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Cardiomyopathies:Morphofu
nctional phenotype
50 YEARS OF HUMAN UNDERSTANDING
1956
• Myocardial diseases classified
as myocarditis (inflammatory
heart muscle disease), and
myocardiosis (other heart
muscle diseases)
• Blankerhorn MA, Gall EA.
Myocarditis and myocardiosis:
a clinicopathologic appraisal.
Circulation 1956;13:217–23.
1957
• The term cardiomyopathy
proposed for uncommon,
noncoronary heart muscle
diseases
• Bridgen W. Uncommon
myocardial diseases:the non-
coronary cardiomyopathies.
Lancet 1957;273:1179–84.
1972
Cardiomyopathy described as
myocardial diseases of unknown
origin, and first classification
proposed as
dilated,hypertrophic, and
restrictive (or obliterative)
cardiomyopathy
• Goodwin JF, Oakley CM. The
cardiomyopathies.Br Heart J
1972;34:545–52.
1980 WHO
Defines cardiomyopathies as
myocardial diseases of unknown
etiology. WHOISFC
adds specific heart muscle
diseases (cause of myocardial
affliction known) to the
classification
• Report of the WHO/ISFC Task
Force on the definition and
classification of
cardiomyopathies.Br Heart J
1980;44:672–3.
1996
WHO-ISFC updates its
classification of
cardiomyopathies (diseases of
myocardium associated
withmyocardial dysfunction). The
update includes arrhythmogenic
right ventricular cardiomyopathy
and unclassified
cardiomyopathy, but excludes
specific heart muscle disease.
Richardson P, McKenna W,
Bristow M, et al.Report of the
1995 World Health
Organization/International
Society and Federation of
Cardiology Task Force on the
Definition and Classificationof
cardiomyopathies. Circulation
1996;93:841–2.
1998
• 1998 ISFC becomes WHF
AHA 2006
Defines cardiomyopathies as diseases of myocardium associated
with mechanical and/or electrical dysfunction, which usually (but not
invariably) exhibit inappropriate ventricular hypertrophy or dilation,due
to a variety of causes that frequently are genetic, classified as
primary or secondary. Presents first visionary attempt to classify
primary cardiomyopathy by genetic origin (genetic, acquired, or
mixed)
ESC 2008
Defines cardiomyopathies as myocardial disorder in which the heart
muscle was structurally and functionally abnormal. Classified dilated,
hypertrophic, restrictive,arrhythmogenic right ventricular, or
unclassified cardiomyopathy subtypes as familial/genetic and
nonfamilial/nongenetic. Maintained the importance of phenotype
preceding genetic classification for clinical practice.
2013 WHF-MOGE(S)
1. M: MORPHO-FUNCTIONAL
PHENOTYPE
2. O: INVOLVED ORGANS
3. G: GENETIC
4. E: ETIOLOGY
5. S: FUNCTIONAL STATUS
1.Arbustini E, Narula N, Dec WG, et al. The
MOGE(S) Classification for a phenotype–genotype
nomenclature of cardiomyopathy. Endorsed by the
World Heart Federation. J Am Coll Cardiol 2013;
62:2046–72.
2. Arbustini E, Narula N, Dec WG, et al. The
MOGE(S) classification for a phenotype–genotype
nomenclature of cardiomyopathy. endorsed by the
World Heart Federation. G Heart 2013;8:355–82.
Inheritance
• Majority :Autosomal dominant
• Minority:Recessive,X-linked, or matrilinear
Classification trend over last 50 yrs
• From pathological(1956) to genetic (2013) to morphofunctional
phenotype-based
One example
Pedigree chart-1
50 years’ effort to unravel this beauty

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MOGES classification of cardiomyopathy 2013

  • 2. 1956 • Myocardial diseases classified as myocarditis (inflammatory heart muscle disease), and myocardiosis (other heart muscle diseases) • Blankerhorn MA, Gall EA. Myocarditis and myocardiosis: a clinicopathologic appraisal. Circulation 1956;13:217–23.
  • 3. 1957 • The term cardiomyopathy proposed for uncommon, noncoronary heart muscle diseases • Bridgen W. Uncommon myocardial diseases:the non- coronary cardiomyopathies. Lancet 1957;273:1179–84.
  • 4. 1972 Cardiomyopathy described as myocardial diseases of unknown origin, and first classification proposed as dilated,hypertrophic, and restrictive (or obliterative) cardiomyopathy • Goodwin JF, Oakley CM. The cardiomyopathies.Br Heart J 1972;34:545–52.
  • 5. 1980 WHO Defines cardiomyopathies as myocardial diseases of unknown etiology. WHOISFC adds specific heart muscle diseases (cause of myocardial affliction known) to the classification • Report of the WHO/ISFC Task Force on the definition and classification of cardiomyopathies.Br Heart J 1980;44:672–3.
  • 6. 1996 WHO-ISFC updates its classification of cardiomyopathies (diseases of myocardium associated withmyocardial dysfunction). The update includes arrhythmogenic right ventricular cardiomyopathy and unclassified cardiomyopathy, but excludes specific heart muscle disease. Richardson P, McKenna W, Bristow M, et al.Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classificationof cardiomyopathies. Circulation 1996;93:841–2.
  • 7. 1998 • 1998 ISFC becomes WHF
  • 8. AHA 2006 Defines cardiomyopathies as diseases of myocardium associated with mechanical and/or electrical dysfunction, which usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilation,due to a variety of causes that frequently are genetic, classified as primary or secondary. Presents first visionary attempt to classify primary cardiomyopathy by genetic origin (genetic, acquired, or mixed)
  • 9. ESC 2008 Defines cardiomyopathies as myocardial disorder in which the heart muscle was structurally and functionally abnormal. Classified dilated, hypertrophic, restrictive,arrhythmogenic right ventricular, or unclassified cardiomyopathy subtypes as familial/genetic and nonfamilial/nongenetic. Maintained the importance of phenotype preceding genetic classification for clinical practice.
  • 10. 2013 WHF-MOGE(S) 1. M: MORPHO-FUNCTIONAL PHENOTYPE 2. O: INVOLVED ORGANS 3. G: GENETIC 4. E: ETIOLOGY 5. S: FUNCTIONAL STATUS 1.Arbustini E, Narula N, Dec WG, et al. The MOGE(S) Classification for a phenotype–genotype nomenclature of cardiomyopathy. Endorsed by the World Heart Federation. J Am Coll Cardiol 2013; 62:2046–72. 2. Arbustini E, Narula N, Dec WG, et al. The MOGE(S) classification for a phenotype–genotype nomenclature of cardiomyopathy. endorsed by the World Heart Federation. G Heart 2013;8:355–82.
  • 11. Inheritance • Majority :Autosomal dominant • Minority:Recessive,X-linked, or matrilinear
  • 12. Classification trend over last 50 yrs • From pathological(1956) to genetic (2013) to morphofunctional phenotype-based
  • 15.
  • 16. 50 years’ effort to unravel this beauty