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Andersons pediatric cardiology 1152

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FIG.43.12 ContinuousDopplertracethroughthetricuspidvalveofan
infantwithpulmonaryatresiaandintactventricularseptum.Thereishighvelocitytricuspidregurgitation,withapeakvelocityofmorethan5m/s,
indicativeofsuprasystemicrightventricularpressures.

TheRVshouldbeassessedtodetermineitsoverallsizeandthedegreeof
muralovergrowth(Fig.43.13).Ajudgmentshouldbemadeastowhetherthe
cavitypossessesallthreeofitscomponents,oronlyoneortwo(Videos43.2and
43.3).Inourexperience,therightventricularcavitymayseemsmaller
echocardiographicallythanitappearsangiographically,largelybecausethe
apicaltrabecularzonemayseemcompletelyobliteratedwhen,inreality,there
areintertrabecularspaces.Thepresenceoftinyventricularseptaldefectsshould
benoted.Anassessmentofthepatencyoftheinfundibulumshouldbemade
(Video43.4),particularlyfromthesubcostalparaobliqueview.Theatresiamay
bemembranous(Fig.43.14)ormusculardependingontheextentofmuscular
muralhypertrophy.Thepresenceofanyforwardorretrogradeflowacrossthe
pulmonaryvalveshouldbeassessedtoexcludecriticalpulmonarystenosisor
functionalatresia.Thepresenceofanyfistulouscommunicationtothecoronary
arteriesshouldbesought(Fig.43.15;Video43.5).


FIG.43.13 Echocardiogramsshowingthefourchambersandillustrating
therangeinsizeoftherightventricularcavity.(A)Tripartiterightventricle
ofnear-normalsize(arrow)withminimalmuralhypertrophy.(B)Unipartite
rightventriclewithconsiderablemuralhypertrophyandobliterationofthe
cavity(arrow).

FIG.43.14 Echocardiogramsshowinganimperforatepulmonaryvalve
suitableforballoonperforation.(A)Parasternalshort-axisviewin
ventriculardiastoledemonstratingnormalappearanceofthevalveleaflets.
(B)Imperforatevalveinsystole,withexcursionofthefusedvalvarleaflets.



Thisistheechocardiographicequivalentofthemorphologicspecimen
showninFig.43.3.AV,Aorticvalve;PA,pulmonarytrunk;PV,pulmonary
valve;RV,rightventricle.(FromAbramsDJR,RigbyML,DaubeneyPEF.
Membranouspulmonaryatresiatreatedbyradiofrequency-assistedballoon
pulmonaryvalvotomy.Circulation.2003;107:e98–e99.)

FIG.43.15 Parasternalshort-axisechocardiographicimageshowing
fistulouscommunicationsfromtherightventricletotherightcoronary
artery.

Thepulmonarytrunkandbranchesshouldbemeasuredtoascertaintheirsize,
andthesourceofbloodflowtothelungsdetermined.Normalpulmonaryvenous
returnshouldbeconfirmed.Structureandfunctionoftheleftventricleshouldbe
assessed,includingregionalabnormalitiesofmuralmotion.
Followingsuchinvestigations,itshouldbepossibletodecidewhetherthe
long-termstrategyisforbiventricularasopposedtouniventricularrepair,andto
plantheinitialintervention.

CardiacCatheterizationandAngiography
Inadditiontoechocardiography,fromadiagnosticperspective,cardiac
catheterizationandangiographymaybehelpfulintheevaluationofaninfant
withthislesion,particularlytoassessthesizeoftheRVandthepresenceand
severityoffistulouscommunications.Theusualapproachisviathefemoralvein
becauseitcanbedifficulttoentertheRVfromtheumbilicalvein.58Thelatter



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