Fatal obliterative coronary vasculitis in Kawasaki disease☆,☆☆,★

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Abstract

We report a unique case of Kawasaki disease with late sudden death from obliteration of the lumen of the full length of the left anterior descending coronary artery. Sequential echocardiograms showed early uniform coronary dilatation that resolved before sudden death. The implications of obliterative “healing” of coronary ectasia are unknown. (J Pediatr 1998;133:259-61)

Section snippets

Case Report

A 3 ½-year-old black child with a history of asthma had signs of lower respiratory infection and reactive airway disease. He received antibiotics, inhaled and systemic steroids, and ibuprofen. Ten days later his respiratory symptoms improved, and during the next 3 days he had fever to 101°F, a rash, scleral injection, peeling of the skin in the groin area, oral mucous membrane changes, edema of his hands and feet, and bilateral inguinal adenopathy. After 4 days of these symptoms, Kawasaki

Discussion

Angiographic or echocardiographic resolution of mild coronary artery dilatation or smaller aneurysms after Kawasaki disease is common.5 Pathologic studies suggest that intimal proliferation results in remodeling of the enlarged coronary lumen.6, 7 However, recent literature suggests that late coronary events might occur in such patients. Kurisu et al8 demonstrated that coronary arteries that were “normal” on angiographic evaluation had late vasomotor dysfunction, presumably from endothelial

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    However, there were cases of restenosis or formation of new aneurysms at the site of intervention, often requiring repeat intervention or surgical revascularization (Fig. 7). This is a rare complication of KD with only a few reported cases.28–30 Typically, the patient is known to develop diffuse mild-to-moderate coronary artery dilation.

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    Healing is by fibrointimal proliferation, often accompanied by calcification, and vascular reactivity does not return to normal despite grossly normal appearance [66]. Children should thus be followed indefinitely after KD, a point highlighted by a report of the sudden death of a 3.5-year-old child 3 months after dilated coronary arteries had regained a normal echocardiographic appearance [67]. Autopsy revealed obliteration of the lumen of the left anterior descending coronary artery by fibrosis, with evidence of ongoing active inflammation in the epicardial arteries.

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From the Department of Pathology, East Carolina University School of Medicine, Pediatric Department, Brody Medical Sciences Building, Greenville, North Carolina.

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Reprint requests: Michael Ellis McConnell, MD, Associate Professor of Pediatrics, Division of Pediatric Cardiology, East Carolina University School of Medicine, 600 Moye Blvd, Greenville, NC 27858-4354.

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0022-3476/98/$5.00 + 0  9/22/89012

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