Andersons pediatric cardiology 789
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FIG.30.7 Heartexhibitingpostnatalpersistenceofthevalvesofthe
embryonicvenoussinus.UnliketheheartshowninFig.30.6,thevalves
havenotbecomefenestrated,sotheyproduceapartitionbetweenthe
systemicvenoussinusandtheappendageofthemorphologicallyright
atrium.Inthisheart,however,thereiscoexistingtricuspidatresiadueto
absenceoftherightatrioventricularconnectionsothatthepartitionmerely
exaggeratestheflowofbloodfromtheinferiorcavalveintothepatentoval
fossa.
Thehemodynamiceffectofthepartitionitselfisoflittlesignificancesincethe
bloodisrequiredtocrosstheovalfossa,andthepersistentvalveaugmentsthis
patternofflow.Theopeningofthesuperiorcavalvein,however,maynotbe
includedwithinthepartitionedvenoussinus(seeFig.30.7).Thelesionisof
moresignificancewhenthetricuspidandpulmonaryvalvesarepatent.The
persistingvalvecanthenbecomeaneurysmalandprotrudeintotheright
ventriclelikeawindsock(Fig.30.8).
FIG.30.8 Windsockremovedfromapatientwhosevalvesofthe
embryonicvenoussinushadbecomeaneurysmalandherniatedthrough
thetricuspidvalve,obstructingtheflowofbloodthroughtherightsideof
theheart.
Surgicalremovaliseasyoncediagnosishasbeenmade.10Diagnosisisnow
readilymadeusingcross-sectionalechocardiography(Fig.30.9),althoughthe
aneurysmalvalvedoesnotalwaysproducesymptomsandcanbeachance
finding.11
FIG.30.9 Cross-sectionalechocardiogramshowingdivisionofthe
morphologicallyrightatriumproducedbypersistenceofthevalvesofthe
embryonicsystemicvenoussinus.LA,Leftatrium;LV,leftventricle;RA,
rightatrium;RV,rightventricle.(CourtesyDr.I.B.Vijayalakshmi,Sri
JayadevaInstituteofCardiovascularSciencesandResearch,Bangalore,
India.)
