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Background Accurate risk prediction of coronary artery aneurysms ( CAAs ) in North American children with Kawasaki disease remains a clinical challenge. We sought to determine the predictive utility of baseline coronary dimensions adjusted for body surface area ( z scores) for future CAAs in Kawasaki disease and explored the extent to which addition of established Japanese risk scores to baseline coronary artery z scores improved discrimination for CAA development. Methods and Results We explored the relationships of CAA with baseline z scores; with Kobayashi, Sano, Egami, and Harada risk scores; and with the combination of baseline z scores and risk scores. We defined CAA as a maximum z score (zMax) ≥2.5 of the left anterior descending or right coronary artery at 4 to 8 weeks of illness. Of 261 patients, 77 patients (29%) had a baseline zMax ≥2.0. CAAs occurred in 15 patients (6%). CAAs were strongly associated with baseline zMax ≥2.0 versus P Conclusions In a North American center, baseline zMax ≥2.0 in children with Kawasaki disease demonstrated high predictive utility for later development of CAA . Future studies should validate the utility of our findings.

Aim: At a single pediatric tertiary care center, we assessed the performance of established Japanese risk scores (RS) to identify patients (pts) with Kawasaki Disease (KD) at higher risk for re-treatment (re-tx). We also sought to determine if the addition of baseline coronary Z-scores to RS could improve the prediction re-tx. Methods: We reviewed available clinical, lab and echocardiogram (echo) data for KD pts treated from 1/2006 to 5/2014. The maximum z-score (zMax) of the right coronary artery (RCA) or left anterior descending artery (LAD) at baseline was used. Abnormal coronary arteries at baseline echo were defined as zMax ≥2 in the RCA or LAD. Kobayashi, Sano, Egami and Harada scores were calculated, comparing low and high risk scores to re-tx. The zMax was incorporated into each RS with 1 point added (+1) if baseline zMax ≥2. In an alternative approach, zMax was added as a covariate in a logistic regression model. The discrimination of each model was assessed using the c statistic. Results: Of 339 pts, 214 (63%) were male, and median age was 3.2 y (range 0.1-14.1 y). At diagnosis, 29% (97/339) had ≤3 classical criteria for KD, and 25% (85/339) received IVIG re-tx. At baseline, the median zMax was 1.37 (-1.64-14.2) and 26% (88/339) had zMax ≥2. Classification as high risk by all four RS was significantly associated with re-tx (p Conclusions: Published RS for predicting IVIG resistance are associated with higher rates of re-tx in our patient population, but are not sufficient to discriminate patients at risk for re-tx. With the addition of baseline zMax as a covariate to the RS, prediction for re-tx was improved, although still modest. Further study in larger, multi-center studies are needed to validate these findings and to better understand the risk factors for re-tx in KD in North American children.

Background: The late natural history of coronary artery aneurysms (CAA) after IVIG treatment in the US is not well described. Methods: We evaluated all KD patients (pts) at 2 centers from 1984-2014. Entry criteria were: 1) IVIG treatment; 2) CAA, defined as LAD or RCA z-score ≥ 3 or Japanese Ministry of Health criteria; and 3) ≥1 follow-up (f/u) echo. Kaplan Meier curves evaluated time to CAA regression (z < 2.5) and Cox regression examined factors associated with persistent CAA and major adverse cardiac events (MACE= death, MI, CABG, PCI, occluded CA). Results: Of 2592 KD pts, 408 (15%) met entry criteria and were 72% male; 54% white, 21% Asian, 7% black, 18% other race. Median age at fever onset was 1.8 y [IQR: 0.7-4.4y], 74% had complete KD, and fever days before 1 st IVIG were 7d [IQR 6-10d]. IVIG retreatment occurred in 35% and adjunctive anti-inflammatory therapy in 37%, both increased over time (p Over median f/u of 2.6 y (0.01-29.0y), 313 (77%) had CAA regression at median of 1.1 mo (IQR =0.3-16.9mo). Univariate risk factors for CAA persistence were z ≥ 8 at diagnosis (HR 0.22, p Day 10 (HR 0.46, p8 at diagnosis (p Conclusion: Most CAA regress within the first year after treatment. Persistent CAA are more likely in pts with larger CAA, multi-vessel CAA and late IVIG treatment. In pts with large CAA, time to regression has gotten shorter in more recent eras, possibly related to greater use of adjunctive therapies.

Background: Echocardiography (echo) is recommended in patients (pts) with KD at baseline, 2 wks, and 6 wks after fever onset to detect coronary artery (CA) abnormalities. However, echo imaging is expensive and in infants and toddlers often requires sedation, which is burdensome and carries some risk. Objective: To assess the benefit of additional echo imaging in uncomplicated KD pts with normal CAs at baseline and 2 wks. Methods: In this retrospective two-center study, eligibility criteria were: 1) admission and treatment at center for acute KD; 2) no significant congenital heart disease; 3) available echo measurements of both the right (R) CA and left anterior descending (LAD) at ≤ Day 10 (baseline), 2 (±1) wks and 6 (±3) wks; and 4) normal CAs = RCA and LAD z scores [zMax] ≤2.0 and no aneurysms. Results: Of 2,600 pts treated for KD from 1976 - 2014, 457 had normal CAs at baseline and 2 wks and met other inclusion criteria. Of these, 57% were male; median age was 3.3 yrs [IQR 1.8 to 5.4 yrs]; 77% had complete KD; and 14% received IVIG retreatment. At 6 wks, 450 (98.5%) had normal CAs. Of the remaining 7 pts, zMax was 2.0-2.4 in 5 (1.1%); their z scores subsequently normalized at 1 yr. The zMax was 2.5-2.9 in 1 (0.2%) pt who remained on low dose aspirin until 6 mos, when ECHO normalized. The zMax was ≥3.0 in 1 pt (0.2%, 95% CI [0.0%, 1.2%]) with a dilated LAD, which was normal 3 wks later. Sensitivity analysis using zMax of Conclusion: New CA abnormalities are rarely detected at 6 wks in KD pts with normal CAs at baseline and 2 wks. The 6 wk echo may be unnecessary for uncomplicated KD pts with normal CAs at baseline and 2 wks. Figure - CA zMax shown as mean (+), median, 25th to 75th %tile.

Purpose: Kawasaki disease (KD) awareness and therapy have evolved since its description in 1967. We explored historical trends since 1984 in patients (pts) treated for KD at a single institution. Methods: Inclusion criteria were 1) first episode of KD, and 2) treated with IVIG within 3 weeks of illness onset. Exclusion criteria were 1) evaluation solely for a second opinion, and 2) presence of congenital heart disease. We reviewed age at diagnosis, sex, race, presence of complete vs. incomplete (fever + Results: Of 1739 pts, 1133 met eligibility criteria. Race included 61% White, 10% Black, 11% Asian: 6% other, and 12% unknown. At diagnosis, 16% were age Conclusions: At a single center over 3 decades, treatment of incomplete KD, as well as retreatment with IVIG and use of adjunctive therapies have become increasingly common. Pts who had incomplete diagnostic criteria received IVIG later in their illness. Future studies should assess whether these secular trends have improved outcomes in pts with KD.