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Andersons pediatric cardiology 767

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Incidence
Interatrialcommunicationsaccountforupto10%ofallcongenitalcardiac
anomalies.Probepatencyoftheovalforamen,furthermore,isremarkably
frequent,beingfoundinupto33%ofheartsexaminedatautopsyirrespectiveof
age.1Thesuggestionthatprobepatencymayunderscoresomecasesofmigraine
broughtthisnormalfindingtoclinicalattention,2althoughthesuggested
associationhasyettobeproven.


Etiology
Therearenoknownintrauterineeventsthatpredisposetodeficientatrial
septation.Thusmostcasesoccursporadically,withnofamilyhistoryof
congenitalcardiacdisease.Nonetheless,thereisasignificantfamilialincidence
whenthelesionisassociatedwithcertainskeletalabnormalitiesoftheforearm
andhand,calledHolt-Oramsyndrome.Thishasnowbeenshowntobedueto
mutationsoftheTbx5gene,amemberoftheBrachyuryfamilyofgenes.3When
observedinfamilies,atrialseptaldefectsarealsoassociatedwithprolongationof
thePRinterval.4


MorphologyandClassification
NormalAtrialSeptum
Anunderstandingofthemorphologyandclassificationofinteratrial
communicationsdependsonknowledgeoftheextentofthenormalatrial
septum.5This,inturn,requiresanappreciationofthedifferencebetween
partitionsthatseparatetheatrialcavities,whichcanberemovedwithout
transgressingonthepericardialcavity,asopposedtotheremovaloffolds,which
doproduceacommunicationwiththeextracavitaryspace(Fig.29.1).6

FIG.29.1 (A)Arrangementofthepartitionsbetweentheatrialchambers
asseeninfrontalprojection.(B)Howtheflooroftheovalfossacanbe


removedwithouttransgressingonextracardiacspace.(C)Removalofthe
cranialrimofthefossa,however,createsacommunicationwiththe
pericardialcavity,sincetherimisaninfoldingbetweentheatrialwalls.We



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