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Transcript
CARDIOMYOPATHIES • “Heart disease resulting from a primary abnormality in the myocardium” Figure 12-35 Pathways of dilated and hypertrophic cardiomyopathy, emphasizing several important concepts. Some forms of dilated cardiomyopathy (others are caused by myocarditis, alcohol, and other toxic injury or the peripartum state) and virtually all forms of hypertrophic cardiomyopathy are genetic in origin. The genetic causes of dilated cardiomyopathy involve mutations in any of a wide variety of proteins, predominantly of the cytoskeleton, but also the sarcomere, mitochondria, and nuclear envelope. In contrast, the mutated genes that cause hypertrophic cardiomyopathy encode proteins of the sarcomere. Although these two forms of cardiomyopathy differ greatly in subcellular basis and morphologic phenotypes, they share a common pathway of clinical complications. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 27 October 2005 05:18 AM) © 2005 Elsevier CARDIOMYOPATHIES Dilated cardiomyopathy: Progressive cardiac dilation and contractile dysfunction Hypertrophic cardiomyopathy: Myocardial hypertrophy and abnormal diastolic function Restrictive cadiomyopathy: Decrease ventricular compliance impaired diastolic function DILATED CARDIOMYOPATHY(DCM) “A form of cardiomyopathy characterized by progressive cardiac hypertrophy, dilation and contractile dysfunction.” DILATED CARDIOMYOPATHIES (DCM) Pathogenesis: Genetics: • Autosomal Dominant • Autosomal Reccessive • X Linked • Mitochondria gene mutation Acquired: • Myocarditis • Alcohol • Other toxins • Child birth DILATED CARDIOMYOPATHIES (DCM) • Progressive cardiac dilation and contractile dysfunction • Enlarged, flabby, weight 2-3 times of normal • Mural thrombi emboli • Functional regurgitation of mitral/tricuspid valve • Myocytes: hypertrophied, Nuclei enlarged, irregular, stretched • Interstial and endocardial fibrosis CLINICAL FEATURES – DCM • • • • • Commonly affects 20 to 60 years old Slowly progressive CHF Less ejection fraction Death due to arrhythmia, cardiac failure Embolization DILATED CARDIOMYOPATHIES (DCM) Prognosis: • 50% death- 2 years • 25% death- 5 years • Some improvement with therapy • Transplantation frequently done CARDIOMYOPATHIES Arrythmogenic right ventricle cardiomyopathy Transmural replacement by fat and fibrosis HYPERTROPHIC CARDIOMYOPATHY (HCM) • “It is characterized by myocardial hypertrophy, abnormal diastolic filling and in about 1/3 cases intermittent left ventricular outflow obstruction”. HYPERTROPHIC CARDIOMYOPATHY (HCM) Etiology: Genetic:100% AD • Missence mutation • Alpha heavy chain myosin • Alpha Tropomyosin • MYBP- C (myosin body protien) Stroke volume: decreased HYPERTROPHIC CARDIOMYOPATHY (HCM) Gross: Hypertrophy outflow tract obstruction Microscopy: • Myocytes hypertrophy • Septal hypertrophy • Interstial fibrosis • Thickened septal vessels • Transvers diameter 40um(15um) CARDIOMYOPATHIES Restrictive Cardiomyopathy Decrease in ventricular complianceImpaired filling Endomyocardial fibrosis Loeffler endomyocarditis Endomyocardial fibroelastosis CARDIOMYOPATHIES Restrictive Cardiomyopathy Decrease in ventricular complianceImpaired filling Endomyocardial fibrosis- Children, Africa, Tropical countries Vent- endocardium, sub endocardium fibrosis- mitral, tricuspid valves Mural thrombi CARDIOMYOPATHIES Restrictive Cardiomyopathy Loeffler endomyocarditisEndomyocardial fibrosis, mural thrombi Peripheral eosinophilia Toxin- eosinophils Myeloproliferative disorders Rearrangement- PDGFRA alpha, PDGFRA beta Restrictive Cardiomyopathy Endocardial fibroelastosis • Uncommon • 2 years • Left ventricle: Fibroelastic thickening of endocardium • Aortic valve obstruction or other cardiac anomalies • Cardiac decompensation death RESTRICTIVECARDIOMYOPA THY (RCM) • Reduced chamber size and stroke volume • Impaired diastolic filling • Exertional dyspnoea • Anginal pain • Atrial fibrillation • Ventricular arrhythmia • Sudden death RESTRICTIVECARDIOMYOPATH Y (RCM) Etiology: Idiopathic,Radiation fibrosis,Amyloidosis, Sarcoidosis, Metastatic tumours Metabolic diseases- inborn error of metabolism Morphology: Ventricles: enlarged, cavities not dilated, Myocardium: firm, non compliant, I/S fibrosis, Amyloidosis TUMOURS OF THE HEART • PRIMARY ( RARE) – Myxoma, Rhabdomyoma, Fibroma, Lipoma, Papillary Fibroelastoma, Angiosarcoma, Other Sarcomas • METASTATIC (5% Cancerous patients) CARDIAC MYXOMA • • • • • Most common primary tumour Atria (90%) - Fossa ovalis Mostly single (<1 cm-10 cm) Hard to soft gelatinous mass Myxoma cells, EC, Smooth Muscle & undiff. Cells • Hamartoma / thrombus? CARDIAC MYXOMA - (contd) • • • • • • Mobile, wrecking - ball effect Ball - valve obstruction Embolization Constitutional symptoms due to IL-6 Surgical removal - curative Familial cardia myxoma (10%) CARDIAC RHABDOMYOMA • • • • Most common - infants & children Obstruction - valve / chamber Grayish-white mass Spider cells, large rounded cells. CARDIAC LIPOMA • Subendocardium, myocardium or subpericardium. • LV, right atrium or atrial septum • Asymptomatic • Ball - valve obstruction • Arrhythmia PAPILLARY FIBROELASTOMA • • • • Incidental finding - autopsy Located on valves Hair-like projections Myxoid connective tissue, elastic fibers & mucopolysaccharides CARDIAC EFFECTS OF TUMOURS NON-CARDIAC • Melanoma, Leukemia, Lymphoma, Carcinoma breast, lung, oesophagus & kidney • Effects due to infiltration, mediators or tumour therapy • Pericardial effusion, tamponade CARDIOVASCULAR EFFECTS OF NON-CARDIAC NEOPLASMS MYOCARDITIS Inflammation of myocardium causing myocardial injury WHO- 14 polysmorphs/ square milimeter, 1 macrophage MAJOR CAUSES OF MYOCARDITIS INFECTIONS Viruses (e.g. coxsackievirus, ECHO, influenza, HIV cytomegalovirus) Chlamydia (e.g. C, psittaci) Rickettsiae (e.g. R, typhi, typhus fever) Bacteria e.g. Corynebacterium diphtheriae, Neisseria meningococcus, Borrelia (Lyme disease) Fungi (e.g. Candida) Protozoa (e.g. Trypanosoma, Chagas disease, disease, toxoplasmosis) Helminths (e.g. trichinosis) MAJOR CAUSES OF MYOCARDITIS (CONTD) Immune-Mediated Reactions Postviral Poststreptococcal (rheumatic fever) Systemic lupus erythematosus Drug hypersensitivity (e.g. methyldopa, sulfonamides) Transplant rejection Unknown Sarcoidosis Giant cell mycoarditis MYOCARDITIS Gross: Flabby, Dilated, Mottled Mural thrombi Endomyocardial biopsy: I/S inflammation composed of lymphocytes, monocytes MYOCARDITIS Hypersensitivity Myocarditis Perivascular Lymphocytes, monocytes and eosinophils MYOCARDITIS Giant cell Myocarditis Giant cells, lymphocytes, monocytes, plasma cells, macrophages Poor prognosis MYOCARDITIS Chagas diseaseTrypansoma cruzi South America Acute attach- 10% mortality Cardiac insufficiency- 10- 20 yrs MYOCARDITIS Diphtheria- Toxin Borellia burgdorferi- conducting system HIVInflammation, myocyte damadge Myocarditis MYOCARDITIS LABSerology Biopsy- Viral DNA, RNA CLINICAL FEATURES - MYOCARDITIS • Broad clinical spectrum • Asymptomatic to CHF or sudden death • Fever, dyspnoea, fatigue, palpitation, chest pain • May mimic MI • May proceed to DCM PERICARDITIS & EFFUSION • Normal fluid 30-50 ml • Blood, Pus, Straw coloured fluid • Slow accumulation (<500 ml) - Globular Heart • Rapid collection - tamponade CAUSES OF PERICARDITIS MORPHOLOGICAL CLASSIFICATION • • • • • • • Serous pericarditis Serofibrinous / fibrinous pericarditis Purulent pericarditis Haemorrhagic pericarditis Caseous pericarditis Adehesive mediastinopericarditis Constrictive pericarditis HAEMORRHAGIC PERICARDITIS • CAUSES – – – – – – T.B Malignancy Trauma Cardiac surgery Bleeding diathesis Anti - coagulant therapy CASEOUS PERICARDITIS • Tuberculosis • Fungi • Results in calcific, fibrocalcific pericarditis FIBRINOUS & SEROFIBRINOUS PERICARDITIS • MI, post infraction syndrome, uraemia, radiations, rheumatic fever, SLE, trauma • Friction rub • Yellow cloudy exudate • Dry rough, granular surface • Bread and Butter appearance PURULENT PERICARDITIS • Source of infection – Direct spread – seeding from blood & lymphatics – introduction during surgery • Thin to creamy pus (400500 ml) • May lead to constrictive or mediastino- pericarditis CONSTRICTIVE PERICARDITIS • Fibrous / Fibrocalcific case • Concretio cordis • Limits diastolic function • Low cardiac output • Pericardiotomy ADHESIVE MEDIASTINOPERICARDITIS • Suppurative, caseous, irradiation, cardiac surgery • Obliterated pericardial sac • Strain on cardiac function • Retraction of ribs, diaphragm & pulsus paradoxus • Leads to cardiac hypertrophy or dilatation COMPLICATIONS OF PERICARDITIS • • • • • Tamponade Adhesive pericarditis Impairment of cardiac function Adhesion with surrounding structures Constriction CARCINOD HD Figure 12-29 Carcinoid heart disease. A, Characteristic endocardial fibrotic lesion involving the right ventricle and tricuspid valve. B, Microscopic appearance of carcinoid heart disease with intimal thickening. Movat stain shows underlying myocardial elastic tissue black and acid mucopolysaccharides blue-green. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 27 October 2005 04:48 AM) © 2005 Elsevier Figure 12-29 Carcinoid heart disease. A, Characteristic endocardial fibrotic lesion involving the right ventricle and tricuspid valve. B, Microscopic appearance of carcinoid heart disease with intimal thickening. Movat stain shows underlying myocardial elastic tissue black and acid mucopolysaccharides blue-green. Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 27 October 2005 04:48 AM) © 2005 Elsevier TRANSPLANTATION Figure 12-39 Complications of heart transplantation. A, Cardiac allograft rejection typified by lymphocytic infiltrate, with associated damage to cardiac myocytes. B, Graft coronary arteriosclerosis, demonstrating severe diffuse concentric intimal thickening producing critical stenosis. The internal elastic lamina (arrow) and media are intact (Movat pentachrome stain, elastin black). (B, reproduced by permission from Salomon RN, et al: Human coronary transplantation-associated arteriosclerosis. Evidence for chronic immune reaction to activated graft endothelial cells. Am J Pathol 138:791, 1991.) Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 27 October 2005 05:18 AM) © 2005 Elsevier TRANSPLANTATION 3000/ year First human-human: 1967 Improved outcomeImmunosuressive therapy- Cyclosporin A Glucocorticoids Selection of candidates Early diagnosis of allograft rejection COMPLICATIONS OF TRANSPLANTATION Rejection A0- No rejection R1- mild rejection R2- moderate rejection- pri vascular lympho R3- severe rejection- myocyte injury,Neu Eo, lym D/D myocarditis- fatal COMPLICATIONS OF TRANSPLANTATION Rejection Stenosing intimal proliferation- I/Mural vessels Silent infarction Pathogenesis: Chronic inflammation- GF- Smooth muscles proliferation Extracellular matrix- intimal expansion