Related Subjects:
Acute Kidney Injury
|Acute Rhabdomyolysis
|Hyperkalaemia
|Neuroleptic Malignant Syndrome
|Malignant Hyperpyrexia (Malignant Hyperthermia)
|Serotonin syndrome
|Cholinergic crisis-syndrome
|Anticholinergic syndrome
📉 Advances in understanding and treatment have reduced MH mortality from >80% thirty years ago to <5% today.
📖 About
- Triggered by certain anaesthetic drugs in genetically susceptible patients.
- Previous uneventful anaesthesia with triggers does not exclude MH risk.
- Characterised by: hypermetabolism, acidosis, rhabdomyolysis, and hyperkalaemia.
- 🚨 Invariably fatal if untreated.
🧬 Aetiology
- Autosomal dominant predisposition (~1 in 20,000).
- Commonly due to Ryanodine receptor (RYR1) mutation on chromosome 19q13.
- Excess intracellular calcium → sustained muscle contraction & breakdown.
⚙️ Pathogenesis
Uncontrolled calcium release from sarcoplasmic reticulum → hypermetabolic crisis with ↑ O₂ demand, acidosis, and muscle breakdown.
💉 Causes (Triggers)
- Inhalational anaesthetics: Halothane, Sevoflurane, Desflurane.
- Depolarising muscle relaxant: Succinylcholine.
- Rare: Vigorous exercise or extreme heat.
🩺 Clinical Features
- ⬆️ End-tidal CO₂ (ETCO₂) – one of the earliest signs.
- Generalised muscle rigidity (esp. trunk).
- Masseter spasm (early warning sign).
- Hyperthermia >40°C 🌡️, tachycardia, dark urine (myoglobinuria).
- Rapid progression → arrhythmias, cardiac arrest if untreated.
🔍 Differential Diagnosis
- Light anaesthesia.
- Hypoventilation.
- Thyroid storm.
- Overheating.
- Phaeochromocytoma.
🧪 Investigations
- Bloods: U&E (renal function), ↑ CK, ↑ K⁺.
- ABG: Mixed metabolic + respiratory acidosis, ↑ lactate.
- ECG: Monitor for arrhythmias and hyperkalaemia changes.
- Urine: Myoglobinuria (dark red/brown urine).
⚠️ Complications
- Acute Kidney Injury (AKI) 🚰.
- Cardiac arrhythmias → arrest ❤️.
- Severe hyperkalaemia ⚡.
- Disseminated Intravascular Coagulation (DIC).
🚑 Management (Do NOT use Calcium Channel Blockers)
- ABC resuscitation: MH is fatal if not rapidly treated. Call for help immediately.
- Stop triggers: Discontinue volatile agents & succinylcholine. Switch to IV anaesthetics (e.g. propofol).
- 100% Oxygen: Hyperventilate with O₂ ≥10 L/min using clean circuit or Ambu bag.
- Dantrolene: 2–3 mg/kg IV bolus, then 1 mg/kg as needed (max 10 mg/kg). ⚠️ Risk of extravasation injury.
- Cooling: Cool IV fluids, ice packs, cold lavage if needed. Antipyretics ineffective.
- Correct acidosis/hyperkalaemia: IV bicarbonate (if pH <7.2), insulin/dextrose, calcium gluconate if ECG changes.
- ICU admission: Central line, urinary catheter, monitor for AKI, rhabdomyolysis, arrhythmias.
📚 References