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在转甲状腺素蛋白源性淀粉样心肌病的临床谱系中,基底室间隔下壁节段极易发生变形。

Basal inferoseptal segment is highly susceptible to deformation in the clinical spectrum of transthyretin-derived amyloid cardiomyopathy.

作者信息

Tsuruda Toshihiro, Nakada Hiroshi, Yamamura Yoshimasa, Matsuura Yunosuke, Ogata Miyuki, Tanaka Miyo, Suiko Yosuke, Komaki Soichi, Tanaka Hiroki, Moribayashi Kohei, Ideguchi Takeshi, Terada Tamasa, Ota Tomomi, Yamamoto Keisuke, Nishihira Kensaku, Shibata Yoshisato, Kaikita Koichi

机构信息

Cardiorenal Research Laboratory, Department of Hemo-Vascular Advanced Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan.

Division of Cardiovascular Medicine and Nephrology, Department of Internal Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan.

出版信息

Eur Heart J Open. 2024 Sep 2;4(5):oeae076. doi: 10.1093/ehjopen/oeae076. eCollection 2024 Sep.

DOI:10.1093/ehjopen/oeae076
PMID:39286751
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11404357/
Abstract

AIMS

While the prevalence of transthyretin-derived amyloid cardiomyopathy (ATTR-CM) is on the rise, detailed understanding of its morphological and functional characteristics within the left ventricle (LV) across heart failure (HF) remains limited.

METHODS AND RESULTS

Utilizing two-dimensional (2D) speckle-tracking echocardiography, we assessed longitudinal strain (LS) in 63 histology-confirmed ATTR-CM patients. Additionally, cardiac magnetic resonance (CMR) images measured native T1 and extracellular volume (ECV), compared with LS across 18 LV segments. Patients were categorized into three groups based on HF status: Group 1 (no HF symptoms), Group 2 (HF with preserved LV ejection fraction), and Group 3 (HF with reduced LV ejection fraction). LS analysis unveiled susceptibility to deformation in the basal inferoseptal segment, persisting even in asymptomatic cases. CMR demonstrated increasing native T1 deviation, particularly evident in segments distant from the inferoseptal region. Contrastingly, maximal ECV was consistently observed in the basal and mid-ventricular inferior-septum, even in asymptomatic individuals. Segmental LS decline correlated with ECV expansion but not with native T1 values.

CONCLUSION

Our findings suggest that the inferoseptal segment is highly susceptible to amyloid infiltration, and 2D speckle-tracking echocardiography and CMR may serve as a valuable tool for its early detection.

摘要

目的

虽然转甲状腺素蛋白源性淀粉样心肌病(ATTR-CM)的患病率呈上升趋势,但对于其在心力衰竭(HF)患者左心室(LV)中的形态和功能特征的详细了解仍然有限。

方法和结果

利用二维(2D)斑点追踪超声心动图,我们评估了63例经组织学确诊的ATTR-CM患者的纵向应变(LS)。此外,心脏磁共振(CMR)图像测量了固有T1和细胞外容积(ECV),并与18个LV节段的LS进行了比较。根据HF状态将患者分为三组:第1组(无HF症状)、第2组(HF且LV射血分数保留)和第3组(HF且LV射血分数降低)。LS分析揭示了基底室下间隔节段的变形易感性,即使在无症状病例中也持续存在。CMR显示固有T1偏差增加,在远离室下间隔区域的节段中尤为明显。相反,即使在无症状个体中,最大ECV也始终出现在基底和心室中部下间隔。节段性LS下降与ECV扩大相关,但与固有T1值无关。

结论

我们的研究结果表明,室下间隔节段极易受到淀粉样蛋白浸润,二维斑点追踪超声心动图和CMR可能是早期检测该疾病的有价值工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/55f8d413be81/oeae076f6.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/55f8d413be81/oeae076f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/a10cce7e427b/oeae076_ga.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/e5670a2a79a8/oeae076f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/5e7996ceccd0/oeae076f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/b19381cb4e8f/oeae076f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/7ddaa747f416/oeae076f4.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb44/11404357/55f8d413be81/oeae076f6.jpg

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Coronary microvascular dysfunction affects left ventricular global longitudinal strain response to dipyridamole stress echocardiography: a pilot study.冠状动脉微血管功能障碍影响左心室整体纵向应变对双嘧达莫负荷超声心动图的反应:一项初步研究。
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