CYANOTIC SPELLS

CYANOTIC SPELLS paroxysmal hyperpnea hypoxic spells anoxic spells blue spells

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

CYANOTIC SPELLS -- CLINICAL PROFILE Morgan et al - Circulation 1965 :31;66-69 190 pts requiring shunt 38% had spells ---- 80 % TOF & 20% tricuspid atresia Age -- onset -- 2 months – 1 year Time of day -- morning hours Duration --- 15- 60 min Ppting factors -- awakening,crying , defecation , feeding, unpleasant stimuli eg. needle prick

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

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

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

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

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

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

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.

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

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

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

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

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

 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)

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

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

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