Warfarin plus aspirin or aspirin alone for patients with giant coronary artery aneurysms secondary to Kawasaki disease?

flammatory process and reducing coronary artery damage [5–7] . However, despite the growing armamentarium of anti-inflammatory treatments, 5–10% of patients with Kawasaki disease are left with coronary artery injury, ranging from mild dilatation to severe and persistent aneurysms [8] . Mid-term follow-up into the second and third decade of life of children who have had normal echocardiograms provides reassurance that these young adults have no observable cardiovascular sequelae of their Kawasaki disease [9] . Follow-up of patients who suffered coronary artery injury as manifested by CAAs measuring less than 10 standard deviations from the mean normalized for body surface area (Z score) is in progress and the outcomes are still being defined [10] . However, for those patients who have developed giant CAAs (with diameters in excess of 8 mm) the long-term outcome is extremely worrying [11– 13] . In a review of 245 patients with giant aneurysms, Tsuda et al. [11] reported that 60% of children followed for a median of 20 years after the onset of Kawasaki disease required coronary artery interventions, such as angioplasty, coronary artery bypass grafting or cardiac transplantation; 10% died and only a third remained well without either suffering a myocardial infarct or requiring cardiac intervention. Considering that most children Kawasaki disease is an acute inflammatory disorder predominantly affecting young children. Since its initial description by Dr. Kawasaki in children in Japan, the disease has emerged as a relatively common childhood condition with an incidence varying from 240/100,000 children aged under 5 years in Japan to 8–19/100,000 in the USA and Europe [1, 2] . Although epidemiological features strongly suggest an infectious aetiology, the cause of Kawasaki disease remains unknown, and current thinking suggests that the disease is triggered by one or more as yet unidentified pathogens or their toxins, which initiate an inflammatory process in genetically predisposed individuals [3] . Prior to the introduction of intravenous immunoglobulin, which is now the standard recommended treatment, 20–30% of children with Kawasaki disease developed coronary artery aneurysms (CAAs) [4] . Although intravenous immunoglobulin reduces the risk of CAAs to approximately 5–10%, a significant proportion of affected children fail to respond to immunoglobulin or are treated too late to prevent coronary artery damage. For those patients who fail to respond to intravenous immunoglobulin there is growing evidence that steroids or other antiinflammatory agents, such as infliximab, anakinra or cyclosporine, may be effective in controlling the inReceived: July 21, 2014 Accepted: July 21, 2014 Published online: October 7, 2014