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Cardiac imaging and biomarkers for assessing myocardial fibrosis in children with hypertrophic cardiomyopathy - 13/09/23

Doi : 10.1016/j.ahj.2023.06.005 
Sonya Kirmani, MD a, Pamela K. Woodard, MD b, Ling Shi, PhD c, Taye H. Hamza, PhD d, Charles E. Canter, MD e, Steven D. Colan, MD f, Elfriede Pahl, MD g, Jeffrey A. Towbin, MD h, Steven A. Webber, MBChB i, Joseph W. Rossano, MD, MS j, Melanie D. Everitt, MD k, Kimberly M. Molina, MD l, Paul F. Kantor, MBBCh, MSc m, John L. Jefferies, MD n, Brian Feingold, MD, MS o, Linda J. Addonizio, MD p, Stephanie M. Ware, MDPhD q, Wendy K. Chung, MDPhD p, Jean A. Ballweg, MD r, Teresa M. Lee, MD p, Neha Bansal, MD s, Hiedy Razoky, MBA t, Jason Czachor, MS t, Fatima I. Lunze, MD, ScD, PhD f, u, Edward Marcus, MSc f, Paul Commean, BEE b, James D. Wilkinson, MD, MPH i, Steven E. Lipshultz, MD v,
a Department of Pediatrics, University of Wisconsin-Madison, Madison, WI 
b Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO 
c New England Research Institute, Watertown, MA 
d HealthCore, Watertown, MA 
e Department of Pediatrics, Washington University School of Medicine, St. Louis, MO 
f Department of Cardiology, Boston Children's Hospital, Boston, MA 
g Department of Pediatrics, Northwestern Feinberg School of Medicine, Chicago, IL 
h Heart Institute, Le Bonheur Children's Hospital, Memphis, TN 
i Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN 
j Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 
k Department of Pediatrics, Children's Hospital of Colorado, Aurora, CO 
l Department of Pediatrics, Primary Children's Hospital, Salt Lake City, UT 
m Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, CA 
n Department of Medicine, University of Tennessee, Memphis, TN 
o Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA 
p Department of Pediatrics, Columbia University Irving Medical Center, New York, NY 
q Department of Pediatrics and Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 
r Department of Pediatrics, Helen DeVos Children's Hospital, Grand Rapids, MI 
s Department of Pediatrics, Children's Hospital at Montefiore, Bronx, NY 
t Department of Pediatrics, Children's Hospital of Michigan, Detroit, MI 
u German Heart Center Berlin, Charité Medical School, Berlin, Germany 
v Department of Pediatrics, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY 

Reprint requests: Steven E. Lipshultz, MD, FAAP, FAHA, Department of Pediatrics, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Clinical and Translational Research Center, 875 Ellicott Street, Suite 5018, Buffalo, New York 14203.Department of PediatricsUniversity at Buffalo Jacobs School of Medicine and Biomedical Sciences, Clinical and Translational Research Center875 Ellicott Street, Suite 5018, BuffaloNew York14203

Résumé

Background

Myocardial fibrosis, as diagnosed on cardiac magnetic resonance imaging (cMRI) by late gadolinium enhancement (LGE), is associated with adverse outcomes in adults with hypertrophic cardiomyopathy (HCM), but its prevalence and magnitude in children with HCM have not been established. We investigated: (1) the prevalence and extent of myocardial fibrosis as detected by LGE cMRI; (2) the agreement between echocardiographic and cMRI measurements of cardiac structure; and (3) whether serum concentrations of N-terminal pro hormone B-type natriuretic peptide (NT-proBNP) and cardiac troponin-T are associated with cMRI measurements.

Methods

A cross-section of children with HCM from 9 tertiary-care pediatric heart centers in the U.S. and Canada were enrolled in this prospective NHLBI study of cardiac biomarkers in pediatric cardiomyopathy (ClinicalTrials.gov Identifier: NCT01873976). The median age of the 67 participants was 13.8 years (range 1-18 years). Core laboratories analyzed echocardiographic and cMRI measurements, and serum biomarker concentrations.

Results

In 52 children with non-obstructive HCM undergoing cMRI, overall low levels of myocardial fibrosis with LGE >2% of left ventricular (LV) mass were detected in 37 (71%) (median %LGE, 9.0%; IQR: 6.0%, 13.0%; range, 0% to 57%). Echocardiographic and cMRI measurements of LV dimensions, LV mass, and interventricular septal thickness showed good agreement using the Bland-Altman method. NT-proBNP concentrations were strongly and positively associated with LV mass and interventricular septal thickness (P < .001), but not LGE.

Conclusions

Low levels of myocardial fibrosis are common in pediatric patients with HCM seen at referral centers. Longitudinal studies of myocardial fibrosis and serum biomarkers are warranted to determine their predictive value for adverse outcomes in pediatric patients with HCM.

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Abbreviations : BNP, BSA, CLIA, cMRI, HF, HCM, IQR, ICD, LGE, LV, LVEDV, LVESV, NHLBI, NT-proBNP, SCD


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Vol 264

P. 153-162 - octobre 2023 Retour au numéro
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