mivacurium and Metabolism--Inborn-Errors

Butyrylcholinesterase deficiency prolongs the effects of the drugs it degrades; succinylcholine and mivacurium. Existing literature on butyrylcholinesterase deficiency is dominated by genetic and biochemical studies. We searched MEDLINE, Embase, Web of Science and Biosis to systematically review the causes and clinical consequences of butyrylcholinesterase deficiency. We considered outcomes clinically relevant if neuromuscular blockade, induced by succinylcholine or mivacurium, was assessed using clinical criteria or neuromuscular monitoring. We included 66 studies: 25 randomised controlled trials; 13 clinically controlled trials; 26 prospective observational studies; 1 retrospective study; and 1 qualitative study. Data heterogeneity precluded quantitative synthesis. Studies described genetic, physiological, acquired or pharmacologically induced causes of butyrylcholinesterase deficiency. The prolongation of neuromuscular blockade by butyrylcholinesterase deficiency was most pronounced with homozygosity of a genetic variant, but other more common factors included increasing age, pregnancy, severe liver disease, burn injuries and drug interactions.

Topics: Anesthesia; Apnea; Butyrylcholinesterase; Humans; Metabolism, Inborn Errors; Mivacurium; Neuromuscular Blockade; Neuromuscular Monitoring; Succinylcholine

Takotsubo cardiomyopathy is an acute syndrome characterized by cardiac failure from disturbances in the contractility of the left ventricle. It is presumably caused by sympathetic over stimulation. We describe a case of postoperatively developed Takotsubo cardiomyopathy in a 69-year-old female. The syndrome developed in connection with awareness during complete residual paralysis. The literature on this syndrome is reviewed and implications for anaesthesia described.

Topics: Aged; Anesthesia, Intravenous; Apnea; Butyrylcholinesterase; Delayed Emergence from Anesthesia; Female; Humans; Intraoperative Awareness; Isoquinolines; Laryngeal Diseases; Metabolism, Inborn Errors; Mivacurium; Myocardial Infarction; Neuromuscular Nondepolarizing Agents; Polyps; Postoperative Complications; Takotsubo Cardiomyopathy; Vocal Cords

Pseudocholinesterase deficiency, sometimes called butyrylcholinesterase deficiency, is a rare disorder in which the neuromuscular blocking drugs succinylcholine and mivacurium cannot be metabolized properly in the blood plasma. This disorder can either be acquired as a result of certain comorbidities or it can be inherited genetically. Anesthesia providers must understand the pathophysiology of pseudocholinesterase deficiency and be prepared to safely and effectively manage patients who show signs and symptoms consistent with the disorder after the use of the indicated neuromuscular blocking drugs. This article summarizes the pharmacologic and physiologic data relevant to understanding the basic pathophysiology associated with pseudocholinesterase deficiency and illustrates a case study of a young woman suspected of having the disorder after a prolonged delay in emergence from general anesthesia.

Topics: Apnea; Butyrylcholinesterase; Female; Humans; Metabolism, Inborn Errors; Mivacurium; Succinylcholine

Pseudocholinesterase or butyrylcholinesterase (BChE) inactivates the relaxant drugs mivacurium and suxamethonium. A deficiency in plasma activity of this enzyme may result in prolonged muscular paralysis and subsequently the need for an extended duration of mechanical ventilation. We report the case of a 65-year-old patient who was diagnosed with butyrylcholinesterase deficiency for the first time during elective surgery. Neuromuscular monitoring constitutes a central diagnostic asset in ensuring patient safety.

Topics: Aged; Anesthesia Recovery Period; Anesthesia, General; Apnea; Butyrylcholinesterase; Humans; Intraoperative Awareness; Isoquinolines; Male; Metabolism, Inborn Errors; Mivacurium; Monitoring, Intraoperative; Neuromuscular Depolarizing Agents; Neuromuscular Nondepolarizing Agents; Preanesthetic Medication; Succinylcholine

Mutations in the butyrylcholinesterase gene can lead to a prolonged effect of the neuromuscular blocking agents, succinylcholine and mivacurium. If the anaesthesiologist is not aware of this condition, it may result in insufficient respiration after tracheal extubation. However, this can be avoided with the use of objective neuromuscular monitoring if used adequately. Three case reports of prolonged effect of succinylcholine or mivacurium were presented to illustrate the importance of neuromuscular monitoring during anaesthesia. In the first case, continuous intraoperative neuromuscular monitoring allowed a prolonged neuromuscular blockade to be discovered prior to tracheal extubation of the patient. The patient was extubated after successful reversal of the neuromuscular blockade. On the contrary, neuromuscular monitoring was not used during anaesthesia in the second patient; hence, the prolonged effect of the neuromuscular blocking agent was not discovered until after extubation. In the third patient, the lack of response to nerve stimulation was interpreted as a technical failure and the prolonged effect of succinylcholine was discovered when general anaesthesia was terminated. Both patients had insufficient respiration. They were therefore re-sedated, transferred to the intensive care unit and the tracheas were extubated after full recovery from neuromuscular blockade. We recommend the use of monitoring every time these agents are used, even with short-acting drugs like succinylcholine and mivacurium.

Topics: Accelerometry; Aged; Antidotes; Apnea; Appendicitis; Butyrylcholinesterase; Cholecystectomy, Laparoscopic; DNA Mutational Analysis; Female; Femoral Neck Fractures; Genotype; Humans; Hypnotics and Sedatives; Isoquinolines; Laparoscopy; Metabolism, Inborn Errors; Middle Aged; Mivacurium; Neostigmine; Neuromuscular Blockade; Neuromuscular Depolarizing Agents; Neuromuscular Monitoring; Neuromuscular Nondepolarizing Agents; Respiration, Artificial; Respiratory Paralysis; Succinylcholine; Time Factors; Young Adult

Topics: Apnea; Butyrylcholinesterase; Cholinesterases; Documentation; Drug Hypersensitivity; Follow-Up Studies; Humans; Isoquinolines; Medical Records; Metabolism, Inborn Errors; Mivacurium; Neuromuscular Depolarizing Agents; Neuromuscular Nondepolarizing Agents; Succinylcholine

Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adjuvants, Anesthesia; Anesthesia Recovery Period; Anesthesia, Intravenous; Anesthetics, Intravenous; Endoscopy; Gastrostomy; Humans; Infant; Isoquinolines; Male; Metabolism, Inborn Errors; Midazolam; Mivacurium; Monitoring, Intraoperative; Neuromuscular Nondepolarizing Agents; Piperidines; Remifentanil