Our knowledge about malignant hyperthermia (MH) has been markedly improved since 10 years, primarily due to molecular genetics. Susceptibility to MH is based on an abnormal calcium metabolism within the skeletal muscle fibre. This is most probably caused by a defective calcium release channel in the sarcoplasmic reticulum, e.g. the ryanodine receptor. This defect is due to genetic mutations essentially located into the RYR1 gene. Mortality of MH crisis has been dramatically decreased since the widespread use of capnograph in operating theatre and the immediate infusion of dantrolene sodium. However, incomplete and non typical forms of MH do exist, and require further testing for MH susceptibility for the probant and his (her) family. The reference test is currently the in vitro contracture test (IVCT), which exposes muscle strips to incremental concentration of halothane and caffeine. Genetic analysis has emerged as a viable supplement to IVCT. Safe anesthesia for MH susceptible patients must avoid the use of volatile agents and succinylcholine. Central core disease and maybe exercise-induced rhabdomyolysis have an increased risk for MH susceptibility.
- Contracture test
- Malignant hyperthermia
ASJC Scopus subject areas
- Critical Care and Intensive Care Medicine
- Anesthesiology and Pain Medicine