Availability of dantrolene for the management of malignant hyperthermia crises: European Malignant Hyperthermia Group guidelines

Abstract

Faced with a malignant hyperthermia crisis, the immediate access to sufficient dantrolene is essential to achieve the best possible outcome for the patient. However, malignant hyperthermia crises are rare, and there may be administrative pressures to limit the amount of dantrolene stocked or, in some countries, not to stock dantrolene at all. There are no published guidelines to support anaesthetic departments in their effort to ensure availability of sufficient dantrolene for the management of malignant hyperthermia crises. After a literature review that confirmed a lack of clinical trials to inform this guideline, we undertook a formal consensus development process, in which 25 members of the European Malignant Hyperthermia Group participated. The consensus process used a modified web-based Delphi exercise, in which participants rated the appropriateness of statements that covered the dosing regimen for dantrolene in a malignant hyperthermia crisis, the types of facility that should stock dantrolene, and the amount of dantrolene that should be stocked. The resulting guidelines are based on available evidence and the opinions of international malignant hyperthermia experts representing a large group of malignant hyperthermia laboratories from around the world. Key recommendations include: the dosing regimen of dantrolene should be based on actual body weight, dantrolene should be available wherever volatile anaesthetics or succinylcholine are used, and 36 vials of dantrolene should be immediately available with a further 24 vials available within 1 h.

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

“…Previous guidelines have been aimed at improving outcomes of MH crises, but we are frequently contacted for advice from anaesthesiologists tasked with providing anaesthesia for a known MHsusceptible patient. 2,3 Patients may be suspected of being at risk of developing MH based on a personal or family history: susceptibility may be confirmed through identification of genetic variants known to be pathogenic for MH or through in vitro muscle contracture tests. 4 Malignant hyperthermiasusceptible patients presenting for surgery require special precautions and perioperative preparations to minimise the likelihood of triggering an MH crisis.…”

Maligne Hyperthermie und Schwangerschaft – Empfehlungen der Europäischen Malignen Hyperthermie Gruppe

“…Während in der 2018 durch die Deutsche Gesellschaft für Anästhesiologie und Intensivmedizin publizierten S1-Leitlinie eine Mindestbevorratung von 10 mg/kg Dantrolen entsprechend 36-48 Ampullen gefordert wird [16], empfiehlt die EMHG in ihrer im August 2020 veröffentlichten Richtlinie eine Vorhaltung von mindesten 36 Dantrolen-Ampullen je 20 mg unter der Prämisse, dass weitere 24 Ampullen innerhalb 1 h zur Verfügung stehen [17].…”

Atypical symptoms of malignant hyperthermia: A rare causative mutation in the RYR1 gene

“…MH occurrence is associated with a high mortality rate if it cannot be recognized immediately and diagnosed earlier. Timely treatment using dantrolene sodium, a muscle relaxant that inhibits the release of Ca + from the endoplasmic reticulum by acting on RYR1 [ 8 ], can reduce the mortality from 80% in the 1970s to <5% nowadays [ 1 , 2 ].…”