先天性心臟病英文課件

CongenitalHeartDiseaseJieTianM.D.Children’sHospitalofCUMSCongenitalHeartDiseaseJieTi1先天性心臟病英文課件2先天性心臟病英文課件3先天性心臟病英文課件4先天性心臟病英文課件5先天性心臟病英文課件6先天性心臟病英文課件7EpidemiologyofCHDPrevalenceCHDoccursin0.5-0.8%oflivebirths;Theincidenceishigheramongstillborns

死產(chǎn)(3-4%),abortuses流產(chǎn)(10-25%),andprematureinfants(about2%excludingPDA);About2-3in1,000newborninfantswillbesymptomaticwithheartdiseaseinthefirstyearoflife.EpidemiologyofCHDPrevalence8EpidemiologyofCHDPrevalenceThediagnosisisestablishedby1weekofagein40-50%ofpatientswithCHDandby1monthofagein50-60%patients;Withtheadvancesinbothpalliative姑息andcorrectivesurgeryofthelast20years,thenumberofchildrenwithCHDsurvivingtoadulthoodhasincreaseddramatically;EpidemiologyofCHDPrevalence9EpidemiologyofCHDPrevalenceDespitetheseadvances,CHDremainstheleadingcauseofdeathinchildrenwithcongenitalmalformations;Mostcongenitaldefectsarewelltoleratedinthefetusbecauseoftheparallelnatureofthefetalcirculation.EpidemiologyofCHDPrevalence10EpidemiologyofCHDEtiologyThecauseofmostCHDisunknown.GeneticfactorsplaysomeroleinCHD.Abouteightpercentofcasesresultmainlyfromgeneticfactors.TheheartdefectisusuallypartofachromosomaldisordersuchasDown'ssyndrome.However,nospecificgenelocusforCHDhasbeenidentified.EpidemiologyofCHDEtiology11EpidemiologyofCHDEtiologyAchildborntoaparentwithCHDhasasubstantially大體上increasedlikelihoodofhavingasimilarcongenitallesion.Theriskmaybeashighas15percent.Forcertainlesions,thereappearstobeagreaterriskoftransmissionfromthemotherthanfromthefather.EpidemiologyofCHDEtiology12EpidemiologyofCHDEtiologyAbouttwopercentofcasesofCHDareprimarilytheresultofenvironmentalorexternalfactors.Suchfactorsincluderubella風疹infectionandingestionofcertaindrugs,suchaslithium鋰.CHDisaprominentcomponentofthefetalalcoholsyndrome.Theetiologicroleofotheragents,suchasanticonvulsantmedicationsandexogenous外源femalesexhormones,isuncertain.

EpidemiologyofCHDEtiology13NoninvasiveDiagnosticStudies

ChestXray

ThelocationoftheheartThesizeoftheheartTherelationshipbetweenheartandgreatvesselThebloodflowofthelungNoninvasiveDiagnosticStudies14先天性心臟病英文課件15NoninvasiveDiagnosticStudies

Echocardiography

Echocardiographyhasgreatvalueinassessingcongenitalcardiacanomaliesandshouldusuallybethefirstadvanceddiagnosticstudytobecarriedoutifthehistory,thephysicalexamination,thechestXray,andtheelectrocardiogramsuggestthepresenceofcongenitalheartdisease.NoninvasiveDiagnosticStudies16NoninvasiveDiagnosticStudies

Echocardiography

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

NoninvasiveDiagnosticStudies17NoninvasiveDiagnosticStudies

Dopplerultrasonography

Dopplerultrasonographyisusefulindetectingseptaldefectsanddirectlyassessingtheamountofbloodthatshuntsthroughthedefect.ThesizeoftheshuntthroughaseptaldefectcanalsobeestimatedfromDopplerultrasoundstudiesbycomparingthevelocityofthebloodflowthroughtheaortawithvelocitythroughthepulmonaryartery.

NoninvasiveDiagnosticStudies18先天性心臟病英文課件19NoninvasiveDiagnosticStudies

Transesophagealechocardiography

Transesophagealechocardiographyis

particularlyvaluableforassessingatrialseptaldefects,butitalsovisualizesotherlesionseffectively.Dopplerstudiesareusefulinassessingvalvularstenosisandregurgitationaswell.NoninvasiveDiagnosticStudies20先天性心臟病英文課件21NoninvasiveDiagnosticStudies

Computedtomography(CT)

CTprovidesagooddisplayoftheanatomicabnormalitiesassociatedwithcongenitalheartdiseaseandoffersadvantagesoverechocardiographyindemonstratinganomaliesinvolvingthegreatvessels.NoninvasiveDiagnosticStudies22ComputedtomographyComputedtomography23NoninvasiveDiagnosticStudies

Magneticresonanceimaging(MRI)

MRIprovidesinformationsimilartothatprovidedbyCT.MRIoffersbetterresolutionthanCTwithoutthedisadvantagesoftheradiopaque不透射線的contrastmediumusedinthattechnique.NoninvasiveDiagnosticStudies24先天性心臟病英文課件25先天性心臟病英文課件26NoninvasiveDiagnosticStudies

Cardiaccatheterizationandselectiveangiocardiography

arethemostdefinitivediagnostictechniquescurrentlyavailablefor

useincongenitalheartdisease.However,noninvasivestudiesoftenprovideinformationthatisequivalenttothatobtainedfromcardiaccatheterizationandissufficientforplanningsurgicaltreatment.NoninvasiveDiagnosticStudies27先天性心臟病英文課件28CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefect(VSD)VSDisthemostcommoncongenitalcardiacanomalyininfants.ItisrarelyseeninadultsbecausesubstantialVSDthatarenotcorrectedsurgicallyareassociatedwithahighmortality.Inaddition,theincidenceofspontaneousclosureofVSDisrelativelyhigh;closureoccursparticularlyoftenininfancybutalsoinlateryears.CongenitalCardiacAnomaliesi29先天性心臟病英文課件30CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectTheVSDthatdoappearinadultsasisolatedanomaliesareusuallylessthan1cmindiameter.Becausetheopeningisquitesmall,normalsystolicpressurecanbemaintainedintherightventricleandinthepulmonaryartery.CongenitalCardiacAnomaliesi31CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectIninfantswithalargeVSD,medicalmanagementhastwoaims:tocontrolheartfailureandtopreventthedevelopmentofpulmonaryvasculardisease.Therapeuticmeasuresareaimedatthecontrolofheartfailuresymptomsandthemaintenanceofnormalgrowth.CongenitalCardiacAnomaliesi32CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefect

IndicationsforsurgicalclosureofVSDincludepatientsatanyagewithlargedefectsinwhomclinicalsymptomsandfailuretothrivecannotbecontrolledmedically.Infantsbetween6and12moofagewithlargedefectsassociatedwithpulmonaryhypertension,evenifsymptomsarecontrolledbymedication.CongenitalCardiacAnomaliesi33CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectSurgicalclosureisusuallyundertakentopreventinfectiveendocarditis.Theincidenceofthiscomplicationisnotwellestablished,butsurgeryappearstobehighlyeffectiveasaprophylactic

measure.CongenitalCardiacAnomaliesi34CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect(ASD)ASDisthemostfrequentcongenitallesionofmajorimportanceinadults.Itisoftennotdiagnoseduntiladultlife,eveninthepresentera,becauseitrarelyproducessymptomsinchildhoodandtheassociatedphysicalsignsareeasilyconfusedwiththecardiacfindingsinnormalchildren.CongenitalCardiacAnomaliesi35CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectThreetypesofatrialseptaldefectareclassifiedonananatomicbasis:ostiumsecundum第二孔,sinusvenosus,andostiumprimum第一孔.Allthreetypesareassociatedwithaleft-to-rightshuntattheatriallevelandvolumeoverworkoftherightventricle.CongenitalCardiacAnomaliesi36先天性心臟病英文課件37CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Bloodischronicallyovercirculatedthroughthelungsatnormalintracardiacpressurelevels.Increasedflowthroughthepulmonaryvalveproducesacharacteristicpulmonarysystolicejectionmurmur.Thepulmonaryvalvecloseslatebecauseofthereducedimpedance阻抗inthepulmonaryarterialsystem,causingawidesplittingofthesecondheartsound,theotherclassicfindinginASD.CongenitalCardiacAnomaliesi38CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectThesplittingremainsrelativelyfixedinrelationtorespiration;

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

CongenitalCardiacAnomaliesi39CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectTwomajorcomplicationsofASDarepulmonaryarterialhypertensionandrightventricularfailure.Pulmonaryarterialhypertensioniscausedbyelevatedpulmonaryvascularresistance;itdevelopsafteradolescenceinabout15percentofcases.Inthemostseverecases,anirreversibleplexiformarteriopathy叢狀的動脈病,similartothatseeninEisenmengersyndromeorprimarypulmonaryhypertension,ispresent.CongenitalCardiacAnomaliesi40CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

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

CongenitalCardiacAnomaliesi41CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Rightventricularfailuredevelopsasaresultoflong-standingvolumeoverload;itusuallyaffectspatientsolderthan40years.Rightventricularfailureisusuallyassociatedwithatrialflutterorfibrillationandisoftenlinkedtotricuspidregurgitation.Eventually,asyndromeofright-andleft-sidedcongestiveheartfailuredevelops,andatthisstage,itmaybedifficulttodifferentiateclinicallybetweenASDandsuchconditionsascardiomyopathyandmitralvalvedisease.CongenitalCardiacAnomaliesi42CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectSurgicalclosureofASDisaverysafeandhighlyeffectiveprocedure.Prophylacticsurgeryisthereforeindicatedinanypatientinwhomtheratioofpulmonarybloodflowtosystemicbloodflowis2:1orgreater.NearlyallpatientsinwhomASDcanbeclinicallydiagnosedexhibitatleastthisdegreeofleft-to-rightshunt.CongenitalCardiacAnomaliesi43CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Surgeryiscontraindicated治療或處置不當whenpulmonaryhypertensionapproachesthepressurelevelofthesystemiccirculationbecauseinsuchpatientstheoperativemortalityishighandtheelevatedpulmonaryvascularresistancedoesnotfallaftersurgery.CongenitalCardiacAnomaliesi44CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectNonsurgicalclosureusingcardiaccatheterizationwithanumbrellalikedevicehasbeenaccomplishedinpatientswithdefectslessthan2cmindiameter,mostofwhomhavebeeninfantsorsmallchildren.

CongenitalCardiacAnomaliesi45CongenitalCardiacAnomaliesinChildrenTetralogyofFallot

TheessentialanatomiccomponentsoftetralogyofFallotareventricularseptaldefectandpulmonarystenosis.Thedegreeofstenosisisusuallysevereenoughtocauseapredominantlyright-to-leftshunt,cyanosis,anddiminishedpulmonarybloodflow.Thepulmonarystenosismaybeeithervalvularorinfundibular;theinfundibularformusuallypredominatesinadults.CongenitalCardiacAnomaliesi46CongenitalCardiacAnomaliesinChildrenTetralogyofFallotTheothertwoanatomicfeaturespresentintetralogyofFallotareanoverridingaortaandrightventricularhypertrophy.Variationsexistinthedegreeofdextropositionoftheaorta,butthisanatomicdefectrarelyhasfunctionalimportance.CongenitalCardiacAnomaliesi47先天性心臟病英文課件48CongenitalCardiacAnomaliesinChildrenTetralogyofFallotThetechniqueofthetotallycorrectiveoperationhasbeenwellestablishedsincethe1960s,andthedefectisusuallyrepairedininfancyorchildhood.Patientswhosurvivetoadulthoodwithoutanoperationorwithonlyapalliativeshuntoperation,inwhichasystemicarteryisanastomosed匯合tothepulmonaryartery,maydisplayfairlygoodefforttolerance,showlittleornocyanosis,andhaveaseeminglygoodp