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CE Article: Malignant Hypothermia
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Malignant Hyperthermia
Malignant hyperthermia (MH) is a life-threatening syndrome associated with an anesthetic trigger. Awareness of MH by all perioperative team members, from those working in the preoperative holding area to those in the Post Anesthesia Care Unit (PACU), is important in preventing negative patient outcomes. The preoperative nurse plays a crucial role in averting an MH crisis by interviewing every surgical patient for a personal and family history of MH.
The goal of this continuing education program is to provide OR nurses, physicians, and surgical technologists with information about malignant hyperthermia, including perioperative signs and management of patients who develop malignant hyperthermia.
Imagine that you are an OR nurse assessing your first patient of the day. You help the patient to the OR and onto the table. Standard monitors are applied. You note that this is the 30-year-old patient’s first surgery. He receives routine anesthesia induction medications that include fentanyl IV (Sublimaze®), propofol (Diprivan®), and succinylcholine (Anectine®). Intubation is uneventful. The patient is placed on air and oxygen at 50% flow for each with desflurane (Suprane®) 6% inhalational agent. Within minutes, the anesthesia provider notes muscle rigidity in the patient and an increase in exhaled carbon dioxide, heart rate, and blood pressure. The provider suspects malignant hyperthermia based on these initial findings. As a vital part of the team, what will you do to help? What is the best plan of action? How can you help save this patient’s life?
Malignant hyperthermia is a potentially life-threatening hypermetabolic state of muscle activity resulting from a defect in skeletal muscle receptors that allows excessive calcium accumulation. It is primarily encountered intraoperatively after the administration of a triggering anesthetic agent. In rare cases, MH can manifest within one hour postoperatively in the PACU. MH is triggered by depolarizing neuromuscular agents such as succinylcholine or volatile halogenated anesthetic agents such as ether, enflurane, methoxyflurane, desflurane, sevoflurane (Ultane®), or isoflurane (Forane®) (MHAUS, 2017a). Providers who work in dental and emergency care settings should also be aware of MH as they frequently administer some of the triggering agents.
MH is an autosomal dominant pharmacogenetic clinical syndrome during which a hypermetabolic state develops and becomes a life-threatening emergency (MHAUS, 2017a; Phillips, 2016). The patient’s body becomes hyperthermic because of increased metabolic activity within the skeletal muscle.
During an MH crisis, the skeletal muscles are stimulated to contract, muscle metabolism increases, and depolarization occurs with passage of calcium into the intracellular space. The muscles cannot relax and exposure to one or more of the triggering agents causes a rapid intracellular and extracellular imbalance of calcium that leads to significant energy use and heat production. At the cellular level, as continuing attempts to correct the hypercalcemia are made, heat production increases. Muscle cell relaxation occurs when reuptake of calcium by the sarcoplasmic reticulin occurs. In patients with MH, it appears as if they have an unregulated passage of calcium from the sarcoplasmic reticulum into the intracellular space causing sustained muscle contraction (Zhou, 2015).
Calcium is an extracellular ion of the soft tissue that is necessary for nerve impulse transmission, muscle contraction, cardiac function, and blood coagulation.
Relias LLC guarantees this educational program free from bias. The planners and authors have declared no relevant conflicts of interest that relate to this educational activity. See Page 37 to learn how to earn CE credit for this module.
Goal and Objectives
After taking this course, you should be able to: • Identify patients at risk for malignant hyperthermia • Differentiate the early and late signs and symptoms of malignant hyperthermia • Describe the diagnosis and treatment of malignant hyperthermia
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There is also a build-up of lactic acid and carbon dioxide. The increased amount of intracellular calcium causes sustained muscle rigidity that increases metabolism in both oxygen-dependent and independent pathways, increasing overall oxygen consumption as well as leading to severe lactic acidosis. Lactic acidosis occurs when the body obtains energy by breaking down glucose reserves without the use of oxygen, also known as anaerobic metabolism. As the levels of lactic acid increase, the muscle membranes break down, releasing myoglobin and potassium stores. The elevated levels of myoglobin, also termed rhabdomyolysis, can lead to kidney damage. As extracellular potassium increases, high levels may predispose patients to cardiac conduction abnormalities, arrhythmias, and even sudden death (MHAUS, 2017a; Phillips, 2016; Zhou, et al., 2015).
The etiology of MH, in most cases, is a defect of the genetic receptor that controls calcium release from the sarcoplasmic reticulum of the muscle cell wall. No single gene mutation causes MH susceptibility. However, more than 170 variations in the ryanodine receptor 1 gene (the intracellular calcium channel gene, also called RYR1) have been linked to MH susceptibility (Phillips, 2016; Zhou, et al., 2015; Litman, et al 2020).
History
MH was identified in the 1960s in Australia by researchers when a 21-year-old patient told his physicians he was more concerned about receiving anesthesia than having surgery for his broken leg. Ten close relatives had died when under anesthesia during minor procedures. The anesthesiologist thought the deaths were caused by ether so he gave the young man a new anesthetic gas, halothane. The patient became cyanotic and displayed erratic vital signs, including hyperthermia. Monitoring end-tidal carbon dioxide and body temperature were not the standard of care at that time. The anesthesiologist treated the patient symptomatically and packed him in ice. The patient became the first recorded person to survive an MH crisis. One of the researchers traced the autosomal dominant inheritance of the family’s severe reaction to anesthesia over three generations. He later published his findings in The Lancet (Denborough et al., 1970). The patient had surgery later under spinal anesthesia without incident.
Malignant hyperthermia does not discriminate among races. All ethnic groups are affected in all parts of the world. Reactions develop more frequently in males than females by a 2-to-1 ratio. MH is not X-linked as are many other muscular diseases such as muscular dystrophy. The incidence of MH reactions ranges from 1 in 100,000 for adults to 1 in 30,000 pediatric anesthetic administrations, with the prevalence of the genetic abnormalities being as great as 1 in 2,000 people. Overall, this amounts to approximately 500 to 800 surgical cases that are complicated by MH each year. The true prevalence is difficult to define because of unrecognized mild or aborted reactions. Some patients have had previous surgery under general anesthesia without any reactions (Phillips, 2016).
The highest incidence of MH is in males with a median age of 39 (Phillips, 2016). Patients who have an increased muscular build or muscular deformity are associated with a greater risk of death during an MH crisis. Early studies concluded that mortality ranged between 70% and 90% before treatment options and monitoring devices for carbon dioxide levels were developed. Later studies showed that with appropriate diagnosis and treatment, the mortality rate fell to around 5% (MHAUS, 2017b).
Animal studies have shown that some genetically similar mammals, particularly swine, display hypermetabolic symptoms similar to those in humans when exposed to triggers such as depolarizing neuromuscular blockers and volatile anesthetic gases (MHAUS, 2017a; Phillips, 2016). Nondepolarizing neuromuscular agents, barbiturates, sedatives, and nitrous oxide do not trigger the syndrome and can be used to perform a surgical procedure without danger. These anesthetic agents and drugs keep the patient anesthetized for surgery and allow for endotracheal intubation, which is frequently required. Local and regional anesthetics are also safe in that they have not been implicated in MH reactions (MHAUS, 2017a).
Signs and Symptoms
The most common initial sign of acute MH is an unexplained rise in end-tidal carbon dioxide, known as hypercarbia. Carbon dioxide levels rise above the normal 35 to 45 mmHg range despite increasing the respiratory rate for the ventilator settings. This rise in carbon dioxide of more than 60 mmHg is a hallmark indicator of MH (MHAUS, 2017c).
Muscle rigidity after the administration of a depolarizing agent, especially masseter spasm or trismus (contraction of the jaw muscles), is another potential sign of MH. Trismus can be present in the absence of MH and is not uncommon in small children (Phillips, 2016). Unexplained tachycardia and tachypnea are also early signs of MH. Ventricular fibrillation, a lethal heart dysrhythmia that can be seen on an electrocardiogram, can develop within minutes of the onset of MH and may rapidly lead to death (Phillips, 2016; MHAUS, 2017c).
Later signs of MH include metabolic acidosis, hyperthermia sometimes as high as 107 degrees Fahrenheit, and evidence of rhabdomyolysis. Rhabdomyolysis is the catabolism (breakdown) of skeletal muscle associated with excretion of myoglobin (a protein in muscle fibers) in the urine (MHAUS, 2017c). Rhabdomyolysis is characterized by dark brown urine caused by myoglobinuria with hyperkalemia (increased potassium). There is increased myoglobin and total creatine kinase (CK) levels in blood samples, resulting from muscle membrane breakdown. The myoglobin in the urine causes damage to the renal tubules and ultimately causes renal failure (Phillips, 2016; Chavez, 2016).
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Arterial blood gas analysis and lab findings reveal metabolic acidosis. Severe hyperthermia (core temperature greater than 104 F [40 C]) results from a marked increase in oxygen consumption and increased carbon dioxide production during sustained muscular contraction. Remember, hyperthermia is usually a later sign of MH (Walter & Carraretto, 2016). Patients exposed to extreme body temperature elevation for prolonged periods of time experience more central nervous system (CNS) complications post event. An example of a CNS complication is seizure activity (Walter & Carraretto, 2016).
Vasoconstriction associated with protracted muscle contraction may cause widespread multisystem organ failure and disseminated intravascular coagulation (DIC). Further life-threatening conditions arising from MH include compartment syndrome of the limbs secondary to profound muscle swelling (Litman et al., 2020; Gupta & Hopkins, 2017).
Diseases That Mimic
Other diseases may be confused with MH including trismus, central core disease, and neuroleptic malignant syndrome. Patients who exhibit trismus during induction of anesthesia are difficult to intubate, and loss of airway is a significant concern. The safest course of action is to assume that masseter spasm is due to MH and to postpone elective surgery under general anesthetic (MHAUS, 2017a; Phillips, 2016).
Patients with masseter muscle rigidity are at greater risk for MH and should be tested. Because masseter muscle rigidity can be one of the initial signs of MH and half of the patients exhibiting masseter muscle rigidity are susceptible to progressing to MH, it is recommended to stop infusion of the triggering agent. Refer the patient for testing rather than continue to expose the patient to a possible MH incident (Gupta & Hopkins, 2017). Central core disease is an inherited myopathy characterized by muscle weakness and hypotonia. Central core disease shares the same RYR1 gene mutations that MH exhibits. Patients with central core disease are more susceptible to MH and should be treated carefully (Litman et al., 2020).
Neuroleptic malignant syndrome (NMS) is characterized by hyperthermia, muscle rigidity, autonomic instability, and altered consciousness in patients receiving antidopaminergic agents. NMS is caused by an imbalance of neurotransmitters in the CNS and shares many symptoms with MH including an elevated temperature (although mild), muscle rigidity, tachycardia, and elevated CK levels. Although this syndrome is separate from MH, many clinicians believe it is safer to avoid triggering agents in patients who have experienced neuroleptic malignant syndrome to decrease the possibility of MH (Zhou, 2015; Gupta & Hopkins, 2017).
A variety of unusual conditions may resemble MH under anesthesia including sepsis, thyroid storm, pheochromocytoma, and iatrogenic overheating from sources such as warming blankets, heat lamps, and room temperature. MH is associated with more dramatic degrees of metabolic acidosis and venous desaturation (the decrease of oxygen in the venous blood) than these other diseases and conditions. Sepsis shares several characteristics with MH including fever, tachycardia, and metabolic acidosis (Gupta & Hopkins, 2017).
In patients with poorly controlled hyperthyroidism, thyroid storm can cause tachycardia, tachydysrhythmias (especially atrial fibrillation), hyperthermia, and hypotension. Thyroid storm presents with hypokalemia and generally develops postoperatively. Pheochromocytoma is a vascular tumor in the adrenal glands that produces and secretes norepinephrine and epinephrine. It is associated with dramatic increases in heart rate and blood pressure but not the rise in end-tidal carbon dioxide or hyperthermia as in an MH crisis. Iatrogenic overheating can come from sources such as heat lamps and humidifiers on the ventilator (Litman et al., 2020; Gupta & Hopkins, 2017).
After succinylcholine is administered, hyperkalemia can occur in young patients who have muscular dystrophy, causing sudden cardiac arrest. Patients with crush injury, burn patients, and patients with muscular dystrophy should not receive succinylcholine. Often confused with MH, sudden hyperkalemic cardiac arrest syndrome (increased levels of potassium that cause arrhythmia/asystole) can occur in young males during or shortly after receiving anesthesia (MHAUS, 2017a, Phillips, 2016).
When exposed to anesthetic triggering agents, these patients can develop life-threatening potassium levels that lead to dysrhythmias and muscle catabolism. The rise in potassium after giving succinylcholine mimics the hyperkalemia associated with MH. Therefore, it is best to avoid this drug with this patient population. These patients do not exhibit the classic rise in temperature or the marked muscle rigidity of MH (Rosenberg et al., 2005).
At-Risk Patients
MH is a genetically associated entity which is more commonly seen in males than in females. MH is either diagnosed after sustaining an acute crisis of MH after receiving an offending agent or via the caffeine halothane contracture test (CHCT). The CHCT is considered the gold standard for diagnosis of MH in suspected or at-risk patients and uses skeletal muscle that is newly biopsied. The test involves assessing for contracture of muscle fibers in the presence of halothane or caffeine (Gupta & Hopkins, 2017). The contracture test is performed after an open thigh muscle biopsy at a specially designated testing center. The fresh muscle is then exposed to increasing concentrations of halothane and caffeine within one hour of procurement. Because of the relative complexity of the test, only a few centers worldwide perform it. Four are in the U.S., and one
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is in Canada. If a muscle biopsy cannot be performed, genetic analysis is a reasonable method by which to identify mutations of the RYR receptor on the skeletal muscle. However, this is not always a conclusive method of diagnosing MH. Molecular genetics testing can be performed and sent by postal mail through several centers in the U.S. The Malignant Hypothermia Association of the United States lists locations for caffeine contracture and genetic testing for MH syndrome susceptibility at its website (Gupta & Hopkins, 2017).
DNA analysis, started in 1990, requires only a blood test and offers an alternative to CHCT. A negative DNA result, however, cannot be used alone to rule out susceptibility because of the heterogeneity of the disorder as well as dissimilarity within families (Gupta & Hopkins, 2017). Because of the vast number of mutations that may cause MH, a specific family mutation must be identified to perform a genetic test. Molecular genetic testing via RYR1 screening analysis is in its beginning stages and will become more useful as additional research identifying causative mutations is completed. It is worth noting that testing is expensive and not always covered by insurance (Gupta & Hopkins, 2017).
A sample test from buccal cells, white blood and muscle cells, or other tissues can be used for mutation analysis of RYR1. There are now screens for many common mutations (MHAUS, 2017b). Preoperative testing will be recommended for relatives of patients with known MH susceptibility and patients who experience suspicious clinical episodes of MH (Phillips, 2016; Gupta & Hopkins, 2017; Denholm, 2016).
Additional methods for testing have been used including the insertion of a microdialysis catheter directly into skeletal muscle and testing B-lymphocytes that harbor RYR1 protein and release calcium when stimulated with caffeine. Neither has been sufficiently validated to be clinically useful. Susceptible patients should undergo validated, trusted testing to protect themselves and their families from future surgery/anesthetic preventable MH complications (Phillips, 2016; Gupta & Hopkins, 2017).
Treatment
Rapid, effective treatment of MH requires simultaneous actions by all team members including nurses, surgical technologists, anesthesia staff, and other types of physicians. All staff should be familiar with the location of the MH supplies and the treatment protocol. Early recognition of MH is critical so that treatment can be started expediently. Interaction among team members is crucial to manage an MH crisis. An important step in the immediate treatment of suspected MH is for the anesthesiologist to stop the administration of triggering agents and flush the ventilator with 100% oxygen (Phillips, 2016; MHAUS, 2017b; MHAUS, 2017c; Isaak & Stiegler, 2016). Every member of the surgical team should have a specific role in the effort to prevent confusion and duplication of the treatment efforts (Isaak & Stiegler, 2016).
Rapid administration of dantrolene sodium (Dantrium® or Revonto®) is the first-line medication treatment for suspected MH. It is a direct-acting muscle relaxant. Introduced into clinical practice in 1979, dantrolene sodium continues to be the primary treatment for MH. The dantrolene sodium molecule is highly lipophilic (capable of dissolving in fats) which ensures a rapid crossing of the blood-brain barrier. It works by binding to the RYR1 receptor, thus inhibiting calcium release from the sarcoplasmic reticulum. Dantrolene sodium impairs the excitation-contraction coupling of the muscle cell membrane in muscular contraction. This leads to skeletal muscle relaxation and resolution of MH by restoring intracellular calcium balance and decreasing metabolism (MHAUS, 2017c).
Dantrolene sodium is supplied in glass vials that contain 20 mg of lyophilized dantrolene sodium, 3 grams of mannitol (Osmitrol®), and sodium hydroxide (for pH balance at 9.5), which are in a yellow powder form. Each vial requires 60 mL of sterile injectable water as a diluent and requires vigorous shaking for several minutes. Only preservative-free sterile injectable water should be used to reconstitute any form of dantrolene sodium because of the large quantities (around 700 mL) required for administration. It can take up to 36 vials of dantrolene sodium to stabilize and maintain the patient in an MH crisis (Phillips, 2016; MHAUS, 2017c).
The initial dose of dantrolene sodium is 2.5 mg/kg given IV every five minutes and then titrated to a suggested upper limit of 10 mg/kg as necessary based on lab results and clinical response (Phillips, 2016; Denborough, 1970; MHAUS, 2017b). A response is evident with muscular relaxation, lowered end tidal carbon dioxide levels, and improvement in tachycardia (MHAUS, 2017c). More may be given as needed. For example, a 70-kg person would require an initial dose of 175 mg, or nine vials, reconstituted every five minutes until MH symptoms subside, followed by repeated dosage as clinically indicated and/ or every 10 to 15 hours for 24 to 48 hours post-event (Zhou et al.,2015). The weight-based dosage for pediatric patients is the same as for adults (Phillips, 2016; MHAUS, 2017b). A critical preoperative assessment factor is to document every patient’s weight in kilograms for rapid use in the event of need for emergency medication (Phillips, 2016; MHAUS, 2017b; Denholm, 2016; Litman et al., 2019).
Summary of doses of dantrolene: • Initial Dose of dantrolene • 2.5 mg/kg given IV every five minutes a suggested upper limit of 10 mg/kg • Sample dose of dantrolene • 175 mg, or nine vials, reconstituted every five minutes until MH symptoms subside
Large quantities of reconstituted dantrolene sodium are needed. Therefore, a central line may be necessary.
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Muscular contractions make peripheral veins unreliable and incapable of handling large infusions. Tissue necrosis can result if the drug extravasates from a peripheral IV line. Dantrolene sodium is continued at 1 mg/kg every four to six hours after the crisis is controlled and is continued for 72 hours after the episode (Phillips, 2016; MHAUS, 2017b; MHAUS, 2017c). The perioperative nurse plays a helpful role in mixing this medication (Isaak & Stiegler, 2016; Litman et al., 2019). Slightly warmed sterile water, 96.8 F to 100.4 F (36 C to 38 C), can speed up the process. Dantrolene sodium is available in generic form. The recommended shelf supply is 36 vials to stabilize and treat the patient during an MH crisis. More drug should be available to maintain the patient postoperatively in the critical care or intensive care unit (Phillips, 2016; MHAUS, 2017b).
In July 2014, the U.S. Food and Drug Administration approved a new form of dantrolene sodium, Ryanodex®. It is supplied in 250-mg vials with 125 mg mannitol and requires only 5 mL of room temperature, non-bacteriostatic sterile water for reconstitution, minimizing the risk of fluid overload (MHAUS, 2017b). It can be mixed in 20 seconds by the same person who administers the drug. The benefits of this newer medication include that it can be mixed and given more quickly and efficiently along with a lower likelihood of fluid overload. A central line may not be necessary. The drug must be administered via syringe and not incorporated into an IV bag infusion. The rapid-dissolving formula in one vial is enough to stabilize the patient in crisis. Reconstituted dantrolene sodium should be protected from direct sunlight and must be used within six hours of mixing (Phillips, 2016).
Other diluents such as D5W or saline change the effects of dantrolene sodium. Lactated Ringer’s solution can increase metabolic acidosis and should not be used. Mannitol is included to protect the kidneys against myoglobinemia through diuresis (muscle damage causes myoglobin to be released into the bloodstream). The use of additional mannitol during the crisis should take into account the amount contained in the dantrolene during dosage calculations (Phillips, 2016; MHAUS, 2017b).
Dantrolene sodium should only be used in pregnant and lactating women if the benefits outweigh the risks (Phillips, 2016). The long-term risks of dantrolene on breastfeeding has not been fully researched. Some studies suggest that it can cause muscle weakness in the breastfed infant and also respiratory depression. It crosses the placenta, and it also passes into breast milk. Although considered relatively nontoxic and having no absolute contraindications, dantrolene sodium is used with caution in patients with hepatic disease and the elderly, because the liver metabolizes it (Phillips, 2016; MHAUS, 2017b).
Another treatment measure is to cool the patient by all routes available if core temperature is greater than 102.2 F (39 C). This can be done by instilling nasogastric lavage with iced solution; applying ice packs along heat transfer points like the groin, axilla, and neck or base of skull; and taking more aggressive measures as needed. Care needs to be taken so that as the crisis diminishes, the patient does not become hypothermic. Cooling procedures may conclude at a core temperature of < 100.4 F (<38 C) (MHAUS, 2017c). A Foley catheter should be inserted to monitor renal function. This catheter should not be used for cooling irrigations, because the irrigant could be confused with output. The goal is to maintain urine output at 300 mL per hour (Phillips, 2016).
Labs and blood gases should be drawn to evaluate electrolytes, potassium, CK levels, and coagulopathies. Sodium bicarbonate may be administered in the setting of severe acidosis. (MHAUS, 2017c). The urine should be observed for myoglobin. Hyperkalemia should be treated by typical protocols including hyperventilation with 100% oxygen and the administration of bicarbonate, albuterol, glucose, and insulin IV as needed. Administration of calcium chloride or calcium gluconate should be considered in cases of lifethreatening hyperkalemia (MHAUS, 2017c). Hyperkalemic patients with EKG changes should receive calcium to stabilize the cardiac membrane. However, there is a potential risk to worsen the MH as it is a calciumdriven pathology. CK levels track the severity of rhabdomyolysis, and repeated serum chemistry panels monitor renal function. Dysrhythmias, which might include sinus or ventricular tachycardia or ventricular fibrillation, should be treated symptomatically (Phillips, 2016). Calcium channel blockers, such as verapamil (Calan®, Verelan®), are contraindicated with dantrolene sodium as this drug combination might cause hyperkalemia (Phillips, 2016; Litman et al, 2020).
The Malignant Hyperthermia Association of the United States sponsors a 24-hour emergency hotline for medical professionals, 800-644-9737. Because many tasks and interventions must be completed rapidly, anesthesia staff should solicit extra help in caring for the patient (Isaak & Stiegler, 2016). This is one example of many in which the perioperative team member is a tremendous asset in caring for the patient with MH. After team members control the crisis, they continue with monitoring of
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and caring for the patient. The following interventions should be part of the patient’s care (Phillips, 2016; Litman et al, 2020; MHAUS, 2017c; Litman et al, 2019):
Because the patient is at risk for developing acute myoglobinuric renal failure, urine output should be maintained at 2mL/kg/hour by administering IV fluids followed by furosemide (Lasix) and mannitol. Kidney dysfunction can worsen if urine volume is reduced. Ideal output is 300 mL per hour.
The perioperative nurse should monitor the patient’s core body temperature. Patients can become hypothermic because of cooling measures. If the patient is hypothermic (97 F [36.1 C] or less), a warming blanket or forced air warmer may be provided and IV fluids warmed before infusion, if necessary.
High or low potassium levels (outside the normal range of 3.5 mEq/L to 5 mEq/L) should be assessed to prevent continued heart dysrhythmias. IV potassium boluses may be given slowly for hypokalemia. Hyperkalemia may be lowered by typical protocols which may include administering glucose and insulin; sodium bicarbonate; and albuterol, and by increasing ventilation, which drives potassium back into the cell.
The patient should be observed in the critical care or intensive care unit for 24 to 72 hours. The patient should be evaluated for the need for continued mechanical ventilation and administration of maintenance dantrolene sodium.
Elevated liver function values are often observed 12 hours to 36 hours after the event, as well as DIC with coagulopathy, thrombocytopenia, and abnormal bleeding.
Post-event, the patient may complain of muscle pain and weakness caused by the prolonged contractions. Pain medication may be necessary (Phillips, 2016).
The patient and family should be referred for MH testing, and required forms should be submitted to the MH registry at the North American Malignant Hyperthermia Registry(Phillips, 2016; Denholm, 2016; MHAUS, 2017a).
Preventive Measures
A thorough anesthetic history determines whether a patient or family member has experienced an MH episode in the past. If there is any likelihood of an episode, no triggering agents should be used, and tape should be placed over the inhalation agents in the OR. Regional or local anesthetics may be a more suitable choice for patients at risk for MH (MHAUS, 2017a). Several types of patients should avoid triggering agents because of their health history. For instance, patients with Duchenne muscular dystrophy and central core disease should not receive triggering agents. Patients younger than age 12 are at risk for undiagnosed muscular dystrophy. Therefore, succinylcholine should be avoided in elective procedures to prevent a hyperkalemic response (MHAUS, 2017a; Phillips, 2016).
All patients should have their core temperatures and end-tidal carbon dioxide monitored when under general anesthesia. The anesthesia machine should be prepared for a susceptible patient by flushing it with 100% oxygen at a rate of 10 L/minute for 20 minutes, using a fresh circuit and removing all the vaporizers. Examples of safe medications that may be used are barbiturates, benzodiazepines, opioids, and nitrous oxide (Phillips, 2016; Walter & Carraretto, 2016; Denholm, 2016; Litman et al., 2019).
Malignant hyperthermia is a life-threatening syndrome. Awareness of MH by all perioperative team members from those working in the preoperative holding area to those in the PACU is important in preventing negative patient outcomes (Phillips, 2016; Denholm, 2016; MHAUS, 2017a). The preoperative nurse plays a crucial role in averting an MH crisis by interviewing every surgical patient for a personal and family history of MH. Perioperative and perianesthesia nurses can help patients by being knowledgeable about the signs and symptoms of MH, implementing evidence-based care, and educating patients and families about MH. Finally, the entire OR team should coordinate its efforts to work efficiently together to ensure the best possible care for their patients (Phillips, 2016; Walter & Carraretto, 2016; Denholm, 2016; Litman et al., 2019).
Summary
Now that you have finished viewing the course content, you should have learned the following: • How to identify patients at risk for malignant hyperthermia • Information used to differentiate the early and late signs and symptoms of malignant hyperthermia • The diagnosis and treatment of malignant hyperthermia
Malignant hyperthermia is a lifethreatening syndrome associated with an anesthetic trigger. Perioperative and perianesthesia team members can help patients by being knowledgeable of the risks of malignant hyperthermia and its signs and symptoms, implementing evidence-based care, and educating patients and families about MH. Awareness of MH by all perioperative team members, from those working in the preoperative holding area to those in the PACU, is important in preventing negative patient outcomes. The preoperative nurse plays a crucial role in averting an MH crisis by interviewing every surgical patient for a personal and family history of MH.
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Course Contributors:
The content for this course was revised by Laura Stallings, CST, BS. Laura Stallings is a certified surgical technologist with a Bachelor of Science degree. She has worked in the OR since 2007. Laura is currently an instructor in the surgical technology program at Southeast Community College.
This course was reviewed by Daniel Migliaccio, MD. Dr. Migliaccio is currently faculty at the University of North Carolina at Chapel Hill, North Carolina. He is on the editorial board for Pediatric Emergency Medicine Reports. He completed his residency in Emergency Medicine at Stanford University. He has an Honors Certificate in Medical Education from the Clinical Teaching Scholars Honors Program at Stanford University. He is the Vice President of the Young Physician Section of American Academy of Emergency Medicine and has had editorial roles in various journals.
This course was edited by Relias staff writer Olive Peart, MS, RT (R) (M). Olive Peart is an established author, educator, and radiographer. She has authored several textbooks and regularly presents mammography and other radiography-related topics at seminars throughout the United States and Canada plus internationally via webinars.
Acknowledgment: Nancymarie Phillips, PhD, RN, RNFA, CNOR(E), and Dawn Demangone-Yoon, MD, were the previous authors of this educational activity but have not influenced the content of the current version of this course.
Clinical Vignette
Juan Martinez, a 20-year-old male, arrives at the OR for a laparoscopic cholecystectomy, and standard monitors are applied. His initial blood pressure is 133/74 mmHg, heart rate 95, and oxygen saturation 98% on room air. Juan is 5 feet 10 inches tall and weighs 195 pounds. He is given fentanyl (Sublimaze®) 150 mcg IV, propofol (Diprivan®) 200 IV, lidocaine (Xylocaine®) 50 mg IV, and succinylcholine (Anectine®) 180 mg IV as determined by his health history. After an uneventful induction and intubation with a No. 8 endotracheal tube, the patient is placed on oxygen at a 2-liter flow rate with desflurane (Suprane®) inhalation agent at 6% concentration. The nurse notes an elevated heart rate of 120 beats/minute and blood pressure of 180/105 mmHg, but she assumes it is in response to intubation. The nurse anesthetist adjusts the ventilator settings for a tidal volume of 900 mL and a respiratory rate of 10 breaths/minute. The end-tidal carbon dioxide (ETCO2) level reads 44 mmHg. She places an esophageal temperature probe and gets a core reading of 96.9 F (36.1 C). After several minutes, the patient’s vital signs are HR 144 beats/minute, BP 205/115 mmHg, ETCO2 55 mmHg, and temperature 98.2 F (36.8 C). Working with the nurse anesthetist, the RN troubleshoots the anesthesia circuit, increases the respiratory rate to blow off more carbon dioxide and draws a venous blood gas to send for evaluation. The nurse anesthetist gives an additional 100 mcg of fentanyl IV with no decrease in the patient’s heart rate. Upon closer examination, Juan exhibits muscle contracture in his jaw, alarming the staff and alerting the team to the possibility of malignant hyperthermia.
1. What venous blood gas and lab results would the clinician expect to find for Juan?
A. Hyperkalemia, hypercarbia with a PaCO2 greater than 60 mEq B. Hypokalemia, hypocarbia with a PaCO2 less than 45 mEq C. Hyponatremia, pH greater than 7.45 D. Hypernatremia, pH greater than 7.45
Feedback: High potassium results from the breakdown of muscle, and a rise in carbon dioxide of more than 60 mEq is the hallmark indicator of malignant hyperthermia.
2. What is the first step the anesthesia provider should take in caring for Juan?
A. Call for a stat portable chest X-ray B. Assess for a high fever C. Discontinue all triggering anesthetic agents and provide 100% oxygen D. Proceed with the surgery
Feedback: Rapid, effective treatment of MH requires simultaneous actions by the perioperative nurses and physicians. An important step in the immediate treatment of suspected MH is for the anesthesiologist to stop the administration of triggering agents and flush the ventilator with 100% oxygen.
3. Which agent was a trigger for Juan’s malignant hyperthermia episode?
A. Propofol B. Fentanyl C. Desflurane D. Lidocaine
Feedback: Inhalation agents, like desflurane (Suprane®), can trigger episodes of malignant hyperthermia.
4. Which test can definitively determine malignant hyperthermia in Juan?
a. DNA testing b. MRI c. CT scan d. Caffeine halothane contracture test
Feedback: Caffeine halothane contracture test is the best test for definitive diagnosis of malignant hyperthermia in the patient.
References
1. Chavez, L.O., Leon, M., Einav, S., Varon, J. (2016). Beyond muscle destruction: a systematic review of rhabdomyolysis for clinical practice. Critical Care, 20(1):135. doi: 10.1186/s13054-016-1314-5.
2. Denborough, M.A., Forster, J.F.A., Hudson, M.C., Carter, N.G., Zapf, P. (1970). Biochemical changes in malignant hyperpyrexia. Lancet, 295(7657):1137-1138. doi: 10.1016/S0140-6736(70)91214-6.
3. Denholm, B.G. (2016). Using informatics to improve the care of patients susceptible to malignant hyperthermia. AORN Journal, 103(4):365-376. e1-4. doi: 10.1016/j.aorn.2016.02.001. 4. Gupta, P.K., Hopkins, P.M. (2017). Diagnosis and management of malignant hyperthermia, BJA Education, 17(7), 249–254. https://doi. org/10.1093/bjaed/mkw079 5. Isaak, R.S., Stiegler, M.P. (2016). Review of crisis resource management (CRM) principles in the setting of intraoperative malignant hyperthermia. Journal of Anesthesia, 30(2):298-306. doi:10.1007/s00540-0152115-8.
6. Litman, R.S., Jones, S.B., Crowley, M. (2020). Malignant hyperthermia: Diagnosis and management of acute crisis. UpToDate. https://www. uptodate.com/contents/malignant-hyperthermia-diagnosis-and-management-of-acute-crisis 7. Litman, R.S., Smith, V.I.; Larach, M.G., Mayes, L.; Shukry, M.; Theroux, M.C.,Watt, S., Wong, C.A. (2019). Consensus statement of the malignant hyperthermia association of the United States on unresolved clinical questions concerning the management of patients with malignant hyperthermia, Anesthesia & Analgesia, 128(4), 652-659. doi: 10.1213/ ANE.0000000000004039
8. Malignant Hyperthermia Association of the United States (MHAUS). (2017a). Safe and unsafe anesthetics. http://www.mhaus.org/healthcareprofessionals/be-prepared/safe-and-unsafe-anesthetics 9. Malignant Hyperthermia Association of the United State (MHAUS). (2017b). FAQs: dantrolene. http://www.mhaus.org/faqs/dantrolene 10. Malignant Hyperthermia Association of the United States (MHAUS). (2017c). Managing an MH crisis. http://www.mhaus.org/healthcareprofessionals/managing-a-crisis 11. Phillips, N. (2016). Berry and Kohn’s Operating Technique. 13th ed. Elsevier; 617-619.
12. Rosenberg, H., Ganesh, A., Saubermann, A. J., & Nicolson, S. C. (2005). MHAUS reports 3 unique cases of hyperkalemic cardiac arrest. Anesthesia Patient Safety Foundation, 20(2). https://www.apsf.org/article/ mhaus-reports-3-unique-cases-of-hyperkalemic-cardiac-arrest/ 13. Walter, E.J., & Carraretto, M. (2016). The neurological and cognitive consequences of hyperthermia. Critical Care, 20(1):199. doi: 10.1186/ s13054-016-1376-4.
14. Zhou, J., Diptiman, B., Allen, P.D., Pessah, I.N. (2015). Malignant hyperthermia and muscle-related disorders. In: Miller RD, Cohen NJ, Eriksson LI, et al, eds. Miller’s Anesthesia. 8th ed. Saunders.
Answer: D, Caffeine halothane contracture test. agents and provide 100% oxygen. 3. Answer: C, Desflurane. 4. than 60 mEq. 2. Answer: C, Discontinue all triggering anesthetic 1. Answer: A, Hyperkalemia, hypercarbia with a PaCO2 greater
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Hospitals and ambulatory surgery centers (ASCs) must comply with myriad rules, regulations and laws from an alphabet soup of regulatory entities. These include CMS, OSHA, HHS, the CDC and HIPPA, to name just a few.
Penalties and fines for failure to comply with these entities’ various rules and regs can be severe. This makes compliance a critical issue for every health care organizations.
What is Health Care Compliance?
Beverly Kirchner, BSN, RN, CNOR, CNAMB, has been actively involved in helping hospitals and ASCs understand and meet their compliance responsibilities. She defines compliance for health care organizations as “an ongoing process undertaken to meet or surpass the legal, principled (ethical and moral) and professional standards that are valid and relate to the health care organization and its departments.”
Kirchner says that CMS regulations for hospitals and ASCs are a major part of compliance. “Each entity has its regulations, but in the case of hospitals ORs, several regulations cross to both departments, such as life safety, construction and HIPAA.”
“OSHA and HIPAA are also a big part of ASC and hospital compliance requirements and responsibilities,” Kirchner adds. “And states have licensure laws with which hospitals and ASCs must comply.”
David Hoffman is the founder of David Hoffman & Associates, PC, a consulting firm that focuses on legal, regulatory and clinical compliance for health care organizations. Before starting his firm, Hoffman was a federal prosecutor specializing in health care fraud.
“Compliance for hospitals and ASCs is a very complex, highly regulated issue,” says Hoffman. “It takes a lot of teamwork among health care staff to make sure that all of the applicable rules and regulations are being followed.”
According to Hoffman, health care organizations are subject to the Federal False Claims Act, which is a federal civil fraud statute. The penalties for filing false medical claims currently range from $11,803 and $23,607 per claim, he says.
But that’s just the beginning – treble damages, or triple this amount, are also added. “Now you’re into some real money,” says Hoffman.
The intent standard under the False Claims Act covers knowledge of false information resulting in submission or causing the submission of a false claim. According to Hoffman, this is defined not only as having actual knowledge of a false claim, but also as deliberate ignorance of the truth or falsity of the information or reckless disregard of the truth or falsity of the information.
“So the ‘ostrich’ defense doesn’t work,” says Hoffman. “Physicians can’t put their heads in the sand and say billing and proper coding are not their responsibility.”
The Main Question
manager, surgical services division at Parkland Hospital in Dallas, Texas, the main question when it comes to the consequences of not following laws and regulations is whether the event falls under ordinary non-compliance or gross negligence?
“In other words, did the facility know whether they placed the patient in danger or not?” says Buchert. “Non-compliant facilities tend to be at risk for higher financial losses, license revocation, business disruption, poor patient outcomes, loss of trust from patients and staff, and reputation damage. And this is all in addition to potential fines.”
Loss of Medicare certification is another potential consequence of non-compliance, says Kirchner. “In a worst-case scenario, the facility could actually close,” she says.
According to Kirchner, health care facilities that are found to be non-compliant may be able to avoid closure by working with the Office of Inspector General (OIG). “The OIG has the authority to enter into an agreement known as a corporate integrity agreement, or CIA, in exchange for not sanctioning an organization,” says Kirchner. “We have learned that these agreements usually last five years and the organization must hire a monitor to oversee the quality of care.”
Role of the Compliance Officer
One of the most important keys to remaining in compliance is formalizing an ethics and compliance program. And this starts with appointing a compliance officer.
“A compliance officer is an employee who ensures the organization is compliant with outside regulatory and legal requirements,” says Kirchner. “The compliance officer also ensures that internal
- J.D. Buchert, MSN, M.Ed., MS, RN
policies, procedures and bylaws are honored and followed while monitoring day-to-day behaviors within the organization.”
A compliance officer will also audit and test processes to ensure compliance, disclose any breaches, and check the compliance hotline regularly, investigating any complaints. Kirchner lists five key tasks of a health care compliance officer: 1. Identification of problems or potential problems 2. Problem prevention 3. Monitoring and detecting problems 4. Problem resolution 5. Providing advice about compliance
According to Kirchner, there are two kinds of health care compliance officers:
• A Corporate Compliance Officer (or Chief Compliance
Officer) leads large groups of people toward obeying standards of conduct. These include bylaws, codes of conduct and policies and procedures.
• A Regulatory Compliance Of-
ficer ensures the organization follows all industry standards, rules and regulations. “He or she is responsible for ensuring the organization has internal controls to manage risks, monitor for compliance, identify potential compliance risk, develop strategies to mitigate risk and oversee policy development,” says Kirchner.
Buchert elaborates. “The corporate compliance officer’s focus is on ensuring that hospital processes and procedures comply with the law,” he says. “The regulatory compliance officer focuses on the internal aspect of controlling and managing risk by integrating standards from outside sources into the practice setting.”
Kirchner says that health care compliance officers should possess a number of key skills including: • Risk assessment • Conflict management • Integrity • The ability to interpret data • Detail orientation • Communication • Problem-solving
“The health care compliance officer reports directly to the facility’s governing board,” says Kirchner. “The compliance officer assures the governing board that the facility’s leadership and team are compliant with all relevant laws, rules and regulations; that there is a compliance plan and code of conduct in place; and that compliance reporting is completed per state, federal and accreditation body requirements.”
Staffing in ASCs usually doesn’t allow for a dedicated compliance officer with no other responsibilities, says Kirchner.
“This is unlike most hospitals, where the compliance officer usually has a whole team helping oversee auditing and compliance,” she says.
“If ASCs can afford to hire a dedicated compliance officer,” Kirchner adds, “I recommend they do so to help ensure the facility remains in compliance.”
Biggest Compliance Challenges
Hospitals and ASCs face a number of challenges when it comes to remaining in compliance. At the top of the list is simply keeping up with all of the laws, rules and regulations they have to follow, along with changes that are made, usually at least annually.
Updating their facilities’ compliance plan and its policies and procedures and performing audits are other big compliance challenges faced by hospitals and ASCs.
Kirchner says that staff education is critical to meeting the biggest compliance challenges. “How can you achieve compliance if the staff doesn’t know what regulations, laws and accrediting standards must be followed?” she says.
Hoffman agrees. “Knowledge and education are key,” he says. “Physicians and billing departments need to be aware of their compliance obligations and know what all the rules and risks are.”
Buchert says high levels of physician and nurse turnover magnify compliance challenges. “The recent mass exodus of health care workers is definitely making compliance more difficult,” he says.
Buchert points to recent changes in filing for Medicare and private insurance reimbursement as an example of how turnover can lead to compliance problems. “If a health care facility improperly files reimbursement by accident, this can be construed as fraud and abuse of the payment system,” he says. “That’s why compliance with proper coding and billing is critical.”
Hoffman believes that the biggest compliance issue facing hospitals and ASCs is medical necessity. “Is there really a need for the surgery?” he says. “Is it medically necessary? Billing for unnecessary procedures is a big compliance concern today.”
Create a Culture of Compliance
According to Hoffman, the most important thing hospitals and ASCs can do to remain in compliance is to create a culture of compliance that starts at the top.
“You’ve got to have buy-in from the C-suite,” he says. “Compliance is everyone’s responsibility, including upper management.”
“HHS OIG guidance calls for hospitals and other health care organizations to create a culture of compliance,” adds Kirchner. “This type of culture endorses preventing, detecting and resolving behavior that does not follow government and state laws, including public and private health care program requirements.”
Buchert stresses the importance of building employee engagement.
“All employees need to understand their obligations and the role they play in keeping the facility compliant,” he says. “This includes identifying areas of weakness and focusing on a plan to rectify potential problems.”
Hospitals and ASCs should also maintain all documentation and data related to their compliance efforts. “This includes audit procedure records from the patients’ first moments in the facility to when they are discharged to determine if policies and procedures were followed,” says Buchert.
“This will provide a foundation for understanding what is happening in the facility,” he adds. “It’s a quality role that can save the facility money in the long run.”
Buchert also recommends forming an interdisciplinary team to focus on compliance. “The compliance officer should chair this team and set forth responsibilities for administration and enforcement of the compliance process,” he says.
Kirchner points out that even with a full-time compliance officer, the officer’s scope is limited.
“It’s hard to keep up with all the different departments and all the rules, regulations and laws that govern health care,” she says. “Department leaders and staff need to be aware of the compliance requirements for their areas and work with the compliance officer to ensure the department is meeting and exceeding them.”
Benefits of Focusing on Compliance
Taking appropriate steps to make sure your hospital or ASC remains in compliance brings benefits beyond minimizing financial losses, business disruption and license and certification revocation.
“As a former military man, I find that compliance brings organization and serenity to a health care facility,” says Buchert. “When a facility is compliant, it runs like a smoothly oiled machine. All parts work well together to move the whole unit forward successfully.”
“A compliant facility can reward its staff and patient population on many levels,” Buchert adds. “But the pride carried by a compliant facility truly does lead to higher employee retention, success as a business and better patient outcomes.”
“Simply put, compliance means safety,” says Kirchner. “It helps ensure that the staff, patients and visitors are in an environment that promotes respect, transparency and quality care. Compliance not only protects the people, but it protects the business as well.”
