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Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy A Multicenter Cohort Study
Indexado
WoS WOS:001321492200003
DOI 10.1001/JAMACARDIO.2024.2824
Año 2024
Tipo artículo de investigación

Citas Totales

Autores Afiliación Chile

Instituciones Chile

% Participación
Internacional

Autores
Afiliación Extranjera

Instituciones
Extranjeras


Abstract



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] 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.

Revista



Revista ISSN
Jama Cardiology 2380-6583

Métricas Externas



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Disciplinas de Investigación



WOS
Cardiac & Cardiovascular Systems
Cardiac & Cardiovascular System
Scopus
Cardiology And Cardiovascular Medicine
SciELO
Sin Disciplinas

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Publicaciones WoS (Ediciones: ISSHP, ISTP, AHCI, SSCI, SCI), Scopus, SciELO Chile.

Colaboración Institucional



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Autores - Afiliación



Ord. Autor Género Institución - País
1 Chan, Raymond H. - Univ Hlth Network - Canadá
2 van der Wal, Laurine - Univ Groningen - Países Bajos
3 Liberato, Gabriela - UNIV SAO PAULO - Brasil
4 Rowin, Ethan - Lahey Hosp & Med Ctr - Estados Unidos
5 Soslow, Jonathan - Vanderbilt Univ - Estados Unidos
6 Maskatia, Shiraz - Stanford Univ - Estados Unidos
Texas Childrens Hosp - Estados Unidos
7 Chan, Sherwin - Childrens Mercy Hosp - Estados Unidos
8 Shah, Amee - Columbia Univ - Estados Unidos
9 Fogel, Mark - UNIV PENN - Estados Unidos
10 Hernandez, Lazaro - Joe DiMaggio Childrens Hosp - Estados Unidos
11 Anwar, Shafkat - UNIV CALIF SAN FRANCISCO - Estados Unidos
12 Voges, Inga - Univ Hosp Schleswig Holstein - Alemania
Univ Childrens Hosp - Alemania
13 Carlsson, Marcus - Lund Univ - Suecia
14 Buddhe, Sujatha - UNIV WASHINGTON - Estados Unidos
15 Laser, Kai Thorsten - Ruhr Univ Bochum - Alemania
16 Greil, Gerald - Univ Texas Southwestern Med Ctr - Estados Unidos
17 Valsangiacomo-Buechel, Emanuela - Univ Childrens Hosp - Suiza
18 Olivotto, Iacopo - Careggi Univ Hosp - Italia
Meyer Childrens Hosp IRCCS - Italia
19 Wong, Derek - Childrens Hosp Eastern Ontario - Canadá
20 Wolf, Cordula - TECH UNIV MUNICH - Alemania
21 Grotenhuis, Heynric - Univ Med Ctr Utrecht - Países Bajos
22 Rickers, Carsten - Univ Heart Ctr - Alemania
23 Hor, Kan - Nationwide Childrens Hosp - Estados Unidos
24 Rutz, Tobias - Lausanne Univ Hosp - Suiza
25 Kutty, Shelby - Univ Nebraska Med Ctr - Estados Unidos
JOHNS HOPKINS UNIV HOSP - Estados Unidos
26 Samyn, Margaret - MED COLL WISCONSIN - Estados Unidos
27 Johnson, Tiffanie - Indiana Univ Sch Med - Estados Unidos
28 Hasbani, Keren - UT Hlth Austin - Estados Unidos
29 Moore, Jeremy P. - UNIV CALIF LOS ANGELES - Estados Unidos
30 Sieverding, Ludger - UNIV TUBINGEN - Alemania
31 Detterich, Jon - Univ Southern Calif - Estados Unidos
Childrens Hosp Los Angeles - Estados Unidos
32 Parra, Rodrigo - Pontificia Universidad Católica de Chile - Chile
33 Chungsomprasong, Paweena - Mahidol Univ - Tailandia
34 Toro-Salazar, Olga - Connecticut Childrens Med Ctr - Estados Unidos
35 Roest, Arno A.W. Hombre Leiden Univ - Países Bajos
36 Dittrich, Sven - Friedrich Alexander Univ Erlangen Nurnberg - Alemania
37 Brun, Henrik - Oslo Univ Hosp - Noruega
38 Spinner, Joseph - Texas Childrens Hosp - Estados Unidos
39 Lai, Wyman - Childrens Hosp Orange Cty - Estados Unidos
40 Dyer, Adrian - Univ Texas Southwestern Med Ctr - Estados Unidos
Cook Childrens Med Ctr - Estados Unidos
41 Jablonowsk, Robert - Lund Univ - Suecia
42 Meierhofer, Christian - TECH UNIV MUNICH - Alemania
43 Gabbert, Dominik - Univ Hosp Schleswig Holstein - Alemania
44 Prsa, Milan - Lausanne Univ Hosp - Suiza
45 Patel, Jyoti Kandlikar - Indiana Univ Sch Med - Estados Unidos
46 Hornung, Andreas - UNIV TUBINGEN - Alemania
47 Diab, Simone Goa - Oslo Univ Hosp - Noruega
48 House, Aswathy Vaikom - UNIV OKLAHOMA - Estados Unidos
49 Rakowski, Harry - Univ Hlth Network - Canadá
50 Benson, Lee - Hosp Sick Children - Canadá
51 Maron, Martin S. - Lahey Hosp & Med Ctr - Estados Unidos
52 Grosse-Wortmann, Lars - Oregon Hlth & Sci Univ - Estados Unidos

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Financiamiento



Fuente
Children's Cardiomyopathy Foundation

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Agradecimientos



Agradecimiento
This study was supported by the Children's Cardiomyopathy Foundation.

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