Andersons pediatric cardiology 1818
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thatdevelopmenthadbeennormalatleastuntiltheeighthweekofgestation.
Theselesions,therefore,reflectacquireddiseaseduringfetallife.Ithasstillto
bedeterminedwhytheheartshoulddevelopwitheitherhypoplasiaoftheleft
ventricleoranimperforatepulmonaryvalve.Asyet,despitemultipleefforts,
therehasbeenfailuretoidentifyanyspecificgenesthatmightbeinvolvedin
producingthevariouslesions.
AnnotatedReferences
AndersonRH,BeckerAE,TynanM,etal.The
univentricularatrioventricularconnection:
gettingtotherootofathornyproblem.AmJ
Cardiol.1984;54:822–882.
Inthisreview,themembersoftheEuropeanschool
ofnomenclature,supportedbythelateRobert
Freedom,recognizedthewisdomofthe
“morphologicmethod.”Theypointedoutthat,
inheartswith“singleventricles”or
“univentricularhearts”itwasveryrareforthe
ventricularmasstocontainbutonechamber.In
reality,itwastheatrioventricularconnections
thatwereuniventricularinthesesettings.Since
then,theEuropeanschoolhasbasedits
definitionsexclusivelyonthemorphologic
method,asexplainedintheintroductorysection
ofthischapter..
JacobsML,AndersonRH.Nomenclatureofthe
functionallyuniventricularheart.CardiolYoung.
2006;16(suppl1):3–8.
Thisreviewshowedhow,bytheadditionofa
simpleadverb,namelyfunctionally,itwas
possibletodefuseallthemultipleargumentsthat
continuedtosurround“univentricular”hearts.
Almostallheartsdescribedinthisfashionhave
onebigandonesmallventricle.Thekeypointis
thatonlythebigventricleiscapableof
supportingoneorotherofthecirculationsor,in
mostinstancesbothcirculations.Hencethis
arrangement,thoughnotanatomically
univentricular,iscertainlyfunctionally
univentricular..
KiralyL,HubayM,CookAC,HoSY,Anderson
RH.Morphologicfeaturesoftheuniatrialbut
biventricularatrioventricularconnection.J
ThoracCardiovascSurg.2007;133:229–234.
Areviewofaseriesofheartshavingabsenceof
oneatrioventricularconnectionbutwith
straddlingandoverridingofthesolitary
atrioventricularvalve.Itisexplainedthatthis
producesauniatrialbutbiventricular
connection.Suchheartscanexistwithabsence
ofeithertherightorleftatrioventricular
connectionandwithright-orleft-side
ventriculartopology.Thislesionisonevariant
ofthe“double-outletatrium.”.
TchervenkovCI,JacobsJP,WeinbergPM,etal.
Thenomenclature,definitionandclassification
ofhypoplasticleftheartsyndrome.Cardiol
Young.2006;16:339–368.
Thisextensivereviewwaspreparedbythe
