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European Heart Journal Advance Access published online on June 14, 2006
European Heart Journal, doi:10.1093/eurheartj/ehl095
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European Heart Journal © The European Society of Cardiology 2006; all rights reserved
Received February 20, 2006
Revised May 11, 2006
Accepted May 26, 2006

Preclinical research

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

Cristina Basso 1, Elzbieta Czarnowska 2, Mila Della Barbera 1, Barbara Bauce 3, Giorgia Beffagna 4, Elzbieta K Wlodarska 5, Kalliopi Pilichou 4, Angelo Ramondo 3, Alessandra Lorenzon 4, Olgierd Wozniek 5, Domenico Corrado 3, Luciano Daliento 3, Gian Antonio Danieli 4, Marialuisa Valente 1, Andrea Nava 3, Gaetano Thiene 1 *, and Alessandra Rampazzo 4

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

* To whom correspondence should be addressed.
Gaetano Thiene, E-mail: cardpath{at}unipd.it


  Abstract

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, desmoplekin in three and plekophilin-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.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Intercalated disc; Myocyte; Pathology; Transmission electron microscopy; Ultrastructure.
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