QJM Advance Access published August 8, 2012

Q J Med doi:10.1093/qjmed/hcs150

Clinical picture
Adult Kawasaki disease
A 44-year-old male presented with 1 h history of substernal, persistent pressure-like chest pain. The pain had no radiation and started while he was watching TV. He denied associated nausea, vomiting, palpitations or shortness of breath. He has history of hypertension controlled with irbesartan. He never smoked or drank alcohol. Exam showed normal vital signs; cardiovascular examination showed regular rate and rhythm, no gallops, murmurs or pericardial rub, had symmetric and bounding distal pulses. Electrocardiogram showed normal sinus rhythm with no ST or T wave changes, and with normal intervals. Chest X-ray was normal. Laboratory studies showed elevated cardiac enzymes: CK 662 U/L (reference range: 30220 U/L) and CK MB 8.1% (reference range: 0.04.0%), troponin T 2.28 ng/ml (reference range: 0.000.10 ng/ml). He underwent an emergent left heart catheterization (LHC) for non-ST-elevation myocardial infarction (NSTEMI). Surprisingly, he had diffuse triple coronary artery ectasia with no evidence of focal stenosis or thrombosis (Figure 1). No intervention was done since there was no coronary stenosis. Echocardiography showed preserved left ventricular function with ejection fraction of 60% and no evidence of valvular disease. Erythrocyte sedimentation rate (ESR) was 3 mm/h. He denied childhood history of vasculitisincluding Kawasakisor any autoimmune disease. Based on the LHC findings and work up, a diagnosis of adult Kawasakis disease was made in the absence of significant cardiac risk factors. The patient was started on aspirin, clopidogrel and enoxaparin with bridging to coumadin on discharge. Coronary artery ectasia is defined as an arterial segment dilatation at least 1.5 times that of an adjacent normal coronary artery.1,2 Most cases of reported adult Kawasakis disease diagnosis are based on findings of diffuse coronary artery ectasia and patients do not report or recall a childhood illness compatible with Kawasakis disease.3 Coronary artery ectasia usually occurs in patients with significant cardiac risk factors and usually affects only one or two coronary vessels. In this case, the presence of

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Figure 1. Right coronary angiogram recorded in the right anterior oblique (cranial) view showing severe diffuse coronary artery ectasia of the posterior descending and right coronary artery (arrow).

triple coronary artery ectasia with absence of significant cardiac risk factors prompted the diagnosis of adult Kawasaki disease.35 The aim of management of coronary artery ectasia is preventing formation of thrombus within these vessels. A paucity of evidence evaluating treatment of coronary artery ectasia is available; however, recommendations are to start patients on an antiplatelet agent such as aspirin and long-term anticoagulation with warfarin to prevent thrombus formation.1,2 Coronary spasm has been hypothesized as the etiology of angina in patients with coronary artery ectasia and calcium-channel-blockers have been recommended for their antispasmodic properties.2,6 Kawasaki disease is predominately a vasculitis disease of childhood; 2025% of patients develop coronary artery aneurysms.1 Increased awareness

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Clinical picture that the condition can occur in adults is important, as well as that a history of Kawasakis disease in childhood is not required for diagnosis. Photographs and text from: A. Ataya, Department of Medicine, Medicine Institute, Cleveland Clinic, 9500 Euclid Avenue, NA1, Cleveland, OH 44195, USA; A. H. Alraiyes, Department of Pulmonary Diseases, Critical Care, & Environmental Medicine, Tulane University Health Sciences Centre, 1430 Tulane Avenue, SL-9, New Orleans, LA 70112, USA; M. Auron, Department of Hospital Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, 9500 Euclid Avenue, A13, Cleveland, OH 44195, USA; M C. Alraies, Department of Hospital Medicine Institute, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, 9500 Euclid Avenue, A13, Cleveland, OH 44195, USA. email: alraiec@ccf.org Conflict of interest: None declared.

References
1. Mavrogeni S. Coronary artery ectasia: from diagnosis to treatment. Hellenic J Cardiol. 2010; 51:15863. 2. Sorrell VL, Davis MJ, Bove AA. Current knowledge and significance of coronary artery ectasia: a chronologic review of the literature, recommendations for treatment, possible etiologies, and future considerations. Clin Cardiol. 1998; 21:15760. 3. Gomard-Mennesson E, Landron C, Dauphin C, Epaulard O, Petit C, Green L, et al. Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore). 2010; 89:14958. 4. Seve P, Stankovic K, Smail A, Durand DV, Marchand G, Broussolle C. Adult Kawasaki disease: report of two cases and literature review. Semin Arthritis Rheum. 2005; 34:78592. 5. Canino-Rodriguez A, Cox RA. Giant coronary aneurysms in a young adult patient with Kawasaki disease. P R Health Sci J. 2008; 27:3826. 6. Perlman PE, Ridgeway NA. Thrombosis and anticoagulation therapy in coronary ectasia. Clin Cardiol. 1989; 12:5412.

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