Andersons pediatric cardiology 1655
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Electrocardiography
TheECGfindingsinDCMarenonspecificandmaybenormal(Fig.61.9).
Patientswithheartfailuretypicallypresentwithsinustachycardia,andthere
maybeatrialorventricularhypertrophyand/orectopy.Qwavesmaybepresent
insituationswherethereisfocalmyocardialfibrosis.DeepQwavesinleadsI
andaVLraisethepossibilityofanomalousoriginoftheleftcoronaryarteryfrom
thepulmonaryartery.Thepresenceofconductiondiseaseraisessuspicionof
SCN5AandLMNAmutationsinadditiontomyocarditis.
FIG.61.9 12-leadelectrocardiogramfroma2-year-oldwithfamilial
idiopathicdilatedcardiomyopathyshowsnonspecificST-segmentandTwaveabnormalities.
ChestRadiograph
Thechestradiographtypicallyshowscardiomegalywithevidenceofpulmonary
edemawithorwithouteffusions.Incasesofacutemyocarditisorearly
cardiotoxictherapy-induceddisease,thecardiacsizemayberelativelysmalland
dilationwillevolvewithtime.
Echocardiography
Thediagnosisistypicallymadebyechocardiographybasedonthepresenceof
leftventriculardilation(z-scoregreaterthan+2)andsystolicdysfunction(Fig.
61.10).RightventriculardysfunctionanddilationmaycoexistwithLVsystolic
dysfunction.Thedegreeofleftventriculardilationandseverityofsystolic
dysfunctionareassociatedwithworseprognosisinpediatricDCM.295–298Threedimensionalechocardiographyisincreasinglybeingusedtoquantifyleft
ventricularindicesandfunctiongiventhatitappearstobemoreaccurateand
reproduciblethanM-modeandtwo-dimensionalmeasurement.299Regionalwall
motionabnormalitiesmaybepresentinthesettingoffocalfibrosisorcoronary
anomalies.Echocardiographyshouldalsobeusedtoassessforevidenceofatrial
orventricularthrombus.300
FIG.61.10 Echocardiographicfeaturesofdilatedcardiomyopathy.(A)
Apicalfour-chamberviewfroma15-month-oldwithdilatedcardiomyopathy,
dilatedleftventricularcavitywiththinwalls.(B)Parasternallong-axisview,
againshowingadilated,thin-walledleftventriclefromthesamepatient.
Abnormalventricularfillingpatternsandevidenceofdiastolicdysfunctionare
commoninpatientswithDCMandmaypredictdiseaseprogression.295,301
Dopplerevaluationofthetricuspidandpulmonaryvalvesshouldbeperformed
toevaluateforevidenceofelevatedpulmonaryarterypressures.
CardiacMagneticResonanceImaging
CMRIhasbeenusedtoassessforthepresenceofmyocardialfibrosisinpatients
withDCM.ThepresenceoflategadoliniumenhancementLGEonCMRIis
associatedwithaworseprognosisinadultnonischemicDCM.302Childrenand
adolescentswithDCMrarelyhavemyocardialfibrosisintheabsenceof
myocarditis,andtheprognosticvalueofLGEremainsuncleartodate.303The
differencesinthefrequencyofLGEmaybeareflectionoftheage-dependent
phenotype,andT1mappingmayprovideabettermeasureofadversemyocardial
remodeling.304TheprognosticroleofCMRIindystrophinopathyismore
established.305,306
CardiacCatheterization
Cardiaccatheterizationmaybeusedforinvasivehemodynamicassessmentas
wellashistologicevaluationincasesofdiagnosticuncertainty.However,
noninvasiveassessmentofpulmonaryarterypressuresandmyocardialtissue
characterizationbyCMRIhavelargelysupplantedcatheterizationinthe
evaluationofDCM.
CardiopulmonaryExerciseTesting
Cardiopulmonaryexercisetestingisfeasibleindevelopmentallyappropriate
childrenandmayhaveaprognosticrole.Lowerpeakoxygenuptake,in
particular,portendsaworseoutcome.307,308
Management
ConsensusmanagementguidelinesaregenerallylackinginpediatricDCM.The
