Cyanotic episodes occur in children with cyanotic congenital heart disease, in particular tetralogy of Fallot and pulmonary atresia. There may be a previous history of squatting. The episodes usually occur early in the morning, or in the context of stress or dehydration with periods of increased oxygen demand or increased oxygen use. The pathophysiology is not fully understood, but relates to decreased pulmonary blood flow. Most episodes are self-limiting but cause hypoxic-ischemic brain injury or indeed may be fatal.
Assessment
The assessment includes consideration of the severity of the cyanosis or pallor with either distress and hyperpnea (not tachypnea), or lethargy and a depressed conscious state.
There may be evidence of structural heart disease occasionally with lessening or absence of a previously documented heart murmur.
Treatment and Management
This can be divided into initial measures for short periods of cyanosis and measures for more prolonged problems.
Initial measures
• Put the child in knee to chest position
• Give high flow oxygen via mask or headbox
• Avoid exacerbating distress
• Give morphine 0.2 mg kg−1 s.c.
• Do continuous ECG and oxygen saturation monitoring and frequent blood pressure
measurements
• Correct any underlying cause/secondary problems, which may exacerbate episode, e.g.
cardiac arrhythmia, hypothermia, hypoglycemia.
If the attack is prolonged
• Consult a pediatrician or a cardiologist
• Give intravenous fluids 10 ml/kg bolus followed by maintenance fluids
• Give sodium bicarbonate 2–3 mmol/kg i.v. (ensure adequate ventilation)
• Consider admission
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