Malignant hyperthermia is a serious complication of general anaesthesia. It is a genetically determined disposition with an autosomal dominant transfer. The development of MH is conditioned by the exposure to triggering agents – inhalation anaesthetics and suxamethonium.The nature ofMHis a disorder of calcium metabolismin sarcoplasmic reticulum receptors in skeletal muscle. The review presents an overview of the history and current knowledge of pathophysiology, genetics, clinical picture and treatment of MH, gives important information for anaesthesia practice, summarizes current means of pre-symptomatic testing and informs on the state of MH diagnostics world-wide and in the Czech Republic.

        Key words: malignant hyperthermia – pre-symptomatic diagnostics – National Center for Diagnostics of Malignant Hyperthermia in the Czech Republic