A&R：平均肺动脉压达临界值的系统性硬化症患者更易进展为肺动脉高压

来自英国伦敦皇家自由医院全国肺动脉高压中心的Christopher J. Valerio等人进行了一项研究，研究目的是探讨心脏导管检查肺动脉压（PAP）临界的系统性硬化症（SSc）患者是否比肺动脉压力正常的患者更易发展为肺动脉高压（PH）。研究结果在线发布在2013年4月的ARTHRITIS & RHEUMATISM（关节炎与风湿病）上。研究者发现，平均PAP临界，或TPG升高的SSc患者亦发展为PH。这些患者需要密切监视肺动脉PH的进展。心脏导管检查数据对怀疑有PAH风险的患者是有益的。

该项前瞻性队列研究招募SSc患者，但SSc合并PH和严重间质性肺病的患者在基线时被除外。研究者对随访数据资料进行分析，对符合预先设定标准的患者再次行心脏导管检查评估发展为PH的可能。研究者还采用乘积极限法（Kaplan-Meier法）、受试者工作曲线和Cox回归模型来分析发展为PH和死亡的情况。

研究结果如下：该研究共纳入229位患者，86位患者基线时平均PAP临界（21-24mmHg）。依据预先设定的标准，76位患者再次进行心脏导管检查，其中的29位发展为PH。2位患者存在左心疾患。随访时，平均PAP较基线时的20.2mmHg平均增长至24.3mmHg。（Student's检：P < 0.05）。平均PAP临界的患者比平均PAP≤20mmHg的患者更易发展为PH（对数秩检验：P < 0.001，风险比[HR] 3.7）。基线时跨肺梯度（TPG）≥11mmHg者也更易发展为PH（对数秩检验：P < 0.001，HR 7.9）。肺动脉高压症（PAH）在SSc患者是一种不良事件，3年内死亡率达18%。

研究数据暗示平均PAP临界，或TPG升高的SSc患者易发展为PH。这些患者需要密切监视肺动脉PH的进展。该研究揭示，心脏导管检查数据对怀疑有PAH风险的患者是有益的。

Borderline mean pulmonary artery pressure in patients with systemic sclerosis: transpulmonary gradient predicts risk of developing pulmonary hypertension.
OBJECTIVE
To determine whether patients with systemic sclerosis (SSc) and borderline mean pulmonary artery pressure (PAP) at cardiac catheterization are more likely to develop pulmonary hypertension (PH) than those in whom pulmonary pressure is normal.
METHODS
Patients with SSc in whom PH and significant interstitial lung disease had been excluded at baseline were enrolled in our prospective cohort. Analysis of followup data identified patients who met prespecified criteria prompting repeat catheterization to reassess for possible PH. Using Kaplan-Meier, receiver operating characteristic, and Cox regression methods, we studied the development of PH and death.
RESULTS
Of 228 patients in this study, 86 had borderline mean PAP (21-24 mm Hg) at baseline. Following prespecified criteria, 76 patients underwent repeat catheterization, and 29 of these developed PH. Two cases were related to disease of the left side of the heart. The average mean PAP increased from baseline (20.2 mm Hg) to followup (24.3 mm Hg) (P<0.05 by Student's t-test). Patients with borderline mean PAP were more likely to develop PH than patients with mean PAP≤20 mm Hg (P<0.001 by log rank test, hazard ratio [HR] 3.7). A transpulmonary gradient (TPG)≥11 mm Hg at baseline also predicted PH (P<0.001 by log rank test, HR 7.9). Incident development of pulmonary arterial hypertension (PAH) was not benign, with a mortality of 18% within 3 years.
CONCLUSION
Our findings indicate that borderline mean PAP and an elevated TPG in patients with SSc predict progression to PH. These patients should be monitored closely for the development of PH. Our findings indicate that catheterization data are useful in patients considered at risk of PAH.