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(shí)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(shí)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(dòng)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(jìn)展，臨床上對(duì)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(dòng)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(dòng)脈導(dǎo)管未閉、法洛四聯(lián)癥的血流動(dòng)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(jìn)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(dòng)脈導(dǎo)管)維持動(dòng)靜脈血并非絕對(duì)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(dòng)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(dòng)脈2根，臍靜脈1根)(6-8周)

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

動(dòng)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(dòng)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(jí)預(yù)防：孕婦保健Primaryprevention：pregnantwomanhealth二級(jí)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(jí)預(yù)防：治療出生后畸形Tertiaryprevention:treatborndeformity預(yù)防一級(jí)預(yù)防：孕婦保健二級(jí)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(dòng)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(dòng)力學(xué)示意圖

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

可無血流動(dòng)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(dòng)脈高壓

ObstructedPH艾森門格綜合征

EisenmengerSyndrome肺動(dòng)脈高壓PulmonaryHypertension(PH)動(dòng)力型肺動(dòng)脈高壓

DynamicPH室間隔缺損血流動(dòng)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(jí)粗糙全收縮期吹風(fēng)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(dòng)脈瓣第二音亢進(jìn)ThepulmonarycomponentofS2isaccentuationinintensity合并主動(dòng)脈瓣關(guān)閉不全時(shí)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(dòng)力學(xué)示意圖

Hemodynamics

Figure

of

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

右心房（擴(kuò)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(dòng)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(dòng)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(jí)柔和收縮期噴射音，無震顫Agrade1-3/6ejectionSMisheardbestattheLSBinthe2ndintercostalspace，nothrillP2固定分裂S2atthepulmonaryareaiswidelysplitandoftenfixed肺動(dòng)脈瓣第二音亢進(jìn)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(dòng)脈段突出，右房、右室增大。XRayofsecundumASD肺野充血，肺動(dòng)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(dòng)脈導(dǎo)管未閉

Patentductusarteriosus(PDA）動(dòng)脈導(dǎo)管未閉動(dòng)脈導(dǎo)管未閉

血流動(dòng)力學(xué)示意圖

HemodynamicsFigureofPDA動(dòng)脈導(dǎo)管未閉血流動(dòng)力學(xué)示意圖HemodynamicsAnatomopathologicalDiagramofPDAAnatomopathologicalDiagramof右心室血流體循環(huán)舒張壓肺動(dòng)脈主動(dòng)脈供血減少脈壓增寬肺血流量肺動(dòng)脈高壓艾森門格綜合征左房、左室擴(kuò)大（差異性紫紺）（右心室肥大）動(dòng)脈導(dǎo)管未閉血流動(dòng)力學(xué)變化HemodynamicsofPDAPDA右心室血流動(dòng)脈導(dǎo)管未閉血流動(dòng)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(jī)器樣雜音，占據(jù)整個(gè)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(dòng)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(dòng)脈炎Infectivearteritis●充血性心力衰竭

Congestiveheartfailure動(dòng)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(diǎn)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(diǎn)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(dòng)脈狹窄Obstructiontorightventricularoutflow●室間隔缺損

Ventricularseptaldefect

●主動(dòng)脈騎跨

Overridingoftheaorta●右心室肥厚Rightventricularhypertrophy

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

血流動(dòng)力學(xué)示意圖

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

肺動(dòng)脈狹窄主動(dòng)脈

（血流量、擴(kuò)張）肺血流量減少（肺野清晰）混合血進(jìn)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(dòng)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(jí)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(dòng)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(shí)顯影，大動(dòng)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(shí)顯影，大動(dòng)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(dòng)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(qiáng)度≥3/6級(jí)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(hù)理Anticongestiveheartfailuremeasures,goodnursing

●外科治療Operationtreatment開胸心臟修補(bǔ)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(dòng)脈導(dǎo)管未閉，動(dòng)脈導(dǎo)管最窄直徑≥2.0mm，年齡≥6個(gè)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(shí)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(shí)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(dòng)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(jìn)展，臨床上對(duì)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(dòng)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(dòng)脈導(dǎo)管未閉、法洛四聯(lián)癥的血流動(dòng)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(jìn)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(dòng)脈導(dǎo)管)維持動(dòng)靜脈血并非絕對(duì)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(dòng)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(dòng)脈2根，臍靜脈1根)(6-8周)

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

動(dòng)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(dòng)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(jí)預(yù)防：孕婦保健Primaryprevention：pregnantwomanhealth二級(jí)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(jí)預(yù)防：治療出生后畸形Tertiaryprevention:treatborndeformity預(yù)防一級(jí)預(yù)防：孕婦保健二級(jí)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(dòng)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(dòng)力學(xué)示意圖

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

可無血流動(dòng)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(dòng)脈高壓

ObstructedPH艾森門格綜合征

EisenmengerSyndrome肺動(dòng)脈高壓PulmonaryHypertension(PH)動(dòng)力型肺動(dòng)脈高壓

DynamicPH室間隔缺損血流動(dòng)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(jí)粗糙全收縮期吹風(fēng)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(dòng)脈瓣第二音亢進(jìn)ThepulmonarycomponentofS2isaccentuationinintensity合并主動(dòng)脈瓣關(guān)閉不全時(shí)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(dòng)力學(xué)示意圖

Hemodynamics

Figure

of

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

右心房（擴(kuò)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(dòng)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(dòng)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(jí)柔和收縮期噴射音，無震顫Agrade1-3/6ejectionSMisheardbestattheLSBinthe2ndintercostalspace，nothrillP2固定分裂S2atthepulmonaryareaiswidelysplitandoftenfixed肺動(dòng)脈瓣第二音亢進(jìn)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(dòng)脈段突出，右房、右室增大。XRayofsecundumASD肺野充血，肺動(dòng)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(dòng)脈導(dǎo)管未閉

Patentductusarteriosus(PDA）動(dòng)脈導(dǎo)管未閉動(dòng)脈導(dǎo)管未閉

血流動(dòng)力學(xué)示意圖

HemodynamicsFigureofPDA動(dòng)脈導(dǎo)管未閉血流動(dòng)力學(xué)示意圖HemodynamicsAnatomopathologicalDiagramofPDAAnatomopathologicalDiagramof右心室血流體循環(huán)舒張壓肺動(dòng)脈主動(dòng)脈供血減少脈壓增寬肺血流量肺動(dòng)脈高壓艾森門格綜合征左房、左室擴(kuò)大（差異性紫紺）（右心室肥大）動(dòng)脈導(dǎo)管未閉血流動(dòng)力學(xué)變化HemodynamicsofPDAPDA右心室血流動(dòng)脈導(dǎo)管未閉血流動(dòng)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(jī)器樣雜音，占據(jù)整個(gè)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(dòng)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(dòng)脈炎Infectivearteritis●充血性心力衰竭

Congestiveheartfailure動(dòng)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(diǎn)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(diǎn)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(dòng)脈狹窄Obstructiontorightventricularoutflow●室間隔缺損

Ventricularseptaldefect

●主動(dòng)脈騎跨

Overridingoftheaorta●右心室肥厚Rightventricularhypertrophy

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

血流動(dòng)力學(xué)示意圖

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

肺動(dòng)脈狹窄主動(dòng)脈

（血流量、擴(kuò)張）肺血流量減少（肺野清晰）混合血進(jìn)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(dòng)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(jí)收縮期噴射性雜音,P2減弱Thereisagrade2-4/6,rough,ejection-typeSMthatismaximalattheLSBinthe2ndto3rdintercostalspaceandthatradiateswelltotheback.ThepulmonarycomponentofS2