Cardiomyopathy and myocarditis
Heart Disease
Braunwald
CV R4 李威廷醫師Supervisor: 劉秉彥醫師
Cardiomyopathy
• Dilated cardiomyopathy• Hypertrophic cardiomyopathy• Restrictive cardiomyopathy• Arrhythmogenic right ventricular cardiomyopathy• Unclassified cardiomyopathy
Dilated Cardiomyopathy (DCM)
• Idiopathic DCM: incidence: > 5—8 / 100,000 per year
¼ die < 1 year; ½ die < 5 year
¼ fresh cases improve
Poor prognosis: S3
Ventricular arrhythmia
advanced age
greater ventricular enlargement
worse ventricular dysfunction
cardiopulmonary exercise test
DCM-- pathology
• Macroscopic heart enlargement
cardiac valve, coronary artery: normal
intracavitary thrombi (apex)
• Microscopic interstitial and perivascular fibrosis
myocytes size variation
DCM-- etiology
• Genetic: 20%
autosomal dominant, recessive, X-link
• Viral infection:
• Immunological abnormalities: HLA-DR4, T-cell
• Cytokines, sympathetic nervous system
DCM• History: virus, alcohol, chest pain
• Physical: blood pressure, pulsus alternans, JVE,
Cheyne-Stokes breathing, edema, S4, S3
• Laboratory: Ca/P/Fe, thyroid function, HIV,
CXR: pleural effusion, cardiomegaly
• EKG: sinus tachycardia, PRWP, LBBB, Q wave, VT
• Echo: LV, valve, pericardium,
• Radionuclide (Tl-201): LV function, wall motion
• Cath: biopsy, LV, LVEDP/PCWP, CAD,
DCM--treatment
• Beta-antagonist: Dilatrend, Bisoprolol, Metoprolol
• CCB: (not first line therapy)
• Anti-arrhythmia: ICD• Anti-coagulant: coumadin (atrial fibrillation, stroke, thrombus)
• Immunosuppressant:
• Dual chamber pacing:• Surgery: MVR, heart transplantation
• Diet, physical exercise
Alcoholic Cardiomyopathy• 1/3 DCM in western world
• Alcoholic consumer >10 years
• Insidious, Direct toxin and thiamine deficiency
• Atrial fibrillation/ flutter, VPCs, PRWP, 1-AVB, LBBB/RBBB, QT prolong
• Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, palpitation, syncope, atypical chest pain
• Pleural effusion• Alcohol abstinence as early as possible
Arrhythmogenic right ventricular cardiomyopathy (ARVD)
• Myocardial loss with adipose or fibrous replacement at RV
• Reentrant VT with RV origin• 1/3 autosomal dominant• Antiarrhythmia agents, ablation, ICD
Hypertrophic cardiomyoathy
• Idiopathic hypertrophic subaortic stenosis (IHSS), muscular subaortic stenosis
• Most people do not have an outflow gradient or stenosis of the LV outflow tract
HCM• LV hypertrophy: disproportinate involvement (ASH,
asymmetrical septal hypertrophy), small LV cavity
• LV diastolic dysfunction: • Mitral valve elongation, anterior displacement of mitral
valve, MAC, LVOT pressure gradient• Disarray: disorganization of the myofibrillar architecture• Myosin-binding protein C
• Apical HCM: spadelike, giant negative precordial T wave
HCM• Autosomal dominant: 50% (myosin heavy chain,
troponin, tropomyosin gene)• Septal hypertrophy • Systolic anterior motion of mitral leaflet• Elevated filling pressure
• Myocardial ischemia: increased muscle mass inadequate capillary density elevated diastolic filling pressures abnormal intramural coronary arteries impaired vasodilatory reserve systolic compression of arteries enhanced myocardial oxygen demand
HCM• Young: syncope, sudden death
• Old: dyspnea (90%), angina pectoris, syncope, near-syncope, palpitation, fatigue, sudden death
• Transmural infarction: absence of narrowing of the coronary artery (impaired diastolic relaxation)
• S4, double apical impulse, JVE, brisk carotid pulse, S3 (+/-)
• Holosystolic murmur: LVOT turbulence + MR apex and LSB, not into neck vessels
• Diastolic rumbling murmur (MR)
HCM
• EKG: LAD, LVH, Q (II, III, aVF, V2—V6)
• Arrhythmia: VT, Af
• CXR: cardiomegaly (LAE)
Systolic anterior motion (SAM)
• Abnormal papillary muscle or elongated leaflets• Abnormal position in the outflow tract• Venturi effect: lower pressure as blood is ejected at a
high velocity through a narrowed outflow tract
• Small ventricular cavity• Increased wall motion of posterior wall• Relative septal wall hypokinesis• Mitral valve prolapse• Turbulent flow at outflow tract
catheterization• ¼ Pulmonary hypertension• MR• LV spadelike (apical hypertrophy)
• Valsalva maneuver, • Postextrasystolic potentiation: Brocken-brough-
Braunwald phenomenon• Postitive inotropic effect: digitalis, isoproterenol, NTG
(hypovolemia)• Exclude CAD
HCM—natural course
• 1% annual mortality• Sudden death: young age (children), family history,
non-sustained VT, abnormal BP in exercise, genetic abnormality, bradyarrhythmia (abnormal AV conduction
system),
• Atrial fibrillation
HCM –management (1) • Digitalis: avoid• Diuretics: cautious use• Beta agonist: worsen outflow gradient
• Beta-antagonist: improve diastolic filling, for angina, pre-syncope, dyspnea, and sudden death
• Calcium antagonist (verapamil): improve diastolic filling
• Calcium antagonist (Nifedipine): improve chest pain
• Anti-arrhythmia: amiodarone, sotalol• Anticoagulant: for chronic Af
HCM –management (2)
• Dual chamber DDD pacemaker: reduce 25% gradient, improve exercise capacity, worsen hemodynamic variables
• ICD: benefit in improving sudden death
• Septal ablation:
• Surgical treatment: myectomy, mitral valve replacement
DCM v.s. HCM
Restrictive cardiomyopathy
• Abnormal diastolic function• Chronic constrictive pericarditis (MRI, or radionucleotide
image )
• Square-Root Sign (dip and plateau)• Elevated ventricular filling pressure: increase early LV
filling velocity, and decreased atrial filling velocity
• Exercise intolerance, exertional chest pain, weakness, dyspnea
Constrictive - Restrictive Pattern“Square-Root Sign” or “Dip-and-Plateau”
Amyloidosis• Monoclonal plasma cells produce light chain
• Autosomal dominant
• Cardiomyopathy, neuropathy, nephropathy
• Myocardial fibers of papillary muscle
• Peripheral edema, congestive heart failure (R’t side), angina pectoris, orthostatic hypotension, conduction disturbance
• S4 is less common (reduced atrial function)
Amyloidosis
• EKG: Af, AV dissociation, diffuse diminished voltage
• CXR: pulmonary congestion
• Echo: thickened wall, small ventricle, atrial dilatatiom, normal systolic function, abnormal diastolic function, pericardial effusion,
• Tc-99m:
• Biopsy:
Amyloidosis
• Digoxin: increase arrhythmia• Nefidipine: CHF exacerbation (enhanced negative
inotropic effect)
• Permanent pacemaker:
• Diuretics & vasodilator: low dose
• Anticoagulant: even no atrial arrhythmia
• Autologous stem cell transplantation:
• Heart transplantation:
Inherited infiltrative disorders
• Fabry disease: glycolipid, in endothelium, myocardium, and mitral valve HTN, CHF, MR
• Gaucher disease: cerebrosides LV dysfunction, hemorrhagic pericardial effusion
• Hemochromatosis: ventricular wall thickening, DCM + RCM, ventricular and conduction system involvement
• Glycogen storage diseases
Sarcoidosis• Granulomatous disorder, esp. lung (fibrosis)• <5% myocardial involvement (ventricular free wall and
interventricular septum)• Transmural involvement, pericardial effusion• Sudden death, congestive heart failure, conduction
disturbance, syncope, arrhythmia• Varying degrees of intraventricular or atrioventricular
block, pathological Q• Negative biopsy results did not exclude sarcoidosis• Abnormal regional wall motion• Immunosuppressant, steroid, ICD
Endomyocardial disease
• Most frequent in equatorial Africa• Loffler endocarditis and endomyocardial
fibrosis• Fibrotic thickening at apex and subvalvular
area• Necrotic stage thrombotic stage
fibrotic stage• Eosinophil’s granule may be cardiotoxic
Endomyocardial disease
• Loffler endicarditis
• Endomyocardial fibrosis
• Carcinoid heart disease
Lőffler endocarditis• Hypereosinophilic syndrome (eosinophil >1500/mm3, >6
months or till death, with organ involvement)
• 40+ years, male• Cardiac involvement (bi-ventricular, apex and inflow part
endocardial thickening)
Pathology
• Acute inflammatory eosinophilic myocarditis
• Thrombosis change involving small intramural coronary vessel
• Mural thormbosis with eosinophil
• Fibrotic thickening up to several millimeters
Lőffler endocarditis
• BW loss, fever, cough, rash, congestive heart failure• Systemic embolism• EKG: Af ± RBBB• Echo, Cath: probable MR, TR
• Steroid, cytotoxic agents (hydroxyurea)• s/s control
Endomyocardial fibrosis
• Tropical and subtropical Africa• Children and young adults• Large pericardial effusion (diminished QRS voltage)
• Thormbus and fibrous tissue filled the ventricle, especially apex
• Af, MR, TR, pulmonary hypertension
• No effective medical treatment
Carcinoid heart disease• Carcinoid syndrome: Cutaneous flushing, diarrhea,
endocartial plaques, and bronchoconstriction • Small bowel and appendix tumor• Usually only carcinoid tumors that invade the liver result
in carcinoid heart disease ( right heart involvement )• TR, other regurgitation
• Digitalis, diuretics, pulmonary valvuloplasty
Myocarditis • Cardiac inflammation / infection process
• Invasion of myocardium
• Production of myocardial toxin
• Immune-mediated cardiac injury (virus)
• Intercellular adhesion molecule-1 (ICAM-1)
Myocarditis• Asymptomatic (most), congestive heart failure,
ventricular arrhythmia or idiopathic DCM later• Fatigue, dyspnea, palpitation, precordial discomfort
(ischemia), chest pain (pericarditis), • Gallop, transient systolic murmur• ST change, T wave, VT, conduction defects (CAVB),• PMN (bacteria), lymphocyte (virus), macrophage,
plasma cells, eosinophils (hypersensitivity), giant cells• Supportive care, bed rest, athletic activity
limitation, steroid (?), NSAID (contraindication),
gamma globulin, ACEI
Viral myocarditis• Coxsackievirus B & A: most common, avoid
anticoagulant (hemorrhagic pericardial effusion)
• Cytomegalovirus: children
• Dengue:• Influenza: increase mortality in CV patients
• Poliomyelitis:
• Rickettsial myocarditis: Q fever, Rocky mountain spotted fever, scrub typhus
• Bacterial myocarditis: brucellosis, clostridial, diphtheria (most common cause of death), meningococcus, streptococcus (rheumatic fever), tuberculosis
• Spirochetal infection: Weil disease (aortits, coronary
arteritis, epicardial hemorrhage), Lyme carditis (AVB), syphilis (aortitis)
• Fungal infection: aspergillosis, candidiasis (endocarditis), cryptococcus, mucormycosis
• Protozoal myocarditis: trypanosomiasis (Chagas disease)
• Metazoal myocarditis: echinococcus (hydatid cyst)
Toxin and chemical damage to heart
• Cocaine: 20x MI, alpha-blocker, avoid beta-blocker
• Doxorubicin: free radical
• Interleukin: • TCA: sinus tachycardia, postural hypotension, abnormal conduction
• Lithium: ventricular arrhythmia, abnormal conduction
• Catecholamines (pheochromocytoma)
• CO: direct toxic effect + myocardial hypoxia
• Hypo-Ca, hypo-P, hypo-Mg
Hypersensitivity
• Myocardial allergic reaction• Sulfonamides, methyldopa, hydrochlorothiazide,
penicillin• Perivascular infiltration of myocardium with eosinophils,
lymphocytes, and histocytes• Asymptom, arrhythmia, sudden death• Discontinuation of drug, steroid, immunosupressant
Physical agents
• Radiation: chronic, (total dose / irradiated mass / dose schedule),
coronary capillary endothelial injury,
acute or chronic pericarditis
symptomatic MR, AR
• Heat stroke: sinus tachycardia, RA, RV dilate
• Hypothermia: bradycardia, arrhythmia, reversible cardiac suppression
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