Outcomes, Arrhythmic Burden and Ambulatory Monitoring of Pediatric Patients With Left Ventricular Non-Compaction and Preserved Left Ventricular Function

Pediatric patients with left ventricular noncompaction (LVNC) and severe ventricular dysfunction are at risk for sudden death. The aims of this study were to (1) evaluate outcomes, (2) describe arrhythmic burden on Holter monitoring, and (3) analyze the utility of Holter monitoring and its impact on care in pediatric patients with LVNC and preserved or mild ventricular dysfunction. This was a retrospective study including patients <21 years of age with LVNC and ejection fractions ≥45%. Demographic and outcome data were analyzed. Individual and cumulative Holter data were evaluated for all patients. Arrhythmias, conduction system disease, and symptoms were analyzed for each Holter recording. The incidence of significant findings and the impact on care were determined for each study. Outcome and Holter data were compared between patients on the basis of the ejection fraction (≥55% [normal] or ≥45% to <55% [mild]). This study included 72 patients, 65 with normal function and 7 with mild dysfunction (mean age 13 years). There was a single death in the cohort, which was sudden in nature. Simple ventricular ectopy was common on Holter monitoring and more common in patients with mild dysfunction (86% vs 27%, p = 0.005). Significant Holter findings (4% vs 6%) and changes to patient care (2% vs 4%) improved with cumulative Holter monitoring. In conclusion, in contrast to patients with severe dysfunction, pediatric patients with LVNC and normal or mild dysfunction have significantly better outcomes. However, worsening LV systolic function was correlated with increasing ventricular ectopy. The role of Holter monitoring is unknown, but it may have utility in patient care if used as part of ongoing screening.