Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy A Multicenter Cohort Study

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dc.contributor.authorChan, Raymond H.
dc.contributor.authorvan der Wal, Laurine
dc.contributor.authorLiberato, Gabriela
dc.contributor.authorRowin, Ethan
dc.contributor.authorSoslow, Jonathan
dc.contributor.authorMaskatia, Shiraz
dc.contributor.authorChan, Sherwin
dc.contributor.authorShah, Amee
dc.contributor.authorFogel, Mark
dc.contributor.authorHernandez, Lazaro
dc.contributor.authorAnwar, Shafkat
dc.contributor.authorVoges, Inga
dc.contributor.authorCarlsson, Marcus
dc.contributor.authorBuddhe, Sujatha
dc.contributor.authorLaser, Kai Thorsten
dc.contributor.authorGreil, Gerald
dc.contributor.authorValsangiacomo-Buechel, Emanuela
dc.contributor.authorOlivotto, Iacopo
dc.contributor.authorWong, Derek
dc.contributor.authorWolf, Cordula
dc.contributor.authorGrotenhuis, Heynric
dc.contributor.authorRickers, Carsten
dc.contributor.authorHor, Kan
dc.contributor.authorRutz, Tobias
dc.contributor.authorKutty, Shelby
dc.contributor.authorSamyn, Margaret
dc.contributor.authorJohnson, Tiffanie
dc.contributor.authorHasbani, Keren
dc.contributor.authorMoore, Jeremy P.
dc.contributor.authorSieverding, Ludger
dc.contributor.authorDetterich, Jon
dc.contributor.authorParra, Rodrigo
dc.contributor.authorChungsomprasong, Paweena
dc.contributor.authorToro-Salazar, Olga
dc.contributor.authorRoest, Arno A. W.
dc.contributor.authorDittrich, Sven
dc.contributor.authorBrun, Henrik
dc.contributor.authorSpinner, Joseph
dc.contributor.authorLai, Wyman
dc.contributor.authorDyer, Adrian
dc.contributor.authorJablonowsk, Robert
dc.contributor.authorMeierhofer, Christian
dc.contributor.authorGabbert, Dominik
dc.contributor.authorPrsa, Milan
dc.contributor.authorPatel, Jyoti Kandlikar
dc.contributor.authorHornung, Andreas
dc.contributor.authorDiab, Simone Goa
dc.contributor.authorHouse, Aswathy Vaikom
dc.contributor.authorRakowski, Harry
dc.contributor.authorBenson, Lee
dc.contributor.authorMaron, Martin S.
dc.contributor.authorGrosse-Wortmann, Lars
dc.date.accessioned2025-01-20T16:07:53Z
dc.date.available2025-01-20T16:07:53Z
dc.date.issued2024
dc.description.abstractImportance 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] follow-up period of 1.9 (0.5-4.1) [0.1-14.8] years, 35 patients (5.0%) experienced SCD or equivalent events. LGE was present in 230 patients (32.9%), which constituted an mean (SD) burden of 5.9% (7.3%) of left ventricular myocardium. The LGE amount was higher in older patients and those with greater left ventricular mass and maximal wall thickness; patients with LGE had lower left ventricular ejection fractions and larger left atrial diameters. The presence and burden of LGE was associated with SCD, even after correcting for existing risk stratification tools. Patients with 10% or more LGE, relative to total myocardium, had a higher risk of SCD (unadjusted hazard ratio [HR], 2.19; 95% CI, 1.59-3.02; P < .001). Furthermore, the addition of LGE burden improved the performance of the HCM Risk-Kids score (before LGE addition: 0.66; 95% CI, 0.58-0.75; after LGE addition: 0.73; 95% CI, 0.66-0.81) and Precision Medicine in Cardiomyopathy score (before LGE addition: 0.68; 95% CI, 0.49-0.77; after LGE addition: 0.73; 95% CI, 0.64-0.82) SCD predictive models. Conclusions and Relevance In this retrospective cohort study, quantitative LGE was a risk factor for SCD in patients younger than 21 years with HCM and improved risk stratification.
dc.description.funderChildren's Cardiomyopathy Foundation
dc.fuente.origenWOS
dc.identifier.doi10.1001/jamacardio.2024.2824
dc.identifier.eissn2380-6591
dc.identifier.issn2380-6583
dc.identifier.urihttps://doi.org/10.1001/jamacardio.2024.2824
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/90031
dc.identifier.wosidWOS:001321492200003
dc.language.isoen
dc.revistaJama cardiology
dc.rightsacceso restringido
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleMyocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy A Multicenter Cohort Study
dc.typeartículo
sipa.indexWOS
sipa.trazabilidadWOS;2025-01-12
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