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Introduction:Kobayashi score (2 points for aspartate aminotransferase ≥100 IU/L, sodium < 133 mmol/L, Fever days ≤4 days, and % neutrophil ≥ 80%, 1 point for C-reactive protein ≥10mg/dL, Age ≤1 year, and platelets ≤30 x 10&sup4;/mm³) is frequently used to predict immunoglobulin (IVIG) resistance and subsequent complications including coronary artery aneurysms (CAA) in children with Kawasaki disease (KD).Hypothesis:We have previously reported that inflammation aggravates heterogeneity of ventricular repolarization in children with KD. Therefore we hypothesized that the index of instability of transmural repolarization (Tpeak to Tend (Tp-e)/QT) could predict a response to IVIG therapy and subsequent CAA formation.Methods:Consecutive 102 children with KD (1.9±1.7 years old, 60 boys) who performed electrocardiographic recordings before IVIG treatment at Fujita Health University Hospital from January 2013 to January 2019 were included. Tp-e/QT was calculated by the tangent method in lead V5 and corrected using Fridericia?s formula. The relationship between corrected Tp-e/QT (Tp-ec/QTc) and Kobayashi score was evaluated. Further, we assessed whether Tp-ec/QTc in combination with Kobayashi score can effectively predict subsequent CAA formation using receiver operating characteristic (ROC) analysis.Results:1) CAA (including transient ectasia) formation was observed in 15 (15%) children. 2) Positive correlation was observed between Tp-ec/QTc and Kobayashi score (r = 0.313, p = 0.0014). 3) Tp-ec/QTc was larger in children with CAA (0.298 ± 0.036 vs. 0.252 ± 0.045, p <0.0001). ROC analysis revealed that the cut off value of 0.269 could be used to predict CAA formation. 4) When Tp-ec/QTc was added to the predictor model based on Kobayashi score (1 point for Tp-ec/QTc ≥0.269), C-statistic improved from 66% to 70% (sensitivity from 84% to 94%, specificity from 44% to 40%) as compared with the original model.Conclusions:Tp-ec/QTc correlates with the severity of KD assessed by Kobayashi score. Both Tp-ec/QTc and Kobayashi score can be useful markers of subsequent CAA formation and combination of these can effectively predict this complication.