Surgery for Congenital Heart Defects, Third Edition

730 Pages·2006·146.24 MB·English

S
URGERY for
CONGENITAL
HEART
DEFECTS
Third Edition
SurgeryforCongenitalHeartDefects,ThirdEdition. EditedbyJ.F.Stark,M.R.deLevalandV.T.Tsang
 2006 John Wiley & Sons, L td. ISBN: 0-470-09316-1
S
URGERY for
CONGENITAL
HEART
DEFECTS
Third Edition
Editors
J STARK, M. de LEVAL and VT TSANG
GreatOrmondStreetHospital
forChildrenNHSTrust,London
Illustratedby
MICHAEL COURTNEY
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Dedicated to the Children with Congenital Heart Disease
Contents
PrefacetotheThirdEdition . . . . . . . . . . . . . . . . . . . . . . . . . xix
Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . xxi
Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xxiii
ForewordtotheThirdEdition . . . . . . . . . . . . . . . . . . . . . . . . . xxix
ForewordtotheSecondEdition . . . . . . . . . . . . . . . . . . . . . . . . xxxi
ForewordtotheFirstEdition . . . . . . . . . . . . . . . . . . . . . . . . . xxxiii
Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xxxv
GENERALCONSIDERATIONS . . . . . . . . . . . . . . . . . . . . . . .
1: ClassificationandNomenclatureofCongenitalHeartDefects,R.H.Andersonand
F.J.Macartney . . . . . . . . . . . . . . . . . . . . . . 3
IdentificationoftheCardiacSegments,4 AtrialSitus(AtrialArrangement)and
Morphology,4 VentricularMorphology,5 MuscleBundlesWithintheRight
Ventricle,6 GreatArterialMorphology,7 JunctionsBetweentheCardiac
Segments,7 TypesofAtrio-ventricularConnection,7 ModesofAtrio-ventricular
Connection,8 TypesofVentriculo-arterialConnection,8 ModesofArterial
Connection,9 RelationsandCardiacPosition,9 ImplicationsforReporting
SurgicalResults,9 AdditionalCardiovascularAnomalies,9
2: SurgicalAnatomy,R.H.AndersonandA.E.Becker . . . . . . . . . . 11
CardiacShapeandPosition,11 PericardialCavity,11 RelationshipsoftheHeart,13
AnatomyofCardiacChambers,15 TheRightAtrium,15 TheLeftAtrium,19 The
Ventricles,20 TheVentricularSeptum,27 TheAtrio-ventricularJunctions,28
TheCardiacSkeleton,29 TheAtrio-ventricularConductionSystemandBundle
Branches,29 TheCoronaryCirculation,30
3: Echocardiography,I.D.SullivanandV.M.Gooch . . . . . . . . . . 33
BasicPrinciples,33 M-ModeandTwo-dimensionalEchocardiography,33 Doppler
FlowEchocardiography,33 Colour-flowDopplerInterrogation,35 TissueDoppler
Imaging,35 Three-dimensionalEchocardiography,35 QuantitativeMeasurement,
35 PracticalConsiderations,37 ImageOrientation,37 Trans-oesophageal
Echocardiography,38 Archiving,40 SequentialSegmentalAnatomy,41 Atrial
Situs(Arrangement)andVenousConnections,41 Atrio-ventricularConnection,44
Ventriculo-arterialConnection,45 AtriaandAtrialSeptum,48 Atrio-ventricular
Septum,48 VentriclesandVentricularSeptum,48 CardiacValves,51
viii CONTENTS
TheGreatArteries,51 CoronaryArteries,53 IntraoperativeEchocardiography,54
PostoperativeAssessment,57 Conclusion,60 Appendix,60 Abbreviationsin
FiguresSpecifictoThisChapter,60
4: FoetalEchocardiography,L.D.Allan . . . . . . . . . . . . . . . 63
OrganizationofFoetalEchocardiography,63 NormalFoetalCardiacAnatomy,64
TheFour-chamberView,64 ImagingtheGreatArteries,64 CardiacMalformations,
66 AbnormalitiesofConnection,66 AdditionalAbnormalities,68 Implicationsof
FoetalEchocardiography,69 Conclusions,70
5: MagneticResonanceImagingandComputedTomographyintheDiagnosisof
CongenitalHeartDefects,V.Mutharangu,A.M.TaylorandR.Razavi . . . . 73
MagneticResonance(MR)Imaging,73 Basics,73 Techniques,73 Spin-echo
Imaging,73 Gradient-echoImaging,74 Two-dimensionalGradient-echoImaging,
74 Three-dimensionalGradient-echoImaging,74 Velocity-encodedPhaseContrast
MR,74 GadoliniumContrast-enhancedMRAngiography,74 AcyanoticHeart
Defects,74 AtrialSeptalDefect,74 VentricularSeptalDefect,75 Coarctationof
theAorta,76 CyanoticHeartDefects,77 TetralogyofFallot,77 Pulmonary
Atresia,78 TranspositionoftheGreatArteries,80 Congenitallycorrected
transposition(ccTGA),81 Double-outletRightVentricle,82 HypoplasticLeftHeart
Syndrome,82 Conclusion,83 ComputedTomography(CT),84 MDCT
Technique,84 IndicationsforCardiovascularMDCT,84 VascularAnatomy,85
CardiacAnatomy,85 MRIvs.MDCT,86 FutureProspects,87
6: InvasiveInvestigationandExerciseTesting,G.DerrickandJ.F.N.Taylor . . . 91
InvasiveInvestigation,91 GeneralConductofanInvasiveInvestigation,91
VascularAccess,91 BloodOxygenSaturationMeasurements,93 Pressure
Measurements,93 BloodGasMeasurements,93 MeasurementsofPulmonary
VascularResistance,94 TestingthePulmonaryVascularResponse,94 Catheter
PlacementandHaemodynamicMeasurement,94 Angiography,95 Postoperative
Investigation,96 RisksofInvasiveInvestigation,97 Summary,97
CardiopulmonaryExerciseTestinginChildrenandAdolescents,98 Limitations,98
PotentialImpact,98
7: InterventionalCatheterizationinCongenitalHeartDefects,P.Bonhoefferand
Y.Boudjemline . . . . . . . . . . . . . . . . . . . . . . 101
Indications,101 OpeningofAbnormallyorNormallyClosedStructures,101
BalloonDilatation,101 ValvuloplastyforCongenitalValvularDiseases,101
Angioplasty,104 AtrialSeptum,106 EndovascularStents,106 Limitationsand
GeneralConclusionsonStentTechnology,109 ClosureofAbnormallyOpen
Structures,109 PatentDuctusArteriosus(PDA),109 AtrialSeptalDefect,110
MuscularVentricularSeptalDefects,112 PerimembranousVSD,113 Other
VascularAbnormalitiesandSurgicallyCreatedStructures,114 Trans-catheter
PulmonaryValveInsertion,114 Hybridapproaches,116
8: TheAnatomyofVentricularSeptalDefectsandTheirConductionTissues,
R.H.AndersonandA.E.Becker . . . . . . . . . . . . . . . . 121
BasicRulesRegardingDispositionoftheConductionSystem,121 Anatomyof
IsolatedVentricularSeptalDefects,122 DefectswithFibrousContinuityBetweenthe
LeafletsoftheAorticandTricuspidValves,123 DefectswithAortictoPulmonary
CONTENTS ix
FibrousContinuity,127 DefectswithEntirelyMuscularRims,128 Anatomyofthe
VentricularSeptalDefectinTetralogyofFallot,130 VentricularSeptalDefectsin
HeartswithConcordantAtrio-ventricularandDiscordantVentriculo-arterial
Connections,131 TheInterventricularCommunicationinDouble-outletVentricle,
133 VentricularSeptalDefectsinSingleOutletoftheHeart,136 VentricularSeptal
DefectswithDiscordantAtrio-ventricularConnections,136 HeartswithStraddling
Atrio-ventricularValves,139 StraddlingLeftAtrio-ventricularValves,140
StraddlingRightAtrio-ventricularValve,141
9: UseofPacemakersandDefibrillatorsinChildren,P.G.Rees . . . . . . . 145
PacemakerTerminology,146 IndicationsforPacemakerImplantationinChildren,
146 ChoiceofSystemtobeImplanted,148 WireImplantation,148 Implantation
Techniques,149 TemporarySystems,149 PermanentSystems,149 Pacemaker
Problems,156 ElectrodeDislodgement,156 HighPacingThreshold,156 Lead
Fracture,156 MuscleTwitching,157 Erosion,157 LeftVentricularLead
Malposition,157 Infection,157 Non-functioningWire,157 EndocardialLead
Extraction,157 ElectiveGeneratorReplacement,158 PacemakerFollow-up,158
Conclusion,158 ImplantableDefibrillators,158 Technique,159
10: CatheterAblationofArrhythmias,M.Lowe . . . . . . . . . . . . . 161
Conditions,161 AtrialSeptalDefect(ASD),161 TetralogyofFallot(TOF),161
TranspositionoftheGreatArteries,162 FunctionalSingleVentricleandFontan
Operation,162 OtherDefects,163 SurgicalAnatomy,163 CaseSelectionand
Preparation,163 ElectrophysiologicalStudy,163 Equipment,163 Conventional
ContactMapping,163 AdvancedMappingTechniques,164 AblationStrategies,
164 AtrialFlutterandIART,164 AtrialFibrillation,165 Ventricular
Tachycardia,165 ComplicationsofCatheterAblation,165
11: PerfusionTechniques,T.JonesandM.J.Elliott . . . . . . . . . . . . 167
Pathophysiology,167 Hypothermia,168 AlteredPerfusion,169 Haemodilution,
169 InflammatoryResponse,170 Emboli,171 TheExtracorporealCircuit,171
Prime,171 VenousReservoirsandVenousDrainage,172 BloodPumps,173
Oxygenators,173 HeatExchanger,173 ConductofCPB,174 HeparinDosage
andHeparinNeutralization,174 SurgicalAspects,174 Cannulation,174 Arterial
Cannulation,174 VenousCannulation,176 Venting,178 IntracardiacSuction,
179 BloodGasManagement,179 HypothermicCirculatoryArrestandLow-Flow
CPB,180 MyocardialPreservation,181 ManagementofVentilation,182 Special
Circumstances,182 LeftSuperiorVenaCava,182 OtherCannulationTechniques,
182 ControlofShunts,183 Ultrafiltration(UF),183 Conclusion,184
12: Anaesthesia,E.Sumner . . . . . . . . . . . . . . . . . . . 187
CardiacDefects,188 DefectswithHighPulmonaryBloodFlow,188 Defectswith
LowPulmonaryBloodFlow,189 TranspositionoftheGreatArteries,189 Left
VentricularOutflowTractObstruction,189 SystemicandPulmonaryVascular
Resistances,189 PulmonaryVascularResistance,189 SystemicVascular
Resistance,191 PreoperativeManagement,191 Induction,192 VascularAccess
Lines,193 MaintananceofAnaesthesia,194 CardiopulmonaryBypass,194
Post-bypass,196 Fast-tracking,197 PulmonaryHypertension,197 Re-do
Surgery,197 SpecialConsiderations,197 NorwoodOperation,197
UniventricularHeart,198 Transplantation,198 ClosedCardiacOperations,199
x CONTENTS
13: IntensiveCareManagementofthePostoperativePatient,D.Macraeand
J.Larovere . . . . . . . . . . . . . . . . . . . . . . . 203
CardiovascularSystem,203 Monitoring,203 VascularAccess,204 LowCardiac
Output,204 PharmacologicalManagementofLowCardiacOutput(LCO),205
Non-pharmacologicalStrategiesintheManagementofPostoperativeLowCardiac
Output,208 OtherCardiovascularDrugs,209 PulmonaryHypertension,209
CardiacTamponade,210 CardiopulmonaryResuscitation,210 Postresuscitation
Stabilization,211 FluidTherapyafterCardiacSurgery,211 VolumeReplacement,
211 BleedingandBloodTransfusion,211 ArrhythmiasandPacing,211 Early
PostoperativeTachyarrhythmias,212 PostoperativeBradycardiaandPacing,214
RespiratorySystem,214 OxygenTherapy,214 PositivePressureVentilation,215
WeaningandExtubation,215 VentilationinSpecialSituations,216 Analgesiaand
Sedation,216 GastrointestinalSystem,217 Nutrition,217 Gastroduodenal
Paralysis,218 StressUlceration,219 Chylothorax,219 NecrotizingEnterocolitis,
219 RenalSystemandFluidTherapy,219 PostoperativeFluidManagement,219
Electrolytes,220 CapillaryLeakSyndrome,220 PerioperativeRenalDysfunction,
220 AcuteRenalFailure(ARF),221 Sepsis,221 NeurologicalDysfunction,222
Tracheostomy,223 SurgicalTechnique,223 PosttracheostomyCare,225
Decannulation,226
14: PaediatricCardiacMechanicalSupport,A.KarimovaandA.Goldman . . . . 229
AimsofCardiacMechanicalSupport,229 ChoiceofMechanicalAssistanceDevice,
229 ExtracorporealMembraneOxygenation(ECMO),230 VentricularAssistance
Devices(VADs),230 Intra-aorticBalloonPump(IABP),231 Indicationsand
TimingforMechanicalSupport,232 PostcardiotomyCirculatoryFailure,232
CirculatoryFailureDuetoMyocarditisorCardiomyopathy,232 RapidDeployment
ECMOorExtracorporealCardiopulmonaryResuscitation(ECPR),233 Patient
SelectionCriteria,233 PracticalAspectsofMechanicalSupport,233 Cannulation,
233 ManagementofBleeding,234 DeterminingAdequacyofECMO,234
ManagementofECMOinPatientswithSystemic–PulmonaryShunt,235
ManagementofPatientsonMechanicalSupport,235 WeaningfromECMO,235
WhentoStopMechanicalSupport(HowLongIsLongEnough?),235 Complications
ofMechanicalSupport,236 Outcomes,236 FutureDevelopments,236
15: SurgicalApproaches,J.StarkandV.T.Tsang . . . . . . . . . . . . 239
MidlineSternotomy,239 Cannulation,242 ClosureofSternotomy,243
PosterolateralThoracotomy,244 Muscle-sparingIncision,246 Bilateral
Thoracotomy,246 EndoscopicApproach,247
SURGICALPROCEDURES . . . . . . . . . . . . . . . . . . . . . . . . .
16: Systemic-to-PulmonaryArteryShunts,C.vanDoornandM.R.deLeval . . . 251
Indications,251 Disadvantages,251 SurgicalTechnique,251 Classical
Blalock–TaussigShunt,251 PottsShuntandWaterstonShunt,254 Prosthetic
Systemic–PulmonaryArteryShunt,255 Results,259
17: PulmonaryArteryBanding,V.T.TsangandJ.Stark . . . . . . . . . . 261
ClassicalIndications,261 CurrentIndications,261 VentricularSeptalDefect,261
TranspositionoftheGreatArteries,262 Double-inletVentricleandTricuspidAtresia
withoutPulmonaryStenosis,262 OtherIndications,263 HypoplasticLeftHeart
(HLH),263 OperativeTechnique,263 Approach,263 Technique,263
CONTENTS xi
TheDegreeofPulmonaryArteryBanding,265 MaterialUsedforPulmonaryArtery
Banding,267 OtherapproachestoPAB,267 SpecialConsiderations,267
TranspositionoftheGreatArterieswithVentricularSeptalDefect,267 Double-inlet
VentricleandTricuspidAtresia,267 AbsentLeftAtrio-ventricularConnection,268
BandinginVerySmall/YoungInfants,268 Complications,268 PulmonaryArtery
ReconstructionafterBanding(Debanding),268 Results,268
18: AtrialSeptectomy,V.T.TsangandJ.Stark . . . . . . . . . . . . . 271
Indications,271 TGA,271 UniventricularHeart,271 SurgicalTechnique,271
Result,274
19: PersistentDuctusArteriosus,J.StarkandV.Hjortdal . . . . . . . . . . 275
Diagnosis,275 Indications,275 MethodsforClosureofPersistentDuctus
Arteriosus,276 Medical,276 SurgicalTechnique,276 Complications,282
Results,282
20: CoarctationoftheAorta,V.T.TsangandJ.Stark . . . . . . . . . . . 285
DiagnosisandMedicalManagement,285 SymptomaticInfants,285 Older
Children,286 SurgicalTechnique,286 ResectionandEnd-to-endAnastomosis,287
SubclavianFlapTechnique,289 ReversedSubclavianFlap,290 Extended
ResectionandAnastomosisoftheDistalAortatotheUndersurfaceoftheAorticArch,
291 PatchGraftAortoplasty,292 SimultaneousRepairofCoarctationoftheAorta
andIntracardiacDefects,293 EarlyComplications,293 Bleeding,293
ParadoxicalorReboundHypertension,293 Paraplegia,293 RenalFailure,293
AbdominalPain,293 Chylothorax,294 LateComplications,294 Re-coarctation,
294 RepairofRe-coarctation,294 Results,295 Long-termFollow-up,296
Hypertension,296 Aneurysm,296
21: InterruptionoftheAorticArch,J.L.Monro . . . . . . . . . . . . . 299
Anatomy,299 DiagnosisandMedicalManagement,299 SurgicalTechnique,300
MidlineApproach,300 RepairoftheAorticArchthroughtheLeftChest,302
Results,303 LateFollow-up,303 AnastomoticObstruction,304 LeftVentricular
OutflowTractObstruction,304 Summary,304
22: VascularRings,M.R.deLevalandM.J.Elliott . . . . . . . . . . . . 307
SurgicalAnatomy,307 DoubleAorticArch,307 VascularCompressionAssociated
withaLeft-sidedAorticArch,307 VascularCompressionAssociatedwithLeft-sided
AorticArchandRight-sidedUpperDescendingAorta,308 VascularCompression
AssociatedwithRight-sidedAorticArch,308 CervicalAorta,309 Pulmonary
ArterySling,309 Diagnosis,309 SurgicalTechnique,310 DoubleAorticArch,
310 VascularCompressionAssociatedwithLeft-sidedAorticArch,311 Vascular
CompressionAssociatedwithRight-sidedAorticArch,311 CervicalAorta,311
PulmonaryArterySling,311 TrachealStenosisandMalaciaAssociatedwithVascular
Rings,312 AssessmentoftheAirway,313 ManagementoftheMajorAirwaysin
AssociationwithPASling,313 PostoperativeSurveillance,316 Managementof
Tracheobronchomalacia,316 Results,317
23: AnomaliesoftheSystemicVenousReturn,C.vanDoornandM.R.deLeval . . 319
AnomalousVenousReturntotheSystemicVenousAtrium,320 Anatomy,320
SurgicalTechnique,320 SpecificSurgicalSituations,321 AtrialSwitch(Mustardor
xii CONTENTS
Senning),321 Atrio-ventricularSeptalDefect,321 FontanOperation,321
AnomalousVenousReturntothePulmonaryVenousAtrium,322 Anatomy,322
SurgicalTechnique,323 DrainageoftheLeft-sidedSuperiorVenaCavatotheLeft
Atrium,323 UnroofedCoronarySinuswithoutLeft-sidedSuperiorVenaCava,324
DrainageofInferiorVenaCavaorHepaticVeinstotheLeftAtrium,325 Anomalous
VenousReturntoBothAtria,325 CombinedAnomalousSystemicandPulmonary
VenousReturn,325 Results,326
24: TotalAnomalousPulmonaryVenousReturnandCorTriatriatum,V.T.TsangandJ.
Stark . . . . . . . . . . . . . . . . . . . . . . . . . 327
TotalAnomalousPulmonaryVenousReturn,327 Diagnosis,327 Indications,327
SurgicalTechnique,328 VerticalVein,329 SupracardiacType,329 Cardiac
Type,331 InfracardiacType,332 MixedType,334 EarlyComplications,335
PulmonaryOedema,335 PulmonaryHypertensiveCrises,335 PhrenicNerve
Damage,336 LateComplications,336 PulmonaryVenousObstruction,336
AnastomoticStricture,336 PulmonaryVenousStenosis,336 RepairofPulmonary
VenousObstruction(PVO),336 CongenitalStenosisofPulmonaryVeins,336
Heterotaxy,337 Results,337 AfterPrimaryRepair,337 Resultsafter
ReoperationsforPulmonaryVenousObstruction(PVO),338 CorTriatriatum,338
Diagnosis,338 Indications,339 SurgicalTechnique,339 Results,339
25: SecundumAtrialSeptalDefectandPartialAnomalousPulmonaryVenousReturn,
J.StarkandV.T.Tsang . . . . . . . . . . . . . . . . . . . 343
Diagnosis,343 Indications,343 TypesofAtrialSeptalDefect(ASD),344 Types
ofPartialAnomalousPulmonaryVenousDrainage,344 SurgicalTechnique,345
DirectClosure,345 PatchClosure,346 TechniquesforSpecialConditions,347
SinusVenosusDefect,347 RepairofCoronarySinusAtrialSeptalDefect,347
RepairofPartialAnomalousPulmonaryVenousReturn,347 RepairofDrainage
fromtheRightPulmonaryVeinstotheRightAtrium,348 RepairofDrainagefromthe
RightPulmonaryVeinstotheInferiorVenaCava(ScimitarSyndrome),349
AnomalousDrainagefromtheLeftLungintotheInnominateVein,351 ‘‘Residual’’
ASD,352 Complications,352 LatePericardialEffusionandTamponade,352
Arrhythmias,352 Results,352 ASDClosure,352 ClosureviaRightThoracotomy,
352 DeviceClosureofASD,353 RepairofSinusVenosusDefect,353 Repairof
ScimitarSyndrome,353
26: VentricularSeptalDefects,C.vanDoornandM.R.deLeval . . . . . . . 355
Diagnosis,355 Indications,355 LargeVSD,355 ModeratelyLargeVSD,356
SmalltoModerateVSDs,356 DoublyCommittedSubarterialVSDs,356 Surgical
Techniques,356 ClosurethroughtheRightAtrium,357 ClosurethroughtheRight
Ventricle,364 ClosurethroughaGreatArtery,366 ClosurethroughtheApex,369
VentricularSeptalDefectandAorticArchAnomalies,369 Trans-catheterDevice
ClosureofVentricularSeptalDefects,370 Results,370 ResidualVSD,370
ConductionDisturbances,370 HospitalMortality,370 Long-termResults,370
27: Atrio-ventricularSeptalDefects,A.D.Pacifico . . . . . . . . . . . 373
Diagnosis,374 PartialForm,374 Indications,374 SurgicalTechnique,375
Results,377 ComplicationsandLateResults,377 CompleteForm,377
Indications,377 SurgicalTechnique,378 Results,384 ComplicationsandLate
Results,384