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. 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.Electronic supplementary materialThe online version of this article (doi:10.1007/s00246-017-1590-0) contains supplementary material, which is available to authorized users.

Outcomes of Kawasaki disease with giant coronary aneurysms: a single-centre study in southwest China

“…15 In a Dutch study, the rate of the normalisation of coronary aneurysm internal lumen diameter decreased gradually in patients with z-scores of 10-15, 15-20, and >20. 16 In the current study, the incidences of cardiovascular events in the group with z-scores ≥ 15 were higher than those of the group with z-scores < 15. Nevertheless, whether there are differences between groups of z-scores ≥ 15 and <15 in other populations remains to be further studied.…”

“…In our study, 1-, 2-, and 5-year regression-free rates were similar to the rates of 0.86, 0.78, and 0.65, respectively, reported for a Dutch cohort. 16 This suggests that the normalisation of internal lumen diameter of coronary aneurysms also most often occurs at the early stage of Kawasaki disease. It is worth noting that the normalisation of internal lumen diameter of giant coronary aneurysms is not equal to normalisation of the arterial wall, with the proliferation of luminal myofibroblasts and intraluminal thrombus formation, progressive stenosis can develop.…”

Coronary artery assessment in Kawasaki disease with dual-source CT angiography to uncover vascular pathology

“…The timing to perform early cCTA following acute disease will strongly depend on the clinical presentation but seems justified to be performed after 2–3 months when increasing diameter has become less likely and before regression occurs [5].…”

“…For the circumflex (Cx), Z scores are not available. Aneurysms in the Cx were defined when the luminal diameter was ≥ 4.0 mm [5, 8]. In addition, coronary artery lesions, vascular stenosis, vessel wall calcification, intravascular thrombus, and/or plaque formations were registered.…”

Complete Regression of Giant Aneurysms in an Infant with Delayed Diagnosis and Refractory Kawasaki Disease via Combination Anticytokine Therapy: Case Report and Review of Similar Cases

“…e patient's coronary arteries, by size as well as z-score, showed continued regression over time, consistent with a remodeling process. In a study of z-scoredefined giant aneurysm regression, only 14% of children showed regression within 1 year, and only 35% after 5 years, making our patient's response atypical [15]. Given this and the other cases described, use of IL-1β and TNF-α blockade in the form of a combination/high-intensity therapy for KD should be considered for prospective study in patients with giant aneurysms.…”