Top Things to Know: Update on Diagnosis and Management of Kawasaki Disease
Published: November 13, 2024
- Kawasaki Disease (KD) remains a clinical diagnosis with significant evidence that early diagnosis is imperative to reduce the risk of coronary artery aneurysms (CAA).
- Although the classification system has remained unchanged since the 2017 scientific statement, there is a stronger emphasis on acquiring precise Z-scores (a statistical measure comparing coronary artery size to population norms), enabling centers to consistently use the same Z-score equation over time for effective risk assessment comparisons.
- While further trials are necessary, there is an improved understanding of the dosing of anti-inflammatory therapies in patients with KD.
- Dual therapy, combining intravenous immunoglobulin (IVIG) with intensified anti-inflammatory treatment, is increasingly supported for high-risk patients with KD, including infants under six months and those with right coronary artery or left anterior descending artery Z-score ≥2.5 at diagnosis.
- Direct oral anticoagulants (DOACs) are being considered for thromboprophylaxis in patients with KD and large CAAs, pending further research on safety and efficacy.
- Centers following patients with KD with giant CAAs are encouraged to establish multidisciplinary heart teams and protocols for addressing major adverse cardiac events.
- Advancements in coronary computed tomography angiography (CTA) with reduced radiation can serve as a baseline for monitoring patients with KD with CAA and identifying coronary artery stenoses.
- Long-term follow-up and imaging are essential for patients with KD and coronary artery involvement. Depending on the coronary complexity, clinical circumstances, and available resources, many advanced imaging options exist, including low-radiation CTA, ferumoxytol-enhanced magnetic resonance angiography (MRA), invasive angiography, or stress echocardiography.
- Medical teams caring for patients with KD with CAA are advised to establish health care transition plans as these patients move into adulthood.
- Managing KD involves early diagnosis, precise Z-scores for consistent risk assessment, and dual therapy for high-risk patients, supported by advanced imaging techniques and multidisciplinary care. Long-term follow-up and transition plans into adulthood are crucial for patients with coronary artery involvement.
Citation
Jone P-N, Tremoulet A, Choueiter N, Dominguez SR, Harahsheh AS, Mitani Y, Zimmerman M, Lin M-T, Friedman KG; on behalf of the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Radiology and Intervention; and Council on Clinical Cardiology. Update ondiagnosis and management of kawasaki disease: a scientific statement from the American Heart Association. Circulation. Published online November 13, 2024. doi: 10.1161/CIR.0000000000001265