Abstract 2957: New AHA Recommendations Improve Recognition and Treatment of Kawasaki Disease: A Multicenter Retrospective Review of Patients With Coronary Aneurysms.
Background: Prompt IVIG treatment is important for prevention of coronary artery aneurysms (CAA), but incomplete criteria can delay diagnosis. AHA 2004 recommendations included an epidemiologic case definition and an algorithm for diagnosing and treating suspected incomplete Kawasaki disease (KD). We explored the performance of these recommendations in a large series of U.S. KD patients (pts) with CAA.
Methods: We retrospectively reviewed records of KD pts with CAA at 3 centers from 1980–2007. CAA were defined by Z score >3 or Japanese Ministry of Health criteria. Our primary outcome was the % of pts presenting ≤ Day 10 who would have received IVIG treatment by application of AHA recommendations.
Results: Of 262 pts, 191 (73%) presented ≤ Day 10, 17% were ≤ 6 months. A subset of 158 pts had data adequate to apply the 2004 AHA guidelines. Of these, 123 pts (78%) met the case definition and would have received IVIG; 28 pts (18%) had suspected incomplete KD and were eligible for algorithm application, of whom 22 pts would have received IVIG at presentation and 6 pts would not have been treated initially but referred for serial evaluation. Overall, application of the AHA algorithm would have referred 145 pts (92%) for immediate IVIG treatment and 6 pts (4%) for serial evaluation; 7 pts (4%) did not meet the case definition or qualify for the algorithm at presentation because of fever <5 days (n=6) or <2 clinical criteria in pt >6 mo of age (n = 1). Pts ≤ 6 mo, compared to older pts, were more likely to have incomplete KD, although this did not reach statistical significance (26% vs. 16%, p=0.27). Among the 27 pts ≤ 6 mo old, 26 pts (96%) would have been referred for IVIG treatment and 1 (4%) had fever <4 days so was ineligible for the algorithm at presentation.
Conclusion: In this retrospective review of KD pts with CAA first evaluated ≤ Day 10, application of the AHA guidelines substantially expanded the number of pts (from 78% to 92%) who were appropriately diagnosed and treated with IVIG at presentation. However, 8% of KD pts with CAA would not have been treated with IVIG at initial presentation, suggesting the importance of serial evaluation. Prospective multi-center assessment of AHA recommendations should guide further refinement of the algorithm for the diagnosis and treatment of incomplete KD.