二氢嘧啶脱氢酶定量与野生型转甲状腺素蛋白心脏淀粉样变性的细胞外容积及疾病严重程度相关。
DPD Quantification Correlates With Extracellular Volume and Disease Severity in Wild-Type Transthyretin Cardiac Amyloidosis.
作者信息
Rettl René, Calabretta Raffaella, Duca Franz, Kronberger Christina, Binder Christina, Willixhofer Robin, Poledniczek Michael, Hofer Felix, Doná Carolina, Beitzke Dietrich, Loewe Christian, Nitsche Christian, Hengstenberg Christian, Badr Eslam Roza, Kastner Johannes, Bergler-Klein Jutta, Hacker Marcus, Kammerlander Andreas A
机构信息
Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria.
Division of Nuclear Medicine, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria.
出版信息
JACC Adv. 2024 Sep 12;3(10):101261. doi: 10.1016/j.jacadv.2024.101261. eCollection 2024 Oct.
BACKGROUND
The pathophysiological hallmark of wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is the deposition of amyloid within the myocardium.
OBJECTIVES
This study aimed to investigate associations between quantitative cardiac Tc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) uptake and myocardial amyloid burden, cardiac function, cardiac biomarkers, and clinical status in ATTRwt-CM.
METHODS
Forty ATTRwt-CM patients underwent quantitative DPD single photon emission computed tomography/computed tomography to determine the standardized uptake value (SUV) retention index, cardiac magnetic resonance imaging to determine extracellular volume (ECV) and cardiac function (RV-LS), and assessment of cardiac biomarkers (N-terminal prohormone of brain natriuretic peptide [NT-proBNP], troponin T) and clinical status (6-minute walk distance [6MWD], National Amyloidosis Centre [NAC] stage). ATTRwt-CM patients were divided into 2 cohorts based on median SUV retention index (low uptake: <5.19 mg/dL, n = 20; high uptake: ≥5.19 mg/dL, n = 20). Linear regression models were used to assess associations of the SUV retention index with variables of interest and the Mann-Whitney U or chi-squared test to compare variables between groups.
RESULTS
ATTRwt-CM patients (n = 40) were elderly (78.0 years) and predominantly male (75.0%). Univariable linear regression analyses revealed associations of the SUV retention index with ECV (r = 0.669, β = 0.139, < 0.001), native T1 time (r = 0.432, β = 0.020, = 0.005), RV-LS (r = 0.445, β = 0.204, = 0.004), NT-proBNP (log) (r = 0.458, β = 2.842, = 0.003), troponin T (r = 0.422, β = 0.048, = 0.007), 6MWD (r = 0.385, β = -0.007, = 0.017), and NAC stage (r = 0.490, β = 1.785, = 0.001). Cohort comparison demonstrated differences in ECV ( = 0.001), native T1 time ( = 0.013), RV-LS ( = 0.003), NT-proBNP ( < 0.001), troponin T ( = 0.046), 6MWD ( = 0.002), and NAC stage (I: < 0.001, II: = 0.030, III: = 0.021).
CONCLUSIONS
In ATTRwt-CM, quantitative cardiac DPD uptake correlates with myocardial amyloid load, longitudinal cardiac function, cardiac biomarkers, exercise capacity, and disease stage, providing a valuable tool to quantify and monitor cardiac disease burden.
背景
野生型转甲状腺素蛋白淀粉样变心肌病（ATTRwt-CM）的病理生理标志是淀粉样物质在心肌内沉积。
目的
本研究旨在探讨定量心肌Tc-3,3-二膦酸-1,2-丙二酸（DPD）摄取与ATTRwt-CM患者心肌淀粉样物质负荷、心功能、心脏生物标志物及临床状态之间的关联。
方法
40例ATTRwt-CM患者接受了定量DPD单光子发射计算机断层扫描/计算机断层扫描以测定标准化摄取值（SUV）滞留指数，心脏磁共振成像以测定细胞外容积（ECV）和心功能（右心室纵向应变[RV-LS]），并评估心脏生物标志物（脑钠肽N末端前体激素[NT-proBNP]、肌钙蛋白T）及临床状态（6分钟步行距离[6MWD]、国家淀粉样变性中心[NAC]分期）。根据SUV滞留指数中位数将ATTRwt-CM患者分为2组（低摄取组：<5.19mg/dL，n = 20；高摄取组：≥5.19mg/dL，n = 20）。采用线性回归模型评估SUV滞留指数与感兴趣变量之间的关联，并采用曼-惠特尼U检验或卡方检验比较组间变量。
结果
40例ATTRwt-CM患者年龄较大（78.0岁），男性占主导（75.0%）。单变量线性回归分析显示，SUV滞留指数与ECV（r = 0.669，β = 0.139，P < 0.001）、固有T1时间（r = 0.432，β = 0.020，P = 0.005）、RV-LS（r = 0.445，β = 0.204，P = 0.004）、NT-proBNP（log）（r = 0.458，β = 2.842，P = 0.003）、肌钙蛋白T（r = 0.422，β = 0.048，P = 0.007）、6MWD（r = 0.385，β = -0.007，P = 0.017）及NAC分期（r = 0.490，β = 1.785，P = 0.001）相关。组间比较显示，ECV（P = 0.001）、固有T1时间（P = 0.013）、RV-LS（P = 0.003）、NT-proBNP（P < 0.001）、肌钙蛋白T（P = 0.046）、6MWD（P =
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