先心双语(七年制)-广西医科大学医学医药

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目的先天性心脏病血液动力学改变与临床表现的关系教学难点教学重点详细介绍先天性心脏病的临床表现和诊断方法Contents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第一节小儿心血管系统解剖生理

特点及检查方法一心脏的胚胎发育

Cardiacdevelopmentduringfetalperiod

Theprimitivehearttubeformsby3W

Cardiacloopingby22~24daysBendventrallyandtowardtherightCardiacseptation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室间隔形成心室间隔组成三个来源

肌隔

心内膜垫向下生长与肌隔相合

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AorticarchdevelopmentRightandlefttruncoconalridgesTheaorticandpulmonaryoutflowtractsarefullyseparatedThecoronarysinusThesemilunarvalves主肺动脉由总干呈螺旋形分隔AOPAPAAOPAPAAOAO大动脉位置变移系列示意图PAPAPAPATOFTGADORVTaussig-Bing

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周四腔心脏形成正常血液循环途径二胎儿新生儿循环转换Theplacenta:gasexchange,metabolicexchangeThelung:constrictedThreecardiovascularstructure:DuctusvenosusForamenovaleDuctusarteriosusTheFetalCirculation01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02绝大部分为混合血03供应心脏、脑、肝及上肢血氧含量远较下半身高04肺处于压缩状态无气体交换05卵圆孔、动脉导管和静脉导管开放06右心室优势胎儿血循环特点TheFetalCirculation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%1/3CirculationChangesafterbirthTheumbilicalcordclampedbreathingcommencesPulmonarycirculationProvidinggasexchangeTheforamenovaleandductusarteriosus:functionallyclosed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CommonsymtomsCongestiveheartfailureFeedingdifficulties/ExerciseintoleranceRespiratorydistressPoorgrowthCracked,coughSquattingSyncopeCyanosis三儿童心血管疾病体格检查方法PhysicalexaminationCommonlymanifestationsEvaluationforBWandBHRespiratorydistressCyanosisClubbingdigitsHepatomegalyDistendedneckveinsSignofliver-jugularveinPeripheraledemaCardiovascularExaminationInspection:ProminenceoftheprecordiumCardiomegaly

Palpation:heave,thrillPercussion:sizeandpositionoftheheartAuscultation:HR,rhythm(1)Normalheartsounds:S1,S2,S3,S4(2)Abnormalheartsounds:P2fixedsplitting(3)Murmurs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LocationandradiationTiming:Systolic,diastolicandcontinuousQuality:Harsh,musical,orrough;high, mediumorlowinpitchIntensity:gradeⅠ,Ⅱ,Ⅲ,Ⅳ,Ⅴ,ⅥVariationwithpositionMurmur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四肢脉搏及血压周围血管征Chestx-ray1Electrocardiogram2Echocardiogram3Cardiaccatheterization4Angiocardiography5Radionuclideangiocardiography6Magneticresonanceimaging7Computedtomography8四心脏的特殊检查characterofthepulmonaryvasculature1Positionofthecardiacapex2cardiacsize(cardiothoracicratio)3cardiacconfiguration,heartshape4Positionoftheabdominalviscera5ChestRadiographNormalcardiothoracicimaginglandmarks,PAviewAnteriorradiographicviewofcardiacanatomyX-ray你的X线诊断分别是什么？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选择性造影Selectiveangiocardiography1数字减影造影(DSA)2诊断复杂先心病的主要手段3常用成角造影4长轴斜位：左前斜60～75°＋向头成角20～30°5肝锁位：左前斜40～45°＋向头成角40°6坐观位：正位向头成角40°7Angiocardiography心血管造影心血管造影Radionuclideangiocardiography99m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MagneticresonanceimagingPaulC.Lauterbur,UnitedStates,andSirPeterMansfield,Britain,winedthe2003NobelPrizefordiscoveriesinmagneticresonanceimaging04Providetheimagingofvascularstructuresofthethorax05Noninvasivetool01Evaluatetheheartandthegreatarteries03Follow-up02SEAOPAAOLVLARVCineMRICine-MRIMRAMRIisinvaluableinthelongtermfollow-upofcoarctationoftheaortaafterangioplasty.(surgicalprocedure)3-DMRIComputedtomographyFollow-upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethorax010302A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B发病率:6～8‰，上海6.87‰C我国每年出生15万CHDD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病因内因：与遗传有关，染色体异常或基因突变。外因：早期宫内感染：风疹、流感、腮腺炎、柯萨奇病毒等。孕母接触大量放射线服用药物史（抗癌药、抗癫痫药)代谢性疾病:糖尿病、高钙血症宫内慢性缺氧妊娠早期酗酒、吸食毒品环境因素＋遗传因素CategoriesofcongenitalheartdiseaseTheleft-to-rightshunttype(ASDVSDPDA)AcyanoticearlyPulmonarybloodincreasedSystemicblooddecreasedPulmonaryhypertensionEisenmengersyndrome:cyanoticlatelyduetoright-to-leftshuntsTheright-to-leftshunttypeTetralogyofFallot(TOF),transpositionofthegreatarteries(TGA)CyanoticearlyCyanoticlesionswithincreasedpulmonarybloodflow12345ThesystemiccirculationishypoxemiaCyanoticlesionswithdecreasedpulmonarybloodflowPulmonarystenosis(PS),Coarctationoftheaorta(Coa)AcyanoticHypertrophyresultinginincreasedpressureloadNo-shuntlesionsVanPraagh顺序分段诊断方法“三层楼结构”“四层楼结构”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DiagnosticprocessforCHDTwogroupsbasedonPEacyanoticandcyanoticlesionsFurtherbesubdividedbasedonX-rayincreased,normalordecreasedpulmonaryvascularmarkingsDeterminewhetherright,left,orbiventricularhypertrophyConfirmthediagnosisbyECHOorCatheterization,orboth第三节几种常见的先天性心脏病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亢进伴固定分裂，喀喇音QRSaxis:normalorrightaxisdeviationwithaclockwiseloopinthefrontalplaneRVhypertrophyAminorrightventricularconductiondelayVolumeoverloadoftheRV:01Intherightprecordialleads,arsR’patternP-Rdelay02ASDECGIAVBASDECGASDX-rayThepulmonaryvascularityincreasedEnlargementoftheRVandRAThepulmonaryarterydilatedMmode:RVvolumeoverloa