Materiały źródłowe

• #1 Malignant Hyperthermia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430828/

  Malignant hyperthermia (MH) is a hereditary disorder of skeletal muscle that classically presents as a hypermetabolic response to halogenated anesthetic gasses and/or the depolarizing muscle relaxant succinylcholine. […] This activity reviews the evaluation and treatment of malignant hyperthermia and the interprofessional team’s role in evaluating and treating this condition. […] Outline the treatment of malignant hyperthermia. […] The critical element in the treatment of malignant hyperthermia is immediate dantrolene administration. Once a malignant hyperthermia episode is suspected, all triggering agents must be discontinued and the patient hyperventilated with 100% oxygen with non-triggering anesthetic agents utilized for patient care, and surgery should be ended as soon as possible. Dantrolene in a dose of 2.5 mg/kg must be administered intravenously as soon as possible, up to a maximum dose of 10mg/kg until the reaction subsides.

• #2 RYANODEX® | What is malignant hyperthermia (MH)?

  https://www.ryanodex.com/about-mh/

  Malignant hyperthermia (MH) is a pharmacogenetic disease that causes hypermetabolism, a fast rise in body temperature and severe muscle contractions when an affected person receives general anesthesia using volatile anesthetics or the paralytic succinylcholine. […] Immediate treatment with the drug dantrolene sodium usually reverses the signs of MH. […] An initial 20-minute delay in administration of dantrolene sodium raises the risk of complications to 30%. […] Stopping the triggering agents and administering dantrolene sodium to the patient as quickly as possible are the greatest priorities in an MH crisis. […] Every delay in treatment increases the risk of further complication during an MH crisis. […] The risk of complications may increase to 30% with a 20-minute delay in treating MH from its first symptom.

• #3 Management of malignant hyperthermia: diagnosis and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4027921/

  Malignant hyperthermia is a potentially lethal inherited disorder characterized by disturbance of calcium homeostasis in skeletal muscle. […] Since the clinical presentation of malignant hyperthermia is highly variable, survival of affected patients depends largely on early recognition of the symptoms characteristic of malignant hyperthermia, and immediate action on the part of the attending anesthesiologist. […] Treatment includes immediate administration of dantrolene 2 mg/kg, which should be repeated every 5 minutes until the cardiac and respiratory systems are stabilized. Dantrolene, a hydantoin derivative, acts as a specific ryanodine receptor antagonist and inhibits release of calcium from the sarcoplasmic reticulum without improving its reuptake. […] The prognosis of an MH crisis depends on how soon MH is suspected and how rapidly appropriate treatment is initiated.

• #4 Managing A Crisis – MHAUS

  https://www.mhaus.org/healthcare-professionals/managing-a-crisis/

  Notify surgeon to halt the procedure ASAP: Discontinue volatile agents and succinylcholine. […] If surgery must be continued, maintain general anesthesia with IV non-triggering anesthetics (e.g., IV sedatives, narcotics, amnestics and non-depolarizing neuromuscular blockers as needed). […] Get dantrolene/MH cart. (Call 911 if surgicenter). […] Call for help within your institution; also, call the MHAUS Hotline (1-800-644-9737) for additional advice. (Outside the US, please call: 001-209-417-3722). […] Hyperventilate with 100% oxygen at flows of 10L/min to flush volatile anesthetics and lower ETCO2. […] Give IV dantrolene 2.5 mg/kg rapidly through large-bore IV, if possible. Repeat as frequently as needed until the patient responds with a decrease in ETCO2, decreased muscle rigidity, and/or lowered heart rate. Large doses (10mg/kg) may be required for patients with persistent contractures or rigidity.

• #5 Malignant Hyperthermia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430828/

  The management algorithm includes: Stop the triggering agent. Call for help. Administer dantrolene 2.5 mg/kg until the reaction subsides up to a maximum of 10 mg/kg. Increase ventilation to lower end-tidal CO2. Cooling measures (cold IV fluids and ice packs to exposed surfaces). Treat arrhythmias (e.g., amiodarone; avoid calcium channel blockers). […] Better patient outcomes are associated with the rapidity of diagnosis, rapid treatment with dantrolene, and prevention of the rapid rise in core temperature by using cooling measures. […] After the patient has been stabilized, they must be taken to the intensive care unit for at least 24 hours for monitoring and to watch for signs of recrudescence. […] Despite having been performed frequently in the past, pretreating malignant hyperthermia-susceptible patients with dantrolene does not play a role in their care and should not be done. Instead, these patients should receive a non-triggering anesthetic.

• #6 Malignant Hyperthermia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430828/

  Dantrolene works by inhibiting calcium ion release from the sarcoplasmic reticulum. Its mechanism of action is through antagonizing the ryanodine receptors, which lessens the excitation-contraction coupling of muscle cells. […] Dantrolene is currently the only specific medication used for treating a malignant hyperthermia crisis. […] Keep in mind that additional doses of dantrolene might be necessary to treat malignant hyperthermia triggering events adequately; a dose of 1 mg/kg every 4 to 6 hours is recommended for the first 24 to 48 hours after an episode of malignant hyperthermia. […] All facilities where malignant hyperthermia triggering anesthetics are administered are recommended to stock an adequate amount of dantrolene and other medications and rescue equipment needed to treat a malignant hyperthermia crisis. […] Even though dantrolene is recommended for treatment, good data supporting its efficacy are still lacking. The patient should be managed in an ICU setting with close monitoring.

• #7 Management of malignant hyperthermia: diagnosis and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4027921/

  Malignant hyperthermia is a potentially lethal inherited disorder characterized by disturbance of calcium homeostasis in skeletal muscle. […] Since the clinical presentation of malignant hyperthermia is highly variable, survival of affected patients depends largely on early recognition of the symptoms characteristic of malignant hyperthermia, and immediate action on the part of the attending anesthesiologist. […] Treatment includes immediate administration of dantrolene 2 mg/kg, which should be repeated every 5 minutes until the cardiac and respiratory systems are stabilized. Dantrolene, a hydantoin derivative, acts as a specific ryanodine receptor antagonist and inhibits release of calcium from the sarcoplasmic reticulum without improving its reuptake. […] The prognosis of an MH crisis depends on how soon MH is suspected and how rapidly appropriate treatment is initiated.

• #8 Malignant Hyperthermia Treatment & Management: Dantrolene and Supportive Care, Cancellation or Modification of Surgical Procedure

  https://emedicine.medscape.com/article/2231150-treatment

  Dantrolene and Supportive Care […] Indications for treatment of malignant hyperthermia (MH) with dantrolene include signs of hypermetabolism, a rapid rise in carbon dioxide in the face of an increase in the minute ventilation, tachycardia, muscle and or jaw rigidity (after succinylcholine), and fever (a late sign). […] If an acute MH reaction appears likely, it is best to start giving dantrolene and other recommended treatment modalities promptly rather than wait too long and have a bad outcome. The longer the wait before initiation of therapy, the lower the likelihood of a complete recovery. […] A fulminant, rapidly progressive MH reaction requires early diagnosis and early rapid administration of dantrolene, discontinuance of triggering agents, and assistance from extra personnel. […] Cooling and early treatment of hyperkalemia are desirable. […] An MH tote or cart containing dantrolene and the necessary supplies should be readily available to help reverse the process more quickly. […] Dantrolene is a hydantoin derivative that directly interferes with muscle contraction by inhibiting calcium ion release from the sarcoplasmic reticulum, possibly by binding to ryanodine receptor type 1 (RYR-1). […] The initial dose is 2.5 mg/kg, repeated every 5 minutes until reversal of the reaction occurs or a total dose of 10 mg/kg (or 20 mg/kg, according to some practitioners) is reached. […] Once the initial reaction is controlled, continued monitoring in the intensive care unit (ICU) for 24-48 hours is recommended, along with administration of dantrolene (1 mg/kg q4-6 hr, or an equivalent amount given as a continuous infusion). […] A rapid formulation of dantrolene has been developed that dissolves in 15 seconds and thereby enhances the promptness of treatment. […] Some case reports have described successful treatment of MH in settings where dantrolene is unavailable. […] The patient and the family members will need to be educated about MH and should be referred to a testing center for a caffeine halothane contracture test (CHCT).

• #9 Malignant Hyperthermia: An Overview

  https://www.uspharmacist.com/article/malignant-hyperthermia-an-overview

  It is important that hospitals respond appropriately to cases of MH. The MHAUS recommends that dantrolene be accessible within 10 minutes after the decision to treat is made and that at least 700 mg enough to treat a 70-kg patient be available; this equates to 35 20-mg vials or three 250-mg vials. […] Healthcare facilities using agents that can trigger MH – volatile anesthetics and succinylcholine – must be prepared to rapidly detect and initiate treatment for MH.

• #10 RYANODEX® | What is malignant hyperthermia (MH)?

  https://www.ryanodex.com/about-mh/

  Malignant hyperthermia (MH) is a pharmacogenetic disease that causes hypermetabolism, a fast rise in body temperature and severe muscle contractions when an affected person receives general anesthesia using volatile anesthetics or the paralytic succinylcholine. […] Immediate treatment with the drug dantrolene sodium usually reverses the signs of MH. […] An initial 20-minute delay in administration of dantrolene sodium raises the risk of complications to 30%. […] Stopping the triggering agents and administering dantrolene sodium to the patient as quickly as possible are the greatest priorities in an MH crisis. […] Every delay in treatment increases the risk of further complication during an MH crisis. […] The risk of complications may increase to 30% with a 20-minute delay in treating MH from its first symptom.

• #11 Managing A Crisis – MHAUS

  https://www.mhaus.org/healthcare-professionals/managing-a-crisis/

  If giving large doses (10 mg/kg) without symptom resolution, consider alternative diagnoses. […] Consider administration of sodium bicarbonate, 1-2 mEq/kg dose, for base excess greater than -8. […] Cool the patient if core temperature is 39C or less if rapidly rising. Stop cooling when the temperature has decreased to 38C. […] If hyperkalemia (K 5.9 or less with ECG changes) is present, treat with: Calcium chloride 10 mg/kg (maximum dose 2,000 mg) or calcium gluconate 10-50 mg/kg (maximum dose 3,000 mg) for life-threatening hyperkalemia. […] For refractory hyperkalemia, consider albuterol (or other beta-agonist), dialysis, or ECMO if patient is in cardiac arrest. […] Treat dysrhythmias with standard medication but avoid calcium channel blockers. […] Diurese to 1ml/kg/hr urine output. If CK or K+ rise, assume myoglobinuria and give bicarbonate infusion of 1 mEq/kg/hr, to alkalinize urine.

• #12 What evidence-based interventions are recommended to alleviate hyperthermia associated with Malignant Hyperthermia? – MHAUS

  https://www.mhaus.org/healthcare-professionals/mhaus-recommendations/what-evidence-based-interventions-are-recommended-to-alleviate-hyperthermia-associated-with-malignant-hyperthermia/

  What evidence-based interventions are recommended to alleviate hyperthermia associated with Malignant Hyperthermia? […] The most important treatment of Malignant Hyperthermia (MH) is discontinuing MH triggering agents, hyperventilation, and timely administration of dantrolene. However, prolonged hyperthermia worsens patients’ outcomes and should also be treated when occurs. […] Many experts believe that hyperthermia is a sign of inadequate physiological treatment and clinicians’ priority should be to stop MH with dantrolene and adequately treat hypercarbia and acidosis before focusing time and efforts on thermal management. […] Pharmacologic treatment of hyperthermia includes dantrolene, acetaminophen, and nonsteroidal anti-inflammatory drugs. Dantrolene is the only clinically available specific treatment for MH and, after discontinuation of triggering agents, should always be the initial treatment for any suspected MH episode. The recommended initial dose of dantrolene is 2.5 mg/kg bolus with repeated boluses as needed until hypermetabolism is controlled.

• #13 What evidence-based interventions are recommended to alleviate hyperthermia associated with Malignant Hyperthermia? – MHAUS

  https://www.mhaus.org/healthcare-professionals/mhaus-recommendations/what-evidence-based-interventions-are-recommended-to-alleviate-hyperthermia-associated-with-malignant-hyperthermia/

  External cooling methods such as circulating-water mattresses or ice packs should be considered first. If external cooling is insufficient, an easy, effective, and safe next cooling strategy is to infuse 20 mL/kg of refrigerated intravenous fluid. Other treatments should rarely be necessary, but peritoneal lavage is probably the safest and most effective of the invasive approaches if the peritoneum is already open or the patient is in an emergency department with the requisite equipment and skills.

• #14 Malignant Hyperthermia (MH) – EMCrit Project

  https://emcrit.org/ibcc/mh/

  Once stabilized, continue a maintenance dose of 1 mg/kg dantrolene IV q4-6hr (although fulminant cases may require a continuous infusion at 0.25 mg/kg/hour). […] Continuous monitoring of the core temperature is essential (preferably via a bladder or esophageal temperature probe). […] Initiate cooling if the core temperature is 39C (102.2F), or if the temperature is rapidly rising. […] Treatment involves adequate fluid resuscitation. The initial fluid of choice for resuscitation might be isotonic bicarbonate (D5W with three 50-mEq ampules of bicarbonate per liter), as this may be beneficial for hyperkalemia, acidosis, and rhabdomyolysis. […] Hemodialysis may be needed if medical therapies fail to control hyperkalemia. […] Immediate management of MH will often be necessary before a definitive diagnosis is made. Dantrolene is very safe, so when in doubt empiric therapy should be provided without delay.

• #15 Malignant Hyperthermia Acute Treatment: MH Investigation Unit, PIE, hospital education, anesthesia, CHCT

  http://pie.med.utoronto.ca/MH/MH_content/acuteTreatment.html

  If you suspect an MH crisis in a patient under a triggering anesthesia, follow these steps to manage the MH crisis. […] Immediately begin dantrolene sodium 2.5 mg/kg. Rapidly administer the initial bolus intravenously with supplemental increments up to 10mg/kg. […] Administer bicarbonate to correct metabolic acidosis as guided by blood gas analysis. […] If dantrolene has been administered, do not use calcium channel blockers as they can interact to produce fatal hyperkalemia and cardiovascular collapse. […] Hyperkalemia is common and should be treated with hyperventilation, bicarbonate and intravenous glucose and insulin (e.g. 10 units regular insulin in 50 mL 50% glucose titrated to potassium level). […] Ensure urine output of greater than 2 mL/kg/hr by hydration and/or administration of mannitol or furosemide.

• #16 Malignant Hyperthermia: An Overview

  https://www.uspharmacist.com/article/malignant-hyperthermia-an-overview

  Dysrhythmias should be treated with standard medications; however, calcium channel blockers must be avoided during an MH crisis because they can worsen hyperkalemia and hypotension. […] Once the initial MH reaction is under control and the patient is stable, the patient should be continuously monitored in a post anesthesia care unit or ICU for at least 24 hours. […] Immediately following an acute MH reaction, dantrolene should be continued for at least 24 hours at a dosage of 1 mg/kg by IV injection every 4 to 6 hours or by IV infusion at a dosage of 0.25 mg/kg/hour. […] The MHAUS suggests that dantrolene can be discontinued or the dosing interval increased to up to every 12 hours if all of the following criteria are met: metabolic stability for 24 hours, core temperature less than 38C, decreasing CK, no evidence of myoglobinuria, and no muscle rigidity.

• #17 Managing A Crisis – MHAUS

  https://www.mhaus.org/healthcare-professionals/managing-a-crisis/

  Institute appropriate monitoring including: core temperature, urine output with bladder catheter, and consider arterial and/or central venous monitoring if warranted by the clinical severity of the patient. […] When stable, transfer to post anesthesia care unit or intensive care unit for at least 24 hours. Key indicators of stability include: ETCO2 is declining or normal, heart rate is stable or decreasing with no signs of ominous dysrhythmias, hyperthermia is resolving, and if present, generalized muscular rigidity has resolved.

• #18 Malignant Hyperthermia, Dantrolene, and the Pharmacist

  https://www.pharmacytimes.com/view/malignant-hyperthermia-dantrolene-and-the-pharmacist

  If resolution does not occur following a cumulative dose of 10 mg/kg, it may indicate a differential diagnosis. […] Greater emphasis should be placed on cooling as dantrolene may not be effective in severe muscle necrosis. […] Reoccurrence of this hypermetabolic state appears in up to 25% of patients in the first 48 to 72 hours in the post-reaction phase. […] If required, a further bolus of dantrolene is determined by the reaction time: 2.5 mg/kg if greater than 6 hours, or 1 mg/kg every 6 hours (if within 6 hours) for at least 24 hours or clinically indicated. […] Mortality in MH has been reduced from 80% in the 1960s to less than 10% today, thanks to the progress in understanding the clinical manifestation of the syndrome.

• #19 Management of malignant hyperthermia: diagnosis and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4027921/

  Subsequent steps might be necessary to avoid further harm to the patient, and volume resuscitation and administration of vasopressors might be needed to stabilize hemodynamics. […] Hyperthermia should be treated by internal cooling with cold infusion fluids and external surface cooling with ice packs placed in the axillae and groin or specific cooling devices until body temperature reaches 38.5C. […] In clinics that use known MH-triggering agents for induction and maintenance of general anesthesia, dantrolene must be available for immediate treatment and to reduce the risk of serious harm to the patient in the event of an episode of MH.

• #20 Malignant hyperthermia – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/malignant-hyperthermia/symptoms-causes/syc-20353750

  Treatments for malignant hyperthermia include the medication dantrolene (Dantrium, Revonto, Ryanodex), ice packs and other measures to cool body temperature, as well as supportive care. […] If you have a family history of malignant hyperthermia or a relative who has problems with anesthesia, tell your health care provider or anesthesiologist before surgery or any procedure that requires anesthesia. […] Evaluating your risk of malignant hyperthermia allows your anesthesiologist to avoid certain anesthesia drugs.

• #21 Malignant Hyperthermia Treatment & Management: Dantrolene and Supportive Care, Cancellation or Modification of Surgical Procedure

  https://emedicine.medscape.com/article/2231150-treatment

  Cancellation or Modification of Surgical Procedure […] When the anesthesia provider first suspects that an MH reaction may be occurring, the surgeon should be notified promptly, and a decision should be made about whether the procedure is to be continued or canceled. […] If the procedure is to be performed, the following considerations should be kept in mind in planning anesthesia for an MH-susceptible patient: Avoid triggering agents (eg, major inhalational agents and succinylcholine).

• #22 RYANODEX® | What is malignant hyperthermia (MH)?

  https://www.ryanodex.com/about-mh/

  With low-concentration, high-volume MH treatment, the MHAUS recommendation of 2.5 mg/kg loading dose of dantrolene sodium requires considerable volumes of sterile water for injection for the majority of patients (eg, 750 mL for a 100 kg patient). […] RYANODEX® (dantrolene sodium) for injectable suspension is indicated for the treatment of malignant hyperthermia in conjunction with appropriate supportive measures, and for the prevention of malignant hyperthermia in patients at high risk.

• #23 Malignant Hyperthermia: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/2231150-overview

  Dantrolene, the antidote, decreases the loss of calcium from the sarcoplasmic reticulum in the skeletal muscle and restores normal metabolism. […] Dantrolene inhibits the release of calcium from the SR and reverses the process. […] If an MH reaction is treated early in the process, complete recovery can be expected. […] Before the approval of dantrolene by the US Food and Drug Administration (FDA) in the late 1970s for use in the treatment of MH, the mortality of an acute MH reaction was greater than 70%. Currently, the mortality of acute MH is less than 5%. […] In 1982, MHAUS started an around-the-clock hotline to answer questions about MH and to give guidance concerning treatment of acute cases.

• #24 Malignant hyperthermia – Wikipedia

  https://en.wikipedia.org/wiki/Malignant_hyperthermia

  Treatment is with dantrolene and rapid cooling along with other supportive measures. […] The current treatment of choice is the intravenous administration of dantrolene, the only known antidote, discontinuation of triggering agents, and supportive therapy directed at correcting hyperthermia, acidosis, and organ dysfunction. Treatment must be instituted rapidly on clinical suspicion of the onset of malignant hyperthermia. […] Dantrolene is a muscle relaxant that appears to work directly on the ryanodine receptor to prevent the release of calcium. After the widespread introduction of treatment with dantrolene, the mortality of malignant hyperthermia fell from 80% in the 1960s to less than 5%. Dantrolene remains the only drug known to be effective in the treatment of MH. The recommended dose of dantrolene is 2.5 mg/kg, repeated as necessary. It is recommended that each hospital keeps a minimum stock of 36 dantrolene vials (720 mg), sufficient for four doses in a 70-kg person.

• #25 Malignant Hyperthermia in PICU—From Diagnosis to Treatment in the Light of Up-to-Date Knowledge

  https://www.mdpi.com/2227-9067/9/11/1692

  Boluses of dantrolene should be repeated every 10 min until meeting the treatment goal (EtCO2 < 6 kPa with normal minute ventilation and body temperature < 38.5 °C). [...] Continuous monitoring of body temperature is an absolute must-have—not only to be aware of ongoing hyperthermia but also to avoid the opposite extreme, hypothermia, caused by excessive cooling, especially, in babies and small children. [...] The occurrence of DIC during an MH crisis is associated with poor outcomes. Empirical treatment with plasma and platelet transfusions and coagulation factors cryoprecipitates should be commenced. [...] Every suspected MH crisis should also be referred to the MH diagnostic center, and the patient and his family must be informed.

• #26 Malignant Hyperthermia Preparedness: Stocking, Drilling, and Offsite Considerations – Anesthesia Patient Safety Foundation

  https://www.apsf.org/article/malignant-hyperthermia-preparedness-stocking-drilling-and-offsite-considerations/

  Malignant hyperthermia (MH) is a rare life-threatening condition that is triggered by commonly used anesthetic medications. Early recognition and treatment is essential to improve survival rates, and being prepared for an MH crisis can decrease clinical response time and the associated morbidity and mortality with the disease. Stocking a dedicated MH cart and routinely performing simulated MH crisis drills can improve the facility and providers’ management. […] The Malignant Hyperthermia Association of the United States (MHAUS) recommends medications and supplies be readily available for use, within 10 minutes of recognizing an MH crisis. Since the likelihood of complications increases 1.6 times with every 30-minute delay in treatment with dantrolene, having a centrally located cart with the necessary medications and equipment expedites the initiation of treatment. […] Early recognition and treatment of MH is essential to improve survival rates. Stocking a dedicated MH cart, routinely performing simulated MH crisis drills, and having enough dantrolene stocked can save lives.

• #27 Malignant Hyperthermia (MH) – EMCrit Project

  https://emcrit.org/ibcc/mh/

  Treatment must be initiated empirically, when the MH diagnosis is considered probable. The treatments are fairly benign, so it’s generally better to err on the side of treatment. […] Call for an MH cart (if this is available in the operating room). If no MH cart is available, immediately seek help from your pharmacy. You may need: […] Dantrolene vials (enough to prepare 10 mg/kg at the bedside if needed). Sterile water will be needed to reconstitute the dantrolene. […] Hyperventilate with 100% oxygen at 2-3 times the normal minute ventilation, to clear any residual volatile anesthetic. […] Titrate the respiratory rate and tidal volume in order to achieve an end-tidal pCO2 of ~25 mm (~3.3 kPa), if possible. […] 2.5 mg/kg IV immediately, then administer an additional 1-2.5 mg/kg IV q5min until symptoms subside

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