CYANOTIC SPELLS paroxysmal hyperpnea hypoxic spells anoxic 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 Pa. CO 2 , Pa. O 2, Sa. O 2, p. H On waking up reverse occurs

CYANOTIC SPELLS -physiology TOF Sleep - reduced O 2 demend --- better Sa. O 2 Waking up --- ventilation, activity Pa. CO 2, Sa. O 2, Pa. O 2, p. H

CYANOTIC SPELLS -physiology Effect of hyperventilation E f f Normal -- Sa. O 2 , Pa. O 2 & p. H e Pa. CO 2 c TOF t -consistent fall in Sa. O 2 Hyperventilation R L shunt venous return to RV PCO 2 , Pa. O 2, p. H, Sao 2

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 PCO 2 , a p. H, Sao 2 Pa. O 2, R SVR L shunt venous return to RV with fixed RVOT resistance

CYANOTIC SPELLS -physiology • Hyperpnea • increased activity O 2 requirement PCO 2 , Pa. O 2, p. H, Sao 2 • ? RVOT “ spasm ” X spells seen in pulmonary atresia also • Tachycardia --- increased RVOT contractility increased C. O.

CYANOTIC SPELLS -TREATMENT X- Hyperpnea PCO 2 , a p. H, Sao 2 Pa. O 2, R SVR L shunt venous return to RV with fixed RVOT resistance

CYANOTIC SPELLS -TREATMENT Hyperpnea PCO 2 , a p. H, Sao 2 Pa. O 2, SVR X venous return to RV with fixed RVOT resistance X R L shunt

CYANOTIC SPELLS -TREATMENT Hyperpnea PCO 2 , a p. H, Sao 2 Pa. O 2, R SVR x L shunt venous return to RV with fixed RVOT resistance

CYANOTIC SPELLS -TREATMENT • knee chest position • O 2 inhalation • morphine • propranolol • alpha stimulants • sodabicarb • general anesthesia • emergency shunt in recurrent spells

CYANOTIC SPELLS -TREATMENT MORPHINE X- Hyperpnea PCO 2 , a p. H, Sao 2 Pa. O 2, SVR venous return to RV with fixed RVOT resistance X R L shunt

CYANOTIC SPELLS -TREATMENT Knee – chest position • intra abdominal pressure SVR reduced R > L shunt splanchnic venous return – high O 2 saturation • kinking of femoral veins reduces venous return lower limbs( low O 2 saturation)

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 Sa. O 2 with activity / hyperventilation

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