Association of Wolff-Parkinson-White With Left Ventricular Noncompaction Cardiomyopathy in Children

J Card Fail. 2019 Dec;25(12):1004-1008. doi: 10.1016/j.cardfail.2019.09.014. Epub 2019 Oct 15.


Background: Wolff-Parkinson-White (WPW) has been associated with left ventricular noncompaction (LVNC) in children. Little is known about the prevalence of this association, clinical outcomes, and treatment options.

Methods: Retrospective review of subjects with LVNC. LVNC was defined by established criteria; those with congenital heart disease were excluded. Electrocardiograms (ECGs) were reviewed for presence of pre-excitation. Outcomes were compared between those with isolated LVNC and those with WPW and LVNC.

Results: A total of 348 patients with LVNC were identified. Thirty-eight (11%) were found to have WPW pattern on ECG, and 84% of those with WPW and LVNC had cardiac dysfunction. In Kaplan-Meier analysis, there was significantly lower freedom from significant dysfunction (ejection fraction ≤ 40%) among those with WPW and LVNC (P < .001). Further analysis showed a higher risk of developing significant dysfunction in patients with WPW and LVNC versus LVNC alone (hazard ratio 4.64 [2.79, 9.90]). Twelve patients underwent an ablation procedure with an acute success rate of 83%. Four patients with cardiac dysfunction were successfully ablated, 3 having improvement in function.

Conclusion: WPW is common among children with LVNC and is associated with cardiac dysfunction. Ablation therapy can be safely and effectively performed and may result in improvement in function.

Keywords: Arrhythmia; cardiomyopathy; pediatrics; trabeculation.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Electrocardiography / methods
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Isolated Noncompaction of the Ventricular Myocardium / diagnosis*
  • Isolated Noncompaction of the Ventricular Myocardium / epidemiology*
  • Isolated Noncompaction of the Ventricular Myocardium / physiopathology
  • Male
  • Retrospective Studies
  • Wolff-Parkinson-White Syndrome / diagnosis*
  • Wolff-Parkinson-White Syndrome / epidemiology*
  • Wolff-Parkinson-White Syndrome / physiopathology