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Cardiomyopathies
1995 WHO/International Society and Federation
of Cardiology (ISFC) Task Force on the Definition
• All diseases affecting heart muscle
and to take into consideration etiology
• Dilated cardiomyopathy (DCM)
• Hypertrophic cardiomyopathy (HCM)
• Restrictive cardiomyopathy (RCM)
• Arrhythmogenic right ventricular
cardiomyopathy/dysplasia (ARVC/D)
• Unclassified cardiomyopathies
The MOGE(S) classification - 2013
• The morphofunctional (M) notation indicates a descriptive
phenotypic diagnosis (eg, MD = dilated cardiomyopathy)
• The organ involvement (O) notation
• The genetic or familial inheritance (G)
• The etiological annotation (E) provides description of the specific
cause
• functional status (S) -[NYHA] functional class
BEGNING TO END
• Phenome to Genome but not always
Heart failure vs NYHA FC
Athlete's heart: A close differential diagnosis
• LVH is generally symmetric and wall thickness is ≤12 mm; however, in
some athletes, LVH reaches 14 to 16 mm
• Reliance on LV wall thickness alone may be problematic and
diagnostic evaluations of athletes with suspected cardiovascular
disease should include a family history, 12-lead electrocardiogram,
and echocardiographic assessment of the distinguishing features of
athlete's heart versus HCM.
Etiologic classification of cardiomyopathy
• INFECTIVE
• GENETIC
• METABLIC
• FAMILIAL STORAGE DISORDERS
• NUTRIONAL
• OTHER
• Hematologic/oncologic
• Deposits
• Heredofamilial neurologic and
neuromuscular diseases
• Endomyocardial diseases
• Inflammatory
• Connective tissue diseases
• Granulomatous
• Other inflammation
How
cardiovascular
system works in
heart failure ?
FRANKSTARLING’S
LAW
DILATED CARDIOMYOPATHY
FAMILIAL CMP
NON FAMILIAL CMP
DCMP
DCM
Genes of
DCM
Hypertrophic cardiomyopathy
HCM VS NORMAL
• Myocyte disarray with an
irregular arrangement of
abnormal shaped myocytes that
contain bizarre nuclei and
surrounding areas of increased
connective tissue.
Restrictive cardiomyopathy
RCM
• The echocardiographic features
typical of an idiopathic
restrictive cardiomyopathy
include a nondilated,
nonhypertrophied ventricle with
dilated atria
Doppler in flow pattern in RCM
• The pulse wave Doppler in a
patient with an idiopathic
restrictive cardiomyopathy
demonstrates an increased E/A
ratio (2.4), with shortened
deceleration time (DT = 128
milliseconds).
ARVD
• transmural fatty replacement of
the right ventricular free wall
and the translucent
infundibulum.
• the replacement of the
myocardium by fat is largely
confined to the right ventricle
(arrow) and substantially spares
the interventricular septum as
well as the left ventricular free
wall
NON COMPACTION
• Compacted epicardial layer and
noncompacted endocardial layer
with marked hypertrabeculation
and deep recesses.
DIAGNSOIS OF CMP
• Clinical
• ECG
• CHEST X-RAY
• ECHO
• CMRI
• Genetic
Symptomatic Treatment
• SYMPTOMATIC :PRELOAD ,AFTERLOAD,HR,SV,BLOOD PRESSURE
Treatment towards prolonging life
• Beta blocker
• ACEI/ARB
• ARNI
• NITRATE+VASODILATOR
• TREATMENT OF CAUSE
THANK YOU
It doesn't matter
The incognito from where
The world does appreciate
Anyone who does care

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Cardiomyopathies

  • 2. 1995 WHO/International Society and Federation of Cardiology (ISFC) Task Force on the Definition • All diseases affecting heart muscle and to take into consideration etiology • Dilated cardiomyopathy (DCM) • Hypertrophic cardiomyopathy (HCM) • Restrictive cardiomyopathy (RCM) • Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) • Unclassified cardiomyopathies
  • 3. The MOGE(S) classification - 2013 • The morphofunctional (M) notation indicates a descriptive phenotypic diagnosis (eg, MD = dilated cardiomyopathy) • The organ involvement (O) notation • The genetic or familial inheritance (G) • The etiological annotation (E) provides description of the specific cause • functional status (S) -[NYHA] functional class
  • 4. BEGNING TO END • Phenome to Genome but not always
  • 6. Athlete's heart: A close differential diagnosis • LVH is generally symmetric and wall thickness is ≤12 mm; however, in some athletes, LVH reaches 14 to 16 mm • Reliance on LV wall thickness alone may be problematic and diagnostic evaluations of athletes with suspected cardiovascular disease should include a family history, 12-lead electrocardiogram, and echocardiographic assessment of the distinguishing features of athlete's heart versus HCM.
  • 7. Etiologic classification of cardiomyopathy • INFECTIVE • GENETIC • METABLIC • FAMILIAL STORAGE DISORDERS • NUTRIONAL • OTHER • Hematologic/oncologic • Deposits • Heredofamilial neurologic and neuromuscular diseases • Endomyocardial diseases • Inflammatory • Connective tissue diseases • Granulomatous • Other inflammation
  • 10.
  • 14. DCMP
  • 15. DCM
  • 18. HCM VS NORMAL • Myocyte disarray with an irregular arrangement of abnormal shaped myocytes that contain bizarre nuclei and surrounding areas of increased connective tissue.
  • 20. RCM • The echocardiographic features typical of an idiopathic restrictive cardiomyopathy include a nondilated, nonhypertrophied ventricle with dilated atria
  • 21. Doppler in flow pattern in RCM • The pulse wave Doppler in a patient with an idiopathic restrictive cardiomyopathy demonstrates an increased E/A ratio (2.4), with shortened deceleration time (DT = 128 milliseconds).
  • 22. ARVD • transmural fatty replacement of the right ventricular free wall and the translucent infundibulum. • the replacement of the myocardium by fat is largely confined to the right ventricle (arrow) and substantially spares the interventricular septum as well as the left ventricular free wall
  • 23. NON COMPACTION • Compacted epicardial layer and noncompacted endocardial layer with marked hypertrabeculation and deep recesses.
  • 24. DIAGNSOIS OF CMP • Clinical • ECG • CHEST X-RAY • ECHO • CMRI • Genetic
  • 25. Symptomatic Treatment • SYMPTOMATIC :PRELOAD ,AFTERLOAD,HR,SV,BLOOD PRESSURE
  • 26. Treatment towards prolonging life • Beta blocker • ACEI/ARB • ARNI • NITRATE+VASODILATOR • TREATMENT OF CAUSE
  • 27. THANK YOU It doesn't matter The incognito from where The world does appreciate Anyone who does care