Malignant Hyperthermia
Kristin Barkley, DNP, CRNA
Quick Facts
Clinical Progression
Signs
Procedure
Workstation Preparation
Differential Diagnosis
Scope Guide
Clinical Progression
Signs
Procedure
Workstation Preparation
Differential Diagnosis
Scope Guide
Quick Facts
- What: Malignant Hyperthermia (MH) is a rare, life-threatening hypermetabolic crisis triggered by certain anesthetic agents.
- How: Autosomal dominant genetic disorder of the RYR1 receptor gene. Defective skeletal muscle calcium channels allow uncontrolled calcium release from the sarcoplasmic reticulum, causing sustained muscle contraction and hypermetabolism.
- Triggers: Volatile anesthetic gases and succinylcholine.
- Who: Occurs in genetically susceptible patients (may present despite prior uneventful anesthetics). Over 80 genetic defects identified; associated with:
- King Denborough syndrome (congenital myopathy)
- Central core disease (skeletal muscle weakness)
- Carnitine palmitoyl transferase deficiency
- Muscular dystrophies (mimic MH, not true crisis — avoid succinylcholine)
- When: MH may occur immediately after trigger administration or several hours later.
- Incidence: ~1:100,000 adult anesthetics.
Clinical Progression
- Excessive calcium release → sustained muscle rigidity
- Hypermetabolic state → increased heat production
- Marked increase in CO₂ production → metabolic & respiratory acidosis
- Increased O₂ consumption, tachycardia, tachypnea, sweating
- Muscle breakdown → rhabdomyolysis:
- Myoglobinemia/Myoglobinuria → acute renal failure
- Hyperkalemia → arrhythmias
- Elevated creatine kinase
Signs
Early Signs
- Increased ETCO₂ (2–3x normal)
- Masseter muscle spasm (after succinylcholine)
- Tachycardia
- Generalized muscle rigidity
- Tachypnea
- Profuse sweating
Late Signs
- Rapidly rising temperature (1–2°C every 5 minutes)
- Dark urine (myoglobinuria)
- Skin mottling, cyanosis, hypoxemia
Procedure
Treatment Mnemonic: “Some Hot Dude Better Get Iced”
- S – Stop triggering agents
- Call for help; bring MH cart
- Convert to TIVA if needed
- H – Hyperventilate
- High fresh gas flow with 100% O₂
- Increase minute ventilation
- Add activated charcoal filters if available (replace hourly)
- D – Dantrolene (RYR1 antagonist)
- Initial dose: 2.5 mg/kg IV (up to 10 mg/kg as needed)
- Mix 20 mg vial with 60 ml sterile water until clear
- Ryanodex: 250 mg/vial, mix with 5 ml sterile water
- Continue dosing until symptoms resolve
- Blocks further calcium release (not neuromuscular transmission)
- B – Bicarbonate
- Treat metabolic acidosis: Sodium Bicarbonate 1–2 mEq/kg IV
- G – Glucose & Insulin
- Treat hyperkalemia
- Calcium chloride may also be given (avoid calcium channel blockers)
- I – Iced
- Active cooling if temperature >39°C
- Cool IV fluids, ice packs (groin, axilla, neck), cooling blanket
- Lavage stomach, bladder, or open cavities with cold saline
- Stop cooling when patient reaches 38°C
Other Treatment Measures
- Treat arrhythmias (beta blockers, lidocaine; avoid calcium channel blockers)
- Draw labs: ABG, electrolytes, CK, urine myoglobin, coagulation panel, LDH
- Insert Foley → monitor UOP (if <0.5 ml/kg/hr, give Lasix for diuresis)
- Secure extra IV access; consider central line & arterial line
- Insert central temperature probe
Post-Event
- Admit to ICU for ≥24 hours
- Ongoing dantrolene therapy, close monitoring, and labs
Prep Anesthesia Workstation
- Remove vaporizers (or tape off).
- Place new circuit and bag.
- Flush anesthesia machine:
- High fresh gas flow (10 L/min O₂/air), vent on, TV 700, RR 12
- New machines: ~104 min; older: ~20 min
- GE machines:
- Aisys – 35 min
- Aestiva – 40 min
- Avance/Aespire – 30 min
- Charcoal filters:
- High FGF for 90 sec before filters placed
- Apply to inspiratory and expiratory limbs
- Change out circuit and CO₂ absorber after flush.
- Flush 10 sec before connecting patient.
Differential Diagnosis
- Neuroleptic malignant syndrome
- Serotonin syndrome
- Light plane anesthesia
- Pheochromocytoma
- Thyroid storm
- Hypoventilation / CO₂ insufflation
Tip: All may cause hypermetabolic signs; not all present with rigidity, fever, rhabdomyolysis. When in doubt, call the MH Hotline.
SCOPe Guide
Clinical Optimization
- Early recognition is critical — rising ETCO₂ is the most sensitive sign.
- Temperature rise is late.
- Call for help early; grab MH cart.
Pearls
- Remember mnemonic: Some Hot Dude Better Get Iced
- Stop triggering agents immediately, give dantrolene promptly.
- Use sterile water only for mixing dantrolene or Ryanodex.
Resources
- MHAUS: www.mhaus.org
- MHAUS Hotline: 1-800-644-9737
- GE Healthcare Guidance on MH Preparation
References
- Malignant Hyperthermia Association of the United States (MHAUS). Available at: https://www.mhaus.org.
- GE Healthcare. Preparing Anesthesia Systems for MH-Susceptible Patients. 2021. Available at: <a href=”https://www.gehealthcare.com/-/media/b19b04665517