The following are key points to remember from an American Heart Association scientific statement on the diagnosis and management of Kawasaki disease:

1. Kawasaki disease (KD), an acute self-limited febrile illness that primarily affects children <5 years old, is the leading cause of acquired heart disease in developed countries, with the potential of leading to coronary artery dilation and coronary artery aneurysms (CAAs) in 25% of untreated patients.
2. KD remains a clinical diagnosis characterized by fever, unilateral lymphadenopathy, rash, bilateral nonexudative conjunctival injection, swelling and erythema of the hands and feet, and oropharyngeal findings, including strawberry tongue and erythematous lips.
3. Advances in cardiovascular imaging have improved the ability to identify coronary artery stenosis in patients with KD, yet knowledge gaps remain regarding optimal frequency of serial imaging and the best imaging modality to identify those at risk for inducible myocardial ischemia.
4. Echocardiography remains the primary noninvasive imaging method for assessing the coronary arteries, and accurate measurement of the coronary arteries is crucial in patients with KD.
5. Intensification of primary therapy with adjunctive anti-inflammatory therapy (dual therapy) may benefit high-risk patients with KD. Patients with large CAAs require antiplatelet and anticoagulation therapy.
6. New direct oral anticoagulants (DOACs) are not as affected by vitamin K intake as warfarin and do not require the therapeutic monitoring challenges of warfarin or low molecular weight heparin (LMWH). DOACs may provide a more convenient and safer alternative than warfarin or LMWH.
7. Medical centers that follow patients with KD with giant CAA need to have a multidisciplinary heart team and a protocol in place to address major adverse cardiac events.
8. Long-term surveillance is necessary in patients with CAA, especially in those with large or giant aneurysms 1 year after KD onset. This may be performed with low-radiation computed tomography angiography, magnetic resonance imaging with ferumoxytol, or invasive angiography depending on the patient’s coronary complexity and clinical circumstances, as well as institutional resources.
9. Invasive coronary angiography provides the finest delineation of coronary architecture, and its use must be balanced against risks of an invasive procedure on the basis of patient and institutional factors. Invasive coronary angiography is used for patients with myocardial ischemia and intervention for revascularization.
10. Finally, formal health care transition programs and care teams are needed for adult patients with KD with CAA to ensure uninterrupted transition of care.

Clinical Topics: Anticoagulation Management, Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Congenital Heart Disease, CHD and Pediatrics and Imaging, Cardiovascular Care Team

Keywords: Anticoagulants, Diagnostic Imaging, Heart Defects, Congenital, Patient Care Team

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