Li Jingyi, Lu Yang, Xu Xiqi, Tian Zhuang, Li Jian, Zhang Shuyang
Department of Cardiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Orphanet J Rare Dis. 2025 Jan 13;20(1):19. doi: 10.1186/s13023-024-03451-z.
There is no unified prognostic scoring system for light chain cardiac amyloidosis (AL-CA), particularly stage IIIb AL-CA. This study aimed to use invasive haemodynamic information to investigate markers that can more accurately evaluate the prognosis of patients with stage IIIb AL-CA.
In this retrospective cohort study, we conducted invasive haemodynamic measurements concurrently with myocardial biopsies to diagnose AL-CA. We used Cox regression analysis and time-dependent receiver operating characteristic curve analysis to study the associations between these measurements and overall mortality. Echocardiographic parameters were also recorded and analysed via logistic regression to explore their relationships with haemodynamic changes.
Although traditional haemodynamic parameters, such as the cardiac index (CI), pulmonary artery wedge pressure (PAWP), pulmonary artery pressure, and vascular resistance, did not correlate with mortality, the PAWP/CI ratio emerged as a vital prognostic marker. Patients with a PAWP/CI ratio above 11 mmHg/L/min/m had markedly poorer survival. Kaplan‒Meier analysis highlighted the prognostic significance of the ratio, revealing distinct survival differences. Furthermore, logistic regression confirmed that echocardiographically measured pulmonary artery systolic pressure independently correlated with increases in the PAWP/CI ratio.
In stage IIIb AL-CA patients, the PAWP/CI ratio, which surpasses traditional haemodynamic indicators, significantly predicts all-cause mortality, emphasizing its prognostic value. Our findings suggest that echocardiography-derived PASP could alternatively reflect the PAWP/CI ratio.
轻链型心脏淀粉样变（AL-CA），尤其是Ⅲb期AL-CA，目前尚无统一的预后评分系统。本研究旨在利用有创血流动力学信息，探究能够更准确评估Ⅲb期AL-CA患者预后的标志物。
在这项回顾性队列研究中，我们在进行心肌活检以诊断AL-CA的同时进行了有创血流动力学测量。我们使用Cox回归分析和时间依赖性受试者工作特征曲线分析，研究这些测量值与总死亡率之间的关联。还记录了超声心动图参数，并通过逻辑回归进行分析，以探讨它们与血流动力学变化的关系。
尽管传统的血流动力学参数，如心脏指数（CI）、肺动脉楔压（PAWP）、肺动脉压和血管阻力，与死亡率无关，但PAWP/CI比值成为一个重要的预后标志物。PAWP/CI比值高于11 mmHg/L/min/m的患者生存率明显较差。Kaplan-Meier分析突出了该比值的预后意义，显示出明显的生存差异。此外，逻辑回归证实，超声心动图测量的肺动脉收缩压与PAWP/CI比值的升高独立相关。
在Ⅲb期AL-CA患者中，PAWP/CI比值超越了传统的血流动力学指标，能显著预测全因死亡率，强调了其预后价值。我们的研究结果表明，超声心动图得出的肺动脉收缩压（PASP）可替代反映PAWP/CI比值。
