(Circulation. 1996;93:841-842.)
© 1996 American Heart Association, Inc.
Articles |
Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies
Correspondence to W.J. McKenna, MD, Department of Cardiological Sciences, St George's Hospital Medical School, Cranmer Terrace, London SW17 0RE, England.
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Introduction |
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A classification serves to bridge the gap between ignorance and knowledge.1 Previously the cardiomyopathies were defined as "heart muscle diseases of unknown cause" and were differentiated from specific heart muscle disease (of known cause).2 With increasing understanding of etiology and pathogenesis, the difference between cardiomyopathy and specific heart muscle disease has become indistinct. The original classification described three types, which have become established clinical entities, and this terminology has been preserved. The cardiomyopathies are now classified by the dominant pathophysiology or, if possible, by etiological/pathogenetic factors.
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Definition and Classification |
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Cardiomyopathies are defined as diseases of the myocardium associated with cardiac dysfunction. They are classified as dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy.
Dilated Cardiomyopathy
Dilated cardiomyopathy is
characterized by
dilatation and impaired contraction of the left ventricle or both
ventricles. It may be idiopathic, familial/genetic,
viral3 4 5 and/or
immune,6 7 alcoholic/toxic,
or associated with recognized cardiovascular disease in
which the degree of myocardial dysfunction is not explained by the
abnormal loading conditions or the extent of ischemic damage
(see below). Histology is nonspecific. Presentation is
usually with heart failure, which is often progressive.
Arrhythmias, thromboembolism, and sudden death are common and
may occur at any stage.
Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy is
characterized
by left and/or right ventricular hypertrophy,
which is usually asymmetric and involves the
interventricular septum.8 Typically, the
left ventricular volume is normal or reduced.
Systolic gradients are common. Familial disease with autosomal
dominant inheritance predominates. Mutations in sarcomeric contractile
protein genes cause disease.9 Typical morphological
changes include myocyte hypertrophy and disarray
surrounding areas
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J. Davis, H. Wen, T. Edwards, and J. M. Metzger Thin Filament Disinhibition by Restrictive Cardiomyopathy Mutant R193H Troponin I Induces Ca2+-Independent Mechanical Tone and Acute Myocyte Remodeling Circ. Res., May 25, 2007; 100(10): 1494 - 1502. [Abstract] [Full Text] [PDF]
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S. E. Petersen, M. Jerosch-Herold, L. E. Hudsmith, M. D. Robson, J. M. Francis, H. A. Doll, J. B. Selvanayagam, S. Neubauer, and H. Watkins Evidence for Microvascular Dysfunction in Hypertrophic Cardiomyopathy: New Insights From Multiparametric Magnetic Resonance Imaging Circulation, May 8, 2007; 115(18): 2418 - 2425. [Abstract] [Full Text] [PDF]
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C. Marcassa, R. Campini, E. Verna, L. Ceriani, and P. Giannuzzi Assessment of cardiac asynchrony by radionuclide phase analysis: Correlation with ventricular function in patients with narrow or prolonged QRS interval Eur J Heart Fail, May 1, 2007; 9(5): 484 - 490. [Abstract] [Full Text] [PDF]
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R. Macedo, K. Prakasa, C. Tichnell, F. Marcus, H. Calkins, J. A. C. Lima, and D. A. Bluemke Marked Lipomatous Infiltration of the Right Ventricle: MRI Findings in Relation to Arrhythmogenic Right Ventricular Dysplasia Am. J. Roentgenol., May 1, 2007; 188(5): W423 - W427. [Abstract] [Full Text] [PDF]
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A. Staudt, P. Eichler, C. Trimpert, S. B. Felix, and A. Greinacher Fc{gamma} Receptors IIa on Cardiomyocytes and Their Potential Functional Relevance in Dilated Cardiomyopathy J. Am. Coll. Cardiol., April 24, 2007; 49(16): 1684 - 1692. [Abstract] [Full Text] [PDF]
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C. E. Canter, R. E. Shaddy, D. Bernstein, D. T. Hsu, M. R.K. Chrisant, J. K. Kirklin, K. R. Kanter, R. S.D. Higgins, E. D. Blume, D. N. Rosenthal, et al. Indications for Heart Transplantation in Pediatric Heart Disease: A Scientific Statement From the American Heart Association Council on Cardiovascular Disease in the Young; the Councils on Clinical Cardiology, Cardiovascular Nursing, and Cardiovascular Surgery and Anesthesia; and the Quality of Care and Outcomes Research Interdisciplinary Working Group Circulation, February 6, 2007; 115(5): 658 - 676. [Abstract] [Full Text] [PDF]
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A. L.P. Caforio, N. G. Mahon, M. K. Baig, F. Tona, R. T. Murphy, P. M. Elliott, and W. J. McKenna Prospective Familial Assessment in Dilated Cardiomyopathy: Cardiac Autoantibodies Predict Disease Development in Asymptomatic Relatives Circulation, January 2, 2007; 115(1): 76 - 83. [Abstract] [Full Text] [PDF]
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A. Perrot, R. Dietz, and K. J. Osterziel Is there a common genetic basis for all familial cardiomyopathies? Eur J Heart Fail, January 1, 2007; 9(1): 4 - 6. [Full Text] [PDF]
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