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37 results on '"Surget, Elodie"'

1. Distinct Substrates of Idiopathic Ventricular Fibrillation Revealed by Arrhythmia Characteristics on Implantable Cardioverter-Defibrillator

Author

Haïssaguerre, Michel, Sellal, Jean-Marc, Benali, Karim, de Becker, Benjamin, Defaye, Pascal, Pascale, Patrizio, Martins, Raphael, Mabo, Philippe, Xhaet, Olivier, Extramiana, Fabrice, Surget, Elodie, Lavergne, Thomas, Marijon, Eloi, Adragao, Pedro, Carvalho, Maria Salomé, Milliez, Paul-Ursmar, Laredo, Mickael, Gandjbakhch, Estelle, Giustetto, Carla, Gaita, Fiorenzo, Tilz, Roland, Jesel-Morel, Laurence, Steinfurt, Johannes, Arentz, Thomas, Knecht, Sebastien, Duytschaever, Mattias, Roten, Laurent, Reichlin, Tobias, Fatemi, Marjaneh, Mansourati, Jacques, Kouakam, Claude, Bessière, Francis, Chevalier, Philippe, Tadros, Rafik, Macle, Laurent, Gallego, Francisco, Hadjis, Alexios, Sacher, Frederic, Pereira, Dylan, Hourdain, Jerome, Deharo, Jean-Claude, Eschalier, Romain, Massoulié, Grégoire, Maury, Philippe, Latcu, Decebal Gabriel, Anselme, Frederic, Duchateau, Josselin, Tixier, Romain, Nademanee, Koonlawee, Nogami, Akihiko, de Groot, Natasja, Vigmond, Edward, Bernus, Olivier, Strik, Marc, Bordachar, Pierre, Cathala, Aude, Bouteiller, Xavier, Dubois, Remi, and Ploux, Sylvain

Published
2024
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3. Type 3 long QT syndrome: Is the effectiveness of treatment with beta-blockers population-specific?

Author

Hermida, Alexis, Gourraud, Jean-Baptiste, Denjoy, Isabelle, Fressart, Véronique, Kyndt, Florence, Maltret, Alice, Khraiche, Diala, Klug, Didier, Mabo, Philippe, Sacher, Frédéric, Maury, Philippe, Winum, Pierre, Defaye, Pascal, Clerici, Gael, Babuty, Dominique, Elbez, Yedid, Morgat, Charles, Surget, Elodie, Messali, Anne, De Jode, Patrick, Clédel, Aurélien, Minois, Damien, Maison-Blanche, Pierre, Bloch, Adrien, Leenhardt, Antoine, Probst, Vincent, and Extramiana, Fabrice

Published
2024
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4. Left Ventricular Abnormal Substrate in Brugada Syndrome

Author

Cheniti, Ghassen, Haissaguerre, Michel, Dina, Christian, Kamakura, Tsukasa, Duchateau, Josselin, Sacher, Frederic, Racine, Hugo-Pierre, Surget, Elodie, Simonet, Floriane, Gourraud, Jean-Baptiste, Sridi, Soumaya, Cochet, Hubert, Andre, Clementine, Bouyer, Benjamin, Chauvel, Remi, Tixier, Romain, Derval, Nicolas, Pambrun, Thomas, Dubois, Remi, Jais, Pierre, Nademanee, Koonlawee, Redon, Richard, Schott, Jean-Jacques, Probst, Vincent, Hocini, Meleze, Barc, Julien, and Bernus, Olivier

Published
2023
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6. Malignant Purkinje ectopy induced by sodium channel blockers

Author

Escande, William, Gourraud, Jean-Baptiste, Haissaguerre, Michel, Gandjbakhch, Estelle, Lavergne, Thomas, Martins, Raphael, Cheniti, Ghassen, Krisai, Philipp, Hermida, Jean Sylvain, Maury, Philippe, Merino, Jose-Louis, Pasquié, Jean-Luc, Combes, Nicolas, Surget, Elodie, Duchateau, Josselin, Pambrun, Thomas, Derval, Nicolas, Hocini, Mélèze, Jaïs, Pierre, Postema, Pieter G., Nademanee, Koonlawee, Vigmond, Ed, Bernus, Olivier, Sacher, Frederic, and Probst, Vincent

Published
2022
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9. Sex differences in the origin of Purkinje ectopy-initiated idiopathic ventricular fibrillation

Author

Surget, Elodie, Cheniti, Ghassen, Ramirez, F. Daniel, Leenhardt, Antoine, Nogami, Akihiko, Gandjbakhch, Estelle, Extramiana, Fabrice, Hidden-Lucet, Françoise, Pillois, Xavier, Benoist, David, Krisai, Philipp, Nakatani, Yosuke, Nakashima, Takashi, Takagi, Takamitsu, Kamakura, Tsukasa, André, Clémentine, Welte, Nicolas, Chauvel, Rémi, Tixier, Romain, Duchateau, Josselin, Pambrun, Thomas, Derval, Nicolas, Jaïs, Pierre, Sacher, Frédéric, Bernus, Olivier, Hocini, Mélèze, and Haïssaguerre, Michel

Published
2021
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10. Genetic characterization of KCNQ1 variants improves risk stratification in type 1 long QT syndrome patients.

Author

Morgat, Charles, Fressart, Véronique, Porretta, Alessandra Pia, Neyroud, Nathalie, Messali, Anne, Temmar, Yassine, Algalarrondo, Vincent, Surget, Elodie, Bloch, Adrien, Leenhardt, Antoine, Denjoy, Isabelle, and Extramiana, Fabrice

Abstract

Aims KCNQ1 mutations cause QTc prolongation increasing life-threatening arrhythmias risks. Heterozygous mutations [type 1 long QT syndrome (LQT1)] are common. Homozygous KCNQ1 mutations cause type 1 Jervell and Lange–Nielsen syndrome (JLNS) with deafness and higher sudden cardiac death risk. KCNQ1 variants causing JLNS or LQT1 might have distinct phenotypic expressions in heterozygous patients. The aim of this study is to evaluate QTc duration and incidence of long QT syndrome–related cardiac events according to genetic presentation. Methods and results We enrolled LQT1 or JLNS patients with class IV/V KCNQ1 variants from our inherited arrhythmia clinic (September 1993 to January 2023). Medical history, ECG, and follow-up were collected. Additionally, we conducted a thorough literature review for JLNS variants. Survival curves were compared between groups, and multivariate Cox regression models identified genetic and clinical risk factors. Among the 789 KCNQ1 variant carriers, 3 groups were identified: 30 JLNS, 161 heterozygous carriers of JLNS variants (HTZ-JLNS), and 550 LQT1 heterozygous carriers of non-JLNS variants (HTZ-Non-JLNS). At diagnosis, mean age was 3.4 ± 4.7 years for JLNS, 26.7 ± 21 years for HTZ-JLNS, and 26 ± 21 years for HTZ-non-JLNS; 55.3% were female; and the mean QTc was 551 ± 54 ms for JLNS, 441 ± 32 ms for HTZ-JLNS, and 467 ± 36 ms for HTZ-Non-JLNS. Patients with heterozygous JLNS mutations (HTZ-JLNS) represented 22% of heterozygous KCNQ1 variant carriers and had a lower risk of cardiac events than heterozygous non-JLNS variant carriers (HTZ-Non-JLNS) [hazard ratio (HR) = 0.34 (0.22–0.54); P < 0.01]. After multivariate analysis, four genetic parameters were independently associated with events: haploinsufficiency [HR = 0.60 (0.37–0.97); P = 0.04], pore localization [HR = 1.61 (1.14–1.2.26); P < 0.01], C-terminal localization [HR = 0.67 (0.46–0.98); P = 0.04], and group [HR = 0.43 (0.27–0.69); P < 0.01]. Conclusion Heterozygous carriers of JLNS variants have a lower risk of cardiac arrhythmic events than other LQT1 patients. [ABSTRACT FROM AUTHOR]

Published
2024
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12. Type 3 long QT syndrome: is the effectiveness of treatment with beta-blockers population-specific?

Author

Hermida, Alexis, primary, Gourraud, Jean-Baptiste, additional, Denjoy, Isabelle, additional, Fressart, Véronique, additional, Kyndt, Florence, additional, Maltret, Alice, additional, Khraiche, Diala, additional, Klug, Didier, additional, Mabo, Philippe, additional, Sacher, Frédéric, additional, Maury, Philippe, additional, Winum, Pierre, additional, Defaye, Pascal, additional, Clerici, Gael, additional, Babuty, Dominique, additional, Elbez, Yedid, additional, Morgat, Charles, additional, Surget, Elodie, additional, Messali, Anne, additional, De Jode, Patrick, additional, Clédel, Aurélien, additional, Minois, Damien, additional, Maison-Blanche, Pierre, additional, Bloch, Adrien, additional, Leenhardt, Antoine, additional, Probst, Vincent, additional, and Extramiana, Fabrice, additional

Published
2023
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18. A case study: High resolution optical mapping of a 19 year old male victim of sudden cardiac death during a marathon

Author

Pallares-Lupon, Néstor, primary, Surget, Elodie, additional, Martinez, Marine E., additional, Renard, Estelle, additional, Guillot, Bastien, additional, Constantin, Marion, additional, Rogier, Julien, additional, Hocini, Mélèze, additional, Haissaguerre, Michel, additional, Bernus, Olivier, additional, and Walton, Richard, additional

Published
2022
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21. Strategy for repeat procedures in patients with persistent atrial fibrillation: Systematic linear ablation with adjunctive ethanol infusion into the vein of Marshall versus electrophysiology‐guided ablation

Author

Nakashima, Takashi, primary, Pambrun, Thomas, additional, Vlachos, Konstantinos, additional, Goujeau, Cyril, additional, André, Clémentine, additional, Krisai, Philipp, additional, Ramirez, F. Daniel, additional, Pintican, Gabriela, additional, Kamakura, Tsukasa, additional, Takagi, Takamitsu, additional, Nakatani, Yosuke, additional, Surget, Elodie, additional, Cheniti, Ghassen, additional, Tixier, Romain, additional, Chauvel, Remi, additional, Duchateau, Josselin, additional, Sacher, Frédéric, additional, Cochet, Hubert, additional, Hocini, Mélèze, additional, Haïssaguerre, Michel, additional, Jaïs, Pierre, additional, and Derval, Nicolas, additional

Published
2022
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23. Purkinje network and myocardial substrate at the onset of human ventricular fibrillation: implications for catheter ablation

Author

Haissaguerre, Michel, primary, Cheniti, Ghassen, additional, Hocini, Meleze, additional, Sacher, Frederic, additional, Ramirez, F. Daniel, additional, Cochet, Hubert, additional, Bear, Laura, additional, Tixier, Romain, additional, Duchateau, Josselin, additional, Walton, Rick, additional, Surget, Elodie, additional, Kamakura, Tsukasa, additional, Marchand, Hugo, additional, Derval, Nicolas, additional, Bordachar, Pierre, additional, Ploux, Sylvain, additional, Takagi, Takamitsu, additional, Pambrun, Thomas, additional, Jais, Pierre, additional, Labrousse, Louis, additional, Strik, Mark, additional, Ashikaga, Hiroshi, additional, Calkins, Hugh, additional, Vigmond, Ed, additional, Nademanee, Koonlawee, additional, Bernus, Olivier, additional, and Dubois, Remi, additional

Published
2022
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24. Malignant Purkinje ectopy induced by sodium channel blockers

Author

Escande, William, Gourraud, Jean-Baptiste, Haissaguerre, Michel, Gandjbakhch, Estelle, Lavergne, Thomas, Martins, Raphael, Cheniti, Ghassen, Krisai, Philipp, Hermida, Jean Sylvain, Maury, Philippe, Merino, Jose-Louis, Pasquié, Jean-Luc, Combes, Nicolas, Surget, Elodie, Duchateau, Josselin, Pambrun, Thomas, Derval, Nicolas, Hocini, M. lèze, Jaïs, Pierre, Postema, Pieter G., Nademanee, Koonlawee, Vigmond, Ed, Bernus, Olivier, Sacher, Frederic, Probst, Vincent, IHU-LIRYC, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux], Institut du Thorax [Nantes], Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Institut de Cardiométabolisme et Nutrition = Institute of Cardiometabolism and Nutrition [CHU Pitié Salpêtrière] (IHU ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), CHU Pontchaillou [Rennes], Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Mécanismes physiopathologiques et conséquences des calcifications vasculaires - UR UPJV 7517 (MP3CV), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cardiology, and ACS - Heart failure & arrhythmias

Subjects
Adult, Male, Ajmaline, Flecainide, Idiopathic ventricular fibrillation, Reproducibility of Results, Middle Aged, Purkinje system, Ventricular Premature Complexes, Electrocardiography, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Physiology (medical), Catheter Ablation, Tachycardia, Ventricular, Humans, [SDV.IB]Life Sciences [q-bio]/Bioengineering, Cardiology and Cardiovascular Medicine, Premature ventricular complex, Sodium Channel Blockers
Abstract

International audience; Background: Sodium channel blocker (SCB) infusion is used to unmask the electrocardiographic pattern of Brugada syndrome. The test may also induce premature ventricular complexes (PVCs) in individuals without Brugada pattern, the clinical relevance of which is little known.Objective: The purpose of this study was to describe the prevalence of short-coupled (Sc) PVCs induced by ajmaline or flecainide in patients with suspected or documented severe ventricular arrhythmias.Methods: We reviewed the SCB tests performed in 335 patients with suspected ventricular arrhythmias and structurally normal hearts in 9 centers. ScPVCs were defined as frequent and repetitive PVCs with an R-on-T pattern on SCB tests. Repeated SCB tests were performed in 7 patients and electrophysiological mapping of ScPVCs in 9.Results: Sixteen patients (8 men; mean age 36 ± 11 years) showed ScPVCs and were included. ScPVCs appeared 229 ± 118 seconds after the initiation of infusion and displayed coupling intervals of 288 ± 28 ms. ScPVC patterns were monomorphic in 12 patients, originating from the Purkinje system in mapped patients. Repetitive PVCs were induced in 15 patients (94%) including polymorphic ventricular tachycardias in 9 (56%). SCB infusion was repeated 45 (interquartile range 0.6-46) months later and induced identical ScPVC in all. SCB test was the only mean to reveal the malignant arrhythmia in 6 patients. Catheter ablation was performed in 9 patients, resulting in arrhythmia elimination in 8 with a follow-up of 6 (interquartile range 2-9) years.Conclusion: SCB can induce ScPVC, mostly from Purkinje tissue, in a small subset of patients with idiopathic ventricular arrhythmias. Its high reproducibility suggests a distinct individual mechanism.

Published
2021

25. Sex differences in ventricular arrhythmia: epidemiology, pathophysiology and catheter ablation

Author

Krisai, Philipp, primary, Cheniti, Ghassen, primary, Takagi, Takamitsu, primary, Kamakura, Tsukasa, primary, Surget, Elodie, primary, André, Clémentine, primary, Duchateau, Josselin, primary, Pambrun, Thomas, primary, Derval, Nicolas, primary, Sacher, Frédéric, primary, Jaïs, Pierre, primary, Haïssaguerre, Michel, primary, and Hocini, Mélèze, primary

Published
2022
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26. B-PO05-013 A CASE STUDY: HIGH RESOLUTION OPTICAL MAPPING OF A 19 YEAR OLD MALE VICTIM OF SUDDEN CARDIAC DEATH DURING A MARATHON

Author

Lupon, Néstor Pallares, primary, Surget, Elodie, additional, Martinez, Marine E., additional, Renard, Estelle, additional, Guillot, Bastien, additional, Constantin, Marion, additional, Rogier, Julien, additional, Hocini, Meleze, additional, Haissaguerre, Michel, additional, Bernus, Olivier, additional, and Walton, Richard D., additional

Published
2021
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27. B-AB01-01 LOCAL REPOLARIZATION HETEROGENEITY IS MORE ARRHYTHMOGENIC THAN STRUCTURAL AND FUNCTIONAL CONDUCTION HETEROGENEITIES IN AN EX VIVO PORCINE MODEL

Author

Renard, Estelle, primary, Surget, Elodie, additional, Michel, Cindy, additional, Benoist, David, additional, Dubes, Virginie, additional, Walton, Richard D., additional, Martinez, Marine E., additional, Guillot, Bastien, additional, Constantin, Marion, additional, Hocini, Meleze, additional, Haissaguerre, Michel, additional, and Bernus, Olivier, additional

Published
2021
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28. B-PO04-171 SUBTLE ABNORMALITIES OF REPOLARIZATION IN PATIENTS WITH IDIOPATHIC VF

Author

Surget, Elodie, primary, Cheniti, Ghassen, additional, Nakatani, Yosuke, additional, Nakashima, Takashi, additional, Takagi, Takamitsu, additional, Kamakura, Tsukasa, additional, Krisai, Philipp, additional, André, Clémentine, additional, Welte, Nicolas, additional, Chauvel, Rémi, additional, Tixier, Romain, additional, Duchateau, Josselin, additional, Pambrun, Thomas, additional, Derval, Nicolas, additional, Jais, Pierre, additional, Sacher, Frederic, additional, Hocini, Meleze, additional, and Haissaguerre, Michel, additional

Published
2021
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29. Local abnormal ventricular activity detection in scar‐related VT: Microelectrode versus conventional bipolar electrode

Author

Nakashima, Takashi, primary, Cheniti, Ghassen, additional, Takagi, Takamitsu, additional, Vlachos, Konstantinos, additional, Goujeau, Cyril, additional, André, Clémentine, additional, Krisai, Philipp, additional, Ramirez, F. Daniel, additional, Pintican, Gabriela, additional, Kamakura, Tsukasa, additional, Nakatani, Yosuke, additional, Surget, Elodie, additional, Roux, Jean‐Rodolphe, additional, Meillet, Valentin, additional, Carapezzi, Aline, additional, Tixier, Romain, additional, Chauvel, Remi, additional, Pambrun, Thomas, additional, Duchateau, Josselin, additional, Derval, Nicolas, additional, Pillois, Xavier, additional, Cochet, Hubert, additional, Hocini, Mélèze, additional, Haïssaguerre, Michel, additional, Jaïs, Pierre, additional, and Sacher, Frédéric, additional

Published
2021
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30. Inherited Cardiomyopathies Revealed by Clinically Suspected Myocarditis

Author

Ader, Flavie, Surget, Elodie, Charron, Philippe, Redheuil, Alban, Zouaghi, Amir, Maltret, Alice, Marijon, Eloi, Denjoy, Isabelle, Hermida, Alexis, Fressart, Véronique, Gandjbakhch, Estelle, Unité Fonctionnelle de Cardiogénétique et Myogénétique Moléculaire et Cellulaire, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Service de Biochimie Métabolique [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Institut de cardiologie [CHU Pitié-Salpêtrière], Service de Génétique Cytogénétique et Embryologie [CHU Pitié-Salpêtrière], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), and CHU Amiens-Picardie

Subjects
[SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2020

31. Evaluation of the QT interval in patients with drug‐induced QT prolongation and torsades de pointes

Author

Krisai, Philipp, primary, Vlachos, Konstantinos, additional, Ramirez, F. Daniel, additional, Nakatani, Yosuke, additional, Nakashima, Takashi, additional, Takagi, Takamitsu, additional, Kamakura, Tsukasa, additional, Surget, Elodie, additional, André, Clémentine, additional, Cheniti, Ghassen, additional, Welte, Nicolas, additional, Chauvel, Rémi, additional, Tixier, Romain, additional, Duchateau, Josselin, additional, Pambrun, Thomas, additional, Derval, Nicolas, additional, Hocini, Mélèze, additional, Jaïs, Pierre, additional, Haïssaguerre, Michel, additional, and Sacher, Frédéric, additional

Published
2020
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32. Idiopathic Ventricular Fibrillation

Author

Haïssaguerre, Michel, primary, Duchateau, Josselin, additional, Dubois, Remi, additional, Hocini, Mélèze, additional, Cheniti, Ghassen, additional, Sacher, Frederic, additional, Lavergne, Thomas, additional, Probst, Vincent, additional, Surget, Elodie, additional, Vigmond, Ed, additional, Welte, Nicolas, additional, Chauvel, Remi, additional, Derval, Nicolas, additional, Pambrun, Thomas, additional, Jais, Pierre, additional, Nademanee, Wee, additional, and Bernus, Olivier, additional

Published
2020
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33. Étudier des numérations orales en classe : quels savoirs mathématiques et langagiers ?

Author

Poisard, Caroline, Kervran, Martine, Surget, Elodie, Moumin, Estelle, Centre de Recherche sur l'Education, les apprentissages et la didactique (CREAD EA 3875), Université de Brest (UBO)-Université de Rennes 2 (UR2), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut Brestois des Sciences de l'Homme et de la Société (IBSHS), Université de Brest (UBO), and Académie de Rennes

Subjects
numération de position, [MATH.MATH-GM]Mathematics [math]/General Mathematics [math.GM], multilingualism, numérations orales, [SHS.EDU]Humanities and Social Sciences/Education, language awareness, didactique des mathématiques, oral number system, éveil aux langues, place-value system, mathematics education, plurilinguisme
Abstract

International audience; This paper deals with a multilingual approach of language awareness to teach and learn mathematics. The analysis of oral number systems in four languages (French, English, Breton, Maori) shows linguistic particularities to "say numbers". Numbers decompositions (additive and multiplicative) are proposed in the four languages regarding to place-value system with the usual polynomial decomposition. The notion of regular oral system number is as well presented. Cultural and linguistic aspects concern in particular written comprehension and the notion of translation. The multilingual approach allows to look beyond the language and culture of school and to consider mathematics through linguistic particularities.; Cet article propose une approche plurilingue d'éveil aux langues pour enseigner et apprendre les mathématiques. L'analyse des numérations orales en quatre langues (français, anglais, breton et maori) montre des spécificités linguistiques pour « dire les nombres ». Des décompositions de nombres (additives et multiplicatives) sont proposées dans les quatre langues et mises au regard de la numération décimale de position avec la décomposition polynomiale usuelle. La notion de numération orale régulière est également présentée. Les aspects culturels et langagiers concernent en particulier la compréhension écrite et la notion de traduction. L'approche plurilingue permet de se décentrer de la langue et de la culture de l'école et d'envisager les mathématiques au regard de spécificités linguistiques.

Published
2018

34. Remote monitoring and early detection of sub-clinical atrial fibrillation in implantable cardioverter defibrillator recipients: incidence, risk factors and prognostic significance (Preprint)

Author

Surget, Elodie, primary, Kouakam, Claude, additional, Sandro, Ninni, additional, Finat, Loic, additional, Klein, Cédric, additional, Marquié, Christelle, additional, Potelle, Charlotte, additional, Brigadeau, François, additional, Klug, Didier, additional, Lacroix, Dominique, additional, and Guédon-Moreau, Laurence, additional

Published
2018
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35. Contribution of exome sequencing for genetic diagnostic in arrhythmogenic right ventricular cardiomyopathy/dysplasia

Author

Fedida, Joel, Fressart, Veronique, Charron, Philippe, Surget, Elodie, Hery, Tiphaine, Richard, Pascale, Donal, Erwan, Keren, Boris, Duthoit, Guillaume, Hidden-Lucet, Françoise, Villard, Eric, Gandjbakhch, Estelle, Institut de cardiologie [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de cardiologie et maladies vasculaires [CHU Ambroise Paré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Ambroise Paré [AP-HP], CHU Pontchaillou [Rennes], HAL UPMC, Gestionnaire, Service de Biochimie Métabolique [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], and Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN)

Subjects
Male, Genetic Screens, Molecular biology, Gene Identification and Analysis, lcsh:Medicine, Database and Informatics Methods, Sequencing techniques, Medicine and Health Sciences, Exome, Gene Regulatory Networks, DNA sequencing, lcsh:Science, Arrhythmogenic Right Ventricular Dysplasia, Sequence Deletion, RNA-Binding Proteins, Genomics, Middle Aged, Genomic Databases, Pedigree, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Deletion Mutation, Female, Cardiomyopathies, Research Article, Adult, Adolescent, Cardiology, [SDV.GEN.GH] Life Sciences [q-bio]/Genetics/Human genetics, Electron Transport Complex IV, Young Adult, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Presenilin-1, Genetics, Humans, Genetic Predisposition to Disease, Aged, lcsh:R, Dideoxy DNA sequencing, Biology and Life Sciences, Computational Biology, Human Genetics, Sequence Analysis, DNA, Genome Analysis, Research and analysis methods, Molecular biology techniques, Biological Databases, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Mutation, Mutation Databases, Trans-Activators, lcsh:Q, Plakophilins, Genome-Wide Association Study
Abstract

International audience; Background: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited cardiomyopathy mainly caused by heterozygous desmosomal gene mutations, the major gene being PKP2. The genetic cause remains unknown in ~50% of probands with routine desmosomal gene screening. The aim of this study was to assess the diagnostic accuracy of whole exome sequencing (WES) in ARVC/D with negative genetic testing.Methods: WES was performed in 22 patients, all without a mutation identified in desmosomal genes. Putative pathogenic variants were screened in 96 candidate genes associated with other cardiomyopathies/channelopathies. The sequencing coverage depth of PKP2, DSP, DSG2, DSC2, JUP and TMEM43 exons was compared to the mean coverage distribution to detect large insertions/deletions. All suspected deletions were verified by real-time qPCR, Multiplex-Ligation-dependent-Probe-Amplification (MLPA) and cGH-Array. MLPA was performed in 50 additional gene-negative probands.Results: Coverage-depth analysis from the 22 WES data identified two large heterozygous PKP2 deletions: one from exon 1 to 14 and one restricted to exon 4, confirmed by qPCR and MLPA. MLPA identified 2 additional PKP2 deletions (exon 1–7 and exon 1–14) in 50 additional probands confirming a significant frequency of large PKP2 deletions (5.7%) in gene-negative ARVC/D. Putative pathogenic heterozygous variants in EYA4, RBM20, PSEN1, and COX15 were identified in 4 unrelated probands.Conclusion: A rather high frequency (5.7%) of large PKP2 deletions, undetectable by Sanger sequencing, was detected as the cause of ARVC/D. Coverage-depth analysis through next-generation sequencing appears accurate to detect large deletions at the same time than conventional putative mutations in desmosomal and cardiomyopathy-associated genes.

Published
2017

36. Genetic characterization of KCNQ1 variants improves risk stratification in type 1 long QT syndrome patients.

Author

Morgat C, Fressart V, Porretta AP, Neyroud N, Messali A, Temmar Y, Algalarrondo V, Surget E, Bloch A, Leenhardt A, Denjoy I, and Extramiana F

Subjects
Humans, Female, Male, Risk Assessment, Risk Factors, Child, Electrocardiography, Child, Preschool, Heterozygote, Mutation, Jervell-Lange Nielsen Syndrome genetics, Jervell-Lange Nielsen Syndrome physiopathology, Genetic Predisposition to Disease, Infant, Adult, Adolescent, Phenotype, Retrospective Studies, Death, Sudden, Cardiac etiology, Young Adult, Incidence, KCNQ1 Potassium Channel genetics, Romano-Ward Syndrome genetics, Romano-Ward Syndrome physiopathology, Romano-Ward Syndrome diagnosis
Abstract

Aims: KCNQ1 mutations cause QTc prolongation increasing life-threatening arrhythmias risks. Heterozygous mutations [type 1 long QT syndrome (LQT1)] are common. Homozygous KCNQ1 mutations cause type 1 Jervell and Lange-Nielsen syndrome (JLNS) with deafness and higher sudden cardiac death risk. KCNQ1 variants causing JLNS or LQT1 might have distinct phenotypic expressions in heterozygous patients. The aim of this study is to evaluate QTc duration and incidence of long QT syndrome-related cardiac events according to genetic presentation., Methods and Results: We enrolled LQT1 or JLNS patients with class IV/V KCNQ1 variants from our inherited arrhythmia clinic (September 1993 to January 2023). Medical history, ECG, and follow-up were collected. Additionally, we conducted a thorough literature review for JLNS variants. Survival curves were compared between groups, and multivariate Cox regression models identified genetic and clinical risk factors. Among the 789 KCNQ1 variant carriers, 3 groups were identified: 30 JLNS, 161 heterozygous carriers of JLNS variants (HTZ-JLNS), and 550 LQT1 heterozygous carriers of non-JLNS variants (HTZ-Non-JLNS). At diagnosis, mean age was 3.4 ± 4.7 years for JLNS, 26.7 ± 21 years for HTZ-JLNS, and 26 ± 21 years for HTZ-non-JLNS; 55.3% were female; and the mean QTc was 551 ± 54 ms for JLNS, 441 ± 32 ms for HTZ-JLNS, and 467 ± 36 ms for HTZ-Non-JLNS. Patients with heterozygous JLNS mutations (HTZ-JLNS) represented 22% of heterozygous KCNQ1 variant carriers and had a lower risk of cardiac events than heterozygous non-JLNS variant carriers (HTZ-Non-JLNS) [hazard ratio (HR) = 0.34 (0.22-0.54); P < 0.01]. After multivariate analysis, four genetic parameters were independently associated with events: haploinsufficiency [HR = 0.60 (0.37-0.97); P = 0.04], pore localization [HR = 1.61 (1.14-1.2.26); P < 0.01], C-terminal localization [HR = 0.67 (0.46-0.98); P = 0.04], and group [HR = 0.43 (0.27-0.69); P < 0.01]., Conclusion: Heterozygous carriers of JLNS variants have a lower risk of cardiac arrhythmic events than other LQT1 patients., Competing Interests: Conflict of interest: none declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Published
2024
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37. Inherited Cardiomyopathies Revealed by Clinically Suspected Myocarditis: Highlights From Genetic Testing.

Author

Ader F, Surget E, Charron P, Redheuil A, Zouaghi A, Maltret A, Marijon E, Denjoy I, Hermida A, Fressart V, and Gandjbakhch E

Subjects
Arrhythmias, Cardiac complications, Arrhythmias, Cardiac pathology, Cardiomyopathies congenital, Cardiomyopathies pathology, Desmoplakins genetics, Genetic Testing, Heart Ventricles physiopathology, Humans, Myocarditis etiology, Myocarditis pathology, Plakophilins genetics, Polymorphism, Single Nucleotide, Ryanodine Receptor Calcium Release Channel genetics, Cardiomyopathies genetics, Myocarditis genetics
Published
2020
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