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Malignant Hyperthermia (MH)

What is MH? | Testing | Patient Referral | Genetic Testing for MH | Management of Perioperative Rise in Temperature | Treatment of MH | MH Poster | MH Australia


What is MH?

Malignant Hyperthermia (MH) is a life-threatening inherited pharmacogenetic disorder of skeletal muscle. It is caused by exposure of susceptible individuals to volatile anaesthetics or suxamethonium. The genetics are heterogeneous with an autosomal dominant pattern. To date, causative genetic mutations can be detected in 50-70% of families with the disease. Predictive testing still relies heavily on the invasive & expensive in-vitro contracture test. Despite recognition and aggressive management of an MH crisis, it still has a reported mortality as high as 11%.


Testing

Both muscle testing (with an IVCT) and DNA based diagnosis can be organised through the Department of Anaesthesia MH Investigation Unit at Royal Perth Hospital. We perform IVCT according to the European MH Group protocol in Neuropathology located on-site at RPH. IVCT requires a fresh specimen of vastus lateralis muscle (approximately 2 x 2 x 1cm). Patients are admitted for day procedure and given a safe non-triggering general anaesthetic with a prepared workstation.


Patient Referral

Patients should be referred to:

The MH Investigation Unit
Department of Anaesthesia & Pain Medicine
Royal Perth Hospital
GPO Box X2213
Perth WA 6001

Phone: 08 9224 1038
Fax: 08 9224 1111

Patients will be contacted by a member of the MH Investigation Unit for a pre-counselling appointment.  Staff include: Dr Phillip Nelson, Dr Kristine Wardle, Jennifer Fraser and Sue Chinnery.


Genetic Testing for MH

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Genetic mutations in the RYR1 gene (encodes the calcium release channel of the sarcoplasmic reticulum) on chromosome 19 and the CACNA1S gene (encodes the alpha-1 subunit of the dihydropyridine recetor) on chromosome 1 have been linked to MH.  DNA tests can be ordered through Neurogenetics (Mark Davis) at QEII in collaboration with our unit and if a causative gene in that family can be identified, we can test other family members with a blood test.  Currently a negative genetic test still requires a confirmatory IVCT in order to rule out the diagnosis of MH.


Management of Perioperative Rise in Temperature

Many differential diagnoses:

 


Manifestations of MH

Multiple MH signs are often reported simultaneously.
Most frequent clinical signs are:
Unexpected increase in CO2  production

Tachycardia

Hyperthermia

Muscle Rigidity

Cyanosis

Abnormal Laboratory Findings

 

Treatment of MH

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Prompt recognition is the key to a safe outcome.
MH is a true operating room crisis – need to declare an emergency, involve all personnel & get more help.
Task cards should be available on an MH trolley to help prompt treatment and delegate tasks.

Download from http://www.anaesthesia.mh.org.au/mh-resource-kit/wl/i1002692/

Treatment protocol focusses on:

Complications occur in 20% of patients who survive a fulminant crisis; most commonly renal dysfunction.
Arrange transfer to an Intensive Care Unit & referral to an MH Investigation Unit for testing at a later stage.

[Author: K Wardle 27 May 2015]

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What is MH? | Testing | Patient Referral | Genetic Testing for MH | Management of Perioperative Rise in Temperature | Treatment of MH | MH Poster | MH Australia