Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts.
Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts.
Am J Cardiol. 2019 Jul 1;124(1):122-130. doi: 10.1016/j.amjcard.2019.03.035. Epub 2019 Apr 9.
Wild-type transthyretin amyloidosis (ATTRwt) results in an infiltrative cardiomyopathy often culminating in symptomatic heart failure. The use of cardiopulmonary exercise testing (CPET) in determining outcomes in ATTRwt cardiac amyloidosis is unknown. Given the emergence of novel therapies to treat transthyretin amyloidosis, we sought to investigate the utility of CPET on outcomes in patients with ATTRwt cardiomyopathy. Fifty-six patients, with biopsy and immunohistochemically proved ATTRwt, were enrolled between 2005 and 2015, as part of an NIH ATTRwt substudy at the Boston University Amyloidosis Center. Patients were prospectively studied, which included laboratory tests, electrocardiogram, echocardiography, in addition to CPET. In this cohort of ATTRwt patients who performed CPET were elderly, all were male, and predominantly white (69.9%). The overall median survival was 59.01 months (95% confidence interval [CI] 49.29 to 88.69). By multivariate analysis, C-reactive protein (CRP; hazard ratio [HR] 1.10 [1.03 to 1.18]), decreased sodium (HR 0.75 [0.58 to 0.97]), creatinine (HR 7.48 [2.44 to 22.98]) and VE/VCO (HR 1.10 [1.05 to 1.16]) were significant risk factors for mortality (p <0.05). Peak VO was insignificant by both univariate and multivariate analyses. ATTRwt patients with VE/VCO >40 had a worse median survival of 38.54 months (95% CI 32.63 to 51.47) versus 88.69 months (95% CI 56.26 to 89.49) than patients with VE/VCO slope ≤40. Receiver-operating characteristic curve showed that the combination of VE/VCO CRP, sodium, and creatinine (Area under the ROC Curve [AUC], 0.89) predicted 1-year mortality in ATTRwt cardiac amyloidosis. In conclusion, increased VE/VCO, in combination with CRP, sodium, and creatinine, may identify patients at increased risk of death in ATTRwt cardiomyopathy. VE/VCO might have a role in objectively assessing therapeutic response in ATTRwt cardiac amyloidosis.
野生型转甲状腺素蛋白淀粉样变（ATTRwt）可导致浸润性心肌病，最终常导致有症状的心力衰竭。CPET 在确定 ATTRwt 心脏淀粉样变性患者的结局方面的作用尚不清楚。鉴于新型治疗转甲状腺素蛋白淀粉样变性的出现，我们试图研究 CPET 在 ATTRwt 心肌病患者结局中的效用。
2005 年至 2015 年期间，作为波士顿大学淀粉样变性中心 NIH ATTRwt 子研究的一部分，有 56 名经活检和免疫组织化学证实为 ATTRwt 的患者被纳入研究。前瞻性研究包括实验室检查、心电图、超声心动图以及 CPET。在这组进行 CPET 的 ATTRwt 患者中，年龄较大，均为男性，且主要为白人（69.9%）。整体中位生存期为 59.01 个月（95%置信区间[CI]为 49.29 至 88.69）。多变量分析显示，C 反应蛋白（CRP；危险比[HR]为 1.10[1.03 至 1.18]）、降低的钠（HR 为 0.75[0.58 至 0.97]）、肌酐（HR 为 7.48[2.44 至 22.98]）和 VE/VCO（HR 为 1.10[1.05 至 1.16]）是死亡率的显著危险因素（p<0.05）。VE/VCO 在单变量和多变量分析中均无意义。VE/VCO>40 的 ATTRwt 患者中位生存期更差，为 38.54 个月（95%CI 为 32.63 至 51.47），而 VE/VCO 斜率≤40 的患者中位生存期为 88.69 个月（95%CI 为 56.26 至 89.49）。ROC 曲线显示，VE/VCO、CRP、钠和肌酐的组合（ROC 曲线下面积[AUC]，0.89）可预测 1 年 ATTRwt 心脏淀粉样变性的死亡率。
总之，VE/VCO 升高，加上 CRP、钠和肌酐，可能识别出 ATTRwt 心肌病中死亡风险增加的患者。VE/VCO 可能在客观评估 ATTRwt 心脏淀粉样变性的治疗反应方面发挥作用。
