先天性心脏病(英文版) 课件

CongenitalHeartDisease

(CHD)IntroductionCHD,themostcommoncardiacdiseaseinchildren先天性心臟病,兒童最常見的心臟疾病Criticaltimeofembryoticcardiacdevelopment:secondtoeighthweekofgestation心臟發育的危險期:第二至第八周妊娠Incidence:7～8/1000發生率CHDresultfrominteractionbetweengeneticandenvironmentalfactorsCHD是遺傳和環境因素相互作用的結果Geneticfactors:singlemutantgenes/chromosomalabnormalities/multifactorialgenefactors遺傳因素:單一突變基因染色體異常/多因數的基因因素Environmentalfactors:fetalenvironment/viralinfection環境因素:胎兒的環境/病毒感染Etiology

Classification

AccordingtoHemodynamics:根據血液動力學:left-to-rightshunts(withoutcyanosis)

egVSD,ASD,PDA,etal左向右分流型right-to-leftshunts(withcyanosis)egTOF,TGA,etal右向左分流型noshuntatall(withoutcyanosis)

egPS,AS,etalVentricularSeptalDefect(VSD)AtriaSeptalDefect(ASD)PatentDuctusArteriosus(PDA)TetralogyofFallot(TOF)

VentricularSeptalDefect室間隔缺損(VSD)Outline

Incidenceandrateofnaturalclosure發病率和自然關閉Pathologicalanatomy病理解剖學PathophysiologyandHemodynamics病理生理學和血液動力學ClinicalManifestations臨床表現AssistantExaminationes輔助檢查Electrocardiogram/X-ray/Echocardiography/CardiacCatheterizationandAngiocardiography心電圖、x射線、超聲心動圖、心導管和心血管造影術Complications併發症Treatment治療

IncidenceandrateofnaturalclosureVSDisthemostcommonofallCHD,accountingforapproximately30to60%ofallfull-termnewbornwithCHD.室間隔缺損是最常見,占足月新生兒中患先天性心臟病約30-60%Mosttinymuscularandperimembranousdefects（＜0.5cm）havehighchanceofspontaneousclosurewithin6to12monthsafterbirth.肌部小梁部缺損和膜周部缺損缺陷(<0.5釐米)高的機會自然出生後6-12個月內關閉。Pathologicalanatomy

Supracristal脊上的perimembranous：60-70%膜周部Musculardefect：肌肉缺陷

PathophysiologyandHemodynamicsSmalldefect(diameter<0.5cm)，smallshuntandnoobviousabnormalityinhemodynamicsModeratedefect(0.5～1.0cm)，lefttorightshunt,pulmonaryflowincreased分流、肺流量增加，LA、LVenlargement擴大,ordoubleventricleenlargementhypertension雙心室擴大高血壓inRV,PA

Largedefect(>1.0cm)，shunt/pulmonaryflow>60％，LV,RVenlargement，pulmonaryhypertension肺動脈高血壓doubleshuntorRtoL，finally,Eisenmenger’ssyndromEisenmenger的症侯群PathophysiologyandHemodynamicsLA,LVenlargementandhypertrophy左房左室擴大和肥RVflowincreased、enlargement、hypertrophy右室流增加,擴大,肥大Pulmonarycirculationflowincreased肺循環流量增加Systemiccirculationflowinsufficiency體循環流量不足ClinicalManifestationsTwodeterminantsforclinicalmanifestationofisolatedVSD

sizeofdefect缺陷的大小volumeofshunt分流量（心室間壓差）ClinicalManifestationsSmallVSD:noobvioussymptomsModerateVSD:

Symptoms:shortness

ofbreathaftercryingorsuckingininfant;dyspneaafterexercise,heart-throb(心悸),chestdistress,growingdevelopmentbackward,andrespiratorytractinfectionrepeatedlyinthesenior.氣短在嬰兒哭泣或吸吮後,運動後呼吸困難,心悸,胸悶,生長發育落後,在年長中反復呼吸道感染。ClinicalManifestationsSigns

inspection:precordiumhunch(隆起),apexbeatdiffusion視查：心前區隆起,心尖搏動擴散palpation:systolicthrill（震顫）inL3-4觸診：肋3-4收縮期震顫percuss:heartboundaryexpandinbilateral叩診:心界擴大雙邊auscultation:3-4/6systolicmurmurinL3-4P2strengthen/hyperfunction聽診:3-4/6的收縮期雜音L3-4P2（肺動脈第二心音）加強/機能亢進

ClinicalManifestations

LargeVSDwithpulmonaryresistanceincreased大型缺損和肺動脈阻力增加

Symptoms:

cyanosisafterexerciseorcontinuecyanosis,sporadichemoptysis(喀血）,clubbing,growthdevelopmentbackwardobviously症狀:黃萎病運動後或繼續黃萎病,零星的咳血,杵狀指,成長發展明顯落後

SmallVSD:

normalormildLVhypertrophyMorderate-largeVSD:LVhypertrophyorbothventricularhypertrophyPApressureobviouslyincreased:mainlyRVhypertrophyElectrocardiogram(ECG)AssistantExaminationesSmallVSD:

normalormildLVhypertrophyMorderate-largeVSD:LVhypertrophyorbothventricularhypertrophyPApressureobviouslyincreased:mainlyRVhypertrophyChestX-ray

SmallVSD:

pulmonaryflowslightlyincreasednormalinlunghilarshadowPAsegmentnormalormildheaveheartshapenormalormildlarge,CTR0.5-0.55aorticknob(主動脈結)reducing小缺損:肺流稍微增加正常肺門的影子PA部分正常或輕度脹心形正常或輕度大,CTR0.5-0.55主動脈旋鈕(主動脈結)減少

ChestX-rayMorderate-largeVSD:

pulmonaryflowobviouslyincreasedlunghilarshadowaugment,thicken,hilardancinginchestfluoroscopyPAsegmentheaveheartshapeobviouslylarge,CTR0.56-0.7,bothventricularlarge,mainlyinLV,aorticknobreducing中度缺損:肺流明顯增加肺門的陰影增大,變厚,門的舞蹈在胸部透視段絞心形顯然很大,CTR0.56--0.7,兩心室大,主要在LV,主動脈旋鈕減少ChestX-rayPApressureseriouslyincreased:

pulmonarycirculatoryflowlowerPAsegmentseriouslyheavelungarteryrough,twist,discontinue,likerattailorresidualrootheartshapenotobviouslylarge,mainlyRVlargeaorticknobreducing肺動脈壓力嚴重增加:肺循環流低PA段嚴重絞肺部動脈粗糙、扭曲、停止,像老鼠的尾巴或殘根心形顯然不是很大,主要是大型主動脈旋鈕降低房車2DEchocardiogram：ventricularseptalechodiscontinue，LA、LVenlargement，ventricularseptalthickenandmovingwidthaugment二維超聲心動圖:室間隔回聲中斷,LA,LV增大,室間隔增厚和移動寬度增加ColorDopplerFlowImage：VSDsizeandposition，pressuredifference(壓差)betweenLVandRV，pulmonarypressure,RVpressure、lungresistanceandshuntflowcanbetaken彩色多普勒血流圖像:缺損大小和位置,壓差之間的LV和RV,肺動脈壓力,房車壓力,肺阻力和並聯可以流動EchocardiographyAssistantExaminationesCardiacCatheterizationandAngiocardiography心導管和心血管造影術

Rightcardiaccatheterization：右心導管檢查Exceptionalchannel特殊的管道：RVLVBloodoxygendata血氧數據：RVoxygensaturation(SO2,氧飽和度)>RASO2Pressuredata：RVpressureincreasedAssistantExaminationesCardiacCatheterizationandAngiocardiographyWhatcanbefoundinleftcardiaccatheterization?WhatcanbeobservedinLVangiocardiography?

Complications(LeftRightshunt)Pneumonia肺炎Congestiveheartfailure充血性心力衰竭Pulmonaryarteryhypertension肺動脈高血壓Infectionendocarditis感染性心內膜炎TreatmentForVSD

Internalmedicaltreatment國內醫療

preventandtreatallkindsofcomplications,預防和治療各種併發症

Interventionalcardiaccatheterization

percutaneousVSDocclusion 經皮缺損閉塞Adaptations：-perimembraousVSD-age≥2歲-aloneVSDVSDupperedgedistancetorightAovalve≥2.0mmnorightcoronaryAovalveprolapseintoVSDandaortareturn沒有右冠狀動脈Ao瓣脫垂，主動脈回流-residualshuntaftersurgery

TreatmentForVSD

Surgery外科Unsuitabilitypercutaneousocclusion不適合經皮閉塞middle-largeVSD:infantswhooftensufferedinflexibleheartfailure,pneumonia,growingbackwardandpulmonaryhypertensionshouldbeoperatedearlyin6monthsold.中度缺損:嬰兒經常遭受的心力衰竭、肺炎、發展落後和肺動脈高壓應該早在6個月大。AtrialSeptalDefect

（ASD）OutlineIncidenceandrateofnaturalclosurePathologicalanatomyPathophysiologyandHemodynamicsClinicalManifestationsAssistantExaminationesElectrocardiogram/X-ray/Echocardiography/CardiacCatheterizationandAngiocardiographyComplicationsTreatmentIncidenceandrateofnaturalclosureASDisoneofcommonCHDinchildrenIncidence:20-30%ofCHDSecondaryholeASDisthemosttype，primaryholeASDandcoronaryveinsinus(冠狀靜脈竇)ASDarefewer。繼發性ASD是最類型,主孔ASD和冠狀靜脈竇ASD更少PartofsecondaryholeASDscannaturalclose(therate<VSD),othertypeASDcann’tnaturalclose。繼發性ASD的一部分可以自然關閉,其他類型ASD不能自然關閉AtrialseptelformingStartfrom4thweekofembryo從4周的胚胎開始Firstseptal/primaryhole原發性Secondaryseptal/secondaryhole繼發性(self-studyP242)PathologicalanatomyAccordingtoASDposition：①centraltype（卵圓窩型），約占76％②superiorvenacava(上腔型,靜脈竇型）③inferiorvenacava(下腔型)④coronaryveinsinus(冠狀靜脈竇型)mixedtype(混合型)PathophysiologyandHemodynamicsPathophysiologyandHemodynamicsRAflowvolumeincreased,RVenlargementandhypertrophyRA流量增加,RV擴大和肥大Pulmonarycirculationcongestion(多血）Systemiccirculationbloodsupplyinsufficient(少血）ClinicalManifestationsAccordingtosizeofASDandvolumeofshunt

SymptomsSmallASDandlittleshunt,havenotanysymptomLargeASDandmoreshunt（Qp/Qs>1.5:1）Systemicinsufficient：pale,slim，hypodynamia(乏力)PAcongestion充血：cardiopalmus(心悸)andbreathhard(氣短)afterexercising,temporarycyanose,respiratorytractinfection臨時發紺病、呼吸道感染Infant,mayhavenotsymptom,oftenbefoundinphysicalexamination嬰兒,可能沒有症狀,常在體檢中被發現ClinicalManifestationsSigns

Mostnormal,nocyanoseHeartexaminationRightsideexpand右邊擴大moreorlessNothrill(震顫)2~3／6systolicmurmur收縮期雜音in2~3ribleftalongbreastbone在2~3根肋骨左胸骨P2strengthen，S2fixedspliting(固定分裂)AssistantExaminationes

Electrocardiography

MajorfeaturesElectricalaxisinclinedtoright(電軸右偏)RAenlargementLeadVlappearsrsR,conductiondelay(傳導延遲)ElectrocardiogramChestX-RaySmallASD:normalLargeASD,largeshunt:Vascularshadowinbothlungsincreased，pulmonarycirculationengorged兩肺血管陰影增加，肺循環充血Segmentofpulmonaryarteryprominent段肺動脈突出，“lunghilumdancing”phenomenon(肺門舞蹈)RA、RVenlargementAortaknotless（主動脈結縮小）CTRincreased（心胸比例）ThediagramsofchestroentgenogramsofASDvascularshadowinbothlungsincreased兩肺血管影增加pulmonarycirculationcongestion肺循環充血EchocardiographyM-modeEchocardiogramM型超聲心動圖:

98％RA、RVenlargement，ventricularseptelandLVpostero-wallmovesinsameway98％RA、RV擴大、心室走勢相同2DEchocardiogram二維超聲心動圖：atriasepteldiscontinue,theendlikematch-stick心房停止,最終像火柴棍ColorDopplerFlowImage：multi-coloredflowshuntfromLAtoRAthroughASDCardiacCatheterization

Rightcardiaccatheterization右心導管檢查

(1)dataofbloodoxygen:RASO2>SVC,IVC

(2)dataofpressure:RA、RV、PA

(3)abnormalchannel：RAtoLA,PVComplications(LeftRightshunt)PneumoniaCongestiveheartfailurePulmonaryarteryhypertensionInfectionendocarditis:fewerTreatmentforASDInternalmedicaltreatment

preventandtreatallkindsofcomplications,monitorPApressure預防和治療各種併發症,監控PA的壓力Interventionaltherapy

TranscatheterASDocclusion

ItisanimportanttechnicalrenovationinmedicalscienceBeginningfrom1976Amplatzertwotrays(雙盤)ASDoccluderhavingbeenusedfrom199780%ASDcanbecuredbyoccluder封堵器Adaptations適應症age≥2yearsolddiameter4-36mm,secondarycentralASDthedistanceofdefectedgetocoronaryveinsinus(冠狀靜脈竇)，SVC、IVCandPV≥5mm,tochambervalve瓣膜≥7mmthemaxmalatriaseptelextension範圍≥ASDdiameter＋14mmNoothermalformationneedsurgery沒有其他的畸形需要手術

ProcessoftranscathterASDocclusionuseAmplatzerOccluder

TreatmentforASDsurgeryLargeASDASDsthatcannottreatbyocclusion阻塞PatentDuctusArteriosus

（PDA）

OutlineIncidenceandrateofnaturalclosurePathologicalanatomyPathophysiologyandHemodynamicsClinicalManifestationsAssistantExaminesElectrocardiogram/X-ray/Echocardiography/CardiacCatheterizationandAngiocardiographyComplicationsTreatment

IncidenceandrateofnaturalclosureIncidence:15％inCHDTheductusfunctionalitycloseafterborn15hours,anatomicclosetimein3monthesafterbirth導管功能性關閉出生15小時後,解剖性關閉在出生後3個月Iftheductuscontinueopenandhavepathophysiologychange如果導管繼續開放和病理生理學改變，bediagnosedCHD(PDA)Pathologicalanatomy

3types:①tubetype管型②funneltype漏斗形③windowtype窗型

PathophysiologyandHemodynamics

PathophysiologyandHemodynamicsLA,LVhypervolemia(血容量增加),enlargement，hypertrophyPAengorgement(充血)Systemiccirculationblood-supplyinsufficient(供血不足)Peripheralarterydiastolicfalling(舒張壓力下降)，(pulsepressurebroadening(脈壓增寬)

ClinicalManifestations

Symptoms

SmallPDA，symptomlessMiddleandlargePDA，respiratoryrateincreasing,acratia(乏力),andcardiopalmus(心悸),shortofbreath(氣喘),coughafteractivityin6monthesold.MostlargePDA，repeatedlyrespiratorytractinfection(pneumonia)andCHFininfant

ClinicalManifestationsPhysicalexaminationIngeneral:thin,thoracocyllosis(胸廓畸形),differentialcyanose（差異性青紫）(cyanoseinlowerlimbs>upperlimbs,left>right)inPAH(RtoLshunt)Heart:

LA,LVenlargementmoreorlessTypicalsign:rough/loud/mechinery/continuousmurmurin2ndribleftborderofsternum,andconducttoleftclavicle,neckandback;thrillP2strengthen典型的跡象:粗糙/大聲/機械/連續雜音2肋胸骨左邊界,並進行左鎖骨,脖子和背部,震顫P2加強ClinicalManifestationsPeripheralbloodvesselsign:systemiccirculationdiastolicpressuredropping,pulsepressuredifferenceincreasing,justlikethesignofaorticvalveinsufficiency周邊血管的跡象:體循環舒張壓下降,脈壓差增加,就像主動脈瓣閉鎖Waterhammerpulse(水沖脈)Signofcapillarypulsation(毛細血管搏動征)Peripherallargearterygunshot(周圍大動脈槍擊聲)AssistantExaminationes

Electrocardiography:LVhypertrophy

ChestRadiography

SmallPDA:normalComparativelylargePDA:LA,LVenlargement,segmentofPAprominent,shadowofpumonaryvesselthicken,lungfieldcongestion,aortabroadening.PDA較大:LA,LV擴大,部分PA突出,肺紋理變增厚,肺野充血,主動脈增寬。LargePDAandPAH:LV,RVenlargement,obviousforRV,segment段ofPAprominent突出,peripheryvesselbecomethin週邊血管變得細,lungfieldcongestionmaynotobviousthen,likedeadwood(枯枝或截枝狀)EchocardiographyM-modeEchocardiogram:LV、LAenlargement，aortaanterior-posterioraugmentation主動脈前後增加，theactivityrangeofLVbackwallandventricularseptumincreasing他的活動範圍的LV後牆和心室隔增加2DEchocardiogram：directindicationPDAbetweenMPAanddescendingaortaColorDopplerFlowImage：redshuntfromDAOtoMPAthroughPDACardiacCatheterizationandAngiocardiographyRightcardiaccathterization：

Abnormalway：catheterfromPAtoDAOthroughPDAdirectlydataofbloodoxygen：PA>RV，means?dataofpressure：monitorPApressureandwedgepressurePA(肺小動脈契壓)，andcalculatetotalpulmonaryresistanceandpulmonarysmallarteryresistance數據的壓力:監測壓力和楔壓PA(肺小動脈契壓),並計算總肺阻力和肺小動脈阻力CardiacCatheterizationandAngiocardiographyAortaangiocardiography主動脈心血管造影術ascendingaortaandaorticarchenlargement，PAandarterialductdeveloping(顯影)atthesametime，andmeasurabilityofdiameter,andshapeofduct升主動脈和主動脈弓膨大,PA和動脈導管發展(顯影)同時,直徑和可測性,管的形狀Complications(LefttoRightshunt)PneumoniaCongestiveheartfailure充血性心力衰竭PulmonaryarteryhypertensionInfectionendocarditis:fewerTreatmentForPDA

Internalmedicine

內科醫學 topreventandtreatcomplications預防和治療併發症TreatmentForPDA

Interventionaltherapy,transcatheterPDAocclusion,firstchoiceatpresent經導管PDA閉塞介入治療,目前的第一選擇

Coil(springcoil)：線圈PDAnarrowest<2.0mm/weight≥4kg/age≥6mon/residualshuntaftersurgeryAmplatzer：蘑菇傘PDAnarrowest≥2.0mm，≤12mm/weight≥4kg/age≥6mon/residualshuntaftersurgeryTreatmentForPDA

Surgery

Greatductus,windowtypeTetralogyofFallot(TOF)OutlineIncidencePathologicalanatomyPathophysiology&hemodynamicClinicalManifestationsAssistantexaminationesElectrocardiography/ChestX-ray/Echocardiography/Cardiaccatheterizationandangiocardiography

ComplicationsTreatmentIncidence發生率TOF，about10～15％inCHDThemostcommoncyanosisCHDinsurvivinginfant倖存的嬰兒About70%incyanosis紫紺CHDafteroneyearoldPathologicalanatomyTypicalTOFincludingfourmalformations：(1)pulmonarystenosis(PS)肺動脈瓣狹窄,themostimportantpathologicalchangeandfactorassociatingwithpathophysiologyandclinicalmanifestation最重要的病理改變和因素與病理生理學和臨床表現(2)VSD,perchmembranous室間隔缺損(3)aortaoverriding主動脈騎跨(4)Rightventricularhypertrophy右心室肥厚

PathophysiologyandHemodynamics

RVhypertrophy過度肥厚，RAenlargementPulmonarycirculatorybloodflowdecrease肺動脈迴圈血流量減少Systemiccirculatorymixedblood體循環混血ClinicalManifestationsMainlycorrelatedtoextentofPSSymptomsCyanosis:紫紺Oftenappearsin3～6monthesoldCyanosisbefoundinnewbornforseriousPSMildcyanosis輕度發紺befounduntilseniorfornothingseriousMainlyinplaceofabundantofcapillaryvessel，suchaslip,nailbedoffingerandtoe.主要在地方豐富的毛細血管,如唇、甲床的手指和腳趾。ClinicalManifestations

Hypoxiaspells(缺氧發作):

dysphoria(煩躁不安)、dyspnea(呼吸困難)、morecyanosis(發紺加重)、weakcry(哭聲微弱)、syncope(暈厥)、lowmuscletension(肌張力低下)forseveralminutesorhours

Inducers誘導性因素:cry、infection、anemia、coldnessmainlyattackininfant，incidenceabout20％～25％,

trendofnaturalimprovementafter2yearsoldClinicalManifestations

Activityendurancedecreasing活動耐力降低Squatting(蹲踞現象）

ItisoutstandingfeatureofTOFArterialbloodoxygencontentdecreasing,dismayed(心慌)、breathehard(氣短)、morecyanosisTheseniorpatientlikepostureofsquatting,andcannotstandorwalkforalongtime。Infantlikepostureofarchedbodyorchest-knee顯著特點TOF動脈的血氧含量減少,沮喪(心慌),呼吸困難(氣短),更多的黃萎病高級病人像蹲的姿勢,和受不了走了很長一段時間。嬰兒像拱形的身體姿勢或chest-kneeClinicalManifestationsSignsGeneral:Growingdevelopmentretardation生長發育遲緩,cyanosis紫紺Clubbingoffingersortoenails(杵狀指趾)Prominentafter1yearsold1歲後突出Heartexaminationes

inspection:precordiumnotfull檢驗:心前區不完整palpation:systolicthrillinL2-4觸診:L2-4收縮期震顫percussion:heartboundarynormalorexpand叩診:心臟邊界正常或擴大auscultation:2-4/6systolicmurmurinL2-4,P2weakeningordisappear聽診:2-4/6L2-4收縮期雜音,P2減弱或消失AssistantexaminationesElectrocardiography

cardiacelectricalaxisinclinedtorightside,RVhypertrophy,V1presentrightbranchblock,tall-sharpPwave心臟電軸傾向於右側臥,房車肥大,V1右分支塊,tall-sharpP波Ifcardiacelectricalaxisnotinclinedtorightside,orLVhypertrophy,orbothventricularhypertrophyinECG,oftenbelongtonon-cyanositictetralogy,mayaccompanywithPDA如果心臟電軸不傾向於右邊,或LV肥大,或兩個心室肥大心電圖,通常屬於non-cyanositic四部曲,可能伴隨著PDAElectrocardiographyAssistantexaminationesChestX-rayTypicalX-ray:“bootsheart(靴型心)”，cardiacapexroundbluntupwarp(心尖圓鈍上翹)，cardiacwaistsunkenorstraight(心腰凹陷或平直)Cardiothoracicratio:normal，RV,RAslightlyenlarge心胸比率:正常、RV、RA略有放大Shadowoflunghilum(肺門)reducing,bloodvesseloflungfieldslim肺門陰影(肺門)減少,血管的肺野變少Aortashiftstorightfront，causingshadowofsuperiormediastinum(上縱隔)broaden主動脈轉向正確,造成陰影的優越縱隔(上縱隔)擴大Increasinglungmarkings(肺紋理)andLVenlargement,meansaccompanywithPDAorcollateralcirculation(側支迴圈)增加肺的標記(肺紋理)和LV擴大,意味著伴隨著PDA或側枝迴圈(側支迴圈)AssistantexaminationesEchocardiograph2Decho:specialvalueindiagnosisTOFContinuitybreaksoffbetweenventricularseptelandaortafrontwall連續性中斷了心室壁和主動脈前壁之間Frontwallofaortarootshiftforward,aortaoverridingonventricularseptel前壁的主動脈根轉變,主動脈覆蓋在心室壁RVenlargement,RVoutletnarrowing，RVfrontwallandventricularthickenRV擴大，RV出口縮小,房車前壁和心室增厚CardiacCatheterizationandangiocardiography

rightcardiaccatheterization

dataofbloodoxygen:LV

dataofpressure:RV/PA,continuouspressurecurves持續的壓力曲線fromPAtoRV

abnormalway:RVtoAO,RVtoLV

RVangiographyComplicationsCerbralthrombosis(腦血栓):incidence4％。HCT(血球壓積)increased，bloodstreamslowingbloodsticknessincreased血流減慢血液粘性增加，resultinthrombosis血栓形成Brainabscess(腦膿腫):manyhappeninsenior(>2yearsold)andadult。ItshouldbeconsideredinTOFwithfever,headache,vomit嘔吐,hypersomnia(嗜睡),convu