2012 accf/aha/hrs focused update incorporated into the ... · practice guideline 2012 accf/aha/hrs...

Journal of the American College of Cardiology Vol. 61, No. 3, 2013© 2013 by the American College of Cardiology Foundation, the American Heart Association, Inc., ISSN 0735-1097/$36.00

PRACTICE GUIDELINE

2012 ACCF/AHA/HRS Focused Update IncorporatedInto the ACCF/AHA/HRS 2008 Guidelines forDevice-Based Therapy of Cardiac Rhythm AbnormalitiesA Report of the American College of Cardiology Foundation/American Heart AssociationTask Force on Practice Guidelines and the Heart Rhythm Society

Developed in Collaboration With the American Association for Thoracic Surgeryand Society of Thoracic Surgeons

2008 WRITING COMMITTEE MEMBERSAndrew E. Epstein, MD, FACC, FAHA, FHRS, Chair; John P. DiMarco, MD, PHD, FACC, FHRS;

Kenneth A. Ellenbogen. MD, FACC, FAHA, FHRS; N.A. Mark Estes III, MD, FACC, FAHA, FHRS;Roger A. Freedman, MD, FACC, FHRS; Leonard S. Gettes, MD, FACC, FAHA;A. Marc Gillinov, MD, FACC, FAHA; Gabriel Gregoratos, MD, FACC, FAHA;

Stephen C. Hammill, MD, FACC, FHRS; David L. Hayes, MD, FACC, FAHA, FHRS;Mark A. Hlatky, MD, FACC, FAHA; L. Kristin Newby, MD, FACC, FAHA;

Richard L. Page, MD, FACC, FAHA, FHRS; Mark H. Schoenfeld, MD, FACC, FAHA, FHRS;Michael J. Silka, MD, FACC; Lynne Warner Stevenson, MD, FACC#; Michael O. Sweeney, MD, FACC

Developed in Collaboration With the American Association for Thoracic Surgery,Heart Failure Society of America, and Society of Thoracic Surgeons

2012 WRITING GROUP MEMBERS*Cynthia M. Tracy, MD, FACC, FAHA, Chair; Andrew E. Epstein, MD, FACC, FAHA, FHRS, Vice Chair*;

Dawood Darbar, MD, FACC, FHRS†; John P. DiMarco, MD, PHD, FACC, FHRS*‡;Sandra B. Dunbar, RN, DSN, FAAN, FAHA†; N.A. Mark Estes III, MD, FACC, FAHA, FHRS*§;

T. Bruce Ferguson, JR, MD, FACC, FAHA*�¶; Stephen C. Hammill, MD, FACC, FHRS‡;Pamela E. Karasik, MD, FACC, FHRS†; Mark S. Link, MD, FACC, FHRS*†;

Joseph E. Marine, MD, FACC, FHRS†; Mark H. Schoenfeld, MD, FACC, FAHA, FHRS*†;Amit J. Shanker, MD, FACC, FHRS‡; Michael J. Silka, MD, FACC†; Lynne Warner Stevenson, MD, FACC*#;

William G. Stevenson, MD, FACC, FAHA, FHRS***; Paul D. Varosy, MD, FACC, FHRS†

*The 2012 writing group members were required to recuse themselves from voting on sections to which their specific relationships with industry and otherentities may apply; see Appendix 4 for recusal information. †ACCF/AHA Representative. ‡Heart Rhythm Society Representative. §ACCF/AHA TaskForce on Performance Measures Liaison. �American Association for Thoracic Surgery Representative. ¶Society of Thoracic Surgeons Representative.#Heart Failure Society of America Representative. **ACCF/AHA Task Force on Practice Guidelines Liaison.

This document was approved by the American College of Cardiology Foundation Board of Trustees, the American Heart Association Science Advisoryand Coordinating Committee, and the Heart Rhythm Society Board of Trustees in May 2012.

The American College of Cardiology Foundation requests that this document be cited as follows: Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAMIII, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ,Stevenson LW, Sweeney MO 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy ofcardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines andthe Heart Rhythm Society. J Am Coll Cardiol 2013;61:e6–75.

This article is copublished in Circulation.Copies: This document is available on the World Wide Web sites of the American College of Cardiology (http://www.cardiosource.org) and the

American Heart Association (my.americanheart.org). For copies of this document, please contact Elsevier Inc. Reprint Department, fax (212) 633-3820,e-mail [email protected].

Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not permitted without the express permission of the

and the Heart Rhythm Society http://dx.doi.org/10.1016/j.jacc.2012.11.007Published by Elsevier Inc.

American Heart College of Cardiology Foundation. Please contact Elsevier’s permission department at [email protected].

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e7JACC Vol. 61, No. 3, 2013 Epstein et al.January 22, 2013:e6–75 Device Guideline: 2012 Update Incorporated

ACCF/AHA TASK FORCE MEMBERSJeffrey L. Anderson, MD, FACC, FAHA, Chair; Alice K. Jacobs, MD, FACC, FAHA, Immediate Past Chair;

Jonathan L. Halperin, MD, FACC, FAHA, Chair-Elect; Nancy M. Albert, PHD, CCNS, CCRN;Mark A. Creager, MD, FACC, FAHA; David DeMets, PHD; Steven M. Ettinger, MD, FACC;

Robert A. Guyton, MD, FACC; Judith S. Hochman, MD, FACC, FAHA;Frederick G. Kushner, MD, FACC, FAHA; E. Magnus Ohman, MD, FACC;

William Stevenson, MD, FACC, FAHA; Clyde W. Yancy, MD, FACC, FAHA

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TABLE OF CONTENTS

reamble (UPDATED) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e8

. Introduction (UPDATED). . . . . . . . . . . . . . . . . . . . . . . .e10

1.1. Organization of Committee. . . . . . . . . . . . . . . . . . .e101.2. Document Review and Approval . . . . . . . . . . . . . .e111.3. Methodology and Evidence. . . . . . . . . . . . . . . . . . .e11

. Indications for Pacing . . . . . . . . . . . . . . . . . . . . . . . . .e13

2.1. Pacing for Bradycardia Due to Sinus andAtrioventricular Node Dysfunction . . . . . . . . . . . .e132.1.1. Sinus Node Dysfunction . . . . . . . . . . . . . . . . .e132.1.2. Acquired Atrioventricular Block in Adults . . .e142.1.3. Chronic Bifascicular Block . . . . . . . . . . . . . . .e162.1.4. Pacing for Atrioventricular Block Associated

With Acute Myocardial Infarction . . . . . . . . .e172.1.5. Hypersensitive Carotid Sinus Syndrome

and Neurocardiogenic Syncope . . . . . . . . . . . .e182.2. Pacing for Specific Conditions . . . . . . . . . . . . . . .e19

2.2.1. Cardiac Transplantation . . . . . . . . . . . . . . . . .e192.2.2. Neuromuscular Diseases . . . . . . . . . . . . . . . . .e202.2.3. Sleep Apnea Syndrome . . . . . . . . . . . . . . . . . .e202.2.4. Cardiac Sarcoidosis . . . . . . . . . . . . . . . . . . . . .e20

2.3. Prevention and Termination ofArrhythmias by Pacing . . . . . . . . . . . . . . . . . . . . . . .e202.3.1. Pacing to Prevent Atrial Arrhythmias . . . . . .e212.3.2. Long-QT Syndrome . . . . . . . . . . . . . . . . . . . .e212.3.3. Atrial Fibrillation (Dual-Site, Dual-Chamber,

Alternative Pacing Sites) . . . . . . . . . . . . . . . . .e212.4. Pacing for Hemodynamic Indications . . . . . . . . .e22

2.4.1. Cardiac Resynchronization Therapy(UPDATED). . . . . . . . . . . . . . . . . . . . . . . . . .e22

2.4.2. Obstructive Hypertrophic Cardiomyopathy. . . . . .e252.5. Pacing in Children, Adolescents, and Patients

With Congenital Heart Disease . . . . . . . . . . . . . . .e262.6. Selection of Pacemaker Device . . . . . . . . . . . . . .e28

2.6.1. Major Trials Comparing Atrial or Dual-Chamber Pacing With Ventricular Pacing . . .e29

2.6.2. Quality of Life and Functional StatusEnd Points . . . . . . . . . . . . . . . . . . . . . . . . . . . .e30

2.6.3. Heart Failure End Points . . . . . . . . . . . . . . . .e312.6.4. Atrial Fibrillation End Points . . . . . . . . . . . . .e312.6.5. Stroke or Thromboembolism End Points . . .e312.6.6. Mortality End Points. . . . . . . . . . . . . . . . . . . .e312.6.7. Importance of Minimizing Unnecessary

Ventricular Pacing . . . . . . . . . . . . . . . . . . . . . .e312.6.8. Role of Biventricular Pacemakers . . . . . . . . . .e32

2.7. Optimizing Pacemaker Technology and Cost . . . . . .e322.8. Pacemaker Follow-Up . . . . . . . . . . . . . . . . . . . . . . . .e33

2.8.1. Length of Electrocardiographic Samples
for Storage . . . . . . . . . . . . . . . . . . . . . . . . . . . .e34
2.8.2. Frequency of Transtelephonic Monitoring . . .e352.8.3. Remote Follow-Up and Monitoring

(NEW SECTION). . . . . . . . . . . . . . . . . . . . .e35. Indications for Implantable

Cardioverter-Defibrillator Therapy . . . . . . . . . . . . . .e35

3.1. Secondary Prevention ofSudden Cardiac Death . . . . . . . . . . . . . . . . . . . . . . .e363.1.1. Implantable Cardioverter-Defibrillator Therapy

for Secondary Prevention of Cardiac Arrestand Sustained Ventricular Tachycardia. . . . . .e36

3.1.2. Specific Disease States and SecondaryPrevention of Cardiac Arrest or SustainedVentricular Tachycardia . . . . . . . . . . . . . . . . . .e37

3.1.3. Coronary Artery Disease . . . . . . . . . . . . . . . . .e373.1.4. Nonischemic Dilated Cardiomyopathy . . . . . .e373.1.5. Hypertrophic Cardiomyopathy . . . . . . . . . . . .e373.1.6. Arrhythmogenic Right Ventricular

Dysplasia/Cardiomyopathy . . . . . . . . . . . . . . .e373.1.7. Genetic Arrhythmia Syndromes . . . . . . . . . . .e373.1.8. Syncope With Inducible Sustained

Ventricular Tachycardia . . . . . . . . . . . . . . . . . .e383.2. Primary Prevention of Sudden Cardiac Death . . . . .e38

3.2.1. Coronary Artery Disease . . . . . . . . . . . . . . . . .e383.2.2. Nonischemic Dilated Cardiomyopathy . . . . . .e383.2.3. Long-QT Syndrome . . . . . . . . . . . . . . . . . . . .e403.2.4. Hypertrophic Cardiomyopathy . . . . . . . . . . . .e403.2.5. Arrhythmogenic Right Ventricular Dysplasia/

Cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . .e403.2.6. Noncompaction of the Left Ventricle. . . . . . .e413.2.7. Primary Electrical Disease (Idiopathic

Ventricular Fibrillation, Short-QT Syndrome,Brugada Syndrome, and CatecholaminergicPolymorphic Ventricular Tachycardia) . . . . . .e41

3.2.8. Idiopathic Ventricular Tachycardias . . . . . . . .e423.2.9. Advanced Heart Failure and

Cardiac Transplantation . . . . . . . . . . . . . . . . .e423.3. Implantable Cardioverter-Defibrillators in

Children, Adolescents, and Patients WithCongenital Heart Disease . . . . . . . . . . . . . . . . . . . .e443.3.1. Hypertrophic Cardiomyopathy . . . . . . . . . . . .e45

3.4. Limitations and Other Considerations. . . . . . . . .e453.4.1. Impact on Quality of Life

(Inappropriate Shocks). . . . . . . . . . . . . . . . . . .e453.4.2. Surgical Needs . . . . . . . . . . . . . . . . . . . . . . . . .e463.4.3. Patient Longevity and Comorbidities . . . . . . .e473.4.4. Terminal Care . . . . . . . . . . . . . . . . . . . . . . . . .e48

3.5. Cost-Effectiveness of ImplantableCardioverter-Defibrillator Therapy . . . . . . . . . . . .e49

3.6. Selection of ImplantableCardioverter-Defibrillator Generators . . . . . . . . .e40

3.7. Implantable Cardioverter-Defibrillator
Follow-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e50

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e8 Epstein et al. JACC Vol. 61, No. 3, 2013Device Guideline: 2012 Update Incorporated January 22, 2013:e6–75

3.7.1. Elements of ImplantableCardioverter-Defibrillator Follow-Up . . . . . . .e50

3.7.2. Focus on Heart Failure After FirstAppropriate Implantable Cardioverter-Defibrillator Therapy . . . . . . . . . . . . . . . . . . . .e51

. Areas in Need of Further Research . . . . . . . . . . . . .e52

eferences (UPDATED) . . . . . . . . . . . . . . . . . . . . . . . . . . .e52

ppendix 1. 2008 Author Relationships Withdustry and Other Entities (Relevant) . . . . . . . . . . . .e66

ppendix 2. 2008 Reviewer Relationships Withdustry and Other Entities (Relevant) . . . . . . . . . . . .e68

ppendix 3. Abbreviations List (UPDATED). . . . . . . . .e70

ppendix 4. 2012 Author Relationships Withdustry and Other Entities (Relevant) (NEW) . . . . . .e71

ppendix 5. 2012 Reviewer Relationships Withdustry and Other Entities (Relevant) (NEW) . . . . . .e73

ppendix 6. 2012 Indications for CRTerapy–Algorithm (NEW) . . . . . . . . . . . . . . . . . . . . . . . . .e75

Preamble (UPDATED)

is important that the medical profession play a significantle in critically evaluating the use of diagnostic proceduresd therapies as they are introduced and tested in thetection, management, or prevention of disease states. Rig-ous and expert analysis of the available data documentingsolute and relative benefits and risks of those proceduresd therapies can produce helpful guidelines that improve the

fectiveness of care, optimize patient outcomes, and favor-ly affect the overall cost of care by focusing resources one most effective strategies.The American College of Cardiology Foundation (ACCF)d the American Heart Association (AHA) have jointlygaged in the production of such guidelines in the area ofrdiovascular disease since 1980. The ACCF/AHA Task

orce on Practice Guidelines (Task Force), whose charge is tovelop, update, or revise practice guidelines for importantrdiovascular diseases and procedures, directs this effort.riting committees are charged with the task of performingassessment of the evidence and acting as an independent

oup of authors to develop, update, or revise written recom-endations for clinical practice.Experts in the subject under consideration have beenlected from both organizations to examine subject-specificta and write guidelines. The process includes additionalpresentatives from other medical practitioner and specialtyoups when appropriate. Writing committees are specificallyarged to perform a literature review, weigh the strength ofidence for or against a particular treatment or procedure,d include estimates of expected health outcomes where
ta exist. Patient-specific modifiers, comorbidities, and is- pr
es of patient preference that may influence the choice ofrticular tests or therapies are considered, as well as fre-ency of follow-up and cost-effectiveness. When available,formation from studies on cost will be considered; however,view of data on efficacy and clinical outcomes will consti-te the primary basis for preparing recommendations in theseidelines.The guidelines will be reviewed annually by the Task

orce. Each guideline is considered current unless it isdated, revised, or a published addendum declares it out ofte and no longer official ACCF/AHA policy. Keeping paceith the stream of new data and evolving evidence on whichideline recommendations are based is an ongoing challengetimely development of clinical practice guidelines. In an

fort to respond promptly to new evidence, the Task Forces created a “focused update” process to revise the existingideline recommendations that are affected by evolving dataopinion. New evidence is reviewed in an ongoing fashionmore efficiently respond to important science and treatment

ends that could have a major impact on patient outcomesd quality of care.The 2012 focused update was prompted following aorough review of late-breaking clinical trials presented attional and international meetings, in addition to other newblished data deemed to have an impact on patient careection 1.3, “Methodology and Evidence”). Through aoad-based vetting process, the studies included are identi-

ed as being important to the relevant patient population. Thecused update is not intended to be based on a complete

terature review from the date of the previous guidelineblication but rather to include pivotal new evidence thatay affect changes to current recommendations. See the 2012cused update for the complete preamble and evidenceview period (1).In analyzing the data and developing recommendations andpporting text, the focused update writing group usesidence-based methodologies developed by the Task Forcea). The Class of Recommendation (COR) is an estimate ofe size of the treatment effect, with consideration given tosks versus benefits, as well as evidence and/or agreementat a given treatment or procedure is or is not useful/effectived in some situations may cause harm. The Level of

vidence (LOE) is an estimate of the certainty or precision ofe treatment effect. The writing group reviews and ranksidence supporting each recommendation, with the weightevidence ranked as LOE A, B, or C, according to specificfinitions that are included in Table 1. Studies are identifiedobservational, retrospective, prospective, or randomized,appropriate. For certain conditions for which inadequate

ta are available, recommendations are based on expertnsensus and clinical experience and are ranked as LOE C.hen recommendations at LOE C are supported by historicalinical data, appropriate references (including clinical re-ews) are cited if available. For issues for which sparse datae available, a survey of current practice among the clinicianembers of the writing group is the basis for LOE Ccommendations, and no references are cited. The schemar COR and LOE is summarized in Table 1, which also
ovides suggested phrases for writing recommendations

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ithin each COR. A new addition to this methodology for the12 focused update is separation of the Class III recommen-tions to delineate whether the recommendation is deter-ined to be of “no benefit” or is associated with “harm” to thetient. (This version of the COR/LOE table was used forvelopment of the 2012 Focused Update and is included ine current document. (1)) In addition, in view of thecreasing number of comparative effectiveness studies, com-rator verbs and suggested phrases for writing recommen-tions for the comparative effectiveness of one treatment or

rategy versus another have been added for COR I and IIa,OE A or B only.In view of the advances in medical therapy across theectrum of cardiovascular diseases, the Task Force has

ble 1. Applying Classification of Recommendations and Level

A recommendation with Level of Evidence B or C does not imply that the reconot lend themselves to clinical trials. Although randomized trials are unavaila

eful or effective.*Data available from clinical trials or registries about the usefulness/efficacy

yocardial infarction, history of heart failure, and prior aspirin use.†For comparative effectiveness recommendations (Class I and IIa; Level of Evi

rect comparisons of the treatments or strategies being evaluated.

signated the term guideline-directed medical therapy sp

DMT) to represent optimal medical therapy as defined byCCF/AHA guideline (primarily Class I)–recommendederapies. This new term, GDMT, is incorporated into the12 focused update and will be used throughout all futureidelines.Because the ACCF/AHA practice guidelines address pa-

ent populations (and healthcare providers) residing in Northmerica, drugs that are not currently available in Northmerica are discussed in the text without a specific COR. Forudies performed in large numbers of subjects outside Northmerica, each writing group reviews the potential impact offferent practice patterns and patient populations on theeatment effect and relevance to the ACCF/AHA targetpulation to determine whether the findings should inform a

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ation is weak. Many important clinical questions addressed in the guidelinesre may be a very clear clinical consensus that a particular test or therapy is

rent subpopulations, such as sex, age, history of diabetes, history of prior

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The ACCF/AHA practice guidelines are intended to assistalthcare providers in clinical decision making by describ-g a range of generally acceptable approaches to the diag-sis, management, and prevention of specific diseases ornditions. The guidelines attempt to define practices thateet the needs of most patients in most circumstances. Thetimate judgment about care of a particular patient must beade by the healthcare provider and patient in light of all thercumstances presented by that patient. As a result, situationsay arise in which deviations from these guidelines may bepropriate. Clinical decision making should consider theality and availability of expertise in the area where care isovided. When these guidelines are used as the basis forgulatory or payer decisions, the goal should be improve-ent in quality of care. The Task Force recognizes thattuations arise in which additional data are needed to informtient care more effectively; these areas will be identifiedithin each respective guideline when appropriate.Prescribed courses of treatment in accordance with thesecommendations are effective only if they are followed.ecause lack of patient understanding and adherence mayversely affect outcomes, physicians and other healthcareoviders should make every effort to engage the patient’stive participation in prescribed medical regimens and life-yles. In addition, patients should be informed of the risks,nefits, and alternatives to a particular treatment and shouldinvolved in shared decision making whenever feasible,

rticularly for COR IIa and IIb, for which the benefit-to-risktio may be lower.The Task Force makes every effort to avoid actual, poten-

al, or perceived conflicts of interest that may arise as a resultindustry relationships or personal interests among the

embers of the writing group. All writing group membersd peer reviewers of the guideline are required to disclose allrrent healthcare–related relationships, including those ex-

ting 12 months before initiation of the writing effort.For the 2008 guidelines, all members of the writingmmittee, as well as peer reviewers of the document, wereked to provide disclosure statements of all such relation-ips that may be perceived as real or potential conflicts ofterest. Writing committee members are also strongly en-uraged to declare a previous relationship with industry thatay be perceived as relevant to guideline development.In December 2009, the ACCF and AHA implemented aw policy for relationships with industry and other entitiesWI) that requires the writing group chair plus a minimum50% of the writing group to have no relevant RWI

ppendix 4 includes the ACCF/AHA definition of rele-nce). These statements are reviewed by the Task Force andl members during each conference call and/or meeting ofe writing group and are updated as changes occur. Allideline recommendations require a confidential vote by the

riting group and must be approved by a consensus of theting members. Members may not draft or vote on any textrecommendations pertaining to their RWI. The 2012

embers who recused themselves from voting are indicatedthe list of writing group members, and specific section

cusals are noted in Appendix 4. 2008 and 2012 authors’ and
er reviewers’ RWI pertinent to this guideline are disclosed re
Appendixes 1, 2, 4, and 5, respectively. Additionally, tosure complete transparency, writing group members’ com-ehensive disclosure information—including RWI not per-

nent to this document—is available as an online supplement.omprehensive disclosure information for the Task Force isso available online at http://cardiosource.org/ACC/About-CC/Who-We-Are/Leadership/Guidelines-and-Documents-ask-Forces.aspx. The work of the 2012 writing group ispported exclusively by the ACCF, AHA, and the Hearthythm Society (HRS) without commercial support. Writingoup members volunteered their time for this activity.uidelines are official policy of both the ACCF and AHA.In April 2011, the Institute of Medicine released 2 reports:

inding What Works in Health Care: Standards for System-ic Reviews and Clinical Practice Guidelines We Can Trustb,1c). It is noteworthy that the ACCF/AHA practice guide-

nes were cited as being compliant with many of theandards that were proposed. A thorough review of theseports and our current methodology is under way, withrther enhancements anticipated.The current document is a republication of the “ACCF/

HA/HRS 2008 Guidelines for Device-Based Therapy ofardiac Rhythm Abnormalities,” (1d) revised to incorporatedated recommendations and text from the 2012 Focused

pdate (1). For easy reference, this online-only versionnotes sections that have been updated.

Jeffrey L. Anderson, MD, FACC, FAHAChair, ACCF/AHA Task Force on Practice Guidelines

1. Introduction (UPDATED)

.1. Organization of Committeehis 2008 revision of the ACCF/AHA/HRS Guidelines forevice-Based Therapy of Cardiac Rhythm Abnormalitiesormally named “ACC/AHA/NASPE Guidelines for Implan-tion of Cardiac Pacemakers and Antiarrhythmia Devices”)dates the previous versions published in 1984, 1991, 1998,d 2002. Revision of the statement was deemed necessaryr multiple reasons: 1) Major studies have been reported thatve advanced our knowledge of the natural history ofadyarrhythmias and tachyarrhythmias, which may beeated optimally with device therapy; 2) there have beenemendous changes in the management of heart failure thatvolve both drug and device therapy; and 3) major advancesthe technology of devices to treat, delay, and even prevent

orbidity and mortality from bradyarrhythmias, tachyar-ythmias, and heart failure have occurred. The writingmmittee was composed of physicians who are experts ine areas of device therapy and follow-up and senior clini-ans skilled in cardiovascular care, internal medicine, car-ovascular surgery, ethics, and socioeconomics. The com-ittee included representatives of the American Associationr Thoracic Surgery, Heart Failure Society of America, and

ociety of Thoracic Surgeons.For the 2012 focused update, selected members of the 2008

evice-Based Therapy (DBT) Writing Committee were in-ted to participate on the basis of areas of expertise,
quirements for committee rotation, and the current RWI
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licy; those who agreed are referred to as the 2012 Focusedpdate Writing Group. The HRS was invited to be a partner

this focused update and has provided representation. Theriting group also included representatives from the Ameri-n Association for Thoracic Surgery, Heart Failure SocietyAmerica, and Society of Thoracic Surgeons.

.2. Document Review and Approvalhe 2008 Guideline document was reviewed by 2 officialviewers nominated by each of the ACC, AHA, and HRSd by 11 additional peer reviewers. Of the total 17 peerviewers, 10 had no significant relevant relationships withdustry. In addition, this document has been reviewed andproved by the governing bodies of the ACC, AHA, andRS, which include 19 ACC Board of Trustees membersone of whom had any significant relevant relationships withdustry), 15 AHA Science Advisory Coordinating Commit-e members (none of whom had any significant relevantlationships with industry), and 14 HRS Board of Trusteesembers (6 of whom had no significant relevant relationshipsith industry). All guideline recommendations underwent armal, blinded writing committee vote. Writing committeeembers were required to recuse themselves if they had agnificant relevant relationship with industry. The guidelinecommendations were unanimously approved by all mem-rs of the writing committee who were eligible to vote. Thection “Pacing in Children and Adolescents” was reviewed

additional reviewers with special expertise in pediatricectrophysiology. The committee thanks all the reviewers foreir comments. Many of their suggestions were incorporatedto the final document.The 2012 focused update was reviewed by 2 officialviewers each nominated by the ACCF, AHA, and HRS, asell as 1 reviewer each from the American Association forhoracic Surgery, Heart Failure Society of America, andociety of Thoracic Surgeons, and 21 individual contentviewers. All information on reviewers’ RWI was collectedd distributed to the writing group and is published in thiscument (Appendix 5). The 2012 focused update was approvedr publication by the governing bodies of the ACCF, AHA, andRS and was endorsed by the American Association forhoracic Surgery, Heart Failure Society of America, and Society

Thoracic Surgeons.

.3. Methodology and Evidencehe recommendations listed in this document are, wheneverssible, evidence based. An extensive literature survey wasnducted that led to the incorporation of 595 references.

earches were limited to studies, reviews, and other evidencenducted in human subjects and published in English. Keyarch words included but were not limited to antiarrhythmic,tibradycardia, atrial fibrillation, bradyarrhythmia, cardiac,

RT, defibrillator, device therapy, devices, dual chamber,art, heart failure, ICD, implantable defibrillator, deviceplantation, long-QT syndrome, medical therapy, pace-

aker, pacing, quality-of-life, resynchronization, rhythm,nus node dysfunction, sleep apnea, sudden cardiac death,ncope, tachyarrhythmia, terminal care, and transplantation.
dditionally, the committee reviewed documents related to fa
e subject matter previously published by the ACC, AHA,d HRS. References selected and published in this documente representative and not all-inclusive.The focus of the 2008 guidelines is the appropriate use ofart pacing devices (e.g., pacemakers for bradyarrhythmiasd heart failure management, cardiac resynchronization, andplantable cardioverter-defibrillators [ICDs]), not the treat-

ent of cardiac arrhythmias. The fact that the use of a devicer treatment of a particular condition is listed as a Class Idication (beneficial, useful, and effective) does not precludee use of other therapeutic modalities that may be equallyfective. As with all clinical practice guidelines, the recom-endations in this document focus on treatment of an averagetient with a specific disorder and may be modified bytient comorbidities, limitation of life expectancy because ofexisting diseases, and other situations that only the primary

eating physician may evaluate appropriately.These guidelines include sections on selection of pacemak-s and ICDs, optimization of technology, cost, and follow-upimplanted devices. Although the section on follow-up is

latively brief, its importance cannot be overemphasized:irst, optimal results from an implanted device can betained only if the device is adjusted to changing clinicalnditions; second, recent advisories and recalls serve asarnings that devices are not infallible, and failure ofectronics, batteries, and leads can occur (2,3).The committee considered including a section on extrac-

on of failed/unused leads, a topic of current interest, butected not to do so in the absence of convincing evidence topport specific criteria for timing and methods of leadtraction. A policy statement on lead extraction from theorth American Society of Pacing and Electrophysiologyow the HRS) provides information on this topic (4).

imilarly, the issue of when to discontinue long-term cardiaccing or defibrillator therapy has not been studied suffi-ently to allow formulation of appropriate guidelines (5);wever, the question is of such importance that this topic isdressed to emphasize the importance of patient-family-ysician discussion and ethical principles.The text that accompanies the listed indications should bead carefully, because it includes the rationale and support-g evidence for many of the indications, and in severalstances, it includes a discussion of alternative acceptableerapies. Many of the indications are modified by the termotentially reversible.” This term is used to indicate abnor-al pathophysiology (e.g., complete heart block) that may bee result of reversible factors. Examples include completeart block due to drug toxicity (digitalis), electrolyte abnor-alities, diseases with periatrioventricular node inflammationyme disease), and transient injury to the conduction systemthe time of open heart surgery. When faced with a

tentially reversible situation, the treating physician mustcide how long of a waiting period is justified before deviceerapy is begun. The committee recognizes that this state-ent does not address the issue of length of hospital stays-à-vis managed-care regulations. It is emphasized thatese guidelines are not intended to address this issue, which
lls strictly within the purview of the treating physician.

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The term “symptomatic bradycardia” is used in this docu-ent. Symptomatic bradycardia is defined as a documentedadyarrhythmia that is directly responsible for developmentthe clinical manifestations of syncope or near syncope,

ansient dizziness or lightheadedness, or confusional statessulting from cerebral hypoperfusion attributable to slowart rate. Fatigue, exercise intolerance, and congestive heartilure may also result from bradycardia. These symptomsay occur at rest or with exertion. Definite correlation ofmptoms with a bradyarrhythmia is required to fulfill theiteria that define symptomatic bradycardia. Caution should

exercised not to confuse physiological sinus bradycardias occurs in highly trained athletes) with pathological bra-arrhythmias. Occasionally, symptoms may become appar-t only in retrospect after antibradycardia pacing. Neverthe-ss, the universal application of pacing therapy to treat aecific heart rate cannot be recommended except in specificrcumstances, as detailed subsequently.In these guidelines, the terms “persistent,” “transient,” andot expected to resolve” are used but not specifically definedcause the time element varies in different clinical condi-

ons. The treating physician must use appropriate clinicaldgment and available data in deciding when a condition isrsistent or when it can be expected to be transient. Section1.4, “Pacing for Atrioventricular Block Associated Withcute Myocardial Infarction,” overlaps with the “ACC/AHAuidelines for the Management of Patients With ST-levation Myocardial Infarction” (6) and includes expandeddications and stylistic changes. The statement “incidentalnding at electrophysiological study” is used several times inis document and does not mean that such a study isdicated. Appropriate indications for electrophysiologicaludies have been published (7).The section on indications for ICDs has been updated toflect the numerous new developments in this field and theluminous literature related to the efficacy of these devicesthe treatment and prophylaxis of sudden cardiac death

CD) and malignant ventricular arrhythmias. As previouslyted, indications for ICDs, cardiac resynchronization ther-y (CRT) devices, and combined ICDs and CRT devicesereafter called CRT-Ds) are continuously changing and can

expected to change further as new trials are reported.deed, it is inevitable that the indications for deviceerapy will be refined with respect to both expanded used the identification of patients expected to benefit theost from these therapies. Furthermore, it is emphasizedat when a patient has an indication for both a pacemakerhether it be single-chamber, dual-chamber, or biven-

icular) and an ICD, a combined device with appropriateogramming is indicated.In this document, the term “mortality” is used to indicate

l-cause mortality unless otherwise specified. The committeeected to use all-cause mortality because of the variablefinition of sudden death and the developing consensus toe all-cause mortality as the most appropriate end point ofinical trials (8,9).These guidelines are not designed to specify training oredentials required for physicians to use device therapy.
evertheless, in view of the complexity of both the cognitive
d technical aspects of device therapy, only appropriatelyained physicians should use device therapy. Appropriateaining guidelines for physicians have been published previ-sly (10–13).The 2008 revision reflects what the committee believes aree most relevant and significant advances in pacemaker/ICDerapy since the publication of these guidelines in the Journal ofe American College of Cardiology and Circulation in 20024,15).All recommendations assume that patients are treated withtimal medical therapy according to published guidelines, asd been required in all the randomized controlled clinical

ials on which these guidelines are based, and that humansues related to individual patients are addressed. Themmittee believes that comorbidities, life expectancy, andality-of-life (QOL) issues must be addressed forthrightly

ith patients and their families. We have repeatedly used therase “reasonable expectation of survival with a goodnctional status for more than 1 year” to emphasize thistegration of factors in decision-making. Even when physi-ans believe that the anticipated benefits warrant deviceplantation, patients have the option to decline intervention

ter having been provided with a full explanation of thetential risks and benefits of device therapy. Finally, themmittee is aware that other guideline/expert groups haveterpreted the same data differently (16–19).In preparing this revision, the committee was guided by thellowing principles:

Changes in recommendations and levels of evidence weremade either because of new randomized trials or becauseof the accumulation of new clinical evidence and thedevelopment of clinical consensus.The committee was cognizant of the health care, logistic,and financial implications of recent trials and factored inthese considerations to arrive at the classification ofcertain recommendations.For recommendations taken from other guidelines, word-ing changes were made to render some of the originalrecommendations more precise.The committee would like to reemphasize that the recom-mendations in this guideline apply to most patients butmay require modification because of existing situationsthat only the primary treating physician can evaluateproperly.All of the listed recommendations for implantation of adevice presume the absence of inciting causes that may beeliminated without detriment to the patient (e.g., nones-sential drug therapy).The committee endeavored to maintain consistency ofrecommendations in this and other previously publishedguidelines. In the section on atrioventricular (AV) blockassociated with acute myocardial infarction (AMI), therecommendations follow closely those in the “ACC/AHAGuidelines for the Management of Patients With ST-Elevation Myocardial Infarction” (6). However, becauseof the rapid evolution of pacemaker/ICD science, it hasnot always been possible to maintain consistency with
other published guidelines.

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or the 2012 focused update, late-breaking clinical trialsesented at the annual scientific meetings of the ACC, AHA,RS, and European Society of Cardiology (2008 through10), as well as other selected data reported through Febru-y 2012, were reviewed by the guideline writing group alongith the Task Force and other experts to identify trials andher key data that might affect guideline recommendations.tudies relevant to the management of patients treated withBT for cardiac rhythm abnormalities were identified andviewed. On the basis of these data, the writing grouptermined that updates to the 2008 guideline were necessaryr cardiac resynchronization therapy (CRT) and devicellow-up. The writing group also thoroughly reviewed otherctions from the 2008 DBT guideline on hypertrophicrdiomyopathy, arrhythmogenic right ventricular dysplasia/rdiomyopathy, genetic arrhythmia syndromes, congenitalart disease, primary electrical disease, and terminal care;d determined that although some new information may beailable, the recommendations remain current. See the 2012cused update for a complete review of the scope (1).

2. Indications for Pacing

.1. Pacing for Bradycardia Due to Sinus andtrioventricular Node Dysfunctionsome patients, bradycardia is the consequence of essen-

al long-term drug therapy of a type and dose for whichere is no acceptable alternative. In these patients, pacingerapy is necessary to allow maintenance of ongoingedical treatment.

.1.1. Sinus Node Dysfunctioninus node dysfunction (SND) was first described as ainical entity in 1968 (20), although Wenckebach reportede electrocardiographic (ECG) manifestation of SND in23. SND refers to a broad array of abnormalities in sinusde and atrial impulse formation and propagation. Theseclude persistent sinus bradycardia and chronotropic incom-tence without identifiable causes, paroxysmal or persistent

nus arrest with replacement by subsidiary escape rhythms ine atrium, AV junction, or ventricular myocardium. Theequent association of paroxysmal atrial fibrillation (AF) andnus bradycardia or sinus bradyarrhythmias, which maycillate suddenly from one to the other, usually accompaniedsymptoms, is termed “tachy-brady syndrome.”

SND is primarily a disease of the elderly and is presumedbe due to senescence of the sinus node and atrial muscle.

ollected data from 28 different studies on atrial pacing forND showed a median annual incidence of complete AVock of 0.6% (range 0% to 4.5%) with a total prevalence of1% (range 0% to 11.9%) (21). This suggests that thegenerative process also affects the specialized conductionstem, although the rate of progression is slow and does notminate the clinical course of disease (21). SND is typicallyagnosed in the seventh and eighth decades of life, which isso the average age at enrollment in clinical trials ofcemaker therapy for SND (22,23). Identical clinical man-

estations may occur at any age as a secondary phenomenon
any condition that results in destruction of sinus node cells, ph
ch as ischemia or infarction, infiltrative disease, collagenscular disease, surgical trauma, endocrinologic abnormali-

es, autonomic insufficiency, and others (24).The clinical manifestations of SND are diverse, reflecting

e range of typical sinoatrial rhythm disturbances. The mostamatic presentation is syncope. The mechanism of syncopea sudden pause in sinus impulse formation or sinus exit

ock, either spontaneously or after the termination of anrial tachyarrhythmia, that causes cerebral hypoperfusion.he pause in sinus node activity is frequently accompanied

an inadequate, delayed, or absent response of subsidiarycape pacemakers in the AV junction or ventricular myo-rdium, which aggravates the hemodynamic consequences.However, in many patients, the clinical manifestations of

ND are more insidious and relate to an inadequate heart ratesponse to activities of daily living that can be difficult toagnose (25). The term “chronotropic incompetence” is used

denote an inadequate heart rate response to physicaltivity. Although many experienced clinicians claim tocognize chronotropic incompetence in individual patients,single metric has been established as a diagnostic standardon which therapeutic decisions can be based. The mostvious example of chronotropic incompetence is a mono-nic daily heart rate profile in an ambulatory patient. Variousotocols have been proposed to quantify subphysiologicalart rate responses to exercise (26,27), and many cliniciansould consider failure to achieve 80% of the maximumedicted heart rate (220 minus age) at peak exercise asidence of a blunted heart rate response (28,29). However,ne of these approaches have been validated clinically, andis likely that the appropriate heart rate response to exerciseindividual patients is too idiosyncratic for standardized

sting.The natural history of untreated SND may be highlyriable. The majority of patients who have experiencedncope because of a sinus pause or marked sinus bradycar-a will have recurrent syncope (30). Not uncommonly, thetural history of SND is interrupted by other necessaryedical therapies that aggravate the underlying tendency toadycardia (24). MOST (Mode Selection Trial) includedmptomatic pauses greater than or equal to 3 seconds ornus bradycardia with rates greater than 50 bpm, whichstricted the use of indicated long-term medical therapy.upraventricular tachycardia (SVT) including AF was pres-t in 47% and 53% of patients, respectively, enrolled in arge randomized clinical trial of pacing mode selection inND (22,31). The incidence of sudden death is extremelyw, and SND does not appear to affect survival whethertreated (30) or treated with pacemaker therapy (32,33).The only effective treatment for symptomatic bradycardiapermanent cardiac pacing. The decision to implant a

cemaker for SND is often accompanied by uncertainty thatises from incomplete linkage between sporadic symptomsd ECG evidence of coexisting bradycardia. It is crucial tostinguish between physiological bradycardia due to auto-mic conditions or training effects and circumstantiallyappropriate bradycardia that requires permanent cardiaccing. For example, sinus bradycardia is accepted as a
ysiological finding that does not require cardiac pacing in

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ained athletes. Such individuals may have heart rates of 4050 bpm while at rest and awake and may have a sleeping

te as slow as 30 bpm, with sinus pauses or progressive sinusowing accompanied by AV conduction delay (PR prolon-tion), sometimes culminating in type I second-degree AVock (34,35). The basis of the distinction between physio-gical and pathological bradycardia, which may overlap inCG presentation, therefore pivots on correlation of episodicadycardia with symptoms compatible with cerebral hypo-rfusion. Intermittent ECG monitoring with Holter monitorsd event recorders may be helpful (36,37), although theration of monitoring required to capture such evidence mayvery long (38). The use of insertable loop recorders offers

e advantages of compliance and convenience during veryng-term monitoring efforts (39).The optimal pacing system for prevention of symptomaticadycardia in SND is unknown. Recent evidence suggestsat ventricular desynchronization due to right ventricularical (RVA) pacing may have adverse effects on leftntricular (LV) and left atrial structure and function (40–47).

hese adverse effects likely explain the association of RVAcing, independent of AV synchrony, with increased risks ofF and heart failure in randomized clinical trials of pace-aker therapy (45,48,49) and, additionally, ventricular ar-ythmias and death during ICD therapy (50,51). Likewise,though simulation of the normal sinus node response toercise in bradycardia patients with pacemaker sensorsems logical, a clinical benefit on a population scale hast been demonstrated in large randomized controlled

ials of pacemaker therapy (52). These rapidly evolvingeas of clinical investigation should inform the choice ofcing system in SND (see Section 2.6, “Selection of

acemaker Device”).

ecommendations for Permanent Pacing ininus Node Dysfunction

ASS I

Permanent pacemaker implantation is indicated for SND withdocumented symptomatic bradycardia, including frequent si-nus pauses that produce symptoms. (Level of Evidence: C)(53–55)Permanent pacemaker implantation is indicated for symp-tomatic chronotropic incompetence. (Level of Evidence: C)(53–57)Permanent pacemaker implantation is indicated for symptom-atic sinus bradycardia that results from required drug therapyfor medical conditions. (Level of Evidence: C)

ASS IIa

Permanent pacemaker implantation is reasonable for SND withheart rate less than 40 bpm when a clear association betweensignificant symptoms consistent with bradycardia and theactual presence of bradycardia has not been documented.(Level of Evidence: C) (53–55,58–60)Permanent pacemaker implantation is reasonable for syncopeof unexplained origin when clinically significant abnormalitiesof sinus node function are discovered or provoked in electro-
physiological studies. (Level of Evidence: C) (61,62) bl
ASS IIb

Permanent pacemaker implantation may be considered inminimally symptomatic patients with chronic heart rate lessthan 40 bpm while awake. (Level of Evidence: C) (53,55,56,58–60)

ASS III

Permanent pacemaker implantation is not indicated for SND inasymptomatic patients. (Level of Evidence: C)Permanent pacemaker implantation is not indicated for SND inpatients for whom the symptoms suggestive of bradycardiahave been clearly documented to occur in the absence ofbradycardia. (Level of Evidence: C)Permanent pacemaker implantation is not indicated for SNDwith symptomatic bradycardia due to nonessential drug ther-apy. (Level of Evidence: C)

.1.2. Acquired Atrioventricular Block in AdultsV block is classified as first-, second-, or third-degreeomplete) block; anatomically, it is defined as supra-, intra-,infra-His. First-degree AV block is defined as abnormal

olongation of the PR interval (greater than 0.20 seconds).econd-degree AV block is subclassified as type I and type II.ype I second-degree AV block is characterized by progres-ve prolongation of the interval between the onset of atrial (Pave) and ventricular (R wave) conduction (PR) before anconducted beat and is usually seen in conjunction with

RS. Type I second-degree AV block is characterized byogressive prolongation of the PR interval before a noncon-cted beat and a shorter PR interval after the blocked beat.

ype II second-degree AV block is characterized by fixed PRtervals before and after blocked beats and is usuallysociated with a wide QRS complex. When AV conductioncurs in a 2:1 pattern, block cannot be classified unequivo-lly as type I or type II, although the width of the QRS cansuggestive, as just described. Advanced second-degree AV

ock refers to the blocking of 2 or more consecutive P wavesith some conducted beats, which indicates some preserva-on of AV conduction. In the setting of AF, a prolongeduse (e.g., greater than 5 seconds) should be considered todue to advanced second-degree AV block. Third-degree

V block (complete heart block) is defined as absence of AVnduction.Patients with abnormalities of AV conduction may beymptomatic or may experience serious symptoms related toadycardia, ventricular arrhythmias, or both. Decisions re-rding the need for a pacemaker are importantly influencedthe presence or absence of symptoms directly attributablebradycardia. Furthermore, many of the indications for

cing have evolved over the past 40 years on the basis ofperience without the benefit of comparative randomizedinical trials, in part because no acceptable alternativetions exist to treat most bradycardias.Nonrandomized studies strongly suggest that permanentcing does improve survival in patients with third-degreeV block, especially if syncope has occurred (63–68).lthough there is little evidence to suggest that pacemakersprove survival in patients with isolated first-degree AV

ock (69), it is now recognized that marked (PR more than

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0 milliseconds) first-degree AV block can lead to symp-ms even in the absence of higher degrees of AV block (70).hen marked first-degree AV block for any reason causesrial systole in close proximity to the preceding ventricularstole and produces hemodynamic consequences usuallysociated with retrograde (ventriculoatrial) conduction, signsd symptoms similar to the pacemaker syndrome may occur1). With marked first-degree AV block, atrial contractioncurs before complete atrial filling, ventricular filling ismpromised, and an increase in pulmonary capillary wedgeessure and a decrease in cardiac output follow. Smallcontrolled trials have suggested some symptomatic andnctional improvement by pacing of patients with PR inter-ls more than 0.30 seconds by decreasing the time for AVnduction (70). Finally, a long PR interval may identify abgroup of patients with LV dysfunction, some of whomay benefit from dual-chamber pacing with a short(er) AVlay (72). These same principles also may be applied totients with type I second-degree AV block who experiencemodynamic compromise due to loss of AV synchrony,en without bradycardia. Although echocardiographic orvasive techniques may be used to assess hemodynamicprovement before permanent pacemaker implantation,ch studies are not required.Type I second-degree AV block is usually due to delay in

e AV node irrespective of QRS width. Because progressionadvanced AV block in this situation is uncommon (73–75),cing is usually not indicated unless the patient is symptom-ic. Although controversy exists, pacemaker implantation ispported for this finding (76–78). Type II second-degree AVock is usually infranodal (either intra- or infra-His), espe-ally when the QRS is wide. In these patients, symptoms areequent, prognosis is compromised, and progression toird-degree AV block is common and sudden (73,75,79).hus, type II second-degree AV block with a wide QRSpically indicates diffuse conduction system disease andnstitutes an indication for pacing even in the absence ofmptoms. However, it is not always possible to determinee site of AV block without electrophysiological evaluation,cause type I second-degree AV block can be infranodalen when the QRS is narrow (80). If type I second-degreeV block with a narrow or wide QRS is found to be intra- orfra-Hisian at electrophysiological study, pacing should bensidered.Because it may be difficult for both patients and theirysicians to attribute ambiguous symptoms such as fatiguebradycardia, special vigilance must be exercised to ac-owledge the patient’s concerns about symptoms that maycaused by a slow heart rate. In a patient with third-degree

V block, permanent pacing should be strongly considereden when the ventricular rate is more than 40 bpm, becausee choice of a 40 bpm cutoff in these guidelines was nottermined from clinical trial data. Indeed, it is not the escapete that is necessarily critical for safety but rather the site ofigin of the escape rhythm (i.e., in the AV node, the Hisndle, or infra-His).AV block can sometimes be provoked by exercise. If notcondary to myocardial ischemia, AV block in this circum-
ance usually is due to disease in the His-Purkinje system
d is associated with a poor prognosis; thus, pacing isdicated (81,82). Long sinus pauses and AV block can alsocur during sleep apnea. In the absence of symptoms,ese abnormalities are reversible and do not requirecing (83). If symptoms are present, pacing is indicatedin other conditions.Recommendations for permanent pacemaker implantationpatients with AV block in AMI, congenital AV block, and

V block associated with enhanced vagal tone are discussedseparate sections. Neurocardiogenic causes in young pa-

ents with AV block should be assessed before proceedingith permanent pacing. Physiological AV block in theesence of supraventricular tachyarrhythmias does not con-itute an indication for pacemaker implantation except asecifically defined in the recommendations that follow.In general, the decision regarding implantation of a pace-

aker must be considered with respect to whether AV blockill be permanent. Reversible causes of AV block, such asectrolyte abnormalities, should be corrected first. Someseases may follow a natural history to resolution (e.g.,yme disease), and some AV block can be expected toverse (e.g., hypervagotonia due to recognizable and avoid-le physiological factors, perioperative AV block due topothermia, or inflammation near the AV conduction systemter surgery in this region). Conversely, some conditionsay warrant pacemaker implantation because of the pos-bility of disease progression even if the AV blockverses transiently (e.g., sarcoidosis, amyloidosis, anduromuscular diseases). Finally, permanent pacing forV block after valve surgery follows a variable naturalstory; therefore, the decision for permanent pacing is ate physician’s discretion (84).

ecommendations for Acquiredtrioventricular Block in Adults

ASS I

Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel associated with bradycardia with symptoms (includingheart failure) or ventricular arrhythmias presumed to be due toAV block. (Level of Evidence: C) (59,63,76,85)Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel associated with arrhythmias and other medical conditionsthat require drug therapy that results in symptomatic bradycar-dia. (Level of Evidence: C) (59,63,76,85)Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel in awake, symptom-free patients in sinus rhythm, withdocumented periods of asystole greater than or equal to 3.0seconds (86) or any escape rate less than 40 bpm, or with anescape rhythm that is below the AV node. (Level of Evidence: C)(53,58)Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel in awake, symptom-free patients with AF and bradycardiawith 1 or more pauses of at least 5 seconds or longer. (Level of
Evidence: C)

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Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel after catheter ablation of the AV junction. (Level ofEvidence: C) (87,88)Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel associated with postoperative AV block that is not expectedto resolve after cardiac surgery. (Level of Evidence: C)(84,85,89,90)Permanent pacemaker implantation is indicated for third-degree and advanced second-degree AV block at any anatomiclevel associated with neuromuscular diseases with AV block,such as myotonic muscular dystrophy, Kearns-Sayre syndrome,Erb dystrophy (limb-girdle muscular dystrophy), and peronealmuscular atrophy, with or without symptoms. (Level of Evi-dence: B) (91–97)Permanent pacemaker implantation is indicated for second-degree AV block with associated symptomatic bradycardiaregardless of type or site of block. (Level of Evidence: B) (74)Permanent pacemaker implantation is indicated for asymptom-atic persistent third-degree AV block at any anatomic site withaverage awake ventricular rates of 40 bpm or faster if cardio-megaly or LV dysfunction is present or if the site of block isbelow the AV node. (Level of Evidence: B) (76,78). Permanent pacemaker implantation is indicated for second-

or third-degree AV block during exercise in the absence ofmyocardial ischemia. (Level of Evidence: C) (81,82)

ASS IIa

Permanent pacemaker implantation is reasonable for persis-tent third-degree AV block with an escape rate greater than 40bpm in asymptomatic adult patients without cardiomegaly.(Level of Evidence: C) (59,63,64,76,82,85)Permanent pacemaker implantation is reasonable for asymp-tomatic second-degree AV block at intra- or infra-His levelsfound at electrophysiological study. (Level of Evidence: B)(74,76,78)Permanent pacemaker implantation is reasonable for first- orsecond-degree AV block with symptoms similar to those ofpacemaker syndrome or hemodynamic compromise. (Level ofEvidence: B) (70,71)Permanent pacemaker implantation is reasonable for asymp-tomatic type II second-degree AV block with a narrow QRS.When type II second-degree AV block occurs with a wide QRS,including isolated right bundle-branch block, pacing becomes aClass I recommendation. (See Section 2.1.3, “Chronic Bifas-cicular Block.”) (Level of Evidence: B) (70,76,80,85)

ASS IIb

Permanent pacemaker implantation may be considered forneuromuscular diseases such as myotonic muscular dystrophy,Erb dystrophy (limb-girdle muscular dystrophy), and peronealmuscular atrophy with any degree of AV block (includingfirst-degree AV block), with or without symptoms, becausethere may be unpredictable progression of AV conductiondisease. (Level of Evidence: B) (91–97)Permanent pacemaker implantation may be considered for AVblock in the setting of drug use and/or drug toxicity when theblock is expected to recur even after the drug is withdrawn.
(Level of Evidence: B) (98,99) in
ASS III

Permanent pacemaker implantation is not indicated for asymp-tomatic first-degree AV block. (Level of Evidence: B) (69) (SeeSection 2.1.3, “Chronic Bifascicular Block.”)Permanent pacemaker implantation is not indicated for asymp-tomatic type I second-degree AV block at the supra-His (AVnode) level or that which is not known to be intra- or infra-Hisian. (Level of Evidence: C) (74)Permanent pacemaker implantation is not indicated for AVblock that is expected to resolve and is unlikely to recur (100)(e.g., drug toxicity, Lyme disease, or transient increases invagal tone or during hypoxia in sleep apnea syndrome in theabsence of symptoms). (Level of Evidence: B) (99,100)

.1.3. Chronic Bifascicular Blockifascicular block refers to ECG evidence of impairednduction below the AV node in the right and left bundles.lternating bundle-branch block (also known as bilateralndle-branch block) refers to situations in which clear ECGidence for block in all 3 fascicles is manifested onccessive ECGs. Examples are right bundle-branch blockd left bundle-branch block on successive ECGs or rightndle-branch block with associated left anterior fascicularock on 1 ECG and associated left posterior fascicular block

another ECG. Patients with first-degree AV block insociation with bifascicular block and symptomatic, ad-nced AV block have a high mortality rate and a substan-

al incidence of sudden death (64,101). Although third-gree AV block is most often preceded by bifascicularock, there is evidence that the rate of progression offascicular block to third-degree AV block is slow (102).urthermore, no single clinical or laboratory variable,cluding bifascicular block, identifies patients at high risk

death due to a future bradyarrhythmia caused byndle-branch block (103).Syncope is common in patients with bifascicular block.

lthough syncope may be recurrent, it is not associated withincreased incidence of sudden death (73,102–112). Even

ough pacing relieves the neurological symptoms, it does notduce the occurrence of sudden death (108). An electrophys-logical study may be helpful to evaluate and direct theeatment of inducible ventricular arrhythmias (113,114) thate common in patients with bifascicular block. There isnvincing evidence that in the presence of permanent or

ansient third-degree AV block, syncope is associated withincreased incidence of sudden death regardless of the

sults of the electrophysiological study (64,114,115). Fi-lly, if the cause of syncope in the presence of bifascicularock cannot be determined with certainty, or if treatments useduch as drugs) may exacerbate AV block, prophylactic perma-nt pacing is indicated, especially if syncope may have beene to transient third-degree AV block (102–112,116).Of the many laboratory variables, the PR and HV intervalsve been identified as possible predictors of third-degree AVock and sudden death. Although PR-interval prolongation ismmon in patients with bifascicular block, the delay is oftenthe level of the AV node. There is no correlation between

e PR and HV intervals or between the length of the PR
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ath (107,109,116). Although most patients with chronic ortermittent third-degree AV block demonstrate prolongation

the HV interval during anterograde conduction, somevestigators (110,111) have suggested that asymptomatictients with bifascicular block and a prolonged HV intervalould be considered for permanent pacing, especially if theV interval is greater than or equal to 100 milliseconds (109).lthough the prevalence of HV-interval prolongation is high,e incidence of progression to third-degree AV block is low.ecause HV prolongation accompanies advanced cardiacsease and is associated with increased mortality, death isten not sudden or due to AV block but rather is due to thederlying heart disease itself and nonarrhythmic cardiacuses (102,103,108,109,111,114–117).Atrial pacing at electrophysiological study in asymptom-

ic patients as a means of identifying patients at increasedsk of future high- or third-degree AV block is controver-al. The probability of inducing block distal to the AVde (i.e., intra- or infra-His) with rapid atrial pacing isw (102,110,111,118–121). Failure to induce distal blocknnot be taken as evidence that the patient will not developird-degree AV block in the future. However, if atrial pacingduces nonphysiological infra-His block, some consider thisindication for pacing (118). Nevertheless, infra-His block

at occurs during either rapid atrial pacing or programmedimulation at short coupling intervals may be physiologicald not pathological, simply reflecting disparity betweenfractoriness of the AV node and His-Purkinje systems22).

ecommendations for Permanent Pacing inhronic Bifascicular Block

ASS I

Permanent pacemaker implantation is indicated for advancedsecond-degree AV block or intermittent third-degree AV block.(Level of Evidence: B) (63–68,101)Permanent pacemaker implantation is indicated for type IIsecond-degree AV block. (Level of Evidence: B) (73,75,79,123)Permanent pacemaker implantation is indicated for alternatingbundle-branch block. (Level of Evidence: C) (124)

ASS IIa

Permanent pacemaker implantation is reasonable for syncopenot demonstrated to be due to AV block when other likelycauses have been excluded, specifically ventricular tachycar-dia (VT). (Level of Evidence: B) (102–111,113–119,123,125)Permanent pacemaker implantation is reasonable for an inci-dental finding at electrophysiological study of a markedlyprolonged HV interval (greater than or equal to 100 millisec-onds) in asymptomatic patients. (Level of Evidence: B) (109)Permanent pacemaker implantation is reasonable for anincidental finding at electrophysiological study of pacing-induced infra-His block that is not physiological. (Level ofEvidence: B) (118)

ASS IIb

Permanent pacemaker implantation may be considered in thesetting of neuromuscular diseases such as myotonic muscular
dystrophy, Erb dystrophy (limb-girdle muscular dystrophy), and in
peroneal muscular atrophy with bifascicular block or any fas-cicular block, with or without symptoms. (Level of Evidence: C)(91–97)

ASS III

Permanent pacemaker implantation is not indicated for fascicularblock without AV block or symptoms. (Level of Evidence: B)(103,107,109,116)Permanent pacemaker implantation is not indicated for fascic-ular block with first-degree AV block without symptoms. (Levelof Evidence: B) (103,107,109,116)

.1.4. Pacing for Atrioventricular Block Associatedith Acute Myocardial Infarction

dications for permanent pacing after myocardial infarctionI) in patients experiencing AV block are related in large

easure to the presence of intraventricular conduction de-cts. The criteria for patients with MI and AV block do notcessarily depend on the presence of symptoms. Further-ore, the requirement for temporary pacing in AMI does not

itself constitute an indication for permanent pacing (seeCC/AHA Guidelines for the Management of Patients With

T-Elevation Myocardial Infarction” (6)). The long-termognosis for survivors of AMI who have had AV block islated primarily to the extent of myocardial injury and thearacter of intraventricular conduction disturbances ratheran the AV block itself (66,126–130). Patients with AMIho have intraventricular conduction defects, with the excep-on of isolated left anterior fascicular block, have an unfa-rable short- and long-term prognosis and an increased risksudden death (66,79,126,128,130). This unfavorable prog-sis is not necessarily due to development of high-grade AVock, although the incidence of such block is higher instinfarction patients with abnormal intraventricular con-ction (126,131,132).When AV or intraventricular conduction block compli-tes AMI, the type of conduction disturbance, location offarction, and relation of electrical disturbance to infarctionust be considered if permanent pacing is contemplated.ven with data available, the decision is not always straight-rward, because the reported incidence and significance ofrious conduction disturbances vary widely (133). Despitee use of thrombolytic therapy and primary angioplasty,hich have decreased the incidence of AV block in AMI,ortality remains high if AV block occurs (130,134–137).Although more severe disturbances in conduction havenerally been associated with greater arrhythmic and non-rhythmic mortality, (126–129,131,133) the impact of pre-isting bundle-branch block on mortality after AMI isntroversial (112,133). A particularly ominous prognosis issociated with left bundle-branch block combined withvanced second- or third-degree AV block and with rightndle-branch block combined with left anterior or leftsterior fascicular block (105,112,127,129). Regardless of

hether the infarction is anterior or inferior, the developmentan intraventricular conduction delay reflects extensive

yocardial damage rather than an electrical problem inolation (129). Although AV block that occurs during
ferior MI can be associated with a favorable long-term

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inical outcome, in-hospital survival is impaired irrespectivef temporary or permanent pacing in this situation34,135,138,139). Pacemakers generally should not be im-anted with inferior MI if the peri-infarctional AV block ispected to resolve or is not expected to negatively affectng-term prognosis (136). When symptomatic high-degree

third-degree heart block complicates inferior MI, evenhen the QRS is narrow, permanent pacing may be consid-ed if the block does not resolve. For the patient with recentI with a left ventricular ejection fraction (LVEF) less thanequal to 35% and an indication for permanent pacing,

nsideration may be given to use of an ICD, a CRT deviceat provides pacing but not defibrillation capability (CRT-P),a CRT device that incorporates both pacing and defibril-

tion capabilities (CRT-D) when improvement in LVEF ist anticipated.

ecommendations for Permanent Pacing After thecute Phase of Myocardial Infarction*

ASS I

Permanent ventricular pacing is indicated for persistentsecond-degree AV block in the His-Purkinje system with alter-nating bundle-branch block or third-degree AV block within orbelow the His-Purkinje system after ST-segment elevation MI.(Level of Evidence: B) (79,126–129,131)Permanent ventricular pacing is indicated for transient ad-vanced second- or third-degree infranodal AV block and asso-ciated bundle-branch block. If the site of block is uncertain, anelectrophysiological study may be necessary. (Level of Evi-dence: B) (126,127)Permanent ventricular pacing is indicated for persistent andsymptomatic second- or third-degree AV block. (Level of Evi-dence: C)

ASS IIb

Permanent ventricular pacing may be considered for persistentsecond- or third-degree AV block at the AV node level, even inthe absence of symptoms. (Level of Evidence: B) (58)

ASS III

Permanent ventricular pacing is not indicated for transient AVblock in the absence of intraventricular conduction defects.(Level of Evidence: B) (126)Permanent ventricular pacing is not indicated for transient AVblock in the presence of isolated left anterior fascicular block.(Level of Evidence: B) (128)Permanent ventricular pacing is not indicated for new bundle-branch block or fascicular block in the absence of AV block.(Level of Evidence: B) (66,126)Permanent ventricular pacing is not indicated for persistentasymptomatic first-degree AV block in the presence of bundle-branch or fascicular block. (Level of Evidence: B) (126)

.1.5. Hypersensitive Carotid Sinus Syndrome andeurocardiogenic Syncopehe hypersensitive carotid sinus syndrome is defined asncope or presyncope resulting from an extreme reflex

hese recommendations are consistent with the “ACC/AHA Guidelines for the
(eanagement of Patients With ST-Elevation Myocardial Infarction” (6).
sponse to carotid sinus stimulation. There are 2 componentsthe reflex:Cardioinhibitory, which results from increased parasym-thetic tone and is manifested by slowing of the sinus rate orolongation of the PR interval and advanced AV block,one or in combination.Vasodepressor, which is secondary to a reduction inmpathetic activity that results in loss of vascular tone andpotension. This effect is independent of heart rate changes.Before concluding that permanent pacing is clinically

dicated, the physician should determine the relative contri-tion of the 2 components of carotid sinus stimulation to thedividual patient’s symptom complex. Hyperactive responsecarotid sinus stimulation is defined as asystole due to either

nus arrest or AV block of more than 3 seconds, a substantialmptomatic decrease in systolic blood pressure, or both40). Pauses up to 3 seconds during carotid sinus massagee considered to be within normal limits. Such heart rate andmodynamic responses may occur in normal subjects andtients with coronary artery disease. The cause-and-effectlation between the hypersensitive carotid sinus and thetient’s symptoms must be drawn with great caution (141).

pontaneous syncope reproduced by carotid sinus stimulationould alert the physician to the presence of this syndrome.inimal pressure on the carotid sinus in elderly patients maysult in marked changes in heart rate and blood pressure yetay not be of clinical significance. Permanent pacing fortients with an excessive cardioinhibitory response to ca-tid stimulation is effective in relieving symptoms (142,143).ecause 10% to 20% of patients with this syndrome mayve an important vasodepressive component of their reflexsponse, it is desirable that this component be defined beforee concludes that all symptoms are related to asystole alone.

mong patients whose reflex response includes both car-oinhibitory and vasodepressive components, attention to thetter is essential for effective therapy in patients undergoingcing.Carotid sinus hypersensitivity should be considered in

derly patients who have had otherwise unexplained falls. Instudy, 175 elderly patients who had fallen without loss ofnsciousness and who had pauses of more than 3 secondsring carotid sinus massage (thus fulfilling the diagnosis ofrotid sinus hypersensitivity) were randomized to pacing ornpacing therapy. The paced group had a significantly lower

kelihood of subsequent falling episodes during follow-up44).Neurocardiogenic syncope and neurocardiogenic syn-omes refer to a variety of clinical scenarios in whichiggering of a neural reflex results in a usually self-limitedisode of systemic hypotension characterized by both bra-cardia and peripheral vasodilation (145,146). Neurocardio-nic syncope accounts for an estimated 10% to 40% ofncope episodes. Vasovagal syncope is a term used tonote one of the most common clinical scenarios within thetegory of neurocardiogenic syncopal syndromes. Patientsassically have a prodrome of nausea and diaphoresis (oftensent in the elderly), and there may be a positive familystory of the condition. Spells may be considered situational
.g., they may be triggered by pain, anxiety, stress, specific

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dily functions, or crowded conditions). Typically, no evi-nce of structural heart disease is present. Other causes ofncope such as LV outflow obstruction, bradyarrhythmias,d tachyarrhythmias should be excluded. Head-up tilt-tablesting may be diagnostic.The role of permanent pacing in refractory neurocardio-nic syncope associated with significant bradycardia orystole remains controversial. Approximately 25% of pa-

ents have a predominant vasodepressor reaction withoutgnificant bradycardia. Many patients will have a mixedsodepressive/cardioinhibitory cause of their symptoms. Its been estimated that approximately one third of patientsill have substantial bradycardia or asystole during head-uplt testing or during observed and recorded spontaneousisodes of syncope. Outcomes from clinical trials have noten consistent. Results from a randomized controlled trial47) in highly symptomatic patients with bradycardia dem-strated that permanent pacing increased the time to the firstncopal event. Another study demonstrated that DDD (aal-chamber pacemaker that senses/paces in the atrium/ntricle and is inhibited/triggered by intrinsic rhythm) pac-g with a sudden bradycardia response function was morefective than beta blockade in preventing recurrent syncopehighly symptomatic patients with vasovagal syncope and

lative bradycardia during tilt-table testing (148). In VPSasovagal Pacemaker Study) (149), the actuarial rate of

current syncope at 1 year was 18.5% for pacemaker patientsd 59.7% for control patients. However, in VPS-II (Vasova-l Pacemaker Study II) (150), a double-blind randomized

ial, pacing therapy did not reduce the risk of recurrentncopal events. In VPS-II, all patients received a permanentcemaker and were randomized to therapy versus no therapycontrast to VPS, in which patients were randomized to

cemaker implantation versus no pacemaker. On the basis ofPS-II and prevailing expert opinion (145), pacing therapy ist considered first-line therapy for most patients with neu-cardiogenic syncope. However, pacing therapy does have ale for some patients, specifically those with little or noodrome before their syncopal event, those with profoundadycardia or asystole during a documented event, and thosewhom other therapies have failed. Dual-chamber pacing,

refully prescribed on the basis of tilt-table test results withnsideration of alternative medical therapy, may be ef-ctive in reducing symptoms if the patient has a signifi-nt cardioinhibitory component to the cause of theirmptoms. Although spontaneous or provoked prolongeduses are a concern in this population, the prognosisithout pacing is excellent (151).The evaluation of patients with syncope of undeterminedigin should take into account clinical status and should noterlook other, more serious causes of syncope, such asntricular tachyarrhythmias.

ecommendations for Permanent Pacing in Hypersensitivearotid Sinus Syndrome and Neurocardiogenic Syncope

ASS I

Permanent pacing is indicated for recurrent syncope caused by
spontaneously occurring carotid sinus stimulation and carotid tr
sinus pressure that induces ventricular asystole of more than 3seconds. (Level of Evidence: C) (142,152)

ASS IIa

Permanent pacing is reasonable for syncope without clear,provocative events and with a hypersensitive cardioinhibitoryresponse of 3 seconds or longer. (Level of Evidence: C) (142)

ASS IIb

Permanent pacing may be considered for significantly symp-tomatic neurocardiogenic syncope associated with bradycar-dia documented spontaneously or at the time of tilt-tabletesting. (Level of Evidence: B) (147,148,150,153)

ASS III

Permanent pacing is not indicated for a hypersensitive car-dioinhibitory response to carotid sinus stimulation withoutsymptoms or with vague symptoms. (Level of Evidence: C)Permanent pacing is not indicated for situational vasovagalsyncope in which avoidance behavior is effective and preferred.(Level of Evidence: C)

.2. Pacing for Specific Conditionshe following sections on cardiac transplantation, neuromus-lar diseases, sleep apnea syndromes, and infiltrative andflammatory diseases are provided to recognize develop-ents in these specific areas and new information that hasen obtained since publication of prior guidelines. Some ofe information has been addressed in prior sections butrein is explored in more detail.

.2.1. Cardiac Transplantationhe incidence of bradyarrhythmias after cardiac transplanta-on varies from 8% to 23% (154–156). Most bradyarrhyth-ias are associated with SND and are more ominous afteransplantation, when the basal heart rate should be high.ignificant bradyarrhythmias and asystole have been associ-ed with reported cases of sudden death (157). Attempts toeat the bradycardia temporarily with measures such aseophylline(158) may minimize the need for pacing. Tocelerate rehabilitation, some transplant programs recom-end more liberal use of cardiac pacing for persistentstoperative bradycardia, although approximately 50% oftients show resolution of the bradyarrhythmia within 6 tomonths (159–161). The role of prophylactic pacemaker

plantation is unknown for patients who develop bradycar-a and syncope in the setting of rejection, which may besociated with localized inflammation of the conductionstem. Posttransplant patients who have irreversible SND orV block with previously stated Class I indications shouldve permanent pacemaker implantation, as the benefits ofe atrial rate contribution to cardiac output and to chrono-opic competence may optimize the patient’s functionalatus. When recurrent syncope develops late after transplan-tion, pacemaker implantation may be considered despitepeated negative evaluations, as sudden episodes of brady-rdia are often eventually documented and may be a sign of

ansplant vasculopathy.

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ecommendations for Pacing Afterardiac Transplantation

ASS I

Permanent pacing is indicated for persistent inappropriate orsymptomatic bradycardia not expected to resolve and for otherClass I indications for permanent pacing. (Level of Evidence: C)

ASS IIb

Permanent pacing may be considered when relative bradycar-dia is prolonged or recurrent, which limits rehabilitation ordischarge after postoperative recovery from cardiac transplan-tation. (Level of Evidence: C)Permanent pacing may be considered for syncope after cardiactransplantation even when bradyarrhythmia has not been doc-umented. (Level of Evidence: C)

.2.2. Neuromuscular Diseasesonduction system disease with progression to complete AVock is a well-recognized complication of several neuromus-lar disorders, including myotonic dystrophy and Emery-reifuss muscular dystrophy. Supraventricular and ventricu-r arrhythmias may also be observed. Implantation of armanent pacemaker has been found useful even in asymp-matic patients with an abnormal resting ECG or with HVterval prolongation during electrophysiological study (162).dications for pacing have been addressed in previousctions on AV block.

.2.3. Sleep Apnea Syndromevariety of heart rhythm disturbances may occur in obstruc-

ve sleep apnea. Most commonly, these include sinus brady-rdia or pauses during hypopneic episodes. Atrial tachyar-ythmias may also be observed, particularly during theousal phase that follows the offset of apnea. A smalltrospective trial of atrial overdrive pacing in the treatment

sleep apnea demonstrated a decrease “in episodes ofntral or obstructive sleep apnea without reducing the totaleep time” (163). Subsequent randomized clinical trials havet validated a role for atrial overdrive pacing in obstructive

eep apnea (164,165). Furthermore, nasal continuous posi-ve airway pressure therapy has been shown to be highlyfective for obstructive sleep apnea, whereas atrial overdrivecing has not (166,167). Whether cardiac pacing is indicatedong patients with obstructive sleep apnea and persistent

isodes of bradycardia despite nasal continuous positiverway pressure has not been established.Central sleep apnea and Cheyne-Stokes sleep-disorderedeathing frequently accompany systolic heart failure and aresociated with increased mortality (168). CRT has beenown to reduce central sleep apnea and increase sleepality in heart failure patients with ventricular conductionlay (169). This improvement in sleep-disordered breathingay be due to the beneficial effects of CRT on LV functiond central hemodynamics, which favorably modifies theuroendocrine reflex cascade in central sleep apnea.

.2.4. Cardiac Sarcoidosisardiac sarcoidosis usually affects individuals aged 20 to 40
ars and is associated with noncaseating granulomas with an pr
finity for involvement of the AV conduction system, whichsults in various degrees of AV conduction block. Myocar-al involvement occurs in 25% of patients with sarcoidosis,

many as 30% of whom develop complete heart block.wing to the possibility of disease progression, pacemakerplantation is recommended even if high-grade or complete

V conduction block reverses transiently (170–172).Cardiac sarcoidosis can also be a cause of life-threateningntricular arrhythmias with sustained monomorphic VT duemyocardial involvement (173–175). Sudden cardiac arrest

ay be the initial manifestation of the condition, and patientsay have few if any manifestations of dysfunction in organstems other than the heart (173,174). Although there are norge randomized trials or prospective registries of patientsith cardiac sarcoidosis, the available literature indicates thatrdiac sarcoidosis with heart block, ventricular arrhythmias,

LV dysfunction is associated with a poor prognosis.herapy with steroids or other immunosuppressant agentsay prevent progression of the cardiac involvement. Brady-rhythmias warrant pacemaker therapy, but they are notfective in preventing or treating life-threatening ventricularrhythmias. Sufficient clinical data are not available toratify risk of SCD among patients with cardiac sarcoidosis.ccordingly, clinicians must use the available literature alongith their own clinical experience and judgment in makinganagement decisions regarding ICD therapy. Considerationould be given to symptoms such as syncope, heart failureatus, LV function, and spontaneous or induced ventricularrhythmias at electrophysiological study to make individu-ized decisions regarding use of the ICD for primaryevention of SCD.

.3. Prevention and Termination ofrrhythmias by Pacingnder certain circumstances, an implanted pacemaker may beeful to treat or prevent recurrent ventricular and SVTs76–185). Re-entrant rhythms including atrial flutter, parox-mal re-entrant SVT, and VT may be terminated by a varietypacing techniques, including programmed stimulation andort bursts of rapid pacing (186,187). Although rarely usedcontemporary practice after tachycardia detection, these

titachyarrhythmia devices may automatically activate acing sequence or respond to an external instruction (e.g.,plication of a magnet).Prevention of arrhythmias by pacing has been demon-

rated in certain situations. In some patients with long-QTndrome, recurrent pause-dependent VT may be prevented

continuous pacing (188). A combination of pacing andta blockade has been reported to shorten the QT intervald help prevent SCD (189,190). ICD therapy in combinationith overdrive suppression pacing should be considered ingh-risk patients.Although this technique is rarely used today given theailability of catheter ablation and antiarrhythmic drugs,rial synchronous ventricular pacing may prevent recur-nces of reentrant SVT (191). Furthermore, although ven-icular ectopic activity may be suppressed by pacing in othernditions, serious or symptomatic arrhythmias are rarely
evented (192).

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Potential recipients of antitachyarrhythmia devices thatterrupt arrhythmias should undergo extensive testing beforeplantation to ensure that the devices safely and reliably

rminate the tachyarrhythmias without accelerating thechycardia or causing proarrhythmia. Patients for whom antitachycardia pacemaker has been prescribed have usuallyen unresponsive to antiarrhythmic drugs or were receivingents that could not control their cardiac arrhythmias. Whenrmanent antitachycardia pacemakers detect and interrupt

VT, all pacing should be done in the atrium because of thesk of ventricular pacing–induced proarrhythmia (176,193).ermanent antitachycardia pacing (ATP) as monotherapy forT is not appropriate given that ATP algorithms are availabletiered-therapy ICDs that have the capability for cardiover-

on and defibrillation in cases when ATP is ineffective oruses acceleration of the treated tachycardia.

ecommendations for Permanent Pacemakers Thatutomatically Detect and Pace to Terminate Tachycardias

ASS IIa

Permanent pacing is reasonable for symptomatic recurrentSVT that is reproducibly terminated by pacing when catheterablation and/or drugs fail to control the arrhythmia orproduce intolerable side effects. (Level of Evidence: C)(177–179,181,182)

ASS III

Permanent pacing is not indicated in the presence of anaccessory pathway that has the capacity for rapid anterogradeconduction. (Level of Evidence: C)

.3.1. Pacing to Prevent Atrial Arrhythmiasany patients with indications for pacemaker or ICD therapyve atrial tachyarrhythmias that are recognized before orter device implantation (194). Re-entrant atrial tachyar-ythmias are susceptible to termination with ATP. Addition-ly, some atrial tachyarrhythmias that are due to focaltomaticity may respond to overdrive suppression. Accord-gly, some dual-chamber pacemakers and ICDs incorporateites of atrial therapies that are automatically applied upontection of atrial tachyarrhythmias.The efficacy of atrial ATP is difficult to measure, primarilycause atrial tachyarrhythmias tend to initiate and terminateontaneously with a very high frequency. With device-assified efficacy criteria, approximately 30% to 60% ofrial tachyarrhythmias may be terminated with atrial ATP intients who receive pacemakers for symptomatic bradycar-a (195–197). Although this has been associated with aduction in atrial tachyarrhythmia burden over time inlected patients (195,196), the success of this approach hast been duplicated reliably in randomized clinical trials97). Similar efficacy has been demonstrated in ICD patients94,198,199) without compromising detection of VT, ven-icular fibrillation (VF), or ventricular proarrhythmia (200).

either situation, automatic atrial therapies should not betivated until the atrial lead is chronically stable, becauseslodgement into the ventricle could result in the induction
VT/VF. te
.3.2. Long-QT Syndromehe use of cardiac pacing with beta blockade for prevention

symptoms in patients with the congenital long-QT syn-ome is supported by observational studies (189,201,202).he primary benefit of pacemaker therapy may be in patientsith pause-dependent initiation of ventricular tachyarrhyth-ias (203) or those with sinus bradycardia or advanced AVock in association with the congenital long-QT syndrome04,205), which is most commonly associated with a sodiumannelopathy. Benson et al (206) discuss sinus bradycardiae to a (sodium) channelopathy. Although pacemaker im-antation may reduce the incidence of symptoms in thesetients, the long-term survival benefit remains to be deter-ined (189,201,204).

ecommendations for Pacing to Prevent Tachycardia

ASS I

Permanent pacing is indicated for sustained pause-dependentVT, with or without QT prolongation. (Level of Evidence: C)(188,189)

ASS IIa

Permanent pacing is reasonable for high-risk patients withcongenital long-QT syndrome. (Level of Evidence: C) (188,189)

ASS IIb

Permanent pacing may be considered for prevention of symp-tomatic, drug-refractory, recurrent AF in patients with coexist-ing SND. (Level of Evidence: B) (31,184,207)

ASS III

Permanent pacing is not indicated for frequent or complexventricular ectopic activity without sustained VT in the ab-sence of the long-QT syndrome. (Level of Evidence: C) (192)Permanent pacing is not indicated for torsade de pointes VTdue to reversible causes. (Level of Evidence: A) (190,203)

.3.3. Atrial Fibrillation (Dual-Site, Dual-Chamber,lternative Pacing Sites)some patients with bradycardia-dependent AF, atrial pac-

g may be effective in reducing the frequency of recurrences08). In MOST, 2,010 patients with SND were randomizedtween DDDR and VVIR pacing. After a mean follow-up ofmonths, there was a 21% lower risk of AF (p�0.008) in

e DDDR group than in the VVIR group (209). Other trialse under way to assess the efficacy of atrial overdrive pacinggorithms and algorithms that react to premature atrialmplexes in preventing AF, but data to date are sparse andconsistent (197,210). Dual-site right atrial pacing or alter-te single-site atrial pacing from unconventional sites (e.g.,rial septum or Bachmann’s bundle) may offer additionalnefits to single-site right atrial pacing from the appendagepatients with symptomatic drug-refractory AF and con-

mitant bradyarrhythmias; however, results from these stud-s are also contradictory and inconclusive (211,212). Addi-onally, analysis of the efficacy of pacing preventiongorithms and alternative pacing sites is limited by short-
rm follow-up (213). In patients with sick sinus syndrome

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ecommendation for Pacing to Prevent Atrial FibrillationASS III

Permanent pacing is not indicated for the prevention of AF inpatients without any other indication for pacemaker implanta-tion. (Level of Evidence: B) (215)

.4. Pacing for Hemodynamic Indicationslthough most commonly used to treat or prevent abnormalythms, pacing can alter the activation sequence in the pacedambers, influencing regional contractility and centralmodynamics. These changes are frequently insignificantinically but can be beneficial or harmful in some condi-ons. Pacing to decrease symptoms for patients withstructive hypertrophic cardiomyopathy (HCM) is dis-ssed separately in Section 2.4.2, “Obstructive Hypertro-ic Cardiomyopathy.”

.4.1. Cardiac Resynchronization TherapyPDATED)ee Online Data Supplement for additional data on the trialsat comprise the basis for the recommendations from thisction.)The present document proposes several changes incommendations for CRT, compared with the 2008 doc-

ent. The most significant changes are 1) limitation ofe Class I indication to patients with QRS duration �150s; 2) limitation of the Class I indication to patients with leftndle-branch block (LBBB) pattern; 3) expansion of Class Idication to New York Heart Association (NYHA) class IInd with LBBB with QRS duration �150 ms); and 4) thedition of a Class IIb recommendation for patients who have

VEF �30%, ischemic etiology of heart failure (HF), sinusythm, LBBB with a QRS duration �150 ms, and NYHAass I symptoms. These changes may have important impli-tions for patient selection in clinical practice, and thestification for these changes is discussed in the followingragraphs.Progression of LV systolic dysfunction to clinical HF is

equently accompanied by impaired electromechanical cou-ing, which may further diminish effective ventricular con-actility. The most common disruptions are prolonged atrio-ntricular conduction (first-degree atrioventricular block)d prolonged interventricular conduction, most commonly

BBB. Prolonged interventricular and intraventricular con-ction causes regional mechanical delay within the leftntricle that can result in reduced ventricular systolic func-

on, altered myocardial metabolism, functional mitral regur-tation, and adverse remodeling with ventricular dilatation58). Prolongation of the QRS duration occurs in approxi-ately one third of patients with advanced HF (559,560) ands been associated with ventricular electromechanical delaydyssynchrony”), as identified by multiple sophisticatedhocardiographic indices. QRS duration and dyssynchronyth have been identified as predictors of worsening HF,dden cardiac death, and total death (561).Modification of ventricular electromechanical delay with

ultisite ventricular pacing (commonly called “biventricular th

cing” or CRT) can improve ventricular systolic function,duce metabolic costs, ameliorate functional mitral regurgi-tion, and, in some patients, induce favorable remodelingith reduction of cardiac chamber dimensions (562,563,564).unctional improvement has been demonstrated for exercisepacity, with peak oxygen consumption in the range of 1 tomL/kg/min and a 50- to 70-meter increase in 6-minute

alking distance, as well as a 10-point or greater reduction ofF symptoms on the 105-point Minnesota Living with Heartailure scale (542,565,566).Meta-analyses of initial clinical experiences and largerbsequent trials of CRT confirmed an approximately 30%crease in hospitalizations and a mortality rate benefit of% to 36% (567). In the COMPANION (Comparison ofedical Therapy, Pacing, and Defibrillation in Heart Failure)

ial (NYHA class III/IV HF, QRS duration �120 ms, andVEF �35% on GDMT), GDMT was compared to CRTacing therapy without backup defibrillation (CRT-acemaker) and to CRT therapy with defibrillation backupRT-D) (543). Both CRT-Pacemaker and CRT-D reducede risk of the primary composite endpoint by approximately% as compared with GDMT alone. CRT-D reduced theortality rate by 36% compared with medical therapy, butere was insufficient evidence to conclude that CRT-acemaker was inferior to CRT-D. The CARE-HF (Cardiacesynchronization in Heart Failure) trial (544) limited sub-cts to a QRS duration �150 ms (89% of patients) or QRSration 120 to 150 ms with echocardiographic evidence ofssynchrony (11% of patients). It was the first study to showsignificant (36%) reduction in death rate for resynchroni-tion therapy unaccompanied by backup defibrillation com-red with GDMT (544).In the present document, we give a Class I recommenda-

on for CRT in patients with QRS duration �150 ms. Thefferential classification seen in this document related toRS duration is based on the results of multiple analyses ofRT benefit. The prevalence of mechanical dyssynchrony hasen documented in �40% of patients with dilated cardio-yopathy and QRS duration �120 ms, and is as high as 70%ong patients with QRS duration �150 ms and intraven-

icular mechanical delay, as identified by several echocar-ographic techniques (561,568). However, the aggregateinical experience has consistently demonstrated that agnificant clinical benefit from CRT is greatest amongtients with QRS duration �150 ms (569,570). In a meta-alysis of 5 trials involving 6501 patients, CRT significantlycreased the primary endpoint of death or hospitalization forF in patients with QRS duration �150 ms (HR: 0.58; 95%I: 0.50 to 0.68; p�0.00001) but not in patients with QRSration �150 ms (HR: 0.95; 95% CI: 0.83 to 1.10; p�0.51)69). In addition, subgroup analyses from several studiesve suggested that a QRS duration �150 ms is a risk factorr failure to respond to CRT therapy (570,571). The ob-rved differential benefit of CRT was seen across patients inYHA classes I through IV. It has not been possible toliably identify those with shorter QRS durations who maynefit. Patients with shorter QRS durations who otherwisealify for CRT are afforded Class II recommendations in
ese guidelines.
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An additional difference in the present document comparedith the 2008 DBT guideline (1d) is the limitation of thecommendation for Class I indication to patients with LBBBttern as compared to those with non-LBBB. For patientsith QRS duration �120 ms who do not have a completeBBB (non-LBBB patterns), evidence for benefit with CRT

less compelling than in the presence of LBBB72,573,574). The impact of the specific QRS morphology

clinical event reduction with CRT was evaluated in aeta-analysis of 4 clinical trials including 5,356 patients71a). In those with LBBB, CRT significantly reducedmposite adverse clinical events (RR: 0.64; 95% CI: 0.52 to77; p�0.00001). No benefit was observed for patients withn-LBBB conduction abnormalities (RR: 0.97; 95% CI:82 to 1.15; p�0.75). Specifically, there was no benefit intients with right bundle-branch block (RR: 0.91; 95% CI:69 to 1.20; p�0.49) or nonspecific intraventricular conduc-on delay (RR: 1.19; 95% CI: 0.87 to 1.63; p�0.28). Overall,e difference in effect of CRT between LBBB versusn-LBBB patients was highly statistically significant�0.0001) (571a). Nevertheless, other studies have shownat CRT is more likely to be effective in patients withvanced HF and non-LBBB morphologies if they have aarkedly prolonged QRS duration (547,557) (see RAFTesynchronization-Defibrillation for Ambulatory Heart Fail-e Trial] (547) discussion below). Furthermore, patients withRS prolongation due to frequent right ventricular apicalcing may benefit from CRT when other criteria for CRT areet (549,551,575). No large trial has yet demonstratedinical benefit among patients without QRS prolongation,en when they have been selected with echocardiographiceasures of dyssynchrony (576).The observed heterogeneity of response even among those

ho would appear to be excellent candidates for CRT alsoay result from factors such as suboptimal lead location ande location of conduction block from fibrosis in relation toe pacing site. Several recent studies have emphasized theportance of LV lead placement. For example, wider LV-

ght ventricular lead separation has been shown to providetter results (577). A subanalysis of MADIT-CRT (Multi-nter Automatic Defibrillator Implantation Trial with Car-ac Resynchronization Therapy) (546) showed that an apicalV lead position, as compared with a basal or midventricularsition, resulted in a significant increased risk for HF orath (578).Clinical trials of resynchronization included mainly pa-

ents in sinus rhythm. However, prospective experienceong patients with permanent atrial fibrillation and with

creased LV systolic function suggests that benefit maysult from biventricular pacing when the QRS duration is120 ms, although it may be most evident in patients inhom atrioventricular nodal ablation has been performed,ch that right ventricular pacing is obligate (550,552,579).

he benefit of CRT in patients with atrial fibrillation is moreonounced in those with depressed ejection fraction (551).

imilarly, patients receiving prophylactic ICDs often evolveogressively to dominant ventricular pacing, which mayflect both intrinsic chronotropic incompetence and aggres-
ve up-titration of beta-adrenergic-blocking agents. N
When device implantation or reimplantation is being con-dered for patients who require ventricular pacing, it isudent to recall the results of the DAVID (Dual Chamberd VVI Implantable Defibrillator) trial (580). In this trial,al-chamber rate-responsive pacing increased HF admis-

ons and mortality rate as compared to sinus rhythm. A cutoffapproximately 40% right ventricular pacing was seen as

leterious (581). Similarly, in a substudy from MADIT-IIulticenter Automatic Defibrillator Implantation Trial II),

tients who were right ventricular paced �50% of the timed a higher rate of new or worsened HF than those rightntricular paced �50% of the time (582).The major experience with resynchronization derives fromtients with NYHA class III symptoms of HF and LVEF35%. Patients with NYHA class IV symptoms of HF havecounted for only 10% of all patients in clinical trials ofsynchronization therapy. These patients were highly se-cted ambulatory outpatients who were taking oral medica-ons and had no history of recent hospitalization (583).lthough a benefit has occasionally been described in pa-ents with more severe acute decompensation that requiredief positive intravenous inotropic therapy to aid diuresis,RT is not generally used as a “rescue therapy” for suchtients. Patients with dependence on intravenous inotropicerapy, refractory fluid retention, or advanced chronic kid-y disease represent the highest-risk population for compli-tions of any procedure and for early death after hospitalscharge, and they are also unlikely to receive a meaningfulortality risk benefit from concomitant defibrillator therapy45,584).Patients with NYHA class IV HF symptoms who derivenctional benefit from resynchronization therapy may returna better functional status, in which prevention of suddenath becomes a relevant goal. Even among the selectedYHA class IV patients identified within the COMPANIONial (543), there was no difference in 2-year survival ratetween the CRT patients with and without backup defibril-tion, although more of the deaths in the CRT-Pacemakeroup were classified as sudden deaths (583).Perhaps the most significant changes in the present docu-

ent compared to the 2008 DBT Guideline 1d are thepansion of the Class I recommendation for CRT to includetients with LBBB, QRS duration �150 ms, and NYHAass II and the addition of a Class IIb recommendation fortients who have LVEF �30%, ischemic etiology of HF,

nus rhythm, LBBB with a QRS duration of �150 ms, andYHA class I symptoms. These recommendations are based

4 studies in which CRT was evaluated in patients withinimal or mild symptoms of HF in the setting of low LVEF.hese include MADIT-CRT, RAFT, REVERSE (Resynchro-zation Reverses Remodeling in Systolic Left Ventricularysfunction), and MIRACLE ICD II (Multicenter InSyncD Randomized Clinical Evaluation II), all of which arescussed in the following paragraphs (547,548,585), ran-mized patients with NYHA class I or II ischemic and

YHA class II nonischemic cardiomyopathy, LVEF �30%,d QRS duration �130 ms on GDMT to CRT-D or ICDone. Of note, only 15% of the total cohort of patients were
YHA class I. The primary endpoint, a composite of death or

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F event, was reduced by 34% by CRT-D (HR: 0.66), withmparable benefit for both ischemic and nonischemic etiol-y of HF. HF events were reduced by 41%, without

gnificant reduction in mortality rate. CRT-D therapy wasmonstrated to be of more benefit in women than in menR: 0.37 and 0.76, respectively) and in patients with QRSration �150 ms than in patients with QRS duration �150s (HR: 0.48 and 1.06, respectively) (546). Patients withBBB had a significant reduction in ventricular tachycardia,ntricular fibrillation, and death compared to non-LBBBtients, who derived no benefit (HR: 0.47 and 1.24, respec-

vely) (540).RAFT (547) reported the use of CRT-D in patients with

YHA class II or class III ischemic or nonischemic cardio-yopathy, LVEF �30%, and QRS duration �120 ms, asmpared to those treated with an ICD alone. The primarytcome of death or hospitalization for HF occurred in 33%patients receiving CRT-D and in 40% of patients receivingD only. RAFT not only showed a significant reduction inspitalization for HF (HR: 0.68; 95% CI: 0.56 to 0.83;0.001) but also was the first study to show a statistically

gnificant reduction in death (HR: 0.75; 95% CI: 0.62 to91; p�0.003) in mildly symptomatic patients with NYHAass II symptoms. However, CRT-D was associated with agher risk of adverse device- or implantation-related com-ications at 30 days after implantation (p�0.001) comparedith an ICD and no CRT. Patients with LBBB had a bettertcome than did non-LBBB patients, but the statisticalteraction between benefit and QRS morphology waseak in this trial (p�0.046). CRT-D therapy was effectivepatients with QRS duration �150 ms but of no benefitpatients with QRS duration �150 ms (HR for QRS

ration �150 ms: 0.59; 95% CI: 0.48 to 0.73; HR for QRSration �150 ms: 0.99; 95% CI: 0.77 to 1.27; p�0.002 forteraction). Thus, both MADIT-CRT and RAFT showednefit in NYHA class II patients treated with CRT-D andmonstrated that the benefit was primarily achieved intients with QRS duration �150 ms and LBBB (546,547).The REVERSE trial consisted of 610 patients. This studysessed CRT-D therapy in patients with NYHA class I or IIF symptoms on maximum medical therapy, LVEF �40%,d QRS duration �120 ms followed for 12 months andowed that 16% of patients receiving CRT and 21% withoutRT worsened (p�0.10). The time to first HF hospitalizationas delayed in patients receiving CRT therapy (HR: 0.47).he primary echocardiographic endpoint of ventricular re-odeling assessed by LV end-systolic volume index wasgnificantly improved (reduction in end-systolic volumedex) in patients treated with CRT therapy (p�0.0001).EVERSE did not report a mortality rate benefit of CRT-Derapy (548). The lack of reported mortality rate benefit mayrelated to the higher ejection fraction enrollment criterion

VEF �40%) and the relatively short-term follow-up (12onths) (548).MIRACLE ICD II included patients with NYHA class II

F on GDMT with LVEF �35% and QRS duration �130 msho were undergoing implantation of an otherwise indicatedD (585). In these patients, CRT did not alter exercise
pacity but did result in significant improvement in cardiac w
ructure and function and composite clinical response over 6onths.Analysis of the multiple clinical trials of CRT is compli-ted because trials encompass a range of LVEFs in theirtry criteria, as well as a range of measured outcomes. Forortality rate, the trials showing benefit in NYHA class IIId IV patients typically included those with LVEF �35%48,585). For patients with NYHA class II, trials showingortality rate benefit included those with LVEF �30%46,547). A mortality rate benefit with CRT has not beenown for patients who are NYHA class I (547). In terms ofmonstrating improvement in cardiac function (e.g., signif-ant reduction in LV size and improvement in ejectionaction), trials have included patients with LVEF �35% whoe NYHA class III and IV (585). Similarly, for patients withVEF �40%, trials demonstrating improvement in functionve included those who are NYHA class I and II (548). Thengruence of results from the totality of CRT trials withgard to remodeling and HF events provides evidencepporting a common threshold of 35% for benefit from CRTpatients with NYHA class II through IV HF symptoms.

lthough there is evidence for benefit in both CRT-D andRT-Pacemaker patients with NYHA class III and IV symp-ms, for NYHA class I and II HF, all of the trials tested onlyRT-D and not CRT-Pacemaker, and as such, recommenda-ons for these classes of patients can be made only forRT-D (546,547,548,585).Taken together, the evidence from the randomized trials of

RT-D in patients with reduced LVEF and NYHA class I orshows that CRT can provide functional improvement andcrease the risk of HF events and composite outcomes46,548,585,586). Still, CRT-D also has been shown tocrease the mortality rate for patients with NYHA class IIt not for those who have NYHA class I HF (546,547). As

result, the data support a Class I recommendation for CRTplantation in patients with LBBB and QRS duration �150

s and NYHA class II. Because of the lack of mortality ratenefit and smaller sample size, we believe CRT may bensidered for patients who have LVEF �30%, ischemiciology of HF, sinus rhythm, LBBB with a QRS duration150 ms, and NYHA class I symptoms on GDMT (Classb; LOE: B).For all patients, optimal outcomes with CRT require

fective placement of ventricular leads, ongoing HF manage-ent with neurohormonal antagonists and diuretic therapy,d in some cases, later optimization of device programming,pecially atrioventricular (A-V) and interventricular (V-V)tervals (578,587).Consistent with entry criteria for studies upon which these

commendations are based, CRT implantation should berformed only when the LVEF meets guideline criteria fortients with nonischemic cardiomyopathy who have re-ived �3 months of GDMT, or for patients with ischemicrdiomyopathy �40 days after myocardial infarction receiv-g GDMT when there was no intervening revascularization,

�3 months if revascularization was performed. It issumed that the final decision to recommend CRT will besed on an assessment of LVEF made after any appropriate
aiting period has concluded, during which GDMT has been

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plied. Finally, the pivotal trials demonstrating the efficacyCRT took place in centers that provided expertise in deviced HF therapy both at implantation and during long-termllow-up.Two other organizational guidelines by the Heart Failure

ociety of America (588) and the European Society ofardiology (589) have recently been published that addressdications for CRT. For the patient categories in commontween the Heart Failure Society of America document ande present focused update, there was a good deal of concor-nce. Although there are many areas of agreement, somefferences exist between the present guideline and theuropean Society of Cardiology document. One difference isat in the present guideline, CRT is recommended in NYHAass I patients who have LVEF �30%, have ischemic heartsease, are in sinus rhythm, and have a LBBB with a QRSration �150 ms (Class IIb; LOE: C) (546,547). There is no

milar recommendation in the European Society of Cardiol-y document. The European Society of Cardiology recom-endations include patients with QRS duration �120 ms. Weve not recommended CRT for any functional class orection fraction with QRS durations �120 ms. We also haveected to consider the presence of LBBB versus non-LBBBthe class of recommendations, on the basis of perceived

fferential benefit by functional class, QRS morphology, andRS duration.

ecommendations for Cardiacesynchronization Therapy

ee Appendix 6, “Indications for CRT Therapy–Algorithm.”ASS I

CRT is indicated for patients who have LVEF less than or equalto 35%, sinus rhythm, LBBB with a QRS duration greater thanor equal to 150 ms, and NYHA class II, (546,547) III, orambulatory IV (542–545); symptoms on GDMT. (Level ofEvidence: A for NYHA class III/IV; Level of Evidence: B forNYHA class II)

ASS IIa

CRT can be useful for patients who have LVEF less than orequal to 35%, sinus rhythm, LBBB with a QRS duration 120 to149 ms, and NYHA class II, III, or ambulatory IV symptoms onGDMT (542–544,546–548). (Level of Evidence: B)CRT can be useful for patients who have LVEF less than or equalto 35%, sinus rhythm, a non-LBBB pattern with a QRS durationgreater than or equal to 150 ms, and NYHA class III/ambulatory class IV symptoms on GDMT (542–544,547).(Level of Evidence: A)CRT can be useful in patients with atrial fibrillation and LVEFless than or equal to 35% on GDMT if a) the patient requiresventricular pacing or otherwise meets CRT criteria and b) AV nodalablation or pharmacologic rate control will allow near 100% ventric-ular pacing with CRT (549–553,575). (Level of Evidence: B)CRT can be useful for patients on GDMT who have LVEF lessthan or equal to 35% and are undergoing new or replacementdevice placement with anticipated requirement for significant(>40%) ventricular pacing (551,554,555,556). (Level of Evi-
dence: C) bl
ASS IIb

CRT may be considered for patients who have LVEF less than orequal to 30%, ischemic etiology of heart failure, sinus rhythm,LBBB with a QRS duration of greater than or equal to 150 ms,and NYHA class I symptoms on GDMT (546,547). (Level ofEvidence: C)CRT may be considered for patients who have LVEF less than orequal to 35%, sinus rhythm, a non-LBBB pattern with QRSduration 120 to 149 ms, and NYHA class III/ambulatory classIV on GDMT (547,557). (Level of Evidence: B)CRT may be considered for patients who have LVEF less than orequal to 35%, sinus rhythm, a non-LBBB pattern with a QRSduration greater than or equal to 150 ms, and NYHA class IIsymptoms on GDMT (546,547). (Level of Evidence: B)

ASS III: NO BENEFIT

CRT is not recommended for patients with NYHA class I or IIsymptoms and non-LBBB pattern with QRS duration less than150 ms (546,547,557). (Level of Evidence: B)CRT is not indicated for patients whose comorbidities and/orfrailty limit survival with good functional capacity to less than1 year (545). (Level of Evidence: C)

.4.2. Obstructive Hypertrophic Cardiomyopathyarly nonrandomized studies demonstrated a fall in the LVtflow gradient with dual-chamber pacing and a short AVlay and symptomatic improvement in some patients withstructive HCM (232–235). One long-term study (236) in 8tients supported the long-term benefit of dual-chambercing in this group of patients. The outflow gradient wasduced even after cessation of pacing, which suggests thatme ventricular remodeling had occurred as a consequencepacing. Two randomized trials (235,237) demonstrated

bjective improvement in approximately 50% of studyrticipants, but there was no correlation with gradientduction, and a significant placebo effect was present. Aird randomized, double-blinded trial (238) failed to demon-rate any overall improvement in QOL with pacing, althoughere was a suggestion that elderly patients (more than 65ars of age) may derive more benefit from pacing.In a small group of patients with symptomatic, hyper-

nsive cardiac hypertrophy with cavity obliteration, VDDcing with premature excitation statistically improvedercise capacity, cardiac reserve, and clinical symptoms39). Dual-chamber pacing may improve symptoms andV outflow gradient in pediatric patients. However, rapidrial rates, rapid AV conduction, and congenital mitrallve abnormalities may preclude effective pacing in sometients (240).There are currently no data available to support thentention that pacing alters the clinical course of the diseaseimproves survival or long-term QOL in HCM. Therefore,

utine implantation of dual-chamber pacemakers should notadvocated in all patients with symptomatic obstructive

CM. Patients who may benefit the most are those withgnificant gradients (more than 30 mm Hg at rest or morean 50 mm Hg provoked). (235,241–243). Complete heart
ock can develop after transcoronary alcohol ablation of

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ptal hypertrophy in patients with HCM and should beeated with permanent pacing (244).For the patient with obstructive HCM who is at high riskr sudden death and who has an indication for pacemakerplantation, consideration should be given to completion of

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ecommendations for Pacing in Patients Withypertrophic Cardiomyopathy

ASS I

Permanent pacing is indicated for SND or AV block in patientswith HCM as described previously (see Section 2.1.1, “SinusNode Dysfunction,” and Section 2.1.2, “Acquired Atrioventric-ular Block in Adults”). (Level of Evidence: C)

ASS IIa

Permanent pacing may be considered in medically refractory symp-tomatic patients with HCM and significant resting or provoked LVoutflow tract obstruction. (Level of Evidence: A) As for Class Iindications, when risk factors for SCD are present, consider a DDDICD (see Section 3, “Indications for Implantable Cardioverter-Defibrillator Therapy”) (233,235,237,238,246,247).

ASS III

Permanent pacemaker implantation is not indicated for pa-tients who are asymptomatic or whose symptoms are medi-cally controlled. (Level of Evidence: C)Permanent pacemaker implantation is not indicated for symp-tomatic patients without evidence of LV outflow tract obstruc-tion. (Level of Evidence: C)

.5. Pacing in Children, Adolescents, andatients With Congenital Heart Diseasehe most common indications for permanent pacemakerplantation in children, adolescents, and patients with con-nital heart disease may be classified as 1) symptomatic

nus bradycardia, 2) the bradycardia-tachycardia syndromes,d 3) advanced second- or third-degree AV block, eitherngenital or postsurgical. Although the general indicationsr pacemaker implantation in children and adolescents (de-

ned as less than 19 years of age) (248) are similar to thoseadults, there are several important considerations in youngtients. First, an increasing number of young patients areng-term survivors of complex surgical procedures for con-nital heart defects that result in palliation rather thanrrection of circulatory physiology. The residua of impairedntricular function and abnormal physiology may result inmptoms due to sinus bradycardia or loss of AV synchronyheart rates that do not produce symptoms in individuals

ith normal cardiovascular physiology (249,250). Hence, thedications for pacemaker implantation in these patients needbe based on the correlation of symptoms with relative

adycardia rather than absolute heart rate criteria. Second,
e clinical significance of bradycardia is age dependent; th
hereas a heart rate of 45 bpm may be a normal finding in anolescent, the same rate in a newborn or infant indicatesofound bradycardia. Third, significant technical challengesay complicate device and transvenous lead implantation inry small patients or those with abnormalities of venous ortracardiac anatomy. Epicardial pacemaker lead implanta-on represents an alternative technique for these patients;wever, the risks associated with sternotomy or thoracotomyd the somewhat higher incidence of lead failure must bensidered when epicardial pacing systems are required51). Fourth, because there are no randomized clinical trials

cardiac pacing in pediatric or congenital heart diseasetients, the level of evidence for most recommendations isnsensus based (Level of Evidence: C). Diagnoses thatquire pacing in both children and adults, such as long-QTndrome or neuromuscular diseases, are discussed in spe-fic sections on these topics in this document.Bradycardia and associated symptoms in children are often

ansient (e.g., sinus arrest or paroxysmal AV block) andfficult to document (252). Although SND (sick sinus syn-ome) is recognized in pediatric patients and may besociated with specific genetic channelopathies (206), it ist itself an indication for pacemaker implantation. In theung patient with sinus bradycardia, the primary criterionr pacemaker implantation is the concurrent observation of amptom (e.g., syncope) with bradycardia (e.g., heart ratess than 40 bpm or asystole more than 3 seconds)3,86,253). In general, correlation of symptoms with brady-rdia is determined by ambulatory ECG or an implantableop recorder (254). Symptomatic bradycardia is an indica-on for pacemaker implantation provided that other causesve been excluded. Alternative causes to be consideredclude apnea, seizures, medication effects, and neurocardio-nic mechanisms (255,256). In carefully selected cases,rdiac pacing has been effective in the prevention of recur-nt seizures and syncope in infants with recurrent pallideath-holding spells associated with profound bradycardia orystole (257).A variant of the bradycardia-tachycardia syndrome, sinusadycardia that alternates with intra-atrial re-entrant tachy-rdia, is a significant problem after surgery for congenitalart disease. Substantial morbidity and mortality have beenserved in patients with recurrent or chronic intra-atrial-entrant tachycardia, with the loss of sinus rhythm andependent risk factor for the subsequent development ofis arrhythmia (258,259). Thus, both long-term atrial pacingphysiological rates and atrial ATP have been reported astential treatments for sinus bradycardia and the preventiontermination of recurrent episodes of intra-atrial re-entrant

chycardia (260,261). The results of either mode of pacingr this arrhythmia have been equivocal and remain a topic ofnsiderable controversy (262,263). In other patients, phar-acological therapy (e.g., sotalol or amiodarone) may befective in the control of intra-atrial re-entrant tachycardiat also result in symptomatic bradycardia (264). In thesetients, radiofrequency catheter ablation of the intra-atrial-entrant tachycardia circuit should be considered as anternative to combined pharmacological and pacemaker
erapies (265). Surgical resection of atrial tissue with con-

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mitant atrial pacing has also been advocated for congenitalart disease patients with intra-atrial re-entrant tachycardiafractory to other therapies (266).The indications for permanent pacing in patients withngenital complete AV block continue to evolve on the basisimproved definition of the natural history of the disease

d advances in pacemaker technology and diagnostic meth-s. Pacemaker implantation is a Class I indication in themptomatic individual with congenital complete AV blockthe infant with a resting heart rate less than 55 bpm, or less

an 70 bpm when associated with structural heart disease67,268). In the asymptomatic child or adolescent withngenital complete AV block, several criteria (average heartte, pauses in the intrinsic rate, associated structural heartsease, QT interval, and exercise tolerance) must be consid-ed (208,269). Several studies have demonstrated that pace-aker implantation is associated with both improved long-rm survival and prevention of syncopal episodes inymptomatic patients with congenital complete AV block70,271). However, periodic evaluation of ventricular func-

on is required in patients with congenital AV block aftercemaker implantation, because ventricular dysfunctionay occur as a consequence of myocardial autoimmunesease at a young age or pacemaker-associated dyssyn-rony years or decades after pacemaker implantation72,273). The actual incidence of ventricular dysfunctione to pacemaker-related chronic ventricular dyssynchronymains undefined.A very poor prognosis has been established for congenitalart disease patients with permanent postsurgical AV blockho do not receive permanent pacemakers (209). Therefore,vanced second- or third-degree AV block that persists forleast 7 days and that is not expected to resolve after cardiacrgery is considered a Class I indication for pacemakerplantation (274). Conversely, patients in whom AV con-ction returns to normal generally have a favorable progno-

s (275). Recent reports have emphasized that there is a smallt definite risk of late-onset complete AV block years orcades after surgery for congenital heart disease in patientsith transient postoperative AV block (276,277). Limitedta suggest that residual bifascicular conduction block andogressive PR prolongation may predict late-onset AV block78). Because of the possibility of intermittent complete AVock, unexplained syncope is a Class IIa indication forcing in individuals with a history of temporary postopera-

ve complete AV block and residual bifascicular conductionock after a careful evaluation for both cardiac and noncar-ac causes.Additional details that need to be considered in pacemakerplantation in young patients include risk of paradoxicalbolism due to thrombus formation on an endocardial lead

stem in the presence of residual intracardiac defects and thefelong need for permanent cardiac pacing (279). Decisionsout pacemaker implantation must also take into account theplantation technique (transvenous versus epicardial), witheservation of vascular access at a young age a primary
jective (280).
ecommendations for Permanent Pacing in Children,dolescents, and Patients With Congenital Heart Disease

ASS I

Permanent pacemaker implantation is indicated for advancedsecond- or third-degree AV block associated with symptomaticbradycardia, ventricular dysfunction, or low cardiac output.(Level of Evidence: C)Permanent pacemaker implantation is indicated for SND withcorrelation of symptoms during age-inappropriate bradycardia.The definition of bradycardia varies with the patient’s age andexpected heart rate. (Level of Evidence: B) (53,86,253,257)Permanent pacemaker implantation is indicated for postoper-ative advanced second- or third-degree AV block that is notexpected to resolve or that persists at least 7 days aftercardiac surgery. (Level of Evidence: B) (74,209)Permanent pacemaker implantation is indicated for congenitalthird-degree AV block with a wide QRS escape rhythm, complexventricular ectopy, or ventricular dysfunction. (Level of Evi-dence: B) (271–273)Permanent pacemaker implantation is indicated for congenitalthird-degree AV block in the infant with a ventricular rate lessthan 55 bpm or with congenital heart disease and a ventricularrate less than 70 bpm. (Level of Evidence: C) (267,268)

ASS IIa

Permanent pacemaker implantation is reasonable for patientswith congenital heart disease and sinus bradycardia for theprevention of recurrent episodes of intra-atrial reentrant tachy-cardia; SND may be intrinsic or secondary to antiarrhythmictreatment. (Level of Evidence: C) (260,261,264)Permanent pacemaker implantation is reasonable for congen-ital third-degree AV block beyond the first year of life with anaverage heart rate less than 50 bpm, abrupt pauses in ventric-ular rate that are 2 or 3 times the basic cycle length, orassociated with symptoms due to chronotropic incompetence.(Level of Evidence: B) (208,270)Permanent pacemaker implantation is reasonable for sinusbradycardia with complex congenital heart disease with aresting heart rate less than 40 bpm or pauses in ventricularrate longer than 3 seconds. (Level of Evidence: C)Permanent pacemaker implantation is reasonable for patientswith congenital heart disease and impaired hemodynamics dueto sinus bradycardia or loss of AV synchrony. (Level of Evi-dence: C) (250)Permanent pacemaker implantation is reasonable for unexplainedsyncope in the patient with prior congenital heart surgery compli-cated by transient complete heart block with residual fascicularblock after a careful evaluation to exclude other causes ofsyncope. (Level of Evidence: B) (273,276–278)

ASS IIb

Permanent pacemaker implantation may be considered fortransient postoperative third-degree AV block that reverts tosinus rhythm with residual bifascicular block. (Level of Evi-dence: C) (275)Permanent pacemaker implantation may be considered forcongenital third-degree AV block in asymptomatic children or
adolescents with an acceptable rate, a narrow QRS complex,

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and normal ventricular function. (Level of Evidence: B)(270,271)Permanent pacemaker implantation may be considered forasymptomatic sinus bradycardia after biventricular repair ofcongenital heart disease with a resting heart rate less than 40bpm or pauses in ventricular rate longer than 3 seconds. (Levelof Evidence: C)

ASS III

Permanent pacemaker implantation is not indicated for tran-sient postoperative AV block with return of normal AV conductionin the otherwise asymptomatic patient. (Level of Evidence: B)(274,275)Permanent pacemaker implantation is not indicated for asymp-tomatic bifascicular block with or without first-degree AV blockafter surgery for congenital heart disease in the absence ofprior transient complete AV block. (Level of Evidence: C)Permanent pacemaker implantation is not indicated for asymp-tomatic type I second-degree AV block. (Level of Evidence: C)Permanent pacemaker implantation is not indicated for asymp-tomatic sinus bradycardia with the longest relative risk intervalless than 3 seconds and a minimum heart rate more than 40bpm. (Level of Evidence: C)

.6. Selection of Pacemaker Devicence the decision has been made to implant a pacemaker ingiven patient, the clinician must decide among a largember of available pacemaker generators and leads. Gener-

or choices include single- versus dual-chamber versusventricular devices, unipolar versus bipolar pacing/sensingnfiguration, presence and type of sensor for rate response,vanced features such as automatic capture verification,rial therapies, size, and battery capacity. Lead choicesclude diameter, polarity, type of insulation material, andxation mechanism (active versus passive). Other factors that

ble 2. Choice of Pacemaker Generator in Selected Indications

Pacemaker Generator Sinus Node Dysfunction

ngle-chamber atrialcemaker

No suspected abnormality of atrioventricularconduction and not at increased risk for futureatrioventricular block

Maintenance of atrioventricular synchronyduring pacing desired

ngle-chamber ventricularcemaker

Maintenance of atrioventricular synchronyduring pacing not necessary

Rate response available if desired

al-chamber pacemaker Atrioventricular synchrony during pacing desired

Suspected abnormality of atrioventricularconduction or increased risk for futureatrioventricular block

Rate response available if desired

ngle-lead, atrial-sensingntricular pacemaker

Not appropriate

portantly influence the choice of pacemaker system com- an

nents include the capabilities of the pacemaker program-er, local availability of technical support, and remoteonitoring capabilities.Even after selecting and implanting the pacing system, theysician has a number of options for programming thevice. In modern single-chamber pacemakers, programma-e features include pacing mode, lower rate, pulse width andplitude, sensitivity, and refractory period. Dual-chamber

cemakers have the same programmable features, as well asaximum tracking rate, AV delay, mode-switching algo-thms for atrial arrhythmias, and others. Rate-responsivecemakers require programmable features to regulate thelation between sensor output and pacing rate and to limite maximum sensor-driven pacing rate. Biventricular pace-akers require the LV pacing output to be programmed, andten the delay between LV and RV pacing must also beogrammed. With the advent of more sophisticated pace-aker generators, optimal programming of pacemakers hascome increasingly complex and device-specific and re-ires specialized knowledge on the part of the physician.Many of these considerations are beyond the scope of thiscument. Later discussion focuses primarily on the choicegarding the pacemaker prescription that has the greatest impactprocedural time and complexity, follow-up, patient outcome,

d cost: the choice among single-chamber ventricular pacing,ngle-chamber atrial pacing, and dual-chamber pacing.Table 2 summarizes the appropriateness of different pace-akers for the most commonly encountered indications forcing. Figure 1 is a decision tree for selecting a pacingstem for patients with AV block. Figure 2 is a decision treer selecting a pacing system for patients with SND.An important challenge for the physician in selecting acemaker system for a given patient is to anticipate progres-

on of abnormalities of that patient’s cardiac automaticity

cing

Atrioventricular BlockNeurally Mediated Syncope orCarotid Sinus Hypersensitivity

ropriate Not appropriate

atrial fibrillation or other atrialhythmia or maintenance oftricular synchrony during pacing notry

Chronic atrial fibrillation or otheratrial tachyarrhythmia

ponse available if desired Rate response available ifdesired

ponse available if desired Sinus mechanism present

tricular synchrony during pacing desired Rate response available ifdesired

cing desired

ponse available if desired

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commodate these developments. Thus, it is reasonable tolect a pacemaker with more extensive capabilities thaneded at the time of implantation but that may prove usefulthe future. Some patients with SND and paroxysmal AF,

r example, may develop AV block in the future (as a resultnatural progression of disease, drug therapy, or catheter

lation) and may ultimately benefit from a dual-chambercemaker with mode-switching capability.Similarly, when pacemaker implantation is indicated, con-

deration should be given to implantation of a more capablevice (CRT, CRT-P, or CRT-D) if it is thought likely thate patient will qualify for the latter within a short timeriod. For example, a patient who requires a pacemaker forart block that occurs in the setting of MI who also has antremely low LVEF may be best served by initial implan-tion of an ICD rather than a pacemaker. In such cases, thevantage of avoiding a second upgrade procedure should belanced against the uncertainty regarding the ultimate needr the more capable device.

.6.1. Major Trials Comparing Atrial orual-Chamber Pacing With Ventricular Pacingver the past decade, the principal debate with respect tooice of pacemaker systems has concerned the relativeerits of dual-chamber pacing, single-chamber ventricularcing, and single-chamber atrial pacing. The physiological

gure 1. Selection of pacemaker systems for patients with atriovedicate type of pacemaker. AV indicates atrioventricular.

tionale for atrial and dual-chamber pacing is preservation of th

V synchrony; therefore, trials comparing these modes haveten combined patients with atrial or dual-chamber pace-akers in a single treatment arm. There have been 5 majorndomized trials comparing atrial or dual-chamber pacingith ventricular pacing; they are summarized in Table 3. Ofe 5 studies, 2 were limited to patients paced for SND, 1 wasmited to patients paced for AV block, and 2 includedtients paced for either indication. Only the Danish study81) included a true atrial pacing arm; among patients in theAI/DDD arm in CTOPP (Canadian Trial of Physiologicacing), only 5.2% had an atrial pacemaker (282). A significant

itation of all of these studies is the percentage of patients (up37.6%) who crossed over from 1 treatment arm to another or

herwise dropped out of their assigned pacing mode.An important consideration in the assessment of trials thatmpare pacing modes is the percent of pacing among the

udy patients. For example, a patient who is paced only forry infrequent sinus pauses or infrequent AV block willobably have a similar outcome with ventricular pacing asith dual-chamber pacing, regardless of any differentialfects between the 2 pacing configurations. With the excep-on of the MOST study (31) and limited data in theK-PACE trial (United Kingdom Pacing and Cardiovascularvents) (283), the trials included in Table 3 do not includeformation about the percent of atrial or ventricular pacing in

lar block. Decisions are illustrated by diamonds. Shaded boxes
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.6.2. Quality of Life and Functional Statusnd Pointsumerous studies have shown significant improvement inported QOL and functional status after pacemaker implan-tion, (22,23,285,286) but there is also a well-documentedacebo effect after device implantation (222). This section

gure 2. Selection of pacemaker systems for patients with sinusxes indicate type of pacemaker. AV indicates atrioventricular.

ble 3. Randomized Trials Comparing Atrium-Based Pacing Wit

Characteristics Danish Study (281) PASE (23)

cing indication SND SND and AVB

. of patients randomized 225 407

ean follow-up (years) 5.5 1.5

cing modes AAI vs VVI DDDR* vs VVIR*

rium-based pacing superiorith respect to:

Quality of life or functionalstatus

NA ● SND patients: y● AVB patients: n

Heart failure Yes No

Atrial fibrillation Yes No

Stroke orthromboembolism

Yes No

Mortality Yes No

Cross-over or pacingdropout

● VVI to AAI/DDD:4%

● AAI to DDD: 5%● AAI to VVI: 10%

VVIR* to DDDR*: 2

R* added to pacing mode designation indicates rate-responsive pacemakte-responsive pacemakers implanted in some patients.AAI indicates atrial demand; AVB, atrioventricular block; CTOPP, Canadian Tr
cemaker Selection in the Elderly; SND, sinus node dysfunction; UK-PACE, United Kin
ill focus on differences between pacing modes with respectthese outcomes.In the subset of patients in the PASE (Pacemaker Selectionthe Elderly) study who received implants for SND,

al-chamber pacing was associated with greater improve-ent than was ventricular pacing with regard to a minority of

ysfunction. Decisions are illustrated by diamonds. Shaded

ricular Pacing

CTOPP (282,284,285)MOST

(22,31,48,49,286,287) UK-PACE (283)

SND and AVB SND AVB

2,568 2,010 2,021

6.4 2.8 3

DDD/AAI vs VVI(R) DDDR vs VVIR* DDD(R) vs VVI(R)

No Yes NA

No Marginal No

Yes Yes No

No No No

No No No

● VVI(R) dropout: 7%● DDD/AAI dropout:

25%

VVIR* to DDDR*:37.6%

● VVI(R) to DDD(R):3.1%

● DDD(R) dropout:8.3%

lanted in all patients. (R) added to pacing mode designation indicates

ysiologic Pacing; DDD, fully automatic; MOST, Mode Selection Trial; PASE,

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OL and functional status measures, but there were no suchfferences among patients paced for AV block (23). In theOST patients, all of whom received implants for SND,al-chamber–paced patients had superior outcomes in somet not all QOL and functional status measures (22,286).

TOPP, which included patients who received implants forth SND and AV block, failed to detect any differencetween pacing modes with respect to QOL or functional

atus in a subset of 269 patients who underwent thisaluation; a breakdown by pacing indication was not re-rted (284).Older cross-over studies of dual-chamber versus ventricu-

r pacing, which allowed for intrapatient comparisons be-een the 2 modes, indicate improved functional status andtient preference for dual-chamber pacing. For instance,

ulke et al (288) studied 22 patients who received dual-amber rate-responsive pacemakers for high-grade AVock and found improved exercise time, functional status,d symptoms with DDDR compared with VVIR pacing, asell as vastly greater patient preference for DDDR pacing.

.6.3. Heart Failure End PointsDanish study showed an improvement in heart failure

atus among atrially paced patients compared with ventricu-rly paced patients, as measured by NYHA functional classd diuretic use (281). MOST showed a marginal improve-ent in a similar heart failure score with dual-chamber versusntricular pacing, as well as a weak association betweenal-chamber pacing and fewer heart failure hospitalizations2). None of the other studies listed in Table 3 detected afference between pacing modes with respect to new-onsetart failure, worsening of heart failure, or heart failurespitalization. A meta-analysis of the 5 studies listed in

able 3 did not show a significant difference between atriallyced- or dual-chamber–paced patients compared with ven-

icularly paced patients with respect to heart failure hospi-lization (289).

.6.4. Atrial Fibrillation End Pointshe Danish study, MOST, and CTOPP showed significantlyss AF among the atrially paced or dual-chamber–pacedtients than the ventricularly paced patients (22,281,282). InOST, the divergence in AF incidence became apparent at 6onths, whereas in CTOPP, the divergence was apparently at 2 years. PASE, a much smaller study, did not detecty difference in AF between its 2 groups (23). The UK-

ACE trial did not demonstrate a significant difference in AFtween its 2 treatment arms; however, a trend toward lessF with dual-chamber pacing began to appear at the end ofe scheduled 3-year follow-up period (28). The meta-alysis of the 5 studies listed in Table 3 showed a significantcrease in AF with atrial or dual-chamber pacing comparedith ventricular pacing, with a hazard ratio (HR) of 0.8089).

.6.5. Stroke or Thromboembolism End Pointsf the 5 studies listed in Table 3, only the Danish studytected a difference between pacing modes with respect to

roke or thromboembolism (281). However, the meta-
alysis of the 5 studies in Table 3 showed a decrease of th
rderline statistical significance in stroke with atrial oral-chamber pacing compared with ventricular pacing, withHR of 0.81 (289).

.6.6. Mortality End Pointshe Danish study showed significant improvement in botherall mortality and cardiovascular mortality among the

rially paced patients compared with the ventricularly pacedtients (281). None of the other studies showed a significantfference between pacing modes in either overall or cardio-scular mortality. The meta-analysis of the 5 studies in

able 3 did not show a significant difference between atriallyced or dual-chamber–paced patients compared with ventricu-rly paced patients with respect to overall mortality (289).Taken together, the evidence from the 5 studies most

rongly supports the conclusion that dual-chamber or atrialcing reduces the incidence of AF compared with ventric-ar pacing in patients paced for either SND or AV block.here may also be a benefit of dual-chamber or atrial pacingith respect to stroke. The evidence also supports a modestprovement in QOL and functional status with dual-amber pacing compared with ventricular pacing in patientsith SND. The preponderance of evidence from these trialsgarding heart failure and mortality argues against anyvantage of atrial or dual-chamber pacing for these 2 endints.

.6.7. Importance of Minimizing Unnecessaryentricular Pacingthe past 5 years, there has been increasing recognition of

e deleterious clinical effects of RVA pacing, both in patientsith pacemakers (48,49,215) and in those with ICDs0,51,290). Among the patients in MOST with a normaltive QRS duration, the percent of ventricular pacing wasrrelated with heart failure hospitalization and new onset ofF (48). It has been speculated that the more frequentntricular pacing in patients randomized to DDDR pacing0%) compared with patients randomized to VVIR pacing8%) may have negated whatever positive effects may havecrued from the AV synchrony afforded by dual-chambercing in this study. A possible explanation for the strikingnefits of AAI pacing found in the Danish study (281)scribed above is the obvious absence of ventricular pacingpatients with single-chamber atrial pacemakers (281).In a subsequent Danish study, patients with SND were

ndomized between AAIR pacing, DDDR pacing with ang AV delay (300 milliseconds), and DDDR pacing with aort AV delay (less than or equal to 150 milliseconds) (45).

he prevalence of ventricular pacing was 17% in the DDDR–ng-AV-delay patients and 90% in the DDDR–short-AV-lay patients. At 2.9 years of follow-up, the incidence of AFas 7.4% in the AAIR group, 17.5% in the DDDR–long-AV-lay group, and 23.3% in the DDDR–short-AV-delay group.

here were also increases in left atrial and LV dimensionsen in both DDDR groups but not the AAIR group. Thisudy supports the superiority of atrial over dual-chambercing and indicates that there may be deleterious effects

om even the modest amount of ventricular pacing thatpically occurs with maximally programmed AV delays in
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Patients included in studies showing deleterious effects ofV pacing were either specified as having their RV leadsitioned at the RV apex (40,43,280) or can be presumed inost cases to have had the lead positioned there based onevailing practices of pacemaker and defibrillator implanta-

on (45,46,277). Therefore, conclusions about deleteriousfects of RV pacing at this time should be limited to patientsith RVA pacing. Studies are currently under way thatmpare the effects of pacing at alternative RV sites (septum,tflow tract) with RVA pacing.Despite the appeal of atrium-only pacing, there remainsncern about implanting single-chamber atrial pacemakerspatients with SND because of the risk of subsequent AV

ock. Also, in the subsequent Danish study comparing atrialith dual-chamber pacing, the incidence of progression tomptomatic AV block, including syncope, was 1.9% perar, even with rigorous screening for risk of AV block at the

me of implantation (45). Programming a dual-chambervice to the conventional DDD mode with a maximallyogrammable AV delay or with AV search hysteresis doest eliminate frequent ventricular pacing in a significant

action of patients (291,292). Accordingly, several pacinggorithms that avoid ventricular pacing except during peri-s of high-grade AV block have been introduced recently93). These new modes dramatically decrease the prevalence

ventricular pacing in both pacemaker and defibrillatortients (294–296). A recent trial showed the frequency of

V pacing was 9% with one of these new algorithmsmpared with 99% with conventional dual-chamber pacing,d this decrease in RV pacing was associated with a 40%lative reduction in the incidence of persistent AF (296).dditional trials are under way to assess the clinical benefitsthese new pacing modes (297).

.6.8. Role of Biventricular Pacemakerss discussed in Section 2.4.1, “Cardiac Resynchronizationherapy,” multiple controlled trials have shown biventricularcing to improve both functional capacity and QOL andcrease hospitalizations and mortality for selected patientsith Class III to IV symptoms of heart failure. Althoughtients with a conventional indication for pacemaker im-antation were excluded from these trials, it is reasonable tosume that patients who otherwise meet their inclusioniteria but have QRS prolongation due to ventricular pacingight also benefit from biventricular pacing.Regardless of the duration of the native QRS complex,tients with LV dysfunction who have a conventionaldication for pacing and in whom ventricular pacing ispected to predominate may benefit from biventricularcing. A prospective randomized trial published in 2006ncerning patients with LV enlargement, LVEF less than orual to 40%, and conventional indications for pacingowed that biventricular pacing was associated with im-oved functional class, exercise capacity, LVEF, and serumain natriuretic peptide levels compared with RV pacing98). It has also been demonstrated that LV dysfunction ine setting of chronic RV pacing, and possibly as a result ofV pacing, can be improved with an upgrade to biventricular
cing (299). be
Among patients undergoing AV junction ablation forronic AF, the PAVE (Left Ventricular-Based Cardiac

timulation Post AV Nodal Ablation Evaluation) trial pro-ectively randomized patients between RVA pacing andventricular pacing (300). The patients with RVA pacing hadterioration in LVEF that was avoided by the patients withventricular pacing. The group with biventricular pacing alsod improved exercise capacity compared with the groupith right apical pacing. The advantages of biventricularcing were seen predominantly among patients with reduced

VEF or heart failure at baseline. Other studies have shownat among AF patients who experience heart failure after AVnction ablation and RV pacing, an upgrade to biventricularcing results in improved symptomatology and improved

V function (301,302).These findings raise the question of whether patients witheserved LV function requiring ventricular pacing wouldnefit from initial implantation with a biventricular devicer one with RV pacing at a site with more synchronousntricular activation than at the RV apex, such as pacing ate RV septum, the RV outflow tract (303,304), or the area ofe His bundle) (305). Some patients with normal baselineV function experience deterioration in LVEF after chronicV pacing (47,306). The concern over the effects of long-rm RV pacing is naturally greatest among younger patientsho could be exposed to ventricular pacing for many de-des. Studies have suggested that chronic RVA pacing inung patients, primarily those with congenital completeart block, can lead to adverse histological changes, LVlation, and LV dysfunction (41,306,307).There is a role for CRT-P in some patients, especially those

ho wish to enhance their QOL without defibrillationckup. Elderly patients with important comorbidities arech individuals. Notably, there is an important survivalnefit from CRT-P alone (224,225).

.7. Optimizing Pacemaker Technologynd Costhe cost of a pacemaker system increases with its degree ofmplexity and sophistication. For example, the cost of aal-chamber pacemaker system exceeds that of a single-amber system with respect to the cost of the generator ande second lead (increased by approximately $2,500 [287]),ditional implantation time and supplies (approximately60 [287]), and additional follow-up costs (approximately50 per year [287]). A biventricular pacemaker entails eveneater costs, with the hardware alone adding $5,000 to0,000 to the system cost. With respect to battery life, thata dual-chamber generator is shorter than that of a single-amber generator (287,308) and that of a biventricularvice is shorter still. There are also QOL concerns associ-ed with the more complex systems, including increasedvice size and increased frequency of follow-up. Againstese additional costs are the potential benefits of the morephisticated systems with respect to QOL, morbidity, andortality. Furthermore, when a single-chamber system re-ires upgrading to a dual-chamber system, the costs are

gnificant; one study estimated the cost of such an upgrade to
$14,451 (287).

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An analysis of MOST found that the cost-effectiveness ofal-chamber pacemaker implantation compared with ven-

icular pacemaker implantation (287) was approximately3,000 per quality-adjusted year of life gained over 4 yearsfollow-up. Extended over the expected lifetime of a typicaltient, the calculated cost-effectiveness of dual-chambercing improved to $6,800 per quality-adjusted year of lifeined.It has been estimated that 16% to 24% of pacemakerplantations are for replacement of generators; of those,% are replaced because their batteries have reached their

ective replacement time (309,310). Hardware and software.e., programming) features of pacemaker systems that pro-ng useful battery longevity may improve the cost-fectiveness of pacing. Leads with steroid elution and/or highcing impedance allow for less current drain. Optimalogramming of output voltages, pulse widths, and AVlays can markedly decrease battery drain; one studyowed that expert programming of pacemaker generatorsn have a major impact on longevity, prolonging it by anerage of 4.2 years compared with nominal settings (311).enerators that automatically determine whether a pacingpulse results in capture allow for pacing outputs closer to

reshold values than conventional generators. Althoughese and other features arguably should prolong generatorfe, there are other constraints on the useful life of acemaker generator, including battery drain not directlylated to pulse generation and the limited life expectancy ofany pacemaker recipients; rigorous studies supporting theerall cost-effectiveness of these advanced pacing featurese lacking.

.8. Pacemaker Follow-Upfter implantation of a pacemaker, careful follow-up andntinuity of care are required. The writing committeensidered the advisability of extending the scope of theseidelines to include recommendations for follow-up andvice replacement but deferred this decision given otherblished statements and guidelines on this topic. These aredressed below as a matter of information; however, nodorsement is implied. The HRS has published a series ofports on antibradycardia pacemaker follow-up (312,313).he Canadian Working Group in Cardiac Pacing has alsoblished a consensus statement on pacemaker follow-up14). In addition, the Centers for Medicare and Medicaid

ervices has established guidelines for monitoring of patientsvered by Medicare who have antibradycardia pacemakers,though these have not been updated for some time (315).Many of the same considerations are relevant to follow-uppacemakers, ICDs, and CRT systems. Programming un-

rtaken at implantation should be reviewed before discharged changed accordingly at subsequent follow-up visits asdicated by interrogation, testing, and patient needs. Withreful attention to programming pacing amplitude, pulseidth, and diagnostic functions, battery life can be enhancedgnificantly without compromising patient safety. Takingvantage of programmable options also allows optimization
pacemaker function for the individual patient. m
The frequency and method of follow-up are dictated byultiple factors, including other cardiovascular or medicaloblems managed by the physician involved, the age of thecemaker, and geographic accessibility of the patient to medi-l care. Some centers may prefer to use remote monitoring withtermittent clinic evaluations, whereas others may prefer to doe majority or all of the patient follow-up in a clinic.For many years, the only “remote” follow-up was transtele-onic monitoring (TTM). Available for many years, TTMovides information regarding capture of the chamber(s) beingced and battery status. TTM may also provide the caregiverith information regarding appropriate sensing. However, incent years, the term “remote monitoring” has evolved todicate a technology that is capable of providing a great deal ofditional information. Automatic features, such as automaticreshold assessment, have been incorporated increasingly intower devices and facilitate follow-up for patients who live far

om follow-up clinics (316). However, these automatic func-ns are not universal and need not and cannot supplant thenefits of direct patient contact, particularly with regard tostory taking and physical examination.A more extensive clinic follow-up usually includes assess-ent of the clinical status of the patient, battery status, pacingreshold and pulse width, sensing function, and lead integ-ty, as well as optimization of sensor-driven rate responsed evaluation of recorded events, such as mode switchingr AF detection and surveillance and ventricular tachyar-ythmia events. The schedule for clinic follow-up should bethe discretion of the caregivers who are providing pace-

aker follow-up. As a guideline, the 1984 Health Careinancing Administration document suggests the following:r single-chamber pacemakers, twice in the first 6 monthster implantation and then once every 12 months; foral-chamber pacemakers, twice in the first 6 months, thence every 6 months (315).Regulations regarding TTM have not been revised since84 (315). Guidelines that truly encompass remote monitor-g of devices have not yet been endorsed by any of the majorofessional societies. The Centers for Medicare and Medic-d Services have not provided regulations regarding the use

this technology, but have provided limited directiongarding reimbursement. The Centers for Medicare andedicaid Services have published a statement that physiciansould use the existing current procedural terminology codesr in-office pacemaker and ICD interrogation codes formote monitoring of cardiac devices (317). Clearly statedidelines from professional societies are necessary andould be written in such a way as to permit remoteonitoring that achieves specific clinical goals. Guidelinese currently in development given the rapid advancement inmote monitoring technology.Appropriate clinical goals of remote monitoring should beentified and guidelines developed to give caregivers theility to optimize the amount of clinical information that canderived from this technology. Appropriate clinical goals of

TM should be divided into those pieces of informationtainable during nonmagnet (i.e., free-running) ECG assess-ent and assessment of the ECG tracing obtained during
agnet application. The same goals should be achieved

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hether the service is being provided by a commercial orncommercial monitoring service.Goals of TTM nonmagnet ECG assessment are as follows:

Determine whether the patient displays intrinsic rhythm oris being intermittently or continuously paced at the pro-grammed settings.Characterize the patient’s underlying atrial mechanism, forexample, sinus versus AF, atrial tachycardia, etc.If intrinsic rhythm is displayed, determine that normal(appropriate) sensing is present for 1 or both chambersdepending on whether it is a single- or dual-chamberpacemaker and programmed pacing mode.

Goals of TTM ECG assessment during magnet applicatione as follows:

Verify effective capture of the appropriate chamber(s)depending on whether it is a single- or dual-chamberpacemaker and verify the programmed pacing mode.Assess magnet rate. Once magnet rate is determined, thevalue should be compared with values obtained on previ-ous transmissions to determine whether any change hasoccurred. The person assessing the TTM should also beaware of the magnet rate that represents elective replace-ment indicators for that pacemaker.If the pacemaker is one in which pulse width is 1 of theelective replacement indicators, the pulse width shouldalso be assessed and compared with previous values.If the pacemaker has some mechanism to allow transtele-phonic assessment of threshold (i.e., Threshold MarginTest [TMT™]) and that function is programmed “on,” theresults of this test should be demonstrated and analyzed.If a dual-chamber pacemaker is being assessed and magnetapplication results in a change in AV interval duringmagnet application, that change should be demonstratedand verified.

.8.1. Length of Electrocardiographic Samplesr Storageis important that the caregiver(s) providing TTM assess-ent be able to refer to a paper copy or computer-archivedpy of the transtelephonic assessment for subsequent care.

he length of the ECG sample saved should be based on theinical information that is required (e.g., the points listedove). It is the experience of personnel trained in TTM thatcarefully selected ECG sample of 6 to 9 seconds canmonstrate all of the points for each of the categories listedove (i.e., a 6- to 9-second strip of nonmagnet and 6- tosecond strip of magnet-applied ECG tracing).

.8.2. Frequency of Transtelephonic Monitoringhe follow-up schedule for TTM varies among centers, andere is no absolute schedule that needs be mandated. Re-rdless of the schedule to which the center may adhere,

TM may be necessary at unscheduled times if, for example,e patient experiences symptoms that potentially reflect anteration in rhythm or device function.The majority of centers with TTM services follow thehedule established by the Health Care Financing Adminis-
ation (now the Centers for Medicare and Medicaid Ser-
ces). In the 1984 Health Care Financing Administrationidelines, there are 2 broad categories for follow-up (asown in Table 4): Guideline I, which was thought to applythe majority of pacemakers in use at that time, and

uideline II, which would apply to pacemaker systems forhich sufficient long-term clinical information exists tosure that they meet the standards of the Inter-Society

ommission for Heart Disease Resources for longevity andd-of-life decay. The standards to which they referred are% cumulative survival at 5 years after implantation and and-of-life decay of less than a 50% drop in output voltaged less than a 20% deviation in magnet rate, or a drop of 5m or less, over a period of 3 months or more. As of 2000,appears that most pacemakers would meet the specifica-

ons in Guideline II.Note that there is no federal or clinical mandate that these

TM guidelines be followed. The ACC, AHA, and HRS havet officially endorsed the Health Care Financing Adminis-

ation guidelines. Nevertheless, they may be useful as aamework for TTM. An experienced center may choose to

less frequent TTM and supplement it with in-clinicaluations as stated previously.Goals of contemporary remote monitoring are as follows:

Review all programmed parametersReview stored events (e.g., counters, histograms, andelectrograms)If review of programmed parameters or stored eventssuggests a need for reprogramming or a change in therapy,

ble 4. Device Monitoring Times Postimplantation: Healthre Financing Administration 1984 Guidelines foranstelephonic Monitoring

ostimplantation Milestone Monitoring Time

ideline I

Single chamber

1st month Every 2 weeks

2nd to 36th month Every 8 weeks

37th month to failure Every 4 weeks

Dual chamber



7th to 36th month Every 8 weeks


ideline II

Single chamber




Dual chamber



31st to 48th month Every 8 weeks


Modified from the US Department of Health and Human Services (315). In theblic domain.

arrange a focused in-clinic appointment.

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.8.3. Remote Follow-Up and MonitoringEW SECTION)

ince the publication of the 2008 DBT guideline, importantanges have occurred related to follow-up and remoteonitoring of cardiovascular implantable electronic devicesIEDs). (1d,541,590). CIEDs include pacemakers, ICDs,

RTs, implantable loop recorders, and implantable cardio-scular monitors. The current technology for follow-up,idence supporting its use, and clinical practice of CIEDonitoring have evolved. Routine in-person office follow-uppplemented by transtelephonic monitoring with limitedmote follow-up for pacemakers was the standard approachfore 2008 (1d,541). Transtelephonic monitoring, with mon-

ors that transmit the patient’s heart rhythm by convertingectrocardiographic information to sound and transmitting ita telephone lines to a decoding machine that then convertse sound back into a rhythm strip, is now a dated techniqued,541,590). because it allows for limited monitoring ofart rate, rhythm, and battery status of only pacemakers90).Contemporary remote monitoring uses bidirectional telem-

ry with encoded and encrypted radiofrequency signals,lowing transmission and receipt of information from CIEDsacemakers, ICDs, CRTs, implantable loop recorders, andplantable hemodynamic monitors) (590). All major CIED

anufacturers have developed proprietary systems to allowtients to have their devices interrogated remotely, andany use wireless cellular technology to extend the bidirec-onal telemetry links into the patient’s location (541,590).he information is analyzed, formatted, and transmitted to antral server, where it can be accessed by clinicians throughe Internet. Information provided through remote follow-upcludes virtually all of the stored information that would betained in an in-office visit, including battery voltage, charge

me in ICDs, percent pacing, sensing thresholds, automati-lly measured pacing thresholds when available, pacing andock impedance, and stored arrhythmia events with electro-ams (541,590). CIEDs with wireless telemetry capabilityay be programmed at a face-to-face evaluation to subse-ently send automatic alerts for a variety of issues that the

inician deems significant, such as abnormal battery voltage,normal lead parameters, or increased duration or frequencyarrhythmia episodes (541). Remote transmissions can be

ade at predetermined intervals or at unscheduled times forespecified alerts related to device function or activated bye patient for clinical reasons (590). A detailed description ofchniques, indications, personnel, and frequency has beenblished as a consensus document (541).Several prospective randomized trials have been con-cted evaluating the effect of remote monitoring on

inical outcomes (591–594) since the publication of the08 DBT Guideline (1d). Collectively, these trials havemonstrated that remote monitoring is a safe alternativeoffice visits to evaluate CIEDs. Compared with in-

rson office visits to evaluate CIEDs, remote monitoringads to early discovery of clinically actionable events,creased time to clinical decision in response to theseents, and fewer office visits (591–594). Long-term sur-
val rates of patients monitored remotely with ICDs in a tr
actice setting compare favorably with survival rates oftients in clinical trials (595).Current suggestions for the minimum frequency of in-

fice and remote monitoring of patients with CIEDs aremmarized in Table 4a (541). Issues such as lead malfunc-

on, unreliable battery life indicators, and other device orad recalls influence clinical decisions, which may changee appropriate minimum follow-up.

3. Indications for ImplantableCardioverter-Defibrillator Therapy

dications for ICDs have evolved considerably from initialplantation exclusively in patients who had survived 1 or

ore cardiac arrests and failed pharmacological therapy18). Multiple clinical trials have established that ICD usesults in improved survival compared with antiarrhythmicents for secondary prevention of SCD (16,319–326). Largeospective, randomized, multicenter studies have also estab-

shed that ICD therapy is effective for primary prevention ofdden death and improves total survival in selected patientpulations who have not previously had a cardiac arrest orstained VT (16–19,327–331).We acknowledge that the “ACC/AHA/ESC 2006 Guide-

nes for Management of Patients With Ventricular Arrhyth-ias and the Prevention of Sudden Cardiac Death” (16) used

LVEF of less than 40% as a critical point to justify ICDplantation for primary prevention of SCD. The LVEF usedclinical trials assessing the ICD for primary prevention of

CD ranged from less than or equal to 40% in MUSTTulticenter Unsustained Ventricular Tachycardia Trial) to

ss than or equal to 30% in MADIT II (Multicenter Auto-atic Defibrillator Implantation Trial II) (329,332). Two

ble 4a. Minimum Frequency of CIED In-Person oremote Monitoring*

Type and Frequency Method

cemaker/ICD/CRT

Within 72 h of CIED implantation In person

2–12 wk postimplantation In person

Every 3–12 mo for pacemaker/CRT-Pacemaker In person or remote

Every 3–6 mo for ICD/CRT-D In person or remote

Annually until battery depletion In person

Every 1–3 mo at signs of battery depletion In person or remote

plantable loop recorder

Every 1–6 mo depending on patient symptomsand indication

In person or remote

plantable hemodynamic monitor

Every 1–6 mo depending on indication In person or remote

More frequent assessment as clinically indicated In person or remote

CIED indicates cardiovascular implantable electronic device; CRT, cardiacsynchronization therapy; CRT-D, cardiac resynchronization therapy defibril-tor; CRT-Pacemaker, cardiac resynchronization therapy pacemaker; and ICD,plantable cardioverter-defibrillator.*More frequent in-person or remote monitoring may be required for all theove devices as clinically indicated.Modified from Wilkoff et al (541).

ials, MADIT I (Multicenter Automatic Defibrillator Implan-

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tion Trial I) (327) and SCD-HeFT (Sudden Cardiac DeathHeart Failure Trial) (333), used LVEFs of less than or

ual to 35% as entry criteria. The present writing committeeached the consensus that it would be best to have ICDsfered to patients with clinical profiles as similar to thosecluded in the trials as possible. Having given carefulnsideration to the issues related to LVEF for these updatedD guidelines, we have written these indications for ICDssed on the specific inclusion criteria for LVEF in the trials.

ecause of this, there may be some variation from previouslyblished guidelines (16).We also acknowledge that the determination of LVEF

cks a “gold standard” and that there may be variationong the commonly used clinical techniques of LVEF

termination. All clinical methods of LVEF determinationck precision, and the accuracy of techniques varies amongstboratories and institutions. Given these considerations, theesent writing committee recommends that the clinician usee LVEF determination that they believe is the most clini-lly accurate and appropriate in their institution.Patient selection, device and lead implantation, follow-up,d replacement are parts of a complex process that requiresmiliarity with device capabilities, adequate case volume,ntinuing education, and skill in the management of ven-

icular arrhythmias, thus mandating appropriate training andedentialing. Training program requirements for certificationograms in clinical cardiac electrophysiology that includeD implantation have been established by the American

oard of Internal Medicine and the American Osteopathicoard of Internal Medicine. Individuals with basic certifica-on in pediatric cardiology and cardiac surgery may receivemilar training in ICD implantation. In 2004, requirementsr an “alternate training pathway” for those with substantialior experience in pacemaker implantation were proposed bye HRS with a scheduled expiration for this alternatethway in 2008 (11,12). Fifteen percent of physicians whoplanted ICDs in 2006 reported in the national ICD registry

at they had no formal training (electrophysiology fellow-ip, cardiac surgical training, or completion of the alternatethway recommendation) (11,12,334).The options for management of patients with ventricularrhythmias include antiarrhythmic agents, catheter ablation,d surgery. The “ACC/AHA/ESC 2006 Guidelines foranagement of Patients With Ventricular Arrhythmias ande Prevention of Sudden Cardiac Death” have been pub-shed with a comprehensive review of management options,cluding antiarrhythmic agents, catheter ablation, surgery,d ICD therapy (16).

.1. Secondary Prevention ofudden Cardiac Death

.1.1. Implantable Cardioverter-Defibrillator Therapyr Secondary Prevention of Cardiac Arrest and

ustained Ventricular Tachycardiaecondary prevention refers to prevention of SCD in thosetients who have survived a prior sudden cardiac arrest orstained VT (16). Evidence from multiple randomized
ntrolled trials supports the use of ICDs for secondary dr
evention of sudden cardiac arrest regardless of the typeunderlying structural heart disease. In patients resusci-

ted from cardiac arrest, the ICD is associated withinically and statistically significant reductions in suddenath and total mortality compared with antiarrhythmicug therapy in prospective randomized controlled trials6,319 –326).Trials of the ICD in patients who have been resuscitated

om cardiac arrest demonstrate survival benefits with ICDerapy compared with electrophysiologically guided drugerapy with Class I agents, sotalol, and empirical amioda-ne therapy (320,323). A large prospective, randomizedcondary prevention trial comparing ICD therapy with ClassI antiarrhythmic drug therapy (predominantly empirical

iodarone) demonstrated improved survival with ICD ther-y (319). Unadjusted survival estimates for the ICD groupd the antiarrhythmic drug group, respectively, were 89.3%rsus 82.3% at 1 year, 81.6% versus 74.7% at 2 years, and.4% versus 64.1% at 3 years (p�0.02). Estimated relative

sk reduction with ICD therapy was 39% (95% CI 19% to%) at 1 year, 27% (95% CI 6% to 48%) at 2 years, and 31%5% CI 10% to 52%) at 3 years. Two other reports of largeospective trials in similar patient groups have shownmilar results (322,323).The effectiveness of ICDs on outcomes in the recent large,ospective secondary prevention trials—AVID (Antiar-ythmics Versus Implantable Defibrillators) (319), CASHardiac Arrest Study Hamburg) (321), and CIDS (Canadianplantable Defibrillator Study) (322)—were consistent with

ior investigations (320). Specifically, the ICD was associ-ed with a 50% relative risk reduction for arrhythmic deathd a 25% relative risk reduction for all-cause mortality24). Thus, the secondary prevention trials have been robustd have shown a consistent effect of improved survival withD therapy compared with antiarrhythmic drug therapyross studies (324).Some individuals are resuscitated from cardiac arrest duepossible transient reversible causes. In such patients,

yocardial revascularization may be performed when appro-iate to reduce the risk of recurrent sudden death, withdividualized decisions made with regard to the need forD therapy (16). Sustained monomorphic VT with prior MIunlikely to be affected by revascularization (16). Myocar-al revascularization may be sufficient therapy in patientsrviving VF in association with myocardial ischemiahen ventricular function is normal and there is no historyan MI (16).Unless electrolyte abnormalities are proven to be the soleuse of cardiac arrest, survivors of cardiac arrest in whomectrolyte abnormalities are discovered in general should beeated in a manner similar to that of cardiac arrest survivorsithout electrolyte abnormalities (16). Patients who experi-ce sustained monomorphic VT in the presence of antiar-ythmic drugs or electrolyte abnormalities should also bealuated and treated in a manner similar to patients with VTVF without electrolyte abnormalities or antiarrhythmic

ugs (16).

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.1.2. Specific Disease States and Secondaryrevention of Cardiac Arrest or Sustainedentricular Tachycardiahe majority of patients included in prior prospective ran-mized trials of patients resuscitated from cardiac arrestve had coronary artery disease with impaired ventricularnction (320,322,323,325,326). Patients with other types ofructural heart disease constitute a minority of patients in thecondary prevention trials. However, supplemental observa-

onal and registry data support the ICD as the preferredrategy over antiarrhythmic drug therapy for secondaryevention for patients resuscitated from cardiac arrest due toT or fibrillation with coronary artery disease and otherderlying structural heart disease.

.1.3. Coronary Artery Diseaseatients with coronary artery disease represent the majority oftients receiving devices in prior reports of patients surviv-g cardiac arrest. Evidence strongly supports a survivalnefit in such patients with an ICD compared with othererapy options (319,322,323). Between 73% and 83% oftients enrolled in the AVID, CASH, and CIDS trials hadderlying coronary artery disease (319,321,322). The mean

VEF ranged from 32% to 45% in these trials, whichdicates prior MI in the majority of patients (319,322,323).ultiple analyses have supported the notion that patients withduced LV function may experience greater benefit withD therapy than with drug therapy (320,335–338). Alltients undergoing evaluation for ICD therapy should beven optimum medical treatment for their underlying car-ovascular condition (16).Patients experiencing cardiac arrest due to VF that occurs

ore than 48 hours after an MI may be at risk for recurrentrdiac arrest (16). It is recommended that such patients bealuated and optimally treated for ischemia (16). If there isidence that directly and clearly implicates ischemia imme-ately preceding the onset of VF without evidence of a priorI, the primary therapy should be complete coronary revas-larization (16). If coronary revascularization is not possibled there is evidence of significant LV dysfunction, theimary therapy for patients resuscitated from VF should bee ICD (16).Patients with coronary artery disease who present withstained monomorphic VT or VF and low-level elevationscardiac biomarkers of myocyte injury/necrosis should be

eated similarly to patients who have sustained VT and nocumented rise in biomarkers (16). Prolonged episodes ofstained monomorphic VT or VF may be associated with a

se in cardiac troponin and creatine phosphokinase levels duemyocardial metabolic demands that exceed supply in

tients with coronary artery disease. Evaluation for ischemiaould be undertaken in such patients (16). However, whenstained VT or VF is accompanied by modest elevations ofrdiac enzymes, it should not be assumed that a new MI wase cause of the sustained VT (16). Without other clinical datasupport the occurrence of a new MI, it is reasonable tonsider that such patients are at risk for recurrent sustainedT or VF (16). With these considerations in mind, these
tients should be treated for this arrhythmia in the same V
anner as patients without biomarker release accompanyingT (16).

.1.4. Nonischemic Dilated Cardiomyopathyatients with nonischemic DCM and prior episodes of VF orstained VT are at high risk for recurrent cardiac arrest.

mpirical antiarrhythmic therapy or drug therapy guided byectrophysiological testing has not been demonstrated toprove survival in these patients. The ICD has been shownbe superior to amiodarone for secondary prevention of VTd VF in studies in which the majority of patients hadronary artery disease (322,323,336), but the subgroups withnischemic DCM in these studies benefited similarly19,322,323) or more than the group with ischemic heartilure (324). On the basis of these data, the ICD is theeferred treatment for patients with nonischemic DCMsuscitated from prior cardiac arrest from VF or VT.

.1.5. Hypertrophic CardiomyopathyCM is an inherited heart muscle disease that affects approx-ately 1 of every 500 persons in the general population andthe most common cause of cardiac arrest in individualsunger than 40 years of age (339). HCM should be sus-cted as the cause of cardiac arrest in young individualsring exertion, because exercise increases the risk of life-reatening ventricular arrhythmias with this condition (339).udden death may also be the first manifestation of thesease in a previously asymptomatic individual. A history ofior cardiac arrest indicates a substantial risk of future VT orF with this condition (339). Prospective randomized trialsICD versus pharmacological therapy for patients with priorrdiac arrest and HCM have not been performed; however,gistry data and observational trials are available (339,340).In those patients with HCM resuscitated from prior cardiac

rest, there is a high frequency of subsequent ICD therapyr life-threatening ventricular arrhythmias (339). On thesis of these data, the ICD is the preferred therapy for suchtients with HCM resuscitated from prior cardiac arrest39,340).

.1.6. Arrhythmogenic Right Ventricularysplasia/Cardiomyopathyrrhythmogenic RV dysplasia/cardiomyopathy (ARVD/C) isgenetic condition characterized by fibrofatty infiltration ofe RV and less commonly the LV. It usually manifestsinically with sustained monomorphic VT with left bundleorphology in young individuals during exercise. There are

prospective randomized trials of pharmacological therapyrsus ICD therapy in patients with ARVD/C for secondaryevention of SCD; however, observational reports fromultiple centers consistently demonstrate a high frequency ofpropriate ICD use for life-threatening ventricular arrhyth-ias and a very low rate of arrhythmic death in patients withRVD/C treated with an ICD (341–348).

.1.7. Genetic Arrhythmia Syndromesenetic syndromes that predispose to sustained VT or VFclude the long- and short-QT syndromes, Brugada syn-ome, idiopathic VF, and catecholaminergic polymorphic
T (338,349–356). These primary electrical conditions typ-

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ally exist in the absence of any underlying structural heartsease and predispose to cardiac arrest. Although contro-rsy still exists with regard to risk factors for sudden deathith these conditions, there is consensus that those with priorrdiac arrest or syncope are at very high risk for recurrentrhythmic events. On the basis of the absence of any clear ornsistent survival benefit of pharmacological therapy forose individuals with these genetic arrhythmia syndromes,e ICD is the preferred therapy for those with prior episodes

sustained VT or VF and may also be considered forimary prevention for some patients with a very strongmily history of early mortality (see Section 3.2.4, “Hyper-ophic Cardiomyopathy,” and Section 3.2.7, “Primary Elec-ical Disease”).

.1.8. Syncope With Inducible Sustainedentricular Tachycardiaatients with syncope of undetermined origin in whominically relevant VT/VF is induced at electrophysiologicaludy should be considered candidates for ICD therapy. Inese patients, the induced arrhythmia is presumed to be theuse of syncope (341,357–366). In patients with hemody-mically significant and symptomatic inducible sustainedT, ICD therapy can be a primary treatment option. Appro-iate ICD therapy of VT and VF documented by storedectrograms lends support to ICD therapy as a primaryeatment for DCM patients with syncope (341,367).

.2. Primary Prevention ofudden Cardiac Deathrimary prevention of SCD refers to the use of ICDs individuals who are at risk for but have not yet had an episodesustained VT, VF, or resuscitated cardiac arrest. Clinical

ials have evaluated the risks and benefits of the ICD inevention of sudden death and have improved survival inultiple patient populations, including those with prior MId heart failure due to either coronary artery disease ornischemic DCM. Prospective registry data are less robustt still useful for risk stratification and recommendations forD implantation in selected other patient populations, suchthose with HCM, ARVD/C, and the long-QT syndrome. In

ss common conditions (e.g., Brugada syndrome, cat-holaminergic polymorphic VT, cardiac sarcoidosis, and LVncompaction), clinical reports and retrospectively analyzedries provide less rigorous evidence in support of currentcommendations for ICD use, but this constitutes the bestailable evidence for these conditions.

.2.1. Coronary Artery Diseasehere now exists a substantial body of clinical trial data thatpport the use of ICDs in patients with chronic ischemicart disease. A variety of risk factors have been used toentify a high-risk population for these studies. MADIT I27) and MUSTT (329) required a history of MI, spontane-s nonsustained VT, inducible VT at electrophysiological

udy, and a depressed LVEF (less than or equal to 35% orss than or equal to 40%, respectively) to enter the study.ADIT I showed a major relative risk reduction of 54% with
e ICD. MUSTT was not specifically a trial of ICD therapy, w
cause it compared no therapy with electrophysiologicallyided therapy, but in the group randomized to electrophysi-ogically guided therapy, benefit was seen only among thoseho received an ICD.MADIT II (332) enrolled 1,232 patients with ischemicrdiomyopathy and an LVEF less than or equal to 30%. Noontaneous or induced arrhythmia was required for enroll-ent. All-cause mortality was 20% in the control group and.2% in the ICD group (relative risk 31%; p�0.016).

CD-HeFT included patients with both ischemic and non-chemic cardiomyopathies, an LVEF less than or equal to%, and NYHA Class II or III congestive heart failure (333).

mong the 1,486 patients with ischemic heart disease ran-mized to either placebo or ICD therapy, the 5-year eventtes were 0.432 and 0.359, respectively (HR 0.79; p�0.05).wo recent meta-analyses of these trials have supported theerall conclusion that ICD therapy in high-risk individuals

ith coronary artery disease results in a net risk reduction fortal mortality of between 20% and 30% (325,368).Two trials, however, have failed to show improved sur-

val with ICD therapy in patients either at the time ofrgical revascularization or within 40 days of an acute MI. Ine CABG-Patch (Coronary Artery Bypass Graft-Patch) trial28), routine ICD insertion did not improve survival intients with coronary artery disease undergoing bypassrgery who were believed to be at high risk of sudden deaththe basis of an abnormal signal-averaged ECG and severe

V dysfunction (LVEF less than or equal to 35%). Similarta about the effects of percutaneous revascularization aret available. In DINAMIT (Defibrillator in Acute Myocar-al Infarction Trial) (331), 674 patients with a recent MIithin 6 to 40 days), reduced LV function (LVEF less thanequal to 35%), and impaired cardiac autonomic function

epressed heart rate variability or elevated average heartte) were randomized to either ICD therapy or no ICDerapy. Although arrhythmic death was reduced in the ICDoup, there was no difference in total mortality (18.7%rsus 17.0%; HR for death in the ICD group 1.08; p�0.66).

ee Table 5 for further information.

.2.2. Nonischemic Dilated Cardiomyopathyultiple randomized prospective trials now supplement theailable observational studies that have reported on the rolethe ICD in primary prevention of SCD in patients withnischemic DCM (16,224,333,369 –379) Observational

udies suggest that up to 30% of deaths in patients withCM are sudden (380). Mortality in medically treated pa-ents with DCM and a prior history of syncope may exceed% at 2 years, whereas those treated with an ICD experiencehigh frequency of appropriate ICD therapy (16,372,373).CAT (Cardiomyopathy Trial) enrolled patients with re-ntly diagnosed DCM with randomization to medical ther-y versus medical therapy with an ICD (377). The study wasrminated before the primary end point was reached because

a lower-than-expected incidence of all-cause mortality77). There was no statistical probability of finding agnificant survival advantage with either strategy. With 50tients in the ICD arm and 54 in the control group, the study
as underpowered to find a difference in survival with ICD

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erapy. At the time of 5-year follow-up, there were feweraths in the ICD group than in the control group (13 versus, respectively) (377).Another inconclusive trial was the AMIOVIRT (Amioda-ne Versus Implantable Defibrillator in Patients with Non-chemic Cardiomyopathy and Asymptomatic Nonsustainedentricular Tachycardia) study (378). The trial randomized3 patients with DCM, LVEF less than or equal to 35%, andnsustained VT to amiodarone or ICD. The study was

opped prematurely due to statistical futility in reaching theimary end point of reduced total mortality (378). TheEFINITE (Defibrillators in Nonischemic Cardiomyopathyreatment Evaluation) trial randomized 458 patients withnischemic cardiomyopathy, NYHA Class I to III heartilure, LVEF less than or equal to 35%, and more than 10emature ventricular complexes per hour or nonsustainedT to optimal medical therapy with or without an ICD (369).ith a primary end point of all-cause mortality, statistical

gnificance was not reached, but there was a strong trendward reduction of mortality with ICD therapy (p�0.08).fter 2 years, mortality was 14.1% in the standard therapyoup versus 7.9% among those receiving an ICD, whichsulted in a 6.2% absolute reduction and a 35% relative riskduction with ICD implantation (369). The results werensistent and comparable to those of other similar trials6,333,379).SCD-HeFT compared amiodarone, ICD, and optimal med-

al therapy in 2,521 patients with coronary artery disease ornischemic cardiomyopathy with NYHA functional Class IIIII heart failure and LVEF less than or equal to 35% (333).

he amiodarone treatment group received the drug by way ofdouble-blinded, placebo-controlled design (333). The me-an follow-up was 45.5 months. The absolute mortality

ble 5. Major Implantable Cardioverter-Defibrillator Trials for P

Trial YearPatients

(n)Inclusion Criterion: LVEF %

Less Than or Equal to

ADIT I (327) 1996 196 35

ADIT II (332) 2002 1,232 30

BG-Patch (328) 1997 900 36

FINITE (369) 2004 485 35

NAMIT (331) 2004 674 35

D-HeFT (333) 2005 1,676 35

ID (319) 1997 1,016 40

SH† (323) 2000 191 M: 45�18 at baseline

DS (322) 2000 659 35

AVID indicates Antiarrhythmics Versus Implantable Defibrillators; CABG, coronaplantable Defibrillator Study; DEFINITE, Defibrillators in Nonischemic Cardiomial; EP, electrophysiological study; HRV, heart rate variability; LVD, left ventriculfibrillator Implantation Trial I; MADIT II, Multicenter Automatic Defibrillator Imrdiomyopathy; NS, not statistically significant; NSVT, nonsustained ventricuectrocardiogram; and SCD-HeFT, Sudden Cardiac Death in Heart Failure Trial*Hazard ratios for death due to any cause in the implantable cardioverter-de†Includes only implantable cardioverter-defibrillator and amiodarone patients‡Upper bound of 97.5% confidence interval.§One-tailed.

crease in the medical group was 7.2% after 5 years in the 95

erall population. The ICD group experienced a decreasedsk of death of 23% compared with the placebo group (HR77, 97.5% CI 0.62 to 0.96), and total mortality in theedical group was 7.2% per year, with a risk reduction of% in the ICD group versus placebo (95% CI 0.62 to 0.96;0.007). Relative risk reduction was comparable for the

oup with LV dysfunction due to prior MI and the nonisch-ic group, but absolute mortality was lower in the nonisch-ic group. This resulted in a greater number needed to treat

r life saved among ischemic patients. There was noortality difference between the amiodarone and placebooups. Further risk stratification may decrease the number ofdividuals needed to undergo ICD implantation to save a lifethis population.With the exception of DEFINITE (25% in the ICD arm),

ials assessing ICD therapy in primary prophylaxis of DCMve not generally included asymptomatic patients in NYHAnctional Class I; therefore, the efficacy of ICDs in thispulation is not fully known. Because mortality may be lowthis subgroup, the benefit of ICD therapy is moderate atst (369).The COMPANION trial randomized patients with Class IIIIV heart failure, ischemic or nonischemic DCM, and QRSration greater than 120 milliseconds in a 1:2:2 ratio toceive optimal pharmacological therapy alone or in combi-tion with CRT with either a pacemaker or a pacemaker-fibrillator (224). Of the 1,520 patients randomized in the

ial, 903 were allocated to either the medical therapy orfibrillator arms; of this subset, 397 (44%) had DCM.

ardiac resynchronization with an ICD significantly reducedl-cause mortality compared with pharmacological therapyone in patients with DCM (HR for all-cause death 0.50,

on of Sudden Cardiac Death

Other Inclusion CriteriaHazardRatio*

95% ConfidenceInterval p

VT and positive EP 0.46 0.26 to 0.82 0.009

ior MI 0.69 0.51 to 0.93 0.016

sitive SAECG and CABG 1.07 0.81 to 1.42 0.64

CM, PVCs, or NSVT 0.65 0.40 to 1.06 0.08

to 40 days after MI andimpaired HRV

1.08 0.76 to 1.55 0.66

ior MI or NICM 0.77 0.62 to 0.96 0.007

ior cardiac arrest 0.62 0.43 to 0.82 �0.02

ior cardiac arrest 0.77 1.112‡ 0.081§

ior cardiac arrest,syncope

0.82 0.60 to 1.10 NS

bypass graft surgery; CASH, Cardiac Arrest Study Hamburg; CIDS, CanadianTreatment Evaluation; DINAMIT, Defibrillator in Acute Myocardial Infarction

nction; LVEF, left ventricular ejection fraction; MADIT I, Multicenter Automaticn Trial II; MI, myocardial infarction; NA, not applicable; NICM, nonischemicycardia; PVCs, premature ventricular complexes; SAECG, signal-averaged

r group compared with the non-implantable cardioverter-defibrillator group.ASH.

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Two studies have evaluated the time dependence of risk fordden death relative to the time of diagnosis of nonischemicCM (369,381). An analysis of the DEFINITE study dem-strated that those who have a recent cardiomyopathyagnosis do not benefit less from use of an ICD than thoseith a remote diagnosis (369). On the basis of these data, ICDerapy should be considered in such patients provided that aversible cause of transient LV function has been excludedd their response to optimal medical therapy has beensessed. The optimal time required for this assessment iscertain; however, another analysis determined that patients

ith nonischemic DCM experienced equivalent occurrencestreated and potentially lethal arrhythmias irrespective of

agnosis duration (381). These findings suggest that use of ame qualifier relative to the time since diagnosis of anischemic DCM may not reliably discriminate patients atgh risk for SCD in this selected population (381). Givenese considerations, physicians should consider the timing offibrillator implantation carefully.

.2.3. Long-QT Syndromehe long-QT syndromes represent a complex spectrum ofectrophysiological disorders characterized by a propensityr development of malignant ventricular arrhythmias, espe-ally polymorphic VT (382,383). Because this is a primaryectrical disorder, with most patients having no evidence ofructural heart disease or LV dysfunction, the long-termognosis is excellent if arrhythmia is controlled. Long-termeatment with beta blockers, permanent pacing, or leftrvicothoracic sympathectomy may be helpful (384–386).D implantation is recommended for selected patients withcurrent syncope despite drug therapy, sustained ventricularrhythmias, or sudden cardiac arrest (349,351,352,387,388).urthermore, use of the ICD for primary prevention of SCDay be considered when there is a strong family history ofCD or when compliance or intolerance to drugs is a concern49,351,352,387,388).The clinical manifestations of a long-QT mutation may be

fluenced by the specific gene involved and the functionalnsequences of the mutation in that gene. Risk stratificationpatients with long-QT syndrome continues to evolve, withta from genetic analysis becoming increasingly useful forinical decision making (389–394).

.2.4. Hypertrophic Cardiomyopathyost individuals with HCM are asymptomatic, and the firstanifestation of the condition may be SCD (245,395–400).CD in patients with HCM is generally related to ventricularrhythmia thought to be triggered by factors such as isch-ia, outflow obstruction, or AF (339). SCD is less fre-ently due to bradycardia (16,339). Among selected high-

sk patients, the annual mortality from HCM has beentimated to be as high as 6% in reports from tertiary centers45,395–398). However, community-based studies suggest aore benign disease in the majority of individuals, with annual mortality rate in the range of 1% or less (16,401–403).Risk factors for SCD have been derived from multipleservational studies and registries (339,404–408). A con-nsus document on HCM from the ACC and the European

ociety of Cardiology categorized known risk factors for 34

CD as “major” and “possible” in individual patients (395).he major risk factors include prior cardiac arrest, spontane-s sustained VT, spontaneous nonsustained VT, familystory of SCD, syncope, LV thickness greater than or equal

30 mm, and an abnormal blood pressure response toercise (395). This consensus document also noted possible

sk factors, which included AF, myocardial ischemia, LVtflow obstruction, high-risk mutations, and intense (com-titive) physical exertion (395). The severity of other symp-ms, such as dyspnea, chest pain, and effort intolerance, hast been correlated with increased risk of SCD (16,395). A

at or hypotensive response to upright or supine exercisesting in patients younger than 40 years old has been shownbe a risk factor for SCD, although the positive predictivelue of this finding is low (395). A normal blood pressuresponse identifies a low-risk group (16,395). The presence ofnsustained VT on Holter monitoring has been associated

ith a higher risk of SCD, although the positive predictivecuracy is relatively low (395). Recent analyses indicate thata high-risk HCM cohort, ICD interventions were frequentd were highly effective in restoring normal sinus rhythm45). However, an important proportion of ICD dischargescur in primary prevention patients who undergo implanta-

on of the ICD for a single risk factor. Therefore, a single riskarker of high risk for sudden cardiac arrest may be sufficientjustify consideration for prophylactic ICD implantation in

lected patients (245).Although no randomized studies are available, the ICD hasen used in patients with cardiac arrest, sustained VT, or VF,ith a high percentage of patients receiving appropriate ICDscharge during follow-up at a rate of 11% per year45,339). In a nonrandomized study of ICD implantation inCM, ICD implantation in a subgroup of patients for primaryophylaxis on the basis of perceived high risk for SCDyncope, family history of SCD, nonsustained VT, inducibleT, or septal thickness greater than or equal to 30 mm)sulted in a lower rate of appropriate discharge of 5% perar (245,339). The ICD is not indicated in the majority ofymptomatic patients with HCM, who will have a relativelynign course. Its role is individualized in the patient consid-ed to be at high risk for SCD (245,339,395). Althoughecise risk stratification has not been validated, patients withultiple risk factors (especially severe septal hypertrophy,eater than or equal to 30 mm) and those with SCDspecially multiple SCDs) in close relatives appear to be atfficiently high risk to merit consideration of ICD therapy6,245).

.2.5. Arrhythmogenic Right Ventricular Dysplasia/ardiomyopathyelected patients with ARVD/C may be at risk for SCD.ecause clinical series have reported favorable outcomesith this therapy for primary prevention of SCD in ARVD/C,e ICD has assumed a larger role in therapy (16,341,342,5–348,409,410). On the basis of the available clinical data

om observational studies, it is reasonable to conclude thate ICD is a reasonable therapy for secondary prevention ofdden cardiac arrest in patients with ARVD/C (16,341,
2,345–348,409,410).

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When the ICD is being considered for primary prevention,should be kept in mind that predictive markers of SCD intients with ARVD/C have not yet been defined in largeospective studies focusing on survival (16,341,342,345–8,409,410). Risk factors that have clinical utility in iden-

fying patients with ARVD/C who are at risk for life-reatening ventricular arrhythmias include induction of VTring electrophysiological testing, detection of nonsustained

T on noninvasive monitoring, male gender, severe RVlation, and extensive RV involvement (16,341,342,345–8,409,410). Young age at presentation (less than 5 years),

V involvement, prior cardiac arrest, and unexplained syn-pe serve as markers of risk (341,342,346–348,411,412).

atients with genotypes of ARVD/C associated with a highsk for SCD should be considered for ICD therapy (345).Although the role of ICD therapy for primary prevention ofdden death in patients with ischemic heart disease andlated, nonischemic cardiomyopathy is well established one basis of multiple clinical trials with a consistent finding ofnefit, the data supporting ICD use in patients with ARVD/Ce less extensive (16,341,342,345–348,409,410). Some au-orities have proposed that an ICD should be implanted intients with ARVD/C and an increased risk for SCD basedthe presence of a previous cardiac arrest, syncope due to

T, evidence of extensive RV disease, LV involvement, oresentation with polymorphic VT and RVA aneurysm,hich is associated with a genetic locus on chromosome42–43 (16,341,342,345–348,409,410).It is evident that there is not yet clear consensus on theecific risk factors that identify those patients with ARVD/Cwhom the probability of SCD is sufficiently high to warrantICD for primary prevention. In the future, the results of

rge prospective registries with rigorous enrollment criteriar patients with ARVD/C in whom ICDs have been placedr primary prevention will give insights into the optimal riskratification techniques for primary prevention. In the mean-me, individualized decisions for primary prevention of SCDust be based on experience, judgment, and the availableta. In considering this decision, the clinician should beindful that in patients with ARVD/C, the ICD has provedfe and reliable in sensing and terminating sustained ven-icular arrhythmias. Sudden death is rare in the availableinical series, whereas appropriate ICD shocks are common6,341,342,345–348,409,410).

.2.6. Noncompaction of the Left Ventricleoncompaction of the LV is a rare congenital cardiomyopa-y characterized anatomically by excessive prominent tra-culae and deep intertrabecular recesses in the LV withouther major congenital cardiac malfunction (410,413–421).he origin of the anatomic abnormalities is likely due to anrest of normal embryogenesis of the endocardium andicardium of the ventricle during development. This leads tospension of the normal compaction process of the looseyocardial meshwork. Diagnosis is difficult and is frequentlyissed or delayed owing to lack of knowledge about thiscommon disease. Echocardiography is considered by manybe the diagnostic procedure of choice, but some cases are
tected by computed tomography or magnetic resonance sy
aging. Abnormalities in the resting ECG, including bundle-anch block or ST-segment depression, are found in mosttients, but the findings do not have a high degree ofnsitivity or specificity (410,413–421).Ventricular arrhythmias and sudden death are among the

ajor complications of this disorder. Sudden death can occurany age, and there are currently no techniques clinicallyeful for risk stratification for life-threatening ventricularrhythmias with noncompaction. Although there is no im-irment of systolic function, ventricular arrhythmias are

equent in noncompaction. Approximately 40% of childrenith noncompaction demonstrate complex ventricular ar-ythmias. Available clinical data indicate that sudden deaththe most common cause of mortality. Although there are noospective trials or registry data, there are sufficient obser-tional data to indicate that placement of an ICD as a

rategy to reduce the risk of sudden death is a reasonableinical strategy (410,413–421).

.2.7. Primary Electrical Disease (Idiopathicentricular Fibrillation, Short-QT Syndrome,rugada Syndrome, and Catecholaminergicolymorphic Ventricular Tachycardia)he Brugada syndrome is characterized by ST-segmentevation across the right precordial leads in association withhigh risk of SCD (16,422–425). Although the Brugada-ttern ECG most commonly shows J-point segment eleva-

on in leads V1 to V3 and right bundle-branch block, the ECGttern can be intermittent (16). Less commonly, the J-pointevation occurs in the inferior leads (16). Patients with therugada syndrome have a structurally normal heart with aimary channelopathy (16,426). This is transmitted with antosomal dominant pattern of inheritance, and more than% of those affected are male. The genetic basis for the

rugada syndrome involves the cardiac sodium channel geneCN5A) (16,426).Cardiac events such as syncope or cardiac arrest occuredominantly in the third and fourth decades of life, al-ough presentation with cardiac arrest in neonates or chil-en has been reported (16,422,424). Fever can acutelyedispose to cardiac arrest in the Brugada syndrome6,422–424.)Risk stratification for SCD in patients with the Brugadandrome is of clinical importance, because implantation ofICD is the only prophylactic measure able to prevent SCD

6,422–424). As with long-QT syndrome, there are no dataowing that family history predicts cardiac events amongmily members with the Brugada syndrome (16). Accord-gly, asymptomatic individuals with the characteristic ECGt with no family history are not necessarily at low risk (16).

dditionally, family members of an individual with SCD dueBrugada syndrome should not be assumed to be at

creased risk of SCD (16). Patients with a spontaneousrugada pattern have a worse prognosis than individuals inhom the typical ECG is observed only after pharmacolog-al drug challenge (16,422–424). Patients with syncope ande ECG pattern of spontaneous ST-segment elevation have afold higher risk of cardiac arrest than patients without
ncope and the spontaneous ECG pattern (16,422,424).

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The role of electrophysiological testing remains controver-al in the Brugada syndrome. Although some investigatorsggest that electrophysiological testing has a useful role in

sk stratification, others have not confirmed this observation.lectrophysiological testing had a low positive predictivelue (23%), but over a 3-year follow-up, it had a very highgative predictive value (93%) (16,422,424). By contrast,

riori et al. reported that electrophysiological testing has aw accuracy in predicting individuals who will experiencerdiac arrest (16,410). Priori et al. have proposed thatninvasive risk stratification based on the ECG and symp-ms provides an accurate alternative for risk stratification6,410).Because only a single gene has been linked to the Brugadandrome, there is still insufficient information about thentribution of genetic defects in predicting clinical outcome6,410,426). Specific mutations in the SCN5A gene do notentify a subset of patients at higher risk of cardiac events6,410,426). SCD is caused by rapid polymorphic VT or VFat frequently occurs at rest or during sleep (16). Patientsith Brugada syndrome usually do not have ventriculartrasystoles or nonsustained runs of VT at Holter recording.

herefore, the therapeutic approach for these patients isntered on the prevention of cardiac arrest.Catecholaminergic polymorphic VT is characterized byntricular tachyarrhythmias that develop in relation to phys-al or emotional stress in the presence of a resting ECG thatows no diagnostic abnormalities at rest (16,428–431). Theitial symptoms often manifest during childhood, althoughte-onset cases have been described (16,385,410,427–431).atecholaminergic polymorphic VT is transmitted by either

autosomal dominant or recessive inheritance pattern.pproximately one-half of the autosomal dominant cases areused by mutations in the gene encoding the cardiac ryan-ine receptor (RyR2) (16). This receptor is responsible forlcium release from the stores of the sarcoplasmic reticulum6). Mutations in the gene that encodes calsequestrinASQ2), a calcium buffering protein in the sarcoplasmicticulum, have been associated with the recessive form oftecholaminergic polymorphic VT (16).Risk stratification for SCD in catecholaminergic polymor-ic VT is not possible given the relatively small number oftients reported. Most clinical reports indicate that betaockers appear to be an effective treatment. Patients whove had an episode of VF are considered at higher risk ande usually treated with an ICD in addition to beta-blockererapy (16,385,410,431). The recurrence of sustained VT,modynamically untolerated VT, or syncope for whichuses other than VT are excluded while the patient isceiving a beta blocker are similarly considered markers ofgher risk (16). In such patients, an ICD is a commonly usedd reasonable approach (16). Furthermore, electrophysio-gical testing is not useful in the management of patientsith catecholaminergic polymorphic VT since the arrhythmiausually not inducible with programmed ventricular stimu-

tion (16,385,410,431). Both supraventricular and ventricu-r arrhythmias are usually reproducibly induced by exerciseress test (16,385,410,431). Isolated premature ventricular
mplexes generally precede runs of nonsustained VT (16). T
ith continued exercise, the runs of VT typically increase inration, and VT may become sustained (16). A beat-to-beat

ternating QRS axis that changes by 180° (“bidirectionalT”) is a typical pattern of catecholaminergic polymorphicT-related arrhythmias (16). Catecholaminergic polymorphicT patients can also present with irregular polymorphic VTVF (16). Beta blockers are generally effective in preventing

currences of syncope even when arrhythmias can still beicited during an exercise stress test (16). If syncope occursa patient taking a beta blocker, implantation of an ICD is

commended (16).VF has been reported in patients with abnormal repolar-

ation due to ion channel mutations that result in a markedlyortened QT interval (432). Only a few small series of suchtients have been described, and at present, evidence-basedcommendations about management of asymptomatic indi-duals with a short QT interval cannot be made. Sometients who survive a clinical episode of VF have noentifiable structural heart disease, no documented transientuse for arrhythmia, and no known ion channel defect. Inch patients, VF is termed “idiopathic.” ICD therapy ispropriate for secondary prevention in patients with theort-QT syndrome and idiopathic VF.

.2.8. Idiopathic Ventricular Tachycardiasonomorphic VT may be seen in individuals with structur-ly normal hearts who have no known ion channelopathies.he most common sites of origin are the RV outflow tract, thescicular region of the LV, structures in the LV outflow tract,d the mitral annular region. The risk for sudden deathlated to these arrhythmias is low (433).

.2.9. Advanced Heart Failure andardiac Transplantationatients with moderate to severe heart failure face the twinsks of terminal heart failure decompensation and death dueunanticipated ventricular tachyarrhythmias. When ICD or

RT-D implantation is discussed with these patients, thekelihood of both life-saving and inappropriate shocksould be placed in the context of the overall anticipatedortality with heart failure, the expected duration of lifeolongation after effective therapies, and the likely evolutionlimiting symptoms and ultimately death due to pump

ilure (434). The relative contribution of preventable suddenath to mortality decreases with repeated hospitalizationsd multiple comorbidities, particularly in the setting ofdney dysfunction or advanced age. These factors, whetherrdiac or noncardiac, also influence the value that patientsace on quality versus length of life remaining. However,dividual preferences cannot be assumed and should beplored with each patient.Candidates for transplantation constitute a special casesevere heart failure because of the likelihood of pro-

nged survival after transplantation, with 50% of patientsrrently surviving at 10 years after transplantation. Thegh rate of sudden death on the transplant waiting listerits ICD implantation in most candidates with heartilure who are awaiting transplantation out of the hospital.
he ICD has been highly effective as a bridge to transplan-

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tion for these individuals both with and without a priorstory of life-threatening arrhythmias.Class IV status itself is a heterogenous and dynamic state35) in which the absolute incidence of sudden deathcreases but the proportion of sudden deaths prevented byDs declines, and heart failure deaths account for a greateroportion of overall mortality. Once patients have persistentfrequently recurrent Class IV symptoms despite optimal

anagement, life expectancy is less than 12 months, and ICDplantation is not indicated, regardless of patient and familyeferences. Occasionally, patients cannot be weaned fromtravenous inotropic infusions and are discharged withronic inotropic infusion therapy for symptom palliation,ith the expectation that death due to heart failure will likelycur within the next 6 months. Despite the proarrhythmictential of inotropic agents, these patients receiving chronicfusions should not be given an ICD (unless awaitingansplantation or other definitive therapy).Often, patients hospitalized with Class IV symptoms willdergo substantial improvement and can be discharged onal therapy with minimal or no symptoms at rest. For thesetients who can remain stable at 1 month after discharge,ithout evidence of recurrent congestion or worsening renalnction, survival is similar to that of other Class III patientsho have not been recently hospitalized. In this situation,D implantation can be discussed and may be expected toprove survival.Patients with Class IV symptoms of heart failure witholonged QRS duration and optimal lead placement mayturn to Class III status or better for both function andrvival, at which point prevention of sudden death againcomes a relevant goal. Information on this group is limitedcause only 10% of the almost 4,000 patients in resynchro-zation trials have had Class IV symptoms. In the COM-ANION trial (224), there were Class IV patients for whomsynchronization improved QOL and reduced rehospitaliza-on and mortality; however, these patients had been stable atme before study entry and may not represent typical Class

patients. Even in this selected group, there was nofference in 2-year survival between CRT patients with andithout the defibrillator feature (230). In patients with Class

symptoms in whom resynchronization is inadequate tostore clinical stability, the presence of a defibrillator oftenmplicates the impending transition to end-of-life care.

ecommendations for Implantable Cardioverterefibrillatorsecondary prevention refers to the prevention of SCD inose patients who have survived a prior cardiac arrest orstained VT. Primary prevention refers to the prevention of

CD in individuals without a history of cardiac arrest orstained VT. Patients with cardiac conditions associatedith a high risk of sudden death who have unexplainedncope that is likely to be due to ventricular arrhythmias arensidered to have a secondary indication.Recommendations for consideration of ICD therapy, par-

cularly those for primary prevention, apply only to patientsho are receiving optimal medical therapy and have a
asonable expectation of survival with a good functional
atus for more than 1 year. It is difficult to estimate survivalith heart failure in the general population, for whommorbidities and age differ from those in trial populations

om which the predictive models have been derived. Patientsith repeated heart failure hospitalizations, particularly in theesence of reduced renal function, are at high risk for earlyath due to heart failure (436–438). See above for discus-

on regarding the use of LVEFs based on trial inclusioniteria.We acknowledge that the “ACC/AHA/ESC 2006 Guide-

nes for Management of Patients With Ventricular Arrhyth-ias and the Prevention of Sudden Cardiac Death” (16) usede LVEF of less than 40% as a critical point to justify ICDplantation for primary prevention of SCD. The LVEF usedclinical trials assessing the ICD for primary prevention of

CD ranged from less than or equal to 40% in MUSTT to lessan or equal to 30% in MADIT II (329,332). Two trials,ADIT I (18) and SCD-HeFT (19) used LVEFs of less thanequal to 35% as entry criteria for the trial. This writing

mmittee reached consensus that it would be best to haveDs offered to patients with clinical profiles as similar toose included in the trials as possible. Having given carefulnsideration to the issues related to LVEF for these updatedD guidelines, we have written these indications for ICDsthe basis of the specific inclusion criteria for LVEF in the

ials. Because of this, there may be some variation fromeviously published guidelines (16).We also acknowledge that the determination of LVEF

cks a “gold standard” and that there may be variationong the commonly used clinical techniques of LVEF

termination. All clinical methods of LVEF determinationck precision and the accuracy of techniques varies amongstboratories and institutions. Based on these considerations,is writing committee recommends that the clinician use theVEF determination that they feel is the most clinicallycurate and appropriate in their institution.

ASS I

ICD therapy is indicated in patients who are survivors ofcardiac arrest due to VF or hemodynamically unstable sus-tained VT after evaluation to define the cause of the event andto exclude any completely reversible causes. (Level of Evi-dence: A) (16,319–324)ICD therapy is indicated in patients with structural heartdisease and spontaneous sustained VT, whether hemodynam-ically stable or unstable. (Level of Evidence: B) (16,319–324)ICD therapy is indicated in patients with syncope of undeter-mined origin with clinically relevant, hemodynamically signifi-cant sustained VT or VF induced at electrophysiological study.(Level of Evidence: B) (16,322)ICD therapy is indicated in patients with LVEF less than orequal to 35% due to prior MI who are at least 40 days post-MIand are in NYHA functional Class II or III. (Level of Evidence: A)(16,333)ICD therapy is indicated in patients with nonischemic DCMwho have an LVEF less than or equal to 35% and who are inNYHA functional Class II or III. (Level of Evidence: B)(16,333,369,379)ICD therapy is indicated in patients with LV dysfunction due to
prior MI who are at least 40 days post-MI, have an LVEF less

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than or equal to 30%, and are in NYHA functional Class I. (Levelof Evidence: A) (16,332)ICD therapy is indicated in patients with nonsustained VT dueto prior MI, LVEF less than or equal to 40%, and inducible VF orsustained VT at electrophysiological study. (Level of Evidence: B)(16,327,329)

ASS IIa

ICD implantation is reasonable for patients with unexplainedsyncope, significant LV dysfunction, and nonischemic DCM.(Level of Evidence: C)ICD implantation is reasonable for patients with sustained VTand normal or near-normal ventricular function. (Level of Evi-dence: C)ICD implantation is reasonable for patients with HCM who have1 or more major† risk factors for SCD. (Level of Evidence: C)ICD implantation is reasonable for the prevention of SCD inpatients with ARVD/C who have 1 or more risk factors for SCD.(Level of Evidence: C)ICD implantation is reasonable to reduce SCD in patientswith long-QT syndrome who are experiencing syncope and/or VT while receiving beta blockers. (Level of Evidence: B)(349–354)ICD implantation is reasonable for nonhospitalized patientsawaiting transplantation. (Level of Evidence: C)ICD implantation is reasonable for patients with Brugadasyndrome who have had syncope. (Level of Evidence: C)ICD implantation is reasonable for patients with Brugadasyndrome who have documented VT that has not resulted incardiac arrest. (Level of Evidence: C)ICD implantation is reasonable for patients with catecholaminer-gic polymorphic VT who have syncope and/or documented sus-tained VT while receiving beta blockers. (Level of Evidence: C). ICD implantation is reasonable for patients with cardiac

sarcoidosis, giant cell myocarditis, or Chagas disease. (Levelof Evidence: C)

ASS IIb

ICD therapy may be considered in patients with nonischemicheart disease who have an LVEF of less than or equal to 35%and who are in NYHA functional Class I. (Level of Evidence: C)ICD therapy may be considered for patients with long-QTsyndrome and risk factors for SCD. (Level of Evidence: B)(16,349–354)ICD therapy may be considered in patients with syncope andadvanced structural heart disease in whom thorough invasive andnoninvasive investigations have failed to define a cause. (Level ofEvidence: C)ICD therapy may be considered in patients with a familialcardiomyopathy associated with sudden death. (Level of Evi-dence: C)ICD therapy may be considered in patients with LV noncompac-tion. (Level of Evidence: C)

ASS III

ICD therapy is not indicated for patients who do not have areasonable expectation of survival with an acceptable func-tional status for at least 1 year, even if they meet ICDimplantation criteria specified in the Class I, IIa, and IIb
recommendations above. (Level of Evidence: C) co
ICD therapy is not indicated for patients with incessant VT orVF. (Level of Evidence: C)ICD therapy is not indicated in patients with significant psychiat-ric illnesses that may be aggravated by device implantation orthat may preclude systematic follow-up. (Level of Evidence: C)ICD therapy is not indicated for NYHA Class IV patients withdrug-refractory congestive heart failure who are not candidatesfor cardiac transplantation or CRT-D. (Level of Evidence: C)ICD therapy is not indicated for syncope of undetermined causein a patient without inducible ventricular tachyarrhythmias andwithout structural heart disease. (Level of Evidence: C)ICD therapy is not indicated when VF or VT is amenable tosurgical or catheter ablation (e.g., atrial arrhythmias associ-ated with the Wolff-Parkinson-White syndrome, RV or LV out-flow tract VT, idiopathic VT, or fascicular VT in the absence ofstructural heart disease). (Level of Evidence: C)ICD therapy is not indicated for patients with ventriculartachyarrhythmias due to a completely reversible disorder in theabsence of structural heart disease (e.g., electrolyte imbal-ance, drugs, or trauma). (Level of Evidence: B) (16)

.3. Implantable Cardioverter-Defibrillators inhildren, Adolescents, and Patients Withongenital Heart Diseasehe indications for ICD implantation in young patients and thoseith congenital heart disease have evolved over the past 15 yearssed on data derived primarily from adult randomized clinical

ials. Similar to adults, ICD indications have evolved from thecondary prevention of SCD to the treatment of patients withstained ventricular arrhythmias to the current use of ICDs forimary prevention in patients with an increased risk of SCD.owever, in contrast to adults, there are minimal prospectiveta regarding ICD survival benefit, because fewer than 1% ofl ICDs are implanted in pediatric or congenital heart diseasetients (439). Considerations such as the cumulative lifetime

sk of SCD in high-risk patients and the need for decades oftiarrhythmic therapy make the ICD an important treatmenttion for young patients.SCD in childhood and adolescence is associated with 3incipal forms of cardiovascular disease: 1) congenital heartsease, 2) cardiomyopathies, and 3) genetic arrhythmia syn-omes (440,441). Prospective identification and treatment ofung patients at risk for sudden death is crucial becausempared with adults, a very low percentage of children under-ing resuscitation survive to hospital discharge (442).The indications for ICD therapy in pediatric patients whove been resuscitated or who are at high risk for SCD are

milar to those for adults. Data from nonrandomized studiesovide support for the Class I recommendation that youngtients who have been resuscitated from SCD should un-rgo ICD implantation after a careful evaluation to excludey potentially reversible causes (440,443–445). Spontane-s sustained VT or unexplained syncope with induciblestained hypotensive VT in patients with congenital heartsease are also considered Class I ICD indications whenher remediable causes (hemodynamic or arrhythmic) haveen excluded (446). Catheter ablation or surgical therapiesay provide an alternative to use of an ICD in patients with
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Recommendations regarding ICD implantation for primaryevention of SCD in young patients are based on limitedinical experience and extrapolation of data from adultudies. No randomized clinical trials have been performed tote, and given the relative infrequency of SCD in youngtients, they are unlikely to be completed in the near future.

ecause the risk of unexpected sudden death is greater inung patients than in adults with genetic diseases such as

CM or the long-QT syndrome, a family history of suddenath, possibly with genetic confirmation, may influence thecision to implant an ICD for primary prevention. Addi-

onal risk factors to be considered in these diseases arescussed in specific sections in this document (354,382,448).With regard to primary prevention of SCD in patients withngenital heart disease, the marked heterogeneity of defectsecludes generalization of risk stratification. Unexpected sud-n death is reported in 1.2% to 3.0% of patients per decade afterrgical treatment of tetralogy of Fallot, with risk factorscluding ventricular dysfunction, QRS duration, and atrial andntricular arrhythmias (249). A significantly greater risk of

CD has been identified for patients with transposition of theeat arteries or aortic stenosis, with most cases presumed to bee to a malignant ventricular arrhythmia associated with

chemia, ventricular dysfunction, or a rapid ventricular responseatrial flutter or fibrillation (449–451).The risk of SCD associated with systemic ventricular dys-nction in congenital heart disease patients remains controver-al (452,453). The ability to define the risk associated withpaired function is complicated by the fact that right (pulmo-ry) ventricular dysfunction is more common than left (sys-mic) ventricular dysfunction and that a variety of atrial arrhyth-ias and conduction blocks may independently predispose thesetients to arrhythmias or syncope. The lack of prospective andndomized clinical trials precludes exact recommendationsgarding risk stratification and indications for ICD implantationr primary prevention of SCD in patients with postoperativengenital heart disease and ventricular dysfunction. One othertential ICD indication in young patients, which is similar toults, is the patient with congenital coronary anomalies orronary aneurysms or stenoses after Kawasaki disease, inhich an ischemic substrate for malignant arrhythmias may beesent (441).Because of concern about drug-induced proarrhythmia andyocardial depression, an ICD (with or without CRT) may beeferable to antiarrhythmic drugs in young patients with DCMother causes of impaired ventricular function who experiencencope or sustained ventricular arrhythmias. ICDs may also bensidered as a bridge to orthotopic heart transplantation indiatric patients, particularly given the longer times to donorocurement in younger patients (454,455).

ecommendations for Implantableardioverter-Defibrillators in Pediatric Patientsd Patients With Congenital Heart Disease

ASS I

ICD implantation is indicated in the survivor of cardiac arrestafter evaluation to define the cause of the event and to exclude
any reversible causes. (Level of Evidence: B) (440,443–445) lo
ICD implantation is indicated for patients with symptomaticsustained VT in association with congenital heart disease whohave undergone hemodynamic and electrophysiological evalu-ation. Catheter ablation or surgical repair may offer possiblealternatives in carefully selected patients. (Level of Evidence: C)(447)

ASS IIa

ICD implantation is reasonable for patients with congenitalheart disease with recurrent syncope of undetermined origin inthe presence of either ventricular dysfunction or inducibleventricular arrhythmias at electrophysiological study. (Level ofEvidence: B) (18,446)

ASS IIb

ICD implantation may be considered for patients with recurrentsyncope associated with complex congenital heart diseaseand advanced systemic ventricular dysfunction when thoroughinvasive and noninvasive investigations have failed to define acause. (Level of Evidence: C) (451,454)

ASS III

All Class III recommendations found in Section 3, “Indicationsfor Implantable Cardioverter-Defibrillator Therapy,” apply topediatric patients and patients with congenital heart disease,and ICD implantation is not indicated in these patient popula-tions. (Level of Evidence: C)

.3.1. Hypertrophic Cardiomyopathyrior studies of ICD therapy for primary and secondaryevention of SCD in HCM are discussed in Section 3.1.5 and

ection 3.2.4; most of these studies have included bothdiatric and adult patients. The indications for ICDs indiatric patients with HCM for primary and secondaryevention of sudden cardiac arrest are the same as those forults. Clinical decisions should be based on risks andnefits that may be unique to pediatric patients. In thediatric population, recommendations for ICD therapyould be made with careful consideration of the risks ofvice implantation, which may be increased on the basis ofdy size. Additionally, consideration should be given to theditional years of benefit that could potentially result fromevention of SCD in this population.

.4. Limitations and Other Considerations

.4.1. Impact on Quality of Lifenappropriate Shocks)espite its life-saving potential, the use of ICD therapyrries a risk for psychological consequences and may lead todecrement in QOL, particularly among patients who haveperienced shocks (456). Reports of significant behavioralsorders, including anxiety, device dependence, or socialithdrawal, have been described with ICD implantation57–459). However, QOL substudies from large, random-ed clinical trials of ICD therapy demonstrated that overall,OL was no different or was somewhat better among patientsndomized to ICD therapy than among those in the controloups, with decreases in physical, emotional, and psycho-
gical measures of health-related QOL concentrated among

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tients who experienced ICD shocks (328,367,460). Givene broader indication for and marked increase in implanta-on of ICDs for primary prevention that is being driven bye results of the SCD-HeFT and MADIT II trials (332,333),derstanding the frequency and causes of inappropriateocks and devising management strategies to mitigate bothappropriate therapies and their psychological and QOLnsequences will be important for an increasingly largegment of the population.A systematic review summarized the frequency of inap-opriate ICD therapies reported in randomized clinical trialsprimary and secondary prevention (461). In these trials,ring follow-up that ranged from 20 to 45 months, inappro-iate ICD therapy was delivered in 10% to 24% of patients.

the PainFREE Rx II (Pacing Fast VT Reduces Shockherapies II) trial, in which patients were randomized tother ATP or shocks as first therapy for fast VT, at least 1appropriate detection occurred in 15% of patients duringproximately 11 months of follow-up (294). The proportiondetections that were inappropriate was modestly but not

gnificantly higher among primary prevention patients thanong secondary prevention patients (46% versus 34%;0.09). Both older and more recent registry reports suggest

milar rates of inappropriate therapy in unselected popula-ons (462,463).By far, the leading cause of inappropriate therapy is the misclas-

fication of SVT, most commonly AF (294,358,462,463). But ICDad malfunction and other causes, such as oversensing of T waves,uble counting of prolonged QRS, and electromagnetic interfer-ce, may account for 4% to 30% of inappropriate therapy05,367,462,464). Patients with multiple ICD shocks should bealuated immediately to determine the cause of the shocksd to direct urgent management. Short-term therapy withxiolytic drugs may be instituted early for patients aftercurrent device firings to minimize acute and delayed anxi-y reactions.A variety of approaches to reduce the occurrence of

appropriate shocks are currently available, and selectionpends on the cause of the shocks and the type of deviceplanted. Although there has been debate as to the utility ofal-chamber versus single-chamber devices in reducingtes of inappropriate ICD therapy, a recently publishedndomized trial suggests that optimal programming of dual-amber devices can reduce the rate of inappropriate detec-

ons and therapies due to SVTs (465). In the multicenteretect Supraventricular Tachycardia Study, 400 patients withclinical indication for an ICD received dual-chambervices and were randomized in a single-blind fashion totimal single- or dual-chamber detection programming.

VT occurred in 34% of subjects (31% in the single-chamberm and 37% in the dual-chamber arm). Rates of inappropri-e detection of SVT were substantial in both arms (39.5% ine single-chamber arm and 30.9% in the dual-chamber arm),t the adjusted odds ratio of inappropriate detection of SVTthe dual-chamber arm compared with the single-chamber

m was 0.53 (95% CI 0.30 to 0.94; p�0.03). This reductioninappropriate detection translated to a similar reduction in

appropriate therapy, with no compromise of VT detection,
hich makes this trial the first to show superiority of du
al-chamber devices when optimally programmed. Othereas of active research include the development of enhancedathematically modeled detection protocols for evaluation ofternal electrograms to improve discrimination of SVT fromT and to increase the ability to detect lead failures (466–8). Regardless of the cause of or solution for inappropriateD therapy (particularly shocks), careful attention to a

am-based approach that includes the patient and family inotional and psychological support is also recommended

56,469).

.4.2. Surgical Needsurgically placed epicardial pacing leads are indicated inlected instances when standard transvenous lead place-ent is either not feasible or contraindicated. Examples ofch circumstances include: 1) inability or failure to place

adequate LV lead in patients requiring biventricularcing, 2) indications for permanent pacing in certain pedi-

ric patients and in pediatric or adult patients with tricuspidlve prostheses or recurrent or prolonged bacteremia, andcongenital acquired venous anomalies that preclude trans-nous access to the heart.The reported success rate of coronary venous lead implan-

tion for biventricular pacing ranges from 81% to 99%70,471). Causes of failed percutaneous lead placement mayanatomic (superior vena cava or coronary sinus obstruc-

on or inadequate coronary venous anatomy) or technicalailure to cannulate the coronary sinus, coronary sinusssection, inadequately high pacing thresholds with intermit-nt capture, diaphragmatic pacing due to proximity of therenic nerve to the target coronary sinus branch, or leadslodgement) (470,472,473). When coronary sinus lead im-antation fails, several nonrandomized studies have demon-rated that surgical LV lead placement is almost alwaysccessful (470–473). In this setting, the key advantage ofrgical lead placement is access to the entire posterior andteral walls of the LV, which enables the choice of the bestcing site (471,474). The combination of echocardiographyith tissue Doppler imaging and electrophysiological mea-rements may facilitate the choice of a transthoracicallyrected LV epicardial pacing site (473). Implantation of 2icardial leads may be considered to provide backup capa-lity if 1 lead should fail or become dislodged (475).teroid-eluting epicardial leads may be preferable torew-on leads (473).The choice of surgical procedure appears to influencespital morbidity. Surgical approaches for placement of LVicardial leads include left thoracotomy, left thoracoscopy,d robotically assisted port-based placement. Thoracotomyfragile patients with heart failure has been associated with

eeding, stroke, hypotension, and arrhythmias (470,476). Inntrast, thoracoscopic and robotic approaches have beenported to be associated with minimal morbidity and may beeferred (472,473,475). These less invasive procedures gen-ally require 60 to 90 minutes of operative time and a meanspital stay of 4 to 5 days (472). However, not all patientse candidates for minimally invasive or robotic proce-
res. Subjects who have undergone prior thoracotomy or

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ernotomy operations may have limited pericardial/epicardialcessibility.In certain instances, it may be advisable to place an LVicardial lead at the time of concomitant cardiac surgery. Intients who are currently or in the future may be candidatesr CRT who require coronary artery bypass grafting oritral valve surgery and have medically refractory, symp-matic heart failure, ischemic cardiomyopathy or DCM,olonged QRS interval, LV end-diastolic diameter greateran or equal to 55 mm, and LVEF less than or equal to 35%,e surgeon may elect to place an LV epicardial lead (477).he lead is tunneled to a prepectoral pocket for intraoperative

postoperative attachment to an appropriate pacing gener-or. This approach is probably not indicated for the patientho is expected to have substantial improvement in LVEFter cardiac surgery (e.g., the patient with extensive viableyocardium who is undergoing revascularization). There aremited data documenting outcomes of this “preemptive”rategy.Epicardial leads may be necessary in some pediatrictients. The most common indications for permanent pace-aker implantation in the pediatric population are SND orV block after surgery for congenital heart disease andngenital AV block (478). In most instances, such pacingstems can be placed by standard transvenous techniques79). However, epicardial leads may be needed in childrena result of their small size, the presence of congenital heartfects with a right-to-left shunt, or an inability to pace theamber desired because of anatomic barriers (e.g., after a

ontan procedure) (478–480). In such instances, steroid-uting leads provide excellent durability (479).Epicardial leads are suggested in some pediatric or adulttients who need pacing and who have recurrent or pro-nged bacteremia (481). For a single episode of device-lated bacteremia, extraction of all hardware followed byimplantation by the transvenous route at a later date ispropriate.Implantation of permanent epicardial pacing leads is indi-ted in the pacemaker-dependent patient undergoing me-anical tricuspid valve replacement. A prosthetic mechani-l tricuspid valve represents an absolute contraindication toacement of transvenous RV leads, because such leads willoss the valve and may interfere with valve function. Thisenario occurs commonly in patients with tricuspid valvedocarditis and a transvenous pacemaker. At surgery, allrdware should be removed. If the tricuspid valve is repair-le, standard transvenous pacing leads can be placed post-eratively. However, if tricuspid valve replacement is nec-sary, epicardial ventricular leads should be implanted at the

me of surgery.

.4.3. Patient Longevity and Comorbiditieshysicians, patients, and their families increasingly will beced with decisions about device-based therapies (ICD andRT) in elderly patients who meet conventional criteria forplantation. These decisions require not only evidence of

inical benefit demonstrated in randomized clinical trials butso estimates of life expectancy, consideration of comorbidi-
es and procedural risk, and patient preferences. Although 5.
ese factors are important when device implantation isnsidered in any age group, they assume greater weight ininical decision-making among the elderly.Unfortunately, few clinical trials of device-based therapyve enrolled enough elderly patients (age greater than 75ars) to reliably estimate the benefits of device-based ther-y in this group. Indeed, patients in device trials havenerally had an average age less than 65 years and littlemorbidity. In contrast, the average patient hospitalized withart failure and low LVEF is 75 years old with 2 comor-dities. The 1-year mortality rate for this population is in thenge of 30% to 50%, with a 2-fold higher risk of death intients with estimated creatinine clearance less than 60 mlr minute (326,482). The presence of chronic pulmonarysease and dementia further increases the risk for death.ewer than 10% of deaths in this population could betributed to presumed SCD in patients living independently82). After 3 hospitalizations for heart failure in a commu-ty population, median survival declines to 1 year and would

prolonged by only 0.3 years even if all presumed SCDsere prevented (5). For all patients, the likelihood of mean-gful prolongation of life by prevention of SCD must besessed against the background of other factors that limittient function and survival.Among 204 elderly patients with prior MI and LVEF less

an or equal to 30% enrolled in MADIT II (total n�1,223),trial of primary prevention of SCD with ICD therapy, theR for mortality with ICD therapy was 0.56 (95% CI 0.29 to08; p�0.08), which was similar to that for younger patientsR 0.63, 95% CI 0.45 to 0.88; p�0.01) (482a). Furthermore,

OL scores were similar among older and younger patients.ubgroup analyses by age (less than or equal to 65 versuseater than 65 years) from COMPANION and SCD-HeFTowed some erosion of benefit among the older group, butere were no significant treatment interactions with age24,333).In a study of 107 consecutive patients greater than 80 years

d (82% with ischemic cardiomyopathy) and 241 consecu-ve patients 60 to 70 years of age (80% with ischemicrdiomyopathy), life expectancy after device implantationredominantly ICD alone) among the octogenarians was 4.2ars compared with 7 years among those 60 to 70 years old83). Thus, although survival after implantation is shorterong the elderly than among younger groups, survival is

bstantial, and age itself should not be the predominantnsideration in the use of device-based therapy among thederly.The presence and number of noncardiac comorbidities areother important consideration in the decision to proceedith device-based therapy in the elderly. In one registry,though age greater than 75 years and heart failure wereportant predictors of death at 1 and 2 years of follow-up,

ter adjustment for age, heart failure, and patient sex, thember of noncardiac comorbidities was statistically signif-

antly associated with survival among 2,467 patients whoceived ICD therapy (484). The presence of 3 or morencardiac comorbidities was associated with a nearly 3-foldcrease in the hazard for mortality (HR 2.98, 95% CI 1.74 to
10). Therefore, as much as age, the presence and number of

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ncardiac comorbidities are critical considerations in thecision to use device-based therapy.A meta-analysis of secondary prevention trials (AVID,

ASH, and CIDS) revealed that although ICD therapyduced all-cause and arrhythmic death among patients lessan 75 years old, among 252 patients older than 75 years, theR for all-cause mortality (predominantly due to progressiveart failure) was 1.06 (95% CI 0.69 to 1.64; p�0.79), andr arrhythmic death, it was 0.90 (95% CI 0.42 to 1.95;0.79) (485). The interaction p value was 0.09, which

ggests that the elderly may derive less benefit from ICDerapy in secondary prevention than younger patients.In summary, these data suggest that although age is anportant predictor of outcome after ICD therapy, meanrvival of more than 4 years may be expected even amongtogenarians, and age alone should not be used as a soleiterion to withhold device-based therapy. However, impor-nt considerations in the decision to use device-based ther-y should include the indication for device implantation (forDs, primary versus secondary prevention), the number ofmorbidities, and patient preferences.Considerations specific to elderly patients are also relevantpacing, CRT, and ICD therapies. Similar to enrollment inD trials, few patients older than 75 years have beenrolled in trials of CRT. However, subgroup analyses from

ARE-HF (age less than 66.4 versus greater than or equal to.4 years) and COMPANION (age less than or equal to 65rsus greater than 65 years) suggest that older patientsrive similar benefit from CRT as younger patients24,225).The “ACC/AHA/ESC 2006 Guidelines for Management of

atients With Ventricular Arrhythmias and the Prevention ofudden Cardiac Death” addressed ICD implantation in thederly (16). Many of those considerations are relevant toher types of device implantation. Because of underrepre-ntation of the elderly in clinical trials, much of the rationaler implanting devices in these patients rests on subgroupalyses that were not prespecified and is therefore relativelyeak. Furthermore, not only relative efficacy but also proce-ral complication rates in older versus younger patients are

rgely unexplored. These unknowns must be balancedainst the fact that many elderly patients remain functionaltil shortly before death and reasonably deserve similar

eatment options as younger patients in many cases. Thehical principles of autonomy, beneficence (“do good andoid evil”), and nonmaleficence (“do no harm”) must alwaysevail.

.4.4. Terminal Carethe United States, the withholding and withdrawal of

fe-sustaining treatments (e.g., cardiopulmonary resuscita-on, mechanical ventilation, or hemodialysis) from termi-lly ill patients who do not want the treatments is ethical andgal (486). Honoring these requests is an integral aspect oftient-centered care and should not be regarded asysician-assisted suicide or euthanasia.When terminally ill patients (or their surrogates) requestcemaker, ICD, or CRT deactivation, questions related to
e ethics of device deactivation may arise. Questions com-
only asked include: Are implantable devices life-sustainingeatments? Is deactivation the same as physician-assistedicide or euthanasia? Is deactivation ethical? Is it legal?nder what conditions (e.g., code status) should deactivation

performed? Who should carry out deactivation? Whatcumentation should exist?The prevalence of implantable devices in patients dying ofncardiac diseases makes this an increasingly encountered

inical issue. Patients and families fear that devices willolong the dying process, and some dying patients withDs fear uncomfortable defibrillations. In fact, investigatorsve found that some patients with ICDs experience uncom-rtable defibrillations throughout the dying process, includ-g moments before death. Cardiologists who implant devices

not commonly have discussions with patients aboutd-of-life issues and device deactivation. Furthermore, pub-

shed experience with deactivation of devices is limited87).There is general consensus regarding the ethical and legalrmissibility of deactivating ICDs in dying patients whoquest deactivation (488). However, caregivers involved invice management generally make a distinction betweenactivating a pacemaker and deactivating an ICD or CRTvice. Given the clinical context, all 3 can be considered

fe-sustaining treatments. Notably, all of these devices mayrefused by patients, and to impose them on patients who dot want them is unethical and illegal (battery). Furthermore,

hics and law make no distinction between withholding andithdrawing treatments.An approach to dying patients who request pacemaker,D, or CRT deactivation should include the following:

A dying patient (or, if the patient lacks decision-makingcapacity, the patient’s surrogate decision maker) whorequests device deactivation should be fully informed ofthe consequences and alternatives to device deactivation,and a summary of the conversation should be recorded inthe medical record.An order for device deactivation should be accompaniedby a do-not-resuscitate (DNR) order; these orders shouldbe recorded in the patient’s medical record.Psychiatric consultation should be sought in any situationin which a dying patient who requests device deactivationis thought to have impaired decision-making capacity.Ethics consultation should be sought in any situation inwhich the clinician or clinicians disagree, based on theirclinical judgment, with a request for device deactivation.If the clinician asked to deactivate a device has personalbeliefs that prohibit him or her from carrying out devicedeactivation (conscientious objection), then the patientshould be referred to another clinician.If the patient is remote from the implanting medical center,the clinician who is responsible for the patient’s care at thelocal site should document the information noted above inthe medical record, and someone capable of programmingthe device to “inactive” status should be recruited toreprogram the device under the direction of the local
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Clinicians involved in device education at the time ofplantation may need to provide more comprehensive in-rmation with regard to end-of-life issues. For example,inicians should encourage patients undergoing device im-antation to complete advanced directives and specificallydress the matter of device management and deactivation ife patient is terminally ill.

.5. Cost-Effectiveness of Implantableardioverter-Defibrillator Therapyong-term follow-up studies have consistently demonstratedat cumulative medical costs are increased substantiallyong patients receiving an ICD (17–19,489–491). Several

udies have attempted to weigh whether these added costse worthwhile in light of the potential for improved survivalong patients receiving ICD therapy (492). These studies

lculate a cost-effectiveness ratio that is defined as thefference in the total cost of patients receiving an ICD andtients receiving alternative therapy, divided by the addi-

onal life-years of survival provided by an ICD comparedith alternative therapy. A benchmark for comparison isovided by renal dialysis, which costs approximately0,000 to add 1 life-year of survival. Cost-effectiveness, likeher outcome measures in clinical research studies, must beterpreted in light of the characteristics of the study popu-tions and the length of follow-up available.The early studies of ICD cost-effectiveness were based onathematical models and relied on nonrandomized studies totimate clinical efficacy and cost. These studies foundst-effectiveness ratios of $17,000 (493), $18,100 (494), and9,200 per year of life saved (495). Another model incor-rated costs of nonthoracotomy ICDs and efficacytimates based on randomized trials and found ICDst-effectiveness was between $27,300 and $54,000 per

fe-year gained, which corresponded to risk reductions of% and 20%, respectively (496).Several randomized clinical trials have measured both costd clinical outcomes and thus can directly estimate ICDst-effectiveness. MADIT found a 54% reduction in totalortality and a cost-effectiveness ratio of $27,000 per life-ar added (18). In contrast, CIDS found a 20% reduction intal mortality and a cost-effectiveness ratio of $139,000 perfe-year added (322,490). The cost-effectiveness ratio frome AVID trial was $66,677 per life-year added (491).ADIT II found a 32% reduction in total mortality and9,200 higher costs among ICD-assigned patients thanong those treated with conventional therapy (17). The

st-effectiveness ratio in MADIT II was measured as35,000 per year of life added at 2 years of follow-up but

as projected to be between $78,600 and $114,000 per yearlife added by 12 years of follow-up. SCD-HeFT reported

at total mortality was reduced by 23% and costs increased$19,000 over 5 years of follow-up in patients assigned to

Ds compared with patients assigned to placebo (19).CD-HeFT estimated the lifetime cost-effectiveness ratio ofe ICD strategy was $38,400 per year of life added. Thisnge of results from randomized studies is primarily due tofferent estimates of the effectiveness of the ICD in reducing
ortality, because all showed similar increases in the cost of pr
re among ICD recipients. When the results of all clinicalials were used in a model that used a consistent frameworkproject the full gain in life expectancy and lifetime costs inch trial (497), the cost-effectiveness of the ICD ranged from5,300 to $50,700 per life-year added in the randomized

ials in which the ICD reduced mortality. In the CABG-Patchial and DINAMIT, however, patients assigned to an ICDd lower survival and higher costs than patients assigned tonventional therapy, and the ICD strategy was not cost-

fective. The evidence suggests that proper patient selectionnecessary for ICD implantation to be cost-effective; whenD implantation is restricted to appropriately selected pa-

ents, it has a cost-effectiveness ratio similar to other ac-pted cardiovascular therapies and compares well to theandard benchmark of renal dialysis ($30,000 to $50,000 perar of life saved). In principle, ICD implantation will beore cost-effective when used for patients at high risk ofrhythmic death and at low risk of other causes of death.dditional risk stratification of patients with a reducedVEF may improve patient selection for the ICD andereby enhance its cost-effectiveness (498). Cost-fectiveness of the ICD would also be improved bywering the cost of the device itself and further improvings reliability and longevity.The cost-effectiveness of CRT has not been evaluatedtensively. A CRT-P device reduces hospitalization for heartilure patients, and these cost savings partially offset theitial cost of device implantation. CRT-P devices are alsofective in improving QOL and may improve survival. Thest-effectiveness of CRT-P devices versus medical therapypears to be favorable. There are few data on the cost-

fectiveness of CRT-D compared with CRT-P devices.

.6. Selection of Implantableardioverter-Defibrillator Generatorssingle RV lead for sensing and defibrillation is mandatoryr all currently available ICD systems. Single-chamber ICDstems are capable of bradycardia support in the ventricled ATP. Dual-chamber ICD systems (right atrial and RVads) are additionally capable of AV sequential pacing.riple-chamber ICD systems (right atrial, RV, and LV leads)e capable of CRT (CRT-D). Despite these increasingmplexities, the optimal hardware system for ICD indica-

ons derived from mortality studies has not been fullyaluated. There is increasing evidence that choice of hard-are may affect important outcomes in ICD patients. Thislates primarily to 2 considerations: 1) management ofntricular pacing and 2) pain associated with high-voltageocks. Conventional ICD therapy in any form may besociated with worsening heart failure, VT, VF, and noncar-ac death that can be related to the adverse effects of RVAcing (50,51). This is consistent with the increased risks ofF and heart failure attributable to RVA pacing in pacemakerials (45,48). The issue of QOL in the ICD patient populations been evaluated extensively (460,499–502). AlthoughD therapy is generally well tolerated by most patients,proximately 30% to 50% experience some degree ofychological distress after implantation (503). One of the
incipal limitations of ICD therapy is the discomfort asso-

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ated with high-voltage shocks. Several studies have noted arect correlation between poor QOL scores and the experi-ce of ICD shocks (460,499–501).Any hardware system that increases unnecessary ventric-ar pacing from any site may increase the risk of heartilure, particularly in patients with poor cardiac ventricularstolic function (293). The risk of heart failure is increaseden in hearts with initially normal ventricular systolicnction and with part-time ventricular pacing. RVA pacingeates abnormal contraction, reduced ventricular systolicnction, hypertrophy, and ultrastructural abnormalities. Theagnitude of the effect relates to the frequency of ventricularcing and the degree of pacing-induced mechanical dyssyn-rony rather than the hardware system (49). Although these

fects have been demonstrated most clearly during RVAcing, biventricular or LV pacing may also induce dyssyn-rony in hearts with normal ventricular conduction (504)d can reduce LV systolic function in patients with noseline dyssynchrony (505).In patients with no AV block and no intraventricularnduction abnormalities, ventricular pacing should beoided as much as possible. For many ICD patients who dot have an indication for bradycardia support, this can behieved by programming a very low backup ventricularcing rate (i.e., 30 to 40 bpm). The optimal management ofrdiac pacing in ICD patients in whom bradycardia supportrequired, desired, or emerges is unknown. For ICD patientsith SND in whom bradycardia support is required orsired, ventricular pacing may be minimized by use ofwer techniques specifically designed to promote intrinsicnduction (292,506). In patients with AV block, alternate

ngle-site RV or LV pacing or biventricular pacing (CRT-/CRT-D) may be superior to RVA pacing. Efforts totimize pacing mode or site should be greater in patients

ith longer expected duration of pacing, poorer cardiacnction, and larger mechanical asynchrony. Awareness ofe problem of dyssynchrony should also lead to more regularonitoring of cardiac ventricular systolic function and me-anical asynchrony in any patient with ventricular pacing.ATP refers to the use of pacing stimulation techniques for

rmination of tachyarrhythmias. Tachycardias that requireentry to persist are susceptible to termination with pacing.he most common mechanism of VT in ICD patients isar-related reentry. The sine qua non of a re-entrant arrhyth-ia is the ability to reproducibly initiate and terminate thechycardia by critically timed extrastimuli (124). Therefore,e possibility of successful termination of tachycardias withcing can be anticipated on the basis of the mechanism.

uch techniques can be applied automatically with ICDs andfer the potential for painless termination of VT.Adjudicated analysis of stored electrograms has revealedat the majority (approximately 85% to 90%) of spontaneousntricular tachyarrhythmias in ICD patients are due to VTd fast VT, whereas only approximately 10% are due to VF07,508). Numerous older studies have consistently demon-rated that ATP can reliably terminate approximately 85% to% of slow VTs (cycle lengths less than 300 milliseconds to0 milliseconds) with a low risk of acceleration (1% to 5%)
09–511). More recently, similarly high rates of success and by
w acceleration and syncope rates for fast VTs (averagecle length 240 milliseconds to 320 milliseconds) have beenmonstrated (507,508). These observations have reposi-

oned the ICD as primarily an ATP device with defibrillationckup only as needed. Reduction in painful shocks mayprove patient QOL (508) and extend ICD pulse-generator

ngevity. It is not yet clear whether important differences intimal application of ATP exist in different ICD patientpulations. In general, secondary prevention patients have aeater frequency of spontaneous ventricular arrhythmia thanimary prevention patients. However, differences in thecidence of specific ventricular rhythms (VT, fast VT, andF), response to therapy (ATP or shocks), and susceptibilityspurious therapies due to SVT are incompletely character-

ed (294,512). Differences in substrate may be important asell. Monomorphic VT associated with chronic ischemicart disease is most commonly due to classic reentry and iserefore susceptible to termination by ATP. MonomorphicT is less commonly due to reentry and occurs with lowerequency in nonischemic DCM.

.7. Implantable Cardioverter-Defibrillatorollow-Upll patients with ICDs require periodic and meticulousllow-up to ensure safety and optimal device performance,well as to monitor a patient’s clinical status (513). The

als of ICD follow-up include monitoring of device systemnction; optimization of performance for maximal clinicalfectiveness and system longevity; minimization of compli-tions; anticipation of replacement of system componentsd tracking devices under advisory; ensuring timely inter-ntion for clinical problems; patient tracking, education, andpport; and maintenance of ICD system records. The impor-nce of device surveillance and management should bescussed with patients before ICD implantation. Complianceith device follow-up is an important element in the evalu-ion of appropriate candidates for device therapy and totain the best long-term result.ICD follow-up is best achieved in an organized programalogous to pacemaker follow-up at outpatient clinics12). Physicians and institutions performing implantationthese devices should maintain follow-up facilities for

patient and outpatient use. Such facilities should obtaind maintain implantation and follow-up support devicesr all ICDs used at that facility. The facility should beaffed or supported by a cardiologist and/or electrophysi-ogist, who may work in conjunction with trained associatedofessionals (312,514,515). Continuous access to these ser-ces should be available as much as feasible on both agularly scheduled and more emergent basis. The implanta-on and/or follow-up facility should be able to locate andack patients who have received ICDs or who have enterede follow-up program.

.7.1. Elements of Implantableardioverter-Defibrillator Follow-Uphe follow-up of an ICD patient must be individualizedaccordance with the patient’s clinical status and conducted
a physician fully trained in ICD follow-up(12); if this is

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t a physician fully trained in all aspects of ICD implanta-on and follow-up, then such an individual should be avail-le for any problems that may develop. Direct patientntact is ideal, allowing for interval history taking, physicalamination of the implantation site, and device program-ing changes that may be warranted. Six-month intervals forvice follow-up appear to be safe (516), but more frequentaluations may be required depending on the device char-teristics and the patient’s clinical status. Manufacturers’idelines for device follow-up may vary with individualodels and should be available. Device automaticity hascilitated follow-up (316), as has the implementation ofmote monitoring techniques (513,517). Depending on theanufacturer, remote device interrogation is achievedrough Internet-based systems or via radiofrequency trans-issions from the ICD via a phone device to a centralonitoring center; remote reprogramming of devices is notailable currently. Remote monitoring may lessen the de-ndence on clinic visits, particularly in patients who live atconsiderable distance from the follow-up clinic, and maylow for the earlier detection of real or potential problemssociated with the device. Guidelines for remote monitoringve yet to be established. It should be recognized, further-ore, that remote monitoring is an adjunct to follow-up andnnot entirely supplant clinic visits (518,519).In general, device programming is initiated at implantationd may be reviewed periodically. It is often necessary toprogram the initially selected parameters either in thetpatient clinic or during electrophysiological testing. Whenvice function or concomitant antiarrhythmic therapy isodified, electrophysiological testing may be warranted toaluate sensing, pacing, or defibrillation functions of thevice. Particular attention should be given to review ofnsing parameters, programmed defibrillation and pacingerapies, device activation, and event logs. Technical ele-ents that require review include battery status, leadstem parameters, and elective replacement indicators.tervening evaluation of device function is often neces-ry. In general, when ICD therapy is delivered, the deviceould be interrogated.After implantation of a device, its performance should be

viewed, limitations on the patient’s specific physical activ-ies established, and registration accomplished. Current pol-ies on driving advise patients with an ICD implanted forcondary prevention to avoid operating a motor vehicle formonths after the last arrhythmic event if it was associatedith loss or near loss of consciousness to determine thettern of recurrent VT/VF (520,521). For patients with ICDsplanted for primary prevention, avoidance of driving for at

ast 7 days to allow healing has been recommended (522).teractions with electromagnetic interference sources poten-

ally affect employment. Sports involvement(523) and rec-mendations regarding safeguards for future surgical pro-

dures (524) should be discussed. There are currently notough data to make recommendations regarding antibioticophylaxis for procedures or operations required in the firstmonths after ICD implantation; physicians must weigh thesks and benefits of antibiotic prophylaxis and use their
dgment in each case. ICD recipients should be encouraged de
carry proper identification and information about theirvice at all times. Patients receiving these devices canperience transient or sustained device-related anxiety. Ed-ation and psychological support before, during, and afterD insertion are highly desirable and can improve thetient’s QOL (457,458).Increasing attention has been paid to the safety and efficacyimplantable devices. It is incumbent upon the follow-upysician to be aware of advisories issued in relation totential device malfunction (2). Specific recommendationsr clinicians managing such advisories are to considerad/device replacement if death is a likely result of devicealfunction; the mechanism of device/lead failure is known,tentially recurrent, and possibly life-threatening; the patientpacemaker-dependent; the risk of replacement is substan-

ally lower than the risk of device malfunction; or the deviceapproaching its elective replacement indicator (3). Com-

ications related to replacement of ICD generators undervisory have been well documented, including infection, theed for reoperation, and death (525). The estimated deviceilure rate and the likelihood of mortality resulting fromvice failure must be weighed against the risk of proceduralorbidity and mortality associated with device replacement.general, for pacemaker-dependent patients, advisory de-

ce failure rates in excess of 0.3% warrant consideration ofvice replacement; in patients with ICD generators undervisory, an estimated failure rate of 3% favors replacementthe majority of cases, decreasing to 1% when procedural

ortality rates are 0.1% or less and/or risk of fatal arrhyth-ias increases to 20% per year (526). It is anticipated that theove general recommendations and estimates will vary as anction of the specific nature of the advisory, how thealfunction presents, whether early detection and/or repro-amming may be employed in addressing the potentialvice failure, and whether the lead (versus the generator) is

fected. This has been demonstrated, for example, in the casea recent lead advisory associated with spurious shocks

tributable to lead fracture, oversensing, and high imped-ce; reprogramming to minimize overdetection of noise,abling of alert features to detect changes in impedance, andcreasing utilization of remote monitoring to follow suchads may have an effect on future rates of invasive leadplacement and/or extraction (527).

.7.2. Focus on Heart Failure After First Appropriateplantable Cardioverter-Defibrillator Therapypatients with heart failure who have not previously had a

fe-threatening arrhythmia, the first event identifies them asing at higher risk than before for both sudden death andath due to heart failure, with the majority of patientsrviving less than 2 years (17,19). It is not known to whattent these herald events serve as markers or as contributorsprogression of disease. They should trigger reevaluation of

eatable causes of heart failure and of the medical regimen.addition, the treatment regimen should be evaluated for

terventions that may decrease the risk of arrhythmia recur-nce. Particular care should be paid to the titration ofta-adrenergic blockers. These agents have been shown to
crease disease progression and improve outcomes, but

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titration can lead to heart failure exacerbation and must betempted gradually in small dose increments. Many patientsith symptomatic heart failure cannot tolerate “target doses”

beta-adrenergic blockers, whether used primarily for thedication of heart failure or to prevent recurrent arrhythmias.lthough patients with heart failure who have had deviceerapy would ideally be followed up by specialists in bothrhythmia management and heart failure management, mosttients do not have routine access to such settings. Toaximize the benefit after a sudden death has been prevented,is crucial that the management team evaluate the heart

ilure profile, review the medical regimen, and plan forgoing care.

4. Areas in Need of Further Research

he ACC/AHA Task Force on Practice Guidelines hasarged writing committees to suggest areas in need ofrther research. To this end, the present writing committeefers the following suggestions. They are presented inbular form for ease of readability. Their order does notply any order of priority.

Optimal access to device therapy should be provided to alleligible populations irrespective of sex and ethnicity.Risk stratification of patients meeting current clinicalindications for primary prevention ICD implantationshould be improved to better target therapy to those mostlikely to benefit from it.Identification of patients most likely to benefit from/respond to CRT must be improved.Identify patients without current pacemaker or ICD indi-cations among those who may benefit from such therapies.Indicators should be identified that provide direction aboutwhen it is safe to not replace an ICD that has reached theend of its effective battery life.The cost-effectiveness of device therapy should be ex-plored furtherGuidelines for remote monitoring should be developedWays to improve reliability and longevity of leads andgenerators must be found, as well as methods to ensurediscovery of performance issues when they arise.Representation of the elderly in clinical trials should beincreased. The influence of age on procedural complication rates

and the risk/benefit ratio for device implantation shouldbe defined.

. The effect (positive, negative, or neutral) of biventricularor LV stimulation in patients with normal ventricularfunction should be determined.

. The need for pacing after MI in the current era should bedetermined

. Long-term outcomes and risk factors for patients receiv-ing ICDs in general practice compared with trial popu-lations and at academic centers should be identified anddescribed.

. Guidelines for device management in patients with ter-minal illness or other requests to terminate device ther-
apy should be developed.
. The role of ICDs in primary prevention for children withgenetic channelopathies, cardiomyopathies, and congen-ital heart defects should be defined more precisely.

. The efficacy of biventricular pacing in children withcongenital heart disease and dilated cardiomyopathyshould be determined. Appendix 3

2012 Presidents and Staff

merican College of Cardiology Foundationilliam A. Zoghbi, MD, FACC, President

homas E. Arend, Jr, Esq, CAE, Interim Chief Staff Officerharlene L. May, Senior Director, Science and Clinical Policy

merican College of Cardiology Foundation/merican Heart Associationisa Bradfield, CAE, Director, Science and Clinical Policyebjani Mukherjee, MPH, Associate Director, Evidence-Based Medicine

zaldeen Ramadhan III, Specialist, Science and Clinical Policy

merican Heart Associationordon F. Tomaselli, MD, FAHA, Presidentancy Brown, Chief Executive Officerose Marie Robertson, MD, FAHA, Chief Science Officerayle R. Whitman, PhD, RN, FAHA, FAAN, Senior VicePresident, Office of Science Operationsdy L. Bezanson, DSN, RN, CNS-MS, FAHA, Science andMedicine Advisor, Office of Science Operationsdy Hundley, Production Manager, Scientific Publications,Office of Science Operations

References1. Tracy CM, Epstein AE, Darbar D, et al. 2012 ACCF/AHA/HRS focused

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1a.ACCF/AHA Task Force on Practice Guidelines. Methodology Manualand Policies From the ACCF/AHA Task Force on Practice Guidelines.American College of Cardiology Foundation and American HeartAssociation, Inc. 2010. Available at: http://assets.cardiosource.com/Methodology_Manual_for_ACC_AHA_Writing_Committees.pdf andhttp://my.americanheart.org/idc/groups/ahamah-public/@wcm/@sop/documents/downloadable/ucm_319826.pdf. Accessed May 16, 2012.

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http://assets.cardiosource.com/Methodology_Manual_for_ACC_AHA_Writing_Committees.pdf

http://assets.cardiosource.com/Methodology_Manual_for_ACC_AHA_Writing_Committees.pdf

http://my.americanheart.org/idc/groups/ahamah-public/@wcm/@sop/documents/downloadable/ucm_319826.pdf

http://my.americanheart.org/idc/groups/ahamah-public/@wcm/@sop/documents/downloadable/ucm_319826.pdf

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http://www.seattleheartfailuremodel.org

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4. Al-Ahmad A, Wang PJ, Homoud MK, Estes NA III, Link MS. FrequentICD shocks due to double sensing in patients with bi-ventricularimplantable cardioverter defibrillators. J Interv Card Electrophysiol.2003;9:377–81.

5. Friedman PA, McClelland RL, Bamlet WR, et al. Dual-chamber versussingle-chamber detection enhancements for implantable defibrillatorrhythm diagnosis: the detect supraventricular tachycardia study. Circu-lation. 2006;113:2871–9.

6. Williams JL, Shusterman V, Saba S. A pilot study examining theperformance of polynomial-modeled ventricular shock electrograms forrhythm discrimination in implantable devices. Pacing Clin Electro-physiol. 2006;29:930–9.

7. Gunderson BD, Gillberg JM, Wood MA, Vijayaraman P, Shepard RK,Ellenbogen KA. Development and testing of an algorithm to detectimplantable cardioverter-defibrillator lead failure. Heart Rhythm. 2006;3:155–62.

8. Gunderson BD, Patel AS, Bounds CA, Shepard RK, Wood MA,Ellenbogen KA. An algorithm to predict implantable cardioverter-defibrillator lead failure. J Am Coll Cardiol. 2004;44:1898–902.

9. Sears SF Jr, Shea JB, Conti JB. Cardiology patient page. How to respondto an implantable cardioverter-defibrillator shock. Circulation. 2005;111:e380–e382.

0. Shah RV, Lewis EF, Givertz MM. Epicardial left ventricular leadplacement for cardiac resynchronization therapy following failed coro-nary sinus approach. Congest Heart Fail. 2006;12:312–6.

1. Koos R, Sinha AM, Markus K, et al. Comparison of left ventricular leadplacement via the coronary venous approach versus lateral thoracotomyin patients receiving cardiac resynchronization therapy. Am J Cardiol.2004;94:59–63.

2. Gabor S, Prenner G, Wasler A, Schweiger M, Tscheliessnigg KH,Smolle-Juttner FM. A simplified technique for implantation of leftventricular epicardial leads for biventricular re-synchronization usingvideo-assisted thoracoscopy (VATS). Eur J Cardiothorac Surg. 2005;28:797–800.

3. Navia JL, Atik FA, Grimm RA, et al. Minimally invasive left ventricularepicardial lead placement: surgical techniques for heart failure resyn-chronization therapy. Ann Thorac Surg. 2005;79:1536–44.

4. Mair H, Sachweh J, Meuris B, et al. Surgical epicardial left ventricularlead versus coronary sinus lead placement in biventricular pacing. EurJ Cardiothorac Surg. 2005;27:235–42.

5. DeRose JJ, Ashton RC, Belsley S, et al. Robotically assisted leftventricular epicardial lead implantation for biventricular pacing. J AmColl Cardiol. 2003;41:1414–9.

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9. Walsh EP, Cecchin F. Recent advances in pacemaker and implantabledefibrillator therapy for young patients. Curr Opin Cardiol. 2004;19:91–6.

0. Cohen MI, Rhodes LA, Spray TL, Gaynor JW. Efficacy of prophylacticepicardial pacing leads in children and young adults. Ann Thorac Surg.2004;78:197–202.

1. Vogt PR, Sagdic K, Lachat M, Candinas R, von Segesser LK, TurinaMI. Surgical management of infected permanent transvenous pacemakersystems: ten year experience. J Card Surg. 1996;11:180–6.

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6. Owens DK, Sanders GD, Harris RA, et al. Cost-effectiveness ofimplantable cardioverter defibrillators relative to amiodarone for pre-vention of sudden cardiac death. Ann Intern Med. 1997;126:1–12.

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0. Schron EB, Exner DV, Yao Q, et al. Quality of life in the antiarrhyth-mics versus implantable defibrillators trial: impact of therapy andinfluence of adverse symptoms and defibrillator shocks. Circulation.2002;105:589–94.

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2. Exner DV. Quality of life in patients with life-threatening arrhythmias: doeschoice of therapy make a difference?. Am Heart J. 2002;144:208–11.

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4. Wyman BT, Hunter WC, Prinzen FW, Faris OP, McVeigh ER. Effectsof single- and biventricular pacing on temporal and spatial dynamics ofventricular contraction. Am J Physiol Heart Circ Physiol. 2002;282:H372–9.

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6. Sweeney MO. The nature of the game. J Cardiovasc Electrophysiol.2005;16:483–5.

7. Wathen MS, Sweeney MO, DeGroot PJ, et al. Shock reduction usingantitachycardia pacing for spontaneous rapid ventricular tachycardia inpatients with coronary artery disease. Circulation. 2001;104:796–801.

8. Wathen MS, DeGroot PJ, Sweeney MO, et al. Prospective randomizedmulticenter trial of empirical antitachycardia pacing versus shocks forspontaneous rapid ventricular tachycardia in patients with implantablecardioverter-defibrillators: Pacing Fast Ventricular Tachycardia ReducesShock Therapies (PainFREE Rx II) trial results. Circulation. 2004;110:
2591–6.

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9. Winters SL, Packer DL, Marchlinski FE, et al. Consensus statement onindications, guidelines for use, and recommendations for follow-up ofimplantable cardioverter defibrillators. North American Society ofElectrophysiology and Pacing. Pacing Clin Electrophysiol. 2001;24:262–9.

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EY WORDS: ACCF/AHA Practice Guidelines � arrhythmia �rdiomyopathy � cardiac resynchronization � device-based therapy �
cused update � heart failure � pacing.

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ppendix 1. 2008 Author Relationships With Industry and Other Entities (Relevant)—ACC/AHA/HRS 2008 Guidelines for Device-Basederapy of Cardiac Rhythm Abnormalities

Committee MemberConsulting

Fees/Honoraria Speakers’ Bureau

Ownership/Partnership/

Principal Research GrantsInstitutional or Other

Financial Benefit

drew E. Epstein* ● Boston Scientific● CryoCath● Medtronic● Sanofi-Aventis● St. Jude†

● Boston Scientific● Medtronic● Reliant Pharmaceuticals● Sanofi-Aventis● St. Jude

None ● Biotronik†● Boston Scientific†● C. R.

Bard/ElectrophysiologyDivision†

● Irving Biomedical†● Medtronic†● St. Jude†

Electrophysiology fellowshipsupport from:● Medtronic†● St. Jude†

hn P. DiMarco* ● Boston Scientific†● CV Therapeutics†● Daiichi Sankyo● Medtronic†● Novartis†● Sanofi-Aventis● Solvay● St. Jude

None None ● Boston Scientific†● CV Therapeutics†● Medtronic● Sanofi-Aventis● St. Jude

None

nneth A. Ellenbogen* ● Ablation Frontiers● Atricure● Biosense Webster● Biotronik● Boston Scientific● Medtronic● Sorin/ELA● St. Jude

● Reliant Pharmaceuticals● Sanofi-Aventis

None ● Biosense Webster● Boston Scientific†● Cameron Medical● Impulse Dynamics● Medtronic†● St. Jude

None

A. Mark Estes III ● Medtronic ● Boston Scientific● Medtronic● St. Jude

None None None

ger A. Freedman* ● Boston Scientific● Medtronic● Sorin/ELA● St. Jude

● Boston Scientific● St. Jude

● St. Jude ● Boston Scientific†● Medtronic†● St. Jude†

University of Utah Division ofCardiology receiveselectrophysiology fellowshipsupport grants from:● Boston Scientific†● Medtronic†● St. Jude†

onard S. Gettes None None None None NoneMarc Gillinov* ● AtriCure

● Edwards†● Medtronic

● Guidant● St. Jude

● Viacor† None None

briel Gregoratos None None None None Noneephen C. Hammill ● Biosense Webster ● Boston Scientific None ● Medtronic Nonevid L. Hayes* ● AI Semi

● Blackwell/Futura†● Boston Scientific†● Medtronic†● Sorin/ELA● St. Jude

None None ● Boston Scientific†● Medtronic†● St. Jude

● Biotronik● Boston Scientific†● Medtronic†● Sorin/ELA● St. Jude

rk A. Hlatky ● Blue Cross/BlueShield TechnologyEvaluation Center

None None None None

Kristin Newby ● AstraZeneca/Atherogenics● Biosite● CV Therapeutics● Johnson &

Johnson● Novartis● Procter & Gamble● Roche

Diagnostics

None None ● Adolor● American Heart

Association†● BG Medicine● Bristol-Myers

Squibb/Sanofi†● Inverness

Medical†● Medicure†● Schering-Plough†

None

hard L. Page ● Astellas● Berlex● Pfizer● Sanofi-Aventis†

None None ● Procter & Gamble ● Boston Scientific†● Medtronic†● St. Jude†

(Continued)

A

MaMiLy

Mi

coneowfuit


ppendix 1. Continued

Committee MemberConsulting

Fees/Honoraria Speakers’ Bureau


Principal Research GrantsInstitutional or Other

Financial Benefit

rk H. Schoenfeld None None None None Nonechael J. Silka None None None None Nonenne Warner Stevenson ● Biosense

Webster‡● Boston Scientific‡● CardioMEMS● Medtronic● Medtronic‡● Scios

None None ● BiosenseWebster‡

● Medtronic

None

chael O. Sweeney ● Medtronic† ● Boston Scientific● Medtronic†

None None None

This table represents the relationships of committee members with industry that were reported orally at the initial writing committee meeting and updated innjunction with all meetings and conference calls of the writing committee during the document development process (last revision, January 16, 2008). It does notcessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest representsnership of 5% or more of the voting stock or share of the business entity, or ownership of $10,000 or more of the fair market value of the business entity, or if

nds received by the person from the business entity exceed 5% of the person’s gross income for the previous year. A relationship is considered to be modest ifis less than significant under the preceding definition. Relationships noted in this table are modest unless otherwise noted.*Recused from voting on guideline recommendations.†Indicates significant-level relationship (more than $10,000).
‡Indicates spousal relationship.

Afo

Pe

Mi

Fre

Br

Sa

Pa

Stu

Pa

Ma

Je

Ch

No


ppendix 2. 2008 Reviewer Relationships With Industry and Other Entities (Relevant)—ACC/AHA/HRS 2008 Guidelinesr Device-Based Therapy of Cardiac Rhythm Abnormalities

er Reviewer* Representation Consulting Fees/Honoraria Speakers’ Bureau


Principal Research Grant

Institutional orOther Financial

Benefit

na K. Chung Official—Heart Rhythm Society ● American College ofCardiology Foundation

● Boston Medical Center● Boston Scientific (honoraria

donated)● Elsevier● Medtronic (honoraria

donated)● Nexcura (no honoraria

received)● University of Texas Health

Science Center● WebMD Health (for CryoCath

Technologies, Inc.)

None None ● Biotronik† (researchgrants toelectrophysiologysection, ClevelandClinic)

● Boston Scientific†(research grants toelectrophysiologysection, ClevelandClinic)

● Medtronic†(research grants toelectrophysiologysection, ClevelandClinic)

● ReliantPharmaceuticals†(research grants toelectrophysiologysection, ClevelandClinic)

● St. Jude Medical†(research grants toelectrophysiologysection, ClevelandClinic)

None

d Kusumoto Official—Heart Rhythm Society ● Boston Scientific● Medtronic

None None None None

uce Lindsay Official—American College ofCardiology Board ofTrustees

None None None None None

mir Saba Official—American HeartAssociation

None None None ● Boston Scientific● Medtronic● St. Jude Medical

None

ul Wang Official—American HeartAssociation;Content—American HeartAssociationElectrocardiography andArrhythmias Committee

● Boston Scientific†● Lifewatch†● Medtronic● St. Jude

● Boston Scientific†● Medtronic● St. Jude

● Hansen Medical† ● Boston Scientific†● Medtronic● St. Jude

None

art Winston Official—American College ofCardiology Board ofGovernors

● Boston Scientific None None None None

trickMcCarthy

Organizational—Society ofThoracic Surgeons

● CV Therapeutics†● Medtronic

None None None None

ndeep Mehra Organizational—Heart FailureSociety of America

● Astellas● Boston Scientific● Cordis● Debiopharma● Medtronic● Novartis● Roche Diagnostics● Scios● Solvay● St. Jude

None None ● Maryland IndustrialPartnerships†

● National Institutesof Health†

● Other Tobacco-Related Diseases†

● University ofMaryland†(salary);

● Legal consultant

nniferCummings

Content—American College ofCardiology FoundationClinical ElectrophysiologyCommittee

● Reliant● Signalife● St. Jude● Zin

None None None None

ristopherFellows

Content—American College ofCardiology FoundationClinical ElectrophysiologyCommittee

● Boston Scientific● St. Jude

None None None None

raGoldschlager

Content—Individual ● St. Jude None None None None

(Continued)

A

P

Pe

Ra

J.

Ge

Ed

Cly

thofexRe



eer Reviewer* Representation Consulting Fees/Honoraria Speakers’ Bureau


Principal Research Grant

Institutional orOther Financial

Benefit

ter Kowey Content—American College ofCardiology Foundation ClinicalElectrophysiology Committee

None ● Medtronic† ● CardioNet† None None

chel Lampert Content—Heart RhythmSociety Scientific and ClinicalDocuments Committee

None None None ● Boston Scientific†● Medtronic†● St. Jude†

None

Philip Saul Content—Pediatric Expert andAmerican College of CardiologyFoundation ClinicalElectrophysiology Committee


orge Van Hare Content—Individual ● St. Jude None None ● Medtronic† (fellowshipfunding)

None

ward P. Walsh Content—Individual PediatricExpert


de Yancy Content—American College ofCardiology/American HeartAssociation Lead Task ForceReviewer and 2005 ChronicHeart Failure Guideline WritingCommittee

● AstraZeneca● GlaxoSmithKline● Medtronic● NitroMed● Otsuka● Scios

● GlaxoSmithKline● Novartis

None ● GlaxoSmithKline● Medtronic● NitroMed● Scios

None

This table represents the relationships of reviewers with industry that were reported at peer review. It does not necessarily reflect relationships with industry ate time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of 5% or more of the voting stock or sharethe business entity, or ownership of $10,000 or more of the fair market value of the business entity, or if funds received by the person from the business entityceed 5% of the person’s gross income for the previous year. A relationship is considered to be modest if it is less than significant under the preceding definition.lationships noted in this table are modest unless otherwise noted.*Names are listed in alphabetical order within each category of review. Participation in the peer review process does not imply endorsement of this document.
†Indicates significant-level relationship (more than $10,000).

A

AC

AC

AF

AH

AM

AM

AR

AT

AV

AV

CA

CA

CA

CA

CI

CI

CI

CA

CO

CR

CR

CR

CT

D

D

D

D

D

EC

G

H

A

HR

HR

IC

LB

LV

LV

M

M

M

M

M

M

M

NY

Pa

PA

PA

Q

RA

RE

RR

RV

RV

RW

SC

SC

SN

SV

TF

TT

UK

VF

VP

VP

VT


ppendix 3. Abbreviations List

C � American College of Cardiology

CF � American College of Cardiology Foundation

� Atrial fibrillation

A � American Heart Association

I � Acute myocardial infarction

IOVIRT � Amiodarone Versus Implantable Defibrillator in Patients withNonischemic Cardiomyopathy and Asymptomatic Nonsustained VentricularTachycardia

VD/C � Arrhythmogenic right ventricular dysplasia/cardiomyopathy

P � Antitachycardia pacing

� Atrioventricular

ID � Antiarrhythmics Versus Implantable Defibrillators

BG-Patch � Coronary Artery Bypass Graft-Patch

RE-HF � Cardiac Resynchronization in Heart Failure

SH � Cardiac Arrest Study Hamburg

T � Cardiomyopathy Trial

� Confidence interval

DS � Canadian Implantable Defibrillator Study

ED � Cardiovascular implantable electronic devices

RE-HF � Cardiac Resynchronization in Heart Failure

MPANION � Comparison of Medical Therapy, Pacing, and Defibrillation inHeart Failure Trial

T � Cardiac resynchronization therapy

T-D � Cardiac resynchronization therapy device incorporating bothpacing and defibrillation capabilities

T-P � Cardiac resynchronization device providing pacing but notdefibrillation capability

OPP � Canadian Trial of Physiologic Pacing

AVID � Dual Chamber and VVI Implantable Defibrillator

CM � Dilated cardiomyopathy

DD � Dual-chamber pacemaker that senses/paces in the atrium/ventricleand is inhibited/triggered by intrinsic rhythm

EFINITE � Defibrillators in Nonischemic Cardiomyopathy TreatmentEvaluation

INAMIT � Defibrillator in Acute Myocardial Infarction Trial

G � Electrocardiograph

DMT � Guideline-directed medical therapy

CM � Hypertrophic cardiomyopathy

(Continued)


� Hazard ratio

S � Heart Rhythm Society

D � Implantable cardioverter-defibrillator

BB � Left bundle-branch block

� Left ventricular/left ventricle

EF � Left ventricular ejection fraction

ADIT-CRT � Multicenter Automatic Defibrillator Implantation Trial withCardiac Resynchronization Therapy

ADIT I � Multicenter Automatic Defibrillator Implantation Trial I

ADIT II � Multicenter Automatic Defibrillator Implantation Trial II

I � Myocardial infarction

IRACLE ICD II � Multicenter InSync ICD Randomized Clinical Evaluation II

OST � Mode Selection Trial

USTT � Antiarrhythmic Drug Therapy in the Multicenter UnSustainedTachycardia Trial

HA � New York Heart Association

inFREE Rx II � Pacing Fast VT Reduces Shock Therapies Trial II

SE � Pacemaker Selection in the Elderly

VE � Left Ventricular-Based Cardiac Stimulation Post AV Nodal AblationEvaluation Study

OL � Quality of life

FT � Resynchronization-Defibrillation for Ambulatory Heart Failure Trial

VERSE � Resynchronization Reverses Remodeling in Systolic LeftVentricular Dysfunction

� Relative risk

� Right ventricular/right ventricle

A � Right ventricular apical

I � Relationships with industry and other entities

D � Sudden cardiac death

D-HeFT � Sudden Cardiac Death in Heart Failure Trial

D � Sinus node dysfunction

T � Supraventricular tachycardia

� Task Force

M � Transtelephonic monitoring

-PACE � United Kingdom Pacing and Cardiovascular Events

� Ventricular fibrillation

S � Vasovagal Pacemaker Study I

S-II � Vasovagal Pacemaker Study II

� Ventricular tachycardia

AIn

C

Cy

An

Da

Jo

Sa

N.

T.

Ste

Pa

Ma

Jo

Ma

Am


ppendix 4. 2012 Author Relationships With Industry and Other Entities (Relevant)—2012 ACCF/AHA/HRS Focused Updatecorporated Into the ACCF/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities

ommittee Member Employment ConsultantSpeaker’s

Bureau


Principal Personal Research

Institutional,Organizational, orOther Financial

BenefitExpert

Witness

VotingRecusals

bySection*

nthia M. Tracy,Chair

George Washington UniversityMedicalCenter—AssociateDirector and Professor ofMedicine

None None None None None None None

drew E. Epstein,Vice Chair

University of Pennsylvania—Professor of MedicinePhiladelphia VA MedicalCenter—Chief, CardiologySection

● Boston Scientific(DSMB)‡

● Medtronic‡● Medtronic/Cryocath

(DSMB)● St. Jude Medical

(DSMB)‡● Zoll—Advisory

Board

None None ● Biotronik● Boston Scientific‡● Cameron Health‡● Medtronic‡● St. Jude Medical‡

● BostonScientific,Medtronic, St.Jude Medical—industrysupporters offellowshipprogram

None 2.4.1

wood Darbar Vanderbilt University Schoolof Medicine—C. SydneyBurwell AssociateProfessor MedicinePharmacology


Vanderbilt ArrhythmiaService—Director

hn P. DiMarco University ofVirginia—Director, ClinicalEP Laboratory

● Medtronic● St. Jude Medical

None None ● Boston Scientific None None 2.4.1

ndra B. Dunbar Emory University, NellHodgson Woodruff Schoolof Nursing—AssociateDean for AcademicAdvancement, CharlesHoward Candler Professor


A. Mark Estes III Tufts University—Professor ofMedicine

● Boston Scientific‡● Boston Scientific,

EP FellowshipEducationalSymposium‡

● Medtronic

None None ● Boston Scientific—MADIT-RIT (Co-PI)

None None 2.4.1

Bruce Ferguson, Jr East Carolina University—Professor of Surgery andPhysiology

● UnitedHealthcare—AdvisoryBoard

None None None None None 2.4.1

phen C. Hammill Mayo Clinic—Professor ofMedicine


mela E. Karasik Georgetown UniversityMedicalSchool—AssociateProfessor of Medicine


VA Medical Center,Washington, DC—ActingChief of Cardiology

rk S. Link Tufts MedicalCenter—Professor ofMedicine

None None None ● Boston Scientific—MADIT-RIT (Co-PI)

None None 2.4.1

seph E. Marine Johns Hopkins University—Associate Professor ofMedicine


rk H. Schoenfeld Yale University School ofMedicine—ClinicalProfessor of Medicine

● United HealthcareAdvisory Board

None None None None None 2.4.1

it J. Shanker Center for AdvancedArrhythmia Medicine—Director


Columbia University Collegeof Physicians andSurgeons—AssistantProfessor of Medicine

(Continued)

A

Mi

Ly

Wi

Pa

reThrethex

orinfo

Tr



Committee Member Employment ConsultantSpeaker’s

Bureau


Principal Personal Research

Institutional,Organizational, orOther Financial

BenefitExpert

Witness

VotingRecusals

bySection*

chael J. Silka University of SouthernCalifornia—Professor ofPediatrics


Children’s Hospital LosAngeles—Chief, Divisionof Cardiology

nne Warner Stevenson Brigham & Women’sHospital—Director,Cardiomyopathy and HeartFailure

None None None ● Biosense Webster† None None 2.4.1

lliam G. Stevenson Brigham & Women’sHospital—Director, ClinicalCardiac EP

None None None ● Biosense Webster† None None 2.4.1

ul D. Varosy VA Eastern Colorado HealthCare System—Director ofCardiac Electrophysiology


University of ColoradoDenver—AssistantProfessor of Medicine

This table represents the relationships of committee members with industry and other entities that were determined to be relevant to this document. Theselationships were reviewed and updated in conjunction with all meetings and/or conference calls of the writing group during the document development process.e table does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interestpresents ownership of �5% of the voting stock or share of the business entity, or if the interest represents ownership of �$10,000 of the fair market value ofe business entity, or if funds received by the person from the business entity exceed 5% of the person’s gross income for the previous year. Relationships thatist with no financial benefit are also included for the purpose of transparency. Relationships in this table are modest unless otherwise noted.*Writing group members are required to recuse themselves from voting on sections to which their specific relationships with industry and other entities may apply.†No financial benefit.‡Indicates significant relationship.

According to the ACCF/AHA, a person has a relevant relationship IF: (a) the relationship or interest relates to the same or similar subject matter, intellectual propertyasset, topic, or issue addressed in the document; or (b) the company/entity (with whom the relationship exists) makes a drug, drug class, or device addressedthe document or makes a competing drug or device addressed in the document; or (c) the person or a member of the person’s household has a reasonable potentialr financial, professional, or other personal gain or loss as a result of the issues or content addressed in the document.DSMB indicates Data Safety Monitoring Board; EP, electrophysiology; HRS, Heart Rhythm Society; MADIT-RIT, Multicenter Automatic Defibrillator Implantation

ial–Reduce Inappropriate Therapy; PI, principal investigator; and VA, Veterans Affairs.

AIn

Pe

Sa

Hu

Ja

Mi

Jo

Bra

Th

He

C.

Pa

Jo

Ge

Je

Ke

Ro

Ga

Da

MaSa

Sa


ppendix 5. 2012 Reviewer Relationships With Industry and Other Entities (Relevant)—2012 ACCF/AHA/HRS Focused Updatecorporated Into the ACCF/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities

er Reviewer Representation Employment Consultant Speaker’s Bureau

Ownership/

Partnership/

Principal

Personal

Research

Institutional,

Organizational, or

Other Financial

Benefit Expert Witness

na Al-Khatib Official Representative—

AHA

Duke Clinical Research Institute and Duke

University Medical Center

None ● Medtronic None None None None

gh Calkins Official Representative—

HRS

Johns Hopkins Hospital ● Biosense

Webster

● Boston

Scientific

● Medtronic*

None None ● Boston

Scientific*

● Medtronic*

● St. Jude

Medical*

None None

mes R.

Edgerton

Official Representative—

STS

The Heart Hospital Baylor Plano None None None None None None

chael M.

Givertz


HFSA

Brigham and Women’s Hospital None None None None None None

nathan L.

Halperin


ACCF/AHA Task Force

on Practice Guidelines

Lead Reviewer

Mount Sinai Medical Center ● Biotronik* None None None None None

dley P.

Knight


HRS

Northwestern Medical Center ● Boston

Scientific

● Cameron

Health†

● Biosense

Webster

● Biotronik

● Boston Scientific

● Medtronic

None ● Cameron

Health*

None None

omas J.

Lewandowski


ACCF Board of

Governors

Appleton Cardiology Thedacare None None None None None None

nry M.

Spotnitz


AATS

Columbia University None None ● Strategic

Pacing

Systems†

None None None

Michael

Valentine


ACCF Board of

Trustees

The Cardiovascular Group ● Medtronic* None None None None None

ul J. Wang Official Representative—

AHA

Stanford University Medical Center ● Medtronic None None ● Medtronic* None None

hn F. Beshai Content Reviewer—ACCF

EP Committee

University of Chicago Medical Center None None None None ● Medtronic*

● St. Jude Medical*

None

orge H.

Crossley

Content Reviewer St. Thomas Heart ● Boston

Scientific

● Medtronic


● Medtronic

None None ● Boston Scientific

● Medtronic*

None

nnifer E.

Cummings

Content Reviewer—ACCF

EP Committee

University of Toledo None ● Boston Scientific

● Medtronic

● St. Jude

None None None None

nneth A.

Ellenbogen

Content Reviewer Virginia Commonwealth University Medical

Center

● Cameron Health

● Boston

Scientific

● Medtronic

● Biotronik


● Medtronic

● St. Jude Medical

None None ● Biosense

Webster*

● Boston Scientific*

● Medtronic*


None

ger A.

Freedman

Content Reviewer University of Utah Health Sciences Center ● Boston

Scientific

● Sorin

● Spectranetics

● St. Jude

Medical

None None ● Medtronic*

● St. Jude

Medical*

None ● Defendant, 2011,

pacemaker battery

depletion

briel

Gregoratos

Content Reviewer University of California–San Francisco None None None None None None

vid L. Hayes Content Reviewer Mayo Clinic ● Biotronik

● Boston

Scientific

● Medtronic*

● Sorin

● St. Jude

Medical


rk A. Hlatky Content Reviewer Stanford University School of Medicine None None None None None Nonendeep K.

Jain

Content Reviewer University of Pittsburgh Physicians, UPMC

Heart and Vascular Institute

None None None ● Medtronic* None None

muel O.

Jones

Content Reviewer San Antonio Military Medical Center None None None None ● Medtronic†

● St. Jude

Medical†

None

(Continued)

A

Ko

MiSte

MaSimL.Bri

Ric

AllJoSte

Itrethcoof

orinfo

PhDe



usik Krishnan Content Reviewer Rush University Medical Center ● Boston Scientific


None None None ● Biotronik*

● Boston

Scientific—Multicenter

MADIT-RIT study*

● Medtronic*

None

chael Mansour Content Reviewer Cardiovascular Physicians None None None None None Noneven M. Markowitz Content Reviewer—ACCF

EP Committee

New York Hospital ● Biotronik


● Medtronic


None None None ● Biosense

Webster*

● Boston Scientific*

● Medtronic*


None

rco A. Mercader Content Reviewer George Washington University None None None None ●None Noneone Musco Content Reviewer Saint Patrick Hospital ● Boston Scientific None None None None None

Kristin Newby Content Reviewer Duke University Medical Center None None None None None Nonean Olshansky Content Reviewer—ACCF

EP Committee

University of Iowa Hospitals ● Boston Scientific–

Guidant

● Medtronic


hard L. Page Content Reviewer University of Wisconsin Hospital

and Clinics

None None None None None None

en J. Solomon Content Reviewer Medical Faculty Associates None None None None None Nonehn S. Strobel Content Reviewer Internal Medicine Associates None None None ● Medtronic* None Nonephen L. Winters Content Reviewer Morristown Medical Center ● Biosense Webster None None None ● Boston Scientific

● Medtronic


● Defendant,

2011,

complication of

ICD placement

This table represents the relationships of reviewers with industry and other entities that were disclosed at the time of peer review and determined to be relevant.does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interestpresents ownership of �5% of the voting stock or share of the business entity, or if the interest represents ownership of �$10,000 of the fair market value ofe business entity, or if funds received by the person from the business entity exceed 5% of the person’s gross income for the previous year. A relationship isnsidered to be modest if it is less than significant under the preceding definition. Relationships that exist with no financial benefit are also included for the purposetransparency. Relationships in this table are modest unless otherwise noted. Names are listed in alphabetical order within each category of review.*Significant relationship.†No financial benefit.

According to the ACCF/AHA, a person has a relevant relationship IF: (a) the relationship or interest relates to the same or similar subject matter, intellectual propertyasset, topic, or issue addressed in the document; or (b) the company/entity (with whom the relationship exists) makes a drug, drug class, or device addressedthe document or makes a competing drug or device addressed in the document; or (c) the person or a member of the person’s household has a reasonable potentialr financial, professional, or other personal gain or loss as a result of the issues or content addressed in the document.AATS indicates American Association for Thoracic Surgery; ACCF, American College of Cardiology Foundation; AHA, American Heart Association; EP, Emergencyysicians; HFSA, Heart Failure Society of America; HRS, Heart Rhythm Society; ICD, implantable cardioverter-defibrillator; MADIT-RIT, Multicenter Automatic
fibrillator Implantation Trial–Reduce Inappropriate Therapy; and STS, Society of Thoracic Surgeons.

A

caNY


ppendix 6. 2012 Indications for CRT Therapy—Algorithm

CRT indicates cardiac resynchronization therapy; CRT-D, cardiac resynchronization therapy defibrillator; GDMT, guideline-directed medical therapy; ICD, implantablerdioverter-defibrillator; IV, intravenous; LV, left ventricular; LVEF, left ventricular ejection fraction; LBBB, left bundle-branch block; MI, myocardial infarction; and
HA, New York Heart Association.