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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 <6 months, Asian race, and C-reactive protein ≥13 mg/ dL (c=0.82 in the development cohort, c=0.93 in the validation cohort). The CAA risk score assigned 2 points for baseline Z score of left anterior descending or right coronary artery ≥2.0 and 1 point for each of the other variables, with creation of low- (0-1), moderate- (2), and high- (3-5) risk groups. The odds of CAA s were 16-fold greater in the high- versus the low-risk groups in the development cohort (odds ratio, 16.4; 95% CI , 9.71-27.7 [ P<0.001]), and >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.

Background: We hypothesize that a measure of the immediate postoperative severity of illness after the stage I Norwood operation reflects technical performance or the adequacy of anatomic repair and can serve as a predictor of hospital mortality, reinterventions, and clinical outcomes., Methods: One hundred thirty-five patients undergoing stage I were retrospectively studied (2004 to 2007). The severity of illness on postoperative day 1 (POD1) was measured using the Pediatric Risk of Mortality III (PRISM) scoring system. Technical performance scores (optimal, adequate, inadequate) were calculated before hospital discharge. Hospital mortality, postoperative reinterventions, and complications were recorded. Postoperative reintervention was defined as need for cardiac catheterization laboratory or operating room based procedure that included balloon dilation or repair of arch obstruction, shunt revision, reoperations for bleeding, and extracorporeal membrane oxygenation support., Results: Hospital mortality was 14.1% (n=19). The rate of complications and reinterventions was, respectively, 28.1% (n=38) and 26.7% (n=36). The POD1 PRISM score was associated with technical performance (p=0.003). Higher POD1 PRISM scores were associated with mortality (p<0.001), complications (p<0.001), and reinterventions (p=0.001). The POD1 PRISM score had high discrimination for mortality, complications, reinterventions, and inadequate technical performance (areas under the receiver operating characteristic curve were 0.835, 0.776, 0.773, and 0.710, respectively; p≤0.001 for all)., Conclusions: The severity of illness as measured by PRISM score on POD1 after the stage I Norwood operation has strong association and discrimination with hospital mortality, postoperative reinterventions, inadequate technical performance, and major postoperative complications. It may be used as an early surrogate of technical performance to initiate a search for and correction of technical deficiencies., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)