Early Currents
Developmental Electrophysiology and Arrhythmia in Pediatric Congenital Heart Disease
Lixia Dai (Erasmus MC)
Weilin Liu (Erasmus MC)
Vehpi Yildirim (Erasmus MC)
Mathijs S. van Schie (Erasmus MC)
Yannick J.H.J. Taverne (Erasmus MC)
N.M.S. de Groot (Erasmus MC, TU Delft - Signal Processing Systems)
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Abstract
Arrhythmias significantly contribute to morbidity and mortality in patients with congenital heart disease (CHD). While postoperative factors predisposing to arrhythmias are well-established, early electrophysiological alterations in pediatric CHD remain poorly understood. This review summarizes current knowledge on postnatal cardiac maturation, conduction-system development, and electrophysiological abnormalities in pediatric patients with and without CHD. Importantly, arrhythmia prevalence, mechanisms, and clinical relevance are systematically discussed across three pediatric groups, including healthy children and patients with unrepaired and repaired CHD. Understanding developmental arrhythmogenic mechanisms may facilitate early risk stratification, guide clinical management decisions, and improve long-term outcomes for pediatric patients with CHD. This review discusses the complex interplay between cardiac maturation, congenital defects, and arrhythmogenesis. It also outlines future directions that include noninvasive monitoring, selective intraoperative mapping, animal model studies, and standardized data collection to improve early risk stratification and long-term outcomes in children with CHD.