Cyanotic Congential Heart Disease With Hypoxaemia Attack/Spells (Hypercyanotic Spells)
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10 code: Q24.9 DESCRIPTION Acute worsening of central cyanosis in patients with a tetralogy of Fallot and certain other cyanotic heart diseases with pulmonary stenosis and a ventricular septal defect. DIAGNOSTIC CRITERIA Clinical Rapid worsening of central cyanosis, tachypnoea/dyspnoea, anxiety a
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Referral
ICD-10 code: Q24.9
DESCRIPTION
Acute worsening of central cyanosis in patients with a tetralogy of Fallot and certain other cyanotic heart diseases with pulmonary stenosis and a ventricular septal defect.
DIAGNOSTIC CRITERIA
Clinical
- Rapid worsening of central cyanosis, tachypnoea/dyspnoea, anxiety and alteration in consciousness in the presence of congenital cyanotic heart disease.
- Restless and crying in the presence of congenital cyanotic heart disease.
- Decrease in intensity or disappearance of the systolic murmur in Tetralogy of Fallot during crying.
GENERAL AND SUPPORTIVE MEASURES
- Exclude and treat precipitants such as fever or dehydration.
- Calm the patient and keep on mother’s lap, if possible.
- Place the patient in the knee-chest position to raise systemic blood pressure and increase systemic venous return.
- Monitor SaO2, heart rate, respiratory rate and acid-base status.
- Ensure adequate hydration.
MEDICINE TREATMENT
- Oxygen, 100%, by facemask or by nasal cannula
- Volume expander e.g. sodium chloride 0.9%, IV bolus, 20mL/kg administered over 5 minutes.
- Morphine, IV, 0.1–0.2 mg/kg as a single dose.
- May cause impairment of airway reflexes and respiratory depression.
If clinically acidotic or pH < 7.2:
- Sodium bicarbonate 4.2%, IV, 2 mL/kg.
If failure to improve the cyanotic spell, consider:
- Ketamine, IV, 0.5–1 mg/kg.
OR
- Phenylephrine (in consultation with a specialist).
After resolution of spell:
If Hb < 10 g/dL, the child is anaemic:
- Packed red cells, 10 mL/kg administered over 3 hours.
- Propranolol, oral, 0.5–1 mg/kg/dose 6 hourly.
- Do not exceed 5 mg/kg/day.
REFERRAL
- If above measures do not work refer urgently.
- Refer all cases for assessment.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter 4: Cardiovascular System , 4.2 Congenital Heart Disease, 4.2.1 Cyanotic Congential Heart Disease With Hypoxaemia Attack/Spells (Hypercyanotic Spells)