ElectrophysiologicTesting
Therecognitionandmanagementofarrhythmiasisanintegralpartofthe
practiceofpediatriccardiology.Arrhythmiasoccurinpatientswithoutstructural
orfunctionalheartdiseasebutareubiquitousinpatientswithheartdisease,
increasingwithdiseasecomplexity,patientage,andattimesofsurgicalor
catheter-basedintervention.Arrhythmiasareanintrinsicpartofthelifeofan
adultwithcongenitalheartdiseaseandareoftenthenidusforhospitalizationand
thecauseofdeteriorationinclinicalstatus.44Inallpatientswithstructuralheart
disease,theonsetorincreaseinarrhythmiaburdenmaybeaharbingerof
worseninghemodynamicstatus.
DocumentationofArrhythmia
Symptoms:Clinicalcluestothepresenceofanarrhythmiaincludecomplaints
ofpalpitations,chestpain,dizzinessandsyncope.Becauseofaninabilityto
fullycharacterizesymptoms,childrencanhavevagueorunusualcomplaints.
RecordingMethods
▪12-LeadECG:Thisisanecessarypartofthe
assessmentofallpatientswithsymptomssuggesting
anarrhythmia.Althoughoftennormal,theresultcan
uncoverevidenceofstructuralheartdisease,WPW
syndrome,LQTSandotherchannelopathic
conditions.Whenventricularectopyispresent,the
originoftheectopycanoftenbedetermined.
▪ECGorrhythmstrips:These—obtainedatthe
timeofaneventintheinpatientsetting,emergency
department,orbyparamedics—areinvaluable,often
diagnostic,butrarelyavailable.
▪Ambulatorymonitoring:Whensymptomsarevery
commonoranarrhythmiaisthoughttobeincessant,
24-hourambulatorymonitoringcanbehelpful.
Additionally,thistoolcanallowdeterminationofan
arrhythmiaorectopyburden,changesinheartrate
withsleepandactivities,andpointtofindingssuchas
intermittentpreexcitationorAVblock.Finally,
patientsoftencomplainofsymptomswherethereis
noarrhythmia,andthismonitorishelpfulinruling
outaproblemandconfirmingsinusrhythm.Thistest
canbeusedinplaceofanexercisetestinachildtoo
youngorunabletoundergoformalexercisetesting.
▪Eventmonitors:Therehasbeenanexplosioninthe
technologyusedineventmonitoring.Thesedevices
areusefulinconfirminginfrequentarrhythmiasand
sinusrhythminpatientswithsymptomsthatarenot
arrhythmic.
▪Theserecordersareplacedexternallyandcan
record10to30daysofdata.Thepatientis
responsibleforrecordingevents,although
newer-generationdevicescanrecord
automaticallyandsomeactasHolter
monitoringdevices,providingdataonthe
averageandrangeofheartrateandectopy
burden.Therearesomelimitationswith
compliance,asthiscanbeinconvenient.
▪Implantablelooprecorders:Thesedevices
recordECGdataforyears.Thesedevicesare
verysmallandcanbeimplantedwithalocal
anestheticand/orminimal
sedation/anesthesia.Theycanrecorddatain
apatient-triggeredoradevice-triggered
mannerandareusefulforinfrequentevents
andeventswherethepatientcannotactivate
thedeviceowingtolossofconsciousness.
Thesedevicescanautomaticallyrecorddata
outsideofthepresetnormalanddataare
downloadedintheofficeorremotely,similar
topacemakerinterrogation.
▪Smartphone-enabledECGdevices:These
canbepurchasedbyfamiliesandgenerate
tracingsofdiagnosticqualityinchildren.In
pediatricseriesofthistechnology,user
satisfactionwasextremelypositiveanduse
ofthedeviceallowedsuccessfuloutpatient
managementofarrhythmiasinchildrenby
potentiallyreducingemergencydepartment
visitsandhealthcarecosts.156
▪Exercisetesting:Thisisusefulforarrhythmiasthat
occurwithexercise.Thistestisparticularly
convenientforestablishingthediagnosisofCPVT
andsomeformsofLQTS,RVOT,andfascicularVT.
Atesttailoredtotheclinicaleventmaybemore
usefulthanthestandardprotocoltypicalofadult-