Timely Diagnosis and Aggressive Care Essential for High-Risk Kawasaki Disease Cases

The latest statement, titled “Update on Diagnosis and Management of Kawasaki Disease,” encapsulates findings published since the 2017 American Heart Association Scientific Statement on Kawasaki Disease, focusing on diagnostic standards, risk assessment scores, and treatment alternatives for affected children and adults.

Key points from the statement include:

• Kawasaki Disease is a rare yet serious condition primarily affecting children under the age of five. It leads to inflammation of blood vessels throughout the body and is the foremost cause of acquired heart disease in children in developed nations.
• The precise cause of Kawasaki Disease remains unidentified, but it is highly suspected to be linked to an infectious agent, even though no specific pathogen has been confirmed. Healthcare professionals use established symptoms like prolonged fever, skin rashes, bloodshot eyes, and swelling in the hands and feet to diagnose the disease. If treatment is delayed, one out of four children may experience coronary artery dilation and/or aneurysms.
• To assess coronary abnormalities and determine risk, healthcare professionals apply coronary artery Z-scores. This metric compares the diameter of the coronary arteries in a child with Kawasaki Disease to what is typically expected for healthy children of similar age, sex, and size. Children with Z-scores over 2.5 are at a higher risk for developing coronary aneurysms. While the Z-score alone should not dictate the evaluation of coronary artery damage, the statement emphasizes the importance of using a consistent Z-score equation over time to maintain accurate risk classifications.
• Recognizing children at risk for coronary artery aneurysms early in a diverse population remains a challenge, making the timely diagnosis of Kawasaki Disease crucial for effective management. A new risk scoring system for North American children incorporates factors such as being under six months old, Asian ethnicity, elevated coronary artery Z-scores of 2 or more, and higher levels of C-reactive protein (a marker of inflammation). These developments enable clinicians to pinpoint high-risk patients who may need more aggressive initial treatment to mitigate coronary artery complications.
• The statement recommends performing echocardiograms at regular intervals, particularly for patients with Z-scores above 2.5, to monitor any progression of coronary artery concerns during hospital stays or after discharge. Techniques for echocardiography have advanced since 2017, improving the detection of coronary artery dilation or aneurysms, which can help prevent more serious and prolonged heart issues.
• Intravenous immunoglobulin (IVIG), which contains antibodies to combat infections, remains the primary treatment for Kawasaki Disease. Research indicates that adjusting dosages based on patients’ lean body mass, especially in those with obesity, can minimize complication risks.
• The statement reassesses aspirin use during the acute phase of Kawasaki Disease. Recent studies suggest that low- or medium-dose aspirin could be as effective as high-dose aspirin, and ongoing trials are testing various dosages to validate these conclusions.
• Recent research shows that incorporating additional treatments, such as corticosteroids or infliximab, can benefit children with Kawasaki Disease who are at a heightened risk of IVIG resistance. These alternative options may effectively prevent coronary artery complications and provide healthcare professionals with more tools for treatment-resistant scenarios.
• For children with Kawasaki Disease who have larger coronary aneurysms, anticoagulant therapies like aspirin (typically 81 mg daily) paired with other anticoagulants, such as warfarin or low molecular weight heparin, may prevent dangerous blood clots. Emerging evidence indicates that using direct oral anticoagulants for large coronary artery aneurysms might be more effective, safer, involve less monitoring, and have fewer side effects, though further research is needed.
• The COVID-19 pandemic led to a rise in a related condition known as Multisystem Inflammatory Syndrome in Children (MIS-C), which presented diagnostic challenges due to overlapping symptoms such as fever, rash, and heart issues. New findings have clarified distinctions between Kawasaki Disease and MIS-C by identifying additional MIS-C symptoms, like gastrointestinal issues, low platelet counts leading to bruising or bleeding, and lower white blood cell counts. Conversely, coronary artery involvement stays a defining characteristic of Kawasaki Disease, aiding in more accurate diagnoses. Additionally, machine learning algorithms are being developed to assist clinicians in differentiating between the two conditions.
• All medical centers managing Kawasaki Disease and large coronary artery aneurysms should have a multidisciplinary heart team and a structured protocol to handle significant adverse cardiac events.
• This updated scientific statement underscores the importance of a formal transition plan to ensure ongoing care for adolescents and adults who have a history of Kawasaki Disease. Patients with large aneurysms remain highly vulnerable to heart attacks and necessitate lifelong monitoring. Further research is essential to refine the timing and methods for overseeing long-term heart health in these individuals.
• Individuals with Kawasaki Disease considering pregnancy are at an elevated risk for adverse cardiac events and should receive care from obstetricians experienced with Kawasaki Disease, alongside consultations from cardiologists.

This scientific statement was developed by a volunteer writing group on behalf of the American Heart Association’s committees focused on Kawasaki Disease and related cardiovascular health councils. American Heart Association scientific statements aim to raise awareness about cardiovascular conditions and strokes and facilitate informed healthcare choices. They outline current knowledge on various topics and identify areas needing further investigation. While these statements inform guideline development, they do not provide specific treatment recommendations; official clinical practice recommendations come from the American Heart Association’s guidelines.

The authors of the writing group and their disclosures are detailed in the manuscript.

The Association’s funding primarily comes from individual contributions, but foundations and corporations, including pharmaceutical and device manufacturers, also donate to support specific programs and events. The Association adheres to strict guidelines to prevent these relationships from altering the scientific content.