|Risk Level||Pharmacological Therapy||Follow-Up and Diagnostic Testing||Invasive Testing|
|I (no coronary artery changes at any stage of illness)||None beyond first 6–8 weeks||Cardiovascular risk assessment, counseling at 5-yr intervals||None recommended|
|II (transient coronary artery ectasia disappears within first 6–8 weeks)||None beyond first 6–8 weeks||Cardiovascular risk assessment, counseling at 3- to 5-yr intervals||None recommended|
|III (1 small-to-medium coronary artery aneurysm/major coronary artery)||Low-dose aspirin (3–5 mg/kg aspirin/day), at least until aneurysm regression documented||Annual cardiology follow-up with echocardiogram + ECG, combined with cardiovascular risk assessment, counseling; biennial stress test/evaluation of myocardial perfusion scan||Angiography, if noninvasive test suggests ischemia|
|IV (≥1 large or giant coronary artery aneurysm, or multiple or complex aneurysms in same coronary artery, without obstruction)||Long-term antiplatelet therapy and warfarin (target international normalized ratio 2.0–2.5) or low-molecular-weight heparin (target: antifactor Xa level 0.5–1.0 U/ml) should be combined in giant aneurysms||Biannual follow-up with echocardiogram + ECG; annual stress test/evaluation of myocardial perfusion scan||First angiography at 6–12 months or sooner if clinically indicated; repeated angiography if noninvasive test, clinical, or laboratory findings suggest ischemia; elective repeat angiography under some circumstances|
|V (coronary artery obstruction)||Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of beta-blockers to reduce myocardial O2consumption||Biannual follow-up with echocardiogram and ECG; annual stress test/evaluation of myocardial perfusion scan||Angiography recommended to address therapeutic options|
Adapted from Newburger et al. (6).
ECG = electrocardiogram.