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育異常而導致的先天性畸形CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater出生缺陷監測先天性心血管發育畸形出現率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育異常概述

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

Objective＆

Request了解胎兒血液循環及出生后血流動力學變化

Tounderstand

changesoffetuscirculation

andhemodynamics

beforeandafterbirth熟悉先天性心臟病的病因、分類、預防及治療原則Tofamiliarwiththe

etiology

and

classificationofCHD掌握室間隔缺損、房間隔缺損、動脈導管未閉、法洛四聯癥的血流動力學、臨床表現及常見并發癥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Fetuscirculation胎兒營養及代謝物質交換、氧氣及二氧化碳交換均靠胎盤進行胎兒左右兩側心臟均向全身供血，肺循環血少，循環通過異常通道(卵圓孔、動脈導管)維持動靜脈血并非絕對清楚，循環效率不如成人高，但胎兒循環途徑既適合于胎內由胎盤吸氧，又適合于出生后轉換為以肺取氧的循環通道，胎兒循環有效而靈活胎兒血液循環胎兒營養及代謝物質交換、氧氣及二氧化碳交換均靠胎出生后血流動力學變化六條通道關閉，肺循環建立

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卵圓孔關閉(5-7月)

動脈導管閉合(3-4月)

靜脈導管閉合(斷臍后)出生后血流動力學變化六條通道關閉，肺循環建立臍血管阻斷形成病因

Etiology

遺傳因素(內在因素)

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

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

病因Etiology遺傳因素(內在因素)與基因突變、預防

Prevention一級預防：孕婦保健Primaryprevention：pregnantwomanhealth二級預防：提高產前診斷技術，選擇性終止妊娠，減少先心病發生Secondaryprevention:improveprenataldiagnosistechnology,selectiveterminationofpregnancyandreducehappencongenitalheartdisease三級預防：治療出生后畸形Tertiaryprevention:treatborndeformity預防一級預防：孕婦保健二級預防：提高產前診斷技術，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)●動脈導管未閉

Patentductusarteriosus(PDA)●法洛四聯癥

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

血流動力學示意圖

HemodynamicsFigureofVSD室間隔缺損血流動力學示意圖HemodynamicsAnatomopathologicalDiagramofVSDAnatomopathologicalDiagramof室間隔缺損血流動力學變化HemodynamicsofVSD小室缺

可無血流動力學變化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變化小室缺可VSD臨床表現ClinicalfindingsofVSD●癥狀

Symptoms

反復呼吸道感染，生長發育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessVSD臨床表現ClinicalfindingsofVSVSD臨床表現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，伴震顫Agrade3-4/6,medium-to-highpitched,harshpansystolicmurmurattheleftsternalborder(LSB)inthe3rdand4thintercostalspaces肺動脈瓣第二音亢進ThepulmonarycomponentofS2isaccentuationinintensity合并主動脈瓣關閉不全時可聞舒張期雜音

AdiastolicmurmurindicateVSDcombinewithaorticregurgitation

小中VSD臨床表現ClinicalfindingsofVS室間隔缺損并發癥ComplicationofVSD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●肺水腫

Pulmonaryedema●感染性心內膜炎

Infectiveendocarditis室間隔缺損并發癥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

血流動力學示意圖

Hemodynamics

Figure

of

ASD房間隔缺損血流動力學示意圖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(少數病人晚期）房間隔缺損血流動力學變化HemodynamicsofASDASD上、下腔靜脈血ASD臨床表現ClinicalfindingsofASD●癥狀

Symptoms

同室缺相似

SymptomsofASDaresimilarwiththatofVSD

反復呼吸道感染，生長發育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessASD臨床表現ClinicalfindingsofASASD臨床表現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三尖瓣區可聞舒張期雜音Amid-diastolicmurmurcanoftenbeheardintricuspidareaASD臨床表現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癥ComplicationofASD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●感染性心內膜炎

Infectiveendocarditis房間隔缺損并發癥ComplicationofASD●支氣動脈導管未閉

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

血流動力學示意圖

HemodynamicsFigureofPDA動脈導管未閉血流動力學示意圖HemodynamicsAnatomopathologicalDiagramofPDAAnatomopathologicalDiagramof右心室血流體循環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）動脈導管未閉血流動力學變化HemodynamicsofPDAPDA右心室血流動脈導管未閉血流動力學變化HemodynaPDA臨床表現ClinicalfindingsofPDA●癥狀

Symptoms

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

SymptomsofPDAaresimilarwiththatofVSDandASDPDA臨床表現ClinicalfindingsofPDPDA臨床表現ClinicalfindingsofPDA●體征

Signs

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

心尖區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動脈導管未閉并發癥ComplicationofPDA●支氣管肺炎

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

Congestiveheartfailure動脈導管未閉并發癥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●心前區有粗糙的雜音Aroughmurmurisheardinprecordium●肺循環血量多，易患肺炎Pulmonaryflowincreaseandeasytosufferfrombronchopneumonia●體循環血量少，影響生長發育Systemicflowdecreaseandthepatientgrowslowly左向右分流先心病共同臨床特點Commonfeatures法洛四聯癥TetralogyofFallot（TOF）法洛四聯癥法洛四聯癥四種解剖畸形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

法洛四聯癥四種解剖畸形●肺動脈狹窄Obstructiont法洛四聯癥

血流動力學示意圖

HemodynamicsFigureofTOF法洛四聯癥血流動力學示意圖Hemodynamics法洛四聯癥血流動力學變化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

（青紫、發育落后、乏力血氧合不足

（杵狀指趾等）

蹲踞、陣發性昏厥）分流法洛四聯癥血流動力學變化HemodynamicsoTOF臨床表現ClinicalfindingsofTOF

●青紫本病最突出的癥狀Cyanosisisthemainsymptoms

●氣促和缺氧發作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，大動脈關系正常，RVOT變窄，AO內徑增寬并騎跨于室間隔上。右室造影AO和PA同時顯影，大動脈關系正常，RVOT變窄，法洛四聯癥并發癥ComplicationofTOF●腦血栓

Cerebralembolism●腦膿腫

Cerebralabscess●感染性心內膜炎

Infectiveendocarditis法洛四聯癥并發癥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Repairthedefectthroughtransthoraciccardiacsurgeryoperation●自然閉合

Spontaneousclosure

先天性心臟病的治療TreatmentofCHD●內科治先天性心臟病的治療TreatmentofCHD

●介入治療

Interventionaltreatment

利用堵閉器材導管關閉PDA、繼發孔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Allkindsofinterventionaltherapyincardiovasculardisease心血管各種介入治療技術PDA介入治療InterventionaltreatmentofPDAPDA介入治療InterventionaltreatmenPDA介入治療InterventionaltreatmentofPDA適應證（Amplatzer法：①左向右分流不合并需外科手術的心臟畸形的動脈導管未閉，動脈導管最窄直徑≥2.0mm，年齡≥6個月，體重≥4kg。②外科術后殘余分流。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育異常而導致的先天性畸形CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater出生缺陷監測先天性心血管發育畸形出現率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育異常概述

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

Objective＆

Request了解胎兒血液循環及出生后血流動力學變化

Tounderstand

changesoffetuscirculation

andhemodynamics

beforeandafterbirth熟悉先天性心臟病的病因、分類、預防及治療原則Tofamiliarwiththe

etiology

and

classificationofCHD掌握室間隔缺損、房間隔缺損、動脈導管未閉、法洛四聯癥的血流動力學、臨床表現及常見并發癥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Fetuscirculation胎兒營養及代謝物質交換、氧氣及二氧化碳交換均靠胎盤進行胎兒左右兩側心臟均向全身供血，肺循環血少，循環通過異常通道(卵圓孔、動脈導管)維持動靜脈血并非絕對清楚，循環效率不如成人高，但胎兒循環途徑既適合于胎內由胎盤吸氧，又適合于出生后轉換為以肺取氧的循環通道，胎兒循環有效而靈活胎兒血液循環胎兒營養及代謝物質交換、氧氣及二氧化碳交換均靠胎出生后血流動力學變化六條通道關閉，肺循環建立

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卵圓孔關閉(5-7月)

動脈導管閉合(3-4月)

靜脈導管閉合(斷臍后)出生后血流動力學變化六條通道關閉，肺循環建立臍血管阻斷形成病因

Etiology

遺傳因素(內在因素)

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

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

病因Etiology遺傳因素(內在因素)與基因突變、預防

Prevention一級預防：孕婦保健Primaryprevention：pregnantwomanhealth二級預防：提高產前診斷技術，選擇性終止妊娠，減少先心病發生Secondaryprevention:improveprenataldiagnosistechnology,selectiveterminationofpregnancyandreducehappencongenitalheartdisease三級預防：治療出生后畸形Tertiaryprevention:treatborndeformity預防一級預防：孕婦保健二級預防：提高產前診斷技術，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)●動脈導管未閉

Patentductusarteriosus(PDA)●法洛四聯癥

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

血流動力學示意圖

HemodynamicsFigureofVSD室間隔缺損血流動力學示意圖HemodynamicsAnatomopathologicalDiagramofVSDAnatomopathologicalDiagramof室間隔缺損血流動力學變化HemodynamicsofVSD小室缺

可無血流動力學變化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變化小室缺可VSD臨床表現ClinicalfindingsofVSD●癥狀

Symptoms

反復呼吸道感染，生長發育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessVSD臨床表現ClinicalfindingsofVSVSD臨床表現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，伴震顫Agrade3-4/6,medium-to-highpitched,harshpansystolicmurmurattheleftsternalborder(LSB)inthe3rdand4thintercostalspaces肺動脈瓣第二音亢進ThepulmonarycomponentofS2isaccentuationinintensity合并主動脈瓣關閉不全時可聞舒張期雜音

AdiastolicmurmurindicateVSDcombinewithaorticregurgitation

小中VSD臨床表現ClinicalfindingsofVS室間隔缺損并發癥ComplicationofVSD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●肺水腫

Pulmonaryedema●感染性心內膜炎

Infectiveendocarditis室間隔缺損并發癥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

血流動力學示意圖

Hemodynamics

Figure

of

ASD房間隔缺損血流動力學示意圖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(少數病人晚期）房間隔缺損血流動力學變化HemodynamicsofASDASD上、下腔靜脈血ASD臨床表現ClinicalfindingsofASD●癥狀

Symptoms

同室缺相似

SymptomsofASDaresimilarwiththatofVSD

反復呼吸道感染，生長發育落后、乏力、氣短、聲嘶Repeatedrespiratoryinfection,growthanddevelopmentlagbehind,fatigue,shortnessofbreath,hoarsenessASD臨床表現ClinicalfindingsofASASD臨床表現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三尖瓣區可聞舒張期雜音Amid-diastolicmurmurcanoftenbeheardintricuspidareaASD臨床表現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癥ComplicationofASD●支氣管肺炎

Bronchopneumonia●充血性心力衰竭

Congestiveheartfailure●感染性心內膜炎

Infectiveendocarditis房間隔缺損并發癥ComplicationofASD●支氣動脈導管未閉

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

血流動力學示意圖

HemodynamicsFigureofPDA動脈導管未閉血流動力學示意圖HemodynamicsAnatomopathologicalDiagramofPDAAnatomopathologicalDiagramof右心室血流體循環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）動脈導管未閉血流動力學變化HemodynamicsofPDAPDA右心室血流動脈導管未閉血流動力學變化HemodynaPDA臨床表現ClinicalfindingsofPDA●癥狀

Symptoms

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

SymptomsofPDAaresimilarwiththatofVSDandASDPDA臨床表現ClinicalfindingsofPDPDA臨床表現ClinicalfindingsofPDA●體征

Signs

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

心尖區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動脈導管未閉并發癥ComplicationofPDA●支氣管肺炎

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

Congestiveheartfailure動脈導管未閉并發癥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●心前區有粗糙的雜音Aroughmurmurisheardinprecordium●肺循環血量多，易患肺炎Pulmonaryflowincreaseandeasytosufferfrombronchopneumonia●體循環血量少，影響生長發育Systemicflowdecreaseandthepatientgrowslowly左向右分流先心病共同臨床特點Commonfeatures法洛四聯癥TetralogyofFallot（TOF）法洛四聯癥法洛四聯癥四種解剖畸形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

法洛四聯癥四種解剖畸形●肺動脈狹窄Obstructiont法洛四聯癥

血流動力學示意圖

HemodynamicsFigureofTOF法洛四聯癥血流動力學示意圖Hemodynamics法洛四聯癥血流動力學變化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

（青紫、發育落后、乏力血氧合不足

（杵狀指趾等）

蹲踞、陣發性昏厥）分流法洛四聯癥血流動力學變化HemodynamicsoTOF臨床表現ClinicalfindingsofTOF

●青紫本病最突出的癥狀Cyanosisisthemainsymptoms

●氣促和缺氧發作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