Consensus guidelines on perioperative management of malignant hyperthermia suspected or susceptible patients from the European Malignant Hyperthermia Group

Abstract

Malignant hyperthermia is a potentially fatal condition, in which genetically predisposed individuals develop a hypermetabolic reaction to potent inhalation anaesthetics or succinylcholine. Because of the rarity of malignant hyperthermia and ethical limitations, there is no evidence from interventional trials to inform the optimal perioperative management of patients known or suspected with malignant hyperthermia who present for surgery. Furthermore, as the concentrations of residual volatile anaesthetics that might trigger a malignant hyperthermia crisis are unknown and manufacturers' instructions differ considerably, there are uncertainties about how individual anaesthetic machines or workstations need to be prepared to avoid inadvertent exposure of susceptible patients to trigger anaesthetic drugs. The present guidelines are intended to bundle the available knowledge about perioperative management of malignant hyperthermia-susceptible patients and the preparation of anaesthesia workstations. The latter aspect includes guidance on the use of activated charcoal filters. The guidelines were developed by members of the European Malignant Hyperthermia Group, and they are based on evaluation of the available literature and a formal consensus process. The most crucial recommendation is that malignant hyperthermia-susceptible patients should receive anaesthesia that is free of triggering agents. Providing that this can be achieved, other key recommendations include avoidance of prophylactic administration of dantrolene; that preoperative management, intraoperative monitoring, and care in the PACU are

Referral Indications for Malignant Hyperthermia Susceptibility Diagnostics in Patients without Adverse Anesthetic Events in the Era of Next-generation Sequencing

“…On the other hand, 60.8% of the patients without a personal or family history of an adverse anesthetic event were diagnosed as non-MH-susceptible, indicating the importance of MH susceptibility diagnostics; a non-MH-susceptible test result enables anesthesiologists to treat carriers of RYR1 variants and their family members without MH precaution measures. 4,22 In our study, 16 of 42 of the patients with exertional and/or recurrent rhabdomyolysis were diagnosed as MH-susceptible, which compared to previous case studies is less frequent than 11 of 12 23 and 5 of 6 24 but more frequent than 2 of 14. 18 This variability can, at least partly, be explained by selection bias concerning some of these study cohorts.…”

Remimazolam in a Pediatric Patient With a Suspected Family History of Malignant Hyperthermia

“…The primary cellular defect responsible for MH has been identified as the calcium release channel of the sarcoplasmic reticulum, leading to excessive calcium release in response to triggering agents [1]. In an in vitro experiment, the responsiveness to caffeine was compared in HEK-293 cells expressing wild-type RYR1 with that of mutant RYR1 following perfusion with remimazolam or propofol [16].…”

“…Malignant hyperthermia (MH) is an acute hypermetabolic syndrome, triggered in susceptible patients by the administration of succinylcholine or a volatile anesthetic agent. The primary cellular defect responsible for MH has been identified as the calcium release channel of the sarcoplasmic reticulum (RYR1, ryanodine receptor 1) [1]. Following exposure to triggering agents, excessive calcium release into the cytoplasm of skeletal muscle fibers results in myofibrillar contraction, depletion of high energy phosphate compounds, lactic acid and carbon dioxide production, and hyperthermia.…”

Preparation of Dräger Atlan A350 and General Electric Healthcare Carestation 650 anesthesia workstations for malignant hyperthermia susceptible patients

“…This may be problematic in pediatric anesthesia, were a low FGF is recommended to avoid loss of heat and moisture. If using ACF, the filters should be remained in place for the whole anesthetic procedure [ 2 ]. The manufacturer recommend changing ACF after 12 h, but in previews studies we demonstrated a safe use of the ACF for 24 h [ 7 ].…”

“…Unfortunately, cleaning modern anesthetic machines highly differs from manufacturer and device because volatile anesthetics adsorb and desorb to different amount of rubber and plastic components of the anesthetic machine [ 1 ]. The European Malignant Hyperthermia Group (EMHG) have developed consensus guidelines on perioperative management of MHS patients that contains recommendations on the elimination of residual trace concentrations of volatile anesthetics in the machine to ensure a trigger-free anesthesia [ 2 ]. Basically, three different approaches are recommended: First: Using a spare anesthetic machine which had never contact to any volatile anesthetic.…”