應(yīng)激性心肌病課件

Kawasaki

Disease宋雷Kawasaki

Disease宋雷川崎富作（1925.2.7—）Firstintroducedin1967川崎富作（1925.2.7—）Firstintro概念原因未明全身性血管炎綜合征，主要影響中小動(dòng)脈全世界均有發(fā)病，以亞裔兒童多見1976年我國(guó)首例報(bào)導(dǎo)5歲以下占80%，平均年齡1.5-2歲，男：女=1.5：1微生物超抗原激活具有遺傳易感性患兒的T細(xì)胞，引發(fā)異常免疫反應(yīng)，導(dǎo)致免疫性損傷概念原因未明全身性血管炎綜合征，主要影響中小動(dòng)脈JettTravolta,SonofJohnTravoltaJettTravolta,16yrsold,diedaftersufferingaseizureatthefamily’svacationhomeintheBahamas.JettwassufferingfromKawasakiSyndromesincehewas2-years-oldJettTravolta,SonofJohnTra臨床癥狀和診斷標(biāo)準(zhǔn)發(fā)熱>5d球結(jié)膜充血口唇潮紅、楊梅舌、黏膜充血不定形皮疹，多形紅斑急性期手足硬腫，掌跖及肢端紅斑；恢復(fù)期指趾端脫皮非化膿性頸淋巴結(jié)腫大6個(gè)主要癥狀滿足5個(gè)即可診斷，如只有4個(gè)，但超聲或造影證實(shí)冠狀動(dòng)脈瘤或擴(kuò)張，也可確診臨床癥狀和診斷標(biāo)準(zhǔn)發(fā)熱>5d心臟受累表現(xiàn)心臟受累表現(xiàn)典型病理表現(xiàn)Discreteregionsofmyointimalproliferationassociatedwithdisruptedinternalelasticlaminaandmedialsmoothmusclecellnecrosiswithreplacementbyfibrosisandcalcification典型病理表現(xiàn)Discreteregionsofmyoi冠狀動(dòng)脈損害20%未治療及5%經(jīng)IVIG治療后的KD患者發(fā)生冠脈損害以冠狀動(dòng)脈瘤或擴(kuò)張多見絕大多數(shù)冠狀動(dòng)脈瘤發(fā)生在冠狀動(dòng)脈主干近端，最常見為L(zhǎng)M近端，其次為RCA近端、LAD、RCA中段?。⊿mall）<5mm，中等（Medium）5-8mm，大（Giant）>8mm除冠脈外，KD還可累及心臟瓣膜、心肌和心包冠狀動(dòng)脈損害20%未治療及5%經(jīng)IVIG治療后的KD患者發(fā)生<40歲CAG患者261例冠狀動(dòng)脈瘤/擴(kuò)張16例4例確診繼發(fā)于KD，9例可以繼發(fā)于KD<40歲CAG患者261例預(yù)后急性和亞急性期出現(xiàn)的輕度冠脈擴(kuò)張多可在40d內(nèi)恢復(fù)多數(shù)小到中等的冠狀動(dòng)脈瘤在發(fā)病后1～2年消失巨大冠狀動(dòng)脈瘤或多個(gè)呈串珠狀中等冠狀動(dòng)脈瘤可能發(fā)生心肌梗死、破裂導(dǎo)致的猝死及狹窄導(dǎo)致的缺血性心臟病川崎病并發(fā)心肌梗死者約占0.1-1％，多發(fā)生在第一年預(yù)后急性和亞急性期出現(xiàn)的輕度冠脈擴(kuò)張多可在40d內(nèi)恢復(fù)并發(fā)心梗的高危因素冠狀動(dòng)脈瘤最大內(nèi)徑>8mm冠狀動(dòng)脈瘤形態(tài)為囊狀、念珠狀、香腸狀急性期發(fā)熱時(shí)間>21d急性期單獨(dú)使用糖皮質(zhì)激素發(fā)病年齡>2歲并發(fā)心梗的高危因素冠狀動(dòng)脈瘤最大內(nèi)徑>8mm心臟超聲顯示冠脈近端病變超聲心動(dòng)圖是檢測(cè)冠狀動(dòng)脈并發(fā)癥的最重要的非侵人性方法，與冠狀動(dòng)脈造影和尸檢結(jié)果符合率達(dá)86％-100％心臟超聲顯示冠脈近端病變超聲心動(dòng)圖是檢測(cè)冠狀動(dòng)脈并發(fā)癥的最重CT在川崎病診斷中的價(jià)值CTAshowingextensivecalcificationofaLADaneurysmina12-year-oldmalepatient7yearsafteracuteKawasakidisease.Coronaryarteryaneurysmsina9-yearoldboy,3yearsafterdiagnosisofKawasakiDiseaseCT在川崎病診斷中的價(jià)值CTAshowingextensCase13-year-11-month-oldboywithKDandgiantcoronaryaneurysms.CoronaryangiogramofleftcoronaryarteryshowsaneurysmsofLADandLCX.Case13-year-11-month-Case2OccludedRCAaneurysmina33-year-oldmanwhoexperiencedaKDat6yearsoldandwasasymptomaticuntilhismyocardialinfarction.InjectionoftheoccludedRCARCAafterPTCA.NotesmoothappearanceofdistalbranchesoftheRCAPatentRCAaneurysm1monthafterPTCA,aspirin,andwarfarintherapymaintaininganINRof2-2.5Case2OccludedRCAaneurysminCase3A33-year-oldwomandevelopedshockanddied16hoursafterlaborwithmoderatehemorrhage.

Whenthepatientwas16yearsold,shewasdiagnosisasKawasakidisease(KD).Thiswoman’sson,theproductofthatpregnancy,wasadmitted5yearslaterwithclassicKD.Case3A33-year-oldwomandeve成人疑診川崎病幼年病史：長(zhǎng)期發(fā)熱、皮疹、硬腫、結(jié)膜充血尚無(wú)特異性回顧性診斷指標(biāo)影像學(xué)：胸片或CT示原動(dòng)脈瘤形成部位動(dòng)脈壁鈣化鑒別診斷：病變多局限IVUS有助于觀察增厚的動(dòng)脈壁成人疑診川崎病幼年病史：長(zhǎng)期發(fā)熱、皮疹、硬腫、結(jié)膜充血治療急性期大劑量丙種球蛋白靜脈滴注腸溶阿司匹林30～50mg/kg·d，

熱退后3天漸減為3～5mg/kg·d，持續(xù)6～8周，冠狀動(dòng)脈損害者應(yīng)延長(zhǎng)治療糖皮質(zhì)激素不宜單獨(dú)使用，對(duì)IVIG無(wú)效者可考慮加用恢復(fù)期口服抗血小板或抗凝藥物定期復(fù)查，避免過量運(yùn)動(dòng)必要時(shí)PTCA或CABG，主要取決于缺血癥狀治療急性期冠狀動(dòng)脈瘤的進(jìn)展50%恢復(fù)正常25%變小25%無(wú)變化7-20%發(fā)生動(dòng)脈瘤相關(guān)的狹窄或心肌梗死冠狀動(dòng)脈瘤的進(jìn)展50%恢復(fù)正常小或中等冠狀動(dòng)脈瘤藥物治療口服小劑量阿司匹林直到恢復(fù)正常體育活動(dòng)<11歲，在發(fā)病6-8周內(nèi)避免體育活動(dòng)11-20歲，基于2年1次的負(fù)荷試驗(yàn)/心肌灌注掃描結(jié)果不鼓勵(lì)接觸性/高負(fù)荷運(yùn)動(dòng)隨訪每年復(fù)查心臟超聲及心電圖如有缺血癥狀需冠脈造影小或中等冠狀動(dòng)脈瘤藥物治療無(wú)狹窄的巨大冠狀動(dòng)脈瘤藥物治療長(zhǎng)期口服小劑量阿司匹林+華法林或低分子肝素體育活動(dòng)避免接觸性/高負(fù)荷運(yùn)動(dòng)其它運(yùn)動(dòng)基于負(fù)荷試驗(yàn)/心肌灌注結(jié)果隨訪每年2次復(fù)查心臟超聲及心電圖每年1次負(fù)荷試驗(yàn)/心肌灌注顯像侵入性檢查6-12月時(shí)冠脈造影，根據(jù)臨床需要隨時(shí)復(fù)查無(wú)狹窄的巨大冠狀動(dòng)脈瘤藥物治療伴狹窄的冠狀動(dòng)脈瘤藥物治療長(zhǎng)期口服小劑量阿司匹林+華法林或低分子肝素β受體阻滯劑減低心肌氧耗體育活動(dòng)禁止接觸性或高負(fù)荷運(yùn)動(dòng)其它運(yùn)動(dòng)基于負(fù)荷試驗(yàn)/心肌灌注結(jié)果隨訪每年2次復(fù)查心臟超聲及心電圖每年1次負(fù)荷試驗(yàn)/心肌灌注顯像侵入性檢查常規(guī)冠脈造影，癥狀變化隨時(shí)復(fù)查伴狹窄的冠狀動(dòng)脈瘤藥物治療Coronaryarteriesaneurysms&ectasia

冠狀動(dòng)脈局部或彌漫的擴(kuò)張直徑超過正常參考血管的1.5倍Coronaryarteriesaneurysms&Fusiform&SaccularFusiform&Saccular冠狀動(dòng)脈瘤/擴(kuò)張的形態(tài)學(xué)分型冠狀動(dòng)脈瘤/擴(kuò)張的形態(tài)學(xué)分型鑒別診斷川崎病：

病變多局限，管壁增厚，前后血管光滑正常動(dòng)脈粥樣硬化遺傳性家族性高膽固醇血癥結(jié)節(jié)性多動(dòng)脈炎大動(dòng)脈炎其它結(jié)締組織?。豪钳彙兹?、馬凡感染：敗血癥、梅毒、真菌感染外傷或冠脈介入相關(guān)損傷鑒別診斷川崎?。翰∽兌嗑窒?，管壁增厚，前后血管光滑正常鑒別診斷病因年齡形態(tài)診斷病生理機(jī)制動(dòng)脈粥樣硬化成人E/A最普遍原因，多動(dòng)脈受累、伴有冠脈狹窄，RCA最常見粥樣硬化累及中膜川崎病兒童A兒童最常見原因，多可恢復(fù)自身免疫因素、血管炎炎性疾病青年E大動(dòng)脈炎、SLE、RA、Wegener肉芽腫、Churg-Strauss綜合征、MPA、APS、白塞氏病、Sarcoid、PAN、CREST綜合征，AS，Reiter綜合征，psoriaticarthritis炎性介質(zhì)：VCAM-1,ICAM-1,Eselectin鑒別診斷病因年齡形態(tài)診斷病生理機(jī)制動(dòng)脈粥樣硬化成人E/A最普鑒別診斷病因年齡形態(tài)診斷病生理機(jī)制瘺任何年齡E大部分為先天性，多源于RCA繼發(fā)于高血流的代償性擴(kuò)張畸形（如ALCAPA）嬰兒型或成人型E嬰兒型多死于早期心梗，成人型多見左右冠系統(tǒng)間側(cè)支繼發(fā)于缺血的代償性擴(kuò)張結(jié)締組織病青年、成人EEhlers-Danlos綜合征，馬凡、囊性中膜壞死（CMN）IL-6,C-reactiveprotein,MMP-2,MMP-9感染任何年齡A金葡、綠膿、霉菌、梅毒感染、萊姆病滋養(yǎng)血管微栓塞、直接侵犯動(dòng)脈壁或免疫復(fù)合物沉積外傷/醫(yī)源性損傷成人A相應(yīng)病史損傷后愈合不全毒品成人A相關(guān)病史發(fā)作性高血壓、血管痙攣或粥樣硬化鑒別診斷病因年齡形態(tài)診斷病生理機(jī)制瘺任何年齡E大部分為先天性Coronaryarteryatherosclerosis55yearsold,hypertensive,obesefemale,withinfero-posteriorandrightSTEMI.CoronaryarteryatherosclerosiCoronaryfistulaAn11-year-oldboywithaCTdemonstratedgiantleftmaincoronaryartery(15mm)duetoacongenitalfistulawiththerightatrium.Reformattedimagedemonstratestheentirecourseofthe

fistula.Thepatientunderwentsurgicalclosureofthefistulaandremainsasymptomatic.CoronaryfistulaAn11-year-oldALCAPAsyndromeA25-year-oldwomanwithchestpainwhohadpositivefindingsfromanuclearmedicinestresstest.CTclearlyshowsdiffusecoronaryectasiaasacompensatoryresponsethatisduetoananomalousoriginoftheleftmaincoronaryarteryfromthepulmonaryartery(ALCAPA）ALCAPAsyndromeA25-year-oldHeterozygousFamilialHypercholesterolemiaCoronaryangiogramsfroma43-year-oldmanwithfamilialhypercholesterolemia(A)anda37-year-oldmanwithfamilialhypercholesterolemia(B)intheleftanteriorobliqueprojections,showinglocalizedectasiaoftherightcoronaryartery.Arrowsindicateectaticregions.HeterozygousFamilialHyperchoPolyarteritisNodosaThis25-year-oldwomanhadbeengivenadiagnosisofpolyarteritisnodosa3yearsearlierandwasreceivingprednisolonemaintenancetherapywhenshepresentedwithcardiacarrest.Althoughseverecardiacinvolvementinpolyarteritisnodosaisunusual,itcanresultinmyocardialinfarctionandconfersapoorerprognosis.Despitetreatment,thepatientdiedafewmonthslaterPolyarteritisNodosaThis25-yeTakayasuDiseaseA30yearoldwomenwasadmittedforsyncope.Bloodpressurewas100/70mmHgintheleftarm,whilenotrecordableintheright.Therewasablowingdiastolicmurmurovertheleftsternalborder.Totalocclusionoftherightsubclavianartery,narrowingofthepro