Warfarin plus Aspirin or Aspirin Alone for Patients with Giant Coronary Artery Aneurysms Secondary to Kawasaki Disease?

Editorial Comment Cardiology 2014;129:174–177 DOI: 10.1159/000366052 Received: July 21, 2014 Accepted: July 21, 2014 Published online: October 7, 2014 Warfarin plus Aspirin or Aspirin Alone for Patients with Giant Coronary Artery Aneurysms Secondary to Kawasaki Disease? Michael Levin a Jane C. Burns b, d John B. Gordon c, e a Section for Paediatrics, Division of Medicine, Imperial College London, London , UK; Departments of b Pediatrics and Medicine, University of California San Diego School of Medicine, d Rady Children’s Hospital, and e San Diego Cardiac Center, San Diego, Calif. , USA c © 2014 S. Karger AG, Basel E-Mail karger@karger.com www.karger.com/crd flammatory process and reducing coronary artery dam- age [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 an- eurysms [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 ob- servable cardiovascular sequelae of their Kawasaki dis- ease [9] . Follow-up of patients who suffered coronary ar- tery 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 dis- ease required coronary artery interventions, such as an- gioplasty, 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 Michael Levin, FRCPCH, FMedSci Section for Paediatrics, Division of Medicine Imperial College London, Norfolk Place London W2 1PG (UK) E-Mail m.levin @ imperial.ac.uk Downloaded by: 66.75.57.150 - 2/22/2015 8:18:50 PM Kawasaki disease is an acute inflammatory disorder predominantly affecting young children. Since its initial description by Dr. Kawasaki in children in Japan, the dis- ease has emerged as a relatively common childhood con- dition with an incidence varying from 240/100,000 chil- dren aged under 5 years in Japan to 8–19/100,000 in the USA and Europe [1, 2] . Although epidemiological fea- tures strongly suggest an infectious aetiology, the cause of Kawasaki disease remains unknown, and current think- ing suggests that the disease is triggered by one or more as yet unidentified pathogens or their toxins, which initi- ate an inflammatory process in genetically predisposed individuals [3] . Prior to the introduction of intravenous immunoglob- ulin, which is now the standard recommended treatment, 20–30% of children with Kawasaki disease developed cor- onary artery aneurysms (CAAs) [4] . Although intrave- nous immunoglobulin reduces the risk of CAAs to ap- proximately 5–10%, a significant proportion of affected children fail to respond to immunoglobulin or are treat- ed too late to prevent coronary artery damage. For those patients who fail to respond to intravenous immunoglob- ulin there is growing evidence that steroids or other anti- inflammatory agents, such as infliximab, anakinra or cyclosporine, may be effective in controlling the in-