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Overview of antithrombotic therapy in patients with Kawasaki disease according to the size of coronary artery abnormalities[1,2]

Overview of antithrombotic therapy in patients with Kawasaki disease according to the size of coronary artery abnormalities[1,2]
This algorithm is intended for use in conjunction with additional UpToDate content on KD and CAAs. Refer to UpToDate topics on the evaluation and management of cardiovascular sequelae of KD for additional details of our approach to treatment and the overall efficacy of these treatments.
CAA: coronary artery aneurysm; LMWH: low molecular weight heparin; KD: Kawasaki disease.
* CAA size is classified according to the echocardiographic luminal diameter, normalized for body surface area as Z-scores or standard deviation units. Echocardiography should be performed in all patients with KD as soon as the diagnosis is suspected in order to establish a baseline for longitudinal follow-up. Repeat echocardiograms are usually obtained at 1 to 2 weeks and again 4 to 6 weeks after discharge.
¶ Some children who have coronary artery dimensions within the normal range (ie, Z-score <2) have substantial reduction in coronary artery dimension over time, suggesting that the coronary artery was initially dilated. Such patients are categorized as "dilation only," despite always having coronary artery dimensions within the normal range.
Δ For details regarding long-term follow-up for patients with KD and CAAs, refer to UpToDate content on management of cardiovascular sequelae of KD.
Other antiplatelet agents (eg, clopidogrel) are reasonable alternatives for patients who are intolerant to aspirin.
§ Some experts use dual antiplatelet therapy for patients with "high-risk" CAAs that do not meet criteria for systemic anticoagulation. The evidence to support this practice is limited, and consensus is lacking as to the appropriate criteria for identification of high-risk patients. In our practice, we use dual antiplatelet therapy in patients with a maximum Z-score of approximately 7.5 to <10. Other experts may consider additional high-risk features, such as long length and distal location of aneurysms, large total number of CAAs, multiple branches affected, luminal irregularities, vessel wall abnormalities (calcification, luminal myofibroblastic proliferation), functional abnormalities (impaired vasodilation, impaired flow reserve), absence or poor quality of collateral vessels, previous revascularization performed, previous coronary artery thrombosis, previous myocardial infarction, and ventricular dysfunction.
¥ When "triple therapy" is used, the second antiplatelet agent is generally discontinued after 6 months.
References:
  1. McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation 2017; 135:e927.
  2. Manlhiot C, Millar K, Golding F, McCrindle BW. Improved classification of coronary artery abnormalities based only on coronary artery z-scores after Kawasaki disease. Pediatr Cardiol 2010; 31:242.
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