Andersons pediatric cardiology 1918

hypoplasticleftheartsyndrome,andwithpostoperativechylothorax,many
affectedindividualshavenoobviousriskfactors.192Theonsetisofteninsidious,
butoncePLEisestablished,patientswiththisconditionareamongthemost
chronicallydebilitatedofallthosewithcongenitalheartdisease.
Althoughitiswellunderstoodthatabnormallymphaticconnectionstothe
intestineareresponsibleforthelossoflymphaticfluidthatoccurswithPLE,
therearelikelyothercontributingfactorstothisdisease.Endoscopyoften
demonstratesaninflammatorypatternsimilartothatobservedinchildrenwith
inflammatoryboweldisease.Likeinflammatoryboweldisease,therearecases
ofPLEthatcanbecontrolledthroughtheuseofcorticosteroids.202Oral
controlled-releasebudesonideisasteroidformulatedtotargetits
antiinflammatorypropertiestothedistalsmallintestine.Budesonidehasthe
addedadvantageoffirstpasshepaticmetabolism,theoreticallyleadingtoa
lowerdegreeofsystemicabsorptionthanwithothercorticosteroids.
Unfortunately,somecasesofPLEaresteroidresistant.Inolderadolescents
andyoungadults,controlledreleasebudesonidehasnotbeenaseffectivea
treatment.203Althoughtheresponsetosteroidsinyoungerchildrensuggestsan
inflammatorycomponentofthedisease,theabsenceofaresponseinadolescents
andyoungadultssuggeststhatinflammationmayplaylessofaroleinolder
patients.Althoughtheunderlyingabnormalconnectionsbetweenthelymphatic
vesselsandtheintestinearepresentinbothagegroups,thedifferentialresponse
totreatmentsuggeststhatthebreakdownoftheintegrityoftheintestinalmucosa
mayhavemoreofarelationshiptoinflammationinyoungerpatients.
Inanyagegroup,thetreatmentstrategyforPLEbeginswithoptimizingthe
overallFontanphysiology.Diureticsmaybehelpfulbydecompressingthe
lymphaticsystemandmayalsotreatsomeofthesymptomsassociatedwith
PLE.186,198Pulmonaryvasodilatorsmaybeusefuladjunctsbecausethe
reductioninpulmonaryvascularresistanceleadstoadropinCVPandtherefore
areductioninintralymphaticpressure.Otherpotentialtherapieshavebeen
reportedandincludetheadministrationofheparinandeventreatmentwithlowdosedopamineinfusion.204,205Unfortunately,althoughmedicaltreatmenthas
ledtoimprovedoutcomes,theburdenofmorbidityandmortalityremains

substantial.
Asinthecaseofplasticbronchitis,theemergenceoflymphaticimagingand
interventionhasthepotentialtoalterthestrategyforthetreatmentofthosewith
PLE.187,188Byaccessingandoccludingtheabnormallymphaticconnections
fromthehepaticlymphaticstotheintestines,onecouldsubstantiallyalterthe


rateoflossoflymphaticfluid.However,althoughpromising,lymphatic
interventionforPLEisnotassuccessfulasithasbeenforplasticbronchitis.
Nevertheless,recentreportsoflymphaticinterventionsuggestthatwithfurther
technicalrefinement,thereisthepotentialtofundamentallyalterthetrajectory
ofthosewhodevelopthisfearedcomplication.

PeripheralVascularDisease
Increasedarterialstiffnessandsmallarteriallumendiameterhavebeenreported
inFontanpatients,thelatterpresumablyaresponsetoachronicreductionin
cardiacoutput.53,206–208Thesearterialabnormalities,incombinationwiththose
oflymphaticdrainagedescribedearlierandchronicvenousinsufficiency,
manifestinthelowerextremitiesasvaricoseveins,edema,and,inseriouscases,
chroniculceration.Theselowerextremityproblemsoccurinapproximately20%
ofFontanpatientsolderthan18yearsandaremorecommoninthosewhohave
hadmultiplecardiaccatheterizationproceduresorcentralvenouscathetersvia
thefemoralveins.Theyarealsoassociatedwithdeepvenousthrombosisandas
suchmaypredisposetopulmonaryandsystemicembolism.209


NeurocognitiveandPsychosocial
Function
Thediagnosisofalife-threateningillnessduringachild'sformativeyearscan
havefar-reachingeffectsthatripplethroughthefamilyandacrossalifetime.

ChildrenfollowingtheFontanpathwayexperienceprofoundphysical,
emotional,behavioral,neurodevelopmental,andsocialchallengesintheearly
yearsoflife,andthesechallengeshavethepotentialforlifelongconsequences,
particularlyinrelationtofuturehealth,well-being,andQOL.

QualityofLife
TheWorldHealthOrganizationdefinesqualityoflifeasadynamic,
multidimensionalconcept,uniquetoeachindividual'sperceptionofhisorher
positioninlifeandhisorherphysicalhealth,psychologicalwell-being,levelof
independence,relationships,personalbeliefsandvalues,andenvironmental
context.210Inthecontextoffunctionallyuniventricularcongenitalheartdisease,
stressorsassociatedwithdiagnosisandtreatment,theuncertaintyofprogressive
functionallimitations,andthepossibilityofheartfailure,hearttransplantation,
arrhythmias,andsuddendeathmaycompromiseQOL.Overall,themajorityof
publishedstudiesreportlowerQOLforchildrenandadultswithaFontan
circulationcomparedwithnormativedata,age-matchedcontrols,orhealthy
siblings211,212;however,asmallnumberofstudiesreportQOLsimilartothe
generalpopulation.213,214Althoughsomestudieshavedemonstratedan
associationbetweenlowerQOLandgreatercomplexityofcardiacabnormality,
dailymedicationuse,greaterlengthofhospitalstay,andgreaternumberof
medicalinterventions,215,216mostresearchhasfoundthatsocialand
psychologicalconstructs,suchasgreaterpsychologicalstress,fewersocial
supports,andlowerfamilysocioeconomicstatus,playamoreinfluentialrolein
determiningQOL.215–219Consensusontheroleoftheseriskfactorsinthe
Fontanpopulationhasnotbeenestablished.

NeurocognitiveOutcomes
Fromaneurocognitiveperspective,itiswellestablishedthatchildrenwith