The following articles discuss the use of anaesthesia in people with mitochondrial disease:

  • Hsieh VC, Krane EJ, Morgan PG.  Mitochondrial Disease and Anesthesia.  J Inborn Errors Metab Screen.  2017, Volume 5: 1–5.  DOI:  10.1177/2326409817707770

This article seems to provide the most comprehensive set of recommendations in a free text article, e.g.

  • … mitochondrial dysfunction most commonly affects the function of the central nervous system, the heart, the gastrointestinal (GI) tract, and the muscular system.  These same systems are strongly affected by anesthetics.
  • Mitochondrial patients often require smaller doses of general anesthetics, local anesthetics, sedatives, analgesics, and neuromuscular blockers (paralytics) to achieve the desired end points.  Furthermore, it is important to avoid increasing the metabolic burden of patients with MD by not requiring prolonged fasting, and preventing hypoglycemia, postoperative nausea and vomiting, hypothermia (with resulting shivering), prolonged orthopedic tourniquet application, acidosis, and hypovolemia.
  • It is worth reminding the reader that MD is not 1 disorder but represents hundreds of different enzymatic mitochondrial defects, both genetic and environmental in origin.
  • … and often add L-Carnitine to their IV fluids in patients who have shown a strong clinical response to the drug. 
  • The depolarizing muscle relaxant succinylcholine should not be administered in any patient with myopathy out of concern for their upregulation of nicotinic acetylcholine receptors in skeletal muscle, resulting in potentially lethal acute hyperkalemia and acute myolysis after succinylcholine administration.
  • Maintenance anesthetics (volatile agents or intravenous drugs) should be titrated incrementally, slowly, and carefully, while monitoring anesthetic depth clinically or ideally with the BIS or other processed electroencephalography system if available.
  • The following have been safely, but anecdotally, used to provide anesthesia without causing recognizable mitochondrial or physiologic decompensation:
    • small intravenous boluses of propofol, benzodiazepines, or ketamine;
    • continuous infusion of dexmedetomidine;
    • inhalation of sevoflurane; and
    • bolus dosing or continuous infusion of short- or ultrashort-acting opioids such as fentanyl, sufentanil, alfentanil, or remifentanil.
  • ... the anesthesiologist must recognize that patients with MD have heightened sensitivity or even untoward responses to many medications routinely used in anesthesiology.  Intravascular volume and hydration status require close attention, with no administration of lactate and, when appropriate, addition of intravenous glucose.
  • Brody KM.  Anesthetic Management of the Patient with Mitochondrial Disease:  A Review of Current Best Evidence.  AANA J. 2022 Apr;90(2):148-154.  PMID:  35343897.

No free text version available so it hasn’t been possible to review this article, but as it is more recent than most of the other articles here, it would be worth reviewing.

Anaesthesia Tutorial of the Week by authors at The Children’s Hospital at Westmead.  The main difference to the Hsieh article above is:

  • Evidence regarding propofol is conflicting:  a recent review [the Hsieh article] suggests that although propofol boluses are probably safe in mitochondrial diseases, infusions may not be.  Our opinion, from assessment of published literature, is that propofol is not the anaesthetic agent of choice; careful titration of propofol boluses in patients without severe forms of mitochondrial diseases and without critical illness is probably safe.  However, propofol infusions should probably be avoided.

Includes a table of perioperative medications and their safety in patients with mitochondrial disease.

  • Yeoh C, Teng H, Jackson J, Hingula L, Irie T, Legler A, Levine C, Chu I, Chai C, Tollinche L.  Metabolic Disorders and Anesthesia. Curr Anesthesiol Rep 9, 340–359 (2019).  DOI:  10.1007/s40140-019-00345-w  Epub 2019 Jul 12.  PMID:  31406490; PMCID:  PMC6690620.

This article covers anaesthesia in people with a range of metabolic disorders.

The section on mitochondrial disease (starting on page 14 of the PMC version) summarises the use of anaesthetics in two paragraphs and so provides a very good reference, while the earlier paragraphs summarise the causes of primary mitochondrial disease and their implications for the use of anaesthetics.