TY - JOUR
T1 - Pre-operative exercise and pyrexia as modifying factors in malignant hyperthermia (MH)
AU - Riazi, Sheila
AU - Bersselaar, Luuk R.van den
AU - Islander, Gunilla
AU - Heytens, Luc
AU - Snoeck, Marc M.J.
AU - Bjorksten, Andrew
AU - Gillies, Robyn
AU - Dranitsaris, George
AU - Hellblom, Anna
AU - Treves, Susan
AU - Kunst, Gudrun
AU - Voermans, Nicol C.
AU - Jungbluth, Heinz
N1 - Funding Information:
We like to thank our patients for their participation in this study.
Publisher Copyright:
© 2022 The Authors
PY - 2022/8
Y1 - 2022/8
N2 - Malignant hyperthermia (MH) is a life-threatening reaction triggered by volatile anesthetics and succinylcholine. MH is caused by mutations in the skeletal muscle ryanodine receptor (RYR1) gene, as is rhabdomyolysis triggered by exertion and/or pyrexia. The discrepancy between the prevalence of risk genotypes and actual MH incidence remains unexplained. We investigated the role of pre-operative exercise and pyrexia as potential MH modifying factors. We included cases from 5 MH referral centers with 1) clinical features suggestive of MH, 2) confirmation of MH susceptibility on Contracture Testing (IVCT or CHCT) and/or RYR1 genetic testing, and a history of 3) strenuous exercise within 72 h and/or pyrexia >37.5 °C prior to the triggering anesthetic. Characteristics of MH-triggering agents, surgery and succinylcholine use were collected. We identified 41 cases with general anesthesias resulting in an MH event (GA+MH, n = 41) within 72 h of strenuous exercise and/or pyrexia. We also identified previous general anesthesias without MH events (GA-MH, n = 51) in the index cases and their MH susceptible relatives. Apart from pre-operative exercise and/or pyrexia, trauma and acute abdomen as surgery indications, emergency surgery and succinylcholine use were also more common with GA+MH events. These observations suggest a link between pre-operative exercise, pyrexia and MH.
AB - Malignant hyperthermia (MH) is a life-threatening reaction triggered by volatile anesthetics and succinylcholine. MH is caused by mutations in the skeletal muscle ryanodine receptor (RYR1) gene, as is rhabdomyolysis triggered by exertion and/or pyrexia. The discrepancy between the prevalence of risk genotypes and actual MH incidence remains unexplained. We investigated the role of pre-operative exercise and pyrexia as potential MH modifying factors. We included cases from 5 MH referral centers with 1) clinical features suggestive of MH, 2) confirmation of MH susceptibility on Contracture Testing (IVCT or CHCT) and/or RYR1 genetic testing, and a history of 3) strenuous exercise within 72 h and/or pyrexia >37.5 °C prior to the triggering anesthetic. Characteristics of MH-triggering agents, surgery and succinylcholine use were collected. We identified 41 cases with general anesthesias resulting in an MH event (GA+MH, n = 41) within 72 h of strenuous exercise and/or pyrexia. We also identified previous general anesthesias without MH events (GA-MH, n = 51) in the index cases and their MH susceptible relatives. Apart from pre-operative exercise and/or pyrexia, trauma and acute abdomen as surgery indications, emergency surgery and succinylcholine use were also more common with GA+MH events. These observations suggest a link between pre-operative exercise, pyrexia and MH.
KW - (Exertional) rhabdomyolysis
KW - Malignant hyperthermia
KW - RYR1
UR - http://www.scopus.com/inward/record.url?scp=85132908875&partnerID=8YFLogxK
U2 - 10.1016/j.nmd.2022.06.003
DO - 10.1016/j.nmd.2022.06.003
M3 - Article
C2 - 35738978
AN - SCOPUS:85132908875
SN - 0960-8966
VL - 32
SP - 628
EP - 634
JO - Neuromuscular Disorders
JF - Neuromuscular Disorders
IS - 8
ER -
