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中国循证儿科杂志

中国循证儿科杂志 ›› 2023, Vol. 18 ›› Issue (1): 32-36.DOI: 10.3969/j.issn.1673-5501.2023.01.005

• 论著 • 上一篇    下一篇

川崎病所致严重冠状动脉病变单中心队列研究

王程浩,梁雪村,储晨,何岚,赵璐,林怡翔,黄国英,刘芳   

  1. 复旦大学附属儿科医院心血管中心，国家儿童医学中心,上海，201102
  • 收稿日期:2022-12-06 修回日期:2022-12-20 出版日期:2023-02-25 发布日期:2023-02-25
  • 通讯作者: 刘芳

Severe coronary artery lesions due to Kawasaki disease: A single-center cohort study

#br# WANG Chenghao, LIANG Xuecun,CHU Chen, HE Lan, ZHAO Lu, LIN Yixiang, HUANG Guoying, LIU Fang#br#   

  1. Heart Center, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai 201102, China
  • Received:2022-12-06 Revised:2022-12-20 Online:2023-02-25 Published:2023-02-25
  • Contact: LIU Fang, email: liufang@fudan.edu.cn

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摘要： 背景：川崎病（KD）所致严重冠状动脉病变（CAL）容易导致患儿发生冠状动脉事件或死亡，对远期预后及生存质量具有重要影响。 目的：总结分析伴严重CAL的KD患儿的中远期临床转归，为KD患儿长期随访管理及预后评估提供依据。 设计：队列研究。 方法：检索复旦大学附属儿科医院（我院）HIS系统，纳入2008年1月至2019年12月出院诊断为KD，经我院冠状动脉造影（CAG）确诊为KD所致严重CAL的＜18岁患儿。排除合并其他引起严重CAL和影响严重CAL预后疾病者。所有患儿在门诊及病房由资深心血管专科医生随访，均以药物治疗为基础，伴冠状动脉严重狭窄及心肌缺血的患儿经多学科讨论后予手术治疗。以确诊及随访期间出现心肌缺血、心肌梗死或死亡的患儿为预后不良组，反之则为预后良好组，观察出现不良预后及CAL的演变情况，分析不良预后的影响因素。 主要结局指标：不良预后发生情况和CAL的恢复情况。 结果：144例KD所致严重CAL患儿纳入本文分析。其中男117例（81.2%），女27例；KD中位发病年龄2.2（1.0，4.5）岁，中位随访时间1.6（0，3.4）年，确诊为严重CAL时病程为5.0（3.0，21.3）月。144例KD所致严重CAL患儿共累及276支冠状动脉，56例（38.9%）累及单支冠状动脉，88例（61.1%）累及≥2支冠状动脉。发生巨大冠状动脉瘤（GCAA)122例，单支冠状动脉多发中小型冠状动脉瘤（CAA)8例，GCAA合并单支冠状动脉多发中小型CAA 6例，受累冠状动脉均狭窄8例；62例发生冠状动脉狭窄，其中冠状动脉闭塞31例。预后不良组54例（37.5%），均存在心肌缺血，其中14例患儿缺血心肌处发生过有记录的急性心肌梗死，1例患儿因心肌梗死所致严重心力衰竭死亡。多因素分析结果显示，≥2支冠状动脉受累的患儿是单支冠状动脉受累的患儿发生不良预后风险的2.6倍（OR=2.602，95%CI：1.201~5.634），合并冠状动脉狭窄的患儿是未合并冠状动脉狭窄的患儿发生不良预后风险的3倍（OR=3.055，95%CI：1.450~6.437）。共行179次CAG，114例（208支受累冠状动脉）行1次CAG，30例（68支受累冠状动脉）行≥2次CAG。68支受累冠状动脉首次确诊时，51支为GCAA，末次随访时好转率为4.0%；3支为多发中小型CAA，末次随访时好转率为66.6%；2支为单个中小型CAA，至末次随访均发生狭窄（狭窄程度约50%）；12支为狭窄，至末次随访时均未好转。 结论：GCAA是KD所致严重CAL的主要类型，且难以恢复，单支冠状动脉多发中小型CAA好转率较高。冠状动脉狭窄及多支冠状动脉受累是KD所致严重CAL患儿发生不良预后的影响因素。

关键词: 川崎病, 冠状动脉病变, 冠状动脉瘤

Abstract: Background：Severe coronary artery lesions (CAL) caused by Kawasaki disease (KD) can lead to coronary events or death in children and has an important impact on long-term prognosis and quality of life. Objective：To summarize and analyze the medium-term and long-term clinical outcomes of KD children with severe CAL and provide the basis for long-term follow-up management and prognosis evaluation of KD children. Design：Cohort study. Methods：The HIS system of Children's Hospital of Fudan University was searched to include children < 18 years of age who were discharged from the hospital from January 2008 to December 2019 with a diagnosis of KD and were diagnosed with severe CAL due to KD by coronary angiography (CAG) in our hospital. Patients with other diseases causing severe CAL and affecting the prognosis of severe CAL were excluded. All children were followed up by senior cardiovascular specialists in the outpatient clinic and ward, and were given medical treatment. Children with severe coronary artery stenosis and myocardial ischemia were treated surgically after multidisciplinary discussion. Children with myocardial ischemia, myocardial infarction or death during diagnosis and follow-up were taken as the poor prognosis group, and the opposite was the good prognosis group. The poor prognosis and the evolution of CAL were observed, and the influencing factors of poor prognosis were analyzed. Main outcome measures：Poor prognosis and improvement of CAL. Results：A total of 144 children with severe CAL due to KD were included in this analysis. Among them, 117(81.2%) were males and 27 were females with a median KD onset age of 2.2(1.0, 4.5) years, a median follow-up time of 1.6(0, 3.4) years, and a disease duration of 5.0(3.0, 21.3) months at the diagnosis of severe CAL. A total of 276 coronary arteries were involved in 144 children with KD and severe CAL. Single coronary arteries and ≥2 coronary arteries were involved in 56 cases(38.9%) and 88 cases(61.1%) respectively. There were 122 cases of giant coronary artery aneurysm(GCAA), 8 cases of multiple small and medium-sized coronary artery aneurysms(CAA) in the single coronary artery, 6 cases of GCAA with multiple small and medium-sized CAA in the single coronary artery, and 8 cases of stenosis in all affected coronary arteries. Sixty-two cases developed coronary artery stenosis, including 31 cases of coronary artery occlusion. Fifty-four of 144 children(37.5%) developed poor prognosis, and myocardial ischemia was present in all 54 cases, 14 of which had developed recorded acute myocardial infarction at the ischemic myocardium, and one child died of severe heart failure due to myocardial infarction. The results of multivariate analysis showed that the risk of poor prognosis was 2.6 times higher in children with ≥2 coronary artery involvement than in those with only 1 coronary artery involvement(OR=2.602, 95% CI: 1.201 to 5.634), and 3 times higher in children with coronary artery stenosis than in those without coronary artery stenosis(OR=3.055, 95% CI: 1.450 to 6.437). Among 179 CAG, 114 patients(208 affected coronary arteries) underwent one CAG, and 30 patients(68 affected coronary arteries) underwent≥2 CAG. When 68 affected coronary arteries were first diagnosed, 51 of them were GCAA and the improvement rate was 4.0% at the last follow-up; 3 of them were multiple small and medium-sized CAAs and the improvement rate was 66.6% at the last follow-up; 2 were single small and medium-sized CAAs and developed stenosis(stenosis degree about 50%) until the last follow-up; and 12 were stenosis and did not improve until the last follow-up. Conclusions：GCAA is the main type of KD with severe CAL, and it is difficult to recover. The improvement rate of multiple small and medium-sized CAAs in the single coronary artery is high. Coronary artery stenosis and multivessel coronary artery involvement are factors leading to the poor prognosis of children with severe CAL caused by KD.

Key words: Kawasaki disease, Coronary artery lesions, Coronary artery aneurysms