Xiong Y, Zhang Y L, Du Z D
Department of Cardiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Zhonghua Er Ke Za Zhi. 2021 Feb 2;59(2):101-106. doi: 10.3760/cma.j.cn112140-20201124-01052.
To summarize the clinical features, middle-and long-term prognosis of Kawasaki disease (KD) with giant coronary artery aneurysm (GCAA). In this retrospective cohort study, a cross-sectional analysis was conducted on 101 KD children with GCAA in the KD with GCAA database established by Beijing Children's Hospital, Capital Medical University in 2004. GCAA was diagnosed as coronary artery absolute lumen diameter ≥8.0 mm. All patients were followed up regularly. The endpoint was the time of last follow-up or the death time. test or χ test was used for comparison between groups. A total of 101 KD children with GCAA were enrolled, including 82 males (81.2%) and 19 females (18.8%). The age of disease onset was 2.5 (1.0, 4.5) years. The follow-up duration was 4.5 (2.7, 7.5) years, with a longest of 19 years. All children received routine treatment with aspirin and warfarin, and clopidogrel was added in severe cases. At the end of follow-up, 13 cases (12.9%) had cardiac enlargement, 11 cases (10.9%) developed heart failure, 13 cases (12.9%) experienced myocardial infarction, 2 cases (2.0%) underwent coronary artery bypass graft and 6 cases (5.9%) died. A total of 170 coronary arteries were involved, including 24 (14.1%) GCAAs on the main trunk of left coronary artery, 10 (5.9%) GCAAs on left circumflex, 57 (33.5%) GCAAs on left anterior descending, 78 (45.9%) GCAAs on the middle segments of right coronary artery, and 1 (0.6%) GCAA in the distal segments of right coronary artery. Eleven cases (10.9%) recovered with the coronary artery absolute lumen diameter of all GCAAs below 4.0 mm. Among 170 branches with GCAAs, 28 (16.5%) regressed below 4.0 mm. No significant difference was found in the regression rates between right and left GCAA (18.7% (17/91) 13.9% (11/79), χ²=2.473, 0.116). There was no statistically significant difference in retraction between unilateral GCAA and bilateral GCAA (16.1% (9/56) 4.4% (2/45), χ=2.381, 0.123). GCAA of KD occurred more common in the middle segments of right and left anterior descending coronary arteries. The incidence of adverse cardiac events and the mortality rate in children with GCAA complicated with KD was high. Their long-term prognosis was poor.
总结川崎病（KD）合并巨大冠状动脉瘤（GCAA）的临床特征、中长期预后。在这项回顾性队列研究中，对首都医科大学附属北京儿童医院2004年建立的KD合并GCAA数据库中101例KD合并GCAA患儿进行横断面分析。GCAA诊断为冠状动脉绝对管腔直径≥8.0mm。所有患者均定期随访。终点为末次随访时间或死亡时间。组间比较采用检验或χ检验。共纳入101例KD合并GCAA患儿，其中男性82例（81.2%），女性19例（18.8%）。发病年龄为2.5（1.0，4.5）岁。随访时间为4.5（2.7，7.5）年，最长19年。所有患儿均接受阿司匹林和华法林常规治疗，重症病例加用氯吡格雷。随访结束时，13例（12.9%）出现心脏扩大，11例（10.9%）发生心力衰竭，13例（12.9%）发生心肌梗死，2例（2.0%）接受冠状动脉搭桥术，6例（5.9%）死亡。共累及170支冠状动脉，其中左冠状动脉主干24支（14.1%）、左旋支10支（5.9%）、左前降支57支（33.5%）、右冠状动脉中段78支（45.9%）、右冠状动脉远端1支（0.6%）。11例（10.9%）患儿冠状动脉绝对管腔直径均恢复至4.0mm以下。在170支合并GCAA的分支中，28支（16.5%）回缩至4.0mm以下。左右侧GCAA回缩率差异无统计学意义（18.7%（17/91）对13.9%（11/79），χ²=2.473，P=0.116）。单侧GCAA与双侧GCAA回缩情况差异无统计学意义（16.1%（9/56）对4.4%（2/45），χ=2.381，P=0.123）。KD合并的GCAA多见于左右冠状动脉前降支中段。KD合并GCAA患儿不良心脏事件发生率及死亡率高，长期预后差。
