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. 2023 Oct:264:153-162.
doi: 10.1016/j.ahj.2023.06.005. Epub 2023 Jun 12.

Cardiac imaging and biomarkers for assessing myocardial fibrosis in children with hypertrophic cardiomyopathy

Sonya Kirmani  1 , Pamela K Woodard  2 , Ling Shi  3 , Taye H Hamza  4 , Charles E Canter  5 , Steven D Colan  6 , Elfriede Pahl  7 , Jeffrey A Towbin  8 , Steven A Webber  9 , Joseph W Rossano  10 , Melanie D Everitt  11 , Kimberly M Molina  12 , Paul F Kantor  13 , John L Jefferies  14 , Brian Feingold  15 , Linda J Addonizio  16 , Stephanie M Ware  17 , Wendy K Chung  16 , Jean A Ballweg  18 , Teresa M Lee  16 , Neha Bansal  19 , Hiedy Razoky  20 , Jason Czachor  20 , Fatima I Lunze  21 , Edward Marcus  6 , Paul Commean  2 , James D Wilkinson  9 , Steven E Lipshultz  22
Affiliations

Cardiac imaging and biomarkers for assessing myocardial fibrosis in children with hypertrophic cardiomyopathy

Sonya Kirmani et al. Am Heart J. 2023 Oct.

Abstract

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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Conflict of interest statement

Declaration of Interests:

E Pahl is a consultant for Tenaya Therapeutics. JW Rossano is a consultant for Bayer, Abiomed, Novartis, Cytokinetics, and Myokardia. PK Woodard has a research agreement/funding with Siemens Medical Systems and research funding from Bayer. B Feingold is a consultant to Stealth Biotherapeutics. SE Lipshultz has had consultant agreements with Tenaya Therapeutics and Bayer, and has served on an advisory board for Myokardia. He is also the chairman of the medical advisory board of the CCF.

Figures

Figure 1.
Figure 1.
Agreement Between Cardiac Measurements from Transthoracic Echocardiograms and Cardiac Magnetic Resonance Images (cMRI) in Children with Hypertrophic Cardiomyopathy, as Determined with the Bland-Altman Method. All measurements were adjusted for BSA except interventricular septal thickness. A) Left ventricular end-diastolic volume (LVEDV), B) Left ventricular end-systolic volume (LVESV), C) Left ventricular (LV) mass, D) Interventricular septal thickness (ST), E) Interventricular septal thickness (ST) >20 mm by echocardiogram.
Figure 1.
Figure 1.
Agreement Between Cardiac Measurements from Transthoracic Echocardiograms and Cardiac Magnetic Resonance Images (cMRI) in Children with Hypertrophic Cardiomyopathy, as Determined with the Bland-Altman Method. All measurements were adjusted for BSA except interventricular septal thickness. A) Left ventricular end-diastolic volume (LVEDV), B) Left ventricular end-systolic volume (LVESV), C) Left ventricular (LV) mass, D) Interventricular septal thickness (ST), E) Interventricular septal thickness (ST) >20 mm by echocardiogram.
Figure 1.
Figure 1.
Agreement Between Cardiac Measurements from Transthoracic Echocardiograms and Cardiac Magnetic Resonance Images (cMRI) in Children with Hypertrophic Cardiomyopathy, as Determined with the Bland-Altman Method. All measurements were adjusted for BSA except interventricular septal thickness. A) Left ventricular end-diastolic volume (LVEDV), B) Left ventricular end-systolic volume (LVESV), C) Left ventricular (LV) mass, D) Interventricular septal thickness (ST), E) Interventricular septal thickness (ST) >20 mm by echocardiogram.
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