adult congenital heart disease(76).ppt

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1、AdultCongenitalHeartDiseaseBoardReviewSeptember3,2003JimmyKlemis,MDOverviewUS:1,000,000adultswithcongenitalheartdz20,000morepatientsreachadolescentsyearlyCardiologistsmustHavedetailedknowledgeofcongenitaldz,bothrepairedandunrepairedClearlydefineeachpatientssurgicalandcorrectiveprocedures(readsurgica

2、lnotes)*AllfiguresfromACCSAPVunlessotherwisenotedDaniels,CJ.CongenitalHeartDisease.ACCSAPVSurgicalTerminologyinAdultCongenitalHeartDiseaseCongenitalHeartDisease.ACCSAPVAdultCongenitalHeartDiseaseAtrialSeptalDefectCoarctationofAortaTetralogyofFallotTranspositionofGreatArteriesCommonVentricle/FontanPr

3、ocedureEbstiensAnomalyEisenmengerSyndromePregnancyAtrialSeptalDefect1/1500livebirthsSecundummostcommonACHD(6-10%)RADPrimumassociatedwithotherendocardialcushiondefects(cleftAVvalves,inlettypeVSD)LADSinusVenosuslarge,associatedwithanomalouspulmonaryvenousdrainage(usuallyRsuperiorPV)Coronarysinus(rare)

4、associatedwithunroofedcoronarysinusASD-Anatomy/PrevalenceSecundum75%Primum15%SinusVenosus10%CorSinus(rare)BraunwauldsHeartDisease,6thedASD-ClinicalMajorityrepairedinchildhood,butmaypresentinadolescence/adulthoodAsymptomaticmurmur,abnlECG/CXRSymptomaticdyspnea/CHFCVA/emboliAtrialFibrillationAuscultat

5、ioninASDIncreasedflowacrossthepulmonaryvalveproducesasystolicejectionmurmurandfixedsplittingofthesecondheartsound.FixedsplittingofS2mayinpartbeduetodelayedrightbundleconduction.IncreasedflowacrosstheTVproducesadiastolicrumbleatthemidtolowerrightsternalborder.Olderptlosespulmejectionmurmurasshuntbeco

6、mesbidirectionalsignsofpulmHTN/CHFmaypredominateASD:TherapyPercutaneousClosureonlyforsecundum(contrainothers)adequatesuperior/inferiorrimaroundASDnoR-LshuntingSurgicalClosureGoodprognosis:closureage25,PApressure25orPA40,decreasedsurvivalduetoCHF,stroke,andafibCoarctationofAortaNarrowinginproximaldes

7、cendingaortaMaybelong/tubularbutmostcommonlydiscreteridgeNaturalhx:poorprognosisifunrepairedAorticAneurysm/dissectionCHFPrematureCADzCoarctationofAorta:ClinicalMostrepaired,butadultpresentationmaybe:HTNmurmur(continuousorsystolicmurmurheardinbackorSEM/ejectionclickofbicuspidAV)weak/delayedLEpulsesRi

8、bnotchingonCXRpathognomonicRibnotchingCoarctationRepairEdmundsCardiacSurgeryintheAdult,Ch47Surgicalcorrection1)Patchaortoplastywithremovalofsegmentandendtoendanastomosisorsubclavianflaprepair2)bypasstubegraftingaroundsegmentCoarcation:TreatmentDespitesurgery,patientsstillhavesignificantmorbidity/mor

9、talitywithaverageage38Upto70%ofrepairedpatientsstillgoontodevelopHTN,pathologynotwellunderstoodRecurrencein8-54%ofrepairs,canundergorepeatsurgeryorballoonangioplastyAorticAneurysm/ruputuremayoccurdespitesuccessfulrepairandcorrectionofHTN(freqaroundanastomosissiteonpatchrepair30%inonestudy)Coarctatio

10、n:FollowupEvery1-2yearsDocumentarm/legBPScreen/treatCADriskfactorsHTN:rest,provokedbyexerciseorseenonambulatorymonitoringECHO/dopplertoevalrecurrentMRIforaneurysmTetralogyofFallot4featuresMalalignmentVSDOverridingAortaPulmonicStenosisRVHVariabilitycorrelateswithdegreeofRVOTobstructionandsize/anatomy

11、ofPATetralogy:SurgicalTreatmentSystemicPulmonaryShuntBlalock-TaussigWaterston(RPA)Potts(LPA)CompleteRepairtakedownofpriorshuntpatchVSDresectionofsubpulmonicobstructiontransannularpatcharoundpulmvalveannulus(usuallyleadstoseverePI)Tetralogy:Treatment/complicationsSystemic-Pulmshuntleadstohighflowthro

12、ughPA,elevatedPVRandbranchPAdistortionsurvivalafterrepairworseinptwithpriorWaterstonorPottsshunt(?higherflow);someptwithBlalock-TaussigshuntsmaysurviveunrepairedintoadulthoodtheseptshouldbeevaluatedforpulmarterystenosisandPulmHTNTetralogy:Treatment/complicationsPriorpulmonaryvalveatresiaoranomalousL

13、ADmayhavehadprostheticorhomograftconduitvalveplacedbetweenRVandPAConduitscanundergoendothelialovergrowthandobstructionof“pseudoRVOT”canRxwithballoonangioplastyoroperativeconduitreplacementTetralogy:Risk/followupSCD25-100foldriskcanoccur2decadesaftercorrectionrelatedtoQRSduration180msec?DuetoPI/RVcon

14、ductiondefectatrialarrhythmiasalsocommonHemodynamiceffectsofPIChronicRVvolumeoverload,RVdysfunctionandexerciseintolerancePulmonicValveReplacementcandecreaseQRSdurationandstabilizeRVfxn;timingunclearbutearlierbetterthanlaterRVfxn:ECHOorMRITranspositionD-typePAarisesfromLV,AortafromRVandanterior/right

15、ofPAcyanosiscorrectedinitiallywithprostaglandintokeepductusopenandballoonatrialseptostomytoimprovesystemicsaturationrepairvia“atrialswitch”MustardprocedureSVC/IVCbaffledtoLA/LV/PAPulmVeinsbaffledtoRA/RV/AoSymptomfreesurvivaluntil2nd-3rddecadeofliferepairvia“arterialswitch”longtermdata?pulmonicvalve(

16、neo-aorticvalve)competence?,reimplantedcoronariesmaydevelopostialstenosesD-TranspositioncomplicationsComplicationsarrhythmias/SCDOnly18%maintainSR;mostgoontoSSS/Afib/Aflutter;pacemakeroftenneededsystemic(tricuspid)atrioventricularvalveregurgitation?TVRsystemic(RV)ventricularfailure15%haveCHFsxsby2nd

17、-3rddecadeRxtransplantorstagedArterialswitch(pulmbandingto“train”LV)baffleobstructionRare(5%)butseriouscomplication;venousmorecommonSuspectifnewupperextremityedema(venous)ornewCHFsxs(pulmvenous)ECHOorCathtoeval,pulmvenousobstructionRxwithsurgery,systemicvenouswithangioplasty/stentsPacerwiremustgotoL

18、VviaSVCbafflePatientssurvivingarterialswitchL-typeTranspositionAtrial-ventricularANDventricular-arterialdiscordancePhysiologicallycorrect,anatomicallyincorrect“congenitallycorrected”transpositionRVissystemicventricle,TVissystemicAVvalveAsymptomaticformanyyears,oftenintoadulthoodL-typetransposition:c

19、omplicationsAlthoughseeminglybenign,survivalisreducedwithonestudyshowing25%ofpatientsdiedbymeanage38ProgressiveHeartFailureArrhythmiasSCDAVblockAtrialarrhythmiasSevereAV(tricuspid)regurgitationTVRdifficulttoimageusingconventionalECHOMRIbecomingtestofchoiceforRVfunctionTheFontanPatientAnycongenitalan

20、omalywithaneffective“single”or“common”ventriclemayleadtoaFontanprocedureTricuspidAtresiaalsoanyotherformofrightsidedhypoplasiaoratresia.DoubleInletLV.HypoplasticLeftHeart.SomevariationsofDoubleOutletRVStagedProcedureBasicconceptistoprovidesystemicvenousreturndirectlytoPAandbypassventriclesystemic-pu

21、lmshunttostabilizepulmbloodflowbi-directionalGlennorhemi-FontanprocedureSVCflowdirectedtoPAandsys-pulmshuntligatedFinally,FontanprocedurewithIVCdirectedtoPAOlderFontan:includesRAincircuit;newermethodsbypassRAheartcenteronlineFontan:complicationsArrhythmiasmostptdevelopSSS/tachy-bradyHeartFailureRAma

22、ybecomeenlargedandsourceforthrombus(witholderFontan),canundergoFontanrevisionwithbypassofRA/extracardiacgraftUncorrectedpatientsdeveloppolycythemiaandtreatmentbecomespalliativeatthispointEbsteinsAnomalyAtrializationofRV,sail-likeTV,TR50%ASD/PFO50%ECGevidenceofWPWAgeatpresentationvariesfromchildhooda

23、dulthoodanddependsonfactorssuchasseverityofTR,PulmVascularresistanceinnewborn,andassociatedabnormalitiessuchasASDucch.orgMassivecardiomegaly,mainlyduetoRAEEbsteins:ClinicalPresentationPediatricmurmurAdult(unrepairedwithASD)atrialarrhythmiasmurmurcyanosisRLshuntNOTduetoPulmHTNbutTRjetdirectedacrossAS

24、DexerciseintoleranceSurgeryinptswithsignificantTR/sxsEisenmengersSyndromeFinalcommonpathwayforallsignificantLRshuntinginwhichunrestrictedpulmonarybloodflowleadstopulmonaryvaso-occlusivedisease(PVOD);RLshunting/cyanosisdevleopsGenerallyneedQp:Qs2:1EisenmengerComplicationsCoagulopathy/plateletconsumpt

25、ionBrainabcessesCerebralmicroemboliAirwayhemorrhageespeciallymovingfromlowerhigheraltitudes(airtravel,mountains)Eisenmenger:TreatmentSxs+polycythemiaphlebotomyCarefulifmicrocytosis,strongestpredictorofcerebrovasculareventsRULEOUTCORRECTABLEDISEASEOncediagnosisestablished,avoidaggressivetestingasmany

26、patientsdieduringcardiovascularproceduresDiureticsprn,oxygenDefinitive:HeartLungtransplantProstacyclintherapymaydelay,expensivePregnancyPregnancyShuntsgenerallyhandledprettywellunlessPulmvascularobstructivedz;usesamestandardstodecideifclosurewarrantedasinnon-pregLsidedobstructivelesionsAS,MS,Coarcta

27、tioncarrysignificantriskAS:cantoleratepeakgrad50Coarc:repairedneedsMRItoevalanastomosissitesbeforepregnant,ifaneurysmneedrepairbeforepregnantPhysiologymoreimptthantypeoflesionballoonvalvuloplastyifnecessary(besttodx/fixbeforepregnancy)Question1A24-year-oldIndianmanisseenafterasyncopalepisodethatoccu

28、rredwhilehewaswatchingafootballgameonTV.Hiswifenoticedthatafteraparticularlyexcitingplay,thepatientsuddenlyslumpedover.Sheshookhimhard,and,afterabout30seconds,hewokeupandsaidthatherememberednothingoftheincident.Thishasneverhappenedbefore.Upuntilthistime,hehashadnolimitationofphysicalactivity.Hispast

29、medicalhistoryissignificantinthathehadrepairoftetralogyofFallotatage4,atwhichtimeaVSDwaspatchedandarightventricularinfundibulectomywasdone.Physicalexaminationfindsnocyanosis.Bloodpressureis100/70mmHg,andpulseis65perminutewithanoccasionalprematurecontraction.Thelungsarecleartoauscultationandpercussio

30、n.Neckveinsare4cm.Thereisamidsternalincisionthatiswellhealed.Thereisaslightprecordialsystoliclift.S2issingle.ThereisaGradeII/VIsystolicejectionmurmurwithashortGradeII/VIdiastoliclow-pitchedmurmuralongtheleftsternalborder.ThereisnoS3orS4.TheECGshowsrightbundlebranchblockwithleftanteriorhemiblock.TheP

31、Rintervalis0.12seconds.Theechocardiogramrevealsaslightlydilatedrightventricleandparadoxicalmotionoftheinterventricularseptum.Dopplergradientacrosstherightventricularoutflowtractis35mmHg.Thereisevidenceofmoderatelyseverepulmonicregurgitation,andtherearenoleft-to-rightorright-to-leftshunts.Whatisthemo

32、stimportantdiagnostictestneededforthispatient?A.TEE.B.Electrophysiologystudy.C.Cardiaccatheterizationandangiography.D.Tilttabletest.E.24-hourambulatoryECG.Question1AnswerCommentThecorrectanswerisB.Thisyoungmanhadasudden,apparentlyunprovoked,syncopalepisode.HischildhoodrepairofatetralogyofFallotresul

33、tedinarightventricularscar.Hehastheusualpostoperativephysicalfindingsofarepairedtetralogy,withresidualrightventricularobstructionandpulmonicvalveinsufficiency.Suddendeathduetoventriculartachycardia-fibrillationisadangertothesepatients.Theydeserveanelectrophysiologicstudysince,inthiscase,syncopeisequ

34、ivalenttoabortedsuddendeath.2Catheter-deliveredballoonexpansiontechniquesarenowthetreatmentofchoiceforwhichoneofthefollowinglesionsinadults?A.Valvularpulmonicstenosis.B.Valvularaorticstenosis.C.Coarctationoftheaorta.D.Ebsteinsanomalyofthetricuspidvalve.E.Severemitralstenosis/regurgitation.2CommentTh

35、ecorrectanswerisA.Althoughcatheterballoonvalvuloplastyandaortoplastyhavebeenattemptedinalltheseconditions,onlypulmonaryvalvotomyhasachievedasuccesslevelconsistentwithbeingthetreatmentofchoiceinadults.Aorticstenosisrespondsinitiallytoballoonexpansionandmayserveasabridgetovalvereplacementsurgery,butis

36、associatedwithrapidrestenosis.SuccessrateswithcoarctationandEbsteinsanomalyarenotuniformenoughtodisplacesurgeryexceptinselectedpatients.Mitralstenosisintheabsenceofseveresubvalvulardiseasecanbesuccessfullytreatedbyballoonvalvuloplasty,butthepresenceofmoderatetosevereregurgitationisanindicationforsur

37、gery.3Animportantpredisposingcauseforlateatrialfibrillationfollowingclosingofanatrialseptaldefectis:A.Patchversussutureclosureofthedefect.B.Theageofthepatientatthetimeofsurgery.C.Rightventriculardysfunction.D.Sinusvenosusdefect.E.Concomitantmitralregurgitation.3CommentThecorrectanswerisB.Lateatrialf

38、ibrillationmayoccurinasmanyas25%ofpatientswithatrialseptaldefectandisdirectlyrelatedtotheageofthepatientattimeofsurgeryaswellastheageofthepatientatlatefollow-up.Itisalsoinfluencedbywhetherthepatienthadatrialfibrillationearlyinthepreoperativeperiodandmayrepresentinterruptionofinteratrialpathways.4Wha

39、tdeterminesthephysiologyintetralogyofFallot?A.Thesizeoftheventricularseptaldefect.B.Thepositionoftheventricularseptaldefect.C.Thepresenceofanatrialseptaldefect.D.ThedegreeofRVoutflowtractobstruction.E.Thepresenceofaleftsuperiorvenacava.4CommentThecorrectanswerisD.Thesizeoftheventricularseptaldefecti

40、ntetralogyofFallotisquiteuniform.Thepresentationofthepatientastowhetherheorsheisacyanoticorcyanoticisdeterminedbythedegreeoftherightventricularoutflowtractobstruction(RVOT).RVOTobstructiondeterminestheamountofright-to-leftshunting.5A36-year-oldmanisreferredforsuspectedASD.Heisemployed,active,andasym

41、ptomatic.ECGshowsanormalaxisandincompleterightbundlebranchblock.Chestx-rayshowsanenlargedrightheartsilhouetteandincreasedpulmonaryvesselsthroughoutthelungs.Echocardiographyconfirmsa3cmdiametersecundumASDwithalargeshunt.Thereisamildtricuspidregurgitationjetof2m/sec.Yourrecommendationiswhichoneofthefo

42、llowing?A.Yearlyfollow-up.B.Digoxin.C.Warfarin.D.Angiotensinconvertingenzymeinhibitor.E.Cardiacsurgicalrepair.5CommentThecorrectanswerisE.PatientswithlargeASDsshouldhavethemclosedbecausethenaturalhistoryofASDisashortenedlifespanduetoeventualrightheartfailurefromthevolumeoverload.Paradoxicalembolisma

43、ndpulmonaryhypertensionareadditionalconcernsasareatrialfibrillationanditssequelae.Therecommendedclosuretechniquetodayissurgical.Catheter-delivereddevicesarepromisingbuthavenotbeenperfected.DigoxinandACEinhibitorsareofnoknownvalue.Anticoagulationisnotindicatedunlessatrialfibrillationorotherindication

44、sforitarepresent.6Thebestapproachfortheadultpatientwithacalcifiedductusis:A.Medicalmanagement.B.Closureofthedefectatcardiaccatheterization.C.Surgicalclosureofthedefectutilizingcardiopulmonarybypass.D.Leftthoracotomyandsurgicalclosure.CommentThecorrectanswerisB.Thecalcifiedductusintheadultmustbehandl

45、edverycarefully.Inthepast,thetreatmentofchoicewassurgicalclosure,and,becauseofthepliabilityoftheductus,tohaveitdoneoncardiopulmonarybypass.Now,however,thecapabilityofclosingtheductusinthecathlabnegatestheneedforsurgery.7A50-year-oldAsianwomanisseenbecauseoftheonsetofpalpitationsforthepast24hours.She

46、hadfinisheda2-daybusridejustpriortotheonsetofthesymptoms.Shereportsthatforthepastyear,shehashadtostopafteroneflightofstairsbecauseoffatigueandshortnessofbreath.Shedenieschestdiscomfort.Onquestioning,sheadmitsheavyalcoholintake.Physicalexaminationrevealsahealthyappearingwomanwithbloodpressure150/70mm

47、Hgandpulseirregularlyirregularat140perminuteattheapex.Thelungsarecleartoauscultationandpercussion.Theneckveinsare8cmwithapredominantVwave.S2iswidelysplitwithlittlerespiratoryvariation.ThereisnoS3orS4.ThereisaGradeIII/VIsystolicejectionmurmuratthesecondinterspaceattheleftsternalborder.Thereisnoperiph

48、eraledema.Thechestx-rayisshown(Figure1).TheECGshowsatrialfibrillationwithleftaxisdeviationandrightbundlebranchblock.Themostprobablediagnosisis:A.Pulmonaryemboli.B.Alcoholiccardiomyopathy.C.Ostiumsecundumatrialseptaldefect.D.Primarypulmonaryhypertension.E.Ostiumprimumatrialseptaldefect.7CommentThecor

49、rectanswerisE.Patientswithsignificantatrialseptaldefectsoftendevelopatrialfibrillationwhentheygetolder.Thecluetotheatrialseptaldefectonexamisthesystolicejectionmurmuratthebase,accompaniedbyawidelysplitsecondheartsound.Thechestx-ray,withincreasedpulmonaryvascularmarkings,istypical.TheECGwithleftaxisd

50、eviationischaracteristicofASDoftheostiumprimumtype.ThemorecommonsecundumASDwouldbeexpectedtobeassociatedwithrightaxisdeviation.8A48-year-oldmanwithknownEisenmengerssyndrome(ventricularseptaldefectwithhighpulmonaryvascularresistance)isseenforhisannualvisit.Hecontinuestoworkfull-timeasacomputeroperato