Malignant hyperthermia: Clinical sciences

Last updated: January 30, 2025

Malignant hyperthermia: Clinical sciences

CCRN Prep Total

CCRN Prep Total

Anatomic and physiologic dead space
Ventilation
Ventilation-perfusion ratios and V/Q mismatch
Gas exchange in the lungs, blood and tissues
Approach to a cough (pediatrics): Clinical sciences
Reading a chest X-ray
Approach to respiratory distress (newborn): Clinical sciences
Approach to chest pain: Clinical sciences
Acute respiratory distress syndrome
Respiratory distress syndrome: Pathology review
Respiratory failure (pediatrics): Clinical sciences
Acute respiratory distress syndrome: Clinical sciences
Approach to postoperative respiratory distress: Clinical sciences
Approach to dyspnea: Clinical sciences
Upper respiratory tract infection
Apnea of prematurity
Approach to complications of prematurity (early): Clinical sciences
Apnea, hypoventilation and pulmonary hypertension: Pathology review
Hospital-acquired and ventilator-associated pneumonia: Clinical sciences
Acid-base map and compensatory mechanisms
Respiratory acidosis
Approach to respiratory alkalosis: Clinical sciences
Approach to lower airway obstruction (pediatrics): Clinical sciences
Approach to upper airway obstruction (pediatrics): Clinical sciences
Croup and epiglottitis: Clinical sciences
Croup
Pharyngitis, peritonsillar abscess, and retropharyngeal abscess (pediatrics): Clinical sciences
Asthma: Clinical sciences
Bronchodilators: Beta 2-agonists and muscarinic antagonists
Pneumonia: Pathology review
Pneumothorax
Pneumothorax: Clinical sciences
Pleural effusion, pneumothorax, hemothorax and atelectasis: Pathology review
Atelectasis: Clinical sciences
Approach to penetrating chest injury: Clinical sciences
Pulmonary embolism
Pulmonary embolism: Clinical sciences
Pulmonary shunts
Pulmonary hypertension
Pulmonary hypertension: Clinical sciences
Hypertension
Hypertensive emergency
Hypertension: Pathology review
Tracheoesophageal fistula
Esophageal atresia and tracheoesophageal fistula: Year of the Zebra
Bronchiolitis: Clinical sciences
Blood transfusion reactions and transplant rejection: Pathology review
Spinal fractures: Clinical sciences
Anatomy of the descending spinal cord pathways
Approach to differentiating lesions (spinal cord): Clinical sciences
Brain death: Clinical sciences
Pneumonia (pediatrics): Clinical sciences
Brain herniation
Pediatric brain tumors
Delirium
Delirium: Clinical sciences
Approach to encephalopathy (acute and subacute): Clinical sciences
Encephalitis
Approach to altered mental status: Clinical sciences
Approach to traumatic brain injury: Clinical sciences
Approach to traumatic brain injury (pediatrics): Clinical sciences
Traumatic brain injury: Pathology review
Epidural hematoma
Approach to trauma (pediatrics): Clinical sciences
Concussion and traumatic brain injury
Subarachnoid hemorrhage: Clinical sciences
Normal pressure hydrocephalus
Intracerebral hemorrhage
Approach to increased intracranial pressure: Clinical sciences
Subarachnoid hemorrhage
Neurogenic shock: Clinical sciences
Approach to shock (pediatrics): Clinical sciences
Shock: Pathology review
Shock
Approach to shock: Clinical sciences
Ischemic stroke
Acute stroke (ischemic or hemorrhagic) or TIA: Clinical sciences
Cerebral vascular disease: Pathology review
Arteriovenous malformation
Meningitis
Pelvic fractures: Clinical sciences
Subdural hematoma
Community-acquired pneumonia: Clinical sciences
Meningitis (pediatrics): Clinical sciences
Meningitis and brain abscess: Clinical sciences
Central nervous system infections: Pathology review
Syndrome of inappropriate antidiuretic hormone secretion: Clinical sciences
Approach to convulsive status epilepticus: Clinical sciences
Seizures and epilepsy
Approach to epilepsy: Clinical sciences
Approach to altered mental status (pediatrics): Clinical sciences
Nonbenzodiazepine anticonvulsants
Seizures: Pathology review
Spina bifida
Congenital neurological disorders: Pathology review
Electrolyte disturbances: Pathology review
Hyperosmolar hyperglycemic state: Clinical sciences
Compartment syndrome: Clinical sciences
Renal system anatomy and physiology
Intrinsic acute kidney injury (glomerular causes): Clinical sciences
Prerenal acute kidney injury: Clinical sciences
Prerenal azotemia
Intrinsic acute kidney injury (non-glomerular causes): Clinical sciences
Postrenal acute kidney injury: Clinical sciences
Approach to acute kidney injury: Clinical sciences
Approach to postoperative acute kidney injury: Clinical sciences
Renal failure: Pathology review
Chronic kidney disease
Chronic kidney disease: Clinical sciences
Nephrotic syndromes: Pathology review
Approach to hyperkalemia: Clinical sciences
Transplant rejection
Nephritic syndromes (pediatrics): Clinical sciences
The role of the kidney in acid-base balance
Urinary tract infections and kidney stones in pregnancy: Clinical sciences
Hemolytic-uremic syndrome
Approach to bleeding disorders (thrombocytopenia): Clinical sciences
Extrinsic hemolytic normocytic anemia: Pathology review
Thrombotic microangiopathy: Clinical sciences
Platelet disorders: Pathology review
Approach to blunt and penetrating abdominal injury: Clinical sciences
Approach to postoperative abdominal pain: Clinical sciences
Approach to acute abdominal pain (pediatrics): Clinical sciences
Non-accidental trauma and neglect (pediatrics): Clinical sciences
Small bowel ischemia and infarction
Bowel obstruction
Large bowel obstruction: Clinical sciences
Small bowel obstruction: Clinical sciences
Short bowel syndrome: Clinical sciences
Gastrointestinal bleeding: Pathology review
Hypovolemic shock: Clinical sciences
Congenital gastrointestinal disorders: Pathology review
Approach to bleeding disorders (platelet dysfunction): Clinical sciences
Cholestatic liver disease
Non-alcoholic fatty liver disease
Post-transplant lymphoproliferative disorders (NORD)
Transposition of the great vessels
Intussusception
Intussusception: Clinical sciences
Approach to the acute abdomen (pediatrics): Clinical sciences
Vasculitis: Pathology review
Necrotizing enterocolitis: Clinical sciences
Necrotizing enterocolitis: Year of the Zebra 2024
Guillain-Barré syndrome: Clinical sciences
Disseminated intravascular coagulation: Clinical sciences
Disseminated intravascular coagulation
Consumptive coagulopathy from massive transfusion: Clinical sciences
Sepsis: Clinical sciences
Approach to leukemia: Clinical sciences
Thrombosis syndromes (hypercoagulability): Pathology review
Malignant hyperthermia: Clinical sciences
Acute pancreatitis
Adrenal insufficiency: Pathology review
Deep vein thrombosis and pulmonary embolism: Pathology review
Immune thrombocytopenia
Immune thrombocytopenia: Clinical sciences
Hematopoietic medications
Glucocorticoids
Sickle cell disease: Clinical sciences
Anatomy clinical correlates: Spinal cord pathways
Acute coronary syndrome: Clinical sciences
Antidiuretic hormone
Diabetes insipidus and SIADH: Pathology review
Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
Hyponatremia
Approach to hyponatremia: Clinical sciences
Approach to hyponatremia (pediatrics): Clinical sciences
Diabetes insipidus
Diabetes insipidus: Clinical sciences
Approach to hypoglycemia: Clinical sciences
Approach to hypoglycemia (pediatrics): Clinical sciences
Diabetic ketoacidosis: Clinical sciences
Diabetes mellitus (pediatrics): Clinical sciences
Diabetes mellitus: Pathology review
Pulmonary edema
Cerebral palsy
Hepatic encephalopathy: Clinical sciences
Approach to common musculoskeletal injuries (pediatrics): Clinical sciences
Approach to blunt chest injury: Clinical sciences
Pediatric musculoskeletal disorders: Pathology review
Approach to extremity injury: Clinical sciences
Neuroblastoma
Childhood and early-onset psychological disorders: Pathology review
Approach to trauma: Clinical sciences
Anatomy clinical correlates: Skull, face and scalp
Rhabdomyolysis
Compartment syndrome
Hypocalcemia
Hyperphosphatemia
Hyperkalemia
Sepsis (pediatrics): Clinical sciences
Sepsis
Neonatal sepsis
Empyema: Clinical sciences
Necrotizing soft tissue infections: Clinical sciences
Pressure-induced skin and soft tissue injury: Clinical sciences
Diffusion-limited and perfusion-limited gas exchange
Approach to acid-base disorders: Clinical sciences
Definitions of acids and bases
Acid-base disturbances: Pathology review
Catheter-associated urinary tract infection: Clinical sciences
Central line-associated bloodstream infection: Clinical sciences
Approach to medication exposure (pediatrics): Clinical sciences
Approach to household substance exposure (pediatrics): Clinical sciences
Approach to recreational substance exposure (pediatrics): Clinical sciences
Myocarditis: Clinical sciences
Pharmacodynamics: Drug-receptor interactions
Medication overdoses and toxicities: Pathology review
Opioid intoxication and overdose: Clinical sciences
Approach to stimulant use, intoxication, and overdose: Clinical sciences
Approach to hallucinogen, inhalant, and cannabis use, intoxication, and overdose: Clinical sciences
Cholinomimetics: Indirect agonists (anticholinesterases)
Suicide
Burns
Burns: Clinical sciences
Multiple organ dysfunction syndrome (MODS): Clinical sciences
Kawasaki disease
Approach to hypernatremia (pediatrics): Clinical sciences
Approach to a postoperative fever: Clinical sciences
Supraventricular arrhythmias: Pathology review
Aspiration pneumonia and pneumonitis: Clinical sciences
Cardiac preload
Cardiac cycle
Cardiac tumors
Cardiac work
Cardiac tamponade
Cardiac tamponade: Clinical sciences
Cardiac conduction velocity
Cardiac afterload
Cardiac contractility
ECG cardiac hypertrophy and enlargement
Ventricular tachycardia: Clinical sciences
Ventricular arrhythmias: Pathology review
ECG cardiac infarction and ischemia
Approach to tachycardia: Clinical sciences
Stroke volume, ejection fraction, and cardiac output
Dilated cardiomyopathy
Supraventricular tachycardia: Clinical sciences
Class IV antiarrhythmics: Calcium channel blockers and others
Atrial fibrillation and atrial flutter: Clinical sciences
Positive inotropic medications
Class I antiarrhythmics: Sodium channel blockers
Cardiomyopathies: Pathology review
Class III antiarrhythmics: Potassium channel blockers
Hypertrophic cardiomyopathy
Ventricular fibrillation
Aortic stenosis: Clinical sciences
Myocarditis
Brief, resolved, unexplained event (BRUE): Clinical sciences
Mitral stenosis: Clinical sciences
Congestive heart failure: Clinical sciences
Atrial flutter
Pressures in the cardiovascular system
Cardiovascular system anatomy and physiology
Restrictive cardiomyopathy
Airflow, pressure, and resistance
Total anomalous pulmonary venous return
Atrial fibrillation
Hypertrophic cardiomyopathy: Clinical sciences
Hypothermia: Clinical sciences
Hemothorax: Clinical sciences
Anaphylaxis: Clinical sciences
Abdominal aortic aneurysm: Clinical sciences
Muscarinic antagonists
Selective serotonin reuptake inhibitors
General anesthetics
Neuromuscular blockers
Right heart failure: Clinical sciences
Heart failure: Pathology review
Mitral valve disease
Approach to a murmur (pediatrics): Clinical sciences
Tricuspid valve disease
ACE inhibitors, ARBs and direct renin inhibitors
Patent ductus arteriosus
Adrenergic antagonists: Beta blockers
Pheochromocytoma
cGMP mediated smooth muscle vasodilators
Cardiac conduction system
Hypoplastic left heart syndrome
Hypoplastic left heart syndrome: Year of the Zebra 2024
Heart blocks: Pathology review
Rheumatic heart disease
Abnormal heart sounds
Valvular heart disease: Pathology review
Coronary artery disease: Pathology review
Pericarditis: Clinical sciences
Approach to hypertension: Clinical sciences
Deep vein thrombosis
Deep vein thrombosis: Clinical sciences
Approach to a fever: Clinical sciences
Anticoagulants: Heparin
Approach to hypercoagulable disorders: Clinical sciences
Heparin-induced thrombocytopenia
Thrombolytics
Atrial septal defect
Superior vena cava syndrome
Introduction to the somatic and autonomic nervous systems
Anticonvulsants and anxiolytics: Benzodiazepines
Anticonvulsants and anxiolytics: Barbiturates
Approach to congenital heart diseases (acyanotic): Clinical sciences
Tetralogy of Fallot
Cyanotic congenital heart defects: Pathology review
Approach to congenital heart diseases (cyanotic): Clinical sciences
Ventricular septal defect
Aortic valve disease
Pyloric stenosis
Aortic dissection
Pneumonia
Aortic dissection: Clinical sciences
Aortic dissections and aneurysms: Pathology review
Coarctation of the aorta
Acyanotic congenital heart defects: Pathology review
Pulmonary valve disease
Pulmonary chemoreceptors and mechanoreceptors
Zones of pulmonary blood flow
Carotid artery stenosis screening: Clinical sciences
Endocarditis
Endocarditis: Pathology review
Valvular insufficiency (regurgitation): Clinical sciences
Infectious endocarditis: Clinical sciences
Choanal atresia
Tetralogy of Fallot: Year of the Zebra
Mycoplasma pneumoniae
Measles virus
Respiratory alkalosis
Metabolic alkalosis
Approach to metabolic alkalosis: Clinical sciences
Approach to respiratory acidosis: Clinical sciences
Metabolic acidosis
Approach to metabolic acidosis: Clinical sciences
Pericardial disease: Pathology review
Atherosclerosis and arteriosclerosis: Pathology review
Cardiac and vascular tumors: Pathology review
Peripheral artery disease: Pathology review

Decision-Making Tree

Transcript

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Malignant hyperthermia is a serious anesthetic complication that must be emergently recognized and treated. It can develop in susceptible individuals who have a genetic mutation in their dihydropyridine or ryanodine receptors, which normally regulate skeletal muscle contraction by controlling calcium release from the sarcoplasmic reticulum. When these individuals are exposed to a volatile anesthetic like isoflurane or sevoflurane, or less commonly, to the neuromuscular blocking agent succinylcholine, there is an unregulated calcium release in the muscle cells. As a result, intracellular calcium overload causes sustained muscle contraction and breakdown, leading to a hypermetabolic state called malignant hyperthermia.

Alright, malignant hyperthermia typically develops while a patient is getting anesthesia, or less commonly within one hour of stopping the anesthetic. The first step in approaching someone suspected to have malignant hyperthermia is to assess their ventilatory status and vitals. To assess the ventilatory status, check the respiratory rate and see if it matches the ventilator settings. Next, look for hypercarbia, which is the most common presenting sign of malignant hyperthermia. Hypercarbia is an unexplained increase in exhaled CO2 caused by widespread muscle contraction and hypermetabolism despite adequate ventilation. Additionally, be sure to rule out other causes of hypercarbia, such as hypoventilation, rebreathing, or gas absorption during laparoscopy. Finally, check the vital signs for tachypnea, tachycardia, and hyperthermia, which might occur later in the course of malignant hyperthermia.

After checking the vitals, you should obtain a brief physical examination. A physical exam might show signs of hyperthermia, such as warmth and sweating. Individuals might also exhibit generalized muscle rigidity due to widespread skeletal muscle contraction. Less often, this rigidity is confined to the masseter muscles, leading to jaw tightness and airway compromise if the airway has not been secured.

Finally, after a brief physical examination, you should obtain a 12-lead ECG. When it comes to ECG, it usually shows sinus tachycardia. However, as muscle cells break down, they may release intracellular contents like potassium, and the patient might develop hyperkalemia. So, be on the lookout for ECG changes related to hyperkalemia, such as peaked T waves, wide QRS complexes, and ventricular arrhythmias.

If signs point to malignant hyperthermia, check the history for any risk factors. Since malignant hyperthermia usually occurs during anesthesia, the patient is already sedated, so you’ll have to obtain the history from other members of the clinical team or the patient’s health record. You should determine whether the patient received any volatile anesthetics or succinylcholine; and then, determine when the medication was administered.

Now, here are some high-yield facts to keep in mind! A major risk factor for malignant hyperthermia is any family history of known malignant hyperthermia or poor reactions to anesthesia, since the condition is genetic. However, most people who develop malignant hyperthermia will have no known family history of this condition, so even a negative family history can’t rule it out. In addition, keep in mind that some medications can mask the signs and symptoms of malignant hyperthermia. For instance, beta blockers can mask tachycardia, sedatives block respiratory drive and tachypnea, while neuromuscular blocking agents prevent being able to see the rigidity. Thus, if the patient is on one of these medications, a high index of suspicion is important to not miss malignant hyperthermia.

Alright, if there are signs of hypermetabolism in a patient who received one of the common triggering medications, you can suspect malignant hyperthermia. Due to the severity and rapid progression of this condition, you should start acute management right away. First, administer dantrolene intravenously as soon as it’s available. This is the only known antidote for malignant hyperthermia, acting on ryanodine receptors to inhibit calcium release and muscle contraction. Next, stop the offending agent, or if it’s necessary to continue the procedure, switch to a non-triggering anesthetic. This should involve a discussion with the intraoperative team, but in general, elective procedures should be safely terminated, while non-elective procedures should be completed as quickly as possible.

Then, hyperventilate the patient to remove excess CO2, and increase the oxygen flow rate and the fraction of inspired oxygen, or FiO2. If not already present, place an endotracheal tube to secure the airway, as well as an arterial line and a Foley catheter. If the person’s core temperature exceeds 39 degrees celsius, initiate cooling with the goal of normothermia, meaning 38 degrees or less. Lastly, if the person has an arrhythmia, follow ACLS resuscitation protocols, but be sure to avoid calcium channel blockers, which can be harmful in malignant hyperthermia. Because of the high risk of hyperkalemia, you should stabilize related arrhythmias by administering calcium gluconate or calcium chloride, as well as insulin plus glucose, and sodium bicarbonate.

Sources

  1. "New October 2018 Malignant Hyperthermia Guidelines: Is Your Emergency Department Prepared?" J Emerg Nurs (2019)
  2. "Junctional membrane Ca2+ dynamics in human muscle fibers are altered by malignant hyperthermia causative RyR mutation" Proc Natl Acad Sci U S A (2018)
  3. "Recognizing and managing a malignant hyperthermia crisis: guidelines from the European Malignant Hyperthermia Group" Br J Anaesth (2010)
  4. "Malignant hyperthermia 2020: Guideline from the Association of Anaesthetists" Anaesthesia (2021)
  5. "Clinical presentation, treatment, and complications of malignant hyperthermia in North America from 1987 to 2006" Anesth Analg (2010)
  6. "Malignant hyperthermia: update on susceptibility testing" JAMA (2005)
  7. "Malignant hyperthermia: a review" Orphanet J Rare Dis (2015)