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  • Most affected patients were of Venezuelan maternal heritage.
  • Many reportedly received sevoflurane; some received propofol (relative risk unknown).
  • Genetic testing in some cases identified a mitochondrial ND4 mutation (mtND4 m.11232T>C) affecting Complex I of the electron transport chain.
  • Volatile anesthetics and propofol are known to inhibit Complex I.
  • There is no point-of-care screening test available.
  • Negative family history does not exclude risk.
  • All mitochondrial DNA is maternally inherited; therefore, any patient with direct maternal Venezuelan lineage should be considered potentially at risk.
  • Consider asking about maternal Venezuelan ancestry during preoperative assessment.
  • Approach these discussions with care and sensitivity.
  • Use clinical judgment, as evidence remains incomplete and evolving.
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