Scribd
Upload
61 views34 pages

Malignant Hyperthermia: Barbara Robertson, MD, FRCPC Dept of Anesthesia, PAH

Malignant hyperthermia is a potentially fatal inherited disease triggered by volatile anesthetics and succinylcholine. It results in a hypermetabolic state in skeletal muscles due to uncontrolled calcium release. Symptoms include fever, rigidity, acidosis, tachycardia and hyperthermia. Treatment involves stopping triggers, hyperventilation with oxygen, cooling, and administration of dantrolene to reduce calcium levels. Patients susceptible to MH require specialized anesthetic planning and precautions during surgery. Proper preparation and treatment can result in very low mortality from malignant hyperthermia episodes.

Uploaded by

Lee Chee Seng
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPT, PDF, TXT or read online on Scribd
61 views34 pages

Malignant Hyperthermia: Barbara Robertson, MD, FRCPC Dept of Anesthesia, PAH

Malignant hyperthermia is a potentially fatal inherited disease triggered by volatile anesthetics and succinylcholine. It results in a hypermetabolic state in skeletal muscles due to uncontrolled calcium release. Symptoms include fever, rigidity, acidosis, tachycardia and hyperthermia. Treatment involves stopping triggers, hyperventilation with oxygen, cooling, and administration of dantrolene to reduce calcium levels. Patients susceptible to MH require specialized anesthetic planning and precautions during surgery. Proper preparation and treatment can result in very low mortality from malignant hyperthermia episodes.

Uploaded by

Lee Chee Seng
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPT, PDF, TXT or read online on Scribd
You are on page 1/ 34

Malignant Hyperthermia

Barbara Robertson, MD, FRCPC


Dept of Anesthesia, PAH
Overview

• What is it?
• Why is it?
• Who gets it?
• How do you recognize it?
• How do you treat it?
• What to do if patient is
susceptible?
1960 First case described

1962 Inherited component suggested

1960’s Toronto and Wisconsin MH families identified /


similarity to PSS recognized

1971 International symposium Toronto

1971 CHCT muscle biopsy described

1975 Dantrolene found to be specific treatment (FDA


approved 1979)

1981-82 Patient advocacy groups formed

1980’s Use of intraoperative capnography helps early


detection

1990’s RYR1 gene mutation discovered (100% PSS,


50% MH)

2000’s Genetic testing available


Resources at hand

• MH hotline: 1-800-644-9737
• Outside USA: +1-303-389-1647
• Website: www.mhaus.org
• Wall flow chart from MHAUS
• There’s an app for that
(ePocrates, Gas Guide, MHapp)
What is MH?

• Potentially fatal inherited disease


• Fever, rigidity, acidosis
• Hypermetabolic state of skeletal
muscles with high intracellular
calcium levels
• Triggered by exposure to volatile
anesthetics and / or succinylcholine
Why is it?

• It’s complicated!
• Uncontrolled release of calcium
by the ryanodine receptor in the
terminal cisternae of the
sarcoplasmic reticulum
Intracellular
hypercalcemia
• Increased calcium release
• Decreased calcium uptake
• Defect in the muscle membrane
• Altered function / structure of
proteins & fatty acids
Trigger agents

• Volatile anesthetic gases (sevo,


des, etc)
• Succinylcholine
Safe drugs in MH

• N20
• Nondepolarizing relaxants (roc,
vec, atracurium etc)
• Local anesthetics
• Narcotics
• Sedative hypnotics (midaz,
propofol etc)
Who gets it?

• 1:50,000 adult GAs


• 1:15,000 pediatric GAs
• Incidence rising due to better
awareness but mortality
declining (overall 10%)
• Inherited as autosomal
dominant with variable
penetrance
Conditions associated
with MH
• Central core disease
• Myotonia flutuans
• King or King-Denborough
myopathy
• Osteogenesis imperfecta
• Heat / exercise syndromes?
Other conditions

• Muscular dystrophy (hyperkalemia


after sux but not MH)
• Pheochromocytoma & thyrotoxicosis
show hypermetabolism but not MH
• Neuroleptic malignant syndrome
(related to phenothiazine exposure
over long term causing dopamine
depletion)
How to recognize it?

• Classically presents in OR
• Tachycardia
• Tachypnea
• Hypertension
• Arrhythmias
• Rigidity
• Hyperthermia
As the crisis develops….

• Rising ET CO2
• CO2 absorbent gets hot
• Skin colour mottled or cyanotic
• Sweating
• Mixed respiratory & metabolic
acidosis
• Elevated K, lactate, myoglobin,
CK
Later…..

• Myoglobinuric renal failure


• DIC
• Death
Variable presentations

• Delay in onset until emergence,


PAR or ward
• Masseter muscle rigidity
• Several uneventful GA’s in the
past, then MH occurs during
current GA
Diagnostic tests

• Caffeine halothane contracture test


is the gold standard (muscle biopsy)
• 5 centres in North America perform
the accepted protocol (Toronto is
only one in Canada listed on MHAUS
website)
• Genetic testing (2 centres in USA
listed on MHAUS website)
Problems with testing

• False negative (had MH after


testing negative)
• More than one gene mutation
may cause MH because only
50% to 80% of MH susceptible
patients have RYR 1 variant or
mutation
How to treat?

• Early diagnosis and treatment


result in very low mortality
• Any location where anesthetics
are administered should have
MH plan & sufficient dantrolene
• MH cart, practice drills, wall
chart with hotline number
MH treatment memory aid
• Some
• Hot
• Dude
• Better
• Give
• Iced
• Fluids
• Fast
Acute phase
• Call for help & get the MH cart /
crash cart
• Declare an emergency
• Stop the triggering agents
• Hyperventilate with high flow 100%
O2
• Switch to non triggering anesthesia
to finish the surgery if needed /
change circuit?
• Dantrolene
Dantrolene

• Need help to mix


• Initial dose 2.5 mg/kg (175 mg)
• Each bottle is 20 mg = 9 bottles for
first dose
• Each bottle mixed with 60 ml H2O =
540 ml water for 9 bottles
• Acts by reducing intracellular
calcium in skeletal muscles
Acute Episode (cont’d)

• Cool patient (goal = 38)


• ABG, lytes results guide further
treatment for metab & resp
acidosis, hyperkalemia
• Arrhythmias respond to
correction of hypercapnia,
hyperkalemia & acidosis
• CK level, myoglobin
Afterwards, monitor
for….
• Recrudescence (25% of
patients)
• DIC
• Myoglobinuric renal failure
In ICU:

• Dantrolene 1mg/kg q6hr x 24-36


hours then orally?
• EKG, art line, urinary catheter,
temperature, 2nd IV line
• CVC?, capnography?
• Monitor & treatment for specific
abnormalities
ICU care (cont’d)

• Refer patient & family to MH


centre for testing
• Recommend registry in MHAUS
data base
• Recommend Medic Alert
bracelet
ABC’s

• A:
• Aware of recrudescence
• Ask relatives anesthesia /
neuromusc disease history
• B:
• Biopsy
• C:
• Contact MHAUS
ABC’s + D

• D:
• Dantrolene 1 mg/kg IV q6h x 24 -
36 hrs
• Documentation to MHAUS
registry
Management of MH
susceptible patient
• Refer to anesthesia consult
clinic
• Prophylaxis with dantrolene?
• Hospital setting vs private
clinic?
• GA vs local / regional?
GA in MH susceptible
• Flush gas machine / remove
vaporizers / new circuit & CO2
absorber
• Monitor ETCO2 & temp
• MH cart ready & nearby
• TIVA with propofol +/- N2O +/-
nondepolarizing NMB + narcotics =
non trigger anesthesia
• Use high fresh gas flow
MH cart

• Dantrolene
• Sterile water (in warming
cupboard?)
• Bicarb, dextrose, CaCl2,
lidocaine
• Insulin (in fridge)
• NS IV bags (in fridge)
• Other stuff
PAR care for MH
susceptible
• Observe for 4 – 6 hours
• May be appropriate for day
surgery if GA was uneventful
Thank you!

You might also like