Patient Safety Brief:
Anesthetic Recommendations for Patients of Venezuelan Descent with ND4 Mitochondrial Mutation
Recent reports have identified a rare but serious patient safety issue in pediatric and adult patients with Venezuelan ancestry, linked to a maternally inherited mtND4 mutation in a subunit of mitochondrial complex I. This mutation appears to cause hypersensitivity to volatile anesthetics, particularly sevoflurane, as volatile anesthetics inhibit mitochondrial respiration. Reported outcomes include basal ganglia infarcts, failure to wake from anesthesia, severe neurologic injury, and death following otherwise routine anesthetics. Affected patients are typically asymptomatic prior to anesthetic exposure. Optimal anesthetic management for this patient population has not yet been definitively established, therefore anesthesia professionals should employ proactive risk-reduction strategies. AANA offers the following practice considerations:
Pre-Operative Assessment
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Assess family history. Ask the patient about Venezuelan maternal lineage (e.g., Was your mother or maternal grandmother born in Venezuela?) + A negative familial history does not eliminate the potential risk associated with this genetic mutation. + Paternal ancestry is not relevant; mitochondrial DNA is inherited exclusively from the mother.
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Ask about maternal family history of delayed awakening, unexplained neurologic injury, ICU admission after anesthesia, or sudden death following minor procedures.