BackgroundThis study aimed to assess the occurrence of coronary artery lesions (CAL) in patients with Kawasaki disease (KD) according to serum C-reactive protein (CRP) levels. MethodsThis retrospective analysis was based on the nationwide survey of KD conducted in the Republic of Korea between 2015 and 2017. We enrolled 9131 patients and defined low (<3 mg/dL) and high (≥3 mg/dL) CRP groups. Demographic data, clinical characteristics, z-scores, and scores based on the Japanese criteria for CAL were compared between the two groups. Logistic regression analysis was used to identify CAL risk factors.ResultsThe low CRP group accounted for 23% of patients. A significant difference was observed for the mean age at diagnosis (high vs. low CRP, 34.4 ± 24.9 vs. 31.7 ± 24.8 months, p<0.001) and fever duration (high vs. low CRP, 6.6 ± 2.2 vs. 6.3 ± 2.5 days, p<0.001). A non-response to intravenous immunoglobulin treatment was found in 1377 patients (20.1%) and 225 patients (11.7%) in the high and low CRP groups, respectively (p<0.001). CAL were found in 12.9% and 18.3% of the high and low CRP patients, respectively (p<0.001), based on z-scores; and in 9.9% and 12.5%, respectively (p = 0.001), based on the Japanese criteria in the acute phase. The giant coronary artery aneurysm occurrence ratio was similar between groups (p=1.0).ConclusionsCAL occurred in patients with both high and low CRP. Therefore, patients with KD should be carefully monitored regardless of their CRP levels.

A 32-year-old woman diagnosed with Turner syndrome presented to the hospital for an evaluation of cardiovascular complications. Preoperative computed tomography (CT) and echocardiography showed progression of aortic root and ascending aorta dilatation, as well as a bicuspid aortic valve. There was no evidence of aortic regurgitation. We planned valve-sparing aortic root replacement and ascending aorta replacement with a high risk of aortic rupture. Intraoperatively, we incidentally found a juxtacommissural origin of the right coronary artery (RCA). We performed aortic valve reimplantation using a graft designed with a key-shaped hole to wrap the juxtacommissural-origin RCA by modifying the Florida sleeve technique. Coronary blood flow was patent on postoperative CT angiography, and there was no evidence of aortic regurgitation on postoperative echocardiography. The patient was discharged from the hospital on postoperative day 7 without any complications.