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Update on the Diagnosis and Management of Kawasaki Disease
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- Kawasaki disease predominantly affects children under 5 years of age, with coronary artery complications developing in 25% of untreated cases. This scientific statement discusses new criteria for identifying high-risk patients, improvements in imaging for coronary artery stenosis, and management strategies for complications, including anticoagulation, and myocardial infarction.
- This scientific update highlights advancements in diagnosis, cardiac imaging, and anti-inflammatory therapies while emphasizing existing knowledge gaps and areas for future research.
abstract
This abstract is available on the publisher's site.
Access this abstract nowKawasaki 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 in 25% of untreated patients. This update summarizes relevant clinical data published since the 2017 American Heart Association scientific statement on KD related to diagnosis, cardiac imaging in acute KD treatment, and long-term management. Criteria defining North American patients at high risk for developing coronary artery aneurysms who may benefit from more intensive initial treatment have been published. 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. Recent data have advanced the understanding of safety and dosing for several anti-inflammatory therapies in KD. New anticoagulation medication, myocardial infarction management, transition of health care for patients with KD, and future directions in research are discussed.
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Update on Diagnosis and Management of Kawasaki Disease: A Scientific Statement From the American Heart Association
Circulation 2024 Nov 13;[EPub Ahead of Print], PN Jone, A Tremoulet, N Choueiter, SR Dominguez, AS Harahsheh, Y Mitani, M Zimmerman, MT Lin, KG FriedmanFrom MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
Our understanding of Kawasaki disease (KD) has advanced considerably since its initial description approximately 60 years ago. KD is the leading cause of acquired heart disease among children in developed countries, leading to coronary artery aneurysms (CAAs) or dilation in approximately 25% of untreated patients. Since the 2017 American Heart Association (AHA) scientific statement,1 the 2024 statement provides updates on interim clinical data, focusing on diagnosis, imaging, treatment, managing myocardial infarction (MI), and long-term outcomes.
Although the diagnostic criteria for KD remain unchanged, the authors emphasize the importance of early diagnosis and identifying patients at high risk for CAAs. Patients younger than 6 months or those with baseline coronary artery z-scores of 2.5 or greater are at particularly increased risk and should be considered for intensified therapy.
While echocardiography remains the primary imaging modality for evaluating KD, limitations exist in z-score calculations due to the availability of multiple systems. Risk stratification relies on accurate z-score measurements, necessitating consistency in the z-scores used for comparisons over time. Implementing a universal z-score system could address these challenges.
The latest American Heart Association statement introduces an alternative anticoagulation option: direct oral anticoagulants. Compared with warfarin and low–molecular-weight heparin, direct oral anticoagulants require less frequent monitoring, offer oral formulations, and have fewer food and drug interactions. Notably, the indications for anticoagulation have shifted. Previously, anticoagulation was recommended for patients with giant CAAs, transitioning to antiplatelet therapy if the giant CAAs regressed to a medium or smaller size. The 2024 statement highlights the persistent high risk for giant CAAs and recommends continuing anticoagulation even if the luminal diameter regresses to medium — a significant distinction in managing patients with KD.
Although the risk of MI is highest in the first 2 to 3 months following diagnosis, patients with giant CAAs face significant long-term risks. Multidisciplinary teams should be proactively established to address emergencies. The statement includes algorithms for managing MI, recognizing that initial symptoms in infants and children differ from those seen in adults with atherosclerosis. Given the rarity of MI in pediatric populations and the nonspecific nature of symptoms, clinicians must remain vigilant for MI in all patients with a history of KD and giant CAAs.
Even if the CAA luminal diameter normalizes over time, this process often occurs through luminal myofibroblastic proliferation or layering thrombus, maintaining an ongoing risk for ischemia. The current statement outlines long-term surveillance options, emphasizing the value of CT angiography, typically performed 1 year after diagnosis in patients with CAAs. The selection of additional imaging modalities for evaluating inducible ischemia should be based on each center’s expertise in performing and interpreting these studies. Furthermore, with the increasing prevalence of adults with a history of KD, the authors stress the need for a seamless transition to adult cardiology to ensure uninterrupted medical care.
Much remains to be discovered about KD through effective collaboration, and we look forward to future advancements in this field.
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