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MALIGNANT HYPERTHERMIA

MALIGNANT HYPERTHERMIA. Dr. Mary Lehane Malignant Hyperthermia Investigation Unit Cork University Hospital. INCIDENCE. 1:12 000 - 1:40 000 Male = Female No racial difference. MORTALITY. 5 % - 80 %. TRIGGERS. All volatile anaesthetic agents Suxamethonium. GENETICS.

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MALIGNANT HYPERTHERMIA

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  1. MALIGNANT HYPERTHERMIA Dr. Mary Lehane Malignant Hyperthermia Investigation Unit Cork University Hospital

  2. INCIDENCE • 1:12 000 - 1:40 000 • Male = Female • No racial difference

  3. MORTALITY 5 % - 80 %

  4. TRIGGERS • All volatile anaesthetic agents • Suxamethonium

  5. GENETICS • Autosomal dominant • Chromosome 19 • Gene RYR 1 • Mutations • 78 single point mutations identified to date

  6. PRESENTATION • 1 A known MH patient • 2 Unexpected MH crisis

  7. FULMINANT CRISIS • Tachycardia • Metabolic acidosis,  O2 sat,  pCO2 • Muscle rigidity • Electrolyte disturbance • Arrhythmias • Myoglobinuria • Hyperthermia

  8. DIAGNOSIS, consider MH if • Masseter muscle spasm after sux • Unexplained, unexpected tachycardia • Unexplained, unexpected increase in end - tidal CO2

  9. EARLY MANAGEMENT 1 • STOP ALL ANAESTHETIC VAPOURS • CHANGE TO CLEAN ANAESTHETIC BREATHING SYSTEM • ABANDON SURGERY IF FEASABLE

  10. EARLY MANAGEMENT 2 • DANTROLENE • MEASURE ABGs, K+ AND CK • MEASURE CORE TEMP • COOL PATIENT

  11. OTHER COMPLICATIONS • Arrhythmias • Hyperkalaemia • Metabolic Acidosis • Disseminated Intravascular Coagulopathy • Renal Failure

  12. POST CRISIS MANAGEMENT • WARN PATIENT AND FAMILY • REFER FOR INVESTIGATION • ie muscle biopsy • MEDIC ALERT

  13. INVESTIGATION • Family history • Muscle biopsy • In - vitro contracture tests • Histology • Resting CPK etc • Mutation screening

  14. KNOWN MH PATIENT • Inform anaesthetist and theatre • Prepare anaesthetic machine etc • All hospitals should carry dantrolene • All staff carry responsibility

  15. The Cork Experience • 560 Patients biopsied • MHS 131 • MHE (h) 100 • MHE (c) 6 • MHN 333

  16. The Cork Families • 98 Pedigrees identified • 74 Probands • 24 Deaths

  17. CONCLUSION • SURVIVAL • Identification of at-risk patients • Appropriate management

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