Abstract
Malignant hyperthermia is considered as a life-threatening hypermetabolic crisis resulting from exposure to volatile anesthetic agents and depolarizing muscle relaxants. The sequel of event followed by the exposure to these triggers results in massive release of the calcium ions in the skeletal muscle resulting in sustained muscle contraction. The symptoms include hyperventilation, tachycardia, tachypnea, hyperthermia to acidosis, hypercapnia, and even rhabdomyolysis. The introduction of dantrolene has resulted in significant improvement in mortality associated with MH. This chapter aims to discuss the genetic factors associated with MH, the typical clinical presentation along with the diagnostic criteria, and appropriate management protocol used in case of malignant hyperthermia.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Larach MG, Brandom BW, Allen GC, Gronert GA, Lehman EB. Cardiac Arrests and Deaths Associated with Malignant Hyperthermia in North America from 1987 to 2006: A Report from The North American Malignant Hyperthermia Registry of the Malignant Hyperthermia Association of the United States. Anesthesiology. 2008;108:603–11. https://doi.org/10.1097/ALN.0b013e318167aee2.
Halliday NJ. Malignant hyperthermia. J Craniofac Surg. 2003;14(5):800–2.
Ording H. Incidence of malignant hyperthermia in Denmark. Anesth Analg. 1985;64(7):700–4.
Riazi S, Larach MG, Hu C, Wijeysundera D, Massey C, Kraeva N. Malignant hyperthermia in Canada: characteristics of index anesthetics in 129 malignant hyperthermia susceptible probands. Anesth Analg. 2014;118(2):381–7. https://doi.org/10.1213/ANE.0b013e3182937d8b.
Brady JE, Sun LS, Rosenberg H, Li G. Prevalence of malignant hyperthermia due to anesthesia in New York State, 2001–2005. Anesth Analg. 2009;109(4):1162–6. https://doi.org/10.1213/ane.0b013e3181ac1548.
Strazis KP, Fox AW. Malignant hyperthermia: a review of published cases. Anesth Analg. 1993;77(2):297–304.
Voermans NC, Snoeck M, Jungbluth H. RYR1-related rhabdomyolysis: a common but probably underdiagnosed manifestation of defective skeletal muscle ryanodine receptor dysfunction. Rev Neurol. 2016;172:546–58. https://doi.org/10.1016/j.neurol.2016.07.018.
Carpenter D, Ringrose C, Leo V, Morris A, Robinson RL, Halsall PJ, et al. The role of CACNA1S in predisposition to malignant hyperthermia. BMC Med Genet. 2009;10:104. https://doi.org/10.1017/S0003480000008186.
Robinson RL, Curran JL, Ellis FR, Halsall PJ, Hall WJ, Hopkins PM, et al. Multiple interacting gene products may influence susceptibility to malignant hyperthermia. Ann Hum Genet. 2000;64(Pt 4):307–20.
Stamm DS, Powell CM, Stajich JM, Zismann VL, Stephan DA, Chesnut B, et al. Novel congenital myopathy locus identified in native American Indians at 12q13.13–14.1. Neurology. 2008;71:1764–9. https://doi.org/10.1212/01.wnl.0000325060.16532.40.
Schneider banger D, Johannsen S, Roewer N, et al. Management of malignant hyperthermia: diagnosis and treatment. Ther Clin Risk Manag. 2014;10:355–62. https://doi.org/10.2147/TCRM.S47632.
Glahn KP, Ellis FR, Halsall PJ, et al. Recognizing and managing a malignant hyperthermia crisis: guidelines fromt he European Malignant Hyperthermia Group. Br J Anaesth. 2010;105:417–20. https://doi.org/10.1093/bja/aeq243.
Lawal TA, Todd JJ, Meilleur KG. Ryanodine receptor 1-related myopathies: diagnostic and therapeutic approaches. Neurotherapeutics. 2018;15(4):885–99. https://doi.org/10.1007/s13311-018-00677-1. Review.
Guis S, Figarella-Branger D, Monnier N, Bendahan D, Kozak-Ribbens G, Mattei JP, et al. Multiminicore disease in a family susceptible to malignant hyperthermia: histology, in vitro contracture tests, and genetic characterization. Arch Neurol. 2004;61:106–13. https://doi.org/10.1001/archneur.61.1.106.
Stamm DS, Powell CM, Stajich JM, Zismann VL, Stephan DA, Chesnut B, et al. Novel congenital myopathy locus identified in Native American Indians at 12q13.13–14.1. Neurology. 2008;71:1764–9. https://doi.org/10.1212/01.wnl.0000325060.16532.40.
Larach MG, Localio AR, Allen GC, et al. A clinical grading scale to predict malignant hyperthermia susceptibility. Anesthesiology. 1994;80:771–9. https://doi.org/10.1097/00000542-199404000-00008.
A protocol for the investigation of malignant hyperpyrexia (MH) susceptibility. The European Malignant Hyperpyrexia Group. Br J Anaesth 1984; 56(11):1267–9.
Larach MG, Landis JR, Bunn JS, Diaz M. Prediction of malignant hyperthermia susceptibility in low-risk subjects. An epidemiologic investigation of caffeine halothane contracture responses. The North American Malignant Hyperthermia Registry. Anesthesiology. 1992;76(1):16–27.
Ording H, Brancadoro V, Cozzolino S, Ellis FR, Glauber V, Gonano EF, et al. In vitro contracture test for diagnosis of malignant hyperthermia following the protocol of the European MH Group: results of testing patients surviving fulminant MH and unrelated low-risk subjects. The European Malignant Hyperthermia Group. Acta Anaesthesiol Scand. 1997;41(8):955–66. https://doi.org/10.1111/j.1399-6576.1997.tb04820.x.
Allen GC, Larach MG, Kunselman AR. The sensitivity and specificity of the caffeine-halothane contracture test: a report from the North American Malignant Hyperthermia Registry. The North American Malignant Hyperthermia Registry of MHAUS. Anesthesiology. 1998;88(3):579–88.
Stowell KM. DNA testing for malignant hyperthermia: the reality and the dream. Anesth Analg. 2014;118(2):397–406. https://doi.org/10.1213/ANE.0000000000000063.
Broman M, Kleinschnitz I, Bach JE, Rost S, Islander G, Muller CR. Next-generation DNA sequencing of a Swedish malignant hyperthermia cohort. Clin Genet. 2015;88(4):381–5. https://doi.org/10.1111/cge.12508.
Bannister RA. Dantrolene-induced inhibition of skeletal L-type Ca2+ current requires RyR1 expression. Biomed Res Int. 2013;2013:390493. https://doi.org/10.1155/2013/390493.
Malignant Hyperthermia Association of the United States; 2017. https://www.mhaus.org/healthcare-professionals/managing-a-crisis/.
Larach MG, Gronert GA, Allen GC, Brandom BW, Lehman EB. Clinical presentation, treatment, and complications of malignant hyperthermia in North America from 1987 to 2006. Anesth Analg. 2010;110(2):498–507. https://doi.org/10.1213/ANE.0b013e3181c6b9b2.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Kaur, H., Salame, K.G. (2020). Malignant Hyperthermia. In: Arora, N., Govindarajan, R., Kataria, S., Nattanmai Chandrasekaran, P. (eds) Neuromuscular Urgencies and Emergencies. Springer, Cham. https://doi.org/10.1007/978-3-030-53145-4_8
Download citation
DOI: https://doi.org/10.1007/978-3-030-53145-4_8
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-53144-7
Online ISBN: 978-3-030-53145-4
eBook Packages: MedicineMedicine (R0)