Usuku Hiroki, Yamamoto Eiichiro, Miyazaki Kasumi, Higashi Ryudai, Nozuhara Atsushi, Oike Fumi, Kuyama Naoto, Tabata Noriaki, Ishii Masanobu, Hanatani Shinsuke, Hoshiyama Tadashi, Kanazawa Hisanori, Sueta Daisuke, Arima Yuichiro, Oda Seitaro, Kawano Hiroaki, Matsuzawa Yasushi, Izumiya Yasuhiro, Ueda Mitsuharu, Tanaka Yasuhito, Tsujita Kenichi
Department of Laboratory Medicine, Kumamoto University Hospital, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.
Eur Heart J Imaging Methods Pract. 2025 Jan 17;3(1):qyae113. doi: 10.1093/ehjimp/qyae113. eCollection 2025 Jan.
There are few data on the prognostic impact of pulmonary-right ventricular (RV) uncoupling in patients with wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM).
Among the 174 patients who were diagnosed with ATTRwt-CM at Kumamoto University Hospital from 2002 to 2021, 143 patients who met the current Japanese guideline and had sufficient information for two-dimensional speckle tracking echocardiography were retrospectively analysed. During a median follow-up of 1209 days, 39 cardiac deaths occurred. Compared with patients in the non-event group, those in the cardiac death group were significantly older (79.3 ± 6.7 vs. 76.4 ± 6.2, respectively; < 0.05). Additionally, RV global longitudinal strain (RV-GLS)/systolic pulmonary artery pressure (sPAP), an index of pulmonary-RV uncoupling, was significantly lower in patients in the cardiac death group vs. the non-event group [0.29 (0.18-0.35) vs. 0.40 (0.29-0.57), < 0.01]. Multivariate Cox proportional hazards regression analysis demonstrated that RV-GLS/sPAP was significantly associated with cardiac death after adjusting for tricuspid annular plane systolic excursion/sPAP ( < 0.01), sPAP ( < 0.05), and conventional prognostic factors including age and hospitalization for heart failure (<0.01), laboratory finding including high-sensitivity cardiac troponin T, and B-type natriuretic peptide ( < 0.01). Receiver operating characteristic analysis showed that the area under the curve for RV-GLS/sPAP for cardiac death was 0.72 and that the best cut off value for RV-GLS/sPAP was 0.34 (sensitivity, 76%; specificity, 65%). In the Kaplan-Meier analysis, patients with ATTRwt-CM who had low vs. high RV-GLS/sPAP (cut-off value 0.34) had a significantly higher probability of cardiac death ( < 0.01).
Pulmonary-RV uncoupling has significantly higher prognostic value compared with conventional prognostic factors in ATTRwt-CM.
关于野生型转甲状腺素蛋白淀粉样变心肌病(ATTRwt-CM)患者中肺-右心室(RV)失耦联对预后影响的数据较少。
在2002年至2021年于熊本大学医院被诊断为ATTRwt-CM的174例患者中,对143例符合现行日本指南且有足够二维斑点追踪超声心动图信息的患者进行回顾性分析。在中位随访1209天期间,发生了39例心源性死亡。与非事件组患者相比,心源性死亡组患者年龄显著更大(分别为79.3±6.7岁和76.4±6.2岁;P<0.05)。此外,作为肺-RV失耦联指标的RV整体纵向应变(RV-GLS)/收缩期肺动脉压(sPAP),在心源性死亡组患者中显著低于非事件组[0.29(0.18 - 0.35)对0.40(0.29 - 0.57),P<0.01]。多因素Cox比例风险回归分析表明,在调整三尖瓣环平面收缩期位移/sPAP(P<0.01)、sPAP(P<0.05)以及包括年龄和因心力衰竭住院等传统预后因素(P<0.01)、包括高敏心肌肌钙蛋白T和B型利钠肽等实验室检查结果(P<0.01)后,RV-GLS/sPAP与心源性死亡显著相关。受试者工作特征分析显示,RV-GLS/sPAP预测心源性死亡的曲线下面积为0.72,RV-GLS/sPAP的最佳截断值为0.34(敏感性为76%;特异性为65%)。在Kaplan-Meier分析中,RV-GLS/sPAP低(截断值0.34)与高的ATTRwt-CM患者心源性死亡概率显著更高(P<0.01)。
在ATTRwt-CM中,与传统预后因素相比,肺-RV失耦联具有显著更高的预后价值。