Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients.
Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients.
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DOI：
10.1007/s00246-017-1590-0
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发表时间：
2017-04
影响因子：
1.6
通讯作者：
Kuijpers TW
中科院分区：
文献类型：
--
作者：
Dietz SM;Kuipers IM;Koole JCD;Breur JMPJ;Fejzic Z;Frerich S;Dalinghaus M;Roest AAW;Hutten BA;Kuijpers TW
川崎病是一种小儿血管炎。其主要并发症是冠状动脉动脉瘤（CAA）的发展，其中最大的是巨大的CAA。我们使用体表面积而不是绝对直径调整的当前z评分，评估了非亚洲巨型CAA患者队列的回归和无事件率。纳入1999年1月至2015年9月来我院门诊就诊的伴有巨大CAA （z-score≥10）的KD患者。从医疗记录中提取患者特征和临床细节。回归定义为所有冠状动脉z-score≤3。主要不良事件定义为心源性死亡、心肌梗死、心源性休克或任何冠状动脉介入治疗。使用Kaplan-Meier方法计算无回归率和无事件率。我们纳入了52例巨大CAA患者，其中45例自急性期开始监测。1年、2年和5年无回归率分别为0.86、0.78和0.65。5年、10年和15年无事件发生率分别为0.79、0.75和0.65。根据绝对直径而不是z分数，四个孩子的CAA不会被归类为“巨人”，他们在随访期间经历了一个事件。结论：我们发现儿童巨CAA管腔完全正常化的比例很高。4名根据绝对直径未被归类为“巨人”且z评分≥10的儿童经历了心脏事件。因此，使用z分数似乎是合理的。本文的在线版本（doi:10.1007/s00246-017-1590-0）包含补充材料，仅供授权用户使用。
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. The online version of this article (doi:10.1007/s00246-017-1590-0) contains supplementary material, which is available to authorized users.