Koeckerling David, Reddy Rohin K, Eichhorn Christian, Braun Volker, Ahmad Yousif, Howard James P, Aus dem Siepen Fabian, Meder Benjamin, Frey Norbert, Mereles Derliz
Department of Cardiology, Angiology and Pulmonology, Heidelberg University Hospital, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany.
National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW36LY, UK.
Eur Heart J Open. 2025 Aug 22;5(4):oeaf078. doi: 10.1093/ehjopen/oeaf078. eCollection 2025 Jul.
The role of echocardiography in amyloidosis prognostication remains undefined in international guidelines. This meta-analysis aims to evaluate associations between echocardiography-derived measurements and clinical outcomes in light chain (AL) and transthyretin (ATTR) amyloidosis.
MEDLINE, Embase, Cochrane Library, and Google Scholar were systematically searched through July 2024 for studies reporting associations between echocardiographic variables [left ventricular global longitudinal strain (LV-GLS), LV ejection fraction (LVEF), tricuspid annular plane systolic excursion (TAPSE), interventricular septum diameter (IVSd), LV mass index (LVMi) and /' ratios] and adverse events in AL or ATTR amyloidosis. Prespecified demographic items and clinical outcomes were extracted by two blinded, independent reviewers. The prespecified primary outcome was all-cause mortality. Random-effect models were applied to pool hazard ratios (HR). 94 studies comprising 16158 patients ( = 4788 AL, = 8241 ATTR, = 3129 mixed aetiologies) were included. Median follow-up was 22.3 (IQR, 16.9-31.4) months. Higher all-cause mortality risk (HR, 1.10: 95%CI, 1.08-1.12; < 0.001) was observed per 1% LV-GLS decrement, consistent across AL and ATTR subgroups. Lower all-cause mortality risk was seen with increasing LVEF (per 1%) and TAPSE (per 1 mm) in the overall population (HR, 0.98; 95%CI, 0.98-0.98; < 0.001; and HR, 0.94; 95%CI, 0.93-0.95; < 0.001) and in AL and ATTR subgroups. Higher /' ratios (per 1 unit) were associated with all-cause mortality (HR, 1.02; 95%CI, 1.02-1.03; < 0.001), consistent across AL and ATTR subtypes. No reliable associations between structural parameters (IVSd, LVMi) and clinical outcomes were found.
Echocardiographic measures of biventricular deformation, systolic and diastolic function, were consistently associated with mortality in amyloidosis, while structural parameters were not. Echocardiography may have an important role in the initial risk stratification of cardiac amyloidosis.
国际指南中超声心动图在淀粉样变性预后评估中的作用仍不明确。本荟萃分析旨在评估超声心动图测量值与轻链（AL）和转甲状腺素蛋白（ATTR）淀粉样变性临床结局之间的关联。
系统检索了MEDLINE、Embase、Cochrane图书馆和谷歌学术，截至2024年7月，查找报告超声心动图变量[左心室整体纵向应变（LV-GLS）、左心室射血分数（LVEF）、三尖瓣环平面收缩期位移（TAPSE）、室间隔直径（IVSd）、左心室质量指数（LVMi）和/‘比值]与AL或ATTR淀粉样变性不良事件之间关联的研究。两名盲法、独立的审阅者提取了预先指定的人口统计学项目和临床结局。预先指定的主要结局是全因死亡率。应用随机效应模型汇总风险比（HR）。纳入了94项研究，共16158例患者（=4788例AL，=8241例ATTR，=3129例混合病因）。中位随访时间为22.3（IQR，16.9 - 31.4）个月。每降低1%的LV-GLS，全因死亡风险更高（HR，1.10：95%CI，1.08 - 1.12；<0.001），在AL和ATTR亚组中均一致。在总体人群（HR，0.98；95%CI，0.98 - 0.98；<0.001；以及HR，0.94；95%CI，0.93 - 0.95；<0.001）以及AL和ATTR亚组中，随着LVEF（每增加1%）和TAPSE（每增加1 mm）升高，全因死亡风险降低。更高的/‘比值（每增加1个单位）与全因死亡率相关（HR，1.02；95%CI，1.02 - 1.03；<0.001），在AL和ATTR亚型中均一致。未发现结构参数（IVSd、LVMi）与临床结局之间存在可靠关联。
双心室变形、收缩和舒张功能的超声心动图测量与淀粉样变性的死亡率始终相关，而结构参数则不然。超声心动图可能在心脏淀粉样变性的初始风险分层中发挥重要作用。
