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European Heart Journal Advance Access originally published online on June 14, 2006
European Heart Journal 2006 27(15):1847-1854; doi:10.1093/eurheartj/ehl095
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© The European Society of Cardiology 2006. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: an electron microscopy investigation on endomyocardial biopsies

Cristina Basso1, Elzbieta Czarnowska2, Mila Della Barbera1, Barbara Bauce3, Giorgia Beffagna4, Elzbieta K. Wlodarska5, Kalliopi Pilichou4, Angelo Ramondo3, Alessandra Lorenzon4, Olgierd Wozniek5, Domenico Corrado3, Luciano Daliento3, Gian Antonio Danieli4, Marialuisa Valente1, Andrea Nava3, Gaetano Thiene1,* and Alessandra Rampazzo4

1 Institute of Pathological Anatomy University of Padua, Italy
2 Pathology Children's Memorial Health Institute, Warsaw, Poland
3 Department of Cardiothoracic-Vascular Sciences, University of Padua, Italy
4 Department of Biology, University of Padua, Italy
5 Institute of Cardiology, Warsaw, Poland

Received 20 February 2006; revised 11 May 2006; accepted 26 May 2006; online publish-ahead-of-print 16 June 2006.

* Corresponding author: Institute of Pathological Anatomy University of Padua Medical School Via A. Gabelli, 61 35121 Padua, Italy. Tel: +39 049 8272283; fax: +39 049 8272284. E-mail address: cardpath{at}unipd.it

Aims The ultrastructural features of the myocardium in arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been systematically investigated so far. The recent discovery of gene mutations encoding intercalated disc proteins prompted us to perform a transmission electron microscopy study on endomyocardial biopsies.

Methods and results Twenty-one ARVC probands who fulfilled the international Task Force diagnostic criteria underwent right ventricular endomyocardial biopsy and screening of desmosome (D) protein encoding genes. Myocyte intercalated discs were analysed by transmission electron microscope and the data were compared with those of 10 controls and 10 patients with idiopathic dilated cardiomyopathy.

Extensive fibro-fatty replacement with a residual myocardium of 59±23% was found in ARVC biopsy samples. Pathogenic D gene mutations were identified in 10 (48%): desmoglein-2 in four, desmoplakin in three and plakophilin-2 in three. Mean D length and D percent length of intercalated disc were significantly higher, D number was significantly lower and D gap was widened in ARVC. Moreover, abnormally located D in 75%, abnormal small junctions in 52%, and pale internal plaques in 32% of ARVC patients were found in the presence of a normal intercalated disc convolution index.

Conclusion The ultrastructural evidence of intercalated discs remodelling in ARVC, together with the positive screening of D protein encoding genes in half of probands, are in keeping with an intercellular junction cardiomyopathy.

Key Words: Arrhythmogenic right ventricular cardiomyopathy • Intercalated disc • Myocyte • Pathology • Transmission electron microscopy • Ultrastructure


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