Abstract

age 15.1 3.7) and 2 patients died suddenly (mean age 17.5 1.9), corresponding to an event-free survival rate of 98 % at 7.2 years. All of these patients had abnormal ECG findings but, in univariate analyses, only the width of QRS was associated with arrhythmic events (110.3 8.2 ms in the event group vs. 98.1 1.7ms in the no-event group, p1⁄40.03). 71.4 % (n1⁄45) were diagnosed in their first year of life. CONCLUSION: During a follow up nearly 16 years, more than 85% of pediatric patients who suffered from MD1 had ECG abnormalities, which increased over time. Rhythmic events in pediatric population are rare but exist, and occured in patients with conduction abnormalities on basal ECG.

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