Hyper-cyanotic Spells

General
Airway
Breathing
Cardiovascular
Disability
Don't Ever Forget Glucose
Exposure and electrolytes

Hyper-cyanotic spells occur when the RV outflow track goes into spasm resulting in little or no blood flow to the lungs and a R to L shunt via VSD. 

• SIGNS: Severe cyanosis, metabolic acidosis, acidotic breathing. worsening distress, syncope, going floppy and eventually cardiac arrest
• Often triggered by intercurrent illness
• Most common in Tetralogy of Fallot, but also occur with Pulmonary Atresia and Tricuspid Atresia.

General

• Always keep the mother with the child & keep the child as calm as possible
• Leave the child in mothers arms/lap if possible
• Limit painful procedures and crying at all cost (use sedation if needed: see Disability section below for options)
• Maintain knee chest position
• Continuous saturation monitoring

Airway

• Intubate if signs of imminent cardiorespiratory failure
• Limit decrease in BP with induction agents by using more cardio stable induction drugs:
  • Ketamine 2mg/kg IV slowly or 2-5mg/kg IM
    

Breathing

• High flow O2 – give as near to 100% FiO₂ as possible
  • Ideally via paediatric facemask with non-rebreather reservoir 10-15L/min
• If intubated:
  • Use 100% FiO₂,
  • Low ventilation pressures but ensure adequate chest movement

Cardiovascular

• Increase systemic BP to reduce R to L shunt across VSD
  • Fluid 20ml/kg IV crystalloid bolus – 3-4x until liver size begins to increase
  • Ketamine 1-2mg/kg bolus IV 
  • Dopamine 5-15mcg/kg/min 
    • (Mix 6mg/kg Dopamine in 50ml 5%DW: 1ml/h =2mcg/kg/min)
• HR to improve ventricular filling time
  • Propranalol 0.5-1mg/kg stat PO & 8 hourly
  • Esmolol 0.5mg/kg IV bolus or infusion
    • (Mix Esmolol 60mg/kg in 50ml 5% DW: 1.2ml/h = 25mcg/kg/min) 
    • (Run 1.2 to 2.4ml/h)
• Further management:
  • Track intensity of Ejection Systolic Murmur at L sternal border. 
    • Softer murmur = less flow through PS = worsening spell, poor prognosis
    • Louder murmur = more flow through PS = spell improving
  • If thoracic scars, suspect BT shunt procedure. Identify continuous shunt flow murmur below clavicles. If unable to hear - suspect shunt 

Disability

Keep baby calm at all costs, if necessary sedate 

• Chloral hydrate 50mg/kg PO
• Morphine 0.1mg/kg IV slowly (may BP)
• Ketamine bolus 1-2mg/kg IV slowly (CVS stable) or infusion if intubated 
• Ketamine 30mg/kg in 50ml 5%DW 1ml/h=10mcg/kg/min. Run 1-4ml/h
  

Don’t Ever Forget Glucose.

• Treat glucose aggressively with 5ml/kg 10% DW. Recheck glucose.

Exposure & Electrolytes:

• 2ml/kg NaHCO₃ IV slow bolus to reverse acidosis. Dilute to 4.2%. Can repeat
• Treat temp spikes aggressively. Paracetamol 15mg/kg q 6 h PO/PR & cooling. 
• Temp spikes vasodilate patient and BP (worsening R to L shunt)
• Start cefotaxime/ceftriaxone asap 50mg/kg IV if infection suspected
• Maintenance fluid 0.45% saline and 5% DW, keep NPO
• Arrange transfer to tertiary hospital, intubate for transfer