CHDs兒童先天性心臟病各論課件

VentricularSeptalDefect

（VSD）VentricularSeptalDefect

ThedisorderofembryologicaldevelopmentofinterventricularseptumMostcommonformofCHDinchildrenAccountingfor25％PositionofVSD：－Membranous(60-70%):thecommonestlocationt－Subpulmonic(3-6%):riskofaorticvalveprolapse－Muscular(20-30%):occuranywhereinthemuscularpartofseptumAnatomicTypesSubpulmonicMembranousMuscularSizeofVSD－Small:<5－Medium:5～10mm－Large:>10mmBeforePulmonaryhypertensionRightatriumRightventricle(Flow)Pulmonaryartery(expansion)Pulmonarybloodflow

Rightventricle(Hypertrophy)Leftatrium(Hypertrophy)

Leftventricle(Hypertrophy)Ejectionvolume

Systemicbloodflow

ShuntSystemicblood（Mixed）RightatriumLeftatriumAfterPulmonaryhypertensionLeftventriclePulmonaryhypertensionreversible(dynamic)Irreversible(pulmonaryvasculardisease)Eisenmeinger’ssyndromeShuntPulmonaryartery(expansion)Rightventricle(Hypertrophy)HemodynamicCharacteristicsSmallVSD－asymptomatic－Pan-systolicmurmurofgradeⅡ~Ⅳheardatleftsternalborderinthe3rd~4thintercostalspaces,radiatingoverprecordium(3~4LSBSMⅡ~Ⅳo)ClinicalManifestationsMedium～LargeVSD(symptoms)

Pulmonaryplethora---RecurrentchestinfectionSystemicbloodflow

--Failuretothrive(slowweightgain）Poorcardiacfunction:Cyanosiswhenright-to-leftshuntoccurs,mostlyduetoseverepulmonaryhypertension ClinicalManifestationsMedium～LargeVSDPoorcardiacfunction:－

atinfancy：difficultywithfeeding,sweating,tachypnea,andhepatomegaly;－

inolderchildren：dyspneaonexcursion,easyfatigability,palpitation,exerciseintoleranceClinicalManifestationsMedium～LargeVSD(signs)

2～4LSBSMⅢ~Ⅵo

DMatapexduetolargebloodflowacrossnormalmitralvalve (relativemitralstenosis)P2increasedwithsplitCyanosiswithclubbinginlatestageClinicalManefestationElectrocardiogramSmallVSD:ECGusuallynormalMedium~large－LVhypertrophywhenpulmonaryvascularresistanceisnormal

－BothLV&RVhypertrophywhenpulmonaryhypertensionoccursduetoincreasedvascularresistance&increasedflow－RVhypertrophyinEisenmenger’ssyndromeChestX-raySmallVSD:maybenormalMedium~largeVSD:－Increasedvascularmarkingsinlungs－Heart/chestratio:>0.55－EnlargementofLVand/orRV－Dilatedmainpulmonaryarterysegment－SmalleraortainsizeEchocardiogramDisplaypositionandsizeof thedefectDisplayshuntingMeasurepressuregradientDisplaysizeofchambersandvessels:－EnlargedLA,LVand/or RVEchocardiogram2DE&CDEdisplaysVSDPrognosis&ComplicationsAsymptomatic30~50％

closespontaneouslyby2yearsofageCongestiveheartfailurePulmonaryhypertensionInfundibulumstenosis(漏斗部狹窄)Prolapseofaorticvalve(主動(dòng)脈瓣脫垂)Infectiveendocarditis(感染性心內(nèi)膜炎)MedicalManagementPhysicalactivitiesproperlyPreventionandcureofinfectiontimelyFollow-upregularlyAnticongestivemeasures:－digitalis(洋地黃)－diuretics(利尿劑)－vasodilators(擴(kuò)管藥物)Transcatheterclosure(經(jīng)導(dǎo)管封堵術(shù)）IndicationsforSurgicalRepairCongestiveheartfailurewithfailuretothriveorrecurrentpneumoniaProgressivepulmonaryhypertensionEvidenceofinfundibulumstenosisEvidenceofprolaseofaorticvalveSupracristalVSDHistoryofinfectiveendocarditisAtrialSeptalDefect

（ASD）AtrialSeptalDefectThedisorderofembryologicaldevelopmentofinteratrialseptumAccountingfor10％

ofCHD

上腔靜脈下腔靜脈靜脈竇型缺損繼發(fā)孔型缺損原發(fā)孔型缺損主動(dòng)脈冠狀靜脈竇型fossaovalisASD（75%）SinusvenosusASDs（5%）OstiumprimumASD(15%)AnatomicTypesCoronarysinusASD（2%）HemodynamicCharacteristicsSuperiorandInferiorvenacavaRA(Flow

)RV(Hypertrophy)ASDShuntPulmonaryveinLAflow

AortaEjectionvolume

Pulmonaryartery(expansion)Pulmonarybloodflow

LVflow

Systemicbloodflow

SymptomsaresimilartoVSD’s－suchaspoorgrowthanddevelopment,recurrentpneumonia,poorcardiacfunction－butoccurlessfrequentlyininfants－Somepatientsevenremainasymptomaticthroughlife

ClinicalManifestationsSigns：

－2,3LSBSMⅡ~Ⅲo

Themurmuriscausedbyincreased flowacrosspulmonicvalves(i.e.relativepulmonarystenosis)－4LSBDMcanoftenbeheard (relativetricuspidstenosis)－P2increasedwithfixedsplit (固定分裂）ClinicalManifestationsElectrocardiogram

Axisrightdeviation,V1,V3Rhaveincompleterightbundlebranchblockdiagram

Ⅰ導(dǎo)聯(lián)以S為主，Ⅲ導(dǎo)聯(lián)以R為主,電軸右偏。RaVR﹥0.5mv,R／S﹥1,V1呈RSr,QRS﹤0.08，示不完全右束支轉(zhuǎn)導(dǎo)阻滯。RV1＋SV5﹥2.5mv，提示右室大。ChestX-ray

IncreasedvascularmarkingsinlungsHeart/chestratio:>0.55EnlargementofRA,RVDilatedmainpulmonaryarterysegmentSmalleraortainsizeEchocardiogramDisplaypositionandsizeDisplayshuntingDisplayparadoxicmotion(矛盾運(yùn)動(dòng))ofventricularseptumDisplaysizeofchambers andvessels:－EnlargedRAandRV－DilatedMPA－SmallerAOEchocardiogram

ostiumprimumASDostiumsecundumASDPrognosis&ComplicationsAsymptomatic(ofteninchildhood)Heartfailure(occurinmiddleadulthood)Atrialtachyarrhythmias(adulthood)Pulmonaryhypertension(uncommon)Infectiveendocarditis(rarelyoccur）Spontaneousclosure－mostfrequentlyifASD<4mm －frequentlyifASD<8mm－mostlyclosedbeforeage2yearsMedicalManagementNoneedofphysicalrestrictionfor mostpatientsPreventionandcureofinfectiontimelyFollow-upregularlyAnticongestivemeasures:－

digitalis－diuretics－vasodilatorsTranscatheterclosure(經(jīng)導(dǎo)管封堵術(shù)）PatentDuctusArteriosus

（PDA）Accountingfor15％

ofCHDIncidencemaybeashighas20~60%inpreterminfantsweighing<1500gMorecommoninfemaleandtheinfantsbornathighaltitudesPatentDuctusArteriosusAnatomicTypesTubulartype(80%)FunneltypeWindowtypeRAVRPA(Flow

)PulmonaryhypertensionAOLV(expansion)LA(expansion)DescendingaortaSmallerdiameterPeripheralarteriesDiastolicpressuredecreasedHemodynamicCharacteristicsSystemicbloodflow

Pulmonaryartery(expansion)ShuntPulmonarybloodflow

SmallshuntAsymptomaticContinuousmachinerymurmurofgradeII~IIIheardatleftsternalborderinthe2ndintercostalspaces,radiatingtoinferiorleftclavicle(左鎖骨下) (2LSBCMⅡ~Ⅲo)ClinicalManifestationsLargeshunt(symptoms)SymptomssimilartoVSD’s:－suchasfailuretothrive,recurrentpneumonia,poorcardiacfunction－exceptfordifferentialcyanosis(差異性紫紺)duetoseverepulmonaryhypertension ClinicalManifestationsLargeshunt(signs)

2LSBCMIII~IVo

DMatapexduetolargebloodflowacrossnormalmitralvalve (relativemitralstenosis)P2increasedwithsplitDifferentialcyanosiswithclubbingoftoesClinicalManifestationsElectrocardiogramSmallshunt:ECGusuallynormalLargeshunt:－LVhypertrophywhenpulmonaryvascularresistanceisnormal

－BothLV&RVhypertrophywhenpulmonaryhypertensionoccursduetoincreasedvascularresistance&increasedflow－RVhypertrophyinEisenmenger’ssyndromeChestX-raySmallshunt:normalLargeshunt:－Increasedvascularmarkingsinlungs－Heart/chestratio:>0.55－EnlargementofLA,LV－Dilatedmainpulmonaryarterysegment－prominentaortainsizeEchocardiogramductalshuntingPrognosis&ComplicationsAsymptomaticCongestiveheartfailurePulmonaryhypertensionInfectiveendocarditisspontaneousclosureofductalshunt－90%closefunctionallyby4daysafterbirth－80%closeanatomicallyin3month,and95%in1yearofageMedicalManagementPhysicalactivitiesproperlyPreventionandcureofinfectiontimelyFollow-upregularlyAnticongestivemeasures:－

digitalis－diuretics－vasodilators

Transcatheterclosure(經(jīng)導(dǎo)管封堵術(shù)）TetralogyofFallot

（TOF）TetralogyofFallotAccountingfor10％

ofCHDObstructiontoRVoutflow：infundibularand/orvalvularlevelwithhypoplasiaofPALargeVSDAortathatoverridestheVSDHypertrophyofRVAnatomicfeaturesRARVLA(flow

)

LV(flow

)

AO(flow

)MixedbloodenterSystemiccirculation(Expand)(Hypertrophy)

ObstructiontoRVoutflowPulmonarybloodflow

OxygenexchangeisinsufficientVSDShuntOverridingaortaShunt（Right-to-leftshunting

）HypoxiaHemodynamicCharacteristicsSymptoms：Owingtoanoxia－Cyanosis(mostlyseenfrom4monthsofageandprogressive)－Retardedgrowthanddevelopment,easyfatigabilityanddyspneaonexcursion－Squattingwhenwalking－Hypoxemicspell（缺氧發(fā)作）:suddenonsetofdyspnea;deepeningofcyanosis;irritabilityorsyncope;convulsion；absenceofcardiacmurmur（ahallmarkofseveresituation）ClinicalManifestationsSigns：－Cyanosis－Clubbingoffingersandtoes－3LSBSMⅡ～Ⅳ0,radiatingwidely－P2decreased

－S2usuallypredominantlyaorticandsingle

ClinicalManifestationClubbing杵狀