Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients

S. M. Dietz*, I. M. Kuipers, J. C D Koole, J. M P J Breur, Z. Fejzic, S. Frerich, M. Dalinghaus, A. A W Roest, B. A. Hutten, T. W. Kuijpers

*Corresponding author for this work

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Abstract

Kawasaki disease (KD) is a pediatric vasculitis. Its main complication is the development of coronary artery aneurysms (CAA), with giant CAA at the end of the spectrum. We evaluated regression and event-free rates in a non-Asian cohort of patients with giant CAA using the current z-scores adjusted for body surface area instead of absolute diameters. KD patients with giant CAA (z-score ≥10) visiting our outpatient clinic between January 1999 and September 2015 were included. Patient characteristics and clinical details were extracted from medical records. Regression was defined as all coronary arteries having a z-score of ≤3. A major adverse event was defined as cardiac death, myocardial infarction, cardiogenic shock, or any coronary intervention. Regression-free and event-free rates were calculated using the Kaplan–Meier method. We included 52 patients with giant CAA of which 45 had been monitored since the acute phase. The 1-, 2-, and 5-year regression-free rates were 0.86, 0.78, and 0.65, respectively. The 5-year, 10-year, and 15-year event-free rates were 0.79, 0.75, and 0.65, respectively. Four children, whose CAA would not have been classified as ‘giant’ based on absolute diameters instead of z-scores, had experienced an event during follow-up. Conclusion: We found a high percentage of children in whom the lumen of giant CAA completely normalized. Four children not classified as ‘giant’ based on absolute diameters with z-scores of ≥10 experienced a cardiac event. Hence, the use of z-scores seems to be justified.

Original languageEnglish
Pages (from-to)833-839
Number of pages7
JournalPediatric Cardiology
Volume38
Issue number4
DOIs
Publication statusPublished - Apr 2017

Keywords

  • Adolescent
  • Child
  • Child, Preschool
  • Coronary Aneurysm
  • Female
  • Health Status Indicators
  • Heart Diseases
  • Humans
  • Infant
  • Male
  • Mucocutaneous Lymph Node Syndrome
  • Netherlands
  • Remission, Spontaneous
  • Retrospective Studies
  • Young Adult
  • Journal Article

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