MALIGNANT HYPERTHERMIA

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MALIGNANT HYPERTHERMIA

• Dr. Mary Lehane
• Malignant Hyperthermia Investigation Unit
• Cork University Hospital

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INCIDENCE

• 112 000 - 140 000
• Male Female
• No racial difference

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MORTALITY

• 5 - 80

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TRIGGERS

• All volatile anaesthetic agents
• Suxamethonium

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GENETICS

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

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PRESENTATION

• 1 A known MH patient
• 2 Unexpected MH crisis

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FULMINANT CRISIS

• Tachycardia
• Metabolic acidosis, ? O2 sat, ? pCO2
• Muscle rigidity
• Electrolyte disturbance
• Arrhythmias
• Myoglobinuria
• Hyperthermia

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DIAGNOSIS, consider MH if

• Masseter muscle spasm after sux
• Unexplained, unexpected tachycardia
• Unexplained, unexpected increase in end - tidal
  CO2

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EARLY MANAGEMENT 1

• STOP ALL ANAESTHETIC VAPOURS
• CHANGE TO CLEAN ANAESTHETIC BREATHING SYSTEM
• ABANDON SURGERY IF FEASABLE

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EARLY MANAGEMENT 2

• DANTROLENE
• MEASURE ABGs, K AND CK
• MEASURE CORE TEMP
• COOL PATIENT

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OTHER COMPLICATIONS

• Arrhythmias
• Hyperkalaemia
• Metabolic Acidosis
• Disseminated Intravascular Coagulopathy
• Renal Failure

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POST CRISIS MANAGEMENT

• WARN PATIENT AND FAMILY
• REFER FOR INVESTIGATION
• ie muscle biopsy
• MEDIC ALERT

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INVESTIGATION

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

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KNOWN MH PATIENT

• Inform anaesthetist and theatre
• Prepare anaesthetic machine etc
• All hospitals should carry dantrolene
• All staff carry responsibility

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The Cork Experience

• 560 Patients biopsied
• MHS 131
• MHE (h) 100
• MHE (c) 6
• MHN 333

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The Cork Families

• 98 Pedigrees identified
• 74 Probands
• 24 Deaths

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CONCLUSION

• SURVIVAL
• Identification of at-risk patients
• Appropriate management

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