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Cardiomyopathies

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Cardiomyopathies

  1. 1. PRESENTED BY-DR. BISWAJEETA SAHA, DEPT OF PATHOLOGY KIMS,BBSR
  2. 2. Introduction 2006 AHA defined cardiomyopathies as “a heterogeneous group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic.” Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders
  3. 3. Functional classification Dilated cardiomyopathy Hypertrophic cardiomyopathy Restrictive cardiomyopathy Arrhthymogenic Right Ventricular disease(ARVD)
  4. 4. DILATEDCARDIOMYOPATHY
  5. 5. Definition Primary (idiopathic) is a disease of unknown etiology that principally affects the myocardium leading to LV dilation and systolic dysfunction Most common of the cardiomyopathies
  6. 6. Causes Genetic influences- 20-50% are familial Autosomal dominant –predominant pattern Mutations in genes encoding dystrophin,δ sarcoglycan,troponin T,β MHC etc Myocarditis Alcohol and other toxins Childbirth (peripartum cardiomyopathy)
  7. 7. Morphology Heart enlarged,heavy,flabby Mural thrombi common Dilatation of all chambers,both ventricular hypertrophy Microscopically-atrophic and hypertrophic myocardial fibres,cardiac myocytes show degenerative changes Interstitial and endocardial fibrosis
  8. 8. Clinical features- Highest incidence in middle age Symptoms may be gradual in onset Acute presentation  Misdiagnosed as viral URI in young adults Symptoms/Signs of heart failure  Pulmonary congestion (left heart failure) dyspnea (rest, exertional, nocturnal), orthopnea  Systemic congestion (right heart failure) edema, nausea, abdominal pain, nocturia  Low cardiac output  Hypotension, tachycardia, tachypnea  Fatigue and weakness Arrhythmia  Atrial fibrillation, conduction delays,,sudden death
  9. 9. DCM - Incidence and Prognosis Prevalence is 36 per 100,000 population Third most common cause of heart failure Most frequent cause of heart transplantation Complete recovery is rare 50% die within 2yrs and 25% survive longer than 5yrs
  10. 10. HYPERTOPHICCARDIOMYOPATHY
  11. 11. Hypertrophic cardiomyopathy Characterised by myocardial hypertrophy,abnormal diastolic filling,intermittent ventricular outflow obstruction Related to defects in force generation owing to altered sarcomeric function Leading cause of LVH,unexplained by other clinical/pathologic cause Caused by mutation of genes encoding sarcomeric proteins
  12. 12. Pathogenesis Autosomal dominant with variable penetrance Remaining are sporadic Mutations are mostly missense Mutations causing HCM found in genes encoding β MHC,cardiac TnT,α tropomyosin,myosin binding protein C
  13. 13.  The major abnormality of the heart in HCM -- excessive thickening of the muscle. Thickening usually begins during early adolescence and stops when growth has finished. uncommon for thickening to progress after this age left ventricle almost always affected Hypertrophy is usually greatest in the septum, associated with obstruction to the flow of blood into the aorta
  14. 14.  Asymmetric septal hypertrophy with obstruction to the outflow of blood from the heart may occur. The mitral valve touches the septum, blocking the outflow tract. Some blood is leaking back through the mitral valve causing mitral regurgitation
  15. 15. Histologic features Extensive myocyte hypertrophy Myofiber disarray Interstitial and replacement fibrosis
  16. 16. Pathophysiology Impaired diastolic filling-----reduced stroke volume Reduced CO and increase in pulm venous pressure---exertional dyspneoa Diastolic dysfunction  Impaired diastolic filling, filling pressure Myocardial ischemia Mitral regurgitation Arrhythmias
  17. 17. Clinical features Asymptomatic  Echocardiographic finding only Symptomatic  Dyspnea in 90%  Harsh systolic ejection murmur  Angina pectoris in 75%  Fatigue, pre-syncope, syncope, risk of SCD  Palpitation, PND, CHF, dizziness  Atrial fibrillation, thromboembolism
  18. 18. RESTRICTIVECARDIOMYOPATHY
  19. 19. Restrictive cardiomyopathy Hallmark: abnormal diastolic function Rigid ventricular wall with impaired ventricular filling ,contractile functions are normal Much less common then DCM or HCM Characterised by primary disease in ventricular compliance resulting in impaired ventricular filing during diastole
  20. 20. CAUSES Primary---idiopathic Associated with – Radiation fibrosis Amyloidosis Sarcoidosis Metastatic tumors Metabolic deposition diseases
  21. 21. Morphology Ventricles are of normal size Cavities are not dilated Myocardium is firm and non compliant Biatrial dilatation is common Patchy/diffuse interstitial fibrosis
  22. 22. Clinical manifestations• Symptoms of right and left heart failure• Echo-Doppler – Abnormal mitral inflow pattern -Prominent E wave (rapid diastolic filling)  Almost invariably progresses to congestive heart failure,10% survive for 10 yrs
  23. 23. Amyloidosis  Cardiac enlargement without ventricular dilatation  Ventricular walls are thickened and rubbery  Amyloid deposition is most prominent in interstitial,perivascular and endocardial regions
  24. 24. Endomyocardial diseases Endomyocardial fibrosis  Loefflers endomyocarditis
  25. 25. hemochromatosis sarcoidosis
  26. 26. Fabrys disease Pompes disease
  27. 27. Arrythmogenic right ventricularcardiomyopathy Inherited disease of cardiac muscle RVF,rhytm disturbances,ventricular tachycardia,fibrillation Rt ventricular wall is thinned,extensive fatty infiltration and fibrosis Autosomal dominant inheritence
  28. 28. THANK YOU

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