先天性心臟病(英文)課件

先天性心臟病(英文)CongenitalHeartDiseaseJieTianM.D.Children’sHospitalofCUMS先天性心臟病(英文)EpidemiologyofCHDPrevalenceCHDoccursin0.5-0.8%oflivebirths;Theincidenceishigheramongstillborns

死產(chǎn)(3-4%),abortuses流產(chǎn)(10-25%),andprematureinfants(about2%excludingPDA);About2-3in1,000newborninfantswillbesymptomaticwithheartdiseaseinthefirstyearoflife.先天性心臟病(英文)EpidemiologyofCHDPrevalenceThediagnosisisestablishedby1weekofagein40-50%ofpatientswithCHDandby1monthofagein50-60%patients;Withtheadvancesinbothpalliative姑息andcorrectivesurgeryofthelast20years,thenumberofchildrenwithCHDsurvivingtoadulthoodhasincreaseddramatically;先天性心臟病(英文)EpidemiologyofCHDPrevalenceDespitetheseadvances,CHDremainstheleadingcauseofdeathinchildrenwithcongenitalmalformations;Mostcongenitaldefectsarewelltoleratedinthefetusbecauseoftheparallelnatureofthefetalcirculation.先天性心臟病(英文)EpidemiologyofCHDEtiologyThecauseofmostCHDisunknown.GeneticfactorsplaysomeroleinCHD.Abouteightpercentofcasesresultmainlyfromgeneticfactors.TheheartdefectisusuallypartofachromosomaldisordersuchasDown'ssyndrome.However,nospecificgenelocusforCHDhasbeenidentified.先天性心臟病(英文)EpidemiologyofCHDEtiologyAchildborntoaparentwithCHDhasasubstantially大體上increasedlikelihoodofhavingasimilarcongenitallesion.Theriskmaybeashighas15percent.Forcertainlesions,thereappearstobeagreaterriskoftransmissionfromthemotherthanfromthefather.先天性心臟病(英文)EpidemiologyofCHDEtiologyAbouttwopercentofcasesofCHDareprimarilytheresultofenvironmentalorexternalfactors.Suchfactorsincluderubella風(fēng)疹infectionandingestionofcertaindrugs,suchaslithium鋰.CHDisaprominentcomponentofthefetalalcoholsyndrome.Theetiologicroleofotheragents,suchasanticonvulsantmedicationsandexogenous外源femalesexhormones,isuncertain.

先天性心臟病(英文)NoninvasiveDiagnosticStudies

ChestXray

ThelocationoftheheartThesizeoftheheartTherelationshipbetweenheartandgreatvesselThebloodflowofthelung先天性心臟病(英文)NoninvasiveDiagnosticStudies

Echocardiography

Echocardiographyhasgreatvalueinassessingcongenitalcardiacanomaliesandshouldusuallybethefirstadvanceddiagnosticstudytobecarriedoutifthehistory,thephysicalexamination,thechestXray,andtheelectrocardiogramsuggestthepresenceofcongenitalheartdisease.先天性心臟病(英文)NoninvasiveDiagnosticStudies

Echocardiography

ThestandardM-modedisplayandthetwo-dimensionaldisplayprovidesuchinformationaboutcardiacanatomyasthesizeofthecardiacchambers,theconnectionsofthegreatvessels,abnormalitiesofthevalves,andsubvalvularobstructions.

先天性心臟病(英文)NoninvasiveDiagnosticStudies

Dopplerultrasonography

Dopplerultrasonographyisusefulindetectingseptaldefectsanddirectlyassessingtheamountofbloodthatshuntsthroughthedefect.ThesizeoftheshuntthroughaseptaldefectcanalsobeestimatedfromDopplerultrasoundstudiesbycomparingthevelocityofthebloodflowthroughtheaortawithvelocitythroughthepulmonaryartery.

先天性心臟病(英文)NoninvasiveDiagnosticStudies

Transesophagealechocardiography

Transesophagealechocardiographyis

particularlyvaluableforassessingatrialseptaldefects,butitalsovisualizesotherlesionseffectively.Dopplerstudiesareusefulinassessingvalvularstenosisandregurgitationaswell.先天性心臟病(英文)NoninvasiveDiagnosticStudies

Computedtomography(CT)

CTprovidesagooddisplayoftheanatomicabnormalitiesassociatedwithcongenitalheartdiseaseandoffersadvantagesoverechocardiographyindemonstratinganomaliesinvolvingthegreatvessels.Computedtomography先天性心臟病(英文)NoninvasiveDiagnosticStudies

Magneticresonanceimaging(MRI)

MRIprovidesinformationsimilartothatprovidedbyCT.MRIoffersbetterresolutionthanCTwithoutthedisadvantagesoftheradiopaque不透射線的contrastmediumusedinthattechnique.先天性心臟病(英文)NoninvasiveDiagnosticStudies

Cardiaccatheterizationandselectiveangiocardiography

arethemostdefinitivediagnostictechniquescurrentlyavailablefor

useincongenitalheartdisease.However,noninvasivestudiesoftenprovideinformationthatisequivalenttothatobtainedfromcardiaccatheterizationandissufficientforplanningsurgicaltreatment.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefect(VSD)VSDisthemostcommoncongenitalcardiacanomalyininfants.ItisrarelyseeninadultsbecausesubstantialVSDthatarenotcorrectedsurgicallyareassociatedwithahighmortality.Inaddition,theincidenceofspontaneousclosureofVSDisrelativelyhigh;closureoccursparticularlyoftenininfancybutalsoinlateryears.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectTheVSDthatdoappearinadultsasisolatedanomaliesareusuallylessthan1cmindiameter.Becausetheopeningisquitesmall,normalsystolicpressurecanbemaintainedintherightventricleandinthepulmonaryartery.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectIninfantswithalargeVSD,medicalmanagementhastwoaims:tocontrolheartfailureandtopreventthedevelopmentofpulmonaryvasculardisease.Therapeuticmeasuresareaimedatthecontrolofheartfailuresymptomsandthemaintenanceofnormalgrowth.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefect

IndicationsforsurgicalclosureofVSDincludepatientsatanyagewithlargedefectsinwhomclinicalsymptomsandfailuretothrivecannotbecontrolledmedically.Infantsbetween6and12moofagewithlargedefectsassociatedwithpulmonaryhypertension,evenifsymptomsarecontrolledbymedication.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectSurgicalclosureisusuallyundertakentopreventinfectiveendocarditis.Theincidenceofthiscomplicationisnotwellestablished,butsurgeryappearstobehighlyeffectiveasaprophylactic

measure.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect(ASD)ASDisthemostfrequentcongenitallesionofmajorimportanceinadults.Itisoftennotdiagnoseduntiladultlife,eveninthepresentera,becauseitrarelyproducessymptomsinchildhoodandtheassociatedphysicalsignsareeasilyconfusedwiththecardiacfindingsinnormalchildren.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectThreetypesofatrialseptaldefectareclassifiedonananatomicbasis:ostiumsecundum第二孔,sinusvenosus,andostiumprimum第一孔.Allthreetypesareassociatedwithaleft-to-rightshuntattheatriallevelandvolumeoverworkoftherightventricle.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Bloodischronicallyovercirculatedthroughthelungsatnormalintracardiacpressurelevels.Increasedflowthroughthepulmonaryvalveproducesacharacteristicpulmonarysystolicejectionmurmur.Thepulmonaryvalvecloseslatebecauseofthereducedimpedance阻抗

inthepulmonaryarterialsystem,causingawidesplittingofthesecondheartsound,theotherclassicfindinginASD.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectThesplittingremainsrelativelyfixedinrelationtorespiration;

theaorticandpulmonarycomponentsremainaudiblysplitduringexpiration.Achestx-rayusuallyrevealsenlargementoftheheartandsignsofpulmonaryovercirculation,suchasalargepulmonarytrunkandincreasedpulmonaryvascularmarkings.Therelativeseverityoftheseconditionsreflectsthesizeoftheleft-to-rightshunt.

先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectTwomajorcomplicationsofASDarepulmonaryarterialhypertensionandrightventricularfailure.Pulmonaryarterialhypertensioniscausedbyelevatedpulmonaryvascularresistance;itdevelopsafteradolescenceinabout15percentofcases.Inthemostseverecases,anirreversibleplexiformarteriopathy叢狀的動脈病,similartothatseeninEisenmengersyndromeorprimarypulmonaryhypertension,ispresent.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Asaresultofpulmonaryhypertension,theleft-to-rightshuntfirstdecreases,thenbecomesbidirectional,andfinallyreverses;arightventricularpressureoverloaddevelops,pulmonarybloodflowisreduced,andthepatientbecomescyanotic.

先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Rightventricularfailuredevelopsasaresultoflong-standingvolumeoverload;itusuallyaffectspatientsolderthan40years.Rightventricularfailureisusuallyassociatedwithatrialflutterorfibrillationandisoftenlinkedtotricuspidregurgitation.Eventually,asyndromeofright-andleft-sidedcongestiveheartfailuredevelops,andatthisstage,itmaybedifficulttodifferentiateclinicallybetweenASDandsuchconditionsascardiomyopathyandmitralvalvedisease.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectSurgicalclosureofASDisaverysafeandhighlyeffectiveprocedure.Prophylacticsurgeryisthereforeindicatedinanypatientinwhomtheratioofpulmonarybloodflowtosystemicbloodflowis2:1orgreater.NearlyallpatientsinwhomASDcanbeclinicallydiagnosedexhibitatleastthisdegreeofleft-to-rightshunt.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Surgeryiscontraindicated治療或處置不當(dāng)whenpulmonaryhypertensionapproachesthepressurelevelofthesystemiccirculationbecauseinsuchpatientstheoperativemortalityishighandtheelevatedpulmonaryvascularresistancedoesnotfallaftersurgery.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectNonsurgicalclosureusingcardiaccatheterizationwithanumbrellalikedevicehasbeenaccomplishedinpatientswithdefectslessthan2cmindiameter,mostofwhomhavebeeninfantsorsmallchildren.

先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenTetralogyofFallot

TheessentialanatomiccomponentsoftetralogyofFallotareventricularseptaldefectandpulmonarystenosis.Thedegreeofstenosisisusuallysevereenoughtocauseapredominantlyright-to-leftshunt,cyanosis,anddiminishedpulmonarybloodflow.Thepulmonarystenosismaybeeithervalvularorinfundibular;theinfundibularformusuallypredominatesinadults.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenTetralogyofFallotTheothertwoanatomicfeaturespresentintetralogyofFallotareanoverridingaortaandrightventricularhypertrophy.Variationsexistinthedegreeofdextropositionoftheaorta,butthisanatomicdefectrarelyhasfunctionalimportance.先天性心臟病(英文)CongenitalCardiacAnomaliesinChildrenTetralogyofFallotThetechniqueofthetotallycorrectiveoperationhasbeenwellestablishedsincethe1960s,andthedefectisusuallyrepairedininfancyorchildhood.Patientswhosurvivetoadulthoodwithoutanoperationorwithonlyapalliativeshuntoperation,inwhichasystemicarteryisanastomosed匯合tothepulmonaryartery,maydisplayfairlygoodefforttolerance,showlittl