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. 2017 Apr;38(4):833-839.
doi: 10.1007/s00246-017-1590-0. Epub 2017 Feb 24.

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

S M Dietz  1 , I M Kuipers  2 , J C D Koole  3 , J M P J Breur  4 , Z Fejzic  5 , S Frerich  6 , M Dalinghaus  7 , A A W Roest  8 , B A Hutten  4   9 , T W Kuijpers  3
Affiliations

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

S M Dietz et al. Pediatr Cardiol. 2017 Apr.

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.

Keywords: Coronary aneurysms; Major cardiac event; Mucocutaneous lymph node syndrome (Kawasaki disease); Myocardial infarction.

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Conflict of interest statement

Conflict of interest

None.

Ethical Approval

As this was a retrospective study, based on medical records, this article does not contain any studies with human participants performed by any of the authors.

Figures

Fig. 1
Fig. 1
Kaplan–Meier estimates of regression-free survival of patients with giant CAA. + Indicates censored patients
Fig. 2
Fig. 2
CAA size after 1 and 2 years. Small CAA z-score 3–5, medium CAA z-score 5–10, giant CAA ≥10. CAA= coronary artery aneurysms
Fig. 3
Fig. 3
Kaplan–Meier estimates of major adverse event-free survival of patients with giant CAA. Cardiac event- and cardiac intervention-free survival. + Indicates censored patients
See this image and copyright information in PMC

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