Researchers analyzed anesthetic management in patients with compromised fatty acid β-oxidation undergoing surgery and showed that meticulous monitoring is warranted, according to their results published in Medicine.

Carnitine is essential for the transport of long-chain fatty acids from the cytoplasm to the mitochondrial matrix. It hence plays a major role in lipid metabolism and energy production via fatty acid β-oxidation. Fatty acid β-oxidation disorders, such as long chain fatty acid oxidation disorder (LCFAOD), are caused by carnitine deficiency and defects in the carnitine shuttle.

“The stress response to surgery and disease causes a derangement of metabolic and physiological responses, ultimately inducing hypercatabolism and hypermetabolism,” the research team wrote.


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Read more about LCFAOD etiology 

The research team set out to shed light on the importance of anesthetic management in patients with this disease. They conducted a literature review on PubMed from March 11, 2021, to August 15, 2021, using various terms related to “carnitine” and “anesthesia.” They found 31 case reports that they included in their analysis.

From the literature review conducted, the researchers wrote that preanesthetic examinations and tests should include electrocardiography, echocardiography, blood glucose, creatinine kinase, carnitine, and serum transaminase. These are important to ensure that the patient is optimized for surgery and that one of the most important things to avoid during surgery is intraoperative hypoglycemia.

Given that propofol is dissolved in 10% long-chain fatty acids, the research team suggested that it should be avoided and that anesthesia using opioids, midazolam, etomidate, dexmedetomidine, and nondepolarizing neuromuscular blocking agents were more appropriate. General anesthesia using inhalational anesthetics and succinylcholine should be avoided to prevent malignant hyperthermia from occurring.

The research team concluded that meticulous monitoring is warranted when administering anesthesia to patients with compromised fatty acid β-oxidation to ensure that the patients remain safe during surgery. 

Reference

Yu HK, Ok SH, Kim S, Sohn JT. Anesthetic management of patients with carnitine deficiency or a defect of the fatty acid β-oxidation pathway: a narrative review. Medicine. 2022;101(7):e28853. doi:10.1097/MD.0000000000028853