Individuals who have susceptibility to MH should not be exposed to potent inhalation anesthetics or succinylcholine.[1]Hopkins PM, Girard T, Dalay S, et al. Malignant hyperthermia 2020: guideline from the Association of Anaesthetists. Anaesthesia. 2021 May;76(5):655-64.
https://www.doi.org/10.1111/anae.15317
http://www.ncbi.nlm.nih.gov/pubmed/33399225?tool=bestpractice.com
However, they may be cared for safely in both inpatient operative settings and properly resourced ambulatory surgery centers, utilizing non-triggering anesthetic agents.[91]Litman RS, Smith VI, Larach MG, et al. Consensus statement of the Malignant Hyperthermia Association of the United States on unresolved clinical questions concerning the management of patients with malignant hyperthermia. Anesth Analg. 2019 Apr;128(4):652-9.
http://www.ncbi.nlm.nih.gov/pubmed/30768455?tool=bestpractice.com
Prophylactic dantrolene is not recommended in these patients.[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.
https://www.doi.org/10.1016/j.bja.2020.09.029
http://www.ncbi.nlm.nih.gov/pubmed/33131754?tool=bestpractice.com
[104]European Malignant Hyperthermia Group. Perioperative management of known or suspected MHS. Jan 2021 [internet publication].
https://www.emhg.org/recommendations-1/2021/1/19/perioperative-management-of-known-or-suspected-mhs
[105]Urman RD, Rajan N, Belani K, et al. Malignant hyperthermia-susceptible adult patient and ambulatory surgery center: Society for Ambulatory Anesthesia and Ambulatory Surgical Care Committee of the American Society of Anesthesiologists position statement. Anesth Analg. 2019 Aug;129(2):347-9.
https://www.doi.org/10.1213/ANE.0000000000004257
http://www.ncbi.nlm.nih.gov/pubmed/31166228?tool=bestpractice.com
The anesthetic workstation used in MH susceptible patients should either be a dedicated workstation for trigger-free anesthetics or a workstation properly prepared by flushing and/or the use of activated charcoal filters.[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.
https://www.doi.org/10.1016/j.bja.2020.09.029
http://www.ncbi.nlm.nih.gov/pubmed/33131754?tool=bestpractice.com
[105]Urman RD, Rajan N, Belani K, et al. Malignant hyperthermia-susceptible adult patient and ambulatory surgery center: Society for Ambulatory Anesthesia and Ambulatory Surgical Care Committee of the American Society of Anesthesiologists position statement. Anesth Analg. 2019 Aug;129(2):347-9.
https://www.doi.org/10.1213/ANE.0000000000004257
http://www.ncbi.nlm.nih.gov/pubmed/31166228?tool=bestpractice.com
Charcoal filters reliably prepare anesthetic workstations in 90 seconds, while the flushing process otherwise varies according to manufacturer guidelines and requires up to 2 hours in some modern workstations.[101]European Malignant Hyperthermia Group. Preparation of anaesthetic workstations for MH suspected or susceptible patients. February 2018 [internet publication].
https://www.emhg.org/recommendations-1/2018/2/27/preparation-of-anaesthetic-workstations-for-mh-suspected-or-susceptible-patients
[106]Malignant Hyperthermia Association of the United States. Preparing the anesthesia machine for MHS patients [internet publication].
https://www.mhaus.org/healthcare-professionals/be-prepared/preparing-the-anesthesia-machine
Previous safe exposure to a potent inhalation anesthetic should not be taken as evidence that future exposures will be safe; on average MH does not present until the third inhaled anesthetic exposure in an MH susceptible patient.[6]Larach MG, Gronert GA, Allen GC, et al. Clinical presentation, treatment, and complications of malignant hyperthermia in North America from 1987 to 2006. Anesth Analg. 2010 Feb 1;110(2):498-507.
http://journals.lww.com/anesthesia-analgesia/pages/articleviewer.aspx?year=2010&issue=02000&article=00039&type=Fulltext
http://www.ncbi.nlm.nih.gov/pubmed/20081135?tool=bestpractice.com
[7]Rosenberg H, Pollock N, Schiemann A, et al. Malignant hyperthermia: a review. Orphanet J Rare Dis. 2015 Aug 4;10:93.
http://ojrd.biomedcentral.com/articles/10.1186/s13023-015-0310-1
http://www.ncbi.nlm.nih.gov/pubmed/26238698?tool=bestpractice.com
Some myopathic patients can receive inhalation anesthetics safely. All anesthesia providers must have a plan for identifying and means for treating MH in a patient in whom no increased risk was detected preoperatively.
The anesthetic and general medical records of individuals suspected of having experienced an episode of MH or of being susceptible to MH must be reviewed, preferably by an experienced anesthesiologist and ideally by the physicians at an MH Diagnostic Testing Center. The primary care physician of the patient should be involved in this process, and consultation with a neurologist or other specialist may also be indicated.
The Clinical Grading Scale (CGS) is based on observations of muscle tone, respiration, core temperature, family history, and laboratory tests.[55]Larach MG, Localio AR, Allen GC, et al. A clinical grading scale to predict MH susceptibility. Anesthesiology. 1994 Apr;80(4):771-9.
http://www.ncbi.nlm.nih.gov/pubmed/8024130?tool=bestpractice.com
All these data should be collected because the CGS can be used to estimate the likelihood that an observed episode was due to MH. However, a low CGS does not by itself indicate that the event was not due to MH. The CGS assigns a point score primarily based on evidence of (1) muscle rigidity, (2) muscle breakdown (creatine kinase, myoglobinuria, hyperkalemia), (3) unexpected hypercapnia, (4) inappropriate temperature increase, (5) inappropriate tachycardia, and (6) family history of MH. Most items score 15 points and a score >35 makes MH very likely.[55]Larach MG, Localio AR, Allen GC, et al. A clinical grading scale to predict MH susceptibility. Anesthesiology. 1994 Apr;80(4):771-9.
http://www.ncbi.nlm.nih.gov/pubmed/8024130?tool=bestpractice.com
Interestingly, rapid reversal of MH signs following intravenous dantrolene only scores 5 points on the CGS. Clearly, a muscle biopsy with contracture testing and/or genetic analysis are important for confirming that MH susceptibility is present.