Europace Advance Access published November 17, 2016 EHRA POSITION PAPER Europace doi:10.1093/europace/euw301 European Heart Rhythm Association (EHRA) consensus document on the management of supraventricular arrhythmias, endorsed by Heart Rhythm Society (HRS), Asia-Pacific Heart Rhythm Society (APHRS), and Sociedad Latinoamericana de Estimulacio´n Cardiaca y Electrofisiologia (SOLAECE) Demosthenes G. Katritsis, (Chair)1, Giuseppe Boriani2, Francisco G. Cosio3, Gerhard Hindricks4, Pierre Ja¨ıs5, Mark E. Josephson6, Roberto Keegan7, Young-Hoon Kim8, Bradley P. Knight9, Karl-Heinz Kuck10, Deirdre A. Lane10,11, D ow GSarkeigsoTrhyeYm. Hist.oLcilpa1k1i,sH15,eKleantahrMyanlmA.bWorgo1o2d,1H6,aaknadnCOarrailn1a3,BClaormlostPrao¨pmp-oLnuen1d4,qvist, nloaded from (Co-Chair)12 by guest on N RGEhVeoIErWge-EARnSd:rBeui Dleannt1G8,oMreanreckA, (Vreovsi1e9w, GcuolomridrianaKtuodra)1ib7,eNrdikieovlaao20s, DHaagrrryesC4,rijns21, ovember 17, 2016 Kurt Roberts-Thomson22, Yenn-Jiang Lin23, Diego Vanegas24, Walter Reyes Caorsi25, Edmond Cronin26, and Jack Rickard27 1AthensEuroclinic,Athens,Greece;andBethIsraelDeaconessMedicalCenter,HarvardMedicalSchool,Boston,MA,USA;2CardiologyDepartment,ModenaUniversityHospital, UniversityofModenaandReggioEmilia,Modena,Italy;3HospitalUniversitarioDeGetafe,Madrid,Spain;4UniversityofLeipzig,Heartcenter,Leipzig,Germany;5UniversityofBordeaux, CHUBordeaux,LIRYC,France;6BerthIsraelDeaconessMedicalCenter,Boston,MA,USA;7HospitalPrivadodelSuryHospitalEspan˜ol,BahiaBlanca,Argentina;8KoreaUniversity MedicalCenter,Seoul,RepublicofKorea;9NorthwesternMemorialHospital,Chicago,IL,USA;10AsklepiosHospitalStGeorg,Hamburg,Germany;11UniversityofBirmingham InstituteofCardiovascularScience,CityHospital,Birmingham,UK;andAalborgThrombosisResearchUnit,DepartmentofClinicalMedicine,AalborgUniversity,Aalborg,Denmark; 12DepartmentofCardiologyandMedicalScience,UppsalaUniversity,Uppsala,Sweden;13UniversityofMichigan,AnnArbor,MI,USA;14IRCCSPoliclinicoSanDonato,SanDonato Milanese,Italy;15Dell’AngeloHosiptal,Venice-Mestre,Italy;16EmoryUniversitySchoolofNursing,Atlanta,USA;17CardiologyDepartment,EskisehirOsmangaziUniversity,Eskisehir, Turkey;18ColentinaUniversityHospital,‘CarolDavila’UniversityofMedicine,Bucharest,Romania;19DepartmentofMedicalPhysiology,DivisionHeartandLungs,UmcUtrecht,The Netherlands;20Adana,Turkey;21MastrichtUniversityMedicalCentre,Cardiology&CARIM,TheNetherlands;22RoyalAdelaideHospital,Adelaide,Australia;23TaipeiVeterans GeneralHospital,Taipei,Taiwan;24HospitalMilitarCentral-UnidaddeElectrofisiolog`ıa-FUNDARRITMIA,Bogota`,Colombia;25CentroCardiovascularCasadeGalicia,Montevideo, Uruguay;26HartfordHospital,Hartford,USA;and27ClevelandClinic,Cleveland,Ohio,USA Table of contents WideQRS(.120ms)Tachycardias......................9 Acutemanagementintheabsenceofanestablisheddiagnosis Abbreviations...........................................2 AcutemanagementofnarrowQRStachycardia........... 14 Preamble/Definitions AcutemanagementofwideQRStachycardia............. 15 Evidencereview.......................................2 Atrialtachycardias Relationshipswithindustryandotherconflicts..............3 Sinustachycardias ................................... 16 Definitionsandclassification.............................3 Focalatrialtachycardias............................... 17 Epidemiology ...........................................3 Macroreentrantatrialtachycardias...................... 20 Clinicalpresentation .....................................4 Atrioventricularjunctionaltachycardias Differentialdiagnosisoftachycardias Atrioventricularnodalreentranttachycardia ............. 25 NarrowQRS(≤120ms)Tachycardias....................5 Non-paroxysmaljunctionaltachycardia.................. 28 PublishedonbehalfoftheEuropeanSocietyofCardiology.Allrightsreserved.&TheAuthor2016.Forpermissionspleaseemail:[email protected]. Page2of47 D.G.Katritsisetal. Focaljunctionaltachycardia ........................... 28 RV: rightventricle Othernon-reentrantvariants.......................... 28 s: seconds Atrioventricularreentranttachycardias SR: sinusrhythm Wolff–Parkinson–White syndrome and atrioventricular re- SVC: superiorvenacava entranttachycardias ............................... 28 SVT: supraventriculartachycardia Concealedandotheraccessorypathways................ 30 VA: ventriculararrhythmia Theasymptomaticpatientwithventricularpre-excitation... 31 WPW: Wolff–Parkinson–White Supraventriculartachycardiainadultcongenitalheartdisease ..32 Supraventriculartachycardiainpregnancy...................34 Healtheconomics ......................................36 Preamble/definitions Patientpreferences .....................................36 Supraventriculararrhythmiasarecommon,andpatientsareoften Areasforfutureresearch ................................37 symptomaticrequiringmanagementwithdrugtherapiesandelec- trophysiologicalprocedures.TheEuropeanSocietyofCardiology publishedmanagementguidelinesforsupraventriculartachycardias Abbreviations (SVT)in2003,1andcorrespondingUSguidelineshavealsobeen published,themostrecentbeingin2015.2 AAD: antiarrhythmicdrugs Thereisaneedtoprovideexpertrecommendationsforprofes- ACHD: adultcongenitalheartdisease sionalsparticipatinginthecareofpatientspresentingwithSVT.In AF: atrialfibrillation addition,severalassociatedconditionswhereSVTsmayco-exist AFL: atrialflutter needtobeexplainedinmoredetail.Toaddressthistopic,aTask ANP: atrialnatriureticpeptide ForcewasconvenedbytheEuropeanHeartRhythmAssociation AP: accessorypathway (EHRA) with representation from the Heart Rhythm Society ASD: atrialseptaldefect (HRS),Asia-PacificHeartRhythmSociety(APHRS),andSociedad D AV: atrioventricular LatinoamericanadeEstimulacio´nCardiacayElectrofisiologia(SO- ow AAVVNN:RT: aattrriioovveennttrriiccuullaarrnnooddealreentranttachycardia LevAidEeCnEc)e,wavitahilatbhlee,reanmdittotopcuobmlisphreahjeoninsitvecolynrseevniseuwsdthoecupmubelnisthoedn nloaded from AABBVTBR::T: aabtturrniioadlvleteancbthrrayicncuaclhradrbiarloeeckntranttachycardia tmheeTnmhdiaasntidaoognecsmufmoernetncoltinfsiSucVamlTmppraaartciizteiencsets.c,uwrirtehnutpd-teov-edlaotpemcoennstesninsutshreecfioemld-, by guest on N bpm: beatsperminute withfocusonnewadvancessincethelastESCguidelines,andpro- ovem CCIL:: ccoycnlfiedleenncgtehinterval vtiiednetssgbeanseedraolnretchoempmrinecnidpaletsioonfsefvoidretnhceem-baanseadgemmeednictinoef.SVTpa- ber 17, 2016 CTI: cavo-tricuspidisthmus Evidence review DC: directcurrent ECG: electrocardiogram MembersoftheTaskForcewereaskedtoperformadetailedlitera- EPS: electrophysiologystudy turereview,weighthestrengthofevidencefororagainstaparticu- ERP: effectiverefractoryperiod lar treatment or procedure, and include estimates of expected HPS: His-Purkinjesystem healthoutcomeswheredataexist.Patient-specificmodifiers,co- HR: heartrate morbidities,andissuesofpatientpreferencethatmightinfluence IV: intravenous thechoiceofparticulartestsortherapiesareconsidered,asarefre- IVC: inferiorvenacava quencyoffollow-upandcost-effectiveness.Incontroversialareas, LA: leftatrium orwithregardtoissueswithoutevidenceotherthanusualclinical LBBB: leftbundlebranchblock practice,aconsensuswasachievedbyagreementoftheexpertpa- LV: leftventricle nelafterthoroughdeliberations.Thisdocumentwaspreparedby MESA: Marshfield(Wisconsin)EpidemiologicStudyArea the Task Force with representation from EHRA, HRS, APHRS, MRT: macroreentranttachycardia andSOLAECE.Thedocumentwaspeer-reviewedbyofficialexter- ms: milliseconds nalreviewersrepresentingEHRA,HRS,APHRS,andSOLAECE. PJRT: permanentjunctionalreciprocatingtachycardia Consensusstatementsareevidence-based,andderivedprimarily POTS: posturalorthostatictachycardiasyndrome frompublisheddata.Currentsystemsofrankinglevelofevidence PPI: post-pacinginterval arebecomingcomplicatedinawaythattheirpracticalutilitymight QALY: quality-adjustedlifeyears becompromised.3Wehave,therefore,optedforaneasierand, QoL: qualityoflife perhaps,moreuser-friendlysystemofrankingthatshouldallow RA: rightatrium physicianstoeasilyassesscurrentstatusofevidenceandconse- RBBB: rightbundlebranchblock quentguidance(Table1).Thus,agreenheartindicatesarecom- RCT: randomizedcontrolledtrials mended/indicatedtreatmentorprocedureandisbasedonatleast RF: radiofrequency one randomized trial, or is supported by strong observational EHRAconsensusdocumentonthemanagementofSVT Page3of47 Table1 Scientificrationaleofrecommendations Table2 Conventionalclassificationofsupraventricular tachycardias Scientificevidencethatatreatmentor Recommended/ procedureisbeneficialandeffective. indicated Atrialtachycardias Requiresatleastonerandomized Sinustachycardia trial,orissupportedbystrong Physiologicalsinustachycardia observationalevidenceandauthors’ consensus. Inappropriatesinustachycardia Generalagreementand/orscientific Maybeusedor Sinusnodereentranttachycardia evidencefavourtheusefulness/ recommended Atrialtachycardia efficacyofatreatmentorprocedure. Focalatrialtachycardia Maybesupportedbyrandomized Multifocalatrialtachycardia trialsthatare,however,basedon Macro-reentranttachycardia smallnumberofpatientstoallowa greenheartrecommendation. Cavotricuspidisthmus-dependent,counter-clockwiseor clockwise(typicalatrialflutter) Scientificevidenceorgeneral ShouldNOTbeused agreementnottouseorrecommend orrecommended Noncavotricuspidisthmus–dependent,mitral atreatmentorprocedure. isthmus-dependent,andotheratypicalleftorrightatrialflutters Atrioventricularjunctionaltachycardias Thiscategorizationforourconsensusdocumentshouldnotbeconsideredasbeing Atrioventricularnodalreentranttachycardia directlysimilartothatusedforofficialsocietyguidelinerecommendationswhich Typical applyaclassification(I–III)andlevelofevidence(A,B,andC)torecommendations. Atypical Non-reentrantjunctionaltachycardia Non-paroxysmaljunctionaltachycardia evidencethatitisbeneficialandeffective.Ayellowheartindicates Focaljunctionaltachycardia thatgeneralagreementand/orscientificevidencefavourtheuseful- Othernon-reentrantvariants D ness/efficacyofatreatmentorprocedure.Maybesupportedbyran- Atrioventriculartachycardias ow dtoomallizoewdatrgiarlesethnahteaarret,hreocwoemvemre,bnadsaetdioonn.Tsmreaaltlmnuemntbsetrraotfepgaietisenfotrs AtrOiorvthenotdrricoumlaicrreentranttachycardia nloaded from whpaehraimnchfHutlehaaenrrdteRshhhaoysutbhldmeennAotssscboieecnuiatsitfieiodcneargveridaiednnidncigceaottehfdactobtnyhsaeeynresaudrsehseptaoartttee.mnEtueiarnlotlys- Aranretildyr,othmriocu(gwhitahnroetthreorgrpaadtehwcoany)ductionthroughtheAVnodeor, by guest on N doesnothaveseparatedefinitionsofLevelofEvidence.Thecat- ovem etaopgoptrlhyiazatatucioslaensdsuisfifoecrdatosiohffinocui(alIdl–snIoIoI)ctiaebnteydcgloeuvnideseildloienfreeedrveidacseonbmcemeine(gAndd,iraBet,icoatnnlysdswCimh)iilctaohr rwhiyththamQ(TRaSbldeu2r)a.tTiohnet≤er1m20nmarsr.oAw-wQidReS-tQacRhSyctaacrdhiyacianrddiicaatreesfetrhsotsoe ber 17, 2016 recommendations. onewithaQRSduration .120ms(Table3).Inclinicalpractice, Overall,thisisaconsensusdocumentthatincludesevidenceand SVTmaypresentasnarrow-orwide-QRStachycardias,andmost expertopinionsfromseveralcountries.Thepharmacologicand ofthemusually,althoughnotinvariably,manifestasregularrhythms. non-pharmacologic antiarrhythmic approaches discussed may, Thispositionpaperdoesnotcoveratrialfibrillation,whichisthe therefore,includedrugsthatdonothavetheapprovalofgovern- subjectofaseparateclinicalguideline,aswellasvariousconsensus mentalregulatoryagenciesinallcountries. documents. Relationships with industry and other Epidemiology conflicts ItisEHRA/ESCpolicytosponsorpositionpapersandguidelines Supraventriculararrhythmiasarerelativelycommon,butrarelylife withoutcommercialsupport,andallmembersvolunteeredtheir threatening.However,precisedescriptionoftheepidemiologyof time.Thus,allmembersofthewritinggroupaswellasreviewers SVTisdifficultasthereisoftenpoordistinctionbetweenAF,atrial have disclosed anypotential conflictof interest in detail, atthe flutter(AFL)andothersupraventriculararrhythmias.Incontrastto endofthisdocument. theextensiveepidemiologyonAF,specificfocusonSVTpopulation epidemiologyissparse. Definitions and classification Ina3.5%sampleofmedicalrecordsintheMarshfield(Wisconsin) Thetermsupraventricularliterallyindicatestachycardias(atrialand/ EpidemiologicStudyArea(MESA)theprevalenceofparoxysmal orventricularrates.100bpmatrest),themechanismofwhichin- SVTwas 2.25/1000 persons, and the incidencewas 35/100000 volvestissuefromtheHisbundleorabove.1,2Traditionally,SVThas person-years.4Basedontheseolddata,itwasestimatedthatthere beenusedtodescribeallkindsoftachycardiasapartfromventricu- are(cid:3)89000newcasesperyear,and570000patientswithparox- lartachycardiasandatrialfibrillation(AF)andhas,therefore,in- ysmalSVTintheUSA. cluded tachycardias such as atrioventricular reentry due to TheprecipitantsofSVTcanberelatedtoage,sex,andassociated accessoryconnectionsthatisnot,inessence,asupraventricular comorbidities.Thus,inthegeneralpopulationappeartobetwo Page4of47 D.G.Katritsisetal. pattern on ECG tracings in the general population is 0.1% to Table3 DifferentialdiagnosisofnarrowandwideQRS 0.3%. However, not all patients with manifest ventricular pre- tachycardias excitationdevelopparoxysmalSVT.6,8Infemale,middle-agedor NarrowQRS(≤120ms)tachycardias older persons, atrioventricular nodal reentrant tachycardia (AVNRT)ismorecommon.Inyoungersubjects(e.g.adolescents), Regular theprevalencemaybemorebalancedbetweenatrioventricularre- Physiologicalsinustachycardia entranttachycardia(AVRT)andAVNRT.Porteretal.described Inappropriatesinustachycardia 1754patientsundergoing catheterablation,whereAVNRTwas Sinusnodalreentranttachycardia themostcommon(56%)aetiology,followedbyAVRT27%,andat- Focalatrialtachycardia rialtachycardia(AT)17%.9TheproportionofAVRTdecreasedwith Atrialflutter age,whereastheproportionofAVNRTandATincreased.Mostpa- Atrialfibrillationwithveryfastventricularresponse tientswithAVRTweremale(55%),incontrast topatientswith Atrioventricularnodalreentranttachycardia AVNRTandATwhowerepredominantlyfemale(70%and62%,re- Non-paroxysmalorfocaljunctionaltachycardia spectively).Recently,inthefirstLatinAmericanregistryoncatheter Orthodromicatrioventricularreentranttachycardia ablationincluding15099proceduresfrom120centresin13partici- Idiopathicventriculartachycardia(especiallyhighseptalVT) pating countries,AVRTwasthegroup ofarrhythmiasmostfre- Irregular quently ablated (31%), followed by AVNRT (29%), typical AFL Atrialfibrillation (14%),andAF(11%).10 AtrialfocaltachycardiaoratrialflutterwithvaryingAVblock Limitedpopulationdataonothersupraventriculararrhythmias Multifocalatrialtachycardia (apartfromAF)areavailable.ForAFL,onereportfromMESAof WideQRS(.120ms)tachycardias 181newcases ofAFLestimated anoverallincidence of88/100 Regular 000person-years.11Incidenceratesrangedfrom5/100000inthose Antidromicatrioventricularreentranttachycardia ,50yearsoldto587/100000insubjectsolderthan80years.Atrial Anyregularatrialorjunctionalreentranttachycardiaswith: flutterwas2.5-foldmorecommoninmen,3.5-foldmorecommon aberration/bundlebranchblock D insubjectswithheartfailure,and1.9-foldmorecommoninsubjects ow IrreVgeupnlrtaerri-ceuxlacritattaicohny/cbayrsdtaian/dfleurttaecrcessorypathway wweitrheclhabroenlleicdoabsshtrauvcintgiv‘elopnuelmAoFLn’a.rIyndMisEeSaAse,.AOFLnlhya3dsaunbijneccitdse(n1c.7e%o)f nloaded from AcAotnrntiidadlurficotbmerdiilclwaattiitorhinoavoberenratrrtarictiauiollantarcrheyecnatrrdainatwtaitchhyvcaarrydiniagwbliothckavariable 02m.00o09r%e00ca0onnmdem5w8oc%nasionefsmtohefenA,pFtaLhtieiennettlhdseearlUslyoSaAhnadadninnAudFai.vllTiydh,uteahslesewadrairtthhaypttrhraemn-eisalxabitseetiinntogg by guest on N VAconduction heartfailureorchronicobstructivelungdisease. ovem PProely-emxocritpehdicAVFT Clinical presentation ber 17, 2016 Torsadedepointes Ventricularfibrillation TheclinicalpresentationofSVTusuallyreflectsseveralfactorssuch asheartratewhichcanbequitevariabledependingonage,blood pressureduringthearrhythmiaandresultantorganperfusion,asso- ciatedcomorbidities,andtheindividualpatientsymptomthreshold distinctsubsetsofpatientswithparoxysmalSVT:thosewithother (Table 4). Some patients with paroxysmal arrhythmias may be cardiovascular disease and those with lone paroxysmal SVT. In asymptomatic(orminimallysymptomatic)attimeofevaluation. MESA,othercardiovasculardiseasewaspresentin90%ofmales, Otherpatientscanpresentwithavarietyofsymptoms,even,rarely, and48%offemales.Overall,femaleshad2-foldgreaterrelativerisk (RR)ofparoxysmalSVTcomparedwithmales.Comparedwithpa- tientswithothercardiovasculardisease,thosewithloneparoxysmal Table4 Mostcommonsymptomsduringsustained SVTwereyounger(mean37vs.69years),hadafasterparoxysmal SVT SVTheartrate(mean186vs.155beats/min)andweremorelikely tohavetheirconditionfirstdocumentedintheemergencyroom Common Uncommon Rare (69%vs.30%).Theonsetofsymptomsoccurredduringthechildbear- ................................................................................ ingyearsin58%offemaleswithloneparoxysmalSVT,vs.9%offe- Chestdiscomfortor Chestpain Asymptomatic males with other cardiovascular disease. Older individuals (age pressure .65years)had .5-foldriskofdevelopingparoxysmalSVTcom- Dyspnoea Diaphoresis Tachycardiomyopathy paredtoyoungersubjects.4Datafromspecializedcentresreporting Lightheadedness, Nausea SuddendeathwithWPW dizziness,or syndrome onparoxysmalSVTpatientsreferredforelectrophysiologyproce- presyncope dures,indicatethatpatientstendtobeyounger,havesimilaragedis- Palpitations Syncope tribution,andlowerprevalenceofcardiovascularcomorbidities.5–7 Polyuria TheprevalenceofSVTmediatedbyanaccessorypathwayde- creaseswithage.Forexample,manifestpre-excitationorWPW EHRAconsensusdocumentonthemanagementofSVT Page5of47 imitatingpanicdisorders.12Thus,SVTmayhaveaheterogeneous activationoftheHisbundlecanalsooccurinhighseptalventricular clinicalpresentation,mostoftenoccurringintheabsenceofheart tachycardiasthusresultinginnarrowQRScomplexes.Aclassifica- diseaseinyoungerindividuals(‘loneSVT’).Acarefulclinicalhistory tionofnarrowQRStachycardiasisshowninTable3. shouldincludedescriptionofthearrhythmiapatternintermsof numberofepisodes,duration,frequency,modeofonset,andpos- 12-leadECGinsinusrhythm sibletriggers.Irregularpalpitationsmaybeassociatedwithprema- IntheabsenceofECGrecordedduringthetachycardia,anECGin ture depolarizations, AF, or multifocal atrial tachycardia. SRmayprovidecluesforthediagnosisofSVTandshouldbescru- ParoxysmalAFisoftenasymptomaticwhilstparoxysmalSVTisusu- tinizedforanyabnormality.Baselineintra-atrialconductiondelayisa allysymptomatic,13althoughsymptomsmaybeminimaland,oncer- findingwhichsuggestsatrialreentry,butdoesnotruleoutother tainoccasions,prolongedasymptomaticepisodesmayleadtoa SVTs. tachycardiomyopathy.14 Thepresenceofpre-excitationinapatientwithahistoryofregu- Gradualincreaseinheartrateissuggestiveoffocalatrialtachycar- larparoxysmalpalpitationsisstronglysuggestiveofAVRT,whileir- diaorsinusnode(physiologicalorinappropriate)tachycardia.Typ- regularpalpitationsaresuggestiveofAF.Theabsenceofapparent ically,theseformsofSVThaveshorterdurationbutepisodesmay pre-excitationdoesnotruleoutthediagnosisofAVRT,sinceit occurfrequently(oftendailyorweeklyepisodes).IftheSVTsymp- may be due to a more common concealed accessory pathway tomsaresuggestiveofregularandparoxysmalpalpitationswitha (AP)thatconductsonlyretrogradely. suddenonsetandtermination,thesemostcommonlyresultfrom AVRTorAVNRT;sinusnodereentryislesscommon.Episodesof 12-leadECGduringtachycardia AVNRTorAVRTtypicallyarelongerbutlessfrequent(weeklyor AnECGtakenduringtachycardiaisofkeyimportancefortheproper monthly)comparedtoAT.Usuallythesymptomsareabruptorra- diagnosisofSVT,althoughitmayfailtoleadtoaspecificdiagnosis.17It pidinonset,butmayvarydependingonthespecificarrhythmia.Pa- iseasilyobtainedinmostcases,butnotalwaysavailableinpatients tients with underlying cardiovascular comorbidities such as withveryshortorinfrequentperiodsofpalpitations.Figure1demon- ischaemicheartdisease,cardiomyopathyorvalvularheartdisease stratesthecircuitsand12-leadECGsofdifferenttypesofSVT. (withorwithout heartfailure), are more likely to presentwith D breathlessnessorchestdiscomfort/pain,particularlyatfastheart Initiationandterminationofthetachycardia ow ratTees,rsmuicnhataiosn.b1y5v0abgpalmm.anoeuvresfurthersuggestsare-entrant Safutderdeannpatrroialolnegcatotipoincobefatth.eAPnRatinritaelrtvaaclhoyccacrudrisai(nAtTy)pimcaalyAaVlsNoRbTe nloaded from tAtaaVicnNheydRcTasru,dpAirVaaRvieTnnv)t.orPlicvouilnylagurraiaatrrrmihoyavtyehbnmetiraia,curseylamlartpe(tdoAmVto)stuhnpeopdroaerllteiatvisesesouofefaa(steru.isag-l. iPenrRiatiptairtoeondlo(bnwygaaatrnimoan-t.urAipaulpteohcmetonapotiicmcAbeTenasotna,r)be,ucathnisadrnatochtteednreizpdeeedncbdeyelengrrtaaotdinouanmlaa(ccrckoeeold-l by guest on N natriureticpeptideinresponsetoincreasedatrialpressures.This down).PrematureatrialorventricularbeatsmaytriggerAVRT.Pre- ovem itsomaotsatchpyrcoanroduian-cmeeddiinatAeVdNcaRrTd.i1o5mByroepatahthleyssifnethssemunatyrebaeterdelaStVeTd mbuatturarerevlyenintrdiucucelartybpeicaatslAarVeNaRcTo,manmdoonntlryigegxecreopftiaotnyaplilcyaAlATV.NRT, ber 17, 2016 persistsforweekstomonthswithafastventricularresponse,lead- ingtodilationandimpairedleftventricularfunction.14Syncopemay Regularityoftachycardiacyclelength bepresentinupto20%ofpatientspresentingwithanarrowQRS TheregularityoftheRRintervalshouldbeassessed(Figure2).Ir- complextachycardia.16Inmostinstances,theheartrateisnotsora- regulartachycardiasmayrepresentfocalormultifocalAT,AFand pidastoimpairventricularfunctionandcardiacoutput.ASVTwith AFLwithvaryingAVconduction.Patternsofirregularitycansome- averyrapidventricularrate(e.g..250bpm)mightbeseeninper- timesbefound,suchasinAFLorinthecaseofWenckebachphe- sonswithanaccessorypathwayor1:1conductingatrialflutterlead- nomena. Irregular arrhythmias, such as multifocal AT, typically ingtoreducedcardiacoutputandsyncope.Apresentationwith displayvariablePwavemorphologies,andvaryingPP,RR,andPRin- syncopemayalsosuggest concomitantstructuralabnormalities, tervals.AtrialfluttercanhavefixedAVconductionandpresentasa forexample,valvularaorticstenosis,hypertrophiccardiomyopathy, regulartachycardia,andevenAFcanbealmostregularwhenvery etc.Thus,thepresenceofassociatedcardiacdiseaseshouldbepart fast.TheQRSregularityprovidessomeinformationwithrespect oftheinitialpatientevaluation,andanechocardiogramisrecom- tothetachycardiamechanism,buthassignificantlimitationsdue mended.Physicalexaminationduringtachycardiausuallydoesnot tovaryingAVconductionandduetothefactthattheventricles leadtoadefinitivediagnosis,butitmayprovidesignificantcluesto- maynotbepartofthearrhythmiacircuit.MultifocalATsarevery wardthediagnosisofassociatedheartdiseaseorheartfailure. rarebutirregularATsarenot.Whentheymanifestasverydynamic arrhythmiasandevenstartandstopfrequently,theyareusuallydue Differential diagnosis of tofocalmechanisms.Incessanttachycardiasmayalsobetheso- calledpermanentjunctionalreciprocatingtachycardia,and,rarely, tachycardias atypicalAVNRT(seethesections‘atrioventricularjunctionaltachy- cardias’and‘atrioventricularreentranttachycardias’).Reentrantta- Narrow QRS (≤120 ms) tachycardias chycardias,whethermicro-ormacro-reentries,areneverirregular. NarrowQRScomplexesareduetorapidactivationoftheventricles Iftheirregularitydoesnotexceed15%oftheCL,areentryispos- viatheHis-Purkinjesystem,whichsuggeststhattheoriginofthear- sible,whileabovethisthreshold,afocalarrhythmiaismuchmore rhythmia is above or within the His bundle. However, early likely.18 Page6of47 D.G.Katritsisetal. Atrial tachycardia Atrial flutter Typical AVNRT Orthodromic AVRT I II III aVR aVL aVF V 1 V 2 D VVV43 ownloaded from V65 by guest on N cFairgduiar;eAV1RTT,aacthryiocvarednitaricciurclauritreanednttryapnitcatalc1h2y-cleaarddiEa;CAGPs,ainccdeifsfseoreryntptaytphewsaoy.fnarrow-QRSSVT.AVNRT,atrioventricularnodalreentranttachy- ovember 17, 2016 AchangeinventricularCLprecededbythechangeintheatrial Pwavessimilartothoseinnormalsinusrhythmsuggestappropri- CLisseeninAToratypicalAVNRT.AchangeinventricularCLpre- ateorinappropriatesinusnodaltachycardia,sinusnodalreentrant cedingthechangeinsubsequentatrialCLfavourstypicalAVNRTor tachycardia,orATarisingclosetothesinusnode.Pwavesdifferent AVRT.19AfixedVAintervalinthepresenceofvariableRRintervals fromthoseinsinusrhythmandconductedwithaPRintervalequal excludesAT.QRSalternansisararephenomenoninslowSVTsand toorlongerthanthePRinsinusrhythmaretypicallyseeninAT.In suggestsAVRTasthelikelydiagnosis.However,thisisacommon thiscase,themorphologyofthePwavecanalsoprovidecluesasto findinginanyfastSVT. thelocationoftheATfocus.InAT,theQRScanberegularorir- regular,andtheconductiontotheventriclesfast(1:1)orslow,(3 P/QRSrelationship or4:1).ThepossibilityofAFLwith2:1conductionshouldalsobe AccordingtotheirP/QRSrelationships,SVTsareclassifiedashaving considerediftheventricularrateduringSVTis (cid:3)150bpm,since shortorlongRPintervals.AveryshortRPinterval(,70ms)rules theatrialactivityisusually300bpm.Inthepresenceofantiarrhyth- outAVRTandindicatestypicalAVNRTor,lesscommonlyAT,asthe micmedication,loweratrialratesmayalsoresultinlowerventricu- mostlikelydiagnosis.The70mscut-offintervalisbasedonVAin- larrates.Thediagnosismaybeestablishedbyconsideringtheatrial tervalsthathavebeenexaminedinelectrophysiologystudies.An activity.ThePwavesareusuallywellseen(inabsenceoffastcon- cut-offintervalof90mshasbeenshowntobeusefulforsurface ductiontotheventricles)andaremonomorphicandregular. ECGmeasurementsandcanbeusedifPwavesarevisible,20but Incaseofrelativelydelayedretrogradeconductionthatallowsthe dataonactualRPmeasurementduringvarioustypesofSVTare identificationofretrogradePwaves,apseudo-Rdeflectioninlead scarce. V1andapseudoSwaveintheinferiorleadsaremorecommon ShortRPSVTsarethosewithRPintervalsshorterthanhalfthe intypicalAVNRTratherthaninAVRTduetoanaccessorypathway tachycardiaRRinterval,whereaslongRPSVTsdisplayRP.PR(Fig- oratrialtachycardia.22,23Thesecriteriaarespecific(91–100%)but ure2).Rarely,recordingofUwavesduringtypicalAVNRTmay modestlysensitive(58–14%).22AdifferenceofRPintervalsinlead simulatealongRPtachycardia.21 V1andIII.20msisalsoindicativeofAVNRTratherthanAVRTdue EHRAconsensusdocumentonthemanagementofSVT Page7of47 Narrow QRS tachycardia (QRS≤120 ms) Regular tachycardia Yes No Atrial fibrillation No Visible P Atrial tachycardia/flutter with waves? variable AV conduction Multifocal atrial tachycardia Yes Atrial rate greater than ventricular rate? Yes No D ow AtrAiatAlr tViaaNlc fhRluyTtctaaerrdia Vegnraettrariictaeul rlr aathrt earante nloaded from ConsideNro YFeoscal jHunigchti osneaplt atal cVhTycardia by guest on Novem RP interval Nodo-ventricular/AfaVsNcRicTualar-nodal reentrya ber 17, 2016 Short (RP<PR) Long (RP≥PR) RP≤70 msb RP>70 msb Orhodromic AVRT Typical AVNRT Atypical AVNRT Atrial tachycardia Atrial tachycardia Atrial tachycardia AVRT Focal junctional tachycardiac Non-paroxysmal Atypical AVNRT junctional tachycardiad Figure2 DifferentialdiagnosisofnarrowQRStachycardia.aRarecauses.bArbitrarynumberbasedontheVAintervalforwhichdataexist.An intervalof90msmayalsobeusedforsurfaceECGmeasurementsifPwavesarevisible.cItmayalsopresentwithAVdissociation.dItmayalso presentwithashortRP.AVNRT,atrioventricularnodalreentranttachycardia;AVRT,atrioventricularreentranttachycardia;AP,accessory pathway. Page8of47 D.G.Katritsisetal. toaposteroseptalpathway.23ThepresenceofaQRSnotchinlead parts of the circuit. The development of bundle branch block aVLhasalsobeenfoundasareliablecriterionsuggestingAVNRT,24 (BBB)duringSVTmayalsobehelpfulinthediagnosisofAVRT.Bun- whileapseudo-RinaVRwasshowntohavehighersensitivityand dlebranchblockipsilateraltotheAPcanresultinCLprolongation specificitythanapseudo-RinV1.25However,inallofthesestudies, duetoVAprolongation,astheventriculararmofthecircuitispro- casesofatrialtachycardiaoratypicalAVNRTwerelimitedorentire- longedbyconductionthroughtheinterventricularseptumfromthe lyabsent.Thus,electrocardiographiccriteriamayprovidecluessug- conductingHisbundlebranch. gestiveoftypical,slow-fastAVNRT,butare oflimited valuefor appropriatedifferentialdiagnosis. Vagalmanoeuvresandadenosine AVblockordissociationduringnarrow-QRStachycardiaisnot Vagalmanoeuvres,suchascarotidsinusmassageoradenosineinjec- oftenseen,butitrulesoutAVRT,asbothatriaandventriclesare tion,maybeofgreathelpinclinicaldiagnosis,particularlywhenever theECGduringtachycardiaisunclear.Possibleresponsestovagal manoeuvresareshowninTable5. Figure3showsthedifferenttypeofresponsetoadenosine.Ter- Table5 Possibleresponsesofnarrow-QRS minationofthearrhythmiawithaPwaveafterthelastQRScomplex tachycardiastovagalmanoeuvres is very unlikely in AT, and most common in AVRT and typical 1.SlowingofAVNconductionandAVNblock.Atrialelectricalactivity AVNRT. Termination with a QRS complex is often seen in AT, canthusbeunmasked,revealingPwavesorunderlyingatrialflutter andpossiblyinatypicalAVNRT,andAVRT.FascicularVTs,inpar- oratrialfibrillationwaves. ticular,areverapamilbutnotadenosine-sensitive.MostVTs,asop- 2.Temporarydecreaseintheatrialrateofautomatictachycardias(AT posedtoSVTs,donotrespondtocarotidsinusmassage,althougha orsinustachycardia). narrow-QRSVToriginatingfromtheleftbundlebranch,andtermi- 3.Tachycardiatermination.Thiscanhappenbyinterruptingthereentry natedwithcarotidsinusmassagehasbeenreported.26 circuitinAVNRTandAVRTbyactingontheAVNthatispartofthe circuit.Morerarely,ATsduetotriggeredactivitycanslowdownand Electrophysiologystudy terminate. Severalelectrophysiologytechniquesandmanoeuvrescanbeem- 4.Noeffectisobservedinsomecases. ployedintheelectrophysiologylaboratoryfordifferentialdiagnosis Dow ofregular,narrow-QRStachycardias.Asummaryispresentedin nloaded from Regular narrow QRS by guest on N tachycardia ovem ber 17, 2016 IV adenosine Persisting atrial Gradual slowing then tachycardia with No change in rate Sudden termination reacceleration of rate transient high-grade AV block Sinus tachycardia AVNRT Automatic AT AVRT Atrial flutter Inadequate dose/delivery Nonparoxysmal junctional Sinus node reentry Macro (micro)-reentrant AT tachycardia Triggered AT (DADs) Figure3 Responsesofnarrowcomplextachycardiastoadenosine.AVNRT,atrioventricularnodalreciprocatingtachycardia;AVRT,atrioven- tricularreciprocatingtachycardia;AT,atrialtachycardia;AV,atrioventricular;IV,intravenous;DAD,delayedafterdepolarization;VT,ventricular tachycardia. EHRAconsensusdocumentonthemanagementofSVT Page9of47 Table6 Electrophysiologytechniquesforthedifferentialdiagnosisofnarrow-QRStachycardias VpacinginSR Vpacingduringtachycardia ApacinginSR Apacingduring tachycardia ............................................................................................................................................................................... VAratiosduringVpacing27,28 His-synchronousextra-stimuli29 ComparisonofAHduringpacing andtachycardia30 Ventriculoatrialindex31 Overdrivepacing – AAV/AAHVresponse32 – Withandwithoutstablefusion (entrainment)33 – EntrainmentwithSA-VAand cPPI-TCLintervals34–37 – Differentialentrainmentor cessation38 DHAduringVpacingandtachycardia39 Pre-excitationindex40 Differentialentrainment41 VHApattern42 Entrainment – AnterogradeHiscapture43 – Progressivefusionduringorafterthe transitionzone44,45 – DHAduringentrainmentand tachycardia46 – Para-Hisianentrainment47–49 Parahisianpacing50 InductionofretrogradeRBBB51 SA -VAandcPPI -TCLintervalsduring init init inductionoftachycardia52 D ow PAr,oactreidalu;rHe,sHwisit;hcPtePxI,tcionrriteaclitcesdapreosctlpaassciifinegdinatsercvuaml;RbeBrBsBo,mrieg,hwtbhuilendtlheobsreasnhcohwbnloicnk;nSoArm,satilmteuxlutsatroeaetarsiuilmyaipnptelircvaabl;leS.R,sinusrhythm;TCL,tachycardiacyclelength;V,ventricular; nloaded from VMAo,dvifieendtrifcruolmo-aKtartiarilt.sisandJosephson.17 by guest on N ovem Tthaibslpeap6e,r.b17u,2t7–a52detailed discussion is beyond the scope of tBhBeBvmenotrrpichuollaorgmiesyodcuarrindgiuSmV.T54,t5h5aTthmeiymcicasnVbTo.thresultinatypical ber 17, 2016 12-leadECGinsinusrhythm Wide-QRS (>120 ms) tachycardias ComparisonoftheQRSmorphologyduringnormalsinusrhythm Wide-QRStachycardiascanbeventriculartachycardias,andsupra- andwide-QRStachycardiaishelpful,andthepresenceofcontralat- ventriculartachycardiasconductingwithbundle-branchblockaber- eralBBBinwide-QRStachycardiaandsinusrhythmstronglyfavours ration,oroveranaccessorypathway,withareportedproportionof VT. However, identical QRS morphology during normal sinus 80%,15%and5%,respectively(Figure4).53Functionalrightbundle rhythmandwide-QRStachycardia,althoughstronglysuggestiveof branchblock(RBBB)occursmorefrequentlythanfunctionalleft SVT,canalsooccurinbundlebranchreentrantandhighseptalVTs. bundlebranchblock(LBBB)becauseofthelongerrefractoriness oftheformer.BundlebranchblockcanoccurwithanySVT.Arate- 12-leadECGduringtachycardia relatedBBBcandevelopalsoduringorthodromicAVRT,andtachy- Atrioventriculardissociation cardiaratemayslowiftheBBBisipsilateraltotheaccessorypath- AVdissociationisoneofthemostimportantECGcriteriaforthe waylocation.Anaccessorypathwaymayparticipateinthecircuit diagnosisofVT.Therelationbetweenatrialandventricularevents (antidromicAVRT),orbeaby-standerduringatrialtachycardia, is1:1orgreater(moreatrialthanventricularbeats)inmostcasesof AFL,AF,andAVNRT.SVTwithwideningofQRSintervalcanbe SVT(Figure2).Althoughventriculo-atrialconductioncanbefound alsoinducedbydrugorelectrolytedisturbances.Pacemaker-related inupto50%ofpatientswithVTanda1:1relationispossible,most endlesslooptachycardiaandartefactsthatmimicVTcanleadto ofVTshavearelationlessthan1:1(moreQRScomplexesthan misdiagnosisofawide-QRStachycardia. Pwaves). Differentialdiagnosisshouldalwaysbeconsideredinthecontext AVdissociationischaracterizedbyatrialactivitythatiscomplete- oftheunderlyingdisease,withconditionssuchasmyocardialinfarc- ly independentofventricularactivity.This phenomenoncanbe tion,congestiveheartfailure,andrecentanginapectorisfavouring documentedontheECGasPwavesdissociatedfromtheventricu- VT.53Antiarrhythmicdrugtherapyisalsoimportant.ClassIcand larrhythmduetofusionorcapturebeats(Figure5).Atrioventricular Iadrugscauseuse-dependentslowingofconduction,andclassIII dissociationmaybedifficulttorecognizebecausePwavesareoften drugsprolongrefractorinessatHis-Purkinjetissuemorethanin hiddenbywideQRSandTwavesduringawide-QRStachycardia. Page10of47 D.G.Katritsisetal. Concordantnegativity Thecoincidenceofallpositiveornegative(‘concordant’)QRScom- Atypical AVNRT Antidromic AVRT with LBBB aberration due to atriofascicular AP plexesinallprecordialleadsissuggestiveofVT.Whilepositivecon- cordancemayoccurduringantidromicSVTusingaleftposterioror leftlateralaccessorypathwayanegativeconcordanceisnearlyal- ways VT.53 This pattern suggests VT with a specificity of more than90%,but this criterionhaslow sensitivitybeing present in ,20%ofallVTs. Rightbundlebranchblockmorphology LeadV :Therightbundlebranchdoesnotcontributegreatlytothe 1 I initialpartofthenormalQRS;thus,whenblocked,thefirstpart oftheQRSissubstantiallyunchanged.Thisleadstotypicalpat- II ternsofRBBBaberrancythatincluderSR′,rSr′,orrR′ inlead III V .Onthecontrary,amonophasicR,Rsr′,biphasicqRcomplex, 1 orbroadR(morethan40milliseconds)inleadV favoursVT. 1 aVR Additionally,adouble-peakedRwaveinleadV favoursVTif 1 aVL theleftpeakistallerthantherightpeak(theso-calledrabbit earsign).AtallerrightrabbitearcharacterizestheRBBBaber- aVF rancybutdoesnotexcludeVT.57 V1 LeadV6:Asmallamountofnormalrightventricularvoltageisdirec- tedawayfromV .BecausethisisasmallvectorinRBBBaber- 6 V2 rancy, the R:S ratio is .1. In VT, all of the RV voltage, and someoftheleft,isdirectedawayfromV ,leadingtoanR:Sratio V3 ,1(rS,QSpatterns).ARBBBmorpholo6gywithaR:SratioinV Dow VV4 othfelepsasttiehnatnh1asisaseleefntarxariseldyeivniaStVioTnwduitrhinagbseirnruasncrhy,ytmhamin.5ly6when6 nloaded from ecFinicgtutulayrpreepsa4othfTwwaaicdyheyu-cQsaurRadlSliyaScpVirrToc.udAiutncaVteind65sdraotymhpoiiccraiAzloV1nR2t-TaleldaoudreEstCuopGaensriiaontrrdioQifffeaRsrS-- LLLeeefaasdtddtibrcVVuot61onk::dreITslneohotfberftruhaVpenerTcLe.Sh5Bs8ewBbnlBaocv,ceekn,oomafnQobdrrpwdoheaaodlvlaoeygReiysdwpnaravedese,irnsoltufirnSretwhdaeovlerataneroreatlcsphtrreoedncgodorpdwriean-l by guest on November 17, 2016 axis,butnormalaxismayalsooccur,dependingonthewayofin- leads.Therefore,thepresenceofanyQorQSwaveinleadV6 sertionintotherightbundleandfusionovertheleftanteriorfas- favoursVT.56 cicle.AVNRT,atrioventricularnodalreentranttachycardia;AVRT, atrioventricularreentranttachycardia;AP,accessorypathway; Themorphologycriteriaarenotfulfilledinanyleadin4%ofSVTs LBBB,leftbundlebranchreentry. and6%ofVTs,andinathirdofcaseswhenonelead(V orV )fa- 1 6 voursonediagnosis,theotheronefavourstheoppositediagnosis (VTinoneleadandSVTtheotheroneandviceversa).59 Pwavesareusuallymoreprominentininferiorleads,andmodified Algorithmsfordifferentialdiagnosis chestleadplacement(Lewislead).53 RSintervalinprecordialleads QRSduration TheabsenceofRScomplexinprecordialleads(onlyRandScom- AQRSduration.140mswithRBBBor.160mswithLBBBpat- plexesareseenonECG)isonlyfoundinVTs(Figure6).AnRScom- ternsuggestsVT.ThesecriteriaarenothelpfulfordifferentiatingVT plexisfoundinallSVTsandin74%ofVTs.Thelongestintervalfrom fromSVTinspecificsettingssuchaspre-excitedSVTorwhenclass theonsetoftheRwavetothedeepestpartoftheSwavelonger IcorclassIaantiarrhythmicdrugsareadministered. than100ms,irrespectiveofthemorphologyofthetachycardia,is notobservedinanySVTwithaberrantconduction.Abouthalfof QRSaxis theVTshaveanRSintervalof100msorlessandtheotherhalf InSVTpatientswithaberrationpattern,theQRSaxisisconfinedbe- haveanRSintervalofmorethan100ms.TheBrugadaetal.algo- tween2608and+1208.Therefore,wide-QRStachycardiaswitha rithm,whenall4stepsareapplied,hasasensitivityandspecificity QRSaxisoutsidethisrangearelikelytobeVT.Inparticular,ex- of98.7%and96.5%,respectively.59 tremeleftaxisdeviation(axisfrom2908to+1808)stronglysug- QRScomplexinaVRlead gestsVTbothinthepresenceofRBBBandLBBBpatterns.56Thus, predominantlynegativeQRScomplexesinleadsI,II,andIIIarea DuringsinusrhythmandSVT,thewavefrontofdepolarizationpro- usefulcriterionforidentifyingVT. ceedsinadirectionawayfromleadaVR,yieldinganegativeQRS