摘要目的 探讨川崎病(KD)导致的巨大冠状动脉瘤(GCAA)的中远期变化特点,为此类儿童进入青少年期后心血管意外事件的防治提供依据.方法 采用冠状动脉造影(CAG)、超声心动图及心电图检查对12例急性期合并GCAA的KD患儿[男10例,女2例；发病年龄1～10(4.05±2.50)岁]进行2～10年追踪观察,分析该组患儿冠状动脉损害的变化特点.结果 本组患儿除2例急性期超声心动图诊断为单发右冠状动脉巨大瘤外,其余10例均为双侧多发冠状动脉瘤.随访CAG发现8例出现右冠状动脉节段性狭窄,其中右冠状动脉闭塞并桥样新生血管形成3例,多节段局域性狭窄4例,右冠状动脉全程编织样损害(血栓性闭塞并簇状新生血管)1例.本组9例冠状动脉瘤经治疗后仍有不同程度的存在,其中4例左前降支瘤远端血流充盈明显减慢.仅1例患儿(1/12) CAG见双侧冠状动脉瘤回缩至轻度扩张,未见狭窄及其他改变.本组12例患儿随访2 ～ 10年,均无自觉症状,活动未受限.静息心电图1例发现病理Q波,其余11例结果正常.静息心电图正常的11例患儿行药物负荷心电图检查仅1例出现多导联S-T段下移及T波低平；与同期CAG比较,超声心动图检出12例患儿冠状动脉近段形态改变,5例发现可疑室壁运动异常,但对观察冠状动脉中、远段狭窄欠敏感；对4例冠状动脉闭塞并新生血管形成的病例,超声观察均未能提示.结论 KD引起的GCAA损害可在急性期后长期存在.表现为GCAA持续存在、回缩、冠状动脉闭塞、局部狭窄及血管再形成等.本病缺乏临床表现,常规心电图及超声心动图对KD后遗症期冠状动脉形态及功能损害检查欠敏感,CAG对本病的追踪观察有重要价值.

abstractsObjective To observe the mid-and long-term changes of giant coronary artery aneurysm(GCAA) caused by Kawasaki disease (KD) and to provide preventive basis for cardiac accidents of KD-affected adolescents.Methods Twelve cases of KD with GCAA [10 boys,2 girls;1-10 years old;mean age:(4.05 ±2.50) years old] were followed up by coronary angiography (CAG),echocardiography and electrocardiogram for 2-10 years to and the changes of the coronary artery lesion were analyzed.Results The findings showed that 2 cases were single coronary artery aneurysm and 10 cases had multiple coronary artery aneurysms on both sides by echocardiography.Among them 8 cases showed segmental stenosis in the right coronary artery with varying degrees by coronary angiography,including 3 cases with coronary recanalization after right coronary artery complete occlusion,4 cases with segmental local stenosis,and 1 case with thrombotic occlusion and tufted neovascularization.And in 9 cases of coronary artery aneurysms were still present after treatment,and among them the blood flow filling in 4 cases was slowing down in the left anterior descending arteries.Only 1 case with coronary artery aneurysms on both sides became expanded mildly,without stenosis or other lesions.In this observation,all of the cases had no symptoms or any activity limitation.One case was found pathologic Q wave and the other 11 cases left normal by resting electrocardiogram.The results of resting electrocardiogram in 11 cases were normal by pharmacologic stress electrocardiogram except that 1 case showed multi-lead S-T segment depression and T wave flat.Compared with coronary angiography in the same period,12 cases showed proximal morphological changes in coronary artery and 5 cases with wall motion abnormalities by echocardiography.This study showed that echocardiography was not sensitive enough for mid-and distal-stenosis examination because it failed to provide any useful information for 4 cases affected by segmental stenosis and neovascularization.Condusions The GCAA caused by KD will be lasting for a long time after acute stage,including persisting GCAA,recovering GCAA,occlusion,stenosis and neovascularization.The sensitivity of echocardiography and electrocardiogram are not enough to find coronary morphology and function of KD sequelae damages.CAG is important for follow-up observations of KD with GCAA.