© 1996 American Heart Association, Inc.
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,
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
Definition and Classification
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
Dilated cardiomyopathy is
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 is
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 of increased loose connective tissue.
Arrhythmias and premature sudden death are
Restrictive cardiomyopathy is
restrictive filling and reduced diastolic volume of either
or both ventricles with normal or near-normal systolic
function and wall thickness. Increased interstitial
fibrosis may be present. It may be idiopathic or associated with
other disease (eg, amyloidosis; endomyocardial
disease with or without hypereosinophilia).
Arrhythmogenic Right Ventricular
Arrhythmogenic right ventricular
cardiomyopathy is characterized by progressive
fibrofatty replacement of right ventricular
myocardium, initially with typical regional and later
global right and some left ventricular involvement, with
relative sparing of the septum.11 Familial disease is
common, with autosomal dominant inheritance and incomplete penetrance;
a recessive form is described. Presentation with
arrhythmias and sudden death is common, particularly in the
include a few
cases that do not fit readily into any group (eg, fibroelastosis,
noncompacted myocardium, systolic dysfunction with
minimal dilatation, mitochondrial involvement).
Some diseases may
present with features of more than one type of
cardiomyopathy (ie, amyloidosis, systemic
hypertension). It is recognized that arrhythmias and conduction
disease may be primary myocardial disorders. At this time, however, it
was elected not to include them as
The term specific cardiomyopathies is now used
to describe heart muscle diseases that are associated with specific
cardiac or systemic disorders. These were previously defined as
specific heart muscle diseases.
presents as a dilated cardiomyopathy with
impaired contractile performance not explained by the extent of
coronary artery disease or ischemic damage.
presents with ventricular dysfunction that is out of
proportion to the abnormal loading conditions.
Hypertensive cardiomyopathy often
presents with left ventricular hypertrophy
in association with features of dilated or restrictive
cardiomyopathy with cardiac failure.
Inflammatory cardiomyopathy is defined
by myocarditis in association with cardiac dysfunction. Myocarditis is
an inflammatory disease of the myocardium and is diagnosed
by established histological, immunological, and
immunohistochemical criteria. Idiopathic, autoimmune, and infectious
forms of inflammatory cardiomyopathy are
recognized. Inflammatory myocardial disease is involved in the
pathogenesis of dilated cardiomyopathy and other
cardiomyopathies, eg, Chagas' disease, HIV,
enterovirus, adenovirus, and cytomegalovirus.13
includes the following categories: Endocrine, eg, thyrotoxicosis,
hypothyroidism, adrenal cortical insufficiency, pheochromocytoma,
acromegaly, and diabetes mellitus; familial storage disease and
infiltrations, eg, hemochromatosis, glycogen storage disease,
Hurler's syndrome, Refsum's syndrome, Niemann-Pick disease,
Hand-Schüller-Christian disease, Fabry-Anderson disease, and
Morquio-Ullrich disease; deficiency, eg, disturbances of
potassium metabolism, magnesium deficiency, and nutritional
disorders such as kwashiorkor, anemia, beri-beri, and selenium
deficiency; amyloid, eg, primary, secondary, familial, and hereditary
cardiac amyloidoses, familial Mediterranean fever, and senile
General system disease includes connective tissue disorders,
eg, systemic lupus erythematosus, polyarteritis
nodosa, rheumatoid arthritis, scleroderma, and dermatomyositis.
Infiltrations and granulomas include sarcoidosis and leukemia.
Muscular dystrophies include Duchenne, Becker-type, and
Neuromuscular disorders include Friedreich's ataxia,
Noonan's syndrome, and lentiginosis.
Sensitivity and toxic reactions include reactions to
alcohol, catecholamines, anthracyclines, irradiation, and
miscellaneous. Alcoholic cardiomyopathy may be
associated with a heavy alcohol intake. At present we cannot define
a causal versus a conditioning role of alcohol or apply precise
Peripartal cardiomyopathy may first
manifest in the peripartum period. This is probably a
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