Kawasaki disease (KD) is an acute systemic vasculitis syndrome of unknown etiology which mainly affects small and medium-sized arteries, particularly the coronary arteries. The long-term problems from KD are concerned almost entirely with the coronary arterial lesions that produce aneurysms and thrombotic occlusion and lead to premature atherosclerosis and ischemic heart disease. The disease was first recognized in Japan, but now it is found all over the world and is the leading cause of acquired heart disease in children in North America and Japan. It has been 25 years since KD was first reported and a large number of patients have become part of the pool of adults with ischemic heart disease. The long-term consequences and natural history of the cardiovascular involvements from KD remain uncertain. Since 1973 we have made a diagnosis of acute KD in 1664 patients and this cohort has been followed by coronary andiographic studies. From our study of these patients we will review the long-term issues regarding the evaluation and management of coronary artery disease caused by KD. (C) 1997 Elsevier Science Ireland Ltd.