TOF

Malformasi ini bertanggung jawab terhadap sekitar 10 persen dari semua bentuk penyakit
jantung kongenital dan merupakan penyebab paling sering dari bentuk sianotik. Keeempat
komponen tetralogy of fallot adalah defek septum ventrikel, obstruksi aliran keluar ventrikel
kanan, tumpang tindih aorta pada defek septum ventrikel dan hipertrofi ventrikel kanan.
Anomaly dasar akibat deviasi anterior dan superior septum ventrikel infundibuler menjauh lokasi
biasanya dalam jantung antara cabang septum trabekularis. ergeseran ini menyebabkan
obstruksi subpulmonaris, ! tumpang tindih" aorta dan defek septum ventrikel yang besar jenis
malalignment non restruktif.
#etralogy of $allot is the most common cyanotic heart lesion encountered in untreated patients
with cyanotic congenital heart disease who survive beyond infancy. $our structural abnormalities
constitute the tetralogy% right ventricular outflow tract stenosis, ventricular septal defect,
de&troposition of the aorta, and right ventricular hypertrophy '$ig. (()*0 and $ig. (()*1+. #here
is wide anatomic variation, with resultant physiological and clinical variations..
#etralogy of fallot merupakan lesi jantung sianotik yang palin
$,-./0 (()*0 Angiocardiogram in tetralogy of $allot1 right ventricular '/2+ selective injection
showing severe infundibular obstruction 'arrows+ and early filling of aorta 'A3+1 A, 4A, and
/A 5 main, left, and right pulmonary arteries1 6 5 parietal band1 76 5 septal band.
Angiokardiogram pada tetralogy of $allot1 ,njeksi selektif ventrikel de&tra menunjukkan
obstruksi berat infundibular 'anak panah+ dan
$,-./0 (()*1 Angiocardiogram in tetralogy of $allot with pulmonary atresia. 4ateral obli8ue
view showing large ventricular septal defect '279+ confluent with large overriding aortic orifice.
Arrow indicates atretic right ventricular outflow tract.
Embryology and Morphology
#he basic lesion is anterocephalad malalignment of the outlet septum relative to the muscular
septum. #his is associated with une8ual division of the truncus arteriosus into small pulmonary
and large aortic components. #he malalignment together with secondary hypertrophy of the
muscle form the primary site of obstruction to blood flow in the infundibulum or outflow tract of
the right ventricle '$ig. (()*0+. ,n addition, the pulmonary valve is often stenotic, and the
pulmonary valve annulus and pulmonary arteries are often hypoplastic. ,n the most severe form
'tetralogy of $allot with pulmonary atresia+, the distal infundibular outflow tract and pulmonary
valve are atretic '$ig. (()*1+, and the pulmonary artery and main pulmonary arterial branches
may be severely hypoplastic or atretic. 3ften most of the lung is supplied by large
aortopulmonary collaterals. #he ventricular septal defect is usually large, perimembranous with
outlet e&tension, and near the tricuspid and aortic valves. #he aorta arises directly over the
ventricular septal defect1 the degree of overriding varies greatly. An important diagnostic feature
is the fre8uent occurrence ':(*;+ of a right)sided aortic arch. articularly with a right)sided
aortic arch, partial deletion of chromosome (( occurs. #his is diagnosed by $,7< analysis and is
associated with thymic and parathyroid hypoplasia '9i-eorge or =A#=< (( syndrome+ and
facial hypoplasia with soft palate insufficiency 'velocardiofacial or 7hprint>en syndrome+.
Hemodynamics
6ecause of the pulmonary outflow tract obstruction, varying amounts of systemic venous
blood are shunted across the ventricular septal defect into the aorta, resulting in cyanosis.
ulmonary artery pressure and pulmonary blood flow are reduced. #he clinical features reflect
the magnitude of the pulmonary blood flow, which in turn depends on the severity of right
ventricular outflow tract obstruction, the relative resistances to ventricular outflow imposed by
the systemic and pulmonary circulations, and on any systemic)to)pulmonary collateral blood
supply via bronchial arteries or, rarely, a persistent patent ductus arteriosus.
Karena osbtruksi yang terjadi pada aliran keluar traktus pulmonal, bebagai jumlah darah
pada vena sistemik terdorong melewati kelainan septum ventrikel ke dalam aorta, menyebabkan
sianosis. #ekanan arteri pulmonal dan aliran darah pulmonal berkurang. -ambaran klinis
mencerminkan besarnya aliran darah pulmonal, yang bergantung pada beratnya obstruksi pada
aliran keluar ventrikel de&tra, resistensi relatif ke aliran keluar ventrikel dengan sistemik dan
sirkulasi pulmonal, dan suplai darah kolateral sistemik ke pulmonal melalui arteri bronchial atau,
jarang, terdapat paten ductus arteriosus.
6ecause the ventricular septal defect in the tetralogy of $allot is usually large, right ventricular
peak systolic pressure e8uals that in the left ventricle and aorta, and right and left ventricular
pressure contours are similar. /arely, the ventricular septal defect is anatomically or functionally
small because of apposition of a tricuspid valve leaflet to the defect1 right ventricular pressures
then e&ceed left ventricular pressures.
Karena defek septum ventrikel pada tetralogy of fallot biasanya luas,
#he acute severe episodes of dyspnea and hypo&emia, termed blue spells or
hypercyanotic episodes, in some infants with the tetralogy reflect a further acute reduction in the
pulmonary blood flow. #hese spells may occur even if the infant is not cyanotic at rest. #he
precipitating mechanisms are probably multiple% prolonged crying may decrease pulmonary
blood flow because of prolonged e&pirations1 decreases in right ventricular preload and systemic
vascular resistance because of sleeping, fever, or spontaneous vasomotor changes decrease
pulmonary blood flow and increase the right)to)left shunt1 and constriction of the right
ventricular infundibulum may occur, further decreasing pulmonary blood flow, although it is
uncertain whether this truly occurs.
Clinical Manifestations
#he clinical findings at birth vary with the severity of the pulmonary stenosis, but few infants
with the tetralogy of $allot remain asymptomatic or acyanotic. =yanosis may not be present at
birth1 as long as the ductus arteriosus remains patent there may be ade8uate pulmonary blood
flow, or the outflow tract obstruction may not be severe at birth. ?hen right ventricular outflow
tract obstruction and ventricular septal defect of the general morphology of the tetralogy of $allot
are present without significant right)to)left shunt, the anomaly is termed acyanotic $allot.
<ypercyanotic episodes with paro&ysmal hyperpnea may occur spontaneously or after early
morning feedings or prolonged crying. #he attacks may last only a few moments and have no
se8uelae1 they may cause obtundation, limpness, deep e&haustion, or sleep1 rarely, they may end
in unconsciousness, convulsions, or even death. 6ecause appro&imately one)third of patients
with the tetralogy of $allot begin to have hypo&ic spells by @ or * months of age, corrective
surgery is usually done electively within a few months of birth. #hus, the pediatrician rarely sees
these episodes any more. ,n the very rare instance in which an infant does not undergo
correction, e&ercise tolerance in the child varies in proportion to the severity of the cyanosis.
Aoung children with the tetralogy of $allot and severe cyanosis often adopt a characteristic
s8uatting position after e&ertion. #his maneuver results in an increased arterial o&ygen
saturation, probably by increasing systemic arterial resistance. A final group of patients shows
little or no evidence of cyanosis in infancy or early childhood 'acyanotic $allot+1 cyanosis on
e&ertion gradually becomes more manifest as they grow older. 3ccasionally, even in this group,
very rapid clinical change toward the classic severely cyanotic tetralogy can occur.
,n the rare, untreated patient, major late complications include brain abscess, cerebral thrombosis
with hemiplegia, and infective endocarditis. -rowth and development are generally delayed in
proportion to the degree of cyanosis. ,nfective endocarditis is particularly common in children
who have palliative systemic)pulmonary shunts rather than correction. rophylactic antibiotic
therapy should be given when surgical procedures involving teeth, throat, ear, nasal, bronchial,
gastrointestinal, and genitourinary tracts are undertaken.
emeriksaan fisik menunjukkan adanya
hysical e&amination shows a right ventricular systolic heave along the lower left sternal border.
A systolic thrill is usually not appreciated because the ventricular shunt is usually right to left.
#he first heart sound is usually accentuated at the lower left sternal border, and in some patients
an early systolic ejection click, aortic in origin, may be heard ma&imally at the left sternal border
and ape&. A single loud second heart sound corresponding to aortic valve closure is generally
heard best at the lower left sternal border. ?hen closure of the pulmonary valve is audible at the
upper left sternal border, it is delayed and diminished in intensity. ,n patients with moderate right
ventricular outflow tract obstruction, the systolic murmur is loud and harsh, stenotic or
pansystolic in 8uality, and best heard at the middle or lower left sternal border. #he systolic
murmur begins early in systole and stops short of the second heart sound because the murmur is
caused by outflow of blood across the right ventricular outflow tract1 in general, the more severe
the obstruction to pulmonary blood flow, the shorter the murmur. ,n e&treme pulmonary outflow
tract stenosis or pulmonary atresia, and at the height of a paro&ysmal hypo&emic spell, the
murmur may be absent or very short and faint. A faint continuous murmur may be heard over the
anterior or posterior chest, particularly in children with pulmonary atresia1 this bruit represents
flow through enlarged bronchial collateral vessels. /arely, a continuous murmur of persistent
ductus arteriosus is heard at the upper left sternal border.
Roentgenography
#he heart is of normal si>e, and lung fields are poorly vasculari>ed, signifying diminished
pulmonary blood flow '$ig. (()*(+. #he right ventricular outflow tract and main pulmonary
artery segments are usually hypoplastic, resulting in a concavity of the upper left margin of the
cardiac silhouette instead of the normal conve&ity. A characteristic BCDcoeur en sabotBC or
boot)shaped heart may be present, particularly with pulmonary atresia. #he ascending aorta is
generally large. ,n about (*; of the patients a right)sided aortic arch is present and is recogni>ed
by observing a right)sided rather than left)sided indentation on the trachea. #he superior vena
caval shadow may be displaced to the right. ?hen bronchial collateral circulation is well
developed, diffuse fine vascular markings are noted throughout the lung. /arely, markedly
decreased left lung vascular markings indicate absence or stenosis of the left pulmonary artery.
Eantung dalam ukuran normal
$,-./0 (()*( -ambar $oto rontgen pada #etralogy of fallot. Anak panah menunjukkan
=hest roentgenogram in tetralogy of $allot. Arrows indicate right)sided aortic arch and upper
thoracic aorta. 9ashed lines indicate right)sided aortic indentation on the air tracheogram.
Electrocardiography
#he electrocardiogram shows right a&is deviation and right ventricular hypertrophy, although in
the newborn infant the diagnosis of pathologic right ventricular hypertrophy by
electrocardiogram is more difficult because of the normal right ventricular dominance at this age.
,n the acyanotic tetralogy patient, combined ventricular hypertrophy may be noted at first, with
progression into pure right ventricular hypertrophy as cyanosis develops.
0lektrokardiogram menunjukkan deviasi aksis ke kanan dan hipertrofi ventrikel kanan, walaupun
pada bayi)bayi yang baru lahir diagnosis patologi hipertrofi ventrikel kanan dengan
elektrokardiogram lebih sulit karena pada usia ini dominan ventrikel kanan normal. ada pasien
tetralogy asianosis, kombinasi hipertrofi ventrikel dapat dicatat pada awalnya, dengan
perkembangan ke dalam hipertrofi ventrikel murni berkembang sianosis.
Echocardiography
,n tetralogy of $allot, the dilated aortic root overrides a large adjacent ventricular septal defect
'$ig. (()*F+, and varying degrees of right ventricular infundibular obstruction, pulmonary valve
stenosis, and hypoplasia or narrowing of the main pulmonary artery and left pulmonary artery
are revealed. 9oppler e&amination confirms the severity of the obstruction and demonstrates
systolic turbulence in the main pulmonary artery. ?ith pulmonary atresia, no flow can be
detected immediately beyond the right ventricular outflow tract, but in the pulmonary artery
there will be systolic and diastolic turbulence because of flow through the ductus arteriosus or
aortopulmonary collateral vessels into the central pulmonary arteries. 4ast, abnormal courses of
the coronary arteries are fre8uent in tetralogy of $allot and can be defined by echocardiography.
3f particular importance is the left anterior descending artery arising from the right coronary
artery and coursing across the right ventricular outflow tract. #his is particularly important to
recogni>e in the infant with severe right ventricular outflow tract obstruction, in whom a right
ventricular outflow patch is contemplated.
$,-./0 (()*F 4ong)a&is view showing the aorta 'Ao+ entering the right '/2+ and left '42+
ventricles. 4A 5 left atrium1 9 5 ventricular septal defect.
Cardiac Catheterization
,n most patients, careful evaluation and correlation of the clinical findings, roentgenogram,
electrocardiogram, and two)dimensional echocardiogram establish the diagnosis. =ardiac
catheteri>ation with selective arteriography provides more detailed morphologic and
physiological information if re8uired. ,t is usually reserved for the patient with pulmonary atresia
and is done to define the source and distribution of blood flow to the lungs '$ig. (()*@+.
$,-./0 (()*@ Angiocardiogram 'anteroposterior view+ in tetralogy of $allot with pulmonary
atresia and injection into aorta. Multiple large aortopulmonary collateral vessels and patent
ductus arteriosus providing blood flow to distal pulmonary artery branches1 retrograde filling of
small pro&imal pulmonary artery branches '/A and 4A+ and main pulmonary artery
terminating in atretic pulmonary valve '2+.
Angiokardiogram 'tampak anteropoterior+ pada #etralogy of $allot dengan atresia pulmonal dan
injeksi kedalam aorta. embuluh darah aortopulmonal kolateral yang besar dan multipel, serta
duktus arteriosus paten menyediakan aliran darah ke cabang distal arteri pulmonal1 pengisian
balik 'retrograde+ cabang arteri pulmonal proksimal kecil '/A dan 4A+ dan arteri pulmonal
utama berakhir di katup pulmonal atresia.
Treatment
As with many significant congenital heart abnormalities, the treatment is ultimately
surgical. $or the neonate with prominent cyanosis, prompt infusion of -01 is important.
=orrective surgery is usually performed within ( to @ months of birth, but in the rare patient who
has not had palliative or corrective surgery, medical therapy is primarily directed toward acute
relief of hypercyanotic episodes and preventing the complications of right)to)left shunts. A
hypercyanotic episode may be treated by placing the infant on the abdomen in a knee)chest
position or holding the infant with the legs fle&ed on the abdomen. 3&ygen should be given to
lessen dyspnea and cyanosis but is not very helpful because of the very low pulmonary blood
flow. Morphine sulfate '0.( mgGkg body weight subcutaneously+ is especially effective in
terminating a prolonged or severe attack. ,f the spell is protracted and severe and does not
respond to the foregoing therapy, metabolic acidemia ensues, and correction with intravenous
sodium bicarbonate is essential. 2asopressors can be given either early in the attack or if other
therapy fails1 phenylephrine, 0.0( mgGkg ,2 or 0.1 mgGkg ,M, will raise systemic resistance and
thus increase pulmonary blood flow. ,f possible, it should be given by continuous intravenous
infusion, generally at a dose of ( to * HIgGkgGmin. ,n infancy these attacks may be precipitated
by a relative iron)deficiency anemia 'hypochromic microcytic+, and such patients should have
iron therapy until the hematocrit reaches levels of *0 to **;. $urther increase in the hematocrit
results in a marked rise in blood viscosity, with progressive impediment to blood flow and risk of
cerebral thrombosis. Any hypercyanotic episode is an absolute indication for surgery, so it is now
rare to need to treat anemia e&cept in the immediate preoperative period. ,f surgery is
contraindicated for some reason, oral propranolol has been given at a dosage of 0.* to 1.0 mgGkg
orally every J hours to prevent or reduce the fre8uency of paro&ysmal dyspneic attacks.
0arly elective surgery is indicated for infants with tetralogy of $allot or tetralogy with
pulmonary atresia, even in the absence of symptoms. atients with the tetralogy of $allot and
patent right ventricular outflow tracts can have intracardiac surgical repair of the malformation
by skilled congenital heart surgeons in the first months of life with an operative mortality under
*;. #he ventricular septal defect is closed, the infundibular muscle is resected, and sometimes
right ventricular outflow and main pulmonary artery patches are placed to augment the outflow
tract. ulmonary valvotomy is also performed in most patients, but enlargement of the
pulmonary valve annulus with a transannular patch is avoided unless the annulus is critically
small. #his is because a patch is associated with a greater degree of pulmonary valve
insufficiency, often re8uiring homograft interposition between the right ventricle and pulmonary
artery at a later age. /arely, an infant cannot be repaired because of marked pulmonary artery
hypoplasia, and a systemic)pulmonary anastomosis or a right ventricular outflow patch is
performed without closure of the ventricular septal defect. 6alloon dilatation of the pulmonary
arteries can then be performed in the catheteri>ation laboratory in anticipation of later correction.
Although repair of tetralogy of $allot with pulmonary atresia has in recent years become
increasingly successful, the operative risk and late complications and death are higher than for
uncomplicated tetralogy. .nifocali>ation of the often discontinuous sources of pulmonary blood
flow to a central system is re8uired before the standard repair. #his may be done in one or
multiple stages. #he 10) and (0)year survival rates e&cluding operative and early hospital deaths
are appro&imately K0 and J*;, respectively, as against L*; for simple tetralogy over the same
intervals.
7urgical correction for most patients with uncomplicated tetralogy of $allot results in
e&cellent, long)lived functional results with over L0; freedom from reoperation. /esidual or
recurrent small left)to)right shunts are uncommon, but residual mild or moderate right
ventricular)pulmonary outflow tract obstruction and incompetence are common. A few patients
have surgically induced complete atrioventricular block and re8uire an implanted pacemaker.
About 1 to F; have serious late dysrhythmias, particularly ventricular tachycardia, probably
related to reentry mechanisms at the site of right ventricular tissue e&cisions, and about 1 to (;
die suddenly, presumably from dysrhythmias. 9ysrhythmias should be suspected if these patients
after surgery complain of di>>y spells, syncope, or palpitations, and appropriate diagnostic
studies and therapy applied.
Rudolph, Colin D! Rudolph, "braham M! Hostetter, Margaret #! $ister, %eorge! &iegel,
'orman ( Rudolph)s *ediatrics, +,st Edition +--. Mc%ra/0Hill
#etralogy of $allot is one of the conotruncal family of heart lesions in which the primary defect
is an anterior deviation of the infundibular septum 'the muscular septum that separates the aortic
and pulmonary outflows+. #he conse8uences of this deviation are '1+ obstruction to right
ventricular outflow 'pulmonary main pulmonary aftery is often small, and various degrees of
branch pulmonary artery stenosis may be present. =omplete obstruction of right ventricular
outflow 'pulmonary atresia with 279+ is classified as an e&treme form of tetralogy of $allot 'see
=hapter @F0.(+. #he degree of pulmonary outflow obstruction varies, with the severity of the
obstruction determining the degree of the patientMs cyanosis.
#he pulmonary valve annulus may be of or 8uite small. #he valve itself is often bicuspid and,
occasionalln is the only site of stenosis. More commons the subpulmonic or infundibular muscle,
the crista supravenrricularis, is hypertrophic, which contributes to the subvalvar stenosis and
results in an infundibular chamber of variable si>e and contour. ,f hen the right ventricular
outflow tract is completely are similar and at systemic level. A large pressure gradient occurs
across the obstructed right ventricular outflow tract, and pulmonary arterial pressure is normal or
lower than normal. #he degree of right ventricular outflow obstruction determines the timing of
the onset of symptoms, the severity of cyanosis, and when the ductus begins to close.
TETR"$O%1 OF F"$$OT
Etiology and Epidemiology
#etralogy of $allot is the most common cyanotic congenital heart defect, representing about 10;
of all congenital heart defects '$ig. 1@@)1+. Anatomically, there are four structural defects% 2&D,
pulmonary stenosis, o3erriding aorta and right 3entricular hypertrophy. #etralogy of $allot
is believed to be due to abnormalities in the septation of the truncus arteriosus into the aorta and
pulmonary arteries that occur early in gestation 'F to @ weeks+. #he 279 is large and the
pulmonary stenosis is most commonly subvalvular or infundibular. ,t may also be valvular,
supravalvular, or, fre8uently, a combination of levels of obstruction.
#etralogy of $allot merupakan kelainan jantung kongenital sianotik yang paling umum, terdapat
sekitar 10 ; dari semua kelainan jantung kongenital. 7ecara anatomi, terdapat empat kelainan
struktural 1 279, 7tenosis pulmonal, overriding aorta dan hipertrofi vetrikel kanan. #etralogy of
$allot dipercaya menjadi penyeban abnormalitas pada septal dari trunkus aorta kedalam aorta
dan arteri pulmonal yang terjadi pada awal kehamilan 'minggu F sampai @+. 279 luas dan
stenosis pulmonal lebih umum terjadi pada subvalvular atu infundibular. ,ni juga dapat terjadi
pada valvular, supravalvular atau sering kombinasi dari derajat obstruksi.
Clinical Manifestations
#he degree of cyanosis depends on the amount of pulmonary stenosis. ,nfants initially may be
acyanotic. A pulmonary stenosis murmur is the usual initial abnormal finding. ,f the
pulmonary stenosis is more severe, or as it becomes more severe over time, the amount of right)
to)left shunting at the 279 increases, and the patient becomes more cyanotic. ?ith increasing
severity of pulmonary stenosis, the murmur becomes shorter and softer. ,n addition to varying
degrees of cyanosis and a murmur, a single &+ and right 3entricular impulse at the left sternal
border are typical findings.
9erajat sianosis bergantung pada jumlah stenosis pulmonal. 6ayi)bayi awalnya asianotik.
Murmur stenosis pulmonal biasanya merupakan temuan abnormal yang ditemukan awal. Eika
stenosis pulmonal lebih berat, atau dapat menjadi lebih berat , jumlah shunt kanan ke kiri pada
279 meningkat, dan pasien menjadi lebih sianotik. 9engan meningkatnya berat stenosis
pulmonal, murmur menjadi lebih pendek dan halus. 7elain berbagai derajat sianosis dan murmur,
7( tunggal dan impuls ventrikel kanan pada linea sternalis merupakan temuan khas.
$igure 1@@)1 Tetralogy of Fallot A3, aorta1 4A, left atrium1 42, left ventricle1 A, pulmonary
artery1 /A, right atrium1 /2, right ventricle.
?hen hypo4ic 56Tet67 spells occur, they are usually progressive. 9uring a spell, the child
typically becomes restless and agitated and may cry inconsolably. An ambulatory toddler may
s8uat. <yperpnea occurs with gradually increasing cyanosis and loss of the murmur. ,n severe
spells, prolonged unconsciousness and convulsions, hemiparesis, or death may occur.
,ndependent of hypo&ic spells, patients with tetralogy are at increased risk for cerebral
thromboembolism and cerebral abscesses resulting in part from their right)to)left intracardiac
shunt.
7aat hypo4ic 58Tet97 7pells terjadi, biasanya akan berlangsung progresif. 7elama spell, anak
biasanya menjadi gelisah dan agitasi dan dapat menangis sekalipun dihibur. <yperpnea terjadi
dengan Nada spells yang berat, dapat
:maging &tudies
#he 0=- usually has right a4is de3iation and right 3entricular hypertrophy. #he classic chest
&)ray finding is a boot0shaped heart created by the small main pulmonary artery and upturned
ape& secondary to right ventricular hypertrophy. 0chocardiography shows the anatomic features,
including the levels of pulmonary stenosis, and provides 8uantification of the degree of stenosis.
=oronary anomalies, specifically a left coronary artery crossing the anterior surface of the right
ventricular outflow tract, are present in *; of patients with tetralogy of $allot.
0K- biasanya menunjukkan deviasi aksis ke kanan dan hipertrofi ventrikel kanan. O)ray dada
yang klasik ditemukan adanya gambaran jantung berbentuk seperti sepatu boot yang dibentuk
oleh karena arteri pulmonalis kecil dan upturn ape& sekunder ke hipertrofi ventrikel kanan.
0kokardiografi menunjukkan gambaran anatomi, termasuk level stenosis pulmonalis dan
memberikan kuantifikasi derajat stenosis. Anomali koroner, khususnya yang melintasi
permukaan anterior ventrikel kanan, ada pada * ; pasien dengan #etralogy of fallot.
Treatment
#he natural history of tetralogy of $allot is progression of pulmonary stenosis and cyanosis.
#reatment of hypo&ic spells consists of o&ygen administration, placing the child in the knee)
chest position 'to increase venous return+, and giving morphine sulfate 'to rela& the pulmonary
infundibulum and for sedation+. ,f necessary, the systemic vascular resistance can be increased
acutely through the administration of an P)adrenergic agonist 'phenylephrine+. ,f spells are
fre8uent, Q)adrenergic antagonists 'propranolol+ decrease muscular spasm.
/iwayat alami tetralogy of $allot adalah stenosis pulmonal progresif dan sianosis. #erapi spells
hypo&ic terdiri dari pemeberian oksigen menempatkan anak pada posisi knee-chest position
'untuk meningkatkan aliran balik vena+ dan memberikan morfin sulfat 'untuk merelaksasi
infundibulum pulmoner dan untuk sedatif+. Eika perlu, resistensi vascular sistemik dapat
ditingkatkan dengan pemeberian agonis adrenergik 'phenylephrine+. Eika 7pells sering muncul,
antagonis Q)adrenergik 'propranolol+ menurunkan spasme muscular.
Complete surgical repair with closure of the 279 and removal or patching of the pulmonary
stenosis can be performed in infancy. 3ccasionally, palliati3e shunt surgery between the
subclavian artery and pulmonary artery is performed for comple& forms of tetralogy of $allot
with more complete repair at a later time. &ubacute bacterial endocarditis prophyla4is is
indicated.
erbaikan bedah lengkap dengan menutupi 279 dan memindahkan atau menempel stenosis dapt
#liegman, Robert M Marcanted, #aren (, (enson Hal;, ;ehrman, Richard E 'elson
Essentials *ediatrics Fifth Edition Else3ier
R#et spellsR are cyanotic and hypo&ic episodes that occur in patients with tetralogy of $allot. #he
pathophysiology is felt to be related to a change in the balance of systemic to pulmonary vascular
resistance. 7pells may be initiated by events that cause a decrease in systemic vascular resistance
'e.g., fever, crying, hypotension+ or by events that cause an increase in pulmonary outflow tract
obstruction. 6oth types of events cause more right)to)left shunting and increased cyanosis.
<ypo&ia and cyanosis lead to metabolic acidosis and systemic vasodilatation, which cause a
further increase in cyanosis. Anemia may be a predisposing factor. Although most episodes are
self)limited, a prolonged #et spell can lead to stroke or death1 therefore, such a spell is an
indication that surgery may be necessary.
!#et spell" merupakan episode sianosis dan hypo&ic yang terjadi pada pasien dengan tetralogy of
$allot. atofisiologi dihubungkan dengan perubahan keseimbangan terhadap resistensi sistemik
ke resistensi vascular pulmonal. 7pells dapat dipicu oleh kejadian)kejadian yang menyebakan
penurunan resistensi vascular sistemik 'demam, menangis, hipotensi+ atau kejadian)kejadian
yang dapat menyebabkan peningkatan pada obstruksi aliran keluar traktus pulmonal. Kedua tipe
kejadian ini menyebabkan pirau dari kanan)kiri lebih besar dan meningkatkan sianosis. <ipo&ia
dan sianosis menyebabkan asidosis metabolik dan vasodilatasi sistemik, yang mana dapat
menyebabkan peningkatan sianosis lebih lanjut.
*olin, Richard, Ditmar, Mar< F =uestion you /ill be as<ed *ediatric &ecret Fourth
Edition