Andersons pediatric cardiology 718

Diagnosis
Advancesinthefieldofnoninvasivecardiacimaginghavemarkedlyimproved
therateofdetectionofanomaliesofsystemicvenousreturn.Echocardiography
remainstheinitialmodalityofinvestigation.Ashighlightedpreviously,mostof
thesystemicvenousanomaliescanbedetectedinthisfashion.However,
evaluationbyechocardiographyislimitedbythewindowsavailablefor
interrogation.Suchinterrogationisparticularlylimitedindetectinganomalies
locatedintheperipheralportionsofthesystemicveins.Inaddition,
echocardiographicassessmentofthecoronarysinusremainssuboptimal.Not
uncommonly,thediagnosisremainsobscuredespitedetailedechocardiographic
evaluation.Thisisparticularlythecasewithanomalousdrainageasopposedto
anomalousconnection,sinceechocardiographicdiagnosisdependsupondirect
visualizationofananomalousconnection,whichisnotalwayspossible.

SalineContrastEchocardiography
Unlikesimpleechocardiography,useofthesalinecontrastechocardiogram
dependsonanalysisofthesequenceofappearanceofmicrobubblesinthe
differentcardiacchambersfollowinganinjectionofagitatedsaline.Multiple
injectionsfromdifferentlimbsarenecessaryforcompletediagnosis,andto
achievethefulldiagnosticpotentialofthetechnique.27,28,90,91Onthisbasis,we
haverecentlyproposedanalgorithmfordiagnosticevaluationofsuspected
anomalousdrainageofsystemicveins(Fig.27.21).Inourexperience,saline
contrastechocardiographyissafe,inexpensive,andeasytoperform.Inmost
instances,acarefullyperformedandlogicallyinterpretedsalinecontrast
echocardiogramcanclarifytheanomalousdrainageofcavalveins(Fig.27.22).
However,thediagnosticutilityforanomaliesofcoronarysinusandanomaliesof
thecourseofcavalveinsislimited.


FIG.27.21 Algorithmshowingthesequenceofeventsfromsuspicionof
anomaliesofsystemicvenousconnectiontotheirdiagnosis,emphasizing

theroleofsalinecontrastechocardiography.ASD,Atrialseptaldefect;
AVM,arteriovenousmalformation;CHD,congestiveheartdisease;CXR,
chestx-ray;ECG,electrocardiogram;Hb,hemoglobin;ICV,Inferiorcaval
vein;LA,leftatrium;LSCV,leftsuperiorcavalvein;LV,leftventricle;RA,
rightatrium;RSCV,rightsuperiorcavalvein;RV,rightventricle;SCV,
superiorcavalvein.(ModifiedfromGuptaSK,JunejaR,AndersonRH,et
al.Clarifyingtheanatomyandphysiologyoftotallyanomaloussystemic
venousconnection.AnnPediatrCardiol.2017;10:269–277.)

FIG.27.22 Sequenceofsalinecontrastechocardiographyperformedina
childwithapersistentleftsuperiorcavalveinconnectinganddraining
directlytotheleftatrium(LA).Theinjectionofagitatedsalineisfromthe


leftantecubitalvein.(A)MicrobubblesareseentobelimitedtotheLA.(B–
C)Thebubblessubsequentlyappearintheleftventricle(LV),andthen
throughtheleft-to-rightshuntintherightventricle(RV)astheycrossthe
ventricularseptaldefect(asterisk).RA,Rightatrium.

ComputedTomographicAngiography
Computedtomographyprovidesanexcellentrepresentationofcardiacaswellas
extracardiacanatomyandpermitsdiagnosisofalmostallanomaliesofsystemic
veins.However,thediagnosticyieldmaybelimitedincaseswithsuspected
functionaldrainageoftheinferiorcavalvein,particularlyifthecontrastis
injectedfromanarmvein.Therapidacquisitionofhighresolutionimages
permitspromptdiagnosiseveninsickchildren.Althoughmodernscannershave
mechanismstoreduceexposuretoradiation,thisremainsaconcern.Therefore
computedtomographyshouldonlybeperformedifthebenefitsachieved
outweightherisksofexposuretoradiation.

CardiacMagneticResonanceImaging
Thistechniquepermitsthediagnosisofallanomaliesofthesystemicveins
withoutanyriskofradiation.However,suchimagingistimeconsumingand
thereforeisnotsuitableforsickchildren.Four-dimensionalflowimagingwith
magneticresonanceimaging,byprovidinginformationaboutflowpatterns,is
moresuitedifanomalousdrainageofsystemicveinsisbeingevaluated.
Althoughnotevaluatedsystematically,precisedetectionofflowpatternsis
expectedtoprovideusefulinformationrelatedtotheconnectionanddrainageof
systemicveins.92,93

CatheterAngiography
Cardiaccatheterizationispreferableincaseswhereassessmentofboth
hemodynamicandmorphologicchangesisnecessary.28However,unlike
computedtomographyandmagneticresonanceimaging,selectiveinjectionsare
requiredineachsystemicveintoconfirmthediagnosis.Also,likecomputed
tomography,radiationremainsaproblem.