Andersons pediatric cardiology 1934

significantincreaseinexercisecapacityinthose
treatedwithBosentanfor14weeks.Therewere
noimportantsideeffects.Theaccompanying
editorialhighlightsthedifficultyindesigning
trialsforthispopulation,wheredeteriorationis
slowandinsidiousandhardendpointsrelatively
infrequent..
GoldbergDJ,SurreyLF,GlatzAC,etal.Hepatic
fibrosisisuniversalfollowingFontanoperation,
andseverityisassociatedwithtimefrom
surgery:aliverbiopsyandhemodynamicstudy.
JAmHeartAssoc.2017;6(5).
Liverbiopsyandcardiaccatheterizationwere
offeredasroutinescreeninginFontanpatients.
Atameanageof17yearsand15yearsfromthe
Fontanoperation,allhadhepaticfibrosis.The
degreeoffibrosisdidnotcorrelatewithserum
liverfunctiontestsorhemodynamicfindingsor
ventricularmorphologybutincreasedwithtime
fromtheFontanoperation..
HilscherMB,JohnsonJN,CettaF,etal.
Surveillanceforlivercomplicationsafterthe
Fontanprocedure.CongenitHeartDis.
2017;12(2):124–132.
Thisexcellentreviewsummarizesthecurrent
knowledgebaseandprovidessensibleguidance
forthesurveillanceofliverabnormalities,


particularlyhepatocellularcarcinoma..
JohnAS,JohnsonJA,KhanM,etal.Clinical

outcomesandimprovedsurvivalinpatientswith
protein-losingenteropathyaftertheFontan
operation.JAmCollCardiol.2014;64(1):54–62.
Protein-losingenteropathyisassociatedwitha
significantmortalityrisk,previouslyreportedto
beapproximately50%within5yearsof
developmentofthedisease.Inthisreportthe
riskoffailurewassubstantiallylower,with88%
surviving5yearsfromdiagnosis—adifference
theauthorsattributetoimprovementsin
treatment.Worsefunctionalstatus,ventricular
dysfunction,lowercardiacoutput,elevated
pulmonaryarterypressure,andpulmonary
vascularresistancewereassociatedwithapoor
outcome..
CunninghamJW,NathanAS,RhodesJ,etal.
Declineinpeakoxygenconsumptionovertime
predictsdeathortransplantationinadultswitha
Fontancirculation.AmHeartJ.2017;189:184–
192.
Serialexercisetestinginagroupofadultpatients
withaFontancirculationdemonstrateda
declineinpeakoxygenconsumptioninthose
whosubsequentlydiedorunderwentcardiac
transplantation.NYHAfunctionalclasswasnot


predictiveofoutcome,highlightingthe
usefulnessofobjectiveindicesoffunctional
capacity..

GoutonM,NizardJ,PatelM,etal.Maternaland
fetaloutcomesofpregnancywithFontan
circulation:amulticentricobservationalstudy.
IntJCardiol.2015;187:84–89.
Therehasbeenageneralreluctancetorecommend
pregnancyinwomenwithaFontancirculation,
becauseofmaternalandfetal/neonatalrisk.In
thismulticenterretrospectivereview,cardiac
eventsoccurredin10%ofwomen.Therewasno
maternalmortality.Therewasahighincidence
ofmiscarriage,prematurity,andintrauterine
growthretardation..
BookWM,GerardinJ,SarafA,MarieValenteA,
RodriguezF3rd.ClinicalphenotypesofFontan
failure:implicationsformanagement.Congenit
HeartDis.2016;11(4):296–308.
ThisarticlereviewsthevarioustypesofFontan
failureandprovidesausefulconstructfortheir
identificationandfortargetedtreatment..