ESC GUIDELINES
2015 ESC Guidelines for the management
of patients with v entricular arrhythmias
and the prev ention of sudden cardia c death
The Task Force for the Management of Patients with Ventricular
Arrhythmias and the Prevention of Sudden Cardiac Death of the
European Society of Cardiology (ESC)
Endorsed by: Association for European Paediatric and Congenital
Cardiology (AEPC)
Authors/Task Force Members: Silvia G. Priori
*
(Chairperson) (Italy),
Carina Blomstro
¨
m-Lundqvist
*
(Co-chairperson) (Sweden), Andrea Mazzanti
†
(Italy),
Nico Blom
a
(The Netherlands), Martin Borggrefe (Germany), John Camm (UK),
Perry Mark Elliott (UK), Donna Fitzsimons (UK), Robert Hatala (Slovakia),
Gerhard Hindricks (Germany), Paulus Kirchhof (UK/Germany), Keld Kjeldsen
(Denmark), Karl-Heinz Kuck (Germany), Antonio Hernandez-Madrid (Spain),
Nikolaos Nikolaou (Greece), Tone M. Norekva
˚
l (Norway), Christian Spaulding
(France), and Dirk J. Van Veldhuisen (The Netherlands)
* Corresponding authors: Silvia Giuliana Priori, Department of Molecular Medicine University of Pavia, Cardiology & Molecula r Cardio logy, IRCCS Fondazione Salvatore Maugeri,
Via Salvatore Maugeri 10/10A, IT-27100 Pavia, Italy, Tel: +39 0382 592 040, Fax: +39 0382 592 059, Email:
silvia.priori@fsm.it
Carina Blomstro¨m-Lundqvist, Department of Cardiology, Institution of Medical Science, Uppsala University, SE-751 85 Uppsala, Sweden, Tel: +46 18 611 3113, Fax: +46 18 510 243,
Email:
carina.blomstrom.lundqvist@akademiska.se
a
Representing the Association for European Paediatric and Congenital Cardiology (AEPC).
†
Andrea Mazzanti: Coordinator, affiliation listed in the Appendix.
ESC Committee for Practice Guidelines (CPG) and National Cardiac Societies document reviewers: listed in the Appendix.
ESC entities having participated in the development of this document:
ESC Associations: Acute Cardiovascular Care Association (ACCA), European Association of Cardiovascular Imaging (EACVI), European Association of Percutaneous Cardiovascular
Interventions (EAPCI), European Heart Rhythm Association (EHRA), Heart Failure Association (HFA).
ESC Councils : Council for Cardiology Practice (CCP ), Council on Cardiovascular Nursing and Allied Professions (C CNAP), Council on Cardiovascular Primary Care (CCPC),
Council on Hypertension.
ESC Working Groups: Cardiac Cellular Electrophysiology, Cardiovascular Pharmacotherapy, Cardi ovascular Surgery, Grown-up Congenital Heart Disease, Myocardial and
Pericardial Diseases, Pulmonary Circulation and Right Ventricular Function, Thrombosis, Valvular Heart Disease.
The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC
Guidelines may be translated or reproduced in any form without written permission from the ESC. Permiss ion can be obtained upon submission of a writ ten request to Oxford
University Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC.
Disclaimer: The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge and the evidence available at
the time of their publication. The ESC is not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC Guidelines and any other official recom-
mendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encour-
aged to take the ESC Gu idelines fully into account when exercising their clinical judgment, as we ll as in the determination and the implementation of preventive, diagnostic or
therapeutic medical strategies; however, the ESC Guidelines do not override, in any way whatsoever, the individual respons ibility of health professionals to make appropriate and
accurate decisions in consideration of each patient’s health condition and in consultation with that patient and, where appropriate and/or necessary, the patient’s caregiver. Nor
do the ESC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent
public health authorities, in order to manage each patient’s case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the
health professional’s responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.
& The European Society of Cardiology and the European Respiratory Society 2015. All rights reserved. For permissions please email: journals.permissions@oup.com.
European Heart Journal
doi:10.1093/eurheartj/ehv316
European Heart Journal Advance Access published August 29, 2015
by guest on October 4, 2015http://eurheartj.oxfordjournals.org/Downloaded from
Document Reviewers: Philippe Kolh (CPG Review Coordinator) (Belgium), Gregory Y. H. Lip (CPG Review
Coordinator) (UK), Stefan Agewall (Norway), Gonzalo Baro
´
n-Esquivias (Spain), Giuseppe Boriani (Italy),
Werner Budts (Belgium), He
´
ctor Bueno (Spain), Davide Capodanno (Italy), Scipione Carerj (Italy),
Maria G. Crespo-Leiro (Spain), Martin Czerny (Switzerland), Christi Deaton (UK), Dobromir Dobrev (Germany),
Çetin Erol (Turkey), Maurizio Galderisi (Italy), Bulent Gorenek (Turkey), Thomas Kriebel (Germany), Pier Lambiase
(UK), Patrizio Lancellotti (Belgium), Deirdre A. Lane (UK), Irene Lang (Austria), Athanasios J. Manolis (Greece),
Joao Morais (Portugal), Javier Moreno (Spain), Massimo F. Piepoli (Italy), Frans H. Rutten (The Netherlands),
Beata Sredniawa (Poland), Jose L. Zamorano (Spain), and Faiez Zannad (France)
The disclosure forms of all experts involved in the development of these guidelines are available on the ESC website
http://www.escardio.org/guidelines
------------------------------------------------------------------------------------------------------------------------------------------------------
Keywords Acute coronary syndrome † Cardiac resynchronization therapy † Cardiomyopathy † Congenital heart disease
† Defibrillator † Guidelines † Heart failure † Implantable cardioverter defibrillator † Myocardial infarction
† Resuscitation † Stable coronary artery disease † Sudden cardiac death † Tachycardia † Valvular heart
disease † Ventricular arrhythmia
Table of Contents
Abbreviations and acronyms . . . . . . . . . . . . . . . . . . . . . . . .
4
1. Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
2. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
2.1 Structure of the guidelines . . . . . . . . . . . . . . . . . . . . 7
3. Definitions, epidemiology and future perspectives for the
prevention of sudden cardiac death . . . . . . . . . . . . . . . . . . . .
7
3.1 Epidemiology of sudden cardiac death . . . . . . . . . . . . . 7
3.1.1 Causes of sudden cardiac death in different age
groups . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
3.2 Autopsy and molecular autopsy in sudden death victims . 8
3.3 Risk prediction of sudden cardiac death . . . . . . . . . . . 8
3.3.1 Individuals without known heart disease . . . . . . . . 9
3.3.2 Patients with ischaemic heart disease . . . . . . . . . . 9
3.3.3 Patients with inheritable arrhythmogenic diseases . . 9
3.4 Prevention of sudden cardiac death in special settings . . 9
3.4.1 Screening the general population for the risk of
sudden cardiac death . . . . . . . . . . . . . . . . . . . . . . . . . 9
3.4.2 Screening family members of sudden death
victims . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
3.4.3 Screening patients with documented or suspected
ventricular arrhythmias . . . . . . . . . . . . . . . . . . . . . . . 10
3.4.3.1 Clinical history . . . . . . . . . . . . . . . . . . . . . . 10
3.4.3.2 Non-invasive and invasive evaluation . . . . . . . . 11
4. Therapies for ventricular arrhythmias . . . . . . . . . . . . . . . . 14
4.1 Treatment of underlying heart disease . . . . . . . . . . . . 14
4.2 Pharmacotherapy for ventricular arrhythmia and
prevention of sudden cardiac death . . . . . . . . . . . . . . . . .
15
4.2.1 General management . . . . . . . . . . . . . . . . . . . . 15
4.2.2 Anti-arrhythmic drugs . . . . . . . . . . . . . . . . . . . . 15
4.2.2.1 Beta-blockers . . . . . . . . . . . . . . . . . . . . . . . 15
4.2.2.2 Amiodarone . . . . . . . . . . . . . . . . . . . . . . . 15
4.2.2.3 Sotalol/d-sotalol . . . . . . . . . . . . . . . . . . . . . 17
4.2.2.4 Combination therapy . . . . . . . . . . . . . . . . . . 17
4.2.3 Patients with a cardioverter defibrillator . . . . . . . . 17
4.2.4 Electrolytes . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
4.2.5 Other drug therapy . . . . . . . . . . . . . . . . . . . . . . 17
4.3 Device therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
4.3.1 Implantable cardioverter defibrillator . . . . . . . . . . 17
4.3.1.1 Secondary prevention of sudden cardiac death
and ventricular tachycardia . . . . . . . . . . . . . . . . . . . 18
4.3.2 Subcutaneous implantable cardioverter
defibrillator . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
4.3.3 Wearable cardioverter defibrillator . . . . . . . . . . . 19
4.3.4 Public access defibrillation . . . . . . . . . . . . . . . . . 19
4.4 Acute treatement of sustained ventricular arrhythmias . . 20
4.5 Interventional therapy . . . . . . . . . . . . . . . . . . . . . . . 22
4.5.1 Catheter ablation . . . . . . . . . . . . . . . . . . . . . . . 22
4.5.1.1 Patients with scar-related heart disease . . . . . . 22
4.5.1.2 Patients without overt structural heart disease . 22
4.5.2 Anti-arrhythmic surgery . . . . . . . . . . . . . . . . . . . 23
4.6 Psychosocial impact of implantable cardioverter
defibrillator treatment . . . . . . . . . . . . . . . . . . . . . . . . .
23
5. Management of ventricular arrhythmias and prevention of
sudden cardiac death in coronary artery disease . . . . . . . . . . .
24
5.1 Acute coronary syndromes . . . . . . . . . . . . . . . . . . . 24
5.1.1 Ventricular arrhythmias associated with acute
coronary syndromes . . . . . . . . . . . . . . . . . . . . . . . . 24
5.1.2 Prevention and management of sudden cardiac death
associated with acute coronary syndromes: pre-hospital
phase . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24
5.1.3 Prevention of sudden cardiac death associated with
acute coronary syndromes: in-hospital phase . . . . . . . . . 24
5.1.3.1 Ventricular arrhythmias in acute coronary
syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
5.1.3.2 Use of anti-arrhythmic drugs in acute coronary
syndromes—general considerations . . . . . . . . . . . . . 26
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5.1.3.3 Patients with acute coronary syndromes and no
ventricular arrhythmias . . . . . . . . . . . . . . . . . . . . . . 26
5.1.3.4 Premature ventricular complexes . . . . . . . . . . 26
5.1.3.5 Sustained VT and VF . . . . . . . . . . . . . . . . . . 26
5.1.3.6 Catheter ablation of recurrent sustained
ventricular tachycardia, recurrent ventricular fibrillation,
and electrical storm . . . . . . . . . . . . . . . . . . . . . . . . 26
5.1.3.7 Extracorporeal support devices . . . . . . . . . . . 27
5.1.3.8 Bradycardia and heart block . . . . . . . . . . . . . 27
5.1.4 The prognostic role of early ventricular fibrillation . . 27
5.2 Early after myocardial infarction . . . . . . . . . . . . . . . . 27
5.2.1 Risk stratification for sudden cardiac death . . . . . . . 27
5.2.2 Timing of implantable cardioverter defibrillator
placement after myocardial infarction—assessment of left
ventricular dysfunction before and after discharge . . . . . . 27
5.3 Stable coronary artery disease after myocardial infarction
with preserved ejection fraction . . . . . . . . . . . . . . . . . . .
28
5.3.1 Risk stratification . . . . . . . . . . . . . . . . . . . . . . . 28
5.3.2 Recommendations for optimal strategy . . . . . . . . . 28
5.3.3 Use of anti-arrhythmic drugs . . . . . . . . . . . . . . . . 28
5.3.4 Catheter ablation . . . . . . . . . . . . . . . . . . . . . . . 29
6. Therapies for patients with left ventricular dysfunction, with or
without heart failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
29
6.1 Primary prevention of sudden cardiac death . . . . . . . . . 29
6.1.1 Drugs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
6.1.2 Implantable cardioverter defibrillators . . . . . . . . . . 30
6.1.3 Implantable cardioverter defibrillators in patients with
New York Heart Association class IV listed for heart
transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
6.1.4 Cardiac resynchronization therapy . . . . . . . . . . . . 31
6.1.4.1 Heart failure with reduced left ventricular
ejection fraction and New York Heart Association class
III/ambulatory class IV . . . . . . . . . . . . . . . . . . . . . . 31
6.1.4.2 Heart failure with reduced left ventricular
ejection fraction but mild symptoms (New York Heart
Association class II) . . . . . . . . . . . . . . . . . . . . . . . . 33
6.2 Premature ventricular complexes in patients with
structural heart disease/left ventricular dysfunction . . . . . . .
33
6.3 Sustained ventricular tachycardia . . . . . . . . . . . . . . . . 33
6.3.1 Drug therapy . . . . . . . . . . . . . . . . . . . . . . . . . . 33
6.3.2 Catheter ablation . . . . . . . . . . . . . . . . . . . . . . . 34
6.3.2.1 Patients with left ventricular dysfunction . . . . . 34
6.3.2.2 Bundle branch re-entrant tachycardia . . . . . . . 35
6.3.3 Implantable cardioverter defibrillator . . . . . . . . . . 35
7. Cardiomyopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
7.1 Dilated cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . 35
7.1.1 Definitions, epidemiology, and survival data . . . . . . 35
7.1.2 Approach to risk stratification and management . . . 35
7.1.2.1 Trials of implantable cardioverter defibrillator
therapy in dilated cardiomyopathy . . . . . . . . . . . . . . 36
7.1.2.2 Primary prophylaxis . . . . . . . . . . . . . . . . . . . 36
7.1.2.3 Secondary prophylaxis . . . . . . . . . . . . . . . . . 37
7.1.2.4 Cause-specific mortality . . . . . . . . . . . . . . . . 37
7.1.2.5 Management of ventricular arrhythmia in dilated
cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . 37
7.1.2.6 Ablation of ventricular tachycardia . . . . . . . . . 37
7.2 Hypertrophic cardiomyopathy . . . . . . . . . . . . . . . . . . 37
7.2.1 Definitions, epidemiology, and survival data . . . . . . 37
7.2.2 Approach to risk stratification and management . . . 37
7.2.3 Ventricular arrhythmias in hypertrophic
cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
7.2.4 Approach to risk stratification and management in
adults patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
7.2.5 Approach to risk stratification and management in
paediatric patients . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
7.2.6 Prevention of sudden cardiac death . . . . . . . . . . . 38
7.2.6.1 Drugs and lifestyle advice . . . . . . . . . . . . . . . 38
7.2.6.2 Implantable cardioverter defibrillators . . . . . . . 39
7.3 Arrhythmogenic right ventricular cardiomyopathy . . . . . 39
7.3.1 Definitions, epidemiology, and survival . . . . . . . . . 39
7.3.2 Approach to risk stratification and management . . . .39
7.3.3 Ventricular arrhythmias in arrhythmogenic right
ventricular cardiomyopathy . . . . . . . . . . . . . . . . . . . . 39
7.3.3.1 Treatment of ventricular arrhythmia . . . . . . . 40
7.3.3.2 Exercise restriction . . . . . . . . . . . . . . . . . . . 40
7.3.3.3 Implantable cardioverter defibrillators . . . . . . . 40
7.4 Infiltrative cardiomyopathies . . . . . . . . . . . . . . . . . . . 40
7.4.1 Cardiac amyloidosis . . . . . . . . . . . . . . . . . . . . . 40
7.5 Restrictive cardiomyopathy . . . . . . . . . . . . . . . . . . . . 40
7.6 Other cardiomyopathies . . . . . . . . . . . . . . . . . . . . . 41
7.6.1 Left-ventricular non-compaction . . . . . . . . . . . . . 41
7.6.2 Chagas’ cardiomyopathy . . . . . . . . . . . . . . . . . . . 41
8. Inherited primary arrhythmia syndromes . . . . . . . . . . . . . . 41
8.1 Long QT syndrome . . . . . . . . . . . . . . . . . . . . . . . . 41
8.1.1 Definitions and epidemiology . . . . . . . . . . . . . . . 41
8.1.2 Approach to risk stratification and management . . . 42
8.2 Short QT syndrome . . . . . . . . . . . . . . . . . . . . . . . . 43
8.2.1 Definitions and epidemiology . . . . . . . . . . . . . . . 43
8.2.2 Approach to risk stratification and management . . . 43
8.3 Brugada syndrome . . . . . . . . . . . . . . . . . . . . . . . . . 44
8.3.1 Definitions and epidemiology . . . . . . . . . . . . . . . 44
8.3.2 Approach to risk stratification and management . . . 44
8.4 Catecholaminergic polymorphic ventricular tachycardia . 45
8.4.1 Definitions and epidemiology . . . . . . . . . . . . . . . 45
8.4.2 Approach to risk stratification and management . . . 45
8.5 Early repolarization syndrome . . . . . . . . . . . . . . . . . . 46
8.5.1 Definitions and epidemiology . . . . . . . . . . . . . . . 46
9. Paediatric arrhythmias and congenital heart disease . . . . . . . 46
9.1 Management of ventricular arrhythmias in children with a
structurally normal heart . . . . . . . . . . . . . . . . . . . . . . . .
46
9.2 Sudden cardiac death and ventricular arrhythmias in
patients with congenital heart disease . . . . . . . . . . . . . . . .
47
9.3 Implantable cardioverter defibrillator therapy in paediatric
patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
48
10. Ventricular tachycardias and ventricular fibrillation in
structurally normal hearts . . . . . . . . . . . . . . . . . . . . . . . . . .
49
10.1 Outflow tract ventricular tachycardias . . . . . . . . . . . . 49
10.1.1 Right ventricular outflow tract tachycardias . . . . . 50
10.1.2 Left ventricular outflow tract tachycardias . . . . . . 50
10.1.3 Aortic cusp ventricular tachycardias . . . . . . . . . . 50
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10.1.4 Epicardial outflow tract ventricular tachycardias . . 50
10.1.5 Others (including pulmonary arteries) . . . . . . . . . 50
10.2 Ventricular tachycardias of miscellaneous origin . . . . . 50
10.2.1 Idiopathic left ventricular tachycardia . . . . . . . . . . 51
10.2.2 Papillary muscle ventricular tachycardia . . . . . . . . 51
10.2.3 Annular ventricular tachycardia (mitral and
tricuspid) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
10.3 Idiopathic ventricular fibrillation . . . . . . . . . . . . . . . . 51
10.4 Short-coupled torsade de pointes . . . . . . . . . . . . . . 52
11. Inflammatory, rheumatic and valvular heart diseases . . . . . . 52
11.1 Myocarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
11.1.1 Acute and fulminant myocarditis . . . . . . . . . . . . 53
11.1.2 Myocarditis leading to inflammatory
cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
11.2 Endocarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
11.3 Rheumatic heart disease . . . . . . . . . . . . . . . . . . . . . 54
11.4 Pericarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
11.5 Cardiac sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . 54
11.6 Valvular heart disease . . . . . . . . . . . . . . . . . . . . . . 55
12. Arrhythmic risk in selected populations . . . . . . . . . . . . . . 55
12.1 Psychiatric patients . . . . . . . . . . . . . . . . . . . . . . . . 55
12.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . 56
12.1.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
12.1.3 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
12.2 Neurological patients . . . . . . . . . . . . . . . . . . . . . . . 57
12.2.1 Sudden unexplained death in epilepsy . . . . . . . . . 57
12.2.2 Neuromuscular disorders . . . . . . . . . . . . . . . . . 57
12.3 Pregnant patients . . . . . . . . . . . . . . . . . . . . . . . . . 58
12.3.1 Arrhythmias not related to peripartum
cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58
12.3.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . 58
12.3.1.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . 59
12.3.1.3 Treatment . . . . . . . . . . . . . . . . . . . . . . . 59
12.3.2 Arrhythmias related to peripartum cardiomyopathy 59
12.4 Obstructive sleep apnoea . . . . . . . . . . . . . . . . . . . . 60
12.4.1 Bradyarrhythmias and –tachyarrhythmias . . . . . . . 60
12.4.1.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . 60
12.4.1.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . 60
12.4.1.3 Treatment . . . . . . . . . . . . . . . . . . . . . . . . 60
12.5 Drug-related pro-arrhythmia . . . . . . . . . . . . . . . . . . 60
12.5.1 Drug–substrate interaction, due to underlying
disease substrate . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
12.5.2 Drug–drug interaction (due to specific drugs and
combinations) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
12.5.3 Pro-arrhythmic risk of anti-arrhythmic drugs . . . . . 61
12.5.4 Pro-arrhythmia due to triggering factors . . . . . . . 61
12.6 Sudden cardiac death after heart transplantation . . . . 61
12.7 Sudden cardiac death in athletes . . . . . . . . . . . . . . . 61
12.8 Wolff–Parkinson– White syndrome . . . . . . . . . . . . . 62
12.9 Prevention of sudden cardiac death in the elderly . . . . 64
12.10 End-of-life issues . . . . . . . . . . . . . . . . . . . . . . . . . 64
13. Gaps in evidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64
14. To do and not to do messages from the guidelines . . . . . . . 65
15. Web addenda . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
16. Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
17. References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67
Abbreviations and acronyms
ACC American College of Cardiology
ACE angiotensin-converting enzyme
ACS acute coronary syndrome
AF atrial fibrillation
AGNES Arrhythmia Genetics in the Netherlands
AHA American Heart Association
AMIOVIRT AMIOdarone Versus Implantable cardiover -
ter-defibrillator: Randomized Trial in patients
with non-ischaemic dilated cardiomyopathy
and asymptomatic non-su stained ventricular
tachycardia
ARB angiotensin II receptor blocker
ARVC arrhythmogenic right ventricular cardiomyopathy
AV atrio-ventricular
AVID Antiarrhythmic drugs Versus Implantable
Defibrillator
BrS Brugada Syndrome
CAD coronary artery disease
CARE-HF CArdiac REsynchronization – Heart Failure
CASH Cardiac Arrest Study Hamburg
CAST Cardiac Arrhythmia Suppression Trial
CAT CArdiomyopathy Trial
CHD congenital heart disease
CI confidence interval
CIDS Canadian Implantable Defibrillator Study
CMR cardiac magnetic resonance
COMPANION Comparison of Medical Therapy, Pacing, and
Defibrillation in Heart Failure
CPG Committee for Practice Guidelines
CPVT catecholaminergic polymorphic ventricular
tachycardia
CRT cardiac resynchronization therapy
CRT-D cardiac resynchronization therapy defibrillator
CRT-P cardiac resynchronization therapy pacemaker
CT computed tomography
DCM dilated cardiomyopathy
DEFINITE DEFI brill ators in Non-Ischemic cardiomyop-
athy Treatment Evaluation
DFT defibrillation threshold
DIAMOND Dani sh Investigators of Arrhythmia and
Mortality oN Dofetilide
ECG electrocardiogram / electrocardiographic
EHRA European Heart Rhythm Association
EPS electrophysiological study
ESC European Society of Cardiology
GWAS genome-wide association study
HCM hypertrophic cardiomyopathy
HF heart failure
HFpEF heart failure with preserved ejection fraction
HFrEF heart failure with reduced ejection fraction
HR hazard ratio
i.v. intravenous
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ICD implantable cardioverter defibrillator
ILCOR International Liaison Committee On
Resuscitation
IRIS Immediate Risk stratification Improves Survival
LBBB left bundle branch block
LMNA lamin A/C
LQTS long QT syndrome
LQTS1 long QT syndrome type 1
LQTS2 long QT syndrome type 2
LQTS3 long QT syndrome type 3
LV left ventricle / left ventricular
LVEF left ventricular ejection fraction
LVOT left ventricular outflow tract
MADIT Multice nter Automatic Defibrillator Implant-
ation Trial
MIRACLE Multi center InSync Randomized Clinical
Evaluation
MRA mineralocorticoid receptor antagonist
ms millisecond
MUSTT Multicenter UnSustained Tachycardia Trial
NSTEMI non – ST-segment elevation myo cardial
infarction
NSVT non-sustained ventricular tachycardia
NYHA New York Heart Association
OPTIC Optimal Pharmacological Therapy In Cardio-
verter defibrillator patients
OR odds ratio
OT outflow tract
PRESERVE-EF risk stratification in patients with preserved
ejection fraction
PVC premature ventricular complex
PVS programmed ventricular stimulation
QTc corrected QT
RAFT Resynchronization – Defibrillation for Ambu-
latory Heart Failure Trial
RBBB right bundle branch block
RCT randomized controlled trial
REVERSE REsynchronization reVErses Remodeling in
Systolic left vEntricular dysfunction
REVERSE MIRACLE
ICD
Multice nter InSync ICD Randomiz ed Clinical
Evaluation
RR relative risk
RV right ventricular
RVOT right ventricular outflow tract
SA-ECG signal-averaged ECG
SADS sudden arrhythmic death syndrome
SCD sudden cardiac death
SCD-HeFT Sudden Cardiac Death in HEart Failure Trial
SCORE Systematic Coronary Risk Evaluation
SIDS sudden infant death syndrome
SMASH-VT Substrate Mapping and Ablation in Sinus
Rhythm to Halt Ventricular Tachycardia
SPECT single-photon emission computed tomography
SQTS short QT syndrome
STEMI ST-segment elevation myocardial infarction
SUDEP sudden unexpected death in epilepsy
SUDI sudden unexplained death in infancy
SUDS sudden unexplained death syndrome
TdP torsade de pointes
US United States
VA ventricular arrhythmia
VF ventricular fibrillation
VT ventricular tachycardia
VTACH Ventricular Tachycardia Ablation in Coronary
Heart Disease
WCD wearable cardioverter defibrillator
WPW Wolff–Parkinson– White
1. Preamble
Guidelines summarize and evaluate all available evidence on a par-
ticular issue at the time of the writing process, with the aim of assist-
ing health professionals in selecting the best management strategies
for an individual patient with a given condition, taking into account
the impact on outcome, as well as the risk– benefit ratio of particu-
lar diagnostic or therapeutic means. Guidelines and recommenda-
tions should help health professionals to make decisions in their
daily practice. However, the final decisions concerning an individual
patient must be made by the responsibl e health professional(s) in
consultation with the patient and caregiver as appropriate.
A great number of Guidelines have been issued in recent years by
the European Society of Cardiology (ESC) as well as by other soci-
eties and organisations. Because of the impact on clinical practice,
quality criteria for the development of guidelines have been estab-
lished in order to make all decisions transparent to the user. The re-
commendations for formulating and issuing ESC Guidelines can be
found on the ESC website (
http://www.escardio.org/Guidelines-
&-Education/Clinical-Practice-Guidelines/Guideli nes-development/
Writing-ESC-Guidelines
). ESC Guidelines represent the official pos-
ition of the ESC on a given topic and are regularly updated.
Members of this Task Force were selected by the ESC to re-
present profess ional s involved wi th th e med ical c are of pati ents
with this pathology. Selected experts in the field undertook a
comprehensive review of the published evidence for management
(including diagnosis, treatment, prevention and rehabilitation) of
a given condition according to ESC Committee for Practice
Guidelines (CPG) policy. A critical evaluation of diagnostic and
therapeutic procedures was performed, including assessment of
the risk–benefit ratio. Estimates of expected he alth outcomes for
larger populations were included, where data exist. The level of
evidence and the strength of the recommendation of particular
management options were weighed and graded according to prede-
fined scales, as outlined in Tables
1 and 2.
The experts of the writing and reviewing panels provided declara-
tions of interest forms for all relationships that might be perceived as
real or potential sources of conflicts of interest. These forms were
compiled into one file and can be found on the ESC website (
http://
www.escardio.org/guidelines
). Any changes in declarations of interest
that arise during the writing period must be notified to the ESC and
updated. The Task Force received its entire financial support from the
ESC without any involvement from the healthcare industry.
The ESC CPG supervises and coordinates the preparation of new
Guidelines produced by task fo rces, expert groups or consensus
ESC Guidelines Page 5 of 87
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