TABLE 5.

Risk Stratification

Risk LevelPharmacological TherapyPhysical ActivityFollow-Up and Diagnostic TestingInvasive Testing
I (no coronary artery changes at any stage of illness)None beyond 1st 6–8 weeksNo restrictions beyond 1st 6–8 weeksCardiovascular risk assessment, counseling at 5-y intervalsNone recommended
II (transient coronary artery ectasia disappears within 1st 6–8 weeks)None beyond 1st 6–8 weeksNo restrictions beyond 1st 6–8 weeksCardiovascular risk assessment, counseling at 3- to 5-y intervalsNone recommended
III (1 small-medium coronary artery aneurysm/major coronary artery)Low-dose aspirin (3–5 mg/kg aspirin per day), at least until aneurysm regression documentedFor patients <11 y old, no restriction beyond 1st 6–8 weeks; patients 11–20 y old, physical activity guided by biennial stress test, evaluation of myocardial perfusion scan; contact or high-impact sports discouraged for patients taking antiplatelet agentsAnnual cardiology follow-up with echocardiogram + ECG, combined with cardiovascular risk assessment, counseling; biennial stress test/evaluation of myocardial perfusion scanAngiography, if noninvasive test suggests ischemia
IV (≥1 large or giant coronary artery aneurysm, ormultiple or complex aneurysms in same coronary artery, without obstruction)Long-term antiplatelet therapy and warfarin (target INR 2.0–2.5) or low-molecular-weight heparin (target: antifactor Xa level 0.5–1.0 U/mL) should be combined in giant aneurysmsContact 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 outcomeBiannual follow-up with echocardiogram + ECG; annual stress test/evaluation of myocardial perfusion scan1st angiography at 6–12 mo or sooner if clinically indicated; repeated angiography if noninvasive test, clinical, or laboratory findings suggest ischemia; elective repeat angiography under some circumstances (see text)
V (coronary artery obstruction) Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of β-blockers to reduce myocardial O2 consumption Contact or high-impact sports should be avoided because of risk of bleeding; other physical activity recommendations guided by stress test/myocardial perfusion scan outcomeBiannual follow-up with echocardiogram and ECG; annual stress test/evaluation of myocardial perfusion scanAngiography recommended to address therapeutic options