Author: "Jean-Baptiste Gourraud" - Searchworks@Jio Institute Digital Library Search Results

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160 results on '"Jean-Baptiste Gourraud"'

1. TAD boundary deletion causes PITX2-related cardiac electrical and structural defects

Author: Manon Baudic, Hiroshige Murata, Fernanda M. Bosada, Uirá Souto Melo, Takanori Aizawa, Pierre Lindenbaum, Lieve E. van der Maarel, Amaury Guedon, Estelle Baron, Enora Fremy, Adrien Foucal, Taisuke Ishikawa, Hiroya Ushinohama, Sean J. Jurgens, Seung Hoan Choi, Florence Kyndt, Solena Le Scouarnec, Vincent Wakker, Aurélie Thollet, Annabelle Rajalu, Tadashi Takaki, Seiko Ohno, Wataru Shimizu, Minoru Horie, Takeshi Kimura, Patrick T. Ellinor, Florence Petit, Yves Dulac, Paul Bru, Anne Boland, Jean-François Deleuze, Richard Redon, Hervé Le Marec, Thierry Le Tourneau, Jean-Baptiste Gourraud, Yoshinori Yoshida, Naomasa Makita, Claude Vieyres, Takeru Makiyama, Stephan Mundlos, Vincent M. Christoffels, Vincent Probst, Jean-Jacques Schott, and Julien Barc
Subjects: Science
Abstract: Abstract While 3D chromatin organization in topologically associating domains (TADs) and loops mediating regulatory element-promoter interactions is crucial for tissue-specific gene regulation, the extent of their involvement in human Mendelian disease is largely unknown. Here, we identify 7 families presenting a new cardiac entity associated with a heterozygous deletion of 2 CTCF binding sites on 4q25, inducing TAD fusion and chromatin conformation remodeling. The CTCF binding sites are located in a gene desert at 1 Mb from the Paired-like homeodomain transcription factor 2 gene (PITX2). By introducing the ortholog of the human deletion in the mouse genome, we recapitulate the patient phenotype and characterize an opposite dysregulation of PITX2 expression in the sinoatrial node (ectopic activation) and ventricle (reduction), respectively. Chromatin conformation assay performed in human induced pluripotent stem cell-derived cardiomyocytes harboring the minimal deletion identified in family#1 reveals a conformation remodeling and fusion of TADs. We conclude that TAD remodeling mediated by deletion of CTCF binding sites causes a new autosomal dominant Mendelian cardiac disorder.
Published: 2024
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2. The cardiac blood transcriptome predicts de novo onset of atrial fibrillation in heart failure

Author: Guillaume Lamirault, Imen Fellah-Hebia, Catherine Chevalier, Isabelle Guisle, Béatrice Guyomarc'h, Aude Solnon, Jean-Baptiste Gourraud, Laurent Desprets, Selim Abbey, Christophe Leclercq, Paul Bru, Antoine Milhem, Olivier Billon, Frederic Anselme, Arnaud Savouré, Jean-Noël Trochu, Rémi Houlgatte, Gilles Lande, and Marja Steenman
Subjects: Atrial fibrillation, Heart failure, Transcriptomic profiling, Prognostic biomarkers, Coronary sinus blood, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract: Heart failure (HF) increases the risk of developing atrial fibrillation (AF), leading to increased morbidity and mortality. Therefore, better prediction of this risk may improve treatment strategies. Although several predictors based on clinical data have been developed, the establishment of a transcriptome-based predictor of AF incidence in HF has proven to be more problematic. We hypothesized that the transcriptome profile of coronary sinus blood samples of HF patients is associated with AF incidence. We therefore enrolled 192 HF patients who were selected for biventricular cardioverter defibrillator implantation. Both coronary sinus and peripheral blood samples were obtained during the procedure. Patients were followed-up during two years and AF occurrence was based on interrogation of the defibrillator. A total of 96 patients stayed in sinus rhythm (SR) during follow-up, 13 patients developed AF within 1 year and 10 patients developed AF during the second year of follow up. Gene expression profiling of coronary sinus samples led to the identification of 321 AF predictor genes based on their differential expression between patients developing AF within 1 year of blood sampling and patients remaining in SR. The expression levels of these genes were combined to obtain a molecular atrial fibrillation prediction score for each patient which was significantly different between both patient groups (Mann-Whitney, p = 0.00018). We conclude that the cardiac blood transcriptome of HF patients should be further investigated as a potential AF risk prediction tool.
Published: 2024
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3. Generation of a patient-specific induced pluripotent stem cell line carrying the DES p.R406W mutation, an isogenic control and a DES p.R406W knock-in line

Author: Michelle Geryk, Robin Canac, Virginie Forest, Pierre Lindenbaum, Aurore Girardeau, Manon Baudic, Estelle Baron, Anne Bibonne, Caroline Chariau, Florence Kyndt, Richard Redon, Jean-Jacques Schott, Jean-Baptiste Gourraud, Julien Barc, and Flavien Charpentier
Subjects: Biology (General), QH301-705.5
Abstract: Mutations in the DES gene, which encodes the intermediate filament desmin, lead to desminopathy, a rare disease characterized by skeletal muscle weakness and different forms of cardiomyopathies associated with cardiac conduction defects and arrhythmias. We generated human induced pluripotent stem cells (hiPSC) from a patient carrying the DES p.R406W mutation, and employed CRISPR/Cas9 to rectify the mutation in the patient's hiPSC line and introduced the mutation in an hiPSC line from a control individual unrelated to the patient. These hiPSC lines represent useful models for delving into the mechanisms of desminopathy and developing new therapeutic approaches.
Published: 2024
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4. Electrical Storm Ablation in a Patient in Cardiogenic Shock Supported by Impella 5.0

Author: Robin Le Ruz, MD, Gilles Lande, MD, PhD, Thierry Lepoivre, MD, Charles-Henri David, MD, Magali Michel, MD, and Jean-Baptiste Gourraud, MD, PhD
Subjects: case report, cardiogenic shock, electrical storm, Impella 5.0, radiofrequency ablation, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract: Intra-axial pumps are increasingly used to support cardiogenic shock. The occurrence of electrical storms in this setting is a rising issue, and data remain scarce about optimal management. We report the feasibility of ventricular tachycardia ablation in the presence of a recent surgically inserted Impella 5.0 device (Abiomed, Danvers, Massachusetts). (Level of Difficulty: Intermediate.)
Published: 2021
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5. Generation of human induced pluripotent stem cell lines from three patients affected by Catecholaminergic Polymorphic ventricular tachycardia (CPVT) carrying heterozygous mutations in RYR2 gene

Author: Bastien Cimarosti, Robin Canac, Stephan De Waard, Aurore Girardeau, Anne Gaignerie, Aude Derevier, Virginie Forest, Michel Ronjat, Hervé Le Marec, Jean-Baptiste Gourraud, Patricia Lemarchand, Michel De Waard, Guillaume Lamirault, and Nathalie Gaborit
Subjects: Biology (General), QH301-705.5
Abstract: Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an exercise and emotional stress-induced life-threatening inherited heart rhythm disorder, characterized by an abnormal cellular calcium homeostasis. Most reported cases have been linked to mutations in the gene encoding the type 2 ryanodine receptor gene, RYR2. We generated induced pluripotent stem cells (hiPSCs) from peripheral blood mononuclear cells (PBMC) from three CPVT-affected patients, two of them carrying p.R4959Q mutation and one carrying p.Y2476D mutation. These generated hiPSC lines are a useful model to study pathophysiological consequences of RYR2 dysfunction in humans and the molecular basis of CPVT.
Published: 2022
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6. Generation of human induced pluripotent stem cell lines from four unrelated healthy control donors carrying European genetic background

Author: Aurore Girardeau, Diane Atticus, Robin Canac, Bastien Cimarosti, Amandine Caillaud, Caroline Chariau, Floriane Simonet, Bertrand Cariou, Flavien Charpentier, Jean-Baptiste Gourraud, Vincent Probst, Nadjet Belbachir, Laurence Jesel, Patricia Lemarchand, Julien Barc, Richard Redon, Nathalie Gaborit, and Guillaume Lamirault
Subjects: Biology (General), QH301-705.5
Abstract: Four human induced pluripotent stem cell (hiPSC) lines have been generated from healthy control European donors, and validated. This resource represents a useful tool for stem cell-based research, as references for developmental studies and disease modeling linked to any type of human tissue and organ, in an ethnical-, sex- and age-matched context. They providea reliable in-vitro model for single cell- and tissue-based investigations, and are also a valuable tool for genome editing-based studies.
Published: 2022
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7. Generation of human induced pluripotent stem cell lines from two patients affected by catecholamine-induced QT prolongation (CIQTP)

Author: Bastien Cimarosti, Robin Canac, Aurore Girardeau, Marine Arnaud, Quentin Francheteau, Vincent Probst, Patricia Lemarchand, Richard Redon, Jean-Baptiste Gourraud, Nathalie Gaborit, and Guillaume Lamirault
Subjects: Biology (General), QH301-705.5
Abstract: Catecholamine-induced QT prolongation (CIQTP) is an inherited cardiac disease characterized by a normal baseline ECG and a risk of sudden cardiac death by ventricular arrhythmia due to a QT prolongation that only appears during catecholergic stimulation, especially mental stress. Induced pluripotent stem cells (hiPSCs) were generated from peripheral blood mononuclear cells collected from two CIQTP-affected patients from two different families. These two hiPSC lines are a valuable model to study biological alterations due to CIQTP.
Published: 2022
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8. Impact of atrial tachycardia management on outcomes in adult patients with congenital heart disease

Author: Jean Gallet, Victor Waldmann, Laurianne Le Gloan, Patrice Guérin, Jean-Baptiste Gourraud, Denis Amet, Mikael Laredo, Laurence Iserin, Eloi Marijon, and Magalie Ladouceur
Subjects: Atrial arrhythmia, Congenital heart disease, Direct current-cardioversion, Catheter ablation, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract: Background: Atrial arrhythmias are common complications in adults with congenital heart disease (ACHD) and are associated with high morbi-mortality. Atrial tachycardia (AT) including intra-atrial reentrant tachycardia and focal atrial tachycardia are the most prevalent arrhythmias in ACHD. We aimed to compare mid-term outcomes according to arrhythmia management. Methods: All hospitalizations for AT in ACHD were retrospectively identified from medico-administrative database from September 2006 to September 2016 in two centers. Outcomes of patients with AT managed with direct-current cardioversion (DCCV) were compared with patients referred for catheter ablation (CA). Atrial arrhythmia recurrence, stroke, admission for heart failure and death were analyzed during follow-up. Results: One hundred and twenty-one ACHD with AT (median age 41 [IQR 32–52] years, male 62%, 41% moderate CHD and 38% complex CHD) were included. Median follow-up was 3.3 years [IQR 1.4–6.2]. Thirty-seven (30.5%) patients underwent DCCV and 84 (69.5%) CA. No significant difference in age, CHD complexity, history of atrial arrhythmia, heart failure, and CHA2DS2-VASC score were observe. Patients undergoing DCCV had higher NYHA class. Atrial arrhythmia recurrence was lower in CA group (Log-rank test, p = 0.045). Most recurrences were AT, but atrial fibrillation was recorded in 35.1% after DCCV and 9.5% after CA (p = 0.001). At the end of follow-up, patients who were initially treated by CA had a significantly lower incidence of severe cardiovascular events (48.6% in DCCV group vs. 16.7% in CA group, HR: 0.46, CI 95%: 0.22–0.93, p = 0.031). Conclusions: A proactive management of AT by CA is associated with better long-term outcomes in ACHD.
Published: 2021
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9. A consistent arrhythmogenic trait in Brugada syndrome cellular phenotype

Author: Zeina R. Al Sayed, Mariam Jouni, Jean‐Baptiste Gourraud, Nadjet Belbachir, Julien Barc, Aurore Girardeau, Virginie Forest, Aude Derevier, Anne Gaignerie, Caroline Chariau, Bastien Cimarosti, Robin Canac, Pierre Olchesqui, Eric Charpentier, Jean‐Jacques Schott, Richard Redon, Isabelle Baró, Vincent Probst, Flavien Charpentier, Gildas Loussouarn, Kazem Zibara, Guillaume Lamirault, Patricia Lemarchand, and Nathalie Gaborit
Subjects: Medicine (General), R5-920
Published: 2021
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10. Dysfunction of the Voltage‐Gated K+ Channel β2 Subunit in a Familial Case of Brugada Syndrome

Author: Vincent Portero, Solena Le Scouarnec, Zeineb Es‐Salah‐Lamoureux, Sophie Burel, Jean‐Baptiste Gourraud, Stéphanie Bonnaud, Pierre Lindenbaum, Floriane Simonet, Jade Violleau, Estelle Baron, Eléonore Moreau, Carol Scott, Stéphanie Chatel, Gildas Loussouarn, Thomas O'Hara, Philippe Mabo, Christian Dina, Hervé Le Marec, Jean‐Jacques Schott, Vincent Probst, Isabelle Baró, Céline Marionneau, Flavien Charpentier, and Richard Redon
Subjects: Brugada syndrome, cardiac arrhythmia, clinical electrophysiology, genetics, KCNAB2/Kvβ2, potassium ion channels, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract: BackgroundThe Brugada syndrome is an inherited cardiac arrhythmia associated with high risk of sudden death. Although 20% of patients with Brugada syndrome carry mutations in SCN5A, the molecular mechanisms underlying this condition are still largely unknown. Methods and ResultsWe combined whole‐exome sequencing and linkage analysis to identify the genetic variant likely causing Brugada syndrome in a pedigree for which SCN5A mutations had been excluded. This approach identified 6 genetic variants cosegregating with the Brugada electrocardiographic pattern within the pedigree. In silico gene prioritization pointed to 1 variant residing in KCNAB2, which encodes the voltage‐gated K+ channel β2‐subunit (Kvβ2‐R12Q). Kvβ2 is widely expressed in the human heart and has been shown to interact with the fast transient outward K+ channel subunit Kv4.3, increasing its current density. By targeted sequencing of the KCNAB2 gene in 167 unrelated patients with Brugada syndrome, we found 2 additional rare missense variants (L13F and V114I). We then investigated the physiological effects of the 3 KCNAB2 variants by using cellular electrophysiology and biochemistry. Patch‐clamp experiments performed in COS‐7 cells expressing both Kv4.3 and Kvβ2 revealed a significant increase in the current density in presence of the R12Q and L13F Kvβ2 mutants. Although biotinylation assays showed no differences in the expression of Kv4.3, the total and submembrane expression of Kvβ2‐R12Q were significantly increased in comparison with wild‐type Kvβ2. ConclusionsAltogether, our results indicate that Kvβ2 dysfunction can contribute to the Brugada electrocardiographic pattern.
Published: 2016
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11. Rapid ventricular tachycardia in patients with tetralogy of Fallot and implantable cardioverter-defibrillator: Insights from the DAI-T4F nationwide registry

Author: Mikael Laredo, Guillaume Duthoit, Frédéric Sacher, Frédéric Anselme, Caroline Audinet, Francis Bessière, Pierre Bordachar, Abdeslam Bouzeman, Serge Boveda, Sok Sithikun Bun, Morgane Chassignolle, Gaël Clerici, Antoine Da Costa, Maxime de Guillebon, Pascal Defaye, Nathalie Elbaz, Romain Eschalier, Fabrice Extramiana, Laurent Fauchier, Alexis Hermida, Estelle Gandjbakhch, Rodrigue Garcia, Jean-Baptiste Gourraud, Charles Guenancia, Benoit Guy-Moyat, Didier Irles, Laurence Iserin, François Jourda, Linda Koutbi, Fabien Labombarda, Magalie Ladouceur, Philippe Lagrange, Nicolas Lellouche, Jacques Mansourati, Christelle Marquié, Raphael Martins, Grégoire Massoulié, Amel Mathiron, Philippe Maury, Anne Messali, Antoine Milhem, Pierre Mondoly, Cédric Nguyen, Sandro Ninni, Jean Luc Pasquié, Bertrand Pierre, Penelope Pujadas, Jean-Marc Sellal, Jean-Benoit Thambo, Camille Walton, Pierre Winum, Cyril Zakine, Alexandre Zhao, Xavier Jouven, Nicolas Combes, Eloi Marijon, Victor Waldmann, CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), IHU-LIRYC, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux], Institut de rythmologie et modélisation cardiaque [Pessac] (IHU Liryc), 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], 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), 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), Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), 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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)
Subjects: Sudden cardiac death, Rapid ventricular tachycardia, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Physiology (medical), Tetralogy of Fallot, Ventricular arrhythmia, Adult congenital heart disease, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Cardiology and Cardiovascular Medicine, Implantable cardioverter-defibrillator
Abstract: In repaired tetralogy of Fallot (TOF), little is known about characteristics of patients with rapid ventricular tachycardia (VT). Also, whether patients with a first episode of nonrapid VT may subsequently develop rapid VT or ventricular fibrillation (VF) has not been addressed.The objectives of this study were to compare patients with rapid VT/VF with those with nonrapid VT and to assess the evolution of VT cycle lengths (VTCLs) overtime.Data were analyzed from a nationwide registry including all patients with TOF and implantable cardioverter-defibrillator (ICD) since 2000. Patients with ≥1 VT episode with VTCL ≤250 ms (240 beats/min) formed the rapid VT/VF group.Of 144 patients (mean age 42.0 ± 12.7 years; 104 [72%] men), 61 (42%) had at least 1 VT/VF episode, including 28 patients with rapid VT/VF (46%), during a median follow-up of 6.3 years (interquartile range 2.2-10.3 years). Compared with patients in the nonrapid VT group, those in the rapid VT/VF group were significantly younger at ICD implantation (35.2 ± 12.6 years vs 41.5 ± 11.2 years; P = .04), had more frequently a history of cardiac arrest (8 [29%] vs 2 [6%]; P = .02), less frequently a history of atrial arrhythmia (11 [42%] vs 22 [69%]; P = .004), and higher right ventricular ejection fraction (43.3% ± 10.3% vs 36.6% ± 11.2%; P = .04). The median VTCL of VT/VF episodes was 325 ms (interquartile range 235-429 ms). None of the patients with a first documented nonrapid VT episode had rapid VT/VF during follow-up.Patients with TOF and rapid VT/VF had distinct clinical characteristics. The relatively low variation of VTCL over time suggests a room for catheter ablation without a backup ICD in selected patients with well-tolerated VT.Clinicaltrials.gov identifier: NCT03837574.
Published: 2023
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12. Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator

Author: Paloma Jordà, Laurens P Bosman, Alessio Gasperetti, Andrea Mazzanti, Jean Baptiste Gourraud, Brianna Davies, Tanja Charlotte Frederiksen, Zoraida Moreno Weidmann, Andrea Di Marco, Jason D Roberts, Ciorsti MacIntyre, Colette Seifer, Antoine Delinière, Wael Alqarawi, Deni Kukavica, Damien Minois, Alessandro Trancuccio, Marine Arnaud, Mattia Targetti, Annamaria Martino, Giada Oliviero, Daniel C Pipilas, Corrado Carbucicchio, Paolo Compagnucci, Antonio Dello Russo, Iacopo Olivotto, Leonardo Calò, Steven A Lubitz, Michael J Cutler, Philippe Chevalier, Elena Arbelo, Silvia Giuliana Priori, Jeffrey S Healey, Hugh Calkins, Michela Casella, Henrik Kjærulf Jensen, Claudio Tondo, Rafik Tadros, Cynthia A James, Andrew D Krahn, Julia Cadrin-Tourigny, Sociedad Española de Cardiología, Novo Nordisk Foundation, American Heart Association, and Canada Research Chairs
Subjects: Defibrillators, Implantable/adverse effects, Settore MED/11 - Malattie dell'Apparato Cardiovascolare, Arrhythmias, Cardiac, Arrhythmias, Cardiac/etiology, Implantable cardioverter-defibrillator, Defibrillators, Implantable, Sudden cardiac death, Ventricular arrhythmias, Death, Sudden, Cardiac, Arrhythmogenic right ventricular cardiomyopathy, Genetic cardiomyopathies, Risk stratification, Risk Factors, Arrhythmogenic Right Ventricular Dysplasia/complications, Humans, Cardiology and Cardiovascular Medicine, Death, Sudden, Cardiac/epidemiology, Arrhythmogenic Right Ventricular Dysplasia, Retrospective Studies
Abstract: Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC. P.J. is supported by the Daniel Bravo Foundation grant and Spanish Society of Cardiology Magda Heras mobility grant, A.G. by the Wilton W. Webster Fellow of Heart Rhythm Society, The Johns Hopkins ARVD/C Programme by the Leonie-Wild Foundation, Leyla Erkan Family Fund for ARVD Research, The Hugh Calkins, Marvin H. Weiner, and Jacqueline J. Bernstein Cardiac Arrhythmia Center, Dr Francis P. Chiramonte Private Foundation, Dr Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins, Bogle Foundation, Campanella Family, Patrick J. Harrison Family, Peter French Memorial Foundation, and Wilmerding Endowments, H.K.J. by grants from Novo Nordisk Foundation, Denmark (NNF18OC0031258 and NNF20OC0065151), S.A.L. by NIH grant 1R01HL139731 and American Heart Association 18SFRN34250007, R.T. by the Canada Research Chairs programme, J.C.T. by the Philippa and Marvin Carsley Cardiology Research Chair. S.G.P. receives support from Ricerca Corrente funding scheme of the Italian Ministry of Health and Italian Ministry of Research and University Dipartimenti di Eccellenza 2018 to 2022 grant to the Molecular Medicine Department (University of Pavia). Sí
Published: 2022
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13. Characteristics of Patients with Spontaneous Versus Drug-Induced Brugada Electrocardiogram: Sub-Analysis From the SABRUS

Author: Anat Milman, Avi Sabbag, Giulio Conte, Pieter G. Postema, Antoine Andorin, Jean-Baptiste Gourraud, Frederic Sacher, Philippe Mabo, Sung-Hwan Kim, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Jimmy JM Juang, Yoav Michowitz, Eran Leshem, Yuka Mizusawa, Elena Arbelo, Zhengrong Huang, Isabelle Denjoy, Carla Giustetto, Yanushi D. Wijeyeratne, Andrea Mazzanti, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Leonardo Calo, Georgia Sarquella-Brugada, Jacob Tfelt-Hansen, Silvia G. Priori, Masahiko Takagi, Christian Veltmann, Pietro Delise, Domenico Corrado, Elijah R. Behr, Fiorenzo Gaita, Gan-Xin Yan, Josep Brugada, Antoine Leenhardt, Arthur A.M. Wilde, Pedro Brugada, Kengo F. Kusano, Kenzo Hirao, Gi-Byoung Nam, Vincent Probst, Bernard Belhassen, Brussels Heritage Lab, Clinical sciences, Heartrhythmmanagement, Cardio-vascular diseases, Cardiology, and ACS - Heart failure & arrhythmias
Subjects: Physiology (medical), electrocardiography, syncope, Brugada syndrome, Cardiology and Cardiovascular Medicine, ventricular fibrillation, heart arrest
Published: 2023

14. MP-453089-11 DIFFERENCES IN UTILIZATION OF PRIMARY PREVENTION IMPLANTABLE CARDIOVERTER DEFIBRILLATORS IN ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY ACROSS NORTH AMERICA AND EUROPE

Author: Richard Carrick, Corrado De Marco, Alessio Gasperetti, Laurens P. Bosman, JEAN BAPTISTE GOURRAUD, Andrea Mazzanti, Brittney A. Murray, Catherine Pendleton, Crystal Tichnell, Harikrishna Tandri, Katja Zeppenfeld, Arthur A. Wilde, Brianna Davies, Colette M. Seifer, Jason D. Roberts, Jeffrey S. Healey, Ciorsti MacIntyre, Wael Alqarawi, Rafik Tadros, Michael J. Cutler, Mattia Targetti, Leonardo Caló, Francesco Vitali, Matteo Bertini, Paolo Compagnucci, Michela Casella, Antonio Dello Russo, Chiara Cappelletto, Antonio De Luca, Davide Stolfo, Firat Duru, Henrik K. Jensen, Anneli Svensson, Pia Dahlberg, Nina Hasselberg, Andrea Di Marco, Paloma Jorda, Elena Arbelo, Zoraida Moreno weidmann, Karolina Borowiec, Antoine Deliniere, Elzbieta K. Biernacka, Peter van Tintelen, Pyotr G. Platonov, Iacopo Olivotto, Ardan Saguner, Kristina H. Haugaa, Moniek Cox, Claudio Tondo, Marco Merlo, Andrew D. Krahn, Anneline te Riele, Katherine C. Wu, Hugh Calkins, Cynthia A. James, and JULIA CADRIN-TOURIGNY
Subjects: Physiology (medical), Cardiology and Cardiovascular Medicine
Published: 2023
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15. Reply to the Editor- Give to Caesar What Belongs to Caesar

Author: Michel Haissaguerre, William Escande, Jean-Baptiste Gourraud, Olivier Bernus, and Frédéric Sacher
Subjects: Physiology (medical), Cardiology and Cardiovascular Medicine
Published: 2022

16. MP-453092-9 PROGNOSTIC SIGNIFICANCE OF SUSTAINED VENTRICULAR ARRHYTHMIAS OCCURRING UNDER WEARABLE CARDIOVERTER DEFIBRILLATOR PROTECTION IN POST-INFARCT PATIENTS WITH A LEFT VENTRICULAR DYSFUNCTION

Author: Mathieu ECHIVARD, Jean-Marc Sellal, Chloé Ziliox, Eloi Marijon, Pierre BORDACHAR, christelle marquie, Clemence Docq, Romain Eschalier, Baptiste Maille, JEAN-CLAUDE DEHARO, Dominique Babuty, estelle gandjbakhch, Antoine Da Costa, Olivier Piot, Damien Minois, JEAN BAPTISTE GOURRAUD, PIERRE MONDOLY, Philippe Maury, SERGE BOVEDA, Jean-Luc Pasquie, Raphael Martins, Christophe Leclercq, Charles Guenancia, Gabriel LAURENT, Mathieu Becker, JULIEN BERTRAND, Philippe Chevalier, Vladimir Manenti, MACIEJ KUBALA, PASCAL DEFAYE, Peggy Jacon, Antoine Desbiolles, Marc Badoz, Laurence Jesel, Nicolas Lellouche, Paul Milliez, Paul Ollitrault, Samir Fareh, Matthieu Bercker, Jacques Mansourati, Benoit Guy Moyat, Nicolas Luconi, Jean-Pierre Chabert, null pierre winum, Frederic Anselme, Antoine Leenhardt, Fabrice Extramiana, Camille Delahaye, François Jourda, Olivier Bizeau, Mathieu Nasarre, ARNAUD OLIVIER, Stéphane Fromentin, THIBAULT VILLEMIN, Olivier Levavasseur, Néfissa Hammache, Isabelle Magnin, Hugues Blangy, Nicolas Sadoul, Kévin Duarte, Nicolas Girerd, and Christian de Chillou
Subjects: Physiology (medical), Cardiology and Cardiovascular Medicine
Published: 2023
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17. Comparison of defibrillation modalities in the paediatric population: IChildren registry, a French observational and multicentric study

Author: Aurélien Clédel, Victor Waldmann, Christelle Marquié, Linda Koutbi, Laurence Jesel, Jean-Benoît Thambo, Hugues Blangy, Philippe Maury, Grégoire Delavilleon, Dominique Babuty, Raphael Martins, Antoine Da Costa, Romain Eschalier, Sandrine Venier, Eloi Marijon, Vincent Probst, and Jean-Baptiste Gourraud
Subjects: Cardiology and Cardiovascular Medicine
Published: 2023
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18. Electrical Storm Ablation in a Patient in Cardiogenic Shock Supported by Impella 5.0

Author: Gilles Lande, Thierry Lepoivre, Robin Le Ruz, Magali Michel, Jean-Baptiste Gourraud, and Charles-Henri David
Subjects: 0301 basic medicine, medicine.medical_specialty, Radiofrequency ablation, medicine.medical_treatment, 030105 genetics & heredity, law.invention, 03 medical and health sciences, ECLS, extracorporeal life support, 0302 clinical medicine, Ventricular tachycardia ablation, law, Internal medicine, medicine, VT, ventricular tachycardia, Mini-Focus Issue: Electrophysiology, case report, Diseases of the circulatory (Cardiovascular) system, LV, left ventricular, Impella, business.industry, Cardiogenic shock, cardiogenic shock, ES, electrical storm, medicine.disease, Ablation, Optimal management, RC666-701, Cardiology, LVAD, left ventricular assist device, Case Report: Clinical Case, Impella 5.0, radiofrequency ablation, VT - Ventricular tachycardia, Cardiology and Cardiovascular Medicine, business, 030217 neurology & neurosurgery, electrical storm
Abstract: Intra-axial pumps are increasingly used to support cardiogenic shock. The occurrence of electrical storms in this setting is a rising issue, and data remain scarce about optimal management. We report the feasibility of ventricular tachycardia ablation in the presence of a recent surgically inserted Impella 5.0 device (Abiomed, Danvers, Massachusetts). (Level of Difficulty: Intermediate.), Graphical abstract
Published: 2021

19. Author Correction

Author: Michele Nicastro, Stephanie BONNAUD, Rafik Tadros, Philip Van Damme, Sean Jurgens, Pier Lambiase, Elijah Behr, Tomas Robyns, Julien Barc, Andrea Mazzanti, Manon BAUDIC, Sonia Van Dooren, Jean Baptiste Gourraud, Caroline Rooryck, Carlo Napolitano, Katja E Odening, Clinical sciences, Reproduction and Genetics, Heartrhythmmanagement, and Cardio-vascular diseases
Subjects: young adults, sodium channel regulation, cardiac arrhythmia disorder, Genetics, sudden death, Brugada syndrome, Cardiology and Cardiovascular Medicine, risk
Published: 2022
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20. Author Correction: Genome-wide association analyses identify new Brugada syndrome risk loci and highlight a new mechanism of sodium channel regulation in disease susceptibility (Nature Genetics, (2022), 54, 3, (232-239), 10.1038/s41588-021-01007-6)

Author: Julien Barc, Barc, J, Tadros, R, Glinge, C, Chiang, D, Jouni, M, Simonet, F, Jurgens, S, Baudic, M, Nicastro, M, Potet, F, Offerhaus, J, Walsh, R, Hoan Choi, S, Verkerk, A, Mizusawa, Y, Anys, S, Minois, D, Arnaud, M, Duchateau, J, Wijeyeratne, Y, Muir, A, Papadakis, M, Castelletti, S, Torchio, M, Gil Ortuño, C, Lacunza, J, Giachino, D, Cerrato, N, Martins, R, Campuzano, O, Van Dooren, S, Thollet, A, Kyndt, F, Mazzanti, A, Clémenty, N, Bisson, A, Corveleyn, A, Stallmeyer, B, Dittmann, S, Saenen, J, Noël, A, Honarbakhsh, S, Rudic, B, Marzak, H, Rowe, M, Federspiel, C, Le Page, S, Placide, L, Milhem, A, Barajas-Martinez, H, Beckmann, B, Krapels, I, Steinfurt, J, Gregers Winkel, B, Jabbari, R, Shoemaker, M, Boukens, B, Škorić-Milosavljević, D, Bikker, H, Manevy, F, Lichtner, P, Ribasés, M, Meitinger, T, Müller-Nurasyid, M, Group, K, Veldink, J, van den Berg, L, Van Damme, P, Cusi, D, Lanzani, C, Rigade, S, Charpentier, E, Baron, E, Bonnaud, S, Lecointe, S, Donnart, A, Le Marec, H, Chatel, S, Karakachoff, M, Bézieau, S, London, B, Tfelt-Hansen, J, Roden, D, Odening, K, Cerrone, M, Chinitz, L, Volders, P, van de Berg, M, Laurent, G, Faivre, L, Antzelevitch, C, Kääb, S, Al Arnaout, A, Dupuis, J, Pasquie, J, Billon, O, Roberts, J, Jesel, L, Borggrefe, M, Lambiase, P, Mansourati, J, Loeys, B, Leenhardt, A, Guicheney, P, Maury, P, Schulze-Bahr, E, Robyns, T, Breckpot, J, Babuty, D, Priori, S, Napolitano, C, Referral Center for inherited cardiac arrhythmia, N, de Asmundis, C, Brugada, P, Brugada, R, Arbelo, E, Brugada, J, Mabo, P, Behar, N, Giustetto, C, Sabater Molina, M, Gimeno, J, Hasdemir, C, Schwartz, P, Crotti, L, Mckeown, P, Sharma, S, Behr, E, Haissaguerre, M, Sacher, F, Rooryck, C, Tan, H, Remme, C, Postema, P, Delmar, M, Ellinor, P, Lubitz, S, Gourraud, J, Tanck, M, L. George Jr., A, Macrae, C, Burridge, P, Dina, C, Probst, V, Wilde, A, Schott, J, Redon &amp, R, Bezzina, C, Julien Barc, Rafik Tadros, Charlotte Glinge, David Y. Chiang, Mariam Jouni, Floriane Simonet, Sean J. Jurgens, Manon Baudic, Michele Nicastro, Franck Potet, Joost A. Offerhaus, Roddy Walsh, Seung Hoan Choi, Arie O. Verkerk, Yuka Mizusawa, Soraya Anys, Damien Minois, Marine Arnaud, Josselin Duchateau, Yanushi D. Wijeyeratne, Alison Muir, Michael Papadakis, Silvia Castelletti, Margherita Torchio, Cristina Gil Ortuño, Javier Lacunza, Daniela F. Giachino, Natascia Cerrato, Raphaël P. Martins, Oscar Campuzano, Sonia Van Dooren, Aurélie Thollet, Florence Kyndt, Andrea Mazzanti, Nicolas Clémenty, Arnaud Bisson, Anniek Corveleyn, Birgit Stallmeyer, Sven Dittmann, Johan Saenen, Antoine Noël, Shohreh Honarbakhsh, Boris Rudic, Halim Marzak, Matthew K. Rowe, Claire Federspiel, Sophie Le Page, Leslie Placide, Antoine Milhem, Hector Barajas-Martinez, Britt-Maria Beckmann, Ingrid P. Krapels, Johannes Steinfurt, Bo Gregers Winkel, Reza Jabbari, Moore B. Shoemaker, Bas J. Boukens, Doris Škorić-Milosavljević, Hennie Bikker, Federico Manevy, Peter Lichtner, Marta Ribasés, Thomas Meitinger, Martina Müller-Nurasyid, KORA-Study Group, Jan H. Veldink, Leonard H. van den Berg, Philip Van Damme, Daniele Cusi, Chiara Lanzani, Sidwell Rigade, Eric Charpentier, Estelle Baron, Stéphanie Bonnaud, Simon Lecointe, Audrey Donnart, Hervé Le Marec, Stéphanie Chatel, Matilde Karakachoff, Stéphane Bézieau, Barry London, Jacob Tfelt-Hansen, Dan Roden, Katja E. Odening, Marina Cerrone, Larry A. Chinitz, Paul G. Volders, Maarten P. van de Berg, Gabriel Laurent, Laurence Faivre, Charles Antzelevitch, Stefan Kääb, Alain Al Arnaout, Jean-Marc Dupuis, Jean-Luc Pasquie, Olivier Billon, Jason D. Roberts, Laurence Jesel, Martin Borggrefe, Pier D. Lambiase, Jacques Mansourati, Bart Loeys, Antoine Leenhardt, Pascale Guicheney, Philippe Maury, Eric Schulze-Bahr, Tomas Robyns, Jeroen Breckpot, Dominique Babuty, Silvia G. Priori, Carlo Napolitano, Nantes Referral Center for inherited cardiac arrhythmia, Carlo de Asmundis, Pedro Brugada, Ramon Brugada, Elena Arbelo, Josep Brugada, Philippe Mabo, Nathalie Behar, Carla Giustetto, Maria Sabater Molina, Juan R. Gimeno, Can Hasdemir, Peter J. Schwartz, Lia Crotti, Pascal P. McKeown, Sanjay Sharma, Elijah R. Behr, Michel Haissaguerre, Frédéric Sacher, Caroline Rooryck, Hanno L. Tan, Carol A. Remme, Pieter G. Postema, Mario Delmar, Patrick T. Ellinor, Steven A. Lubitz, Jean-Baptiste Gourraud, Michael W. Tanck, Alfred L. George Jr., Calum A. MacRae, Paul W. Burridge, Christian Dina, Vincent Probst, Arthur A. Wilde, Jean-Jacques Schott, Richard Redon &, Connie R. Bezzina, Julien Barc, Barc, J, Tadros, R, Glinge, C, Chiang, D, Jouni, M, Simonet, F, Jurgens, S, Baudic, M, Nicastro, M, Potet, F, Offerhaus, J, Walsh, R, Hoan Choi, S, Verkerk, A, Mizusawa, Y, Anys, S, Minois, D, Arnaud, M, Duchateau, J, Wijeyeratne, Y, Muir, A, Papadakis, M, Castelletti, S, Torchio, M, Gil Ortuño, C, Lacunza, J, Giachino, D, Cerrato, N, Martins, R, Campuzano, O, Van Dooren, S, Thollet, A, Kyndt, F, Mazzanti, A, Clémenty, N, Bisson, A, Corveleyn, A, Stallmeyer, B, Dittmann, S, Saenen, J, Noël, A, Honarbakhsh, S, Rudic, B, Marzak, H, Rowe, M, Federspiel, C, Le Page, S, Placide, L, Milhem, A, Barajas-Martinez, H, Beckmann, B, Krapels, I, Steinfurt, J, Gregers Winkel, B, Jabbari, R, Shoemaker, M, Boukens, B, Škorić-Milosavljević, D, Bikker, H, Manevy, F, Lichtner, P, Ribasés, M, Meitinger, T, Müller-Nurasyid, M, Group, K, Veldink, J, van den Berg, L, Van Damme, P, Cusi, D, Lanzani, C, Rigade, S, Charpentier, E, Baron, E, Bonnaud, S, Lecointe, S, Donnart, A, Le Marec, H, Chatel, S, Karakachoff, M, Bézieau, S, London, B, Tfelt-Hansen, J, Roden, D, Odening, K, Cerrone, M, Chinitz, L, Volders, P, van de Berg, M, Laurent, G, Faivre, L, Antzelevitch, C, Kääb, S, Al Arnaout, A, Dupuis, J, Pasquie, J, Billon, O, Roberts, J, Jesel, L, Borggrefe, M, Lambiase, P, Mansourati, J, Loeys, B, Leenhardt, A, Guicheney, P, Maury, P, Schulze-Bahr, E, Robyns, T, Breckpot, J, Babuty, D, Priori, S, Napolitano, C, Referral Center for inherited cardiac arrhythmia, N, de Asmundis, C, Brugada, P, Brugada, R, Arbelo, E, Brugada, J, Mabo, P, Behar, N, Giustetto, C, Sabater Molina, M, Gimeno, J, Hasdemir, C, Schwartz, P, Crotti, L, Mckeown, P, Sharma, S, Behr, E, Haissaguerre, M, Sacher, F, Rooryck, C, Tan, H, Remme, C, Postema, P, Delmar, M, Ellinor, P, Lubitz, S, Gourraud, J, Tanck, M, L. George Jr., A, Macrae, C, Burridge, P, Dina, C, Probst, V, Wilde, A, Schott, J, Redon &amp, R, Bezzina, C, Julien Barc, Rafik Tadros, Charlotte Glinge, David Y. Chiang, Mariam Jouni, Floriane Simonet, Sean J. Jurgens, Manon Baudic, Michele Nicastro, Franck Potet, Joost A. Offerhaus, Roddy Walsh, Seung Hoan Choi, Arie O. Verkerk, Yuka Mizusawa, Soraya Anys, Damien Minois, Marine Arnaud, Josselin Duchateau, Yanushi D. Wijeyeratne, Alison Muir, Michael Papadakis, Silvia Castelletti, Margherita Torchio, Cristina Gil Ortuño, Javier Lacunza, Daniela F. Giachino, Natascia Cerrato, Raphaël P. Martins, Oscar Campuzano, Sonia Van Dooren, Aurélie Thollet, Florence Kyndt, Andrea Mazzanti, Nicolas Clémenty, Arnaud Bisson, Anniek Corveleyn, Birgit Stallmeyer, Sven Dittmann, Johan Saenen, Antoine Noël, Shohreh Honarbakhsh, Boris Rudic, Halim Marzak, Matthew K. Rowe, Claire Federspiel, Sophie Le Page, Leslie Placide, Antoine Milhem, Hector Barajas-Martinez, Britt-Maria Beckmann, Ingrid P. Krapels, Johannes Steinfurt, Bo Gregers Winkel, Reza Jabbari, Moore B. Shoemaker, Bas J. Boukens, Doris Škorić-Milosavljević, Hennie Bikker, Federico Manevy, Peter Lichtner, Marta Ribasés, Thomas Meitinger, Martina Müller-Nurasyid, KORA-Study Group, Jan H. Veldink, Leonard H. van den Berg, Philip Van Damme, Daniele Cusi, Chiara Lanzani, Sidwell Rigade, Eric Charpentier, Estelle Baron, Stéphanie Bonnaud, Simon Lecointe, Audrey Donnart, Hervé Le Marec, Stéphanie Chatel, Matilde Karakachoff, Stéphane Bézieau, Barry London, Jacob Tfelt-Hansen, Dan Roden, Katja E. Odening, Marina Cerrone, Larry A. Chinitz, Paul G. Volders, Maarten P. van de Berg, Gabriel Laurent, Laurence Faivre, Charles Antzelevitch, Stefan Kääb, Alain Al Arnaout, Jean-Marc Dupuis, Jean-Luc Pasquie, Olivier Billon, Jason D. Roberts, Laurence Jesel, Martin Borggrefe, Pier D. Lambiase, Jacques Mansourati, Bart Loeys, Antoine Leenhardt, Pascale Guicheney, Philippe Maury, Eric Schulze-Bahr, Tomas Robyns, Jeroen Breckpot, Dominique Babuty, Silvia G. Priori, Carlo Napolitano, Nantes Referral Center for inherited cardiac arrhythmia, Carlo de Asmundis, Pedro Brugada, Ramon Brugada, Elena Arbelo, Josep Brugada, Philippe Mabo, Nathalie Behar, Carla Giustetto, Maria Sabater Molina, Juan R. Gimeno, Can Hasdemir, Peter J. Schwartz, Lia Crotti, Pascal P. McKeown, Sanjay Sharma, Elijah R. Behr, Michel Haissaguerre, Frédéric Sacher, Caroline Rooryck, Hanno L. Tan, Carol A. Remme, Pieter G. Postema, Mario Delmar, Patrick T. Ellinor, Steven A. Lubitz, Jean-Baptiste Gourraud, Michael W. Tanck, Alfred L. George Jr., Calum A. MacRae, Paul W. Burridge, Christian Dina, Vincent Probst, Arthur A. Wilde, Jean-Jacques Schott, Richard Redon &, and Connie R. Bezzina
Abstract: In the version of this article initially published, Federico Manevy’s name appeared with a middle initial in error. The name has been corrected in the HTML and PDF versions of the article.
Published: 2022

21. Genome-wide association analyses identify new Brugada syndrome risk loci and highlight a new mechanism of sodium channel regulation in disease susceptibility

Author: Barc, J, Tadros, R, Glinge, C, Chiang, D, Jouni, M, Simonet, F, Jurgens, S, Baudic, M, Nicastro, M, Potet, F, Offerhaus, J, Walsh, R, Hoan Choi, S, Verkerk, A, Mizusawa, Y, Anys, S, Minois, D, Arnaud, M, Duchateau, J, Wijeyeratne, Y, Muir, A, Papadakis, M, Castelletti, S, Torchio, M, Gil Ortuño, C, Lacunza, J, Giachino, D, Cerrato, N, Martins, R, Campuzano, O, Van Dooren, S, Thollet, A, Kyndt, F, Mazzanti, A, Clémenty, N, Bisson, A, Corveleyn, A, Stallmeyer, B, Dittmann, S, Saenen, J, Noël, A, Honarbakhsh, S, Rudic, B, Marzak, H, Rowe, M, Federspiel, C, Le Page, S, Placide, L, Milhem, A, Barajas-Martinez, H, Beckmann, B, Krapels, I, Steinfurt, J, Gregers Winkel, B, Jabbari, R, Shoemaker, M, Boukens, B, Škorić-Milosavljević, D, Bikker, H, Manevy, F, Lichtner, P, Ribasés, M, Meitinger, T, Müller-Nurasyid, M, Group, K, Veldink, J, van den Berg, L, Van Damme, P, Cusi, D, Lanzani, C, Rigade, S, Charpentier, E, Baron, E, Bonnaud, S, Lecointe, S, Donnart, A, Le Marec, H, Chatel, S, Karakachoff, M, Bézieau, S, London, B, Tfelt-Hansen, J, Roden, D, Odening, K, Cerrone, M, Chinitz, L, Volders, P, van de Berg, M, Laurent, G, Faivre, L, Antzelevitch, C, Kääb, S, Al Arnaout, A, Dupuis, J, Pasquie, J, Billon, O, Roberts, J, Jesel, L, Borggrefe, M, Lambiase, P, Mansourati, J, Loeys, B, Leenhardt, A, Guicheney, P, Maury, P, Schulze-Bahr, E, Robyns, T, Breckpot, J, Babuty, D, Priori, S, Napolitano, C, Referral Center for inherited cardiac arrhythmia, N, de Asmundis, C, Brugada, P, Brugada, R, Arbelo, E, Brugada, J, Mabo, P, Behar, N, Giustetto, C, Sabater Molina, M, Gimeno, J, Hasdemir, C, Schwartz, P, Crotti, L, Mckeown, P, Sharma, S, Behr, E, Haissaguerre, M, Sacher, F, Rooryck, C, Tan, H, Remme, C, Postema, P, Delmar, M, Ellinor, P, Lubitz, S, Gourraud, J, Tanck, M, L. George Jr., A, Macrae, C, Burridge, P, Dina, C, Probst, V, Wilde, A, Schott, J, Redon &amp, R, Bezzina, C, Julien Barc, Rafik Tadros, Charlotte Glinge, David Y. Chiang, Mariam Jouni, Floriane Simonet, Sean J. Jurgens, Manon Baudic, Michele Nicastro, Franck Potet, Joost A. Offerhaus, Roddy Walsh, Seung Hoan Choi, Arie O. Verkerk, Yuka Mizusawa, Soraya Anys, Damien Minois, Marine Arnaud, Josselin Duchateau, Yanushi D. Wijeyeratne, Alison Muir, Michael Papadakis, Silvia Castelletti, Margherita Torchio, Cristina Gil Ortuño, Javier Lacunza, Daniela F. Giachino, Natascia Cerrato, Raphaël P. Martins, Oscar Campuzano, Sonia Van Dooren, Aurélie Thollet, Florence Kyndt, Andrea Mazzanti, Nicolas Clémenty, Arnaud Bisson, Anniek Corveleyn, Birgit Stallmeyer, Sven Dittmann, Johan Saenen, Antoine Noël, Shohreh Honarbakhsh, Boris Rudic, Halim Marzak, Matthew K. Rowe, Claire Federspiel, Sophie Le Page, Leslie Placide, Antoine Milhem, Hector Barajas-Martinez, Britt-Maria Beckmann, Ingrid P. Krapels, Johannes Steinfurt, Bo Gregers Winkel, Reza Jabbari, Moore B. Shoemaker, Bas J. Boukens, Doris Škorić-Milosavljević, Hennie Bikker, Federico Manevy, Peter Lichtner, Marta Ribasés, Thomas Meitinger, Martina Müller-Nurasyid, KORA-Study Group, Jan H. Veldink, Leonard H. van den Berg, Philip Van Damme, Daniele Cusi, Chiara Lanzani, Sidwell Rigade, Eric Charpentier, Estelle Baron, Stéphanie Bonnaud, Simon Lecointe, Audrey Donnart, Hervé Le Marec, Stéphanie Chatel, Matilde Karakachoff, Stéphane Bézieau, Barry London, Jacob Tfelt-Hansen, Dan Roden, Katja E. Odening, Marina Cerrone, Larry A. Chinitz, Paul G. Volders, Maarten P. van de Berg, Gabriel Laurent, Laurence Faivre, Charles Antzelevitch, Stefan Kääb, Alain Al Arnaout, Jean-Marc Dupuis, Jean-Luc Pasquie, Olivier Billon, Jason D. Roberts, Laurence Jesel, Martin Borggrefe, Pier D. Lambiase, Jacques Mansourati, Bart Loeys, Antoine Leenhardt, Pascale Guicheney, Philippe Maury, Eric Schulze-Bahr, Tomas Robyns, Jeroen Breckpot, Dominique Babuty, Silvia G. Priori, Carlo Napolitano, Nantes Referral Center for inherited cardiac arrhythmia, Carlo de Asmundis, Pedro Brugada, Ramon Brugada, Elena Arbelo, Josep Brugada, Philippe Mabo, Nathalie Behar, Carla Giustetto, Maria Sabater Molina, Juan R. Gimeno, Can Hasdemir, Peter J. Schwartz, Lia Crotti, Pascal P. McKeown, Sanjay Sharma, Elijah R. Behr, Michel Haissaguerre, Frédéric Sacher, Caroline Rooryck, Hanno L. Tan, Carol A. Remme, Pieter G. Postema, Mario Delmar, Patrick T. Ellinor, Steven A. Lubitz, Jean-Baptiste Gourraud, Michael W. Tanck, Alfred L. George Jr., Calum A. MacRae, Paul W. Burridge, Christian Dina, Vincent Probst, Arthur A. Wilde, Jean-Jacques Schott, Richard Redon &, Connie R. Bezzina, Barc, J, Tadros, R, Glinge, C, Chiang, D, Jouni, M, Simonet, F, Jurgens, S, Baudic, M, Nicastro, M, Potet, F, Offerhaus, J, Walsh, R, Hoan Choi, S, Verkerk, A, Mizusawa, Y, Anys, S, Minois, D, Arnaud, M, Duchateau, J, Wijeyeratne, Y, Muir, A, Papadakis, M, Castelletti, S, Torchio, M, Gil Ortuño, C, Lacunza, J, Giachino, D, Cerrato, N, Martins, R, Campuzano, O, Van Dooren, S, Thollet, A, Kyndt, F, Mazzanti, A, Clémenty, N, Bisson, A, Corveleyn, A, Stallmeyer, B, Dittmann, S, Saenen, J, Noël, A, Honarbakhsh, S, Rudic, B, Marzak, H, Rowe, M, Federspiel, C, Le Page, S, Placide, L, Milhem, A, Barajas-Martinez, H, Beckmann, B, Krapels, I, Steinfurt, J, Gregers Winkel, B, Jabbari, R, Shoemaker, M, Boukens, B, Škorić-Milosavljević, D, Bikker, H, Manevy, F, Lichtner, P, Ribasés, M, Meitinger, T, Müller-Nurasyid, M, Group, K, Veldink, J, van den Berg, L, Van Damme, P, Cusi, D, Lanzani, C, Rigade, S, Charpentier, E, Baron, E, Bonnaud, S, Lecointe, S, Donnart, A, Le Marec, H, Chatel, S, Karakachoff, M, Bézieau, S, London, B, Tfelt-Hansen, J, Roden, D, Odening, K, Cerrone, M, Chinitz, L, Volders, P, van de Berg, M, Laurent, G, Faivre, L, Antzelevitch, C, Kääb, S, Al Arnaout, A, Dupuis, J, Pasquie, J, Billon, O, Roberts, J, Jesel, L, Borggrefe, M, Lambiase, P, Mansourati, J, Loeys, B, Leenhardt, A, Guicheney, P, Maury, P, Schulze-Bahr, E, Robyns, T, Breckpot, J, Babuty, D, Priori, S, Napolitano, C, Referral Center for inherited cardiac arrhythmia, N, de Asmundis, C, Brugada, P, Brugada, R, Arbelo, E, Brugada, J, Mabo, P, Behar, N, Giustetto, C, Sabater Molina, M, Gimeno, J, Hasdemir, C, Schwartz, P, Crotti, L, Mckeown, P, Sharma, S, Behr, E, Haissaguerre, M, Sacher, F, Rooryck, C, Tan, H, Remme, C, Postema, P, Delmar, M, Ellinor, P, Lubitz, S, Gourraud, J, Tanck, M, L. George Jr., A, Macrae, C, Burridge, P, Dina, C, Probst, V, Wilde, A, Schott, J, Redon &amp, R, Bezzina, C, Julien Barc, Rafik Tadros, Charlotte Glinge, David Y. Chiang, Mariam Jouni, Floriane Simonet, Sean J. Jurgens, Manon Baudic, Michele Nicastro, Franck Potet, Joost A. Offerhaus, Roddy Walsh, Seung Hoan Choi, Arie O. Verkerk, Yuka Mizusawa, Soraya Anys, Damien Minois, Marine Arnaud, Josselin Duchateau, Yanushi D. Wijeyeratne, Alison Muir, Michael Papadakis, Silvia Castelletti, Margherita Torchio, Cristina Gil Ortuño, Javier Lacunza, Daniela F. Giachino, Natascia Cerrato, Raphaël P. Martins, Oscar Campuzano, Sonia Van Dooren, Aurélie Thollet, Florence Kyndt, Andrea Mazzanti, Nicolas Clémenty, Arnaud Bisson, Anniek Corveleyn, Birgit Stallmeyer, Sven Dittmann, Johan Saenen, Antoine Noël, Shohreh Honarbakhsh, Boris Rudic, Halim Marzak, Matthew K. Rowe, Claire Federspiel, Sophie Le Page, Leslie Placide, Antoine Milhem, Hector Barajas-Martinez, Britt-Maria Beckmann, Ingrid P. Krapels, Johannes Steinfurt, Bo Gregers Winkel, Reza Jabbari, Moore B. Shoemaker, Bas J. Boukens, Doris Škorić-Milosavljević, Hennie Bikker, Federico Manevy, Peter Lichtner, Marta Ribasés, Thomas Meitinger, Martina Müller-Nurasyid, KORA-Study Group, Jan H. Veldink, Leonard H. van den Berg, Philip Van Damme, Daniele Cusi, Chiara Lanzani, Sidwell Rigade, Eric Charpentier, Estelle Baron, Stéphanie Bonnaud, Simon Lecointe, Audrey Donnart, Hervé Le Marec, Stéphanie Chatel, Matilde Karakachoff, Stéphane Bézieau, Barry London, Jacob Tfelt-Hansen, Dan Roden, Katja E. Odening, Marina Cerrone, Larry A. Chinitz, Paul G. Volders, Maarten P. van de Berg, Gabriel Laurent, Laurence Faivre, Charles Antzelevitch, Stefan Kääb, Alain Al Arnaout, Jean-Marc Dupuis, Jean-Luc Pasquie, Olivier Billon, Jason D. Roberts, Laurence Jesel, Martin Borggrefe, Pier D. Lambiase, Jacques Mansourati, Bart Loeys, Antoine Leenhardt, Pascale Guicheney, Philippe Maury, Eric Schulze-Bahr, Tomas Robyns, Jeroen Breckpot, Dominique Babuty, Silvia G. Priori, Carlo Napolitano, Nantes Referral Center for inherited cardiac arrhythmia, Carlo de Asmundis, Pedro Brugada, Ramon Brugada, Elena Arbelo, Josep Brugada, Philippe Mabo, Nathalie Behar, Carla Giustetto, Maria Sabater Molina, Juan R. Gimeno, Can Hasdemir, Peter J. Schwartz, Lia Crotti, Pascal P. McKeown, Sanjay Sharma, Elijah R. Behr, Michel Haissaguerre, Frédéric Sacher, Caroline Rooryck, Hanno L. Tan, Carol A. Remme, Pieter G. Postema, Mario Delmar, Patrick T. Ellinor, Steven A. Lubitz, Jean-Baptiste Gourraud, Michael W. Tanck, Alfred L. George Jr., Calum A. MacRae, Paul W. Burridge, Christian Dina, Vincent Probst, Arthur A. Wilde, Jean-Jacques Schott, Richard Redon &, and Connie R. Bezzina
Abstract: Brugada syndrome (BrS) is a cardiac arrhythmia disorder associated with sudden death in young adults. With the exception of SCN5A, encoding the cardiac sodium channel NaV1.5, susceptibility genes remain largely unknown. Here we performed a genome-wide association meta-analysis comprising 2,820 unrelated cases with BrS and 10,001 controls, and identified 21 association signals at 12 loci (10 new). Single nucleotide polymorphism (SNP)-heritability estimates indicate a strong polygenic influence. Polygenic risk score analyses based on the 21 susceptibility variants demonstrate varying cumulative contribution of common risk alleles among different patient subgroups, as well as genetic associations with cardiac electrical traits and disorders in the general population. The predominance of cardiac transcription factor loci indicates that transcriptional regulation is a key feature of BrS pathogenesis. Furthermore, functional studies conducted on MAPRE2, encoding the microtubule plus-end binding protein EB2, point to microtubule-related trafficking effects on NaV1.5 expression as a new underlying molecular mechanism. Taken together, these findings broaden our understanding of the genetic architecture of BrS and provide new insights into its molecular underpinnings.
Published: 2022

22. Robustness and relevance of predictive score in sudden cardiac death for patients with Brugada syndrome

Author: Thomas Goronflot, Aurélie Thollet, Vincent Probst, Matthieu Wargny, Paul Bru, S Anys, P. Berthome, Nathalie Behar, F. Kyndt, Jean-Marc Dupuis, Romain Tixier, Frederic Sacher, Jean-Baptiste Gourraud, Nicolas Clementy, Philippe Mabo, Pierre-Antoine Gourraud, Jacques Mansourati, Laurence Jesel, O. Geoffroy, J. Briand, and Béatrice Guyomarch
Subjects: Adult, Male, China, medicine.medical_specialty, 030204 cardiovascular system & hematology, Risk Assessment, Sudden cardiac death, Electrocardiography, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Humans, Medicine, 030212 general & internal medicine, Brugada Syndrome, Brugada syndrome, Framingham Risk Score, business.industry, Area under the curve, Mean age, Sudden cardiac arrest, Middle Aged, medicine.disease, Defibrillators, Implantable, Death, Sudden, Cardiac, Risk stratification, Cohort, Female, medicine.symptom, Cardiology and Cardiovascular Medicine, business
Abstract: Aims Risk stratification of sudden cardiac arrest (SCA) in Brugada syndrome (Brs) remains the main challenge for physicians. Several scores have been suggested to improve risk stratification but never replicated. We aim to investigate the accuracy of the Brs risk scores. Methods and results A total of 1613 patients [mean age 45 ± 15 years, 69% male, 323 (20%) symptomatic] were prospectively enrolled from 1993 to 2016 in a multicentric database. All data described in the risk score were double reviewed for the study. Among them, all patients were evaluated with Shanghai score and 461 (29%) with Sieira score. After a mean follow-up of 6.5 ± 4.7 years, an arrhythmic event occurred in 75 (5%) patients including 16 SCA, 11 symptomatic ventricular arrhythmia, and 48 appropriate therapies. Predictive capacity of the Shanghai score (n = 1613) and the Sieira (n = 461) score was, respectively, estimated by an area under the curve of 0.73 (0.67–0.79) and 0.71 (0.61–0.81). Considering Sieira score, the event rate at 10 years was significantly higher with a score of 5 (26.4%) than with a score of 0 (0.9%) or 1 (1.1%) (P Conclusions In the largest cohort of Brs patient ever described, risk scores do not allow stratifying the risk of arrhythmic event in intermediate-risk patient.
Published: 2020
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23. Long-Term Follow-Up of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator

Author: Victor Waldmann, Abdeslam Bouzeman, Guillaume Duthoit, Linda Koutbi, Francis Bessiere, Fabien Labombarda, Christelle Marquié, Jean Baptiste Gourraud, Pierre Mondoly, Jean Marc Sellal, Pierre Bordachar, Alexis Hermida, Frédéric Anselme, Anouk Asselin, Caroline Audinet, Yvette Bernard, Serge Boveda, Paul Bru, Sok Sithikun Bun, Gael Clerici, Antoine Da Costa, Maxime de Guillebon, Pascal Defaye, Nathalie Elbaz, Romain Eschalier, Rodrigue Garcia, Charles Guenancia, Benoit Guy-Moyat, Franck Halimi, Didier Irles, Laurence Iserin, François Jourda, Magalie Ladouceur, Philippe Lagrange, Mikael Laredo, Jacques Mansourati, Grégoire Massoulié, Amel Mathiron, Philippe Maury, Anne Messali, Kumar Narayanan, Cédric Nguyen, Sandro Ninni, Marie-Cécile Perier, Bertrand Pierre, Penelope Pujadas, Frédéric Sacher, Pascal Sagnol, Ardalan Sharifzadehgan, Camille Walton, Pierre Winum, Cyril Zakine, Laurent Fauchier, Raphael Martins, Jean Luc Pasquié, Jean Benoit Thambo, Xavier Jouven, Nicolas Combes, Eloi Marijon, Fabien Squara, Guillaume Theodore, Didier Scarlatti, Jérome Hourdain, Jean-Claude Deharo, Pierre Ollitrault, Paul Milliez, Laure Champ-Rigot, Hugues Bader, Cécile Duplantier, Antoine Milhem, Audrey Sagnard, Géraldine Bertaux, Gabriel Laurent, Marc Badoz, Agustin Bortone, Guillaume Laborie, Anne Rollin, Quentin Voglimacci Stephanopoli, Franck Mandel, Alexandre Duparc, Guillaume Domain, Jean-Paul Albenque, Christèle Cardin, Stéphane Combes, Nikita Tanese, Karim Hasni, Christophe Leclercq, Vincent Galand, Dominique Pavin, Philippe Mabo, Nathalie Behar, Nicolas Clementy, Christophe Loose, Akli Otmani, Sandrine Venier, Adrien Carabelli, Peggy Jacon, Mouna Ben Kilani, Jean Bapstist Guichard, Cécile Romeyer-Bouchard, Laurianne Le Gloan, Vincent Probst, Luc Freysz, Hugues Blangy, Christian De Chillou, Nicolas Sadoul, Pierre Khattar, Charlotte Potelle, Frederic Jean, Paul Puie, Ziad Khoueiry, Philippe Chevallier, Arnaud Dulac, Sylvie Di Filippo, Kevin Gardey, Pierre Frey, Chrystelle Akret, Antoine Dompnier, Carole Maupain, Xavier Waintraub, Françoise Hidden-Lucet, Thomas Chastre, Estelle Gandjbakhch, Nicolas Badenco, Fabrice Extramiana, Antoine Leenhardt, Amir Zouaghi, Vincent Algalarrondo, Denis Amet, Emilie Varlet, Tej Chalbia, Séverine Philibert, Jacky Ollitrault, Thomas Lavergne, Pierre Baudinaud, Adrian Mirolo, Arnaud Savouré, Bénédicte Godin, Cathy Bertrand, Pierre Fiorello, Nicolas Johnson, Pierre-Marc Lallemand, Alexis Herminda, Jean-Sylvain Hermida, Bruno Degand, Rim El Bouazzaou, Stéphane Mourot, Samuel Goussot, Gaël Jauvert, Arnaud Lazarus, Caroline Grimard, Christine Alonso, Alexandre Zhao, Olivier Thomas, Bruno Cauchemez, Ghassan Moubarak, Nicolas Lellouche, David Hamon, Bogdan Enache, Gabriel Lactu, Françoise Wiart, Olivier Geoffroy, Damien Poindron, Alice Maltret, Cristine Raimondo, Damien Bonnet, Sébastien Hascoët, Nicolas Derval, Michel Haïssaguerre, Mélèze Hocini, and Clinical sciences
Subjects: Adult, Male, medicine.medical_specialty, Long term follow up, Cyanotic congenital heart disease, medicine.medical_treatment, 030204 cardiovascular system & hematology, Sudden death, Sudden cardiac death, 03 medical and health sciences, 0302 clinical medicine, Physiology (medical), Internal medicine, medicine, Humans, Defibrillators, Implantable/trends, Registries, 030212 general & internal medicine, Tetralogy of Fallot, business.industry, medicine.disease, Implantable cardioverter-defibrillator, Tetralogy of Fallot/epidemiology, Defibrillators, Implantable, 3. Good health, Cardiology, Female, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract: Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. Methods: A Nationwide French Registry including all patients with tetralogy of Fallot with an ICD was initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event end point was the time from ICD implantation to first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee. Results: A total of 165 patients (mean age, 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (interquartile range) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least 1 appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively; P =0.03). Overall, 71 (43.0%) patients presented with at least 1 ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) patients in primary prevention, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with, respectively, 0, 1, 2, or ≥3 guidelines-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47 [95% CI, 1.19–10.11]), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 ( P =0.006). Patients with congestive heart failure or reduced left ventricular ejection fraction had a higher risk of nonarrhythmic death or heart transplantation (hazard ratio, 11.01 [95% CI, 2.96–40.95]). Conclusions: Patients with tetralogy of Fallot and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03837574.
Published: 2020
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24. Age at diagnosis of Brugada syndrome: Influence on clinical characteristics and risk of arrhythmia

Author: Jacques Mansourati, O. Geoffroy, Nicolas Clementy, Paul Bru, F. Kyndt, Jean-Baptiste Gourraud, Jean-Marc Dupuis, J. Briand, Aurélie Thollet, Frederic Sacher, Vincent Probst, Béatrice Guyomarch, P. Berthome, Philippe Mabo, Nathalie Behar, Laurence Jesel, Mathilde Minier, and Romain Tixier
Subjects: Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, Scn5a gene, Age at diagnosis, 030204 cardiovascular system & hematology, Risk Assessment, Sudden cardiac death, Electrocardiography, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Age groups, Physiology (medical), medicine, Humans, 030212 general & internal medicine, Aged, Brugada Syndrome, Retrospective Studies, Brugada syndrome, Aged, 80 and over, business.industry, Incidence, Age Factors, Middle Aged, Prognosis, medicine.disease, Defibrillators, Implantable, Survival Rate, Death, Sudden, Cardiac, Female, France, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract: Despite a strong genetic background, Brugada syndrome (BrS) mainly affects middle-age patients. Data are scarce in the youngest and oldest age groups.The purpose of this study was to describe the clinical characteristics and variations in rhythmic risk in BrS patients according to age.Consecutive BrS patients diagnosed in 15 French tertiary centers in France were enrolled from 1993 to 2016 and followed up prospectively. All of the clinical and ECG data were double reviewed.Among the 1613 patients enrolled (age 45 ± 15 years; 69% male), 3 groups were defined according to age (52 patients17 years; 1285 between 17 and 59 years; and 27660 years). In the youngest patients, we identified more female gender (42%), diagnosis by familial screening (63%), previous sudden cardiac death (15%), SCN5A mutation (62%) sinus dysfunction (8%) and aVR sign (37%) (P.001). The oldest patients had the same clinical characteristics except for gender (40% women; P.001). During median follow-up of 5.5 [2.1, 10.0] years, 91 patients experienced an arrhythmic event, including 7 (13%) in the youngest patients, 80 (6%) in middle-age patients, and 4 (1%) in the oldest patients. Annual event rates were 2.1%, 1%, and 0.3%, respectively (P.01).Age on diagnosis changes the clinical presentation of BrS. Although children are identified more during familial screening, they present the highest risk of sudden cardiac death, which is an argument for early and extensive familial screening. The oldest patients present the lowest risk of SCD.
Published: 2020
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25. Inappropriate therapy after catheter ablation and subcutaneous implantable cardioverter-defibrillator

Author: Damien Minois, Aude Solnon, Nicolas Malliet, Gilles Lande, Jean-Baptiste Gourraud, and Vincent Probst
Subjects: medicine.medical_specialty, business.industry, ICD, medicine.medical_treatment, Cardiology, Case Report, Catheter ablation, Implantable cardioverter-defibrillator, Surgery, S-ICD, Subcutaneous ICD, medicine, Cardiology and Cardiovascular Medicine, business, Oversensing, Inappropriate therapy
Published: 2020
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26. Sex Differences in Outcomes of Tetralogy of Fallot Patients With Implantable Cardioverter-Defibrillators

Author: Victor Waldmann, Abdeslam Bouzeman, Guillaume Duthoit, Linda Koutbi, Francis Bessière, Fabien Labombarda, Christelle Marquié, Jean-Baptiste Gourraud, Pierre Mondoly, Jean Marc Sellal, Pierre Bordachar, Alexis Hermida, Alain Al Arnaout, Frédéric Anselme, Caroline Audinet, Yvette Bernard, Serge Boveda, Sok Sithikun Bun, Morgane Chassignolle, Gaël Clerici, Antoine Da Costa, Maxime de Guillebon, Pascal Defaye, Nathalie Elbaz, Romain Eschalier, Rodrigue Garcia, Charles Guenancia, Benoit Guy-Moyat, Franck Halimi, Didier Irles, Laurence Iserin, François Jourda, Magalie Ladouceur, Philippe Lagrange, Mikael Laredo, Jacques Mansourati, Grégoire Massoulié, Amel Mathiron, Philippe Maury, Anne Messali, Kumar Narayanan, Cédric Nguyen, Sandro Ninni, Marie-Cécile Perier, Bertrand Pierre, Penelope Pujadas, Frédéric Sacher, Pascal Sagnol, Ardalan Sharifzadehgan, Camille Walton, Pierre Winum, Cyril Zakine, Laurent Fauchier, Raphaël Martins, Jean-Luc Pasquié, Jean-Benoit Thambo, Xavier Jouven, Nicolas Combes, Eloi Marijon, Chrystelle Akret, Jean-Paul Albenque, Vincent Algalarrondo, Christine Alonso, Denis Amet, Frédéric Ansselme, Anouk Asselin, Nicolas Badenco, Hugues Bader, Marc Badoz, Pierre Baudinaud, Nathalie Behar, Mouna Ben Kilani, Géraldine Bertaux, Cathy Bertrand, Francis Bessiere, Hughes Blangy, Damien Bonnet, Pierre Bordchar, Paul Bru, Adrien Carabelli, Christèle Cardin, Bruno Cauchemez, Tej Chalbia, Laure Champ-Rigot, Thomas Chastre, Philippe Chevallier, Nicolas Clementy, Stéphane Combes, Christian De Chillou, Maxime De Guillebon, Bruno Degand, Jean-Claude Deharo, Nicolas Derval, Sylvie Di Filippo, Guillaume Domain, Antoine Dompnier, Arnaud Dulac, Alexandre Duparc, D Cécile Duplantier, Rim El Bouazzaoui, Bogdan Enache, Fabrice Extramiana, Pierre Fiorello, Pierre Frey, Luc Freysz, Vincent Galand, Estelle Gandjbakhch, Kevin Gardey, Olivier Geoffroy, Bénédicte Godin, Samuel Goussot, Caroline Grimard, Jean-Baptiste Guichard, Michel Haissaguerre, David Hamon, Sébastien Hascoet, Karim Hasni, Jean-Sylvain Hermida, Françoise Hidden-Lucet, Mélèze Hocini, Jérome Hourdain, Peggy Jacon, Gaël Jauvert, Frédéric Jean, Nicolas Johnson, Pierre Khattar, Ziad Khoueiry, Rita Koutbi, Guillaume Laborie, Gabriel Lactu, Pierre-Marc Lallemand, Gabriel Laurent, Thomas Lavergne, Arnaud Lazarus, Laurianne Le Gloan, Christophe Leclercq, Antoine Leenhardt, Nicolas Lellouche, Christophe Loose, Philippe Mabo, Alice Maltret, Franck Mandel, Christelle Marquie, Carole Maupain, Antoine Milhelm, Paul Milliez, Adrian Mirolo, Ghassan Moubarak, Stéphane Mourot, Jacky Ollitrault, Pierre Ollitrault, Akli Otmani, Jean-Luc Pasquie, Dominique Pavin, Séverinne Philibert, Damien Poindron, Charlotte Potelle, Vincent Probst, Paul Puie, Anne Rollin, Cécile Romeyer-Bouchard, Nicolas Sadoul, Audrey Sagnard, Arnaud Savoure, Didier Scarlatti, Fabien Squara, Nikita Tanese, Jean Benoit Thambo, Olivier Thomas, Emie Varlet, Sandrine Venier, Quentin Voglimacci Stephanopoli, Xavier Waintraub, Françoise Wiart, Alexandre Zhao, Amir Zouaghi, Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), 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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), 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), 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), Clinique Pasteur, Clinique Pasteur [Toulouse], and This work was supported by the French Institute of Health and Medical Research, Fédération Française de Cardiologie, and Société Française de Cardiologie. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Subjects: Male, Adult, Heart Defects, Congenital, Sex Characteristics, sudden death, Middle Aged, congenital heart disease, Defibrillators, Implantable, Cohort Studies, implantable cardioverter-defibrillator, Death, Sudden, Cardiac, Tetralogy of Fallot, sex, Humans, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female, women, ventricular arrhythmia
Abstract: International audience; Background: Women with congenital heart disease at high risk for sudden cardiac death have been poorly studied thus far.Objectives: The aim of this study was to assess sex-related differences in patients with tetralogy of Fallot (TOF) and implantable cardioverter-defibrillators (ICDs).Methods: Data were analyzed from the DAI-T4F (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator) cohort study, which has prospectively enrolled all patients with TOF with ICDs in France since 2010. Clinical events were centrally adjudicated by a blinded committee.Results: A total of 165 patients (mean age 42.2 ± 13.3 years) were enrolled from 40 centers, including 49 women (29.7%). Among the 9,692 patients with TOF recorded in the national database, the proportion of women with ICDs was estimated to be 1.1% (95% CI: 0.8%-1.5%) vs 2.2% (95% CI: 1.8%-2.6%) in men (P < 0.001). The clinical profiles of patients at implantation, including the number of risk factors for ventricular arrhythmias, were similar between women and men. During a median follow-up period of 6.8 years (IQR: 2.5-11.4 years), 78 patients (47.3%) received at least 1 appropriate ICD therapy, without significant difference in annual incidences between women (12.1%) and men (9.9%) (HR: 1.22; 95% CI: 0.76-1.97; P = 0.40). The risk for overall ICD-related complications was similar in women and men (HR: 1.33; 95% CI: 0.81-2.19; P = 0.30), with 24 women (49.0%) experiencing at least 1 complication.Conclusions: Our findings suggest that women with TOF at high risk for sudden cardiac death have similar benefit/risk balance from ICD therapy compared with men. Whether ICD therapy is equally offered to at-risk women vs men warrants further evaluation in TOF as well as in other congenital heart disease populations. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574)
Published: 2022
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27. Outcome of patients with early repolarization pattern and syncope

Author: Tsukasa Kamakura, Jean-Baptiste Gourraud, Nicolas Clementy, Philippe Maury, Jacques Mansourati, Didier Klug, Antoine Da Costa, Jean-Luc Pasquie, Philippe Mabo, Pascal Chavernac, Gabriel Laurent, Pascal Defaye, Julien Laborderie, Antoine Leenhardt, Nicolas Sadoul, Jean-Claude Deharo, Cédric Giraudeau, Anne Quentin, Laurence Jesel, Aurelie Thollet, Romain Tixier, Nicolas Derval, Michel Haissaguerre, Vincent Probst, and Frederic Sacher
Subjects: Electrocardiography, Death, Sudden, Cardiac, Physiology (medical), Electrocardiography, Ambulatory, Humans, Arrhythmias, Cardiac, Cardiology and Cardiovascular Medicine, Syncope
Abstract: Syncope in patients with an early repolarization (ER) pattern presents a challenge for clinicians as it has been identified as an indicator of a higher risk of life-threatening ventricular arrhythmias (VAs).This study aimed to analyze the outcome of patients with an ER pattern and syncope and to evaluate the factors predictive of VAs.Over a period of 5 years, we enrolled 143 patients with an ER pattern and syncope in a multicenter prospective registry.After the initial examinations, 97 patients (67.8%) were implanted with a device allowing electrocardiogram monitoring, including 84 (58.7%) with an implantable loop recorder. During a mean follow-up period of 68 ± 34 months, we documented 16 arrhythmias presumably responsible for syncope (5 VAs, 10 bradycardias, and 1 supraventricular tachycardia). Additionally, recurrent syncope not associated with electrocardiogram documentation occurred in 16 patients (11.2%). The cause of syncope was identified in 23 of 97 patients with a monitoring device (23.8%). The 5-year incidence of VAs and arrhythmic events presumably responsible for syncope was 4.9% and 11.0%, respectively. Patients who developed VAs showed no prodromes or specific triggers at the time of syncope. Neither the presence of a family history of sudden cardiac death nor the previously reported high-risk electrocardiographic parameters differed between patients with and without VAs.VAs occurred in 4.9% of patients with an ER pattern and syncope. Device implantation based on detailed history taking seems to be a reasonable strategy. Previously reported high-risk electrocardiographic patterns did not identify patients with VAs.
Published: 2022

28. Atrial flutter radiofrequency ablation in the setting of left isomerism and repaired single atrium: First case

Author: Gilles Lande, Aude Solnon, Jean-Baptiste Gourraud, Marine Arnaud, Antoine Andorin, and Laurianne Le Gloan
Subjects: congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Radiofrequency ablation, Case Report, Intracardiac injection, law.invention, Peritricuspid atrial flutter, law, Internal medicine, Abnormal venous access, medicine, cardiovascular diseases, Conduction abnormalities, business.industry, Atrial arrhythmias, medicine.disease, Scar-related atrial flutter, Heterotaxy Syndrome, cardiovascular system, Cardiology, Single atrium, Cardiology and Cardiovascular Medicine, business, Left isomerism, Atrial flutter
Abstract: Left isomerism is a form of heterotaxy syndrome, characterized by intracardiac defects and caval venous variations, associated with an abnormal arrangement of bronchi and abdominal organs.1 Left isomerism is defined by symmetrical left anatomy of the atria and bronchi.2 Conduction abnormalities and atrial arrhythmias are known manifestations of this condition. However, to the best of our knowledge, atrial flutter is seldom associated with this anomaly.3 We here report for the first time a case of atrial flutter successfully treated by radiofrequency ablation in the setting of left isomerism and repaired single atrium.
Published: 2021

29. Impact of atrial tachycardia management on outcomes in adult patients with congenital heart disease

Author: Laurence Iserin, Mikael Laredo, Jean Gallet, Jean-Baptiste Gourraud, Laurianne Le Gloan, Denis Amet, E. Marijon, Magalie Ladouceur, Victor Waldmann, and Patrice Guérin
Subjects: Tachycardia, medicine.medical_specialty, Heart disease, business.industry, medicine.medical_treatment, Atrial fibrillation, Catheter ablation, medicine.disease, Cardioversion, Atrial arrhythmia, Direct current-cardioversion, Internal medicine, Heart failure, RC666-701, medicine, Cardiology, cardiovascular system, Diseases of the circulatory (Cardiovascular) system, cardiovascular diseases, medicine.symptom, business, Stroke, Atrial tachycardia, Congenital heart disease
Abstract: Background Atrial arrhythmias are common complications in adults with congenital heart disease (ACHD) and are associated with high morbi-mortality. Atrial tachycardia (AT) including intra-atrial reentrant tachycardia and focal atrial tachycardia are the most prevalent arrhythmias in ACHD. We aimed to compare mid-term outcomes according to arrhythmia management. Methods All hospitalizations for AT in ACHD were retrospectively identified from medico-administrative database from September 2006 to September 2016 in two centers. Outcomes of patients with AT managed with direct-current cardioversion (DCCV) were compared with patients referred for catheter ablation (CA). Atrial arrhythmia recurrence, stroke, admission for heart failure and death were analyzed during follow-up. Results One hundred and twenty-one ACHD with AT (median age 41 [IQR 32–52] years, male 62%, 41% moderate CHD and 38% complex CHD) were included. Median follow-up was 3.3 years [IQR 1.4–6.2]. Thirty-seven (30.5%) patients underwent DCCV and 84 (69.5%) CA. No significant difference in age, CHD complexity, history of atrial arrhythmia, heart failure, and CHA2DS2-VASC score were observe. Patients undergoing DCCV had higher NYHA class. Atrial arrhythmia recurrence was lower in CA group (Log-rank test, p = 0.045). Most recurrences were AT, but atrial fibrillation was recorded in 35.1% after DCCV and 9.5% after CA (p = 0.001). At the end of follow-up, patients who were initially treated by CA had a significantly lower incidence of severe cardiovascular events (48.6% in DCCV group vs. 16.7% in CA group, HR: 0.46, CI 95%: 0.22–0.93, p = 0.031). Conclusions A proactive management of AT by CA is associated with better long-term outcomes in ACHD.
Published: 2021

30. Generation of human induced pluripotent stem cell lines from three patients affected by Catecholaminergic Polymorphic ventricular tachycardia (CPVT) carrying heterozygous mutations in RYR2 gene

Author: Bastien Cimarosti, Robin Canac, Stephan De Waard, Aurore Girardeau, Anne Gaignerie, Aude Derevier, Virginie Forest, Michel Ronjat, Hervé Le Marec, Jean-Baptiste Gourraud, Patricia Lemarchand, Michel De Waard, Guillaume Lamirault, and Nathalie Gaborit
Subjects: QH301-705.5, Induced Pluripotent Stem Cells, Mutation, Leukocytes, Mononuclear, Tachycardia, Ventricular, Humans, Calcium, Ryanodine Receptor Calcium Release Channel, Cell Biology, General Medicine, Biology (General), Developmental Biology
Abstract: Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an exercise and emotional stress-induced life-threatening inherited heart rhythm disorder, characterized by an abnormal cellular calcium homeostasis. Most reported cases have been linked to mutations in the gene encoding the type 2 ryanodine receptor gene, RYR2. We generated induced pluripotent stem cells (hiPSCs) from peripheral blood mononuclear cells (PBMC) from three CPVT-affected patients, two of them carrying p.R4959Q mutation and one carrying p.Y2476D mutation. These generated hiPSC lines are a useful model to study pathophysiological consequences of RYR2 dysfunction in humans and the molecular basis of CPVT.
Published: 2021

31. Genotype-Phenotype Correlation of SCN5A Genotype in Patients With Brugada Syndrome and Arrhythmic Events: Insights From the SABRUS in 392 Probands

Author: Giulio Conte, Kengo Kusano, David C Johnson, P. Delise, Shingo Maeda, Domenico Corrado, Belinda Gray, Leonardo Calò, Gi-Byoung Nam, Ruben Casado-Arroyo, Georgia Sarquella-Brugada, Aviram Hochstadt, Jean-Baptiste Gourraud, Christian Veltmann, Jacob Tfelt-Hansen, Silvia G Priori, Camilla H Jespersen, Ramon Brugada, Kenzo Hirao, Anat Milman, Carla Giustetto, Yuka Mizusawa, Jimmy Jm Juang, Giuseppe Allocca, Vincent Probst, Antoine Leenhardt, Pieter G. Postema, Bernard Belhassen, Andrea Mazzanti, Pedro Brugada, Elijah R. Behr, Elena Arbelo, Josep Brugada, T Kamakura, Antoine Andorin, Masahiko Takagi, Isabelle Denjoy, Yoshihide Takahashi, Fiorenzo Gaita, Zhengrong Huang, Arthur A.M. Wilde, Sung Hwan Kim, Takeshi Aiba, Gan-Xin Yan, Cardiology, ACS - Heart failure & arrhythmias, Clinical sciences, Heartrhythmmanagement, and Cardio-vascular diseases
Subjects: Proband, congenital, hereditary, and neonatal diseases and abnormalities, genotype, Brugada syndrome, ethnic groups, mutation, sudden cardiac death, Sudden cardiac death, Genotype phenotype, Correlation, Genotype, medicine, In patient, cardiovascular diseases, Genetics, business.industry, General Medicine, medicine.disease, Mutation (genetic algorithm), cardiovascular system, Cardiology and Cardiovascular Medicine, business
Abstract: Background: Brugada syndrome (BrS) is associated with mutations in the cardiac sodium channel gene, SCN5A. However, genetic studies of patients with BrS with arrhythmic events have been limited. We sought to compare various clinical, ECG, and electrophysiological parameters according to SCN5A genotype in a large cohort of BrS probands with first arrhythmic event. Methods: Survey on Arrhythmic Events in Brugada Syndrome is a survey of 10 Western and 4 Asian countries, gathering 678 patients with BrS with first arrhythmic event. Only probands were included, and SCN5A genotype adjudicated. Patients without appropriate genetic data were excluded. Associations of genotype with clinical features were analyzed. Results: The study group comprised 392 probands: 92 (23.5%) SCN5A+ (44 pathogenic/likely pathogenic [P/LP] and 48 variants of unknown significance) and 300 (76.5%) SCN5A−. SCN5A missense variants and the patients hosting them were similar regardless of adjudication. A higher proportion of patients with P/LP were pediatric (SCN5A− (11.4% versus 3%, P =0.023). The proportion of females was higher among patients with P/LP compared with SCN5A − (18.2% versus 6.3%, P =0.013). P/LP probands were more likely to have a family history of sudden cardiac death compared with SCN5A − (41.9% versus 16.8%, P SCN5A− (87.5% versus 47%, P P P =0.009) were independent variables associated with P/LP genotype following logistic regression. Conclusions: The genetic basis of BrS has a complex relationship with gender, ethnicity, and age. Probands hosting a P/LP variant tended to experience their first arrhythmic event at a younger age and to have events triggered by fever compared with patients with SCN5A− . In addition, they were more likely to be White and to have family history of sudden cardiac death. Among females, a P/LP variant suggests an increased risk of being symptomatic. This association should be further studied on an ethnically specific basis in large prospectively collected international cohorts.
Published: 2021
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32. PO-625-06 CHARACTERISTICS AND PROGNOSIS OF THE CATECHOLAMINE INDUCED QT PROLONGATION SYNDROME

Author: JEAN BAPTISTE GOURRAUD, Jacques Mansourati, Nicolas Clementy, VINCENT PROBST, Raphael Martins, and Frederic Sacher
Subjects: Physiology (medical), Cardiology and Cardiovascular Medicine
Published: 2022
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33. BS-515-01 NON-CODING DELETION INDUCES 3D CHROMATIN REMODELLING AND PITX2 EXPRESSION DYSREGULATION ASSOCIATED WITH A SYNDROMIC CARDIAC DISORDER

Author: Manon Baudic, Hiroshige Murata, Fernanda M. Bosada, Uira Souto Melo, Taisuke Ishikawa, Takanori Aizawa, Amaury Guedon, Estelle Baron, ADRIEN FOUCAL, Pierre Lindenbaum, Solena Le Scouarnec, Wataru Shimizu, JEAN BAPTISTE GOURRAUD, Naomasa Makita, Herve Le Marec, Claude Vieyres, Stephan Mundlos, Vincent M. Christoffels, Takeru Makiyama, VINCENT PROBST, Jean-Jacques Schott, and Julien Barc
Subjects: Physiology (medical), Cardiology and Cardiovascular Medicine
Published: 2022
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34. BS-513-03 SCN5A MUTATIONS AND THE ROLE OF GENETIC BACKGROUND IN THE PATHOPHYSIOLOGY OF BRUGADA SYNDROME

Author: Vincent Probst, Sylvie Geoffroy, Philippe MABO, Jacques Mansourati, Jean-Luc Pasquie, Alain Al Arnaout, Dewey Petit, Olivier Billon, Frederic Sacher, and Jean Baptiste Gourraud
Subjects: Physiology (medical), Cardiology and Cardiovascular Medicine
Published: 2022
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35. Left Ventricular Assist Device Implantation As a Bailout Strategy for the Management of Refractory Electrical Storm and Cardiogenic Shock

Author: Frederic Sacher, Raphaël P. Martins, Baptiste Maille, Christian de Chillou, Pierre Baudinaud, Jean-Baptiste Gourraud, Karim Benali, Francis Bessière, Philippe Maury, Vincent Galand, Vincent Algalarrondo, Charles Guenancia, Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Hôpital de la Timone [CHU - APHM] (TIMONE), Service de chirurgie cardio-vasculaire et thoracique (CHU Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre hospitalier universitaire de Nantes (CHU Nantes), 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), and Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Male, medicine.medical_specialty, medicine.medical_treatment, Shock, Cardiogenic, Hemodynamics, Catheter ablation, 030204 cardiovascular system & hematology, Ventricular tachycardia, hemodynamics, Ventricular Function, Left, 03 medical and health sciences, 0302 clinical medicine, Refractory, Physiology (medical), Internal medicine, catheter ablation, medicine, Humans, 030212 general & internal medicine, hospital, ComputingMilieux_MISCELLANEOUS, business.industry, Cardiogenic shock, informed consent, Middle Aged, medicine.disease, Treatment Outcome, Ventricular assist device, Tachycardia, Ventricular, Cardiology, Female, [SDV.IB]Life Sciences [q-bio]/Bioengineering, Heart-Assist Devices, ventricular tachycardia, Cardiology and Cardiovascular Medicine, business, Bailout
Abstract: International audience
Published: 2021
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36. Genotype-Phenotype Correlation of

Author: Anat, Milman, Elijah R, Behr, Belinda, Gray, David C, Johnson, Antoine, Andorin, Aviram, Hochstadt, Jean-Baptiste, Gourraud, Shingo, Maeda, Yoshihide, Takahashi, Jimmy, Jm Juang, Sung-Hwan, Kim, Tsukasa, Kamakura, Takeshi, Aiba, Pieter G, Postema, Yuka, Mizusawa, Isabelle, Denjoy, Carla, Giustetto, Giulio, Conte, Zhengrong, Huang, Georgia, Sarquella-Brugada, Andrea, Mazzanti, Camilla H, Jespersen, Elena, Arbelo, Ramon, Brugada, Leonardo, Calo, Domenico, Corrado, Ruben, Casado-Arroyo, Giuseppe, Allocca, Masahiko, Takagi, Pietro, Delise, Josep, Brugada, Jacob, Tfelt-Hansen, Silvia G, Priori, Christian, Veltmann, Gan-Xin, Yan, Pedro, Brugada, Fiorenzo, Gaita, Antoine, Leenhardt, Arthur A M, Wilde, Kengo F, Kusano, Gi-Byoung, Nam, Kenzo, Hirao, Vincent, Probst, and Bernard, Belhassen
Subjects: Adult, Male, Electrocardiography, Sex Factors, Adolescent, Genotype, Humans, Female, Middle Aged, Aged, Brugada Syndrome, NAV1.5 Voltage-Gated Sodium Channel
Abstract: Brugada syndrome (BrS) is associated with mutations in the cardiac sodium channel gene,Survey on Arrhythmic Events in Brugada Syndrome is a survey of 10 Western and 4 Asian countries, gathering 678 patients with BrS with first arrhythmic event. Only probands were included, andThe study group comprised 392 probands: 92 (23.5%)The genetic basis of BrS has a complex relationship with gender, ethnicity, and age. Probands hosting a P/LP variant tended to experience their first arrhythmic event at a younger age and to have events triggered by fever compared with patients with
Published: 2021

37. Does sports participation increase risk in patients with long QT syndrome? Results from a large French cohort

Author: Caroline Davydoff, Antoine Andorin, Damien Minois, Marine Arnaud, Mathilde Minier, Frédéric Sacher, Raphael Martins, Nicolas Clementy, Jean Baptiste Gourraud, and Vincent Probst
Subjects: Adult, Male, Electrocardiography, Long QT Syndrome, Physiology (medical), Humans, Female, Cardiology and Cardiovascular Medicine, Defibrillators, Implantable, Retrospective Studies, Sports
Abstract: Aims Sports practice, especially in competition, is usually restrained in patients diagnosed with long QT syndrome (LQTS). Although data are scarce, a low incidence of cardiac arrhythmic events (CAEs) during sports practice is reported. We aim to evaluate the incidence of CAE during sports practice in LQTS patients. Methods and results All consecutive patients above 18 years of age diagnosed with LQTS and prospectively followed at the referral centre for inherited arrhythmia syndrome received a survey to retrospectively assess their sports practice prior to and after the diagnosis of LQTS. Two hundred and forty-six patients were included (57% females). The median age was 43 years, and the median QTc was 457 ms (428; 482). Patients reported a total of 4092 years [1376 (34%) after diagnosis] of sports practice: 2905 (77%) [1138 (39%) after diagnosis] years of leisure practice and 1187 (23%) [238 (20%) after diagnosis] years of competitive practice. One hundred and eighty (73%) patients practiced sport prior to the diagnosis of LQTS and 170 (69%) after. Prior to the diagnosis, four (2%) patients presented a CAE during leisure sports practice and one during competition. After diagnosis, only one patient presented a CAE, appropriately treated by an implantable cardioverter defibrillator discharge, in the context of beta-blocker non-compliance. The CAE event rate was 0.0007 events/year in the 1376 years of total sports practice after the diagnosis of LQTS. Conclusion After the diagnosis of LQTS, the occurrence of CAE is very low during sports practice, even in competitive practice. There was no CAE in patients properly treated with beta-blocker therapy with good compliance.
Published: 2021

38. A consistent arrhythmogenic trait in Brugada syndrome cellular phenotype

Author: Aude Derevier, Robin Canac, Isabelle Baró, Guillaume Lamirault, Vincent Probst, Jean-Jacques Schott, Anne Gaignerie, Aurore Girardeau, Richard Redon, Julien Barc, Flavien Charpentier, Bastien Cimarosti, Patricia Lemarchand, Nathalie Gaborit, Virginie Forest, Zeina R Al Sayed, Nadjet Belbachir, Mariam Jouni, Gildas Loussouarn, Kazem Zibara, Pierre Olchesqui, Eric Charpentier, Jean-Baptiste Gourraud, Caroline Chariau, unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Structure fédérative de recherche François Bonamy (SFR François Bonamy), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche en Santé de l'Université de Nantes (IRS-UN), Lebanese University [Beirut] (LU), gaborit, nathalie, Unité de recherche de l'institut du thorax (ITX-lab), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), and Université de Nantes (UN)-Université de Nantes (UN)
Subjects: Medicine (General), Heart Ventricles, [SDV]Life Sciences [q-bio], Induced Pluripotent Stem Cells, Medicine (miscellaneous), 030204 cardiovascular system & hematology, Letter to Editor, NAV1.5 Voltage-Gated Sodium Channel, 03 medical and health sciences, R5-920, 0302 clinical medicine, Text mining, Humans, Medicine, Myocytes, Cardiac, ComputingMilieux_MISCELLANEOUS, Brugada Syndrome, 030304 developmental biology, Brugada syndrome, Genetics, 0303 health sciences, business.industry, Gene Expression Profiling, Sodium, Electric Conductivity, Arrhythmias, Cardiac, medicine.disease, Cellular phenotype, [SDV] Life Sciences [q-bio], Phenotype, Gene Expression Regulation, Case-Control Studies, Mutation, Trait, Molecular Medicine, business, Biomarkers
Abstract: International audience
Published: 2021
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39. Replacement Myocardial Fibrosis in Patients With Mitral Valve Prolapse: Relation to Mitral Regurgitation, Ventricular Remodeling, and Arrhythmia

Author: Laura Filippetti, J.M. Serfaty, Nicolas Piriou, Pierre-Yves Marie, Jean-Noël Trochu, Gabriella Hossu, Karine Warin-Fresse, Thomas Senage, Antoine Jobbe-Duval, Clément Venner, Claire Toquet, Marine Beaumont, Marie Marrec, Solena Le Scouarnec, Thierry Le Tourneau, Romain Capoulade, Christine Selton-Suty, Caroline Cueff, Aurélie Thollet, Jean-Marc Sellal, Jean-Christian Roussel, Anne-Laure Constant Dit Beaufils, Jean-Jacques Schott, Jean-Baptiste Gourraud, Damien Mandry, Olivier Huttin, Centre hospitalier universitaire de Nantes (CHU Nantes), Université de Nantes (UN), Service de Cardiologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Institut Lorrain du Coeur et des Vaisseaux Louis Mathieu [Nancy], Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre hospitalier universitaire de Nantes (CHU Nantes), unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Imagerie Adaptative Diagnostique et Interventionnelle (IADI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Centre d'Investigation Clinique - Innovation Technologique [Nancy] (CIC-IT), Centre d'investigation clinique [Nancy] (CIC), and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)
Subjects: Male, medicine.medical_specialty, Arterial embolism, [SDV.IB.MN]Life Sciences [q-bio]/Bioengineering/Nuclear medicine, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Fibrosis, Physiology (medical), Internal medicine, medicine, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, Mitral valve prolapse, Humans, In patient, 030212 general & internal medicine, cardiovascular diseases, Ventricular remodeling, Mitral regurgitation, Mitral Valve Prolapse, Ventricular Remodeling, business.industry, Myocardium, Mitral Valve Insufficiency, Arrhythmias, Cardiac, Middle Aged, medicine.disease, 3. Good health, Echocardiography, Heart failure, Cardiology, cardiovascular system, Myocardial fibrosis, Female, Cardiology and Cardiovascular Medicine, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders, and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance in patients with MVP. Methods: Four hundred patients (53±15 years of age, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE cardiac magnetic resonance, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 with myocardial wall including 71 with basal inferolateral wall, 29 with papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate MR, and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45% versus 26%, P P =0.009) and moderate-severe MR (odds ratio, 2.28 [95% CI, 1.21–4.31], P =0.011). LGE+ was associated with worse 4-year cardiovascular event–free survival (49.6±11.7 in LGE+ versus 73.3±6.5% in LGE–, P P =0.002) were associated with poor outcome. Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP; is associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia; and is independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease. Last, cardiac magnetic resonance provides additional information to echocardiography in MVP.
Published: 2021
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40. Impact of pulmonary valve replacement on ventricular arrhythmias in patients with tetralogy of Fallot

Author: Jean-Baptiste Gourraud, Linda Koutbi, S Di Filippo, Pierre Bordachar, Nicolas Combes, Kévin Gardey, Guillaume Duthoit, Fabien Labombarda, Francis Bessière, Victor Waldmann, Pierre Mondoly, Christelle Marquié, Jean-Luc Pasquié, R Martins, and E. Marijon
Subjects: medicine.medical_specialty, business.industry, medicine.disease, Sudden cardiac death, Implantable defibrillators, Cardiovascular monitoring, Physiology (medical), Pulmonary Valve Replacement, Internal medicine, Primary prevention, medicine, Cardiology, In patient, Cardiology and Cardiovascular Medicine, business, Tetralogy of Fallot, Cause of death
Abstract: Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): INSERM - French Society of Cardiology OnBehalf DAIT4F Investigators Background Sudden cardiac death is a major cause of death in tetralogy of Fallot (TOF) and right ventricular overload is commonly considered as a potential trigger for ventricular arrhythmias. Purpose We aimed to assess the impact of pulmonary valve replacement (PVR) on ventricular arrhythmias burden using a population of TOF patients with continuous cardiac monitoring by implantable cardioverter defibrillator (ICD). Methods Nationwide French registry including all TOF patients with an ICD. Survival data with recurrent events were used to compare the burden of appropriate ICD therapies before and after PVR in patients who underwent PVR over the study period. Results A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% males) were included from 40 centers. Over a median (IQR) follow-up period of 6.8 (2.5-11.4) years, 26 (15.8%) patients underwent PVR. Among those patients, 18 (69.2%) experienced at least one appropriate ICD therapy. When considering all ICD therapies delivered before (n = 62) and after (n = 16) PVR, the burden of ICD appropriate therapies was significantly lower after PVR (HR 0.21, 95%CI 0.08-0.56, p = 0.002). In the overall cohort, PVR before ICD implantation was also independently associated with a lower risk of appropriate ICD therapy in primary prevention patients (HR 0.29, 95%CI 0.10-0.89, p = 0.031). Conclusions In this cohort of high-risk TOF patients implanted with an ICD, the burden of appropriate ICD therapies was significantly reduced after PVR. While optimal indications and timing for PVR are debated, these findings suggest the importance of considering ventricular arrhythmias in the overall making-decision process. Abstract Figure.
Published: 2021
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41. Impact of Pulmonary Valve Replacement on Ventricular Arrhythmias in Patients With Tetralogy of Fallot and Implantable Cardioverter-Defibrillator

Author: Cédric Nguyen, François Jourda, Alexis Hermida, Pascal Defaye, Frederic Sacher, Yvette Bernard, Serge Boveda, Marie-Cécile Perier, Philippe Chevalier, Pierre Winum, Mikael Laredo, Abdeslam Bouzeman, C. Marquie, Raphaël P. Martins, Jean Benoit Thambo, Laurence Iserin, Maxime De Guillebon, Kévin Gardey, Antoine Da Costa, Pierre Bordachar, Benoit Guy-Moyat, Anouk Asselin, Jean Marc Sellal, Francis Bessière, Linda Koutbi, Pierre Mondoly, Victor Waldmann, Grégoire Massoulié, Jacques Mansourati, Roland Henaine, Romain Eschalier, Fabien Labombarda, Sandro Ninni, Charles Guenancia, Sylvie Di Filippo, Philippe Maury, Rodrigue Garcia, Bertrand Pierre, Magalie Ladouceur, Caroline Audinet, Amel Mathiron, Camille Walton, Philippe Lagrange, Guillaume Duthoit, Xavier Jouven, Didier Irles, Nicolas Combes, Jean Luc Pasquié, G Clerici, Frédéric Anselme, Jean-Baptiste Gourraud, Eloi Marijon, Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital de la Timone [CHU - APHM] (TIMONE), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre hospitalier universitaire de Nantes (CHU Nantes), CHU Toulouse [Toulouse], Imagerie Adaptative Diagnostique et Interventionnelle (IADI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), CHU Bordeaux [Bordeaux], CHU Amiens-Picardie, CHU Rouen, Normandie Université (NU), Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), 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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Groupe Hospitalier Bretagne Sud (GHBS), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Clinique Pasteur [Toulouse], CHU Sud Saint Pierre [Ile de la Réunion], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Centre hospitalier de Pau, Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Clermont-Ferrand, Centre hospitalier universitaire de Poitiers (CHU Poitiers), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CHU Limoges, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre Hospitalier Chalon-sur-Saône William Morey, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Clinical sciences, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), SELLAL, Jean-Marc, and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Adult, Male, medicine.medical_specialty, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Population, 030204 cardiovascular system & hematology, Sudden death, Sudden cardiac death, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Interquartile range, Internal medicine, medicine, Humans, Implantable cardioverter defibrilator, 030212 general & internal medicine, Cardiac Surgical Procedures, education, Pulmonary Valve/surgery, ComputingMilieux_MISCELLANEOUS, Tetralogy of Fallot, Cause of death, Pulmonary Valve, education.field_of_study, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, business.industry, Ventriculat arrhythmia, Arrhythmias, Cardiac, Middle Aged, medicine.disease, Implantable cardioverter-defibrillator, eye diseases, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Defibrillators, Implantable, 3. Good health, Tetralogy of Fallot/surgery, Arrhythmias, Cardiac/therapy, Pulmonary valve replacement, Cohort, Cardiology, Female, Cardiology and Cardiovascular Medicine, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; Objectives: This study aimed to assess the impact of pulmonary valve replacement (PVR) on ventricular arrhythmias burden in a population of tetralogy of Fallot (TOF) patients with continuous cardiac monitoring by implantable cardioverter-defibrillators (ICDs).Background: Sudden cardiac death is a major cause of death in TOF, and right ventricular overload is commonly considered to be a potential trigger for ventricular arrhythmias.Methods: Data were analyzed from a nationwide French ongoing study (DAI-T4F) including all TOF patients with an ICD since 2000. Survival data with recurrent events were used to compare the burden of appropriate ICD therapies before and after PVR in patients who underwent PVR over the study period.Results: A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% male) were included from 40 centers. Over a median follow-up period of 6.8 (interquartile range: 2.5 to 11.4) years, 26 patients (15.8%) underwent PVR. Among those patients, 18 (69.2%) experienced at least 1 appropriate ICD therapy. When considering all ICD therapies delivered before (n = 62) and after (n = 16) PVR, the burden of appropriate ICD therapies was significantly lower after PVR (HR: 0.21; 95% confidence interval [CI]: 0.08 to 0.56; p = 0.002). Respective appropriate ICD therapies rates per 100 person-years were 44.0 (95% CI: 35.7 to 52.5) before and 13.2 (95% CI: 7.7 to 20.5) after PVR (p < 0.001). In the overall cohort, PVR before ICD implantation was also independently associated with a lower risk of appropriate ICD therapy in primary prevention patients (HR: 0.29 [95% CI: 0.10 to 0.89]; p = 0.031).Conclusions: In this cohort of high-risk TOF patients implanted with an ICD, the burden of appropriate ICD therapies was significantly reduced after PVR. While optimal indications and timing for PVR are debated, these findings suggest the importance of considering ventricular arrhythmias in the overall decision-making process. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574).
Published: 2021
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42. Dose response to nadolol in congenital long QT syndrome

Author: Damien Minois, Annabelle Rajalu, Vincent Probst, S Anys, Aurélie Thollet, Jean-Baptiste Gourraud, Marine Arnaud, and Béatrice Guyomarch
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, medicine.drug_class, Long QT syndrome, 030204 cardiovascular system & hematology, 03 medical and health sciences, Electrocardiography, Young Adult, 0302 clinical medicine, Nadolol, Heart Rate, Physiology (medical), Internal medicine, Heart rate, Medicine, Humans, Cycle exercise, 030212 general & internal medicine, Prospective Studies, Child, Beta blocker, Dose-Response Relationship, Drug, business.industry, Infant, Middle Aged, medicine.disease, Response to treatment, Adaptation, Physiological, Congenital long QT syndrome, Long QT Syndrome, Heart rate change, Child, Preschool, Injections, Intravenous, Cardiology, Exercise Test, Female, Cardiology and Cardiovascular Medicine, business, Anti-Arrhythmia Agents, medicine.drug, Follow-Up Studies
Abstract: Beta-blocker therapy is the cornerstone of treatment for patients with long QT syndrome (LQTS). Few details on the dose to be used are available. As the response is variable between patients, we systematically evaluated the effect of treatment by performing an exercise test.The purpose of this study was to explore dose response to nadolol on exercise test in LQTS patients in order to propose a more personalized therapeutic approach.LQTS patients followed at the Reference Centre for Hereditary Arrhythmic Diseases of Nantes with at least 1 exercise test under nadolol were included retrospectively between 1993 and 2017. All patients underwent gradual cycle exercise tests. Doses adjusted to weight and response to treatment were recorded and evaluated by the percentage of age-predicted maximum heart rate reached on exercise test.Ninety-five patients were included in the study, and 337 stress tests under nadolol were analyzed. No correlation existed between dose and percentage of age-predicted maximum heart rate on exercise tests. Twenty-one patients were overresponders, mostly LQTS1, and 20 were underresponders, mainly LQTS2 (P = .0229). Forty-two patients had at least 3 stress tests under nadolol. We found a negative correlation between dose change and percentage of age-predicted maximum heart rate change (P.0001). We then proposed a table to adapt dose according to exercise test response.Our study demonstrated a major variability of dose response to nadolol in patients with LQTS, thus underlining the need for a tailored dosage for each patient. Intraindividual analysis showed a relatively constant dose-response relationship, allowing guided dose adaptation after the first exercise test.
Published: 2021

43. Long-term follow-up of patients with tetralogy of fallot and implantable cardioverter defibrillator–The DAI-T4F nationwide registry

Author: Guillaume Duthoit, Mikael Laredo, Antoine Da Costa, Romain Eschalier, Fabien Labombarda, Laurent Fauchier, Benoit Guy-Moyat, Laurence Iserin, Pierre Winum, Marie-Cécile Perier, Philippe Maury, Nicolas Combes, Victor Waldmann, Didier Irles, Philippe Lagrange, Magalie Ladouceur, Jean-Benoit Thambo, Bertrand Pierre, Eloi Marijon, Jean-Marc Sellal, Grégoire Massoulié, Maxime De Guillebon, Ardalan Sharifzadehgan, Jacques Mansourati, Sandro Ninni, Penelope Pujadas, Linda Koutbi, Anouk Asselin, Pascal Sagnol, Nathalie Elbaz, Rodrigue Garcia, Dai-T F investigators, Caroline Audinet, G Clerici, Kumar Narayanan, Amel Mathiron, Xavier Jouven, Frédéric Anselme, Camille Walton, Anne Messali, Jean-Baptiste Gourraud, Yvette Bernard, Charles Guenancia, Alexis Hermida, Raphaël P. Martins, Pierre Bordachar, Cyril Zakine, Francis Bessière, Pierre Mondoly, Franck Halimi, Paul Bru, C. Marquie, François Jourda, Pascal Defaye, Frederic Sacher, Sok Sithikun Bun, Jean-Luc Pasquié, Cédric Nguyen, and Abdeslam Bouzeman
Subjects: medicine.medical_specialty, Long term follow up, business.industry, medicine.medical_treatment, Area under the curve, Qrs fragmentation, Implantable cardioverter-defibrillator, medicine.disease, Sudden cardiac death, Internal medicine, medicine, In patient, Cardiology and Cardiovascular Medicine, Complication, business, Tetralogy of Fallot
Abstract: Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. Objective We aimed to describe long-term follow-up of TOF patients implanted with ICD through a nationwide French registry. Methods Nationwide French Registry including all TOF patients with an ICD initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event endpoint was the time from ICD implantation to first appropriate ICD therapy. Clinical events were centrally adjudicated by a blinded committee. Results A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (IQR) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least one appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively, P = 0.03). Overall, 71 (43.0%) patients presented with at least one ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) primary prevention patients, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with respectively no, one, two, or ≥ three guideline-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (HR 3.47, 95% CI 1.19–10.11), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 (P = 0.006) ( Fig. 1 ). Conclusions Patients with TOF and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification.
Published: 2021
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44. SCN5A Mutation Type and a Genetic Risk Score Associate Variably with Brugada Syndrome Phenotype in SCN5A Families

Author: Peter Lichtner, Thomas Meitinger, Wataru Shimizu, Alison Muir, F. Kyndt, Michael W.T. Tanck, Seiko Ohno, Martina Muggenthaler, Michael J. Ackerman, Vincent Probst, Stephen P. Page, Jean-Jacques Schott, Silvia Castelletti, Hariharan Raju, Jean-Baptiste Gourraud, Joseph Galvin, Taisuke Ishikawa, Eline A. Nannenberg, Dan M. Roden, Doris Škorić-Milosavljević, Kazuhiro Takahashi, Pascal P. McKeown, Federica Dagradi, Lia Crotti, Yanushi D. Wijeyeratne, Julien Barc, Yuka Mizusawa, Peter J. Schwartz, Michael Papadakis, Margherita Torchio, Sanjay Sharma, Velislav N. Batchvarov, Naomasa Makita, Richard Redon, Christian Veltmann, Elijah R. Behr, Takeshi Aiba, Martin Borggrefe, Rafik Tadros, Connie R. Bezzina, J. Martijn Bos, David J. Tester, Isabelle Denjoy, Minoru Horie, Arthur A.M. Wilde, St George's, University of London, Academic Medical Center - Academisch Medisch Centrum [Amsterdam] (AMC), University of Amsterdam [Amsterdam] (UvA), unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Centro Cardiologico Monzino [Milano], Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS)-Dpt di Scienze Cliniche e di Comunità [Milano] (DISCCO), Università degli Studi di Milano [Milano] (UNIMI)-Università degli Studi di Milano [Milano] (UNIMI), Mayo Clinic [Rochester], Belfast Health and Social Care Trust, Hannover Medical School [Hannover] (MHH), University of Shiga Prefecture, National Cerebral and Cardiovascular Center (NCCC - OSAKA), Osaka University [Osaka], Leeds Teaching Hospitals NHS Trust, University of Dublin, 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], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Helmholtz-Zentrum München (HZM), Technische Universität Munchen - Université Technique de Munich [Munich, Allemagne] (TUM), Iwate Prefectural University [Takizawa], Vanderbilt University School of Medicine [Nashville], University of Heidelberg, Medical Faculty, Wijeyeratne, Y, Tanck, M, Mizusawa, Y, Batchvarov, V, Barc, J, Crotti, L, Bos, J, Tester, D, Muir, A, Veltmann, C, Ohno, S, Page, S, Galvin, J, Tadros, R, Muggenthaler, M, Raju, H, Denjoy, I, Schott, J, Gourraud, J, Skoric-Milosavljevic, D, Nannenberg, E, Redon, R, Papadakis, M, Kyndt, F, Dagradi, F, Castelletti, S, Torchio, M, Meitinger, T, Lichtner, P, Ishikawa, T, Wilde, A, Takahashi, K, Sharma, S, Roden, D, Borggrefe, M, Mckeown, P, Shimizu, W, Horie, M, Makita, N, Aiba, T, Ackerman, M, Schwartz, P, Probst, V, Bezzina, C, Behr, E, Unité de recherche de l'institut du thorax (ITX-lab), Dpt di Scienze Cliniche e di Comunità [Milano] (DISCCO), Università degli Studi di Milano = University of Milan (UNIMI)-Università degli Studi di Milano = University of Milan (UNIMI)-Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS), 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), 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)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Helmholtz Zentrum München = German Research Center for Environmental Health, HAL-SU, Gestionnaire, Epidemiology and Data Science, APH - Methodology, Graduate School, ACS - Heart failure & arrhythmias, Amsterdam Reproduction & Development (AR&D), Human Genetics, Cardiology, ACS - Pulmonary hypertension & thrombosis, and ACS - Atherosclerosis & ischemic syndromes
Subjects: genetics, human, congenital, hereditary, and neonatal diseases and abnormalities, Scn5a gene, phenotype, BIO/18 - GENETICA, 030204 cardiovascular system & hematology, risk score, 03 medical and health sciences, 0302 clinical medicine, Brugada Syndrome, Genetics, Human, Penetrance, Phenotype, Risk Score, Medicine, genetics, Brugada syndrome, 030212 general & internal medicine, human, cardiovascular diseases, Genetic risk, penetrance, Genetics, Framingham Risk Score, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, business.industry, fungi, General Medicine, Original Articles, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Human genetics, 3. Good health, Brugada ECG Pattern, ComputingMethodologies_DOCUMENTANDTEXTPROCESSING, cardiovascular system, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Supplemental Digital Content is available in the text., Background: Brugada syndrome (BrS) is characterized by the type 1 Brugada ECG pattern. Pathogenic rare variants in SCN5A (mutations) are identified in 20% of BrS families in whom incomplete penetrance and genotype-negative phenotype-positive individuals are observed. E1784K-SCN5A is the most common SCN5A mutation identified. We determined the association of a BrS genetic risk score (BrS-GRS) and SCN5A mutation type on BrS phenotype in BrS families with SCN5A mutations. Methods: Subjects with a spontaneous type 1 pattern or positive/negative drug challenge from cohorts harboring SCN5A mutations were recruited from 16 centers (n=312). Single nucleotide polymorphisms previously associated with BrS at genome-wide significance were studied in both cohorts: rs11708996, rs10428132, and rs9388451. An additive linear genetic model for the BrS-GRS was assumed (6 single nucleotide polymorphism risk alleles). Results: In the total population (n=312), BrS-GRS ≥4 risk alleles yielded an odds ratio of 4.15 for BrS phenotype ([95% CI, 1.45–11.85]; P=0.0078). Among SCN5A-positive individuals (n=258), BrS-GRS ≥4 risk alleles yielded an odds ratio of 2.35 ([95% CI, 0.89–6.22]; P=0.0846). In SCN5A-negative relatives (n=54), BrS-GRS ≥4 alleles yielded an odds ratio of 22.29 ([95% CI, 1.84–269.30]; P=0.0146). Among E1784K-SCN5A positive family members (n=79), hosting ≥4 risk alleles gave an odds ratio=5.12 ([95% CI, 1.93–13.62]; P=0.0011). Conclusions: Common genetic variation is associated with variable expressivity of BrS phenotype in SCN5A families, explaining in part incomplete penetrance and genotype-negative phenotype-positive individuals. SCN5A mutation genotype and a BrS-GRS associate with BrS phenotype, but the strength of association varies according to presence of a SCN5A mutation and severity of loss of function.
Published: 2020
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45. ECG modifications, life-threatening ventricular arrhythmias and relation to mitral valve apparatus phenotype in mitral valve prolapse

Author: Caroline Cueff, V Probst, Jean-Baptiste Gourraud, D. Stevant, Aurélie Thollet, T. Le Tourneau, Nicolas Piriou, and M Plessis
Subjects: medicine.medical_specialty, business.industry, medicine.disease, Sudden death, medicine.anatomical_structure, Internal medicine, Cardiac chamber, T wave, Qt dispersion, Mitral valve, medicine, Cardiology, Mitral valve prolapse, cardiovascular diseases, Systole, PR interval, Cardiology and Cardiovascular Medicine, business
Abstract: Background Mitral valve prolapse (MVP) is a common condition in the general population, which can be associated to non-specific ECG abnormalities described initially as ST segment depression, T waves flattening or inversion, especially in the inferior leads. Lately, this type of ECG abnormalities has been reported in patients with MVP and ventricular arrhythmias (VA) or sudden death (SD). However, the prevalence of ECG abnormalities has never been studied in a large series of patients, and the link between ECG abnormalities, VA and SD to echocardiography examination has never been prospectively assessed. Objective To study the prevalence of ECG abnormalities including ventricular arrhythmias in MVP patients and their link with echocardiographic characteristics. Methods All patients (n=731, MVP = 486, Controls = 245) were prospectively enrolled and underwent a comprehensive echocardiography and ECG. In MVP patients 81 had minimal systolic displacement (MSD), 92 isolated MVP, 108 mild-moderate MR, and 196 severe MR. A comprehensive qualitative and quantitative analysis of ECG obtained from rest ECG, 24-hours ECG recording or exercise stress tests, was carried out. Mean follow-up was 4.4 years. Results The mains ECG abnormalities were an inversion of T wave in the inferior leads found in 12 MVP vs 1 control (2.5 vs 0.4%, P=0.047) or a QRS notch (5.1 vs 2.9%, P=0.13). In bileaflet MVP T wave inversion was more frequent as compared with other MVP patients (3.8 vs 0.8%, P=0.039). In addition there was a progressive prolongation of PR interval, QRS duration and increase QT dispersion associated with worsening of MR and heart chambers remodeling. None of ECG findings were significantly linked with the presence of MVP only. Out of 731 individuals, 27 (3.7%) had an history of VA or SD. In a multivariate analysis, bileaflet prolapse and mitral annulus disjunction were associated with VA or SD, whereas none of ECG criterion was associated with. Conclusion In this large prospective series of MVP patients, prevalence of inferior leads ECG abnormalities is very low. Prolongation of atrio-ventricular and ventricular conduction, as well increased QT dispersion is associated with worsening of MR and LV remodeling. Bileaflet prolapse and mitral annulus disjunction are associated with VA or SD. Funding Acknowledgement Type of funding source: None
Published: 2020
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46. Long-term follow-up of patients with tetralogy of Fallot and implantable cardioverter defibrillator

Author: Waldmann, Magalie Ladouceur, Dai-T F investigators, Francis Bessière, A. Bouzeman, Jean-Benoit Thambo, Pierre Mondoly, Guillaume Duthoit, Fabien Labombarda, R. Koutbi, C. Marquie, Nicolas Combes, F. Sacher, Eloi Marijon, Laurence Iserin, and Jean-Baptiste Gourraud
Subjects: Secondary prevention, medicine.medical_specialty, Ejection fraction, Long term follow up, business.industry, Cyanotic congenital heart disease, medicine.medical_treatment, Implantable cardioverter-defibrillator, medicine.disease, Implantable defibrillators, Sudden cardiac death, Internal medicine, medicine, Cardiology, Cardiology and Cardiovascular Medicine, business, Tetralogy of Fallot
Abstract: Background Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. Purpose We aimed to describe long-term follow-up of patients with TOF and ICD through a large nationwide registry. Methods Nationwide Registry including all TOF patients with an ICD initiated in 2010. The primary outcome was the first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee. Cox proportional hazard models were used to identify predictors of appropriate ICD therapies and ICD-related complications. Results A total of 165 patients (mean age 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (IQR) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least one appropriate ICD therapy, giving an annual incidence of 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively, p=0.03). Overall, 71 (43.0%) patients presented with at least one complication, including inappropriate ICD shocks in 42 (25.5%) patients and lead/generator dysfunction in 36 (21.8%) patients. Among 61 (37.0%) primary prevention patients, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with respectively no, one, two, or ≥ three guideline-recommended risk factors. In our cohort, QRS fragmentation was the only independent predictor of appropriate ICD therapies (HR 4.34, 95% CI 1.42–13.23), and its integration in a model with current criteria increased the area under the curve from 0.61 to 0.72 (p=0.006). No patient with left ventricular ejection fraction (LVEF) ≤35% without at least one other risk factor had appropriate ICD therapy. Patients with congestive heart failure and/or reduced LVEF had a higher risk of non-sudden death or heart transplantation (HR=11.01, 95% CI: 2.96–40.95). Conclusions Our findings demonstrate high rates of appropriate therapies in TOF patients with an ICD, including in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria might improve risk stratification beyond low LVEF. Freedom from appropriate ICD therapy Funding Acknowledgement Type of funding source: None
Published: 2020
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47. Enhancing rare variant interpretation in inherited arrhythmias through quantitative analysis of consortium disease cohorts and population controls

Author: Charles Antzelevitch, Ahmad S. Amin, Bo Gregers Winkel, Dan M. Roden, Stefan Kääb, Jonathan R. Skinner, Seiko Ohno, Julien Barc, Birgit Stallmeyer, Carla Giustetto, Connie R. Bezzina, Francesco Mazzarotto, Tomas Robyns, Carlo Napolitano, Stellan Mörner, Doris Škorić-Milosavljević, Sven Dittmann, Kenichiro Yamagata, Sonia Van Dooren, Anniek Corveleyn, Carlo de Asmundis, Ramon Brugada, K Usuda, Yuji Tanaka, Sven Zumhagen, Tadashi Nakajima, Johan Saenen, Elijah R. Behr, Hector Barajas-Martinez, Stéphane Bézieau, Masao Yoshinaga, Georgia Sarquella-Brugada, Paul G.A. Volders, Juan R. Gimeno, Lia Crotti, Charlotte Glinge, Andrea Mazzanti, Ingrid P.C. Krapels, Nicola Whiffin, Sebastian Clauss, Yoshiaki Kaneko, James S. Ware, Minoru Horie, Keiko Shimamoto, Isabelle Denjoy, Pieter G. Postema, Christian Krijger, Takeshi Aiba, Masahiko Kurabayashi, Pyotr G. Platonov, Regina Sebastiano, Cristina Gil Ortuño, Annika Rydberg, Roddy Walsh, Michael J. Ackerman, Hideki Itoh, M. Benjamin Shoemaker, Can Hasdemir, Pascale Guicheney, J. Martijn Bos, Frederic Sacher, Takeru Makiyama, Julieta Lazarte, Maarten P. van den Berg, Dominique Babuty, David J. Tester, Silvia Castelletti, Jacques Mansourati, Antoine Leenhardt, Paul A. van der Zwaag, Sanjay Sharma, Elena Arbelo, Candan Celen, Pier D. Lambiase, Maria Christina Kotta, Johannes Steinfurt, Jean-Baptiste Gourraud, Pedro Brugada, Wataru Shimizu, Josep Brugada, Jørgen K. Kanters, Eline A. Nannenberg, Silvia G. Priori, Mary N. Sheppard, Richard Redon, Morten S. Olesen, Jeroen Breckpot, Britt M. Beckmann, Naomasa Makita, Martin Borggrefe, Rafik Tadros, Jean-Jacques Schott, Jacob Tfelt-Hansen, Steven A. Lubitz, Hatice Şahin, Najim Lahrouchi, Michael Papadakis, Daisuke Hazeki, Kenshi Hayashi, Oscar Campuzano, Katja E. Odening, Federica Dagradi, Eric Schulze-Bahr, Boris Rudic, Hiroki Kimoto, Vincent Probst, Jason D. Roberts, Raphaël P. Martins, Bart Loeys, Daniela F. Giachino, F. Kyndt, Kimie Ohkubo, Taisuke Ishikawa, Catarina Lundin, Lut Van Laer, Patrick T. Ellinor, Maria Sabater Molina, Peter J. Schwartz, Annika Winbo, Wellcome Trust, Rosetrees Trust, British Heart Foundation, Walsh, R, Lahrouchi, N, Tadros, R, Kyndt, F, Glinge, C, Postema, P, Amin, A, Nannenberg, E, Ware, J, Whiffin, N, Mazzarotto, F, Skoric-Milosavljevic, D, Krijger, C, Arbelo, E, Babuty, D, Barajas-Martinez, H, Beckmann, B, Bezieau, S, Bos, J, Breckpot, J, Campuzano, O, Castelletti, S, Celen, C, Clauss, S, Corveleyn, A, Crotti, L, Dagradi, F, de Asmundis, C, Denjoy, I, Dittmann, S, Ellinor, P, Ortuno, C, Giustetto, C, Gourraud, J, Hazeki, D, Horie, M, Ishikawa, T, Itoh, H, Kaneko, Y, Kanters, J, Kimoto, H, Kotta, M, Krapels, I, Kurabayashi, M, Lazarte, J, Leenhardt, A, Loeys, B, Lundin, C, Makiyama, T, Mansourati, J, Martins, R, Mazzanti, A, Morner, S, Napolitano, C, Ohkubo, K, Papadakis, M, Rudic, B, Molina, M, Sacher, F, Sahin, H, Sarquella-Brugada, G, Sebastiano, R, Sharma, S, Sheppard, M, Shimamoto, K, Shoemaker, M, Stallmeyer, B, Steinfurt, J, Tanaka, Y, Tester, D, Usuda, K, van der Zwaag, P, Van Dooren, S, Van Laer, L, Winbo, A, Winkel, B, Yamagata, K, Zumhagen, S, Volders, P, Lubitz, S, Antzelevitch, C, Platonov, P, Odening, K, Roden, D, Roberts, J, Skinner, J, Tfelt-Hansen, J, van den Berg, M, Olesen, M, Lambiase, P, Borggrefe, M, Hayashi, K, Rydberg, A, Nakajima, T, Yoshinaga, M, Saenen, J, Kaab, S, Brugada, P, Robyns, T, Giachino, D, Ackerman, M, Brugada, R, Brugada, J, Gimeno, J, Hasdemir, C, Guicheney, P, Priori, S, Schulze-Bahr, E, Makita, N, Schwartz, P, Shimizu, W, Aiba, T, Schott, J, Redon, R, Ohno, S, Probst, V, Arnaout, A, Amelot, M, Anselme, F, Billon, O, Defaye, P, Dupuis, J, Jesel, L, Laurent, G, Maury, P, Pasquie, J, Wiart, F, Behr, E, Barc, J, Bezzina, C, unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), CIC - CHU Bichat, Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), CHU Pontchaillou [Rennes], 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], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Ege Üniversitesi, Cardiovascular Centre (CVC), Nantes Referral Ctr Inherited Car, Cardiology, Graduate School, ACS - Heart failure & arrhythmias, Human Genetics, ACS - Amsterdam Cardiovascular Sciences, ACS - Atherosclerosis & ischemic syndromes, MUMC+: DA KG Polikliniek (9), RS: Carim - H02 Cardiomyopathy, Cardiologie, MUMC+: MA Med Staf Spec Cardiologie (9), RS: Carim - H04 Arrhythmogenesis and cardiogenetics, Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Clinical sciences, Heartrhythmmanagement, Medical Genetics, Reproduction and Genetics, and Cardio-vascular diseases
Subjects: 0301 basic medicine, [SDV]Life Sciences [q-bio], Nantes Referral Center for inherited cardiac arrhythmia, Disease, Arrhythmias, 030105 genetics & heredity, ACMG/AMP guidelines, Brugada, LQTS, variant interpretation, Medicine, Genetics (clinical), Brugada Syndrome, Brugada syndrome, Genetics, Genetics & Heredity, education.field_of_study, medicine.diagnostic_test, Molecular pathology, 3. Good health, Long QT Syndrome, Medical genetics, Population Control, Cardiology and Cardiovascular Medicine, Cardiac, Medical Genetics, Life Sciences & Biomedicine, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Long QT syndrome, Population, 610 Medicine & health, BIO/18 - GENETICA, Article, 03 medical and health sciences, Humans, Genetic Testing, cardiovascular diseases, education, Medicinsk genetik, Genetic testing, 0604 Genetics, Science & Technology, business.industry, Genetic heterogeneity, MUTATIONS, ACMG/AMP guideline, Arrhythmias, Cardiac, 1103 Clinical Sciences, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Mutation, 030104 developmental biology, Human medicine, business
Abstract: Purpose: Stringent variant interpretation guidelines can lead to high rates of variants of uncertain significance (VUS) for genetically heterogeneous disease like long QT syndrome (LQTS) and Brugada syndrome (BrS). Quantitative and disease-specific customization of American College of Medical Genetics and Genomics/Association for Molecular Pathology (ACMG/AMP) guidelines can address this false negative rate. Methods: We compared rare variant frequencies from 1847 LQTS (KCNQ1/KCNH2/SCN5A) and 3335 BrS (SCN5A) cases from the International LQTS/BrS Genetics Consortia to population-specific gnomAD data and developed disease-specific criteria for ACMG/AMP evidence classes-rarity (PM2/BS1 rules) and case enrichment of individual (PS4) and domain-specific (PM1) variants. Results: Rare SCN5A variant prevalence differed between European (20.8%) and Japanese (8.9%) BrS patients (p = 5.7 x 10(-18)) and diagnosis with spontaneous (28.7%) versus induced (15.8%) Brugada type 1 electrocardiogram (ECG) (p = 1.3 x 10(-13)). Ion channel transmembrane regions and specific N-terminus (KCNH2) and C-terminus (KCNQ1/KCNH2) domains were characterized by high enrichment of case variants and >95% probability of pathogenicity. Applying the customized rules, 17.4% of European BrS and 74.8% of European LQTS cases had (likely) pathogenic variants, compared with estimated diagnostic yields (case excess over gnomAD) of 19.2%/82.1%, reducing VUS prevalence to close to background rare variant frequency. Conclusion: Large case-control data sets enable quantitative implementation of ACMG/AMP guidelines and increased sensitivity for inherited arrhythmia genetic testing., Amsterdam Cardiovascular Sciences fellowship; Dutch Heart Foundation (CVON Predict-2/Concor-genes); Netherlands Organization for Scientific ResearchNetherlands Organization for Scientific Research (NWO) [VICI 016.150.610]; Fondation LeducqLeducq Foundation, R.W. is supported by an Amsterdam Cardiovascular Sciences fellowship. C.R.B. is supported by the Dutch Heart Foundation (CVON Predict-2/Concor-genes), Netherlands Organization for Scientific Research (VICI 016.150.610), and Fondation Leducq. See Supplement for all Acknowledgements.
Published: 2020
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48. Transethnic Genome-Wide Association Study Provides Insights in the Genetic Architecture and Heritability of Long QT Syndrome

Author: Yvonne M. Hoedemaekers, M. Ben Shoemaker, Pascale Guicheney, Antoine Leenhardt, Andrea Mazzanti, Minoru Horie, Jan H. Veldink, Isabelle Denjoy, Yu Kucho, Chiea Chuen Khor, Tomas Robyns, Carlo Napolitano, Peter Weeke, J. Martijn Bos, David J. Tester, Hanno L. Tan, Annika Rydberg, Patrick T. Ellinor, Pilar Galan, Taisuke Ishikawa, Seiko Ohno, Peter J. Schwartz, Masao Yoshinaga, Thomas Werge, Marta Ribasés, Bart Loeys, Jean-Jacques Schott, Jacob Tfelt-Hansen, Ulla-Britt Diamant, Marko Ernsting, Georgia Sarquella-Brugada, Yuka Mizusawa, Michael Christiansen, Pyotr G. Platonov, Annika Winbo, Thomas Meitinger, Keiko Shimamoto, Cristina Barlassina, Pieter G. Postema, Takeru Makiyama, Maarten P. van den Berg, Yanushi D. Wijeyeratne, Wataru Shimizu, Charles Antzelevitch, Christopher Newton-Cheh, Martina Müller-Nurasyid, Dan M. Roden, Vincent Probst, Takeshi Aiba, Lia Crotti, Daniele Cusi, Britt M. Beckmann, Johan Saenen, Peter Lichtner, Oscar Campuzano, Tin Aung, Nynke Hofman, Morten S. Olesen, Matteo Pedrazzini, Elijah R. Behr, Karen E. Morrison, Najim Lahrouchi, Katja E. Odening, Andrew D. Krahn, Kari L. Turkowski, J. Peter van Tintelen, Steven A. Lubitz, Federica Dagradi, Josep Brugada, Julien Barc, Birgit Stallmeyer, Stefan Kääb, Sven Zumhagen, Jonathan R. Skinner, Michael W.T. Tanck, Christopher Shaw, Brianna Davies, Eric Schulze-Bahr, Mineo Ozaki, Roddy Walsh, Antoine Andorin, Leonard H. van den Berg, Silvia G. Priori, Johannes Steinfurt, Jean-Baptiste Gourraud, Eline A. Nannenberg, Mark Lathrop, Rafik Tadros, Ramon Brugada, Leander Beekman, Peter M. Andersen, Ryan Pfeiffer, Boris Rudic, Reza Jabbari, Kanae Hasegawa, Jeroen Breckpot, Naomasa Makita, Michael J. Ackerman, Arthur A.M. Wilde, Hideki Itoh, Martin Borggrefe, Elena Arbelo, Connie R. Bezzina, Pamela J. Shaw, Ammar Al-Chalabi, Markus Munter, Cardiology, Graduate School, ACS - Heart failure & arrhythmias, ACS - Amsterdam Cardiovascular Sciences, Human Genetics, Epidemiology and Data Science, APH - Methodology, ACS - Atherosclerosis & ischemic syndromes, Heart Center, Department of Clinical and Experimental Cardiology, Amsterdam Cardiovascular Sciences. Amsterdam University Medical Center, University of Amsterdam, European Reference Network for Rare, Low Prevalence, and Complex Diseases of the Heart (ERN GUARD-Heart), Institut de Cardiologie de Montreal, Université de Montréal (UdeM), Istituto Auxologico Italiano, Shiga University of Medical Science, University of Fukui [Bunkyo], Equipe 3: EREN- Equipe de Recherche en Epidémiologie Nutritionnelle (CRESS - U1153), Université Sorbonne Paris Nord-Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Conservatoire National des Arts et Métiers [CNAM] (CNAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Conservatoire National des Arts et Métiers [CNAM] (CNAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), 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], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CIC - CHU Bichat, Institut National de la Santé et de la Recherche Médicale (INSERM), Lahrouchi, N, Tadros, R, Crotti, L, Mizusawa, Y, Postema, P, Beekman, L, Walsh, R, Hasegawa, K, Barc, J, Ernsting, M, Turkowski, K, Mazzanti, A, Beckmann, B, Shimamoto, K, Diamant, U, Wijeyeratne, Y, Kucho, Y, Robyns, T, Ishikawa, T, Arbelo, E, Christiansen, M, Winbo, A, Jabbari, R, Lubitz, S, Steinfurt, J, Rudic, B, Loeys, B, Shoemaker, M, Weeke, P, Pfeiffer, R, Davies, B, Andorin, A, Hofman, N, Dagradi, F, Pedrazzini, M, Tester, D, Bos, J, Sarquella-Brugada, G, Campuzano, Ó, Platonov, P, Stallmeyer, B, Zumhagen, S, Nannenberg, E, Veldink, J, van den Berg, L, Al-Chalabi, A, Shaw, C, Shaw, P, Morrison, K, Andersen, P, Müller-Nurasyid, M, Cusi, D, Barlassina, C, Galan, P, Lathrop, M, Munter, M, Werge, T, Ribasés, M, Aung, T, Khor, C, Ozaki, M, Lichtner, P, Meitinger, T, van Tintelen, J, Hoedemaekers, Y, Denjoy, I, Leenhardt, A, Napolitano, C, Shimizu, W, Schott, J, Gourraud, J, Makiyama, T, Ohno, S, Itoh, H, Krahn, A, Antzelevitch, C, Roden, D, Saenen, J, Borggrefe, M, Odening, K, Ellinor, P, Tfelt-Hansen, J, Skinner, J, van den Berg, M, Olesen, M, Brugada, J, Brugada, R, Makita, N, Breckpot, J, Yoshinaga, M, Behr, E, Rydberg, A, Aiba, T, Kääb, S, Priori, S, Guicheney, P, Tan, H, Newton-Cheh, C, Ackerman, M, Schwartz, P, Schulze-Bahr, E, Probst, V, Horie, M, Wilde, A, Tanck, M, Bezzina, C, and Cardiovascular Centre (CVC)
Subjects: Multifactorial Inheritance, [SDV]Life Sciences [q-bio], Genome-wide association study, 030204 cardiovascular system & hematology, Severity of Illness Index, Sudden cardiac death, Electrocardiography, 0302 clinical medicine, inheritance pattern, Medicine, Cardiac and Cardiovascular Systems, Age of Onset, Genetics, 0303 health sciences, Kardiologi, Genetic disorder, genome-wide association study, Prognosis, 3. Good health, Phenotype, Medical genetics, Cardiology and Cardiovascular Medicine, Adult, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Adolescent, Genotype, Long QT syndrome, 610 Medicine & health, BIO/18 - GENETICA, QT interval, Polymorphism, Single Nucleotide, 03 medical and health sciences, Young Adult, Physiology (medical), long QT syndrome, Humans, Genetic Predisposition to Disease, cardiovascular diseases, Alleles, Genetic Association Studies, MED/01 - STATISTICA MEDICA, 030304 developmental biology, Neurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7], business.industry, inheritance patterns, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, Heritability, medicine.disease, Genetic architecture, Genome-wide Association Study, Inheritance Patterns, Long Qt Syndrome, Case-Control Studies, Human medicine, business
Abstract: Background: Long QT syndrome (LQTS) is a rare genetic disorder and a major preventable cause of sudden cardiac death in the young. A causal rare genetic variant with large effect size is identified in up to 80% of probands (genotype positive) and cascade family screening shows incomplete penetrance of genetic variants. Furthermore, a proportion of cases meeting diagnostic criteria for LQTS remain genetically elusive despite genetic testing of established genes (genotype negative). These observations raise the possibility that common genetic variants with small effect size contribute to the clinical picture of LQTS. This study aimed to characterize and quantify the contribution of common genetic variation to LQTS disease susceptibility. Methods: We conducted genome-wide association studies followed by transethnic meta-analysis in 1656 unrelated patients with LQTS of European or Japanese ancestry and 9890 controls to identify susceptibility single nucleotide polymorphisms. We estimated the common variant heritability of LQTS and tested the genetic correlation between LQTS susceptibility and other cardiac traits. Furthermore, we tested the aggregate effect of the 68 single nucleotide polymorphisms previously associated with the QT-interval in the general population using a polygenic risk score. Results: Genome-wide association analysis identified 3 loci associated with LQTS at genome-wide statistical significance ( P −8 ) near NOS1AP , KCNQ1 , and KLF12 , and 1 missense variant in KCNE1 (p.Asp85Asn) at the suggestive threshold ( P −6 ). Heritability analyses showed that ≈15% of variance in overall LQTS susceptibility was attributable to common genetic variation ( h2SNP 0.148; standard error 0.019). LQTS susceptibility showed a strong genome-wide genetic correlation with the QT-interval in the general population (r g =0.40; P =3.2×10 −3 ). The polygenic risk score comprising common variants previously associated with the QT-interval in the general population was greater in LQTS cases compared with controls ( P P Conclusions: This work establishes an important role for common genetic variation in susceptibility to LQTS. We demonstrate overlap between genetic control of the QT-interval in the general population and genetic factors contributing to LQTS susceptibility. Using polygenic risk score analyses aggregating common genetic variants that modulate the QT-interval in the general population, we provide evidence for a polygenic architecture in genotype negative LQTS.
Published: 2020
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49. Implantable cardiac defibrillator leads dysfunction after LVAD implantation

Author: Gerard Babatasi, Nicolas D'Ostrevy, Pierre-Yves Litzler, Christophe Leclercq, Raphaël P. Martins, Matteo Pozzi, Philippe Gaudard, Edeline Pelcé, Vincent Galand, Eloi Marijon, Nicolas Lellouche, Thierry Bourguignon, Jean-Luc Pasquié, Marie Bielefeld, Aude Boignard, Bertrand Pierre, Marylou Para, André Vincentelli, Fabien Garnier, Frédéric Anselme, Stéphane Boulé, Philippe Maury, David Hamon, Pascal Defaye, Nicolas Welte, Hamed Bourenane, Emilie Varlet, Hugues Blangy, Vincent Probst, Michel Kindo, François Picard, Erwan Flecher, Thomas Cardi, Nicolas Sadoul, Katrien Blanchart, Jean-François Obadia, Vincent Algalarrondo, Jean-Baptiste Gourraud, Pierre Mondoly, Vlad Gariboldi, Romain Eschalier, Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de cardiologie [Toulouse], Hôpital de Rangueil, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Université de Bordeaux (UB), CHU Strasbourg, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire [Grenoble] (CHU), CIC - Grenoble, Université Joseph Fourier - Grenoble 1 (UJF)-Institut National de la Santé et de la Recherche Médicale (INSERM), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital de la Timone [CHU - APHM] (TIMONE), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Service de cardiologie [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Hôpital Henri Mondor, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), CHU Clermont-Ferrand, 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), Service de Cardiologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), NA, Fédération Française de Cardiologie, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service Cardiologie [CHU Toulouse], Pôle Cardiovasculaire et Métabolique [CHU Toulouse], and Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)
Subjects: Male, medicine.medical_specialty, implantable cardioverter defibrillator, medicine.medical_treatment, Lead impedance, [SDV]Life Sciences [q-bio], Icd lead, lead parameters dysfunction, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, left ventricular assist device, Humans, 030212 general & internal medicine, Lead (electronics), ComputingMilieux_MISCELLANEOUS, Aged, Pacing impedance, business.industry, General Medicine, Middle Aged, equipment and supplies, Implantable cardioverter-defibrillator, Defibrillators, Implantable, Electrodes, Implanted, Prosthesis Failure, Ventricular assist device, Cardiology, France, Heart-Assist Devices, Cardiology and Cardiovascular Medicine, business
Abstract: Background Implantable cardioverter-defibrillator (ICD) lead dysfunction has been reported after left ventricular assist device (LVAD) implantation in limited single-center studies. We aimed at describing and characterizing the incidence of ICD lead parameters dysfunction after LVAD implantation. Methods Among the 652 patients enrolled in the ASSIST-ICD study, only patients with an ICD prior to LVAD were included (n = 401). ICD lead parameters dysfunction following LVAD implantation is defined as follows: (a) >50% decrease in sensing threshold, (b) pacing lead impedance increase/decrease by >100Ω, and (c) >50% increase in pacing threshold. Results One hundred twenty-two patients with an ICD prior to LVAD had available ICD interrogation reports prior and after LVAD. A total of 67 (55%) patients exhibited at least one significant lead dysfunction: 17 (15%) exhibited >50% decrease in right ventricular (RV) sensing, 51 (42%) had >100 Ω increase/decrease in RV pacing impedance, and 24 (20%) experienced >50% increase in RV pacing threshold. A total of 52 patients experienced ventricular arrhythmia during follow-up and all were successfully detected and treated by the device. All lead dysfunction could be managed conservatively. Conclusion More than 50% of LVAD-recipients may experience >1 significant change in lead parameters but none had severe clinical consequences.
Published: 2020
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50. From electrophysiological exploration to management of heart arrhythmias: Economic analysis of practices in a high-volume French hospital over two different time periods

Author: Jean-Baptiste Gourraud, Johann Clouet, F. Barge, F. Nativel, N. Prevost, Gilles Lande, Gael Grimandi, Vincent Probst, and Aude Solnon
Subjects: medicine.medical_specialty, Pharmaceutical Science, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Ambulatory care, Health care, medicine, Clinical endpoint, Economic analysis, Humans, 030212 general & internal medicine, health care economics and organizations, Retrospective Studies, Pharmacology, biology, business.industry, Euros, Arrhythmias, Cardiac, biology.organism_classification, Hospitals, Heart Rhythm, Hospitalization, Emergency medicine, Costs and Cost Analysis, Structure based, business
Abstract: Summary Background Medical devices (MD) used to treat arrhythmias range from electrophysiological exploration catheters to intracardiac ablation catheters, and they are continuously undergoing optimization. The inclusion of innovative MD in Diagnosis Related Groups (DRG) of the French healthcare economic system can lead to financial imbalance for health institutions. The objective of this study was to compare cost-revenue analyses for interventional heart rhythm management in a high-volume French hospital between two time periods. Methods For 3 months in 2014 and 3 months in 2017, all of the patients admitted to the interventional rhythmic unit with arrhythmia were included retrospectively in this monocenter study. All arrhythmias were considered. The primary clinical endpoint was the difference between the expenses and incomes, calculated for each patient. The secondary endpoint was the breakdown of costs. Results 217 patients were included. In 2014 period, the analysis revealed a deficit of 409 ± 1717 euros per patient and an overall deficit for the hospital of 44,635 euros. In 2017 period, the same evaluation indicated a deficit of 446 ± 1316 euros per patient and an overall deficit for the hospital of 48,210 euros. The cost of MD accounts for a significant share of total expenses. Conclusion The profitability for the cardiac rhythm activity at our facility was optimized between 2014 and 2017. The reliance on ambulatory care increased. However, the reduction in the expenses incurred did not increase the profitability for the facility. It was offset by a decrease in DRG tariffs. A flowchart-type structure based on these practices analyses for rhythmic disorder treatments was developed.
Published: 2020

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