先天性心臟病課件

先天性心臟病

CongenitalHeartDisease(CHD)先天性心臟病CongenitalHeartDiseasCHD：CongenitalHeartDiseaseVSD：VentricularseptaldefectASD：AtrialseptaldefectPDA：PatentductusarteriosusTOF：TetralogyofFallotPulmonaryhypertensionHemodynamicsShuntHeartmurmurTwo-dimensionalechocardiogramgramECG：electrocardiogramInterventionaltherapyCHD：CongenitalHeartDisease概述

Overview先天性心臟病是胎兒時期心臟血管發(fā)育異常而導(dǎo)致的先天性畸形CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater出生缺陷監(jiān)測先天性心血管發(fā)育畸形出現(xiàn)率25.1/10

000CHDispresentin25.1/10000inChina

概述Overview先天性心臟病是胎兒時期心臟血管發(fā)育異常概述

Overview由于各種心血管檢查技術(shù)（特別是彩色多普勒超聲心動圖）的應(yīng)用，深低溫麻醉和體外循環(huán)下心內(nèi)直視手術(shù)的發(fā)展，先心病介入性治療進展，臨床上對先心病的診斷和治療發(fā)生了很大變化，預(yù)后大為改觀。Bytheprogressionofallkindsofinspectiontechniquesandinterventionaltherapy,theprognosisischangedgreatly.概述Overview由于各種心血管檢查技術(shù)（特別是彩色多普目的要求

Objective＆

Request了解胎兒血液循環(huán)及出生后血流動力學(xué)變化

Tounderstand

changesoffetuscirculation

andhemodynamics

beforeandafterbirth熟悉先天性心臟病的病因、分類、預(yù)防及治療原則Tofamiliarwiththe

etiology

and

classificationofCHD掌握室間隔缺損、房間隔缺損、動脈導(dǎo)管未閉、法洛四聯(lián)癥的血流動力學(xué)、臨床表現(xiàn)及常見并發(fā)癥Tomasterthe

hemodynamicsand

clinicalsituationandthe

diagnosisofcommoncomplicationsinVSD,ASD,PDAandTOF

目的要求Objective＆Request了解胎兒血液循胎兒血液循環(huán)Fetuscirculation胎兒營養(yǎng)及代謝物質(zhì)交換、氧氣及二氧化碳交換均靠胎盤進行胎兒左右兩側(cè)心臟均向全身供血，肺循環(huán)血少，循環(huán)通過異常通道(卵圓孔、動脈導(dǎo)管)維持動靜脈血并非絕對清楚，循環(huán)效率不如成人高，但胎兒循環(huán)途徑既適合于胎內(nèi)由胎盤吸氧，又適合于出生后轉(zhuǎn)換為以肺取氧的循環(huán)通道，胎兒循環(huán)有效而靈活胎兒血液循環(huán)胎兒營養(yǎng)及代謝物質(zhì)交換、氧氣及二氧化碳交換均靠胎出生后血流動力學(xué)變化六條通道關(guān)閉，肺循環(huán)建立

臍血管阻斷形成韌帶(臍動脈2根，臍靜脈1根)(6-8周)

呼吸建立，肺部血管擴張建立肺循環(huán)卵圓孔關(guān)閉(5-7月)

動脈導(dǎo)管閉合(3-4月)

靜脈導(dǎo)管閉合(斷臍后)出生后血流動力學(xué)變化六條通道關(guān)閉，肺循環(huán)建立臍血管阻斷形成病因

Etiology

遺傳因素(內(nèi)在因素)

與基因突變、染色體畸變有關(guān)Geneticfactor(internalfactor)CHDresultfromgenemutationorchromosomeaberration環(huán)境因素(外界因素)

與宮內(nèi)病毒感染有關(guān)Environmentalfactor(externalfactor)CHDismainlycorrelationtotheintrauterineinfection

病因Etiology遺傳因素(內(nèi)在因素)與基因突變、預(yù)防

Prevention一級預(yù)防：孕婦保健Primaryprevention：pregnantwomanhealth二級預(yù)防：提高產(chǎn)前診斷技術(shù)，選擇性終止妊娠，減少先心病發(fā)生Secondaryprevention:improveprenataldiagnosistechnology,selectiveterminationofpregnancyandreducehappencongenitalheartdisease三級預(yù)防：治療出生后畸形Tertiaryprevention:treatborndeformity預(yù)防一級預(yù)防：孕婦保健二級預(yù)防：提高產(chǎn)前診斷技術(shù)，選擇性終分類Classification左向右分流型(潛在青紫型)

left-to-rightshunts(noncyanosis)右向左分流型(青紫型)

right-to-leftshunts(cyanosis)無分流型(無青紫型)

nonshunts(noncyanosis)分類Classification左向右分流型(潛在青紫型)臨床常見的先心病CommonCHDinClinic●室間隔缺損

Ventricularseptaldefect(VSD)

●房間隔缺損

Atrialseptaldefect(ASD)●動脈導(dǎo)管未閉

Patentductusarteriosus(PDA)●法洛四聯(lián)癥

TetralogyofFallot(TOF)臨床常見的先心病●室間隔缺損Ventriculars室間隔缺損

VentricularSeptalDefect（VSD）室間隔缺損室間隔缺損

血流動力學(xué)示意圖

HemodynamicsFigureofVSD室間隔缺損血流動力學(xué)示意圖HemodynamicsAnatomopathologicalDiagramofVSDAnatomopathologicalDiagramof室間隔缺損血流動力學(xué)變化HemodynamicsofVSD小室缺

可無血流動力學(xué)變化SmallVSD

nohemodynamicschanges

大室缺

大量左向右分流LargeVSD

Largeleft-to-rightshunts梗阻型肺動脈高壓

ObstructedPH艾森門格綜合征

EisenmengerSyndrome肺動脈高壓PulmonaryHypertension(PH)動力型肺動脈高壓

DynamicPH室間隔缺損血流動力學(xué)變化小室缺可VSD臨床表現(xiàn)ClinicalfindingsofVSD●癥狀

Symptoms

反復(fù)呼吸道感染，生長發(fā)育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessVSD臨床表現(xiàn)ClinicalfindingsofVSVSD臨床表現(xiàn)ClinicalfindingsofVSD●體征

Signs

LSB3-4可聞3-4/6級粗糙全收縮期吹風樣雜音，向周圍廣泛傳導(dǎo)，伴震顫Agrade3-4/6,medium-to-highpitched,harshpansystolicmurmurattheleftsternalborder(LSB)inthe3rdand4thintercostalspaces肺動脈瓣第二音亢進ThepulmonarycomponentofS2isaccentuationinintensity合并主動脈瓣關(guān)閉不全時可聞舒張期雜音

AdiastolicmurmurindicateVSDcombinewithaorticregurgitation

小中VSD臨床表現(xiàn)ClinicalfindingsofVS室間隔缺損并發(fā)癥ComplicationofVSD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●肺水腫

Pulmonaryedema●感染性心內(nèi)膜炎

Infectiveendocarditis室間隔缺損并發(fā)癥ComplicationofVSD●支氣先天性心臟病課件房間隔缺損

Atrial

SeptalDefect（ASD）房間隔缺損房間隔缺損

血流動力學(xué)示意圖

Hemodynamics

Figure

of

ASD房間隔缺損血流動力學(xué)示意圖Hemodynamics上、下腔靜脈血肺靜脈

右心房（擴大）左心房

右心室（增大）左心室（血量減少）肺血流量明顯增加（肺充血）肺小動脈痙攣、增厚體循環(huán)供血不足

右向左分流

（消瘦、乏力、心悸、氣短等）艾森門格綜合征(少數(shù)病人晚期）房間隔缺損血流動力學(xué)變化HemodynamicsofASDASD上、下腔靜脈血ASD臨床表現(xiàn)ClinicalfindingsofASD●癥狀

Symptoms

同室缺相似

SymptomsofASDaresimilarwiththatofVSD

反復(fù)呼吸道感染，生長發(fā)育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessASD臨床表現(xiàn)ClinicalfindingsofASASD臨床表現(xiàn)ClinicalfindingsofASD●體征

Signs

LSB2-3可聞1-3/6級柔和收縮期噴射音，無震顫Agrade1-3/6ejectionSMisheardbestattheLSBinthe2ndintercostalspace，nothrillP2固定分裂S2atthepulmonaryareaiswidelysplitandoftenfixed肺動脈瓣第二音亢進ThepulmonarycomponentofS2isaccentuationinintensity三尖瓣區(qū)可聞舒張期雜音Amid-diastolicmurmurcanoftenbeheardintricuspidareaASD臨床表現(xiàn)ClinicalfindingsofASXRayofsecundumASD肺野充血，肺動脈段突出，右房、右室增大。XRayofsecundumASD肺野充血，肺動脈先天性心臟病課件房間隔缺損并發(fā)癥ComplicationofASD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●感染性心內(nèi)膜炎

Infectiveendocarditis房間隔缺損并發(fā)癥ComplicationofASD●支氣動脈導(dǎo)管未閉

Patentductusarteriosus(PDA）動脈導(dǎo)管未閉動脈導(dǎo)管未閉

血流動力學(xué)示意圖

HemodynamicsFigureofPDA動脈導(dǎo)管未閉血流動力學(xué)示意圖HemodynamicsAnatomopathologicalDiagramofPDAAnatomopathologicalDiagramof右心室血流體循環(huán)舒張壓肺動脈主動脈供血減少脈壓增寬肺血流量肺動脈高壓艾森門格綜合征左房、左室擴大（差異性紫紺）（右心室肥大）動脈導(dǎo)管未閉血流動力學(xué)變化HemodynamicsofPDAPDA右心室血流動脈導(dǎo)管未閉血流動力學(xué)變化HemodynaPDA臨床表現(xiàn)ClinicalfindingsofPDA●癥狀

Symptoms

反復(fù)呼吸道感染，生長發(fā)育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarseness與VSD及ASD相同

SymptomsofPDAaresimilarwiththatofVSDandASDPDA臨床表現(xiàn)ClinicalfindingsofPDPDA臨床表現(xiàn)ClinicalfindingsofPDA●體征

Signs

LSB2聞及粗糙、響亮的連續(xù)性機器樣雜音，占據(jù)整個收縮期及舒張期Acharacteristicthrillandcontinuousandrough“machinery”murmurwithalatesystolicaccentuation

心尖區(qū)可聞及舒張中期隆隆樣雜音Adiastolicflowmurmurisoftenheardattheapex周圍血管征Peripheralvascularsigh

下半身青紫(差異性紫紺)和杵狀趾Differentialcyanosisandclubbing

PDA臨床表現(xiàn)ClinicalfindingsofPD先天性心臟病課件S1S2PDA堵閉前心音圖S1S2PDA堵閉前心音圖PDA堵閉后心音圖S1S2PDA堵閉后心音圖S1S2動脈導(dǎo)管未閉并發(fā)癥ComplicationofPDA●支氣管肺炎

Bronchopneumonia●感染性心內(nèi)膜炎Infectiveendocarditis●感染性動脈炎Infectivearteritis●充血性心力衰竭

Congestiveheartfailure動脈導(dǎo)管未閉并發(fā)癥ComplicationofPDA●支左向右分流先心病共同臨床特點Commonfeaturesofleft-to-rightshuntingCHD●一般情況下無青紫Ingeneraltherearenoncyanosis●心前區(qū)有粗糙的雜音Aroughmurmurisheardinprecordium●肺循環(huán)血量多，易患肺炎Pulmonaryflowincreaseandeasytosufferfrombronchopneumonia●體循環(huán)血量少，影響生長發(fā)育Systemicflowdecreaseandthepatientgrowslowly左向右分流先心病共同臨床特點Commonfeatures法洛四聯(lián)癥TetralogyofFallot（TOF）法洛四聯(lián)癥法洛四聯(lián)癥四種解剖畸形ThefourmalformationsofTOF●肺動脈狹窄Obstructiontorightventricularoutflow●室間隔缺損

Ventricularseptaldefect

●主動脈騎跨

Overridingoftheaorta●右心室肥厚Rightventricularhypertrophy

法洛四聯(lián)癥四種解剖畸形●肺動脈狹窄Obstructiont法洛四聯(lián)癥

血流動力學(xué)示意圖

HemodynamicsFigureofTOF法洛四聯(lián)癥血流動力學(xué)示意圖Hemodynamics法洛四聯(lián)癥血流動力學(xué)變化HemodynamicsofTOF右心室（肥厚）左心室

肺動脈狹窄主動脈

（血流量、擴張）肺血流量減少（肺野清晰）混合血進入循環(huán)

（青紫、發(fā)育落后、乏力血氧合不足

（杵狀指趾等）

蹲踞、陣發(fā)性昏厥）分流法洛四聯(lián)癥血流動力學(xué)變化HemodynamicsoTOF臨床表現(xiàn)ClinicalfindingsofTOF

●青紫本病最突出的癥狀Cyanosisisthemainsymptoms

●氣促和缺氧發(fā)作Dyspneaandhypoxemicspells

●蹲踞癥狀Squattingposture

●杵狀指趾

Clubbingoftheterminaldigits

●心臟體征LSB2-4聞及2-4/6級收縮期噴射性雜音,P2減弱Thereisagrade2-4/6,rough,ejection-typeSMthatismaximalattheLSBinthe2ndto3rdintercostalspaceandthatradiateswelltotheback.ThepulmonarycomponentofS2weaken

inintensityTOF臨床表現(xiàn)ClinicalfindingsofTO杵狀指Clubbingoffingers

杵狀指Clubbingoffingers杵狀指趾

Clubbingoffingersandtoes杵狀指趾Clubbingoffingersand先天性心臟病課件X線檢查

右室大、心尖上翹呈靴形，肺動脈段凹陷，肺野清晰XRayofTOFX線檢查XRayofTOF先天性心臟病課件

右室造影AO和PA同時顯影，大動脈關(guān)系正常，RVOT變窄，AO內(nèi)徑增寬并騎跨于室間隔上。右室造影AO和PA同時顯影，大動脈關(guān)系正常，RVOT變窄，法洛四聯(lián)癥并發(fā)癥ComplicationofTOF●腦血栓

Cerebralembolism●腦膿腫

Cerebralabscess●感染性心內(nèi)膜炎

Infectiveendocarditis法洛四聯(lián)癥并發(fā)癥ComplicationofTOF●腦血先天性心臟病的診斷DiagnosisofCHD●病史

History●體查

Physicalexamination●心電圖

Electrocardiogram●心臟X線檢查ChestX-ray●超聲心動圖Echocardiogram●心導(dǎo)管檢查和心血管造影Cardiaccatheterizationandangiocardiography先天性心臟病的診斷DiagnosisofCHD●病史H臨床上有診斷意義的心臟雜音Clinicalsignificanceheartmurmur●全收縮期雜音

Pansystolicmurmur●收縮晚期雜音Latesystolicmurmur●舒張期雜音Diastolicmurmur●連續(xù)性雜音

Continuousmurmur●強度≥3/6級的雜音

Grade≥3/6murmur臨床上有診斷意義的心臟雜音Clinicalsignific先天性心臟病的治療TreatmentofCHD

●內(nèi)科治療Medicalmanagement治療心衰，良好護理Anticongestiveheartfailuremeasures,goodnursing

●外科治療Operationtreatment開胸心臟修補手術(shù)Repairthedefectthroughtransthoraciccardiacsurgeryoperation●自然閉合

Spontaneousclosure

先天性心臟病的治療TreatmentofCHD●內(nèi)科治先天性心臟病的治療TreatmentofCHD

●介入治療

Interventionaltreatment

利用堵閉器材導(dǎo)管關(guān)閉PDA、繼發(fā)孔ASD、VSDCatheterizationclosurewithaninterventionaldeviceisgenerallyrecommendedforostiumsecundumASDandPDA

andVSD

先天性心臟病的治療TreatmentofCHD●介入治

心血管各種介入治療技術(shù)Allkindsofinterventionaltherapyincardiovasculardisease心血管各種介入治療技術(shù)PDA介入治療InterventionaltreatmentofPDAPDA介入治療InterventionaltreatmenPDA介入治療InterventionaltreatmentofPDA適應(yīng)證（Amplatzer法：①左向右分流不合并需外科手術(shù)的心臟畸形的動脈導(dǎo)管未閉，動脈導(dǎo)管最窄直徑≥2.0mm，年齡≥6個月，體重≥4kg。②外科術(shù)后殘余分流。PDA介入治療InterventionaltreatmenASD介入治療InterventionaltreatmentofASDASD介入治療InterventionaltreatmenASD介入治療InterventionaltreatmentofASDASD介入治療InterventionaltreatmenVSD介入治療InterventionaltreatmentofVSDVSD介入治療InterventionaltreatmenVSD介入治療InterventionaltreatmentofVSDVSD介入治療Interventionaltreatmen謝謝謝謝先天性心臟病

CongenitalHeartDisease(CHD)先天性心臟病CongenitalHeartDiseasCHD：CongenitalHeartDiseaseVSD：VentricularseptaldefectASD：AtrialseptaldefectPDA：PatentductusarteriosusTOF：TetralogyofFallotPulmonaryhypertensionHemodynamicsShuntHeartmurmurTwo-dimensionalechocardiogramgramECG：electrocardiogramInterventionaltherapyCHD：CongenitalHeartDisease概述

Overview先天性心臟病是胎兒時期心臟血管發(fā)育異常而導(dǎo)致的先天性畸形CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater出生缺陷監(jiān)測先天性心血管發(fā)育畸形出現(xiàn)率25.1/10

000CHDispresentin25.1/10000inChina

概述Overview先天性心臟病是胎兒時期心臟血管發(fā)育異常概述

Overview由于各種心血管檢查技術(shù)（特別是彩色多普勒超聲心動圖）的應(yīng)用，深低溫麻醉和體外循環(huán)下心內(nèi)直視手術(shù)的發(fā)展，先心病介入性治療進展，臨床上對先心病的診斷和治療發(fā)生了很大變化，預(yù)后大為改觀。Bytheprogressionofallkindsofinspectiontechniquesandinterventionaltherapy,theprognosisischangedgreatly.概述Overview由于各種心血管檢查技術(shù)（特別是彩色多普目的要求

Objective＆

Request了解胎兒血液循環(huán)及出生后血流動力學(xué)變化

Tounderstand

changesoffetuscirculation

andhemodynamics

beforeandafterbirth熟悉先天性心臟病的病因、分類、預(yù)防及治療原則Tofamiliarwiththe

etiology

and

classificationofCHD掌握室間隔缺損、房間隔缺損、動脈導(dǎo)管未閉、法洛四聯(lián)癥的血流動力學(xué)、臨床表現(xiàn)及常見并發(fā)癥Tomasterthe

hemodynamicsand

clinicalsituationandthe

diagnosisofcommoncomplicationsinVSD,ASD,PDAandTOF

目的要求Objective＆Request了解胎兒血液循胎兒血液循環(huán)Fetuscirculation胎兒營養(yǎng)及代謝物質(zhì)交換、氧氣及二氧化碳交換均靠胎盤進行胎兒左右兩側(cè)心臟均向全身供血，肺循環(huán)血少，循環(huán)通過異常通道(卵圓孔、動脈導(dǎo)管)維持動靜脈血并非絕對清楚，循環(huán)效率不如成人高，但胎兒循環(huán)途徑既適合于胎內(nèi)由胎盤吸氧，又適合于出生后轉(zhuǎn)換為以肺取氧的循環(huán)通道，胎兒循環(huán)有效而靈活胎兒血液循環(huán)胎兒營養(yǎng)及代謝物質(zhì)交換、氧氣及二氧化碳交換均靠胎出生后血流動力學(xué)變化六條通道關(guān)閉，肺循環(huán)建立

臍血管阻斷形成韌帶(臍動脈2根，臍靜脈1根)(6-8周)

呼吸建立，肺部血管擴張建立肺循環(huán)卵圓孔關(guān)閉(5-7月)

動脈導(dǎo)管閉合(3-4月)

靜脈導(dǎo)管閉合(斷臍后)出生后血流動力學(xué)變化六條通道關(guān)閉，肺循環(huán)建立臍血管阻斷形成病因

Etiology

遺傳因素(內(nèi)在因素)

與基因突變、染色體畸變有關(guān)Geneticfactor(internalfactor)CHDresultfromgenemutationorchromosomeaberration環(huán)境因素(外界因素)

與宮內(nèi)病毒感染有關(guān)Environmentalfactor(externalfactor)CHDismainlycorrelationtotheintrauterineinfection

病因Etiology遺傳因素(內(nèi)在因素)與基因突變、預(yù)防

Prevention一級預(yù)防：孕婦保健Primaryprevention：pregnantwomanhealth二級預(yù)防：提高產(chǎn)前診斷技術(shù)，選擇性終止妊娠，減少先心病發(fā)生Secondaryprevention:improveprenataldiagnosistechnology,selectiveterminationofpregnancyandreducehappencongenitalheartdisease三級預(yù)防：治療出生后畸形Tertiaryprevention:treatborndeformity預(yù)防一級預(yù)防：孕婦保健二級預(yù)防：提高產(chǎn)前診斷技術(shù)，選擇性終分類Classification左向右分流型(潛在青紫型)

left-to-rightshunts(noncyanosis)右向左分流型(青紫型)

right-to-leftshunts(cyanosis)無分流型(無青紫型)

nonshunts(noncyanosis)分類Classification左向右分流型(潛在青紫型)臨床常見的先心病CommonCHDinClinic●室間隔缺損

Ventricularseptaldefect(VSD)

●房間隔缺損

Atrialseptaldefect(ASD)●動脈導(dǎo)管未閉

Patentductusarteriosus(PDA)●法洛四聯(lián)癥

TetralogyofFallot(TOF)臨床常見的先心病●室間隔缺損Ventriculars室間隔缺損

VentricularSeptalDefect（VSD）室間隔缺損室間隔缺損

血流動力學(xué)示意圖

HemodynamicsFigureofVSD室間隔缺損血流動力學(xué)示意圖HemodynamicsAnatomopathologicalDiagramofVSDAnatomopathologicalDiagramof室間隔缺損血流動力學(xué)變化HemodynamicsofVSD小室缺

可無血流動力學(xué)變化SmallVSD

nohemodynamicschanges

大室缺

大量左向右分流LargeVSD

Largeleft-to-rightshunts梗阻型肺動脈高壓

ObstructedPH艾森門格綜合征

EisenmengerSyndrome肺動脈高壓PulmonaryHypertension(PH)動力型肺動脈高壓

DynamicPH室間隔缺損血流動力學(xué)變化小室缺可VSD臨床表現(xiàn)ClinicalfindingsofVSD●癥狀

Symptoms

反復(fù)呼吸道感染，生長發(fā)育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessVSD臨床表現(xiàn)ClinicalfindingsofVSVSD臨床表現(xiàn)ClinicalfindingsofVSD●體征

Signs

LSB3-4可聞3-4/6級粗糙全收縮期吹風樣雜音，向周圍廣泛傳導(dǎo)，伴震顫Agrade3-4/6,medium-to-highpitched,harshpansystolicmurmurattheleftsternalborder(LSB)inthe3rdand4thintercostalspaces肺動脈瓣第二音亢進ThepulmonarycomponentofS2isaccentuationinintensity合并主動脈瓣關(guān)閉不全時可聞舒張期雜音

AdiastolicmurmurindicateVSDcombinewithaorticregurgitation

小中VSD臨床表現(xiàn)ClinicalfindingsofVS室間隔缺損并發(fā)癥ComplicationofVSD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●肺水腫

Pulmonaryedema●感染性心內(nèi)膜炎

Infectiveendocarditis室間隔缺損并發(fā)癥ComplicationofVSD●支氣先天性心臟病課件房間隔缺損

Atrial

SeptalDefect（ASD）房間隔缺損房間隔缺損

血流動力學(xué)示意圖

Hemodynamics

Figure

of

ASD房間隔缺損血流動力學(xué)示意圖Hemodynamics上、下腔靜脈血肺靜脈

右心房（擴大）左心房

右心室（增大）左心室（血量減少）肺血流量明顯增加（肺充血）肺小動脈痙攣、增厚體循環(huán)供血不足

右向左分流

（消瘦、乏力、心悸、氣短等）艾森門格綜合征(少數(shù)病人晚期）房間隔缺損血流動力學(xué)變化HemodynamicsofASDASD上、下腔靜脈血ASD臨床表現(xiàn)ClinicalfindingsofASD●癥狀

Symptoms

同室缺相似

SymptomsofASDaresimilarwiththatofVSD

反復(fù)呼吸道感染，生長發(fā)育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessASD臨床表現(xiàn)ClinicalfindingsofASASD臨床表現(xiàn)ClinicalfindingsofASD●體征

Signs

LSB2-3可聞1-3/6級柔和收縮期噴射音，無震顫Agrade1-3/6ejectionSMisheardbestattheLSBinthe2ndintercostalspace，nothrillP2固定分裂S2atthepulmonaryareaiswidelysplitandoftenfixed肺動脈瓣第二音亢進ThepulmonarycomponentofS2isaccentuationinintensity三尖瓣區(qū)可聞舒張期雜音Amid-diastolicmurmurcanoftenbeheardintricuspidareaASD臨床表現(xiàn)ClinicalfindingsofASXRayofsecundumASD肺野充血，肺動脈段突出，右房、右室增大。XRayofsecundumASD肺野充血，肺動脈先天性心臟病課件房間隔缺損并發(fā)癥ComplicationofASD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●感染性心內(nèi)膜炎

Infectiveendocarditis房間隔缺損并發(fā)癥ComplicationofASD●支氣動脈導(dǎo)管未閉

Patentductusarteriosus(PDA）動脈導(dǎo)管未閉動脈導(dǎo)管未閉

血流動力學(xué)示意圖

HemodynamicsFigureofPDA動脈導(dǎo)管未閉血流動力學(xué)示意圖HemodynamicsAnatomopathologicalDiagramofPDAAnatomopathologicalDiagramof右心室血流體循環(huán)舒張壓肺動脈主動脈供血減少脈壓增寬肺血流量肺動脈高壓艾森門格綜合征左房、左室擴大（差異性紫紺）（右心室肥大）動脈導(dǎo)管未閉血流動力學(xué)變化HemodynamicsofPDAPDA右心室血流動脈導(dǎo)管未閉血流動力學(xué)變化HemodynaPDA臨床表現(xiàn)ClinicalfindingsofPDA●癥狀

Symptoms

反復(fù)呼吸道感染，生長發(fā)育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarseness與VSD及ASD相同

SymptomsofPDAaresimilarwiththatofVSDandASDPDA臨床表現(xiàn)ClinicalfindingsofPDPDA臨床表現(xiàn)ClinicalfindingsofPDA●體征

Signs

LSB2聞及粗糙、響亮的連續(xù)性機器樣雜音，占據(jù)整個收縮期及舒張期Acharacteristicthrillandcontinuousandrough“machinery”murmurwithalatesystolicaccentuation

心尖區(qū)可聞及舒張中期隆隆樣雜音Adiastolicflowmurmurisoftenheardattheapex周圍血管征Peripheralvascularsigh

下半身青紫(差異性紫紺)和杵狀趾Differentialcyanosisandclubbing

PDA臨床表現(xiàn)ClinicalfindingsofPD先天性心臟病課件S1S2PDA堵閉前心音圖S1S2PDA堵閉前心音圖PDA堵閉后心音圖S1S2PDA堵閉后心音圖S1S2動脈導(dǎo)管未閉并發(fā)癥ComplicationofPDA●支氣管肺炎

Bronchopneumonia●感染性心內(nèi)膜炎Infectiveendocarditis●感染性動脈炎Infectivearteritis●充血性心力衰竭

Congestiveheartfailure動脈導(dǎo)管未閉并發(fā)癥ComplicationofPDA●支左向右分流先心病共同臨床特點Commonfeaturesofleft-to-rightshuntingCHD●一般情況下無青紫Ingeneraltherearenoncyanosis●心前區(qū)有粗糙的雜音Aroughmurmurisheardinprecordium●肺循環(huán)血量多，易患肺炎Pulmonaryflowincreaseandeasytosufferfrombronchopneumonia●體循環(huán)血量少，影響生長發(fā)育Systemicflowdecreaseandthepatientgrowslowly左向右分流先心病共同臨床特點Commonfeatures法洛四聯(lián)癥TetralogyofFallot（TOF）法洛四聯(lián)癥法洛四聯(lián)癥四種解剖畸形ThefourmalformationsofTOF●肺動脈狹窄Obstructiontorightventricularoutflow●室間隔缺損

Ventricularseptaldefect

●主動脈騎跨

Overridingoftheaorta●右心室肥厚Rightventricularhypertrophy

法洛四聯(lián)癥四種解剖畸形●肺動脈狹窄Obstructiont法洛四聯(lián)癥

血流動力學(xué)示意圖

HemodynamicsFigureofTOF法洛四聯(lián)癥血流動力學(xué)示意圖Hemodynamics法洛四聯(lián)癥血流動力學(xué)變化HemodynamicsofTOF右心室（肥厚）左心室

肺動脈狹窄主動脈

（血流量、擴張）肺血流量減少（肺野清晰）混合血進入循環(huán)

（青紫、發(fā)育落后、乏力血氧合不足

（杵狀指趾等）

蹲踞、陣發(fā)性昏厥）分流法洛四聯(lián)癥血流動力學(xué)變化HemodynamicsoTOF臨床表現(xiàn)ClinicalfindingsofTOF

●青紫本病最突出的癥狀Cyanosisisthemainsymptoms

●氣促和缺氧發(fā)作Dyspneaandhypoxemicspells

●蹲踞癥狀Squattingposture

●杵狀指趾

Clubbingoftheterminaldigits

●心臟體征LSB2-4聞及2-4/6級收縮期噴射性雜音,P2減弱Thereisagrade2-4/6,rough,ejection-typeSMthatismaximalattheLSBinthe2ndto3rdintercostalspaceandthatradiateswelltotheback.ThepulmonarycomponentofS2