川崎病患者冠状动脉瘤严重程度与后期冠状动脉事件风险的相关性研究。
Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events.
机构信息
Department of Cardiology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
出版信息
JAMA Pediatr. 2018 May 7;172(5):e180030. doi: 10.1001/jamapediatrics.2018.0030.
IMPORTANCE
Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA).
OBJECTIVE
To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA.
DESIGN, SETTING, AND PARTICIPANTS: This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011.
MAIN OUTCOMES AND MEASURES
The time-dependent occurrence of CE, including thrombosis, stenosis, obstruction, acute ischemic events, and coronary interventions, was analyzed for small (z score, <5), medium (z score, ≥5 to <10; actual internal diameter, <8 mm), and large (z score, ≥10 or ≥8 mm) CAA by the Kaplan-Meier method. The Cox proportional hazard regression model was used to identify risk factors for CE after adjusting for age, sex, size, morphology, number of CAA, resistance to initial intravenous immunoglobulin (IVIG) therapy, and antithrombotic medications.
RESULTS
Of 1006 patients, 714 (71%) were male, 341 (34%) received a diagnosis before age 1 year, 501 (50%) received a diagnosis between age 1 and 5 years, and 157 (16%) received a diagnosis at age 5 years or older. The 10-year event-free survival rate for CE was 100%, 94%, and 52% in men (P < .001) and 100%, 100%, and 75% in women (P < .001) for small, medium, and large CAA, respectively. The CE-free rate was 100%, 96%, and 79% in patients who were not resistant to IVIG therapy (P < .001) and 100%, 96%, and 51% in patients who were resistant to IVIG therapy (P < .001), respectively. Cox regression analysis revealed that large CAA (hazard ratio, 8.9; 95% CI, 5.1-15.4), male sex (hazard ratio, 2.8; 95% CI, 1.7-4.8), and resistance to IVIG therapy (hazard ratio, 2.2; 95% CI, 1.4-3.6) were significantly associated with CE.
CONCLUSIONS AND RELEVANCE
Classification using the internal diameter z score is useful for assessing the severity of CAA in relation to the time-dependent occurrence of CE and associated factors in patients with KD. Careful management of CE is necessary for all patients with KD with CAA, especially men and IVIG-resistant patients with a large CAA.
重要性
很少有研究具有足够的统计能力来显示川崎病（KD）并发冠状动脉瘤（CAA）患者冠状动脉内径 z 分数与冠状动脉事件（CE）之间的关联。
目的
阐明 z 分数与并发 CAA 的 KD 患者时间依赖性 CE 发生之间的关系。
设计、地点和参与者：这项多中心合作回顾性队列研究包括 44 个参与机构的 1006 名年龄小于 19 岁的 KD 患者，他们于 1992 年至 2011 年期间接受了冠状动脉造影。
主要结果和测量指标
采用 Kaplan-Meier 法分析小（z 分数，<5）、中（z 分数，≥5 至 <10；实际内径，<8 mm）和大（z 分数，≥10 或≥8 mm）CAA 的 CE 时间依赖性发生情况。使用 Cox 比例风险回归模型，在校正年龄、性别、大小、形态、CAA 数量、对初始静脉注射免疫球蛋白（IVIG）治疗的耐药性和抗血栓药物后，确定 CE 的危险因素。
结果
在 1006 名患者中，714 名（71%）为男性，341 名（34%）在 1 岁前被诊断，501 名（50%）在 1 至 5 岁之间被诊断，157 名（16%）在 5 岁或以上被诊断。CE 的 10 年无事件生存率在男性中分别为 100%、94%和 52%（P<0.001），在女性中分别为 100%、100%和 75%（P<0.001），小、中、大 CAA 患者分别为 100%、96%和 79%（P<0.001），对 IVIG 治疗耐药的患者为 100%、96%和 51%（P<0.001）。Cox 回归分析显示，大 CAA（风险比，8.9；95%CI，5.1-15.4）、男性（风险比，2.8；95%CI，1.7-4.8）和对 IVIG 治疗耐药（风险比，2.2；95%CI，1.4-3.6）与 CE 显著相关。
结论和相关性
使用内径 z 分数分类对于评估 KD 患者 CAA 严重程度与 CE 时间依赖性发生及其相关因素之间的关系是有用的。所有并发 CAA 的 KD 患者均需密切管理 CE，特别是男性和对大 CAA 有 IVIG 耐药性的患者。
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