Aborted cardiac arrest in a patient carrying KCNE1 D85N variant during the postpartum period.

The postpartum period is associated with an increased risk of fatal ventricular tachyarrhythmias in patients with congenital long QT syndrome (LQTS). We describe a 20-year-old female with LQTS who experienced aborted cardiac arrest during the postpartum period. Genetic screening identified a KCNE1 D85N variant, which has been reported to be a LQTS-causing gene variant, in the patient and her younger sister, whose QT interval was also prolonged. Since the allele frequency of this variant is almost 1%, it may be important to clarify whether such variant carriers indeed have an increased risk of fatal ventricular tachyarrhythmias during the postpartum period.