Pediatr. praxi 2017; 18(6): 368-372 | DOI: 10.36290/ped.2017.072

Malignant hyperthermia

MUDr. Jana Vojtíšková

Dětská klinika FZS UJEP v Ústí nad Labem a KZ, a. s. – Masarykova nemocnice v Ústí nad Labem, o. z.

Novorozenecké oddělení Masarykova nemocnice v Ústí nad Labem, o. z., KZ, a. s.

Malignant hyperthermia (MH) is a rare but very dangerous and potentionally fatal complication of general anesthesia. It occurson the basis of an autosomal dominant condition following contact with the trigger. The triggers are volatile anesthetics andsuccinylcholine. The cause of MH is the disorder of calcium metabolism regulation in the muscle cell caused by the abnormalryanodin receptor RYR1. As a result, extreme muscle contraction without relaxation leads to a huge hypermetabolic reaction.Diagnosis of MH disposition can be confirmed by a muscle contraction test in vitro, molecular genetic examination respectively.„Suspicious“ individuals can be identified by careful preoperative examinations and medical history. In patients with MH disposition,if anesthesia is necessary, it is essential to choose special anesthetic procedures without the use of triggers with currentcareful perioperative monitoring with the option of intensive care. Mortality today is about 5–10% (compared to 70–80% in the1970s). Significant increases in patient survival have helped to improve perioperative monitoring, awareness of medical staff and,in particular, the introduction of dantrolene as a causal drug.

Keywords: malignant hyperthermia, children, diagnosis, therapy

Published: December 1, 2017  Show citation