Istratoaie Sabina, Bourg Corentin, Lee K Charlotte, Marut Benjamin, Antonelli Jerome, L'official Guillaume, Wazzan Adrien Al, Donal Erwan
CHU Rennes, Inserm, LTSI-UMR 1099, University of Rennes, 2 Rue Henri le Guilloux, Rennes F-35000, France.
Department of Pharmacology, Toxicology, and Clinical Pharmacology, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Eur Heart J Cardiovasc Imaging. 2025 Jan 31;26(2):239-248. doi: 10.1093/ehjci/jeae242.
The diagnosis of transthyretin amyloidosis (ATTR) significantly impacts the management and prognosis of patients initially presenting with heart failure (HF). Despite recent advancements in treatment, prognosticating ATTR remains challenging. In this study, we aim to assess echocardiographic parameters associated with mid-term prognosis in patients with wild-type ATTR using a biomarker staging system as a reference point.
We studied 182 consecutive patients with wild-type ATTR (91% male and median age 82 years) who were referred to our centre between 2016 and 2022. Using N-terminal pro-B-type natriuretic peptide and estimated glomerular filtration rate cut-offs, we classified patients into the following three stages: Stage I (101 patients, 55.5%), Stage II (53 patients, 29.0%), and Stage III disease (28 patients, 15.5%). We then compared traditional echocardiographic indices and markers of subclinical ventricular dysfunction [left ventricular (LV) global longitudinal strain, right ventricular (RV) free wall strain, and left atrial (LA) strain] among groups. Over a fixed follow-up period of 18 months, which included treatment with tafamidis 61 mg daily, 48 patients (26.4%) experienced the composite outcome of death or HF hospitalization. When compared with Stage I ATTR, the hazard ratio (HR) for death or hospitalization was 1.55 [95% confidence interval (CI) 0.62-3.86] for Stage II ATTR and 4.53 (95% CI 1.66-12.4, P = 0.0116) for Stage III ATTR. Among echocardiographic parameters, reduced RV free wall strrain (FWS) was independently associated with all-cause mortality or HF hospitalization after adjustment for the staging system (HR 2.03, 95% CI 1.07-3.85, P < 0.05).
RV FWS should be routinely assessed for all patients with ATTR. It is an independent predictor of poor prognosis and provides additional value beyond biomarker staging systems.
转甲状腺素蛋白淀粉样变性（ATTR）的诊断对最初表现为心力衰竭（HF）患者的管理和预后有重大影响。尽管近期治疗取得了进展，但对ATTR进行预后评估仍具有挑战性。在本研究中，我们旨在以生物标志物分期系统为参考点，评估野生型ATTR患者中期预后相关的超声心动图参数。
我们研究了2016年至2022年间转诊至我们中心的182例连续的野生型ATTR患者（男性占91%，中位年龄82岁）。利用N末端B型利钠肽原和估计肾小球滤过率的临界值，我们将患者分为以下三个阶段：I期（101例患者，55.5%）、II期（53例患者，29.0%）和III期疾病（28例患者，15.5%）。然后我们比较了各阶段传统超声心动图指标和亚临床心室功能障碍标志物[左心室（LV）整体纵向应变、右心室（RV）游离壁应变和左心房（LA）应变]。在为期18个月的固定随访期内，包括每日服用61毫克他氟米特进行治疗，48例患者（26.4%）经历了死亡或HF住院的复合结局。与I期ATTR相比，II期ATTR死亡或住院的风险比（HR）为1.55[95%置信区间（CI）0.62 - 3.86]，III期ATTR为4.53（95% CI 1.66 - 12.4，P = 0.0116）。在超声心动图参数中，校正分期系统后，右心室游离壁应变（FWS）降低与全因死亡率或HF住院独立相关（HR 2.03，95% CI 1.07 - 3.85，P < 0.05）。
对于所有ATTR患者，应常规评估右心室FWS。它是预后不良的独立预测因素，并且在生物标志物分期系统之外提供了额外价值。
