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Extracardial Vasculopathy After Kawasaki Disease: A Long-Term Follow-up Study
Sanne M Dietz1, Carline E Tacke2, Eric de Groot3
1Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Center (AMC), Amsterdam, The Netherlands s.m.dietz@amc.uva.nl.
Insights
Kawasaki disease (KD) patients show increased carotid intima-media thickness (cIMT), a marker of cardiovascular risk. While cIMT normalizes in some, those with giant coronary artery aneurysms (CAA) may experience persistently elevated cIMT, requiring long-term cardiac monitoring.
Area of Science:
- Pediatric Cardiology
- Vascular Biology
- Rheumatology
Background:
- Kawasaki disease (KD) is a significant pediatric vasculitis.
- Coronary artery aneurysm (CAA) is a major complication of KD.
- Long-term cardiovascular risk in KD patients remains a subject of debate.
Purpose of the Study:
- To assess the long-term cardiovascular risk in KD patients.
- To evaluate if KD patients exhibit increased carotid intima-media thickness (cIMT).
- To correlate cIMT with KD severity and CAA presence.
Main Methods:
- A 15-year longitudinal study measuring cIMT using B-mode ultrasonography.
- Comparison of 319 KD patients with 150 unaffected controls.
- Analysis using a multilevel, repeated-measures, linear mixed-effects model.
Main Results:
- KD patients exhibited significantly increased mean cIMT compared to controls.
- Increased cIMT was observed across all KD subgroups, including CAA-negative patients.
- While cIMT normalized in CAA-negative patients over time, those with giant CAA showed a trend towards persistently increased cIMT progression.
Conclusions:
- KD is associated with increased cIMT, indicating heightened cardiovascular risk.
- Initially elevated cIMT in CAA-negative patients normalizes later in life.
- Patients with giant CAA may require ongoing cardiovascular surveillance due to persistently elevated cIMT.
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.
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