Case Presentation:

A 44–year–old non–smoking, physically active male presented to our hospital with acute onset substernal chest pressure of one hour duration with associated right arm numbness and diaphoresis. His past medical history is relevant for hypertension for which he was on irbesartan daily and proteinuria of indeterminate etiology. Physical examination was normal. EKG on admission showed normal sinus rhythm. Cardiac enzyme biomarkers showed an up–trending pattern. Rest of laboratory work up was normal. Patient was diagnosed with NSTEMI and underwent left heart catheterization (LHC). The LHC showed evidence of diffuse triple coronary artery ectasia. There was no evidence of focal stenosis or thrombosis. ECHO was grossly normal with EF ~59% and no evidence of valvular disease. The patient was initiated on aspirin, clopidogrel, metoprolol and warfarin with enoxaparin bridging. Rheumatologic work up, including cardiac MRI, was normal. Homocystiene levels were also normal. Patient had no family history nor did he exhibit any physical stigmata of connective tissue disease. No childhood history of Kawasaki’s disease. A diagnosis of adult Kawasaki’s disease was made based on LHC findings. Patient was discharged home on warfarin anticoagulation regimen.

Discussion:

In most cases of reported adult Kawasaki disease diagnosed based on findings of diffuse coronary artery ectasia, patients do not report or recall a childhood illness compatible with Kawasaki disease. Coronary artery ectasia usually occurs in patients with significant cardiac risk factors and usually affects only one or two coronary vessels. In the absence of significant cardiac risk factors and with triple coronary artery ectasia the diagnosis of adult Kawasaki disease is made as in this case.

Conclusions:

Kawasaki disease is predominately a vasculitis disease of childhood with approximately 20–25% of patients develop coronary artery sequelae, such as coronary artery aneurysms. Many are unaware that adult Kawasaki disease occurs and that to diagnose it the same criteria does not apply as that of childhood Kawasaki disease. If a physician does come across a finding of diffuse vessel ectasia it is important to be aware that the patient should be initited on antiplatelet therapy in order to prevent thrombus formation in the coronary vessels, even in the asymptomatic patient.

Figure 1Heart catheterization images showing severe diffuse coronary artery ectasia with left circumflex dominant circulation

Figure 2Heart catheterization images showing severe diffuse coronary artery ectasia with left circumflex dominant circulation