TETRALOGY OF FALLOT

Presentators Day, Date Supervisor

: Anditha Namira Rezky S dan Ghazali Akhmad P : Monday, April 22th 2013 : dr. Ridwan Daulay , Sp.A (K)

CHAPTER 1 INTRODUCTION 1.1. Background Congenital heart disease in infants and children is quite commonly found in Indonesia. Reports from various overseas studies show 6-10 of 100 infants born alive had congenital heart disease. Congenital heart disease is still unclear, however, it’s influenced by various factors. There is a tendency to develop some congenital heart disease in one family. Complete the formation of the fetal heart occurs at the end the first half could potentially interfere with the formation of the heart. Such factors include exposure to xrays, physical trauma and psychic, and drink herbal or birth control pills. Broadly speaking heart disease Congenital divided into 2 groups, namely non-cyanotic disease congenital heart disease and congenital cyanotic heart disease. Congenital heart disease is a group of non-cyanotic disease; include about 75% of the congenital heart disease and all the rest is a group of diseases cyanotic congenital heart about 25%. Disease are included in non-cyanotic congenital heart are persistent ductus arteriosus (PDA), atrial septal defect (ASD), ventricular septal defect (VSD). Cyanotic congenital heart disease such as Tetralogy of Fallot (TOF), transposition of the great arteries (TGA), double outlet right ventricle (DORV) .1 Tetralogy of Fallot (TOF) is a congenital cyanotic heart disease. The most commonly found where TOF was fourth disease congenital heart disease in children after ventricular septal defect, atrial septal defect, and persistent ductus arteriosus or approximately 10-15% of all disease. Congenital heart, congenital cyanotic heart disease among TOF is two thirds. In Dr.Soetomo hospital, most TOF patients obtained above age 5 years and the prevalence decreased after age 10 years.2

TOF consists of four abnormalities such as ventricle septal defect, pulmonary stenosis, overiding aorta and the right ventricle hypertrophy. Children with this disorder will be cyanotic at birth due to hypoxia. Growth and development of children will be disturbed compared with peers. This combination of lesions occurs in 3 every 10.000 live births and accounts for 7-10% of all congenital cardiac malformations. Patients nowadays usually present as neonates, which cyanosis of varying intensity based on the degree of obstruction to flow of blood to lungs. The etiology is multifactorial, but reported associations include untreated maternal diabetes, phenylketonuria and intake of retinoid acid. Associated chromosomal anomalies can include trisomies 21, 18 and 13, but recent experience points to the much more frequent associaton of microdeletions of chromosome 22. The risk of reccurence in families is 3%. Useful diagnostic tests are chest radiograph, electrocardiogram and

echocardiogram. The echocardiogram estabilishes the definitive diagnosis and usually provides sufficient information for planning of treatment, which is surgical. Approximately half of patients are now diagnosed antenatally.3

1.2. Objective The aim of this study is to explore more about the theoritical aspects on Tetralogy of Fallot, and to integrate the theory and application of Tetralogy of Fallot case in daily life.

CHAPTER 2 LITERATURE REVIEW

2.1. Definition In 1888, Fallot described a congenital heart defect composed of four characteristics: large ventricular septal defect (VSD), Pulmonary Stenosis, Overriding aorta and right ventricular hypertrophy. 4 The complex of anatomic malformations result from an anterior displacement of the conoceptum toward the right ventricle creating a malalignment VSD and narrowing of the outflow tract of the right ventricel (RV). The aorta is displaced anteriorly, straddling the muscular septum and arising from both ventricles (overriding aorta). 5

2.2. Epidemiology Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease in all age groups, Tetralogy of Fallot represents approximately 10% of cases of congenital heart deseases (CHD), occurs in 3-6 infants for every 10.000 births. This disorder accounts for one third of all CHD in patients younger that 15 years. 6 In most cases, Tetralogy of Fallot in sporadic and nonfamilial. The incidence in siblings of affected parents is 1-5% and it occurs more commonly in males than females. The disorders is associated with extracardiac anomalies such as cleft lip and palate, hypospadias and skeletal abnormalities. Genetics studies indicate that in some patients with tetralogy of fallot, there may be 22q11.2 deletion and other submicroscopic copy number alterations. 7

2.3. Etiology The causes of most congenital heart deseases are unknown, although genetic studies suggest a multifactorial etiology. A study from portugal reported that methylene tetrahydrofolate reductase (MTHFR) gene polymorphysm can be considered a suspectibility gene of Tetralogy of Fallot. 8 Prenatal factors associated with a higher incidence of tetralogy of fallot (TOF) include congenital rubella or other viral illnesses during pregnancy, poor prenatal

maternal alcohol concumption. not enough blood reaches the lungs. Children with down syndrome also have higher incidence of tetralogy of fallot. This wall is called a septum. abnormal facies. Ablation of cells of the neuralcrest has been shown to reproduce conotruncal malformation. oxygen-poor blood from the right ventricle flows through the pulmonary valve and into the pulmonary artery. and diabetes. Cytogenetic analysis may demonstrate deletions of a segment of chromosome band 22q11. the ventricels.nutrition. tetralogy of Fallot can be associated with a spectrum lessions known as CATCH 2(cardiac defects. From there. A VSD is a hole in the septum between the heart’s two lower chambers. Pulmonary Stenosis This defect involves narrowing of the pulmonary valve and the passage from the right ventricle to the pulmonary artery. the blood travels to the lungs to pick up oxygen. hypocalcemia). thymic hipoplasia. The hole allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle.4. Thus . Normally. As one of the conotruncal malformations. As a result. the heart has to work harder to pump blood through the valve. . Pathology of Tetralogy of Fallot Ventricular Septal Defect The heart has an inner wall that separates the two chambers on its left side from the two chambers on its right side. The septum prevents blood from mixing between the two sides of the heart. maternal age older than 40 years. In pulmonary stenosis. 9 2. the pulmonary valve cannot fully open. maternal phenylketonuria birth defects. cleft palate. as do infants with fetal hydantoin syndrome or fetal carbamazepine syndrome.

blood will naturally shunt from the left ventricle . If the resistenceto blood flow across the obstructed right ventricular outflow tract is less than the resistence to flow out of the aorta into the systemic circulation. the pressure in the right ventricle reflects that of the left ventricle. the aorta is located between the left and right ventricles. In a healthy heart. not by the size of the VSD. As a result. the muscle of the right ventricel is thicker than usual. Overriding Aorta This defect occurs in the aorta. the aorta is attached to the left ventricel.5. In tetralogy of fallot. This allows only oxygen-rich blood to flow the body. Since the VSD is typically large and unrestrictive. the main artery that carries oxygen-rich blood from the heart to the body. This occurs because the heart has to work harder than normal to move blood through the narrowed pulmonary valve.Right Ventricular Hypertrophy With this defect. 10 2. As a result. oxygen-poor blood from the right ventricle flows directly into the aorta instead of into the pulmonary valve. Pathophisiology The physiologic consequences of tetralogy of fallot are lergely dependent upon the degree of right ventricular outflow obstruction. directly over the VSD. the direction of blood flow across the VSD will be determined by the path of least resistence for blood flow.

there is predominately a left-to-right shunt and the patient will be a cyanotic. In this situation. The exact etiology of these episodes in unclear. hyperventilation and stimulation of right ventricular mechanoreceptors. Clinical Presentation History The clinical features of tetralogy of Fallot (TOF) are directly related to the severity of the anatomic defects. Occasionally. it will be easier for blood to across the VSD from the right ventricle into the left ventricle and go out the aorta. until they outgrow their pulmonary blood supply. These individuals remain asymptomatic. which now becomes the path of least resistance. Most infants with tetralogy of fallot have difficulty with feeding. At birth. These episodes are often referred as “tet spells” of “hypercyanotic spells”. An individual with minimal cyanosis can develop a dynamic increase in right ventricular outflow tract obstruction with a subsequent increase in right-to-left shunt an the development of cyanosis. including increased infundibular contractility. 11 2. some infants with tetralogy of Fallot do not show signs of cyanosis. Infants with pulmonarry atresia may become profoundly cyanotic as the ductus arterosus closes unless bronchopulmonary colaterals are present.6. although there have been a number of proposed mechanisms. the resitence to blood flow into the pulmonary bed also increases. peripheral vasodilatation. but they may later develop episodes of bluish pale skin during crying or feeding (tet spells). As the degree of right ventricular outflow obstruction increases. and failure to thrive (FTT) is commonly observed. If the right ventricular obstruction is significant enough to increase resistance. there can be near occlusion of the right ventricular outflow tract with profound cyanosis. some children have just enough pulmonary blood flow and do not appear cyanotic. This right-to-left shunt across the VSD will result in a large volume of desaturated blood entering the systemic circulation and cyanosis and polycythemia will ensue. One of the fascinating physiologic characteristics of tetralogy of Fallot is that the right ventricular outflow obstruction can fluctuate.to the right ventricle and into the pulmonary bed. In the most dramatic situation. .

the fingers and toes show clubbing. Although such patients may not appear cyanotic. When the pulmonary stenosis is mild. In some patients. Squatting increases peripheral vascular resistance (PVR) and thus decreases the magnitude of the right-to-left shunt across the ventricular septal defect (VSD). the murmur may be inaudible (more cyanotic patients have greater obstruction and soften . The rare patient may remaun marginally and imperceptibly cyanotic. or acyanotic and asymptomatic. Physical Examination Most infants with tetralogy of fallot (TOF) are smaller than expected for age.Hypoxic tet spells are potentially lethal. into adult life. bidirectional shunting may occur. and highly typical of infants with tetralogy of fallot. Symptoms generally progress secondary to hypertrophy of the infundibular septum. Exertional dyspnea usually worsen with age. after age 3-6 months. A characteristics fashion ini which older children with tetralogy of faqllot increase pulmonary blood flow is to squat. they often have oxygen desaturation in the systemic circulation. The mechanism is thought to include spasm of the infundibular septum. When the right ventricular (RV) outflow tract obstruction (RVOTO) is moderate. the infundibular stenosis is minimal. Worsening of the RVOTO leads to RV hypertrophy. The predominant shunt is from right to left with flow accross the VSD into the left ventricle (LV). Occasionally. increased right-to-left shunting and systemic hypoxemia. Cyanosis of the lips and nail bed is usually pronounced at birth. of diagnostic significance. hemoptysisi due to rupture of the bronchial collaterals may result in the older child. Squatting is a compensatory mechanism . These spells can be aborted with relatively simple procedurs. producing what is called pink tetralogy. and the predominant shunt is from left to right. A harsh systolic ejection murmur (SEM) is hard ovel the pulmonic area and left sternal border. unpredictable episodes that occur even in noncyanotic patients with tetralogy of fallot. A systolic thrill is usually present anteriorly along the left sternal border. which acutely worsens the right ventricular (RV) outflow tract obstruction. which produces cyanosis and an elevated hematocrit value.

Scoliosis (common) 6. the heart murmur heart in TOF is not due to the VSD. Diagnosis Patients with TOF have a number of distinguishing signs and symptoms that can be found on physical exam and elucidated with a detailed history. loud. In individuals with aortapulmonary collaterals. The S2 is usually single (the pulmonaic valve closure is not heard).7. 2. (Remember. an acyanotic patient with tetralogy of fallot (pink tet) has a long. this is most commonly seen on the lips or nail beds. Cyanosis: If patients are cyanotic. an isolated VSD murmur is a holosystolic murmur. 12 The following may also be noted: 1. It may radiate to the right lower sternal border. It is appreciated best along the left mid to upper sternal border with radiation posteriorly. continuous murmurs may be auscultated. During cyanotic episodes. It is infact due to the right ventricular outflow obstruction.) Patients will have a normal S1 and possibly a single S2 due to diminished P2 component. systolic murmur with a thrill along the RVOT. May have a bulging left hemithorax 3. Hemoptysis. Thus. best heard in the tricuspid area. The murmur is typically crescendo decrescendo with a harsh systolic ejection quality.murmur). Retinal engorgement 7. . Aortic ejection click 4. Squatting position (compensatory mechanism) 5. RV predominance on palpation 2. murmur may disappearm which is suggestive of lessened RV outflow to the pulmonary arteries. Cardiac exam: Most importantly.

Additionally. patients with TOF have right ventricular hypertrophy. Echocardiogram: Findings on echocardiogram are the mainstay of diagnosis in TOF. The classic description is of a patient who becomes cyanotic and then assumes a squatting position to relieve the cyanosis and hypoxia. crying). thereby increasing the pressure in the left heart. and subsequently forcing blood back into the pulmonary circulation. patients with TOF will show increased right ventricular forces as evidenced by tall R waves in V1. or they may occur without warning. a “boot shaped” heart and decreased pulmonary vascular markings.g. Right ventricular hypertrophy is demonstrated by a rightward deviated axis.Tet spells: Tet spells are hypercyanotic episodes precipitated by a sudden increase in right ‐to left shunting of blood. right atrial enlargement is manifested by prominent P waves in V1. Squatting serves to increase peripheral vascular resistance. Chest X‐Ray: As seen on the chest x‐ray below. feeding. Electrocardiogram: On EKG. They can be elicited by activity (e. Echocardiogram will demonstrate a ventricular septal defect with an overriding of the .

hypoplastic pulmonary arteries. pulmonic stenosis and right ventricular hypertrophy. intravenous fluids. In the interim period. As seen in the echocardiogram below.8. In about 25% of cases. 6 2. morphine. almost all patients undergo corrective surgical repair within the first year of life. surgical correction is indicated. This is used as a palliative procedure in infants who are not acceptable candidates for intracardiac repair due to prematurity. or coronary artery anatomy. some patients may require digoxin or diuretics if signs of heart failure are present. betablockers or pharmacologically increasing systemic vascular resistance by administration of drugs. knee/chest position. the blood (blue) from both the right ventricle and left ventricle enters the overriding aorta across the VSD. These include oxygen. Additionally. There are two common surgical procedures: Blalock‐Taussig shunt creates a shunt between the aorta and the pulmonary artery using the subclavian artery. Treatment of hypercyanotic spells is directed towards improving pulmonary blood flow. . patients will also have a right aortic arch. prostaglandin treatment may be necessary to maintain the patency of the ductus arteriosus. such as phenylephrine. Patients will require additional surgery as this is not a curative surgery. sodium bicarbonate. Once an infant has developed progressive cyanosis or has evidence of hypercyanotic spells. Treatment Once TOF is diagnosed.aorta. This constellation of findings serves to clinch the diagnosis of TOF.

the pulmonary valve may need to be removed to eliminate the obstruction. and enlargement of the RVOT with relief of all sources of obstruction. and atrial arrhythmias. patients undergoing surgical repair for TOF have an excellent prognosis with a 20‐year survival rate of over 90%.Intracardiac repair Is the definitive repair for patients with TOF and is the preferable procedure. patients may experience right ventricular hypertrophy or enlargement due to residual pulmonary stenosis and backward blood flow into the right ventricle. Complications of surgical repair of TOF include arrhythmias particularly ventricular tachycardia (VT). Outcome and Complications: Overall. This consists of patch closure of the ventricular septal defect. Furthermore. . In some cases.

Prognosis Early surgery is not indicated for all infants with tetralogy of Fallot (TOF). Differential Diagnosis 1. Atresia Pulmonal 2. Complication 1. The progression of the disorder depends on the severity of right ventricular (RV) outflow tract obstruction (RVOTO). although. Late outcome data suggest that most survivors are in New York Heart Association (NYHA). Sudden death from ventricular arrhythmias has been reported in 1-5% of patients at a later stage in life. One study found left ventricular longitudinal dysfunction to be associated with a greater risk of developing life-threatening arrhythmias. although maximal exercise capability is reduced in some. and the cause remains unknown. Patients should be followed closely by a pediatric cardiologist to monitor for these short‐term and long‐term complications.8. 2. the natural progression of the disorder indicates a poor prognosis. Chronic Organ Hypoxia. neurodevelopmental delay and myocardial fibrosis. without surgery.9.Cyanotic spell 4.10. Policytemia 3. 6 2. In the present era of cardiac surgery. Transposition of Great Artery 2.Long‐term complications include the need for additional surgeries. Double Outlet Right Ventricle 3. For some time. Abcess Cerebry 2. it . children with simple forms of tetralogy of Fallot enjoy good long-term survival with an excellent quality of life. Continued cardiac monitoring into adult life is necessary. It has been suspected that ventricular dysfunction may be the cause.

a 40% reduction in deaths associated with tetralogy of Fallot was noted from 1979 to 2005. What is often forgotten is that residual shunts or a patent foramen ovale are also known causes of strokes. prolonged hypotension/anoxix and polycythemia. although individuals whose shunts produce minimal hemodynamic compromise have been noted. The mortality rate is highest in the first year and then remains constant until the second decade. and subacute bacterial endocarditis. It is well known that children with congenital heart disease are prone to stroke. Due to advanced surgical techniques. Without surgery. ranging from 30% at age 2 years to 50% by age 6 years. As might be expected. In most of these children the causes of stroke have been related to thromboemboli. pulmonary embolus. albeit rarely. and only 50% survive to age 1 year and 8% to age 10 years. and these individuals achieve a normal life span. and fewer than 5-10% of patients are alive by the end of their second decade. The investigation of strokes in these children usually begins with a CT scan of the brain followed by an ECHO. Most individuals who survive to age 30 years develop congestive heart failure (CHF). A 2012 study by Chiu confirmed this suspicion13 If left untreated.has been suspected that certain children may have inherited a predispostion to developing long QT syndrome. 13 . cases of survival of patients into their 80s have been reported. individuals with tetralogy of Fallot and pulmonary atresia have the worst prognoses. patients with tetralogy of Fallot face additional risks that include paradoxical emboli leading to stroke. However. mortality rates gradually increase. No more than 20% of patients can be expected to reach the age of 10 years.

CHAPTER III CASE REPORT Name Age Sex Date of Admission :MF : 4 years 4 months : Male : March. Cyanotic (+). History of immunisation : Never. Current seizures (-). : 84% : 61% : 72% . and worsen while walking and crying. Cough (+) 3 days ago. cyanotic lips. Currently fever (-) a history of seizures (+) at the age of 3 months to 1 year of age. both fingers and toes (+) . History of illness : Patient was a reffered patient from RS Tanjung Balai and was diagnosed susp. Physical Examination Generalized status Body weight Body length BW/BL BW/age BL/age : 11 kg. patient experienced since the age of 2 years. History of recurrent fever (+) from patients aged 2 years. non-productive cough. : 90 cm. History of growth development : Patient has not able to say words. 26th 2013 Main Complaint : Dyspnea History : Patient has been experiencing dyspnea since two years ago. Red skin spots (+) since 3 days. TOF.

reguler. Liver/Spleen/Renal: Differential Diagnosis Cyanotic Congenital Heart Disease ec .TOF . Compos Mentis. BP: 100/60  Urogenital : Male.Present status Sens. Ear/Mouth/Nose: within normal limit. HR: 136 x/i. Extremities : Upper extremities: Pulse 136x/i.3oC. murmur (+) systolic grd III-IV LMCS. Localized status      Head : Light reflex (+/+). Peristaltic (+) normal. Dyspnea (+). Icteric (-). Pulse: 136x/ minute. adequate pressure and volume. regular. Abdomen undeterminate. Respiratory Rate: 48 x/ minute. Isochoric pupil.TOF . Chest retraction (+) epigastrial. CRT < 3”. Anemic (-). Body temperature: 36. RR: 48x/i. : Symmetrical fusiformis. warm acral. paleness inferior palpebra conjunctiva (-/-). Crackles (-/-). : Soepel. Normal in appearance.TGA Management: O2 nasal canule 1 L/i . reguler. Neck Thorax : Lymph node enlargement (-). Edema (-).TGA Working Diagnosis Cyanotic Congenital Heart Disease ec . Lower extremities: oedem (-/-). Cyanosis (+).

5x103/mm3 37-41% 217-497x103/mm3 82-95 fL 25-29 pg 29-31 g % 11. Lower extremities: oedem (-/-). regular.30% 6.80% 52x103/mm3 74. Thorax Symmetrical fusiformis. reguler. HR: 128x/i. Cyanosis (+). warm acral. Body weight: 11 kg. Anemic (-).50% Normal Value 11. Crackles (-/-). Icteric (-).8 % .1 g % 4.48x106/mm3 4.10 pg 33. Liver/Spleen/Renal: undeterminate. Laboratory Findings : Hematology Lab Results : (26-03-2013) Parameters Hemoglobin (HGB) Eritrosit (RBC) Leukosit (WBC) Hematokrit Trombosit MCV MCH MCHC RDW Diftel Neutrofil Limfosit Monosit 28.10% 37-80% 20-40% 2 .60 g % 16. Head Light reflex (+/+).6-14.94x106/mm3 7.16 FOLLOW UP March 26th 2013 S: Dyspnea O: Sens: CM. Body length: 90 cm. Isochoric pupil. Peristaltic (+) normal. Abdomen Soepel.40 g % 6. Edema (-). adequate pressure and volume. Temp: 36.60 fL 25.04x103/mm3 51.5-13. normal in appearance.7oC. Dyspnea (-).40-4. Genital Male. reguler.90% 56.3-14. paleness inferior palpebra conjunctiva (-/-).8% Value 17. RR: 36x/i. Chest retraction (+) epigastrial. Extremities Upper extremities: Pulse 128x/i. CRT < 3”. Ear/Mouth/Nose: within normal limit. murmur (+) systolic grd III-IV LMCS.

Anisositosis.1x103/μL 0.4 mmol/L -13.04x103/μL 3.600% 2.35-7.45 38-42 mmHg 85-100 mmHg 22-26 mmol/L 19-25 mmol/L (-2) – (+2) g/dL 95-100% 132. Atypical lymfosit .30x103/μL 0-0.4-7.40 mg/dL 0.7 mmol/L 11.Trombosit: Jumlah ↓ .00 mg/dl < 200 mg/dl 13.3x103/μL 1.6x103/μL 0.3 mEq/L 107 mEq/L 135-155 mEq/L 3.4 mmol/L 98.10-0.04x103/μL 1-6% 0 .1 mmHg 122 mmHg 10.5 mEq/L 96-106 mEq/L Morfology: .Leukosit: Normal.47 mg/dL 130 mEq/L 5.1% 2.17 Eosinofil Basofil Neutrofil Absolut Limfosit Absolut Monosit Absolut Eosinofil Absolut Basofil Absolut Blood Gas Analysis Test PH pCO2 pO2 Bicarbonate (HCO3) Total CO2 BE Saturation O2 Glucose Metabolism Blood Glucose ( sewaktu) Renal Ureum Creatinin Electrolit Natrium (Na) Kalium (K) Klorida (Cl) 8.Eritrosit: Normal.6-5.2-0.29 mg/dL < 50 mg/dL 0.10% 0.1x103/μL 7.31-0.323 21.1% 7.96x103/μL 0.43x103/μL 0. Target cell (+) .57x103/μL 0.7-5.

Conclusion: Normal Lungs Appearence. Cardiomegaly (CHD). Cardia (CTR: ± 64%) enlargement boot shape like.TOF .18 Radiology Results: Both sinus and diafragm are normal.TGA P: .O2 nasal canule 1 L/i Diet MB 1050 kcal with 22 gr protein Consultation : Cardiology . suspect TOF. There’s no infiltrat in both lungs.Cyanotic Congenital Heart Disease ec . A: .

warm acral. Cyanosis (-). Extremities Upper extremities: Pulse 110x/i. adequate pressure and volume. CRT < 3”. RR: 30x/i. Liver/Spleen/Renal: undeterminate. Lower extremities: oedem (-/-).Cyanotic Congenital Heart Disease ec . reguler. Isochoric pupil.TGA P : Management March 27th 2013 . murmur (+) systolic grd III-IV LMCS. Edema (-). Ear/Mouth/Nose: within normal limit. regular. Crackles (-/-).O2 nasal canule ½ L/i Diet MB 1050 kcal with 22 gr protein . paleness inferior palpebra conjunctiva (-/-). Temp: 37oC.TOF . Thorax Symmetrical fusiformis. Chest retraction (+) epigastrial. Body weight: 11 kg Head Light reflex (+/+). Abdomen Soepel. Dyspnea (-). normal in appearance.O2 nasal canule ½ L/i Diet MB 1050 kcal with 22 gr protein R/ Echocardiografi Management March 28th 2013 .19 March 27 2013 – March 28 2013 S: Dyspnea (-). Genital Male. reguler. Anemic (-). Peristaltic (+) normal. HR: 110x/i. th th A: . Icteric (-). Cyanotic (-) O: Sens: CM.

Thorax Symmetrical fusiformis. normal in appearance. Crackles (-/-). paleness inferior palpebra conjunctiva (-/-). Cyanotic (-) O: Sens: CM. reguler. HR: 98x/i.O2 nasal canule ½ L/i Diet MB 1050 kcal with 22 gr protein . Temp: 36. Chest retraction (+) epigastrial.20 March 29th 2013 S: Dyspnea (-).6oC. Icteric (-). Peristaltic (+) normal. warm acral. regular. reguler.TOF . Cyanosis (-). Extremities Upper extremities: Pulse 98x/i. Anemic (-). Body weight: 11 kg Head Light reflex (+/+).TGA P: . RR: 26x/i. A: . Abdomen Soepel. murmur (+) systolic grd III-IV LMCS. Ear/Mouth/Nose: within normal limit.Cyanotic Congenital Heart Disease ec . CRT < 3”. Isochoric pupil. Edema (-). Lower extremities: oedem (-/-). Liver/Spleen/Renal: undeterminate. Dyspnea (-). adequate pressure and volume. Genital Male.

Crackles (-/-). Abdomen Soepel.8 mm Overring Aorta >50% PS boat PG 90. Temp: 36. Lower extremities: oedem (-/-).2 mm A: . Icteric (-). Body weight: 11 kg Head Light reflex (+/+). LPA 7. reguler. Edema (-).21 March 30th 2013 – April 1st 2013 S: Dyspnea (-). warm acral. Thorax Symmetrical fusiformis. HR: 100x/i. Cyanosis (-). Peristaltic (+) normal. Anemic (-). adequate pressure and volume.O2 nasal canule ½ L/i Diet MB 1050 kcal with 22 gr protein . Dyspnea (-). Genital Male. Ear/Mouth/Nose: within normal limit.9oC. reguler.TOF P: . paleness inferior palpebra conjunctiva (-/-). Extremities Upper extremities: Pulse 100x/i. normal in appearance. Chest retraction (+) epigastrial. Cyanotic (-) O: Sens: CM. regular. Laboratory Findings : Echocardiografy Results (28/03/2013): Right Ventricular Hypertrophy VSD: 10. murmur (+) systolic grd III-IV LMCS. RR: 26x/i. Isochoric pupil. CRT < 3”.96 mmHg RPA 7 mm. Liver/Spleen/Renal: undeterminate.

80 pg 33. Dyspnea (-).1 g % 4. Genital Male. Crackles (-/-).60% 8. normal in appearance. regular. reguler. CRT < 3”.00 g % 16.10% 4 mm/jam Normal Value 11. Peristaltic (+) normal. Anemic (-). Lower extremities: oedem (-/-).10 fL 24. Isochoric pupil.8% Value 17.5-13.48x106/mm3 4. paleness inferior palpebra conjunctiva (-/-). Thorax Symmetrical fusiformis.8 % <15 mm/jam .5x103/mm3 37-41% 217-497x103/mm3 81-95 fL 25-29 pg 29-31 g % 11.00 g % 6.6-14.50% 114x103/mm3 75. warm acral.40-4.11x103/mm3 51. Cyanotic (-) O: Sens: CM. Icteric (-). murmur (+) systolic grd III-IV LMCS. Chest retraction (+) epigastrial. HR: 104x/i. Cyanosis (-). Laboratory Findings : Hematology Lab Results : (03-04-2013) Parameters Hemoglobin (HGB) Eritrosit (RBC) Leukosit (WBC) Hematokrit Trombosit MCV MCH MCHC RDW LED Diftel Neutrofil Limfosit Monosit 45. Temp: 37oC.86x106/mm3 15. Edema (-). Abdomen Soepel. adequate pressure and volume. RR: 28x/i. Extremities Upper extremities: Pulse 104x/i.10% 37-80% 20-40% 2 .22 April 2nd 2013 – April 4th 2013 S: Dyspnea (-). reguler. Liver/Spleen/Renal: undeterminate. Body weight: 11 kg Head Light reflex (+/+).10% 38.3-14. Ear/Mouth/Nose: within normal limit.

30x103/μL 0-0.90% 0.9 mmHg 114 mmHg 15.6 mmol/L -9.3x103/μL 1.323 30.80x103/μL 5.6x103/μL 0.1x103/μL 0.19x103/μL 0.23 Eosinofil Basofil Neutrofil Absolut Limfosit Absolut Monosit Absolut Eosinofil Absolut Basofil Absolut 7.4-7.10-0.1% 2.80 mg/dl < 200 mg/dl 7.35-7.0 mg/dL < 50 mg/dL 0.1x103/μL Morfology: .8% 7.31-0.Trombosit: Clumping (+) Kesan: Leukositosis + Trombositopenia Blood Gas Analysis Test PH pCO2 pO2 Bicarbonate (HCO3) Total CO2 BE Saturation O2 Glucose Metabolism Blood Glucose ( sewaktu) Renal Ureum Creatinin Uric acid Hepar Total Bilirubin 0.31 mg/dL < 1 mg/dL 13.7-5.0 mmol/L 97.23x103/μL 1.Eritrosit: Hipokrom Mikrositik .29 mg/dL 7.84x103/μL 1.Leukosit: Jumlah meningkat bentuk normal .0 mg/dL 111.40 mg/dL 0.300% 6.47 mg/dL < 7.7 mmol/L 16.05x103/μL 1-6% 0 .45 38-42 mmHg 85-100 mmHg 22-26 mmol/L 19-25 mmol/L (-2) – (+2) g/dL 95-100% .2-0.

O2 nasal canule ½ L/i Diet MB 1050 kcal with 22 gr protein P : Management April 4th 2013 . dengan diagnosa karies R/ Kateterisasi .O2 nasal canule ½ L/i Diet MB 1050 kcal with 22 gr protein Consultation: Gimul.24 Direct Bilirubin Fosfatase alkali (ALP) AST/SGOT ALT/SGPT 0.TOF P : Management April 2nd 2013 .2 mg/dL < 269 U/L < 38 U/L < 41 U/L A: .April 3rd 2013 .16 mg/dL 253 U/L 32 U/L 11 U/L 0-0.

Genital Male. CRT < 3”.April 7th 2013 S: Dyspnea (-). RR: 29x/i. Lower extremities: oedem (-/-).O2 nasal canule ½ L/i Diet M II 750 kcal + 24 gr protein + Pediasure 3x150 cc R/ EKG. HR: 120x/i. reguler. Chest retraction (+) epigastrial. Body weight: 11 kg. adequate pressure and volume.O2 nasal canule ½ L/i Diet M II 750 kcal + 24 gr protein + Pediasure 3x150 cc . Anemic (-). Cyanosis (-).April 7th 2013 . Edema (-). reguler. Peristaltic (+) normal. Liver/Spleen/Renal: undeterminate. Thorax Symmetrical fusiformis. Laboratory Findings : EKG Results (05/04/2013): Right Axis Defiation Bone Age Results (06/04/2013): Umur tulang sesuai dengan 2 tahun A: . Cyanotic (-) O: Sens: CM. Crackles (-/-). Abdomen Soepel. Temp: 37oC. Dyspnea (-). Ear/Mouth/Nose: within normal limit.TOF + Moderate Malnutrisi P : Management April 5th 2013 . BW/BL: -2 sd until -1 sd. paleness inferior palpebra conjunctiva (-/-). Bone Age Management April 6th 2013 . Icteric (-). warm acral. BW/age: -3 sd until -2 sd. Isochoric pupil. normal in appearance. Extremities Upper extremities: Pulse 120x/i. regular. murmur (+) systolic grd III/6 LMCS ICR III-IV. BL/age: < -3 sd Head Light reflex (+/+).25 April 5th 2013 .

70 pg 33. regular. normal in appearance. Body weight: 11 kg.70% 3 mm/jam Normal Value 11.8 % <15 mm/jam . BW/BL: -2 sd until -1 sd.5-13. Anemic (-).26 April 8th 2013 S: Dyspnea (+)↓.6-14. Dyspnea (-). Peristaltic (+) normal. murmur (+) systolic grd III/6 LMCS ICR III-IV. reguler.95x103/mm3 52. adequate pressure and volume.60% 23. Laboratory Findings : Hematology Lab Results : (08-04-2013) Parameters Hemoglobin (HGB) Eritrosit (RBC) Leukosit (WBC) Hematokrit Trombosit MCV MCH MCHC RDW LED Diftel Neutrofil Limfosit Monosit 67.48x106/mm3 4. BW/age: -3 sd until -2 sd. Edema (-).30 g % 17.1 g % 4. Crackles (-/-). reguler.5x103/mm3 37-41% 217-497x103/mm3 81-95 fL 25-29 pg 29-31 g % 11.20 fL 24. Temp: 37oC. Ear/Mouth/Nose: within normal limit.80% 37-80% 20-40% 2 . paleness inferior palpebra conjunctiva (-/-). CRT < 3”. Cyanosis (-).08x106/mm3 14. Isochoric pupil.3-14.60% 6. Liver/Spleen/Renal: undeterminate. BL/age: < -3 sd Head Light reflex (+/+).50% 128x103/mm3 75. HR: 88x/i. Extremities Upper extremities: Pulse 88x/i. Abdomen Soepel. Cyanotic (-) O: Sens: CM.40-4.8% Value 17. warm acral. Icteric (-). Genital Male. RR: 30x/i. Chest retraction (+) epigastrial.50 g % 7. Thorax Symmetrical fusiformis. Lower extremities: oedem (-/-).

53x103/μL 1.30x103/μL 0-0.7-5.70% 0.9/37.1% 2.4-7.8 A: .10-0.26x103/μL 0.27 Eosinofil Basofil Neutrofil Absolut Limfosit Absolut Monosit Absolut Eosinofil Absolut Basofil Absolut Morfology: .Eritrosit: Anisositosis .09x103/μL 3.Trombosit: normal Kesan: Leukositosis + Trombositopenia Hemostasis (08/04/2013): 1.05x103/μL 1-6% 0 .1x103/μL Bleeding Time/PT/APTT/TT: 3/14.Leukosit: normal .6x103/μL 0.3x103/μL 1.2-0.300% 10.O2 nasal canule ½ L/i Diet M II 750 kcal + 24 gr protein + Pediasure 3x150 cc R/ menunggu perbaikan KU untuk kateterisasi .4/15.02x103/μL 0.1x103/μL 0.TOF + Moderate Malnutrisi P : Management April 8th 2013 .

TOF + Moderate Malnutrisi P : Management April 9th 2013 .7 mm Nakata Index: 368 A: . Cyanosis (-). Extremities Upper extremities: Pulse 90x/i. Cyanotic (-) O: Sens: CM. Icteric (-). Catheterization Results: . HR: 90x/i.O2 nasal canule ½-1 L/i Diet M II 750 kcal + 24 gr protein + Pediasure 3x150 cc . Lower extremities: oedem (-/-). murmur (+) systolic grd III/6 LMCS ICR III-IV. BL/age: < -3 sd Head Light reflex (+/+). Liver/Spleen/Renal: undeterminate. Genital Male. Crackles (-/-). Dyspnea (-). Chest retraction (+) epigastrial. reguler. Temp: 36. BW/BL: -2 sd until -1 sd. reguler.Tetralogy of Fallot Severe in fundibular PS LPA 9. warm acral. Isochoric pupil. Edema (-). normal in appearance. paleness inferior palpebra conjunctiva (-/-). Thorax Symmetrical fusiformis. Body weight: 11 kg. BW/age: -3 sd until -2 sd. regular.28 April 9th 2013 S: Dyspnea (+)↓. adequate pressure and volume.8oC.2 mm. Peristaltic (+) normal. CRT < 3”. RR: 32x/i. Anemic (-). RPA 12. Ear/Mouth/Nose: within normal limit. Abdomen Soepel.

Cyanotic (-) O: Sens: CM.O2 nasal canule ½-1 L/i Diet M II 750 kcal + 24 gr protein + Pediasure 3x150 cc R/ PBJ . paleness inferior palpebra conjunctiva (-/-). Thorax Symmetrical fusiformis. reguler. Abdomen Soepel. Peristaltic (+) normal. Genital Male. BL/age: < -3 sd Head Light reflex (+/+). CRT < 3”. HR: 90x/i. RR: 22x/i. Body weight: 11 kg. Dyspnea (-). normal in appearance. Icteric (-). Temp: 37oC. A: . warm acral. Cyanosis (-). Crackles (-/-).TOF + Moderate Malnutrisi P : Management April 9th 2013 . Liver/Spleen/Renal: undeterminate. Ear/Mouth/Nose: within normal limit. Anemic (-). BW/BL: -2 sd until -1 sd.29 April 9th 2013 S: Dyspnea (+)↓. murmur (+) systolic grd III/6 LMCS ICR III-IV. reguler. regular. Isochoric pupil. Edema (-). Extremities Upper extremities: Pulse 90x/i. Lower extremities: oedem (-/-). Chest retraction (+) epigastrial. adequate pressure and volume. BW/age: -3 sd until -2 sd.

we can hear the heart murmur that’s infacted by the right ventricular outflow obstruction. at birth some infants with tetralogy of Fallot do not show signs of cyanotic. Cyanotic (+). was admitted to the Pediatric Non Infection Unit of Haji Adam Malik General Hospital on March 26th 2013 . .30 CHAPTER 4 DISCUSSION AND SUMMARY 4. At patients with TOF. exertional dyspnea usually worsen with age and activity. Rash and increased red blood cell are signs of polycitemia. Currently fever (-) a history of seizures (+) at the age of 3 months to 1 year of age. And worsen while playing and walking. It support the theory. older children with TOF. Current seizures (-).Discussion MF. This is most commonly seen on the lips or nail bed.1. ECG and Echocardiography as the gold standars in diagnosing TOF. as the clinical manifestasion of TOF patients. a 4 years 4 months old boys. Cyanotic experienced since patient was 2 years old. cyanotic lips. patient experienced since the age of 2 years. Policytemia is one of tetralogy of Fallot complication. Physical Diagnostic. nonproductive cough. In this case dyspnea was present since 2 years old. cyanotic develop on lips and on the nail bed of both fingers and toes. Patients will have a normal S1 and possibly a single S2. This patient already got all of these procedurs. Red skin spots (+) since 3 days. History of recurrent fever (+) from patients aged 2 years. Patient’s auscultation examination experienced murmur. progressive by increasing age and worsen while walking and playing. There are some involved in diagnozing TOF.with chief complain of dyspnea. Gold standard to diagnose TOF is echocardiography. and from the chest x-ray we found a boot-shaped appearence that pictured a ventricular hypertrophy. increase pulmonary blood flow is to squat. showed right axis deviation and right ventricle hypertrophy. with body weight of 11 kg. among them is anamnesis. clubbing finger both fingers and toes (+) . Echocardiography at this patient proved he is experiencing TOF. Cough (+) 3 days ago. and body length of 90 cm. Electrocardiography of this patient. it pictured ventricular septal defect. pulmonal stenosis and right ventricular hypertrophy. overriding aorta. The dyspnea started since 2 years ago. Chest X-ray. but they may later develop episodes of bluish pale skin during crying or feeding (tet spells).

31 4. . physical examination. Who was admitted in peadiatrics non infection ward in RSUP Adam Malik on 26th March 2013 with the diagnose of Tetralogy of Fallot.2 Summary A case was reported about a 4 years 4 months old girls with 11 kg of body weight and 90 cm of body length. The patient was diagnosed based on history taking. Chest X-ray and Echocardigraphy.

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