title: Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre (AMC), Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. s.m.dietz@amc.uva.nl.
authors.forename: S M
authors.initials: SM
authors.lastname: Dietz
authors.affiliation: Department of Pediatric Cardiology, Emma Children's Hospital, AMC, Amsterdam, The Netherlands.
authors.forename: I M
authors.initials: IM
authors.lastname: Kuipers
authors.affiliation: Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre (AMC), Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors.forename: J C D
authors.initials: JCD
authors.lastname: Koole
authors.affiliation: Department of Pediatric Cardiology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: J M P J
authors.initials: JMPJ
authors.lastname: Breur
authors.affiliation: Department of Pediatric Cardiology, Amalia Children's Hospital, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Z
authors.initials: Z
authors.lastname: Fejzic
authors.affiliation: Department of Pediatric Cardiology, Academic Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Frerich
authors.affiliation: Department of Pediatric Cardiology, Sophia Children's hospital, Erasmus Medical Centre, Rotterdam, The Netherlands.
authors.forename: M
authors.initials: M
authors.lastname: Dalinghaus
authors.affiliation: Department of Pediatric Cardiology, Willem-Alexander Children's Hospital, Leids University Medical Centre, Leiden, The Netherlands.
authors.forename: A A W
authors.initials: AAW
authors.lastname: Roest
authors.affiliation: Department of Pediatric Cardiology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.|Department of Clinical Epidemiology, Biostatistics and Bioinformatics, AMC, Amsterdam, The Netherlands.
authors.forename: B A
authors.initials: BA
authors.lastname: Hutten
authors.affiliation: Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre (AMC), Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors.forename: T W
authors.initials: TW
authors.lastname: Kuijpers
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003323:Coronary Aneurysm / Q000209:etiology / Q000503:physiopathology*; D005260:Female; D006305:Health Status Indicators; D006331:Heart Diseases / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications*; D009426:Netherlands; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D055815:Young Adult
keywords: Coronary aneurysms; Major cardiac event; Mucocutaneous lymph node syndrome (Kawasaki disease); Myocardial infarction