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

87 Pages·2015·2.03 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
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The Task Force for the Management of Patients with Ventricular ow
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Arrhythmias and the Prevention of Sudden Cardiac Death of the a
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European Society of Cardiology (ESC) fro
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Endorsed by: Association for European Paediatric and Congenital ://a
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Cardiology (AEPC) d
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Authors/Task Force Members: Silvia G. Priori*(Chairperson) (Italy) up
.c
Carina Blomstro¨m-Lundqvist*(Co-chairperson) (Sweden) Andrea Mazzanti† (Italy), om
/e
Nico Bloma (The Netherlands), Martin Borggrefe (Germany), John Camm (UK), urh
e
Perry Mark Elliott (UK), Donna Fitzsimons (UK), Robert Hatala (Slovakia), artj/a
Gerhard Hindricks (Germany), Paulus Kirchhof (UK/Germany), Keld Kjeldsen rtic
le
(Denmark), Karl-Heinz Kuck (Germany), Antonio Hernandez-Madrid (Spain), -a
b
s
Nikolaos Nikolaou (Greece), Tone M. Norekva˚l (Norway), Christian Spaulding tra
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(France), and Dirk J. Van Veldhuisen (The Netherlands) t/3
6
/4
1
/2
7
9
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*Correspondingauthors:SilviaGiulianaPriori,DepartmentofMolecularMedicineUniversityofPavia,Cardiology&MolecularCardiology,IRCCSFondazioneSalvatoreMaugeri, /2
ViaSalvatoreMaugeri10/10A,IT-27100Pavia,Italy,Tel:[phone],Fax:[phone],Email:[email protected] 29
CarinaBlomstro¨m-Lundqvist,DepartmentofCardiology,InstitutionofMedicalScience,UppsalaUniversity,SE-75185Uppsala,Sweden,Tel:[phone],Fax:[phone], 33
6
Email:[email protected] 3
aRepresentingtheAssociationforEuropeanPaediatricandCongenitalCardiology(AEPC). by
†AndreaMazzanti:Coordinator,affiliationlistedintheAppendix. gu
e
ESCCommitteeforPracticeGuidelines(CPG)andNationalCardiacSocietiesdocumentreviewers:listedintheAppendix. st o
ESCentitieshavingparticipatedinthedevelopmentofthisdocument: n
0
ESCAssociations:AcuteCardiovascularCareAssociation(ACCA),EuropeanAssociationofCardiovascularImaging(EACVI),EuropeanAssociationofPercutaneousCardiovascular 6
Interventions(EAPCI),EuropeanHeartRhythmAssociation(EHRA),HeartFailureAssociation(HFA). Ap
ESCCouncils:CouncilforCardiologyPractice(CCP),CouncilonCardiovascularNursingandAlliedProfessions(CCNAP),CouncilonCardiovascularPrimaryCare(CCPC), ril 2
CouncilonHypertension. 0
1
ESCWorkingGroups:CardiacCellularElectrophysiology,CardiovascularPharmacotherapy,CardiovascularSurgery,Grown-upCongenitalHeartDisease,Myocardialand 9
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 ad
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† Defibrillator † Guidelines † Heartfailure † Implantablecardioverterdefibrillator † Myocardialinfarction d
† Resuscitation † Stablecoronaryarterydisease † Suddencardiacdeath † Tachycardia † Valvularheart fro
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disease † Ventriculararrhythmia h
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Table of Contents 4.2.3 Patientswithacardioverterdefibrillator . . . . . . . .2809 ic
.o
4.2.4 Electrolytes . . . . . . . . . . . . . . . . . . . . . . . . . . .2809 u
p
Abbreviationsandacronyms . . . . . . . . . . . . . . . . . . . . . . . .2796 4.2.5 Otherdrugtherapy. . . . . . . . . . . . . . . . . . . . . .2809 .co
m
1. Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2797 4.3 Devicetherapy . . . . . . . . . . . . . . . . . . . . . . . . . . .2809 /e
2. Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2798 4.3.1 Implantablecardioverterdefibrillator . . . . . . . . . .2809 urh
e
2.1 Structureoftheguidelines . . . . . . . . . . . . . . . . . . . .2799 4.3.1.1 Secondarypreventionofsuddencardiacdeath a
3. Definitions,epidemiologyandfutureperspectivesforthe andventriculartachycardia . . . . . . . . . . . . . . . . . . .2810 rtj/a
preventionofsuddencardiacdeath. . . . . . . . . . . . . . . . . . . .2799 4.3.2 Subcutaneousimplantablecardioverter rtic
le
3.1 Epidemiologyofsuddencardiacdeath. . . . . . . . . . . . .2799 defibrillator . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2810 -a
b
3.1.1 Causesofsuddencardiacdeathindifferentage 4.3.3 Wearablecardioverterdefibrillator . . . . . . . . . . .2811 stra
groups . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2800 4.3.4 Publicaccessdefibrillation . . . . . . . . . . . . . . . . .2811 c
3.2 Autopsyandmolecularautopsyinsuddendeathvictims.2800 4.4 Acutetreatementofsustainedventriculararrhythmias . .2812 t/36
/4
3.3 Riskpredictionofsuddencardiacdeath . . . . . . . . . . .2800 4.5 Interventionaltherapy . . . . . . . . . . . . . . . . . . . . . . .2814 1
/2
3.3.1 Individualswithoutknownheartdisease . . . . . . . .2801 4.5.1 Catheterablation . . . . . . . . . . . . . . . . . . . . . . .2814 7
9
3
3.3.2 Patientswithischaemicheartdisease . . . . . . . . . .2801 4.5.1.1 Patientswithscar-relatedheartdisease . . . . . .2814 /2
2
3.3.3 Patientswithinheritablearrhythmogenicdiseases . .2801 4.5.1.2 Patientswithoutovertstructuralheartdisease .2814 9
3
3.4 Preventionofsuddencardiacdeathinspecialsettings . .2801 4.5.2 Anti-arrhythmicsurgery. . . . . . . . . . . . . . . . . . .2815 36
3
3.4.1 Screeningthegeneralpopulationfortheriskof 4.6 Psychosocialimpactofimplantablecardioverter b
y
suddencardiacdeath. . . . . . . . . . . . . . . . . . . . . . . . .2801 defibrillatortreatment . . . . . . . . . . . . . . . . . . . . . . . . .2815 gu
e
3.4.2 Screeningfamilymembersofsuddendeath 5. Managementofventriculararrhythmiasandpreventionof s
victims . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2802 suddencardiacdeathincoronaryarterydisease . . . . . . . . . . .2816 t on
0
3.4.3 Screeningpatientswithdocumentedorsuspected 5.1 Acutecoronarysyndromes . . . . . . . . . . . . . . . . . . .2816 6
A
ventriculararrhythmias . . . . . . . . . . . . . . . . . . . . . . .2802 5.1.1 Ventriculararrhythmiasassociatedwithacute p
3.4.3.1 Clinicalhistory . . . . . . . . . . . . . . . . . . . . . .2802 coronarysyndromes . . . . . . . . . . . . . . . . . . . . . . . .2816 ril 2
0
3.4.3.2 Non-invasiveandinvasiveevaluation. . . . . . . .2803 5.1.2 Preventionandmanagementofsuddencardiacdeath 19
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 D
o
ventriculardysfunctionbeforeandafterdischarge . . . . . .2819 7.3.1 Definitions,epidemiology,andsurvival . . . . . . . . .2831 w
n
5.3 Stablecoronaryarterydiseaseaftermyocardialinfarction 7.3.2 Approachtoriskstratificationandmanagement . . . .39 loa
d
withpreservedejectionfraction . . . . . . . . . . . . . . . . . . .2820 7.3.3 Ventriculararrhythmiasinarrhythmogenicright e
d
5.3.1 Riskstratification . . . . . . . . . . . . . . . . . . . . . . .2820 ventricularcardiomyopathy . . . . . . . . . . . . . . . . . . . .2831 fro
m
5.3.2 Recommendationsforoptimalstrategy . . . . . . . . .2820 7.3.3.1 Treatmentofventriculararrhythmia . . . . . . .2832 h
5.3.3 Useofanti-arrhythmicdrugs. . . . . . . . . . . . . . . .2820 7.3.3.2 Exerciserestriction . . . . . . . . . . . . . . . . . . .2832 ttp
s
5.3.4 Catheterablation . . . . . . . . . . . . . . . . . . . . . . .2821 7.3.3.3 Implantablecardioverterdefibrillators. . . . . . .2832 ://a
c
6. Therapiesforpatientswithleftventriculardysfunction,withor 7.4 Infiltrativecardiomyopathies . . . . . . . . . . . . . . . . . . .2832 a
d
withoutheartfailure . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2821 7.4.1 Cardiacamyloidosis . . . . . . . . . . . . . . . . . . . . .2832 em
6.1 Primarypreventionofsuddencardiacdeath. . . . . . . . .2821 7.5 Restrictivecardiomyopathy. . . . . . . . . . . . . . . . . . . .2832 ic.o
u
6.1.1 Drugs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2821 7.6 Othercardiomyopathies . . . . . . . . . . . . . . . . . . . . .2833 p
.c
6.1.2 Implantablecardioverterdefibrillators. . . . . . . . . .2822 7.6.1 Left-ventricularnon-compaction . . . . . . . . . . . . .2833 o
m
6.1.3 Implantablecardioverterdefibrillatorsinpatientswith 7.6.2 Chagas’cardiomyopathy. . . . . . . . . . . . . . . . . . .2833 /e
u
NewYorkHeartAssociationclassIVlistedforheart 8. Inheritedprimaryarrhythmiasyndromes . . . . . . . . . . . . . .2833 rh
e
t6r.a1n.4spClaanrtdatiaiocnre.s.yn.c.h.ro.n.iz.a.ti.o.n.th.e.r.ap.y. .. .. .. .. .. .. .. .. .. .. .. ..22882233 8.18.L1o.1ngDQefiTnsityiondnrsoamndeep.i.de.m. i.o.lo.g.y. .. .. .. .. .. .. .. .. .. .. .. .. .. .. ..22883333 artj/a
6.1.4.1 Heartfailurewithreducedleftventricular 8.1.2 Approachtoriskstratificationandmanagement . . .2834 rticle
ejectionfractionandNewYorkHeartAssociationclass 8.2 ShortQTsyndrome . . . . . . . . . . . . . . . . . . . . . . . .2835 -a
b
s
III/ambulatoryclassIV . . . . . . . . . . . . . . . . . . . . . .2823 8.2.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2835 tra
6.1.4.2 Heartfailurewithreducedleftventricular 8.2.2 Approachtoriskstratificationandmanagement . . .2835 ct/3
ejectionfractionbutmildsymptoms(NewYorkHeart 8.3 Brugadasyndrome . . . . . . . . . . . . . . . . . . . . . . . . .2836 6/4
AssociationclassII) . . . . . . . . . . . . . . . . . . . . . . . .2825 8.3.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2836 1/2
7
6.2 Prematureventricularcomplexesinpatientswith 8.3.2 Approachtoriskstratificationandmanagement . . .2836 9
3
structuralheartdisease/leftventriculardysfunction . . . . . . .2825 8.4 Catecholaminergicpolymorphicventriculartachycardia .2837 /2
2
9
6.3 Sustainedventriculartachycardia . . . . . . . . . . . . . . . .2825 8.4.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2837 3
3
6.3.1 Drugtherapy. . . . . . . . . . . . . . . . . . . . . . . . . .2825 8.4.2 Approachtoriskstratificationandmanagement . . .2837 63
6.3.2 Catheterablation . . . . . . . . . . . . . . . . . . . . . . .2826 8.5 Earlyrepolarizationsyndrome. . . . . . . . . . . . . . . . . .2838 by
g
6.3.2.1 Patientswithleftventriculardysfunction . . . . .2826 8.5.1 Definitionsandepidemiology . . . . . . . . . . . . . . .2838 u
e
6.3.2.2 Bundlebranchre-entranttachycardia . . . . . . .2827 9. Paediatricarrhythmiasandcongenitalheartdisease . . . . . . .2838 st o
6.3.3 Implantablecardioverterdefibrillator . . . . . . . . . .2827 9.1 Managementofventriculararrhythmiasinchildrenwitha n 0
7. Cardiomyopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2827 structurallynormalheart . . . . . . . . . . . . . . . . . . . . . . . .2838 6 A
7.17.D1.i1latDedeficnairtdioionms,yeoppidaethmyio.l.o.gy.,.an.d. .su.r.vi.va.l.d.at.a. .. .. .. .. .. ..22882277 9p.a2tieSnutdsdweinthcacrodniagcenditeaalthheaanrdtdviesnetarsiecu.la.r.a.rr.h.y.th.m.ia.s.i.n. . . .2839 pril 20
1
7.1.2 Approachtoriskstratificationandmanagement . . .2827 9.3 Implantablecardioverterdefibrillatortherapyinpaediatric 9
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 D
o
11.2 Endocarditis. . . . . . . . . . . . . . . . . . . . . . . . . . . . .2846 andasymptomaticnon-sustainedventricular w
n
11.3 Rheumaticheartdisease. . . . . . . . . . . . . . . . . . . . .2846 tachycardia loa
11.4 Pericarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2846 ARB angiotensinIIreceptorblocker de
d
11.5 Cardiacsarcoidosis . . . . . . . . . . . . . . . . . . . . . . . .2846 ARVC arrhythmogenicrightventricularcardiomyopathy fro
11.6 Valvularheartdisease . . . . . . . . . . . . . . . . . . . . . .2847 AV atrio-ventricular m h
12. Arrhythmicriskinselectedpopulations . . . . . . . . . . . . . .2847 AVID Antiarrhythmic drugs Versus Implantable ttp
s
12.1 Psychiatricpatients . . . . . . . . . . . . . . . . . . . . . . . .2847 Defibrillator ://a
12.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . .2848 BrS BrugadaSyndrome ca
d
12.1.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . .2848 CAD coronaryarterydisease em
12.1.3 Treatment. . . . . . . . . . . . . . . . . . . . . . . . . . .2848 CARE-HF CArdiacREsynchronization – HeartFailure ic.o
12.2 Neurologicalpatients. . . . . . . . . . . . . . . . . . . . . . .2849 CASH CardiacArrestStudyHamburg up
12.2.1 Suddenunexplaineddeathinepilepsy . . . . . . . . .2849 CAST CardiacArrhythmiaSuppressionTrial .co
m
12.2.2 Neuromusculardisorders . . . . . . . . . . . . . . . . .2849 CAT CArdiomyopathyTrial /e
u
12.3 Pregnantpatients . . . . . . . . . . . . . . . . . . . . . . . . .2850 CHD congenitalheartdisease rh
e
1ca2r.3d.i1omAyrorphaytthhymi.as. .no.t.r.e.la.te.d.t.o.p.e.ri.p.ar.tu.m. . . . . . . . . .2850 CCIIDS cCoannfiaddeiannceIminptleanrvtaablleDefibrillatorStudy artj/a
12.3.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . .2850 CMR cardiacmagneticresonance rticle
12.3.1.2 Diagnosis. . . . . . . . . . . . . . . . . . . . . . . . .2851 COMPANION ComparisonofMedicalTherapy,Pacing,and -a
b
12.3.1.3 Treatment . . . . . . . . . . . . . . . . . . . . . . .2851 DefibrillationinHeartFailure stra
12.3.2 Arrhythmiasrelatedtoperipartumcardiomyopathy2851 CPG CommitteeforPracticeGuidelines ct/3
12.4 Obstructivesleepapnoea. . . . . . . . . . . . . . . . . . . .2852 CPVT catecholaminergic polymorphic ventricular 6/4
12.4.1 Bradyarrhythmiasand –tachyarrhythmias. . . . . . .2852 tachycardia 1/2
CRT cardiacresynchronizationtherapy 7
12.4.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . .2852 9
3
12.4.1.2 Diagnosis. . . . . . . . . . . . . . . . . . . . . . . . .2852 CRT-D cardiacresynchronizationtherapydefibrillator /2
2
12.4.1.3 Treatment . . . . . . . . . . . . . . . . . . . . . . . .2852 CRT-P cardiacresynchronizationtherapypacemaker 93
3
12.5 Drug-relatedpro-arrhythmia. . . . . . . . . . . . . . . . . .2852 CT computedtomography 63
12.5.1 Drug–substrateinteraction,duetounderlying DCM dilatedcardiomyopathy by
diseasesubstrate . . . . . . . . . . . . . . . . . . . . . . . . . . .2852 DEFINITE DEFIbrillatorsinNon-Ischemiccardiomyop- gu
e
12.5.2 Drug–druginteraction(duetospecificdrugsand athyTreatmentEvaluation st o
combinations) . . . . . . . . . . . . . . . . . . . . . . . . . . . . .2853 DFT defibrillationthreshold n 0
12.5.3 Pro-arrhythmicriskofanti-arrhythmicdrugs. . . . .2853 DIAMOND Danish Investigators of Arrhythmia and 6 A
12.162.S5u.4ddPerno-caarrrdhiyatchdmeiaathduaeftetorhtreiagrgtertirnagnsfapclatnotrastio.n. . .. .. .. ..22885533 ECG MeleocrttraolictyarodNiogDraomfet/ileidleectrocardiographic pril 20
12.7 Suddencardiacdeathinathletes . . . . . . . . . . . . . . .2853 EHRA EuropeanHeartRhythmAssociation 19
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 D
o
w
LVOT leftventricularoutflowtract 1. Preamble n
lo
MADIT MulticenterAutomaticDefibrillatorImplant- a
d
ationTrial Guidelinessummarizeandevaluateallavailableevidenceonapar- ed
MIRACLE Multicenter InSync Randomized Clinical ticularissueatthetimeofthewritingprocess,withtheaimofassist- fro
m
Evaluation inghealthprofessionalsinselectingthebestmanagementstrategies h
MRA mineralocorticoidreceptorantagonist foranindividualpatientwithagivencondition,takingintoaccount ttp
s
ms millisecond theimpactonoutcome,aswellastherisk–benefitratioofparticu- ://a
c
MUSTT MulticenterUnSustainedTachycardiaTrial lardiagnosticortherapeuticmeans.Guidelinesandrecommenda- a
d
e
NSTEMI non–ST-segment elevation myocardial tionsshouldhelphealthprofessionalstomakedecisionsintheir m
infarction dailypractice.However,thefinaldecisionsconcerninganindividual ic.o
u
NSVT non-sustainedventriculartachycardia patientmustbemadebytheresponsiblehealthprofessional(s)in p
.c
NYHA NewYorkHeartAssociation consultationwiththepatientandcaregiverasappropriate. o
m
OPTIC OptimalPharmacologicalTherapyInCardio- AgreatnumberofGuidelineshavebeenissuedinrecentyearsby /e
u
verterdefibrillatorpatients theEuropeanSocietyofCardiology(ESC)aswellasbyothersoci- rh
e
OOPRRTESERVE-EF ooridusktdflssotrwraattitoirfiaccattioninpatientswithpreserved eqlistuhiaeelsidtayinncdroiotredrregiaarntfioosarmttiaohkenesd.aeBllvedecelaocupissmieoenonsftttrohafengsiumpiadpreaelcnintteotsonhtcahlveineuicbsaeelrep.nTraehcsettiarcbee--, artj/article
ejectionfraction commendationsforformulatingandissuingESCGuidelinescanbe -ab
s
PVC prematureventricularcomplex foundontheESCwebsite(http://www.escardio.org/Guidelines- tra
c
PVS programmedventricularstimulation &-Education/Clinical-Practice-Guidelines/Guidelines-development/ t/3
QTc correctedQT Writing-ESC-Guidelines).ESCGuidelinesrepresenttheofficialpos- 6/4
1
RAFT Resynchronization–DefibrillationforAmbu- itionoftheESConagiventopicandareregularlyupdated. /2
7
latoryHeartFailureTrial MembersofthisTaskForcewereselectedbytheESCtore- 9
3
RBBB rightbundlebranchblock presentprofessionalsinvolvedwiththemedicalcareofpatients /2
2
9
RCT randomizedcontrolledtrial with this pathology. Selected experts in the field undertook a 3
3
REVERSE REsynchronizationreVErsesRemodeling in comprehensivereviewofthepublishedevidenceformanagement 63
b
SystolicleftvEntriculardysfunction (includingdiagnosis,treatment,preventionandrehabilitation)of y
g
REVERSEMIRACLE MulticenterInSyncICDRandomizedClinical a given condition according to ESC Committee for Practice u
e
s
ICD Evaluation Guidelines (CPG) policy.A critical evaluationof diagnosticand t o
RR relativerisk therapeuticprocedureswasperformed,includingassessmentof n 0
6
RV rightventricular therisk–benefitratio.Estimatesofexpectedhealthoutcomesfor A
p
RSAV-OECTG rsiigghntalv-aevnetrraicgueldarEoCuGtflowtract leavrigdeernpceopaunldattiohnesswtreernegtihncolufdtehde,rwechoemremdeantadaetxioisnt.oTfhpealretviceullaorf ril 20
1
SADS suddenarrhythmicdeathsyndrome managementoptionswereweighedandgradedaccordingtoprede- 9
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. D
o
w
Class IIa Weight of evidence/opinion is in Should be considered n
lo
favour of usefulness/efficacy. a
d
e
Class IIb Uessteafbullinsheessd/ ebfyfi ceavciyd eisn clees/so pwienlilon. May be considered d fro
m
h
Class III Evidence or general agreement Is not recommended ttp
that the given treatment or s
procedure is not useful/effective, ://a
c
and in some cases may be harmful. a
d
e
m
ic
.o
u
p
.c
o
m
panels.TheCommitteeisalsoresponsiblefortheendorsement consultationwiththatpatientandthepatient’scaregiverwhereap- /e
u
processoftheseGuidelines.TheESCGuidelinesundergoextensive propriateand/ornecessary.Itisalsothehealthprofessional’sre- rh
e
rseiovnieswthbeyGthueidCelPinGesanadreeaxpteprrnoavleedxbpyeratlsl.tAhefteerxappeprtrsopinrviaotleverdeviin- sapnodndseibviilciteystaottvheeritfyimteheofruplreesscarnidptrioengu.lationsapplicabletodrugs artj/a
theTaskForce.ThefinalizeddocumentisapprovedbytheCPG rticle
for publication in the European Heart Journal. The Guidelines -a
b
s
weredevelopedaftercarefulconsiderationofthescientificand Table2 Levelsofevidence tra
medical knowledge and the evidence available at the time of ct/3
theirdating. Level of Data derived from multiple randomized 6/4
ThetaskofdevelopingESCGuidelinescoversnotonlyintegra- evidence A clinical trials or meta-analyses. 1/2
7
tailotnooolfstahnedmimosptlermeceennttarteiosneaprrcohg,rbaumtmalseosftohretchreeareticoonmomfeednudcaattioionns-. Level of Dcliantiac adle trriivael do rfr loamrg ea nsionng-lrea rnadnodmomizeizde d 93/2
evidence B 2
Toimplementtheguidelines,condensedpocketguidelinesversions, studies. 93
3
summaryslides,bookletswithessentialmessages,summarycards Consensus of opinion of the experts and/ 63
f(osmrnarotnp-hsopneecisa,liesttcs.,)aanrdeapnroedleuccterdo.nTichveesresivoenrsfioornsdiagritealabapripdlgiceadtiaonnds Leveivdeeln ocfe C orerg simstrailel ss.tudies, retrospective studies, by gu
e
thus,ifneeded,oneshouldalwaysrefertothefulltextversion, st o
whichisfreelyavailableontheESCwebsite.TheNationalSocieties n 0
oftheESCareencouragedtoendorse,translateandimplementall 6 A
EhSasCbGeeunidsehlionwesn.Imthpatletmheenotuattcioonmperoogfrdaimsemaseesmaraeynbeeedfaevdobuercaabulyseini-t 2. Introduction pril 20
1
fluencedbythethoroughapplicationofclinicalrecommendations. ThepresentdocumenthasbeenconceivedastheEuropeanupdate 9
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 D
sionally,however,wordingchangesweremadetorendersomeof o
w
theoriginalrecommendationsmoreuserfriendlyandprecise. Inthepast20years,cardiovascularmortalityhasdecreasedinhigh- n
lo
The committee was composed of physicians and associated income countries19 in response to the adoption of preventive ad
e
healthcare providers who are experts in the areas of SCD and measurestoreduce the burdenofCAD andHF.Despite these d
prevention,complexVA,interventionalelectrophysiology,coron- encouragingresults,cardiovasculardiseasesareresponsibleforap- from
ary artery disease (CAD), HF and cardiomyopathy, paediatric proximately17milliondeathseveryyearintheworld,approximate- h
cardiologyandarrhythmias,devicetherapy,cardiovascularcare,car- ly25%ofwhichareSCD.20TheriskofSCDishigherinmenthanin ttps
diovasculargeneticsandnursing.Expertsindifferentsubspecialties women,anditincreaseswithageduetothehigherprevalenceof ://a
c
incardiologywereidentifiedwiththehelpoftherelatedworking CADinolderage.21Accordingly,theSCDrateisestimatedtorange ad
e
groupsoftheESC. from1.40per100000person-years[95%confidenceinterval(CI) m
ic
Allmembersofthewritingcommitteeapprovedtheguidelinere- 0.95,1.98]inwomento6.68per100000person-years(95%CI .o
u
commendations.Seventy-fourpeerreviewersreviewedthedocu- 6.24,7.14)inmen.21SCDinyoungerindividualshasanestimatedin- p.c
ment.Anextensiveliteraturesurvey wasconductedthatled to cidenceof0.46–3.7eventsper100000person-years,22,23corre- om
theincorporationof810references.Theguidelinesreviewedcon- spondingtoaroughestimateof1100–9000deathsinEuropeand /eu
cerningpreventionofSCDarelistedinWebTable1.3–13 800–6200deathsintheUSAeveryyear.24 rhe
a
rtj/a
rtic
le
-a
Table3 Definitionsofcommonlyusedterms bs
tra
c
Term Refa t/3
6
/4
Sudden death Non-traumatic, unexpected fatal event occurring within 1 hour of the onset of symptoms in an apparently healthy 1 1/2
subject. 7
9
If death is not witnessed, the applies when the victim was in good health 24 hours before the event. 3
/2
2
SUDS and SUDI Sudden death without an apparent cause and in which an autopsy has not been performed in an adult (SUDS) or in an 14 9
3
infant <1 year of age (SUDI). 3
6
3
SCD The term is used when: 1, 14, by
(cid:129)A congenital, or acquired, potentially fatal cardiac condition was known to be present during life; OR 15 g
u
(cid:129)Autopsyhas a cardiac or vascular anomaly as the probable cause of the event; OR e
s
(cid:129)Noobviousextra-cardiac causes have been by post-mortem examination and therefore an arrhythmic event t o
is a likely cause of death. n 0
6
SADS and SIDS Both autopsy and toxicology investigations are inconclusive, the heart is structurally normal at gross and histological 16 A
p
examination and non-cardiac aetiologies are excluded in adults (SADS) and in infants (SIDS). ril 2
0
Aborted cardiac Unexpected circulatory arrest, occurring within 1 hour of onset of acute symptoms, which is reversed by successful - 1
9
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 D
o
malitiesareepidemiologicallyrelevantinthisagegroup. autopsy,targetedguidelineshavebeendevelopedtodefineproto- w
n
colsforheartexaminationandhistologicalsampling,aswellasfor loa
d
3.2 Autopsy and molecular autopsy in toxicologyandmolecularinvestigation.17,50Overall,aproperlycon- ed
sudden death victims ducted autopsyshould provide answerstothe following issues: fro
m
(i)whetherthedeathisattributabletoacardiacdisease,(ii)thena- h
tureofthecardiacdisease(ifpresent),(iii)whetherthemechanism ttp
s
Indicationsforautopsyandmolecularautopsyin ofdeathwasarrhythmic,(iv)whetherthereisevidenceofacardiac ://a
suddendeathvictims diseasethatmaybeinheritedandthusrequiresscreeningandcoun- ca
d
sellingofrelativesand(v)thepossibilityoftoxicorillicitdruguseor em
Recommendations Classa Levelb Ref.c othercausesofunnaturaldeaths. ic.o
u
A standard histological examination of the heart should p
Anautopsyisrecommendedto .c
includemappedlabelledblocksofmyocardiumfromrepresentative o
investigatethecausesofsudden m
deathandtodefinewhetherSCDis transverseslicesofbothventricles.Weencouragepathologiststo /eu
secondarytoarrhythmicor I C 17 contactspecializedcentresandsendthehearttothemforexamin- rh
e
nruopnt-uarrerhoyfthamniacomrteicchananeiusrmyssm(e)..g. aotfiothne.Thheearpta,tihnoclluodgiisntgsahotrualdnspveerrfsoermapiacasltasnedcatirodng,raonsdsteaxkaemtiinsastuieosn, artj/a
Wheneveranautopsyisperformed, bloodandotherfluidsfortoxicologyandmolecularpathologybefore rticle
astandardhistologicalexaminationof fixingtheheartinformalin.Furthermore,thecollectionandstorage -a
b
theheartisrecommendedandit s
sbhloocukldsoinfcmluydoecmaradpiupmedfrlaobmelled I C 17 oofpsbyioilsoegniccaolusaramgpedle.s17foMroDleNcuAlaerxaturtaocptisoynistoanallimowpoart‘manotleacdudliatiro’nautto- tract/3
representativetransverseslicesof thestandardautopsy,asitallowsthediagnosispost-mortemofthe 6/4
bothventricles. presenceofcardiacchannelopathiesthatmayexplain15–25%ofsud- 1/2
Theanalysisofbloodandother denarrhythmicdeathsyndrome(SADS)cases.17Thevalueofthe 793
adequatelycollectedbodyfluidsfor post-mortemdiagnosisinavictimofSCDliesinextendinggenetic /2
2
toxicologyandmolecularpathologyis I C 17 screeningtothefamilymembersofSADSorSIDSvictims.Recentex- 93
3
recommendedinallvictimsof pertconsensusdocumentsforthediagnosisandmanagementofin- 63
unexplainedsuddendeath. heritable arrhythmias state that the use of a focused molecular by
Targetedpost-mortemgenetic autopsy/post-mortem genetic testing should be considered for gu
agnenaleyssisshoofupldobteenctoianllsyiddeisreeadsien-caallussuindgden 17,50, SCDvictimswhenthepresenceofchannelopathiesissuspected. est o
deathvictimsinwhomaspecific IIa C 51 Weendorsethisrecommendationandreferinterestedreadersto n 0
inheritablechannelopathyor themostrecentconsensusdocumentsonthistopic.14,52 6 A
cardiomyopathyissuspected. 3.3 Risk prediction of sudden cardiac pril 2
0
death 1
SCD¼suddencardiacdeath. 9
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 D
o
venwasoneofthefirsttoinvestigatethepredictivevalueoffamilial accurateandhighlyreproducibleclinicalparameter,itisstillusedto w
n
recurrenceofsuddendeath.Theauthorsdemonstrated,intheParis selectpatientsforICDimplantationintheprimarypreventionofSCD. loa
d
studypublishedin1999,57thatoneparentalhistoryofsuddendeath Among emergingvariablesthat lookpromisingforpredicting e
d
hadarelativerisk(RR)ofsuddendeathof1.89,whichincreasedto SCDarebiochemicalindicatorssuchastheB-typenatriureticpep- fro
m
9.44inthosewithtwoparentalhistoriesofsuddendeath(P¼0.01). tide and N-terminal pro-B-type natriureticpeptide,which have h
Atthesametime,Friedlanderetal.58confirmed,inacase-basedco- shownencouragingresultsinpreliminaryinvestigations.65,66 ttp
s
hortstudyfromtheFraminghamstudy,analmost50%increase[RR ://a
c
1.46(95%CI1.23,1.72)]inthelikelihoodofsuddendeathinthe 3.3.3 Patientswithinheritablearrhythmogenicdiseases a
d
presenceofafamilyhistoryofSCD.In2006,Dekkeretal.59showed Theavailabilityofriskstratificationschemesishighlyheterogeneous em
thatfamilialsuddendeathoccurssignificantlymorefrequentlyinin- amongthedifferentchannelopathiesandcardiomyopathies:forex- ic.o
u
dividualsresuscitatedfromprimaryVFthanincontrols[oddsratio ample,whilethedurationofthecorrectedQT(QTc)intervalisa p
.c
(OR)2.72(95%CI1.84,4.03)].Theimpressiveconsistencyofthese reliableindicatorofriskofcardiaceventsinlongQTsyndrome o
m
resultssuggeststhatthepredispositiontodiesuddenlyiswrittenin (LQTS),67andseptalhypertrophypredictsoutcomeinhypertroph- /e
u
the genes, even in the absence of a Mendelian disease, and en- iccardiomyopathy(HCM),68inotherdiseases,suchasBrugadasyn- rh
e
cdoicuAtrmSaCgoeDnsgmitnhotelhesectuugldeairneeisnrtvahelaspttoihgpaauvtileoatnsioesantro.chideednftoifrysDinNglAenmuacrlekoertisdteopporley-- dnuosreotmorofebtohuerstsI,ChloeDra.tvSiQnogTfausrny,cngederrntoeamitnicetiie(nSsfoQornTmSha)ot,iworinstkomstatrayarbtgiefietcuatstheioednptomrogeputhridiycelsarcaitrsiekc artj/article
morphismsthatpredisposetoSCD,theresultsoftwogenome-wide stratificationonlyinafewdiseasessuchasLQTSandlaminA/Cdi- -ab
associationstudies(GWAS)arerelevant:theArrhythmiaGeneticsin latedcardiomyopathy.69–71 stra
theNEtherlandS(AGNES)study,61whichinvolvedpatientswitha ct/3
fiorfsptamtieynotcsawrditiahlainfifarsrtctmioynocaanrddViaFlinanfadrcctoiomnpwaritehdotuhteVmF.wOitnhlyaocnoehsoinr-t 3.4 Prevention of sudden cardiac death 6/41/2
glenucleotidepolymorphismlocatedinthe21q21locusachieved in special settings 79
3
genome-widesignificance,withanORof1.78(95%CI1.47,2.13; 3.4.1 Screeningthegeneralpopulationfortheriskof /2
2
P¼3.36×10210).Thiscommonsinglenucleotidepolymorphism suddencardiacdeath 93
3
(47%frequencyoftheallele)isinanintergenicregionandtheclosest Vigilanceforelectrocardiographic(ECG)andechocardiographicsigns 63
gene,CXADR((cid:2)98kbaway),encodesaviralreceptorimplicatedin ofinheritablearrhythmogenicdiseasesseemstobeanimportantpart by
viralmyocarditis.ThesecondGWASstudy62wasaverylargestudy ofclinicalpracticeandcancontributetotheearlyidentificationof gu
e
thatidentifiedastrongsignalatthe2q24.2locus,whichcontainsthree patientsatriskofSCD.Whethersuchacarefulapproachshould st o
geneswithunknownfunctionthatareallexpressedintheheart.This beextendedtomassscreeninginpopulationsatriskofsuddendeath n 0
locusincreasestheriskofSCDby1.92(95%CI1.57,2.34).Thestudy iscurrentlyunclear.ItalyandJapanhaveimplementedECGscreening 6 A
dcoidncneortn,shtohwateveeitrh,erretphliecastizeethoertrheesudltessiognftohfethAeGANGENSEstSusdtyu,dryaipsrineg- sayrsrtheymthsm,wohgiecnhicmadyisiedaesnetsif.y72a–s7y4mpWtohmileaticcopnasteiennstussweitxhisitnshearmitaobnleg pril 20
1
sentedlimitations.Thesegeneticdataarenotyetbeingappliedin expertsinEurope and the United States(US) thatsupportpre- 9
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- D
o
Overall,we cannotproviderecommendationsforpopulation toms(includingsyncopeor‘epilepsy’),togetherwithanexhaustive w
n
screeningatthistimebecausetheconsequencesofscreeningstrat- explorationofthe circumstancesofdeathandthecollectionof loa
d
egiesthatdetectastill-undefinednumberof‘falsepositives’andmiss ante-mortem clinical cardiac investigations, is recommended. e
d
anunknownpercentageofaffectedcases(‘falsenegatives’)havenot Whenthevictimis .40yearsofage,thepresenceofriskfactors fro
m
beenestablished.Thisinabilitytoderivearecommendationfrom forCADshouldbeassessed(e.g.activeorpassivesmoking,dyslipo- h
theevidenceobtainedfromexistingscreeningprogrammesillus- proteinaemia,hypertensionordiabetes).Inaddition,acomplete ttp
s
tratesthe need forfurtherworktocollectquantitativedataon three-generationpedigreeshouldbecreated,recordingallsudden ://a
thecost–benefitprofileofperformingECGscreeningindifferent deathsandcardiacdiseases.14Effortstoretrieveoldmedicalre- ca
d
populationsandindifferenthealthcaresystemsandsettings.Con- cordsand/orpost-mortemexaminationsshouldbemade.Family em
versely,inconsiderationofthehigherriskofarrhythmiasandthe memberswithsymptomssuggestiveofthepresenceofacardiac ic.o
u
worseningofstructuralorgeneticdiseasesinindividualsexposed condition,suchassyncope,palpitationsorchestpain,shouldbe p
tointensephysicalexercise,81,82wedosupporttheexistingrecom- prioritizedforevaluation. .co
m
mendationsforpre-participationscreeninginathletes.InEurope Therecommendedcoreevaluationofafirst-degreerelativeofa /e
u
thereisconsensusthatclinicalevaluation,personalorfamilyhistory suddendeathvictimisillustratedinTable4.Intheabsenceofadiag- rh
e
tpaokpinuglataiondn(arebfaesretloinese1ct2io-lnea1d2.E7C).G should be performed in this nwoitshisainbathseelifnaemEilyC,Gvearnydyoanunegchchoicldarredniosghraomul.dbescreenedatleast artj/a
Asmanyinheritablearrhythmogenicdiseasesarecharacterized rticle
3.4.2 Screeningfamilymembersofsuddendeathvictims byage-relatedpenetranceandincompleteexpression,youngerin- -a
b
s
Thediagnosisofaninheritablearrhythmogenicdisorderisestab- dividualsshouldbefollowed-upatregularintervals.Asymptomatic tra
lishedinupto50%83offamilieswithaSADSvictim,especiallychan- andfullygrownadultscanbedischargedfromcareunlesssymp- ct/3
nelopathies[e.g.LQTS,Brugadasyndromeandcatecholaminergic toms appear or new information from the family becomes 6/4
polymorphic ventricular tachycardia (CPVT)] and occasionally available. 1/2
7
subtleformsofcardiomyopathy[HCMandarrhythmogenicright Whenaninheritablearrhythmogenicdiseaseissuspected,DNA 9
3
ventricularcardiomyopathy(ARVC)inparticular]orfamilialhyper- samplesfromthevictimarethebestsourceofinformationwhen /2
2
9
cholesterolaemia.As aconsequence of these findings, when an performingamolecularautopsy.Ifthereisapositiveresult,family 3
3
autopsyiseithernotavailableforthevictim(i.e.SUDSorSUDI) membersshouldbeofferedtheopportunitytoundergopredictive 63
and/orwhenthepost-mortemexaminationfailstodetectstructural geneticscreening,inacascadefashion.The‘rightnottoknow’and by
g
abnormalitiesandtoxicologyresultsarenormal(i.e.SADSorSIDS), thepossibilitytodeclinemolecularscreeningshouldbeincludedin u
e
first-degreerelativesofthevictimshouldbeinformedofthepoten- anypre-informativecommunicationwiththerelatives. st o
tialriskofsimilareventstothemselvesandshouldundergocardiac Intheabsenceofbiologicalsamplesfromthedeceasedperson, n 0
evaluation.AfamilyhistoryofrecurrentprematureSUDSorinher- targetedmolecularscreeninginfirst-degreerelativesmaybeconsid- 6 A
iattaibolneshteroarntgdlyisreeacsoemrempernedseendt.sa‘redflag’thatmakesfamilialevalu- edriseedaswehinenfatmheilryemisemthbeesruss.pCicoinovneorsfetlhy,egperneesteicncsceroeefnainnginohfearitlaarbglee pril 20
1
Familyscreeningoffirst-degreerelativesofvictimsofsudden panelofgenesshouldnotbeperformedinSUDSorSADSrelatives 9
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