心血管系统疾病-先天性心脏病（英文） .ppt

congenitalheartdisease jietianm d children shospitalofcums epidemiologyofchd prevalencechdoccursin0 5 0 8 oflivebirths theincidenceishigheramongstillborns死产 3 4 abortuses流产 10 25 andprematureinfants about2 excludingpda about2 3in1 000newborninfantswillbesymptomaticwithheartdiseaseinthefirstyearoflife epidemiologyofchd prevalencethediagnosisisestablishedby1weekofagein40 50 ofpatientswithchdandby1monthofagein50 60 patients withtheadvancesinbothpalliative姑息andcorrectivesurgeryofthelast20years thenumberofchildrenwithchdsurvivingtoadulthoodhasincreaseddramatically epidemiologyofchd prevalencedespitetheseadvances chdremainstheleadingcauseofdeathinchildrenwithcongenitalmalformations mostcongenitaldefectsarewelltoleratedinthefetusbecauseoftheparallelnatureofthefetalcirculation epidemiologyofchd etiologythecauseofmostchdisunknown geneticfactorsplaysomeroleinchd abouteightpercentofcasesresultmainlyfromgeneticfactors theheartdefectisusuallypartofachromosomaldisordersuchasdown ssyndrome however nospecificgenelocusforchdhasbeenidentified epidemiologyofchd etiologyachildborntoaparentwithchdhasasubstantially大体上increasedlikelihoodofhavingasimilarcongenitallesion theriskmaybeashighas15percent forcertainlesions thereappearstobeagreaterriskoftransmissionfromthemotherthanfromthefather epidemiologyofchd etiologyabouttwopercentofcasesofchdareprimarilytheresultofenvironmentalorexternalfactors suchfactorsincluderubella风疹infectionandingestionofcertaindrugs suchaslithium锂 chdisaprominentcomponentofthefetalalcoholsyndrome theetiologicroleofotheragents suchasanticonvulsantmedicationsandexogenous外源femalesexhormones isuncertain noninvasivediagnosticstudies chestxraythelocationoftheheartthesizeofthehearttherelationshipbetweenheartandgreatvesselthebloodflowofthelung noninvasivediagnosticstudies echocardiographyechocardiographyhasgreatvalueinassessingcongenitalcardiacanomaliesandshouldusuallybethefirstadvanceddiagnosticstudytobecarriedoutifthehistory thephysicalexamination thechestxray andtheelectrocardiogramsuggestthepresenceofcongenitalheartdisease noninvasivediagnosticstudies echocardiographythestandardm modedisplayandthetwo dimensionaldisplayprovidesuchinformationaboutcardiacanatomyasthesizeofthecardiacchambers theconnectionsofthegreatvessels abnormalitiesofthevalves andsubvalvularobstructions noninvasivediagnosticstudies dopplerultrasonographydopplerultrasonographyisusefulindetectingseptaldefectsanddirectlyassessingtheamountofbloodthatshuntsthroughthedefect thesizeoftheshuntthroughaseptaldefectcanalsobeestimatedfromdopplerultrasoundstudiesbycomparingthevelocityofthebloodflowthroughtheaortawithvelocitythroughthepulmonaryartery noninvasivediagnosticstudies transesophagealechocardiographytransesophagealechocardiographyisparticularlyvaluableforassessingatrialseptaldefects butitalsovisualizesotherlesionseffectively dopplerstudiesareusefulinassessingvalvularstenosisandregurgitationaswell noninvasivediagnosticstudies computedtomography ct ctprovidesagooddisplayoftheanatomicabnormalitiesassociatedwithcongenitalheartdiseaseandoffersadvantagesoverechocardiographyindemonstratinganomaliesinvolvingthegreatvessels computedtomography noninvasivediagnosticstudies magneticresonanceimaging mri mriprovidesinformationsimilartothatprovidedbyct mrioffersbetterresolutionthanctwithoutthedisadvantagesoftheradiopaque不透射线的contrastmediumusedinthattechnique noninvasivediagnosticstudies cardiaccatheterizationandselectiveangiocardiographyarethemostdefinitivediagnostictechniquescurrentlyavailableforuseincongenitalheartdisease however noninvasivestudiesoftenprovideinformationthatisequivalenttothatobtainedfromcardiaccatheterizationandissufficientforplanningsurgicaltreatment congenitalcardiacanomaliesinchildren ventricularseptaldefect vsd vsdisthemostcommoncongenitalcardiacanomalyininfants itisrarelyseeninadultsbecausesubstantialvsdthatarenotcorrectedsurgicallyareassociatedwithahighmortality inaddition theincidenceofspontaneousclosureofvsdisrelativelyhigh closureoccursparticularlyoftenininfancybutalsoinlateryears congenitalcardiacanomaliesinchildren ventricularseptaldefectthevsdthatdoappearinadultsasisolatedanomaliesareusuallylessthan1cmindiameter becausetheopeningisquitesmall normalsystolicpressurecanbemaintainedintherightventricleandinthepulmonaryartery congenitalcardiacanomaliesinchildren ventricularseptaldefectininfantswithalargevsd medicalmanagementhastwoaims tocontrolheartfailureandtopreventthedevelopmentofpulmonaryvasculardisease therapeuticmeasuresareaimedatthecontrolofheartfailuresymptomsandthemaintenanceofnormalgrowth congenitalcardiacanomaliesinchildren ventricularseptaldefectindicationsforsurgicalclosureofvsdincludepatientsatanyagewithlargedefectsinwhomclinicalsymptomsandfailuretothrivecannotbecontrolledmedically infantsbetween6and12moofagewithlargedefectsassociatedwithpulmonaryhypertension evenifsymptomsarecontrolledbymedication congenitalcardiacanomaliesinchildren ventricularseptaldefectsurgicalclosureisusuallyundertakentopreventinfectiveendocarditis theincidenceofthiscomplicationisnotwellestablished butsurgeryappearstobehighlyeffectiveasaprophylacticmeasure congenitalcardiacanomaliesinchildren atrialseptaldefect asd asdisthemostfrequentcongenitallesionofmajorimportanceinadults itisoftennotdiagnoseduntiladultlife eveninthepresentera becauseitrarelyproducessymptomsinchildhoodandtheassociatedphysicalsignsareeasilyconfusedwiththecardiacfindingsinnormalchildren congenitalcardiacanomaliesinchildren atrialseptaldefectthreetypesofatrialseptaldefectareclassifiedonananatomicbasis ostiumsecundum第二孔 sinusvenosus andostiumprimum第一孔 allthreetypesareassociatedwithaleft to rightshuntattheatriallevelandvolumeoverworkoftherightventricle congenitalcardiacanomaliesinchildren atrialseptaldefectbloodischronicallyovercirculatedthroughthelungsatnormalintracardiacpressurelevels increasedflowthroughthepulmonaryvalveproducesacharacteristicpulmonarysystolicejectionmurmur thepulmonaryvalvecloseslatebecauseofthereducedimpedance阻抗inthepulmonaryarterialsystem causingawidesplittingofthesecondheartsound theotherclassicfindinginasd congenitalcardiacanomaliesinchildren atrialseptaldefectthesplittingremainsrelativelyfixedinrelationtorespiration theaorticandpulmonarycomponentsremainaudiblysplitduringexpiration achestx rayusuallyrevealsenlargementoftheheartandsignsofpulmonaryovercirculation suchasalargepulmonarytrunkandincreasedpulmonaryvascularmarkings therelativeseverityoftheseconditionsreflectsthesizeoftheleft to rightshunt congenitalcardiacanomaliesinchildren atrialseptaldefecttwomajorcomplicationsofasdarepulmonaryarterialhypertensionandrightventricularfailure pulmonaryarterialhypertensioniscausedbyelevatedpulmonaryvascularresistance itdevelopsafteradolescenceinabout15percentofcases inthemostseverecases anirreversibleplexiformarteriopathy丛状的动脉病 similartothatseenineisenmengersyndromeorprimarypulmonaryhypertension ispresent congenitalcardiacanomaliesinchildren atrialseptaldefectasaresultofpulmonaryhypertension theleft to rightshuntfirstdecreases thenbecomesbidirectional andfinallyreverses arightventricularpressureoverloaddevelops pulmonarybloodflowisreduced andthepatientbecomescyanotic congenitalcardiacanomaliesinchildren atrialseptaldefectrightventricularfailuredevelopsasaresultoflong standingvolumeoverload itusuallyaffectspatientsolderthan40years rightventricularfailureisusuallyassociatedwithatrialflutterorfibrillationandisoftenlinkedtotricuspidregurgitation eventually asyndromeofright andleft sidedcongestiveheartfailuredevelops andatthisstage itmaybedifficulttodifferentiateclinicallybetweenasdandsuchconditionsascardiomyopathyandmitralvalvedisease congenitalcardiacanomaliesinchildren atrialseptaldefectsurgicalclosureofasdisaverysafeandhighlyeffectiveprocedure prophylacticsurgeryisthereforeindicatedinanypatientinwhomtheratioofpulmonarybloodflowtosystemicbloodflowis2 1orgreater nearlyallpatientsinwhomasdcanbeclinicallydiagnosedexhibitatleastthisdegreeofleft to rightshunt congenitalcardiacanomaliesinchildren atrialseptaldefectsurgeryiscontraindicated治疗或处置不当whenpulmonaryhypertensionapproachesthepressurelevelofthesystemiccirculationbecauseinsuchpatientstheoperativemortalityishighandtheelevatedpulmonaryvascularresistancedoesnotfallaftersurgery congenitalcardiacanomaliesinchildren atrialseptaldefectnonsurgicalclosureusingcardiaccatheterizationwithanumbrellalikedevicehasbeenaccomplishedinpatientswithdefectslessthan2cmindiameter mostofwhomhavebeeninfantsorsmallchildren congenitalcardiacanomaliesinchildren tetralogyoffallottheessentialanatomiccomponentsoftetralogyoffallotareventricularseptaldefectandpulmonarystenosis thedegreeofstenosisisusuallysevereenoughtocauseapredominantlyright to leftshunt cyanosis anddiminishedpulmonarybloodflow thepulmonarystenosismaybeeithervalvularorinfundibular theinfundibularformusuallypredominatesinadults congenitalcardiacanomaliesinchildren tetralogyoffallottheothertwoanatomicfeaturespresentintetralogyoffallotareanoverridingaortaandrightventricularhypertrophy variationsexistinthedegreeofdextropositionoftheaorta butthisanatomicdefectrarelyhasfunctionalimportance congenitalcardiacanomaliesinchildren tetralogyoffallotthetechniqueofthetotallycorrectiveoperationhasbeenwellestablishedsincethe1960s andthedefectisusuallyrepairedininfancyorchildhood patientswhosurvivetoadulthoodwithoutanoperationorwithonlyapalliativeshuntoperation inwhichasystemicarteryisanastomosed汇合tothepulmonaryartery maydisplayfairlygoodefforttolerance showlittle