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  1. Disease of The Myocardium Professor mohammed Ahmed Bamashmos Professor of internal medicine Sanaa university
  2. Myocarditis Definition • Inflammatory process involving the myocardium ranging from acute to chronic. - An important cause of dilated cardiomyopathy . Etiology • Idiopathic • Infectious - viral (most common): coxsackie B, echovirus, poliovirus, HIV, mumps - bacterial: S. aureus, C. perfringens, C. diphtheriae, Mycoplasma, Rickettsia - fungi - spirochetal (Lyme disease - Borrelia burgdorferi) - Chagas disease (Trypanosoma cruzi), toxoplasmosis • Toxic: catecholamines, chemotherapy, cocaine • Hypersensitivity/eosinophilic: drugs (antibiotics, diuretics, lithium, clozapine), insect/snake bites • Systemic diseases: collagen vascular diseases (SLE, RA, others), sarcoidosis, autoimmune • Other: giant cell myocarditis, acute rheumatic fever
  3. Signs and Symptoms • constitutional symptoms • acute CHF • chest pain - due to pericarditis or cardiac ischemia • arrhythmias • systemic or pulmonary emboli • sudden death Investigations • ECG: non-specific ST-T changes ± conduction defects • Bloodwork - increased CK, troponin, LDH, and AST with acute myocardial necrosis ± increased WBC,ESR, ANA, rheumatoid factor - blood culture, viral titres • CXR: enlarged cardiac silhouette • Echo: dilated, hypokinetic chambers, segmental wall motion abnormalities • Myocardial biopsy
  4. • Management : – Supportive care – Restrict physical activity – Treat CHF – Treat arrhythmias – Anticoagulation – Treat underlying cause if possible • Prognosis: – Usually self- limited and often unrecognized – Most recover – May be fulminant death in 24-48 hrs – Sudden death in young adults – May progress to dilated cardimyopathy – Few may have recurrent or chronic myocarditis
  5. Dilated Cardiomyopathy (DCM) Definition • Unexplained dilation and impaired systolic function of one or both ventricles. Etiology • Idiopathic (presumed viral or idiopathic) - 50% ofDCM • Alcohol • Familial/genetic • Uncontrolled tachycardia (e.g. persistent rapid AF) • Collagen vascular disease: SLE, polyarteritis nodosa, dermatomyositis, progressive systemic sclerosis • Infectious: viral (coxsackie B, HIV), Chagas disease, Lyme disease, Rickettsial diseases, acute rheumatic fever, toxoplasmosis • Neuromuscular disease: Duchenne muscular dystrophy, myotonic dystrophy • Metabolic: uremia, nutritional deficiency (thiamine, selenium) • Endocrine: hyper/hypothyroidism, DM, pheochromocytoma • Peripartum • Toxic: cocaine, heroin • Drugs: chemotherapies (doxorubicin, cyclophosphamide), anti-retrovirals, chloroquine,clozapine, TCA • Radiation
  6. Pathophysiology : – Impaired contractile function of the myocardium 🡪 progressive cardiac dilatation and eventually, decrease ejection fraction Clinical manifestations: • CHF • Systemic or pul. Emboli • Arrhythmias • Sudden death(major cause of mortality due to fatal arrhythmia)
  7. Investigations • Bloodwork: CBC, electrolytes, Cr, bicarbonate, BNP, CK, troponin, LFTs, TSH, TIBC • ECG: variable ST -T wave abnormalities, poor R wave progression, conduction defects(e.g. BBB), arrhythmias • CXR: global cardiomegaly (globular heart), signs of CHF, pleural effusion • Echo: chamber enlargement, global hypokinesis, depressed LVEF, MR and TR, mural thrombi • Endomyocardial biopsy: not routine, used to rule out a treatable cause • Angiography: in selected patients to exclude ischemic heart disease
  8. • Management : – Treat underlying disease – Treat CHF – Anticoagulation to prevent thromboembolism – Treat symptomatic or serious arrythmias – Immunize against influenza and pneumococcus – Surgical therapy – • Cardiac transplant • Vol. reduction surgery • cardiomyoplasty
  9. Hypertrophic Cardiomyopathy (HCM) • Also known as hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic subaortic stenosis . • Issues are obstuction;arrythmia;diastolic dysfunction Pathophysiology – Symmetrical or asymmetrical hypertrophy of the myocardium either: – Non obstructive • Symptoms secondary to decreased compliance and impaired diastolic filling – Obstructive (latent or resting) • Symptoms secondary to dynamic ventricular outflow obstruction dimnishing cardiac output
  10. • Clinical manifestation : – Asymptomatic – Dyspnea – Cardiac ischemia – Presyncope, syncope – CHF – Arrhythmias – Sudden death • Hallmark signs : – Pulses • Rapid upstroke pulse • Bifid or bisferiens pulse – Precordial palpation • Localized , sustained , double / triple impulse apex beat – Percordial auscultaion • Normal or paradoxical S2 • S4 • Harse, systolic, diamond shaped murmur at apex
  11. • Factors that influence obstruction – These include any factors that • Increase ventricular contractility • Decrease preload • Decrease afterload • Investigation : – ECG – • LVH – Echocardigraphy • LVH • Diastolic dysfunction • Resting or dynamic ventricular outflow tract obstruction
  12. Treatment : • Supportive care • Avoid factors which increase obstructions • Avoid strenous exercise • Treat arrhythmias • Infective endocarditis prophylaxis • Obstruction – Beta blockers, verapamil or diltiazem • Consider surgical options • Dual chamber pacing to decrease obstruction • Arrhythmias 🡪 amiodarone Natural history : – Variable ; some improve and stabilize over time while others suffer from the complications – AF; Infective endocarditis, sudden death
  13. Restrictive Cardiomyopathy (RCM) Definition • Impaired ventricular filling with preserved systolic function in a non-dilated, non-hypertrophied ventricle secondary to factors that decrease myocardial compliance (fibrosis and/or infiltration) Etiology • Infiltrative: amyloidosis, sarcoidosis • Non-infiltrative: scleroderma, idiopathic myocardial fibrosis • Storage diseases: hemochromatosis, Gaucher's disease, glycogen storage diseases • Endomyocardial - endomyocardial fibrosis, Loeffler's endocarditis or eosinophilic endomyocardial disease - radiation heart disease - carcinoid syndrome (may have associated tricuspid valve or pulmonary valve dysfunction)
  14. • Pathophysiology : – Infiltration of the myocardium 🡪 decreased ventricular compliance 🡪 diastolic dysfunction • Clinical manifestation: – CHF – diastolic dysfunction predominates – Arrhythmias – Systemic and pulmonary embolism
  15. Investigations • ECG: low voltage, non-specific, diffuse ST-T wave changes ± non- ischemic Q waves • CXR: mild cardiac enlargement • Echo: LAE, RAE; specific Doppler findings with no significant respiratory variation • Cardiac catheterization: increased end -diastolic ventricular pressures • Endomyocardial biopsy: to determine etiology (especially for infiltrative RCM) Management • exclude constrictive pericarditis • treat underlying disease: control HR, anticoagulate if AF • supportive care and treatment for CHF, arrhythmias • heart transplant: might be considered for CHF refractory to medical therapy Prognosis • depends on etiology
  16. Disease of the Pericardium
  17. Acute Pericarditis • Most common pathologic process involving the pericardium • Pericardial inflammation Etiology of Pericarditis : • idiopathic is most common: usually presumed to be viral • infectious - viral: Coxsackie virus A, B (most common), echovirus - bacterial: S. pneumoniae, S. aureus - TB • fungal: histoplasmosis, blastomycosis • post -MI: acute (direct extension of myocardial inflammation, 1-7 d post -MI), Dressler's syndrome (autoimmune reaction, 2-8 wks post- MI) • post-cardiac surgery (e.g. CABG), other trauma • metabolic: uremia (common), hypothyroidism • neoplasm: Hodgkin's, breast, lung, renal cell carcinoma, melanoma • collagen vascular disease: SLE, polyarteritis, RA, scleroderma • vascular: dissecting aneurysm • other: drugs (e.g. hydralazine), radiation, infiltrative disease (sarcoid
  18. Presentation : – Diagnostic traid – • Chest pain • Friction rub • ECG changes – Chest pain – alleviated by sitting up and leaning forward, pleuritic, worse with deep breathing and supine position – Percardical friction rub – may be uni , bi or triphasic – Fever +/- • Investigation : – ECG – initially elevated ST in ant., lateral, and inferior leads • Depressed PR segment • ST segment is concave upwards • 🡪 2-5 days later ST isoelectric with T wave flattening and inversion – Chest xray – normal size , pulmonary infiltrates – Echo – pericardial effusion
  19. • Treatment : – Treat the underlying disease – Anti inflammatory agent ; analgesics • Complication : – Recurrences, atrial arrhythmias, pericardial effusions, tamponade, residual contrictive pericarditis
  20. Pericardial Effusion Etiology • Transudative (serous) - CHF, hypoalbuminemia/hypoproteinemia, hypothyroidism • exudative (serosanguinous or bloody) - causes similar to the causes of acute pericarditis - may develop acute effusion secondary to hemopericardium (trauma, post-MI myocardial rupture, aortic dissection) • physiologic consequences depend on type and volume of effusion, rate of effusion development, and underlying cardiac disease Signs and Symptoms • may be asymptomatic or similar to acute pericarditis • dyspnea, cough • JVP increased • arterial pulse normal to decreased volume, decreased pulse pressure • auscultation: distant heart sounds ± rub • Ewart's sign -Bronchial breathing and dullness to percussion at the lower angle of the left scapula in pericardial effusion due to effusion compressing left lower lobe of lung.
  21. Investigations • ECG: low voltage, flat T waves • CXR: cardiomegaly, rounded cardiac contour • Echo (procedure of choice): fluid in pericardial sac • Pericardiocentesis: definitive method of determining transudate vs. exudate, identify infectious agents, neoplastic involvement Treatment • mild: frequent observation with serial echos, treat underlying cause, anti-inflammatory agents • severe: treat as in tamponade
  22. Water bottle sign – cardiac silhouette
  23. Cardiac Tamponade • Major complication of pericardial effusion • Accumulation of fluid in the pericardium in a quantity sufficient to cause serious obstruction to the inflow of blood to the ventricles results in cardiac tamponade • Pathophysiology and symptomatology – High intra pericardial pressure🡪 decreased venous return🡪 decreased diastolic ventricular filling🡪 decreased CO🡪 hypotension + venous congestion • Symptoms :tachypnoea , dyspnoea , shock • Sign – JVP raised , hepatic congestion
  24. Clinical pearl : – Classic quartet – hypotension , increased JVP, tachycardia, pulsus paradoxus(inspiratory fall in systolic BP > 10 mmHg during quiet breathing) – Beck’s triad – hypotension, increased JVP, muffled heart sounds Investigations: • ECG: electrical alternans (pathognomonic variation in R wave amplitude), low voltage • Echo: pericardial effusion, compression of cardiac chambers (RA and RV) in diastole • Cardiac catheterization
  25. • Management : – Urgent Pericardiocentesis – under ECHO, FLUOROSCOPIC – PERICARDIOTOMY – Avoid diuretics and vasodilators( these decreased venous return to already under filled RV 🡪 decrease LV preload 🡪 decrease in CO ) – Fluid administration may temporarily increase CO – Treat underlying cause
  26. Constrictive Pericarditis Etiology - Progressive thickening , fibrosis and calcification of pericardium. • chronic pericarditis resulting in fibrosed, thickened, adherent, and/or calcified pericardium • any cause of acute pericarditis may result in chronic pericarditis • major causes are idiopathic, post-infectious (viral, TB), radiation, post-cardiac surgery, uremia, MI - Tubercular pericarditis is a common cause
  27. Symptoms & sign: – Dyspnoea , fatigue, palpitations – Abdominal pain – Mimics CHF ( ascites, hepatosplenomegaly, edema) (especially right-sided HF) – Increased JVP, kussmaul’s sign(paradoxical increase in JVP with inspiration). – Pericardial knock (early diastolic sound) – BP usually normal (and usually no pulsus paradoxus)
  28. Investigations • ECG: non-specific • CXR: pericardial calcification, effusions • Echo/CT/MRI: pericardial thickening • Cardiac catheterization: equalization of end-diastolic chamber pressures (diagnostic) Treatment • Medical: diuretics, salt restriction • Surgical: pericardiectomy (only if refractory to medical therapy) • Prognosis best with idiopathic or infectious cause and worst in post-radiation. Death may result from heart failure