Miocardite Si CMP

MIOCARDITE & CARDIOMIOPATII

Myocarditis

Inflammation Resulting in injury to cardiac myocytes (necrosis and/or

degeneration) not typical of IHD Inflammation of myocardium, that can be caused by: •Drugs. •Pathogen: Bacteria, Fungus, Virus,… •Immune reaction. •Deposition of certain substances. Infections Cardiac allograft rejection Collagen vascular diseases Drug hypersensitivity Sarcoidosis

These cause inflammatory reaction & cell damage in myocarditis NOT in ischemic disease.

Myocarditis

Infectious Etiologic Agents Viruses (most common cause in USA) Can effect young age group.

Coxsackieviruses A & B and other enteroviruses (most common agents)

CytomegalovirusHIV HIV Myocarditis.

Other

Myocarditis

Infectious Etiologic Agents Parasites

Chagas’ disease (Trypanosoma cruzi) Epidemiologically is found in South America (Brazil).

ToxoplasmosisTrichinosis T.Spiralis.

Myocarditis

Infectious Etiologic Agents Bacteria The injury is by their toxins.

Lyme disease Due to Spirochete infection.

It causes arthritis, fever & skin rash.

Diphtheria (injury from toxins of Corynebacterium diphtheriae)

Associated with weakness, hypovolemia & heart failure.

When examining the heart there will be NO Bacteria.

medslides.com 611/98

Viral Infection

Coxsackie (A, B) Echo Influenza (A, B) Polio Herpes simplex Varicella-zoster Epstein-Barr Cytomegalovirus Mumps

Rubella Rubeola Vaccinia Coronavirus Rabis Hepatitis B Arbovirus Junin virus Human immunodeficiency


Bacterial, rickettsial, spirochetal

Corynebacterium diphtheriae salmonella typhi Beta-hemolytic streptococci Neisseria meningitidis Legionella pneumophila Listeria monocytogenes Camphylobacter jejuni Coxiella burnetii (Q fever)

Chlamydia trachomatis Mycoplasma pneumoniae Chlamydia psittaci (psittacosis) Rickettsia rickettsii (Rocky Mountain spotted fever) Borrelia burgdorferi (Lyme disease) Mycobacterium tuberculosis


Protozoal, Metazoal, Fungal

Protozoal Trypnosoma cruzi (Chagas’ disease) Toxoplasma gondi

Metazoal Trichinosis Ehinococcosis

Fungal Aspergillosis Blastomycosis Candidiasis Coccidioidomycosis Cryptococcosis Histoplasmosis Mucormycosis

Noninfectious

Systemic Diseases:1. SLE2. Sarcoidosis3. Vasculitides(Wegener’s)4. Celiac disease

Neoplastic infiltration

Drugs & toxins:1. Ethanol2. Cocaine3. Radiation4. Chemotherapeutic agents - Doxorubicin


Drugs Causing Hypersensitivity Myocarditis

Antibioticsamphoericin Bampicillinchloramphenicolpenicillintetracyclinestreptomycin

Sulfonamidessufadiazinesufisoxazole

Anticonvulsantsphenindionephenytoincarbamazepine

Antitubercuousisoniazidparaaminosalicylate

Anti-inflammatoryindomethacinoxyphenbutazonephenylbutazone

Diureticsacetazolamidechlorthalidonehydrochlorothiazidespironolactone

Otheramitriptylinemethyldopasulfonylureastetanus toxoid

Myocarditis

Morphology (Gross) Cardiac dilation Myocardium flabby, pale, with focal

hemorrhages The myocardium will be flabby (soft), weak & the patient will have sever heart failure.

The heart is congested, edematous, flabby, soft, dailated & weak in pumping.

Myocarditis

Morphology (Microscopic) Acute viral

Edema Inflammatory infiltrate of lymphocytes and other

mononuclear cells Myocyte degeneration and/or necrosis Viral inclusions may be present

Parasitic Organisms identified

Bacterial Neutrophilic inflammatory infiltrate

Histology: there are edema, inflammatory exudates, damage to cells & inclusions of Virus or Bacteria.

Toxoplasma.

Giant cell myocarditis

“Fiedler’s” myocarditis MN giant cells Lymphocytes, eosinophils, necrosis Differential: Sarcoid, hypersensitivity,TB

Aggressive clinical course: indication for transplant

Giant cell myocarditis is granulomatous reaction.

Giant cell.

Hypersensitivity myocarditis

Interstitial infiltrate of macrophages, eosinophils No or little necrosis No granulomas, but multinucleated cells may be

present Fewer clinical manifestations than other forms of

myocarditis Associated with long list of drugs (classically,

methyldopa)

Eosinophils.

Cardiac sarcoidosis Cardiac involvement in 20-30% of patients with

clinical sarcoid at autopsy 5% of sarcoid patients will have symptomatic heart

disease (cardiomyopathy, arrhythmias) Sites of predilection: IVS, LV free wall, but all sites,

including pericardium, may be involved

Sarcoidosis is multi-systemic granulomatous disease that produces Non-Caseating / Non-Necrotising granuloma in lymph nodes, liver, spleen, lungs & heart.

When arrhythmia develops, the conduction system of myocardium is involved (Pirkenje fibers & nodes).

Rheumatic carditis

Uncommon cause of myocarditis Aschoff nodule

Anitschkow cell Macrophages Collagenolysis Interstitial fibrosis

PancarditisRheumatic carditis: disease of the endo-cardium (valves: chordae tendineae & cusps).

Clinical Presentation may be asymptomatic, inferred only from ST-T

changes on the ECG May also present as a fulminant condition

characterized by heart failure and arrhythmias and death

May simulate AMI and have elevated cardiac enzymes

PE is often normal but in the severe forms may have S3 and murmur of MR

Clinical Presentation May be self limited without any sequelae but

severe involvement may occur Likely progresses to a chronic form and to

dilated cardiomyopathy Patients with viral myocarditis often present

with flulike illness Patients who present with CHF may respond

to usual measures Death due to arrhythmias had been reported


Clinical Manifestations

Most cases of acute myocarditis are clinically silent

60% of pts had antecedent flulike symptoms Large number identified by heart failure

symptoms 35% of pts with myocarditis and HF have chest

pain May mimic an acute MI with ventricular

dysfunction, ischemic chest pain, ECG evidence of injury or Q waves



May present with syncope, palpitation with AV block or ventricular arrhythmia

May cause sudden death myocarditis found at autopsy in 20% of Air Force

recruits with sudden death* May present with systemic or plumonary

thromboembolic disease

JAMA 1986; 256:2696-2699


Blood studies

Sed rate elevation 60% White count elevation 25% CK-MB elevation 12%

a 4 fold rise in IgG titer over a 4-6 wk period is required to document an acute viral infection

Heart specific antibodies are nonspecific for myocarditis; also found in dilated cardiomyopathy


Electrocadiogram

sinus tachycardia is most common diffuse ST-T wave changes myocardial infarction pattern conduction delay and LBBB in 20% complete heart block causing Stokes-Adams

attacks (particularly in Japan), but rarely require a permanent pacer

supraventricular and ventricular arrhythmias


Myocarditis

H9925 9-8-98


H9925 8-30-98


Echocardiography

Useful tool in managing patients with acute myocarditis LV systolic dysfuntion is common with segmental

wall motion abnormalities LV size is typically normal or mildly dilated wall thickness may be increased ventricular thrombi detected in 15%


Endomyocardial Biopsy

RV bioptome permit repetitive sampling biopsy should be applied early after onset of

symptoms to maximize yield - resolution may be seen in four days on serial biopsies

Dallas criteria for active myocarditis“an inflammatory infiltrate of the myocardium with necrosis and/or degeneration of adjacent myocytes not typical of the ischemic changes associated with coronary artery disease”

Clinical Indications for Endomyocardial Biopsy Definite

monitoring of cardiac allograft rejection monitoring of anthracycline cardiotoxicity

Possible detection and monitoring of myocarditis diagnosis of secondary cardiomyopathies differentiation between restrictive and constrictive

heart disease

Two-dimensional parasternal long-axis view depicting disproportionate thickening, increased echogenicity,

and dyskinesis of the inferolateral wall relative to the septum; findings are consistent with tissue oedema.


Imaging Studies

Gallium 67 (avid for inflammation) showed promise for screening active myocarditis

Indium 111-antimyosin monoclonal antibody (avid for injured myocardium)

sensitivity 83% specificity 53% + predictive value 92%

endomyocardial biopsy remains the diagnostic standard

Gallium-67 scintigraphy of the myocardium, showing on top, a positive study, with hypercaptation on the heart projection

and, on the bottom, a negative study

The delayed contrast enhanced image of the same patient showed diffuse hyperenhanced areas in the typical midmyocardial location (▲▲).

LV: left ventricle. RV: right ventricle


Myocardial Treatment Trial111 patients with active myocarditis

Age 42 14 yearsSex 62% maleEjection fraction 0.24 0.10Chest pain 35%Increase MB-CK 12%Flulike symptoms 59%Increased ESR 61%Elevated WBC 24%Fever 18%

Circulation 1991;84(suppl II):II-2


Treatment

majority of patients have a self-limited disease management of LV dysfunction similar to other

forms of congestive heart failure ? exercise may intensify inflammatory response consider anticoagulation to prevent

thromboemboli consider temporary pacer for complete AV block ? prednisone and azathioprine - no apparent

benefit seen in the Myocarditis Treatment Trial

CARDIOMYOPATHIES

Definition

“A primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures … myocardial infarction, systemic hypertension, valvular stenosis or regurgitation”

Cardiomyopathies

Definition: Heart muscle disorder of unknown etiolog Disease of myocardium; the muscle it self (myocyte).

Diagnosis of exclusion Excluding the causes: ischemia, drugs, virus,…

Confusion (some exclude any disorder of known etiology, while some do not)

Can be divided to 3 groups:

1. Dilated (congestive) (90%) •Commonest type.

•Big heart on chest X-Ray.

•Patient comes with heart failure.

2. Hypertrophic (< 10%)3. Restrictive (< 2%) •Least common.

Classification

Etiology- primary or secondary

gross anatomy histology genetics biochemistry

immunology hemodynamics functional prognosis treatment

Copyright ©2006 American Heart Association

Maron, B. J. et al. Circulation 2006;113:1807-1816

Primary cardiomyopathies in which the clinically relevant disease processes solely or predominantly involve the myocardium

Functional Classification

Dilatated (congestive, DCM, IDC) ventricular enlargement and syst dysfunction

Hypertrophic (IHSS, HCM, HOCM) inappropriate myocardial hypertrophy

in the absence of HTN or aortic stenosis Restrictive (infiltrative)

abnormal filling and diastolic function

IDIOPATHIC DILATED CARDIOMYOPATHY

Definition

a disease of unknown etiology that principally affects the myocardium

Diagnosis by exclusion. LV and/or right dilatation and systolic

dysfunction, arrhythmias, heart failure pathology

increased heart size and weight ventricular dilatation, normal wall thickness heart dysfunction out of portion to fibrosis

Incidence and Prognosis

3-10 cases per 100,000 20,000 new cases per year in the U.S.A. death from progressive pump failure

1-year 25%2-year 35-40%5-year 40-80%

stabilization observed in 20-50% of patient complete recovery is rare

Idiopathic Dilated CardiomyopathyObserved Survival of 104 Patients

104

72

56 5145

37 35 3124 19 16

0

20

40

60

80

100

120

0 1 2 3 4 5 6 7 8 9 10

Years

Am J Cardiol 1981; 47:525

Predicting Prognosis in IDC

Predictive Possible Not PredictiveClinical factors symptoms alcoholism age

peripartum durationfamily history viral illness

Hemodynamics LVEF LV sizeCardiac index atrial pressure

Dysarrhythmia LV cond delay AV blocksimple VPCcomplex VPC atrial fibrillation

Histology myofibril volume other findingsNeuroendocrine hyponatremia

plasma norepinephrineatrial natriuretic factor

Dilated Cardiomyopathy

Morphology (Gross) Heart enlarged and flabby

Weight may exceed 900 grams Dilation & hypertrophy of all four chambers

Mural thrombi due to stasis CAD minimal or absent

Normal heart.

Dilated cardiomyopathy.

Core povenum ( like heart of a cow).

Dilated Cardiomyopathy

Morphology (Microscopic) Nonspecific Myocyte hypertrophy Interstitial fibrosis Wavy fiber change or myofiber loss Scanty mononuclear inflammatory infiltrate


Highest incidence in middle age blacks 2x more frequent than whites men 3x more frequent than women

symptoms may be gradual in onset acute presentation

misdiagnosed as viral URI in young adults uncommon to find specific myocardial disease on

endomyocardial biopsy

History and Physical Examination

Symptoms of heart failure pulmonary congestion (left HF)

dyspnea (rest, exertional, nocturnal), orthpnea systemic congestion (right HF)

edema, nausea, abdominal pain, nocturia low cardiac output

fatigue and weakness hypotension, tachycardia, tachypnea, JVD

Cardiac Imaging Chest radiogram Electrocardiogram 24-hour ambulatory ECG (Holter)

lightheadedness, palpitation, syncope Two-dimensional echocardiogram Radionuclide ventriculography Cardiac MRI Cardiac catheterization

age >40, ischemic history, high risk profile, abnormal ECG

Management of DCM

Limit activity based on functional status salt restriction of a 2-g Na+ (5g NaCl) diet fluid restriction for significant low Na+ initiate medical therapy

ACE inhibitors, diuretics digoxin, carvedilol hydralazine / nitrate combination

Management of DCM

consider adding ß-blocking agents if symptoms persists

anticoagulation for EF <30%, history of thromboemoli, presence of mural thrombi

intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors

cardiac transplantation

HYPERTROPHIC CARDIOMYOPATHY

Hypertrophic Cardiomyopathy

First described by the French and Germans around 1900

uncommon with occurrence of 0.02 to 0.2% a hypertrophic and non-dilated left ventricle in

the absence of another disease small LV cavity, asymmetrical septal

hypertrophy (ASH), systolic anterior motion of the mitral valve leaflet (SAM)


Autosomal dominant

Abnormalities in the Beta myosin heavy chain.

Predominant Features

Asymmetric hypertrophy of the LV

Normal ventricular systolic function

Impaired LV diastolic function

+/- Subaortic dynamic obstruction

Hypertrophic Cardiomyopathies

CAUSES OF CARDIAC HYPERTROPHY

1. Pressure overload

hypertension, valvular stenosis (aortic stenosis)

2. Volume overload

valvular insufficiency

3. Myocardial injury

M.I., myocarditis, cardiomyopathy MI & myocarditis: damage to a part of myocardium, the other parts will compensate by

under going hypertrophy.

Septum.

Hypertrophy in all the layers in every whereConcentric hypertrophy. •Commonest cause is long standing systemic hypertension. Pressure over-load in the aortic valve stenosis or regurgitation that will end up in dilatation & heart failure.

Morphology (Microscopic) Haphazard arrangement of

hypertrophied myocytes, surrounded by loose, basophilic ground substance

Myocardial fibrosis may occur late


Very thick fibers.

Type Feature

I Hypertrophy confined to Anterior segment of the IVS

II Hypertrophy confined to Anterior and Inferior segments of the IVS

III Extensive hypertrophy of LV with sparing of the basal segment of the posterior wall.

IV Apical hypertrophy



Familial HCM

First reported by Seidman et al in 1989 occurs as autosomal dominant in 50% 5 different genes on at least 4 chromosome

with over 3 dozen mutations chromosome 14 (myosin) chromosome 1 (troponin T) chromosome 15 (tropomyosin) chromosome 11 (?)

Pathophysiology

Systole dynamic outflow tract gradient

Diastole impaired diastolic filling, filling pressure

Myocardial ischemia muscle mass, filling pressure, O2 demand vasodilator reserve, capillary density abnormal intramural coronary arteries systolic compression of arteries

Clinical Manifestation

Asymptomatic, echocardiographic finding Symptomatic

dyspnea in 90% angina pectoris in 75% fatigue, pre-syncope, syncope

risk of SCD in children and adolescents palpitation, PND, CHF, dizziness less frequent

Natural History

annual mortality 3% in referral centersprobably closer to 1% for all patients

risk of SCD higher in children may be as high as 6% per yearmajority have progressive hypertrophy

clinical deterioration usually is slow progression to DCM occurs in 10-15%

Risk Factors for SCD

Young age (<30 years) “Malignant” family history of sudden death Gene mutations prone to SCD (ex. Arg403Gln) Aborted sudden cardiac death Sustained VT or SVT Recurrent syncope in the young Nonsustained VT (Holter Monitoring) Brady arrhythmias (occult conduction disease)

Br Heart J 1994; 72:S13

Recommendations for Athletic Activity

Avoid most competitive sports (whether or not symptoms and/or outflow gradient are present)

Recommendations for Athletic Activity

Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent ventricular tachycardia on Holter monitoring family history of sudden death due to HCM history of syncope or episode of impaired consciousness severe hemdynamic abnormalities, gradient 50 mmHg exercise induced hypotension moderate or sever mitral regurgitation enlarged left atrium (50 mm) paroxysmal atrial fibrillation abnormal myocardial perfusion

Management

beta-adrenergic blockers calcium antagonist disopyramide amiodarone, sotolol DDD pacing myotomy-myectomy plication of the anterior mitral leaflet

HCM vs Aortic Stenosis

HCM Fixed Obstruction

carotid pulse spike and dome parvus et tardus

murmur radiate to carotids valsalva, standing squatting, handgrip passive leg elevation

systolic thrill 4th left interspace 2nd right interspacesystolic click absent present

Other Causes of Hypertrophy

Clinical mimics glycogen storage, infants of diabetic mothers, amyloid

Genetic Noonan’s, Friedreich’s ataxia, Familial restrictive

cardiomyopathy with disarray Exaggerated physiologic response

Afro-Caribbean hypertension, old age hypertrophy, athlete’s heart

HCM vs Athlete’s Heart

HCM Athlete+ Unusual pattern of LVH -+ LV cavity <45 mm -- LV cavity >55 mm ++ LA enlargement -+ Bizarre ECG paterns -+ Abnormal LV filling -+ Female gender -- thickness with deconditioning ++ Family history of HCM -

Circulation 1995; 91:1596

Hypertensive HCM of the Elderly

Characteristics modest concentric LV hypertrophy (<22 mm) small LV cavity size associated hypertension ventricular morphology greatly distorted with

reduced outflow tract sigmoid septum and “grandma SAM”

RESTRICTIVE CARDIOMYOPATHY

Restrictive Cardiomyopathies

Hallmark: abnormal diastolic function rigid ventricular wall with impaired ventricular

filling bear some functional resemblance to

constrictive pericarditis importance lies in its differentiation from

operable constrictive pericarditis

Description Primary decrease in ventricular

compliance, resulting in impaired ventricular filling during diastole

Restricting the heart movement. Constrictive / restrictive precarditis precardium is enclosed in

fibrous wall.

Least common type of cardiomyopathy

Restrictive Cardiomyopathy

Causes1. Endomyocardial fibrosis

Most common cause worldwide 10% of cases of childhood heart disease in

tropical regions The heart becomes less compliance due to fibrosis during diastole the heart will

not dilate properly.

2. Infiltrative heart disease (amyloid, HH)Hemosedrin deposition.

3. Idiopathic


Morphology (Gross) Varies depending on cause Atrial dilation (bilateral) Endocardium thickened and opaque Valvular thickening Mural thrombi may be present

Morphology (Microscopic) Dense endocardial fibrosis, extending into subendocardial

myocardium Eosinophils in some cases


Idiopathic restrictive cardiomyopathy:Biatrial dilatation, grossly normal ventricles

Dilated atria.

Classification

Idiopathic Myocardial

1. Noninfiltrative Idiopathic Scleroderma2. Infiltrative Amyloid Sarcoid Gaucher disease Hurler disease

3. Storage Disease Hemochromatosis Fabry disease Glycogen storage

Endomyocardial endomyocardial fibrosis Hyperesinophilic synd Carcinoid metastatic malignancies radiation, anthracycline


Symptoms of right and left heart failure Jugular Venous Pulse

prominent x and y descents Echo-Doppler

abnormal mitral inflow pattern prominent E wave (rapid diastolic filling) reduced deceleration time ( LA pressure)

Constrictive - Restrictive Pattern“Square-Root Sign” or “Dip-and-Plateau”

Restriction vs Constriction

History provides important clues Constrictive pericarditis

history of TB, trauma, pericarditis, sollagen vascular disorders

Restrictive cardiomyopathy amyloidosis, hemochromatosis

Mixed mediastinal radiation, cardiac surgery

Treatment

No satisfactory medical therapy Drug therapy must be used with caution

diuretics for extremely high filling prssures vasodilators may decrease filling pressure ? Calcium channel blockers to improve diastolic

compliance digitalis and other inotropic agents are not

indicated

Cardiac amyloid Three main types

AL (light chains, plasma cell dyscrasias) AA (chronic inflammatory diseases, rare in heart) AS (transthyretin, senile amyloid)

Pathology 10 nm non-branching extracellular fibrils Homogeneous waxy material present in interstitium,

vessels or nodular Congo red positive, apple green birefringence

Clinical Setting Constrictive vs. restrictive disease patient presents with

congested neck veins, synosis & edema. Amyloid causes malignancy called Multiple Myloma.

Deposition of amyloid fibers.

Hemochromatosis

Iron not normally found in heart Pathology

Perinuclear distribution Epicardial portions preferentially involved C282Y or H63D mutation (chromosome 6) HLA-associated TfR gene

Clinical Setting Dilated or restrictive cardiomyopathy Hemochromatosis: iron deposition in all orgens.

Iron deposition.

Drug-induced cardiomyopathy

Adriamycin commonest drug.

Other drugs (chloroquine, amiodarone)

These drugs treat cancer & are cardiotoxic.

Arrhythmogenic RV Dysplasia

Uncommon, familial form that affects young adults causing sudden death

RV is markedly thinned & replaced by fat CPC required (fat may be seen in normal)

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Miocardite Si CMP

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