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1 REVIEW ARTICLES Received: June 21, 2018 Accepted: June 29, 2018 Corresponding Authors: * The first two authors contributed equally to this work. Two authors contributed equally to this work as corresponding author Copyright 2018 The Official Journal of Korean Heart Rhythm Society Editorial Board ABSTRACT The general concept and initial approach to syncope patients have been mentioned in the general sections. This special section describes the clinical characteristics, diagnosis, patient education and treatment for the each syncope. The contents are in order of reflex syncope, orthostatic hypotension, postural orthostatic tachycardia syndrome (POTS), cardiac syncope, and unexplained syncope. Several clinical issues will have been dealt with in special issues. Neurological assessment is added when the patients were diagnosed with psychogenic pseudosyncope (PPS). Although most of syncope in children and adolescents is reflex syncope, they may be caused by cardiovascular events in patients with congenital heart disease. In the elderly patients, syncope occurs not only by a single cause but a combination of various conditions. When they visit the emergency department, a standardized systematic approach is required to determine whether hospitalize them according to the risk and the needs for the specialized syncope management. We also described the limitations put on driving, exercising and social life style that are relevant to syncope. In this guideline, we also reviewed the Korean published literatures and European/American guidelines on syncope. American College of Cardiology ACC American Heart Association AHA Arrhythmogenic right ventricular cardiomyopathy/dysplasia ARVC/D Atrioventicular block Atrioventricular nodal disease Autonomic nervous system Bifascicular block Benefit/risk ratio Brugada syndrome 145

2 Bundle branch block Cardiac syncope Cardiac output Cardioinhibitory Cerebral hypoperfusion Cascade Class of recommendation COR Cognitive behavioral therapy Compression garments Conduction system delay Conversion disorder Convulsive syncope Counter-pressure maneuver Cyanotic breath holding spell Early repolarization Ejection fraction Electrophysiological study EPS Emergency department observation units EDOU Epilepsy European Heart Rhythm Association EHRA European Society of Cardiology ESC Fludrocortisone Hazard ratio Head-up tilt test HUT Heart Rhythm Society HRS Hypertrophic cardiomyopathy Implantable cardioverter defibrillator ICD Implantable loop recorder ILR Ischemic cardiomyopathy Isometric contraction Korean Heart Rhythm Society KHRS Korean Society for Holter and Noninvasive Electrocardiology KSHNE Left cardiac sympathetic denervation 146

3 2018 KHRS guideline for the evaluation and management of syncope Leg crossing Level of evidence LOE Long QT syndrome Loss of consciousness LOC Mineralocorticoid Multiple systemic atrophy Neurally mediated syncope NMS Neurodegenerative disorder Neurogenic orthostatic hypotension Orthostatic hypotension OH Postural orthostatic tachycardia syndrome POTS Pre-excited QRS complexes Primary electrical disease Psychogenic pseudoseizure Psychogenic pseudosyncope PPS Psychogenic transient loss of consciousness Psychogenic non-epileptic seizure PNES Reflex syncope RS Sinus node disease Sinus node recovery time SNRT Situational syncope Squatting Subarachnoid hemorrhage Sudden cardiac death Syncope Syncope management unit Syncope unit Tonic clonic seizure Transient ischemic attack TIA Transient loss of consciousness TLOC Tunnel vision Unexplained syncope Vasodepressor 147

4 Vasovagal syncope VVS Venous return Vertebrobasilar insufficiency Video-EEG monitoring 1. Class of Recommendation (COR) Class of recommendation ClassI Class II Class IIa Class IIb Class III Definition Evidence and/or general agreement that a given treatment or procedure is beneficial, useful, effective. Conflicting evidence and/or divergence of opinion about usefulness/efficacy of the given treatment or procedure. Weight of evidence/opinion is in favor of usefulness/efficacy. Usefulness/efficacy is less well established by evidence/opinion. Evidence or general agreement that the given treatment or procedure is not useful/effective, and in some cases may be harmful. 2. Level of Evidence (LOE) Class of recommendation Level of Evidence A Level of Evidence B Level of Evidence C Definition Data derived from multiple randomized clinical trials or meta-analyses. Data derived from a single randomized clinical trial or large nonrandomized studies. Consensus of opinion of the experts and/or small studies, retrospective studies, registries. 148

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23 2018 KHRS guideline for the evaluation and management of syncope LOC, loss of consciousness; OH: orthostatic hypotension, VVS: vasovagal syncope 167

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33 2018 KHRS guideline for the evaluation and management of syncope Kremastinos DT. Provocation of neurocardiogenic syncope by clomipramine administration during the head-up tilt test in vasovagal syndrome J Am Coll Cardiol 2000;36: ) Marquez MF U-MK, Gomez-Flores J, Sobrino A, Sotomayor- Gonzalez A, Gonzalez-Hermosillo A, Cardenas M. Comparison of metoprolol vs clonazepam as a first treatment choice among patients with neurocardiogenic syncope. Gac Med Mex 2008;144: ) Kanjwal K SB, Karabin B, Kanjwal Y, Grubb BP. Use of octreotide in the treatment of refractory orthostatic intolerance. Am J Ther 2017;19: ) Freeman R, Wieling W, Axelrod FB, et al. Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome. Clin Auton Res 2011;21: ) Zia A, Kamaruzzaman SB, Tan MP. Blood pressure lowering therapy in older people: Does it really cause postural hypotension or falls? Postgrad Med 2015;127: ) McLachlan CY, Yi M, Ling A, Jardine DL. Adverse drug events are a major cause of acute medical admission. Intern Med J 2014;44: ) Poon IO, Braun U. High prevalence of orthostatic hypotension and its correlation with potentially causative medications among elderly veterans. J Clin Pharm Ther 2005;30: ) Huang JJ, Desai C, Singh N, et al. Summer syncope syndrome redux. Am J Med 2015;128: ) Schroeder C, Bush VE, Norcliffe LJ, et al. Water drinking acutely improves orthostatic tolerance in healthy subjects. Circulation 2002;106: ) Jordan J, Shannon JR, Grogan E, Biaggioni I, Robertson D. A potent pressor response elicited by drinking water. Lancet 1999;353: ) Jordan J, Shannon JR, Black BK, et al. The pressor response to water drinking in humans : a sympathetic reflex? Circulation 2000;101: ) Shannon JR, Diedrich A, Biaggioni I, et al. Water drinking as a treatment for orthostatic syndromes. Am J Med 2002;112: ) Claydon VE, Hainsworth R. Salt supplementation improves orthostatic cerebral and peripheral vascular control in patients with syncope. Hypertension 2004;43: ) El-Sayed H, Hainsworth R. Salt supplement increases plasma volume and orthostatic tolerance in patients with unexplained syncope. Heart 1996;75: ) Canney M, O'Connell MD, Murphy CM, et al. Single Agent Antihypertensive Therapy and Orthostatic Blood Pressure Behaviour in Older Adults Using Beat-to-Beat Measurements: The Irish Longitudinal Study on Ageing. PLoS One 2016;11:e ) Verwoert GC, Mattace-Raso FU, Hofman A, et al. Orthostatic hypotension and risk of cardiovascular disease in elderly people: the Rotterdam study. J Am Geriatr Soc 2008;56: ) Valbusa F, Labat C, Salvi P, et al. Orthostatic hypotension in very old individuals living in nursing homes: the PARTAGE study. J Hypertens 2012;30: ) Romero-Ortuno R, O'Connell MD, Finucane C, Soraghan C, Fan CW, Kenny RA. Insights into the clinical management of the syndrome of supine hypertension--orthostatic hypotension (SH- OH): the Irish Longitudinal Study on Ageing (TILDA). BMC Geriatr 2013;13:73. 69) Fogari R, Zoppi A, Mugellini A, et al. Efficacy and safety of two treatment combinations of hypertension in very elderly patients. Arch Gerontol Geriatr 2009;48: ) van Lieshout JJ, ten Harkel AD, Wieling W. Physical manoeuvres for combating orthostatic dizziness in autonomic failure. Lancet 1992;339: ) Ten Harkel AD, van Lieshout JJ, Wieling W. Effects of leg muscle pumping and tensing on orthostatic arterial pressure: a study in normal subjects and patients with autonomic failure. Clin Sci (Lond) 1994;87: ) Tutaj M, Marthol H, Berlin D, Brown CM, Axelrod FB, Hilz MJ. Effect of physical countermaneuvers on orthostatic hypotension in familial dysautonomia. J Neurol 2006;253: ) Podoleanu C, Maggi R, Brignole M, et al. Lower limb and abdominal compression bandages prevent progressive orthostatic hypotension in elderly persons: a randomized single-blind controlled study. J Am Coll Cardiol 2006;48: ) Figueroa JJ, Singer W, Sandroni P, et al. Effects of patientcontrolled abdominal compression on standing systolic blood pressure in adults with orthostatic hypotension. Arch Phys Med Rehabil 2015;96: ) Henry R, Rowe J, O'Mahony D. Haemodynamic analysis of efficacy of compression hosiery in elderly fallers with orthostatic hypotension. Lancet 1999;354:

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