Andersons pediatric cardiology 1822

GuidingPrinciplesofStaged
Reconstruction
Surgicalandmedicalmanagementinthefirstfewyearsoflifeisundertaken
toachieveasingulargoal:asuccessfulFontanoperationwithmaximum
durabilityandqualityoflife.Sucha“successful”outcomeisachievablein
manyandhasbeenimprovinginrecentdecades.12–15Nonetheless,successful
outcomesforstagedreconstructionlagbehindthesurgicalandmedicaladvances
innearlyeveryotherformofCHD,bothformortalityandmorbidity.15–21
Improvementinthisfundamentalgoalismostlikelytobeachievedby
incrementalandcumulativeadvancementsinmanagementduringstaged
reconstructionandbeyond.ThespecificgoalsoutlinedinBox70.1arediscussed
inmoredetailinotherchaptersofSection6,FunctionallyUniventricularHeart,
aswellaselsewherethroughoutthistext.


Box70.1

StrategiestoImproveOverallOutcome
DuringStagedReconstruction
Minimizethecumulativemortalityriskofsurgicalandcatheter
interventions
Minimizethecumulativemorbidityofperioperativecaretoallorgans,
particularlytheheart,brain,andkidney
Minimizetheriskandfrequencyofunplannedreinterventions
Minimizetheriskofinterstagemortalityandmorbidity
Maximizegrowth,nutrition,neurodevelopment,psychosocialadaptation,
andcardiovascularfitnessduringstagedreconstructionandbeyond
Maximizepatientandfamilyqualityoflife
Improvecollaborationbetweencenters,andinvolvepatientsandfamilies,
toshareandvalidatebestpractices

StrategiesandPhysiologicGoalsto
ObtaintheOptimalOutcomeofStaged
Reconstruction
Ratherthanbeginningthissectionwithareviewofthephysiologicand
managementstrategiesgoverningthecareofnewbornsandinfants,wehave
chosentostartthisreviewwiththephysiologicandsurgicalprinciplesabove
thatcontributetoasuccessfulFontanoperation.Wediscussthetenetsofa
successfulFontalprocedurebeforethediscussionofnewbornmanagement
because–althoughtheneonatalproceduresoccurfirstorathighrisk—itis
necessarytounderstandtherationaleofthehigherriskprocedurestoachievethe
primarygoal.Toachievethisgoal,long-termfollow-updataandourclinical
experiencesuggestthattheoptimalFontanoutcomewillmostconsistentlybe
achievedbyprovidingthehighestpossiblecardiacoutput,atrestandwith
exertion,atthelowestpossiblecentralvenouspressure.Thewell-described
riskfactorsforsuboptimaloutcomesaredescribedinTable70.1andpicturedin
Fig.70.1AandB.12,21–27
Table70.1
RiskFactorsforMortalityandMorbidityFollowingtheFontan
Operation
Systemicvenousobstruction

■Cavopulmonaryanastomoses
■Extracardiacconduitorlateraltunnel
Hypoplasticand/ornarrowedcentral ■Congenital
pulmonaryarteries
■Surgicallyrelated
■Asymmetricflowrelated
Elevatedpulmonaryvascular
■Long-standingincreasedpulmonarybloodflow

resistance
■Long-standingincreasedpulmonaryarterypressure
■Long-standingpulmonaryvenoushypertension
■Highaltitude
■Airwayobstructionwithchronichypoventilation,chroniclungdisease,
ongoinglong-termmechanicalventilation
■Lunghypoplasia
Pulmonaryveinstenosis
■Congenital
■Acquired
Elevatedpulmonaryvenousatrial
■Pulmonaryvenousobstructionoftheatrialoutlet
pressure
■Restrictiveatrialseptaldefect
■Atrioventricularvalvestenosis
■Elevatedventricularend-diastolicpressure(multifactorial,seebelow)


Elevatedend-diastolicpressure

■Hypertrophy
■Persistentobstructionofventricularoutfloworaorticarch
■Long-standingpressureorvolumeload
■Hypertension
■Ventricularscarring
■Atrioventricularvalveregurgitation
■Semilunarvalveregurgitation
■Aortopulmonarycollaterals
■Sinusnodedysfunctionwithjunctionalescaperhythmandcannonwaves
■Tachyarrhythmias

■Ventricularpacingsecondarytoatrioventricularblock