Nagai Tomoo, Horinouchi Hitomi, Hashimoto Kaho, Ijichi Takeshi, Yoshioka Koichiro, Ikari Yuji
Division of Cardiology, Department of Internal Medicine, Tokai University School of Medicine, Shimokasuya 143, Isehara-shi, Kanagawa, 259-1193, Japan.
Int J Cardiovasc Imaging. 2025 Jan;41(1):93-101. doi: 10.1007/s10554-024-03293-7. Epub 2024 Nov 28.
Baseline cardiac functions are known to be potent predictors of cardiovascular events in the natural history of transthyretin amyloid cardiomyopathy (ATTR-CM). However, previous studies have not shown functional and morphological changes in the heart during tafamidis administration. This study aimed to evaluate the effect of tafamidis on cardiac function by measuring right ventricular strain in patients with ATTR-CM. We performed a retrospective analysis of serial transthoracic echocardiography examinations (at baseline and one year after tafamidis introduction) using a vendor-independent speckle-tracking analyzer in patients with ATTR-CM. The entire cohort (n = 33; 30 men; mean age, 81 ± 5 years) was divided into two subgroups: the tafamidis treatment (n = 18) and the control (n = 15). After one-year tafamidis administration (oral tafamidis meglumine, 80 mg once daily), the average value of the right ventricular (RV) free wall longitudinal strain (RVFWLS) significantly improved (-17.8 ± 7.9% vs. -24.5 ± 9.1%, p = 0.001), with a significant decrease in the frequency of positive RVFWLS test result (≥-22.0%) in the treatment subgroup (72% vs. 44%, p = 0.033). Moreover, tafamidis administration had large impact on RVFWLS improvement (p = 0.007, odds ratio: 22.0, 95% confidence interval: 2.344-206.480). A significant recovery of RV function was measured using RVFWLS. This may be one of the pathophysiological mechanisms underlying the favorable effects of tafamidis.
已知基线心脏功能是转甲状腺素蛋白淀粉样心肌病（ATTR-CM）自然病程中心血管事件的有力预测指标。然而，先前的研究并未显示在服用他氟米特期间心脏的功能和形态变化。本研究旨在通过测量ATTR-CM患者的右心室应变来评估他氟米特对心脏功能的影响。我们使用独立于供应商的斑点追踪分析仪，对ATTR-CM患者的系列经胸超声心动图检查（基线时和引入他氟米特一年后）进行了回顾性分析。整个队列（n = 33；30名男性；平均年龄，81±5岁）分为两个亚组：他氟米特治疗组（n = 18）和对照组（n = 15）。服用他氟米特一年后（口服他氟米特葡甲胺，80 mg每日一次），右心室（RV）游离壁纵向应变（RVFWLS）的平均值显著改善（-17.8±7.9%对-24.5±9.1%，p = 0.001），治疗亚组中RVFWLS检测结果为阳性（≥-22.0%）的频率显著降低（72%对44%，p = 0.033）。此外，服用他氟米特对RVFWLS的改善有很大影响（p = 0.007，优势比：22.0，95%置信区间：2.344 - 206.480）。使用RVFWLS测量发现RV功能有显著恢复。这可能是他氟米特产生有利作用的病理生理机制之一。
