先天性心脏病医学PPT课件.ppt

CongenitalHeartDisease(CHD),ShengjingHospitalPediatricsYuXuexin,Introduction,CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater.Incidence:6.9inaliveneonatal.150,000neonatalsufferfromCHDinChinaperyear.Newtreatments：catheterization、developmentofoperation,etc.,ObjectandRequest,FamiliarwiththeetiologyandclassificationofCHD.Masterthehemodynamics、clinicalmanifestationanddiagnosisofcommoncomplicationsinVSD,ASD,PDAandTOF.,Etiology,Internalfactors：genemutationorchromosomeaberration.Adefectinthelongarmofchromosome22associatedwiththeDiGeorge,Shprintzen,andconotruncalanomalyfacesyndromes.ThesechildrentendtohaveeitherinterruptedaorticarchorconotruncalabnomalitiessuchasTOFordoubleoutletrightventricle.Externalfactors:intraureteralinfection(rubellavirus)、ray、drug、metabolicdiseases、intraureteralhypoxia.Theincidenceinchildrenofaffectedmothersmaybeashighas10%-15%.,Classification,left-to-rightshunts,CyanosismaybeVSD、PDA、ASD,right-to-leftshunts,non-shunts,CyanosisTOF、dislocationofmainartery,Pulmonaryarterystenosis、aorticstenosis,Basedonshuntbetweenrightandleftheart,PatentductusarteriosusPDA,CommonCHDinClinic,AtrialseptaldefectASD,VentricularseptaldefectVSD,TetralogyofFallotTOF,1,2,3,4,VentricularSeptalDefect（VSD）,1、membranedefect85%2、musculardefect3、funneldefect10%,20-50VSDcancloseupwithouttreatment.,1、minordefect2、mediadefect3、majordefect,Anatomy,Mostcommon,30%inCHD.,Pathobiology,RVblood,pulmonaryhypertension,persistentcyanosis(Eisenmengersyndrome),LVblood,bodycirculation,Hemodynamics,Beforepulmonaryhypertension,RA,RV（blood）,Pulmonaryartery(dilation),Pulmonarycirculation(congestion),RV(dilation),LA(hypertrophy),LV(hypertrophy)（volumeejection）,bodycirculationBloodvolume,shunt,Hemodynamics,Bodycirculation（mixedblood）,RA,LA,PulmonaryArterydilation,RV（Dilation),Afterpulmonaryhypertension,LV,DynamicPulmonaryhypertesion,Obstructivepulmonaryhypertesion,shunt,ClinicalManifestation,Symptoms：frequentrespiratoryinfections,growslowly,poorweight,dyspnea,exerciseintolerance,fatigue,hoarseness(PApressrecurrentlaryngealnerve).Signs：pansystolicmurmur,loud-,harshwideconduction,3rd-4thintercostalspaceatleftsternalborder.P2accent.,Examination,X-ray:aorticknobsmallerthannormalmainPAsegmentprotrusionLVenlargementincreaselungmarkingsECG:LVhypertrophyUS:interventricularseptumintermitted,ComplicationsandTreatment,ComplicationsbronchopneumoniacongestiveheartfailurepulmonaryedemainfectiousendocarditisTreatmentminordefect：mayavoidoperation.mediadefect：operationat5-6yearsold.majordefectwithcomplications:operationat6m-2y.,AtrialSeptalDefect（ASD）,5%-10%Bioanatomy：1.ostiumprimum：15%.2.ostiumsecundum：mostcommon，75%.3.venoussinus：5%.4.coronaryvenoussinus：2%.,AtrialSeptalDefect（ASD）,Hemodynamic,Venaecavae,RA(dilation),RV(dilation),PulmonarycirculationcongestionArteriolespasm、thickened,Righttoleftshunt,Eisenmengersyndrome,Pulmonaryvein,LA,LV(blood),Bodycirculationischemia,ASDEtibiology,Pulmonarycirculationbloodvolumeincrease,Bodycirculationbloodvolumedecrease,ClinicalManifestation,Symptoms：similartoVSD.Auscultation：S1accent，P2accentejectivesystolicmurmurat2ndleftintercostalspace,loud-(pulmonaryarteryvalverelativelynarrow).,Test,X-ray：RAandRVenlargement.“hilusdance”,pear-shapedheart.ECG：rightaxisdeviation，incompleterightbundlebranchblock.B-US：RAandRVenlargement,paradoxicalmovementofinterventricularseptum.,ComplicationsandTreatment,Complicationsbronchopneumonia、heartfailure.Treatmentoperationbeforeschoolcatheterization,15%ofCHD,PatentDuctusArteriosus（PDA）,Anatomy,TubulartypeInfundibulartypeWindowtype,Pathobiology,1、Pulmonarycirculationcongestion2、Bodycirculationischemia3、Whenpulmonarypressurehigherthanaorta,righttoleftshuntoccurs,lowerlimbscyanosisdifferentialcyanosis,PDA,Clinicalmanifestation,Syndromes：similartoASDandVSDSigns：continuousmachinerymurmuratthe2ndleftintercostalspace,occupiesthewholesystolicanddiastolicstages.Peripheralbloodsigns:widepulsepressure,capillarypulsation,waterhammerpulse.,Test,X-ray：LVandLAenlargement,PAprotrusion,aorticknobprotrude.ECG：LVenlargement.USCathererization,ComplicationsandTreatment,Complicationspneumonia,heartfailure,subacuteinfectiousendocarditisTreatmentmedicineattheneonatalstage:ibuprofen,TetralogyofFallotTOF,Occupies10-15%CHDThemostcommoncyanoticCHD,70%ofcyanoticCHDafter1y.,Pathoanatomy,RVoutflowobstructionVSDaorticoverrideRVhypertrophy,Pathobiology,RVoutflowductobstruction，partofthevenousbloodinRVruntoLVthroughVSD，anotherpartruntotheoverrideaorta,whichcausethepersistentwhole-bodycyanosis；thepulmonarycirculationbloodvolumedecrease,exchangeofoxygendecrease,whichaggravatescyanosis.,Hemodynamics,Clinicalmanifestation,Cyanosisshowupat3-6m，lipsandnailsusually,causedbyPAstenosis.Squattingpositiontohelpthebloodrunbacktoheart.Dyspneaandhypoxemicspellsinfantstage,suddenonset,dyspnea,cyanosisaggravated,coma,convulsion,unconsciousness.,Clinicalmanifestation,Clubbingofthefingersandtoesthehypoxiccapillarydilation，hypertrophyofthesofttissueandbones.Heartsignsleft2nd-4thintercostalsystolicharshmurmur(rightventricularoutflowstenosis)Complicationscerebralembolism,cerebralabcess,subacuteendocarditis.,Test,Bloodroutine：RBCandHb.X-ray：“bootshapeheart”，aortaknobprotrude，RVhypertrophy,PAknobconcave.Pulmonarymarkingsdecrease.ECG：RVhypertrophy.Echocardiography：aortadilationandoverridingtheseptum,RVoutflowstenosis.Catheterization,Treatment,1、Generaltreatmenttreatthehypoxicspellschest-kneepositionoxygeninhalationsedationcorrec