因肥厚性心肌病而行肌切除术的患者中淀粉样蛋白沉积的患病率。

IF 1.9 4区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Cardiovascular Pathology Pub Date : 2025-09-11 DOI:10.1016/j.carpath.2025.107779

David S. Katzianer , Deborah H. Kwon , Maran Thamilarasan , J. Emanuel Finet , Wael Jaber , Milind Desai , Nicholas Smedira , E. Rene Rodriguez , Carmela D. Tan , Mazen Hanna

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引用次数: 0

摘要

背景：心脏淀粉样变性（CA）和肥厚性心肌病（HCM）都具有左心室壁厚度增加的表型特征，虽然在HCM中更为常见，但这两种疾病都可导致动态左心室流出（LVOT）梗阻。我们试图检查淀粉样蛋白沉积的发生率在一个高容量中心的肌瘤切除手术标本，并描述病理特征和患者群体。方法和结果：我们回顾了我院的手术肌瘤切除数据库，在肌瘤切除标本的病理检查中发现3292例中有27例（0.8%）患有心脏淀粉样变性。其中许多患者术前影像学特征符合肥厚性心肌病伴不对称肥厚和左心室血栓梗阻性生理特征。结论：近1%的患者在病理检查中发现了意想不到的淀粉样蛋白沉积。认识到这一发现，虽然非常罕见，但对这些患者的长期管理很重要。

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Prevalence of amyloid deposition in patients undergoing surgical myectomy for presumed hypertrophic cardiomyopathy

Background

Cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) share the phenotypic characteristic of increased left ventricular wall thickness and, while more common with HCM, both conditions can result in dynamic left ventricular outflow (LVOT) obstruction. We sought to examine the incidence of amyloid deposition in myectomy specimens at a high-volume center for surgical myectomy and describe the pathologic characteristics and patient population.

Methods and Results

We reviewed the surgical myectomy database at our institution and found a total of 27 of 3,292 (0.8 %) with cardiac amyloidosis on pathologic examination of myectomy specimens. Many of these had preoperative imaging features consistent with hypertrophic cardiomyopathy with asymmetric hypertrophy and LVOT obstructive physiology.

Conclusion

Nearly 1 % of patients referred for surgical myectomy were found to have unexpected amyloid deposits on pathologic examination. Recognition of this finding, although very infrequent, is important for long-term management of these patients.

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来源期刊

Cardiovascular Pathology 医学-病理学

CiteScore

7.50

自引率

2.70%

发文量

审稿时长

18 days

期刊介绍： Cardiovascular Pathology is a bimonthly journal that presents articles on topics covering the entire spectrum of cardiovascular disease. The Journal''s primary objective is to publish papers on disease-oriented morphology and pathogenesis from clinicians and scientists in the cardiovascular field. Subjects covered include cardiovascular biology, prosthetic devices, molecular biology and experimental models of cardiovascular disease.