An Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL Registry
An Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL Registry
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DOI：
10.1378/chest.11-1460
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发表时间：
2012-08-01
期刊：
影响因子：
9.6
通讯作者：
McGoon, Michael D.
中科院分区：
文献类型：
--
作者：
Benza, Raymond L.;Miller, Dave P.;McGoon, Michael D.
背景资料：建立了评价早期和长期肺动脉高压疾病管理的登记研究（REVEAL登记研究），以描述美国肺动脉高压（PAH）患者的临床病程、治疗和结局预测因素。到目前为止，已经建立并报告了基于患者入组时间的估计生存期。为了确定近几十年来PAH患者的生存率是否有所改善，我们评估了REVEAL登记研究队列从诊断时起的生存率，并将这些结果与使用美国国立卫生研究院（NIH）预后方程估计的生存率进行比较。方法：新诊断或既往诊断患者2006年3月至2009年12月在55家美国中心入组的PAH患者（诊断时年龄>= 3个月）纳入当前分析。共有2，635名患者符合本分析的条件。诊断性右心导管插入术后1年、3年、5年和7年生存率分别为85%、68%、57%和49%。对于特发性/家族性PAH患者，生存率分别为91% +/-2%、74% +/-2%、65% +/-3%和59% +/- 3%，而使用NIH方程估计的生存率分别为68%、47%、36%和32%。对一个大型PAH患者队列从诊断时起的生存期进行的综合分析表明，自NIH登记研究建立以来，其影响很可能反映了治疗变化、患者支持策略改善以及PAH人群可能与其他队列不同的组合。试验注册：ClinicalTrials.gov;编号：NCT 00370214; URL：clinicaltrials.gov. CHEST 2012;142（2）：448-456
Background: The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry) was established to characterize the clinical course, treatment, and predictors of outcomes in patients with pulmonary arterial hypertension (PAH) in the United States. To date, estimated survival based on time of patient enrollment has been established and reported. To determine whether the survival of patients with PAH has improved over recent decades, we assessed survival from time of diagnosis for the REVEAL Registry cohort and compared these results to the estimated survival using the National Institutes of Health (NIH) prognostic equation.Methods: Newly or previously diagnosed patients (aged >= 3 months at diagnosis) with PAH enrolled from March 2006 to December 2009 at 55 US centers were included in the current analysis.Results: A total of 2,635 patients qualified for this analysis. One-, 3-, 5-, and 7-year survival rates from time of diagnostic right-sided heart catheterization were 85%, 68%, 57%, and 49%, respectively. For patients with idiopathic/familial PAH, survival rates were 91% +/- 2%, 74% +/- 2%, 65% +/- 3%, and 59% +/- 3% compared with estimated survival rates of 68%, 47%, 36%, and 32%, respectively, using the NIH equation.Conclusions: Comprehensive analysis of survival from time of diagnosis in a large cohort of patients with PAH suggests considerable improvements in survival in the past 2 decades since the establishment of the NIH registry, the effects of which most likely reflect a combination of changes in treatments, improved patient support strategies, and possibly a PAH population at variance with other cohorts.Trial Registry: ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov. CHEST 2012;142(2):448-456