Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease (Jun 2019)

Risk Model Development and Validation for Prediction of Coronary Artery Aneurysms in Kawasaki Disease in a North American Population

  • Mary Beth F. Son,
  • Kimberlee Gauvreau,
  • Adriana H. Tremoulet,
  • Mindy Lo,
  • Annette L. Baker,
  • Sarah de Ferranti,
  • Fatma Dedeoglu,
  • Robert P. Sundel,
  • Kevin G. Friedman,
  • Jane C. Burns,
  • Jane W. Newburger

DOI
https://doi.org/10.1161/JAHA.118.011319
Journal volume & issue
Vol. 8, no. 11

Abstract

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Background Accurate prediction of coronary artery aneurysms (CAAs) in patients with Kawasaki disease remains challenging in North American cohorts. We sought to develop and validate a risk model for CAA prediction. Methods and Results A binary outcome of CAA was defined as left anterior descending or right coronary artery Z score ≥2.5 at 2 to 8 weeks after fever onset in a development cohort (n=903) and a validation cohort (n=185) of patients with Kawasaki disease. Associations of baseline clinical, laboratory, and echocardiographic variables with later CAA were assessed in the development cohort using logistic regression. Discrimination (c statistic) and calibration (Hosmer‐Lemeshow) of the final model were evaluated. A practical risk score assigning points to each variable in the final model was created based on model coefficients from the development cohort. Predictors of CAAs at 2 to 8 weeks were baseline Z score of left anterior descending or right coronary artery ≥2.0, age 40‐fold greater in the validation cohort (odds ratio, 44.0; 95% CI, 10.8–180 [P<0.001]). Conclusions Our risk model for CAA in Kawasaki disease consisting of baseline demographic, laboratory, and echocardiographic variables had excellent predictive utility and should undergo prospective testing.

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