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1 Conflict of Interest and Funding Funding: French National grant («Programme Hospitalier de Recherche Clinique») The authors have no conflicts to declare

2 Outcome of patients with syncope and Early Repolarization pattern A Multi-centric prospective registry Mathieu Le Bloa, Frederic Sacher, MD, Philippe Maury, MD, Jean-Baptiste Gourraud, MD, Philippe Mabot, MD, Christophe Leclercq, MD, Jacques Mansourati, MD, PhD, Dominique Babuty, MD, Meleze Hocini, MD, Gabriel Laurent, MD, Pierre Jaïs, MD, Dominique Lamaison, MD, Jean-Luc Pasquié, MD, PhD, Pierre Bordachar, MD, PhD, Alain Furber, MD, Philippe Ritter, MD, Jean-Sylvain Hermida, MD, Nicolas Derval, MD, Didier Klug, MD, PhD, Arnaud Denis, MD, Philippe Rumeau, MD, Pascal Chavernac, MD, Emanuelle Boiffard, MD, Bertrand Petit, MD, Pierre Hausman MD, Michel Haïssaguerre, MD and Vincent Probst, MD, PhD.

3 Background Early Repolarization Syndrome in inferolateral leads has been associated with Ventricular Fibrillation and sudden cardiac death (Haïssaguerre and al. NEJM 2008, Rosso and al. JACC 2008, Haruta and al.circulation 2011 ) Electrocardiographic phenotypes seem to be associated with different longterm outcomes: ERP location, ST segment (Tikkanen and al. NEJM 2009, Tikkanen and al. Circulation 2011) Association between ERS and SCD is still debated (Uberoi and al. Circulation 2011) heterogeneous risk of sudden cardiac death (SCD), maybe syncope should raise concerns

4 Aim of the study Evaluate prevalence of SCD and/or ventricular arrhythmia (VA) in patient with Early Repolarization Pattern (ERP) after an episode of syncope Clinical and ECG characteristics of patients with VA during follow-up

5 Methods: Population: Unexplained syncope ERP on resting 12 leads ECG J-wave elevation 0,1mV QRS slurring or notching 2 leads in the inferior (II, III, avf) or lateral (I, avl, V5, V6)

6 Methods: Population: Unexplained syncope ERP on resting 12 leads ECG Patient from 17 centers Exclusion: Structural heart disease QTc>440ms in males or >460ms in females

7 Methods: ECG Analysis All 12 lead ECG recorded during hospitalization Blinded independent analysis by 2 cardiologists

8 Methods: Collected data: Age, Sex History of previous unexplained syncope Family history of sudden cardiac death 12-lead ECG Clinical examinations In hospital continuous ECG monitoring Investigations to eliminate heart disease

9 Methods: Management at the appreciation of the referring physician ILR, ICD or clinical follow up depending on clinical characteristics Collected Data: At each patient visit (schedulded, new clinical event) At least every 6 months by phone call

10 Results 76 patients: 17 centres inclusion from 01/01/2009 to 01/08/ males (87%), 10 women (13%) 33 ± 14.5 yo Ethnic origins: Caucasian: 66 (87%) African: 10 (13%) Asian: 0 Previous unexplained syncope: 13 (17%) Family history of SCD: 17 (22%) Intensive sport activity (>10h/week): 5 (7%)

11 Results Echocardiography and 24h ECG monitoring : 76 (100%) =>Ventricular arrhythmia recorded during initial workup: 2 (3%) Prolonged rhythm monitoring: 39 (51%) ILR: 29 (38%) ILR and ICD: 3 (4%) ICD: 7 (14%)

12 ECG characteristics Heart rate: 71 ± 17 beats/min QRS: 92 ± 12.6 ms Conduction abnormalities: 5 (6.6%) AVB 1: 4 (5%) RBB: 1 (1.3%) Sokolov: 2.6 ± 0.9 mv Electrical LVH: 20 (26%) QTc: 392 ± 32.8 ms

13 ERP characteristics ERP Position:

14 ERP characteristics Baseline J-point elevation (mv) [0.1-0,15] 36 (47%) [ ] 27 (36%) (17%)

15 Follow up Clinical follow up: 19 [10-30] months No patient lost to follow up No death Asymptomatic: 72 (95%) Recurrent syncope: 4 (5%) ILR recording in 2 No rhythm anomaly 1 (1%) Sinus pause of 5s 1 (1%) Neurocardiogenic symptoms: 2 (3%)

16 Results 3 (4%) patients with ILR experienced a ventricular arrhythmia recorded on ILR

17 Results Among asymptomatic patient: 2 (3%) ventricular tachycardia during sleep or rest (ILR recording) 1 VT after physical exercise No arrhythmia recorded on ICD

18 Results 5 VT recording in our population (6.6%): 5 males previous unexplained syncope: 2 no family history of SCD ERP characteristics: No QRS fragmentation ERP: 3 inferior, 1 inferolateral and 1 lateral ST segment: ascending : 3 Horizontal/descending: 2

19 Conclusions Syncope could be the first symptom in ERS before VA Syncope could be an element of bad long terme outcome J-wave elevation after an episode of syncope may help identifying people with an increased risk of VA

20 Aknowledgements: Dr Sacher Pr Probst and Dr Gourraud Dr Maury Dr Leclerq Pr Mansourati Dr Giraudeau Dr Rumeau Dr Babuty Dr Laurent Dr Lamaison Dr Pasquié Pr Furber Pr Hermida Dr Klug Dr Petit, Dr Hausman

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