Ms. Lamia, a 10-year old female from Potuakhali, Barisal, presented with breathlessness for 2-3 weeks and bluish discoloration of her skin since childhood, which worsens with exertion. Examination found central cyanosis, clubbing, and a grade 3/6 ejection systolic murmur, suggesting congenital cyanotic heart disease, most likely Tetralogy of Fallot. Medical management and potential surgical options were discussed.

1. Department of Cardiology
dr. md. Abu faisal
D-card student
DMCH

2.  Name - Ms. Lamia
 Age -10 years
 Sex -Female
 Occupation - Student
 Religion - Islam
 Address – Potuakhali,Barisal
 Date of admission - 13/11/2017 through OPD
 Date of Examination - 14/11/2017
Cardiology DMCH

3.  Severe Breathlessness for 2-3weeks
 Bluish discoloration of tongue,lips,fingers & toes
during exertion since childhood
Cardiology DMCH

4. According to the statement of patient’s attendant she has been
suffering from shortness of breath during exertion since her
childhood. Initially it was on moderate to severe activity. But it
is progressively increased, Now it occurs on mild exertion for
last few weeks. She felt comfortable at rest and on lying flat and
there was no H/O sudden awakening from sleep at mid night
due to breathlessness. Breathlessness occurs during playing
and relieved on squatting position.
Breathlessness is not associated with cough, chest pain, fever,
coughing out of blood, and there is no seasonal or diurnal
variation, wheeze, h/o allergy or swelling of any part of the
body. It does not aggravate on exposure to cold, dust, fumes
or any allergens.
Her mother give H/O of bluish discoloration of skin, fingers,
nails, toes and lips since her childhood, which is more marked
during exertion. It persistent and gradually progressive
becoming more marked on exertion and not associated with
change in temperature or exposure to cold.
Cardiology DMCH

5.  On query she mentioned fatigue during competitive
activities during childhood.
 On repeated query her mother mentioned that the
patient used to become bluish and breathlessness
while feeding or crying during her childhood.
 But there was no h/o palpitation dizziness, sudden
transient loss of consciousness, headache ,
blurring of vision, nausea, vomiting, bleeding from
any site of the body, fever, joint pain or swelling,
repeated RTI, weakness of any part of the body. But
she has stunted growth.
 Her birth history was uneventful and her growth
were delayed.
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6.  Not significant
 Pt is non diabetic and normotensive
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7.  No such illness runs in her family
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8.  She belongs to a below standard socio-economic
family.
 She lives in pucca house, drinks arsenic-free
tubewell water and uses sanitary latrine.
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9.  Her menstruation has not started yet
 Immunization History
 She was immunized as per EPI schedule
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10.  Appearance: The patient is malnourished and short
statured.
 Co-operation: cooperative
 Decubitus: on choice
 Conjunctiva: Suffused
 Jaundice: absent
 Oedema:absent
 Cyanosis:Central cyanosis
 There is generalized clubbing(Involving all fingers &
toes)
 Koilonychias, leukonychia, lymphadenopathy : absent
 Thyroid gland not palpable.
Cardiology DMCH

11.  Pulse: 104beats/min, regular in rhythm, no radio
radial and radio femoral delay
 BP: 100/70 mm of Hg on both upper limbs
 Respiratory rate: 29 breaths/min
 Temp: 99 degree Fahrenheit
 JVP: Not raised
Cardiology DMCH

12.  Pulse:
 104beats/min, normal volume, character, regular in rhythm
 No radio radial and radio femoral delay
 Condition of the vessel wall is normal
 All the peripheral pulses are normal
 Pericardium :
 Inspection:
 No visible apical impulse
 No epigastric pulsation.
 No scar mark or bony deformity.
Cardiology DMCH

13.  Palpation:-
 Apex beat is located in left 5th intercostal space, 7cm from
midsternal line, just medial to midclavicular line, normal in
nature
 No Left parasternal heave
 No Thrill
 No palpable pulmonary component
 No epigastric pulsation
Cardiology DMCH

14.  First heart sound is normal in all areas
 Second heart sound is single
 There is an ejection systolic murmur in the
pulmonary area(left 2nd & 3rd intercostals space)
increasing intensity with breath hold in inspiration.
 Grading of the murmur is 3/6
 There is no added sound
 Lung base are clear
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15.  Reveals no abnormality.
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16.  The pt Ms. Lamia, 10-years-old, hailing from
Potuakhali, Barisal got admitted into DMCH
through outpatient department on 13/11/2017
with the complaints of breathlessness for 2-3
weeks ,bluish discoloration of tongue, lips, fingers
and toes. According to the statement of patient’s
attendant her breathlessness was less marked in
early age, only felt during moderate to severe
activity. But it is progressively increasing, now it
occurs with mild exertion for last few weeks and
she felt comfortable at rest and on lying flat and
there was no h/o awakening from sleep at mid
night due to breathlessness.
Cardiology DMCH

17. Breathlessness is not associated with cough, chest
pain, fever, coughing out of blood, diurnal and
seasonal variation, wheeze. On query her mother
also noticed bluish discoloration skin, fingers,
nails, toes and lips since childhood, which is
marked more during exertion and less by taking
squatting position. Her mother mentioned that
the patient used to become bluish and
breathlessness occurs more on feeding &
crying. There was no h/o fever, joint pain or
swelling, headache, palpitation and dizziness
Cardiology DMCH

18. On general examination, the patient is ill looking and
malnourished, central cyanosis(involving
tongue,lips,fingers and toes),clubbing(involving all
fingers and toes),
Examination of cardiovascular system, there is
no visible cardiac impulse, apex beat is
located in left fifth intercostal space just
medial to the midclavicular line, and there is
no thrill and no left parasternal heave. on
auscultation first heart sound is normal in all
area, second heart sound is usually single.
Cardiology DMCH

19. There is an ejection systolic murmur in the
pulmonary area(left 2nd & 3rd intercostal
space) increasing intensity with breath hold in
inspiration. Grade 3/6,there is no added
sound, Lung base are clear.
Cardiology DMCH

20. Congenital cyanotic heart disease, most likely
Tetralogy of fallot
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21. 1. DORV with Severe Pulmonary Stenosis
2. VSD and PS
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22.  CBC:
Hb : 18.9 gm/dl
TWBC : 7000/cumm
Neu : 47 %
Lym : 42%
ESR : 10 mm in 1st hour
HCT : 66%
Cardiology DMCH

23. Cardiology DMCH

24. Cardiology DMCH

25. Cardiology DMCH

26.  Cardiac catheterization
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27. Tetralogy of Fallot
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28.  Medical management
 Interventional management
 Surgical management
Cardiology DMCH

29.  Immediate positional change(knee-chest
position or squatting position)
 High flow oxygen inhalation
 Sedation-preferably Injection Morphine
sulphate(0.1 mg/kg)
 Sodium bicarbonate-to combat acidosis
 Volume expansion with IV fluid
 Beta blocker(Propranolol) –oral route or in
emergency IV(0.5 to 1.5 mg/kg every 6
hourly)
Cardiology DMCH

30. Palliative surgery:
 Children can undergo surgery at an early age.
 Most common operation was the Blalock-
Taussing shunt(subvlavian artery to
pulmonary artery graft anastomosis)
 Waterston shunt(left PA to ascending aorta)
 Pot’s anastomosis(left PA to descending
aorta.
Cardiology DMCH

31.  Complete corrective procedures in Tetralogy
of Fallot involve VSD closure, incorporating
the aorta into the LV and relieving RV outflow
tract obstruction. This usually involves
resecting the hypertrophied infundibular
myocardium, with patch enlargement of the
outflow tract.
Cardiology DMCH

32. Cardiology DMCH