Andersons pediatric cardiology 1145
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PulmonaryAtresiaWithIntact
VentricularSeptum
MichaelA.Quail,RobertH.Anderson,DianeE.Spicer,PiersE.F.Daubeney
Abstract
Therightventriclecanbehypoplasticinvarioussettings.Itcanbesmallin
thepresenceofdeficientventricularoratrioventricularseptation,producing
so-calledleftventriculardominance.Thechambercanalsobesmalland
incompleteinthesettingofuniventricularatrioventricularconnectionssuch
asdouble-inletleftventricleortricuspidatresia.Theventriclecanbe
hypoplasticwhentheventricularseptumisintactandthereiscritical
stenosisofthepulmonaryvalve.Alloftheseentitiesaredealtwith
elsewhereinthisbook.Thischapterisconcernedwithpulmonaryatresiain
thesettingofanintactventricularseptum(seeFig.43.1)andincludesthe
situationinwhichthecavityoftherightventricleisdilated,aswellas
hypoplastic.IntwoofthefinalencyclopedicreviewsledbyFreedom,
attentionwasdirectedtothehugeproblemsproducedbylesionsofthe
coronaryarteriesortricuspidvalveinthissetting,makingitoneofthemost
lethalofcurrentcongenitalcardiacmalformations.
Keywords
Pulmonaryatresia
Therightventricle(RV)canbehypoplasticinvarioussettings.Itcanbesmallin
thepresenceofdeficientventricularoratrioventricularseptation,producingsocalledleftventriculardominance.Thechambercanalsobesmallandincomplete
inthesettingofuniventricularatrioventricularconnectionssuchasdouble-inlet
leftventricle(LV)ortricuspidatresia.Theventriclecanbehypoplasticwhenthe
ventricularseptumisintactandthereiscriticalstenosisofthepulmonaryvalve.
Alloftheseentitiesaredealtwithelsewhereinthisbook.Thischapteris
concernedwithpulmonaryatresiainthesettingofanintactventricularseptum
(Fig.43.1)andincludesthesituationinwhichthecavityoftheRVisdilated,as
wellashypoplastic.IntwoofthefinalencyclopedicreviewsledbyFreedom,1,2
attentionwasdirectedtothehugeproblemsproducedbylesionsofthecoronary
arteriesortricuspidvalveinthissetting,makingitoneofthemostlethalof
currentcongenitalcardiacmalformations.Itwasnotwithoutreasonthat
Freedom3hadpreviouslycommented,inoneofhismanyperceptivereviewsof
thislesion,“Howcansomethingsosmallcausesomuchgrief?”
