Table 1

 Ion channelopathies causing arrhythmias in children

Ion channelSyndromeGene defectArrhythmia
Na+BrugadaSCN5aVentricular fibrillation
Sudden death
Na+Long QT 3SCN5aTorsade de Pointes
Sudden death
K+Short QT syndromeKCNH2Atrial arrhythmias
Long QT 1KCNE1Torsades de Pointes
Long QT 2HERG
Ca++Catecholaminergic ventricular tachycardia (Coumel’s)hRyR2 CASQZBidirectional ventricular tachycardia