cvs k17 cardiomyopathies

8/17/2019 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

cvs k17 cardiomyopathies

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