PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in ...

64 Pages·2014·4.27 MB·English

by  Paul Khairy

PACES/HRS Expert Consensus Statement on the
Recognition and Management of Arrhythmias in Adult
Congenital Heart Disease
Developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES)
and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS,
the American College of Cardiology (ACC), the American Heart Association (AHA),
the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS),
and the International Society for Adult Congenital Heart Disease (ISACHD)
Paul Khairy, MD, PhD, FRCPC (Chair),* George F. Van Hare, MD, FACC, FHRS (Co-Chair),†
† † ‡
Seshadri Balaji, MBBS, PhD, Charles I. Berul, MD, FHRS, Frank Cecchin, MD, FACC,
MitchellI.Cohen,MD,FACC,FHRS,†CurtJ.Daniels,MD,FACC,**BarbaraJ.Deal,MD,FACC,†
Joseph A. Dearani, MD, FACC,* Natasja de Groot, MD, PhD,¶ Anne M. Dubin, MD, FHRS,†
Louise Harris, MBChB, FHRS,# Jan Janousek, MD, PhD,¶ Ronald J. Kanter, MD, FHRS,†
Peter P. Karpawich, MD, FACC, FAHA, FHRS,† James C. Perry, MD, FACC, FHRS,*
StephenP.Seslar,MD,PhD,†MaullyJ.Shah,MBBS,FHRS,†MichaelJ.Silka,MD,FACC,FAHA,§
† †
John K. Triedman, MD, FACC, FHRS, Edward P. Walsh, MD, FACC, FHRS,
Carole A. Warnes, MD, FRCP, FACC, FAHA**
TABLE OF CONTENTS 4. Deliveryofcareandensuringaccesstocare .............. e108
4.1. Recommendations for the coordination and
Preamble ...............................................................................e103
delivery of care for adults with CHD and
1. Methodologyand evidence ...........................................e103 arrhythmias .............................................................e108
2. Document review and approval ..................................e105 4.2. Recommendations for adults with CHD
3. Epidemiologyand scope of arrhythmias in adults requiring invasive electrophysiologic
with CHD ............................................................................e105 interventions ...........................................................e109
3.1. Changingmortality ..............................................e105 5. Evaluation and diagnosis of arrhythmias ................e110
3.2. Spectrum of arrhythmias ...................................e105 5.1. Introduction ............................................................e110
3.3. Heart failure andarrhythmogenesis ..............e106 5.2. General rhythm assessmentbased on
3.4. Systemic right ventricle and univentricular cardiac history and symptom status ..............e110
heart ...........................................................................e107 5.3. Approach to the symptomatic patient ..........e110
5.4. Approach to the asymptomatic patient ........e113
6. Medical therapy .................................................................e115
6.1. Atrial tachyarrhythmias .....................................e115
*PediatricandCongenitalElectrophysiologySociety(PACES)representa- 6.2. Ventricular tachyarrhythmias ..........................e121
tive; †HeartRhythmSociety (HRS)representative; ‡AmericanCollege of 7. Catheter ablation ...............................................................e122
Cardiology (ACC) representative; §American Heart Association (AHA) 7.1. General considerations for catheter ablation
representative;¶EuropeanHeartRhythmAssociation(EHRA)representa- in adults with CHD .............................................e122
tive;#CanadianHeartRhythmSociety(CHRS)representative;**Interna- 7.2. AV reciprocating tachycardia and AV
tionalSocietyforAdultCongenitalHeartDisease(ISACHD)representative nodal reentrant tachycardia ..............................e122
7.3. Atrial tachyarrhythmias .....................................e123
KEYWORDSAdultcongenitalheartdisease;Congenitalheartdisease(Heart 7.4. Atrial fibrillation ...................................................e124
Rhythm2014;11:e102–e165)
7.5. Recommendations for catheter ablation of
atrial tachycarrhythmias in adults
Address reprint requests and correspondence: Dr. Paul Khairy, Adult
with CHD ................................................................e125
Congenital Heart Center, Montreal Heart Institute, 5000 Belanger St. E.,
Montreal,QC,Canada,H1T1C8.E-mailaddress:[email protected]. 7.6. Ventricular tachycardia ......................................e125
1547-5271/$-seefrontmatterB2014HeartRhythmSociety.Allrightsreserved. http://dx.doi.org/10.1016/j.hrthm.2014.05.009
Khairy et al PACES/HRSExpertConsensusStatementonArrhythmiasinAdultCongenitalHeartDisease e103
8. Bradyarrhythmias andpacemakers ............................e127 CHD.6 Arrhythmias range in symptomatology and signifi-
8.1. Introduction ............................................................e127 cance, from inconsequential and benign to poorly tolerated
8.2. Sinus node dysfunction ......................................e127 and potentially fatal. Taken together, arrhythmias are a
8.3. AV conductionsystem dysfunction ..............e128 leading cause of morbidity, impaired quality of life, and
8.4. Preimplantconsiderations .................................e129 mortality in adults with CHD.
8.5. Issues related to specific congenital In light of the unique issues, challenges, and consider-
heart defects ...........................................................e130 ations involved in managing arrhythmias in this growing,
8.6. Lead extraction ......................................................e131 aging,andheterogeneouspatientpopulation,7itappearsboth
8.7. Recommendations for permanent pacing in timely and essential to critically appraise and synthesize
adults withCHD ...................................................e132 optimal treatment strategies. The purpose of this consensus
9. Sudden cardiac death andICDs ..................................e132 statement is, therefore, to define optimal conditions for the
9.1. Introduction ............................................................e132 delivery of care regarding arrhythmias in adults with CHD
9.2. Suddenand total late mortality .......................e132 and provide expert and, where possible, evidence-based
9.3. Arrhythmic causes of sudden cardiac death e132 recommendations on best practice procedures for the eval-
9.4. Recommendations for ICD therapy in adults uation,diagnosis,andmanagementofarrhythmias,including
with CHD ................................................................e135 medicaltreatment,catheter-basedinterventions,devicether-
9.5. Uniqueconsiderations for ICDs .....................e135 apy, andsurgical options.
9.6. Resultsand outcomes of ICD therapy .........e136
9.7. ConsiderationsregardingICDprogramming .e136
1. Methodology and evidence
10. Cardiac resynchronization therapy .............................e137
10.1. Dyssynchronous heart failure ..........................e137 The Pediatric and Congenital Electrophysiology Society
10.2. Clinical studies on CRT in CHD ...................e137 (PACES), in conjunction with the Heart Rhythm Society
10.3. Technical aspects .................................................e138 (HRS), appointed a 22-member writing committee from
10.4. Recommendations ................................................e140 the United States, Canada, and Europe with complemen-
11. Surgical options .................................................................e141 tary multidisciplinary expertise in pediatric and adult
11.1. Introduction ............................................................e141 electrophysiology, adult CHD, and CHD surgery. The
11.2. Preoperative arrhythmia evaluation ...............e142 writingcommitteeincludedrepresentationfromtheAmer-
11.3. Recommendations for electrophysiologic ican College of Cardiology (ACC), American Heart
study prior to adult CHD surgery ..................e143 Association (AHA), European Heart Rhythm Association
11.4. Role of surgery in treating preexisting (EHRA), Canadian Heart Rhythm Society (CHRS), and
arrhythmias .............................................................e143 International Society for Adult Congenital Heart Disease
11.5. Recommendations for concomitant ventricular (ISACHD). The committee was divided into subgroups to
arrhythmia surgery in adults withCHD review key aspects in the recognition and management of
undergoingopen cardiac surgery ...................e145 arrhythmias in adults with CHD. Experts in the topics
11.6. The role of surgery in preventing the under consideration were tasked with performing formal
development of arrhythmias ............................e145 literaturereviews,weighingthestrengthofevidenceforor
11.7. Recommendations for prophylactic atrial or against diagnostic and therapeutic interventions, estimat-
ventricular arrhythmia surgery in adults with ingexpectedhealthoutcomeswhererelevant,andpropos-
CHD ..........................................................................e146 ing practical clinical recommendations. Wherever
Appendix 1 ..........................................................................e146 possible, recommendations are evidence-based. However,
unlike some practice guidelines, there is not a sizeable
bodyofliteraturewithdefinitiveevidencetosupportmost
Preamble recommendations in this emerging field of cardiology. In
Nearlyonethirdofallmajorcongenitalanomaliesareheart order to maximize the value and credibility of consensus-
defects, with an estimated 9 per 1000 live births afflicted based recommendations, a high-threshold (i.e., 80% or
bycongenitalheartdisease(CHD)worldwide.1Remarkable greater agreement among writing members) was required
advancesincarehaveresultedinimpressivegainsinsurvival toconstituteaconsensus.Supportiveevidenceisindicated
such that over 90% of children with CHD in developed whereappropriate,andvariationsinopinionarenuancedin
countries today are expected to survive into adulthood.2 the text. As a general recommendation, the committee
Consequently, the past decades have witnessed historical strongly supports expanding the evidence base related to
shiftsinpopulationdemographics,asadultsnowoutnumber arrhythmias in adults with CHD through participation in
childrenwithCHD.Population-basedestimatesindicatethat research and clinical registries.
there are currently over 1 million adults with CHD in the The consensus statement was organized by arrhythmia-
UnitedStatesalone,over100,000inCanada,and1.8million relatedtopicsratherthanbyheartdefect.Depending,inpart,
in Europe.3–5 Rhythm disorders, which span the entire on the particular issue and available evidence, recommen-
spectrum of brady- and tachyarrhythmias, are among the dations range from being broadly applicable to adults
most prominent complications encountered by adults with with CHD at large to a more focused lesion-specific scope.
e104 Heart Rhythm, Vol11, No 10, October 2014
Table1.1 ClassificationofCHDcomplexityinadults
Complexity Typeofcongenitalheartdiseaseintheadultpatients
Simple Nativedisease
Isolatedcongenitalaorticvalvedisease
Isolatedcongenitalmitralvalvedisease(exceptparachutevalve,cleftleaflet)
Smallatrialseptaldefect
Isolatedsmallventricularseptaldefect(noassociatedlesions)
Mildpulmonarystenosis
Smallpatentductusarteriosus
Repairedconditions
Previouslyligatedoroccludedductusarteriosus
Repairedsecundumorsinusvenosusatrialseptaldefectwithoutresidua
Repairedventricularseptaldefectwithoutresidua
Moderate Aorto-leftventricularfistulas
Anomalouspulmonaryvenousdrainage,partialortotal
Atrioventricularseptaldefects,partialorcomplete
Coarctationoftheaorta
Ebsteinanomaly
Infundibularrightventricularoutflowobstructionofsignificance
Ostiumprimumatrialseptaldefect
Patentductusarteriosus,notclosed
Pulmonaryvalveregurgitation,moderatetosevere
Pulmonaryvalvestenosis,moderatetosevere
SinusofValsalvafistula/aneurysm
Sinusvenosusatrialseptaldefect
Subvalvularorsupravalvularaorticstenosis
TetralogyofFallot
Ventricularseptaldefectwith:
Absentvalveorvalves
Aorticregurgitation
Coarctationoftheaorta
Mitraldisease
Rightventricularoutflowtractobstruction
Straddlingtricuspidormitralvalve
Subaorticstenosis
Severe/complex Conduits,valvedornonvalved
Cyanoticcongenitalheartdisease,allforms
Double-outletventricle
Eisenmengersyndrome
Fontanprocedure
Mitralatresia
Singleventricle(alsocalleddoubleinletoroutlet,common,orprimitive)
Pulmonaryatresia,allforms
Pulmonaryvascularobstructivedisease
Transpositionofthegreatarteries
Tricuspidatresia
Truncusarteriosus/hemitruncus
Otherabnormalitiesofatrioventricularorventriculoarterialconnectionnotincludedabove(e.g.,crisscross
heart,isomerism,heterotaxysyndromes,ventricularinversion)
AdaptedfromWarnesCA,etal.ACC/AHA2008guidelinesforthemanagementofadultswithcongenitalheartdisease.JAmCollCardiol.2008;52:1890–
1947.8
Thedetailedindexshouldassistthereaderinrapidlylocating Recommendationsweresubjecttoapreviouslydescribed
sectionsofinterestforspecificheartdefects.Inaddition,the standardized classification process (Methodology Manual
writingcommitteeretainedthenomenclatureforcomplexity and Policies from the ACCHF and AHA Task Force on
of CHD (i.e., simple, moderate, complex/severe) proposed Practice Guidelines June 2010)9 that ranked each item
bytheACC/AHAtaskforceonpracticeguidelinesforadults (Classes I, IIa, IIb, III) and its accompanying level of
with CHD,8summarized in Table 1.1. evidence (Levels A, B, C), as summarized in Table 1.2.
Khairy et al PACES/HRSExpertConsensusStatementonArrhythmiasinAdultCongenitalHeartDisease e105
Table1.2 Classificationofrecommendationsandlevelsofevidence9
ClassificationofRecommendations
ClassI Conditionsforwhichthereisevidenceand/orgeneralagreementthatagivenprocedureor
treatmentplanisbeneficial,useful,andeffective
ClassII Conditionsforwhichthereisconflictingevidenceand/ordivergenceofopinionabouttheusefulness/
efficacyofaprocedureortreatment
ClassIIa Weightofevidence/opinionisinfavorofusefulness/efficacy
ClassIIb Usefulness/efficacyislesswellestablishedbyevidence/opinion
ClassIII Conditionsforwhichthereisconflictingevidenceand/orgeneralagreementthataprocedureor
treatmentisnotuseful/effectiveandinsomecasesmaybeharmful
LevelsofEvidence
LevelofevidenceA Dataderivedfrommultiplerandomizedclinicaltrialsormeta-analyses
LevelofevidenceB Dataderivedfromasinglerandomizedtrialornonrandomizedstudies
LevelofevidenceC Onlyconsensusopinionofexperts,casestudies,orstandardofcare
2. Document review and approval 3.2. Spectrum of arrhythmias
ThePACES/HRSTaskForcemadeeveryefforttoavoidall ArrhythmiasincreaseinprevalenceasadultswithCHDage
potential conflicts of interest relevant to this consensus andarethemostfrequentreasonforhospitaladmission.16,17
statement, whether actual or perceived, among members of Alongwithheartfailure,arrhythmiasaretheleadingcauseof
the writing committee. Members of the writing committee death.18–21 Factors associated with pre- and postoperative
(Appendix1)andpeerreviewers(Appendix2)wererequired arrhythmias in CHD are schematically depicted in
to disclose all actual or potential direct or indirect conflicts. Figure3.1.22Arrhythmiasmayreflectcongenitallydisplaced
Committeememberswereobligedtorefrainfromvotingon or malformed sinus nodes or atrioventricular (AV) conduc-
issues related to the potential conflict. The document was tionsystems, abnormal hemodynamics, primary myocardial
reviewed by the PACES executive committee, additional disease, hypoxic tissue injury, residual or postoperative
membersofHRS,andofficialreviewersnominatedbyACC, sequelae, and genetic influences.23–25
AHA, EHRA, CHRS, and ISACHD. All writing members The entire spectrum of arrhythmias may be encountered
approved this final version. inadults withCHD,withseveral subtypesoftencoexisting.
Bradyarrhythmias may involve disorders of the sinus node,
AVnode,His–Purkinjesystem,orintra-atrialpropagation.It
3. Epidemiology and scope of arrhythmias in
has been estimated that approximately 50% of 20-year-olds
adults with CHD
withCHDwilldevelopanatrialtachyarrhythmiaduringtheir
3.1. Changing mortality lifetime.26 Table 3.1 summarizes atrial tachyarrhythmias
The advent of cardiopulmonary bypass and early surgical typically encountered in common forms of CHD.27 Atrial
innovations for CHD of the 1960s and 1970s, coupled with tachyarrhythmias may be mediated by accessory pathways,
advances in clinical care, have culminated in an increasing AV node reentry, twin AV nodes,28,29 macroreentrant
andagingcohortwithCHD.10Survivalbeyondthefirstyear circuits, automatic foci, or nonautomatic foci.30 Intra-atrial
of life has risen from an estimated 25% 50 years ago to reentry is the most common tachyarrhythmia in adults with
490% expected survival into adulthood.11,12 In a CHD,31–33althoughtheprevalenceofatrialfibrillationison
population-based cohort study of patients with CHD, an the rise as the population ages.31,34 Ventricular arrhythmias
overallmortalityreductionof31%wasobservedfrom1987 arethoughttobetheleadingcauseofsuddendeathinseveral
to 2005, largely driven by improved survival in infants.2 subtypesofCHD,withanoverallriskthatisupto100-fold
Mostnotably,themedianageofdeathinpatientswithsevere higher than in age-matched controls.18,19 Fortunately, the
formsofCHDincreasedfrom2to23yearsofage.Theolder absolute incidence of these devastating events remains
adultwithCHDcanalsoanticipateaconsiderablylongerlife relatively low, at approximately 0.1% per year.
expectancy, with one study reporting a median age at death Atabularrepresentationofapproximateexpectedrisksfor
of 57 years in 2007 compared to 37 years in 2002.13 atrial arrhythmia, ventricular arrhythmia, AV block, and
Although causes of death appear to have remained more or ventriculardyssynchronyaresummarizedinFigure3.2.The
less consistent over the past two decades, recent years have prevalenceandmechanismofarrhythmiasvaryaccordingto
seenashiftintheprofileofthe patientatrisk.While lesion factorssuchasage,underlyinganatomicdefect,andmethod
severity and surgical results are major determinants of of surgical repair.31 For example, while 3%–5% of patients
outcome in infants and children, heart failure, arrhythmias, with congenitally corrected transposition will be born with
andpulmonaryhypertensionbecomeincreasinglyimportant complete AV block, it is estimated that an additional 20%
inadulthood.Additionalprognosticfactorsinolderpatients will develop complete heart block by adulthood.35,36 For
include systemic ventricular dysfunction, chronic renal others, prior surgery in the region of the sinus node or its
disease, coronary artery disease, malignancies, and conven- arterial supply (e.g., Mustard, Senning, Glenn, or Fontan)
tional risk factors such as diabetes, hypertension, and will leave them predisposed to later sinus node
obesity.10,14,15 dysfunction.32,37,38
e106 Heart Rhythm, Vol11, No 10, October 2014
Figure3.1 Schematicoffactorsleadingtoarrhythmiasin(A)pre-and(B)postoperativecongenitalheartdisease.AV¼atrioventricular.(Reproduced
withpermissionfromEscuderoC,etal.Electrophysiologicconsiderationsincongenitalheartdiseaseandtheirrelationshiptoheartfailure.CanJCardiol
2013;29(7):821–829.22)
3.3. Heart failure and arrhythmogenesis aspects. Inevitably, the incidence of arrhythmias in the adult
The relationship of heart failure to arrhythmogenesis and CHDpopulationfarexceedsthatseeninyoungerpatients.
sudden cardiac death risk is increasingly appreciated.22 Unique forms of heart failure can also be encountered,
Hemodynamicandelectrophysiologicconditionsthatleadto includingdysfunctionofasystemicrightventricleoruniven-
heart failure, clinical arrhythmias, and adverse outcomes in tricular heart. Systemic left ventricular failure is often
adults with CHD often extend over several decades. These associated with congenital left-sided cardiac lesions. Left
include long-standing effects of prior atrial or ventricular ventricular dysfunction in tetralogy of Fallot and Ebstein
volume loading, scarring, patches, baffles and surgical bar- malformation of the tricuspid valve is more widely appreci-
riers, electromechanical dyssynchrony, ongoing deleterious ated as a sequela associated with heightened risk for sudden
effectsoncell–cellelectricalcoupling,andunderlyinggenetic cardiac death.31,39–41 Right–left ventricular interactions are
Table3.1 SummaryofatrialtachyarrhythmiasencounteredincommonformsofCHD
Congenitalheartdiseasetype Tachyarrhythmia
Atrialseptaldefect IART/AFwithincreasingage,particularlyiflateclosure
Atrioventricularseptaldefect IART/AFfollowingsurgicalrepair
Ebsteinanomaly IART;AVoratriofascicular(Mahaim)AP;suddendeathifhighriskormultipleAPs;ectopic
atrialtachycardia;AF
Left-sidedobstructivelesions IART/AF
TGAwithintraatrialbaffle IART,NAFAT,AVNRT;VT/VFmaybesecondarytoatrialarrhythmias
CongenitallycorrectedTGA AccessorypathwayifEbstein-likesystemicAVvalve
TetralogyofFallot IART;NAFATalongtheanterolateralrightatrium
Heterotaxysyndrome TwinAVnode-mediatedreentranttachycardia
SingleventriclewithFontan IART;NAFAT;AF;maybepoorlytolerated
Eisenmengerphysiology MAT;IART;AF
AF¼atrialfibrillation;AP¼accessorypathway;AV¼atrioventricular;AVNRT¼AVnodalreentranttachycardia;IART¼intraatrialreentranttachycardia;
MAT¼multifocalatrialtachycardia;NAFAT¼nonautomaticfocalatrialtachycardia;TGA¼transpositionofthegreatarteries;VF¼ventricularfibrillation;VT¼
ventricular tachycardia. Adapted with permission from Khairy P. In: Shenasa M et al (eds). Cardiac Mapping. FourthEdition. Oxford, UK: Wiley-Blackwell;
2013:771–777.27
Khairy et al PACES/HRSExpertConsensusStatementonArrhythmiasinAdultCongenitalHeartDisease e107
Figure3.2 Approximateriskestimatesforatrialtachycardia(AT),atrialfibrillation(AF),othersupraventriculararrhythmias,ventriculararrhythmia,sinus
node dysfunction (SND), atrioventricular (AV) block, and ventricular dyssynchrony are shown across various congenital heart defects (CHD) of simple,
moderate,andseverecomplexity.Thecolor-codedpatternrangesfromminimal(noshading)tomild(lightblue),moderate(mediumblue),andhigh(dark
blue)risk.
increasingly acknowledged, and subpulmonary right ventric- scarring, witha high incidence of atrial tachyarrhythmias.44
ular failure itself contributes to the complex interplay of Rapid AV conduction in the setting of an already compro-
factorsassociatedwithsuddendeath.42,43Ventriculardyssyn- mised systemic right ventricle can result in induction of a
chrony due to intrinsic or pacing-induced ventricular con- secondary ventricular tachycardia.45,46 Primary ventricular
duction delay can likewise have deleterious effects on arrhythmias may also occur, most often in association with
systemic ventricular function. In adults with CHD, right systemicrightventricularfailure.36,46–48Myocardialoxygen
bundle branch block (RBBB) is more common than left supply–demandmismatchcanincreaseovertime,leadingto
bundle branch block (LBBB), particularly in the setting of ongoing fibrosis, worsening systemic ventricular function,
tetralogy of Fallot, ventricular septal defects, double-outlet and accrued risk of sudden death.49 Adults with single
right ventricle variants, Rastelli surgery, AV septal defects, ventricle physiology and Fontan palliation are also at risk
and Ebstein malformation of the tricuspid valve. In most for developing sinus node dysfunction and atrial tachyar-
cases,RBBBisacomplicationofsurgicalrepair. rhythmias.50,51 Atrial arrhythmias occur in up to 60% of
Fontanrecipientsandareassociatedwithsubstantialmorbid-
3.4. Systemicrightventricleanduniventricularheart ity and mortality.52 Approximately 90% of Fontan patients
Adults with systemic right ventricles and atrial switch with heart failure–related deaths have concomitant atrial
surgery (e.g., Mustard or Senning) have extensive atrial tachyarrhythmias.51
e108 Heart Rhythm, Vol11, No 10, October 2014
4. Delivery of care and ensuring access to care
4.1. Recommendations for the coordination and delivery of care for adults with CHD and arrhythmias
Recommendations
ClassI 1. HealthcareforadultswithCHDandarrhythmiasshouldbecoordinatedbyregionaladultCHD(ACHD)centersofexcellence
thatservethesurroundingmedicalcommunityasaresourceforconsultationandreferral(Levelofevidence:C).53
2. AregionalACHDcenterthatcaresforadultswithCHDandarrhythmiasshouldbestaffedbyatleastone
cardiacelectrophysiologistwithexpertiseinCHD,inadditiontoassociatedCHDsubspecialistsinimaging,
interventionalcardiology,andcardiacsurgery(Levelofevidence:C).8,54,55
3. Diagnosticandinterventionalcatheter-anddevice-basedelectrophysiologicproceduresinadultswithmoderateor
complexCHDorcomplexarrhythmiasshouldbeperformedinaregionalACHDcenterbyacardiacelectrophysiologist
withexpertiseinCHD,andinalaboratorywithappropriatepersonnelandequipment(Levelofevidence:C).7,56,57
The32ndBethesdaConferencereportcalledattentiontothe programs specific to arrhythmias in adults with CHD are
needforhealthcareprofessionals,patientsandtheirfamilies, considered key factors in ensuring access and delivery of
and regulatory agencies to develop a strategic plan to high-quality care. Health care needs, particularly for adults
improve care access and delivery to the adult with CHD.53 with moderate and complex forms of CHD, should be
Recognition and management of arrhythmias is an integral coordinated by regional ACHD centers of excellence.8,54,59
component of such specialized care.18,19,24,58 Coordinating Personnelandservicespreviouslyrecommendedforregional
care across subspecialties and the development of training ACHD centers are summarized in Table 4.1.8
Table4.1 PersonnelandservicesrecommendedforregionalACHDcenters
Typeofservice Personnel/resources
CardiologistspecializinginACHD Oneorseveral24/7
Congenitalcardiacsurgeon Twoorseveral24/7
Nurse/physicianassistant/nursepractitioner Oneorseveral
Cardiacanaesthesiologist Several24/7
Echocardiography* Twoorseveral24/7
(cid:2) IncludesTEE,intraoperativeTEE
Diagnosticcatheterization* Yes,24/7
Noncoronaryinterventionalcatheterization* Yes,24/7
Electrophysiology/pacing/ICDimplantation* Oneorseveral
Exercisetesting (cid:2) Echocardiography
(cid:2) Radionuclide
(cid:2) Cardiopulmonary
(cid:2) Metabolic
Cardiacimaging/radiology* (cid:2) CardiacMRI
(cid:2) CTscanning
(cid:2) Nuclearmedicine
Multidisciplinaryteams (cid:2) High-riskobstetrics
(cid:2) Pulmonaryhypertension
(cid:2) Heartfailure/transplant
(cid:2) Genetics
(cid:2) Neurology
(cid:2) Nephrology
(cid:2) Cardiacpathology
(cid:2) Rehabilitationservices
(cid:2) Socialservices
(cid:2) Vocationalservices
(cid:2) Financialcounselors
Informationtechnology (cid:2) Databasecollection
(cid:2) Databasesupport
(cid:2) Qualityassessmentreview/protocols
ReproducedwithpermissionfromWarnesCA,etal.ACC/AHA2008guidelinesforthemanagementofadultswithcongenitalheartdisease.JAmCollCardiol
2008;52:1890–1947.8
24/7¼availability24hoursperday,7daysperweek;ACHD¼adultcongenitalheartdisease;CT¼computedtomography;ICD¼implantablecardioverter-
defibrillator;MRI¼magneticresonanceimaging;TEE¼transesophagealechocardiography.
*Thesemodalitiesmustbesupervised/performedandinterpretedbyphysicianswithexpertiseandtrainingincongenitalheartdisease.
Khairy et al PACES/HRSExpertConsensusStatementonArrhythmiasinAdultCongenitalHeartDisease e109
Table4.2 BasicrequirementsforelectrophysiologistswithexpertiseinadultCHD
Completionofspecializedfellowshiptraininginadultorpediatricelectrophysiologywithdemonstratedacquisitionofrequiredclinical
competencies62–65
FormalaffiliationwithanestablishedACHDcenter8,64
Fundamentalknowledgeofcongenitalheartdisease,including:
(cid:2)
Anatomy and physiologyof simple, moderate,andcomplex forms of congenitalheart disease
(cid:2)
Surgical procedures for congenital heart disease
(cid:2)
Natural and unnatural (postsurgical) short- and long-term arrhythmia sequelae
(cid:2)
Particularities essential to safely and effectively execute arrhythmia interventions, including an appreciation for complex
access issues and displaced or malformedatrioventricular conduction systems6,7,68
Experienceandskillsinmanagingadultswithcongenitalheartdiseaseandarrhythmias,64including:
(cid:2)
Noninvasive testing
(cid:2)
Electrophysiologicstudies
(cid:2)
Catheter ablation, including with 3-dimensional electroanatomic mapping systems and large-tip/irrigated catheters
(cid:2)
Intraoperative procedures
(cid:2)
Cardiac rhythm management devices
ACHD¼adultcongenitalheartdisease.
Because arrhythmias account for the majority of emer- consensus document, basic competencies are summarized
gency room visits in adults with CHD, emergency care in Table 4.2.62–65 Currently, there is a paucity of formally
facilities should ideally have access to, and an affiliation trainedadultCHDelectrophysiologists,and,therefore,close
with,aregionalACHDcenter.60Theprovisionofsupportfor collaborations between adult and pediatric electrophysiolo-
local emergency centers is critically important considering gistsandACHDspecialistsmayberequiredtodeliverhigh-
thatthesecentersmayhavelittleornofamiliaritywithCHD quality care to adults with CHD and arrhythmias. These
anatomy, hemodynamics, and complex management arrangements are viewed by the committee as acceptable
issues.61 In other less urgent situations, coordination by a methods of optimizing quality of care. In certain circum-
regional ACHD center should include the availability of stances, a broader team approach to managing adults with
consultation services for arrhythmia-related issues, with CHDandarrhythmiasmaybebeneficial,includinginterven-
consideration given to transferring care whenever subspeci- tional cardiologists, heart failure specialists, and/or adult
alty expertise are required, including for electrophysiologic CHD surgeons. Examples include hybrid surgical
studies, catheter ablation,or device implantation.8 approaches to managing arrhythmias,66 recanalization of
Although detailed recommendations regarding training obstructed baffles or conduits to allow catheter or lead
and skills required to qualify as an electrophysiologist with access,67presurgicalelectrophysiologicmapping,epicardial
expertise in adult CHD are beyond the scope of this leadimplantation,7andarrhythmiasurgery(seeSection11).
4.2. Recommendations for adults with CHD requiring invasive electrophysiologic interventions
Recommendations
ClassI 1. ConsultationwithanACHDspecialistshouldbesoughtpriortoinvasiveelectrophysiologicinterventionsinadultswithCHD
(Levelofevidence:C).8,54,55
2. Preproceduralplanningshouldincludeadetailedreviewofoperativenotespertainingtoallpreviouscardiacandvascular
surgeries,patientanatomy,vascularandintracardiacaccessissues,priorinterventions,andalldocumentedarrhythmias
(Levelofevidence:C).6,7,68
3. InvasiveelectrophysiologicinterventionsinadultswithmoderateorcomplexCHDthatrequireconscioussedationorgeneral
anesthesiashouldbeperformedincollaborationwithananesthesiologistfamiliarwithCHD(Levelofevidence:C).69
4. TheelectrophysiologylaboratoryandpostprocedurerecoveryunitshouldbesuitableforthecareofadultswithCHD,
including:
1. Adultappropriateequipment(Levelofevidence:C);
2. Nursingandtechnicalstaffcertifiedinadultcardiaclifesupport(ACLS)andtrainedinbasicCHDanatomy(Levelof
evidence:C);
3. ACHDcardiothoracicsurgicalbackupandoperatingroomaccess(Levelofevidence:C).70
e110 Heart Rhythm, Vol11, No 10, October 2014
Invasive electrophysiologic procedures in adults with The presence or absence of symptoms is a practical
CHD should be performed by electrophysiologists with startingpointforevaluatingadultswithCHDforarrhythmias.
expertise in adults with CHD and within an environment In patients with symptoms, the primary task involves
suitableforCHDandadultpatientcare.59Thetechnicaland determining whether the complaint is rhythm-related and, if
nursing staff involved with preprocedural, procedural, and so,documentingorreplicatingtherhythmdisturbancesothat
postprocedural care must be ACLS certified and familiar appropriate treatment can be instituted. In asymptomatic
with basic CHD anatomy and physiology. Preprocedural patients, the task is to detect or predict arrhythmias and
evaluation may include risk analysis that considers associ- institute therapy in advance of serious symptoms through a
ated comorbidities, additional subspecialty consultation processofsurveillancetestingandriskassessment,whichis
(e.g., pulmonary medicine, nephrology, infectious disease), inmanywaysafarmorechallengingandimperfectexercise.6
anesthesia assessment, and supplementary preprocedural
imaging or functional analysis, as needed.
5.3. Approach to the symptomatic patient
5. Evaluation and diagnosis of arrhythmias Careful history and physical examination reveal a mix of
electrophysiologic and hemodynamic data that are vital to
5.1. Introduction
determining the pace and setting of the subsequent evalua-
Arrhythmias and their attendant clinical consequences become
tion.Attributesofthesymptoms,includingtiming,duration,
increasingly prevalent in adults with CHD as they age.
context, and severity, are helpful in guiding the subsequent
Ramifications are often dependent as much on the clinical
selection of tests. In patients with an in situ cardiac rhythm
contextinwhichthearrhythmiaoccursasthearrhythmiaitself.
management device, device interrogation may provide the
Forthatreason,thissectionstressestheimportancenotonlyof
required information to clinch a diagnosis.75 In the absence
electrophysiologic testing from which one might elucidate the
of such a device or in the event of an unrevealing inter-
correct “electrical” diagnosis but also of a broader evaluation
rogation,subsequentworkupisdeterminedbasedonlevelof
that allows care providers to understand the arrhythmia within
clinician suspicion and severity of symptoms.
thecontextofthepatient’scardiovascularstatus.Althoughitis
Inpatientswithabortedsuddencardiacdeathorunexplained
possible to make some generic recommendations regarding
syncope, consideration should be given to performing a
adults with CHD as a collective entity or within defined
diagnostic electrophysiologic study with programmed atrial
subgroups, in many cases, the natural and modified history of
and ventricular stimulation.75,76 For milder symptoms, some
specific anatomic forms of CHD and/or associated palliative
formofambulatorymonitoringisusuallyindicated.Frequentor
corrections dictate more precise targeting of recommendations
incessantsymptomsmaybewellsuitedfor24-hourambulatory
to specific substrates. Finally, although the presence of symp-
ECG (Holter) evaluation. In contrast, infrequent, brief symp-
toms will drive the majority of arrhythmia evaluations, it is
toms are better evaluated using an event recorder or longer-
recognizedthatsurveillancetestinginselectcircumstancesmay
durationHoltermonitoring.Whilepursuingtheelectrophysio-
alerttheprovidertoanimpendingorunrecognizedarrhythmia.
logic evaluation, the clinician must also assess the patient’s
anatomy andhemodynamic/functional status.Critical parame-
5.2. General rhythm assessment based on cardiac ters include ventricular size and function, AV valve function,
history and symptom status andvesselorbafflepatency,whichcanoftenbeelucidatedby
Arrhythmia risks in adults with CHD vary according to the transthoracic echocardiography. When relevant, additional
underlying heart lesion, hemodynamics, and features in a functional and anatomic data can be obtained through 3-
patient’s clinical history. Certain rhythm disorders are well dimentional imaging such as cardiac computed tomography
known to be lesion-specific, such as accessory pathway– (CT)ormagneticresonanceimaging(MRI),ormoreinvasive
mediated tachycardia in Ebstein anomaly71 and AV block in means such as transesophageal echocardiography or cardiac
thesettingofL-loopedtranspositionofthegreatarteries.72The catheterization. Like history and physical examination, ECG
tendency for atrial tachycardias and sinus node dysfunction to andcardiopulmonaryexercisetestingprovideavaluablemixof
developinpatientswhohaveundergoneextensiveatrialbaffling bothelectrophysiologicandhemodynamic/functionaldata.
proceduresisalsoclearlyestablished,32,73asistheassociationof
monomorphic ventricular tachycardia with such lesions as
surgically repaired tetralogy of Fallot,31 and polymorphic 5.3.1. Rhythm testing for symptomatic patients
ventriculartachycardiaorventricularfibrillationinpatientswith 5.3.1.1. Electrocardiogram. Documentation of an active
advanceddegreesofventriculardysfunction.40,74Moreover,late arrhythmia by 12-lead ECG at the time of symptoms is a
age at time of complete surgical repair and incomplete or cornerstone of diagnosis, but this luxury is not always
imperfectrepairwithresidualcyanosisorpressure/volumeloads available. Observations such as marked bradycardia, AV
areamongthemanyhistoricalitemsthathavebeenimplicated andintraventricularconductiondisturbances,QRSduration,
asgeneralriskfactorsforbothatrialandventriculararrhythmias. repolarization pattern, and varied degrees of atrial and
Individualpatientanatomy,surgicalhistory,andhemodynamic ventricularectopicactivitymayallproveusefulindecipher-
statusmust,therefore,beascertainedfullywheneverarrhythmia ing a patient’s complaint. Typical ECG features in adults
risksarebeingestimated. withcommonformsofCHDaresummarizedinTable5.1.77
K
Table5.1 TypicalECGfeaturesinadultswithcommonformsofCHD h
a
ir
Congenital Atrial y
diagnosis Rhythm PRinterval QRSaxis QRSconfiguration enlargement Ventricularhypertrophy Particularities et
a
Secundum NSR;↑IART/AFwith 1oAVB6%– 0o–180o;RAD;LADinHolt- rSr0 orrsR0 with RAE35% Uncommon “Crochetage”pattern l
atrialseptal age 19% OranorLAHB RBBBi4RBBBc P
A
defect C
E
Ventricular NSR;PVCs Normalormild RADwithBVH;LAD Normalorrsr0;possibleRBBB PossibleRAE BVH23%–61%;RVHwith Katz-Wachtelphenomenon S
/
septal ↑;1oAVB 3%–15% (cid:1)LAE Eisenmenger H
R
defect 10% S
AVcanal NSR;PVCs30% 1oAVB450% ModtoextremeLAD; rSr0 orrsR0 PossibleLAE Uncommoninpartial;BVHin Inferoposteriorly Ex
p
defect Normalwithatypical complete;RVHwith displacedAVN e
r
Eisenmenger t
C
Patentductus NSR;↑IART/AFwith ↑PR10%–20% Normal DeepSV1,tallRV5andV6 LAEwith Uncommon Ofteneitherclinically on
arteriosus age moderate silentorEisenmenger se
n
PDA s
u
Pulmonary NSR Normal Normalifmild;RADwith Normal;severity PossibleRAE RVH;severitycorrelateswithR: Axisdeviationcorrelates s
S
stenosis moderate/severe SinV1andV6 withRVP ta
Aortic NSR Normal NormalorLAD Normal PossibleLAE LVH,especiallybyvoltage PersistentRVHrarebeyond te
m
coarctation criteria infancy
e
Ebstein NSR;possibleEAR, 1oAVB NormalorLAD Low-amplitudemultiphasic RAEwith DiminutiveRV Accessorypathway n
t
anomaly SVT;AF/IART40% common; atypicalRBBB Himalayan common;QII,III,aVF o
n
shortif Pwaves andV1–V4 A
WPW rr
h
Surgically NSR;PVCs;IART10%; Normalormild NormalorRAD; RBBB90% PeakedP RVHpossibleifRVOT QRSduration(cid:1)QTd y
t
repaired VT12% ↑ LAD5%–10% waves;RAE obstructionorPHT predictiveofVT/SCD hm
TOF possible ia
L-TGA NSR 1o4AV5B0%;AVB LAD AbasveFncaendserpigtahltqp;reQcoinrdIiIuIm, Noatsisfoncioated Notifnoassociateddefects AnptreercioorrdAiVaNl;;WPPoWsitwiviethT sin
A
2%/year defects Ebstein d
u
D-TGA/intra- Sinusbrady60%;EAR; Normal RAD Absenceofq,smallr,deepS PossibleRAE RVH;diminutiveLV PossibleAVBifVSDorTV lt
atrialbaffle junctional;IART inleftprecordium surgery Co
n
25% g
UVHwith Sinusbrady15%;EAR; NormalinTA; LADinsingleRV,TA, Variable;↑↑RandS RAEinTA RVHwithsingleRV;possible AbsentsinusnodeinLAI; en
Fontan junctional;IART 1oAVBin singleLVwith amplitudesinlimband LVHwithsingleLV AVblockwithL-loopor ita
450% DILV noninvertedoutlet precordialleads AVCD lH
Dextrocardia NS1R0;5Po-–w1a6v5eoawxiitsh Normal RAD Invreeprsoeladriezpaotiloarnizationand Noitnvweirtshussitus LVsHm:atlalllRRVV1a–nVd2;tRaVllHR:rdiegehptQ, Sitwuasvseolaixtuiss:anndorsmeavlerPe eart
1 D
situsinversus lateral CHD is
ALCAPA NSR Normal PossibleLAD Pathologicant-latQwaves; PossibleLAE Selectivehypertrophyof Possibleischemia ea
s
possibleant-septQwaves posterobasalLV e
AF¼atrialfibrillation;ALCAPA¼anomalousleftcoronaryarteryfromthepulmonaryartery;AVB¼atrioventricularblock;AVCD¼atrioventricularcanaldefect;AVN¼AVnode;BVH¼biventricularhypertrophy;
CHD¼congenitalheartdisease;DILV¼double-inletleftventricle;EAR¼ectopticatrialrhythm;IART¼intra-atrialreentranttachycardia;LAD¼left-axisdeviation;LAE¼leftatrialenlargement;LAHB¼left
anteriorhemiblock;LAI¼leftatrialisomerism;LV¼leftventricle;LVH¼leftventricularhypertrophy;NSR¼normalsinusrhythm;PDA¼patentductusarteriosus;PHT¼pulmonaryhypertension;RAD¼right-axis
deviation;RAE¼rightatrialenlargement;,PVC¼prematureventricularcontraction;RBBB¼rightbundlebranchblock(c¼complete;i¼incomplete);RV¼rightventricle;RVH¼rightventricularhypertrophy;
RVOT¼rightventricularoutflowtract;RVP¼rightventricularpressure;SCD¼suddencardiacdeath;SVT¼supraventriculartachycardia;TA¼tricuspidatresia;TOF¼tetralogyofFallot;TV¼tricuspidvalve;
VSD¼ventricularseptaldefect;VT¼ventriculartachycardia;WPW¼Wolff-Parkinson-Whitesyndrome.
ReproducedwithpermissionfromKhairyP,MarelliAJ.Clinicaluseofelectrocardiographyinadultswithcongenitalheartdisease.Circulation2007;116:2734–2746.77 e
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