Titre
Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy: A Multicenter Cohort Study.
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Chan, R.H.
Auteure/Auteur
van der Wal, L.
Auteure/Auteur
Liberato, G.
Auteure/Auteur
Rowin, E.
Auteure/Auteur
Soslow, J.
Auteure/Auteur
Maskatia, S.
Auteure/Auteur
Chan, S.
Auteure/Auteur
Shah, A.
Auteure/Auteur
Fogel, M.
Auteure/Auteur
Hernandez, L.
Auteure/Auteur
Anwar, S.
Auteure/Auteur
Voges, I.
Auteure/Auteur
Carlsson, M.
Auteure/Auteur
Buddhe, S.
Auteure/Auteur
Laser, K.T.
Auteure/Auteur
Greil, G.
Auteure/Auteur
Valsangiacomo-Buechel, E.
Auteure/Auteur
Olivotto, I.
Auteure/Auteur
Wong, D.
Auteure/Auteur
Wolf, C.
Auteure/Auteur
Grotenhuis, H.
Auteure/Auteur
Rickers, C.
Auteure/Auteur
Hor, K.
Auteure/Auteur
Rutz, T.
Auteure/Auteur
Kutty, S.
Auteure/Auteur
Samyn, M.
Auteure/Auteur
Johnson, T.
Auteure/Auteur
Hasbani, K.
Auteure/Auteur
Moore, J.P.
Auteure/Auteur
Sieverding, L.
Auteure/Auteur
Detterich, J.
Auteure/Auteur
Parra, R.
Auteure/Auteur
Chungsomprasong, P.
Auteure/Auteur
Toro-Salazar, O.
Auteure/Auteur
Roest, AAW
Auteure/Auteur
Dittrich, S.
Auteure/Auteur
Brun, H.
Auteure/Auteur
Spinner, J.
Auteure/Auteur
Lai, W.
Auteure/Auteur
Dyer, A.
Auteure/Auteur
Jablonowsk, R.
Auteure/Auteur
Meierhofer, C.
Auteure/Auteur
Gabbert, D.
Auteure/Auteur
Prsa, M.
Auteure/Auteur
Patel, J.K.
Auteure/Auteur
Hornung, A.
Auteure/Auteur
Diab, S.G.
Auteure/Auteur
House, A.V.
Auteure/Auteur
Rakowski, H.
Auteure/Auteur
Benson, L.
Auteure/Auteur
Maron, M.S.
Auteure/Auteur
Grosse-Wortmann, L.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
2380-6591
Statut éditorial
Publié
Date de publication
2024-11-01
Volume
9
Numéro
11
Première page
1001
Dernière page/numéro d’article
1008
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Publication Status: ppublish
Résumé
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.
To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years.
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.
Natural history of HCM.
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.
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.
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.
To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years.
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.
Natural history of HCM.
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.
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.
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.
Sujets
PID Serval
serval:BIB_496998815296
PMID
Date de création
2024-09-30T11:49:57.260Z
Date de création dans IRIS
2025-05-20T17:03:19Z