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Malignant hyperthermia

Malignant hyperthermia. Dr S Spijkerman. Pathogenesis. P harmacogenetic disorder A utosomal dominant inheritance Patients inherit a defected ryanodine 1 (RYR1) receptor.

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Malignant hyperthermia

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  1. Malignant hyperthermia Dr S Spijkerman

  2. Pathogenesis

  3. Pharmacogenetic disorder • Autosomal dominant inheritance • Patients inherit a defected ryanodine 1 (RYR1) receptor. • This receptor is responsible for regulating the calcium flow from the sarcoplasmic reticulum (SR) to the cytoplasm. • When a MH susceptible patient is exposed to a triggering anaesthetic agent (suxamethonium or inhalants), continuous activation of the Ryanodine 1 (RY1) receptor occurs, resulting in supraphysiological levels of sarcoplasmic reticulum calcium release with a compensatory increase in activity of the SR calcium re-uptake pump (an ATP dependent pump). • The increase in utilization of ATP stimulates metabolism, resulting in increased oxygen consumption, increased carbon dioxide production, thermogenesis and sympathetic stimulation (tachycardia and arrhythmias) • As ATP demand exceeds supply, muscle rigidity is seen with subsequent rhabdomyolysis, with release of potassium and myoglobin • This is followed by multi-organ failure and death.

  4. MH triggers N2O is safe

  5. Clinical presentation

  6. DDx

  7. Treatment

  8. Treatment (cont)

  9. Treatment (cont)

  10. Anaesthesia for MH sensitive patient

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