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Arrhythmogenic RV Cardiomyopathy/Dysplasia: Recent Advances by Gaetano Thiene MD, Andrea Nava MD (auth.), Frank I. Markus,

By Gaetano Thiene MD, Andrea Nava MD (auth.), Frank I. Markus, Andrea Nava, Gaetano Thiene (eds.)

This e-book covers all features (biological, pathological, genetic, medical and therapeutical) of arrhythmogenic correct ventricular cardiomyopathy/dysplasia, a up to date cardiomyopathy which represents a really excessive threat of surprising dying within the younger and in athletes. The monograph provides the result of 5-year learn software on ARVC/D supported by way of can provide of either the ecu fee and the NIH, which enabled the invention of 7 disease-causing genes, therefore establishing new avenues for the early id of affected sufferers and for unexpected dying prevention.

A panel of most sensible scientists, either eu and americans, contributed to this quantity, as a way to be a vital reference for execs and citizens in cardiology, radiology, human genetics, and game medicine.

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Nava A, Canciani B, Daliento L et al (1988) Juvenile sudden death and effort ventricular tachycardias in a 27 family with right ventricular cardiomyopathy. Int J Cardiol 21:111-126 13. Bauce B, Nava A, Rampazzo A et al (2000) Familial effort polymorphic ventricular arrhythmias in apparently normal heart map to chromosome 1q42-q43. Am J Cardiol 85:573-579 14. Bauce B, Rampazzo A, Basso C et al (2002) Screening for RyR2 mutations in families with effort-induced polymorphic ventricular arrhythmias and sudden death: Early diagnosis of asymptomatic carrier.

ARVC/D At present, considerable importance is given to the finding of fatty infiltration of the myocardium, since cardiac magnetic resonance imaging has the ability to identify adipose tissue in vivo with consequent diagnostic and therapeutic implications. It is still a matter of debate whether fatty infiltration of the right ventricle per se should be considered a morphologic hallmark of ARVC/D [19]. , fatty and fibro-fatty, has been a source of confusion, since it has not been sufficiently appreciated that even in the fatty variant a certain amount of replacementtype fibrosis and myocyte abnormalities should be found to label it as ARVC/D [3].

B) At histology, the almost transmural fatty tissue replacement reaches the endocardium; spots of replacement-type fibrosis are also visible (fatty variant) (Heidenhain trichrome). (c) At higher magnification, myocytolysis and fibro-fatty replacement are visible (haematoxylin-eosin) CHAPTER 4 • Autopsy and Endomyocardial Biopsy Findings a c b d 33 Fig. 5 • Histologic features of ARVC/D. (a) Contraction band myocyte necrosis and mononuclear inflammatory infiltrates (haematoxylin-eosin). (b) Myocytolisis with early adipocytes and fibroblasts infiltration (haematoxylin-eosin).

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