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| 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
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.
| Ord. | Autor | Género | Institución - País |
|---|---|---|---|
| 1 | Chan, Raymond H. | - |
Univ Hlth Network - Canadá
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| 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
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| 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
|