Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia; Respiratory Department, Sunshine Coast University Hospital, Birtinya, QLD, Australia.
Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Camperdown, NSW, Australia.
Chest. 2020 Jan;157(1):162-172. doi: 10.1016/j.chest.2019.08.2203. Epub 2019 Sep 26.
Pulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies; however, the long-term outlook remains poor. Objective multiparametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score is a new model proposed for the follow-up of patients with PAH but has not been externally validated.
The REVEAL 2.0 risk score was applied to a mixed prevalent and incident cohort of patients with PAH (n = 1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier survival was estimated for each REVEAL 2.0 risk score strata and for a simplified three-category (low, intermediate, and high risk) model. Sensitivity analysis was performed on an incident-only cohort.
The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ Registry cohort, with a C statistic of 0.74 (both for full eight-tier and three-category models). When applied to incident cases only, the C statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12- and 60-month survival estimates (all risk category comparisons P < .001). Although the full eight-tier REVEAL 2.0 model separated patients at low, intermediate, and high risk, survival estimates overlapped within some of the intermediate- and high-risk strata.
The REVEAL 2.0 risk score was validated in a large external cohort from the PHSANZ Registry. The REVEAL 2.0 model can be applied for risk assessment of patients with PAH at follow-up. The simplified three-category model may be preferred for clinical use and for future comparison with other prognostic models.
肺动脉高压（PAH）的靶向治疗改善了预后，但长期预后仍然较差。建议采用多参数综合风险评估来识别有早期发病和死亡风险的患者，并优化治疗。美国评估肺动脉高压早期和长期疾病管理的登记处（REVEAL）2.0 风险评分是一种新的模型，用于 PAH 患者的随访，但尚未经过外部验证。
REVEAL 2.0 风险评分应用于澳大利亚和新西兰肺高血压学会（PHSANZ）登记处的 PAH 混合现患和新发队列患者（n=1011）。为每个 REVEAL 2.0 风险评分层和简化的三分类（低、中、高风险）模型估计 Kaplan-Meier 生存。对仅发生的队列进行敏感性分析。
REVEAL 2.0 模型在大型外部 PHSANZ 登记处队列中有效地区分了风险，C 统计量为 0.74（对于完整的八分层和三分类模型均如此）。仅应用于新发病例时，C 统计量为 0.73。三分类 REVEAL 2.0 模型可明确区分 12 个月和 60 个月的生存估计（所有风险类别比较 P<0.001）。尽管完整的八分层 REVEAL 2.0 模型可将患者分为低、中、高风险组，但一些中危和高危分层的生存估计值重叠。
REVEAL 2.0 风险评分在 PHSANZ 登记处的大型外部队列中得到验证。REVEAL 2.0 模型可用于 PAH 患者的随访风险评估。简化的三分类模型可能更适合临床应用，并可用于未来与其他预后模型的比较。
