Section of Cardiology, Dept of Medicine, University of Chicago, Chicago, IL, USA.
Eur Respir J. 2010 May;35(5):1079-87. doi: 10.1183/09031936.00072709. Epub 2009 Dec 23.
The aim of the present study was to determine contemporary survival in pulmonary arterial hypertension (PAH), and to investigate whether or not the National Institutes of Health (NIH) equation remains an accurate predictor of survival. In 576 patients with PAH referred during 1991-2007, observed survival was described using the Kaplan-Meier method. In patients with idiopathic, familial and anorexigen-associated PAH (n = 247), observed versus NIH equation predicted survival was compared. A new survival prediction equation was developed using exponential regression analysis. The observed 1-, 3- and 5-yr survival in the total cohort were 86, 69 and 61%, respectively. In patients with idiopathic, familial and anorexigen-associated PAH, the observed 1-, 3- and 5-yr survival (92, 75 and 66%, respectively) were significantly higher than the predicted survival (65, 43 and 32%, respectively). The new equation (P(t) = e(-A(x,y,z)t), where P(t) is probability of survival, t the time interval in years, A(x,y,z) = e((-1.270-0.0148x+0.0402y-0.361z)), x the mean pulmonary artery pressure, y the mean right atrial pressure and z the cardiac index) performed well when applied to published contemporary studies of survival in PAH. Contemporary survival in the PAH cohort was better than that predicted by the NIH registry equation. The NIH equation underestimated survival in idiopathic, familial and anorexigen-associated PAH. Once prospectively validated, the new equation may be used to determine prognosis.
本研究旨在确定肺动脉高压（PAH）的当代存活率，并探讨美国国立卫生研究院（NIH）方程是否仍然是存活率的准确预测指标。在 1991-2007 年间转诊的 576 例 PAH 患者中，采用 Kaplan-Meier 法描述观察生存率。在特发性、家族性和食欲抑制剂相关性 PAH 患者（n=247）中，比较观察生存率与 NIH 方程预测生存率。采用指数回归分析建立新的生存预测方程。在总队列中，观察到的 1、3 和 5 年生存率分别为 86%、69%和 61%。在特发性、家族性和食欲抑制剂相关性 PAH 患者中，观察到的 1、3 和 5 年生存率（分别为 92%、75%和 66%）显著高于预测生存率（分别为 65%、43%和 32%）。新方程（P(t) = e(-A(x,y,z)t)，其中 P(t)是生存率，t 是时间间隔（以年为单位），A(x,y,z) = e((-1.270-0.0148x+0.0402y-0.361z))，x 为平均肺动脉压，y 为平均右心房压，z 为心脏指数）在应用于 PAH 当代生存的已发表研究中表现良好。PAH 队列的当代存活率优于 NIH 登记方程预测的存活率。NIH 方程低估了特发性、家族性和食欲抑制剂相关性 PAH 的存活率。一旦前瞻性验证，新方程可用于确定预后。
