Appropriate management of Kawasaki disease patients with coronary artery aneurysms: an interim report on a multicentric prospective registry study three years after commencement

Abstract The remote prognosis of patients with Kawasaki disease (KD) complicated by coronary artery aneurysms (CAA) is still unclear. The present, multicentric registry study aimed to study the factors associated with coronary events (CE) and determine an appropriate management method for patients with KD complicated with medium-sized or large CAA from 2015 to the present. The primary endpoint was the time-dependent incidence of CE. Associated factors were also examined. In total, 179 patients from 53 centers were enrolled and followed up for a median of 501 days. The median age at KD onset was 2.2 years, 137 patients were male (77%), 47 had incomplete KD (26%), and 36 had large CAA (20%). CE occurred in 13 patients (7%; 95% confidence interval: 4-12%); eight (62%) experienced CE within one year, and all the patients experienced a CE within two years. All but one patient received antiplatelet drugs and warfarin. Patients with a large CAA had significantly more CAA (2.8 vs. 1.7, p < 0.001), more cases of warfarin use (86% vs. 43%, p < 0.001), and were more likely to have CE (28% vs. 2%, p < 0.001) than those with a medium-sized CAA. On Cox proportional hazards analysis, the factors significantly associated with CE were large CAA (hazard ratio (HR): 17.0), three or more CAA (HR: 23.3), and beaded CAA (HR: 15.9).Conclusion: Patients with a large CAA were more likely to have a CE within two years, suggesting that antithrombotic therapy with warfarin may have limited efficacy. Patients with multiple or beaded CAA should be managed carefully.