LAUSR

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 were reviewed for presence of preexcitation. 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<0.001). Further analysis showed a higher risk of developing significant dysfunction in patients with WPW and LVNC vs. LVNC alone (HR 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.