Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis
Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis
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DOI：
10.1378/chest.129.3.746
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发表时间：
2006-03-01
期刊：
影响因子：
9.6
通讯作者：
Shorr, AF
中科院分区：
文献类型：
--
作者：
Lettieri, CJ;Nathan, SD;Shorr, AF
研究目的：肺动脉高压(PAH)可并发多种间质性肺疾病，包括特发性肺纤维化(IPF)。我们试图确定晚期IPF患者PAH的患病率及其对生存的影响。设计：连续接受移植前右心插管的IPF患者的回顾性分析。地点：肺移植和IPF转诊中心。方法：PAH定义为25 mm Hg的平均肺动脉压(MPAP)。我们比较了有PAH和无PAH患者的人口统计学、肺活量、6分钟步行试验(6MWT)结果和生存结果。测量和结果：79名患者纳入研究。31.6%的患者出现PAH(平均[+/-SD]mPAP，29.5+/-3.3对19.1+/-3.7 mm Hg)。肺高压患者的肺一氧化碳平均弥散量较低(分别为37.6%±11.3%和31.1%/-10.1%；P=0.04)，且更有可能需要补充氧气(分别为66.7%和17.6%；p<；0.0001)。平均步行距离(143.5+/-65.5vs 365.9+/-81.8m；p<；0.001)和平均脉搏血氧饱和度最低值(分别为80.10+/-3.7%vs88.0+/-3.5%；p<；0.001)也较低。在研究期间，PAH与更高的死亡风险相关(死亡率，分别为60.0%和29.9%；优势比，2.6；95%可信区间[CI]，2.3到3.1；p=0.001)。肺动脉高压者的一年死亡率较高(28.0%比5.5%；P=0.002)。PAH作为预测死亡率的指标，其敏感性、特异性和准确性分别为57.1%、79.3%和73.4%。压力越高，死亡风险越大，mPAP与预后呈线性相关(风险比，1.09；95%CI，1.02至1.16)。FVC和DLCO不能预测预后。结论：PAH在晚期IPF患者中很常见，对生存有显著影响。DLCO降低、补充氧量或6分钟步行能力差应引起对潜在PAH存在的怀疑。确定PAH可能是监测疾病进展、对移植进行分类和指导治疗的重要辅助手段。
Study objectives: The development of pulmonary arterial hypertension (PAH) can complicate many interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF). We sought to characterize the prevalence of PAH and its impact on survival in patients with advanced IPF.Design: Retrospective analysis of consecutive IPF patients undergoing pretransplantation right heart catheterization.Setting: Lung transplant and IPF referral center.Methods: PAH was defined as a mean pulmonary artery pressure (mPAP) of > 25 mm Hg. We compared demographic, spirometric, 6-min walk test (6MWT) results, and survival outcomes between those with PAH and those without PAH.Measurements and results: Seventy-nine patients were included in the study. PAH was present in 31.6% of patients (mean [+/- SD] mPAP, 29.5 +/- 3.3 vs 19.1 +/- 3.7 mm Hg, respectively). Those patients with PAH had a lower mean diffusing capacity of the lung for carbon monoxide (DLCO) (37.6 +/- 11.3% vs 31.1 +/- 10.1%, respectively; p = 0.04) and were more likely to require supplemental oxygen (66.7% vs 17.6%, respectively; p < 0.0001). Mean distance walked (143.5 +/- 65.5 vs 365.9 +/- 81.8 m, respectively; p < 0.001) and mean pulse oximetric saturation nadir (80.1 +/- 3.7% vs 88.0 +/- 3.5%, respectively; p < 0.001) during the 6MWT were also lower among those with PAH. PAH was associated with a greater risk of death during the study period (mortality rate, 60.0% vs 29.9%, respectively; odds ratio, 2.6; 95% confidence interval [CI], 2.3 to 3.1; p = 0.001). One-year mortality rates were higher in those with PAH (28.0% vs 5.5%, respectively; p = 0.002). As a predictor of mortality, PAH had a sensitivity, specificity, and accuracy of 57.1%, 79.3%, and 73.4%, respectively. There was a linear correlation between mPAP and outcomes with higher pressures associated with a greater risk of mortality (hazard ratio, 1.09; 95% CI, 1.02 to 1.16). FVC and DLCO did not predict outcomes.Conclusions: PAH is common in advanced cases of IPF and significantly impacts survival. A reduced DLCO, supplemental oxygen requirement, or poor 6-min walk performance should raise suspicion of the presence of underlying PAH. Identifying PAH might be an important adjunct in monitoring disease progression, triaging for transplantation, and guiding therapy.