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探讨幼年皮肌炎的心血管影响:从主动脉僵硬度分析和 3D 超声心动图获得的见解。

Exploring cardiovascular implications of juvenile dermatomyositis: Insights from aortic stiffness analysis and 3D echocardiography.

机构信息

Department of Pediatric Cardiology, İstanbul University-Cerrahpaşa Medical School, Istanbul, Turkey.

Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa Medical School, Istanbul, Turkey.

出版信息

Echocardiography. 2024 Feb;41(2):e15776. doi: 10.1111/echo.15776.

Abstract

OBJECTIVE

Our goal was to use three dimensional (3D) strain analysis to evaluate myocardial function and ascending aorta elasticity changes in juvenile dermatomyositis (JDM).

METHODS

Between 2019 and 2021, 23 JDM patients and 20 healthy volunteers participated. Both groups underwent 2D and 3D strain analysis, assessing aortic stiffness using aortic distensibility, stiffness index, strain, and elastic modulus.

RESULTS

JDM patients had a median age of 13.3 ± 5.2 years, while controls had a median age of 13.8 ± 4.76 years. 3D strain analysis revealed significantly lower global longitudinal (GLS) and circumferential strain (GCS) in JDM patients compared to controls. Specifically, 3D GLS was notably reduced in patients (-28.1% vs. -31%, p = .047) compared to controls, and 3D GCS was also lower in patients (-27.5% vs. -30.5%, p = .019) compared to controls. Aortic strain and elastic modulus were significantly lower in JDM patients, while aortic stiffness index and distensibility showed no significant differences. Correlation analyses within the JDM group revealed a negative correlation between 3D GLS and age at diagnosis (r = -.561, p = .04), as well as a positive correlation between 3D GLS and both aortic strain (r = .514, p = .0001) and elastic modulus (r = .320, p = .03) in JDM patients.

CONCLUSION

Our study demonstrated a trend towards lower ejection fraction and strain in patients with JDM, along with increased aortic stiffness using 3D echocardiography. These findings suggest potential cardiovascular involvement in juvenile dermatomyositis, emphasizing the importance of comprehensive cardiac assessments in these patients.

摘要

目的

本研究旨在使用三维(3D)应变分析评估青少年皮肌炎(JDM)患者的心肌功能和升主动脉弹性变化。

方法

2019 年至 2021 年间,共有 23 例 JDM 患者和 20 名健康志愿者参与了本研究。两组均接受了 2D 和 3D 应变分析,通过主动脉可扩张性、僵硬度指数、应变和弹性模量评估主动脉僵硬度。

结果

JDM 患者的中位年龄为 13.3±5.2 岁,而对照组的中位年龄为 13.8±4.76 岁。3D 应变分析显示,JDM 患者的整体纵向应变(GLS)和整体圆周应变(GCS)明显低于对照组。具体来说,与对照组相比,3D GLS 在患者中明显降低(-28.1%对-31%,p=0.047),3D GCS 也在患者中降低(-27.5%对-30.5%,p=0.019)。JDM 患者的主动脉应变和弹性模量明显降低,而主动脉僵硬度指数和可扩张性无明显差异。JDM 组内的相关性分析显示,3D GLS 与诊断时年龄呈负相关(r=-0.561,p=0.04),与主动脉应变呈正相关(r=0.514,p=0.0001)和弹性模量呈正相关(r=0.320,p=0.03)。

结论

本研究表明,JDM 患者的射血分数和应变呈下降趋势,使用 3D 超声心动图检测主动脉僵硬度增加。这些发现提示青少年皮肌炎可能存在潜在的心血管受累,强调了在这些患者中进行全面心脏评估的重要性。

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