Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients
Dietz, S. M.; Kuipers, I. M.; Koole, J. C D; Breur, J. M P J; Fejzic, Z.; Frerich, S.; Dalinghaus, M.; Roest, A. A W; Hutten, B. A.; Kuijpers, T. W.
(2017) Pediatric Cardiology, volume 38, issue 4, pp. 833 - 839
(Article)
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
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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.
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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, Pediatrics, Perinatology, and Child Health, Cardiology and Cardiovascular Medicine, Journal Article
ISSN: 0172-0643
Publisher: Springer New York
Note: Funding Information: This work was supported by the Stinafo foundation (The Hague, The Netherlands) and the Schumacher Kramer Foundation (Amsterdam, The Netherlands). The sponsors had no role in the study design, the data collection and analysis, the writing of the report, or the decision to submit the manuscript for publication. Publisher Copyright: © 2017, The Author(s).
(Peer reviewed)