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CARDIOMYOPATHY
• Cardiac diseases due to intrinsic myocardial
dysfunction.
Primary & secondary.
• The three major types are
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
RESTRICTIVE CARDIOMYOPATHY
• Characterized by a primary decrease in ventricular
compliance, resulting in impaired ventricular filling
during diastole.
• Diastolic dysfunction ; but systolic function is
unaffected.
• May be confused with constrictive pericarditis or
HCM.
• Idiopathic or associated with systemic diseases.
MORPHOLOGY
• Ventricular size – approx. normal or slightly enlarged
in size.
• Cavities – not dilated
• Myocardium – firm
• Atria – biatrial dilation due to poor ventricular filling
& pressure overloads.
• Microscopic examination – various degrees of
interstitial fibrosis.
• Three major forms
a. Amyloidosis
b. Endomyocardial fibrosis
c. Loeffler endomyocarditis
AMYLOIDOSIS
• Restricted to heart (senile cardiac amyloidosis)or can
occur with systemic amyloidosis.
• Deposition of normal or mutant forms of
transthyretin.
• Mutant form – 4% of African Americans, four fold
increased risk.
• Firm & rubbery consistency with small , semi-
translucent nodules.
ENDOMYOCARDIAL FIBROSIS
• Most common form of RCM.
• Children & young adults of Africa & tropical regions.
• Dense diffuse fibrosis of the ventricular endocardium
& subendocardium.
• The fibrous tissue markedly decreases volume &
compliance.
• Usually invovles tricuspid & mitral valves.
• ETIOLOGY
a. Nutritional deficiencies
b. Inflammations related to helminthic infections.
c. Viral infections
• MICROSCOPY
a. Endocardium & inner third myocardium –
fibrous tissue deposition.
b. Mononuclear inflammatory cell infiltrate.
LOEFFLER ENDOMYOCARDITIS
• Similar to endocardial fibrosis but, differs in;
No geographic predilection
Peripheral blood eosinophilic leucocytosis
Inflammatory infiltrate mainly consists of
eosinophills.
Worse prognosis.
• Histologic examination – peripheral hypereosinophilia &
eosinophillic tissue infiltrates.
• Release of eosinophilic granule contents, especially
major basic protien , causes endo & myocardial necrosis ,
scarring & layering of endocardium by thrombus (
thrombus formation).
• Underlying hypereosinophilic myeloproliferative disorder
driven by constitutionally active PDGFR tyrosine kinases.
• Treatment with tyrosine kinase inhibitors can result in
hemotologic remission & reversal of endomyocardial
lesions.
SYMPTOMS OF RCM
• Shortness of breath (initially with exertion , over time
at rest.)
• Fatigue
• Edema in legs & feet.
• Weight gain
• Nausea, bloating & poor appetite.
• Palpitations
OTHER FORMS OF RCM
• Heamochromatosis
• Myocardial sarcoidosis
• Carcinoid syndrome
• Scleroderma
• Neoplastic infiltration
THANK YOU

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Restrictive cardiomyopathy

  • 1.
  • 2. CARDIOMYOPATHY • Cardiac diseases due to intrinsic myocardial dysfunction. Primary & secondary. • The three major types are Dilated cardiomyopathy Hypertrophic cardiomyopathy Restrictive cardiomyopathy
  • 3. RESTRICTIVE CARDIOMYOPATHY • Characterized by a primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole. • Diastolic dysfunction ; but systolic function is unaffected. • May be confused with constrictive pericarditis or HCM. • Idiopathic or associated with systemic diseases.
  • 4. MORPHOLOGY • Ventricular size – approx. normal or slightly enlarged in size. • Cavities – not dilated • Myocardium – firm • Atria – biatrial dilation due to poor ventricular filling & pressure overloads. • Microscopic examination – various degrees of interstitial fibrosis.
  • 5. • Three major forms a. Amyloidosis b. Endomyocardial fibrosis c. Loeffler endomyocarditis
  • 6. AMYLOIDOSIS • Restricted to heart (senile cardiac amyloidosis)or can occur with systemic amyloidosis. • Deposition of normal or mutant forms of transthyretin. • Mutant form – 4% of African Americans, four fold increased risk. • Firm & rubbery consistency with small , semi- translucent nodules.
  • 7. ENDOMYOCARDIAL FIBROSIS • Most common form of RCM. • Children & young adults of Africa & tropical regions. • Dense diffuse fibrosis of the ventricular endocardium & subendocardium. • The fibrous tissue markedly decreases volume & compliance. • Usually invovles tricuspid & mitral valves.
  • 8. • ETIOLOGY a. Nutritional deficiencies b. Inflammations related to helminthic infections. c. Viral infections
  • 9. • MICROSCOPY a. Endocardium & inner third myocardium – fibrous tissue deposition. b. Mononuclear inflammatory cell infiltrate.
  • 10. LOEFFLER ENDOMYOCARDITIS • Similar to endocardial fibrosis but, differs in; No geographic predilection Peripheral blood eosinophilic leucocytosis Inflammatory infiltrate mainly consists of eosinophills. Worse prognosis.
  • 11. • Histologic examination – peripheral hypereosinophilia & eosinophillic tissue infiltrates. • Release of eosinophilic granule contents, especially major basic protien , causes endo & myocardial necrosis , scarring & layering of endocardium by thrombus ( thrombus formation). • Underlying hypereosinophilic myeloproliferative disorder driven by constitutionally active PDGFR tyrosine kinases. • Treatment with tyrosine kinase inhibitors can result in hemotologic remission & reversal of endomyocardial lesions.
  • 12. SYMPTOMS OF RCM • Shortness of breath (initially with exertion , over time at rest.) • Fatigue • Edema in legs & feet. • Weight gain • Nausea, bloating & poor appetite. • Palpitations
  • 13. OTHER FORMS OF RCM • Heamochromatosis • Myocardial sarcoidosis • Carcinoid syndrome • Scleroderma • Neoplastic infiltration