Malignant hyperthermia: Difference between revisions
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==Background== | ==Background== | ||
* | *Life-threatening hypermetabolic reaction to volatile anesthetic agents or succinylcholine | ||
* | *Caused by uncontrolled skeletal muscle calcium release via ryanodine receptor (RyR1) mutations | ||
* | *Autosomal dominant inheritance with variable penetrance<ref name="rosenberg">Rosenberg H, et al. Malignant hyperthermia: a review. ''Orphanet J Rare Dis''. 2015;10:93. PMID 26238698.</ref> | ||
*Incidence: ~1:5,000 to 1:50,000 anesthetics | |||
*Mortality: <5% with early recognition and dantrolene; historically >70% without treatment | |||
*Can also be triggered by extreme heat and exertion in susceptible individuals (exertional heat stroke variant) | |||
== | ==Triggering Agents== | ||
* | *Volatile inhalational anesthetics: sevoflurane, desflurane, isoflurane, halothane | ||
*[[Succinylcholine]] | |||
* | *Safe agents: propofol, etomidate, ketamine, nitrous oxide, all non-depolarizing paralytics, opioids, benzodiazepines, local anesthetics | ||
* | |||
== | ==Clinical Features== | ||
*Often occurs intraoperatively but can present in the PACU or ED | |||
*Earliest sign: unexplained rise in end-tidal CO2 and tachycardia | |||
*'''Masseter muscle rigidity''' (particularly after succinylcholine) — early warning sign | |||
*Generalized skeletal muscle rigidity | |||
*Rapidly rising temperature (may exceed 40°C; >1°C rise every 5 min) | |||
*Tachycardia, dysrhythmias, unstable blood pressure | |||
*Dark urine ([[Rhabdomyolysis|myoglobinuria]]) | |||
*Metabolic and respiratory acidosis | |||
*[[Hyperkalemia]], elevated CK, myoglobinuria | |||
*Late: [[DIC]], [[Acute kidney injury|renal failure]], cardiac arrest | |||
==Diagnosis== | ==Differential Diagnosis== | ||
*[[Neuroleptic malignant syndrome]] (NMS) — antipsychotics, slower onset (days) | |||
*[[Serotonin syndrome]] — serotonergic drugs, clonus prominent | |||
*[[Heat stroke]] — environmental exposure | |||
*Thyroid storm | |||
*Pheochromocytoma crisis | |||
*Sepsis | |||
*Drug-induced hyperthermia (cocaine, amphetamines, MDMA) | |||
==Evaluation== | |||
*Elevated and rapidly rising end-tidal CO2 | |||
*ABG: combined respiratory and metabolic acidosis | |||
*BMP: [[Hyperkalemia|hyperkalemia]], hypercalcemia | |||
*CK: markedly elevated (often >10,000 U/L) | |||
*Coagulation studies: may show DIC | |||
*Urinalysis: myoglobinuria | |||
*Core temperature monitoring | |||
*Definitive diagnosis: caffeine-halothane contracture test (done later, not acutely) | |||
=== | ==Management== | ||
* | ===Immediate=== | ||
* | *'''STOP all triggering agents immediately''' | ||
* | *Call for Malignant Hyperthermia Hotline: 1-800-644-9737 (MHAUS) | ||
*Hyperventilate with 100% O2 at high fresh gas flows | |||
*Change anesthesia circuit and CO2 absorber if possible | |||
=== | ===Dantrolene=== | ||
*[[Dantrolene]] 2.5 mg/kg IV bolus, repeat every 5-10 minutes until symptoms resolve<ref name="glahn">Glahn KP, et al. Recognizing and managing a malignant hyperthermia crisis. ''Br J Anaesth''. 2010;105(4):417-420. PMID 20837722.</ref> | |||
*Maximum total dose: up to 10 mg/kg (no absolute ceiling if still symptomatic) | |||
*Reconstitute with sterile water (each 20 mg vial in 60 mL) — time-consuming; assign dedicated team | |||
*Newer formulation (Ryanodex): 2.5 mg/kg in single vial, faster to prepare | |||
*Continue dantrolene 1 mg/kg IV q4-6h for 24-48 hours to prevent recrudescence | |||
== | ===Cooling=== | ||
*Aggressive active cooling: ice packs to axillae/groin, cooling blankets, cold IV saline | |||
*Target temperature <38.5°C | |||
*Avoid overcooling | |||
== | ===Supportive=== | ||
*Treat [[Hyperkalemia]]: calcium gluconate, insulin + glucose, sodium bicarbonate | |||
*IV fluids to maintain urine output >2 mL/kg/hr (prevent myoglobin-induced AKI) | |||
*Treat dysrhythmias (avoid calcium channel blockers with dantrolene — risk of hyperkalemia) | |||
*Monitor for [[DIC]], [[Rhabdomyolysis|rhabdomyolysis]], [[Compartment syndrome]] | |||
== | ==Disposition== | ||
*ICU admission for all MH episodes | |||
*Monitor for recrudescence (recurrence in 25% within 24 hours) | |||
*Genetic counseling and testing for patient and family | |||
=== | ==See Also== | ||
*[[Neuroleptic malignant syndrome]] | |||
*[[Serotonin syndrome]] | |||
*[[Heat stroke]] | |||
*[[Rhabdomyolysis]] | |||
== | ==References== | ||
<references/> | |||
[[Category: | [[Category:Critical Care]] | ||
[[Category: | [[Category:Pharmacology]] | ||
Latest revision as of 09:12, 22 March 2026
Background
- Life-threatening hypermetabolic reaction to volatile anesthetic agents or succinylcholine
- Caused by uncontrolled skeletal muscle calcium release via ryanodine receptor (RyR1) mutations
- Autosomal dominant inheritance with variable penetrance[1]
- Incidence: ~1:5,000 to 1:50,000 anesthetics
- Mortality: <5% with early recognition and dantrolene; historically >70% without treatment
- Can also be triggered by extreme heat and exertion in susceptible individuals (exertional heat stroke variant)
Triggering Agents
- Volatile inhalational anesthetics: sevoflurane, desflurane, isoflurane, halothane
- Succinylcholine
- Safe agents: propofol, etomidate, ketamine, nitrous oxide, all non-depolarizing paralytics, opioids, benzodiazepines, local anesthetics
Clinical Features
- Often occurs intraoperatively but can present in the PACU or ED
- Earliest sign: unexplained rise in end-tidal CO2 and tachycardia
- Masseter muscle rigidity (particularly after succinylcholine) — early warning sign
- Generalized skeletal muscle rigidity
- Rapidly rising temperature (may exceed 40°C; >1°C rise every 5 min)
- Tachycardia, dysrhythmias, unstable blood pressure
- Dark urine (myoglobinuria)
- Metabolic and respiratory acidosis
- Hyperkalemia, elevated CK, myoglobinuria
- Late: DIC, renal failure, cardiac arrest
Differential Diagnosis
- Neuroleptic malignant syndrome (NMS) — antipsychotics, slower onset (days)
- Serotonin syndrome — serotonergic drugs, clonus prominent
- Heat stroke — environmental exposure
- Thyroid storm
- Pheochromocytoma crisis
- Sepsis
- Drug-induced hyperthermia (cocaine, amphetamines, MDMA)
Evaluation
- Elevated and rapidly rising end-tidal CO2
- ABG: combined respiratory and metabolic acidosis
- BMP: hyperkalemia, hypercalcemia
- CK: markedly elevated (often >10,000 U/L)
- Coagulation studies: may show DIC
- Urinalysis: myoglobinuria
- Core temperature monitoring
- Definitive diagnosis: caffeine-halothane contracture test (done later, not acutely)
Management
Immediate
- STOP all triggering agents immediately
- Call for Malignant Hyperthermia Hotline: 1-800-644-9737 (MHAUS)
- Hyperventilate with 100% O2 at high fresh gas flows
- Change anesthesia circuit and CO2 absorber if possible
Dantrolene
- Dantrolene 2.5 mg/kg IV bolus, repeat every 5-10 minutes until symptoms resolve[2]
- Maximum total dose: up to 10 mg/kg (no absolute ceiling if still symptomatic)
- Reconstitute with sterile water (each 20 mg vial in 60 mL) — time-consuming; assign dedicated team
- Newer formulation (Ryanodex): 2.5 mg/kg in single vial, faster to prepare
- Continue dantrolene 1 mg/kg IV q4-6h for 24-48 hours to prevent recrudescence
Cooling
- Aggressive active cooling: ice packs to axillae/groin, cooling blankets, cold IV saline
- Target temperature <38.5°C
- Avoid overcooling
Supportive
- Treat Hyperkalemia: calcium gluconate, insulin + glucose, sodium bicarbonate
- IV fluids to maintain urine output >2 mL/kg/hr (prevent myoglobin-induced AKI)
- Treat dysrhythmias (avoid calcium channel blockers with dantrolene — risk of hyperkalemia)
- Monitor for DIC, rhabdomyolysis, Compartment syndrome
Disposition
- ICU admission for all MH episodes
- Monitor for recrudescence (recurrence in 25% within 24 hours)
- Genetic counseling and testing for patient and family