Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension
Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension
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DOI：
10.1183/09031936.00057010
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发表时间：
2010-09-01
影响因子：
24.3
通讯作者：
Simonneau, G.
中科院分区：
文献类型：
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作者：
Humbert, M.;Sitbon, O.;Simonneau, G.
肺动脉高压（PAH）是一种进行性、致死性疾病，我们研究了674例连续的成人PAH患者，这些患者前瞻性地纳入法国PAH登记研究（121例新发病例和553例流行病例）。进行了两次生存分析。首先，对674例患者的队列在研究进入后进行了3年的随访，并描述了生存率。然后，我们集中研究了56例原发性、家族性和与肺动脉高压相关的PAH患者，以及134例在研究开始前3年确诊的PAH患者，在674例患者中，1年、2年和3年生存率为87%。（95% CI 84-90）、76%（95% CI 73-80）和67%（95% CI 63-71）。在流行的特发性、家族性和阿托伐他汀相关PAH中，1年、2年和3年生存率高于偶发患者（p=0.037）。多变量分析显示，在特发性、家族性和多西他赛相关PAH患者的联合队列中，可以通过使用患者性别、6分钟步行距离和诊断时的心输出量的新型风险预测方程来估计生存率。特发性、家族性和多西他赛相关PAH的生存率可通过使用患者诊断时特征的新型风险预测方程进行表征。
Pulmonary arterial hypertension (PAH) is a progressive, fatal disease.We studied 674 consecutive adult patients who were prospectively enrolled in the French PAH registry (121 incident and 553 prevalent cases). Two survival analyses were performed. First, the cohort of 674 patients was followed for 3 yrs after study entry and survival rates described. Then, we focused on the subset with incident idiopathic, familial and anorexigen-associated PAH (n=56) combined with prevalent patients who were diagnosed,3 yrs prior to study entry (n=134).In the cohort of 674 patients, 1-, 2-, and 3-yr survival rates were 87% (95% CI 84-90), 76% (95% CI 73-80), and 67% (95% CI 63-71), respectively. In prevalent idiopathic, familial and anorexigen-associated PAH, 1-, 2-, and 3-yr survival rates were higher than in incident patients (p=0.037). In the combined cohort of patients with idiopathic, familial and anorexigen-associated PAH, multivariable analysis showed that survival could be estimated by means of a novel risk-prediction equation using patient sex, 6-min walk distance, and cardiac output at diagnosis.This study highlights survivor bias in prevalent cohorts of PAH patients. Survival of idiopathic, familial and anorexigen-associated PAH can be characterised by means of a novel risk-prediction equation using patients' characteristics at diagnosis.