TETRALOGY OF FALLOT Supervisor dr. Muhaad A!i" Sp.A#$% Prese&'a'ors: Ci'ra Ar(a&'i )*)+))),) Maria&'o )*)+))++- DEPARTMENT OF PEDIATRICS MEDICAL FAC.LTY .NI/ERSITY OF S.MATERA .TARA HA0I ADAM MALI$ GENERAL HOSPITAL CENTER MEDAN -)+1 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 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 iii TA2LE OF CONTENTS Pages CO/ER.......................................................................................................... i PREFACE..................................................................................................... ii A2STRACT.................................................................................................. iii TA2LE OF CONTENTS............................................................................. i# LIST OF PICT.RES" CHARTS" AND TA2LES..................................... # CHAPTER + INTROD.CTION............................................................ , ,.,. 1acground........................................................................ , ,... 2b3ecti#es........................................................................... 0 ,.0. 1enefits.............................................................................. 0 CHAPTER - LITERAT.RE 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 DISC.SSION................................................................... 69 CHAPTER , S.MMARY...................................................................... 7/ REFERENCES i# LIST OF FIG.RES" 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. <ariables accounts for the mortality of surgical for tetralogy of fallot............................................................................................... ., Figure ..9. Perpeuating cycle in pathophysiology of hypoxic spells............... .6 Char's 8hart ..,. $ortality rate of surgical for tetralogy of fallot............................. .0 Ta6!es Table ..,. Gene mutations in tetralogy of fallot............................................. 9 Table .... 5#idence based management approach for tetralogy of fallot....... .4 # CHAPTER + INTROD.CTION +.+. 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% '4.9D,/!/// births #s ..0D,/!/// births! respecti#ely). The total pre#alence of all other cyanotic defects combined was 6.6D,/ /// births. 4 T2F is also the most common cyanotic 8HD that is liely to result in sur#i#al to infancy and adulthood. 6 5pidemiologic study showed that T2F occurs in ,/ per thousand li#e births worldwide. 7 There 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 '<&D)! right #entricular outflow tract obstruction! o#erriding of the aorta 'E6/=7/ percent)! and right #entricular hypertrophy. 9!:!; Tetralogy of Fallot was first described by Fiels &tenson in ,79, and its precise anatomical description was illustrated by Gilliam Hunter at &t Georges Hospital $edical &chool in *ondon in ,9:4. @egardless of it! T2F was credited to 5tienne=*ouis Fallot in ,::: based on his description of $%anatomie pathologi&ue de la maladie , 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. Githout surgical treatment! the estimated , year sur#i#al rate is 77> and the estimated 0/ year sur#i#al rate is 7>. Gith surgical treatment o#er :6> of children sur#i#e to adulthood. The patients who sur#i#e into adulthood without surgical treatment reHuire uniHue 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. Ghile in other hand! there needs to be further in#estigation to tailor the management of this global health problem. ,6 Indoubtedly! 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. . 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&e:i's This case report is expected to pro#ide benefits to authors and readers about better understanding in tetralogy of fallot cases. 0 CHAPTER - LITERAT.RE RE/IE3 -.+. De:i&i'io& Tetralogy of Fallot 'T2F) is a cardiac malformation comprising #entricular septal defect '<&D)! right #entricular outflow tract obstruction! o#erriding of the aorta 'J6/ percent)! and right #entricular hypertrophy. The outflow tract obstruction can be found in different types such as! obstruction in the form of infundibular stenosis '46>)! 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 acHuired 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 4 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 <%T5@). 6/> 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 '8F<s)! microdeletions ..H,,..! are nown to be the most common cause of T2F ,,!./!., with a pre#alence of , in 4/// li#e births! up to ./> of patients with classic T2F! and 4/> of those with T2F with pulmonary atresia! will ha#e microdeletions of chromosome ..H,,... The ..H,,.. 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 ..H,,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 ..H,,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! ,H.,., deletionsDduplications! 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. <arious single gene mutations are also associated with T2F. The nown mutations to date collecti#ely are 6 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 <&D! anterior malaligned #entricular septal defect! enlarged aortic root which o#errides the <&D! and right #entricular hypertrophy de#elops as a result of the compensation. 9!.6!.7 ?n .//,! the presence of a secondary! or anterior! heart field in the #entral pharyngeal mesoderm was demonstrated. This region contains a pool of F(M..6= and G%T%=4=positi#e precardiac cells!which migrate to the arterial pole of the primary heart tube.Ghen these cells reach the 2FT of the primary heart tube! they undergo myocardial differentiation induced by bone morphogenic protein . '1mp.)! a local factor that dri#es the normal de#elopment of the conotruncal segment. (elly! et al. found that the secondary heart field is also responsible for the normal @< de#elopment! at least in the mouse. @ecently! it has been demonstrated that the secondary heart field contributes cells to the early de#elopment of the myocardial wall and later to the de#elopment of the caudal wall of the aortic sac! which e#entually becomes the #ascular smooth muscle of 2FT #essels abo#e the le#el of the semilunar #al#es. The ablation of the secondary heart field leads to malformations typical of T2F. .7!.9!.: 7 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! Fx..6! dH%FD! 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 fallot 0/ 1one morphogenetic proteins! Gnt! 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 8sxDFx=..6 in cardiac mesoderm through signaling pathways that in#ol#e &mad and TGF=N inase , mediators. ?n 9 fact! the promoter of 8sxDFx=..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%FD.). 0, There are three Gnt signaling pathwaysC canonical! noncanonical and GntDcalcium. 2f these pathways! both the canonical and noncanonical pathways ha#e clear roles in early heart de#elopment. 8anonical Gnt signaling inhibits cardiac induction and maintains the secondary heart field 'pharyngeal mesoderm) in an undifferentiated state. ?ts inhibition is essential for myocardial differentiation. Foncanonical Gnt signaling! on the other hand! promotes cardiac differentiation and is specifically needed for outflow tract de#elopment. %ntagoni+ing the canonical Gnt signal by D=, or 8rescent is necessary for the expression of cardiogenic transcription factors 8sxDFx=..6 and Tbx6. %fter the heart tube has formed! canonical Gnt signaling is maintained in the secondary heart field. b=catenin is needed in ?sl,=expressing secondary heart field progenitors. *oss of Gnt signaling reduces the number of ?sl,=positi#e cells! leading to outflow tract and right #entricular defects! whereas excess Gnt signaling expands the ?sl,=positi#e population. These suggest suggest that canonical Gnt signaling is responsible for promoting proliferation and maintaining cells in a progenitor state. 0. 1eta=catenin induces both @egarding=4 and noncanonical Gnt,, expression in the secondary heart field! which also suggests that the early canonical Gnt signaling sets the stage for differentiation. Ghile the canonical Gnts are important for their ability to inhibit the initial cardiac induction! the noncanonical Gnts promote cardiac differentiation. *ie the canonical pathway! noncanonical Gnts also bind transmembrane receptorsO signaling proceeds through Dishe#eled! which modifies actin #ia the @hoD@28( and @acD-F( pathways. Foncanonical Gnt6%! in combination with canonical Gnt inhibitor D=,! induces cardiac differentiation in stromal #ascular cells. ?n addition! Gnt,, also induces cardiac differentiation in Menopus! and Gnt,,= null mice ha#e both arch artery patterning and outflow tract defects. Howe#er! ?sl, expression appears normal in these mice! : suggesting that the initial specification of the secondary heart field occurs correctly. This expression pattern supports the idea that noncanonical Gnt 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 8sxDFx=..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 8sxDFx=..6 and G%T%=4. These findings may be explained because P?0=inase could be in#ol#ed in the transcriptional loop between 8sxDFx=..6 and G%T%=4. 04 Four percents of patients with ToF ha#e an F(M..6 mutation. 06 F(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 F(M..6) cause se#eral congenital heart malformations. % dominant locus associated with cardio#ascular malformations and conduction abnormalities has been mapped to chromosome 6H06. 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 Fotch pathway and is thus a component of an intracellular signaling pathway shown to be crucial for deciding a cardiogenic progenitor cellsP 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 Fotch receptor! an e#olutionarily conser#ed signaling pathway in#ol#ed in cell fate specification. $utations in the signaling regulator Fotch, ha#e recently been implicated in ; aortic #al#e disease. $utations in PTPF,, encoding a protein tyrosinephosphatase '&HP=.) ha#e been proposed to play a role in the pathogenesis of Foonan syndrome characteri+ed by conduction defects! pulmonary stenosis! and hypertrophic cardiomyopathy!; and ha#e been also recently implicated in the pathogenesis of *52P%@D syndrome! which liely represents an allelic disorder. 0/ &ome researchers identified hetero+ygosity for a mutation in the +inc=finger protein! multi=type . 'QFP$.) 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$. gene maps to :H... 09 QFP$.! which is a +inc finger co=factor! interacts with the F=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 %DT G%T% %DG motif of DF% 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 '%FF)! 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 ./H,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 reHuires only two abnormalitiesKa <&D large enough to eHuali+e systolic pressures in both #entricles and a stenosis of the right #entricular outflow tract '@<2T) in the form of infundibular stenosis! #al#ular stenosis! or both. ,/ Gith mild stenosis! the shunt is left to right! and the clinical picture resembles that of a ,/ <&D. This is called acyanotic or pin T2F. @<H is secondary to P&! and the degree of o#erriding of the aorta #aries widely and it is not always present. ; ?n acyanotic T2F! a small to moderate left=to=right #entricular shunt is present! and the systolic pressures are eHual in the @<! *<! and aorta '). There is a mild to moderate pressure gradient between the @< and P%! and the P% pressure may be slightly ele#ated 'because of a less se#ere stenosis of the @<2T). 1ecause 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 <&D. 9!:!; ?n cyanotic T2F! the presence of se#ere P& produces a right=to=left shunt at the #entricular le#el 'i.e.! cyanosis)! with decreased P1F The P%s are small! and the *% and *< may be slightly smaller than normal because of a reduction in the pulmonary #enous return to the left side of the heart. 9!:!; ?n study! @<D*< systolic pressure ratio was found larger than /!96. 6 Figure .... Hemodynamics of acyanotic '%) and cyanotic '1) tetralogy of Fallot. Fumbers within the diagram denote oxygen saturation #alues! and those outside the diagram denote pressure #alues ; Inder pathological conditions! such as pressure and #olume o#erload! there are some indications that cardiomyocytes reinitiate myocyte differentiation in the case of compensatory hypertrophy. ?mmediate early genes 'lie proto=oncogenes! c=fos! c=3un and c=myc) and genes from the fetal period 'lie %FF! S=$H8 and R= seletal actin) ha#e been implicated in the cellular alterations leading to ,, #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 @< compliance and the e3ection fraction may be decreased. 4. -.4. Dia5&osis 8linical findings associated with se#ere! longstanding cyanosis include polycythemia 'abnormal increase of red blood cells)! difficulty in feeding! failure to gain weight! retarded growth and physical de#elopment! dyspnea! on exertion! and some times cerebral thrombosis. Fowadays clubbing of the fingers and toes! pulmonary haemorrhage! stroe and infections of the brain and se#ere hypoxic spells rarely de#elop. 9!:!; ?n physical examination! we can find centrally or peripherally cyanotic! clubbing fingers! dyspnea! failure to thri#e! and abnormality in heart auscultation. 8yanotic! se#erity regarding of the degree of @<2T2 and the worsening of infundibular stenosis and polycythemia. 2nset of cyanosis usually obser#ed wees after deli#ery when the ductus begins to close as PD% in the early postnatal life redirect a large portion of partially oxygenated blood lea#ing the heart for the body to the lungs increasing flow through the pulmonary circulation with relati#ely better oxygenation. ?f the cyanosis is around the lips! or on the face! hands or feet but not the tongue! then this is more liely to be due to reduced sin circulation and is not caused by T2F. 9!; 8lubbing finger is defined by strctural changes at the base of nails that results in a con#ex distal phalanges. The sub3ecti#e sign of clubbing finger can be determined by the schamroth sign test. The unpresented of diamond shape hole is being called clubbing fingers. The ob3ecti#e test for clubbing finger can be done by measuring D?P. D?P ratio of , or greater at any single digit is suggesti#e of ,. clubbing! the sum of the ,/ ratios is more specific for clubbing. 8alculation of the phalangeal depth ratio ratio of distal phalangeal depth to interphalangeal 3oint depth of index finger ratio E, also indicates clubbing. 9!40 Figure ..0. 2b3ecti#e measurement of clubbing fingers 40 8lubbing is caused by soft tissue growth under the nail bed as a conseHuence of central cyanosis. ?t was hypothesi+ed is that megaaryocytes present in the systemic #enous blood may be responsible for the change. ?n normal persons! platelets are formed from the cytoplasm of the megaaryocytes by fragmentation during their passage through the pulmonary circulation. The cytoplasm of megaaryocytes contains growth factors 'e.g.! platelet=deri#ed growth factor and transforming growth factor 1). ?n patients with right=to=left shunts! megaaryocytes with their cytoplasm may enter the systemic circulation! become trapped in the capillaries of the digits! and release growth factors! which in turn cause clubbing. 8lubbing usually does not occur until a child is 7 months or older! and it is seen first and is most pronounced in the thumb. ?n the early stage! it appears as shininess and redness of the finger tips. Ghen fully de#eloped! the fingers and toes become thic and wide and ha#e con#ex nail beds. 8lubbing is also seen in patients with li#er disease or subacute bacterial endocarditis and on a hereditary basis without cyanosis. 9!;!40 $ost infants with T2F will also experience failure to thri#e and difficulty in feeding. %symmetric crying facies can be found in T2F. ?t is caused by agenesis or hypoplasia of the depressor anguli oris muscle on one side of the mouth. 44 Hemoptysis may also be found in older child. ?t happens due to the rupture of bronchial colaterals. 46 2n heart auscultation! can be heard a single &. sound because only aortic component can be heard while the pulmonary component is too soft to be heard. ,0 % long! loud e3ection type systolic murmur is heard at the mid and upper left sternal borders because of pulmonal stenosis. Pansystolic murmur was resulted from <&D and infundibular stenosis that audible along at the entire left sternal border. % low=pitched early ending diastolic murmur suggests se#ere pulmonary regurgitation. % long! loud e3ection systolic murmur indicates @<2T2! a high= pitched diastolic murmur indicates aortic regurgitation. 9!;!47 ?n blood laboratory analaysis! polycythemia and trombositopenia will present. ?schemia and hypoperfusion will acti#ate -%(. =&T%T0 pathway!and subseHuently acti#ateP?0(D%t pathway that will lead to polychytemia. The -%(. protein is especially important for controlling the production of blood cells from hematopoietic stem cells. These stem cells are located within the bone marrow and ha#e the potential to de#elop into red blood cells. $any findings also demonstrate that -%(. <7,9F induces 5po=independent expansion of the erythroid lineage in #i#o. 49 Four pathogenetic mechanisms are potentially responsible for thrombocytopenia in were decreased platelet production! decreased megaaryocyte production! increased platelet destruction! and increased platelet acti#ation. ?t hypothesi+ed that right=to=left shunts deli#er whole megaaryocytes into the system arterial circulation! bypassing the lungs where megaaryocytic cytoplasm is fragmented into platelets! thus reducing platelet production. $egaaryocytes that lodge in the capillaries of the digits and periostium release PDGF and TGF=N. Therefore! platelet production will decrease. 4: ?n M=@ay! the cardiac silhouette in the %P pro3ection shows Tcoer in cabotT features due to the lac of pulmonary arch and ele#ation of the heart apex. @ight atrial enlargement '.6>) and right aortic arch '.6>) may be present. ?n the lateral #iew! there is approximation of the cardiac shadow to the thorax anterior wall '@<H). *ung fields indicate normal or diminished blood flow depending on the degree of pulmonic stenosis. &ometimes increased collateral #asculature can be seen. 9!; 5lectrocardiography examination shows righ axis de#iation 'U,./ to U,6/ degree) in ;/> cases. Howe#er! in cyanotic form! V@& axis can be normal. ?n ,4 general! 58G will show complete right bundle branch bloc! with the V@& width E,:/ ms! reflecting the degree of @< dilation. P wa#e higher 6/> 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 < , and < . will be obser#ed with probably normal &T=T wa#e configuration if the systolic pressure in the right #entricle is between 7/=:/ mm Hg. ?f the pressure inside the right #entricle surpasses the ,// mm Hg barrier! taller @ wa#e in lead <, 'more than ,/ mm Hg) with secondary &T=T changes in the right precordial leads! ?! ???! a<F and a<@ will be present. ?n any e#ent! the absence of significant secondary &T=T changes does not rule out extreme systolic right #entricular o#erload. ;!47!4; 5chocardiogram is #ery helpful in confirming the diagnosis and in e#aluating se#eral of the issues related to T2F. 5nlargement of the @<! large <&D! aortic o#erride and right #entricular outflow tract obstruction can be imaged. &i+e of the main and proximal branch pulmonary arteries and any additional sources of flow to the lungs can be e#aluated. %lthough! the distal pulmonary arteries cannot easily be seen by echocardiogram. ;!46!4; 8omputeri+ed tomography and magnetic resonance imaging '$@?) are 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 reHuired. 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 ,6 used to Huantitae 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. Formal #alues are .=..6 and good Fontan candidates should ha#e ratio of E,.:. Faata 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. Formal #alues is 00/ W 0/ mm . D1&% 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, /./,=/../ XgDgDmin 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 childPs 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 mgDg 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 ,7 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 reHuiring treatment de#eloped in ; of :9 patients ',/>)! atrial fibrillation or flutter in 9 ':>)! and #entricular arrhythmias in . '.>). the difference in freHuency. 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 <i#ien Thomas! a 1laloc=Taussig shunt! done in a child at -ohn Hopins Hospital in ,;64. 6. This shunt is anastomosed between the subcla#ian artery and the ipsilateral pulmonary artery! a right=sided shunt is performed in patients with left aortic archO a left=sided shunt is performed for right aortic arch. 60 ?n ,;7.! 1T& was taen further by (linner and de *e#al using a 0.6 or 4./ mm thin=walled polytetraZuoroethylene prosthetic graft! called gore=Tex interposition shunt. 64 1T& is more liely to done for infants older than 0 months and gore=Tex interposition shunt is the procedure of choice for older infants. ; ?n .//0 &ano and colleagues reported a modification of the Forwood procedure by placing a larger ,9 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 'VpDVs). 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.! Gaterson introduced a Gaterson shunt! anastomosed between the ascending aorta and the right pulmonary artery. Potts and Gaterson shunt is no longer performed either because these procedures included too large a shunt leading to 8HF andDor 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 fallot .6 ,: The first total correction of a tetralogy of Fallot was achie#ed in %pril ,;64 by *illehei! using the cross circulation techniHue in a patient aged ten months! and subseHuently 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 #entricular
infundibulum. 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 of
transanular 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 fallot 69 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 for patients. For at least . decades! initial palliation
followed by reparati#e operation later in childhood was the
most 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 primary
repair was extended to symptomatic neonates! and low operati#e
mortality 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 ,; 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 subseHuent 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 techniHue 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 conseHuences in terms of #entricular
dimensions and function! exercise capacity! arrhythmias! heart
failure! and sudden cardiac death leading to late reoperation
to address pulmonary insufficiency and right #entricular #olume
o#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 adeHuate 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 transatrialDtranspulmonary 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 eHual 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/ <ariables that is responsible for higher mortality wereC ./ Figure ..7. <ariables accounts for the mortality of surgical for tetralogy of fallot 6;!7/ (anter! et al. study in 09 neonates with T2F or its #ariants showed that no statistically significant differences in early mortality comparing shunted patients with primarily repaired patients 'pJ/!/6). 2n follow=up out to se#en years! the freedom from death or reoperation was not different between the two groups. The primary repair patients more freHuently had a transannular patch and a tendency to more freHuent delayed sternal closure while the shunted patients had shorter intensi#e care unit and hospital stays for the first operation! which became eHui#alent when the second hospitali+ation 'repair) #alues were added. 7.
This single stage repair has been prefered in infants! more recently in neonates! especially in uncomplicated T2F. ?n single stage repair! the most feared prominent complication was late ad#erse outcome of surgery. ?n two stage repair! the complications were long=term hypoxaemia contributes to cardiomyocytic degeneration and interstitial fibrosis! which ha#e been implicated in myocardial dysfunction and #entricular arrhythmias. 6 The exception comes when the baby is se#erely cyanotic where shunting should be done as soon as possible to pre#ent se#ere hypoxemia. 64!7/ $any authors proposed a selecti#e indications for two staged procedure! with indications for two stage procedures were in se#erely cyanotic infants or unmanageable hypoxic spells especially with low birth weight and younger than 0 months of age! T2F with pulmonary atresia or hy hypoplastic pulmonary annulus. 6. The procedure of surgery is performed after standard median sternotomy using full=Zow ',6/ to .6/ m*Dm . ) cardiopulmonary bypass with moderate ., systemic hypothermia '.6L8 to .:L8) and blood or crystalloid prime. 70 The <&D was closed with a double #elour Dacron patch using simple! interrupted! 6=/ sil sutures and opens the right #entricular outflow tract by remo#ing some thicened muscle below the pulmonary #al#e! repairing or remo#ing the obstructed pulmonary #al#e and! if needed! enlarging the branch pulmonary arteries that go to each lung with autologous pericardium. 6; Vuestions were also remaning about timing to do the repair surgical. This is because absence of consensus persists regarding the optimal procedure
and timing for the surgical treatment of young infants with
symptomatic tetralogy of Fallot. 74 Thus many recent surgical series ha#e reported successful outcomes with a #ery low mortality rate! many surgeons are still debated about the optimal age for surgery. 64 $any studies had indicated the mortality rate regarding of the age of surgery althoung in &chult+! et al. showed that age at operation less than 0 months or weight at operation less than 7 g did not ha#e an effect on operati#e or long= term mortality 'pJ/!//,). 6; The author said! only #ariables listed abo#e are responsible for mortality rate in T2F surgery. ?n fact! surgical repair was preferred in babies aged E0 months because J0 months! is still occur physiological changes and if correction was done #ery early! neonatal left #entricle has less ability to adapt to sudden increases in stroe #olume and ha#e less ability to and neonates are more susceptible to the ad#erse effect of cardiopulmonary bypass. 4. ?n ?ndian concensus of T2F surgery! if patient remained stable! surgery was done on ,=. years of age! if the patient cyanotic! J 0 months! shuting is preferred! and if E 0 monts! surgical inter#ention is preferred '8lass ?). 76 ?n Par! et al. textboo! the suggested surgical timing is soon in symptomatic infants! 0=.4 months in asymptomatic or minimally cyanotic children! ,=. years in mildly cyanotic infants with pre#ious shunting! ,=. years in pin fallot! and 0=4 years in asymptomatic children with coronary artery anomalies. ; ?n (arl! et al. study! it showed that 0 months of age has been associated with longer intensi#e care and hospital stay than in those older than 0 months! suggesting that the optimum age of electi#e repair is 0=7 months of age. 6 .. &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). Fo 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. Fo 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 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! @<H! $%P8%&! and hypoxemia. 64 Howe#er! the need of postoperati#e follow up is important. ?t is recommended to follow up e#ery 7=,. months! watching out for arrythmias and &15! and limitation of #arying le#els of acti#ity. ; Table .... 5#idence based management approach for tetralogy of fallot 47 -.,.-. Ma&a5ee&' o: h(po;i7 spe!!s Tet spell also called hypoxic spell! cyanotic spell! hypercyanotic spell! paroxysmal dyspnea is an episodic central cyanosis due to total occlusion of right #entricle outflow. Tet spell are characterised by period of uncontrollable crying! irritability! rapid and deep breathing 'hyperpnea)! deepening of cyanosis! decreased intensity of heart murmur. ?f not treated in time it may lead to limpness! sei+ures! neurological deficit and death. % spell is most liely to be seen a child less than . year old! upon waing up in the morning and following a crying episode. 7; Tet spell occured because imbalance between pulmonary and systemic #ascular resistance fa#ouring decreased pulmonary flow and increased right=to= left shunting. The precise mechanism of these episodes is not clear! but presumably results from a transient increase in pulmonary resistance. Precipitating .4 factors such as crying! defecation! feeding! waing from naps 'low systemic resistance)! fe#er! dehydration! tachypneaDtachycardia 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 spells 9, $anagement of hypoxic spellsC ;!9. ,. (nee=to=chestDsHuatting. *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 <&D. 1lood will be oxygenated and infant will temporarily relie#ed. This posture also increased .6 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 /!,=/!. mgDg 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 ,/=./ mlDg 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 , m5HDg i.#. repeat in ,/=,6 minutes may reduce the respiratory centre stimulating effect of acidosis. 7. Propranolol starting at .=6 XgDg and titrating up to ,/=./ XgDg ?< 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 /!/. mgDg i#! etamin ,=0 mgDg i# o#er , minute increases the systemic #ascular resistance and sedates the infant. -.<. Cop!i7a'io& 8omplications of tetralogy of fallot areC 9!;!6: - 1acterial endocarditis may occur in the right #entricular infundibulum or on the pulmonic! aortic! or! rarely! tricuspid #al#es. - %rrythmia! heart bloc - 8erebral thrombosis because of polycythemia and dehydration. - 1rain abscess - 8oagulopathy because of long standing cyanosis - $ental retardation - Heart failure - ?ron deficiency anemia .7 - 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>. Fearly 9/> of patients with TF reHuire 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#al
at .6 years postoperati#ely was ;4.4>. There was no significant
relationship 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 <&D. @e=operation for pulmonary #al#e replacement is reported to be performed in about ;> of the TF patients. The actuarial freedom from reoperation was :6=:;> at ,6 years. 6!4.!96 CHAPTER 1 .9 CASE REPORT IDENTITY Fame 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! .6 th ./,. $Q! a 7 years old boy! weight 0., g! height ;: cm! presented to Pediatrics Department at Ha3i %dam $ali General Hospital 8enter on December .6 th ./,. 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 sHuatting 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 freHuently 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. FreHuency .= 0 times a day and each attac lasted 6=./ minutes. &ince 6 years old! the attac was decreasing in freHuency ',=. times in a wee). Patient used sHuatting position to relie#e his breath. This complaint mostly appeared when the patient was crying! feeding! dan waing up in the morning. .: 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. Fo 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. Fo 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 .; 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 Galing 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 Huestions 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/ 1GDage C ., ,4 x ,//> Y 79> 1*Dage C ,,7 ;: x ,//> Y :4> 1GD1* C ,6 ,4 x ,//> Y ;0> Presence &tatus &ensoriumC alert! 1PY ,//D7/ mmHg! H@Y ,,/ bpm! @@Y 0/ xDmin! temperatureC 09 o 8. %nemic '=)! dyspnea 'U)! cyanotic 'U)! edema '=)! icteric '=). 1ody weight '1G)C ,4 g. 1ody length '1*)C ;: cm. 8D8C 1GD%ge Y 79>! 1*D%ge Y :4>! 1GD1* Y ;0>. *ocali+ed &tatus Head C 5yeC light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=). FoseC nasal flaring 'U) 5arsC within normal limits $outhC lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec 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 ???D<?. %bdomen C &oepel 'U)! peristaltic 'U)! epigastric pain '=)! li#er! spleen! and renal not palpable. 5xtremities C pulse ;. bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). Genitalia C $ale! within normal limit. 2!ood La6ora'or( Resu!' -, 'h De7e6er -)+- 0, 8omplete 1lood 8ount @esult Init @eferral Hb .... gr> ,,.0 K ,4., G18 ,7.0; x ,/ 0 Dmm[ 4.6 K ,0.6 @18 ;.7, x ,/ 7 Dmm[ 4.4/ K 4.4: Hematocrite 9, > 09 K 4, P*T ,/6 x ,/[Dmm[ ,6/ K 46/ $8< 90.; f* :, K ;6 $8H .0., Pg .6 K .; $8H8 0,.0 g> .; K 0, @DG .6.4 > ,,.7 K ,4.: Feutrophil :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 mmolD* ..=.7 Total 82. ;./ mmolD* ,;=.6 1ase5xcess =,9.; mmolD* '=.) = 'U.) 2. &aturation ;/.; > ;6 K ,// 8onclusionCse#ere acidocis metabolic with partial compensation! normoxemia 5lectrolyte @esult Init @eferral &odium ,4. m5HD* ,06=,66 (alium 4., m5HD* 0.6=6.6 8hloride ,,0 m5HD* ;7=,/7 Ches' @ARa( 0. %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 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<*! &T=T changes 'U)! T tall 'U) on <0 and <6! *<H '=). Di::ere&'ia! Dia5&osis: 04 8yanotic 8ongenital Heart Disease ec. ddD ,. 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 . ,=. *Di nasal cannule = ?<FD D6> Fa8l /!46> ,// gttDi micro = *actulac syr . x 8th ?? 'if necessary) = Gor upC balance e#ery 7 hours! urine disptic = PlanC 5chocardiography! consultation to pediatric cardiology module Fo!!o> up Follow up .7 th December ./,. 'Day .) & C 1lusih sin 'U)! shortness of breath 'U) 2 C sensYcompos mentis 1PY,//D7/ mmHg! H@Y;. xDi! @@Y.: xDi! TY07!:L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! 06 and renal are not palpable 5xtremities C pulse ;. bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C 8yanotic congenital heart disease ec. ddD ,. 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 . . *Di nasal cannule ?<FD D6> Fa8l /!46> ,// gttDi micro *actulac syr . x 8th ? 'if necessary) 1lood laboratory analysis Test @esult Init @eferral 8omplete blood count Hb .,.4 gr> ,,.0 K ,4., G18 :.;7 x ,/ 0 Dmm[ 4.6 K ,0.6 @18 7.;7 x ,/ 7 Dmm[ 4.4/ K 4.4: Hematocrite 76.6 > 09 K 4, P*T ,,4 x ,/[Dmm[ ,6/ K 46/ $8< 90., f* :, K ;6 $8H .0.; pg .6 K .; $8H8 0..9 g> .; K 0, @DG .6 > ,,.7 K ,4.: Feutrophil 64.: > 09 K :/ *ymphocyte 04.9 > ./ K 4/ $onocyte 9.; > . K : 5osinophil ,.; > , K 7 1asophil /.9 > / K , Fluid balance '/7.//) ?nputY ?<FD U Diet Y 46/ cc U ,// cc Y 46/ cc 2utputY ?G* U I2P Y 9/ cc U .6/ cc Y 0./ cc 1alanceY ?nput K 2utput Y 46/ cc K 0./ cc Y ,0/ cc Fluid reHuirement for the next 7 hours Y 0// cc K ,0/ cc Y ,9/ cc ',..//) ?nputY ?<FD U Diet Y 06/ cc U ,6/ cc Y 6// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput K 2utput Y 6// cc K ,9/ cc Y 00/ cc Fluid reHuirement for the next 7 hours Y 0// cc K 00/ cc Y =0/ cc ',:.//) ?nputY ?<FD U Diet Y 06/ cc U .// cc Y 66/ cc 2utputY ?G* U I2P Y 9/ cc U ,.6 cc Y ,;6 cc 1alanceY ?nput K 2utput Y 66/ cc K ,;6 cc Y 006 cc Fluid reHuirement for the next 7 hours Y 0// cc K 006 cc Y =06 cc Irine disptic ',:.//) 07 leu '=)! nit '=)! uro /!.! pro W! pH 6! blo '=)! &G ,!/.! et '=)! bil '=)! glu '=) Follow up .9 th December ./,. 'Day 0) & C 1lusih sin 'U)! shortness of breath 'U) 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y,/: xDi! @@Y.: xDi! TY07!6L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ,/: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C 8yanotic congenital heart disease ec. ddD ,. 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 . . *Di nasal cannule ?<FD D6> Fa8l /!46> ,// gttDi micro Propranolol 4 x,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC echocardiography! phlebomtomy Fluid balance '//.//) ?nputY ?<FD U Diet Y ,// cc U .// cc Y 0// cc 2utputY ?G* U I2P Y 9/ cc U .6/ cc Y 0./ cc 1alanceY ?nput = 2utput Y 0// cc = 0./ cc Y =./ cc '/7.//) ?nputY ?<FD U Diet Y ,// cc U ,// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput K 2utput Y .// cc K ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',..//) 09 ?nputY ?<FD U Diet Y ,// cc U ,// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U 6/ cc Y ,./ cc 1alanceY ?nput K 2utput Y .// cc K ,./ cc Y :/ cc Fluid reHuirement for the next 7 hours Y 0// cc = :/ cc Y ../ cc ',:.//) ?nputY ?<FD U Diet Y .// cc U ,6/ cc Y 06/ cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y 06/ cc = ,9/ cc Y ,:/ cc Fluid reHuirement for the next 7 hours Y 0// cc = ,:/ cc Y ,./ cc Irine dipstic '//.//) leu '=)! nit '=)! uro /!.! pro W! pH 6! blo '=)! &G ,!//6! et '=)! bil '=)! glu '=) ',..//) leu '=)! nit '=)! uro /!.! pro U! pH 7! blo '=)! &G ,!/,6! et '=)! bil '=)! glu '=) ',:.//) leu '=)! nit '=)! uro /!.! pro U! pH 7! blo '=)! &G ,!/.6! et '=)! bil '=)! glu '=) Follow up .: th December ./,. 'Day 4) & C 1lusih sin 'U)! shortness of breath 'U) 2 C sensYcompos mentis 1PY;/D7/ mmHg! H@Y,,/ xDi! @@Y.4 xDi! TY07!9L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ,,/ bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C 8yanotic congenital heart disease ec. ddD ,. T2F U failure to thri#e .. TG% 0. Pulmonary atresia P C = = = = = = 1ed rest @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule ?<FD D6> Fa8l /!46> 6/ gttDi micro Propranolol 4 x,/ mg *actulac syr . x 8th ?? 'if necessary) @esults of echocardiography 0: 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 collateral image. 8onclusionC tetralogy of fallot. Fluid balance '//.//) ?nputY ?<FD U Diet Y ,// cc U ,6/ cc Y 06/ cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y 06/ cc = ,9/ cc Y ,:/ cc Fluid reHuirement for the next 7 hours Y 0// cc = ,:/ cc Y ,./ cc '/7.//) ?nputY ?<FD U Diet Y 6/ cc U = Y 6/ cc 2utputY ?G* U I2P Y 9/ cc U = Y 9/ cccc 1alanceY ?nput = 2utput Y 6/ cc = 9/ cc Y =./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ./ cc Y 0./ cc ',..//) ?nputY ?<FD U Diet Y ,// cc U ,// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U .6/ cc Y 0./ cc 1alanceY ?nput = 2utput Y .// cc = 0./ cc Y =,./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,./ cc Y 4./ cc ',:.//) ?nputY ?<FD U Diet Y .// cc U ,// cc Y 0// cc 2utputY ?G* U I2P Y 9/ cc U .6/ cc Y 0./ cc 1alanceY ?nput = 2utput Y 0// cc = 0./ cc Y =./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ./ cc Y 0./ cc Irine dipstic '//.//) leu '=)! nit '=)! uro /!.! pro W! pH 6! blo '=)! &G ,!//6! et '=)! bil '=)! glu '=) ',..//) leu '=)! nit '=)! uro /!.! pro U! pH 7! blo '=)! &G ,!/,6! et '=)! bil '=)! glu '=) ',:.//) leu '=)! nit '=)! uro /!.! pro U! pH 7! blo '=)! &G ,!/.6! et '=)! bil '=)! glu '=) Follow up .; th December ./,. 'Day 6) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y,// xDi! @@Y.. xDi! TY07L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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! 0; murmur 'U)! systolic e3ection murmur grade ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ,// bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = = = = = 1ed rest! nee chest position @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule ?<FD D6> Fa8l /!46> 6/ gttDi microaff Propranolol 4 x,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation! phlebotomy Fluid balance '//.//) ?nputY ?<FD U Diet Y ,// cc U ,// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput K 2utput Y .// cc K ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc '/7.//) ?nputY ?<FD U Diet Y ,// cc U / cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U 6/ cc Y ,./ cc 1alanceY ?nput = 2utput Y ,// cc = ,./ cc Y =./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ./ cc Y 0./ cc ',..//) ?nputY ?<FD U Diet Y 6/ cc U ,./ cc Y ,9/ cc 2utputY ?G* U I2P Y 9/ cc U 6/ cc Y ,./ cc 1alanceY ?nput = 2utput Y ,9/ cc = ,./ cc Y 6/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 6/ cc Y .6/ cc '/:.//) ?nputY ?<FD U Diet Y ,// cc U ,// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U 6/ cc Y ,./ cc 1alanceY ?nput = 2utput Y .// cc = ,./ cc Y :/ cc Fluid reHuirement for the next 7 hours Y 0// cc = :/ cc Y ../ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 6! blo '=)! &G ,!/./! et '=)! bil '=)! glu '=) Follow up 0/ th December ./,. 'Day 7) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y;: xDi! @@Y.. xDi! TY07!0L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits 4/ $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti 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 ?<FD D6> Fa8l /!46> 6/ gttDi microaff 2 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation! phlebotomy Fluid balance '//.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U 6/ cc Y ,./ cc 1alanceY ?nput = 2utput Y .// cc = ,./ cc Y :/ cc Fluid reHuirement for the next 7 hours Y 0// cc = :/ cc Y ../ cc '/7.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',..//) ?nputY ?<FD U Diet Y = U ,./ cc Y ,./ cc 2utputY ?G* U I2P Y 9/ cc U 6/ cc Y ,./ cc 1alanceY ?nput = 2utput Y ,./ cc = ,./ cc Y / cc Fluid reHuirement for the next 7 hours Y 0// cc = / cc Y 0// cc ',:.//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y .6/ cc = 09/ cc Y =,./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,./ cc Y 4./ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 6! blo '=)! &G ,!/./! et '=)! bil '=)! glu '=) 4, Follow up 0, st December ./,. 'Day 9) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y,/4 xDi! @@Y.: xDi! TY07!0L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ,/4 bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = = = = 1ed rest! nee chest position @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation! phlebotomy Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y ,// cc = 09/ cc Y =.9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U .9/ cc Y 69/ cc '/7.//) ?nputY ?<FD U Diet Y = U ,96 cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U .6 cc Y ;6 cc 1alanceY ?nput = 2utput Y .// cc = ;6 cc Y ,/6 cc Fluid reHuirement for the next 7 hours Y 0// cc = ,/6 cc Y ,;6 cc ',..//) ?nputY ?<FD U Diet Y = U 0// cc Y 0// cc 2utputY ?G* U I2P Y 9/ cc U ,6/ cc Y ../ cc 1alanceY ?nput = 2utput Y 0// cc = ../ cc Y :/ cc 4. Fluid reHuirement for the next 7 hours Y 0// cc = :/ cc Y ../ cc ',:.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y .// cc = 0/ cc Y ,9/ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 6! blo '=)! &G ,!/,/! et '=)! bil '=)! glu '=) Follow up , st -anuary ./,0 'Day :) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y,// xDi! @@Y.7 xDi! TY07!6L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ,// bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti 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 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation! phlebotomy Fluid balance '//.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,6/ cc Y ../ cc 1alanceY ?nput = 2utput Y .// cc = ../ cc Y =./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ./ cc Y 0./ cc '/7.//) 40 ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y .9/ cc Fluid reHuirement for the next 7 hours Y ,// cc = .9/ cc Y =,9/ cc ',..//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',:.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y .// cc = 0/ cc Y ,9/ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/,6! et '=)! bil '=)! glu '=) Follow up . nd -anuary ./,0 'Day :) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y;: xDi! @@Y.: xDi! TY07!6L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = = = = 1ed rest! nee chest position @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation! phlebotomy 44 Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc '/7.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y .9/ cc Fluid reHuirement for the next 7 hours Y ,// cc = .9/ cc Y =,9/ cc ',..//) ?nputY ?<FD U Diet Y = U 0// cc Y 0// cc 2utputY ?G* U I2P Y 9/ cc U .// cc Y .9/ cc 1alanceY ?nput = 2utput Y 0// cc = .9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',:.//) ?nputY ?<FD U Diet Y = U .6/ cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,6/ cc Y ../ cc 1alanceY ?nput = 2utput Y .// cc = ../ cc Y ./ cc Fluid reHuirement for the next 7 hours Y .// cc U ./ cc Y ../ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/./! et '=)! bil '=)! glu '=) Follow up 0 rd -anuary ./,0 'Day :) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y;: xDi! @@Y.4 xDi! TY07!,L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). 46 % C Tetralogy of fallot U failure to thri#e P C = = = = = 1ed rest @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation! phlebotomy Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc '/7.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',..//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y .6/ cc = 09/ cc Y =,./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,./ cc Y 4./ cc ',:.//) ?nputY ?<FD U Diet Y = U 0// cc Y 0// cc 2utputY ?G* U I2P Y 9/ cc U .6/ cc Y 0./ cc 1alanceY ?nput = 2utput Y 0// cc = 0./ cc Y =./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ./ cc Y 0./ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/./! et '=)! bil '=)! glu '=) Follow up 4 th -anuary ./,0 'Day ;) & C 1lusih sin 'U)! shortness of breath 'U)\! fe#er 'U) 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y;. xDi! @@Y.4 xDi! TY09!;L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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! 47 murmur 'U)! systolic e3ection murmur grade ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;. bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = = = = = 1ed rest! nee chest position @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg Paracetamol 0 x ,6/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation and phlebotomy on 9 th -anuary ./,0 1lood laboratory test for cardiac catheteri+ation preparation Test @esult Init @eferral 8omplete blood count Hb ..., gr> ,,.0 K ,4., G18 9.9. x ,/ 0 Dmm[ 4.6 K ,0.6 @18 ;.69 x ,/ 7 Dmm[ 4.4/ K 4.4: Hematocrite 9/.4 > 09 K 4, P*T 9/ x ,/[Dmm[ ,6/ K 46/ $8< 90.7 f* :, K ;6 $8H .0., pg .6 K .; $8H8 0,.4 g> .; K 0, @DG .7.. > ,,.7 K ,4.: Feutrophil 7,.4 > 09 K :/ *ymphocyte .6.0 > ./ K 4/ $onocyte ;., > . K : 5osinophil 0.4 > , K 7 1asophil /.: > / K , *i#er function test Total bilirubin /.9, mgDd* J , Direct bilirubin /.47 mgDd* /=/.. %*P .,/ ID* J.7; %&TD&G2T :0 ID* J0: %*TD&GPT ,/ ID* J4, @enal function test Ireum 06 mgDd* J6/ 8reatinin /.00 mgDd* /.0.=/.6; Iric %cid ;.6 mgDd* J9./ ?mmunoserologic Hbs%g Fegati#e 8ut off index E ,/ %nti=Hbs Fegati#e Positi#e %nti H%< ?g$ Fegati#e Fegati#e J ,./ 49 %nti H8< Fegati#e 8ut off index E ,/ Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc '/7.//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .6/ cc = ,9/ cc Y :/ cc Fluid reHuirement for the next 7 hours Y 0// cc = :/ cc Y ../ cc ',..//) ?nputY ?<FD U Diet Y = U 0// cc Y 0// cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y 0// cc = 09/ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc ',:.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y .// cc = 0/ cc Y ,9/ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/./! et '=)! bil '=)! glu '=) Follow up 6 th -anuary ./,0 'Day ,/) & C 1lusih sin 'U)! shortness of breath 'U)\! fe#er '=) 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y:: xDi! @@Y.4 xDi! TY07L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse :: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti 4: 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 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg Paracetamol 0 x ,6/ mg 'if necessary) *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation and phlebotomy on 9 th -anuary ./,0 Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc '/7.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',..//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y .6/ cc = 09/ cc Y =,./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,./ cc Y 4./ cc ',:.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y .9/ cc Fluid reHuirement for the next 7 hours Y 0// cc = .9/ cc Y =,9/ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/,6! et '=)! bil '=)! glu '=) Follow up 6th -anuary ./,0 'Day ,,) & C 1lusih sin 'U)! shortness of breath 'U)\! fe#er '=) 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y;: xDi! @@Y.7 xDi! TY07!0L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 Thorax C symmetric fusiform! retraction '=)O tactile fremitus leftYright! normal impressionO percussion resonant in 4; both lungsO respiratory sound #esicular! no additional soundO ictus cordis un#isibleO single &. heart sound! murmur 'U)! systolic e3ection murmur grade ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = = = = 1ed rest! nee chest position @egular meals ,6// cal with .: gram protein 2 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation and phlebotomy on 9 th -anuary ./,0 Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U .// cc Y .9/ cc 1alanceY ?nput = 2utput Y ,// cc = .9/ cc Y =,9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,9/ cc Y 49/ cc '/7.//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y .6/ cc = 09/ cc Y =,./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,./ cc Y 4./ cc ',..//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U .6/ cc Y 0./ cc 1alanceY ?nput = 2utput Y .6/ cc = 0./ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 9/ cc Y .0/ cc ',:.//) ?nputY ?<FD U Diet Y = U 06/ cc Y 06/ cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y 06/ cc = 09/ cc Y =./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ./ cc Y =0./ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/,/! et '=)! bil '=)! glu '=) Follow up 7 th -anuary ./,0 'Day ,.) & C 1lusih sin 'U)! shortness of breath 'U)\! fe#er '=) 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y;: xDi! @@Y.7 xDi! TY07!:L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 6/ 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti 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 . . *Di nasal cannule 'if necessary) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) PlanC cardiac catheteri+ation and phlebotomy on 9 th -anuary ./,0 Preparation for cardiac catheteri+ation ,. ?nformed consent .. %ntibiotic prophylaxis cefotaxime 6/ mgDg '9// mg for this patient) ] = , hour before catheteri+ation 0. Fasting min. 4 hours before catheteri+ation 1lood laboratory analysis Test @esult Init @eferral Hemostatic test 1leeding time 4"0/"" minutes J6 PT U ?F@ Protrombin time 8ontrol ,0.0 seconds Patient .,.. seconds ?F@ ,.74 %PTT 8ontrol 0,.9 seconds Patient 06.6 seconds Trombin time 8ontrol ,:.0 seconds Patient ,7.: seconds Fluid balance '//.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 6, 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y =9/ cc Fluid reHuirement for the next 7 hours Y 0// cc U 9/ cc Y 09/ cc '/7.//) ?nputY ?<FD U Diet Y = U .// cc Y .// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y .// cc = ,9/ cc Y 0/ cc Fluid reHuirement for the next 7 hours Y 0// cc = 0/ cc Y .9/ cc ',..//) ?nputY ?<FD U Diet Y = U .6/ cc Y .6/ cc 2utputY ?G* U I2P Y 9/ cc U 0// cc Y 09/ cc 1alanceY ?nput = 2utput Y .6/ cc = 09/ cc Y =,./ cc Fluid reHuirement for the next 7 hours Y 0// cc U ,./ cc Y 4./ cc ',:.//) ?nputY ?<FD U Diet Y = U ,// cc Y ,// cc 2utputY ?G* U I2P Y 9/ cc U ,// cc Y ,9/ cc 1alanceY ?nput = 2utput Y ,// cc = ,9/ cc Y .9/ cc Fluid reHuirement for the next 7 hours Y ,// cc = .9/ cc Y =,9/ cc Irine dipstic '/7.//) leu '=)! nit '=)! uro /!.! pro W! pH 7! blo '=)! &G ,!/,/! et '=)! bil '=)! glu '=) Follow up 9 th -anuary ./,0 'Day ,0) & C 1lusih sin 'U)! shortness of breath 'U)\ 2 C sensYcompos mentis 1PY,//D9/ mmHg! H@Y:: xDi! @@Y.4 xDi! TY07!6L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra hyperemiaDhyperemia! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 Thorax C symmetric fusiform! retraction '=)O tactile fremitus leftYright! normal impressionO percussion resonant in both lungsO respiratory sound #esicular! no additional soundO ictus cordis un#isibleO single &. heart sound! murmur 'U)! systolic e3ection murmur grade ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse :: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = 1ed rest! nee chest position Fasting for catheteri+ation from /4.// 6. = = = = = = 2 . . *Di nasal cannule 'if necessary) Three way ?<FD Fa8l /!;> 6/ gttDi 'micro) ?n3. cefotaxime 9// mgD,. hours 'day ,) 'started /9.0/) Propranolol 4 x ,/ mg *actulac syr . x 8th ?? 'if necessary) Cardiac catheterization report (08.48-09.27) Angiography: Hand injection at innominate ein: no per!i!tent "e#t !$%c"aian ein &ight entric"e: "arge '()* aortic oerriding* !eere in#$ndi%$"ar p$"monary !teno!i!* +,A 7*- mm* &,A 8*- mm Aortogram: good coronary artery po!tion* no co""atera"* no patent d$ct$! arterio!$! 60 8onclusion C Tetralogy of fallot Faata index ,7:! $c Goon ratio ,!0, Fote C During procedure! ,6/ cc blood has been taen 'phlebotomy) %d#ice C (eep following until the patient fully awae Gatchout 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 2 C sensYcompos mentis 1PY;/D9/ mmHg! H@Y,.: xDi! @@Y0. xDi! TY0:L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra normal! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 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 ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ,.: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti 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 . ,=. *Di nasal cannule Three way ?<FD Fa8l /!;> ./ gttDi 'micro) ?n3. cefotaxime 9// mgD,. hours 'day .) Propranolol 4 x ,/ mg Paracetamol 0 x ,6/ mg *actulac syr . x 8th ?? 'if necessary) Follow up ; th -anuary ./,0 'Day ,6) & C Post cardiac catheteri+ation day ,! 1lusih sin 'U)! shortness of breath 'U)\! fe#er '=) 2 C sensYcompos mentis 1PY;/D9/ mmHg! H@Y;: xDi! @@Y.7 xDi! TY07!6L8 Head C 5yes C light reflex 'UDU)! isochoric pupil 0 mm! inferior con3ucti#a palpebra normal! icteric sclera '=D=) Fose C nasal flaring 'U)! eutrophy concha 5ars C within normal limits $outh C lips cyanosis 'U)! cyanotic tongue 'U)! oral candidiasis '=)! atrophy papilla '=) Fec C lymph node enlargement! 3ugular #enous pressure @U. cmH . 2 Thorax C symmetric fusiform! retraction '=)O tactile fremitus leftYright! normal impressionO percussion resonant in 66 both lungsO respiratory sound #esicular! no additional soundO ictus cordis un#isibleO single &. heart sound! murmur 'U)! systolic e3ection murmur grade ???D<? on upper left sternal border. %bdomen C soepel! epigastric pain '=)! normoperistalticO li#er! spleen! and renal are not palpable 5xtremities C pulse ;: bpm! regular! adeHuate pressureD#olume! warm axilla! capillary refill time J0! clubbing fingers 'U) digiti manus and pedis! cyanosis 'U). % C Tetralogy of fallot U failure to thri#e P C = = = = = = = = = 1ed rest! nee chest position @egular meals ,6// cal with .: gram protein 2 . ,=. *Di nasal cannuleaff Three wayaff ?<FD Fa8l /!;> ./ gttDi 'micro)aff ?n3. cefotaxime 9// mgD,. hoursaff Propranolol 4 x ,/ mg Paracetamol 0 x ,6/ mg *actulac syr . x 8th ?? 'if necessary) Patient was discharged from hospital on ; th -anuary ./,0 because he was referred to -aarta for surgical preparation and inter#ention. 67 CHAPTER 4 DISC.SSION $Q! 7 years old! presented to Pediatrics Department at Ha3i %dam $ali General Hospital 8enter on December .6 th ./,. 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 freHuent of sHuatting 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% '4.9D,/!/// births #s ..0D,/!/// 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 ,//D9/ mmHg! apex beat was localised in the 6 th left intercostal space within mid cla#icular line. 2n auscultation! there was loud single second heart sound of pulmonary component! and grade ???D<? systolic e3ection murmur which was loudest at the upper left sternal border. The chest was clinically clear. 8lubbing is caused by soft tissue growth under the nail bed as a conseHuence of central cyanosis and long hypoxemia. 9!;!40 2n heart auscultation! can be heard a single &. sound because only aortic component can be heard. % long! loud e3ection type systolic murmur is heard at the mid and upper left sternal borders because of pulmonal stenosis. 9!;!47 The presentator also assume that there 69 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 sHuatting. 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 ..!. gDd* 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 9 th -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 @<H. M=@ay examination can differ in some cases. ?n T2F! there is a boot shape appearance while in TG%! there is an egg=shaped cardiac silhoutte appearance. Howe#er! echocardiography should be done to confirm the specific diagnosis of a cyanotic 8HD. ;!0/!46 5chocardiography was done on .: th December ./,.! late in day 4 of hospitali+ation because of the processing in medical health assurance. 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 collateral image. This test confirm the diagnosis to uncomplicated tetralogy of fallot. 6: ?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. Faata 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; CHAPTER , S.MMARY $Q! 7 years old! presented to Pediatrics Department at Ha3i %dam $ali General Hospital 8enter on December .6 th ./,. 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 sHuatting. 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 ???D<? systolic e3ection murmur which was loudest at the upper left sternal border. 1lood laboratory analysis showed hemoconcentration! thrombocytopenia! and acidosis metabolic with partial compensation. 8hest radiography showed a boot shaped presentation. Fext! echocardiography was done to confirm diagnosis tetralogy of fallot. ?n this 7=year=old boy! surgical inter#ention correction will be preferred. %ngiography was done with Faata index ,7: and $cGoon ratio ,.0,. 2n ; th -anuary ./,0! patient was discharged from Ha3i %dam $ali Hospital 8enter because he was referred to capital city for surgical preparation and inter#ention. 7/ REFERENCES ,. 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