This Circulation review addresses the growing population of Kawasaki disease survivors at risk for acute coronary syndrome, highlighting how KD-specific coronary pathology and thrombosis risk diverge from traditional atherosclerotic disease. It synthesizes current evidence on advanced coronary imaging, tailored revascularization strategies, and optimized antithrombotic regimens to guide management. The authors emphasize the necessity of coordinated care between pediatric and adult cardiologists to bridge existing guideline gaps. For clinicians, this review provides a practical framework for the acute and long-term management of KD-associated ACS.
Acute coronary syndrome (ACS) remains underrecognized in patients with Kawasaki disease (KD). The population at risk for KD-associated ACS is increasing, yet current ACS guidelines are largely derived from atherosclerotic disease and do not adequately address the distinctive mechanisms, anatomy, and thrombosis risk associated with KD. Because the pathophysiology and management of KD-associated ACS differ in fundamental ways from those of atherosclerotic coronary artery disease, its management requires a coordinated team response, with collaboration between pediatric and adult cardiologists. Recent advances in coronary imaging, interventional techniques, and antithrombotic therapies provide an opportunity to establish a more disease-specific approach. This review synthesizes current evidence on the epidemiology and pathophysiology of coronary artery aneurysms, interventional coronary imaging, acute management, revascularization strategies, antithrombotic therapy, and post-ACS surveillance in patients with KD to guide clinical decision-making and treatment for patients with KD presenting with ACS.
