Tetralogy of Fallot-ppt

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Tetralogy of Fallot Mauren Lusi Selfiana (0961050115) Pembimbing : Dr. Heru S., Sp. A

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Tetralogy of Fallot

Mauren Lusi Selfiana (0961050115)

Pembimbing : Dr. Heru S., Sp.A

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Definisi pena!i" #an"ung !$ngeni"al %engan

!elainan s"ru!"ur #an"ung ang mun&ul

pa%a saa" la'ir %an "er#a%i peruba'analiran %ara' %i #an"ung

Menuru" AHA ( American Heart Assosiation, 009) e"ral$gi *all$"memili!i empa" !elainan :

Defe! Sep"um +en"ri!el (Ventricular

Septal Defect, +SD)

S"en$sis Pulm$nal (Pulmonary

Stenosis, PS)

Overriding Aorta

Hiper"r$fi +en"ri!el anan

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Etiologi

Penebab %ari pena!i" #an"ung ba-aan "i%a! %i!e"a'ui, mes!ipun s"u%i gmenun#u!!an e"i$l$gi mul"ifa!"$rial

Sebua' s"u%i %ari P$r"ugal melap$r!an ba'-a p$lim$rfisme re%u!"aseme"'lene"e"ra'%r$f$la"e (MH*) gen %apa" %ianggap sebagai gen !ereun"u! "e"ral$gi *all$"

*a!"$r Prena"al "er!ai" %engan insi%en ang lebi' "inggi %ari "e"ral$gi *all"ermasu! ibu ang "er!ena rubella (a"au pena!i" irus lainna) selama !e!urangna gii saa" !e'amilan, penggunaan al!$'$l ibu, usia ibu ang lebi20 "a'un

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Manifestasi Klinis

Sian$sis merupa!an "an%a pen"ing %ari e"ral$gi *all$"

3ai4bai ang "ela' %i %iagn$sis e"ral$gi *all$" !a%ang4!a%ang memili!i ini "er#a%i #i!a bai mela!u!an !egia"an seper"i menangis a"au se%ang a%ausus

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Suli" 3ernafas

Men#a%i sanga" lela' %an lemas

i%a! menanggapi suara %an sen"u'an $rang "ua

Men#a%i sanga" re-el

Pingsan

esuli"an ma!an

7agal ber!embang

Dspnea saa" a!"ii"as, biasana memburu! %engan usia

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Anamnesis

Pemeri!saan *isi! :

8nspe!si :

lubbing finger "ampa! se"ela' usia 6

bulan

S!$li$sis (!e ara' !anan)

3en"u! %a%a bai masi' n$rmal, namun

pa%a ana! ang lebi' besar "ampa!

men$n#$l a!iba" pelebaran en"ri!el

!anan

Ana! a!an sering Sua""ing (#$n

ana! %apa" ber#alan, se"ela' be

beberapa lama ana! a!an ber#$

beberapa -a!"u sebelum ia ber#

!embali.

Serangan sian$"i! men%a%a! (bl

spells;&an$"i& spells;par$<sm

'p$<i& spells) %i"an%ai %engan

napas !usmaul,lemas,!e#ang,sin

sampai !$ma %an !ema"ian

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Palpasi

eraba ge"aran bising sepan#ang "epi s"ernum !iri

Aus!ul"asi

Pa%a aus!ul"asi "er%engar bising sis"$li! ang !eras %i%aera' pulm$nal ang sem

melema' %engan ber"amba'na %era#a" $bs"ru!si

3uni #an"ung 8 !eras (penu"upan "ri!uspi% ang !ua").

3uni #an"ung 88 "erpisa' %engan !$mp$nen pulm$nal ang lema'

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Pemeriksaan Penunjang Lab$ra"$rium

A%ana pening!a"an 'em$gl$bin %an'ema"$!ri" (H") a!iba" sa"urasi $!sigenang ren%a'

A7D : pening!a"an "e!anan par"ial!arb$n%i$!si%a (P/), penurunan

"e!anan parsial $!sigen (P/) %an

penurunan PH

*$"$ '$ra!s ampa! #an"ung berben"u! sepa"u (ape!s

"erang!a", &l$g4li!e) %engan !$nus

pulm$nalis &e!ung %an penurunanas!ularisasi %i paru

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=7 (ele!"r$!ar%i$gram) es ini un"u! menge"a'ui en"ri!el !anan membesar ('iper"r$fi en"ri!

%eiasi a!sis !e !anan (right axis deviation). 7el$mbang P %i'an"aran 8

pulm$nal). a%ang4!a%ang "er%apa" gel$mbang > %i +8

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=!$!ar%i$gragi =!$!ar%i$grafi a%ala' "es pen"ing un"u! men%iagn$sis "e"ral$gi *all$" !arena m

empa" &a&a" #an"ung

7ambaran e!$!ar%i$grafi ang !'as pa%a /* a%ala' %efe! sep"um en"ri!el beoverriding aorta

a"up pulm$nal "i%a! selalu %apa" %ili'a" #elas

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a"e"erisasi ?an"ung

Diperlu!an sebelum "in%a!an pembe%a'an un"u! menge"a'ui :

Defe! sep"um en"ri!el mul"iple

Men%e"e!si !elainan ar"eri !$r$nari

Men%e"e!si s"en$sis pulm$nal perifer

Meli'a" u!uran ar"erial pulm$nalis

Men%e"e!si a%ana penurunan sa"urasi $!sigen

Pening!a"an "e!anan en"ri!el !anan, %engan "e!anan pulm$nalis n$rmal a"au ren%a'

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Tatalaksana

Serangan Sian$"i!

P$sisi lu"u" !e %a%a.

M$rfin Sulfa" 0,140, mg;!g33 S, 8M, a"au 8+ un"u! mene!an

pusa" pernafasan %an menga"asi "a!ipne.

3i!arb$nas na"ri!us 1 m=;!g33 8+ un"u! menga"asi asi%$sis.

D$sis ang sama %apa" %iulangi %alam 10415 meni". /!sigen %apa" %iberi!an

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Dapa" %ilan#u"!an %engan pemberian :

Pr$pan$l$l 0,0140,5 mg;!g33 (ra"a4ra"a 0,05 mg;!g33) 8+ b$lus perla'an un"u!

menurun!an %enu" #an"ung se'ingga serangan %apa" %i a"asi.

e"amin 14@ mg;!g33 (ra"a4ra"a mg;!g33) 8+ perla'an (%alam 60 %e"i!). Prep

be!er#a %engan mening!a"!an resis"ensi as!ular sis"emi! %an #uga sebagai se%

+as$!$ns"ri!"$r seper"i fenilefrin 0,0 mg;!g33 8+ mening!a"!an resis"ensi as!

sis"emi!, se'ingga aliran %ara' !e paru mening!a".

Penamba'an $lume &airan "ubu' %engan infus efe!"if 

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3ai %engan ri-aa" sian$sis :

Pa%a bai a"au ana! %engan ri-aa" sian$sis 'arus %iberi!an pr$pan$l$l

%engan %$sis 0,541,5 mg;!g33;64 #am a"au 46 mg;!g33;'ari sampai %

$perasi

3ila serangan sian$sis masi' sering berulang %an !ea%aan ma!in membu

B 6 bulan : %ila!u!an $perasi Blalock-aussig Shunt (3S) semen"ara menung

lebi' besar a"au !ea%aan umum lebi' bai! un"u! $perasi %efini"if 

C6 bulan : $perasi !$re!si "$"al biasana %apa" langsung %ila!u!an

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Daftar Pustaka

*re%eriue 3. an% $ber" H. A. /rp'ane" ?$urnal $f are Disease : etralo

!allot. 009E1410.

Fa"i$nal Hear", Lung, an% 3l$$% 8ns"i"u"e. etralogy of !allot. 009.

Ameri&an Hear" Ass$&ia"i$n. etralogy of !allot. 011.

La"ief A, Fapi"upulu P. M, %!!. 3u!u ulia' 8lmu ese'a"an Ana!. ?a!ar"a

8lmu ese'a"an Ana! *a!ul"as e%$!"eran 8n%$nesia, 00G, 'al : G14G12E Shabir B. and Mary C. M. Emedicine Me%s&ape : etralogy of !allot. 01

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Arifpu"ra A, A%ani F, 8%ris F. S. api"a Sele!"a e%$!"eran. ?a!ar"a : 3ala

*a!ul"as e%$!"eran niersi"as 8n%$nesia, 012, 'al : @42

Hull Dai%, ?$'ns"$n Dere! 8. Dasar 4 %asar pe%ia"ri. =%isi @. ?a!ar"a : =7

1@0451.

Fapi"upulu P. M., 7'aali M.+. Pu"ra S. .,%!!. 8lmu !ese'a"an ana!. =%isi

?a!ar"a : niersi"as 8n%$nesiaE 01.'.G54.

3e'rman . =. +aug'an +. 8lmu ese'a"an Ana!. =%isi . ?a!ar"a: =7E 01

@@

Shabir B. and Mary C. M. =me%i&ine Me%s&ape : etralogy of !allot "ana

and reatment. 015.

Pu"ra S. , A%ani F., %!!. Pe%$man Pelaanan Me%is 8!a"an D$!"er Ana! 8

?a!ar"a : 8DA8E 009.'. @194

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