Abstract

Objective: Risk and natural progression of coronary artery stenosis in Kawasaki Disease is not well-defined and is a potential cause of long term morbidity, despite treatment with IVIG. We present a novel study at a US tertiary pediatric care center identifying risk factors for stenosis. Methods: We reviewed charts of all children that underwent cardiac catheterization for coronary artery abnormalities from 1998 to January 2014 at a tertiary pediatric care center. All demographic and diagnostic data was recorded including time intervals to echocardiographic changes and catheterization confirmed cases of stenosis. Multivariate survival analysis was used to identify risk factors with stenosis formation as the main outcome measure. Results: Fifty-two children met inclusion criteria and 18 (34.6%) developed stenosis. The highest risk group overall were children under the age of 6 months (HR 3.66, p=0.005) and those with giant coronary aneurysms (GCA). In a subset of only cases of GCA (33), children under the age of six months were at highest risk (HR 2.62, p=0.04). IVIG administration, gender, and ethnicity were statistically insignificant. The majority of individuals with GCA went on to develop stenosis (19/33). The presence of GCA was 100% sensitive for cases of stenosis. Conclusions: This is a novel study in an American population and demonstrates a relatively high incidence of stenosis in children with KD and coronary vascular abnormalities. Overall, a majority of GCA cases progressed into stenosis, with children under the age of 6 months being at highest risk.

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