CHDs儿童先天性心脏病各论课件

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(漏斗部狭窄)Prolapseofaorticvalve(主动脉瓣脱垂)Infectiveendocarditis(感染性心内膜炎)MedicalManagementPhysicalactivitiesproperlyPreventionandcureofinfectiontimelyFollow-upregularlyAnticongestivemeasures:－digitalis(洋地黄)－diuretics(利尿剂)－vasodilators(扩管药物)Transcatheterclosure(经导管封堵术）IndicationsforSurgicalRepairCongestiveheartfailurewithfailuretothriveorrecurrentpneumoniaProgressivepulmonaryhypertensionEvidenceofinfundibulumstenosisEvidenceofprolaseofaorticvalveSupracristalVSDHistoryofinfectiveendocarditisAtrialSeptalDefect

（ASD）AtrialSeptalDefectThedisorderofembryologicaldevelopmentofinteratrialseptumAccountingfor10％

ofCHD

上腔静脉下腔静脉静脉窦型缺损继发孔型缺损原发孔型缺损主动脉冠状静脉窦型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导联以S为主，Ⅲ导联以R为主,电轴右偏。RaVR﹥0.5mv,R／S﹥1,V1呈RSr,QRS﹤0.08，示不完全右束支转导阻滞。RV1＋SV5﹥2.5mv，提示右室大。ChestX-ray

IncreasedvascularmarkingsinlungsHeart/chestratio:>0.55EnlargementofRA,RVDilatedmainpulmonaryarterysegmentSmalleraortainsizeEchocardiogramDisplaypositionandsizeDisplayshuntingDisplayparadoxicmotion(矛盾运动)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(经导管封堵术）PatentDuctusArteriosus

（PDA）Accountingfor15％

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

)PulmonaryhypertensionAOLV(expansion)LA(expansion)DescendingaortaSmallerdiameterPeripheralarteriesDiastolicpressuredecreasedHemodynamicCharacteristicsSystemicbloodflow

Pulmonaryartery(expansion)ShuntPulmonarybloodflow

SmallshuntAsymptomaticContinuousmachinerymurmurofgradeII~IIIheardatleftsternalborderinthe2ndintercostalspaces,radiatingtoinferiorleftclavicle(左锁骨下) (2LSBCMⅡ~Ⅲo)ClinicalManifestationsLargeshunt(symptoms)SymptomssimilartoVSD’s:－suchasfailuretothrive,recurrentpneumonia,poorcardiacfunction－exceptfordifferentialcyanosis(差异性紫绀)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(经导管封堵术）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（缺氧发作）:suddenonsetofdyspnea;deepeningofcyanosis;irritabilityorsyncope;convulsion；absenceofcardiacmurmur（ahallmarkofseveresituation）ClinicalManifestationsSigns：－Cyanosis－Clubbingoffingersandtoes－3LSBSMⅡ～Ⅳ0,radiatingwidely－P2decreased

－S2usuallypredominantlyaorticandsingle

ClinicalManifestationClubbing杵状