Malignant hyperthermia, a Scandinavian update. Issue 8 (18th May 2015)

• Record Type: Journal Article
• Authors: Broman, M.; Islander, G.; Müller, C. R.
• Is Part Of: Acta anaesthesiologica scandinavica. Volume 59:Issue 8(2015:Sep.)
• Page Start: 951
• Page End: 961
• Publication Date: 2015-05-18
• Subjects: Anesthesiology -- Periodicals; Critical care medicine -- Periodicals; 617.9605
• DOI: 10.1111/aas.12541
• Languages: English
• ISSNs: 0001-5172

Abstract

<abstract abstract-type="main" id="aas12541-abs-0001"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="aas12541-sec-0001" sec-type="section"> <title>Background</title> <p>Malignant Hyperthermia (MH) is a rare pharmacogenetic disorder, triggered by halogenated anesthetics and/or succinylcholine. In susceptible individuals, these drugs can activate an explosive life threatening clinical reaction. Leading symptoms are hypercarbia, muscle rigidity, and metabolic acidosis. MH is inherited in an autosomal‐dominant manner and linked to mutations in the large ryanodine 1 gene (RYR1) gene in the majority of cases. Very few MH patients have been found to carry mutations in the CACNA1S gene.</p> </sec> <sec id="aas12541-sec-0002" sec-type="section"> <title>Methods</title> <p>For this review a large litterature search was carried out and the Swedish MH database consisting of 436 probands who have undergone in vitro muscle contraction test (IVCT) during 1984–2014 was analyzed.</p> </sec> <sec id="aas12541-sec-0003" sec-type="section"> <title>Results</title> <p>Twelve different MH causative mutations have been found in Swedish patients so far. These mutations lead to a disturbed calcium balance in striated muscle tissue. A muscle biopsy for the IVCT or finding of an approved causative mutation are required for the diagnosis.</p> </sec> <sec id="aas12541-sec-0004" sec-type="section"> <title>Conclusion</title> <p>A Malignant Hyperthermia susceptible (MHS) patient should be anesthetized with trigger‐free anesthesia. There are a few reports of MH‐like reactions in patients unrelated to anesthesia. The outcome is dependent on early recognizing of the reaction and fast disconnection of the trigger agents and administration of dantrolene.</p> </sec> </abstract>