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  • 1. Myocarditis
  • 2. • An inflammatory process of myocardium that result into the injury to cardiac myocytes.
  • 3. Major Causes of Myocarditis INFECTIONS Viruses(coxsackie,influenza,HIV,CMV) Chlamydia(C.Psittaci) Rickettsiae(R.typhi) Bacteria(Cornybacterium diphtheriae,Nisseria meningococcus,Borellia) Fungi(Candida) Protozoa(trypanosoma Cruzi,toxoplasmosis) Helminths(trichinosis)
  • 4. IMMUNE-MEDIATED REACTIONS • • • • Post viral Post Streptococcal SLE Drug hypersensitivity(e.g.methyldopa,sulphonamides) • Transplant rejections UNKNOWN Sarcoidosis Giant cell myocarditis
  • 5. Morphology • Active phase – heart may be normal or dilated • Advanced stages: the ventricular myocardium is flabby and often mottled. • Mural thrombi may be present in any chamber. • Mononuclear interstitial inflammatory infiltrate associated with focal myocyte necrosis. • Later-inflammatory lesions either resolve, leaving no residual changes, or heal by progressive fibrosis.
  • 6. Hypersensitivity myocarditis- perivascular interstitial infiltrates, composed of lymphocytes, macrophages, and a high proportion of eosinophils Giant-cell myocarditis (uncertain cause)• inflammatory cellular infiltrate containing multinucleate giant cells interspersed with lymphocytes, eosinophils, plasma cells, and macrophages. • Chagas disease: trypanosoma cruzi, mixed inflammatory cell infiltrate.
  • 7. Chagas disease - scattered trypanosomes accompanied by an inflammatory infiltrate of neutrophils, lymphocytes, macrophages, and occasional eosinophils
  • 8. Clinical Features • May be asymptomatic- recover completely without sequelae • Features of heart failure or arrhythmias, occasionally with sudden death. • fatigue, dyspnea, palpitations, precordial discomfort, and fever. • Occasionally, patients develop DCM - late complication of myocarditis.
  • 9. OTHER CAUSES OF MYOCARDIAL DISEASE Adriamycin, doxorubicin and daunorubicin Catecholamines Cyclophosphamide Amyloidosis
  • 10. Amyloidosis • caused by deposition of an insoluble extracellular fibrillar deposits of protein fragments that are prone to forming β-pleated sheets. • Cardiac amyloidosis may appear along with systemic amyloidosis • restricted to the heart, senile cardiac amyloidosis. • amyloid deposits generally occur in the ventricles and atria. • caused by the deposition of transthyretin
  • 11. Morphology • • • • normal to firm and rubbery. Usually the chambers are of normal size Can be dilated and have thickened walls. Numerous small, semi-translucent nodules resembling drips of wax • Eosinophilic deposits of amyloid may be found in the interstitium, conduction tissue, valves, endocardium, pericardium, and small intramural coronary arteries • special stains - Congo red- classic apple-green birefringence when viewed under polarized light
  • 12. • Amyloid deposits often form rings around cardiac myocytes and capillaries. • Intramural arteries and arterioles may have sufficient amyloid in their walls to compress and occlude their lumens, inducing myocardial ischemia (“smallvessel disease”).
  • 13. Pericarditis • Diseases of the pericardium include inflammatory conditions and effusions. • Isolated pericardial disease is unusual, and pericardial lesions are almost always associated with disease in other portions of the heart or surrounding structures, or are secondary to a systemic disorder.
  • 14. Pericarditis • Primary pericarditis is uncommon. • In most cases it is caused by infection. – Viruses, bacteria and fungi – Secondary to acute MI, cardiac surgery, irradiation to the mediastinum, or processes involving other thoracic structures (e.g., pneumonia or pleuritis). – Uremia is the most common systemic disorder associated with pericarditis. – Rheumatic fever, SLE, and metastatic malignancies.
  • 15. Morphology Fibrinous pericarditis (uremia): an irregular appearance to the pericardial surface (so-called bread-and-butter pericarditis). Fibrinopurulent (in acute bacterial pericarditis):Areas of frank pus; Tuberculous pericarditis: areas of caseation. Chronic pericarditis: The appearance of chronic pericarditis ranges from delicate adhesions to dense, fibrotic scars that obliterate the pericardial space. In extreme cases the heart is so completely encased by dense fibrosis that it cannot expand normally during diastole, so-called constrictive pericarditis.
  • 16. Clinical Features • Atypical chest pain, not related to exertion and often worse on reclining, and a prominent friction rub. • • When associated with significant fluid accumulation, acute pericarditis can cause cardiac tamponade, with declining cardiac output and shock. • Chronic constrictive pericarditis produces a combination of right-sided venous distention and
  • 17. • Cardiomyopathies
  • 18. Cardiomyopathies • The cardiomyopathies are a group of diseases that primarily affect the heart muscle and are not the result of congenital, acquired valvular, hypertensive, coronary arterial, or pericardial abnormalities • Primary cardiomyopathies -confined to the heart muscle • Secondary cardiomyopathies -myocardial involvement as a component of a systemic or multiorgan disorder.
  • 19. Classification of cardiomyopathies • Primary myocardial involvement 1. 2. 3. 4. Idiopthic (D,R,H) Familial (D,R,H) Eosinophilic endomyocardial disease (R) Endomyocardial fibrosis (R)
  • 20. Secondary myocardial involvement • CARDIAC INFECTIONS – Viruses, Chlamydia; Rickettsia; Bacteria; Fungi; Protozoa • TOXINS – Alcohol, Cobalt, Catecholamines, CO, Lithium, Hydrocarbons, Arsenic, Cyclophosphamide, Doxorubicin and daunorubicin
  • 21. • METABOLIC – Hyperthroidism, Hypothyroidism, Hyperkalemia, Hypokalemia, Nutritional deficiency (protein, thiamine, other avitaminoses), Hemochromatosis • NEUROMUSCULAR DISEASE – Friedreich ataxia, Muscular dystrophy, Congenital atrophies
  • 22. • STORAGE DISORDERS AND OTHER DEPOSITIONS   – Hunter-Hurler syndrome, Glycogen storage disease,  Fabry disease, Amyloidosis • INFILTRATIVE   – Leukemia, Carcinomatosis,Sarcoidosis, Radiationinduced fibrosis • IMMUNOLOGICAL   – Myocarditis (several forms) , Post-transplant rejection
  • 23. Clinical Classification of  Cardiomyopathies • Dilated: Left and/or right ventricular enlargement,  impaired systolic function, congestive heart failure,  arrhythmias, emboli • Restrictive: Endomyocardial scarring or myocardial  infiltration resulting in restriction to left and/or right  ventricular filling • Hypertrophic: Disproportionate left ventricular  hypertrophy, typically involving septum more than free  wall, with or without an intraventricular systolic pressure  gradient; usually of a nondilated left ventricular cavity
  • 24. Diagnosed as an isolated finding or associated  with other congenital heart anomalies
  • 25. DILATED CARDIOMYOPATHY (DCM) • Progressive four chamber hypertrophy and dilation • Contractile (systolic) dysfunction • Can occur at any age • Slow progressive to CHF.  • It is sometimes called congestive  cardiomyopathy.
  • 26. Pathogenesis • • • • • Genetic influence  Various acquired myocardial insults  Myocarditis  Alcohol or other toxicity Pregnancy associated. 
  • 27. Genetic influences • 20%- 50% - is familial and caused by inherited  genetic abnormalities. • Autosomal-dominant inheritance is the  predominant pattern • Most commonly affect genes that encode  cytoskeletal proteins (dystrophin). Duchene and  Beker MD  • Mutation in enzyme involved in beta-oxidation of  fatty acid
  • 28. • Myocarditis (postviral).  – DCM is a consequence of myocarditis. • Alcohol and other toxins – No morphologic features serve to distinguish  alcoholic cardiomyopathy from DCM of other  etiologies.  – Chronic alcoholism may be associated with  thiamine deficiency. 
  • 29. Morphology • The histologic abnormalities in DCM are nonspecific and usually do not point to a specific etiologic agent.  • Heart is usually enlarged, heavy (>2-3X), and  flabby  • Mural thrombi are common • No primary valvular alterations • Most muscle - are hypertrophied with enlarged  nuclei • Some are attenuated, stretched, and irregular.  • Interstitial and endocardial fibrosis –present
  • 30. Clinical Features • • • • • • • • May occur at any age ( common 20 –50yrs).  CHF   MR & Arrhythmias Ejection fractions < 25%  50%- die within 2 years 25%  survive > 5 years Death - cardiac failure or arrhythmia Embolism from dislodgment of an intracardiac thrombus  can occur.  • Cardiac transplantation is frequently done,     
  • 31. HYPERTROPHIC CARDIOMYOPATHY • Characterized by myocardial hypertrophy, abnormal diastolic filling and in 1/3 casesintermittent ventricular outflow obstruction. • The heart is thick-walled, heavy, and hypercontracting • Causes primarily diastolic dysfunction; systolic function is usually preserved.
  • 32. Pathogenesis • Mutations of genes encoding sarcomeric proteins. • Mutations - gene encoding β-myosin heavy chain (β-MHC, cardiac TnT, αtropomyosin, and myosin-binding protein C (MYBP-C)
  • 33. Morphology • Massive myocardial hypertrophy, without ventricular dilation • Classic pattern is disproportionate thickening of the ventricular septum as compared with the free wall of the left ventricle • On cross-section- “banana-like” left ventricle
  • 34. • Histologic Features – – Extensive myocyte hypertrophy to a degree unusual in other conditions, with transverse myocyte diameters frequently greater than 40 μm (15 μm); – Haphazard disarray of bundles of myocytes, individual myocytes, and contractile elements in sarcomeres within cells (termed myofiber disarray) – Interstitial and replacement fibrosis
  • 35. Clinical Features • • • • • • Exertional dyspnea Systolic ejection murmur Atrial fibrillation Mural thrombus formation Ventricular arrhythmias Sudden
  • 36. RESTRICTIVE CARDIOMYOPATHY • Characterized by primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole. • May be idiopathic or associated with distinct diseases - principally radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumors, inborn errors of metabolism.
  • 37. Morphology • Ventricles are of normal size or slightly enlarged • Cavities are not dilated • Myocardium is firm and noncompliant Microscopy: • May be only patchy or diffuse interstitial fibrosis
  • 38. • Endomyocardial fibrosis - disease of children and young adults • Fibrous tissue markedly diminishes the volume and compliance of affected chambers - induces a restrictive functional defect. • Ventricular mural thrombi sometimes develop,