Tennøe Anders H, Murbræch Klaus, Andreassen Johanna C, Fretheim Håvard, Midtvedt Øyvind, Garen Torhild, Dalen Håvard, Gude Einar, Andreassen Arne, Aakhus Svend, Molberg Øyvind, Hoffmann-Vold Anna-Maria
Oslo University Hospital, Oslo, Norway and University of Oslo Oslo Norway.
Oslo University Hospital Oslo Norway.
ACR Open Rheumatol. 2019 May 31;1(4):258-266. doi: 10.1002/acr2.1037. eCollection 2019 Jun.
Primary cardiac involvement is presumed to account for a substantial part of disease-related mortality in systemic sclerosis (SSc). Still, there are knowledge gaps on the evolution and total burden of systolic dysfunction in SSc. Here we evaluated prospective left ventricular (LV) and right ventricular (RV) systolic function in an unselected SSc cohort and assessed the burden of systolic dysfunction on mortality.
From the Oslo University Hospital cohort, 277 SSc patients were included from 2003-2016 and compared with healthy controls. Serial echocardiographies were reevaluated in order to detect change in systolic function. Right heart catheterization was performed on patients suspected of pulmonary hypertension. Descriptive and regression analyses were conducted.
At baseline, LV systolic dysfunction by ejection fraction less than 50%, or a global longitudinal strain greater than -17.0%, was found in 12% and 24%, respectively. RV systolic dysfunction measured by tricuspid annular plane systolic excursion (TAPSE) less than 17 mm was evident in 10%. Follow-up echocardiography was performed after a median of 3.3 years (interquartile range [IQR] 1.5-5.6). At follow-up, LV systolic function remained stable, whereas RV function evaluated by TAPSE deteriorated (mean 23.1 to 21.7 mm, = 0.005) equaling a 15% prevalence of RV systolic dysfunction. RV systolic function predicted mortality in multivariable models (hazard ratio 0.41, 95% confidence interval [CI] 0.19-0.90, value 0.027), whereas LV systolic function lost predictive significance when adjusted for TAPSE.
In this unselected and prospective study, systolic dysfunction of the LV and RV was a frequent complication of SSc. LV systolic function remained stable across the observation period, whereas RV function deteriorated and predicted mortality.
原发性心脏受累被认为是系统性硬化症（SSc）相关疾病死亡率的重要组成部分。然而，关于SSc患者收缩功能障碍的演变过程和总体负担仍存在知识空白。在此，我们评估了一个未经选择的SSc队列中左心室（LV）和右心室（RV）的前瞻性收缩功能，并评估了收缩功能障碍对死亡率的影响。
从奥斯陆大学医院队列中，纳入了2003年至2016年期间的277例SSc患者，并与健康对照进行比较。对系列超声心动图进行重新评估，以检测收缩功能的变化。对疑似肺动脉高压的患者进行右心导管检查。进行描述性和回归分析。
在基线时，射血分数低于50%或整体纵向应变大于-17.0%的LV收缩功能障碍分别在12%和24%的患者中被发现。通过三尖瓣环平面收缩期位移（TAPSE）小于17mm测量的RV收缩功能障碍在10%的患者中明显。中位随访3.3年（四分位间距[IQR]1.5 - 5.6）后进行了随访超声心动图检查。随访时，LV收缩功能保持稳定，而通过TAPSE评估的RV功能恶化（平均从23.1mm降至21.7mm，P = 0.005），相当于RV收缩功能障碍的患病率为15%。在多变量模型中，RV收缩功能可预测死亡率（风险比0.41，95%置信区间[CI]0.19 - 0.90，P值0.027），而在调整TAPSE后，LV收缩功能失去了预测意义。
在这项未经选择的前瞻性研究中，LV和RV的收缩功能障碍是SSc的常见并发症。在观察期内，LV收缩功能保持稳定，而RV功能恶化并可预测死亡率。
