Sinigiani Giulio, De Michieli Laura, d'Addazio Matteo, Portalone Lisa, De Gaspari Monica, Lupi Alessandro, Zorzi Alessandro, Tona Francesco, Basso Cristina, Perazzolo Marra Martina, Iliceto Sabino, Corrado Domenico, Nistri Stefano, Mele Donato, Cipriani Alberto
Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua, Padua, Italy.
Cardiology Unit, University Hospital of Padua, Via N. Giustiniani 2, 35121, Padua, Italy.
Int J Cardiovasc Imaging. 2025 Apr 10. doi: 10.1007/s10554-025-03394-x.
Non-invasive right ventricular to pulmonary artery (RV-PA) uncoupling assessment has prognostic value in patients with heart failure (HF). Little is known about its application in patients with wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM). This single-centre retrospective study included consecutive patients with wtATTR-CM diagnosis undergoing 2D echocardiogram. RV-PA uncoupling was evaluated with the ratios between tricuspid annular plane systolic excursion (TAPSE), RV free wall longitudinal strain (RVFWLS) or RV four-chamber longitudinal strain (RV4CLS) and pulmonary artery systolic pressure (sPAP). Primary endpoint was the composite of all-cause mortality and HF hospitalisation. Overall, 100 patients (91% males, median age 81 years, 85% in National Amyloid Centre (NAC) stage ≤ 2, 18% in NAC stage Ia and 82% in New York Heart Association class ≤ II) were enrolled. Over a 16-months follow up (Q1-Q3:12-24), the primary endpoint occurred in 37 patients (37%). TAPSE/sPAP (HR 0.04, 95% CI 0.01-0.24, p < 0.001), RVFWLS/sPAP (HR 0.07, 95% CI 0.01-0.41, p = 0.003) and RV4CLS/sPAP (HR 0.06, 95% CI 0.01-0.53, p = 0.011) emerged as independent predictors of the primary endpoint and showed incremental risk prediction compared with TAPSE, RVFWLS and RV4CLS, considered as separate parameters. No differences in outcome risk prediction were observed among TAPSE/sPAP, RVFWLS/sPAP and RV4CLS/sPAP (p > 0.05). RV-PA uncoupling, as assessed by different echocardiography modalities, is an early predictor of poor outcome in patients with wtATTR-CM.
非侵入性右心室至肺动脉（RV-PA）解偶联评估对心力衰竭（HF）患者具有预后价值。关于其在野生型转甲状腺素蛋白淀粉样心肌病（wtATTR-CM）患者中的应用知之甚少。这项单中心回顾性研究纳入了连续接受二维超声心动图检查且诊断为wtATTR-CM的患者。通过三尖瓣环平面收缩期位移（TAPSE）、右心室游离壁纵向应变（RVFWLS）或右心室四腔纵向应变（RV4CLS）与肺动脉收缩压（sPAP）的比值评估RV-PA解偶联。主要终点是全因死亡率和HF住院的复合终点。总体而言，共纳入100例患者（91%为男性，中位年龄81岁，85%处于国家淀粉样蛋白中心（NAC）分期≤2期，18%处于NAC Ia期，82%处于纽约心脏协会心功能分级≤II级）。在16个月的随访期间（第一季度至第三季度：12 - 24个月），37例患者（37%）发生了主要终点事件。TAPSE/sPAP（HR 0.04，95%CI 0.01 - 0.24，p < 0.001）、RVFWLS/sPAP（HR 0.07，95%CI 0.01 - 0.41，p = 0.003）和RV4CLS/sPAP（HR 0.06，95%CI 0.01 - 0.53，p = 0.011）成为主要终点的独立预测因素，并且与单独作为参数考虑的TAPSE、RVFWLS和RV4CLS相比，显示出递增的风险预测能力。在TAPSE/sPAP、RVFWLS/sPAP和RV4CLS/sPAP之间未观察到结局风险预测的差异（p > 0.05）。通过不同超声心动图模式评估的RV-PA解偶联是wtATTR-CM患者不良结局的早期预测指标。
