EHRA/HRS/APHRS Expert Consensus on Ventricular Arrhythmias

31 Pages·2014·1.58 MB·English

by  Christian Torp Pedersen

EHRA/HRS/APHRS Expert Consensus on
Ventricular Arrhythmias
ChristianTorpPedersen (EHRAChairperson,Denmark), G. NealKay(HRS Chairperson,USA),
Jonathan Kalman (APHRS Chairperson, Australia), Martin Borggrefe (Germany),
PaoloDella-Bella(Italy),TimmDickfeld(USA),PaulDorian(Canada),HeikkiHuikuri(Finland),
Youg-Hoon Kim (Korea), Bradley Knight (USA), Francis Marchlinski (USA), David Ross
(Australia), Frédéric Sacher (France), John Sapp (Canada), Kalyanam Shivkumar (USA),
Kyoko Soejima (Japan), Hiroshi Tada (Japan), Mark E. Alexander (USA),
John K. Triedman (USA), Takumi Yamada (USA), and Paulus Kirchhof (Germany)
Document Reviewers: Gregory Y.H. Lip (UK), Karl Heinz Kuck (Germany),
Lluís Mont (Spain), David Haines (USA), Jukia Indik (USA), John Dimarco (USA),
Derek Exner (Canada), Yoshito Iesaka (Japan), Irene Savelieva (on behalf of EP-Europace, UK)
Introduction This document addresses the indications for diagnostic
testing, the present state of prognostic risk stratification, and
ThisinternationalconsensusstatementoftheEuropeanHeart
thetreatmentstrategiesthathavebeendemonstratedtoimprove
RhythmAssociation(EHRA),HeartRhythmSociety(HRS),
andAsiaPacificHeartRhythmSocietyisintendedtoprovide the clinical outcome of patients with VAs. In addition, this
document includes recommendations for referral of patients to
clinical guidance for the management of patients with
centres with specialized expertise in the management of
ventricular arrhythmias (VAs). It summarizes the consensus
arrhythmias. Wherever appropriate, the reader is referred to
oftheinternationalwritinggroupmembersandisbasedona
other publications regarding the indications for implantable
systematic reviewofthemedicalliteratureregardingVAs.
cardioverter-defibrillator (ICD) implantation,1,2 catheter abla-
ThespectrumofVAsrangesfromthosethatarebenignand
tion,3inheritedarrhythmiasyndromes,4,5congenitalheartdisease
asymptomatic to those that produce severesymptomsinclud-
(CHD),6theuseofamiodarone,7andthemanagementofpatient
ing sudden cardiac death (SCD). In addition, many patients
withICDshocks,8syncope,9orthosenearingendoflife.10The
exhibitmultipleformsofVAsovertime.Thus,clinicianswho
consensus recommendations in this document use the standard
encounterpatientswithVAsfaceimportantquestionsregard-
Class I, IIa, IIb, and III classification11 and the corresponding
ingwhichdiagnostictestsareneededandwhichtreatments,if
language: ‘is recommended’ for Class I consensus recommen-
any,shouldbeoffered.TheWritingCommitteerecognizesthat
dation;‘can be useful’ for a Class IIa consensus recommenda-
themannerinwhichpatientspresentwithVAsvariesgreatly.
The electrocardiographic recording of a VA may be the first tion; ‘may be considered’ to signify a Class IIb consensus
recommendation; ‘should not’ or ‘is not recommended’ for a
andonlymanifestationofacardiacabnormality;alternatively,
Class III consensus recommendation (failure to provide any
patients with a prior diagnosis of cardiac disease may later
develop these arrhythmias. Thus, the specific arrhythmia and additionalbenefitand/ormaybeharmful).Thelevelofevidence
supportingtheserecommendationsisdefinedas‘A’,‘B’,or‘C’
theunderlyingstructuralheartdisease(SHD),ifany,mayhave
dependingonthe number of populationsstudied,whetherdata
importantprognosticandtreatmentimplications.
are derived from randomized clinical trials, non-randomized
studies, or, in the absence of large studies, the consensus
DevelopedinpartnershipwiththeEuropeanHeartRhythmAssociation opinionsofexpertsfromcasestudiesorstandardsofcare.Most
(EHRA),aregisteredbranchoftheEuropeanSocietyofCardiology(ESC),
medicalinterventionstopreventsuddendeathandtotreatVAs
theHeartRhythmSociety(HRS),theAsiaPacificHeartRhythmSociety
weredevelopedinanerawhenpatientcohortsweresmalland
(APHRS),andincollaborationwiththePediatricandCongenitalElectro-
theacceptedstandardstodemonstrateeffectivenesswerelower
physiologySociety(PACES).EndorsedbyEHRA,APHRS,theAssociation
fortheEuropeanPediatricCardiology(AEPC),andPACESinMay2014 than today. Many interventions to terminate or suppress VAs
andbyHRSinJune2014.Thearticlehasbeenco-publishedwithpermission havesincebeenusedinmanypatients,andovertimedifferent
in EP-Europace, Journal of Arrhythmia and Heart Rhythm. All rights treatment ‘patterns’ have developed in different regions of the
reservedinrespectofJournalofArrhythmiaandEP-Europace.Correspon-
world.Thewritinggrouphastriedtoaccommodatereasonable
denceto:HannahPeachey-CentreforCardiovascularSciences,Institute
variationsintreatmentinourrecommendations,andhaverelied
for Biomedical Research College of Medical and Dental Sciences, Uni-
versity of Birmingham, Edgbaston Birmingham, UK. Tel: þ44 121 414 upon expert consensus for many of the recommendations put
5916;fax:þ441214158817.E-mailaddress:[email protected]. forwardinthisdocument.Thisisreflectedbytherelativelylow
1547-5271/$-seefrontmatterBTheAuthor2014.ForEP-Europace,BTheAuthor2014. http://dx.doi.org/10.1016/j.hrthm.2014.07.024
Pedersen etal EHRA/HRS/APHRS Expert Consensus on Ventricular Arrhythmias e167
level of evidence that supports the majority of our recommen- twoparts[prematureventricularcomplexes(PVCs)andnon-
dations. Each of the recommendations was voted upon by the sustained ventricular tachycardia (NSVT)].
WritingCommitteeandonlythosewheretherewasatleast80% The consensus group believes that patients with non-
agreementhavebeenincluded. sustainedVAsneedastandardized diagnosticworkup.This
The consensus group has approached VAs by whether issummarizedhere, andexplained in the two sections.
they are sustained or non-sustained. The first part of this For treatment of patients with non-sustained VAs, we
documentdealswithnon-sustainedarrhythmias,discussedin propose the following consensus recommendations.
Expert consensus recommendations on general diagnostic work-up
(1) Allpatientswithdocumentednon-sustainedorsustainedVAsshouldhavearesting12-leadelectrocardiogram(ECG)andatransthoracic
echocardiogramtodetectunderlyingheartdiseaseincludinginheritedandacquiredcardiomyopathies.Especiallyinpatientsinwhomthe
arrhythmiamorphologysuggestssuchaspecificaetiology,valvularandrightheartmorphologyandfunctionshouldbeassessed.IIaLOEB
(2) Repeat12-leadECGsshouldbeconsideredwheneveraninheritedarrhythmiasyndromewithvaryingelectrocardiographic
manifestationsoratransientcondition(e.g.coronaryspasm)issuspected.IIaLOEC
(3) Inselectedpatients,andespeciallyinthosewithsustainedarrhythmias,asecondimagingmodality(e.g.amagneticresonancestudy,
stresstestingwithperfusionscanning,orechocardiography)shouldbeconsideredtodetectsubtleSHD.IIaLOEB
(4) AtestformyocardialischaemiashouldbeconsideredinallpatientswithVAsinwhomtheclinicalpresentationand/orthetypeof
arrhythmiasuggeststhepresenceofcoronaryarterydisease.IIaLOEC
(5) Theriskofcardiaceventsisoftendictatedbyanunderlyingheartdiseaseratherthanthearrhythmia.Therefore,optimaltreatmentof
underlyingcardiovasculardiseasesandriskfactorsisrecommended.ILOEA
(6) ProlongedECGmonitoringbyHolterECG,prolongedECGeventmonitoring,orimplantablelooprecordersshouldbeconsideredwhen
documentationoffurther,potentiallylongerarrhythmiaswouldchangemanagement.IIaLOEC
(7) InpatientswithincompletelycharacterizedarrhythmiaswithwideQRScomplexes,bothsupraventricularandVAsshouldbeconsidered
indevelopingacareplan.IIaLOEC
Expert consensus recommendations on non-sustained
(1) Infrequentventricularectopicbeats,couplets,andtripletswithoutothersignsofanunderlyingSHDoraninheritedarrhythmia
syndromeshouldbeconsideredasanormalvariantinasymptomaticpatients.IIaLOEC
(2) Aninvasiveelectrophysiologicalstudy(EPS)shouldbeconsideredinpatientswithsignificantSHDandnon-sustainedVAsespeciallyif
accompaniedbyunexplainedsymptomssuchassyncope,near-syncope,orsustainedpalpitationsIIaLOEC
(3) NotreatmentotherthanreassuranceisneededforpatientswithneitherSHDnoraninheritedarrhythmogenicdisorderwhohave
asymptomaticormildlysymptomaticPVCs.ILOEC
(4) Itisrecommendedtotreatsurvivorsofamyocardialinfarction(MI)andotherpatientwithreducedleftventricular(LV)functionand
non-sustainedVAswithabeta-blockerunlesstheseagentsarecontraindicated.ILOEA
(5) Atherapeutictrialofbeta-blockersmaybeconsideredinsymptomaticpatientswithnon-sustainedVAs.IIbLOEC
(6) InsuitablepatientswithoutSHD,anon-dihydropyridinecalciumchannelantagonistmaybeconsideredasanalternativetobeta-
blockertreatment.IIbC
(7) Inpatientswhosufferfromsymptomaticnon-sustainedVAsonanadequatelydosedbeta-blockeroranon-dihydropyridinecalcium
channelantagonist,treatmentwithanantiarrhythmicdrug(AAD;amiodarone,flecainide,mexiletine,propafenone,sotalol)maybe
consideredtoimprovesymptomsassociatedwitharrhythmiaepisodes.IIbLOEC
(a) FlecainideandpropafenonearenotrecommendedtosuppressPVCsinpatientswithreducedLVfunction(unlesscausedby
ventricularectopyitself),myocardialischaemia,ormyocardialscar.IIILOEA
(b) SotalolshouldbeusedwithcautioninpatientswithchronickidneydiseaseandshouldbeavoidedinpatientswithaprolongedQT
intervalatbaselineorwithexcessiveprolongationofQTinterval(.0.50s)upontherapyinitiation.ILOEB
(c) Amiodaroneappearstohavelessoverallpro-arrhythmicriskthanotherAADsinpatientswithheartfailureandmaybepreferredto
othermembrane-activeAADsunlessafunctioningdefibrillatorhasbeenimplanted.IIbLOEC
(8) CatheterablationmaybebeneficialbyimprovingsymptomsorLVdysfunctioninpatientssufferingfromfrequentnon-sustainedVAs
(e.g..PVC10000per24h)inpatientswithsignificantsymptomsorLVdysfunctionwithoutanotherdetectablecause.IIaLOEB
(9) Amiodarone,sotalol,and/orotherbeta-blockersareusefulpharmacologicaladjunctstoimplantationofadefibrillator(e.g.toreduceshocks)andto
suppresssymptomaticNSVTinpatientswhoareunsuitableforICDtherapy,inadditiontooptimalmedicaltherapyforpatientswithheartfailure.
IIbLOEB
e168 Heart Rhythm, Vol11, No 10, October 2014
Premature ventricular complexes Diagnostic evaluation
Prematureventricularcomplexes(PVCs)arecommonboth Electrocardiogram andambulatory monitoring
in patients with and without SHD and may be asympto- The presence of at least some PVCs during 24 h ambulatory
maticevenforpatientswithhighfrequencyofthesebeats. monitoring is extremely common and may be considered
Other patients may be highly symptomatic with relatively normal.BecausethefindingofPVCsduring24hambulatory
few ectopic beats.12 Although a recent meta-analysis13 of monitoringisverylikely,anyconclusionthattheyarerelatedto
patients without clinically apparent SHD demonstrated an symptomsrequirescarefulcorrelation.Intwostudiesinwhich
increased incidence of adverse events in patients with SHDwasrigorouslyexcluded,only2and4%had.50or.100
frequent PVCs, only one of the included studies used PVCs/24 h, respectively.33,34 The vast majority of patients
echocardiography to establish structural disease. The without SHD who have PVCs have a benign prognosis. An
independent prognostic importance of PVCs in the pres- exceptionmaybeaverysmallsubsetofpatientswithPVCsthat
ence of structural disease is not clear. Early studies haveashortcouplinginterval(,300ms)betweenthepremature
demonstratedanassociationwithincreasedcardiovascular andtheprecedingbeats,afindingwhichsuggeststheshortQT
mortalityafterMI14,15andwithincreasedtotalmortalityin syndromeandincreasestheriskofmalignantVAs.35Itshould
patients with LV hypertrophy (LVH).16 However, these beemphasizedthatthisisaverysmallminorityofpatientswith
studieswereobservationalandperformedinanerapriorto PVCs.AswithotherVAs,thefirststepintheevaluationofa
modern management.17 In a study of patients with con- patient with PVCs is to determine the presence or absence of
gestive heart failure [ejection fraction (EF), 35%], PVC SHD (Figures 1 and 2). For patients with arrhythmic or other
frequencydidnot predict the riskof suddendeathanddid cardiac symptoms, a resting 12-lead ECG is very helpful to
not provide prognostic information beyond other clinical evaluate the presence of myocardial scar (Q-waves or fractio-
variables.18 nated QRS complexes), the QT interval, ventricular hyper-
trophy, and other evidence of SHD. An echocardiogram
providesassessmentofrightventricular(RV)andLVstructure
Premature ventricular complex-induced and function, valvular abnormalities, and pulmonary artery
cardiomyopathy systolic pressure and is recommended for patients with
Several studieshave demonstratedanassociationbetween symptomaticPVCs,ahighfrequencyofPVCs(.10%burden),
frequent PVCs and a potentially reversible cardiomyop- orwhenthepresenceofSHDissuspected.
athy, which in selected patients resolves after catheter
ablation.19–24The number ofPVCs/24hthatisassociated Exercise testing
with impaired LV function has generally been reported at For selected patients, especially when there is a suggestion of
burdensabove15–25%ofthetotalcardiacbeats,though symptomsassociatedwithexercise,exercisestresstestingshould
this maybe as low as10%21–30(Table 1).However,since be considered to determine whether PVCs are potentiated or
PVCsmaybetheresultofanunderlyingcardiomyopathy, suppressed by exercise, to assess whether longer duration VAs
it may be difficult to prospectively determine which of are provoked. A negative exercise test can decrease the
these sequences is operative in a given patient.31 Impor- probabilitythatcatecholaminergicpolymorphicventriculartachy-
tantly, the vast majority of patients with frequent PVCs cardia (CPVT) is the underlying cause. Premature ventricular
will not go on to develop cardiomyopathy but currently complexes that worsen with exercise should prompt further
available data do not allow for accurate risk prediction. A investigationasthesepatientsaremorelikelytorequiretreatment.
recent longitudinal study followed 239 patients with
frequent PVCs (.1000 per day) and no SHD [echo and Imaging investigations
magnetic resonance imaging (MRI)] for 5.6 years with no Although the majority of patients with PVCs can be
adverse cardiac events and no decline in overall LV accurately assessed with a 12-lead ECG and echocardiog-
ejection fraction (LVEF).32 raphy, contrastenhanced MRI may provide additional
Table1 PVCburdenassociatedwithLVdysfunction
n %LVd %VEsLVd %VEsnormalLV P PredictivePVCburden
Banetal.21 127(28LVd) 22% 31þ11% 22þ10% 0.001 26%
Deyelletal.25 90(24LVd) 27% 32þ12% 27þ12% 0.077 –
Munozetal.26 70(LVd17) 24% 29þ15% 17þ14% 0.004 10%RV;20%LV
Olgunetal.27 51(21LVd) 41% 30þ11% 14þ15% 0.0001 –
Hasdemiretal.28 249(17LVd) 7% 29þ9% 8þ7% 0.001 16%
Bamanetal.29 174(57LVd) 33% 33þ13% 13þ12% 0.0001 24%
Kaneietal.30 108(21LVd) 19% 13þ11%a 7þ9%a 0.004 –
LowestPVCcountassociatedwithLVdysfunctionwas10%(Baman).
LV¼leftventricle;LVD¼leftventriculardysfunction;PVC¼prematureventricularcomplexes;VE¼ventricularectopic.
aAssuming100000beats/24h.
Pedersen etal EHRA/HRS/APHRS Expert Consensus on Ventricular Arrhythmias e169
No evidence of History and PE Abnormal
SHD or sustained VAs ECG basic evaluation
No SHD Echo SHDa
Amb monitoring
Family History
>10 000
LV dysfunction PVCs/24 h Assess PVC
and high PVC burden burden
<10,000
PVCs/24 hr
Possible reversible
LV dysfunction
Reassurance (consider MRI-DE)c Reassurance re PVCs
Rx for SHDb
Continued PVC symptoms Treat PVCs - Ongoing PVC symptoms
- PVC interference with CRT
Catheter ablation
Medical treatment Consider if:
Failed/intolerant/declined medical Rx,
Single/dominant PVC morphology,
Probable reversible LV dysfunction
Figure1 ManagementofPVCs.a.Seetablefordefinitionsofstructuralheartdisease;b.MedicaltherapyþICD;c.Absenceofhighscarburdensuggestsreversibility.
CRT¼cardiacresynchronisationtherapy;ICD¼implantablecardioverter-defibrillator;LV¼leftventricular;MRI-DE¼magneticresonanceimagingwithdelayed
enhancement;PE¼physicalexamination;PVC¼prematureventricularcomplexes;Rx¼therapy;SHD¼structuralheartdisease;VAs¼ventriculararrhythmias.
diagnosticandprognosticdatawhenthepresenceorabsence amyloidosis, and arrhythmogenic right ventricular cardio-
of SHD remains in doubt.36 While there are no large-scale myopathy(ARVC).37–39Intheseconditions,thepresenceof
studies investigating which patients should undergo MRI, ventricular wall motion abnormalities or myocardial scar
the management of several forms of SHD associated with detected by delayed gadolinium enhancement may provide
PVCs may be guided by MRI, including dilated cardiomy- usefulprognosticinformation.Inselectedpatientsforwhom
opathy, hypertrophic cardiomyopathy (HCM), sarcoidosis, the diagnosis of ARVC is suspected, the signal-averaged
Patient history
Prior diagnosis of SHD?
Risk factors for SHD?
Symptoms of SHD?
Non-cardiac disorders that may
affect the heart?
Syncope?
Family Hx SCD?
Family Hx of SHD?
Physical examination
evidence for SHD?
Electrocardiogram
baseline 12-lead ECG
12-lead ECG during VA
ECG monitoring
Echocardiogram
Exercise testing / Imaging
coronary arteriography if
indicated
Abnormalbasicevaluation Normalbasicevaluation
Inconclusive
Structural heart basic evaluation No structural
disease heart disease
Advanced imaging
MRI-DE
Abnormal advanced imaging CT Normal advanced imaging
coronary arteriography
Figure2 Evaluationforthepresenceorabsenceofstructuralheartdisease.CT¼computedtomography;MRI-DE¼magneticresonanceimagingwith
delayedenhancement;VA¼ventriculararrhythmia.
e170 Heart Rhythm, Vol11, No 10, October 2014
ECG(SAECG)mayprovideusefulinformationandformsa Catheter ablation
minor diagnosticcriterion for this disorder. Randomized trials of PVC suppression with catheter
ablation have not been performed. However, multiple
studies indicate high efficacy of ablation with PVC
Treatment eliminationin74–100%ofpatients.44–57However,these
studies have typically included highly symptomatic
Indications fortreatment inpatientswithout structural heart
patientstypicallywithaveryhighburdenofPVCs.Thus,
disease
catheter ablation should only be considered for patients
In the absence of SHD, the most common indication for
whoaremarkedlysymptomaticwithveryfrequentPVCs.
treatingPVCsremainsthepresenceofsymptomsthatarenot
In addition, procedural success may be dependent on site
improvedbyexplanationoftheirbenignnatureandreassur-
oforiginwithlowerefficacyreportedforcoronaryvenous
ance from the physician. In addition, some patients may
and epicardial foci than for other sites.49,58 Although
require treatment for frequent asymptomatic PVCs if longi-
complete PVC elimination is the goal of ablation, it
tudinal imaging surveillance reveals an interval decline in
shouldbenotedthatpartialsuccessmaystillbeassociated
LVsystolicfunctionoranincreaseinchambervolume.For
withsignificantimprovementinLVsystolicfunction.The
patients with .10 000 PVCs/24 h, follow-up with repeat
efficacy of catheter ablation may be reduced for patients
echocardiography and Holter monitoring should be consid-
with multiple morphologies of PVCs or those for whom
ered. In patients with fewer PVCs, further investigation is
the clinical PVC morphology cannot be induced at the
onlynecessary shouldsymptoms increase. Itshouldalso be
recognizedthat PVC burden often fluctuates over time. timeoftheprocedure.Thepublishedcomplicationratesof
catheter ablation for PVC suppression are generally low
("1%). Catheter ablation of PVCs is recommended for
highly selected patients who remain very symptomatic
Indicationsfortreatmentinpatientswithstructuralheartdisease
despiteconservativetreatmentorforthosewithveryhigh
InpatientswithSHD,symptomsformtheprimarygroundsfor
PVC burdens associated with a decline in LV systolic
consideringwhethertreatmentisindicated.Eliminationofhigh
function.
burdenPVCs(.10%)inpatientswithimpairedLVfunctioncan
be associated with significant improvement of LV func-
tion,19,20 even when significant scarring is present.22,23 Cath- Non-sustained ventricular tachycardia
eterablationmayalsobehelpfulwhenfrequentPVCsinterfere Although several different definitions have been used,59
withcardiacresynchronizationtherapy.40 NSVTisdefinedasrunsofbeatsarisingfromtheventricles
withdurationbetween3beatsand30sandwithcyclelength
of ,600 ms (.100 b.p.m.).60 Similar to PVCs, NSVT is a
relativelycommonfindinginpatientswitheitherstructurally
Management of premature ventricular complexes
normalorabnormalhearts.59,61,62Non-sustainedventricular
(options)
tachycardia is found in nearly 6% of patients evaluated for
Medical therapy
palpitations.63Diagnosticandtherapeuticconsiderationsfor
ForpatientswithoutSHDandmildsymptoms,thefirststep
NSVT are included in several 3,60,64 recent guideline and
intreatmentofpatientswithPVCsiseducationofthebenign
consensusdocuments.Ingeneral,therapyfortheunderlying
nature of this arrhythmia and reassurance. No large-scale
cardiac disease is indicated rather than for the arrhythmia
randomizedtrialsofdrugtreatmentforPVCsintheabsence
itself. However, the finding of NSVT should always trigger
of heart disease have been performed. For patients whose
furtherevaluationofthepatientandapracticalapproachcan
symptomsarenoteffectivelymanagedinthismanner,atrial
be usefully divided into a general approach (Table 2),
ofbeta-blockersornon-dihydropyridinecalciumantagonists
patientswithanapparentlynormalheart(Table3)andthose
may be considered though the efficacy of these agents is
withSHD (Table 4).
quitelimitedwithonly10–15%ofpatientsachieving.90%
PVC suppression,41 similar to placebo.42 It should also be
Non-sustained ventricular tachycardia in the
recognized that the data supporting the use of calcium
structurally normal heart
blockersarelessthanforbeta-blockersandthattheseagents
may themselves produce significant symptoms. While Exercise-related NSVT is relatively common and appears
membrane-active AADs are more effective to suppress to be associated with a worse prognosis when it occurs
PVCs, the risk – benefit ratio has not been carefully during recovery.65,66 Polymorphic NSVT requires exten-
evaluated in patients without SHD. Nevertheless, these sive evaluation in both symptomatic and asymptomatic
agents are highly effective and may significantly improve patients with careful assessment for the presence of
symptomsinmarkedlysymptomaticpatients.Becausethese coronary ischaemia. An important inherited arrhythmia
agents may increase the risk of mortality in patients with which may present as exercise-induced NSVT is
significantSHD,perhapswiththeexceptionofamiodarone, CPVT.72,73 This condition is typically manifested by
caution is advised before using them for PVC polymorphic or bidirectional VT which are triggered by
suppression.41,43 sympathetic stimulation and exercise (commonly
Pedersen etal EHRA/HRS/APHRS Expert Consensus on Ventricular Arrhythmias e171
Table2 Evaluationofpatientswithnonsustainedventricular the significance of NSVT in athletes without a structural
tachycardia cardiacdisease,discontinuationoftrainingisnotgenerally
Standardevaluation recommended.
History
Priorcardiovasculardisease?
Non-sustained ventricular tachycardia in structural
Hypertension,knowncardiacdisease?
Syncopeornear-syncope? heart disease
Sustainedpalpitations? Non-sustainedventriculartachycardiaiscommoninischae-
Relationofsymptomstoexercise? micheartdiseaseandcanberecordedin30–80%ofpatients
Familyhistory
during long-term ECG monitoring where it is usually
SCD,inheritedarrhythmiasyndromes,coronaryarterydisease,
cardiomyopathy? asymptomatic.60 No studies have demonstrated a mortality
Medications benefit of suppressing NSVT with either AADs or catheter
QTprolongingdrugs,sodiumchannelblockers,drug ablation and treatment is usually not indicated in asympto-
interactions?
matic patients. A range of studies have demonstrated that
Physicalexamination
NSVT occurring during the first few days after an acute
Evidenceofcardiacdisease?
Twelve-leadECG coronary eventhasnoadverse long-term prognosticsignifi-
Q-waves,ischaemicchanges,prolongedorfractionatedQRS,QT cance.However,whenNSVToccurs48hormoreafterMI,
prolongationorshortening,STelevationV1–V3,early there is an increased mortality and morbidity even when
repolarization,epsilonwaves,oranteriorT-waveinversion asymptomatic.74 For a patient with non-ischaemic dilated
Echocardiography
cardiomyopathy, the prognostic significance of NSVT is
Ventricularchamberdimensionsandthickness,wallmotion,
systolicanddiastolicfunction,valvularfunction,congenital uncertainandnostudieshaveprovidedpreciseguidancefor
anomalies,pulmonaryarterialsystolicpressure treatment in this group of patients.75
Laboratory TheoccurrenceofNSVTinpatientswithanimplantedICD
Serumelectrolytes,renalfunction
is associated with an increased frequency of shocks and all-
Furtherevaluation causemortality.76Forthesepatients,programmingtheICDto
Exercisetesting a long VT detection time and a high ventricular fibrillation
Suspicionofcoronaryarterydisease,exercise-relatedsymptoms, (VF)detectionratemaybeespeciallyimportant.77,78
borderlineQTinterval
Coronaryarteriography
Suspicionofcoronaryarterydiseaseorcoronaryarteryanomaly Diagnostic evaluation
CardiacMRI For patients with an apparently normal heart, the 12-lead
SuspicionofARVC,HCM,cardiacsarcoidosis,congenitalanomalies ECG should be scrutinized for evidence of typical outflow
Genetictesting
tract VT,53,54,56,62 (Figure 3) polymorphic VT (PMVT),
Suspicionofinheritedarrhythmiasyndrome,familyhistoryof
inheritedarrhythmiasyndrome includingtorsadesdepointes(TdP),oraninheritedarrhyth-
Electrophysiologicaltesting mia syndrome, such as the long QT, short QT, Brugada, or
Sustainedpalpitationswithoutdiagnosis,suspicionofAVblock, early repolarization syndromes (ERS)4 (Figure 4). Outflow
coronaryarterydiseasewithNSVT,andmoderateLVdysfunction
tract VAs typically have an inferior axis with either RV or
ARVC ¼ arrhythmogenic right ventricular cardiomyopathy; AV ¼ LVorigin.Whentheprecordialtransitionis,V3andtheratio
atrioventricular;HCM¼hypertrophiccardiomyopathy;LV¼leftventricular; oftheR-andS-wavesinleadV2duringPVCsorVTdivided
MRI ¼ magnetic resonance imaging; NSVT ¼ non-sustained ventricular bythisratioduringsinusrhythmexceeds0.6,aLVoutflow
tachycardia;SCD=suddencardiacdeath.
tractoriginisstronglysuggested.InadditiontotheECG,an
echocardiogram to assess the presence or absence of SHD
occurring at an exercise level of 120 – 130 b.p.m.) and is should also be considered for all patients with NSVT. For
cases where SHD is suspected but cannot be definitively
associated with an increased risk of sudden death. The
diagnosed with echocardiography, cardiac MRI may be
underlying mechanism of CPVT is calcium overload
especially useful to confirm the presence or absence of
leading to delayed afterdepolarizations as a result of
myocardialscarorwallmotionabnormalities.Classification
mutations in the genes coding for ryanodine receptor or
of NSVT should be attempted using a scheme similar to
calsequestrinproteins.Non-sustainedventriculartachycar-
dia is a relatively common finding among athletes.67,68 Tables3and4.EvaluationinCHDisdescribedinaseparate
section.
Other causes of NSVT in the absence of SHD include QT
interval prolongation caused by mutations in proteins
regulating repolarizing currents drugs (LQTS) or electro- Treatment
lyte abnormalities. Athletes with NSVT should be eval- Non-sustained ventricular tachycardia inthe absence of
uated for the presence of HCM, a diagnosis which may structural heartdisease
overlap with some degree of LVH as an adaptation to Most short-lasting monomorphic NSVTs originate from
exercise. Because of this challenging distinction, expert the RV or LV outflow tracts (Table 3, Figure 3). These
consultation should be obtained if this diagnosis is sus- arrhythmias only require treatment if they are sympto-
pected.Althoughonlylimiteddataareavailableregarding matic,incessant,orproduceLVdysfunction.Suddendeath
e
1
7
2
Table3 Non-sustainedventriculartachycardiawithapparentnormalheart
Alternative
NSVTclinical Riskofsudden Diagnostic diagnostic Treatmenttobe
presentation ECG cardiacdeath evaluation considerations Treatment considered Keyreferences
TypicalRVOT LBBB,infaxis,axis Veryrare Standard Differentiatefrom Beta-blocker, Catheterablation Latifetal.62
transitionV3–V4 ARVC verapamil,ICdrugs
withsymptoms
TypicalLVOT Inferioraxis, Veryrare Standard RVOTVT Beta-blocker, Catheterablation Latifetal.62
transition,V3 verapamil,ICdrugs
withsymptoms
Idiopathic RBBB,LSaxis Veryrare StandardEPtesting Ischaemicheart Verapamilif Catheterablation Latifetal.62
reentrantLV disease,CM symptomatic
tachycardia
OtherfocalVT Multiple Uncommon Exercisetestingor Ischaemicheart Beta-blockerforthe Catheterablation Latifetal.62
morphologies, catecholamine disease,CM arrhythmia
monomorphic stimulation
Exercise Multiple Increasedriskwhen Ischaemicheart CPVT Underlyingdisease Beta-blockers, Jouvenetal.65,Frolkis
NSVTinrecovery disease, flecainide etal.66
cardiomyopathy
Athlete Multiple Ifitdisappearswith Evaluateforlatent HCM Notreatmenttraining None Biffietal.67,68
increased HCMorischaemic cancontinue
exerciselowrisk heartdisease
Hypertension Multiple Aswithout Considerischaemic Ischaemicheart TreatHTN Beta-blocker
valvulardisease morphology arrhythmia heartdisease disease,CM
PolymorphicVT Polymorphic High EvaluatedforCAD, Purkinjefibre Underlyingdisease Revascularization, Zipesetal.60 H
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ARVC ¼ arrhythmogenic right ventricular cardiomyopathy; CAD ¼ coronary artery disease; CM ¼ cardiomyopathy; HTN ¼ hypertension; CPVT ¼ catecholaminergic polymorphic ventricular tachycardia; o
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Pedersen etal EHRA/HRS/APHRS Expert Consensus on Ventricular Arrhythmias e173
Keyreferences Hohnloser70etal.60Zipesetal. 60Zipesetal.60Zipesetal. 60Zipesetal. 60Zipesetal.60Zipesetal.60Zipesetal. 3Aliotetal. ardiomyopathy; oawisfinvtthoehrnecys-ahertyahardererrteohinrpyytaphrbamitldiaeiitaninosetsnisc.w6ae0ilitctNhihuooemrnu-tmflsbuoelosdwtciacktiarnealrec,wdtciVvtlhaeTsn.astTrbIihcCeeutaltda-rbrreulatogtamcsck,heeyonr-rt,
c cardia with a focal mechanism may also occur from the
c
Treatmenttobeconsidered ICD ICD,seerelevantguidelinesAntiarrhythmicmedicaltherapyorablationwithsymptomsAdditionalantiarrhytmictherapyorablationICD,seerelevantguidelines ICD Quinidine ¼polarisation;HCMhypertrophi trpaCVaeiedertbaaTnelrilpaohtradhitpytolaieilvatanonathtebednrshmlryl.yia5csciatm8ahaib,oh7oNnuli9niasusg,S8tclbhit0VdloeceeIrnaTsnbmrnteeariaicstenndhuabcdadeartoeterftierntefdeidesniorssciecpndwbtceio,eyhovirrntimeeehrigdniedhsmfnttvoklroteyeaerrvnvabroieadsneednnpynettiaamodoL-wmopubVprashilftalotooeV,hlvcrnmieTktctrhaehtpauhtorrepaitesiucritasealigomienpzahrsntyiinurctnl.adss.wa7gnt1tCaiht,fdwti8aaehn1rLltitu,eshte8Vdghea2-r
e
r refractory, especially if it is exercise-induced.
Treatment Beta-blockers Beta-blockers ICDwithinducibleVT/VFICD ICDwithinducibleVT/VF UncertainBeta-blocker,ICDBeta-blocker Withsyncopeorcardiacarrest:ICD ¼ysiologicalstudy;ERearly¼ventriculartachycardia. NsTtadshtusiohrorsuneerttc-caohsttiruuueenegrsdecathpdoalrirehniPtdvmoeeiMaandalrguVrtviyamedTotniipfsttocherrnaoiaepcnvsrfuoaeoillbnpayregrymthtcoaeflccroapphosrrhysreociitsficahnereiandsNdrciyaeSaaVsiornrfpTahpecyCraotsftrhPuhiomesVonniuioaTtalsrnd,y.wtsihphisteIrochfohurmailsdenpkmotbonieaaf-
h
nformias ectropon;VT lthifeer-athpryeawteinthingpotaernrhtiyatlhmpliaaceimsenhtigohf aanndICbDetai-sblorecckoamde-
Diagnosticstobeconsidered Monitoring Continuedevaluatiorepetitivearrhyth Monitoring EPtestingMRI-DE Geneticscreening ¼ophysiology;EPSel¼fiventricularbrillati bebwmleeieetcAanahtddlrratdeoohdrllsoyee.i4usgtfe,sgn8oe3hidrdfiip.sacIrtnnauonrgItbcCrlyaaaDsnmrecemssedheutoohdcufaeletddlTpeLbdcroetPVrlioEccnVoaFgnlTs(ss,,it0rdiem.ae3prnu5oeyl)dla,a8trfm4iioo–zer8nad6taiiitlnochlanespterisaoelhteniomceutneaoltdydsr
instructuralheartdisease thmiaalistationDiagnosticevaluation Coronaryarterydisease ConsiderEPSifmoderateLVdysfunctionEPSNon-drivenbyarrhythmia EPtesting,ischaemiatestingUncertainEcho,MRIGeneticscreeningProvocativetestingProvocativetesting ¼¼athy;EFejectionfraction;EPelectr¼ventricular;MImyocardialinfarction;VF Nomsu4ctwplehhaf0aliSseiarott%ssndirsVhueysdigtlnToaenderptdvcvocdsriohseeouoswtureyrppogtessoceierttgabLroa,ah-runmsifVddaneiNoednpimertadfivSedadyeetbiVtuidnsVdrLresfTeinauVuTlaieltnnnaataslEmbnlectalaFoctiedyseifrtoypnrii.Vinnsmiatrcm,cd4etF(apihuqu0Lpeltasuc%onloVueaiisrtmbmran,EteiwlsltamFlieEauycti.tisPstu,ichhwtNpo0Slaeapen.iioaa4ttcnaiinhriis0toeptfis-i)dInesrdgc.Cirun.8oie6eV7sDts0rnc,ate8wTaoenaM8iiSrmmistnIaitiieIsmhaemlmp,lrdwlyiapraieanhllhvnnaLdarhyoedrtenuViatlnecshtychtEatdtoiim,aroobbmFivricnlilinyeceef--,.
Non-sustainedventriculartachycardiaTable4 ArrhyRiskofsuddenspeciClinicalsettingcardiacdeathevalu ACSwithin48hNoincreasedriskNo ACSafter48hRiskincreasedYes –PreviousMI,EF3140IncreasedriskYesrPreviousMI,EF30IncreasedriskYesChronicheartfailure,rEF30SyncopewithchronicIncreasedriskYes−CAD,EF40Non-ischaemicdilatedCMUncertainYesHCMIncreasedriskYesLQTSIncreasedriskYesShortQTsyndromeIncreasedriskYesBrugadasyndromeIncreasedriskYes ERsyndromeIncreasedriskYes ¼=CADcoronaryarterydisease;CMcardiomyop¼fi¼ICDimplantablecardioverterdebrillator;LVleft tlawmsrryheiynziaviemtanyrehatagprioppbsotuphioyenbtrem,,olcoaeapooartnhtrtpnfiideadtscaircii,tmtdstemhoreeieearzacrcsesajog.uoedtnoir.rosrafrIeinolindnsriekotstprhfaNfoaeatmtpSicisotetVeeinonmdtTrttiissiicnfz.na6wge0NlodiotIttSnhhftrVreegHeSrsTaeaHoCtnpmliMDevysree,a,nwdpolIt,rhCrebAoosyDtfrAeeentrtDxahethtpevemwtearhureasieintcnperhdutyanelpcooairyes-r-f
e174 Heart Rhythm, Vol11, No 10, October 2014
Left Coronary Cusp VT
Figure3 (A)Rightventricular(RV)outflowtractVT.(B)LeftcoronarycuspVT.
II Long QT syndromes
Short QT syndromes
WPW syndrome
Brugada syndrome(s)
ARVC
V1 HCM
Figure4 Electrocardiograms(ECGs)inlongOTsyndrome,shortOTsyndrome,Brugadasyndrome,arrhythmogenicrightventricularcardiomyopathy,
hypertrophiccardiomyopathyWPWsyndrome.
Pedersen etal EHRA/HRS/APHRS Expert Consensus on Ventricular Arrhythmias e175
Sustained monomorphic ventricular tachycardia
Expert consensus recommendations on SMVT
(1) A12-leadECGshouldberecordedduringsustainedVTswheneverpossibleandpractical.ILOEB
(2) ForpatientswithnewlydiagnosedsustainedmonomorphicVT(SMVT)andnoevidenceofSHDonrestingECGorechocardiography
(a) cardiacMRImayprovideadditionalinformationIIb,LOEB
(b) signal-averagedECGmayprovideadditionalinformationIIb.LOEC
(c) exercisetestingmayprovideadditionalinformationIIb.LOEB
(3) ForpatientswithawideQRScomplextachycardiainwhomthediagnosisisuncertain,aninvasiveEPSshouldbeconsideredtoidentify
thetachycardiamechanism.IIaLOEC
(4) ForpatientswithSHDandSMVT,anICDisrecommendedintheabsenceofcontraindications.ILOEA
(5) ForpatientswithSHDandrecurrentSMVT,specifictreatmentofVaswithAADs(amiodarone,mexiletine,orsotalol),catheterablation,
and/orantitachycardiapacing(ATP)fromanICDshouldbeconsideredinadditiontoanICD.TreatmentoftheunderlyingSHDor
ischaemiawillinmostcasesnotbesufficienttopreventmonomorphicVT(MMVT)recurrences.IIaLOEB
(6) ForpatientswithanICDasprimaryprophylaxis,programmingtoalongVTdetectionintervalandahighVFdetectionrateshouldbe
considered.IIaLOEA.
MonomorphicVTisdefinedassustainedwhenlastinglonger of sustained MMVT or VF is less certain. Although it has not
than30sorrequiresearlierinterventionduetohaemodynamic been determined whether suppression of VT by either pharma-
instability.89 Most commonly, sustained MMVT occurs in the cologicalmeansorcatheterablationimprovessurvivalinpatients
setting of diseased myocardium, but may also be idiopathic, withsustainedMMVT,treatmenttoavoidrecurrentsymptomsis
occurringinpatientswithnodetectablemyocardialdisease. appropriateandthesetherapiesmayimprovesurvivalinpatients
presentingwithrecurrentVTstorm.104,105
Importance and prognosis
No structural disease—idiopathic ventricular tachycardia Diagnostic evaluation
In the absence of SHD, SMVT is generally associated with Electrocardiogram
an excellent prognosis.60,90–92 The presence of syncope or ThekeydistinctiontomakeintheinvestigationofSMVTis
PMVT is unusual in the absence of SHD or an inherited to discern the presence or absence of SHD, see Table 2. A
arrhythmia syndrome. Rarely, idiopathic VT can have a 12-lead ECG helps to confirm the diagnosis of VT,106–110
malignantclinicalcourse,usuallywithaveryrapidrateora provide important insight into the underlying mechanism
short initiatingcoupling interval.93 (Tables 3 and 4), identify the presence of SHD,111 and
suggestthesiteoforigin.Thisisespeciallyimportantwhen
catheterablationisplanned.112,113ArestingECGshouldbe
Sustained monomorphic ventricular tachycardiainpatients
performedinallpatientswithsustainedVT.Thepresenceof
with structural heart disease
Q-waves or fragmentation of the QRS complex suggests
The large majority of patients with SMVT who present for underlying structural disease (Figure 5).114,115
therapy have significant SHD. The most frequent aetiology is
ischaemic heart disease, comprising 54 – 59% of patients for
Cardiac imaging
whom an ICD is implanted94 or who are referred for catheter
ablation.92 Sustained VT is associated with increased mortality Thepresenceofmyocardialscarismorelikelytobeassociated
risk in the setting of reduced ventricular systolic function.95–98 withpoorlytoleratedVT,haemodynamiccollapse,degeneration
toVF,andsuddendeath.Inmostcases,echocardiographycan
The mortality risk attributable to VT in patients with preserved
ventricularfunctionislesswelldefined.Implantablecardioverter- adequately demonstrate myocardial structure and function. If
defibrillator shocks are also associated with inherent risk and echocardiography is normal, more detailed imaging using
multiplestudieshavedemonstratedthatdefibrillatorshocks,both cardiac MRI can exclude less clearly evident myocardial scar,
arrhythmogenicRVcardiomyopathy,non-ischaemiccardiomy-
appropriate and inappropriate, are associated with increased
mortalityandreducedqualityoflife.78,99–103Theassociationof opathy with preserved EF, HCM, or cardiac sarcoidosis.116 It
may also be helpful to reevaluate ventricular function when a
ICDshocksandtotalmortalityappearsmainlytobeafunctionof
worseningcardiacdiseaseratherthanaspecificconsequenceof patientwithpreviouslyknownSHDpresentswithSMVT.
shocks. Programming of ICDs with long VT detection times
prior to the delivery of antitachycardia therapies and rapid VF Signal-averaged electrocardiogram
detectionratesreducesshocksandimprovesmortalityinpatients AnSAECG,recordedduringthebaselinerhythmmaypermitthe
receiving an ICD for primary prophylaxis.77 The value of identificationof slow myocardial conductionbyrecordinglow-
programmingalongVTdetectiontimeinpatientswithahistory amplitude potentials but does not help in scar localization.