2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the

87 Pages·2015·1.83 MB·English

ESC GUIDELINES
EuropeanHeartJournal(2015)36,2793–2867
doi:10.1093/eurheartj/ehv316
2015 ESC Guidelines for the management
of patients with ventricular arrhythmias
and the prevention of sudden cardiac death
The Task Force for the Management of Patients with Ventricular
Arrhythmias and the Prevention of Sudden Cardiac Death of the
European Society of Cardiology (ESC)
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Endorsed by: Association for European Paediatric and Congenital o
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Cardiology (AEPC) a
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Authors/Task Force Members: Silvia G. Priori*(Chairperson) (Italy) h
Carina Blomstro¨m-Lundqvist*(Co-chairperson) (Sweden) Andrea Mazzanti† (Italy), ttp://e
Nico Bloma (The Netherlands), Martin Borggrefe (Germany), John Camm (UK), urh
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Perry Mark Elliott (UK), Donna Fitzsimons (UK), Robert Hatala (Slovakia), rtj.o
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Gerhard Hindricks (Germany), Paulus Kirchhof (UK/Germany), Keld Kjeldsen fo
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(Denmark), Karl-Heinz Kuck (Germany), Antonio Hernandez-Madrid (Spain), jou
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Nikolaos Nikolaou (Greece), Tone M. Norekva˚l (Norway), Christian Spaulding als
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(France), and Dirk J. Van Veldhuisen (The Netherlands) brg/
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*Correspondingauthors:SilviaGiulianaPriori,DepartmentofMolecularMedicineUniversityofPavia,Cardiology&MolecularCardiology,IRCCSFondazioneSalvatoreMaugeri, n A
ViaSalvatoreMaugeri10/10A,IT-27100Pavia,Italy,Tel:[phone],Fax:[phone],Email:[email protected] p
CarinaBlomstro¨m-Lundqvist,DepartmentofCardiology,InstitutionofMedicalScience,UppsalaUniversity,SE-75185Uppsala,Sweden,Tel:[phone],Fax:[phone], ril 2
Email:[email protected] 0, 2
aRepresentingtheAssociationforEuropeanPaediatricandCongenitalCardiology(AEPC). 01
†AndreaMazzanti:Coordinator,affiliationlistedintheAppendix. 6
ESCCommitteeforPracticeGuidelines(CPG)andNationalCardiacSocietiesdocumentreviewers:listedintheAppendix.
ESCentitieshavingparticipatedinthedevelopmentofthisdocument:
ESCAssociations:AcuteCardiovascularCareAssociation(ACCA),EuropeanAssociationofCardiovascularImaging(EACVI),EuropeanAssociationofPercutaneousCardiovascular
Interventions(EAPCI),EuropeanHeartRhythmAssociation(EHRA),HeartFailureAssociation(HFA).
ESCCouncils:CouncilforCardiologyPractice(CCP),CouncilonCardiovascularNursingandAlliedProfessions(CCNAP),CouncilonCardiovascularPrimaryCare(CCPC),
CouncilonHypertension.
ESCWorkingGroups:CardiacCellularElectrophysiology,CardiovascularPharmacotherapy,CardiovascularSurgery,Grown-upCongenitalHeartDisease,Myocardialand
PericardialDiseases,PulmonaryCirculationandRightVentricularFunction,Thrombosis,ValvularHeartDisease.
ThecontentoftheseEuropeanSocietyofCardiology(ESC)Guidelineshasbeenpublishedforpersonalandeducationaluseonly.Nocommercialuseisauthorized.NopartoftheESC
GuidelinesmaybetranslatedorreproducedinanyformwithoutwrittenpermissionfromtheESC.PermissioncanbeobtaineduponsubmissionofawrittenrequesttoOxford
UniversityPress,thepublisheroftheEuropeanHeartJournalandthepartyauthorizedtohandlesuchpermissionsonbehalfoftheESC.
Disclaimer:TheESCGuidelinesrepresenttheviewsoftheESCandwereproducedaftercarefulconsiderationofthescientificandmedicalknowledgeandtheevidenceavailableat
thetimeoftheirpublication.TheESCisnotresponsibleintheeventofanycontradiction,discrepancyand/orambiguitybetweentheESCGuidelinesandanyotherofficialrecom-
mendationsorguidelinesissuedbytherelevantpublichealthauthorities,inparticularinrelationtogooduseofhealthcareortherapeuticstrategies.Healthprofessionalsareencour-
agedtotaketheESCGuidelinesfullyintoaccountwhenexercisingtheirclinicaljudgment,aswellasinthedeterminationandtheimplementationofpreventive,diagnosticor
therapeuticmedicalstrategies;however,theESCGuidelinesdonotoverride,inanywaywhatsoever,theindividualresponsibilityofhealthprofessionalstomakeappropriateand
accuratedecisionsinconsiderationofeachpatient’shealthconditionandinconsultationwiththatpatientand,whereappropriateand/ornecessary,thepatient’scaregiver.Nor
dotheESCGuidelinesexempthealthprofessionalsfromtakingintofullandcarefulconsiderationtherelevantofficialupdatedrecommendationsorguidelinesissuedbythecompetent
publichealthauthorities,inordertomanageeachpatient’scaseinlightofthescientificallyaccepteddatapursuanttotheirrespectiveethicalandprofessionalobligations.Itisalsothe
healthprofessional’sresponsibilitytoverifytheapplicablerulesandregulationsrelatingtodrugsandmedicaldevicesatthetimeofprescription.
&TheEuropeanSocietyofCardiologyandtheEuropeanRespiratorySociety2015.Allrightsreserved.Forpermissionspleaseemail:[email protected].
2794 ESCGuidelines
DocumentReviewers:PhilippeKolh(CPGReviewCoordinator)(Belgium),GregoryY.H.Lip(CPGReview
Coordinator)(UK),StefanAgewall(Norway),GonzaloBaro´n-Esquivias(Spain),GiuseppeBoriani (Italy),
WernerBudts(Belgium),He´ctorBueno(Spain),DavideCapodanno(Italy),ScipioneCarerj (Italy),
MariaG.Crespo-Leiro(Spain),MartinCzerny(Switzerland),ChristiDeaton(UK),DobromirDobrev(Germany),
ÇetinErol(Turkey),MaurizioGalderisi(Italy),BulentGorenek(Turkey),ThomasKriebel(Germany),PierLambiase
(UK),PatrizioLancellotti(Belgium),DeirdreA.Lane(UK),IreneLang(Austria),AthanasiosJ.Manolis(Greece),
JoaoMorais(Portugal),JavierMoreno(Spain),MassimoF.Piepoli (Italy),FransH.Rutten(TheNetherlands),
BeataSredniawa(Poland),JoseL.Zamorano(Spain),andFaiezZannad(France)
ThedisclosureformsofallexpertsinvolvedinthedevelopmentoftheseguidelinesareavailableontheESCwebsitehttp
://www.escardio.org/guidelines
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Keywords Acutecoronarysyndrome † Cardiacresynchronizationtherapy † Cardiomyopathy † Congenitalheartdisease
† Defibrillator † Guidelines † Heartfailure † Implantablecardioverterdefibrillator † Myocardialinfarction
† Resuscitation † Stablecoronaryarterydisease † Suddencardiacdeath † Tachycardia † Valvularheart
disease † Ventriculararrhythmia
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Table of Contents 4.2.3 Patientswithacardioverterdefibrillator . . . . . . . .2809 fro
4.2.4 Electrolytes . . . . . . . . . . . . . . . . . . . . . . . . . . .2809 m
h
Abbreviationsandacronyms . . . . . . . . . . . . . . . . . . . . . . . .2796 4.2.5 Otherdrugtherapy. . . . . . . . . . . . . . . . . . . . . .2809 ttp
1. Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2797 4.3 Devicetherapy . . . . . . . . . . . . . . . . . . . . . . . . . . .2809 ://e
u
2. Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2798 4.3.1 Implantablecardioverterdefibrillator . . . . . . . . . .2809 rh
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3. D2e.1finSittirouncst,uerpeidoefmthioelogugyidaenlindefsut.ur.e.p.e.rs.p.e.ct.iv.e.s.fo.r.t.h.e. . . .2799 4an.3d.1v.1enStreiccuolnadratraychpyrceavrednitaio.n.o.f.su.d.d.e.n.c.ar.d.ia.c.d.e.at.h. .2810 rtj.ox
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preventionofsuddencardiacdeath. . . . . . . . . . . . . . . . . . . .2799 4.3.2 Subcutaneousimplantablecardioverter rd
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3.1 Epidemiologyofsuddencardiacdeath. . . . . . . . . . . . .2799 defibrillator . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2810 u
rn
3.1.1 Causesofsuddencardiacdeathindifferentage 4.3.3 Wearablecardioverterdefibrillator . . . . . . . . . . .2811 als
groups . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2800 4.3.4 Publicaccessdefibrillation . . . . . . . . . . . . . . . . .2811 .org
3.2 Autopsyandmolecularautopsyinsuddendeathvictims.2800 4.4 Acutetreatementofsustainedventriculararrhythmias . .2812 b/
y
3.3 Riskpredictionofsuddencardiacdeath . . . . . . . . . . .2800 4.5 Interventionaltherapy . . . . . . . . . . . . . . . . . . . . . . .2814 gu
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3.3.1 Individualswithoutknownheartdisease . . . . . . . .2801 4.5.1 Catheterablation . . . . . . . . . . . . . . . . . . . . . . .2814 st o
3.3.2 Patientswithischaemicheartdisease . . . . . . . . . .2801 4.5.1.1 Patientswithscar-relatedheartdisease . . . . . .2814 n A
3.43.P3r.3evPenattiieonntsowfsiuthddinehnecriatradbilaecadrerhatyhthimnospgeecniiacldseistetainsgess .. ..22880011 4.54.2.5.A1.n2ti-Paartrihenytthsmwiicthsouurgteorvye.rt.s.tr.u.ct.u.ra.l.h.e.ar.t.d.is.ea.s.e. ..22881154 pril 20
3.4.1 Screeningthegeneralpopulationfortheriskof 4.6 Psychosocialimpactofimplantablecardioverter , 20
1
suddencardiacdeath. . . . . . . . . . . . . . . . . . . . . . . . .2801 defibrillatortreatment . . . . . . . . . . . . . . . . . . . . . . . . .2815 6
3.4.2 Screeningfamilymembersofsuddendeath 5. Managementofventriculararrhythmiasandpreventionof
victims . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2802 suddencardiacdeathincoronaryarterydisease . . . . . . . . . . .2816
3.4.3 Screeningpatientswithdocumentedorsuspected 5.1 Acutecoronarysyndromes . . . . . . . . . . . . . . . . . . .2816
ventriculararrhythmias . . . . . . . . . . . . . . . . . . . . . . .2802 5.1.1 Ventriculararrhythmiasassociatedwithacute
3.4.3.1 Clinicalhistory . . . . . . . . . . . . . . . . . . . . . .2802 coronarysyndromes . . . . . . . . . . . . . . . . . . . . . . . .2816
3.4.3.2 Non-invasiveandinvasiveevaluation. . . . . . . .2803 5.1.2 Preventionandmanagementofsuddencardiacdeath
4. Therapiesforventriculararrhythmias . . . . . . . . . . . . . . . .2806 associatedwithacutecoronarysyndromes:pre-hospital
4.1 Treatmentofunderlyingheartdisease . . . . . . . . . . . .2806 phase. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2816
4.2 Pharmacotherapyforventriculararrhythmiaand 5.1.3 Preventionofsuddencardiacdeathassociatedwith
preventionofsuddencardiacdeath . . . . . . . . . . . . . . . . .2807 acutecoronarysyndromes:in-hospitalphase . . . . . . . . .2816
4.2.1 Generalmanagement . . . . . . . . . . . . . . . . . . . .2807 5.1.3.1 Ventriculararrhythmiasinacutecoronary
4.2.2 Anti-arrhythmicdrugs . . . . . . . . . . . . . . . . . . . .2807 syndromes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2818
4.2.2.1 Beta-blockers. . . . . . . . . . . . . . . . . . . . . . .2807 5.1.3.2 Useofanti-arrhythmicdrugsinacutecoronary
4.2.2.2 Amiodarone . . . . . . . . . . . . . . . . . . . . . . .2807 syndromes—generalconsiderations . . . . . . . . . . . . .2818
4.2.2.3 Sotalol/d-sotalol . . . . . . . . . . . . . . . . . . . . .2809 5.1.3.3 Patientswithacutecoronarysyndromesandno
4.2.2.4 Combinationtherapy. . . . . . . . . . . . . . . . . .2809 ventriculararrhythmias. . . . . . . . . . . . . . . . . . . . . .2818
ESCGuidelines 2795
5.1.3.4 Prematureventricularcomplexes . . . . . . . . . .2818 7.2.1 Definitions,epidemiology,andsurvivaldata . . . . . .2829
5.1.3.5 SustainedVTandVF . . . . . . . . . . . . . . . . . .2818 7.2.2 Approachtoriskstratificationandmanagement . . .2829
5.1.3.6 Catheterablationofrecurrentsustained 7.2.3 Ventriculararrhythmiasinhypertrophic
ventriculartachycardia,recurrentventricularfibrillation, cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . .2830
andelectricalstorm. . . . . . . . . . . . . . . . . . . . . . . .2818 7.2.4 Approachtoriskstratificationandmanagementin
5.1.3.7 Extracorporealsupportdevices . . . . . . . . . . .2819 adultspatients . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2830
5.1.3.8 Bradycardiaandheartblock . . . . . . . . . . . . .2819 7.2.5 Approachtoriskstratificationandmanagementin
5.1.4 Theprognosticroleofearlyventricularfibrillation. .2819 paediatricpatients. . . . . . . . . . . . . . . . . . . . . . . . . . .2830
5.2 Earlyaftermyocardialinfarction . . . . . . . . . . . . . . . .2819 7.2.6 Preventionofsuddencardiacdeath . . . . . . . . . . .2830
5.2.1 Riskstratificationforsuddencardiacdeath. . . . . . .2819 7.2.6.1 Drugsandlifestyleadvice . . . . . . . . . . . . . . .2830
5.2.2 Timingofimplantablecardioverterdefibrillator 7.2.6.2 Implantablecardioverterdefibrillators. . . . . . .2831
placementaftermyocardialinfarction—assessmentofleft 7.3 Arrhythmogenicrightventricularcardiomyopathy. . . . .2831
ventriculardysfunctionbeforeandafterdischarge . . . . . .2819 7.3.1 Definitions,epidemiology,andsurvival . . . . . . . . .2831
5.3 Stablecoronaryarterydiseaseaftermyocardialinfarction 7.3.2 Approachtoriskstratificationandmanagement . . . .39
withpreservedejectionfraction . . . . . . . . . . . . . . . . . . .2820 7.3.3 Ventriculararrhythmiasinarrhythmogenicright
5.3.1 Riskstratification . . . . . . . . . . . . . . . . . . . . . . .2820 ventricularcardiomyopathy . . . . . . . . . . . . . . . . . . . .2831
5.3.2 Recommendationsforoptimalstrategy . . . . . . . . .2820 7.3.3.1 Treatmentofventriculararrhythmia . . . . . . .2832
5.3.3 Useofanti-arrhythmicdrugs. . . . . . . . . . . . . . . .2820 7.3.3.2 Exerciserestriction . . . . . . . . . . . . . . . . . . .2832
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5.3.4 Catheterablation . . . . . . . . . . . . . . . . . . . . . . .2821 7.3.3.3 Implantablecardioverterdefibrillators. . . . . . .2832 ow
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6. Therapiesforpatientswithleftventriculardysfunction,withor 7.4 Infiltrativecardiomyopathies . . . . . . . . . . . . . . . . . . .2832 lo
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withoutheartfailure . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2821 7.4.1 Cardiacamyloidosis . . . . . . . . . . . . . . . . . . . . .2832 de
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6.1 Primarypreventionofsuddencardiacdeath. . . . . . . . .2821 7.5 Restrictivecardiomyopathy. . . . . . . . . . . . . . . . . . . .2832 fro
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6.1.1 Drugs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2821 7.6 Othercardiomyopathies . . . . . . . . . . . . . . . . . . . . .2833 h
6.1.2 Implantablecardioverterdefibrillators. . . . . . . . . .2822 7.6.1 Left-ventricularnon-compaction . . . . . . . . . . . . .2833 ttp
6.1.3 Implantablecardioverterdefibrillatorsinpatientswith 7.6.2 Chagas’cardiomyopathy. . . . . . . . . . . . . . . . . . .2833 ://e
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NewYorkHeartAssociationclassIVlistedforheart 8. Inheritedprimaryarrhythmiasyndromes . . . . . . . . . . . . . .2833 rhe
a
transplantation. . . . . . . . . . . . . . . . . . . . . . . . . . . . .2823 8.1 LongQTsyndrome . . . . . . . . . . . . . . . . . . . . . . . .2833 rtj.o
6.1.4 Cardiacresynchronizationtherapy . . . . . . . . . . . .2823 8.1.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2833 xfo
6.1.4.1 Heartfailurewithreducedleftventricular 8.1.2 Approachtoriskstratificationandmanagement . . .2834 rd
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ejectionfractionandNewYorkHeartAssociationclass 8.2 ShortQTsyndrome . . . . . . . . . . . . . . . . . . . . . . . .2835 urn
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III/ambulatoryclassIV . . . . . . . . . . . . . . . . . . . . . .2823 8.2.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2835 ls
.o
6.1.4.2 Heartfailurewithreducedleftventricular 8.2.2 Approachtoriskstratificationandmanagement . . .2835 rg
ejectionfractionbutmildsymptoms(NewYorkHeart 8.3 Brugadasyndrome . . . . . . . . . . . . . . . . . . . . . . . . .2836 by/
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AssociationclassII) . . . . . . . . . . . . . . . . . . . . . . . .2825 8.3.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2836 u
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6.2 Prematureventricularcomplexesinpatientswith 8.3.2 Approachtoriskstratificationandmanagement . . .2836 t o
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structuralheartdisease/leftventriculardysfunction . . . . . . .2825 8.4 Catecholaminergicpolymorphicventriculartachycardia .2837 A
p
6.3 Sustainedventriculartachycardia . . . . . . . . . . . . . . . .2825 8.4.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2837 ril 2
6.3.1 Drugtherapy. . . . . . . . . . . . . . . . . . . . . . . . . .2825 8.4.2 Approachtoriskstratificationandmanagement . . .2837 0
, 2
6.3.2 Catheterablation . . . . . . . . . . . . . . . . . . . . . . .2826 8.5 Earlyrepolarizationsyndrome. . . . . . . . . . . . . . . . . .2838 0
1
6
6.3.2.1 Patientswithleftventriculardysfunction . . . . .2826 8.5.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2838
6.3.2.2 Bundlebranchre-entranttachycardia . . . . . . .2827 9. Paediatricarrhythmiasandcongenitalheartdisease . . . . . . .2838
6.3.3 Implantablecardioverterdefibrillator . . . . . . . . . .2827 9.1 Managementofventriculararrhythmiasinchildrenwitha
7. Cardiomyopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2827 structurallynormalheart . . . . . . . . . . . . . . . . . . . . . . . .2838
7.1 Dilatedcardiomyopathy. . . . . . . . . . . . . . . . . . . . . .2827 9.2 Suddencardiacdeathandventriculararrhythmiasin
7.1.1 Definitions,epidemiology,andsurvivaldata . . . . . .2827 patientswithcongenitalheartdisease. . . . . . . . . . . . . . . .2839
7.1.2 Approachtoriskstratificationandmanagement . . .2827 9.3 Implantablecardioverterdefibrillatortherapyinpaediatric
7.1.2.1 Trialsofimplantablecardioverterdefibrillator patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2840
therapyindilatedcardiomyopathy . . . . . . . . . . . . . .2828 10. Ventriculartachycardiasandventricularfibrillationin
7.1.2.2 Primaryprophylaxis. . . . . . . . . . . . . . . . . . .2828 structurallynormalhearts . . . . . . . . . . . . . . . . . . . . . . . . . .2841
7.1.2.3 Secondaryprophylaxis. . . . . . . . . . . . . . . . .2829 10.1 Outflowtractventriculartachycardias. . . . . . . . . . . .2841
7.1.2.4 Cause-specificmortality . . . . . . . . . . . . . . . .2829 10.1.1 Rightventricularoutflowtracttachycardias . . . . .2842
7.1.2.5 Managementofventriculararrhythmiaindilated 10.1.2 Leftventricularoutflowtracttachycardias . . . . . .2842
cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . .2829 10.1.3 Aorticcuspventriculartachycardias . . . . . . . . . .2842
7.1.2.6 Ablationofventriculartachycardia . . . . . . . . .2829 10.1.4 Epicardialoutflowtractventriculartachycardias . .2842
7.2 Hypertrophiccardiomyopathy. . . . . . . . . . . . . . . . . .2829 10.1.5 Others(includingpulmonaryarteries). . . . . . . . .2842
2796 ESCGuidelines
10.2 Ventriculartachycardiasofmiscellaneousorigin . . . . .2842 Abbreviations and acronyms
10.2.1 Idiopathicleftventriculartachycardia. . . . . . . . . .2843
10.2.2 Papillarymuscleventriculartachycardia . . . . . . . .2843
10.2.3 Annularventriculartachycardia(mitraland ACC AmericanCollegeofCardiology
tricuspid) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2843 ACE angiotensin-convertingenzyme
10.3 Idiopathicventricularfibrillation. . . . . . . . . . . . . . . .2843 ACS acutecoronarysyndrome
10.4 Short-coupledtorsadedepointes . . . . . . . . . . . . . .2844 AF atrialfibrillation
11. Inflammatory,rheumaticandvalvularheartdiseases . . . . . .2844 AGNES ArrhythmiaGeneticsintheNetherlands
11.1 Myocarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2845 AHA AmericanHeartAssociation
11.1.1 Acuteandfulminantmyocarditis . . . . . . . . . . . .2845 AMIOVIRT AMIOdaroneVersusImplantablecardiover-
11.1.2 Myocarditisleadingtoinflammatory ter-defibrillator:RandomizedTrialinpatients
cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . .2846 withnon-ischaemicdilatedcardiomyopathy
11.2 Endocarditis. . . . . . . . . . . . . . . . . . . . . . . . . . . . .2846 andasymptomaticnon-sustainedventricular
11.3 Rheumaticheartdisease. . . . . . . . . . . . . . . . . . . . .2846 tachycardia
11.4 Pericarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2846 ARB angiotensinIIreceptorblocker
11.5 Cardiacsarcoidosis . . . . . . . . . . . . . . . . . . . . . . . .2846 ARVC arrhythmogenicrightventricularcardiomyopathy
11.6 Valvularheartdisease . . . . . . . . . . . . . . . . . . . . . .2847 AV atrio-ventricular
12. Arrhythmicriskinselectedpopulations . . . . . . . . . . . . . .2847 AVID Antiarrhythmic drugs Versus Implantable
D
12.1 Psychiatricpatients . . . . . . . . . . . . . . . . . . . . . . . .2847 Defibrillator ow
12.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . .2848 BrS BrugadaSyndrome nlo
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12.1.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . .2848 CAD coronaryarterydisease de
d
12.1.3 Treatment. . . . . . . . . . . . . . . . . . . . . . . . . . .2848 CARE-HF CArdiacREsynchronization – HeartFailure fro
12.2 Neurologicalpatients. . . . . . . . . . . . . . . . . . . . . . .2849 CASH CardiacArrestStudyHamburg hm
12.2.1 Suddenunexplaineddeathinepilepsy . . . . . . . . .2849 CAST CardiacArrhythmiaSuppressionTrial ttp
12.2.2 Neuromusculardisorders . . . . . . . . . . . . . . . . .2849 CAT CArdiomyopathyTrial ://e
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12.3 Pregnantpatients . . . . . . . . . . . . . . . . . . . . . . . . .2850 CHD congenitalheartdisease rhe
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1ca2r.3d.i1omAyrorphaytthhymi.as. .no.t.r.e.la.te.d.t.o.p.e.ri.p.ar.tu.m. . . . . . . . . .2850 CCIIDS cCoannfiaddeiannceIminptleanrvtaablleDefibrillatorStudy rtj.oxfo
12.3.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . .2850 CMR cardiacmagneticresonance rd
jo
12.3.1.2 Diagnosis. . . . . . . . . . . . . . . . . . . . . . . . .2851 COMPANION ComparisonofMedicalTherapy,Pacing,and urn
12.3.1.3 Treatment . . . . . . . . . . . . . . . . . . . . . . .2851 DefibrillationinHeartFailure als
12.3.2 Arrhythmiasrelatedtoperipartumcardiomyopathy2851 CPG CommitteeforPracticeGuidelines .org
12.4 Obstructivesleepapnoea. . . . . . . . . . . . . . . . . . . .2852 CPVT catecholaminergic polymorphic ventricular by/
12.4.1 Bradyarrhythmiasand –tachyarrhythmias. . . . . . .2852 tachycardia gu
e
12.4.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . .2852 CRT cardiacresynchronizationtherapy st o
12.4.1.2 Diagnosis. . . . . . . . . . . . . . . . . . . . . . . . .2852 CRT-D cardiacresynchronizationtherapydefibrillator n A
12.5 1D2r.4u.g1-.r3elaTtreedatpmroe-natrr.h.yt.h.m.ia. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. ..22885522 CCRTT-P ccaormdipauctreedsytoncmhorogrnaizpahtyiontherapypacemaker pril 20
12.5.1 Drug–substrateinteraction,duetounderlying DCM dilatedcardiomyopathy , 20
1
diseasesubstrate . . . . . . . . . . . . . . . . . . . . . . . . . . .2852 DEFINITE DEFIbrillatorsinNon-Ischemiccardiomyop- 6
athyTreatmentEvaluation
12.5.2 Drug–druginteraction(duetospecificdrugsand
DFT defibrillationthreshold
combinations) . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2853
12.5.3 Pro-arrhythmicriskofanti-arrhythmicdrugs. . . . .2853 DIAMOND Danish Investigators of Arrhythmia and
MortalityoNDofetilide
12.5.4 Pro-arrhythmiaduetotriggeringfactors . . . . . . .2853
ECG electrocardiogram/electrocardiographic
12.6 Suddencardiacdeathafterhearttransplantation . . . .2853
12.7 Suddencardiacdeathinathletes . . . . . . . . . . . . . . .2853 EHRA EuropeanHeartRhythmAssociation
EPS electrophysiologicalstudy
12.8 Wolff–Parkinson–Whitesyndrome . . . . . . . . . . . . .2854
ESC EuropeanSocietyofCardiology
12.9 Preventionofsuddencardiacdeathintheelderly . . . .2856
12.10 End-of-lifeissues . . . . . . . . . . . . . . . . . . . . . . . . .2856 GWAS genome-wideassociationstudy
HCM hypertrophiccardiomyopathy
13. Gapsinevidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2856
HF heartfailure
14. Todoandnottodomessagesfromtheguidelines . . . . . . .2857
15. Webaddenda . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2858 HFpEF heartfailurewithpreservedejectionfraction
HFrEF heartfailurewithreducedejectionfraction
16. Appendix. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2858
HR hazardratio
17. References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2859
i.v. intravenous
ESCGuidelines 2797
ICD implantablecardioverterdefibrillator SUDS suddenunexplaineddeathsyndrome
ILCOR InternationalLiaisonCommitteeOn TdP torsadedepointes
Resuscitation US UnitedStates
IRIS ImmediateRiskstratificationImprovesSurvival VA ventriculararrhythmia
LBBB leftbundlebranchblock VF ventricularfibrillation
LMNA laminA/C VT ventriculartachycardia
LQTS longQTsyndrome VTACH VentricularTachycardiaAblationinCoronary
LQTS1 longQTsyndrometype1 HeartDisease
LQTS2 longQTsyndrometype2 WCD wearablecardioverterdefibrillator
LQTS3 longQTsyndrometype3 WPW Wolff–Parkinson–White
LV leftventricle/leftventricular
LVEF leftventricularejectionfraction
LVOT leftventricularoutflowtract 1. Preamble
MADIT MulticenterAutomaticDefibrillatorImplant-
ationTrial Guidelinessummarizeandevaluateallavailableevidenceonapar-
MIRACLE Multicenter InSync Randomized Clinical ticularissueatthetimeofthewritingprocess,withtheaimofassist-
Evaluation inghealthprofessionalsinselectingthebestmanagementstrategies
MRA mineralocorticoidreceptorantagonist foranindividualpatientwithagivencondition,takingintoaccount
D
ms millisecond theimpactonoutcome,aswellastherisk–benefitratioofparticu- ow
n
MUSTT MulticenterUnSustainedTachycardiaTrial lardiagnosticortherapeuticmeans.Guidelinesandrecommenda- lo
a
d
NSTEMI non–ST-segment elevation myocardial tionsshouldhelphealthprofessionalstomakedecisionsintheir e
d
infarction dailypractice.However,thefinaldecisionsconcerninganindividual fro
m
NSVT non-sustainedventriculartachycardia patientmustbemadebytheresponsiblehealthprofessional(s)in h
NYHA NewYorkHeartAssociation consultationwiththepatientandcaregiverasappropriate. ttp
OPTIC OptimalPharmacologicalTherapyInCardio- AgreatnumberofGuidelineshavebeenissuedinrecentyearsby ://eu
verterdefibrillatorpatients theEuropeanSocietyofCardiology(ESC)aswellasbyothersoci- rhe
a
OR oddsratio etiesandorganisations.Becauseoftheimpactonclinicalpractice, rtj.o
OT outflowtract qualitycriteriaforthedevelopmentofguidelineshavebeenestab- xfo
PRESERVE-EF riskstratificationinpatientswithpreserved lishedinordertomakealldecisionstransparenttotheuser.There- rdjo
u
ejectionfraction commendationsforformulatingandissuingESCGuidelinescanbe rn
a
PVC prematureventricularcomplex foundontheESCwebsite(http://www.escardio.org/Guidelines- ls
.o
PVS programmedventricularstimulation &-Education/Clinical-Practice-Guidelines/Guidelines-development/ rg
QTc correctedQT Writing-ESC-Guidelines).ESCGuidelinesrepresenttheofficialpos- by/
g
RAFT Resynchronization–DefibrillationforAmbu- itionoftheESConagiventopicandareregularlyupdated. u
e
s
latoryHeartFailureTrial MembersofthisTaskForcewereselectedbytheESCtore- t o
n
RBBB rightbundlebranchblock presentprofessionalsinvolvedwiththemedicalcareofpatients A
p
RCT randomizedcontrolledtrial with this pathology. Selected experts in the field undertook a ril 2
REVERSE REsynchronizationreVErsesRemodeling in comprehensivereviewofthepublishedevidenceformanagement 0
, 2
SystolicleftvEntriculardysfunction (includingdiagnosis,treatment,preventionandrehabilitation)of 0
1
6
REVERSEMIRACLE MulticenterInSyncICDRandomizedClinical a given condition according to ESC Committee for Practice
ICD Evaluation Guidelines (CPG) policy.A critical evaluationof diagnosticand
RR relativerisk therapeuticprocedureswasperformed,includingassessmentof
RV rightventricular therisk–benefitratio.Estimatesofexpectedhealthoutcomesfor
RVOT rightventricularoutflowtract largerpopulationswereincluded,wheredataexist.Thelevelof
SA-ECG signal-averagedECG evidence andthe strengthofthe recommendationofparticular
SADS suddenarrhythmicdeathsyndrome managementoptionswereweighedandgradedaccordingtoprede-
SCD suddencardiacdeath finedscales,asoutlinedinTables1and2.
SCD-HeFT SuddenCardiacDeathinHEartFailureTrial Theexpertsofthewritingandreviewingpanelsprovideddeclara-
SCORE SystematicCoronaryRiskEvaluation tionsofinterestformsforallrelationshipsthatmightbeperceivedas
SIDS suddeninfantdeathsyndrome realorpotentialsourcesofconflictsofinterest.Theseformswere
SMASH-VT Substrate Mapping and Ablation in Sinus compiledintoonefileandcanbefoundontheESCwebsite(http://
RhythmtoHaltVentricularTachycardia www.escardio.org/guidelines).Anychangesindeclarationsofinterest
SPECT single-photonemissioncomputedtomography thatariseduringthewritingperiodmustbenotifiedtotheESCand
SQTS shortQTsyndrome updated.TheTaskForcereceiveditsentirefinancialsupportfromthe
STEMI ST-segmentelevationmyocardialinfarction ESCwithoutanyinvolvementfromthehealthcareindustry.
SUDEP suddenunexpecteddeathinepilepsy TheESCCPGsupervisesandcoordinatesthepreparationofnew
SUDI suddenunexplaineddeathininfancy Guidelinesproducedbytaskforces,expertgroupsorconsensus
2798 ESCGuidelines
Table1 Classesofrecommendations
Classes of Suggested wording to use
recommendations
Class I Evidence and/or general Is recommended/is
agreement that a given treatment indicated
or procedure is beneficial, useful,
effective.
Class II Conflicting evidence and/or a
divergence of opinion about the
usefulness/efficacy of the given
treatment or procedure.
Class IIa Weight of evidence/opinion is in Should be considered
favour of usefulness/efficacy.
Class IIb Usefulness/efficacy is less well May be considered
established by evidence/opinion.
Class III Evidence or general agreement Is not recommended
that the given treatment or D
o
procedure is not useful/effective, w
n
and in some cases may be harmful. lo
a
d
e
d
fro
m
h
ttp
panels.TheCommitteeisalsoresponsiblefortheendorsement consultationwiththatpatientandthepatient’scaregiverwhereap- ://e
u
processoftheseGuidelines.TheESCGuidelinesundergoextensive propriateand/ornecessary.Itisalsothehealthprofessional’sre- rhe
a
reviewbytheCPGandexternalexperts.Afterappropriaterevi- sponsibilitytoverifytherulesandregulationsapplicabletodrugs rtj.o
sionstheGuidelinesareapprovedbyalltheexpertsinvolvedin anddevicesatthetimeofprescription. xfo
theTaskForce.ThefinalizeddocumentisapprovedbytheCPG rd
jo
for publication in the European Heart Journal. The Guidelines urn
a
weredevelopedaftercarefulconsiderationofthescientificand Table2 Levelsofevidence ls
.o
medical knowledge and the evidence available at the time of rg
theirdating. Level of Data derived from multiple randomized by/
g
ThetaskofdevelopingESCGuidelinescoversnotonlyintegra- evidence A clinical trials or meta-analyses. u
e
s
tionofthemostrecentresearch,butalsothecreationofeducation- Data derived from a single randomized t o
altoolsandimplementationprogrammesfortherecommendations. Level of clinical trial or large non-randomized n A
evidence B p
Toimplementtheguidelines,condensedpocketguidelinesversions, studies. ril 2
summaryslides,bookletswithessentialmessages,summarycards Level of Consensus of opinion of the experts and/ 0, 2
fornon-specialists,andanelectronicversionfordigitalapplications or small studies, retrospective studies, 0
evidence C 1
(smartphones,etc.)areproduced.Theseversionsareabridgedand registries. 6
thus,ifneeded,oneshouldalwaysrefertothefulltextversion,
whichisfreelyavailableontheESCwebsite.TheNationalSocieties
oftheESCareencouragedtoendorse,translateandimplementall
ESCGuidelines.Implementationprogrammesareneededbecauseit 2. Introduction
hasbeenshownthattheoutcomeofdiseasemaybefavourablyin-
fluencedbythethoroughapplicationofclinicalrecommendations. ThepresentdocumenthasbeenconceivedastheEuropeanupdate
Surveysandregistriesareneededtoverifythatreal-lifedailyprac- totheAmericanCollegeofCardiology(ACC)/AmericanHeartAs-
ticeisinkeepingwithwhatisrecommendedintheguidelines,thus sociation(AHA)/ESC2006Guidelinesformanagementofpatients
completingtheloopbetweenclinicalresearch,writingofguidelines, withventriculararrhythmias(VA)andthepreventionofsuddencar-
disseminatingthemandimplementingthemintoclinicalpractice. diacdeath(SCD).1Inlightoftheveryrecentconsensusdocuments
HealthprofessionalsareencouragedtotaketheESCGuidelines forthemanagementofpatientswithVAreleasedbythemajorinter-
fullyintoaccountwhenexercisingtheirclinicaljudgment,aswellas nationalheartrhythmsocieties,2,3theESCGuidelinesCommittee
inthedeterminationandtheimplementationofpreventive,diagnos- decidedtofocusthecontentofthisdocumentontheprevention
ticortherapeuticmedicalstrategies.However,theESCGuidelines ofSCD.The update istimely,consideringthe new insightsinto
donotoverrideinanywaywhatsoevertheindividualresponsibility thenaturalhistoryofdiseasespredisposingtoSCDandthecomple-
ofhealthprofessionalstomakeappropriateandaccuratedecisions tionofmajorstudiesthatwillimpactmanagementstrategiesfor
in consideration of each patient’s health condition and in heartfailure(HF)involvingbothdruganddevicetherapies.
ESCGuidelines 2799
2.1 Structure of the guidelines 3. Definitions, epidemiology
Thedocumentisdividedinsectionsthatcoverspecifictopics.The and future perspectives for
riskevaluationschemeandtreatmentofferedshouldbetailoredin
the prevention of sudden cardiac
considerationofco-morbidities,limitationoflifeexpectancy,impact
onqualityoflifeandothercircumstances. death
Whilepreparingthisupdate,thecommitteereviewedthemost
Thedefinitionsusedforsuddendeath,abortedcardiacarrest,idio-
recent recommendations foreach topic and modified the class
pathicventricularfibrillation(VF)andforthepreventionofsudden
and/or the strength of recommendations, considering whether
deatharedetailedinTable3.
newresultsfromrandomizedtrials,meta-analysesorclinicalevi-
dencewouldcallforachange.Specialcarewastakentomaintain
consistencyintheuseoflanguagewithexistingguidelines.Occa-
3.1 Epidemiology of sudden cardiac death
sionally,however,wordingchangesweremadetorendersomeof
theoriginalrecommendationsmoreuserfriendlyandprecise. Inthepast20years,cardiovascularmortalityhasdecreasedinhigh-
The committee was composed of physicians and associated income countries19 in response to the adoption of preventive
healthcare providers who are experts in the areas of SCD and measurestoreduce the burdenofCAD andHF.Despite these
prevention,complexVA,interventionalelectrophysiology,coron- encouragingresults,cardiovasculardiseasesareresponsibleforap-
ary artery disease (CAD), HF and cardiomyopathy, paediatric proximately17milliondeathseveryyearintheworld,approximate-
cardiologyandarrhythmias,devicetherapy,cardiovascularcare,car- ly25%ofwhichareSCD.20TheriskofSCDishigherinmenthanin D
o
diovasculargeneticsandnursing.Expertsindifferentsubspecialties women,anditincreaseswithageduetothehigherprevalenceof w
n
incardiologywereidentifiedwiththehelpoftherelatedworking CADinolderage.21Accordingly,theSCDrateisestimatedtorange loa
d
groupsoftheESC. from1.40per100000person-years[95%confidenceinterval(CI) ed
Allmembersofthewritingcommitteeapprovedtheguidelinere- 0.95,1.98]inwomento6.68per100000person-years(95%CI fro
m
commendations.Seventy-fourpeerreviewersreviewedthedocu- 6.24,7.14)inmen.21SCDinyoungerindividualshasanestimatedin- h
ment.Anextensiveliteraturesurvey wasconductedthatled to cidenceof0.46–3.7eventsper100000person-years,22,23corre- ttp://e
theincorporationof810references.Theguidelinesreviewedcon- spondingtoaroughestimateof1100–9000deathsinEuropeand urh
cerningpreventionofSCDarelistedinWebTable1.3–13 800–6200deathsintheUSAeveryyear.24 ea
rtj.o
x
fo
rd
jo
u
Table3 Definitionsofcommonlyusedterms rna
ls
.o
rg
Term Refa b/
y
g
Sudden death Non-traumatic, unexpected fatal event occurring within 1 hour of the onset of symptoms in an apparently healthy 1 u
e
sIfu dbejeactth. is not witnessed, the applies when the victim was in good health 24 hours before the event. st on
A
p
SUDS and SUDI iSnufdandte n< 1d eyaetahr wofi tahgoeu (tS aUnD aIp)p. arent cause and in which an autopsy has not been performed in an adult (SUDS) or in an 14 ril 2
0
, 2
SCD The term is used when: 1, 14, 0
1
(cid:129)A congenital, or acquired, potentially fatal cardiac condition was known to be present during life; OR 15 6
(cid:129)Autopsyhas a cardiac or vascular anomaly as the probable cause of the event; OR
(cid:129)Noobviousextra-cardiac causes have been by post-mortem examination and therefore an arrhythmic event
is a likely cause of death.
SADS and SIDS Both autopsy and toxicology investigations are inconclusive, the heart is structurally normal at gross and histological 16
examination and non-cardiac aetiologies are excluded in adults (SADS) and in infants (SIDS).
Aborted cardiac Unexpected circulatory arrest, occurring within 1 hour of onset of acute symptoms, which is reversed by successful -
arrest resuscitation manoeuvres (e.g.
Idiopathic ventricular Clinical investigations are negative in a patient surviving an episode of ventricular 17, 18
Primary prevention Therapies to reduce the risk of SCD in individuals who are at risk of SCD but have not yet experienced an aborted -
of SCD cardiac arrest or life-threatening arrhythmias.
Secondary Therapies to reduce the risk of SCD in patients who have already experienced an aborted cardiac arrest or life- 1
prevention of SCD threatening arrhythmias.
SADS¼suddenarrhythmicdeathsyndrome;SCD¼suddencardiacdeath;SIDS¼suddeninfantdeathsyndrome;SUDI¼suddenunexplaineddeathininfancy;SUDS¼sudden
unexplaineddeathsyndrome.
aReferences.
2800 ESCGuidelines
3.1.1 Causesofsuddencardiacdeathindifferentagegroups AlthoughCADaccountsforalargeproportionofsuddendeaths,
CardiacdiseasesassociatedwithSCDdifferinyoungvs.olderindivi- especiallyforpersons .40yearsofage,othercausesshouldbe
duals.Intheyoungthereisapredominanceofchannelopathiesand takenintoaccount,includinggeneticdisordersthataffecteither
cardiomyopathies(WebTable2),21,25–48myocarditisandsubstance theintegrityoftheheart’smuscle(seesection7)oritselectrical
abuse,49whileinolderpopulations,chronicdegenerativediseasespre- function(seesection8).Everytimeaheritablediseaseisidentified
dominate(CAD,valvularheartdiseasesandHF).Severalchallenges inadeceasedindividual,therelativesofthevictimmaybeatriskof
undermineidentificationofthecauseofSCDinbothagegroups:older beingaffectedanddyingsuddenlyunlessatimelydiagnosisismade
victims,forinstance,maysufferfrommultiplechroniccardiovascular andpreventivemeasurestaken.
conditionssothatitbecomesdifficulttodeterminewhichcontributed Unfortunately,evenwhenanautopsyisperformed,aproportion
mosttoSCD.Inyoungerpersons,thecauseofSCDmaybeelusive ofsuddendeaths,rangingfrom2to54%,48remainunexplained
evenafterautopsy,becauseconditionssuchasinheritedchannelopa- (WebTable2):thisbroadrangeofvaluesislikelyduetoheterogen-
thiesordrug-inducedarrhythmiasthataredevoidofstructuralabnor- eityoftheautopsyprotocols.Topromoteacommonstandardfor
malitiesareepidemiologicallyrelevantinthisagegroup. autopsy,targetedguidelineshavebeendevelopedtodefineproto-
colsforheartexaminationandhistologicalsampling,aswellasfor
3.2 Autopsy and molecular autopsy in toxicologyandmolecularinvestigation.17,50Overall,aproperlycon-
sudden death victims ducted autopsyshould provide answerstothe following issues:
(i)whetherthedeathisattributabletoacardiacdisease,(ii)thena-
tureofthecardiacdisease(ifpresent),(iii)whetherthemechanism
D
Indicationsforautopsyandmolecularautopsyin ofdeathwasarrhythmic,(iv)whetherthereisevidenceofacardiac ow
suddendeathvictims diseasethatmaybeinheritedandthusrequiresscreeningandcoun- nlo
a
sellingofrelativesand(v)thepossibilityoftoxicorillicitdruguseor de
d
Recommendations Classa Levelb Ref.c othercausesofunnaturaldeaths. from
A standard histological examination of the heart should h
Anautopsyisrecommendedto includemappedlabelledblocksofmyocardiumfromrepresentative ttp
investigatethecausesofsudden transverseslicesofbothventricles.Weencouragepathologiststo ://e
deathandtodefinewhetherSCDis u
secondarytoarrhythmicor I C 17 contactspecializedcentresandsendthehearttothemforexamin- rhe
a
non-arrhythmicmechanisms(e.g. ation.Thepathologistshouldperformastandardgrossexamination rtj.o
ruptureofanaorticaneurysm). oftheheart,includingatransverseapicalsection,andtaketissues, xfo
Wheneveranautopsyisperformed, bloodandotherfluidsfortoxicologyandmolecularpathologybefore rdjo
astandardhistologicalexaminationof fixingtheheartinformalin.Furthermore,thecollectionandstorage urn
theheartisrecommendedandit a
ofbiologicalsamplesforDNAextractiontoallowa‘molecular’aut- ls
shouldincludemappedlabelled I C 17 .o
brelopcreksseonftamtiyvoectarradnisuvmerfsreomslicesof tohpesystiasnednacroduaruagtoepd.s1y7,Masoilteaclulolawrsauthtoepdsiyagisnoansisimppoostr-tmanotratedmditioofnthtoe byrg/
g
bothventricles. presenceofcardiacchannelopathiesthatmayexplain15–25%ofsud- u
e
Theanalysisofbloodandother denarrhythmicdeathsyndrome(SADS)cases.17Thevalueofthe st o
n
adequatelycollectedbodyfluidsfor post-mortemdiagnosisinavictimofSCDliesinextendinggenetic A
p
troecxoicmolmogeyndaenddimnoallelcvuicltairmpsaothfologyis I C 17 spcerretecnoinngsetonstuhsedfaomcuilmymenetmsbfoerrsthoefSdAiaDgnSoosrisSaIDndSmvicatnimagse.mReencetnotfeinx-- ril 20
unexplainedsuddendeath. , 2
heritable arrhythmias state that the use of a focused molecular 0
1
Targetedpost-mortemgenetic autopsy/post-mortem genetic testing should be considered for 6
analysisofpotentiallydisease-causing
SCDvictimswhenthepresenceofchannelopathiesissuspected.
genesshouldbeconsideredinallsudden 17,50,
IIa C Weendorsethisrecommendationandreferinterestedreadersto
deathvictimsinwhomaspecific 51
inheritablechannelopathyor themostrecentconsensusdocumentsonthistopic.14,52
cardiomyopathyissuspected.
3.3 Risk prediction of sudden cardiac
death
SCD¼suddencardiacdeath.
aClassofrecommendation. PredictionofSCDisthephilosopher’sstoneofarrhythmology,and
bLevelofevidence.
attemptstoprovidereliableindicatorsofSCDhavefuelledoneof
cReference(s)supportingrecommendations.
themostactiveareasofinvestigationinarrhythmologyduringre-
centdecades.53Itisnowclearthatthepropensitytodiesuddenly
Identificationofthecauseofanunexpecteddeathprovidesthefam- originatesasa‘perfectstorm’—interactionofavulnerablesubstrate
ilywithpartialunderstandingandrationalizationoftheunexpected (geneticoracquiredchangesintheelectricalormechanicalproper-
tragedy,whichfacilitatesthecopingprocessandallowsanunder- tiesoftheheart)withmultipletransientfactorsthatparticipatein
standingofwhethertheriskofsuddendeathmayextendtofamily triggeringthefatalevent.Inthenextsectionweprovideabriefover-
members.Accordingly,itappearsreasonablethatallunexplained viewofthepaucityofrisk-stratificationschemesforSCDinnormal
suddendeathvictimsundergopost-mortemexpertexamination subjects,inpatientswithischaemicheartdiseaseandinpatientswith
to investigate whether a cardiac origin should be suspected. channelopathiesandcardiomyopathies.
ESCGuidelines 2801
3.3.1 Individualswithoutknownheartdisease including,amongothers,programmedventricularstimulation(PVS),
Approximately50%ofcardiacarrestsoccurinindividualswithouta latepotentials,heartratevariability,baroreflexsensitivity,QTinterval
knownheartdisease,butmostsufferfromconcealedischaemicheart dispersion,microvoltT-wavealternansandheartrateturbulence.
disease.54Asaconsequence,themosteffectiveapproachtoprevent However, despite the promising outcomes of the early studies,
SCDinthegeneralpopulationresidesinquantificationoftheindivid- noneofthese‘predictors’hasinfluencedclinicalpractice.Asaconse-
ualriskofdevelopingischaemicheartdiseasebasedonriskscore quence,theonlyindicatorthathasconsistentlyshownanassociation
charts,followedbythecontrolofriskfactorssuchastotalserum withincreasedriskofsuddendeathinthesettingofmyocardialinfarc-
cholesterol,glucose,bloodpressure,smokingandbodymassindex.55 tion and left ventricular (LV) dysfunction is LV ejection fraction
Approximately40%oftheobservedreductioninSCDisthedirect (LVEF).63,64Thisvariablehasbeenusedformorethanadecadetotar-
consequenceofareductionofCADandothercardiacconditions.56 gettheuseofanimplantablecardioverterdefibrillator(ICD)forpri-
Severalstudies57–61haveprovidedevidencethatthereisagenet- marypreventionofSCD,oftenincombinationwithNewYorkHeart
icpredispositiontodiesuddenly.TheresearchgroupledbyX.Jou- Association (NYHA) class. Despite the fact that LVEF is not an
venwasoneofthefirsttoinvestigatethepredictivevalueoffamilial accurateandhighlyreproducibleclinicalparameter,itisstillusedto
recurrenceofsuddendeath.Theauthorsdemonstrated,intheParis selectpatientsforICDimplantationintheprimarypreventionofSCD.
studypublishedin1999,57thatoneparentalhistoryofsuddendeath Among emergingvariablesthat lookpromisingforpredicting
hadarelativerisk(RR)ofsuddendeathof1.89,whichincreasedto SCDarebiochemicalindicatorssuchastheB-typenatriureticpep-
9.44inthosewithtwoparentalhistoriesofsuddendeath(P¼0.01). tide and N-terminal pro-B-type natriureticpeptide,which have
Atthesametime,Friedlanderetal.58confirmed,inacase-basedco- shownencouragingresultsinpreliminaryinvestigations.65,66
D
hortstudyfromtheFraminghamstudy,analmost50%increase[RR ow
n
1.46(95%CI1.23,1.72)]inthelikelihoodofsuddendeathinthe 3.3.3 Patientswithinheritablearrhythmogenicdiseases lo
a
presenceofafamilyhistoryofSCD.In2006,Dekkeretal.59showed Theavailabilityofriskstratificationschemesishighlyheterogeneous de
d
thatfamilialsuddendeathoccurssignificantlymorefrequentlyinin- amongthedifferentchannelopathiesandcardiomyopathies:forex- fro
m
dividualsresuscitatedfromprimaryVFthanincontrols[oddsratio ample,whilethedurationofthecorrectedQT(QTc)intervalisa h
(OR)2.72(95%CI1.84,4.03)].Theimpressiveconsistencyofthese reliableindicatorofriskofcardiaceventsinlongQTsyndrome ttp
resultssuggeststhatthepredispositiontodiesuddenlyiswrittenin (LQTS),67andseptalhypertrophypredictsoutcomeinhypertroph- ://eu
the genes, even in the absence of a Mendelian disease, and en- iccardiomyopathy(HCM),68inotherdiseases,suchasBrugadasyn- rhe
a
couragesmolecularinvestigationstoidentifyDNAmarkerstopre- dromeorshortQTsyndrome(SQTS),riskstratificationmetricsare rtj.o
dictSCDinthegeneralpopulation. notrobust,leavinguncertaintiesonhowtotargettheprophylactic xfo
Amongthestudiesthathavesearchedforsinglenucleotidepoly- useoftheICD.Sofar,geneticinformationmaybeusedtoguiderisk rdjo
morphismsthatpredisposetoSCD,theresultsoftwogenome-wide stratificationonlyinafewdiseasessuchasLQTSandlaminA/Cdi- urn
associationstudies(GWAS)arerelevant:theArrhythmiaGeneticsin latedcardiomyopathy.69–71 als
.o
theNEtherlandS(AGNES)study,61whichinvolvedpatientswitha rg
firstmyocardialinfarctionandVFandcomparedthemwithacohort by/
3.4 Prevention of sudden cardiac death g
ofpatientswithafirstmyocardialinfarctionwithoutVF.Onlyonesin- u
e
glenucleotidepolymorphismlocatedinthe21q21locusachieved in special settings st o
n
genome-widesignificance,withanORof1.78(95%CI1.47,2.13; 3.4.1 Screeningthegeneralpopulationfortheriskof A
p
P¼3.36×10210).Thiscommonsinglenucleotidepolymorphism suddencardiacdeath ril 2
(47%frequencyoftheallele)isinanintergenicregionandtheclosest Vigilanceforelectrocardiographic(ECG)andechocardiographicsigns 0
gene,CXADR((cid:2)98kbaway),encodesaviralreceptorimplicatedin ofinheritablearrhythmogenicdiseasesseemstobeanimportantpart , 20
1
viralmyocarditis.ThesecondGWASstudy62wasaverylargestudy ofclinicalpracticeandcancontributetotheearlyidentificationof 6
thatidentifiedastrongsignalatthe2q24.2locus,whichcontainsthree patientsatriskofSCD.Whethersuchacarefulapproachshould
geneswithunknownfunctionthatareallexpressedintheheart.This beextendedtomassscreeninginpopulationsatriskofsuddendeath
locusincreasestheriskofSCDby1.92(95%CI1.57,2.34).Thestudy iscurrentlyunclear.ItalyandJapanhaveimplementedECGscreening
didnot,however,replicatetheresultsoftheAGNESstudy,raising systems,whichmayidentifyasymptomaticpatientswithinheritable
concernsthateitherthesizeorthedesignoftheAGNESstudypre- arrhythmogenic diseases.72–74 While consensus exists among
sentedlimitations.Thesegeneticdataarenotyetbeingappliedin expertsinEurope and the United States(US) thatsupportpre-
clinics,buttheyshowthatgeneticsmayevolveintoapromisingap- participation screening in athletes (an approach that has been
proachtoquantifytheriskofSCDearlyinlife.Theavailabilityofnovel endorsedbytheInternationalOlympicCommittee),75–77arecent
technologiesthatallowfasterandcheapergenotypingmaysoonpro- studyreportednochangeinincidenceratesofSCDincompetitive
videdataonverylargepopulationsanddeliverthestatisticalpower athletesfollowingimplementationofscreeningprogramsinIsrael.78
requiredfortheseinvestigations. Similarly,therearenocleardatasupportingthebenefitofbroad
screeningprogramsinthegeneralpopulation.Narainetal.79screened
3.3.2 Patientswithischaemicheartdisease 12000unselectedhealthyindividuals14–35yearsofage.Screening
Formorethantwodecadesinvestigatorsthroughouttheworldhave wasperformedatacostofGB£35perindividualandconsistedofa
envisionedabroadrangeof‘indicators’forSCDoccurringintheset- healthquestionnaire,12-leadECGandconsultationwithacardio-
tingofischaemicheartdisease.Severalnon-invasivemarkersofriskof logist.Individualswithabnormalitiesunderwentatransthoracicecho-
SCDhavebeenproposedforpatientswithmyocardialischaemia, cardiogramonthesamedayorwerereferredforfurtherevaluation.
2802 ESCGuidelines
Althoughthescreeningidentifiedonlyafewpatientswithinheritable Variousprotocolshavebeenproposedforscreeningfamilymem-
channelopathiesorcardiomyopathies(4/12000),theauthorscon- bersofsuddendeathvictims.14,91Theseprotocolsusuallyfollowa
cludedthatthecosttoidentifyindividualsatincreasedriskofSCD stepwiseapproach,startingwithlower-costandhigher-yieldinves-
mightstillsupportamass-screeningprogramme. tigationsandmovingontofurtherexaminationsbasedonboththe
Itisclearthatthecost–benefitassessmentofECGpopulation initialfindingsandthefamilyhistory.91Wheneveradiagnosisissus-
screeningisinfluencedlargelybythecostofidentifyingasingleaf- pected,basedonthepresenceofstructuralorelectricalabnormal-
fectedindividual.SuchacosthasnotbeendeterminedbytheItalian ities,thestandardprocedureforthediagnosisofthesuspected
nationalhealthcaresystemdespitethefactthatauniversalscreening diseaseshouldbefollowed.
programmehasbeeninplaceforthepast35years,andwillvaryde- Accuratehistorytakingisthefirststeptoreachapost-mortem
pendingontheregionalorganizationofhealthcare.TheUScostes- diagnosis,preliminarytoactiveexplorationofthefamilymembers.
timateforscreeningathletesrangesfromUS$300million–US$2 Whenthevictimisyoung,thefocusshouldbeoncardiomyopathies
billionperyearaccordingtoKaltmanetal.80 andchannelopathies.Theevaluationofpremonitorycardiacsymp-
Overall,we cannotproviderecommendationsforpopulation toms(includingsyncopeor‘epilepsy’),togetherwithanexhaustive
screeningatthistimebecausetheconsequencesofscreeningstrat- explorationofthe circumstancesofdeathandthecollectionof
egiesthatdetectastill-undefinednumberof‘falsepositives’andmiss ante-mortem clinical cardiac investigations, is recommended.
anunknownpercentageofaffectedcases(‘falsenegatives’)havenot Whenthevictimis .40yearsofage,thepresenceofriskfactors
beenestablished.Thisinabilitytoderivearecommendationfrom forCADshouldbeassessed(e.g.activeorpassivesmoking,dyslipo-
theevidenceobtainedfromexistingscreeningprogrammesillus- proteinaemia,hypertensionordiabetes).Inaddition,acomplete
D
tratesthe need forfurtherworktocollectquantitativedataon three-generationpedigreeshouldbecreated,recordingallsudden ow
thecost–benefitprofileofperformingECGscreeningindifferent deathsandcardiacdiseases.14Effortstoretrieveoldmedicalre- nlo
a
populationsandindifferenthealthcaresystemsandsettings.Con- cordsand/orpost-mortemexaminationsshouldbemade.Family de
d
versely,inconsiderationofthehigherriskofarrhythmiasandthe memberswithsymptomssuggestiveofthepresenceofacardiac fro
m
worseningofstructuralorgeneticdiseasesinindividualsexposed condition,suchassyncope,palpitationsorchestpain,shouldbe h
tointensephysicalexercise,81,82wedosupporttheexistingrecom- prioritizedforevaluation. ttp
mendationsforpre-participationscreeninginathletes.InEurope Therecommendedcoreevaluationofafirst-degreerelativeofa ://e
u
thereisconsensusthatclinicalevaluation,personalorfamilyhistory suddendeathvictimisillustratedinTable4.Intheabsenceofadiag- rhe
a
taking and a baseline 12-lead ECG should be performed in this nosisinthefamily,veryyoungchildrenshouldbescreenedatleast rtj.o
population(refertosection12.7). withabaselineECGandanechocardiogram. xfo
Asmanyinheritablearrhythmogenicdiseasesarecharacterized rd
jo
3.4.2 Screeningfamilymembersofsuddendeathvictims byage-relatedpenetranceandincompleteexpression,youngerin- urn
a
Thediagnosisofaninheritablearrhythmogenicdisorderisestab- dividualsshouldbefollowed-upatregularintervals.Asymptomatic ls
.o
lishedinupto50%83offamilieswithaSADSvictim,especiallychan- andfullygrownadultscanbedischargedfromcareunlesssymp- rg
nelopathies[e.g.LQTS,Brugadasyndromeandcatecholaminergic toms appear or new information from the family becomes by/
g
polymorphic ventricular tachycardia (CPVT)] and occasionally available. u
e
s
subtleformsofcardiomyopathy[HCMandarrhythmogenicright Whenaninheritablearrhythmogenicdiseaseissuspected,DNA t o
n
ventricularcardiomyopathy(ARVC)inparticular]orfamilialhyper- samplesfromthevictimarethebestsourceofinformationwhen A
p
cholesterolaemia.As aconsequence of these findings, when an performingamolecularautopsy.Ifthereisapositiveresult,family ril 2
autopsyiseithernotavailableforthevictim(i.e.SUDSorSUDI) membersshouldbeofferedtheopportunitytoundergopredictive 0
, 2
and/orwhenthepost-mortemexaminationfailstodetectstructural geneticscreening,inacascadefashion.The‘rightnottoknow’and 0
1
6
abnormalitiesandtoxicologyresultsarenormal(i.e.SADSorSIDS), thepossibilitytodeclinemolecularscreeningshouldbeincludedin
first-degreerelativesofthevictimshouldbeinformedofthepoten- anypre-informativecommunicationwiththerelatives.
tialriskofsimilareventstothemselvesandshouldundergocardiac Intheabsenceofbiologicalsamplesfromthedeceasedperson,
evaluation.AfamilyhistoryofrecurrentprematureSUDSorinher- targetedmolecularscreeninginfirst-degreerelativesmaybeconsid-
itableheartdiseaserepresentsa‘redflag’thatmakesfamilialevalu- eredwhenthereisthesuspicionofthepresenceofaninheritable
ationstronglyrecommended. diseaseinfamilymembers.Conversely,geneticscreeningofalarge
Familyscreeningoffirst-degreerelativesofvictimsofsudden panelofgenesshouldnotbeperformedinSUDSorSADSrelatives
deathisanimportantinterventiontoidentifyindividualsatrisk,ad- withoutclinicalcluesforaspecificdiseaseafterclinicalevaluation.
vise on available treatment and adequately prevent sudden ThisisespeciallytrueinSIDScases,wheremolecularautopsyiden-
death.14,84Currentlyonly40%offamilymembersarescreened,85 tifiesalowerburdenofionchanneldiseasecomparedwithSADS
partiallydue to a lackof adequate screening infrastructure, but andsporadicgeneticdiseaseasacauseofsuddendeathmaybe
alsoduetotheanxietyanddistressassociatedwiththepersonalex- morefrequent.
perienceofalife-threateningarrhythmiaorarecentfamilybereave-
mentfromaninheritablecardiaccondition.86,87Thepsychosocial 3.4.3 Screeningpatientswithdocumentedorsuspected
needsofthesepatientsandtheirfamiliesshouldbeevaluatedand ventriculararrhythmias
amultidisciplinaryapproachwithinspecializedcentresshouldbe 3.4.3.1 Clinicalhistory
followed,asrecentlyrecommended.14,84,88Thevalueofthisap- Palpitations(orsensationofsuddenrapidheartbeats),presyncope
proachhasbeendemonstrated.89,90 and syncope are the three most important symptoms that