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Cyanotic Spell (Tetralogy of Fallot)
Cyanotic Spell (Tetralogy of Fallot)
Clinical features
- Acute worsening of central cyanosis in patients with a confirmed or suspected underlying cyanotic congenital heart disease such as Tetralogy of Fallot.
- Rapid worsening of central cyanosis, tachypnoea/dyspnoea
- Anxiety and alteration in consciousness
- Restless and crying in the presence of congenital cyanotic heart disease
- Decrease in intensity or disappearance of the systolic murmur in Tetralogy of Fallot
- Children are initially agitated, but may become floppy and can progress to loss of consciousness, seizures and death
Investigations
No investigations should be done during a spell - may cause agitation and aggravate the episode
Management
- Call for help!
- Console child, allow parents to hold on lap in knee chest position/be present
- Oxygen 100%, by rebreather facemask or by nasal cannula
- Place patient in knee-chest position to raise systemic blood pressure and increase systemic venous return (squatting)
- Monitor SaO2, heart rate, respiratory rate and acid-base status
- Fluid bolus 10 ml/kg of ringers lactate or normal saline(can repeat 10ml/kg bolus)
- Morphine 0.1-0.2 mg/kg IV for one dose
- Alternative: Chloral hydrate 50 mg/kg orally OR Midazolam 0,5 mg/kg PO or PR
- If clinically acidotic or pH < 7.2: Sodium bicarbonate 4.2 % (2 ml/kg IV)
- β-Blocker:
- Propranolol (Inderal)PO 0.5–1 mg/kg/dose 6 hourly
- Esmolol (first choice, but only available in tertiary centre) load with 500 mcg/kg, Maintenance: 50 mcg/kg/min infusion. If inadequate response, increase as necessary by 50 mcg/kg every 1–4 minutes to a maximum of 300 mcg/kg/min.
- If no response, intubate and ventilate
After resolution of spell:
- If Hb <10 g/dL, child is anaemic: Packed red cells, 10 mL/kg over 4 hours
- Propranolol (Inderal)PO 0.5–1 mg/kg/dose 6 hourly. Increase to a maximum of 5 mg/kg/day as required.