1. CYANOTIC SPELLS

2. CYANOTIC SPELLS
paroxysmal hyperpnea
hypoxic spells
anoxic spells
blue spells

3. CYANOTIC SPELLS
rate & depth of respiration
increasing cyanosis
Limpness , syncope, convulsions, CVA
DEATH

4. CYANOTIC SPELLS -- CLINICAL PROFILE
Morgan et al - Circulation 1965 :31;66-69
190 pts requiring shunt
38% had spells % TOF & 20% tricuspid atresia
Age -- onset months – 1 year
Time of day -- morning hours
Duration min
Ppting factors -- awakening,crying , defecation , feeding,
unpleasant stimuli eg. needle prick

5. CYANOTIC SPELLS -- CLINICAL PROFILE
not always correlate with severity of TOF
infrequent in high altitude natives
due to diminished sensitivity of
respiratory centre to hypoxia
frequency decreases after morning hours
as respiratory centre gets adapted

8. CYANOTIC SPELLS -physiology
HYPERVENTILATION is the main factor
Normal – sleep is attended by reduction in ventilation
 PaCO2 ,  PaO2,SaO2,pH
On waking up reverse occurs

9. CYANOTIC SPELLS -physiology
TOF
Sleep - reduced O2 demend --- better SaO2
Waking up ---  ventilation,  activity
 PaCO2,  SaO2, PaO2,pH

10. CYANOTIC SPELLS -physiology
Effect of hyperventilation
Normal --  SaO2 , PaO2 & pH
 PaCO2
TOF consistent fall in SaO2
Hyperventilation  venous return to RV
 R L shunt PCO2 , PaO2, pH,Sao2
Effect

11. CYANOTIC SPELLS -physiology
TOF
High pulm . resistance
RVOT resistance( high
and fixed )
vasc . Resistance( low )
Pulm.resistance > systemic
Systemic vascular resistance is dynamic
Any change in SVR modifies the right to left shunt

12. CYANOTIC SPELLS -physiology
Hyperpnea
 venous return
to RV with fixed
RVOT resistance
PCO2 ,
PaO2, pH,Sao2 a 
 SVR
 R L shunt

13. CYANOTIC SPELLS -physiology
Hyperpnea
increased activity  O2 requirement PCO2 ,
PaO2, pH,Sao2
? RVOT “ spasm ”
X spells seen in pulmonary atresia also
Tachycardia increased RVOT contractility
increased C.O.

14. X - CYANOTIC SPELLS -TREATMENT Hyperpnea  venous return a  SVR
to RV with fixed
RVOT resistance
PCO2 ,
PaO2, pH,Sao2 a 
 SVR
 R L shunt

15. X CYANOTIC SPELLS -TREATMENT Hyperpnea  venous return a X  SVR
to RV with fixed
RVOT resistance
PCO2 ,
PaO2, pH,Sao2 a  X
 SVR X
 R L shunt

16. x CYANOTIC SPELLS -TREATMENT Hyperpnea  venous return a  SVR
to RV with fixed
RVOT resistance
PCO2 ,
PaO2, pH,Sao2 a  x
 SVR
 R L shunt

17. CYANOTIC SPELLS -TREATMENT
knee chest position
O2 inhalation
morphine
propranolol
alpha stimulants
sodabicarb
general anesthesia
emergency shunt in recurrent spells

18. X - X MORPHINE Hyperpnea  venous return a  SVR  R L shunt
CYANOTIC SPELLS -TREATMENT
MORPHINE
X -
Hyperpnea
 venous return
to RV with fixed
RVOT resistance
PCO2 ,
PaO2, pH,Sao2 a 
 SVR X
 R L shunt

19.  intra abdominal pressure
CYANOTIC SPELLS -TREATMENT
Knee – chest position
 intra abdominal pressure
 SVR reduced R > L shunt
 splanchnic venous return – high
O2 saturation
kinking of femoral veins reduces venous return
lower limbs( low O2 saturation)

20. reduces HR, RVOT contractility increases SVR
CYANOTIC SPELLS -TREATMENT
PROPRANOLOL
0.2 mg/kg IV
reduces HR, RVOT contractility
increases SVR
More useful in those with
little cyanosis at rest
marked reduction in SaO2 with activity /
hyperventilation

21. CYANOTIC SPELLS
PARADOX
RECURRENT SPELLS AT HOME –
GET SPONTANEOUSLY ABORTED
SPELLS IN HOSPITALS —
LAND UP IN ANESTHESIA

22. THANK YOU