From the Department of Cardiology, Heart Lung Center, and the Department of Rheumatology, Leiden University Medical Center, Leiden, the Netherlands.
S.E. van Wijngaarden, MD, MSc, Department of Cardiology, Leiden University Medical Center; S. Ben Said-Bouyeri, MD, MSc, Department of Rheumatology, Leiden University Medical Center; M.K. Ninaber, MD, PhD, Department of Cardiology, Heart Lung Centre, Leiden University Medical Center; T.W. Huizinga, MD, PhD, Department of Cardiology, Heart Lung Centre, Leiden University Medical Center; M.J. Schalij, MD, PhD, Department of Cardiology, Leiden University Medical Center; J.J. Bax, MD, PhD, Department of Cardiology, Leiden University Medical Center; V. Delgado, MD, PhD, Department of Cardiology, Leiden University Medical Center; J.K. de Vries-Bouwstra, MD, PhD, Department of Rheumatology, Leiden University Medical Center; N. Ajmone Marsan, MD, PhD, Department of Cardiology, Leiden University Medical Center.
J Rheumatol. 2019 Apr;46(4):405-415. doi: 10.3899/jrheum.171207. Epub 2019 Mar 1.
Cardiac involvement is a main cause of mortality in systemic sclerosis (SSc). Its detection remains challenging using conventional echocardiography and little is known about its potential progression. This study assessed changes in cardiac performance over time in a prospective cohort of patients with SSc, including echocardiographic speckle-tracking strain analysis.
The study included 234 patients with SSc [196 women, age 52 ± 14 yrs, 165 limited SSc, time since diagnosis 5.2 yrs, interquartile range (IQR) 2.9-11.3]. Clinical variables, laboratory tests, pulmonary function tests, and echocardiographic measures were recorded at baseline and followup (median 2.3 yrs, IQR 1.3-3.9). Additionally, left ventricular (LV) systolic function was assessed with global longitudinal strain (GLS) by echocardiographic speckle-tracking analysis.
At followup, GLS had significantly worsened (-21% ± 2 vs -19% ± 2, p < 0.001) while LV ejection fraction had not changed (62% ± 7 vs 61% ± 8, p = 0.124). In particular, 39 patients showed a significant deterioration of GLS as defined by a ≥ 15% decrease, which was accompanied by a concomitant worsening of proximal muscle weakness, lung fibrosis, renal function, LV diastolic function, and right ventricular systolic function. Baseline variables associated with ≥ 15% deterioration in GLS were proximal muscle weakness (OR 3.437, 95% CI 1.13-10.43, p = 0.020), decreased DLCO (OR 3.621, 95% CI 1.25-10.51, p = 0.049), and LV diastolic dysfunction (OR 2.378, 95% CI 1.07-5.27, p = 0.033).
In patients with SSc, progression of LV systolic dysfunction was demonstrated by GLS but not by LV ejection fraction. Proximal muscle weakness, DLCO, and LV diastolic dysfunction may identify patients at risk for progressive LV systolic dysfunction and in need of closer cardiac monitoring.
心脏受累是系统性硬化症（SSc）患者死亡的主要原因。使用常规超声心动图检测仍然具有挑战性，并且对于其潜在进展知之甚少。本研究评估了前瞻性 SSc 患者队列中心脏功能随时间的变化，包括超声心动图斑点追踪应变分析。
该研究纳入了 234 例 SSc 患者[196 名女性，年龄 52±14 岁，165 例局限型 SSc，诊断后时间为 5.2 年，四分位距（IQR）为 2.9-11.3]。基线和随访时（中位数 2.3 年，IQR 1.3-3.9）记录临床变量、实验室检查、肺功能检查和超声心动图指标。此外，通过超声心动图斑点追踪分析评估左心室（LV）收缩功能的整体纵向应变（GLS）。
随访时，GLS 明显恶化（-21%±2 比-19%±2，p<0.001），而 LV 射血分数无变化（62%±7 比 61%±8，p=0.124）。特别是，39 例患者的 GLS 显著恶化（定义为下降≥15%），同时伴有近端肌肉无力、肺纤维化、肾功能、LV 舒张功能和右心室收缩功能的恶化。与 GLS 恶化≥15%相关的基线变量为近端肌肉无力（OR 3.437，95%CI 1.13-10.43，p=0.020）、DLCO 降低（OR 3.621，95%CI 1.25-10.51，p=0.049）和 LV 舒张功能障碍（OR 2.378，95%CI 1.07-5.27，p=0.033）。
在 SSc 患者中，GLS 显示 LV 收缩功能进行性恶化，但 LV 射血分数未显示。近端肌肉无力、DLCO 和 LV 舒张功能障碍可能识别出 LV 收缩功能进行性恶化风险较高、需要更密切心脏监测的患者。
