Kawasaki disease

Kawasaki disease is a leading cause of acquired heart disease in children i n the USA. An acute vasculitis of unknown etiology, it occurs predominantly in infancy and early childhood, and more rarely in teenagers. Coronary art ery aneurysms or ectasia develop in approximately 15-25% of children with t he disease. Treatment with intravenous gamma globulin, 2 g per kg, in the a cute phase reduces this risk three- to fivefold. Angiographic resolution oc curs in approximately one-half of aneurysmal arterial segments, but these s how persistent histologic and functional abnormalities. The remainder conti nue to be aneurysmal, often with development of progressive stenosis or occ lusion. The worst prognosis occurs in children with so-called 'giant aneury sms', i.e. those with a maximum diameter greater than 8 mm, because thrombo sis is promoted both by sluggish blood flow within the massively dilated va scular space and by the frequent development of stenotic lesions. Serial st ress tests with myocardial imaging are mandatory in the management of patie nts with Kawasaki disease and significant coronary artery disease to determ ine the need for coronary angiography and transcatheter interventions or co ronary bypass surgery. Continued long-term surveillance in patients with an d without detected coronary abnormalities is necessary to determine the nat ural history of Kawasaki disease.