Codullo Veronica, Caporali Roberto, Cuomo Giovanna, Ghio Stefano, D'Alto Michele, Fusetti Chiara, Borgogno Elena, Montecucco Carlomaurizio, Valentini Gabriele
IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
Arthritis Rheum. 2013 Sep;65(9):2403-11. doi: 10.1002/art.38043.
Patients with systemic sclerosis (SSc) in whom pulmonary hypertension (PH) is not suspected have been reported to develop an inappropriate increase of pulmonary artery systolic pressure as estimated by Doppler echocardiography under conditions of exercise (pulmonary artery systolic pressure under exercise). We undertook this study to investigate whether this increase or any other parameter detectable by stress Doppler echocardiography has utility in predicting the development of PH in SSc.
We enrolled a total of 170 patients with SSc previously investigated using standard and stress Doppler echocardiography and tissue Doppler imaging. Each patient was evaluated at baseline and yearly for skin and internal organ involvement. Right-sided heart catheterization was carried out when PH was suspected. The baseline Cochin Risk Prediction Score was calculated retrospectively.
During followup, 6 patients (3.5%) developed PH. Compared with patients without any feature suggesting PH, the Cochin Risk Prediction Score was higher in this group (mean ± SD 4.2 ± 0.9 versus 3.4 ± 0.9; P < 0.05), as was the difference between pulmonary artery systolic pressure under exercise and pulmonary artery systolic pressure (Δpulmonary artery systolic pressure) (18.2 ± 7 mm Hg versus 9.4 ± 6.5 mm Hg; P < 0.001), even when adjusted for cardiac index changes. In multivariate analysis, Δpulmonary artery systolic pressure (hazard ratio [HR] 3.4 [95% confidence interval 1.4-8], P < 0.01) and Cochin Risk Prediction Score within the fifth quintile of the values registered in our series (HR 9.3 [95% confidence interval 1.4-63.7], P < 0.05) were the only factors independently predictive of PH during followup. A Δpulmonary artery systolic pressure cutoff of >18 mm Hg, identified by receiver operating characteristic curve analysis, had a sensitivity of 50% and a specificity of 90% for the development of PH during followup.
An inappropriate response to exercise among patients with SSC is detectable by stress Doppler echocardiography. Independently of other clinical associations, increased Δpulmonary artery systolic pressure heralds PH. Stress Doppler echocardiography may represent an additional screening tool for this severe complication.
据报道，未怀疑患有肺动脉高压（PH）的系统性硬化症（SSc）患者在运动状态下经多普勒超声心动图评估会出现肺动脉收缩压不适当升高（运动时肺动脉收缩压）。我们开展本研究以调查这种升高或应激多普勒超声心动图可检测到的任何其他参数是否有助于预测SSc患者PH的发生。
我们共纳入了170例先前使用标准和应激多普勒超声心动图及组织多普勒成像进行过检查的SSc患者。每位患者在基线时及每年评估皮肤和内脏器官受累情况。怀疑有PH时进行右心导管检查。回顾性计算基线科钦风险预测评分。
在随访期间，6例患者（3.5%）发生了PH。与无任何提示PH特征的患者相比，该组的科钦风险预测评分更高（均值±标准差 4.2±0.9 对 3.4±0.9；P<0.05），运动时肺动脉收缩压与肺动脉收缩压之差（Δ肺动脉收缩压）也更高（18.2±7 mmHg对9.4±6.5 mmHg；P<0.001），即使校正了心脏指数变化。在多变量分析中，Δ肺动脉收缩压（风险比[HR] 3.4 [95%置信区间 1.4 - 8]，P<0.01）和处于我们系列中登记值第五分位数的科钦风险预测评分（HR 9.3 [95%置信区间 1.4 - 63.7]，P<0.05）是随访期间独立预测PH的唯一因素。通过受试者工作特征曲线分析确定的Δ肺动脉收缩压截断值>18 mmHg对随访期间PH发生的敏感性为50%，特异性为90%。
应激多普勒超声心动图可检测到SSc患者对运动的不适当反应。独立于其他临床关联因素，Δ肺动脉收缩压升高预示着PH。应激多普勒超声心动图可能是这种严重并发症的一种额外筛查工具。
