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.