Malignant hyperthermia (MH) was first described by Denborough in 1962 when deaths were occurring during and immediately (within 24 hours) following the administration of anesthesia medications. The common denominator in these patients was sudden and critical increases in body temperature. These occurrences were quick to reveal that MH is passed down through families (Denborough, Forster, Lovell, Maplestone, & Villiers, 1962).
Patients at risk for MH are very difficult to screen. Although the exact incidence of MH is unknown, the occurrence is estimated to range from about 1 in 100,000 adults and 1 in 30,000 in children (Watt & McAllister, 2022). Anesthesia agents trigger MH, and it is silent until it has already occurred.
Half of the patients who have an MH crisis have had prior exposure to anesthesia agents known to trigger a crisis without any such reaction. Males have a higher incidence than females, and patients less than the age of 50 are more likely to have MH. Pediatric patients are most frequently affected. Pediatric patients with a history of rheumatoid arthritis have the highest incidence of anesthesia-induced malignant hyperthermia (Butterworth, Mackey, & Wasnick, 2013). Although the most common patients are surgical patients, MH has also been reported after anesthesia medications are used for sedation in surgeon’s offices, intensive care units, dental offices, and emergency departments (National Institute of Arthritis and Musculoskeletal and Skin Diseases, 2020).
The most common medications administered prior to an MH event include succinylcholine, sevoflurane, desflurane, isoflurane, and halothane. Some non-triggering anesthetic agents that are recommended for patients with a previous diagnosis of MH include thiopental sodium and pancuronium, droperidol, benzodiazepines, and ester-type local anesthetics.
An uncontrolled increase in skeletal muscle metabolism is a characteristic of MH. The calcium stores in muscle cells are released, and the muscles contract and stiffen at the same time. The patient experiences a sudden and quite dramatic increase in body temperature that can be as dramatic as 1–2 degrees Celsius every five minutes.
If appropriately treated, the mortality rate is less than 10%. If not recognized and treated, the mortality rate can be expected to be as high as 80%. Most professionals believe that this could be much lower with proper preparedness for rapid recognition and treatment of malignant hyperthermia (McCarthy, 2004). One of the ways that the surgical team can better prepare is by maintaining an emergency cart dedicated to the treatment of malignant hyperthermia. The recommended contents of such a cart are found at the end of this article.
The increase in body temperature is not the first sign in MH. This is usually a late but confirming occurrence. Because of the increase in skeletal muscle metabolism and damage, a number of key changes in baseline lab results are common. Blood gases are helpful in diagnosis.
In malignant hyperthermia you would expect to see:
You would expect to see a result above normal in the following lab results:
You would expect to see a result below normal in the following lab results:
The following treatment protocol is published by the Malignant Hyperthermia Association of the United States (MHAUS) (Malignant Hyperthermia Association of the United States, n.d). Although written in a specific order, many of the following interventions should be done simultaneously. Early diagnosis and treatment are essential to limit mortality.
Treatment | Dosage or action |
---|---|
Immediately discontinue anesthesia, including succinylcholine | Continue a life-threatening surgery but with the use of a different anesthetic agent and machine to prevent residual inhalation from triggering a second episode. |
Hyperventilate | 100% oxygen at a high flow rate of 10 L/min to treat the effects of hypercapnia, metabolic acidosis, and increased oxygen consumption. |
Dantrolene | 2.5 mg/kg IV as soon as possible, given every 5 minutes until the symptoms subside. |
Change ventilator tubing and soda-lime canister | Some anesthesia providers may still perform this action, but research has shown that it is not necessary to change the circuit and anesthesia machine since the oxygen delivery rapidly clears the machine of any residual gases. |
Sodium bicarbonate | 1–2 mEq/kg IV to combat metabolic acidosis due to an increase of lactate in the circulatory system. |
Ice packs | Apply to the groin area, axillary regions, and sides of the neck—where major arteries are located. Also, apply to wrists and ankles/feet. |
Iced lavage | Lavage the stomach and rectum with cold fluids to lower temperature. It is recommended not to lavage the bladder since the fluids can alter the true amount of urine being excreted by the patient, which can alter the measurement of output. |
Mannitol or furosemide | Muscle cells are destroyed during an MH crisis and the myoglobin that is released accumulates in the kidneys, obstructing urinary flow, referred to as myoglobinuria. Diuretics are given IV to promote and maintain urinary flow in order to prevent renal damage. Mannitol 0.25 g/kg IV; furosemide 1 mg/kg IV up to four doses each. Urinary output of 2 ml/kg/hr or higher must be maintained to prevent renal failure. |
Procainamide | 200 mg IV to treat arrhythmias secondary to electrolyte imbalances. |
Dextrose and insulin | Treat hyperkalemia due to the release of potassium into the circulatory system as muscle cells are destroyed. Dextrose 25–50 g IV, regular insulin 10 units in 50 ml of 50% dextrose in water given IV. |
Monitor urine output | Insert Foley catheter if one is not in place. |
Monitor electrolyte levels | Blood samples were taken every 10 minutes to measure sodium, potassium, chlorides, calcium, phosphate, and magnesium levels. |
Perform clotting studies | PT, or prothrombin time; PTT, or partial thromboplastin time. |
Arterial blood gasses (ABGs) | PT, or prothrombin time; PTT, or partial thromboplastin time. |
Arterial blood pressure (AVP) | Insert arterial line if one is not in place. |
Central venous pressure (CVP) | Insert central line if one is not in place. |
Capnography | Monitor carbon dioxide (CO2) levels in the respiratory gasses continuously. |
Dantrolene, a skeletal muscle relaxant specifically developed for the treatment of MH, is the backbone of the treatment regimen. The administration is every 6–8 hours for 24–72 hours after the initial episode. The dose is 1 mg/kg. The standard dose is based on an adult that weighs 70 kg; thirty-six 20 mg vials of dantrolene will be needed to stabilize the patient. Rarely does the total required dosage exceed 10 mg/kg? A common complication of dantrolene is phlebitis; therefore, it is recommended that it be given via a central line (Butterworth, 2013).
To reduce the time needed to implement a definitive treatment, it is recommended that each operating room, outpatient surgery center, and physician office where surgical procedures are performed have a cart dedicated to MH (MHAUS, n.d.).
The supplies, equipment, and medications for the cart are as follows:
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