Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies

Karim Wahbi; Rabah Ben Yaou; Estelle Gandjbakhch; Frédéric Anselme; Thomas Gossios; Neal Lakdawala; Caroline Stalens; Frederic Sacher; Dominique Babuty; Jean-Noël Trochu; Ghassan Moubarak; Kostantinos Savvatis; Raphaël Porcher; Pascal Laforet; Abdallah Fayssoil; Eloi Marijon; Tanya Stojkovic; Anthony Behin; Sarah Léonard-Louis; Guilhem Sole; Fabien Labombarda; Pascal Richard; Corinne Metay; Susana Quijano-Roy; Ivana Dabaj; Didier Klug; Marie-Christine Vantyghem; Philippe Chevalier; Pierre Ambrosi; Emmanuelle Salort-Campana; Nicolas Sadoul; Xavier Waintraub; Khadija Chikhaoui; Philippe Mabo; Nicolas Combes; Philippe Maury; Jean-Marc Sellal; Usha Tedrow; Jonathan Kalman; Jitendra Vohra; Alexander F.A. Androulakis; Katja Zeppenfeld; Tina Thompson; Christine Barnerias; Henri-Marc Becane; Eric Bieth; Franck Boccara; Damien Bonnet; Françoise Bouhour; Stéphane Boulé; Anne-Claire Bréhin; Françoise Chapon; Pascal Cintas; Jean-Marie Cuisset; Jean-Marc Davy; Annachiara de Sandre-Giovannoli; Florence Demurger; Isabelle Desguerre; Klaus Dieterich; Julien Durigneux; Andoni Echaniz-Laguna; Romain Eschalier; Ana Ferreiro; Xavier Ferrer; Christine Francannet; Mélanie Fradin; Benedicte Gaborit; Arnaud Gay; Albert A. Hagège; Arnaud Isapof; Isabelle Jéru; Raul Juntas Morales; Emmanuelle Lagrue; Nicolas Lamblin; Olivier Lascols; Vincent Laugel; Arnaud Lazarus; France Leturcq; Nicolas Lévy; Véronique Manel; Raphaël Martins; Michèle Mayer; Sandra Mercier; Christophe Meune; Maud Michaud; Marie-Christine Minot-Myhié; Antoine Muchir; Aleksandra Nadaj-Pakleza; Yann Péréon; Philippe Petiot; Florence Petit; Julien Praline; Anne Rollin; Pascal Sabouraud; Catherine Sarret; Stéphane Schaeffer; Frederic Taithe; Celine Tard; Vincent Tiffreau; Annick Toutain; Camille Vatier; Ulrike Walther-Louvier; Bruno Eymard; Philippe Charron; Corinne Vigouroux; Gisèle Bonne; Saurabh Kumar; Perry Elliott; Denis Duboc

doi:10.1161/CIRCULATIONAHA.118.039410

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Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies

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Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies

Karim Wahbi, Rabah Ben Yaou, Estelle Gandjbakhch, Frédéric Anselme, Thomas Gossios, Neal Lakdawala, Caroline Stalens, Frederic Sacher, Dominique Babuty, Jean-Noël Trochu, …

Circulation, Vol.140(4), pp.293-302

23/07/2019

DOI: https://doi.org/10.1161/CIRCULATIONAHA.118.039410

PMID: 31155932

Abstract

death

ventricular

tachycardia

sudden

defibrillators

implantable

death

Background - An accurate estimation of the risk of life-threatening (LT) ventricular tachyarrhythmia (VTA) in patients with LMNA mutations is crucial to select candidates for implantable cardioverter-defibrillator implantation. Methods - We included 839 adult patients with LMNA mutations, including 660 from a French nationwide registry in the development sample, and 179 from other countries, referred to 5 tertiary centers for cardiomyopathies, in the validation sample. LTVTA was defined as (1) sudden cardiac death or (2) implantable cardioverter defibrillator-treated or hemodynamically unstable VTA. The prognostic model was derived using the Fine-Gray regression model. The net reclassification was compared with current clinical practice guidelines. The results are presented as means (SD) or medians [interquartile range]. Results - We included 444 patients, 40.6 (14.1) years of age, in the derivation sample and 145 patients, 38.2 (15.0) years, in the validation sample, of whom 86 (19.3%) and 34 (23.4%) experienced LTVTA over 3.6 [1.0-7.2] and 5.1 [2.0-9.3] years of follow-up, respectively. Predictors of LTVTA in the derivation sample were: male sex, nonmissense LMNA mutation, first degree and higher atrioventricular block, nonsustained ventricular tachycardia, and left ventricular ejection fraction (https://lmna-risk-vta.fr). In the derivation sample, C-index (95% CI) of the model was 0.776 (0.711-0.842), and the calibration slope 0.827. In the external validation sample, the C-index was 0.800 (0.642-0.959), and the calibration slope was 1.082 (95% CI, 0.643-1.522). A 5-year estimated risk threshold ≥7% predicted 96.2% of LTVTA and net reclassified 28.8% of patients with LTVTA in comparison with the guidelines-based approach. Conclusions - In comparison with the current standard of care, this risk prediction model for LTVTA in laminopathies significantly facilitated the choice of candidates for implantable cardioverter defibrillators. Clinical trial registration - URL: https://www.clinicaltrials.gov. Unique identifier: NCT03058185.

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Title: Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies
Creators - without role: Karim Wahbi - Hôpital Cochin
Rabah Ben Yaou - Sorbonne Université
Estelle Gandjbakhch - Sorbonne Université
Frédéric Anselme - Université de Rouen Normandie
Thomas Gossios - St Bartholomew's Hospital
Neal Lakdawala - Brigham and Women's Hospital
Caroline Stalens - Paris Cardiovascular Research Center
Frederic Sacher - Electrophysiology and Heart Modeling Institute
Dominique Babuty - Université de Tours
Jean-Noël Trochu - Institut du Thorax
Ghassan Moubarak - Clinique Ambroise Paré
Kostantinos Savvatis - St Bartholomew's Hospital
Raphaël Porcher - Sorbonne Paris Cité
Pascal Laforet - Hôpital Raymond-Poincaré
Abdallah Fayssoil - Hôpital Raymond-Poincaré
Eloi Marijon - Hôpital Européen
Tanya Stojkovic - Institut de Myologie
Anthony Behin - Institut de Myologie
Sarah Léonard-Louis - Institut de Myologie
Guilhem Sole - Hôpital Pellegrin
Fabien Labombarda - Université de Caen Normandie
Pascal Richard - Laboratoire d'informatique scientifique et industrielle [EA 1232]
Corinne Metay - Sorbonne Université
Susana Quijano-Roy - Hôpital Raymond-Poincaré
Ivana Dabaj - Inserm
Didier Klug - Lille’s Cardiology Hospital
Marie-Christine Vantyghem - European Genomic Institute for Diabetes
Philippe Chevalier - Fondation pour l’innovation en Cadiométabolisme et Nutrition
Pierre Ambrosi - Aix-Marseille Université
Emmanuelle Salort-Campana - Centre de Génétique Médicale de Marseille
Nicolas Sadoul - Centre Hospitalier Régional Universitaire de Nancy
Xavier Waintraub - Institut de cardiologie [CHU Pitié-Salpêtrière]
Khadija Chikhaoui - Institut de Myologie
Philippe Mabo - Centre Hospitalier Universitaire de Rennes
Nicolas Combes - Clinique Pasteur
Philippe Maury - Inserm
Jean-Marc Sellal - Imagerie Adaptative Diagnostique et Interventionnelle
Usha Tedrow - Brigham and Women's Hospital
Jonathan Kalman - The Royal Melbourne Hospital
Jitendra Vohra - University of Melbourne
Alexander F.A. Androulakis - Leiden University Medical Center
Katja Zeppenfeld - Leiden University Medical Center
Tina Thompson - The Royal Melbourne Hospital
Christine Barnerias - Hôpital Necker-Enfants Malades
Henri-Marc Becane - Institut de Myologie
Eric Bieth - Hôpital Purpan
Franck Boccara - Hôpital Saint-Antoine
Damien Bonnet - Hôpital Necker-Enfants Malades
Françoise Bouhour - Hôpital Saint-Antoine
Stéphane Boulé - Hôpital Privé Le Bois Ramsay Santé [Lille]
Anne-Claire Bréhin - Service de génétique [Rouen]
Françoise Chapon - Centre Hospitalier Universitaire de Caen
Pascal Cintas - Centre Hospitalier Universitaire de Toulouse
Jean-Marie Cuisset - Hôpital Roger Salengro
Jean-Marc Davy - Centre Hospitalier Régional Universitaire [Montpellier]
Annachiara de Sandre-Giovannoli - Inserm
Florence Demurger - Centre Hospitalier Universitaire de Rennes
Isabelle Desguerre - Hôpital Necker-Enfants Malades
Klaus Dieterich - Hôpital Couple Enfant
Julien Durigneux - Centre Hospitalier Universitaire d'Angers
Andoni Echaniz-Laguna - Hôpitaux Universitaires de Strasbourg
Romain Eschalier - Université Clermont Auvergne
Ana Ferreiro - Université Paris Cité
Xavier Ferrer - Hôpital Pellegrin
Christine Francannet - Service Génétique Médicale [CHU Clermont-Ferrand]
Mélanie Fradin - Hôpital Couple Enfant
Benedicte Gaborit - Hôpital de la Conception
Arnaud Gay - Centre Hospitalier Universitaire de Rouen
Albert A. Hagège - Délégation Paris 5
Arnaud Isapof - Sorbonne Université
Isabelle Jéru - Pitié-Salpêtrière Hospital
Raul Juntas Morales - Département de neurologie [Montpellier]
Emmanuelle Lagrue - Inserm
Nicolas Lamblin - Centre Hospitalier Universitaire de Lille
Olivier Lascols - Pitié-Salpêtrière Hospital
Vincent Laugel - Hôpitaux Universitaires de Strasbourg
Arnaud Lazarus - Clinique Ambroise Paré
France Leturcq - Hôpital Cochin
Nicolas Lévy - Inserm
Véronique Manel - Hospices Civils de Lyon
Raphaël Martins - Centre Hospitalier Universitaire de Rennes
Michèle Mayer - Sorbonne Université
Sandra Mercier - Génétique Médicale & Génomique Fonctionelle
Christophe Meune - Sorbonne Université
Maud Michaud - Université de Lorraine
Marie-Christine Minot-Myhié - Centre Hospitalier Universitaire de Rennes
Antoine Muchir - Inserm
Aleksandra Nadaj-Pakleza - Service de neurologie [Angers]
Yann Péréon - Centre Hospitalier Universitaire de Nantes
Philippe Petiot - Hospices Civils de Lyon
Florence Petit - Centre de Recherche Jean Pierre Aubert
Julien Praline - Imaging, Brain & Neuropsychiatry
Anne Rollin - Centre Hospitalier Universitaire de Toulouse
Pascal Sabouraud - Hôpital Robert-Debré
Catherine Sarret - Institut Pascal
Stéphane Schaeffer - Université de Caen Normandie
Frederic Taithe - Centre Hospitalier Universitaire de Clermont-Ferrand
Celine Tard - Institut Cochin
Vincent Tiffreau - Laboratoire Motricité Humaine Éducation Sport Santé
Annick Toutain - Service de génétique [Tours]
Camille Vatier - Pitié-Salpêtrière Hospital
Ulrike Walther-Louvier - Centre Hospitalier Universitaire de Montpellier
Bruno Eymard - Institut de Myologie
Philippe Charron - Université de Montréal
Corinne Vigouroux - Pitié-Salpêtrière Hospital
Gisèle Bonne - Institut de Myologie
Saurabh Kumar - Laboratoire de génie des procédés pour la bioraffinerie, les matériaux bio-sourcés et l’impression fonctionnelle
Perry Elliott - University College Hospital at Westmoreland Street
Denis Duboc - Université Paris Cité
Publication Details: Circulation, Vol.140(4), pp.293-302
Grant note: This study was funded by grants from the AFM-Téléthon (French Alliance against Myopathies), which was not involved in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Some of this work was undertaken at University College London (United Kingdom) and St. Bartholomew’s Hospital (London, United Kingdom), which received a portion of funding from the United Kingdom Department of Health’s National Institute for Health Research Biomedical Research Centres funding scheme.
Identifiers: 9937891609311
Academic Unit: Institut des Neurosciences de Montpellier - INM
Language: English
Resource Type: Journal article
Local Fields: hal-02237297