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Contributors
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- By Yasir Abu-Omar, Matthew E. Atkins, Joseph E. Arrowsmith, Alan Ashworth, Rubia Baldassarri, Craig R. Bailey, David J. Barron, Christiana C. Burt, David Cardone, Coralie Carle, Jose Coddens, Alan M. Cohen, Simon Colah, Sarah Conolly, David J. Daly, Helen M. Daly, Stefan G. De Hert, Ravi J. De Silva, Mark Dougherty, John J. Dunning, Maros Elsik, Betsy Evans, Florian Falter, Nigel Farnum, Jens Fassl, Juliet E. Foweraker, Simon P. Fynn, Andrew I. Gardner, Margaret I. Gillham, Martin J. Goddard, Maximilien J. Gourdin, Jon Graham, Stephen J. Gray, Cameron Graydon, Fabio Guarracino, Roger M. O. Hall, Michael Haney, Charles W. Hogue, Ben W. Howes, Bevan Hughes, Siân I. Jaggar, David P. Jenkins, Jörn Karhausen, Todd Kiefer, Khalid Khan, Andrew A. Klein, John D. Kneeshaw, Andrew C. Knowles, Catherine V. Koffel, R. Clive Landis, Trevor W. R. Lee, Clive J. Lewis, Jonathan H. Mackay, Amod Manocha, Jonathan B. Mark, Sarah Marstin, William T. McBride, Kenneth H. McKinlay, Alan F. Merry, Berend Mets, Britta Millhoff, Kevin P. Morris, Samer A. M. Nashef, Andrew Neitzel, Stephane Noble, Rabi Panigrahi, Barbora Parizkova, J. M. Tom Pierce, Mihai V. Podgoreanu, Hans-Joachim Priebe, Paul Quinton, C. Ramaswamy Rajamohan, Doris M. Rassl, Tom Rawlings, Fiona E. Reynolds, Andrew J. Richardson, David Riddington, Andrew Roscoe, Paul H. M. Sadleir, Ving Yuen See Tho, Herve Schlotterbeck, Maura Screaton, Shitalkumar Shah, Harjot Singh, Jon H. Smith, M. L. Srikanth, Yeewei W. Teo, Kamen P. Valchanov, Jean-Pierre van Besouw, Isabeau A. Walker, Stephen T. Webb, Francis C. Wells, John Whitbread, Charles Willmott, Patrick Wouters
- Edited by Jonathan H. Mackay, Joseph E. Arrowsmith
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- Book:
- Core Topics in Cardiac Anesthesia
- Published online:
- 05 April 2012
- Print publication:
- 15 March 2012, pp x-xiii
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5 - The role of the pathologist in the diagnosis of cardiomyopathy: a personal view
- Nigel Kirkham, Neil A. Shepherd
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- Book:
- Progress in Pathology
- Published online:
- 06 January 2010
- Print publication:
- 12 April 2007, pp 101-134
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Summary
INTRODUCTION
Primary cardiomyopathies are defined as diseases intrinsic to the myocardium associated with cardiac dysfunction leading to congestive heart failure, arrhythmia and sudden cardiac death. The primary cardiomyopathies are classified by their haemodynamic and morphological characteristics and include dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and unclassified cardiomyopathy [1], [2]. A familial cause has been shown in patients with DCM, HCM and ARVC. Advances in molecular genetics have led to the identification of single gene defects and candidate disease loci responsible for these cardiomyopathies as well as previously unclassified cardiomyopathies of unknown cause, such as isolated left ventricular non-compaction (LVNC). These advances, coupled with phenotype–genotype correlation analyses, have shown that the pathology of several types of cardiomyopathy encompasses a much broader morphological spectrum than previously anticipated. This chapter will focus on recent advances in our understanding of the pathology of the inherited cardiomyopathies and the role of the pathologist in the diagnosis of cardiomyopathy at autopsy and in endomyocardial biopsy specimens.
DILATED CARDIOMYOPATHY
Idiopathic DCM is the most common cause of congestive heart failure worldwide with an estimated prevalence in a general population of 36.5 cases per 100 000 [3]. It is characterised by progressive heart failure due to impaired contraction of the left or both ventricles accompanied by ventricular dilatation. Although the aetiology of idiopathic DCM is largely unknown [4], up to 35% of patients have familial disease [5].
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