Risk stratification and follow-up recommendations for children with Kawasaki disease6
| Risk level | Pharmacological therapy | Physical activity | Follow-up and diagnostic testing | Invasive testing |
|---|---|---|---|---|
| Level I (no coronary artery changes at any stage of illness) | None beyond first 6–8 weeks | No restrictions beyond first 6–8 weeks | Cardiovascular risk assessment, counselling at 5-year intervals | None recommended |
| Level II (transient coronary artery ectasia that disappears within 6–8 weeks) | None beyond first 6–8 weeks | No restrictions beyond first 6–8 weeks | Cardiovascular risk assessment, counselling at 3-year to 5–year intervals | None recommended |
| Level III (one small-medium coronary artery aneurysm/major coronary artery) | Low-dose aspirin (3–5 mg/kg aspirin per day), at least until aneurysm regression documented | For patients <11y old, no restriction beyond 1st 6–8 weeks; patients 11– 20 years old, physical activity guided by biennial stress test, myocardial perfusion scan; contact or high-impact sports discouraged for patients taking antiplatelet agents | Annual cardiology follow-up with echocardiogram +ECG, combined with cardiovascular risk assessment, counselling; biennial stress test/evaluation of myocardial perfusion scan; consider CAA imaging using CT or MR angiography | Angiography, if non-invasive test suggests ischaemia |
| Level IV (>1 large or giant coronary artery aneurysm, or multiple or complex aneurysms in same coronary artery, without obstruction) | Long-term antiplatelet therapy combined with warfarin (target INR 2.0–2.5) or low molecular-weight heparin (target: antifactor Xa level 0.5–1.0 U/mL) should be considered in all patients with giant aneurysms | Contact or high-impact sports should be avoided because of risk of bleeding; other physical activity recommendations guided by stress test/evaluation of myocardial perfusion scan outcome | Biannual follow-up with echocardiogram +ECG; annual stress test/evaluation of myocardial perfusion scan 1st angiography at 6–12 mo or sooner if clinically indicated; repeated angiography if non-invasive test, clinical, or laboratory findings suggest ischemia; elective repeat angiography under some circumstances; consider CAA imaging using CT or MR angiography | 1st angiography at 6–12 months or sooner if clinically indicated; repeated angiography if non-invasive test, clinical, or laboratory findings suggest ischaemia; elective repeat angiography under some circumstances |
| Level V (coronary artery obstruction) | Long-term low-dose aspirin; warfarin or low molecular-weight heparin if giant aneurysm persists; consider TPA to dissolve clot; consider use of β-blockers to reduce myocardial O2 consumption; consider statins and/or ACE inhibitors | Contact or high-impact sports should be avoided because of risk of bleeding; other physical activity recommendations guided by stress test/myocardial perfusion scan outcome | Biannual follow-up with echocardiogram and ECG; annual stress test/evaluation of myocardial perfusion scan | Angiography recommended to address therapeutic options; consider CAA imaging using CT or MR angiography intermittently to monitor |
CAA, coronary artery aneurysms;; TPA, tissue plasminogen activator.