先天性心脏病(英文)

Congenitalheartdiseaseareabnormalitiesintheheart'sstructurethatarepresentatbirth.

Approximately8outofevery1,000newbornshavecongenitalheartdefects,rangingfrommildtosevere.Overview当前第1页\共有50页\编于星期三\21点1.Geneticfactor(internalfactor):Geneticandchromosomalaberrations2.Environmentalfactor(externalfactor):Highaltitude4.Inheritedfactor3.Otherrelatedfactors:Viralinfectionsofpregnancy,Motherswhoarediabetic,alcoholicsordrugaddictiveDrugsandmetabolicfactorsEtiology当前第2页\共有50页\编于星期三\21点Thehealthprotectionofpregnantwomanshouldbeenhanced.Highriskfactors,suchasdrugs,radiation,viralinfection,et.shouldbeavoided.SuitdosageFolicAcidshouldbefilledupinearlypregnancystage.Prevention当前第3页\共有50页\编于星期三\21点Doesthechildhaveheartdisease?EvaluatingachildwithaheartmurmurIsitcongenitalheartdisease?Ifitiscongenitalheartdisease,whatisthelesion?Whatistheseverityofthelesion?当前第4页\共有50页\编于星期三\21点Assessmentofachildforthepresenceofheartdisease

MajorSystolicmurmurgardeIIIormorespeciallywithathrillDiastolicmurmurCyanosisCongestiveheartfailureMinorSystolicmurmurlessthangradeIIIinintensity2.AbnormalS23.AbnormalECG4.AbnormalX-ray5.AbnormalBP当前第5页\共有50页\编于星期三\21点CHDAcyanoticCHDCyanoticCHDLtoRshuntsASDVSDPDAObstructivelesionsPulmonicstenosisAorticstenosisCoarctationofaortaMitralregurgitationRtoLshuntsTOFCompleteTGA当前第6页\共有50页\编于星期三\21点TOFPDAVSDASDFourCongenitalCardiacAnomaliesinChildren返回当前第7页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect(ASD)ASDisthemostfrequentcongenitallesionofmajorimportanceinadults.Itisoftennotdiagnoseduntiladultlife,eveninthepresentera,becauseitrarelyproducessymptomsinchildhoodandtheassociatedphysicalsignsareeasilyconfusedwiththecardiacfindingsinnormalchildren.当前第8页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectThreetypesofatrialseptaldefectareclassifiedonananatomicbasis:ostiumsecundum第二孔,sinusvenosus,andostiumprimum第一孔.Allthreetypesareassociatedwithaleft-to-rightshuntattheatriallevelandvolumeoverworkoftherightventricle.当前第9页\共有50页\编于星期三\21点当前第10页\共有50页\编于星期三\21点房间隔缺损当前第11页\共有50页\编于星期三\21点房间隔缺损—原发孔型位于房间隔的下部，紧邻房室瓣。当前第12页\共有50页\编于星期三\21点房间隔缺损—继发孔型亦称中央型或卵圆孔型当前第13页\共有50页\编于星期三\21点房间隔缺损—静脉窦型位置接近上腔静脉当前第14页\共有50页\编于星期三\21点房间隔缺损当前第15页\共有50页\编于星期三\21点当前第16页\共有50页\编于星期三\21点当前第17页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Bloodischronicallyovercirculatedthroughthelungsatnormalintracardiacpressurelevels.Increasedflowthroughthepulmonaryvalveproducesacharacteristicpulmonarysystolicejectionmurmur.Thepulmonaryvalvecloseslatebecauseofthereducedimpedance阻抗inthepulmonaryarterialsystem,causingawidesplittingofthesecondheartsound,theotherclassicfindinginASD.当前第18页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectThesplittingremainsrelativelyfixedinrelationtorespiration;

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

当前第19页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectTwomajorcomplicationsofASDarepulmonaryarterialhypertensionandrightventricularfailure.Pulmonaryarterialhypertensioniscausedbyelevatedpulmonaryvascularresistance;itdevelopsafteradolescenceinabout15percentofcases.Inthemostseverecases,anirreversibleplexiformarteriopathy丛状的动脉病,similartothatseeninEisenmengersyndromeorprimarypulmonaryhypertension,ispresent.当前第20页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

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

当前第21页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Rightventricularfailuredevelopsasaresultoflong-standingvolumeoverload;itusuallyaffectspatientsolderthan40years.Rightventricularfailureisusuallyassociatedwithatrialflutterorfibrillationandisoftenlinkedtotricuspidregurgitation.Eventually,asyndromeofright-andleft-sidedcongestiveheartfailuredevelops,andatthisstage,itmaybedifficulttodifferentiateclinicallybetweenASDandsuchconditionsascardiomyopathyandmitralvalvedisease.当前第22页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectSurgicalclosureofASDisaverysafeandhighlyeffectiveprocedure.Prophylacticsurgeryisthereforeindicatedinanypatientinwhomtheratioofpulmonarybloodflowtosystemicbloodflowis2:1orgreater.NearlyallpatientsinwhomASDcanbeclinicallydiagnosedexhibitatleastthisdegreeofleft-to-rightshunt.当前第23页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefect

Surgeryiscontraindicated治疗或处置不当whenpulmonaryhypertensionapproachesthepressurelevelofthesystemiccirculationbecauseinsuchpatientstheoperativemortalityishighandtheelevatedpulmonaryvascularresistancedoesnotfallaftersurgery.当前第24页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenAtrialSeptalDefectNonsurgicalclosureusingcardiaccatheterizationwithanumbrellalikedevicehasbeenaccomplishedinpatientswithdefectslessthan2cmindiameter,mostofwhomhavebeeninfantsorsmallchildren.

当前第25页\共有50页\编于星期三\21点房间隔缺损封堵伞当前第26页\共有50页\编于星期三\21点房间隔缺损封堵过程当前第27页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefect(VSD)VSDisthemostcommoncongenitalcardiacanomalyininfants.ItisrarelyseeninadultsbecausesubstantialVSDthatarenotcorrectedsurgicallyareassociatedwithahighmortality.Inaddition,theincidenceofspontaneousclosureofVSDisrelativelyhigh;closureoccursparticularlyoftenininfancybutalsoinlateryears.当前第28页\共有50页\编于星期三\21点当前第29页\共有50页\编于星期三\21点VSD当前第30页\共有50页\编于星期三\21点VSD当前第31页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectTheVSDthatdoappearinadultsasisolatedanomaliesareusuallylessthan1cmindiameter.Becausetheopeningisquitesmall,normalsystolicpressurecanbemaintainedintherightventricleandinthepulmonaryartery.当前第32页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectIninfantswithalargeVSD,medicalmanagementhastwoaims:tocontrolheartfailureandtopreventthedevelopmentofpulmonaryvasculardisease.Therapeuticmeasuresareaimedatthecontrolofheartfailuresymptomsandthemaintenanceofnormalgrowth.当前第33页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefect

IndicationsforsurgicalclosureofVSDincludepatientsatanyagewithlargedefectsinwhomclinicalsymptomsandfailuretothrivecannotbecontrolledmedically.Infantsbetween6and12moofagewithlargedefectsassociatedwithpulmonaryhypertension,evenifsymptomsarecontrolledbymedication.当前第34页\共有50页\编于星期三\21点CongenitalCardiacAnomaliesinChildrenVentricularSeptalDefectSurgicalclosureisusuallyundertakentopreventinfectiveendocarditis.Theincidenceofthiscomplicationisnotwellestablished,butsurgeryappearstobehighlyeffectiveasaprophylactic

measure.当前第35页\共有50页\编于星期三\21点室间隔缺损封堵伞当前第36页\共有50页\编于星期三\21点室间隔缺损封堵当前第37页\共有50页\编于星期三\21点PatentDuctusArteriosusPDA–PersistenceofthenormalfetalvesselthatjoinsthePAtotheAorta.Normallyclosesinthe1stwkoflife.Accountsfor10%ofallCHD,seenin10%ofothercongenitalhrtlesionsandcanoftenplayacriticalroleinsomelesions.Female:Maleratioof2:1Oftenassociatedw/coarctation&VSD.当前第38页\共有50页\编于星期三\21点动脉导管未闭PDA当前第39页\共有50页\编于星期三\21点PatentDuctusArteriosusQuestion: WhatTORCHinfectionisPDAassociatedwith?•

Answer: Rubella当前第40页\共有50页\编于星期三\21点PatentDuctusArteriosusHemodynamicsAsaresultofhigheraorticpressure,bloodshuntsLtoRthroughtheductusfromAortatoPA.Extentoftheshuntdependsonsizeoftheductus&PVR:SVR.SmallPDA,pressuresinPA,RV,RAarenormal.当前第41页\共有50页\编于星期三\21点PatentDuctusArteriosusHemodynamicsLargePDA,PApressuresareequaltosystemicpressures.Inextremecases70%ofCOisshuntedthroughtheductustopulmonarycirculation.Leadstoincreasedpulmonaryvasculardisease.当前第42页\共有50页\编于星期三\21点PatentDuctusArteriosusClinicalSigns&SymptomsSmallPDA’sareusuallyasymptomaticLargePDA’s