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轻链型淀粉样变性患者左心房重构及特征追踪衍生的左心房应变的预后价值:一项心血管磁共振研究

Left atrial remodeling and the prognostic value of feature tracking derived left atrial strain in patients with light-chain amyloidosis: a cardiovascular magnetic resonance study.

作者信息

Tan Zekun, Yang Yuelong, Wu Xinyi, Li Sheng, Li Liwen, Zhong Liye, Lin Qiongwen, Fei Hongwen, Liao Pengjun, Wang Wenjian, Liu Hui

机构信息

The Second School of Clinical Medicine, Southern Medical University, Guangzhou, Guangdong, China.

Department of Radiology, Guangdong Provincial Key Laboratory of Artificial Intelligence in Medical Image Analysis and Application, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.

出版信息

Int J Cardiovasc Imaging. 2022 Jul;38(7):1519-1532. doi: 10.1007/s10554-022-02534-x. Epub 2022 Feb 3.

Abstract

Systemic light-chain (AL) amyloidosis is characterized by the aggregation of misfolded immunoglobulin light chain, predominantly infiltrating in the heart, including left atrium (LA). LA remodeling, such as increased interatrial septal thickness and enlarged size, has been observed. However, LA strain assessed by cardiac magnetic resonance feature tracking (CMR-FT) and its prognostic role remains to be further determined. Using CMR, the current study sought to investigate the characteristic of LA remodeling and the prognostic value of LA strain in patients with AL. Eighty-seven consecutive patients who underwent CMR with histologically confirmed systemic light-chain amyloidosis were retrospectively enrolled. LA strain parameters were analyzed based on CMR-FT algorithm. Amyloid infiltration and burden loads were assessed with CMR late gadolinium enhancement (LGE) and extracellular volume (ECV). Patients were categorized according to the extent of amyloid infiltration in cardiac myocardium. The primary endpoint was defined as all-cause mortality. The prognosis value of LA strain indices was evaluated using Cox proportional hazards regression and Kaplan-Meier curves. Interatrial septal thickness (3 [2-5] vs. 4 [3-5] mm, p = 0.007) and indexed LA volume (34.6 [26.9-44.6] vs. 50.5 [36.1-58.5] ml/m, p = 0.001) were significantly higher in patients with atrial involvement (LA-LGE). Compared with patients with low amyloid burden loads (ECV group I), those at moderate and high (ECV group II and III) show progressive impairment in LA reservoir, conduit, and booster strains and strain rates. A total of 44 patients died during a median follow-up of 12 months. In multivariate analysis, LA reservoir strain, New York Heart Association (NYHA), and ECV remained independently associated with survival. On Kaplan-Meier analyses, low LA reservoir strain (< 8.6%) increased the risk of mortality. In moderate amyloid burden loads patients, low LA reservoir strain provides additive prognosis value. Progress left atrial remodeling and dysfunction are common findings in AL cardiac amyloidosis. The CMR-FT-derived LA reservoir strain provides independent and additive prognostic value for all-cause mortality in patients with AL cardiac amyloidosis.

摘要

系统性轻链(AL)淀粉样变性的特征是错误折叠的免疫球蛋白轻链聚集,主要浸润心脏,包括左心房(LA)。已观察到LA重塑,如房间隔厚度增加和尺寸增大。然而,通过心脏磁共振特征追踪(CMR-FT)评估的LA应变及其预后作用仍有待进一步确定。本研究利用CMR,旨在探讨AL患者LA重塑的特征及LA应变的预后价值。回顾性纳入了87例经组织学证实为系统性轻链淀粉样变性且接受CMR检查的连续患者。基于CMR-FT算法分析LA应变参数。采用CMR延迟钆增强(LGE)和细胞外容积(ECV)评估淀粉样蛋白浸润和负荷。根据心肌淀粉样蛋白浸润程度对患者进行分类。主要终点定义为全因死亡率。使用Cox比例风险回归和Kaplan-Meier曲线评估LA应变指标的预后价值。心房受累(LA-LGE)患者的房间隔厚度(3[2-5] vs. 4[3-5]mm,p = 0.007)和LA指数容积(34.6[26.9-44.6] vs. 50.5[36.1-58.5]ml/m,p = 0.001)显著更高。与低淀粉样蛋白负荷患者(ECV I组)相比,中高负荷患者(ECV II组和III组)LA的储存、管道和增强应变及应变率逐渐受损。在中位随访12个月期间,共有44例患者死亡。多因素分析显示,LA储存应变、纽约心脏协会(NYHA)分级和ECV仍与生存独立相关。Kaplan-Meier分析显示,低LA储存应变(<8.6%)增加死亡风险。在中度淀粉样蛋白负荷患者中,低LA储存应变具有额外的预后价值。进展性左心房重塑和功能障碍是AL心脏淀粉样变性的常见表现。CMR-FT衍生的LA储存应变可为AL心脏淀粉样变性患者的全因死亡率提供独立且额外的预后价值。

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