Department of Cardiovascular Medicine, University of Kansas Medical Center, 3901 Rainbow Blvd, Kansas City, Kansas.
Department of Internal Medicine, University of Kansas Medical Center, Kansas City, Kansas.
J Card Fail. 2021 Nov;27(11):1285-1289. doi: 10.1016/j.cardfail.2021.06.022. Epub 2021 Jul 16.
The prognostic value of cardiopulmonary exercise testing (CPET) in patients with wild-type transthyretin cardiac amyloidosis treated with tafamidis is unknown.
This retrospective study included patients with wtATTR who underwent baseline cardiopulmonary exercise testing and were treated with tafamidis from August 31, 2018, until March 31, 2020. Univariate logistic and multivariate cox-regression models were used to predict the occurrence of the primary outcome (composite of mortality, heart transplant, and palliative inotrope initiation). A total of 33 patients were included (median age 82 years, interquartile range [IQR] 79-84 years), 84% were Caucasians and 79% were males). Majority of patients had New York Heart Association functional class III disease at baseline (67%). The baseline median peak oxygen consumption (VO) and peak circulatory power (CP) were 11.35 mL/kg/min (IQR 8.5-14.2 mL/kg/min) and 1485.8 mm Hg/mL/min (IQR 988-2184 mm Hg/mL/min), respectively, the median ventilatory efficiency was 35.7 (IQR 31-41.2). After 1 year of follow-up, 11 patients experienced a primary end point. Upon multivariate analysis, the low peak VO (hazard ratio [HR] 0.43, 95% confidence interval [CI] 0.23-0.79, P = .007], peak CP (HR 0.98, 95% CI 0.98-0.99, P = .02), peak oxygen pulse (HR 0.62, 95% CI 0.39-0.97, P = .03), and exercise duration of less than 5.5 minutes (HR 5.82, 95% CI 1.29-26.2, P = .02) were significantly associated with the primary outcome.
Tafamidis-treated patients with wtATTR who had baseline low peak VO, peak CP, peak O pulse, and exercise duration of less than 5.5 minutes had worse outcomes.
在接受塔法米替治疗的野生型转甲状腺素蛋白心脏淀粉样变患者中，心肺运动测试（CPET）的预后价值尚不清楚。
这项回顾性研究纳入了自 2018 年 8 月 31 日至 2020 年 3 月 31 日期间接受基线心肺运动测试并接受塔法米替治疗的 wtATTR 患者。采用单变量逻辑和多变量 cox 回归模型预测主要结局（包括死亡率、心脏移植和姑息性正性肌力药起始）的发生。共纳入 33 例患者（中位年龄 82 岁，四分位距 [IQR] 79-84 岁），84%为白种人，79%为男性）。大多数患者在基线时为纽约心脏协会功能分级 III 级疾病（67%）。基线时的中位峰值氧耗量（VO）和峰值循环功率（CP）分别为 11.35 mL/kg/min（IQR 8.5-14.2 mL/kg/min）和 1485.8 mm Hg/mL/min（IQR 988-2184 mm Hg/mL/min），中位通气效率为 35.7（IQR 31-41.2）。在 1 年的随访后，有 11 例患者出现了主要终点。多变量分析显示，低峰值 VO（风险比 [HR] 0.43，95%置信区间 [CI] 0.23-0.79，P=0.007）、峰值 CP（HR 0.98，95% CI 0.98-0.99，P=0.02）、峰值氧脉冲（HR 0.62，95% CI 0.39-0.97，P=0.03）和运动时间少于 5.5 分钟（HR 5.82，95% CI 1.29-26.2，P=0.02）与主要结局显著相关。
在接受塔法米替治疗的 wtATTR 患者中，基线时峰值 VO、CP、O 脉冲和运动时间低于 5.5 分钟的患者预后较差。
