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Restrictive cardiomyopathy

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Restrictive cardiomyopathy

  1. 1. Restrictive cardiomyopathy
  2. 2. Restrictive cardiomyopathy • Disorder characterized by a primary decrease in ventricular compliance resulting in impaired ventricular filling during diastole
  3. 3.  The myocardium is partially infiltrated by noncontractile tissue or extracellular material  This infiltration of the myocardium impairs the ability of the heart to dilate.
  4. 4. ETIOLOGY • ■ Infiltrative causes: Amyloid, sarcoid • ■ Noninfiltrative causes: Idiopathic, scleroderma. • ■ Other causes: Hemochromatosis , diffuse interstitial fibrosis,sarcoidosis, postradiation fibrosis,metastatic tumors,inborn errors of metabolism
  5. 5. Amyloidosis-heart
  6. 6. sarcoidosis
  7. 7. MORPHOLOGY • Ventricles- normal/slightly enlarged Cavities- not dilated • Myocardium- firm and non compliant • Atria- biatreal dilation • Microscopy-interstitial fibrosis
  8. 8. Clinical features  Usually causes diastolic dysfunction.  Patients have signs and symptoms of CHF with prominent right-sided features.
  9. 9. Other restrictive conditions Endomyocardial fibrosis Loeffler endomyocarditis Endocardial fibroelastosis
  10. 10. Endomyocardial fibrosis Fibrosis of ventricular endocardium and subendocardium Extends from apex upward often invovling tricuspid & mitral valves Children & young adults Africa & other tropical regions
  11. 11. Endomyocardial fibrosis • Fibrous tissue markedly diminishes the volume & compliance of affected chambers • Induces a restrictive functional defect • Ventricular mural thrombi & fibrous tissue results from its organisation • Etiology unknown