Skip to main content
Log in

Arrhythmien aus dem rechten Ventrikel

Right ventricular arrhythmias

  • Schwerpunkt: Der kranke rechte Ventrikel
  • Published:
Der Internist Aims and scope Submit manuscript

Zusammenfassung

Arrhythmien aus dem rechten Ventrikel treten bevorzugt bei jungen Patienten mit seltenen kardialen Erkrankungen auf. Wesentliche Grunderkrankungen sind die idiopathische rechtsventrikuläre Ausflusstrakt-Tachykardie (RVOT-VT), die arrhythmogene rechtsventrikuläre Kardiomyopathie (ARVC), das Brugada-Syndrom und angeborene Herzfehler (v. a. Fallot-Tetralogie). In Abhängigkeit von der zugrunde liegenden Erkrankung bestehen erhebliche Unterschiede in der Diagnostik, Therapie und Prognose, welche wesentlich durch den plötzlichen Herztod bestimmt wird. Für die optimale Versorgung der Patienten ist eine detaillierte Diagnostik und Risikostratifikation erforderlich. Die individuelle Therapiestrategie richtet sich nach der Grunderkrankung, Arrhythmiecharakteristik und dem Risiko des plötzlichen Herztodes und beinhaltet die antiarrhythmische Pharmakotherapie, die Katheterablation und die Implantation eines Cardioverter-Defibrillators.

Diese Übersicht fasst das aktuelle Wissen über die Pathogenese, Diagnostik, Therapie und Prognose der wesentlichen Erkrankungen zusammen, die mit Arrhythmien aus dem rechten Ventrikel einhergehen.

Abstract

Right ventricular arrhythmias predominantly occur in young patients with rare cardiac diseases. Underlying cardiac conditions include idiopathic right ventricular outflow-tract tachycardia (RVOT-VT), arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome, and postoperative congenital heart disease (i.e. tetralogy of Fallot). According to the underlying cardiac disease, there are significant differences in the diagnostic and therapeutic management and prognosis which is mainly determined by life-threatening ventricular arrhythmia recurrences and sudden cardiac death. To provide optimal treatment for affected patients, a detailed diagnostic evaluation and risk stratification is mandatory. Tailored treatment strategies aim at the suppression or effective termination of recurrent ventricular tachyarrhythmias and prevention of sudden death by antiarrhythmic drug therapy, catheter ablation, and the implantation of cardioverter-defibrillators.

This review summarizes the current knowledge on pathogenesis, diagnosis, treatment and prognosis of those conditions that are associated with arrhythmias originating from the 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.

Abb. 1a, b
Abb. 2
Abb. 3a–c
Abb. 4
Abb. 5
Abb. 6
Abb. 7

Literatur

  1. Marcus FI, Fontaine G, Guiraudon G et al. (1982) Right ventricular dysplasia: a report of 24 cases. Circulation 65: 384–399

    CAS  PubMed  Google Scholar 

  2. Wichter T, Borggrefe M, Breithardt G (1998) Die arrhythmogene rechtsventrikuläre Kardiomyopathie: Ätiologie, Diagnostik und Therapie. Med Klin 93: 268–277

    CAS  PubMed  Google Scholar 

  3. Corrado D, Basso C, Thiene G (2000) Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart 83: 588–595

    Article  CAS  PubMed  Google Scholar 

  4. Paul M, Schulze-Bahr E, Breithardt G, Wichter T (2003) Genetics of arrhythmogenic right ventricular cardiomyopathy—status quo and future perspectives. Z Kardiol 92: 128–136

    Article  CAS  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

    CAS  PubMed  Google Scholar 

  6. Wichter T, Schulze-Bahr E, Eckardt L et al. (2002) Molecular mechanisms of inherited ventricular arrhythmias. Herz 27: 712–739

    Article  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  8. Wichter T, Borggrefe M, Block M, Breithardt G (1998) Differentialtherapie der arrhythmogenen rechtsventrikulären Kardiomyopathie. Herzschr Elektrophys 9: 169–182

    Article  Google Scholar 

  9. Turrini P, Corrado D, Basso C, Nava A, Thiene G (2003) Noninvasive risk stratification in arrhythmogenic right ventricular cardiomyopathy. Ann Noninvasive Electrocardiol 8: 161–169

    PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  11. O’Donnell D, Cox D, Bourke J, Mitchell, Furniss S (2003) Clinical and electrophysiological differences between patients with arrhythmogenic right ventricular dysplasia and right ventricular outflow tract tachycardia. Eur Heart J 24: 801–810

    Article  PubMed  Google Scholar 

  12. Corrado D, Leoni L, Link MS et al. (2003) Implantable cardioverter-defibrillator therapy for the prevention o sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 108: 3084–3091

    Article  PubMed  Google Scholar 

  13. Wichter T, Paul M, Wollmann C et al. (2004) Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: single-center experience of long-term follow-up and complications in 60 patients. Circulation 109: 1503–1508

    Google Scholar 

  14. Corrado D, Fontaine G, Marcus FI et al. (2000) Arrhythmogenic right venricular dysplasia/cardiomyopathy: need for an international registry. Circulation 101: e101–e106

    CAS  PubMed  Google Scholar 

  15. Marcus FI, Towbin JA, Zareba W et al. (2003) Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C): A multidisciplinary study—design and protocol. Circulation 107: 2975–2978

    Article  PubMed  Google Scholar 

  16. Lerman BB, Stein KM, Markowitz SM, Mittal S, Slotwiner DJ (2002) Right ventricular outflow tract tachycardia: an update. Card Electrophysiol Rev 6: 68–71

    Article  PubMed  Google Scholar 

  17. Marcus FI (1997) Is arrhythmogenic right ventricular dysplasia, Uhl’s anomaly, and right ventricular outflow tract tachycardia a spectrum of the same disease? Cardiol Rev 5: 25–29

    Google Scholar 

  18. Cole CR, Marrouche NF, Natale A (2002) Evaluation and management of ventricular outflow tract tachycardias. Card Electrophysiol Rev 6: 442–447

    Article  PubMed  Google Scholar 

  19. Brugada P, Brugada J (1992) Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome: a multicenter report. J Am Coll Cardiol 20: 1391–1396

    CAS  PubMed  Google Scholar 

  20. Wilde AAM, Antzelevitch C, Borggrefe M et al. (2002) Proposed diagnostic criteria for the Brugada syndrome. Circulation 106: 2514–2519

    Article  PubMed  Google Scholar 

  21. Antzelevitch C (2001) The Brugada syndrome: ionic and arrhythmia mechanisms. J Cardiovasc Electrophysiol 12: 268–272

    Article  CAS  PubMed  Google Scholar 

  22. Brugada J, Brugada R, Brugada P (2003) Determinants of sudden death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation 108: 3092–3096

    Article  PubMed  Google Scholar 

  23. Priori SG, Napolitano C, Gasparini M et al. (2002) Natural history of Brugada syndrome: insights for risk stratification and management. Circulation 105: 1342–1347

    Article  PubMed  Google Scholar 

  24. Brugada J, Brugada R, Antzelevitch C et al. (2002) Long-term follow-up of individuals with the electrocardiographic pattern of right bundle branch block and ST-segment elevation in precordial leads V1 to V3. Circulation 105: 73–78

    Article  PubMed  Google Scholar 

  25. Gatzoulis MA, Balaji S, Webber SA et al. (2000) Risk factors for arrhythmia and sudden death late after repair of tetralogy of Fallot: a multicentre study. Lancet 356: 975–981

    Article  CAS  PubMed  Google Scholar 

  26. Saul JP, Alexander ME (1999) Preventing sudden death after repair of tetralogy of Fallot: complex therapy for complex patients. J Cardiovasc Electrophysiol 10: 1271–1287

    CAS  PubMed  Google Scholar 

  27. Khairy P, Landzberg MJ, Gatzoulis MA et al. (2004) Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study. Circulation 109: 1994–2000

    Google Scholar 

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

    Google Scholar 

Download references

Danksagung

Die Arbeit wurde erstellt mit Unterstützung durch die Deutsche Forschungsgemeinschaft (DFG), Bonn, Sonderforschungsbereich 556 (Projekt C4) und die Europäische Kommission, Brüssel, Belgien, 5th Framework Program 1998–2002 (QLG1-CT-2000–01091).

Interessenkonflikt:

Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to T. Wichter.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wichter, T., Paul, M., Eckardt, L. et al. Arrhythmien aus dem rechten Ventrikel. Internist 45, 1125–1135 (2004). https://doi.org/10.1007/s00108-004-1279-3

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-004-1279-3

Schlüsselwörter

Keywords

Navigation