Advertisement

Risk Stratification and Antiarrhythmic Drug Therapy

  • Thomas Wichter
  • Domenico Corrado
  • Matthias Paul

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an important cause of ventricular tachyarrhythmias and sudden death in young patients and athletes with apparently normal hearts. The disease is characterized by localized or diffuse atrophy of predominantly right ventricular myocardium with subsequent replacement by fatty and fibrous tissue. As a result of these pathomorphological alterations, global and regional right (and left) ventricular dysfunction and ventricular tachyarrhythmias due to areas of slow conduction and dispersion of refractoriness are the major clinical findings and manifestations of ARVC/D [1, 2, 3, 4].

Keywords

Sudden Death Ventricular Tachycardia Ventricular Arrhythmia Catheter Ablation Antiarrhythmic Drug 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Marcus FI, Fontaine GH, Guiraudon G et al (1982) Right ventricular dysplasia: A report of 24 adult cases. Circulation 65:384–398PubMedGoogle Scholar
  2. 2.
    Thiene G, Nava A, Corrado D et al (1988) Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318:129–133PubMedCrossRefGoogle Scholar
  3. 3.
    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
  4. 4.
    Fontaine G, Fontaliran F, Frank R (1998) Arrhythmogenic right ventricular cardiomyopathies: Clinical forms and main differential diagnoses. Circulation 97:1532–1535PubMedGoogle Scholar
  5. 5.
    Nava A, Bauce B, Basso C et al (2000) Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol 36:2226–2233PubMedCrossRefGoogle Scholar
  6. 6.
    McKenna WJ, Thiene G, Nava A et al (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 71:215–218PubMedCrossRefGoogle Scholar
  7. 7.
    Marcus FI, Towbin JA, Zareba W et al (2003) Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C): A multidisciplinary study-Design and protocol. Circulation 107:2975–2978PubMedCrossRefGoogle Scholar
  8. 8.
    Basso C, Wichter T, Danieli GA et al (2004) Arrhythmogenic right ventricular cardiomyopathy: Clinical registry and database, evaluation of therapies, pathology registry, DNA banking. Eur Heart J 25:531–534PubMedCrossRefGoogle Scholar
  9. 9.
    Wichter T, Hindricks G, Lerch H et al (1994). Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy: An analysis using 123I-Meta-Iodobenzylguanidine scintigraphy. Circulation 89:667–683PubMedGoogle Scholar
  10. 10.
    Wichter T, Schäfers M, Borggrefe M et al (2000) Abnormalities of cardiac sympathetic innervation in arrhythmogenic right ventricular cardiomyopathy: Quantitative assessment of presynaptic norepinephrine re-uptake and postsynaptic β-adrenoceptor density using positron emission tomography. Circulation 101:1552–1558PubMedGoogle Scholar
  11. 11.
    Daliento L, Turrini P, Nava A et al (1995) Arrhythmogenic right ventricular cardiomyopathy in young versus adult patients: Similarities and differences. J Am Coll Cardiol 25:655–664PubMedCrossRefGoogle Scholar
  12. 12.
    Corrado D, Basso C, Pavei A et al (2006) Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA 296:1593–1601PubMedCrossRefGoogle Scholar
  13. 13.
    Gerull B, Heuser A, Wichter T et al (2004) Mutations in the desmosomal arm repeat protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet 36:1162–1164PubMedCrossRefGoogle Scholar
  14. 14.
    Garcia-Gras E, Lombardi R, Giocondo MJ et al (2006) Suppression of canonical Wnt/b-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. J Clin Invest 116:2012–2021PubMedCrossRefGoogle Scholar
  15. 15.
    Kirchhof P, Fabritz L, Zwiener M et al (2006) Age-and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice.Circulation 114:1799–1806PubMedCrossRefGoogle Scholar
  16. 16.
    Wichter T, Paul M, Breithardt G (2005) Arrhythmogene rechtsventrikuläre Kardiomyopathie: Sportmedizinische Aspekte. Dtsch Z Sportmed 56:118–125Google Scholar
  17. 17.
    Corrado D, Basso C, Thiene G (1996) Pathological findings in victims of sport-related sudden cardiac death. Sports Exerc Injury 2:78–86Google Scholar
  18. 18.
    Blomström-Lundqvist C, Sabel KG, Olsson SB (1987) A long-term follow up of 15 patients with arrhythmogenic right ventricular dysplasia. Br Heart J 58:477–488PubMedCrossRefGoogle Scholar
  19. 19.
    Canu G, Atallah G, Claudel JP et al (1993) Prognostic et évolution à long terme de la dysplasie arythmogène du ventricule droit. Arch Mal Coeur 86:41–48PubMedGoogle Scholar
  20. 20.
    Leclercq JF, Coumel P, Denjoy I et al (1991) Long-termfollow-up after sustained monomorphic ventricular tachycardia: Causes, pump failure, and empiric antiarrhythmic therapy that modify survival. Am Heart J 121:1685–1692PubMedCrossRefGoogle Scholar
  21. 21.
    Marcus FI, Fontaine GH, Frank R et al (1989) Longterm follow-up in patients with arrhythmogenic right ventricular disease. Eur Heart J 10:68–73PubMedGoogle Scholar
  22. 22.
    Turrini P, Corrado D, Basso C et al (2003) Noninvasive risk stratification in arrhythmogenic right ventricular cardiomyopathy. Ann Noninvasive Electrocardiol 8:161–169PubMedCrossRefGoogle Scholar
  23. 23.
    Dalal D, Nasir K, Bomma C et al (2005) Arrhythmogenic right ventricular dysplasia: A United States experience. Circulation 112:3823–3832PubMedCrossRefGoogle Scholar
  24. 24.
    Hulot S, Jouven X, Empana JP et al (2004) Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 110:1879–1884PubMedCrossRefGoogle Scholar
  25. 25.
    Wichter T, Paul M, Eckardt L et al (2005) Arrhythmogenic right ventricular cardiomyopathy: Antiarrhythmic drugs, catheter ablation, or ICD. Herz-Cardiovasc Dis 30:91–101Google Scholar
  26. 26.
    Peters S, Peters H, Thierfelder L (1999) Risk stratification of sudden cardiac death and malignant ventricular arrhythmias in right ventricular dysplasia-cardiomyopathy. Int J Cardiol 71:243–250PubMedCrossRefGoogle Scholar
  27. 27.
    Lemola K, Brunckhorst C, Helfenstein U et al (2005) Predictors of adverse outcome in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy: Long term experience of a tertiary care centre. Heart 91:1167–1172PubMedCrossRefGoogle Scholar
  28. 28.
    Kiès P, Bootsma M, Bax J et al (2006) Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Screening, diagnosis, and treatment. Heart Rhythm 3:225–234PubMedCrossRefGoogle Scholar
  29. 29.
    Turrini P, Corrado D, Basso C et al (2001) Dispersion of ventricular depolarization-repolarization. A noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy. Circulation 103:3075–3080PubMedGoogle Scholar
  30. 30.
    Corrado D, Leoni L, Link MS et al (2003) Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 108:3084–3091PubMedCrossRefGoogle Scholar
  31. 31.
    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 109:1503–1508PubMedCrossRefGoogle Scholar
  32. 32.
    Piccini JP, Dalal D, Roguin A et al (2005) Predictors of appropriate implantable defibrillator therapies in patients with arrhythmogenic right ventricular dysplasia. Heart Rhythm 2:1188–1194PubMedCrossRefGoogle Scholar
  33. 33.
    Pezawas T, Stix G, Kastner J et al (2006) Ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Clinical presentation, risk stratification and results of long-term follow-up. Int J Cardiol 107:360–368PubMedCrossRefGoogle Scholar
  34. 34.
    Fontaine G, Zenati O, Tonet J et al (1997) The treatment of ventricular arrhythmias. In: Nava A, Rossi L, Thiene G (eds) Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Elsevier Science, Amsterdam, BV:315–363Google Scholar
  35. 35.
    Tonet J, Frank R, Fontaine G et al (1989) Efficacité de l’association de faibles doses de bêta-bloquants ”a l’amiodarone dans le traitment des tachycardies ventriculaires réfractaires. Arch Mal Coeur 82:1511–1517PubMedGoogle Scholar
  36. 36.
    Berder V, Vauthier M, Mabo P et al (1995) Characteristics and outcome in arrhythmogenic right ventricular dysplasia. Am J Cardiol 75:411–415PubMedCrossRefGoogle Scholar
  37. 37.
    Wichter T, Borggrefe M, Haverkamp W et al (1992) Efficacy of antiarrhythmic drugs in patients with arrhythmogenic right ventricular disease. Results in patients with inducible and noninducible ventricular tachycardia. Circulation 86:29–37PubMedGoogle Scholar
  38. 38.
    Hamid MS, Norman M, Quraishi A et al (2002). Prospective evaluation of relatives for familial arrhythmogenic right ventricular cardiodmyopathy/dysplasia reveals a need to broaden diagnostic criteria. J Am Coll Cardiol 40:1445–1450PubMedCrossRefGoogle Scholar
  39. 39.
    Wichter T, Breithardt G (2005) Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: A role for genotyping in decision-making? J Am Coll Cardiol 45:409–411PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2007

Authors and Affiliations

  • Thomas Wichter
    • 1
  • Domenico Corrado
    • 2
  • Matthias Paul
    • 1
  1. 1.Department of Cardiology and AngiologyUniversity Hospital of MünsterMünsterGermany
  2. 2.Department of Cardiological, Thoracic and Vascular SciencesUniversity of PaduaItaly

Personalised recommendations