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Arytmogen høyre ventrikkel kardiomyopati (ARVC)

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 med autosomal dominant arvegang. 
Forekomst:
Ca. 8 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. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies: Developed by the task force on the management of cardiomyopathies of the European Society of Cardiology (ESC). Eur Heart J 2023. doi:10.1093/eurheartj/ehad194 DOI  
  2. 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  
  3. 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  
  4. Basso C, Corrado D, Marcus FI, Nava A, Thiene G. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009;373(9671):1289–1300.
  5. 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.
  6. 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  
  7. 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  
  8. 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  
  9. Murray B. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C): a review of molecular and clinical literature. J Genet Couns. 2012;21(4):494–504.
  10. 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  
  11. Corrado D, Link MS, Calkins H. Arrhythmogenic Right Ventricular Cardiomyopathy. N Engl J Med. 2017;376(1):61-72. PubMed  
  12. McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis. UpToDate, last updated Dec 16, 2019. UpToDate  
  13. 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  
  14. Fontaine G, Fontaliran F, Frank R. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation 1998; 97: 1532-5. Circulation  
  15. 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  
  16. 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  
  17. 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  
  18. 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  
  19. McKenna WJ. Treatment and prognosis of arrhythmogenic right ventricular cardiomyopathy. UpToDate, last updated Aug 03, 2015 . UpToDate  
  20. McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis. UpToDate, last updated Nov 11, 2021. UpToDate  
  21. 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