Andersons pediatric cardiology 51
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Terminology
RobertH.Anderson,DianeE.Spicer
Abstract
Nomenclaturerelativetocongenitalcardiacdiseaseisfarlesscontentious
nowthaninthepreviousmillennium.Itwouldbeabraveperson,
nonetheless,whobelievedthatthefieldofdescriptionandcategorization
wasnowfullyresolved.Inthisregard,thereisnorightorwrongwayof
describinghearts,simplydifferentways.Ongoingdifferencesshouldnow
beresolvedsimplybydescribingtheabnormalanatomyasitisobserved.
Theneedforastandardizedapproachreflectsthefactthatthenumberof
individuallesionsthatcancoexistwithinmalformedheartsisconsiderable.
Addtothisthepossibilitiesforcombinationsoflesions,andtheproblemof
providing“pigeonholes”foreachentitybecomesimmense.Simplicityis
providedbyrecognizingthatthehearthasthreebasicbuildingblocks,
namelytheatriums,ventricularmass,andarterialtrunks.Thesystemused
inthisbookconcentratesonthevariationspossibleacrossthejunctions
betweenthesebuildingblocks.Suchanalysiscannotbeginwithoutprecise
knowledgeofthesegmentalanatomyandtopology.Thereforethesystem
startswithestablishingthefeaturesofthemorphologyofthecardiac
components,continuingwithdeterminingthewaytheyarejoinedornot
joinedtogether,andtakingnoteoftherelationsbetweenthem,treating
thesefeaturesasthreedifferentfacetsofthecardiacmake-up.Implicitin
thesystemistheabilitytodistinguishthemorphologyoftheindividual
atriumsandventriclesandtorecognizethetypesofarterialtrunktaking
originfromtheventricles.Thisisachievedusingtheconceptofthesocalledmorphologicmethod,whichstatesthatstructuresshouldbe
recognizedintermsoftheirownintrinsicmorphologyandthatonepartof
theheart,whichisitselfvariable,shouldnotbedefinedonthebasisof
anothervariablestructure.Buildingontheseapproaches,thechaptershows
howitispossiblesimplytoidentifyanddescribeallabnormal
combinations,eventhoughthespecificcombinationinquestionmaynever
previouslyhavebeenencountered.
Keywords
Sequentialsegmentalanalysis;Nomenclature;Concordantconnections;
Discordantconnections;Relations;Infundibularmorphology
Introduction
Itmightreasonablybethoughtthatthosewhodiagnoseandtreatpatientswith
congenitallymalformedheartswould,bynow,havereachedconsensus
concerningthemostappropriatewayofdescribingthemalformationswith
whichtheyareconfronted.Itiscertainlythecasethatnomenclatureisfarless
contentiousnowthaninthepreviousmillennium.Itwouldbeabraveperson,
nonetheless,whostatedthatthefieldofdescriptionandcategorizationwasnow
fullyresolved.Itisnotourintention,inthischapter,toextendthesepolemics.
Rather,wedescribeourownsystemfordescription,leavingthereaderstodecide
whetherthisissatisfactoryfortheirneeds.Byandlarge,thereisnorightor
wrongwayofdescribinghearts,simplydifferentways.1,2Eventhesedifferent
wayshavebeenmitigatedtoconsiderableextentbythecross-mappingof
existingsystems.3Theongoingdifferencesshouldnowberesolvedsimplyby
describingtheabnormalanatomyasitisobserved.
Theneedforastandardizedapproachreflectsthefactthatthenumberof
individuallesionsthatcancoexistwithinmalformedheartsisconsiderable.Add
tothisthepossibilitiesforcombinationsoflesions,andtheproblemofproviding
“pigeonholes”foreachentitybecomesimmense.Straightforwardlesions,such
asseptaldeficienciesorvalvarstenoses,aretypicallyencounteredinheartsthat
areotherwisestructurallynormal.Itiswhentheheartscontainingthelesionsare
themselvesbuiltingrosslyabnormalfashionthatdifficultiesareproduced.If
theseallegedcomplexlesionsareapproachedinasimpleandstraightforward
fashion,noneneedbedifficulttounderstandanddescribe.
Thesimplicityisprovidedbyrecognizingthatthehearthasthreebasic
buildingblocks,namelytheatriums,ventricularmass,andarterialtrunks(Fig.
1.1).Thesystemfordescriptionandcategorizationbasedonrecognitionofthe
limitedpotentialforvariationineachofthesecardiacsegmentswasdeveloped
independentlyinthe1960sbytwogroups:onebasedintheUnitedStatesand
ledbyRichardVanPraagh,1andtheotherfromMexicoCity,headedbyMaria
VictoriadelaCruz.4Bothofthesesystemsconcentratedonthedifferent
topologicarrangementsofthecomponentswithineachcardiacsegment.When
VanPraaghandcolleagues5,6introducedtheconceptofconcordanceand
discordancebetweenatriumsandventricles,theywereconcernedprimarilywith
theharmonyordisharmonytobefoundbetweenthetopologicarrangementsof
