先心雙語(七年制)-廣西醫(yī)科大學(xué)醫(yī)學(xué)醫(yī)藥

龐玉生授課對(duì)象：本科（七年制）廣西醫(yī)科大學(xué)兒科學(xué)教研室Chapter13循環(huán)系統(tǒng)疾病Circulationsystemdisease初步熟悉、了解先天性心臟病掌握先天性心臟病的診斷和治療教學(xué)目的先天性心臟病血液動(dòng)力學(xué)改變與臨床表現(xiàn)的關(guān)系教學(xué)難點(diǎn)教學(xué)重點(diǎn)詳細(xì)介紹先天性心臟病的臨床表現(xiàn)和診斷方法Contents小兒心血管系統(tǒng)解剖生理特點(diǎn)及檢查方法小兒先天性心臟病概述房間隔缺損室間隔缺損動(dòng)脈導(dǎo)管未閉法洛四聯(lián)癥第一節(jié)小兒心血管系統(tǒng)解剖生理

特點(diǎn)及檢查方法一心臟的胚胎發(fā)育

Cardiacdevelopmentduringfetalperiod

Theprimitivehearttubeformsby3W

Cardiacloopingby22~24daysBendventrallyandtowardtherightCardiacseptation第一隔原發(fā)孔心內(nèi)膜墊靜脈竇瓣膜第二隔繼發(fā)孔第一隔室間孔第二隔繼發(fā)孔第一隔卵園孔第一隔室間孔第二隔靜脈竇瓣膜胚胎房室分隔及房室間隔發(fā)育室間隔形成心室間隔組成三個(gè)來源

肌隔

心內(nèi)膜墊向下生長與肌隔相合

動(dòng)脈總干及心球分化成主動(dòng)脈與肺動(dòng)脈時(shí)的中隔向下延伸部分室間隔膜部AorticarchdevelopmentRightandlefttruncoconalridgesTheaorticandpulmonaryoutflowtractsarefullyseparatedThecoronarysinusThesemilunarvalves主肺動(dòng)脈由總干呈螺旋形分隔AOPAPAAOPAPAAOAO大動(dòng)脈位置變移系列示意圖PAPAPAPATOFTGADORVTaussig-Bing

房室管、心房、及心室的分隔室間隔膜部的形成及室間隔的封閉第2周原始心臟形成第4周心臟起循環(huán)作用第8周四腔心臟形成正常血液循環(huán)途徑二胎兒新生兒循環(huán)轉(zhuǎn)換Theplacenta:gasexchange,metabolicexchangeThelung:constrictedThreecardiovascularstructure:DuctusvenosusForamenovaleDuctusarteriosusTheFetalCirculation01營養(yǎng)和氣體代謝通過臍血管和胎盤與母體進(jìn)行彌漫式交換02絕大部分為混合血03供應(yīng)心臟、腦、肝及上肢血氧含量遠(yuǎn)較下半身高04肺處于壓縮狀態(tài)無氣體交換05卵圓孔、動(dòng)脈導(dǎo)管和靜脈導(dǎo)管開放06右心室優(yōu)勢胎兒血循環(huán)特點(diǎn)TheFetalCirculation胎兒血液循環(huán)途徑通過動(dòng)脈導(dǎo)管通過卵圓孔血氧含量較高血氧含量較低通過靜脈導(dǎo)管下腔靜脈右心房右心室左心房左心室升主動(dòng)脈心腦及上肢肺動(dòng)脈降主動(dòng)脈下半身上半身靜脈血上腔靜脈臍靜脈動(dòng)脈血門靜脈靜脈血母體下半身靜脈血肺循環(huán)50%1/3CirculationChangesafterbirthTheumbilicalcordclampedbreathingcommencesPulmonarycirculationProvidinggasexchangeTheforamenovaleandductusarteriosus:functionallyclosed胎兒與出生后血液循環(huán)比較A胎兒期B出生后由母體循環(huán)完成氣體交換由肺循環(huán)完成氣體交換多為混合血，心、腦、上半身血氧含量高于下半身靜脈血和動(dòng)脈血分開卵圓孔、動(dòng)脈導(dǎo)管、靜脈導(dǎo)管開放卵圓孔、動(dòng)脈導(dǎo)管、靜脈導(dǎo)管閉合肺動(dòng)脈壓與主動(dòng)脈相似，肺循環(huán)阻力高肺動(dòng)脈壓下降，肺循環(huán)阻力低右心室高負(fù)荷左心室高負(fù)荷AB返回CommonsymtomsCongestiveheartfailureFeedingdifficulties/ExerciseintoleranceRespiratorydistressPoorgrowthCracked,coughSquattingSyncopeCyanosis三兒童心血管疾病體格檢查方法PhysicalexaminationCommonlymanifestationsEvaluationforBWandBHRespiratorydistressCyanosisClubbingdigitsHepatomegalyDistendedneckveinsSignofliver-jugularveinPeripheraledemaCardiovascularExaminationInspection:ProminenceoftheprecordiumCardiomegaly

Palpation:heave,thrillPercussion:sizeandpositionoftheheartAuscultation:HR,rhythm(1)Normalheartsounds:S1,S2,S3,S4(2)Abnormalheartsounds:P2fixedsplitting(3)Murmurs小兒聽診常用聽診區(qū)二尖瓣聽診區(qū)肺動(dòng)脈瓣聽診區(qū)主動(dòng)脈瓣聽診區(qū)三尖瓣聽診區(qū)LocationandradiationTiming:Systolic,diastolicandcontinuousQuality:Harsh,musical,orrough;high, mediumorlowinpitchIntensity:gradeⅠ,Ⅱ,Ⅲ,Ⅳ,Ⅴ,ⅥVariationwithpositionMurmur主動(dòng)脈縮窄：股動(dòng)脈搏動(dòng)減弱或消失下肢血壓低于上肢動(dòng)脈導(dǎo)管未閉或主動(dòng)脈關(guān)閉不全：脈壓增寬毛細(xì)血管搏動(dòng)股動(dòng)脈槍擊音四肢脈搏及血壓周圍血管征Chestx-ray1Electrocardiogram2Echocardiogram3Cardiaccatheterization4Angiocardiography5Radionuclideangiocardiography6Magneticresonanceimaging7Computedtomography8四心臟的特殊檢查characterofthepulmonaryvasculature1Positionofthecardiacapex2cardiacsize(cardiothoracicratio)3cardiacconfiguration,heartshape4Positionoftheabdominalviscera5ChestRadiographNormalcardiothoracicimaginglandmarks,PAviewAnteriorradiographicviewofcardiacanatomyX-ray你的X線診斷分別是什么？ThesequenceofECG:rate,rhythm,Pwave,PRinterval,QRScomplex(axis,amplitude,andduration),QTinterval,STsegment,andTwave.AssessatrioventricularhypertrophyDiagnosisofdysrhythmiasElectrocardiogram,ECGNormalECGEchocardiographyApowerfulnoninvasivemethod:M-mode,twoandthreedimensional,colorandDopplerEvaluatecardiacstructureEstimateintracardiacpressureandgradientacrossstenoticvalvesandvesselsQuantitatecardiaccontractilefunctionDeterminethedirectionofflowacrossadefectExaminetheintegrityofthecoronaryarteriesEvaluateendocarditis,pericardiacfluid,cardiactumors,orchamberthrombi.M-modeECHOLong-axisviewforLV2-DECHOchamberviewatapex2-DECHOShort-axisviewatthegreatartery2-DcolorECHO(four-chamberview)2-DcolorDopplerECHO3-DReconstructionSVCIVCRVCardiacCatheterizationEstimateCardiacoutputaccordingtoOxygencontentandsaturation,pulmonaryandsystemicbloodflow.DeterminethepressuresinallchambersandvesselsCalculatethepulmonaryandsystemicvascularresistanceInterventionalcatheterization(therapeuticprocedures)20%12~14%30/1212~14%12~14%4~80~5100/1030/5100/60選擇性造影Selectiveangiocardiography1數(shù)字減影造影(DSA)2診斷復(fù)雜先心病的主要手段3常用成角造影4長軸斜位：左前斜60～75°＋向頭成角20～30°5肝鎖位：左前斜40～45°＋向頭成角40°6坐觀位：正位向頭成角40°7Angiocardiography心血管造影心血管造影Radionuclideangiocardiography99m锝iv釋放γ射線初次循環(huán)心臟造影、平衡心臟血池造影檢測左向右分流評(píng)估心功能MagneticresonanceimagingPaulC.Lauterbur,UnitedStates,andSirPeterMansfield,Britain,winedthe2003NobelPrizefordiscoveriesinmagneticresonanceimaging04Providetheimagingofvascularstructuresofthethorax05Noninvasivetool01Evaluatetheheartandthegreatarteries03Follow-up02SEAOPAAOLVLARVCineMRICine-MRIMRAMRIisinvaluableinthelongtermfollow-upofcoarctationoftheaortaafterangioplasty.(surgicalprocedure)3-DMRIComputedtomographyFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethorax010302A概念：CHD是胎兒期心臟及大血管發(fā)育異常而致的心血管畸形，是小兒最常見的心臟病B發(fā)病率:6～8‰，上海6.87‰C我國每年出生15萬CHDD診治研究取得很大進(jìn)展，預(yù)后大為改觀第二節(jié)小兒先天性心臟病概述病因內(nèi)因：與遺傳有關(guān)，染色體異?；蚧蛲蛔儭Ｍ庖颍涸缙趯m內(nèi)感染：風(fēng)疹、流感、腮腺炎、柯薩奇病毒等。孕母接觸大量放射線服用藥物史（抗癌藥、抗癲癇藥)代謝性疾病:糖尿病、高鈣血癥宮內(nèi)慢性缺氧妊娠早期酗酒、吸食毒品環(huán)境因素＋遺傳因素CategoriesofcongenitalheartdiseaseTheleft-to-rightshunttype(ASDVSDPDA)AcyanoticearlyPulmonarybloodincreasedSystemicblooddecreasedPulmonaryhypertensionEisenmengersyndrome:cyanoticlatelyduetoright-to-leftshuntsTheright-to-leftshunttypeTetralogyofFallot(TOF),transpositionofthegreatarteries(TGA)CyanoticearlyCyanoticlesionswithincreasedpulmonarybloodflow12345ThesystemiccirculationishypoxemiaCyanoticlesionswithdecreasedpulmonarybloodflowPulmonarystenosis(PS),Coarctationoftheaorta(Coa)AcyanoticHypertrophyresultinginincreasedpressureloadNo-shuntlesionsVanPraagh順序分段診斷方法“三層樓結(jié)構(gòu)”“四層樓結(jié)構(gòu)”心房位置判斷心室位置判斷大血管位置判斷房室連接診斷心室大動(dòng)脈連接診斷心臟位置DiagnosticprocessforCHDTwogroupsbasedonPEacyanoticandcyanoticlesionsFurtherbesubdividedbasedonX-rayincreased,normalordecreasedpulmonaryvascularmarkingsDeterminewhetherright,left,orbiventricularhypertrophyConfirmthediagnosisbyECHOorCatheterization,orboth第三節(jié)幾種常見的先天性心臟病CommoncongenitalheartdiseasesAtrialSeptaldefect（ASD)ASDGeneralconsiderationsDefinition:anopeningintheatrialseptumpermittingtheshuntingofbloodbetweentheatriaIncidence:10%inallCHDThreemajortypes:(1)Theostiumsecundumtype(2)Thesinusvenosustype(3)TheostiumprimumtypeASDPathophysiologyThedegreeofL-Rshunt(1)Thesizeofthedefect(2)TherelativecompliancesofRVandLV(3)therelativevascularresistancesinthepulmonaryandsystemiccirculationASDPathophysiologyInneonateandearlyinfant

limitingtheLRshuntSmallASDLargeASDQp:Qs2~4:1PAH：RLshunt可在臨床上表現(xiàn)出來ASD分流右心室擴(kuò)大肺動(dòng)脈充血肺循環(huán)充血肺動(dòng)脈高壓肺靜脈動(dòng)脈血左心房左心室主動(dòng)脈搏血減少體循環(huán)缺血上下腔靜脈右心房擴(kuò)大ASD血液循環(huán)途徑LVRVLARAWhytheLAisnotenlarged？SmallASD：MostoftenasymptomaticdiscoveredduringPELargeASD（largeshunt）：(1)Pulmonryblood：repeatedURIorpneumonia(2)Systemicblood：poorgrowth,pale,lackofpower,hidrosis,tachypneaafterexercise(3)Pulmonaryhypertension,Eisenmenggersyndrome（cyanosis）

ASDClinicalmanifestationASDCardiacsignInspection:leftprecordialbulgeandincreasedcardiacactivityPalpation:systoliclift,seldomwiththrillPercussion:cardiomegalyAuscultation:loudS12,3LSBsystolicejectionmurmur,gradeⅡ~Ⅲ

fixedsplittingofthe2ndheartsoundLowerLSBdiastolicmurmurEarlysystolicclick,P2accentuated(PAH)ASDⅡ-Ⅲ/ⅥSMP2亢進(jìn)伴固定分裂，喀喇音QRSaxis:normalorrightaxisdeviationwithaclockwiseloopinthefrontalplaneRVhypertrophyAminorrightventricularconductiondelayVolumeoverloadoftheRV:01Intherightprecordialleads,arsR’patternP-Rdelay02ASDECGIAVBASDECGASDX-rayThepulmonaryvascularityincreasedEnlargementoftheRVandRAThepulmonaryarterydilatedMmode:RVvolumeoverloa