Abstract
We reviewed all 950 cases of KD at our center from 1971 to 2001. Of these, 190 patients developed coronary artery abnormalities (CAA) diagnosed by serial echocardiograms. Among patients treated with either 400mg/kg of IVIG times 4 doses or a single dose of 2 grams/kg, the overall CAA rate was reduced from 19% in untreated patients to 6% (P< 0.001). For patients under 1 year of age, the CAA rate decreased from 25% of untreated patients to 9% (P<0.002). There was no significant difference in CAA rate between the two IVIG regimens. Three of the first 88 patients encountered during 1971 to 1978 died within 1 month of onset (8, 21, and 23 days). Three other children who died from sudden cardiac death had autopsy findings consistent with KD. None of these patients had been diagnosed before death, although one had compatible symptoms for 14 days while the other had an illness compatible with KD 17 years prior, as documented by another hospital's record. The early death at 8 days had acute inflammatory pancarditis, severe coronary intimal and adventitial inflammation with inflammatory infiltrates separating myocardial fibers but no coronary thrombosis. 4 deaths had thrombosis in aneurysmaly dilated coronaries. The sudden death 17 years after presumed KD had extensively remodeled coronaries and multiple braided lumina with remote and acute thrombosis.Six patients have required advanced cardiac interventions. One child with giant aneurysm had massive thrombosis 2 months after onset and was successful treated with thrombolytics. Two children developed severe peripheral gangrene and were treated with heparin, corticosteroid pulse therapy, and Prostaglandin-E infusion. Two other children with giant aneurysms which progressed to stenosis 12 and 15 years later had angioplasty (1) and stent (1). One child, who had asymptomatic myocardial infarction 1 year after KD, underwent successful coronary artery bypass.
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Chen, S., Knowles, K., Melish, M. et al. Thirty Year Perspective of Cardiac Sequelae of Kawasaki Disease (KD) in Hawaii. Pediatr Res 53, 165 (2003). https://doi.org/10.1203/00006450-200301000-00071
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DOI: https://doi.org/10.1203/00006450-200301000-00071