作者
Alessio Gasperetti, Richard Carrick, Alexandros Protonotarios, Brittney A Murray, MIKAEL LAREDO, Iris van der Schaaf, Ronald Lekanne, Petros Syrris, Douglas Cannie, Crystal Tichnell, Chiara Cappelletto, Marta Gigli, Kristen Medo, Ardan Saguner, Firat Duru, Nisha Gilotra, Stefan Zimmerman, Robyn Hylind, Dominic J Abrams, Neal K Lakdawala, JULIA CADRIN-TOURIGNY, Mattia Targetti, Iacopo Olivotto, Maddalena Graziosi, Moniek Cox, Elena Biagini, Philippe Charron, Michela Casella, Claudio Tondo, Momina Yazdani, James S Ware, Sanjay Prasad, Leonardo Caló, Eric D Smith, Adam Helms, Sophie Hespe, Jodie Ingles, Harikrishna Tandri, Flavie Ader, Giovanni Peretto, Stacey Peters, Ari Horton, Jess Yao, Svenn Dittmann, Eric Schulze-Bahr, Maria Qureshi, Katelyn Young, Eric Carruth, Christopher Haggerty, Victoria Parikh, Matthew R Taylor, Luisa Mestroni, Arthur A Wilde, Gianfranco Sinagra, Marco Merlo, Peter van Tintelen, Anneline te Riele, Perry Elliott, Hugh Calkins, Cynthia A James
发表日期
2024/5/1
期刊
Heart Rhythm
卷号
21
期号
5
页码范围
S20
出版商
Elsevier
简介
Background
Pathogenic variants in the desmoplakin (DSP) gene are associated with a distinct arrhythmogenic cardiomyopathy phenotype not fully captured by either dilated (DCM) or arrhythmogenic right ventricular (ARVC) cardiomyopathy. Prior studies have described baseline DSP-cardiomyopathy genetic, inflammatory, and structural characteristics. However, cohort sizes have limited full clinical characterization and identification of clinical and demographic predictors of sustained ventricular arrhythmias (VA), heart failure (HF) hospitalizations, and transplant/death.
Objective
To fully charactherize and report outcomes from a large, worldwide and well-phenotyped cohort of patients (pts) with DSP cardiomyopathy
Methods
All pts with pathogenic/likely pathogenic (P/LP) DSP variants in the world-wide DSP-ERADOS Network (26 academic institutions across 9 countries) were included. The primary outcomes were the …
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A Gasperetti, R Carrick, A Protonotarios, BA Murray… - Heart Rhythm, 2024