The objective of this study was to investigate the prognostic impact of right ventricular (RV) function at rest and during exercise in patients with systemic sclerosis (SSc) presenting for a screening for pulmonary hypertension (PH). In this study, data from SSc patients who underwent routinely performed examinations for PH screening including echocardiography and right heart catheterization at rest and during exercise were analysed. Uni- and multivariable analyses were performed to identify prognostic parameters. Out of 280 SSc patients screened for PH, 225 were included in the analysis (81.3% female, mean age 58.1±13.0 years, 68% limited cutaneous SSc, WHO-FC II–III 74%, 24 manifest PH). During the observation period of 3.2±2.7 (median 2.6) years 35 patients died. Tricuspid annular plane systolic excursion (TAPSE) at rest <18 mm (p=0.001), RV output reserve as increase of cardiac index (CI) during exercise <2 l/min (p<0.0001), RV pulmonary vascular reserve (Δ mean pulmonary artery pressure/Δ cardiac output) ≥3 mmHg/l/min (p<0.0001), peak CI <5.5 l/min/m2 (p=0.001), pulmonary arterial compliance <2 ml/mmHg (p=0.002), TAPSE/systolic pulmonary arterial pressure (sPAP) ratio ≤0.6 ml/mmHg (p<0.0001) and echocardiographic qualitative RV function at rest (p<0.0001) significantly predicted worse survival. In the multivariable analysis TAPSE/sPAP ratio and diffusion capacity for carbon monoxide ≤65% were identified as independent prognostic predictors and had 75% sensitivity and 69% specificity to predict future development of pulmonary vascular disease (PVD) during follow-up. This study demonstrates that assessment of RV function at rest and during exercise may provide crucial information to identify SSc patients who are at a high risk of poor outcome and for the development of PH and/or PVD.

中文翻译：

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系统性硬化症患者右心室功能和输出储备的预后意义

本研究的目的是调查在静息和运动期间右心室 (RV) 功能对筛查肺动脉高压 (PH) 的系统性硬化症 (SSc) 患者的预后影响。在这项研究中，分析了在休息和运动期间接受常规检查以进行 PH 筛查的 SSc 患者的数据，包括超声心动图和右心导管插入术。进行单变量和多变量分析以确定预后参数。在筛查 PH 的 280 名 SSc 患者中，225 名被纳入分析（81.3% 的女性，平均年龄 58.1±13.0 岁，68% 的局限性皮肤 SSc，WHO-FC II-III 74%，24 名明显的 PH）。在 3.2±2.7（中位数 2.6）年的观察期内，有 35 名患者死亡。静息时三尖瓣环平面收缩偏移 (TAPSE) <18 mm (p=0.001)，RV 输出储备作为运动期间心脏指数 (CI) 的增加 <2 l/min (p<0.0001)，RV 肺血管储备（Δ 平均肺动脉压/Δ 心输出量）≥3 mmHg/l/min (p<0.0001 )，峰值 CI <5.5 l/min/m2 (p=0.001)，肺动脉顺应性 <2 ml/mmHg (p=0.002)，TAPSE/肺动脉收缩压 (sPAP) 比率 ≤0.6 ml/mmHg (p<0.0001 ) 和静息时超声心动图定性 RV 功能 (p<0.0001) 显着预测较差的生存率。在多变量分析中，TAPSE/sPAP 比率和一氧化碳 ≤65% 的扩散能力被确定为独立的预后预测因子，在随访期间预测肺血管疾病 (PVD) 未来发展的敏感性为 75%，特异性为 69%。