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