Incidence of life-threatening events in children with Wolff-Parkinson-White syndrome: Analysis of a large claims database

Heart Rhythm. 2022 Apr;19(4):642-647. doi: 10.1016/j.hrthm.2021.12.009. Epub 2021 Dec 10.


Background: Previous estimates of life-threatening event (LTE) risk in Wolff-Parkinson-White (WPW) syndrome are limited by selection bias inherent to tertiary care referral-based cohorts.

Objective: This analysis sought to measure LTE incidence in children with WPW syndrome in a large contemporary representative population.

Methods: A retrospective cohort study was conducted using claims data from the IBM MarketScan Research Databases, evaluating subjects with WPW syndrome (age 1-18 years) from any encounter between January 1, 2013, and December 31, 2018. Subjects with congenital heart disease and cardiomyopathy were excluded. The primary outcome was diagnosis of ventricular fibrillation (VF); a composite outcome, LTE, was defined as occurrence of VF and/or cardiac arrest. VF and LTE rates were compared to matched representative controls without WPW syndrome (3:1 ratio).

Results: The prevalence of WPW syndrome was 0.03% (8733/26,684,581) over a median follow-up of 1.6 years (interquartile range 0.7-2.9 years). Excluding congenital heart disease/cardiomyopathy, 6946 subjects were analyzed. An LTE occurred in 49 subjects (0.7%), including VF in 20 (0.3%). The incidence of VF was 0.8 events per 1000 person-years, and the incidence of LTE was 1.9 events per 1000 person-years. There were no occurrences of VF in controls; the rate of LTE was 70 times higher in subjects with WPW syndrome (0.7%; 95% confidence interval 0.5%-0.9%) than in controls (0.01%; 95% confidence interval 0%-0.02%).

Conclusion: The use of a large claims data set allowed for an evaluation of VF and LTE risk in an unselected pediatric population with WPW syndrome. The observed range of 0.8-1.9 events per 1000 person-years is consistent with prior reports from selected populations. A comparison of event rates to matched controls confirms and quantifies the significant elevation in VF and LTE risk in pediatric WPW syndrome.

Keywords: Atrial fibrillation; Life-threatening event; Outcomes; Sudden cardiac death; Ventricular fibrillation; Wolff-Parkinson-White syndrome.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Humans
  • Incidence
  • Infant
  • Prevalence
  • Retrospective Studies
  • Ventricular Fibrillation / epidemiology
  • Wolff-Parkinson-White Syndrome* / complications
  • Wolff-Parkinson-White Syndrome* / diagnosis
  • Wolff-Parkinson-White Syndrome* / epidemiology