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

IF 4.2 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

Journal of Cardiovascular Magnetic Resonance Pub Date : 2024-08-02 DOI:10.1016/j.jocmr.2024.101072

Shiro Nakamori, Ethan J Rowin, Jennifer Rodriguez, Long H Ngo, Warren J Manning, Martin Maron, Reza Nezafat

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引用次数: 0

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.

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肥厚型心肌病中青年患者心肌加速纤维化

背景：肥厚型心肌病（HCM）患者心血管磁共振（CMR）上的晚期钆增强（LGE）程度与心脏性猝死事件的风险增加有关。然而，LGE 的年龄特异性纵向变化在 HCM 中的临床意义尚不明确。我们试图评估中青年（20-59 岁）和老年（≥ 60 岁）HCM 患者的 LGE 进展风险是否存在差异：共有 102 名 HCM 患者（年龄在 6 SD 以上）对远端心肌进行了归零，并手动调整了灰度阈值：102 例 HCM 患者中有 61 例（60%）在基线时发现 LGE，占左心室（LV）质量的 4.8 ± 3.9%。在随访期结束时，61 名患者中有 53 名（87%）的 LGE 扩大到 7.7 ± 5.4%，8 名患者没有变化。有 5 名患者（5%）的 LGE 扩大到 LV 质量的 15%以上。LGE 的进展速度为 0.7 ± 1.0%/年，其中 21 例患者（21%）的进展速度特别快，≥1%/年。结论：在 HCM 患者中，进展性纤维化更多发生在中青年患者中，这就强调了重复 CMR 的重要性，以重新评估这一年龄组患者潜在的 LGE 进展。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Cardiovascular Magnetic Resonance 医学-核医学

CiteScore

10.90

自引率

12.50%

发文量

审稿时长

6-12 weeks

期刊介绍： Journal of Cardiovascular Magnetic Resonance (JCMR) publishes high-quality articles on all aspects of basic, translational and clinical research on the design, development, manufacture, and evaluation of cardiovascular magnetic resonance (CMR) methods applied to the cardiovascular system. Topical areas include, but are not limited to: New applications of magnetic resonance to improve the diagnostic strategies, risk stratification, characterization and management of diseases affecting the cardiovascular system. New methods to enhance or accelerate image acquisition and data analysis. Results of multicenter, or larger single-center studies that provide insight into the utility of CMR. Basic biological perceptions derived by CMR methods.