Cardiomyopathy

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Cardiomyopathy

• A disease due to Primary Pathology in the
  Myocardium
• i.e. No evidence of significant occlusion of the
  Coronary Arteries.
• i.e. No evidence of Ischemic Cardiomyopathy

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Classification

• Dilated (Common)
• (Systolic Disorder)
• Hypertrophic (Uncommon)
• (Diastolic Disorder)
• Restrictive (Rare)
• (Both Systolic Diastolic)

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Dilated Cardiomyopathy

• Clinical Any Age
• Patients present with
• Arrythmia (Atrial Fib)
• Congestive Failure
• Pul. Edema
• J.V.P. ? ?
• Thromboembolic Disease
• Echo ? Poor contraction of L.V. or R.V. during
  systole
• Causes Idiopathic
• Post Viral
• Alcohol
• Familial Right Ventricular Cardiomyopathy
  (Italy)
• Muscular Dystrophy
• Fredreichs Ataxia

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Pathology

• Heart heavy 600 grams
• All 4 chambers dilated flabby
• L.V. wall may appear thin or normal because of
  dilation of microfibres
• L.V. wall 12-15mm thick
• Valves normal
• Coronary Arteries normal
• HistologyHypertrophy of cardiac
  nuclei
• Interstitial Fibrosis

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Hypertrophic Cardiomyopathy H.O.C.M.

• Clinical
• Sudden Death of a young adult esp. during
  exercise!
• History of Drop Attacks esp. during vigorous
  exercise.
• Dyspnoea Angina Intramyocardial Coronary
  Arteries occluded
• Harsh Systolic Murmur

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Pathogenesis

• Genetic Defect Hereditary
• Autosomal Dominant
• 4 genes encode
• Proteins of contraction
• Myosin genes Troponin
• 50 different mutations
• Echo Diagnostic
• Septal Hypertrophy
• Vigorous Contractions of L.V.
• Poor Diastolic Filling

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• Gross
• Disproportionate thickening of septum compared to
  Free Wall Typical
• Microscopic
• A Microfibre Disarray in sections from Septum
• B Massive enlargement of cardiac nuclei

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Restrictive Cardiomyopathy

• So called because ventricular filling is severely
  restricted because muscle does not relax during
  diastole.
• Clinical Because of above
• get elevated J.V.P.
• Pul. Edema and
• Rt Heart Failure
• Liver
• Ankle Edema etc
• Echo Impaired Ventricular filling
• Normal systole

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Causes

• Amyloid of Heart
• Sarcoid of Heart
• Hemochromatosis
• (Iron storage disease)

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Microscopic

• Deposits of Amyloid Protein between the
  microfibres
• Diagnosis- Congo Red
• Stain Amyloid
• Red with
• Green Briefringence
• Under polarised light

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• Bio - ? pleated sheet arrangement of amyloid

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Amyloid Heart (500 grams)

• Gross
• Laying down of insoluble protein called amyloid
  between the microfibres
• Causes
• Causes stiffness in the tissue and they take
  on a firm waxy appearance.
• Heart muscle becomes non-compliant and get
  failure to Contract Relax during systole and
  diastole

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