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Follow-up of patients tested for malignant hyperthermia susceptibility

Published online by Cambridge University Press:  19 June 2006

D. Scala
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
A. Di Martino
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
S. Cozzolino
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
A. Mancini
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
A. Bracco
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
B. Andria
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
A. Tammaro
Affiliation:
Cardarelli Hospital, Biotechnology Centre, Naples, Italy
G. Savoia
Affiliation:
Cardarelli Hospital, Anestesia e Rianimazione del Dipartimento Materno Infantile, Naples, Italy
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Abstract

Summary

Background and objective: Malignant hyperthermia is an inherited disorder of skeletal muscle characterized by muscle contracture and hypermetabolic crisis following exposure to halogenated anaesthetics and depolarizing muscle relaxants. We planned this follow-up to get more information about the safety of non-triggering anaesthesia in susceptible patients; the safety of the use of trigger agents in non-susceptible patients and any minor sequelae following the biopsy. Methods: A questionnaire was sent to 244 patients tested for susceptibility between 1998 and 2004 enquiring about sequelae from the biopsy, subsequent experience with anaesthesia and difficulties encountered because of the investigation. Results: Replies were received from 129 patients. Thirty-four complained about sequelae from the biopsy: 10 reported headache and nausea; 16 experienced pain and a lack of strength in the biopsed leg and 8 found the scar less than satisfactory. Ten patients found it difficult to find a diagnostic centre. Eighteen reported problems and/or delay when they had needed a subsequent anaesthetic. Fourteen patients found the anaesthesiologist reluctant to anaesthetize them and four experienced a delay. Forty-three patients received anaesthesia since their biopsy. Complete medical records were available for 24 anaesthetic exposures in 23 patients. No documented perioperative complications occurred. Only three non-susceptible patients received one trigger agent. Conclusions: It is safe to use trigger-free anaesthesia in susceptible patients. The difficulties encountered by patients to be anaesthetized and the management of the majority of non-susceptible patients during general anaesthesia show the need of more accurate educational programmes and methods for promoting patient-centred care.

Type
Original Article
Copyright
2006 European Society of Anaesthesiology

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References

McCarthy EJ. Malignant hyperthermia: pathophysiology, clinical presentation, and treatment. AACN Clin Issues 2004; 15 (2): 231237.Google Scholar
Litman RS, Rosenberg H. Malignant hyperthermia: update on susceptibility testing. JAMA 2005; 293 (23): 29182924.Google Scholar
Kobayashi S, Bannister ML, Gangopadhyay JP, Hamada T, Parness J, Ikemoto N. Dantrolene stabilizes domain interactions within the ryanodine receptor. J Biol Chem 2005; 280 (8): 65806587.Google Scholar
Krause T, Gerbershagen MU, Fiege M, Weisshorn R, Wappler F. Dantrolene – a review of its pharmacology, therapeutic use and new developments. Anaesthesia 2004; 59 (4): 364373.Google Scholar
Islander G, Ording H, Bendixen D, Ranklev Twetman E. Reproducibility of in vitro contracture test results in patients tested for malignant hyperthermia susceptibility. Acta Anaesthesiol Scand 2002; 46 (9): 11441149.Google Scholar
Ording H, Brancadoro V, Cozzolino Set al. In vitro contracture test for diagnosis of malignant hyperthermia following the protocol of the European MH Group: results of testing patients surviving fulminant MH and unrelated low-risk subjects. The European Malignant Hyperthermia Group. Acta Anaesthesiol Scand 1997; 41 (8): 955966.Google Scholar
Ben Abraham R, Adnet P, Glauber V, Perel A. Malignant hyperthermia 1. Postgrad Med J 1998; 74 (867): 1117.Google Scholar
Heggie JE. Malignant hyperthermia: considerations for the general surgeon. Can J Surg 2002; 45 (5): 369372.Google Scholar
Farbu E, Softeland E, Bindoff LA. Anaesthetic complications associated with myotonia congenita: case study and comparison with other myotonic disorders. Acta Anaesthesiol Scand 2003; 47 (5): 630634.Google Scholar
Allen GC, Rosenberg H, Fletcher JE. Safety of general anesthesia in patients previously tested negative for malignant hyperthermia susceptibility. Anesthesiology 1990; 72 (4): 619622.Google Scholar
Islander G, Ranklev-Twetman E. Evaluation of anaesthesias in malignant hyperthermia negative patients. Acta Anaesthesiol Scand 1995; 39 (6): 819821.Google Scholar
Ording H, Hedengran AM, Skovgaard LT. Evaluation of 119 anaesthetics received after investigation for susceptibility to malignant hyperthermia. Acta Anaesthesiol Scand 1991; 35 (8): 711716.Google Scholar
Halsall PJ, Cain PA, Ellis FR. Retrospective analysis of anaesthetics received by patients before susceptibility to malignant hyperpyrexia was recognized. Br J Anaesth 1979; 51 (10): 949954.Google Scholar
Claxton BA, Cross MH, Hopkins PM. No response to trigger agents in a malignant hyperthermia-susceptible patient. Br J Anaesth 2002; 88 (6): 870873.Google Scholar
Carr AS, Lerman J, Cunliffe M, McLeod ME, Britt BA. Incidence of malignant hyperthermia reactions in 2,214 patients undergoing muscle biopsy. Can J Anaesth 1995; 42 (4): 281286.Google Scholar
Wackym PA, Dubrow TJ, Abdul-Rasool IH, Peacock WJ. Neurosurgery in the malignant hyperthermia-susceptible patient. Neurosurgery 1988; 22 (6 Part 1): 10321036.Google Scholar
Dudley JP, Reynolds R, Dubrow TJ. Malignant hyperthermia in the otolaryngologic patient: prospective anesthetic and surgical management of eight children. Ann Otol Rhinol Laryngol 1990; 99 (4 Part 1): 297299.Google Scholar