Andersons pediatric cardiology 2295
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Introduction
Thecomplexityofcongenitalcardiaclesionsandincreasedrequirementsfor
imagingstudiesandinterventionsinthisgrowingpatientpopulationnecessitate
thespecializedskillsofthepediatriccardiacanesthesiologist.Thegreatmajority
ofthesepractitionersundergoprimarytraininginanesthesiology,followedbya
fellowshipinpediatricanesthesiaandadditionaltraininginpediatriccardiac
anesthesia,mostrecentlyasa1-yearsubspecialtyfellowship.1Moreover,
specificallytrainedcertifiedregisterednurseanesthetistsmaytakepartinthe
careofchildrenwithcongenitalcardiacdisease.Thesemembersofthepediatric
cardiacanesthesiacareteamtreatpatientsinawidevarietyofsettings,suchas
theechocardiographylaboratory,cardiaccatheterizationlab,magneticresonance
imagingsuites,cardiacoperatingrooms,generaloperatingrooms(OR),and
cardiacintensivecareunits.Youngchildrenwithcongenitalcardiacdisease
undergorepeateddiagnostic,curative,orpalliativeproceduresinthesesettings,
whichfrequentlyrequiredeepsedationorgeneralanesthesiatoprovidethe
requiredimmobility.
PreoperativeAssessment
Sincethespectrumofcongenitalandacquiredcardiaclesionsisextremely
variedandpatienthistoriesoftencomplex,performingafocusedphysical
examinationandobtainingpertinentinformationregardingthespecificcardiac
anatomy,previouscardiacevaluationsandcatheterizations,historyofsurgical
proceduresandcomorbidities,arecriticaltodevisingasafeanestheticplan.
Particularattentionisgiventofactorsimpactingairwaymanagement,
cardiopulmonaryfunctionandreserve,andneurologicstatus.Forneonates,
detailsofprenatalandperinataleventsareobtained.Complicationsduringlabor
anddelivery,suchasneedforpostnatalmechanicalventilatorsupport,shouldbe
elicited.2Preparationincludestheappreciationforgeneticsyndromesthatcanbe
associatedwithcongenitalheartdisease(CHD)andmaysignificantlyimpact
anestheticmanagement(Table90.1).Forexample,certainsyndromescomplicate
airwaymanagement(e.g.,PierreRobin,choanalatresia),whileothersmayaffect
managementofbloodproductsandelectrolytemanagement,forexample
DiGeorgesyndrome.
Table90.1
SelectedGenetic/MetabolicDisordersWithCardiacInvolvementandMajorImplicationsfor
AnestheticManagement
Syndrome/Disorder
Trisomy13(Patausyndrome)
Trisomy18(Edwardssyndrome)
Trisomy21(Downsyndrome)
22q11.2Deletionsyndrome
(DiGeorgesyndrome)
Noonansyndrome
Turnersyndrome
Marfansyndrome
Williams-Beurensyndrome
Ehlers-DanlostypeIV
Holt-Oramsyndrome
Cri-du-Chatsyndrome
Mucopolysaccharidosis
(cardiomyopathy)
Fucosidosis(cardiomyopathy)
Tetrasomy12p(Pallister-Killian
syndrome)
AnestheticImplications
Centralsleepapnea;seizures
Difficultairway
Atlantoaxialinstability;upperairwayobstruction;bradycardiawith
inhalationalinduction
Hypocalcemia;immunodeficiency
Difficultairway
Difficultairway;hypothyroidism;diabetes
Duralectasia;jointlaxity;spontaneouspneumothorax;difficultairway
Hypercalcemia;difficultairway;anxietydisorder;jointcontractures;
prolongedneuromuscularblockade
Jointlaxity;positioninginjuries;difficultairway;atlantoaxialinstability
Arrhythmias;difficultairway;dysplasticupperextremities
Difficultairway;hypotonia
Difficultairway
Difficultairway
Difficultairway;hypertonia;hypotonia
Alagillesyndrome
CHARGE
VACTERL
Kabukisyndrome
Wolf-Hirschhorn
Liverfailure
Difficultairway
Difficultairway
Difficultairway;seizures;obstructivesleepapnea;hypotonia;jointlaxity
Difficultairway;hypotonia
Failuretothrive,difficultyfeeding,tachypneaorsweatingwithfeeding,
decreasedactivitylevel,andeasyfatigabilityaremarkersforimpaired
cardiopulmonaryreservethataretakenintoconsiderationwhenchoosing
anestheticdrugs,airwaymanagement,andpostoperativeventilatorstrategy.
Degreeofcyanosis(viapulseoximetry,visualinspection,orpolycythemia)and
theincidenceofhypoxemicspells(e.g.,intetralogyofFallot),orsyncope(e.g.,
inleft-sidedobstructivelesions)arecriticalforriskstratification.
Obtainingacardiovascularmedicationhistoryispartofanypreoperative
assessment,asmanyanestheticagentsinteractwithavarietyofmedicationsand
canresultinpotentiallysevereadversereactions.Childrenonmedicationsfor
congestiveheartfailure,suchasdiureticsandinotropicagents(e.g.,digitalis),
shouldhavepreoperativeelectrolytesandappropriatedrugserumlevels
checked.Patientswhoareonanticoagulantsmayneedtohavetheirmedications
eitherheld(e.g.,enoxaparin)ortransitioned(e.g.,Coumadin)priortotheir
plannedprocedure.Aspirintherapytopreventshuntthrombosisshouldusually
becontinued;manyrecommenda5-to10-dayperiod.Althoughsome
institutionsmayprefertocontinueallmedicationsthroughthedayofsurgery,
otherpractitionersmayrequestholdingangiotensin-convertingenzyme
inhibitorsorangiotensinIIreceptorblockersanddiureticsthedayofthe
procedureduetothepotentialforseverearterialhypotension.Thispracticeis
basedonstudiesofadultswithischemicheartdiseaseandhypertensioninwhich
hypotensiononinductionofanesthesiawasreported.However,otheradultdata
dismissestheseclaimsanddatainchildrenarelacking.3
Detailsofrecentillnesses,particularlyupperrespiratoryinfections,shouldbe
elicitedaschildrenwithongoingupperrespiratoryinfections(URIs)havebeen
showntohaveahigherincidenceofrespiratoryandpostoperativecomplications
aftercardiacsurgery.4ThereisevidencethatpatientswithanactiveURIhavea
higherincidenceofbronchospasm,laryngospasm,hypoxemia,atelectasis,and
extubationfailures.2Inaddition,postoperativelengthofstayisprolongedin
patientswithURIs,whileoverallmorbidityandmortalitymaynotbe
increased.2,4However,respiratorytractinfectionsmayhaveagreaterimpacton
pulmonaryvascularresistanceinchildrenwithpulmonaryhypertensionor
cavopulmonaryanastomosis.5,6Itisthereforegenerallyrecommendedthat
