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To evaluate whether heart rate (HR) was associated with intravenous immunoglobulin (IVIG) responsiveness or development of coronary artery lesions (CALs) in patients with Kawasaki disease.We conducted a retrospective cohort study using data from in patients with Kawasaki disease who were hospitalized in our institution from 2006 to 2016. The patients were divided into 5 groups according to the age- and temperature-adjusted HR z score (HRZage/temp) just before IVIG administration. The ORs of outcomes were estimated by using logistic regression models, with the middle group set as the reference.Of the 322 patients, a total of 98 patients (30%) were refractory to initial IVIG treatment. The patients whose HRZage/temp belonged to the lowest group were at the highest risk of being refractory to the initial IVIG treatment (OR 2.10 [95% CI 1.01-4.37]). Multivariable analyses showed the same trend, though this was not statistically significant. The patients with the highest HRZage/temp were most likely to develop CALs (OR 2.61 [95%CI 0.86-7.92]).In patients with Kawasaki disease, HRs has a different relationship with IVIG responsiveness and CALs. Low HRZage/temp might be associated with high risk of being refractory to the initial IVIG treatment, while the risk of developing CALs increased among those whose HRs were high. Further studies are necessary to investigate the mechanisms regarding HR and these outcomes in Kawasaki disease.