Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy

作者：Raymond H. Chan, Laurine van der Wal, Gabriela Liberato, Ethan J. Rowin, Jonathan H. Soslow, Shiraz A. Maskatia, Sherwin S. Chan, Amee Shah, Mark A. Fogel, Lázaro Hernández, Shafkat Anwar, Inga Voges, Marcus Carlsson, Sujatha Buddhe, Kai Thorsten Laser, Gerald Greil, Emanuela Valsangiacomo-Buechel, Iacopo Olivotto, Derek Wong, Cordula M. Wolf, Heynric B. Grotenhuis, Carsten Rickers, Kan N. Hor, Tobias Rutz, Shelby Kutty, Margaret M. Samyn, Tiffanie R. Johnson, Keren Hasbani, Jeremy P. Moore, Ludger Sieverding, Jon Detterich, Rodrigo Parra, Paweena Chungsomprasong, Olga Toro-Salazar, Arno A.W. Roest, Sven Dittrich, Henrik Brun, Joseph A. Spinner, Wyman W. Lai, Adrian Dyer, Robert Jablonowski, Christian Meierhofer, Dominik Daniel Gabbert, Milan Prša, Jyoti Patel, Andreas Hornung, Simone Goa Diab, Aswathy Vaikom House, Harry Rakowski, Lee Benson, Martin S. Maron, Lars Grosse‐Wortmann · 发表于：JAMA Cardiology · 年份：2024 · DOI：10.1001/jamacardio.2024.2824 · 被引用次数：23 · 研究领域：Cardiomyopathy and Myosin Studies、Cardiac Imaging and Diagnostics、Cardiovascular Function and Risk Factors

Importance: The ability to predict sudden cardiac death (SCD) in children and adolescents with hypertrophic cardiomyopathy (HCM) is currently inadequate. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) imaging is associated with SCD events in adults with HCM. Objective: To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years. Design, Setting, and Participants: This multicenter, retrospective cohort study was conducted from April 8, 2015, to September 12, 2022, in patients with HCM who were younger than 21 years and had undergone CMR imaging across multiple sites in the US, Europe, and South America. Observers of CMR studies were masked toward outcomes and demographic characteristics. Exposure: Natural history of HCM. Main Outcome and Measures: The primary outcome was SCD and surrogate events, including resuscitated cardiac arrest and appropriate discharges from an implantable defibrillator. Continuous and categorical data are expressed as mean (SD), median (IQR), or number (percentage), respectively. Survivor curves comparing patients with and without LGE were constructed by the Kaplan-Meier method, and likelihood of subsequent clinical events was further evaluated using univariate and multivariable Cox proportional hazards models. Results: Among 700 patients from 37 international centers, median (IQR) age was 14.8 (11.9-17.4) years, and 518 participants (74.0%) were male. During a median (IQR) [range] foll...