Andersons pediatric cardiology 1923

Progesteroneimplant

Notthrombogenic
Longlasting(3years)
Highintrinsicefficacy

Progesterone
intramuscular
injection

Notthrombogenic
Long-lasting
contraception(12wk)
Highintrinsicefficacy
Notthrombogenic
Nonhormonal
Additionalprotection
againstsexually
transmittedinfections
Notthrombogenic
Long-lasting
contraception(5years)

Condoms/barrier
methods

Intrauterinedevice

compliance
Reducedefficacywith
Bosentan

Sideeffects,especially
menstrualirregularity
Reducedefficacywith
Bosentan
Smallriskofhematoma
withwarfarin
Dependentonstrict12
weeklycompliance
Userdependency
Generallowerefficacy

Recommended

Recommended

Recommendedideallyin
conjunctionwithother
method

Potentialforseverevagal Secondline
reactionduringinsertion recommendation
Coppercoilshigherrisk
ofmenorrhagiaand
dysmenorrhea
Infectionrisk,especially
duringinsertion


CardiacCatheterizationand
TranscatheterIntervention

Inthelong-termfollow-upoftheFontanpatient,cardiaccatheterizationmaybe
indicatedtoclosebaffleorconduitfenestrations.Otherspecificindications
includetheinvestigationand/ortreatmentof

•unexpectedsystemicarterialhypoxemia,
•excessiveaortopulmonarycollateralvesselsor
persistentventricletopulmonaryconnectioncausing
pulmonaryovercirculationandventricularvolume
load,
•thefailingFontancirculation,includingthose
presentingwithPLEorplasticbronchitis.
Outsidetheseindications,theutilityofroutinecardiaccatheterizationisnot
wellestablished,althoughsomeconsiderthatknowledgeoftime-relatedtrends
intheCVP,systemicventriclefillingpressure,andsystolicfunctionis
advantageous.Thereisastrongerargumentforintermittentimagingofthe
Fontanpathwayandpulmonaryarteries,especiallyinthosewithanextracardiac
conduit.WiththistypeofFontancirculation,theconduitportionofthepathway
hasafixedlengthandsomaticgrowthmaystretchthepulmonaryarteriestoward
thediaphragm,resultinginstenosis.Infact,thisisarareoccurrencebecausein
mostinstancesIVCelongationaccommodatessomaticgrowth.CardiacMRIcan
usuallybeusedtoobtainthisinformationand,althoughitdoesnotprovidea
directmeasureofFontanpathwaypressureorsystemicventriclefillingpressure,
hastheadvantageofprovidingadditionalinformationincludingquantificationof
systemicfunction,assessmentofproportionalpulmonarybloodflow,and
quantificationoftheregurgitantfractionwhenvalveregurgitationispresent.
CardiaccatheterizationorCTangiographycanbeusedifMRIiscontraindicated
orunavailable.


FenestrationClosure

Manypatientshaveafenestrationcreatedbetweenthesystemicvenouspathway
andthepulmonaryvenousatriumatthetimeoftheFontanproceduretoreduce
operativeandpostoperativerisk(seeChapter71).Fenestrationsclose
spontaneouslyin25%to40%ofcases.347Thosethatpersistcanbeclosedwitha
relativelystraightforwardtranscatheterintervention,butthebenefitsofclosure
areunclear,andtimingandindicationsforclosurearecontroversial(Videos73.3
and73.4).
Thefenestrationallowssystemicvenousbloodtobypassthepulmonary
circulation.Asaresult,CVPtendstobelowerandcardiacoutputhigherthan
wouldotherwisebethecase.GiventhatelevatedCVPandlowcardiacoutput
areassociatedwithmanyofthelong-termcomplicationsoftheFontan
circulation,apersistentfenestrationmaybeadvantageousinthelongterm.
However,theobligatoryright-to-leftshuntresultsinsystemicarterial
hypoxemia.Thismaylimitexercisecapacityandcontributetomyocardial
fibrosisandneurologiccomplications.Inonestudy,anaverageof30%of
systemicventriclecardiacoutputwasprovidedbythefenestrationwhen
measuredbycardiacMR,andinnearlytwo-thirdsofpatients,alloftheinferior
venacavaflowpassedthroughthefenestration,bypassingthelungs.348Asone
wouldexpect,theamountoffenestrationflow(andthedegreeofcyanosis)
correlatedwithelevatedpulmonaryvascularresistance.Interestinglyitalso
correlatedwithdiastolicfunction—thebetterthediastolicrecoil,themoreflow
therewasacrossthefenestration.Thesefindingsimplythattheamountofflow
acrosstheFontanfenestrationandthedegreeofcyanosisarepoormarkersofthe
statusoftheFontancirculation.
Anumberofstudieshavecomparedthehealthofpatientswithapersistent
fenestrationtothosewithout.Alargemulticenterstudydemonstratedno
differenceinoutcomemeasuresincludinggrowth,functionalhealth,exercise
performance,andb-typenatriureticpeptide.137Likewiseasinglecenterstudy,in
whichlessthanathirdofpatientshadfenestrationclosureatthetimeofreview,
foundnodifferenceinoutcome.349Conversely,inapopulationwithahighrate

ofclosure,apatentfenestrationwasassociatedwithahigherriskofdeath(Fig.
73.24).350Inthatstudy,onlyasmallminorityofpatientshadapersistent
fenestrationandtheyeitherdemonstratedunfavorablehemodynamicchanges
withtestocclusion(seelater)orwerenotreferredforclosurebecauseofrisk
factorsidentifiedaftertheFontanoperation.Perhapsmostimportantlythepre-