PM1009: An International Survey on the Medical Management of Children with Giant Coronary Artery Aneurysm After Kawasaki Disease

Background: Giant coronary artery aneurysms (CAA) are a rare complication of Kawasaki disease (KD). Despite available guidelines, most recommendations are based on experts’ consensus due to limited evidence-based data, and vary between scientific societies.

Methods: A standardized, anonymous survey was sent to physicians caring for patients with KD through cardiology associations’ distribution lists and personal contacts. Participants answered questions with regards to demographics, clinical experience and how they monitor and manage patients with giant CAA. The survey included clinical vignettes of patients in different age groups. Chi-squared test was used to assess variations between groups, p-value < 0.05 was deemed significant. We also invited respondents to comment on their practice with optional open-ended questions.

Results: The survey was completed by 134 physicians, with representation from North-America (35%), South-America (37%), Asia (15%), Europe (8%) and Middle-East-North-Africa region (5%). Most physicians (53%) reported following their patients with giant CAA every 6 months beyond the first year of diagnosis, with 35% reporting follow-up every 3 months and 11% every 9-12 months. Physicians from North-America were more likely to report follow-up every 6 months versus other regions (80% vs 39%, p< 0.001). Most respondents (80%) reported using aspirin and anticoagulant (warfarin, low molecular weight heparin or direct oral anticoagulant) regardless of the patient age (2 years-old vs. 12 years-old) or presence of coronary artery stenosis on angiography (normal vs. 75% stenosis). Physicians were significantly more likely to recommend beta-blockers for patients with coronary stenosis than without (52% vs 18%, p< 0.001); however patient age did not alter opinion on use of beta-blockers. On the other hand, the use of statins varied both with patient age (43% in 12 years-old patient vs 12% in 2 years-old patient, p< 0.001) and presence of stenosis (35% in patients with stenosis vs 19% in patients without, p< 0.001). The use of statins also varied across regions – in a 12 years-old patient with stenosis, 46% of respondents from North-America and 88% of respondents from other regions recommended statins (p=0.021). Additionally, 4 respondents commented that statin use was debated in their institutions, as evidence was not well established.

Conclusion: We identified significant practice variation based on location of practice, with significant differences in North-America versus the rest of the world. While there was overall agreement on using a combination of aspirin and anticoagulant, there was more variation with the use of beta-blockers and statins, likely due to the absence of supportive evidence.