先心病外科治疗

SurgeryforCongenitalHeartDiseases SeoulNationalUniversityChildren sHospitalYongJinKim M D EtiologicBasisofCongenitalHeartDiseases 1 Primarygeneticfactors 10 1 Chromosomal 5 10 2 Singlemutantgene 3 RecessiveDominant2 Genetic environmentalinteraction 90 1 Multifactorialinheritance majority2 Riskstooffspringofanaffectedparent3 EnvironmentalcontributionDrugsInfectionsMaternalconditions PotentialCardiovascularTeratogens 1 DrugsAlcoholAmphetaminesAnticonvulsantsChemotherapySexhormoneThalidomideRetinoicacid 2 InfectionsRubellaCoxsakievirus3 MaternalconditionsOldageDiabetesLupusPhenylketonuria4 Others MaternalRiskFactors FactorsMalformationAdvancedageTrisomy21MaternalCHDVariousDiabetesmellitusVSD TGA cardiomyopathySLEHeartblockPhenylketonuriaTOF VSD COA HLHSVirusesTeratogenic myocarditis cytomegalovirus herpes coxsackiB parvovirus MaternalDrugExposures DrugMalformationDiphenylhydantoinPS ASTrimetadioneVSD TOF TGA HLHSThalidomodeTOF TruncusarteriosusLithiumEbsteinanomaliesAlcoholVSD ASD PDA TOFAmphetamineVSD ASD PDA TGABirthcontrolpillsVSD TOF TGA StagesofHeartFormation 1 Earlybloodvesselformation1 Intraembryonicbloodvesselat13days2 Extraembryonicbloodvesselsat17days2 Developmentofheart1 Positionandcardiactubeat23daysHeartbeatingat26days 2 Formationofheartloopat8somites3 Formationofventricle4 Developmentofsinusvenosus3 Formationofcardiacsepta4 Formationofcardiacvalves5 Formationofarterialsystem6 Formationofgreatsystemicveins IncidenceofCongenitalHeartDiseases 1 LttoRtShunt 53 PDA17 ASD16 5 VSD13 AVSD3 5 Abn PVreturn3 2 RttoLtShunt 11 TOF4 5 TA3 PA VSD2 5 PA IVS0 5 3 AdmixtureLesion 15 TGA5 Univ Ht 5 Atrialisomerism 2 DORV 2 Truncus0 8 CorrectedTGA 0 5 4 ObstructiveLesion 15 Coarctation9 5 PS2 MSetc 1 5 LVOTO1 3 HLHS0 9 IAA0 6 5 ValvularLesionEbstein 1 AR 0 5 MR 0 5 SVaneurysm 0 5 6 MiscellaneousArrhythmia5 Vascularring0 5 EvaluationofCHDbyHistoryTaking 1 Infants1 Murmur2 SymptomsofCHFpoorfeeding lowweightgain tachypnea tachycardia sweating anxiety irritability frequentURI3 Symptomsofhypoxemiacyanosis hypoxicspell 2 Children1 Murmur2 SymptomsofCHFexerciseintolerance dyspneaonexertion frequentURI palpitation3 Syncope chestpain4 SymptomsofHypoxemiacyanosis hypoxicspell clubbing ToBeCorrectedinNeonate CriticalASHypoplasticleftheartsyndromeMitralvalvehypoplasiaAorticvalveandarchhypoplasiaHypoplasticleftventricleInterruptedaorticarchSymptomaticCOATGAObstructiveTAPVCTruncusArteriosusPAwithIVSPAwithVSDOthersymptomaticcomplexheartdiseases ToBeCorrectedinInfancy I CardiacanomalieswithpulmonaryoutflowtractobstructionDoubleinletventricleCriticalPSTricuspidatresiaDORVDOLVTGATOFPAwithorwithoutVSDCorrectedTGA ToBeCorrectedinInfancy II CardiacanomalieswithCHFLargeVSDAVSDDoubleinletventricleTricuspidatresiaTAPVCCOADOLVPAwithIVSCorrectedTGACardiacmass TGATruncusarteriosusSevereAS ARSevereMS MRInterruptedaorticarchDORVAortopulmonarywindowALCAPAPDATAPVR ToBeCorrectedinInfancy Childhood ASDAS LVOTO PartialAVSDEbstein sanomalyVSDwithPS VSDPSMitralstenosisAtrialisomerism Coronaryarteryanomaly A Vfistula AnomalouspulmonaryvenousdrainageValvularheartdiseasesComplexanomalieswithpreviouspalliationLatepresentingcardiacanomaliesCardiomyopathy SurgicalIndicationsandOptimalTimingofOperation PalliativeSurgery Systemic pulmonaryarteryshuntBlalock TaussigshuntUnifocalizationandshuntCavopulmonaryshunt BCPS RVOTreconstructionValvotomyPatchwideningValvedconduitPulmonaryarterybandingAtrialseptectomy Systemic PulmonaryArteryShunt Systemic pulmonaryarteryshuntisindicatedduetoage size anatomyorotherconditionswhen Complexanomalywithseverecyanosis irritability hypoxicepisodeCriticallyillneonatesorinfantsduetodecreasedpulmonaryflowFacilitatinggrowthofhypoplasticpulmonaryartery PulmonaryArteryBanding Pulmonaryarterybandingisindicatedtodecreasepulmonarybloodflow protectvasculardiseasewhen ControlofcongestiveheartfailureComplexormultipleVSD coarctation Singleventricle TricuspidatresiawithoutPSCPBmedicallycontraindicatedProtectionofpulmonaryvascularbedSingleventricle FontanoperationPreparationofLVforarterialswitchoperationTGAwithIVS restrictiveVSD AtrialSeptectomy FortheincreasingofeffectivepulmonaryflowandsystemicoxygensaturationIndicationofatrialseptectomy TGATricuspidatresiaPulmonaryatresia IVSMVandLVhypoplasiaDecreasingtendencyofindicationduetoearlytotalcorrectionorintervention ReparativeSurgery Non openheartsurgeryOpenheartsurgeryPalliativeprocedureCorrectiveprocedureAnatomiccorrectionPhysiologiccorrection Non openHeartSurgery PalliativeprocedureCorrectiveprocedurePDACOAVascularringandslingCoronaryarteryanomaliesStenoticvalvulardiseasesInflowocclusiontechniqueInstrumentaldilatation PatentDuctusArteriosus OpencommunicationusuallybetweenupperdescendingAoandproximalportionofLPASignificantPDA indicatedafter1stmonthProphylacticclosure 6 12moSxofheartfailureorfailuretothrive indicatedatanytimeSeverepulmonaryvasculardisease contraindicated CoarctationoftheAorta CongenitalnarrowingofupperthoracicaortaadjacenttotheductusarteriosusOperationisindicatedwhen Reductionofluminaldiameter 50 UpperbodyHT 150mmHginyounginfantWithCHFatanyageCOAwithVSDTwostagerepairOnestagerepairCOAwithotherimportantintracardiacdefectsOnestagerepair VascularRing AnomaliesofthegreatarteriesthatcompressthetracheaoresophagusDoubleaorticarchRtaorticarchwithretroesophagealanomalousLt SCAandligamentumarteriosumRtaorticarchwithretroesophagealligamentumarteriosumOperationisindicatedwhen ObstructiveSxandradiologicsignsofobstructionDivisionbythoracotomyCPBinassociatedcardiacanomaly VascularSling Lt pulmonaryarteryarisesanomalouslyfromRtpulmonaryarteryextrapericardially formaslingaroundtracheaSx Signsofobstruction indicatedTrachealanomalyorotherairwayproblems ASD VSD PDA LtSVC ThoracotomywithoutCPBMediansternotomywithCPB OpenHeartSurgery ASDandPAPVR Aholeofvariablesizeintheatrialseptumandismostcommoncardiacmalformationwithvariouslocationofdefect fossaovalis posterior ostium primum coronarysinus subcaval sinusvenosus UncomplicatedASDorofPAPVCwithRVvolumeoverload Qp Qs 1 5or2 0 anindicationScimitarsyndromeIsolatedPAPVCOptimalage under5yearsbutrecently1 2yearstoavoidRVvolumeoverload UnroofedCoronarySinusSyndrome AspectrumofcardiacanomaliesinwhichpartorallofthecommonwallbetweenthecoronarysinusandleftatriumisabsentOperationisadvisablewithdiagnosiswhenWithpersistentLtSVCArterialdesaturationRiskofcerebralembolismGoodresultsofoperationWithoutpersistentLtSVC CoronarysinusASD SameasforothertypeASDAssociatedwithothermajorcardiacanomaliesClearindicationforoperation TotalAnomalousPulmonaryVenousConnection ThesearenodirectconnectionbetweenanypulmonaryveinandtheLA Butrather allthepulmonaryveinsconnecttotheRAoroneofitstributariesDxisanindicationofoperationImmediaterepairwithDxinanyillneonate PreoppreparationisnotneededRepairshouldbedonenearlyalwaysbefore6moDxat6 12mo promptrepairisindicated VentricularSeptalDefect Ahole ormultipleholes betweenLt RtventricleSymptomaticlargeVSD anindicationofoperationBefore3mo indicatedinlargeVSDswithCHForrespiratorysymptomsModeratesizedVSDs Qp Qs 3 0 withfewsymptoms observationduringinfancySmallVSDs Qp Qs 1 5 notindicated riskofbacterialendocarditisSubarterialtype earlyrepairisindicatedbeforechildhood AtrioventricularSeptalDefect Abnormalitiesofatrioventricularvalveform functionandinteratrial interventricularcommunicationfrommaldevelopmentoftheendocardialcushionsPresenceofAVSD indicatedwithDxPartialAVSD 1 2yearsofageexceptCHForgrowthfailureCompleteAVSDwithgoodcondition 3 6moCompleteAVSDwithrefractoryCHForrespiratorySx indicatedpromptlyDevelopmentofpulmonaryvascularobstructivedisease notindicated CongenitalAorticStenosis ThevariousformsofLVOTOoccurincombinationwithothercardiaclesions IAA COA MVanomalies LVhypoplasia andobstructivetypesaresupravalvular valvular subvalvular intraventricularCriticalASinneonates urgent severeCHF LVdilatation hypertrophy InfantsandchildrenPressuregradient 75mmHgSxofangina syncope exerciseintolerance LVH pressuregradient 50mmHgPressuregradientover40mmHginsubvalvularlesiontopreventprogression AortopulmonaryWindow Abnormaldevelopmentofaortopulmonaryseptumcausedbyincompleteformationoftherightandleftconotruncalridges SymptomaticAPwindowisanindicationSymptomaticinfants indicatedwithDxRepairisadvisedbefore3moofage size increasedshunt pulmonaryhypertension OldchildrenshouldbeoperatedonunlessPVRrendertheminoperable AneurysmofSinusofValsalva Thinwalled tubularoutpouchings nearlyalwaysRtsinusoradjacenthalfofthenoncoronarysinusandwithanentirelyintracardiaccourse thatmayruptureintotheright rarelyleft heartchambertoformafistulaRupturedsinusofValsalva indicatedpromptlybecauseofabruptdevelopmentofCHFWithVSDorVSD AI promptrepairisindicatedLargeaneurysmsproducinghemodynamicderangement indicatedSmallandmoderatesizedaneurysmwithoutsymptom notindicated CorTriatriatum Ararecongenitalcardiacanomalyinwhichpulmonaryveinstypicallyenteraproximalleftatrialchamberseparatedfromthedistalleftatrialchamberbyadiaphragminthichthereareoneormorerestrictiveostia sinister dexter IndicationforoperationRestrictiveapertureinthepartitionisanurgentindicationSymptomsusuallydevelopearly andoperationisnecessaryinthe1styearoflifeLifeexpectancyafterrepairininfancyisexcellent CongenitalMSandMR AdevelopmentalmalformationofoneormoreofthecomponentsofMVapparatus includingLAwalladjacentinsufficiencyoracombinedlesionInfancyMildandmoderateSxwithoutMVR Supravalvularring commmissuralfusionOthercircumstances onlyforinfantswithheartfailureChildhoodConsiderationsforoperationaresimilarCHFSeverepulmonaryHTMVRshouldbewithheldwheneverpossible Ebsteinsanomaly AcongenitaldefectoftricuspidvalveinwhichtheoriginofseptalandposteriorleafletsorbotharedisplaceddownwardintotherightventricleandtheleafletsarevariablydeformedSymptomaticEbstein sanomalyisanindicationNeonatespresentinginextremes StarnesprocedureinfirstweekValverepairandASDclosure withimportantTRmoderateandseverecyanosisWPWsyndromes ablationofaccessoryconductionpathway PulmonaryStenosis AformofRVoutflowobstructioninwhichstenosisisusuallyvalvarorbothvalvar infundibularoronlyinfundibularCriticalPSinneonate indicatedwithDxPercutaneousballoonvalvotomyValvotomywithCPBTransannularRVOTpatchwideningValvotomywithinflowocclusiontechniquePSininfantsandchildren indicatedwithSx Prgradientover50mmHgSurgicaltreatmentisnotindicatedwithmildstenosis PAwithIntactVentricularseptum Acardiacanomalyinwhichthepulmonaryvalveisatretic coexistingwithvariabledegreeofrightventricleandtricuspidvalvehypoplasiaDxisanindicationofoperationSizeoftheTV Z valueofthetricuspidvalve 2 RVOTpatchEvaluationafter6 12moafterinitialprocedure TwoventriclerepairOneandhalfventriclerepairFontanprocedure TetralogyofFallot CharacterizedbyunderdevelopmentofRVinfundibulumwithanteriorandleftwarddisplacementDxisanindicationofoperationSymptomaticcomplicatedinearlylife EarlytotalcorrectionorShunt 1 2mo andtotalcorrection 1year Asymptomaticuncomplicated Totalcorrectionat3 24moMultipleVSDs LADfromRCA Initialshuntandtotalcorrection PulmonaryAtresiawithVSD ThesearenoluminalcontinuitybetweenRV pulmonarytrunkamongthegroupofTOF Itisusuallycongenital butmaybeacquired DxisanindicationofoperationConfluentandnormallydistributingPAs CompleterepairinearlylifePalliationandrepair 3 5year withconduitConfluentPAsdistributingtothemajorsegments Pul Segments 15 neednotunifocalizationrepairwithconduitin3 5yearsNonconfluentPAsdistributingtotheminorsegment MmultistageoperationwithunifocalizationPalliativeshuntonly DoubleOutletRightVentricle AcongenitalcardiacanomalieswhichbothgreatarteriesrisewhollyorinlargepartfromtheRV Itis then atypeofventriculoarterialconnection DxisanindicationofoperationSimpleDORVwithsubaorticVSD repairby6mowithPS repairlikeTOFDORVwithsubpulmonicVSD Taussig Bingheart arterialswitchoperationwithin1mowithPS REVorRastellioperationat3 5yearsDORVwithnoncommittedVSD VSDenlargementandintraventriculartunnelFontanoperation TranspositionofGreatArteries AcardiacanomalyinwhichtheAoarisesentirelyorinlargepartfromtheRV andPAfromLV atrioventricularconcordantconnectionandventriculoarterialdiscordantconnection SimpleTGAinneonate arterialswitchoperationwithin1moSimpleTGAbeyond30days rapidtwo stageoperationatrialswitchoperation Mustard Senning TGAwithVSD arterialswitchoperationasearlyTGAwithVSDandLVOTOLecompteoperationat6 18moRastelliorLecompteoperationat3 5years TricuspidAtresia AcardiacanomalyinwhichRVfailstoopenintoaventriclethroughaAVvalve ThereisthusauniventricularAVconnectionPVRisanimportantindicator 4unit contraindicaton2 4unit BCPS 2unit FontanoperationSymptomaticinearlylifeearlyshuntorPABBCPSorhemi Fontanat6 12moFontanat12 24moNonsymptomaticFontancandidate 12 30mo DoubleInletVentricle AcardiacanomalyinwhichbothatriaconnecttoonlyoneventricularchamberbyeithertwoseparateAVvalveoracommonAVvalveDxisanindicationofoperationSeptation 1 2yearsenlargeddominantventricletwocompetentnonstenoticAVvalvenopulmonaryorsystemicoutflowobstructionFontanoperation 1 2years 3 5mo systemicoutflowobstruction6 12mo BCPS1 2years FontanoperationCardiactransplantation InterruptedAorticArch Completeluminalandanatomicdiscontinuitybetweentwosegmentsoftheaorticarch andgeneralizednarrowingofLVOT posteriormalalignment muscleofMoulaert smallaorticannulus aortichypoplasiaDxisanindicationofoperationCoexistingcardiacanomaly notcontraindicationOne stagerepair preferredTwo stagerepair incomplicatedintracardiacanomaliesSingleventricleassociated alternativeplan HypoplasticLeftHeartSyndrome AwidespectrumofcardiacanomalywithvariousdegreeofhypoplasiaofthestructureoftheleftsideoftheheartDxisanindicationofoperationFirststagepalliation 1 30daysNorwoodoperationSecondstagepalliation 6 12moBCPSHemi FontanThirdstagecorrection 18 24moCompletionFontanCardiactransplantation Aorticdiameter 2 5mm TruncusArteriosus Acardiacanomalyinwhichonegreatartery arisingfromthebaseoftheheartbywayofatruncalvalve PAsproximaltotheoriginofthebrachiocephalicbranches Presenceoftruncusarteriosus anabsolutesurgicalindication Neonatalrepairisrecommended HomograftinterpositionProstheticvalveconduitAutologoustissuereconstructionRepairshouldbedonebefore6moPulmonaryvascularobstructivedisease contraindication CongenitallyCorrectedTGA Acardiacanomalywithventriculoarterialdiscordantconnection atrioventriculardiscordantconnection ThecirculatorypathwaysarethereforeinseriesThepresenceofCCTGAperseisnotanindication WithVSD indicationsforVSDWithVSD PS indicationsforTOFIsolatedTR indicationforMRWithcompleteheartblock pacingFontanprocedureindicated straddlingtricuspidvalvecompleteAVSDleftsidedtricuspidvalveincompetenceDoubleSwitchoperation anatomiccorrection AnatomicallyCorrectedMalpositionofGreatArteries Acardiacanomalyinthepositionofthegreatarteriesandnotincardiacconnection TheLVprobablyalwaysexhibitsawellformedsubaorticconusandtheRVhaslesswelldevelopedinfundibulumthannormalDiagnosisisnotanindicationforoperationCoexistingcardiacanomaliesareindicationsLargeVSDalwayspresentPulmonarystenosisisusualSubaorticstenosismayoccurTricuspidatresiaorTVhypop