Department of Cardiology, Tokyo Women's Medical University, 8-1 Kawada-Cho, Shinjyuku-Ku, Tokyo, 162-8666, Japan.
Department of Diagnostic Imaging & Nuclear Medicine, Tokyo Women's Medical University, 8-1 Kawada-Cho, Shinjyuku-Ku, Tokyo, 162-8666, Japan.
Heart Vessels. 2024 Feb;39(2):135-143. doi: 10.1007/s00380-023-02320-2. Epub 2023 Oct 1.
Interstitial lung disease and cardiac involvement are common manifestations and prognostic factors of systemic sclerosis. However, it is unclear whether impaired right atrial function associated with interstitial lung disease in systemic sclerosis can be used as a prognostic factor in this patient population. Therefore, this study aimed to investigate the relationship between right atrial function, interstitial lung disease, and prognosis in patients with systemic sclerosis using tissue tracking analysis with cine cardiac magnetic resonance imaging. In this retrospective observational study, a total of 40 patients with systemic sclerosis were enrolled. Tissue tracking analysis was used to obtain time curves of right atrial strain. Reservoir (total strain), conduit (passive strain), and booster (active strain) pump function were calculated, and right atrial strain, interstitial lung disease, and clinical outcomes were examined. An adverse clinical event was defined as all-cause death. Overall, 23 patients had interstitial lung disease (58%). Six patients died during the follow-up (median, 44 months). The total skin score and right ventricular systolic pressure on echocardiography were higher in patients with an event than in those without an event (28 ± 16% vs. 13 ± 13%, P = 0.02; 46.3 ± 10.7 mmHg vs. 36.0 ± 8.5 mmHg, P = 0.01, respectively). Further, right atrial total strain and active strain were significantly lower in patients with an event than in those without an event (14.3 ± 11.3% vs. 25.8 ± 11.4%, P = 0.03; 3.48 ± 2.37 vs. 11.7 ± 6.78, P = 0.007, respectively). Multivariate analysis revealed that active strain was an independent predictor of all-cause death (hazard ratio 0.76, P = 0.029). Kaplan-Meier analysis revealed that the survival rate was significantly higher in patients with right atrial active strain levels above the cutoff 7.4 (P < 0.05). In systemic sclerosis, right atrial booster function was predictive of mortality. Hence, right atrial functional assessment may have incremental prognostic value for patients with systemic sclerosis, leading to better risk stratification.
特发性肺间质纤维化和心脏受累是系统性硬化症的常见表现和预后因素。然而，尚不清楚系统性硬化症中与特发性肺间质纤维化相关的右心房功能障碍是否可以作为该患者人群的预后因素。因此，本研究旨在使用心脏磁共振电影组织追踪分析研究特发性肺间质纤维化患者的右心房功能、特发性肺间质纤维化和预后之间的关系。
在这项回顾性观察性研究中，共纳入 40 名系统性硬化症患者。使用组织追踪分析获得右心房应变的时间曲线。计算储备（总应变）、传导（被动应变）和助推（主动应变）泵功能，并检查右心房应变、特发性肺间质纤维化和临床结果。不良临床事件定义为全因死亡。
总的来说，23 名患者患有特发性肺间质纤维化（58%）。6 名患者在随访期间死亡（中位时间 44 个月）。有事件的患者总皮肤评分和超声心动图右心室收缩压高于无事件的患者（28±16%对 13±13%，P=0.02；46.3±10.7mmHg 对 36.0±8.5mmHg，P=0.01）。此外，有事件的患者的右心房总应变和主动应变明显低于无事件的患者（14.3±11.3%对 25.8±11.4%，P=0.03；3.48±2.37 对 11.7±6.78，P=0.007）。多变量分析显示，主动应变是全因死亡的独立预测因子（危险比 0.76，P=0.029）。Kaplan-Meier 分析显示，右心房主动应变水平高于截点 7.4 的患者生存率显著提高（P<0.05）。
在系统性硬化症中，右心房助推功能可预测死亡率。因此，右心房功能评估可能对系统性硬化症患者具有额外的预后价值，从而实现更好的风险分层。
