Instituto do Coração (InCor), Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Instituto do Coração (InCor), Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Curr Probl Cardiol. 2025 Jan;50(1):102867. doi: 10.1016/j.cpcardiol.2024.102867. Epub 2024 Oct 10.
Amyloidosis is a group of diseases characterized by the deposition of misfolded protein fragments, forming insoluble fibrils in organs and tissues. Transthyretin (ATTR) amyloidosis, particularly cardiac amyloidosis (CA), leads to myocardial stiffness and heart failure. Right ventricular (RV) involvement is common in CA, but assessing RV stiffness noninvasively is challenging. This study aimed to evaluate RV stiffness using shear wave elastography (SWE) and correlate the findings with clinical, laboratory, and echocardiographic parameters.
In this prospective, single-center, cross-sectional study, 60 patients were divided into three groups: 20 with cardiac ATTR amyloidosis (ATTR-CM), 20 with non-cardiac ATTR amyloidosis (ATTR non-CM), and 20 healthy controls. Myocardial stiffness was measured using SWE in the free wall of the RV. Pearson's and Spearman's correlation coefficients were used for statistical analysis, with significance set at p < 0.05.
RV SWE values showed a strong positive correlation with functional class and a moderate correlation with BNP and troponin I levels. A significant negative correlation was found between RV SWE values and the 6-minute walk test distance. SWE also correlated with echocardiographic variables like interventricular septum thickness and RV basal diameter. An SWE cutoff of ≥ 4.6. kPa was associated with cardiac involvement, showing 65 % sensitivity and 76 % specificity.
SWE is a valuable noninvasive technique for assessing RV stiffness in CA patients, correlating well with clinical and echocardiographic parameters. An RV SWE value of ≥ 4.6 kPa could aid in early detection of cardiac involvement in ATTR amyloidosis, improving diagnosis and management.
淀粉样变性是一组以错误折叠的蛋白质片段沉积为特征的疾病，在器官和组织中形成不溶性纤维。转甲状腺素蛋白（ATTR）淀粉样变性，特别是心脏淀粉样变性（CA），导致心肌僵硬和心力衰竭。右心室（RV）受累在 CA 中很常见，但无创评估 RV 僵硬具有挑战性。本研究旨在使用剪切波弹性成像（SWE）评估 RV 僵硬，并将这些发现与临床、实验室和超声心动图参数相关联。
在这项前瞻性、单中心、横断面研究中，将 60 名患者分为三组：20 名患有心脏ATTR 淀粉样变性（ATTR-CM），20 名患有非心脏ATTR 淀粉样变性（ATTR 非-CM），20 名健康对照者。使用 SWE 测量 RV 游离壁的心肌僵硬。使用 Pearson 和 Spearman 相关系数进行统计学分析，以 p < 0.05 为显著性水平。
RV SWE 值与功能分级呈强正相关，与 BNP 和肌钙蛋白 I 水平呈中度相关。RV SWE 值与 6 分钟步行试验距离呈显著负相关。SWE 还与超声心动图变量如室间隔厚度和 RV 基底直径相关。SWE 截值≥4.6 kPa 与心脏受累相关，敏感性为 65%，特异性为 76%。
SWE 是一种评估 CA 患者 RV 僵硬的有价值的无创技术，与临床和超声心动图参数相关性良好。RV SWE 值≥4.6 kPa 可能有助于早期发现 ATTR 淀粉样变性中的心脏受累，从而改善诊断和管理。
