Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy

ABSTRACT: Background: The extent of late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM) is associated with an increased risk of sudden cardiac death events. However, the clinical significance of age-specific longitudinal ch...

Full description

Saved in:
Bibliographic Details
Main Authors: Shiro Nakamori, Ethan J. Rowin, Jennifer Rodriguez, Long H. Ngo, Warren J. Manning, Martin Maron, Reza Nezafat
Format: Article
Language:English
Published: Elsevier 2024-01-01
Series:Journal of Cardiovascular Magnetic Resonance
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1097664724010998
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850119339095621632
author Shiro Nakamori
Ethan J. Rowin
Jennifer Rodriguez
Long H. Ngo
Warren J. Manning
Martin Maron
Reza Nezafat
author_facet Shiro Nakamori
Ethan J. Rowin
Jennifer Rodriguez
Long H. Ngo
Warren J. Manning
Martin Maron
Reza Nezafat
author_sort Shiro Nakamori
collection DOAJ
description ABSTRACT: Background: The extent of late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM) is associated with an increased risk of sudden cardiac death events. However, the clinical significance of age-specific longitudinal changes in LGE is not well characterized in HCM. We sought to assess whether the risk of LGE progression diverges between young to middle-aged (ages 20-59 years) and older (≥ 60) adults with HCM. Methods: A total of 102 HCM patients (age <60 years; n=75, age ≥60 years; n=27) undergoing serial CMR studies from two tertiary medical centers were evaluated. The median time interval between initial and follow-up CMR scans was 3.7 years. LGE was semiautomatically quantified by measuring regions with signal intensity >6 SD above the nulled remote myocardium and manually adjusting a grayscale threshold. Results: LGE was identified at baseline in 61 of the 102 HCM patients (60%), occupying 4.8 ± 3.9% of the left ventricular (LV) mass. At the end of the follow-up period, 53 of the 61 patients (87%) demonstrated an increase in the extent of LGE to 7.7 ± 5.4%, and 8 patients had no change. In 5 patients (5%), LGE increased to extensive with >15% of the LV mass. The rate of LGE progression was 0.7 ± 1.0%/year, including 21 patients (21%) with particularly accelerated progression of ≥1%/year. The risk of LGE progression ≥1%/year was significantly higher in patients <60 years than those ≥ 60 years (25% vs. 7%, p=0.03). The odds of LGE progression ≥1%/year was almost 4 times greater for patients <60 years compared with those ≥ 60 years (odds ratio, 4.2; 95%CI, 1.1-27.9). Age <60 years and LGE extent ≥ 10% were significant baseline predictors for future LGE progression ≥1%/year, even after adjustment for other potential risk factors. Conclusion: In HCM, progressive fibrosis occurs more frequently in young to middle-aged patients, underscoring the importance of repeating CMR to re-evaluate for potential LGE progression in this age group.
format Article
id doaj-art-4597a5cdcd7a4d74b1fe39f0c1fec5c5
institution OA Journals
issn 1097-6647
language English
publishDate 2024-01-01
publisher Elsevier
record_format Article
series Journal of Cardiovascular Magnetic Resonance
spelling doaj-art-4597a5cdcd7a4d74b1fe39f0c1fec5c52025-08-20T02:35:40ZengElsevierJournal of Cardiovascular Magnetic Resonance1097-66472024-01-0126210107210.1016/j.jocmr.2024.101072Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathyShiro Nakamori0Ethan J. Rowin1Jennifer Rodriguez2Long H. Ngo3Warren J. Manning4Martin Maron5Reza Nezafat6Departments of Medicine (Cardiovascular Division) Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA; Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Tsu, JapanHypertrophic Cardiomyopathy Center, Lahey Medical Center, Burlington, Massachusetts, USA; Tufts University School of Medicine, Boston, Massachusetts, USADepartments of Medicine (Cardiovascular Division) Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USADepartments of Medicine (Cardiovascular Division) Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USADepartments of Medicine (Cardiovascular Division) Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA; Departments of Radiology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USAHypertrophic Cardiomyopathy Center, Lahey Medical Center, Burlington, Massachusetts, USA; Tufts University School of Medicine, Boston, Massachusetts, USADepartments of Medicine (Cardiovascular Division) Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA; Corresponding author. Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, Massachusetts 02215, USA.ABSTRACT: Background: The extent of late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM) is associated with an increased risk of sudden cardiac death events. However, the clinical significance of age-specific longitudinal changes in LGE is not well characterized in HCM. We sought to assess whether the risk of LGE progression diverges between young to middle-aged (ages 20-59 years) and older (≥ 60) adults with HCM. Methods: A total of 102 HCM patients (age <60 years; n=75, age ≥60 years; n=27) undergoing serial CMR studies from two tertiary medical centers were evaluated. The median time interval between initial and follow-up CMR scans was 3.7 years. LGE was semiautomatically quantified by measuring regions with signal intensity >6 SD above the nulled remote myocardium and manually adjusting a grayscale threshold. Results: LGE was identified at baseline in 61 of the 102 HCM patients (60%), occupying 4.8 ± 3.9% of the left ventricular (LV) mass. At the end of the follow-up period, 53 of the 61 patients (87%) demonstrated an increase in the extent of LGE to 7.7 ± 5.4%, and 8 patients had no change. In 5 patients (5%), LGE increased to extensive with >15% of the LV mass. The rate of LGE progression was 0.7 ± 1.0%/year, including 21 patients (21%) with particularly accelerated progression of ≥1%/year. The risk of LGE progression ≥1%/year was significantly higher in patients <60 years than those ≥ 60 years (25% vs. 7%, p=0.03). The odds of LGE progression ≥1%/year was almost 4 times greater for patients <60 years compared with those ≥ 60 years (odds ratio, 4.2; 95%CI, 1.1-27.9). Age <60 years and LGE extent ≥ 10% were significant baseline predictors for future LGE progression ≥1%/year, even after adjustment for other potential risk factors. Conclusion: In HCM, progressive fibrosis occurs more frequently in young to middle-aged patients, underscoring the importance of repeating CMR to re-evaluate for potential LGE progression in this age group.http://www.sciencedirect.com/science/article/pii/S1097664724010998AgeCardiovascular magnetic resonanceHypertrophic cardiomyopathyLate gadolinium enhancementProgressive fibrosis
spellingShingle Shiro Nakamori
Ethan J. Rowin
Jennifer Rodriguez
Long H. Ngo
Warren J. Manning
Martin Maron
Reza Nezafat
Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy
Journal of Cardiovascular Magnetic Resonance
Age
Cardiovascular magnetic resonance
Hypertrophic cardiomyopathy
Late gadolinium enhancement
Progressive fibrosis
title Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy
title_full Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy
title_fullStr Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy
title_full_unstemmed Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy
title_short Accelerated myocardial fibrosis in young to middle-aged patients with hypertrophic cardiomyopathy
title_sort accelerated myocardial fibrosis in young to middle aged patients with hypertrophic cardiomyopathy
topic Age
Cardiovascular magnetic resonance
Hypertrophic cardiomyopathy
Late gadolinium enhancement
Progressive fibrosis
url http://www.sciencedirect.com/science/article/pii/S1097664724010998
work_keys_str_mv AT shironakamori acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy
AT ethanjrowin acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy
AT jenniferrodriguez acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy
AT longhngo acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy
AT warrenjmanning acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy
AT martinmaron acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy
AT rezanezafat acceleratedmyocardialfibrosisinyoungtomiddleagedpatientswithhypertrophiccardiomyopathy