Carnitine is essential for the transport of long-chain fatty acids from the cytoplasm to the mitochondrial matrix. The carnitine shuttle transports long-chain fatty acylcarnitine to the mitochondrial matrix. Subsequently, long-chain fatty acyl CoA, which is split from long-chain fatty acylcarnitine by carnitine palmitoyltransferase II, undergoes fatty acid β-oxidation. Acetyl CoA is produced from long-chain fatty acyl CoA via fatty acid β-oxidation and aids in the synthesis of adenosine triphosphate via the tricarboxylic acid cycle and electron transport chain. In addition, in the fasting state, it leads to ketone body production in the liver and glucose production via gluconeogenesis. However, patients with compromised fatty acid β-oxidation, owing to carnitine deficiency as well as defects in carnitine transport and the fatty acid β-oxidation pathway, develop hypoglycemia, cardiomyopathy, arrhythmia, and hypotonia. These conditions are attributed to the accumulation of released fatty acids and acylcarnitine. This review aimed to shed light on the anesthetic management of patients with compromised fatty acid β-oxidation undergoing various surgeries by assessing relevant case reports associated with fatty acid β-oxidation disorder in PubMed. Pre-anesthetic and intraoperative evaluation should include monitoring of glucose and carnitine levels and specific cardiac tests, such as echocardiography. Considering that propofol is dissolved in 10% long-chain fatty acids, propofol infusion should be avoided because of increased long-chain fatty acid loading in patients with compromised fatty acid β-oxidation. Thus, anesthesia using opioids (remifentanil and fentanyl), midazolam, dexmedetomidine, etomidate, and non-depolarizing neuromuscular blocking agents would be appropriate in such patients.

A 46-year-old woman demonstrated refractory Kounis syndrome (KS) after induction of anesthesia. Despite conventional management of anaphylaxis and advanced cardiac life support, her cardiovascular function continued to deteriorate until she had a cardiac arrest, and after extracorporeal membrane oxygenation (ECMO) therapy, electrical cardiac activity reappeared. A large number of patients with KS—“allergic angina syndrome”—has been known to recover well with vasodilators; however, this patient showed antibiotics-induced refractory KS during general anesthesia. Severe bronchospasms with desaturation appeared as initial anaphylactic features; however, these did not respond to conventional treatment for anaphylaxis. Patient’s hemodynamic signs eventually worsened, leading to cardiac arrest despite ephedrine administration and chest compressions. During cardiopulmonary cerebral resuscitation, the central line was secured, and epinephrine, atropine, as well as sodium bicarbonate were administered repeatedly; nevertheless, cardiac arrest was sustained. After initiation of veno-arterial ECMO, atrial fibrillation was observed, which was later converted to sinus tachycardia by electrical cardioversions and amiodarone. Coronary angiography was performed before the patient was admitted to the intensive care unit; there were no indications of an impending cardiac arrest. The patient was discharged uneventfully owing to early use of ECMO despite the emergence of KS symptoms that were initially masked by anesthesia but later worsened abruptly.

Lipid emulsion is used to treat systemic toxicity caused by local anesthetics. In addition, lipid emulsion was reported to be effective in ameliorating cardiovascular depression evoked by non-local anesthetic drug toxicity with high lipid solubility. A 47-year-old woman underwent local anesthetic infiltration with 40 mL of 2% lidocaine (20 and 20 mL) to remove a mass in the upper back. After operation, she experienced convulsions and loss of consciousness due to lidocaine toxicity. Midazolam followed by lipid emulsion was administered to treat central nervous system symptoms including unconsciousness and decreased Glasgow Coma Scale. The patient recovered from unconsciousness and presented improved Glasgow Coma Scale after lipid emulsion administration, and then fully recovered from local anesthetic systemic toxicity. This case suggests that early lipid emulsion treatment, before further progression of local anesthetic systemic toxicity, provides an enhanced recovery from unconsciousness and decreased Glasgow Coma Scale due to lidocaine toxicity.