Case Report Tetralogy of Fallot-complete

8/21/2019 Case Report Tetralogy of Fallot-complete

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CASE REPORT

CYANOTIC CONGENITAL HEART DISEASE:

TETRALOGY OF FALLOT

Supervisor

dr. Muhaad A!i" Sp.A#$%

Prese&'a'ors:

Ci'ra Ar(a&'i )*)+))),)

Maria&'o )*)+))++-

DEPARTMENT OF PEDIATRICS

MEDICAL FACLTY

NI/ERSITY OF SMATERA TARA

HA0I ADAM MALI$ GENERAL HOSPITAL CENTER

MEDAN -)+1


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PREFACE

Praise the God for His gratitude and blessing so that we are finally able to

finish this case report entitled Tetralogy of Fallot well. This case report is

written in order to fulfill the duties of senior clinical assitance of clinical rotation

in Pediatrics Department. This paper is expected to increase the nowledge and

insight! especially about tetralogy of fallot.

This case report is done to fulfill the clinical rotation"s duty in pediatrics

department. The authors would also lie to than the super#isor! dr. $uhammad

%li! &p.%'()! who gi#e guidance! suggesstion! and assessment for this case report.

*ast of all! we reali+e that despite the effort we ha#e put into this case

report! there are still errors and flaws in our wor. Therefore! we encourage and

welcome suggestions! ad#ices! and criticisms that will facilitate the impro#ement

of this paper. Hopefully this scientific wor can contribute to the de#elopment of

medical science in particular.

$edan! ,,th -anuary /,0

%uthors

ii


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A2STRACT

Tetralogy of fallot is the most common cyanotic congenital heart disease found in infant.

Tetralogy of Fallot is a tetra cardiac malformation comprising ventricular septal defect,

right ventricular outflow tract obstruction, overriding of the aorta, and right ventricular

hypertrophy. This disease can be asymptomatic and symptomatic while majority will

remain asymptomatic until they outgrow their pulmonal blood supply. It is important of

early diagnosis of TOF because surgical procedures should be done precisely. This case

report presented a 6 year old boy with main complaint history of bluish skin since he was

months. !orkup diagnostic has been made and patient has been referred for surgical

intervention.

"eywords# tetralogy of fallot, bluish

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TA2LE OF CONTENTS

PagesCO/ER.......................................................................................................... i

PREFACE..................................................................................................... ii

A2STRACT.................................................................................................. iii

TA2LE OF CONTENTS............................................................................. i#

LIST OF PICTRES" CHARTS" AND TA2LES..................................... #

CHAPTER + INTRODCTION............................................................ ,

,.,. 1acground........................................................................ ,

,.. 2b3ecti#es........................................................................... 0

,.0. 1enefits.............................................................................. 0

CHAPTER - LITERATRE RE/IE3................................................ 4

.,. Definition........................................................................... 4

.. 5tiology and Pathogenesis................................................. 4

.0. Pathophysiology................................................................. ,/

.4. Diagnosis............................................................................ ,

.6. $anagement....................................................................... ,7

.6.,. $anagement tetralogy of fallot.............................. ,7

.6.. $anagement of hypoxic spell................................ 4

.7. 8omplication...................................................................... 7

.9. Prognosis............................................................................ 9

CHAPTER 1 CASE REPORT............................................................... :

CHAPTER 4 DISCSSION................................................................... 69

CHAPTER , SMMARY...................................................................... 7/

REFERENCES

i#


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LIST OF FIGRES" CHARTS" AND TA2LES

Fi5ures

Figure .,. De#elopment of cardiac structures................................................ 9

Figure .. Hemodynamics of acyanotic '%) and cyanotic '1) tetralogy of

fallot............................................................................................... ,,

Figure .0. 2b3ecti#e measurement of clubbing fingers.................................. ,0

Figure .4. Palliati#e shunts for tetralogy of fallot.......................................... ,:

Figure .6. &urgical correction for tetralogy of fallot...................................... ,;

Figure .7.


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CHAPTER +

INTRODCTION

+.+. 2a785rou&d

8ongenital heart disease '8HD) accounts for nearly one=third of all ma3or

congenital anomalies. *inde! et al. systematic re#iew included ,,4 papers

identified ,74!0;7 indi#iduals '/.7:>) of total 4!/;,!:79 li#e births identified.

?ncidence of congenital heart diseases '8HDs) among infants born ali#e is about

,.>.,@eported total 8HD birth pre#alence increased substantially o#er time!

from /.7 per ,!/// li#e births ';6> confidence inter#al A8?BC /.4 to /.:) in ,;0/ to

,;04 to ;., per ,!/// li#e births ';6> 8?C ;./ to ;.) after ,;;6. %sia reported the

highest 8HD birth pre#alence! with ;.0 per ,!/// li#e births ';6> 8?C :.; to ;.9)!

with relati#ely more pulmonary outflow obstructions and fewer left #entricular

outflow tract obstructions. ?n ?ndonesia! same of that with %sia! incidence of

8HD was ;=,/ in ,/// li#e births.0

8ongenital heart diase can be simply di#ided into acyanotic and cyanotic

groups. ?n the cyanotic 8HD group! there were predominant defects! tetralogy

of Fallot 'T2F) and transposition of the great arteries 'TG%)! while T2F was the

most common and twice as pre#alent as TG% '4.9,/!/// births #s .0,/!///

births! respecti#ely). The total pre#alence of all other cyanotic defects combined

was 6.6,/ /// births.4T2F is also the most common cyanotic 8HD that is liely

to result in sur#i#al to infancy and adulthood.65pidemiologic study showed that

T2F occurs in ,/ per thousand li#e births worldwide.7There hasn"t been done an

epidemiological studies for pre#alence of T2F in ?ndonesia.

Tetralogy of Fallot 'T2F) is a cardiac malformation comprising #entricular

septal defect '


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bleu and the term tetralogy of Fallot a tetrad of #entricular septal defect!

o#erriding of the aorta! right #entricular outflow obstruction! and right #entricular

hyperthropy.,/

1oth en#ironmental and genetic factors were thought to be strongly

associated with T2F. $any of gene mutations has been identified to be

responsible for de#eloping of T2F. Thus! forty percent of the T2F patients are

associated with other congenital cardiac abnormalities.,,-enins! et al. postulated

that en#ironmental factors played more important to cause T2F because smoe

influenced gene mutations and destruction of tissues.,

Tetralogy of fallot can be asymptomatic and symptomatic. $a3ority will

remain asymptomatic until they outgrow their pulmonal blood supply.,0 ?nfant

with tetralogy of fallot will come with presentation of cyanosis! shortess of breath!

easy fatiguability! clubbing finger! nee chest position to relie#e spells. ?n many

conditions! spell will occur and need treatment as soon as possible. ,4

This clinical presentation will be improtant to consider the option for

management although many contro#ersies came out wheter to do early correction

or to precede shunting procedure. ithout surgical treatment! the estimated , year

sur#i#al rate is 77> and the estimated 0/ year sur#i#al rate is 7>. ith surgical

treatment o#er :6> of children sur#i#e to adulthood. The patients who sur#i#e

into adulthood without surgical treatment reuire uniue management based on

their indi#idual anatomy and physiology. $anagement should be directional and

precise because prolonged hypoxia will bring many complications entire of

human body.;!,,

1ased on the rising of morbidity and mortality of tetralogy of fallot! it

became the biggest challenge to impro#e the care globally. %ccess to health care

is still limited in many parts of the world! as well as the diagnostic facilities.

hile in other hand! there needs to be further in#estigation to tailor the

management of this global health problem.,6Indoubtedly! the authors feels that it

is necessary to re#iew more about tetralogy of fallot. Therefore! the authors

presented a case report of uncomplicated tetralogy of fallot in a 7=year=old boy.


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The authors expected that this case report will gi#e more understanding about

tetralogy of fallot! its diagnostic and management approach.

+.-. O69e7'ives

The ob3ecti#es of this case report is to raise the nowledge and preferences of

tetralogy of fallot and to complete the clinical rotation tas in pediatrics

department.

+.1. 2e&ei's

This case report is expected to pro#ide benefits to authors and readers about better

understanding in tetralogy of fallot cases.

0


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CHAPTER -

LITERATRE RE/IE3

-.+. Dei&i'io&

Tetralogy of Fallot 'T2F) is a cardiac malformation comprising #entricular septal

defect ')! rarely at the pulmonary #al#e le#el ',/>)! combination of both

'0/>). %nother finding! atretic pulmonary #al#e! is the most se#ere form of the

anomaly ',6>). ?n some children! pulmonary atresia can de#elop o#er time

'tetralogy of Fallot with acuired pulmonary atresia).9!:!;!,6!,7

-.-. E'io!o5( a&d Pa'ho5e&esis

1oth en#ironmental and genetic factors were thought to be strongly associated

with T2F! although en#ironmental factors recei#ed more attention. $aternal

factors such as rubella and other #iral infection! diabetes mellitus and exposures to

teratogenic agents such as thalidomide during pregnancy ha#e been shown to be

associated with 8HD including T2F in #arious epidemiological studies. The

o#erriding theory is that these en#ironmental exposures occurred during a critical

period in cardiac de#elopment! which then resulted in formation of T2F. The

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clear relationship between certain exposures and T2F and the limitations with

pro#ing genetic studies in pre#ious decades led some in#estigators to postulate

that en#ironmental factors were probably more important than genetic factors in

T2F causation.:!,

?t was reported that T2F were associated with chromosomal abnormalities

'such as trisomies ,0!,:! and ,) and syndromes '#elocardiofacial syndrome)! and

associations 'such as 8H%@G5 and of cases with trisomy , ha#e

congenital heart defects! pre#alent atrio#entricular septal defects 'isolated or

associated with Tetralogy of Fallot).,9 8hromosomal anomalies are nown to

accounts for twel#e percent of the T2F cases.,:!,;

&maller changes in#ol#ing deletions or duplications of segments of

chromosomes! copy number #ariants '8 of patients with classic T2F! and 4/> of those with T2F with

pulmonary atresia! will ha#e microdeletions of chromosome ,,.. The

,,. deletion e#en occurs as a de novo mutation in o#er ;/> of newly

diagnosed cases. These deletions and duplications occur by a #ariety of

mechanisms and can result in haploinsufficiency or o#er expression of a number

of contiguous genes! often with a negati#e effect on phenotype and it is inherited

by autosomal dominant fashion.0!4 ,,D& is a de#elopmental disorder that

affects third and fourth pharyngeal pouch structures! namely the heart and great

#essels! thymus! parathyroid glands and craniofacial structures. %blation of the

cardiac neural crest cells has been shown to result in cardiac phenotypes similar to

those seen in ,,D& and it has been postulated that this syndrome may be

lined to improper neural crest cell migration into the pharyngeal arch structures.

%nother #ariants! ,,., deletionsduplications! was found to be another

significant #ariants which could cause T2F. ?t was also reported that methylene

tetrahydrofolate reductase '$THF@) gene polymorphism can be considered a

susceptibility gene for tetralogy of Fallot.,:!,; ?n 6K6/> of cases! maternal

phenyletonuria also results in Tetralogy of Fallot.


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estimated to cause about ,=6> of T2F cases.,; and can be associated with

syndromic 'e.g. %lagille syndrome) as well as nonsyndromic presentations.

The heart starts as a tube. Two sections of the tube! the truncus arteriosus

and the bulbus cordis! grow towards each other. The truncus arteriosus twists

,:/L as it grows down towards the bulbus cordis. This twisting separates the aorta

and the pulmonary artery. %ntero=cephaled of the twisting causes tetralogy of

Fallot 'T2F) such as right #entricular outflow tract obstruction! non restricti#e


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Figure .,. De#elopment of cardiac structures;

?ncreasing e#idence suggests that single gene mutations are present in a

broad spectrum of genes in#ol#ed in cardiac structure and function. Pleiotropic

cardiac malformations can result from discrete mutations in specific nuclear

transcription factors! proteins recogni+ed as playing ey regulatory roles during

cardio#ascular de#elopment and morphogenesis. Factors such as G%T%4!

x.6! dH%D! TF%P! and Tbx6 are among the earliest transcription factors

expressed in the de#eloping heart and are crucial in the acti#ation of cardiac=

specific genes. $utations in each of these genes result in se#ere cardiac

abnormalities including tetralogy of fallot.;

Table .,. Gene mutations in tetralogy of fallot0/

1one morphogenetic proteins! nt! and FGF are the main growth factors

modulating the expression of cardiogenic transcription factors. The proteins

@egarding= and =4 are soluble factors belonging to the superfamily of TGF=b

growth factors. They induce the expression of 8sxx=.6 in cardiac mesoderm

through signaling pathways that in#ol#e &mad and TGF=N inase , mediators. ?n

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fact! the promoter of 8sxx=.6 presents binding sites for &mad is an important

role for its function to regulate post=translational modifications and it interacts

with other transcriptional factors 'G%T%=4! &@F! Tbx6! Tbx! Tbx/ and

H%D).0,

There are three nt signaling pathwaysC canonical! noncanonical and

ntcalcium. 2f these pathways! both the canonical and noncanonical pathways

ha#e clear roles in early heart de#elopment. 8anonical nt signaling inhibits

cardiac induction and maintains the secondary heart field 'pharyngeal mesoderm)

in an undifferentiated state. ?ts inhibition is essential for myocardial

differentiation. oncanonical nt signaling! on the other hand! promotes cardiac

differentiation and is specifically needed for outflow tract de#elopment.

%ntagoni+ing the canonical nt signal by D=, or 8rescent is necessary for the

expression of cardiogenic transcription factors 8sxx=.6 and Tbx6. %fter the

heart tube has formed! canonical nt signaling is maintained in the secondary

heart field. b=catenin is needed in ?sl,=expressing secondary heart field

progenitors. *oss of nt signaling reduces the number of ?sl,=positi#e cells!

leading to outflow tract and right #entricular defects! whereas excess nt

signaling expands the ?sl,=positi#e population. These suggest suggest that

canonical nt signaling is responsible for promoting proliferation and

maintaining cells in a progenitor state.0

1eta=catenin induces both @egarding=4 and noncanonical nt,, expression

in the secondary heart field! which also suggests that the early canonical nt

signaling sets the stage for differentiation. hile the canonical nts are important

for their ability to inhibit the initial cardiac induction! the noncanonical nts

promote cardiac differentiation. *ie the canonical pathway! noncanonical nts

also bind transmembrane receptorsO signaling proceeds through Dishe#eled! which

modifies actin #ia the @ho@28( and @ac-( pathways. oncanonical nt6%!

in combination with canonical nt inhibitor D=,! induces cardiac

differentiation in stromal #ascular cells. ?n addition! nt,, also induces cardiac

differentiation in Menopus! and nt,,= null mice ha#e both arch artery patterning

and outflow tract defects. Howe#er! ?sl, expression appears normal in these mice!

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suggesting that the initial specification of the secondary heart field occurs

correctly. This expression pattern supports the idea that noncanonical nt

signaling is important not for the early steps of induction or specification! but for

later differentiation.00

% recent study by a -apanese group has in#estigated the role of

phosphatidylinositol 0=inase 'P?0=inase) at a #ery early stage of cardiomyocyte

differentiation! possibly by modulating the expression of the cardiac transcription

factors 8sxx=.6 and G%T%=4. Through these downstream proteins! P?0=

inase plays an essential role in a wide #ariety of cellular processes and in the

differentiation of se#eral cell lineages! including cardiomyocytes. ?n this study! it

was demonstrated that cardiomyocyte differentiation was bloced by P?0=inase

inhibitors from days / to 4 after induction of differentiation! suppressing the

expression of 8sxx=.6 and G%T%=4. These findings may be explained

because P?0=inase could be in#ol#ed in the transcriptional loop between

8sxx=.6 and G%T%=4.04

Four percents of patients with ToF ha#e an (M.6 mutation. 06(M.6 is

a cardiac homeobox protein that is essential for normal cardiac de#elopment

especially #entricular cardiomyocytes maturation during the final stages of heart

de#elopment and mutations in the 8&M gene 'which encodes (M.6) cause

se#eral congenital heart malformations. % dominant locus associated with

cardio#ascular malformations and conduction abnormalities has been mapped to

chromosome 606. These mutations ha#e also been found to cause #arious other

cardiac structural abnormalities.07

-%G, encodes a highly conser#ed cell surface protein! which is a ligand

in#ol#ed in the otch pathway and is thus a component of an intracellular

signaling pathway shown to be crucial for deciding a cardiogenic progenitor cells

fate. $utations in -%G, ha#e been found in indred studies in association with

%lagille syndrome! a complex autosomal=dominant disorder presenting with 8HD

including T2F. -%G, encodes a ligand that binds the otch receptor! an

e#olutionarily conser#ed signaling pathway in#ol#ed in cell fate specification.

$utations in the signaling regulator otch, ha#e recently been implicated in

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aortic #al#e disease. $utations in PTP,, encoding a protein

tyrosinephosphatase '&HP=) ha#e been proposed to play a role in the

pathogenesis of oonan syndrome characteri+ed by conduction defects!

pulmonary stenosis! and hypertrophic cardiomyopathy!; and ha#e been also

recently implicated in the pathogenesis of *52P%@D syndrome! which liely

represents an allelic disorder.0/

&ome researchers identified hetero+ygosity for a mutation in the +inc=finger

protein! multi=type 'QFP$) gene andsuggested that mutations in this gene may

contribute to some sporadic cases of ToF. Pi++uti et al. '//0) disco#ered that the

QFP$ gene maps to :.09QFP$! which is a +inc finger co=factor! interacts

with the =terminal domain of the transcription factor G%T% binding protein 4

'G%T%=4) and modulates its transcriptional acti#ity both in #itro and in #i#o

during heart de#elopment. ?n mice! the QFP$ protein is also nown as F2G=. 0:

G%T%=4 binds specifically to the %T G%T% %G motif of D% and isin#ol#ed

in heart de#elopment in many organisms.0;

G%T%6 is an upstream regulator of se#eral genes expressed during

embryogenesis and cardiac morphogenesis! including the genes that encode atrial

natriuretic factor '%F)! brain natriuretic peptide! R=myosin hea#y chain! N

myosin hea#y chain! and cardiac troponin 8 and ?.4/The human '(T( gene

maps to chromosome /,0.00 by fluorescence in situ hybridi+ation! encoding a

protein of 0;9 amino acids.0:The G%T%6 mutations of p.@,:9G and p.H/9@

identified in this study are both located in QF,! thus may be expected to ha#e

impact on the transcriptional acti#ation of G%T%6 by interfering with the

recognition and binding of G%T%6 with target gene promoter.4,

-.1. Pa'hoph(sio!o5(

T2F reuires only two abnormalitiesKa


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the presence of the P& minimi+es the magnitude of the left=to=right shunt! the

heart si+e and the pulmonary #ascularity increase only slightly to moderately.

These increases are indistinguishable from those of a small to moderate


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#entricular hypertrophy. ?nsulin lie growth factor '?GF)! acidic and basic

fibroblast growth factor 'FGF=, and FGF=)! plateletderi#ed growth factor

'PDGF) and epidermal growth factor '5GF) are all growth factors in#ol#ed in the

myosin R to N=isoform shift to increases the efficiency of force de#elopment by

producing the same absolute muscle tension at a slower rate because the R=form

has a three to se#en fold greater %TPase acti#ity than N=myosin. Depending on the

importance and duration of o#erload and concomitant pathology! the @ of cases! tall @

wa#es! and &T=T changes. ?f the systolic pressure in the right #entricle is between

4/ and 7/ mm Hg! signs of moderate right #entricular hypertrophy will be present

in about 06=46 > of patients. % tall @ wa#e in leads

useful in defining issues that could not be addressed by con#entional

echocardiography. This could be particularly important when defining #ascular

anatomy alterations. The catheteri+ation is not routinely reuired. Howe#er! it

could be performed when the necessary data for surgical correction decision

maing cannot be obtained by other exams. $cGoon ratio and naata index are

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used to uantitae the degree of pulmonary stenosis. $cGoon ratio is the ratio of

the sum of the diameter of the immediately prebranching portion of the right

pulmonary artery plus left one di#ided by the diameter of the descending aorta

3ust abo#e the diaphragm. ormal #alues are =.6 and good Fontan candidates

should ha#e ratio of E,.:. aata index is the cross sectional area of right

pulmonary artery and left one 'in mm) di#ided by the 1&%. The a#erage

diameters of both right and left pulmonary artery are measured at the points

immediately proximal to the origin of the first lobar branches at maximal and

minimal during one cardiac cycle in the anteroposterior #iew of the pulmonary

arteriogram. ormal #alues is 00/ W 0/ mm1&% and good @astelli candidate

should ha#e an index E//.;!46

-.,. Ma&a5ee&'

-.,.+.Ma&a5ee&' o 'e'ra!o5( o a!!o'

There are some supporti#e and medical management of tetralogy of fallot listed

below. Howe#er! the correcti#e treatment remains inter#ention surgery.9!:!;!,/!46!47

,. ?n newborn! hypercyanotic condition can be treated by administering

prostalglandin 5, /./,=/./ Xggmin infusion. Prostalglandin wors as a

potent and specific relaxant of ductal smooth muscle to eep the ductus

arteriosus open which will pro#ide additional pulmonary blood flow and

increase the childs oxygen le#el. This can be continued through the

preoperati#e period and during cardiac catheteri+ation.,/

. ?n infant! tet spell can be pre#ented by commencement of oral propanolol /.6=

,.6 mgg e#ery 7 hours while waiting for an optimal time for correcti#e

surgery. blocade of beta receptor will reduced cardiac contractility. Graham!

et al. study in 0 patients who use preoperati#e propranolol therapy and 76 as

control! showed that there were no differences in postoperati#e inotrope

scores in the first , hours. There was a trend toward increased inotrope

scores at 4 and 4: hours 'pY/./6). 1ut! no differences found between groups

in length of mechanical #entilation! ?8I stay! or total hospital postoperati#e

stay. ?n conclusion! propranolol therapy can be used in patients with tetralogy

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of Fallot until the time of surgery! without important effects on their

postoperati#e courses. %ny blunting of inotropic or chronotropic acti#ity in

propranolol=treated patients appears to be easily o#ercome with increased

inotropic medications or temporary pacing! without increased morbidity or

mortality. 5#en in $ahmouda! et al. study! using preoperati#e propanolol

therapy will pre#ent postoperati#e -5T.6/&tephenson! et al. study showed that

propranolol will pre#ent cardiac arrhytmias post surgery 'pJ/!/6). ?n the

control group! cardiac arrhythmias for which treatment was necessary

de#eloped in 0, of ,07 patients '0>)! atrial fibrillation or flutter in 4

patients 'l:>)! and #entricular arrhythmias in 9 '6>). ?n the group recei#ing

propranolol! cardiac arrhythmias reuiring treatment de#eloped in ; of :9

patients ',/>)! atrial fibrillation or flutter in 9 ':>)! and #entricular

arrhythmias in '>). the difference in freuency.6,

0. Prophylaxis against bacterial endocarditis.

4. Treatment of iron deficiency anemia! heart failure! heart bloc! and other

comorbidities.

6. *ifestyle! pre#ent cold! pre#ent dehydration! home remedies! exercising! and

play.

1efore inter#ention of pallati#e shunt in T2F treatment! newborn T2F

babies will absolutely died. 1ut after surgical inter#ention in past decades!

numbers of sur#i#al increased. The first palliati#e shunt in#ented by 1laloc!

Taussig! and


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gore tex graft between the right #entricle and the pulmonary arteries. This right

#entricle to pulmonary arteries conduit has been proposed as a better alternati#e to

a modified 1T& and preliminary data from one or two centres has suggested a

better outcome. ?t is hypothesi+ed that the conduit will pro#ide a more stable post

operati#e balance between pulmonary and systemic circulations with a lower

pulmonaryCsystemic blood flow ratio 'VpVs).66 ?n ,;6;! Glenn introduced a

Glenn shunt! anastomosed between superior #ena ca#a and pulmonary artery.

Pulmonary artery pressures must be low for this to be successful. 67?n ,;47! Potts

introduced a Potts shunt! anastomosed between the descending aorta and the left

pulmonary artery. ?n ,;7! aterson introduced a aterson shunt! anastomosed

between the ascending aorta and the right pulmonary artery. Potts and aterson

shunt is no longer performed either because these procedures included too large a

shunt leading to 8HF andor pulmonary hypertension! and narrowing and ining

of the pulmonary artery at the site of the anastomosis.64 The cooley shunt!

intrapericardial modified waterston shunt also is no longer commonly used.67

Figure .4. Palliati#e shunts for tetralogy of fallot6

,:


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The first total correction of a tetralogy of Fallot was achie#ed in %pril ,;64

by *illehei! using the cross circulation techniue in a patient aged ten months!

and subseuently in another ten patients of whom six were under two years of

age. The first patient sur#i#ed and is still li#ing today. &hortly thereafter! *illehei!

et al. introduced patch enlargement of the right #entricularinfundibulum. The first

successful correction of a tetralogy of Fallot using extracorporeal circulation was

carried out by (irlin in ,;66 using pump or oxygenation for repair. ?n ,;6;!

(irlin et al. again reported the use oftransanular patching. Howe#er! in spite of

the initial successes in the correction of tetralogy of Fallot in infancy by

pioneering cardiac surgeons such as &humway! further attempts had

disappointingly high mortality! and this led to the uni#ersal acceptance of

correction in two stages! with an initial palliati#e procedure and total correction at

a greater age.64!69

Figure .6. &urgical correction for tetralogy of fallot69

8ontro#ersies in T2F management nowadays is that either chosing one

stage early surgical repair or two stage procedures! first shunting then late

surgical repair! usually ,!6 years after! which will introduced a better outcome forpatients. For at least decades! initial palliationfollowed by reparati#e operation

later in childhood was themost pre#alent strategy. 1y the early ,;:/s! primary

repair in early infancy had been championed by 8astaneda! 1arrat=1oyes! and

others.69 The concept of early primaryrepair was extended to symptomatic

neonates! and low operati#emortality was achie#ed.6:

$any studies had been carried out and #ariation of early and late outcomes

were showed by these studies. Palliati#e shunts ha#e become less common

,;


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treatments for T2F since the ad#ent of initial correcti#e procedures during

infancy to reduce complication of two times surgical inter#ention. 67 Howe#er!

patients undergoing staged treatment of T2F! using a palliati#e shunt and

subseuent total correction! accrue the ris of two operati#e procedures and the

potential complications of right=to=left intracardiac shunting! compromised

#entricular function! and fibrosis with ongoing hypoxemia and myocardial

ischemia.6;

During ,;6/=,;;/! which classical trans#entricular surgical techniue was

used! early surgical correction leads to many complications such as significant

pulmonary #al#e insufficiency and is often accompanied by increased mortality. 6:

$ortality approached 6/>! but current ris has fallen to consistently /=6>.6

Pulmonary insufficiency and the resultant right #entricular#olume o#erload ha#e

important conseuences in terms of #entriculardimensions and function! exercise

capacity! arrhythmias! heartfailure! and sudden cardiac death leading to late

reoperationto address pulmonary insufficiency and right #entricular

#olumeo#erload is becoming more pre#alent.6:%lthough (inner et al..! identified

the use of a transannular patch as a ris factor for late mortality! the results of the

study of -ames et al.! and a large study by (irlin et al.! suggested that the

compensatory responses to right #entricular o#erload were adeuate for a /=year

period! at least with respect to mortality.7/1ut after Hudspeth and 5dmunds and

later promulgated by (awashima! Pacifico! and $ee introduced

transatrialtranspulmonary correction! it was showed lower mortality and

morbidity compared to two stages repair. Giannopoulous et al. study showed that

0=year=follow up in patients corrected with transatrial or transpulmonal correction

results in no mortality and ;0!> had normal right #entricular function.7,

?n many studies! mortality rate is eual between single and two staged

procedures. Hashem+adeh! et al. showed mortality rate of 6./:> for primary

complete repair of T2F without an initial palliati#e procedure compares #ery

fa#orably with the mortality of 3ust an initial shunt operation for this congenital

defect.7/


26/72

Figure .7.


27/72

systemic hypothermia '6L8 to :L8) and blood or crystalloid prime. 70The


28/72

&tudy of 9 children by %rsdell! et al. showed that age 0 to ,, months is the

greatest physiological tolerance to do repair surgery. ?nfants aged 0 to ,, months

had the most rapid reco#ery from operati#e therapy! as measured by lesser time to

normali+ation of serum lactate! time to extubation! and length of hospital stay!

compared to infants aged J 0 months 'pY/!/0). o deaths occurred in infants aged

0 to ,, months. Despite the fact that each of these deaths occurred in a child who

did not ha#e a pre#ious shunt! the multi#ariate model showed pre#ious palliation

to be a ris for longer hospitali+ation by #olume=loading pro#ided by the systemic

to pulmonary artery shunt protects against the unfa#orable right #entricular

diastolic stiffness.77%nother different results came from Gerling et al. study. ?n

,4 patients! they showed that early repair of T2F within the first year of life can

be recommended! because mortality is lower than in patients treated at a higher

age. o significant difference in the reinter#ention rate between patients treated

within the ,st year of life or later.79

?n Poorsi! et al. study! for patients J,/ years and ,/K,; years of age at

the time of surgery who were ali#e , year after repair of T2F! excess mortality

persisted at least 0/ years after surgery and did not #ary a great deal with duration

of followup. 1ased on limited data! excess mortality for the age group /K0;

years was higher compared to that of younger ages during the first ,/ years after

surgery and decreased thereafter.7:

Graphic .,. $ortality rate of surgical for tetralogy of fallot 7:

The potential benefits of neonatal correction of the tetralogy or of correction

in e#ery case in early infancy include the following a#oids the riss and

0


29/72

complications of a palliati#e aortopulmonary shunt! mingling of #enous blood in

the systemic circulation 'cerebral embolism)! only small patch need for closure

before maturity! @


30/72

factors such as crying! defecation! feeding! waing from naps 'low systemic

resistance)! fe#er! dehydration! tachypneatachycardia due to any cause!

medications may stimulate mechanoreceptor in right #entricle to pro#ide

sufficient oxygen. 8athecholamines will be released causing increased

contractility while #ascular will compensate by #asodilating. @espiratory center

will be also stimulated. %ll of this compensation e#en worsening and results in

self perpeuating cycle.;!7;!7/

Figure .9. Perpeuating cycle in pathophysiology of hypoxic spells9,

$anagement of hypoxic spellsC;!9

,. (nee=to=chestsuatting. *aaso! et al. found that there was a statistically

significant reduction in the posterior tibial artery flow #elocity! maximally

0,.7>! when the sub3ect was mo#ed from the prone to the nee=chest

position. ?n ,/ of the , #olunteers! no flow in the posterior tibial #ein was

detected in doppler I&G in the nee=chest position. There was also increase

in diastolic arterial pressure 'pJ/.//,).90%lthough! @igamonti! et al. didn"t

found any intraabdominal pressure difference between prone and nee chest

postion. @egardless of it! nee chest postion was pro#en to increased

afterload! the systemic #ascular resistance. &o that! now left #entricle

offered more resistance that right #entricle! blood will rather go to

pulmonary circulation than leas through the


31/72

arterial oxygen saturation! probably by temporarily trapping desaturated

blood in the lower extremities.

. 2xygen ,//> can be administered but usually has minimal effect. 2xygen

gi#en ,//> ensuring the little blood entering pulmonal will be oxygenated

masimally.

0. $orphine /!,=/! mgg i.m. or s.c. that will reduces #entilatory dri#es!

reduces infudibular spasm. $orphine will induce release of histamine and

nitrogen oxide causing #asodilation of systemic #ascular.94

4. 8rystaloid or colloid fluid bolus ,/=/ mlg by rapid i.#. push maximises

preload and should be gi#en prior to the following drugs which may include

hypotension.

6. %cidocis correction by sodium bicarbonate , m5g i.#. repeat in ,/=,6

minutes may reduce the respiratory centre stimulating effect of acidosis.

7. Propranolol starting at =6 Xgg and titrating up to ,/=/ Xgg ?< slow

push. Iltimately! if the preceding steps do not relie#e the spell or if the

infant is rapidly deteriorating! intubation with muscle paralysis and general

anesthesia may be necessary.

9. &econdary drugsC phenylephrine /!/ mgg i#! etamin ,=0 mgg i# o#er ,

minute increases the systemic #ascular resistance and sedates the infant.

-.


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- Failure to thri#e

-.=. Pro5&osis

Gi#en no correcti#e surgery! up to 0/=06> of children will die within the first

year of life! 6/> by the third year! and only few will sur#i#e into adulthood. 2ne!

three! and ten year sur#i#al probabilities are only 77>! 4;>! and 4>. early

9/> of patients with TF reuire an operation during their first year of life because

of hypoxic spells or persistent hypoxemia defined as resting arterial oxygen

saturation less than 9/>.6!96

8umulati#e sur#i#alat 6 years postoperati#ely was ;4.4>. There was no

significantrelationship between sur#i#al and year of operation! age atoperation!

sex! or presence of a prior shunt. $idterm reoperations are reported in =,;> of

the patients! mostly because of residual pulmonary stenosis and to a lesser extent

due to residual of the TF patients. The actuarial freedom from

reoperation was :6=:;> at ,6 years.6!4!96

CHAPTER 1

9


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CASE REPORT

IDENTITY

ame C $uhammad Quhri

%ge C 7 years

&ex C $ale

$@ C 64.,.7,

%ddress C Dusun ? Desa &artono Tebing Tinggi

Date of %dmission C December! 6th/,

$Q! a 7 years old boy! weight 0., g! height ;: cm! presented to Pediatrics

Department at Ha3i %dam $ali General Hospital 8enter on December 6th/,

at ,7.// with the main complaint history of bluish sin. 1luish sin was first

experienced by the patient when he was 6 months. 1luish sin was found initially

on the finger nail and spreaded slowly to the lips! head! and entire body.

&hortness of breath was experienced by the patient all the time! especially when

the patient was crying. &hortness of breath was not associated with the weather

but strong associated with exertion. The child may play for only a short time

before sitting or lying down. 2nce able to wal! the child often assumes a

suatting position to catch his breath and then resumes physical acti#ity within a

few minutes.

The patient also encounter an easy of fatiguability. The child usually tires easily

and begins panting with any form of exertion. The patient found it comfortable to

curl at sleep and rest. History of freuently discontinued breast feeding was found.

The patient often experienced rapid worsening shortness of breath along with

di++iness and muscle rigidity while sometimes ended with syncope. Freuency =

0 times a day and each attac lasted 6=/ minutes. &ince 6 years old! the attac

was decreasing in freuency ',= times in a wee). Patient used suatting position

to relie#e his breath. This complaint mostly appeared when the patient was crying!

feeding! dan waing up in the morning.

:


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The parents also felt that the patient was shorter than his peers. Irination and

defecation were within normal limit. 8ough and fe#er was not found. History of

family experienced the same complaint was not found.

His'or( o Previous i!!&ess

The patient was pre#iously admitted to a general hospital at Tebing Tinggi with

the same complaint fi#e years ago. The patient was diagnosed with the heart

disease by the general physician. Diagnosis was made by history and physical

examination. Physicians there referred the patient to Ha3i %dam $ali Hospital

but the patient refused to came by.

His'or( o Previous edi7a'io&

Innown herbs.

His'or( o Pre5&a&7(

The patient"s mother pregnant at age :. Patient was the third child in his family.

%ntenatal care was ne#er done by his mother. His mother felt #ery easily tired and

wea when the pregnancy but ne#er consult physicians for the complaint. o

history of fe#er! infection! drugs! herbs and alcohol consumption was found. The

mother was exposed to cigarette smoe when the pregnancy since his father was a

smoer. History of stillbirth was not found.

His'or( o 2ir'h

The patient was born at the family"s house and assisted by a nurse. Gestational

age was 09 wees. Patient was deli#ered on spontaneous labor and cried

immediately. o icteric and cyanosis. 1irth weight was 0!,// grams! birth length

was not measured! and history of cyanosis was not found during the birth.

Feedi&5 His'or(

From birth to 7 months C 1reast mil! formula mil! and rice porridge

From 7 months to ; months C 1reast mil! formula mil! rice porridge! and soft

;


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rice

From ; months to years C 1reast mil! formula mil! rice porridge! and soft

rice

From years until now C Formula mil and Family food

His'or( o Gro>'h a&d Deve!ope&'

&itting C , months

8rawling C ,: months

&tanding C 4 months

Taling C 4 months

aling C 7/ months

@eading C hasn"t been sent to school yet

De#elopmental screening test on this patientC

,. The patient can recogni+e colors well

. The patient can"t hopping on one foot se#eral times

0. The patient can"t write! draw! and read well

4. The patient hardly able to maintain balance when standing on one foot

6. The patient also hardly to answer some uestions well

?t concluced that the patient has de#elopment delay.

His'or( o Iu&i?a'io&

18G! DPT ',x)! measles! polio ',x)

Ph(si7a! E;ai&a'io&

Generali+ed status

1ody weight C ,4 g

1ody length C ;: cm

1ody weight in 6/th percentile according to age C , g

1ody length in 6/th percentile according to age C ,,7 cm

1ody weight in 6/th percentile according to body length C ,6 g

0/


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1age C.,

,4 x ,//> Y 79>

1*age C,,7

;:x ,//> Y :4>

11* C,6

,4 x ,//> Y ;0>

Presence &tatus

&ensoriumC alert! 1PY ,//7/ mmHg! H@Y ,,/ bpm! @@Y 0/ xmin! temperatureC

09o8. %nemic '=)! dyspnea 'U)! cyanotic 'U)! edema '=)! icteric '=). 1ody weight

'1)C ,4 g. 1ody length '1*)C ;: cm. 8D8C 1%ge Y 79>! 1*%ge Y :4>!

11* Y ;0>.

*ocali+ed &tatus

Head C 5yeC light reflex 'UU)! isochoric pupil 0 mm! inferior

con3ucti#a palpebra hyperemiahyperemia! icteric sclera '==).

oseC nasal flaring 'U)

5arsC within normal limits

$outhC lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis

'=)! atrophy papilla '=)

ec C *ymph node enlargement '=)

Thorax C &ymmetric fusiform! retraction '=)O tactile fremitus leftYright!

normal impressionO percussion resonant in both lungsO

respiratory sound #esicular! no additional soundO ictus cordis

un#isibleO single & heart sound! murmur 'U)! systolic e3ection

murmur grade ???


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8omplete 1lood 8ount @esult Init @eferral

Hb . gr> ,,.0 K ,4.,

18 ,7.0; x ,/0mm[ 4.6 K ,0.6

@18 ;.7, x ,/7mm[ 4.4/ K 4.4:

Hematocrite 9, > 09 K 4,

P*T ,/6 x ,/[mm[ ,6/ K 46/

$8< 90.; f* :, K ;6

$8H 0., Pg 6 K ;

$8H8 0,.0 g> ; K 0,

@D 6.4 > ,,.7 K ,4.:

eutrophil :0., > 09 K :/

*ymphocyte ,.: > / K 4/$onocyte 0.: > K :

5osinophil /., > , K 7

1asophil /. > / K ,

1lood Gas %nalysis @esult Init @eferral

pH 9.,:; 9.06=9.46

p82 . mmHg 0:=4

p2 9;./ mmHg :6=,//

1icarbonat :.0 mmol* =7

Total 82 ;./ mmol* ,;=6

1ase5xcess =,9.; mmol* '=) = 'U)

2 &aturation ;/.; > ;6 K ,//

8onclusionCse#ere acidocis metabolic with partial compensation! normoxemia

5lectrolyte @esult Init @eferral

&odium ,4 m5* ,06=,66

(alium 4., m5* 0.6=6.6

8hloride ,,0 m5* ;7=,/7

Ches' @Ra(

0


38/72

%symmetrical photo! heart enlarged with upward apex! aorta arch elongated!

decreased pulmonary #ascularity was found! both hillus was blurred! hillus

position was in the center! diaphragm and costophrenicus sinus angle was sharp.

1ones was intact and normal.

8onclusionC 8ongenital heart disease with increased #ascularity

00


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E!e7'ro7ardio5raph(

I&'erpre'a'io&:

&inus rhythm! V@& rate ,06 bpm! V@& axisC left axis de#iation! P pulmonale'U)!

P@ inter#al /!,7 s! V@& pathologist on lead ? and a


40/72

8yanotic 8ongenital Heart Disease ec. dd ,. Tetralogy of fallot U failure to thri#e

. Transposition of great artery

0. Pulmonary atresia

Teporar( Dia5&osis:

8yanotic congenital heart disease ec. tetralogy of fallot U failure to thri#e

Ma&a5ee&':

= 1ed rest! nee chest position

= @egular meals ,6// cal with : gram protein

= Irine catheter

= 2,= *i nasal cannule

= ? a8l /!46> ,// gtti micro

= *actulac syr x 8th ?? 'if necessary)

= or upC balance e#ery 7 hours! urine disptic

= PlanC 5chocardiography! consultation to pediatric cardiology module

Fo!!o> up

Follow up 7thDecember /, 'Day )

& C 1lusih sin 'U)! shortness of breath 'U)

2 C sensYcompos mentis 1PY,//7/ mmHg! H@Y; xi! @@Y: xi! TY07!:L8

Head C 5yes C light reflex 'UU)! isochoric pupil 0 mm!

inferior con3ucti#a palpebra

hyperemiahyperemia! icteric sclera '==)

ose C nasal flaring 'U)! eutrophy concha

5ars C within normal limits$outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral

candidiasis '=)! atrophy papilla '=)

ec C lymph node enlargement! 3ugular #enous pressure @U

cmH2

Thorax C symmetric fusiform! retraction '=)O tactile fremitus

leftYright! normal impressionO percussion resonant in

both lungsO respiratory sound #esicular! no additional

soundO ictus cordis un#isibleO single & heart sound!

murmur 'U)! systolic e3ection murmur grade ???


41/72

and renal are not palpable

5xtremities C pulse ; bpm! regular! adeuate pressure#olume! warm

axilla! capillary refill time J0! clubbing fingers 'U) digitimanus and pedis! cyanosis 'U).

% C 8yanotic congenital heart disease ec. dd ,. T2F U failure to thri#e

. TG%

0. Pulmonary atresia

P C =

=

=

=

=

1ed rest! nee chest position

@egular meals ,6// cal with : gram protein

2 *i nasal cannule

? a8l /!46> ,// gtti micro

*actulac syr x 8th ? 'if necessary)

1lood laboratory analysis

Test @esult Init @eferral

8omplete blood count

Hb ,.4 gr> ,,.0 K ,4.,

18 :.;7 x ,/0mm[ 4.6 K ,0.6

@18 7.;7 x ,/7mm[ 4.4/ K 4.4:

Hematocrite 76.6 > 09 K 4,

P*T ,,4 x ,/[mm[ ,6/ K 46/

$8< 90., f* :, K ;6

$8H 0.; pg 6 K ;

$8H8 0.9 g> ; K 0,

@D 6 > ,,.7 K ,4.:eutrophil 64.: > 09 K :/

*ymphocyte 04.9 > / K 4/

$onocyte 9.; > K :

5osinophil ,.; > , K 7

1asophil /.9 > / K ,

Fluid balance

'/7.//)

?nputY ?


42/72

leu '=)! nit '=)! uro /!! pro W! pH 6! blo '=)! &G ,!/! et '=)! bil '=)! glu '=)

Follow up 9thDecember /, 'Day 0)

& C 1lusih sin 'U)! shortness of breath 'U)2 C sensYcompos mentis 1PY,//9/ mmHg! H@Y,/: xi! @@Y: xi!

TY07!6L8





5ars C within normal limits

$outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral



cmH2







43/72

?nputY ?


44/72

The echocardiogram re#elead se#ere infundibular pulmonary stenosis with a large

but mild alignment #entricular septal defect and a large aortic o#erride 'E6/>).

There was no patent ductus arteriosus! no pericardial effusion! and no collateralimage.

8onclusionC tetralogy of fallot.

Fluid balance

'//.//)

?nputY ?


45/72



46/72



ec C lymph node enlargement! 3ugular #enous pressure @UcmH2







47/72

Follow up 0,stDecember /, 'Day 9)

& C 1lusih sin 'U)! shortness of breath 'U)\

2 C sensYcompos mentis 1PY,//9/ mmHg! H@Y,/4 xi! @@Y: xi!TY07!0L8









cmH2







48/72

Fluid reuirement for the next 7 hours Y 0// cc = :/ cc Y / cc

',:.//)

?nputY ?


49/72

?nputY ?


50/72

Fluid balance

'//.//)

?nputY ?


51/72

% C Tetralogy of fallot U failure to thri#e

P C =

==

=

=

1ed rest

@egular meals ,6// cal with : gram protein2 *i nasal cannule 'if necessary)

Propranolol 4 x ,/ mg

*actulac syr x 8th ?? 'if necessary)

PlanC cardiac catheteri+ation! phlebotomy

Fluid balance

'//.//)

?nputY ?


52/72



53/72

%nti H8< egati#e 8ut off index E ,/

Fluid balance

'//.//)?nputY ?


54/72

manus and pedis! cyanosis 'U).

% C Tetralogy of fallot U failure to thri#e

P C ==

=

=

=

=

1ed rest@egular meals ,6// cal with : gram protein

2 *i nasal cannule 'if necessary)


Paracetamol 0 x ,6/ mg 'if necessary)


PlanC cardiac catheteri+ation and phlebotomy on 9th-anuary /,0

Fluid balance

'//.//)

?nputY ?


55/72





56/72



candidiasis '=)! atrophy papilla '=)ec C lymph node enlargement! 3ugular #enous pressure @U

cmH2







57/72

2utputY ?* U I2P Y 9/ cc U ,// cc Y ,9/ cc

1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y =9/ cc

Fluid reuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc'/7.//)

?nputY ?


58/72

=

=

==

=

=

2 *i nasal cannule 'if necessary)

Three way

? 6/ gtti 'micro)?n3. cefotaxime 9// mg, hours 'day ,) 'started /9.0/)



Cardiac catheterization report (08.48-09.27)

Angiography:Hand injection at innominate ein: no per!i!tent "e#t !$%c"aian ein&ight entric"e: "arge '* aortic oerriding* !eere in#$ndi%$"ar p$"monary!teno!i!* +,A 7* mm* &,A 8* mmAortogram: good coronary artery po!tion* no co""atera"* no patent d$ct$!

arterio!$!

60


59/72

8onclusion C Tetralogy of fallot

aata index ,7:! $c Goon ratio ,!0,

ote C During procedure! ,6/ cc blood has been taen 'phlebotomy)

%d#ice C (eep following until the patient fully awae

atchout for bleeding at puncture site

8ontinue cefotaxime i.#. for total days

Follow up :th-anuary /,0 'Day ,4)

& C Post cardiac catheteri+ation day ,! 1lusih sin 'U)! shortness of breath 'U)\

64


60/72

2 C sensYcompos mentis 1PY;/9/ mmHg! H@Y,: xi! @@Y0 xi! TY0:L8


inferior con3ucti#a palpebra normal! ictericsclera '==)






cmH2







61/72





62/72

CHAPTER 4

DISCSSION

$Q! 7 years old! presented to Pediatrics Department at Ha3i %dam $ali General

Hospital 8enter on December 6th/, at ,7.// with the main complaint history

of bluish sin since he was 6 months. The complaint was experienced along with

the shortness of breath! easy of fatiguability! and hypoxic spells. Parents pro#ed

there was freuent of suatting since that. The patient was admitted to emergency

unit because he arri#ed late and pediatric"s cardiology polyclinic subunit has

closed.

%ccording to the history taing! the patient was admitted to the hospital with

cyanotic presentation. *ooing at complaint of shortness of breath and easy of

fatiguability! physicians would lie to mae diagnosis to cardiopulmonary

disease. 2nset of disease since childhood mae the diagnosis to a congenital

disease. ?n the cyanotic 8HD group! there were predominant defects! tetralogy

of Fallot 'T2F) and transposition of the great arteries 'TG%)! while T2F was the

most common and twice as pre#alent as TG% '4.9,/!/// births #s .0,/!///

births! respecti#ely).4 $ost of the newborns with TG% will die immediately while

T2F commonly result in sur#i#al to infancy and adulthood.6!;

2n examination! the patient was both centrally dan peripherally cyanotic. ?n

extremities! there was shown a grade 0 clubbing fingers. 8ardio#ascular system

re#ealed a pulse of ,// bpm! moderate #olume! and regular. The blood pressure

was ,//9/ mmHg! apex beat was localised in the 6 thleft intercostal space within

mid cla#icular line. 2n auscultation! there was loud single second heart sound of

pulmonary component! and grade ???


63/72

was a pansystolic murmur at the entire left sternal border! but seems to be

o#erlapping with e3ection systolic murmur. This physical examination mae a

stronger clinical consideration to a cyanotic congenital heart disease.

History taing of both T2F and TG% were presented similar. 1oth

presented with cyanosis! dyspnea on exertion! easy fatiguability! difficulty in

feeding! spells! and suatting.9!;?n T2F! infant will o#ercome a failure to thri#e

complication while in TG%! infant growth and de#elopment normal or sometimes

bigger. ?n auscultation! no murmur will be heard in infants with an intact

#entricular spetum except there is complication of pulmonary stenosis.; ?n this

case! the patient showed failure to thri#e condition and there was a systolic

e3ection murmur in auscultation.

?n blood laboratory analysis! the hemoglobin was ! gd* and haematocrit

was 9,>. Platelet count was low and blood gas analysis showed acidosis

metabolic with partial compensation. His serum electrolyte was normal. 8hest

radiography showed an enlarged cardiac silhoutee with left #entricular

preponderance! left sided aortic arch! and dilated pulmonary artery.

1lood laboratory analysis only showed the se#erity of hemoconcentration

and thrombocytopenia and can"t differ both T2F and TG%. ?n this patient!

planning of phlebotomy has been made and done in 9th -anuary /,0 in the

procedure of catheteri+aation ',6/ cc blood was collected). 5lectrocardiography

won"t help to differentiate both diseases also! where both showed @%D and @


64/72

?n symptomatic 7=year=old infant! surgical consideration will be preferred.76

Howe#er! surgery should ha#e been carried out in 0=,, months of age because this

option had the most rapid reco#ery from operati#e therapy.77&tudy by Poorsi!

et al. showed that complications of surgery will increase significantly after /

years of age.7:The patient"s parents has been told that his child suffered from

congenital heart disease but no furhter action was taen because lac of education

and nowledge.

Furtherly! an angiography procedure was done in order to planning surgical

inter#ention.;!6;&urgery can be done by Fontan or @astelli procedure. aata

index was ,7: and $cGoon ratio ,.0, which both didn"t fulfill the criteria for

surgery. &o! supporti#e management should be done awating for the surgery. The

patient referred to -aarta for surgical preparation and inter#ention.

6;


65/72

CHAPTER ,

SMMARY

$Q! 7 years old! presented to Pediatrics Department at Ha3i %dam $ali General

Hospital 8enter on December 6th/, at ,7.// with the main complaint history

of bluish sin since he was 6 months. The complaint was experienced along with

the shortness of breath! easy of fatiguability! hypoxic spells! and suatting. 2n

inspection and palpation! there was a presentation of cyanosis and clubbing

fingers. 2n heart auscultation! there was loud single second heart sound of

pulmonary component! and grade ???


66/72

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. @oger


67/72

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Case Report Tetralogy of Fallot-complete

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