彩色多普勒超声诊断川崎病冠状动脉损害及远期随访

目的 探讨川崎病（KD）患儿冠状动脉损害的发生及恢复期变化特点。方法 采用彩色多普勒超声心动图检查川崎病患儿388例,对伴冠状动脉损害者进行远期随访。结果 冠状动脉损害168例（43.3%）,随访6个月-5年,超声心动图显示冠状动脉扩张组106例冠状动脉全部恢复正常;中小型冠状动脉瘤组82.4%（42/51例）冠状动脉恢复正常;巨大型冠状动脉瘤组11例无一恢复正常。不同年龄组间冠状动脉损害率差异有统计学意义（P〈0.05）。结论 川崎病急性期冠状动脉扩张多可恢复;大部分中小型冠状动脉瘤改变可恢复;但巨大型冠状动脉瘤可长期存在。〈1岁年龄组冠状动脉瘤发生率较高。     

冠状动脉造影和三磷酸腺苷负荷超声心动图对川崎病冠状动脉损害远期追踪的价值

目的 探讨选择性冠状动脉造影及三磷酸腺苷负荷超声心动图对川崎病（KD）冠状动脉损害远期追踪的价值。方法 选取1999至2007年广州市儿童医院KD急性期超声心动图检查发现合并冠状动脉损害的患儿，同时采用三磷酸腺苷负荷超声心动图及选择性冠状动脉造影于恢复期进行远期随访。结果依据纳入标准和排除标准逐层筛选，确定9例KD患儿为本研究的样本。男8例，女1例，急性期发病年龄为1～10岁，平均（4.44±3.09）岁。检查时年龄4～12岁，平均（7.89±2.62）岁。追踪时间1.5~7年，平均（3.44±1.67）年。急性期超声心动图示9例患儿中冠状动脉巨瘤5例，冠状动脉瘤3例，冠状动脉扩张1例。三磷酸腺苷负荷超声心动图示：节段性室壁运动异常6/9例；冠状动脉血流储备下降5/9例。冠状动脉造影示：双侧主干冠状动脉瘤4例，同时伴远端动脉瘤3处，冠状动脉左前降支狭窄1处，并冠状动脉扭曲，狭窄或充盈缺损3处，附近侧支血管形成1处；左前降支动脉瘤、右冠状动脉闭塞伴侧支循环形成1例，冠状动脉扩张4例。与同期超声心动图检查比较 ，冠状动脉造影新发现冠状动脉远端瘤3处，右冠状动脉远端狭窄2处，左前降支狭窄1处，右冠状动脉闭塞伴侧支血管形成1例。5例三磷酸腺苷负荷超声心动图检查结果阳性者与冠状动脉造影比较：均发现相应冠状动脉支狭窄或充盈缺损表现；1例三磷酸腺苷负荷超声心动图检查结果阳性，冠状动脉造影仅见轻度冠状动脉扩张。结论 KD合并冠状动脉损害特别是动脉瘤造成的心脏损害可长期存在。对于远期追踪观察KD患儿冠状动脉病变，三磷酸腺苷负荷超声心动图具无创、安全可靠的优点，是判断心肌缺血的重要方法；选择性冠状动脉造影可明确显示冠状动脉病变的位置、形态、数目及严重程度，特别对冠状动脉狭窄、闭塞及远端病变能做出准确的评估。两种方法结合检查对KD冠状动脉损害的远期追踪有重要帮助。     

放射性核素心肌灌注显像评价川崎病恢复期心肌缺血的意义

目的探讨放射性核素锝甲氧基异丁基异腈(99Tcm-MIBI)心肌灌注显像评价川崎病(KD)恢复期心肌缺血的意义。方法对21例KD恢复期患儿行超声心动图检查。根据超声检查结果分为冠状动脉扩张和无冠状动脉扩张组。各组均行双嘧达莫负荷心肌灌注显像,显像阳性病例隔日再次接受静息心肌显像。结果21例KD恢复期中9例阳性,阳性率为42.8%。超声心动图发现4例冠状动脉扩张,单光子发射计算断层显像仪(SPECT)显像均显示心肌缺血。17例无冠脉扩张患者SPECT显像5例缺血,阳性率29.4%。结论99Tcm-MIBI负荷心肌灌注显像较超声心动图更能客观评价心肌缺血部位、范围和程度,可作为KD随访过程中判断心肌缺血的常规检查方法。     

超声心动图和64层螺旋CT冠状动脉成像在川崎病冠状动脉瘤随访中的应用价值

目的探讨超声心动图和64层螺旋CT冠状动脉成像（64SCTCA）在川崎病（KD）冠状动脉瘤临床诊断和随访中的应用价值。方法选择2006年12 月至2007年5月在首都医科大学附属北京儿童医院门诊随访的KD并发冠状动脉瘤患儿作为研究对象。患儿观察终点随访时依据病变程度除行超声 心动图检查外,同时行64SCTCA检查,对各项检查结果进行详细描述,并分析其应用价值。结果研究期间纳入15例患儿,急性期KD超声心动图检 查显示急性期累及冠状动脉48/60支（80％）。患儿观察终点随访9个月至8.6年,平均（2.8±2.0）年,超声心动图示14/48支(29.2％)冠状动 脉瘤或扩张消退,其余进一步扩张或无明显变化,累及冠状动脉34/60支（56.7%）,同时检测出2处血栓,未发现狭窄及钙化。64SCTCA示累及 冠状动脉30/60支（50％）,主要累及右冠状动脉和左前降支,分布部位与超声心动图检查基本一致,64SCTCA与超声心动图检查对冠状动脉瘤 最大内径的测量值具有较好的相关性（r =0.837,P<0.001）,64SCTCA发现4处血栓、5处钙化及3处狭窄。结论64SCTCA和超声心动图在KD并发 冠状动脉瘤患儿的随访中具有各自的应用价值,两种方法的联合应用可增加冠状动脉病变的检出率。     

川崎病并发巨大冠状动脉瘤的远期预后研究

目的探讨川崎病(KD)并发GCAA患儿的远期预后,为进一步探索KD引起心血管损害后期的诊断治疗提供依据。方法选取2001年5月至2006年5月在广州市妇女儿童医疗中心儿童医院诊断为KD并发冠状动脉损害的患儿为研究对象。依据冠状动脉损害严重程度分为冠状动脉扩张(CAD)组、中小冠状动脉瘤(CAA)组和巨大冠状动脉瘤(GCAA)组。随访起止时间为急性期出院后至2009年5月。所有纳入随访的患儿均于急性期出院后6个月、1年、2年和3年各随访超声心动图1次,观察冠状动脉形态;对急性期3个月后常规超声心动图提示冠状动脉形态未恢复患儿行ATP负荷超声心动图及冠状动脉造影检查,观察心脏室壁运动、左右冠状动脉血流储备和冠状动脉分支病变情况。死亡患儿行尸解及冠状动脉病理学检查。结果CAD组纳入166例,中小CAA组纳入59例,GCAA组纳入15例。①随访3年时超声心动图显示GCAA组2/15例(13.3%)恢复正常,显著低于CAD组和中小CAA组(P均0.001);GCAA组1例(6.7%)显示冠状动脉扩张;10例(66.7%)表现为CAA,其中3例为GCAA,3例合并冠状动脉狭窄或闭塞。②ATP负荷超声心动图显示,GCAA组室壁运动异常的发生率显著高于CAD组(6/9例vs8/55例,P0.001),与CAA组差异无统计学意义(6/9例vs17/31例,P0.05);非参数检验提示,3组间室壁运动异常发生率有统计学差异,以GCAA组发生率较高(Z=-4.2,P0.001)。③GCAA组6/15例行冠状动脉造影,4/6例仍存在CAA,1/6例GCAA退缩为轻度CAD。5/6例出现冠状动脉狭窄或(和)闭塞,其中1例闭塞后再通,1例合并侧支血管形成。④GCAA组死亡2例,尸解冠状动脉大体可见,左右冠状动脉各分支呈串珠样膨大,瘤样扩张的冠状动脉管腔内可见充满暗红色血栓样物质,管壁显著增厚。冠状动脉分支和前降支病理学检查均显示:内膜增厚,炎症细胞浸润。结论KD并发GCAA患儿的预后较差,冠状动脉损害可持续存在;后期可发生冠状动脉狭窄或闭塞,导致缺血性心脏病;常规超声心动图与ATP负荷超声心动图、冠状动脉造影结合应用,对KD并发GCAA的远期随访有重要意义。     

单中心9年不完全川崎病回顾性分析

目的 探讨不完全川崎病（KD）的临床特征,以提高临床诊治水平。方法 回顾性分析2002年1月至2010年12月KD住院患儿的临床资料,比较不完全KD与典型KD在发病年龄、性别、临床表现、实验室检查、治疗及冠状动脉损害等方面的差异。结果 1 484例KD患儿进入分析,其中不完全KD 262例(17.6%),典型KD 1 222例;<1岁患儿中不完全KD占24.9%。不完全KD和典型KD患儿的平均发热时间分别为（7.8±5.0）和（6.7±3.6）d,差异有统计学意义。不完全KD 四肢改变、多形皮疹、眼结膜充血、口唇改变、颈部淋巴结肿大和肛周改变的发生率显著高于典型KD;卡疤改变、扁桃体肿大、阴囊或外阴改变、呕吐和腹泻的发生率与典型KD差异无统计学意义。两组CRP、ESR、Hb、WBC、PLT、ALT、AST、CK-MB和LDH等实验室指标差异无统计学意义。不完全KD与典型KD患儿对IVIG无反应的发生率差异无统计学意义（14.1％ vs 17.5％）;不完全KD患儿冠状动脉扩张、冠状动脉瘤和巨大冠状动脉瘤的发生率分别为57.5%、14.1％和1.9%,典型KD患儿分别为31.5%、5.9% 和0.6%,两组差异有统计学意义。结论 不完全KD较典型KD发热时间长,且冠状动脉损害发生率高,但实验室指标差别不大。     

冠状动脉造影追踪观察川崎病引起的巨大冠状动脉瘤

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.     

川崎病患儿冠状动脉血流储备测定与影像形态学诊断的对照研究

目的评价持续冠状动脉病变对川崎病（KD）患儿冠状动脉血液动力学的影响, 探讨冠状动脉血流储备（CFR）检测在KD患儿冠状动脉病变远期随访中的应用价值。方法选择因胸痛、心悸以及各类偶发早搏的患儿为对照组，KD并发持续左冠状动脉病变患儿为KD组，均予以ATP 160 μg· kg-1·min-1持续静脉注射，共6 min。运用经胸多普勒超声心动图（TTDE）检测冠状动脉左前降支(LAD) 在ATP注射前后的舒张期峰值流速(PDV)及平均流速(MDV)，并通过计算比值得出相应CFR（PDV）和CFR（MDV）值。结果 对照组纳入25例，CFR（PDV）和CFR（MDV）值均与年龄呈显著正相关(r=0.596, 0.591, P=0.002, 0.002)；<2岁、～8岁和>8岁组的CFR (PDV) 95%参考值范围分别为≥1.22、≥1.94和≥2.22。KD组纳入8例，静息状态下PDV和MDV值分别为（50.0±15.07） cm· s-1和(35.16±11.52) cm· s-1，显著高于对照组(P=0.004, 0.015)；而CFR（PDV）和CFR（MDV）值明显降低，分别为（1.67±0.38） cm· s-1和（1.69±0.47） cm· s-1(P=0.002,0.018)。与对照组比较，KD组7/8例(87.5%)患儿CFR（PDV）值显著降低。结论持续冠状动脉病变可导致KD患儿CFR值显著降低，TTDE检测患儿CFR具无创、安全和便捷的优点，对于KD冠状动脉病变的长期随访与临床诊断治疗策略的选择具有指导意义。     

川崎病并发冠状动脉病变的超声心动图表现的动态观察

目的 探讨川崎病(KD)所引起的冠状动脉扩张(CAD)以及冠状动脉瘤(CAA)的超声心动图特点.方法 用彩色多普勒超声心动图检测KD患儿冠状动脉开口和内径,并动态观察静脉免疫球蛋白治疗前后冠状动脉内径的变化情况.结果 本组46例KD患儿,41例患儿行超声心动图检查,合并CAD 12例,左冠状动脉(LCA)较右冠状动脉(RCA)更易受累及(P<0.05);CAA 4例,均为双侧冠状动脉病变,最大内径10 mm.治疗后6～18 d复查超声心动图,CAD组LCA较治疗前明显回缩(P<0.05),RCA内径无变化(P>0.05);CAA组LCA和RCA内径均无明显变化(P>0.05).对10例冠状动脉病变患儿进行随访,其中8例CAD冠状动脉内径均恢复正常,1例CAA冠状动脉病变加重,1例CAA完全恢复正常.结论 (1)在KD所引起的CAD中,LCA比RCA更易受累及,静脉免疫球蛋白治疗后短期内LCA明显回缩.(2)超声心动图是动态观察KD并发CAD和CAA的无创手段.     

氯吡格雷联合阿司匹林对川崎病并发中小型冠状动脉瘤患儿抗血栓治疗的疗效及安全性研究

目的 探讨川崎病（KD）合并冠状动脉瘤（CAA）患儿使用氯吡格雷联合阿司匹林抗血栓治疗的疗效及安全性。方法 选取2013年1月至2018年6月经超声心动图诊断为合并多发中小型CAA的KD患儿77例为研究对象,随机分为观察组（n=38）和对照组（n=39）,分别接受氯吡格雷+阿司匹林和低分子肝素+阿司匹林治疗。所有患儿定期随访,以病程前3个月为观察期,总结两组患儿的冠状动脉变化及其他并发症发生情况。结果 随访至3个月时,观察组6例冠状动脉恢复正常,11例冠状动脉较前回缩,19例冠状动脉稳定,2例发展为巨大冠状动脉瘤;对照组7例冠状动脉恢复正常,12例冠状动脉较前回缩,19例冠状动脉稳定,1例发展为巨大冠状动脉瘤;两组冠状动脉变化情况比较差异无统计学意义（P > 0.05）。观察组合并鼻衄2例,皮肤瘀斑6例;对照组合并鼻衄1例,出现注射部位瘀点瘀斑7例;两组均未出现其他严重出血事件。结论 氯吡格雷联合小剂量阿司匹林对KD合并CAA患儿的抗血栓治疗安全有效。     

超声心动图探查川崎病冠状动脉病变259例

目的 探讨超声心动图在诊断及随访川崎病冠状动脉病变中作用。方法 超声心动图探查小儿川崎病259例,对其中97例进行了随访。结果 发现冠状动脉病变48例(18.5％),90％是在病程3周内出现,左冠状动脉比右冠状动脉更易发生扩张性病变。随访表明轻度扩张性病变较易恢复正常,2例冠状动脉瘤持续存在,动脉瘤内径大小是影响预后的主要因素。结论 超声心动图是诊断川崎病并发冠状动脉病变安全和准确的方法。     

Re-treatment for immune globulin-resistant Kawasaki disease: A comparative study of additional immune globulin and steroid pulse therapy

BACKGROUND: We compared the efficacy and safety of additional intravenous immune globulin (IVIG) therapy with steroid pulse therapy in patients with IVIG-resistant Kawasaki disease. METHODS: Two-hundred and sixty-two consecutive patients had been treated with a single dose of IVIG (2 g/kg) and aspirin (30 mg/kg per day). Thirty-five patients (13.4%) were not clinical responders to the initial IVIG treatment. They received an additional IVIG treatment (1 g/kg) within 48 h after the initial treatment. Seventeen patients (6.5%) did not respond to the additional IVIG treatment. We randomly divided these patients into two groups: group 1 consisted of eight patients who were treated with a single additional dose of IVIG (1 g/kg), while group 2 consisted of nine patients who were treated with steroid pulse therapy. RESULTS: The IVIG-resistant patients had a high incidence of coronary artery lesions (CAL; 48.6%). Five patients (62.5%) in group 1 had CAL, including two patients who each had a giant aneurysm and three patients who each had a small aneurysm. Seven patients (77.8%) in group 2 had CAL, including two patients who each had a giant aneurysm, two patients who each had a small coronary aneurysm and three patients who each showed transient dilatation during steroid pulse therapy. There was no significant difference in the incidence of CAL between the two groups. The duration of high fever in group 2 (1.4~0.7 days) was significantly shorter than in group 1 (4.8~3.4 days; P<0.05). The medical costs for the treatment of patients in group 2 (113, 012 yen +/- 22,084) were significantly lower than those for group 1 (144,194 yen +/- 12,914; P<0.05). CONCLUSIONS: Steroid pulse therapy may be useful in the treatment of patients with IVIG-resistant Kawasaki disease who experience prolonged fever. However, transient dilatation of the coronary artery is observed during steroid pulse therapy, so careful echocardiographic examination should be performed for those patients receiving steroid pulse therapy for the sake of early detection of coronary artery abnormalities.     

川崎病并冠状动脉损害的危险因素

目的探讨川崎病(KD)并冠状动脉损害(CAL)的危险因素。方法收集1999年1月-2001年12月住院的145例KD患儿的临床资料,分析无CAL93例和CAL52例患儿的实验室检查资料及治疗、随访结果。结果无CAL组发热日程(8.7±3.4)d.血沉(79.5±34.6)mm/1h,CAL组发热日程(11.5±4.8)d,血沉(91.9±36.6)mm/1h,两组比较差异有显著性(P<0.01,0.05)。发病10d内接受静脉注射人血丙种球蛋白(IVIG)治疗患儿CAL发生率为18.8%(18/96),未予IVIG治疗患儿CAL发生率为69.4%(34/49),两者比较差异有显著性(P<0.001)。随访并CAL患儿38例,冠状动脉扩张20例均恢复正常;14例冠状动脉瘤中12例恢复正常,2例缩小;4例巨大冠状动脉瘤2例缩小,另2例超过2年仍未恢复。结论KD并CAL与热程过长及血沉显著增快密切相关,病程早期未予IVIG治疗的患儿并CAL的危险性明显增高,应用IVIG治疗对防治冠状动脉损害有重要作用。     

Prevalence of coronary artery lesions on the initial echocardiogram in Kawasaki syndrome

OBJECTIVES: To determine whether coronary artery lesions (ectasia and aneurysm) are commonly observed on the initial echocardiogram of patients with acute Kawasaki syndrome, whether coronary artery ectasia and/or aneurysms occur more frequently in patients with incomplete Kawasaki syndrome than in those patients with complete findings, and whether earlier diagnosis and treatment of Kawasaki syndrome are associated with less frequent occurrence of coronary artery ectasia and/or aneurysm. DESIGN: A retrospective medical record review. SETTING: A tertiary care pediatric hospital. PARTICIPANTS: One hundred patients treated for Kawasaki syndrome between July 1, 1998, and June 30, 2003, who were identified by a medical record search. MAIN OUTCOME MEASURE: Prevalence of coronary artery lesions (ectasia and aneurysm) on the initial and subsequent echocardiograms. RESULTS: Forty-four percent of patients had a coronary artery lesion (31% with ectasia, 13% with aneurysm) on the initial echocardiogram. Patients with incomplete Kawasaki syndrome were treated significantly later (median, 10 days) and had a significantly higher occurrence of coronary artery aneurysms over the course of their illness (37%) than those with complete Kawasaki syndrome, who were treated at a median of 7 days (P<.001) and had a 12% aneurysm occurrence (P = .009). Patients treated by day 7 of illness had a less frequent occurrence of aneurysm (6%) compared with those patients treated between days 8 and 10 of illness (27%) (P = .03). CONCLUSIONS: Coronary artery lesions are frequently detected on the initial echocardiogram of children with Kawasaki syndrome. If future studies show ectasia to have a relatively high degree of specificity for Kawasaki syndrome, the initial echocardiography may be a useful adjunctive diagnostic test.     

川崎病冠状动脉损害超声心动图及心电图分析

目的　探讨超声心动图与心电图对川崎病冠状动脉损害的诊断价值。方法　对川崎病患儿 78例行超声心动图及心电图检查 ,根据超声检查结果分为 3组 ,并对各组进行分析。结果　超声检出冠状动脉病变 45例 ,其中冠状动脉扩张 32例 ,冠状动脉瘤 1 3例 ;心电图异常 1 6例。冠状动脉损害组心电图异常率高于无冠状动脉损害组 (P <0 .0 1 ) ;冠状动脉瘤组心电图异常率明显高于冠状动脉扩张组 (P <0 .0 0 5)。结论　超声心动图与心电图相结合可更全面地反映川崎病的冠状动脉损害。     

Long-term prognosis of giant coronary aneurysm in Kawasaki disease: an angiographic study

The incidence of coronary obstruction subsequent to giant coronary aneurysm in Kawasaki disease was studied. In 20 cases, aneurysms with a maximal diameter greater than 8 mm were identified by coronary angiography 2 to 120 months (mean 16.9 months) after onset. There were 25 giant aneurysms among these 20 patients, all of whom underwent coronary angiography between 12 and 134 months (mean 31.7 months) after initial examination. Coronary obstruction occurred in six cases (30.0%), all within 4 years of onset of disease. There were five obstructive aneurysms in the right coronary artery (5/12; 41.7%) and two in the left coronary artery (2/13; 15.4%). One of these patients developed symptomatic myocardial infarction. Two had abnormal electrocardiographic findings suggesting myocardial infarction. In five cases, persistent perfusion defects were found by myocardial imaging. On the other hand, in two patients giant aneurysms persisted without obstructive changes for greater than 10 years. In both cases the aneurysm was in the left coronary artery and obstruction of the right coronary artery was found at initial angiography. These results seem to indicate the limited efficacy of antiplatelet therapy for giant coronary aneurysms in Kawasaki disease and that giant aneurysms are likely to progress to become obstructive within a few years even if antiplatelet therapy is given. Other forms of treatment, including surgery, should therefore be considered in such patients.     

Coronary arterial lesions of Kawasaki disease: Cardiac catheterization findings of 1100 cases

Summary In our institute, 1100 patients with a history of Kawasaki disease have been catheterized for selective coronary arteriography. Their age at examination ranged from four months to 13 years. Coronary artery lesions (CAL) were found in 262 patients. As far as the type of the CAL was concerned, occlusion was noted in 20 (7.6%), segmental stenosis in 15 (5.7%), localized stenosis in 62 (23.7%), aneurysm in 93 (35.5%), and dilatation in 72 patients (27.5%). In terms of the total number of lesions, there were 23 occlusions, 19 segmental stenoses, 109 localized stenoses, 449 aneurysms and 307 dilatations. The 262 patients with CAL were analyzed according to the interval from the onset to the time of selective coronary arteriography. The incidence of both occlusion and segmental stenosis was lowest in the group who were catheterized shortly after the onset of disease, whereas the prevalence of aneurysm was highest in this group. But the prevalence of dilatation was highest in the group of patients who were catheterized late. A total of 12 patients had to undergo femoral arterial thrombectomy for arterial thrombosis following the catheterization, but no other major complication was experienced.