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Review
Lifetime cardiovascular management of patients with previous Kawasaki disease
  1. Paul Brogan1,
  2. Jane C Burns2,3,
  3. Jacqueline Cornish4,
  4. Vinod Diwakar5,
  5. Despina Eleftheriou1,
  6. John B Gordon6,
  7. Huon Hamilton Gray7,
  8. Thomas William Johnson8,
  9. Michael Levin9,
  10. Iqbal Malik10,
  11. Philip MacCarthy11,
  12. Rachael McCormack12,
  13. Owen Miller13,
  14. Robert M R Tulloh14,15
  15. Kawasaki Disease Writing Group, on behalf of the Royal College of Paediatrics and Child Health, and the British Cardiovascular Society
  1. 1 Infection, Inflammation, and Rheumatology, UCL Institute of Child Health, London, UK
  2. 2 Pediatrics, University of California, San Diego, California, USA
  3. 3 Pediatrics, Rady Children’s Hospital San Diego, San Diego, California, USA
  4. 4 National Clinical Director Children, Young People and Transition to Adulthood, Medical Directorate, NHS England, London, UK
  5. 5 NHS Improvement, NHS England, London, UK
  6. 6 Cardiology, Sharp Memorial Hospital and San Diego Cardiac Center, San Diego, California, USA
  7. 7 National Clinical Director for Heart Disease, NHS England, London, UK
  8. 8 Cardiology, Bristol Heart Institute, Bristol, UK
  9. 9 Paediatrics, Imperial College London, London, UK
  10. 10 Imperial College London, International Centre for Circulatory Health, London, UK
  11. 11 Cardiology, Kings College Hospital, London, UK
  12. 12 Societi, The UK Foundation for Kawasaki Disease, Newark, UK
  13. 13 Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St. Thomas’ NHS Foundation Trust, London, UK
  14. 14 Department of Congenital Heart Disease, Bristol Royal Hospital for Children, Bristol, UK
  15. 15 University of Bristol, Bristol Heart Institute, Bristol, UK
  1. Correspondence to Professor Robert M R Tulloh, Department of Congenital Heart Disease, Bristol Royal Hospital for Children, Bristol BS2 8BJ, UK; robert.tulloh{at}bristol.ac.uk

Abstract

Kawasaki disease (KD) is an inflammatory disorder of young children, associated with vasculitis of the coronary arteries with subsequent aneurysm formation in up to one-third of untreated patients. Those who develop aneurysms are at life-long risk of coronary thrombosis or the development of stenotic lesions, which may lead to myocardial ischaemia, infarction or death. The incidence of KD is increasing worldwide, and in more economically developed countries, KD is now the most common cause of acquired heart disease in children. However, many clinicians in the UK are unaware of the disorder and its long-term cardiac complications, potentially leading to late diagnosis, delayed treatment and poorer outcomes. Increasing numbers of patients who suffered KD in childhood are transitioning to the care of adult services where there is significantly less awareness and experience of the condition than in paediatric services. The aim of this document is to provide guidance on the long-term management of patients who have vascular complications of KD and guidance on the emergency management of acute coronary complications. Guidance on the management of acute KD is published elsewhere.

  • Kawasaki disease
  • lifetime cardiovascular management
  • coronary artery aneurysm
  • late sequelae
  • acute coronary syndrome
  • cardiovascular risk
  • person specific protocol
  • transitional care
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Footnotes

  • Contributors All authors contributed to the design of the manuscript and attended multiple meetings over a 2-year period in order to achieve a consensus document. All authors have approved the final version of the manuscript. Members of the writing group are; Brogan PA, Burns JC, Cornish J, Diwakar V, Eleftheriou D, Gordon JB, Gray HH, Johnson T, Levin M, Malik I, MacCarthy P, McCormack R, Miller OI, Tulloh RMR.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests PB has received institutional grants from SOBI, Roche, Novartis and Novimmune and consultancy fees from SOBI, Novartis, Roche and UCB. RMRT has received grants and speaker fees from Actelion, Abbvie, GSK, Bayer, Pfizer, Jansen. Societi Foundation (RMcC) has received grants from SOBI and Roche.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.