Cardiomyopathies: Realisations and Expectations

314 Pages·1993·16.464 MB·English

by  E. G. J. Olsen, J. F. Goodwin (auth.), J. F. Goodwin, E. G. J. Olsen (eds.)

Cardiomyopathies
Realisations and Expectations
Edited by
1. F. Goodwin and E. G. J. Olsen
With 77 Figures, Partly in Colour
Springer-Verlag
Berlin Heidelberg New York
London Paris Tokyo
Hong Kong Barcelona
Budapest
JOHN F. GOODWIN, MD, FRCP, F ACP (Hon), F ACC, FESC, Dr. he.
U Lisbon
Emeritus Professor of Clinical Cardiology
Royal Postgraduate Medical School, Du Cane Road,
London W12 ONN, UK
ECKHARDT G. J. OLSEN, MD, FRCPath, F ACC, FESC
Consultant Histopathologist (Cardiovascular Division)
Royal Brompton National Heart and Lung Hospital London
Department of Histopathology
Fulham Road, London SW3 6HP, UK
ISBN-13:978-3-642-77600-7 e-ISBN-13:978-3-642-77598-7
DOl: 10.1007/978-3-642-77598-7
Library of Congress Cataloging·in-Publication Data. Cardiomyopathies / J. Goodwin, E. Olsen
(eds.). p. cm. Includes bibliographical references and index. ISBN 3-540-55608-7 (alk.
paper). - ISBN 0-387-55608-7 (alk. paper) 1. Myocardium - Diseases. I. Goodwin, John
F. II. Olsen, E. G. J. (Eckhardt G. J.) [DNLM: 1. Cardiomyopathy, Congestive. 2. Car
diomyopathy, Hypertrophic. 3. Cardiomyopathy, Restrictive. WG 280 C2667] RC685.M9C355
1993616. 1'24 - dc20 DNLM/DLC for Library of Congress 92-49589
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Dedicated
to the memory of
ALAN STENHOUSE GOURLEY
1909-1991
Preface
This book reviews the basic knowledge about the cardiomyopathies
and re-enforces the well-known definitions and classification of
cardiomyopathies and specific heart muscle diseases, respectively. It
emphasizes the importance of maintaining the classification into
hypertrophic, dilated and restrictive cardiomyopathies, even though
there are exceptions.
The term "realisations" in the title is used in the sense of
"achievements" and alludes to the progress in the understanding of
heart muscle diseases over the last three decades and also to the areas
of knowledge still not fully explored, for example, the as yet un
classified putative cardiomyopathies such as arrhythmogenic right
ventricular dysplasia, long QT syndrome and syndrome X. The
accurate and sensitive identification of major risk factors for sudden
death in hypertrophic cardiomyopathy needs further study, as do the
implications of a familial basis in some patients with dilated cardio
myopathy.
The "expectations" mentioned in the title centre around the
molecular biological aspects of viral myocarditis and dilated cardio
myopathy and their relationship to each other; around the auto
immune basis for dilated cardiomyopathy and its implications for
drug therapy and cardiac transplantation; and around molecular
genetic techniques for identifying the genes involved in hypertrophic
cardiomyopathy that will be developed further. These will have
implications for pre-natal recognition and for diagnosis in early life
in apparently fit and active persons, and offer better prospects of
prevention and cure.
The prospects for realising these expectations are bright, and
many of the outstanding problems should be resolved in the near
future. The availability of refined clinical investigation techniques,
such as echocardiography and Doppler methods, and the develop
ment of molecular biological testing have provided vital additions to
the tools for clinical assessment. Thus the prospects of unravelling
the mysteries of the cardiomyopathies are challenging and exciting.
VIII Preface
Finally, it is imperative to maintain the crucial clinical skills and
approach to guide, advise and treat patients: this book therefore
emphasises the clinician's vital role in managing these life-threatening
conditions.
London,August 1992 J. F. GOODWIN
E. G. J. OLSEN
Contents
Part I. Introduction
Chapter 1
Definition, Classification and Terminology. E. G. J. OLSEN and
J. F. GOODWIN ..... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Part II. Dilated Cardiomyopathy
Chapter 2
Clinical Aspects. J. F. GOODWIN. (With 2 Figures) . . . . . . . . . . 9
Chapter 3
Pathology. E. G. J. OLSEN and S. E. TROTTER.............. 19
Chapter 4
Clinical Investigation: New Approaches. P. J. RICHARDSON
and H. J. F. WHY. (With 7 Figures) ...................... 27
Chapter 5
Prognosis and Management. E. M. GILBERT, A. DI LENARDA,
and J. B. O'CONNELL. (With 5 Figures) ................... 46
Part III. Hypertrophic Cardiomyopathy
Chapter 6
Clinical Aspects. J. F. GOODWIN. (With 5 Figures) . . . . . . . . . . 67
Chapter 7
Pathology. E. G. J. OLSEN and S. E. TROTTER. (With 2 Figures) 85
Chapter 8
Clinical Investigation: Current Approaches. P. J. RICHARDSON
and H. J. F. WHY. (With 9 Figures) ...................... 94
X Contents
Chapter 9
Haemodynamic Disorders: Management and Prognosis
J. T. STEWART and W. J. McKENNA. . . . . . . . . . . . . . . . . . . . . . 108
Part IV. Restrictive Cardiomyopathy
Chapter 10
Clinical Aspects. J. F. GOODWIN. (With 10 Figures) . . . . . . . . . 147
Chapter 11
Pathology. E. J. G. OLSEN and S. E. TROTTER
(With 3 Figures) . . .. . . . . . . . . . . . . . .. . . . . . . . . ... . . . . . . . . . 165
Chapter 12
Doppler Echocardiographic Investigations. H. ACQUATELLA
(With 6 Figures) .. . . . . . . . . . . . . . . . .. . . . . . . . . ... . . . . . . . . . 173
Part V. Amyloid Heart Disease and Others
Chapter 13
Amyloid Heart Disease and Cardiomyopathies Difficult
to Classify. C. M. OAKLEY. (With 1 Figure) .... . ... ... . .. . . 193
Part VI. Experimental, Clinical, Immunological and
Molecular Biological Research
Chapter 14
The Significance of Autoantibodies and the Role of the
ADP/ATP Carrier in the Pathogenesis of
Viral Heart Disease, Myocarditis and Dilated Cardiomyopathy
H.-P. SCHULTHEISS. (With 11 Figures) .................... 215
Chapter 15
Experimental Studies of Humoral and Cellular
Immunological Parameters of Viral Heart Disease
B. MAISCH and M. HERZUM. (With 6 Figures) . . . . . . . . . . . . . . 233
Chapter 16
Enteroviral Heart Muscle Disease: Current Molecular
Studies and Future Research. L. CUNNINGHAM,
P. J. RICHARDSON, and L. C. ARCHARD. (With 3 Figures) 275
Contents XI
Chapter 17
The Molecular Genetics of Familial Hypertrophic
Cardiomyopathy. H. WATKINS, C. E. SEIDMAN, and
W. J. McKENNA. (With 7 Figures) ... . . . . . . . . . . . . . . . . . . . . 289
Subject Index ......................................... 307
List of Contributors
ACQUATELLA, H., Centro Medico 275, San Bernardino, Caracas 1011,
Venezuela
ARCHARD, L. c., Department of Biochemistry, Charing Cross &
Westminster Medical School, London W6 8RF, UK
CUNNINGHAM, L., Department of Biochemistry, Charing Cross &
Westminster Medical School, London, UK
GILBERT, E. M., Division of Cardiology, 4A-l00 University of Utah
Medical Centre, 50 North Medical Drive, Salt Lake City, UT
84132, USA
GOODWIN, J. F., Department of Cardiology, Royal Postgraduate
Medical School, Du Cane Road, London W12 ONN, UK
HERZUM, M., Department of Internal Medicine and Cardiology,
Philipps-University Marburg, W-3550 Marburg, Germany
LENARDA, A. Di, Division of Cardiology, 4A-100 University of
Utah Medical Centre, 50 North Medical Drive, Salt Lake City,
UT 84132, USA
MAISCH, B., Department of Internal Medicine and Cardiology
Philipps-University Marburg, W-3550 Marburg, Germany
McKENNA, W. J., Department of Cardiological Sciences, St. George's
Hospital Medical School, Cranmer Terrace, London SW17 ORE,
UK
OAKLEY, C. M., Department of Cardiology, Royal Postgraduate
Medical School, Hammersmith Hospital, Du Cane Road, London
W120NN, UK
O'CONNELL, J. B., Department of Medicine, University of Mississippi
Medical Center 2500 North State Street, Jackson, MS 39216
4505, USA