Pei-Ni Jone, Jane W Newburger, Jane C Burns, Matthew I Tomey, André Jakob, Yasutsugu Shiono, Satsuki Fukushima, Yoshihide Mitani, Ming-Tai Lin, Alain Fraisse, Hirosyohi Yokoi, Tosihiro Tamura, Nadine Choueiter, Jiken Bhatt, Luis G Quinonez, Etsuko Tsuda, Nagib Dahdah, John B Gordon, Kenji Suda
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.