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Myocarditis
ā¢Definition:
ā¢ Myocarditis is an inflammatory
disease of the heart muscle
(myocardium) that can result from
a variety of causes. While most
cases are produced by a viral
infection, an inflammation of the
heart muscle may also be
instigated by toxins, drugs, and
hypersensitive immune reactions.
ā¢ Myocarditis is a rare but
serious condition that affects
both males and females of
any age.
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Historical Aspects
ā¢ 1837 - J.F. Soberenheim first used the term āmyocarditisā
ā¢ 1897 ā M.O. Abramov described pathological changes in
myocarditis
ā¢ 1990 - F. Fidler described primary idiopathic myocarditis
ā¢ In 1920s - there was overdiagnosis of myocarditis
ā¢ In the 1920-50s the term Ā«myocarditisĀ» replaced the term
Ā«degeneration of the myocardiumĀ»
ā¢ 1949 - I. Gore., O. Sapfir described viral and rikettsial
myocarditis
ā¢ In 1980s - there was an introduction of transvenous
myocardial biopsy into clinical practice
1992 M Paleev
ā¢ Myocarditis - is a heart muscle lesion, mainly of
inflammatory nature caused by influence of infection,
parasitic or protozoan invasion, chemical or physical
agents, or when develops in allergic or autoimmune
diseases. ...
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Sponsored
Medical Lecture Notes ā All Subjects
USMLE Exam (America) ā Practice
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ŠŠŠŠ”ŠŠ¤ŠŠŠŠ¦ŠŠÆ ŠŠŠŠŠŠ ŠŠŠ¢ŠŠ
Etiology, course
(stage)
Prevale
nce
Severity
Clinical
variant
Heart
failure
I Acute (up to 3 months),
subacute (to 6mis):
a) with established etiology
(infectious, bacterial, viral,
parasitic etc.)
b) unspecified.
II Chronic (over 6 months):
primary chronic, recurrent,
latent
III Miocardiofibrosis
(myocardyosklerosis)
Focal
Diffuse
Mild
Moderate
Severity
Cardiac
rhythm and
conduction
Violation of
repolarizati
on
Cardialgia
stage
variant
dysfunc
tion
Functio
nal
class
ā¢
Acute viral (postinfluenzal) myocarditis, focal form, mild
disease course, atrioventricular block first degree, HF I,
with normal systolic function FC I
ā¢
Chronic unspecified etiology of myocarditis, diffuse form,
Classification
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Important Causes of Myocarditis
ā¢Infection
āViral
āBacterial, rickettsial, spirochetal
āProtozoal, Metazoal
āFungal
ā¢Toxic
āanthracyclines, catecholamines,
Interleukin-2, alpha 2 interferon
ā¢Hypersensitivity
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Viral Infection
ā¢ Coxsackie (A, B)
ā¢ Echo
ā¢ Influenza (A, B)
ā¢ Polio
ā¢ Herpes simplex
ā¢ Varicella-zoster
ā¢ Epstein-Barr
ā¢ Cytomegalovirus
ā¢ Mumps
ā¢ Rubella
ā¢ Rubeola
ā¢ Vaccinia
ā¢ Coronavirus
ā¢ Hepatitis B, C
ā¢ Arbovirus
ā¢ Junin virus
ā¢ Human immunodeficiency
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Bacterial, rickettsial, spirochetal
ā¢ Corynebacterium diphtheriae
ā¢ salmonella typhi
ā¢ Beta-hemolytic streptococci
ā¢ Neisseria
ā¢ Legionella pneumophila
ā¢ Listeria monocytogenes
ā¢ Camphylobacter jejuni
ā¢ Coxiella burnetii
ā¢ Chlamydia trachomatis
ā¢ Mycoplasma pneumoniae
ā¢ Chlamydia psittaci
(psittacosis)
ā¢ Rickettsia (Rocky
Mountain spotted fever)
ā¢ Borrelia burgdorferi
(Lyme disease)
ā¢ Mycobacterium
tuberculosis
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Protozoal, Metazoal, Fungal
Protozoal
ā¢ Trypnosoma cruzi
(Chagasā disease)
ā¢ Toxoplasma gondi
Metazoal
ā¢ Trichinosis
ā¢ Ehinococcosis
Fungal
ā¢ Aspergillosis
ā¢ Blastomycosis
ā¢ Candidiasis
ā¢ Coccidioidomycosis
ā¢ Cryptococcosis
ā¢ Histoplasmosis
ā¢ Mucormycosis
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Drugs Causing
Hypersensitivity Myocarditis
Antibiotics
amphoericin B
ampicillin
chloramphenicol
penicillin
tetracycline
streptomycin
Sulfonamides
sufadiazine
sufisoxazole
Anticonvulsants
phenindione
phenytoin
carbamazepine
Antitubercuous
isoniazid
paraaminosalicylate
Anti-inflammatory
indomethacin
oxyphenbutazone
phenylbutazone
Diuretics
acetazolamide
chlorthalidone
hydrochlorothiazide
spironolactone
Other
amitriptyline
methyldopa
sulfonylureas
tetanus toxoid
As of 1996, research has shown that illegal drugs and toxic substances
may also produce acute or chronic injury to the myocardium.
These studies also indicate an increase in the incidence of
toxic results from the use of cocaine.
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particles of myocardial inflammation
inflamation
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Clinical Manifestations
ā¢ Most cases of acute myocarditis are clinically
silent
ā¢ 60% of pts had antecedent flulike symptoms
ā¢ Large number identified by heart failure
symptoms
ā¢ 35% of pts with myocarditis and HF have chest
pain
ā¢ May mimic an acute MI with ventricular
dysfunction, ischemic chest pain, ECG
evidence of injury or Q waves
ā¢ Sed rate elevation 60%
ā¢ White count elevation 25%
ā¢ 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
ā¢ Heart specific antibodies are nonspecific for
myocarditis; also found in dilated
cardiomyopathy
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
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Diagnosis
ā¢ Physical data: weakening tones
protodiastolyc gallop, the mumur
is presences, border of the heart
increased .
ā¢ X-ray examination: an increase in
heart size.
ā¢ ECG: depression/elevation of ST
segment, di-phase or inversion of
the T wave, prevalence of QRS
complex and prolongation of QT
interval, bifascicular and
atrioventricular heart blocks
ā¢ Echocardiography:
ā¢ no myocardial hypertrophy (the
heart is enlarged due to the
thickening of ventricular walls)
ā¢ type bi-ventricular systolic and
diastolic dysfunction of the heart,
ā¢ zones of dyskinesia, hypokinesia,
akinesia
ā¢ dilatation of cavities
ā¢ decrease in ejection fraction <40%.
Laboratory criteria: increased levels of Ī±2 and Ī³-
globulin, C-reactive protein, sialic acid, seromucoid,
SLA-O, accelerated ESR, cTn-I, STN-T, AST, etc.
Serological diagnosis: antibodies to Coxsackie virus
group A, B, cytomegalovirus, chlamydia, herpes
simplex type II, trypanosomy, toxoplasma,
mycoplasma, Trichinella;
Antibodies to cardiomyocytes: antibodies in the IgM is
a marker of recent primary infection, antibodies in the
IgG is a marker of a carrier state
Immunological criteria: activation of T-cell markers-CD
25, B-cell marker-CD 23.
Polymerase chain reaction for identification of the
causative agent (DNA, RNA).
Radioisotope scanning with technetium-99
pyrophosphate or gallium-67, indium 111 (damaged
cardiomyocytes actively accumulate isotope).
Endomiocardial biopsy. The Dallas criteria: the
presence of an inflammatory cell infiltrate of the
myocardium with necrosis or cardiomyocyte
degeneration.
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Electrocadiogram
ā¢ sinus tachycardia is most
common
ā¢ diffuse ST-T wave changes
ā¢ myocardial infarction pattern
ā¢ conduction delay
ā¢ complete heart block causing
Stokes-Adams attacks
(particularly in Japan), but rarely
require a permanent pacer
ā¢ supraventricular and ventricular
arrhythmias
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Myocarditis ECG
H9925 9-8-98
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Definition of cardiothorax index( CTI)
Š°
Š²Ń
NORMAL50%
When LV systolic
dysfunction CTI>
50%
In diastolic
dysfunction CTI ā¤
50%
Chest
width=c
Heart width =
Š°+Š²,CTI = Ń 100%
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Prognosis & X- ray
before treatment after treatment
CTI =75% CTI=50%
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Fiedler (1899) Myocarditis -
50% of fatal
ā¢ Severe acute myocarditis,
which quickly leads to
irreversible congestive heart
failure
ā¢ cardiomegaly with mitral
and tricuspid regurgitation,
protodiastolic gallop
ā¢ paroxysms of severe
arrhythmias
ā¢ refractory to treatment
failure setseva
ā¢ thromboembolic
complications
ā¢ sudden cardiac death
ā¢ Rhythm disturbances of the
heart
ā¢ Rapid heartbeat (
Ventricular tachycardia)
ā¢ Left or right ventricular
enlargement
ā¢ Shortness of breath
(Dyspnea)
ā¢ Pulmonary edema (the
accumulation of fluid in the
lungs due to left-sided
heart failure)
ā¢ Swollen legs.
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Treatment & Prognosis
ā¢ Antibiotics if specific agent is
identified
ā¢ Standard HF therapy
ā¢ Arrhythmia suppression
ā¢ Limited exercise role during
recovery
ā¢ steroids are controversial
ā¢ Fulminant myocarditis need
aggressive short term support
from intra-aortic balloon pumps
ā¢ If severe rhythm
disturbances are involved,
cardiac assist devices, an
"artificial heart," or
heart transplantation may
be the only option for
complete recovery.
ā¢ Prognosis
ā¢ Fulminant myocarditis pt may
present with cardiogenic shock
ā¢ Ventricles are thickened, usually
not dilated
ā¢ Subacute pt have dilated
cardiomyopathy
ā¢ Chronic pt may have mildly dilated
LV and more of a restrictive
cardiomyopathy
ā¢ Inflammation of the
myocardium may also cause
acute pericarditis
(inflammation of the outer
lining of the heart).
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New European guidelines on treatment of myocarditis
Empirical antibiotic therapy / in the first three days of the transition to
the following strategies:
1.Switching from intravenous to oral therapy ā āstep downā, the same
antibiotic which was used intravenously is used orally.
2. Sequential treatment strategy: penicillin G or amoxyclav, augmentin (4
million units a day intramuscular injection - 10 days), ceftriaxone (2 g a day
intramuscular injection - 10 days), fluconazole (200 mg a day - 5 days),
Acyclovir (1 g a day orally - 10 days).
Pathogenetic therapy: prednisone (25 mg / d, then 60 mg / d -
30 days) and diclofenac (100 mg / d - 8 weeks).
Symptomatic therapy of heart failure:
ā¢ ACE inhibitors (captopril, ramipril, lisinopril, trandalopryl,
enalapril)
ā¢ Ī-blockers: carvedilol (dose titration with 6 mg/d).
ā¢ Diuretics: furosymid, veroshpiron, tryfaz
ā¢ Cardiac glycosides: digoxin 0.25 mg/d- intake twice a day
ā¢ Non-glycoside inotropic agents: LEVOSYMENDAM,
NESYRETYD.
Symptomatic therapy of heart failure:
ā¢ ACE inhibitors (captopril, ramipril, lisinopril, trandalopryl,
enalapril)
ā¢ Ī-blockers: carvedilol (dose titration with 6 mg/d).
ā¢ Diuretics: furosymid, veroshpiron, tryfaz
ā¢ Cardiac glycosides: digoxin 0.25 mg/d- intake twice a day
ā¢ Non-glycoside inotropic agents: LEVOSYMENDAM,
NESYRETYD.
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Nonsteroidal anti-inflammatory drugs
(indicated for medium severity, and in
case of high severity should be prescribed
with caution)
HSI COX-2 inhibitors:
celecoxib (Celebrex) - 200 mg 2 times a day orally within 4-6 weeks
Selective COX-2 inhibitors:
mesulid - 100 mg per day orally within 4-6 weeks
Movalis - 7.5 mg per day orally within 4-6 weeks
NON Selective COX:
diclofenac sodium (Voltaren, naklofen, ortofen) - 75 mg (3 mL)
intramuscularly 10 days + 100 mg per os, per rectum 6 weeks
Disease modifying therapy
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Disease modifying therapy - Glucocorticoids
ā¢ Inhibit the synthesis of COX-1 and COX-2, and
lipoxygenase, this way they inhibit the synthesis of
leukotrienes (LT-B4, LT-C4), that is , they influence the
cellular component of inflammation.
ā¢ Stimulate the synthesis of inhibitor of phospholipase
A2, thus block the synthesis of all metabolites of arachidonic
acid.
ā¢ Reduce the adhesion of leucocytes to the endothelium,
reduce the production of free radicals, redistribute T
lymphocytes and monocytes from the blood circulation in the
blood pool.
ā¢ Slow down the migration of neutrophils in the area of
inflammation, inhibit the formation of blood pressure.
ā¢ Slow down the proliferation of fibroblasts and the
influence of the histamine, serotonin, kinins on them,
influence the genetic apparatus of the target cells.
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Variations of CSC prescriptions
ā¢ Short-acting (24-36 hours): cortisone,
hydrocortisone, prednisolone,
methylprednisolone;
ā¢ average duration (48 hours): triamcinolone,
polkortolon, kenoloh;
long-term effects (over 48 hours):
betamethasone, parametazon,
dexamethasone.
Prednisolon. Synthetic derivatives of
hydrocortisone. Well absorbed in the
gastrointestinal tract, peak concentrations
within 1.5-2 h. In tablets of 5 and 10 mg in
capsules of 25 and 30 mg.
Begin treatment with daily doses of 30 or 60
mg (rarely 80 or 120 mg).
It is divided into 3 or 2 doses or 1 admission.
Best for long-term use is methylprednisolone.
4 mg methylprednisolone = 5 mg prednisolone.
ā¢ Daily dose of 16-48 mg.
ā¢ Variations appointment
GCS:
ā¢ 1. Pulse therapy - extremely high
single dose or within 3 days;
methylprednisolone 1000 mg in 500
ml 5% glucose solution.
ā¢ Sometimes 500 mg of
cyclophosphamide.
ā¢ 2. Limited scheme - the average
dose is prescribed for several
months.
ā¢ 3. Prolonged purpose small
(minimal) doses.
ā¢ 4. Circadian therapy - the total daily
dose in a single administration.
ā¢ 5. Alternating therapy - 2 dose every
other day.
ā¢ 6. Intermittent therapy - a patient
intakes prednisolone for 3-4 days
which follow 3-4-day break. On non-
steroid days etymizol is prescribed.
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Harris Rule:
Bioequivalent to the 5 mg prednisone are:
ā¢ 25 mg - hydrocortisone;
ā¢ 4 mg - methylprednisolone;
ā¢ 4 mg - triamcinolone;
ā¢ 0.75 mg - betamethasone;
ā¢ 0.75 mg - dexamethasone.
Bioavailability of parenteral corticosteroids 50%
lower than oral.
Cancellation should be gradual ā prednisolone dose
should be reduced gradually every 2 weeks (less often-
every week) by 2.5 mg (1/2 a pill)
Minimumal dose - 5-7,5 mg / day.
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Symptomatic treatment
European
Society of
Cardiology
Patients with thromboembolic syndrome are indecated low molecular weight
heparins:
KLEKSAN (enoxaparin) - 1 mg / kg body weight paraumbilically x 12 hours, 6
Ā± 2 days, in combination with aspirin 160-325 mg x 1 per day or PLAVYKS
(clopidogrel) - 75 mg / day.
Patients with thromboembolic syndrome are indecated low molecular weight
heparins:
KLEKSAN (enoxaparin) - 1 mg / kg body weight paraumbilically x 12 hours, 6
Ā± 2 days, in combination with aspirin 160-325 mg x 1 per day or PLAVYKS
(clopidogrel) - 75 mg / day.
The following antihistamines are indicated:
dimedrol, suprastin, tavegil, fenkarol,
feksofenadine.
METABOLIC THERAPY
Preductal MR 35 mg x 2 g / d
CORVITIN 500mh/day, ā 10 IV
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Prevention
Although myocarditis is an unpredictable disease, the following
measures may help prevent its onset.
ā¢ Take extra measures to avoid infections, and obtain
appropriate treatment for infections.
ā¢ Limit alcohol consumption to no more than one or two
drinks a day, if any.
ā¢ Maintain current immunizations against diphtheria,
tetanus, measles, rubella, and polio.
ā¢ Avoid anything that may cause the abnormal heart to
work too hard, including salt and vigorous exercise.
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Cardiomyopathy.
People with cardiomyopathy
are often at risk of dangerous
forms of irregular heart beat
and sudden cardiac death.
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29 of 48
Cardiomyopathy. Definition
ā¢ The term cardiomyopathy is purely descriptive, meaning
disease of the heart muscle
ā¢ 2009 AHA defined cardiomyopathies as
ā¢ āa heterogeneous group of diseases of the myocardium
associated with mechanical &/or electrical dysfunction that
usually (but not invariably) exhibit inappropriate ventricular
hypertrophy or dilatation and are due to a variety of causes that
frequently are genetic.
ā¢ Cardiomyopathyes either are confined to the heart or are a
part of generalized systemic disorders, often leading to
cardiovascular death or progressive heart failure-related
disability.ā
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Types of cardiomyopathy
ā¢ Primary/intrinsic cardiomyopathies
ā Genetic
ā¢ Hypertrophic cardiomyopathy
(HCM or HOCM)
ā¢ Arrhythmogenic right ventricular cardiomyopathy
(ARVC)
ā¢ Isolated ventricular non-compaction
ā¢ Mitochondrial myopathy
ā Mixed
ā¢ Dilated cardiomyopathy
(DCM)
ā¢ Restrictive cardiomyopathy
(RCM)
ā Acquired
ā¢ Cardiomyopathy
ā¢ Loeffler endocarditis
ā¢ Secondary/extrinsic
cardiomyopathies
ā Metabolic/storage
ā¢ amyloidosis
ā¢ hemochromatosis
ā Inflammatory
ā¢ Chagas disease
ā Endocrine
ā¢ diabetic cardiomyopathy
ā¢ hyperthyroidism
ā¢ acromegaly
ā Toxicity
ā¢ chemotherapy
ā¢ Alcoholic cardiomyopathy
ā Neuromuscular
ā¢ muscular dystrophy
ā Nutritional diseases
ā¢ Obesity-associated
cardiomyopathy[7]
ā Other
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31 of 48
Functional Classification
ā¢ Dilated
ā Ventricular dilation, hypokinetic left
ventricle, and systolic dysfunction
ā¢ Hypertrophic
ā Inappropriate myocardial hypertrophy, with
or without left ventricular obstruction
ā¢ Restrictive (Infiltrative)
ā Abnormal ventricular filling with diastolic
dysfunction
ā¢ Arrhthymogenic Right Ventricular
ā Fibroadipose replacement of right ventricle
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32 of 48
CardiomyopathyCardiomyopathy
Nursing Review, 2001
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33 of 48
Dilated Cardiomyopathy
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34 of 48
Dilated Cardiomyopathy - Definition
ā¢ Primary (idiopathic) is a disease of
unknown etiology that principally
affects the myocardium leading to LV
dilation and systolic dysfunction
ā¢ Secondary causes include ischemia,
alcoholic, peripartum, post-infectious,
viral
ā¢ Most common of the cardiomyopathies
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35 of 48
Schematic of Dilated CM
Medslides.com
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36 of 48
Clinical Manifestations
ā¢ Highest incidence in middle
age
ā Blacks 2x more frequent
than whites
ā Men 3x more frequent
than women
ā¢ Symptoms may be gradual in
onset
ā¢ Acute presentation
ā Misdiagnosed as viral in
young adults
ā Uncommon to find
specific myocardial
disease on
endomyocardial biopsy
ā¢ Symptoms/Signs of heart
failure
ā Pulmonary congestion
(left heart failure)
dyspnea (rest, exertional,
nocturnal), orthopnea
ā Systemic congestion
(right heart failure)
edema, nausea,
abdominal pain
ā Low cardiac output
ā Hypotension, tachycardia,
tachypnea
ā Fatigue and weakness
ā¢ Arrhythmia
ā Atrial fibrillation,
conduction delays,
sudden death
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37 of 48
Diagnostics
ā¢ RTG (enlarged heart, CHF)
ā¢ Electrocardiogram (tachycardia, A-V block) Flat or
inverted T waves are most common, often with low-
voltage QRS complexes. Intraventricular conduction
defects and bundle branch block, especially left
bundle branch block
ā¢ 24-hour Holter monitor
ā if lightheadedness, palpitation, syncope
ā¢ Echocardiogram (left ventricular dilation, global hypokinesis,
low EF< 45%, end-diastolic volume> 165 cm3, end-diastolic
size> 6.5 cm)
ā¢ Myocardial biopsy, rare
ā¢ Cardiac catheterization if age >40, ischemic history,
high risk profile, abnormal ECG
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38 of 48
DCM - Treatment
ā¢ Limit activity based on
functional status
ā¢ Salt restriction
ā¢ Fluid restriction
ā¢ Initiate medical therapy
ā ACE inhibitors, diuretics
ā Digoxin
ā Hydralazine/nitrate
combination
ā Anticoagulation (EF
<30%, embolic events)
ā Implantable
defibrillators
ā¢ Cardiac transplantation
ā This disorder is the most
common indication for
cardiac transplantation
ā Survival after transplant
is
ā¢ 80% one year
ā¢ 70% 5 years
Left Ventricular Reduction
Procedures
ā LV-reshaping
Treatment of cardiomyopathy
(and other heart diseases)
using alternative methods
such as stem cell therapy
is commercially available
but is not supported by
convincing evidence.
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Hypertrophic Cardiomyopathy (HCM)
ļClinical presentation from infancy to old age
ļVariable clinical course 25 % of cohort achieve normal longevity
ļAnnual mortality 3% in referral centers
ļCourse may be punctuated by adverse clinical events: sudden cardiac death,
embolic stroke, and consequences of heart failure
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40 of 48
Hypertrophic Cardiomyopathy (HCM)
ā¢ Defined
ā First described in 1869 and accepted as a
clinical entity in the 1950ās
ā Prevalence 2:1000
ā Genetic disease characterized by hypertrophy of the left
ventricle with marked variable clinical manifestations
morphologic and hemodynamic abnormalities
ā Small LV cavity, septal hypertrophy which can be
asymmetric (ASH), systolic anterior motion of the mitral
valve leaflet (SAM), +/- obstruction of left ventricular
outflow with low stroke volume, but elevated EF
Source: UpToDate (www.uptodate.com) āNatural history of hypertrophic
cardiomyopathy" Perry M Elliott, MD, William J McKenna, MD, last updated 9/18/07
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42 of 48
8 - 10%
15 - 20%
Variants of HCM
65 - 70%
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43 of 48
HCM ā ASH Without Obstruction
ā¢ The major abnormality of the heart in
HCM is an excessive thickening of the
muscle. Thickening usually begins
during early adolescence and stops
when growth has finished. It is
uncommon for thickening to progress
after this age
ā¢ The left ventricle is almost always
affected, and in some patients the
muscle of the right ventricle also
thickens
ā¢ Hypertrophy is usually greatest in the
septum. The muscle thickening in this
region may be sufficient to narrow the
outflow tract. This thickening is
associated with obstruction to the
flow of blood out of the heart into the
aorta
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44 of 48
HCM ā ASH With Obstruction
ā¢ Asymmetric septal
hypertrophy with
obstruction to the
outflow of blood from
the heart may occur.
The mitral valve
touches the septum,
blocking the outflow
tract. Some blood is
leaking back through
the mitral valve
causing mitral
regurgitation
www.kanter.com/hcm
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45 of 48
Pathophysiology of HCM
Left Ventricular Outflow Tract Gradient
ā¢ Dynamic LV outflow tract
obstruction
ā Outflow tract gradient (>30
mm Hg), considered
severe if >50 mm Hg
(occurs in 25-30% of cases
leading to name
hypertrophic obstructive
cardiomyopathy)
ā¢ Diastolic dysfunction
ā Impaired diastolic filling, ā
filling pressure
ā¢ Myocardial ischemia
ā¢ Mitral regurgitation
ā¢ Arrhythmias
ā¢ Approximately 25% of patients with
HCM have a dynamic systolic
pressure gradient in the left
ventricular outflow tract caused by
contact between the mitral valve
leaflet(s) and the interventricular
septum under resting conditions
ā¢ Outflow tract gradient in excess of
30 mmHg is an important cause of
symptoms
ā¢ LVH usually develops between 5-15
years of age in HCM
ā¢ A normal ECHO in a young child
does not exclude the diagnosis
ā¢ Serial ECHOs are recommended up
to the age of 20 yr where there is a
family history of HCM
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46 of 48
Clinical Manifestations
ā¢ Asymptomatic
ā Echocardiographic
finding only
ā¢ Symptomatic
ā Dyspnea in 90%
ā Angina pectoris in 75%
ā Fatigue, pre-syncope,
syncope
ā Palpitation, CHF,
dizziness
ā Atrial fibrillation,
thromboembolism
ECG
ā¢ Abnormal in 85-90% of
cases
ā¢ LVH, Strain pattern
ā¢ Abnormal ST-Tās, giant T
wave inversions
ā¢ Abnormal Qās,
ā¢ Bundle Branch Block
ā¢ Left atrial enlargment
ā¢ Ventricular arrhthymias
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47 of 48
Left Ventricular Outflow Tract Gradient
ā¢ Gradient greater than 50
mmHg, outflow tract
gradient can cause
dyspnea, chest pain,
syncope, and predisposes
to the development of
atrial arrhythmias -
independent predictor of
disease progression and
adverse outcome,
including sudden death
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Echocardiogram
ā¢ Left ventricular
hypertrophy >1.3 cm
(usually >1.5 cm)
ā¢ Septal to posterior
wall ratio >1.3:1
ā¢ Mitral regurgitation
ā¢ Systolic anterior
motion of the mitral
valve (SAM)
ā¢ Premature
midsystolic closure
of the aortic valve
ā¢ Asymmetric septal
hypertrophy (ASH)
ā¢ Diastolic dysfunction
ā¢ Left ventricular
outflow tract
obstruction (LVOT)
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Natural History & Clinical Course
ā¢ Risk of SCD higher in children, may be
as high as 6% per year, majority have
progressive hypertrophy
ā¢ Accounts for 36% of deaths in athletes
<35 years
ā¢ Clinical deterioration usually is slow
ā¢ Poor prognosis in males, young age of
onset, family Hx of SCD, Hx of
syncope, exercise induced
hypotension (worst)
Risk Factors For SCD
ā¢ Young age (<35 years)
ā¢ āMalignantā family history of sudden
death
ā¢ Aborted sudden cardiac deat
ā¢ Atrial fibrillation
ā¢ Dilated left ventricle
ā¢ NYHA classes III or IV
Risk Factors For SCD
ā¢ Syncope
ā¢ Severe hypertrophy (>3.0
cm)
ā¢ Abnormal BP response to
exercise
ā¢ Coronary artery disease
ā¢ Strenuous exercise or work
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Recommendations for Athletic Activity
ā¢ Low-risk older patients (>30 years) may participate in
athletic activity if all of the following are absent:
ā Ventricular tachycardia on Holter monitoring
ā Family history of sudden death due to HCM
ā History of syncope
ā Severe hemodynamic abnormalities, gradient ā„50
mmHg
ā Exercise induced hypotension
ā Moderate or severe mitral regurgitation
ā Enlarged left atrium (ā„5.0 cm)
ā Paroxysmal atrial fibrillation
ā Abnormal myocardial perfusion
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HCM vs. Athletic Heart
HCM
ā¢ Can be
asymmetric
ā¢ Wall thickness: >
15 mm
ā¢ LA: > 40
ā¢ Diastolic
function: always
abnormal
ā¢ wall thickness
average in
Athletic heart
ā¢ Concentric & regresses
ā¢ < 15 mm
ā¢ < 40 mm
ā¢ Normal
ā¢ Occurs in about 2% of elite althetes ā
typical sports, rowing, cycling, canoeing
ā¢ Former athletes & weekend warriors do
NOT develop athletic heart
ā¢ Elite female athletes do NOT develop
athletic heartwall thickness average in
HCM is 2.2 cm
ā¢ - in athletic heart, a trial of deconditi
oning for 3 months leads to average
0.3 cm reduction in wall thickness
(0.2-0.5 cm)
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Hypertensive HCM of the Elderly
ā¢Characteristics
ā Small LV cavity size
ā Associated hypertension
ā Ventricular morphology greatly distorted with
reduced outflow tract
ā Sigmoid septum
ā Modest concentric LV hypertrophy (<22 mm)
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Management of HCM
ā¢ Beta-adrenergic blockers (atenolol, toprol,
sotalol, tenormin etc)
ā¢ Calcium channel blockers (norvasc, cardizem,
etc)
ā¢ Anti-arrhythmics ā Amiodarone.etc.
ā¢ Pacemakers (ICD)
ā¢ Myomectomy (resection of septum)
ā¢ Transplantation
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Restrictive (Obliterative
cardiomyopathy) Cardiomyopathy
ā¢ Hallmark: abnormal diastolic
function
ā¢ Rigid ventricular wall with
impaired ventricular filling
ā¢ Bear some functional
resemblance to constrictive
pericarditis
ā¢ Importance lies in its
differentiation from operable
constrictive pericarditis
ā¢ Much less common then DCM or
HCM outside the tropics, but
frequent cause of death in Africa,
India, South and Central America
and Asia primarily because of the
high incidence of endomyocardial
fibrosis in those regions
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ā¢ Idiopathic
ā¢ Myocardial
ā Noninfiltrative
ā¢ Idopathic
ā¢ Scleroderma
ā Infiltrative
ā¢ Amyloid
ā¢ Sarcoid
ā¢ Gaucher disease
ā Storage Disease
ā¢ Hemochromatosis
ā¢ Fabry disease
ā¢ Glycogen storage
ā¢ Endomyocardial
ā Endomyocardial
fibrosis
ā Hyperesinophilic synd
ā Carcinoid
ā Metastatic
malignancies
ā Radiation,
anthracycline
Classification
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Clinical Manifestations
ā¢ Symptoms of right and left heart
failure
ā¢ Jugular Venous Pulse
ā¢ Echo-Doppler
ā Abnormal mitral inflow pattern
ā Prominent E wave (rapid
diastolic filling)
ā Reduced deceleration time (ā
LA pressure)
ā¢ History can provide
important clues
Constrictive pericarditis
ā¢ History of TB,
trauma,
pericarditis,
collagen vascular
disorders
ā¢ Restrictive
cardiomyopathy
ā¢ Amyloidosis,
hemochromatosis
ā Mixed
ā¢ Mediastinal
radiation, cardiac
surgery
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Treatment
ā¢ No satisfactory medical therapy
ā¢ Drug therapy must be used with caution
ā Diuretics for extremely high filling pressures
ā Vasodilators may decrease filling pressure
ā Calcium channel blockers to improve diastolic
compliance
ā Digitalis and other inotropic agents are not indicated
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Arrhythmogenic
Right Ventricular
Cardiomyopathy
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Arrhythmogenic RV Cardiomyopathy
ā¢ Characterized by fibroadipose replacement
of segments of the free wall of the right
ventricle
ā¢ Familial and progressive
ā¢ Predominately found in young adults
ā¢ Cause of young adult sudden death
ā¢ ICD implantation in ALL patients who are
symptomatic with arrhythmias
ā¢ ICD implantation vs anti-arrhythmic meds in
asymptomatic patients
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Unfavorable Cases of Cardiomyopathy
Young age (<30 years)
Family history of sudden death
Aborted sudden death
History of heart block
Syncopal attacks
Pacer and/or implanted defibrillator
Prohibited from participating in any exercise
Enlarged heart (CT >55%)
EF <40% and/or generalized mod/severe hypokinesis
CHF
Substantial hypertrophy (> 2cm)
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Thank you, questions
Editor's Notes
--diagnosis of DCM requires evidence of dilatation and impaired contraction of the LV or both ventricles (LVEF of 40% or less or fractional shortening less then 25%
--stress induced cardiomyopathy ā uncommon, also called transient LV apical ballooning,, takotsubo cardiomyopathy and broken heart syndrome ā typically preciptiated by intense psychologic stress and primarily occurs in postmenopausal women, complete recovery normally 1 ā 4 weeks
-- wall thickness average in HCM is 2.2 cm
--in athletic heart, a trial of deconditioning for 3 months leads to average 0.3 cm reduction in wall thickness (0.2-0.5 cm)