Search

The European Malignant Hyperthermia Group has published updated guidelines for the investigation of malignant hyper-thermia (MH) susceptibility. This article gives a short description of the investigation of MH susceptibility in Denmark and an introduction to the new guidelines in Danish.

It is 30 yr since the British Journal of Anaesthesia published the first consensus protocol for the laboratory diagnosis of malignant hyperthermia susceptibility from the European Malignant Hyperthermia Group. This has subsequently been used in more than 10 000 individuals worldwide to inform use of anaesthetic drugs in these patients with increased risk of developing malignant hyperthermia during general anaesthesia, representing an early and successful example of stratified medicine. In 2001, our group also published a guideline for the use of DNA-based screening of malignant hyperthermia susceptibility. We now present an updated and complete guideline for the diagnostic pathway for patients potentially at increased risk of developing malignant hyperthermia. We introduce the new guideline with a narrative commentary that describes its development, the changes to previously published protocols and guidelines, and new sections, including recommendations for patient referral criteria and clinical interpretation of laboratory findings., (© The Author 2015. Published by Oxford University Press on behalf of the British Journal of Anaesthesia. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Survival from a malignant hyperthermia (MH) crisis is highly dependent on early recognition and prompt action. MH crises are very rare and an increasing use of total i.v. anaesthesia is likely to make it even rarer, leading to the potential risk of reduced awareness of MH. In addition, dantrolene, the cornerstone of successful MH treatment, is unavailable in large areas around the world thereby increasing the risk of MH fatalities in these areas. The European Malignant Hyperthermia Group collected and reviewed all guidelines available from the various MH centres in order to provide a consensus document. The guidelines consist of two textboxes: Box 1 on recognizing MH and Box 2 on the treatment of an MH crisis.

Malignant hyperthermia susceptibility is a rare pharmacogenetic muscular disorder that may lead to potentially fatal complications in routine anesthesia. Malignant hyperthermia is triggered by volatile anesthetics and succinylcholine. In this article the pathophysiology of malignant hyperthermia, its signs, symptoms and treatment as well as safe anesthetic agents for individuals susceptible to malignant hyperthermia are discussed. Although progress is made in our knowledge of the genetics behind malignant hyperthermia, muscle biopsy and in-vitro contracture testing is still the only way to diagnose malignant hyperthermia susceptibility in the vast majority of cases. Danish Malignant Hyperthermia Center at the Anesthetic Department of Herlev University Hospital, DK-2730 Herlev, takes care of all testing for malignant hyperthermia in Denmark and keeps a registry of all Danes with known or suspected susceptibility to malignant hyperthermia.