Abstract 15148: How Reassuring is an Initial Echocardiogram With Normal Coronary Arteries for Patients Presenting With Acute Kawasaki Disease?

Abstract

Background: We sought to determine the utility of current echocardiography surveillance recommendations for coronary artery (CA) involvement for children with Kawasaki disease (KD) with normal baseline studies. Methods: The International KD Registry enrolled 1200 patients (36 sites, 7 countries) with a site diagnosis of KD from 01/2020 to 01/2023; 139 with positive/possible COVID-19 infection/exposure and 174 with no echo within 10 days of admission were excluded, leaving 887 patients for analysis of results of serial echos and associated factors. Results: An initial echo was performed within 5 days of admission for 96% and within 10 days of symptom onset for 83% of patients; the max Z score in any CA branch at initial echo was normal (Z <2) 78.9%, dilation (Z 2-<2.5) 6.6%, small aneurysm (CAA; Z 2.5-<5) 10.5%, medium CAA (Z 5-<10) 2.9% and large CAA (Z ≥10) for 1.2% of patients. Higher CA Z score/Z score category were both significantly related to greater time from symptom onset to echo (p<0.001). For those with initial normal CAs, a second echo (median of 16 days after admission) was normal for 94.2%, dilation 1.9%, small CAA 2.7%, medium CAA 0.6%, and large CAA for 0.6%. For those with 2 normal echos, a third echo (median of 44 days after admission) was normal for 97.6%, dilation 1.4%, small CAA 0.5%, and 1 patient each with medium and large CAA. Additionally, after up to 6 normal echos, 3 patients had developed small CAA, 1 medium and 1 large CAA. Overall, a total of 24 (2.8%) patients had large CAA (13 admitted >10 days after symptom onset). Of these, 10 had large CAA evident at initial echo performed at day 0-3 after admission, 9 had lesser involvement at initial echo that then progressed to large CAA, and 5 had normal initial and subsequent echos and then were noted to have large CAA at an echo performed 14, 17, 21, 23 and 53 days after admission (the patient with a large CAA first detected at 53 days had no interim echos performed since their first normal echo). Conclusions: Current recommendations for serial echo assessment are effective in detecting all patients with large CAA. For rare patients, despite a normal initial echo large CAA may nonetheless develop. Additionally, important CA involvement may be evident at presentation (often delayed presentation), precluding prevention.