Journal article

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

Karim Wahbi, Rabah Ben Yaou, Estelle Gandjbakhch, Frederic Anselme, Thomas Gossios, Neal K Lakdawala, Caroline Stalens, Frederic Sacher, Dominique Babuty, Jean-Noel Trochu, Ghassan Moubarak, Kostantinos Savvatis, Raphael Porcher, Pascal Laforet, Abdallah Fayssoil, Eloi Marijon, Tanya Stojkovic, Anthony Behin, Sarah Leonard-Louis, Guilhem Sole Show all

Circulation | LIPPINCOTT WILLIAMS & WILKINS | Published : 2019


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..

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Funding Acknowledgements

This study was funded by grants from the AFM-Telethon (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.