Predicting Survival in Patients With Pulmonary Arterial Hypertension The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies
Predicting Survival in Patients With Pulmonary Arterial Hypertension The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies
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DOI：
10.1016/j.chest.2019.02.004
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发表时间：
2019-08-01
期刊：
影响因子：
9.6
通讯作者：
Frantz, Robert P.
中科院分区：
文献类型：
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作者：
Benza, Raymond L.;Gomberg-Maitland, Mardi;Frantz, Robert P.
背景：肺动脉高压是一种进行性、致命的疾病。已发布的治疗指南建议在定期患者评估的基础上升级治疗，以达到或维持低风险状态。有多种策略可用于确定风险状态。该分析描述了评估早期和长期 PAH 疾病管理 (REVEAL) 风险计算器登记处 (REVEAL 2.0) 的更新，并将其与最近发布的欧洲心脏病学会/呼吸学会指南衍生的风险评估策略进行比较。 方法：对来自美国登记处 REVEAL 的入组后存活 1 年以上的亚群（该队列的基线）进行了分析。对于 REVEAL 2.0，重新评估了点值和分界点，并评估了新变量。 Kaplan-Meier 方法用于估计基线后 12 个月的生存率；使用 c 统计量对歧视进行量化。对 REVEAL 2.0 与新启动肺动脉高压治疗的前瞻性比较登记处 (COMPERA) 和法国肺动脉高压登记处 (FPHR) 风险评估策略的死亡率估计和歧视进行了比较。为了进行比较，计算了三类 REVEAL 2.0 评分，其中将患者分为低风险、中风险或高风险。 结果：REVEAL 2.0 在该亚群中表现出与原始计算器类似的区分能力（c 统计量 = 0.76 vs 0.74），在风险类别之间提供了出色的风险分离，并预测了随访 >= 1 年的患者的临床恶化和死亡率。 REVEAL 2.0 三类分数的辨别力（c 统计量 = 0.73）比 COMPERA（c 统计量 = 0.62）或 FPHR（c 统计量 = 0.64）具有更大的辨别力。与 REVEAL 2.0 相比，COMPERA 和 FPHR 均低估和高估了风险。结论：REVEAL 2.0 对 REVEAL 入组的患者表现出比 COMPERA 和 FPHR 风险评估策略更大的风险歧视。经过外部验证后，REVEAL 2.0 计算器可以帮助临床医生和患者根据个人风险状况做出明智的治疗决策。
BACKGROUND: Pulmonary arterial hypertension is a progressive, fatal disease. Published treatment guidelines recommend treatment escalation on the basis of regular patient assessment with the goal of achieving or maintaining low-risk status. Various strategies are available to determine risk status. This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (REVEAL 2.0) and compares it with recently published European Society of Cardiology/ Respiratory Society guideline-derived risk assessment strategies.METHODS: A subpopulation from the US-based registry REVEAL that survived >= 1 year postenrollment (baseline for this cohort) was analyzed. For REVEAL 2.0, point values and cutpoints were reassessed, and new variables were evaluated. The Kaplan-Meier method was used to estimate survival at 12 months postbaseline; discrimination was quantified using the c-statistic. Mortality estimates and discrimination were compared between REVEAL 2.0 and Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and French Pulmonary Hypertension Registry (FPHR) risk assessment strategies. For this comparison, a three-category REVEAL 2.0 score was computed in which patients were classified as low-, intermediate-, or high-risk.RESULTS: REVEAL 2.0 demonstrated similar discrimination as the original calculator in this subpopulation (c-statistic = 0.76 vs 0.74), provided excellent separation of risk among the risk categories, and predicted clinical worsening as well as mortality in patients who were followed >= 1 year. The REVEAL 2.0 three-category score had greater discrimination (c-statistic = 0.73) than COMPERA (c-statistic = 0.62) or FPHR (c-statistic = 0.64). Compared with REVEAL 2.0, COMPERA and FPHR both underestimated and overestimated risk.CONCLUSION: REVEAL 2.0 demonstrates greater risk discrimination than the COMPERA and FPHR risk assessment strategies in patients enrolled in REVEAL. After external validation, the REVEAL 2.0 calculator can assist clinicians and patients in making informed treatment decisions on the basis of individual risk profiles.