Andersons pediatric cardiology 1917

behindaproteinaceousmaterialcomposedoffibrinandinflammatorycellsthat
ultimatelycoalesceinto“plasticcasts”oftheairway(Fig.73.21).191Thesecasts
causeobstructionoftheairways,leadingtocough,toventilation-perfusion
mismatch,and,inseverecases,toasphyxia(Table73.2).186,191–193
Exacerbationsaremorecommoninthewintermonthsandmaybeprecipitated
byrespiratoryinfections.Theonsetoftenoccurswithinafewyearsofthe
Fontanprocedure,andtheconditionismorecommoninthosewhohad
chylothoraxatthetimeofsurgery.193

FIG.73.21 Expectoratedcastfromapatientwithplasticbronchitisafter
Fontanoperation.(FromAvitabileCM,GoldbergDJ,DoddsK,etal.A
multifacetedapproachtothemanagementofplasticbronchitisafter
cavopulmonarypalliation.AnnThoracSurg.2014;98[2]:634–640.)

Table73.2
LymphaticInsufficiencyintheFontanCirculation
Plasticbronchitis

Etiology
Elevatedintralymphaticpressure
Increasedlymphaticproduction

Protein-losing
enteropathy

Abnormallymphaticconnectionstothe
airways
Elevatedintralymphaticpressure
Increasedlymphaticproduction
Abnormallymphaticconnectionstothe
intestines

Manifestations
Cough
Lowoxygen
saturations
Asphyxia

Treatment
Pulmonaryvasodilation
InhaledtPA

Diarrhea
Ascites
Peripheraledema

Diuretics
Pulmonaryvasodilation
Controlled-release
budesonide
Lymphaticintervention

Musclewasting

Lymphaticintervention


Lymphopenia

tPA,Tissueplasminogenactivator.

Treatmentforplasticbronchitishasevolvedrapidlyoverthepastdecadeas
themechanismofthediseasehasbecomeclear.194Medicalmanagement
strategiesforplasticbronchitisincludetreatmentwithbronchodilators,inhaled
steroids,andpulmonaryvasodilators.191,195Forthosewithchroniccast
production,inhaledtissueplasminogenactivatorcanbeaddedtothemedical
regimentodissolvethefibrinwithinthecasts.Thistreatmentcanbequite
successfulatcontrollingthesymptomsandseverityofthedisease.191,196
However,althoughmedicaltherapiesmaybeeffectiveatcontrollingsymptoms,
theydonotchangetheunderlyingabnormalitiesofthelymphaticvesselsnordo
theyeliminatetheconnectionsbetweenthelymphaticvesselsandtheairways.
Recentadvancesinlymphaticimagingandinterventionhaveallowedfora
moreselectiveapproachtothetreatmentofplasticbronchitis.UsingT2MRI
imagingordyeinjectionintothelymphnodes,thelymphaticsystemcanbe
visualizedandabnormallymphaticnetworkscanbemapped.187Oncemapped,
newinterventionaltechniquesforaccessingthelymphaticsystemcanbeused
forthedeliveryofagentstoembolizethenetworkofabnormallymphatic
vessels,therebyreducingtheleakageoflymphaticfluidintotheairwaysand
eliminatingtheformationofcasts.189,194,197Theearlyexperiencewiththis
techniqueisencouraging.Itsuseisexpanding,andithasthepotentialtobecome
adefinitivetherapyforthosewhodevelopthiscomplication(Fig.73.22).197


FIG.73.22 Dynamiccontrast-enhancedmagneticresonance
lymphangiogram(A)andlymphangiogram(B)imagesofthecentral
lymphaticsystemofapatientwithplasticbronchitis.Bothfigures
demonstrateadilatedandtortuousthoracicduct(arrow),pulmonary
lymphaticvesselswithretrogradeflow(arrowheads)andareasof
pulmonarylymphaticperfusion(box).(FromDoriY,KellerMS,RomeJJ,et
al.Percutaneouslymphaticembolizationofabnormalpulmonarylymphatic
flowastreatmentofplasticbronchitisinpatientswithcongenitalheart

disease.Circulation.2016;133[12]:1160–1170.)

Protein-LosingEnteropathy
Likeplasticbronchitis,PLEisaconsequenceoflymphaticinsufficiency.Inthe
caseofPLE,theabnormallymphaticconnectionsformbetweenthelymphatics
originatingintheliverandthesmallintestine.188Theseabnormalconnections
allowprotein-richlymphaticfluidtodrainfromthehigh-pressurelymphatic
systemintothelow-pressuregastrointestinaltract.Overtime,thisresultsina
profoundlossofproteinsandaphenotypecharacterizedbydiarrhea,extreme
musclewasting,particularlyoftheextremities,andoftenacombinationof
peripheraledemaandchronicascites.186,198Although5-yearsurvivalafterPLE
diagnosishasimproveddramaticallyoverthepast2decades(from50%to
88%),thediseaseremainsseverelydebilitating,withasignificantimpacton
qualityanddurationoflife.199,200Inadditiontothephenotypicabnormalities,
patientswithPLEhaveabnormalitiesinbonestructureandinthecharacteristics
oftheimmunesystem.148,201ThediseasespresentsomeyearsaftertheFontan
operation.Althoughthereisanassociationwithcomplexheartdisease,including