FATAL KAWASAKI DISEASE DUE TO EARLY OCCLUSIVE CORONARY ARTERY DISEASE

Heaton PAJ a, Wilson N b

a Taranaki Base Hospital, New Plymouth, New Zealand

b Green Lane Hospital, Auckland, New Zealand

 

       We report 2 boys with Kawasaki Disease (KD) who died within 4 months from myocardial infarction due to vaso-occlusive disease. Both received intravenous gammaglobulin (IVIG) within 10 days of the start of fever. In neither were aneurysmal dilatation nor intramural thrombus terminal features.

       Case 1: 6 month Caucasian received IVIG and Aspirin on day 8 of illness. Echocardiogram normal day 8. 2nd dose IVIG day 20 for continued irritability. L coronary artery origin dilated to 3mm day 50. Sudden deterioration and death day 95. Autopsy showed extensive myocardial infarction, diffuse luminal stenosis in all coronary arteries and their branches. Only mild aneurysmal dilatation of proximal LAD and R coronary arteries present. No thrombi  seen.

       Case 2: Caucasian 4 yrs received IVIG and Aspirin on day 10 of illness. Echo showed dilated L and R coronary arteries. 2nd dose IVIG after 2 days for continued fever. Unstable angina 10 weeks from diagnosis. Echo showed dilated L main coronary artery with 8mm aneurysm on LAD, also poor left ventricular function. Died during induction of anaesthesia for cardiac catheterisation. Autopsy showed extensive stenosis and dilatation of coronary and visceral arteries, focal active arteritis and myocardial infarction. No thrombi seen.

       Discussion: Early deaths in KD usually result from mural thrombosis or rupture of aneurysmal coronary arteries. We know of only one other reported case of rapidly evolving vaso-occlusive disease (1). Our cases suggest that IVIG administered after the 7th day of illness may be inadequate to prevent this complication. Echocardiography alone will not detect vaso-occlusive disease. (1) Pediatr 1997:100; 695-9

 

 
0704