Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades

Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades.

Osborne J, Friedman K, Runeckles K, Choueiter NF, Giglia TM, Dallaire F, Newburger JW, Low T, Mathew M, Mackie AS, Dahdah N, Yetman AT, Harahsheh AS, Raghuveer G, Norozi K, Burns JC, Jain S, Mondal T, Portman MA, Szmuszkovicz JR, Crean A, McCrindle BW; International Kawasaki Disease Registry Pediatr Cardiol. 2021 Jan 13. doi: 10.1007/s00246-020-02529-2. PMID: 33439285


Take Home Points:

This is an observational registry study.

  • In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) risk stratification was added for long-term management.
  • Generally, past practice was consistent with the latest AHA guidelines.
  • However, the important exception was that 35% of patients with persistent giant coronary artery aneurysms were not on anticoagulants.
  • Physician education could improve guideline compliance and patient outcomes.

Manoj Gupta


Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch:


Kawasaki disease (KD) is a self-limiting, systemic vasculitis of unclear etiology that mostly occurs in children. Coronary artery aneurysms form in approximately 25% of untreated patients and in approximately 4% of patients treated within 10 days of the onset of illness. The long-term consequences of KD may include myocardial infarction and death. In 2017, the American Heart Association (AHA) updated the approach to risk stratification and long-term management of KD patients (

This manuscript compared the Kawasaki disease management practice from 1999 to 2016 with the most recent AHA 2017 recommendations for thromboprophylaxis and medical therapy based on the new risk stratification. The study included the eligible patients from registry who were diagnosed with Kawasaki disease under the age of eighteen years from 1999 to 2016 and had coronary artery aneurysms (Defined as at least one coronary artery diameter with a maximum z-score ≥ 2.5 at any point in their initial work up and subsequent follow -up).

Figure 1: Risk level (RL) classification of coronary artery aneurysms per the 2017 AHA guidelines using maximum z-score, luminal dimension, and amount of regression.

Risk Level 1: No involvement

Risk Level 2: Dilation only




Prevalence of medication use by risk levels (RL) was explored across 3 eras (1999–2004, 2005–2010, and 2011–2016). Acetylsalicylic Acid (ASA): Use of ASA/aspirin ranged from 88 to 96% for RLs for which use was “indicated” (RLs 3.1, 4.1, 4.2, 5.1, 5.2, and 5.3); this represents patients with coronary artery aneurysms of any size that have not regressed to normal luminal dimensions or dilation only (z-score <2.5)


Systemic anticoagulation is only “recommended” for patients for RL 5.1 (persistent large/giant coronary artery aneurysms). Despite this, only 65% of patients in RL 5.1 were receiving anticoagulation and there was no trend toward increased usage over the different year groups.


Dual antiplatelet therapy (ASA and clopidogrel) was in use at 16% of patient visits for RL 5.2 where use was “reasonably indicated.” For visits for RLs 5.3 and 5.4, dual antiplatelet therapy was used for 11% and 9%, respectively (“not indicated”).


There was an increase in ASA use for RL 3.2 from 54% in 1999–2004 to 73% in 2011–2016 and there was increased single antiplatelet (ASA only) use from 43% in 1999–2004 to 67% in 2011–2017.

Conclusions and Implications for Clinical Practice:


The management of patients with Kawasaki disease and coronary artery aneurysms from 1999 to 2016 was generally similar to 2017 AHA Kawasaki disease guidelines.


Physician education could improve guideline compliance and patient outcomes, and knowledge translation efforts are needed to further optimize practice in anticoagulation KD patients. Additionally, more research is needed to advance future recommendations, particularly for statins, beta-blockers, and direct oral anticoagulants.