先心雙語(七年制)-醫(yī)學醫(yī)藥課件

龐玉生授課對象：本科（七年制）廣西醫(yī)科大學兒科學教研室Chapter13循環(huán)系統(tǒng)疾病Circulationsystemdisease龐玉生授課對象：本科（七年制）Chapter13循環(huán)系統(tǒng)1教學目的

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重點

詳細介紹先天性心臟病的臨床表現(xiàn)和診斷方法教學難點

先天性心臟病血液動力學改變與臨床表現(xiàn)的關系教學目的2Contents小兒心血管系統(tǒng)解剖生理特點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脈導管未閉法洛四聯(lián)癥Contents小兒心血管系統(tǒng)解剖生理特點及檢查方法3第一節(jié)小兒心血管系統(tǒng)解剖生理

特點及檢查方法第一節(jié)小兒心血管系統(tǒng)解剖生理

特點及檢查方法4一心臟的胚胎發(fā)育

Cardiacdevelopmentduringfetalperiod

Theprimitivehearttubeformsby3W

Cardiacloopingby22~24daysBendventrallyandtowardtherightCardiacseptation一心臟的胚胎發(fā)育

Cardiacdevelopment5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6室間隔形成

心室間隔組成三個來源

①肌隔

②心內(nèi)膜墊向下生長與肌隔相合

③動脈總干及心球分化成主動脈與肺動脈時的中隔向下延伸部分室間隔膜部室間隔形成

心室間隔組成三個來源

①肌隔

7AorticarchdevelopmentRightandlefttruncoconalridgesTheaorticandpulmonaryoutflowtractsarefullyseparatedThecoronarysinusThesemilunarvalvesAorticarchdevelopmentRighta8主肺動脈由總干呈螺旋形分隔AOPAPAAOPAPAAOAO主肺動脈由總干呈螺旋形分隔AOPAPAAOPAPAAOAO9PAPAPAPA大動脈位置變移系列示意圖TOFTGADORVTaussig-BingPAPAPAPA大動脈位置變移系列示意圖TOFTGADORV10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周四腔心臟形成房室管、心房、室間隔膜部的形成及室間隔的封閉第2周原始心臟11正常血液循環(huán)途徑正常血液循環(huán)途徑12二胎兒新生兒循環(huán)轉換二胎兒新生兒循環(huán)轉換13Theplacenta:gasexchange,metabolicexchangeThelung:constrictedThreecardiovascularstructure:DuctusvenosusForamenovaleDuctusarteriosus

TheFetalCirculationTheplacenta:TheFetalCircula14胎兒血循環(huán)特點營養(yǎng)和氣體代謝通過臍血管和胎盤與母體進行彌漫式交換絕大部分為混合血供應心臟、腦、肝及上肢血氧含量遠較下半身高肺處于壓縮狀態(tài)無氣體交換卵圓孔、動脈導管和靜脈導管開放右心室優(yōu)勢胎兒血循環(huán)特點營養(yǎng)和氣體代謝通過臍血管和胎盤與母體進行彌漫式15TheFetalCirculationTheFetalCirculation16胎兒血液循環(huán)途徑通過動脈導管通過卵圓孔血氧含量較高血氧含量較低通過靜脈導管下腔靜脈右心房右心室左心房左心室升主動脈心腦及上肢肺動脈降主動脈下半身上半身靜脈血上腔靜脈臍靜脈動脈血門靜脈靜脈血母體下半身靜脈血肺循環(huán)50%1/3胎兒血液循環(huán)途徑通過動脈導管通過卵圓孔血氧含量較高血氧含量較17CirculationChangesafterbirthTheumbilicalcordclampedbreathingcommencesPulmonarycirculationProvidinggasexchange

Theforamenovaleandductusarteriosus:functionallyclosedCirculationChangesafterbirt18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脈血分開卵圓孔、動脈導管、靜脈導管開放卵圓孔、動脈導管、靜脈導管閉合肺動脈壓與主動脈相似，肺循環(huán)阻力高肺動脈壓下降，肺循環(huán)阻力低右心室高負荷左心室高負荷AB返回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19Commonsymtoms

CongestiveheartfailureFeedingdifficulties/ExerciseintoleranceRespiratorydistressPoorgrowthCracked,coughSquattingSyncopeCyanosisCommonsymtomsCongestivehear20三兒童心血管疾病體格檢查方法三兒童心血管疾病體格檢查方法21PhysicalexaminationCommonlymanifestationsEvaluationforBWandBHRespiratorydistressCyanosisClubbingdigitsHepatomegalyDistendedneckveinsSignofliver-jugularveinPeripheraledemaPhysicalexaminationCommonlym22CardiovascularExaminationInspection:ProminenceoftheprecordiumCardiomegaly

Palpation:heave,thrillPercussion:sizeandpositionoftheheartAuscultation:HR,rhythm(1)Normalheartsounds:S1,S2,S3,S4(2)Abnormalheartsounds:P2fixedsplitting(3)MurmursCardiovascularExaminationInsp23小兒聽診常用聽診區(qū)二尖瓣聽診區(qū)肺動脈瓣聽診區(qū)主動脈瓣聽診區(qū)三尖瓣聽診區(qū)小兒聽診常用聽診區(qū)24(1)Locationandradiation(2)Timing:Systolic,diastolicandcontinuous(3)Quality:Harsh,musical,orrough;high, mediumorlowinpitch(4)Intensity:gradeⅠ,Ⅱ,Ⅲ,Ⅳ,Ⅴ,Ⅵ(5)VariationwithpositionMurmur(1)LocationandradiationMurm25周圍血管征四肢脈搏及血壓主動脈縮窄：股動脈搏動減弱或消失下肢血壓低于上肢動脈導管未閉或主動脈關閉不全：脈壓增寬毛細血管搏動股動脈槍擊音周圍血管征四肢脈搏及血壓26四心臟的特殊檢查

(1)Chestx-ray(2)Electrocardiogram(3)Echocardiogram(4)Cardiaccatheterization(5)Angiocardiography(6)Radionuclideangiocardiography(7)Magneticresonanceimaging（8）Computedtomography四心臟的特殊檢查27ChestRadiographcharacterofthepulmonaryvasculaturePositionofthecardiacapexcardiacsize(cardiothoracicratio)cardiacconfiguration,heartshapePositionoftheabdominalvisceraChestRadiographcharacteroft28先心雙語(七年制)_醫(yī)學醫(yī)藥課件29Normalcardiothoracicimaginglandmarks,PAview

Normalcardiothoracicimaging30Anteriorradiographicviewofcardiacanatomy

Anteriorradiographicviewof31X-rayX-ray32你的X線診斷分別是什么？你的X線診斷分別是什么？33ThesequenceofECG:rate,rhythm,Pwave,PRinterval,QRScomplex(axis,amplitude,andduration),QTinterval,STsegment,andTwave.AssessatrioventricularhypertrophyDiagnosisofdysrhythmiasElectrocardiogram,ECGThesequenceofECG:Electroca34先心雙語(七年制)_醫(yī)學醫(yī)藥課件35NormalECGNormalECG36Apowerfulnoninvasivemethod:M-mode,twoandthreedimensional,colorandDopplerEvaluatecardiacstructureEstimateintracardiacpressureandgradientacrossstenoticvalvesandvesselsQuantitatecardiaccontractilefunctionDeterminethedirectionofflowacrossadefectExaminetheintegrityofthecoronaryarteriesEvaluateendocarditis,pericardiacfluid,cardiactumors,orchamberthrombi.EchocardiographyApowerfulnoninvasivemethod:37先心雙語(七年制)_醫(yī)學醫(yī)藥課件38M-modeECHOM-modeECHO39Long-axisviewforLV2-DECHOLong-axisviewforLV2-DECHO40Four-chamberviewatapex2-DECHOFour-chamberviewatapex2-DE41Short-axisviewatthegreatarteryShort-axisviewatthegreata422-DcolorECHO(four-chamberview)2-DcolorECHO(four-chamberv432-DcolorDopplerECHO2-DcolorDopplerECHO443-DReconstruction3-DReconstruction45SVCIVCRVSVCIVCRV46CardiacCatheterizationEstimateCardiacoutputaccordingtoOxygencontentandsaturation,pulmonaryandsystemicbloodflow.DeterminethepressuresinallchambersandvesselsCalculatethepulmonaryandsystemicvascularresistanceInterventionalcatheterization(therapeuticprocedures)20%12~14%30/1212~14%12~14%4~80~5100/1030/5100/60CardiacCatheterizationEstimat47先心雙語(七年制)_醫(yī)學醫(yī)藥課件48Angiocardiography選擇性造影Selectiveangiocardiography數(shù)字減影造影(DSA)診斷復雜先心病的主要手段常用成角造影長軸斜位：左前斜60～75°＋向頭成角20～30°肝鎖位：左前斜40～45°＋向頭成角40°坐觀位：正位向頭成角40°Angiocardiography選擇性造影Selecti49心血管造影心血管造影50心血管造影心血管造影51Radionuclideangiocardiography

99m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估心功能Radionuclideangiocardiography52MagneticresonanceimagingNoninvasivetoolFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxPaulC.Lauterbur,UnitedStates,andSirPeterMansfield,Britain,winedthe2003NobelPrizefordiscoveriesinmagneticresonanceimagingMagneticresonanceimagingNoni53SESE54AOPAAOPA55AOLVLARVCineMRIAOLVLARVCineMRI56Cine-MRICine-MRI57MRAMRA583-DMRIMRIisinvaluableinthelongtermfollow-upofcoarctationoftheaortaafterangioplasty.(surgicalprocedure)

3-DMRIMRIisinvaluableint59ComputedtomographyFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxComputedtomographyFollow-up60第二節(jié)小兒先天性心臟病概述概念：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發(fā)病率:6～8‰，上海6.87‰我國每年出生15萬CHD診治研究取得很大進展，預后大為改觀第二節(jié)小兒先天性心臟病概述概念：CHD是胎兒期心臟及大血61

病因

內(nèi)因：與遺傳有關，染色體異?；蚧蛲蛔儭?/p>

外因：（1）早期宮內(nèi)感染：風疹、流感、腮腺炎、柯薩奇病毒等。（2）孕母接觸大量放射線服用藥物史（抗癌藥、抗癲癇藥)（3）代謝性疾病:糖尿病、高鈣血癥（4）宮內(nèi)慢性缺氧（5）妊娠早期酗酒、吸食毒品環(huán)境因素＋遺傳因素病因內(nèi)因：與遺傳有關，染色體異常或基因突變。62CategoriesofcongenitalheartdiseaseCategoriesofcongenitalheart63Theleft-to-rightshunttype(ASDVSDPDA)AcyanoticearlyPulmonarybloodincreasedSystemicblooddecreasedPulmonaryhypertensionEisenmengersyndrome:cyanoticlatelyduetoright-to-leftshuntsTheleft-to-rightshunttypeAc64Theright-to-leftshunttypeTetralogyofFallot(TOF),transpositionofthegreatarteries(TGA)CyanoticearlyCyanoticlesionswithincreasedpulmonarybloodflowCyanoticlesionswithdecreasedpulmonarybloodflowThesystemiccirculationishypoxemia

Theright-to-leftshunttypeTe65No-shuntlesionsPulmonarystenosis(PS),Coarctationoftheaorta(Coa)AcyanoticHypertrophyresultinginincreasedpressureloadNo-shuntlesionsPulmonarysten66VanPraagh順序分段診斷方法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脈連接診斷心臟位置“三層樓結構”“四層樓結構”VanPraagh順序分段診斷方法心房位置判斷“三層樓結67DiagnosticprocessforCHDTwogroupsbasedonPEacyanoticandcyanoticlesionsFurtherbesubdividedbasedonX-rayincreased,normalordecreasedpulmonaryvascularmarkingsDeterminewhetherright,left,orbiventricularhypertrophyConfirmthediagnosisbyECHOorCatheterization,orboth

DiagnosticprocessforCHDTwo68第三節(jié)幾種常見的先天性心臟病Commoncongenitalheartdiseases第三節(jié)幾種常見的先天性心臟病69先心雙語(七年制)_醫(yī)學醫(yī)藥課件70AtrialSeptaldefect（ASD)AtrialSeptaldefect（ASD)71ASDGeneralconsiderationsDefinition:anopeningintheatrialseptumpermittingtheshuntingofbloodbetweentheatriaIncidence:10%inallCHDThreemajortypes:(1)Theostiumsecundumtype(2)Thesinusvenosustype(3)TheostiumprimumtypeASDGeneralconsiderationsDefi72ASDPathophysiologyThedegreeofL-Rshunt(1)Thesizeofthedefect(2)TherelativecompliancesofRVandLV(3)therelativevascularresistancesinthepulmonaryandsystemiccirculationASDPathophysiologyThedegree73ASDPathophysiologyInneonateandearlyinfant

limitingtheLRshuntSmallASDLargeASDQp:Qs2~4:1PAH：RLshuntASDPathophysiologyInneonate74可在臨床上表現(xiàn)出來ASD分流右心室擴大肺動脈充血肺循環(huán)充血肺動脈高壓肺靜脈動脈血左心房左心室主動脈搏血減少體循環(huán)缺血上下腔靜脈右心房擴大ASD血液循環(huán)途徑可在臨床上表現(xiàn)出來ASD分流右心室擴大肺動脈充血肺循環(huán)充血肺75LVRVLARAWhytheLAisnotenlarged？WhytheLAisnotenlarged？76SmallASD：Most

oftenasymptomaticdiscoveredduringPELargeASD（largeshunt）：(1)Pulmonryblood：repeatedURIorpneumonia(2)Systemicblood：poorgrowth,pale,lackofpower,hidrosis,tachypneaafterexercise(3)Pulmonaryhypertension,Eisenmenggersyndrome（cyanosis）

ASDClinicalmanifestationSmallASD：Mostoftenasymptom77ASDCardiacsignInspection:

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

fixedsplittingofthe2ndheartsoundLowerLSBdiastolicmurmurEarlysystolicclick,P2accentuated(PAH)ASDCardiacsignInspection:le78P2亢進伴固定分裂，喀喇音ASDⅡ-Ⅲ/ⅥSMP2亢進伴固定分裂，喀喇音ASDⅡ-Ⅲ/ⅥSM79ASDECGVolumeoverloadoftheRV:QRSaxis:normalorrightaxisdeviationwithaclockwiseloopinthefrontalplaneRVhypertrophyAminorrightventricularconductiondelayP-RdelayIntherightprecordialleads,arsR’patternASDECGVolumeoverloadofthe80ASDECG

IAVBASDECG

IAVB81ASDX-rayThepulmonaryvascularityincreasedEnlargementoftheRVandRAThepulmonaryarterydilated

ASDX-rayThepulmonaryvascu82Mmode:RVvolu