摘要目的 评估基于“严重程度临床分级”的川崎病冠状动脉病变的诊断、治疗及随访方案的效果及安全性.方法 2005年7月至2013年12月复旦大学附属儿科医院收治的川崎病合并冠状动脉病变患儿52例,男44例,女8例,患儿中位年龄41(26,83)个月.病程2个月以上,超声心动图初步诊断冠状动脉病变严重程度Ⅳ级及以上,并行冠状动脉造影确定冠状动脉病变程度.依据“川崎病冠状动脉病变的临床处理建议”,冠状动脉造影确定诊断为Ⅳ级以上患儿给予华法林及小剂量阿司匹林口服,并监测国际标准化比值(INR)值;Ⅴb级患儿详细评估后决定是否行冠状动脉旁路移植术(CABG)治疗.并依据建议进行规范随访,分析诊断治疗过程中的并发症以及随访终期的结局.结果 (1)52例患儿均获得理想冠状动脉造影图像.45例(86％)为巨大冠状动脉瘤或多发性冠状动脉瘤,冠状动脉瘤内血栓发生率为22％(10/45).52例患儿共有138支冠状动脉发生病变,以左前降支和右冠状动脉病变为最多见,分别为47支(34％)和48支(35％).(2)52例患儿经冠状动脉造影后仍为Ⅳ级及以上49例,3例改为Ⅲ级.最终冠状动脉病变严重程度临床分级为:Ⅲ级3例,Ⅳ级31例,Ⅴa级9例,Ⅴb级9例.(3)38例正规服用阿司匹林及华法林治疗患儿,2例INR严重升高出现膝关节和胃部出血,均治愈,INR为1.5 ～2.5的患儿未出现出血.(4) 52例患儿随访时间6个月～8年,1例患儿再次造影证实有血栓形成,但未引起狭窄;4例患儿由Ⅳ级恢复为Ⅲ级或Ⅱ级;其余均未显示新的血栓形成或狭窄.(5)9例Ⅴb级患儿中5例行CABG,年龄最小的22个月的患儿术中死亡.另4例术后恢复顺利,术后随访8～ 90个月,1例术前左心室射血分数(LVEF)仅32.8％患儿,术后8个月LVEF为35％ ～44％;余3例均正常.另4例Ⅴb级患儿2例因严重心功能不全及心肌活性丧失无手术指征,2例因年龄仅9个月在密切随访中.结论 冠状动脉造影在儿童甚至小婴儿安全有效,目前仍是冠状动脉病变临床分级的金标准;正规抗凝治疗可明显降低川崎病冠状动脉病变患儿冠状动脉阻塞发生率,服用华法林时调整INR值在1.5 ～2.5为安全范围;CABG为冠状动脉严重病变伴心肌缺血的有效治疗方法.

abstractsObjective To evaluate the efficacy and safety of evaluation,treatment and follow-up of Kawasaki coronary artery disease based on the clinical severity classification.Method This study evaluated 52 patients admitted to the Children's Hospital of Fudan University between July 2005 and December 2013 who were diagnosed with Kawasaki Disease with coronary artery disease.Inclusion criteria were a disease course of more than two months,initial echocardiography showing severity of grade Ⅳ and above,and confirmation of disease severity by angiography.Of those studies,44 were male and eight were female,aged 6 to 142 (average 41) months.Treatment was planned according to protocols in "Suggestions for Management of Kawasaki Coronary Artery Disease" with follow-up.Those patients with grade Ⅳ and above confirmed by angiogram were given oral low-dose asprin and warfarin,and those with grade Ⅴ b were given coronary artery bypass grafting(CABG) after comprehensive evaluation.Analysis was carried out for diagnosis,treatment,complications,and results of follow-up.Result (1) Satisfied images were shown by the angiography of all 52 cases.Forty five patients (86％) had giant aneurysm or multiple aneurysms,with thrombosis found in 10 of 45 patients (22％).Coronary artery lesions occurred in 138 coronary branches,and more common in left anterior descending branch (47 branches,with incidence 34％) and right coronary artery (48 branches,with incidence 35％).There were no complications during or after angiography.(2) After angiography,49 patients remained at grade Ⅳ or above,and three improved to grade Ⅲ.Ultimately,clinical severities of coronary artery disease included three patients at grade Ⅲ,31 patients at grade Ⅳ,nine patients at grade Ⅴ a,and nine patients at grade Ⅴ b.(3) Thirty-eight patients were properly using aspirin and warfarin,and two patients with severely elevated international normalized ratio(INR) levels presented with knee joint and gastric hemorrhage,both of which were treated successfully.Patients with INR levels between 1.5 and 2.5 did not show signs of hemorrhage.(4) In follow-up visits between 6 months and 8 years,one patient had representation of thrombosis on angiography,but did not lead to coronary stenosis;four patients were improved from grade Ⅳ to either grade Ⅲ or Ⅱ.The remaining showed no new thrombotic formation or stenosis.(5) Of the nine grade Ⅴ b patients,five underwent coronary artery bypass grafting.The youngest of these patients,a 22 months old girl,died intraoperatively.The remaining four recovered postoperatively and were followed up for 8 to 90 months.One patient had a preoperative left ventricular ejection fraction (LVEF) of only 32.8％,with LVEF remaining abnormal post-CABG,between 35％ and 44％.The remaining three patients had normal heart size,cardiac function,and electrocardiogram.Of the other four grade Ⅴ b patients,two were contraindicated for surgery due to severe heart failure and loss of myocardial activity.Two other cases are being followed up closely due to their young age of 9 months.Conclusion Coronary angiography is safe and efficacious in children,and even in infants.It is the current gold standard tool for grading Kawasaki coronary artery disease.Proper anticoagulation therapy can markedly decrease the incidence of coronary artery occlusion in patients with Kawasaki coronary artery disease.Safe ranges of corrected INR should be between 1.5 and 2.5 after taking warfarin.CABG is an effective treatment for severe coronary artery disease with myocardial ischemia.