Department of Rheumatology, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Department of Cardiology, Oslo University Hospital, Oslo, Norway.
J Am Coll Cardiol. 2018 Oct 9;72(15):1804-1813. doi: 10.1016/j.jacc.2018.07.068.
Primary cardiac affection is common and is a major cause of death in systemic sclerosis (SSc), but there are knowledge gaps regarding the effect of cardiac dysfunction on mortality.
The purpose of this study was to evaluate diastolic function in a large, unselected SSc cohort and assess the effect of diastolic dysfunction (DD) on mortality.
SSc patients followed prospectively at the Oslo University Hospital from 2003 to 2016 with available echocardiographies and matched control subjects were included. DD was assessed by echocardiography according to the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging guidelines. Pulmonary hypertension (PH) was diagnosed by right heart catheterization. Vital status was available for all patients. Cox regression analyses with hazards ratios (HRs) were conducted.
Diastolic function was assessed in 275 SSc patients at baseline and in 186 patients at follow-up. At baseline, 46 of the 275 SSc patients (17%) were diagnosed with DD and 195 (71%) had normal diastolic function. After a median follow-up of 3.4 years (interquartile range: 1.6 to 6.2 years), the proportion of DD increased from 17% to 29%. During follow-up, 57% of patients with DD at baseline died, compared with 13% of patients with normal diastolic function. At baseline, 86 patients had performed right heart catheterization, and 43 were diagnosed with PH; of these 60% deceased. In multivariable Cox regression analyses, DD was a stronger predictor of death (HR: 3.7; 95% CI: 1.69 to 8.14; c-index 0.89) than PH (HR: 2.0; 95% CI: 1.1 to 3.9; c-index 0.84).
DD is frequent in SSc, and the presence of DD is associated with high mortality. DD exceeds PH with respect to predicting mortality.
原发性心脏疾病在系统性硬化症（SSc）中较为常见，是其主要死亡原因，但对于心脏功能障碍对死亡率的影响，我们的认识仍存在空白。
本研究旨在评估一个大型、未选择的 SSc 队列的舒张功能，并评估舒张功能障碍（DD）对死亡率的影响。
纳入了 2003 年至 2016 年在奥斯陆大学医院接受前瞻性随访且有超声心动图资料的 SSc 患者和匹配的对照者。根据 2016 年美国超声心动图学会/欧洲心血管成像协会指南评估 DD。通过右心导管检查诊断肺动脉高压（PH）。所有患者的生存状态均可用。采用风险比（HRs）的 Cox 回归分析。
275 例 SSc 患者在基线时和 186 例患者在随访时评估了舒张功能。在基线时，275 例 SSc 患者中有 46 例（17%）被诊断为 DD，195 例（71%）有正常的舒张功能。在中位数为 3.4 年（四分位间距：1.6 至 6.2 年）的随访期间，DD 的比例从 17%增加到 29%。在随访期间，基线时存在 DD 的患者中有 57%死亡，而舒张功能正常的患者中有 13%死亡。在基线时，86 例患者进行了右心导管检查，其中 43 例诊断为 PH；其中 60%死亡。在多变量 Cox 回归分析中，DD 是死亡的更强预测因素（HR：3.7；95% CI：1.69 至 8.14；C 指数 0.89），而 PH 则为（HR：2.0；95% CI：1.1 至 3.9；C 指数 0.84）。
DD 在 SSc 中很常见，存在 DD 与高死亡率相关。DD 预测死亡率的能力超过 PH。
