Predicting Survival in Pulmonary Arterial Hypertension Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)
Predicting Survival in Pulmonary Arterial Hypertension Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)
复制标题
DOI：
10.1161/circulationaha.109.898122
复制
发表时间：
2010-07-13
期刊：
影响因子：
37.8
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：决定肺动脉高压（PAH）患者生存的因素驱动着临床治疗。定量生存预测工具尚未建立用于研究或临床应用。方法和结果：对2716名PAH患者的数据进行分析，这些患者在美国早期和长期PAH疾病管理评估登记处（REVEAL）连续登记，以评估1年生存率的预测因素。我们确定了独立的生存预后因素，并推导出一个多变量、加权风险公式供临床使用。入组之日起一年生存率为91.0%（95%可信区间[CI]， 89.9 ~ 92.1）。在Cox比例风险的多变量分析中，与死亡率增加独立相关的变量包括肺血管阻力bbb32 Wood单位（风险比[HR]， 4.1; 95% CI， 2.0至8.3），PAH与门静脉高压相关（HR, 3.6; 95% CI， 2.4至5.4），修改后的纽约心脏协会/世界卫生组织功能等级IV (HR, 3.1; 95% CI， 2.2至4.4)，男性>60岁（HR, 2.2; 95% CI， 1.6至3.0），以及PAH家族史(HR, 2.2；95% CI, 1.2 - 4.0)。肾功能不全、与结缔组织疾病相关的PAH、功能III级、平均右心房压、静息收缩压和心率、6分钟步行距离、脑利钠肽、预测一氧化碳扩散能力的百分比、超声心动图上的心包积液都能预测死亡率。基于这些多变量分析，推导了一个预测方程，并通过自举技术进行了验证。结论：我们根据患者最近的评估确定了关键的生存预测因素，并制定了当代预后方程。使用这一工具可能允许个体化和优化治疗策略。有必要进行连续随访和重新评估。
Background-Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use.Methods and Results-Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique.Conclusions-We identified key predictors of survival based on the patient's most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted.