先心病-(ASD)课件.ppt

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1、Atrial Septal Defects ASDPresentedbyDr.MaysaAbdulHaqDirectedbyDr.AliHalabiJordanHospital11-9-2005Background:Anatrialseptaldefect(ASD)isadeficiencyoftheatrialseptum.ASDsaccountforabout10-15%ofallcongenitalcardiacanomalies.Types of ASDs:1-Ostiumsecundumdefect70%ofASDs.2-Ostumprimumdefect20%ofASDs.3-si

2、nusvenosusdefect.10%ofASDs.4-coronarysinusseptaldefect1%ofASDs.Normal heart for comparison.Cardiac Development:Cardiactissuesarefirstdetectableonthe18thor19thdayoffetallife.Cardiacdevelopmentcontinuesforthenextseveralweeks.Theatrialseptumbeginstoformduringthefourthweekandiscompletebytheendofthefifth

3、week.Age of presentation:ASD,ispresentatbirth;however,inmostcases,amurmurisnotaudibleuntilthechildisafewmonthsold.SymptomsusuallydonotoccurinindividualswithASDuntillatechildhood,adolescence,oradulthood.Secundumtype,sinusvenosus,andunroofedcoronarysinusdefectssometimesarenotdiagnoseduntilthethirddeca

4、deoflife.OstiumprimumASDsusuallyarediagnosedinthefirstfewyearsoflifebecauseofthepresenceofamitralregurgitationmurmur.Ostium Secundum:AdefectintheregionofthefossaovalisThemostcommonformofASDAssociatedwithstructurallynormalAVvalves.Itmaybesingleormultiple(fenestratedatrialseptum)Female:male3:1incidenc

5、e.Associatedlesions:partiallyanomalouspulmonaryvenousreturn.&Holt-Oram-syndrome.Ostium secundum ASD is the most common type of ASD.It occurs in the center of the septum between the right and left atrium.A variant of this type of ASD is called a patent foramen ovale(PFO)and is very smallContinueHeart

6、defects:ThemostcommonlesionisasecundumASD.Othersincludeventriculoseptaldefect(VSD),atrioventricular(AV)block,pulmonicstenosis(includingperipheralarterial),andmitralvalveprolapse.Continuerighthandofa6-month-oldinfantwithHolt-Oramsyndrome,showinghypoplasticrightthumb.ContLargebloodflowthroughtherights

7、ideoftheheartcauseenlargementofrightatriumandventricle,dilatationofthepulmonaryarterybutpulmonaryatrialBPisusuallynormal(absenceofhighpressurecommunicationbetweenPulm.andSys.Circulation.)PVRmayincreaseinadulthoodandresultinrighttoleftshunt.Continue4-Systolicejectionmurmuratmiddleandupperleftsternalb

8、order.3-Middiastolicrumblingmurmuratthelowerleftsternalborder.ThesefindingsindicateQp:Qsratioatleast2:1.Diagnosis:ChestX-Ray:shows*variabledegreesofrightventricleandatrialenlargement.*Enlargementofpulmonaryartery.*Increasepulmonaryvascularity.2-ECG:signsofrightventriclevolumeoverload.Rightaxisdeviat

9、ionRightventricularconductiondelay(rsR).3-Echocardiogram:Increasedrightventricularenddiastolicdimensions.AbnormalmotionofventricularseptumLocationandsizeofASDConfirmationoftheshunt.4-CardiacCatheterization:confirmationofthedefectMeasuringoftheshuntandPVP.(itsofconsiderablevolumewhenifmorethanorequal

10、s20/min/m2.Note:inthecaseofclassicalfeaturesofASDonphysicalexamination,CXRandECHOwhichIdentifyisolatedseccundumASD,thereisnoneedforthecardiancathbeforesurgery.Continue.Note:inthecaseofclassicalfeaturesofASDonphysicalexamination,CXR,andECHOwhichIdentifyisolatedsecundumASD,thereisnoneedforthecardiacca

11、thbeforesurgery.left-to-rightshunt,isofconsiderablevolumewhenifmorethanorequals20L/min/m2.Prognosis&Complications:15%ostiumsecunduminterminfantsclosespontaneously.Symptomsdontusuallyappearuntilthethirddecadeinlifeorlater:Pulmonaryhypertension,atrialdysrhythmias,AVvalveinsufficiency,andheartfailure,i

12、nitiallyappearsduringvolumeoverloadinpregnancy.InfectiveEndocarditisisextremelyrareandthereisnoneedforantibioticprophylaxis.Treatment:Surgeryortranscatheterdeviceclosure.Indications:1-allsymptomaticpatients.2-asymptomaticpatientswithQP:QSratioofatleast2:1.Timing:electiveclosureafteroneyearandbeforee

13、ntryofschool.ContinuePostOpcomplications:(HF,AF)Theriskisgreaterifdoneafter20yearsofage.PostOpprognosis:1-symptomsdisappearrapidly.2-enhancedphysicaldevelopmentandactivity.3-Heartsizedecreasestonormal.Ostium primum:Anostiumprimumatrialseptaldefect(ASD)islocatedinthemostanteriorandinferioraspectofthe

14、atrialseptum.Cleftofanteriorleafletofthemitralvalveisnoticed.Tricuspidvalveisusuallyfunctionallynormal.Development:Duringfetaldevelopment,therudimentaryatriumisdividedbytheseptumprimum,exceptforananteriorandinferiorspacethatistheostiumprimum.Theostiumprimumissealedbyfusionofthesuperiorandinferiorend

15、ocardialcushionsaroundthefifthweekofgestation.FailuretodosoresultsinanostiumprimumASD.OstiumprimumASDsaremostcommonlyassociatedwithDownsyndrome(trisomy21).ContinueSex:Themale-to-femaleratiois1:1.Age:Patientswithsmallerdefectsandlittleornomitralregurgitationmaypresentatanyagewithamurmurand/oranabnorm

16、alelectrocardiogram(ECG).ThosewithmoreseveremitralregurgitationtypicallypresentwithCHFinthefirst1-2yearsoflife.Pathophysiology:Shuntingispredominantlyleft-to-rightintheabsenceofpulmonaryvasculardiseaseorsignificantrightventricularoutflowtractobstruction.Thisresultsinvolumeoverloadoftherightatriumand

17、ventricleandpulmonaryovercirculation.Ifthemitralvalvecleftcausessignificantmitralregurgitation,theleftsideoftheheartbecomesvolumeoverloaded.Aleftventricletorightatriumshuntcanbepresent,whichfurtheroverloadsboththerightandleftheart.Treatment:theprimumformofASDisnotamenabletodeviceclosureinthecardiacc

18、atheterizationlaboratory.Thedeviceisunabletobeadequatelyseatedsecondarytoaninadequateinferiorrimofatrialseptaltissue.DefinitivemanagementofhemodynamicallysignificantprimumASDsandpartialAVcanaldefectsisoperativerepair.ContinueTiming:PatientswithanisolatedostiumprimumASDusuallyarereferredforelectivere

19、pairwhenaged3-5years.repairmayberecommendedatanearlieragebecauseofsignificantCHForbecauseoffailuretothrive,allpatientswithdocumentedmitralregurgitationbereferreduponpresentation,asonceregurgitationdevelops,makingrepairlesssuccessful.Sinus venosus:SituatedintheupperpartofAS,incloserelationtoentryofSV

20、C.SinusvenosusASDsaccountforonly10%ofASDsalwaysassociatedwithanomalouspulmonaryvenousdrainageoftherightupperpulmonaryveinintotheSVC.A sinus venosusdefectistheleastcommontypeofASDandislocatedintheupperportionoftheatrialseptum.AsinusvenosusASDoftenhasanabnormalpulmonaryveinconnectionassociatedwithit.F

21、ourpulmonaryveins,twofromtherightlungandtwofromtheleftlung,normallyreturnredbloodtotheleftatrium.UsuallywithasinusvenosusASD,apulmonaryveinfromtherightlungwillbeabnormallyconnectedtotherightatriuminsteadoftheleftatrium.Thisiscalledananomalouspulmonaryvein.ContinueAlmostallpatientswithsinusvenosusASD

22、becomsymptomaticwhentheyareyoungerthan40years.Thehaemodynamicdisturbance,clinicalpicture;ECG;C-Xrayaresimillertoostiumsecundum.DiagnosisbyEchocardiography&cardiaccath.Anatomicalcorrection:theinsertionofapatchtoclosethedefect.excellentsurgicalresultswithamortalityratenear0%canbeexpected.Ifrepairdoneb

23、eforeage15years.Coronary sinus:TheleastcommontypeofASDistermedanunroofedcoronarysinusorcoronarysinusseptaldefect.Aportionoftheroofofthecoronarysinusismissing,allowingshuntingofbloodfromtheleftatriumintothecoronarysinusandsubsequentlyintotherightatrium.usuallyassociatedwithotherformsofcongenitalheartdisease(eg.persistantLSVC.alsomaybeassociatedwithotherformsofASD,suchasthesecundumorprimumtypes,ContinuePatientsusuallypresentwithsymptomstypicalofotherASDsorwithsymptomsrelatedtotheirconcomitantdefects.InthepresenceofaLSVC,thesefenestrationscanresultinahistoryofbrainabscessorcerebralembolism.

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