MH

Dr. Henry Rosenberg

Introduction
Question patients about MH:
o Unexpected death
o Waking up packed in ice
o Readmission to hosp with signs of muscle breakdown after anesthesia
o Pain
o Brown or cola colored urine without exposure to anesthesia
25% will experience recrudescence
must be in icu for 36 hrs
children/siblings 50/50 chance
It is not wrong to admin dantrolene before definitive diagnosis
Better to give early
If MH develops
o Prepare dantrolene
o Obtain blood specimens
o Cool the patient if hyperthermic
S/s
o Muscle rigidity (mild)
o Increased HR (gradual increase)
o Rapidly elevating ETCO2 (or gradual increase)
o Heart rate irregularity
o Rapidly rising body temp
Once a patient enters the rapidly progressive stage:
o Sign accelerate
o Life threatening events within minutes
Only a few moments to begin treatment with dantrolene
Dantrolene must be available within 5 minutes

What is MH
Inherited disorder
Underlying problem: hypermetabolism
o Increased HR, RR and depth, body temp, sweating
o Normal body responses do not spin out of control
Acid/base balance is disturbed
K leaks out of cells
Skeletal or voluntary muscle is the site during the crisis
increased metabolism = increased acidity acid leaks into the blood stream lowers pH affects
pumping ability of the heart
Dantrolene
o Halts calcium release
o Ends MH reaction
o Initial dose 2.5 mg/kg quickly IV, may be repeated multiple times until reaction is under
control
o Comes 20 mg dantrolene, 3 grams of mannitol, mix with 60 mL of sterile water
o 1 mg/kg every 4-6 hrs for 24 hrs
may be given longer if CK levels have not decreased

Pt must be observed in ICU for 24 hrs since 25% of patients get MH again
Labs
o ABGs: Acid/base balance must be maintained every 6 hrs
o K must be normal
o Coag studies to see if bleeding
o CK and myoglobin for detecting muscle breakdown every 6 hrs
Consult nephrologist concerning alkalinization of urine
Factors affecting severity:
o Duration, depth, and other drugs involved
May present at any time during the anesthetic even in the post op period
Grading scale:
o CO2 levels
o Muscle breakdown
o Temp elevation
o Increased acidity and creatine kinase
o High score = higher MH likelihood
o Guide not definitive

MH Outside the OR
Occasional reports of people with MH family history dying in stressful situations
Awake MH rare < 20 cases reported in the US
MH presentation
May resemble other syndromes
May develop immediately or gradually
if signs go unrecognized:
o rapid decompensation resulting in hyperthermia, hypercarbia, acidosis, cardiac arrest
Masseter rigidity (MMR) may be an important warning sign
o Jaw tension or jaws of steel after succs
o Transient lasts less than 5 mins
Discontinue elective surgery
Switch to non triggering tech begin MH therapy
Generalized rigidity: MH assured
Most pts will develop rhabdo and myoglobinuria
Pt hospitalized for 24 hrs
Increased RR secondary to hypercarbia
Hyperkalemia manifested by arrhythmia, peaked T waves immediately treat with insulin,
O2 desat is unusual unless O2 stores are used up from hypermetabolism
Mottling skin due to high levels of catacholamines
Resemble
o Sepsis- difficult to differentiate from MH (appendectomy example)
o Rapid absorption of CO2 during laparoscopic procedures
o Masseter muscle tightness with succs- increased muscle tone mistaken for jaws of steel
o Equipment issues- stuck flow valve allowing rebreathing to occur, misplaced LMA
o Hyperthyroidism- muscle rigidity is not observed
o Rhabdo- occurs in postop period, muscle pressure with vascular compromise
o Cardiac arrest in a young male patient: hyperkalemia should be suspected and treated,
duchenne muscular dystrophy
o Stimulants prior to anesthesia: tachycardia, HTN

The post acute phase: ICU, predetermine MH transfer protocol, see dantrolene stuff above

MH and muscle disorders

Disorder of skeletal muscle
1 in 2000
1 in 15000 50000 anesthetics
Mortality 5%
Usual victim is young and healthy
Incidence higher in males
Mortality 20% higher when episode takes place out of a hospital
MH mortality
o Age
o gender
o concomitant drug admin
o Comorbidities
Pretreatment with dantrolene is not recommended
Risk of complications 1.6 fold per 3 mins delayed
Mixing dantrolene
Diluent: sterile water ONLY 60 mL
Rapid onset less than 10 mins
Want to see a decrease in ETCO2
If muscle rigidity is present, should go away
HR should decrease and acidosis should resolve
Repeat doses until the desired effects
If no improvement, consider other causes of hypercarbia
If mix with saline undesirable results orange cloudy solution with insoluble particles, amt of
dantrolene soln per ml is reduced by 40%

MH cart

Have at least 36 vials of dantrolene available

Drugs
o Therapy should be aimed at prompt treatment of hyperkalemia, administration of
dantrolene, hyperventilation, and cooling to target core temperature 38C.
o Dantrolene 36 vials should be available in each institution where MH can occur, each to be
diluted at the time of use with 60 ml sterile water for injection USP (without a bacteriostatic
agent).
o Sterile water for injection USP (without a bacteriostatic agent) Each vial of dantrolene
should be reconstituted by adding 60 ml of sterile water for injection USP (without a
bacteriostatic agent) and the vial shaken until the solution is clear. If the MH episode is
proceeding rapidly, simply mix and inject. It is mandatory to get dantrolene to its effective
site, the skeletal muscle. We advise that the sterile water be stored in 100 ml vials, not bags,
to avoid accidental IV administration of this hypotonic solution.
Do not use cold water
o Sodium bicarbonate (8.4%) 50 ml x 5
o For treatment of hyperkalemia
Dextrose 50% 50 ml vials x 2
Calcium chloride (10%) 10 ml vial x 2

 Regular insulin 100 units/ml x 1 (refrigerated)

Lidocaine* for injection (2%) 100 mg/5 ml or 100 mg/10 ml in preloaded syringes (3).
Amiodarone (3 mL vial x 3) is also acceptable. ACLS protocols, as prescribed by the AHA,
would be followed when treating all cardiac derangements caused by MH.
o Lasix 40 mg per
o Refrigerated cold saline solution A minimum of 3,000 ml of for IV cooling
o * Lidocaine or procainamide should not be given if a wide-QRS complex arrhythmia is likely
due to hyper-kalemia; this may result in asystole.
General Equipment
o Syringes - (60 ml x 5) to dilute dantrolene
o Intravenous catheters - 16G, 18G, 20G, 2-inch; 22G, 1-inch; 24G, 3/4-inch (4 each) (for IV
access and arterial line)
o NG tubes - (sizes appropriate for your patient population)
o Toomy irrigation syringes - (60 ml x 2) with adapter for NG irrigation
Monitoring Equipment
o Esophageal or other core (e.g., nasopharyngeal, tympanic membrane, rectal, bladder,
pulmonary artery catheter) temperature probes
o CVP kits (sizes appropriate to your patient population)
o Transducer kits for arterial and central venous cannulation
Nursing Supplies
o Large sterile Steri-Drape (for rapid drape of wound)
o Urine meter x 1
o Irrigation tray with piston (60cc irrigation) syringe
o Large clear plastic bags for ice x 4
o Small plastic bags for ice x 4
o Bucket for ice
o Test strips for urine analysis
o Ten 60 mL Luer lock syringes
o Toomy irrigation syringes for NGT w/ cold 0.9 NS
o Temp monitors (do not rely on skin temp)
o Blood pump infusion set
Laboratory Testing Supplies
o Syringes (3 ml) for blood gas analysis or ABG kits x 6 or point of care monitors; ISTAT with
TB syringes (the point of care ISTAT device has replaced lab blood gene and electrolyte
measurement).
o Blood specimen tubes for CK, myoglobin, SMA 19 (LDH, electrolytes, thyroid studies),
PT/PTT, fibrinogen, fibrin split products; and lactate, CBC, platelets. If no
immediate laboratory analysis is available, samples should be kept on ice for later analysis.
This may well prove useful on retrospective review and diagnosis. Blood cultures are very
useful and should be included to rule out bacteremia.
o Urine collection container for myoglobin level. Pigrnenturia (e.g , brown or red urine and
heme positive dipstick) indicates that renal protection is mandated, when the urine is
centrifuged or allowed to settled, and the sample shows clear supernatant, i.e., the
coloration is due to red cells in the sample.
o

Start active cooling if > 38.5

Prepare anesthesia machine:
Ensure that anesthetic vaporizers are disabled by removing, or taping in the OFF position.

Most vaporizers have a significant reservoir of anesthetic that cannot be drained, thus draining
is not an acceptable choice.
Some Hotline consultants recommend changing CO2 absorbent (soda lime or baralyme).
Flow 10 L/min O2 through circuit via the ventilator for at least 20 minutes (refer to the consensus
statement on page 36).
During this time a disposable, unused breathing bag should be attached to the Y-piece of the circle
system and the ventilator set to inflate the bag periodically.
Use new or disposable breathing circuit. Use the expired gas analyzer to confirm absence of volatile
gases, as some newer machines are not so easily cleaned of volatile agents.
Changing the CO2 absorbent (soda lime or baralyme) is not recommended if these procedures are
followed.
Newer anesthesia work stations may require up to 60 minutes for purging residual gases; consult
manufacturers information and information on the MHAUS website.
Adding commercially available charcoal filters to the circuit will remove anesthetic gases and
therefore obviate the need for purging the system as described
However, the filters should be replaced every hour.

Not safe for use in MH-susceptible patients, the following anesthetic agents are known triggers of MH:
Inhaled General Anesthetics
Desflurane
Enflurane
Ether
Halothane
Isoflurane
Methoxyflurane
Sevoflurane
Succinylcholine (warning)
All other anesthetic agents outside of these two categories of Volatile anesthetic agents and depolarizing muscle
relaxants are considered safe.
Safe Anesthetic Agents for MH Patients:
Barbiturates / Intravenous Anesthetics
o Diazepam
o Etomidate (Amidate)
o Hexobarbital
o Ketamine (Ketalar)
o Methohexital (Brevital)
o Midazolam
o Pentobarbital
o Propofol (Diprivan)
o Thiopental (Pentothal)
Inhaled Non-Volatile General Anesthetic
o Nitrous Oxide
Local Anesthetics
o Amethocaine
o Articaine
o Bupivicaine
o Dibucaine

o Etidocaine
o Eucaine
o Lidocaine (Xylocaine)
o Levobupivacaine
o Mepivicaine (Carbocaine)
o Procaine (Novocain)
o Prilocaine (Citanest)
o Ropivacaine
o Stovaine
o Proparacaine Hydrochloride ALCAINE
o (proparacaine hydrochloride ophthalmic solution)
Narcotics (Opioids)
o Alfentanil (Alfenta)
o Anileridine
o Codeine (Methyl Morphine)
o Diamorphine
o Fentanyl (Sublimaze)
o Hydromorphone (Dilaudid)
o Meperidine (Demerol)
o Methadone
o Morphine
o Naloxone
o Oxycodone
o Phenoperidine
o Remifentanil
o Sufentanil (Sufenta)
Safe Muscle Relaxants
o Arduan (Pipecuronium)
o Curare (The active ingredient is Tubocurraine)
o Gallamine
o Metocurine
o Mivacron (Mivacurium)
o Neuromax (Doxacurium)
o Nimbex (Cisatracurium)
o Norcuron (Vecuronium)
o Pavulon (Pancuronium)
o Tracrium (Atracurium)
o Zemuron (Rocuronium)
Anxiety Relieving Medications
o Ativan (Lorazepam)
o Centrax
o Dalmane (Flurazepam)
o Halcion (Triazolam)
o Klonopin
o Librax
o Librium (Chlordiazepoxide)
o Midazolam (Versed)
o Paxil (paroxetine)
o Paxipam (Halazepam)

o
o
o
o

Restoril (Temazepam)
Serax (Oxazepam)
Tranxene (Clorazepate)
Valium (Diazepam)