Ichikawa Yasushi, Oota Eri, Odajima Susumu, Kintsu Masayuki, Todo Saki, Takeuchi Kimikazu, Yamauchi Yuki, Shiraki Hiroaki, Yamashita Kentaro, Fukuda Terunobu, Hisamatsu Eriko, Hirata Ken-Ichi, Tanaka Hidekazu
Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine.
Circ J. 2023 Mar 24;87(4):508-516. doi: 10.1253/circj.CJ-22-0683. Epub 2023 Mar 4.
The efficacy of a therapy for patients with transthyretin amyloid cardiomyopathy (ATTR-CM) has not been proven, but tafamidis has been associated with favorable outcomes. However, echocardiographic details of the association of tafamidis with cardiac morphology remain undetermined. Moreover, whether the efficacy of tafamidis varies with the degree of cardiac involvement remains unknown. Using echocardiography, this study investigated the impact of tafamidis on the cardiac morphology of patients with ATTR-CM.
Of 52 consecutive patients with biopsy-proven ATTR-CM at Kobe University Hospital, we included 41 for whom details of follow-up echocardiographic examinations after the administration of tafamidis were available. All patients underwent standard and speckle-tracking echocardiography before and a mean (±SD) of 16±8 months after the administration of tafamidis. No significant changes were observed in any representative echocardiographic parameters after the administration of tafamidis. Furthermore, there were no significant changes observed in subgroup analyses (e.g., left ventricular [LV] ejection fraction ≥50% vs. <50%; LV mass index <150 vs. ≥150 g/m; New York Heart Association Class I-II vs. Class III; age ≥80 vs. <80 years).
Tafamidis may prevent worsening of various representative echocardiographic parameters of patients with ATTR-CM. This effect is also seen in patients with relatively advanced disease and in those who are elderly.
转甲状腺素蛋白淀粉样变心肌病（ATTR-CM）患者的一种治疗方法的疗效尚未得到证实，但氯苯唑酸已显示出良好的治疗效果。然而，氯苯唑酸与心脏形态学之间关联的超声心动图细节仍未明确。此外，氯苯唑酸的疗效是否随心脏受累程度而变化仍不清楚。本研究采用超声心动图，调查了氯苯唑酸对ATTR-CM患者心脏形态的影响。
在神户大学医院连续52例经活检证实为ATTR-CM的患者中，我们纳入了41例在服用氯苯唑酸后有超声心动图随访检查详细资料的患者。所有患者在服用氯苯唑酸前及服用后平均（±标准差）16±8个月接受了标准和斑点追踪超声心动图检查。服用氯苯唑酸后，任何代表性的超声心动图参数均未观察到显著变化。此外，在亚组分析中（例如，左心室[LV]射血分数≥50%与<50%；LV质量指数<150与≥150 g/m；纽约心脏协会心功能分级I-II级与III级；年龄≥80岁与<80岁）也未观察到显著变化。
氯苯唑酸可能会防止ATTR-CM患者各种代表性超声心动图参数的恶化。这种效果在病情相对较重的患者和老年患者中也可见。
