CVS K17 Cardiomyopathies
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Transcript of CVS K17 Cardiomyopathies
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Cardiomyopathies
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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”
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WHO Classification
• n!no"n cause
#primary$
– Dilated
– Hypertrophic – %estrictive
– unclassified
• &pecific heart muscle
disease #secondary$
– 'nfective
– (etabolic – &ystemic disease
– Heredofamilial
– &ensitivity
– )o*ic
Br Heart J 1980; 44:672-673
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+unctional Classification
• Dilatated #congestive, DC(, 'DC$
– ventricular enlargement and syst dysfunction
•Hypertrophic #'H&&, HC(, HOC($ – inappropriate myocardial hypertrophy
in the absence of H) or aortic stenosis
• %estrictive #infiltrative$
– abnormal filling and diastolic function
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'diopathic Dilated Cardiomyopathy
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'DC - Definition
• a disease of un!no"n etiology that
principally affects the myocardium
• ./ dilatation and systolic dysfunction
• pathology
– increased heart si0e and "eight
– ventricular dilatation, normal "all thic!ness
– heart dysfunction out of portion to fibrosis
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'ncidence and 1rognosis
• 2-34 cases per 344,444
• 54,444 ne" cases per year in the 6&6A6
• death from progressive pump failure
3-year 5785-year 27-948
7-year 94-:48
• stabili0ation observed in 54-748 of patient
• complete recovery is rare
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'ncidence < Clinical (anifestations
• Highest incidence in middle age
– blac!s 5* more fre=uent than "hites
– men 2* more fre=uent than "omen
• symptoms may be gradual in onset• acute presentation
– misdiagnosed as viral %' in young adults
–uncommon to find specific myocardial diseaseon endomyocardial biopsy
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History and 1hysical ;*amination
• &ymptoms of heart failure
– pulmonary congestion #left H+$
dyspnea (rest, exertina!, n"t#rna!$, rt%pnea
– systemic congestion #right H+$ede&a, na#sea, a'd&ina! pain, n"t#ria
– lo" cardiac output
ati)#e and *ea+ness
• hypotension, tachycardia, tachypnea, >/D
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Cardiac 'maging
• Chest radiogram
• ;lectrocardiogram
• 59-hour ambulatory ;C? #Holter$
– !i)%t%eadedness, pa!pitatin, syn"pe• )"o-dimensional echocardiogram
• %adionuclide ventriculography
• Cardiac catheteri0ation
– a)e 40, is"%e&i" %istry, %i)% ris+ pri!e, a'nr&a! ./
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(anagement of DC(
• .imit activity based on functional status
• salt restriction of a 5-g aB #7g aCl$ diet
• fluid restriction for significant lo" aB
• initiate medical therapy
– AC; inhibitors, diuretics
– digo*in, carvedilol
– hydrala0ine nitrate combination
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(anagement of DC(
• consider adding -bloc!ing agents if
symptoms persists
• anticoagulation for ;+ E248, history of
thromboemoli, presence of mural thrombi• intravenous dopamine, dobutamine
andor phosphodiesterase inhibitors
• cardiac transplantation
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Hypertrophic Cardiomyopathy
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Hypertrophic Cardiomyopathy
• +irst described by the +rench and ?ermans
around 3F44
• uncommon "ith occurrence of 4645 to 4658
• a hypertrophied and non-dilated left ventricle inthe absence of another disease
• small ./ cavity, asymmetrical septal
hypertrophy #A&H$, systolic anterior
motion of the mitral valve leaflet #&A($
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6 3
10
***+anter"&%"&
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+amilial HC(
• +irst reported by &eidman et al in 3F:F
• occurs as autosomal dominant in 748
• 7 different genes on at least 9 chromosome
"ith over 2 do0en mutations – chromosome 39 #myosin$
– chromosome 3 #troponin )$
– chromosome 37 #tropomyosin$
– chromosome 33 #G$
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Clinical (anifestation
• Asymptomatic, echocardiographic finding
• &ymptomatic
– dyspnea in F48
– angina pectoris in 78
– fatigue, pre-syncope, syncope
ris! of &CD in children and adolescents
– palpitation, 1D, CH+, di00iness less fre=uent
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'ncrease in ?radient and (urmur
Contractility 1reload Afterloadvalsalva #strain$ ---
standing --- --
poste*trasystole --
isoproterenol
digitalis --
amyl nitrite --
nitroglycerine ---
e*ercise
tachycardia --
hypovolemia
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Decrease in ?radient and (urmur
Contractility 1reload Afterload(ueller meneuver ---
valsalva #overshoot$ ---
s=uatting ---
passive leg elevation --- --
phenylephrine --- --
beta-bloc!er --
general anesthesia -- --
isometric grip --- --
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atural History
• annual mortality 28 in referral centers
probably closer to 38 for all patients
• ris! of &CD higher in children
may be as high as I8 per year
maority have progressive hypertrophy
• clinical deterioration usually is slo"
• progression to DC( occurs in 34-378
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%is! +actors for &CD
• Joung age #E24 years$
• “(alignant” family history of sudden death
• ?ene mutations prone to &CD #e*6 Arg942?ln$
• Aborted sudden cardiac death• &ustained /) or &/)
• %ecurrent syncope in the young
• onsustained /) #Holter (onitoring$
• @rady arrhythmias #occult conduction disease$
Br Heart J 1994; 72:13
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(anagement
• beta-adrenergic bloc!ers
• calcium antagonist
• disopyramide
• amiodarone, sotalol
• DDD pacing
• myotomy-myectomy
•plication of the anterior mitral leaflet
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HC( vs Aortic &tenosis
HC( +i*ed Obstruction
carotid pulse spi!e and dome parvus et tardus
murmur radiate to carotids
valsalva, standing s=uatting, handgrip
passive leg elevation
systolic thrill 9th left interspace 5nd right interspace
systolic clic! absent present
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Other Causes of Hypertrophy
• Clinical mimics
– glycogen storage, infants of diabetic mothers,
amyloid
• ?enetic
– oonanKs, +riedreichKs ata*ia, +amilial restrictive
cardiomyopathy "ith disarray
• ;*aggerated physiologic response
– Afro-Caribbean hypertension, old age hypertrophy,
athleteKs heart
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HC( vs AthleteKs Heart
HC( Athlete
B nusual pattern of ./H -
B ./ cavity E97 mm -
- ./ cavity L77 mm B
B .A enlargement -B @i0arre ;C? paterns -
B Abnormal ./ filling -
B +emale gender -
- thic!ness "ith deconditioning B
B +amily history of HC( -
.ir"#!atin 199; 91:196
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Hypertensive HC( of the ;lderly
• Characteristics
– modest concentric ./ hypertrophy #E55 mm$
– small ./ cavity si0e
– associated hypertension
– ventricular morphology greatly distorted "ith
reduced outflo" tract
– sigmoid septum and “grandma &A(”
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%estrictive Cardiomyopathy
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%estrictive Cardiomyopathies
• Hallmar!M abnormal diastolic function
• rigid ventricular "all "ith impaired
ventricular filling
• bear some functional resemblance to
constrictive pericarditis
• importance lies in its differentiation from
operable constrictive pericarditis
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;*clusion “?uidelines”
• ./ end-diastolic dimensions ≥ cm
• (yocardial "all thic!ness≥
36 cm
• ./ end-diastolic volume ≥ 374 m.m5
• ./ eection fraction E 548
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Classification
• 'diopathic• (yocardial
36 oninfiltrative
– 'diopathic
– &cleroderma
56 'nfiltrative
– Amyloid
– &arcoid
– ?aucher disease
– Hurler disease
26 &torage Disease – Hemochromatosis
– +abry disease
– ?lycogen storage
• ;ndomyocardial
– endomyocardial fibrosis
– Hyperesinophilic synd
– Carcinoid
– metastatic malignancies
– radiation, anthracycline
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Clinical (anifestations
• &ymptoms of right and left heart failure
• >ugular /enous 1ulse
– prominent x and y descents
• ;cho-Doppler
– abnormal mitral inflo" pattern
– prominent ; "ave #rapid diastolic filling$
– reduced deceleration time #
.A pressure$
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%estriction vs Constriction
History provide can important clues
• Constrictive pericarditis
– history of )@, trauma, pericarditis, sollagen
vascular disorders
• %estrictive cardiomyopathy
– amyloidosis, hemochromatosis
• (i*ed
– mediastinal radiation, cardiac surgery
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)reatment
• o satisfactory medical therapy
• Drug therapy must be used "ith caution
– diuretics for e*tremely high filling prssures
– vasodilators may decrease filling pressure – G Calcium channel bloc!ers to improve
diastolic compliance
– digitalis and other inotropic agents are not
indicated