Post on 01-Jun-2018
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PENYAKIT JANTUNG
KARDIOMIOPATI
Dr.TONIPRASETIA.SpPD
8/9/2019 PENYAKIT JANTUNG KARDIOMIOPATI
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Definition
A primary disorder of the heart
mus!e that auses a"#orma!myoardia! performa#e a#d is #ot
the resu!t of disease or dysfu#tio# of
other ardia strutures $ myoardia!i#fartio#% systemi hyperte#sio#%
&a!&u!ar ste#osis or re'ur'itatio#(
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WHO Classification
U#)#o*# ause+primary,
Di!ated -ypertrophi
Restriti&e
u#!assified
Specific heart muscle
disease (secondary)InfectiveMetabolicSystemic diseaseHeredofamilialSensitivityToxic
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Functional Classification
Di!atated+o#'esti&e% D.M% ID.,
&e#triu!ar e#!ar'eme#t a#d syst dysfu#tio# -ypertrophi+I-//% -.M% -O.M,
i#appropriate myoardia! hypertrophy
i# the a"se#e of -TN or aorti ste#osis
Restriti&e+i#fi!trati&e, a"#orma! fi!!i#' a#d diasto!i fu#tio#
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Idiopathic Dilated
Cardiomyopathy
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ID. 0 Defi#itio#
a disease of u#)#o*# etio!o'y that
pri#ipa!!y affets the myoardium
12 di!atatio# a#d systo!i dysfu#tio#
#reased heart si3e a#d *ei'ht
2e#triu!ar di!atatio#% #orma! *a!! thi)#ess heart dysfu#tio# out of portio# to fi"rosis
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I#ide#e a#d Pro'#osis
4056 ases per 566%666
76%666 #e* ases per year i# the U8/8A8 death from pro'ressi&e pump fai!ure
50year 79:
70year 490;6:
90year ;60
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.!i#ia! Ma#ifestatio#s
-i'hest i#ide#e i# midd!e a'e symptoms may "e 'radua! i# o#set
aute prese#tatio#o
misdia'#osed as &ira! URI i# you#' adu!tso u#ommo# to fi#d speifi myoardia!
disease o# e#domyoardia! "iopsy
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-istory a#d Physia!E=ami#atio#
/ymptoms of heart fai!ure
pu!mo#ary o#'estio# +!eft ->,
dysp#ea +rest% e=ertio#a!% #otur#a!,% orthp#ea systemi o#'estio# +ri'ht ->,
edema% #ausea% a"domi#a! pai#% #oturia
!o* ardia output
fati'ue a#d *ea)#ess hypote#sio#% tahyardia% tahyp#ea% J2D
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Cardiac Imaging
.hest radio'ram
E!etroardio'ram
7;0hour am"u!atory E.G +-o!ter, !i'htheaded#ess% pa!pitatio#% sy#ope
T*o0dime#sio#a! ehoardio'ram
Radio#u!ide &e#triu!o'raphy .ardia atheteri3atio#
a'e ?;6% ishemi history% hi'h ris) profi!e%
a"#orma! E.G
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Management of DCM
1imit ati&ity "ased o# fu#tio#a! status
sa!t restritio# of a 70' Na@+9' Na.!, diet f!uid restritio# for si'#ifia#t !o* Na@
i#itiate media! therapy
A.E i#hi"itors% diuretis di'o=i#% ar&edi!o!
hydra!a3i#e B #itrate om"i#atio#
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{Hypertrophic
Cardiomyopathy
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-ypertrophi .ardiomyopathy
>irst desri"ed "y the >re#h a#d Germa#s
arou#d 5C66 u#ommo# *ith ourre#e of 6867 to 687:
a hypertrophied a#d #o#0di!ated !eft &e#tri!e
i# the a"se#e of a#other disease
sma!! 12 a&ity% asymmetria! septa!hypertrophy +A/-,% systo!i a#terior motio# of
the mitra! &a!&e !eaf!et +/AM,
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12/98
medslides.com14
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Pathophysio!o'y
/ysto!e
dy#ami outf!o* trat 'radie#t Diasto!e
impaired diasto!i fi!!i#'% fi!!i#' pressure
Myoardia! ishemia
mus!e mass% fi!!i#' pressure% O7 dema#d
&asodi!ator reser&e% api!!ary de#sity
a"#orma! i#tramura! oro#ary arteries
systo!i ompressio# of arteries
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.!i#ia! Ma#ifestatio#
Asymptomati% ehoardio'raphi fi#di#'
/ymptomati dysp#ea i# C6:
a#'i#a petoris i# 9:
fati'ue% pre0sy#ope% sy#ope
ris) of /.D i# hi!dre# a#d
ado!ese#ts
pa!pitatio#% PND% .->% di33i#ess !ess
freue#t
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Management
"eta0adre#er'i "!o)ers
a!ium a#ta'o#ist
disopyramide
amiodaro#e% Feta"!o)er
myotomy0myetomy
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Restriti&e .ardiomyopathy
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RestrictiveCardiomyopathies
Hallmark: abnormal diastolic function rigid ventricular wall with impaired ventricularfilling
bear some functional resemblance toconstrictive pericarditis
importance lies in its differentiation fromoperable constrictive pericarditis
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Classification
Idiopathi
Myoardia! 58 No#i#fi!trati&e
Idiopathi
/!eroderma
78 I#fi!trati&e Amy!oid
/aroid
Gauher disease
-ur!er disease
48 /tora'e Disease -emohromatosis
>a"ry disease
G!yo'e# stora'e E#domyoardia!
e#domyoardia! fi"rosis
-yperesi#ophi!i sy#d
.ari#oid
metastati ma!i'#a#ies
radiatio#% a#thray!i#e
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Clinical Manifestations
Symptoms of right and left heart failureJugular Venous Pulse prominentxandydescents
Echo-Doppler abnormal mitral inflow pattern
prominent E wave (rapid diastolic filling) reduced deceleration time (LA pressure)
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Treatment
No satisfatory media! therapy
Dru' therapy must "e used *ith autio# diuretis for e=treme!y hi'h fi!!i#' prssures
&asodi!ators may derease fi!!i#' pressure .a!ium ha##e! "!o)ers to impro&e
diasto!i omp!ia#e
di'ita!is a#d other i#otropi a'e#ts are #ot
i#diated
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