Andersons pediatric cardiology 959
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ExtracardiacEvaluation
Inadditiontoappropriatecardiovascularevaluation,thoroughpreoperative
assessmentandmedicalintakeisimportanttoidentifyextracardiacconditions
thatmayaffectthemanagementandpostoperativeoutcomeofpatientswith
TOF/PA/MAPCAs,assummarizedinBox36.2.
Box36.2
PreoperativeEvaluationofExtracardiac
FeaturesThatCanAffecttheManagement
andOutcomeofPatientsWith
TOF/PA/MAPCAs
GeneticEvaluation
▪Allpatientsreceivegenetictestingforachromosome22q11deletion.
▪TestingforAlagilleorothersyndromesisperformedasindicated.
▪Childrenwithachromosome22q11deletionundergoimmunologic
evaluation.
Feeding/NutritionalEvaluation
▪Feedingevaluationbyoccupationaltherapy.
▪Higher-riskpatients(e.g.,22q11deletion,<1yearofage)undergoa
modifiedbariumswallowstudytoidentifysilenttrachealaspirationof
feeds.
▪Childrenwithsymptomsofgastroesophagealrefluxaretreatedwitha
protonpumpinhibitor.
Airway/RespiratoryEvaluation
▪Directedhistoryandphysicalexamtoelicitrespiratory/airwaysymptomsor
signs.
▪Patientsmeetinganyofthefollowingareconsideredformicrodirect
laryngoscopyandbronchoscopy.
▪Age<1year
▪Chromosome22q11deletion
▪Baselinestridorornoisybreathing
▪Historyofpostextubationstridor
▪Historyofdifficultairway,airwaycompression,orairwaymalacia
▪Historyoftracheostomy,prolongedpostoperativemechanical
ventilation,ordependenceonnoninvasivepositive-pressure
ventilationduetoairwayproblems.
▪Positiveswallowstudyorclinicalhistoryofaspiration.
OtherEvaluations
▪Localsubspecialistreferralsaremadeforpatientswithpositivefindingson
reviewofsystemsattheinitialmedicalintake.
MAPCA,Majoraortopulmonarycollateralartery;PA,pulmonaryatresia;
TOF,tetralogyofFallot.
GeneticTesting
AllchildrenundergoingunifocalizationsurgeryforTOF/PA/MAPCAsshould
havegenetictestingtoidentifythepresenceofachromosome22q11deletion,as
approximatelyone-thirdofpatientswithTOF/PA/MAPCAshaveadeletionof
chromosome22q11.51Thepresenceofachromosome22q11deletionhasbeen
reportedasariskfactorforadverseearlyoutcomeaftercardiacsurgeryandhas
beenassociatedwithworselateoutcomesafterunifocalization.18,45,52
Chromosome22q11deletionhasvariablephenotypicexpression,withDiGeorge
syndromeattheextremeendofthespectrum.Thus,inadditiontoairwayor
feedingissues(discussedlater),patientswitha22q11deletionmayalsobeat
riskforhypocalcemiaandperioperativeinfection,giventhemetabolicand
immunologicabnormalitiesassociatedwiththisgenotype.
ThereisalsoanassociationbetweenTOF/PA/MAPCAsandAlagille
syndrome,whichiscausedbymutationoftheJAG1gene.53Childrenwiththe
Alagillephenotypeand/orrecurrentpulmonaryarterystenosisshouldundergo
genetictesting.Thereislimitedinformationaboutanatomicdifferencesbetween
TOF/PA/MAPCAspatientswithAlagillesyndromeandthosewitha
chromosome22q11deletionornoidentifiedgeneticanomaly,butitisour
impressionthatthepulmonarycirculationtendstobemorehypoplasticinthe
former(seeFig.36.12).Inarecentstudyofourexperiencewithalmost500
patientswithTOF/PA/MAPCAs,wefoundthatthosewithAlagillesyndrome
werelesslikelytoundergocompletesingle-stagerepairandhadworseoutcomes
thanotherTOF/PA/MAPCAspatients,althoughresultswerestillgoodinmany
Alagillepatients.45
Respiratory,Aerodigestive,andNutritional
Evaluation
Someofthemostcommonandimportantsourcesofmorbidityafter
unifocalizationsurgeryrelatetorespiratoryandfeedingissues.Suchmorbidity
maybeespeciallyrelevantinpatientswithachromosome22q11deletion,who
areatriskforupperandlowerairwayandoropharyngealabnormalities,feeding
problems,andcompromisedairwayprotection,54–57allofwhichmayaffect
perioperativeoutcomes(seeBox36.2).Inourexperience,childrenwith
TOF/PA/MAPCAs,especiallythosewitha22q11deletion,oftenrequire
perioperativebronchoscopy.58Accordinglywenowconsiderpreoperative
bronchoscopyinpatientswithperceivedriskfactors(seeBox36.2)withthegoal
ofprospectivelyidentifyingairwayabnormalitiesthatmayaffectthehospital
course.Forexample,compressionofthelargeairwaysbyMAPCAsor
previouslyreconstructedpulmonaryarteriescanberelievedduringsurgeryifthe
problemisidentifiedbeforehand.Childrenwithchronicpulmonaryaspiration
mayrequireextensivesupportwithsecretionclearanceaftersurgery,andmay
benefitfromabaselinebronchoscopystudytohelpinformdecisionmakingin
theeventofprolongedpostoperativerespiratoryfailure.
Inaddition,chronicpulmonaryaspirationcancontributetothedevelopment
oflungdiseaseandconsequentelevationoftotalpulmonaryresistance,which
canexacerbatebothrightventricularhypertensionandrespiratorymorbidityin
theperioperativeperiod.Theimpactoflungdiseaseinchildrenwith
TOF/PA/MAPCAsmaybeparticularlyimportantinsofaraslong-termoutcomes
aredirectlyrelatedtorightventricularpressure.59Patientswithachromosome
