Single Ventricle Mhi Belum Sempurna.docx

Case Report - EBP

To:

Non Infection Unit TRANSPOSITION OF GREAT ARTERIES WITH SINGLE VENTRICLE AND SEVERE MALNUTRITION CAUSES BRAIN ABSCESS IN 5 YEARS 7 MONTHS OLD GIRLS Presentator

: Muhammad Hidayat

Day / Date

: / Maret 2019

Supervisor in charge

: dr. Muhammad Ali, Sp.A(K)

Supervisor

: dr. Tina Christina L.Tobing, Sp.A(K) dr. Muhammad Ali, Sp.A(K) dr. Rizky Adriansyah ,M.Ked (Ped), Sp.A(K) dr. Hafaz Zakky Abdillah, M.Ked (Ped), Sp.A (K) dr. Putri Amelia, M.Ked (Ped), Sp.A

Introduction Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia. Transposition of Great Arteries (D-TGA) is one of the most common cyanotic congenital heart defect in newborn, having atrioventricular concordance with ventriculoarterial discordance where aorta arises from right ventricle and pulmonary artery from left ventricle. Patients with cyanotic congenital heart disease (cCHD) are prone to develop frequent brain abscesses. Mortality rate remains very high in these patients.1 Multiple brain abscesses are a rare and potentially life-threatening condition requiring early diagnosis and early treatment. Congenital heart disease (CHD) is an important predisposing factor for hematogenous brain abscess, especially in children and adolescents with severe congenital heart disease.2 It is reported that single brain abscess in children accounts for 69.49%, and multiple abscesses account for 30.5-42%. Multiple abscess is more common in children with cyanotic congenital heart disease. (D-TGA) accounts approximately 5% to 7% of all congenital heart diseases. There is discordance of the ventriculoarterial connection.3 Management of brain abscess has been a challenge. Small brain abscesses have been treated empirically with antibiotics. Initial therapy should be commenced with broad spectrum antibiotics which cross blood-cerebrospinal fluid (blood-CSF) barriers 1

in adequate concentrations. Empirical antibiotic therapy should be started on the basis of the likely associated pathogens which depend on the presumptive precipitating source of infection and the gram stain results.4 The aim of this paper is to report the case of Transposition of great artery with mulitiple abscess in a 5 years 3 months old girls. HISTORY TAKING (alloanamnesis with patient’s parents) History of present illness AW, a 5 years 7 month old girls, come to emergency department at Haji Adam Malik hospital with chief complaint of headache. Headache was experienced since a month before admission. Headache was all over the head, happened frequently. Fever was suffered since 3 weeks before, fluctuated, and diminished with antipyretics. No history of seizure and shivering was not found. Bluish discoloration of fingers, mouth, and lips was noticed by parents since 3 years of age. History of squatting position was denied. Patient was looked very thin because of lack of appetite a month ago. Patient was only able consumed 2 spoons of rice. The highest weight was 13 kilogram a month ago. There was no history of ear discharge, head trauma, and vomiting. Defecation and micturition within normal limit. History of previous illness The patient was referred from Grand Medistra General Hospital Lubuk Pakam by general practitioner. Patient had bluish discoloration of fingers, lips and mouth since 3 years of age and had done echocardiography examination. The result of examination came up with congenital heart disease and suggested for having surgical procedure. The parents had chosen to delay the surgery because want to ask another expert opinion. Since then, she was treated as outpatient by cardiologist for a year. A month before admission she was treated by pharmacologist with digoxin, folic acid, multivitamin for 20 days. History of family Illness History of heart disease in the family was denied. Antenatal history Mother was 20 years old when pregnant. During the pregnancy the mother was healthy and got regular antenatal care with midwife and obstetrician. No history of taking any medicine. There was no complaint of vomiting, bleeding, hypertension or infectious illness. She was not smoking cigarette, not consuming traditional herb and also not drinking alcohol.

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History of labor The patient was born full term with spontaneous delivery by midwife. Birth weight was 3000 gram, with birth length and head circumference were not known. Patient was crying immediately, with no cyanosis and jaundice at birth. Postnatal history There was no history of bluish discoloration, shortness of breath, seizure, pale or yellowish in this patient. History of food intake  Age 0 - 5 months: breast milk (ASI)  Age 6 - 12 months: formula milk, rice milk  Age 1 year - 2 year: formula milk, soft porridge  Age 2 year - present: formula milk and family food with the amount of feeding 3 times a day, with 1 portion of rice and side dishes such as fish, eggs, and chicken. Since 1 month ago patient only consumed 2 – 3 tablespoon rice and side dishes once a day, 2 – 4 slices of bread and 2 – 4 pieces of biscuits once a day as snacks. Total calories for this one month was 600 calories / day Conclusion: poor quality and quantity of food intake History of immunization status According to patient’s mother, the basic immunization was given completely until 9 months and booster at 24 months old. BCG scar was found. Conclusion : Basic immunization of patient was complete.

Summary of medical reports before the case is accepted (Treatment from September 29th – October 10th 2018) Patient was hospitalized with chief complaint of headache, prolonged fever, weight loss and bluish discoloration of lips, mouth and fingers. Body weight in admission was 12 kg (weight for age 61.5 % 2000 CDC growth chart) with conclusion severely wasted, body height when admission was 107 cm (height for age 92 % 2000 CDC growth chart) with conclusion normal, weight for height was 66.7 % (2000 CDC growth chart) with conclusion severe malnutrition.. Mid upper arm circumference is 11 cm (
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– 100/40 – 60 mmHg (normal range 90-105 / 53-67 mmHg), with pain scale 3 out of 10 (mild pain). General condition was moderate and nutritional status was severe malnutrition. From the head examination, the patient had normal hair, prominent cheekbone. The patient did not have old man face. From the eye examination, we found that conjunctiva was not anemic, isochoric pupils, diameter 3 mm, positive light reflexes, no palpebral edema and subconjunctival bleeding. There was no abnormalities in the ears. There was cyanosis in the tongue, mouth and lips with no nasal flare. There was no nuchal rigidity, no increasing of jugular vein pressure and no lymph node enlargement in the neck examination. From chest examination, the ribs were clearly visible, without retraction. The heart rate was around 82 - 96 beats per minute (N: 65 – 130 beats per minute), reguler, grade III/VI systolic murmur in the 2nd and 3rd upper right sternal border. The respiratory rate was around 20 – 22 breaths per minute (N: 20 – 25 breaths per minute), reguler, with no rales. Prominent spine was clearly visible. From the abdomen examination, there was no distension, normal bowel sound, no shifting dulness, tympanic sound. Liver and spleen was not palpable. From the extremities, there was muscle hypotrophy, thinning of subcutaneous fat, and clubbing finger. The extremities were warm, pulse was around 82 - 96 beats per minute, same quality in the four extremities, reguler, capillary refill time less than 2 seconds, oxygen saturation 75 – 80 % in four extremities, and the blood pressure was 90/40 mmHg. Treatment that given during hospitalization: ceftriaxone injection 600 mg/12 hours/IV, paracetamol injection 150 mg/6 hours/IV, folic acid 1 x 5 mg (1st day) and 1 x 1 mg for the next days, Vitamin A 1 x 200 000 IU (once), Vitamin B complex 1 x 1 tablet , vitamin C 1 x 100 mg. Dietetic therapy was F75 135 cc per 3 hours with good monitoring of diet tolerance, gradually increased to F100 135 cc per 3 hours.

Table 1. Laboratory results Type of examination

Results

Complete Blood Count

27th-9-2018

Hemoglobin (g/dL)

18.4

10.8-15.6

60

33-45

Leucocyte (10 /mm )

18.61

4.5 – 13.5

Trombocyte (103/mm3)

450

181 – 521

Erythrocyte (million/uL)

9.84

4.5 - 6.5

MCV (fL)

61

69 – 93

MCH (pg)

18.7

22 – 34

MCHC (gr/dL)

30.9

32 – 36

Hematocryte (%) 3

3

Reference value

4

RDW (%)

20.9

11 – 15

Differential count (%) -

Eosinophil

0

1–5

-

Basophil

0.4

0–1

-

Neutrophil

76.3

25 – 70

-

Lymphocyte

15.9

25 – 40

-

Monocyte

7.4

1–6

Blood glucose ad random (mg/dL)

96

< 200

Calcium (mg/dL)

8.7

8.4 – 10.2

Sodium (mEq/L)

135

135 – 155

Potassium (mEq/L)

3.6

3.6 – 5.5

Chloride (mEq/L)

100

96 – 106

pH

7.47

7.35 – 7.45

pCO2 (mmHg)

30

38 – 42

pO2 (mmHg)

150

85 – 100

HCO3 (U/L)

21.8

22 – 26

TCO2 (U/L)

22.7

19 – 25

BE (U/L)

-1

(-2) – (+2)

SatO2 (%)

99

95 – 100

Ureum (mg/dL)

11

19 – 44

Creatinin (mg/dL)

0.48

0.7 – 1.3

Blood Gas Analysis

Table 2. Urinalysis result October 10th 2018

References

Colour

Light yellow

Yellow

Glucose

Negative

Negative

Bilirubin

Negative

Negative

Ketone

Negative

Negative

SG

1.005

1.005-1.030

pH

6.0

5-8

Protein

+2

Negative

Nitrite

Negative

Negative

Leukocyte

Negative

Negatifve

Blood

Negative

Negative

Erythrocyte (HPF)

0-1

<3

Leukocyte (HPF)

0-1

<6

Squamous Epithelial Cells (HPF)

0-1

Types of tests Urinalysis

Microscopic

5

Casts (HPF)

Negative

Negative

Crystals (HPF)

Negative

Negative

Electrocardiography result (September 26th 2018) Sinus bradycardia, with QRS rate 65 beats/minute Normoaxis, Left Ventricular Hypertrophy with Right Bundle Branch Block Chest XRay result (September 26th 2018) Suggested pneumonia Echocardiography (September 28th 2018) Double inlet left ventricle Single ventricle Transpostion of great artery (TGA) Severe pulmonary stenosis (PS) Microbiology Blood Culture (October 3rd 2018) No bacterial or fungal growth

Consultation results  Neurology division: DD SOL, brain abscess + double inlet left ventricle + single ventricle + TGA + severe PS + severe malnutrition. Suggestion : brain head CT-Scan with contrast.  Nutrition and metabolic disease division: severe malnutrition + double inlet left ventricle + single ventricle + TGA + severe PS + DD SOL, brain abscsess + oral thrust. Suggestion : severe malnutrition management based on pediatric nutrition care.  Growth and Development Social Pediatric division: Poor quality of life Suggestion: repeate the examination when patient discharge from hospital. Physical examination when receiving this patient (October 11th 2018) General examination Patient was fully alert, afebrile, and looked cyanosis. There was no anemic, edema, dyspnoe, and jaundice.

Vital sign:  Temperature

: 37.3 0C 6

 Heart beat

: 96 beats per minute

 Respiratory rate

: 20 breaths per minute

 Blood pressure

: 90/60 mmHg

Conclusion : normal vital sign

Nutritional status and anthropometry: Body weight

: 12.3 kg (under 5th percentile, 2000 CDC

Growth Chart) Weight / Age

: 63 % (2000 CDC, Growth Chart)

Body height

: 107 cm (between 10th and 25th

percentile, 2000 CDC growth chart) Height / Age

: 92 % (2000 CDC growth chart)

Body weight/ Height

: 68.3 % (2000 CDC growth chart)

Height age

: 5 years old (CDC 2000 growth chart)

Ideal body weight

: 18 kg

Mid upper arm circumference : 11 cm (under 5th percentile Frisancho curve) Head circumference

: 50 cm (between -2 and 2 SD

Nellhaus curve) Conclusion: severe malnutrition Specific examination 1. Head There was normal hair, prominent cheekbone, and no old man face. Eye : conjunctiva was not anaemic, isochoric pupils, diameter 3 mm, positive light reflex, no palpebral edema and subconjunctival bleeding. Ears : no discharge Nose : nasal flare, no nasal discharge Mouth : cyanosis in the lips and tongue 2. Neck Normal jugular vein pressure, no nuchal rigidity and no lymph node enlargement. 3. Chest The ribs were clearly visible, symmetrical fusiform, and no retraction. The heart rate was 92 beats per minute, reguler, grade III/VI systolic murmur in the 2nd and 3rd upper right sternal border. The respiratory rate was 22 breaths per minute, reguler, no rales. Prominent spine was clearly visible. 4. Abdomen

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No distension, normal bowel sound, no shifting dulness, tympanic sound. Liver and spleen was not palpable. 5. Extremities There was muscle hypotrophy, thinning of subcutaneous fat, and no clubbing finger. The extremities was warm, pulse was 92 beats per minute, same quality in the four extremities, reguler, and strong. Capillary refill time was less than 2 seconds, oxygen saturation 77-80% in four extremities, and the blood pressure was 90/60 mmHg. 6. Neurological examination Reflexes

: Normal physiology reflexes, pathological reflexes (-)

Sensoric

: Normal limit

Motoric

: Normal strength of four body limbs

Meningeal sign : Nuchal rigidity, (-) Brudzinki I and II (-) Kernig sign (-) Cranial nerves examination :  N I,II,III,IV,V,VI,VII,VIII,IX,X,XI,XII was normal.

DIFFERENTIAL DIAGNOSIS 1. Brain abscess (G.06.0) + Double inlet left ventricle + single ventricle (Q20.4) + TGA (Q20.3) + severe PS (Q22.1) + severe malnutrition (E43) 2. Intracranial Space Occupying lesion (R.90.0) + Double inlet left ventricle + single ventricle (Q20.4) + TGA (Q20.3) + severe PS (Q22.1) + severe malnutrition (E43) 3. Cerebral ischemia (I67.82) + double inlet left ventricle + single ventricle (Q20.4) + TGA (Q20.3) + severe PS (Q22.1) + severe malnutrition (E43)

WORKING DIAGNOSIS Brain abscess (G.06.0) + double inlet left ventricle + single ventricle (Q20.4) + TGA (Q20.3) + severe PS (Q22.1) + severe malnutrition (E43)

LIST OF PROBLEMS 1. Brain abscess - Diagnostic: history taking, physical examination, and head CT-scan with IV contrast. - Management: o Medical management: empirical antibiotics therapy and steroid injection. o Surgical management: consultation to neurosurgery department for abscess drainage.

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2. Double inlet left ventricle + single ventricle + TGA + severe PS - Diagnosis was established by echocardiographic examination. - The patient had not done the correction surgery. Correction of heart defect is neeeded to improve the patient’s quality of life

3. Severe malnutrition - Diagnosis was based on clinical sign and anthropometry - Severe malnutrition in this patient has many risk factors. Her congenital heart disease, pulmonary stenosis, hypoxia, and intake have contribution in developing this condition. The correction of her basic disease is the primary management to improve the nutritional status in addition to the adequate nutrition

PLANNING MANAGEMENT a. Emergency management plan The emergency situation in the patient with DILV and pulmonary stenosis is the hypoxic spell. Hypoxic spell should be recognized and managed immediately. If we faced this condition, we should give oxygen inhalation, placed the patient in knee chest position, administered morphine sulfate 0.1 – 0.2 mg/kg subcutan, intramuscular, or intravenous, sodium bicarbonate 1 mEq/kg/IV, and propranolol 0.01-0.25 mg/kg intravenous.

b. Action plan - Performing Head CT-Scan with IV Contrast to confirm the possibility of brain abscess. - Evaluation of routine blood examination - Consultation to neurosurgery department if the diagnosis of brain abscess established. - Refer to referral hospital with complete facilities for CHD surgical correction c. Pharmacologic therapy plan - Ceftriaxone injection 600 mg/12 hours/IV - Paracetamol injection 180 mg/6 hours/IV

d. Pediatric nutritional management - Nutrition problem : severe malnutrition

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- Calorie requirement : RDA x IBW = 1620 kkal - Nutritional intake : Oral - Food type : F100 formula 135 cc per 3 hours (50 – 75 % RDA x IBW) - Evaluation of response and tolerance to intake and monitoring of weight

e. Monitoring plan - Monitoring general appearance and vital sign - Monitoring signs of increase intracranial pressure - Monitoring nutritional intake, fluid intake, fluid balance, and diuresis. - Monitoring therapy, evaluation of response and side effect.

FOLLOW-UP First monitoring (October 11th 2018)

S

Fluctuated fever was found, no headache, no vomiting and diarrhea. Patients finished all formula.

O

Alertness: Compos mentis

Temperature (axilla): 37.00C

Wong Baker Faces Pain Rating Scales: 1 Body weight: 12.3 kg

Height: 107 cm

Upper arm circumference: 11 cm (< P5th) Head circumference: 50 cm ( -2<SD<2) W/A: 63 % H/A: 92 % W/H: 68.3 %

Head : Prominent cheek bone (+) Eyes: light reflexes (+), isochoric pupil, diameter 3 mm / 3

mm, pale

inferior palbebral conjungtiva (-), icteric sclera (-) Ears: no discharge Nose: no nasal flare Mouth: cyanosis (+), dental caries (+) Neck : nuchal rigidity (-), JVP : (R-2)cmH2O

Thorax: Symmetrical fusiform, without retraction, prominent ribs (+) HR: 92 beats/minute, reguler, systolic murmur grade III/VI ICS 2nd – 3rd URSB

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(normal range: 65-130 beats/minute) RR: 20 breaths/minute, without rales and wheezing (normal range: 20-24 breaths/minute) Abdomen: soft, normal peristaltic sound. No palpable liver and spleen Extremities: Warm, capillary refill time < 2 seconds, muscle hypotrophy and loss of subcutaneous fat (+), clubbing fingers (+), cyanosis (+) Blood pressure: 90/50 mmHg (normal limit: 90-105/ 53-67 mmHg) SatO2 : 77 – 80 % in four extremities

A

 Brain abscess  Double inlet left ventricle + single ventricle + TGA + severe PS  Severe malnutrition

P

 Ceftriaxone injection 1.5 gr/day/IV (1st day)  Ampicillin injection 1 gr/6 hours/IV (1st day)  Metronidazole loading dose 180 mg/IV continued with maintenance dose of 90 mg/8 hours/IV (1st day)  Dexamethasone injection 2 mg/6 hours/IV (1st day)  Paracetamol injection 180 mg/6 hours/IV  Vitamin B complex 1 x 1 tablet  Vitamin C 1 x 100 mg  Folic acid 1x1 mg  Diet F100 135 cc/3 hours/oral Head CT-Scan IV Contrast Result In accordance with brain abscesses Consultation Result from Neurology Division  Assessment: brain abscess + Double inlet left ventricle + single ventricle + TGA + severe PS + severe malnutrition  Suggestion: ampicillin injection 1 gr/6 hours/IV, ceftriaxone injection 1.5 gr/day/IV, metronidazole loading dose 180 mg/IV continued with maintenance dose of 90 mg/8 hours/IV, dexamethasone injection 2 mg/6 hours/IV and consulted to neurosurgery department.

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Consultation Result from Neurosurgery Department  Intracranial SOL with differential diagnosis of brain abscess. Suggestion : waiting for assessment.

Consultation Result from Department of Dentistry 

Assessment : rampant caires Suggestion : clean all teeth and tongue with warm water gauze, teeth brushing if possible, clean the oral cavity with betadhine gauze.

Second monitoring (October 12th 2018) S

Fever was not found, no headache, no vomiting and diarrhea. Patients finished all formula.

O

Alertness: Compos mentis

Temperature (axilla): 36.7 0C

Wong Baker Faces Pain Rating Scales: 1 Body weight: 12.3 kg

Height: 107 cm

Upper arm circumference: 11 cm (< P5th) Head circumference: 50 cm ( -2<SD<2) W/A: 63 % H/A: 92 % W/H: 68.3 %

Head : Prominent cheek bone (+) Eyes: light reflexes (+), isochoric pupil, diameter 3 mm / 3

mm, pale

inferior palbebral conjungtiva (-), icteric sclera (-) Ears: no discharge Nose: no nasal flare Mouth: cyanosis (+), dental caries (+) Neck : nuchal rigidity (-), JVP : (R-2)cmH2O

Thorax: Symmetrical fusiform, without retraction, prominent ribs (+) HR: 90 beats/minute, reguler, systolic murmur grade III/VI ICS 2nd – 3rd URSB (normal range: 65-130 beats/minute) RR: 20 breaths/minute, without rales and wheezing (normal range: 20-24 breaths/minute) Abdomen: soft, normal peristaltic sound. No palpable liver and spleen

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Extremities: Warm, capillary refill time < 2 seconds, muscle hypotrophy and loss of subcutaneous fat (+), clubbing fingers (+), cyanosis (+) Blood pressure:100/60 mmHg (normal limit: 90-105/ 53-67 mmHg) SatO2 : 78 – 80 % in four extremities

A

 Brain abscess  Double inlet left ventricle + single ventricle + TGA + severe PS  Severe malnutrition

P

 Ceftriaxone injection 1.5 gr/day/IV (2nd day)  Ampicillin injection 1 gr/6 hours/IV (2nd day)  Metronidazole maintenance dose of 90 mg/8 hours/IV (2nd day)  Dexamethasone injection 2 mg/6 hours/IV (2nd day)  Paracetamol injection 180 mg/6 hours/IV  Vitamin B complex 1 x 1 tablet  Vitamin C 1 x 100 mg  Folic acid 1x1 mg  Diet F100 180 cc/8 hours/oral + Regular meal 1500 kcals and 30 gr of protein

Consultation Result from Neurosurgery Department  Assessment : brain abscess. Suggestion : craniotomy for abscess evacuation Parents refused for craniotomy abscess evacuation. Consultation Result from Infection and Tropical Medicine Division Ceftriaxone injection change with vancomycin injection  allergic reaction Monitoring (October 13-16th 2018) S

Fever was not found, no headache, no vomiting and diarrhea. Patients finished all formula.

O

Alertness: Compos mentis

Temperature (axilla): 36.8 0C

Wong Baker Faces Pain Rating Scales: 1 Body weight: 12.3 kg

Height: 107 cm

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Upper arm circumference: 11 cm (< P5th) Head circumference: 50 cm ( -2<SD<2) W/A: 63 % H/A: 92 % W/H: 68.3 %

Head : Prominent cheek bone (+) Eyes: light reflexes (+), isochoric pupil, diameter 3 mm / 3

mm, pale

inferior palbebral conjungtiva (-), icteric sclera (-) Ears: no discharge Nose: no nasal flare Mouth: cyanosis (+), dental caries (+) Neck : nuchal rigidity (-), JVP: (R-2)cmH2O Thorax: Symmetrical fusiform, without retraction, prominent ribs (+) HR: 94 beats/minute, reguler, systolic murmur grade III/VI ICS 2nd – 3rd URSB (normal range: 65-130 beats/minute) RR: 22 breaths/minute, without rales and wheezing (normal range: 20-24 breaths/minute) Abdomen: soft, normal peristaltic sound. No palpable liver and spleen Extremities: Warm, capillary refill time < 2 seconds, muscle hypotrophy and loss of subcutaneous fat (+), clubbing fingers (+), cyanosis (+) Blood pressure:100/60 mmHg (normal limit: 90-105/ 53-67 mmHg) SatO2 : 78 – 80 % in four extremities

A

 Brain abscess  Double inlet left ventricle + single ventricle + TGA + severe PS  Severe malnutrition

P

 Ceftriaxone injection 1.5 gr/day/IV (3rd day)  Ampicillin injection 1 gr/6 hours/IV (3rdday)  Metronidazole maintenance dose of 90 mg/8 hours/IV (3rd day)  Dexamethasone injection 2 mg/6 hours/IV (3rd day)  Paracetamol injection 180 mg/6 hours/IV  Vitamin B complex 1 x 1 tablet

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 Vitamin C 1 x 100 mg  Folic acid 1x1 mg  Diet F100 180 cc/8 hours/oral + Regular meal 1500 kcals and 30 gr of protein

Prognosis (Ad vitam, ad functionam, ad sanationam) - Ad vitam

: dubia ad malam

- Ad functionam

: dubia ad malam

- Ad sanationam

: dubia ad malam

Discusion Congenital heart disease (CHD) is one of the most common structural malformation and comprises up to 25% of all congenital anomalies. It contributes significantly to infant morbidity and mortality. It occurs in 0.5–0.8% of live births, and each year there are about 1.5 million new cases worldwide. Congenital cardiac defects are grossly divided into two types; acynotic and cyanotic heart diseases.1 Cyanotic CHD is CHD with lesions that cause a mixture of oxygenated/oxygen-poor blood (deoxygenated blood) to the systemic circulation through intracardiac or extracardiac shunts.2 Clinical manifestations of patients with cyanotic congenital heart disease vary greatly. some patients show cyanotic symptoms due to hypoxemia with or without heart failure, some experienced shock, some did not show symptoms. In patients with cyanotic congenital heart disease, 65% are diagnosed under 2 years of age, 17% between of 2-5 years, and 18% over 5 years.3 By definition, cyanotic CHD does not include cyanosis due to intrapulmonary right-to-left shunting and pulmonary venous desaturation secondary to congestive heart failure. There are usually multiple defects of the heart causing right-to-left shunt. Obstruction to pulmonary blood flow (for example tetralogy of Fallot), complete admixture of pulmonary and systemic venous returns (for example, total anomalous pulmonary venous return and double-inlet left ventricle) and parallel rather than in-series circulation (transposition of the great arteries) are the causes of right-to-left shunts and cyanosis.4 Double-inlet left ventricle (DILV) or single ventricle is one of cyanotic CHD with admixture of pulmonary and systemic venous returns. Double-inlet ventricle occurs in fewer than 1% of all CHD. With a single ventricle, both atria empty through a common atrioventricular valve or via two separate valves into a single ventricular chamber, with

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total mixing of systemic and pulmonary venous return. Both atrioventricular (AV) valves are connected to a main, single ventricular chamber, and the main chamber is in turn connected to a rudimentary chamber through the bulboventricular foramen (BVF). One great artery arises from the main chamber, and the other arises from the rudimentary chamber. In about 80% of cases, the main ventricular chamber has anatomic characteristics of the left ventricle (double-inlet LV). Occasionally, the main chamber has anatomic characteristics of the right ventricle (double-inlet RV). Rarely does the ventricle have an intermediate trabecular pattern without a rudimentary chamber (common ventricle). Also, both atria rarely empty via a common AV valve into the main ventricular chamber with either LV or RV morphology (common-inlet ventricle). Either D-TGA or L-TGA is present in 85% of cases. The most common form of single ventricle is double-inlet LV with L-TGA with the aorta arising from the rudimentary chamber. This type occurs in 70% to 75% of single ventricle. The mitral valve is right sided; the tricuspid valve is left sided. PS or pulmonary atresia is present in about 50% of cases. COA and interrupted aortic arch are also common. Less commonly, D-TGA is present with the aorta arising from the right and anterior rudimentary chamber.5,6 The clinical picture is variable and depends on the associated intracardiac anomalies. If pulmonary out flow is obstructed, the findings are usually similar to those of tetralogy of Fallot: marked cyanosis without heart failure. If pulmonary out flow is unobstructed, the findings are similar to those of transposition with VSD: minimal cyanosis with increasing heart failure. In patients with pulmonary stenosis, cyanosis is present in early infancy. Cardiomegaly is mild or moderate, a left parasternal lift is palpable, and a systolic thrill is common. The systolic ejection murmur is usually loud; an ejection click may be audible, and the 2nd heart sound is single and loud. In patients with unobstructed pulmonary flow, as pulmonary vascular resistance drops, torrential pulmonary blood flow develops, and these patients present with tachypnea, dyspnea, failure to thrive, and recurrent pulmonary infections. Cyanosis is only mild or moderate. Cardiomegaly is generally marked, and a left parasternal lift is palpable. A systolic ejection murmur is present but is not usually loud or harsh, and the 2nd heart sound is loud and closely split. A 3rd heart sound is common and may be followed by a short mid-diastolic rumbling murmur caused by increased flow through the atrioventricular valves. The eventual development of pulmonary vascular disease reduces pulmonary blood flow so that the cyanosis increases and signs of cardiac failure appear to improve.5,6 The case was a 5 years 7 months old girl, which from

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history taking and physical findings suggest that patient experienced cyanotic CHD. Initial laboratory examination revealed that patient’s Hemoglobin 18.4 g/dL with leucocytosis 18610/mm3. There was grade III/VI systolic murmur in the 2 nd and 3rd upper right sternal border with clubbing finger on extremities. Patient was having headache and fever. Patient was planned to do head CT-Scan with IV contrast. Diagnosis of DILV can establish from history taking, physical examination, electrocardiography (ECG), radiography and echocardiography. From history taking, cyanosis of varying degrees may be present from birth. History of failure to thrive or pneumonia may be present in infants with increased pulmonary blood flow (signs of CHF). Physical findings depend on the magnitude of PBF. In cases of DILV with increased PBF, physical findings resemble those of TGA plus VSD or even those of large VSD. Mild cyanosis and CHF with growth retardation are present in early infancy. The S2 is single or narrowly split with a loud P2. A grade 3 to 4 of 6 long systolic murmur is audible along the left sternal border. An apical diastolic rumble may be audible. A diastolic murmur of PR may be present along the upper left sternal border as a result of pulmonary hypertension. In DILV with decreased PBF, physical findings resemble those of TOF, moderate to severe cyanosis is present. CHF is not present. Clubbing may be seen in older infants and children. The S2 is loud and single. A grade 2 to 4 of 6 ejection systolic murmur may be heard at the upper right or left sternal border.5,6 From electrocardiography, an unusual ventricular hypertrophy pattern with similar QRS complexes across most or all precordial leads is common (e.g., RS, rS, or QR pattern). Abnormal Q waves (representing abnormalities in septal depolarization) are also common and take one of the following forms: Q waves in the right precordial leads, no Q waves in any precordial leads, or Q waves in both the right and left precordial leads. Either first or second degree AV block may be present. Arrhythmias such as SVT or wandering pacemaker may occur. In radiography examination, with increased PBF, the heart size enlarges and the pulmonary vascularity increases. When PBF is normal or decreased, the heart size is normal and the pulmonary vascularity is normal or decreased. A narrow upper mediastinum in radiography, suggests TGA.5,6 In the case, ECG examination revealed sinus bradycardia, with QRS rate 65 beats/minute, normoaxis, Left Ventricular Hypertrophy with Right Bundle Branch Block. Echocardiography will confirm the absence or near absence of the ventricular septum and can usually determine whether the single ventricle has right, left , or mixed

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morphologic features. The presence of a rudimentary out flow chamber under one of the great vessels can be identified, and pulsed Doppler can be used to determine whether flow through this communication (known as a bulboventricular foramen) is obstructed. The following anatomic and functional information is important from a surgical point of view. Efforts should be made to obtain information on all of these aspects in each patient with single ventricle5,6 : - Morphology of the single ventricle (e.g., double-inlet LV? double-inlet RV?). - Location of the rudimentary out flow chamber, which is usually left and anterior. - Size of the BVF and whether there is an obstruction at the foramen. Obstruction of the foramen is considered present if the Doppler gradient is more than 1.5 m/sec or if the area of the foramen is less than 2 cm2/m2. A foramen that is nearly as large as the aortic annulus is considered ideal. - Presence or absence of D-TGA or L-TGA, stenosis of the pulmonary or aortic valve, and size of the PAs. - Anatomy of the AV valves. The position of the mitral and tricuspid valves, in addition to the presence of stenosis, regurgitation, hypoplasia, or straddling of these valves, should be checked. - The size of the ASD. - Associated defects such as COA, interrupted aortic arch, or PDA. Echocardiographic and Doppler studies provide most of the anatomic and hemodynamic information needed for the initial management of single ventricle. Cardiac catheterization is performed only when certain preoperative information is not available before the initial stage of surgical management. It is, however, routinely indicated before stages II and III surgical intervention.5,6 In the case, from echocardiography finding we found DILV, single ventricle, TGA and severe pulmonary PS. Patient was planned for having cardiac catheterization to measure the actual pressure gradient of PS and other possibility of cardiac anatomy abnormalities in referral hospital. Management of DILV consists of medical and surgical management. Medical management in DILV is usually needed in newborns with severe PS or pulmonary atresia and those with interrupted aortic arch or coarctation which require PGE1 infusion and other supportive measures before surgery. Anticongestive measures are indicated if CHF develops in patients with DILV. Surgical management in DILV consists of 5,7: Initial surgical palliative procedure

18

a. The purpose of the first-stage operation is to make patients acceptable candidates for bidirectional Glenn or hemi-Fontan operation. Cyanosis with oxygen saturation less than 85% or increased pulmonary blood flow with possible future pulmonary hypertension is an indication for the operation. The presence or absence of PS or of obstructive BVF results in one of the following four situations. PS (or pulmonary atresia) is present in about 50% of the patients. When there is no PS, pulmonary overcirculation can lead to pulmonary hypertension, which jeopardizes future Fontan operation. 1. In patients with no PS and large PBF with resulting CHF and pulmonary edema, PA banding may be done, although the banding carries a high mortality rate (≈25% or even higher). The major risk factor for the banding is the presence or development of an obstructed BVF. Most infants with an obstructed foramen do not tolerate the banding well. Therefore, PA banding is performed, only when the BVF is normal or unobstructed. In addition, these patients should be watched for the development of obstruction after the banding. 2. In patients with no PS, if the BVF is too small, the Damus-Kaye-Stansel operation is performed rather than the PA banding. The operation involves a PA-to-aorta anastomosis, which is accomplished by transection of the main PA and anastomosis of the proximal PA to the ascending aorta. This operation is combined with a right-sided BT shunt or a single ventricle–to–the main PA (Sano) shunt (not shown). A Fontantype operation can be performed later. 3. If PS or pulmonary atresia is present (with O2 saturation <85%), a BT shunt is necessary to improve cyanosis. Shunt to the right PA is preferable because any distortion of the RPA can be incorporated later in the Fontan anastomosis. The mortality rate is low (5%–10%). In PGE1-dependent neonates, the PDA is ligated after placement of the shunt. Recently, a hybrid procedure consisting of PDA stenting, bilateral PA banding, and balloon atrial septostomy (with or without balloon dilatation), as discussed under HLHS, has been used as an alternative to the BT shunt. 4. If PS is present and the BVF is obstructive, enlargement of the BVF by a transaortic approach and without cardiopulmonary bypass may be performed. The surgical mortality rate is about 15%. An additional BT shunt may be needed to provide adequate pulmonary blood ow. b. Surgery for interrupted aortic arch or coarctation should be performed, if present. c. After the first-stage operation,the infant should be watched closely, until the time of the second-stage palliation, for cyanosis (with O2 saturation <75%) or signs of CHF

19

(too large a pulmonary blood ow for which tightening of the PA band should be considered). Second-stage surgical palliative procedures a. A bidirectional Glenn operation is carried out between the age of 3 and 6 months, before proceeding with the Fontan operation. Alternatively, a hemi Fontan procedure can be performed. b. After the second-stage surgical procedure, the child needs to be followed up with attention to the O2 saturation. Defenitive (Fontan) procedures The Fontan-type operation is performed at 18 to 24 months of age. Many centers consider the lateral tunnel Fontan procedure the procedure of choice. Some centers make a 4- to 6-mm fenestration in the baffle, and others do not. Some centers prefer the extracardiac conduit modification of the Fontan procedure, which may reduce the incidence of late atrial arrhythmias. If an AV valve is incompetent, it may need to be closed during surgery. The surgical mortality rate of the Fontan-type operation has been reduced to 5% to 10%, similar to that for tricuspid atresia.

Figure 3. Surgical approach for single ventricle. BDG, bidirectional Glenn; BVF, bulboventricular foramen; BT, Blalock-Taussig; CHF, congestive heart failure; PBF, pulmonary blood ow; PS, pulmonary stenosis; RPA, right pulmonary artery.5

Some survivors of surgery, if performed late, remain symptomatic with cyanosis, dyspnea as a result of ventricular dysfunction, and arrhythmias. These symptoms require regular follow-up. Early surgery as outlined above tends to reduce unfavorable results.5,7 The patient with this kind of cyanotic CHD usually need early palliative surgery, but this patient had already passed the periode when diagnosis was established. The patient planned to do the surgical correction in referral hospital.

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Unoperated, some patients succumb during infancy from heart failure. Others may survive to adolescence and early adult life but finally succumb to the effects of chronic hypoxemia or, in the absence of pulmonary stenosis, to the effects of pulmonary vascular disease. Patients with moderate pulmonary stenosis have the best prognosis because pulmonary blood flow, though restricted, is still adequate.6 Patients with unoperated SV physiology rarely survive into adulthood with good functional status and may develop Eisenmenger’s syndrome (ES).8 A recent cohort study in United States, revealed that selected patients with unoperated SV and PS may live up to the eighth decade of life with preserved ventricular function and functional class with meticulous medical management.9 Brain abscess is a focal, intracerebral infection that begins as a localized area of infection and develops into a collection of pus surrounded by a well-vascularized capsule.10 It results from spread of infection from surrounding non-neuronal tissue such as middle ear, fracture skull, intracranial surgery, hematogenous spread as in congenital heart disease with a right to left shunt (5%-18.7%) or a direct introduction into the brain.11 Average age of clinical presentation of affected children was 9,2±5,5 years.12 The most common cardiac anomaly associated with brain abscess such as tetralogy of fallot, transposition of great vessels, tricuspid atresia, pulmonary stenosis, and double-outlet right ventricle have also been reported as predisposing factors.9 Most of these abscesses are supratentorial in location.13 A study in Pakistan about predisposing factors of brain abscess in children, showed that DILV had only 1 % compared with other cyanotic CHD.14 A study of case series in China reported a patient with multiple brain abscesses and DILV, which is very rare, and treated with antibiotics and surgery.15 Common pathogens isolated in the brain abscess include: Streptococci (aerobic, anaerobic and microaerophili), Staphylococcus aureus, Bacteroides, enteric gram negative bacilli, Pseudomonas spp, Haemophilus influenzae, fungi and protozoa.16 Peptostreptococcus and Streptococcus (esp. viridians and microaerophilics) are mostly identified in patient with cardiac origin cyanotic heart disease and right-to-left shunts.17 In addition, decreased arterial oxygenation can result in compensatory polycythemia and increased blood viscosity can cause a focal area of ischemia that serves as a nidus for infection.10 The latter results in sluggish blood flow in cerebral microcirculation, microthrombi formation and direct entry of organisms, emboli, infected seed to cerebral circulation forming cerebral abscess.11,17 Intracardiac right to left shunt by-

21

pass allows direct entry of blood containing bacteria to the cerebral circulation without pulmonary filtration where bacteria are intercepted by phagocytosis, may allow direct entry to cerebral circulation.10,14, 17 Brain abscess should be suspected in children with fever, headaches, new onset seizures, vomiting, focal neurologic deficits, decreased consciousness and signs of increased intracranial pressure. Because on early evaluation most children with cerebral abscess present with vague or nonspecific signs and symptoms, the physician must have a high index of suspicion in order to recognize the condition as early as possible (table 2). Brain abscess development can be divided into four stages. Staging of brain abscess in humans has been based on findings obtained during CT or MRI scans.17,18 Table 1. Stages of brain abscess formation18 Stage

Time

Characteristics

Early cerebritis

1-4 days

This stage is typified by neutrophil accumulation, tissue necrosis and edema. This is the moment for microgial and astrocyte activation that longs through abscess development.

Late cerebritis

4-10 days

This phase is associated with predominant macrophage and lymphocyte infiltrate Associated with the formation of a well-

Early capsule formation

11-14 days

vascularized abscess wall, this wall is crucial

sequestering

the

lession;

maintaining the integrity of the brain function and limiting the expansion of the infective process At 3-4 weeks, the abscess capsule Late capsule formation

>14 days

becomes thick and is anenable to excision

Table 2. Common presenting signs and symptoms in brain abscess18 Sign/symptom

Frequency (%)

Fever

54,5-60

Headache

72-92,8

Hemiparesis/cranial nerve

14,5

Hemiparesis

20,2

No neurological deficit

39,8

Meningism

52,2

22

Altered level of consciousness

10-100

Seizure

21-25,5

Nausea/vomiting

31-40

Papilledema

4,1-50

GCS, at admission 3-8

10,3

9-11

28,0

13-15

61,7

The peripheral white blood cell count can be normal or elevated, and blood culture is positive in 10% of cases. Culture of pus from the neurological drainage is the key to establishing a bacteriologic diagnosis. Head CT-scan with contrast and MRI are the most reliable methods of demonstrating cerebritis and abscess formation.19 From head CT-scan, we can exact localization, and accurate characterization, determination of number, size and staging of the abscess. It also detects hydrocephalus, raised intracranial pressure (ICP), edema and associated infections like subdural empyema, ventriculitis and thus helps in treatment planning.17 The treatment of brain abscess has been a challenge. Small brain abscesses have been treated empirically with antibiotics. Initial therapy should be commenced with broad spectrum antibiotics which cross blood-cerebrospinal fluid (blood-CSF) barriers in adequate concentrations.17 Empirical antibiotic therapy should be started on the basis of the likely associated pathogens which depend on the presumptive precipitating source of infection and the gram stain results.10,19 When the cause is unknown, the combination of vancomycin, a third generation cephalosporin and metronidazole is commonly used. When cyanotic congenital heart disease is the predisposing factor, ampicillin-sulbactam alone or a third-generation cephalosporin plus metronidazole may be used.19 Metronidazole readily penetrates brain abscesses, it has excellent bactericidal activity against many anaerobes.17 The duration of the antibiotics course is usually 6–8 weeks and longer for immunocompromised patients.20 A brain abscess can be treated with antibiotics without surgery if the abscess is < 2 cm in diameter, the illness is of short duration (<2 weeks), there is no signs of increased intracranial pressure and the child is neurogically intact.17,19 If the decision is made to treat with antibiotics alone, the child should have follow up neuroimaging to ensure the abscess is decreasing in size.17 It is important to follow by CT or MR imaging until the abscess has completely resolved.16,17 Steroids reduce brain edema but diminish the effectiveness of the host defense mechanisms that assist in containment of infection.

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Steroids inhibit collagen capsule formation and also inhibit migration of leucocytes. They are therefore, avoided in the stages of early and late cerebritis. Steroids when used are tapered as rapidly as possible.17 Majority of the patients underwent aspiration as a primary modality of treatment, if the abscesses are of <2.5 cm size.17 However, if abscess enlarges after two weeks of antibiotics or fails to resolve after three to four weeks, larger than 2 cm in diameter, causing midline shift, multiple abscesses, gas is present in the abscess, neurological deficits, the lession is located in the fossa posterior or a fungus is identified require surgical excision under general anaesthesia in small children.11,19, 21 If the abscess is superficial and located close to the surface of the brain, a small craniotomy or burr hole may be used for abscess drainage.20 About 90% of the supratentorial hemispheric abscesses resolve with burhole drainage. The indication craniotomy are multiloculated abscess, thick pus, performed in cerebellar, postoperative and posttraumatic abscesses. Deep seated (thalamic, brain stem) and hemispheric eloquent area abscesses underwent frame based stereotactic or neuronavigation guided aspiration.17 This patient came with chief complaint of headache accompanied with fever and cyanotic CHD as presdisposing factor. Patient was planned to do head CT Scan with iv contrast to confirm the possibility of brain abscess. The result shows multiple brain abscesses on right and left frontotemporoparietal lobes with diameter > 2.5 cm. The patient was treated with antibiotics: ceftriaxone, ampicillin, metronidazole and dexamethasone injection. And consulted to neurosurgery department for brain abscess evacuation, but the parents refused for having the surgery. Follow up on neuroimaging examination is still needed to evaluate the treatment’s response. Congenital heart disease (CHD) is often associated with malnutrition and failure to grow. The prevalence of acute malnutrition (wasting) in children with CHD is quite high, about 41.1%, while chronic malnutrition (stunting) is 28.8%. Acute malnutrition (wasting) is more common in acyanotic CHD (58.3%) while chronic malnutrition (stunting) is more common in cyanotic type CHD, which is 68.0%.21 There is a strong relationship between energy intake, energy use, nutritional status and growth in infants and children. Although children with CHD have normal birth weight but malnutrition and failure to thrive often appear in the first months of life. Children with CHD who require surgery or intervention are more likely to have body weight, body length and head circumference below the third percentile on the growth chart.22 A retrospective cohort study in United States that compared longitudinal growth of 856 children with CHD and healthy children reported the biggest difference in achievement of body

24

weight occurred at 4 months and after 36 months catch up growth was no longer seen in children with CHD.23 The mechanism of malnutrition in CHD is complex and multifactorial involving several factors that interact in a complex system such as body immunity, imbalance of the body's catabolic and anabolic processes, and neurohormonal. There are several factors that contribute the mechanism of malnutrition and failure to thrive in children with CHD (Table 3).24 Table 3. The underlying mechanism of malnutrition in congenital heart diseases.24 Mechanism

Etiology

Inadequate intake

Loss of appetite Feeding difficulty Respiratory tract infection Fluidrestriction Side effects of drugs

Bad Absorption

Vein congestion Decreased lymphatic flow

Increased loss

Excessive vomiting Diarrhea Gastroosephageal reflux

Increased demands

Increased energy expenditure Greater proportion of lean body mass Catabolic state

Decreased growth potential

Lack of IGF-1 hormones Chronic hypoxemia Poor glucose tolerance Low of serum insulin level

Extracardiac factors

Cromosomal anomaly Genetical syndrome Chronic systemic illness Psychosocial problems

Children with CHD often have inadequate calorie intake, poor absorption and excessive loss. Some researchers have shown that there is a correlation between inadequate calorie intake and malnutrition. Inadequate calorie intake can occur when a child with CHD loses their appetite or may be secondary to the body's inability to utilize nutrients for growth due to anoxia, acidosis, malabsorption and or increased

25

nutritional needs. Children with CHD will usually eat in the following ways: a few minutes of sucking and swallowing, followed by a decrease in appetite, increased breathing rate and sweating.25, 26 Anorexia and satiety can also be associated with medications such as diuretics. Hepatomegaly that occurs secondary to congestive heart failure causes compressive pressure on the stomach, thereby reducing gastric volume and increasing the gastroesophageal potential of reflux and aspiration. In addition, congestive heart failure is responsible for the development of intestinal edema and hypoxia resulting in dysmotility and malabsorption. In children with severe CHD the process of maturation and gastrointestinal function slower than healthy children due to chronic hypoxia.27 A study in Egypt found that children with anemia, low oxygen saturation, heart failure, poor feeding history, pulmonary hypertension have a risk of malnutrition in patients with congenital heart defects.28 In the case, patient already had congenital heart disease since the age of 3 years and had not performed the surgery. On physical and anthropometric examination the patient was diagnosed with severe malnutrition accompanied by congenital heart disease and pulmonary stenosis. The management of malnutrition in cyanotic CHD patients is similar with other malnutrition, including 10 steps of management according to WHO and the Ministry of Health of the Republic of Indonesia.29 Patient was given initial treatment and malnutrition management, including dietary management, single-dose vitamin A 200.000 IU capsules, 5 mg folic acid in first day and 1 mg once daily for the next days, non-iron multivitamins during stabilization and transition phase. Patient shows good tolerance in diet, with weight gain and no vomiting or diarrhea. Patient’s prognosis is divided into ad vitam, ad functionam, and ad sanationam. Ad vitam prognosis is dubia ad malam, because five years survival rates for DILV (Single Ventricle) is 56.1 % (95 % CI).30 Ad functionam prognosis is dubia ad malam. Ad sanationam prognosis is dubia ad malam. Family education is an important thing. Educations that can be given to her family include patient’s condition, diagnosis, treatment plan, and disease progression. Good knowledge creates a good family environmental which support treatment process so the patient can reach optimal growth and development with a good quality of life. REFERENCES 1. Khan I, Muhammad A, Muhammad T. 2011. Pattern of Congenital Heart Disease at Lady Reading Hospital Peshawar. Gomal Journal of Medical Sciences, vol. 9, no. 2, hlm. 174-177. 2. Report of the New England Regional Infant Cardiac Program. Pediatrics. 1980;

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3. 4. 5. 6.

7. 8. 9.

10.

11.

12. 13. 14. 15.

16. 17. 18.

19.

20. 21.

22.

23.

65:375–461. Prasodo AM. Penyakit jantung bawaan sianotik. In: Sastroasmoro S, Madiyono B, editor. Buku ajar kardiologi anak. Jakarta: Binarupa Aksara; 1994. p. 234-5. Rao PS. Diagnosis and Management of Cyanotic Congenital Heart Disease: Part I. Indian J Pediatr. 2009; 76(1) : 57-70. Park MK. Cyanotic congenital heart disease. In: Park MK, Salamat M, editors. Park’s The Pediatric Cardiology Handbook. 5th ed. Elsevier. 2016: p.272-5. Bernstein D. Single ventricle (double-inlet ventricle, univentricular heart). In: Kligman RM, editor. Nelson Text Book of Pediatrics 20th ed. Elsevier. 2011: p.229-30. Alsoufi B. Management of the single ventricle and potentially obstructive systemic ventricular outflow tract. J Saudi Heart Assoc. 2013;25:191–202 Ammash NM, Warnes CA. Survival into adulthood of patients with unoperated single ventricle. Am J Cardiol. 1996;77:542–4. Poterucha JT, Anavekar NS, Egbe AC, Julsrud PR, Connoly HM, Ammash NM, et al. Survival and outcomes patients with unoperated single ventricle. Heart. 2015; 0:1-7. Lumbiganon P, Chaikitpinyo A. Antibiotics for brain abscesses in people with cyanotic congenital heart disease (Review). Cochrane Database of Systematic Reviews. 2013; 3:2-5. Basantwani S, Karnik H, Govardhane B, Tendolkar B. A case of uncorrected DTGA for craniotomy in cerebral abscess: anaesthesia management. Pediatric Anesthesia and Critical Care Journal.2015; 3(2):99-102 Jaddah RH. Thirteen-Year-Old Boy with Undiagnosed Congenital Heart Disease Presented with Brain Abscess. Bahrain Medical Bulletin. 2013; 35(3):1-5. Moorthy RK. Rajshekhar V. Management of brain abscess: an overview. Neurosurg Focus.2008; 24 (6):1-6. Atiq M, Uhmed AS, Allana SS, Chisti KN. Brain abscess in children. Indian J Pediatr 2006; 73 (5) : 401-404. Jiang J, Wei L, Zhang Y, Sun Z. Atypical multiple brain abscesses with congenital single ventricle: a case report. Int J Clin Exp Med. 2018;11(3):26802684. Mustafa M, Iftikhfar M, Latif MI, Munaidy RK. Brain abscess: pathogenesis, diagnosis and management strategies. IMPACT: IJRANSS. 2014;2(5):299-307. Muzumdar D, Jhawar S, Goel A. Brain abscess: An overview. International Journal of Surgery 9. 2011:136-144. Miranda HA, Leones SMC, Elzain MA, Moscote-Salazar LR. Brain abscess: Current management. Journal of Neurosciences in Rural Practice. 2013; 4(1): S67-81. Charles G, Prober, Mathew R. Brain abscess. In: Kliegman RM, Stanton BF, St Geme III JW, Schor NF. Nelson textbook of Pediatrics Edition 20. Elsevier. 2016. 2949-50. Fraizer JL, Ahn ES, Jallo GI. Management of brain abscesses in children. Neurosurgery focus. 2008;24(6): 1-10. Routry SS, Raut K, Mishra D, Mishra R. Cerebral Abscess in a 8 years old with uncorrected tetralogy of Fallot: Anaesthetic challeng. IJBAR. 2013;04(11): 8435. Okoromah CAN, Ekure EE, Lesi FEA, Okunowo WO, Tijani BO, Ekeiye JC. Prevalence, profile and predictors of malnutrition in children with congenital heart defects: a case- control observational study. Arch Dis Child 2011;96:35460. Daymont C, Neal A, Prosnitz A, Cohen MS. Growth in children with congenital heart disease. Pediatrics 2013;131:e236-e42

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24.

25. 26. 27. 28.

29. 30.

Venugopalan P. Malnutrition and the Heart: A Pediatric Perspective. In: Watson RR&Preedy VR, editor. Nutrition and heart disease causation and prevention. Parkway:Taylor&Francis group; 2004. p.237-79. Nydegger A, Bines JE. Energy metabolism in infants with congenital heart disease. Nutrition 2001 (22):697-704 Forchielli ML, McColl R, Walker WA, Lo C. Children with congenital heart disease: A nutrition challenge. Nutr Rev 1994(52): 348-53 Shondheimer JM, Hamilton JR. Intestinal function in infants with severe congenital heart disease. J Pediatr 1987;92:572-8 Hassan BA, Albanna EA, Morsy SM, Siam AG, Al Shafie MM, Elsaadany HF, et al. Nutritional status in children with un-operated congenital heart disease: an Egyptian Center Experience. Front Pediatr. 2015;3:1-5. Departemen Kesehatan Republik Indonesia. Buku bagan tata laksana anak gizi buruk. Buku I dan II. Edisi ke-4. Jakarta; 2003 Fixler DE, Nembhard WN, Salemi JL, Ethen MK, Canfield MA. Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003. Circulation. 2010;121:644-650.

Journal references for EBP Fixler DE, Nembhard WN, Salemi JL, Ethen MK, Canfield MA. Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003. Circulation. 2010;121:644-650

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Appraising articles on Prognostic Aspect (Evidence-Based Practice)

Are the results of the study valid? 1. Was the defined representative sample of patients assembled at a common (usually early) point in the course of their disease ? √ Yes

No

Unclear

Comment : The sample was patients with hypoplastic left heart syndrome, pulmonary atresia intact ventricular septum, single ventricle, and tricuspid atresia born in 1996 to 2003 were identified from the Texas Birth Defects Registry and linked to state and national birth and death vital records. 2. Were the patients sufficiently homogenous with respect to prognostic factor ? √ Yes

No

Unclear

Comment : Yes, this study was follow up 5 years from infants born with functional single ventricles, namely HLHS, PA-IVS, single ventricle complex (SV), and tricuspid atresia (TA). 3. Was there adjustment for important prognostic variables? √ Yes

No

Unclear

Comment : In this study patient was classified into 2 groups based on the presence or absence of life-threatening noncardiac congenital anomalies and also examined the effect of demographic variables on survival, including maternal race/ethnicity, education, age. 4. Were objective and unbiased outcome criteria used? √ Yes

No

Unclear

Comment : This study desribed the survival in first 5 years of patients with functional single ventricle. What are the results? 1. How likely are the outcomes over time? Patient with single ventricle have risk of death 4.1 fold higher D-TGA and the presence of an extracardiac defect reduced crude 5-year survival from 63.5% to 28.1%. 2. How precise are the prognostic estimates?

29

In this study, patient with single ventricle have corresponding mortality cure 0.47 (CI 95%).

Can I apply this evidence about prognosis to my patient? 1. Are your patients similar to those of the study? √ Yes

No

Unclear

Comment : My patient is similar with the typical group of single ventricle in this study. 2. Will this evidence make a clinically important impact on my conclusions about what to offer to tell my patients ? √ Yes

No

Unclear

Comment : This evidence could help us to explain about the prognosis of the patient, that the five years survival of patient with single ventricle is about 56 %.

Conclusion : This journal is valid, important and applicable. We can explain to the parents that the five years survival of children with single ventricle is 56 % and there are important factors affecting this survival; include birth era, birth weight, presence of extracardiac defects, maternal race/ethnicity, and maternal residence location.

30