Longterm Outcomes in Patients with Giant Aneurysms Secondary to Kawasaki Disease

DEBORAH M. LEVY, EARL D. SILVERMAN, M. PATRICIA MASSICOTTE, BRIAN W. McCRINDLE, and RAE S.M. YEUNG

ABSTRACT.

Objective. Kawasaki disease (KD) has potentially serious cardiac complications including coronary artery aneurysms. Children who develop giant aneurysms (GA) are at increased risk of thrombosis and ischemia, and although longterm oral anticoagulation with warfarin is recommended, its efficacy has not been studied. We examined the longterm outcome of patients with GA secondary to KD, to determine if anticoagulation with warfarin aids in the prevention of myocardial ischemia.

Methods. We studied patients with KD followed between May 1990 and April 2000.

Results. Thirty-nine GA occurred in 2.2% of patients with KD (22/997 patients), and 33 non-GA were also identified in these patients. Patients were divided into 2 groups, those taking warfarin and no warfarin. Most patients in both groups were also taking antiplatelet agents. The demographics of the 2 groups were statistically similar, except the median duration of followup was significantly longer for patients in the no-warfarin group (6.9 vs 13.3 yrs; p = 0.008). Four early ischemic events (< 1 year after KD diagnosis) occurred (3 myocardial infarctions and one stroke). Screening for late ischemic events by stress nuclear medicine myocardial perfusion imaging revealed only one patient, in the no-warfarin group, with reversible perfusion defects. No patient had clinical signs or symptoms of late myocardial ischemia. Echocardiographic regression of aneurysms was observed in both groups. In the warfarin vs no-warfarin group, the diameters of the GA regressed a median 22% vs 32% (p = 0.27), and non-GA regressed a median of 30% vs 25% (p = 0.61). Compliance with anticoagulation was good, and no major bleeding complication of anticoagulation occurred.

Conclusion. Regression of GA occurred in most of our patients, and minimal late ischemia was observed. Further studies are required to evaluate the use of oral anticoagulation in patients with GA secondary to KD. (J Rheumatol 2005;32:928-34)

Key Indexing Terms:

KAWASAKI DISEASE
MUCOCUTANEOUS LYMPH NODE SYNDROME
WARFARIN
THROMBOSIS
CORONARY ARTERY ANEURYSM

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From the Departments of Pediatrics and Immunology, Divisions of Rheumatology, Hematology/Oncology, and Cardiology, Hospital for Sick Children Research Institute, University of Toronto, Toronto, Ontario, Canada.

Dr. Yeung is supported by the Canadian Institutes of Health Research and The Arthritis Society.

D.M. Levy, MD, FRCPC; R.S.M. Yeung, MD, PhD, FRCPC, Department of Pediatrics, Division of Rheumatology; E.D. Silverman, MD, FRCPC, Departments of Pediatrics and Immunology, Division of Rheumatology; M.P. Massicotte, MSc, MD, FRCPC, Department of Pediatrics, Division of Hematology/Oncology; B.W. McCrindle, MD, MPH, FRCPC, Department of Pediatrics, Division of Cardiology.

Address reprint requests to Dr. R.S.M. Yeung, Division of Rheumatology, Room 8124 – Elm Wing, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. E-mail: rae.yeung@sickkids.ca

Accepted for publication January 24, 2005.

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