Andersons pediatric cardiology 1658
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FIG.61.11 Electrocardiographicpatterninrestrictivecardiomyopathy.A
12-leadelectrocardiogram(ECG)froma1-year-oldwithfamilialrestrictive
cardiomyopathy.TheECGshowsbiatrialenlargementandnonspecificTwavechanges.
ChestRadiograph
Thechestradiographtypicallyshowsevidenceofpulmonaryedemaaswellas
cardiomegalyduetosignificantatrialenlargement.
Echocardiography
Echocardiographyrevealsbiatrialdilationwithnormal-tosmall-appearing
nonhypertrophiedventriclesandnondilatedandevensmallventricularcavities
(Fig.61.12).Patientswithamixedphenotype(HCM/RCM)mayhavemild
hypertrophyoftheventricles.
FIG.61.12 Echocardiographicpatterninrestrictivecardiomyopathy.
Massivelydilatedleftandrightatriumswithoutleftventricularhypertrophy
ordilation.
Diastolicevaluationisconsistentwithincreasedmyocardialstiffnessand
increasedfillingpressures.ThereisincreasedE-wavevelocity,decreasedAwavevelocity,reversedpulmonaryveinA-wavevelocityandduration,
decreasedisovolumicrelaxationtime,decreaseddiastolicannularvelocities,and
increasedE/e’ratios.Hepaticveinreversaliscommonandmaybeaccentuated
withinspiration.
CardiopulmonaryExerciseTesting
Theroleofcardiopulmonaryexercisetestinginassessing/predictingclinical
outcomesinRCMisnotwellestablished.Exercisetestingmayhelptocorrelate
subjectivesymptomswithECGchangesathigherheartrates.
CardiacCatheterization
Cardiaccatheterizationisintegraltodecisionmakingandtheassessmentof
diseaseprogression.Thecharacteristicfindingofelevatedend-diastolicpressure
isuniformlypresent.Somepatientsalsoexhibitaclassicearlydiastolicdipin
ventricularpressurefollowedbyarapidriseandplateau(squarerootsign).As
notedpreviously,therecanbeprecipitousincreasesinpulmonaryarterypressure
andpulmonaryvascularresistance,thusroutineserialcatheterizationis
common.Endomyocardialbiopsyisnottypicallyindicatedinchildren,giventhe
typicaletiologiesandthenoninvasivemethodsavailabletomakeadiagnosis.
Management
SymptomaticTherapy
Medicaltherapyisfocusedonsymptommanagement.Diureticsareusedto
managesystemicandpulmonaryvenouscongestion.Fluidmanagementis
integral,asoverdiuresismayincreasesymptoms,giventheneedforadequate
preload.Ratecontrolmaybebeneficialinsomepatientsinordertomaintain
adequatefillingtimesanddecreasesymptoms.Afterload-reducingagentsare
typicallypoorlytoleratedandshouldbeavoided.Althoughthereislimited
literatureonthetrueriskofthromboembolisminRCMinchildren,giventhe
atrialsizeandpredispositiontoatrialarrhythmia,systemicanticoagulationwith
warfarinorantiplateletagentsiscommon.
Pacemaker/Defibrillator
Giventheriskofsuddendeath,somecentershaveadvocatedforthe
implantationofICDsforprimaryprevention.Therearecurrentlynodataonthe
efficacyofthisapproach,andgiventhatmanycentersalsoproceedwithearly
listingandhaverelativelyshortwaitlisttimes,thiswilllikelyprecludeany
assessmentofthebenefit.367
