inhal anat cor
TRANSCRIPT
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INHAL ANATOMI
PENGGUNAANZ-SCOREUNTUK MELIHAT STENOSIS PADA PARU
DALAM PENEGAKKAN DIAGNOSIS TETRALOGY OF FALLOT
ULFA PUSPITA RACHMA
G0013227
27 MEI 2015
UNIVERSITAS SEBELAS MARET
FAKULTAS KEDOKTERAN
PROGAM STUDI PENDIDIKAN DOKTER
2015
ACC
Mas Rico
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PENGGUNAANZ-SCOREUNTUK MELIHAT STENOSIS PADA PARU
DALAM PENEGAKKAN DIAGNOSIS TETRALOGY OF FALLOT
Tetralogy of fallot (ToF) mer!a"a# !e#$a"i% &a#%#' aaa# sia#o%i"
$a#' %er*iri *ari em!a% "e+ai#a# ",as- $ai% *e.e" se!%m /e#%ri"e+ (ventricular
septal defect, S)- s%e#osis i#.#*i+m /e#%ri"e+ "a#a# a%a iasa *ise%
s%e#osis !+mo#a+- ,i!er%ro.i /e#%ri"e+ "a#a#- *a# overriding aorta
ToF mer!a"a# &e#is !e#$a"i% &a#%#' aaa# %erseri#' Se"i%ar 354
a$i $a#' +a,ir *e#'a# !e#$a"i% &a#%#' aaa# me#*eri%a &e#is ToF i AS- 104
"ass !e#$a"i% &a#%#' "o#'e#i%a+ a*a+a, ToF- se*i"i% +ei, a#$a" !a*a +a"i+a"i
*ia#*i#'"a# !erem!a# Seiri#' *e#'a# me#i#'"a%#$a a#'"a "e+a,ira# *i
I#*o#esia- &m+a, a$i $a#' +a,ir *e#'a# !e#$a"i% &a#%#' &'a me#i#'"a% a
!er %i'a "ass !e#$a"i% &a#%#' aaa# *i I#*o#esia mem!er+i,a%"a# 'e&a+a !a*a
masa #eo#a%s Sea#$a" 25304 !e#*eri%a !e#$a"i% &a#%#' aaa# $a#'
mem!er+i,a%"a# 'e&a+a !a*a masa #eo#a%s me#i#''a+ !a*a +a# !er%ama
sia#$a &i"a %a#!a !e#a#'a#a# $a#' ai" Se"i%ar 254 !asie# ToF $a#' %i*a"
*i%era!i a"a# me#i#''a+ *a+am 1 %a,# !er%ama "e,i*!a#- 04 me#i#''a+sam!ai sia %a,#- 704 me#i#''a+ sam!ai sia 10 %a,#- *a# 654 me#i#''a+
sam!ai sia 0 %a,#
Pe#$a"i% &a#%#' aaa# seri#' *a!a% *i*e%e"si *e#'a# USG !a*a masa
"e,ami+a# Pemeri"saa# fetal echocardiography &'a ai" *i+a""a# !a*a
!e+a$a#a# a#%e#a%a+ sea'ai sa+a, sa% cara *e%e"si *i#i !e#$a"i% &a#%#' aaa#
ia'#osis *i#i ToF *a!a% me#e#%"a# +a#'"a, se+a#&%#$a ,ars *iami+
Pe#e%a!a# +a#'"a, $a#' %e!a% se%e+a, *e%e"si *i#i !e#$a"i% &a#%#' aaa# ToF
!a*a a#a" *a!a% me#'ra#'i mor%a+i%as *a# mori*i%as e#'a# !e#e'a"a#
*ia'#osis $a#' %e!a% *a# ce!a%- "om!+i"asi !e#$a"i% &a#%#' aaa# ToF *a!a%
*imi#ima+"a#
Sir"+asi *ara, !e#*eri%a ToF ere*a *ia#*i#' !a*a a#a" #orma+
e+ai#a# $a#' meme'a#' !era#a# !e#%i#' a*a+a, s%e#osis !+mo#a+ *a# S
Te"a#a# a#%ara /e#%ri"e+ "iri *a# "a#a# !a*a !asie# ToF a*a+a, sama a"ia%
a*a#$a S Ha+ i#i me#$ea"a# *ara, eas me#'a+ir o+a"a+i" me+a+i
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ce+a, i#i Ti#'"a% "e!ara,a# ,ama%a# !a*a &a+a# "e+ar *ara, *i /e#%ri"e+ "a#a#
a"a# me#e#%"a# ara, a+ira# *ara, !asie# ToF A+ira# *ara, "e !ar a"a#
me#r# a"ia% a*a#$a ,ama%a# !a*a &a+a# a+ira# *ara, *ari /e#%ri"e+ "a#a#8
,ama%a# $a#' %i#''i *i si#i a"a# me#$ea"a# ma"i# a#$a" *ara, er'era"
*ari /e#%ri"e+ "a#a# "e "iri Ha+ i#i erar%i ma"i# a#$a" *ara, mis"i# o"si'e#
$a#' a"a# i"% mas" "e *a+am aor%a se,i#''a a"a# me#r#"a# sa%rasi o"si'e#
*ara, $a#' ere*ar "e se+r, %,- *a!a% me#$ea"a# sia#osis 9i"a %er&a*i
,ama%a# !ara,- %, a"a# er'a#%#' !a*a *"%s ar%erioss *a# caa#'
caa#' ar%eri !+mo#a+is #%" me#*a!a%"a# s!+ai *ara, $a#' me#'a#*#'
o"si'e# :#se% 'e&a+a- %i#'"a% "e!ara,a# sia#osis $a#' %er&a*i sa#'a% er'a#%#'
!a*a %i#'"a% "e!ara,a# ,ama%a# $a#' %er&a*i !a*a &a+a# "e+ar a+ira# *ara, *i
/e#%ri"e+ "a#a#
era&a% s%e#osis !+mo#a+ er!e#'ar, +a#'s#' !a*a era'ai macam
ma#i.es%asi "+i#is $a#' *a!a% *i%em"a# !a*a !asie# ToF Seora#' !asie# *e#'a#
s%e#osis !+mo#a+ ri#'a# m#'"i# %i*a" memi+i"i 'e&a+a a!a !# sam!ai a",ir
masa "a#a""a#a"- seme#%ara !asie# *e#'a# s%e#osis !+mo#a+ era% memi+i"i
"em#'"i#a# +ei, %i#''i m#c+ 'e&a+a "+i#is *a+am +a# !er%ama "e,i*!a#
;a$i %i*a" me##&""a# sia#osis !a*a saa% +a,ir- 'e&a+a m+ai er"ema#' a#%ara
mr 2< +a# Ma#i.es%asi "+i#is !a+i#' mm a*a+a, mrmr asim%oma%i" *a#
sia#osis Sa%rasi o"si'e# ar%eri a$i ToF isa %ia%ia me#r# *e#'a# #$a%a
Fe#ome#a i#i *ise% =hypercyanotic spell>- iasa#$a mer!a"a# ,asi+
!e#$em!i%a# secara me#*a*a" a+ira# *ara, "e !ar Sera#'a# *a!a% %er&a*i se%ia!
a"% a#%ara sia 1 +a# *a# 12 %a,#- %er%ama %er&a*i a#%ara +a# "e2 *a# "e
3 Pa+i#' seri#' %er+i,a% se%e+a, a#'# %i*r- me#a#'is- a#' air esar- *a#
ma"a# Sera#'a# *i%a#*ai *e#'a# me#i#'"a%#$a "ece!a%a# *a# "e*a+ama#
!er#a!asa# (,i!er!#ea) *e#'a# sia#osis $a#' er%ama, !ara,
A#a" ToF me#&a*i iri%a%i. *a+am "ea*aa# "a*ar o"si'e# er"ra#'- a%a
memer+"a# as!a# o"si'e# $a#' +ei, a#$a"- a#a" *a!a% me#&a*i m*a, +e+a,-
me#'a#%"- a%a a,"a# %i*a" meres!o#s "e%i"a *i!a#''i+- me#$s $a#'
%er!%s!%s A#a" *e#'a# hypercyanotic spell a"a# me+a""a# 'era"a#
&o#'"o" (squating)- a'ar a+ira# *ara, "e !ar me#&a*i er%ama,- *a# sera#'a#
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sia#osis *a# sesa" me#&a*i er"ra#' Pa*a a#a" ToF- iasa#$a *i&m!ai
"e%er+ama%a# !er%m,a#- %i#''i *a# era% a*a# *a# "ra# %, "rs $a#'
%i*a" sesai *e#'a# sia a#a"
ToF *a!a% *i*ia'#osis see+m a$i saa% 'amara# a#a%omi &a#%#' m+ai
%er+i,a% &e+as !a*afetal echocardiography- iasa#$a !a*a sia 'es%asi 12 mi#''
Se'era se%e+a, ToF *i*ia'#osis- *isara#"a# !e#'ama%a# a#%e#a%a+ seria+ *e#'a#
i#%er/a+ < mi#'' #%" me#'i"%i !er%m,a# ar%eri !+mo#a+is-#%" me#i+ai
"ema+i ara, ar%eri !ar %ama *a# a+ira# *"%a+ *a# #%" me#'e/a+asi- &i"a
a*a- "e+ai#a# *i +ar &a#%#'
Pa*a !asie# ToF iasa#$a %er*a!a% "e+,a# %ama sia#osis- !er#a.asa#
ce!a% Se+a#&%#$a !er+ *i%a#$a"a# "e!a*a ora#' %a a%a !e#'as, !asie#- "a!a#
!er%ama "a+i m#c+#$a sia#osis- a!a"a, sia#osis *i%em"a# se&a" +a,ir- %em!a%
sia#osis m#c+- misa+#$a !a*a m"osa memra# iir *a# m+%- &ari %a#'a#
a%a "a"i- a!a"a, m#c+#$a %a#*a%a#*a sia#osis *i*a,+i o+e, .a"%or !e#ce%s-
sa+a, sa%#$a a"%i/i%as er+ei,a# a%a me#a#'is
Ria$a% sera#'a# sia#o%i" (hypercyanotic spell) &'a ,ars *i%a#$a"a#
"e!a*a ora#' %a !asie# a%a !e#'as, !asie# 9i"a a#a" s*a, *a!a% er&a+a#
a!a"a, seri#' &o#'"o" (squating) se%e+a, er&a+a# eera!a +a#'"a, see+m
me+a#&%"a# "ema+i er&a+a# Pe#%i#' &'a *i%a#$a"a# .a"%or risi"o $a#'
m#'"i# me#*"#' *ia'#osis ToF $ai%se!er%i .a"%or 'e#e%i"- ria$a% "e+ar'a
$a#'mem!#$ai !e#$a"i% &a#%#' aaa# Ria$a% %m, "ema#' a#a" &'a
!er+ *i%a#$a"a#- !emeri"saa# %m, "ema#' *a!a% *i'#a"a# &'a #%"
me#'e%a,ia!a"a, %er&a*i 'a'a+ %m, "ema#' a"ia%!er&a+a#a# !e#$a"i% ToF
Sia#osis se#%ra+ *a!a% *iama%i !a*a sea'ia# esar "ass ToF8 *esa%rasi
ar%eri ri#'a# m#'"i# %i*a" me#im+"a# sia#osis "+i#is Clubbing fingers *a!a%
*iama%i !a*a eera!a +a# !er%ama "e,i*!a# Ta#*a%a#*a 'a'a+ &a#%#'
"o#'es%i. &'a &ara#' *i%em"a#- "eca+i !a*a "ass re'r'i%asi !+mo#a+ era%
a%a ToF $a#' *iare#'i *e#'a# %i*a" a*a#$a "a%! !+mo#a+
Im!+s /e#%ri"e+ "a#a# $a#' +ei, "a% m#'"i# *i*a!a%"a# !a*a !a+!asi
Systolic thrillisa *i*a!a%"a# *i !era%asa# s%er#a+ "iri aa, Mrmr sis%o+i"
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grade III *a# I *isea"a# o+e, a+ira# *ara, *ari /e#%ri"e+ "a#a# "e sa+ra#
!ar Se+ama sera#'a# hypercyanotic spell m#c+- mrmr me#',i+a#' a%a
me#&a*i sa#'a% +em% Sama ,a+#$a !a*a ToF *e#'a# a%resia !ar- %i*a" a"a#
%er*e#'ar mrmr "are#a %i*a" a*a a+ira# *ara, a+i" "e /e#%ri"e+ "a#a# A+ira#
*ara, $a#' me#& a%a me+ea%i ce+a, a#%ar /e#%ri"e+ %i*a" me#im+"a#
%r+e#si- se,i#''a iasa#$a %i*a" %er*e#'ar "e+ai#a# as"+%asi Mrmr e&e"si
sis%o+i" %er'a#%#' *ari *era&a% os%r"si a+ira# *ara, *i /e#%ri"e+ "a#a# Ma"i#
sia#osis erar%i memi+i"i os%r"si +ei, ,ea% *a# mrmr +ei, ,a+s Pasie#
asia#o%i" *e#'a# ToF (pinktet) memi+i"i mrmr sis%o+i" $a#' !a#&a#' *a# "eras
*e#'a# thrill se!a#&a#' a+ira# *ara, /e#%ri"e+ "a#a# Se+ai# i% isa *i%em"a#
"+i" e&e"si aor%a- S2 %#''a+ (!e#%!a# "a%! !+mo#a+ %i*a" %er*e#'ar) Seri#'
!+a !asie# ToF me#'a+ami s"o+iosis *a# retinalengorgement
Pa*a !emeri"saa# +aora%orim *ara, *a!a% *i&m!ai !e#i#'"a%a# &m+a,
eri%rosi% *a# ,ema%o"ri% (!o+isi%emia /era) $a#' sesai *e#'a# *esa%rasi *a#
s%e#osis< :"sime%ri *a# a#a+isis 'as *ara, ar%eri me#*a!a%"a# sa%rasi o"si'e#
$a#' er/ariasi- %e%a!i !H *a# !C:2 #orma+ "eca+i !a*a "o#*isi tetspell
:"sime%ri er'#a !a*a !asie# "+i% ,i%am a%a !asie# a#emia $a#' %i#'"a%
sia#o%i"#$a %i*a" &e+as Sia#osis %i*a" a"a# %am!a" "eca+i i+a ,emo'+oi#
%ere*"si me#ca!ai 5 m'?*L Pe#r#a# resis%e#si /as"+ar sis%emi" se+ama
a"%i/i%as- ma#*i- ma!# *emam a"a# me#ce%s"a# !ira "a#a# "e "iri *a#
me#$ea"a# ,i!o"semia
Pemeri"saa# e+e"%ro"ar*io'ram *a!a% me#em"a# *e/iasi a"sis "e "a#a#
(@120 @150)- ,i!er%ro.i /e#%ri"e+ "a#a# a%a "e*a /e#%ri"e+- ma!#
,i!er%ro.i a%rim "a#a# e"a%a# /e#%ri"e+ "a#a# $a#' me#o#&o+ %er+i,a% *e#'a#
'e+oma#' R esar *i sa*a!a# !re"or*ia+ a#%erior *a# 'e+oma#' S esar *i
sa*a!a# !re"or*ia+ +a%era+is
Pemeri"saa# .o%o ro#%'e# %,oraB *a!a% me#em"a# 'amara# &a#%#'
ere#%" se!a% (boot-shaped heart/ couer-en-sabot) *a# !e#r#a#
/as"+arisasi !ar "are#a er"ra#'#$a a+ira# *ara, $a#' me#& "e !ar a"ia%
!e#$em!i%a# "a%! !+mo#a+ !ar (s%e#osis !+mo#a+)
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MRI *a!a% me#'"r /o+me /e#%ri"e+ "a#a# *a# "iri- me#i+ai &a+r a+ira#
*ara, /e#%ri"e+ "a#a#- ar%eri !+mo#a+- aor%a- *e.e" se!%m /e#%ri"e+ MRI &'a
*a!a% me#i+ai s%e#osis caa#' ar%eri !+mo#a+ $a#' er"o#%risi *a+am
me#$ea"a# i#s.i sie#si !+mo#a+ *a# "o+a%era+ aor%o!+mo#a+ $a#' *a!a%
me#$ea"a# overload /o+me /e#%ri"e+ "iri Ha+ i#i seri#' *i&m!ai !a*a !asie#
$a#' *iser%ai a%resia !+mo#a+
E"o"ar*io'ram sa#'a% mema#% me#'o#.i rmasi *ia'#osis *a#
me#'e/a+asi eera!a masa+a, $a#' %er"ai% *e#'a# ToF Pemesara# /e#%ri"e+
"a#a#- *e.e" se!%m /e#%ri"e+- overriding aorta- *a# os%r"si sa+ra# /e#%ri"e+
"a#a# *a!a% *i%am!i+"a# secara &e+as8 *a!a% *i%#&""a# shunting $a#' me+ea%i
S *a# !e#i#'"a%a# "ece!a%a# a+ira# o!!+er $a#' me+ea%i /e#%ri"e+ "a#a#
U"ra# caa#' %ama ar%eri !+mo#a+is *a# !ro"sima+ ser%a se%ia! a+ira# *ara,
%ama,a# +ai# me#& "e !ar *a!a% *ie/a+asi- %e%a!i ar%eri !+mo#a+is a'ia#
*is%a+ %i*a" *a!a% *e#'a# m*a, *i+i,a% o+e, e"o"ar*io'ram
a%e%erisasi "a# !emeri"saa# $a#' r%i#8 *a!a% *i+a""a# &i"a *a%a $a#'
*i!er+"a# #%" !e#'ami+a# "e!%sa# "ore"si e*a, %i*a" *a!a% *i!ero+e,
*e#'a# !emeri"saa# !e##&a#' +ai##$a Pe#%i#' #%" me#*a!a%"a# *a%a sa%rasi
o"si'e# ar%eri sis%emi" *a# *esa%rasi er,#'a# *e#'a# s%e#osis sa+ra# "e+ar
/e#%ri"e+ "a#a# T&a# "a%e%erisasi &a#%#' a*a+a, #%" me#i+ai "ra# a#+s
!+mo#a+ *a# ar%eri !+mo#a+- me#i+ai "e!ara,a# os%r"si a+ira# *ara, /e#%ri"e+
"a#a#- +o"asi *a# "ra# *e.e" se!%m /e#%ri"e+- ser%a me#$i#'"ir"a#
"em#'"i#a# a#oma+i ar%eri "oro#er
A#'io'ra.i mer!a"a# a'ia# i#%e'ra+ *ari "a%e%erisasi &a#%#' A#'io'ra.i
!ar &'a ,ars *i+a""a# #%" me#'e%a,i "ra# ar%eri !+mo#a+is %ama *a#
caa#' ser%a #%" me#$i#'"ir"a# "em#'"i#a# a*a#$a s%e#osis caa#' ar%eri
!+mo#a+ A#'io'ra.i aor%a &'a *i!er+"a# #%" mem/isa+isasi"a# a#a%omi
ar%eri "oro#er- %er%ama #%" me#$i#'"ir"a# a*a#$a ar%eri "oro#er me+i#%asi
i#.#*i+m /e#%ri"e+ "a#a#
om!o#e# *asar "ore"si e*a, %e%ra+o'i o. Fa++o% (T:F) a*a+a, /e#%ri"e+
!e#%!a# *e.e" se!%m *a# re+ie. /e#%ri"e+ "a#a# os%r"si sa+ra# "e+ar $a#'
si'#i.i"a# (R:T:) ;a#%a# os%r"si !ar a##+ar si'#i.i"a# *ica!ai o+e,
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'a#''a# a#+s "a%! !+mo#a+ (PA) *a# !e#$isi!a# !a%c, %ra#sa##+ar (TAP)
Mes"i!# /e#%ri"e+ "a#a# *i+a%asi *a# *is.#'si "o#se"e#si a",ir !e#%i#'
re'r'i%asi !ar- a*a "%i a,a "e'a'a+a# #%" memas""a# TAP "e%i"a sa+a,
sa% *ii#*i"asi"a# me#i#'"a% e*a, "ema%ia#
Mes"i!# a#$a" "ri%eria $a#' a*a #%" TAP !e#$isi!a#- %i*a" a*a
"ese!a"a%a# $a#' %erai" Aa+ e"er&a me#',asi+"a# !e#'ema#'a# *ari me&a
"a%! !ar *i%erima mi#ima+ *iame%er ci#ci# #%" mema#% e*a,
!e#'ami+a# "e!%sa# A+%er#a%i. a#% !e#'ami+a# "e!%sa# *i*asar"a# !a*a
PA Ds"or Ta" sa% !# *ari "ri%eria i#i secara o&e"%i. me##&""a#
"em#'"i#a# memi+i"i %i*a" *a!a% *i%erima 'ra*ie# %e"a#a# !asca o!erasi *i
se+r, PA %erse%
Sea, +a!ora# see+m#$a *ari !sa% mema,as *am!a" /ariasi *a+am
!e#i+aia# #orma%i. #%" s%r"%r &a#%#' !a*a a#a" !ra"%e" e*a, &a#%#' *a#
*ire"ome#*asi"a# o!%ima+ "m!+a# *a%a *ime#si #orma%i. i%a !ri,a%i# a,a
!e#''#aa# $a#' %i*a" Ds"or *a+am e*a, !e#'ami+a# "e!%sa# *a!a%
mem!e#'ar,i ,asi+ o!erasi Pe#e+i%ia# *i+a""a# #%" me#'&i ,#'a# a#%ara
*iame%er PA Dscore *a# !asca o!erasi %ra#s!ar 'ra*ie# me#''#a"a# sa% se%
*a%a "+i#is "o#%em!orer +o"a+ a%a i#i "em*ia# *a!a% *i'#a"a# sea'ai
"e+om!o" &i coa #%" mema#% mem!erai"i "e!%sa# e*a, mema%
se,#'a# *e#'a# TAP !e#$isi!a#
Si#'"a%#$a- "ami ",aa%ir a,a T:F saa% !ra"%e" !erai"a# m#'"i#
%i*a" %e!a% me#era!"a# PA *iame%er Ds"or #%" "ri%eria !e#$isi!a# TAP
Pra"%e" i#i *a!a% me#'a"ia%"a# !e+es%aria# si'#i.i"a# "ra# !ar "a%! a#+s-
$a#' me#$ea"a# %i*a" *a!a% *i%erima !asca o!erasi !#ca" 'ra*ie# %e"a#a#
PA ;er*asar"a# *a%a- mes"i!# er*asar"a# *a%a se% "eci+- me#''amar"a#
,emo*i#ami" $a#' %er&a*i !a*a "a%! !ar *a+am ,a+ !ascaT:F mem!erai"i
%e"a#a# 'ra*ie# 'ra*ie# PA *a+am "ai%a##$a *e#'a# "ra# PA *i&e+as"a#
me#''#a"a# Ds"or o!%ima+ ami %e+a, me#'"r "em#'"i#a# %er&a*i#$a
'ra*ie# !asca o!erasi %i*a" *a!a% *i%erima *i se+r, PA ami !erca$a a,a
*a%a i#i *a!a% *i'#a"a# #%" mem!erai"i !roses !e#'ami+a# "e!%sa# *a#
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er!era# *a+am memi#ima+"a# &m+a, !asie# *e#'a# PA 'ra*ie# !ascaT:F
$a#' s+i% #%" *i!erai"i8 *i!er+"a# !e#$e+i*i"a# +ei, +a#&%
DAFTAR PUSTAKA
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Aori- M et al., 2012 Te%ra+o'$ o. Fa++o% re!air o!%ima+ Dscore se .or
%ra#sa##+ar !a%c, i#ser%io# uropean !ournal of Cardio-Thoracic
Surgery 1
Rs+i- RH- arma*i 2013 ia'#osis *a# Ta%a+a"sa#a Te%ra+o'$ o.
Fa++o% C"#-$%%$0(3) 17