Highlights & Basics
- Malignant hyperthermia is a potentially lethal syndrome usually triggered by inhalation anesthetics or succinylcholine.
- The underlying genetic susceptibility is most often due to a range of autosomal dominant mutations in RYR1.
- Caused by an increase in metabolic rate driven by an increase in intracellular calcium levels in muscle.
- Presents with increased carbon dioxide production, muscle rigidity, tachycardia, hyperthermia, and mixed metabolic and respiratory acidosis.
- A definitive diagnosis requires testing after the acute episode has resolved. Genetic testing may be the first test performed in selected patients; it may form part of the autopsy if MH is suspected as the cause of death. Muscle contracture testing can be used to exclude susceptibility to MH.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Diagnostic pathway for investigation of MH susceptibility. IVCT, in vitro contracture test; MH, malignant hyperthermia; MHN (MH negative or normal), classification applied when all contracture tests are negative; MHShc, MHSh, and MHSc, classifications are applied when contracture responses to both halothane and caffeine are abnormal, response to halothane alone is abnormal, or response to caffeine alone is abnormal, respectively. *Patients who should be asked to take part in research studies of the genetic basis of malignant hyperthermia
The mechanical response of normal and MH-susceptible muscle (positive) to direct stimulation in the presence of 3% halothane
The mechanical response of normal and MH-susceptible muscle (positive) to direct stimulation in the presence of incremental administration of caffeine
Citations
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Larach MG, Dirksen SJ, Belani KG, et al; Society for Ambulatory Anesthesiology; Malignant Hyperthermia Association of the United States; Ambulatory Surgery Foundation; Society for Academic Emergency Medicine; National Association of Emergency Medical Technicians. Special article: creation of a guide for the transfer of care of the malignant hyperthermia patient from ambulatory surgery centers to receiving hospital facilities. Anesth Analg. 2012 Jan;114(1):94-100.[Abstract][Full Text]
Larach MG, Localio AR, Allen GC, et al. A clinical grading scale to predict MH susceptibility. Anesthesiology. 1994 Apr;80(4):771-9.[Abstract]
Hopkins PM, Rüffert H, Snoeck MM, et al. European Malignant Hyperthermia Group guidelines for investigation of malignant hyperthermia susceptibility. Br J Anaesth. 2015 Oct;115(4):531-9.[Abstract][Full Text]
Larach MG, Dirksen SJ, Belani KG, et al; Society for Ambulatory Anesthesiology; Malignant Hyperthermia Association of the United States; Ambulatory Surgery Foundation; Society for Academic Emergency Medicine; National Association of Emergency Medical Technicians. Special article: creation of a guide for the transfer of care of the malignant hyperthermia patient from ambulatory surgery centers to receiving hospital facilities. Anesth Analg. 2012 Jan;114(1):94-100.[Abstract][Full Text]
Rüffert H, Bastian B, Bendixen D, et al. Consensus guidelines on perioperative management of malignant hyperthermia suspected or susceptible patients from the European Malignant Hyperthermia Group. Br J Anaesth. 2021 Jan;126(1):120-30.[Abstract][Full Text]
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100. Rüffert H, Bastian B, Bendixen D, et al. Consensus guidelines on perioperative management of malignant hyperthermia suspected or susceptible patients from the European Malignant Hyperthermia Group. Br J Anaesth. 2021 Jan;126(1):120-30.[Abstract][Full Text]
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