CAJ | 학술논문

目的总结川崎病合并冠状动脉巨大瘤(GCAA)的临床特点和转归.方法 2003年1月至2012年12月广州市妇女儿童医疗中心确诊川崎病合并GCAA 55例,失访7例,纳入本组研究48例(男43例,女5例).按照GCAA发生部位分为双侧GCAA组和单侧GCAA组.按照随访时间分为≤1年、＞1 ～3年、＞3 ～5年、＞5 ～10年组.入组患儿分别于急性期、出院后2周、1个月、3个月、6个月、1年复诊,1年以后每3～6个月复诊1次;所有纳入患儿均行超声心动图及心电图检查,部分患儿行冠状动脉造影(CAG)及CT冠状动脉成像(CTA)检查.随访其临床表现及不同随访时间和不同部位GCAA受累血管的变化情况.结果 (1)川崎病合并GCAA患儿的发病年龄2个月～10岁,其中1岁以内20例(42％),3岁以内30例(62％),随访时间(4.0±3.1)年.观察终点年龄1.5 ～19岁,平均(6.8±4.2)岁.(2)随访≤1年组GCAA无明显变化、回缩及狭窄的比例分别为48％、48％及4％;随访＞1～3年组GCAA无明显变化、回缩及狭窄的比例分别为39％、39％及22％;随访＞3 ～5年组GCAA无明显变化、回缩及狭窄的比例分别为30％、35％及35％;随访＞5～10年组GCAA无明显变化、回缩及狭窄的比例分别为30％、20％及50％.＞1～3年、＞3 ～5年、＞5～10年与随访≤1年组相比较,GCAA发生狭窄的比例差异有统计学意义(x2=6.026、11.121、15.652,P=0.019、0.002、0.001).(3) 48例患儿中双侧GCAA组20例,单侧GCAA组28例,双侧GCAA组发生回缩比例低于单侧组[15％(3/20)比36％(10/28)].两组患儿受累冠状动脉转归差异无统计学意义(P＞0.05).(4)48例患儿中严重缺血性心肌病6例,其中死亡2例.(5)CTA发现冠状动脉管壁增厚、附壁血栓及钙化改变,CAG可显示冠状动脉闭塞、机化再通及侧支血管形成.结论川崎病合并GCAA好发于1岁以下婴儿,尤其6个月以下婴儿.随访时间增加,GCAA发生狭窄的比例明显升高.双侧GCAA发生缺血性心肌病和死亡的比例高于单侧.超声心动图、CTA和CAG结合应用,对发现冠状动脉血栓、狭窄及闭塞有重要帮助. 目的總結川崎病閤併冠狀動脈巨大瘤(GCAA)的臨床特點和轉歸.方法 2003年1月至2012年12月廣州市婦女兒童醫療中心確診川崎病閤併GCAA 55例,失訪7例,納入本組研究48例(男43例,女5例).按照GCAA髮生部位分為雙側GCAA組和單側GCAA組.按照隨訪時間分為≤1年、＞1 ～3年、＞3 ～5年、＞5 ～10年組.入組患兒分彆于急性期、齣院後2週、1箇月、3箇月、6箇月、1年複診,1年以後每3～6箇月複診1次;所有納入患兒均行超聲心動圖及心電圖檢查,部分患兒行冠狀動脈造影(CAG)及CT冠狀動脈成像(CTA)檢查.隨訪其臨床錶現及不同隨訪時間和不同部位GCAA受纍血管的變化情況.結果 (1)川崎病閤併GCAA患兒的髮病年齡2箇月～10歲,其中1歲以內20例(42％),3歲以內30例(62％),隨訪時間(4.0±3.1)年.觀察終點年齡1.5 ～19歲,平均(6.8±4.2)歲.(2)隨訪≤1年組GCAA無明顯變化、迴縮及狹窄的比例分彆為48％、48％及4％;隨訪＞1～3年組GCAA無明顯變化、迴縮及狹窄的比例分彆為39％、39％及22％;隨訪＞3 ～5年組GCAA無明顯變化、迴縮及狹窄的比例分彆為30％、35％及35％;隨訪＞5～10年組GCAA無明顯變化、迴縮及狹窄的比例分彆為30％、20％及50％.＞1～3年、＞3 ～5年、＞5～10年與隨訪≤1年組相比較,GCAA髮生狹窄的比例差異有統計學意義(x2=6.026、11.121、15.652,P=0.019、0.002、0.001).(3) 48例患兒中雙側GCAA組20例,單側GCAA組28例,雙側GCAA組髮生迴縮比例低于單側組[15％(3/20)比36％(10/28)].兩組患兒受纍冠狀動脈轉歸差異無統計學意義(P＞0.05).(4)48例患兒中嚴重缺血性心肌病6例,其中死亡2例.(5)CTA髮現冠狀動脈管壁增厚、附壁血栓及鈣化改變,CAG可顯示冠狀動脈閉塞、機化再通及側支血管形成.結論川崎病閤併GCAA好髮于1歲以下嬰兒,尤其6箇月以下嬰兒.隨訪時間增加,GCAA髮生狹窄的比例明顯升高.雙側GCAA髮生缺血性心肌病和死亡的比例高于單側.超聲心動圖、CTA和CAG結閤應用,對髮現冠狀動脈血栓、狹窄及閉塞有重要幫助.
목적 총결천기병합병관상동맥거대류(GCAA)적림상특점화전귀.방법 2003년1월지2012년12월엄주시부녀인동의료중심학진천기병합병GCAA 55례,실방7례,납입본조연구48례(남43례,녀5례).안조GCAA발생부위분위쌍측GCAA조화단측GCAA조.안조수방시간분위≤1년、＞1 ～3년、＞3 ～5년、＞5 ～10년조.입조환인분별우급성기、출원후2주、1개월、3개월、6개월、1년복진,1년이후매3～6개월복진1차;소유납입환인균행초성심동도급심전도검사,부분환인행관상동맥조영(CAG)급CT관상동맥성상(CTA)검사.수방기림상표현급불동수방시간화불동부위GCAA수루혈관적변화정황.결과 (1)천기병합병GCAA환인적발병년령2개월～10세,기중1세이내20례(42％),3세이내30례(62％),수방시간(4.0±3.1)년.관찰종점년령1.5 ～19세,평균(6.8±4.2)세.(2)수방≤1년조GCAA무명현변화、회축급협착적비례분별위48％、48％급4％;수방＞1～3년조GCAA무명현변화、회축급협착적비례분별위39％、39％급22％;수방＞3 ～5년조GCAA무명현변화、회축급협착적비례분별위30％、35％급35％;수방＞5～10년조GCAA무명현변화、회축급협착적비례분별위30％、20％급50％.＞1～3년、＞3 ～5년、＞5～10년여수방≤1년조상비교,GCAA발생협착적비례차이유통계학의의(x2=6.026、11.121、15.652,P=0.019、0.002、0.001).(3) 48례환인중쌍측GCAA조20례,단측GCAA조28례,쌍측GCAA조발생회축비례저우단측조[15％(3/20)비36％(10/28)].량조환인수루관상동맥전귀차이무통계학의의(P＞0.05).(4)48례환인중엄중결혈성심기병6례,기중사망2례.(5)CTA발현관상동맥관벽증후、부벽혈전급개화개변,CAG가현시관상동맥폐새、궤화재통급측지혈관형성.결론 천기병합병GCAA호발우1세이하영인,우기6개월이하영인.수방시간증가,GCAA발생협착적비례명현승고.쌍측GCAA발생결혈성심기병화사망적비례고우단측.초성심동도、CTA화CAG결합응용,대발현관상동맥혈전、협착급폐새유중요방조.
Objective To evaluate the prognosis and clinical features of patients with giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD).Method KD complicated with GCAA was diagnosed in 55 patients between January 2003 and December 2012 in Guangzhou Women and Children's Medical Center.Of the 55 patients,48 were studied (43 boys,5 girls).According to the follow-up time,these patients were divided into four groups:≤ 1 year follow-up group,＞ 1-3 years follow-up group,＞ 3 -5 years follow-up group,and ＞ 5-10 years follow-up group.These patients were investigated at the acute stage,and were followed up respectively at two weeks,one month,three months,six months,and one year after hospitalization and every 3 to 6 months after a year.All patients regularly accepted echocardiography and electrocardiographic examination,while some cases accepted CT coronary angiography (CTA)examination and coronary angiography (CAG) examination to confirm the condition of GCAA.Result (1)The age of 48 patients ranged from 2 months to 10 years.Twenty cases were less than 1 age (42％),30 cases were under 3 years of age (62％) and follow-up time was (4.0 ±3.1) years.Age at endpoint was 1.5 to 19 years,mean (6.8 ±4.2) years respectively.(2) In ≤1 year follow-up group,the proportions of no significant changes,retraction,and stenosis were 48％,48％ and 4％ respectively.In ＞ 1-3 years follow-up group,the proportions of no significant changes,retraction,and stenosis were 39％,39％ and 22％ respectively.In ＞ 3-5 years follow-up group,the proportions of no significant changes,retraction,and stenosis were 30％,35％ and 35％ respectively.In ＞5-10 years follow-up group,the proportions of no significant changes,retraction,stenosis were 30％,20％ and 50％ respectively.Compared with the ≤ 1 year group,a significant increase in the proportion of coronary artery stenosis occurred at the other three groups.Significant difference in the stenosis rate could be found between the ≤ 1 year group and the other three groups respectively (x2 =6.026,11.121,15.652 ; P =0.019,0.002,0.001).(3) The rate of retraction in bilateral GCAA group(20 cases)was lower than unilateral GCAA group (28 cases) ; bilateral GCAA group had lower rate than the unilateral group(15％ (3/20) vs.36％ (10/28)).There was no significant difference in coronary artery outcome between two groups (P ＞ 0.05).(4) There were six severe ischemic heart disease in 48 cases including 2 deaths.(5) CTA showed coronary artery wall thickening,mural thrombus and calcification,the CAG could display coronary artery occlusion and recanalization and collateral vessels formation.Conclusion KD complicated with GCAA may occur in infants under one year of age,especially infants under 6 months of age.A significant increase in the proportion of coronary artery stenosis occurred with the follow-up time extended.The proportion of bilateral GCAA patients who had ischemic heart disease and died was higher than the unilateral cases.The combined application of echocardiography,CTA and CAG may greatly help to discover coronary thrombosis,stenosis and occlusion.