Super Giant Coronary Aneurysm in Kawasaki Disease

Giant coronary artery aneurysms caused by Kawasaki disease are rare; however, they are one of the most serious complications and can be lethal. We report a 3.5-month-old boy referred to us because of high fever for fifteen days, generalized maculopapular rash, irritability and cough. Transthoracic echocardiography showed dilatation of right coronary (RCA) and left main coronary (LCA) arteries. Serial echocardiography revealed rapidly progressive dilatation of coronary artery aneurysms of RCA and LCA. We performed invasive cardiac catheterization with selective coronary angiography when the boy was 16 months old. Selective right and left coronary arteriography showed a super giant fusiform aneurysm of RCA and a diffuse giant aneurysm of the proximal LCA. Regression of coronary artery aneurysms was not observed during 6 years of follow up. Pediatricians should be alert for possibility of incomplete Kawasaki disease in young infants with atypical presentation. They are at higher risk of coronary aneurysm formation. The diagnosis often was late with higher complication rate of coronary aneurysm. Echocardiography is an important tool for diagnosis of incomplete Kawasaki disease. Selective coronary angiography is the gold standard for diagnosis, and estimation of shape and size of aneurysms.