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JSA guideline for the management of malignant hyperthermia crisis 2016

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Abstract

Malignant hyperthermia (MH) can be fatal if the crisis is not appropriately treated. It is an inherited disease usually triggered by the administration of volatile inhalational anesthetics and/or succinylcholine, a muscle relaxant. In a patient with suspected MH, the mechanism of calcium release from storage in the sarcoplasmic reticulum in the skeletal muscle is abnormally accelerated. Unexplained hypercarbia representing >55 mmHg of end-tidal carbon dioxide, tachycardia, and muscle rigidity (including masseter muscle rigidity) are early signs of the initiation of MH, because the metabolism is accelerated. The body temperature can rise by >0.5 °C/15 min and may reach ≥40 °C. Respiratory and metabolic acidosis, arrhythmia, cola-colored urine, increased levels of serum potassium, and tented T-waves on electrocardiogram are common and can lead to cardiac arrest. MH should be treated by discontinuation of the triggering agents, administration of intravenous dantrolene (initially 1 mg/kg), and reduction of the body temperature. Early diagnosis and sufficient dantrolene with body temperature reduction are essential to relieve the patient’s MH crisis. This guideline in Japanese translation has been posted on the website: http://www.anesth.or.jp/guide/pdf/guideline_akuseikounetsu.pdf.

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Abbreviations

MH:

Malignant hyperthermia

PMH:

Postoperative malignant hyperthermia

References

  1. Harrison GG. Control of the malignant hyperpyrexia syndrome in MHS swine by dantrolene sodium. Br J Anaesth. 1975;47:62–5.

    Article  CAS  PubMed  Google Scholar 

  2. Kolb ME, Horne ML, Martz R. Dantrolene in human malignant hyperthermia. A multicenter study. Anesthesiology. 1982;56:254–62.

    Article  CAS  PubMed  Google Scholar 

  3. Larach MG, Localio AR, Allen GC, Denborough MA, Ellis FR, Gronert GA, Kaplan RF, Muldoon SM, Nelson TE, Ording H, Rosenberg H, Waud BE, Wedel DJ. A clinical grading scale to predict malignant hyperthermia susceptibility. Anesthesiology. 1994;80:771–9.

    Article  CAS  PubMed  Google Scholar 

  4. Morio M, Kikuchi H, Yuge O, Murata K, Fujioka Y, Mukaida K, Mori K. Clinical criteria of malignant hyperthermia – Revised criteria (in Japanese with English abstract). Anesth Resusc. 1988;24(Supple 11):104–10.

    Google Scholar 

  5. 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;118:381–7.

    Article  CAS  PubMed  Google Scholar 

  6. Visoiu M, Young MC, Wieland K, Brandom BW. Anesthetic drugs and onset of malignant hyperthermia. Anesth Analg. 2014;118:388–96.

    Article  CAS  PubMed  Google Scholar 

  7. Glahn KP, Ellis FR, Halsall PJ, Müller CR, Snoeck MM, Urwyler A, Wappler F. European malignant hyperthermia group. recognizing and managing a malignant hyperthermia crisis: guidelines from the European malignant hyperthermia group. Br J Anaesth. 2010;105:417–20.

    Article  CAS  PubMed  Google Scholar 

  8. Lin HT, Wang SC1, Zuo Z, Tsou MY, Chan KH, Yuan HB. Increased requirement for minute ventilation and negative arterial to end-tidal carbon dioxide gradient may indicate malignant hyperthermia. J Chin Med Assoc. 2014;77:209–12.

    Article  PubMed  Google Scholar 

  9. Kwetny I, Finucane BT. Anesth Analg. Negative arterial to end-tidal carbon dioxide gradient: an additional sign of malignant hyperthermia during desflurane anesthesia. Anesth Analg. 2006;102:815–7.

    Article  CAS  PubMed  Google Scholar 

  10. Jones C, Bennett K, Kim TW, Bulger TF, Pollock N. Preparation of Datex-Ohmeda Aestiva and Aisys anaesthetic machines for use in malignant hyperthermia susceptible patients. Anaesth Intensive Care. 2012;40:490–7.

    CAS  PubMed  Google Scholar 

  11. Nelson P, Litman RS. Malignant hyperthermia in children: an analysis of the North American malignant hyperthermia registry. Anesth Analg. 2014;118:369–74.

    Article  PubMed  Google Scholar 

  12. Burkman JM, Posner KL, Domino KB. Analysis of the clinical variables associated with recrudescence after malignant hyperthermia reactions. Anesthesiology. 2007;106:901–6.

    Article  PubMed  Google Scholar 

  13. Bandschapp O, Girard T. Malignant hyperthermia. Swiss Med Wkly. 2012;142:w13652.

    PubMed  Google Scholar 

  14. Migita T, Mukaida K, Hamada H, Kawamoto M. Analysis of postoperative malignant hyperthermia (in Japanese with English abstract). Anesth Resusc. 2013;49:7–11.

    Google Scholar 

  15. Litman RS, Flood CD, Kaplan RF, Kim YL, Tobin JR. Postoperative malignant hyperthermia: an analysis of cases from the north American malignant hyperthermia registry. Anesthesiology. 2008;109:825–9.

    Article  PubMed  Google Scholar 

  16. Rosenberg H, Pollock N, Schiemann A, Bulger T, Stowell K. Malignant hyperthermia: a review. Orphanet J Rare Dis. 2015;10:93.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Tobin JR, Jason DR, Challa VR, Nelson TE, Sambuughin N. Malignant hyperthermia and apparent heat stroke. JAMA. 2001;11:168–9.

    Article  Google Scholar 

  18. Thomas J, Crowhurst T. Exertional heat stroke, rhabdomyolysis and susceptibility to malignant hyperthermia. Intern Med J. 2013;43:1035–8.

    Article  CAS  PubMed  Google Scholar 

  19. Dlamini N, Voermans NC, Lillis S, Stewart K, Kamsteeg EJ, Drost G, Quinlivan R, Snoeck M, Norwood F, Radunovic A, Straub V, Roberts M, Vrancken AF, van der Pol WL, de Coo RI, Manzur AY, Yau S, Abbs S, King A, Lammens M, Hopkins PM, Mohammed S, Treves S, Muntoni F, Wraige E, Davis MR, van Engelen B, Jungbluth H. Mutations in RYR1 are a common cause of exertional myalgia and rhabdomyolysis. Neuromuscul Disord. 2013;23:540–8.

    Article  CAS  PubMed  Google Scholar 

  20. Sagui E, Montigon C, Abriat A, Jouvion A, Duron-Martinaud S, Canini F, Zagnoli F, Bendahan D, Figarella-Branger D, Brégigeon M, Brosset C. Is there a link between exertional heat stroke and susceptibility to malignant hyperthermia? PLoS One. 2015;10:e0135496.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Weglinski MR, Wedel DJ, Engel AG. Malignant hyperthermia testing in patients with persistently increased serum creatine kinase levels. Anesth Analg. 1997;84:1038–41.

    Article  CAS  PubMed  Google Scholar 

  22. Malandrini A, Orrico A, Gaudiano C, Gambelli S, Galli L, Berti G, Tegazzin V, Dotti MT, Federico A, Sorrentino V. Muscle biopsy and in vitro contracture test in subjects with idiopathic HyperCKemia. Anesthesiology. 2008;109:625–8.

    Article  PubMed  Google Scholar 

  23. Mukaida K, Kawamoto M. Malignant Hyperthermia-up-to-date topics (in Japanese with English abstract). J Jpn Soc Clin Anesth. 2012;32:682–90.

    Article  Google Scholar 

  24. Monsieurs KG, Van Broeckhoven C, Martin JJ, Van Hoof VO, Heytens L. Gly341Arg mutation indicating malignant hyperthermia susceptibility: specific cause of chronically elevated serum creatine kinase activity. J Neurol Sci. 1998;154:62–5.

    Article  CAS  PubMed  Google Scholar 

  25. Sano K, Miura S, Fujiwara T, ujioka R, Yorita A, Noda K, Kida H, Azuma K, Kaieda S, Yamamoto K, Taniwaki T, Fukumaki Y, Shibata H. Novel missense mutation of RYR1 in familial idiopathic hyper CK-emia. J Neurol Sci. 2015;356:142–7.

    Article  CAS  PubMed  Google Scholar 

  26. Brislin RP, Theroux MC. Core myopathies and malignant hyperthermia susceptibility: a review. Paediatr Anaesth. 2013;23:834–41.

    Article  PubMed  Google Scholar 

  27. Maggi L, Scoto M, Cirak S, Robb SA, Klein A, Lillis S, Cullup T, Feng L, Manzur AY, Sewry CA, Abbs S, Jungbluth H, Muntoni F. Congenital myopathies—clinical features and frequency of individual subtypes diagnosed over a 5-year period in the United Kingdom. Neuromuscul Disord. 2013;23:195–205.

    Article  CAS  PubMed  Google Scholar 

  28. Weigl LG, Hohenegger M, Kress HG. Dihydropyridine-induced Ca2+ release from ryanodine-sensitive Ca2+ pools in human skeletal muscle cells. J Physiol. 2000;525(Pt 2):461–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Migita T, Mukaida K, Yasuda T, Hamada H, Kawamoto M. Calcium channel blockers are inadequate for malignant hyperthermia crisis. J Anesth. 2012;26:579–84.

    Article  PubMed  Google Scholar 

  30. Wappler F. Anesthesia for patients with a history of malignant hyperthermia. Curr Opin Anaesthesiol. 2010;23:417–22.

    Article  PubMed  Google Scholar 

  31. Kim TW, Nemergut ME. Preparation of modern anesthesia workstations for malignant hyperthermia-susceptible patients: a review of past and present practice. Anesthesiology. 2011;114:205–12.

    Article  PubMed  Google Scholar 

  32. Kim TW, Wingate JR, Fernandez AM, Whitaker E, Tham RQ. Washout times of desflurane, sevoflurane and isoflurane from the GE Healthcare Aisys® and Avance®, Carestation®, and Aestiva® anesthesia system. Paediatr Anaesth. 2013;23:1124–30.

    PubMed  Google Scholar 

  33. Barnes C, Stowell KM, Bulger T, Langton E, Pollock N. Safe duration of postoperative monitoring for malignant hyperthermia patients administered non-triggering anaesthesia: an update. Anaesth Intensive Care. 2015;43:98–104.

    CAS  PubMed  Google Scholar 

  34. Hopkins PM. Malignant hyperthermia: advances in clinical management and diagnosis. Br J Anaesth. 2000;85:118–28.

    Article  CAS  PubMed  Google Scholar 

  35. Paasuke RT, Brownell AK. Serum creatine kinase level as a screening test for susceptibility to malignant hyperthermia. JAMA. 1986;255:769–71.

    Article  CAS  PubMed  Google Scholar 

  36. Wappler F, Fiege M, Antz M, Schulte Am Esch J. Hemodynamic and metabolic alterations in response to graded exercise in a patient susceptible to malignant hyperthermia. Anesthesiology. 2000;92:268–72.

    Article  CAS  PubMed  Google Scholar 

  37. Wappler F, Fiege M, Steinfath M, Agarwal K, Scholz J, Singh S, Matschke J, Schulte Am Esch J. Evidence for susceptibility to malignant hyperthermia in patients with stress-induced rhabdomyolysis. Anesthesiology. 2001;94:95–100.

    Article  CAS  PubMed  Google Scholar 

  38. López JR, Rojas B, Gonzalez MA, Terzic A. Myoplasmic Ca2+ concentration during exertional rhabdomyolysis. Lancet. 1995;345:424–5.

    Article  PubMed  Google Scholar 

  39. Tobin JR, Jason DR, Challa VR, Nelson TE, Sambuughin N. Malignant hyperthermia and apparent heat stroke. JAMA. 2001;286:168–9.

    Article  CAS  PubMed  Google Scholar 

  40. Hackl W, Winkler M, Mauritz W, Sporn P, Steinbereithner K. Muscle biopsy for diagnosis of malignant hyperthermia susceptibility in two patients with severe exercise-induced myolysis. Br J Anaesthesia. 1991;66:138–40.

    Article  CAS  Google Scholar 

  41. Allen GC. Oral dantrolene. Anaesthesia. 2004;59:1139–40.

    Article  CAS  PubMed  Google Scholar 

  42. Allen GC, Cattran CB, Peterson RG, Lalande M. Plasma levels of dantrolene following oral administration in malignant hyperthermia-susceptible patients. Anesthesiology. 1988;69:900–4.

    Article  CAS  PubMed  Google Scholar 

  43. Rosenberg H, Davis M, James D, Pollock N, Stowell K. Malignant hyperthermia. Orphanet J Rare Dis. 2007;2:21.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Fujioka Y, Matsui K, Mukaida K, Yuge O, Kikuchi H, Morio M. Effect of lidocaine on Ca2+ recruitment in the skinned skeletal muscle of the guinea pig (in Japanese with English abstract). Anesth Resusc. 1988;24(Supple 19):19–23.

    CAS  Google Scholar 

  45. Kalow W, Britt BA, Terreau ME, Haist C. Metabolic error of muscle metabolism after recovery from malignant hyperthermia. Lancet. 1970;2(7679):895–8.

    Article  CAS  PubMed  Google Scholar 

  46. http://www.asahq.org/resources/standards-and-guidelines/.

  47. Isaacs H, Badenhorst M. False-negative results with muscle caffeine halothane contracture testing for malignant hyperthermia. Anesthesiology. 1993;79:5–9.

    Article  CAS  PubMed  Google Scholar 

  48. Mukaida K, Yuge O. Diagnosis of malignant hyperthermia In: Kikuchi H, editor. Malignant hyperthermia for professional anesthesiologists (Japanese). Kokuseido; 2006. pp.155–8.

  49. Ibarra CAM, Wu S, Murayama K, Minami N, Ichihara Y, Kikuchi H, Noguchi S, Hayashi YK, Ochiai R, Nishino I. Malignant hyperthermia in Japan: mutation screening of the entire ryanodine receptor type 1 gene coding region by direct sequencing. Anesthesiology. 2006;104:1146–54.

    Article  Google Scholar 

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Acknowledgements

This guideline was the achievement by Masashi Kawamoto, Keiko Mukaida, Yasuko Ichihara, Shiroh Isono, and Hirosato Kikuchi, Malignant Hyperthermia working group of Safety Committee, the Japanese Society of Anesthesiologists.

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Safety Committee of Japanese Society of Anesthesiologists. JSA guideline for the management of malignant hyperthermia crisis 2016. J Anesth 31, 307–317 (2017). https://doi.org/10.1007/s00540-016-2305-z

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  • DOI: https://doi.org/10.1007/s00540-016-2305-z

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