Tetralogy of Fallot

Presented By:
Rifqi Mahfuzh Al-maaarij 110100028
Yogashree Rajendran

110100435

Supervisor :
Dr. Emil Azlin, Sp.A(K)

Pediatric Department
H. Adam Malik Hospital
Medical Faculty of
Sumatera Utara University

ToF
4

anatomic
malformations:
-Right Ventricular
Hypertrophy
-Pulmonary Valve
Stenosis
-Transposition of
the aorta
-Ventricular Septal
Defect

ToF
RVH

-secondary to PA Stenosis
-Increased P on RV leads to RVH
Transposition of Aorta
-aorta is displaced
VSD
-hole in the heart
-mixing of oxygenated and unoxygenated blood
-cyanosis
PVS
-more severe, less blood transported to the
lungs and more deoxygenated blood will pass
through VSD to aorta to be circulated throughout
the body

Epidemiology
The

Most Frequently cyanotic

congenital abnormalities of heart
3-6 : 10.000 live birth
7-10% of all congenital cardiac
malformation

Etiology
Theory:

destruction of the
neuronal crest cells during
embryogenesis
In the laboratory setting,
destruction of these cells
reproduced results displayed with
certain cardiac malformations.
Untreated maternal diabetes,
PKU, and intake of retinoic acid.
Chromosomal anomalies

Clinical Presentation
Clinical

presentation is directly
related to the degree of pulmonary
stenosis.
Ejection Sistolic Murmur
Severe stenosis results in immediate
cyanosis following birth. Mild
stenosis will not present until later.
Growth is retarded insufficient
oxygen and nutrients

Tet Spell
Tet

spells at
2-3yo , child
becomes
cyanotic, may
experience
syncope

Exams and Tests

CBC

- hematocrit
ECG
-RVH, RAD
CXR
-boot shaped
heart, right sided
aortic arch
Echocardiogram
To Confirm Diagnosis
-VSD

 Diagnosis

Differential
Pulmonary Atresia
Double outlet right ventricle and
pulmonary stenosis
Transposisi of great arteri and pulmonary
stenosis
Management
Paliative treatment: Blalock-Taussig shunt
Definitive: total correction

Case Report
Objective

The objective of this case is to report a

case of 16 year old girl with a diagnosis
of Tetralogy of Fallot.
Case

SS,16 years old girl , with 52 kg came

to emergency unit in Haji Adam Malik
General Hospital Medan on 5th June
2015 at 19.30 WIB. Chief of complain
was dyspnea with cyanosis in the
perifer.

History of Disease :

SS,16 years old girl , with 52 kg came to emergency unit in

Haji Adam Malik General Hospital Medan on 5th June 2015 at
19.30 WIB. Chief of complain was dyspnea . Dyspnea was
experienced by SS past 4 days and get worsen 1 day before
addmitted in hospital. History of tiredness was founded
since she was in 4SD class. She has a history of dyspnea
since she was in 4 SD She takes a squat position when she
has a lot of activity during the walk. Complain of dyspnea
was irrelevant with change in wether and daily
activities.There is cyanosis in patient and its been four
days.History of cyanosis present since the patient in 4SD
class. Cyanosis found on the finggertips, lip, and
face.Recurrent fever was (+) in this past 4 days . High fever
was not found .Shivering was not found .Seizure was not
found.SS has a history of treatment in RSUPHAM while she
was in 4SD and their parent doesnt remember the
diagnosis of the patient and after this the patient was never
treated again in RSUPHAM again. SS , was treated with
Sp.PD with diagnosis narrow of the heart. History of Disease
.History of Medication Aspilet,Digoxin,Spironolacton and
Levofloxacin

History

of Medication
Aspilet,Digoxin,Spironolacton,Levofloxacin
Family History :
Her cousin has a congenital heart disease
History of Pregnancy :
The age of the mom was 38 years old during pregnancy
with G3P3A0. The gestation age was 38 weeks. Her mom
regularly control pregnancy. History of hypertension post
partum was found. History of Diabetes Melitus was not
found. Usage of drugs (-), Usage of herbs (-).
History of birth :
Birth assisted by obstetricians, baby was born by sectio
cesarea. The baby immediately cry. Blue or cyanosis was
not found. Birth body weight : 2700 gr, birth body length :
50 cm, and head circumference was unclear.
History of breast feeding :
Exclusive breastfeeding until 6 months.

06 JUNE 2015

Dyspneu (+) , Fever (+)

S : Sensorium: alert; T: 37.8oC; BW: 52 kg, BH: 160 cm BB/U : 94% , TB/U :97% ,
BB/TB : 106% , Oedem (-) , Cyanosis (+) , Dyspnea (+)
Head :
Eye: light reflex (+/+), isochoric pupil, pale inferior conjunctiva palpebra (-/-)
Ear: within normal range
Nose: O2 Nasal canal
Mouth: cyanosis (+)
Neck: lymph nodes enlargement (-)
Thorax: symmetrical fusiform, retraction (-)
HR: 105 bpm, reg, ejection sistolik murmur (+) grade III/6 left linea midclavicularis
ICR II/III
RR: 27 bpm, reg, rales (-/-)
Abdominal: soft, tender, peristaltic (+) N, Liver and Spleen not palpable
Extremities: pulse 105 bpm, reg, p/v adequate, warm acral, CRT < 3, fingertip
cyanosis (+)

A: DD / Tetralogy of Fallot
-Transposition of Great Arteries (TGA)
-DORV

Oxygen Nasal Canul 1-2 L/Menit

Injection Ampicillin 1 gram / 6 hours/IV

BB/U :94%
TB/U:97%
BB/TB:106%

Hematology

Diftel

HGB 16g% (11.3-14.1)

RBC 50.86/mm3 (4.40-4.48)
WBC 14.47103/mm3 (4.5-13.5)
Ht 50.60% (37-41)
PLT 339 103/mm3 (150-450)
MCV 78.10 fl (81-95)
MCH 27.00 pg (25-29)
MCHC 34.60 g% (29-31)
RDW 15.70% (11.6-14.8)

Neutrophil 15.70% (37-80)

Lymphocyte 68.30% (20-40)
Monocyte 11.50% (2-8)
Eosinophil 1.50% (1-6)
Basophil 3.000% (0-1)
Absolute neutrophil 2.26 103
/L (2.4-7.3)
Absolute lymphocyte 9.88 103/
L (1.7-5.1)
Absolute monocyte 1.67 103/
L (0.2-0.6)
Absolute eosinophil 0.22 103/
L (0.1-0,3)
Absolute basophil 0.44 103/ L
(0-0.1)

07 JUNE2015
S

Dyspnea (+), Oedema (-), fever (-),

Sensorium: alert; T: 36. 7oC; BW: 52 kg, BH: 160 cm , BB/U : 94% , TB/U :97% , BB/TB : 100% ,
Oedem (-) , Cyanosis (+) , Dyspnea (+)
Head :
Eye: light reflex (+/+), isochoric pupil, pale inferior conjunctiva palpebra (-/-)
Ear: within normal range
Nose: Oxygen Nasal Canul
Mouth: cyanosis (-)
Neck: lymph nodes enlargement (-)
Thorax: symmetrical fusiform, retraction (-)
HR: 100 bpm, reg, ejection sistolic murmur grade III/6 mid clavicularis line ICR II/III
RR: 28 bpm, reg, rales (-/-)
Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpable
Extremities: pulse 100 bpm, reg, p/v adequate, warm acral, CRT < 3

-TOF

O2 Nasal Canul 1-2 L/Menit

Injection Ampicillin 1 gr / 6 hour / IV
Diet MB 2500 kkal with 100 gr Protein

Echocardiography
-Overiding aorta
-Dilated RA-RV
-Pulmonary Stenosis
-Right aortic arch
-No Pericardial Effusion
-Well contracting Ventricle
-VSD
-Right to Left Shunt
Coclusion :
-TOF

Electrocardiography

: Sinus Tachycardia, QRS Rate: 103 x/m, Axis: RAD, P Wave : P

Mitral (RAE +) , PR Interval : 0,16s, QRS duration :0,08s, QRS Changes : -, ST-T Changes;
-, RVH : + (R>S in V1, Persistent S wave in V5, V6), LVH: -, VES: -.
Conclusion : Sinus Tachycardia, Right Axis Deviation, Right Atrial Enlargement, Right
Ventricle Hiperthropy

08 JUNE 2015
S

S: Dyspneu (+) , Oedema (-), fever (-)

O : Sensorium : alert, T:370C

Head :
Eye: light reflex (+/+), isochoric pupil, superior conjunctiva palpebra edema (-/-), pale inferior
conjunctiva palpebra (-/-)
Ear: within normal range
Nose: within normal range
Mouth: cyanosis
Neck: lymph nodes enlargement (-)
Thorax: symmetrical fusiform, retraction (-)
HR: 104 bpm, reg, ejection sistolic murmur (+) grade III/6 mid clavicularis line ICR II/III
RR: 27 bpm, reg, rales (-/-)
Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpable
Extremities: pulse 110 bpm, reg, p/v adequate, warm acral, CRT < 3

Tetralogy of Fallot

O2 nasal kanul 1-2 l/i

Inj.Ampicillin 1 gr/6jam/iv (H4)
Diet MB 2000 kkal dengan 100 gr protein

09 JUNE 2015
S

Dyspneu (+) , fever (-)

Sensorium : alert, T:36,80C BB:52kg

Head :
Eye: light reflex (+/+), isochoric pupil, superior conjunctiva
palpebra edema (-/-), pale inferior conjunctiva palpebra (-/-)
Ear: within normal range
Nose: O2 Nasal Canul
Mouth: within normal range
Neck: lymph nodes enlargement (-)
Thorax: symmetrical fusiform, retraction (-)
HR: 100 bpm, reg, ejection sistolic murmur (+) grade III/6 mid
clavicularis line ICR II/III
RR: 24 bpm, reg, rales (-/-)
Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen
not palpable
Extremities: pulse 100 bpm, reg, p/v adequate, warm acral, CRT
< 3 110 bpm, reg, p/v adequate, warm acral, CRT
< 3

Tetralogy of Fallot

-Oxygen O2 Nasal Canul 1- 2 l/i

-Inj.Ampicilin 1gr/6jam/iv
-Diet MB 2000 kkal dengan 100 gr protein

10 JUNE 2015
S

Dyspneu (-) , fever (-)

Sensorium : alert, T:36,80C BB:52kg

Head :
Eye: light reflex (+/+), isochoric pupil, superior conjunctiva palpebra edema (-/-), pale inferior
conjunctiva palpebra (-/-)
Ear: within normal range
Nose: O2 Nasal Canul
Mouth: within normal range
Neck: lymph nodes enlargement (-)
Thorax: symmetrical fusiform, retraction (-)
HR: 100 bpm, reg, ejection sistolic murmur (+) grade III/6 mid clavicularis line ICR II/III
RR: 20 bpm, reg, rales (-/-)
Abdominal: soft, non tender, peristaltic (+) N, Liver and Spleen not palpable
Extremities: pulse 100 bpm, reg, p/v adequate, warm acral, CRT < 3 110 bpm, reg, p/v adequate, warm
acral, CRT
< 3

Tetralogy of Fallot

-Oxygen O2 Nasal Canul 1- 2 l/i

-Inj.Ampicillin 1gr/6jam/iv (H1)
-Diet MB 2000 kkal dengan 100 gr protein

Discussion
Theory

Case

The etilogy of Tetralogy Of

Fallot is multifactorial , but
reported associations include
untreated maternal diabetes ,
phenyketonuria, and intake of
retinoic acid.Associated
chromosomal anomalies can
include trisomies 21, 18,and
13 but resence experience
points to much frequent
association of microdeletions
of chromosome 22.The risk of
recurence in families is 3%

The patient has genetic risk

that is where both families
have a history of similar
disease.

Theory

Case

From the echocardiography result

for Tetralogy Fallot case there is
present with 4 features:
1.VSD
2.Pulmonary stenosis
3.Right to left shunt
4.Overiding aorta

1.Overiding aorta
2.Dilated RA-RV
3.Pulmonary Stenosis
4.Right aortic arch
5.MPCA (+)
6.No Pericardial Effusion
7.Well contracting Ventricle
8.VSD
9.Right to Left Shunt
Coclusion :
-TOF

Theory

Case

Cyanosis of the lips and nail bed

is usualy pronaounced at birth ,
after age 3-6months, the finger
and toes shows clubbing.
A sistolic thrill is usually present
anteriorly along the left sternal
border.

Cyanosis was found in the perifer.

Ejection sistolic murmur (+) grade III / 6
LMCS ICR II/III

Theory

Case

Chest x-rays showed generally

the size of the heart is
enlarged .Heart shape generally
will show a picture like boot
shaped and decreased pulmonary
vascularity.

The results of chest x-ray shows

the size of the heart is enlarged
by the CTR of
> 50% and
found a picture like boot shaped

SS,16

years old girl , with 52 kg came to

emergency unit in Haji Adam Malik General
Hospital Medan on 5th June 2015 at 19.30 WIB.
Chief of complain was dyspnea .Dyspnea was
experienced by SS past 4 days before.
Complain of dyspnea was irrelevant with
change in weather and daily activities.
According to anamnesis , physical
examination and echocardiography
assesment he was diagnosed with Tetralogy
of Fallot .She was treated with Oxygen O2 Nasal
Canul 1- 2 l/I Inj , Ampicillin 1gr/6jam/iv , Diet MB
2000 kkal dengan 100 gr protein