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European Heart Journal Advance Access originally published online on August 14, 2009
European Heart Journal 2009 30(20):2430; doi:10.1093/eurheartj/ehp312
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2009. For permissions please email: journals.permissions@oxfordjournals.org
The online version of this article has been published under an open access model. Users are entitled to use, reproduce, disseminate, or display the open access version of this article for non-commercial purposes provided that the original authorship is properly and fully attributed; the Journal, Learned Society and Oxford University Press are attributed as the original place of publication with correct citation details given; if an article is subsequently reproduced or disseminated not in its entirety but only in part or as a derivative work this must be clearly indicated. For commercial re-use, please contact journals.permissions@oxfordjournals.org

Acute episode of an arrhythmogenic right ventricular cardiomyopathy with vast necroses exclusively in right ventricular myocardium

Stefan Gattenlöhner1,*, Philipp Demmer1, Martin Oberhoff2 and Georg Ertl3

1 Institute of Pathology, University of Würzburg, Josef-Schneiderstr.2, D-97080 Würzburg, Germany
2 Department of Internal Medicine II, St-Elisabeth Hospital, Bad Kissingen, Germany
3 Department of Internal Medicine I, University of Würzburg, Würzburg, Germany

* Corresponding author. Tel: +49 931 201 47420, Fax: +49 931 201 47440, Email: stefan.gattenloehner{at}mail.uni-wuerzburg.de

We describe the case of an athlete aged 18 years who died of sudden cardiac arrest without previously having been diagnosed with heart disease. The autopsy did not reveal signs of intoxication or endo-/myocarditis, including negative results for cardiotropic virus, and coronary heart disease/vasculitis was excluded by coronary angiography and histology.

In contrast, cardiac morphology showed classical signs of arrhythmogenic right ventricular cardiomyopathy (ARVC) with diffuse replacement of RV myocardium by fibro-fatty tissue (Panel A), detection of abnormal long desmosomes (Panel A, inset) and immunohistochemical lack of plakoglobin1 (Panel B) but strong expression of N-cadherin (Panel B, inset). More interestingly and exclusively in RV myocardium but not in left ventricle nor both atrias, large areas of acute/subacute cardiomyocyte necroses were detected (Panels C and D) with mild inflammatory infiltrates (Panel C), myocytolysis with loss of myofibrils2 (Panel C, inset) and myocardial contraction bands (Panel D) with hypercontracted sarcomeres (Panel D, inset)

Although death of single myocytes has been reported in ARVC,3 the vast necroses in RV myocardium shown here with an increase of the MB isoform of creatine kinase (CK-MB 85U/l; CK 530 U/l) detected in a blood sample collected immediately after start of reanimation have so far not been described and might be the morphological correlate of an acute episode of ARVC. In our opinion, these data confirm the ‘degenerative hypothesis’4 suggesting that the replacement of the RV myocardium is progressive with time starting from the epicardium and expanding transmurally to the endocardium3 and underline the relevance of markers as plakoglobin in preventing such fatal courses of ARVC.


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Funding to pay open access publication charges for this article was provided by the Wilhelm-Sander-Stiftung (grant 2007.068.01) and the Deutsche Forschungs-Gesellschaft (DFG-SFB-Transregio 52, TPA8).

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  1. Asimaki A, Tandri H, Huang H, Halushka MK, Gautam S, Basso C, Thiene G, Tsatsopoulou A, Protonotarios N, McKenna WJ, Calkins H, Saffitz JE. A new diagnostic test for arrhythmogenic right ventricular cardiomyopathy. N Engl J Med (2009) 360:1075–1084.[Abstract/Free Full Text]
  2. Turillazzi E, Baroldi G, Silver MD, Parolini M, Pomara C, Fineschi V. A systematic study of a myocardial lesion: colliquative myocytolysis. Int J Cardiol (2005) 104:152–157.[CrossRef][Web of Science][Medline]
  3. Thiene G, Corrado D, Basso C. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Orphanet J Rare Dis (2007) 2:45.[CrossRef][Medline]
  4. Thiene G, Corrado D, Nava A, Rossi L, Poletti A, Boffa GM, Daliento L, Pennelli N. Right ventricular cardiomyopathy: is there evidence of an inflammatory aetiology? Eur Heart J (1991) 12(Suppl. D):22–25.[Free Full Text]

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This Article
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