中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2015年
9期
690-695
,共6页
刘芳%赵璐%吴琳%梁雪村%储晨%何岚%黄国英
劉芳%趙璐%吳琳%樑雪村%儲晨%何嵐%黃國英
류방%조로%오림%량설촌%저신%하람%황국영
粘膜皮肤淋巴结综合征%冠状血管%疾病管理
粘膜皮膚淋巴結綜閤徵%冠狀血管%疾病管理
점막피부림파결종합정%관상혈관%질병관리
Mucocutaneous lymph node syndrome%Coronary vessels%Disease management
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.
目的 評估基于“嚴重程度臨床分級”的川崎病冠狀動脈病變的診斷、治療及隨訪方案的效果及安全性.方法 2005年7月至2013年12月複旦大學附屬兒科醫院收治的川崎病閤併冠狀動脈病變患兒52例,男44例,女8例,患兒中位年齡41(26,83)箇月.病程2箇月以上,超聲心動圖初步診斷冠狀動脈病變嚴重程度Ⅳ級及以上,併行冠狀動脈造影確定冠狀動脈病變程度.依據“川崎病冠狀動脈病變的臨床處理建議”,冠狀動脈造影確定診斷為Ⅳ級以上患兒給予華法林及小劑量阿司匹林口服,併鑑測國際標準化比值(INR)值;Ⅴb級患兒詳細評估後決定是否行冠狀動脈徬路移植術(CABG)治療.併依據建議進行規範隨訪,分析診斷治療過程中的併髮癥以及隨訪終期的結跼.結果 (1)52例患兒均穫得理想冠狀動脈造影圖像.45例(86％)為巨大冠狀動脈瘤或多髮性冠狀動脈瘤,冠狀動脈瘤內血栓髮生率為22％(10/45).52例患兒共有138支冠狀動脈髮生病變,以左前降支和右冠狀動脈病變為最多見,分彆為47支(34％)和48支(35％).(2)52例患兒經冠狀動脈造影後仍為Ⅳ級及以上49例,3例改為Ⅲ級.最終冠狀動脈病變嚴重程度臨床分級為:Ⅲ級3例,Ⅳ級31例,Ⅴa級9例,Ⅴb級9例.(3)38例正規服用阿司匹林及華法林治療患兒,2例INR嚴重升高齣現膝關節和胃部齣血,均治愈,INR為1.5 ～2.5的患兒未齣現齣血.(4) 52例患兒隨訪時間6箇月～8年,1例患兒再次造影證實有血栓形成,但未引起狹窄;4例患兒由Ⅳ級恢複為Ⅲ級或Ⅱ級;其餘均未顯示新的血栓形成或狹窄.(5)9例Ⅴb級患兒中5例行CABG,年齡最小的22箇月的患兒術中死亡.另4例術後恢複順利,術後隨訪8～ 90箇月,1例術前左心室射血分數(LVEF)僅32.8％患兒,術後8箇月LVEF為35％ ～44％;餘3例均正常.另4例Ⅴb級患兒2例因嚴重心功能不全及心肌活性喪失無手術指徵,2例因年齡僅9箇月在密切隨訪中.結論 冠狀動脈造影在兒童甚至小嬰兒安全有效,目前仍是冠狀動脈病變臨床分級的金標準;正規抗凝治療可明顯降低川崎病冠狀動脈病變患兒冠狀動脈阻塞髮生率,服用華法林時調整INR值在1.5 ～2.5為安全範圍;CABG為冠狀動脈嚴重病變伴心肌缺血的有效治療方法.
목적 평고기우“엄중정도림상분급”적천기병관상동맥병변적진단、치료급수방방안적효과급안전성.방법 2005년7월지2013년12월복단대학부속인과의원수치적천기병합병관상동맥병변환인52례,남44례,녀8례,환인중위년령41(26,83)개월.병정2개월이상,초성심동도초보진단관상동맥병변엄중정도Ⅳ급급이상,병행관상동맥조영학정관상동맥병변정도.의거“천기병관상동맥병변적림상처리건의”,관상동맥조영학정진단위Ⅳ급이상환인급여화법림급소제량아사필림구복,병감측국제표준화비치(INR)치;Ⅴb급환인상세평고후결정시부행관상동맥방로이식술(CABG)치료.병의거건의진행규범수방,분석진단치료과정중적병발증이급수방종기적결국.결과 (1)52례환인균획득이상관상동맥조영도상.45례(86％)위거대관상동맥류혹다발성관상동맥류,관상동맥류내혈전발생솔위22％(10/45).52례환인공유138지관상동맥발생병변,이좌전강지화우관상동맥병변위최다견,분별위47지(34％)화48지(35％).(2)52례환인경관상동맥조영후잉위Ⅳ급급이상49례,3례개위Ⅲ급.최종관상동맥병변엄중정도림상분급위:Ⅲ급3례,Ⅳ급31례,Ⅴa급9례,Ⅴb급9례.(3)38례정규복용아사필림급화법림치료환인,2례INR엄중승고출현슬관절화위부출혈,균치유,INR위1.5 ～2.5적환인미출현출혈.(4) 52례환인수방시간6개월～8년,1례환인재차조영증실유혈전형성,단미인기협착;4례환인유Ⅳ급회복위Ⅲ급혹Ⅱ급;기여균미현시신적혈전형성혹협착.(5)9례Ⅴb급환인중5례행CABG,년령최소적22개월적환인술중사망.령4례술후회복순리,술후수방8～ 90개월,1례술전좌심실사혈분수(LVEF)부32.8％환인,술후8개월LVEF위35％ ～44％;여3례균정상.령4례Ⅴb급환인2례인엄중심공능불전급심기활성상실무수술지정,2례인년령부9개월재밀절수방중.결론 관상동맥조영재인동심지소영인안전유효,목전잉시관상동맥병변림상분급적금표준;정규항응치료가명현강저천기병관상동맥병변환인관상동맥조새발생솔,복용화법림시조정INR치재1.5 ～2.5위안전범위;CABG위관상동맥엄중병변반심기결혈적유효치료방법.
Objective 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.
