Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart and Vascular Institute, Cleveland Clinic Foundation, Cleveland, Ohio; The George M. and Linda H. Kaufman Center for Heart Failure and Recovery, Cleveland Clinic, Cleveland, Ohio.
Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart and Vascular Institute, Cleveland Clinic Foundation, Cleveland, Ohio.
J Card Fail. 2022 Oct;28(10):1509-1518. doi: 10.1016/j.cardfail.2022.06.005. Epub 2022 Jul 14.
Transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure. Given the expansion of noninvasive diagnosis with Tc-pyrophosphate (Tc-PYP) scanning, and clinical use of the transthyretin stabilizer, tafamidis, we sought to examine the interplay of planar imaging heart-to-contralateral lung (H/CL) ratio, cardiac biomarkers, and survival probability in a contemporary cohort of patients referred for noninvasive evaluation of ATTR-CM.
This single-center retrospective cohort study included 351 consecutive patients who underwent a standardized imaging protocol with Tc-PYP scanning for the evaluation of ATTR-CM from January 1, 2018, to January 1, 2020. After the exclusion of light chain amyloidosis, patients were characterized as scan consistent with ATTR (+ATTR-CM) or scan not consistent with ATTR (-ATTR-CM) using current guidelines. Linear regression was used to examine the relationship between biomarkers and H/CL and univariate Cox proportional hazards models were used to assess the probability of transplant-free survival.
We included 318 patients in the analysis (n = 86 patients +ATTR-CM; n = 232 patients -ATTR-CM). The median follow-up time was 20.1 months. During the study period, 67% of +ATTR-CM patients received tafamidis (median treatment duration, 17 months). The median H/CL ratio was 1.58 (interquartile range, 1.40-1.75). An H/CL ratio of more than 1.6 or less than 1.6 did not seem to have an impact on survival probability in +ATTR-CM patients (P = .30; hazard ratio, 0.65; 95% confidence interval, 0.31-1.41). Cardiac biomarkers were poorly correlated with H/CL (troponin T, R = 0.024; N-terminal pro-B-type natriuretic peptide, R =0.023). The Gillmore staging system predicted survival probability in +ATTR-CM as well as in the entire cohort referred for scanning. There was a trend toward longer survival among those who were -ATTR-CM compared with +ATTR-CM (P = .051; hazard ratio, 0.64; 95% confidence interval, 0.40-1.00).
At a large referral center, the intensity of Tc-PYP uptake (H/CL ratio) has neither correlation with cardiac biomarker concentrations nor prognostic usefulness in an analysis of intermediate term outcomes in the early therapeutics era. The H/CL ratio has diagnostic value, but offers little prognostic value in patients with ATTR-CM. Established staging schema were predictive of survival in this contemporary cohort, re-emphasizing the importance of cardiac biomarkers and renal function in assessing disease severity and prognosis.
转甲状腺素蛋白淀粉样心肌病（ATTR-CM）是心力衰竭日益公认的病因。鉴于 Tc-焦磷酸盐（Tc-PYP）扫描的无创诊断扩展，以及转甲状腺素蛋白稳定剂他法米汀的临床应用，我们试图在当代接受非侵入性 ATTR-CM 评估的患者队列中检查平面成像心脏与对侧肺（H/CL）比值、心脏生物标志物和生存概率之间的相互作用。
这项单中心回顾性队列研究纳入了 351 例连续患者，他们在 2018 年 1 月 1 日至 2020 年 1 月 1 日期间接受了 Tc-PYP 扫描的标准成像方案，用于评估 ATTR-CM。排除轻链淀粉样变性后，根据现行指南，使用当前指南将患者特征描述为扫描符合 ATTR（+ATTR-CM）或扫描不符合 ATTR（-ATTR-CM）。线性回归用于检查生物标志物与 H/CL 之间的关系，单因素 Cox 比例风险模型用于评估无移植生存概率。
我们对 318 例患者进行了分析（n=86 例+ATTR-CM；n=232 例-ATTR-CM）。中位随访时间为 20.1 个月。在研究期间，67%的+ATTR-CM 患者接受了他法米汀治疗（中位治疗持续时间为 17 个月）。H/CL 比值中位数为 1.58（四分位间距 1.40-1.75）。在+ATTR-CM 患者中，H/CL 比值大于 1.6 或小于 1.6 似乎并未对生存概率产生影响（P=0.30；风险比，0.65；95%置信区间，0.31-1.41）。心脏生物标志物与 H/CL 的相关性较差（肌钙蛋白 T，R=0.024；N 末端 B 型利钠肽前体，R=0.023）。吉尔莫尔分期系统在+ATTR-CM 以及整个接受扫描的队列中均能预测生存概率。与+ATTR-CM 相比，-ATTR-CM 患者的生存时间更长（P=0.051；风险比，0.64；95%置信区间，0.40-1.00）。
在一个大型转诊中心，Tc-PYP 摄取的强度（H/CL 比值）与心脏生物标志物浓度既没有相关性，在早期治疗时代分析中期结果时也没有预后价值。H/CL 比值具有诊断价值，但在 ATTR-CM 患者中预后价值有限。既定的分期方案在这一当代队列中具有预测生存的能力，再次强调了心脏生物标志物和肾功能在评估疾病严重程度和预后方面的重要性。
