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