Atypical Kawasaki syndrome: how many symptoms have to be present?

Abstract

A 20 year old woman with acute myocardial infarction exhibited a huge aneurysm of the left main coronary artery that was occluded by a large intraluminal thrombus. After exclusion of other vascular or systemic diseases, atypical Kawasaki syndrome was diagnosed. Other major symptoms usually required for this diagnosis were absent. As patients with Kawasaki syndrome in childhood are surviving longer, acute coronary symptoms may occur in young adults, and coronary aneurysms might be the only symptom of atypical Kawasaki syndrome.

Figure 1

Left anterior oblique view with cranial angulation of the left coronary artery. On 29 April 1992 the left main was occluded initially (A). After thrombolysis (20 mg intracoronary rt-PA) and PTCA the vessel could be reopened, thrombotic material can still be seen in the proximal aneurysm of the left main (B, right anterior oblique).

Figure 2

Right anterior oblique view with cranial angulation of the left coronary artery. Control angiography on 2 January 1995 showed a giant aneurysm of the left main with a circumscript dissection. Within three years stenosis of ⩽75% developed.