Restrictive cardiomyopathy-definition, pathogenesis.pptx

Restrictive cardiomyopathy. Definition, pathogenesis

1. RESTRICTIVE CARDIOMYOPATHY Pridwik A Roll No: 150

2. CARDIOMYOPATHIES Cardiac disease due to intrinsic myocardial dysfunction are termed as cardiomyopathies MAJOR PATTERNS Dilated cardiomyopathy - Systolic dysfunction Hypertrophic cardiomyopathy - Diastolic dysfunction Restrictive cardiomyopathy - Diastolic dysfunction

3. CARDIOMYOPATHIES

4. RESTRICTIVE CARDIOMYOPATHY Characterized by a decrease in ventricular compliance,resulting in impaired ventricular filling during diastole Ventricular wall is stiffer , while contractile(systolic) function is normal Due to unaffected contractile function , this abnormality can be confused with constrictive pericarditis or HCM

5. PATHOGENESIS Replacement of normal ventricular muscle with abnormal tissue Ventricle wall become stiff and rigid Ventricle become less elastic (decreased compliance)

6. PATHOGENESIS CONT…… Venticle unable to expand and fill properly during diastole Atria become enlarged due to pressure overload Restrictive cardiomyopathy

7. ETIOPATHOGENESIS Idiopathic Associated with systemic disease which affect myocardium - Radiation fibrosis , Amyloidosis , Sarcoidosis , accumulation of mucopolysaccharides & Sphingolipids

8. FORMS OF RESTRICTIVE CARDIOMYOPATHY AMYLOIDOSIS ENDOMYOCARDIAL FIBROSIS LOEFFLER ENDOMYOCARDITIS ENDOCARDIAL FIBROELASTOSIS OTHER - RADIATION FIBROSIS,SARCOIDOSIS,PRODUCTS OF INBORN ERROR OF METABOLISM

9. AMYLOIDOSIS Deposition of extracellular proteins with Beta-pleated sheet configuration Cardiac Amyloidosis can occur as a part of systemic Amyloidosis ( Multiple Myeloma) or it can be localised , ie restricted to heart ( Senile Cardiac Amyloidosis )

10. Pathogenesis of localized Amyloidosis Deposition of normal or mutant form of transthyretin in the heart of older adult patients Transthyretin- liver synthesised circulating protein that transports thyroxine and retinol Immunoglobulin light chains in AL-type amyloid not only deposit as amyloid but also have cardiotoxic property

11. ENDOMYOCARDIAL FIBROSIS Most common form of restrictive cardiomyopathy Principally disease of children and young adults in Africa and other tropical areas. Characterized by diffuse fibrosis of ventricular endocardium and subendocardium Linked to nutitional deficiency and inflammation related to helminthic infection( Hypereosinophilia)

12. Diffuse fibrosis of ventricular endocardium Decrease in volume and compliance Restrictive physiology

13. LOEFFLER ENDOMYOCARDITIS Associated with formation large mural thrombi No geographic or population predilection Characterized by - HYPEREOSINOPHILIA & EOSINOPHILIC TISSUE INFILTRATE

14. Endocardial and myocardial necrosis Scarring and layering of endocardium by thrombus Thrombus organisation Loeffler endomyocarditis Release of eosinophil granule contents (Major basic protein) Pathogeneis

15. MORPHOLOGY GROSS Ventricle are of almost normal size or only slightly enlarged Cavities are not dilated Firm myocardium Both atria dilated- due to restricted ventricular filling and pressure overloads

16. Dilated atria Normal sized ventricle

17. MICROSCOPY Variable degree of interstitial fibrosis Eosinophilic tissue infiltrate seen in case of endomyocardial fibrosis(helminth infection) and Loeffler endomyocarditis Endomyocardial biopsy reveals a specific ethology (amyloid,endomyocardial fibrosis)

18. Endomyocardial fibrosis intensely stained blue

19. Eosinophilic aggregation within cytoplasm of numerous myocytes

20. Fine interstitial Fibrosis on routine section and high lighted by trichome stain

21. Endocardium is thickened and covered by thromus Eosinophils and mononuclear inflammatory cells are present in endocardium and infiltrate into adjacent myocardium LOEFFLER ENDOMYOCARDITIS