Department of Internal Medicine, University of California, Los Angeles, California, USA.
Ann Rheum Dis. 2012 Aug;71(8):1335-42. doi: 10.1136/annrheumdis-2011-200546. Epub 2012 Feb 2.
Patients with normal (mean pulmonary arterial pressure (mPAP) ≤20 mm Hg) and borderline mean pulmonary pressures (21-24 mm Hg) are "at risk" of developing pulmonary hypertension (PH). The objectives of this analysis were to examine the baseline characteristics in systemic sclerosis (SSc) with normal and borderline mPAP and to explore long-term outcomes in SSc patients with borderline mPAP versus normal haemodynamics.
PHAROS is a multicentre prospective longitudinal cohort of patients with SSc "at risk" or recently diagnosed with resting PH on right heart catheterisation (RHC). Baseline clinical characteristics, pulmonary function tests, high-resolution CT, 2-dimensional echocardiogram and RHC results were analysed in normal and borderline mPAP groups.
206 patients underwent RHC (results showed 35 normal, 28 borderline mPAP, 143 resting PH). There were no differences in the baseline demographics. Patients in the borderline mPAP group were more likely to have restrictive lung disease (67% vs 30%), fibrosis on high-resolution CT and a higher estimated right ventricular systolic pressure on echocardiogram (46.3 vs 36.2 mm Hg; p<0.05) than patients with normal haemodynamics. RHC revealed higher pulmonary vascular resistance and more elevated mPAP on exercise (≥30; 88% vs 56%) in the borderline mPAP group (p<0.05 for both). Patients were followed for a mean of 25.7 months and 24 patients had a repeat RHC during this period. During follow-up, 55% of the borderline mPAP group and 32% of the normal group developed resting PH (p=NS).
Patients with borderline mPAP have a greater prevalence of abnormal lung physiology, pulmonary fibrosis and the presence of exercise mPAP ≥30 mm Hg.
平均肺动脉压（mPAP）正常（≤20mmHg）和边界平均肺动脉压（21-24mmHg）的患者存在发展为肺动脉高压（PH）的“风险”。本分析的目的是检查特发性肺动脉高压（PH）患者的基线特征，并探讨边界平均肺动脉压与正常血流动力学患者的长期结局。
PHAROS 是一项多中心前瞻性纵向队列研究，纳入了在右心导管检查（RHC）中被诊断为静息 PH 的特发性肺动脉高压（PH）“风险”或近期诊断为特发性肺动脉高压（PH）的患者。分析了正常和边界 mPAP 组的基线临床特征、肺功能检查、高分辨率 CT、二维超声心动图和 RHC 结果。
206 例患者接受了 RHC（结果显示 35 例正常，28 例边界 mPAP，143 例静息 PH）。两组患者的基线人口统计学特征无差异。边界 mPAP 组患者更可能患有限制性肺疾病（67% vs 30%）、高分辨率 CT 纤维化和超声心动图估计右心室收缩压较高（46.3 vs 36.2mmHg；p<0.05）。边界 mPAP 组在运动时 RHC 显示更高的肺血管阻力和更高的 mPAP（≥30mmHg；88% vs 56%；p<0.05）。患者平均随访 25.7 个月，在此期间 24 例患者重复接受了 RHC。随访期间，边界 mPAP 组 55%和正常组 32%的患者发展为静息 PH（p=NS）。
边界 mPAP 患者更常见异常肺生理、肺纤维化和运动时 mPAP≥30mmHg。
