Extracardial Vasculopathy After Kawasaki Disease: A Long-Term Follow-up Study

Background-Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysm (CAA) as a major complication. Controversy exists about cardiovascular risk later in life. The aim of our study was to evaluate whether KD patients are at increased risk, as assessed by carotid intima-media thickness (cIMT). Methods and Results-We measured cIMT over 15 years by B-mode ultrasonography in KD patients during follow-up and in unaffected controls (mostly siblings). A multilevel, repeated-measures, linear mixed-effects model was used to evaluate the association between KD and cIMT. A total of 319 patients with 528 measurements were compared with 150 controls. In KD patients, the mean cIMT was increased compared with controls (0.375 mm [95% CI 0.372-0.378 mm] versus 0.363 mm [95% CI 0.358-0.368 mm]; P<0.001). Furthermore, mean cIMT of CAA-negative patients was 0.373 mm (P<0.01 compared with controls), of patients with small-medium CAA was 0.374 mm (P<0.05 compared with controls), and of patients with giant CAA was 0.381 mm (P<0.01 compared with controls). Compared with controls, CAA-negative participants started with an increased cIMT (+0.0193 +/- 0.0053 mm, P<0.001) but showed slower progression (-0.0014 +/- 0.0006 mm/year, P=0.012). Patients with giant CAA showed a trend toward increased cIMT progression (0.0013 +/- 0.0007 mm/year, P=0.058). Conclusions-We observed a positive correlation between cIMT and KD severity of coronary arteritis at the acute stage. Although initially increased, the cIMT in CAA-negative patients normalized at a later age. In contrast, patients with a history of KD complicated by giant CAA showed a trend toward persistently increased cIMT. These patients may need cardiovascular counseling and follow-up beyond the heart.