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CASE REPORT

TETRALOGY OF FALLOT

Compiled By:

Putra Baruna 110100037

Swapna A/P Chandrasegaran 110100380

Supervisor :

dr. Johannes Harlan Saing, .!ed "Ped#, Sp.A "!#

CHILD HEALTH DEPARTMET

HA!I ADAM MALI" GEERAL HOSPITAL

FAC#LTY OF MEDICIE

S#MATERA #TARA #I$ERSITY

MEDA

%&'(

1



COTETS

CHAPTER I ITROD#CTIO )))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))) '

1.1. Ba$%ground ...................................................................... 3

1.&. '()e$ti*e .......................................................................3

CHAPTER II LITERAT#RE RE$IE* )))))))))))))))))))))))))))))))))))))))))))))))))))) +

&.1. +einition ........................................................................................ -

&.&. Histori$al noration .................................................................... -

&.3. pideiolog ...................................................................... -

&.-. tiolog ................................................................................ 2

&.2. (riolog .............................................................................

&..Anato ....................................................................................... 7

&.7. Pathophisiolog and Cir$ulation in 4'5......................................11

&.8. +iagnosis ...................................................................................... 1-

&.6. 4reatent...................................................................................... 16

&.10.Prognosis...................................................................................... &8

&.11.Copli$ation............................................................................... &6

CHAPTER III CASE REPORT)))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))),&

CHAPTER I$ DISC#SSIO - S#MMARY))))))))))))))))))))))))))))))))))) )))))))))) (.

-.1. +is$ussion.............................................................................. .......... 26-.&. Suar .................................................................................. .......... 1

REFERECES)))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))))) )))))))))) /%

2



CHAPTER I

ITROD#CTIO

')' B0123rou4d

4etralog o 5allot is a $anoti$ $ongenital a(noralities ost reuentl.

Pre*alention is 3 o e*er 10.000 li*e (irth, and a$$ounts or 7109 all $ongenital $ardia$

aloration1. 4etralog o 5allot is a $ongenital aloration that $onsists o an

inter*entri$ular $ouni$ation, also %nown as a *entri$ular septal dee$t, o(stru$tion o the

right *entri$ular outlow tra$t, o*erride o the *entri$ular septu ( the aorti$ root, and right

*entri$ular hpertroph. &

4he etiolog is ultia$torial, (ut reported asso$iations in$lude untreated aternal

dia(etes, phenl%etonuria, and inta%e o retinoi$ a$id. Asso$iated $hroosoal anoalies

$an in$lude trisoies &1, 18, and 13, (ut resen$e e:perien$e points to u$h reuent

asso$iation o i$rodeletions o $hroosoe &&. 4he ris% o re$$uren$e in ailies is 39. &,3

4he $lini$al eatures o tetralog o 5allot are dire$tl related to the se*erit o the

anatoi$ dee$ts. nants oten displa the ollowing;+ii$ult with eeding5ailure to thri*e

pisodes o (luish pale s%in during $ring or eeding "ie, <4et< spells# :ertional dspnea,

usuall worsening with age Phsi$al indings in$lude the ollowing; ost inants are saller

than e:pe$ted or ageCanosis o the lips and nail (ed is usuall pronoun$ed at (irthAter age

3 onths, the ingers and toes show $lu((ing. A sstoli$ thrill is usuall present anteriorl

along the let sternal (order. A harsh sstoli$ e)e$tion urur "S# is heard o*er the

puloni$ area and let sternal (order . -

4he $lini$al eatures o tetralog o 5allot are generall tpi$al, and a preliinar

$lini$al diagnosis $an alost alwas (e ade. Be$ause ost inants with this disorder reuire

surger, it is ortunate that the a*aila(ilit o $ardiopulonar (pass "CPB#, $ardioplegia,and surgi$al te$hniues is now well esta(lished. ost surgi$al series report e:$ellent $lini$al

results with low or(idit and ortalit rates.-

')% O56e17ive

4his paper is $opleted in order to ulill one o the reuireents in the Senior

Clini$al Assistan$e progra in +epartent o Child Health o Ha)i Ada ali% =eneral

Hospital, >ni*ersit o ?orth Suatera. n addition, this paper passes the %nowledge o

tetralog o 5allot and its anageent.

3



CHAPTER II

LITERAT#RE RE$IE*

%)') De8i4i7io4

4etralog o allot results or a single de*elopent dee$t ; an a(noral anterior

and $ephalad displa$eent o the inandi(ular portion o the inter*entri$ular septu. 4he

$onseuen$es o this de*iations are o(stru$tion o the right *entri$ular outlow "pulonar

stenosis#, *entri$ular septal dee$t "@S+#, o*erriding aorta that re$ei*es (lood ro (oth

*entri$les, and right *entri$uar hpertroph owing to the high pressure load pla$ed on the

@ ( the pulonali$ stenosis.1

%)%) His7ori10l I48orm07io4

4he irst anatoi$ des$ription o this aloration is $redited to the +anish

anatoist ?iels Stensen, in 17&. t was 5allot, howe*er, in 1888 who $orrelated the

pathologi$ and $lini$al aniestations o this $ardia$ aloration, whi$h he tered la

aladie (leue. He ound the $hara$teristi$ anato at autops in two patients with long

standing $anosis. Su(seuentl, 5allot prospe$ti*el diagnosed a $anoti$ patient and

was pro*en $orre$t at the tie o the postorte e:aination.

4he e*olution o surgi$al treatent or $anoti$ heart disease was $losel lin%ed to

4'5. n 16-2, Alred Blalo$%, @i*ien 4hoas, and Helen 4aussig $on$ei*ed o and

ipleented the irst surgi$al aortopulonar shunt or palliation o $anosis in a oung girl

with 4'5. 5urther inno*ation $ulinated in the e*olution o $ardiopulonar (pass and

intra$ardia$ $orre$tion o $ongenital heart lesions. 4en ears ater Blalo$% and 4aussig irst

reported theirs and 4hoas landar% a$$oplishent, illihei a$hie*ed intra$ardia$ repair,

using $ontrolled $ross$ir$ulation, in a oung (o with 4'5. Su(seuent de$ades ha*e

resulted in urther reineents o surgi$al te$hniues so that intra$ardia$ repair, in e*en

oung inants, is $oonpla$e.&,3

%),) Epidemiolo3y

4etralog o 5allot is one o the ost $oon, i not the ost $oon, or o

$anoti$ $ongenital heart disease "CH+#. 4he pre*alen$e o 4'5 *aries (etween studies

whose designs dier su(stantiall in ethods o as$ertainent, diagnosti$ te$hniues, length

4



o ollowup, and orphologi$ $lassii$ation o dee$ts. 5or e:aple, soe o the

in*estigations easuring the pre*alen$e o 4'5 in li*e (irths were perored prior to the

$onsistent identii$ation and reporting o CH+, and ost were perored prior to the use o

e$ho$ardiograph. Howe*er, the ore re$ent use o e$ho$ardiograph signii$antl ipa$ts

on the as$ertainent o $ardia$ dee$ts and the a$$ura$ o diagnosis. oreo*er, i sui$ient

ollowup was not perored, then soe patients ight (e o*erloo%ed or is$lassiied as

siple *entri$ular septal dee$ts with pulonar stenosis "PS# rather than 4'5. n addition,

ost studies group all ors o 4'5 together regardless o pulonar *al*e anato, so that

the spe$ii$ pre*alen$e o 4'5 with PS rather than PA or AP@ is not usuall deined.3

4ogether, a nu(er o studies indi$ate that the pre*alen$e o 4'5 "regardless o

pulonar *al*e orpholog# ranges ro 0.& to 0.-8 per 1,000 li*e (irths 'ne re$ent

stud ro alta reported a pre*alen$e o 0.8 per 1,000 li*e (irths. 4he proportion o

patients with CH+ who ha*e 4'5 ranged ro 3.29 to 69. 4hese studies also indi$ate that

CH+ in general and 4'5 in parti$ular appear to (e euall pre*alent in populations o

dierent ra$e or ethni$ (a$%ground. 3

4he Baltioreae Dashington nant Stud "BDS#, $ondu$ted (etween 1681 and

1686, is the ost re$ent and perhaps ost a$$urate stud to assess the pre*alen$e o the

dierent su(tpes o 4'5. 4he BDS was a population(ased stud that as$ertained an

inant diagnosed ( either e$ho$ardiogra, $ardia$ $atheteriEation, $ardia$ surger, or

autops with CH+ within 1 ear o lie in the Baltiorae Dashington area. 4his stud,

thereore, in$reased as$ertainent and deinition o lesions ( e:tensi*e in*estigation in a

deined area, use o e$ho$ardiograph, and sui$ient ollowup to identi presua(l all

$ases. n this stud, 4'5 o$$urred in 0.33 per 1,000 li*e (irths, was the ith ost $oon

dee$t o*erall, a$$ounting or .89 o all ors o CH+, and was the ost $oon or o

$anoti$ CH+ . ' those with 4'5, 76.79 had 4'5 with PS, as $opared with &0.39 with

4'5 and PA.

n parti$ular, 4'5 with PS had a pre*alen$e o 0.& per 1,000 li*e (irths and

a$$ounted or 2.-9 o all o the lesions o(ser*ed in the BDS $ohort . As was suggested (

pre*ious studies, the BDS deonstrated a ale predoinan$e in patients who had 4'5 with

PS "2.-9 ale#, although this did not rea$h statisti$al signii$an$e as $opared with the

$ontrol group. 5urtherore, there was no dieren$e in the pre*alen$e o CH+ or in the

distri(ution o $ases (etween the dierent ra$es as $opared with the $ontrol population. -

5



%)+) E7iolo3y

4he $ause o 4etralog o 5allot is un%nown. 4here are ultia$torial etiologF

in$ludes (oth en*ironental and geneti$ a$tors that ost li%el intera$t with one another

in $ertain $ases. A stud ro Portugal reported that ethlene tetrahdroolate redu$tase

"4H5# gene polorphis $an (e $onsidered a sus$epti(ilit gene or tetralog o

allot -

+i =eorge sndroe "$hara$teriEed ( pharingeal dee$ts, hpo$al$eia due to

a(sent parathroid gland, and 4 $ell dsun$tion se$ondar hipoplasia o the thus# is

asso$iated with $ongenital a(noralities o the $ardia$ outlow tra$t, in$luding tetralog

o allot, trun$us arteriosus, and interupted aorti$ ar$h. ost patients with +i=eorge

sndroe ha*e a i$rodeletion within $hroosoe && "&&11#, a region that $ontains the

4BG1 gene. 4his gene en$odes a trans$ription a$tor that appears to pla $riti$al role in

de*elopent patterning o the $ardia$ outlow tra$ts1

Prenatal a$tor asso$iated with a higher insiden$e o tetralog o allot in$lude

aternal ru(ella "or other *iral ine$tion# during pregnan$, poor prenatal nutrition,

aternal al$ohol use, aternal age older than -0 ears, aternal phenl%etonuria (irth

dee$ts, and dia(etes. Se*eral en*ironental teratogens ha*e (een shown spe$ii$all to

in$rease the ris% o de*eloping 4'5 with PS, in$luding aternal dia(etes, retinoi$ a$ids,

aternal phenl%etonuria "P!>#, and triethadione. 4he inant o an o*ertl dia(eti$

other is at a threeold in$reased ris% o de*eloping 4'5 with PS "and at in$reased ris% o

de*eloping other $onotrun$al alorations# as $opared with the inant o a nondia(eti$

other . Siilarl, ingestion o retinoi$ a$ids during the irst triester o pregnan$ is

asso$iated with an in$reased ris% o $ranioa$ial, $ardio*as$ular, and $entral ner*ous

sste dee$ts . 4he ost reuent $ardio*as$ular dee$ts in$lude 4'5. others with P!>

who do not $ontrol their dietar inta%e o phenlalanine during the pregnan$ are also at

in$reased ris% o ha*ing an inant with ultiple anoalies in$luding CH+, o whi$h 4'5

appears to (e one o the ore $oon dee$ts.. 5inall, aternal treatent with

triethadione or paraethadione during the pregnan$ has (een asso$iated with the

de*elopent o ultiple anoalies, in$luding $ardia$ septal dee$ts and 4'5&,3

6



%)( Em5riolo3y

?oral de*elopent o the $onotrun$us in*ol*es proper septation and alignent o

the pulonar and aorti$ outlow tra$ts a(o*e their respe$ti*e *entri$les. 4he e(rologi$

pre$ursors to the *entri$ular outlow tra$ts and great arteries are the distal (ul(us $ordis and

trun$us arteriosus, respe$ti*el. 4he anatoi$ transition point, (etween the (ul(us $ordis and

trun$us arteriosus, $oin$ides with the le*el at whi$h the seilunar *al*es or ro the

growth and usion o the trun$al(ul(ar $ushions. 5or purposes o dis$ussion, this region

en$opassing the distal (ul(us $ordis and trun$us arteriosus will (e reerred to, in the

aggregate, as the $onotrun$us. 3

4he $onotrun$us, in noral de*elopent, is initiall rightwardl situated o*er the

e(rologi$ right *entri$le. 4his region undergoes a spatiall $ople: pro$ess o rotation,

septation, and dierential $ell growth and death that results in the proper alignent o the

outlet septu with the *entri$ular tra(e$ular septu. 4he transition (etween these two

stru$tures is ultiatel spanned and $losed ( the e(ranous septu. 4he net anatoi$

result o this regional orphogenesis is the proper posterior alignent o the let outlow

tra$t with the let *entri$le and esta(lishent o aorti$itral $ontinuit. 4he right *entri$ular

outlow tra$t undergoes siilar ultiate alignent with the right *entri$le. n $ontrast to the

let *entri$ular outlow tra$t, howe*er, the right *entri$ular outlow tra$t retains its us$ular

properties in the or o a su(puloni$ inundi(ulu, or $onus. 3

4he pre$ise ole$ular and de*elopental e$haniss that are responsi(le or the

e*olution o noral $onotrun$al anato reain un$ertain. At the $ellular le*el, the pre$ise

spatial relationships reuired are, in part, or$hestrated ( regional dieren$es in (oth $ell

prolieration and senes$en$e, or apoptosis. Both o these pro$esses ha*e (een shown to (e

a$ti*e during $onotrun$al de*elopent in a*ian e(ros. At a a$ros$opi$ le*el, the

anato seen in 4'5 is (elie*ed to result ro in$oplete rotation and ault partitioning o

the $onotrun$us during septation. 4his pro$ess norall o$$urs ( proper spatial growth and

rotation o the trun$al(ul(ar ridges. alrotation o these ridges results in isalignent o

the outlet and tra(e$ular septu and $onseuent straddling o the aorta o*er the alaligned

*entri$ular septal dee$t. 4he su(puloni$ o(stru$tion, then, is $reated ( a(norall

anterior septation o the $onotrun$us ( the (ul(otrun$al ridges. An alternate e$hanis, put

orth ( @an Praagh, postulates that hpoplasia and underde*elopent o the pulonar

inundi(ulu are responsi(le or the inundi(ular o(stru$tion and alalignent o the outlet

septu. orphoetri$ studies, howe*er, ha*e suggested that the su(puloni$ inundi(ulu

7



in 4'5 is, in an hearts, noral or longer than noral. A $lear e$hanisti$ e:planation or

a(noral $onotrun$al de*elopent thus reains un$ertain3

%)/) A407omy

Although the epon that $arries 5allots nae reers to the tetrad o right

*entri$ular outlow o(stru$tion, aorti$ o*erride, *entri$ular septal dee$t, and right

*entri$ular hpertroph, attepts to ore ull deine whi$h anatoi$ indings are

essential and uniue to 4'5 ha*e generated u$h dis$ussion. t is sae to sa, howe*er,

that the ost $hara$teristi$ and hallar% inding is the su(puloni$ stenosis $reated (

the de*iation o the outlet, or $onal, septu. All patients with 4'5 deonstrate anterior

and $ephalad de*iation o this outlet septu, and the degree and nature o this de*iation

deterine the se*erit o su(puloni$ o(stru$tion. oreo*er, the de*iation o the $onalseptu $an e:plain the su(seuent presen$e o (oth the *entri$ular septal dee$t and the

o*erriding aorta. Be$ause in *irtuall all patients the *entri$ular septal dee$t is large and

nonrestri$ti*e, the right *entri$ular hpertroph is a$$epted to (e se$ondar to the

resultant right *entri$ular hpertension 2

Puloni$ Stenosis

Signii$ant su(puloni$ o(stru$tion e:ists in *irtuall all patients with 4'5.

Pulonar arter pressures are, $onseuentl, noral or low. 5urtherore, additional areas o

o(stru$tion along the entire $ourse o the right *entri$ular outlow tra$t and pulonar

arteries $oonl e:ist. n general, the ore se*ere the pro:ial o(stru$tion, the greater the

li%elihood that other distal areas o o(stru$tion will (e present. n 4'5 with puloni$

stenosis, howe*er, onl a ew patients ha*e prohi(iti*el sall pulonar arteries ro the

perspe$ti*e o surgi$al repair. +istal o(stru$tion to right *entri$ular output a (e present

within the pulonar *al*ular apparatus, supra*al*ular region, and (oth pro:ial and distal

pulonar arterial (ed. n the e:tree $ase o pulonar atresia and @S+, there a (ese*ere hpoplasia, or e*en a(sen$e, o true pulonar arteries. n this setting, pulonar

(lood low is oten pro*ided ( the persisten$e o e(rologi$ aortopulonar $ollateral

arteries. 3

Su(puloni$ '(stru$tion

4he su(puloni$, or inundi(ular, o(stru$tion in 4'5 is $hara$teriEed ( anterior and

$ephalad de*iation o the outlet, or inundi(ular, septu. 4his de*iation o the outlet septu

8



results in us$ular su(*al*ular narrowing. 4he o(stru$tion is urther e:a$er(ated (

hpertroph o the us$ular outlet septu, the parietal right *entri$ular ree wall, and

$oponents o the septoarginal tra(e$ulations. Anatoi$all, the outlet septu is norall

situated within the li(s o the septoarginal tra(e$ulations and is aligned with the

tra(e$ular septu that partitions the *entri$ular $a*ities. 4his transition Eone (etween the

outlet and tra(e$ular septu is norall $losed ( esen$hal tissue and represents the

area o the perie(ranous *entri$ular septu. 4he antero$ephalad de*iation o the outlet

septu, while resulting in us$ular o(stru$tion, also siultaneousl gi*es rise to the large

perie(ranous *entri$ular septal dee$t ( *irtue o the alalignent (etween the outlet

and tra(e$ular septu.

'(stru$tion within the right *entri$ular (od also a (e present. 4here a (e

hpertroph o the septoparietal us$le (undles with urther e:tension o us$le to the right

*entri$ular ree wall. n addition, anatoi$ displa$eent o the noral oderator (and

atta$hent is thought to $ontri(ute to intra$a*itar o(stru$tion pro:ial to the inundi(ulu.

4his us$ular o(stru$tion is reerred to as anoalous right *entri$ular us$le (undles or (

the anatoi$ des$ription, dou(le$ha(ered right *entri$le. 4his intra$a*itar o(stru$tion

a (e present prior to surger, (ut it also $an e*ol*e ollowing surgi$al $orre$tion. A

retrospe$ti*e, ediuter ollowup stud ound that this tpe o o(stru$tion e*ol*ed in

appro:iatel 39 o patients ollowing su$$essul initial surger.

Pulonar @al*ular and Arterial Anato

n addition to the su(puloni$ o(stru$tion, additional areas o stenoses are $oon

at the *al*ular and supra*al*ular le*els. 4he pulonar *al*e is $oonl sall and

stenoti$. n a stud ( ao et al., the pulonar *al*e was ound to (e either (i$uspid or

uni$uspid in a a)orit o patientns. oreo*er, there a (e dis$rete supra*al*ular puloni$

o(stru$tion at the le*el o the atta$hents o the pulonar lealets.

4he pulonar arteries are also prone to o$al or diuse o(stru$tion or hpoplasia.

Bran$h pulonar arter o(stru$tion, pro:iall or distall, is $oon. n $ases o PA

deonstrating the a(sen$e o antegrade pulonar output, true e(rologi$ pulonar

arteries are oten a(sent or se*erel hpoplasti$. n this situation, pulonar (lood low is

pro*ided ( the persisten$e o aortopulonar $ollateral arteries. n the setting o siple

4'5, with esta(lisheantegrade low into the pulonar arteries, these aortopulonar

$ollateral arteries are un$oon and less $ople: in distri(ution than with PA.

9



ost $oonl seen with the let pulonar arter, pulonar arter anato a

(e urther $opli$ated ( narrowing or atresia o a (ran$h pulonar arter. Bran$h

pulonar arter atresia is ost $oonl re$ogniEed in patients with pulonar *al*e

atresia, (ut narrowing is not un$oon in $hildren with antegrade pulonar low. t a

de*elop postnatall as a result o the $losure o the du$tus arteriosus and the su(seuent

distortion o its insertion site as the du$tal tissue in*olutes. 3,2

@entri$ular Septal +ee$t

4he *entri$ular septal dee$t in 4'5 ost reuentl has i(rous $ontinuit (etween the

tri$uspid and aorti$ *al*e, and hen$e a (e $onsidered a true perie(ranous dee$t. 4his

tpe o anato was do$uented in -& o 23 autops spe$iens with 4'5 . n the reaining

spe$iens, a ri o us$ular tissue was present along the posterior and inerior ri o thedee$t. n this situation, there is tri$uspidaorti$ dis$ontinuit. n either s$enario, howe*er, the

*entri$ular septal dee$t arises as a result o the antero$ephalad de*iation o the outlet septu

and lies in a su(arterial lo$ation.

n addition to the isolated large su(arterial dee$t, additional *entri$ular septal dee$ts

also a (e present o$$asionall. 4here a (e inlet e:tension o the su(arterial dee$t, or, in

soe patients, there a (e an asso$iated $oplete atrio*entri$ular septal dee$t. Although

the *entri$ular septal dee$t is large and nonrestri$ti*e ( deinition, a ew patients a ha*e

restri$ti*e dee$ts. n these patients, howe*er, restri$tion results ro the presen$e o

a$$essor or redundant tri$uspid *al*e tissue. 4he a$$essor tissue atta$hes to the *entri$ular

septal $rest or prolapses into the dee$t, resulting in o(stru$tion. Although $hildren with

supra$ristal @S+ with *al*ar puloni$ stenosis or id$a*itar su(puloni$ stenosis la$% the

$hara$teristi$ anterior de*iation o the $onal septu, the phsiologi$ out$oe is within the

spe$tru o 4'5.

Aorti$ '*erride

4he signii$an$e o aorti$ o*erride priaril relates to terinolog and in distinguishing

whether the anatoi$ entit in uestion is ore appropriatel deeed to (e dou(leoutlet

right *entri$le or 4'5. 4his issue a (e a*oided i the orphologi$ deinition o dou(le

outlet right *entri$le is adopted. n this approa$h, dou(leoutlet right *entri$le denotes the

a(sen$e o aorti$itral $ontinuit and reuires the presen$e o (oth a su(aorti$ and

su(puloni$ us$ular $onus. using this deinition, and a*oiding uestions o whether the

aorta is 209 $oitted to the right *entri$le, the diagnoses o 4'5 and dou(leoutlet right

10



*entri$le (e$oe utuall e:$lusi*e. n an e*ent, deterination o the e:a$t degree o

$oitent o the aorta to either the right or let *entri$le is soewhat su()e$ti*e and a

*ar with the iaging odalit used.

t is $lear, howe*er, that aorti$ alposition in 4'5 is an anatoi$ realit. 4he degree

o aorti$ o*erride in one e$ho$ardiographi$ stud was in the range o 129 to 629 . n an

anatoi$all noral heart, the right aorti$ sinus does o*erlie the noral plane o the

*entri$ular septu. n the setting o a *entri$ular septal dee$t, then, the ipression o soe

straddling o the aorta o*er the dee$t would (e present. 4his o*erride is urther a$$entuated

( the alaligned nature o the *entri$ular septal dee$t. +ilation o the aorta, whi$h li%el is

related to the alseptation o the $onotrun$us in 4'5, urther $ontri(utes to the ipression o

an aorta $oitted to (oth *entri$ular outlow tra$ts. 5inall, the aorti$ position does e:hi(it

additional rotational $hanges, with rotation o the right aorti$ sinus toward a ore let and

anterior orientation than usual 3,2

%)9) Cir1ul07io4 04d P07opisiolo3y Te7r0lo3y 04d F0llo7

4he $ir$led nu(ers represent o:gen saturation *alues. 4he nu(ers the ne:t arrows

represent *olues de$reased (e$ause o the sistei$ hpo:eia. A *olue o 3 /in/3

o desaturated (lood enters the right atriu and tra*erses the tri$uspid *al*e. 4wo liters

lows through the right *entri$ular outlow tra$t into the lungs, whereas 1 shunts right to

11



let throught the *entri$ular septal dee$t "@S+# into the as$ending aorta. 4hus, pulonar

(lood low into thirds noral "Ip; Is "pulonar to sstei$ (lood low ratio# o 0.7;1#.

Blood returning to the let atriu is ull saturated. 'nl & o (lood lows a$ross the

itral *al*e ':gen saturation in the let *entri$le a (e slightl de$reased (e$ause the

right to let shunting a$ross the @S+. 4wo liters o saturated let *entri$ular (lood i:ing

with 1 o desaturated right *entri$ular (lood is e)e$ted into the as$ending aorta. Aorti$

saturation is de$reased, and $ardia$ output is noral.

4he se*erit o tetralog o allot (ased ( o(stru$tion outlow o right *entri$ular

"pulonal stenosis#. ore se*ere pulonar stenosis , then a lot o (lood ro the right

*entri$le into the aorta. n ild stenosis, (lood ro right *entri$le low to pulonal, and

shunt ro right to let o$$urs onl in phsi$al a$ti*it. 4he pulonar *al*e annulus

a (e o nearl noral siEe or uite sall. 4he *al*e itsel is oten (i$uspid and,

o$$asionall, is the onl site o stenosis. ore $oonl, the su(puloni$ us$le, the

$rista supra*entri$ularis, is hpertrophi$, whi$h $ontri(utes to the inundi(ular stenosis

and results in an inundi(ular $ha(er o *aria(le siEe and $ontour. Dhen the right

*entri$ular outlow tra$t is $opletel o(stru$ted "pulonar atresia#, the anato o the

(ran$h pulonar arteries is e:treel *aria(leF a ain pulonar arter segent a (e

in $ontinuit with right *entri$ular outlow, separated ( a i(rous (ut iperorate

pulonar *al*e, or the entire ain pulonar arter segent a (e a(sent.

'$$asionall, the (ran$h pulonar arteries a (e dis$ontinuous. n these ore se*ere

$ases, pulonar (lood low a (e supplied ( a patent du$tus arteriosus "P+A# and (

major aortopulmonary collateral arteries (MAPCAs) arising ro the aorta.

4he @S+ is usuall nonrestri$ti*e and large, is lo$ated )ust (elow the aorti$ *al*e,

and is related to the posterior and right aorti$ $usps. arel, the @S+ a (e in the inlet

portion o the *entri$ular septu "atrio*entri$ular septal dee$t#. 4he noral i(rous

$ontinuit o the itral and aorti$ *al*es is usuall aintained. 4he aorti$ ar$h is right

sided in &09, and the aorti$ root is usuall large and o*errides the @S+ to a *aring

degree. Dhen the aorta o*errides the @S+ ore than 209 and i us$le is signii$antl

separating the aorti$ *al*e and the itral annulus "su(aorti$ $onus#, this dee$t is usuall

$lassiied as a or o double-outlet right ventricle; the pathophsiolog is the sae as

that or tetralog o 5allot. -

Sstei$ *enous return to the right atriu and right *entri$le is noral. Dhen the

right *entri$le $ontra$ts in the presen$e o ar%ed pulonar stenosis, (lood is shunted

a$ross the @S+ into the aorta. Persistent arterial desaturation and $anosis result.

12



Pulonar (lood low, when se*erel restri$ted ( the o(stru$tion to right *entri$ular

outlow, a (e suppleented ( the (ron$hial $ollateral $ir$ulation "APCAs# and, in

the new(orn, ( a P+A. Pea% sstoli$ and diastoli$ pressures in ea$h *entri$le are siilar

and at the sstei$ le*el. A large pressure gradient o$$urs a$ross the o(stru$ted right

*entri$ular outlow tra$t, and pulonar arterial pressure is noral or lower than noral.

4he degree o right *entri$ular outlow o(stru$tion deterines the tiing o the onset o

sptos, the se*erit o $anosis, and the degree o right *entri$ular hpertroph. Dhen

o(stru$tion to right *entri$ular outlow is ild to oderate and a (alan$ed shunt is present

a$ross the @S+, the patient a not (e *isi(l $anoti$ (acyanotic or “pink tetralogy o!

"allot).

%);) Cli4i10l M04i8es707io4s

nants with ild degrees o right *entri$ular outlow o(stru$tion a initiall (e

seen with heart ailure $aused ( a *entri$ularle*el lettoright shunt. 'ten, $anosis is not

present at (irth, (ut with in$reasing hpertroph o the right *entri$ular inundi(ulu and

patient growth, $anosis o$$urs later in the 1st r o lie. t is ost proinent in the u$ous

e(ranes o the lips and outh and in the ingernails and toenails. n inants with se*ere

degrees o right *entri$ular outlow o(stru$tion, neonatal $anosis is noted iediatel. n

these inants, pulonar (lood low a (e dependent on low through the du$tus arteriosus.

Dhen the du$tus (egins to $lose in the 1st ew hours or das o lie, se*ere $anosis and

$ir$ulator $ollapse a o$$ur. 'lder $hildren with longstanding $anosis who ha*e not

undergone surger a ha*e dus% (lue s%in, gra s$lerae with engorged (lood *essels, and

ar%ed clubbing o the ingers and toes.

#yspnea o$$urs on e:ertion. nants and toddlers pla a$ti*el or a short tie and

then sit or lie down. All o these $ases are dspnea to a greater or lesser degree, depending

largel on the adeua$ o the (lood low to the lungs 7.

'lder $hildren a (e a(le to wal% a (lo$% or so (eore stopping to rest.

Chara$teristi$all, $hildren assue a s$uatting position or the relie o dspnea $aused (

phsi$al eortF the $hild is usuall a(le to resue phsi$al a$ti*it within a ew inutes.

4hese indings o$$ur ost oten in patients with signii$ant $anosis at rest. 4he ee$t o

the a$ute le:ion at %nee and thigh a (e to trap (lood in the legs and so redu$e the load o

returning *enous (lood to the heart. 'n the other hand, ( in$reasing the sstei$ *as$ular

resistan$e the ee$t a (e to di*ert ore (lood ro the aorta to the lungs3

.

13



Paro%ysmal hypercyanotic attacks (hypo%ic& “blue& or “tet spells) are a parti$ular

pro(le during the 1st & r o lie. 4he inant (e$oes hperpnei$ and restless, $anosis

in$reases, gasping respirations ensue, and sn$ope a ollow. 4he spells o$$ur ost

reuentl in the orning on initiall awa%ening or ater episodes o *igorous $ring.

4eporar disappearan$e or a de$rease in intensit o the sstoli$ urur is usual as low

a$ross the right *entri$ular outlow tra$t diinishes. 4he spells a last ro a ew inutes

to a ew hours (ut are rarel atal. Short episodes are ollowed ( generaliEed wea%ness and

sleep. Se*ere spells a progress to un$ons$iousness and, o$$asionall, to $on*ulsions or

heiparesis. 4he onset is usuall spontaneous and unpredi$ta(le. Spells are asso$iated with

redu$tion o an alread $oproised pulonar (lood low, whi$h when prolonged results

in se*ere sstei$ hpo:ia and eta(oli$ a$idosis. nants who are onl ildl $anoti$ at

rest are oten ore prone to the de*elopent o hpo:i$ spells (e$ause the ha*e not

a$uired the hoeostati$ e$haniss to tolerate rapid lowering o arterial o:gen saturation,

su$h as pol$theia.

'roth and development a (e delaed in patients with se*ere untreated tetralog

o 5allot, parti$ularl when o:gen saturation is $hroni$all less than 709. Pu(ert a also

(e delaed in patients who do not undergo surger. 4he pulse is usuall noral, as is *enous

and arterial pressure. 4he let anterior heithora: a (ulge anteriorl (e$ause o right

*entri$ular hpertroph. 4he heart is generall noral in siEe, and a substernal right

ventricular impulse $an (e dete$ted. n a(out hal the $ases, a systolic thrill is elt along the

let sternal (order in the 3rd and -th parasternal spa$es. 4he systolic murmur is usuall loud

and harshF it a (e transitted widel, espe$iall to the lungs, (ut is ost intense at the let

sternal (order. 4he urur is generall e)e$tion in ualit at the upper sternal (order, (ut it

a sound ore holosstoli$ toward the lower sternal (order. t a (e pre$eded ( a $li$%.

4he urur is $aused ( tur(ulen$e through the right *entri$ular outlow tra$t. t tends to

(e$oe louder, longer, and harsher as the se*erit o pulonar stenosis in$reases ro ild

to oderateF howe*er, it $an a$tuall (e$oe less proinent with se*ere o(stru$tion,

espe$iall during a hper$anoti$ spell. ither the &nd heart sound is single, or the puloni$

$oponent is sot. nreuentl, a $ontinuous urur a (e audi(le, espe$iall i

proinent $ollaterals are present.

14



%). Di034osis

%).)' A47e4070l Di034osis

4etralog o 5allot $an (e diagnosed antenatall as earl as 1& wee%s o gestation. n a

population (ased stud, howe*er, onl hal o the $ases were dete$ted douring routine

o(stetri$ ultrasoni$ s$reening. n general, patients who are reerred or etal e$ho$ardiograph

with a suspi$ion o tetralog o allot ha*e the ost se*ere phenotpe. 'ther reasons or

reerral or oetal e$ho$ardiograph in$lude dis$o*er o e:tra$ardia$ alorations, or

%nown $hroosoal a(noralities. As a result, patients reerred or oetal e$ho$ardiograph

tends to ha*e worse out$oes when $opared to patients who are diagnosed postnatall. 4he

etus with tetralog $an (e deli*ered *aginall (ut eorts should (e ade or deli*err to

o$$ur in a $entre where pediatri$ $ardiologist are a*alai(le to aid in the postnatal $are 3.

%).)% A40m4esis

4he ain $oplaint patients usuall $anosis, dspnea, 4he $lini$al eatures o

tetralog o 5allot "4'5# are dire$tl related to the se*erit o the anatoi$ dee$ts. ost

inants with tetralog o 5allot ha*e dii$ult with eeding, and ailure to thri*e "544# is

$oonl o(ser*ed. nants with pulonar atresia a (e$oe prooundl $anoti$ as the

du$tus arteriosus $loses unless (ron$hopulonar $ollaterals are present. '$$asionall, soe

$hildren ha*e )ust enough pulonar (lood low and do not appear $anoti$F these

indi*iduals reain asptoati$, until the outgrow their pulonar (lood suppl. 7

At (irth, soe inants with tetralog o 5allot do not show signs o $anosis, (ut the

a later de*elop episodes o (luish pale s%in during $ring or eeding "ie, <4et< spells#.

Hpo:i$ tet spells are potentiall lethal, unpredi$ta(le episodes that o$$ur e*en in

non$anoti$ patients with tetralog o 5allot. 4he e$hanis is thought to in$lude spas o

the inundi(ular septu, whi$h a$utel worsens the right *entri$ular "@# outlow tra$t

o(stru$tion "@'4'#. &,3

A $hara$teristi$ ashion in whi$h older $hildren with tetralog o 5allot in$rease

pulonar (lood low is to suat. Suatting is a $opensator e$hanis, o diagnosti$

signii$an$e, and highl tpi$al o inants with tetralog o 5allot. Suatting in$reases

peripheral *as$ular resistan$e "P@# and thus de$reases the agnitude o the righttolet

shunt a$ross the *entri$ular septal dee$t "@S+#. :ertional dspnea usuall worsens with

age. '$$asionall, heoptsis due to rupture o the (ron$hial $ollaterals a result in the

older $hild. 4he rare patient a reain arginall and iper$epti(l $anoti$, or a$anoti$

15



and asptoati$, into adult lie.Canosis generall progresses with age and outgrowth o

pulonar *as$ulature and deands surgi$al repair.3,7

%).),) Pysi10l E<0mi407io4

ost inants with tetralog o 5allot "4'5# are saller than espe$ted or age. Canosis o

the lips and nail (ed is usuall pronoun$ed at (irth, ater age 3 onths, the ingers and

toes show $lu((ing.

5ingger $lu((ing is a $harateristi$ eature o the $ondition, the degree o $lu((ing

is usall proportional to the se*erit o the $anosis. 4he toes also show $lu((ing and in

se*erel $anoti$ $ases the tip o the nose also a (e $lu((ed. 7

A sstoli$ thrill is usuall present anteriorl along the let sternal (order. A harsh

sstoli$ e)e$tion urur is heard o*er the puloni$ area and let sternal (order. Dhen the

right *entri$ular outlow tra$t o(stru$tion is oderate, the urur a (e inaudi(le. 4he

S& is usuall singgle dissappear, whi$h is suggesti*e o lessened @ outlow to the

pulonar arteries. n indi*iduals with aortopulonar $ollaterals, $ontinuous ururs

a (e aus$ultated. 4hus, an a$anoti$ patient with tetralog o allot "pin% tet# has a long,

loud, sstoli$ urur with a thrill along the @'4. 3,

%).)+ L05or07orium E<0mi407io4

Heoglo(in and heato$rit *alues are usuall ele*ated in proportion to the degree

$anosis. Prolonged sanosis $auses rea$ti*e pol$theia that in$reases o:gen$arring

$apa$it. 4he o:gen saturation in sstei$ arterial (lood tpi$all *aries ro 2709.

All patients with tetralog o 5allot who e:prian$e signii$ant $anosis ha*e a tenden$ to

(leed (e$ause o de$reassed $lotting a$tors and low platelet $ount. Hper*is$osit and

$oagulapath oten ensue and are parti$ular deleterious in patients with a right to let

intra$ardia$ shunt. 4he usual indings are deinshed $oagulation a$tors and diinished

total i(rinogen, whi$h are asso$iated with prolonged prothro(in and $oagulation ties.

Arterial (lood gas results show *aring o:gen saturation, (ut ph and partial

pressure o $ar(on dio:ide "P$o&# are noral, unless the patient is in e:treis, su$h as

during a tet spell. 3,7

%).)( R0dio3r0py

4he total heart siEe is usuall noral on $hest roentgenograph, (ut right *entri$ular

enlargeent is present in the lateral *iew. 4he aorta ar$hes to the right in an $ases.

16



Pulonar low is diinished. 4he pulonar segent is $on$a*e and the ape: is

ele*ated, gi*ing the $oeur en sa(ot "(ootshaped# $ontour. A *er oung inant a ha*e

onl diinished pulonar low. 3,

Pi$ture 1 oentgenogra o an 8rold (o with the tetralog o 5allot

%).)/ Ele17ro10rdio3r0m

!= in neonates are not dierent ro noral $hildren. 4he ele$tro$ardiogra

deonstrates right a:is de*iation and e*iden$e o right *entri$ular hpertroph. A doinant

wa*e appears in the right pre$ordial $hest leads or an S pattern. n soe $ases, the onl

sign o right *entri$ular hpertroph a initiall (e a positi*e 4 wa*e in leads @3 and @1.

4he P wa*e is tall and pea%ed or soeties (iid. ,7

%).)9) E1o10rdio3r0py

$ho$ardiograph is a gold standart or diagnosis tetralog o allot. o-dimensional

echocardiography esta(lishes the diagnosis and pro*ides inoration a(out the e:tent o

aorti$ o*erride o the septu, the lo$ation and degree o the right *entri$ular outlow tra$t

o(stru$tion, the siEe o the pro:ial (ran$h pulonar arteries, and the side o the aorti$

ar$h. 4he e$ho$ardiogra is also useul in deterining whether a P+A is suppling a portion

o the pulonar (lood low. t a o(*iate the need or $atheteriEation. 3,,7

17



Pi$ture & $ho$ardiogra in a patient with the tetralog o 5allot. 4his shorta:is,

su(:iphoid, twodiensional e$ho$ardiographi$ pro)e$tion deonstrates anterior/superior

displa$eent o the outlow *entri$ular septu that resulted in stenosis o the su(puloni$

right *entri$ular outlow tra$t and an asso$iated anterior *entri$ular septal dee$t "@S+#. @ right *entri$leF A' o*erriding aorti$ *al*eF @ let *entri$le.

%).); C0rdi01 C07e7eri=07io4 04d A43io3r0py

4here is a higher than usual ris% asso$iated with $ardia$ $athteriEation (e$ause o

the reuen$ o rhth distur(an$es. Proper pre$autions and propt use o $ardio*ersion

when ne$esser iniiEe this ris%. n ost $ases, e$ho$ardiograph and $olor +oppler

e*aluation are sui$ient, and $atheteriEation is perored less $oonl than it was

pre*iousl. 7

Cardia$ $atheteriEation was not reuired when palliati*e surger li%e surger Blalo$%

4ausig. CatheteriEation was perored (eore surger ais to deterine the ultiple

*entri$ular septal dee$ts "29# , a(noralities o the $oronar arteries "29# and pulonar

stenosis "&89# 8

Cardiac catheteri*ation deonstrates a sstoli$ pressure in the right *entri$le eual to

sstei$ pressure. the pulonar arter is entered, the pressure is ar%edl de$reased,

although $rossing the right *entri$ular outlow tra$t, espe$iall in se*ere $ases, a

pre$ipitate a tet spell. Pulonar arterial pressure is usuall lower than noral, in the range

o 2K10 Hg. 4he le*el o arterial o:gen saturation depends on the agnitude o the

righttolet shuntF in Lpin% tets,M sstei$ saturation a (e noral, whereas in a

oderatel $anoti$ patient at rest, it is usuall 72K829.

+elective right ventriculography (est deonstrates the anato o the tetralog o

5allot. Contrast ediu outlines the hea*il tra(e$ulated right *entri$le. 4he inundi(ular

stenosis *aries in length, width, $ontour, and distensi(ilit. 4he pulonar *al*e is usuall

18



thi$%ened, and the annulus a (e sall. n patients with pulonar atresia and @S+, the

anato o the pulonar *essels a (e e:treel $ople:, or e:aple, dis$ontinuit

(etween the right and let pulonar arteries. Coplete and a$$urate inoration regarding

the anato o the pulonar arteries is iportant when e*aluating these $hildren as surgi$al

$andidates. 7

,e!t ventriculography deonstrates the siEe o the let *entri$le, the position o the

@S+, and the o*erriding aortaF it also $onirs itralaorti$ $ontinuit, there( ruling out a

dou(leoutlet right *entri$le. 7

Aortography or coronary arteriography outlines the $ourse o the $oronar arteries. n

2K109 o patients with the tetralog o 5allot, an a(errant a)or $oronar arter $rosses o*er

the right *entri$ular outlow tra$tF this arter ust not (e $ut during surgi$al repair.

@erii$ation o noral $oronar arteries is iportant when $onsidering surger in oung

inants who a need a pat$h a$ross the pulonar *al*e annulus. $ho$ardiograph a

delineate the $oronar arter anatoF angiograph is reser*ed or $ases in whi$h uestions

reain.7

%)'&) Tre07me47

4reatent o the tetralog o 5allot $onsisted o edi$al treatent and surger. 4wo

anangeent is supported ea$h other, edi$al anageent is ne$essar or preparation o

preoperati*e and postoperati*e surger.

4reatent o the tetralog o 5allot depends on the se*erit o the right *entri$ular

outlow tra$t o(stru$tion. nants with se*ere tetralog reuire edi$al treatent and surgi$al

inter*ention in the neonatal period. 4herap is aied at pro*iding an iediate in$rease in

pulonar (lood low to pre*ent the seuelae o se*ere hpo:ia. 4he inant should (e

transported to a edi$al $enter adeuatel euipped to e*aluate and treat neonates with

$ongenital heart disease under optial $onditions. t is $riti$al that o:genation and noral

(od teperature (e aintained during the transer. Prolonged, se*ere hpo:ia a lead to

sho$%, respirator ailure, and intra$ta(le a$idosis and will signii$antl redu$e the $han$e o

sur*i*al, e*en when surgi$all aena(le lesions are present. Cold in$reases o:gen

$onsuption, whi$h pla$es additional stress on a $anoti$ inant, whose o:gen deli*er is

alread liited. Blood glu$ose le*els should (e onitored (e$ause hpogl$eia is ore

li%el to de*elop in inants with $anoti$ heart disease. 7

19



%)'&)') Hyper1y04o7i1 Spell

anageent o patients with hipersianosis spel in whi$h patients should (e pla$ed the inant

on the a(doen in the %nee$hest position while a%ing $ertain that the inants $lothing is

not $onstri$ti*e, . t ais to in$rease sstei$ *as$ular resitensi and lowering the sstei$

*enous return.

':gen is gi*en to redu$e the peripheral pulonar *aso$onstri$tion and in$rease

o:genation to the lungs ater (lood low to the lungs is (alan$ed. n)e$tion o orphine

su($utaneousl in a dose not in e:$ess o 0.& g/%g. Caling and holding the inant in a

%nee$hest position a a(ort progression o an earl spell. Preature attepts to o(tain

(lood saples a $ause urther agitation and (e $ounterprodu$ti*e.7

Be$ause eta(oli$ a$idosis de*elops when arterial P'& is less than -0 Hg, rapid

$orre$tion "within se*eral inutes# with intra*enous adinistration o sodiu (i$ar(onate is

ne$essar i the spell is unusuall se*ere and the $hild shows a la$% o response to the

oregoing therap. e$o*er ro the spell is usuall rapid on$e the pH has returned to

noral. epeated (lood pH easureents a (e ne$essar (e$ause rapid re$urren$e o

a$idosis a ensue. 8

4he a(o*e treatent is e:pe$ted that $hildren no longer ta$hpnea , redu$ed $anosis

and the $hild (e$oes uiet . does not happen , it $an (e $ontinued with this anageent ;

n)e$tion NAdrenergi$ (lo$%ade ( the intra*enous adinistration o propranolol

"0.01 g/%gBB 0.0&2 g/%gBB gi*en slowl#. 4otal dose re$onstituted with 10 l o

liuid in the sringe . nitial dose is gi*en hal with i* (olus . the atta$% is not

resol*ed , gi*e the rest graduall within 2 to 10 inutes . At ea$h in)e$tion

propanalol , isoprotenol should (e prepared to $ope with an o*erdose

!etain 13 g/%gBB "the ean & g/%gBB# @ graduall "0 se$onds#. !etain

wor%s ( in$reasing sstei$ *as$ular resistan$e and as a sedati*e.

+rugs that in$rease sstei$ *as$ular resistan$e, intra*enous phenlephrine 0.0&

g/%gBB, ipro*e right *entri$ular outlow, de$rease the righttolet shunt, and thus

ipro*e the sptos.

Awarding (od luid *olue with intra*enous luids $an (e ee$ti*e in the treatent

o atta$%s o $anosis. (lood *olue $ould ae$t the le*el o o(stru$tion . 4he

addition o the (lood *olue $an also in$rease $ardia$ output , there( in$reasing

(lood low to the lungs and sstei$ (lood low $arring o:gen throughout the (od

also in$reases.8

20



%)'&)% I48047 04d 1ild >i7 1y04osis is7ory

nants with less se*ere right *entri$ular outlow tra$t o(stru$tion who are sta(le and

awaiting surgi$al inter*ention reuire $areul o(ser*ation. Pre*ention or propt treatent o

dehdration is iportant to a*oid heo$on$entration and possi(le thro(oti$ episodes.

Paro:sal dspnei$ atta$%s in inan$ or earl $hildhood a (e pre$ipitated ( a relati*e

iron dei$ien$F iron therap a de$rease their reuen$ and also ipro*e e:er$ise

toleran$e and general well(eing. ed (lood $ell indi$es should (e aintained in the

noro$ti$ range. 'ral propranolol "0.2K1 g/%g e*er hr# a de$rease the reuen$ and

se*erit o hper$anoti$ spells, (ut with the e:$ellent surger a*aila(le, surgi$al treatent is

indi$ated as soon as spells (egin.

nants with sptos and se*ere $anosis in the 1st o o lie ha*e ar%ed

o(stru$tion o the right *entri$ular outlow tra$t or pulonar atresia. 4wo options are

a*aila(le in these inants; the irst is a palliati*e sstei$toKpulonar arter shunt

perored to augent pulonar arter (lood low. 4he rationale or this surger, pre*iousl

the onl option or these patients, is to de$rease the aount o hpo:ia and ipro*e linear

growth, as well as augent growth o the (ran$h pulonar arteries. 4he se$ond option is

$orre$ti*e open heart surger perored in earl inan$ and e*en in the new(orn period in

$riti$all ill inants. 4his approa$h has gained ore widespread a$$eptan$e as e:$ellent short

and interediateter results ha*e (een reported. 4he ad*antages o $orre$ti*e surger in

earl inan$ *s a palliati*e shunt and $orre$tion in later inan$ are still (eing de(ated. n

inants with less se*ere $anosis who $an (e aintained with good growth and a(sen$e o

hper$anoti$ spells, priar repair is perored ele$ti*el at (etween - and 1& o o age.

nants with ar%ed right *entri$ular outlow tra$t o(stru$tion a deteriorate rapidl

(e$ause as the du$tus arteriosus (egins to $lose, pulonar (lood low is urther

$oproised. 4he intra*enous adinistration o prostaglandin 1 "0.02K0.&0 g/%g/in#, a

potent and spe$ii$ rela:ant o du$tal sooth us$le, $auses dilatation o the du$tus

arteriosus and usuall pro*ides adeuate pulonar (lood low until a surgi$al pro$edure $an

(e perored. 4his agent should (e adinistered intra*enousl as soon as $anoti$ $ongenital

heart disease is $lini$all suspe$ted and $ontinued through the preoperati*e period and during

$ardia$ $atheteriEation. Postoperati*el, the inusion a (e $ontinued (riel as a pulonar

*asodilator to augent low through a palliati*e shunt or through a surgi$al *al*uloto. ,7,8

%)'&), I47erve47io40l C07e7eri=07io4 Pro1edures

21



ost inter*entional pro$edures, when perored or patients with 4'5, are underta%en owing

to two general indi$ations; elie o *arious le*els o pulonar o(stru$tion and

e(oliEation o a$$essor and dupli$ated sour$es o pulonar (lood low. 4he reuen$

and indi$ations or $atheter(ased inter*ention are to a large degree deterined ( $lini$ian

and institutional preeren$es, whi$h are then weighed against the relati*e ris%s and (eneits o

surgi$al inter*ention at an gi*en age. n the preoperati*e setting, palliation o signii$ant

$anosis ( (alloon *al*uloplast or right *entri$ular outlow tra$t stent pla$eent has (een

ad*o$ated ( soe as a eans or redu$ing sptoati$ $anosis in patients with se*ere

annular hpoplasia pro*eent in antegrade low is thought to siultaneousl enhan$e

pulonar arterial growth ( augenting pulonar (lood low. 4he indi$ation or palliation

in this setting assues that deiniti*e surgi$al inter*ention a (e done ore sael and

appropriatel at an older age. Siilarl, this approa$h a*oids an possi(le surgi$al

$opli$ations and or pulonar arter distortion that a (e seen ollowing a odiied

Blalo$%ae 4hoase 4aussig shunt. Coil e(oliEation o aortopulonar $ollateral arteries is

also an appropriate inter*ention prior to surgi$al $orre$tion. Coiling o *essels that peruse

pulonar segents alread supplied ( pulonar arterial low ser*es to redu$e let

*entri$ular *olue loading as well as to eliinate runo into the pulonar arterial (ed

during $ardiopulonar (pass.

n the postoperati*e setting, (alloon angioplast in$luding with $utting (alloons and

stenting pro*ide iportant tools with whi$h to address an residual pulonar arterial

o(stru$tion, espe$iall distal o(stru$tion not readil a$$essi(le ro a edian sternoto.

Su$$ess rates or these pro$edures are su(stantial with o*erall low or(idit. ntraarterial

stent pla$eent a (e used when siple angioplast pro*ides inadeuate relie. 4his

usuall is (e$ause o *essel re$oil, whi$h pre$ludes sustained relie o stenosis with

angioplast alone.7

Sur3i10l I47erve47io4

=i*en the trend toward earlier $oplete repair or 4'5, the reuen$ with whi$h palliati*e

pro$edures su$h as the odiied Blalo$%ae 4hoasae 4aussig shunt are perored has

de$reased. 4here are potential short$oings with peroring an initial palliati*e pro$edure,

in$luding pulonar arter distortion, additional *entri$ular *olue loading, and the surgi$al

ris% attendant with a thora$oto. pro*eents in the $oprehensi*e surgi$al approa$h

ha*e led to the assertion ( soe (ut not all $enters that all patients with siple 4'5 should

(e a(le to undergo priar repair without additional palliati*e pro$edures . :$eptions to this

approa$h ight in$lude neonates with se*ere pulonar arter hpoplasia and soe patients

22



with an a(errant $ourse o the anterior des$ending $oronar arter ro the right $oronar

arter.

Surgi$al $orre$tion o 4'5 is dire$ted at relie*ing all possi(le sour$es o right

*entri$ular outlow tra$t o(stru$tion. anatoi$all and surgi$all possi(le, pulonar

*al*e un$tion is preser*ed ( a*oiding a transannular pat$h. Cardiopulonar (pass is

initiated through a edian sternoto. +eep hpotheria with $ir$ulator arrest is usuall

not needed e*en in inants. n older patients, $orre$tion a (e perored using oderate

hpotheria. 5or purposes o right *entri$ular outlow tra$t pat$hing, glutaraldehdetreated

peri$ardiu a (e used and is har*ested while $ooling ta%es pla$e. Alternati*el, either

sntheti$ pat$h aterial a (e used.

Ater $ooling, the aorta is $ross$laped and $ardioplegi$ solution is gi*en. A *erti$al

inundi(ular and right *entri$ular in$ision is then ade. the pulonar annulus is

prohi(iti*el hpoplasti$, then the in$ision is $arried a$ross the annular *al*ular apparatus.

the pulonar annulus is o adeuate siEe, then the annulus a (e spared. 4he de$ision to

pla$e a transannular pat$h rests, in part, on appearan$e and on the su()e$ti*e ipression o

the surgeon at the tie o operation. A preoperati*e O *alue or the pulonar *al*e annulus

o& $orrelated with an ele*ated postoperati*e right;let *entri$ular pressure ratio in a series

( !ir%lin et al.. 4heir re$oendations were that a transannular pat$h (e used or patients

with this e:tent o annular hpoplasia. 4he in$ision in the pulonar arter a urther (e

e:tended onto either (ran$h pulonar arter i needed to relie*e an additional stenosis.

:posure through the *entri$uloto also allows or rese$tion o an signii$ant us$le

(undles and inundi(ular o(stru$tion, thus urther ipro*ing e:posure or the *entri$ular

septal dee$t repair. Pulonar *al*ae sparing operations in inan$ a (e possi(le with

pulonar *al*e annulus Os$ores o - with a$$epta(le postoperati*e right *entri$ular

pressures and reoperation rates .5or patients with a transannular pat$h, the pla$eent o a

ono$usp peri$ardial *al*e $an potentiall redu$e the pulonar regurgitation, (ut it a

not signii$antl ipa$t on ortalit, hospital length o sta, or postoperati*e heodnai$s.

4he *entri$ular septal dee$t a (e $losed ro either a *entri$ular or atrial

approa$h. A $o(ined transatrial and transpulonar approa$h has (een proposed as a

relia(le and sae ethod or $oplete repair in inants and oung $hildren. 4his approa$h

a*oids a *entri$uloto i a transannular pat$h is not reuired and a *er liited one i the

annulus needs to (e $rossed. ese$tion o signii$ant right *entri$ular o(stru$tion $an (e

a$hie*ed through an atrial e:posure, i reuired. portantl, this approa$h has (een

ad*o$ated as a eans or a*oiding hoograt interposition or patients with surgi$all

23



iportant $oronar arter anoalies. 4his approa$h was su$$essul in 3- o 3 patients in a

stud ( BriEard et al. 4he *entri$ular septal dee$t is $losed using a +a$ron pat$h. 4he

dee$t a (e $losed using either $ontinuous or interrupted sutures reinor$ed with 4elon

pledgets. 4he sutures, along the posterior inerior (order, are an$hored to the ri o i(rous

tissue along that aspe$t o the tissue, whi$h is ree o $ondu$tion tissue, or alternati*el, to the

us$ular septu awa ro the dee$t ri. there is signii$ant hpoplasia or a(sen$e o the

$onal septu, the anterosuperior aspe$t o the pat$h is sewn to partition the i(rous tissue that

separates the aorti$ ro the pulonar *al*e. An signii$ant AS+s a (e $losed, although

a sall patent oraen o*ale a (e let as a possi(le sour$e or righttolet atrial

de$opression in the postoperati*e period.3

4he presen$e o signii$ant aortopulonar $ollateral arteries or a patent du$tus

arteriosus does alter surgi$al strateg signii$antl. Pre$ise preoperati*e deinition o the

*essels is iperati*e to a$$uratel guide perioperati*e anageent. Collateral arteries, whi$h

$onstitute the sole sour$e o (lood low to a pulonar segent, are snared prior to initiation

o (pass and in$orporated into the inal repair. 4his a reuire an initial pro$edure *ia

thora$oto to (ring the *essel to an area o the $hest that a (e rea$hed during surger.

@essels that pro*ide dupli$ate low to a lung segent should (e $oiled prior to surgi$al repair

to eliinate a steal phenoenon during $ardiopulonar (pass with the asso$iated ris% o

neurologi$ seuelae.3,,7

%)'&)+ P0lli07ive Pro1edures

4he odiied lalock-aussig shunt is $urrentl the ost $oon aortopulonar shunt

pro$edure and $onsists o a =ore4e: $onduit anastoosed side to side ro the su($la*ian

arter to the hoolateral (ran$h o the pulonar arter Soeties the $onduit is (rought

dire$tl ro the as$ending aorta to the ain pulonar arter and is $alled a central shunt .

4he Blalo$%4aussig operation $an (e su$$essull perored in the new(orn period with

shunts 3K- in diaeter and has also (een used su$$essull in preature inants.7

5or patients who ha*e se*ere pulonar arterial hpoplasia, these pro$edures do

pro*ide soe augentation o pulonar (lood low and pro(a(l do en$ourage urther

arterial growth. >nortunatel, in this situation the do not pro*ide a $onsistent route (

whi$h (alloon angioplast a (e perored. ight *entri$ular outlow tra$t pat$hing a

(e used on o$$asion to esta(lish additional antegrade low into the hpoplasti$ pulonar

arteries while siultaneousl pro*iding a route ( whi$h $atheter(ased reha(ilitation o the

pulonar arteries a ta%e pla$e. 4his $learl is indi$ated onl in the sall su(set o

24



patients with *er diinuti*e pulonar arteries (e$ause in this situation the *entri$ular

septal dee$t reains open and would otherwise result in se*ere pulonar o*er$ir$ulation.

there is $on$ern a(out se*ere right *entri$ular hpertension (e$ause o arginal pulonar

arter siEe and anato, the *entri$ular septal dee$t pat$h a (e enestrated to allow right

*entri$ular de$opression.

Daterston shunts "anastoosis o the as$ending to the right pulonar arter# or Potts

shunts "des$ending aorta to let pulonar arter# are largel o histori$al interest (ut will

o$$asionall ha*e (een perored in patients who are now seen as oung adults. Both

pro$edures resulted in a signii$ant in$iden$e o pulonar arter distortion along with

in$onsistent transission o low and pressure to the pulonar arterial (ed. Pulonar

arterial stenosis or e*olution o pulonar *as$ular disease pre$luded routine use o these

palliati*e pro$edures. 7

4he postoperati*e $ourse o patients with a su$$essul shunt pro$edure is relati*el

une*entul. Postoperati*e $opli$ations a o$$ur ater a lateral thora$oto and in$lude

$hlothora:, diaphragati$ paralsis, and Horner sndroe. Cylo7or0< a reuire

repeated thora$o$entesis and, on o$$asion, reoperation to ligate the thora$i$ du$t.

Di0pr03m07i1 p0r0lysis ro in)ur to the phreni$ ner*e a result in a ore dii$ult

postoperati*e $ourse. Prolonged *entilator support and *igorous phsi$al therap a (e

reuired, (ut diaphragati$ un$tion usuall returns in 1K& o unless the ner*e was

$opletel di*ided. Surgi$al pli$ation o the diaphrag a (e indi$ated. Hor4er sy4drome

is usuall teporar and does not reuire treatent. Postoperati*e cardiac !ailure a (e

$aused ( a large shunt. @as$ular pro(les other than a diinished radial pulse and

o$$asional longter ar length dis$repan$ are rarel seen in the upper e:treit supplied

( the su($la*ian arter used or the anastoosis.7

Ater a su$$essul shunt pro$edure, $anosis diinishes. 4he de*elopent o a

$ontinuous urur o*er the lung ields ater the operation indi$ates a un$tioning

anastoosis. A good shunt urur a not (e heard until se*eral das ater surger. 4he

duration o sptoati$ relie is *aria(le. As the $hild grows, ore pulonar (lood low is

needed and the shunt e*entuall (e$oes inadeuate. Dhen in$reasing $anosis de*elops, a

$orre$ti*e operation should (e perored i the anato is a*ora(le. not possi(le "e.g.,

(e$ause o hpoplasti$ (ran$h pulonar arteries# or i the 1st shunt lasts onl a (rie period

in a sall inant, a se$ond aortopulonar anastoosis a (e reuired on the opposite side.

Se*eral groups ha*e reported su$$essul palliation o the tetralog o 5allot in inants (

(alloon pulonar *al*uloplast.3,7

25



%)'&)( Sur3i10l Resul7s

Current surgi$al sur*i*al, e*en or sptoati$ inants ounger than 3 onths o age, is

e:$ellent. Hospital and 1onth sur*i*al rates o 1009 ha*e (een reported in this patient

population. ntensi*e $are or(idit is in$reased or those repaired in the irst 3 onths o

lie . n the stud ro the >ni*ersit o i$higan , there were no late deaths in the 4'5 with

PS group at edian ollowup o &- onths. n this group, howe*er, &29 reuired

reoperation or *arious indi$ations, in$luding residual pulonar arterial o(stru$tion and

right *entri$ular o(stru$tion. 4hese indi$ations or reoperation *aried su(stantiall ro those

in older patients, or who repair o a residual *entri$ular septal dee$t was a u$h ore

$oon indi$ation. Strategies to urther ipro*e the reoperation rate are prin$ipall aied at

redu$ing an residual outlow tra$t or pulonar arterial o(stru$tion, espe$iall at the site o

insertion o the du$tus arteriosus. A larger, longter analsis o results o (oth single and

twostage repair strategies or 4'5 with PS do$uented a relati*el a*ora(le out$oe or

all singlestage earl repair *ia a transatrial approa$h. 4here were no statisti$al dieren$es in

the need or reinter*ention (e$ause o residual outlow tra$t o(stru$tion or patients

undergoing priar *ersus staged repair. arlier age at repair "1 ear o age# siilarl did

not ad*ersel ae$t the rate o reinter*ention, leading in*estigators to $on$lude that priar

repair should (e regarded as the preerred anageent strateg, an assessent that has (een

e$hoed ( other groups. 4wentear sur*i*al or hospital sur*i*ors, irrespe$ti*e o

anageent strateg, was 689 or patients who ha*e 4'5 with PS and slightl lower or

patients with PA, rele$ting the o*erall e:$ellent longter sur*i*al o these patients

Corre$ti*e surgi$al therap $onsists o relie o the right *entri$ular outlow tra$t

o(stru$tion ( reo*ing o(stru$ti*e us$le (undles and pat$h $losure o the @S+. the

pulonar *al*e is stenoti$, a *al*oto is perored. the pulonar *al*e annulus is

sall or the *al*e is e:treel thi$%ened, a *al*e$to a (e perored, the pulonar

*al*e annulus split open, and a transannular patch pla$ed a$ross the pulonar *al*e ring.

An pre*iousl esta(lished sstei$topulonar shunt ust (e o(literated (eore ull

repair. 4he surgi$al ris% o total $orre$tion is less than 29. A right *entri$uloto was the

standard approa$hF howe*er, a transatrialtranspulonar approa$h $an (e used to redu$e the

longter ris%s o a *entri$uloto. n$reased (leeding in the iediate postoperati*e period

a (e a $opli$ating a$tor in e:treel pol$thei$ patients.

Ater su$$essul total $orre$tion, patients are generall asptoati$ and are a(le to

lead unrestri$ted li*es. ediate postoperati*e pro(les in$lude right *entri$ular ailure,

26



transient heart (lo$%, residual @S+ with lettoright shunting, o$ardial inar$tion ro

interruption o an a(errant $oronar arter, and disproportionatel in$reased let atrial

pressure (e$ause o residual (ron$hial $ollaterals. Postoperati*e heart ailure "parti$ularl in

patients with a transannular outlow pat$h# reuires a positi*e inotropi$ agent su$h as

digo:in. 4he longter ee$ts o isolated, surgi$all indu$ed pulonar *al*ular

insui$ien$ are un%nown, (ut insui$ien$ is generall well tolerated. 4he a)orit o

patients ater tetralog repair and all o those with transannular pat$h repairs ha*e a toand

ro urur at the let sternal (order, usuall indi$ati*e o ild outlow o(stru$tion and ild

to oderate pulonar insui$ien$. Patients with ore ar%ed pulonar *al*e

insui$ien$ also ha*e oderate to ar%ed heart enlargeent. Patients with a se*ere residual

gradient a$ross the right *entri$ular outlow tra$t a reuire reoperation, (ut ild to

oderate o(stru$tion is *irtuall alwas present and does not reuire reinter*ention.

5ollowup o patients 2K&0 r ater surger indi$ates that the ar%ed ipro*eent in

sptos is generall aintained. Asptoati$ patients ha*e lower than noral e:er$ise

$apa$it, a:ial heart rate, and $ardia$ output. 4hese a(noral indings are ore $oon

in patients who underwent pla$eent o a transannular outlow tra$t pat$h and a (e less

reuent when surger is perored at an earl age.

Condu$tion distur(an$es $an o$$ur ater surger. 4he atrio*entri$ular node and the

(undle o His and its di*isions are in $lose pro:iit to the @S+ and a (e in)ured during

surger. A peranent $oplete heart (lo$% ater surger is rare. Dhen present, it should (e

treated ( pla$eent o a peranentl iplanted pa$ea%er. *en a transient $oplete heart

(lo$% in the iediate postoperati*e period is rare in tetralog patientsF it a (e asso$iated

with an in$reased in$iden$e o lateonset $oplete heart (lo$% and sudden death. ight

(undle (ran$h (lo$% is uite $oon on the postoperati*e ele$tro$ardiogra. 4he duration

o the IS inter*al has (een shown to predi$t (oth the presen$e o residual heodnai$

derangeent and the longter ris% o sudden death.

A nu(er o $hildren ha*e preature *entri$ular (eats ater repair o the tetralog o

5allot. 4hese (eats are o $on$ern in patients with residual heodnai$ a(noralitiesF &-hr

ele$tro$ardiographi$ "Holter# onitoring studies should (e perored to (e $ertain that o$$ult

short episodes o *entri$ular ta$h$ardia are not o$$urring. :er$ise studies a (e useul in

pro*o%ing $ardia$ arrhthias that are not apparent at rest. n the presen$e o $ople:

*entri$ular arrhthias or se*ere residual heodnai$ a(noralities, prophla$ti$

antiarrhthi$ therap is warranted. erepair is indi$ated i signii$ant residual right

*entri$ular outlow o(stru$tion or se*ere pulonar insui$ien$ is present 3,7

27



%)'' Pro34osis

arl surger is not indi$ated or all inants with tetralog o 5allot "4'5#, although, without

surger, the natural progression o the disorder indi$ates a poor prognosis. 4he progression o

the disorder depends on the se*erit o right *entri$ular "@# outlow tra$t o(stru$tion

"@'4'#.n the present era o $ardia$ surger, $hildren with siple ors o tetralog o

5allot en)o good longter sur*i*al with an e:$ellent ualit o lie. ate out$oe data

suggest that ost sur*i*ors are in ?ew Qor% Heart Asso$iation "?QHA# $lassii$ation ,

although a:ial e:er$ise $apa(ilit is redu$ed in soe. Sudden death ro *entri$ular

arrhthias has (een reported in 129 o patients at a later stage in lie, and the $ause

reains un%nown. t has (een suspe$ted that *entri$ular dsun$tion a (e the $ause. 'ne

stud ound let *entri$ular longitudinal dsun$tion to (e asso$iated with a greater ris% o

de*eloping liethreatening arrhthias. Continued $ardia$ onitoring into adult lie is

ne$essar. 5or soe tie, it has (een suspe$ted that $ertain $hildren a ha*e inherited a

predispostion to de*eloping long I4 sndroe. A &01& stud ( Chiu $onired this

suspi$ion -

let untreated, patients with tetralog o 5allot a$e additional ris%s that in$lude

parado:i$al e(oli leading to stro%e, pulonar e(olus, and su(a$ute (a$terial

endo$arditis. t is well %nown that $hildren with $ongenital heart disease are prone to stro%e.

n ost o these $hildren the $auses o stro%e ha*e (een related to thro(oe(oli, prolonged

hpotension/ano:i: and pol$theia. Dhat is oten orgotten is that residual shunts or a

patent oraen o*ale are also %nown $auses o stro%es. 4he in*estigation o stro%es in these

$hildren usuall (egins with a C4 s$an o the (rain ollowed ( an CH'

Dithout surger, ortalit rates graduall in$rease, ranging ro 309 at age & ears

to 209 ( age ears. 4he ortalit rate is highest in the irst ear and then reains $onstant

until the se$ond de$ade. ?o ore than &09 o patients $an (e e:pe$ted to rea$h the age o 10ears, and ewer than 2109 o patients are ali*e ( the end o their se$ond de$ade.

ost indi*iduals who sur*i*e to age 30 ears de*elop $ongesti*e heart ailure "CH5#,

although indi*iduals whose shunts produ$e inial heodnai$ $oproise ha*e (een

noted, al(eit rarel, and these indi*iduals a$hie*e a noral lie span. Howe*er, $ases o

sur*i*al o patients into their 80s ha*e (een reported. +ue to ad*an$ed surgi$al te$hniues, a

-09 redu$tion in deaths asso$iated with tetralog o 5allot was noted ro 1676 to &002.

As ight (e e:pe$ted, indi*iduals with tetralog o 5allot and pulonar atresia ha*e

the worst prognoses, and onl 209 sur*i*e to age 1 ear and 89 to age 10 ears.-

28



%)'% Compli107io4

Beore $orre$tion, patients with the tetralog o 5allot are sus$epti(le to se*eral

serious $opli$ations. 5ortunatel, ost $hildren undergo palliation or repair in inan$, and

these $opli$ations are rare. Cerebral thromboses, usuall o$$urring in the $ere(ral *eins or

dural sinuses and o$$asionall in the $ere(ral arteries, are $oon in the presen$e o e:tree

pol$theia and dehdration. 4hro(oses o$$ur ost oten in patients ounger than & r.

4hese patients a ha*e iron dei$ien$ aneia, reuentl with heoglo(in and heato$rit

le*els in the noral range. 4herap $onsists o adeuate hdration and supporti*e easures.

Phle(oto and *olue repla$eent with resh roEen plasa are indi$ated in e:treel

pol$thei$ patients. Heparin is o little *alue and is $ontraindi$ated in patients with

heorrhagi$ $ere(ral inar$tion. Phsi$al therap should (e instituted as earl as possi(le.

rain abscess is less $oon than $ere(ral *as$ular e*ents and e:treel rare when

ost patients are repaired at u$h ounger ages. Patients with a (rain a(s$ess are usuall

older than & r. 4he onset o the illness is oten insidious and $onsists o lowgrade e*er or a

gradual $hange in (eha*ior, or (oth. Soe patients ha*e an a$ute onset o sptos that a

de*elop ater a re$ent histor o heada$he, nausea, and *oiting. SeiEures a o$$urF

lo$aliEed neurologi$ signs depend on the site and siEe o the a(s$ess and the presen$e o

in$reased intra$ranial pressure. C4 or $onirs the diagnosis. Anti(ioti$ therap a

help %eep the ine$tion lo$aliEed, (ut surgi$al drainage o the a(s$ess is usuall ne$essar. 7

acterial endocarditis a o$$ur in the right *entri$ular inundi(ulu or on the

puloni$, aorti$, or rarel, the tri$uspid *al*es. ndo$arditis a $opli$ate palliati*e

shunts or, in patients with $orre$ti*e surger, an residual puloni$ stenosis or @S+.

Anti(ioti$ prophla:is is essential (eore and ater dental and $ertain surgi$al pro$edures

asso$iated with a high in$iden$e o (a$tereia. 7

.eart !ailure is not a usual eature in patients with the tetralog o 5allot. t ao$$ur in a oung inant with Lpin%M or a$anoti$ tetralog o 5allot. As the degree o

pulonar o(stru$tion worsens with age, the sptos o heart ailure resol*e and

e*entuall the patient e:perien$es $anosis, oten ( K1& o o age. 4hese patients are at

in$reased ris% or hper$anoti$ spells at this tie. ,7

CHAPTER III

CASE REPORT

29



,)' O56e17ive

4he o()e$ti*e o this paper is to report a $ase o 1 ears 3 onths old (o with a

diagnosis o 4etralog o 5allot.

,)% C0se Repor7

0me : S

A3e : '/ ye0rs , mo47

Se< : M0le

D07e o8 Admissio4 : %. Sep7em5er %&'(

Cie8 Compl0i47 : Canosis

His7ory o8 dise0se :

4his pro(le started sin$e the $hild aged - onths and it (e$ae worst sin$e a da

(eore $oing to the hospital. 4he patient had $anosis en$ountered in the area o the

tongue, lips, e:treities, and it did not disappear with the adinistration o o:gen.

Shortness o (reath "R# e:perien$ed within 1 da, shortness o (reath asso$iated with

a$ti*ities su$h as wal%ing a distan$e o 2 eters. 5e*er "R# e:perien$ed S? sin$e two

das ago, the e*er is not that high, the e*er $oes down whene*er its gi*en the e*erlowering drugs. @oiting en$ountered sin$e 1 da ago, a reuen$ o*er 2 ties a da, the

*olue o T $up, the $ontents o what is in the eating and drin%ing. S? is a di*ision o

$ardiolog old patient with a diagnosis o 4'5 and has perored $atheteriEation.

His7ory o8 medi107io4 : un$lear

His7ory o8 80mily : un$lear

His7ory o8 p0re47?s medi107io4 ; un$lear

His7ory o8 pre34041y : 4he age o the o was &- ears old during her

pregnan$ with =3P3A0. 4he gestation age was 38 wee%s. His o went or regular

pregnan$ $ontrol. Histor o +ia(etes elitus was not ound. >sage o drugs "#, >sage

o her(s "#.

His7ory o8 5ir7 : Birth assisted ( idwi*es, (a( was (orn ( noral.

4he (a( iediatel $r. Blue or $anosis was not ound. Birth (od weight ; 3&00 gr,

(irth (od length ; was un$lear, and head $ir$ueren$e was un$lear.

30



His7ory o8 8eedi43 : :$lusi*e (reasteeding until - onths. 5orula il%

(egined when he was - onth. PAS (egined he was - onth.

His7ory o8 immu4i=07io4 : Coplete iuniEation

His7ory o8 3ro>7 04d developme47: un$lear

Pysi10l E<0mi407io4:

Prese47 s707us :

e*el o $ons$iousness; $opos entis, (od teperature; 37UC, BD; 3- %g, BH; 127 $,

BD/A; Os$ore229 , B/A; Os$ore 689, BD/B; Os$ore 7-.-9, anei$ "#,

$anosis "R#, dpnea "R#.

Lo10li=ed s707us:

Head ; Hair was (la$%, har all easil was nod ound. es; ight rele:

R/R, iso$hori$ pupil, $on)un$ti*a palpe(ra inerior pale "/#

$teri$ s$lera "/#, inerior and superior palpe(ra edea "/#

ars; within noral range

?ose ; within noral range

outh ; $anosis "R#

?e$% ; ph node enlargeent "#, 4@J R&$ H&'

4hora: ; setri$al usior, $hest retra$tion "#, thrill "#

H; 7- (p, regular, e)e$tion sistoli% urur gr @/ "R# in CS @ in

let id$la*i$ularis line.

; 30 (p regular, rales "/#, (reath sound ; *esi$ular.

Additional sound "#.A(doen ; Sot, non tender, noral peristalti$, li*er and spleen was not

Palpa(le. As$ites "# 4uor "#

:treities ; pulse 7- (p regular, p/* adeuate, war a$ral, C4 3M,

$lu((ing inger"R# $anosis in ingers "R#

Anogenitalia ; ale, within noral liit, anus "R#

*or2i43 di034osis : 4etralog o 5allot

Fu7er Pl04 :

1. Cardia$ Catheter

31



&. Che$% $oplete (loud $ount, seru ele%trolit, Blood glu$ose rando

3. le%tro$ardiograph

-. Chest Gra

2. $ho$ardiograph

,aboratory "inding/

.ematology

4est esult >nit eeren$e

Heoglo(in &3,&0 g9 11.31-.1

BC 7,6- 10/3 -.-0-.-8

eu$o$te -,-0 103/3 .017.2

4hro(o$te -7 103/3 &17-67

Heato%rit 6.109 9 37-1#C@ 87,00 l "8162#

CH &6,&0 Pg &2&6

CHC 33,0 g9 &631

+D 16,10 9 11.1-.8

osinophil 1.10 9 1

Basophil 2.700 9 01

?eutrophil &7,80 9 3780

pho$te 2,80 9 8.30

ono$te 8,0 9 11.20

?eutrophil a(solute 1,&& 103 /V &.-7.3

phosite a(solute &,20 103 /V 1.722.1

ono$te a(solute 0,38 103 /V 0.&0.

osinophl a(solute 0,02 103 /V 0.10.3

Basophl a(solute 0,&2 103 /V 00.1

Chei$al 4est

esult >nit eeren$e

Blood =as Analsis

• pH ; 7,3&0 "7,327,-2#

• pC'& ; &,0 Hg "38-&#

• p'& ; 102,0 Hg "82100#

• Bi%ar(onat"HC'3# ; 13,- ol/ "&&&#

32



• 4otal C'& ; 1-,& ol/ "16&2#

• B ; 11,1 ol/ "&# K "R&#

• '& saturation ; 68, 9 "62100#

Blood glu$ose ; 80,00 g/d "&00#

le$trolte;

• Cal$iu ; 7,8 g/d "8.-10.-#

• ?atriu ; 138 /d "132122#

• !aliu ; 2,0 /d "3.2.2#

• Clorida ; 110 g/d "610#

Chest Gra

33



Costophreni$us angle is taper.

+iarag is sooth $ontour.

4he heart is Cardioegali "C4 209#.

4he tra$hea is in the iddle.

Bone stru$ture and sot tissue appear noral

Co41lusio4; Cardioegali

34



,)%)' Follo> #p

FOLLO* #P

Sep7em5er %.7 %&'(

S;

Canosis "R#,

+spnea "R#,

5e*er "R#,

Pu%e "R#

' ; Sensoriu; CF 4; 37oCF BD; 3- %g, BH; 127 $

Head ;

e; light rele: "R/R#, iso$hori$ pupil, pale inerior

$on)un$ti*a palpe(ra "/#

ar; within noral range

?ose; within noral range

outh; $anosis

?e$%; lph nodes enlargeent "#

4hora:; setri$al usior, retra$tion "#

H; 7- (p, reg, e)e$tion sistoli% urur "R# grade @/

let linea id$la*i$ularis C /@

; 30 (p, reg, rales "/#

A(doinal; sot, non tender, peristalti$ "R# ?, i*er and Spleen

not palpa(le

:treities; pulse 7- (p, reg, p/* adeuate, war a$ral, C4

3M, $anosis "R#, $lu((ing 5inger

A; 4etralog o 5allot P;

'& W 1 /i nasal $anule

@5+ +2 9 ?aCl 0,-29 30

gtt/i i$ro

Sep7em5er ,&7 %&'(

S ;

Canosis "R#

' ; Sensoriu; CF 4; 3. 6oCF BD; 3- %g, BH; 127 $

Head ;

A ;4etralog o 5allot P ;


35



+spnea "R# ↓

5e*er ↓

Pu%e "#





outh; $anosis



H; 78 (p, reg, e)e$tion sistoli$ urur grade

@/ id $la*i$ularis line C /@

; 30 (p, reg, rales "/#

A(doinal; sot, non tender, peristalti$ "R# ?, i*er and

Spleen not palpa(le

:treities; pulse 78 (p, reg, p/* adeuate, war a$ral,

C4 3M

Canosis "R#, Clu((ing 5inger

@5+ 02 9 ?aCl 0,-29 30 gtt/i

i$ro.

' O27o5er7 %&'(

S ;

Canosis "R#

+spnea "R# ↓

5e*er "#

Pu%e "#

' ; Sensoriu; CF 4; 3,3oCF BD; 3- %g, BH; 127 $

Head ;





outh; $anosis


A ; 4etralog o 5allot P;


@5+ +2 9 ?aCl 0,-29 30 gtt/ii$ro

36




H; 80 (p, reg, e)e$tion sistoli% urur "R# grade

@/ let linea id$la*i$ularis C /@

; 30 (p, reg, rales "/#


Spleen not palpa(le


C4 3M, $anosis "R#, $lu((ing 5inger

Answer ro Heatolog +ept ;

4he patient is suggested to do Phle(oto .

%7 O27o5er %&'(

S ;

+spnea "R# ↓

' ; Sensoriu; CF 4; 37,1oCF BD; 3- %g, BH; 127 $

Head ;

e; light rele: "R/R#, iso$hori$ pupil, pale

inerior $on)un$ti*a palpe(ra "/#



outh; within noral range



H; 8& (p, reg, e)e$tion sistoli$ urur grade

A ; 4etralog o 5allot P; '& W 1 /i nasal $anule

@5+ +2 9 ?aCl 0,-29 30 gtt/i

i$ro

@5+ ?a$l 39 170$$/1& )a

37




; 30 (p, reg, rales "/#


Spleen not palpa(le


C4 3M


, 7 O27o5er %&'(

S ;

+spnea "R# ↓


Head ;








H; 80 (p, reg, e)e$tion sistoli$ urur grade @/ id

$la*i$ularis line C /@ ; 30 (p, reg, rales "/#

A(doinal; sot, non tender, peristalti$ "R# ?, i*er and Spleen not

palpa(le

:treities; pulse 80 (p, reg, p/* adeuate, war a$ral, C4 3M

A; 4etralog o 5allot P; '& W 1 /i nasal

$anule

@5+ +2 9 ?aCl

0,-29 30 gtt/i i$ro

@5+ ?a$l 39

170$$/1& )a

Ple(otoi plan

38




.ematology

H=B &&,70g9 "11.31-.1#

BC 7,710/3 "-.-0-.-8#

DBC 2,6103/3 "-.213.2#

Ht ,609 "37-1#

P4 103 103/3 "120-20#

C@ 87,&0 l "8162#

CH &6,0 pg "&2&6#

CHC 33,60 g9 "&631#

+D &0,309 "11.1-.8#

#i!tel/

?eutrophil -3,709 "3780#

pho$te 36,109 "&0-0#

ono$te 13,-09 "&8#

osinophil &,009 "1#

Basophil 1,8009 "01#

A(solute neutrophil &.0 103 /V "&.-7.3#

A(solute lpho$te &,33 103/ V "1.72.1#

A(solute ono$te 0,80 103/ V "0.&0.#

A(solute eosinophil 0.1& 103/ V "0.10,3#

A(solute (asophil 0.11 103/ V "00.1#

Clinic chemist/

Al(uin -,2g/d "3,&-,2#

>reu &3,70g/d "20#

Creatinin 0,3g/d "0,701,&0#

39



+7 O27o5er %&'(

S ;

Post ple(otoi+spnea "R# ↓


Head ; e; light rele: "R/R#, iso$hori$ pupil, pale

inerior $on)un$ti*a palpe(ra "/#







@/ id $la*i$ularis line C /@ ; &- (p, reg, rales "/#

A(doinal; sot, non tender, peristalti$ "R# ?, i*er andSpleen not palpa(le

:treities; pulse 80 (p, reg, p/* adeuate, war

a$ral, C4 3M, Clu((ing 5inger

A; 4etralog o 5allot P; '& W 1 /i nasal $anule

@5+ +2 9 ?aCl 0,-29 30

gtt/i i$ro

@5+ ?a$l 39 170$$/1&

hour

onitor post ple(otoi

"*ital signs, the patients

$ondition#

40



.ematology

H=B &0,80g9 "11.31-.1#

BC 7,010/3 "-.-0-.-8#

DBC ,38103/3 "-.213.2#

Ht 1,609 "37-1#

P4 1&8 103/3 "120-20#

C@ 87,70l "8162#

CH &6,200 pg "&2&6#

CHC 33,0 g9 "&631#

+D 16,809 "11.1-.8#

P@ 13,30 "7,010,&#

PC4 0,179

P+D &&,

#i!tel/

?eutrophil -0,809 "3780#

pho$te -1,-09 "&0-0#

ono$te 1-,109 "&8#

osinophil &,809 "1#


A(solute neutrophil &.0 103 /V "&.-7.3#

A(solute lpho$te &,- 103/ V "1.72.1#

A(solute ono$te 0,60 103/ V "0.&0.#

A(solute eosinophil 0.18 103/ V "0.10,3#


41



(7 O27o5er %&'(

S ;

+spnea "R#

' ; Sensoriu; CF 4; 37,&oCF BD; 3- %g, BH; 127 $

Head ;







A; 4etralog o 5allot P; '& W 1 /i nasal $anule

@5+ +2 9 ?aCl 0,-29 30

gtt/i i%ro

@5+ ?a$l 39 170$$/1&

hour

42




H; 6& (p, reg, e)e$tion sistoli$ urur grade


; & (p, reg, rales "/#


Spleen not palpa(le

:treities; pulse 6& (p, reg, p/* adeuate, war a$ral,

C4 3M, Clu((ing 5inger

Consule ;

4o +epartent o ?utrition and ndo$rine

Answer ro $onsule ;

Patient is diagnosed as alnutrition

.ematology

H=B &0,20g9 "11.31-.1#

BC ,70 10/3 "-.-0-.-8#

DBC 2,86 103/3 "-.213.2#

Ht 1,809 "37-1#

P4 1-8 103/3 "120-20#

C@ 88,70l "8162#

CH &6,-0 pg "&2&6#

CHC 33,&0 g9 "&631#

#i!tel/

?eutrophil -,109 "3780#

pho$te 37,-09 "&0-0#

ono$te 1&,609 "&8#

osinophil &,609 "1#


A(solute neutrophil &.7& 103 /V "&.-7.3#

A(solute lpho$te &,&,&0 103/ V "1.72.1#

A(solute ono$te 0,7 103/ V "0.&0.#

43



+D 16,209 "11.1-.8#

P@ 1&,20 "7,010,&#

PC4 0,189

P+D 18,8

A(solute eosinophil 0,17 103/ V "0.10,3#

A(solute (asophil 0.0- 103/ V "00.1#

/7 O27o5er %&'(

S ;

+spnea "±#

' ; Sensoriu; CF 4; 37oCF BD; 37 %g, BH; 127 $

Head ;




?ose; within noral range outh; within noral range



H; 6 (p, reg, e)e$tion sistoli$ urur grade @/ id

A; 4etralog o

5allot

P; '& W 1 /i nasal

$anule

@5+ +2 9 ?aCl

0,-29 30 gtt/i i%ro

@5+ ?a$l 39

170$$/1& hour

44



$la*i$ularis line C /@

; &- (p, reg, rales "/#


palpa(le

:treities; pulse 68 (p, reg, p/* adeuate, war a$ral, C4 3M

Clu((ing 5inger

.ematology

H=B &0,60g9 "11.31-.1#

BC 7,&0 10/3 "-.-0-.-8#

DBC 7,01 103/3 "-.213.2#

Ht 2,009 "37-1#

P4 17 103/3 "120-20#

C@ 60,30l "8162#

CH &6,00 pg "&2&6#

CHC 3&,&0 g9 "&631#

+D 18,609 "11.1-.8#

P@ 1&,80 "7,010,&#

PC4 0,&39

#i!tel/

?eutrophil -3,309 "3780#

pho$te -3,-09 "&0-0#

ono$te 10,709 "&8#

osinophil 1,609 "1#


A(solute neutrophil 3,0- 103 /V "&.-7.3#

A(solute lpho$te 3,0- 103/ V "1.72.1#

A(solute ono$te 0,72 103/ V "0.&0.#



"aal hemostasis/

Protro(in 4ie

Patient; &3,

Control; 1-,00

?; 1,73

AP44

Patient; 2&,2

Control; 3-,0

4ro(in tie

Patient; &3,0

Control; 17,-

45



P+D 16,3

97 O27o5er %&'(

S ;

+spneu "R#

' ; Sensoriu; CF 4; 3,6oCF BD; 37,3 %g, BH; 127 $

Head ;








H; 6- (p, reg, e)e$tion sistoli$ urur grade @/ id

$la*i$ularis line C /@

; &- (p, reg, rales "/#


A; 4etralog o

5allotR alnutrition

P; '& W 1 /i nasal

$anule

@5+ +2 9 ?aCl

0,-29 30 gtt/i i$ro

@5+ ?a$l 39 170$$/1&

hour @it C 1: 100g

@it B $op 1:1 ta(

5oli$ a$id 1:1 g

46



palpa(le

:treities; pulse 6- (p, reg, p/* adeuate, war a$ral, C4

3M, Clu((ing 5inger

;7 O27o5er %&'(

S;

+spnea "R#


Head ;








H; 6 (p, reg, e)e$tion sistoli$ urur grade @/ id$la*i$ularis line C /@

; && (p, reg, rales "/#


palpa(le

:treities; pulse 6 (p, reg, p/* adeuate, war a$ral, C4

A; 4etralog o

5allotR

alnutrition

P;


@5+ +2 9 ?aCl 0,-29

30 gtt/i i%ro

@5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g

@it B $op 1:1 ta(

5oli$ a$id 1:1 g

47



3M, Clu((ing 5inger

.ematology

H=B 10,00g9 "11.31-.1#

BC 3,38 10/3 "-.-0-.-8#

DBC 3,&3 103/3 "-.213.2#

Ht 30,709 "37-1#

P4 6 103/3 "120-20#

C@ 60,80l "8162#

CH &6,0 pg "&2&6#

CHC 3&,00 g9 "&631#

+D 17,-09 "11.1-.8#

P@ 11,60 "7,010,&#

PC4 0,119

P+D 13,6

#i!tel/

?eutrophil 3,209 "3780#

pho$te 20,&09 "&0-0#

ono$te 11,209 "&8#

osinophil 1,209 "1#


A(solute neutrophil 1,18 103 /V "&.-7.3#

A(solute lpho$te 1,&103/ V "1.72.1#

A(solute ono$te 0,37 103/ V "0.&0.#



48



.7 O27o5er %&'(

S;

+spnea ↓


Head ;








H; 6- (p, reg, e)e$tion sistoli$ urur grade @/

id $la*i$ularis line C /@

; &- (p, reg, rales "/#


Spleen not palpa(le

:treities; pulse 6- (p, reg, p/* adeuate, war a$ral,


A; 4etralog o 5allotR

alnutrition

P;


@5+ +2 9 ?aCl 0,-29

30 gtt/i i%ro

@5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g

@it B $op 1:1 ta(

5oli$ a$id 1:1 g

+iet 5100 300 $$ R $$

ineral i:

49



'&7 O27o5er %&'(

S;

+spnea ↓


Head ;








H; 68 (p, reg, e)e$tion sistoli$ urur grade @/


; &- (p, reg, rales "/#


Spleen not palpa(le




alnutrition

P; '& W 1 /i nasal $anule

@5+ +2 9 ?aCl 0,-29

30 gtt/i i%ro

@5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g

@it B $op 1:1 ta(

5oli$ a$id 1:1 g

+iet 5100 300 $$ R $$

ineral i:

50



''7 O27o5er %&'(

S;

+spnea ↓


Head ;










; &- (p, reg, rales "/#


Spleen not palpa(le




alnutrition

P;


@5+ +2 9 ?aCl 0,-29

30 gtt/i i$ro @5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g

@it B $op 1:1 ta(

5oli$ a$id 1:1 g

+iet 5100 300 $$ R $$

ineral i:

51



'%7 O27o5er %&'(

S;

+spnea ↓


Head ;










; &- (p, reg, rales "/#


Spleen not palpa(le




alnutrition

P;


@5+ +2 9 ?aCl 0,-29

30 gtt/i i%ro

@5+ ?a$l 39 170$$/1&hour

@it C 1: 100g

@it B $op 1:1 ta(

5oli$ a$id 1:1 g

+iet 5100 300 $$ R $$

ineral i:

52



',7 O27o5er %&'(

S;

+spnea ↓

$anosis


Head ;




?ose; within noral range outh; within noral range




id $la*i$ularis line C /@ ; &- (p, reg, rales "/#


Spleen not palpa(le




alnutrition

P;


@5+ - gtt/i i$ro

@5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g

@it B $op 1:1 ta( 5oli$ a$id 1:1 g

+iet 5100 300 $$ R $$

in i:

53



.ematology

H=B 17,70g9 "11.31-.1#

BC ,10 10/3 "-.-0-.-8#

DBC 7,73 103/3 "-.213.2#

Ht 2-,-09 "37-1#

P4 172 103/3 "120-20#

C@ 86,&0l "8162#CH &6,00 pg "&2&6#

CHC 3&,20 g9 "&631#

+D &0,109 "11.1-.8#

P@ 11,60 "7,010,&#

PC4 0,&19

P+D 1-,&

+ 1/hour "12#

#i!tel/

?eutrophil 36,109 "3780#

pho$te -6,209 "&0-0#

ono$te 10,309 "&8#

osinophil 0,209 "1#


A(solute neutrophil 3,01 10

3

/V "&.-7.3#A(solute lpho$te 3,83103/ V "1.72.1#

A(solute ono$te 0,80 103/ V "0.&0.#

A(solute eosinophil 0,0- 103/ V "0.10,3#


54



'+7 O27o5er %&'(

S;

+spnea ↓

Canosis


Head ;










; &- (p, reg, rales "/#


Spleen not palpa(le




alnutrition

P; '& W 1 /i nasal

$anulel

@5+ - gtt/i i$ro

@5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g


+iet 5100 300 $$ R $$

ineral i:

55



'(7 O27o5er %&'(

S;

+spnea ↓

Canosis


Head ;










; &- (p, reg, rales "/#


Spleen not palpa(le



A; 4etralog o 5allot R

alnutrition

P; '& W 1 /i nasal

$anule

@5+ - gtt/i i$ro

@5+ ?a$l 39 170$$/1&

hour

@it C 1: 100g


+iet 5100 300 $$ R $$

ineral i:

56



+'@ @SP Su(pulonale

inial P

57



CHAPTER I$

DISC#SSIO

4etralog o 5allot is the ost $oon or o $anoti$ $ongenital heart disease

ater inan$, o$$urring in 2 o 10,000 li*e (irths. 4he etiolog is ultia$torial, (ut reported

asso$iations in$lude untreated aternal dia(etes, phenl%etonuria, and inta%e o retinoi$ a$id.

Asso$iated $hroosoal anoalies $an in$lude trisoies &1, 18, and 13, (ut resen$e

e:perien$e points to u$h reuent asso$iation o i$rodeletions o $hroosoe &&. 4he ris%

o re$$uren$e in ailies is 39. - n this $ase, the patient does not has a geneti$ ris% and

ail histor o siilar disease.

4he se*erit o right *entri$ular outlow tra$t o(stru$tion deterines the $lini$al

sptos that $an o$$ur in 4'5 . n patients with degrees o(stru$tion e:it right *entri$le

weight, $anosis $an appear aster . +spnea , atigue, ta%ing a suatting position while

tired is a $lini$al sptos o tetralog o 5allot . n this $ase , the patient easil tired and

li%es to ta%e a suatting position while wal%ing .

'n phsi$al e:aination will (e ound sstoli$ e)e$tion urur in the upper or

iddle third o the let sternal linia . ?oise is asso$iated with the degree o sstoli$ right

*entri$ular outlow o(stru$tion . 'n the degree o o(stru$tion is ore se*ere , the sstoli$

urur is heard short and wea% . 7 n the $ase , the patient was a gu aged 1 ear 3

onths present with (lue on the lips and ingertips and toes 1 da (eore $oing to the

hospital ., and on phsi$al e:aination ound sstoli$ urur grade @ / CS C

/ @

Chest Gras showed generall the siEe o the heart is enlarged . Heart shape

generall will show a pi$ture li%e (oot shaped and de$reased pulonar *as$ularit . 7 n this

$ase , the results o $hest Gra shows the siEe o the heart is enlarged ( the C4 o 209 .

CatheteriEation $an (e used to $onir the diagnosis , espe$iall disorders o $ople:

$ongenital heart disease , $ardia$ heodnai$s e*aluate , assess the ee$ts o anoalies or

lesions o the $ardio*as$ular sste , heart anato apping in detail so as to help deterine

the operating a$tions that will (e ta%en ro the results o $atheteriEation were perored to

plan surger patients .

58



Canoti$ $ongenital heart disease patients who did not $orre$ti*e surger will

e:perien$e a state o pol$theia and $lu((ing as well as share other $opli$ations . 4'5

patients with adeuate sstei$ o:gen saturation will aintain H( 12 g / dl to 17 g / d with

a heato$rit o -2 9 to 20 9.7 Howe*er, i the heato$rit in$reases a(o*e 2 9 , there will

(e hper*is$osit with *arious $onseuen$es. Copli$ations o the $entral ner*ous sste

$an (e a heada$he thro(osis or stro%e and $ere(ral a(s$ess . n the $ase o the (lood test

showed H( ; &3gr / dl and a heato$rit o - 9.

edi$al treatent o patient 4'5 is in the or o $anoti$ atta$%s in the e*ent

handler , a*oid or ui$%l treat dehdration , %eeping dental hgiene , prophla$ti$ anti(ioti$s

to pre*ent endo$arditis . Patients at ris% o de*eloping (a$terial endo$arditis so it needs to (e

gi*en prophla$ti$ anti(ioti$s prior to dental e:tra$tions and surgi$al pro$edures spe$ii$ to

the high in$iden$e o (a$tereia . Prophla$ti$ anti(ioti$s should (e adinistered in a single

dose (eore the pro$edure 6. the dose was not inad*ertentl gi*en anti(ioti$s (eore the

pro$edure . 4he dose $an (e adinistered up to & hours ater the pro$edure . Howe*er ,

adinistration o the dose ater the pro$edure should (e $onsidered onl i the patient did not

re$ei*e pre K pro$edure.

+ental e:aination results not ound o$al inlaation and ine$tion o the teeth

and outh . Parents are en$ouraged to aintain dental and oral hgiene dental patient

(e$ause the patient is still in the growth stage 6. n this $ase prior to the a$t o $atheteriEation ,

the patient was gi*en anti(ioti$s $etria:one 1gr or prophla:is to pre*ent (a$tereia and

endo$arditis .

Currentl 4'5 $orre$tion surger is re$oended in the irst ear o lie . 4wo

studies in nggir who studied 4'5 $orre$ti*e surger on $hildren ounger than 1 ear showed

good results and a low ortalit rate and good output .10 A stud in Canada to get the

optiu age or surger is (etween 3 to 11 onths.

?ot all patients $an (e diagnosed earl and underwent surger under the age o 1

ear . A stud in South Ari$a showed that patients who underwent surger 4'5 slower on

the a*erage age o 6 ears , (ut the result is uite good with the death o onl 6 9 . 11 Siilar

results were o(tained ( a stud in ran where the surger ortalit rate .6 9 and lie

e:pe$tan$ o patients 61 9 at 1.2 and 10 ears o post $orre$tion . 4here was no dieren$e

(etween patients who underwent priar surger or gradual $orre$tion . 'nl &.1 9 ound

slow death .

59



S#MMARY

t has (een reported, a gu with the ain $oplain o $anosis and was diagonsed with

4etralog o 5allot. 4he diagnose was esta(lished (ased on histor ta%ing, $lini$al

aniestation, la(orator inding, ele%tro$ardiograhph, e$ho$ardiograph, and $ardia$

$atheteriEation.

Re88ere41es

60



1. Berg +, &011. 4etralog o 5allot. n ; il eonar, editor. Pathophsiolog o Heart +isease.

5ith edition. Philadelphia ; ippin$ot Dillias and Dil%ins; &001 pg 38038&

&. Bhi)i, S., &011. etralogy o! "allot . A*aila(le ro;

http;//eedi$ine.eds$ape.$o/arti$le/&0326-6o*er*iew X&- April &012Y

3. Siwi% S, Patel C, Oah%a !=, =olduntE . 4etralog o 5allot. n ; Allen H+, Clar% B, ,

+adolesents. dition si:th, *olue se$ond. Philadelphia ; ippin$ot Dillias and Dil%insF

&001, h. 88066

-. Bailliard, 5. and Anderson, .H., &006. etralogy o! "allot . 'rphanet Journal o are

+iseases *olue -F &8

2. oss, +?. 4he surgi$al anageent o 5allotZs 4etralog. Postgrad. ed J. 161. 37. 26.

. Pradoso A. Pena%it Jantung sianoti%. n; Sastroasoro S, adiono B, editor. Bu%u A)ar

!ardiologi ana%. disi %e1. Ja%arta; Binarupa A%saraF 166-. Pg.&37787. Bernstein +. Canoti$ $ongenital heart lessions; lessions asso$iated with de$reased

pulonar (lood low. n; Berhan , !leigan , Jenson HB, editor. ?elson te:t(oo%

o pediatri$s. dition 18. Philadelphia ; SaundersF &007. P.16018.

8. Putra,S., Ad*ani, ?., et all. 4etralog o 5allot. n ; Pud)iadi, A. Hegar, Badriul. Pedoan

Pelaanan edis %atan +o%ter Ana% ndonesia. &006 pg 3163&&

6. Dilson D, 4au(ert !A, =ewitE , o$%hart PB, Baddour , e*ision , et all. Pre*enti*e

o ine$ti*e endo$arditis; =uidelines ro the Aeri$an Heart Asso$iation. Cir$ulation.

&007F11;1732-10. Ale:io C, ahoud H, Al!haddour A, =nananpragasa J, Salon AP, !eeton B, et all.

'ut$oe ater repair o tetralog o allot in the irst ear o lie. Ann 4hora$ Surg.

&001F71;-6-200

11. 'oi A, oor)ani ?, Baliulis =, !eeton B, Salon AP, onro J, et all. ediu ter

out$oe or inant repair in tetralog o allot; indi$ators or tiing surger. ur J

Cardiothora$ Surg. &00F30;617&&

1&. @an Arsdell =S, ahara) =S, 4o J, ao @!, Coles J=, 5reedo , et all. Dhat is the

optial age or repair o tetralog o allot[ Cir$ulation. &000F 10& Suppl ;1&36

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