Andersons pediatric cardiology 1158
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Theelectrocardiogrammayshowthepresenceofarrhythmias,atrialor
ventricular.ThereisoftenPpulmonaleduetorightatrialdilatation.TheQRS
axisusuallyshowsleftventriculardominance.
Echocardiographyshouldbeusedtodocumentsystematicallyandsequentially
allthemorphologicfeaturespreviouslydiscussed.Residuallesionssuchasatrial
septaldefectsandpatentsystemicshuntsshouldbesought.Forthosewitha
biventricularcirculation,thepresenceanddegreeoftricuspidregurgitation,
pulmonaryregurgitation,andstenosisshouldbedocumented.Thesizeandzscoreofthetricuspidvalveshouldbedocumented.ForthosewithaFontan
circulation,carefulassessmentoftheanastomosesisneededaswellas
assessmentofleftventricularfunction,mitralregurgitation,andthepresenceof
rightatrialthrombus.Thereshouldbelow-velocityphasicflowintheinferior
andsuperiorvenouspathwaysoftheFontan.Imagingofthecoronaryartery
originsandtheirsizemaysuggestsignificantfistulouscommunications.
Cardiaccatheterizationmayberequiredtoassessthehemodynamicsofthe
Fontancircuit.Coronaryarteriographyisessentialbecausestenosesand
interruptionsmayplayasignificantpartintheprognosis.Assessmentofthe
pulmonaryarterialanatomyisimportantbecausethepatientmayhavehada
systemic-to-pulmonaryarterialshuntinthepast,withpulmonaryarterial
distortion.IncontrasttopulmonaryatresiawithtetralogyofFallot,native
stenosesorhypoplasiaofthepulmonaryarteriesarerelativelyuncommon.
Baffleleaksandvenovenouscollateralizationshouldbesoughtinthosewitha
Fontancirculation.
Cardiacmagneticimagingandcomputertomographymaysupplant
catheterizationasameansofnoninvasiveassessmentoftherangeof
morphologiclesionsfoundinthiscondition.Theygiveexcellentanatomicand,
inthecaseofmagneticimaging,excellentfunctionalinformation.
Nuclearimagingconveysimportantinformationaboutmyocardialperfusion
andischemia,particularlyforthosepatientswithsignificantcoronaryartery
lesions,althoughabnormalanatomyimposestechnicalchallenges.
ImplicationsinAdultLife
Physiologic
Manypatientswiththisconditionarereachingadulthood.Theirwell-beingin
adultlifedependsonthecumulativeeffectoftheconsequencesoftheabnormal
physiologyandproceduresthroughouttheirlife(betheycardiacand
noncardiac),reasonablecardiacfunction,andavoidanceofresiduallesions.66
Forthosewithbiventricularorone-and-one-halfventriclerepairs,residual
lesionscomprisepulmonarystenosis,regurgitation,andtricuspidregurgitation.
Thesecanleadtoatrialandventriculararrhythmias,syncope,suddendeath,
reducedexercisetolerance,fatigability,andangina.Increasingly,patientswith
significantpulmonaryregurgitationanddilationoftheRVarebeingmanaged
withpulmonaryvalvereplacement,eithersurgicallyorinthecatheterization
laboratory.Forthosewithafunctionallyuniventricularrepair,suchasatotal
cavopulmonaryconnectionorFontan-typecirculation,residuallesionscomprise
rightatrialdilation,coronaryarterialstenoses,highsystemicvenouspressures,
andsystemic-to-pulmonaryarterialandvenouscollateralization.Thesecanlead
toatrialandventriculararrhythmias,suddendeath,thromboembolism,right
pulmonaryvenousocclusion,angina,leftventriculardysfunction,protein-losing
enteropathy,hepaticdysfunction,andcyanosis.Forthosewithincompletely
separatedcirculations,thereiscommonmixingofsystemicandpulmonary
circulationsleadingtocyanosis,erythrocytosis,thromboembolism,fatigability,
andarrhythmias(seeChapter73).
ContraceptionandPrepregnancy
Prepregnancycounselingisessential.Adviceshouldbetailoredtotheindividual
withconsiderationofcardiacstructureandfunction.Patientswithongoing
hypoxemiaoraFontan-typecirculationshouldavoidthecombined
contraceptivepill.Detailedadviceisbeyondthescopeofthischapterbutis
thoroughlycoveredelsewhere(seeChapter80).130
BiventricularRepairWithResidualPulmonary
StenosisorRegurgitation
Theincreasedhemodynamicloadofpregnancymayprecipitaterightheart
failure,atrialarrhythmias,ortricuspidregurgitation.Balloondilationcanbe
performedduringpregnancy,preferablyafterorganogenesis,althoughitisbetter
totreatbeforeconception.
FunctionallyUniventricularCirculation
Thereareincreasedrisksofsystemicvenouscongestion,deteriorationin
ventricularfunction,atrialarrhythmias,thromboembolism,andparadoxic
embolizationifthereisafenestrationintheatrialbaffle.Successfulpregnancyis
possible,however,withmeticulouscardiacandobstetricplanningand
supervision.Ifanticoagulationisrequired,additionalriskstothefetusare
involved.
MixedCirculationsWithCyanosis
Thereisanincreasedriskofmaternalcardiovascularcomplications,prematurity,
andfetaldeath,particularlywhenbaselinematernalrestingoxygensaturations
arelessthan85%.
Allpatientsshouldhavecardiologiccounsellingpriortoconception,and
follow-upbyanadultcongenitalcardiologistandahigh-riskobstetricianduring
pregnancyandtheperipartum.130,131Fetalechocardiographyisrecommended.
Employment
Employmentwilldependontheoriginaldiagnosisandresiduallesions.There
maynotbespecificstratificationofriskforthisparticularlesion,andparallels
fromotherlesions,suchaspulmonarystenosis,tetralogyofFallot,andpatients
withFontan-typecirculations,mayhavetobetaken.Employabilitywilldepend
onexerciseability,avoidanceofcomplications,andongoingparticularly
neurologicmorbidity.Employmentwouldneedtobetailoredtoindividual
needs.Detaileddiscussionofthesetopicsisbeyondthescopeofthischapter,
althoughtheyarethoroughlycoveredelsewhere.130
