2018ESC Guidelines For The Diagnosisand Management Ofsyncope
European Heart Journal (2018) 00, 1–69doi:10.1093/eurheartj/ehy037ESC GUIDELINES2018 ESC Guidelines for the diagnosis andmanagement of syncopeThe Task Force for the diagnosis and management of syncope ofthe European Society of Cardiology (ESC)Developed with the special contribution of the European HeartRhythm Association (EHRA)Endorsed by: European Academy of Neurology (EAN), EuropeanFederation of Autonomic Societies (EFAS), European Federation ofInternal Medicine (EFIM), European Union Geriatric Medicine Society(EUGMS), European Society of Emergency Medicine (EuSEM)Authors/Task Force Members: Michele Brignole* (Chairperson) (Italy),Angel Moya* (Co-chairperson) (Spain), Frederik J. de Lange (The Netherlands),Jean-Claude Deharo (France), Perry M. Elliott (UK), Alessandra Fanciulli (Austria),Artur Fedorowski (Sweden), Raffaello Furlan (Italy), Rose Anne Kenny (Ireland),Alfonso Mart ın (Spain), Vincent Probst (France), Matthew J. Reed (UK),Ciara P. Rice (Ireland), Richard Sutton (Monaco), Andrea Ungar (Italy), andJ. Gert van Dijk (The Netherlands)* Corresponding authors: Michele Brignole, Department of Cardiology, Ospedali Del Tigullio, Via Don Bobbio 25, IT-16033 Lavagna, (GE) Italy. Tel: þ39 0185 329 567,Fax: þ39 0185 306 506, Email: mbrignole@asl4.liguria.it; Angel Moya, Arrhythmia Unit, Hospital Vall d’Hebron, P Vall d’Hebron 119-129, ES-08035 Barcelona, Spain.Tel: þ34 93 2746166, Fax: þ34 93 2746002, Email: amoyamitjans@gmail.com.ESC Committee for Practice Guidelines (CPG) and National Cardiac Societies document reviewers: listed in the Appendix.1Representing the European Academy of Neurology (EAN)2Representing the European Federation of Internal Medicine (EFIM)3Representing the European Society of Emergency Medicine (EuSEM)ESC entities having participated in the development of this document:Associations: European Heart Rhythm Association (EHRA)Councils: Council on Cardiovascular Nursing and Allied Professions, Council for Cardiology Practice, Council on Cardiovascular Primary CareWorking Groups: Myocardial and Pericardial DiseasesThe 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 theESC Guidelines may be translated or reproduced in any form without written permission from the ESC. Permission can be obtained upon submission of a written request to OxfordUniversity Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC er. The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge and the evidence availableat 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 recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the ESC Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic ortherapeutic medical strategies; however, the ESC Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate andaccurate 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 dothe ESC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competentpublic 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 thehealth professional’s responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.C The European Society of Cardiology 2018. All rights reserved. For permissions please email: journals.permissions@oxfordjournals.orgVDownloaded from -abstract/doi/10.1093/eurheartj/ehy037/4939241by gueston 20 March 2018
2ESC GuidelinesDocument Reviewers: Adam Torbicki (CPG Review Coordinator) (Poland), Javier Moreno (CPG ReviewCoordinator) (Spain), Victor Aboyans (France), Stefan Agewall (Norway), Riccardo Asteggiano (Italy),Jean-Jacques Blanc (France), Natan Bornstein1 (Israel), Serge Boveda (France), Héctor Bueno (Spain),Haran Burri (Switzerland), Antonio Coca (Spain), Jean-Philippe Collet (France), Giorgio Costantino2(Italy), Ernesto D ıaz-Infante (Spain), Victoria Delgado (The Netherlands), Faas Dolmans(The Netherlands), Oliver Gaemperli (Switzerland), Jacek Gajek (Poland), Gerhard Hindricks (Germany),Josef Kautzner (Czech Replublic), Juhani Knuuti (Finland), Piotr Kulakowski (Poland),Ekaterini Lambrinou (Cyprus), Christophe Leclercq (France), Philippe Mabo (France), Carlos A. Morillo(Canada), Massimo Francesco Piepoli (Italy), Marco Roffi (Switzerland), Win K. Shen (USA),Iain A. Simpson (UK), Martin Stockburger (Germany), Peter Vanbrabant3 (Belgium),Stephan Windecker (Switzerland), and Jose Luis Zamorano (Spain)The disclosure forms of all experts involved in the development of these Guidelines are available on theESC website nes Syncope Transient loss of consciousness Vasovagal syncope Reflexsyncope Orthostatic hypotension Cardiac syncope Sudden cardiac death Electrophysiologicalstudy Prolonged ECG monitoring Tilt testing Carotid sinus massage Cardiac pacing Implantablecardioverter defibrillator Syncope unit Emergency departmentTable of Contents1. Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52.1 What is new in the 2018 version? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63. Definitions, classification, and pathophysiology . . . . . . . . . . . . . . . . . . . . . . 73.1 Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73.2 Classification and pathophysiology of syncope and transientloss of consciousness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83.2.1 Syncope. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83.2.2 Non-syncopal forms of (real or apparent) transientloss of consciousness. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84. Diagnostic evaluation and management according torisk stratification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104.1 Initial evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104.1.1 Diagnosis of syncope. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114.1.2 Management of syncope in the emergency departmentbased on risk stratification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134.2 Diagnostic tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 174.2.1 Carotid sinus massage. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 174.2.2 Orthostatic challenge . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184.2.2.1 Active standing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184.2.2.2 Tilt testing. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 204.2.3 Basic autonomic function tests. . . . . . . . . . . . . . . . . . . . . . . . . . . . 214.2.3.1 Valsalva manoeuvre. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214.2.3.2 Deep breathing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214.2.3.3 Other autonomic function tests . . . . . . . . . . . . . . . . . . . 214.2.3.4 Twenty-four-hour ambulatory and homeblood pressure monitoring. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214.2.4 Electrocardiographic monitoring (non-invasiveand invasive) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23.4.2.4.1 In-hospital monitoring . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234.2.4.2 Holter monitoring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234.2.4.3 Prospective external event recorders. . . . . . . . . . . . . . 234.2.4.4 Smartphone applications . . . . . . . . . . . . . . . . . . . . . . . . . . 234.2.4.5 External loop recorders. . . . . . . . . . . . . . . . . . . . . . . . . . . 234.2.4.6 Remote (at home) telemetry . . . . . . . . . . . . . . . . . . . . . . 234.2.4.7 Implantable loop recorders . . . . . . . . . . . . . . . . . . . . . . . 234.2.4.8 Diagnostic criteria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234.2.5 Video recording in suspected syncope . . . . . . . . . . . . . . . . . . . . 254.2.5.1 In-hospital video recording . . . . . . . . . . . . . . . . . . . . . . . . 254.2.5.2 Home video recording . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254.2.6 Electrophysiological study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254.2.6.1 Asymptomatic sinus bradycardia – suspectedsinus arrest causing syncope . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254.2.6.2 Syncope in bifascicular bundle branch block(impending high-degree atrioventricular block) . . . . . . . . . . . . 254.2.6.3 Suspected tachycardia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254.2.7 Endogenous adenosine and other biomarkers . . . . . . . . . . . . . 274.2.7.1 Adenosine (triphosphate) test and plasmaconcentration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 274.2.7.2 Cardiovascular biomarkers. . . . . . . . . . . . . . . . . . . . . . . . . 274.2.7.3 Immunological biomarkers . . . . . . . . . . . . . . . . . . . . . . . . . 274.2.8 Echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 274.2.8.1 Exercise stress echocardiography . . . . . . . . . . . . . . . . . . 274.2.9 Exercise stress testing. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284.2.10 Coronary angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285. Treatment. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285.1 General principles of treatment of syncope. . . . . . . . . . . . . . . . . . . . . 285.2 Treatment of reflex syncope. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295.2.1 Education and lifestyle modifications . . . . . . . . . . . . . . . . . . . . . . 295.2.2 Discontinuation/reduction of hypotensive therapy . . . . . . . . 31Downloaded from -abstract/doi/10.1093/eurheartj/ehy037/4939241by gueston 20 March 2018
3ESC Guidelines5.2.3 Physical counter-pressure manoeuvres . . . . . . . . . . . . . . . . . . . 315.2.4 Tilt training . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315.2.5 Pharmacological therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315.2.5.1 Fludrocortisone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315.2.5.2 Alpha-agonists. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325.2.5.3 Beta-blockers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325.2.5.4 Other drugs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325.2.5.5 Emerging new therapies in specific subgroups . . . . . . . 325.2.6 Cardiac pacing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325.2.6.1 Evidence from trials in suspected orcertain reflex syncope and electrocardiogramdocumented asystole . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325.2.6.2 Evidence from trials in patients with carotidsinus syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335.2.6.3 Evidence from trials in patients with tilt-inducedvasovagal syncope . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335.2.6.4 Evidence from trials in patients withadenosine-sensitive syncope . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345.2.6.5 Choice of pacing mode . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345.2.6.6 Selection of patients for pacing andproposed algorithm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345.3 Treatment of orthostatic hypotension and orthostatic intolerancesyndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375.3.1 Education and lifestyle measures. . . . . . . . . . . . . . . . . . . . . . . . . . 375.3.2 Adequate hydration and salt intake . . . . . . . . . . . . . . . . . . . . . . . 375.3.3 Discontinuation/reduction of vasoactive drugs . . . . . . . . . . . . 375.3.4 Counter-pressure manoeuvres. . . . . . . . . . . . . . . . . . . . . . . . . . . 375.3.5 Abdominal binders and/or support stockings . . . . . . . . . . . . . . 375.3.6 Head-up tilt sleeping . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375.3.7 Midodrine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375.3.8 Fludrocortisone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375.3.9 Additional therapies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385.3.10 Emerging new pharmacological therapy in specificsubgroups. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385.4 Cardiac arrhythmias as the primary cause . . . . . . . . . . . . . . . . . . . . . . 385.4.1 Syncope due to intrinsic sinoatrial or atrioventricularconduction system disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385.4.1.1 Sinus node disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385.4.1.2 Atrioventricular conduction system disease. . . . . . . . . 395.4.1.3 Bundle branch block and unexplained syncope . . . . . . 395.4.2 Syncope due to intrinsic cardiac tachyarrhythmias . . . . . . . . . 405.4.2.1 Paroxysmal supraventricular tachycardia. . . . . . . . . . . . 415.4.2.2 Paroxysmal ventricular tachycardia . . . . . . . . . . . . . . . . . 415.5 Treatment of syncope secondary to structural cardiac,cardiopulmonary, and great vessel disease . . . . . . . . . . . . . . . . . . . . . . . . . 425.6 Treatment of unexplained syncope in patients at high risk ofsudden cardiac death . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425.6.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425.6.2 Left ventricular systolic dysfunction . . . . . . . . . . . . . . . . . . . . . . . 425.6.3 Hypertrophic cardiomyopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . 435.6.4 Arrhythmogenic right ventricular cardiomyopathy. . . . . . . . . 435.6.5 Patients with inheritable arrhythmogenic disorders . . . . . . . . 435.6.5.1 Long QT syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435.6.5.2 Brugada syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445.6.5.3 Other forms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446. Special issues . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446.1 Syncope in patients with comorbidity and frailty . . . . . . . . . . . . . . . . 44.6.1.1 Comorbidity and polypharmacy . . . . . . . . . . . . . . . . . . . . . . . . . . 446.1.2 Falls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 456.1.3 Cognitive assessment and physical performance tests. . . . . . 456.2 Syncope in paediatric patients. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 466.2.1 Diagnostic evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 466.2.2 Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467. Psychogenic transient loss of consciousness and its evaluation. . . . . . . 467.1 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467.1.1 Historical criteria for attacks. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467.1.2 Documentation of key features during an attack . . . . . . . . . . . 467.1.2.1 Management of psychogenic pseudosyncope . . . . . . . 478. Neurological causes and mimics of syncope . . . . . . . . . . . . . . . . . . . . . . . . 478.1 Clinical conditions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 478.1.1 Autonomic failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 478.1.2 Epilepsy and ictal asystole . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 478.1.3 Cerebrovascular disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488.1.4 Migraine. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 498.1.5 Cataplexy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 498.1.6 Drop attacks . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 498.2 Neurological tests. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 498.2.1 Electroencephalography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 508.2.2 Brain computed tomography and magneticresonance imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 508.2.3 Neurovascular studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 508.2.4 Blood tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509. Organizational aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509.1 Syncope (transient loss of consciousness) management unit . . . . . 509.1.1 Definition of a syncope unit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509.1.2 Definition of syncope specialist . . . . . . . . . . . . . . . . . . . . . . . . . . . 509.1.3 Goal of a syncope unit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509.1.4 Model of a syncope unit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529.1.
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