Arrhythmic Risk Stratification of Carriers of Filamin C Truncating Variants

Marta Gigli, Davide Stolfo, Giulia Barbati, Sharon Graw, Suet Nee Chen, Marco Merlo, Kristen Medo, Caterina Gregorio, Matteo Dal Ferro, Alessia Paldino, Maria Perotto, J. Peter van Tintelen, Anneline S.J.M. te Riele, Annette F. Baas, Arthur M. Wilde, Ahmad S. Amin, Arjan C. Houweling, Perry Elliott, Douglas Cannie, Michelle Michels, Stephan A C Schoonvelde, Sanjay Prasad, Paz Upasana Tayal, Momina Yazdani, Déborah Morris-Rosendahl, Pablo García‐Pavía, Eva Cabrera-Romero, Barbara Bauce, Kalliopi Pilichou, Diane Fatkin, Renée Johnson, Daniel P. Judge, Kimberly Foil, Stéphane Heymans, Job A.J. Verdonschot, Sophie Stroeks, Neal K. Lakdawala, Purohit Anisha, Matthew J. O’Neill, M. Benjamin Shoemaker, Dan M. Roden, Hugh Calkins, Cynthia A. James, Brittney Murray, Victoria N. Parikh, Euan A. Ashley, Chloe M. Reuter, Massimo Imazio, Marco Canepa, Pietro Ameri, Jiangping Song, Gianfranco Sinagra, Matthew R. Taylor, Luisa Mestroni

Results suggest that the risk of SCD/MVA in phenotype-positive carriers of FLNCtv was high. A 5-variable predictive model derived from this cohort allows risk estimation and could support clinicians in the shared decision for prophylactic ICD implantation. External cohort validation is warranted.