先心双语(七年制)-医学医药课件

庞玉生授课对象：本科（七年制）广西医科大学儿科学教研室Chapter13循环系统疾病Circulationsystemdisease庞玉生授课对象：本科（七年制）Chapter13循环系统1教学目的

初步熟悉、了解先天性心脏病掌握先天性心脏病的诊断和治疗教学重点

详细介绍先天性心脏病的临床表现和诊断方法教学难点

先天性心脏病血液动力学改变与临床表现的关系教学目的2Contents小儿心血管系统解剖生理特点及检查方法小儿先天性心脏病概述房间隔缺损室间隔缺损动脉导管未闭法洛四联症Contents小儿心血管系统解剖生理特点及检查方法3第一节小儿心血管系统解剖生理

特点及检查方法第一节小儿心血管系统解剖生理

特点及检查方法4一心脏的胚胎发育

Cardiacdevelopmentduringfetalperiod

Theprimitivehearttubeformsby3W

Cardiacloopingby22~24daysBendventrallyandtowardtherightCardiacseptation一心脏的胚胎发育

Cardiacdevelopment5第一隔原发孔心内膜垫静脉窦瓣膜第二隔继发孔第一隔室间孔第二隔继发孔第一隔卵园孔第一隔室间孔第二隔静脉窦瓣膜胚胎房室分隔及房室间隔发育第一隔原发孔心内膜垫静脉窦瓣膜第二隔继发孔第一隔室间孔第二隔6室间隔形成

心室间隔组成三个来源

①肌隔

②心内膜垫向下生长与肌隔相合

③动脉总干及心球分化成主动脉与肺动脉时的中隔向下延伸部分室间隔膜部室间隔形成

心室间隔组成三个来源

①肌隔

7AorticarchdevelopmentRightandlefttruncoconalridgesTheaorticandpulmonaryoutflowtractsarefullyseparatedThecoronarysinusThesemilunarvalvesAorticarchdevelopmentRighta8主肺动脉由总干呈螺旋形分隔AOPAPAAOPAPAAOAO主肺动脉由总干呈螺旋形分隔AOPAPAAOPAPAAOAO9PAPAPAPA大动脉位置变移系列示意图TOFTGADORVTaussig-BingPAPAPAPA大动脉位置变移系列示意图TOFTGADORV10房室管、心房、及心室的分隔室间隔膜部的形成及室间隔的封闭第2周原始心脏形成第4周心脏起循环作用第8周四腔心脏形成房室管、心房、室间隔膜部的形成及室间隔的封闭第2周原始心脏11正常血液循环途径正常血液循环途径12二胎儿新生儿循环转换二胎儿新生儿循环转换13Theplacenta:gasexchange,metabolicexchangeThelung:constrictedThreecardiovascularstructure:DuctusvenosusForamenovaleDuctusarteriosus

TheFetalCirculationTheplacenta:TheFetalCircula14胎儿血循环特点营养和气体代谢通过脐血管和胎盘与母体进行弥漫式交换绝大部分为混合血供应心脏、脑、肝及上肢血氧含量远较下半身高肺处于压缩状态无气体交换卵圆孔、动脉导管和静脉导管开放右心室优势胎儿血循环特点营养和气体代谢通过脐血管和胎盘与母体进行弥漫式15TheFetalCirculationTheFetalCirculation16胎儿血液循环途径通过动脉导管通过卵圆孔血氧含量较高血氧含量较低通过静脉导管下腔静脉右心房右心室左心房左心室升主动脉心脑及上肢肺动脉降主动脉下半身上半身静脉血上腔静脉脐静脉动脉血门静脉静脉血母体下半身静脉血肺循环50%1/3胎儿血液循环途径通过动脉导管通过卵圆孔血氧含量较高血氧含量较17CirculationChangesafterbirthTheumbilicalcordclampedbreathingcommencesPulmonarycirculationProvidinggasexchange

Theforamenovaleandductusarteriosus:functionallyclosedCirculationChangesafterbirt18胎儿与出生后血液循环比较A胎儿期B出生后由母体循环完成气体交换由肺循环完成气体交换多为混合血，心、脑、上半身血氧含量高于下半身静脉血和动脉血分开卵圆孔、动脉导管、静脉导管开放卵圆孔、动脉导管、静脉导管闭合肺动脉压与主动脉相似，肺循环阻力高肺动脉压下降，肺循环阻力低右心室高负荷左心室高负荷AB返回胎儿与出生后血液循环比较A胎儿期B出生后由母体循环完成气体交19Commonsymtoms

CongestiveheartfailureFeedingdifficulties/ExerciseintoleranceRespiratorydistressPoorgrowthCracked,coughSquattingSyncopeCyanosisCommonsymtomsCongestivehear20三儿童心血管疾病体格检查方法三儿童心血管疾病体格检查方法21PhysicalexaminationCommonlymanifestationsEvaluationforBWandBHRespiratorydistressCyanosisClubbingdigitsHepatomegalyDistendedneckveinsSignofliver-jugularveinPeripheraledemaPhysicalexaminationCommonlym22CardiovascularExaminationInspection:ProminenceoftheprecordiumCardiomegaly

Palpation:heave,thrillPercussion:sizeandpositionoftheheartAuscultation:HR,rhythm(1)Normalheartsounds:S1,S2,S3,S4(2)Abnormalheartsounds:P2fixedsplitting(3)MurmursCardiovascularExaminationInsp23小儿听诊常用听诊区二尖瓣听诊区肺动脉瓣听诊区主动脉瓣听诊区三尖瓣听诊区小儿听诊常用听诊区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周围血管征四肢脉搏及血压主动脉缩窄：股动脉搏动减弱或消失下肢血压低于上肢动脉导管未闭或主动脉关闭不全：脉压增宽毛细血管搏动股动脉枪击音周围血管征四肢脉搏及血压26四心脏的特殊检查

(1)Chestx-ray(2)Electrocardiogram(3)Echocardiogram(4)Cardiaccatheterization(5)Angiocardiography(6)Radionuclideangiocardiography(7)Magneticresonanceimaging（8）Computedtomography四心脏的特殊检查27ChestRadiographcharacterofthepulmonaryvasculaturePositionofthecardiacapexcardiacsize(cardiothoracicratio)cardiacconfiguration,heartshapePositionoftheabdominalvisceraChestRadiographcharacteroft28先心双语(七年制)_医学医药课件29Normalcardiothoracicimaginglandmarks,PAview

Normalcardiothoracicimaging30Anteriorradiographicviewofcardiacanatomy

Anteriorradiographicviewof31X-rayX-ray32你的X线诊断分别是什么？你的X线诊断分别是什么？33ThesequenceofECG:rate,rhythm,Pwave,PRinterval,QRScomplex(axis,amplitude,andduration),QTinterval,STsegment,andTwave.AssessatrioventricularhypertrophyDiagnosisofdysrhythmiasElectrocardiogram,ECGThesequenceofECG:Electroca34先心双语(七年制)_医学医药课件35NormalECGNormalECG36Apowerfulnoninvasivemethod:M-mode,twoandthreedimensional,colorandDopplerEvaluatecardiacstructureEstimateintracardiacpressureandgradientacrossstenoticvalvesandvesselsQuantitatecardiaccontractilefunctionDeterminethedirectionofflowacrossadefectExaminetheintegrityofthecoronaryarteriesEvaluateendocarditis,pericardiacfluid,cardiactumors,orchamberthrombi.EchocardiographyApowerfulnoninvasivemethod:37先心双语(七年制)_医学医药课件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先心双语(七年制)_医学医药课件48Angiocardiography选择性造影Selectiveangiocardiography数字减影造影(DSA)诊断复杂先心病的主要手段常用成角造影长轴斜位：左前斜60～75°＋向头成角20～30°肝锁位：左前斜40～45°＋向头成角40°坐观位：正位向头成角40°Angiocardiography选择性造影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释放γ射线初次循环心脏造影、平衡心脏血池造影检测左向右分流评估心功能Radionuclideangiocardiography52MagneticresonanceimagingNoninvasivetoolFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxPaulC.Lauterbur,UnitedStates,andSirPeterMansfield,Britain,winedthe2003NobelPrizefordiscoveriesinmagneticresonanceimagingMagneticresonanceimagingNoni53SESE54AOPAAOPA55AOLVLARVCineMRIAOLVLARVCineMRI56Cine-MRICine-MRI57MRAMRA583-DMRIMRIisinvaluableinthelongtermfollow-upofcoarctationoftheaortaafterangioplasty.(surgicalprocedure)

3-DMRIMRIisinvaluableint59ComputedtomographyFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxComputedtomographyFollow-up60第二节小儿先天性心脏病概述概念：CHD是胎儿期心脏及大血管发育异常而致的心血管畸形，是小儿最常见的心脏病发病率:6～8‰，上海6.87‰我国每年出生15万CHD诊治研究取得很大进展，预后大为改观第二节小儿先天性心脏病概述概念：CHD是胎儿期心脏及大血61

病因

内因：与遗传有关，染色体异常或基因突变。

外因：（1）早期宫内感染：风疹、流感、腮腺炎、柯萨奇病毒等。（2）孕母接触大量放射线服用药物史（抗癌药、抗癫痫药)（3）代谢性疾病:糖尿病、高钙血症（4）宫内慢性缺氧（5）妊娠早期酗酒、吸食毒品环境因素＋遗传因素病因内因：与遗传有关，染色体异常或基因突变。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顺序分段诊断方法心房位置判断心室位置判断大血管位置判断房室连接诊断心室大动脉连接诊断心脏位置“三层楼结构”“四层楼结构”VanPraagh顺序分段诊断方法心房位置判断“三层楼结67DiagnosticprocessforCHDTwogroupsbasedonPEacyanoticandcyanoticlesionsFurtherbesubdividedbasedonX-rayincreased,normalordecreasedpulmonaryvascularmarkingsDeterminewhetherright,left,orbiventricularhypertrophyConfirmthediagnosisbyECHOorCatheterization,orboth

DiagnosticprocessforCHDTwo68第三节几种常见的先天性心脏病Commoncongenitalheartdiseases第三节几种常见的先天性心脏病69先心双语(七年制)_医学医药课件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可在临床上表现出来ASD分流右心室扩大肺动脉充血肺循环充血肺动脉高压肺静脉动脉血左心房左心室主动脉搏血减少体循环缺血上下腔静脉右心房扩大ASD血液循环途径可在临床上表现出来ASD分流右心室扩大肺动脉充血肺循环充血肺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亢进伴固定分裂，喀喇音ASDⅡ-Ⅲ/ⅥSMP2亢进伴固定分裂，喀喇音ASDⅡ-Ⅲ/ⅥSM79ASDECGVolumeoverloadoftheRV:QRSaxis:normalorrightaxisdeviationwithaclockwiseloopinthefrontalplaneRVhypertrophyAminorrightventricularconductiondelayP-RdelayIntherightprecordialleads,arsR’patternASDECGVolumeoverloadofthe80ASDECG

IAVBASDECG

IAVB81ASDX-rayThepulmonaryvascularityincreasedEnlargementoftheRVandRAThepulmonaryarterydilated

ASDX-rayThepulmonaryvascu82Mmode:RVvolumeoverload2-D：sizeColor：directionDoppler：pressure3-D：ASD3-DASDECHOMmode:ASDECHO83ASDcatheterization

OxygencontentinRA:higherThecatheter:RAtoLAthroughASDThePApressure:usuallynormalpulmonaryvascularresistanceQp:Qs:

1.5:1to4:1.ASDcatheterizationOxy84ASDcatheterizationASDcatheterization85ASDPrognosisandComplicationsPneumoniaHeartfailureInfectiveendocarditisPulmonaryhypertensionAtrialarrhythmiaTricuspidregurgitationASDPrognosisandComplication86ASDtreatmentSurgicalrepair:2~6yrsEarlysurgery:InfantswithCHForsignificantpulmonaryhypertension,“mini”sternotomyposteriorthoracotomyapproachClosurewithdevice(Amplazer)ASDtreatmentSurgicalrepair:87ASD封堵适应证(Amplatzer)年龄：通常≥3岁，<60岁，体重>8kg继发孔型左向右分流ASD直径<30mm缺损边缘至肺静脉、腔静脉、二尖瓣口及冠状静脉窦口的距离>5mm房间隔的伸展径大于房缺直径14mm以上不合并必须外科手术的其他心脏畸形外科术后残余分流

ASD封堵适应证(Amplatzer)年龄：通常≥3岁，<688ASD封堵禁忌症明显紫绀，肺动脉高压，右向左分流；部分或完全肺静脉畸形引流；筛网状、冠状窦型及多发ASD；左心房发育不良；复杂型先天性心脏病；全身感染，出凝血功能障碍，阿司匹林禁忌。ASD封堵禁忌症明显紫绀，肺动脉高压，右向左分流；89自膨胀双伞镍钛合金网结构；左房侧比右房侧大4mm；腰部为3层膜结构，直径即封堵器大小，腰长3~4mm。ASD封堵器自膨胀双伞镍钛合金网结构；ASD封堵器90ClosureprocedureClosureprocedure91庞玉生授课对象：本科（七年制）广西医科大学儿科学教研室Chapter13循环系统疾病Circulationsystemdisease庞玉生授课对象：本科（七年制）Chapter13循环系统92教学目的

初步熟悉、了解先天性心脏病掌握先天性心脏病的诊断和治疗教学重点

详细介绍先天性心脏病的临床表现和诊断方法教学难点

先天性心脏病血液动力学改变与临床表现的关系教学目的93Contents小儿心血管系统解剖生理特点及检查方法小儿先天性心脏病概述房间隔缺损室间隔缺损动脉导管未闭法洛四联症Contents小儿心血管系统解剖生理特点及检查方法94第一节小儿心血管系统解剖生理

特点及检查方法第一节小儿心血管系统解剖生理

特点及检查方法95一心脏的胚胎发育

Cardiacdevelopmentduringfetalperiod

Theprimitivehearttubeformsby3W

Cardiacloopingby22~24daysBendventrallyandtowardtherightCardiacseptation一心脏的胚胎发育

Cardiacdevelopment96第一隔原发孔心内膜垫静脉窦瓣膜第二隔继发孔第一隔室间孔第二隔继发孔第一隔卵园孔第一隔室间孔第二隔静脉窦瓣膜胚胎房室分隔及房室间隔发育第一隔原发孔心内膜垫静脉窦瓣膜第二隔继发孔第一隔室间孔第二隔97室间隔形成

心室间隔组成三个来源

①肌隔

②心内膜垫向下生长与肌隔相合

③动脉总干及心球分化成主动脉与肺动脉时的中隔向下延伸部分室间隔膜部室间隔形成

心室间隔组成三个来源

①肌隔

98AorticarchdevelopmentRightandlefttruncoconalridgesTheaorticandpulmonaryoutflowtractsarefullyseparatedThecoronarysinusThesemilunarvalvesAorticarchdevelopmentRighta99主肺动脉由总干呈螺旋形分隔AOPAPAAOPAPAAOAO主肺动脉由总干呈螺旋形分隔AOPAPAAOPAPAAOAO100PAPAPAPA大动脉位置变移系列示意图TOFTGADORVTaussig-BingPAPAPAPA大动脉位置变移系列示意图TOFTGADORV101房室管、心房、及心室的分隔室间隔膜部的形成及室间隔的封闭第2周原始心脏形成第4周心脏起循环作用第8周四腔心脏形成房室管、心房、室间隔膜部的形成及室间隔的封闭第2周原始心脏102正常血液循环途径正常血液循环途径103二胎儿新生儿循环转换二胎儿新生儿循环转换104Theplacenta:gasexchange,metabolicexchangeThelung:constrictedThreecardiovascularstructure:DuctusvenosusForamenovaleDuctusarteriosus

TheFetalCirculationTheplacenta:TheFetalCircula105胎儿血循环特点营养和气体代谢通过脐血管和胎盘与母体进行弥漫式交换绝大部分为混合血供应心脏、脑、肝及上肢血氧含量远较下半身高肺处于压缩状态无气体交换卵圆孔、动脉导管和静脉导管开放右心室优势胎儿血循环特点营养和气体代谢通过脐血管和胎盘与母体进行弥漫式106TheFetalCirculationTheFetalCirculation107胎儿血液循环途径通过动脉导管通过卵圆孔血氧含量较高血氧含量较低通过静脉导管下腔静脉右心房右心室左心房左心室升主动脉心脑及上肢肺动脉降主动脉下半身上半身静脉血上腔静脉脐静脉动脉血门静脉静脉血母体下半身静脉血肺循环50%1/3胎儿血液循环途径通过动脉导管通过卵圆孔血氧含量较高血氧含量较108CirculationChangesafterbirthTheumbilicalcordclampedbreathingcommencesPulmonarycirculationProvidinggasexchange

Theforamenovaleandductusarteriosus:functionallyclosedCirculationChangesafterbirt109胎儿与出生后血液循环比较A胎儿期B出生后由母体循环完成气体交换由肺循环完成气体交换多为混合血，心、脑、上半身血氧含量高于下半身静脉血和动脉血分开卵圆孔、动脉导管、静脉导管开放卵圆孔、动脉导管、静脉导管闭合肺动脉压与主动脉相似，肺循环阻力高肺动脉压下降，肺循环阻力低右心室高负荷左心室高负荷AB返回胎儿与出生后血液循环比较A胎儿期B出生后由母体循环完成气体交110Commonsymtoms

CongestiveheartfailureFeedingdifficulties/ExerciseintoleranceRespiratorydistressPoorgrowthCracked,coughSquattingSyncopeCyanosisCommonsymtomsCongestivehear111三儿童心血管疾病体格检查方法三儿童心血管疾病体格检查方法112PhysicalexaminationCommonlymanifestationsEvaluationforBWandBHRespiratorydistressCyanosisClubbingdigitsHepatomegalyDistendedneckveinsSignofliver-jugularveinPeripheraledemaPhysicalexaminationCommonlym113CardiovascularExaminationInspection:ProminenceoftheprecordiumCardiomegaly

Palpation:heave,thrillPercussion:sizeandpositionoftheheartAuscultation:HR,rhythm(1)Normalheartsounds:S1,S2,S3,S4(2)Abnormalheartsounds:P2fixedsplitting(3)MurmursCardiovascularExaminationInsp114小儿听诊常用听诊区二尖瓣听诊区肺动脉瓣听诊区主动脉瓣听诊区三尖瓣听诊区小儿听诊常用听诊区115(1)Locationandradiation(2)Timing:Systolic,diastolicandcontinuous(3)Quality:Harsh,musical,orrough;high, mediumorlowinpitch(4)Intensity:gradeⅠ,Ⅱ,Ⅲ,Ⅳ,Ⅴ,Ⅵ(5)VariationwithpositionMurmur(1)LocationandradiationMurm116周围血管征四肢脉搏及血压主动脉缩窄：股动脉搏动减弱或消失下肢血压低于上肢动脉导管未闭或主动脉关闭不全：脉压增宽毛细血管搏动股动脉枪击音周围血管征四肢脉搏及血压117四心脏的特殊检查

(1)Chestx-ray(2)Electrocardiogram(3)Echocardiogram(4)Cardiaccatheterization(5)Angiocardiography(6)Radionuclideangiocardiography(7)Magneticresonanceimaging（8）Computedtomography四心脏的特殊检查118ChestRadiographcharacterofthepulmonaryvasculaturePositionofthecardiacapexcardiacsize(cardiothoracicratio)cardiacconfiguration,heartshapePositionoftheabdominalvisceraChestRadiographcharacteroft119先心双语(七年制)_医学医药课件120Normalcardiothoracicimaginglandmarks,PAview

Normalcardiothoracicimaging121Anteriorradiographicviewofcardiacanatomy

Anteriorradiographicviewof122X-rayX-ray123你的X线诊断分别是什么？你的X线诊断分别是什么？124ThesequenceofECG:rate,rhythm,Pwave,PRinterval,QRScomplex(axis,amplitude,andduration),QTinterval,STsegment,andTwave.AssessatrioventricularhypertrophyDiagnosisofdysrhythmiasElectrocardiogram,ECGThesequenceofECG:Electroca125先心双语(七年制)_医学医药课件126NormalECGNormalECG127Apowerfulnoninvasivemethod:M-mode,twoandthreedimensional,colorandDopplerEvaluatecardiacstructureEstimateintracardiacpressureandgradientacrossstenoticvalvesandvesselsQuantitatecardiaccontractilefunctionDeterminethedirectionofflowacrossadefectExaminetheintegrityofthecoronaryarteriesEvaluateendocarditis,pericardiacfluid,cardiactumors,orchamberthrombi.EchocardiographyApowerfulnoninvasivemethod:128先心双语(七年制)_医学医药课件129M-modeECHOM-modeECHO130Long-axisviewforLV2-DECHOLong-axisviewforLV2-DECHO131Four-chamberviewatapex2-DECHOFour-chamberviewatapex2-DE132Short-axisviewatthegreatarteryShort-axisviewatthegreata1332-DcolorECHO(four-chamberview)2-DcolorECHO(four-chamberv1342-DcolorDopplerECHO2-DcolorDopplerECHO1353-DReconstruction3-DReconstruction136SVCIVCRVSVCIVCRV137CardiacCatheterizationEstimateCardiacoutputaccordingtoOxygencontentandsaturation,pulmonaryandsystemicbloodflow.DeterminethepressuresinallchambersandvesselsCalculatethepulmonaryandsystemicvascularresistanceInterventionalcatheterization(therapeuticprocedures)20%12~14%30/1212~14%12~14%4~80~5100/1030/5100/60CardiacCatheterizationEstimat138先心双语(七年制)_医学医药课件139Angiocardiography选择性造影Selectiveangiocardiography数字减影造影(DSA)诊断复杂先心病的主要手段常用成角造影长轴斜位：左前斜60～75°＋向头成角20～30°肝锁位：左前斜40～45°＋向头成角40°坐观位：正位向头成角40°Angiocardiography选择性造影Selecti140心血管造影心血管造影141心血管造影心血管造影142Radionuclideangiocardiography

99m锝iv释放γ射线初次循环心脏造影、平衡心脏血池造影检测左向右分流评估心功能Radionuclideangiocardiography143MagneticresonanceimagingNoninvasivetoolFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxPaulC.Lauterbur,UnitedStates,andSirPeterMansfield,Britain,winedthe2003NobelPrizefordiscoveriesinmagneticresonanceimagingMagneticresonanceimagingNoni144SESE145AOPAAOPA146AOLVLARVCineMRIAOLVLARVCineMRI147Cine-MRICine-MRI148MRAMRA1493-DMRIMRIisinvaluableinthelongtermfollow-upofcoarctationoftheaortaafterangioplasty.(surgicalprocedure)

3-DMRIMRIisinvaluableint150ComputedtomographyFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxComputedtomographyFollow-up151第二节小儿先天性心脏病概述概念：CHD是胎儿期心脏及大血管发育异常而致的心血管畸形，是小儿最常见的心脏病发病率:6～8‰，上海6.87‰我国每年出生15万CHD诊治研究取得很大进展，预后大为改观第二节小儿先天性心脏病概述概念：CHD是胎儿期心脏及大血152

病因

内因：与遗传有关，染色体异常或基因突变。

外因：（1）早期宫内感染：风疹、流感、腮腺炎、柯萨奇病毒等。（2）孕母接触大量放射线服用药物史（抗癌药、抗癫痫药)（3）代谢性疾病:糖尿病、高钙血症（4）宫内慢性缺氧（5）妊娠早期酗酒、吸食毒品环境因素＋遗传因素病因内因：与遗传有关，染色体异常或基因突变。153CategoriesofcongenitalheartdiseaseCategoriesofcongenitalheart154Theleft-to-rightshunttype(ASDVSDPDA)AcyanoticearlyPulmonarybloodincreasedSystemicblooddecreasedPulmonaryhypertensionEisenmengersyndrome:cyanoticlatelyduetoright-to-leftshuntsTheleft-to-rightshunttypeAc155Theright-to-leftshunttypeTetralogyofFallot(TOF),transpositionofthegreatarteries(TGA)CyanoticearlyCyanoticlesionswithincreasedpulmonarybloodflowCyanoticlesionswithdecreasedpulmonarybloodflowThesystemiccirculationishypoxemia

Theright-to-leftshunttypeTe156No-shuntlesionsPulmonarystenosis(PS),Coarctationoftheaorta(Coa)AcyanoticHypertrophyresultinginincreasedpressureloadNo-shuntlesionsPulmonarysten157VanPraagh顺序分段诊断方法心房位置判断心室位置判断大血管位置判断房室连接诊断心室大动脉连接诊断心脏位置“三层楼结构”“四层楼结构”VanPraagh顺序分段诊断方法心房位置判断“三层楼结158DiagnosticprocessforCHDTwogroupsbasedonPEacyanoticandcyanoticlesionsFurtherbesubdividedbasedonX-rayincreased,normalordecreasedpulmonaryvascularmarkingsDeterminewhetherright,left,orbiventricularhypertrophyConfirmthediagnosisbyECHOorCatheterization,orboth

DiagnosticprocessforCHDTwo159第三节几种常见的先天性心脏病Commoncongenitalheartdiseases第三节几种常见的先天性心脏病160先心双语(七年制)_医学医药课件161AtrialSeptaldefect（ASD)AtrialSeptaldefect（ASD)162ASDGeneralconsiderationsDefinition:anopeningintheatrialseptumpermittingtheshuntingofbloodbetweentheatriaIncidence:10%inallCHDThreemajortypes:(1)Theostiumsecundumtype(2)Thesinusvenosustype(3)TheostiumprimumtypeASDGeneralconsiderationsDefi163ASDPathophysiologyThedegreeofL-Rshunt(1)Thesizeofthedefect(2)TherelativecompliancesofRVandLV(3)therelativevascularresistancesinthepulmonaryandsystemiccirculationASDPathophysiologyThedegree164ASDPathophysiologyInneonateandearlyinfant

limitingtheLRshuntSmallASDLargeASDQp:Qs2~4:1PAH：RLshuntASDPathophysiologyInneonate165可在临床上表现出来ASD分流右心室扩大肺动脉充血肺循环充血肺动脉高压肺静脉动脉血左心房左心室主动脉搏血减少体循环缺血上下腔静脉右心房扩大ASD血液循环途径可在临床上表现出来ASD分流右心室扩大肺动脉充血肺循环充血肺166LVRVLARAWhytheLAisnotenlarged？WhytheLAisnotenlarged？167SmallASD：Most

oftenasymptomaticdiscoveredduringPELargeASD（large