People with alternating hemiplegia of childhood undergoing general anesthesia are at risk for severe complications including critical bradycardia and hemiplegic incidents. We performed a 10-year historical cohort study and completed a subgroup analysis of patients who specifically underwent general anesthesia. Patients with ATP1A3 positive variants were compared with those without such variants. The 14 patients reviewed underwent general anesthesia a combined total of 39 times. Two patients had a short QTc documented prior to anesthesia and presented with bradycardia in the recovery room. Two other patients experienced a suspected seizure and hemiplegic incident; one of those patients received no facility-administered medications prior to experiencing a hemiplegic incident, and the other experienced a hemiplegic incident on emergence from anesthesia with subsequent cardiac arrest and successful resuscitation. No apparent association was identified between type of anesthesia and the development of bradycardia or hemiplegic incidences. Of the four patients experiencing critical events, three had the ATP1A3-D801N variant. Although additional research is necessary to confirm such associations, patients with alternating hemiplegia of childhood, specifically the ATP1A3-D801N variant, appear to be at elevated risk for complications associated with general anesthesia, suggesting the need for an increased anticipation of complications for this population.
Keywords: ATP1A3 syndrome; alternating hemiplegia of childhood (AHC); pediatric anesthesia; short QT syndrome.
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