Strategy for Localized Stenosis Caused by Kawasaki Disease: Midterm Results of Percutaneous Transluminal Coronary Balloon Angioplasty in Two Infants

We report the midterm results of percutaneous transluminal coronary balloon angioplasty (PCBA) for localized stenosis (LS) caused by Kawasaki disease (KD). Two 2-year-olds, a girl and a boy, underwent PCBA for severe LS of the left anterior descending artery caused by KD. After 4 years, there was no ischemia and no significant restenosis in the target vessels. PCBA provided a good result for early appearing LS caused by KD in two small infants. The 5-years-old boy was the youngest patient to undergo percutaneous transluminal coronary rotablator. Although the postprocedure result was very effective, 1 year later asymptomatic occlusion occurred.     

川崎病所致严重冠状动脉病变单中心队列研究

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。     

儿童冠状动脉疾病32例临床分析

目的探讨儿童冠状动脉疾病的病因分布及临床特点,以提高诊断和治疗儿童冠状动脉疾病水平。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院儿童心脏中心收治的冠状动脉疾病患儿（不包括复杂心脏畸形合并冠状动脉异常者）的临床资料,分析临床表现、辅助检查、诊断方法和治疗。结果32例冠状动脉疾病患儿进入分析,男20例,女12例,年龄1个月至18岁。经ECG、超声心动图、64排CT和心导管造影检查明确诊断。左冠状动脉异常起源于肺动脉10例(31.2%),8例以心力衰竭起病,其中长期误诊为“心内膜弹力纤维增生症”4例,“扩张性心肌病”1例,“二尖瓣重度关闭不全”2例,均行外科手术移植左冠状动脉,1例术后死亡,余9例术后随访临床症状好转,左室缩小,二尖瓣反流减轻;冠状动脉瘘13例(40.6%),9例以心脏杂音、4例以心力衰竭起病,外科手术行瘘缝扎或补片治疗12例,介入治疗1例,术后随访仅1例存在少量残余分流;川崎病并发冠状动脉瘤6例(18.8%),出现左心扩大、心肌缺血征象,予长期抗凝等治疗;家族性高胆固醇血症3例(9.4%),表现为冠状动脉粥样硬化性心脏病,予降胆固醇、改善心肌缺血等药物及饮食控制治疗,随访病情尚稳定,无明显加重。结论儿童冠状动脉疾病少见,先天性包括左冠状动脉异常起源于肺动脉和冠状动脉瘘,后天性以高胆固醇血症和川崎病累及冠状动脉多见,对于有左心扩大和心肌缺血表现者,应重视冠状动脉疾病的可能,早期诊断、合理治疗是改善预后的关键。     

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。     

Coronary diameter in normal infants, children and patients with Kawasaki disease

BACKGROUND: The coronary assessments in Kawasaki disease are mainly based on the Japanese Ministry of Health and Welfare criteria, which is simply classified according to the patient's age, over 5 years and less than 4-years-old. METHODS: We obtained normal values of coronary diameters adjusted for the body surface area and for the coronary anatomical site from 71 healthy children using 2-D echocardiography. We also studied patients with Kawasaki disease at three stages from the onset of illness: (i) 43 patients at admission; (ii) the subsequent 2-3 weeks; and (iii) 62 children followed at a clinic, for a median 2.2 years after the onset. No patients showed any coronary abnormalities by several echographic exams. RESULTS: The coronary diameters were strongly correlated with the body surface area (r = 0.81 in left main, r = 0.89 in proximal right, r = 0.89 in left anterior descending artery). The coronary diameters in the patient groups at admission and at 2-3 weeks later were significantly larger than those in the normal group (P < 0.01). Although the coronary diameters in the follow-up group did not show a significant difference compared with those of normal, 19% retained their coronary diameters at greater than two standard deviations above the expected mean in at least one coronary artery. CONCLUSIONS: The more strictly defined criteria adjusted for body size and for the anatomical site should be used to detect the subtle changes and to prevent the misclassification of the coronary artery abnormalities in KD.     

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??Objective To analyze the clinical characteristics of patients with CAL complicated by KD??and to improve the understanding of this disease. Methods Clinical data of 19 cases diagnosed as KD complicated by CAL from September 2008 to August 2015 in the Pediatric Department of First Hospital of China Medical University were retrospectively analyzed. Results KD children complicated with CAL was more in male than in female??male??female was 3.75??1??children with coronary aneurysm were more in male than in female??male??female was 8??1??it was more often in less than 1-year old children??accounting for 36.8%. The incidence rate of coronary artery lesions in this group was 16.4%??CAD was in 10 cases??CAA in 5 cases??GCAA in 4 cases??the incidence rate being 8.6%??4.3% and 3.5%??respectively??in terms of CAL time 73.7% was 2-3 weeks after the disease?? according to the number of branches involved??both coronary artery involved accounted for 68.4%??and according to the location involved??right coronary artery involvement accounted for 41.5%??left main coronary artery involvement accounted for 39.0%??the left anterior descending coronary artery involvement accounted for 17.1%??and left circumflex artery involvement accounted for 2.4%. The clinical grade of severity in CAL children were???? grade in 5 cases??accounting for 26.3%???? grade in 8 cases??accounting for 42.1%???? grade in 6 cases??accounting for 31.6%. During follow-up??71.4% patients had coronary artery aneurysm or dilatation significantly retracted??the average time of retraction being ??1.5±0.7??years??of which 1 case was completely dissipated. Conclusion The incidence rate of the KD patients with CAL is 16.4%??in which CAD has the highest incidence rate while GCAA the lowest. CAL mostly occurs at 2-3 weeks after the disease. Any branch can be damaged??with the right coronary artery to be most likely involved. As to coronary artery disease severity??grade ?? is of the highest proportion. Most coronary artery aneurysm or dilatation has remarkable retraction within 1-2 years.     

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。     

Emerging role of computed tomography coronary angiography in evaluation of children with Kawasaki disease

Coronary artery abnormalities are the most important complications in children with Kawasaki disease (KD). Two-dimensional transthoracic echocardiography currently is the standard of care for initial evaluation and follow-up of children with KD. However, it has inherent limitations with regard to evaluation of mid and distal coronary arteries and, left circumflex artery and the poor acoustic window in older children often makes evaluation difficult in this age group. Catheter angiography (CA) is invasive, has high radiation exposure and fails to demonstrate abnormalities beyond lumen. The limitations of echocardiography and CA necessitate the use of an imaging modality that overcomes these problems. In recent years advances in computed tomography technology have enabled explicit evaluation of coronary arteries along their entire course including major branches with optimal and acceptable radiation exposure in children. Computed tomography coronary angiography (CTCA) can be performed during acute as well as convalescent phases of KD. It is likely that CTCA may soon be considered the reference standard imaging modality for evaluation of coronary arteries in children with KD.     

川崎病并发严重冠状动脉病变患儿旁路移植术术前评估及术后随访

目的回顾性总结川崎病(KD)并发严重冠状动脉病变患儿的影像学诊断、冠状动脉旁路移植术结果及随访情况。方法复旦大学附属儿科医院2006年8月至2008年3月收治5例(男3例,女2例)KD并发严重冠状动脉病变患儿,年龄12个月至10岁4个月。所有患儿均在KD急性期予IVIG治疗,其中3例予2次。病程第15～21天均行超声心动图(ECHO)检查发现冠状动脉病变;之后常规随访ECG和ECHO,并口服阿司匹林和双嘧达莫或氯吡格雷。1例患儿行64排螺旋CT(MSCT)检查,2例行99Tcm-MIBI心肌灌注显像检查。在病程6～65个月行选择性冠状动脉造影检查,并行冠状动脉旁路移植术及冠状动脉成形术。结果 1/5例在病程中有心绞痛发作,3/5例有心功能不全,1/5例无症状。1/5例有心肌梗死发生并在恢复过程有ECG记录;2/5例ECG有ST-T改变;2/5例ECG未见异常。ECHO除均显示多发性冠状动脉瘤(CAA)外,3/5例还显示左心房和左心室增大,左室射血分数(LVEF)和短轴缩短率的降低,2/5例左室壁运动不协调,与99Tcm-MIBI检查结果一致。冠状动脉造影均可见多发性CAA且至少一处为中等或巨大CAA,并伴有血栓形成和远端闭塞。MSCT与冠状动脉造影检查结果一致。年龄最小的1例患儿(手术时22个月)在冠状动脉移植术中死亡,其余4例术后即刻效果满意。在8～24个月的随访中,1例LVEF较低,随访1年未达到正常;1例术后18个月复发KD,治疗顺利,复查ECHO和MSCT未见冠状动脉进一步损害。4例患儿目前仍在随访中。结论 KD并发冠状动脉病变应定期随访ECG、ECHO,必要时进行99Tcm-MIBI和MSCT检查;如考虑手术需行冠状动脉造影仔细评估冠状动脉病变部位和程度。有心肌缺血表现需及时行冠状动脉旁路移植术和冠状动脉成形术,手术的近期效果显著,远期疗效有待长期随访结果。     

Diagnosis, treatment and outcome of Kawasaki disease in an Australian tertiary setting: a review of three years experience

OBJECTIVE: To examine the patient characteristics, diagnostic criteria, treatment, complications and outcomes of Kawasaki disease (KD) in children diagnosed and treated in an Australian tertiary hospital setting and compare this to previously reported experience. METHODS: Data were collected prospectively for 14 patients in 2002 and retrospectively for 31 patients in 2000 and 2001. Demographics, clinical features, laboratory features, treatment and outcome details were collected, analysed and compared to previous experience. RESULTS: The median age at diagnosis was 2.77 years (0.28-8.84) and diagnosis was made after a mean 6.1 days of fever. Twenty-eight patients (62.2%) had classical KD and those with less than four criteria were younger than those with four or more criteria (P = 0.04). Patients were treated with intravenous immunoglobulin and aspirin. Twelve (26.7%) required re-treatment. Re-treatment was associated with treatment within 5 days of fever onset (P = 0.03). Twelve patients (27.3%) developed coronary artery abnormalities and the development of such abnormalities was associated with lower serum albumin at diagnosis (P = 0.008) and need for re-treatment (P = 0.03). There was no difference in coronary outcome between classical and incomplete presentations. Only two patients had coronary lesions at 12 months follow-up. CONCLUSIONS: We found a trend towards earlier diagnosis and treatment of KD in the tertiary hospital setting, which may reflect heightened physician awareness of the disease. Our study confirms that children who present with incomplete disease are at risk of coronary artery abnormality. We recommend that clinicians continue to maintain high levels of suspicion, even in the absence of the complete clinical picture of KD, particularly in infants below 1 year of age.     

川崎病合并冠状动脉瘤63例临床分析

目的分析川崎病(KD)合并冠状动脉(以下简称冠脉)瘤患儿的临床特点。方法对首都医科大学附属北京儿童医院2000—2007年收治的63例超声心动图诊断为冠脉瘤的KD患儿临床资料、实验室检查、超声及心电图检查结果、治疗情况及随诊资料进行回顾性分析。结果(1)冠脉瘤患儿男性明显多于女性,男∶女为5.3∶1;冠脉巨大瘤男女比例为8.3∶1;<1岁患儿多发,占28.6%。(2)本组患儿中不完全KD、静脉注射丙种球蛋白(IVIG)抵抗以及KD复发的发生率均较高,分别为36.5%、30.2%和7.9%;急性期57例(90.5%)患儿使用IVIG冲击治疗,3例未用,3例使用情况不详;36例(57.1%)患儿发病至丙种球蛋白应用的时间间隔大于10 d。(3)超声检查发现小冠脉瘤患儿7例,中等冠脉瘤19例,巨大瘤37例,左冠脉受累者占76.2%,其中58.3%发生在前降支;右冠脉受累者达87.3%,其中47.3%发生在右冠Ⅱ段;双侧冠脉同时受累者占63.5%。(4)随诊发现71.4%冠脉瘤呈现回缩趋势,45.2%的受累分支冠脉瘤消退,平均消退时间为(2.1±1.5)年。结论对于男性、发病年龄<1岁、不完全KD、发生IVIG抵抗、复发患儿及应用IVIG治疗较晚患儿要警惕冠脉瘤的发生;左冠前降支及右冠脉瘤样病变最多见,多数冠脉瘤在恢复期发生回缩。     

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??ObjectiveTo investigate the clinical characteristics and prognosis of coronary aneurysm in children with Kawasaki disease.MethodsCoronary aneurysm was identified by using echocardiography in 63 children with Kawasaki disease from 2000 to 2007.Their gender,age,clinical presentations,use of intravenous gamma immunoglobulin (IVIG),laboratory study results and echocardiographic study results in the acute and convalescent phase were examined retrospectively.Results(1) Boys and infants were more likely to develop coronary aneurysms,the male to female ratio being 5.3??1,and for patients with giant aneurysms the male to female ratio was 8.3??1.(2)The rate of incomplete KD,IVIG non-responders and recurrence in this group of patients were 36.5%,30.2% and 7.9%,respectively,which were higher than the whole KD group.In the acute phase,57 of 63 patients were treated by IVIG with different doses,3 of them did not receive IVIG treatment and the other 3 patients?? IVIG treatments were unknown.The duration from onset to IVIG treatment was longer than 10 days in 36 of the 63 (57.1%) children.(3) Small aneurysms were detected by 2-D echo in 7 patients,medium aneurysms in 19 patients and giant aneurysm in 37 patients.In all of these patients,76.2% of them had left coronary artery aneurysms,and 87.3% of them had right coronary artery aneurysms;47.3% of them had both left and right aneurysms.In the patients with left coronary aneurysms,58.3% of them have left anterior descending branch aneurysms; and in patients with right coronary aneurysms,47.3% of them were located in the right coronary artery segment II.(4) Coronary aneurysm regressed in 71.4 % of the branches.The lumen diameter of the affected branches returned to normal in 45.2% of them,with a mean regression time of 2.1??1.5 years.ConclusionBeing male,younger than 1 year old,incomplete KD,IVIG non-responder,recurrence and later IVIG treatment are possible risk factors of coronary aneurysms in children with Kawasaki disease.Most children have multi-coronary artery aneurysms and left anterior descending branch and right coronary artery are the most commonly affected branches??and most of the coronary aneurysms have the tendency to regress during the convalescent phase.     

Long-term outcome of coronary abnormalities in patients after Kawasaki disease

From January 1973 through December 1992, a total of 302 patients (183 males, 119 females) with Kawasaki disease (KD) underwent coronary angiography. The age at onset of KD ranged from 2 months to 12.3 years (median 1.4 years). The age at the first angiographic evaluation ranged from 6 months to 17 years (median 3.5 years). Most of the patients (85%) had suffered from KD before 1985 and thus were treated without benefit of γ-globulin. Follow-up varied from 6 months to 25.8 years (median 13.6 years). Coronary abnormalities were confirmed in 71 (23.5%) of 302 patients; the left coronary artery (LCA) alone was involved in 36 cases, the right coronary artery (RCA) alone in 10 cases, and both arteries in 25 cases. Serial angiographic evaluation of the 42 cases revealed different attitudes in the progress of coronary abnormalities. All large aneurysms showed a tendency to regress, although some progressed to stenotic lesions. Moderate aneurysms stayed unchanged, regressed, or progressed to stenosis or obstruction. Small aneurysm never became stenotic and frequently regressed to normal internal diameter. aneurysms of the RCA tended to regress relatively early during the follow-up period, whereas those of the LCA gradually progressed to stenotic lesions. In 7 of 35 patients with RCA lesions, aneurysms progressed to complete obstruction and subsequent recanalization within 0.5 to 7.7 years (median 3.6 years) after the onset of KD. Most of the patients with coronary artery sequelae after KD remain asymptomatic. Serial angiographic observation is indicated for those patients who develop large coronary aneurysms during the acute phase of KD. The standard 12-lead electrocardiogram, chest roentgenogram, and exercise stress test are less sensitive for detecting and evaluating patients with coronary sequelae. For the screening of myocardial ischemia after KD, stress thallium 201 scintigraphy with dipyridamole infusion is recommended.     

川崎病合并冠状动脉损害的远期转归与随访

近年对川崎病的追踪随访发现,大部分患儿预后良好,但仍有少数患儿长期遗留心血管后遗症,可发展为缺血性心脏病,一旦发生心肌梗死,病死率高.因此,美国、日本及我国等均制订了相应的川崎病患儿长期随访方案,应用各种随访手段以观察其冠状动脉损害及转归,预防川崎病引起的心血管意外发生.     

Competition Between Native Flow and Graft Flow After Coronary Artery Bypass Grafting. Impact on Indications for Coronary Artery Bypass Grafting for Localized Stenosis with Giant Aneurysms Due to Kawasaki Disease

We report the postoperative course of native and graft flow after coronary artery bypass grafting (CABG) in two patients with giant aneurysms and localized stenosis due to Kawasaki disease (KD). Although both patients had undergone CABG to the left anterior descending artery (LAD) with the left internal thoracic artery (ITA), at 5 and 10 years old, respectively, the ITA grafts were occluded 1 month postsurgery. However, when the two patients suffered complete occlusion of the native LAD more than 10 years after surgery, angiograms showed that the ITA grafts had reopened. We believe that this postoperative course reflects competition between the native artery flow and graft flow after CABG. CABG in patients with severely delayed coronary flows or recurrence of thrombus in giant aneurysms was ineffective in preventing myocardial infarction or damage. We conclude that CABG in giant aneurysm without significant localized stenosis should be avoided.     

Kawasaki disease with severe cardiac sequelae: lessons from recent New Zealand experience

OBJECTIVES: To review recent cases of Kawasaki disease (KD) with significant cardiac sequelae in New Zealand. It is known that intravenous immunoglobulin (IVIG) reduces the risk of coronary artery aneurysm formation if given within 8-10 days of onset of KD. METHODS: Retrospective review of medical course, criteria for KD, laboratory and cardiac findings for six children identified with KD and significant coronary artery sequelae. RESULTS: There was delay in diagnosis of KD in three of the six children. Three cases were atypical by extremes of age (2 months, 10 years, 14 years). By definition all six children had significant coronary artery involvement. One patient had a thrombus detected in a coronary aneurysm 3 weeks after KD. One patient underwent coronary artery bypass grafting for unstable angina 2 years after KD. One patient developed coronary artery aneurysms after an initial 'toxic shock' type illness evolving to KD. Three patients died, one due to rupture of a coronary artery aneurysm, two from rapid early coronary artery obstruction occurring at three and 4 months after initial KD. CONCLUSIONS: Kawasaki disease remains an important cause of mortality and morbidity for children. Diagnostic delay beyond 8 days reduces the chances of successful IVIG therapy in KD. Current studies supported by the Paediatric Surveillance Unit should establish the epidemiology of KD in New Zealand.     

Congenital coronary artery fistulae: A review of 18 cases with special emphasis on spontaneous closure

Summary Between 1972 and 1990, 18 patients (median age 3 years, range 0.1–14 years) with coronary artery fistulae (CAF) were seen at this institution. Of the 16 patients without associated heart defects, two patients presented with congestive heart failure and the remaining 14 were asymptomatic. Fifteen patients had normal orgin of two coronary arteries. Two patients has atresia of the proximal right coronary artery and, in one patient, the right coronary artery originated from the left main coronary artery. The QP/QS ranged between 1.0 and 2.8, with a mean of 1.4. Fifteen patients underwent operative closure without any deaths. One patient is being followed medically at present. Two patients showed spontaneous clinical improvement of CAF (complete closure in one and near complete in the other) and remained asymptomatic during a decade of follow-up. Review of the literature suggests operative closure of symptomatic and asymptomatic small CAF during childhood. However, the natural history of minute CAF remains unclear. In addition, symptoms from CAF may spontaneously improve with time.     

川崎病合并冠状动脉损害的远期转归与随访

近年对川崎病的追踪随访发现,大部分患儿预后良好,但仍有少数患儿长期遗留心血管后遗症,可发展为缺血性心脏病,一旦发生心肌梗死,病死率高.因此,美国、日本及我国等均制订了相应的川崎病患儿长期随访方案,应用各种随访手段以观察其冠状动脉损害及转归,预防川崎病引起的心血管意外发生.     

Improvement of left ventricular function after ligation of the anomalous left coronary artery during infancy

Summary Considerable controversy surrounds the optimal management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery; this includes the timing and type of surgical intervention. Long-term follow-up was obtained on three patients who had simple ligation of the anomalous left coronary artery at or before 8 months of age; long-term follow-up on an adolescent treated with a saphenous vein bypass graft from the aorta to the coronary artery was available for comparison. Remarkable increase in left ventricular contractility was observed in the three patients treated only by simple ligation of the anomalous left coronary artery during infancy. Thallous chloride T1 201 exercise studies disclosed no perfusion abnormalities in any of the patients despite angiographically demonstrable wall-motion abnormalities in two of the four patients. Our study suggests that simple ligation of the anomalous left coronary artery near its origin in patients with angiographically detectable left-to-right shunting can provide long-term survival with good left ventricular function and minimal clinical complaints.     

Immunohistochemical study of apparently intact coronary artery in a child after Kawasaki disease

BACKGROUND: Coronary arterial lesions (CAL) due to Kawasaki disease (KD) often show progressive intimal hyperplasia even many years after the disease. However, most patients have no CAL after the acute phase, and it is an important issue whether or not coronary arteries without CAL have significant intimal hyperplasia, and whether or not there is the potential for this to progress to stenosis and/or atherosclerosis. METHODS: The authors examined formalin-fixed specimens of the coronary arteries immunohistochemically, using antibodies against vascular growth factors (GFs), the receptors of transforming growth factor-beta (TbetaRs) and inducible nitric oxid synthesis (iNOS) in a KD patient without CAL, and also in four control patients: two with CAL due to KD and two without a history of KD. RESULTS: Vascular endothelial GFs, Platelet-derived growth factor-A (PDGF-A) and TbetaRs were expressed in the vascular smooth muscle cells of all patients. PDGF-A, transforming Gfbeta1 and iNOS were expressed in the intimal smooth muscle cells of the KD but not the normal coronary artery without a history of KD. The number of TbetaR-II-positive cells were fewer than TbetaR-I-positive cells in the intima of CAL due to KD, but the number was of both almost same in the intima of coronary artery without CAL after KD and in the normal coronary. CONCLUSION: The intact coronary artery 13 months after KD still showed the influence of the inflammation of KD. Although the authors speculate that the intimal proliferation will not continue beyond the acute phase, those patients may have a risk factor for atherosclerosis.