Andersons pediatric cardiology 1191
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FIG.44.28 Surgicalapproachesforpatientswithdiscretesubaortic
stenosis(subAS)basedonsizeandfunctionoftheaorticvalve(AoV).
LVOTO,Leftventricularoutflowtractobstruction.
Inthemajorityofcases,circumferentialexcisionofthefibrousridgewith
septalmyectomyprovidesefficientreliefofobstruction(Video44.18).A
modifiedKonnooperation,whichinvolvesmyectomy,resectionoffibrous
subaortictissue,andcreationofaventricularseptaldefectthatispatchclosed,
provideseffectiverelieveofsubaorticobstructioniftheaorticannulusandvalve
arenormallydeveloped.TheRoss-Konnooperationistheprocedureofchoicein
childrenwithamultileveltypeofleftventricularoutflowtractobstructionand
severeabnormalityoftheaorticvalve(Video44.19).
Long-TermOutcomes
Earlysurgicalmortalityfollowingrepairofdiscretesubaorticmembraneislow,
lessthan5%,withthesurvivalrategreaterthan90%at15years.184However,
patientsareatriskforbothrecurrenceofsubaorticstenosisandprogressive
aorticregurgitation.Needforreoperationforrecurrenceofsubaorticstenosis
afterresectionofadiscretesubaorticmembranerangesfrom7.1%to
20%.182,185,186Theriskforrecurrenceislesswhenmembraneresectionis
accompaniedbymyectomycomparedwithmembraneresectionalone.187
Aggressiveresectionofallstructurescausingflowturbulence(anomalousseptal
insertionofthemitralvalve,accessorymitralvalvetissue,anomalouspapillary
muscle,anomalousmuscularband,etc.)andremovalofpathologictissuefrom
thevalveleafletsandfromthesubcommissuraltrigonesmaypreventthe
developmentorprogressionofaorticregurgitationandmayeliminatethe
substrateforrecurrentobstruction.188Aorticregurgitationmayprogresseven
aftersuccessfulrepairofthesubaorticstenosis.182Postoperativecompleteheart
blockisacomplicationwhenthereistooaggressiveanapproachduring
extended,circumferentialmyectomy.
AnnotatedReferences
AndersonRH.Demystifyingtheanatomic
arrangementoftheaorticvalve.EurJ
CardiothoracSurg.2006;29(6):1006–1007.
Apublicationshowingthattheleafletsoftheaortic
valvearesuspendedinasemilunarfashion
withintheaorticroot,makingitdifficultto
describeanypartoftherootconsistentlyin
termsofanannulus..
PrakashSK,BosseY,MuehlschlegelJD,etal.A
roadmaptoinvestigatethegeneticbasisof
bicuspidaorticvalveanditscomplications:
insightsfromtheinternationalBAVCon
(bicuspidaorticvalveconsortium).JAmColl
Cardiol.2014;64(8):832–839.
Areviewarticlethatdiscussesthecomplex
inheritancepatternsandthecurrent
understandingofthegeneticetiologyofbicuspid
aorticvalvedisease..
NishimuraRA,OttoCM,BonowRO,etal.2014
AHA/ACCguidelineforthemanagementof
patientswithvalvularheartdisease:areportof
theAmericanCollegeofCardiology/American
heartassociationtaskforceonpractice
guidelines.JAmCollCardiol.2014;63(22):e57–
