Hopp til hovedinnhold

Arytmogen høyre ventrikkel kardiomyopati

Sist revidert:
Sist revidert av:


Definisjon:
ARVC er en form for kardiomyopati hvor muskelvev i høyre ventrikkel gradvis erstattes av fett- og bindevev. Dette kan føre til arytmier og plutselig hjertedød. Sykdommen er genetisk. 
Forekomst:
Ca. 2-5 tilfeller per 10 000 voksne personer, med stor variasjon mellom ulike land
Symptomer:
Mange er symptomfri. Symptomer kan være hjertebank, hurtig puls, brystsmerter, besvimelse
Funn:
Ved undersøkelse kan det tidlig høres spaltet 2. tone, ellers som regel normale funn
Diagnostikk:
EKG kan vise arytmier eller ledningsfeil. Ved ekko- eller kontrastundersøkelser kan man påvise svekkelse og feilfunksjon av høyre hjertekammer. MR kan vise typisk fettinfiltrasjon
Behandling:
Pasienter som vurderes å ha høy risiko for plutselig hjertedød, behandles med hjertestarter (ICD), ev. med tillegg av antiarytmika
  1. Anderson EL. Arrhythmogenic right ventricular dysplasia. Am Fam Physician 2006; 73: 1391-8. American Family Physician  
  2. Brieler J, Breeden MA, Tucker J. Cardiomyopathy: An Overview. Am Fam Physician. 2017;96(10):640-646. PubMed  
  3. Priori SG, Blomström-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36(41):2793-2867. PubMed  
  4. Corrado D, Link MS, Calkins H. Arrhythmogenic Right Ventricular Cardiomyopathy. N Engl J Med. 2017;376(1):61-72. PubMed  
  5. Firat Duru, Richard N W Hauer, Multiple facets of arrhythmogenic cardiomyopathy: the Fuwai classification of a unique disease based on clinical features, histopathology, and genotype, European Heart Journal, Volume 40, Issue 21, 1 June 2019, Pages 1704–1706. PMID: 31152552 PubMed  
  6. Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation 1982; 65: 384-98. Circulation  
  7. Murray B. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C): a review of molecular and clinical literature. J Genet Couns. 2012;21(4):494–504.
  8. Basso C, Corrado D, Marcus FI, Nava A, Thiene G. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009;373(9671):1289–1300.
  9. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J. 2010;31(7):806–814.
  10. Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 1998; 339: 364. New England Journal of Medicine  
  11. te Riele AS, James CA, Groeneweg JA, et al. Approach to family screening in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Eur Heart J 2016; 37: 755. pmid:26314686 PubMed  
  12. Te Riele AS, James CA, Philips B, et al. Mutation-positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced. J Cardiovasc Electrophysiol 2013; 24: 1311-20. pmid:23889974 PubMed  
  13. Marcus FL, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation 2010; 121: 1533. Circulation  
  14. Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm 2019; 16:e301. PubMed  
  15. McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis. UpToDate, last updated Dec 16, 2019. UpToDate  
  16. Fontaine G, Fontaliran F, Frank R. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation 1998; 97: 1532-5. Circulation  
  17. Corrado D, Wichter T, Link MS, et al. Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement. Eur Heart J. 2015;36(46):3227–3237. PMID: 26216920 PubMed  
  18. Maron BJ, Zipes DP, Kovacs RJ. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Preamble, principles, and general considerations: A scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015; 66: 2343-9. pmid:26542655 PubMed  
  19. Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol 2006; 48: e247. PubMed  
  20. Corrado D, Calkins H, Link MS, et al. Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 2010; 122: 1144. Circulation  
  21. McKenna WJ. Treatment and prognosis of arrhythmogenic right ventricular cardiomyopathy. UpToDate, last updated Aug 03, 2015 . UpToDate  
  22. McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis. UpToDate, last updated Nov 11, 2021. UpToDate  
  23. Groeneweg JA, Bhonsale A, James CA, et al. Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members. Circ Cardiovasc Genet 2015; 8: 437-46. pmid:25820315 PubMed  
  • Ingard Løge, spesialist allmennmedisin, redaktør NEL, universitetslektor, Institutt for samfunnsmedisinske fag, NTNU