Department of Cardiology, Rangueil University Hospital, Toulouse, France.
Department of Cardiology, Rangueil University Hospital, Toulouse, France; Cardiac Imaging Centre, Rangueil University Hospital, France.
Int J Cardiol. 2024 Dec 1;416:132485. doi: 10.1016/j.ijcard.2024.132485. Epub 2024 Aug 24.
Background - Laboratory liver anomalies are common in cardiac amyloidosis; however, their significance regarding liver stiffness is unknown. The aim of this study was to investigate the prevalence, clinical significance, and prognostic value of liver stiffness measurement (LSM) anomalies in transthyretin cardiac amyloidosis (ATTR-CA). Methods - Consecutive patients diagnosed with ATTR-CA who underwent liver stiffness assessment were included in the study. Demographic, clinical, laboratory, transthoracic echocardiography and liver stiffness data were retrospectively collected. LSM was obtained through either transient elastography or supersonic shear imaging. Patient cohort was divided in two groups according to a 10 kPa threshold. Follow up data were collected for the occurrence of hospitalization for heart failure and all-cause death. Results - Two hundred and eighty-four patients with ATTR-CA - 26 (9 %) hereditary variant ATTR, 258 (91 %) wild-type ATTR - were included. A LSM over 10 kPa was found in 4 (15 %) and 98 (38 %) patients with ATTRv and ATTRwt respectively (p = 0.02). Among patients with ATTRwt, high LSM was more frequent in advanced stages of ATTR-CA and was associated with increased risk of hospitalization for heart failure after multivariate analysis with a hazard ratio of 2.41 [1.05-5.55] (p = 0.04). Among patients with NYHA stage 1, 28 % presented high LSM associated with high NT-proBNP levels. Integration of high LSM with NT-proBNP and estimated glomerular filtration rate provided a better estimate of patient survival. Conclusion - LSM over 10 kPa is found in up to 36 % of patients with ATTR-CA and is associated with advanced stages of cardiomyopathy and increased risk of hospitalization for heart failure in ATTRwt patients.
背景 - 心脏淀粉样变性患者常出现实验室肝异常；然而，其与肝硬度的关系尚不清楚。本研究旨在探讨转甲状腺素蛋白心脏淀粉样变（ATTR-CA）患者肝硬度测量（LSM）异常的发生率、临床意义和预后价值。
方法 - 本研究纳入了经肝硬度评估诊断为 ATTR-CA 的连续患者。回顾性收集了人口统计学、临床、实验室、经胸超声心动图和肝硬度数据。LSM 通过瞬态弹性成像或超声剪切波成像获得。根据 10kPa 的阈值将患者队列分为两组。收集随访数据，以记录心力衰竭住院和全因死亡的发生情况。
结果 - 共纳入 284 例 ATTR-CA 患者，其中 26 例（9%）为遗传性变异 ATTR，258 例（91%）为野生型 ATTR。在 ATTRv 和 ATTRwt 患者中，分别有 4 例（15%）和 98 例（38%）患者的 LSM 超过 10kPa（p=0.02）。在 ATTRwt 患者中，高 LSM 在 ATTR-CA 的晚期更为常见，并且在多变量分析中与心力衰竭住院风险增加相关，风险比为 2.41[1.05-5.55]（p=0.04）。在 NYHA 1 期的患者中，有 28%的患者出现高 LSM，与高 NT-proBNP 水平相关。高 LSM 与 NT-proBNP 和估计肾小球滤过率的结合可更好地预测患者的生存情况。
结论 - 在多达 36%的 ATTR-CA 患者中发现 LSM 超过 10kPa，与心肌病的晚期阶段相关，并增加了 ATTRwt 患者心力衰竭住院的风险。
