151 - A GLOBAL SURVEY PERSPECTIVE ON THE SURVEILLANCE OF CHILDREN WITH GIANT CORONARY ARTERY ANEURYSM FOLLOWING KAWASAKI DISEASE

Background: Giant coronary artery aneurysms (GiAn) are rare but potentially fatal complications of KD, as they are particularly at risk for thrombosis, stenosis, leading eventually to myocardial infarction and sudden cardiac death. Guidelines differ between institutions and countries with a limited evidence-based recommendations on management and treatment. We sought to identify variations and discrepancies pertaining to the long-term follow-up of GiAn to highlight areas requiring further research.

Methods: An anonymous online survey was validated and distributed globally to physicians attending to patients who have or have had KD. Responses with regard to surveillance and imaging practices, pharmacological management and surgical or percutaneous interventions were collected and analyzed. Additionally, physicians’ characteristics (region, type and length of practice) were used for correlation and comparison.

Results: Responses were collected from 134 physicians from 5 regions. The majority (75%) practice in University Hospital setting, 90% were pediatric cardiologists (1/3^rd interventionalists) (Figure 1). The average years in practice was 15±9.6 (highest in North America, Asia and the Middle-East/North-Africa ~18±10, compared to South America and Europe ~12±9; p=0.03).  The largest number of patients with GiAn per respondent was from Asia (90% follow >10 GiAn patients) versus other regions (55%) (p < 0.001).  Cardiac catheterization was the preferred (52%) 1^st choice for coronary surveillance followed by Coronary CT-angiography (29%), PET/SPECT, stress echo, coronary MRA, and stress MRI (19-12%). North American practitioners were more likely to use Aspirin in association with anticoagulants compared to other regions (Figure-3). Finally, beta-blockers and statins were more likely to be used in teenagers as opposed to young children. Use of statins remains rare in young children (21%-8%, depending on stenosis status). Beta-blockers were more readily used at either age (65%-70%) in the case of stenosis, but less likely (19%-23%) in the absence of stenosis.
Conclusion: This international survey of KD experts, identified several areas of practice variations in the long-term management of GiAn after KD, including discrepancies between practice and available guidelines, as well as differences between geographical regions of practice. Whereas the follow-up interval and the use of anticoagulants depend on regional recommendations, other preferences do not. Despite recent trends towards non-invasive coronary assessment, catheterization remains the first preferred imaging for instance.  Variations in the use of ancillary medications reflect the absence of evidence-based data. Improved international collaborative efforts are needed to provide more robust evidence in the management of KD patients with GiAn.