Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.
Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.
Eur Heart J Cardiovasc Imaging. 2022 Jul 21;23(8):1029-1039. doi: 10.1093/ehjci/jeac049.
In patients with transthyretin amyloid cardiomyopathy (ATTR-CM), the effect of tafamidis on myocardial function using serial speckle tracking echocardiography has not been reported. The purpose of this study was to describe the natural history of myocardial function in untreated ATTR-CM and determine the effect of tafamidis on myocardial functional parameters over 12 months of treatment.
A total of 45 subjects with ATTR-CM were retrospectively studied: 23 treated with tafamidis and 22 untreated. Two-dimensional speckle tracking echocardiography was analysed at baseline and 1 year. Serial longitudinal, circumferential, and radial strain, twist, torsion, and myocardial work were measured. Over 1 year, absolute global longitudinal strain (GLS) deteriorated more in the untreated group by a median of 1.1% [inter-quartile range (IQR) 0.95] compared with 0.3% (IQR 1) in the tafamidis group (P = 0.02). Myocardial work index and efficiency also deteriorated to a greater degree: 142.5 mmHg% (IQR 197) and 4% (IQR 8), respectively, in the untreated group compared with 61.5 mmHg% (IQR 210) and 1% (IQR 7) in the tafamidis group (P = 0.04). There were no significant between group differences in left ventricular ejection fraction (LVEF), tissue Doppler velocities, circumferential or radial strain, LV twist or torsion at 1 year. The stabilization effect of tafamidis on myocardial function at 1 year did not differ according to baseline GLS, LVEF, or National Amyloidosis Centre disease stage.
In ATTR-CM, tafamidis resulted in a lesser deterioration in GLS, myocardial work index, and efficiency over a 12-month period compared with a cohort not treated with tafamidis.
在转甲状腺素蛋白淀粉样心肌病（ATTR-CM）患者中，使用连续斑点追踪超声心动图检测塔法米迪对心肌功能的影响尚未见报道。本研究的目的是描述未经治疗的 ATTR-CM 患者心肌功能的自然病史，并确定塔法米迪在 12 个月治疗期间对心肌功能参数的影响。
共回顾性研究了 45 例 ATTR-CM 患者：23 例接受塔法米迪治疗，22 例未治疗。在基线和 1 年时分析二维斑点追踪超声心动图。测量连续的纵向、周向和径向应变、扭转、扭转和心肌做功。在 1 年内，未经治疗组的整体纵向应变（GLS）绝对值恶化更明显，中位数为 1.1%[四分位距（IQR）0.95]，而塔法米迪组为 0.3%（IQR 1）（P=0.02）。心肌做功指数和效率也恶化得更明显：未经治疗组分别为 142.5mmHg%（IQR 197）和 4%（IQR 8），而塔法米迪组分别为 61.5mmHg%（IQR 210）和 1%（IQR 7）（P=0.04）。两组间左心室射血分数（LVEF）、组织多普勒速度、周向或径向应变、LV 扭转或扭转无显著差异在 1 年时。1 年后，塔法米迪对心肌功能的稳定作用与基线 GLS、LVEF 或国家淀粉样变性中心疾病分期无关。
在 ATTR-CM 中，与未接受塔法米迪治疗的患者相比，塔法米迪在 12 个月内使 GLS、心肌做功指数和效率的恶化程度降低。
