Corrado D, Angelini A, Nava A, Valente M. Arrhythmogenic right ventricular cardiomyopathy: dysplasia, dystrophy or myocarditis? Circulation 1996;94:983–91.

    PubMed  CAS  Google Scholar 

  4. Blake LM, Scheinman MM, Higgins CB. MR features of arrhythmogenic right ventricular dysplasia. Am J Radiolol 1994;162:809–12.

    CAS  Google Scholar 

  5. Blomström-Lundqvist C, Beckmann-Suurküla M, Wallentin I, Jonsson R, Olsson SB. Ventricular dimensions and wall motion assessed by echocardiography in patients with arrhythmogenic right ventricular dysplasia. Eur Heart J 1988;9:1291–302.

    PubMed  Google Scholar 

  6. Blomström-Lundqvist C, Hirsch I, Olsson SB. Quantitative analysis of the signal averaged QRS in patients with the syndrome of arrhythmogenic right ventricular dysplasia. Eur Heart J 1988;9:301–12.

    PubMed  Google Scholar 

  7. Blomström-Lundqvist C, Sabel KG, Olsson SB. A long-term follow up of 15 patients with arrhythmogenic right ventricular dysplasia. Br Heart J 1987;58:477–88.

    Article  PubMed  Google Scholar 

  8. Bourguignon MH, Sebag C, Le Guludec D, et al. Arrhythmogenic right ventricular dysplasia demonstrated by phase mapping of gated equilibrium radioventriculography. Am Heart J 1986;111:997–1000.

    Article  PubMed  CAS  Google Scholar 

  9. Breithardt G, Borggrefe M, Wichter T. Catheter ablation of idiopathic right ventricular tachycardia (editorial comment). Circulation 1990;82:2273–6.

    PubMed  CAS  Google Scholar 

  10. Breithardt G, Wichter T, Haverkamp W, et al. Implantable cardioverter defibrillator therapy in patients with arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, or no structural heart disease. Am Heart J 1994;127:Suppl:1151–8.

    Article  PubMed  CAS  Google Scholar 

  11. Brooks R, Burgess JH. Idiopathic ventricular tachyardia —a review. Medicine (Baltimore) 1988;67:271–94.

    CAS  Google Scholar 

  12. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrophysiologic syndrome. J Am Coll Cardiol 1992;20:1391–6.

    PubMed  CAS  Google Scholar 

  13. Canu G, Atallah G, Claudel JP, et al. Prognostic et évolution à long terme de la dysplasie arythmogène du ventricule droit. Arch Mal Coeur 1993;86:41–8.

    PubMed  CAS  Google Scholar 

  14. Corrado D, Nava A, Buja G, Martini B, Fasoli G, Oselladore L, et al. Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death. J Am Coll Cardiol 1996;27: 443–8.

    Article  PubMed  CAS  Google Scholar 

  15. Corrado D, Thiene G, Nava A, et al. Exercise-related sudden death in the young. Eur Heart J 1993;14:Suppl: 368A.

    Google Scholar 

  16. Daliento L, Rizzoli G, Thiene G, et al. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 1990;60: 741–5.

    Article  Google Scholar 

  17. Dalla Volta S, Battaglia G, Zerbini E. “Auricularisation” of right ventricular pressure curve. Am Heart J 1961;61: 25–33.

    Article  PubMed  CAS  Google Scholar 

  18. Daubert JC, Descaves C, Foulgoc JL, Bourdonnec C, Laurent M, Gouffault J. Critical analysis of cineangiographic criteria for diagnosis of arrhythmogenic right ventricular dysplasia. Am Heart J 1988;115:448–59.

    Article  PubMed  CAS  Google Scholar 

  19. Fontaine G, Frank R, Rougier I, et al. Electrode catheter ablation of resistant ventricular tachycardia in arrhythmogenic right ventricular dysplasia. Experience in 13 patients with a mean follow-up of 45 months. Eur Heart J 1989;10:Suppl D:74–81.

    PubMed  Google Scholar 

  20. Fontaine G, Guiraudon G, Frank R, et al. Stimulation studies and epicardial mapping in ventricular tachycardia: study of mechanism and selection for surgery. In: Kulbertus HE, ed. Reentrant arrhythmias. Lancaster: MTP, 1977:334–50.

    Google Scholar 

  21. Fontaine G, Umemura J, DiDonna P, et al. La durée des complexes QRS dans la dysplasie ventriculaire droites arythmogène. Un nouveau marqueur diagnostique noninvasif. Ann Cardiol Angeiol (Paris) 1993;42:399–405.

    CAS  Google Scholar 

  22. Guiraudon G, Klein GJ, Gulamhusein SS. Total disconnection of the right ventricular free wall: surgical treatment of right ventricular tachycardia associated with right ventricular dysplasia. Circulation 1983;67:463–70.

    PubMed  CAS  Google Scholar 

  23. Haissaguerre M, Chavemac P, Le Metayer P, et al. Valeur complementaire du test a l’isoprénaline et de l’ECG a haute amplification dans le diagnostic de la dysplasie arythmogène du ventricule droit. Ann Cardiol Angeiol (Paris) 1992;41:425–32.

    CAS  Google Scholar 

  24. James TN. Normal and abnormal consequences of apoptosis in the human heart. From postnatal morphogenesis to paroxysmal arrhythmias. Circulation 1994;90: 556–73.

    PubMed  CAS  Google Scholar 

  25. Kinoshita O, Fontaine G, Rosas F, et al. Time- and frequency domain analyses of the signal-averaged ECG in patients with arrhythmogenic right ventricular dysplasia. Circulation 1995;91:715–21.

    PubMed  CAS  Google Scholar 

  26. Leclercq JF, Coumel P. Late potentials in arrhythmogenic right ventricular dysplasia. Prevalence, diagnostic and prognostic values. Eur Heart J 1993;14:Suppl H: 67–73.

    PubMed  Google Scholar 

  27. Lerman BB, Stein KM, Markowitz SM. Idiopathic right ventricular outflow tract tachycardia: a clinical approach. Pace 1996;19:2120–37.

    PubMed  CAS  Google Scholar 

  28. Link MS, Wang PJ, Haugh CJ, et al. Arrhythmogenic right ventricular dysplasia: clinical results with implantable cardioverter-defibrillators. J Intervent Card Electrophysiol 1997;1:41–8.

    Article  CAS  Google Scholar 

  29. Mallat Z, Tedgui A, Fontaliran F, Frank R, Durigon M, Fontaine G. Evidence of apoptosis in arrhythmogenic right ventricular dyspasia. N Engl J Med 1996;335: 1190–6.

    Article  PubMed  CAS  Google Scholar 

  30. Marcus FI, Fontaine G, Frank R, et al. Long-term follow-up in patients with arrhythmogenic right ventricular disease. Eur Heart J 1989;10:Suppl D:68–73.

    PubMed  Google Scholar 

  31. Marcus FI, Fontaine G, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 cases. Circulation 1982;65:384–99.

    PubMed  CAS  Google Scholar 

  32. McKenna WJ, Thiene G, Nava A, et al. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 1994;71:215–8.

    Article  PubMed  CAS  Google Scholar 

  33. Nava A, Thiene G, Canciani B, et al. Familial occurrence of right ventricular dysplasie: a study involving nine families. J Am Coll Cardiol 1988;12:1222–8.

    PubMed  CAS  Google Scholar 

  34. Nimkhedkar K, Hilton CJ, Furniss SS, et al. Surgery for ventricular tachycardia associated with right ventricular dysplasia: disarticulation of right ventricue in 9 of 10 cases. J Am Coll Cardiol 1992;19:1079–84.

    Article  PubMed  CAS  Google Scholar 

  35. Peters S, Reil GH. Risk factors of cardiac arrest in arrhythmogenic right ventricular dysplasia. Eur Heart J 1995;16:77–80.

    PubMed  CAS  Google Scholar 

  36. Pinamonti B, Singara G, Salvi A, et al. Left ventricular involvement in right ventricular dysplasia. Am Heart J 1992;123:711–24.

    Article  PubMed  CAS  Google Scholar 

  37. Rampazzo A, Nava A, Danieli GA, et al. The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23–q24. Hum Mol Genet 1994;3: 959–62.

    Article  PubMed  CAS  Google Scholar 

  38. Rampazzo A, Nava A, Erne P, et al. A new locus for arrhythmogenic right ventricular cardiomyopathy (AR VD2) maps to chromosome 1q42–q43. Hum Mol Genet 1995;4:2151–4.

    Article  PubMed  CAS  Google Scholar 

  39. Rampazzo A, Nava A, Miorin M, et al. A new locus for arrhythmogenic right ventricular cardiomyopathy (AR VD4) maps to chromosome 2q32. Genomics (in press).

  40. Ricci C, Longo R, Pagnan L, et al. Magnetic resonance imaging in right ventricular dysplasia. Am J Cardiol 1992;70:1589–95.

    Article  PubMed  CAS  Google Scholar 

  41. Richardson P, McKenna WJ, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies. Circulation 1996;93:841–2.

    PubMed  CAS  Google Scholar 

  42. Scognamiglio R, Fasoli G, Nava A, Miraglia G, Thiene G, Dalla Volta S. Relevance of subtle echocardiographic findings in early diagnosis of the concealed form of right ventricular dysplasia. Eur Heart J 1989;10:Suppl. D: 27–8.

    PubMed  Google Scholar 

  43. Severini GM, Krajinovic M, Pinamonti, et al. A new locus for arrhythmogenic right ventricular dysplasia on the long arm of chromosome 14. Genomics 1996;31:193–200.

    Article  PubMed  CAS  Google Scholar 

  44. Shoda M, Kasanuki H, Ohnishi S, Umemura J. Recurrence of new ventricular tachycardia after successful catheter ablation in patients with arrhythmogenetic right ventricular dysplasia. Circulation 1992;86:1–580.

    Google Scholar 

  45. Strain JE, Grose RM, Factor SM, Fisher JD. Results of endomyocardial biopsy in patients with spontaneous ventricular tachycardia but without strctural heart disease. Circulation 1983;68:1171–81.

    PubMed  CAS  Google Scholar 

  46. Thiene G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988;318:129–33.

    PubMed  CAS  Google Scholar 

  47. Uhl HS. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1972;91:197–204.

    Google Scholar 

  48. Wichter T, Böcker D, Borggrefe M, Hammel D, Breithardt G, Block M. Cardioverter-defibrillator therapy. In: Nava A, Rossi L, Thiene G, eds. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Amsterdam: Elsevier Science BV, 1997:364–75.

    Google Scholar 

  49. Wichter T, Borggrefe M, Breithardt G. Die arrhythmogene rechtsventrikuläre Erkrankung. Z Kardiol 1991;80:107–25.

    PubMed  CAS  Google Scholar 

  50. Wichter T, Borggrefe M, Haverkamp W, Chen X, Breithardt G. Efficacy of antiarrhythmic drugs in patients with arrhythmogenic right ventricular disease. Results in patients with inducible and noninducible ventricular tachycardia. Circulation 1992;86:29–37.

    PubMed  CAS  Google Scholar 

  51. Wichter T, Breithardt G, Borggrefe M. Ventricular tachycardia in the normal heart. In: Podrid PJ, Kowey PR, eds. Cardiac arrhythmias—mechanisms, diagnosis and management. Baltimore: Williams & Wilkins 1995:1219–38.

    Google Scholar 

  52. Wichter T, Fetsch T. Time-domain-analysis and spectral-turbulence-analysis of the signal-averaged ECG identify high-risk-subgroups in arrhythmogenic right ventricular cardiomyopathy. Circulation 1994;90:Suppl I:1–229.

    Google Scholar 

  53. Wichter T, Haverkamp W, Martinez-Rubio A, Borggrefe M. Long-term prognosis and risk-stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 1995;92:Suppl I:I-97.

    Google Scholar 

  54. Wichter T, Hindricks G, Kottkamp H, Breithardt G Borggrefe M. Catheter ablation of ventricular tachy cardia. In: Nava A, Rossi L, Thiene G, eds. Arrhyth mogenic right ventricular cardiomyopathy/dysplasia Amsterdam: Elsevier Science BV, 1997:376–91.

    Google Scholar 

  55. Wichter T, Hindricks G, Lerch H, et al. Regional myocardial sympathetic dysionnervation in arrhythmogenic right ventricular cardiomyopathy: an analysis using123I-Meta-Iodobenzylguanidine scintigraphy. Circulation 1994;89:667–83.

    PubMed  CAS  Google Scholar 

  56. Wichter T, Lentschig MG, Reimer P, Borggrefe M, Breithardt G. Magnetic resonance imaging. In: Nava A, Rossi L, Thiene G, eds. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Amsterdam: Elsevier Science BV, 1997:269–84.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Medizinische Klinik und Poliklinik, Innere Medizin C (Kardiologie und Angiologie) und Institut für Arterioskleroseforschung, Westfälische Wilhelms-Universität Münster, Albert-Schweitzer-Straße 33, D-48129, Münster

    Thomas Wichter, Martin Borggrefe & Günter Breithardt

Authors
  1. Thomas Wichter
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Martin Borggrefe
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Günter Breithardt
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Thomas Wichter.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wichter, T., Borggrefe, M. & Breithardt, G. Arrhythmogene rechtsventrikuläre Kardiomyopathie. Med Klin 93, 268–277 (1998). https://doi.org/10.1007/BF03044803

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03044803

Schlüsselwörter

Key Words

Search

Navigation