Congenital Cyanotic Heart Disease & TOF

1. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of FallotCYANOTIC CONGENITAL HEART
DISEASE
Dr. D. Gunasekaran
Consultant Paediatrician

2. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What is the criteria used to identify Congenital
Heart diseases?

3. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
NADAS CRITERIA
MAJOR CRITERIA MINOR CRITERIA
SYSTOLIC MURMUR Grade
3 OR MORE IN INTENSITY
SYS. MURMUR Grade 2 or
less IN INTENSITY
DIASTOLIC MURMUR Abnormal SECOND SOUND
CYANOSIS Abnormal ECG
CHF Abnormal CxR
Abnormal BP
DIAGNOSIS OF CHD

4. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
How do we broadly classify congenital Heart
diseases?
How do you further classify Cyanotic
congenital heart diseases?

5. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Congenital heart disease
Cyanotic CHD Acyanotic CHD
PBF PBF
Volume Pressure
PS
AS
CoA
ASD
VSD
AVSD
PDA
TOF
Pulmonary atresia
Tricuspid atresia
Ebstein anomaly
TGA
TAPVC
Truncus arteriosus
Single ventricle

6. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What is the most common CCHD?
a) TOF
b) Truncus arteriosus
c) TGA
d) TAPVC

7. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
TOF

8. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
TOF
What are the four components of TOF?
1. RV Outlet obstruction
2. Non restrictive VSD
3. Right ventricular hypertrophy
4. Right sided aortic arch
(25%)

9. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot

10. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot

11. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Blood from SVC & IVC
Major amount goes to
LV (No murmur)
Oxygenated blood
In Pulmonary veins
Small amount
Goes to PA (turbulent blood flow ESM)
P2 soft or absent)
Aorta
Systemic circulation
Right atrium
Right ventricle
Oxygenated blood
In Left atrium

12. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What is the most common CCHD,
manifesting after 2 months of life?
a) TOF
b) Truncus arteriosus
c) TGA
d) TAPVC

13. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What is the usual time of appearance of cyanosis in
TOF?
a) At birth
b) 8-12 weeks
c) 5-6 months
d) 1 year
What are the reasons?

14. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What is the usual time of appearance of cyanosis in TOF?
8-12 weeks ; Reasons:
1)Foetal Hb starts declining after birth
2)Obstruction to PBF starts increasing after birth
3)O2 req. increases as child grows (activity increases)
4)Ductus closure is delayed.
FOOD

15. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What are the symptoms of TOF?
Bluish extremities
Exertional breathlessness
Exercise intolerance
Feeding difficulties
Failure to thrive
But, the most common symptom is
Cyanotic spell (often with squatting)

16. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Cyanotic spell
What is the cyanotic spell?:
Exaggeration of existing cyanosis
with or without loss of consciousness and /or
seizures.
Usual time of appearance:
2-6 months of age

17. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Sequence of events during a spell
Gotting up from sleep 
Strains 
Becomes more cyanosed & more breathless

18. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Pathophysiology during Spell
Got up from sleep (vulnerable resp. centre)
Strain (Sympathetic over activity & excess Catecholamine release)
Ionotropic & Chronotropic & Spasm of Infundibulum (RVO part)
More blood pumped out of heart & less blood going in to pulmonary system
Increased venous return & increased RL shunt
More cyanosis
Increased hypoxia & acidosis
More Respiratory Centre stimulation
Hyperpnoea, which again stimutes Resp. centreHyperpnoea which again stimulates respiratory centre

19. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Squatting
What is squatting?
Child who gets spells repeatedly, slowly learns to squat - sits as if
he / she is sitting on the Indian toilet;
By squatting, cyanosis decreases. How?
1. Compression of major veins in LL 
de-oxygenated blood returning to the heart decreases
2. Compression of major arteries in LL
Systemic vascular resistance increases
Pulmonary blood flow increases
3. As a result of 1 & 2, Cyanosis decreases

20. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot

21. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Squatting
What are the Squatting equivalents?
Sitting with legs drawn underneath
Legs crossed while standing / sitting

22. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Vitals in TOF
Pulse: Volume? Rate? Character? Rhythm?
Blood pressure?

23. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
General examination
Undernourished (M, K & Vit def)
Suffused conjunctiva
Central cyanosis
Clubbing
Always look for dental hygiene – Why?

24. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Examination of heart
Inspection:
No Precordial bulge

25. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Examination of heart
Palpation:
Mild para sternal heave
(due to RVH)
No thrills

26. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Examination of heart
Auscultation:
Heart sounds:
S2 usually single (P2 soft or absent, depending on severity of PS)
Ejection click may be + (if prominent aortic root dilatation is +)
Murmurs:
ESM – in the Pulmonary area
(VSD remains silent)

27. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
What are the possible Complications
for any child with Heart disease?
FTT
Infective Endocarditis
CCF
PHT

28. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Complications
Spell (some times, with hypoxic seizures)
Failure to thrive
Cerebral thrombosis (<2 years of age)
Cerebral abscess (>2 years of age)
Infective Endocarditis – rare
CCF – Never occurs unless there are complications
like anemia or Infective endocarditis
PHT- Never occurs

29. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Investigations
What are the three basic investigations
necessary in any child with suspected
congenital heart disease?

30. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Investigations
CXR:
NO CUP

31. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Investigations
ECG
Chamber hypertrophy
ECHO
Anatomical diagnosis

32. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Management
Medical:
Anemia prophylaxis & correction
Proper nutrition
Dental Hygiene
Infective Endocarditis Prophylaxis
Management of spell & Prevention of spell

33. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Management of spell –
1.Knee-chest position
2. Oxygen
3. Morphine 0.1-0.2 mg/kg (reduces anxiety, releases infundibular spasm)
4. Sodium bicarbonate 7.5% soln. (1ml/kg + equal amount of D.W) given
slow IV
5. IV fluids
Prevention:
Propanolol (1mg/kg/day)
KOMSIP

34. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Management - Surgical
Good anatomy (of Pulmonary system and LA+):
Complete correction (4-6 months of age)
Poor anatomy:
Blalock-Taussig shunt: Subclavian artery  PA
(Increases the PBF oxygenation improves)
Total repair done at 2 years of age

35. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Different types of TOF
Fallot’s Triology----- PS+RVH+PFO
Pentalogy of TOF ----- TOF + ASD
Pink TOF (Acyanotic TOF) ---------- VSD + PS

36. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot
Points to remember
What is the most common CCHD?
It usually presents ----age with ---------------
Children with TOF learn to ----- during spells
Cardiomegaly?
Mild parasternal heave, single S2 & ESM at PA
CXR: NO CUP
Medical: Anemia, dental care, food, IE prophylaxis & KOMSIP
Surgery at 4-6 months, if anatomy is good
If anatomy is poor, BT shunt; surgery 2 yrs latter

37. Dr. D. Gunasekaran D. MD., Dept., of Paediatrics, MGMCRI
Cyanotic Congenital Heart Disease – Tetrology of Fallot