Rettl René, Duca Franz, Kronberger Christina, Binder Christina, Willixhofer Robin, Ermolaev Nikita, Poledniczek Michael, Hofer Felix, Nitsche Christian, Hengstenberg Christian, Eslam Roza Badr, Kastner Johannes, Bergler-Klein Jutta, Hacker Marcus, Calabretta Raffaella, Kammerlander Andreas A
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, Waehringer Guertel 18-20, 1090 Vienna, Austria.
Eur Heart J Cardiovasc Imaging. 2025 Jan 31;26(2):251-260. doi: 10.1093/ehjci/jeae295.
Quantification of cardiac [99mTc]-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) uptake enhances diagnostic capabilities and may facilitate prognostic stratification in patients with transthyretin cardiac amyloidosis (ATTR-CA). This study aimed to evaluate the association of quantitative left ventricular (LV) DPD uptake with myocardial structure and function, and their implications on outcome in ATTR-CA.
Consecutive ATTR-CA patients (n = 100) undergoing planar DPD scintigraphy with Perugini grade 2 or 3, alongside quantitative DPD single-photon emission computed tomography/computed tomography imaging and speckle-tracking echocardiography between 2019 and 2023, were included and divided into two cohorts based on median DPD retention index (low DPD uptake: ≤5.4, n = 50; high DPD uptake: >5.4, n = 50). The DPD retention index showed significant, albeit weak to modest, correlations with LV global longitudinal strain (LV-GLS: r = 0.366, P < 0.001), right ventricular free wall longitudinal strain (RV-FW-LS: r = 0.316, P = 0.002), LV diastolic function (E/e' average: r = 0.304, P = 0.013), NT-proBNP (r = 0.332, P < 0.001), troponin T (r = 0.233, P = 0.022), 6 min walk distance (6MWD: r = -0.222, P = 0.033), and National Amyloidosis Centre (NAC) stage (r = 0.294, P = 0.003). ATTR-CA patients in the high DPD uptake cohort demonstrated more advanced disease severity regarding longitudinal cardiac function (LV-GLS: P = 0.012, RV-FW-LS: P = 0.036), LV diastolic function (E/e' average: P = 0.035), cardiac biomarkers (NT-proBNP: P = 0.012, troponin T: P = 0.044), exercise capacity (6MWD: P = 0.035), and disease stage (NAC stage I: P = 0.045, III: P = 0.006), and experienced adverse outcomes compared with the low DPD uptake cohort [composite endpoint: all-cause death or heart failure hospitalization, HR: 2.873 (95% CI: 1.439-5.737), P = 0.003; DPD retention index: adjusted HR 1.221 (95% CI: 1.078-1.383), P = 0.002].
In ATTR-CA, enhanced quantitative LV DPD uptake indicates advanced disease severity and is associated with adverse outcome. DPD quantification may facilitate prognostic stratification when diagnosing patients with ATTR-CA.
量化心脏[99mTc]-3,3-二膦酰基-1,2-丙二羧酸（DPD）摄取可提高诊断能力，并可能有助于转甲状腺素蛋白心脏淀粉样变性（ATTR-CA）患者的预后分层。本研究旨在评估左心室（LV）DPD摄取定量与心肌结构和功能的相关性及其对ATTR-CA患者预后的影响。
纳入2019年至2023年间连续接受平面DPD闪烁扫描且Perugini分级为2级或3级的ATTR-CA患者（n = 100），同时进行定量DPD单光子发射计算机断层扫描/计算机断层扫描成像和斑点追踪超声心动图检查，并根据DPD保留指数中位数将其分为两个队列（低DPD摄取：≤5.4，n = 50；高DPD摄取：>5.4，n = 50）。DPD保留指数与左心室整体纵向应变（LV-GLS：r = 0.366，P < 0.001）、右心室游离壁纵向应变（RV-FW-LS：r = 0.316，P = 0.002）、左心室舒张功能（E/e'平均值：r = 0.304，P = 0.013）、N末端脑钠肽前体（NT-proBNP：r = 0.332，P < 0.001）、肌钙蛋白T（r = 0.233，P = 0.022）、6分钟步行距离（6MWD：r = -0.222，P = 0.033）和国家淀粉样变性中心（NAC）分期（r = 0.294，P = 0.003）均存在显著相关性，尽管相关性较弱至中等。高DPD摄取队列中的ATTR-CA患者在心脏纵向功能（LV-GLS：P = 0.012，RV-FW-LS：P = 0.036）、左心室舒张功能（E/e'平均值：P = 0.035）、心脏生物标志物（NT-proBNP：P = 0.012，肌钙蛋白T：P = 0.044）、运动能力（6MWD：P = 0.035）和疾病分期（NAC I期：P = 0.045，III期：P = 0.006）方面显示出更严重的疾病程度，与低DPD摄取队列相比，不良结局发生率更高[复合终点：全因死亡或心力衰竭住院，HR：2.873（95%CI：1.439 - 5.737），P = 0.003；DPD保留指数：校正后HR 1.221（95%CI：1.078 - 1.383），P = 0.002]。
在ATTR-CA中，左心室DPD摄取定量增加表明疾病严重程度较高，且与不良结局相关。DPD定量在诊断ATTR-CA患者时可能有助于预后分层。
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